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Sample records for epiphyses

  1. Multiple epiphyseal dysplasia

    PubMed Central

    2009-01-01

    Background Multiple epiphyseal dysplasia (MED) is a common genetically and clinically heterogeneous skeletal dysplasia characterized by early-onset osteoarthritis, mainly in the hip and knee, and mild-to-moderate short stature. Here we report on a 6-generation MED family with 17 affected members. Method The clinical and radiographic data on the 12 affected members still living were scrutinized. A structured inquiry comprising state of health and MED-related symptoms since birth up to the present time and the osteoarthritis outcome (KOOS) questionnaire were sent to all living family members with MED. The 5 known gene loci for autosomal dominant MED were analyzed for linkage, using fluorescence-labeled microsatellite markers. Linkage was ascertained with markers close to the COL9A2 gene, which was analyzed for mutations by sequencing. Results We identified an exon 3 donor splice mutation in the COL9A2 gene in all affected family members. Clinical, radiographic, and questionnaire data from affected family members suggested that MED caused by COL9A2 mutations starts in early childhood with knee pain accompanied by delayed ossification of femoral epiphyses. The disease then either stabilizes during puberty or progresses with additional joints becoming affected; joint surgery might be necessary. The progression of the disease also affects muscles, with increasing atrophy, resulting in muscle fatigue and pain. Muscular atrophy has not been reported earlier in cases with COL9A2 mutations. Interpretation In a patient with clinically suspected or verified MED, it is important to perform DNA-based analysis to identify a possible disease-causing mutation. This information can be used to carry out genetic risk assessment of other family members and to achieve an early and correct diagnosis in the children. PMID:19995321

  2. Genetics Home Reference: multiple epiphyseal dysplasia

    MedlinePlus

    ... health and development? More about Mutations and Health Inheritance Pattern Multiple epiphyseal dysplasia can have different inheritance patterns. This condition can be inherited in an autosomal ...

  3. Photodynamic impact on the epiphyseal plates.

    PubMed

    Kurchenko, S; Shashko, A; Dudin, M; Mikhailov, V; Netylko, G; Ashmarov, V

    2012-01-01

    This study was carried out to prove the possibility of inhibition of long bones epiphyseal plates activity with photodynamic impact. Comparative analysis of the Chlorin E6 accumulation with transcutaneous and intraperitoneal administration mode, carried out on 175 laboratory mice showed the drug accumulates selectively in the epiphyseal plates of long bones, regardless of the mode of administration. 15 mice (males and females) at the age of active grownig were subjected to the single laser radiation impact on the knee joints area: 5 ones with transcutaneous Chlorine E6 administration, another 5 ones with intraperitoneal administration and the rest 5 without the drug. Histological samples of 15 experimental mice epiphyseal plates were examined by light microscopy, compared with 10 intact control mice. Influence of the laser radiation without administration of Chlorin E6 leads to intracellular swelling of epiphyseal plates chondrocytes. Influence of the laser radiation after transcutaneous or intraperitoneal injection of Chlorine E6 reduces significantly the total number of epiphyseal plates chondrocytes, without reducing the proportion of terminally-differentiated chondrocytes. Thus, the photodynamic impact inhibits the activity of epiphyseal plates of the mice.

  4. Epiphyseal and physeal cartilage: normal gadolinium-enhanced MR imaging.

    PubMed

    Li, Xiaoming; Wang, Renfa; Li, Yonggang; Tang, Lihua; Hu, Junwu; Xu, Anhui

    2005-01-01

    To evaluate the normal appearance of epiphyseal and physeal cartilage on Gadolinium (Gd)-enhanced MR imaging. The appearance and enhancement ratios of 20 proximal and distal femoral epiphyses in 10 normal piglets were analyzed on Gd-enhanced MR images. The correlation of the MR imaging appearance with corresponding histological findings of immature epiphyses was examined. Our results showed that Gd-enhanced MRI could differentiate the differences in enhancement between physeal and epiphyseal cartilage and show vascular canals within the epiphyseal cartilage. Enhanced ratios in the physeal were greater than those in the epiphyseal cartilage (P < 0.005). It is concluded that Gd-enhanced MR imaging reveals epiphyseal vascular canals and shows difference in enhancement of physeal and epiphyseal cartilage.

  5. Genetic heterogeneity in multiple epiphyseal dysplasia

    SciTech Connect

    Deere, M.; Blanton, S.H.; Scott, C.I.

    1994-09-01

    Multiple epiphyseal dysplasia (MED) is generally an autosomal dominant hereditary chondrodystrophy characterized by abnormal epiphyseal centers of the long bones. There are at least two clinical and radiographical MED phenotypes, Fairbank and Ribbing forms, with the former having been better characterized. While less frequent, there are also reports of an autosomal recessive type which does not differ radiographically from the autosomal dominant type. Recently, a family with MED has been shown to map to the pericentromeric region of chromosome 19. We have tested linkage to six short tandem repeat markers from chromosome 19 in three multigenerational families with Fairbank MED and another MED family in which there were three of seven affected siblings with unaffected parents. The three families with autosomal dominant MED were linked to D19S215 with a maximum lod score of 3.82 at {theta} = 0.0. Linkage to chromosome 19 was excluded in the fourth family under autosomal recessive and autosomal dominant models with either reduced penetrance or germline mosaicism. Lod scores were -{infinity} and -2.37 at {theta} = 0.0 for D19S215, respectively. Linkage to candidate genes, Col9A1, Col9A2, and Col11A1 was tested and excluded for both models in this family. Col11A1 was excluded under a recessive model. We have confirmed linkage of MED, Fairbank, to chromosome 19 and demonstrated that MED is genetically heterogeneous.

  6. Development and validation of new model for microvascular transplantation of epiphyseal plate allografts with minimal adjoining epiphyseal and metaphyseal bone.

    PubMed

    Bray, Peter W; Neligan, Peter C; Bowen, C Vaughan A; Danska, Jayne S; Boyer, Martin I

    2003-01-01

    A model for the free allograft microvascular transplantation of rabbit proximal tibial epiphyseal plate allografts was developed, validated, and tested in an in vivo animal model. Transplants contained the minimum amount of adjacent epiphyseal and metaphyseal bone compatible with preservation of the epiphyseal-plate vascular supply, as determined by corrosion casting. Perfusion to this graft was evaluated quantitatively using radioactive microspheres, and qualitatively using India-ink injection. Female New Zealand White rabbits at 12 weeks of age were utilized. Vascularized transplantation of epiphyseal plate allografts was performed either into a defect of matched size in the iliac crest or into a soft-tissue pocket without bone contact. Cyclosporine A immunosuppression (CSA) was administered daily for 6 weeks. Two control groups underwent identical surgical procedures, but had no postoperative immunosuppression. Epiphyseal plates both with and without bone contact, in rabbits immunosuppressed postoperatively with CSA, demonstrated longitudinal growth and preserved viability as determined by positive bromodeoxyuridine uptake. Control epiphyseal plates transferred without postoperative immunosuppression were uniformly nonviable. This new model has value as a basis for further studies into the clinical applicability of isolated epiphyseal-plate transplants.

  7. Isolation and Characterization of Chick Epiphyseal Cartilage Matrix Vesicle Proteolipid

    DTIC Science & Technology

    1988-01-01

    Epiphyseal growth plate cartilage from the proximal portion of 49-52 day old broiler strain chickens was digested in collagenase for 15 hours. Plasma...cartilage from the proximal portion of 49-52 day old broiler strain chickens was digested in collagenase for 15 hours. Plasma membranes and matrix...ATPASE ACTIVITY. Epiphyseal growth plate cartilage from the proximal portion of 49-52 day old broiler strain chickens was digested in collagenase for 15

  8. [Development and functional structure of the epiphyseal plate].

    PubMed

    Milz, S; Boszczyk, A; Putz, R

    2002-09-01

    The longitudinal growth of long bones occurs in the epiphyseal plates at their ends. After a center of ossification has developed in the epiphysis, a cartilaginous plate, characterized by a typical zonal arrangement is formed. The cartilaginous plate proliferates using cells from a reservoir at the epiphyseal (i.e. articular) side of the plate. The cells are orientated to longitudinal rows and become thickened towards the metaphysis. The extracellular matrix between the hypertrophic chondrocytes becomes calcified and is subsequently replaced in an ossification process by lamellar bone. Cell proliferation in the epiphyseal plate is regulated by a number of different factors (e.g. IGF-I and BMP-7). The extracellular matrix is rich in glycosaminoglycans, proteoglycans and type II collagen, whereas especially at the edges type I and type VI collagen can be observed, too. The three dimensional orientation of the epiphyseal plate is determined by local mechanical influences. The adjacent bony endplates exhibit a higher central mineralization pattern with lower density values at their edges. The region of failure predominantly concerns the proximal zone of the longitudinal rows. The mechanical properties of the epiphyseal plate are influenced by endocrine as well as by metabolic factors. Any case of an epiphysiolysis is caused by a misproportion between acting shear forces and cartilaginous strength.

  9. Microvascular transplantation of epiphyseal plates: studies utilizing allograft donor material.

    PubMed

    Boyer, Martin I; Bowen, C Vaughan A

    2007-01-01

    Compromised function of an epiphyseal plate caused by trauma, tumor, infection, or congenital malformation can result in significant musculoskeletal deformity. Techniques used to correct or minimize the extent of these deformities include autogenous or allogeneic cancellous bone grafts, nonvascularized cortical allografts, vascularized bone and composite tissue transfers, and distraction osteogenesis. These solutions are not ideal for children because they do not adequately address the actively growing nature of the extremity. Microvascular techniques have enabled the experimental transplantation of vascularized epiphyseal plates with high levels of postoperative viability and subsequent growth and offer a potential advantage over conventional treatments.

  10. [Epiphyseal fractures of the distal tibia. Treatment and results].

    PubMed

    Wicky, B; Stauffer, U G

    1982-11-01

    Epiphyseal fractures of the distal end of the tibia generally require operative treatment. A conservative treatment can be recommended only in minimally displaced fractures with undisturbed ankle joint congruity. Among 48 patients treated (40 operatively, 8 conservatively) 6 developed angular deformities, 3 of which required a corrective osteotomy. After the initial treatment follow-up is necessary until bone growth is complete.

  11. Effects of a sliding plate on morphology of the epiphyseal plate in goat distal femur.

    PubMed

    Lin, Da-sheng; Lian, Ke-jian; Hong, Jia-yuan; Ding, Zhen-qi; Zhai, Wen-liang

    2012-01-01

    The aim of this study was to observe the effects of a sliding plate on the morphology of the epiphyseal plate in goat distal femur. Eighteen premature female goats were divided randomly into sliding plate, regular plate and control groups. Radiographic analysis and histological staining were performed to evaluate the development of epiphyseal plate at 4 and 8 weeks after surgery. In the sliding plate group, the plate extended accordingly as the epiphyseal plate grows, and the epiphyseal morphology was kept essential normal. However, the phenomenon of the epiphyseal growth retardation and premature closure were very common in the regular plate group. In addition, the sliding plate group exhibited more normal histologic features and Safranin O staining compared to the regular plate group. Our results suggest that the sliding plate can provide reliable internal fixation of epiphyseal fracture without inhibiting epiphyseal growth.

  12. Effects of a Sliding Plate on Morphology of the Epiphyseal Plate in Goat Distal Femur

    PubMed Central

    LIN, Da-sheng; LIAN, Ke-jian; HONG, Jia-yuan; DING, Zhen-qi; ZHAI, Wen-liang

    2012-01-01

    The aim of this study was to observe the effects of a sliding plate on the morphology of the epiphyseal plate in goat distal femur. Eighteen premature female goats were divided randomly into sliding plate, regular plate and control groups. Radiographic analysis and histological staining were performed to evaluate the development of epiphyseal plate at 4 and 8 weeks after surgery. In the sliding plate group, the plate extended accordingly as the epiphyseal plate grows, and the epiphyseal morphology was kept essential normal. However, the phenomenon of the epiphyseal growth retardation and premature closure were very common in the regular plate group. In addition, the sliding plate group exhibited more normal histologic features and Safranin O staining compared to the regular plate group. Our results suggest that the sliding plate can provide reliable internal fixation of epiphyseal fracture without inhibiting epiphyseal growth. PMID:22359485

  13. Effects of estrogen on growth plate senescence and epiphyseal fusion.

    PubMed

    Weise, M; De-Levi, S; Barnes, K M; Gafni, R I; Abad, V; Baron, J

    2001-06-05

    Estrogen is critical for epiphyseal fusion in both young men and women. In this study, we explored the cellular mechanisms by which estrogen causes this phenomenon. Juvenile ovariectomized female rabbits received either 70 microg/kg estradiol cypionate or vehicle i.m. once a week. Growth plates from the proximal tibia, distal tibia, and distal femur were analyzed after 2, 4, 6, or 8 weeks of treatment. In vehicle-treated animals, there was a gradual senescent decline in tibial growth rate, rate of chondrocyte proliferation, growth plate height, number of proliferative chondrocytes, number of hypertrophic chondrocytes, size of terminal hypertrophic chondrocytes, and column density. Estrogen treatment accelerated the senescent decline in all of these parameters. In senescent growth plates, epiphyseal fusion was observed to be an abrupt event in which all remaining chondrocytes were rapidly replaced by bone elements. Fusion occurred when the rate of chondrocyte proliferation approached zero. Estrogen caused this proliferative exhaustion and fusion to occur earlier. Our data suggest that (i) epiphyseal fusion is triggered when the proliferative potential of growth plate chondrocytes is exhausted; and (ii) estrogen does not induce growth plate ossification directly; instead, estrogen accelerates the programmed senescence of the growth plate, thus causing earlier proliferative exhaustion and consequently earlier fusion.

  14. Peri-epiphyseal and Overuse Injuries in Adolescent Athletes

    PubMed Central

    Frush, Todd J.; Lindenfeld, Thomas N.

    2009-01-01

    Context: Overuse injuries of the musculoskeletal system in immature athletes are commonly seen in medical practice. Evidence Acquisition: An analysis of published clinical, outcome, and biomechanical studies of adolescent epiphyseal and overuse injuries was performed through 2008 to increase recognition and provide treatment recommendations. Results: Adolescent athletes can sustain physeal and bony stress injuries. Recovery and return to play occur more swiftly if such injuries are diagnosed early and immobilized until the patient is pain-free, typically about 4 weeks for apophyseal and epiphyseal overuse injuries. Certain epiphyseal injuries have prolonged symptoms with delayed treatment, including those involving the bones in the hand, elbow, and foot. If such injuries are missed, prolonged healing and significant restrictions in athletic pursuits may occur. Conclusion: Some of these injuries are common to all weightbearing sports and are therefore widely recognized. Several are common in gymnastics but are rarely seen in other athletes. Early recognition and treatment of these conditions lead to quicker recovery and so may prevent season-ending, even career-ending, events from occurring. PMID:23015873

  15. Traumatic separation of epiphyses. An experimental study in rats.

    PubMed

    Gomes, L S; Volpon, J B; Goncalves, R P

    1988-11-01

    Four types of physeal fracture-separations, defined by the Salter-Harris classification, were created experimentally in the proximal physis of the right tibia of immature rats. The four types are: Type I, pure epiphyseal separation; Type II, separation of the epiphysis with a metaphyseal fracture; Type III, partial epiphyseal separation with a vertical fracture of the epiphysis; and Type IV, vertical epiphyseal and metaphyseal fractures. A sham operation performed on the left tibia served as the control. The animals were killed at various intervals up to 25 days after the operation. The findings were assessed by roentgenographic, histologic, and vessel injection methods. For Types I and II morphometric analyses were also performed. The influence of various types of lesions on the growth rate and the healing process was documented. For Types I and II lesions there was a transitory growth arrest and an increased thickening of the zone of hypertrophic cells. The alterations regressed after Day 15, and by day 25, a nearly normal plate was seen. For Type III lesions an angular deformity of the tibia occurred that increased with time. For Type IV lesions, a step-off developed on the articular surface that became more severe with time. Early vascular anastomoses between the epiphysis and the metaphysis led to the formation of bone bridges in Type III and Type IV lesions.

  16. Does the epiphyseal cartilage of the long bones have one or two ossification fronts?

    PubMed

    Delgado-Martos, María Jesús; Touza Fernández, Alberto; Canillas, Fernando; Quintana-Villamandos, Begoña; Santos del Riego, Sergio; Delgado-Martos, Emilio; Martos-Rodriguez, Antonia; Delgado-Baeza, Emilio

    2013-10-01

    Epiphyseal cartilage is hyaline cartilage tissue with a gelatinous texture, and it is responsible for the longitudinal growth of the long bones in birds and mammals. It is located between the epiphysis and the diaphysis. Epiphyseal cartilage also is called a growth plate or physis. It is protected by three bone components: the epiphysis, the bone bar of the perichondrial ring and the metaphysis. The epiphysis, which lies over the epiphyseal cartilage in the form a cupola, contains a juxtaposed bone plate that is near the epiphyseal cartilage and is in direct contact with the epiphyseal side of the epiphyseal cartilage. The germinal zone corresponds to a group of cells called chondrocytes. These chondrocytes belong to a group of chondral cells, which are distributed in rows and columns; this architecture is commonly known as a growth plate. The growth plate is responsible for endochondral bone growth. The aim of this study was to elucidate the causal relationship between the juxtaposed bone plate and epiphyseal cartilage in mammals. Our hypothesis is that cells from the germinal zone of the epiphyseal side of the epiphyseal cartilage are involved in forming a second ossification front that is responsible for the origin of the juxtaposed bone plate. We report the following: (a) The juxtaposed bone plate has a morphology and function that differs from that of the epiphyseal trabeculae; (b) on the epiphyseal edge of the epiphyseal cartilage, a new ossification front starts on the chondrocytes of the germinal area, which forms the juxtaposed bone plate. This ossification front is formed by chondrocytes from the germinal zone through a process of mineralisation and ossification, and (c) the process of mineralisation and ossification has a certain morphological analogy to the process of ossification in the metaphyseal cartilage of amphibians and differs from the endochondral ossification process in the metaphyseal side of the growth plate. The close relationship between

  17. Preliminary clinical research on epiphyseal distraction in osteosarcoma in children

    PubMed Central

    2014-01-01

    Background The feasibility of distal femur epiphysis preservation through epiphyseal distraction by external fixator in childhood osteosarcoma was explored. Methods Between July 2007 and May 2011, 10 children who were suffering from distal femur osteosarcoma received epiphyseal distraction by external fixator, combined with tumor resection and repair with massive allograft bone to preserve the epiphysis of the distal femur and knee function. There were six male and four female patients, 9- to 14-years old (average 10.5 years old). The tumors were staged clinically according to the Enneking staging method: six cases were classified as stage in IIA and four cases as stage in IIB. All patients were diagnosed by biopsy, then received chemotherapy before and after surgery. All patients received tumor bone resection and the defects of the bone were repaired with massive allograft bone that was fixed by intramedullary nails; the distracted epiphysis and allograft bone were fixed with cancellous screws. Results All cases received follow-up from 15 to 56 months (average 38.5 months). There were no local recurrences. One case died of lung metastasis and one case had poor incision healing for rejection of allograft bone. According to the functional evaluation criteria of the International Society of Limb Salvage (ISOLS) after operation, five cases were rated excellent, four cases good and one case fair. The ratio of excellent or good was 90.0%. There was no statistically significant difference in length between the operated and the normal lower limbs during the last review. Conclusions Epiphyseal distraction by external fixator can result in satisfactory limb length and joint function for children with a malignant bone tumor. PMID:25099460

  18. The persistence of epiphyseal scars in the distal radius in adult individuals.

    PubMed

    Davies, Catriona; Hackman, Lucina; Black, Sue

    2016-01-01

    The use of radiographic imaging in the estimation of chronological age facilitates the analysis of structures not visible on gross morphological inspection. Following the completion of epiphyseal fusion, a thin radio-opaque band, the epiphyseal scar, may be observed at the locus of the former growth plate. The obliteration of this feature has previously been interpreted as the final stage of skeletal maturation and consequently has been included as a criterion in several methods of age estimation, particularly from the distal radius. Due to the recommendations relating to age estimation in living individuals, accurate assessment of age from the distal radius is of great importance in human identification; however, the validity of the interpretation of the obliteration of the epiphyseal scar as an age-related process has not been tested. A study was undertaken to assess the persistence of epiphyseal scars in adults between 20 and 50 years of age through the assessment of 616 radiographs of left and right distal radii from a cross-sectional population. This study found that 86% of females and 78% of males retained some remnant of the epiphyseal scar in the distal radius. The relationships between chronological age, biological sex and the persistence of the epiphyseal scar were not statistically significant. The findings of this study indicate that the epiphyseal scars may persist in adult individuals until at least 50 years of age. No maximum age should therefore be applied to the persistence of an epiphyseal scar in the distal radius.

  19. Epiphyseal growth plate and secondary peripheral chondrosarcoma: the neighbours matter.

    PubMed

    de Andrea, Carlos E; Hogendoorn, Pancras C W

    2012-01-01

    Chondrocytes interact with their neighbours through their cartilaginous extracellular matrix (ECM). Chondrocyte-matrix interactions compensate the lack of cell-cell contact and are modulated by proteoglycans and other molecules. The epiphyseal growth plate is a highly organized tissue responsible for long bone elongation. The growth plate is regulated by gradients of morphogens that are established by proteoglycans. Morphogens diffuse across the ECM, creating short- and long-range signalling that lead to the formation of a polarized tissue. Mutations affecting genes that modulate cell-matrix interactions are linked to several human disorders. Homozygous mutations of EXT1/EXT2 result in reduced synthesis and shortened heparan sulphate chains on both cell surface and matrix proteoglycans. This disrupts the diffusion gradients of morphogens and signal transduction in the epiphyseal growth plate, contributing to loss of cell polarity and osteochondroma formation. Osteochondromas are cartilage-capped bony projections arising from the metaphyses of endochondral bones adjacent to the growth plate. The osteochondroma cap is formed by cells with homozygous mutation of EXT1/EXT2 and committed stem cells/wild-type chondrocytes. Osteochondroma serves as a niche (a permissive environment), which facilitates the committed stem cells/wild-type chondrocytes to acquire secondary genetic changes to form a secondary peripheral chondrosarcoma. In such a scenario, the micro-environment is the site of the initiating processes that ultimately lead to cancer.

  20. VESICLES ASSOCIATED WITH CALCIFICATION IN THE MATRIX OF EPIPHYSEAL CARTILAGE

    PubMed Central

    Anderson, H. Clarke

    1969-01-01

    Vesicles have been identified within the cartilage matrix of the upper tibial epiphyseal plate of normal mice. They were seen at all levels within the plate and usually did not appear to be in contact with cartilage cells. Vesicles were concentrated within the matrix of the longitudinal septa from the proliferative zone downward. They varied considerably in size (∼300 A to ∼1 µ) and in shape. They were bounded by unit membranes, and contained materials of varying density including, rarely, ribosomes. A close association was demonstrated between matrix vesicles and calcification: in the lower hypertrophic and calcifying zones of the epiphysis, vesicles were found in juxtaposition to needle-like structures removed by demineralization with ethylenediaminetetraacetate and identified by electron diffraction as hydroxyapatite and/or fluorapatite crystal structure—the former being indistinguishable from the latter for most cases in which electron diffraction methods are employed. Decalcification also revealed electron-opaque, partially membrane-bounded structures within previously calcified cartilage of the epiphyseal plate and underlying metaphysis which corresponded in size and distribution to matrix vesicles. It is suggested that matrix vesicles are derived from cells and that they may play a role in initiating calcification at the epiphysis. PMID:5775794

  1. Neuro-epiphyseal injury around the ankle: a case report.

    PubMed

    Chauhan, Devendra Kumar; Dhillon, Mandeep S; Tripathy, Sujit Kumar

    2009-06-01

    An epiphyseal injury without obvious trauma is a diagnostic dilemma; in neurologically impaired children, the lack of sensation and repetitive trauma can lead to neuropathic injuries of the physeal plate, with bizarre X-ray changes; the problem is compounded by lack of awareness in the average orthopaedist, leading to treatment delays and unnecessary procedures like biopsy, or even surgery for osteomyelitis or malignancy, with increased morbidity and disability. We report a 12-year male child with myelomeningocele and neurological deficit; he presented with painless swelling and redness of left leg and sensory-motor deficit below the knee joints. X-rays showed distal tibial epiphysis plate widening and irregularity. A diagnosis of neuropathic epiphyseal plate injury was considered; the clinico-radiological picture only resolved after rigid immobilization in below knee POP casts and abstinence of weight bearing. Lesser changes were noted in the opposite side also. The injured physis displaced somewhat posteromedially over 8 months, but ultimately fused; contra-lateral changes subsided with partial physeal fusion. The objective of reporting this unusual case is to increase the awareness levels, and to report the bizarre radiological changes. Management involves non-weight bearing immobilization; diagnostic delays lead to more reactive changes with higher incidence of subsequent physeal closure.

  2. Epiphyseal fusion in Pan troglodytes relative to dental age.

    PubMed

    Brimacombe, Conrad S; Kuykendall, Kevin L; Nystrom, Pia

    2015-05-01

    Previous studies on different aspects of chimpanzee growth and development have documented dental eruption and development, long bone and somatic growth, and to a lesser extent, skeletal fusion. Such data are useful in comparative and evolutionary studies of growth and some aspects of life history evolution in apes and early hominids. However, few studies have integrated dental development and other aspects of skeletal development, and none of these have been able to incorporate a large study sample. This study documents dental mineralization and skeletal epiphyseal fusion in a mixed-sex sample of 155 Pan troglodytes skeletons, and aims to: a) document the pattern of dental and skeletal developmental in chimpanzees; b) compare male and female developmental patterns in chimpanzees; and c) compare these chimpanzee developmental patterns to general patterns of dental and skeletal development in published human studies. The analysis of both dental and skeletal development in this sample demonstrates clearly that dental development is complete before the fusion of the many skeletal epiphyses, in contrast to the pattern observed in humans. Age estimates for individuals were calculated using previously published regression equations for dental development and used to estimate fusion ages. These appear to be accurate in that our estimates are similar to published ranges. These data improve our understanding about chimpanzee dental and skeletal development and provide a basis for further comparison between extant apes and humans, as well as those extinct species represented by fossil partial skeletons.

  3. Epiphyseal osteosarcoma revisited: four illustrative cases with unusual histopathology and literature review.

    PubMed

    Chow, Louis Tsun Cheung; Wong, Simon Kwok Chuen

    2015-01-01

    Osteosarcomas arising in the epiphysis are extremely rare and easily missed in the diagnostic consideration of epiphyseal tumors. It is the purpose of this study to delineate the clinical pathological characteristics of 'epiphyseal osteosarcoma' under the definition of 'a solitary long bone osteosarcoma radiographically considered an epiphyseal tumor for which the main radiologic differential diagnosis would encompass giant cell tumor, chondroblastoma and clear cell chondrosarcoma'. Four such cases with unusual histopathology were retrieved among 110 cases of osteosarcoma. Their clinical, radiological and pathological features, together with all 10 reported cases, were analyzed. The radiographic diagnoses of our four cases include two giant cell tumors, one chondroblastoma and one clear cell chondrosarcoma but turn out to be fibroblastic, giant cell rich, telangiectatic and epithelioid variant of epiphyseal osteosarcoma. Including our patients, the 14 reported epiphyseal osteosarcomas comprise 8 males and 6 females, the age at presentation ranges from 11 to 39 years, two-third in the second decade, 71.4% affect the femur. Due to their epiphyseal locations, many carry benign radiological diagnoses notably giant cell tumor and chondroblastoma. Epiphyseal osteosarcomas may not only masquerade as benign radiological bony lesions but also assume many histological patterns; orthopedic surgeons, radiologists and pathologists should be aware of such possibility. Their behavior and prognosis are dictated by the histologic types, grading and staging rather than location.

  4. Age estimation from stages of epiphyseal union in the presacral vertebrae.

    PubMed

    Cardoso, Hugo F V; Ríos, Luis

    2011-02-01

    The presacral vertebrae have various secondary centers of ossification, whose timing of fusion can be used for age estimation of human skeletal remains up to the middle to the latter third decade. However, detailed information about the age at which these secondary centers of ossification fuse has been lacking. In this study, the timing of epiphyseal union in presacral vertebrae was studied in a sample of modern Portuguese skeletons (57 females and 47 males) between the ages of 9 and 30, taken from the Lisbon documented skeletal collection. A detailed photographic record of these epiphyses and the age ranges for the different stages of epiphyseal union are provided. Partial union of epiphyses was observed from 11 to 27 years of age. In general, centers of ossification begin to fuse first in the cervical and lumbar vertebrae, followed by centers of ossification in the thoracic region. The first center of ossification to complete fusion is usually that of the mammillary process in lumbar vertebrae. This is usually followed by that of the transverse process, spinous transverse process, and annular ring, regardless of vertebra type. There were no statistically significant sex differences in timing of fusion, but there was a trend toward early maturation in females for some vertebra or epiphyses. Bilateral epiphyses did not show statistically significant differences in timing of fusion. This study offers information on timing of fusion of diverse epiphyseal locations useful for age estimation of complete or fragmented human skeletal remains.

  5. Multiparametric MRI of Epiphyseal Cartilage Necrosis (Osteochondrosis) with Histological Validation in a Goat Model

    PubMed Central

    Wang, Luning; Nissi, Mikko J.; Tóth, Ferenc; Shaver, Jonah; Johnson, Casey P.; Zhang, Jinjin; Garwood, Michael; Carlson, Cathy S.; Ellermann, Jutta M.

    2015-01-01

    Purpose To evaluate multiple MRI parameters in a surgical model of osteochondrosis (OC) in goats. Methods Focal ischemic lesions of two different sizes were induced in the epiphyseal cartilage of the medial femoral condyles of goats at 4 days of age by surgical transection of cartilage canal blood vessels. Goats were euthanized and specimens harvested 3, 4, 5, 6, 9 and 10 weeks post-op. Ex vivo MRI scans were conducted at 9.4 Tesla for mapping the T1, T2, T1ρ, adiabatic T1ρ and TRAFF relaxation times of articular cartilage, unaffected epiphyseal cartilage, and epiphyseal cartilage within the area of the induced lesion. After MRI scans, safranin O staining was conducted to validate areas of ischemic necrosis induced in the medial femoral condyles of six goats, and to allow comparison of MRI findings with the semi-quantitative proteoglycan assessment in corresponding safranin O-stained histological sections. Results All relaxation time constants differentiated normal epiphyseal cartilage from lesions of ischemic cartilage necrosis, and the histological staining results confirmed the proteoglycan (PG) loss in the areas of ischemia. In the scanned specimens, all of the measured relaxation time constants were higher in the articular than in the normal epiphyseal cartilage, consistently allowing differentiation between these two tissues. Conclusions Multiparametric MRI provided a sensitive approach to discriminate between necrotic and viable epiphyseal cartilage and between articular and epiphyseal cartilage, which may be useful for diagnosing and monitoring OC lesions and, potentially, for assessing effectiveness of treatment interventions. PMID:26473611

  6. Time course of epiphyseal growth plate fusion in rat tibiae.

    PubMed

    Martin, E A; Ritman, E L; Turner, R T

    2003-03-01

    Although the rat is the most common animal model used in studying osteoporosis, it is often used inappropriately. Osteoporosis is a disease that most commonly occurs in humans long after growth plate fusion with the associated cessation of longitudinal bone growth, but there has been a question as to when or to what extent the rat growth plate fuses. To investigate this question, we used microcomputed X-ray tomography, at voxel resolutions ranging from (5.7 micro m)(3) to (11 micro m)(3), to image the proximal epiphyseal growth plates of both male (n = 19) and female (n = 15) rat tibiae, ranging in age from 2 to 25 months. The three-dimensional images were used to evaluate fusion of the epiphyseal growth plate by quantitating the amount of cancellous bone that has bridged across the growth plate. The results suggest that the time course of fusion of the epiphyseal growth plate follows a sigmoidal pattern, with 10% of the maximum number of bridges having formed by 3.9 months in the male tibiae and 5.8 months in the female tibiae, 50% of the maximum number of bridges having formed by 5.6 months in the male tibiae and 5.9 months in the female tibiae, and 90% of the total maximum of bridges have formed by 7.4 months for the males and 6.5 months for the females. The total volume of bridges per tibia at the age at which the maximum number of bridges per tibia has first formed is 0.99 mm(3)/tibia for the males and 0.40 mm(3)/tibia for the females. After the maximum number of bridges (-290 for females, -360 for males) have formed the total volume of bridges per tibia continues to increase for an additional 7.0 months in the males and 17.0 months for the females until they reach maximum values (-1.5 mm(3)/tibia for the males and -2.2 mm(3)/tibia for the females).

  7. Time course of epiphyseal growth plate fusion in rat tibiae

    NASA Technical Reports Server (NTRS)

    Martin, E. A.; Ritman, E. L.; Turner, R. T.

    2003-01-01

    Although the rat is the most common animal model used in studying osteoporosis, it is often used inappropriately. Osteoporosis is a disease that most commonly occurs in humans long after growth plate fusion with the associated cessation of longitudinal bone growth, but there has been a question as to when or to what extent the rat growth plate fuses. To investigate this question, we used microcomputed X-ray tomography, at voxel resolutions ranging from (5.7 micro m)(3) to (11 micro m)(3), to image the proximal epiphyseal growth plates of both male (n = 19) and female (n = 15) rat tibiae, ranging in age from 2 to 25 months. The three-dimensional images were used to evaluate fusion of the epiphyseal growth plate by quantitating the amount of cancellous bone that has bridged across the growth plate. The results suggest that the time course of fusion of the epiphyseal growth plate follows a sigmoidal pattern, with 10% of the maximum number of bridges having formed by 3.9 months in the male tibiae and 5.8 months in the female tibiae, 50% of the maximum number of bridges having formed by 5.6 months in the male tibiae and 5.9 months in the female tibiae, and 90% of the total maximum of bridges have formed by 7.4 months for the males and 6.5 months for the females. The total volume of bridges per tibia at the age at which the maximum number of bridges per tibia has first formed is 0.99 mm(3)/tibia for the males and 0.40 mm(3)/tibia for the females. After the maximum number of bridges (-290 for females, -360 for males) have formed the total volume of bridges per tibia continues to increase for an additional 7.0 months in the males and 17.0 months for the females until they reach maximum values (-1.5 mm(3)/tibia for the males and -2.2 mm(3)/tibia for the females).

  8. Epiphyseal ewing sarcoma: first reported case with molecular confirmation.

    PubMed

    Pérez-González, Yosmar; García-Esparza, Elena; Conde, Esther; Azorín, Daniel

    2013-04-01

    Ewing sarcoma is the second most common pediatric malignant bone neoplasm after osteosarcoma. Ewing sarcoma comprises "small, round, blue-cell" tumors thought to arise from neural crest cells. The authors report the case of a 14-year-old boy that presented with a nonpainful circumscribed lesion. The radiographs showed a lytic lesion at the tibial epiphysis with a large soft tissue mass, best depicted in the magnetic resonance imaging scan that suggested an aggressive lesion. A needle biopsy of the lesion was performed. The diagnosis of Ewing sarcoma was made based on microscopic, immunohistochemical, polymerase chain reaction, and fluorescence in situ hybridization. This is the third case report about a primary epiphyseal Ewing sarcoma and the fist one with molecular confirmation.

  9. Bilateral Second Carpal Row Duplication Associated with Multiple Epiphyseal Dysplasia

    PubMed Central

    Cladiere-Nassif, Victoire; Delaroche, Caroline; Pottier, Edwige; Feron, Jean-Marc

    2015-01-01

    We report a case of a 75-year-old woman presenting a hitherto undescribed condition of bilateral second carpal row duplication. She was diagnosed in childhood with both Marfan and Ehlers-Danlos syndromes, with no clear evidence and no further medical follow-up. She presented throughout her life with various articular symptoms, which appeared to be compatible with a diagnosis of multiple epiphyseal dysplasia, and underwent several surgical procedures on her knees and hips. Most recently, she was reporting pain at the base of the fifth metacarpal bone of the left hand. X-ray images and computed tomography (CT) were obtained for exploration and showed a total second row duplication in both carpi, with a total number of 18 carpal bones in each wrist. PMID:26649258

  10. Morphology and physiology of the epiphyseal growth plate.

    PubMed

    Burdan, Franciszek; Szumiło, Justyna; Korobowicz, Agnieszka; Farooquee, Rabia; Patel, Sagar; Patel, Ankit; Dave, Anjalee; Szumiło, Michał; Solecki, Michał; Klepacz, Robert; Dudka, Jarosław

    2009-01-01

    The epiphyseal growth plate develops from the cartilaginous-orientated mesenchymal cells that express SOX family genes. This multilayer structure is formed by the proliferation and hypertrophy of cells that synthesize the extracellular matrix composed of collagen (mainly type II, IX, X, XI) and proteoglycans (aggrecan, decorin, annexin II, V and VI). The resting zone is responsible for protein synthesis and maintaining a germinal structure. In the proliferative zone, cells rapidly duplicate. The subsequent morphological changes take place in the transformation zone, divided into the upper and lower hypertrophic layers. In the degenerative zone, the mineralization process becomes intensive due to increased release of alkaline phosphate, calcium and matrix vesicles by terminally differentiated chondrocytes and some other factors e.g., metaphyseal ingrowth vessels. At this level, as well as in the primary and secondary spongiosa zones, chondrocytes undergo apoptosis and are physiologically eliminated. Unlike adult cartilage, in fetal and early formed growth plates, unusual forms such as authophagal bodies, paralysis and dark chondrocytes were also observed. Their ultrastructure differs greatly from apoptotic and normal cartilage cells. Chondrocyte proliferation and differentiation are regulated by various endocrine, paracrine, and autocrine agents such as growth, thyroid and sex hormones, beta-catenin, bone morphogenetic proteins, insulin-like growth factor, iodothyronine deiodinase, leptin, nitric oxide, transforming growth factor beta and vitamin D metabolites. However, the most significant factor is parathyroid hormone-related protein (PTHrP) which is synthesized in the perichondrium by terminally differentiated chondrocytes. Secondary to activation of PTH/PTHrP receptors, PTHrP stimulates cell proliferation by G protein activation and delays their transformation into prehypertrophic and hypertrophic chondrocytes. When proliferation is completed, chondrocytes

  11. Structural differences in epiphyseal and physeal hypertrophic chondrocytes

    PubMed Central

    Shapiro, Frederic; Flynn, Evelyn

    2015-01-01

    We have observed that epiphyseal and physeal hypertrophic chondrocytes in BALB/c mice show considerable differences of light microscopic and ultrastructural appearance, even when the cells are at the same stage of differentiation. In addition, cell structure maintenance improved with tissue preparation controlled for osmolarity and for membrane stabilization using 0.5% ruthenium hexammine trichloride (RHT) for both light microscopy (LM) and electron microscopy (EM) or 0.5% lanthanum nitrate for LM. Physeal hypertrophic chondrocytes showed a gradual increase in size closer to the metaphysis and a change in shape as cells elongated along the long axis. The nucleus remained central, with uniformly dispersed chromatin, and the rough endoplasmic reticulum (RER) was randomly dispersed throughout cytoplasm with little to no presence against the cell membrane. Even the lowermost cells showed thin elongated or dilated cisternae of RER and intact cell membranes. Epiphyseal chondrocytes remained circular to oval with no elongation. Nucleus and RER were positioned as a complete transcellular central nucleocytoplasmic column or as an incomplete bud with RER of the column/bud always continuous with RER peripherally against the intact cell membrane. RER was densely packed with parallel cisternae with adjacent cytoplasm empty of organelles but often filled with circular deposits of moderately electron-dense material consistent with fat. Optimal technique for LM involved fixation using glutaraldehyde (GA) 1.3%, paraformaldehyde (PFA) 1% and RHT 0.5% (mOsm 606) embedded in JB-4 plastic and stained with 0.5% toluidine blue. Optimal technique for EM used fixation with GA 1.3%, PFA 1%, RHT 0.5% and cacodylate buffer 0.03 M (mOsm 511) and post-fixation including 1% osmium tetroxide. These observations lead to the possibility that the same basic cell, the hypertrophic chondrocyte, has differing functional mechanisms at different regions of the developing bone. PMID:25987982

  12. Automated Classification of Epiphyses in the Distal Radius and Ulna using a Support Vector Machine.

    PubMed

    Wang, Ya-hui; Liu, Tai-ang; Wei, Hua; Wan, Lei; Ying, Chong-liang; Zhu, Guang-you

    2016-03-01

    The aim of this study was to automatically classify epiphyses in the distal radius and ulna using a support vector machine (SVM) and to examine the accuracy of the epiphyseal growth grades generated by the support vector machine. X-ray images of distal radii and ulnae were collected from 140 Chinese teenagers aged between 11.0 and 19.0 years. Epiphyseal growth of the two elements was classified into five grades. Features of each element were extracted using a histogram of oriented gradient (HOG), and models were established using support vector classification (SVC). The prediction results and the validity of the models were evaluated with a cross-validation test and independent test for accuracy (PA ). Our findings suggest that this new technique for epiphyseal classification was successful and that an automated technique using an SVM is reliable and feasible, with a relative high accuracy for the models.

  13. Ontogenetic changes in the epiphyseal cartilage of Rana (Pelophylax) caralitana (Anura: Ranidae).

    PubMed

    Erismis, Ugur Cengiz; Chinsamy, Anusuya

    2010-11-01

    We document histological changes through ontogeny in the epiphyseal cartilage of the third phalanx of Rana caralitana from Turkey and provide an assessment of the maturation of the epiphysis from newly metamorphosed froglets to 10-year-old individuals. The epiphysis of R. caralitana is compared to other Rana taxa previously studied, and we report on novel histological data pertaining to later stages of epiphyseal growth in this taxon. In addition, we document the development of endochondral ossification in late stages of ontogeny in R. caralitana. Our results suggest a correlation between the long lifespan of R. caralitana and the developmental changes and maturation of the epiphyseal cartilage in this taxon. This study also provides a quantitative assessment of the different regions of the epiphyseal cartilage in the epiphysis of Rana through ontogeny, and has therefore permitted quantifiable deductions about the relative maturation and differentiation of the chondrocytes of the epiphysis through time.

  14. Slipped femoral capital epiphyses in irradiated children: dose, volume and age relationships

    SciTech Connect

    Silverman, C.L.; Thomas, P.R.M.; McAlister, W.H.; Walker, S.; Whiteside, L.A.

    1981-10-01

    Between 1960 and 1979, 50 patients under 15 years of age received radiotherapy to the pelvis including the non-fused capital femoral epiphyseal plate. A total of 83 epiphyseal plates were at risk. Eight epiphyseal plates (9.6%) in five patients were abnormal: symptomatic capital femoral epiphyseal slippage--4, asymptomatic slippage--1, severe epiphyseal abnormalities of radiographs--3. No complication occurred below doses of 2,500 rad (25 Gy). Children under the age of 4 at time of irradiation were at a higher risk (7/15-47%) than those over 4 years of age (1/21-4.7%). Most slippages occurred at ages between 8 and 10 years. No dose response curve was obtained--higher doses above the threshold dose of 2,500 rad did not increase the incidence of slippage. A mechanism of action is postulated. This is a preventable complication; judicious use of primary or secondary blocking systems can eliminate or limit the dose to the non-fused epiphyseal plate and prevent later morbid complications.

  15. Histology of epiphyseal cartilage calcification and endochondral ossification.

    PubMed

    Amizuka, Norio; Hasegawa, Tomoka; Oda, Kimimitsu; Luiz de Freitas, Paulo Henrique; Hoshi, Kazuto; Li, Minqi; Ozawa, Hidehiro

    2012-01-01

    Cartilage calcification is carried out by chondrocytes as they hypertrophy and begin to secrete matrix vesicles. Calcification initiates when calcium phosphates appear inside these matrix vesicles, forming hydroxyapatite crystals that eventually break through the membrane to form calcifying globules, as in bone calcification. However, the extracellular environment in cartilage is different from that in bone: cartilage is abundant in proteoglycans but contains a small amount of osteopontin. Hypertrophic chondrocytes secrete vesicles in the cartilaginous matrix of intercolumnar septae only, forming well-calcified longitudinal septae and poorly-calcified transverse partitions. Such pattern of vesicle deposition permits the invasion of endothelial cells, which infiltrate into cartilage and induce migration of osteogenic and osteoclastic cells. Osteoclasts resorb the excess of calcified globules in the partitions, shaping calcified cartilage cores paralleling the longitudinal axis of long bones. After the formation of these calcified cartilage cores, endochondral ossification involves a series of well-defined events in which osteogenic cells deposit new bone onto the cartilage core and form primary trabecules. This review presents the histology of epiphyseal cartilage calcification and endochondral ossification.

  16. Isolation and Characterization of Calcifying Matrix Vesicles from Epiphyseal Cartilage*

    PubMed Central

    Ali, S. Y.; Sajdera, S. W.; Anderson, H. C.

    1970-01-01

    Matrix vesicles, associated with initial calcification in cartilage, have been isolated from bovine fetal epiphyseal cartilage. Cartilage was digested with collagenase, then partitioned into seven fractions by differential centrifugation. The cellular fractions contained over 80% of the DNA in the digest. The extracellular fraction that contained matrix vesicles, in which apatite crystals were often seen on electron microscopy, also displayed the highest specific activity for alkaline phosphatase, pyrophosphatase, ATPase, and 5′-AMPase (EC 3.1.3.1., 3.6.1.1, 3.6.1.3, and 3.1.3.5, respectively). Most of the acid phosphatase (EC 3.1.3.2) activity, on the other hand, was found in the cellular fractions, indicating that matrix vesicles are quite distinct from lysosomes. This appears to be the first instance of isolation of membrane-bounded extracellular particles from any normal tissue. The matrix vesicles possess enzymes that can increase the local concentration of orthophosphate and thus could lead to the formation of hydroxyapatite. The membrane-bounded matrix vesicles may also provide a mechanism for ATP-dependent transport of calcium or phosphate into the lumen of the vesicles with resultant mineralization. Images PMID:5274475

  17. Histological and biological changes in the epiphyseal plate during fracture healing.

    PubMed

    Papavasiliou, Athanasios V

    2002-01-01

    The alterations that the epiphyseal plate undergoes during fracture healing are well documented microscopically, yet there are no reports in the literature which discuss the cellular and molecular changes that accompany this process. We studied fracture healing in 49 Wistar rats (5 weeks old) in which we inflicted a fracture to the distal third of the femur of the right hind leg (experimental side). The rats were killed 2 weeks later, and we dissected both hind legs from the hip joint to the knee joint, detaching all the surrounding soft tissues. We manually detached the distal epiphyses and the epiphyseal plates from both femurs. A piece of the epiphyseal plate was removed from the epiphyseal side of the femurs. In 25 animals, and we analyzed the DNA content. In 8 animals, the specimen was studied under an electron microscope, and in the remaining 16 animals, the control and experimental sides were studied histologically. We found that healing was accompanied by an increase in DNA content, by a change in cellular activity, and by greatly accelerated apoptosis.

  18. Cerebral Epiphyseal Proteins and Melatonin Modulate the Hepatic and Renal Antioxidant Defense of Rats

    PubMed Central

    Bharti, Vijay K.; Srivastava, R. S.; Subramaian, P.; Warren Spence, D.; Pandi-Perumal, S. R.; Brown, Gregory M.

    2011-01-01

    The cerebral epiphysis (pineal gland) secrets melatonin and number of other proteins and peptides. It was thus hypothesized that antioxidant properties of epiphyseal proteins and melatonin could potentially benefit from exogenous therapies. In view of the therapeutic potential of these proteins, the present experiment was conducted to investigate the effect of buffalo epiphyseal proteins (BEP, at 100 μg/kg BW, i.p.) and melatonin (MEL, at 10 mg/kg BW, i.p) on changes in hepatic and renal antioxidant enzymes of adult female Wistar rats. Buffalo epiphyseal proteins significantly (P < .05) increased hepatic lipid peroxidation (LPO), superoxide dismutase (SOD), glutathione reductase (GR), glutathione peroxidase (GPx), reduced glutathione (GSH), and renal LPO, catalase (CAT), GR, GSH, GPx levels as compared to control animals. Similarly, MEL treatment significantly (P < .05) up-regulated hepatic SOD and GPx activity, whereas CAT, GR, GPx, and GSH levels in renal tissues were increased while SOD and LPO remained unaffected. Buffalo epiphyseal protein treatment produced greater effects on hepatic GPx and renal CAT and GSH levels than did MEL. These findings support the conclusion that buffalo epiphyseal proteins and melatonin activate a number of antioxidant mechanisms in hepatic and renal tissues. PMID:21660111

  19. Humeral epiphyseal shape in the felidae: the influence of phylogeny, allometry, and locomotion.

    PubMed

    Walmsley, Anthony; Elton, Sarah; Louys, Julien; Bishop, Laura C; Meloro, Carlo

    2012-12-01

    Bone morphology of the cats (Mammalia: Felidae) is influenced by many factors, including locomotor mode, body size, hunting methods, prey size and phylogeny. Here, we investigate the shape of the proximal and distal humeral epiphyses in extant species of the felids, based on two-dimensional landmark configurations. Geometric morphometric techniques were used to describe shape differences in the context of phylogeny, allometry and locomotion. The influence of these factors on epiphyseal shape was assessed using Principal Component Analysis, Linear Discriminant functions and multivariate regression. Phylogenetic Generalised Least Squares was used to examine the association between size or locomotion and humeral epiphyseal shape, after taking a phylogenetic error term into account. Results show marked differences in epiphyseal shape between felid lineages, with a relatively large phylogenetic influence. Additionally, the adaptive influences of size and locomotion are demonstrated, and their influence is independent of phylogeny in most, but not all, cases. Several features of epiphyseal shape are common to the largest terrestrial felids, including a relative reduction in the surface area of the humeral head and increased robusticity of structures that provide attachment for joint-stabilising muscles, including the medial epicondyle and the greater and lesser tubercles. This increased robusticity is a functional response to the increased loading forces placed on the joints due to large body mass.

  20. Cartilaginous epiphyses in extant archosaurs and their implications for reconstructing limb function in dinosaurs.

    PubMed

    Holliday, Casey M; Ridgely, Ryan C; Sedlmayr, Jayc C; Witmer, Lawrence M

    2010-09-30

    Extinct archosaurs, including many non-avian dinosaurs, exhibit relatively simply shaped condylar regions in their appendicular bones, suggesting potentially large amounts of unpreserved epiphyseal (articular) cartilage. This "lost anatomy" is often underappreciated such that the ends of bones are typically considered to be the joint surfaces, potentially having a major impact on functional interpretation. Extant alligators and birds were used to establish an objective basis for inferences about cartilaginous articular structures in such extinct archosaur clades as non-avian dinosaurs. Limb elements of alligators, ostriches, and other birds were dissected, disarticulated, and defleshed. Lengths and condylar shapes of elements with intact epiphyses were measured. Limbs were subsequently completely skeletonized and the measurements repeated. Removal of cartilaginous condylar regions resulted in statistically significant changes in element length and condylar breadth. Moreover, there was marked loss of those cartilaginous structures responsible for joint architecture and congruence. Compared to alligators, birds showed less dramatic, but still significant changes. Condylar morphologies of dinosaur limb bones suggest that most non-coelurosaurian clades possessed large cartilaginous epiphyses that relied on the maintenance of vascular channels that are otherwise eliminated early in ontogeny in smaller-bodied tetrapods. A sensitivity analysis using cartilage correction factors (CCFs) obtained from extant taxa indicates that whereas the presence of cartilaginous epiphyses only moderately increases estimates of dinosaur height and speed, it has important implications for our ability to infer joint morphology, posture, and the complicated functional movements in the limbs of many extinct archosaurs. Evidence suggests that the sizes of sauropod epiphyseal cartilages surpassed those of alligators, which account for at least 10% of hindlimb length. These data suggest that

  1. Cartilaginous Epiphyses in Extant Archosaurs and Their Implications for Reconstructing Limb Function in Dinosaurs

    PubMed Central

    Holliday, Casey M.; Ridgely, Ryan C.; Sedlmayr, Jayc C.; Witmer, Lawrence M.

    2010-01-01

    Extinct archosaurs, including many non-avian dinosaurs, exhibit relatively simply shaped condylar regions in their appendicular bones, suggesting potentially large amounts of unpreserved epiphyseal (articular) cartilage. This “lost anatomy” is often underappreciated such that the ends of bones are typically considered to be the joint surfaces, potentially having a major impact on functional interpretation. Extant alligators and birds were used to establish an objective basis for inferences about cartilaginous articular structures in such extinct archosaur clades as non-avian dinosaurs. Limb elements of alligators, ostriches, and other birds were dissected, disarticulated, and defleshed. Lengths and condylar shapes of elements with intact epiphyses were measured. Limbs were subsequently completely skeletonized and the measurements repeated. Removal of cartilaginous condylar regions resulted in statistically significant changes in element length and condylar breadth. Moreover, there was marked loss of those cartilaginous structures responsible for joint architecture and congruence. Compared to alligators, birds showed less dramatic, but still significant changes. Condylar morphologies of dinosaur limb bones suggest that most non-coelurosaurian clades possessed large cartilaginous epiphyses that relied on the maintenance of vascular channels that are otherwise eliminated early in ontogeny in smaller-bodied tetrapods. A sensitivity analysis using cartilage correction factors (CCFs) obtained from extant taxa indicates that whereas the presence of cartilaginous epiphyses only moderately increases estimates of dinosaur height and speed, it has important implications for our ability to infer joint morphology, posture, and the complicated functional movements in the limbs of many extinct archosaurs. Evidence suggests that the sizes of sauropod epiphyseal cartilages surpassed those of alligators, which account for at least 10% of hindlimb length. These data suggest that

  2. [Growth prognosis after para-epiphyseal tangential epiphysial injuries of the lower extremity exemplified by the distal femur: outcome after injuries of epiphyses of the distal femur].

    PubMed

    Weinberg, A M; Castellani, C; Werner, C; Mayr, J

    2002-01-01

    Fractures of the distal end of the femur are rare. Premature partial closure of the physis may occur after metaphyseal fractures. After epiphyseal fractures it has a high incidence of occurrence. The cause of physeal arrest is not known. Stimulation of the physes ends in leg length discrepancy and was seen in 50% of all cases. Spontaneous correction of an axis deviation is possible after antecurvation up to an age of 5 years. But this should be only accepted during therapy of metaphyseal fractures of the distal femur. Side-to-Side deviations remodel in all cases. Aim of therapy in distal epiphyseal fractures should be an anatomical reduction and a definitive retention. Screw osteosynthesis seems to have an advantage.

  3. Gadolinium-enhanced MR imaging of epiphyseal and metaphyseal marrow in normal piglets.

    PubMed

    Li, Xiaoming; Wang, Renfa; Qi, Jianpin; Tang, Lihua

    2005-01-01

    The purpose of this study is to define the appearance of normal epiphyseal and metaphyseal marrow and normal changes of marrow due to fatty conversion on Gadolinium (Gd)-enhanced MR Imaging. Unenhanced and enhanced T1-weighted MR imaging were performed in proximal and distal femoral ends of 8 healthy piglets at the ages of 2, 4, 6 and 8 weeks, respectively. The changes with age in signal intensity and enhancement ratio of the epiphyseal and metaphyseal marrow with age were examined. The correlation of MRI characteristics with histological findings was studied. Our study showed that marrow of the metaphysis and of periphery of the 2nd ossification center were well vascularized hematopoietic marrow and had great enhancements. The enhancement ratio of metaphysis was greater than that of epiphyseal marrow and both enhancement ratios degraded gradually with age. The central regions of the epiphyseal ossification center and of the diaphysis were of fatty marrow and had little enhancement. It is concluded that on Gd-enhanced MR imaging the hematopoietic marrow of metaphysis and of periphery of the 2nd ossification center had greater enhancement than that of fatty marrow of central region of the 2nd ossification center. All of their enhancements decreased gradually with age.

  4. [Zinc deficiency on pathological changes of femur epiphyseal growth plate in rats].

    PubMed

    Zhang, Yuehong; Cheng, Yiyong; Hong, Yan; Li, Shutian

    2003-01-01

    To study zinc deficiency on pathological changes of femur epiphyseal growth plate in rats thirty Wistar rats were randomly divided into three groups: zinc-deficient group (Zd), control group (Ctr), and pair-fed group (Zp) to study the effects of zinc deficiency on femur epiphyseal growth plate of rats and the mechanism involved. After 8 weeks feeding, the histomorphology of right femur indicated that chondrocytes in the epiphyseal growth plate of Zd group were generally ill-organized and mis-shaped. The number of chondrocytes decreased. Trabecular bone in the epiphyseal of Zd group were also ill-organized, scarce and slim. The cavities of marrow in Zd animals were significantly larger than those of Ctr and Zp. The volume of trabecular bone, the mean trabecular plate density of Zd rats were significantly decreased, but their mean trabecular plate space was significantly increased. In addition, the Zd animals had significantly lower concentration of osteocalcin and growth hormone in sera. The above results suggested that zinc deficiency impaired proliferation and differentiation of chondrocytes and balance between osteoblast and osteoclast function by reduce growth hormone levels in sera. The lack of zinc in diet resulted in disorder of bone molding and ultrastructure.

  5. Growth arrest lines and intra-epiphyseal silhouettes: a case series

    PubMed Central

    2014-01-01

    Background Growth arrest lines can develop within the skeleton after physiological stress or trauma. They are usually evident on radiographs as transverse lines in the metaphyses and have been used in fields from palaeontology to orthopaedics. This report consists of three cases, two of which describe growth arrest lines in an intra-epiphyseal site hitherto rarely documented, and a third demonstrating their clinical application. Case presentation Case 1 describes a 9-year-old who suffered a knee hyperflexion injury requiring anterior cruciate ligament and posterior cruciate ligament reattachments. She subsequently developed a marked distal femoral intra-epiphyseal arrest silhouette, as well as metaphyseal arrest lines in the femur, tibia and fibula. Case 2 describes an 8-year-old who sustained a tibial spine fracture and underwent open reduction and internal fixation. Subsequent imaging shows a further example of femoral intra-epiphyseal arrest silhouette as well as tibia and fibula metaphyseal arrest lines. Case 3 describes a 10-year-old who sustained a distal tibia fracture which was managed with open reduction and internal fixation. Subsequently the metaphyseal growth arrest line was parallel to the physis, suggesting no growth arrest (a danger with such a fracture). Conclusion This case series describes two examples of rarely described intra-epiphyseal growth arrest silhouettes and demonstrates the usefulness of arrest lines when assessing for growth plate damage. PMID:24410952

  6. Abnormality of epiphyseal plate induced by selenium deficiency diet in two generation DA rats.

    PubMed

    Min, Zixin; Zhao, Wenxiang; Zhong, Nannan; Guo, Yuanxu; Sun, Mengyao; Wang, Quancheng; Zhang, Rui; Yan, Jidong; Tian, Lifang; Zhang, Fujun; Han, Yan; Ning, Qilan; Meng, Liesu; Sun, Jian; Lu, Shemin

    2015-08-01

    This study aimed to observe the effects of Se deficiency on epiphyseal plates of two generation DA rats fed with artificial total synthetic low Se diet. All F0 and F1 DA rats were fed with synthetic low Se diet (SeD group) and low Se diet supplied with Se (SeS group). The levels of selenium and enzyme activities of GPx were detected in plasma of the rats. General growth of bone and articular cartilage was measured macroscopically and microscopically. The epiphyseal plate of femur heads or tibia were obtained to histological and immunohistochemical examinations. The cartilage from left knee joints and femur heads was used to detect the gene expression of collagens, ADAMTSs and several selenoproteins by RT-qPCR. Two generation SeD rats showed Se insufficiency status. The thicknesses of the femur and tibial epiphyseal plates in both F0 and F1 SeD rats were significantly less than that of SeS rats. In F1 generation, SeD rats showed much fewer proliferative chondrocyte layers than SeS ones. Importantly, two generation SeD rats both showed significantly more serious pathological changes of epiphyseal plates. In two generation rats, gene expressions of COL II, GPx1 and GPx4 were significantly down-regulated in SeD rats than SeS ones; meanwhile ADAMTS-4 showed an up-regulated expression in cartilage. Dietary Se deficiency can apparently cause epiphyseal plate lesion and decrease cartilage type II collagen production and GPx1 activity in two generation DA rats fed with the artificial total synthesis low Se diet.

  7. [Morphometric measurements in the area of the epiphyseal groove of the parietal vertebra in experimentally-induced scoliosis in rabbits].

    PubMed

    Kneer, W; Mitzkat, K

    1986-01-01

    In 3 rabbits we provoked a short-curved dorsolumbal scoliosis by costo-lumbal cerclage. In order to quantify with morphometric methods the morphologically by various authors described effect of pressure parallel to the axis of epiphyseal growth under Hueter-Volkmann's law, we measured the height and area of different parts of the epiphyseal plate in frontal semi-thin sections (Alcian-blue) by means of a pictoral analysis system based on a mini-computer. On the concave side, the side of increased pressure, we found a relative atrophy of the epiphyseal plate of 40% and a diminished area of 49%.

  8. Intra-epiphyseal stress injury of the proximal tibial epiphysis: preliminary experience of magnetic resonance imaging findings.

    PubMed

    Tony, G; Charran, A; Tins, B; Lalam, R; Tyrrell, P N M; Singh, J; Cool, P; Kiely, N; Cassar-Pullicino, V N

    2014-11-01

    Stress induced injuries affecting the physeal plate or cortical bone in children and adolescents, especially young athletes, have been well described. However, there are no reports in the current English language literature of stress injury affecting the incompletely ossified epiphyseal cartilage. We present four cases of stress related change to the proximal tibial epiphysis (PTE) along with their respective magnetic resonance imaging (MRI) appearances ranging from subtle oedema signal to a pseudo-tumour like appearance within the epiphyseal cartilage. The site and pattern of intra-epiphyseal injury is determined by the type of tissue that is affected, the maturity of the skeleton and the type of forces that are transmitted through the tissue. We demonstrate how an awareness of the morphological spectrum of MRI appearances in intra-epiphyseal stress injury and the ability to identify concomitant signs of stress in other nearby structures can help reduce misdiagnosis, avoid invasive diagnostic procedures like bone biopsy and reassure patients and their families.

  9. Changes in rat epiphyseal cartilage after treatment with dexamethasone and glycosaminoglycan-peptide complex.

    PubMed

    Annefeld, M

    1992-06-01

    In 3-month-old rats, systemic administration of glucocorticosteroids induced regressive changes in the epiphyseal plate cartilage. Dexamethasone treatment resulted in inhibition of both chondrocyte proliferation and cartilage matrix production. The inhibition of chondrocyte proliferation was determined histologically from the thickness of the epiphyseal plate, the number of cell columns and the ratio of proliferating to hypertrophied chondrocytes. The decrease in cartilage matrix production was measured autoradiographically by incorporation of radioactive 35S-sulphate. Concomitant treatment with glycosaminoglycan-peptide complex (GP-C = RUMALON) overcame the negative effect of dexamethasone. The values in the dexamethasone + GP-C group approached those of the control group and differed significantly from the group given dexamethasone alone.

  10. Sudden death in spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type.

    PubMed

    Dias, Cristina; Cairns, Robyn; Patel, Millan S

    2009-01-01

    The spondylo-meta-epiphyseal dysplasias are an expanding group of skeletal dysplasias with specific features differentiating each subtype. We review the precocious carpal mineralization, unique metacarpal shape, triangular distal phalanges and mushroom cloud-shaped proximal phalanges present at an early age in spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type (SMED SL-AC) and report two patients with clinical and radiographic features consistent with SMED SL-AC, who died suddenly because of spinal cord compression. The patients presented are female siblings, providing further evidence for autosomal recessive inheritance. Cervical cord compression is found in half of reported patients and is the major cause of mortality. SMED SL-AC should be added to the list of genetic causes of sudden death. Radiological features in the hand may be used in the first few years of life to support an early diagnosis and thus allow for prevention of premature demise.

  11. The free vascularized fibular epiphyseal transfer: long-term results of wrist reconstruction in young patients.

    PubMed

    Papadopulos, Nikolaos A; Weigand, Christian; Kovacs, Laszlo; Biemer, Edgar

    2009-01-01

    To date, the dominant blood supply to the head of the fibula and to the growth plate is known to be the anterior tibial artery. The peroneal artery had been used before, among other donor pedicles, for microvascular transfers of this epiphyseal region. This study presents the long-term results of this now obsolete pedicle and compares them to other reports in the literature. Follow-up was performed in 1996 and in 2003 with six patients who underwent wrist reconstruction in the 1980s. Procedures were performed following one resection of a malignant synovialoma, two traumatic hand amputations, and three radial aplasias. Evaluation was performed with functional and radiographic examinations. Three cases that were examined in 2003 are presented in detail. The study shows that if growth plates are closed at the time of procedure or the transplanted fibula is long enough to ensure anastomotic flow between metaphyseal and epiphyseal vessels, results are good. If any of these two conditions is not fulfilled, vascular supply to the epiphysis is insufficient. Long bone deviation or bone necrosis will result. These results confirm clinically current knowledge about the epiphyseal and metaphyseal blood supply to the fibula.

  12. All-Epiphyseal, All-Inside Anterior Cruciate Ligament Reconstruction Technique for Skeletally Immature Patients

    PubMed Central

    McCarthy, Moira M.; Graziano, Jessica; Green, Daniel W.; Cordasco, Frank A.

    2012-01-01

    Anterior cruciate ligament (ACL) injuries are an increasingly recognized problem in the juvenile population. Unfortunately, outcomes with conservative treatment are extremely poor. Adult reconstruction techniques are inappropriate to treat skeletally immature patients because of the risk of physeal complications, including limb-length discrepancy and angular deformities. “Physeal-sparing” reconstruction techniques exist, but their ability to restore knee stability is not well understood. We describe an all-epiphyseal ACL reconstruction for use in skeletally immature patients. This is an all-inside technique with the femoral tunnel drilled retrograde and the tibial tunnel drilled retrograde; both tunnels are entirely within the epiphysis. Fixation of the hamstring autograft is achieved with soft-tissue buttons on both the femur and tibia. We present case examples for 2 patients who underwent the all-inside, all-epiphyseal reconstruction and our postoperative rehabilitation protocol. We present a novel surgical technique for an all-inside, all-epiphyseal ACL reconstruction in skeletally immature patients. PMID:23767001

  13. A Study of Epiphyses in the Young Prepubescent Knee Using Magnetic Resonance Imaging

    PubMed Central

    Davis, Derik L.; Chen, Lina; Ehinger, Melanie

    2014-01-01

    Background: Questions have been raised concerning the safety of intra-articular anterior cruciate ligament (ACL) reconstruction in prepubescent children aged <7 years. However, normal values for the width of the lateral femoral condylar epiphysis and height of the tibial epiphysis have yet to be established through the use of magnetic resonance imaging (MRI). Purpose: To determine normal values for the width of the lateral femoral condylar epiphysis and height of the tibial epiphysis at the knee in prepubescent children aged <7 years by use of MRI and to compare this age group with an older cohort of prepubescent children aged <10 years. Study Design: Cross-sectional study; Level of evidence, 3. Methods: An electronic search was conducted for pediatric knee MRI examinations at the authors’ institution from March 2003 to March 2013. The total and ossified lateral femoral condylar widths were determined on coronal proton density–weighted images. The total and ossified tibial epiphyseal heights were recorded on the sagittal T1-weighted image best containing the ACL footplate. The intraclass correlation coefficient (ICC) was calculated to determine interobserver agreement. Knees were stratified by age into 2 groups: children between the ages of 3 and 6 years (group 1) and children between the ages of 7 and 9 years (group 2). Each cohort was further stratified by sex. Results: Group 1 consisted of 10 children (mean age, 4.3 years) and group 2 consisted of 10 children (mean age, 8.5 years). There were a total of 20 knees. There was a statistically significant difference between groups 1 and 2 for the ossified lateral femoral condylar width where femoral tunnel location would be expected (20.00 ± 4.20 vs 26.27 ± 4.12 mm, respectively; P = .0035) and for total lateral femoral condylar width (25.57 ± 3.47 vs 29.43 ± 4.04 mm, respectively; P = .0339). No difference was found for total tibial epiphyseal height between the 2 groups. However, there was a difference

  14. Delayed hypertrophic differentiation of epiphyseal chondrocytes contributes to failed secondary ossification in mucopolysaccharidosis VII dogs

    PubMed Central

    Peck, Sun H.; O'Donnell, Philip J.M.; Kang, Jennifer L.; Malhotra, Neil R.; Dodge, George R.; Pacifici, Maurizio; Shore, Eileen M.; Haskins, Mark E.; Smith, Lachlan J.

    2015-01-01

    Mucopolysaccharidosis (MPS) VII is a lysosomal storage disorder characterized by deficient β-glucuronidase activity, which leads to the accumulation of incompletely degraded glycosaminoglycans (GAGs). MPS VII patients present with severe skeletal abnormalities, which are particularly prevalent in the spine. Incomplete cartilage-to-bone conversion in MPS VII vertebrae during postnatal development is associated with progressive spinal deformity and spinal cord compression. The objectives of this study were to determine the earliest postnatal developmental stage at which vertebral bone disease manifests in MPS VII and to identify the underlying cellular basis of impaired cartilage-to-bone conversion, using the naturally-occurring canine model. Control and MPS VII dogs were euthanized at 9 and 14 days-of-age, and vertebral secondary ossification centers analyzed using micro-computed tomography, histology, qPCR, and protein immunoblotting. Imaging studies and mRNA analysis of bone formation markers established that secondary ossification commences between 9 and 14 days in control animals, but not in MPS VII animals. mRNA analysis of differentiation markers revealed that MPS VII epiphyseal chondrocytes are unable to successfully transition from proliferation to hypertrophy during this critical developmental window. Immunoblotting demonstrated abnormal persistence of Sox9 protein in MPS VII cells between 9 and 14 days-of-age, and biochemical assays revealed abnormally high intra and extracellular GAG content in MPS VII epiphyseal cartilage at as early as 9 days-of-age. In contrast, assessment of vertebral growth plates and primary ossification centers revealed no significant abnormalities at either age. The results of this study establish that failed vertebral bone formation in MPS VII can be traced to the failure of epiphyseal chondrocytes to undergo hypertrophic differentiation at the appropriate developmental stage, and suggest that aberrant processing of Sox9 protein

  15. Delayed hypertrophic differentiation of epiphyseal chondrocytes contributes to failed secondary ossification in mucopolysaccharidosis VII dogs.

    PubMed

    Peck, Sun H; O'Donnell, Philip J M; Kang, Jennifer L; Malhotra, Neil R; Dodge, George R; Pacifici, Maurizio; Shore, Eileen M; Haskins, Mark E; Smith, Lachlan J

    2015-11-01

    Mucopolysaccharidosis (MPS) VII is a lysosomal storage disorder characterized by deficient β-glucuronidase activity, which leads to the accumulation of incompletely degraded glycosaminoglycans (GAGs). MPS VII patients present with severe skeletal abnormalities, which are particularly prevalent in the spine. Incomplete cartilage-to-bone conversion in MPS VII vertebrae during postnatal development is associated with progressive spinal deformity and spinal cord compression. The objectives of this study were to determine the earliest postnatal developmental stage at which vertebral bone disease manifests in MPS VII and to identify the underlying cellular basis of impaired cartilage-to-bone conversion, using the naturally-occurring canine model. Control and MPS VII dogs were euthanized at 9 and 14 days-of-age, and vertebral secondary ossification centers analyzed using micro-computed tomography, histology, qPCR, and protein immunoblotting. Imaging studies and mRNA analysis of bone formation markers established that secondary ossification commences between 9 and 14 days in control animals, but not in MPS VII animals. mRNA analysis of differentiation markers revealed that MPS VII epiphyseal chondrocytes are unable to successfully transition from proliferation to hypertrophy during this critical developmental window. Immunoblotting demonstrated abnormal persistence of Sox9 protein in MPS VII cells between 9 and 14 days-of-age, and biochemical assays revealed abnormally high intra and extracellular GAG content in MPS VII epiphyseal cartilage at as early as 9 days-of-age. In contrast, assessment of vertebral growth plates and primary ossification centers revealed no significant abnormalities at either age. The results of this study establish that failed vertebral bone formation in MPS VII can be traced to the failure of epiphyseal chondrocytes to undergo hypertrophic differentiation at the appropriate developmental stage, and suggest that aberrant processing of Sox9 protein

  16. Angel-shaped phalango-epiphyseal dysplasia (ASPED): identification of a new genetic bone marker.

    PubMed

    Giedion, A; Prader, A; Fliegel, C; Krasikov, N; Langer, L; Poznanski, A

    1993-10-01

    We describe a "new" mild malformation of the phalanx, which we call the "angel-shaped phalanx" (ASP) because of its resemblance to the little angels used for the decoration of Christmas trees. A particular middle phalangeal type of ASPs is found in a distinct variety of multiple epiphyseal dysplasia with marked retardation of bone age and severe coxarthrosis in adult life, previously reported as "hereditary peripheral dysostosis" [Bachman, 1967: Proc R Soc Med 60:21-22; Giedion, 1969: Fortschr Rontgenstr 110:507-524]. However, these authors overlooked the unique configuration of the middle phalanges. We renamed the condition "angel-shaped phalango-epiphyseal dysplasia (ASPED)", which may be transmitted in an autosomal-dominant manner. Six new patients are added, bringing the total to nine patients (two families and two isolated patients). ASPs were seen in five of six children. The ASPs grew into inconspicuous brachydactyly after physeal closure (3/3). The most important additional radiological finding is late and dysplastic development of both femoral heads (5/5), leading to Perthes-like and osteoarthritic changes and severe hip pain in the early thirties (2/2 adults, having reached this age). The marked retardation of carpal bone age may lead to unnecessary clinical evaluation for endocrine disorders. Less frequent clinical manifestations of ASPED are hyperextensibility of the interphalangeal joints (7/9) and hypodontia (4/7). Other types of ASPs are observed in brachyphalangy type C, spondylo-megepiphyseal-metaphyseal dysplasia, and other conditions. The concept of mild bone abnormalities as specific markers for genetic disease, as with cone-shaped epiphyses and now evident in ASPED, may also be useful for ASPs in general.

  17. Influence of doxycycline on the epiphyseal plate cartilage of the rats in experimental osteoarthrosis, induced by iodoacetate.

    PubMed

    Cylwik, J; Kita, K; Barwijuk-Machała, M; Reszeć, J; Klimiuk, P; Sierakowski, S; Sulkowski, S; Cylwik, M

    2004-01-01

    In 36 Wistar rats with the iodoacetate-induced experimental osteoarthrosis (OA), effects of doxycycline, given orally, were determined on histochemical reactions of glycosaminoglycans (GAG) in the epiphyseal plate cartilage. The epiphyseal plate of rats with OA was reduced in height (especially the proliferative zone), cell columns were disorganized, many chondrocytes were irregular and polygonal, their nuclei were pycnotic, the intensity of GAG staining was irregular and predominantly reduced, which can be interpreted as signs of degeneration. A concomitant administration of doxycycline in the second group of rats prevented, to some extent, the negative effects of iodoacetate on chondrocytes and led to a more pronounced intensity of GAG reactions in the matrix of the epiphyseal plate.

  18. Age of fusion of the distal radial and ulnar epiphyses from hand radiographs-A study in Kashmiri population.

    PubMed

    Hassan, Nida; Noor, Farida; Ahmad, Shabir; Fazili, Khalid Majid

    2016-12-01

    Age estimation is a crucial parameter involved in investigations pertaining to civil and criminal procedures. It also aids in various examinations in forensic medicine, pediatrics, endocrinology and radiology. One of the important methods for skeletal age estimation is the age of fusion of the epiphyses. But there occur variations in the skeletal ages due to environmental, hormonal, ethnic and other factors. Hence, there arises the need for separate standards of ossification for different regions. The present study was conducted to ascertain the age of fusion of the distal radial and ulnar epiphyses in Kashmiri population. A total of 160 healthy subjects, including 80 males and 80 females with ages ranging from 12 to 20years were studied. Their chronological age was obtained and the X-ray of their left hand was taken in the A.P view after taking the consent from their parents. The starting of epiphyseal fusion in lower end of radius in male was observed at 15-16years in 20% of the male population and for females, it was observed at 13-14years in 10% of the female population. The completion of epiphyseal fusion in lower end of radius in 100% males was noticed at 18-19years and for 100% females, it was noticed at 17-18years. The starting up of epiphyseal fusion in lower end of ulna in males was observed at 14-15years in 10% of the male population and for females, it was observed at 13-14years in 10% of the female population. The completion of epiphyseal fusion in lower end of ulna in 100% males was noticed at 18-19years and for 100% females, it was noticed at 17-18years. In case of males the age of fusion of the epiphyses of lower end of radius and lower end of ulna was found to be in the same age group 18 to 19years. Also in case of females the age of fusion of the epiphyses of lower end of radius and lower end of ulna was found to be in the same age group 17 to 18years. It was further observed that females showed fusion in advance of male subjects. The findings of

  19. Remodeling of Salter-Harris type II epiphyseal plate injury of the distal radius.

    PubMed

    Houshian, Shirzad; Holst, Anette Koch; Larsen, Morten S; Torfing, Trine

    2004-01-01

    The authors studied the relation between residual angulation at the time of healing and final orientation of the distal radius as well as the clinical outcome in patients after Salter-Harris type II epiphyseal plate injury of the distal radius. Eighty-five patients were reviewed with a median follow-up of 8.5 years. Anteroposterior and lateral radiographs were taken at follow-up. The mobility of both wrists and forearms was examined, together with grip strength. Pain with activities and sports was evaluated. At follow-up, 73 patients (86%) were anatomically normal on radiographs; the remaining 12 patients had an incomplete remodeling of the volar and/or radial inclination of the distal radius. Premature closure of the growth plate in the distal radius or ulna did not occur in any of these children. Complete remodeling was seen in children aged up to 10 years in all but one patient. Remodeling after Salter-Harris type II epiphyseal plate injury occurs in all age groups, but the potential is greater in children up to 10 years of age. The incomplete remodeling does not seem to have any substantial long-term negative effect on mobility of the wrist and grip strength.

  20. Use of microarray analysis to study gene expression in the avian epiphyseal growth plate.

    PubMed

    Horvat-Gordon, M; Praul, C A; Ramachandran, R; Bartell, P A; Leach, R M

    2010-03-01

    Longitudinal bone growth depends upon the execution of an intricate series of cellular activities by epiphyseal growth plate chondrocytes. In order to better understand these coordinated events, microarray analysis was used to compare gene expression in chondrocytes isolated from the proliferative and hypertrophic zones of the avian growth plate. RT-PCR was used to confirm the identity of a select number of genes. The expression of 745 genes was found to differ 3-fold or greater at the 0.05 level of probability. Transferrin was the most highly up-regulated (321-fold) gene associated with chondrocyte hypertrophy. Immunohistochemistry localized this peptide adjacent to the penetrating blood vessels in the growth plate of 3-week-old chicks. Fibulin, OC-116, DMP-1 and PHEX were among the expanded number of genes associated with extracellular matrix metabolism. The presence of NELL2, ATOH8 and PLEXIN suggests a neuronal involvement in growth plate physiology. In addition, the expression of a large number of genes associated with angiogenesis and cellular stress was up-regulated. These processes are important to the physiology and survival of chondrocytes in the unique and stressful environment of the epiphyseal growth plate.

  1. A rapid, valid and inexpensive assay for measuring epiphyseal plates in mouse tibia.

    PubMed

    Interlichia, Jillian P; Williams, Nolann G; Rodgers, Buel D

    2010-04-01

    One of the most accurate indices of changes in somatic tissue growth rate in rodents is the width of tibial epiphyseal plates as unlike most mammals, rodent growth plates never ossify. Unfortunately, the original procedure to measure tibial epiphyseal plate width (TEPW) was developed for rats and yields poor results with mice. This paper demonstrates a simple method for silver staining growth plates that can be used to inexpensively and quickly measure the TEPW of mice. Poor visualization due to overstaining and the shattering of growth plates necessitated several revisions to the original protocol. These include exposing the growth plate prior to acetone dehydration, reducing the silver nitrate concentration from 2% to 1.5% and staining time from 2 min to 10 s and finally, the use of reflective light rather than transmissive light when imaging. The optimized protocol was then validated by generating an age-dependent TEPW growth curve that matched changes in tibia length. A total of 120 tibias were processed in a combined time of less than one day and for less than $30. By contrast, histological processing in the university's core facility would have cost $1440 and taken approximately three weeks. Thus, the revised protocol is vastly more cost effective, reliable and can be performed considerably quicker with minimal training.

  2. FGF upregulates osteopontin in epiphyseal growth plate chondrocytes: implications for endochondral ossification.

    PubMed

    Weizmann, S; Tong, A; Reich, A; Genina, O; Yayon, A; Monsonego-Ornan, E

    2005-12-01

    Fibroblast growth factor receptor 3 (FGFR3) signaling pathways are essential for normal longitudinal bone growth. Mutations in this receptor lead to various human growth disorders, including Achondroplasia, disproportionately short-limbed dwarfism, characterized by narrowing of the hypertrophic region of the epiphyseal growth plates. Here we find that FGF9, a preferred ligand for FGFR3 rapidly induces the upregulation and secretion of the matrix resident phosphoprotein, osteopontin (OPN) in cultured chicken chondrocytes. This effect was observed as early as two hours post stimulation and at FGF9 concentrations as low as 1.25 ng/ml at both mRNA and protein levels. OPN expression is known to be associated with chondrocyte and osteoblast differentiation and osteoclast activation. Unexpectedly, FGF9 induced OPN was accompanied by inhibition of differentiation and increased proliferation of the treated chondrocytes. Moreover, FGF9 stimulated OPN expression irrespective of the differentiation stage of the cells or culture conditions. In situ hybridization analysis of epiphyseal growth plates from chicken or mice homozygous for the Achondroplasia, G369C/mFGFR3 mutation demonstrated co-localization of OPN expression and osteoclast activity, as evidenced by tartarate resistant acid phosphatase positive cells in the osteochondral junction. We propose that FGF signaling directly activates OPN expression independent of chondrocytes differentiation. This may enhance the recruitment and activation of osteoclasts, and increase in cartilage resorption and remodeling in the chondro-osseus border.

