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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. The persistence of epiphyseal scars in the adult tibia.

    PubMed

    Davies, Catriona; Hackman, Lucina; Black, Sue

    2014-03-01

    Estimation of chronological age from skeletal material is dependent upon estimation of maturational stage observed. Following completion of epiphyseal fusion, a transverse radio-opaque line, termed "epiphyseal scar", may be observed in the region of the former growth plate. According to the literature, this line is likely to become obliterated shortly after completion of epiphyseal fusion. Consequently, presence of an epiphyseal scar has been interpreted as an indication of recent epiphyseal fusion; however, this has not been validated by quantitative research. A study was undertaken to determine persistence of the epiphyseal scars in a cross-sectional population of adults between 20 and 50 years of age. This study examined 1,216 radiographs of proximal and distal tibiae from both sexes and sides of the body. This study suggested that 98.05% of females and 97.74% of males retained some remnant of the epiphyseal scar at the proximal tibia whilst 92.72% of females and 92.95% of males retained some remnant of the epiphyseal scar at the distal tibia. General linear model (GLM) analysis determined that chronological age accounted for 2.7% and 7.6% of variation in persistence of the epiphyseal scar at the proximal and distal tibiae, respectively. This study suggests that obliteration of the epiphyseal scar is not as dependent on chronological age as previously thought. It is, therefore, recommended that this feature not be used as an indicator of chronological age during forensic age assessment.

  6. Epiphyseal, vertebral, and ear (EVE) dysplasia: a new syndrome?

    PubMed Central

    Amiel, J.; Cormier-Daire, V.; Journeau, P.; Mussat, P.; Munnich, A.; Lyonnet, S.

    1999-01-01

    We report on the association of epiphyseal, vertebral, and ear dysplasia in two sisters with normal stature and psychomotor development born to distantly related, healthy parents. This distinctive association has not been reported previously and is likely to represent a new condition with an autosomal recessive mode of inheritance. For this syndrome, we propose the acronym EVE standing for epiphyseal, vertebral, and ear dysplasia.


Keywords: epiphyseal; verterbal; and ear (EVE) dysplasia; new syndrome PMID:10424819

  7. 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.

  8. 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.

  9. 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

  10. Genetic heterogeneity in multiple epiphyseal dysplasia

    SciTech Connect

    Deere, M.; Hecht, J.T.; Blanton, S.H.; Scott, C.I.; Langer, L.O.; Pauli, R.M.

    1995-03-01

    Multiple epiphyseal dysplasia (MED) comprises a group of hereditary chondrodysplasias in which there are major anatomic abnormalities of the long tubular bones. The Fairbank and Ribbing types are the most frequently cited types of MED. They are primarily defined radiographically and are autosomal dominant conditions. Recently, MED in one family was shown to map to the pericentromeric region of chromosome 19 and is probably allelic to pseudoachondroplasa. We have tested linkage with six short tandem repeat markers from chromosome 19 to autosomal dominant MED in one four-generation family and to MED in a unique family with three of seven siblings affected and with unaffected parents. Autosomal dominant MED in family 1 was linked with a maximum LOD score, at D19S212, of 3.22 at a recombination fraction ({theta}) of .00. Linkage to chromosome 19 was excluded with MED in the other family, under both autosomal recessive and autosomal dominant, with either reduced-penetrance or germ line-mosaicism models. Linkage to candidate genes COL9A1, COL9A2, and COL11A2 was tested and excluded for both genetic models in this family. COL11A1 was excluded under a recessive model. We have confirmed linkage of autosomal dominant Fairbank MED to chromosome 19 and have demonstrated that MED is genetically heterogeneous. 16 refs., 9 figs., 3 tabs.

  11. [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.

  12. 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.

  13. [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.

  14. Avascular necrosis of the hip in multiple epiphyseal dysplasia

    SciTech Connect

    Mackenzie, W.G.; Bassett, G.S.; Mandell, G.A.; Scott, C.I. Jr. )

    1989-11-01

    We observed radiographic changes of avascular necrosis (AVN) of the capital femoral epiphysis in 9 hips of 11 patients with multiple epiphyseal dysplasia (MED). Plain roentgenography, bone scintigraphy, and magnetic resonance imaging (MRI) studies all revealed characteristic asymmetric changes in the presence of AVN superimposed on dysplastic femoral heads.

  15. 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.

  16. 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

  17. 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

  18. 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.

  19. 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.

  20. BILATERAL OLECRANON EPIPHYSEAL FRACTURE NON-UNION IN A COMPETITIVE ATHLETE

    PubMed Central

    Clark, Randy R.; McKinley, Todd O.

    2010-01-01

    Olecranon epiphyseal stress fractures and epiph-yseal non-unions have been described in throwing athletes, weight lifters and gymnasts. We present a case in which bilateral olecranon epiphyseal fractures were diagnosed in a competitive NCAA Division One wrestler who presented with chronic elbow pain. Given the rigors and physical demands of collegiate wrestling, we present a novel techique for open reduction internal fixation, grafting and supplementation with BMP for accelerated healing and return to competition. PMID:21045994

  1. 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

  2. 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

  3. 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.

  4. 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. © 2014 Wiley Periodicals, Inc.

  5. 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.

  6. 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.

  7. 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

  8. 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

  9. 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. © 2014 APMIS. Published by John Wiley & Sons Ltd.

  10. 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.

  11. 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).

  12. 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).

  13. 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).

  14. 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

  15. Oldest epiphyseal osteochondroma in a subadult from Ancient Egypt.

    PubMed

    Isidro, Albert; Catalán, Josep Maria; Prat, Carme; Torner, Ferran

    2017-08-01

    Benign bone tumours are pathologies frequently encountered in archaeological human remains, with the most common being osteoma and osteochondroma. We present the case of a juvenile individual recovered from the Necropolis of Sharuna, Middle Egypt and dated to the end of Old Kingdom and First Intermediate Period of Ancient Egypt (circa 2150 BC), showing an osteochondroma arising from the proximal epiphysis of the right tibia which, in all likelihood, affected the patellar tendon in life. Osteochondromas are usually discovered during childhood and adolescence. These lesions are commonly located at the metaphysis and diaphysis of long bones and directed away from the joint, with the epiphysis being a rare location. To our knowledge, there have been no similar cases published to date from ancient times and we conclude that this is the oldest case of epiphyseal osteochondroma reported.

  16. 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.

  17. 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

  18. 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

  19. 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.

  20. 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.

  1. 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.

  2. 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.

  3. 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

  4. Molecular differentiation in epiphyseal and physeal cartilage. Prominent role for gremlin in maintaining hypertrophic chondrocytes in epiphyseal cartilage.

    PubMed

    Shapiro, Frederic; Flynn, Evelyn; Calicchio, Monica L

    2009-12-18

    We have studied hypertrophic and immediately adjacent pre-hypertrophic chondrocytes at the same stage of histologic development in 7 day old post-natal Balb/C mouse physes and epiphyses. Laser capture microdissection (LCM) and GeneChip microarray analysis compared the molecular composition of the two hypertrophic chondrocyte regions. Molecules upregulated in dramatically higher levels in the epiphysis were gremlin (58-fold), epidermal growth factor-containing fibulin-like extracellular matrix protein 1 (25-fold), and frizzled related protein (6.4-fold and 5.7-fold). Molecules upregulated in higher levels in the physis were proline arginine-rich end leucine-rich repeat protein (PRELP) (15.6-fold), pyrophosphatase (inorganic) 1 (10-fold) and hedgehog-interacting protein (7.3-fold). Immunocytochemistry for gremlin confirmed specific localization patterns. This study indicates a critical site-specific role for hypertrophic chondrocytes with different synthesis patterns in separate regions even though they appear structurally the same and are at the same stage of development.

  5. 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

  6. 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.

  7. 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.

  8. Trevor Disease (Hemimelic Epiphyseal Displasia): 12-year Follow-up Case Report and Literature Review

    PubMed Central

    Baumfeld, DS; Pires, RES; Macedo, BD; Abreu-e-Silva, GMD; Alves, TA; Raduan, FC; Nery, CADS

    2014-01-01

    Trevor disease or hemimelic epiphyseal dysplasia is a rare skeletal developmental disorder characterized by asymmetric overgrowth of cartilage in the epiphyses. Histologically, it is an epiphysis osteochondroma. The symptom onset occurs primarily during childhood. Males are 3 times more affected than females. The most common symptom is a painless bony mass around the ankle or knee, followed by swelling, restricted range of motion and deformity. Imaging diagnosis is based on plain radiographs, computed tomography scans and magnetic resonance imaging. Treatment depends on the deformities, symptoms, location and amount of epiphysis involvement. Asymptomatic patients require no treatment. When no deformities are identified, simple mass excision is the treatment choice. If the mass causes epiphyses asymmetry, resection must be combined with osteotomies. The present study reports a case of Trevor disease in a female patient with 12-year follow-up. A general review of Trevor disease was also performed. PMID:25031921

  9. Proximal humeral epiphyseal plate fracture in a collegiate track and field decathlete: a case study.

    PubMed

    Bowman, Thomas G; Palmieri-Smith, Riann

    2008-02-01

    To present the case of an 18-year-old collegiate decathlete with a Salter-Harris type I epiphyseal plate fracture of the proximal humerus. A collegiate decathlete was playing flag football and fell on an outstretched arm. He was taken to the emergency room and diagnosed with a type I epiphyseal plate fracture. AC sprain, dislocation or subluxation, rotator cuff tear, labral tear. Active and passive range of motion exercises were completed after two days of immobilization. He then started strengthening exercises and returned to competitive activity in 10 weeks. Proximal humeral epiphyseal plate fractures are uncommon injuries, especially in athletes over the age of 15. If an accurate diagnosis is made, an appropriate conservative rehabilitation program can be implemented to safely return an athlete to participation without permanent deformity following a type I Salter-Harris fracture.

  10. 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

  11. 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

  12. [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.

  13. 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

  14. 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.

  15. [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.

  16. 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.

  17. [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%.

  18. 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.

  19. Macrocephaly with multiple epiphyseal dysplasia: a second example of Al Gazali-Bakalinova syndrome?

    PubMed

    Karaer, K; Rosti, R O; Torun, D; Sanal, H T; Guran, S

    2012-01-01

    Four individuals from one consanguineous family affected with macrocephaly, multiple epiphyseal dysplasia and distinctive facies were presented by Al Gazali and Bakalinova in 1998 (1) as a distinct clinical entity. To the best of our knowledge, no other similarly affected family has been presented in the literature. Here, we present an affected macrocephalic male, from a consanguineous family, with facial anomalies, cranial MRI findings and multiple epiphyseal dysplasia findings. We believe this is the second family with a similar clinical spectrum and the same inheritance pattern as those of the family presented by Al Gazali and Bakalinova. Pectus carinatum, hip dislocation and a history of prenatal polyhydramnios have been noted as additional findings in our patient.

  20. 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.

  1. The use of microwaves ablation in the treatment of epiphyseal osteoid osteomas.

    PubMed

    Basile, Antonio; Failla, Giovanni; Reforgiato, Angelo; Scavone, Giovanni; Mundo, Elena; Messina, Martina; Caltabiano, Giuseppe; Arena, Francesco; Ricceri, Viola; Scavone, Antonio; Masala, Salvatore

    2014-06-01

    This study was designed to demonstrate the feasibility and the reliability of microwave ablation (MWA) of epiphyseal osteoid osteomas (OO). From February to November 2012, 7 patients (4 males and 3 females; age range 16-30 years) with epiphyseal OOs were treated with MWA. The treatment was performed with 16 G antennas with a power of 20 W for 2 min. The OOs were approached by using coaxial needles inserted with hammer or with automatic drill. All patients underwent spinal anaesthesia, with posttreatment 6-8 h observation before discharging. We treated epiphyseal OOs placed away from nervous and vascular nontarget structures, located in: femoral head (n = 2), femoral lesser trochanter (n = 2), femoral neck (n = 2), and proximal tibial epiphysis (n = 1). CT was used to visualize the nidus and to insert the needle for thermal ablation and for postprocedure control. Technical success was considered the positioning of the antenna in the nidus, while the efficacy of treatment was clinically evaluated as the complete remission of pain after the procedure by using the visual analogue score (VAS). Follow-up was performed by using VAS score 1 day, 1 week, and 1, 3, and 6 months after the procedure, whereas MRI examination was performed immediately after the procedure, at 1 month, and in any case of recurrence. Complications were also recorded. All patients experienced resolution of the symptomatology (VAS = 0) in ~1 week until the last follow-up, with residual VAS < 2 points occurring only from 1 to 7 days after the procedure. No intraprocedural complication was noted, whereas one patient had back pain for 2 months after the procedure, likely due to spinal analgesic injection. In our experience, MWA can be safely performed with excellent results without complications in selected cases of epiphyseal OOs; however, the clinical significance of this report is limited because there were only few patients included in this study. Thus, these data must be confirmed by further and

  2. 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.

  3. 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

  4. [Experimental study of vascularized fibular grafting including the epiphyseal growth plate--autogenous orthotopic grafting].

    PubMed

    Yoshizaki, K

    1984-08-01

    Advancements of microsurgery opened up a new horizon in various facets of surgery. Vascularized bone grafting is one of them and promised viable bone grafting with superb results. Whether this bone grafting along with the epiphyseal growth plate yields continuous bone growth is a matter of great concern and the purpose of the present study. Thirty-nine puppies were used for the experiment, and they were divided into two groups. The proximal portion of the fibula was resected with the popliteal artery and vein including their branches to the epiphysis and the metaphysis, and then, the fibula was returned to the original place and fixed with two Kirschner wires. In group 1, the artery and vein were anastomosed with 10-0 Nylon sutures under operative microscope. In group 2, the bone was treated in a same fashion, but without vascular anastomosis. The roentgenological and histopathological changes were sequentially studied in the both groups. Results were summarized as follows: In group 1, 13 dogs were followed until the epiphyseal closure completed. In two dogs, the treated fibula was of equal length with the non-treated opposite fibula, whereas in five dogs there was slight hypogrowth (average 1.8 mm) and in six dogs there was slight overgrowth (average 2.2 mm). Otherwise, The roentgenological appearance was essentially normal. There was no significant difference in the histopathology of the epiphyseal plates in the treated and the non-treated fibulas. In group 2, all the treated fibulas failed to show epiphyseal growth. Roentgenologically destruction and absorption appeared as early as in the first and second week postoperatively, followed by a fracture line through the metaphysis with displacement and nearly complete absorption of the grafted bone. Histopathological findings consisted of early destruction of the growth plate and early necrosis of the bone marrow as well as the bone trabeculae of the epiphysis, metaphysis and diaphysis. Some reparative new bone

  5. 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

  6. The vascular supply of the chondro-epiphyses of the elbow joint in young swine.

    PubMed Central

    Visco, D M; Van Sickle, D C; Hill, M A; Kincaid, S A

    1989-01-01

    The vasculature of the elbow joint was examined in 39 pigs between one day and 15 weeks of age. Each pig was anaesthetised, exsanguinated and the thoracic limbs were perfused with India ink or a silicone rubber injection compound. The humerus, ulna and radius were dissected free, examined, fixed in formalin or ethyl alcohol, cleared by the modified Spalteholz technique and examined mesoscopically. Features of interest were photographed and then a limb from two pigs in each age group was cut into slabs and examined mesoscopically. The vascular supply of the distal part of the humerus was complex. It was supplied by vessels on both the cranial and caudal aspects and locally each aspect had a dual blood supply. Vessels anastomosed and on the cranial aspect formed a vascular ring. The proximal part of the ulna was supplied by vessels that were on its medial and lateral surfaces. The vessel on the lateral surface continued distally and supplied the lateral aspect of the proximal part of the radius. The proximal part of the radius was also supplied by arteries that were on the cranial and medial surfaces. Blood vessels provided branches to numerous cartilage canals of the articular-epiphyseal cartilage complexes, epiphyseal centres of ossification, and growth plates. The patterns of blood vessels in cartilage canals which were in sagittal or transverse planes were best exemplified by those in the distal part of the humerus. Perforating cartilage canals emerged from the epiphyseal centres of ossification. The pattern of cartilage canals was consistent in a general configuration, but individual variation did occur. Although cartilage canals were abundant in the youngest pigs, with increasing age the distribution of cartilage canals changed and the numbers of cartilage canals decreased. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 (Cont.) Fig. 4 Fig. 5 Fig. 6 Fig. 7 Fig. 8 PMID:2606774

  7. The vascular supply of the chondro-epiphyses of the elbow joint in young swine.

    PubMed

    Visco, D M; Van Sickle, D C; Hill, M A; Kincaid, S A

    1989-04-01

    The vasculature of the elbow joint was examined in 39 pigs between one day and 15 weeks of age. Each pig was anaesthetised, exsanguinated and the thoracic limbs were perfused with India ink or a silicone rubber injection compound. The humerus, ulna and radius were dissected free, examined, fixed in formalin or ethyl alcohol, cleared by the modified Spalteholz technique and examined mesoscopically. Features of interest were photographed and then a limb from two pigs in each age group was cut into slabs and examined mesoscopically. The vascular supply of the distal part of the humerus was complex. It was supplied by vessels on both the cranial and caudal aspects and locally each aspect had a dual blood supply. Vessels anastomosed and on the cranial aspect formed a vascular ring. The proximal part of the ulna was supplied by vessels that were on its medial and lateral surfaces. The vessel on the lateral surface continued distally and supplied the lateral aspect of the proximal part of the radius. The proximal part of the radius was also supplied by arteries that were on the cranial and medial surfaces. Blood vessels provided branches to numerous cartilage canals of the articular-epiphyseal cartilage complexes, epiphyseal centres of ossification, and growth plates. The patterns of blood vessels in cartilage canals which were in sagittal or transverse planes were best exemplified by those in the distal part of the humerus. Perforating cartilage canals emerged from the epiphyseal centres of ossification. The pattern of cartilage canals was consistent in a general configuration, but individual variation did occur. Although cartilage canals were abundant in the youngest pigs, with increasing age the distribution of cartilage canals changed and the numbers of cartilage canals decreased.

  8. 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

  9. 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

  10. 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.

  11. Computer-assisted bone age assessment: image preprocessing and epiphyseal/metaphyseal ROI extraction.

    PubMed

    Pietka, E; Gertych, A; Pospiech, S; Cao, F; Huang, H K; Gilsanz, V

    2001-08-01

    Clinical assessment of skeletal maturity is based on a visual comparison of a left-hand wrist radiograph with atlas patterns. Using a new digital hand atlas an image analysis methodology is being developed. To assist radiologists in bone age estimation. The analysis starts with a preprocessing function yielding epiphyseal/metaphyseal regions of interest (EMROIs). Then, these regions are subjected to a feature extraction function. Accuracy has been measured independently at three stages of the image analysis: detection of phalangeal tip, extraction of the EMROIs, and location of diameters and lower edge of the EMROIs. Extracted features describe the stage of skeletal development more objectively than visual comparison.

  12. [A novel mutation of cartilage oligomeric matrix protein gene underlies multiple epiphyseal dysplasia].

    PubMed

    Wang, Hui; Xie, Jiansheng; Wu, Weiqing; Xu, Zhiyong; Luo, Fuwei; Geng, Qian

    2013-06-01

    To perform mutation analysis for a female with multiple epiphyseal dysplasia (MED) and provide pre-symptomatic and prenatal diagnosis. Mutation screening of cartilage oligomeric matrix protein (COMP) gene was carried out through targeted next-generation DNA sequencing and Sanger sequencing. A novel c.956 A>T resulting in substitution of Aspartic acid 319 for Valine (p.Asp319Val) has been identified in exon 9 of the COMP gene in the patient. As predicted by a SIFT software, above mutation can cause damage to the structure of COMP protein. A novel c.956 A>T substitution mutation has been identified in a patient featuring MED.

  13. 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.

  14. 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

  15. 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

  16. 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.

  17. 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

  18. Ca2+ transport by chondrocyte mitochondria of the epiphyseal growth plate.

    PubMed

    Lee, N H; Shapiro, I M

    1978-07-18

    In a study of the Ca2+ kinetics of mitochondria of chick epiphyseal chondrocytes, the rate of Ca2+ uptake was linear up to a medium Ca2+ concentration of 30 mum. The half maximal transport rate occurred at 34 mum Ca2+. The Ca2+ uptake rate, expressed as a function of time, was 35 nmoles/mg protein/min; the presence of Mg2+ had little effect on Ca2+ accumulation. While these kinetic parameters did not differ significantly from mitochondria of cells of nonmineralizing tissues, the respiratory characteristics of the chondrocyte organelles exhibited functional differences. Thus, up to 350 nmoles Ca2+/mg protein, chondrocyte mitochondria performed coupled oxidative phosphorylation. Calcium uptake was energy supported, while Ca2+ binding was low. Addition of respiratory inhibitors and uncouplers to these mitochondria resulted in a rapid loss of more than 80% of the total Ca2+. The Ca/Pi ratio of the extrudate was very similar to the ratio of the ions in cartilage septum fluid. In the most mineralized zones of the epiphyseal plate, there was little change in the state 4 respiratory rate, but nonspecific Ca2+ binding was elevated and a high percentage of the total Ca2+ was in a nonextrudable form. The results indicate that in cells preparing for mineralization, much of the total mitochondrial Ca2+ is in a form that can be transported to the calcification front. In cells close to the calcification front, nonextrudable Ca2+ may form calcium phosphate granules described by other investigators.

  19. 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.

  20. 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.

  1. 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.

  2. Wide-spread cone-shaped epiphyses in two Saudi siblings with Ellis-van Creveld syndrome.

    PubMed

    Al-Fardan, Abeer; Al-Qattan, Mohammad M

    2017-08-24

    Ellis-van Creveld (EVC) syndrome is one of the rarest ciliopathy syndromes. It is caused by mutations of the EVC and EVC2 genes which encode the EVC proteins present in the basal body of the primary cilium. We report on a Saudi family with two affected children. Gene analysis revealed a homozygous c.2T >A in exon 1 of the EVC gene. The most interesting finding in our patients was the wide - spread cone-shaped epiphyses in the hands and feet. Although cone-shaped epiphyses is a known feature of EVC syndrome, it usually limited to the middle or proximal phalanges. The wide-spread cone-shaped epiphyses seen in our patients have not been previously reported. EVC syndrome is very rare in the Middle East. We report on the first Saudi family with EVC syndrome confirmed by gene analysis. The most unique finding in our patients was the wide-spread cone-shaped epiphyses in the hands and feet. The abnormality is probably related to abnormal Indian hedgehog signaling in the primary cilium. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  3. 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. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  4. 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

  5. Femoral epiphyseal cartilage matrix changes at predilection sites of equine osteochondrosis: Quantitative MRI, second-harmonic microscopy, and histological findings.

    PubMed

    Martel, Gabrielle; Couture, Charles-André; Gilbert, Guillaume; Bancelin, Stéphane; Richard, Hélène; Moser, Thomas; Kiss, Sabrina; Légaré, François; Laverty, Sheila

    2016-10-01

    Osteochondrosis is an ischemic chondronecrosis of epiphyseal growth cartilage that results in focal failure of endochondral ossification and osteochondritis dissecans at specific sites in the epiphyses of humans and animals, including horses. The upstream events leading to the focal ischemia remain unknown. The epiphyseal growth cartilage matrix is composed of proteoglycan and collagen macromolecules and encases its vascular tree in canals. The matrix undergoes major dynamic changes in early life that could weaken it biomechanically and predispose it to focal trauma and vascular failure. Subregions in neonatal foal femoral epiphyses (n = 10 osteochondrosis predisposed; n = 6 control) were assessed for proteoglycan and collagen structure/content employing 3T quantitative MRI (3T qMRI: T1ρ and T2 maps). Site-matched validations were made with histology, immunohistochemistry, and second-harmonic microscopy. Growth cartilage T1ρ and T2 relaxation times were significantly increased (p < 0.002) within the proximal third of the trochlea, a site predisposed to osteochondrosis, when compared with other regions. However, this was observed in both control and osteochondrosis predisposed specimens. Microscopic evaluation of this region revealed an expansive area with low proteoglycan content and a hypertrophic-like appearance on second-harmonic microscopy. We speculate that this matrix structure and composition, though physiological, may weaken the epiphyseal growth cartilage biomechanically in focal regions and could enhance the risk of vascular failure with trauma leading to osteochondrosis. However, additional investigations are now required to confirm this. 3T qMRI will be useful for future non-invasive longitudinal studies to track the osteochondrosis disease trajectory in animals and humans. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 34:1743-1752, 2016. © 2016 Orthopaedic Research Society. Published by Wiley

  6. 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.

  7. 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

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

    PubMed

    Gulabi, Deniz; Erdem, Mehmet; Bulut, Guven; Avci, Cem Coskun; Asci, Murat

    2013-05-31

    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. 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. 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.

  9. Fixation of distal radial epiphyseal fracture: Comparison of K-wire and prebent intramedullary nail.

    PubMed

    Cai, Haoqi; Wang, Zhigang; Cai, Haiqing

    2016-02-01

    To compare the use of crossed K-wire and prebent intramedullary nail techniques for the fixation of distal radius metaphyseal fracture in children. Intraoperative and follow-up data for children with distal radius metaphyseal fracture, treated using crossed K-wire or prebent intramedullary nail fixation, were retrospectively analysed. Patient groups were matched for age, sex and clinical parameters (fracture location, affected side, fracture type). Patients treated using prebent intramedullary nail fixation (n = 52) had significantly shorter surgery duration, fewer intraoperative X-radiographs, and lower prevalence of postoperative redisplacement and malalignment deformity than those treated using crossed K-wire fixation (n = 52). Both techniques resulted in similar postoperative complications and recovery of forearm rotation. Prebent intramedullary nail fixation has a better functional outcome than crossed K-wire fixation in the treatment of distal radial epiphyseal fracture in children. © The Author(s) 2015.

  10. 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.

  11. Age estimation using thoracic and first two lumbar vertebral ring epiphyseal union.

    PubMed

    Albert, Midori; Mulhern, Dawn; Torpey, Melissa A; Boone, Edward

    2010-03-01

    Union of the vertebral centra or "ring" epiphyses occurs during adolescence and early adulthood, providing valuable age at death information. We present a system for estimating age based on the timing and pattern of vertebral ring union. Data from 57 known individuals aged 14-27 years were used to establish age ranges for various patterns of union in females and males. Female age ranges were more well defined with less overlap in patterns of union than male age ranges. The age ranges are accompanied by descriptions of the stages of union observed that aid in applying this method. A test of interobserver error in scoring stages of union demonstrated strong consistency among three observers (r = 0.91-0.97). Estimating age by observing all stages documented resulted in 78%, 88%, and 100% accuracies using vertebral data alone. We encourage the continued use of this method, in conjunction with other age indicators.

  12. 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.

  13. 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

  14. 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.

  15. 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.

  16. 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.

  17. 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

  18. 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.

  19. 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.

  20. 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

  1. 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.

  2. 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.

  3. [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.

  4. Analysis on the risk of contralateral proximal femoral epiphyseal slippage using the modified Oxford score.

    PubMed

    Soni, Jamil Faissal; Valenza, Weverley Rubele; Ueda, Wellington Keity; Schelle, Gisele Cristine; Costa, Anna Carolina Pavelec; Ferraz Faria, Fernando

    2015-01-01

    To determine the application of the modified Oxford score among patients with proximal femoral epiphyseal slippage (PFES) as an aid to indicating prophylactic surgical treatment on the contralateral hip. Retrospective analysis on the medical files of patients attended at the institution where the authors work. From these, patients attended between 2008 and 2011 who presented unilateral PFES and were followed up for a minimum of two years were selected. Patients were excluded if they presented endocrine disease, metabolic disease, Down syndrome or radiographs that were inadequate for determining the modified Oxford score. The initial radiographs received scores ranging from 16 to 26. Statistical analysis was used to determine whether the scoring was predictive of future development of contralateral slippage. Among the 15 patients with unilateral PFES that were selected, five (33.3%) evolved with contralateral slippage. The patients were divided into two groups. Four patients were considered to present risk and three of them developed contralateral slippage. In the group that was considered not to present risk, there were 11 patients and two of these evolved with contralateral slippage. Thus, there was a tendency for the patients in the group that developed the disease to differ from the group that did not develop it, in relation to the risk classification. Although application of the modified Oxford score was not statistically significant in our sample, we noted a tendency toward contralateral slippage among hips with low scores.

  5. 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.

  6. 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.

  7. 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

  8. Automatic Segmentation of Phalanx and Epiphyseal/Metaphyseal Region by Gamma Parameter Enhancement Algorithm

    NASA Astrophysics Data System (ADS)

    Hsieh, C. W.; Chen, C. Y.; Jong, T. L.; Liu, T. C.; Chiu, C. H.

    2012-01-01

    The performance of bone age assessment is highly correlated with the extraction of bony tissue from soft tissues, and the key problem is how to successfully separate epiphyseal/metaphyseal region of interests (EMROIs) from the background and soft tissue. In our experiment, a series of image preprocessing procedures are used to exclude the background and locate the EMROIs of left-hand radiographs. Subsequently, automatic gamma parameter enhancement is applied to test the two segmentation methods (adaptive two-means clustering algorithm and gradient vector flow snake) among children of different age (the age from 2 to 16 years for 80 girls and boys). Four error measurements of misclassification error, relative foreground area error, modified Hausdorff distances, and edge mismatch, are included to evaluate the segmentation performance. The result shows that the two segmentation algorithms are corresponding to different ranges of optimal gamma parameters. Furthermore, the margin of EMROIs can be obtained more precisely by developing an automatic bone age assessment method with the gamma parameter enhancement.

  9. 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.

  10. 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.

  11. 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.

  12. [Metabolism of sex steroid hormones in neuroendocrine organs. I. In vitro androgen metabolism in the hypophyses and epiphyses of rats during the puberal period].

    PubMed

    Degtiar', V G; Pavlinov, S A; Loseva, L A; Isachenkov, V A

    1977-01-01

    Metabolism of tritium-labeled testosterone, delta4-androstane-3, 17-dion, dihydrotestosterone. 5alpha-androstane-3alpha, 17beta-diod and 5alpha-androstane-3beta, 17beta-dion by the isolated hypophyses and epiphyses of male and female 30-day rats was studied. The glands were incubated in Eagle's medium containing the mentioned androgens with the concentration of 1-2.10(-8) M for 90 min at 37 degrees C. As revealed, restoration of testosterone and delta4 androstane-3, 17-dion by 5alpha-reductase occurred in the epiphyses and hypophyses; under conditions of incubation androgen hydroxylation was realized only in the 3alpha- and 17beta-positions. Organ and sexual specificity of the androgen metabolism in the hypophyses and the epiphyses was quantitative in character: the metabolism level of all the androgens was greater in female rats than in the male both in the hypophysis and in the epiphyses; the activity of 5-reductase was lower in the epiphyses of animals of the both sexes than in the hypophyses. It appeared that in the process of biotransformation androgens in the organs under study served as precursors of the polar unidentified metabolites poorly retained by the tissues and eliminated from the organs into the incubation medium during the incubation.

  13. 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.

  14. 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.

  15. 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.

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

    PubMed Central

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

    2015-01-01

    individual epiphyseal plates. PMID:26241802

  17. 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.

  18. 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.

  19. 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

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

    PubMed

    J, Ashish Suthar; V, Ashish Kothari

    2014-01-01

    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. 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. 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 especially growth arrest in asymptomatic patient.

  1. Autosomal Recessive Multiple Epiphyseal Dysplasia in a Korean Girl Caused by Novel Compound Heterozygous Mutations in the DTDST (SLC26A2) Gene

    PubMed Central

    Kim, Ok-Hwa; Lee, Hye-Ran; Shin, Sung Jin; Yoo, Won Joon; Park, Woong Yang; Park, Sung Sup; Cho, Sung Im; Choi, In Ho

    2010-01-01

    Multiple epiphyseal dysplasia is caused by heterogenous genotypes involving more than six genes. Recessive mutations in the DTDST gene cause a phenotype of recessive multiple epiphyseal dysplasia (rMED). The authors report a 9-yr old Korean girl with the rMED phenotype having novel compound heterozygous mutations in the DTDST gene, which were inherited from both parents. This is the first Korean rMED case attributed to DTDST mutations, and expands the spectrum of diseases caused by DTDST mutations. PMID:20592910

  2. Comparative histological study of the effects of high calcium diet and vitamin D supplements on epiphyseal plates of vitamin-D-deficient chicks.

    PubMed

    Jande, S S; Dickson, I R

    1980-01-01

    A comparative histological and microradiographic study of the tibial epiphyseal plates of chickens raised on: (1) a vitamin-D-deficient diet; (2) a vitamin-D-deficient diet supplemented with cholecalciferol, and (3) a vitamin-D-deficient diet to which extra calcium had been added, has revealed that a high-calcium diet did not normalize the epiphyseal plates completely. However, it restored the normal length and chondrocyte arrangement to the proliferative zone. The degenerative zone became elongated and this seems to be related to the hypophosphataemic condition which has developed as a result of the special diet.

  3. Multipurpose contrast enhancement on epiphyseal plates and ossification centers for bone age assessment

    PubMed Central

    2013-01-01

    Background The high variations of background luminance, low contrast and excessively enhanced contrast of hand bone radiograph often impede the bone age assessment rating system in evaluating the degree of epiphyseal plates and ossification centers development. The Global Histogram equalization (GHE) has been the most frequently adopted image contrast enhancement technique but the performance is not satisfying. A brightness and detail preserving histogram equalization method with good contrast enhancement effect has been a goal of much recent research in histogram equalization. Nevertheless, producing a well-balanced histogram equalized radiograph in terms of its brightness preservation, detail preservation and contrast enhancement is deemed to be a daunting task. Method In this paper, we propose a novel framework of histogram equalization with the aim of taking several desirable properties into account, namely the Multipurpose Beta Optimized Bi-Histogram Equalization (MBOBHE). This method performs the histogram optimization separately in both sub-histograms after the segmentation of histogram using an optimized separating point determined based on the regularization function constituted by three components. The result is then assessed by the qualitative and quantitative analysis to evaluate the essential aspects of histogram equalized image using a total of 160 hand radiographs that are implemented in testing and analyses which are acquired from hand bone online database. Result From the qualitative analysis, we found that basic bi-histogram equalizations are not capable of displaying the small features in image due to incorrect selection of separating point by focusing on only certain metric without considering the contrast enhancement and detail preservation. From the quantitative analysis, we found that MBOBHE correlates well with human visual perception, and this improvement shortens the evaluation time taken by inspector in assessing the bone age. Conclusions

  4. 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

  5. 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.

  6. 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

  7. Hawkins Group I fracture of neck of talus and Salter Harris Type III tibial epiphyseal injury of medial malleolus.

    PubMed

    Prasad, Kodali Siva R K; Vali, Hamza; Hussain, Altaf

    2013-01-01

    We are reporting an unusual combination of Hawkins Group I fracture of the neck of left talus in association with Salter Harris Type III distal tibial epiphyseal injury of medial malleolus in a child with cerebral palsy and hemiplegia of contralateral limbs and discussed the possible mechanism as well as management. Fractures of medial malleolus usually occur in Hawkins Group III fracture-dislocations in adults. Forced dorsiflexion of talus against the anterior edge of tibia appears to be the accepted common mechanism, despite limited experimental and clinical evidence incriminating axial compression. Fracture of medial malleolus implicates supination. We managed this unusual pattern of injury conservatively. At 15 months, the child was asymptomatic with no radiological evidence of avascular necrosis of body of talus or growth disturbance of distal tibial epiphysis. Copyright © 2013 Elsevier Ltd. All rights reserved.

  8. 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.

  9. Accuracy of scoring of the epiphyses at the knee joint (SKJ) for assessing legal adult age of 18 years.

    PubMed

    Galić, Ivan; Mihanović, Frane; Giuliodori, Alice; Conforti, Federica; Cingolani, Mariano; Cameriere, Roberto

    2016-07-01

    Important aspects of forensic practice are age estimation and discrimination of individuals of unknown age as adults and minors. The developing knee joint was recognized as a potential site for age examination in late adolescence. We analyzed a sample of anteroposterior x-rays of the knee joints from 446 living individuals from Umbria, Italy (234 males and 212 females), aged between 12 and 26 years. We evaluated the ossification of the distal femoral (DF), proximal tibial (PT), and proximal fibular (PF) epiphyses. We took into account possible persistence of the epiphyseal scars in the ossified epiphyses by the adopted stages of those previously introduced by Cameriere et al. (2012). We also used measurements from all three epiphyses to calculate the total score of maturation for the knee joint (SKJ). Cohen Kappa coefficients of intrarater agreement for staging the DF, PT, and PF epiphyses were 0.839, 0.894, and 0.907, while interrater agreement was 0.919, 0.791, and 0.907, respectively. The resulting receiver operating characteristic (ROC) curves of SKJ show better discriminatory power than those for DF, PT, and PF epiphyses in predicting that the participant, either male or female, was an adult or a minor. The areas under the curves for SKJ were 0.991 and 0.968 vs. 0.944, 0.962, 0.974 and 0.891, 0.910, 0.918 for males and females, respectively. The results of the 2 by 2 contingency tables showed that SKJ score of 4 in males and SKJ score of 5 in females were the most suitable cut-off value in discriminating between adults and minors. Principally, the sensitivity test for males was 0.94, with 95 % confidence interval (95 % CI) 0.90 to 0.97 and specificity was 0.96 (95 % CI 0.91 to 0.98). The proportion of correctly classified individuals was 0.95 (95 % CI 0.91 to 0.97). For females, the sensitivity test was 0.89 (95 % CI 0.84 to 0.92) and specificity was 0.92 (95 % CI 0.87 to 0.96), the proportion of correctly classified individuals was 0.90 (95 % CI 0

  10. 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.

  11. 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.

  12. 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.

  13. Diverse mutations in the gene for cartilage oligomeric matrix protein in the pseudoachondroplasia-multiple epiphyseal dysplasia disease spectrum.

    PubMed Central

    Briggs, M D; Mortier, G R; Cole, W G; King, L M; Golik, S S; Bonaventure, J; Nuytinck, L; De Paepe, A; Leroy, J G; Biesecker, L; Lipson, M; Wilcox, W R; Lachman, R S; Rimoin, D L; Knowlton, R G; Cohn, D H

    1998-01-01

    Pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED) are autosomal dominant osteochondrodysplasias that result in mild to severe short-limb dwarfism and early-onset osteoarthrosis. PSACH and some forms of MED result from mutations in the gene for cartilage oligomeric matrix protein (COMP; OMIM 600310 [http://www3.ncbi.nlm. nih.gov:80/htbin-post/Omim/dispmim?600310]). We report the identification of COMP mutations in an additional 14 families with PSACH or MED phenotypes. Mutations predicted to result in single-amino acid deletions or substitutions, all in the region of the COMP gene encoding the calmodulin-like repeat elements, were identified in patients with moderate to severe PSACH. We also identified within this domain a missense mutation that produced MED Fairbank. In two families, one with mild PSACH and the second with a form of MED, we identified different substitutions for a residue in the carboxyl-terminal globular region of COMP. Both the clinical presentations of these two families and the identification of COMP-gene mutations provide evidence of phenotypic overlap between PSACH and MED. These data also reveal a role for the carboxyl-terminal domain in the structure and/or function of COMP. PMID:9463320

  14. 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.

  15. What Happens to the Articular Surface After Curettage for Epiphyseal Chondroblastoma? A Report on Functional Results, Arthritis, and Arthroplasty.

    PubMed

    Farfalli, Germán L; Slullitel, Pablo A I; Muscolo, D Luis; Ayerza, Miguel A; Aponte-Tinao, Luis A

    2017-03-01

    Chondroblastoma is an uncommon, benign, but locally aggressive bone tumor that occurs in the apophyses or epiphyses of long bones, primarily in young patients. Although some are treated with large resections, aggressive curettage and bone grafting are more commonly performed to preserve the involved joint. Such intralesional resection may result in damage to the growth plate and articular cartilage, which can result in painful arthritis. Prior studies have focused primarily on oncologic outcomes rather than long-term joint status and functional outcomes. (1) What local complications can be expected after aggressive intralesional curettage of epiphyseal chondroblastoma? (2) What is the joint survival of a joint treated in this way for chondroblastoma? (3) What additional procedures are used in treating symptomatic joint osteoarthritis after treatment of the chondroblastoma? (4) What are the functional outcomes in this group of patients? A retrospective study of our prospectively collected database between 1975 and 2013 was done. We found 64 patients with a diagnosis of chondroblastoma of bone. After applying our selection criteria, 53 patients were involved in this study. We excluded seven patients with tumors initially treated with en bloc resection (five located in the extremities and two in the axial skeleton) and two patients with apophyseal tumors. One patient who underwent nonsurgical treatment and one patient lost to followup were also excluded. The mean age was 18 years (range, 11-39 years); the minimum followup was 2 years with a mean followup 77 months (range, 24-213 months). We analyzed all patients with a diagnosis of epiphyseal chondroblastoma of the limb treated with aggressive curettage and joint preservation surgery. During the period in question, our general indications for curettage were patients with active, painful tumors and those with more aggressive ones that remained intracompartmental, whereas initial wide en bloc resection was indicated in

  16. 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.

  17. 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.

  18. 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

  19. 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.

  20. 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.

  1. Cartilage calcification: an ultrastructural, histochemical, and analytical x-ray microprobe study of the zone of calcification in the normal avian epiphyseal growth plate

    SciTech Connect

    Davis, W.L.; Jones, R.G.; Knight, J.P.; Hagler, H.K.

    1982-03-01

    Sections from the zone of calcification of ruthenium red-fixed normal avian epiphyseal growth plates were analyzed by various morphological (histochemical) and analytical techniques. Calcium and phosphorus were identified in the chondrocyte pericellular rim, the uncalcified extracellular (territorial) matrix, and in both the peripheral and central aspects of the calcified accumulations within extracellular matrix. Cartilage proteoglycan, as determined by ruthenium binding, positive staining with acidic phosphotungstic acid, and the X-ray spectroscopic detection of sulfur, was identified in the same four zones. Thus, it appears that proteoglycans, in some form, are indeed retained at sites of biological calcification. Additionally, these macromolecules, synthesized in chondrocytes, may be involved in extracellular calcium translocation.

  2. 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.

  3. 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

  4. 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.

  5. 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.

  6. The Femoro-Epiphyseal Acetabular Roof (FEAR) Index: A New Measurement Associated With Instability in Borderline Hip Dysplasia?

    PubMed

    Wyatt, Michael; Weidner, Jan; Pfluger, Dominik; Beck, Martin

    2017-03-01

    The definition of osseous instability in radiographic borderline dysplastic hips is difficult. A reliable radiographic tool that aids decision-making-specifically, a tool that might be associated with instability-therefore would be very helpful for this group of patients. (1) To compare a new radiographic measurement, which we call the Femoro-Epiphyseal Acetabular Roof (FEAR) index, with the lateral center-edge angle (LCEA) and acetabular index (AI), with respect to intra- and interobserver reliability; (2) to correlate AI, neck-shaft angle, LCEA, iliocapsularis volume, femoral antetorsion, and FEAR index with the surgical treatment received in stable and unstable borderline dysplastic hips; and (3) to assess whether the FEAR index is associated clinical instability in borderline dysplastic hips. We defined and validated the FEAR index in 10 standardized radiographs of asymptomatic controls using two blinded independent observers. Interrater and intrarater coefficients were calculated, supplemented by Bland-Altman plots. We compared its reliability with LCEA and AI. We performed a case-control study using standardized radiographs of 39 surgically treated symptomatic borderline radiographically dysplastic hips and 20 age-matched controls with asymptomatic hips (a 2:1 ratio), the latter were patients attending our institution for trauma unrelated to their hips but who had standardized pelvic radiographs between January 1, 2016 and March 1, 2016. Patient demographics were assessed using univariate Wilcoxon two-sample tests. There was no difference in mean age (overall: 31.5 ± 11.8 years [95% CI, 27.7-35.4 years]; stable borderline group: mean, 32.1± 13.3 years [95% CI, 25.5-38.7 years]; unstable borderline group: mean, 31.1 ± 10.7 years [95% CI, 26.2-35.9 years]; p = 0.96) among study groups. Treatment received was either a periacetabular osteotomy (if the hip was unstable) or, for patients with femoroacetabular impingement, either an open or arthroscopic

  7. 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.

  8. 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

  9. COMP mutation screening as an aid for the clinical diagnosis and counselling of patients with a suspected diagnosis of pseudoachondroplasia or multiple epiphyseal dysplasia

    PubMed Central

    Kennedy, Jason; Jackson, Gail; Ramsden, Simon; Taylor, Jacky; Newman, William; Wright, Michael J; Donnai, Dian; Elles, Rob; Briggs, Michael D

    2009-01-01

    The skeletal dysplasias are a clinically and genetically heterogeneous group of conditions affecting the development of the osseous skeleton and fall into the category of rare genetic diseases in which the diagnosis can be difficult for the nonexpert. Two such diseases are pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED), which result in varying degrees of short stature, joint pain and stiffness and often resulting in early onset osteoarthritis. PSACH and some forms of MED result from mutations in the cartilage oligomeric matrix protein (COMP) gene and to aid the clinical diagnosis and counselling of patients with a suspected diagnosis of PSACH or MED, we developed an efficient and accurate molecular diagnostic service for the COMP gene. In a 36-month period, 100 families were screened for a mutation in COMP and we identified disease-causing mutations in 78% of PSACH families and 36% of MED families. Furthermore, in several of these families, the identification of a disease-causing mutation provided information that was immediately used to direct reproductive decision-making. PMID:15756302

  10. Epiphyseal abnormalities, trabecular bone loss and articular chondrocyte hypertrophy develop in the long bones of postnatal Ext1-deficient mice1

    PubMed Central

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

    2014-01-01

    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 Ext1f/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 2 weeks 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 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, osteoarthritis and HME. PMID:23958822

  11. 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. Copyright © 2016 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

  12. 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.

  13. 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

  14. Intermittent Internal Fixation With a Locking Plate to Preserve Epiphyseal Growth Function During Limb-Salvage Surgery in a Child With Osteosarcoma of the Distal Femur

    PubMed Central

    Mei, Jiong; Ni, Ming; Jia, Guang-Yao; Chen, Yan-Xi; Zhu, Xiao-Zhong

    2015-01-01

    Abstract Limb shortening is a problem associated with surgery for osteosarcoma of the lower extremity in adolescents, as the tumors frequently occur near the epiphysis. Herein we report the use of a less invasive stabilization system (LISS) and an intermittent fixation method to preserve the growth function of epiphysis in an 11-year-old patient with an osteosarcoma of the distal femur. The 11-year-old male presented with left knee enlargement and pain for 2 weeks, and magnetic resonance imaging (MRI) and biopsy were consistent with osteosarcoma of the left distal femur. After preoperative chemotherapy, en bloc tumor resection was performed with margins based on MRI findings preserving the epiphyseal growth plate, the tumor cavity was filled with inactivated bone and bone cement, and a LISS was used to stabilize the femur. Aggressive postoperative chemotherapy was given. Approximately 105 weeks after surgery radiography showed that the distal end of the plate had moved superior to the epiphysis along with bone growth. Locking screws were placed in the distal part of the LISS plate to stabilize the re-implanted bone, and external fixation was not needed. The patient was able to walk with the crutches 1 week postoperatively, and bear weight on the extremity 6 weeks postoperatively. At 6 years after surgery, the patient's height had increased 52 cm, shortening of the affected limb was only 1 cm, and the circumference of the affected limb was 2 cm smaller than that of the contralateral limb. There was no significant discomfort in the affected limb, and there was no gait abnormality. The patient could jump and run, and could participate in sports including basketball and badminton to the same degree as his peers. In summary, the novel method of bone reconstruction and fixation provided good results in a child with an osteosarcoma of the distal femur. This fixation method preserves the osteogenic function of the epiphysis and restored bone integrity simultaneously

  15. A novel mutation in DDR2 causing spondylo-meta-epiphyseal dysplasia with short limbs and abnormal calcifications (SMED-SL) results in defective intra-cellular trafficking

    PubMed Central

    2014-01-01

    Background The rare autosomal genetic disorder, Spondylo-meta-epiphyseal dysplasia with short limbs and abnormal calcifications (SMED-SL), is reported to be caused by missense or splice site mutations in the human discoidin domain receptor 2 (DDR2) gene. Previously our group has established that trafficking defects and loss of ligand binding are the underlying cellular mechanisms of several SMED-SL causing mutations. Here we report the clinical characteristics of two siblings of consanguineous marriage with suspected SMED-SL and identification of a novel disease-causing mutation in the DDR2 gene. Methods Clinical evaluation and radiography were performed to evaluate the patients. All the coding exons and splice sites of the DDR2 gene were sequenced by Sanger sequencing. Subcellular localization of the mutated DDR2 protein was determined by confocal microscopy, deglycosylation assay and Western blotting. DDR2 activity was measured by collagen activation and Western analysis. Results In addition to the typical features of SMED-SL, one of the patients has an eye phenotype including visual impairment due to optic atrophy. DNA sequencing revealed a novel homozygous dinucleotide deletion mutation (c.2468_2469delCT) on exon 18 of the DDR2 gene in both patients. The mutation resulted in a frameshift leading to an amino acid change at position S823 and a predicted premature termination of translation (p.S823Cfs*2). Subcellular localization of the mutant protein was analyzed in mammalian cell lines, and it was found to be largely retained in the endoplasmic reticulum (ER), which was further supported by its N-glycosylation profile. In keeping with its cellular mis-localization, the mutant protein was found to be deficient in collagen-induced receptor activation, suggesting protein trafficking defects as the major cellular mechanism underlying the loss of DDR2 function in our patients. Conclusions Our results indicate that the novel mutation results in defective trafficking

  16. A novel mutation in DDR2 causing spondylo-meta-epiphyseal dysplasia with short limbs and abnormal calcifications (SMED-SL) results in defective intra-cellular trafficking.

    PubMed

    Al-Kindi, Adila; Kizhakkedath, Praseetha; Xu, Huifang; John, Anne; Sayegh, Abeer Al; Ganesh, Anuradha; Al-Awadi, Maha; Al-Anbouri, Lamya; Al-Gazali, Lihadh; Leitinger, Birgit; Ali, Bassam R

    2014-04-11

    The rare autosomal genetic disorder, Spondylo-meta-epiphyseal dysplasia with short limbs and abnormal calcifications (SMED-SL), is reported to be caused by missense or splice site mutations in the human discoidin domain receptor 2 (DDR2) gene. Previously our group has established that trafficking defects and loss of ligand binding are the underlying cellular mechanisms of several SMED-SL causing mutations. Here we report the clinical characteristics of two siblings of consanguineous marriage with suspected SMED-SL and identification of a novel disease-causing mutation in the DDR2 gene. Clinical evaluation and radiography were performed to evaluate the patients. All the coding exons and splice sites of the DDR2 gene were sequenced by Sanger sequencing. Subcellular localization of the mutated DDR2 protein was determined by confocal microscopy, deglycosylation assay and Western blotting. DDR2 activity was measured by collagen activation and Western analysis. In addition to the typical features of SMED-SL, one of the patients has an eye phenotype including visual impairment due to optic atrophy. DNA sequencing revealed a novel homozygous dinucleotide deletion mutation (c.2468_2469delCT) on exon 18 of the DDR2 gene in both patients. The mutation resulted in a frameshift leading to an amino acid change at position S823 and a predicted premature termination of translation (p.S823Cfs*2). Subcellular localization of the mutant protein was analyzed in mammalian cell lines, and it was found to be largely retained in the endoplasmic reticulum (ER), which was further supported by its N-glycosylation profile. In keeping with its cellular mis-localization, the mutant protein was found to be deficient in collagen-induced receptor activation, suggesting protein trafficking defects as the major cellular mechanism underlying the loss of DDR2 function in our patients. Our results indicate that the novel mutation results in defective trafficking of the DDR2 protein leading to loss of

  17. 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.