  3. Efficiency of Human Epiphyseal Chondrocytes with Differential Replication Numbers for Cellular Therapy Products

    PubMed Central

    Nasu, Michiyo; Takayama, Shinichiro

    2016-01-01

    The cell-based therapy for cartilage or bone requires a large number of cells; serial passages of chondrocytes are, therefore, needed. However, fates of expanded chondrocytes from extra fingers remain unclarified. The chondrocytes from human epiphyses morphologically changed from small polygonal cells to bipolar elongated spindle cells and to large polygonal cells with degeneration at early passages. Gene of type II collagen was expressed in the cells only at a primary culture (Passage 0) and Passage 1 (P1) cells. The nodules by implantation of P0 to P8 cells were composed of cartilage and perichondrium. The cartilage consisted of chondrocytes with round nuclei and type II collagen-positive matrix, and the perichondrium consisted of spindle cells with type I collage-positive matrix. The cartilage and perichondrium developed to bone with marrow cavity through enchondral ossification. Chondrogenesis and osteogenesis by epiphyseal chondrocytes depended on replication number in culture. It is noteworthy to take population doubling level in correlation with pharmaceutical efficacy into consideration when we use chondrocytes for cell-based therapies. PMID:27999805

  4. Comparative studies of type X collagen expression in normal and rachitic chicken epiphyseal cartilage

    PubMed Central

    1989-01-01

    The levels of type X collagen in mineralizing normal chicken epiphyses and nonmineralizing rachitic chicken tibial epiphyses were measured and compared. Qualitative immunoperoxidase studies with anti-chick type X collagen monoclonal antibodies on sections from normal and rachitic cartilage demonstrated that the type X collagen levels in rachitic growth plates are reduced. Northern hybridization of mRNA and biosynthetic studies have confirmed that type X collagen synthesis in rickets is also decreased. In hypocalcemic rickets, the level of type X collagen mRNA is reduced by 80% whereas the level of type X collagen mRNA is only reduced by 50% in normocalcemic rickets. These observations provide additional evidence that type X collagen is involved in the process of cartilage mineralization and also suggest that the partial recovery of type X collagen synthesis in normocalcemic rickets may be related to the elevated plasma concentration of calcium. Calcium concentration may therefore play an important role in the control of type X collagen synthesis. PMID:2477383

  5. Discontinuities in the endothelium of epiphyseal cartilage canals and relevance to joint disease in foals.

    PubMed

    Hellings, Ingunn Risnes; Ekman, Stina; Hultenby, Kjell; Dolvik, Nils Ivar; Olstad, Kristin

    2016-01-01

    Cartilage canals have been shown to contain discontinuous blood vessels that enable circulating bacteria to bind to cartilage matrix, leading to vascular occlusion and associated pathological changes in pigs and chickens. It is also inconsistently reported that cartilage canals are surrounded by a cellular or acellular wall that may influence whether bacterial binding can occur. It is not known whether equine cartilage canals contain discontinuous endothelium or are surrounded by a wall. This study aimed to examine whether there were discontinuities in the endothelium of cartilage canal vessels, and whether canals had a cellular or acellular wall, in the epiphyseal growth cartilage of foals. Epiphyseal growth cartilage from the proximal third of the medial trochlear ridge of the distal femur from six healthy foals that were 1, 24, 35, 47, 118 and 122 days old and of different breeds and sexes was examined by light microscopy (LM), transmission electron microscopy (TEM) and immunohistochemistry. The majority of patent cartilage canals contained blood vessels that were lined by a thin layer of continuous endothelium. Fenestrations were found in two locations in one venule in a patent cartilage canal located deep in the growth cartilage and close to the ossification front in the 118-day-old foal. Chondrifying cartilage canals in all TEM-examined foals contained degenerated endothelial cells that were detached from the basement membrane, resulting in gap formation. Thirty-three percent of all canals were surrounded by a hypercellular rim that was interpreted as contribution of chondrocytes to growth cartilage. On LM, 69% of all cartilage canals were surrounded by a ring of matrix that stained intensely eosinophilic and consisted of collagen fibres on TEM that were confirmed to be collagen type I by immunohistochemistry. In summary, two types of discontinuity were observed in the endothelium of equine epiphyseal cartilage canal vessels: fenestrations were observed in

  6. Ipsilateral distal femoral and proximal tibial epiphyseal growth plate injury: a case report

    PubMed Central

    2013-01-01

    Introduction Both the isolated distal femoral epiphysiolysis and the isolated proximal tibial epiphysiolysis are the least common epiphyseal injuries. Even though they are uncommon, they have a high incidence rate of complications. Case presentation We present a case with Gustilo-Anderson grade 3b open and Salter-Harris type 1 epiphysiolysis of the distal femur and proximal tibia caused by a farm machinery accident. The patient was a 10-year-old boy, treated by open reduction and internal fixation. Conclusion Although distal femoral and proximal tibial growth plate injuries are rarely seen benign fractures, their management requires meticulous care. Anatomic reduction is important, especially to minimize the risk of growth arrest and the development of degenerative arthritis. However, there is a high incidence of growth arrest and neurovascular injury with these type of fractures. PMID:23724954

  7. Stimulation of body weight increase and epiphyseal cartilage growth by insulin like growth factor

    NASA Technical Reports Server (NTRS)

    Ellis, S.

    1981-01-01

    The ability of insulin-like growth factor (IGF) to induce growth in hypophysectomized immature rats was tested by continuous infusion of the partially purified factor at daily doses of 6, 21, and 46 mU for an 8-day period. A dose-dependent growth of the proximal epiphyseal cartilage of the tibia and an associated stimulation of the primary spongiosa were produced by these amounts of IGF. The two highest doses of IGF also resulted in dose-dependent increases of body weight. Gel permeation of the sera at neutrality showed that the large-molecular-weight IGF binding protein was not induced by the infusion of IGF, whereas it ws generated in the sera of hypophysectomized rats that were infused with daily doses of 86 mU of human growth hormone.

  8. Epiphyseal distraction and centrally located bone bar: an experimental study in the rabbit.

    PubMed

    Cottalorda, J; Jouve, J L; Bollini, G; Panuel, M; Guisiano, B; Jimeno, M T

    1996-01-01

    The purpose of this study was to analyze the force required for an epiphysiolysis in vitro in the distal femoral growth plate of rabbits with different sized central physeal bone bars. The bars were operatively induced by drilling through the physis. An epiphyseal distraction was then performed, and the response was evaluated. Forty rabbits (67 femurs) divided into five groups were used for experimentation. Group A had not been operated on and manifested no physeal bone bridge. Groups B, C, D, and E manifested some bone bridges obtained surgically by drills 2, 2.5, 3.5, and 4.5 mm in diameter, respectively. The average force required to obtain an epiphysiolysis is not statistically significant in the different groups. However, we noted that the epiphysiolysis differed depending on the diameter of the surgically performed bridge. Group E was Salter-Harris type II fractures, whereas groups A, B, and C were type I fractures. Group D comprised both types.

  9. Refinement of the localization of the gene responsible for spondylo epiphyseal dysplasia in Xp22

    SciTech Connect

    Hors-Cayla, M.C.; Heuertz, S.; Smahi, A.

    1994-09-01

    Spondylo epiphyseal dysplasia tarda (SEDL) (MIM 313400) is an X-linked recessive disease characterized by short stature which is caused by a growth defect of the vertebral bodies. Using RFLP markers, we have previously localized the gene responsible to Xp22 between DXS16 and DXS92 in a 13% recombination fraction interval. Further genetic analysis was carried out using microsatellite markers. As their localization with respect to the RFLP markers was unknown, we first obtained a physical map integrating RFLP and microsatellite markers, with the help of a panel of radiation reduced hybrids. Studying recombinants between the SEDL gene and RFLP and microsatellite markers, we refined the position of the gene on Xp22 between DXS16 and DXS987 on a 1 to 2 Mb chromosomal segment.

  10. Fate of the hypertrophic chondrocyte: microenvironmental perspectives on apoptosis and survival in the epiphyseal growth plate.

    PubMed

    Shapiro, Irving M; Adams, Christopher S; Freeman, Theresa; Srinivas, Vickram

    2005-12-01

    The goal of this review is to examine the fate of the hypertrophic chondrocyte in the epiphyseal growth plate and consider the impact of the cartilage microenvironment on cell survival and apoptosis. Early investigations pointed to a direct role of the hypertrophic chondrocyte in osteogenesis. The terminally differentiated cells were considered to undergo a dramatic change in shape, size, and phenotype, and assume the characteristics of an osteoblast. While some studies have supported the notion of transdifferentiation, much of the evidence in favor of reprogramming epiphyseal chondrocytes is circumstantial and based on microscopic evaluation of cells that are present at the chondro-osseous junction. Although these investigations provided a novel perspective on endochondral bone formation, they were flawed by the failure to consider the importance of stem cells in osseous tissue formation. Subsequent studies indicated that many, if not all, of the cells of the cartilage plate die through the induction of apoptosis. With respect to agents that mediate apoptosis, at the chondro-osseous junction, solubilization of mineral and hydrolysis of organic matrix constituents by septoclasts generates high local concentrations of ions, peptides, and glycans, and secreted matrix metalloproteins. Individually, and in combination, a number of these agents serve as potent chondrocyte apoptogens. We present a new concept: hypertrophic cells die through the induction of autophagy. In the cartilage microenvironment, combinations of local factors cause chondrocytes to express an initial survival phenotype and oxidize their own structural macromolecules to generate ATP. While delaying death, autophagy leads to a state in which cells are further sensitized to changes in the local microenvironment. One such change is similar to ischemia reperfusion injury, a condition that leads to tissue damage and cell death. In the growth cartilage, an immediate effect of this type of injury is

  11. Histological disorders related to the focal disappearance of the epiphyseal growth plate in rats induced by high dose of vitamin A.

    PubMed

    Soeta, S; Mori, R; Kodaka, T; Naito, Y; Taniguchi, K

    2000-03-01

    The histological disorders related to the focal disappearance of the epiphyseal growth plate were examined histochemically in the proximal tibia of rats administered a high dose of vitamin A. Animals were given 100,000 IU/100 g body weight/day of vitamin A for 5 days from 4 weeks after birth (VA rats) or given deionized water as control and sacrificed on Day 12 and 19 of the experiment. Tibiae were examined by immunohistochemistry for type I, II and X collagens, lectin-histochemistry for Helix pomatia and backscattered electron imaging. On Day 12, the abnormally developed calcified cartilage matrix was detected within the epiphyseal growth plate in VA rats. The uncalcified cartilage matrix contained type I collagen but lacked type II collagen. In addition, the eroded regions accompanied with numerous osteoclasts and osteoblasts were detected in the epiphyseal growth plate. On day 19, eroded regions penetrated the epiphyseal growth plate to result in its focal disappearances with the eroded surfaces entirely covered with bone tissue in VA rats. These findings suggested that the cartilage matrix of the epiphyseal growth plate was abnormally calcified and showed the phenotypes like bone matrix. The eroded regions of the epiphyseal growth plate seemed to be caused by the invasion of osteoclasts into the altered cartilage matrix and might develop to the focal disappearances by the modeling or remodeling due to action of osteoclasts and osteoblasts.

  12. Responses of articular and epiphyseal cartilage zones of developing avian radii to estrone treatment and a 2-G environment

    NASA Technical Reports Server (NTRS)

    Negulesco, J. A.; Kossler, T.

    1978-01-01

    Histological measurements of radii from chickens exposed to estrone and hypergravity are reported. Female chicks at two weeks post-hatch were maintained for two weeks at earth gravity or 2 G with daily injections of 0.2 or 0.4 mg estrone. Animals were sacrificed after the last injection, and the radii were processed by described histological techniques. The results suggest that proximal and distal epiphyses of developing radii show different morphological responses to estrone and hypergravity.

  13. The Effects of Low-Level Laser Therapy, 670 nm, on Epiphyseal Growth in Rats

    PubMed Central

    de Andrade, Adriana Regina; Meireles, Anamaria; Artifon, Elisangela Lourdes; Brancalhão, Rose Meire Costa; Ferreira, José Roberto Leonel; Bertolini, Gladson Ricardo Flor

    2012-01-01

    The longitudinal growth of long bones is attributed to epiphyseal growth. However, the effects of low-level laser therapy (LLLT) in such structures has still not been studied extensively in the literature. Therefore, the aim of this study was to evaluate the use of LLLT, 670 nm, at three different doses on the epiphyseal growth of the right tibia of rats. Twenty-one Wistar rats, aged four weeks, were subjected to the application of LLLT, with dosage according to the group (G4: were submitted to the application of 4 J/cm2; G8: were submitted to the application of 8 J/cm2; G16: were submitted to the application of 16 J/cm2). After completion of protocol they were kept until they were 14 weeks of age and then submitted to a radiological examination (evaluation of limb length) and euthanised. The histological analysis of the growth plates (total thickness and hypertrophic and proliferative zones) was then performed. Comparisons were made with the untreated left tibia. No differences were observed in any of the reviews (radiological and histological), when comparing the right sides (treated) to the left (untreated). It was concluded that the treatment with LLLT within the parameters used caused changes neither in areas of the epiphyseal cartilage nor in the final length of limbs. PMID:22654576

  14. Premature epiphyseal closure in an adolescent treated by retinoids for acne: an unusual cause of anterior knee pain.

    PubMed

    Luthi, François; Eggel, Yan; Theumann, Nicolas

    2012-05-01

    Retinoids are effective and widely prescribed in the treatment of severe acne. However their use can be associated with numerous side effects. Some rare cases of premature epiphyseal closure were reported. We present the case of a sixteen-year-old soccer player referred for progressive anterior pain in both knees, evoking a patellar problem. Careful pharmacological questioning revealed use of isotretinoin for several months. MRI findings showed an irregularity of the growth plate and an important metaphyso-epiphyseal oedema, more noticeable in the left knee. Retinoid-induced premature epiphyseal closure was diagnosed. The treatment was stopped, with a resolution of pain within two months. After recovery a persisting small sequelar thumbprint-like growth plate lesion was observed on the control MRI. Retinoids induce an invasion of the growth plate by osteoclasts and a decrease in proteoglycans synthesis. It seems that the knee is the most affected joint. This complication being rare, a radiological follow-up of the young patients treated by retinoids is not proposed.

  15. Extensive risk analysis of mechanical failure for an epiphyseal hip prothesis: a combined numerical-experimental approach.

    PubMed

    Martelli, S; Taddei, F; Cristofolini, L; Gill, H S; Viceconti, M

    2011-02-01

    There has been recent renewed interest in proximal femur epiphyseal replacement as an alternative to conventional total hip replacement. In many branches of engineering, risk analysis has proved to be an efficient tool for avoiding premature failures of innovative devices. An extensive risk analysis procedure has been developed for epiphyseal hip prostheses and the predictions of this method have been compared to the known clinical outcomes of a well-established contemporary design, namely hip resurfacing devices. Clinical scenarios leading to revision (i.e. loosening, neck fracture and failure of the prosthetic component) were associated with potential failure modes (i.e. overload, fatigue, wear, fibrotic tissue differentiation and bone remodelling). Driving parameters of the corresponding failure mode were identified together with their safe thresholds. For each failure mode, a failure criterion was identified and studied under the most relevant physiological loading conditions. All failure modes were investigated with the most suitable investigation tool, either numerical or experimental. Results showed a low risk for each failure scenario either in the immediate postoperative period or in the long term. These findings are in agreement with those reported by the majority of clinical studies for correctly implanted devices. Although further work is needed to confirm the predictions of this method, it was concluded that the proposed risk analysis procedure has the potential to increase the efficacy of preclinical validation protocols for new epiphyseal replacement devices.

  16. Clinical and radiographic findings in multiple epiphyseal dysplasia caused by MATN3 mutations: description of 12 patients.

    PubMed

    Mäkitie, Outi; Mortier, Geert R; Czarny-Ratajczak, Malwina; Wright, Michael J; Suri, Mohnish; Rogala, Piotr; Freund, Margarida; Jackson, Gail C; Jakkula, Eveliina; Ala-Kokko, Leena; Briggs, Michael D; Cole, William G

    2004-03-15

    Multiple epiphyseal dysplasia (MED) is characterized by pain and stiffness in joints and delayed and irregular ossification of epiphyses. Causative mutations have been recognized in six different genes. We have identified disease-causing mutations in the gene encoding matrilin-3, an extracellular matrix protein, in seven families with autosomal dominant MED. Review of the clinical and radiographic features in 12 of the affected family members shows a uniform pattern of skeletal anomalies in all patients with considerable degree of variability in severity, both between and within families. The characteristic clinical findings are onset of symptoms in early childhood with predominance of knee and hip related complaints, normal stature, and early-onset osteoarthritis. Radiographs show small and irregular epiphyses and mild metaphyseal irregularities and striations, especially at the knees and hips and mild spinal changes. Despite overlap, both clinically and radiographically, with other forms of MED, the described features may help to differentiate this particular form from other entities within the MED spectrum.

  17. Study on the effect of T-2 toxin combined with low nutrition diet on rat epiphyseal plate growth and development.

    PubMed

    Yao, Yun-fen; Kang, Peng-de; Li, Xing-bo; Yang, Jing; Shen, Bing; Zhou, Zong-ke; Pei, Fu-xing

    2010-12-01

    The purpose of this study was to observe early lesions of rat epiphyseal plates and metaphysis caused by T-2 toxin and T-2 toxin combined with a low nutrition diet to determine possible pathogenic factors of Kashin-Beck disease (KBD). Ninety Wistar rats were divided into three groups. Group A was fed with a normal diet as control; group B was fed with a normal diet and T-2 toxin; and group C was fed with a low nutrition diet and T-2 toxin. The left knee specimens were collected, fixed in formaldehyde solution, stained by hematoxylin and eosin and Masson. After two weeks, the epiphyseal plate showed necrosis of chondrocytes in groups B and C. After four weeks, more obvious chondrocyte necrosis appeared. The positive rate of Lamellar necrosis in group C was significantly higher than that in groups B and A (P < 0.01). Metaphyseal trabecular bone showed sparse disorder and disruption in group C. T-2 toxin combined with a low nutrition diet could lead to more serious chondrocyte necrosis in the epiphyseal plate and disturb metaphyseal trabecular bone formation.

  18. All-Epiphyseal Anterior Cruciate Ligament Reconstruction in Skeletally Immature Patients: A Surgical Technique Using a Split Tibial Tunnel

    PubMed Central

    Lykissas, Marios G.; Nathan, Senthil T.; Wall, Eric J.

    2012-01-01

    Many techniques have been described for anterior cruciate ligament (ACL) reconstruction in skeletally immature patients, including extra-articular, complete or partial transphyseal, and physeal-sparing techniques. An all-epiphyseal technique places the tendon and its tunnels and fixation all within the child's epiphysis, leaving the growth plates untouched. We describe an all-epiphyseal quadruple-hamstring ACL reconstruction using a split tibial tunnel. The split tibial tunnels drop the tunnel size down to 4.5 to 5.5 mm from 7 to 8 mm because only half the total graft diameter passes through each of the split tunnels. This increases the safety margin for keeping the tunnel within the tibial epiphysis, in addition to avoiding damage into the growth plate. The bone bridge between the 2 tunnels serves as a solid low-profile fixation post. Femoral graft fixation is achieved with an interference screw, which allows precise tensioning and low-profile fixation entirely within the femoral tunnel. By placing the graft at the native ACL's anatomic attachment points without spanning or violating the growth plates at any step of the procedure, an all-epiphyseal ACL reconstruction with a split tibial tunnel theoretically minimizes the risk of growth disturbance in an ACL-deficient child. PMID:23766968

  19. PIM-2 is an independent regulator of chondrocyte survival and autophagy in the epiphyseal growth plate.

    PubMed

    Bohensky, Jolene; Shapiro, Irving M; Leshinsky, Serge; Watanabe, Hitoshi; Srinivas, Vickram

    2007-10-01

    The overall goal of the investigation was to examine the activity and role of the PIM serine/threonine protein kinases in the growth plate. We showed for the first time that PIM-2 was highly expressed in epiphyseal chondrocytes and that the kinase was required for critical activities linked to cell survival. These activities were independent of those mediated by Akt-1. It was noted that PIM-2 protected chondrocytes from rapamycin sensitized (TOR inhibited) cell death. Since inhibition of mTOR caused autophagy, we examined the autophagic response of PIM-2 silenced cells. We showed that PIM-2 promoted expression and organization of autophagic proteins LC3, and Beclin-1 and enhanced lysosomal acidification. At the same time, PIM-2 modulated the activity of a key regulator of apoptosis, BAD. Since BAD inhibition and Beclin-1 expression activated autophagy, it is likely that induction of the autophagic pathway would serve to inhibit apoptosis and preserve the life of the terminally differentiated chondrocyte. We conclude that PIM-2 regulates a new intermediate stage in the differentiation pathway, the induction of autophagy.

  20. Evaluation of activity of epiphyseal plates in growing males and females.

    PubMed

    Yang, K-T A; Yang, A D

    2006-06-01

    To investigate the age-related activity of the epiphyseal plates, a retrospective study of (99m)Tc-methylene diphosphonate bone scans was undertaken. The study comprised 81 males and 46 females aged 2 weeks to 24 years. The total percentage (%) whole-body (ratio of total physis activity to whole-body activity) and the regional % whole-body (ratio of physis activity of one region to whole-body activity) were derived. The ratio of physis activity of one region to the total physis activity was defined as % physis. Before age 12, total physis activity was found to contribute about 10% to whole-body activity. All total and regional % whole-body activities followed sigmoid curves with age. The differences of the parameters (transition centers and widths) suggested that there might be a later and longer period for the disappearance of physis activity in males than in females. For all the regions, % physis changed little with age until after puberty. At age <1, the proportion of bone activity in the body was about 30-35% for skull, 20-25% for lower limbs, and 5-15% for the rest of the regions. The maximal changes during growth occurred in the skull and the lower limbs. The age-related changes of physis activity during growth reflect a combination of the potential of bone to grow and the processes of bone growth and bone turnover. Bone scintigraphy is useful in understanding the changes of physis activity during growth.

  1. Epiphyseal fusion in the human growth plate does not involve classical apoptosis.

    PubMed

    Emons, Joyce; Chagin, Andrei S; Hultenby, Kjell; Zhivotovsky, Boris; Wit, Jan M; Karperien, Marcel; Sävendahl, Lars

    2009-12-01

    By the end of puberty, growth ceases and epiphyseal fusion occurs through mechanisms not yet completely understood. Human growth plate tissues were collected in various pubertal stages including a unique late pubertal growth plate, which was about to fuse. Apoptosis was studied by TUNEL staining, immunolocalization of pro- and antiapoptotic proteins, and electron microscopy (EM). Morphologic analyses of the fusing growth plate revealed disorganized, large chondrocytes surrounded by a border of dense, cortical-like bone. In the unfused growth plates, few chondrocytes were TUNEL positive. In contrast, the fusing growth plate contained no single TUNEL-positive cell. Antiapoptotic (Bcl-2 and Bcl-XL) and proapoptotic (Bax, Bad, and cleaved caspase-3) proteins were detected in all growth plate zones without change in intensity during pubertal progression. Expression of antiapoptotic proteins was found in the fusing growth plate but of the proapoptotic proteins only Bad was detected. EM revealed no typical signs of apoptosis or autophagy in any of the growth plates. In contrast, morpohological signs of hypoxia and necrosis were observed. We conclude that classical apoptosis is not likely to be involved in the process of human growth plate fusion.

  2. Genetic linkage mapping of multiple epiphyseal dysplasia to the pericentromeric region of chromosome 19

    SciTech Connect

    Oehlmann, R.; Summerville, G.P.; Yeh, G.; Weaver, E.J.; Jimenez, S.A.; Knowlton, R.G. )

    1994-01-01

    Multiple epiphyseal dysplasia (MED) is an inherited chondrodystrophy that results in deformity of articular surfaces and in subsequent degenerative joint disease. The disease is inherited as an autosomal dominant trait with high penetrance. An MED mutation has been mapped by genetic linkage analysis of DNA polymorphisms in a single large pedigree. Close linkage of MED to 130 tested chromosomal markers was ruled out by discordant inheritance patterns. However, strong evidence for linkage of MED to markers in the pericentromeric region of chromosome 19 was obtained. The most closely linked marker was D19S215, with a maximum LOD score of 6.37 at [theta] = .05. Multipoint linkage analysis indicated that MED is located between D19S212 and D19S215, a map interval of 1.7 cM. Discovery of the map location of MED in this family will facilitate identification of the mutant gene. The closely linked DNA polymorphisms will also provide the means to determine whether other inherited chondrodystrophies have underlying defects in the same gene. 29 refs., 3 figs., 1 tab.

  3. Desepiphysiodesis--elimination of partial premature epiphyseal closure. Experience of 17 cases.

    PubMed

    Bronfen, C; Rigault, P; Glorion, C; Touzet, P; Padovani, J P; Finidori, G; Chaumien, J P; Arlet, V; Wioland, M; Bonnerot, V

    1994-02-01

    Between 1975 and 1990, 17 growth plates have been operated on by epiphyseal bridge resection. The children were from 4 years and 10 months to 13 years and 10 months old. The etiology of partial closure was traumatic (10 times), caused by therapeutic mistakes (3 times), septic osteomyelitis (1 case), purpura fulminans (1 case), unknown (2 cases). There was always length discrepancy or deformity of bone. The regions that have been subjected to treatment were distal femur, proximal tibia, distal tibia, distal radius. Evaluation of the bone bridge was made by tomoscintigraphies and recently by MR imaging and computed tomoscintigraphy. The bone bridge size was from 2.5% to 60% of the growth plate surface; surgical technique consists of resection of bone bridge connecting epiphysis and metaphysis which is replaced by methyl metacrylate. In 16 cases simultaneous corrective osteotomy was performed. Results are poor, there were only two good results and 8 failures; seven results were medium. The failures can all be explained by mistakes in technique or indication, except one. Indications are post-traumatic narrow bridges in young children. It would be useful to know the vitality of the residual growth plate.

  4. Epiphyseal Sparing and Reconstruction by Frozen Bone Autograft after Malignant Bone Tumor Resection in Children

    PubMed Central

    Hamed Kassem Abdelaal, Ahmed; Yamamoto, Norio; Hayashi, Katsuhiro; Takeuchi, Akihiko; Miwa, Shinji; Tsuchiya, Hiroyuki

    2015-01-01

    Limb salvage surgery has become the standard treatment for malignant primary bone tumors in the extremities. Limb salvage represents a challenge in skeletally immature patients. Several treatment options are available for limb reconstruction after tumor resection in children. We report our results using the technique of epiphyseal sparing and reconstruction with frozen autograft bone in 18 children. The mean follow-up period for the all patients included in this study is 72 ± 26 m. Eight patients remained disease-free, seven patients lived with no evidence of disease, two were alive but with disease, and one patient died of the disease. Five- and ten-year rates of survival were 94.4%. Graft survival at 5 and 10 years was 94.4%. Functional outcome using the Enneking scale was excellent in 17 patients (94.4%) and poor in one patient (5.5%). Complications include 2 nonunions, 2 fractures, 2 deep infections, 1 soft tissue recurrence, and leg length discrepancy in 7 cases. This technique is a good reconstructive choice in a child with a nonosteolytic primary or secondary bone tumor, responsive to chemotherapy, without involvement of the articular cartilage. It is a straight forward, effective, and biological technique, which affords immediate mobilization of joints and possible cryoimmune effects, with excellent long term functional outcome and less complication. PMID:27034614

  5. [Multiple epiphyseal separations in a child with cerebral palsy and scurvy].

    PubMed

    Iacono, O; Datola, A; Barbagallo, M L; Greco, F; Sorge, G

    2009-08-01

    Scurvy is a dietary disease due to Vitamin C deficiency. Vitamin C is related to collagen synthesis and metabolism. Malnutrition, problems in bowel absorption, alcoholism and cerebral palsy are clearly often linked with scurvy, even it is no more common in the industrialized countries. Its clinical features are: asthenia, weight loss, appetite decrease, irritability, gingival or mucous lesions, porpora, follicular hyperkeratosis, musculoskeletal pain due to multiple fractures and subperiosteal bleedings, pseudoparalisis (frog-like position of legs) and refuse to walk. Authors report on a nine year-old girl with spastic quadriplegic cerebral palsy, who at the first examination showed deep anemia, fever and multiple epiphyseal separation of the right shoulder and the left knee. Diagnosis of scurvy was been made. The aim of this article was to underline the rarity and gravity of this disease, and its even more frequent appearance in children affected of cerebral palsy. Substitutive therapy consists on ascorbic acid supplementation. Complete restitutio ad integrum of skin-mucous injuries, such as gingival bleedings, was achieved within three months.

  6. Isometric Scaling in Developing Long Bones Is Achieved by an Optimal Epiphyseal Growth Balance.

    PubMed

    Stern, Tomer; Aviram, Rona; Rot, Chagai; Galili, Tal; Sharir, Amnon; Kalish Achrai, Noga; Keller, Yosi; Shahar, Ron; Zelzer, Elazar

    2015-08-01

    individual epiphyseal plates.

  7. Induction and characterization of metallothionein in chicken epiphyseal growth plate cartilage chondrocytes.

    PubMed

    Sauer, G R; Nie, D; Wu, L N; Wuthier, R E

    1998-01-01

    Following exposure to cadmium or zinc, chickens were sacrificed and the liver, kidney, and bone epiphyseal growth plates harvested. When cytosolic extracts of the growth plate cartilage were fractionated by gel filtration chromatography, a protein with high metal-binding capacity and low ultraviolet (UV) absorbance eluted in the same position as liver metallothionein (MT) and a MT standard. Cd or Zn treatment resulted in a 25-fold or 5-fold induction in growth plate MT, respectively. In liver the greatest level of MT induction was seen with short-term Cd exposures. In contrast, MT levels in the growth plate increased as the duration of Cd exposure increased. Induction of MT in growth plate chondrocyte cell cultures was observed for media Cd concentrations of > or = 0.1 microM and Zn concentrations of > or = 100 microM. Basal and inducible levels of MT declined through the culture period and were lowest in the terminally differentiated mineralized late stages of the culture. Alkaline phosphatase activity was also lowest in the late-stage cultures, while total cellular protein increased throughout the culture period. Treatment of chondrocytes with Zn prior to Cd exposure resulted in a protective induction of MT. Pre-treatment of chondrocytes with dexamethasone resulted in suppressed synthesis of MT upon Cd exposure and greater Cd toxicity. Both Cd and Zn resulted in significantly increased levels of MT mRNA in chondrocyte cell cultures. Dexamethasone treatment resulted in an approximate 2- to 3-fold increase in MT mRNA. This is contrary to the finding that MT protein levels were decreased by dexamethasone. The findings suggest that an increased rate of MT degradation in dexamethasone-treated and late-stage chondrocyte cultures may be associated with the terminally differentiated phenotype.

  8. A mathematical model of epiphyseal development: hypothesis on the cartilage canals growth.

    PubMed

    Garzon-Alvarado, Diego Alexander; Peinado Cortes, Liliana Mabel; Cardenas Sandoval, Rosy Paola

    2010-12-01

    The role of cartilage canals is to transport nutrients and biological factors that cause the appearance of the secondary ossification centre (SOC). The SOC appears in the centre of the epiphysis of long bones. The canal development is a complex interaction between mechanical and biological factors that guide its expansion into the centre of the epiphysis. This article introduces the 'Hypothesis on the growth of cartilage canals'. Here, we have considered that the development of these canals is an essential event for the appearance of SOC. Moreover, it is also considered to be important for the transport of molecular factors (RUNX2 and MMP9) at the ends of such canals. Once the canals are merged in the centre of the epiphysis, these factors are released causing hypertrophy of adjacent cells. This RUNX2 and MMP9 release occurs due to the action of mechanical loads that supports the epiphysis. In order to test this hypothesis, we use a hybrid approach using the finite element method to simulate the mechanical stresses present in the epiphysis and the cellular automata to simulate the expansion of the canals and the hypertrophy factors pathway. By using this hybrid approach, we have obtained as a result the spatial-temporal patterns for the growth of cartilage canals and hypertrophy factors within the epiphysis. The model is in qualitative agreement with experimental results previously reported by other authors. Thus, we conclude that this model may be used as a methodological basis to present a complete mathematical model of the processes involved in epiphyseal development.

  9. Beneficial effects of beta-Ecdysone on the joint, epiphyseal cartilage tissue and trabecular bone in ovariectomized rats.

    PubMed

    Kapur, P; Wuttke, W; Jarry, H; Seidlova-Wuttke, D

    2010-04-01

    Ecdysteroids are steroids found in invertebrates and plants. In mammals they have protein anabolic effects. We have recently published antiosteoporotic effects of Tinospora cordifolia (TC) extract and the search for the possible active ingredients yielded the presence of beta-Ecdysone (Ecd). Therefore, we investigated the effects of pure Ecd in ovariectomized rats on morphological changes in joint, epiphyseal cartilage and trabecular tissue. Following ovariectomy rats were fed for 1 month with Ecd containing food at a dose of 52.8 mg/day/animal. Positive and negative control animals received 17-beta Estradiol (E(2), 132 microg/day/animal) and soy free (sf) food respectively. At sacrifice, specimens consisting of upper tibiae-lower femurs and knee joint were harvested and processed for histomorphometry. The parameters measured included thickness of the joint cartilage, thickness of the whole epiphyseal growth plate and its three zones. Furthermore, the percentage of trabecular bone in the metaphysis region of tibiae was quantified. Ecd and E(2) induced a significant increase in the thickness of joint cartilage. The whole epiphyseal growth plate and its proliferative and hypertrophic zones were also increased by Ecd whereas E(2) reduced their size. The percentage of trabecular area in the metaphysis of tibia was significantly increased in Ecd and E(2) treated animals. Results provide a plausible explanation for the antiosteoporotic effects of TC. Hence, TC as well as other Ecd producing plants or pure Ecd may be of value in the prevention and treatment of osteoporosis and osteoarthritis which is of increasing importance due to aging and obesity among individuals.

  10. An unusual form of spondyloepiphyseal dysplasia, with advanced carpal and spinal end-plate ossification mimicking COMP-mutation-like multiple epiphyseal dysplasia.

    PubMed

    Pazzaglia, Ugo Ernesto; Beluffi, Giampiero; Zarattini, Guido

    2008-07-01

    We present a child with irregular ossification of tubular bone epiphyses, short bones, and spine. The radiographic evolution of bones undergoing endochondral ossification was followed from the age of 1 year 9 months to 6 years. The unusual features demonstrated in this child made classification difficult: pseudoachondroplasia was excluded because no mutations of the COMP gene were found. Considering the evolution of the radiographic appearances, the most likely diagnosis would seem to be an unusual form of spondyloepiphyseal dysplasia, mimicking some aspects of multiple epiphyseal dysplasia. Endochondral ossification was diffusely altered with a mixture of epiphyseal ossification delay associated with acceleration and early fusion. This case was a unique presentation within the family, suggesting a mutation in the affected child.

  11. Galeazzi - Equivalent Pronation Type Injury with Splitting of Ulnar Epiphyseal Plate into Two Fragments – A Rare Case Report and Review of Literature

    PubMed Central

    J, Ashish Suthar; V, Ashish Kothari

    2014-01-01

    Introduction: In children and adolescents distal forearm physeal fractures are common. Usually distal forearm physeal injuries of are common injuries in children and adolescents. Epiphyseal injuries to the distal radius are common in children, but involvement of the distal ulna is rare. Fracture of the distal radius with dislocation of the DRUJ is known as a True Galeazzi fracture dislocation and an epiphyseal separation of the distal ulna occurred instead of dislocation of DRUJ or both)[10] is called Galeazzi equivalent lesions. Galeazzi fractures in children are less common than in adults. [4] These injuries are uncommon and there are few descriptions of them in the current literature. Case Report: Here we report the case of a 13-year-old boy, student with history of RTA presented with pain and swelling of distal forearm diagnosed with closed injury of Galeazzi equivalent type. Here injury to the distal ulnar epiphyseal plate is in the form of epiphyseal separation (Salter Harris type I / Peterson type III) with splitting of epiphysis into two fragment – [ulnar styloid & radial side of ulnar epiphyseal plate] (Salter Harris type III / Peterson type IV) with fracture of metaphysis of lower end radius (Peterson type I) without neurovascular deficit. Patient was given surgical treatment in the form of closed reduction and K-wire fixation for fracture of distal radius and open reduction using extended ulnar approach and fixation with K-wire for ulnar epiphyseal fracture as closed reduction was not possible due to soft tissue interposition. Conclusion: Galeazzi equivalent injury is rare. It may require radiographic comparison of opposite uninvolved distal forearm with wrist, CT or MR imaging to define injury accurately. It may also require open reduction for anatomical or acceptable reduction of fracture to minimize chances of growth arrest which may occur as a complication of injury. It is also necessary for frequent follow up to identify complication early

  12. Role of simple cold storage in preventing epiphyseal growth plate impairment after replantation surgery in immature rats.

    PubMed

    Sunagawa, Toru; Ishida, Osamu; Ikuta, Yoshikazu; Yasunaga, Yuji; Ochi, Mitsuo

    2005-10-01

    The purpose of this study was to investigate the effect of ischemia time on longitudinal bone growth after replantation, and to evaluate the usefulness of simple cold storage of an amputated limb in preventing epiphyseal growth-plate impairment. A hind-limb replantation model was produced with 5-week-old rats after various ischemia times. With more than 6 hr of warm ischemia, growth disturbance was observed, and extensive necrosis was histologically apparent in the central region of the growth plate of the proximal tibia, even at 1 week postoperatively. Destruction of the growth plate was complete at 4 weeks after surgery. By simple cold storage of the amputated limb, growth disturbance was not observed, even following ischemia for 9 hr, and no abnormal findings were observed histologically. Segmental necrosis and destruction of the growth plate observed histologically suggested the possibility of indirect impairment of the epipyseal chondrocytes due to disturbance of the epiphyseal arterial system, attributable to ischemia. The study confirmed experimentally that cold storage of the amputated part may prevent longitudinal bone-growth disturbance after extension of ischemia time.

  13. Immunohistochemical examination of epiphyseal growth plates of Japanese Brown cattle with chondrodysplasia.

    PubMed

    Soeta, S; Shimoura, H; Hatakeyama, N; Kodaka, T; Amasaki, H; Yamano, S; Taniguchi, K; Naito, Y

    2007-01-01

    A new type of inherited chondrodysplasia is described in Japanese Brown cattle, but the basic defects of the epiphyseal growth plate (EGP) in the limb long bones, and proliferation and differentiation of the chondrocytes in the EGP, are not yet understood. In the present study, the EGPs of the limb long bones in eight cases of chondrodysplasia and four normal (control) cattle were examined histologically and immunohistochemically. In the control cattle, proliferative chondrocytes (PCs) and hypertrophic chondrocytes (HCs) were arranged in columns parallel to the long axis of the bone, and HCs were situated on the metaphyseal side of the EGP. In all the affected cattle, many chondrocytes with a hypertrophic appearance were detected in the inner areas of the central portion of the EGP. The PC columns were short and arranged irregularly. Bone tissue and small blood vessels were found frequently in these areas. Six affected cattle showed complete EGP-closure. Backscattered electron (BSE) imaging showed that the calcified cartilage matrix was restricted to the lower region of the hypertrophic zone (HZ) of the EGP in the control cattle, while the calcified cartilage matrix and bone tissue were scattered in the inner areas of the EGP in all the chondrodysplastic cattle. Immunohistochemistry revealed type X collagen in the HCs and cartilage matrix of the HZ in the control cattle. In all the affected cattle, type X collagen was detected in apparently hypertrophic chondrocytes in the inner areas of the EGP. Type II collagen was detected in the entire EGP in all the affected cattle, as in the controls. BrdU (5-bromo-2'-deoxyuridine), injected intravenously 1h before euthanasia was detected in many PCs in the EGP in the control cattle; none, however, was detected in the central portion of the EGP in any affected animal. These observations indicate that differentiation into HCs and calcification of cartilage matrix occur in the inner areas of the central portion of the EGP in

  14. Epiphyseal dysplasia of the femoral head, mild vertebral abnormality, myopia, and sensorineural deafness: report of a pedigree with autosomal dominant inheritance.

    PubMed Central

    MacDermot, K D; Roth, S C; Hall, C; Winter, R M

    1987-01-01

    A family is presented with short stature, femoral epiphyseal dysplasia, mild vertebral changes, and sensorineural deafness inherited as an autosomal dominant trait. Myopia and retinal detachment presenting in adult life were also present in some affected members. We suggest that this disorder may be a distinct entity within the spondyloepiphyseal dysplasia group of disorders. Images PMID:3681905

  15. Chondrocytes embedded in the epiphyseal growth plates of long bones undergo autophagy prior to the induction of osteogenesis.

    PubMed

    Srinivas, Vickram; Shapiro, Irving M

    2006-01-01

    Bone growth takes place through the activities of chondrocytes embedded in the epiphyseal growth plate. Stress conditions in the plate can promote the autophagic response through the modulation of genes controlling metabolite utilization. mTOR plays a critical role in autophagy serving as the sensor that integrates metabolic and growth factor signals. Ongoing studies indicate that terminal chondrocytes exhibit autophagic characteristics. Morphologically, the arrested cells contain double membrane vacuoles; there is a loss of membrane structure, limited staining and organelle destruction. Since the life history of the growth plate chondrocyte is very short, even minor disturbances in the metabolic state can result in gross impairment of growth. We contend that the induction of the autophagic response, permits the terminally differentiated cells to survive the brief rigors of the harsh local microenvironment. Whether chondrocytes can recover from this state, and possibly participate in osteogenesis, is not known at this time.

  16. Leber's congenital amaurosis associated with familial juvenile nephronophthisis and cone-shaped epiphyses of the hands (the Saldino-Mainzer syndrome).

    PubMed

    Ellis, D S; Heckenlively, J R; Martin, C L; Lachman, R S; Sakati, N A; Rimoin, D L

    1984-02-01

    Three affected children (a 13-year-old girl and her 7- and 8-year-old brothers) in a sibship of eight had findings consistent with the Saldino-Mainzer syndrome (skeletal dysplasia associated with Leber's congenital amaurosis, familial juvenile nephronophthisis, and cone-shaped epiphyses of the hands). Two also had pigmented midline nevi. Although tapetoretinal degeneration and familial juvenile nephronophthisis are associated in the inherited Senior-Loken syndrome, the rare association of these abnormalities with cone-shaped epiphyses of the hands suggested an autosomal recessive syndrome with variable expression remarkably similar to the Saldino-Mainzer syndrome, which may or may not be distinct from the Senior-Loken syndrome. The association of tapetoretinal degeneration with skeletal dysplasia may indicate asymptomatic renal or hepatic disease.

  17. ESET histone methyltransferase is essential to hypertrophic differentiation of growth plate chondrocytes and formation of epiphyseal plates.

    PubMed

    Yang, Liu; Lawson, Kevin A; Teteak, Colin J; Zou, Junhui; Hacquebord, Jacques; Patterson, David; Ghatan, Andrew C; Mei, Qi; Zielinska-Kwiatkowska, Anna; Bain, Steven D; Fernandes, Russell J; Chansky, Howard A

    2013-08-01

    The ESET (also called SETDB1) protein contains an N-terminal tudor domain that mediates protein-protein interactions and a C-terminal SET domain that catalyzes methylation of histone H3 at lysine 9. We report here that ESET protein is transiently upregulated in prehypertrophic chondrocytes in newborn mice. To investigate the in vivo effects of ESET on chondrocyte differentiation, we generated conditional knockout mice to specifically eliminate the catalytic SET domain of ESET protein only in mesenchymal cells. Such deletion of the ESET gene caused acceleration of chondrocyte hypertrophy in both embryos and young animals, depleting chondrocytes that are otherwise available to form epiphyseal plates for endochondral bone growth. ESET-deficient mice are thus characterized by defective long bone growth and trabecular bone formation. To understand the underlying mechanism for ESET regulation of chondrocytes, we carried out co-expression experiments and found that ESET associates with histone deacetylase 4 to bind and inhibit the activity of Runx2, a hypertrophy-promoting transcription factor. Repression of Runx2-mediated gene transactivation by ESET is dependent on its H3-K9 methyltransferase activity as well as its associated histone deacetylase activity. In addition, knockout of ESET is associated with repression of Indian hedgehog gene in pre- and early hypertrophic chondrocytes. Together, these results provide clear evidence that ESET controls hypertrophic differentiation of growth plate chondrocytes and endochondral ossification during embryogenesis and postnatal development.

  18. Experiment K-6-06. Morphometric and EM analyses of tibial epiphyseal plates from Cosmos 1887 rats

    NASA Technical Reports Server (NTRS)

    Duke, P. J.; Montufar-Solis, D.; Durnova, G.

    1990-01-01

    Light and electron microscopy studies were carried out on decalcified tibial epiphyseal plates of rats flown aboard Cosmos 1887 (12.5d flight plus 53.5h recovery). Analysis of variance showed that the proliferative zone of flight animals was significantly higher than that of synchronous controls, while the hypertrophic/calcification zone was significantly reduced. Flight animals had more cells than synchronous controls in the proliferative zone, and less in the hypertrophic/calcification region. The total number of cells, however, was significantly higher in flight animals. No differences were found for perimeter or shape factor of growth plates, but area was significantly lower in flight animals in comparison to synchronous controls. Collagen fibrils in flight animals were shorter and wider than in synchronous controls. The time required for a cell to cycle through the growth plate is 2 to 3 days, so most of the cells and matrix present were formed after the animals had returned to 1 g, and probably represent stages of recovery from microgravity exposure, which in itself is an interesting question.

  19. The matrix components of the epiphyseal growth plate and articular cartilages from dogs treated with ammonium tetrathiomolybdate, a copper antagonist.

    PubMed

    Read, R; Sutherland, J; Ghosh, P

    1986-12-01

    As part of a project to study the effect of copper deficiency (CD) on bone development in young dogs, the composition and metabolism of proteoglycans (PGs) and extractability of collagens in the epiphyseal growth plate cartilage (EGPC) and articular cartilages (AC) were investigated. Copper deficiency was induced by feeding ammonium tetrathiomolybdate (TTM) a copper antagonist. The collagen of cartilages from TTM-treated animals was significantly more soluble in 0.5 saline than control tissues. While no distinction between TTM-treated and control cartilages was evident in terms of PG content or extractability under associative (0.5 M-GuHCl) or dissociative (4.0 M-GuHCl) conditions, the sedimentation behaviour of the PG aggregates following CsCl density gradient ultracentrifugation suggested less polydispersity of PGs in preparations from the TTM-treated animals. Moreover, analysis of the PG monomers from EGPC of TTM animals showed galactosamine/glucosamine ratios higher than control preparations, suggesting a reduced keratan sulphate content in these preparations. Organ culture of EGPC showed a significant reduction in the incorporation of 35S into PGs and of 3H-thymidine into DNA in the tissues of TTM-treated animals relative to controls. From these findings we deduce that the catabolism of PGs and the extent of collagen cross-linking in EGPC of TTM-treated animals may be reduced relative to age-matched control tissues.

  20. Precise localization of multiple epiphyseal dysplasia and pseudoachondroplasia mutations by genetic and physical mapping of chromosome 19

    SciTech Connect

    Knowlton, R.G.; Cekleniak, J.A.; Cohn, D.H.

    1994-09-01

    Multiple epiphyseal dysplasia (EDM1), a dominantly inherited chondrodysplasia resulting in peripheral joint deformities and premature osteoarthritis, and pseudoachondroplasia (PSACH), a more severe disorder associated with short-limbed dwarfism, have recently been mapped to the pericentromeric region of chromosome 19. Chondrocytes from some PSACH patients accumulate lamellar deposits in the endoplasmic reticulum that are immunologically cross-reactive with aggrecan. However, neither aggrecan nor any known candidate gene maps to the EDM1/PSACH region of chromosome 19. Genetic linkage mapping in two lage families had placed the disease locus between D19S215 (19p12) and D19S212 (19p13.1), an interval of about 3.5 Mb. With at least five potentially informative cross-overs within this interval, recombination mapping at greater resolution was undertaken. From cosmids assigned to the region by fluorescence in situ hybridization and contig assembly, dinucleotide repeat tracts were identified for use as polymorphic genetic markers. Linkage data from three new dinucleotide repeat markers from cosmids mapped between D19S212 and D19S215 limit the EDM1/PSACH locus to an interval spanning approximately 2 Mb.

  1. Short-term zinc deficiency inhibits chondrocyte proliferation and induces cell apoptosis in the epiphyseal growth plate of young chickens.

    PubMed

    Wang, Xibin; Fosmire, Gary J; Gay, Carol V; Leach, Roland M

    2002-04-01

    The purpose of this study was to investigate the effect of zinc deficiency on chondrocyte proliferation, differentiation and apoptosis in the epiphyseal growth plate of juvenile chickens. Newly hatched broiler chickens were fed either a low zinc (10 mg/kg diet) or a zinc-adequate (68 mg/kg diet) soy protein-based purified diet. Cell proliferation in the growth plate was evaluated with bromodeoxyuridine (BrdU) labeling. Apoptosis was assessed using the terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL) method. Chondrocyte differentiation was evaluated with immunostaining of osteonectin as a marker of maturation. As early as d 3 of feeding, zinc deficiency significantly inhibited chondrocyte proliferation, promoted cell differentiation and induced cell apoptosis in the growth plate. These effects were manifested primarily in areas remote from the blood supply. Immunostaining for local growth factors such as insulin-like growth factor-1 (IGF-1), parathyroid hormone-related protein (PTHrP) and fibroblast growth factor-2 (FGF-2) did not reveal any differences between growth plates of zinc-deficient and zinc-adequate chickens after 3 d of feeding. By d 7, severe growth plate lesions characterized by reduced cellularity and abnormally shaped cells were formed in areas remote from blood vessels. Immunoreactive IGF-1, PTHrP and FGF-2 were all greatly reduced in the lesion. However, the growth rate and food intake of zinc-deficient chickens were not different from those of the controls during the 7-d experiment. Therefore, a direct effect of zinc deficiency on proliferation, differentiation, and apoptosis of growth plate chondrocytes was indicated.

  2. Epiphyseal abnormalities, trabecular bone loss and articular chondrocyte hypertrophy develop in the long bones of postnatal Ext1-deficient mice.

    PubMed

    Sgariglia, Federica; Candela, Maria Elena; Huegel, Julianne; Jacenko, Olena; Koyama, Eiki; Yamaguchi, Yu; Pacifici, Maurizio; Enomoto-Iwamoto, Motomi

    2013-11-01

    Long bones are integral components of the limb skeleton. Recent studies have indicated that embryonic long bone development is altered by mutations in Ext genes and consequent heparan sulfate (HS) deficiency, possibly due to changes in activity and distribution of HS-binding/growth plate-associated signaling proteins. Here we asked whether Ext function is continuously required after birth to sustain growth plate function and long bone growth and organization. Compound transgenic Ext1(f/f);Col2CreERT mice were injected with tamoxifen at postnatal day 5 (P5) to ablate Ext1 in cartilage and monitored over time. The Ext1-deficient mice exhibited growth retardation already by 2weeks post-injection, as did their long bones. Mutant growth plates displayed a severe disorganization of chondrocyte columnar organization, a shortened hypertrophic zone with low expression of collagen X and MMP-13, and reduced primary spongiosa accompanied, however, by increased numbers of TRAP-positive osteoclasts at the chondro-osseous border. The mutant epiphyses were abnormal as well. Formation of a secondary ossification center was significantly delayed but interestingly, hypertrophic-like chondrocytes emerged within articular cartilage, similar to those often seen in osteoarthritic joints. Indeed, the cells displayed a large size and round shape, expressed collagen X and MMP-13 and were surrounded by an abundant Perlecan-rich pericellular matrix not seen in control articular chondrocytes. In addition, ectopic cartilaginous outgrowths developed on the lateral side of mutant growth plates over time that resembled exostotic characteristic of children with Hereditary Multiple Exostoses, a syndrome caused by Ext mutations and HS deficiency. In sum, the data do show that Ext1 is continuously required for postnatal growth and organization of long bones as well as their adjacent joints. Ext1 deficiency elicits defects that can occur in human skeletal conditions including trabecular bone loss

  3. Evidence that estrogen hastens epiphyseal fusion and cessation of longitudinal bone growth by irreversibly depleting the number of resting zone progenitor cells in female rabbits.

    PubMed

    Nilsson, Ola; Weise, Martina; Landman, Ellie B M; Meyers, Jodi L; Barnes, Kevin M; Baron, Jeffrey

    2014-08-01

    With age, growth plate cartilage undergoes programmed senescence, eventually causing cessation of bone elongation and epiphyseal fusion. Estrogen accelerates this developmental process. We hypothesized that senescence occurs because progenitor cells in the resting zone are depleted in number and that estrogen acts by accelerating this depletion. To test this hypothesis, juvenile ovariectomized rabbits received injections of estradiol cypionate or vehicle for 5 weeks, and then were left untreated for an additional 5 weeks. Exposure to estrogen accelerated the normal decline in growth plate height and in the number of proliferative and hypertrophic chondrocytes. Five weeks after discontinuation of estrogen treatment, these structural parameters remained advanced, indicating an irreversible advancement in structural senescence. Similarly, transient estrogen exposure hastened epiphyseal fusion. Estrogen also caused a more rapid decline in functional parameters of growth plate senescence, including growth rate, proliferation rate, and hypertrophic cell size. However, in contrast to the structural parameters, once the estrogen treatment was discontinued, the growth rate, chondrocyte proliferation rate, and hypertrophic cell size all normalized, suggesting that estrogen has a reversible, suppressive effect on growth plate function. In addition, estrogen accelerated the normal loss of resting zone chondrocytes with age. This decrease in resting zone cell number did not appear to be due to apoptosis. However, it was maintained after the estrogen treatment stopped, suggesting that it represents irreversible depletion. The findings are consistent with the hypothesis that estrogen causes irreversible depletion of progenitor cells in the resting zone, thus irreversibly accelerating structural senescence and hastening epiphyseal fusion. In addition, estrogen reversibly suppresses growth plate function.

  4. Proteoglycans from bovine fetal epiphyseal cartilage. Sedimentation velocity and light scattering studies of the effect of link protein on proteoglycan aggregate size and stability.

    PubMed

    Tang, L H; Rosenberg, L C; Reihanian, H; Jamieson, A M; Blackwell, J

    1989-01-01

    Proteoglycan monomer and link proteins were isolated from bovine fetal epiphyseal cartilage and characterized. The physical characteristics of proteoglycan monomer were: s0(20) = 21.3 S, D0t,z = 4.25 x 10(-8)cm2/sec, Mw = 3 x 10(6) and Rg,z = 980A. Link protein preparations contained link proteins 1 and 2, but little or none of the fragment, link protein 3. Link protein-stabilized and link protein-free proteoglycan aggregates were reassembled from proteoglycan monomer, link protein and hyaluronate. The effect of epiphyseal cartilage link protein on aggregate size and stability was examined in sedimentation velocity studies. Compared with link protein from mature bovine nasal and articular cartilages, which contain appreciable amounts of link protein 3, epiphyseal cartilage link protein dramatically stabilized aggregates at pH 5. In the presence of link protein, 92% of the proteoglycan monomers were bound as aggregate at pH 7, and 81% were bound at pH 5. In the absence of link protein, 51% of monomers were bound at pH 7, and only 32% were bound at pH 5. The progressive dissociation of link protein-free aggregates as a function of decreasing pH, and of increasing temperature, was also examined in dynamic light scattering studies. The results of the light scattering studies were in perfect accord with the results of the sedimentation velocity studies. However, compared with the sedimentation velocity studies, the dynamic light scattering studies provided a more detailed and informative description of the dissociation of the link-free aggregate as a function of pH, as a function of temperature, and of the capacity of link protein to stabilize aggregate against dissociation at decreased pH or elevated temperature.

  5. Decreased chondrocyte proliferation and dysregulated apoptosis in the cartilage growth plate are key features of a murine model of epiphyseal dysplasia caused by a matn3 mutation.

    PubMed

    Leighton, Matthew P; Nundlall, Seema; Starborg, Tobias; Meadows, Roger S; Suleman, Farhana; Knowles, Lynette; Wagener, Raimund; Thornton, David J; Kadler, Karl E; Boot-Handford, Raymond P; Briggs, Michael D

    2007-07-15

    Disruption to endochondral ossification leads to delayed and irregular bone formation and can result in a heterogeneous group of genetic disorders known as the chondrodysplasias. One such disorder, multiple epiphyseal dysplasia (MED), is characterized by mild dwarfism and early-onset osteoarthritis and can result from mutations in the gene encoding matrilin-3 (MATN3). To determine the disease mechanisms that underpin the pathophysiology of MED we generated a murine model of epiphyseal dysplasia by knocking-in a matn3 mutation. Mice that are homozygous for the mutation develop a progressive dysplasia and have short-limbed dwarfism that is consistent in severity with the relevant human phenotype. Mutant matrilin-3 is retained within the rough endoplasmic reticulum of chondrocytes and is associated with an unfolded protein response. Eventually, there is reduced proliferation and spatially dysregulated apoptosis of chondrocytes in the cartilage growth plate, which is likely to be the cause of disrupted linear bone growth and the resulting short-limbed dwarfism in the mutant mice.

  6. Vascularisation and osteochondrosis of the epiphyseal growth cartilage of the distal femur in pigs--development with age, growth rate, weight and joint shape.

    PubMed

    Ytrehus, B; Carlson, C S; Lundeheim, N; Mathisen, L; Reinholt, F P; Teige, J; Ekman, S

    2004-03-01

    Until recently, the cartilage canals of the epiphyseal growth cartilage have not been associated with any specific disease. However, data support the hypothesis that osteochondrosis could be related to inadequate blood supply from vessels in these channels. We have done a study to investigate the relationship between the regression of cartilage canals and the formation of osteochondrosis latens in the epiphyseal growth cartilage of the distal femur in pigs, and the relationship between these events and age, growth rate, weight and femoral shape of the individual animals. This involved, in part, a comprehensive study of the distribution and pattern of regression of the cartilage canals. We found that the regression is a highly predictable process that follows an age-dependent pattern. However, we failed to prove any association between overall vascular regression and osteochondrosis, between vascular regression and weight, growth rate or femoral shape or between osteochondrosis and weight, growth rate or femoral shape. This may indicate that osteochondrosis latens is not caused by a general failure of vascular supply or general factors such as growth rate, but rather a consequence of local conditions affecting a limited number of vessels. A factor fitting this description is local compression.

  7. Premature Epiphyseal Closure of the Lower Extremities Contributing to Short Stature after cis-Retinoic Acid Therapy in Medulloblastoma: A Case Report

    PubMed Central

    Noyes, Jessica J.; Levine, Michael A.; Belasco, Jean B.; Mostoufi-Moab, Sogol

    2015-01-01

    Background Prolonged cis-Retinoic Acid (cis-RA) exposure contributes to premature epiphyseal closure. Cis-RA is administered in various treatment regimens for pediatric cancers, thus increasing the risk for bone deformities and compromised growth. Results We present a case of premature epiphyseal closure in a 9 year-old female with history of medulloblastoma and treatment with a multimodal regimen including cis-RA. She was subsequently diagnosed with radiation-induced endocrine late effects including hypothyroidism and growth hormone deficiency (GHD). Seven months after initiation of GH, increased prominence of wrists and knees combined with deceleration in growth velocity prompted further evaluation; radiographs revealed bilateral premature closure of distal femur and proximal tibia growth plates despite normal left wrist bone age. Conclusion High doses of vitamin A and its analogs are linked to premature closure of lower extremity growth plates in animals and children. Pediatric brain tumor patients are at increased risk of growth failure due to concurrent radiation-induced GHD, damage to spinal bones, and cis-RA associated premature closure of lower extremity growth plates, with significant reduction in adult stature. A better appreciation of the detrimental effect of cis-RA on the growing skeleton is needed to monitor at-risk patients and to provide timely interventions. PMID:26457578

  8. Spondylo-meta-epiphyseal dysplasia (SMED), short limb-hand type: a congenital familial skeletal dysplasia with distinctive features and histopathology.

    PubMed

    Borochowitz, Z; Langer, L O; Gruber, H E; Lachman, R; Katznelson, M B; Rimoin, D L

    1993-02-01

    We report on a "new" severe short-limb bone dysplasia which can be labeled descriptively a spondylo-meta-epiphyseal dysplasia. The 3 patients were born to 2 unrelated Sepharadic Jewish families and a Puerto Rican family. Clinical abnormalities include small stature with short limbs including short hands, a short nose with wide nasal bridge and wide nostrils, a long philtrum, ocular hypertelorism, retro/micrognathia, and a narrow chest. Radiological abnormalities include platyspondyly, short tubular bones with very abnormal metaphyses and epiphyses beyond early infancy, short ribs, and a typical evolution of bony changes over time. Chondroosseous morphology and ultrastructure document sparse matrix and degenerating chondrocytes surrounded by dense amorphous material in the 1 patient studied. Consanguinity is present in 1 family. In addition to the described patient, 2 other short-limb sibs, who did not survive infancy, were born into this family. Even in the absence of any photographic or radiologic documentation of these other 2 infants, autosomal recessive mode of inheritance seems probable.

  9. Novel DDR2 mutation identified by whole exome sequencing in a Moroccan patient with spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type.

    PubMed

    Mansouri, Maria; Kayserili, Hülya; Elalaoui, Siham Chafai; Nishimura, Gen; Iida, Aritoshi; Lyahyai, Jaber; Miyake, Noriko; Matsumoto, Naomichi; Sefiani, Abdelaziz; Ikegawa, Shiro

    2016-02-01

    Spondylo-meta-epiphyseal dysplasia (SMED), short limb-abnormal calcification type (SMED, SL-AC), is a very rare autosomal recessive disorder with various skeletal changes characterized by premature calcification leading to severe disproportionate short stature. Twenty-two patients have been reported until now, but only five mutations (four missense and one splice-site) in the conserved sequence encoding the tyrosine kinase domain of the DDR2 gene has been identified. We report here a novel DDR2 missense mutation, c.370C > T (p.Arg124Trp) in a Moroccan girl with SMED, SL-AC, identified by whole exome sequencing. Our study has expanded the mutational spectrum of this rare disease and it has shown that exome sequencing is a powerful and cost-effective tool for the diagnosis of clinically heterogeneous disorders such as SMED.

  10. 32Pi- and 45Ca-metabolism by matrix vesicle-enriched microsomes prepared from chicken epiphyseal cartilage by isosmotic Percoll density-gradient fractionation.

    PubMed

    Warner, G P; Hubbard, H L; Lloyd, G C; Wuthier, R E

    1983-05-01

    Matrix vesicle-enriched fractions were isolated from different zones of epiphyseal cartilage by nonenzymatic methods involving tissue homogenization, differential centrifugation, and isosmotic Percoll gradient fractionation. Uptakes of both 32Pi and 45Ca were studied concomitantly over periods from 20 min to 24 h. Percoll density gradients separated epiphyseal microsomes into two alkaline phosphatase-rich fractions: a low-density noncalcifiable fraction (P-I), and a higher-density fraction (P-II) which readily mineralized. The P-II fraction was found only in calcifying regions of the growth plate. Based on chemical and physical properties and enzyme activities, both fractions were similar except that P-II contained significantly higher levels of mineral ions than did P-I, and had lower levels of alkaline phosphatase. The mineral appeared to be primarily in a noncrystalline form. Metabolism of 32Pi and 45Ca by P-II followed a complex kinetic pattern in which accumulation of large amounts of both ions was preceded by an initial limited burst of uptake and a lag-phase of variable duration. During mineral ion loading, the density of the P-II fraction progressively increased as evidenced by co-migration of 45Ca, 32Pi, and alkaline phosphatase to increasingly higher densities. During the period of early mineral deposition (1-5 h), Ca/P uptake ratios were very low (1.0-1.2) and X-ray diffraction patterns showed a predominantly amorphous pattern. This suggests that the mineral accumulated in matrix vesicles is initially some form of noncrystalline calcium monohydrogenphosphate. L-tetramisole, a potent inhibitor of alkaline phosphatase, inhibited accumulation of both 45Ca and 32Pi in the absence of organic P substrates, 32Pi being preferentially inhibited over 45Ca. This finding, coupled with recent studies on the behavior of alkaline phosphatase at physiological pH, suggests that the protein is not acting as a phosphohydrolase, but rather as a Pi-binding or transport agent

  11. Visualization of sulfur-containing components associated with proliferating chondrocytes from rat epiphyseal growth plate cartilage: Possible proteoglycan and collagen co-migration

    SciTech Connect

    Landis, W.J.; Hodgens, K.J. )

    1990-02-01

    Electron microscopy of epiphyseal growth plate cartilage from normal 4-5-week-old rats has revealed extensive fibrillar aggregates and globules in the pericellular spaces of proliferating chondrocytes. These cells contained small globules and diffusely coiled, fine filaments located within large, membrane-invested vacuoles. All such structures were observed after a variety of different tissue fixation regimes, including glutaraldehyde, osmium tetroxide, and potassium pyroantimonate. The fibrillar aggregates and globules were often overlapping and intermeshed and extended to 0.5 micron in length from their point of origin at cell membranes. Vacuoles were usually found at the periphery of cells, and some, by membrane fusion with the cell envelope, appeared contiguous with extracellular spaces wherein their contents could be discharged. Fine filaments and globules were occasionally observed in the Golgi complex and cisternae of endoplasmic reticulum of the chondrocytes. Further characterization of the cellular and pericellular components by electron microscopic radioautography, electron probe microanalysis, and electron spectroscopic imaging indicated the presence of sulfur, a result suggesting these aggregates, filaments, and globules in part represent proteoglycans in various stages of synthesis, secretion, and assembly. Additional radioautography utilizing 3H-proline implied that filament bundles are also composed of collagen, a result posing the possibility that this protein and the putative proteoglycans may co-migrate both intracellularly and within pericellular matrices. In extracellular matrices adjacent to cell lacunae, the fibrillar aggregates appeared in close association with typical collagen type II fibrils, an observation providing evidence for proteoglycan-collagen network formation in this region of the rat epiphysis.

  12. A new formula for assessing skeletal age in growing infants and children by measuring carpals and epiphyses of radio and ulna.

    PubMed

    De Luca, Stefano; Mangiulli, Tatiana; Merelli, Vera; Conforti, Federica; Velandia Palacio, Luz Andrea; Agostini, Susanna; Spinas, Enrico; Cameriere, Roberto

    2016-04-01

    The aim of this study is to develop a specific formula for the purpose of assessing skeletal age in a sample of Italian growing infants and children by measuring carpals and epiphyses of radio and ulna. A sample of 332 X-rays of left hand-wrist bones (130 boys and 202 girls), aged between 1 and 16 years, was analyzed retrospectively. Analysis of covariance (ANCOVA) was applied to study how sex affects the growth of the ratio Bo/Ca in the boys and girls groups. The regression model, describing age as a linear function of sex and the Bo/Ca ratio for the new Italian sample, yielded the following formula: Age = -1.7702 + 1.0088 g + 14.8166 (Bo/Ca). This model explained 83.5% of total variance (R(2) = 0.835). The median of the absolute values of residuals (observed age minus predicted age) was -0.38, with a quartile deviation of 2.01 and a standard error of estimate of 1.54. A second sample test of 204 Italian children (108 girls and 96 boys), aged between 1 and 16 years, was used to evaluate the accuracy of the specific regression model. A sample paired t-test was used to analyze the mean differences between the skeletal and chronological age. The mean error for girls is 0.00 and the estimated age is slightly underestimated in boys with a mean error of -0.30 years. The standard deviations are 0.70 years for girls and 0.78 years for boys. The obtained results indicate that there is a high relationship between estimated and chronological ages.

  13. Forensic age estimation via 3-T magnetic resonance imaging of ossification of the proximal tibial and distal femoral epiphyses: Use of a T2-weighted fast spin-echo technique.

    PubMed

    Ekizoglu, Oguzhan; Hocaoglu, Elif; Inci, Ercan; Can, Ismail Ozgur; Aksoy, Sema; Kazimoglu, Cemal

    2016-03-01

    Radiation exposure during forensic age estimation is associated with ethical implications. It is important to prevent repetitive radiation exposure when conducting advanced ultrasonography (USG) and magnetic resonance imaging (MRI). The purpose of this study was to investigate the utility of 3.0-T MRI in determining the degree of ossification of the distal femoral and proximal tibial epiphyses in a group of Turkish population. We retrospectively evaluated coronal T2-weighted and turbo spin-echo sequences taken upon MRI of 503 patients (305 males, 198 females; age 10-30 years) using a five-stage method. Intra- and interobserver variations were very low. (Intraobserver reliability was κ=0.919 for the distal femoral epiphysis and κ=0.961 for the proximal tibial epiphysis, and interobserver reliability was κ=0.836 for the distal femoral epiphysis and κ=0.885 for the proximal tibial epiphysis.) Spearman's rank correlation analysis indicated a significant positive relationship between age and the extent of ossification of the distal femoral and proximal tibial epiphyses (p<0.001). Comparison of male and female data revealed significant between-gender differences in the ages at first attainment of stages 2, 3, and 4 ossifications of the distal femoral epiphysis and stage 1 and 4 ossifications of the proximal tibial epiphysis (p<0.05). The earliest ages at which ossification of stages 3, 4, and 5 was evident in the distal femoral epiphysis were 14, 17, and 22 years in males and 13, 16, and 21 years in females, respectively. Proximal tibial epiphysis of stages 3, 4, and 5 ossification was first noted at ages 14, 17, and 18 years in males and 13, 15, and 16 years in females, respectively. MRI of the distal femoral and proximal tibial epiphyses is an alternative, noninvasive, and reliable technique to estimate age.

  14. Local Morphological Response of the Distal Femoral Articular–Epiphyseal Cartilage Complex of Young Foals to Surgical Stab Incision and Potential Relevance to Cartilage Injury and Repair in Children

    PubMed Central

    Hendrickson, Eli H.S.; Ekman, Stina; Carlson, Cathy S.; Dolvik, Nils I.

    2013-01-01

    Objective: Describe the local morphological response of the articular–epiphyseal cartilage complex to surgical stab incision in the distal femur of foals, with emphasis on the relationship between growth cartilage injury, enchondral ossification, and repair. Design: Nine foals were induced into general anesthesia at the age of 13 to 15 days. Four full-thickness stab incision defects were created in the cartilage on the lateral aspect of the lateral trochlear ridge of the left distal femur. Follow-up examination was carried out from 1 to 49 days postoperatively, including examination of intact bones, sawed slabs, and histological sections. Results: Incision defects filled with cells displaying fibroblast-, chondrocyte-, and osteoblast-like characteristics, potentially validating the rationale behind the drilling of stable juvenile osteochondritis dissecans lesions in children. Incisions induced necrosis within the cartilage on the margins at all depths of the defects. Sharp dissection may therefore be contraindicated in cartilage repair in young individuals. Incisions caused a focal delay in enchondral ossification in 2 foals, apparently related to the orientation of the incision defect relative to the direction of ossification. Defects became progressively surrounded by subchondral bone, in which granulation tissue containing clasts and foci of osteoblast-like cells was observed. Continued enchondral ossification was therefore likely to result in healing of uncomplicated defects to morphologically normal bone. Conclusions: Epiphyseal growth cartilage injury had the potential to exert a negative effect on enchondral ossification. Enchondral ossification exerted a beneficial effect on repair. This relationship warrants consideration in future studies of cartilage injury and repair within the articular–epiphyseal cartilage complex of all species. PMID:26069670

  15. Calmodulin-dependent kinase 1beta is expressed in the epiphyseal growth plate and regulates proliferation of mouse calvarial osteoblasts in vitro.

    PubMed

    Pedersen, Mona E; Fortunati, Dario; Nielsen, Marit; Brorson, Sverre-Henning; Lekva, Tove; Nissen-Meyer, Lise Sofie H; Gautvik, Vigdis T; Shahdadfar, Aboulghassem; Gautvik, Kaare M; Jemtland, Rune

    2008-10-01

    The Ca(2+)/Calmodulin-dependent protein kinase (CaMK) family is activated in response to elevation of intracellular Ca(2+), and includes CaMK1 (as well as CaMK2 and CaMK4), which exists as different isoforms (alpha, beta, gamma and delta). CaMK1 is present in several cell types and may be involved in various cellular processes, but its role in bone is unknown. In situ hybridization was used to determine the spatial and temporal expression of CaMK1beta during endochondral bone development in mouse embryos and newborn pups. The cellular and subcellular distribution of CaMK1 was assessed by quantitative immunogold electron microscopy (EM). The role of CaMK1beta in mouse calvarial osteoblasts was investigated by using small interfering RNA (siRNA) to silence its expression, while in parallel monitoring cell proliferation and levels of skeletogenic transcripts. cRNA in situ hybridization and EM studies show that CaMK1beta is mainly located in developing long bones and vertebrae (from ED14.5 until day 10 after birth), with highest expression in epiphyseal growth plate hypertrophic chondrocytes. By RT-PCR, we show that CaMK1beta2 (but not beta1) is expressed in mouse hind limbs (in vivo) and mouse calvarial osteoblasts (in vitro), and also in primary human articular chondrocyte cultures. Silencing of CaMK1beta in mouse calvarial osteoblasts by siRNA significantly decreases osteoblast proliferation and c-Fos gene expression (approx. 50%), without affecting skeletogenic markers for more differentiated osteoblasts (i.e. Cbfa1/Runx2, Osterix (Osx), Osteocalcin (Oc), Alkaline phosphatase (Alp) and Osteopontin (Opn)). These results identify CaMK1beta as a novel regulator of osteoblast proliferation, via mechanisms that may at least in part involve c-Fos, thus implicating CaMK1beta in the regulation of bone and cartilage development.

  16. Contact Stress and Kinematic Analysis of All-Epiphyseal and Over-the-Top Pediatric Reconstruction Techniques for the Anterior Cruciate Ligament

    PubMed Central

    McCarthy, Moira M.; Tucker, Scott; Nguyen, Joseph T.; Green, Daniel W.; Imhauser, Carl W.; Cordasco, Frank A.

    2014-01-01

    Background Anterior cruciate ligament (ACL) injuries are an increasingly recognized problem in the pediatric population. Unfortunately, outcomes with conservative treatment are extremely poor. Furthermore, adult reconstruction techniques may be inappropriate to treat skeletally immature patients due to the risk of physeal complications. “Physeal-sparing” reconstruction techniques exist but their ability to restore knee stability and contact mechanics is not well understood. Purpose (1) To assess the ability of the all-epiphyseal (AE) and over-the-top (OT) reconstructions to restore knee kinematics; (2) to assess whether these reconstructions decrease the high posterior contact stresses seen with ACL deficiency; (3) to determine whether the AE or OT produce abnormal tibiofemoral contact stresses. Hypothesis The AE reconstruction will restore contact mechanics and kinematics similarly to that of the ACL intact knee. Methods Ten fresh-frozen human cadaveric knees were tested using a robotic manipulator. Tibiofemoral motions were recorded with the ACL intact, after sectioning the ACL, and after both reconstructions in each of the 10 specimens. The AE utilized an all-inside technique with tunnels exclusively within the epiphysis and fixed with suspensory cortical fixation devices. The OT had a central and vertical tibial tunnel with an over-the-top femur position and was fixed with staples and posts on both ends. Anterior stability was assessed with 134N anterior force at 0, 15, 30, 60, and 90° of knee flexion. Rotational stability was assessed with combined 8 Nm and 4 Nm of abduction and internal rotation, respectively, at 5, 15, and 30° of knee flexion. Results Both reconstruction techniques offloaded the posterior aspect of the tibial plateau compared to the ACL deficient knee in response to both anterior loads and combined moments as demonstrated by reduced contact stresses in this region at all flexion angles. Compared to the ACL intact condition, both the AE

  17. Measurements of developing teeth, and carpals and epiphyses of the ulna and radius for assessing new cut-offs at the age thresholds of 10, 11, 12, 13 and 14 years.

    PubMed

    Cameriere, R; De Luca, S; Cingolani, M; Ferrante, L

    2015-08-01

    The minimum age of criminal responsibility is the youngest age at which children may be held liable for infringements of penal laws. New cut-offs at the age thresholds of 10, 11, 12, 13 and 14 years were determined by applying three different methods: measurement of open apices in tooth roots (T); the ratio between the total area of carpal bones and epiphyses of the ulna and radius (HW); and the combined method (THW). The sample consisted of 291 Italian children (152 boys, 139 girls), aged between 5 and 15 years. The sensitivity and specificity were established. As regards THW, specificity reached the maximum of 95% in boys aged 10, and the minimum of 87% in boys aged 11. The best score of the Positive Predictive Value (PPV) was obtained in boys at 10 years with the THW method and the worst in girls of 12 with the HW method.

  18. Growth sensitivity in the epiphyseal growth plate, liver and muscle of SD rats is significantly enhanced by treatment with a fermented soybean product (cheonggukjang) through stimulation of growth hormone secretion.

    PubMed

    Hwang, In Sik; Kim, Ji Eun; Lee, Young Ju; Kwak, Mun Hwa; Lee, Hong Gu; Kim, Hye Sung; Lee, Hee Seob; Hwang, Dae Youn

    2014-01-01

    Cheonggukjang (CKJ), a fermented soybean product, has been reported to have beneficial effects on various chronic diseases, including cardiovascular disease, cancer and immune diseases. To investigate whether CKJ induces growth sensitivity in mammals, alterations of key parameters related to their growth were analyzed. Sprague‑Dawley (SD) rats were treated with a high concentration of CKJ (H‑CKJ) or a low concentration of CKJ (L‑CKJ) for 10 days, and compared with vehicle-treated rats. The CKJ contained a high concentration of total flavonoids, phenolic compounds, daidzein and genistein, compared with the non-fermented soybean product. Body weight was higher in the H‑CKJ‑treated group compared with that in the vehicle‑ and L‑CKJ‑treated groups, whereas the weights of three organs (the brain, liver and kidney) were higher in the L‑CKJ‑treated group compared with the remaining two groups. However, no significant differences in femur length and weight were detected between the CKJ‑ and vehicle‑treated groups. The thickness of the epiphyseal growth plate in proximal femoral epiphysis was broadest in the H‑CKJ‑treated group compared with the vehicle- and L‑CKJ‑treated groups. Furthermore, the level of growth hormone (GH) was highest in the serum of the L‑CKJ‑treated group, although that of the H‑CKJ‑treated group was lower compared with that in the L‑CKJ group. Moreover, the expression levels of the GH receptor increased in the liver tissue, but not in the muscle tissue, of the L‑CKJ‑ and H‑CKJ‑treated groups. In the downstream signaling pathway of the GH receptor, the phosphorylation levels of Akt and Erk were differentially regulated between the liver and muscle. These results suggest that CKJ extract may enhance the sensitivity of the femur, liver and muscle epiphyseal growth plate in SD rats, through the upregulation of GH secretion.

  19. Transphyseal osseous bridges in experimental osteonecrosis of the femoral head of the rat. Histologic study of the bony bridges connecting the epiphyseal with the metaphyseal bony trabeculae through gaps in the physeal cartilage.

    PubMed

    Peskin, B; Shupak, A; Misselevich, I; Zinman, C; Levin, D; Jacob, Z; Reis, D N; Boss, J H

    2001-07-01

    In view of the lifelong persistence of the physis, the femoral head of rats may serve to model Perthes disease and slipped capital femoral epiphysis. To produce osteonecrosis, the blood supply of one femoral head of 133, 6-month-old animals was severed by circumferentially incising the periosteum of the neck and cutting the ligamentum teres. The rats were killed 7 days to 90 days postoperatively. Associated with resorption of the necrotic bone and marrow, remodeling of the epiphysis was characterized by an ingrowth of vascularized fibrous tissue, formation of new bone and some cartilage, architectural deformation and flattening of the head. In 22 of 83 rats killed 30 days or more postoperatively, gaps in the continuity of the physeal cartilage were occupied by osseous bridges, connecting newly formed epiphyseal bony trabeculae with either the preexisting or newly formed metaphyseal osseous trabeculae. This healing mode may follow ischemic death of physeal chondrocytes or be owing to another mechanism, e.g., release of mediatory substances of inflammation. These findings raise the possibility that fixation of the healing epiphysis of a child's previously necrotic femoral head to the metaphysis occurs by transphyseal osseous growth in cases in which the physis is involved in the necrotic process.

  20. Hypoxia-inducible factor-1 (HIF-1) but not HIF-2 is essential for hypoxic induction of collagen prolyl 4-hydroxylases in primary newborn mouse epiphyseal growth plate chondrocytes.

    PubMed

    Aro, Ellinoora; Khatri, Richa; Gerard-O'Riley, Rita; Mangiavini, Laura; Myllyharju, Johanna; Schipani, Ernestina

    2012-10-26

    Hypoxia-inducible factors (HIFs) are the master regulators of hypoxia-responsive genes. They play a critical role in the survival, development, and differentiation of chondrocytes in the avascular hypoxic fetal growth plate, which is rich in extracellular matrix (ECM) and in its main component, collagens. Several genes involved in the synthesis, maintenance, and degradation of ECM are regulated by HIFs. Collagen prolyl 4-hydroxylases (C-P4Hs) are key enzymes in collagen synthesis because the resulting 4-hydroxyprolines are necessary for the stability of all collagen molecules. The vertebrate C-P4Hs are α(2)β(2) tetramers with three isoforms of the catalytic α subunit, yielding C-P4Hs of types I-III. C-P4H-I is the main form in most cells, but C-P4H-II is the major form in chondrocytes. We postulated here that post-translational modification of collagens, particularly 4-hydroxylation of proline residues, could be one of the modalities by which HIF regulates the adaptive responses of chondrocytes in fetal growth plates. To address this hypothesis, we used primary epiphyseal growth plate chondrocytes isolated from newborn mice with conditionally inactivated genes for HIF-1α, HIF-2α, or the von Hippel-Lindau protein. The data obtained showed that C-P4H α(I) and α(II) mRNA levels were increased in hypoxic chondrocytes in a manner dependent on HIF-1 but not on HIF-2. Furthermore, the increases in the C-P4H mRNA levels were associated with both increased amounts of the C-P4H tetramers and augmented C-P4H activity in hypoxia. The hypoxia inducibility of the C-P4H isoenzymes is thus likely to ensure sufficient C-P4H activity for collagen synthesis occurring in chondrocytes in a hypoxic environment.