  18. 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

  19. Revision pediatric anterior cruciate ligament reconstruction after failure of iliotibial band technique treated with all-epiphyseal technique in a prepubescent with Ehlers-Danlos syndrome: a case report.

    PubMed

    Erdman, Mary K; Warnick, Drew E

    2017-09-01

    The increasing number of pediatric anterior cruciate ligament (ACL) injuries and the failure of nonoperative management of these patients has elicited a concordant response in the surgical community to devise reconstruction techniques that stabilize the knee joint without causing iatrogenic growth disturbances. Likewise, the rise in pediatric ACL reconstructions forecasts a consequent rise in revision ACL reconstructions in patients with open physes. In this case report, we describe a prepubescent with Ehlers-Danlos syndrome and congenital ACL deficiency, who underwent physeal-sparing iliotibial band ACL reconstruction and revision to an all-epiphyseal ACL reconstruction with allograft. We also underscore the lack of evidence addressing graft choice and outcomes for ACL reconstructions in patients with connective tissue disorders.

  20. Sequential MRI Study of Graft Integrity and Signal Following Pediatric All-epiphyseal ACL Reconstruction: Does the “Sharp Turn” at the Socket of the Distal Femoral Aperture Matter?

    PubMed Central

    Tuca, Maria; Greditzer, Harry Gus; Gausden, Elizabeth Bishop; Uppstrom, Tyler J.; Potter, Hollis G.; Cordasco, Frank A.; Green, Daniel W.

    2017-01-01

    Objectives: To analyze graft structure and signal with particular emphasis on the distal femoral socket aperture following all-epiphyseal ACLR using hamstring autografts with sequential MRI in skeletally immature athletes. Methods: Retrospective cohort study of 23 skeletally immature patients who underwent ACLR by the same surgical team at a tertiary center during 2011-2013. Athletes had at least two follow-up MRIs, the first MRI 6-12 months after surgery and the second MRI >18 months, were included. Exclusion criteria included those athletes with inMRI follow-up (6) or with a failure of their reconstructions (1). All athletes were treated with an arthroscopic all-inside, all-epiphyseal ACLR, using hamstring autograft, secured with adjustable loop cortical buttons on both tibia and femur. MRI images were analyzed independently and blinded by an orthopaedic surgery fellow and a musculoskeletal radiology fellow. Using a GE Functional Analysis Software, the signal intensity (SI) of the graft was measured in 5 different locations: 1) femoral tunnel, 2) intra-articular proximal turn, 3) midsubstance, 4) intra-articular distal turn, and 5) tibial tunnel. Values were normalized to cortical bone density. The amount of perigraft scarring and synovitis was analyzed. An intraclass correlation coefficient was used to quantify inter-rater reliability, non-parametric Wilcoxon test for perigraft scarring and synovitis, one-way ANOVA to test if significant differences of SI were seen between the different graft locations, and a 2-tailed student t-test for SI changes from 1st to 2nd MRI. Results: The study included 16 patients (5 girls and 11 boys), with an average age at surgery of 11.9 years (range 10-15). The first follow-up MRI was on average at 8.4 months (range 6-12 months), while the 2nd MRI was on average 30.7 months (range 18-40) after surgery. Intra-class correlation coefficients were above 0.7 for all measurements, indicating an excellent concordance between observers

  1. 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.

  2. 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.

  3. 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.

  4. Pseudoachondroplasia and Multiple Epiphyseal Dysplasia: A 7-Year Comprehensive Analysis of the Known Disease Genes Identify Novel and Recurrent Mutations and Provides an Accurate Assessment of Their Relative Contribution

    PubMed Central

    Jackson, Gail C; Mittaz-Crettol, Laureane; Taylor, Jacqueline A; Mortier, Geert R; Spranger, Juergen; Zabel, Bernhard; Le Merrer, Martine; Cormier-Daire, Valerie; Hall, Christine M; Offiah, Amaka; Wright, Michael J; Savarirayan, Ravi; Nishimura, Gen; Ramsden, Simon C; Elles, Rob; Bonafe, Luisa; Superti-Furga, Andrea; Unger, Sheila; Zankl, Andreas; Briggs, Michael D

    2012-01-01

    Pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED) are relatively common skeletal dysplasias resulting in short-limbed dwarfism, joint pain, and stiffness. PSACH and the largest proportion of autosomal dominant MED (AD-MED) results from mutations in cartilage oligomeric matrix protein (COMP); however, AD-MED is genetically heterogenous and can also result from mutations in matrilin-3 (MATN3) and type IX collagen (COL9A1, COL9A2, and COL9A3). In contrast, autosomal recessive MED (rMED) appears to result exclusively from mutations in sulphate transporter solute carrier family 26 (SLC26A2). The diagnosis of PSACH and MED can be difficult for the nonexpert due to various complications and similarities with other related diseases and often mutation analysis is requested to either confirm or exclude the diagnosis. Since 2003, the European Skeletal Dysplasia Network (ESDN) has used an on-line review system to efficiently diagnose cases referred to the network prior to mutation analysis. In this study, we present the molecular findings in 130 patients referred to ESDN, which includes the identification of novel and recurrent mutations in over 100 patients. Furthermore, this study provides the first indication of the relative contribution of each gene and confirms that they account for the majority of PSACH and MED. Hum Mutat 33:144–157, 2012. © 2011 Wiley Periodicals, Inc. PMID:21922596

  5. The High Risk of Infection With Delayed Treatment of Open Seymour Fractures: Salter-Harris I/II or Juxta-epiphyseal Fractures of the Distal Phalanx With Associated Nailbed Laceration.

    PubMed

    Reyes, Bryan A; Ho, Christine A

    2017-06-01

    To describe treatment methods and complication rates of all open Seymour fractures (Salter-Harris I/II or juxta-epiphyseal fractures of the distal phalanx of the hand with associated nailbed laceration) treated at or referred to a pediatric level 1 trauma center over a 10-year time period. We hypothesized that delayed treatment of Seymour fractures results in higher infectious complication rates. We identified all patients treated for open Seymour fractures at the orthopaedic hand clinic at our level 1 pediatric trauma center between August 2002 and December 2012. All charts were reviewed retrospectively. Patients were divided into groups based on timing and completeness of treatment. "Appropriate" treatment was defined as irrigation and debridement, fracture reduction, and antibiotic administration. "Partial" treatment was defined as any type of incomplete treatment; "Acute" treatment was defined as management within 24 hours of the injury; and "Delayed" as having received treatment after 24 hours from the time of injury. Statistical comparisons were performed using the Fisher exact test. A total of 34 patients treated for 35 Seymour fractures met the inclusion criteria. In total, 31% (11/35) received acute, appropriate treatment, 37% (13/35) received acute, partial treatment, and 31% (11/35) received delayed treatment. There were 7 infectious complications: 2 superficial and 5 osteomyelitis. No infections occurred in the acutely, appropriately treated group (infection rate 0%, 0/11), 2 occurred in the acutely, partially treated group (15%, 2/13), and 5 occurred in the delayed treatment group (45%, 5/11). The timing and quality of treatment of open Seymour fractures significantly influences infection rates. This study highlights the importance of timely and appropriate treatment of this seemingly innocuous fracture. Level III-retrospective study, therapeutic.

  6. Recessive multiple epiphyseal dysplasia (rMED) with homozygosity for C653S mutation in the DTDST gene - Phenotype, molecular diagnosis and surgical treatment of habitual dislocation of multilayered patella: Case report

    PubMed Central

    2010-01-01

    Background Multiple epiphyseal dysplasia (MED) is one of the more common generalised skeletal dysplasias. Due to its clinical heterogeneity diagnosis may be difficult. Mutations of at least six separate genes can cause MED. Joint deformities, joint pain and gait disorders are common symptoms. Case Presentation We report on a 27-year-old male patient suffering from clinical symptoms of autosomal recessive MED with habitual dislocation of a multilayered patella on both sides, on the surgical treatment and on short-term clinical outcome. Clinical findings were: bilateral hip and knee pain, instability of femorotibial and patellofemoral joints with habitual patella dislocation on both sides, contractures of hip, elbow and second metacarpophalangeal joints. Main radiographic findings were: bilateral dislocated multilayered patella, dysplastic medial tibial plateaus, deformity of both femoral heads and osteoarthritis of the hip joints, and deformity of both radial heads. In the molecular genetic analysis, the DTDST mutation g.1984T > A (p.C653S) was found at the homozygote state. Carrier status was confirmed in the DNA of the patient's parents. The mutation could be considered to be the reason for the patient's disease. Surgical treatment of habitual patella dislocation with medialisation of the tibial tuberosity led to an excellent clinical outcome. Conclusions The knowledge of different phenotypes of skeletal dysplasias helps to select genes for genetic analysis. Compared to other DTDST mutations, this is a rather mild phenotype. Molecular diagnosis is important for genetic counselling and for an accurate prognosis. Even in case of a multilayered patella in MED, habitual patella dislocation could be managed successfully by medialisation of the tibial tuberosity. PMID:20525296

  7. 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.

  8. Intermittent internal fixation with a locking plate to preserve epiphyseal growth function during limb-salvage surgery in a child with osteosarcoma of the distal femur: a case report.

    PubMed

    Mei, Jiong; Ni, Ming; Jia, Guang-Yao; Chen, Yan-Xi; Zhu, Xiao-Zhong

    2015-05-01

    Limb shortening is a problem associated with surgery for osteosarcoma of the lower extremity in adolescents, as the tumors frequently occur near the epiphysis. Herein we report the use of a less invasive stabilization system (LISS) and an intermittent fixation method to preserve the growth function of epiphysis in an 11-year-old patient with an osteosarcoma of the distal femur.The 11-year-old male presented with left knee enlargement and pain for 2 weeks, and magnetic resonance imaging (MRI) and biopsy were consistent with osteosarcoma of the left distal femur. After preoperative chemotherapy, en bloc tumor resection was performed with margins based on MRI findings preserving the epiphyseal growth plate, the tumor cavity was filled with inactivated bone and bone cement, and a LISS was used to stabilize the femur. Aggressive postoperative chemotherapy was given. Approximately 105 weeks after surgery radiography showed that the distal end of the plate had moved superior to the epiphysis along with bone growth. Locking screws were placed in the distal part of the LISS plate to stabilize the re-implanted bone, and external fixation was not needed.The patient was able to walk with the crutches 1 week postoperatively, and bear weight on the extremity 6 weeks postoperatively. At 6 years after surgery, the patient's height had increased 52 cm, shortening of the affected limb was only 1 cm, and the circumference of the affected limb was 2 cm smaller than that of the contralateral limb. There was no significant discomfort in the affected limb, and there was no gait abnormality. The patient could jump and run, and could participate in sports including basketball and badminton to the same degree as his peers.In summary, the novel method of bone reconstruction and fixation provided good results in a child with an osteosarcoma of the distal femur. This fixation method preserves the osteogenic function of the epiphysis and restored bone integrity simultaneously, and

  9. 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.

  10. 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.

  11. 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.

  12. An uncommon Salter-Harris I fracture of the distal femoral physis with marked epiphyseal dislocation.

    PubMed

    Hale, Christopher; Forstater, Alan; O'Malley, Gerald

    2013-02-01

    This is a report of an uncommon Salter-Harris I fracture of the distal femoral physis with complete dislocation of the distal epiphysis. These fractures are most frequently sustained during sports injuries, with motor vehicle accidents as another leading cause. They are at high risk of nonanatomic healing with subsequent varus or valgus deformity or premature growth arrest of the physis with subsequent leg length difference. Although certain fractures of the distal femoral physis may be very rarely encountered in the emergency department, the unavailability of appropriate consult service or risk of vascular compromise may necessitate reduction by the emergency physician. This fracture-dislocation was reduced with intravenous opioid analgesia, gentle traction along the axis of the lower extremity, and firm but gentle manual reduction of the proximal femoral fragment. External casts alone have a high rate of failure in sustaining anatomic alignment, so percutaneous pinning is recommended as the definitive treatment. After reduction, this patient was transferred to a pediatric hospital capable of performing the necessary operative fixation.

  13. Osteochondrosis and epiphyseal bone abnormalities associated with copper deficiency in bison calves.

    PubMed Central

    Woodbury, M R; Feist, M S; Clark, E G; Haigh, J C

    1999-01-01

    Two bison calves were submitted to the Western College of Veterinary Medicine to confirm suspected copper deficiency. In addition to clinical signs, there were pathologic changes in the cartilage and subchondral bone of several joints. Water analysis indicated high levels of sulfate in the drinking water, contributing to a secondary copper deficiency. Images Figure 1. Figure 2. PMID:10646064

  14. All-epiphyseal semitendinosus PCL reconstruction in a 10-year-old child.

    PubMed

    Warme, Winston J; Mickelson, Dayne

    2010-01-01

    Tears of the posterior cruciate ligament (PCL) in pediatric patients, especially avulsions from its tibial insertion, are not frequently encountered by physicians. However, with an increasing incidence of ligamentous injuries to the knee in skeletally immature patients, orthopaedic surgeons will more frequently need to decide how best to manage a PCL injury in children with open physes. A 10-year-old boy sustained an avulsion of the PCL from its tibial insertion site after a bicycle motocross racing accident. He required a PCL reconstruction after failed conservative treatment and an unsuccessful attempt at primary repair. This successful physeal sparing reconstruction was accomplished using a modified method of femoral tunnel placement in combination with the tibial inlay technique. A magnetic resonance imaging was acquired 2 years postoperatively when physical examination demonstrated both legs of equal length, no varus or valgus deformity, and a normal posterior drawer examination. Four years after the reconstruction, he continues to bicycle motocross race while experiencing no further symptoms and complete restoration to his prior quality of life. The use of the tibial inlay technique together with the modified femoral tunnel location prevented transphyseal drilling while attaining a favorable anatomic placement of the graft while avoiding the "killer" turn associated with the transtibial approach. Level IV (Therapeutic Study).

  15. 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.

  16. Susceptibility Weighted Imaging of Cartilage Canals in Porcine Epiphyseal Growth Cartilage Ex Vivo and In Vivo

    PubMed Central

    Nissi, Mikko J.; Toth, Ferenc; Zhang, Jinjin; Schmitter, Sebastian; Benson, Michael; Carlson, Cathy S.; Ellermann, Jutta M.

    2014-01-01

    Purpose High-resolution visualization of cartilage canals has been restricted to histological methods and contrast-enhanced imaging. In this study, the feasibility of non-contrast-enhanced susceptibility weighted imaging (SWI) for visualization of the cartilage canals was investigated ex vivo at 9.4 T, further explored at 7 and 3 T and demonstrated in vivo at 7 T, using a porcine animal model. Methods SWI scans of specimens of distal femur and humerus from 1 to 8 week-old piglets were conducted at 9.4 T using 3D-GRE sequence and SWI post-processing. The stifle joints of a 2-week old piglet were scanned ex vivo at 7 and 3 T. Finally, the same sites of a 3-week-old piglet were scanned, in vivo, at 7 T under general anesthesia using the vendor-provided sequences. Results High-contrast visualization of the cartilage canals was obtained ex vivo, especially at higher field strengths; the results were confirmed histologically. In vivo feasibility was demonstrated at 7 T and comparison of ex vivo scans at 3 and 7 T indicated feasibility of using SWI at 3 T. Conclusions High-resolution 3D visualization of cartilage canals was demonstrated using SWI. This demonstration of fully noninvasive visualization opens new avenues to explore skeletal maturation and the role of vascular supply for diseases such as osteochondrosis. PMID:23857631

  17. Acute Posterior Shoulder Dislocation with Reverse Hill-Sachs Lesion of the Epiphyseal Humeral Head

    PubMed Central

    Patrizio, Luigi; Sabetta, Ettore

    2011-01-01

    Posterior dislocation of the shoulder is an unfrequent event that often occurs as a consequence of a direct trauma or epileptic crisis. Frequently the posterior dislocations are misunderstood, so they become chronic lesions. We reported a case of an acute posterior left shoulder dislocation with lesser tuberosity fracture and reverse Hill-Sachs lesions which involved more than 25% of the articular surface of the humeral head, in a 57-old-year man with right hemiparesis. We performed a synthesis of the lesser tuberosity with a screw, and we restored the shape of the humeral head with allograft. We achieved a good result that allows the patient to be able to do his previous activities of daily living. PMID:22084780

  18. Acute posterior shoulder dislocation with reverse hill-sachs lesion of the epiphyseal humeral head.

    PubMed

    Patrizio, Luigi; Sabetta, Ettore

    2011-01-01

    Posterior dislocation of the shoulder is an unfrequent event that often occurs as a consequence of a direct trauma or epileptic crisis. Frequently the posterior dislocations are misunderstood, so they become chronic lesions. We reported a case of an acute posterior left shoulder dislocation with lesser tuberosity fracture and reverse Hill-Sachs lesions which involved more than 25% of the articular surface of the humeral head, in a 57-old-year man with right hemiparesis. We performed a synthesis of the lesser tuberosity with a screw, and we restored the shape of the humeral head with allograft. We achieved a good result that allows the patient to be able to do his previous activities of daily living.

  19. 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. © 2016 Wiley Periodicals, Inc.

  20. Severity of Asynchronous Slipped Capital Femoral Epiphyses in Skeletally Immature Versus More Skeletally Mature Patients.

    PubMed

    Nowicki, Philip D; Silva, Selina; Toelle, Lisa; Strohmeyer, Greg; Wahlquist, Trevor; Li, Ying; Farley, Frances A; Caird, Michelle S

    2017-01-01

    Routine prophylactic screw fixation for skeletally immature patients with slipped capital femoral epiphysis (SCFE) continues to be debated. The purpose of this study was to assess the slip severity of a second SCFE in skeletally immature versus more mature patients and determine necessity of contralateral hip prophylactic screw fixation. All patients treated for SCFE at 3 pediatric hospitals over a 10-year time period (January 1, 2002 to December 31, 2011) were evaluated. Patients were included if they had a unilateral SCFE and a contralateral asynchronous SCFE, and were divided into immature (Oxford triradiate score 1) versus more mature (Oxford triradiate score 2 and 3) groups. Data evaluation included age, time between slips, body mass index, Southwick angles of first then second SCFEs, and follow-up duration. There were a total of 45 patients: 16 patients in the skeletally immature and 29 patients in the more mature group. Average age at first SCFE in immature patients was 10.9 years and in more mature patients 12.1 years (P=0.70). Age at second SCFE in immature patients was 11.5 years and in more mature patients 13.0 years (P=0.023). Average time between SCFEs was 6.6 months for immature and 11.4 months for more mature patients (P=0.093). Southwick angles for immature patient first and second SCFEs were 25 and 12.9 degrees, respectively, and for more mature patient first and second SCFEs were 31 and 21 degrees, respectively. Southwick angles were higher at first and second slips in the more mature group, significant only at the second slip (P=0.032). SCFE severity at initial event was predictive of severity of second SCFE regardless of maturity (P=0.043). Regression analysis of slip severity against multiple patient factors demonstrated triradiate score was not a factor assessing subsequent SCFE magnitude (P=0.099). There was no significant difference between first and second SCFEs regardless of skeletal maturity but severity of initial SCFE did correlate with severity of the second SCFE. Deciding not to prophylactically pin an unaffected hip does not lead to worse deformity if a second SCFE occurs in skeletally immature or more mature patients, unless the initial event is severe. Prophylactic pin fixation in skeletally immature patients should occur as a shared decision between patient, guardians, and treating surgeon. Level III-retrospective comparative study.

  1. 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.

  2. 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.

  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. 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.

  5. 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.

  6. Palmitoyl acyltransferase, Zdhhc13, facilitates bone mass acquisition by regulating postnatal epiphyseal development and endochondral ossification: a mouse model.

    PubMed

    Song, I-Wen; Li, Wei-Ru; Chen, Li-Ying; Shen, Li-Fen; Liu, Kai-Ming; Yen, Jeffrey J Y; Chen, Yi-Ju; Chen, Yu-Ju; Kraus, Virginia Byers; Wu, Jer-Yuarn; Lee, M T Michael; Chen, Yuan-Tsong

    2014-01-01

    ZDHHC13 is a member of DHHC-containing palmitoyl acyltransferases (PATs) family of enzymes. It functions by post-translationally adding 16-carbon palmitate to proteins through a thioester linkage. We have previously shown that mice carrying a recessive Zdhhc13 nonsense mutation causing a Zdhcc13 deficiency develop alopecia, amyloidosis and osteoporosis. Our goal was to investigate the pathogenic mechanism of osteoporosis in the context of this mutation in mice. Body size, skeletal structure and trabecular bone were similar in Zdhhc13 WT and mutant mice at birth. Growth retardation and delayed secondary ossification center formation were first observed at day 10 and at 4 weeks of age, disorganization in growth plate structure and osteoporosis became evident in mutant mice. Serial microCT from 4-20 week-olds revealed that Zdhhc13 mutant mice had reduced bone mineral density. Through co-immunoprecipitation and acyl-biotin exchange, MT1-MMP was identified as a direct substrate of ZDHHC13. In cells, reduction of MT1-MMP palmitoylation affected its subcellular distribution and was associated with decreased VEGF and osteocalcin expression in chondrocytes and osteoblasts. In Zdhhc13 mutant mice epiphysis where MT1-MMP was under palmitoylated, VEGF in hypertrophic chondrocytes and osteocalcin at the cartilage-bone interface were reduced based on immunohistochemical analyses. Our results suggest that Zdhhc13 is a novel regulator of postnatal skeletal development and bone mass acquisition. To our knowledge, these are the first data to suggest that ZDHHC13-mediated MT1-MMP palmitoylation is a key modulator of bone homeostasis. These data may provide novel insights into the role of palmitoylation in the pathogenesis of human osteoporosis.

  7. 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.

  8. 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.

  9. 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.

  10. 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. Copyright © 2015 Elsevier Ltd. All rights reserved.

  11. 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.

  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. © 2014 Orthopaedic Research Society. Published by Wiley Periodicals, Inc.

  13. 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.

  14. Whole-exome sequencing reveals a novel COL2A1 mutation in a patient with spondylo-epiphyseal dysplasia congenita.

    PubMed

    Sangsin, A; Srichomthong, C; Pongpanich, M; Suphapeetiporn, K; Shotelersuk, V

    2016-03-11

    Skeletal dysplasia is a group of disorders with more than 450 entities, many of which cannot be differentiated, especially during infancy, but could lead to different clinical courses and prognoses. In this study, we have described a case of a Thai infant with short stature, flat face, pectus carinatum, indirect inguinal hernia, platyspondyly, and generalized delayed endochondral ossification. Using whole-exome sequencing (WES), we successfully identified a de novo heterozygous mutation, c.2024G>A (p.G675D), in the COL2A1 gene, which, to our knowledge, has not been previously reported. These molecular findings helped provide a definite diagnosis of spondyloepiphyseal dysplasia congenita, aiding in proper management of the disease and improved genetic counseling. We demonstrated that WES is an efficient and cost-effective tool for molecular diagnosis for a type II collagenopathy.

  15. 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.

  16. 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.

  17. Repopulation of laser-perforated chondroepiphyseal matrix with xenogeneic chondrocytes: An experimental model

    SciTech Connect

    Caruso, E.M.; Lewandrowski, K.U.; Ohlendorf, C.; Tomford, W.W.; Zaleske, D.J.

    1996-01-01

    Growth of chondrocytes into a xenogeneic chondroepiphyseal matrix was investigated in an in vitro experimental model by combining viable calf chondrocytes with chick epiphyseal matrix devoid of viable chondrocytes. The chondrocytes were harvested from the wrist joints of newborn calves and cultured for 2 days. The epiphyses were harvested from the distal femurs and the proximal tibias of fetal chicks after development was arrested at 17 days by freezing. The epiphyseal specimens were prepared in four ways. These included femoral and tibial epiphyses without holes and femoral and tibial epiphyses with holes made by a laser. These epiphyseal specimens were co-cultured with calf chondrocytes for various periods. After digestion of the epiphyseal matrix, viable chondrocytes were counted in suspension. Chondrocyte division in the matrix was assessed by [{sup 3}H]thymidine incorporation. The growth of calf chondrocytes into the xenogeneic chick matrix was evaluated by fluorescence microscopy on fresh thick epiphyseal sections. The percentage of viable chondrocytes in the xenogeneic epiphyseal matrix increased with culture time to a maximum at day 21. The addition of laser-drilled holes was found to extend a plateau of chondrocyte viability until day 29. A decrease in cell viability was detected at later observation points. This study demonstrates that xenogeneic matrix may serve as a morphogenetic scaffold for chondrocytic growth. 22 refs., 3 figs.

  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. [Bone development trend in the knee joint of Tibetan teenagers in Aba Prefecture of Sichuan Province].

    PubMed

    Ding, Shi-Rong; Ying, Chong-Liang; Wan, Lei; Wei, Hua; Wang, Ya-Hui; Zhu, Guang-You

    2013-08-01

    To discuss bone development trend in the knee joint of Tibetan teenagers in Sichuan province and to effectively update the database for estimating the living age of Tibetan teenagers in terms of bone age of the knee joint. Radiographs including epiphysis of distal femur, proximal tibia and proximal fibula were taken from 483 Tibetan male and female teenagers aged from 14 to 19 years old in Aba prefecture of Sichuan province in order to observe epiphyseal growth situation. The descriptive data of the epiphyseal closure ages of these teenagers' knee joints were statistically analyzed by SPSS 16.0 software. The distal femur epiphyseal closure occurred earliest, while the proximal fibula epiphyseal closure occurred latest. The epiphyseal growth of knee joints of females occurred about one year earlier than that of males. The forensic information and data related to Tibetan teenagers' bone age identification should be updated regularly. These results provide potential value for the practice of forensic medicine, anthropology and clinical medicine.

  20. Supraintercondylar fractures of the humerus--treatment by the Vigliani osteosynthesis.

    PubMed

    Fama, G

    1987-03-01

    Nineteen cases of supraintercondylar fractures of the humerus treated by the Vigliani osteosynthesis technique are described. This consists of wide transolecranon exposure of the fracture, stabilisation of the epiphyseal fragments with a transcondylar screw, and of the metaphyseo-epiphyseal junction with two condylo-diaphyseal "Eiffel Tower" Kirschner wires. The ulnar nerve is transposed anteriorly and the operation is carried out early in order to prevent neural disturbances and intra and/or periarticular ossification. The results confirm the validity of this method, which respects the delicate structure of this part of the humerus. Postoperative plaster is necessary as an indispensable complement to "minimum" osteosynthesis but this is quite free from risk. The importance of careful and gradual mobilisation of the elbow is also confirmed. Finally, this method combines the advantages of stable osteosynthesis in compression (screw and nut) at the epiphyseal site, with those of elastic osteosynthesis (Kirschner wires) at the metaphyseo-epiphyseal junction.

  1. 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.

  2. 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.

  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. The development of centres of ossification of bones forming elbow joints in young swine.

    PubMed

    Visco, D M; Hill, M A; Van Sickle, D C; Kincaid, S A

    1990-08-01

    Epiphyseal centres of ossification in the bones forming the elbow joints of pigs between one day and 15 weeks of age were examined radiographically, macroscopically, mesoscopically and microscopically. Thoracic limbs from 39 pigs were perfused with India ink or silicone rubber injection compound and the bones were dissected free of soft tissues. The humerus, ulna and radius were fixed in formalin or ethyl alcohol and then cleared by the modified Spalteholz technique. Bones were radiographed, examined grossly, and then cut into slabs for mesoscopical evaluation. Foci considered to be calcifying within cartilaginous anlage were selected for microscopical examination. It was concluded that the epiphyseal centre of ossification develops at different times in different sites in the bones forming the elbow joint. Centres of ossification are initiated when foci of chondrocytes adjacent to one side of a cartilage canal undergo hypertrophy and the inter-territorial matrix becomes calcified. Osteogenesis then proceeds in the calcified focus, presumably with osteoprogenitor cells that originate within the cartilage canals. Subsequently, each epiphyseal centre of ossification enlarges by one of two methods. Firstly, the layer of cartilage adjacent to the centre undergoes endochondral ossification, thus allowing for the circumferential growth of the epiphyseal centre of ossification. Secondly, foci of calcification develop adjacent to the ends of cartilage canals near the epiphyseal centre of ossification and eventually the focus of calcification coalesces with the developing epiphyseal centre of ossification, thus establishing a new ossification front. Endochondral ossification continues at the periphery of the mass of bone. Mesoscopical examination is more useful than radiographical evaluation for identifying small foci of calcification which precede epiphyseal centres of ossification.

  5. The development of centres of ossification of bones forming elbow joints in young swine.

    PubMed Central

    Visco, D M; Hill, M A; Van Sickle, D C; Kincaid, S A

    1990-01-01

    Epiphyseal centres of ossification in the bones forming the elbow joints of pigs between one day and 15 weeks of age were examined radiographically, macroscopically, mesoscopically and microscopically. Thoracic limbs from 39 pigs were perfused with India ink or silicone rubber injection compound and the bones were dissected free of soft tissues. The humerus, ulna and radius were fixed in formalin or ethyl alcohol and then cleared by the modified Spalteholz technique. Bones were radiographed, examined grossly, and then cut into slabs for mesoscopical evaluation. Foci considered to be calcifying within cartilaginous anlage were selected for microscopical examination. It was concluded that the epiphyseal centre of ossification develops at different times in different sites in the bones forming the elbow joint. Centres of ossification are initiated when foci of chondrocytes adjacent to one side of a cartilage canal undergo hypertrophy and the inter-territorial matrix becomes calcified. Osteogenesis then proceeds in the calcified focus, presumably with osteoprogenitor cells that originate within the cartilage canals. Subsequently, each epiphyseal centre of ossification enlarges by one of two methods. Firstly, the layer of cartilage adjacent to the centre undergoes endochondral ossification, thus allowing for the circumferential growth of the epiphyseal centre of ossification. Secondly, foci of calcification develop adjacent to the ends of cartilage canals near the epiphyseal centre of ossification and eventually the focus of calcification coalesces with the developing epiphyseal centre of ossification, thus establishing a new ossification front. Endochondral ossification continues at the periphery of the mass of bone. Mesoscopical examination is more useful than radiographical evaluation for identifying small foci of calcification which precede epiphyseal centres of ossification. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 Fig. 7 Fig. 8 PMID:2081708

  6. 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.

  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. 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. © The Author(s) 2014.

  9. 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.

  10. Bulbous epiphysis and popcorn calcification as related to growth plate differentiation in osteogenesis imperfecta.

    PubMed

    Brizola, Evelise; McCarthy, Edward; Shapiro, Jay Robert

    2015-01-01

    Osteogenesis Imperfecta (OI) is an heritable systemic disorder of connective tissue due to different sequence variants in genes affecting both the synthesis of type I collagen and osteoblast function. Dominant and recessive inheritance is recognized. Approximately 90% of the OI cases are due to mutations in COL1A1/A2 genes. We clinically and radiologically describes an adult male with type III osteogenesis imperfecta who presents a rare bone dysplasia termed bulbous epiphyseal deformity in association with popcorn calcifications. Popcorn calcifications may occur with bulbous epiphyseal deformity or independently. Molecular analysis was performed for COL1A1, COL1A2, LEPRE1 and WNT1 genes. An uncommon COL1A1 mutation was identified. Clinical and radiological exams confirmed a distinctive bulbous epiphyseal deformity with popcorn calcifications in distal femurs. We have identified four additional OI patients reported in current literature, whose X-rays show bulbous epiphyseal deformity related to mutations in CR-TAP, LEPRE1 and WNT1 genes. The mutation identified here had been previously described twice in OI patients and no previous correlation with bulbous epiphyseal deformity was described. The occurrence of this bone dysplasia focuses attention on alterations in normal growth plate differentiation and the subsequent effect on endochondral bone formation in OI.

  11. Bulbous epiphysis and popcorn calcification as related to growth plate differentiation in osteogenesis imperfecta

    PubMed Central

    Brizola, Evelise; McCarthy, Edward; Shapiro, Jay Robert

    2015-01-01

    Summary Background Osteogenesis Imperfecta (OI) is an heritable systemic disorder of connective tissue due to different sequence variants in genes affecting both the synthesis of type I collagen and osteoblast function. Dominant and recessive inheritance is recognized. Approximately 90% of the OI cases are due to mutations in COL1A1/A2 genes. We clinically and radiologically describes an adult male with type III osteogenesis imperfecta who presents a rare bone dysplasia termed bulbous epiphyseal deformity in association with popcorn calcifications. Popcorn calcifications may occur with bulbous epiphyseal deformity or independently. Methods Molecular analysis was performed for COL1A1, COL1A2, LEPRE1 and WNT1 genes. Results An uncommon COL1A1 mutation was identified. Clinical and radiological exams confirmed a distinctive bulbous epiphyseal deformity with popcorn calcifications in distal femurs. We have identified four additional OI patients reported in current literature, whose X-rays show bulbous epiphyseal deformity related to mutations in CR-TAP, LEPRE1 and WNT1 genes. Conclusion The mutation identified here had been previously described twice in OI patients and no previous correlation with bulbous epiphyseal deformity was described. The occurrence of this bone dysplasia focuses attention on alterations in normal growth plate differentiation and the subsequent effect on endochondral bone formation in OI. PMID:26604951

  12. Dysplasia epiphysealis hemimelica of the lower limb.

    PubMed

    Celıkyay, Ruken Yuksekkaya; Celikyay, Fatih; Bilgic, Erkal; Ascı, Murat; Koseoglu, Dogan

    2017-01-01

    Dysplasia epiphysealis hemimelica (DEH) is a rare developmental disorder characterized by osteocartilaginous overgrowth in one or more epiphyses. The disease usually involves a single limb or is hemimelic (lateral or medial compartment), and lower extremities are more frequently affected than upper extremities. Here we present clinical and radiological findings for a male DEH patient at 1.5 and 3.5 years of age. The radiographs obtained at first presentation showed minimal osseous overgrowth and irregularity at the epiphyses around the left knee and ankle joints, respectively. Radiographs obtained at the second presentation showed osteocartilaginous masses at most epiphyses of the left lower extremity. Two months after diagnosis (at 3.7 years old), the patient had surgery on his left knee to relieve increased joint restriction. The histopathological diagnosis was consistent with an osteocartilaginous lesion. This case report presents imaging features and age-related progression of DEH in this patient.

  13. 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.

  14. [Radiologische Besonderheiten einer bilateral vererblichen Mikro-Epiphysendysplasie - deutliche Entität einer Skelettdysplasie].

    PubMed

    Mostert, A K; Dijkstra, P F; Van Horn, J R; Jansen, B R H; Heutink, P; Lindhout, D

    2002-07-01

    To prove that bilateral hereditary micro-epiphyseal dysplasia (BHMED), first described by Elsbach in 1959, is a distinct disorder radiologically as well as clinically, compared with multiple epiphyseal dysplasia (MED). We used the data of the revised pedigree with 84 family members, performed a medical history, physical examination and made a radiological evaluation for defining a clinical and radiological phenotype of BHMED family members. We used blood samples for genetic analysis. Although there is a clear clinical picture of the dysplasia, the radiological signs are more reliable for making the diagnosis. Especially the typical deformity of the hip and knee joint are diagnostic for BHMED. By linkage analysis we excluded linkage with the three known MED-loci (EDM1, EDM2 and EDM3). BHMED is indeed an entity that is distinct from common multiple epiphyseal dysplasia (MED), clinically, as well as radiologically and genetically.

  15. 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

  16. Scurvy: An unusual presentation of cerebral palsy

    PubMed Central

    Gupta, Sumit; Kanojia, Rajesh; Jaiman, Ashish; Sabat, Dhananjaya

    2012-01-01

    Scurvy is caused by prolonged severe dietary deficiency of ascorbic acid, in which the breakdown of intercellular cement substances leads to capillary hemorrhages and defective growth of fibroblasts, osteoblasts and odontoblasts, resulting in impaired synthesis of collagen, osteoid and dentine. It is characterized by hemorrhagic gingivitis, subperiosteal hemorrhages, perifollicular hemorrhages, and frequently petechial hemorrhages (especially on the feet). People with abnormal dietary habits, mental illness or physical disability are prone to develop this disease. Epiphyseal separation is known to occur in scurvy but is rarely seen now. Epiphyseal separation from the metaphysis is always through the zone of calcified cartilage, known as “scorbutic lattice”, which in the radiographs is represented as “the white line of Frenkel”. We report a case of multiple epiphyseal separations in a cerebral palsy child because of vitamin C deficiency. The child was treated with splintage of extremity and nutritional supplementation. All physeal separation healed completely without any deformity. PMID:22655223

  17. 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. © 2014 Wiley Periodicals, Inc.

  18. 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.

  19. 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.

  20. [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.

  1. 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.

  2. 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.

  3. The Galeazzi-equivalent lesion in children revisited.

    PubMed

    Imatani, J; Hashizume, H; Nishida, K; Morito, Y; Inoue, H

    1996-08-01

    A fracture at the medial end of the distal third of the radius with an epiphyseal separation of the distal ulna in a 16-year-old boy is described. This injury, known as the Galeazzi-equivalent lesion in children, is characterized by complete distal ulnar epiphyseal separation without rupture of the distal ligamentous stabilizing system between the radius and ulna, which includes the triangular fibrocartilage complex, interosseous ligaments and periosteal tube of the ulnar. The Galeazzi fracture-dislocation and the Galeazzi-equivalent lesion appear to be completely dissimilar in their pathological anatomy. We suggest calling the latter a "pseudo-Galeazzi injury".

  4. 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

  5. ( 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.

  6. 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)…

  7. 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.

  8. Maturation and reproduction of northern flying squirrels in Pacific Northwest forests.

    Treesearch

    Lisa J. Villa; Andrew B. Carey; Todd M. Wilson; Karma E. Glos

    1999-01-01

    Northern flying squirrels are the primary prey of northern spotted owls and are important dispersers of fungal spores in Pacific Northwest forests. Despite the importance of these squirrels in forest ecosystems, information is lacking on life history and methods for determining age and reproductive condition. In the laboratory, we measured epiphyseal notch, femur...

  9. Mucopolysaccharidosis IVA (Morquio A syndrome) and VI (Maroteaux-Lamy syndrome): under-recognized and challenging to diagnose.

    PubMed

    Lachman, Ralph S; Burton, Barbara K; Clarke, Lorne A; Hoffinger, Scott; Ikegawa, Shiro; Jin, Dong-Kyu; Kano, Hiroki; Kim, Ok-Hwa; Lampe, Christina; Mendelsohn, Nancy J; Shediac, Renée; Tanpaiboon, Pranoot; White, Klane K

    2014-03-01

    Mucopolysaccharidosis IVA (MPS IVA, or Morquio A syndrome) and VI (MPS VI, or Maroteaux-Lamy syndrome) are autosomal recessive lysosomal storage disorders. Skeletal abnormalities are common initial presenting symptoms and, when recognized early, may facilitate timely diagnosis and intervention, leading to improved patient outcomes. Patients with slowly progressing disease and nonclassic phenotypes can be particularly challenging to diagnose. The objective was to describe the radiographic features of patients with a delayed diagnosis of MPS IVA or VI. This was a retrospective study. The records of 5 MPS IVA and 3 MPS VI patients with delayed diagnosis were reviewed. Radiographs were evaluated by a radiologist with special expertise in skeletal dysplasias. An important common theme in these cases was the appearance of multiple epiphyseal dysplasia (MED) with epiphyseal changes seemingly confined to the capital (proximal) femoral epiphyses. Very few patients had the skeletal features of classical dysostosis multiplex. Radiologists should appreciate the wide phenotypic variability of MPS IVA and VI. The cases presented here illustrate the importance of considering MPS in the differential diagnosis of certain skeletal dysplasias/disorders, including MED, some forms of spondylo-epiphyseal dysplasia (SED), and bilateral Perthes-like disease. It is important to combine radiographic findings with clinical information to facilitate early testing and accurate diagnosis.

  10. 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.

  11. 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

  12. 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

  13. 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)…

  14. Galeazzi-equivalent lesions in adolescence.

    PubMed

    Kamano, Masayuki; Honda, Yoshinobu

    2002-07-01

    Two cases of a Galeazzi-equivalent lesion in adolescence are described. Accurate diagnosis of the epiphyseal injury of the distal ulna, rigid fixation of the distal radius, and stabilization of the distal radioulnar joint are keys in obtaining a good result in the treatment of Galeazzi-equivalent lesion in adolescence.

  15. 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

  16. 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.

  17. 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

  18. 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.

  19. 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.

  20. Radiologic findings of Patterson-Lowry rhizomelic dysplasia in two sisters.

    PubMed

    Damar, Çağrı; Boyunağa, Öznur; Derinkuyu, Betül Emine; Battaloğlu, Nergiz; Ezgü, Fatih Süheyl

    2014-11-01

    We report two sisters who have a rare skeletal abnormality termed Patterson-Lowry rhizomelic dysplasia. The typical findings of these cases on bone survey are isolated shortening and proximal metaphyseal enlargement and cupping of the bilateral humeri. The elder sister also has coxa vara deformity and dysplastic proximal femoral epiphyses on both sides. The younger sister has normal hip joint bones bilaterally, but her proximal femoral epiphyses are smaller than normal. All other bones of the sisters are of normal size and configuration. Our patients are two siblings, and their parents are first degree relatives, suggesting autosomal-recessive (AR) inheritance. The present patients help us to understand the genetic relationships and skeletal variabilities of this rare entity.

  1. 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

  2. [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.

  3. Jackson-Weiss syndrome: Clinical and radiological findings in a large kindred and exclusion of the gene from 7p21 and 5qter

    SciTech Connect

    Ades, L.C.; Haan, E.A.; Mulley, J.C.; Senga, I.P.; Morris, L.L.; David, D.J.

    1994-06-01

    We describe the clinical and radiological manifestations of the Jackson-Weiss syndrome (JWS) in a large South Australian kindred. Radiological abnormalities not previously described in the hands include coned epiphyses, distal and middle phalangeal hypoplasia, and carpal bone malsegmentation. New radiological findings in the feet include coned epiphyses, hallux valgus, phalangeal, tarso-navicular and calcaneo-navicular fusions, and uniform absence of metatarsal fusions. Absence of linkage to eight markers along the short arm of chromosome 7 excluded allelian between JWS and Saethre-Chotzen syndrome at 7p21. No linkage was detected to D5S211, excluding allelism to another recently described cephalosyndactyly syndrome mapping to 5qter. 35 refs., 5 figs., 4 tabs.

  4. Severe valgus deformity of the knee with permanent patellar dislocation associated with melorheostosis: a case report and review of the literature.

    PubMed

    Kitta, Yuki; Niki, Yasuo; Udagawa, Kazuhiko; Enomoto, Hiroyuki; Toyama, Yoshiaki; Suda, Yasunori

    2014-03-01

    We present a case of an 8-year-old boy diagnosed with melorheostosis who was suffering from severe genu valgum, permanent dislocation of the patella, knee flexion contracture and leg length shortening. Soft tissue contracture of the limb and subsequent joint deformities were reported to represent clinical manifestations of pediatric melorheostosis. As the epiphyseal plate had not closed, patellar reduction was achieved by soft tissue surgical stabilization, including lateral retinacular release, medial retinaculum plication, and transfer of the lateral half of the patellar tendon. At 4 years postoperatively, as a result of improved limb alignment and knee flexion contracture, the leg length shortening has improved, and the patient does not limp and participates in sports activities. Surgical intervention should be performed as early as possible, because genu valgum and external rotation of the tibia may deteriorate with age, rendering the patellar dislocation irreversible in patients with melorheostosis before epiphyseal closure. Copyright © 2012 Elsevier B.V. All rights reserved.

  5. [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.

  6. [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.

  7. 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.

  8. 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

  9. Compartment syndrome with an isolated Salter Harris II fracture of the distal tibia.

    PubMed

    Cox, George; Thambapillay, Siva; Templeton, Peter A

    2008-02-01

    A 14-year-old boy sustained a Salter Harris II fracture to his right distal tibia after a fall from his skateboard. He rapidly went on to develop the signs and symptoms of compartment syndrome, and he underwent emergency fasciotomy. This resulted in relief of his symptoms. After this procedure, his fracture was fixed with a single anteroposterior screw. He made a full and uncomplicated recovery, with no clinical or radiological evidence of epiphyseal growth arrest.

  10. Atypical growth plate closure: a possible chronic Salter and Harris Type V injury.

    PubMed

    Sato, Takahisa; Shinozaki, Tetsuya; Fukuda, Toshio; Watanabe, Hideomi; Aoki, Jun; Yanagawa, Takashi; Takagishi, Kenji

    2002-04-01

    Salter-Harris Type V epiphyseal injury is relatively uncommon. The authors present a case involving the proximal tibia diagnosed both by radiologic and histologic findings. The findings suggest that Salter-Harris Type V injury can be a possible outcome when the disappearance of the physeal plate is reported in a patient who has great physical activity in childhood, even if the patient has suffered from no major injury.

  11. A rare lethal case of chondrodysplasia punctata with extensive airway involvement.

    PubMed

    Dewan, Pooja; Rai, Anuradha; Gupta, Natasha; Shah, Dheeraj; Faridi, M M A

    2012-06-01

    Stippled cartilaginous calcification, an important radiologic sign, is described as ''chondrodysplasia punctata'' when seen in association with limb shortening and maxillofacial hypoplasia. We report chondrodysplasia punctata in a male neonate who presented with limb shortening, midfacial hypoplasia, and laryngeal stenosis leading to death within a few minutes of birth. A post-mortem radiograph revealed generalized punctate calcification of cartilaginous structures, including airways, rib ends, spine, long bone epiphyses, tarsus and pinna, and brachytelephalangy.

  12. 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.

  13. [Postoperative function exercise for replantation of severed limbs in children].

    PubMed

    Hu, J; Liu, X Y; Zhen, P

    1996-05-01

    The speciality of limb replantation in children included thread and thin for blood vessels, growth development for muscles and epiphyses, morable and incooperation in clinical. The function exercises after limb replantation in children must be connected with the clinical speciality of vascular microsurgery, the anthor divided it into four stages; vascular crises stage, limb aliving stage, clinical healing of bone stage, and function recovery stage. The different treatment performed in four stages, and excellent clinical results were achieved.

  14. 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.

  15. 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.

  16. 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.

  17. 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.

  18. [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.

  19. 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.

  20. Multicentric GCT of tarsal bones in an immature skeleton: a case report with review of literature.

    PubMed

    Varshney, Atul; Rao, Harish; Sadh, Raghvendra

    2010-01-01

    Multicentric giant cell tumor of the foot is rare. Multicentric giant cell tumor of the tarsal bones in an immature skeleton is unheard of. We report a case of synchronous multicentric giant cell tumor involving the talus and calcaneum in a 14-year-old boy with open epiphyseal plate for its rarity and successful treatment by curettage and synthetic bone graft. Copyright 2010 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

  1. 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.

  2. Slipped capital femoral epiphysis following radiation and chemotherapy

    SciTech Connect

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

    1981-01-01

    Patients who received radiation to the proximal femoral epiphysis and chemotherapy in childhood appear to have an increased risk of subsequently developing epiphyseolysis. Every effort should be made to exclude the proximal femoral epiphysis from the radiation port whenever possible. If the epiphyseal plate is widened and irregular and the adjacent bone is sclerotic, prophylactic pinning may be indicated even in the absence of a grossly visible slip.

  3. Structural scene analysis and content-based image retrieval applied to bone age assessment

    NASA Astrophysics Data System (ADS)

    Fischer, Benedikt; Brosig, André; Deserno, Thomas M.; Ott, Bastian; Günther, Rolf W.

    2009-02-01

    Radiological bone age assessment is based on global or local image regions of interest (ROI), such as epiphyseal regions or the area of carpal bones. Usually, these regions are compared to a standardized reference and a score determining the skeletal maturity is calculated. For computer-assisted diagnosis, automatic ROI extraction is done so far by heuristic approaches. In this work, we apply a high-level approach of scene analysis for knowledge-based ROI segmentation. Based on a set of 100 reference images from the IRMA database, a so called structural prototype (SP) is trained. In this graph-based structure, the 14 phalanges and 5 metacarpal bones are represented by nodes, with associated location, shape, as well as texture parameters modeled by Gaussians. Accordingly, the Gaussians describing the relative positions, relative orientation, and other relative parameters between two nodes are associated to the edges. Thereafter, segmentation of a hand radiograph is done in several steps: (i) a multi-scale region merging scheme is applied to extract visually prominent regions; (ii) a graph/sub-graph matching to the SP robustly identifies a subset of the 19 bones; (iii) the SP is registered to the current image for complete scene-reconstruction (iv) the epiphyseal regions are extracted from the reconstructed scene. The evaluation is based on 137 images of Caucasian males from the USC hand atlas. Overall, an error rate of 32% is achieved, for the 6 middle distal and medial/distal epiphyses, 23% of all extractions need adjustments. On average 9.58 of the 14 epiphyseal regions were extracted successfully per image. This is promising for further use in content-based image retrieval (CBIR) and CBIR-based automatic bone age assessment.

  4. 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

  5. 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.

  6. 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

  7. 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

  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. 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.

  10. 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

  11. 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.

  12. 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

  13. /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.

  14. Brief communication: a probabilistic approach to age estimation from infracranial sequences of maturation.

    PubMed

    Coqueugniot, Hélène; Weaver, Timothy D; Houët, Francis

    2010-08-01

    Infracranial sequences of maturation are commonly used to estimate the age at death of nonadult specimens found in archaeological, paleoanthropological, or forensic contexts. Typically, an age assessment is made by comparing the degree of long-bone epiphyseal fusion in the target specimen to the age ranges for different stages of fusion in a reference skeletal collection. While useful as a first approximation, this approach has a number of shortcomings, including the potential for "age mimicry," being highly dependent on the sample size of the reference sample and outliers, not using the entire fusion distribution, and lacking a straightforward quantitative way of combining age estimates from multiple sites of fusion. Here we present an alternative probabilistic approach based on data collected on 137 individuals, ranging in age from 7- to 29-years old, from a documented skeletal collection from Coimbra, Portugal. We then use cross validation to evaluate the accuracy of age estimation from epiphyseal fusion. While point estimates of age can, at least in some circumstances, be both accurate and precise based on the entire skeleton, or many sites of fusion, there will often be substantial error in these estimates when they derive from one or only a few sites. Because a probabilistic approach to age estimation from epiphyseal fusion is computationally intensive, we make available a series of spreadsheets or computer programs that implement the approach presented here. Copyright 2010 Wiley-Liss, Inc.