  1. The epiphyseal plate: physiology, anatomy, and trauma.

    PubMed

    von Pfeil, Dirsko J F; DeCamp, Charles E

    2009-08-01

    This article reviews the development of long bones, the microanatomy and physiology of the growth plate, the closure times and contribution of different growth plates to overall growth, and the effect of, and prognosis for, traumatic injuries to the growth plate. Details on surgical treatment of growth plate fractures are beyond the scope of this article.

  2. Spaceflight and age affect tibial epiphyseal growth plate histomorphometry

    NASA Technical Reports Server (NTRS)

    Montufar-Solis, Dina; Duke, Pauline J.; Durnova, G.

    1992-01-01

    Growth plate histomorphometry of rats flown aboard the Soviet biosatellite Cosmos 2044, a 14-day spaceflight, was compared with that of control groups. In growth plates of flight animals, there was a significant increase in cell number per column and height of the proliferative zone and a reduction in height and cell number in the hypertrophy/calcification zone. No significant differences were found in matrix organization at the ultrastructural level of flight animals, indicating that although spacefligfht continues to affect bone growth of 15-wk-old rats, extracellular matrix is not altered in the same manner as seen previously in younger animals. All groups showed growth plate characteristics attributed to aging: lack of calcification zone, reduced hypertrophy zone, and unraveling of collagen fibrils. Tail-suspended controls did not differ from other controls in any of the parameters measured. The results suggest that growth plates of older rats are less responsive to unloading by spaceflight or suspension than those of younger rats and provide new evidence about the modifying effect of spaceflight on the growth plate.

  3. Primary epiphyseal arteriopathy in a mouse model of steroid-induced osteonecrosis.

    PubMed

    Janke, Laura J; Liu, Chengcheng; Vogel, Peter; Kawedia, Jitesh; Boyd, Kelli L; Funk, Amy J; Relling, Mary V

    2013-07-01

    Patients undergoing glucocorticoid therapy for a variety of disorders, including autoimmune diseases and hematological malignancies, are at risk of developing osteonecrosis. Despite extensive research in both patients and animal models, the underlying pathogenesis remains unclear. Proposed inciting mechanisms include intravascular thrombotic occlusion, marrow fat hypertrophy, osteocyte and/or endothelial cell apoptosis, hypercoagulability, and vasoconstriction of specific arteries and arterioles supplying bone. Our laboratory has developed a model of steroid-induced osteonecrosis in BALBcJ mice which reflects clinically relevant exposures to glucocorticoids in which treated mice develop osteonecrosis of the distal femoral epiphysis when administered 4 to 8 mg/L dexamethasone in drinking water for 6 weeks. We identified lesions in arterioles supplying this area, with the mildest occurring in knees without any evidence of osteonecrosis. However, arteriopathy was more common among mice that did versus did not develop osteonecrosis (P < 0.0001); in mice with osteonecrosis, the associated vessels showed transmural necrosis and thickening of the vessel wall progressing to the point of luminal obstruction. In the most severe cases of osteonecrosis, end-stage lesions consisted of fully occluded vessels with marrow and bone necrosis involving the entire epiphysis. We propose that a primary arteriopathy is the initiating event in the genesis of steroid-induced osteonecrosis and provides a basis for future investigation of this disease process.

  4. Histomorphometric and electron microscopic analyses of tibial epiphyseal plates from Cosmos 1887 rats

    NASA Technical Reports Server (NTRS)

    Duke, P. J.; Durnova, G.; Montufar-Solis, D.

    1990-01-01

    Previous studies have shown that the changes seen in the bones of growing rats exposed to microgravity are due in part to changes that occur in the growth plate during spaceflight. In this study, growth plates of rats flown aboard Cosmos 1887 (12.5-day flight plus 53.5-h recovery at 1 g) were analyzed using light and electron microscopy and computerized planimetry. The proliferative zone of flight animals was found to be significantly (P less than or equal to 0.01) larger than that of controls, while the reserve and hypertrophic/calcification zones were significantly reduced. Flight animals also had more cells per column in the proliferative zone than did controls and less in the hypertrophic/calcification region. The total number of cells, however, was significantly greater in flight animals. No difference was found in perimeter or in shape factor, but area was significantly less in flight animals. Electron microscopy showed that collagen fibrils in flight animals were wider than in controls. Since the time required for a cell to cycle through the growth plate is 2-3 days at 1 g, the results reported here represent both the effects of exposure to microgravity and the initial stages of recovery from that exposure.

  5. Overuse epiphyseal injury of the coracoid process as a result of archery.

    PubMed

    Naraen, A; Giannikas, K A; Livesley, P J

    1999-01-01

    A previously unreported overuse injury of the coracoid process in an eleven years old archer is presented. Diagnosis was made on clinical grounds in association with the radiographic appearance of the unfused physis. The pathology and the management are presented and discussed.

  6. Patterns of proteoglycan degradation by a neutral protease from human growth-plate epiphyseal cartilage

    SciTech Connect

    Ehrlich, M.G.; Armstrong, A.L.; Neuman, R.G.; Davis, M.W.; Mankin, H.J.

    1982-12-01

    The hypothesis is widely held that proteolytic degradation of proteoglycans in the lower hypertrophic zone of the growth plate may be involved in the initiation of mineralization in the zone of provisional calcification. However, a neutral protease that is responsible for the degradation of proteoglycans in the growth plate has not been identified, isolated, and characterized. In the work reported here, neutral protease activity in the growth plate is demonstrated for the first time, and some of the properties of the enzyme are described. Proteoglycans subunits were prepared from bovine nasal cartilage and calf costal cartilage by equilibrium density-gradient centrifugation under dissociative conditions. The proteoglycan subunits were labeled with /sup 14/C-formaldehyde. Homogenates from human growth plates were examined for neutral protease activity using the proteoglycan subunits as substrates. Following incubation of the proteoglycan subunits with growth-plate homogenates at pH 5.3 and at pH 7.5 in the presence and absence of ten-millimolar magnesium chloride and calcium chloride, the digestion products were examined by gel chromatography on Sepharose-2B and 6B columns. Column eluants containing proteoglycan-subunit degradation products were monitored for uronic acid, hexose, and radio-activity. Maximum extensive degradation of proteoglycan subunits occurred at pH 7.5 in the presence of ten-millimolar magnesium chloride and calcium chloride.

  7. The locomotion of Babakotia radofilai inferred from epiphyseal and diaphyseal morphology of the humerus and femur.

    PubMed

    Marchi, Damiano; Ruff, Christopher B; Capobianco, Alessio; Rafferty, Katherine L; Habib, Michael B; Patel, Biren A

    2016-09-01

    Palaeopropithecids, or "sloth lemurs," are a diverse clade of large-bodied Malagasy subfossil primates characterized by their inferred suspensory positional behavior. The most recently discovered genus of the palaeopropithecids is Babakotia, and it has been described as more arboreal than Mesopropithecus, but less than Palaeopropithecus. In this article, the within-bone and between-bones articular and cross-sectional diaphyseal proportions of the humerus and femur of Babakotia were compared to extant lemurs, Mesopropithecus and Palaeopropithecus in order to further understand its arboreal adaptations. Additionally, a sample of apes and sloths (Choloepus and Bradypus) are included as functional outgroups composed of suspensory adapted primates and non-primates. Results show that Babakotia and Mesopropithecus both have high humeral/femoral shaft strength proportions, similar to extant great apes and sloths and indicative of forelimb suspensory behavior, with Babakotia more extreme in this regard. All three subfossil taxa have relatively large femoral heads, also associated with suspension in modern taxa. However, Babakotia and Mesopropithecus (but not Palaeopropithecus) have relatively small femoral head surface area to shaft strength proportions suggesting that hind-limb positioning in these taxa during climbing and other behaviors was different than in extant great apes, involving less mobility. Knee and humeral articular dimensions relative to shaft strengths are small in Babakotia and Mesopropithecus, similar to those found in modern sloths and divergent from those in extant great apes and lemurs, suggesting more sloth-like use of these joints during locomotion. Mesopropithecus and Babakotia are more similar to Choloepus in humerofemoral head and length proportions while Palaeopropithecus is more similar to Bradypus. These results provide further evidence of the suspensory adaptations of Babakotia and further highlight similarities to both extant suspensory primates and non-primate slow arboreal climbers and hangers. J. Morphol. 277:1199-1218, 2016. © 2016 Wiley Periodicals, Inc.

  8. Thompson and Hamilton type IV Freiberg's disease with involvement of multiple epiphyses of both feet.

    PubMed

    Lui, Tun Hing

    2015-02-26

    A 17-year-old boy reported left second and third toe pain after axial loading injury to his left foot. Radiographs showed collapse of the second metatarsal heads and epiphysial irregularities of the fifth metatarsal heads and the condyle of the proximal phalanx of the hallux of both feet. The patient was diagnosed to have Thompson and Hamilton type IV Freiberg's disease. He was screened for epiphysial dysplasia of the other sites. He had on and off bilateral hip and knee pain. Radiographs showed bilateral symmetrical epiphysial abnormalities with morphological change as focal concavity in bilateral femoral heads and fragmentation of the patellar articular surface with preservation of the patellofemoral joint space.

  9. The therapeutic effect of bone marrow-derived stem cell implantation after epiphyseal plate injury is abrogated by chondrogenic predifferentiation.

    PubMed

    Coleman, Rhima M; Schwartz, Zvi; Boyan, Barbara D; Guldberg, Robert E

    2013-02-01

    The goal of this study was to determine the effects of chondrogenic predifferentiation on the ability of bone marrow-derived stromal cells (BMSCs) delivered to growth plate defects to restore growth function. Chondrogenesis was induced with transforming growth factor (TGF)-β1 treatment in high-density monolayer cultures of BMSCs in vitro. The predifferentiated or undifferentiated BMSCs were either seeded into agarose gels for continued in vitro culture, or injected into growth plate defects via an in situ gelling agarose. Predifferentiated BMSCs had higher Sox-9, type II collagen, and aggrecan mRNA levels compared to undifferentiated cells after high-density monolayer culture. After transfer to agarose gels, predifferentiated cells did not produce a cartilaginous matrix, even with continued TGF-β1 stimulation, whereas undifferentiated cells produced a cartilaginous matrix in this system. Three-dimensional images of the growth plate created from microcomputed tomography scans showed that delivery of either predifferentiated or undifferentiated cells to defects resulted in a decrease in mineralized tether formation (fusion) in the growth plate tissue surrounding the defect to normal levels. Limb length discrepancy between injured and control limbs was corrected after treatment with undifferentiated, but not predifferentiated, cells. These results indicate that cell therapy may be an effective treatment to reduce growth dysfunction after growth plate injury, perhaps by maintaining the health of the uninjured growth plate tissue, and that the cell differentiation state plays a role in restoring the growth potential of the injured limb.

  10. Epiphyseal growth plate growth hormone receptor signaling is decreased in chronic kidney disease-related growth retardation.

    PubMed

    Troib, Ariel; Landau, Daniel; Kachko, Leonid; Rabkin, Ralph; Segev, Yael

    2013-11-01

    Linear growth retardation in children with chronic kidney disease (CKD) has been ascribed to insensitivity to growth hormone. This resistance state has been attributed to impaired growth hormone signaling through the JAK2/STAT5 pathway in liver and skeletal muscle leading to reduced insulin-like growth factor-I (IGF-I). Here we determine whether systemic and growth plate alterations in growth hormone signaling contribute to CKD-induced linear growth retardation using partially nephrectomized and pair-fed control 20-day-old rats. Serum growth hormone did not change in rats with CKD, yet serum IGF-I levels were decreased and growth retarded. The tibial growth plate hypertrophic zone was wider and vascularization at the primary ossification center was reduced in CKD. This was associated with a decrease in growth plate vascular endothelial growth factor (VEGF) mRNA and immunostainable VEGF and IGF-I levels. Growth plate growth hormone receptor and STAT5 protein levels were unchanged, while JAK2 was reduced. Despite comparable growth hormone and growth hormone receptor levels in CKD and control rats, relative STAT5 phosphorylation was significantly depressed in CKD. Of note, the mRNA of SOCS2, an inhibitor of growth hormone signaling, was increased. Thus, linear growth impairment in CKD can in part be explained by impaired long bone growth plate growth hormone receptor signaling through the JAK2/STAT5 pathway, an abnormality that may be caused by an increase in SOCS2 expression.

  11. Genome-wide screening in human growth plates during puberty in one patient suggests a role for RUNX2 in epiphyseal maturation.

    PubMed

    Emons, Joyce; Dutilh, Bas E; Decker, Eva; Pirzer, Heide; Sticht, Carsten; Gretz, Norbert; Rappold, Gudrun; Cameron, Ewan R; Neil, James C; Stein, Gary S; van Wijnen, Andre J; Wit, Jan Maarten; Post, Janine N; Karperien, Marcel

    2011-05-01

    In late puberty, estrogen decelerates bone growth by stimulating growth plate maturation. In this study, we analyzed the mechanism of estrogen action using two pubertal growth plate specimens of one girl at Tanner stage B2 and Tanner stage B3. Histological analysis showed that progression of puberty coincided with characteristic morphological changes: a decrease in total growth plate height (P=0.002), height of the individual zones (P<0.001), and an increase in intercolumnar space (P<0.001). Microarray analysis of the specimens identified 394 genes (72% upregulated and 28% downregulated) that changed with the progression of puberty. Overall changes in gene expression were small (average 1.38-fold upregulated and 1.36-fold downregulated genes). The 394 genes mapped to 13 significantly changing pathways (P<0.05) associated with growth plate maturation (e.g. extracellular matrix, cell cycle, and cell death). We next scanned the upstream promoter regions of the 394 genes for the presence of evolutionarily conserved binding sites for transcription factors implicated in growth plate maturation such as estrogen receptor (ER), androgen receptor, ELK1, STAT5B, cyclic AMP response element (CREB), and RUNX2. High-quality motif sites for RUNX2 (87 genes), ELK1 (43 genes), and STAT5B (31 genes), but not ER, were evolutionarily conserved, indicating their functional relevance across primates. Moreover, we show that some of these sites are direct target genes of these transcription factors as shown by ChIP assays.

  12. Distribution of slow-cycling cells in epiphyseal cartilage and requirement of β-catenin signaling for their maintenance in growth plate.

    PubMed

    Candela, Maria Elena; Cantley, Leslie; Yasuaha, Rika; Iwamoto, Masahiro; Pacifici, Maurizio; Enomoto-Iwamoto, Motomi

    2014-05-01

    Slow proliferation is one of the characteristics of stem cells. We examined the presence, distribution, and regulation of slow-cycling cells in the developing and growing skeleton using a pulse-chase method with a new nucleoside derivative, 5-ethynyl-2'-deoxyuridine (EdU). C57BL/6 mice received daily intraperitoneal injections of EdU from postnatal day 4 to day 7. One day after the last EdU injection, a large population of cells in articular cartilage and growth plate was labeled. Six weeks after the last injection, the number of EdU-labeled cells dramatically decreased, but a small number of them were dominantly present in the articular surface, and the labeling index was significantly higher in the surface than that in the rest of articular cartilage. In the growth plate, most EdU-positive cells were found in the top layer that lies immediately below the secondary ossification center. Interestingly, postnatal conditional ablation of β-catenin in cartilage caused a complete loss of the EdU-labeled cells in growth plate that displayed disorganization and dysfunction. Together, our data demonstrate that slow-cycling cells do reside in specific locations and numbers in both articular cartilage and growth plate. The β-catenin signaling pathway appears to play a previously unsuspected role in maintenance of the slow-cycling cells.

  13. Neoepitopes reveal the features of type II collagen cleavage and the identity of a collagenase involved in the transformation of the epiphyses anlagen in development.

    PubMed

    Lee, Eunice R; Lamplugh, Lisa; Kluczyk, Beata; Leblond, Charles P; Mort, John S

    2009-06-01

    In long bone development, the evolution of the cartilaginous anlagen into a secondary ossification center is initiated by the formation of canals. The excavation to create the canals is achieved through lysis of the two major cartilage components, aggrecan, and the type II collagen (COL2) fibril. The present study examines the lysis of the fibril. Because it is known that matrix metalloproteinases (MMPs) cleave COL2 in vitro at the Gly(775)-Leu(776) bond, it has been reasoned that, if such cleavage is detected in relation to the canals, it can be concluded that a collagenase is involved. Furthermore, because MMPs undergo change in domain structure with activation resulting in propeptide domain loss then, if such a loss is revealed in relation to the cleavage of COL2, this MMP is likely involved. The collective findings reveal that COL2 is attacked at the afore-described susceptible peptide bond at the surface of cartilage canals and, that MMP-13 cleaves it. Developmental Dynamics 238:1547-1563, 2009. (c) 2009 Wiley-Liss, Inc.

  14. The proportion of distal fibula Salter-Harris type I epiphyseal fracture in the paediatric population with acute ankle injury: a prospective MRI study.

    PubMed

    Hofsli, Mikael; Torfing, Trine; Al-Aubaidi, Zaid

    2016-03-01

    Ankle injuries are common among the paediatric population. There are few prospective studies utilizing MRI to diagnose a clinically suspected Salter-Harris type I of the distal fibula (SH1FDF). The aim of this study was to examine the proportion of clinically suspected SH1FDF in children. All paediatric patients with ankle injury, seen at the emergency room from September 2012 to May 2013 at a single institution, underwent a standardized clinical examination, and their radiographs were obtained if found necessary. All images and data were recorded prospectively and patients suspected of having SH1FDF were referred for MRI of the ankle joint. Out of 391 paediatric patients seen at the emergency room with ankle injury, 38 patients had a clinical suspicion of SH1FDF. A total of 31 patients, 18 male and 13 female, with a mean age of 10 ± 2.86 years, were included in the study. Only seven patients were excluded from the study. MRI was obtained on an average of 6.9 ± 2.87 days. None of the included patients had evidence of SH1FDF on MRI. Our study and review of the literature verifies the high false-positive rate of clinically suspected SH1FDF. Most children had ligamentous lesions, bone contusion or joint effusion, rather than SH1FDF.

  15. Salter-Harris Type-IV injuries of the distal tibial epiphyseal growth plate, with emphasis on those involving the medial malleolus.

    PubMed

    Cass, J R; Peterson, H A

    1983-10-01

    Salter-Harris Type-IV fractures of the epiphysis extend through the articular cartilage, epiphysis, physis, and metaphysis and have a high rate of complications secondary to premature partial closure of the physis. In this study we attempted to determine which Type-IV fractures of the distal end of the tibia result in premature partial closure, how the various treatment modalities affect the risk of premature physeal closure, and how the complication itself might be best managed. Thirty-two Type-IV fractures of the distal end of the tibia were seen at the Mayo Clinic during a five-year period. Eighteen injuries involved the medial malleolus, thirteen were so-called triplane fractures, and one was a fracture of the lateral part of the plafond. In the eighteen ankles with a fracture that involved the medial malleolus, extension of the fracture into the metaphysis could often be appreciated only on oblique roentgenograms. The patients' ages at the time of fracture ranged from one year and one month to fifteen years and six months old. In nine of the eighteen tibiae with a fracture of the medial malleolus premature partial closure of the distal physis developed, resulting in angular deformity or limb-length discrepancy sufficient to require operative treatment (epiphyseodesis, corrective osteotomy, or excision of a physeal bar). A physeal bar was best detected by tomograms made in two planes and by scanograms. Bar formation may be treated by excision of the bar, arrest of the whole physis, osteotomy, or combinations of these procedures. Of the thirteen patients with a triplane fracture and the one with a Type-IV fracture of the lateral part of the plafond, all fourteen were near maturity at the time of injury, and no growth-arrest problems developed.

  16. A geometric morphometrics comparative analysis of Neandertal humeri (epiphyses-fused) from the El Sidrón cave site (Asturias, Spain).

    PubMed

    Rosas, Antonio; Pérez-Criado, Laura; Bastir, Markus; Estalrrich, Almudena; Huguet, Rosa; García-Tabernero, Antonio; Pastor, Juan Francisco; de la Rasilla, Marco

    2015-05-01

    A new collection of 49,000 year old Neandertal fossil humeri from the El Sidrón cave site (Asturias, Spain) is presented. A total of 49 humeral remains were recovered, representing 10 left and 8 right humeri from adults, adolescents, and a juvenile (not included in the analyses). 3D geometric morphometric (GM) methods as well as classic anthropological variables were employed to conduct a broad comparative analysis by means of mean centroid size and shape comparisons, principal components analysis, and cluster studies. Due to the fragmentary nature of the fossils, comparisons were organized in independent analyses according to different humeral portions: distal epiphysis, diaphysis, proximal epiphysis, and the complete humerus. From a multivariate viewpoint, 3D-GM analyses revealed major differences among taxonomic groups, supporting the value of the humerus in systematic classification. Notably, the Australopithecus anamensis (KP-271) and Homo ergaster Nariokotome (KNM-WT 15000) distal humerus consistently clusters close to those of modern humans, which may imply a primitive condition for Homo sapiens morphology. Australopithecus specimens show a high degree of dispersion in the morphospace. The El Sidrón sample perfectly fits into the classic Neandertal pattern, previously described as having a relatively wide olecranon fossa, as well as thin lateral and medial distodorsal pillars. These characteristics were also typical of the Sima de los Huesos (Atapuerca) sample, African mid-Pleistocene Bodo specimen, and Lower Pleistocene TD6-Atapuerca remains and may be considered as a derived state. Finally, we hypothesize that most of the features thought to be different between Neandertals and modern humans might be associated with structural differences in the pectoral girdle and shoulder joint.

  17. Treatment methods for neoplastic metastates and tumor-like changes in the bodies and epiphyses of long bones using polymethyl methacrylate cement and bone grafting.

    PubMed

    Mrozek, Tomasz; Spindel, Jerzy; Miszczyk, Leszek; Koczy, Bogdan; Chrobok, Adam; Pilecki, Bolesław; Tomasik, Patryk; Matysiakiewicz, Jacek

    2005-10-30

    Background. The objective of our study was to evaluate the stabilization of reconstructed long bones after metastatic tumor resection and defect filling with polymethyl methacrylate (PMMA) or bone allograft. Material and methods. We studied a group of 107 patients who underwent surgery between 1996 and 2004 (55 females and 46 males). A primary neoplasmatic focus was found after histopathological examination in 58 cases, in 29 the histopathology was not evident, and in 20 cases no neoplastic tissue was found. Metastases were found within the femur in 73 cases, in the humerus in 19 cases, and in the tibia in 15 cases. Stabilization was performed using the traditional AO method, intramedullary nailing, or DHS/DCS fixation. Results. Taking into consideration clinical and radiological assessment, outcomes varied from fair to good. Better outcome was obtained in cases treated by polymethyl methacrylate (PMMA) filling combined with intramedullary nailing or DCS/DHS than in cases treated with traditional AO plating. For tumor-like lesions, complete bone graft consolidation was found after bone allograft filling in 14 of 20 cases. Conclusions. The 2 methods of long bone stabilization mentioned above, combined with polymethyl methacrylate (PMMA) or bone allograft filling, is the method of choice. Deep frozen bone grafting is possible only in cases of total tumor resection with the possibility of non-malignant tumor. The effect of reconstruction, besides fair or good outcome, included improved quality of life, less consumption of analgesics, and in many cases successful avoidance of pathological fracture.

  18. Metaphyseal chondrodysplasia with ectodermal dysplasia

    SciTech Connect

    Jequier, S.; Bellini, F.; Mackenzie, D.A.

    1981-11-01

    The first case of metaphyseal chondrodysplasia with marked cupping of the metaphyses and cone epiphyses combined with complete alopecia was described in 1966 by Bellini. A second identical case was found in another Italian patient. Both show extremely early epiphyseal fusion. This is probably a new form of metaphyseal chondrodysplasia.

  19. Biographical sketch: John Albert Key, 1890-1955.

    PubMed

    Brand, Richard A

    2013-07-01

    This biographical sketch on John Albert Key corresponds to the historic text, The Classic: Epiphyseal coxa vara or displacement of the capital epiphysis of the femur in adolescence, available at DOI 10.1007/s11999-013-2913-y.

  20. Pharmacokinetic Modeling of Trivalent and Hexavalent Chromium Based on Ingestion and Inhalation of Soluble Chromium Compounds.

    DTIC Science & Technology

    1991-12-01

    located primarily in the epiphyseal region of -he long bones. The distribution of radiolabel was reported to be similar to that seen after administration...of bone-seeking tracers 9 like Ca45 or Sr 89. Kraintz and Talmage (1952) had also noted localization of radiolabel in the epiphyses of long bones...weight gain, histologic findings, red and white blood cell counts, serum activities of alanine aminotransferase, alkaline phosphatase, total cholesterol

  1. Effects of factors of prolonged space flight on conditions of tortoise skeleton

    NASA Technical Reports Server (NTRS)

    Stupakon, G. P.; Volozhin, A. I.; Korzhenyants, V. A.; Yagodovskiy, V. S.; Polyakov, A. N.; Korolev, V. V.; Elivanov, V. A.

    1980-01-01

    After a 60-90 day space flight mild osteoporosis developed in the epiphyses and metaphyses of long tubular bones of tortoises, which was not attributed to reduced mineral saturation of the preserved bone tissue microstructures. The diminished strength of the cancellous bone of the epiphyses in tortoises after space flight was due to the reduced properties of its structure. The strength of the compact substance did not change under the effect of weightlessness.

  2. A comparative study of trabecular bone mass distribution in cursorial and non-cursorial limb joints.

    PubMed

    Chirchir, Habiba

    2015-05-01

    Skeletal design among cursorial animals is a compromise between a stable body that can withstand locomotor stress and a light design that is energetically inexpensive to grow, maintain, and move. Cursors have been hypothesized to reduce distal musculoskeletal mass to maintain a balance between safety and energetic cost due to an exponential increase in energetic demand observed during the oscillation of the distal limb. Additionally, experimental research shows that the cortical bone in distal limbs experiences higher strains and remodeling rates, apparently maintaining lower mass at the expense of a smaller safety factor. This study tests the hypothesis that the trabecular bone mass in the distal limb epiphyses of cursors is relatively lower than that in the proximal limb epiphyses to minimize the energetic cost of moving the limb. This study utilized peripheral quantitative computed tomography scanning to measure the trabecular mass in the lower and upper limb epiphyses of hominids, cercopithecines, and felids that are considered cursorial and non-cursorial. One-way ANOVA with Tukey post hoc corrections was used to test for significant differences in trabecular mass across limb epiphyses. The results indicate that overall, both cursors and non-cursors exhibit varied trabecular mass in limb epiphyses and, in certain instances, conform to a proximal-distal decrease in mass irrespective of cursoriality. Specifically, hominid and cercopithecine hind limb epiphyses exhibit a proximal-distal decrease in mass irrespective of cursorial adaptations. These results suggest that cursorial mammals employ other energy saving mechanisms to minimize energy costs during running.

  3. Valgus slipped capital femoral epiphysis: subcapital growth plate orientation analysis.

    PubMed

    Koczewski, Paweł

    2013-11-01

    The aim of the study was to determine the risk factors of unusual, lateral direction of epiphyseal displacement in primarily unilateral slipped capital femoral epiphysis (SCFE) patients with a special focus on radiological parameters of an unaffected hip. A total of 115 patients (75 boys, 40 girls), mean age 13.2 years (8.4-18.6), were analyzed. The mean follow-up time was 11 years (2-29). The proportion of valgus slip among SCFE patients was 11 of 115 cases (9.6%). The patients with valgus slip compared with the classic ones were predominantly females (55 vs. 33%), were younger (11.1 vs. 13.4 years), had a greater epiphyseal-shaft angle (67.4 vs. 59.1°), smaller displacement in the frontal plane (absolute value 6.7 vs. 15°), and a lower risk of contralateral slip (27 vs. 65%). There was no difference in the neck-shaft angle and epiphyseal-neck angle value. A more horizontal orientation of the subcapital growth plate, assessed by epiphyseal-shaft angle, can be considered a conducive factor in the valgus direction of epiphyseal slip in SCFE. In valgus SCFE cases, there is a smaller degree of epiphyseal displacement in both the sagittal and the coronal plane and a lower risk of consecutive contralateral slip.

  4. Finding an ideal site for intraosseous infusion of the tibia: an anatomical study.

    PubMed

    Boon, J M; Gorry, D L A; Meiring, J H

    2003-01-01

    Intraosseous infusion is a technique used for the administration of fluids to a hemodynamically shocked child in whom attempts to access the vascular system have been unsuccessful. Although few complications are seen, injury to the epiphyseal growth plate during the performance of this technique remains a serious problem. This study investigates the relationship between the site of insertion of the intraosseous needle and the epiphyseal growth plate, and the ease of needle insertion into various locations of the tibia in newborn infants. Fourteen newborn infant cadavers (28 tibias in total) were dissected after placement of four needles: 1). through the tibial tuberosity (Site A); 2). 10 mm distal to the tibial tuberosity (Site B); 3). 20 mm distal to the tibial tuberosity (Site C) and; 4). 10 mm proximal to the tibial tuberosity (Site D). Distances from the distal end of the epiphyseal growth plate were measured. A high number of needle placements at Site A were inserted into the epiphyseal growth plate. Most placements at Site B were between 10 and 16 mm from the epiphyseal growth plate on the right side and between 10 and 15 mm on the left side, and all were inserted without difficulty. Although far from the epiphyseal growth plate, most placements at Site C were very difficult to insert because of the thick cortical bone. All placements at Site D entered the epiphysis or the epiphysis and joint space of the knee. An insertion site of at least 10 mm distal to the tibial tuberosity is therefore recommended to avoid epiphyseal growth plate injury and ensure ease of insertion.

  5. Secondary avascular necrosis after treatment for congenital dislocation of the hip.

    PubMed

    Burgos, J; Gonzalez-Herranz, P; Ocete, G; Rapariz, J M

    1995-01-01

    We made a radiographic study of 104 unilateral congenital dislocations of the hip (CDH) that had an average age of 12 months (range 4-24), were treated with the same therapeutic protocol, and had an average six years follow-up (range 3-13). Radiographic changes were evident in the proximal femoral epiphysis in 57 hips (55%). In 7 (7%) the changes consisted of central osteoporosis with a cystic aspect, without metaphyseal or physeal changes, and had a normal end result without sequelae. This group was classified as type I. Fifty hips (48%) that were type II showed epiphyseal changes consisting of trabecular rarefaction with osteoporosis and irregular sclerosis, followed by a decrease of epiphyseal height and trabecular recovery. Forty-two hips (40%) were type IIA, with < 75% decrease in epiphyseal height with respect to the healthy side; five of these had a normal evolution, 35 had coxa magna and/or decrease of epiphyseal height, and only two cases had physeal lesion. In type IIB, hips, with > 75% decrease of epiphyseal height, 8 cases (8%) had a final physeal lesion.

  6. Osteochondrosis Can Lead to Formation of Pseudocysts and True Cysts in the Subchondral Bone of Horses.

    PubMed

    Olstad, K; Østevik, L; Carlson, C S; Ekman, S

    2015-09-01

    Osteochondrosis arises as a result of focal failure of the blood supply to growth cartilage. The current aim was to examine the pathogenesis of pseudocysts and true cysts in subchondral bone following failure of the blood supply to the articular-epiphyseal cartilage complex in horses. Cases were recruited based on identification of lesions (n = 17) that were considered likely to progress to or to represent pseudocysts or true cysts in epiphyseal bone in histological sections and included 10 horses ranging in age from 48 days to 5 years old. Cases comprised 3 warmbloods, 3 Standardbreds, 1 Quarter horse and 1 Arabian with spontaneous lesions and 2 Fjord ponies with experimentally induced lesions. Seven lesions consisted of areas of ischemic chondronecrosis and were compatible with pseudocysts. Two lesions were located at intermediate depth in epiphyseal growth cartilage, 2 lesions were located in the ossification front, 2 lesions were located in epiphyseal bone and 1 lesion was located in the metaphyseal growth plate (physis). Ten lesions contained dilated blood vessels and were compatible with true cysts. In 2 lesions the dilated blood vessels were located within the lumina of failed cartilage canals. In the 8 remaining lesions areas of ischemic chondronecrosis were associated with granulation tissue in the subjacent bone and dilated vessels were located within this granulation tissue. Failure of the blood supply and ischemic chondronecrosis can lead to formation of pseudocysts or dilatation of blood vessels and formation of true cysts in the epiphyseal bone of horses.

  7. Development and growth of long bones in European water frogs (Amphibia: Anura: Ranidae), with remarks on age determination.

    PubMed

    Rozenblut, Beata; Ogielska, Maria

    2005-09-01

    Differentiation and development of long bones were studied in European water frogs: Rana lessonae, R. ridibunda, and R. esculenta. The study included premetamorphic larvae (Gosner Stage 40) to frogs that were 5 years old. Femora, metatarsal bones, and proximal phalanges of the hindlimb exhibit the same pattern of periosteal bone differentiation and the same pattern of growth. Longitudinal and radial growth of these bones was studied by examination of the diaphyses and epiphyses, particularly where the edge of periosteal bone is inserted into the epiphysis. The periosteum seems to be responsible for both longitudinal and radial growth. Investigation of the formation, length, and arrangement of lines of arrested growth reveals that the first line is present only in the middle 25-35% of the length of the diaphysis of an adult bone; therefore, only the central portion of the diaphysis should be used for age estimation in skeletochronological studies. Comparison of the shapes and histological structures of epiphyses in the femur, metatarsal bones, and phalanges revealed that epiphyseal cartilages are composed of an inner and outer part. The inner metaphyseal cartilage has distinct zones and plugs the end of the periosteal bone cylinder; its role in longitudinal growth is questioned. The outer epiphyseal cartilage is composed of articular cartilages proper, in addition to lateral articular cartilages. Differences in the symmetry of the lateral articular cartilages of distal epiphyses of the femur and toes may reflect adaptations to different kinds of movements at the knee and in the foot.

  8. A study of wrist injuries in children: the incidence of various injuries and of premature closure of the distal radial growth plate.

    PubMed Central

    Fodden, D I

    1992-01-01

    There were 362 wrist injuries in 359 patients under 18 years of age who attended the Accident & Emergency Department, St James's University Hospital, Leeds, over a 1-year period. These included soft tissue injuries (53%), epiphyseal injuries (4%) and fractures of the distal forearm or carpus (43%) which were reviewed 3 years after injury. At re-examination, a repeat X-ray was undertaken and this showed evidence of premature epiphyseal fusion in three of the 281 who returned for review and in these three patients there was a history of persistent symptoms in the affected wrist or forearm. It is suggested that patients with an obvious epiphyseal injury or those who have persistent symptoms following an injury to the wrist should be followed carefully to skeletal maturity in order to detect early partial or total closure of the growth plate. PMID:1567538

  9. Abnormalities of proximal femoral growth after severe Perthes' disease.

    PubMed

    Sponseller, P D; Desai, S S; Millis, M B

    1989-08-01

    We studied the pattern of proximal femoral growth after severe Perthes' disease (Catterall grade III or IV) by retrospective analysis of serial radiographs in 52 hips (46 patients). Our aim was to determine the relationship between proximal femoral growth abnormalities and metaphyseal cysts, epiphyseal extrusion, physeal narrowing, and extensive epiphyseal necrosis. The average follow-up after treatment was 9.8 years (range 4 to 16 years), and 37 of the hips were followed to skeletal maturity. Slowing of proximal femoral growth was common: symmetrical abnormality was seen in 26 hips and asymmetrical abnormality in nine. However, definite premature closure of the proximal femoral physis was seen in only three hips. Abnormality seemed to be due to altered growth velocity rather than to bar formation in most cases. Metaphyseal cysts, epiphyseal extrusion and physeal narrowing during the active stage of the disease, alone or in combination, were found to be neither sensitive nor specific predictors of the subsequent growth pattern.

  10. Studies of Electrically Stimulated Rat Limb and Peripheral Nerve Regeneration.

    DTIC Science & Technology

    1983-08-25

    joints. The specimen in Fi remarkable perfection ofo 0with the pre-existing proxi JIMI) Jture. An epiphyseal plate i g o l m t 1. S om e b o n t s s...lidr.-Levnerat ion The bonesW andi joints freoromigare ceryrmnseto itlI ocs i bana ibstructures. An epiphyseal plate farrow) has formed in one of the...before the last were placed in the fields, but data analysis and histological preparations did extend for approximately an extra two months. The results

  11. Development of the long bones in the hands and feet of children: radiographic and MR imaging correlation.

    PubMed

    Laor, Tal; Clarke, Jeffrey P; Yin, Hong

    2016-04-01

    The long bones of the hands and feet in children have an epiphyseal end with a secondary center of ossification and an adjacent transverse physis. In contrast to other long bones in the body, the opposite end in the hands and feet, termed the non-epiphyseal end, is characterized by direct metaphyseal extension of bone to complete terminal ossification. The purpose of this pictorial essay is to illustrate the developmental stages of each end of the long bones of the hands and feet with radiographic and MR imaging to provide a foundation from which to differentiate normal from abnormal growth.

  12. Thiemann's finger or toe disease. Follow-up of seven cases.

    PubMed

    Schantz, K; Rasmussen, F

    1986-02-01

    Thiemann's disease is a non-inflammatory disorder of unknown etiology, affecting the epiphyses of the phalanges of the fingers and the first toe in children. We have re-examined seven patients after up to 18 years. Two patients had had pain in the affected digits for several years. In four patients radiographs after closure of the growth plates showed normal phalangeal dimensions without arthrosis. We conclude that different degrees of severity of epiphyseal disturbance consistent with the type described by Thiemann may be encountered. It is possible that trauma may worsen the prognosis by deformation of a susceptible epiphysis.

  13. [Updates on rickets and osteomalacia: etiology and pathophysiology of osteomalacia].

    PubMed

    Suzuki, Hisanori; Takeuchi, Yasuhiro

    2013-10-01

    Impairment of bone mineralization causes rickets and osteomalacia. Rickets develops with impaired mineralization of bone prior to epiphyseal closure, and so does osteomalacia after the closure of epiphyses. Pain in lower extremities and back and bone pain are usually observed in patients with osteomalacia. Chronic hypophosphatemia and/or impairment of vitamin D action are involved in the development of osteomalacia. It is of great importance to suspect osteomalacia from clinical symptoms and laboratory data, such as hypophosphatemia and/or high serum alkaline phosphatase level.

  14. Distal tibial physeal bridge: a complication from a tension band plate and screw construct. Report of a case.

    PubMed

    Oda, Jon E; Thacker, Mihir M

    2013-05-01

    We report on a case of a tension band plate and screw construct (Eight Plate) used over the anterior distal tibia in an 9-year-old girl in an attempt to induce recurvatum of the ankle joint to correct a recalcitrant equinus deformity. With growth of the distal tibial physis, the epiphyseal screw was drawn through the physis into the distal tibial metaphysis, resulting in the creation of a transphyseal bony bar. Caution should be exercised when attempting temporary hemiepiphyseodesis using a plate and screw construct in small epiphyses or in an osteopenic bone.

  15. Congenital hypothyroidism and concurrent renal insufficiency in a kitten.

    PubMed

    Lim, Chee Kin; Rosa, Chantal T; de Witt, Yolanda; Schoeman, Johan P

    2014-11-14

    A 3-month-old male domestic short-hair kitten was presented with chronic constipation and disproportionate dwarfism. Radiographs of the long bones and spine revealed delayed epiphyseal ossification and epiphyseal dysgenesis. Diagnosis of congenital primary hypothyroidism was confirmed by low serum total thyroxine and high thyroid stimulating hormone concentrations. Appropriate supplementation of levothyroxine was instituted. The kitten subsequently developed mild renal azotaemia and renal proteinuria, possibly as a consequence of treatment or an unmasked congenital renal developmental abnormality. Early recognition, diagnosis and treatment are vital as alleviation of clinical signs may depend on the cat's age at the time of diagnosis.

  16. Prolyl Hydroxylase Domain-Containing Protein 2 (Phd2) Regulates Chondrocyte Differentiation and Secondary Ossification in Mice

    PubMed Central

    Cheng, Shaohong; Aghajanian, Patrick; Pourteymoor, Sheila; Alarcon, Catrina; Mohan, Subburaman

    2016-01-01

    Endochondral ossification plays an important role in the formation of the primary ossification centers (POCs) and secondary ossification centers (SOCs) of mammalian long bones. However, the molecular mechanisms that regulate POC and SOC formation are different. We recently demonstrated that Prolyl Hydroxylase Domain-containing Protein 2 (Phd2) is a key mediator of vitamin C effects on bone. We investigated the role of Phd2 on endochondral ossification of the epiphyses by conditionally deleting the Phd2 gene in osteoblasts and chondrocytes. We found that the deletion of Phd2 in osteoblasts did not cause changes in bone parameters in the proximal tibial epiphyses in 5 week old mice. In contrast, deletion of Phd2 in chondrocytes resulted in increased bone mass and bone formation rate (normalized to tissue volume) in long bone epiphyses, indicating that Phd2 expressed in chondrocytes, but not osteoblasts, negatively regulates secondary ossification of epiphyses. Phd2 deletion in chondrocytes elevated mRNA expression of hypoxia-inducible factor (HIF) signaling molecules including Hif-1α, Hif-2α, Vegfa, Vegfb, and Epo, as well as markers for chondrocyte hypertrophy and mineralization such as Col10, osterix, alkaline phosphatase, and bone sialoprotein. These data suggest that Phd2 expressed in chondrocytes inhibits endochondral ossification at the epiphysis by suppressing HIF signaling pathways. PMID:27775044

  17. Compendium 0f Dental Residents’ Research Projects and Literature Reviews

    DTIC Science & Technology

    1989-05-01

    slow digestion to isolate chondrocytes by incubation at 370C in Ham’s F-12 with 0.1% collagenase for 24 h. Trypan-blue dye exclusion assay was used to...characteristics of matrix vesicle proteolipids (MVP). The MV were prepared from the growth cartilages of broiler chick epiphyses (All et al, 1970). Specific

  18. The fine structure of the proximal growth plate of the avian tibia: vascular supply.

    PubMed Central

    Howlett, C R; Dickson, M; Sheridan, A K

    1984-01-01

    The vascular supply to the proximal tibial growth plate of the 7 weeks old chicken is described using various vascular markers. In addition the ultrastructure of metaphyseal and epiphyseal vessels as well as their supporting tissue is reported. The metaphyseal arterioles terminate in large calibre vessels which have occasional endothelial gaps and no basement membrane or supporting cells, and therefore could be classified as venous sinusoids. In contrast the epiphyseal arteriole terminates in a capillary-venule plexus lined by an attenuated and fenestrated but continuous endothelium. This paper definitively establishes that communication of epiphyseal and metaphyseal vessels across the avian growth plate does not occur. The eosinophilic streaks which often join these two vascular supplies have been described ultrastructurally and would appear to be remnants of the 'retreating' epiphyseal vessels. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 Fig. 7 Fig. 8 Fig. 9 Fig. 10 Fig. 11 Fig. 12 Fig. 13 Fig. 14 Fig. 15 Fig. 16 PMID:6469851

  19. Recurrent pendunculated osteochondroma of the tibia.

    PubMed

    Lawrance, Scott E

    2015-01-01

    Osteochondromas have been widely reported in the literature. Surgical management can be a successful primary treatment option, but tumors can occur again in the presence of open epiphyseal growth plates. This case reports a recurrent pedunculated osteochondroma in a 15-year-old female.

  20. Analysis of Long Bone and Vertebral Failure Patterns

    DTIC Science & Technology

    1985-02-14

    have disc-shaped epiphyses on the surfaces of the vertebral bodies (Schmorl and Junghanns, 1959). Humans, ]< orangutans , gorillas...The annular epiphysis has been previously reported in humans, orangutans , gorillas, and marmosets (Schmorl and Junghanns, 1959; Bernick, et al

  1. Growth plate stress distribution implications during bone development: a simple framework computational approach.

    PubMed

    Guevara, J M; Moncayo, M A; Vaca-González, J J; Gutiérrez, M L; Barrera, L A; Garzón-Alvarado, D A

    2015-01-01

    Mechanical stimuli play a significant role in the process of long bone development as evidenced by clinical observations and in vivo studies. Up to now approaches to understand stimuli characteristics have been limited to the first stages of epiphyseal development. Furthermore, growth plate mechanical behavior has not been widely studied. In order to better understand mechanical influences on bone growth, we used Carter and Wong biomechanical approximation to analyze growth plate mechanical behavior, and explore stress patterns for different morphological stages of the growth plate. To the best of our knowledge this work is the first attempt to study stress distribution on growth plate during different possible stages of bone development, from gestation to adolescence. Stress distribution analysis on the epiphysis and growth plate was performed using axisymmetric (3D) finite element analysis in a simplified generic epiphyseal geometry using a linear elastic model as the first approximation. We took into account different growth plate locations, morphologies and widths, as well as different epiphyseal developmental stages. We found stress distribution during bone development established osteogenic index patterns that seem to influence locally epiphyseal structures growth and coincide with growth plate histological arrangement.

  2. ( sup 99m Tc)diphosphonate uptake and hemodynamics in arthritis of the immature dog knee

    SciTech Connect

    Hansen, E.S.; Soballe, K.; Henriksen, T.B.; Hjortdal, V.E.; Buenger, C. )

    1991-03-01

    The relationship between (99mTc)diphosphonate uptake and bone hemodynamics was studied in canine carrageenan-induced juvenile chronic arthritis. Blood flow was determined with microspheres, plasma and red cell volumes were measured by labeled fibrinogen and red cells, and the microvascular volume and mean transit time of blood were calculated. Normal femoral epiphyses had lower central and higher subchondral blood flow and diphosphonate uptake values. Epiphyseal vascular volume was uniform, resulting in a greater transit time of blood centrally. In arthritis, blood flow and diphosphonate uptake were increased subchondrally and unaffected centrally, while epiphyseal vascular volume was increased throughout, leading to prolonged transit time centrally. The normal metaphyses had low blood flow and diphosphonate uptake values in cancellous bone and very high values in growth plates, but a large vascular volume throughout. The mean transit time therefore was low in growth plates and high in adjacent cancellous bone. Arthritis caused decreased blood flow and diphosphonate uptake in growth plates but increased vascular volume and transit time of blood. Diphosphonate uptake correlated positively with blood flow and plasma volume and negatively with red cell volume in a nonlinear fashion. Thus, changes in diphosphonate uptake and microvascular hemodynamics occur in both epiphyseal and metaphyseal bone in chronic synovitis of the immature knee. The (99mTc)diphosphonate bone scan seems to reflect blood flow, plasma volume, and red cell volume of bone.

  3. Injuries to the Young Athlete.

    ERIC Educational Resources Information Center

    Sandusky, Jane C.

    A review of literature on the incidence and nature of injuries to young athletes is presented on the topics of: (1) physiological characteristics of preadolescents, adolescents, and young adults; (2) musculo-skeletal changes in the growing athlete; (3) epiphyseal injuries and their potential for resulting in temporary or permanent impairment; (4)…

  4. A Very Rare Presentation of Type 1 Monteggia Equivalent Fracture with Ipsilateral Fracture of Distal Forearm-approach with Outcome: Case Report

    PubMed Central

    Singh, Dhananjay; Awasthi, Bhanu; Padha, Vikas; Thakur, Sanjay

    2016-01-01

    Introduction: We report a case of Type 1 Monteggia equivalent injury with intact radio-capitellar congruity, associated with epiphyseal fracture of distal radius and distal ulna shaft in an 11-year-old boy. There are only a few cases of Monteggia or Monteggia equivalent injury with ipsilateral forearm fractures in children, and injury pattern being reported by us is not only rare but also the only case reported thus far to the best of our knowledge, Sood et al. described Type 1 equivalent with epiphyseal injuries of both radius and ulna Osada et al. also described injury pattern same as Sood et al. with epiphyseal separation in both distal radius and ulna. Our case was slightly different than above two in that distally, there was ulna shaft fracture with Salter Harris Type 2 epiphyseal separation in the radius. Case Report: An 11-year-old, right-hand dominant boy presented in casualty with a history of fall one day back with pain, swelling and deformity in the left forearm with bleeding from left forearm and loss of movement of fingers and thumb of the left hand. On examination, there was a wound of size one centimeter on mid-forearm over the ulnar aspect. Extension of fingers and thumb at metacarpophalangeal joints was lost with intact sensations suggestive of posterior interosseus nerve involvement. No vascular was deficit was present. X-rays were performed which suggested type two epiphyseal separation proximal radius with fracture shaft ulna with lateral angulation in elbow and proximal forearm. Radiocapitellar joint congruity was maintained in the views performed. X-rays of wrist suggested fracture both bones distal forearm epiphysis in distal radius and distal shaft in ulna. The patient was operated with toileting, debridement, and open reduction of proximal ulnar fracture with K-wire. Proximal radius epiphyseal separation was approached by Kocher approach and fixed with two K-wires, while for distal radius epiphyseal separation open reduction and internal

  5. Sex determination by discriminant function analysis of the tibia for contemporary Croats.

    PubMed

    Slaus, Mario; Bedić, Zeljka; Strinović, Davor; Petrovečki, Vedrana

    2013-03-10

    Previous studies have demonstrated that populations differ from each other in size and proportion, and that these differences can affect metric assessment of sex. This paper establishes standards for determining sex from fragmentary and complete tibiae in the modern Croatian population. Measurements were taken on 180 tibiae (109 male and 71 female) from positively identified victims of the 1991-1995 War in Croatia. Six standard dimensions: length of the tibia (CML), maximum epiphyseal breadth of the proximal tibia (MPEB), maximum epiphyseal breadth of the distal tibia (MDEB), maximum diameter of the tibia at the nutrient foramen (MDNF), transverse diameter of the tibia at the nutrient foramen (TDNF), and circumference of the tibia at the nutrient foramen (CNF), were taken and subjected to different discriminant function analyses. The highest level of accuracy (91.1%) in the analyzed data set was achieved employing the variables: maximum epiphyseal breadth of the proximal tibia, maximum epiphyseal breadth of the distal tibia, maximum diameter of the tibia at the nutrient foramen, transverse diameter of the tibia at the nutrient foramen, and circumference of the tibia at the nutrient foramen. The second highest level of accuracy (90.6%) was achieved using a combination of only three variables: maximum epiphyseal breadth of the proximal tibia, maximum diameter of the tibia at the nutrient foramen, and circumference of the tibia at the nutrient foramen. The lowest accuracy (84.4%) was obtained when only one variable (maximum diameter of the tibia at the nutrient foramen) was employed. The results of this study show that the modern Croatian tibia is a good skeletal component for determining sex. Standardized coefficients of the discriminant functions generated in this study support the results of previous studies that found that breadth dimensions provide better separation of the sexes than length.

  6. Regeneration of Articular Cartilage in Lizard Knee from Resident Stem/Progenitor Cells

    PubMed Central

    Alibardi, Lorenzo

    2015-01-01

    The epiphysis of femur and tibia in the lizard Podarcis muralis can extensively regenerate after injury. The process involves the articular cartilage and metaphyseal (growth) plate after damage. The secondary ossification center present between the articular cartilage and the growth plate is replaced by cartilaginous epiphyses after about one month of regeneration at high temperature. The present study analyzes the origin of the chondrogenic cells from putative stem cells located in the growing centers of the epiphyses. The study is carried out using immunocytochemistry for the detection of 5BrdU-labeled long retaining cells and for the localization of telomerase, an enzyme that indicates stemness. The observations show that putative stem cells retaining 5BrdU and positive for telomerase are present in the superficial articular cartilage and metaphyseal growth plate located in the epiphyses. This observation suggests that these areas represent stem cell niches lasting for most of the lifetime of lizards. In healthy long bones of adult lizards, the addition of new chondrocytes from the stem cells population in the articular cartilage and the metaphyseal growth plate likely allows for slow, continuous longitudinal growth. When the knee is injured in the adult lizard, new populations of chondrocytes actively producing chondroitin sulfate proteoglycan are derived from these stem cells to allow for the formation of completely new cartilaginous epiphyses, possibly anticipating the re-formation of secondary centers in later stages. The study suggests that in this lizard species, the regenerative ability of the epiphyses is a pre-adaptation to the regeneration of the articular cartilage. PMID:26340619

  7. Regeneration of Articular Cartilage in Lizard Knee from Resident Stem/Progenitor Cells.

    PubMed

    Alibardi, Lorenzo

    2015-09-01

    The epiphysis of femur and tibia in the lizard Podarcis muralis can extensively regenerate after injury. The process involves the articular cartilage and metaphyseal (growth) plate after damage. The secondary ossification center present between the articular cartilage and the growth plate is replaced by cartilaginous epiphyses after about one month of regeneration at high temperature. The present study analyzes the origin of the chondrogenic cells from putative stem cells located in the growing centers of the epiphyses. The study is carried out using immunocytochemistry for the detection of 5BrdU-labeled long retaining cells and for the localization of telomerase, an enzyme that indicates stemness. The observations show that putative stem cells retaining 5BrdU and positive for telomerase are present in the superficial articular cartilage and metaphyseal growth plate located in the epiphyses. This observation suggests that these areas represent stem cell niches lasting for most of the lifetime of lizards. In healthy long bones of adult lizards, the addition of new chondrocytes from the stem cells population in the articular cartilage and the metaphyseal growth plate likely allows for slow, continuous longitudinal growth. When the knee is injured in the adult lizard, new populations of chondrocytes actively producing chondroitin sulfate proteoglycan are derived from these stem cells to allow for the formation of completely new cartilaginous epiphyses, possibly anticipating the re-formation of secondary centers in later stages. The study suggests that in this lizard species, the regenerative ability of the epiphyses is a pre-adaptation to the regeneration of the articular cartilage.

  8. Mild Campomelic Dysplasia: Report on a Case and Review

    PubMed Central

    Corbani, S.; Chouery, E.; Eid, B.; Jalkh, N.; Abou Ghoch, J.; Mégarbané, A.

    2011-01-01

    We report on a 10.5-year-old girl with a mild form of campomelic dysplasia. She presented with short stature of prenatal onset, dysmorphic facial features, limitation of supination and pronation of the forearms, dysplastic nails, and bone abnormalities consisting especially of cone-shaped epiphyses of the middle phalanx of the 2nd fingers, brachydactyly and clinodactyly of the middle phalanx of both 5th fingers, short 4th metacarpals, radial and femoral head subluxation, hypoplastic scapulae, humeral and ulnar epiphyseal abnormalities, unossified symphysis pubis, and a significant delay in bone age. Molecular analysis of the SOX9 gene revealed the presence of a de novo missense mutation: p.P170L (c.509C>T). Mild and surviving cases of campomelic dysplasia are reviewed. PMID:21373255

  9. [Macroscopic and functional anatomy of the apophyseal plate].

    PubMed

    Putz, R; Milz, S

    2016-03-01

    Apophyses are growth zones attached to the shaft (corpus) of larger bones. They vary in size and develop their own ossification centres or form as part of an usually fibrocartilaginous tendon or ligament insertion. The structure of the cartilaginous apophyseal plate is very similar to that of an epiphyseal growth plate and like these they are adapted to withstand perpendicular compressive forces without becoming harmed. This is best highlighted by the fact that their mineralized borders always orient themselves perpendicular to the overall resulting force vector. The edges of the apophyseal plates are characteristically bent which allows them to resist moderate shear forces. Like the epiphyseal plates the apophyseal plates exhibit a zonal organization which is not very well adapted to permanently withstand shear forces, especially if they occur under dynamic conditions. In these situations the tendinous insertions with their collagen fibre anchoring system have to provide compensation when balancing the load transmitted across the system.

  10. [The effect of tibial transphyseal reinforcement on the growth and response of leg tissues].

    PubMed

    Popkov, D A; Kononovich, N A; Shutov, R B

    2014-07-01

    Transphyseal reinforcement of right intact tibia performed with thin steel rods in six mongrel dogs at the age of six months. Contralateral segment served as control. The leg growth and blood supply studied under the created conditions for the next six months. Radiographic, physiologic (surface thermometry, photoplethysmography), and statistical methods used for studying. The significant effect of transphyseally inserted rods on the leg longitudinal growth and blood supply has not been revealed. The changes in natural shape-formation oftibial proximal and distal meta-epiphyses observed influenced by the transphyseal rods in the experiment. In order to evaluate the tissue response and the degree of the functional activity of leg bone meta-epiphyseal zones the most informative areas considered to be the following: the area of medial malleolus in the early period of physiological growth completion, and the area of the tibial lateral condyle--at the late stage.

  11. Cucolepis cincta gen.n. et sp.n. (Cestoda: Cyclophyllidea) from the squirrel cuckoo Piaya cayana lesson (Aves: Cuculiformes) from Paraguay.

    PubMed

    Phillips, Anna J; Mariaux, Jean; Georgiev, Boyko B

    2012-12-01

    Cucolepis gen. n. is erected as monotypic for Cucolepis cincta sp. n., a new species of cyclophyllidean cestode of the family Paruterinidae. The new species is described from the squirrel cuckoo, Piaya cayana Lesson (Aves: Cuculiformes), taken from two localities in Paraguay in 1984 and 1985. This new genus is most similar to the genus Triaenorhina Spasskii et Shumilo, 1965 in terms of the hook morphology and large epiphyseal structures extending from both the handle and guard, but differs in several aspects of the strobilar morphology, such as the shape of the cirrus sac, genital atrium, uterus and paruterine organ. The strobilar morphology of the new genus strongly resembles that of the genus Francobona Georgiev et Kornyushin, 1994, especially the shape of the cirrus sac and genital atrium, yet Francobona spp. lack, the developed epiphyseal structures observed in species of Cucolepis and Triaenorhina. Previous records and the nature of parasite-host associations between cuculiform birds and their cestode parasites are discussed.

  12. [OVERUSE INJURIES IN THE YOUNG ATHLETE].

    PubMed

    Journeau, Pierre; Polirzstok, E; Launay, F; Barbier, D

    2015-10-01

    Sport injuries are common in children and teenaggers, especially overuse injuries. Typical musculoskelotal disorders include apophyseal pains or avulsion, stess fractures, and also epiphyseal ostoochondritis. Some lesions are specific according the practice, such spine or wrist lesions. Prevention of sport injury should be the main priority as well for the parents than the coaches and children themselves. Proper education and preparation, specific training and streching are essential for all sports activities.

  13. Increases in bone density during treatment of men with idiopathic hypogonadotropic hypogonadism

    SciTech Connect

    Finkelstein, J.S.; Klibanski, A.; Neer, R.M.; Doppelt, S.H.; Rosenthal, D.I.; Segre, G.V.; Crowley, W.F. Jr. )

    1989-10-01

    To assess the effects of gonadal steroid replacement on bone density in men with osteoporosis due to severe hypogonadism, we measured cortical bone density in the distal radius by 125I photon absorptiometry and trabecular bone density in the lumbar spine by quantitative computed tomography in 21 men with isolated GnRH deficiency while serum testosterone levels were maintained in the normal adult male range for 12-31 months (mean +/- SE, 23.7 +/- 1.1). In men who initially had fused epiphyses (n = 15), cortical bone density increased from 0.71 +/- 0.02 to 0.74 +/- 0.01 g/cm2 (P less than 0.01), while trabecular bone density did not change (116 +/- 9 compared with 119 +/- 7 mg/cm3). In men who initially had open epiphyses (n = 6), cortical bone density increased from 0.62 +/- 0.01 to 0.70 +/- 0.03 g/cm2 (P less than 0.01), while trabecular bone density increased from 96 +/- 13 to 109 +/- 12 mg/cm3 (P less than 0.01). Cortical bone density increased 0.03 +/- 0.01 g/cm2 in men with fused epiphyses and 0.08 +/- 0.02 g/cm2 in men with open epiphyses (P less than 0.05). Despite these increases, neither cortical nor trabecular bone density returned to normal levels. Histomorphometric analyses of iliac crest bone biopsies demonstrated that most of the men had low turnover osteoporosis, although some men had normal to high turnover osteoporosis. We conclude that bone density increases during gonadal steroid replacement of GnRH-deficient men, particularly in men who are skeletally immature.

  14. Rheumatologic and neurological events in an elderly patient with tricho-rhino-phalangeal syndrome type I.

    PubMed

    Rué, Marjory; Lüdecke, Hermann-Josef; Sibon, Igor; Richez, Christophe; Taine, Laurence; Foubert-Samier, Alexandra; Arveiler, Benoit; Schaeverbeke, Thierry; Lacombe, Didier; Tison, François; Goizet, Cyril

    2011-01-01

    Sparse scalp hair, a peculiar shape of the nose, and cone-shaped epiphyses of the phalanges are the hallmarks of the tricho-rhino-phalangeal syndromes (TRPS). Short stature, hip dysplasia, and malformations of inner organs including mitral valve prolpase have also often been described for these conditions. Here, we described a 64-year-old woman with molecularly proved TRPS I and several atypical late-onset rheumatologic and neurological symptoms.

  15. [Slipped capital femoral epiphysis associated with hyperparathyroidism. A case report].

    PubMed

    Khiari, Karima; Cherif, Lotfi; Ben Abdallah, Nejib; Maazoun, Imen; Hadj Ali, Insaf; Bentaarit, Chokri; Turki, Sami; Ben Maïz, Hedi

    2003-12-01

    Slippage of the upper femoral epiphysis can occur in association with multiple endocrine imbalances. A case of slipped femoral epiphysis with primary hyperparathyroidism is reported. The patient was an adolescent, 16 Years of age, who presented bilateral slipped epiphysis. Investigation showed that he had hypercalcemia (3.1 mmol/l) related to primary hyperparathyroidism. A parathyroid adenoma was removed. Outcome was favorable and the slipped femoral epiphyses did not require a specific treatment.

  16. Preliminary Study of the Effects of Prolonged Acceleration on Spinal Dynamics of Baboons. 1. Acceleration. 2. Biomechanical Analysis

    DTIC Science & Technology

    1981-06-01

    cortical thickness, increased diameter/cortlcal thickness ratio, thinned and distorted epiphyseal plate, and thickened condylar cartilage in female rats...histomorphometric and histodynamic (tetracycline fixation) studies were conducted on calcified sections by analyzing six bone parameters (e,g,, bone volume , relative...growth cartilage alterations, a decrease in bone volume without an Increase in osteocytic activity, and (by tetracycline fluroescent analysis) a reduced

  17. The sagging rope sign in Perthes' disease and allied disorders.

    PubMed

    Apley, A G; Wientroub, S

    1981-02-01

    The sagging rope sign is the term used to describe the radiographic appearances which sometimes occur after Perthes' disease. It is severe examples of that disease and indicates damage to the growth plate with a marked metaphysial reaction. The same appearance follows severe epiphysitis after forcible reduction of a congenitally dislocated hip, and certain rare epiphysial dysplasias. The origin and significance of the sign are discussed.

  18. Generalized dysplasia epiphysealis hemimelica with contralateral sacro-iliac joint involvement.

    PubMed

    Karam, Adib R; Birjawi, Ghina A; Saghieh, Saïd; Tawil, Ayman; Khoury, Nabil J

    2008-12-01

    Dysplasia epiphysealis hemimelica (or Trevor's disease) is a rare developmental bone dysplasia characterized by benign osteocartilaginous overgrowth involving one or multiple epiphyses, usually of a single lower extremity. It is classified as localized form, classical form (most common), and generalized form. In this report we describe a case of generalized form of dysplasia epiphysealis hemimelica with involvement of the contralateral sacroiliac joint, which is an extremely rare presentation.

  19. Comparison of calcium and phosphorus excretion with bone density changes during restraint in immature Macaca nemestrina primates.

    NASA Technical Reports Server (NTRS)

    Hoffman, R. A.; Hood, W. N.; Mack, P. B.

    1972-01-01

    Calcium and phosphorus balance data on Macaca nemestrina monkeys during immobilization are presented and correlated with X-ray bone densitometry findings. A positive mineral balance was maintained during the immobilized period. A reduced bone density was observed in most skeletal sites examined with increased density observed in epiphyseal regions. Migration of mineral from one site to another is suggested as a possible explanation for the findings.

  20. Mechanobiology and joint conformity regulate endochondral ossification of sesamoids.

    PubMed

    Sarin, V K; Carter, D R

    2000-09-01

    Sesamoid bones form by the endochondral ossification of sesamoid cartilages. This ossification process is thought to be similar to that responsible for the formation of secondary ossific nuclei in long-bone epiphyses. Sesamoids ossify much later in development than do epiphyses, however, and bone formation within sesamoids often begins by way of multiple ossific nuclei. Endochondral growth and ossification in the formation of secondary ossific nuclei have previously been correlated with distributions of the octahedral shear and hydrostatic stresses generated in vivo within cartilage anlagen. In this study, we used two-dimensional finite element analysis to predict the distributions of octahedral shear and hydrostatic stresses in an idealized model of a sesamoid cartilage subjected to in vivo loading. We examined the influence of sesamoid joint conformity. The distribution of an osteogenic stimulus was calculated with an approach similar to that used to predict epiphyseal ossification. The results suggest that, compared with conforming joints, nonconformity between the sesamoid cartilage and its articulating surface, which arises during early development, produces higher contact pressures within the sesamoid and leads to a thicker articular cartilage layer. For a nonconforming joint surface, the results suggest that ossification is favored anywhere within a broad internal region of the sesamoid, whereas a layer at the articular surface will remain cartilaginous. These findings highlight the subtle differences between ossification processes in epiphyses and sesamoids, indicating that the mechanical stress environment in sesamoids produces a diffuse stimulus leading to the onset of ossification and that the degree of joint nonconformity may influence the thickness of the articular cartilage layer.

  1. Fracture of the distal radial physis complicated by compartment syndrome and premature physeal closure.

    PubMed

    Hernandez, J; Peterson, H A

    1986-01-01

    Complications after injury to the epiphyseal growth plate of the distal radius are uncommon. An 11-year-8-month-old boy developed two complications--compartment syndrome of the forearm and premature closure of the physis--after a Salter-Harris Type I injury. Treatment included closed reduction, decompression, skin grafting, excision of the physeal bar, and epiphysiodesis of the distal ulna and the remaining open physis of the distal radius.

  2. Hereditary multiple exostoses: a case report

    PubMed Central

    Pikula, John R

    1996-01-01

    Hereditary multiple exostoses (HME) is a dominantly inherited skeletal disorder which alters enchondral bone during growth and is characterized by exostoses of the juxta-epiphyseal regions. These exostoses are benign cartilaginous neoplasms that consist of a pedicle of normal bone covered with proliferating cartilage cells. Pathologic, clinical, and radiographic findings are discussed and a case of a nine-year-old male is reviewed. ImagesFigure 1Figure 2Figure 3Figure 4

  3. Effect of Arterial Deprivation on Growing Femoral Epiphysis: Quantitative Magnetic Resonance Imaging Using a Piglet Model

    PubMed Central

    Cheon, Jung-Eun; Kim, In-One; Kim, Woo Sun; Choi, Young Hun

    2015-01-01

    Objective To investigate the usefulness of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) and diffusion MRI for the evaluation of femoral head ischemia. Materials and Methods Unilateral femoral head ischemia was induced by selective embolization of the medial circumflex femoral artery in 10 piglets. All MRIs were performed immediately (1 hour) and after embolization (1, 2, and 4 weeks). Apparent diffusion coefficients (ADCs) were calculated for the femoral head. The estimated pharmacokinetic parameters (Kep and Ve from two-compartment model) and semi-quantitative parameters including peak enhancement, time-to-peak (TTP), and contrast washout were evaluated. Results The epiphyseal ADC values of the ischemic hip decreased immediately (1 hour) after embolization. However, they increased rapidly at 1 week after embolization and remained elevated until 4 weeks after embolization. Perfusion MRI of ischemic hips showed decreased epiphyseal perfusion with decreased Kep immediately after embolization. Signal intensity-time curves showed delayed TTP with limited contrast washout immediately post-embolization. At 1-2 weeks after embolization, spontaneous reperfusion was observed in ischemic epiphyses. The change of ADC (p = 0.043) and Kep (p = 0.043) were significantly different between immediate (1 hour) after embolization and 1 week post-embolization. Conclusion Diffusion MRI and pharmacokinetic model obtained from the DCE-MRI are useful in depicting early changes of perfusion and tissue damage using the model of femoral head ischemia in skeletally immature piglets. PMID:25995692

  4. /sup 99m/Tc-DPD uptake in juvenile hemarthrosis. Scintimetry and autoradiography of the knee in dogs

    SciTech Connect

    Hansen, E.S.; Hjortdal, V.E.; Noer, I.; Christensen, S.B.; Holm, I.E.; Buenger, C.

    1989-03-01

    The pathogenesis of subchondral bone lesions and growth plate affection in hemophilic arthropathy was studied in puppies by means of repeated regional /sup 99m/Tc-diphosphonate scintimetry and contact autoradiography. Unilateral hemarthrosis of the knee was induced by biweekly intraarticular injections of autologous blood for 12 weeks. Hemarthrosis caused an early (2 to 4 weeks) decrease in uptake of /sup 99m/Tc-diphosphonate in the juxtaarticular growth plates (ratio 0.7) and a delayed (8 to 10 weeks) increase in epiphyseal uptake (ratio 1.5). In a recovery phase after hemarthrosis, growth plate uptake returned to normal, while the epiphyseal uptake remained elevated for 8 to 10 weeks. By contact autoradiography, the growth plate uptake was localized to the calcification layer at the metaphyseal aspect of the growth plates, while the epiphyseal uptake mainly was seen in the thin subchondral and subsynovial bone layer and around osteophytes. The changes in uptake of /sup 99m/Tc-diphosphonate following hemarthrosis for 3 months were reversible and could be ascribed to the presence of synovial inflammation.

  5. [Corrective intra-articular surgery in juvenile femur head epiphysiolysis].

    PubMed

    Gekeler, J; Kneer, W

    1984-01-01

    Treatment of severely slipped capital femoral epiphysis depends on the individual pathoanatomic and pathophysiologic conditions. Since the femoral neck vessels remain intact in chronic slip, they should be preserved whenever possible. Bilateral chondrolysis developed in 1 of our 9 cervical osteotomy patients. Long-term radiologic and clinical findings after a cervical osteotomy do not differ appreciably from those after an Imhäuser osteotomy, in some cases, with incomplete realignment of the femoral head-acetabulum relation. Apparently, not only the "quantitative" factor but also the "qualitative" factor plays an important role in epiphyseal separation. By contrast, severe acute slip is a severe irritation of the joint accompanied by intra-articular bleeding and rupture of the femoral neck vessels. Good results are achieved after immediate operative decompression, controlled reduction, and stable fixation of the epiphysis, providing anatomic realignment of the femoral head-acetabulum relation is not forced, but rather carried out only to the limit of tolerance without leverage maneuver and under moderate traction and vision. Epiphyseal necrosis developed after conventional open reduction in 2 of our 16 patients with acute slip. No cases of epiphyseal necrosis have been observed to date in any of our patients (N = 5) treated with this new technique of controlled reduction (i.e., partial reduction in "acute chronic slip").

  6. Expression of the Ellis-van Creveld (Evc) gene in the rat tibial growth plate.

    PubMed

    Tsuji, Takehito; Nakamura, Hiroaki; Hirata, Azumi; Yamamoto, Toshio

    2004-08-01

    Ellis-van Creveld (EvC) syndrome is an autosomal recessive chondrodysplasia characterized by short limbs, postaxial polydactyly, natal teeth, and dysplastic nails. The Ellis-van Creveld (EVC) gene, which is mutated in patients with EvC syndrome, has been identified by positional cloning. However, the physiological roles of the EVC gene have not been elucidated. Histopathological analyses of EvC syndrome have shown disturbed chondrocytic phenotypes during cartilage development. We therefore postulated that the EVC gene is a critical factor for chondrocytes during endochondral ossification. The present study focuses on the relationship between the Evc gene and chondrocytes, and examines Evc gene expression in the rat tibial growth plate at the mRNA and protein levels. Evc mRNA in tibial epiphyseal cartilage was expressed at postnatal day (P) 1, P28, and P56 by RT-PCR. Immunohistochemical analyses localized the Evc protein mainly in prehypertrophic and hypertrophic chondrocytes of the epiphyseal growth plate in the tibia during the embryonic and postnatal periods. Evc mRNA was also detected in prehypertrophic and hypertrophic chondrocytes by in situ hybridization. These results indicate that the Evc gene functions mainly in the prehypertrophic and hypertrophic chondrocytes of the epiphyseal growth plate. The data presented here are important for future studies of the underlying mechanism of chondrodysplasia in EvC syndrome.

  7. Giant Cell Tumor: A Rare Condition in the Immature Skeleton—A Retrospective Study of Symptoms, Treatment, and Outcome in 16 Children

    PubMed Central

    Skeie, Anette Torød; Lobmaier, Ingvild Koren; Zaikova, Olga

    2016-01-01

    Background. Pediatric giant cell tumor (GCT) of bone is rare and the course of the disease in the immature skeleton is sparsely described. We performed a retrospective study addressing symptoms, treatment, and outcome in children with GCT. Methods. Review of medical records and images of patients with GCT. Patients were detected from our hospital prospective database and those with open epiphyseal cartilages were included. Results. 16 children (75% girls) from 6 to 15 years old were identified. Eight lesions (50%) were in long bones and 4 (25%) in flat bones. One lesion appeared to be purely epiphyseal. All patients had pain as the initial symptom. Local recurrence developed in 2 patients. 14 of 16 patients returned to normal activity with no sequelae. One patient developed anisomelia after surgery. Conclusions. The biological tumor behavior in children does not seem to differ from what is reported in adults. Lesions in flat bones are very unusual, but our data alone do not provide enough evidence to conclude that this is more common in the immature skeleton. Literature review showed only one previous case report describing a purely epiphyseal GCT. Intralesional curettage is appropriate treatment and gives good functional results with acceptable recurrence rates. PMID:27999474

  8. A review of the actual knowledge of the processes governing growth and development of long bones.

    PubMed

    Pazzaglia, Ugo Ernesto; Beluffi, Giampiero; Benetti, Anna; Bondioni, Maria Pia; Zarattini, Guido

    2011-01-01

    Autoptic samples of human bones (from 8 weeks of gestation to 12 years of age) and a second group of serial, skeletal x-rays (required for pathologies not related to bone dysplasia in children from 4 months to 17 years of age) provided the material for the analysis of the physes normal growth mechanism presented in this review. Before the appearance of the ossification centers epiphyseal growth rests exclusively on chondrocytes proliferation (interstitial growth), without any detectable differentiated cellular organization. When endochondral ossification starts a defined spatial disposition of chondrocytes and a corresponding organization of the intercellular matrix is set up, so that it is possible to identify a growth vector corresponding to the columns of piled chondrocytes with direction from hypertrophic toward the proliferative cell layers. The complexity of the tubular bones growth process is well represented by the spatial arrangement of the growth vectors. In the late epiphyseal growth another mechanism is active in addition to endochondral ossification, namely, articular cartilage interstitial growth and subchondral remodelling. The knowledge of the normal mode of organization of the physis and its temporal sequence can help to better understand of the deviaton from the normal development of metaphyseal and epiphyseal dysplasias.

  9. CT evaluation of medial clavicular epiphysis as a method of bone age determination in adolescents and young adults

    PubMed Central

    Ufuk, Furkan; Agladioglu, Kadir; Karabulut, Nevzat

    2016-01-01

    PURPOSE We aimed to investigate the use of computed tomography (CT) staging of the medial clavicular epiphysis ossification in forensic bone age determination, and find a CT criterion to determine whether an individual is adult or not. METHODS Chest CT and pulmonary CT angiography exams of 354 patients between 10 and 30 years of age (mean, 21.4 years) were retrospectively evaluated for epiphyseal ossification phase of the bilateral medial clavicles (708 clavicles) and compared with the sex and chronologic age of the individuals. The ossification phase of the medial clavicular epiphyses was classified from stage I to stage V using a modified staging system. RESULTS Epiphyseal ossification center appeared from 11 to 21 years of age. Partial fusion occurred between 16 and 23 years of age. Complete fusion was first achieved at the ages of 18 and 19 years for male and female individuals, respectively. The probability of an individual being ≥18 years old was 70.8% in stage III A and 100% in stages III B, IV, and V in females and males. CONCLUSION CT evaluation of the medial clavicular epiphysis is helpful in forensic age determination and stage III B can be used as a criterion to make the prediction that an individual is older than 18 years. PMID:27015321

  10. PEDF Is Associated with the Termination of Chondrocyte Phenotype and Catabolism of Cartilage Tissue

    PubMed Central

    Klinger, P.; Ferrazzi, F.; Hotfiel, T.; Swoboda, B.; Aigner, T.

    2017-01-01

    Objective. To investigate the expression and target genes of pigment epithelium-derived factor (PEDF) in cartilage and chondrocytes, respectively. Methods. We analyzed the expression pattern of PEDF in different human cartilaginous tissues including articular cartilage, osteophytic cartilage, and fetal epiphyseal and growth plate cartilage, by immunohistochemistry and quantitative real-time (qRT) PCR. Transcriptome analysis after stimulation of human articular chondrocytes with rhPEDF was performed by RNA sequencing (RNA-Seq) and confirmed by qRT-PCR. Results. Immunohistochemically, PEDF could be detected in transient cartilaginous tissue that is prone to undergo endochondral ossification, including epiphyseal cartilage, growth plate cartilage, and osteophytic cartilage. In contrast, PEDF was hardly detected in healthy articular cartilage and in the superficial zone of epiphyses, regions that are characterized by a permanent stable chondrocyte phenotype. RNA-Seq analysis and qRT-PCR demonstrated that rhPEDF significantly induced the expression of a number of matrix-degrading factors including SAA1, MMP1, MMP3, and MMP13. Simultaneously, a number of cartilage-specific genes including COL2A1, COL9A2, COMP, and LECT were among the most significantly downregulated genes. Conclusions. PEDF represents a marker for transient cartilage during all neonatal and postnatal developmental stages and promotes the termination of cartilage tissue by upregulation of matrix-degrading factors and downregulation of cartilage-specific genes. These data provide the basis for novel strategies to stabilize the phenotype of articular cartilage and prevent its degradation. PMID:28191465

  11. Ossification Pattern of Estuarine Dolphin (Sotalia guianensis) Forelimbs, from the Coast of the State of Espírito Santo, Brazil

    PubMed Central

    Botta, Silvina; de Queiroz, Fábio Ferreira; Campos, Adélia Sepúlveda

    2015-01-01

    The estuarine dolphin, Sotalia guianensis, is one of the most abundant cetacean species in Brazil. Determination of age and of aspects associated with the development of this species is significant new studies. Counts of growth layer groups in dentin are used to estimate age of these animals, though other ways to evaluate development are also adopted, like the measurement of total length (TL). This study presents a procedure to evaluate the development of the estuarine dolphin based on the ossification pattern of forelimbs. Thirty-seven estuarine dolphins found in the state of Espírito Santo, Brazil, were examined. Age was estimated, TL was measured and ossification of epiphyses was examined by radiography. We analyzed results using the Spearman correlation. Inspection of radiographs allowed evaluation of the significance of the correlation between age and development of the proximal (r = 0.9109) and distal (r = 0.9092) radial epiphyses, and of the distal ulnar epiphyses (r = 0.9055). Radiographic analysis of forelimbs proved to be an appropriate method to evaluate physical maturity, and may be a helpful tool to estimate age of these animals in ecological and population studies. PMID:26017269

  12. PEDF Is Associated with the Termination of Chondrocyte Phenotype and Catabolism of Cartilage Tissue.

    PubMed

    Klinger, P; Lukassen, S; Ferrazzi, F; Ekici, A B; Hotfiel, T; Swoboda, B; Aigner, T; Gelse, K

    2017-01-01

    Objective. To investigate the expression and target genes of pigment epithelium-derived factor (PEDF) in cartilage and chondrocytes, respectively. Methods. We analyzed the expression pattern of PEDF in different human cartilaginous tissues including articular cartilage, osteophytic cartilage, and fetal epiphyseal and growth plate cartilage, by immunohistochemistry and quantitative real-time (qRT) PCR. Transcriptome analysis after stimulation of human articular chondrocytes with rhPEDF was performed by RNA sequencing (RNA-Seq) and confirmed by qRT-PCR. Results. Immunohistochemically, PEDF could be detected in transient cartilaginous tissue that is prone to undergo endochondral ossification, including epiphyseal cartilage, growth plate cartilage, and osteophytic cartilage. In contrast, PEDF was hardly detected in healthy articular cartilage and in the superficial zone of epiphyses, regions that are characterized by a permanent stable chondrocyte phenotype. RNA-Seq analysis and qRT-PCR demonstrated that rhPEDF significantly induced the expression of a number of matrix-degrading factors including SAA1, MMP1, MMP3, and MMP13. Simultaneously, a number of cartilage-specific genes including COL2A1, COL9A2, COMP, and LECT were among the most significantly downregulated genes. Conclusions. PEDF represents a marker for transient cartilage during all neonatal and postnatal developmental stages and promotes the termination of cartilage tissue by upregulation of matrix-degrading factors and downregulation of cartilage-specific genes. These data provide the basis for novel strategies to stabilize the phenotype of articular cartilage and prevent its degradation.

  13. Altered lumbar spine structure, biochemistry and biomechanical properties in a canine model of mucopolysaccharidosis type VII

    PubMed Central

    Smith, Lachlan J; Martin, John T; Szczesny, Spencer E; Ponder, Katherine P; Haskins, Mark E; Elliott, Dawn M

    2010-01-01

    Mucopolysaccharidosis VII (MPS VII) is a lysosomal storage disorder characterized by a deficiency in β-glucuronidase activity, leading to systemic accumulation of poorly degraded glycosaminoglycans (GAG). Along with other morbidities, MPS VII is associated with paediatric spinal deformity. The objective of this study was to examine potential associations between abnormal lumbar spine matrix structure and composition in MPS VII, and spine segment and tissue-level mechanical properties, using a naturally occurring canine model with a similar clinical phenotype to the human form of the disorder. Segments from juvenile MPS VII and unaffected dogs were allocated to: radiography, gross morphology, histology, biochemistry, and mechanical testing. MPS VII spines had radiolucent lesions in the vertebral body epiphyses. Histologically, this corresponded to a GAG-rich cartilaginous region in place of bone, and elevated GAG staining was seen in the annulus fibrosus. Biochemically, MPS VII samples had elevated GAG in the outer annulus fibrosus and epiphyses, low calcium in the epiphyses, and high water content in all regions except the nucleus pulposus. MPS VII spine segments had higher range of motion and lower stiffness than controls. Endplate indentation stiffness and failure loads were significantly lower in MPS VII samples, while annulus fibrosus tensile mechanical properties were normal. Vertebral body lesions in MPS VII spines suggest a failure to convert cartilage to bone during development. Low stiffness in these regions likely contributes to mechanical weakness in motion segments and is a potential factor in the progression of spinal deformity. PMID:19918911

  14. Effect of pathological fracture on limb salvage surgery with preservation of the epiphysis in children with osteosarcoma of the distal femur: Two case reports.