  15. Neutral Sphingomyelinase (SMPD3) Deficiency Causes a Novel Form of Chondrodysplasia and Dwarfism That Is Rescued by Col2A1-Driven smpd3 Transgene Expression

    PubMed Central

    Stoffel, Wilhelm; Jenke, Britta; Holz, Barbara; Binczek, Erika; Günter, Robert Heinz; Knifka, Jutta; Koebke, Jürgen; Niehoff, Anja

    2007-01-01

    Neutral sphingomyelinase SMPD3 (nSMase2), a sphingomyelin phosphodiesterase, resides in the Golgi apparatus and is ubiquitously expressed. Gene ablation of smpd3 causes a generalized prolongation of the cell cycle that leads to late embryonic and juvenile hypoplasia because of the SMPD3 deficiency in hypothalamic neurosecretory neurons. We show here that this novel form of combined pituitary hormone deficiency is characterized by the perturbation of the hypothalamus-pituitary growth axis, associated with retarded chondrocyte development and enchondral ossification in the epiphyseal growth plate. To study the contribution by combined pituitary hormone deficiency and by the local SMPD3 deficiency in the epiphyseal growth plate to the skeletal phenotype, we introduced the full-length smpd3 cDNA transgene under the control of the chondrocyte-specific promoter Col2a1. A complete rescue of the smpd3−/− mouse from severe short-limbed skeletal dysplasia was achieved. The smpd3−/− mouse shares its dwarf and chondrodysplasia phenotype with the most common form of human achondrodysplasia, linked to the fibroblast-growth-factor receptor 3 locus, not linked to deficits in the hypothalamic-pituitary epiphyseal growth plate axis. The rescue of smpd3 in vivo has implications for future research into dwarfism and, particularly, growth and development of the skeletal system and for current screening and future treatment of combined dwarfism and chondrodysplasia. PMID:17591962

  16. Morquio disease: the role of cartilage canals in the pathogenesis of chondrogenic dwarfism.

    PubMed

    Zustin, Jozef

    2010-12-01

    Chondrogenic dwarfism in Morquio disease (mucopolysaccharidosis IV) has been suggested to be strongly linked to the abnormal lysosomal storage of cartilaginous extracellular matrix waste products within chondrocytes and fibroblasts. The specific genetic defects of enzymes of the keratan sulfate and chondroitin-6-sulfate metabolism have been detected at the molecular level and importantly contributed to the current knowledge on the phenotype of this rare metabolic disorder. However, the pathogenesis of this epiphyseal centered progressive skeletal disease does not seem to be fully explained by the dysfunction of the chondrocyte cytoplasm that presents with vacuolar changes in adult patients. I propose that the accumulation of extracellular matrix degradation product-laden macrophages within epiphyseal cartilage canals during the early postnatal period causes dysregulation in the synchronized process of the neoformation and resorption of the maturing radial growing epiphyses. Similarly, the resorption of pannus tissue following the microtraumatisation of weight-bearing joints and epiphysis-type bones becomes impacted. If the hypothesis is valid, the early pathogenesis in Morquio disease could be because of the inadequate regression of cartilage canals and impaired resorption and restitution of pannus tissue.

  17. 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

  18. [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").

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

    PubMed

    Strøm, Thale M Asp; 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.

  20. Bone age estimation based on multislice computed tomography study of the scapula.

    PubMed

    Nougarolis, Florence; Mokrane, Fatima-Zohra; Sans, Nicolas; Rousseau, Hervé; Dedouit, Fabrice; Telmon, Norbert

    2017-03-01

    Progress in medical imaging has opened new areas of research in forensic anthropology, especially in the context of the study of bone age assessment. The study of bone age has become a useful tool for age estimation at death or age of young adult migrants in an anthropological context. We retrospectively evaluated multislice computed tomography (MSCT) explorations focused on scapulae of 232 individuals (123 males; 109 females) aged between 8 and 30 years old. Computed tomography (CT) scans were viewed in axial and multiplanar reconstructed images using OsiriX 5.9 (64 bit)®. The ossification centers of the scapula studied were as follows: acromial, sub-coracoid, glenoid, coracoid, coracoid apex, and inferior angle epiphyses. Fusion status was scored based on a five-stage system (stage 1: no ossification, stage 2: visualization of an ossification center, stage 3: partial ossification, stage 4: full ossification associated to an epiphyseal scar, and stage 5: full ossification without epiphyseal scar). Intra-observer variability was excellent, and inter-observer variability was good, demonstrating the reliability of this MSCT staging system. The fusion of scapular ossification centers was statistically associated with age (p < 0.001) but not with sex (p > 0.05). In conclusion, MSCT of the scapula is an efficient method for age assessment, which is complementary to preexisting methods particularly for specifying the 18-year threshold. Further studies with larger groups are needed to support our results.

  1. MRI Anatomy of the Tibial ACL Attachment and Proximal Epiphysis in a Large Population of Skeletally Immature Knees: Reference Parameters for Planning Anatomic Physeal-Sparing ACL Reconstruction.

    PubMed

    Swami, Vimarsha Gopal; Mabee, Myles; Hui, Catherine; Jaremko, Jacob Lester

    2014-07-01

    To aid in performing anatomic physeal-sparing anterior cruciate ligament (ACL) reconstruction, it is important for surgeons to have reference data for the native ACL attachment positions and epiphyseal anatomy in skeletally immature knees. To characterize anatomic parameters of the ACL tibial insertion and proximal tibial epiphysis at magnetic resonance imaging (MRI) in a large population of skeletally immature knees. Cross-sectional study; Level of evidence, 3. The ACL tibial attachment site and proximal epiphysis were examined in 570 skeletally immature knees with an intact ACL (age, 6-15 years) using 1.5-T proton density-weighted sagittal MRI; also measured were the tibial anteroposterior diameter; anterior, central, and posterior ACL attachment positions; vertical height of the epiphysis; and maximum oblique epiphyseal depth extending from the ACL tibial attachment center to the tibial tuberosity. In adolescents (11-15 years of age), the center of the ACL's tibial attachment was 51.5% ± 5.7% of the anteroposterior diameter of the tibia, with no significant differences between sexes or age groups (P > .05 in all cases). Mean vertical epiphyseal height was 15.9 ± 1.7 mm in the adolescent group, with significant differences between 11-year-olds (15.2 ± 1.5 mm) and 15-year-olds (16.6 ± 1.6 mm), P < .001, and between males (16.6 ± 1.5 mm) and females (14.8 ± 1.4), P < .001. Mean maximum oblique depth was 30.0 ± 5.3 mm, with a significant difference between 11-year-olds (26.7 ± 4.9 mm) and 15-year-olds (32.7 ± 5.1 mm), P < .001, and between males (29.7 ± 6.4 mm) and females (27.8 ± 5.2 mm), P < .001. The maximum oblique depth occurred at a mean angle of ~50°, and this angle did not change with age or sex. There was a significant moderate correlation (r = 0.39, P < .001) between epiphyseal vertical height and maximum oblique depth. The center of the ACL tibial attachment was consistently near 51% of the anteroposterior diameter, regardless of age or sex

  2. Human long bone development in vivo: analysis of the distal femoral epimetaphysis on MR images of fetuses.

    PubMed

    Nemec, Ursula; Nemec, Stefan F; Weber, Michael; Brugger, Peter C; Kasprian, Gregor; Bettelheim, Dieter; Rimoin, David L; Lachman, Ralph S; Malinger, Gustavo; Prayer, Daniela

    2013-05-01

    To investigate human long bone development in vivo by analyzing distal femoral epimetaphyseal structures and bone morphometrics on magnetic resonance (MR) images of fetuses. An institutional review board approved this retrospective study, and informed consent was waived. Included were 272 MR imaging examinations (April 2004-July 2011) in 253 fetuses with a mean gestational age (GA) of 26 weeks 6 days (range, 19 weeks 2 days to 35 weeks 6 days) without known musculoskeletal abnormalities. Two independent readers qualitatively analyzed epiphyseal and metaphyseal shape, secondary ossification, and the perichondrium on 1.5-T echo-planar MR images and correlated the results with the GA that was derived from previous fetal ultrasonography (US). Diaphyseal and epiphyseal morphometric measurements were correlated with GA by means of the Pearson correlation and linear regression. MR imaging measurements of diaphyseal length and US normative values were compared graphically. Interreader agreement analysis was performed with weighted κ statistics and the intraclass correlation coefficient. With advancing GA, the epiphyseal shape changed from spherical (r(2) = 0.664) to hemispherical with a notch (r(2) = 0.804), and the metaphyseal shape changed from flat (r(2) = 0.766) to clearly undulated (r(2) = 0.669). Secondary ossification (r(2) = 0.777) was not observed until 25 weeks 3 days. The perichondrium decreased (r(2) = 0.684) from 20 weeks onward. Correlation coefficients were 0.897 for diaphyseal length, 0.738 for epiphyseal length, and 0.801 for epiphyseal width with respect to GA. The range of measurements of diaphyseal length was larger than that of the reported US normative values. Interreader agreement was good for bone morphometrics (intraclass correlation coefficient, 0.906-0.976), and moderate for bone characteristics (weighted κ, 0.448-0.848). Prenatal MR imaging allows visualization of human bone development in vivo by means of epimetaphyseal characteristics and

  3. 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

  4. 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.

  5. Injury trends in rock climbers: evaluation of a case series of 911 injuries between 2009 and 2012.

    PubMed

    Schöffl, Volker; Popp, Dominik; Küpper, Thomas; Schöffl, Isabelle

    2015-03-01

    Rock climbing is a widely performed sport. This prospective single-institution study evaluated the demographics of climbing-related injuries to improve our comprehension of current injury characteristics. During a 4-year period, 836 patients with a total of 911 independent climbing injuries were prospectively evaluated using a standard questionnaire and examination protocol. Of all injuries, 833 were on the upper extremities, 58 on the lower. Seventeen injuries were Union International des Associations d'Alpinisme (UIAA) grade 1 injuries, 881 were grade 2, and 13 were grade 3. No higher UIAA graded injuries occurred. Overall, 380 were acute injuries (359 were seen in clinic, 21 were seen through the emergency department), and 531 were overstrain injuries (all seen in clinic). Finger injuries accounted for 52% of all injuries, the shoulder being the second most frequent location. Pulley injuries were the most frequent finger injuries. Of 20 injured young climbers under the age of 15 years, 14 had an epiphyseal fracture (all epiphyseal fractures: mean age 14 years, range 12 to 15 years). Male climbers were significantly older (P < .05), had more climbing years (P < .05), and were climbing at a higher climbing level (P < .01). Older, more experienced climbers had significantly more overstrain injuries than acute injuries (P < .05). When comparing this study with our previous study from 1998 to 2001, there are some notable differences. Although pulley injuries are still the most common climbing injury, there are now more A4 pulley injuries than A2. Shoulder injuries are becoming more common, as are epiphyseal fractures among young climbers. It is important to understand current patterns of climbing injuries so that health providers can target interventions appropriately. Copyright © 2015 Wilderness Medical Society. Published by Elsevier Inc. All rights reserved.

  6. 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

  7. 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.

  8. 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

  9. 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

  10. 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

  11. Avascular necrosis and the Aberdeen splint in developmental dysplasia of the hip.

    PubMed

    Williams, P R; Jones, D A; Bishay, M

    1999-11-01

    Between January 1987 and December 1988 there were 7575 births in the Swansea maternity unit. Of these 823 (10.9%) were considered to be at 'high risk' for developmental dysplasia of the hip (DDH). Static ultrasound examination was performed in each case and the results classified on the basis of the method of Graf. A total of 117 type III-IV hips in 83 infants was splinted using the Aberdeen splint. Radiographs of these hips were taken at six and 12 months. Hilgenreiner's measurements of the acetabular angle were made in all cases and the development of the femoral capital epiphysis was assessed by measuring the epiphyseal area. The effect of splintage on the acetabular angle and the epiphyseal area between the normal and abnormal splinted hips was compared. Radiographs of 16 normal infants (32 normal unsplinted hips) were used as a control group. This cohort has now been followed up for a minimum of nine years. There have been no complications as a result of splintage. The failure rate was 1.7% or 0.25 per 1000 live births. No statistical difference was found when comparing the effect of splintage on the acetabular angle and epiphyseal area between normal and abnormal splinted hips and normal unsplinted hips. Our study has shown that while the Aberdeen splint had a definite but small failure rate, it was safe in that it did not produce avascular necrosis. The current conventional view that a low rate of splintage is always best is therefore brought into question if the Aberdeen splint is chosen for the management of neonatal DDH.

  12. 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.

  13. 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.

  14. 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.

  15. [Skateboard injuries (author's transl)].

    PubMed

    Stürz, H; Rosemeyer, B

    1979-04-06

    Following the introduction of skateboards into Germany in 1976 an increasing number of accidents and injuries have been noted, affecting mainly children 10 to 14 years of age. The causes were lack of experience and the careless use of the boards on public streets. More than 30% of injuries were fractures mainly affecting the upper limb. Because of the frequent involvement of the epiphyseal plates the post-accidental growth may be seriously disturbed. Severe and sometimes fatal head or abdominal injuries have been reported, mainly after collision with cars.

  16. Management of the Anterior Cruciate Ligament-Injured Knee in the Skeletally Immature Athlete.

    PubMed

    Anderson, Christian N; Anderson, Allen F

    2017-01-01

    Intrasubstance tears of the anterior cruciate ligament (ACL) are being diagnosed with increasing frequency in the skeletally immature. Management options include nonoperative/ early surgical, or delayed surgical reconstruction. Nonoperative/delayed reconstruction results in worse functional outcomes than early reconstruction. Physicians are faced with a treatment dilemma; clinical and basic science studies have demonstrated risk of limb-length discrepancy and angular deformity with ACL reconstruction. Vertical drill tunnels decrease physeal damage and minimize growth deformity; however, this technique results in nonanatomic ACL graft placement. All-epiphyseal reconstruction avoids damage to the growth plate. These techniques are biomechanically superior to extraarticular and modified physeal-sparing procedures.

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. 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.

  2. 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

  3. Reversible skeletal changes after treatment with bevacizumab in a child with cutaneovisceral angiomatosis with thrombocytopenia syndrome.

    PubMed

    Smith, Angela R; Hennessy, Jane M; Kurth, Margaret A Heisel; Nelson, Stephen C

    2008-09-01

    Cutaneovisceral angiomatosis with thrombocytopenia (CAT) syndrome is a rare vascular disorder of the skin and gastrointestinal tract for which there is no standard treatment. We present a case in which a child with CAT syndrome was treated with bevacizumab, a vascular endothelial growth factor inhibitor, and subsequently developed asymptomatic metaphyseal bone lesions. Though not previously described as a side effect, we hypothesize that the use of bevacizumab in a child with active epiphyseal growth plates caused these radiographic lesions. Because of the potential for altered bone growth and metabolism, children receiving VEGF inhibitors should be monitored closely for bony toxicity. (c) 2008 Wiley-Liss, Inc.

  4. 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.

  5. An unusual case of sciatic neuropraxia due to melorheostosis.

    PubMed

    Singh, Raj; Singh, Zile; Bala, Renu; Rana, Parveen; Sangwan, Sukhbir Singh

    2010-12-01

    Melorrheostosis is a rare osteosclerotic bone dysplasia of obscure etiology. The typical radiographic features are flowing candle wax, sub-periosteal bone and streaky endosteal bone formation in diaphyseal and epiphyseal area with sclerotomal pattern mainly involving appendicular skeleton. It is rarely associated with nerve palsies. The authors report a case of melorrheostotic mass causing sciatic neuropraxia and to the best of their knowledge it is the first case reported in the English language literature. Copyright © 2010 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  6. Congenital rickets presenting as refractory respiratory distress at birth.

    PubMed

    Tiwari, Soumya; Kumar, Rajesh; Singla, Shilpy; Dudeja, Ajay; Nangia, Sushma; Saili, Arvind

    2014-08-01

    Congenital rickets is a very rare entity in the spectrum of metabolic bone disease in children. The authors report an as yet unreported case of congenital rickets presenting with respiratory distress at birth. The radiographs of long bones and wrist showed generalized osteopenia with cupping and fraying of epiphyseal ends in the second week of life. The patient was managed with very high doses of vitamin D which led to clinico-radiological and biochemical improvement. More than being interesting for its extreme rarity, this report assumes importance as it brings forth the possibility of congenital rickets being a differential diagnosis for a newborn with respiratory distress.

  7. 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

  8. 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

  9. [Effects of zinc deficiency and vitamin D deficiency on bone calcification and development of rats].

    PubMed

    Xu, B; Zheng, D; Qian, Y; Wu, K

    1992-09-01

    Zinc deficiency rat model was made by feeding zinc deficiency diet. The level of bone calcium of the zinc deficiency rats was significantly lower than that of the control rats. Their bone cortex was thinner and bone density decreased. The counts of their cartilage cells and hypertrophic cells of epiphyseal plate were less frequent, and the diameter of their hypertrophic cells was smaller than that of the controls. It suggested that zinc deficiency caused defective bone calcification which was similar to that in vitamin D deficiency. Zinc deficiency seemed to hinder the linear growth of long bone and might be the cause of dwarf.

  10. [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.

  11. Os odontoideum in wolcott-rallison syndrome: a case series of 4 patients.

    PubMed

    Dias, R P; Buchanan, C R; Thomas, N; Lim, S; Solanki, G; Connor, S E J; Barrett, T G; Kapoor, R R

    2016-02-10

    Wolcott-Rallison Syndrome is the commonest cause of neonatal diabetes in consanguineous families. It is associated with liver dysfunction, epiphyseal dysplasia, and developmental delay. It is caused by mutations in eukaryotic translation initiation factor 2-α kinase 3 (EIF2AK3).We report 4 children with WRS and Os Odontoideum resulting in significant neurological compromise. This cervical spine abnormality has not previously been described in this syndrome. This additional evidence broadens the clinical spectrum of this syndrome and confirms the role of EIF2AK3 in skeletal development. Furthermore, Os Odontoideum needs to be actively screened for in WRS patients to prevent neurological and respiratory compromise.

  12. Prenatal mucolipidosis type II (I-cell disease) can present as Pacman dysplasia.

    PubMed

    Saul, Robert A; Proud, Virginia; Taylor, Harold A; Leroy, Jules G; Spranger, Jurgen

    2005-06-15

    Pacman dysplasia has been previously reported to be a lethal skeletal dysplasia with epiphyseal stippling and osteoclastic overactivity. We report on a sibling of a fetus previously reported as Pacman dysplasia. This infant has a clinical course consistent with mucolipidosis type II (I-cell disease) along with confirmatory biochemical, cytologic, and radiographic evidence. This case expands the phenotypic spectrum of mucolipidosis type II. Having redefined the diagnosis in one of the original cases of Pacman dysplasia, we suggest that what is called Pacman dysplasia could very well be Mucolipidosis type II (ML-II) in other published reports.

  13. 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.

  14. 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.

  15. 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.

  16. The effects of a 20-week nutritional insult on the skeletal development of Cebus albifrons during the 1st year of life.

    PubMed

    Thurm, D A; Samonds, K W; Hegsted, D M

    1976-06-01

    Using an atlas constructed from records of a group of normal monkeys in the same colony, the effects of a 20-week low-calorie or low-protein nutritional insult on skeletal development were determined. The most significantly retarded sites were the late-forming ossifications (epiphyses, sesamoids, and tuberosites). A general increase in ossification anomalies and in individual variation was evident. The similarity of these findings to studies previously reported for man indicates the utility of nonhuman primate models for the further study of nutritional deficiency.

  17. 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

  18. AIP mutation in pituitary adenomas in the 18th century and today.

    PubMed

    Chahal, Harvinder S; Stals, Karen; Unterländer, Martina; Balding, David J; Thomas, Mark G; Kumar, Ajith V; Besser, G Michael; Atkinson, A Brew; Morrison, Patrick J; Howlett, Trevor A; Levy, Miles J; Orme, Steve M; Akker, Scott A; Abel, Richard L; Grossman, Ashley B; Burger, Joachim; Ellard, Sian; Korbonits, Márta

    2011-01-06

    Gigantism results when a growth hormone-secreting pituitary adenoma is present before epiphyseal fusion. In 1909, when Harvey Cushing examined the skeleton of an Irish patient who lived from 1761 to 1783, he noted an enlarged pituitary fossa. We extracted DNA from the patient's teeth and identified a germline mutation in the aryl hydrocarbon-interacting protein gene (AIP). Four contemporary Northern Irish families who presented with gigantism, acromegaly, or prolactinoma have the same mutation and haplotype associated with the mutated gene. Using coalescent theory, we infer that these persons share a common ancestor who lived about 57 to 66 generations earlier.

  19. 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.

  20. 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.

  1. Variant of Galeazzi fracture-dislocation in children.

    PubMed

    Landfried, M J; Stenclik, M; Susi, J G

    1991-01-01

    Fractures of the radial shaft associated with disruption of the distal ulnar radial joint (Galeazzi fracture) account for a small percentage of forearm fractures in adults but occur even more rarely in children. An equivalent injury in children is a fracture of the radius with separation of the distal ulnar physis in the form of a Salter-Harris type II epiphyseal fracture. Although this lesion has been mentioned in the literature, to our knowledge it has not been described in detail. We report three cases of this fracture. All cases were treated by open reduction internal fixation.

  2. 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.

  3. Displaced juvenile Tillaux fractures : Surgical treatment and outcome.

    PubMed

    Tiefenboeck, Thomas M; Binder, Harald; Joestl, Julian; Tiefenboeck, Michael M; Boesmueller, Sandra; Krestan, Christian; Schurz, Mark

    2017-03-01

    Approximately 15 % of all juvenile injuries of the long bones involve the epiphyseal growth plate, and 2.9 % of these are juvenile Tillaux fractures. The Tillaux fracture is of great importance because it involves a major weight-bearing articular surface. Treatment protocols in the literature are not uniform for this kind of fracture, and numerous case reports can be found describing various treatment methods. The aim of this study was to present the clinical outcome at long-term follow-up after treatment of displaced Tillaux fractures. In all, 168 children and adolescent patients with physeal injuries of the distal tibia were treated from 2003 to 2012. Seven patients were identified as having Tillaux fractures requiring surgical treatment and therefore were included in our study and evaluated retrospectively. Seven patients with Tillaux fractures underwent surgical reconstruction by open or closed reduction. Excellent results were achieved in 90 % of the patients, with a mean Foot and Ankle Score at the last follow-up of 98.71. Anatomical reduction is required for every displaced epiphyseal fracture via open reduction and internal fixation, especially in cases with ≥2 mm fragment displacement. Plaster cast immobilization and non-weight-bearing mobilization for at least 4 weeks might be a good way of ensuring optimal surgical results and preventing complications.

  4. Further Data on Sesamoid Identity from Two Anuran Species.

    PubMed

    Corina Vera, Miriam; Laura Ponssa, María; Abdala, Virginia

    2015-08-01

    Considering that the identification of equivalent entities is the basis for any comparative analysis, we compare the histology, histochemistry, shape and dimensions of epiphyses, carpal and sesamoids in two anuran frogs. Our goal was to explore the morphological correspondence among these three skeletal elements in order to clarify the sesamoid identity. We studied the skeletogenesis, contour geometric morphometry and dimensions of forelimb elements of juveniles of two anurans species Leptodactylus bufonius and Rhinella arenarum. Skeletogenesis in anurans present a common trait between carpals and sesamoids: both elements exhibit endochondral ossification. A difference between these elements is the presence of fibrocartilage in the development of sesamoids. The geometric morphometry does not allow us to establish a shape pattern that can be compared either between sesamoids and epiphyses or carpals. With regard to dimensions, our data indicate that bones categorization based on these aspects is ambiguous and therefore is useless to classify of skeletal bones. The data about tissue differentiation of sesamoids provide evidence that support the idea that these elements should be considered part of the typical endowment of the vertebrate skeleton. © 2015 Wiley Periodicals, Inc.

  5. 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.

  6. Salter-Harris type II proximal humerus injuries: state-of-the-art treatment.

    PubMed

    Verdano, Michele Arcangelo; Pellegrini, Andrea; Lunini, Enricomaria; Porcellini, Giuseppe; Ceccarelli, Francesco

    2012-12-01

    Proximal epiphyseal injuries of the humerus represent a very low percentage of traumatic growth pathologies, 5 % of all fractures during childhood. In the literature, there have been only a limited number of clinical studies investigating these injuries, basically from an epidemiological point of view, focusing on the incidence of the different types of fractures. We report our experience of 6 young patients with Salter-Harris type II proximal humerus epiphyseal injuries adopting a minimally invasive surgical technique consisting of closed reduction and percutaneous fixation with Kirschner wires. At 2 years of follow-up, the results consisting in constant; disabilities of the arm, shoulder, and hand; simple shoulder test; and Visual Analogue Scale scores obtained have been excellent and all the patients come back to a normal life with sports practice and normal daily activities. The range of motion was completely restored without any deficit in abduction-adduction, flection-extension, or intra-extra rotation. No differences in anthropometric parameters were found with no case of malangulation, vascular, or neurological complications. Our data support with evidence how the close reduction internal fixation with K-wires treatment can give to the surgeons and the patients a better security about the correct fracture healing. Furthermore, with this article, we will provide a detailed review of the literature in order to define the state-of-the-art treatment to better face such a challenging skeletal injury.