    PubMed

    Zheng, Kai; Yu, Xiuchun; Chang, Zhengqi; Xu, Songfeng; Xu, Ming

    2016-04-01

    The outcome of limb salvage treatment for femoral osteosarcoma with pathological fractures in children is currently unknown. The aim of the present study was to present two cases of patients who received limb salvage surgery with preservation of the epiphysis at the Department of Orthopedics of The General Hospital of Jinan Military Commanding Region (Shandong, China). Between January, 2007 and January, 2013, two pediatric patients were admitted to our hospital with pathological fractures. One of the patients was a girl, aged 11 years, with confirmed osteosarcoma of the right distal femur; the other patient was a boy, aged 9 years, with osteosarcoma of the left distal femur. After receiving two cycles of neoadjuvant chemotherapy following tumor biopsy, the patients received limb salvage surgery with epiphyseal preservation, with wide resection of the tumor and biological reconstruction by allogeneic bone and fibular autograft, followed by 10 cycles of adjuvant chemotherapy. With a mean follow-up of 64 months, there were no postoperative complications, local recurrence or metastasis. The limb function recovered well, although limb shortening was observed. The female patient underwent a second fixation and limb lengthening after epiphyseal closure. Therefore, with effective neoadjuvant chemotherapy, limb salvage surgery with epiphyseal preservation is not contraindicated for pediatric patients with pathological fractures from femoral osteosarcoma. Biological reconstruction by allogeneic bone and vascularized fibular autograft following wide tumor resection is a viable option for such patients, with a good postoperative functional outcome.

  15. The topologic and chronologic patterns of hematopoietic cell seeding in host femoral bone marrow after transplantation.

    PubMed

    Askenasy, Nadir; Stein, Jeremiah; Yaniv, Isaac; Farkas, Daniel L

    2003-08-01

    The early stages of homing, seeding, and engraftment of hematopoietic stem and progenitor cells are poorly characterized. We have developed an optical technique that allows in vivo tracking of transplanted, fluorescent-tagged cells in the host femurs. In this study we used fluorescence microscopy to monitor the topologic and chronologic patterns of hematopoietic cell seeding in the femoral bone marrow (BM) of mice. PKH-labeled cells homed to the femur within minutes after injection into a peripheral vein. Most cells drifted within the marrow space and gradually seeded in clusters close to the endosteal surface of the epiphyseal cortex. Three days after transplantation 85% to 94% (14%) of PKH-labeled cells in the femoral marrow were located within 100 microm of the epiphyseal bone surface (P <.001 versus the more central cells), whereas labeled cells were absent in the femoral diaphysis. Primary seeding of juxtaendosteal, epiphyseal marrow occurred independently of recipient conditioning (myeloablated and nonconditioned hosts), donor-recipient antigen disparity, or the phenotype of the injected cells (whole BM and lineage-negative cells) and was consistently observed in secondary recipients of BM-homed cells. Seeding in regions close to the epiphyseal bone was also observed in freshly excised femurs perfused ex vivo and in femurs assessed without prior placement of optical windows, indicating that the site of primary seeding was not affected by surgical placement of optical windows. Four to 5 days after transplantation, cellular clusters appeared in the more central regions of the epiphyses and in the diaphyses. Centrally located cells showed decreased PKH fluorescence, suggesting that they were progeny of the seeding cells, and brightly fluorescent cells (quiescent first-generation seeding cells) were observed close to the bone surface for as long as 24 days after transplantation. These data indicate that the periphery of the femoral marrow hosts primary seeding

  16. Incidental Findings on Knee Radiographs in Children and Adolescents

    PubMed Central

    Seo, Sang Gyo; Chung, Chin Youb; Lee, Kyoung Min; Lee, Seung Yeol; Choi, Young; Kim, Tae Gyun; Baek, Jeong Kook; Kwon, Soon-Sun; Kwon, Dae Gyu; Choi, In Ho; Cho, Tae-Joon; Yoo, Won Joon; Park, Moon Seok

    2014-01-01

    Background Despite the wide use of knee radiography in children and adolescent patients visiting the outpatient clinic, there has been no analysis about the prevalence and type of incidental findings yet. This study was performed to investigate the incidental findings on knee radiographs in children and adolescents according to age. Methods A total of 1,562 consecutive patients younger than 18 years of age were included. They who visited Seoul National University Bundang Hospital's outpatient clinic with a chief complaint of knee pain or malalignment between 2010 and 2011. We reviewed the knee radiographs and analyzed the prevalence and type of incidental findings, such as metaphyseal lucent area, epiphyseal cortical irregularity, osteochondroma and Harris growth arrest line. Results The mean age of the patients was 10.2 years (range, 1 month to 18 years). We identified 355 incidental findings in 335 patients (21.4%) and 98 abnormal findings (6.3%). The most common incidental finding was metaphyseal lucent area (131, 8.4%), followed by epiphyseal cortical irregularity (105, 6.7%), Harris growth arrest line (75, 4.8%), and osteochondroma (44, 2.8%). An epiphyseal cortical irregularity tended to have a higher prevalence at younger age (p < 0.001) and the prevalences of metaphyseal lucent area and Harris growth arrest line were also higher at a younger age (p = 0.001 and p < 0.001, respectively). However, the osteochondroma tended to have a higher prevalence at an older age (p = 0.004). Conclusions This study describes the incidental findings on knee radiographs in children and adolescents and provides effective information from a viewpoint of an orthopedic doctor. The authors recommend considering those incidental findings if unfamiliar findings appear on a knee radiograph in the pediatric outpatient clinic. PMID:25177456

  17. The AO Pediatric Comprehensive Classification of Long Bone Fractures (PCCF)

    PubMed Central

    Joeris, Alexander; Lutz, Nicolas; Blumenthal, Andrea; Slongo, Theddy; Audigé, Laurent

    2017-01-01

    Background and purpose To achieve a common understanding when dealing with long bone fractures in children, the AO Pediatric Comprehensive Classification of Long Bone Fractures (AO PCCF) was introduced in 2007. As part of its final validation, we present the most relevant fracture patterns in the upper extremities of a representative population of children classified according to the PCCF. Patients and methods We included children and adolescents (0–17 years old) diagnosed with 1 or more long bone fractures between January 2009 and December 2011 at the university hospitals in Bern and Lausanne (Switzerland). Patient charts were retrospectively reviewed and fractures were classified from standard radiographs. Results Of 2,292 upper extremity fractures in 2,203 children and adolescents, 26% involved the humerus and 74% involved the forearm. In the humerus, 61%, and in the forearm, 80% of single distal fractures involved the metaphysis. In adolescents, single humerus fractures were more often epiphyseal and diaphyseal fractures, and among adolescents radius fractures were more often epiphyseal fractures than in other age groups. 47% of combined forearm fractures were distal metaphyseal fractures. Only 0.7% of fractures could not be classified within 1 of the child-specific fracture patterns. Of the single epiphyseal fractures, 49% were Salter-Harris type-II (SH II) fractures; of these, 94% occurred in schoolchildren and adolescents. Of the metaphyseal fractures, 58% showed an incomplete fracture pattern. 89% of incomplete fractures affected the distal radius. Of the diaphyseal fractures, 32% were greenstick fractures. 24 Monteggia fractures occurred in pre-school children and schoolchildren, and 2 occurred in adolescents. Interpretation The pattern of pediatric fractures in the upper extremity can be comprehensively described according to the PCCF. Prospective clinical studies are needed to determine its clinical relevance for treatment decisions and prognostication

  18. Effects of 1 alpha,25- and 24R,25-dihydroxyvitamin D3 on aluminum-induced rickets in growing uremic rats.

    PubMed

    Vukicević, S; Krempien, B; Stavljenić, A

    1987-12-01

    Rats were subjected to a two-stage subtotal nephrectomy or sham operation, and treated with aluminum (Al) or both aluminum and vitamin D3 metabolites for 5 weeks with a cumulative dose of 13.6 mg aluminum. Animals were injected with 3H-thymidine and 3H-proline. The following analyses were performed: quantitative histology of tibial metaphyses and cytomorphometric electron microscopy of osteoclasts, quantitative (ICP-spectroscopy) and qualitative determination (histochemical staining) of aluminum within organs, and serum biochemistry (Ca, P, Mg, vitamin D3 metabolites, alkaline phosphatase, urea). The following new facts of the aluminum-related bone disease became evident: (a) Application of aluminum to growing uremic rats induced rickets, whose major epiphyseal growth plate changes were 1 alpha,25(OH)2D3-dependent. Addition of 1 alpha,25(OH)2D3 prevented the formation of rachitic metaphysis, but failed to prevent osteoid accumulation on epiphyseal and metaphyseal trabecular surfaces. Moreover, calcitriol produced hyperosteoidosis and osteosclerosis in the same rats. Aluminum did not alter the function of osteoblasts, while osteoclasts seemed inactivated. (b) The development of rickets was associated with suppressed serum levels of 1,25(OH)2D3, reduced phosphorus level and the high content of aluminum in the bone, kidney, and liver. The addition of 24R,25(OH)2D3 markedly exaggerated the reduction of serum levels of calcitriol. We suggested that aluminum induces rickets in growing uremic rats, which consists of two components: vitamin D refractory osteomalacia and 1 alpha,25(OH)2D3-dependent epiphyseal growth plate changes.

  19. The AO Pediatric Comprehensive Classification of Long Bone Fractures (PCCF).

    PubMed

    Joeris, Alexander; Lutz, Nicolas; Blumenthal, Andrea; Slongo, Theddy; Audigé, Laurent

    2017-04-01

    Background and purpose - To achieve a common understanding when dealing with long bone fractures in children, the AO Pediatric Comprehensive Classification of Long Bone Fractures (AO PCCF) was introduced in 2007. As part of its final validation, we present the most relevant fracture patterns in the upper extremities of a representative population of children classified according to the PCCF. Patients and methods - We included children and adolescents (0-17 years old) diagnosed with 1 or more long bone fractures between January 2009 and December 2011 at the university hospitals in Bern and Lausanne (Switzerland). Patient charts were retrospectively reviewed and fractures were classified from standard radiographs. Results - Of 2,292 upper extremity fractures in 2,203 children and adolescents, 26% involved the humerus and 74% involved the forearm. In the humerus, 61%, and in the forearm, 80% of single distal fractures involved the metaphysis. In adolescents, single humerus fractures were more often epiphyseal and diaphyseal fractures, and among adolescents radius fractures were more often epiphyseal fractures than in other age groups. 47% of combined forearm fractures were distal metaphyseal fractures. Only 0.7% of fractures could not be classified within 1 of the child-specific fracture patterns. Of the single epiphyseal fractures, 49% were Salter-Harris type-II (SH II) fractures; of these, 94% occurred in schoolchildren and adolescents. Of the metaphyseal fractures, 58% showed an incomplete fracture pattern. 89% of incomplete fractures affected the distal radius. Of the diaphyseal fractures, 32% were greenstick fractures. 24 Monteggia fractures occurred in pre-school children and schoolchildren, and 2 occurred in adolescents. Interpretation - The pattern of pediatric fractures in the upper extremity can be comprehensively described according to the PCCF. Prospective clinical studies are needed to determine its clinical relevance for treatment decisions and

  20. Climate-mediated shifts in Neandertal subsistence behaviors at Pech de l'Azé IV and Roc de Marsal (Dordogne Valley, France).

    PubMed

    Hodgkins, Jamie; Marean, Curtis W; Turq, Alain; Sandgathe, Dennis; McPherron, Shannon J P; Dibble, Harold

    2016-07-01

    Neandertals disappeared from Europe just after 40,000 years ago. Some hypotheses ascribe this to numerous population crashes associated with glacial cycles in the late Pleistocene. The goal of this paper is to test the hypothesis that glacial periods stressed Neandertal populations. If cold climates stressed Neandertals, their subsistence behaviors may have changed-requiring intensified use of prey through more extensive nutrient extraction from faunal carcasses. To test this, an analysis of Neandertal butchering was conducted on medium sized bovid/cervid remains composed of predominately red deer (Cervus elaphus), reindeer (Rangifer tarandus), and roe deer (Capreolus caprelous) deposited during global warm and cold phases from two French sites: Pech de l'Azé IV (Pech IV, Bordes' excavation) and Roc de Marsal (RDM). Analysis of surface modification on high survival long bones and proximal and middle phalanges demonstrates that skeletal elements excavated from the cold levels (RDM Level 4, Pech IV Level I2) at each cave have more cut marks and percussion marks than elements from the warm levels (RDM Level 9, Pech IV Level Y-Z) after controlling for fragment size. At both sites, epiphyseal fragments are rare, and although this pattern can result from carnivore consumption, carnivore tooth marks are almost nonexistent (<0.1%). Alternatively, processing epiphyseal ends for bone grease may have been a Neandertal survival strategy, and epiphyses were more intensively percussed in cold levels than in warm levels at both RDM and Pech IV. The exploitation of low marrow yield elements such as phalanges does not show a consistent pattern relating to climate, but may have been a general Neandertal behavioral characteristic, suggesting that these hominids were regularly on the edge of sufficient nutrient availability even during interglacials. Overall, the faunal assemblages from Roc de Marsal and Pech IV provide some support for the hypothesis that Neandertals were processing

  1. Dietary lipids modify the fatty acid composition of cartilage, isolated chondrocytes and matrix vesicles.

    PubMed

    Xu, H; Watkins, B A; Adkisson, H D

    1994-09-01

    The effects of dietary lipids on the fatty acid composition of hyaline cartilage, epiphyseal chondrocytes (EC) and matrix vesicles (MV) were evaluated in chicks. A basal semipurified diet was fed to chicks containing one of the following lipid sources at 70 g/kg: soybean oil, butter+corn oil, margarine+corn oil or menhaden oil+corn oil (MEC). Articular and epiphyseal growth cartilage were isolated from the proximal tibiotarsus; EC and MV were subsequently released by trypsin (EC 3.4.21.4) and collagenase (EC 3.4.24.3) digestion followed by ultracentrifugation. The fatty acid composition of polar lipids in chick epiphyseal cartilage at three and six weeks, as well as articular cartilage, EC and MV at eight weeks of age revealed the presence of high levels of saturated and monounsaturated fatty acids (up to 85.5%) but low levels of n-6 polyunsaturated fatty acids (PUFA) (2.6-10.2%). Mead acid (20:3n-9, > 3%) was also present in cartilage, EC and MV lipids, and was unaffected by the dietary lipid treatments. Total n-3 PUFA concentrations were the highest in cartilage, EC and MV of chicks consuming MEC. Feeding MEC lowered the levels of 20:4n-6 in cartilage, but increased 20:5n-3 levels. The data are consistent with those reported previously which showed that cartilage tissues are low in n-6 PUFA and that they contain 20:3n-9. We furthermore demonstrated that the PUFA composition of cartilage can be modified by dietary lipids.

  2. Prevention of arthrofibrosis after arthroscopic screw fixation of tibial spine fracture in children and adolescents.

    PubMed

    Parikh, Shital N; Myer, David; Eismann, Emily A

    2014-01-01

    Arthrofibrosis is a major complication of tibial spine fracture treatment in children, potentially resulting in knee pain, quadriceps weakness, altered gait, decreased function, inability to return to sports, and long-term osteoarthritis. Thus, prevention rather than treatment of arthrofibrosis is desirable. The purpose of this study was to evaluate an aggressive postoperative rehabilitation and early intervention approach to prevent permanent arthrofibrosis after tibial spine fracture treatment and to compare epiphyseal and transphyseal screws for fixation. A consecutive series of 24 patients younger than age 18 with displaced type II and III tibial spine fractures who underwent arthroscopic reduction and screw fixation between 2006 and 2011 were retrospectively reviewed. Final range of motion was compared between patients with epiphyseal (n=12) and transphyseal (n=9) screws. One-third (4 of 12) of patients with epiphyseal screws underwent arthroscopic debridement and screw removal approximately 3 months postoperatively; 3 patients lacked 5° to 15° of extension, 1 experienced pain with extension, and 1 had radiographic evidence of screw pullout, loss of reduction, and resultant malunion. In the transphyseal screw group, 3 patients had 10° loss of extension, and all corrected after arthroscopic debridement and screw removal. The two groups did not significantly differ in time to hardware removal or return to sports or final range of motion. No growth disturbances were identified in patients after transphyseal screw removal. An aggressive approach of postoperative rehabilitation and early intervention after arthroscopic reduction and screw fixation of tibial spine fractures in children was successful in preventing permanent arthrofibrosis.

  3. Free Fat Graft for Congenital Hand Differences

    PubMed Central

    Ishigaki, Daisuke; Satake, Hiroshi; Iba, Kousuke

    2012-01-01

    Background Free fat graft has been used for the treatment of congenital hand differences. However, there have been a few reports about the outcome of that treatment. In this study, the outcome of free fat grafts for congenital hand and foot differences was investigated. Methods Fourteen bones with longitudinal epiphyseal bracket, 3 wrists with Madelung deformity, and 5 cases of osseous syndactyly were treated with free fat graft with osteotomy, physiolysis, or separation of osseous syndactyly. Of the fourteen bones with longitudinal epiphyseal bracket, 9 were treated with open wedge osteotomy with free fat graft and 5 with physiolysis and free fat graft. The Madelung deformity was treated with physiolysis with free fat graft. For osseous syndactyly, syndactyly release with free fat graft was performed five times on four hands. Results In the fourteen cases with longitudinal epiphyseal bracket, lateral deviation improved in all except two cases after surgery. The average lateral deviation angle changed from 32.5 degrees before surgery to 15.2 degrees after surgery. The average improvement of the lateral deviation angle was 12.2 degrees in the osteotomy group and 20.6 degrees in the physiolysis group. The mean ratio of improvement of the lateral deviation angle to the lateral deviation angle before surgery was 39.4% in the osteotomy group and 51.2% in the physiolysis group. The Madelung deformity improved after surgery in two cases but there was no improvement in one case. For these conditions, the results were not good enough when surgery was done after age 13 or at age four for severely hypoplastic brachymesophalangy. Of the 5 cases of osseous syndactyly, reunion of the separated bones occurred in one case. The grafted free fat should be deep enough to cover the osteotomy site of the bones to prevent reunion of the separated bones. Conclusions Physiolysis and free fat graft performed during the growth period can correct the deviation due to longitudinal epiphyseal

  4. Combined autologous chondrocyte implantation (ACI) with supra-condylar femoral varus osteotomy, following lateral growth-plate damage in an adolescent knee: 8-year follow-up

    PubMed Central

    2011-01-01

    We report the 8-year clinical and radiographic outcome of an adolescent patient with a large osteochondral defect of the lateral femoral condyle, and ipsilateral genu valgum secondary to an epiphyseal injury, managed with autologous chondrocyte implantation (ACI) and supracondylar re-alignment femoral osteotomy. Long-term clinical success was achieved using this method, illustrating the effective use of re-alignment osteotomy in correcting mal-alignment of the knee, protecting the ACI graft site and providing the optimum environment for cartilage repair and regeneration. This is the first report of the combined use of ACI and femoral osteotomy for such a case. PMID:21418566

  5. Adult-onset idiopathic chondrolysis of the hip.

    PubMed

    Yapp, Liam Z; McClymont, Liusaidh; Beggs, Ian; Gaston, Paul; Salter, Donald M

    2017-05-01

    We report the case of a 23-year-old man diagnosed with adult-onset idiopathic chondrolysis of the hip. Chondrolysis of the hip is a disorder most frequently seen in children who have suffered with slipped capital femoral epiphyses. Idiopathic chondrolysis of the hip is extremely rare and to our knowledge, its onset has never been documented in adults aged over 20. With reference to the available medical literature, we summarise the current clinical management of this unusual but important cause of young adult hip pain.

  6. Sclerosteosis (craniotubular hyperostosis-syndactyly) with complex hyperphalangy of the index finger.

    PubMed

    Yagi, Hiroko; Takagi, Masaki; Hasegawa, Yukihiro; Kayserili, Hülya; Nishimura, Gen

    2015-07-01

    We report a 4-year-old boy with sclerosteosis associated with severe digital dysostosis. The initial medical consultation was prompted by bilateral, asymmetrical syndactyly of the index and middle fingers. The left index finger had complicated phalangeal anomalies: hyperphalangy (supernumerary phalanx distal to the middle phalanx) and hypoplasia with bracket epiphyses of the proximal and middle phalanges. Development of facial nerve palsy, hearing impairment and generalized osteosclerosis had occurred between 3 years and 4 years of age, with the subsequent identification of a homozygous SOST mutation. Bilateral second and third fingers syndactyly associated with abnormal patterning of the same fingers should be considered prodromal signs of sclerosteosis.

  7. Combined autologous chondrocyte implantation (ACI) with supra-condylar femoral varus osteotomy, following lateral growth-plate damage in an adolescent knee: 8-year follow-up.

    PubMed

    Vijayan, Sridhar; Bentley, George

    2011-03-18

    We report the 8-year clinical and radiographic outcome of an adolescent patient with a large osteochondral defect of the lateral femoral condyle, and ipsilateral genu valgum secondary to an epiphyseal injury, managed with autologous chondrocyte implantation (ACI) and supracondylar re-alignment femoral osteotomy. Long-term clinical success was achieved using this method, illustrating the effective use of re-alignment osteotomy in correcting mal-alignment of the knee, protecting the ACI graft site and providing the optimum environment for cartilage repair and regeneration. This is the first report of the combined use of ACI and femoral osteotomy for such a case.

  8. Dysplasia Epiphysealis Hemimelica (Trevor Syndrome) of Talus in a 21-Year Old Woman; Case Report

    PubMed Central

    Sadeghifar, Amir R; Heshmati, Afshin Ahmadzadeh

    2014-01-01

    Dysplasia epiphysealis hemimelica is a rare nonhereditary epiphyseal disease that mimics synovial chondromatosis and osteochondroma of the joints. The disease mainly involves long bones of the lower extremities and tarsal bones. Herein we report 21-year old woman who presented with pain and limited range of ankle motion, who underwent surgical excision of talus exostosis after preoperative imaging and careful planning. after that she was discharged and her problem improved and she have no problem in three year follow up. pathologic examination of specimen confirm the diagnosis. PMID:25207317

  9. More on the Liang Bua finds and modern human cretins.

    PubMed

    Oxnard, Charles; Obendorf, Peter J; Kefford, Ben J; Dennison, John

    2012-12-01

    Brown (2012: LB1 and LB6 Homo floresiensis are not modern human (Homo sapiens) cretins, Journal of Human Evolution) makes errors of fact, omission and interpretation. Brown's comments refer, among others, to (1) delayed growth and development indicated by unfused epiphyses, (2) postcranial limb proportions: limbs to trunk, between limbs, and within limbs, (3) postcranial bone torsions and angles, (4) postcranial robusticity, real and apparent, (5) skull features, and (6) cretinism on Flores. In each of these areas, much information about cretins is incorrect and much information (Oxnard et al., 2010) comparing the Liang Bua remains with cretins is ignored.

  10. Proton microprobe analysis of zinc in skeletal tissues. [Proton induced x-ray emission analysis

    SciTech Connect

    Doty, S B; Jones, K W; Kraner, H W; Shroy, R E; Hanson, A L

    1980-06-01

    A proton microprobe with windowless exit port was used to study zinc distributions in various types of skeletal tissues. The use of an external beam facilitated positioning of the targets for examination of particular points of interest. The proton microprobe is uniquely suited to this work since it combines high sensitivity for zinc determinations in thick samples with good spatial resolution. Measurements on rat and rabbit Achilles tendon showed a significant increase in zinc concentrations as the beam moved from the unmineralized collagen into the mineralized attachment site. Cartilage gave a similar result, with calcified cartilage having a greater zinc level than the articular surface on unmineralized epiphyseal cartilage.

  11. Re-implantation of a degloving amputation of distal index finger caused by fireworks: a case report and literature review.

    PubMed

    Zhang, Q; Cao, X C

    2013-07-01

    Amputations associated with fireworks are customarily treated by stump revision resulting in permanent disability. In this case report, we present an eight-year old boy who suffered an amputation of his right distal index finger at the level of the epiphyseal disk with degloving injury of the amputated finger caused by fireworks. Successful re-implantation was achieved. Two-year follow-up revealed fair cosmesis and acceptable functional and aesthetic recovery though the free distal phalanx had been absorbed completely. Re-implantation of a degloving amputation finger caused by fireworks is possible and can provide good distal soft tissue coverage and recovery of sensory and motor functions.

  12. Imaging of physeal bars in children.

    PubMed

    Wang, David C; Deeney, Vincent; Roach, James W; Shah, Amisha J

    2015-08-01

    The growth plate, also known as the physis or epiphyseal plate, is essential for longitudinal growth of bones in the immature skeleton. A variety of insults to the growth plate from trauma to infection to idiopathic causes can lead to physeal bar formation, an interruption in normal growth plate cartilage, where a bony or fibrous bridge develops between the metaphysis and epiphysis. This bridge restricts subsequent bone growth, leading to limb shortening and/or angular deformities. Early recognition of the presence of a physeal bar can help direct appropriate surgical management to restore linear growth of the bone.

  13. [Osseous bridge after physeal-injury to the distal tibia with spontaneous resolution].

    PubMed

    Trnka, J; Sýkora, L; Bibza, J

    2008-12-01

    The risk of osseous bridge development after certain types of physeal injury is well established. Once formed, the bridge continues to grow and results in a progressive deformity. The authors present an unusual case of a five-year-old girl who had a Salter-Harris Type-IV fracture of the distal tibial epiphyseal plate, with subsequent osseous bridge formation and deformity development. The bridge resolved spontaneously in 16 months, and joint mechanical axis alignment was gradually restored with normal growth of the distal tibia. Key words: physis, paediatric fractures, bony bridge, growth arrest.

  14. Syndecan-3 in limb skeletal development.

    PubMed

    Kosher, R A

    1998-10-15

    Syndecan-3 is a member of a family of heparan sulfate proteoglycans that function as extracellular matrix receptors and as co-receptors for growth factors and signalling molecules. A variety of studies indicate that syndecan-3 is involved in several aspects of limb morphogenesis and skeletal development. Syndecan-3 participates in limb outgrowth and proliferation in response to the apical ectodermal ridge; mediates cell-matrix and/or cell-cell interactions involved in regulating the onset of chondrogenesis; may be involved in regulating the onset of osteogenesis and joint formation and, plays a role in regulating the proliferation of epiphyseal chondrocytes during endochondral ossification.

  15. Del(12)(p11.21p12.2) associated with an asphyxiating thoracic dystrophy or chondroectodermal dysplasia-like syndrome

    SciTech Connect

    Nagai, T.; Kato, R.; Hasegawa, T.

    1995-01-02

    We describe a 5-year-old Japanese boy who has some radiographic findings characteristic of asphyxiating thoracic dystrophy (ATD)-chondroectodermal dysplasia with a de novo chromosome abnormality. He also has mild mental retardation, short stature, hypoplastic hair and skin, oligodontia, small thoracic cage, hypoplastic pelvis and cone-shaped epiphyses of hands. On cytogenetic studies he was found to have a de novo del(12)(p11.21p12.2). These results suggest that the locus of the gene associated with ATD-chondroectodermal dysplasia may be situated at 12p11.21p12.2. 11 refs., 2 figs.

  16. Clinical and intraoral findings of a patient with tricho-rhino-phalangeal syndrome type I.

    PubMed

    Karacay, S; Saygun, I; Tunca, Y; Imirzalioglu, N; Guvenc, G

    2007-03-01

    Tricho-rhino-phalangeal syndrome (TRPS) is a rare and an autosomal dominant disorder having the following characteristics: slowly growing sparse hair, medially thick and laterally thin eyebrows, bulbous tip of the nose, long flat philtrum and thin upper lip with vermilion border, protruding ears, cone-shaped epiphyses and swelling. Our report intends to introduce TRPS to the dental literature and to present oral, clinical and radiological data of a patient with TRPS. A rare association of supernumerary teeth was also diagnosed and one of them was extracted as it impeded on the eruption path of left premolar tooth.

  17. Operative management of bilateral Salter-Harris type III fractures of the proximal phalanges of the great toes of a 10-year-old female ballet dancer: a case report.

    PubMed

    Csonka, Akos; Sikarinkul, Eakachit; Gargyan, Istvan; Boa, Kristof; Varga, Endre

    2016-07-01

    Differentiation between the normal variant cleft epiphysis and Salter-Harris type III fracture of the first proximal phalanges of the foot in children might be challenging. The authors describe a case of a 10-year-old ballet dancer girl with bilateral epiphyseal segmentation of the first proximal phalanges of the foot, unresponsive to conservative treatment. Considered a nonhealing stress-induced fracture, operative treatment with closed reduction and Herbert screw insertion was chosen on both sides. Complete union was achieved, with significant reduction of pain. The presented case suggests that internal fixation can be a viable option in the treatment of the problem.

  18. Magnetic resonance imaging in the evaluation of partial growth arrest after physeal injuries in children.

    PubMed

    Havránek, P; Lízler, J

    1991-09-01

    The precise delineation of the size, shape, and location of an osseous bridge is a critical step in the management of children who have a partial growth arrest of the epiphyseal plate. Five children between the ages of ten and fourteen years were diagnosed, with the aid of conventional roentgenograms, as having a partial growth arrest. Magnetic resonance-imaging studies were carried out to determine the exact size, shape, and location of the osseous bridge. The information derived from the imaging studies was essential for the determination of the appropriate treatment and for the planning and undertaking of any operative intervention.

  19. Opening-wedge osteotomy, allografting with dual buttress plate fixation for severe genu recurvatum caused by partial growth arrest of the proximal tibial physis: a case report.

    PubMed

    Chen, Liang-Chin; Chan, Yi-Sheng; Wang, Ching-Jen

    2004-07-01

    Injuries to the proximal tibial physis are among the least common epiphyseal injuries. We present a case of severe genu recurvatum deformity (45 degrees) with leg length discrepancy (4 cm) following a neglected proximal tibial physeal injury incurred 6 years previously. The 16-year-old patient was successfully treated by open-wedge osteotomy, allograft reconstruction, and dual buttress plate fixation. At 3 years' follow-up, the patient was asymptomatic, fully active with a full range of motion (0 - 140 degrees) of the leg, and equal leg lengths. There were no signs of genu recurvatum clinically.

  20. Experimental determinations of carcass processing by Plio-Pleistocene hominids and carnivores at FLK 22 (Zinjanthropus). Olduvai Gorge, Tanzania.

    PubMed

    Capaldo, S D

    1997-11-01

    Published and unpublished skeletal and surface mark data from the large, well-preserved, bovid dominated FLK 22 (Zinjanthropus) archaeofauna are analyzed using data derived from four different experimental control samples. The control samples are realistic because they are based on natural history and paleoecological data collected from FLK 22, and other Olduvai Gorge assemblages; they are precise because independent experimental studies following the same methods have generated the same results; and they restore generality to the study of site formation because each one models a different hominid and/or carnivore scenario of site formation. Comparability between FLK 22 and the control samples is established by excluding specimens from the former which do not meet identification and reporting standards derived from the latter. As in two previous studies, a comprehensive analysis of tooth marks and tool marks on long bone specimens from FLK 22 indicates that they were processed in three stages. In stage one, carnivores defleshed long bones, as inferred from the high percentage of tooth marks on midshaft fragments. In stage two, hominids processed intact long bones for marrow, as inferred from percussion mark percentages. Cut marks suggest that long bones retained flesh, but the amount, as yet, cannot be determined using cut mark percentages. In stage three, carnivores processed long bone epiphyses for grease, as inferred from the under-representation of long bone epiphyses and the high percentage of tooth marks on near-epiphyses and surviving epiphyses. The lack of comprehensive skeletal and surface mark data on cranial, axial, compact, and other specimens currently limits the application of experimental results. However, the available data suggest that the condition and representation of these items in the FLK 22 assemblage are also consistent with a carnivore to hominid to carnivore sequence of site formation. The variety of elements present, and their extensive

  1. Radiology of postnatal skeletal development. Pt. 6

    SciTech Connect

    McCarthy, S.M.; Ogden, J.A.

    1982-11-01

    Thirty-six pairs of proximal radioulnar and elbow units from cadavers and prepared skeletons ranging in age from full-term neonates to fourteen years, were studied morphologically and roentgenographically. Air/cartilage interfacing was used to demonstrate the osseous and cartilaginous portions of the developing epiphyses. These roentgenographic aspects are discussed and illustrated to provide a reference index. The skeletal development is outlined with regard to the diagnosis of several traumatic skeletal diseases as dislocation of elbow or radial head. Moteggia fracture dislocation and Nursemaid's elbow.

  2. Expression of transforming growth factor-beta 1 in normal and dyschondroplastic articular growth cartilage of the young horse.

    PubMed

    Henson, F M; Schofield, P N; Jeffcott, L B

    1997-11-01

    This study describes the distribution pattern of transforming growth factor-beta 1 (TGF-beta 1) mRNA and protein in normal pre- and post natal growth cartilage and alterations present in lesions of dyschondroplasia (osteochondrosis). TGF-beta 1 expression and immunoreactivity have been investigated by in situ hybridisation and immunolocalisation in the articular/epiphyseal growth cartilage of the lateral trochlear ridge of the distal femur. Cartilage was obtained from 19 normal Thoroughbred horses (5 prenatal and 14 post natal horses) and 15 post natal horses with dyschondroplasia (DCP). TGF-beta 1 mRNA expression and immunoreactivity were detected in the proliferative and upper hypertrophic zones in both pre- and post natal normal articular/epiphyseal cartilage. However, mRNA itself was only detected in the mid- and lower hypertrophic zones. Immunoreactivity was identified intracellularly with some nuclear staining observed. In focal lesions of DCP mRNA expression and immunoreactivity were reduced compared to normal cartilage, but strong mRNA expression was observed in the chondrocyte clusters immediately surrounding a lesion of DCP. The results described in this study demonstrate alterations in TGF-beta 1 dyschondroplastic lesions and indicate that it could be involved in the pathogenesis of this condition in the horse.

  3. The role of forensic medicine and forensic dentistry in estimating the chronological age of living individuals in Hamburg, Germany.

    PubMed

    Mansour, Hussam; Fuhrmann, Andreas; Paradowski, Ioana; van Well, Eilin Jopp; Püschel, Klaus

    2017-03-01

    Age estimation represents one of the primary responsibilities of forensic medicine and forensic dentistry. It is an integral procedure aiming to estimate the chronological age of an individual, whose age is either unknown or doubtful, by means of assessing the stage of dental, skeletal, and physical development. The present publication reviews the methods and procedures used in estimating the age of young living individuals as well as the experiences of the Institute of Legal Medicine in Hamburg-Eppendorf, Germany, during the last 25 years. From 1990 to 2015, 4223 age estimations were carried out in Hamburg. During this time, forensic age estimation was requested by different concerned authorities including courts, the foreigners' registration office (Zentrale Ausländerbehörde), and the state office of education and consultation (Landesbetrieb Erziehung und Beratung). In the context of judicial proceedings, orthopantomograms, as well as X-ray examinations of both the left hand and the medial clavicular epiphyses were carried out in accordance with AGFAD recommendations. For investigations not associated with judicial proceedings, orthopantomogram examinations play a key role in the process of age estimation, due to their high diagnostic value and low radiation exposure. Since 2009, mainly unaccompanied young refugees were examined for age estimation. Orthopantomograms and clinical-physical examinations have been used as essential steps in this context to determine whether an individual is 18 years or less. Additional X-ray examinations of the left hand and the medial clavicular epiphyses have been used less frequently.

  4. Osteoporosis in men with idiopathic hypogonadotropic hypogonadism

    SciTech Connect

    Finkelstein, J.S.; Klibanski, A.; Neer, R.M.; Greenspan, S.L.; Rosenthal, D.I.; Crowley, W.F. Jr.

    1987-03-01

    To assess the effect of testosterone deficiency on skeletal integrity in men, we determined bone density in 23 hypogonadal men with isolated gonadotropin-releasing hormone deficiency and compared those values with ones from controls. Cortical bone density, as assessed by single-photon absorptiometry of the nondominant radius, ranged from 0.57 to 0.86 g/cm2 (mean +/- SE, 0.71 +/- 0.02) in patients with fused epiphyses and from 0.57 to 0.67 g/cm2 (mean, 0.61 +/- 0.01) in patients with open epiphyses, both of which were significantly (p less than 0.001) lower than normal. Spinal trabecular bone density, as assessed by computed tomography, was similarly decreased (p less than 0.0001) and ranged from 42 to 177 mg K2HPO4/cm3 (mean, 112 +/- 7). Cortical bone density was at least 2 SD below normal in 16 of 23 men, and 8 men had spinal bone densities below the fracture threshold of 80 to 100 mg K2HPO4/cm3. Osteopenia was equally severe in men with immature and mature bone ages, suggesting that abnormal bone development plays an important role in the osteopenia of men with idiopathic hypogonadotropic hypogonadism.

  5. Kniest dysplasia is characterized by an apparent abnormal processing of the C-propeptide of type II cartilage collagen resulting in imperfect fibril assembly.

    PubMed Central

    Poole, A R; Pidoux, I; Reiner, A; Rosenberg, L; Hollister, D; Murray, L; Rimoin, D

    1988-01-01

    Epiphyseal and growth plate cartilages from four cases of Kniest dysplasia have been studied. In each case collagen fibril organization appeared abnormal by electron microscopy compared with age-matched normal cartilages: fibrils were much thinner, of irregular shape and did not exhibit the characteristic banding pattern. This was associated with the absence (compared with normal cartilage) of the C-propeptide of type II collagen (chondrocalcin) from the extracellular matrix of epiphyseal cartilages, although it was detected (as in normal cartilages) in the lower hypertrophic zone of the growth plate in association with calcifying cartilage. The C-propeptide was abnormally concentrated in intracellular vacuolar sites in Kniest cartilages and its total content was reduced in all cases but not in all cartilages. Moreover, it was not a part of the procollagen molecule. In contrast, type II collagen alpha-chain size was normal, indicating the formation of a triple helix. Also type II collagen content was normal and it was present in extracellular sites and only occasionally detected intracellularly. These observations suggest that the defect in Kniest dysplasia may result from the secretion of type II procollagen lacking the C-propeptide and abnormal fibril formation, and that the C-propeptide is normally required for fibril formation. Images PMID:3276736

  6. Age assessment by magnetic resonance imaging of the knee: a preliminary study.

    PubMed

    Dedouit, Fabrice; Auriol, Julien; Rousseau, Hervé; Rougé, Daniel; Crubézy, Eric; Telmon, Norbert

    2012-04-10

    The authors developed an original magnetic resonance imaging (MRI) staging system for epiphyseal fusion of growth plate maturation of the knee and evaluated its reliability and validity for age assessment of living individuals. A total of 290 MRI scans of the knee were reviewed retrospectively in patients aged from 10 to 30 years old (138 males, 152 females). Five original MRI stages were defined to assess the degree of maturation of the distal femoral and proximal tibial epiphyses. Intra-observer variability was excellent and inter-observer variability was good, demonstrating the reliability and the validity of this original MRI staging system. In both sexes, the changes of growth plates (proximal tibial or distal femoral) were associated with age (p<0.001). Our results agreed with classic data on skeletal maturation of the knee, with globally earlier maturation in females than in males, and also earlier maturation of the proximal tibial epiphysis than of the distal femoral epiphysis. MRI of the knee is an efficient non-invasive method of age assessment, without the disadvantage of X-ray exposure. Further studies with larger groups are needed to support our results.

  7. Morphological alterations in the growth plate cartilage of ovariectomized mice.

    PubMed

    Yao, Xianfeng; Chen, Huayue; Ohtake, Norihiro; Shoumura, Shizuko

    2006-12-01

    The effects of ovariectomy on growth and estrogen receptor (ER) expression level in the epiphyseal growth plate in mice have been estimated by histomorphometry and immunohistochemistry. Twelve female ddY mice, 8-9 weeks of age, were subjected to bilateral ovariectomy and 12 others were sham operated. They were then killed 8 weeks later. Ovariectomy significantly increased the total thickness of the distal femoral and proximal tibial growth plate cartilage. Ovariectomy caused a 1.4-fold increase in the thickness of the proliferative layer in the distal growth plate of the femur and a 1.3-fold increase in the thickness of the proliferative layer in the proximal growth plate of the tibia. ERalpha and ERbeta immunoreactivity was detected in chondrocytes of the growth plate and the expression level of ERs in epiphyseal plates was increased in ovariectomized mice compared with controls. These data suggest that ERalpha and ERbeta are coexpressed in the growth plates of the mice and that the cartilage growth and the level of expression of ERs in these tissues are hormonally regulated.

  8. Alpha-gamma equalization-enhanced hand radiographic image segmentation scheme

    NASA Astrophysics Data System (ADS)

    Lin, Hsiu-Hsia; Shu, San-Ging; Kuo, Shiau-Wei; Wang, Chien-Hsuan; Chan, Ya-Ping; Yu, Shyr-Shen

    2009-10-01

    Bone age assessment of children is a procedure frequently performed in pediatric radiology. The feature extractions of metaphyseal and epiphyseal regions are crucial to automatic bone age assessment. The first step of feature extraction is applying a segmentation scheme to find exact regions of epiphysis and metaphysis. A segmentation method is normally based on both intensity information and the relative location of pixels. There is a fundamental problem; when the intensity contrast of soft tissue and bony tissue is poor, bony and soft tissue cannot easily be separated. We propose an α-gamma equalization method to increase the intensity contrast between bony and soft tissue. Sobel, two-means, Canny edge-detection, and watershed methods are applied to illustrate the effect of this method on initial segmentation. Adaptive two-means and gradient vector flow snake are adopted for the final segmentation. Experimental results reveal that α-gamma equalization-enhanced two-means initial segmentation with an adaptive two-means clustering scheme can greatly reduce the average error measurements of segmentations. These are evaluated according to the following measurements: misclassification error, edge mismatch, region nonuniformity, relative foreground area eror, and modified Hausdorff distance. Furthermore, the experimental results show that the proposed scheme provides increased stable performance for the segmentation of epiphyseal/metaphyseal regions.

  9. In vitro effects of caffeine in growth cartilage of rats

    PubMed Central

    Reis, Amanda Maria Sena; Raad, Raquel Viana; Ocarino, Natália de Melo; Serakides, Rogéria

    2013-01-01

    Objective To evaluate the in vitro effetcs of caffeine on proliferation, apoptosis a nd gene transcripts expression of chondrogenic differentiation in growth cartilage. Methods The cartilaginous epiphyses of femurs of newborn rats, which were divided into two subgroups: treated with caffeine and control group, both observed over the time periods of 0, 7, 14 and 21 days. The cartilaginous epiphyses of femurs of each subgroup and each time span were subjected to histomorphometric, immunohistochemical analysis, Tunel technique and RT-PCR in real time. Results The decrease in proliferative activity and the increase of apoptotic chondroblasts at 21 days were found regardless of the subgroup. However, the decrease in cell proliferation caused by caffeine was lower than in the control group and significantly increased the expression of gene transcripts for chondrogenic differentiation, represented by Sox-9 and Runx-2. However, the in vitro culture with caffeine revealed antagonistic effects: despite the positive effect on chondroblasts proliferation and differentiation, caffeine increased apoptosis, characterized by increased expression of caspase 3 and of the number of cells undergoing apoptosis (p<0.05). Conclusion Caffeine presents antagonistic effects in vitro on growth cartilage, increasing the proliferation, differentiation and cell apoptosis. Experimental Study. PMID:24453686

  10. A Sheep Model for Cancellous Bone Healing

    PubMed Central

    Malhotra, Angad; Pelletier, Matthew Henry; Yu, Yan; Christou, Chris; Walsh, William Robert

    2014-01-01

    Appropriate well-characterized bone defect animal models remain essential for preclinical research. This pilot study demonstrates a relevant animal model for cancellous bone defect healing. Three different defect diameters (8, 11, 14 mm) of fixed depth (25 mm) were compared in both skeletally immature (18-month-old) and aged sheep (5-year-old). In each animal, four defects were surgically created and placed in the cancellous bone of the medial distal femoral and proximal tibial epiphyses bilaterally. Animals were euthanized at 4 weeks post-operatively to assess early healing and any biological response. Defect sites were graded radiographically, and new bone formation quantified using μCT and histomorphometry. Fibrous tissue was found within the central region in most of the defects with woven bone normally forming near the periphery of the defect. Bone volume fraction [bone volume (BV)/TV] significantly decreased with an increasing defect diameter. Actual BV, however, increased with defect diameter. Bone ingrowth was lower for all defect diameters in the aged group. This pilot study proposes that the surgical creation of 11 mm diameter defects in the proximal tibial and distal femoral epiphyses of aged sheep is a suitable large animal model to study early healing of cancellous bone defects. The refined model allows for the placement of four separate bone defects per animal and encourages a reduction in animal numbers required for preclinical research. PMID:25593961

  11. Mutations in the heat-shock protein A9 (HSPA9) gene cause the EVEN-PLUS syndrome of congenital malformations and skeletal dysplasia

    PubMed Central

    Royer-Bertrand, Beryl; Castillo-Taucher, Silvia; Moreno-Salinas, Rodrigo; Cho, Tae-Joon; Chae, Jong-Hee; Choi, Murim; Kim, Ok-Hwa; Dikoglu, Esra; Campos-Xavier, Belinda; Girardi, Enrico; Superti-Furga, Giulio; Bonafé, Luisa; Rivolta, Carlo; Unger, Sheila; Superti-Furga, Andrea

    2015-01-01

    We and others have reported mutations in LONP1, a gene coding for a mitochondrial chaperone and protease, as the cause of the human CODAS (cerebral, ocular, dental, auricular and skeletal) syndrome (MIM 600373). Here, we delineate a similar but distinct condition that shares the epiphyseal, vertebral and ocular changes of CODAS but also included severe microtia, nasal hypoplasia, and other malformations, and for which we propose the name of EVEN-PLUS syndrome for epiphyseal, vertebral, ear, nose, plus associated findings. In three individuals from two families, no mutation in LONP1 was found; instead, we found biallelic mutations in HSPA9, the gene that codes for mHSP70/mortalin, another highly conserved mitochondrial chaperone protein essential in mitochondrial protein import, folding, and degradation. The functional relationship between LONP1 and HSPA9 in mitochondrial protein chaperoning and the overlapping phenotypes of CODAS and EVEN-PLUS delineate a family of “mitochondrial chaperonopathies” and point to an unexplored role of mitochondrial chaperones in human embryonic morphogenesis. PMID:26598328

  12. Interrelationship of the Risser sign, knee epiphysis, and bone age in determining skeletal maturity: a case-control study.

    PubMed

    Kim, Hak Jun; Yoon, Jung-Ro; Modi, Chetna; Modi, Hitesh; Song, Hae-Ryong; Song, Sang-Youn

    2011-05-01

    The purpose of our study was to correlate the chronological age with Risser staging, knee epiphyseal closure, and bone age by the Tanner and Whitehouse (TW3) or Greulich and Pyle (GP) method simultaneously, to find out the most correlated methods used to calculate the age in a Korean population. A case-control study was carried out in 293 children between the age of 9 and 18 years. Skeletal age was estimated by using the atlas of the GP and TW3 methods; knee epiphysis closure and the Risser staging were also noted. Spearman's correlation coefficient test showed that in both the sexes the GP method is more correlated (r=0.58 for female patients, range: 0.55-0.61; and 0.58 for male patients, range: 0.54-0.61) with the Risser staging and physeal stages of the knee joint than the TW3 method (r=0.52 for female patients, range: 0.44-0.61; and 0.55 for male patients, range: 0.48-0.61) in Korean children. Our results suggested that by using the combination of Risser sign, knee epiphyseal closure, and GP bone age, one can calculate a person's chronological age most accurately.

  13. The pathophysiological role of PEDF in bone diseases.

    PubMed

    Broadhead, M L; Akiyama, T; Choong, P F M; Dass, C R

    2010-04-01

    First discovered in 1991 as a factor secreted by retinal pigment epithelial cells, the potency of pigment epithelium derived factor (PEDF) as an anti-angiogenic has led to examination of its role in active bone growth, repair and remodelling. In the musculoskeletal system, PEDF expression occurs particularly at sites of active bone formation. Expression has been noted in osteoblasts and to a lesser degree osteoclasts, the major classes of bone cells. In fact, PEDF is capable of inducing differentiation of precursor cells into mature osteoblasts. Expression and localisation are closely linked with that of vascular endothelial growth factor (VEGF). Studies at the epiphyseal plate have revealed that PEDF expression plays a key role in endochondral ossification, and beyond this may account for the epiphyseal plate's innate ability to resist neoplastic cell invasion. Collagen-1, the major protein in bone, is avidly bound by PEDF, implicating an important role played by this protein on PEDF function, possibly through MMP-2 and -9 activity. Surprisingly, the role of PEDF has not been evaluated more widely in bone disorders, so the challenge ahead lies in a more diverse evaluation of PEDF in various osteologic pathologies including osteoarthritis and fracture healing.

  14. Sports-related overuse injuries in children.

    PubMed

    Launay, F

    2015-02-01

    Increased intensity of sports activities combined with a decrease in daily physical activity is making overuse injuries in children more common. These injuries are located mainly in the epiphyseal cartilage. The broad term for these injuries is osteochondrosis, rather than osteochondritis, which more specifically refers to inflammatory conditions of bone and cartilage. The osteochondrosis may be epiphyseal, physeal, or apophyseal, depending on the affected site. The condition can either be in the primary deformans form or the dissecans form. While there is no consensus on the etiology of osteochondrosis, multiple factors seem to be involved: vascular, traumatic, or even microtraumatic factors. Most overuse injuries involve the lower limbs, especially the knees, ankle and feet. The most typical are Osgood-Schlatter disease and Sever's disease; in both conditions, the tendons remain relatively short during the pubescent grown spurt. The main treatment for these injuries is temporary suspension of athletic activities, combined with physical therapy in many cases. Surgery may be performed if conservative treatment fails. It is best, however, to try to prevent these injuries by analyzing and correcting problems with sports equipment, lifestyle habits, training intensity and the child's level of physical activity, and by avoiding premature specialization. Pain in children during sports should not be considered normal. It is a warning sign of overtraining, which may require the activity to be modified, reduced or even discontinued.

  15. [Total replacement of a middle phalanx by free non-vascularized chondral graft, after failure of sclerotherapy for treatment of an aneurysmal bone cyst].

    PubMed

    Salon, A; Rémi, J; Brunelle, F; Drapé, J L; Glorion, Ch

    2005-01-01

    We treated an eleven year-old boy for an aneurysmal bone cyst of the middle phalanx of the long finger. Diagnosis was established after total curettage. The tumor involved the whole phalanx and grew steadily after two attempts at sclerotherapy (with absolute alcohol and Ethibloc). After two years, en-bloc resection had to be performed, and raised the problem of reconstructing a complete finger phalanx with its proximal and distal epiphyses. A free cartilaginous graft from the non-ossified iliac crest was shaped to the exact dimensions of the phalanx and set in its place, with minimal damage to the surrounding tissues during dissection and fixation. By six months an almost normal range of motion was achieved in the PIP (10 to 90 degrees ) and DIP (5 to 30 degrees) joints and radiographs showed complete metaplasia of the chondral graft into an ossified phalanx at 20 months follow-up. The joint spaces also remodelled, and this was confirmed with MRI scanning. Reports on partial replacement of diaphysis or epiphyses in the digits are discussed, but the only valid comparison of total phalanx replacement is free toe phalanx grafting. We did not choose this solution in a normal hand because of the length discrepancy between finger and toe phalanges. This case shows that, in this particular paediatric situation, the free non-vascularised transfer of a chondral graft restored excellent function, with remodelling of the phalanx and joint spaces of the finger.

  16. Estimation of forensic age using substages of ossification of the medial clavicle in living individuals.

    PubMed

    Ekizoglu, Oguzhan; Hocaoglu, Elif; Inci, Ercan; Can, Ismail Ozgur; Aksoy, Sema; Sayin, Ibrahim

    2015-11-01

    Forensic age estimation based on staging of ossification of the medial clavicular bone is one of the methods recommended by the Study Group on Forensic Age Diagnostics of the German Association of Forensic Medicine. In the present study, we analyzed the stages of ossification of the medial clavicular epiphyses on thin-sliced (1 mm) computed tomography (CT) images using the substages defined within stages 2 and 3. The retrospective CT analysis involved 193 subjects (129 males, 64 females) ranging in age from 13 to 28 years. Spearman's correlation analysis revealed a positive correlation between age and ossification stage in both male and female subjects. Stage 3c was first observed at 19 years of age in both sexes and may thus serve as a valuable forensic marker for determining an age of 18 years. Although further research is needed on the ossification stages of the medial clavicular epiphyses, the present findings could contribute to existing reports on observers' experiences using CT analysis of ossification combined with analysis of substages.

  17. Two new cestode species of Neoskrjabinolepis Spasskii, 1947 (Cyclophyllidea: Hymenolepididae) from the tundra shrew Sorex tundrensis Merriam (Mammalia: Soricidae) in Alaska and Chukotka.

    PubMed

    Kornienko, Svetlana A; Dokuchaev, Nikolaj E

    2012-11-01

    Descriptions, illustrations and differential diagnoses for two new species of Neoskrjabinolepis Spassky, 1947, i.e. N. (Neoskrjabinolepis) fertilis n. sp. and N. (Neoskrjabinolepidoides) hobergi n. sp., are given. Neoskrjabinolepis (Neoskrjabinolepis) fertilis was found in the shrew Sorex tundrensis Merriam on the Seward Peninsula (Alaska, USA) and from the mouth of the Anadyr River (Chukotka, Russia), and N. (Neoskrjabinolepidoides) hobergi was found in Sorex tundrensis on the Seward Peninsula (Alaska, USA). Neoskrjabinolepis fertilis n. sp. is characterised by having: rostellar hooks 38-42 μm long and provided with a small epiphyseal thickening of the handle; a long cirrus (85-100 μm), consisting of a basal region with claw-shaped spines and a parabasal region with small, thin needle-shaped spines; a cirrus-sac extending well into the median field; and 55-70 eggs per gravid uterus. Neoskrjabinolepis (Neoskrjabinolepidoides) hobergi n. sp. is characterised by having: rostellar hooks 63-65 μm long and provided with a large epiphyseal thickening of the handle; a short cirrus (45-50 μm), consisting of a basal region with small claw-shaped spines and a parabasal region with thin, needle-shaped spines; cirrus-sac reaching slightly into the median field; and 36-45 eggs per gravid uterus.

  18. The development of a mature collagen network in cartilage from human bone marrow stem cells in Transwell culture.

    PubMed

    Murdoch, Alan D; Hardingham, Timothy E; Eyre, David R; Fernandes, Russell J

    2016-03-01

    Damaged hyaline cartilage shows a limited capacity for innate repair. Potential sources of cells to augment the clinical repair of cartilage defects include autologous chondrocytes and mesenchymal stem cells. We have reported that culture of human bone marrow mesenchymal stem cells with specific growth and differentiation factors as shallow multilayers on Transwell permeable membranes provided ideal conditions for chondrogenesis. Rigid translucent cartilaginous disks formed and expressed cartilage-specific structural proteins aggrecan and type II collagen. We report here the analysis of the collagen network assembled in these cartilage constructs and identify key features of the network as it became mature during 28 days of culture. The type II collagen was co-polymerized with types XI and IX collagens in a fibrillar network stabilized by hydroxylysyl pyridinoline cross-links as in epiphyseal and hyaline cartilages. Tandem ion-trap mass-spectrometry identified 3-hydroxylation of Proline 986 and Proline 944 of the α1(II) chains, a post-translational feature of human epiphyseal cartilage type II collagen. The formation of a type II collagen based hydroxy-lysyl pyridinoline cross-linked network typical of cartilage in 28 days shows that the Transwell system not only produces, secretes and assembles cartilage collagens, but also provides all the extracellular mechanisms to modify and generate covalent cross-links that determine a robust collagen network. This organized assembly explains the stiff, flexible nature of the cartilage constructs developed from hMSCs in this culture system.

  19. Growth hormone induces multiplication of the slowly cycling germinal cells of the rat tibial growth plate.

    PubMed

    Ohlsson, C; Nilsson, A; Isaksson, O; Lindahl, A

    1992-10-15

    To study the effect of locally infused growth hormone (GH) or insulin-like growth factor I(IGF-I) on slowly cycling cells in the germinal cell layer of the tibial growth plate, osmotic minipumps delivering 14.3 microCi of [3H]thymidine per day were implanted s.c. into hypophysectomized rats, and GH (1 microgram) or IGF-I (10 micrograms) was injected daily through a cannula implanted in the proximal tibia. The opposite leg served as a control. After 12 days of treatment, the osmotic minipumps were removed, and three rats in each group were given GH (20 micrograms/day, s.c.) for an additional 14 days to chase the labeled cells out of the proliferative layers. Labeled cells remained in the germinal layer, in the perichondrial ring, and on the surface of the articular cartilage close to the epiphyseal plate. GH administered together with labeled thymidine significantly increased the number of labeled cells in the germinal cell layer compared to that in the control leg (ratio = 1.95 +/- 0.13), whereas IGF-I showed no stimulatory effect (ratio = 0.96 +/- 0.04). Therefore GH but not IGF-I stimulates the multiplication of the slowly cycling (label-retaining) cells in the germinal layer of the epiphyseal plate. IGF-I acts only on the proliferation of the resulting chondrocytes.

  20. In vivo demonstration of cell types in bone that harbor epidermal growth factor receptors

    SciTech Connect

    Martineau-Doize, B.; Lai, W.H.; Warshawsky, H.; Bergeron, J.J.

    1988-08-01

    The binding and internalization of (/sup 125/I)iodoepidermal growth factor (EGF) by bone cells of the rat was demonstrated in situ by quantitative radioautography. Specific binding sites were observed on a cell profile enriched in endocytic components, including lysosome-like structures, a rough endoplasmic reticulum-rich cell profile, and a cell profile that histologically resembles an undifferentiated precursor cell. By the criteria of gel filtration and precipitability by trichloroacetic acid, most of the bound (/sup 125/I)iodo-EGF was considered intact. By morphological criteria none of the cell profiles that bound (/sup 125/I)iodo-EGF corresponded to fully formed osteoclasts or osteoblasts. The endocytic cell was found in the epiphyseal plate between the invading capillary and the transverse and longitudinal cartilage septa as well as near osteoclasts in the zone of mixed spicules. The rough endoplasmic reticulum-rich cell was present in vacated chondrocyte lacunae of the epiphyseal plate close to the metaphysis, and the poorly differentiated cell was observed between the mixed spicules of the metaphysis. Similar cell types were also found in the alveolar bone surrounding the incisors. These cells may be the origin of established bone cell lines that harbor high concentrations of EGF receptors and may also be responsible for the humoral hypercalcemia in response to the reported actions of injected EGF or transforming growth factor-alpha as well as that of malignancy.

  1. Morphological analysis of the flippers in the Franciscana dolphin, Pontoporia blainvillei, applying X-ray technique.

    PubMed

    Del Castillo, Daniela Laura; Panebianco, María Victoria; Negri, María Fernanda; Cappozzo, Humberto Luis

    2014-07-01

    Pectoral flippers of cetaceans function to provide stability and maneuverability during locomotion. Directional asymmetry (DA) is a common feature among odontocete cetaceans, as well as sexual dimorphism (SD). For the first time DA, allometry, physical maturity, and SD of the flipper skeleton--by X-ray technique--of Pontoporia blainvillei were analyzed. The number of carpals, metacarpals, phalanges, and morphometric characters from the humerus, radius, ulna, and digit two were studied in franciscana dolphins from Buenos Aires, Argentina. The number of visible epiphyses and their degree of fusion at the proximal and distal ends of the humerus, radius, and ulna were also analyzed. The flipper skeleton was symmetrical, showing a negative allometric trend, with similar growth patterns in both sexes with the exception of the width of the radius (P ≤ 0.01). SD was found on the number of phalanges of digit two (P ≤ 0.01), ulna and digit two lengths. Females showed a higher relative ulna length and shorter relative digit two length, and the opposite occurred in males (P ≤ 0.01). Epiphyseal fusion pattern proved to be a tool to determine dolphin's age; franciscana dolphins with a mature flipper were, at least, four years old. This study indicates that the flippers of franciscana dolphins are symmetrical; both sexes show a negative allometric trend; SD is observed in radius, ulna, and digit two; and flipper skeleton allows determine the age class of the dolphins.

  2. An extremely rare case: osteosclerotic metaphyseal dysplasia.

    PubMed

    Kasapkara, C S; Küçükçongar, A; Boyunağa, O; Bedir, T; Oncü, F; Hasanoğlu, A; Tümer, L

    2013-01-01

    OMD (osteosclerotic metaphyseal dysplasia) is a very rare sclerosing bone disorder, first described by G. Nishimura in two Japanese siblings in 1993 (6). We report the case of a 12-month-old male with hypotonia, developmental delay and sclerosis of the metaphyses and epiphyses of specific bones. This 36-week gestation boy was born to a 26 year old gravida 5 para 1 Turkish mother and a 27 year old nonconsanguineous father. Radiographic findings obtained during the hospital stay included bilateral symmetrical osteosclerosis of the metaphyseal portions of the long bones in the upper and lower extremities with osteopenic shafts. Narrow bands of metaphyseal osteosclerosis were detected in the short tubular bones of the hands and feet. Growing parts of bilateral scapula, iliac, pubic and ischial bones show sclerotic bands. In addition superior and inferior plates of vertebras, transverse processes of sacral vertebras, all visible epiphyses, carpal and tarsal bones also show sclerotic changes. The scalp was unaffected. Based on the clinical, radiographic, and laboratory findings, a diagnosis of OMD was made. We do not know any of the osteosclerotic bone disorder with changes including hypotonia, mental and motor developmental delay and metaphyseal sclerosis of the bones with a unique distribution except OMD. The syndrome is characterized by developmental delay of a progressive nature, hypotonia, elevated alkaline phosphatase, and late-onset spastic paraplegia 18 years ago. Our patient is the 4th case of OMD described in the literature share some clinical and radiological similarities with other three reported cases of osteosclerotic metaphyseal dysplasias.

  3. The time of onset of abnormal calcification in spondylometaepiphyseal dysplasia, short limb-abnormal calcification type.

    PubMed

    Tüysüz, Beyhan; Gazioğlu, Nurperi; Ungür, Savaş; Aji, Dolly Yafet; Türkmen, Seval

    2009-01-01

    A 1-month-old boy with shortness of extremities on prenatal US was referred to our department with a provisional diagnosis of achondroplasia. His height was normal but he had short extremities and platyspondyly, premature carpal epiphyses on both hands, and short tubular bones with irregular metaphyses on radiographs. Re-evaluation of the patient at the age of 1 year revealed very short height and premature calcification of the costal cartilages and epiphyses. Spondylometaepiphyseal dysplasia (SMED), short limb-abnormal calcification type was diagnosed. This condition is a very rare autosomal recessively inherited disorder, and most of the patients die in early childhood due to neurological involvement. At the age of 2 years and 5 months, a CT scan showed narrowing of the cervical spinal canal. One month later he died suddenly because of spinal cord injury. In conclusion early diagnosis is very important because the recurrence risk is high and patients may die due to early neurological complications. The time of onset of abnormal calcifications, a diagnostic finding of the disease, is at the age of around 1 year in most patients. When abnormal calcifications are not yet present, but radiological changes associated with SMED are present, this rare disease must be considered.

  4. Physeal fractures, part I: histologic features of bone, cartilage, and bar formation in a small animal model.

    PubMed

    Wattenbarger, J Michael; Gruber, Helen E; Phieffer, Laura S

    2002-01-01

    Physeal fractures and the formation of physeal bars can pose significant problems in skeletal development for the injured, growing child. Regrettably, little experimental attention has been directed toward this clinical disturbance. The current study documents early histologic changes (days 2-6) and subsequent alterations (day 21) following a physeal fracture in the rat proximal tibia model. The fracture plane was usually contained within the physis but could involve many regions of the physis. In some instances, the fracture plane extended to the physeal epiphyseal border. When the fracture was contained within the physis, healing was uneventful. However, when the fracture extended through the physis to the epiphyseal physeal border, there was greater physeal disorganization and formation of vertical septa leading to physeal bars. Physeal bars appeared to form at sites of vertical fibrotic septa into which marrow cells, osteoclasts, and osteoblasts had migrated. Bar formation mediated by primary osteogenesis (rather than by endochondral bone formation) followed. This study examines the changes in the histologic features of the rat proximal tibial physis, epiphysis, and metaphysis after a physeal fracture and identifies key factors associated with physeal bar formation.

  5. Expression of tenascin in joint-associated tissues during development and postnatal growth.

    PubMed

    Mackie, E J; Ramsey, S

    1996-02-01

    The extracellular matrix protein, tenascin, is selectively expressed in a variety of connective tissues during development. In this study, the distribution of tenascin in tissues contributing to the knee joint during embryonic development and postnatal growth in the rat has been investigated by immunohistochemistry. In recently formed embryonic knee joints, tenascin expression was abundant in the territorial matrix of superficial articular cartilage. Site of attachment of cruciate and patellar ligaments to cartilage were strongly stained; staining of ligaments weakened with distance from the attachment site. In rapidly growing 4-wk-old rats, tenascin was present in a fine line on the surface of articular cartilage, but at 10 wk of age tenascin staining was absent from most of the articular surface. In postnatal rats, there was strong tenascin staining of the synovial lining, but not of subintimal tissue. Cruciate ligaments were histologically fibrocartilaginous in 4 and 10-wk-old rats; within these ligaments strong pericellular tenascin staining was seen in association with rounded chondrocyte-like cells. Tenascin was absent from the cartilaginous growth plates of 4 and 10-wk-old rats, but intense tenascin staining was seen at the junction between epiphyseal bone and growth plate. Within the metaphysis, tenascin staining on bone surfaces increased with distance from the hypertrophic chondrocytes. Osteocytes within epiphyseal trabecular bone were strongly stained for tenascin, whereas those in the metaphysis were mostly unstained. The results presented here demonstrate that tenascin expression in joint-associated tissues changes markedly with cell type and stage of differentiation.

  6. Effects of Multiple Drilling on the Ischemic Capital Femoral Epiphysis of Immature Piglets

    PubMed Central

    Gong, Sun Young; Kim, Hyun Woo; Park, Hui-Wan; Lee, Seung Yup

    2011-01-01

    Purpose This study investigated the effects of multiple drilling on the immature capital femoral epiphysis following ischemic injury in a piglet model. Materials and Methods Ischemic necrosis of capital femoral epiphysis was induced bilaterally in 12 piglets using a cervical ligation method. Three weeks later, medial, central, and lateral 3 drill holes were made on the left femoral head using 0.062" K-wire. At 3, 6, 9, and 12 weeks following the multiple drilling, femoral heads were harvested from each three piglets. On histologic examination, percent of revascularization, percent of osteoblast surface, capital femoral epiphyseal quotient and proximal femoral growth plate height were evaluated. Untreated right femoral heads served as control. Results While percent of revascularization of left capital femoral epiphysis with multiple drilling was significantly higher than untreated control side (p<0.001), percent of osteoblast surface, capital femoral epiphyseal quotient and proximal femoral growth plate height showed no significant difference. Conclusion This study indicates that multiple drilling could promote revascularization of ischemic capital femoral epiphysis, and multiple drilling does not appear to produce bony physeal bars at short-term, if using small diameter drill. However, multiple drilling alone does not seem to prevent femoral head deformity or to promote new bone formation. PMID:21786447

  7. Spaceflight-induced bone loss alters failure mode and reduces bending strength in murine spinal segments.

    PubMed

    Berg-Johansen, Britta; Liebenberg, Ellen C; Li, Alfred; Macias, Brandon R; Hargens, Alan R; Lotz, Jeffrey C

    2016-01-01

    Intervertebral disc herniation rates are quadrupled in astronauts following spaceflight. While bending motions are main contributors to herniation, the effects of microgravity on the bending properties of spinal discs are unknown. Consequently, the goal of this study was to quantify the bending properties of tail discs from mice with or without microgravity exposure. Caudal motion segments from six mice returned from a 30-day Bion M1 mission and eight vivarium controls were loaded to failure in four-point bending. After testing, specimens were processed using histology to determine the location of failure, and adjacent motion segments were scanned with micro-computed tomography (μCT) to quantify bone properties. We observed that spaceflight significantly shortened the nonlinear toe region of the force-displacement curve by 32% and reduced the bending strength by 17%. Flight mouse spinal segments tended to fail within the growth plate and epiphyseal bone, while controls tended to fail at the disc-vertebra junction. Spaceflight significantly reduced vertebral bone volume fraction, bone mineral density, and trabecular thickness, which may explain the tendency of flight specimens to fail within the epiphyseal bone. Together, these results indicate that vertebral bone loss during spaceflight may degrade spine bending properties and contribute to increased disc herniation risk in astronauts.

  8. Metatarsal fusion pattern and developmental morphology of the Olduvai Hominid 8 foot: Evidence of adolescence.

    PubMed

    Susman, Randall L; Patel, Biren A; Francis, Megan J; Cardoso, Hugo F V

    2011-01-01

    The morphology of the Olduvai Hominid (OH) 8 foot and the sequence of metatarsal epiphyseal fusion in modern humans and chimpanzees support the hypothesis that OH 8 belonged to an individual of approximately the same relative age as the OH 7 subadult, the holotype of Homo habilis. Modern humans and chimpanzees exhibit a variety of metatarsal epiphyseal fusion patterns, including one identical to that observed in OH 8 in which metatarsal 1 fuses before metatarsals 2-5. More than the metatarsal fusion sequence, however, the principal evidence of the youthful age of OH 8 lies in the morphology of metatarsals 1, 2, and 3. Because both OH 8 and OH 7 come from the same stratum at the FLK NN type site, the most parsimonious explanation of the OH 8 and OH 7 data is that this material belonged to the same individual, as originally proposed by Louis Leakey. The proposition that OH 8 belonged to an adult is unsupported by morphology, including radiographic evidence, and the fusion sequences in human and chimpanzee skeletal material reported here and in the literature.

  9. Sex determination by discriminant function analysis of the right tibia in the prehispanic population of the Canary Islands.

    PubMed

    González-Reimers, E; Velasco-Vázquez, J; Arnay-de-la-Rosa, M; Santolaria-Fernández, F

    2000-02-28

    This study has been performed in order to define standards usable to determine the sex of prehispanic individuals from the Canary Islands from their skeletal remains. Osteometric information at the right tibia was obtained from 59 complete skeletons from Gran Canaria, housed in the Museo Canario (Las Palmas), 45 males and 14 females (this constitutes the totality of complete prehispanic skeletons known from Gran Canaria). The parameters measured were: tibial length, proximal and distal epiphyseal breadth, transverse and anteroposterior diameter, perimeter at the nutrition foramen levels and minimum shaft perimeter. These parameters were subjected to different SPSS discriminant function analysis, combining all of them, or only the proximal or distal ones, without tibial length, etc., in order to obtain functions usable even if only bone fragments are available. Transverse diameter, proximal epiphyseal breadth and minimum shaft perimeter showed the highest discriminant power. The functions obtained showed high average accuracies, ranging from 94.9 to 98.3%, with female accuracies of 100%. The functions obtained were further applied to a test prehispanic population (ten males and ten females) from El Hierro. Overall accuracies of the functions when applied to this population ranged from 65 to 94.7%, with female accuracies ranging 80% to 100%.

  10. Rapid Activation of Transforming Growth Factor β–Activated Kinase 1 in Chondrocytes by Phosphorylation and K63‐Linked Polyubiquitination Upon Injury to Animal Articular Cartilage

    PubMed Central

    Ismail, Heba M.; Didangelos, Athanasios; Vincent, Tonia L.

    2017-01-01

    Objective Mechanical injury to cartilage predisposes to osteoarthritis (OA). Wounding of the articular cartilage surface causes rapid activation of MAP kinases and NF‐κB, mimicking the response to inflammatory cytokines. This study was undertaken to identify the upstream signaling mechanisms involved. Methods Cartilage was injured by dissecting it from the articular surface of porcine metacarpophalangeal (MCP) joints or by avulsing murine proximal femoral epiphyses. Protein phosphorylation was assayed by Western blotting of cartilage lysates. Immunolocalization of phosphorylated activating transcription factor 2 (ATF‐2) and NF‐κB/p65 was detected by confocal microscopy. Messenger RNA (mRNA) was measured by quantitative reverse transcriptase–polymerase chain reaction (qRT‐PCR). Receptor associated protein 80 (RAP‐80) ubiquitin interacting motif agarose was used in a pull‐down assay to obtain K63‐polyubiquitinated proteins. Ubiquitin linkages on immunoprecipitated transforming growth factor β–activated kinase 1 (TAK‐1) were analyzed with deubiquitinases. Results Sharp injury to porcine cartilage caused rapid activation of JNK and NF‐κB pathways and the upstream kinases MKK‐4, IKK, and TAK‐1. Pharmacologic inhibition of TAK‐1 in porcine cartilage abolished JNK and NF‐κB activation and reduced the injury‐dependent inflammatory gene response. High molecular weight species of phosphorylated TAK‐1 were induced by injury, indicating its ubiquitination. An overall increase in K63‐linked polyubiquitination was detected upon injury, and TAK‐1 was specifically linked to K63‐ but not K48‐polyubiquitin chains. In mice, avulsion of wild‐type femoral epiphyses caused similar intracellular signaling that was reduced in cartilage‐specific TAK‐1–null mice. Epiphyseal cartilage of MyD88‐null and TRAF‐6–null mice responded to injury, suggesting the involvement of a ubiquitin E3 ligase other than TRAF‐6. Conclusion

  11. The anatomy of the proximal tibia in pediatric and adolescent patients: implications for ACL reconstruction and prevention of physeal arrest.

    PubMed

    Shea, Kevin G; Apel, Peter J; Pfeiffer, Ronald P; Traughber, Paul D

    2007-04-01

    Although the treatment of anterior cruciate ligament (ACL) tears in skeletally immature patients is still controversial, several studies have advocated ACL reconstruction in selected patients to prevent secondary injury. The proximal tibial physis is a structure at risk during ACL reconstruction in young patients, and physeal growth complications have been reported after surgery in this area. The relationship between the ACL and the proximal tibial physeal/apophyseal regions is poorly understood. This study examined the MRI anatomy of the ACL and the proximal tibia apophysis and epiphysis. MRIs of 59 skeletally immature knees were reviewed (Average age = 12.75 years, range 6-15) to define the anatomy of the epiphyseal and apophyseal regions. Measurements were recorded in three parasagittal planes: (1) at the lateral border of the patellar tendon, (2) the lateral edge of the ACL insertion, and (3) the medial edge of the ACL insertion. A single three-dimensional (3D) computed tomography (CT) scan was used to evaluate the position of standard drill holes used in ACL reconstruction to assess for potential degree of injury to the epiphyseal and apophyseal growth plates. In the parasagittal planes, the average height of the epiphysis was 19.6, 20.7, and 21.5 mm at the lateral border of the patellar tendon, the lateral border of the ACL, and the medial border of the ACL, respectively. At the level of the same landmarks, the apophysis extended below the physis at an average of 20.2, 16.8, and 7.0 mm, respectively. Expressed as a percentage of epiphysis height this was an average of 104, 82, and 33%, respectively. Examination of 3D CT images revealed that variations in drill hole placement had effects on the volume of injury to the proximal tibial physis and apophysis. Drill holes that started more medial, distal, and with a steeper angle of inclination reduced the amount of physis and apophysis violated when compared with holes placed more lateral, proximal, and with a

  12. Femoral Head Bone Loss Following Short and Long-Duration Spaceflight

    NASA Technical Reports Server (NTRS)

    Blaber, E. A.; Cheng-Campbell, M.; Almeida, E. A. C.

    2016-01-01

    Exposure to mechanical unloading during spaceflight is known to have significant effects on the musculoskeletal system. Our ongoing studies with the mouse bone model have identified the failure of normal stem cell-based tissue regeneration, in addition to tissue degeneration, as a significant concern for long-duration spaceflight, especially in the mesenchymal and hematopoietic tissue lineages. The 30-day BionM1 and the 37-day Rodent Research 1 (RR1) missions enabled the possibility of studying these effects in long-duration microgravity experiments. We hypothesized that the inhibition of stem cell-based tissue regeneration in short-duration spaceflight would continue during long-duration spaceflight and furthermore would result in significant tissue alterations. MicroCT analysis of BionM1 femurs revealed 31% decrease in bone volume ratio, a 14% decrease in trabecular thickness, and a 20% decrease in trabecular number in the femoral head of space-flown mice. Furthermore, high-resolution MicroCT and immunohistochemical analysis of spaceflight tissues revealed a severe disruption of the epiphyseal boundary, resulting in endochondral ossification of the femoral head and perforation of articular cartilage by bone. This suggests that spaceflight in microgravity may cause rapid induction of an aging-like phenotype with signs of osteoarthritic disease in the hip joint. However, mice from RR1 exhibited significant bone loss in the femoral head but did not exhibit the severe aging and disease-like phenotype observed during BionM1.This may be due to increased physical activity in the RH hardware. Immunohistochemical analysis of the epiphyseal plate and investigation of cellular proliferation and differentiation pathways within the marrow compartment and whole bone tissue is currently being conducted to determine alterations in stem cell-based tissue regeneration between these experiments. Our results show that the observed inhibition of stem cell-based tissue regeneration

  13. Femoral Head Bone Loss Following Short and Long-Duration Spaceflight

    NASA Technical Reports Server (NTRS)

    Blaber, Elizabeth A.; Cheng-Campbell, Margareth A.; Almeida, Eduardo A. C.

    2016-01-01

    Exposure to mechanical unloading during spaceflight is known to have significant effects on the musculoskeletal system. Our ongoing studies with the mouse bone model have identified the failure of normal stem cell-based tissue regeneration, in addition to tissue degeneration, as a significant concern for long-duration spaceflight, especially in the mesenchymal and hematopoietic tissue lineages. The 30-day BionM1 and the 37-day Rodent Research 1 (RR1) missions enabled the possibility of studying these effects in long-duration microgravity experiments. We hypothesized that the inhibition of stem cell-based tissue regeneration in short-duration spaceflight would continue during long-duration spaceflight and furthermore would result in significant tissue alterations. MicroCT analysis of BionM1 femurs revealed 31 decrease in bone volume ratio, a 14 decrease in trabecular thickness, and a 20 decrease in trabecular number in the femoral head of space-flown mice. Furthermore, high-resolution MicroCT and immunohistochemical analysis of spaceflight tissues revealed a severe disruption of the epiphyseal boundary, resulting in endochondral ossification of the femoral head and perforation of articular cartilage by bone. This suggests that spaceflight in microgravity may cause rapid induction of an aging-like phenotype with signs of osteoarthritic disease in the hip joint. However, mice from RR1 exhibited significant bone loss in the femoral head but did not exhibit the severe aging and disease-like phenotype observed during BionM1. This may be due to increased physical activity in the RH hardware. Immunohistochemical analysis of the epiphyseal plate and investigation of cellular proliferation and differentiation pathways within the marrow compartment and whole bone tissue is currently being conducted to determine alterations in stem cell-based tissue regeneration between these experiments. Our results show that the observed inhibition of stem cell-based tissue regeneration

  14. Osteomalacia.

    PubMed

    Francis, R M; Selby, P L

    1997-04-01

    Osteomalacia is a generalized bone disorder characterized by impairment of mineralization, leading to accumulation of unmineralized matrix or osteoid in the skeleton. The classical clinical features of osteomalacia include musculoskeletal pain, skeletal deformity, muscle weakness and symptomatic hypocalcaemia. In childhood the features of osteomalacia are accompanied by rickets, with widening of the epiphyses and impaired skeletal growth. The major cause of osteomalacia is vitamin D deficiency, which is most often due to reduced cutaneous production of vitamin D in housebound elderly people, immigrants to Northern countries and women who adopt strict dress codes which prohibit exposure of uncovered skin. Vitamin D deficiency osteomalacia may also occur with malabsorption, liver disease and anticonvulsant therapy. Less commonly, osteomalacia may result from abnormal vitamin D metabolism, resistance to the action of vitamin D, hypophosphataemia or toxic effects on osteoblast function.

  15. Short adolescence in early hominids: infantile and adolescent growth of the human femur.

    PubMed

    Tardieu, C

    1998-10-01

    Did the first hominids have a short developmental period similar to that of the great apes or a longer period closer to that of modern humans? Evidence from studies on dental and facial growth favors the first point of view. Additional evidence presented in this report is provided by a morphogenetic analysis of the lower limb. Some morphological modifications undergone by the human femur during infantile and adolescent growth are shown to be excellent markers of different developmental stages. The angular remodelling of the femoral diaphysis, which results in femoral bicondylar angle, is a marker of infancy, while the reshaping of the distal femoral epiphysis is a marker of adolescence. This reshaping of the bony epiphysis consists of the strong projection of the external lip of the femoral trochlea, the increase of the radius of curvature of the external condyle, and the anteroposterior lengthening of the whole epiphysis. The growth spurt in linear dimensions of the femur, characteristic of human adolescence, is shown to be associated with qualitative changes of the distal femoral epiphysis engendered by the late closure of the distal epiphysis. The femur of the first hominids (Australopithecus afarensis) shows only features of infantile growth, whereas characters of both precocious and later growth are typical of later hominids (Homo). The absence of the derived epiphyseal features in Australopithecus would be linked to their early epiphyseal closure and short adolescent growth period; their presence in Homo would have been promoted by their delayed epiphyseal closure and prolonged adolescent growth period. The transition from Australopithecus to Homo appears to have involved a heterochronic process of time hypermorphosis (Gould, [1977], Ontogeny and Phylogeny [Cambridge: Harvard University Press]) in which the size of the femur increases, the epiphysis is modified, and the period of peripubertal growth is prolonged. The shape of the distal epiphyses of KNM

  16. Modifying bone scaffold architecture in vivo with permanent magnets to facilitate fixation of magnetic scaffolds.

    PubMed

    Panseri, S; Russo, A; Sartori, M; Giavaresi, G; Sandri, M; Fini, M; Maltarello, M C; Shelyakova, T; Ortolani, A; Visani, A; Dediu, V; Tampieri, A; Marcacci, M

    2013-10-01

    The fundamental elements of tissue regeneration are cells, biochemical signals and the three-dimensional microenvironment. In the described approach, biomineralized-collagen biomaterial functions as a scaffold and provides biochemical stimuli for tissue regeneration. In addition superparamagnetic nanoparticles were used to magnetize the biomaterials with direct nucleation on collagen fibres or impregnation techniques. Minimally invasive surgery was performed on 12 rabbits to implant cylindrical NdFeB magnets in close proximity to magnetic scaffolds within the lateral condyles of the distal femoral epiphyses. Under this static magnetic field we demonstrated, for the first time in vivo, that the ability to modify the scaffold architecture could influence tissue regeneration obtaining a well-ordered tissue. Moreover, the association between NdFeB magnet and magnetic scaffolds represents a potential technique to ensure scaffold fixation avoiding micromotion at the tissue/biomaterial interface.

  17. Cartilage Canals in Newborn Dogs: Histochemical and Immunohistochemical Findings

    PubMed Central

    Di Giancamillo, A.; Andreis, M.E.; Taini, P.; Veronesi, M.C.; Di Giancamillo, M.; Modina, S.C.

    2016-01-01

    Cartilage canals (CCs) are microscopic structures involved in secondary ossification centers (SOCs) development. The features of CCs were investigated in the humeral and femoral proximal epiphyses of small-sized newborn dogs (from premature to 28 days after birth) with histochemical and immunohistochemical approaches. Masson’s Trichrome revealed a ring-shaped area around CCs, which changes in colour from green (immature collagen) to red (mature collagen) as ossification progresses; perichondrium staining always matched the ring color. Safranin-O was always negative. Immunohistochemical analysis revealed immunopositivity for both collagen type I and V around the CCs; collagen type II was negative. CCs count showed a tendency to be higher in the humerus than in the femur. This work enlightened for the first time changes in composition of CCs surrounding matrix during SOCs development in dogs, paving the way to further investigations. PMID:27734993

  18. A Critical Review of the Incidence and Risk Factors for Finger Injuries in Rock Climbing.

    PubMed

    Jones, Gareth; Johnson, Mark I

    Rock climbing is a popular sporting activity and indoor sport climbing has been accepted for inclusion in the 2020 Olympic Games. The aim of this article is to critically review research on the incidence and risk factors associated with injuries during rock climbing. A semisystematic approach in reviewing literature on incidence and prevalence was applied. Articles were identified after searches of the following electronic databases: Discover, Academic Search Complete (EBSCO), PubMed, Embase, SPORTDiscus, and ScienceDirect. Despite methodological shortcomings of the studies contained within the review, the frequency of climbing-related injuries is high and can be challenging to diagnose. The fingers are the most common site of injury with previous injury a significant risk factor for reinjury. The annular pulleys of the fingers are the most commonly injured structure and evidence suggests epiphyseal fractures in adolescent sport climbers is increasing. A diagnostic and therapeutic algorithm for climbing-related finger injuries is proposed.

  19. Skeletal abnormalities in homocystinuria.

    PubMed Central

    Brenton, D. P.

    1977-01-01

    The skeletal changes of thirty-four patients with the biochemical and clinical features of cystathionine synthase deficiency are described. It is emphasized that there is clinical evidence of excessive bone growth and the formation for bone which is structurally weaker than normal. The similarities and differences between this condition and Marfan's syndrome are stressed and the possible nature of the connective tissue defect leading to the skeletal changes discussed. The most characteristic skeletal changes in homocystinuria are the skeletal disproportion (pubis-heel length greater than crown-pubis length), the abnormal vertebrae, sternal deformities, genu valgum and large metaphyses and epiphyses. Images Fig. 2 Fig. 3 Fig. 4 Fig. 8 Fig. 9 Fig. 10 PMID:917963

  20. Sagging rope sign in achondroplasia is different from Perthes disease.

    PubMed

    Oh, Chang-Wug; Shingade, Viraj Uttamrao; Song, Hae-Ryong; Suh, Seung-Woo; Hong, Jun-Seok; Lee, Seok-Hyun

    2005-01-01

    The "sagging rope" sign is a radiopaque line seen on radiographs of hips with Perthes disease. The main purpose of this study was to determine the incidence, cause, and importance of this sign in achondroplasia and to reveal how it differs from in Perthes disease. Serial radiograms, along with two- and three-dimensional CT images were studied in 42 patients with achondroplasia. The sign was observed bilaterally in all patients. Evaluation of CT images revealed spherical heads with the presence of circumferential overhang in all hips. This circumferential overhang seen on three-dimensional CT images corresponded to the sagging rope sign on plain radiographs. The presence of the sagging rope sign in bilateral hips is a characteristic feature of achondroplasia. It usually appears before epiphyseal closure. Its cause, incidence, and nature differ from in Perthes disease, and its presence does not carry a negative prognosis in achondroplasia.

  1. Role of oestrogen in the regulation of bone turnover at the menarche.

    PubMed

    Eastell, Richard

    2005-05-01

    The rise in oestrogen levels at menarche in girls is associated with a large reduction in bone turnover markers. This reduction reflects the closure of the epiphyseal growth plates, the reduction in periosteal apposition and endosteal resorption within cortical bone, and in bone remodelling within cortical and cancellous bone. Oestrogen promotes these changes, in part, by promoting apoptosis of chondrocytes in the growth plate and osteoclasts within cortical and cancellous bone. The period of early puberty is associated with an increased risk of fracture, particularly of the distal forearm, and this may be related to the high rate of bone turnover. A late menarche is a consistent risk factor for fracture and low bone mineral density in the postmenopausal period; models that might explain this association are considered.

  2. [Therapy principles of distal fractures of the forearm in childhood].

    PubMed

    Laurer, H; Sander, A; Wutzler, S; Walcher, F; Marzi, I

    2009-11-01

    Fractures of the forearm in children represent one of the most frequent injuries. Most are monotraumatic occurring during sport or leisure activities. Diagnosis can be made by conventional X-ray examination in 2 planes. Distal forearm fractures are classified into epiphyseolysis and epiphyseal fractures, metaphyseal greenstick and buckle fractures, complete distal radius and forearm fractures as well as Galeazzi lesions. The vast majority of cases without relevant dislocation can be treated with immobilization employing a cast. The remaining fractures have to be repositioned and restored depending on age and degree of dislocation.The state of the art in surgical therapy is the Kirschner wire osteosynthesis after closed reduction. Especially meta-diaphyseal fractures can alternatively be stabilized with plate osteosynthesis or external fixation. Following these therapeutic principles, prognosis is excellent and most of the injuries heal without any functional impairment.

  3. TMEM165 Deficiency Causes a Congenital Disorder of Glycosylation

    PubMed Central

    Foulquier, François; Amyere, Mustapha; Jaeken, Jaak; Zeevaert, Renate; Schollen, Els; Race, Valérie; Bammens, Riet; Morelle, Willy; Rosnoblet, Claire; Legrand, Dominique; Demaegd, Didier; Buist, Neil; Cheillan, David; Guffon, Nathalie; Morsomme, Pierre; Annaert, Willem; Freeze, Hudson H.; Van Schaftingen, Emile; Vikkula, Miikka; Matthijs, Gert

    2012-01-01

    Protein glycosylation is a complex process that depends not only on the activities of several enzymes and transporters but also on a subtle balance between vesicular Golgi trafficking, compartmental pH, and ion homeostasis. Through a combination of autozygosity mapping and expression analysis in two siblings with an abnormal serum-transferrin isoelectric focusing test (type 2) and a peculiar skeletal phenotype with epiphyseal, metaphyseal, and diaphyseal dysplasia, we identified TMEM165 (also named TPARL) as a gene involved in congenital disorders of glycosylation (CDG). The affected individuals are homozygous for a deep intronic splice mutation in TMEM165. In our cohort of unsolved CDG-II cases, we found another individual with the same mutation and two unrelated individuals with missense mutations in TMEM165. TMEM165 encodes a putative transmembrane 324 amino acid protein whose cellular functions are unknown. Using a siRNA strategy, we showed that TMEM165 deficiency causes Golgi glycosylation defects in HEK cells. PMID:22683087

  4. Leber's congenital amaurosis.

    PubMed

    De Laey, J J

    1991-01-01

    Leber's congenital amaurosis is an autosomal recessive disorder, characterized by the onset of blindness before the age of 6 months, a variable fundus aspect and an absent or extremely pathological ERG. The disorder may be isolated or associated with systemic involvement, such as nephronophtisis (Senior-Loken syndrome), nephronophtisis, cone-shaped epiphyses of the hand and cerebellar ataxia (Saldino-Mainzer syndrome), vermis hypoplasia, oculomotor anomalies and respiratory problems in the neonatal period (Joubert syndrome) or cardiomyopathy. It should be differentiated from other forms or chorioretinal dystrophies (juvenile retinitis pigmentosa or congenital stationary night blindness), cortical blindness or maturation delay and metabolic disorders. Children with possible congenital Leber amaurosis should not only have a thorough ophthalmological examination, but should also be seen by a paediatrician experienced in metabolic disorders.

  5. Matrilin-3 is dispensable for mouse skeletal growth and development.

    PubMed

    Ko, Yaping; Kobbe, Birgit; Nicolae, Claudia; Miosge, Nicolai; Paulsson, Mats; Wagener, Raimund; Aszódi, Attila

    2004-02-01

    Matrilin-3 belongs to the matrilin family of extracellular matrix (ECM) proteins and is primarily expressed in cartilage. Mutations in the gene encoding human matrilin-3 (MATN-3) lead to autosomal dominant skeletal disorders, such as multiple epiphyseal dysplasia (MED), which is characterized by short stature and early-onset osteoarthritis, and bilateral hereditary microepiphyseal dysplasia, a variant form of MED characterized by pain in the hip and knee joints. To assess the function of matrilin-3 during skeletal development, we have generated Matn-3 null mice. Homozygous mutant mice appear normal, are fertile, and show no obvious skeletal malformations. Histological and ultrastructural analyses reveal endochondral bone formation indistinguishable from that of wild-type animals. Northern blot, immunohistochemical, and biochemical analyses indicated no compensatory upregulation of any other member of the matrilin family. Altogether, our findings suggest functional redundancy among matrilins and demonstrate that the phenotypes of MED disorders are not caused by the absence of matrilin-3 in cartilage ECM.

  6. Cartilage oligomeric matrix protein and its binding partners in the cartilage extracellular matrix: interaction, regulation and role in chondrogenesis.

    PubMed

    Acharya, Chitrangada; Yik, Jasper H N; Kishore, Ashleen; Van Dinh, Victoria; Di Cesare, Paul E; Haudenschild, Dominik R

    2014-07-01

    Thrombospondins (TSPs) are widely known as a family of five calcium-binding matricellular proteins. While these proteins belong to the same family, they are encoded by different genes, regulate different cellular functions and are localized to specific regions of the body. TSP-5 or Cartilage Oligomeric Matrix Protein (COMP) is the only TSP that has been associated with skeletal disorders in humans, including pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED). The pentameric structure of COMP, the evidence that it interacts with multiple cellular proteins, and the recent reports of COMP acting as a 'lattice' to present growth factors to cells, inspired this review of COMP and its interacting partners. In our review, we have compiled the interactions of COMP with other proteins in the cartilage extracellular matrix and summarized their importance in maintaining the structural integrity of cartilage as well as in regulating cellular functions.

  7. Definition and subcategorization of idiopathic short stature: between consensus and controversy.

    PubMed

    Wit, Jan M

    2011-01-01

    Although various expert and consensus meetings have been held, there remains uncertainty about the definition of idiopathic short stature (ISS) and its subgroups. In this short review, the hypothetical pathophysiology, diagnosis and subcategorization of ISS are discussed. ISS in childhood may be the result of a combination of variants of three groups of genes: (1) single genes (or combinations of a few single genes) of which mutations, deletions or duplications are associated with a relatively large negative effect on height; (2) genes of which relatively frequently occurring polymorphisms are associated with a small negative effect on height; and (3) genes associated with delayed maturation of the epiphyseal plate. Differentiation between ISS and short children born small for gestational age and those with dysmorphic syndromes, systemic diseases or growth hormone deficiency and resistance can be difficult. Subcategorization based on distance to target height and onset of puberty is arbitrary, but useful.

  8. Orthopaedic aspects of mucopolysaccharidoses.

    PubMed

    White, Klane K

    2011-12-01

    Skeletal abnormalities are an early and prominent feature of most mucopolysaccharide (MPS) disorders, with the degree of skeletal involvement varying between and within MPS subtypes. Most patients exhibit a constellation of radiographic abnormalities known as dysostosis multiplex, consisting of abnormally shaped vertebrae and ribs, enlarged skull, spatulate ribs, hypoplastic epiphyses, thickened diaphyses and bullet-shaped metacarpals. Thoracolumbar kyphosis or the gibbus deformity is often a key diagnostic clue. Also common are hip dysplasia, genu valgum and, later in the course of the disease, spinal cord compression, which can be life-threatening. Short stature is ubiquitous. Treatment of skeletal manifestations usually involves surgical intervention. All patients with MPS should be considered at high risk for surgical intervention requiring anaesthesia because of airway and cardiac disease manifestations. Regular imaging of the cervical, thoracic and lumbar spine, the hips and the lower extremities is recommended for patients with MPS.

  9. Klinefelter's syndrome with renal tubular acidosis: impact on height.

    PubMed

    Jebasingh, F; Paul, T V; Spurgeon, R; Abraham, S; Jacob, J J

    2010-02-01

    A 19-year-old Indian man presented with a history of proximal muscle weakness, knock knees and gynaecomastia. On examination he had features of rickets and bilateral small testes. Karyotyping revealed a chromosomal pattern of 47,XXX, confirming the diagnosis of Klinefelter's syndrome. He was also found to have hyperchloraemic metabolic acidosis with hypokalaemia, hypophosphataemia, phosphaturia and glycosuria, which favoured a diagnosis of proximal renal tubular acidosis. Patients with Klinefelter's syndrome typically have a tall stature due to androgen deficiency, resulting in unfused epiphyses and an additional X chromosome. However, this patient had a short stature due to associated proximal renal tubular acidosis. To the best of our knowledge, this is the second case of Klinefelter's syndrome with short stature due to associated renal tubular acidosis reported in the literature. This report highlights the need to consider other causes when patients with Klinefelter's syndrome present with a short stature.

  10. Deletion at 12p in a Japanese child with brachydactyly overlaps the assignment locus of brachydactyly with hypertension in a Turkish family

    SciTech Connect

    Baehring, S.; Toka, H.R.; Nitz, I.

    1997-03-01

    Positional cloning is occasionally facilitated by the identification of a chromosomal aberration. We are studying a Turkish family, first described by Bilginturan et al., who have a monogenic form of hypertension, and we have reason to believe that a chromosomal aberration in a Japanese child may facilitate our cloning of the responsible gene. The hypertension gene in our family is unique in that it causes, by as yet undefined mechanisms, increased peripheral vascular resistance. Pedigree analysis of the Turkish kindred allowed us to map the gene to 12p11-p12. The kindred has a second trait, brachyclactyly, which cosegregates with the hypertension. The brachyclactyly, which involves both hands and feet, features shortened metacarpals and phalanges, as well as cone-shaped epiphyses. 11 refs., 2 figs.

  11. [Treatment of beginning juvenile detachment of the femoral head, taking growth of the femoral neck into account (author's transl)].

    PubMed

    Engelhardt, P

    1979-10-01

    Prevention of further detachment is the primary aim in the immediate treatment of beginning juvenile detachment of the femoral head. Screwing of the epiphysis of the head, first introduced by M. E. Mueller (1965), has proved successful. Besides providing immediate mechanical stability, this method, however, results in premature ossification of the joint of the femoral neck. Epiphysiodesis has a particularly unfavourable effect in early childhood, because it inhibits proper growth of the leg and development of the mechanism of the hip joint on account of the shortened femoral neck. Spiking of the epiphysis with Krischner screw wires guarantees safe fixation of the epiphyseal head on the one hand, and sufficient freedom of femoral neck growth on the other. Surgical treatment requires knowledge of the changed hip joint anatomy of the child. Preoperative planning via drawing to determine the length and position of the implantate on the basis of standardised x-ray films, will help to prevent operative failures.

  12. Paleopathological Study of Dwarfism-Related Skeletal Dysplasia in a Late Joseon Dynasty (South Korean) Population.

    PubMed

    Woo, Eun Jin; Lee, Won-Joon; Hu, Kyung-Seok; Hwang, Jae Joon

    2015-01-01

    Skeletal dysplasias related to genetic etiologies have rarely been reported for past populations. This report presents the skeletal characteristics of an individual with dwarfism-related skeletal dysplasia from South Korea. To assess abnormal deformities, morphological features, metric data, and computed tomography scans are analyzed. Differential diagnoses include achondroplasia or hypochondroplasia, chondrodysplasia, multiple epiphyseal dysplasia, thalassemia-related hemolytic anemia, and lysosomal storage disease. The diffused deformities in the upper-limb bones and several coarsened features of the craniofacial bones indicate the most likely diagnosis to have been a certain type of lysosomal storage disease. The skeletal remains of EP-III-4-No.107 from the Eunpyeong site, although incomplete and fragmented, provide important clues to the paleopathological diagnosis of skeletal dysplasias.

  13. Genetic linkage of mild pseudoachondroplasia (PSACH) to markers in the pericentromeric region of chromosome 19

    SciTech Connect

    Briggs, M.D.; Rasmussen, M.; Garber, P.; Rimoin, D.L.; Cohn, D.H. ); Weber, J.L. ); Yuen, J.; Reinker, K. )

    1993-12-01

    Pseudoachondroplasia (PSACH) is a dominantly inherited form of short-limb dwarfism characterized by dysplastic changes in the spine, epiphyses, and metaphyses and early onset osteoarthropathy. Chondrocytes from affected individuals accumulate an unusual appearing material in the rough endoplasmic reticulum, which has led to the hypothesis that a structural abnormality in a cartilage-specific protein produces the phenotype. The authors recently identified a large family with a mild form of pseudoachondroplasia. By genetic linkage to a dinucleotide repeat polymorphic marker (D19S199), they have localized the disease gene to chromosome 19 (maximum lod score of 7.09 at a recombination fraction of 0.03). Analysis of additional markers and recombinations between the linked markers and the phenotype suggests that the disease gene resides within a 6.3-cM interval in the immediate pericentromeric region of the chromosome. 39 refs., 2 figs., 1 tab.

  14. Characterization of a gene from the EDM1-PSACH region of human chromosome 19p

    SciTech Connect

    Lennon, G.G.; Giorgi, D.; Martin, J.R.

    1994-09-01

    Genetic linkage mapping has indicated that both multiple epiphyseal dysplasia (EDM1), a dominantly inherited chondrodysplasia, and pseudoachondroplasia (PSACH), a skeletal disorder associated with dwarfism, map to a 2-3 Mb region of human chromosome 19p. We have isolated a partial cDNA from this region using hybrid selection, and report on progress towards the characterization of the genomic structure and transcription of the corresponding gene. Sequence analysis of the cDNA to date indicates that this gene is likely to be expressed within extracellular matrix tissues. Defects in this gene or neighboring gene family members may therefore lead to EDM1, PSACH, or other connective tissue and skeletal disorders.

  15. Effects of radiation therapy on skeletal growth in childhood

    SciTech Connect

    Goldwein, J.W. )

    1991-01-01

    Ionizing radiation was used to treat childhood cancer long before the advent of chemotherapy, and it took little time for physicians to appreciate the deleterious effects it had on skeletal growth. The cause of this complication results predominantly from alteration of chondroblastic activity. This may stem directly from irradiation at the epiphyseal plate or indirectly from irradiation of glands that secrete growth-mediating hormones. The complication can go far beyond the obvious physical afflictions and extend into the psychologic domain, rendering deeper, more permanent scars. Presently, many of these effects are predictable, reducible, and treatable without compromising the cure that so often depends on the use of irradiation. Because of the complexities of childhood cancer therapy, strategies aimed at diminishing these effects are challenging. It is imperative that these effects be understood so that they can be reduced in current patients and prevented in future patients.33 references.

  16. Short-lasting accumulation in osteoid bone seams of radioactive iron injected as citrate into mice.

    PubMed

    Huser, H; Gerber, L; Eichenberger, P; Waelti, E; Cottier, H

    1988-05-01

    The possible role in vivo of osseous structures in binding radioactive iron injected as a low-molecular-weight complex was studied in mice, using combined autoradiography and histomorphometry on sections of undecalcified, plastic-embedded femur epiphyses/metaphyses. A single intraperitoneal injection of 10 microCi 59Fe (1.2 micrograms Fe) per animal as citrate within 3 hours led to a preferential accumulation of this metal in the osteoid mineralized tissue interphase (osteoid seams) of bone. Within the next 2 days the labeling intensity in this localization diminished markedly to approximate levels of the bone marrow and calcified bone. The bulk of the injected radioiron was utilized according to known erythrokinetics. Findings suggest a direct entry of "free," ie, not transferrin-bound, iron into osteoid seams and its consecutive rapid removal from this site.

  17. [Double mutant alleles in the EXT1 gene not previously reported in a teenager with hereditary multiple exostoses].

    PubMed

    Cammarata-Scalisi, Francisco; Cozar, Mónica; Grinberg, Daniel; Balcells, Susana; Asteggiano, Carla G; Martínez-Domenech, Gustavo; Bracho, Ana; Sánchez, Yanira; Stock, Frances; Delgado-Luengo, Wilmer; Zara-Chirinos, Carmen; Chacín, José Antonio

    2015-04-01

    Hereditary forms of multiple exostoses, now called EXT1/EXT2-CDG within Congenital Disorders of Glycosylation, are the most common benign bone tumors in humans and clinical description consists of the formation of several cartilage-capped bone tumors, usually benign and localized in the juxta-epiphyseal region of long bones, although wide body dissemination in severe cases is not uncommon. Onset of the disease is variable ranging from 2-3 years up to 13-15 years with an estimated incidence ranging from 1/18,000 to 1/50,000 cases in European countries. We present a double mutant alleles in the EXT1 gene not previously reported in a teenager and her family with hereditary multiple exostoses.

  18. [Fluoride effect on bone formation--an overview].

    PubMed

    Mohr, H

    1990-12-01

    The purpose of this review is to evaluate our present knowledge of fluoride effect on bone formation on basis of the literature. It is likely that fluoride affects the remodelling processes of the skeleton as well as growth related bone formation. During bone remodelling the amount of bone and osteoid tissue is increased by alteration of the balance between resorption and formation. This finding may be accompagnied by impaired mineralization. In studies of fluoride effect on growth related bone formation a number of quantitative histologic alterations have been observed. These include reduction in epiphyseal plate thickness and changes in cellular morphology as well as a retardation of mineralization. The pathogenetic mechanisms behind the observed effects and the variation in tissue response are still unexplained. Fluoride may have a direct cellular effect causing disturbances in cell morphology and metabolism, but the effects may also involve local supracellular mechanisms as well as the general homeostasis of the individual.

  19. Thumb reconstruction with extended twisted toe flap.

    PubMed

    Iglesias, M; Butron, P; Serrano, A

    1995-09-01

    Twelve amputated thumbs were reconstructed with a neurovascular cutaneous flap from the great toe and an osteotendinous flap from the second toe. Both transfers were dependent on a single vascular pedicle. One of the 12 reconstructions failed. In the remaining 11, the mobility of the metacarpophalangeal joint ranged from 10 degrees to 50 degrees, and that of the interphalangeal joint from 10 degrees to 30 degrees. Opposition and key pinch were restored, and the shape and volume were similar to those of the normal thumb. Likewise, the great toe was preserved, and all patients had a normal gait. This technique preserves the epiphyses for future growth in children and maintains the aesthetic appearance of the foot, but it has the disadvantage of involving a lengthy surgical procedure with a 17% rate of major complications.

  20. Aromatase Activity and Bone Loss in Men

    PubMed Central

    Merlotti, Daniela; Gennari, Luigi; Stolakis, Konstantinos; Nuti, Ranuccio

    2011-01-01

    Aromatase is a specific component of the cytochrome P450 enzyme system responsible for the transformation of androgen precursors into estrogens. This enzyme is encoded by the CYP19A1 gene located at chromosome 15q21.2, that is, expressed in ovary and testis, but also in many extraglandular sites such as the placenta, brain, adipose tissue, and bone. The activity of aromatase regulates the concentrations of estrogens with endocrine, paracrine, and autocrine effects on target issues including bone. Importantly, extraglandular aromatization of circulating androgen precursors is the major source of estrogen in men. Clinical and experimental evidences clearly indicate that aromatase activity and estrogen production are necessary for longitudinal bone growth, the attainment of peak bone mass, pubertal growth spurt, epiphyseal closure, and normal bone remodeling in young individuals. Moreover, with aging, individual differences in aromatase activity may significantly affect bone loss and fracture risk in men. PMID:21772971

  1. Achondrogenesis type II, abnormalities of extracellular matrix.

    PubMed

    Horton, W A; Machado, M A; Chou, J W; Campbell, D

    1987-09-01

    Immune and lectin histochemical and microchemical methods were employed to study growth cartilage from seven cases of achondrogenesis type II (Langer-Saldino). The normal architecture of the epiphyseal and growth plate cartilage was replaced by a morphologically heterogeneous tissue. Some areas were comprised of vascular canals surrounded by extensive fibrous tissue and enlarged cells that had the appearance and histochemical characteristics of hypertrophic chondrocytes. Other areas contained a mixture of cells ranging from small to the enlarged chondrocytes. The extracellular matrix in the latter areas was more abundant and had characteristics of both precartilage mesenchymal matrix and typical cartilage matrix; it contained types I and II collagen, cartilage proteoglycan, fibronectin, and peanut agglutinin binding glycoconjugate(s). Peptide mapping of cyanogen bromide cartilage collagen peptides revealed the presence of types I and II collagen. These observations could be explained by a defect in the biosynthesis of type II collagen or in chondrocyte differentiation.

  2. A second family with autosomal recessive spondylometaphyseal dysplasia and early death.

    PubMed

    Mégarbané, André; Mehawej, Cybel; El Zahr, Amir; Haddad, Soha; Cormier-Daire, Valérie

    2014-04-01

    We report on a consanguineous Lebanese family in which two sibs had pre- and post-natal growth retardation, developmental delay, large anterior fontanel, prominent forehead, low-set ears, depressed nasal bridge, short nose, anteverted nares, increased nasal width, prominent abdomen, and short limbs. Radiographs disclosed the presence of wormian bones, platyspondyly, decreased interpedicular distance at the lumbar vertebrae, square iliac bones, horizontal acetabula, trident acetabula, hypoplastic ischia, partial agenesis of the sacrum, ribs with cupped ends, short long bones with abnormal modeling, slight widening of the distal femoral metaphyses, and delayed epiphyseal ossification. Both sibs had a severe cardiomegaly and died at around 24 months from a heart failure. Differential diagnosis suggests that this is a second family presenting a newly described early lethal chondrodysplasia first reported by [Mégarbané et al. (2008); Am J Med Genet Part A 146A:2916-2919].

  3. Paleopathological Study of Dwarfism-Related Skeletal Dysplasia in a Late Joseon Dynasty (South Korean) Population

    PubMed Central

    Woo, Eun Jin; Lee, Won-Joon; Hu, Kyung-Seok; Hwang, Jae Joon

    2015-01-01

    Skeletal dysplasias related to genetic etiologies have rarely been reported for past populations. This report presents the skeletal characteristics of an individual with dwarfism-related skeletal dysplasia from South Korea. To assess abnormal deformities, morphological features, metric data, and computed tomography scans are analyzed. Differential diagnoses include achondroplasia or hypochondroplasia, chondrodysplasia, multiple epiphyseal dysplasia, thalassemia-related hemolytic anemia, and lysosomal storage disease. The diffused deformities in the upper-limb bones and several coarsened features of the craniofacial bones indicate the most likely diagnosis to have been a certain type of lysosomal storage disease. The skeletal remains of EP-III-4-No.107 from the Eunpyeong site, although incomplete and fragmented, provide important clues to the paleopathological diagnosis of skeletal dysplasias. PMID:26488291

  4. Postnatal skeletal ontogeny in Callisaurus draconoides and Uta stansburiana (Iguania: Phrynosomatidae).

    PubMed

    Maisano, Jessica Anderson

    2002-02-01

    The mineralization of the skeleton from hatching to near maximum size in two phrynosomatid lizards, Callisaurus draconoides and Uta stansburiana, is described in detail. Observed patterns in the appearance of epiphyseal secondary centers, ossification centers, apophyseal ossifications, and calcifications, the distribution of sesamoids, and the timing of fusions, are compared and contrasted with observations of other squamates available in the literature. Overall, Callisaurus and Uta exhibit an advanced state of ossification in the hatchling relative to other squamate neonates and share a similar sequence of braincase fusions and appearance of secondary centers. Preliminary observations suggest that patterns of postnatal skeletal development are highly conserved and independent of patterns of prenatal morphogenesis, and thus a potentially rich source of character data for systematic investigations.

  5. Reconstructive surgery for the post-hemiepiphysiodesis residual deformity in congenital scoliosis.

    PubMed

    Tauchi, Ryoji; Tsuji, Taichi; Ohara, Tetsuya; Suzuki, Yoshitaka; Saito, Toshiki; Nohara, Ayato; Sugawara, Ryo; Imagama, Shiro; Kawakami, Noriaki

    2013-07-01

    Spinal hemiepiphysiodesis is a well-known surgical procedure for correcting angular deformity in which the anterior and/or posterior epiphyseal plates of several vertebrae are fused on the convex side of the curvature with the expectation of spontaneous correction through continued growth of the concave side. We report on a patient with congenital scoliosis that had problems after hemiepiphysiodesis and required a salvage, reconstructive, antero-posterior combined surgery with instrumentation and osteotomy. After hemiepiphysiodesis, a marked fusion mass and a loss of bony landmarks made osteotomy and correction with a salvage surgery extremely difficult. We suggest that confirming the fusion area after hemiepiphysiodesis and determining the appropriate osteotomy site by 3D reconstruction images are important before salvage operations.

  6. [The mathematical modeling of the injurious impact on the tibial bone for the evaluation of the conditions leading to its fracture].

    PubMed

    Leonov, S V; Pinchuk, P V; Krupin, K N; Panfilov, D A

    2017-01-01

    We have undertaken the mathematical modeling of the process associated with the destruction of the diaphyseal and proximal epiphyseal parts of the tibial bone by means of the finite element analysis. The main emphasis was laid on the elucidation of the topography of force stresses in the model bone. It was shown that loading the upper third of the bone either along its axis or perpendicular to the surface (i.e. in the region formed largely by the cancellous tissue) results in the depressed fracture at the site of the impact. Loading of the mid-third region of the bone (characterized by the predominance of the compact tissue) under the same conditions led to the transverse fracture originating from the side opposite to the impact application site.

  7. Physeal-Sparing Technique for Femoral Tunnel Drilling in Pediatric Anterior Cruciate Ligament Reconstruction Using a Posteromedial Portal

    PubMed Central

    Lemos, Stephen E.; Keating, Patrick M.; Scott, Timothy P.; Siwiec, Ryan M.

    2013-01-01

    Pediatric anterior cruciate ligament (ACL) tears present a technical dilemma for orthopaedic surgeons. Multiple surgical techniques have been described to protect the distal femoral and proximal tibial physes. We present an ACL reconstruction technique performed on a 12-year-old girl with open physes who sustained an ACL tear after a noncontact twisting injury while playing soccer. A hamstring autograft reconstruction was performed by use of a posteromedial portal to drill the femoral tunnel in an all-epiphyseal fashion at the anatomic footprint of the native ACL. This case provides a new surgical technique to achieve anatomic fixation for ACL reconstruction in a skeletally immature individual using a posteromedial portal to drill a physeal-sparing lateral femoral tunnel for anatomic ACL reconstruction. This advancement may make drilling the femoral tunnel less technically challenging compared with other proposed methods while maintaining the lateral wall of the distal femur. PMID:24892013

  8. A specific collagen type II gene (COL2A1) mutation presenting as spondyloperipheral dysplasia

    SciTech Connect

    Zabel, B.; Hilbert, K.; Spranger, J.; Winterpacht, A.; Stoeb, H.; Superti-Furga, A.

    1996-05-03

    We report on a patient with a skeletal dysplasia characterized by short stature, spondylo-epiphyseal involvement, and brachydactyly E-like changes. This condition has been described as spondyloperipheral dysplasia and the few published cases suggest autosomal dominant inheritance with considerable clinical variability. We found our sporadic case to be due to a collagen type II defect resulting from a specific COL2A1 mutation. This mutation is the first to be located at the C-terminal outside the helical domain of COL2A1. A frameshift as consequence of a 5 bp duplication in exon 51 leads to a stop codon. The resulting truncated C-propeptide region seems to affect helix formation and produces changes of chondrocyte morphology, collagen type II fibril structure and cartilage matrix composition. Our case with its distinct phenotype adds another chondrodysplasia to the clinical spectrum of type II collagenopathies. 16 refs., 4 figs.

  9. Slipped Capital Femoral Epiphysis and Primary Hyperparathyroidism: A Case Report

    PubMed Central

    Alghamdi, Anas A.; Ahmad, Maswood M.; Almalki, Mussa H.

    2016-01-01

    The aim of reporting this case is to highlight the association of two disorders, primary hyperparathyroidism (PHPT) and slipped capital femoral epiphysis (SCFE). They are usually seen in two different age groups and rarely together. PHPT is a rare cause of SCFE and only 10 cases have been reported in the literature worldwide. The patient in our report is a 13-year-old girl who presented to our clinic with bilateral knee pain and a waddling gait. Subsequent investigations showed that she had PHPT and SCFE with low bone mass. On admission, a parathyroidectomy was performed; then, the slipped femoral epiphyses were fixed with satisfactory results. A systematic algorithmic approach that was illustrated in a previously published case was used. Such cases should be managed with a systematic approach based on the patient’s clinical status to prevent future morbidity. A literature review was conducted by performing a Medline search of all reported cases of PHPT and SCFEs. PMID:27920593

  10. Successful management of a childhood osteosarcoma with epiphysiolysis and distraction osteogenesis

    PubMed Central

    Xu, S.F.; Yu, X.C.; Xu, M.; Chen, X.

    2014-01-01

    In an 11-year-old boy with osteosarcoma in the proximal tibia (type iii), 2 cycles of dia chemotherapy (cisplatin, ifosfamide, doxorubicin) were administered preoperatively while epiphysiolysis was performed. Clinical response was determined to be complete by radiography and histopathology. Marginal excision was then performed with preservation of the proximal tibial epiphysis. Metaphyseal reconstruction was performed using distraction osteogenesis. Six cycles of dia chemotherapy were administered postoperatively. Twenty months later, the patient had developed no complications and experienced full bone healing, with no limb discrepancy. In selected adolescent patients with osteosarcoma, in whom the tumour is in full contact with the epiphysis, epiphyseal preservation by epiphysiolysis and reconstruction by distraction osteogenesis can provide an excellent outcome, resulting in a stable reconstruction that functionally restores the native limb. PMID:25089114

  11. Juxtaphyseal Intraosseous Hemangioma of Proximal Femur causing Coxa vara and Coxa breva deformity in a growing child

    PubMed Central

    Song, Hae Ryong; Shyam, Ashok K.

    2011-01-01

    Introduction: Bony hemangiomas are rare lesions in growing skeleton. Affection of the epiphyseal plate by a bony hemangioma leading to growth retardation is rare. Case report: We report the radiological picture of a juxtaphyseal osseous hemangioma affecting the capital femoral physis leading to coxa vara and coxa breva deformity. This hemangioma is also a rarity as it has both intracortical and intra medullary components. A diagnostic and therapeutic CT guided core needle biopsy/decompression was performed to confirm the histopathological diagnosis and to decompress the lesion. Patient was treated conservatively with shoe raise and regular checkups and at two year follow-up there were no interval changes noted on the radiographs with patient completely asymptomatic. Conclusion: Juxtaphyseal hemangiomas may be amenable to needle decompression, however longer follow will be required to assess the further response.

  12. Bone kinetics of calcium-45 and pyrophosphate labeled with technetium-96: an autoradiographic evaluation. [Rabbits

    SciTech Connect

    Guillemart, A.; Le Pape, A.; Galy, G.; Besnard, J.C.

    1980-05-01

    The uptake of calcium-45 and of pyrophosphate labeled with the long-lived technetium-96 isotope was compared by means of liquid-emulsion microautoradiograms of the epiphyseal plates of 10-week-old rabbits, at 30 min, and 3 and 48 hr after i.v administration. For both tracers, thin sections confirm the significant role of the blood supply, especially shortly after injection. However, other more specific mechanisms lead to a mixing of the calcium in the mineral mass and to a linear deposition of technetium facing the osteoid surfaces. These findings suggest that the tropism of tin-reduced technetium pyrophosphate is not governed by the mineral pool but rather by exchanges inside a still poorly calcified organic matrix.

  13. Pituitary tumor with gigantism, acromegaly and preclinical Cushing's disease diagnosed from the 10th row.

    PubMed

    Tourtelot, John B; Vesely, David L

    2013-08-01

    A 7'3" basketball player was noted to have 2 to 3 times thicker tissue in his hands than 6'10" players by an endocrinologist sitting 10 rows above the player in a basketball arena. This led to the diagnosis of pituitary gigantism where the history revealed that he was 7'3" at 15 years of age. At age 19 when the acryl enlargement was noted, a diagnostic workup revealed elevated growth hormones and insulin-like growth factor 1 (IGF-1) with a 2 × 1.3 cm pituitary tumor. His history suggested that his epiphyseal plates had closed at age 15, and because he continued to produce IGF-1, he now has acromegaly. His elevated adrenocorticotropic hormone (ACTH) before surgery suggests that he also had preclinical Cushing's disease. After pituitary transsphenoidal surgery, all acryl enlargement in hands and ligaments disappeared. His growth hormone, IGF-1 and ACTH returned to normal 2 weeks after surgery.

  14. Management of type 2 diabetes mellitus associated with pituitary gigantism.

    PubMed

    Ali, Omar; Banerjee, Swati; Kelly, Daniel F; Lee, Phillip D K

    2007-01-01

    Pituitary gigantism, a condition of endogenous growth hormone (GH) hypersecretion prior to epiphyseal closure, is a rare condition. In the adult condition of GH excess, acromegaly, the occurrence of type 2 diabetes mellitus (T2DM) and diabetic ketoacidosis (DKA) have been reported, with resolution following normalization of GH levels. We report the case of a 16-year-old male with pituitary gigantism due to a large invasive suprasellar adenoma who presented with T2DM and DKA. Despite surgical de-bulking, radiotherapy and medical treatment with cabergoline and pegvisomant, GH and insulin-like growth factor-I (IGF-I) levels remained elevated. However, the T2DM and recurrent DKA were successfully managed with metformin and low-dose glargine insulin, respectively. We review the pathophysiology of T2DM and DKA in growth hormone excess and available treatment options.

  15. Short stature in patients with 45,X/46,XY mosaicism: report of three cases.

    PubMed

    Lee, Chih-Fand; Su, Pen-Hua; Chen, Jia-Yuh; Chen, Suh-Jen; Yang, Kai-Chi; Lin, Li-Ling

    2006-01-01

    Chromosomal analysis is routinely considered in all girls--but not boys-of short stature to screen for Turner syndrome. We report three patients of short stature (body height < 3rd percentile) with 45,X/46,XY mosaicism karyotype. They were brought to our outpatient department at the ages of 10, 13, and 18 years, respectively. Two of them had some stigmata of Turner syndrome. Two were treated with growth hormone. In the first patient, body height increased by 20 cm in 3 years 7 months. The second case increased by 6.5 cm in 8 months. We suggest that boys with unexplained short stature should be screened with chromosomal analysis, and growth hormone treatment should be offered to boys of short stature and 45,X/46,XY mosaicism before the epiphyseal plates close.

  16. Trichorhinophalangeal syndrome type 1: A case report with literature review

    PubMed Central

    Candamourty, Ramesh; Venkatachalam, Suresh; Karthikeyan, B.; Babu, M. R. Ramesh

    2012-01-01

    Trichorhinophalangeal syndrome is a very rare genetic disorder, where damage and mutation to the number 8 chromosome affects sufferers in numerous ways. The syndrome has three types, all characterized by abnormally short stature, sparse hair, short deformed fingers with cone-shaped epiphyses visible in radiographs. Type I is the most common. Type II is characterized by the development of multiple bony exostoses and frequently, mental disability. Type III is a more severe form of type I and is associated with short stature. This report presents a 28-year-old man who had the characteristic features of type I with the presence of multiple erupted supernumerary teeth with normal mentation and karyotyping with high resolution G banding displayed normal chromosomal complements. PMID:23225991

  17. Marked variability in the radiographic features of cartilage-hair hypoplasia: case report and review of the literature.

    PubMed

    Kwan, Andrea; Manning, M A; Zollars, Linda K; Hoyme, H Eugene

    2012-11-01

    Cartilage-hair hypoplasia (CHH) is a rare recessive metaphyseal chondrodysplasia characterized by severe short stature, ectodermal dysplasia, anemia in childhood, immune deficiency, susceptibility to malignancy, and normal intelligence. Short, thick long bones, metaphyseal flaring and irregularities, and globular epiphyses at the knees and ankles are the typical radiographic findings. The diagnosis is primarily made on the basis of clinical features, although mutations in the RMRP gene have recently been described in affected individuals, facilitating confirmation of the clinical diagnosis in atypical patients. We present a patient with two RMRP mutations whose stature and ectodermal features supported the diagnosis of CHH, but whose radiographic findings and other extraskeletal findings did not. We propose that the most consistent and reliable features of CHH are short stature of prenatal onset and ectodermal dysplasia, and suggest that the diagnosis of CHH be considered and mutation analysis pursued even when typical radiographic findings are absent.

  18. The mecanism of cementing line formation in the bones of cestrum-fed chicks.

    PubMed

    Bélanger, L F; Narbaitz, R

    1978-03-01

    Chicks fed a rachitogenic diet for five weeks after hatching were then treated with a daily oral dose of 1,000 I.U. Vitamin D3 or a 1% addition to the feed of powdered leaves of Cestrum diurnum for periods of 1, 2, 4, 8, 16 and 30 days. Comparative studies were made on stained sections, microradiographs of undermineralized sections and alpharadiographs of demineralized sections. The present dose of Cestrum diurnum caused at first, a rapid maturation and mineralization of the epiphyseal cartilage and an intense growth and osteocytic osteolysis in the diaphysis of the tibia and femur. After 8 days however, growth decreased and the diaphysis gradually became petrotic. Under these conditions, the osteocytes degenerated and died. The areas of polysaccharide-rich, low density matrix which surrounded them, decreased gradually to become cementing lines, persistent after 30 days.

  19. Defects in the cartilaginous growth plates of brachymorphic mice

    PubMed Central

    1977-01-01

    Homozygous brachymorphic (bm/bm) mice are characterized by disproportionately short stature. Newborn bm/bm epiphyseal cartilages are shorter than normal although the cells in the different zones of growth are relatively well organized. The extracellular matrix reacts poorly with stains specific for sulfated glycosaminoglycans. The ultrastructural appearance of the cartilage matrix indicates normal collagen fibrils; however, proteoglycan aggregate granules are smaller than normal and are present in reduced numbers, particularly in the columnar and hypertrophic zones of the growth plate. In addition, a prominent network of fine filaments, which are extractable in 4 M guanidine hydrochloride, are present in the bm/bm cartilage matrix. These findings suggest that a defect affecting the proteoglycan component of cartilage occurs in bm/bm mice. PMID:67117

  20. Bilateral Simultaneous Avulsion Fractures of the Proximal Tibia in a 14-Year-Old Athlete with Vitamin-D Deficiency

    PubMed Central

    Harb, Ziad; Malhi, Arfan

    2015-01-01

    Fractures involving the proximal tibial epiphysis are rare and form 0.5% of all epiphyseal injuries. The specific anatomical and developmental features of the proximal tibial epiphysis make it vulnerable to unique patterns of fractures. Vitamin-D plays a vital role in bone homeostasis and its deficiency has an impact on fracture risk and healing. We present the first ever reported case of simultaneous bilateral proximal tibial physeal fractures in an athlete with vitamin-D deficiency. Treatment consisted of plaster immobilisation, and the patient made a full recovery and returned to preinjury level of activities. We report this case for its uniqueness and as an educational review of the importance of the developmental anatomy of the proximal tibia. We review the literature and discuss how the stages of the growing physis determine the type of fracture sustained. PMID:26425381

  1. Neandertal postcranial remains from the Sima de las Palomas del Cabezo Gordo, Murcia, southeastern Spain.

    PubMed

    Walker, Michael J; Ortega, Jon; López, Mariano V; Parmová, Klára; Trinkaus, Erik

    2011-04-01

    The Sima de las Palomas, southeastern Spain, has yielded a series of Neandertal postcranial remains, including immature and mature isolated elements and the fragmentary partial skeleton of a young adult (Palomas 92). The remains largely conform to the general late archaic/Neandertal morphological pattern in terms of humeral diaphyseal shape, pectoralis major tuberosity size and pillar thickness, ulnar coronoid process height, manual middle phalangeal epiphyseal breadth, manual distal phalangeal tuberosity shape and breadth, femoral diaphyseal shape, and probably body proportions. Palomas 92 contrasts with the Neandertals in having variably gracile hand remains, a more sellar trapezial metacarpal 1 facet, more anteroposteriorly expanded mid-proximal femoral diaphysis, and less robust pedal proximal phalanges. The Palomas Neandertals contrast with more northern European Neandertals particularly in various reflections of overall body size.

  2. Football injury: a literature review *

    PubMed Central

    Kos, John J.

    1979-01-01

    A great deal of concern is recently being expressed relative to the playing of tackle football by adolescent Canadians. The purpose of this literature review is to try to summarize the important data from the available world literature. Very few Canadian statistics are available. Most of the data comes from United States experience. Tackle football injury is examined from various perspectives: 1. Equipment 2. Mechanisms of injury 3. Types of injury, with some emphasis on epiphyseal injury 4. Prevention 5. Comparison with other sports Although no “hard and fast” conclusion is drawn, the paper tends to show that: 1. Football is dangerous 2. Football is damaging to many body systems 3. Prevention of injury is difficult under present conditions 4. Alternate games, such as soccer and rugby seem to provide the same benefits with less catastrophic injuries

  3. Osteochondroma of maxillofacial region: Tumor arising from two different developmental bones

    PubMed Central

    Mohanty, Sujata; Gupta, Himanshu; Dabas, Jitender; Kumar, Priyadarshan

    2016-01-01

    Osteochondromas are benign bony tumors which are commonly believed to originate by the proliferation of epiphyseal cartilage into the surrounding tissues. However, this hypothesis cannot explain the occurrence of this tumor in the intramembranous bones and soft tissue. Since most of the craniofacial bones have intramembranous origin, the occurrence of this lesion in this territory is considered rare. Contrary to the above hypothesis, Lichtenstein proposed that this entity arises from the metaplastic changes in the periosteum which explains the occurrence of this tumor in endochondral as well as intramembranous bones and also in soft tissues. Complying with Lichtenstein's hypothesis, the authors are presenting two cases of osteochondromas with one arising from the endochondral bone (the coronoid process of the mandible) and the other from an intramembranous bone (lateral pterygoid plate of the sphenoid). PMID:27601834

  4. Trichorhinophalangeal syndrome type II presenting with short stature in a child.

    PubMed

    Hazan, Filiz; Korkmaz, Hüseyin A; Yararbaş, Kanay; Wuyts, Wim; Tükün, Ajlan

    2016-12-01

    Trichorhinophalangeal syndrome type II (TRPSII) (synonym: Langer-Giedon syndrome) is a rare autosomal dominant contiguous gene syndrome, resulting from a microdeletion encompassing the EXT1 and the TRPS1 gene at 8q24 (MIM#150230). This syndrome combines the clinical features of two autosomal dominant disorders, trichorhinophalangeal syndrome type I (MIM#190350) and hereditary multiple osteochondromas type I (MIM # 133700). TRPSII is characterized by sparse scalp hair, a long nose with a bulbous tip, long flat philtrum, cone-shaped epiphyses of the phalanges, retarded bone age in infancy and multiple cartilaginous osteochondromas. We report a Turkish patient who had the clinical features and skeletal signs of TRPSII in whom a 13.8Mb deletion in 8q23.1- 8q24.13 was detected.

  5. Trevor's Disease: Management Difficulties and Proposed Classification.

    PubMed

    Clarke, Daine O

    2016-09-01

    Dysplasia epiphysealis hemimelica, also known as Trevor's disease, is a rare developmental disorder with osteocartilagenous overgrowth of the epiphysis or epiphyseal equivalent. The condition bears similarities to osteochondroma in terms of its radiographic appearance, but differs in its pathobiology and geographic occurrence. Unlike the metaphyseal occurrence of osteochondromata, it arises from the epiphysis. The clinical presentation is wide and varied, but mechanical symptoms and deformities predominate. Early reports of the condition suggested involvement of the lower limbs only. However, since then, numerous reports have indicated a more generalized distribution. Difficulties in management and recurrence rates seem to hinge on whether its origin is intra-articular or extra-articular. A new classification system to include these parameters is discussed. [Orthopedics.2016; 39(5):e967-e969.].

  6. Ectopic bone formation and chondrodysplasia in transgenic mice carrying the rat C3(1)/T{sub AG} fusion gene

    SciTech Connect

    Green, J.E.; Maroulakou, I.G.; Anver, M.

    1994-09-01

    Transgenic mice expressing the SV40 large T-antigen (T{sup AG}) under the regultory control of the hormone-responsive rat C3(1) prostatein promoter develop unusual bone and cartilage lesions, as well as ectopic bone and cartilage formation. Two lines of transgenic animals have been propagated in which the expression of the transgene in chondrocytes results in a mild to moderate generalized disorganization of cartilage growth which appears to affect multiple tissues, including the trachea, ear pinna and articular cartilage. The epiphyseal plates are also affected with normal architecture of the zones of proliferation and maturation, but marked elongation of the zone of hypertrophy. Immunocytochemistry demonstrates that expression of T{sup AG} is limited to the zone of hypertropny in the epiphyseal plates, suggesting that the chondrocytes become hormone-responsive at this particular stage of differentiation. Normal mineralization and trabecular formation in long bone appears to occur. Ectopic bone and cartilage formation occurs in the foot pads of the fore- and hind- feet over the course of several months. This is preceded by proliferation of sweat gland epithelial cells followed by the appearance of nodules of cartilage and bone. The nodules are closely associated with proliferating epithelium but are not contiguous with bony structures normally found in the feet. The roles of BMP`s, growth factors, oncogenes and hormones in the development of these lesions will be presented. These transgenic animals may provide new insights into hormone-responsiveness of chondrocytes, as well as factors involved in the processes of bone and cartilage differentiation and growth. These transgenic animals may serve as a useful model for human heterotopic bone formation.

  7. Osteochondritis dissecans and Osgood Schlatter disease in a family with Stickler syndrome

    PubMed Central

    Al Kaissi, Ali; Klaushofer, Klaus; Grill, Franz

    2009-01-01

    Purpose Stickler syndrome is among the most common autosomal dominant connective tissue disorders but is often unrecognised and therefore not diagnosed by clinicians. Despite much speculation, the cause of osteochondrosis in general and osteochondritis dissecans (OCD) and Osgood Schlatter syndrome (OSS) in particular remain unclear. Etiological understanding is essential. We describe a pair of family subjects presented with OCD and OSS as a symptom complex rather than a diagnosis. Methods Detailed clinical and radiographic examinations were undertaken with emphasis on the role of MRI imaging. Magnetic resonance imaging may allow early prediction of articular lesion healing potential in patients with Stickler syndrome. Results The phenotype of Stickler syndrome can be diverse and therefore misleading. The expectation that the full clinical criteria of any given genetic disorder such as Stickler syndrome will always be present can easily lead to an underestimation of these serious inheritable disorders. We report here two family subjects, a male proband and his aunt (paternal sister), both presented with the major features of Stickler syndrome. Tall stature with marfanoid habitus, astigmatism/congenital vitreous abnormality and submucus cleft palate/cleft uvula, and enlarged painful joints with early onset osteoarthritis. Osteochondritis dissecans (OCD) and Osgood Schlatter syndrome (OSS) were the predominating joint abnormalities. Conclusion We observed that the nature of the articular and physeal abnormalities was consistent with a localised manifestation of a more generalised epiphyseal dysplasia affecting the weight-bearing joints. In these two patients, OCD and OSS appeared to be the predominant pathologic musculoskeletal consequences of an underlying Stickler's syndrome. It is empirical to consider generalised epiphyseal dysplasia as a major underlying causation that might drastically affect the weight-bearing joints. PMID:19193224

  8. Nutrition-induced catch-up growth increases hypoxia inducible factor 1alpha RNA levels in the growth plate.

    PubMed

    Even-Zohar, N; Jacob, J; Amariglio, N; Rechavi, G; Potievsky, O; Phillip, M; Gat-Yablonski, G

    2008-03-01

    Although catch-up growth is a well-known phenomenon, the local pathways at the epiphyseal growth plate that govern this process remain poorly understood. To study the mechanisms governing catch-up growth in the growth plate, we subjected prepubertal rats to 10 days of 40% food restriction, followed by a renewal of the regular food supply to induce catch-up growth. The animals were weighed daily, and their humeral length was measured at sacrifice. The proximal tibial epiphyseal growth plates (EGPs) were studied, and findings were compared with EGPs from animals fed ad libitum and animals under food restriction. The gene expression profile in the growth plates was examined using DNA microarrays, and the expression levels of selected genes were validated by real-time polymerase chain reaction. To localize gene expression in different growth plate zones, microdissection was used. Protein levels and localization were examined using immunohistochemistry. We showed that the expression level of 550 genes decreased during food restriction and increased during catch-up growth, starting already one day after refeeding. HIF-1alpha, as well as several of its downstream targets, was found among these genes. Immunohistochemistry showed a similar pattern for HIF-1alpha protein abundance. Additionally, HIF-1alpha mRNA and protein levels were higher in the proliferating than in the hypertrophic zone, and this distribution was unaffected by nutritional status. These findings indicate that nutrition has a profound effect on gene expression level during growth plate growth, and suggest an important role for HIF-1alpha in the growth plate and its response to nutritional manipulation.

  9. Effect of administration of retinol and etidronate on bone histomorphometric parameters in ovariectomized rats.

    PubMed

    Kaczmarczyk-Sedlak, Ilona; Pytlik, Maria; Sliwińiski, Leszek; Nowińska, Barbara; Juszczyk, Joanna

    2004-01-01

    Retinol belongs to factors affecting bone remodeling. The effect of retinol on the osseous tissue depends on the dose and duration of treatment. Retinol can cause bone damage and deformation. Retinol is frequently administered chronically in too high doses, sometimes by osteoporotic patients. The aim of the present study was to examine the interaction between retinol and an antiresorptive drug--disodium etidronate in bilaterally ovariectomized rats. The experiments were carried out on Wistar rats (200 +/- 30 g), divided into 7 groups: I--sham operated control rats. II--ovariectomized control rats (OVX), III--OVX + editronate (10 mg/kg p.o.), IV--OVX + retinol (700 IU/kg p.o.). V--OVX + retinol (3500) IU/kg p.o.), VI--OVX + etidronate (10 mg/kg p.o.) + retinol (700 IU/kg p.o.), VII--OVX + etidronate (10 mg/kg p.o.) + retinol (3500 IU/kg p.o.). The drugs were administered for 4 weeks. Bone macrometric and histomorphometric parameters of the tibia (transverse growth, width of periosteal and endosteal osteoid, area of the transverse cross-section of the diaphysis and area of the transverse cross-section of the marrow cavity) and the femur (width of epiphyseal and metaphyseal trabeculae, width of epiphyseal cartilage) were examined. Editronate partially counteracted the development of changes induced by ovariectomy. Retinol (700 IU/kg p.o.) caused decreases in the area of the transverse cross-section of the marrow cavity and the width of osteoid, and an increase in the width of trabeculae. Retinol (3500 IU/kg p.o.) caused decreases in bone mass and the area of the transverse corss-section of the marrow cavity, and an increase in the width of trabecula. Concurrent administration of etidronate and retinol in ovariectomized rats seemed not to affect bone histomorphometric parameters in a way suggesting any interaction between them.

  10. Automated assessment of bone changes in cross-sectional micro-CT studies of murine experimental osteoarthritis

    PubMed Central

    Vincent, Tonia L.; Marenzana, Massimo

    2017-01-01

    Objective The degradation of articular cartilage, which characterises osteoarthritis (OA), is usually paired with excessive bone remodelling, including subchondral bone sclerosis, cysts, and osteophyte formation. Experimental models of OA are widely used to investigate pathogenesis, yet few validated methodologies for assessing periarticular bone morphology exist and quantitative measurements are limited by manual segmentation of micro-CT scans. The aim of this work was to chart the temporal changes in periarticular bone in murine OA by novel, automated micro-CT methods. Methods OA was induced by destabilisation of the medial meniscus (DMM) in 10-week old male mice and disease assessed cross-sectionally from 1- to 20-weeks post-surgery. A novel approach was developed to automatically segment subchondral bone compartments into plate and trabecular bone in micro-CT scans of tibial epiphyses. Osteophyte volume, as assessed by shape differences using 3D image registration, and by measuring total epiphyseal volume was performed. Results Significant linear and volumetric structural modifications in subchondral bone compartments and osteophytes were measured from 4-weeks post-surgery and showed progressive changes at all time points; by 20 weeks, medial subchondral bone plate thickness increased by 160±19.5 μm and the medial osteophyte grew by 0.124±0.028 μm3. Excellent agreement was found when automated measurements were compared with manual assessments. Conclusion Our automated methods for assessing bone changes in murine periarticular bone are rapid, quantitative, and highly accurate, and promise to be a useful tool in future preclinical studies of OA progression and treatment. The current approaches were developed specifically for cross-sectional micro-CT studies but could be applied to longitudinal studies. PMID:28334010

  11. Aromatase deficiency, a rare syndrome: case report.

    PubMed

    Baykan, Emine Kartal; Erdoğan, Mehmet; Özen, Samim; Darcan, Şükran; Saygılı, L Füsun

    2013-01-01

    Aromatase deficiency (AD) is a rare autosomal recessive inheritance syndrome. Its worldwide incidence is unknown, and there are few case reports in the literature. Aromatase dysfunction develops due to CYP19A1 gene mutation and a decrease in estrogen synthesis. Estrogen deficiency can induce delayed epiphyseal closure, eunuchoid body habitus, osteopenia, and osteoporosis in both genders. Our patient was a 27-year-old male who presented with bone pain, recurrent bone fractures associated with minimal trauma starting in puberty, and a progressive increase in height. Laboratory tests revealed that the blood levels of follicle-stimulating hormone and luteinizing hormone were above normal, testosterone level was normal, and estrogen was undetectable. Plain bone radiography of the left wrist and hand demonstrated that the epiphyses were still unfused. Lumbar osteoporosis was detected in bone densitometry. In the genetic analysis, homozygous R375H guanine-adenine (G-A) mutation was detected in the CYP19A1 gene, and a diagnosis of AD was reached. Treatment with 25 μg transdermal estradiol was started. All family members were examined. Homozygous R375H G-A mutation was detected in the patient's younger brother. Heterozygous R375H G-A mutation was found in his mother, father, and older brother. In conclusion, this AD patient requires lifetime estrogen replacement in order to provide sufficient bone mineralization, to reduce the risk of bone fractures, and to lead a healthy life. The best method to prevent the possible complications is to diagnose the AD syndrome at early ages and to provide adequate estrogen replacement starting at puberty.

  12. Treatment of Late-Onset Legg-Calve-Perthes Disease by Arthrodiastasis

    PubMed Central

    Kim, Sung Soo; Lee, Chan Woo; Kim, Hyeon Jun; Kim, Hyun Ho

    2016-01-01

    Background To evaluate the efficacy of arthrodiastasis for Legg-Calve-Perthes disease. Methods Arthrodiastasis was conducted using external fixator devices (Orthofix) in 7 patients at least 8 years of age with a diagnosis of Legg-Calve-Perthes disease. The average follow-up was 80 months (range, 32 to 149 months), and their average age was 9.1 years (range, 8 to 12 years). The results of treatment were evaluated by measuring the degree of hip pain and the range of motion of the hip at 6 months after the operation and comparing the values with preoperative measurements. Radiological recovery was evaluated by the epiphyseal index and compared with the preoperative values. At the final follow-up, clinical and radiological results were evaluated using the Iowa hip score and the Stulberg classification. Results On the clinical evaluation performed at 6 months after arthrodiastasis, the degree of pain decreased by 1.8 points on average, and the average flexion, internal rotation, and abduction increased by 35°, 16°, and 11°, respectively. Based on radiological findings, the epiphyseal index showed a remarkable increase of 6.6 on average (from 19 preoperatively to 26 postoperatively). At the final follow-up, the average Iowa hip score improved from 65 points preoperatively to 84 points. There were 1 Stulberg class I hip, 2 Stulberg class II hips, 3 Stulberg class III hips, 1 Stulberg class IV hip, and no Stulberg class V hip. Conclusions We conclude that arthrodiastasis using an external fixator can be a relatively promising surgical procedure for the treatment of late-onset Legg-Calve-Perthes disease. PMID:27904729

  13. Accuracy of Various MRI Sequences in Determining the Tumour Margin in Musculoskeletal Tumours

    PubMed Central

    Putta, Tharani; Gibikote, Sridhar; Madhuri, Vrisha; Walter, Noel

    2016-01-01

    Summary Background It is imperative that bone tumour margin and extent of tumour involvement are accurately assessed pre-operatively in order for the surgeon to attain a safe surgical margin. In this study, we comprehensively assessed each of the findings that influence surgical planning, on various MRI sequences and compared them with the gold standard – pathology. Material/Methods In this prospective study including 21 patients with extremity bone tumours, margins as seen on various MRI sequences (T1, T2, STIR, DWI, post-gadolinium T1 FS) were measured and biopsies were obtained from each of these sites during the surgical resection. The resected tumour specimen and individual biopsy samples were studied to assess the true tumour margin. Margins on each of the MRI sequences were then compared with the gold standard – pathology. In addition to the intramedullary tumour margin, we also assessed the extent of soft tissue component, neurovascular bundle involvement, epiphyseal and joint involvement, and the presence or absence of skip lesions. Results T1-weighted imaging was the best sequence to measure tumour margin without resulting in clinically significant underestimation or overestimation of the tumour extent (mean difference of 0.8 mm; 95% confidence interval between −0.9 mm to 2.5 mm; inter-class correlation coefficient of 0.998). STIR and T1 FS post-gadolinium imaging grossly overestimated tumour extent by an average of 16.7 mm and 16.8 mm, respectively (P values <0.05). Post-gadolinium imaging was better to assess joint involvement while T1 and STIR were the best to assess epiphyseal involvement. Conclusions T1-weighted imaging was the best sequence to assess longitudinal intramedullary tumour extent. We suggest that osteotomy plane 1.5 cm beyond the T1 tumour margin is safe and also limits unwarranted surgical bone loss. However, this needs to be prospectively proven with a larger sample size. PMID:28058070

  14. Levels of Evidence in the Treatment of Slipped Capital Femoral Epiphysis: A Systematic Review

    PubMed Central

    Moriarity, Andrew; Kennedy, Jim; Baker, Joe; Kiely, Pat

    2016-01-01

    The primary aim of this study was to analyze the current level of evidence available on the surgical management of Slipped Capital Femoral Epiphysis (SCFE). Secondary aims were to correlate the level of evidence with the impact factor of the journal to evaluate the level of evidence over time, and to evaluate the geographic distribution of the studies. Therapeutic studies published in English between January 1991 and August 2014 that reported on SCFE were identified via electronic search was performed using the databases PubMed, EMBASE, and the Cochrane Library. The search terms used included: Slipped capital femoral epiphyses OR SCFE OR Slipped upper femoral epiphyses OR SUFE AND Management OR Treatment. Correlation between the level of evidence and the impact factor of the journal were analyzed together with linear regression models to reveal any significant trends over time. A total of 1516 studies were found, of which 321 were included in the final analysis. The most frequent study type was the case series (51.1%) followed by case reports (22.4%) and expert opinion (14.0%). Randomized control trial accounted for only 0.6%. The Journal of Pediatric Orthopedics (American) had the most studies (22.6%) and the highest number of level 2 (n=1) and level 3 (n=15) type evidence. There was no progression of level of evidence over time. There was no correlation between level of evidence and impact factor of journal. The majority of therapeutic studies on SCFE are of low level of evidence. High-level RCTs are difficult to perform in pediatric orthopedic surgery, however the management of SCFE would benefit from well-designed, multicenter, clinical RCTs to advance evidence-based practice. PMID:27433299

  15. Expression of mRNAs coding for the alpha 1 chain of type XIII collagen in human fetal tissues: comparison with expression of mRNAs for collagen types I, II, and III

    PubMed Central

    1989-01-01

    This paper describes the topographic distribution of the multiple mRNAs coding for a novel human short-chain collagen, the alpha 1 chain of type XIII collagen. To identify the tissues and cells expressing these mRNAs, human fetal tissues of 15-19 gestational wk were studied by Northern and in situ hybridizations. The distribution pattern of the type XIII collagen mRNAs was compared with that of fibrillar collagen types I, II, and III using specific human cDNA probes for each collagen type. Northern hybridization showed the bone, cartilage, intestine, skin, and striated muscle to contain mRNAs for type XIII collagen. An intense in situ hybridization signal was obtained with the type XIII collagen cDNAs in the epidermis, hair follicles, and nail root cells of the skin, whereas the fibrillar collagen mRNAs were detected in the dermis. Cells in the intestinal mucosal layer also appeared to contain high levels of alpha 1(XIII) collagen mRNAs, but contained none of the fibrillar collagen mRNAs. In the bone and striated muscle, alpha 1(XIII) collagen mRNAs were detected in the mesenchymal cells forming the reticulin fibers of the bone marrow and endomycium. The hybridization signal obtained with the alpha 1(XIII) collagen cDNA probe in cartilaginous areas of the growth plates was similar, but less intense, to that obtained with the type II collagen probe. A clear hybridization signal was also detected at the (pre)articular surfaces and at the margins of the epiphyses, whereas it was weaker in the resting chondrocytes in the middle of the epiphyses. The brain, heart, kidney, liver, lung, placenta, spleen, testis, tendon, and thymus did not appear to contain alpha 1(XIII) collagen mRNAs. PMID:2768343

  16. Trafficking defects and loss of ligand binding are the underlying causes of all reported DDR2 missense mutations found in SMED-SL patients.

    PubMed

    Ali, Bassam R; Xu, Huifang; Akawi, Nadia A; John, Anne; Karuvantevida, Noushad S; Langer, Ruth; Al-Gazali, Lihadh; Leitinger, Birgit

    2010-06-01

    Spondylo-meta-epiphyseal dysplasia (SMED) with short limbs and abnormal calcifications (SMED-SL) is a rare, autosomal recessive human growth disorder, characterized by disproportionate short stature, short limbs, short broad fingers, abnormal metaphyses and epiphyses, platyspondyly and premature calcifications. Recently, three missense mutations and one splice-site mutation in the DDR2 gene were identified as causative genetic defects for SMED-SL, but the underlying cellular and biochemical mechanisms were not explored. Here we report a novel DDR2 missense mutation, c.337G>A (p.E113K), that causes SMED-SL in two siblings in the United Arab Emirates. Another DDR2 missense mutation, c.2254C>T (p.R752C), matching one of the previously reported SMED-SL mutations, was found in a second affected family. DDR2 is a plasma membrane receptor tyrosine kinase that functions as a collagen receptor. We expressed DDR2 constructs with the identified point mutations in human cell lines and evaluated their localization and functional properties. We found that all SMED-SL missense mutants were defective in collagen-induced receptor activation and that the three previously reported mutants (p.T713I, p.I726R and p.R752C) were retained in the endoplasmic reticulum. The novel mutant (p.E113K), in contrast, trafficked normally, like wild-type DDR2, but failed to bind collagen. This finding is in agreement with our recent structural data identifying Glu113 as an important amino acid in the DDR2 ligand-binding site. Our data thus demonstrate that SMED-SL can result from at least two different loss-of-function mechanisms: namely defects in DDR2 targeting to the plasma membrane or the loss of its ligand-binding activity.

  17. Femoral remodeling may influence patient outcomes in slipped capital femoral epiphysis.

    PubMed

    DeLullo, James A; Thomas, Eric; Cooney, Timothy E; McConnell, Sharon J; Sanders, James O

    2007-04-01

    Clinical studies of patients treated for slipped capital femoral epiphysis have found limited functional impairment and femoral neck deformity causing eventual coxarthrosis. Since patient-focused assessments minimize bias and reflect health-related quality of life status, we coupled their use to a clinical examination to obtain a more patient-centered picture of slipped capital femoral epiphyseal outcomes. The impact of residual deformity on outcomes also was examined. Of 78 patients treated for slipped capital femoral epiphyses between 1972 and 1998, 29 (38 hips) were evaluated at a mean followup of 7.6 years (range, 1.4-26 years). The average patient age was 21.8 years (range, 14.6-39 years), 55% were female, and the average body mass index was 28.7 (range, 16.1-50.2). Most slips were stable (92%, 35 of 38) and mild or moderate in severity (98%, 36 of 37). Followup examinations revealed slight deficits in range of motion, strength, and limb length. Radiographs showed slight improvements in head-shaft angle and reduced but persistent femoral neck deformity. Osteoarthritic changes were absent or negligible in 84% (32 of 38) of the hips. The average Iowa hip score was 90.5 (range, 51-100). Patient outcome scores for the AAOS Hip/Knee Questionnaire fell slightly below 50th percentile norms. Neither slip stability, severity, nor body mass index impacted outcome. Femoral neck deformity correlated with function, pain, and Boyer grade. Overall, patients had minor functional deficits and pain that may have been related to femoral neck deformity, but longer followup is warranted.

  18. Levels of Evidence in the Treatment of Slipped Capital Femoral Epiphysis: A Systematic Review.

    PubMed

    Moriarity, Andrew; Kennedy, Jim; Baker, Joe; Kiely, Pat

    2016-06-27

    The primary aim of this study was to analyze the current level of evidence available on the surgical management of Slipped Capital Femoral Epiphysis (SCFE). Secondary aims were to correlate the level of evidence with the impact factor of the journal to evaluate the level of evidence over time, and to evaluate the geographic distribution of the studies. Therapeutic studies published in English between January 1991 and August 2014 that reported on SCFE were identified via electronic search was performed using the databases PubMed, EMBASE, and the Cochrane Library. The search terms used included: Slipped capital femoral epiphyses OR SCFE OR Slipped upper femoral epiphyses OR SUFE AND Management OR Treatment. Correlation between the level of evidence and the impact factor of the journal were analyzed together with linear regression models to reveal any significant trends over time. A total of 1516 studies were found, of which 321 were included in the final analysis. The most frequent study type was the case series (51.1%) followed by case reports (22.4%) and expert opinion (14.0%). Randomized control trial accounted for only 0.6%. The Journal of Pediatric Orthopedics (American) had the most studies (22.6%) and the highest number of level 2 (n=1) and level 3 (n=15) type evidence. There was no progression of level of evidence over time. There was no correlation between level of evidence and impact factor of journal. The majority of therapeutic studies on SCFE are of low level of evidence. High-level RCTs are difficult to perform in pediatric orthopedic surgery, however the management of SCFE would benefit from well-designed, multicenter, clinical RCTs to advance evidence-based practice.

  19. Corrections of diverse forms of lower limb deformities in patients with mucopolysaccharidosis type IVA (Morquio syndrome)

    PubMed Central

    Al Kaissi, Ali; Kenis, Vladimir; Melchenko, Eugeniy; Ghachem, Maher Ben; Csepan, Robert; Grill, Franz; Ganger, Rudolf

    2016-01-01

    Background: Thoracolumbar kyphosis has been considered as the first presenting deformity and is often a key diagnostic clue noted in children with mucopolysaccharidosis (MPS) type IV (Morquio's syndrome). However, we observed that the progressive irregularities of the epiphyses of the long bones were the most prominent skeletal pathology, causing effectively the development of diverse forms of lower limbs deformities with extreme variation in age of onset. Materials and Methods: Ten patients (seven children and three adults) with an average age of 15 years have been enrolled in this study. Age of diagnosis of MPS IVA has a variable age of onset and a MISLEADING rate of severity. Hip dislocations, genu valgum, protrusio acetabuli and osteoarthritis were the most common lower limbs deformities in these patients. Clinical and radiographic phenotypes were the baseline tools of documentation. Urinary screening and genotypic characterizations have been applied accordingly. Results: Combined pelvic and femoral procedures for hip dislocation, epiphysiodeses and supracondylar osteotomy for genu valgum and hip arthroplasty for protrusio acetabuli have been performed. All patients manifested insufficient activity of N-acetylgalactosamine-6-sulphate sulphatase, an enzyme that degrades keratin sulphate and chondroitin-6 sulphate. Conclusion: The extensive clinical heterogeneity contributed significantly in the delay in establishing the diagnosis particularly in adult patients with MPS IV. The epiphyseal irregularities of the long bones and the progressive flattening pathology of MPS IV A were the reason to falsely diagnose some patients as spondyloepiphyseal dysplasia congenital and/or tarda. Proximal femoral osteotomy, realignment osteotomy and total hip arthroplasty have been performed for coxa vara, genu valgum and protrusio acetabuli, respectively, in children and adult group of patients. The importance of early diagnosis on MPS IV A is to receive enzyme replacement therapy

  20. Prenatal ethanol exposure disrupts the histological stages of fetal bone development.

    PubMed

    Snow, M E; Keiver, K

    2007-08-01

    Maternal ethanol intake during pregnancy results in impairments in general growth and skeletal development in the offspring. We have previously shown that ethanol retards skeletal ossification at doses lower than those that affect growth. Moreover, skeletal sites vary in their sensitivity to ethanol effects, with more severe effects occurring in bones that undergo a greater proportion of their development in utero. Taken together, these data suggest that ethanol has specific effects on bone development, and that later stages in the ossification process may be particularly affected. Such effects could have important implications for the offspring's long-term bone health, as studies suggest that the intrauterine environment can program the skeleton. The present study examined the histological stages of bone development to determine if prenatal ethanol exposure alters the morphological development of the growth plate in the fetal rat. Rats were fed a liquid diet containing ethanol (Ethanol, E group), or without ethanol (Pair-Fed, PF, or Control, C groups) for 6 weeks: 3 weeks prior to breeding and during 3 weeks of pregnancy. Fetal tibiae were fixed, decalcified and stained for histological analysis on day 21 of gestation. Maternal ethanol intake resulted in a significant decrease in fetal total bone and diaphysis lengths, compared with tibiae from PF and C fetuses. Although the lengths of the epiphyses were not affected, ethanol disrupted the organization of the histological zones within the epiphyses. Prenatal ethanol exposure decreased the length of the resting zone, but increased the length of the hypertrophic zone. Enlargement of the hypertrophic zone is consistent with an effect of ethanol on the later stages of bone development; however, ethanol's effect on the resting zone indicates that earlier stages of bone development may also be disrupted. The functional significance of these morphological changes to long-term bone health remains to be determined.

  1. Computer-generated radiological imagery of the structure of the spongious substance in the postnatal development of the tibiotarsal bones of the Peking domestic duck (Anas platyrhynchos var. domestica).

    PubMed

    Charuta, A; Dzierzecka, M; Majchrzak, T; Czerwinski, E; Cooper, R G

    2011-04-01

    The evaluation of the structure of the spongious substance of the tibiotarsal (TT) bones of the domestic duck aged 4 to 8 wk was performed using radiological analysis. The Trabecula program (Czerwiński, 1994) used in the study identified a map of radiological trabeculae and calculated the number, average volume, density, and width of trabeculae. It was stated that the number of trabeculae differed significantly (P ≤ 0.05) variant on age, sex, and a unique fragment of the studied bone. Six-week-old hens whose TT bones were most often exposed to deformities and fractures possessed attenuated bone mass. The number of trabeculae per 1 mm(2) during breeding was the lowest (10.34 and 9.54 mm(2) in the proximal and distal epiphyses, respectively). The tibial bones of the 6-wk-old hens also possessed the lowest volume of trabeculae (44.62 and 39.84% for the proximal and distal epiphyses, respectively). Dependant variances between the BW, the number of recognized radiological trabeculae, and the volume, density, and width of trabeculae were calculated using a selected correlation and regression coefficient (r = 0.41; P ≤ 0.05). Results expounded a unique linear relationship between BW and the volume of trabeculae. Indeed, the larger the BW, the more numerous the trabeculae observed. No significant correlation was determined between the BW and the number of recognized trabeculae nor their density and width. A small number of trabeculae and the lowered density may be the cause of fractures and deformities of the TT bones of the domestic duck.

  2. Growth in patients with isolated gonadotrophin deficiency.

    PubMed Central

    Dickerman, Z; Cohen, A; Laron, Z

    1992-01-01

    The growth pattern of 66 patients (50 males, 16 females) with isolated gonadotrophin deficiency (IGnD), who had reached their final height with epiphyseal closure, was evaluated. For the purpose of analysis the males were divided into two groups according to age at referral: group 1 less than 16 years (n = 23) and group 2 greater than or equal to 16 years (n = 27). Sex hormone treatment was initiated at a mean (SD) chronological age of 15.8 (1.3) and 18.6 (1.2) years in groups 1 and 2 in the males and at 15.3 (1.3) years in the females. The duration of treatment (until epiphyseal closure) in the males was 3.9 (1.5) years in group 1 and 2.1 (1.0) years in group 2 and 2.8 (1.3) years in the females. There was no significant difference between the mean final height in groups 1 and 2, but it was significantly higher than the mean parental height (mean height SD score (HtSDS): 0.1 (1.1) v -0.8 (0.9)) and they were significantly correlated. For females the mean HtSDS compared with parental height was 0.4 (1.5) v -0.6 (1.2). It is concluded that the timing of induction of puberty by sex hormones in males and females with IGnD has no significant effect on final height provided that moderate doses are used. Furthermore final height was significantly correlated to mid-parental height. PMID:1580683

  3. Intraepiphyseal resection of the proximal tibia and its impact on lower limb growth.

    PubMed

    Manfrini, M; Gasbarrini, A; Malaguti, C; Ceruso, M; Innocenti, M; Bini, S; Capanna, R; Campanacci, M

    1999-01-01

    From 1989 through 1996, 10 children affected by high grade bone tumors of the proximal tibia underwent an intraepiphyseal intercalary resection. The residual epiphyseal bone segment measured less than 2 cm in thickness in all cases and reconstruction always was performed using the combination of a vascularized fibular autograft and a massive bone allograft. The proximal epiphyseal osteosynthesis was fixed by small fragment screws. The aim of this study was to report the growth pattern of the residual proximal tibial epiphysis and to evaluate any possible lower limb discrepancy and/or deformity after the end of skeletal maturity. At current followup six patients were available for the final evaluation. Radiographic documentation included computed tomography scan of both knees before surgery, a panoramic radiographic view and a computed tomography scan of both lower limbs after the end of skeletal growth. The length of both femurs and tibias, the size of the tibial plateau and of the opposite distal femur, and any possible deformity of femur or tibia were measured and compared with the preoperative data. No patient had a limb length discrepancy greater than 3.5 cm. In all cases the ipsilateral femur had a valgus deformity of the hip develop. In two patients this deformity was associated with an elongation of the femur, partially compensating for the shortening of the tibia. The tibial plateau close to reconstruction grew less than the contralateral one (range 2%-8%) but maintained its normal relationship with the distal femur. None of these patients reported any restriction in recreational activities. They could walk, run, and jump. Their functional result according the International Society of Limb Salvage functional grading system was satisfactory in all cases.

  4. Structural features of bone marrow

    PubMed Central

    Romaniuk, Anatolii; Lyndina, Yuliia; Sikora, Vladyslav; Lyndin, Mykola; Karpenko, Ludmyla; Gladchenko, Oksana; Masalitin, Igor

    2016-01-01

    Purpose This article is devoted to the investigation of the structural features of the bone marrow of mature rats. Materials and methods The investigation of the structural features of the bone marrow was performed on the femurs of the mature male rats. General structure of the organ was studied with hematoxylin–eosin and Van Gieson staining of samples. Certain features of the bone marrow structure were studied using immunohistochemical method (CD3, CD79α, S100, myeloperoxidase, and cyclin D1). Results We can state that stromal–parenchymal structure is typical for the bone marrow of rats as for any other organ. The stromal component is presented with bone tissue (48.8 ± 3.3% at epiphyses), the net of blood vessels (18.7 ± 2.1%), fat tissue (11 ± 2%), fibrous tissue (0.7 ± 0.2%), and the network of reticular fibers. Hematopoietic tissue covers 20.9 ± 3.7% at the femoral epiphyses and 69.6 ± 2.2% at diaphysis. Among these tissues, myelopoiesis occupies 74.2 ± 4.7%, erythropoiesis – 24.3 ± 4.7%, and lymphopoiesis – less than 5%. Megalokaryocytes take 0.1–0.3%. Conclusion Considering the lack of significant anatomical, morphological, and histological differences of red bone marrow of rats and humans, we can state that hematopoiesis in rats takes place on the basis of the same principles as in humans, although it has certain mechanisms. PMID:28203394

  5. Undersulfation of proteoglycans synthesized by chondrocytes from a patient with achondrogenesis type 1B homozygous for an L483P substitution in the diastrophic dysplasia sulfate transporter.