  7. Hoverboards: spectrum of injury and association with an uncommon fracture.

    PubMed

    Schapiro, Andrew H; Lall, Neil U; Anton, Christopher G; Trout, Andrew T

    2017-04-01

    Self-balancing electric scooters, commonly known as hoverboards, are a new and popular consumer item with recognized fall hazards. The spectrum of injuries associated with hoverboard use has not been studied. The purpose of this study is to determine the spectrum of radiologically apparent injuries associated with hoverboard use. We retrospectively reviewed all imaging studies interpreted at our institution for hoverboard-related injuries during an 8-month period. We recorded patient demographics and injury characteristics. Thirty-two of the 47 pediatric patients imaged for hoverboard-related injury had radiologically detectable injuries, all fractures. Fifty percent of these 32 patients were female and 50% were male, with a mean age of 12.4 years. There were 42 fractures total, all involving the appendicular skeleton, with 74% in the upper extremities and 26% in the lower extremities. Thirty-eight percent of the fractures involved the physis. A distinct injury pattern was seen in three patients who sustained open distal phalanx juxta-epiphyseal fractures. Most of the fractures sustained during hoverboard use are commonly seen in everyday pediatric radiology practice, with an overall pattern paralleling that reported in association with skateboard use. However an otherwise uncommon fracture, the distal phalanx juxta-epiphyseal fracture, was identified in association with hoverboard use, and this finding has important treatment implications including need for irrigation and debridement, antibiotic therapy, and potential surgical fixation.

  8. Prepubertal onset of slipped capital femoral epiphysis associated with hypothyroidism: a case report and literature review.

    PubMed

    Kadowaki, Saori; Hori, Tomohiro; Matsumoto, Hideki; Kanda, Kaori; Ozeki, Michio; Shirakami, Yu; Kawamoto, Norio; Ohnishi, Hidenori; Fukao, Toshiyuki

    2017-09-18

    Slipped capital femoral epiphysis (SCFE) is a common hip disorder characterized by displacement of the capital femoral epiphysis from the metaphysic through the femoral epiphyseal plate. SCFE usually occurs during puberty, with obesity a common risk factor. We experienced a rare case of SCFE associated with hypothyroidism in a prepubescent patient who was not obese. The patient was an 8-year-old boy suffering from bilateral SCFE with hypothyroidism. The patient's growth had started to slow at 4 years of age, and at 8 years he was of short stature. During his evaluation for SCFE management, primary hypothyroidism was diagnosed due to the presence of anti-thyroid peroxidase and anti-thyroglobulin antibodies. After the patient was treated for hypothyroidism, which improved his thyroid function, surgery was performed for bilateral SCFE. Among the 42 patients with SCFE associated with hypothyroidism in the literature, most SCFE occurred during puberty or in adults with delayed epiphyseal closure. Only two patients (4.8%), including the present patient, were ≤9 years old. Although being overweight or obese is common for patients with SCFE associated with hypothyroidism (76.0%), it was not observed in the present case. Persistent hypothyroidism, however, may be a risk factor for SCFE even before puberty and without obesity.

  9. 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. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  10. 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.

  11. Influence of alpha-escin on skeletal changes in ovariectomized rats.

    PubMed

    Pytlik, M; Cegieła, U; Janiec, W

    2000-01-01

    The aim of the present study is to investigate the influence of alpha-escin (35 mg/kg per os daily; administered for 4 weeks) on the development of osteopenia caused by bilateral ovariectomy in 3-month-old female Wistar rats. The experiments were carried out on four groups of animals: I (C)--control sham operated rats, II (OVX)--ovariectomized rats, III (E)--sham operated rats which were administered alpha-escin, IV (OVX + E)--ovariectomized rats which were given alpha-escin. In all groups body weight growth, masses, length and tibia diameter were determined as well as histological specimens of right femur and tibia slices were used for histometric measurements including: the transverse cross-section area of the tibial shaft cortex, transverse cross-section area of the tibial marrow cavity, periosteal osteoid width, endosteal osteoid width, periosteal thickness growth in the tibia, endosteal thickness growth in the tibia, epiphyseal and metaphyseal trabeculae thickness in the femur and epiphyseal cartilage width. Bilateral ovariectomy in matured female rats caused osteopenic skeletal changes. alpha-Escin (35 mg/kg per os daily) administered to the ovariectomized rats for the following 28 days decreased only a little the development of osteopenic skeletal changes caused by bilateral ovariectomy.

  12. 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.

  13. 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.

  14. [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.

  15. 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.

  16. An approach for determining quantitative measures for bone volume and bone mass in the pediatric spina bifida population.

    PubMed

    Horenstein, Rachel E; Shefelbine, Sandra J; Mueske, Nicole M; Fisher, Carissa L; Wren, Tishya A L

    2015-08-01

    The pediatric spina bifida population suffers from decreased mobility and recurrent fractures. This study aimed to develop a method for quantifying bone mass along the entire tibia in youth with spina bifida. This will provide information about all potential sites of bone deficiencies. Computed tomography images of the tibia for 257 children (n=80 ambulatory spina bifida, n=10 non-ambulatory spina bifida, n=167 typically developing) were analyzed. Bone area was calculated at regular intervals along the entire tibia length and then weighted by calibrated pixel intensity for density weighted bone area. Integrals of density weighted bone area were used to quantify bone mass in the proximal and distal epiphyses and diaphysis. Group differences were evaluated using analysis of variance. Non-ambulatory children suffer from decreased bone mass in the diaphysis and proximal and distal epiphyses compared to ambulatory and control children (P≤0.001). Ambulatory children with spina bifida showed statistically insignificant differences in bone mass in comparison to typically developing children at these sites (P>0.5). This method provides insight into tibial bone mass distribution in the pediatric spina bifida population by incorporating information along the whole length of the bone, thereby providing more information than dual-energy x-ray absorptiometry and peripheral quantitative computed tomography. This method can be applied to any population to assess bone mass distribution across the length of any long bone. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Cartilage ultrastructure after high pressure freezing, freeze substitution, and low temperature embedding. I. Chondrocyte ultrastructure--implications for the theories of mineralization and vascular invasion

    PubMed Central

    1984-01-01

    Electron microscopic examination of epiphyseal cartilage tissue processed by high pressure freezing, freeze substitution, and low temperature embedding revealed a substantial improvement in the preservation quality of intracellular organelles by comparison with the results obtained under conventional chemical fixation conditions. Furthermore, all cells throughout the epiphyseal plate, including the terminal chondrocyte adjacent to the region of vascular invasion, were found to be structurally integral. A zone of degenerating cells consistently observed in cartilage tissue processed under conventional chemical fixation conditions was not apparent. Hence, it would appear that cell destruction in this region occurs during chemical processing and is not a feature of cartilage tissue in the native state. Since these cells are situated in a region where tissue calcification is taking place, the implication is that the onset and progression of cartilage calcification are, at least partially, controlled by the chondrocytes themselves. The observation that the terminal cell adjacent to the zone of vascular invasion is viable has important implications in relation to the theory of vascular invasion. This may now require reconceptualization to accommodate the possibility that active cell destruction may be a precondition for vascular invasion. PMID:6707090

  18. The surgical anatomy of the blood supply to the femoral head: description of the anastomosis between the medial femoral circumflex and inferior gluteal arteries at the hip.

    PubMed

    Grose, A W; Gardner, M J; Sussmann, P S; Helfet, D L; Lorich, D G

    2008-10-01

    The inferior gluteal artery is described in standard anatomy textbooks as contributing to the blood supply of the hip through an anastomosis with the medial femoral circumflex artery. The site(s) of the anastomosis has not been described previously. We undertook an injection study to define the anastomotic connections between these two arteries and to determine whether the inferior gluteal artery could supply the lateral epiphyseal arteries alone. From eight fresh-frozen cadaver pelvic specimens we were able to inject the vessels in 14 hips with latex moulding compound through either the medial femoral circumflex artery or the inferior gluteal artery. Injected vessels around the hip were then carefully exposed and documented photographically. In seven of the eight specimens a clear anastomosis was shown between the two arteries adjacent to the tendon of obturator externus. The terminal vessel arising from this anastomosis was noted to pass directly beneath the posterior capsule of the hip before ascending the superior aspect of the femoral neck and terminating in the lateral epiphyseal vessels. At no point was the terminal vessel found between the capsule and the conjoined tendon. The medial femoral circumflex artery receives a direct supply from the inferior gluteal artery immediately before passing beneath the capsule of the hip. Detailed knowledge of this anatomy may help to explain the development of avascular necrosis after hip trauma, as well as to allow additional safe surgical exposure of the femoral neck and head.

  19. Growth of long bones in renal failure: roles of hyperparathyroidism, growth hormone and calcitriol.

    PubMed

    Sanchez, C P; Salusky, I B; Kuizon, B D; Abdella, P; Jüppner, H; Goodman, W G

    1998-12-01

    The treatment of secondary hyperparathyroidism (2 degrees HPT) associated with chronic renal failure adversely affects skeletal growth. We assessed epiphyseal growth plate morphology by quantitative histology and measured mRNA levels for selected markers of chondrocyte proliferation and differentiation by in situ hybridization in the growth plate cartilage of subtotally nephrectomized rats with either mild or advanced 2 degrees HPT. The width of the growth plate cartilage in the proximal tibia and mRNA levels for PTH/PTHrP receptor were unchanged in rats with mild 2 degrees HPT, however, they were markedly less in nephrectomized rats with advanced 2 degrees HPT than in intact controls. Treatment with growth hormone 10 IU/kg/day increased growth plate thickness both in mild and in advanced 2 degrees HPT and raised mRNA levels for type II and type X collagen in rats with advanced 2 degrees HPT. The administration of calcitriol 50 ng/kg/day attenuated these responses in animals with advanced 2 degrees HPT. Overall, PTH/PTHrP receptor mRNA levels did not correspond to the serum levels of PTH indicating that PTH/PTHrP receptor expression is down-regulated in renal failure by a PTH-independent mechanism. Calcitriol counteracts the trophic actions of growth hormone on epiphyseal growth plate cartilage and modifies chondrocyte differentiation in vivo, and these mechanisms may contribute to disturbances in longitudinal bone growth in renal failure.

  20. VITAMIN A AND ENDOCHONDRAL OSSIFICATION IN THE RAT AS INDICATED BY THE USE OF SULFUR-35 AND PHOSPHORUS-32

    PubMed Central

    Dziewiatkowski, Dominic D.

    1954-01-01

    The administration of vitamin A to vitamin A-deficient rats resulted in a decreased concentration of inorganic sulfate-sulfur in the serum from a value of 2.5 mg. per cent to 1.8 mg. per cent, the latter being close to the value of 2.0 mg. per cent found in normal rats of the same age. The uptake of sulfate and phosphate by femurs and tibiae of vitamin A-deficient rats was less than that in normal rats of the same age. An increased uptake followed the administration of vitamin A: radioautography indicated that in the case of sulfate, its uptake was particularly increased in the epiphyseal cartilage; an increased uptake of phosphate was particularly evident in the diaphysis immediately adjacent to the epiphyseal cartilage plate. The specific activity of the sulfate-sulfur in the chondroitin sulfate samples isolated from the skeletons of vitamin A-deficient rats fell progressively as the deficiency continued. Following administration of vitamin A, the specific activity approached and exceeded the value given by the sample from the skeletons of normal rats of the same age. A substantial increase was found in the value of the specific activity of the sulfate-sulfur of sulfomucopolysaccharides isolated from skins of vitamin A-deficient rats that had been given vitamin A. Following administration of vitamin A to rats deficient in this vitamin, an increased accumulation of some sulfur-containing material was found in regions of active calcification. PMID:13163335

  1. Morphological analysis of the proximal femur using quantitative computed tomography

    PubMed Central

    Jacobson, Donald; Carrera, Guilermo

    2006-01-01

    The anatomy of the proximal femur was studied in 35 specimens using quantitative computed tomography (QCT) and compared with anatomical sections studied by plane radiography and gross dissection. We found the primary supporting structure of the femoral head to be the primary compressive strut, which is a dense column of trabecular bone projecting from the pressure buttress of the medial femoral neck to the epiphyseal scar. Trabecular bone mushroomed from the epiphyseal scar and terminated at right angles to the cortex of the femoral head. We believe the primary compressive strut is the predominant load-bearing structure connecting the femoral head to the femoral neck, as many specimens lacked continuity of the head cortex to the femoral neck. Based on the CT number, the primary compressive strut had similar bone density to cortical structures such as the lesser trochanter, calcar femorale and posterior lateral femoral cortex. Ward’s triangle lacked structural integrity in many cases, and we doubt the significance of tensile trabculae for sharing load. Surgical techniques such as femoral fracture fixation, resurfacing hip arthroplasty and allograft transplantation may benefit from this knowledge. PMID:16896872

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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

  7. 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.

  8. 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

  9. 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.

  10. The speed of sound correlates with implant insertion torque in rabbit bone: an in vitro experiment.

    PubMed

    Veltri, M; Valenti, R; Ceccarelli, E; Balleri, P; Nuti, R; Ferrari, M

    2010-07-01

    This study investigated the correlation of quantitative ultrasound with bone quality as evaluable from implant final insertion torque. Implants were planned at diaphyses (group 1) and epiphyses (group 2) of 16 rabbit femurs where amplitude-dependent speed of sound (Ad-SOS) was measured. The insertion torque from 7-mm-long implants placed at planned sites was recorded. The correlation between cutting torque and Ad-SOS was evaluated using Spearman's coefficient. Statistics were based on data from 15 diaphyses and 13 epiphyses. The mean insertion torque was 8.8 N cm while the mean Ad-SOS was 1710.9 m/s. A negative correlation resulted between insertion torque and Ad-SOS. In the rabbit bone model investigated, quantitative ultrasound correlates inversely with implant insertion torque. Although this correlation remains to be verified in humans because rabbit femur does not convincingly represents different human bone qualities, it seems that ultrasound could convey potentially useful, pre-surgical, site-specific, non-invasive information on bone mechanical characteristics therefore deserving further research efforts.

  11. 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.

  12. 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.

  13. 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.

  14. Thyroid diseases and bone health.

    PubMed

    Williams, G R; Bassett, J H D

    2017-08-29

    Thyroid hormones are essential for skeletal development and are important regulators of bone maintenance in adults. Childhood hypothyroidism causes delayed skeletal development, retarded linear growth and impaired bone mineral accrual. Epiphyseal dysgenesis is evidenced by classic features of stippled epiphyses on X-ray. In severe cases, post-natal growth arrest results in a complex skeletal dysplasia. Thyroid hormone replacement stimulates catch-up growth and bone maturation, but recovery may be incomplete dependent on the duration and severity of hypothyroidism prior to treatment. A severe phenotype characteristic of hypothyroidism occurs in children with resistance to thyroid hormone due to mutations affecting THRA encoding thyroid hormone receptor α (TRα). Discovery of this rare condition recapitulated animal studies demonstrating that TRα mediates thyroid hormone action in the skeleton. In adults, thyrotoxicosis is well known to cause severe osteoporosis and fracture, but cases are rare because of prompt diagnosis and treatment. Recent data, however, indicate that subclinical hyperthyroidism is associated with low bone mineral density (BMD) and an increased risk of fracture. Population studies have also shown that variation in thyroid status within the reference range in post-menopausal women is associated with altered BMD and fracture risk. Thus, thyroid status at the upper end of the euthyroid reference range is associated with low BMD and increased risk of osteoporotic fragility fracture. Overall, extensive data demonstrate that euthyroid status is required for normal post-natal growth and bone mineral accrual, and is fundamental for maintenance of adult bone structure and strength.

  15. 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.

  16. Whole-body MRI reveals high incidence of osteonecrosis in children treated for Hodgkin lymphoma.

    PubMed

    Littooij, Annemieke S; Kwee, Thomas C; Enríquez, Goya; Verbeke, Jonathan I M L; Granata, Claudio; Beishuizen, Auke; de Lange, Charlotte; Zennaro, Floriana; Bruin, Marrie C A; Nievelstein, Rutger A J

    2017-02-01

    Osteonecrosis is a well-recognized complication in patients treated with corticosteroids. The incidence of osteonecrosis in children treated for Hodgkin lymphoma is unknown because prospective whole-body magnetic resonance imaging (MRI) studies are lacking in this patient population. Paediatric patients with newly diagnosed Hodgkin lymphoma who were treated according to a uniform paediatric Hodgkin protocol were eligible for inclusion in this prospective study. Whole-body MRI was performed in all 24 included patients (mean age 15·1 years, 12 girls) both before treatment and after 2 cycles of chemotherapy, and in 16 patients after completion of chemotherapy. Osteonecrosis was identified in 10 patients (41·7%, 95% confidence interval: 22·0-61·4%), with a total of 56 osteonecrotic sites. Osteonecrosis was detected in 8 patients after 2 cycles of OEPA (vincristine, etoposide, prednisone, doxorubicin), and in 2 additional patients after completion of chemotherapy. Epiphyseal involvement of long bones was seen in 4 of 10 children. None of the patients with osteonecrosis had any signs of bone collapse at the times of scanning. Whole-body MRI demonstrates osteonecrosis to be a common finding occurring during therapy response assessment of paediatric Hodgkin lymphoma. Detection of early epiphyseal osteonecrosis could allow for treatment before bone collapse and joint damage may occur. © 2016 John Wiley & Sons Ltd.

  17. 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

  18. 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

  19. A pediatric comminuted talar fracture treated by minimal K-wire fixation without using a tourniquet.

    PubMed

    Inal, Sermet; Inal, Canan

    2014-01-01

    Pediatric comminuted talar fractures are reported to be rare, and treatment options such as minimal internal K-wire fixation without using a tourniquet to prevent avascular necrosis have not previously been investigated. We report a case of a comminuted talar body and a non-displaced neck fracture with dislocation of the tibiotalar, talonavicular and subtalar joints with bimalleolar epiphyseal fractures in an 11-year-old boy due to a fall from height. We present radiological findings, the surgical procedure and clinical outcomes of minimal internal K-wire fixation without using a tourniquet. Avascular necrosis rates are reported to be between 0 % and 66 % after fractures of the neck of the talus and the talar body in children. The likelihood of developing avascular necrosis increases with the severity of the fracture. To avoid avascular necrosis in a comminuted talar fracture accompanied by tibiotalar, talonavicular, subtalar dislocations and bimalleolar epiphyseal fractures, a minimal internal K-wire fixation without the use of a tourniquet was performed. The outcome was evaluated by the American Orthopedic Foot and Ankle Society score (AOFAS). A score of 90 (excellent) was found at the end of the second year of follow up. Radiology revealed preservation of the joint with no evidence of avascular necrosis, and clinical findings revealed a favorable functional outcome after two years. 4.

  20. Metaphyseal bone collapse mimicking slipped capital femoral epiphysis in severe renal osteodystrophy.

    PubMed

    Takao, Masaki; Hashimoto, Jun; Sakai, Takashi; Nishii, Takashi; Sugano, Nobuhiko; Yoshikawa, Hideki

    2012-11-01

    Slipped capital femoral epiphysis is a well-recognized skeletal complication of renal osteodystrophy in adolescence, which is distinct from idiopathic slipped capital femoral epiphysis in its etiology. We report a case of severe mixed-type renal osteodystrophy with metaphyseal bone collapse that mimicked slipped capital femoral epiphyses. Case history, laboratory and radiological evaluation, and bone biopsies are discussed. A 14-yr-old girl presented with left hip pain showing bilateral metaphyseal bone collapse accompanied with posterior-inferior displacement of capital femoral epiphyses after 2.5 yr of GH treatment. Blood chemistry, showing high levels of alkaline phosphatase and PTH, and a transiliac bone biopsy, indicating severe osteomalacia with osteitis fibrosa, along with serial computed tomography images of the hips from the presymptomatic stage, led to accurate diagnosis and successful treatment by administration of high-dose vitamin D. This case emphasizes the importance of controlling hyperparathyroidism well in children with chronic renal insufficiency, particularly at adolescence before initiating GH treatment. When children with renal insufficiency present with displacement of capital femoral epiphysis, it is necessary to evaluate the serum levels of alkaline phosphatase and PTH and metaphyseal bone quality below the physis.

  1. Orthopedics management of acromicric dysplasia: follow up of nine patients.

    PubMed

    Klein, Céline; Le Goff, Carine; Topouchian, Vicken; Odent, Sylvie; Violas, Philippe; Glorion, Christophe; Cormier-Daire, Valérie

    2014-02-01

    Acromicric dysplasia (AD) is an autosomal dominant disorder characterized by short stature, short extremities, stiff joint and skeleton features including brachymetacarpia, cone-shaped epiphyses, internal notch of the femoral head, and delayed bone age. Recently, we identified fibrillin 1 (FBN1) as the disease gene of AD. The aim of our study was to further describe the long-term follow up of AD patients with an emphasis on orthopedic management. Nine patients with FBN1 mutations were included in the study ranging in age from 5.5 to 64 years. For all, detailed clinical and radiological data were available. Birth parameters were always normal and patients progressively developed short stature <-3 SD. Carpal tunnel syndrome was observed in four patients. We found discrepancy between the carpal bone age and the radius and ulna epiphysis bone ages, a variable severity of hip dysplasia with acetabular dysplasia, epiphyseal and metaphyseal femoral dysplasia resembling Legg-Perthes-Calvé disease and variable pelvic anteversion and hyperlordosis. Orthopedic surgery was required in two patients for hip dysplasia, in one for limb lengthening and in three for carpal tunnel syndrome. Our observations expand the AD phenotype and emphasize the importance of regular orthopedic survey. © 2013 Wiley Periodicals, Inc.

  2. Detection of acute diazepam exposure in bone and marrow: influence of tissue type and the dose-death interval on sensitivity of detection by ELISA with liquid chromatography tandem mass spectrometry confirmation.

    PubMed

    Watterson, James H; Botman, Jolina E

    2009-05-01

    Enzyme-linked immunosorbent assay (ELISA) and liquid chromatography tandem mass spectrometry (LC/MS/MS) were used to detect diazepam exposure in skeletal tissues of rats (n = 15) given diazepam acutely (20 mg/kg, i.p.), and killed at various times postdose. Marrow, epiphyseal, and diaphyseal bone were isolated from extracted femora. Bone was cleaned, ground, and incubated in methanol. Marrow underwent ultrasonic homogenization. Extracts and homogenates were diluted in phosphate buffer, and then underwent solid-phase extraction and ELISA. Relative sensitivity of detection was examined in terms of relative decrease in absorbance (ELISA) and binary classification sensitivity (ELISA and LC/MS/MS). Overall, the data showed differences in relative sensitivity of detection of diazepam exposure in different tissue types (marrow > epiphyseal bone > diaphyseal bone), which is suggestive of heterogenous distribution in these tissues, and a decreasing sensitivity with increasing dose-death interval. Thus, the tissue type sampled and dose-death interval may contribute to the probability of detection of diazepam exposure in skeletal tissues.

  3. Fetal Mesenchymal Stromal Cells Differentiating towards Chondrocytes Acquire a Gene Expression Profile Resembling Human Growth Plate Cartilage

    PubMed Central

    Leijten, Jeroen C. H.; Decker, Eva; Sticht, Carsten; van Houwelingen, Johannes C.; Goeman, Jelle J.; Kleijburg, Carin; Scherjon, Sicco A.; Gretz, Norbert; Wit, Jan Maarten; Rappold, Gudrun; Post, Janine N.; Karperien, Marcel

    2012-01-01

    We used human fetal bone marrow-derived mesenchymal stromal cells (hfMSCs) differentiating towards chondrocytes as an alternative model for the human growth plate (GP). Our aims were to study gene expression patterns associated with chondrogenic differentiation to assess whether chondrocytes derived from hfMSCs are a suitable model for studying the development and maturation of the GP. hfMSCs efficiently formed hyaline cartilage in a pellet culture in the presence of TGFβ3 and BMP6. Microarray and principal component analysis were applied to study gene expression profiles during chondrogenic differentiation. A set of 232 genes was found to correlate with in vitro cartilage formation. Several identified genes are known to be involved in cartilage formation and validate the robustness of the differentiating hfMSC model. KEGG pathway analysis using the 232 genes revealed 9 significant signaling pathways correlated with cartilage formation. To determine the progression of growth plate cartilage formation, we compared the gene expression profile of differentiating hfMSCs with previously established expression profiles of epiphyseal GP cartilage. As differentiation towards chondrocytes proceeds, hfMSCs gradually obtain a gene expression profile resembling epiphyseal GP cartilage. We visualized the differences in gene expression profiles as protein interaction clusters and identified many protein clusters that are activated during the early chondrogenic differentiation of hfMSCs showing the potential of this system to study GP development. PMID:23144774

  4. 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.

  5. 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.

  6. 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.

  7. 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%.

  8. The development of curvature in the porcine radioulna.

    PubMed

    Pantinople, Jess; McCabe, Kyle; Henderson, Keith; Richards, Hazel L; Milne, Nick

    2017-01-01

    Long bone curvature in animal limbs has long been a subject of interest and much work has explored why long bones should be curved. However, the 'when' and 'how' of curvature development is poorly understood. It has been shown that the rat tibia fails to attain its normal curvature if the action of muscles is removed early in life, but it is not clear if this is because the curvature fails to develop or if the bone becomes straighter without the action of muscles. No studies have examined the development of bone curvature in a normally developing quadruped, so this study tracks the course of curvature formation in the radioulna in a series of growing pigs. We also histologically examined the epiphyseal growth plates of these bones to determine if they contribute to the formation of curvature. In all three epiphyseal plates examined, the proliferative zone is thicker and more densely populated with chondrocytes on the cranial (convex) side than the caudal (concave) side. Frost's chondral modelling theory would suggest that the cranial side of the bone is under more compression than the caudal side, and we conclude that this is due to the action of triceps extending the elbow by pulling on the olecranon process. These results support the idea that bone curvature is an adaptation to habitual loading, where longitudinal loads acting on the curved bone cause bending strains that counter the bending resulting from the habitual muscle action.