    PubMed

    Rossi, A; Bonaventure, J; Delezoide, A L; Cetta, G; Superti-Furga, A

    1996-08-02

    Achondrogenesis type 1B is an autosomal recessive, lethal chondrodysplasia caused by mutations in the gene encoding a sulfate/chloride antiporter of the cell membrane (Superti-Furga, A., Hästbacka, J., Wilcox, W. R., Cohn, D. H., van der Harten, J. J., Rossi, A., Blau, N., Rimoin, D. L., Steinmann, B., Lander, E. S., and Gitzelmann, R.(1996) Nat. Genet. 12, 100-102). To ascertain the consequences of the sulfate transport defect on proteoglycan synthesis, we studied the structure and sulfation of proteoglycans in cartilage tissue and in fibroblast and chondrocyte cultures from a fetus with achondrogenesis 1B. Proteoglycans extracted from epiphyseal cartilage and separated on agarose gels migrated more slowly than controls and stained poorly with alcian blue. The patient's cultured cells showed reduced incorporation of [35S]sulfate relative to [3H]glucosamine, impaired uptake of sulfate, and higher resistance to chromate toxicity compared to control cells. Epiphyseal chondrocytes cultured in alginate beads synthesized proteoglycans of normal molecular size as judged by gel filtration chromatography, but undersulfated as judged by ion exchange chromatography and by the amount of nonsulfated disaccharide. High performance liquid chromatography analysis of chondroitinase-digested proteoglycans showed that sulfated disaccharides were present, although in reduced amounts, indicating that at least in vitro, other sources of sulfate can partially compensate for sulfate deficiency. A t1475c transition causing a L483P substitution in the eleventh transmembrane domain of the sulfate/chloride antiporter was present on both alleles in the patient who was the product of a consanguineous marriage. The results indicate that the defect of sulfate transport is expressed in both chondrocytes and fibroblasts and results in the synthesis of proteoglycans bearing glycosaminoglycan chains which are poorly sulfated but of normal length.

  6. Novel case of Trevor’s disease: Adult onset and later recurrence

    PubMed Central

    Khalsa, Amrit S; Kumar, Neil S; Chin, Matthew A; Lackman, Richard D

    2017-01-01

    Dysplasia epiphysealis hemimelica (DEH), or Trevor’s disease, is an osteocartilaginous epiphyseal overgrowth typically occurring in children. The literature reports 6 adult cases and none describe recurrence requiring additional procedures. We present a new-onset proximal tibial DEH in an adult recurring approximately 3 years after open excision. A 39-year-old female presented with a history of right knee pain, swelling, and instability. Physical examination revealed a firm proximal tibial mass. Computed tomography (CT) imaging showed an exophytic, lobulated, sclerotic mass involving the anterolateral margin of the lateral tibial plateau. Magnetic resonance imaging was suggestive of an osteochondroma. The patient underwent curettage of the lesion due to its periarticular location. Histology revealed benign and reactive bone and cartilage consistent with periosteal chondroma. Two and a half years later, the patient presented with a firm, palpable mass larger than the initial lesion. CT revealed a lateral tibial plateau sclerotic mass consistent with recurrent intra-articular DEH. A complete excision was performed and histology showed sclerotic bone with overlying cartilage consistent with exostosis. DEH is a rare epiphyseal osteocartilaginous outgrowth frequently occurring in the long bones of children less than 8 years old. DEH resembles an osteochondroma due to its pediatric presentation and similar histologic appearance. Adult-onset cases comprise less than 1% of reported cases. Recurrence rate after surgical intervention is unknown. Only 1 such case, occurring in a child, has been described. Clinicians contemplating operative treatment for DEH should note the potential for recurrence and consider complete excision. A follow-up period of several years may be warranted to identify recurrent lesions. PMID:28144583

  7. Fermented soybean product (Cheonggukjang) improved some attributes of protein and growth hormone measurements in Sprague-Dawley rats.

    PubMed

    Hwang, In Sik; Kim, Ji Eun; Lee, Young Ju; Kwak, Moon Hwa; Go, Jun; Son, Hong Joo; Kim, Dong Sup; Hwang, Dae Youn

    2014-04-01

    We hypothesized that the administration of Cheonggukjang (CKJ) would exert positive effects on factors implicated with growth in Sprague-Dawley (SD) rats. To test this hypothesis, we measured specific aspects of bone and organ growth in male SD rats that were treated for 6 weeks with 3 concentrations of CKJ. Although the CKJ extract contained high concentrations of flavonoids and phenolic compounds, no significant differences in body length, organ weights, or femur weight were detected between the CKJ- and vehicle-treated groups. However, thicknesses of the epiphyseal growth plate in the proximal femoral epiphysis and the compact bone in the linea aspera were broadest in the femur of the 2 CKJ-treated groups when compared with the vehicle-treated groups. Furthermore, the levels of growth hormone (GH) and calcium ion were higher in the sera of the high-concentration CKJ-treated groups, whereas the expression level of GH receptor was higher in muscle tissue of all CKJ-treated groups and in the liver tissue of the high-concentration CKJ-treated group. In the GH receptor downstream signaling pathway, the phosphorylation levels of Akt and Erk were expressed differently between liver and muscle tissues upon CKJ treatment. However, the phosphorylation level of STAT5 was very similar to the expression level of the GH receptor in all CKJ-treated groups. These results indicate that CKJ extract may increase the thickness of the epiphyseal growth plate and the compact bone of the femur, elevate GH secretion, and stimulate regulation of the GH receptor downstream signaling pathway in the liver and muscle tissues of SD rats.

  8. Failure of tension band plating: a case series.

    PubMed

    Masquijo, Julio J; Firth, Gregory B; Sepúlveda, Dalia

    2016-07-08

    Growth modulation with tension band plates (TBP) has been shown to be a very useful method for the treatment of angular deformities in growing children. Recently, we have observed cases of failure where the epiphyseal screw was drawn through the physis into the metaphysis. This study describes a series of children who developed this complication. Patients who developed TBP failure after operative treatment of lower limb angular deformities were identified from the databases at four institutions over a 5-year period. The medical records were reviewed to record demographics, primary diagnoses, details of the operative procedure, development of physeal arrest, and recurrence of the original deformity. Six patients (five girls) with nine implant failures were identified. The mean age of the children at the time of implant insertion was 7.2 years (range, 4-10 years). The primary diagnoses included hypophosphatemic rickets (n=7), congenital pseudoarthrosis of the tibia associated with neurofibromatosis 1 (n=1), and post-traumatic malunion after distal tibial fracture (n=1). Of the nine TBP that presented with the complication, four were inserted into the medial distal femur (one bilateral case), two into the medial proximal tibia (one bilateral case), two into the lateral distal tibia, and one into the medial distal tibia. None of these patients developed physeal growth arrest at the last follow-up as assessed on the latest radiographs. The use of TBP for guided growth in patients younger than 10 years old with rickets, neurofibromatosis, or other conditions that produce osteopenia leads to an increased risk for implant failure. In these cases, it is important to confirm that the epiphyseal screw has good purchase. Patients with these features should be monitored closely for early detection of this complication.

  9. Expandable endoprosthetic reconstruction of the skeletally immature after malignant bone tumor resection.

    PubMed

    Eckardt, J J; Safran, M R; Eilber, F R; Rosen, G; Kabo, J M

    1993-12-01

    The mainstay of local control of primary bone malignancies in the skeletally immature has been amputation or, in selected cases, rotationplasty. The development of expandable endoprostheses has permitted an alternative approach for local control in the growing child. Between January 1985 and December 1987, 12 skeletally immature patients with primary malignant bone tumors were treated with extremity reconstruction with cemented custom-expandable endoprostheses after wide resection of their lesions. All patients were observed until death (four) or revision (two) with a minimum two-year follow-up period for the survivors (average, 3.1 years). Seven patients have undergone a total of 11 expansions and one patient was lengthened with a revision-expandable prosthesis. Four patients have not needed expansion. Eight patients have had a total of ten complications. Seven of the ten complications (70%) were prosthesis related and associated with failure of the expansion mechanism. The Musculoskeletal Tumor Society (MSTS) overall rating was good to excellent in seven patients (58%), fair in three (25%), and poor in two (17%). In five distal femoral arthroplasties and one total femoral arthroplasty where the tibial bearing component was cemented through the physis, tibial and epiphyseal growth was observed to be normal and equal to the nonoperative side. This suggests that partial central epiphyseal and physeal ablation does not cause physeal arrest. Although the high rate of expansion mechanism failure necessitates redesign, preliminary results suggest that expandable endoprostheses do offer an alternative to amputation and rotationplasty as a means of local control and extremity reconstruction in children with primary malignant bone tumors.

  10. Development of rat tibia innervation: colocalization of autonomic nerve fiber markers with growth-associated protein 43.

    PubMed

    Gajda, Mariusz; Litwin, Jan A; Tabarowski, Zbigniew; Zagólski, Olaf; Cichocki, Tadeusz; Timmermans, Jean-Pierre; Adriaensen, Dirk

    2010-01-01

    Development of autonomic innervation of the tibia was investigated in rat fetuses on gestational days (GD) 17-21 and in juvenile animals on postnatal days (PD) 1-28. Double immunofluorescence combined with confocal microscopy was applied to study colocalization of neuronal growth- associated protein 43 (GAP-43) and panneuronal marker protein gene product 9.5 (PGP) with markers of the autonomic nervous system: neuropeptide Y (NPY) and dopamine beta-hydroxylase (DbetaH) for adrenergic, as well as vasoactive intestinal polypeptide (VIP) and vesicular acetylcholine transporter (VAChT) for cholinergic fibers. The first GAP-43-immunoreactive (GAP-IR) nerve fibers were seen on GD17 in the perichondrium of the proximal epiphysis. Further GAP- and PGP-IR innervation appeared in the perichondrium/periosteum of the diaphysis and in the distal epiphysis (GD19), then in the bone marrow and in the intercondylar eminence (GD21). On PD1, NPY-IR and DbetaH-IR fibers appeared within the diaphyseal periosteum and on PD4 within the bone marrow. From PD14, GAP-43 immunoreactivity of NPY-positive fibers decreased. From PD7 on, NPY-IR fibers were observed in cartilage canals of both epiphyses and in the intercondylar eminence. In secondary ossification centers, NPY-IR fibers were seen from PD10, and in the bone marrow of the epiphyses from PD14. First VIP-IR and VAChT-IR fibers were observed on PD4 within the periosteum, bone marrow and patellar ligament. From PD10 on, VIP-positive fibers were seen in the intercondylar eminence, and from PD14 in secondary ossification centers. GAP-43 proved to be superior to PGP 9.5 as marker of growing nerve fibers, mostly due to its earlier appearance. The presence of specific nerve fibers may suggest possible involvement of autonomic innervation in regulation of bone development.

  11. The pathology of acute chondro-osseous injury in the child.

    PubMed Central

    Ogden, J. A.; Ganey, T.; Light, T. R.; Southwick, W. O.

    1993-01-01

    Skeletal tissues from children sustaining acute skeletal trauma were analyzed with detailed radiologic and histologic techniques to assess the failure patterns of the developing skeleton. In the physis- and epiphysis-specific fracture propagation varied, usually going through the portion of the hypertrophic zone adjacent to the metaphysis. However, the physeal fracture in types 1 and 2 sometimes involved the germinal zone. There may also be microscopic propagation at oblique angles from the primary fracture plane, splitting cell columns apart longitudinally. The cartilage canals supplying the germinal zone appear to be "weak" areas into which the fracture may propagate, especially in infancy. Incomplete type 1 physeal fractures, which cannot be detected by routine radiography, may occur. Types 1, 2, and 4 physeal injuries may be comminuted. In type 3 injuries, discrete segments of physis that include the germinal zone may "adhere" to the metaphysis, separating the cells from their normal vascularity. In types 2 and 3, comminution may occur at the site of fracture redirection from the physis. Direct type 5 crushing of the physeal germinal zone does not occur, even in the presence of significant pressure-related changes within other areas of the epiphysis. Type 7 separation between cartilage and bone at any chondro-osseous epiphyseal interface may occur, but is similarly impossible to diagnose radiographically. In the metaphysis torus, fractures result from plastic deformation of the cortex, coupled with a partial microfracturing that may be difficult to visualize with clinical radiography. Some of the energy absorption may also be transmitted to the physis, causing metaphyseal hemorrhage adjacent to the growth plate and variable microscopic damage within the physis. In the diaphysis, the greenstick fracture is associated with longitudinal tensile failure through the developing osteons of the "intact" cortex. The inability of these failure patterns to "narrow" after

  12. Mild exercise early in life produces changes in bone size and strength but not density in proximal phalangeal, third metacarpal and third carpal bones of foals.

    PubMed

    Firth, Elwyn C; Rogers, Christopher W; van Weeren, P Rene; Barneveld, Albert; McIlwraith, C Wayne; Kawcak, Christopher E; Goodship, Allen E; Smith, Roger K W

    2011-12-01

    Exercise or lack of it in early life affects chondro-osseous development. Two groups of horses were used to investigate the effects of age and exercise regimen on bone parameters of diaphyseal, metaphyseal, epiphyseal and cuboidal bones of the distal limb of Thoroughbreds. One group had exercised only spontaneously from an early age at pasture (PASTEX group), while the other group of horses were exposed to a 30% greater workload through additional defined exercise (CONDEX). Longitudinal data from peripheral quantitative computed tomography (pQCT) were obtained from eight scan sites of the left forelimb (proximal phalangeal (P(p); 1 site), third metacarpal (Mc3; six sites) and third carpal (C(3); one site) bones) of 32 Thoroughbred foals scanned five times from ∼3 weeks to 17 months of age. The primary outcome measures were bone mineral content (BMC), bone area (BA), and periosteal circumference (Peri C) in diaphyseal bone, with cortical thickness (CortTh), volumetric bone mineral density (BMD(v)) and a bone strength index (SSI) also being analysed. At the P(p) site within the model there was a significant effect (P=0.00-0.025) of conditioning exercise increasing bone parameters, except endosteal circumference (Endo C) and BMD(v). The BMC, BA, and SSI of P(p) were significantly greater in the CONDEX than PASTEX groups at 12 and 17 months (P=0.015-0.042) and CortTh at 17 months (P=0.033). At the M55 site of Mc3 BMC, BA and SSI (P=0.02-0.04), and at the M33 site, SSI (P=0.05) were higher in the CONDEX than PASTEX group. The adaptive responses, consistent with diaphyseal strengthening, were more marked in the diaphysis of P(p) than Mc3. In the Mc3, metaphysis, trabecular BMD(v) was less in the CONDEX than PASTEX group, associated with greater bone mineral accretion in the outer cortical-sub-cortical bone in the CONDEX group. There were no significant between-group differences in any epiphyseal or cuboidal bone parameter. Although the early imposed exercise regimen

  13. Muscle atrophy and bone loss after 90 days' bed rest and the effects of flywheel resistive exercise and pamidronate: results from the LTBR study.

    PubMed

    Rittweger, Jörn; Frost, Harold M; Schiessl, Hans; Ohshima, Hiroshi; Alkner, Björn; Tesch, Per; Felsenberg, Dieter

    2005-06-01

    Muscle atrophy and bone loss pose substantial problems for long-term space flight and in clinical immobilization. We therefore tested the efficacy of flywheel resistive exercise and pamidronate to counteract such losses. Twenty five young healthy males underwent strict bed rest with -6 degrees head-down tilt for 90 days. Subjects were randomized into an exercise group that practiced resistive exercise with a 'flywheel' (FW) device every 2-3 days, a pamidronate group (Pam) that received 60 mg pamidronate i.v. 14 days prior to bed rest and a control group (Ctrl) that received none of these countermeasures. During the study, Ca(++) and protein intake were controlled. Peripheral quantitative computed tomography (pQCT) was used to assess bone mineral content (BMC) and muscle cross sectional area (mCSA) of calf and forearm. Measurements were taken twice during baseline data collection, after 28 and after 89 days bed rest, and after 14 days recovery. On the same days, urinary Pyridinoline excretion and serum levels of alkaline phosphatase, Ca(++) and PTH were measured. Pre-study exercise habits were assessed through the Freiburg questionnaire. Losses in calf mCSA were significantly reduced in FW (Ctrl: -25.6% +/- 2.5% Pam: -25.6% +/- 3.7%, FW: -17.3% +/- 2.7%), but not in the forearm mCSA (Ctrl: -6.4% +/- 4.33%, Pam: -7.7% +/- 4.1%, FW: -7.6% +/- 3.3%). Both diaphyseal and epiphyseal BMC losses of the tibia were mitigated in Pam and FW as compared to Ctrl, although this was significant only at the diaphysis. Inter-individual variability was significantly greater for changes in BMC than in mCSA, and correlation of BMC losses was poor among different locations of the tibia. A significant positive correlation was found between change in tibia epiphyseal BMC and serum cortisol levels. These findings suggest that both countermeasures are only partly effective to preserve BMC (FW and Pam) and mCSA (FW) of the lower leg during bed rest. The partial efficacy of flywheel exercise

  14. Immunolocalization of the cleavage of the aggrecan core protein at the Asn341-Phe342 bond, as an indicator of the location of the metalloproteinases active in the lysis of the rat growth plate.

    PubMed

    Lee, E R; Lamplugh, L; Leblond, C P; Mordier, S; Magny, M C; Mort, J S

    1998-09-01

    In view of the extensive lysis of hyaline cartilage known to take place during endochondral bone formation, the current study was designed to test the hypothesis that metalloproteinases are the agents that mediate this lysis. Since these enzymes have been shown in vitro to cleave the core protein of the major proteoglycan of cartilage, aggrecan, at the Asn341-Phe342 bond, an immunohistochemical method has been developed to find out whether or not there are sites in the growth plate of the rat tibia where cleavage of this bond takes place. The cleavage of aggrecan by metalloproteinases is followed by the retention of the fragment known as G1, for it includes the G1 domain. Since the G1 fragment terminates in the amino acid residues ...FVDIPEN, we prepared an antiserum against FVDIPEN, confirmed its specificity, then applied it to the growth plate of 21-day-old rat tibia in the hope of localizing the G1 fragments. The antiserum specificity was shown by its recognition of the ...FVDIPEN sequence at the C-terminus of peptides and of G1 fragments produced by aggrecan cleavage. When the antiserum was applied to Western blots of guanidinium chloride extracts prepared from epiphyseal growth plate, it recognized two species (56 and 52 kDa), which differed only in the degree of glycosylation. These fragments were comparable in size to the G1 fragments generated by the action of recombinant metalloproteinase in vitro, thus confirming antiserum specificity for these fragments. Applying the antiserum to cryosections of 21-day-old rat tibiae revealed immunostaining at two intensities within the growth plate matrix: a strong staining was observed in a 1-5 microm-wide layer designated "peripheral" matrix, which borders the epiphyseal and metaphyseal marrow spaces as well as the perichondrium, while a weak staining was found in the rest of the plate, designated "central" matrix. The abundance of G1 fragments terminating in ...FVDIPEN in the peripheral matrix indicates that this is

  15. Estimating age from the pubic symphysis: A new component-based system.

    PubMed

    Dudzik, Beatrix; Langley, Natalie R

    2015-12-01

    The os pubis is one of the most widely used areas of the skeleton for age estimation. Current pubic symphyseal aging methods for adults combine the morphology associated with the developmental changes that occur into the mid-30s with the degenerative changes that span the latter portion of the age spectrum. The most popular methods are phase-based; however, the definitions currently used to estimate age intervals may not be adequately defined and/or accurately understood by burgeoning researchers and seasoned practitioners alike. This study identifies patterns of growth and maturation in the pubic symphysis to derive more precise age estimates for individuals under 40 years of age. Emphasis is placed on young adults to provide more informative descriptions of epiphyseal changes associated with the final phases of skeletal maturation before degeneration commences. This study investigated macroscopic changes in forensically relevant modern U.S. samples of known age, sex, and ancestry from the Maricopa County Forensic Science Center in Phoenix, Arizona as well as donated individuals from the William M. Bass Forensic and Donated Collections at the University of Tennessee, Knoxville (n=237). Age-related traits at locations with ontogenetic and biomechanical relevance were broken into components and scored. The components included the pubic tubercle, the superior apex of the face, the ventral and dorsal demifaces, and the ventral and dorsal symphyseal margins. Transition analysis was applied to elucidate the transition ages between the morphological states of each component. The categorical scores and transition analysis ages were subjected to multinomial logistic regression and decision tree analysis to derive accurate age interval estimates. Results of these analyses were used to construct a decision tree-style flow chart for practitioner use. High inter-rater agreement of the individual component traits (linear weighted kappa values ≥0.665 for all traits in the

  16. Sexual dimorphism of the tibia in contemporary Greek-Cypriots and Cretans: Forensic applications.

    PubMed

    Kranioti, E K; García-Donas, J G; Almeida Prado, P S; Kyriakou, X P; Langstaff, H C

    2017-02-01

    Sex estimation is an essential step in the identification process of unknown heavily decomposed human remains as it eliminates all possible matches of the opposite sex from the missing person's database. Osteometric methods constitute a reliable approach for sex estimation and considering the variation of sexual dimorphism between and within populations; standards for specific populations are required to ensure accurate results. The current study aspires to contribute osteometric data on the tibia from contemporary Greek-Cypriots to assist the identification process. A secondary goal involves osteometric comparison with data from Crete, a Greek island with similar cultural and dietary customs and environmental conditions. Left tibiae from one hundred and thirty-two skeletons (70 males and 62 females) of Greek-Cypriots and one hundred and fifty-seven skeletons (85 males, 72 females) of Cretans were measured. Seven standard metric variables including Maximum length (ML), Upper epiphyseal breadth (UB), Nutrient foramen anteroposterior diameter (NFap), Nutrient Foramen transverse diameter (NFtrsv), Nutrient foramen circumference (NFCirc), Minimum circumference (MinCirc) and Lower epiphyseal breadth (LB) were compared between sexes and populations. Univariate and multivariate discriminant functions were developed and posterior probabilities were calculated for each sample. Results confirmed the existence of sexual dimorphism of the tibia in both samples as well as the pooled sample. Classification accuracy for univariate functions ranged from 78% to 85% for Greek-Cypriots and from 69% to 83% for Cretans. The best multivariate equations after cross-validation resulted in 87% for Greek-Cypriots and 90% accuracy for Cretans. When the samples were pooled accuracy reached 87% with over 95% confidence for about one third of the population. Estimates with over 95% of posterior probability can be considered reliable while any less than 80% should be treated with caution. This

  17. Effects of hyperhomocysteinemia during the gestational period on ossification in rat embryo.

    PubMed

    Azizi, Zabih Allah; Zamani, Ali; Omrani, Ladan R; Omrani, Layla; Dabaghmanesh, Mohammad Hossein; Mohammadi, Alireza; Namavar, Mohammad Reza; Omrani, Gholamhossein R

    2010-05-01

    Severe hyperhomocysteinemia, as seen in classic homocystinuria, is associated with several skeletal malformations and osteopenia. Moreover, hyperhomocysteinemia during pregnancy has been associated with multiple developmental defects in the fetus. This study was undertaken to determine whether offspring of hyperhomocysteinemic mothers have demonstrable changes in bone volume and the epiphyseal growth plate. Ten adult female Sprague-Dawley rats were randomly assigned to the control or experimental group. The experimental group received 100 mg/kg/day of homocysteine in their drinking water for 3 weeks before mating and for the total duration of pregnancy. In each group, three pups per mother were randomly selected. The histomorphometric properties of tibial, radial and vertebral growth plates of newborn rats and the volume fraction of bone were compared between groups. The plasma homocysteine concentration at the end of study was significantly higher in dams in the experimental group (16.42+/-1.5 vs. 4.7+/-1.7 mumol/L, P<0.05). In offspring born to dams given the homocysteine supplement, the volume fraction of bone in the tibia (30.7+/-1.5% vs. 36.8+/-1.9%, P<0.05), radius (29.6+/-1.1% vs. 37.4+/-2%, P<0.05) and vertebra (34.4+/-1.8% vs. 41+/-1.9%, P<0.05) were significantly decreased whereas vertical heights of proliferative (423+/-25.1 vs. 301.8+/-28.1 microm for radius and 131.9+/-5.9 vs. 107.8+/-3.5 microm for vertebra) and hypertrophic zones (213.1+/-12 vs. 163.3+/-7.5 microm for tibia, 153.2+/-7.7 vs. 121.1+/-7.9 microm for radius and 112+/-9.9 vs. 88.4+/-10.1 microm for the vertebra) were increased (P<0.05). The results showed that the administration of homocysteine caused osteopenia in newborn rats. In addition, these data suggest that hyperhomocysteinemia may induce disruption of normal development of epiphyseal cartilage in the rat embryo.

  18. Melanocortin 1 Receptor-Signaling Deficiency Results in an Articular Cartilage Phenotype and Accelerates Pathogenesis of Surgically Induced Murine Osteoarthritis

    PubMed Central

    Hackmayer, Gerit; Greth, Carina; Bauer, Richard J.; Kleinschmidt, Kerstin; Bettenworth, Dominik; Böhm, Markus; Grifka, Joachim; Grässel, Susanne

    2014-01-01

    Proopiomelanocortin-derived peptides exert pleiotropic effects via binding to melanocortin receptors (MCR). MCR-subtypes have been detected in cartilage and bone and mediate an increasing number of effects in diathrodial joints. This study aims to determine the role of MC1-receptors (MC1) in joint physiology and pathogenesis of osteoarthritis (OA) using MC1-signaling deficient mice (Mc1re/e). OA was surgically induced in Mc1re/e and wild-type (WT) mice by transection of the medial meniscotibial ligament. Histomorphometry of Safranin O stained articular cartilage was performed with non-operated controls (11 weeks and 6 months) and 4/8 weeks past surgery. µCT–analysis for assessing epiphyseal bone architecture was performed as a longitudinal study at 4/8 weeks after OA-induction. Collagen II, ICAM-1 and MC1 expression was analysed by immunohistochemistry. Mc1re/e mice display less Safranin O and collagen II stained articular cartilage area compared to WT prior to OA-induction without signs of spontaneous cartilage surface erosion. This MC1-signaling deficiency related cartilage phenotype persisted in 6 month animals. At 4/8 weeks after OA-induction cartilage erosions were increased in Mc1re/e knees paralleled by weaker collagen II staining. Prior to OA-induction, Mc1re/e mice do not differ from WT with respect to bone parameters. During OA, Mc1re/e mice developed more osteophytes and had higher epiphyseal bone density and mass. Trabecular thickness was increased while concomitantly trabecular separation was decreased in Mc1re/e mice. Numbers of ICAM-positive chondrocytes were equal in non-operated 11 weeks Mc1re/e and WT whereas number of positive chondrocytes decreased during OA-progression. Unchallenged Mc1re/e mice display smaller articular cartilage covered area without OA-related surface erosions indicating that MC1-signaling is critical for proper cartilage matrix integrity and formation. When challenged with OA, Mc1re/e mice develop a more severe OA

  19. Angular and axial deformities of the legs of children.

    PubMed

    McDonough, M W

    1984-12-01

    Age is often a determining factor in establishing a treatment program for these axial and angular problems. As can be seen, the deformities of torsion are noticeable from early life. Any tibial torsion should be treated early, but an excessive medial range of motion in the infant leg with a corresponding adequate lateral range of motion of the limb may be cautiously observed. Medial femoral torsion is a normal early finding in the infant thigh. The problem becomes evident as the child matures without the corresponding reduction in femoral torsion, leading to a persistence of fetal or infantile alignment. The gait consequences are usually noticed at 4 to 8 years of age. The angular changes generally are a delayed finding noticed in stance. The bowleg may be associated with marked tibial torsion and picked up early but the Blount's patient has been traditionally definable at 2 years of age. Levin and Drennan may hasten the time of diagnosis with their radiographic criteria. Knock-knee is an alignment disturbance noticed during the early to mid-childhood years, age 4 to 8 years. The diagnosis is important, differentiating physiologic from torsion-related deformities, and treatment, if warranted, should not be delayed. Generally the earlier these problems are discovered, the more optimistic the prognosis. Since the pediatric limb is in a constant state of transition, there will be a perpetual argument as to the need or efficacy of various approaches to the problems of knock-knee and bowleg. If observation is the treatment of choice, the percentage of cases which go on to osteotomies and epiphyseal stapling will continue. For those with axial or angular deformities, degenerative arthritis of the knee may be forthcoming. Swanson, Greene, and Allis warned of problems becoming "unphysiologic." If we consider the epiphyseal malleability, not only to deformity but to correction, we can appreciate Lenoir's comment of "every day the problem goes untreated is a golden

  20. Historical ESWT Paradigms Are Overcome: A Narrative Review.

    PubMed

    Lohrer, Heinz; Nauck, Tanja; Korakakis, Vasileios; Malliaropoulos, Nikos

    2016-01-01

    Extracorporeal Shock Wave Therapy (ESWT) is a conservative treatment modality with still growing interest in musculoskeletal disorders. This narrative review aims to present an overview covering 20-year development in the field of musculoskeletal ESWT. Eight historical paradigms have been identified and put under question from a current perspective: energy intensity, focus size, anesthesia, imaging, growth plates, acuteness, calcifications, and number of sessions. All paradigms as set in a historical consensus meeting in 1995 are to be revised. First, modern musculoskeletal ESWT is divided into focused and radial technology and the physical differences are about 100-fold with respect to the applied energy. Most lesions to be treated are easy to reach and clinical focusing plays a major role today. Lesion size is no longer a matter of concern. With the exception of nonunion fractures full, regional, or even local anesthesia is not helpful in musculoskeletal indications. Juvenile patients can also effectively be treated without risk of epiphyseal damage. Further research is needed to answer the question about if and which acute injuries can be managed effectively. Treatment parameters like the number of sessions are still relying on empirical data and have to be further elucidated.

  1. [Sterno-clavicular plasty in anterior dislocation in child. The new surgical technic and review literature].

    PubMed

    Jiménez, Juan Matus; Guerrero, José Suárez; León, Raúl Torres

    2007-01-01

    The sternoclavicle joint takes part in stability and normal rotation of clavicle in shoulder movement. Its injury infrequent and the luxation in children is more rare. It is classified in previous and retrosternal. The most common causes are by sport trauma and car accidents. In children differential diagnosis includes proximal epiphyseal displacement of clavicle. The clinical picture is pain, sternum or thorax deformity and limitation in range of motion of the arm. The treatment is conservative or surgical, and indications to surgical treatment are pain when moving, range of motion limitation or concomitant complications. In the surgical treatment, the reduction of the clavicle is made with a percutaneous clamp and then protecting the position with a bandage in "eight". Other options are open reduction of the clavicle and subclavian plasty, reduction open and to make plastias with grafts of subclavio,fascia latae or proximal third clavicle resection and cerclage with wire. We present a clinical case and surgical treatment with a novel surgical technique.

  2. Electron microscopic studies on the uptake of exogenous marker particles by different cell types in the guinea pig metaphysis.

    PubMed

    Thyberg, J

    1975-01-01

    Guinea pig metaphyseal bone was exposed to horse spleen ferritin in vitro and to colloidal thorium dioxide in vivo. The cellular uptake and intracellular accumulation of these marker particles were studied ultrastructurally. In vitro, the ferritin molecules were found to spread evely throughout the tissue. After 1-2 hours ferritin was mainly found in plasma membrane invaginations and in endocytic vesicles of varying size. At 4-6 hours a successive accumulation of the marker in secondary lysosomes could be observed. In addition to ferritin, the lysosomes and the large endocytic vesicles often contained other inclusions. In vivo, the pattern of intracellular accumulation of the marker particles was identical to that in vitro. Moreover, the presence within the cells of similar amounts of thorium dioxide after 1 and 4 days suggested that these indigestible molecules are stored intracellularly for a considerable time. In accordance therewith there were no definite signs of extrusion of labeled bodies or secretion of the exogenous marker by exocytosis. Ferritin and thorium dioxide were taken up by all cell types in the metaphysis. Both in vitro and in vivo perivascular cells type B ingested large amounts of marker particles, whereas chondroclasts, endothelial cells. perivascular cells type A and osteoblasts showed a more restricted endocytizing ability. On the basis of these observations, the functional significance of different cell types in the resorption of the epiphyseal cartilage and the formation of bone is discussed.

  3. Evidence for reduced cancellous bone mass in the spontaneously hypertensive rat

    NASA Technical Reports Server (NTRS)

    Wang, T. M.; Hsu, J. F.; Jee, W. S.; Matthews, J. L.

    1993-01-01

    The histomorphometric changes in the proximal tibial metaphysis and epiphyseal growth plate and midtibial shaft of 26-week-old spontaneously hypertensive rats (SHR) compared with those of the corresponding normotensive Wistar-Kyoto (WKY) rats were studied. A decrease in body weight, growth plate thickness, and longitudinal growth rate of the proximal tibial epiphysis, trabecular bone volume, trabecular thickness and number, the number of osteoblasts and osteoprogenitor cells per millimeter square surface of the proximal tibial metaphysis, periosteal and endocortical apposition rate and bone formation rate of the tibial diaphysis were observed in the SHR. Additionally, systolic blood pressure, the number of osteoclasts per millimeter square surface and average number of nuclei per osteoclast of the proximal tibial metaphysis were significantly increased. Thus, osteoclastic activity is dominant over osteoblastic and chondroblastic activity in the SHR that results in a cancellous bone deficit in the skeleton. It will require additional work to ascertain the underlying cause for this condition as several factors in the SHR with a potential for causing this change are present, including elevated parathyroid hormone (PTH), depressed 1,25-(OH)2D3, low calcium absorption, reduced body weight (reduced loading) elevated blood pressure and possibly other direct cell differences in the mutant strain. At present elevated PTH and adaptation to underloading from reduced weight are postulated to be a likely cause, but additional studies are required to test this interpretation.

  4. Menopause causes vertebral endplate degeneration and decrease in nutrient diffusion to the intervertebral discs.

    PubMed

    Wang, Yi-Xiang J; Griffith, James F

    2011-07-01

    The vasculature in the outer annulus supplies only the periphery of the disc so that nutrition to the bulk of the disc, including all the inner annulus and nucleus pulposus, is derived from the vertebral epiphyseal end arteries where nutrients diffuse across the cartilaginous endplate to reach the disc. In this regard the vertebral endplate plays an important role in disc nutrition. Compromise of diffusion of nutrients to the disc cells may play a large part in the progression or even initiation of disc degeneration. Increasing evidence suggests that estrogen deficiency also influence the severity of disc degeneration in post-menopausal females. Structural disorganization of the vertebral endplate occurs with disc degeneration, with the most common endplate changes observed clinically being Schmorl's node. Schmorl's node is more commonly seen in post-menopausal women than younger women. Osteosclerosis, osteonecrosis and fibrosis associated with Schmorl's nodes can impede nutrient diffusion into the disc as well as removal of metabolites from the disc. We hypothesize that menopause negatively affects vertebral endplate quality and induces endplate degeneration. This endplate degeneration decreases nutrients diffusion from vertebral body into discs, and also impedes removal of metabolites, leads to further disc degeneration. To confirm our hypothesis, a cross-sectional post-contrast MRI study can be performed in pre-menopausal and post-menopausal women. If the hypothesis is confirmed, then low dose hormone replacement treatment may retard disc degeneration in post menopausal women and thereby limit the consequences associated with disc degeneration such as low back pain.

  5. Radiographic features of the limbs of juvenile and subadult loggerhead sea turtles (Caretta caretta)

    PubMed Central

    Valente, Ana Luisa; Marco, Ignasi; Zamora, Maria Angeles; Parga, Maria Luz; Lavín, Santiago; Alegre, Ferran; Cuenca, Rafaela

    2007-01-01

    This study aimed to provide the normal radiographic anatomic appearance of the limbs of the loggerhead sea turtle, Caretta caretta. Dorsopalmar and dorsoplantar radiographs were taken of the forelimbs and hindlimbs of 15 juvenile and 15 subadult loggerhead sea turtles, 17 alive and 13 dead. For comparison, computed tomographic, gross anatomic, osteologic, and histologic studies were performed on the limbs of 5 of the sea turtles. Bones from the distal part of the fore and hind flippers were seen in detail with a mammographic film–screen combination. The pectoral and pelvic girdles, superimposed by the carapace, were better seen on standard radiographs with the use of rare-earth intensifying screens. Mammographic radiographs of the manus of 5 small juvenile turtles showed active growth zones. Visualization of bone contours in the distal part of the limbs was clearer than in mammals owing to the very few superimpositions. The presence of a substantial amount of cartilage in the epiphyses produced better visibility of limb ends. We conclude that use of a mammography film–screen combination is the best way to evaluate the bony and joint structures of the limbs of sea turtles. Radiography provides reliable images for clinical purposes. Considering the low cost and logistics of this technique, it is a practical ancillary test for marine animal rehabilitation centers to use. PMID:17955906

  6. Historical ESWT Paradigms Are Overcome: A Narrative Review

    PubMed Central

    Nauck, Tanja; Korakakis, Vasileios; Malliaropoulos, Nikos

    2016-01-01

    Extracorporeal Shock Wave Therapy (ESWT) is a conservative treatment modality with still growing interest in musculoskeletal disorders. This narrative review aims to present an overview covering 20-year development in the field of musculoskeletal ESWT. Eight historical paradigms have been identified and put under question from a current perspective: energy intensity, focus size, anesthesia, imaging, growth plates, acuteness, calcifications, and number of sessions. All paradigms as set in a historical consensus meeting in 1995 are to be revised. First, modern musculoskeletal ESWT is divided into focused and radial technology and the physical differences are about 100-fold with respect to the applied energy. Most lesions to be treated are easy to reach and clinical focusing plays a major role today. Lesion size is no longer a matter of concern. With the exception of nonunion fractures full, regional, or even local anesthesia is not helpful in musculoskeletal indications. Juvenile patients can also effectively be treated without risk of epiphyseal damage. Further research is needed to answer the question about if and which acute injuries can be managed effectively. Treatment parameters like the number of sessions are still relying on empirical data and have to be further elucidated. PMID:27493955

  7. Studies on growth cartilage in the horse and their application to aetiopathogenesis of dyschondroplasia (osteochondrosis)

    PubMed

    Jeffcott, L B; Henson, F M

    1998-11-01

    The importance of osteochondrosis (dyschondroplasia) to the horse industry has been well documented since it was first recorded 50 years ago. The condition is known to be multifactorial in origin, arising from focal failure of endochondral ossification at predilection sites in articular/epiphyseal growth cartilage, but specific information on its aetiopathogenesis is sparse. This paper reviews the current knowledge of growth cartilage metabolism and the process of normal endochondral ossification in the horse. It highlights the localization of various protein products of chondrocytes and the differences in the zones of articular cartilage. In the early focal lesions (referred to as dyschondroplasia) there are alterations in the chondrocytes, extracellular matrix and some of the local protein products. The most obvious feature is an alteration in matrix metabolism which may be responsible for triggering a range of other factors leading to the development of a retained core of cartilage and a primary lesion of dyschondroplasia. Based on available evidence, a preliminary hypothesis for pathogenesis is presented. This suggests that there are a number of factors capable of initiating the condition. One of these involves high circulating insulin levels from high energy feeding which may affect chondrocyte maturation leading to altered matrix metabolism and faulty mineralization resulting in the formation of cartilage cores which characterize the condition. Further research to test this hypothesis is needed before there can be a rational basis for prophylaxis.

  8. Somatic mosaicism in trichorhinophalangeal syndrome: a lesson for genetic counseling

    PubMed Central

    Corsini, Carole; Gencik, Martin; Willems, Marjolaine; Decker, Eva; Sanchez, Elodie; Puechberty, Jacques; Schneider, Anouck; Girard, Manon; Edery, Patrick; Bretonnes, Patricia; Cottalorda, Jérôme; Lefort, Geneviève; Jeandel, Claire; Sarda, Pierre; Genevieve, David

    2014-01-01

    Trichorhinophalangeal syndrome type I (TRPSI) is a genetic disorder characterized by sparse hair, a bulbous nasal tip, short stature with severe generalized shortening of all phalanges, metacarpal and metatarsal bones and cone-shaped epiphyses. This syndrome is caused by autosomal dominant mutations in the TRPS1 gene. However, because recurrence has been observed in siblings from healthy parents, an autosomal recessive mode of inheritance has also been suggested. We report on a male patient, born to healthy unrelated parents, with TRPSI. Using Sanger sequencing, we identified a mutation in the TRPS1 gene (c.2735 G>A, P.Cys912Tyr). The same mutation was detected as a 10% mosaic mutation by Pyrosequencing in blood-derived DNA from his healthy mother. To our knowledge, this is the first time that somatic mosaicism has been identified in TRPSI. This data combined with the observations of recurrences in siblings from healthy parents modifies the genetic counseling for TRPSI, which should discuss a 5–10 percent recurrence risk for healthy parents with an affected child because of the possibility of germinal mosaicism. PMID:23572024

  9. Clinical, biochemical, and genetic analysis of two korean patients with trichorhinophalangeal syndrome type I and growth hormone deficiency.

    PubMed

    Sohn, Young Bae; Ki, Chang-Seok; Park, Sung Won; Cho, Sung-Yoon; Ko, Ah-Ra; Kwon, Min-Jung; Kim, Ji-Youn; Park, Hyung-Doo; Kim, Ok-Hwa; Jin, Dong-Kyu

    2012-01-01

    Tricho-rhino-phalangeal syndrome type I (TRPSI) is a rare autosomal dominant hereditary disorder characterized by sparse hair, bulbous nose, long philtrum, thin upper lip, and skeletal abnormalities including cone-shaped epiphyses, shortening of the phalanges, and short stature. TRPSI is caused by mutations in the TRPS1 gene. Herein, we report two Korean cases of TRPSI. Although both patients (a 17-year-old-female and a 14-year-old male) had typical clinical findings, Patient 1 had an additional growth hormone (GH) deficiency. Treatment with recombinant human growth hormone (rhGH) 0.7 IU/kg/week led to an increase in growth velocity. Over 10 years of GH therapy, the mean growth velocity was 5.7 ± 0.9 cm/year. However, the patient 2 did not show apparent GH deficiency by GH stimulation test, had a poor response with rhGH therapy and GH therapy was discontinued after 6 months. Upon genetic analysis of the TRPS1 gene, two mutations were found. Patient 1 had a heterozygous mutation c.2520dupT (p.Arg841LysfsX3) which had not been previously reported. Patient 2 had a known nonsense mutation c.1630C>T (p.Arg544X). In summary, we were the first to report Korean patients with mutation of TRPS1.

  10. Prenatal ethanol exposure increases osteoarthritis susceptibility in female rat offspring by programming a low-functioning IGF-1 signaling pathway.

    PubMed

    Ni, Qubo; Tan, Yang; Zhang, Xianrong; Luo, Hanwen; Deng, Yu; Magdalou, Jacques; Chen, Liaobin; Wang, Hui

    2015-10-05

    Epidemiological evidence indicates that osteoarthritis (OA) and prenatal ethanol exposure (PEE) are both associated with low birth weight but possible causal interrelationships have not been investigated. To investigate the effects of PEE on the susceptibility to OA in adult rats that experienced intrauterine growth retardation (IUGR), and to explore potential intrauterine mechanisms, we established the rat model of IUGR by PEE and dexamethasone, and the female fetus and 24-week-old adult offspring subjected to strenuous running for 6 weeks were sacrificed. Knee joints were collected from fetuses and adult offspring for histochemistry, immunohistochemistry and qPCR assays. Histological analyses and the Mankin score revealed increased cartilage destruction and accelerated OA progression in adult offspring from the PEE group compared to the control group. Immunohistochemistry showed reduced expression of insulin-like growth factor-1 (IGF-1) signaling pathway components. Furthermore, fetuses in the PEE group experienced IUGR but exhibited a higher postnatal growth rate. The expression of many IGF-1 signaling components was downregulated, which coincided with reduced amounts of type II collagen in the epiphyseal cartilage of fetuses in the PEE group. These results suggest that PEE enhances the susceptibility to OA in female adult rat offspring by down-regulating IGF-1 signaling and retarding articular cartilage development.

  11. New Options for Vascularized Bone Reconstruction in the Upper Extremity

    PubMed Central

    Houdek, Matthew T.; Wagner, Eric R.; Wyles, Cody C.; Nanos, George P.; Moran, Steven L.

    2015-01-01

    Originally described in the 1970s, vascularized bone grafting has become a critical component in the treatment of bony defects and non-unions. Although well established in the lower extremity, recent years have seen many novel techniques described to treat a variety of challenging upper extremity pathologies. Here the authors review the use of different techniques of vascularized bone grafts for the upper extremity bone pathologies. The vascularized fibula remains the gold standard for the treatment of large bone defects of the humerus and forearm, while also playing a role in carpal reconstruction; however, two other important options for larger defects include the vascularized scapula graft and the Capanna technique. Smaller upper extremity bone defects and non-unions can be treated with the medial femoral condyle (MFC) free flap or a vascularized rib transfer. In carpal non-unions, both pedicled distal radius flaps and free MFC flaps are viable options. Finally, in skeletally immature patients, vascularized fibular head epiphyseal transfer can provide growth potential in addition to skeletal reconstruction. PMID:25685100

  12. Genetic linkage analysis of hereditary arthro-ophthalmopathy (Stickler syndrome) and the type II procollagen gene.

    PubMed Central

    Knowlton, R G; Weaver, E J; Struyk, A F; Knobloch, W H; King, R A; Norris, K; Shamban, A; Uitto, J; Jimenez, S A; Prockop, D J

    1989-01-01

    Hereditary arthro-ophthalmopathy (AO), or Stickler syndrome, is a dominantly inherited disorder characterized by vitreo-retinal degeneration and frequently accompanied by epiphyseal dysplasia and premature degenerative joint disease. Three large families with AO were analyzed for clinical manifestations of the disease and for coinheritance of the genetic defect with RFLPs in the type II procollagen gene (COL2A1). Genetic linkage between AO and COL2A1 was demonstrated in the largest family, with a maximum LOD score of 3.52 at a recombination distance of zero. Data from a second family also supported linkage of AO and COL2A1, with a LOD score of 1.20 at a recombination distance of zero. These results are consistent with the conclusion that mutations in the COL2A1 gene are responsible for AO in these two families. In a third AO family, however, recombination between AO and COL2A1 occurred in at least one meiosis, and the data were inconclusive with respect to linkage. Images Figure 2 PMID:2573273

  13. Mechanical Influences on Morphogenesis of the Knee Joint Revealed through Morphological, Molecular and Computational Analysis of Immobilised Embryos

    PubMed Central

    Roddy, Karen A.; Prendergast, Patrick J.; Murphy, Paula

    2011-01-01

    Very little is known about the regulation of morphogenesis in synovial joints. Mechanical forces generated from muscle contractions are required for normal development of several aspects of normal skeletogenesis. Here we show that biophysical stimuli generated by muscle contractions impact multiple events during chick knee joint morphogenesis influencing differential growth of the skeletal rudiment epiphyses and patterning of the emerging tissues in the joint interzone. Immobilisation of chick embryos was achieved through treatment with the neuromuscular blocking agent Decamethonium Bromide. The effects on development of the knee joint were examined using a combination of computational modelling to predict alterations in biophysical stimuli, detailed morphometric analysis of 3D digital representations, cell proliferation assays and in situ hybridisation to examine the expression of a selected panel of genes known to regulate joint development. This work revealed the precise changes to shape, particularly in the distal femur, that occur in an altered mechanical environment, corresponding to predicted changes in the spatial and dynamic patterns of mechanical stimuli and region specific changes in cell proliferation rates. In addition, we show altered patterning of the emerging tissues of the joint interzone with the loss of clearly defined and organised cell territories revealed by loss of characteristic interzone gene expression and abnormal expression of cartilage markers. This work shows that local dynamic patterns of biophysical stimuli generated from muscle contractions in the embryo act as a source of positional information guiding patterning and morphogenesis of the developing knee joint. PMID:21386908

  14. Minimally invasive selective osteotomy of the knee: A new surgical technique.

    PubMed

    Leon, H O; Blanco, C E; Guthrie, T B

    2001-05-01

    We present a simple surgical technique created by the authors to address degenerative chondral lesions of the knee and its application in a limited prospective case series. The technique assumes the concept of beneficial epiphyseal changes caused by disruption of the subchondral bone in improving symptoms, as with drilling, microfracture, periarticular osteotomy, and other invasive procedures. Minimally invasive selective osteotomy (MISO) is an expansion of the arthroscopic treatment of the knee, specifically targeting symptomatic lesions with minimal additional trauma and cost, while avoiding disruption of the articular surface of the subchondral bone. The technique involves a mimimal access approach with selective saw cuts placed with a 1-cm oscillating blade parallel to the joint surface 1 to 1.5 cm deep to identified lesions. The technique does not address malalignment but can address lesions not addressed by classic osteotomies and, as such, may be combined with other corrective alignment procedures as necessary. We present the results of MISO of the knee in a case series of 62 outpatients carried out at the Orthopaedic Division of the Clinical and Surgical Hermanos Ameijeiras Hospital in Havana, Cuba. At 2-year follow-up, there was improvement of symptoms without significant complications.

  15. [Snowboard sports technique, injury pattern, prevention].

    PubMed

    Dingerkus, M L; Imhoff, A; Hipp, E

    1997-02-20

    Although, in Europe, snowboarding is a young sport, it has already established itself and, in common with Alpine skiing, is represented in the Olympics. Its biomechanical aspects (technique, shoeboard connection), lead not only to a typical pattern of movements, but also to a snowboard-specific pattern of injuries, which differs from that seen with Alpine skiing. In the case of snowboarding, the upper limbs are appreciably more often involved in injuries than are the lower extremities. The most common injuries are fractures of or close to the wrist. Since many children and adolescents are to be found among snowboarding fans, the percentage of epiphyseal injuries is high. Depending on the style employed-Alpine or freestyle-the risk of lower limb injuries differ in terms of ankles and knee injuries. Through the use of special protective equipment, such as gloves provided with extra protection for wrist and fingers, (possibly also helmets, knee and elbow protectors for beginners), together with improvements in technique in a snowboard school, and the optimisation of the materials used by the industry, the risk of injury can be reduced.

  16. Fluoride absorption: independence from plasma fluoride levels

    SciTech Connect

    Whitford, G.M.; Williams, J.L.

    1986-04-01

    The concept that there are physiologic mechanisms to homeostatically regulate plasma fluoride concentrations has been supported by results in the literature suggesting an inverse relationship between plasma fluoride levels and the absorption of the ion from the gastrointestinal tract of the rat. The validity of the relationship was questioned because of possible problems in the experimental design. The present work used four different methods to evaluate the effect of plasma fluoride levels on the absorption of the ion in rats: (i) the percentage of the daily fluoride intake that was excreted in the urine; (ii) the concentration of fluoride in femur epiphyses; (iii) the net areas under the time-plasma fluoride concentration curves after intragastric fluoride doses; and (iv) the residual amounts or fluoride in the gastrointestinal tracts after the intragastric fluoride doses. None of these methods indicated that plasma fluoride levels influence the rate or the degree or fluoride absorption. It was concluded that, unless extremely high plasma fluoride levels are involved (pharmacologic or toxic doses), the absorption of the ion is independent of plasma levels. The results provide further evidence that plasma fluoride concentrations are not homeostatically regulated.

  17. Immunolocalisation of fibrillin microfibrils in the calf metacarpal and vertebral growth plate.

    PubMed

    Yu, Jing; Urban, Jill

    2013-12-01

    Overgrowth of limbs and spinal deformities are typical clinical manifestations of Marfan syndrome (MFS) and congenital contractural arachnodactyly (CCA), caused by mutations of the genes encoding fibrillin-1 (FBN1) and fibrillin-2 (FBN2), respectively. FBN1 mutations are also associated with acromicric (AD) and geleophysic dysplasias (GD), and with Weill-Marchesani syndrome (WMS), which is characterised by short stature. The mechanisms leading to such abnormal skeletal growth and the involvement of the fibrillins are not understood. Postnatal longitudinal bone growth mainly occurs in the epiphyseal growth plate. Here we investigated the organisation of fibrillin microfibrils in the growth plate of the long bone and vertebra immunohistochemically. Fibrillin-1 was dual-immunostained with elastin, with fibrillin-2 or with collagen X. We report that fibrillin microfibrils are distributed throughout all regions of the growth plate, and that fibrillin-1 and fibrillin-2 were differentially organised. Fibrillin-1 was more abundant in the extracellular matrix of the resting and proliferative zones of the growth plate than in the hypertrophic zone. More fibrillin-2 was found in the calcified region than in the other regions. No elastin fibres were observed in either the proliferative or hypertrophic zones. This study indicates that, as fibrillin microfibrils are involved in growth factor binding and may play a mechanical role, they could be directly involved in regulating bone growth. Hence, mutations of the fibrillins could affect their functional role in growth and lead to the growth disorders seen in patients with MFS, CCA, AD, GD and WMS.

  18. Functional bone histology of zebrafish reveals two types of endochondral ossification, different types of osteoblast clusters and a new bone type.

    PubMed

    Weigele, Jochen; Franz-Odendaal, Tamara A

    2016-07-01

    The zebrafish is as an important vertebrate animal model system for studying developmental processes, gene functions and signalling pathways. It is also used as a model system for the understanding of human developmental diseases including those related to the skeleton. However, surprisingly little is known about normal zebrafish skeletogenesis and osteogenesis. As in most vertebrates, it is commonly known that the bones of adult zebrafish are cellular unlike that of some other teleosts. After careful histological analyses of each zebrafish adult bone, we identified several acellular bones, with no entrapped osteocytes in addition to several cellular bones. We show that both cellular and acellular bones can even occur within the same skeletal element and transitions between these two cell types can be found. Furthermore, we describe two types of osteoblast clusters during skeletogenesis and two different types of endochondral ossification. The epiphyseal plate, for example, lacks a zone of calcification and a degradation zone with osteoblasts. A new bone type that we term tubular bone was also identified. This bone is completely filled with adipose tissue, unlike spongy bones. This study provides important insight on how osteogenesis takes place in zebrafish, and especially on the transition from cellular to acellular bones. Overall, this study leads to a deeper understanding of the functional histological composition of adult zebrafish bones.

  19. Ossification of the mouse metatarsal: differentiation and proliferation in the presence/absence of a defined growth plate.

    PubMed

    Reno, Philip L; McBurney, Denise L; Lovejoy, C Owen; Horton, Walter E

    2006-01-01

    There is significant diversity in growth plate behavior among sites within an individual skeleton and between skeletons of different species. This variation within wild-type animals is an underutilized resource for studying skeletal development. One bone that potentially exhibits the most diverse behavior is the metatarsal. While one end forms a growth plate with an epiphyseal secondary center of ossification as in other long bones, the opposite end undergoes direct ossification in a manner more similar to short bones. Although descriptions of human metatarsal/metacarpal ossification are available, a detailed comparative analysis has yet to be conducted in an animal model amenable to biomolecular analysis. Here we report an analysis of proximal and distal ossification in an age series of mouse metatarsals. Safranin O staining was used for qualitative and quantitative histology, and chondrocyte differentiation and proliferation were analyzed using immunohistochemistry for type X collagen and proliferative cell nuclear antigen expression. We establish that, as in the human, both growth plate formation and direct ossification occur in the mouse metatarsal, with chondrocyte populations showing distinct differentiation patterns at opposite ends of the bone. In addition, growth plate formation is characterized by a peak of proliferation in reserve zone chondrocytes that distinguishes it from both established growth plates and direct ossification. Our analysis demonstrates that the mouse metatarsal is a productive model for investigating natural variation in ossification that can further understanding of vertebrate skeletal development and evolution.

  20. Femoral development in chronically centrifuged rats

    NASA Technical Reports Server (NTRS)

    Smith, S. D.

    1977-01-01

    Groups of 30-d-old male and female rats were centrifuged at 2.00 G (RE, Rotation Experimental), 1.05 G (RC, Rotation Control) or exposed to the noise and wind of the centrifuge at 1.00 G (EC, Earth Control) for periods of 1, 2, 4, 8, and 16 weeks. Measurements of their femurs indicated that exposure to centrifugation a) decreased femoral length in RE animals, b) increased femoral length in RC animals, c) reduced femoral diameter in RE and RC animals, d) increased L/D ratios in RC animals, e) decreased L/D ratios in RE animals, f) increased femur length/body weight in RE animals, g) decreased cortical thickness (CT) in RE animals, h) increased relative CT in RE animals, and decreased it in RC animals, i) accelerated ossification in RC femoral heads, j) thinned and distorted RE epiphyseal plates, and k) thickened condylar cartilage in RE females. The effects tended to be strongly sexually dimorphic, with females more severely affected by the stress than males.

  1. Cyp26b1 within the growth plate regulates bone growth in juvenile mice.

    PubMed

    Minegishi, Yoshiki; Sakai, Yasuo; Yahara, Yasuhito; Akiyama, Haruhiko; Yoshikawa, Hideki; Hosokawa, Ko; Tsumaki, Noriyuki

    2014-11-07

    Retinoic acid (RA) is an active metabolite of vitamin A and plays important roles in embryonic development. CYP26 enzymes degrade RA and have specific expression patterns that produce a RA gradient, which regulates the patterning of various structures in the embryo. However, it has not been addressed whether a RA gradient also exists and functions in organs after birth. We found localized RA activities in the diaphyseal portion of the growth plate cartilage were associated with the specific expression of Cyp26b1 in the epiphyseal portion in juvenile mice. To disturb the distribution of RA, we generated mice lacking Cyp26b1 specifically in chondrocytes (Cyp26b1(Δchon) cKO). These mice showed reduced skeletal growth in the juvenile stage. Additionally, their growth plate cartilage showed decreased proliferation rates of proliferative chondrocytes, which was associated with a reduced height in the zone of proliferative chondrocytes, and closed focally by four weeks of age, while wild-type mouse growth plates never closed. Feeding the Cyp26b1 cKO mice a vitamin A-deficient diet partially reversed these abnormalities of the growth plate cartilage. These results collectively suggest that Cyp26b1 in the growth plate regulates the proliferation rates of chondrocytes and is responsible for the normal function of the growth plate and growing bones in juvenile mice, probably by limiting the RA distribution in the growth plate proliferating zone.

  2. Tetracycline labeling as an aid to complete excision of partial physeal arrest: a rabbit model.

    PubMed

    Post, W R; Jones, E T

    1992-01-01

    We determined whether ultraviolet (UV) visualization of tetracycline-labeled bone allows more complete excision of experimentally created physeal arrests while minimizing required bone resection. Lateral distal femoral growth arrests were surgically created bilaterally in eight 6-week-old New Zealand white rabbits, given 3 daily oxytetracycline doses (10 mg/kg); the animals were killed 6 weeks postoperatively. Femurs were excised and dissected free of soft tissue. Ten of 16 operated femurs randomized into two groups had consistent deformity. In group I, arrest was excised with a high-speed burr without, and, in group II, with ultraviolet visualization. In group II, metaphyseal bone and calcified arrest glowed brightly, contrasting well with the epiphyseal plate. All excised specimens were examined under x 9 magnification to assess remaining bone bridges. Excision defects were filled with modeling clay, which was then removed and weighed. Thorough excision was confirmed in each specimen. Resection was easier and less bone usually was resected in the UV group (difference not statistically significant). This technique would best be used in resection of arrests involving small physes such as the distal radius, in which maximal visualization and minimal resection are essential to preserve all possible growth potential. Tetracycline labeling and intraoperative UV visualization of fluorescence allows thorough excision of partial physeal arrest while minimizing required bone resection.

  3. Imaging of growth disturbance in children.

    PubMed

    Ecklund, K; Jaramillo, D

    2001-07-01

    Growth disturbance of the long bones in children is frequently post-traumatic but also occurs because of physeal, epiphyseal, or metaphyseal ischemia. The imaging features of growth arrest depend more on the anatomic site involved than on the cause. The physes of the distal tibia and femur and proximal tibia are disproportionately at risk because of their complex geometry. The central undulation in the distal femur and the bump in the anteromedial physis (Kump's bump) in the distal tibia are the sites of initial physiologic closure and the most frequent areas of premature fusion. The MR imaging features of growth disturbance are characteristic. T1-weighted images show low signal intensity GRL and variable signal intensity bony bridges. On GRE sequences, a bridge appears as low signal intensity interruption in the otherwise high signal intensity physeal cartilage. Physeal widening on GRE and T2-weighted images implies physeal dysfunction without bridge formation. Proton density and T2-weighted images best reveal associated metaphyseal and soft tissue changes. Regardless of the cause, MR imaging exquisitely depicts cartilaginous pathology at the physis. MR evaluation should be considered in patients at high risk for growth disturbance including young children with extensive residual growth potential; those with involvement of particularly vulnerable growth plates; and those with severe, complex fractures.

  4. Posttraumatic distal ulnar physeal arrest: a case report and review of the literature.

    PubMed

    Chimenti, Peter; Hammert, Warren

    2013-03-01

    We report the case of a 12-year-old male who sustained a Salter-Harris (SH) type IV physeal fracture of the distal ulna and a SH type II fracture of the distal radius. At 34 months later, he presented with activity-related wrist pain and ulnar variance of -17 mm. He successfully underwent ulnar distraction osteogenesis with radial closing wedge osteotomy. At 16-month follow-up, the patient denied wrist pain with activity, and imaging demonstrated ulnar variance of -3 mm. Epiphyseal fracture separations of the distal radius and ulna have the potential to cause early growth arrest and may become symptomatic as a result. High-energy mechanism, open fracture, number of reduction attempts, and age at injury can all increase the risk of premature closure. Therefore, we recommend longitudinal follow-up of patients with these injuries as earlier intervention may improve outcomes. When premature physeal closure is discovered early, treatment may include resection of the physeal bar, osteotomy with or without epiphysiodesis, and distraction osteogenesis.

  5. Chondrocytes are released as viable cells during cartilage resorption associated with the formation of intrachondral canals in the rat tibial epiphysis.

    PubMed

    Alvarez, Jesús; Costales, Lorena; López-Muñiz, Alfonso; López, José M

    2005-06-01

    The development of cartilage canals is the first event of the ossification of the epiphyses in mammals. Canal formation differs from vascular invasion during primary ossification, since the former involves resorption of resting cartilage and is uncoupled from bone deposition. To learn more about the fate of resorbed chondrocytes during this process, we have carried out structural, cell proliferation, and in situ hybridization studies during the first stages of ossification of the rat tibial proximal epiphysis. Results concerning the formation of the cartilage canals implied the release of resting chondrocytes from the cartilage matrix to the canal cavity. Released chondrocytes had a well-preserved structure, expressed type-II collagen, and maintained the capacity to divide. All these data suggested that chondrocytes released into the canals remained viable for a specific time. Analysis of the proliferative activity at different regions of the cartilage canals showed that the percentage of proliferative chondrocytes at areas of active cartilage resorption was significantly higher than that in zones of low resorption. These results are consistent with the hypothesis that resting chondrocytes surrounding canals have a role in supplying cells for the development of the secondary ossification center. Since released chondrocytes are at an early stage of differentiation greatly preceding their entry into the apoptotic pathway and are exposed to a specific matrix, cellular, and humoral microenvironment, they might differentiate to other cell types and contribute to the ossification of the epiphysis.

  6. The effects of burial on drug detection in skeletal tissues.

    PubMed

    Desrosiers, Nathalie A; Watterson, James H

    2010-07-01

    Skeletal tissues have recently been investigated for use in post-mortem toxicology. Variables affecting drug concentration in these tissues, however, are still poorly characterized. In this work, the relative effects of burial on the response of enzyme-linked immunosorbent assay (ELISA) and gas chromatography-mass spectrometry (GC-MS) assays were examined. Rats were acutely exposed to ketamine or diazepam, euthanized and buried outdoors. After one month, the remains were exhumed and skeletal tissue drug levels were compared those of non-buried rats. A climate-controlled burial was also undertaken using defleshed bones to approximate an extended decomposition. Long bones (femora, tibiae) were isolated and separated into tissue type (diaphyseal bone, epiphyseal bone, and marrow), and according to treatment (i.e. buried or non-buried). Following methanolic extraction (bone) or simple homogenization (marrow), samples were analyzed with ELISA. Samples were then pooled according to treatment, extracted by solid phase extraction (SPE) and confirmed with GC-MS. Under the conditions examined, the effects of burial appear to be drug and tissue dependent. Ketamine-exposed tissues demonstrated the greatest differences, especially in bone marrow. In diazepam-exposed tissues, burial did not seem to greatly affect drug response and some gave greater assay response compared to the non-buried set. Overall, the data suggest that fresh tissue samples may not be representative of decomposed samples in terms of skeletal tissue drug levels.

  7. [Basophilic line of the articular cartilage in normal and various pathological states].

    PubMed

    Gongadze, L R

    1987-04-01

    Epiphyses of long tubular bones in the man and animals of various age, as well as experimental material of the adjuvant arthritis, with special reference to the basal part of the articular cartilage have been studied by means of histological, histochemical and histometrical methods. The structural-chemical organization of the basophilic line (tidemark) of the articular cartilage ensures its barrier role and participation in regulating selective permeability. Reconstruction of the tidemark in the process of physiological ageing and in cases of the articular pathology is aimed to preserve its integrity and in this way a complete differentiation of the noncalcified and calcified structures is secured. Disturbance of the basophilic line results in changes of the articular selective permeability, in invasion of vessels and structural elements of the bone marrow, and in development of profound distrophic and destructive changes of the cartilage--in deforming artrosis. Deflations in the structural-chemical organization of the tidemark indicate certain disturbances in the state of the system articular cartilage--subchondral bone. These data can be of prognostic importance.

  8. Mycoplasma corogypsi-associated polyarthritis and tenosynovitis in black vultures (Coragyps atratus).

    PubMed

    Van Wettere, A J; Ley, D H; Scott, D E; Buckanoff, H D; Degernes, L A

    2013-03-01

    Three wild American black vultures (Coragyps atratus) were presented to rehabilitation centers with swelling of multiple joints, including elbows, stifles, hocks, and carpal joints, and of the gastrocnemius tendons. Cytological examination of the joint fluid exudate indicated heterophilic arthritis. Radiographic examination in 2 vultures demonstrated periarticular soft tissue swelling in both birds and irregular articular surfaces with subchondral bone erosion in both elbows in 1 bird. Prolonged antibiotic therapy administered in 2 birds did not improve the clinical signs. Necropsy and histological examination demonstrated a chronic lymphoplasmacytic arthritis involving multiple joints and gastrocnemius tenosynovitis. Articular lesions varied in severity and ranged from moderate synovitis and cartilage erosion and fibrillation to severe synovitis, diffuse cartilage ulceration, subchondral bone loss and/or sclerosis, pannus, synovial cysts, and epiphyseal osteomyelitis. No walled bacteria were observed or isolated from the joints. However, mycoplasmas polymerase chain reactions were positive in at least 1 affected joint from each bird. Mycoplasmas were isolated from joints of 1 vulture that did not receive antibiotic therapy. Sequencing of 16S rRNA gene amplicons from joint samples and the mycoplasma isolate identified Mycoplasma corogypsi in 2 vultures and was suggestive in the third vulture. Mycoplasma corogypsi identification was confirmed by sequencing the 16S-23S intergenic spacer region of mycoplasma isolates. This report provides further evidence that M. corogypsi is a likely cause of arthritis and tenosynovitis in American black vultures. Cases of arthritis and tenosynovitis in New World vultures should be investigated for presence of Mycoplasma spp, especially M. corogypsi.

  9. A Pex7 hypomorphic mouse model for plasmalogen deficiency affecting the lens and skeleton

    PubMed Central

    Braverman, Nancy; Zhang, Rui; Chen, Li; Nimmo, Graeme; Scheper, Sarah; Tran, Tammy; Chaudhury, Rupsa; Moser, Ann; Steinberg, Steven

    2010-01-01

    Rhizomelic chondrodysplasia punctata type 1 is a peroxisome biogenesis disorder with the clinical features of rhizomelia, abnormal epiphyseal calcifications, congenital cataracts, and profound growth and developmental delays. It is a rare autosomal recessive disorder, caused by defects in the peroxisome receptor, Pex7. The pathology results from a deficiency of plasmalogens, a critical class of ether phospholipids whose functions are largely unknown. To study plasmalogens in an animal model, avoid early mortality and facilitate therapeutic investigations in this disease, we engineered a hypomorphic mouse model in which Pex7 transcript levels are reduced to less than 5% of wild type. These mice are born in expected ratios, are fertile and have a normal life span. However, they are petite and develop early cataracts. Further investigations showed delayed endochondral ossification and abnormalities in lens fibers. The biochemical features of reduced Pex7 function were reproduced in this model, including tissue plasmalogen deficiency, phytanic acid accumulation, reduced import of Pex7 ligands and consequent defects in plasmalogen biosynthesis and phytanic acid oxidation. Dietary supplementation with batyl alcohol, a plasmalogen precursor, recovered ether phospholipids in blood, but did not alter the clinical phenotype. The relatively mild phenotype of these mice mimics patients with milder pex7 defects, and highlights the skeleton and lens as sensitive markers of plasmalogen deficiency. The role of plasmalogens in the normal function of these tissues at various ages can now be studied and additional therapeutic interventions tested in this model. PMID:20060764

  10. Co-segregation of Freiberg's infraction with a familial translocation t(5;7)(p13.3;p22.2) ascertained by a child with cri du chat syndrome and brachydactyly type A1B.

    PubMed

    Myśliwiec, Marta; Panasiuk, Barbara; Dębiec-Rychter, Maria; Iwanowski, Piotr Sebastian; Łebkowska, Urszula; Nowakowska, Beata; Marcinkowska, Anna; Stankiewicz, Pawel; Midro, Alina T

    2015-02-01

    The identification of chromosomal breakpoints in association with human abnormal phenotypes can enable elucidation of gene function. We report on epiphyseal aseptic necrosis of the lesser head of the second metatarsal bone, known as Freiberg's infraction (FI), in two female carriers of the apparently balanced t(5;7)(p13.3;p22.2) ascertained by a 16-year-old girl with cri-du-chat syndrome and unusual skeletal features in association with an unbalanced translocation der(5) t(5;7)(p13.3;p22.2). Mapping of the chromosome breakpoints using fluorescent in situ hybridization (FISH) narrowed them to the coding sequence of ADAMTS12 on chromosome 5p13.3 and SDK1 on 7p22.2. In addition, several skeletal abnormalities classified as brachydactyly type A1B (BDA1B) were present in the proband and in both carriers of t(5;7)(p13.3;p22.2), suggesting a potential role of ADAMTS12 in the development of the BDA1B observed in this family.

  11. Skeletal development and performance of broilers

    SciTech Connect

    El-Deeb, M.A.

    1989-01-01

    The effect of high dietary copper supplementation has been shown to promote growth rate. The results of the experiments reported herein indicated that copper supplementation of 100 ppm with zinc at the requirement level had no effect on growth rate or bone mineralization. However, when various levels of copper and zinc were added to the diet, significant changes in bone mineralization and liver zinc and copper concentrations occurred. In further studies, the effect of dietary supplementation with vitamin D{sub 3} or its metabolite 1,25(OH){sub 2}D{sub 3} on the incidence of tibial dyschondroplasia and related bone abnormalities in broiler chicks was investigated. The results indicated that feeding 1,25(OH){sub 2}D{sub 3} at 5ug/Kg diet as the sole source of vitamin D{sub 3} steroid was sufficient to maintain a calcium homeostasis in the chicks. The effect of growth hormone (GH) injections on the metabolism of tibial epiphyseal growth plant (EGP) in broiler chicks either genetically susceptible or resistant to tibial dyschondroplasia (HTD and LTD respectively) was investigated. GH injection increased {sup 3}H-thymidine uptake, indicating a direct effect of GH on cell replication in EGP.

  12. Leptin increases growth of primary ossification centers in fetal mice

    PubMed Central

    Bertoni, Laura; Ferretti, Marzia; Cavani, Francesco; Zavatti, Manuela; Resca, Elisa; Benelli, Augusta; Palumbo, Carla

    2009-01-01

    The effect of peripheral leptin on fetal primary ossification centers during the early phases of bone histogenesis was investigated by administration of leptin to pregnant mice. Fourteen pregnant mice were divided into two groups. The treated pregnant group was subcutaneously injected in the intrascapular region with supraphysiologic doses (2 mg kg−1) of leptin (Vinci Biochem, Firenze, Italy) in a volume of 0.1 mL per 10 g body weight, at the 7th, 9th and 11th day of gestation. The control group was treated with physiological solution in the same manner and same times as the treated group. The new-born mice were killed 1 day after birth and the primary ossification centers were stained with Alizarin Red S after diaphanizing the soft tissues in 1% potassium hydroxide. The development of both endochondral and intramembranous ossification centers was morphometrically analysed in long bones. The results showed that the ossification centers of mice born by mothers treated with leptin grow more rapidly in both length and cross-sectional area compared with mice born by the untreated mothers. As the development of long bones depends on endochondral ossification occurring at proximal and distal epiphyseal plates as well as on intramembranous ossification along the periosteal surface, it appears that leptin activates the differentiation and proliferation of both chondrocytes and osteoblasts. The role of leptin as a growth factor of cartilage and bone is discussed in the light of the data reported in the literature. PMID:19682137

  13. Histochemical evidences on the chronological alterations of the hypertrophic zone of mandibular condylar cartilage.

    PubMed

    Hossain, Kazi Sazzad; Amizuka, Norio; Ikeda, Nobuyki; Nozawa-Inoue, Kayoko; Suzuki, Akiko; Li, Minqi; Takeuchi, Kiichi; Aita, Megumi; Kawano, Yoshiro; Hoshino, Masaaki; Oda, Kimimitsu; Takagi, Ritsuo; Maeda, Takeyasu

    2005-08-15

    The hypertrophic chondrocytes lack the ability to proliferate, thus permitting matrix mineralization as well as vascular invasion from the bone in both the mandibular condyle and the epiphyseal cartilage. This study attempted to verify whether the histological appearance of the hypertrophic chondrocytes is in a steady state during postnatal development of the mouse mandibular condyle. Type X collagen immunohistochemistry apparently distinguished the fibrous layer described previously as the "articular zone," "articular layer," and "resting zone" from the hypertrophic zone. Interestingly, the ratio of the type X collagen-positive hypertrophic zone in the entire condyle seemed higher in the early stages but decreased in the later stages. Some apparently compacted cells in the hypertrophic zone showed proliferating cell nuclear antigen (PCNA) immunoreaction, indicating the potential for cell proliferation at the early stages. As the mice matured, in contrast, they further enlarged and assumed typical features of hypertrophic chondrocytes. Apoptotic cells were also discernible in the hypertrophic zone at the early but not later stages. Consistent with morphological configurations of hypertrophic chondrocytes, immunoreactions for alkaline phosphatase, osteopontin, and type I collagen were prominent at the later stage, but not the early stage. Cartilaginous matrices demonstrated scattered patches of mineralization at the early stage, but increased in their volume and connectivity at the later stage. Thus, the spatial and temporal occurrence of these immunoreactions as well as apoptosis likely reflect the prematurity of hypertrophying cells at the early stage, and imply a physiological relevance during the early development of the mandibular condyles.

  14. Inhibitory effects of a polypeptide thymic factor on the development of 7,12-dimethylbenz(a)anthragene-induced mammary adenocarcinoma in female rats

    SciTech Connect

    Anisimov, V.N.; Danetskaya, E.V.; Morozov, V.G.; Khavinson, V.Kh.

    1980-01-01

    It has come to be recognized that tumor growth is accompanied by inhibition of cellular immunity and the function of the T lymphocytes. Restitution of T lymphocyte function by means of several pharmacologic agents such as levamisole, phenformin, or epithalamin (an epiphyseal factor) has, in a number of cases, been accompanied by growth inhibition of both spontaneous and induced tumors. In addition, the importance of the thymus in the regulation of T lymphocytes and in antitumor immunity has been recognized. Several indicators point to the fact that the thymus contains physiologically active substances which stimulate T cell-dependent immunity and prevent the occurrence of neoplasms. These considerations have led to attempts at isolation of active thymic factors and studies on their effects on the appearance and growth of tumors. Previously, a thymic factor - thymarin - had been isolated which imparted immunocompetence to the T lymphocytes. This factor differs from other thymic preparations, including thymosine, in terms of a number of physicochemical characteristics and is a polypeptide with a molecular weight of 5000. This study is concerned with its effects on tumor development - mammary gland adenocarcinoma induced in animals with a chemical carcinogen.

  15. Clinical and molecular characterization of Diastrophic Dysplasia in the Portuguese population.

    PubMed

    Barbosa, M; Sousa, A B; Medeira, A; Lourenço, T; Saraiva, J; Pinto-Basto, J; Soares, G; Fortuna, A M; Superti-Furga, A; Mittaz, L; Reis-Lima, M; Bonafé, L

    2011-12-01

    SLC26A2-related dysplasias encompass a spectrum of diseases: from lethal achondrogenesis type 1B (ACG1B; MIM #600972) and atelosteogenesis type 2 (AO2; MIM #256050) to classical diastrophic dysplasia (cDTD; MIM #222600) and recessive multiple epiphyseal dysplasia (rMED; MIM #226900). This study aimed at characterizing clinically, radiologically and molecularly 14 patients affected by non-lethal SLC26A2-related dysplasias and at evaluating genotype-phenotype correlation. Phenotypically, eight patients were classified as cDTD, four patients as rMED and two patients had an intermediate phenotype (mild DTD - mDTD, previously 'DTD variant'). The Arg279Trp mutation was present in all patients, either in homozygosity (resulting in rMED) or in compound heterozygosity with the known severe alleles Arg178Ter or Asn425Asp (resulting in DTD) or with the mutation c.727-1G>C (causing mDTD). The 'Finnish mutation', c.-26+2T>C, and the p.Cys653Ser, both frequent mutations in non-Portuguese populations, were not identified in any of the patients of our cohort and are probably very rare in the Portuguese population. A targeted mutation analysis for p.Arg279Trp and p.Arg178Ter in the Portuguese population allows the identification of approximately 90% of the pathogenic alleles.

  16. A technique for developing CAD geometry of long bones using clinical CT data.

    PubMed

    Davis, Matthew L; Vavalle, Nicholas A; Stitzel, Joel D; Gayzik, F Scott

    2015-11-01

    Computed tomography scans are a valuable tool for developing computational models of bones. The objective of this study is to present a method to generate CAD representations of long bones from clinically based CT scans. A secondary aim is to apply the method to six long bones from a sample of three individuals. Periosteal and endosteal bone surfaces were segmented and used to calculate the characteristic cortical thickness, Tc, at 1 mm increments along the bone axis. In the epiphyses where the value of Tc fell below the scanner threshold, the endosteal bone layer was replaced using literature values projected inward from the periosteal surface. On average, 74.7 ± 7.4% of the bone geometry was above the scanner cut-off and was therefore derived from the CT scan data. The thickness measurement was also compared to experimental measurements of cadaveric bone and was found to predict Tc with an error of 3.1%. This method presents a possible solution for the characterization of characteristic thickness along the length of the bone and may also aid in the development of orthopedic implant design and subject specific finite element models.

  17. The humerus of Eusthenopteron: a puzzling organization presaging the establishment of tetrapod limb bone marrow

    PubMed Central

    Sanchez, S.; Tafforeau, P.; Ahlberg, P. E.

    2014-01-01

    Because of its close relationship to tetrapods, Eusthenopteron is an important taxon for understanding the establishment of the tetrapod body plan. Notably, it is one of the earliest sarcopterygians in which the humerus of the pectoral fin skeleton is preserved. The microanatomical and histological organization of this humerus provides important data for understanding the evolutionary steps that built up the distinctive architecture of tetrapod limb bones. Previous histological studies showed that Eusthenopteron's long-bone organization was established through typical tetrapod ossification modalities. Based on a three-dimensional reconstruction of the inner microstructure of Eusthenopteron's humerus, obtained from propagation phase-contrast X-ray synchrotron microtomography, we are now able to show that, despite ossification mechanisms and growth patterns similar to those of tetrapods, it also retains plesiomorphic characters such as a large medullary cavity, partly resulting from the perichondral ossification around a large cartilaginous bud as in actinopterygians. It also exhibits a distinctive tubular organization of bone-marrow processes. The connection between these processes and epiphyseal structures highlights their close functional relationship, suggesting that either bone marrow played a crucial role in the long-bone elongation processes or that trabecular bone resulting from the erosion of hypertrophied cartilage created a microenvironment for haematopoietic stem cell niches. PMID:24648231

  18. Regulation of Articular Chondrocyte Proliferation and Differentiation by Indian Hedgehog and Parathyroid Hormone-related Protein

    PubMed Central

    Chen, Xuesong; Macica, Carolyn; Nasiri, Ali; Broadus, Arthur E.

    2008-01-01

    Objective The chondrocytes of the epiphyseal growth zone are regulated by the Indian hedgehog (Ihh)-parathyroid hormone-related protein (PTHrP) axis. In weight-bearing joints, this growth zone comes to be subdivided by the secondary ossification center into distinct articular and growth cartilage structures. Here, we explored the cells of origin, localization, regulation of expression, and putative functions of Ihh and PTHrP in articular cartilage in the mouse. Methods We assessed Ihh and PTHrP expression in an allelic PTHrP-lacZ knockin mouse and several versions of PTHrP-null mice. Selected joints were unloaded surgically to examine load-induction of PTHrP and Ihh. Results The embryonic growth zone appears to serve as the source of PTHrP-expressing proliferative chondrocytes that populate both the forming articular cartilage and growth plate structures. In articular cartilage, these cells take the form of articular chondrocytes in the mid-zone. In PTHrP-knockout mice, mineralizing chondrocytes encroach upon developing articular cartilage but appear to be prevented from mineralizing the joint space by Ihh-driven surface chondrocyte proliferation. In growing and adult mice, PTHrP expression in articular chondrocytes is load-induced, and unloading is associated with rapid changes in PTHrP expression and articular chondrocyte differentiation. Conclusion We conclude that the PTHrP-Ihh axis participates in the maintenance of articular cartilage. Dysregulation of this system might contribute to the pathogenesis of arthritis. PMID:19035497

  19. Intercondylar fracture of the distal humerus in a 7-year-old child

    PubMed Central

    Tomori, Yuji; Sudo, Yoshihiro; Iizawa, Norishige; Nanno, Mitsuhiko; Takai, Shinro

    2017-01-01

    Abstract Rationale: Intercondylar fracture of the distal humerus is an extremely rare injury in children, especially in those under 8 years of age. To our best knowledge, there have been 55 reported cases of this fracture type in children in the English literature, 12 of which involved children under 8 years of age. Patient Concerns: We report a case of intercondylar fracture of the distal humerus in a7-year-old boy fell in a gymnasium, injuring his left elbow. Interventions: Closed reduction was initially attempted under fluoroscopic guidance, but anatomic reduction could not be achieved because the fragments were extremely unstable and irreducible. Considering the displacement and the failure of closed reduction, ORIF through a posterior approach was performed. Open reduction and double cross-pinning across the medial and lateral condylar fragments were performed through a posterior approach. Diagnoses: Plain radiographs showed a displaced intercondylar fracture of the distal humerus. Arthrography under general anesthesia showed a severely displaced intra-articular fracture, with rotational displacement of the lateral condyle. Outcomes: Thirteen months after surgery, there was no functional disturbance or radiographic evidence of avascular necrosis or epiphyseal growth arrest. Lessons: Open reduction and double cross-pinning through a posterior approach can be a reliable procedure for intercondylar fracture of the distal humerus in children. PMID:28178167

  20. Intrapopulational body size variation and cranial capacity variation in Middle Pleistocene humans: the Sima de los Huesos sample (Sierra de Atapuerca, Spain).