  9. Lead and Zinc Poisoning and the Interaction Between Pb and Zn Poisoning in the Foal

    PubMed Central

    Willoughby, R. A.; MacDonald, E.; McSherry, B. J.; Brown, G.

    1972-01-01

    Groups of young growing horses were fed toxic amounts of lead only, zinc only and the same amounts of lead and zinc together. Those fed Pb only developed pharyngeal and laryngeal paralysis (“roaring”) whereas those fed Zn only and Pb and Zn together developed the same clinical syndrome which included swelling at the epiphyseal region of the long bones, stiffness and lameness. Anemia and decreased weight gains were most pronounced in animals fed Zn for the longest periods. Animals fed Pb only did not become anemic and weight loss did not occur until after there was an interference in swallowing. The clinical signs and tissue Pb values from animals fed toxic amounts of both Pb and Zn continuously, differed markedly from those present in animals fed comparable amounts of Pb only. Th clinical signs were similar to those caused by Zn poisoning. The hepatic and renal tissue Pb values were approximately twice as high and the epiphyseal and cancellous bone sample results were one half as high as the comparable Pb values from animals fed toxic amounts of Pb only. It appeared that toxic amounts of Zn prevented the development of clinical signs of Pb poisoning in the young growing horse. ImagesFig. 2.Fig. 3.Fig. 10. PMID:4263914

  10. 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.

  11. 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

  12. Estimating gestational age in the term pregnancy with a model based on multiple indices of fetal maturity.

    PubMed

    Goldstein, I; Reece, E A; O'Connor, T Z; Hobbins, J C

    1989-11-01

    A prospective ultrasonographic study was conducted in 100 normal pregnant women with gestational ages that ranged from 36 to 42 weeks in which multiple biometric measurements were obtained. Dimensions of the distal femoral, proximal tibial, and proximal humeral epiphyseal ossification centers, as well as the placental and colonic grades, were also evaluated. A high statistical correlation was found between gestational age and each of the five variables (p less than 0.001). With a stepwise linear logistic regression analysis, we determined that gestational ages (36 to 39 weeks and 40 to 42 weeks) could be ascertained with a high probability with the use of a combination of the proximal humeral epiphysis and colonic grades. Probability estimates were not significantly affected by the addition of the distal femoral, proximal tibial epiphyses, placental grade, or amniotic fluid volume. From these data, probability prediction tables were generated. The results of this study provide an alternate method by which gestational age may be estimated in late pregnancy.

  13. Histomorphometric Parameters of the Growth Plate and Trabecular Bone in Wild-Type and Trefoil Factor Family 3 (Tff3)-Deficient Mice Analyzed by Free and Open-Source Image Processing Software.

    PubMed

    Bijelić, Nikola; Belovari, Tatjana; Stolnik, Dunja; Lovrić, Ivana; Baus Lončar, Mirela

    2017-08-01

    Trefoil factor family 3 (Tff3) peptide is present during intrauterine endochondral ossification in mice, and its deficiency affects cancellous bone quality in secondary ossification centers of mouse tibiae. The aim of this study was to quantitatively analyze parameters describing the growth plate and primary ossification centers in tibiae of 1-month-old wild-type and Tff3 knock-out mice (n=5 per genotype) by using free and open-source software. Digital photographs of the growth plates and trabecular bone were processed by open-source computer programs GIMP and FIJI. Histomorphometric parameters were calculated using measurements made with FIJI. Tff3 knock-out mice had significantly smaller trabecular number and significantly larger trabecular separation. Trabecular bone volume, trabecular bone surface, and trabecular thickness showed no significant difference between the two groups. Although such histomorphological differences were found in the cancellous bone structure, no significant differences were found in the epiphyseal plate histomorphology. Tff3 peptide probably has an effect on the formation and quality of the cancellous bone in the primary ossification centers, but not through disrupting the epiphyseal plate morphology. This work emphasizes the benefits of using free and open-source programs for morphological studies in life sciences.

  14. 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.

  15. Galeazzi-equivalent Fractures: Report of Two Cases and Literature Review.

    PubMed

    Suganuma, Seigo; Tada, Kaoru; Yamamoto, Daiki; Tsuchiya, Hiroyuki

    2017-06-01

    The Galeazzi-equivalent fracture is a rare injury that occurs in children. The most important issue is the distal ulnar epiphyseal injury. Although there have been some case reports, most of them performed only short term follow-up. This article describes two cases of this fracture with long term follow-up until epiphyseal closure. First case is a 12-year-old girl who sustained a Galeazziequivalent fracture of her right forearm and underwent emergency surgery. At follow-up of 5 years and 10 months postsurgery, radiographs show ulnar growth arrest of one mm and she has mild pain. Second case is a 15-year-old boy who sustained an open Galeazzi-equivalent fracture of his left forearm and underwent emergency surgery. At follow-up of 3 years and 3 months postsurgery, radiographs show no growth arrest of the distal ulna. He has no residual complaint. Long term follow-up is absolutely necessary to monitor ulnar growth.

  16. Spaceflight-Induced Bone Loss Alters Failure Mode and Reduces Bending Strength in Murine Spinal Segments

    PubMed Central

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

    2017-01-01

    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. PMID:26285046

  17. Intra-articular injections of high-molecular-weight hyaluronic acid have biphasic effects on joint inflammation and destruction in rat antigen-induced arthritis

    PubMed Central

    Roth, Andreas; Mollenhauer, Jürgen; Wagner, Andreas; Fuhrmann, Reneè; Straub, Albrecht; Venbrocks, Rudolf A; Petrow, Peter; Bräuer, Rolf; Schubert, Harald; Ozegowski, Jörg; Peschel, Gundela; Müller, Peter J; Kinne, Raimund W

    2005-01-01

    To assess the potential use of hyaluronic acid (HA) as adjuvant therapy in rheumatoid arthritis, the anti-inflammatory and chondroprotective effects of HA were analysed in experimental rat antigen-induced arthritis (AIA). Lewis rats with AIA were subjected to short-term (days 1 and 8, n = 10) or long-term (days 1, 8, 15 and 22, n = 10) intra-articular treatment with microbially manufactured, high-molecular-weight HA (molecular weight, 1.7 × 106 Da; 0.5 mg/dose). In both tests, 10 buffer-treated AIA rats served as arthritic controls and six healthy animals served as normal controls. Arthritis was monitored by weekly assessment of joint swelling and histological evaluation in the short-term test (day 8) and in the long-term test (day 29). Safranin O staining was employed to detect proteoglycan loss from the epiphyseal growth plate and the articular cartilage of the arthritic knee joint. Serum levels of IL-6, tumour necrosis factor alpha and glycosaminoglycans were measured by ELISA/kit systems (days 8 and 29). HA treatment did not significantly influence AIA in the short-term test (days 1 and 8) but did suppress early chronic AIA (day 15, P < 0.05); however, HA treatment tended to aggravate chronic AIA in the long-term test (day 29). HA completely prevented proteoglycan loss from the epiphyseal growth plate and articular cartilage on day 8, but induced proteoglycan loss from the epiphyseal growth plate on day 29. Similarly, HA inhibited the histological signs of acute inflammation and cartilage damage in the short-term test, but augmented acute and chronic inflammation as well as cartilage damage in the long-term test. Serum levels of IL-6, tumour necrosis factor alpha, and glycosaminoglycans were not influenced by HA. Local therapeutic effects of HA in AIA are clearly biphasic, with inhibition of inflammation and cartilage damage in the early chronic phase but with promotion of joint swelling, inflammation and cartilage damage in the late chronic phase. PMID

  18. 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

  19. 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

  20. 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

  1. Effect of ciprofoxacin on growing cartilage in albino rat pups.

    PubMed

    Channa, Haji Muhammad Aslam; Ashfaq, Muhammad; Mastoi, Shah Murad; Qureshi, Muhammad Azam

    2006-01-01

    Administration of quinolone therapy is controversial during growing age as stated by earlier worker. The flroquinolones are currently not indicated for young children, because of arthropathy and adverse effect on growing cartilage shown by studies. However the effects of ciprofloxacin on epiphyseal growth plate has remained undocumented. This study is therefore, undertaken to determine the risk of ciprofloxacin administration an growing cartilage by prospective experimental animal study model using Wistar albino rat pups. Ciprofloxacin was administered to newly born Wistar albino rat pups with a doze of 20 mg/kg body weight intraperitonealy twice a day from day-1 to day-14 after birth. The animals were sacrificed by deep ether anesthesia. The limbs were disarticulated from axial skeleton, soft tissue was removed. The intact bone mean length in millimeter of right and left humerus and femur was measured with the help of electronic vernier caliper and bones were fixed in 10% buffered farmalin. Decalcification was done in 10% nitric acid and 10% formic acid changes. After paraplast embeding, 4 microm thick longitudinal sections of the proximal long bones were cut by a rotary microtome. Routine staining with haemotoxylin and eosin was performed. Histomorphometry was done measuring the thickness of epiphyseal cartilage and was compared with similar value of control animals. The results were statistically analysed to find out the significance. The ciprofloxacin induces a mordanting effect as abviated by increased basophilia. Our study reveales that cirprofloxacin administration in the newly born pups decreased the width of epiphyseal growth plate cartilage by 10.43% in humerus and 4.72% in femur as compared to the growth of control cartilage. The decrease in the width was brought about mainly by the reduced count of the proliferative cells in the proliferative zone and the diminuation in the average size of the hypertrophic condryocytes in the hypertrophic zone. The

  2. 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

  3. Using next-generation sequencing for the diagnosis of rare disorders: a family with retinitis pigmentosa and skeletal abnormalities.

    PubMed

    Schrader, Kasmintan A; Heravi-Moussavi, Alireza; Waters, Paula J; Senz, Janine; Whelan, James; Ha, Gavin; Eydoux, Patrice; Nielsen, Torsten; Gallagher, Barry; Oloumi, Arusha; Boyd, Niki; Fernandez, Bridget A; Young, Terry-Lynn; Jones, Steven Jm; Hirst, Martin; Shah, Sohrab P; Marra, Marco A; Green, Jane; Huntsman, David G

    2011-09-01

    Linkage analysis with subsequent candidate gene sequencing is typically used to diagnose novel inherited syndromes. It is now possible to expedite diagnosis through the sequencing of all coding regions of the genome (the exome) or full genomes. We sequenced the exomes of four members of a family presenting with spondylo-epiphyseal dysplasia and retinitis pigmentosa and identified a six-base-pair (6-bp) deletion in GNPTG, the gene implicated in mucolipidosis type IIIγ. The diagnosis was confirmed by biochemical studies and both broadens the mucolipidosis type III phenotype and demonstrates the clinical utility of next-generation sequencing to diagnose rare genetic diseases. Copyright © 2011 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  4. 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.

  5. 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.

  6. 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

  7. 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

  8. Hereditary multiple exostoses: Confirmation of linkage to chromosomes 8 and 11

    SciTech Connect

    Blanton, S.H.; Hogue, D.; Hecht, J.T.

    1996-03-15

    Hereditary multiple exostoses (EXT) is an autosomal dominant disorder characterized by the formation of cartilage capped prominences that develop from the epiphyses of the long bones. EXT is heterogeneous with three different locations currently identified on chromosomes 8, 11, and 19. Recently, we identified and studied 12 large multigenerational EXT families. Linkage analyses demonstrates that 6 of these families map to 8q24 and 6 to 11p. None of the families map to the chromosome 19 locus. The results suggest that there are two major loci, on chromosomes 8 and 11, involved in the cause of EXT. The locus on chromosome 19 remains to be confirmed. 19 refs., 6 figs., 6 tabs.

  9. 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.

  10. Salter-harris type 2 fracture of the proximal phalanx of the thumb with a rotational deformity: a case report and review.

    PubMed

    Izadpanah, Ali; Karunanayake, Mihiran; Izadpanah, Arash; Sinno, Hani; Luc, Mario

    2012-03-01

    Hand fractures are the most common site of injury in the pediatric population. They commonly involve the epiphyseal growth plates, and their standard classification is that of Salter-Harris (SH). Rotational deformities after SH fractures are rarely reported in literature. However, only 5 degrees of angulation can cause evident rotational deformity. This could be seen clinically and reconfirmed with radiologic evaluation. Up to this date, there are only a few cases of SH fractures with rotational deformity that have been described. We present 2 cases of SH type 2 with evidence of rotational deformity, which were reduced under local anesthesia in the emergency department. A review of literature is performed. Thus, examination for rotational deformities in SH fractures should be kept in mind. A satisfactory closed reduction under local anesthesia can be obtained.

  11. Variation of the electric properties along the diaphysis of bovine femoral bone.

    PubMed

    De Mercato, G; García Sánchez, F J

    1991-07-01

    A preliminary study is presented of the variability of the electric properties, in the axial, tangential and radial directions, as a function of position in the diaphysis of a femoral bovine bone. The measurements were carried out at three frequencies: 100 Hz, 10 kHz and 1 MHz. It is shown that both the conductivity and the permittivity exhibit significant variations along the diaphysis, and increase in magnitude towards the epiphyses. From this study, the variation of the electric properties cannot be clearly and directly ascribed to the longitudinal variability of the total volumetric fluid content of the bone. The results reflect the orthotropic nature of the electric properties, at any given location, and indicate a position-dependent tendency towards axis symmetry.

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. Chondrodysplasia punctata and maternal autoimmune disease: a new case and review of the literature.

    PubMed

    Shanske, Alan L; Bernstein, Larry; Herzog, Ronit

    2007-08-01

    Classic rhizomelic chondrodysplasia punctata is a rare, autosomal, recessively inherited disorder that is characterized by proximal shortening of the limbs, punctuate calcifications of the epiphyses, cataracts, developmental delay, and early lethality. A distinctive biochemical profile is characteristic for each of the several defects of peroxisomal metabolism. Recently, cases have been described that were not associated with peroxisomal dysfunction. These cases were found to be secondary to teratogen exposure or maternal conditions. Since 1993, there have been 9 reported cases of neonates with rhizomelic chondrodysplasia punctata who were born to mothers with connective tissue disease. We followed a newborn boy with features suggestive of rhizomelic chondrodysplasia punctata whose biochemical studies failed to demonstrate a defect in either plasmalogen or cholesterol biosynthesis. His mother developed systemic lupus erythematosus 8 months after delivery. This case is compared with the previously reported 9 cases from the literature and is instructive in demonstrating a lesser known effect of maternal autoantibodies on the fetus.

  17. Brachytelephalangic chondrodysplasia punctata: prenatal diagnosis and postnatal outcome.

    PubMed

    Boulet, S; Dieterich, K; Althuser, M; Nugues, F; Durand, C; Charra, C; Schaal, J P; Jouk, P S

    2010-01-01

    We report the prenatal management of a brachytelephalangic chondrodysplasia punctata (CDPX1) case and how postnatal findings confirmed the diagnosis. The mother was initially referred after ultrasound revealed an abnormal fetal mid-face and punctuation of upper femoral epiphyses. Chondrodysplasia punctata (CP) with Binder anomaly was suspected. 3D-HCT revealed brachytelephalangy suggesting CDPX1. At birth, mid-face hypoplasia was marked. Postnatal imaging and genetic analysis confirmed the initial diagnosis. Binder anomaly is probably always associated with CP. The newly revised CP classification facilitates the diagnosis. The main etiologies are metabolic and chromosomal abnormalities, and arylsulfatase E enzyme dysfunction. Thus, screening for arylsulfatase E mutation is mandatory for an accurate diagnosis and can lead to better delineation among CP etiologies associated with a Binder phenotype. Copyright © 2010 S. Karger AG, Basel.

  18. 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.

  19. Injectable biphasic calcium phosphate bioceramic: The HYDROS concept.

    PubMed

    Baroth, Serge; Bourges, Xavier; Goyenvalle, Eric; Aguado, Eric; Daculsi, Guy

    2009-01-01

    A new biphasic calcium phosphate ceramic material has been developed in our laboratory. It is composed of 60% of hydroxyapatite and 40% of beta-tricalcium phosphate, based on three granulometries (submicron, round microporous 80-200 mum and macro microporous 0.5-1 mm particles) and hydrated with water leading the formation of a putty filler for bone repair. Biocompatibility and osteogenicity were tested by filling femoral epiphyses critical size bone defect and lumbar muscles in rabbit. After 3, 6 and 12 weeks of implantation, explants were treated for histology. Results revealed the biocompatibility of the material and intensive resorption of the submicron particle fraction followed by important bone ingrowth whereas osteoconduction was provided by the larger particles.

  20. 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.

  1. 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.

  2. 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.

  3. Clinical and radiological assessment of a family with mild brachydactyly type A1: the usefulness of metacarpophalangeal profiles

    PubMed Central

    Armour, C; Bulman, D; Hunter, A

    2000-01-01

    The brachydactylies are a group of conditions in which various subtypes have been defined based upon the specific pattern of digital bones involved. Type A1 brachydactyly is principally characterised by maximal involvement of the middle phalanges. We report an extended family with a mild brachydactyly A1 which was, except for some short stature, not associated with any of the additional clinical findings reported in several published families. While all the hand bones tended to be small, the principal features of the affected members were shortened middle and distal phalanges, proximal 1st phalanges, and 5th metacarpals. The feet were similarly involved and tended to have a broad, slightly adducted forefoot. The two affected children showed multiple coned epiphyses. This paper provides a detailed description of the family including the radiographic signs and metacarpophalangeal profiles, which proved to be useful in distinguishing the mildly affected persons.


Keywords: brachydactyly A1; metacarpophalangeal profile PMID:10745048

  4. 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

  5. [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.

  6. 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.

  7. Medial joint space widening of the ankle in displaced Tillaux and Triplane fractures in children.

    PubMed

    Gourineni, Prasad; Gupta, Asheesh

    2011-10-01

    Tillaux and Triplane fractures occur in children predominantly from external rotation mechanism. We hypothesized that in displaced fractures, the talus would shift laterally along with the distal fibula and the distal tibial epiphyseal fragment increasing the medial joint space. Consecutive cases evaluated retrospectively. Level I and Level II centers. Twenty-two skeletally immature patients with 14 displaced Triplane fractures and eight displaced Tillaux fractures were evaluated for medial joint space widening. Measurement of fracture displacement and medial joint space widening before and after intervention. Thirteen Triplane and six Tillaux fractures (86%) showed medial space widening of 1 to 9 mm and equal to the amount of fracture displacement. Reduction of the fracture reduced the medial space to normal. There were no known complications. Medial space widening of the ankle may be a sign of ankle fracture displacement. Anatomic reduction of the fracture reduces the medial space and may improve the results in Tillaux and Triplane fractures.

  8. [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.

  9. Proximal humeral osteoarticular allografts: technique, pearls and pitfalls, outcomes.

    PubMed

    Farfalli, German L; Ayerza, Miguel A; Muscolo, D Luis; Aponte-Tinao, Luis A

    2015-12-01

    Allograft transplantation is a biologic reconstruction option for massive bone defects after resection of bone sarcomas. This type of reconstruction not only restores bone stock but it also allows us to reconstruct the joint anatomically. These factors are a major concern, especially in a young and active population.We are describing indications, surgical techniques, pearls and pitfalls, and outcomes of proximal humeral osteoarticular allografts, done at present time in our institution.We found that allograft fractures and articular complications, as epiphyseal resorption and subchondral fracture, are the main complications observed in proximal humerus osteoarticular allograft reconstructions. Nevertheless, only fractures need a reconstruction revision. Joint complications may adversely affect the limb function, but for this reason, an allograft revision is rarely performed.

  10. Familial vitamin D resistant rickets: End-organ resistance to 1,25-dihydroxyvitamin D.

    PubMed

    Choudhury, Sangita; Jebasingh, K Felix; Ranabir, Salam; Singh, Th Premchand

    2013-10-01

    Rickets is softening of bones due to defective mineralization of cartilage in the epiphyseal growth plate, leading to widening of ends of long bones, growth retardation, and skeletal deformities in children. The predominant cause is deficiency or impaired metabolism of vitamin D. The observation that some forms of rickets could not be cured by regular doses of vitamin D, led to the discovery of rare inherited abnormalities of vitamin D metabolism or vitamin D receptor. Vitamin D dependent rickets (VDDR) is of two types: Type I is due to defective renal tubular 25-hydroxyvitamin D 1-α hydroxylase and type II is due to end-organ resistance to active metabolite of vitamin D. Typical signs are observed from the first month of life. The patient with rickets described below had markedly increased serum alkaline phosphatase and 1,25-dihydroxyvitamin D. We attribute these abnormalities to impaired end-organ responsiveness to 1,25-dihydroxyvitamin D.

  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. Base of coracoid process fracture with acromioclavicular dislocation in a child

    PubMed Central

    2010-01-01

    Fracture of the coracoid process is a rare injury. It can be easily missed when associated with other injuries to the shoulder girdle, for instance, acromioclavicular joint (ACJ) dislocation. Clinical attention is easily drawn to the more obvious ACJ dislocation, hence, the need for further radiological evaluation. We report an unusual case of fracture of the base of coracoid process associated with a true acromioclavicular joint dislocation in a 12 year old boy, with no separation of the epiphyseal plate, as one might expect. Treatment also remains controversial. Our patient underwent open reduction internal fixation of the acromioclavicular joint and coracoid process. He subsequently made an uneventful progress with pain free full range of shoulder movement at 5 months, and was discharged at 9 months. PMID:20955595

  13. [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.

  14. 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

  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. Osteointegration of bioactive glass-coated zirconia in healthy bone: an in vivo evaluation.

    PubMed

    Stanic, V; Aldini, N Nicoli; Fini, M; Giavaresi, G; Giardino, R; Krajewski, A; Ravaglioli, A; Mazzocchi, M; Dubini, B; Bossi, M G Ponzi; Rustichelli, F

    2002-09-01

    Osteointegration of yttria stabilised tetragonal zirconia (YSTZ), either coated with bioactive glass named RKKP bioglaze (RKKP) or uncoated, was evaluated in an animal model. RKKP-coated and uncoated (controls) YSTZ cylinders were implanted in the distal femoral epiphyses of 14 Sprague Dawley rats under general anaesthesia. At the experimental times of 30 and 60 days after sacrifice, histomorphometry and SEM microanalysis were performed on methylmethacrylate-embedded undecalcified sections to determine the osteointegration rate. At 30 days, a significantly higher affinity index was demonstrated in vivo by histomorphometric evaluation in RKKP-coated versus uncoated YSTZ implants p < 0.05); at 60 days, the coated implants behaved better than controls (affinity index of + 32%), but the difference observed lay within the statistical uncertainty. SEM analysis demonstrated better bone adhesion to the material in RKKP-coated YSTZ at both 30 and 60 days. These findings suggest that YSTZ coated with the bioactive glass named RKKP enhances osteointegration of ceramics.

  17. 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.

  18. 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

  19. 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

  20. 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.

  1. 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.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. Giant intraosseous cyst-like lesions in rheumatoid arthritis report of a case.

    PubMed

    Lohse, Anne; Carbillet, Jean-Pierre; Onimus, Michel; Stevenel, Françoise; Toussirot, Eric; Wendling, Daniel

    2003-02-01

    The term "intraosseous synovial cyst" is used to designate both the epiphyseal cyst-like lesions seen in patients with rheumatoid arthritis (RA) and mucoid cysts, which occur in a different setting. We report the case of a patient in whom a 4-cm cyst-like lesion developed in the left tibia 18 years after onset of RA and 6 years after osmic acid synovectomy of the left knee. Positive contrast arthrography and magnetic resonance imaging visualized a communication between the lesion and the joint space. Preexisting bone and joint lesions and increased intraarticular pressure play a major role in the genesis of cyst-like lesions in RA. In our patient, the osmic acid synovectomy may have contributed to the development of the lesion. "Synovial cyst" is a misnomer for these giant lesions, which are geodes rather than cysts. Despite their low incidence, these lesions deserve attention because they raise diagnostic and therapeutic problems.

  8. 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.

  9. [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.

  10. 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].

  11. 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

  12. [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.

  13. Isolated Adult Tillaux Fracture Associated With Volkmann Fracture-A Unique Combination of Injuries: Report of Two Cases and Review of the Literature.

    PubMed

    Kose, Ozkan; Yuksel, Halil Yalcin; Guler, Ferhat; Ege, Tolga

    2016-01-01

    Avulsion fractures of the anterior inferior tibiofibular ligament from its tibial attachment, Tillaux fractures, are usually seen in adolescents during the interval of the distal tibial epiphyseal closure. However, this pattern of fracture is rare in adult patients, because the ligaments will usually fail before the bone fails. Avulsion fracture of the posterior inferior tibiofibular ligament from its tibial attachment, Volkmann fracture, is the posterolateral counterpart of a similar injury. In the present study, the cases of 2 adult patients with simultaneous Tillaux and Volkmann fractures are reported and the mechanism of injury, diagnosis, and treatment discussed. This fracture pattern is extremely rare and, to the best of our knowledge, has not been previously reported. Copyright © 2015 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

  14. 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

  15. 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.

  16. 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.

  17. Fracture and dislocation classification compendium for children: the AO pediatric comprehensive classification of long bone fractures (PCCF).