    PubMed

    Lorenzo, C; Carretero, J M; Arsuaga, J L; Gracia, A; Martínez, I

    1998-05-01

    A sexual dimorphism more marked than in living humans has been claimed for European Middle Pleistocene humans, Neandertals and prehistoric modern humans. In this paper, body size and cranial capacity variation are studied in the Sima de los Huesos Middle Pleistocene sample. This is the largest sample of non-modern humans found to date from one single site, and with all skeletal elements represented. Since the techniques available to estimate the degree of sexual dimorphism in small palaeontological samples are all unsatisfactory, we have used the bootstraping method to asses the magnitude of the variation in the Sima de los Huesos sample compared to modern human intrapopulational variation. We analyze size variation without attempting to sex the specimens a priori. Anatomical regions investigated are scapular glenoid fossa; acetabulum; humeral proximal and distal epiphyses; ulnar proximal epiphysis; radial neck; proximal femur; humeral, femoral, ulnar and tibial shaft; lumbosacral joint; patella; calcaneum; and talar trochlea. In the Sima de los Huesos sample only the humeral midshaft perimeter shows an unusual high variation (only when it is expressed by the maximum ratio, not by the coefficient of variation). In spite of that the cranial capacity range at Sima de los Huesos almost spans the rest of the European and African Middle Pleistocene range. The maximum ratio is in the central part of the distribution of modern human samples. Thus, the hypothesis of a greater sexual dimorphism in Middle Pleistocene populations than in modern populations is not supported by either cranial or postcranial evidence from Sima de los Huesos.

  1. [Pathology of the knee in the hemophiliac patient].

    PubMed

    Rodríguez-Merchán, E C

    1994-06-01

    Chronic haemophilic arthropathy of the knee is a debilitating condition that also carries the risk of rapid degeneration of the articular cartilage of the affected joint. The swelling is a combination of haemarthroses, irritative synovial effusions, synovial thickening and actual epiphyseal overgrowth that is the result of chronic hyperemia. In the knee joint the enlarged appearance is accentuated by quadriceps wasting. Replacement of deficient factor remains the cornerstone of management, followed by splinting in the position of function, synoviorthesis and surgical synovectomy (open or arthroscopic). End stage arthropathy of the knee is the most frequent cause of severe pain and disability in haemophiliacs. Our surgical experience may be divided into five categories: synovectomy, ostectomy, hamstring tenotomy, debridement and prosthetic arthroplasty. Over the last 20 years advanced arthropathy of the knee has been approached with a broad spectrum of procedures with varying success and durability. Early treatment and prevention of chronic synovitis and progressive arthropathy is the key. Where end stage arthropathy does occur and is severely disabling, durable, functional reconstruction with minimal risk must be our goal.

  2. Regeneration of limb joints in the axolotl (Ambystoma mexicanum).

    PubMed

    Lee, Jangwoo; Gardiner, David M

    2012-01-01

    In spite of numerous investigations of regenerating salamander limbs, little attention has been paid to the details of how joints are reformed. An understanding of the process and mechanisms of joint regeneration in this model system for tetrapod limb regeneration would provide insights into developing novel therapies for inducing joint regeneration in humans. To this end, we have used the axolotl (Mexican Salamander) model of limb regeneration to describe the morphology and the expression patterns of marker genes during joint regeneration in response to limb amputation. These data are consistent with the hypothesis that the mechanisms of joint formation whether it be development or regeneration are conserved. We also have determined that defects in the epiphyseal region of both forelimbs and hind limbs in the axolotl are regenerated only when the defect is small. As is the case with defects in the diaphysis, there is a critical size above which the endogenous regenerative response is not sufficient to regenerate the joint. This non-regenerative response in an animal that has the ability to regenerate perfectly provides the opportunity to screen for the signaling pathways to induce regeneration of articular cartilage and joints.

  3. Comparison of fetal cartilage-derived progenitor cells isolated at different developmental stages in a rat model.

    PubMed

    Kim, Mijin; Kim, Jiyoung; Park, So Ra; Park, Do Young; Kim, Young Jick; Choi, Byung Hyune; Min, Byoung-Hyun

    2016-02-01

    Fetal cartilage-derived progenitor cells (FCPCs) could be a useful cell source in cell-based therapies for cartilage disorders. However, their characteristics can vary depending on the developmental stages. The aim of this study was to compare the characteristics of rat FCPCs from the hind limb on embryonic day 14 (E14), E16 and E20 regarding proliferation, pluripotency, and differentiation. Morphologically, rat fetal cartilage tissue showed an increase in cartilaginous differentiation features (Safranin-O, type II collagen) and decrease in pluripotency marker (Sox2) in the order of E14, E16 and E20. E14 FCPCs showed significantly higher doubling time compared to E16 and E20 FCPCs. While the E14 FCPCs expressed pluripotent genes (Sox2, Oct4, Nanog), the E16 and E20 FCPCs expressed chondrogenic markers (Sox9, Col2a1, Acan). E20 FCPCs showed the highest ability to both chondrogenic and adipogenic differentiation and E14 FCPCs showed relatively better activity in osteogenic differentiation. Further analysis showed that E20 FCPCs expressed both adipogenic (C/ebpß) and osteogenic (Runx2, Sp7, Taz) transcription factors as well as chondrogenic transcription factors. Our results show an inverse relationship overall between the expression of pluripotency genes and that of chondrogenic and lineage-specific genes in FCPCs under development. Due to its exceptional proliferation and chondrogenic differentiation ability, fetal cells from epiphyseal cartilage (E20 in rats) may be a suitable cell source for cartilage regeneration.

  4. Potential of human fetal chorionic stem cells for the treatment of osteogenesis imperfecta.

    PubMed

    Jones, Gemma N; Moschidou, Dafni; Abdulrazzak, Hassan; Kalirai, Bhalraj Singh; Vanleene, Maximilien; Osatis, Suchaya; Shefelbine, Sandra J; Horwood, Nicole J; Marenzana, Massimo; De Coppi, Paolo; Bassett, J H Duncan; Williams, Graham R; Fisk, Nicholas M; Guillot, Pascale V

    2014-02-01

    Osteogenesis imperfecta (OI) is a genetic bone pathology with prenatal onset, characterized by brittle bones in response to abnormal collagen composition. There is presently no cure for OI. We previously showed that human first trimester fetal blood mesenchymal stem cells (MSCs) transplanted into a murine OI model (oim mice) improved the phenotype. However, the clinical use of fetal MSC is constrained by their limited number and low availability. In contrast, human fetal early chorionic stem cells (e-CSC) can be used without ethical restrictions and isolated in high numbers from the placenta during ongoing pregnancy. Here, we show that intraperitoneal injection of e-CSC in oim neonates reduced fractures, increased bone ductility and bone volume (BV), increased the numbers of hypertrophic chondrocytes, and upregulated endogenous genes involved in endochondral and intramembranous ossification. Exogenous cells preferentially homed to long bone epiphyses, expressed osteoblast genes, and produced collagen COL1A2. Together, our data suggest that exogenous cells decrease bone brittleness and BV by directly differentiating to osteoblasts and indirectly stimulating host chondrogenesis and osteogenesis. In conclusion, the placenta is a practical source of stem cells for the treatment of OI.

  5. Longitudinal growth in chronic hypokalemic disorders.

    PubMed

    Gil-Peña, Helena; Mejia, Natalia; Alvarez-Garcia, Oscar; Loredo, Vanessa; Santos, Fernando

    2010-04-01

    Growth retardation remains a major complication in children with primary tubular disorders, despite adequate supplemental treatment with electrolytes, water and bicarbonate. Chronic hypokalemia, characteristic of some tubulopathies, impairs growth by mechanisms that are not well known. Association with growth hormone deficiency has been reported in patients with Bartter's or Gitelman's syndrome. Tissue-specific alterations of growth hormone and insulin-like growth factor I axis have been described in experimental models of potassium depletion. Hypokalemic rats gain less body length and weight than pair-fed normokalemic animals and, by contrast, develop renal hypertrophy. These rats have low circulating concentrations of insulin-like growth factor I, depressed messenger ribonucleic acid (mRNA) levels of this peptide in the tibial growth plate, and they are resistant to the longitudinal growth-promoting effects of exogenous growth hormone. The reason for this resistance remains to be defined. No alterations in the intracellular signaling for growth hormone have been found in the liver of hypokalemic rats. However, treatment with high doses of growth hormone is unable to normalize hypertrophy of the epiphyseal cartilage chondrocytes, which are severely disturbed in potassium depletion and likely play an important role in the pathogenia of growth impairment in this condition.

  6. Surgical Treatment of Congenital Hallux Varus

    PubMed Central

    Shim, Jong Sup; Koh, Kyoung Hwan; Lee, Do Kyung

    2014-01-01

    Background The purpose of this study was to report outcomes of congenital hallux varus deformity after surgical treatment. Methods We evaluated ten feet of eight patients with a congenital hallux varus deformity, including four feet combined with a longitudinal epiphyseal bracket (LEB). There were seven male patients and one female patient with a mean age of 33 months (range, 7 to 103 months) at the time of surgery. Two patients were bilaterally involved. The mean duration of follow-up was 5.9 years (range, 2.3 to 13.8 years). Clinical outcomes were assessed according to the criteria of Phelps and Grogan. Surgical procedures included the Farmer procedure, the McElvenny procedure or an osteotomy at the first metatarsal or proximal phalanx. Results The clinical results were excellent in two feet, good in six and poor in two feet. The LEB was associated with hallux varus in four feet and were treated by osteotomy alone or in conjunction with soft tissue procedure. Conclusions Congenital hallux varus was successfully corrected by surgery with overall favorable outcome. Preoperatively, a LEB should be considered as a possible cause of the deformity in order to prevent recurrent or residual varus after surgery. PMID:24900905

  7. Capital Realignment for Moderate and Severe SCFE Using a Modified Dunn Procedure

    PubMed Central

    Ziebarth, Kai; Zilkens, Christoph; Spencer, Samantha; Leunig, Michael; Ganz, Reinhold

    2009-01-01

    Moderate to severe slipped capital femoral epiphysis leads to premature osteoarthritis resulting from femoroacetabular impingement. We believe surgical correction at the site of deformity through capital reorientation is the best procedure to fully correct the deformity but has traditionally been associated with high rates of osteonecrosis. We describe a modified capital reorientation procedure performed through a surgical dislocation approach. We followed 40 patients for a minimum of 1 year and 3 years from two institutions. No patient developed osteonecrosis or chondrolysis. Slip angle was corrected to 4° to 8° and the mean alpha angle after correction was 40.6°. Articular cartilage damage, full-thickness loss, and delamination were observed at the time of surgery, especially in the stable slips. This technique appears to have an acceptable complication rate and appears reproducible for full correction of moderate to severe slipped capital femoral epiphyses with open physes. Level of Evidence: Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence. PMID:19142692

  8. [Legg-Calve-Perthes disease].

    PubMed

    Dutoit, M

    2007-01-01

    Treatment of Legg-Calvé-Perthes disease remains, in spite of realized progresses, difficult and disturbing. The important factors for establishing diagnosis concerning definition, etiology, physiopathology and clinical manifestations are reminded. The role of present complementary investigations as well as their evolution are very important to better establish the prognosis and the treatment. The most important factors for treating and establishing the prognosis of LPC are: 1) age. The prognosis is good in almost every case before 5 years old, without any treatment; 2) the prognosis is not good after 9 years, also, very often, after surgical treatment; 3) area of the epiphyseal necrosis, particularly of the lateral pillar; 4) extrusion of the hip at any age. Today, there is always no causal treatment of Legg-Calvé-Perthes disease. Conservative and surgical treatment aims to prevent deformities of the femoral head accompanied or not by articular incongruency, responsible for hip arthritis from the fourth decade. The place of conservative and surgical treatment as well as their complications are described; it aims to guide the spontaneous recovery with as little as possible, deformation of the femoral epiphysis.

  9. Scurvy in pediatric age group - A disease often forgotten?

    PubMed

    Agarwal, Anil; Shaharyar, Abbas; Kumar, Anubrat; Bhat, Mohd Shafi; Mishra, Madhusudan

    2015-06-01

    Scurvy is caused by prolonged severe dietary deficiency of vitamin C. Being rare as compared to other nutritional deficiencies, it is seldom suspected and this frequently leads to delayed recognition of this disorder. Children with abnormal dietary habits, mental illness or physical disabilities are prone to develop this disease. The disease spectrum of scurvy is quite varied and includes dermatological, dental, bone and systemic manifestations. Subperiosteal hematoma, ring epiphysis, metaphyseal white line and rarefaction zone along with epiphyseal slips are common radiological findings. High index of suspicion, detailed history and bilateral limb radiographs aids physician in diagnosing this eternal masquerader. We searched Pubmed for recent literature (2009-2014) with search terms "scurvy" "vitamin C deficiency" "ascorbic acid deficiency" "scurvy and children" "scurvy and pediatric age group". There were a total of 36 articles relevant to pediatric scurvy in children (7 reviews and 29 case reports) which were retrieved. The review briefly recapitulates the role of vitamin C, the various disease manifestations and the treatment of scurvy to create awareness of the disease which still is reported from our country, although sporadically. The recent advances related to scurvy and its management in pediatric age group are also incorporated.

  10. Asparaginase Potentiates Glucocorticoid-Induced Osteonecrosis in a Mouse Model.

    PubMed

    Liu, Chengcheng; Janke, Laura J; Kawedia, Jitesh D; Ramsey, Laura B; Cai, Xiangjun; Mattano, Leonard A; Boyd, Kelli L; Funk, Amy J; Relling, Mary V

    2016-01-01

    Osteonecrosis is a common dose-limiting toxicity of glucocorticoids. Data from clinical trials suggest that other medications can increase the risk of glucocorticoid-induced osteonecrosis. Here we utilized a mouse model to study the effect of asparaginase treatment on dexamethasone-induced osteonecrosis. Mice receiving asparaginase along with dexamethasone had a higher rate of osteonecrosis than those receiving only dexamethasone after 6 weeks of treatment (44% vs. 10%, P = 0.006). Similarly, epiphyseal arteriopathy, which we have shown to be an initiating event for osteonecrosis, was observed in 58% of mice receiving asparaginase and dexamethasone compared to 17% of mice receiving dexamethasone only (P = 0.007). As in the clinic, greater exposure to asparaginase was associated with greater plasma exposure to dexamethasone (P = 0.0001). This model also recapitulated other clinical risk factors for osteonecrosis, including age at start of treatment, and association with the systemic exposure to dexamethasone (P = 0.027) and asparaginase (P = 0.036). We conclude that asparaginase can potentiate the osteonecrotic effect of glucocorticoids.

  11. Osteonecrosis in children after allogeneic hematopoietic cell transplantation: study of prevalence, risk factors and longitudinal changes using MR imaging.

    PubMed

    Sharma, S; Leung, W-H; Deqing, P; Yang, J; Rochester, R; Britton, L; Neel, M D; Ness, K K; Kaste, S C

    2012-08-01

    Osteonecrosis after hematopoietic SCT (HCT) has seldom been addressed in pediatric populations. At our institution, since January 2002, children undergoing allogeneic HCT (alloHCT) receive yearly follow-up magnetic resonance imaging (MR) of hips and knees. To estimate the prevalence, longitudinal changes and associated risk factors for osteonecrosis after alloHCT, we reviewed MRs for children who underwent single alloHCT during the study period. We analyzed 149 of 344 patients who had post-HCT MR imaging performed (84 males; median age 11 years (range, 0.5-21 years)), median follow-up time was 32.6 months (range, 2.8-97.2 months). In all, 44 (29.5%) developed osteonecrosis of hips and/or knees; of those, 20 (45%) had at least 30% epiphyseal involvement. In 23 (52%), osteonecrosis lesions were identified in the first and in 43 (98%) by the third yearly scan. Knees were more frequently involved than hips; severity of osteonecrosis was greater in hips. Those who had pre-alloHCT osteonecrosis, two patients' hips and six patients' knees resolved completely; three patients' osteonecrosis lesions regressed after alloHCT. On risk factor analysis, age at time of alloHCT (P=0.051) and osteonecrosis identified by MRs before alloHCT (P=0.001) were the primary risk factors. This analysis shows that preventive strategies for osteonecrosis in this population should focus on measures to minimize risk factors before alloHCT.

  12. Scurvy in pediatric age group – A disease often forgotten?

    PubMed Central

    Agarwal, Anil; Shaharyar, Abbas; Kumar, Anubrat; Bhat, Mohd Shafi; Mishra, Madhusudan

    2015-01-01

    Scurvy is caused by prolonged severe dietary deficiency of vitamin C. Being rare as compared to other nutritional deficiencies, it is seldom suspected and this frequently leads to delayed recognition of this disorder. Children with abnormal dietary habits, mental illness or physical disabilities are prone to develop this disease. The disease spectrum of scurvy is quite varied and includes dermatological, dental, bone and systemic manifestations. Subperiosteal hematoma, ring epiphysis, metaphyseal white line and rarefaction zone along with epiphyseal slips are common radiological findings. High index of suspicion, detailed history and bilateral limb radiographs aids physician in diagnosing this eternal masquerader. We searched Pubmed for recent literature (2009–2014) with search terms “scurvy” “vitamin C deficiency” “ascorbic acid deficiency” “scurvy and children” “scurvy and pediatric age group”. There were a total of 36 articles relevant to pediatric scurvy in children (7 reviews and 29 case reports) which were retrieved. The review briefly recapitulates the role of vitamin C, the various disease manifestations and the treatment of scurvy to create awareness of the disease which still is reported from our country, although sporadically. The recent advances related to scurvy and its management in pediatric age group are also incorporated. PMID:25983516

  13. A review of terminology for equine juvenile osteochondral conditions (JOCC) based on anatomical and functional considerations.

    PubMed

    Denoix, J-M; Jeffcott, L B; McIlwraith, C W; van Weeren, P R

    2013-07-01

    This manuscript describes a new classification of the various joint-related lesions that can be seen in the young, growing horse based on their anatomical and functional aetiopathogenesis. Juvenile osteochondral conditions (JOCC) is a term that brings together specific disorders according to their location in the joint and their biomechanical origin. When a biomechanical insult affects the process of endochondral ossification different types of osteochondrosis (OC) lesions may occur, including osteochondral fragmentation of the articular surface or of the periarticular margins, or the formation of juvenile subchondral bone cysts. In severe cases, osteochondral collapse of the articular surface or the epiphysis or even an entire small bone may occur. Tension on ligament attachments may cause avulsion fractures of epiphyseal (or metaphyseal) ossifying bone, which are classified as JOCC, but do not result from a disturbance of the process of endochondral ossification and are not therefore classified as a form of OC. The same applies to 'physitis' which can result from damage to the physeal growth plate.

  14. [Effect of dosed diet restriction on physiological remodeling and bioelectric properties of bone].

    PubMed

    Levashov, M I; Ianko, R V; Chaka, E G; Safonov, S L

    2014-07-01

    The effect of dosed diet restriction on the physiological remodeling and bioelectric properties of bone tissue was studied in 48 male Wistar rats 3- and 18-months of age. The rate of bone tissue apposition was studied by the dynamic histomorphometry method (intravital tetracycline labeling). Electric potentials on the periosteal surface of the freshly isolated femurs were recorded. The magnitude of dielectric loss factor was determined to assess the quality of bone tissue. The control rats received a standard diet. The experimental rats received a limited diet (60 % of the standard mass) for 28 days. The magnitude and rate of the bone tissue apposition on the endosteal and periosteal surface of the tibia were less by 38.4% and 122.7% respectively in experimental rats after dosed diet restriction. Electric potential in the metaphyseal-epiphyseal growth zones of the femur was 29.7% lower, and the dielectric loss factor increased by 15.8%. The bone tissue apposition rate and the electric potential magnitude were increased 10 days after completion of the dosed diet restriction. The magnitude of the dielectric loss factor decreased after returning to the standard diet. Key words: dosed diet restriction, bone, remodelling, bioelectric properties.

  15. Oxalosis in primary hyperoxaluria in infancy : Report of a case in a 3-month-old baby. Follow-up for 3 years and review of literature.

    PubMed

    Orazi, Cinzia; Picca, Stefano; Schingo, Paolo M S; Fassari, Fausto M; Canepa, Giuseppe

    2009-04-01

    Primary hyperoxaluria (PH1) is a rare inborn autosomal recessive metabolic disorder due to the deficiency of hepatic alanine-glyoxylate-aminotransferase. This deficiency results in excessive synthesis and urinary excretion of oxalate, inducing renal stone formation and deposition of calcium oxalate in the kidney, bone, myocardium, and vessels (systemic oxalosis, SO) in the most severely affected individuals. We report renal and skeletal changes in a 3-month-old girl with PH1 and SO. Intense cortico-medullary hyperechogenicity and increased homogeneous radiopacity of normal-sized kidneys suggested the diagnosis of SO. Skeletal survey showed osteopenia and characteristic symmetrical metaphyseal transverse bands in long bones, progressively becoming more dense and migrating towards the diaphysis. Multiple pathological and slowly healing fractures of the limbs occurred at the dense band level. A radiopaque rim was then observed in flat bones, epiphyseal nuclei, and vertebral bodies. Inflammatory granulomatous reaction, induced by the presence of oxalate crystals in the marrow spaces, coexisted with progressively evident radiological signs of secondary hyperparathyroidism, with partially overlapping features. The patient was treated by peritoneal dialysis and hemodialysis until combined liver-kidney transplantation. There are no previous reports of infants treated with hemodialysis for more than 2 years.

  16. An atypical 0.73 MB microduplication of 22q11.21 and a novel SALL4 missense mutation associated with thumb agenesis and radioulnar synostosis.

    PubMed

    Diehl, Adam; Mu, Weiyi; Batista, Denise; Gunay-Aygun, Meral

    2015-07-01

    We describe a 0.73 Mb duplication of chromosome 22q11.21 between LCR-B and LCR-D and a missense mutation in a conserved C2H2 zinc finger domain of SALL4 in a cognitively normal patient with multiple skeletal anomalies including radioulnar synostosis, thumb aplasia, butterfly vertebrae, rib abnormalities, and hypoplasia of the humeral and femoral epiphyses. 22q11.21 is a common site for microdeletions and their reciprocal microduplications as a result of non-allelic homologous recombination between its multiple low copy repeat regions (LCR). DiGeorge /Velocardiofacial syndrome (DG/VCFS) is classically caused by a 3 Mb deletion between LCR-A and LCR-D or a 1.5 Mb deletion between LCR-A and LCR-B. The reciprocal syndrome to DG/VCFS is the recently described 22q11.2 microduplication, which usually presents with the typical 3 Mb or 1.5 Mb duplication. Numerous atypical deletions and duplications have been reported between other LCRs. Typically, SALL4-related Duane-radial ray syndrome is caused by deletions or nonsense mutations; the only missense SALL4 mutation described prior was thought to result in gain of function and produced cranial midline defects. The skeletal anomalies presented in this report have not been previously described in association with 22q11.2 microduplication nor SALL4 mutations.

  17. Effects of electrical stimulation on rat limb regeneration, a new look at an old model

    PubMed Central

    Leppik, Liudmila P.; Froemel, Dara; Slavici, Andrei; Ovadia, Zachri N.; Hudak, Lukasz; Henrich, Dirk; Marzi, Ingo; Barker, John H.

    2015-01-01

    Limb loss is a devastating disability and while current treatments provide aesthetic and functional restoration, they are associated with complications and risks. The optimal solution would be to harness the body’s regenerative capabilities to regrow new limbs. Several methods have been tried to regrow limbs in mammals, but none have succeeded. One such attempt, in the early 1970s, used electrical stimulation and demonstrated partial limb regeneration. Several researchers reproduced these findings, applying low voltage DC electrical stimulation to the stumps of amputated rat forelimbs reporting “blastema, and new bone, bone marrow, cartilage, nerve, skin, muscle and epiphyseal plate formation”. In spite of these encouraging results this research was discontinued. Recently there has been renewed interest in studying electrical stimulation, primarily at a cellular and subcellular level, and studies have demonstrated changes in stem cell behavior with increased proliferation, differentiation, matrix formation and migration, all important in tissue regeneration. We applied electrical stimulation, in vivo, to the stumps of amputated rat limbs and observed significant new bone, cartilage and vessel formation and prevention of neuroma formation. These findings demonstrate that electricity stimulates tissue regeneration and form the basis for further research leading to possible new treatments for regenerating limbs. PMID:26678416

  18. Lifelong sedentary behaviour and femur structure.

    PubMed

    Fonseca, H; Gonçalves, D; Figueiredo, P; Mota, M P; Duarte, J A

    2011-05-01

    The aim of the present study was to analyze the lifelong differences of femur structure in sedentary and physically active animal models. Thirty male C57BL/6 mice, 2 months old, were either: i) housed in cages with running wheel (AA; n=10), ii) housed in cages without running wheel (AS; n=10), iii) or sacrificed without intervention (Y; n=10). AA and AS animals were sacrificed after 23 months of housing. Right femur structure was analyzed in all animals by histomorphometry. Significant differences in several microarchitectural parameters of cancellous and cortical bone were identified between Y mice and both groups of aged mice, as well as between AA and AS groups. Lifelong physically active mice had significantly higher cancellous bone surface (Cn.BS) and trabecular number (Tb.N) and decreased trabecular separation (Tb.Sp) at both epiphyses when compared to AS animals. No differences were observed between Y and AA groups regarding osteocyte number (N.Ot) despite its significant reduction in AS animals, suggesting that age alone was not a cause for decreases in N.Ot. Our results suggest that the reduced bone quality observed in the elderly is not only a consequence of age but also of lack of physical activity since sedentary behaviour significantly aggravated the degenerative age-related bone differences.

  19. Human bone hardness seems to depend on tissue type but not on anatomical site in the long bones of an old subject.

    PubMed

    Ohman, Caroline; Zwierzak, Iwona; Baleani, Massimiliano; Viceconti, Marco

    2013-02-01

    It has been hypothesised that among different human subjects, the bone tissue quality varies as a function of the bone segment morphology. The aim of this study was to assess and compare the quality, evaluated in terms of hardness of packages of lamellae, of cortical and trabecular bones, at different anatomical sites within the human skeleton. The contralateral six long bones of an old human subject were indented at different levels along the diaphysis and at both epiphyses of each bone. Hardness value, which is correlated to the degree of mineralisation, of both cortical and trabecular bone tissues was calculated for each indentation location. It was found that the cortical bone tissue was harder (+18%) than the trabecular one. In general, the bone hardness was found to be locally highly heterogeneous. In fact, considering one single slice obtained for a bone segment, the coefficient of variation of the hardness values was up to 12% for cortical bone and up to 17% for trabecular bone. However, the tissue hardness was on average quite homogeneous within and among the long bones of the studied donor, although differences up to 9% among levels and up to 7% among bone segments were found. These findings seem not to support the mentioned hypothesis, at least not for the long bones of an old subject.

  20. Multimodal imaging reveals structural and functional heterogeneity in different bone marrow compartments: functional implications on hematopoietic stem cells.

    PubMed

    Lassailly, Francois; Foster, Katie; Lopez-Onieva, Lourdes; Currie, Erin; Bonnet, Dominique

    2013-09-05

    Intravital microscopy of the calvarium is the only noninvasive method for high-resolution imaging of the bone marrow (BM) and hematopoietic stem cell (HSC) niches. However, it is unclear if the calvarium is representative of all BM compartments. Using the combination of whole body optical imaging, intravital microscopy, and "in vivo fluorescence trapping," a thorough comparison of HSCs and putative HSC niches in the calvaria, epiphyses, and diaphyses, at steady state or after HSC transplantation, can be made. We report substantial heterogeneity between different BM compartments in terms of bone-remodeling activity (BRA), blood volume fraction (BVF), and hypoxia. Although BVF is high in all BM compartments, including areas adjacent to the endosteum, we found that compartments displaying the highest BVF and BRA were preferentially seeded and engrafted upon HSC transplantation. Unexpectedly, the macroanatomical distribution of HSCs at steady state is homogeneous across these 3 areas and independent of these 2 parameters and suggests the existence of "reconstituting niches," which are distinct from "homeostatic niches." Both types of niches were observed in the calvarium, indicating that endochondral ossification, the process needed for the formation of HSC niches during embryogenesis, is dispensable for the formation of HSC niches during adulthood.

  1. Phenotypic expressions of a Gly154Arg mutation in type II collagen in two unrelated patients with spondyloepimetaphyseal dysplasia (SEMD)

    SciTech Connect

    Kaitila, I.; Marttinen, E.; Koerkkoe, J.; Ala-Kokko, L.

    1996-05-03

    Type II collagenopathies consist of chondrodysplasia ranging from lethal to mild in severity. A large number of mutations has been found in the COL2A1 gene. Glycine substitutions have been the most common types of mutation. Genotype-phenotype correlations in type II collagenopathies have not been established, partly because of insufficient clinical and radiographic description of the patients. We found a glycine-to-arginine substitution at position 154 in type II collagen in two unrelated isolated propositi with spondyloepimetaphyseal dysplasia and provide a comparative clinical and radiographic analysis from birth to young adulthood for this condition. The clinical phenotype was disproportionate short stature with varus/valgus deformities of the lower limbs requiring corrective osteotomies, and lumbar lordosis. The skeletal radiographs showed an evolution from short tubular bones, delayed epiphyseal development, and mild vertebral involvement to severe metaphyseal dysplasia with dappling irregularities, and hip {open_quotes}dysplasia.{close_quotes} The metaphyseal abnormalities disappeared by adulthood. 27 refs., 11 figs., 1 tab.

  2. Phenotypic features of the domestic pigs bred in the Roman settlements of Pompeii and Caralis.

    PubMed

    Manca, Paolo; Farina, Vittorio; Gadau, Sergio; Lepore, Gianluca; Genovese, Angelo; Zedda, Marco

    2004-01-01

    A reconstruction of the morphological features of domestic pigs from two Roman settlements is here suggested by means of the study of skeletal and dental remains, with the aim at evaluating their degree of selection in comparison with wild boars. Material was formed by 111 bone and tooth fragments and was uncovered during the excavations of Polybius' House in Pompeii and of Roman buildings in the neighbourhood of Caralis harbour (Sardinia). The remains underwent morphological examination. The eruption of permanent teeth and ossification of epiphyseal cartilages let us establish that most animals were over 18-20 months. When possible, the determination of sex was made by detecting tusk features. The presence of anthropic signs on the bone surface provides some information about slaughtering and cooking procedure in the Roman period and supports the hypothesis that the animal remnants were food remains. Osteometric analysis was carried out on long and short bones and teeth through suitable multiplicative parameters, leading to the assessment of the withers height and other main phenotypic features. Logarithmic deviation pointed out the significant osteometric differences between the domestic pigs from the two Roman settlements. These data were also compared with those from wild boars and modern crossbred wild boars X non-selected pigs. In conclusion, our data show that pigs from Caralis bear much resemblance to wild boars, whereas those from Pompeii appear to be improved, so sharing some phenotypic features of modem improved breeds.

  3. Human skeletal dysplasia caused by a constitutive activated transient receptor potential vanilloid 4 (TRPV4) cation channel mutation.

    PubMed

    Kang, Sang Sun; Shin, Sung Hwa; Auh, Chung-Kyoon; Chun, Jaesun

    2012-12-31

    The transient receptor potential vanilloid 4 (TRPV4) cation channel, a member of the TRP vanilloid subfamily, is expressed in a broad range of tissues where it participates in the generation of Ca²⁺ signals and/or depolarization of the membrane potential. Regulation of TRPV4 abundance at the cell surface is critical for osmo- and mechanotransduction. Defects in TRPV4 are the cause of several human diseases, including brachyolmia type 3 (MIM:113500) (also known as brachyrachia or spondylometaphyseal dysplasia Kozlowski type [MIM:118452]), and metatropic dysplasia (MIM:156530) (also called metatropic dwarfism or parastremmatic dwarfism [MIM:168400]). These bone dysplasia mutants are characterized by severe dwarfism, kyphoscoliosis, distortion and bowing of the extremities, and contractures of the large joints. These diseases are characterized by a combination of decreased bone density, bowing of the long bones, platyspondyly, and striking irregularities of endochondral ossification with areas of calcific stippling and streaking in radiolucent epiphyses, metaphyses, and apophyses. In this review, we discuss the potential effect of the mutation on the regulation of TRPV4 functions, which are related to human diseases through deviated function. In particular, we emphasize how the constitutive active TRPV4 mutant affects endochondral ossification with a reduced number of hypertrophic chondrocytes and the presence of cartilage islands within the zone of primary mineralization. In addition, we summarize current knowledge about the role of TRPV4 in the pathogenesis of several diseases.

  4. Longitudinal long-term results of surgical treatment in patients with osteochondritis dissecans of the femoral condyles.

    PubMed

    Bruns, Juergen; Rayf, Mark; Steinhagen, Joern

    2008-05-01

    In a longitudinal study, we performed a second follow-up examination on patients suffering from osteochondritis dissecans at the femoral condyles 10 years after a first follow-up, which had been performed 10 years after surgical treatment. Results (clinical score; radiological signs of OA) were analysed depending on the stage of the epiphyseal plate at the time of surgery, the used surgical procedure was divided into retrograde and anterograde procedures, and removal of loose bodies depending on the stage of the lesion. The analysis clearly exhibited that JOCD patients demonstrated better results than AOCD patients. The clinical score obtained after 10 years improved significantly with time, particularly for JOCD patients. Overall, when a retrograde procedure had been used in cases with an intact cartilage layer clinical results were better than those obtained in patients in whom an anterograde procedure with restoration of the joint surface or simple removal of the loose fragments had been performed. After a mean follow-up of 20 years the mean OA-stage was 0.27 in JOCD patients, whereas in AOCD patients a mean OA-stage of 1.55 was detected. Worst OA-changes were detected in patients in whom acrylic glue had been used for refixation of the loose bodies.

  5. The crystal structure of the signature domain of cartilage oligomeric matrix protein: implications for collagen, glycosaminoglycan and integrin binding.

    PubMed

    Tan, Kemin; Duquette, Mark; Joachimiak, Andrzej; Lawler, Jack

    2009-08-01

    Cartilage oligomeric matrix protein (COMP), or thrombospondin-5 (TSP-5), is a secreted glycoprotein that is important for growth plate organization and function. Mutations in COMP cause two skeletal dysplasias, pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (EDM1). In this study, we determined the structure of a recombinant protein that contains the last epidermal growth factor repeat, the type 3 repeats and the C-terminal domain (CTD) of COMP to 3.15-A resolution limit by X-ray crystallography. The CTD is a beta-sandwich that is composed of 15 antiparallel beta-strands, and the type 3 repeats are a contiguous series of calcium binding sites that associate with the CTD at multiple points. The crystal packing reveals an exposed potential metal-ion-dependent adhesion site (MIDAS) on one edge of the beta-sandwich that is common to all TSPs and may serve as a binding site for collagens and other ligands. Disease-causing mutations in COMP disrupt calcium binding, disulfide bond formation, intramolecular interactions, or sites for potential ligand binding. The structure presented here and its unique molecular packing in the crystal identify potential interactive sites for glycosaminoglycans, integrins, and collagens, which are key to cartilage structure and function.

  6. Maternal Flaxseed Oil During Lactation Enhances Bone Development in Male Rat Pups.

    PubMed

    Pereira, Aline D'Avila; Ribeiro, Danielle Cavalcante; de Santana, Fernanda Carvalho; de Sousa Dos Santos, Aline; Mancini-Filho, Jorge; do Nascimento-Saba, Celly Cristina Alves; Velarde, Luis Guillermo Coca; da Costa, Carlos Alberto Soares; Boaventura, Gilson Teles

    2016-08-01

    Flaxseed oil is an alpha linolenic acid source important in the growth and body development stage; furthermore, this acid acts on adipose tissue and bone health. The aim of this study was to evaluate body composition, fatty acid composition, hormone profile, retroperitoneal adipocyte area and femur structure of pups at weaning, whose mothers were fed a diet containing flaxseed oil during lactation. After birth, pups were randomly assigned: control (C, n = 12) and flaxseed oil (FO, n = 12), rats whose mothers were treated with diet containing soybean or flaxseed oil. At 21 days, the pups were weaned and body mass, length, body composition, biochemical parameter, leptin, osteoprotegerin, osteocalcin, fatty acids composition, intra-abdominal fat mass and femur structure were analyzed. FO showed (p < 0.05): higher body mass (+12 %) and length (+9 %); body fat mass (g, +45 %); bone mineral density (+8 %), bone mineral content (+55 %) and bone area (+35 %), osteocalcin (+173 %) and osteoprotegerin (+183 %). Arachidonic acid was lower (p < 0.0001), alpha-linolenic and eicosapentaenoic were higher (p < 0.0001). Intra-abdominal fat mass was higher (+25 %), however, the retroperitoneal adipocytes area was lower (-44 %). Femur mass (+10 %), distance between epiphyses (+4 %) and bone mineral density (+13 %) were higher. The study demonstrates that adequate flaxseed oil content during a lactation diet plays an important role in the development of pups.

  7. Flaxseed flour, compared to flaxseed oil, contributes to femoral structure in male rats subjected to early weaning.

    PubMed

    Pessanha, Carolina Ribeiro; da Camara Boueri, Bianca Ferolla; da Costa, Luanna Rodrigues; Ferreira, Maitê Rocha; de Abreu, Maíra Duque Coutinho; Pessoa, Letícia Rozeno; Pereira, Aline D'Avila; Ribeiro, Danielle Cavalcante; da Silva, Eduardo Moreira; da Costa, Carlos Alberto Soares; Boaventura, Gilson Teles

    2016-03-01

    The aim of this study was to analyze whether flaxseed flour or flaxseed oil treatment contributes to femoral structure in male rats subjected to early weaning. Pups were weaned for separation from mothers at 14 days (early weaning, EW) or 21 days (control, C). After 21 days, the control (C60) was fed with the control diet. The EW group was divided based on control (EWC60), flaxseed flour (EWFF60) and flaxseed oil (EWFO60) diets until 60 days. Femoral dimension, bone mineral density (BMD), bone mineral content (BMC), area and biomechanical properties were determined. EWFO60 showed lower (P < 0.05) femur mass. EWC60 and EWFO60 showed lower (P < 0.05) distance between epiphyses, diaphysis width and BMD. BMC was lower (P < 0.05) in EWC60 (vs. C60 and EWFF60). EWC60 and EWFO60 showed lower (P < 0.05) maximum force (vs. C60). Breaking strength was lower (P < 0.05) in EWFO60 (vs. C60). EWFF60 showed higher (P < 0.05) rigidity. Flaxseed flour abbreviated the femoral fragility secondary to early weaning.

  8. Mutations in DDR2 gene cause SMED with short limbs and abnormal calcifications.

    PubMed

    Bargal, Ruth; Cormier-Daire, Valerie; Ben-Neriah, Ziva; Le Merrer, Martine; Sosna, Jacob; Melki, Judith; Zangen, David H; Smithson, Sarah F; Borochowitz, Zvi; Belostotsky, Ruth; Raas-Rothschild, Annick

    2009-01-01

    The spondylo-meta-epiphyseal dysplasia [SMED] short limb-hand type [SMED-SL] is a rare autosomal-recessive disease, first reported by Borochowitz et al. in 1993.(1) Since then, 14 affected patients have been reported.(2-5) We diagnosed 6 patients from 5 different consanguineous Arab Muslim families from the Jerusalem area with SMED-SL. Additionally, we studied two patients from Algerian and Pakistani ancestry and the parents of the first Jewish patients reported.(1) Using a homozygosity mapping strategy, we located a candidate region on chromosome 1q23 spanning 2.4 Mb. The position of the Discoidin Domain Receptor 2 (DDR2) gene within the candidate region and the similarity of the ddr2 knockout mouse to the SMED patients' phenotype prompted us to study this gene(6). We identified three missense mutations c.2254 C > T [R752C], c. 2177 T > G [I726R], c.2138C > T [T713I] and one splice site mutation [IVS17+1g > a] in the conserved sequence encoding the tyrosine kinase domain of the DDR2 gene. The results of this study will permit an accurate early prenatal diagnosis and carrier screening for families at risk.

  9. Hunter-McAlpine craniosynostosis phenotype associated with skeletal anomalies and interstitial deletion of chromosome 17q

    SciTech Connect

    Thomas, J.; Prescott, K.; Milner, R.

    1994-09-01

    Syndromic craniosynostosis is frequently associated with skeletal abnormalities, but the biological basis for this association is unclear. Molecular genetic studies have the biological basis for this association is unclear. Molecular genetic studies have identified a number of loci and at least one candidate gene, the MSX2 gene. We recently encountered a 9 y.o. boy with moderate mental retardation, congenital craniosynostosis, and multiple skeletal anomalies. Physical features strongly suggested Hunter-McAlpine syndrome (HMS). Specifically, he had triangular facies with a small mouth prominent chin, bulbous nose, thin vermillion border, malaligned and malformed teeth, and low set, rudimentary ears. Skeletal features included: bilambdoidal, bicoronal, and sagittal craniosynostosis; right preaxial polydactyly; bilateral talipes; coxa valga; genu valgum; bilateral fusion of the hamate and capitate; scoliosis; and small, irregular middle phalangeal epiphyses. High resolution chromosome analysis revealed an interstitial deletion of G negative material of subbands q23.1{r_arrow}23.3 or q23.3{r_arrow}q24.2 of a No. 17 homologue. HMS, a presumed autosomal dominant disorder associated with characteristic facies, variable degrees of mental retardation, craniosynostosis, and minor acral-skeletal anomalies, proved to be the most likely explanation for this patient`s findings. We propose that our patient has a new mutation for HMS with more severe skeletal involvement than previously reported. Linkage studies are in progress to test the hypothesis that familial HMS may be localized to chromosome 17.

  10. A unique point mutation in the fibroblast growth factor receptor 3 gene (FGFR3) defines a new craniosynostosis syndrome

    SciTech Connect

    Muenke, M.; Gripp, K.W.; McDonald-McGinn, D.M.

    1997-03-01

    The underlying basis of many forms of syndromic craniosynostosis has been defined on a molecular level. However, many patients with familial or sporadic craniosynostosis do not have the classical findings of those craniosynostosis syndromes. Here we present 61 individuals from 20 unrelated families where coronal synostosis is due to an amino acid substitution (Pro250Arg) that results from a single point mutation in the fibroblast growth factor receptor 3 gene on chromosome 4p. In this instance, a new clinical syndrome is being defined on the basis of the molecular finding. In addition to the skull findings, some patients had abnormalities on radiographs of hands and feet, including thimble-like middle phalanges, coned epiphyses, and carpal and tarsal fusions. Brachydactyly was seen in some cases; none had clinically significant syndactyly or deviation of the great toe. Sensorineural hearing loss was present in some, and developmental delay was seen in a minority. While the radiological findings of hands and feet can be very helpful in diagnosing this syndrome, it is not in all cases clearly distinguishable on a clinical basis from other craniosynostosis syndromes. Therefore, this mutation should be tested for in patients with coronal synostosis. 54 refs., 4 figs., 2 tabs.

  11. Spontaneous rickets in the wild arctic fox Alopex lagopus

    SciTech Connect

    Ogden, J.A.; Conlogue, G.J.

    1981-10-01

    Normal and rachitic, skeletally immature arctic foxes (Alopex lagopus) were subjected to physical examination, roentgenographic studies, and in some cases histologic studies. The involved animals had active rickets coupled with antecedent normal diaphyseal bone formation. Evaluation of all the long bones showed highly variable manifestations of the disease, which undoubtedly reflect different rates of physeal endochondral transformation and metaphyseal remodeling. Histologic examination showed distinct patterns of widening of the physes and variable osteodystrophy in the trabecular and cortical bone of the metaphyses and epiphyseal ossification centers. These aforementioned factors certainly would necessitate different regional calcium needs and, therefore, different regional responses to an overall calcium deficiency. The physes involved in the most rapid growth rates in this period showed the most widening of the growth plate, and the most dystrophic changes in the metaphysis. Skeletal injuries, including metaphyseal fractures and slow-down of longitudinal growth (particularly in the ulna) were also evident. Because of apparent dietary differences in the affected and normal fox kits, this juvenile-onset disease was presumed due to calcium-deficient intake following weaning. To the best of our knowledge this is the first report of spontaneously occurring rickets in a wild animal in its natural habitat. There are several possible mechanisms for the variable widening of the physis and the loss of bone mineralization in these fox kits: calcium-deficient diet, binding of calcium in the bowel by high phosphorus intake, secondary hyperparathyroidism, and vitamin A toxicity.

  12. Effects of zinc deficiency on pituitary somatotrophs

    SciTech Connect

    Focht, S.; Fosmire, G.; Hymer, W.C. )

    1991-03-15

    To test whether the growth retardation that accompanies zinc deficiency may involve a direct effect of zinc on pituitary, rats were divided into three groups and fed a Zn deficient diet for 10 days. On day 10 rats were anesthetized and blood, femurs, and pituitaries were collected. Pituitaries from each group were either enzymatically dissociated into individual cells or homogenized and centrifuged into three fractions. Growth retardation was evidenced by decreased body weights and narrower epiphyseal widths in PF and ZD rats. Pituitary weights were also lower in PF and ZD rats. Pituitary zinc per unit tended to be highest in PF and lowest in ZD rats, although this trend was significant only for the 250g pellet fraction. Total cell counts from dissociated pituitaries tended to be highest for AL and lowest for ZD rats. Total growth hormone (HGH) per pituitary also followed this trend, although the amount of GH per somatotroph did not buffer between groups. Interestingly, GH released from dissociated pituitary cells cultured in defined media for 3 days was twice as great from ZD vs PF, AL being intermediate. Serum GH levels did not differ between groups although the trend was ZD > PF >AL.

  13. Camptomelia in a rhesus macaque (Macaca mulatta).

    PubMed

    Hopper, Kelly; Morales, Pablo; Garcia, Anapatricia; Wagner, Joseph

    2010-11-01

    An 8.5-mo-old female rhesus macaque was examined for an apparent lump on the right arm, below the elbow. The macaque showed no signs of pain or discomfort. Examination revealed that the lump was actually a bend in the forearm. Radiography demonstrated that some of the long bones of the animal were bowed. Differential diagnoses included rickets, hyperparathyroidism, pseudohyperparathyroidism, and a growth dysplasia. No other similar abnormalities in animals from that cage or any other enclosure in our large colony were observed. Blood chemistries and a complete hemogram were within normal limits for a macaque of this age. Serum was submitted for a vitamin D profile that included assays for parathyroid hormone, 25-hydroxyvitamin D, and ionized calcium. Serum samples from sex- and age-matched normal controls were sent for comparison and to establish a baseline profile. The affected animal had vitamin D levels comparable to unaffected controls. Bone biopsies appeared normal for a macaque of this age. Fluorine levels in the drinking water supply were within acceptable limits. Consistent with the information available, a diagnosis of idiopathic camptomelia, or bowing of the long bones, was made. In humans, developmental camptomelia is associated with several bone dysplasias in infants and children. These conditions are thought to be caused by genetic mutations in enzymes or transcription factors that control development of the epiphyses and are almost always associated with other lethal and nonlethal developmental abnormalities.

  14. Multi-axial mechanical properties of human trabecular bone.

    PubMed

    Rincón-Kohli, Liliana; Zysset, Philippe K

    2009-06-01

    In the context of osteoporosis, evaluation of bone fracture risk and improved design of epiphyseal bone implants rely on accurate knowledge of the mechanical properties of trabecular bone. A multi-axial loading chamber was designed, built and applied to explore the compressive multi-axial yield and strength properties of human trabecular bone from different anatomical locations. A thorough experimental protocol was elaborated for extraction of cylindrical bone samples, assessment of their morphology by micro-computed tomography and application of different mechanical tests: torsion, uni-axial traction, uni-axial compression and multi-axial compression. A total of 128 bone samples were processed through the protocol and subjected to one of the mechanical tests up to yield and failure. The elastic data were analyzed using a tensorial fabric-elasticity relationship, while the yield and strength data were analyzed with fabric-based, conewise generalized Hill criteria. For each loading mode and more importantly for the combined results, strong relationships were demonstrated between volume fraction, fabric and the elastic, yield and strength properties of human trabecular bone. Despite the reviewed limitations, the obtained results will help improve the simulation of the damage behavior of human bones and bone-implant systems using the finite element method.

  15. Osteoporosis in juvenile-onset diabetes mellitus: morphometric and comparative studies.

    PubMed

    Soejima, K; Landing, B H

    1986-01-01

    Ribs and vertebrae of 8 children and young adults aged from 17 months to 24 years with juvenile-onset diabetes mellitus, 4 with diabetes secondary to cystic fibrosis and 2 with diabetes secondary to thalassemia major, were analyzed for osteoporosis by a point-count morphometric method. The mean ratio of bone spicule to marrow space in cancellous bone of ribs of patients with juvenile-onset diabetes mellitus or with diabetes mellitus secondary to cystic fibrosis or thalassemia was 55% that of 10 control patients. The lengths of the zones of proliferating and mature cartilage cells in costal epiphyses of patients with juvenile-onset diabetes mellitus were also below normal. The ratio of bone spicule to marrow space of vertebrae of the diabetic patients was not significantly different from control values. The data confirm clinical reports that osteoporosis is a regular feature of juvenile-onset diabetes mellitus and suggest that the degree of bone matrix and mineral deficiency in such patients is greater than is usually considered.

  16. Association of congenital deficiency of multiple vitamin K-dependent coagulation factors and the phenotype of the warfarin embryopathy: clues to the mechanism of teratogenicity of coumarin derivatives.

    PubMed Central

    Pauli, R M; Lian, J B; Mosher, D F; Suttie, J W

    1987-01-01

    We have evaluated a boy who had excessive bleeding and bruising from birth and showed markedly prolonged prothrombin times, partially correctable by oral vitamin K administration. Additional laboratory studies demonstrated decreased activities of plasma factors II, VII, IX, and X; near normal levels of immunologically detected and calcium binding-independent prothrombin; undercarboxylation of prothrombin; excess circulating vitamin K epoxide; decreased excretion of carboxylated glutamic acid residues; and abnormal circulating osteocalcin. These results all are consistent with effects resulting from decreased posttranslational carboxylation secondary to an inborn deficiency of vitamin K epoxide reductase. This individual also had nasal hypoplasia, distal digital hypoplasia, and epiphyseal stippling on infant radiographs, all of which are virtually identical to features seen secondary to first-trimester exposure to coumarin derivatives. Therefore, by inference, the warfarin embryopathy is probably secondary to warfarin's primary pharmacologic effect (interference with vitamin K-dependent posttranslational carboxylation of glutamyl residues of various proteins) and may result from undercarboxylation of osteocalcin or other vitamin K-dependent bone proteins. Images Fig. 1 Fig. 2 Fig. 3 PMID:3499071

  17. A rare probable chondroblastoma of the calcaneus in a pre-Columbian subadult from Illinois.

    PubMed

    Smith, Maria Ostendorf; Nicosia, Christopher E

    2017-03-01

    Discrete cystic or tumorous intraosseous lesions can arise from a variety of benign and malignant conditions as well as trauma and infection. They are clinically rarely observed in the calcaneus. A fourteen-to-seventeen-year-old subadult recovered from a Late Woodland (∼AD 800-1100) period mortuary context in the Mississippi River Valley of central Illinois presents with a single lytic intraosseous lesion on the posterior right calcaneus that bilaterally perforates the cortex. The lesion, although primarily anterior to the epiphyseal plate, does breach it. There is also a small perforation of the outer cortex of the epiphysis above the insertion of the Achilles' tendon. The lesion is well-defined with a primarily spongy cancellous interior margin. On the body of the calcaneus, there is periostosis and a slightly expansive endosteal reaction. Comparative radiographic assessments undertaken to differentially diagnose the lesion indicate that it was likely not malignant. Based on the posterior location, the radiographic signature, the bilateral cortical perforation and the breach of the epiphysis, the lesion is best interpreted as a chondroblastoma.

  18. IGF-1 and insulin as growth hormones.

    PubMed

    Laron, Zvi

    2004-01-01

    IGF-1 generated in the liver is the anabolic effector and linear growth promoting hormone of the pituitary growth hormone (GH). This is evidenced by dwarfism in states of congenital IGF-1 deficiency, Igf1 gene mutation/deletions or knockouts, and in Laron syndrome (LS), due to GH receptor gene mutations/deletions or IGF-1 receptor blocking. In a positive way, daily IGF-1 administration to stunted patients with LS or hGH gene deletion accelerates linear growth velocity. IGF-1 acts on the proliferative cells of the epiphyseal cartilage. IGF-1 also induces organ and tissue growth; its absence causing organomicria. Insulin shares a common ancestry with IGF-1 and with 45% amino acid homology, as well as very close relationships in the structure of its receptors and post-receptor cascade, also acts as a growth hormone. It has protein anabolic activity and stimulates IGF-1 synthesis. Pancreas agenesis causes short babies, and obese children with hyperinsulinism, with or without pituitary GH, have an accelerated growth rate and skeletal maturation; so do babies with macrosomia. Whether the insulin growth effect is direct, or mediated by IGF-1 or leptin is controversial.

  19. Hyperostosis frontalis interna (HFI) and castration: the case of the famous singer Farinelli (1705-1782).

    PubMed

    Belcastro, Maria Giovanna; Todero, Antonio; Fornaciari, Gino; Mariotti, Valentina

    2011-11-01

    The famous castrato singer Farinelli (1705-1782) was exhumed by our research group in July 2006 for the purpose of gaining some insight into his biological profile through a study of his skeletal remains. Farinelli was castrated before puberty to preserve the treble pitch of the boy's voice into adult life. His powerful and sweet voice became legendary. In spite of its bad preservation state, the skeleton displayed some interesting characteristics that are probably related to the effects of castration, including long limb-bones, persistence of epiphyseal lines and osteoporosis. In particular, the frontal bone was affected by severe hyperostosis frontalis interna (HFI). This condition consists in a symmetrical thickening of the inner table of the bone. The epidemiology of HFI shows that it is relatively common in postmenopausal women but very rare in men. Men affected by this pathology suffer from diseases, syndromes or treatments causing androgen deficiency. In the case of Farinelli, castration was probably responsible for the onset and development of this lesion.

  20. Bone loss at the distal femur and proximal tibia in persons with spinal cord injury: imaging approaches, risk of fracture, and potential treatment options.

    PubMed

    Cirnigliaro, C M; Myslinski, M J; La Fountaine, M F; Kirshblum, S C; Forrest, G F; Bauman, W A

    2017-03-01

    Persons with spinal cord injury (SCI) undergo immediate unloading of the skeleton and, as a result, have severe bone loss below the level of lesion associated with increased risk of long-bone fractures. The pattern of bone loss in individuals with SCI differs from other forms of secondary osteoporosis because the skeleton above the level of lesion remains unaffected, while marked bone loss occurs in the regions of neurological impairment. Striking demineralization of the trabecular epiphyses of the distal femur (supracondylar) and proximal tibia occurs, with the knee region being highly vulnerable to fracture because many accidents occur while sitting in a wheelchair, making the knee region the first point of contact to any applied force. To quantify bone mineral density (BMD) at the knee, dual energy x-ray absorptiometry (DXA) and/or computed tomography (CT) bone densitometry are routinely employed in the clinical and research settings. A detailed review of imaging methods to acquire and quantify BMD at the distal femur and proximal tibia has not been performed to date but, if available, would serve as a reference for clinicians and researchers. This article will discuss the risk of fracture at the knee in persons with SCI, imaging methods to acquire and quantify BMD at the distal femur and proximal tibia, and treatment options available for prophylaxis against or reversal of osteoporosis in individuals with SCI.

  1. The role of energy dissipation of polymeric scaffolds in the mechanobiological modulation of chondrogenic expression.

    PubMed

    Abdel-Sayed, Philippe; Darwiche, Salim E; Kettenberger, Ulrike; Pioletti, Dominique P

    2014-02-01

    Mechanical stimulation has been proposed to induce chondrogenesis in cell-seeded scaffolds. However, the effects of mechanical stimuli on engineered cartilage may vary substantially between different scaffolds. This advocates for the need to identify an overarching mechanobiological variable. We hypothesize that energy dissipation of scaffolds subjected to dynamic loading may be used as a mechanobiology variable. The energy dissipation would furnish a general criterion to adjust the mechanical stimulation favoring chondrogenesis in scaffold. Epiphyseal chondro-progenitor cells were then subject to unconfined compression 2 h per day during four days in different scaffolds, which differ only by the level of dissipation they generated while keeping the same loading conditions. Scaffolds with higher dissipation levels upregulated the mRNA of chondrogenic markers. In contrast lower dissipation of scaffolds was associated with downregulation of chondrogenic markers. These results showed that energy dissipation could be considered as a mechanobiology variable in cartilage. This study also indicated that scaffolds with energy dissipation level close to the one of cartilage favors chondrogenic expression when dynamical loading is present.

  2. Radiological imaging in pediatric rheumatic diseases

    PubMed Central

    Matuszewska, Genowefa; Zaniewicz-Kaniewska, Katarzyna; Włodkowska-Korytkowska, Monika; Smorawińska, Patrycja; Saied, Fadhil; Kunisz, Wojciech; Sudoł-Szopińska, Iwona

    2014-01-01

    Summary Radiological imaging plays a fundamental role in the diagnosis and monitoring of rheumatic diseases. The basic method of imaging is a classic X-ray picture, which for many years has been used as a single method for the recognition and evaluation of the effects of disease management. In today’s modern day treatment of rheumatic diseases, ultrasonography and magnetic resonance are more commonly performed for early detection of inflammatory changes in the region of soft tissue, subchondral bone and bone marrow. In spite of their usefulness and fundamental role in the diagnosis, X-ray still remains an essential tool in the diagnosis of rheumatoid arthritis in children and is complementary to today’s methods of imaging diagnostics. In clinical practice, X-ray imaging is still an important examination performed not only to recognize the disorders, but also to provide a differential diagnosis. It helps estimate disease progression and is used to monitor the effects of treatment and the development of possible complications. Differential diagnosis of rheumatic diseases is performed on the basis of localization and type of radiographic changes. The surrounding periarticular soft tissues, bone structures, joint space, with special attention to articular bone surfaces and epiphyses, are analyzed. The aim of this work is to describe characteristic inflammatory changes present on X-ray imaging typical for the most commonly diagnosed rheumatic diseases in children, such as juvenile idiopathic arthritis, systemic lupus erythematosus, systemic scleroderma, mixed connective tissue disease, juvenile dermatomyositis, juvenile spondyloarthropathy and systemic vascular disease. PMID:24669280

  3. [Chronic recurrent multifocal osteomyelitis-- I. Review].

    PubMed

    Schilling, F; Kessler, S

    2001-01-01

    Juvenile and adolescent "Chronic Recurrent Multifocal Osteomyelitis" (CRMO) is described on the basis of literature and analysis of 43 own cases (23 cases in children or adolescents). This systemic, non-purulent inflammatory disease occurs mainly metaphyseal in long bones, in pelvic bones or as spondylitis and is not as rare as it seemed. Basis of the disease is a primarily chronic, sterile, in phase of onset often monotopic (e.g. clavicle) and later frequently polytopic osteomyelitis, possibly triggered by an immuno-pathological process (e.g. Proprionibacterium acnes), and showing histologically plasmacellular invasion and a sclerosing process in different stages. Association with pustulous dermatosis (psoriasis, acne, palmo-plantar pustulosis) is found in about 25 % of children and adolescents and in more than 50 % of the adult patients. 5 differents types of distribution of osteomyelitic lesions can be found by using Te99m-bone scan primarily, of which the "pelvic type" is the most common. Because of the close neighbourhood of meta-/epiphyseal osteomyelitic focuses, "sympathetic arthritis" with synovitis is seen frequently. A therapeutic approach with azithromycine and calcitonine is presented.

  4. Chronic recurrent multifocal osteomyelitis.

    PubMed

    Roderick, Marion R; Ramanan, Athimalaipet V

    2013-01-01

    Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disease occurring primarily in children and adolescents. Episodes of systemic inflammation occur due to immune dysregulation without autoantibodies, pathogens or antigen-specific T cells. CRMO is characterised by the insidious onset of pain with swelling and tenderness over the affected bones. Clavicular involvement was the classical description; however, the metaphyses and epiphyses of long bones are frequently affected. Lesions may occur in any bone, including vertebrae. Characteristic imaging includes bone oedema, lytic areas, periosteal reaction and soft tissue reaction. Biopsies from affected areas display polymorphonuclear leucocytes with osteoclasts and necrosis in the early stages. Subsequently, lymphocytes and plasma cells predominate followed by fibrosis and signs of reactive new bone forming around the inflammation. Diagnosis is facilitated by the use of STIR MRI scanning, potentially obviating the need for biopsy and unnecessary long-term antibiotics due to incorrect diagnosis. Treatment options include non-steroidal anti-inflammatory drugs and bisphosphonates. Biologics have been tried in resistant cases with promising initial results. Gene identification has not proved easy although research in this area continues. Early descriptions of the disease suggested a benign course; however, longer-term follow up shows that it can cause significant morbidity and longer-term disability. Although it has always been thought of as very rare, the prevalence is likely to be vastly underestimated due to poor recognition of the disease.

  5. Prenatal ethanol exposure increases osteoarthritis susceptibility in female rat offspring by programming a low-functioning IGF-1 signaling pathway

    NASA Astrophysics Data System (ADS)

    Ni, Qubo; Tan, Yang; Zhang, Xianrong; Luo, Hanwen; Deng, Yu; Magdalou, Jacques; Chen, Liaobin; Wang, Hui

    2015-10-01

    Epidemiological evidence indicates that osteoarthritis (OA) and prenatal ethanol exposure (PEE) are both associated with low birth weight but possible causal interrelationships have not been investigated. To investigate the effects of PEE on the susceptibility to OA in adult rats that experienced intrauterine growth retardation (IUGR), and to explore potential intrauterine mechanisms, we established the rat model of IUGR by PEE and dexamethasone, and the female fetus and 24-week-old adult offspring subjected to strenuous running for 6 weeks were sacrificed. Knee joints were collected from fetuses and adult offspring for histochemistry, immunohistochemistry and qPCR assays. Histological analyses and the Mankin score revealed increased cartilage destruction and accelerated OA progression in adult offspring from the PEE group compared to the control group. Immunohistochemistry showed reduced expression of insulin-like growth factor-1 (IGF-1) signaling pathway components. Furthermore, fetuses in the PEE group experienced IUGR but exhibited a higher postnatal growth rate. The expression of many IGF-1 signaling components was downregulated, which coincided with reduced amounts of type II collagen in the epiphyseal cartilage of fetuses in the PEE group. These results suggest that PEE enhances the susceptibility to OA in female adult rat offspring by down-regulating IGF-1 signaling and retarding articular cartilage development.

  6. Harvest of cortico-cancellous intramedullary femoral bone graft using the Reamer-Irrigator-Aspirator (RIA).

    PubMed

    Masquelet, A-C; Benko, P E; Mathevon, H; Hannouche, D; Obert, L

    2012-04-01

    The "Reamer-Irrigator-Aspirator" (RIA) is a device that provides continuous irrigation and aspiration during intramedullary reaming of long bones. The RIA system is first used to collect the reaming material from medullary cavities, a thick paste of finely morselized osseous particles containing significantly elevated levels of stem cells and growth factors as reported by quantitative analyses. The volume of bone graft material available from an adult femur corresponds to the amount of cancellous bone graft obtained from both the anterior and posterior iliac crests. The assembly and technicalities of the RIA system require a training period to prevent any femoral fracture, which appears to be the major RIA-related complication. The elective indications for RIA bone grafting are filling of bone defects in the epiphyseal and metaphyseal regions. Diaphyseal defects may also be managed using the RIA system provided the graft is placed in a constrained system (induced membrane) to prevent dispersion of the graft into the surrounding soft tissues and is aerated with a porous material to promote its revascularization. Other RIA indications include debriding intramedullary infections and reaming for intramedullary nailing of long bone fractures to reduce the risk of fat embolization.

  7. CHANGES IN OUTLYING BONE MARROW ACCOMPANYING A LOCAL INCREASE OF TEMPERATURE WITHIN PHYSIOLOGICAL LIMITS

    PubMed Central

    Huggins, Charles; Blocksom, B. H.

    1936-01-01

    A great difference exists in the adult bone marrow of central bones as compared with outlying bones of the mammalia and avia, the distal bones being at a great disadvantage from the standpoint of blood cell production. Several experimental procedures are reported by which this disadvantage is overcome and in consequence fatty marrow of outlying bones is replaced by red marrow occurring chiefly at the epiphyseal regions, unless a low oxygen stimulus is also provided when marrow of the diaphysis becomes involved. A common factor in all of the experiments was an elevation of temperature beyond that prevailing in these distal regions, and it is felt that the evidence warrants the opinion that the cause of improvement is thermal. In some experiments, blood cell formation was increasing while the heat was adversely affecting the testis. The experiments permit construction of a general theory of fat distribution in bone marrow. In certain grafts of precartilage to other rats, normal differentiation into bone, cartilage, and marrow occurred, while in others cartilage and very small amounts of primitive marrow developed with slight, or no bone formation. Cartilage was always successfully engrafted. The capacity to form sinusoids in bone marrow is determined by the nature of the tissue rather than by the ingrowing endothelium. PMID:19870534

  8. Greater Growth of Proximal Metatarsals in Bird Embryos and the Evolution of Hallux Position in the Grasping Foot.

    PubMed

    Botelho, João Francisco; Smith-Paredes, Daniel; Soto-Acuña, Sergio; Núñez-León, Daniel; Palma, Verónica; Vargas, Alexander O

    2017-01-01

    In early theropod dinosaurs-the ancestors of birds-the hallux (digit 1) had an elevated position within the foot and had lost the proximal portion of its metatarsal. It no longer articulated with the ankle, but was attached at about mid-length of metatarsal 2 (mt2). In adult birds, the hallux is articulated closer to the distal end of mt2 at ground level with the other digits. However, on chick embryonic day 7, its position is as in early theropods at half-length of mt2. The adult distal location is acquired during embryonic days 8-10. To assess how the adult phenotype is acquired, we produced fate maps of the metatarsals of day 6 chicken embryos injecting the lipophilic tracer DiI. The fates of these marks indicate a larger expansion of the metatarsals at their proximal end, which creates the illusory effect that d1 moves distally. This larger proximal expansion occurs concomitantly with growth and early differentiation of cartilage. Histological analysis of metatarsals shows that the domains of flattened and prehypertrophic chondrocytes are larger toward the proximal end. The results suggest that the distal position of the hallux in the avian foot evolved as a consequence of an embryological period of expansion of the metatarsus toward the proximal end. It also brings attention to the developmental mechanisms leading to differential growth between epiphyses and their evolutionary consequences.

  9. Guidelines for the treatment of Crohn's disease in children.

    PubMed

    Konno, Mutsuko; Kobayashi, Akio; Tomomasa, Takeshi; Kaneko, Hiroaki; Toyoda, Shigeru; Nakazato, Yutaka; Nezu, Riichiro; Maisawa, Shun-Ichi; Miki, Kazunori

    2006-06-01

    This paper shows guidelines for the treatment of Crohn's disease in children by the Working Group of the Japanese Society for Pediatric Gastroenterology, Hepatology and Nutrition (Chair: Yuichiro Yamashiro) and the Japanese Society for Pediatric Inflammatory Bowel Disease (Chair: Akio Kobayashi). The points in which these guidelines differ from those for adult patients are as follows. (i) Total enteral nutrition in the form of an elemental formula is indicated as primary therapy for children with Crohn's disease at onset as well as the active stage. Oral mesalazine is used together. (ii) Total parenteral nutrition (TPN) with oral mesalazine is required for children with serious illness. The use of a corticosteroid should be withheld for at least 1 week after TPN has been started. (iii) When TPN is not considered to be effective, additional corticosteroid is used. Full doses of corticosteroid should be used for at least 2 weeks after clinical improvement has been achieved, and then the dose of the corticosteroid should be tapered carefully. (iv) When surgery is indicated in pediatric patients with stricture or fistula formation and complicated by persistent growth failure despite medical therapy, the optimum time for surgery is thought to be before epiphyseal plates have been closed.

  10. Sulfate in fetal development.

    PubMed

    Dawson, Paul A

    2011-08-01

    Sulfate (SO(4)(2-)) is an important nutrient for human growth and development, and is obtained from the diet and the intra-cellular metabolism of sulfur-containing amino acids, including methionine and cysteine. During pregnancy, fetal tissues have a limited capacity to produce sulfate, and rely on sulfate obtained from the maternal circulation. Sulfate enters and exits placental and fetal cells via transporters on the plasma membrane, which maintain a sufficient intracellular supply of sulfate and its universal sulfonate donor 3'-phosphoadenosine 5'-phosphosulfate (PAPS) for sulfate conjugation (sulfonation) reactions to function effectively. Sulfotransferases mediate sulfonation of numerous endogenous compounds, including proteins and steroids, which biotransforms their biological activities. In addition, sulfonation of proteoglycans is important for maintaining normal structure and development of tissues, as shown for reduced sulfonation of cartilage proteoglycans that leads to developmental dwarfism disorders and four different osteochondrodysplasias (diastrophic dysplasia, atelosteogenesis type II, achondrogenesis type IB and multiple epiphyseal dysplasia). The removal of sulfate via sulfatases is an important step in proteoglycan degradation, and defects in several sulfatases are linked to perturbed fetal bone development, including mesomelia-synostoses syndrome and chondrodysplasia punctata 1. In recent years, interest in sulfate and its role in developmental biology has expanded following the characterisation of sulfate transporters, sulfotransferases and sulfatases and their involvement in fetal growth. This review will focus on the physiological roles of sulfate in fetal development, with links to human and animal pathophysiologies.

  11. Prenatal ethanol exposure increases osteoarthritis susceptibility in female rat offspring by programming a low-functioning IGF-1 signaling pathway

    PubMed Central

    Ni, Qubo; Tan, Yang; Zhang, Xianrong; Luo, Hanwen; Deng, Yu; Magdalou, Jacques; Chen, Liaobin; Wang, Hui

    2015-01-01

    Epidemiological evidence indicates that osteoarthritis (OA) and prenatal ethanol exposure (PEE) are both associated with low birth weight but possible causal interrelationships have not been investigated. To investigate the effects of PEE on the susceptibility to OA in adult rats that experienced intrauterine growth retardation (IUGR), and to explore potential intrauterine mechanisms, we established the rat model of IUGR by PEE and dexamethasone, and the female fetus and 24-week-old adult offspring subjected to strenuous running for 6 weeks were sacrificed. Knee joints were collected from fetuses and adult offspring for histochemistry, immunohistochemistry and qPCR assays. Histological analyses and the Mankin score revealed increased cartilage destruction and accelerated OA progression in adult offspring from the PEE group compared to the control group. Immunohistochemistry showed reduced expression of insulin-like growth factor-1 (IGF-1) signaling pathway components. Furthermore, fetuses in the PEE group experienced IUGR but exhibited a higher postnatal growth rate. The expression of many IGF-1 signaling components was downregulated, which coincided with reduced amounts of type II collagen in the epiphyseal cartilage of fetuses in the PEE group. These results suggest that PEE enhances the susceptibility to OA in female adult rat offspring by down-regulating IGF-1 signaling and retarding articular cartilage development. PMID:26434683

  12. Hypoxic induction of UCP3 in the growth plate: UCP3 suppresses chondrocyte autophagy.

    PubMed

    Watanabe, Hitoshi; Bohensky, Jolene; Freeman, Theresa; Srinivas, Vickram; Shapiro, Irving M

    2008-08-01

    The overall goal of the investigation was to examine the role of uncoupling proteins (UCPs) in regulating late stage events in the chondrocyte maturation pathway. We showed for the first time that epiphyseal chondrocytes expressed UCP3. In hypoxia, UCP3 mediated regulation of the mitochondrial transmembrane potential (DeltaPsi(m)) was dependent on HIF-1alpha. We also showed for the first time that UCP3 regulated the induction of autophagy. Thus, suppression of UCP3 enhanced the expression of the autophagic phenotype, even in serum-replete media. Predictably, the mature autophagic chondrocytes were susceptible to an apoptogen challenge. Susceptibility was probably associated with a lowered expression of the anti-apoptotic proteins Bcl2 and BCL(xL) and a raised baseline expression of cytochrome c in the cytosol. These changes would serve to promote sensitivity to apoptogens. We conclude that in concert with HIF-1alpha, UCP3 regulates the activity of the mitochondrion by modulating the transmembrane potential. In addition, it inhibits induction of the autophagic response. When this occurs, it suppresses sensitivity to agents that promote chondrocyte deletion from the growth plate.

  13. Expression of matrix metalloproteinases during impairment and recovery of the avian growth plate.

    PubMed

    Dan, H; Simsa-Maziel, S; Hisdai, A; Sela-Donenfeld, D; Monsonego Ornan, E

    2009-11-01

    Tibial dyschondroplasia (TD) is a prevalent skeletal abnormality associated with rapid growth rate in many avian species. It is characterized by the presence of a nonvascularized, nonmineralized lesion that extends from the epiphyseal growth plate into the metaphysis of the proximal tibiotarsal bones. In this study, we examined the expression of 4 members of the matrix metalloproteinase (MMP) family (MMP-2, -3, -9, and -13) in thiram-induced TD lesions and in the process of recovery from TD, by in situ hybridization analysis and quantitative real-time PCR. A model for the induction and recovery of TD was established, consisting of 3 groups of broilers: (1) thiram group, chicks fed a thiram-enriched diet to induce TD; (2) recovery group, chicks fed a thiram-enriched diet during the first week of the experiment and a normal diet from the second week on; and (3) control group, chicks fed a normal diet throughout the experimental period. In agreement with our previous data, the 4 MMP were diminished in the TD lesion (P < 0.05); however, in the current study we show that the growth plate was able to repair itself and that the MMP reappeared during the process of recovery from TD. Our results strengthen the link between MMP expression and growth-plate impairment, and we suggest that gelatinase activity (MMP-2 and 9) facilitates this process.

  14. Sox5 and Sox6 are needed to develop and maintain source, columnar, and hypertrophic chondrocytes in the cartilage growth plate.

    PubMed

    Smits, Patrick; Dy, Peter; Mitra, Srijeet; Lefebvre, Véronique

    2004-03-01

    Sox5 and Sox6 encode Sry-related transcription factors that redundantly promote early chondroblast differentiation. Using mouse embryos with three or four null alleles of Sox5 and Sox6, we show that they are also essential and redundant in major steps of growth plate chondrocyte differentiation. Sox5 and Sox6 promote the development of a highly proliferating pool of chondroblasts between the epiphyses and metaphyses of future long bones. This pool is the likely cellular source of growth plates. Sox5 and Sox6 permit formation of growth plate columnar zones by keeping chondroblasts proliferating and by delaying chondrocyte prehypertrophy. They allow induction of chondrocyte hypertrophy and permit formation of prehypertrophic and hypertrophic zones by delaying chondrocyte terminal differentiation induced by ossification fronts. They act, at least in part, by down-regulating Ihh signaling, Fgfr3, and Runx2 and by up-regulating Bmp6. In conclusion, Sox5 and Sox6 are needed for the establishment of multilayered growth plates, and thereby for proper and timely development of endochondral bones.

  15. Hyperostosis frontalis interna (HFI) and castration: the case of the famous singer Farinelli (1705–1782)

    PubMed Central

    Belcastro, Maria Giovanna; Todero, Antonio; Fornaciari, Gino; Mariotti, Valentina

    2011-01-01

    The famous castrato singer Farinelli (1705–1782) was exhumed by our research group in July 2006 for the purpose of gaining some insight into his biological profile through a study of his skeletal remains. Farinelli was castrated before puberty to preserve the treble pitch of the boy's voice into adult life. His powerful and sweet voice became legendary. In spite of its bad preservation state, the skeleton displayed some interesting characteristics that are probably related to the effects of castration, including long limb-bones, persistence of epiphyseal lines and osteoporosis. In particular, the frontal bone was affected by severe hyperostosis frontalis interna (HFI). This condition consists in a symmetrical thickening of the inner table of the bone. The epidemiology of HFI shows that it is relatively common in postmenopausal women but very rare in men. Men affected by this pathology suffer from diseases, syndromes or treatments causing androgen deficiency. In the case of Farinelli, castration was probably responsible for the onset and development of this lesion. PMID:21740437

  16. Significant effects of mild endogenous hormonal changes in humans: considerations for low-dose testing.

    PubMed

    Brucker-Davis, F; Thayer, K; Colborn, T

    2001-03-01

    We review the significant and adverse health effects that can occur with relatively small endogenous hormonal changes in pubertal and adult humans. We discuss the effects of hormonal changes that occur within normal physiologic ranges--such as the rising levels of estrogen in peripuberty, which cause growth spurts at low levels and then the fusion of epiphyses at higher levels--and the hormonal variations during the menstrual cycle and their relation to genital phenotypic changes and intercurrent disease evolution. We turn next to adaptive changes in gonadal and other functions during aging, exercise, stress, starvation, and chronic diseases, which can serve as models for the effects of exogenous, hormonally active compounds. Then we review the states of borderline hormonal imbalances such as subclinical (having few or very mild symptoms, if any) hypothyroidism or hyperthyroidism, glucose intolerance, and other endocrine conditions. Finally, we review the deleterious systemic effects of gonadal imbalance. Information stemming from clinical observations leads to the concept of "no threshold" within the endocrine system and thus illustrates the importance of considering low-dose testing for chemicals that interfere with hormonal activity. We also urge attention to more sensitive, less visible end points such as osteoporosis, increased risk for cardiovascular disease, or cognitive changes.

  17. Identification of a novel COL2A1 mutation (c.1744G>A) in a Japanese family: a case report

    PubMed Central

    2014-01-01

    Introduction Mutations in the gene encoding the type II collagen gene (COL2A1) have been found to affect the entire skeletal system. Recently, inheritable skeletal dysplasia caused by novel COL2A1 mutations has been linked to an inherited disease of the hip joint that neither involves the entire skeletal system nor is characterized by the presence of concomitant disorders, such as spinal or ocular abnormalities. Case presentation A 27-year-old Japanese woman previously diagnosed with avasucular necrosis (AVN) of the femoral head on the basis of radiological findings was referred to the study site for surgical management of a painful hip joint. She had no history of disease but suffered from bilateral hip joint lesions. Analysis of her pedigree revealed that bilateral hip joint lesions affected more than three generations of her family. Based on these findings, haplotype analysis of her and her family members was performed by examining select candidate genes from the critical interval for epiphyseal dysplasia of the femoral head on 12q13 and sequencing the promoter and exonic regions of COL2A1. Conclusion A novel COL2A1 mutation (c.1744G>A) was identified within one Japanese family. PMID:25124518

  18. Achondrogenesis type IB: agenesis of cartilage interterritorial matrix as the link between gene defect and pathological skeletal phenotype.

    PubMed

    Corsi, A; Riminucci, M; Fisher, L W; Bianco, P

    2001-10-01

    Achondrogenesis type IB is a lethal osteochondrodysplasia caused by mutations in the diastrophic dysplasia sulfate transporter gene. How these mutations lead to the skeletal phenotype is not known. Histology of plastic-embedded skeletal fetal achondrogenesis type IB samples suggested that interterritorial epiphyseal cartilage matrix was selectively missing. Cartilage was organized in "chondrons" separated by cleft spaces; chondrocyte seriation, longitudinal septa, and, in turn, mineralized cartilaginous septa were absent. Agenesis of interterritorial matrix as the key histologic change was confirmed by immunohistology using specific markers of territorial and interterritorial matrix. Biglycan-enriched territorial matrix was preserved; decorin-enriched interterritorial areas were absent, although immunostaining was observed within chondrocytes. Thus, in achondrogenesis type IB: (1) a complex derangement in cartilage matrix assembly lies downstream of the deficient sulfate transporter activity; (2) the severely impaired decorin deposition participates in the changes in matrix organization with lack of development of normal interterritorial matrix; and (3) this change determines the lack of the necessary structural substrate for proper endochondral bone formation and explains the severe skeletal phenotype.

  19. Mutations Preventing Regulated Exon Skipping in MET Cause Osteofibrous Dysplasia

    PubMed Central

    Gray, Mary J.; Kannu, Peter; Sharma, Swarkar; Neyt, Christine; Zhang, Dongping; Paria, Nandina; Daniel, Philip B.; Whetstone, Heather; Sprenger, Hans-Georg; Hammerschmidt, Philipp; Weng, Angela; Dupuis, Lucie; Jobling, Rebekah; Mendoza-Londono, Roberto; Dray, Michael; Su, Peiqiang; Wilson, Megan J.; Kapur, Raj P.; McCarthy, Edward F.; Alman, Benjamin A.; Howard, Andrew; Somers, Gino R.; Marshall, Christian R.; Manners, Simon; Flanagan, Adrienne M.; Rathjen, Karl E.; Karol, Lori A.; Crawford, Haemish; Markie, David M.; Rios, Jonathan J.; Wise, Carol A.; Robertson, Stephen P.

    2015-01-01

    The periosteum contributes to bone repair and maintenance of cortical bone mass. In contrast to the understanding of bone development within the epiphyseal growth plate, factors that regulate periosteal osteogenesis have not been studied as intensively. Osteofibrous dysplasia (OFD) is a congenital disorder of osteogenesis and is typically sporadic and characterized by radiolucent lesions affecting the cortical bone immediately under the periosteum of the tibia and fibula. We identified germline mutations in MET, encoding a receptor tyrosine kinase, that segregate with an autosomal-dominant form of OFD in three families and a mutation in a fourth affected subject from a simplex family and with bilateral disease. Mutations identified in all families with dominant inheritance and in the one simplex subject with bilateral disease abolished the splice inclusion of exon 14 in MET transcripts, which resulted in a MET receptor (METΔ14) lacking a cytoplasmic juxtamembrane domain. Splice exclusion of this domain occurs during normal embryonic development, and forced induction of this exon-exclusion event retarded osteoblastic differentiation in vitro and inhibited bone-matrix mineralization. In an additional subject with unilateral OFD, we identified a somatic MET mutation, also affecting exon 14, that substituted a tyrosine residue critical for MET receptor turnover and, as in the case of the METΔ14 mutations, had a stabilizing effect on the mature protein. Taken together, these data show that aberrant MET regulation via the juxtamembrane domain subverts core MET receptor functions that regulate osteogenesis within cortical diaphyseal bone. PMID:26637977

  20. Progressive Pseudorheumatoid Dysplasia or JIA?

    PubMed Central

    2017-01-01

    Progressive pseudorheumatoid dysplasia (PPD) or spondyloepiphyseal dysplasia tarda with progressive arthropathy (SEDT-PA) is a rare arthropathy of childhood involving the axial skeleton as well as small peripheral joints. A 10-year-old boy was referred by a general practitioner with pain and deformity in the fingers of hands and limping gait. There was no joint synovitis although the finger joints were bulky on examination with mild flexion deformity. Patient had exaggerated kyphosis and lumbar lordosis with pigeon chest and restricted hip joint movements. Anteroposterior X-rays of the hip joints revealed widened and flattened epiphyses of the femoral heads with narrow and irregular joint spaces. Hand X-rays revealed periarticular osteopenia, significant narrowing of the joint spaces of proximal interphalangeal, and distal interphalangeal joints, together with osseous enlargement of the basis of metacarpal bones and phalanges. Spinal X-rays revealed generalized platyspondyly and anterior beaking of vertebral bodies. There was a clear mega os trigonum in his feet images. All blood investigations were normal with no evidence of inflammation and thyroid hormone levels were normal. The diagnosis of PPD was favored by imaging studies and normal inflammatory markers and the patient was treated with physiotherapy, family counseling, and anti-inflammatory medications. PMID:28316857

  1. Burnt bone assemblages from El Esquilleu cave (Cantabria, Northern Spain): deliberate use for fuel or systematic disposal of organic waste?

    NASA Astrophysics Data System (ADS)

    Yravedra, J.; Uzquiano, P.

    2013-05-01

    Bones or fossil fuels associated with combustion structures have been widely discussed in several works related to Neanderthal lifestyles and subsistence patterns during the MIS 3. El Esquilleu cave (western Cantabria, Spain) can significantly contribute to this issue, particularly with the taphonomic study of layers 21 and 23, which are characterized by the presence of hearths containing abundant burnt and charred faunal remains of ibex. The fragmentation and burning rates as well as bone presence within hearths may suggest that they were used as a supplementary fuel resource. Following previous research on the suitability of bones as a supplement to firewood in hearth combustions, a series of experiments are here presented using goat bones, in consistency with the faunal record present at El Esquilleu. Our experiments proved that small-sized animal (<100 kg in weight) bones also possess appropriate qualities for their use as fuel, particularly epiphyseal and axial parts. This paper critically evaluates whether bones could have been used as fuel by the Neanderthal groups at El Esquilleu or whether their combustion resulted from other behavioural practises. In this sense, we compare our results with different proxy data from this site as well as with the palaeoenvironmental information available for the MIS 3 chronological period in Western Europe.

  2. A systematic review of selected musculoskeletal late effects in survivors of childhood cancer.

    PubMed

    Gawade, Prasad L; Hudson, Melissa M; Kaste, Sue C; Neglia, Joseph P; Wasilewski-Masker, Karen; Constine, Louis S; Robison, Leslie L; Ness, Kirsten K

    2014-01-01

    Survivors of childhood cancer are at risk for treatment-related musculoskeletal late effects. Early detection and orthopedic intervention can help ameliorate musculoskeletal late effects and prevent subsequent complications. This systematic review summarizes the literature describing associations between cancer, its treatment, and musculoskeletal late effects. We searched PubMed and Web of Science for English language articles published between January 1970 and December 2012. The search was limited to investigations with at least 15 participants and conducted at least 2 years after completion of therapy for childhood, adolescent, or young adult cancer. Some late skeletal effects, including low bone mineral density, osteonecrosis, slipped capital femoral epiphyses, oncogenic rickets, and hormonerelated growth disturbances have been previously reviewed and were excluded, as were outcomes following amputation and limb-salvage procedures. Of 2347 references identified, 30 met inclusion criteria and were retained. An additional 54 studies that met inclusion criteria were found in reference lists of retained studies. Of 84 studies, 60 focused on associations between radiotherapy, six between chemotherapy, and 18 between surgery and musculoskeletal late effects. We found that younger age, higher radiation dosage, and asymmetric or partial bone radiation volume influences the effects of radiation on the musculoskeletal system. Methotrexate and vincristine are associated with long-term muscular strength and flexibility deficits. Laminectomy and chest wall resection are associated with spinal malalignment, and enucleation is associated with orbital deformities among survivors. Radiotherapy, chemotherapy, and surgery are associated with musculoskeletal late effects independently and additively. Associations are additionally influenced by host and treatment characteristics.

  3. Long bone histology of the stem salamander Kokartus honorarius (Amphibia: Caudata) from the Middle Jurassic of Kyrgyzstan.

    PubMed

    Skutschas, Pavel; Stein, Koen

    2015-04-01

    Kokartus honorarius from the Middle Jurassic (Bathonian) of Kyrgyzstan is one of the oldest salamanders in the fossil record, characterized by a mixture of plesiomorphic morphological features and characters shared with crown-group salamanders. Here we present a detailed histological analysis of its long bones. The analysis of a growth series demonstrates a significant histological maturation during ontogeny, expressed by the progressive appearance of longitudinally oriented primary vascular canals, primary osteons, growth marks, remodelling features in primary bone tissues, as well as progressive resorption of the calcified cartilage, formation of endochondral bone and development of cartilaginous to bony trabeculae in the epiphyses. Apart from the presence of secondary osteons, the long bone histology of Kokartus is very similar to that of miniaturized temnospondyls, other Jurassic stem salamanders, miniaturized seymouriamorphs and modern crown-group salamanders. We propose that the presence of secondary osteons in Kokartus honorarius is a plesiomorphic feature, and the loss of secondary osteons in the long bones of crown-group salamanders as well as in those of miniaturized temnospondyls is the result of miniaturization processes. Hitherto, all stem salamander long bong histology (Kokartus, Marmorerpeton and 'salamander A') has been generally described as having paedomorphic features (i.e. the presence of Katschenko's Line and a layer of calcified cartilage), these taxa were thus most likely neotenic forms. The absence of clear lines of arrested growth and annuli in long bones of Kokartus honorarius suggests that the animals lived in an environment with stable local conditions.

  4. Relationship of sup(99m)Tc-MDP uptake to regional osseous circulation in skeletally immature and mature dogs

    SciTech Connect

    McKinstry, P.; Schnitzer, J.E.; Light, T.R.; Ogden, J.A.; Hoffer, P.

    1982-05-01

    Uptake of intravenously injected sup(99m)Tc-MDP in multiple regions of healthy skeletally immature and mature dogs was correlated with regional chondro-osseous blood flow determined by radioactively labeled microspheres. Compared to the microsphere distribution, the distribution of sup(99m)Tc-MDP indicated that blood flow is an important, but not exclusive, factor in the uptake of bone-seeking tracers. Other factors such as the affinity of the tracer for the various types of chondro-osseous tissues must also affect tracer uptake. A measure of the relative affinity of sup(99m)Tc-MDP for bone was derived by the ratio of the percentage of tracer uptake to the percentage of blood flow. The juxta-ephyseal region demonstrated the greatest affinity for the tracer, followed in decreasing affinity, by the cortical bone, epiphyseal cartilage, trabecular bone of the metaphysis and secondary ossification center, and marrow space. Within the spongiosa, affinity generally increased as the proportion of osseous trabeculae relative to marrow space increased. sup(99m)Tc-MDP uptake is disproportionately increased in areas of active bone growth and remodeling where the surface area of hydroxyapatite crystals available for adsorption is probably highest.

  5. Matrilin-3 mutations that cause chondrodysplasias interfere with protein trafficking while a mutation associated with hand osteoarthritis does not

    PubMed Central

    Otten, C; Wagener, R; Paulsson, M; Zaucke, F

    2005-01-01

    Several mutations in the extracellular matrix protein matrilin-3 cause a heterogeneous disease spectrum affecting skeletal tissues. We introduced three disease causing point mutations leading to single amino acid exchanges (R116W, T298M, C299S) in matrilin-3 and expressed the corresponding proteins in primary articular chondrocytes to elucidate pathogenic mechanisms at the cellular level. Expression levels, processing, and the secretion pattern of a mutation linked to hand osteoarthritis (T298M) were similar to the wildtype protein, whereas the two other mutants were poorly expressed and hardly detectable in supernatants of transiently transfected cells. Using immunofluorescence staining, we demonstrated that mutants R116W and C299S are retained and accumulate within the endoplasmatic reticulum (ER). Their further trafficking to the Golgi compartment seems to be disturbed, whereas T298M is secreted normally. In cells transfected with the wildtype and T298M constructs, a matrilin-3 containing filamentous network was formed surrounding the cells, whereas in the case of R116W and C299S such structures were completely absent. These observations are similar to those for mutations in the cartilage oligomeric matrix protein (COMP) leading to multiple epiphyseal dysplasia and pseudoachondroplasia suggesting that retention and accumulation of cartilage proteins in the ER might be a general mechanism involved in the pathogenesis of chondrodysplasias. PMID:16199550

  6. Leptin increases growth of primary ossification centers in fetal mice.

    PubMed

    Bertoni, Laura; Ferretti, Marzia; Cavani, Francesco; Zavatti, Manuela; Resca, Elisa; Benelli, Augusta; Palumbo, Carla

    2009-11-01

    The effect of peripheral leptin on fetal primary ossification centers during the early phases of bone histogenesis was investigated by administration of leptin to pregnant mice. Fourteen pregnant mice were divided into two groups. The treated pregnant group was subcutaneously injected in the intrascapular region with supraphysiologic doses (2 mg kg(-1)) of leptin (Vinci Biochem, Firenze, Italy) in a volume of 0.1 mL per 10 g body weight, at the 7th, 9th and 11th day of gestation. The control group was treated with physiological solution in the same manner and same times as the treated group. The new-born mice were killed 1 day after birth and the primary ossification centers were stained with Alizarin Red S after diaphanizing the soft tissues in 1% potassium hydroxide. The development of both endochondral and intramembranous ossification centers was morphometrically analysed in long bones. The results showed that the ossification centers of mice born by mothers treated with leptin grow more rapidly in both length and cross-sectional area compared with mice born by the untreated mothers. As the development of long bones depends on endochondral ossification occurring at proximal and distal epiphyseal plates as well as on intramembranous ossification along the periosteal surface, it appears that leptin activates the differentiation and proliferation of both chondrocytes and osteoblasts. The role of leptin as a growth factor of cartilage and bone is discussed in the light of the data reported in the literature.

  7. Periosteal entrapment in distal femoral physeal fractures: harbinger for premature physeal arrest ?

    PubMed

    Segal, Lee S; Shrader, M Wade

    2011-10-01

    We report on two patients who sustained Salter-Harris II fractures of the distal femur with physeal widening after being tackled in football games. Preoperative MRI indicated entrapped periosteum at the physeal fracture site for both patients. Both patients underwent open reduction of the physeal fracture with removal of the entrapped periosteum and achieving an anatomic reduction. Follow-up MRI's revealed premature physeal arrest. Subsequent procedures were performed to address sequelae of premature physeal arrest. The presence of physeal widening and entrapped periosteum may reflect high-energy trauma to the physis. This can result in injury to both the epiphyseal blood supply and to the physeal cartilage (germinal zone) resulting in physeal arrest despite anatomic reduction after removal of the entrapped periosteum. Upon literature review, pre-operative MRI demonstrating entrapped periosteum has not been previously reported. We hypothesize that the presence of entrapped periosteum following distal femoral physeal fractures may be associated with an increased risk for premature physeal arrest.

  8. Post-traumatic caspase-3 expression in the adjacent areas of growth plate injury site: a morphological study.

    PubMed

    Musumeci, Giuseppe; Castrogiovanni, Paola; Loreto, Carla; Castorina, Sergio; Pichler, Karin; Weinberg, Annelie Martina

    2013-07-29

    The epiphyseal plate is a hyaline cartilage plate that sits between the diaphysis and the epiphysis. The objective of this study was to determine the impact of an injury in the growth plate chondrocytes through the study of histological morphology, immunohistochemistry, histomorphometry and Western Blot analyses of the caspase-3 and cleaved PARP-1, and levels of the inflammatory cytokines, Interleukin-6 (IL-6) and Tumor Necrosis Factor alpha (TNF-α), in order to acquire more information about post-injury reactions of physeal cell turnover. In our results, morphological analysis showed that in experimental bones, neo-formed bone trabeculae-resulting from bone formation repair-invaded the growth plate and reached the metaphyseal bone tissue (bone bridge), and this could result in some growth arrest. We demonstrated, by ELISA, increased expression levels of the inflammatory cytokines IL-6 and TNF-α. Immunohistochemistry, histomorphometry and Western Blot analyses of the caspase-3 and cleaved PARP-1 showed that the physeal apoptosis rate of the experimental bones was significantly higher than that of the control ones. In conclusion, we could assume that the inflammation process causes stress to chondrocytes that will die as a biological defense mechanism, and will also increase the survival of new chondrocytes for maintaining cell homeostasis. Nevertheless, the exact stimulus leading to the increased apoptosis rate, observed after injury, needs additional research to understand the possible contribution of chondrocyte apoptosis to growth disturbance.

  9. A NURBS-based technique for subject-specific construction of knee bone geometry.

    PubMed

    Au, Anthony G; Palathinkal, Darren; Liggins, Adrian B; Raso, V James; Carey, Jason; Lambert, Robert G; Amirfazli, A

    2008-10-01

    Subject-specific finite element (FE) models of bones that form the knee joint require rapid and accurate geometry construction. The present study introduces a semi-automatic non-uniform rational B-spline (NURBS) technique to construct knee bone geometries from computed tomography (CT) images using a combination of edge extraction and CAD surface generation. In particular, this technique accurately constructs endosteal surfaces and can accommodate thin cortical bone by estimating the cortical thickness from well-defined surrounding bone. A procedure is also introduced to overcome the bifurcation at the femoral condyles during surface generation by combining transverse and sagittal plane CT data. Available voxel- and NURBS-based subject-specific construction techniques accurately capture periosteal surfaces but are limited in their ability to capture endosteal geometry. In this study, the proposed NURBS-based technique and a typical voxel mesh technique captured periosteal surfaces within an order of magnitude of image resolution. The endosteum of diaphyseal bone was also captured with similar accuracy by both techniques. However, the voxel mesh model failed to accurately capture the metaphyseal and epiphyseal endosteum due to the poor CT contrast of thin cortical bone, resulting in gross overestimation of cortical thickness. The proposed technique considered both the local and global nature of CT images to arrive at a description of cortical bone thickness accurate to within 2 pixel lengths.

  10. Resistance to thyroid hormone due to defective thyroid receptor alpha

    PubMed Central

    Moran, Carla; Chatterjee, Krishna

    2015-01-01

    Thyroid hormones act via nuclear receptors (TRα1, TRβ1, TRβ2) with differing tissue distribution; the role of α2 protein, derived from the same gene locus as TRα1, is unclear. Resistance to thyroid hormone alpha (RTHα) is characterised by tissue-specific hypothyroidism associated with near-normal thyroid function tests. Clinical features include dysmorphic facies, skeletal dysplasia (macrocephaly, epiphyseal dysgenesis), growth retardation, constipation, dyspraxia and intellectual deficit. Biochemical abnormalities include low/low-normal T4 and high/high-normal T3 concentrations, a subnormal T4/T3 ratio, variably reduced reverse T3, raised muscle creatine kinase and mild anaemia. The disorder is mediated by heterozygous, loss-of-function, mutations involving either TRα1 alone or both TRα1 and α2, with no discernible phenotype attributable to defective α2. Whole exome sequencing and diagnostic biomarkers may enable greater ascertainment of RTHα, which is important as thyroxine therapy reverses some metabolic abnormalities and improves growth, constipation, dyspraxia and wellbeing. The genetic and phenotypic heterogeneity of RTHα and its optimal management remain to be elucidated. PMID:26303090

  11. Post-Traumatic Caspase-3 Expression in the Adjacent Areas of Growth Plate Injury Site: A Morphological Study

    PubMed Central

    Musumeci, Giuseppe; Castrogiovanni, Paola; Loreto, Carla; Castorina, Sergio; Pichler, Karin; Weinberg, Annelie Martina

    2013-01-01

    The epiphyseal plate is a hyaline cartilage plate that sits between the diaphysis and the epiphysis. The objective of this study was to determine the impact of an injury in the growth plate chondrocytes through the study of histological morphology, immunohistochemistry, histomorphometry and Western Blot analyses of the caspase-3 and cleaved PARP-1, and levels of the inflammatory cytokines, Interleukin-6 (IL-6) and Tumor Necrosis Factor alpha (TNF-α), in order to acquire more information about post-injury reactions of physeal cell turnover. In our results, morphological analysis showed that in experimental bones, neo-formed bone trabeculae—resulting from bone formation repair—invaded the growth plate and reached the metaphyseal bone tissue (bone bridge), and this could result in some growth arrest. We demonstrated, by ELISA, increased expression levels of the inflammatory cytokines IL-6 and TNF-α. Immunohistochemistry, histomorphometry and Western Blot analyses of the caspase-3 and cleaved PARP-1 showed that the physeal apoptosis rate of the experimental bones was significantly higher than that of the control ones. In conclusion, we could assume that the inflammation process causes stress to chondrocytes that will die as a biological defense mechanism, and will also increase the survival of new chondrocytes for maintaining cell homeostasis. Nevertheless, the exact stimulus leading to the increased apoptosis rate, observed after injury, needs additional research to understand the possible contribution of chondrocyte apoptosis to growth disturbance. PMID:23899790

  12. One carbon metabolism and bone homeostasis and remodeling: A review of experimental research and population studies.

    PubMed

    Feigerlova, Eva; Demarquet, Lea; Guéant, Jean-Louis

    2016-07-01

    Homocysteine (HCY) is a degradation product of the methionine pathway. The B vitamins, in particular vitamin B12 and folate, are the primary nutritional determinant of HCY levels and therefore their deficiencies result in hyperhomocysteinaemia (HHCY). Prevalence of hyperhomocysteinemia (HHCY) and related dietary deficiencies in B vitamins and folate increase with age and have been related to osteoporosis and abnormal development of epiphyseal cartilage and bone in rodents. Here we provide a review of experimental and population studies. The negative effects of HHCY and/or B vitamins and folate deficiencies on bone formation and remodeling are documented by cell models, including primary osteoblasts, osteoclast and bone progenitor cells as well as by animal and human studies. However, underlying pathophysiological mechanisms are complex and remain poorly understood. Whether these associations are the direct consequences of impaired one carbon metabolism is not clarified and more studies are still needed to translate these findings to human population. To date, the evidence is limited and somewhat conflicting, however further trials in groups most vulnerable to impaired one carbon metabolism are required.

  13. Nutrition-induced catch-up growth at the growth plate.

    PubMed

    Gat-Yablonski, G; Shtaif, B; Abraham, E; Phillip, M

    2008-09-01

    The effect of 40% food restriction (FR) and replenishment on the growth hormone (GH) and insulin-like growth factor-I (IGF-I) axis in the epiphyseal growth plate (EGP) was examined in a mouse model. Changes in RNA and protein levels were evaluated with real time PCR and immunohistochemistry, respectively, and serum levels of IGF-I and leptin were measured with radioimmunoassay. Dramatic changes in weight, tibial length and EGP height were observed following 10 days of 40% FR. The protein levels of IGF-I receptor (IGF-IR) and GH receptor (GHR), which were reduced during FR, increased during catch-up growth without an apparent change in the level of their RNA. The levels of type II and X collagens were unchanged. Serum IGF-I and leptin levels were reduced during FR and increased during catch-up growth. Following 40% FR, there was a significant decrease in the level of GHR and IGF-IR in the EGP which may explain the reduced effect of GH treatment in malnourished animals and children.

  14. Humeral lengthening by distraction osteogenesis: a safe procedure?

    PubMed

    Ruette, Peter; Lammens, Johan

    2013-12-01

    This study was conducted to assess the safety of humeral lengthening using an Ilizarov frame. We retrospectively reviewed 26 humeral segments in 17 patients that were lengthened at our department between 1993 and 2011. There were varying aetiologies including achondroplasia, epiphyseal dysplasia, Oilier disease, trauma or infection of the proximal humeral growth-plate, unicameral bone cyst and brachial plexus injury. Mean age at start of surgery was 17.05 years (range : 5-40). The mean lengthening achieved was 8.85 (3-13) cm. Mean lengthening percentage was 353% (range : 10-48). Average healing index was 30.56 days/cm (range : 17.46-4232). There was a significant difference in healing index between achondroplasia patients (28.79 days/cm) compared to others (33.41 days/cm). Minor problems included pin tract infection (14 segments). More important obstacles were temporary elbow flexion contracture (7 segments), premature consolidation (6 segments), radial nerve dysaesthesia (6 segments) and loosening of a Schanz screw (1 segment). Complications included one fracture and one progressive bowing after frame removal. One planned lengthening was not completely achieved. Despite a lot of obstacles, humeral lengthening using an Ilizarov frame provided a reliable method to treat the functional or cosmetic problems of upper limb shortening.

  15. Surgical approach to bone healing in osteoporosis

    PubMed Central

    Pesce, Vito; Speciale, Domenico; Sammarco, Giulio; Patella, Silvio; Spinarelli, Antonio; Patella, Vittorio

    2009-01-01

    Osteoporotic fractures represent one of the most common cause of disability and one of the major voice in the health economic budget in many countries of the world. Fragility fractures are especially meta-epiphyseal fractures, in skeletal sites with particular biomechanic characteristic (hip, vertebrae), complex and with more fragments, with slow healing process (mineralization and remodeling) and co-morbidity. The healing of a fracture in osteoporotic bone passes through the normal stages and concludes with union of the fracture although the healing process is prolonged. Fractures in the elderly osteoporotic patients represent a challenge to the orthopaedic surgeons. Osteoporosis does not only increase the risk of fracture but also represents a problem in osteofixation of fractures in fracture treatment. The major technical problem that surgeons face, is the difficulty to obtain a stable fixation of an implant due to osteoporotic bone. The load transmitted at the bone-implant interface can often exceed the reduced strain tolerance of osteoporotic bone. In the treatment of osteoporotic fractures it is important to consider different aspects: general conditions of elderly patient and comorbidity, the reduced muscular and bone mass and the increased bone fragility, structural modifications as medullary expansion. The aim of surgical treatment is to obtain a stable fixation that reduces pain and permits an early mobilization. PMID:22461162

  16. Genetics of gigantism and acromegaly.

    PubMed

    Hannah-Shmouni, Fady; Trivellin, Giampaolo; Stratakis, Constantine A

    Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Over the past two decades, our increasing understanding of the molecular and genetic etiologies of pituitary gigantism and acromegaly yielded several genetic causes, including multiple endocrine neoplasia type 1 and 4, McCune-Albright syndrome, Carney complex, familial isolated pituitary adenoma, pituitary adenoma association due to defects in familial succinate dehydrogenase genes, and the recently identified X-linked acrogigantism. The early diagnosis of these conditions helps guide early intervention, screening, and genetic counseling of patients and their family members. In this review, we provide a concise and up-to-date discussion on the genetics of gigantism and acromegaly.

  17. Autosomal recessive Stickler syndrome due to a loss of function mutation in the COL9A3 gene.

    PubMed

    Faletra, Flavio; D'Adamo, Adamo P; Bruno, Irene; Athanasakis, Emmanouil; Biskup, Saskia; Esposito, Laura; Gasparini, Paolo

    2014-01-01

    Stickler syndrome (STL) is a clinically variable and genetically heterogeneous syndrome characterized by ophthalmic, articular, orofacial, and auditory manifestations. STL has been described with both autosomal dominant and recessive inheritance. The dominant form is caused by mutations of COL2A1 (STL 1, OMIM 108300), COL11A1 (STL 2, OMIM 604841), and COL11A2 (STL 3, OMIM 184840) genes, while recessive forms have been associated with mutations of COL9A1 (OMIM 120210) and COL9A2 (OMIM 120260) genes. Type IX collagen is a heterotrimeric molecule formed by three genetically distinct chains: α1, α2, and α3 encoded by the COL9A1, COL9A2, and COL9A3 genes. Up to this time, only heterozygous mutations of COL9A3 gene have been reported in human and related to: (1) multiple epiphyseal dysplasia type 3, (2) susceptibility to an intervertebral disc disease, and (3) hearing loss. Here, we describe the first autosomal recessive Stickler family due to loss of function mutations (c.1176_1198del, p.Gln393Cysfs*25) of COL9A3 gene. These findings extend further the role of collagen genes family in the disease pathogenesis.

  18. PCR assay confirms diagnosis in syndrome with variably expressed phenotype: mutation detection in Stickler syndrome.

    PubMed Central

    Ahmad, N N; McDonald-McGinn, D M; Dixon, P; Zackai, E H; Tasman, W S

    1996-01-01

    Stickler syndrome is an autosomal dominant disease with ocular (severe myopia, vitreal degeneration, and retinal detachment) and other systemic manifestations (hearing loss, cleft palate, epiphyseal dysplasia, and premature osteoarthritis). As with other dominantly inherited conditions, the clinical phenotype of Stickler syndrome varies considerably. To date, all mutations have been located in the type II procollagen (COL2A1) gene. Analysis of a C-->T mutation we had identified previously, in COL2A1 gene in exon 40, in a three generation pedigree showed the loss of a cleavage site for the TaqI restriction enzyme. We designed a rapid PCR based restriction enzyme assay to detect this mutation and used it to establish the diagnosis in a neonate from the same pedigree, presenting with the first occurrence of the Pierre-Robin sequence in the family and minimal ocular findings. These results underline the potential diagnostic value of many as yet undetected DNA mutations in families affected with Stickler syndrome, since the variability of the phenotype can impede accurate diagnosis, appropriate genetic counselling, and effective intervention and prophylactic treatment for affected people. Images PMID:8863161

  19. Inactivation of Fam20B in Joint Cartilage Leads to Chondrosarcoma and Postnatal Ossification Defects

    PubMed Central

    Ma, Pan; Yan, Wenjuan; Tian, Ye; Wang, Jingya; Feng, Jian Q.; Qin, Chunlin; Cheng, Yi-Shing Lisa; Wang, Xiaofang

    2016-01-01

    During endochondral ossification, chondrocytes embed themselves in a proteoglycan-rich matrix during the proliferation-maturation transition. Accumulating evidence shows that proteoglycans are essential components for chondrocyte proliferation and differentiation. When we conditionally inactivated FAM20B (Family with sequence similarity 20 member-B), which is a newly identified xylose kinase essential for glycosaminoglycan (GAG) formation on the protein core of proteoglycans, from the dental mesenchyme using Osr2-Cre, which is also strongly expressed in joint cartilage, we found chondrosarcoma in the knee joint and remarkable defects of postnatal ossification in the long bones. Mechanistic analysis revealed that the defects were associated with gain of function in multiple signaling pathways in the epiphyseal chondrocytes, such as those derived by WNT, BMP, and PTHrP/IHH molecules, suggesting that the FAM20B-catalyzed proteoglycans are critical mediators for a signaling balance in the regulatory network controlling chondrocyte differentiation and proliferation. In particular, we demonstrated that the WNT inhibitor was able to rescue part of the bone defects in Osr2-Cre;Fam20Bfl/fl mice, indicating that FAM20B-catalyzed proteoglycans regulate postnatal endochondral ossification partially through the mediation of WNT signaling. PMID:27405802

  20. Skeletal manifestations of scurvy: a case report from dubai.

    PubMed

    Noordin, Shahryar; Baloch, Naveed; Salat, Muhammad Sohail; Rashid Memon, Abdul; Ahmad, Tashfeen

    2012-01-01

    Introduction. Nutritional deficiencies are rarely reported in developed countries. We report a child of Pakistani origin brought up in Dubai who developed skeletal manifestations of scurvy due to peculiar dietary habits. Case Presentation. A 4.5 year old boy presented with pain and swelling of multiple joints for three months and inability to walk for two months. Dietary history was significant for exclusive meat intake for the preceding two years. On examination the child's height and weight were below the 5th percentile for his age. He was pale and tachycardic. There was significant swelling and tenderness over the wrist, knee and ankle joints, along with painful restriction of motion. Basic blood workup was unremarkable except for anemia. However, X-rays showed delayed bone age, severe osteopenia of the long bones, epiphyseal separation, cortical thinning and dense zone of provisional calcification, suggesting a radiological diagnosis of scurvy. The child was started on vitamin C replacement therapy. Over the following two months, the pain and swelling substantially reduced and the child became able to walk. Repeat X-rays showed improvement in the bony abnormalities. Conclusion. Although scurvy is not a very commonly encountered entity in the modern era, inappropriate dietary intake can lead to skeletal abnormalities which may be confused with rickets. A high index of suspicion is thus required for prompt diagnosis of scurvy in patients with bone and joint symptoms.

  1. Chondroblastoma of the Clivus: Case Report and Review

    PubMed Central

    Liu, Jonathan; Ahmadpour, Arjang; Bewley, Arnaud F.; Lechpammer, Mirna; Bobinski, Matthew; Shahlaie, Kiarash

    2015-01-01

    Background and Importance Chondroblastoma is a benign primary bone tumor that typically develops in the epiphyses of long bones. Chondroblastoma of the craniofacial skeleton is extremely rare, with most cases occurring in the squamosal portion of the temporal bone. In this report, we describe the first case of chondroblastoma of the clivus presenting with cranial neuropathy that was treated with endoscopic endonasal resection. We review the literature on craniofacial chondroblastomas with particular emphasis on extratemporal lesions. Case Presentation A 27-year-old woman presented with severe headache, left facial dysesthesias, and diplopia. Physical examination revealed hypesthesia in the left maxillary nerve dermatome, and complete left abducens nerve palsy. Imaging demonstrated an expansile intraosseous mass originating in the upper clivus with extension superiorly into the sella turcica and laterally to involve the medial wall of the left cavernous sinus. The tumor was completely resected via an endoscopic endonasal approach, with postoperative improvement in lateral gaze palsy. Histopathology was consistent with chondroblastoma. Conclusion Chondroblastoma is a rare tumor of the craniofacial skeleton that should be included in the differential diagnosis of an osteolytic lesion of the clivus. Complete surgical resection remains the mainstay of treatment. PMID:26623238

  2. A unique point mutation in the fibroblast growth factor receptor 3 gene (FGFR3) defines a new craniosynostosis syndrome

    PubMed Central

    Muenke, M.; Gripp, K. W.; McDonald-McGinn, D. M.; Gaudenz, K.; Whitaker, L. A.; Bartlett, S. P.; Markowitz, R. I.; Robin, N. H.; Nwokoro, N.; Mulvihill, J. J.; Losken, H. W.; Mulliken, J. B.; Guttmacher, A. E.; Wilroy, R. S.; Clarke, L. A.; Hollway, G.; Adès, L. C.; Haan, E. A.; Mulley, J. C.; Cohen, M. M.; Bellus, G. A.; Francomano, C. A.; Moloney, D. M.; Wall, S. A.; Wilkie, A. O. M.; Zackai, E. H.

    1997-01-01

    The underlying basis of many forms of syndromic craniosynostosis has been defined on a molecular level. However, many patients with familial or sporadic craniosynostosis do not have the classical findings of those craniosynostosis syndromes. Here we present 61 individuals from 20 unrelated families where coronal synostosis is due to an amino acid substitution (Pro250Arg) that results from a single point mutation in the fibroblast growth factor receptor 3 gene on chromosome 4p. In this instance, a new clinical syndrome is being defined on the basis of the molecular finding. In addition to the skull findings, some patients had abnormalities on radiographs of hands and feet, including thimble-like middle phalanges, coned epiphyses, and carpal and tarsal fusions. Brachydactyly was seen in some cases; none had clinically significant syndactyly or deviation of the great toe. Sensorineural hearing loss was present in some, and developmental delay was seen in a minority. While the radiological findings of hands and feet can be very helpful in diagnosing this syndrome, it is not in all cases clearly distinguishable on a clinical basis from other craniosynostosis syndromes. Therefore, this mutation should be tested for in patients with coronal synostosis. ImagesFigure 2Figure 3Figure 4 PMID:9042914

  3. Whole-body MR imaging, bone diffusion imaging: how and why?

    PubMed

    Jaramillo, Diego

    2010-06-01

    Whole-body MRI (W-B MRI) and diffusion-weighted imaging (DWI) are two novel techniques that greatly facilitate the evaluation of many disorders of childhood. In the musculoskeletal system, these techniques primarily aid in the evaluation of the marrow, although there is increasing interest in the study of soft-tissue abnormalities with W-B MRI and of cartilage with DWI.The normal pattern of marrow transformation affects both modalities throughout childhood. Haematopoietic marrow has a much higher signal intensity than fatty marrow on W-B MRI short tau inversion recovery (STIR) images (Darge et al. Eur J Radiol 68:289-298, 2008). Diffusion is greater in haematopoietic marrow than in fatty marrow and decreases in the skeleton with age (Jaramillo et al. Pediatr Radiol 34:S48, 2004). It is important therefore to remember that the entire skeleton is haematopoietic at birth and that there is a process of marrow transformation to fatty marrow. Marrow conversion proceeds from the fingers to the shoulders and from the toes to the hips. Within each bone, fatty marrow transformation begins in the epiphyses, and within the shaft of the long bones fatty marrow transformation begins at the diaphysis and proceeds towards the metaphyses.

  4. Perlecan displays variable spatial and temporal immunolocalisation patterns in the articular and growth plate cartilages of the ovine stifle joint.

    PubMed

    Melrose, James; Smith, Susan; Cake, Martin; Read, Richard; Whitelock, John

    2005-06-01

    Perlecan is a modular heparan sulphate and/or chondroitin sulphate substituted proteoglycan of basement membrane, vascular tissues and cartilage. Perlecan acts as a low affinity co-receptor for fibroblast growth factors 1, 2, 7, 9, binds connective tissue growth factor and co-ordinates chondrogenesis, endochondral ossification and vascular remodelling during skeletal development; however, relatively little is known of its distribution in these tissues during ageing and development. The aim of the present study was to immunolocalise perlecan in the articular and epiphyseal growth plate cartilages of stifle joints in 2-day to 8-year-old pedigree merino sheep. Perlecan was prominent pericellularly in the stifle joint cartilages at all age points and also present in the inter-territorial matrix of the newborn to 19-month-old cartilage specimens. Aggrecan was part pericellular, but predominantly an extracellular proteoglycan. Perlecan was a prominent component of the long bone growth plates and displayed a pericellular as well as a strong ECM distribution pattern; this may indicate a so far unrecognised role for perlecan in the mineralisation of hypertrophic cartilage. A significant age dependant decline in cell number and perlecan levels was evident in the hyaline and growth plate cartilages. The prominent pericellular distribution of perlecan observed indicates potential roles in cell-matrix communication in cartilage, consistent with growth factor signalling, cellular proliferation and tissue development.

  5. Compound flap from the great toe and vascularized joints from the second toe for posttraumatic thumb reconstruction at the level of the proximal metacarpal bone.

    PubMed

    Tsai, Tsu-Min; D'Agostino, Laura; Fang, You-Sheng; Tien, Huey

    2009-01-01

    The purpose of this study is to describe the harvesting technique, anatomic variations, and clinical applications of a compound flap from the great toe and vascularized joint from the second toe used for thumb reconstruction. Five fresh cadaver dissections were studied, focusing attention on the dorsal or plantar vascular dominance, position of the communicating branch between the dorsal and plantar system, the Gilbert classification, and the size of the first dorsal metatarsal artery (FDMA) and first plantar metatarsal artery (FPMA) to the great toe and second toe. Five compound flaps were performed on five patients with traumatic thumb amputation at the level of proximal metacarpal bone. The patients' ages ranged from 14 to 47. Follow-up period was 11-24 months. The anatomic study showed that FPMA had larger caliber in 40% of dissections, FDMA in 40%, and had the same caliber in 20%. The Gilbert classification of FDMA was 40% class I and 60% class III. In the clinical applications, four patients achieved good functional opposition and motion of transferred joints with good pinch and grip strength. There was one flap failure, and donor-site morbidity was minimal. The compound flap offers advantages over traditional toe transfer by providing two functional joints. It can be used for amputation of the thumb at carpometacarpal joint level. Finally, the compound flap maintains growth potential in children through transfer of vascularized epiphyses. The disadvantages of this compound flap include a technically challenging harvest and a longer operative time.

  6. Detection by cationized /sup 125/I-cytochrome C of proteoglycans (PG) transferred to nylon

    SciTech Connect

    Heimer, R.; Sampson, P.M.; Fishman, A.P.

    1986-05-01

    Cytochrome c, labeled with /sup 125/I, has been used by us for staining glycosaminoglycans (GAG) separated by electrophoresis on cellulose acetate strips. As GAG between 1-10 ng could be quantified by autoradiography and densitometry, the reagent is approximately 100-fold more sensitive than currently used non-radiolabeled stains. The authors extend now the use of radiolabeled cytochrome c to the quantification of PG transblotted to solid supports subsequent to separation on polyacrylamine slab gels. Dot blotting used for exploring optimal conditions for detecting PG indicated that because of low background positively charged Nylon 66 was superior to nitrocellulose. Increasing the positive charge of the staining reagent by cationization decreased background radiation even further so that 1 ng PG could be seen readily after 5 hrs of autoradiography. Use of cationized /sup 125/I-cytochrome c has been made in detecting PG of bovine fetal epiphyseal cartilage (> 1 x 10/sup 6/ D) and PG of bovine aorta (0.25 x 10/sup 6/ D) following slab gel electrophoresis and electrophoretic transblotting. With effective electrophoretic transfer of the PG to positively charged Nylon 66, the sensitivity of detection of the two PG was increased at least 100-fold over that observed when the gels were stained with toluidine blue, the method in current use.

  7. New X linked spondyloepimetaphyseal dysplasia: report on eight affected males in the same family.

    PubMed Central

    Camera, G; Stella, G; Camera, A

    1994-01-01

    We report on a probably new form of spondyloepimetaphyseal dysplasia (SEMD) with an X linked inheritance pattern. Eight males were affected in the same family. We were able to examine three adult patients and we studied the skeletal radiological aspect of one of these patients at 2 years 6 months and at 9 years of age. The main clinical features are severe short trunked dwarfism, brachydactyly, normal facies, and normal intelligence. Radiologically, the diaphyses of all the long bones are short and broad. The epiphyses of the distal portion of the femora and those of the proximal and distal portions of the tibia are embedded in their metaphyses and there is marked narrowing of the intercondylar groove. There is moderate platyspondyly. Several vertebrae show an anterior tongue in infancy and severe irregularities of the upper and lower surfaces are present in adulthood. The 11th or 12th thoracic vertebra is wedge shaped. The pelvis is narrow. The distal ulnae and fibulae are disproportionately long. The hands show radial deviation and brachydactyly is present in the hands and feet. This X linked SEMD was not detectable at birth. Images PMID:8064814

  8. Unique Matrix Structure in the Rough Endoplasmic Reticulum Cisternae of Pseudoachondroplasia Chondrocytes

    PubMed Central

    Merritt, Thomas M.; Bick, Roger; Poindexter, Brian J.; Alcorn, Joseph L.; Hecht, Jacqueline T.

    2007-01-01

    Mutations in cartilage oligomeric matrix protein (COMP) cause two skeletal dysplasias, pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED/EDM1). Because COMP exists as a homopentamer, only one mutant COMP subunit may result in an abnormal complex that is accumulated in expanded rough endoplasmic reticulum (rER) cisternae, a hallmark of PSACH. Type IX collagen and matrilin-3 (MATN3), also accumulate in the rER cisternae of PSACH chondrocytes, but it is unknown how mutant COMP interacts with these proteins. The studies herein focus on defining the organization of these intracellularly retained proteins using fluorescence deconvolution microscopy. A unique matrix organization was identified in which type II procollagen formed a central core surrounded by a protein network of mutant COMP, type IX collagen, and MATN3. This pattern of matrix organization was found in multiple cisternae from single chondrocytes and in chondrocytes with different COMP mutations, indicating a common pattern of interaction. This suggests that stalling of mutant COMP and an interaction between mutant COMP and type II procollagen are initiating events in the assembly of matrix in the rER, possibly explaining why the material is not readily cleared from the rER. Altogether, these data suggest that mutant COMP initiates and perhaps catalyzes premature intracellular matrix assembly. PMID:17200202

  9. In vivo and in vitro studies of cartilage differentiation in altered gravities

    NASA Astrophysics Data System (ADS)

    Montufar-Solis, D.; Duke, P. J.; D'Aunno, D.

    The in vivo model our laboratory uses for studies of cartilage differentiation in space is the rat growth plate. Differences between missions, and in rat age and recovery times, provided differing results from each mission. However, in all missions, proliferation and differentiation of chondrocytes in the epiphyseal plate of spaceflown rats was altered as was matrix organization. In vitro systems, necessary complements to in vivo work, provide some advantages over the in vivo situation. In vitro, centrifugation of embryonic limb buds suppressed morphogenesis due to precocious differentiation, and changes in the developmental pattern suggest the involvement of Hox genes. In space, embryonic mouse limb mesenchyme cells differentiating in vitro on IML-1 had smoother membranes and lacked matrix seen in controls. Unusual formations, possibly highly ruffled membranes, were found in flight cultures. These results, coupled with in vivo centrifugation studies, show that in vivo or in vitro, the response of chondrocytes to gravitational changes follows Hert's curve as modified by Simon, i.e. decreased loading decreases differentiation, and increased loading speeds it up, but only to a point. After that, additional increases again slow down chondrogenesis.

  10. Heavy Metal Ion Regulation of Gene Expression: MECHANISMS BY WHICH LEAD INHIBITS OSTEOBLASTIC BONE-FORMING ACTIVITY THROUGH MODULATION OF THE Wnt/β-CATENIN SIGNALING PATHWAY.

    PubMed

    Beier, Eric E; Sheu, Tzong-Jen; Dang, Deborah; Holz, Jonathan D; Ubayawardena, Resika; Babij, Philip; Puzas, J Edward

    2015-07-17

    Exposure to lead (Pb) from environmental sources remains an overlooked and serious public health risk. Starting in childhood, Pb in the skeleton can disrupt epiphyseal plate function, constrain the growth of long bones, and prevent attainment of a high peak bone mass, all of which will increase susceptibility to osteoporosis later in life. We hypothesize that the effects of Pb on bone mass, in part, come from depression of Wnt/β-catenin signaling, a critical anabolic pathway for osteoblastic bone formation. In this study, we show that depression of Wnt signaling by Pb is due to increased sclerostin levels in vitro and in vivo. Downstream activation of the β-catenin pathway using a pharmacological inhibitor of GSK-3β ameliorates the Pb inhibition of Wnt signaling activity in the TOPGAL reporter mouse. The effect of Pb was determined to be dependent on sclerostin expression through use of the SOST gene knock-out mice, which are resistant to Pb-induced trabecular bone loss and maintain their mechanical bone strength. Moreover, isolated bone marrow cells from the sclerostin null mice show improved bone formation potential even after exposure to Pb. Also, our data suggest that the TGFβ canonical signaling pathway is the mechanism by which Pb controls sclerostin production. Taken together these results support our hypothesis that the osteoporotic-like phenotype observed after Pb exposure is, in part, regulated through modulation of the Wnt/β-catenin pathway.

  11. Histologic Examination of an Assemblage of Psittacosaurus (Dinosauria: Ceratopsia) Juveniles From the Yixian Formation (Liaoning, China).

    PubMed

    Bo, Zhao; Hedrick, Brandon P; Chunling, Gao; Tumarkin-Deratzian, Allison R; Fengjiao, Zhang; Caizhi, Shen; Dodson, Peter

    2016-05-01

    Psittacosaurus is one of the most abundant dinosaurs known, which allows for extensive study of its growth and form. Previous studies have evaluated growth trajectories of Psittacosaurus using bone histology. However, we present the first study of Psittacosaurus comparative juvenile histology and describe the histology of Psittacosaurus within its first year of life based on multiple sections taken from an exquisite monospecific assemblage of juveniles from the Yixian Formation in Liaoning, China. Specimens studied had femur lengths ranging from 30 to 36 mm. The five juveniles examined all have similar histologic patterns in the midshaft and epiphyseal regions showing that there is limited plasticity in bone development in juvenile Psittacosaurus and that all of the specimens in the assemblage were likely the same age. The microstructure patterns are compatible with the hypothesis that Psittacosaurus was precocial and that these juveniles were neonates. Based on comparisons with other juvenile ornithischians, juvenile Psittacosaurus had a growth rate similar to Orodromeus, slower than that of Maiasaura, Dysalotosaurus, or hadrosaurs consistent with small body size. Our results support previous studies that demonstrated that the orientation of vascular canals is likely not solely reflective of growth rate, but is also affected by underlying biomechanical, structural processes. The number of studies done on theropod and sauropodomorph histology dwarfs those of ornithischians. More studies of ornithischian histology are necessary in order to better establish phylogenetic trends in microstructure and to learn more about growth in this important clade.

  12. The clinical approach toward giant cell tumor of bone.

    PubMed

    van der Heijden, Lizz; Dijkstra, P D Sander; van de Sande, Michiel A J; Kroep, Judith R; Nout, Remi A; van Rijswijk, Carla S P; Bovée, Judith V M G; Hogendoorn, Pancras C W; Gelderblom, Hans

    2014-05-01

    We provide an overview of imaging, histopathology, genetics, and multidisciplinary treatment of giant cell tumor of bone (GCTB), an intermediate, locally aggressive but rarely metastasizing tumor. Overexpression of receptor activator of nuclear factor κB ligand (RANKL) by mononuclear neoplastic stromal cells promotes recruitment of numerous reactive multinucleated giant cells. Conventional radiographs show a typical eccentric lytic lesion, mostly located in the meta-epiphyseal area of long bones. GCTB may also arise in the axial skeleton and very occasionally in the small bones of hands and feet. Magnetic resonance imaging is necessary to evaluate the extent of GCTB within bone and surrounding soft tissues to plan a surgical approach. Curettage with local adjuvants is the preferred treatment. Recurrence rates after curettage with phenol and polymethylmethacrylate (PMMA; 8%-27%) or cryosurgery and PMMA (0%-20%) are comparable. Resection is indicated when joint salvage is not feasible (e.g., intra-articular fracture with soft tissue component). Denosumab (RANKL inhibitor) blocks and bisphosphonates inhibit GCTB-derived osteoclast resorption. With bisphosphonates, stabilization of local and metastatic disease has been reported, although level of evidence was low. Denosumab has been studied to a larger extent and seems to be effective in facilitating intralesional surgery after therapy. Denosumab was recently registered for unresectable disease. Moderate-dose radiotherapy (40-55 Gy) is restricted to rare cases in which surgery would lead to unacceptable morbidity and RANKL inhibitors are contraindicated or unavailable.

  13. Evaluation of extremity pain in children using technetium-99m MDP bone scan: A general hospital experience

    SciTech Connect

    Park, H.M.; Rothschild, P.A.; Kernek, C.B.

    1984-01-01

    This study was undertaken to evaluate the efficacy of three-phase bone scan in detection of significant pathology i.e., osteomyelitis (OM), septic joint, cellulitis, etc., in children with symptoms of extremity pain. A total of 100 consecutive patients (age 9 days - 16 yrs, 63 boys and 37 girls) were studied. The authors reviewed their scans, x-rays and hospital records. The final diagnoses were based on the findings of needle aspiration, surgical drainage, biopsy, culture, and on the therapeutic response. In 87%, sufficiently long clinical follow-up was available to confirm the final diagnoses. In the remaining 13%, the symptoms resolved quickly and follow-up was not felt necessary. The scan was essential in pinpointing the lesions in pts with referred or nonlocalizing extremity pain. The +ve and -ve predictive values of the scan and OM were 89% and 96% respectively. One spiral fracture was misinterpreted as diffuse OM. One ''Subacute epiphyseal OM'' was not detected. In two cases, cellulitis and septic joint obscured underlying OM. Prior antibotic therapy resulted in one equivocal scan. Although less sensitive (29%) in early OM, radiographs play an important complimentary role. Bone scans detected underlying pathology for extremity pain in 61% of all pts studied.

  14. The Olduvai Hominid 8 foot: adult or subadult?

    PubMed

    DeSilva, Jeremy M; Zipfel, Bernhard; Van Arsdale, Adam P; Tocheri, Matthew W

    2010-05-01

    Olduvai Hominid 8 (OH 8), an articulating set of fossil hominin tarsal and metatarsal bones, is critical to interpretations of the evolution of hominin pedal morphology and bipedal locomotion. It has been suggested that OH 8 may represent the foot of a subadult and may be associated with the OH 7 mandible, the type specimen of Homo habilis. This assertion is based on the presence of what may be unfused distal metatarsal epiphyses. Accurately assessing the skeletal maturity of the OH 8 foot is important for interpretations of the functional morphology and locomotor behavior of Plio-Pleistocene hominins. In this study, we compare metatarsal fusion patterns and internal bone morphology of the lateral metatarsals among subadult hominines (85 modern humans, 48 Pan, and 25 Gorilla) to assess the likelihood that OH 8 belonged to either an adult or subadult hominin. Our results suggest that if OH 8 is indeed from a subadult, then it displays a metatarsal developmental pattern that is unobserved in our comparative sample. In OH 8, the fully fused base of the first metatarsal and the presence of trabecular bone at the distal ends of the second and third metatarsal shafts make it highly improbable that it belonged to a subadult, let alone a subadult that matches the developmental age of the OH 7 mandible. In total, the results of this study suggest that the OH 8 foot most likely belonged to an adult hominin.

  15. Biomechanics and Mechanobiology of Trabecular Bone: A Review

    PubMed Central

    Oftadeh, Ramin; Perez-Viloria, Miguel; Villa-Camacho, Juan C.; Vaziri, Ashkan; Nazarian, Ara

    2015-01-01

    Trabecular bone is a highly porous, heterogeneous, and anisotropic material which can be found at the epiphyses of long bones and in the vertebral bodies. Studying the mechanical properties of trabecular bone is important, since trabecular bone is the main load bearing bone in vertebral bodies and also transfers the load from joints to the compact bone of the cortex of long bones. This review article highlights the high dependency of the mechanical properties of trabecular bone on species, age, anatomic site, loading direction, and size of the sample under consideration. In recent years, high resolution micro finite element methods have been extensively used to specifically address the mechanical properties of the trabecular bone and provide unique tools to interpret and model the mechanical testing experiments. The aims of the current work are to first review the mechanobiology of trabecular bone and then present classical and new approaches for modeling and analyzing the trabecular bone microstructure and macrostructure and corresponding mechanical properties such as elastic properties and strength. PMID:25412137

  16. Anisotropic Permeability of Trabecular Bone and its Relationship to Fabric and Architecture: A Computational Study.

    PubMed

    Kreipke, T C; Niebur, G L

    2017-02-02

    Trabecular bone is a porous, mineralized tissue found in vertebral bodies, the metaphyses and epiphyses of long bones, and in the irregular and flat shaped bones. The pore space is filled with bone marrow, a highly cellular fluid. Together, the bone and marrow behave as a poroelastic solid. In poroelasticity theory, the permeability is the primary material property that governs the momentum transfer between the solid and fluid constituents. In the linearized theory, the permeability of a material depends on the shape and connectivity of the pores. Developing a model of the relationship between trabecular microarchitecture and permeability could lead to improved simulations of trabecular bone mechanical response, which can be used to investigate bone adaptation, mechanobiological signaling, and progression of diseases such as osteoporosis. This study used finite element models of the trabecular pore space to calculate the complete anisotropic permeability tensor of 12 human and 18 porcine femoral trabecular bone samples. The sensitivity of the simulations to model assumptions and post-processing was analyzed to improve confidence in the result. The orthotropic permeability tensor depended on the fabric tensor, trabecular spacing, and structure model index through a power law relationship. Porosity and fabric alone also provided a reasonable prediction, which may be useful in cases where the image resolution is insufficient to obtain detailed measures of architecture.

  17. [Effects of hypokinesia and hypoxia on the bone static electrogenesis].

    PubMed

    Levashov, M I; Safonov, S L; Lakhin, P V

    2010-01-01

    It was studied the conformities to law of quasistatic electric potential (QSEP) distribution on the periosteal surface of 64 adult Vistar rats-males fresh exited femur and their changes after dosed hypokinesia and hypoxia influences. The maximal negative sizes of QSEP were incorporated in epiphyseal-metaphyseal departments and minimal--in the central part of bone diaphysis. Conformities to law of QSEP distribution on the periosteal surface fresh exited bone represented the physiology unevenness of metabolic processes intensity in the different bone departments. Reparative regeneration was accompanied with strengthening of electro-negativity of reparations and adjacent parts and changing of physiology character of QSEP distribution on the periosteal surface of the damaged bone. Hypokinesia disturbed of bone static electrogenesis. It was accompanied with diminishing of QSEP size especially in bone parts which had hight level of metabolism. Dosed an intermittent normobarical and hypobarical hypoxia activated of bone static electrogenesis. More considerably increasing of QSEP in old rats was obtained after the intermittent hypobarical hypoxia influences.

  18. The humerus of Eusthenopteron: a puzzling organization presaging the establishment of tetrapod limb bone marrow.

    PubMed

    Sanchez, S; Tafforeau, P; Ahlberg, P E

    2014-05-07

    Because of its close relationship to tetrapods, Eusthenopteron is an important taxon for understanding the establishment of the tetrapod body plan. Notably, it is one of the earliest sarcopterygians in which the humerus of the pectoral fin skeleton is preserved. The microanatomical and histological organization of this humerus provides important data for understanding the evolutionary steps that built up the distinctive architecture of tetrapod limb bones. Previous histological studies showed that Eusthenopteron's long-bone organization was established through typical tetrapod ossification modalities. Based on a three-dimensional reconstruction of the inner microstructure of Eusthenopteron's humerus, obtained from propagation phase-contrast X-ray synchrotron microtomography, we are now able to show that, despite ossification mechanisms and growth patterns similar to those of tetrapods, it also retains plesiomorphic characters such as a large medullary cavity, partly resulting from the perichondral ossification around a large cartilaginous bud as in actinopterygians. It also exhibits a distinctive tubular organization of bone-marrow processes. The connection between these processes and epiphyseal structures highlights their close functional relationship, suggesting that either bone marrow played a crucial role in the long-bone elongation processes or that trabecular bone resulting from the erosion of hypertrophied cartilage created a microenvironment for haematopoietic stem cell niches.

  19. Tuberculosis of the knee -- a long term follow-up.

    PubMed

    Chow, S P; Yau, A

    1980-01-01

    Thirty cases of tuberculosis of the knee followed up for an average of 15 years were reviewed. The majority of patients developed the disease during childhood. All had received standard anti-tuberculous drug treatment. Fifteen were treated conservatively alone, while the other 15 had a debridement type of surgery in addition to drugs. At review, one-third had occasional mild pain, but this was only present in the conservatively treated group. Stiffness, however, was more predominant in the operated and in the late onset groups. Some mild deformity was seen in 17 out of 30 patients and was related not so much to disturbance of epiphyseal growth, but rather, to bone collapse. Interesting X-ray appearances at follow-up were found. The factors which could lead to a good outcome included young age of onset, treatment within six months of onset, and early mobilisation. If the disease is well advanced, surgical treatment will lead to a painless joint, but with greater restriction of joint movement.

  20. Further characterization of ATP-initiated calcification by matrix vesicles isolated from rachitic rat cartilage. Membrane perturbation by detergents and deposition of calcium pyrophosphate by rachitic matrix vesicles.

    PubMed

    Hsu, H H; Camacho, N P; Anderson, H C

    1999-01-12

    Although membrane associated enzymes such as ATPase, alkaline phosphatase, and NTP pyrophosphohydrolase in matrix vesicles (MVs) may underlie the mechanisms of ATP-promoted calcification, prior to the current investigation, the role of the MV membrane in calcification had not been addressed. In this study, various perturbations were introduced to the MV membrane in in vitro calcification systems to determine ideal conditions for ATP-initiated calcification by MVs isolated from rachitic rat epiphyseal cartilage. Membrane integrity appears to be required, since the rupture of the vesicular membrane by vigorously mixing with 10% butanol abolished calcification. In contrast, a mild treatment of MVs with low concentrations (e.g., 0.01%, which is much below the critical concentration for micelle formation) of either neutral Triton X-100 or anionic deoxycholate stimulated calcification by >2-fold, without inducing obvious changes in vesicular appearance. Fourier transform infrared spectroscopic studies were done to identify the mineral phase formed in these experiments. For the first time, rachitic MVs were shown to induce the formation of a calcium pyrophosphate dihydrate-like phase after their exposure to calcifying medium with 1 mM ATP. The integration of spectral areas indicated that calcification was enhanced by Triton X-100. The detergent effect was reversible and appeared to be not mediated through activation of ATPase, alkaline phosphatase, or ATP pyrophosphohydrolase. In contrast to neutral Triton X-100 and anionic deoxycholate, cationic cetyltrimethylammonium bromide inhibited both ATPase activity (I50=10 microM) and ATP-initiated calcification. These observations suggest that membrane perturbations can affect calcification and that the presence of NTP-pyrophosphohydrolase in MVs may play a role in the deposition of CaPPi in rachitic cartilage.

  1. Whole-Body and Microenvironmental Localization of Radium-223 in Naïve and Mouse Models of Prostate Cancer Metastasis

    PubMed Central

    Abou, Diane S.; Ulmert, David; Doucet, Michele; Hobbs, Robert F.; Riddle, Ryan C.

    2016-01-01

    Background: Bone-metastatic, castration-resistant prostate cancer (bmCRPC) represents a lethal stage of the most common noncutaneous cancer in men. The recent introduction of Radium-223 dichloride, a bone-seeking alpha particle (α)–emitting radiopharmaceutical, demonstrates statistically significant survival benefit and palliative effect for bmCRPC patients. Clinical results have established safety and efficacy, yet questions remain regarding pharmacodynamics and dosing for optimized patient benefit. Methods: We elucidated the biodistribution of 223Ra as well as interaction with the bone and tumor compartments in skeletally mature mice (C57Bl/6 and CD-1, n = 3–6) and metastasis models (LNCaP and PC3, n = 4). Differences in uptake were evaluated by µCT and histological investigation. Novel techniques were leveraged on whole-mount undecalcified cryosections to determine microdistribution of Radium-223. All statistical tests were two-sided. Results: 223Ra uptake in the bones (>30% injected activity per gram) at 24 hours was also accompanied by non-negligible remnant activity in the kidney (2.33% ± 0.36%), intestines (5.73% ± 2.04%), and spleen (10.5% ± 5.9%) Skeletal accumulation across strains did not correspond with bone volume or surface area but instead to local blood vessel density (P = .04). Microdistribution analysis by autoradiography and α camera revealed targeting of the ossifying surfaces adjacent to the epiphyseal growth plate. In models of PCa metastasis, radioactivity does not localize directly within tumors but instead at the apposite bone surface. Osteoblastic and lytic lesions display similar intensity, which is comparable with uptake at sites of normal bone remodeling. Conclusions: Profiling the macro- and microdistribution of 223Ra in healthy and diseased models has important implications to guide precision application of this emerging α-therapy approach for bmCRPC and other bone metastastic diseases. PMID:26683407

  2. Skeletal dysplasia in ancient Egypt.

    PubMed

    Kozma, Chahira

    2008-12-01

    The ancient Egyptian civilization lasted for over 3000 years and ended in 30 BCE. Many aspects of ancient Egyptian culture, including the existence of skeletal dysplasias, and in particular achondroplasia, are well known through the monuments and records that survived until modern times. The hot and dry climate in Egypt allowed for the preservation of bodies and skeletal anomalies. The oldest dwarf skeleton, the Badarian skeleton (4500 BCE), possibly represents an epiphyseal disorder. Among the remains of dwarfs with achondroplasia from ancient Egypt (2686-2190 BCE), exists a skeleton of a pregnant female, believed to have died during delivery with a baby's remains in situ. British museums have partial skeletons of dwarfs with achondroplasia, humeri probably affected with mucopolysaccharidoses, and a skeleton of a child with osteogenesis imperfecta. Skeletal dysplasia is also found among royal remains. The mummy of the pharaoh Siptah (1342-1197 BCE) shows a deformity of the left leg and foot. A mummified fetus, believed to be the daughter of king Tutankhamun, has scoliosis, spina bifida, and Sprengel deformity. In 2006 I reviewed the previously existing knowledge of dwarfism in ancient Egypt. The purpose of this second historical review is to add to that knowledge with an expanded contribution. The artistic documentation of people with skeletal dysplasia from ancient Egypt is plentiful including hundreds of amulets, statues, and drawing on tomb and temple walls. Examination of artistic reliefs provides a glance of the role of people with skeletal dysplasia and the societal attitudes toward them. Both artistic evidence and moral teachings in ancient Egypt reveal wide integration of individuals with disabilities into the society.

  3. The lameness of King Philip II and Royal Tomb I at Vergina, Macedonia.

    PubMed

    Bartsiokas, Antonis; Arsuaga, Juan-Luis; Santos, Elena; Algaba, Milagros; Gómez-Olivencia, Asier

    2015-08-11

    King Philip II was the father of Alexander the Great. He suffered a notorious penetrating wound by a lance through his leg that was nearly fatal and left him lame in 339 B.C.E. (i.e., 3 y before his assassination in 336 B.C.E.). In 1977 and 1978 two male skeletons were excavated in the Royal Tombs II and I of Vergina, Greece, respectively. Tomb I also contained another adult (likely a female) and a newborn skeleton. The current view is that Philip II was buried in Tomb II. However, the male skeleton of Tomb II bears no lesions to his legs that would indicate lameness. We investigated the skeletal material of Tomb I with modern forensic techniques. The male individual in Tomb I displays a conspicuous case of knee ankylosis that is conclusive evidence of lameness. Right through the overgrowth of the knee, there is a hole. There are no obvious signs that are characteristic of infection and osteomyelitis. This evidence indicates that the injury was likely caused by a severe penetrating wound to the knee, which resulted in an active inflammatory process that stopped years before death. Standard anthropological age-estimation techniques based on dry bone, epiphyseal lines, and tooth analysis gave very wide age ranges for the male, centered around 45 y. The female would be around 18-y-old and the infant would be a newborn. It is concluded that King Philip II, his wife Cleopatra, and their newborn child are the occupants of Tomb I.

  4. Microvascular features and ossification process in the femoral head of growing rats

    PubMed Central

    MORINI, SERGIO; PANNARALE, LUIGI; FRANCHITTO, ANTONIO; DONATI, SAARA; GAUDIO, EUGENIO

    1999-01-01

    In the epiphysis of long bones, different patterns of development of ossification processes have been described in different species. The development of the vascularisation of the femoral head has not yet been fully clarified, although its role in the ossification process is obvious. Our aim was to investigate ossification and vascular proliferation and their relationship, in growing rat femoral heads. Male Wistar rats aged ∼ 1, 5 and 8 wk and 4, 8 and 12 mo were used. Light microscopy frontal sections and vascular corrosion casts observed by scanning electron microscopy were employed. In the rat proximal femoral epiphysis, ossification develops from the medullary circulation of the diaphysis, quickly extending to the neck and the base of the head. Hypertrophic chondrocytes occupy the epiphyseal cartilage, and a physeal plate with regular cell columns is present. Starting from about the end of the third month one or more points of fibrovascular outgrowth, above the physeal line, can be observed in each sample. They are often placed centrally or, sometimes, peripherally. The fibrovascular outgrowths penetrate deeply into the cartilage and extend laterally. At age 8 mo, large fibro-osseous peduncles connect the epiphysis to the diaphyseal tissue. At 12 mo, the entire epiphysis appears calcified with an almost total absence of residual cartilage islands. This situation differs in man and in other mammals due both to differing thickness of the cartilage and to the presence of more extensive sources of blood vessels other than the diaphyseal microcirculation, as supplied by the teres ligament and Hunter's circle. In young rats, subchondral vessels and the synovial fluid could play a role in feeding the ossifying cartilage. Later, a loss of resistance of the physis due to marked degeneration of the cell columns, and extensive chondrocyte hypertrophy permit fibrovascular penetration starting from diaphyseal vessels rather than neighbouring vascular territories, such as

  5. Effect of dietary calcium on bone metabolism in young and aged female rats using a short-term in vivo model

    SciTech Connect

    Sinha, R.; Smith, J.C. Jr.; Soares, J.H. Jr.

    1988-10-01

    The use of high dietary calcium supplementation in the treatment of patients with osteoporosis is controversial. The present study examined the mechanisms underlying the effects of calcium supplementation by investigating the influence of dietary calcium on bone dynamics in young and aged rats. A 2 x 2 x 2 factorial design was utilized with 0.2% (low) or 1.0% (high) calcium, 2- or 24-m-old female Long-Evans rats that were implanted subcutaneously with demineralized (DB) and mineralized (MB) bone powder. The four groups of rats were fed each of the respective diets for 11 wk and then implanted with one number5 gelatin capsule containing 30 mg of DB and another containing 100 mg of MB powder. The animals were injected intraperitoneally with 0.1 microCi/g body weight with 45Ca 14 h before the end of experiment. The ectopic bone as well as the right femurs were harvested 14 d after the rats were implanted. Marker enzyme activities (alkaline-formation and acid-resorption phosphatase), 45Ca uptake and calcium content were measured in the implants and the distal epiphyses of the right femurs. Bone turnover was higher in the young rats than in the old animals, and high dietary calcium in the young animals increased bone formation, as indicated by alkaline phosphatase activity. Dietary calcium level did not affect ectopic bone formation or resorption in the aged rats. The results indicate that high dietary intake of calcium does not affect bone dynamics in aged female rats but does increase bone formation in young rats.

  6. Skeletal development in Pan paniscus with comparisons to Pan troglodytes.

    PubMed

    Bolter, Debra R; Zihlman, Adrienne L

    2012-04-01

    Fusion of skeletal elements provides markers for timing of growth and is one component of a chimpanzee's physical development. Epiphyseal closure defines bone growth and signals a mature skeleton. Most of what we know about timing of development in chimpanzees derives from dental studies on Pan troglodytes. Much less is known about the sister species, Pan paniscus, with few in captivity and a wild range restricted to central Africa. Here, we report on the timing of skeletal fusion for female captive P. paniscus (n = 5) whose known ages range from 0.83 to age 11.68 years. Observations on the skeletons were made after the individuals were dissected and bones cleaned. Comparisons with 10 female captive P. troglodytes confirm a generally uniform pattern in the sequence of skeletal fusion in the two captive species. We also compared the P. paniscus to a sample of three unknown-aged female wild P. paniscus, and 10 female wild P. troglodytes of known age from the Taï National Park, Côte d'Ivoire. The sequence of teeth emergence to bone fusion is generally consistent between the two species, with slight variations in late juvenile and subadult stages. The direct-age comparisons show that skeletal growth in captive P. paniscus is accelerated compared with both captive and wild P. troglodytes populations. The skeletal data combined with dental stages have implications for estimating the life stage of immature skeletal materials of wild P. paniscus and for more broadly comparing the skeletal growth rates among captive and wild chimpanzees (Pan), Homo sapiens, and fossil hominins.

  7. Combined Treatment with Gonadotropin-releasing Hormone Analog and Anabolic Steroid Hormone Increased Pubertal Height Gain and Adult Height in Boys with Early Puberty for Height.

    PubMed

    Tanaka, Toshiaki; Naiki, Yasuhiro; Horikawa, Reiko

    2012-04-01

    Twenty-one boys with a height of 135 cm or less at onset of puberty were treated with a combination of GnRH analog and anabolic steroid hormone, and their pubertal height gain and adult height were compared with those of untreated 29 boys who enter puberty below 135 cm. The mean age at the start of treatment with a GnRH analog, leuprorelin acetate depot (Leuplin(®)) was 12.3 yr, a mean of 1.3 yr after the onset of puberty, and GnRH analog was administered every 3 to 5 wk thereafter for a mean duration of 4.1 yr. The anabolic steroid hormone was started approximately 1 yr after initiation of treatment with the GnRH analog. The mean pubertal height gain from onset of puberty till adult height was significantly greater in the combination treatment group (33.9 cm) than in the untreated group (26.4 cm) (p<0.0001). The mean adult height was significantly greater in the combination treatment group (164.3 cm) than in the untreated group (156.9 cm) (p<0.0001). The percentage of subjects with an adult height of 160 cm or taller was 90.5% (19/21) in the combination treatment group, and it was 13.8% (4/29) in the untreated group (p<0.0001). Since growth of the penis and pubic hair is promoted by the anabolic steroid hormone, no psychosocial problems arose because of delayed puberty. No clinically significant adverse events appeared. Combined treatment with GnRH analog and anabolic steroid hormone significantly increased height gain during puberty and adult height in boys who entered puberty with a short stature, since the period until epiphyseal closure was extended due to deceleration of the bone age maturation by administration of the GnRH analog and the growth rate at this time was maintained by the anabolic steroid hormone.

  8. Effects of long-term estrogen replacement therapy on bone turnover in periarticular tibial osteophytes in surgically postmenopausal cynomolgus monkeys.

    PubMed

    Olson, Erik J; Lindgren, Bruce R; Carlson, Cathy S

    2008-05-01

    The aims of the present study were to assess the effects of long-term estrogen replacement therapy (ERT) on size and indices of bone turnover in periarticular osteophytes in ovariectomized cynomolgus monkeys and to compare dynamic indices of bone turnover in osteophyte bone with those of subchondral bone (SCB) and epiphyseal/metaphyseal cancellous (EMC) bone. One hundred sixty-five adult female cynomolgus macaques were bilaterally ovariectomized and randomly divided into three age- and weight-matched treatment groups for a 36-month treatment period. Group 1 (OVX control) received no treatment, Group 2 (SPE) received soy phytoestrogens, and Group 3 (ERT) received conjugated equine estrogens in the diet; all monkeys were labeled with calcein before necropsy. A midcoronal, plastic-embedded section of the right proximal tibia from 20 randomly selected animals per treatment group was examined histologically. Forty-nine of the sections (OVX control, n=16; SPE, n=16; ERT, n=17) contained lateral abaxial osteophytes, and static and dynamic histomorphometry measurements were taken from osteophyte bone, SCB from the lateral tibial plateau, and EMC bone. Data were analyzed using the ANOVA and Kruskal-Wallis test, correlation and regression methods, and the Friedman and Wilcoxon signed rank test. There was no significant effect of long-term ERT on osteophyte area or on any static or dynamic histomorphometry parameters. The bone volume, trabecular number, and trabecular thickness in osteophyte bone were considerably higher than in EMC bone; whereas, trabecular separation was considerably lower in osteophyte bone. In all three treatment groups, BS/BV was significantly lower in osteophyte bone vs. EMC bone and significantly higher in osteophyte bone vs. lateral SCB. We conclude that osteophyte area and static and dynamic histomorphometry parameters within periarticular tibial osteophytes in ovariectomized cynomolgus monkeys are not significantly influenced by long-term ERT, but

  9. The skeletal developmental toxicity of chlormequat chloride and its underlying mechanisms.

    PubMed

    Huang, Dan; Wu, Shuang; Hou, Xiaohong; Jia, Lixia; Meng, Qinghe; Chu, Hongqian; Jiang, Jianjun; Shang, Lanqin; Hao, Weidong

    2017-04-15

    Chlormequat Chloride (CCC), a widely used plant growth regulator, could decrease body weight in animals; however, the mechanism has not been well studied. This study was designed to evaluate the skeletal development toxicity of CCC on pubertal male Sprague-Dawley (SD) rats and to investigate whether CCC impacts the development of chondrocyte, osteoblast and osteoclast through growth hormone (GH) and insulin like growth factor 1 (IGF-I). Rats from 23 to 70 on postnatal days were exposed to CCC daily by gavage at doses of 0, 75, 150, and 300mg/kg bw/d. The results showed that the size of femurs and tibias, bone mineral density and biomechanical parameters were significantly decreased in the 300mg/kg bw/d group compared with the control group. The concentration of osteocalcin (OCN) and C-terminal telopeptide of type I collagen (CTX-I) in blood in the 150mg/kg bw/d group was also changed. The mRNA expression ratio of the receptor activator of NF-κB ligand (RANKL) and osteoprotegerin (OPG) in 150 and 300mg/kg bw/d group was increased. Histological analysis of proximal and distal epiphyseal plates of the right femurs showed that both the proliferative zone and hypertrophic zone narrowed in CCC-treated groups. The concentration of IGF-I in blood was reduced with an increase in exposure doses of CCC. The mRNA expression of growth hormone receptor (GHR) in tibia was decreased in the CCC-treated group. The results indicated that CCC might indirectly impact the formation and activation of chondrocytes, osteoblasts and osteoclasts because of the decline of GHR and IGF-I, leading to skeletal development damage.

  10. The lameness of King Philip II and Royal Tomb I at Vergina, Macedonia

    PubMed Central

    Bartsiokas, Antonis; Arsuaga, Juan-Luis; Santos, Elena; Algaba, Milagros; Gómez-Olivencia, Asier

    2015-01-01

    King Philip II was the father of Alexander the Great. He suffered a notorious penetrating wound by a lance through his leg that was nearly fatal and left him lame in 339 B.C.E. (i.e., 3 y before his assassination in 336 B.C.E.). In 1977 and 1978 two male skeletons were excavated in the Royal Tombs II and I of Vergina, Greece, respectively. Tomb I also contained another adult (likely a female) and a newborn skeleton. The current view is that Philip II was buried in Tomb II. However, the male skeleton of Tomb II bears no lesions to his legs that would indicate lameness. We investigated the skeletal material of Tomb I with modern forensic techniques. The male individual in Tomb I displays a conspicuous case of knee ankylosis that is conclusive evidence of lameness. Right through the overgrowth of the knee, there is a hole. There are no obvious signs that are characteristic of infection and osteomyelitis. This evidence indicates that the injury was likely caused by a severe penetrating wound to the knee, which resulted in an active inflammatory process that stopped years before death. Standard anthropological age-estimation techniques based on dry bone, epiphyseal lines, and tooth analysis gave very wide age ranges for the male, centered around 45 y. The female would be around 18-y-old and the infant would be a newborn. It is concluded that King Philip II, his wife Cleopatra, and their newborn child are the occupants of Tomb I. PMID:26195763

  11. The Skeletal Phenotype of Chondroadherin Deficient Mice

    PubMed Central

    Wenglén, Christina; Petzold, Christiane; Tanner, Elizabeth K.; Brorson, Sverre-Henning; Baekkevold, Espen S.; Önnerfjord, Patrik; Reinholt, Finn P.; Heinegård, Dick

    2013-01-01

    Chondroadherin, a leucine rich repeat extracellular matrix protein with functions in cell to matrix interactions, binds cells via their α2β1 integrin as well as via cell surface proteoglycans, providing for different sets of signals to the cell. Additionally, the protein acts as an anchor to the matrix by binding tightly to collagens type I and II as well as type VI. We generated mice with inactivated chondroadherin gene to provide integrated studies of the role of the protein. The null mice presented distinct phenotypes with affected cartilage as well as bone. At 3–6 weeks of age the epiphyseal growth plate was widened most pronounced in the proliferative zone. The proteome of the femoral head articular cartilage at 4 months of age showed some distinct differences, with increased deposition of cartilage intermediate layer protein 1 and fibronectin in the chondroadherin deficient mice, more pronounced in the female. Other proteins show decreased levels in the deficient mice, particularly pronounced for matrilin-1, thrombospondin-1 and notably the members of the α1-antitrypsin family of proteinase inhibitors as well as for a member of the bone morphogenetic protein growth factor family. Thus, cartilage homeostasis is distinctly altered. The bone phenotype was expressed in several ways. The number of bone sialoprotein mRNA expressing cells in the proximal tibial metaphysic was decreased and the osteoid surface was increased possibly indicating a change in mineral metabolism. Micro-CT revealed lower cortical thickness and increased structure model index, i.e. the amount of plates and rods composing the bone trabeculas. The structural changes were paralleled by loss of function, where the null mice showed lower femoral neck failure load and tibial strength during mechanical testing at 4 months of age. The skeletal phenotype points at a role for chondroadherin in both bone and cartilage homeostasis, however, without leading to altered longitudinal growth. PMID

  12. Normal bone growth requires optimal estrogen levels: negative effects of both high and low dose estrogen on the number of growth plate chondrocytes.

    PubMed

    Takano, Hiroyuki; Aizawa, Toshimi; Irie, Taichi; Itoi, Eiji; Kokubun, Shoichi; Roach, Helmtrud I

    2008-03-01

    Endochondral bone formation at epiphyseal growth plate consists of the synchronized processes of chondrogenesis and cartilage ossification. Estrogen, the major female sex hormone, plays an important role in this process, particularly during the pubertal growth spurt. However, its effects on the growth plate are not completely understood. The aims of this study were to clarify the effects of estrogen on the kinetics of chondrocytes in the growth plates of 10- to 25-week-old female rabbits by studying the effects of ovariectomy or high-dose administration of estrogen on the balance between cell proliferation and death. Forty-eight Japanese white rabbits were divided into three groups: sham operated, ovariectomized, or ovariectomized with subsequent weekly injection of high dose estrogen from 10 weeks. The chondrocyte kinetics was investigated by histomorphometry and immunohistochemistry, using antibodies for caspase-3, a marker of apoptosis, and for proliferating cell nuclear antigen. Both ovariectomized and estrogen-injected rabbits showed a declination of the chondrocyte number although the latter animals indicated a more dramatic effect. Estrogen-injected rabbits showed a decrease in the cell proliferating ability together with an increase in chondrocytes undergoing apoptosis while ovariectomy mainly reduced the cell proliferating ability. Given the known importance of estrogen for bone growth, one would expect that ovariectomy and high-dose administration of estrogen would have opposite effects. However, the present study indicated that both low and high concentration had a similar effect: a decrease in the chondrocyte number compared with control, suggesting that estrogen has to be maintained within a narrow range for optimal bone growth.

  13. The cartilage-bone interface.

    PubMed

    Hoemann, Caroline D; Lafantaisie-Favreau, Charles-Hubert; Lascau-Coman, Viorica; Chen, Gaoping; Guzmán-Morales, Jessica

    2012-05-01

    In the knee joint, the purpose of the cartilage-bone interface is to maintain structural integrity of the osteochondral unit during walking, kneeling, pivoting, and jumping--during which tensile, compressive, and shear forces are transmitted from the viscoelastic articular cartilage layer to the much stiffer mineralized end of the long bone. Mature articular cartilage is integrated with subchondral bone through a approximately 20 to approximately 250 microm thick layer of calcified cartilage. Inside the calcified cartilage layer, perpendicular chondrocyte-derived collagen type II fibers become structurally cemented to collagen type I osteoid deposited by osteoblasts. The mature mineralization front is delineated by a thin approximately 5 microm undulating tidemark structure that forms at the base of articular cartilage. Growth plate cartilage is anchored to epiphyseal bone, sometimes via a thin layer of calcified cartilage and tidemark, while the hypertrophic edge does not form a tidemark and undergoes continual vascular invasion and endochondral ossification (EO) until skeletal maturity upon which the growth plates are fully resorbed and replaced by bone. In this review, the formation of the cartilage-bone interface during skeletal development and cartilage repair, and its structure and composition are presented. Animal models and human anatomical studies show that the tidemark is a dynamic structure that forms within a purely collagen type II-positive and collagen type I-negative hyaline cartilage matrix. Cartilage repair strategies that elicit fibrocartilage, a mixture of collagen type I and type II, are predicted to show little tidemark/calcified cartilage regeneration and to develop a less stable repair tissue-bone interface. The tidemark can be regenerated through a bone marrow-driven growth process of EO near the articular surface.

  14. Magnetic resonance imaging of trabecular and cortical bone in mice: comparison of high resolution in vivo and ex vivo MR images with corresponding histology.

    PubMed

    Weber, Michael H; Sharp, Jonathan C; Latta, Peter; Sramek, Milos; Hassard, H Thomas; Orr, F William

    2005-01-01

    Measurements of bone morphometry and remodeling have been shown to reflect bone strength and can be used to diagnose degenerative bone disease. In this study, in vivo and ex vivo magnetic resonance imaging (MRI) techniques to assess trabecular and cortical bone properties have been compared to each other and to histology as a novel means for the quantification of bone. Femurs of C57Bl/6 mice were examined both in vivo and ex vivo on an 11.7 T MRI scanner, followed by histologic processing and morphometry. A thresholding analysis technique was applied to the MRI images to generate contour lines and to delineate the boundaries between bone and marrow. Using MRI, an optimal correlation with histology was obtained with an in vivo longitudinal sectioned short echo time gradient-echo versus an in vivo long echo time spin-echo sequence or an ex vivo pulse sequence. Gradient-echo images were acquired with a maximum in-plane resolution of 35 microm. Our results demonstrated that in both the in vivo and ex vivo data sets, the percent area of marrow increases and percent area of trabecular bone and cortical bone thickness decreases moving from the epiphyseal growth plate to the diaphysis. These changes, observed with MRI, correlate with the histological data. Investigations using in vivo MRI gradient-echo sequences consistently gave the best correlation with histology. Our quantitative evaluation using both ex vivo and in vivo MRI was found to be an effective means to visualize non-invasively the normal variation in trabecular and