    PubMed

    Slongo, Theddy F; Audigé, Laurent

    2007-01-01

    The AO Pediatric Expert Group and the AO Pediatric Classification Group, in cooperation with the AO Investigation and Documentation Group introduce and present the first comprehensive classification of pediatric long bone fractures. The anatomy is related to the 4 long bones and their 3 segments defined as proximal (1), shaft (2) and distal (3). It is further described by the fracture subsegment recorded as epiphyseal (E), metaphyseal (M) and diaphyseal (D), whereby proximal and distal fractures are classified as E or M and shaft fractures are always D. The distinction between metaphyseal and diaphyseal fractures is achieved by localizing the center of fracture lines with regard to a square drawn over the respective growth plates. The morphology of the fracture is documented by a subsegment-specific child pattern code, a severity code as well as an additional code for displacement of specific fractures such as supracondylar fractures and radial heads. The classification process requires trained observers to read standard radiographic images.

  18. 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.

  19. Osteopenia and osteoporosis in children.

    PubMed

    Cassidy, J T

    1999-01-01

    The purpose of this paper is to review the normal physiologic processes of skeletal accretion, abnormalities that may occur in children with chronic illnesses, and therapeutic maneuvers that the clinician may be able to employ to prevent or partially correct abnormalities of skeletal growth. Skeletal maturation in children is dependent upon bone formation exceeding resorption, whereas in adults these two fundamental processes of homeostasis are closely balanced. Skeletal growth is effectively completed at the end of the period of adolescent growth acceleration with closure of the epiphyses. An important determinant of future fracture risk and osteoporosis is the peak bone mass achieved during this second decade of life. If the hereditarily determined peak bone mass is not established during that time, the patient will enter young adulthood with osteopenia, an increased fracture risk, and accelerated postmenopausal osteoporosis or involutional osteoporosis. Thus osteopenia and osteoporosis have their origins in childhood and adolescence.

  20. 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.

  1. 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.

  2. Calcium accumulation by chondrocyte mitochondria.

    PubMed

    Shapiro, I M; Lee, N H

    1975-01-01

    Chick epiphyseal plate mitochondria observed in vitro suggest that energy dependent Ca++ uptake was maximal in the presence of ATP and a respiratory substrate. However, nucleotides other than ATP had no effect on this type of cation uptake. The observation that Ca++ accumulation was sensitive to the presence of 2,4-DNP and a number of respiratory inhibitors suggested that the mechanism of cation accumulation was similar to that described in tissues that do not undergo biological mineralization. Non-energy supported Ca++ acumulation was studied in the presence of rotenone and antimycin A. Under these conditions, the amount of Ca++ bound by skeletal tissue mitochondria was greater than bound by mitochondria obtained from noncalcifying tissues. Following isopycnic centrifugation, the Ca++ loaded mitochondria banded at different sucrose densties but the Ca++ affinity of mitochondria at each density band was similar. Hence, no particular mitochondrial species seems to be responsible for cation transport.

  3. 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

  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. 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.

  6. 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.

  7. 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

  8. 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

  9. 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

  10. 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.].

  11. 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.

  12. [Osteochondroma of the clavicle and pain syndrome of the shoulder. Apropos of a case. Review of the literature].

    PubMed

    Vander Maren, C; Guillaumie, B; Huge, J; Bodart, A; Van Ruyssevelt, C

    1994-01-01

    Osteochondroma, a frequent benign tumor of the bone, is in most cases asymptomatic. Localization in the clavicle is extremely rare. The authors report an isolated case occurring in the coraco-clavicular area responsible for a painful shoulder syndrome evoking rotator cuff tendinitis. A 47 years female patient consulted for painful shoulder syndrome. Pain occurred following an effort and at night. On clinical examination, the area around the coracoid process was tender as was the supraspinatus fossa. Passive anterior elevation of the shoulder was limited to 150 degrees. She had a positive Job's sign as well as a painful < Gross armtest >. Initial Radiological assessment showed no anomalies. Arthrographic CT scan revealed an expansive process compatible with osteochondroma. The lesion was in contact with the supraspinatus muscles and the coracoid process. Extraperiosteal resection was performed through a delto-pectoral approach. Recovery of a painless mobile joint was rapid. Clavicular embryology is not yet well understood. The appearance of an exostosis at this localization seems to confirm that the clavicular cartilage behaves as an epiphyseal bone plate. The exostosis, which has a congenital origin, can appear, when it reaches a certain size or when it mechanically interferes with surrounding muscles and tendons. In our observation, the lesion led to irritation of the supraspinatus muscle leading to tendinitis. Diagnosis is usually made on standard roentgenographic evaluation. CT scan and MRI show a cartilaginous coating which should not exceed 10 mm in the case of a benign tumor. There exists a possibility of sarcomatous degeneration, but this is rare on the peripheral skeleton. Treatment should be performed by complete extraperiosteal resection of the exostosis along with its perichondral cover. Clavicular localization of an osteochondroma is very rare. This case report illustrates the possibility of a painful shoulder syndrome associated with this lesion. This

  13. 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.

  14. 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.

  15. Hand fractures in children: epidemiology and misdiagnosis in a tertiary referral hospital.

    PubMed

    Chew, Ee Ming; Chong, Alphonsus K S

    2012-08-01

    To determine the local epidemiology of pediatric hand fractures and the rate of misdiagnosis. A retrospective study was performed on children aged 17 years and younger who were referred for actual or suspected metacarpal and phalangeal fractures. Medical records were reviewed for age at the time of injury, sex, fracture pattern, venue where the injury was sustained, injury mechanism, and diagnoses made by the referring doctor and hand surgeon. Differing diagnoses were considered misdiagnoses. The misdiagnosis rate was calculated as the percentage of misdiagnoses over the number of referrals. Of 204 cases reviewed, emergency physicians referred 146 cases (72%), and primary health care physicians referred the rest. There were 193 cases of actual fractures in 181 patients and 16 cases of misdiagnosis. The fracture incidence peaked at 14 and 15 years. The median ages of children sustaining fractures of the distal phalanges, proximal phalanges, and metacarpals were 9, 12, and 15 years, respectively. The proximal phalanx was most commonly fractured (95 cases, 49%), as was the fifth ray (78 cases, 40%). Most fractures occurred at school (79 cases, 44%). Sports-related injury was the leading cause of fractures (70 cases, 39%). The misdiagnosis rate was 8% (16 of 204). The leading cause of misdiagnosis was misinterpretation of epiphyses (6 of 16), followed by missing multiple fractures (3 of 16). The higher fracture incidence in teenagers is likely related to sports participation. Sports accounted for proximal fractures in older children, whereas young children sustained distal fractures through crushing injuries. Although the misdiagnosis rate seemed low, it might reflect that emergency physicians, who referred most of the cases, were adept at diagnosing fractures. To improve diagnostic accuracy, doctors should familiarize themselves with the location of epiphyses and look carefully for multiple fractures. Economic and Decision Analysis IV. Copyright © 2012 American

  16. Brachytelephalangic chondrodysplasia punctata: a case series to further delineate the phenotype.

    PubMed

    Gupta, Neerja; Ghosh, Manju; Shukla, Rashmi; Das, Ganesh Prasad; Kabra, Madhulika

    2012-07-01

    Chondrodysplasia punctata (CDP) is a heterogenous group of skeletal dysplasias characterized by aberrant bone mineralization, manifesting radiologically as epiphyseal stippling. Among this group, brachytelephalangic dysplasia, a benign form of CDP (CDPX1), is probably under-reported. It is an X-linked recessive disorder and is characterized by a flat nasal tip, short columella and maxillary hypoplasia, involvement of terminal phalanges, and stippled chondrodystrophy. This paper presents a clinical series of 13 patients with brachytelephalangic dysplasia. These patients enrolled during 2002-2006 were re-evaluated and their dysmorphic features were compiled in a predesigned proforma. Skeletal survey, karyotype, cardiac evaluation, and ophthalmic evaluation were planned for all the cases. Out of 13 patients, 10 were males and three were females. All patients had flat facies, a depressed nasal bridge, a hypoplastic nose, a short philtrum, notched alae nasi, brachydactyly, and hypoplastic terminal phalanges. In addition, congenital heart disease, optic nerve hypoplasia, and developmental delay were found in a few patients. Radiography showed hypoplastic terminal phalanges, delayed bone age (1/13), epiphyseal stippling in carpal (3/13) and tarsal bones (2/13), sacral bone (1/13), and bullet-shaped lumbar vertebra (1/13). Cranial neuroimaging, thyroid profile, and karyotype carried out in a few were normal. The present paper discusses various clinical features and associated abnormalities in patients with brachytelephalangic dysplasia (CDPX1) to further delineate the phenotype. The presence of a similar phenotype in females suggests the possibility of another locus or manifestation of disease in heterozygous females. Arylsulfatase E gene analysis would further help in establishing the genotype-phenotype correlation.

  17. 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

  18. Suppressed osteoclast differentiation at the chondro-osseous junction mediates endochondral ossification retardation in long bones of Wistar fetal rats with prenatal ethanol exposure.

    PubMed

    Pan, Zhengqi; Zhang, Xianrong; Shangguan, Yangfan; Hu, Hang; Chen, Liaobin; Wang, Hui

    2016-08-15

    Prenatal ethanol exposure (PEE) inhibits longitudinal growth of fetal bones, but the underlying mechanisms remain unknown. In this study, we aimed to investigate how PEE induces the retardation of long bone development in fetal rats. Pregnant Wistar rats were treated with ethanol or distilled water (control group) by gavage from gestational day (GD) 9 to 20. Fetuses were delivered by cesarean section on GD20. Fetal sera were collected for assessing corticosterone (CORT) level. Fetal long bones were harvested for histochemical, immunohistochemical and gene expression analysis. Primary chondrocytes were treated with ethanol or CORT for analyzing genes expression. PEE fetuses showed a significant reduction in birth weight and body length. The serum CORT concentration in PEE group was significantly increased, while the body weight, body length and femur length all were significantly decreased in the PEE group. The length of the epiphyseal hypertrophy zone was enlarged, whereas the length of the primary ossification center was significantly reduced in PEE fetuses. TUNEL assay showed reduced apoptosis in the PEE group. Further, the gene expression of osteoprotegerin (OPG) was markedly up-regulated. In vitro experiments showed that CORT (but not ethanol) treatment significantly activated the expression of OPG, while the application of glucocorticoid receptor inhibitor, mifepristone, attenuated these change induced by CORT. These results indicated that PEE-induced glucocorticoid over-exposure enhanced the expression of OPG in fetal epiphyseal cartilage and further lead to the suppressed osteoclast differentiation in the chondro-osseous junction and consequently inhibited the endochondral ossification in long bones of fetal rats. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. Transcription Factor Erg Variants and Functional Diversification of Chondrocytes during Limb Long Bone Development

    PubMed Central

    Iwamoto, Masahiro; Higuchi, Yoshinobu; Koyama, Eiki; Enomoto-Iwamoto, Motomi; Kurisu, Kojiro; Yeh, Helena; Abrams, William R.; Rosenbloom, Joel; Pacifici, Maurizio

    2000-01-01

    During limb development, chondrocytes located at the epiphyseal tip of long bone models give rise to articular tissue, whereas the more numerous chondrocytes in the shaft undergo maturation, hypertrophy, and mineralization and are replaced by bone cells. It is not understood how chondrocytes follow these alternative pathways to distinct fates and functions. In this study we describe the cloning of C-1-1, a novel variant of the ets transcription factor ch-ERG. C-1-1 lacks a short 27–amino acid segment located ∼80 amino acids upstream of the ets DNA binding domain. We found that in chick embryo long bone anlagen, C-1-1 expression characterizes developing articular chondrocytes, whereas ch-ERG expression is particularly prominent in prehypertrophic chondrocytes in the growth plate. To analyze the function of C-1-1 and ch-ERG, viral vectors were used to constitutively express each factor in developing chick leg buds and cultured chondrocytes. We found that virally driven expression of C-1-1 maintained chondrocytes in a stable and immature phenotype, blocked their maturation into hypertrophic cells, and prevented the replacement of cartilage with bone. It also induced synthesis of tenascin-C, an extracellular matrix protein that is a unique product of developing articular chondrocytes. In contrast, virally driven expression of ch-ERG significantly stimulated chondrocyte maturation in culture, as indicated by increases in alkaline phosphatase activity and deposition of a mineralized matrix; however, it had modest effects in vivo. The data show that C-1-1 and ch-ERG have diverse biological properties and distinct expression patterns during skeletogenesis, and are part of molecular mechanisms by which limb chondrocytes follow alternative developmental pathways. C-1-1 is the first transcription factor identified to date that appears to be instrumental in the genesis and function of epiphyseal articular chondrocytes. PMID:10893254

  20. 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.

  1. 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.

  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. 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

  5. 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

  6. 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.

  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. Wolcott-Rallison syndrome with 3-hydroxydicarboxylic aciduria and lethal outcome.

    PubMed

    Søvik, O; Njølstad, P R; Jellum, E; Molven, A

    2008-12-01

    Wolcott-Rallison syndrome (WRS) (OMIM 226980) is a rare, autosomal recessive disorder with infancy-onset diabetes mellitus, multiple epiphyseal dysplasia, osteopenia, mental retardation or developmental delay, and hepatic and renal dysfunction as main clinical findings. Patients with WRS have mutations in the EIF2AK3 gene, which encodes the pancreatic eukaryotic translation initiation factor 2-alpha kinase 3. We report a female patient who developed insulin-requiring diabetes at 2.5 months of age. Multiple epiphyseal dysplasia was diagnosed at age 2 years. At age 5.5 years she developed a Reye-like syndrome with hypoketotic hypoglycaemia and renal and hepatic insufficiency and died. A partial autopsy showed fat infiltration in the liver and kidneys. Examination of urine by gas chromatography and mass spectrometry showed large amounts of C(6)-dicarboxylic acid (adipic acid), 3-hydroxy-C(8)-dicarboxylic acid, 3-hydroxy-C(10)-dicarboxylic acid, and 3-hydroxydecenedioic acid. Acetoacetate and 3-hydroxybutyrate were absent. The findings suggested a metabolic block in mitochondrial fatty acid oxidation, but lack of material precluded enzyme analyses. The clinical diagnosis of WRS was suggested in retrospect, and confirmed by sequencing of DNA extracted from stored autopsy material. The patient was compound heterozygous for the novel EIF2AK3 mutations c.1694_1695delAT (Y565X) and c.3044T > C (F1015S). Our data suggest that disruption of the EIF2AK3 gene may lead to defective mitochondrial fatty acid oxidation and hypoglycaemia, thus adding to the heterogeneous phenotype of WRS.

  9. 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

  10. Accuracy of Various MRI Sequences in Determining the Tumour Margin in Musculoskeletal Tumours.

    PubMed

    Putta, Tharani; Gibikote, Sridhar; Madhuri, Vrisha; Walter, Noel

    2016-01-01

    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. 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. 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. 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.

  11. 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

  12. Automatic segmentation of bones from digital hand radiographs

    NASA Astrophysics Data System (ADS)

    Liu, Brent J.; Taira, Ricky K.; Shim, Hyeonjoon; Keaton, Patricia

    1995-05-01

    The purpose of this paper is to develop a robust and accurate method that automatically segments phalangeal and epiphyseal bones from digital pediatric hand radiographs exhibiting various stages of growth. The algorithm uses an object-oriented approach comprising several stages beginning with the most general objects to be segmented, such as the outline of the hand from background, and proceeding in a succession of stages to the most specific object, such as a specific phalangeal bone from a digit of the hand. Each stage carries custom operators unique to the needs of that specific stage which will aid in more accurate results. The method is further aided by a knowledge base where all model contours and other information such as age, race, and sex, are stored. Shape models, 1-D wrist profiles, as well as an interpretation tree are used to map model and data contour segments. Shape analysis is performed using an arc-length orientation transform. The method is tested on close to 340 phalangeal and epiphyseal objects to be segmented from 17 cases of pediatric hand images obtained from our clinical PACS. Patient age ranges from 2 - 16 years. A pediatric radiologist preliminarily assessed the results of the object contours and were found to be accurate to within 95% for cases with non-fused bones and to within 85% for cases with fused bones. With accurate and robust results, the method can be applied toward areas such as the determination of bone age, the development of a normal hand atlas, and the characterization of many congenital and acquired growth diseases. Furthermore, this method's architecture can be applied to other image segmentation problems.

  13. 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

  14. 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.

  15. 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

  16. Pathogenesis of lumbar spine disease in mucopolysaccharidosis VII.

    PubMed

    Smith, Lachlan J; Baldo, Guilherme; Wu, Susan; Liu, Yuli; Whyte, Michael P; Giugliani, Roberto; Elliott, Dawn M; Haskins, Mark E; Ponder, Katherine P

    2012-09-01

    Mucopolysaccharidosis type VII (MPS VII) is characterized by deficient β-glucuronidase (GUSB) activity, which leads to accumulation of chondroitin, heparan and dermatan sulfate glycosaminoglycans (GAGs), and multisystemic disease. MPS VII patients can develop kypho-scoliotic deformity and spinal cord compression due to disease of intervertebral disks, vertebral bodies, and associated tissues. We have previously demonstrated in MPS VII dogs that intervertebral disks degenerate, vertebral bodies have irregular surfaces, and vertebral body epiphyses have reduced calcification, but the pathophysiological mechanisms underlying these changes are unclear. We hypothesized that some of these manifestations could be due to upregulation of destructive proteases, possibly via the binding of GAGs to Toll-like receptor 4 (TLR4), as has been proposed for other tissues in MPS models. In this study, the annulus fibrosus of the intervertebral disk of 6-month-old MPS VII dogs had cathepsin B and K activities that were 117- and 2-fold normal, respectively, which were associated with elevations in mRNA levels for these cathepsins as well as TLR4. The epiphyses of MPS VII dogs had a marked elevation in mRNA for the cartilage-associated gene collagen II, consistent with a developmental delay in the conversion of the cartilage to bone in this region. The spine obtained at autopsy from a young man with MPS VII exhibited similar increased cartilage in the vertebral bodies adjacent to the end plates, disorganization of the intervertebral disks, and irregular vertebral end plate morphology. These data suggest that the pathogenesis of destructive changes in the spine in MPS VII may involve upregulation of cathepsins. Inhibition of destructive proteases, such as cathepsins, might reduce spine disease in patients with MPS VII or related disorders. Copyright © 2012 Elsevier Inc. All rights reserved.

  17. Trafficking defects and loss of ligand binding are the underlying causes of all reported DDR2 missense mutations found in SMED-SL patients

    PubMed Central

    Ali, Bassam R.; Xu, Huifang; Akawi, Nadia A.; John, Anne; Karuvantevida, Noushad S.; Langer, Ruth; Al-Gazali, Lihadh; Leitinger, Birgit

    2010-01-01

    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. PMID:20223752

  18. Characterization of Normal Murine Carpal Bone Development Prompts Re-Evaluation of Pathologic Osteolysis as the Cause of Human Carpal-Tarsal Osteolysis Disorders.

    PubMed

    Lazarus, Syndia; Tseng, Hsu-Wen; Lawrence, Felicity; Woodruff, Maria A; Duncan, Emma L; Pettit, Allison R

    2017-09-01

    Multicentric carpal-tarsal osteolysis; multicentric osteolysis, nodulosis, and arthropathy; and Winchester syndromes, skeletal dysplasias characterized by carpal/tarsal and epiphyseal abnormalities, are caused by mutations in v-maf musculoaponeurotic fibrosarcoma oncogene ortholog B (MAFB), matrix metalloproteinase (MMP) 2, and MMP14, respectively; however, the underlying pathophysiology is unclear. Osteoclast-mediated osteolysis has been regarded as the main mechanism, but does not explain the skeletal distribution. We hypothesized that MAFB, MMP-2, and MMP-14 have integral roles in carpal/tarsal and epiphyseal bone development. Normal neonatal mouse forepaws were imaged by micro-computed tomography and examined histologically. Murine forepaw ossification occurred sequentially. Subarticular regions of endochondral ossification showed morphologic and calcification patterns that were distinct from archetypical physeal endochondral ossification. This suggests that two different forms of endochondral ossification occur. The skeletal sites showing the greatest abnormality in the carpal-tarsal osteolysis syndromes are regions of subarticular ossification. Thus, abnormal bone formation in areas of subarticular ossification may explain the site-specific distribution of the carpal-tarsal osteolysis phenotype. MafB, Mmp-2, and Mmp-14 were expressed widely, and tartrate-resistant acid phosphatase staining notably was absent in the subarticular regions of the cartilage anlagen and entheses at a time point most relevant to the human osteolysis syndromes. Thus, abnormal peri-articular skeletal development and modeling, rather than excessive bone resorption, may be the underlying pathophysiology of these skeletal syndromes. Copyright © 2017 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  19. 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.

  20. 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.

  1. The AO comprehensive classification of pediatric long-bone fractures: a web-based multicenter agreement study.

    PubMed

    Slongo, Theddy; Audigé, Laurent; Clavert, Jean-Michel; Lutz, Nicolas; Frick, Steve; Hunter, James

    2007-03-01

    The first AO comprehensive pediatric long-bone fracture classification system has been proposed following a structured path of development and validation with experienced pediatric surgeons. A Web-based multicenter agreement study involving 70 surgeons in 15 clinics and 5 countries was conducted to assess the reliability and accuracy of this classification when used by a wide range of surgeons with various levels of experience. Training was provided at each clinic before the session. Using the Internet, participants could log in at any time and classify 275 supracondylar, radius, and tibia fractures at their own pace. The fracture diagnosis was made following the hierarchy of the classification system using both clinical terminology and codes. kappa coefficients for the single-surgeon diagnosis of epiphyseal, metaphyseal, or diaphyseal fracture type were 0.66, 0.80, and 0.91, respectively. Median accuracy estimates for each bone and type were all greater than 80%. Depending on their experience and specialization, surgeons greatly varied in their ability to classify fractures. Pediatric training and at least 2 years of experience were associated with significant improvement in reliability and accuracy. Kappa coefficients for diagnosis of specific child patterns were 0.51, 0.63, and 0.48 for epiphyseal, metaphyseal, and diaphyseal fractures, respectively. Identified reasons for coding discrepancies were related to different understandings of terminology and definitions, as well as poor quality radiographic images. Results supported some minor adjustments in the coding of fracture type and child patterns. This classification system received wide acceptance and support among the surgeons involved. As long as appropriate training could be performed, the system classification was reliable, especially among surgeons with a minimum of 2 years of clinical experience. We encourage broad-based consultation between surgeons' international societies and the use of this

  2. Incubation lighting schedules and their interaction with matched or mismatched post hatch lighting schedules: Effects on broiler bone development and leg health at slaughter age.

    PubMed

    van der Pol, Carla W; van Roovert-Reijrink, Inge A M; Aalbers, Gerald; Kemp, Bas; van den Brand, Henry

    2017-07-17

    The incidence of leg pathologies in broiler chickens with a developmental origin may be decreased by stimulating embryonic bone development through lighting schedules during incubation, but this may depend on post hatch lighting conditions. Aim was to investigate how lighting schedules during incubation and their interactions with matched or mismatched lighting schedules post hatch affected bone development and leg health at slaughter age. In a 3×2 factorial designed experiment, eggs were incubated under continuous cool white LED light (Inc24L), 16h of light, 8h of darkness (Inc16L:8D), or continuous darkness (Inc24D) from set till hatch. After hatch, broilers were housed under continuous light (PH24L, to match Inc24L and Inc24D) or 16h of light, 8h of darkness (PH16L:8D, to match Inc16L:8D). Gait scores were determined on D21, D28, and D34. After slaughter on D35, legs were scored for varus-valgus deformities, rotated tibia, tibial dyschondroplasia, bacterial chondronecrosis with osteomyelitis (BCO), epiphyseolysis, and epiphyseal plate abnormalities from 1=absent to 4=severe. Femur and tibia dimensions and mineral density were determined. Inc24L led to more epiphyseal plate abnormalities than Inc16L:8D or Inc24D. Inc24D led to more BCO than Inc16L:8D. Gait scores on D21, D28, and D34, and bone dimensions did not differ between treatments. Inc24L led to higher femur mineral density than Inc24D with Inc16L:8D intermediate. Providing a chicken with a matched post hatch lighting schedule did not affect most measurements of bone development and health. It can be concluded that a circadian incubation lighting schedule may improve leg health in broilers. Copyright © 2017 Elsevier Ltd. All rights reserved.

  3. 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

  4. 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

  5. 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.

  6. 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.

  7. 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.

  8. Immunohistochemical localization of fibroblast growth factor-2 in normal and brachymorphic mouse tibial growth plate and articular cartilage.

    PubMed

    Wezeman, F H; Bollnow, M R

    1997-06-01

    Epiphyses of the proximal tibiae of 7-week-old normal and homozygous recessive brachymorphic mice (bm/bm) were immunostained using a monoclonal antibody to basic fibroblast growth factor to determine its expression in growth plate cartilage, osteoblasts on the surfaces of the primary spongiosa and articular cartilage. In the normal growth plate the immunoreactive factor was present in chondrocytes of the proliferating and upper hypertrophic zones but absent from lower hypertrophic chondrocytes. Immunostaining was present only in the territorial extracellular matrix immediately adjacent to the chondrocytes of the proliferating and upper hypertrophic zones. Osteoblasts of the primary spongiosa stained heavily in normal mice. Strong staining was observed in intermediate zone articular chondrocytes. Cells in the superficial layer of articular cartilage were unstained. The extracellular matrix of the articular cartilage was completely free of immunostaining. In contrast, the reduced size of bm/bm growth plates was accompanied by significantly reduced staining intensity in proliferating and upper hypertrophic chondrocytes, and staining was absent from the territorial extracellular matrix of all zones of the bm/bm growth plate. Osteoblasts of the primary spongiosa of bm/bm mice stained less than those of normal mice. Articular cartilage chondrocytes in the intermediate zone stained with less intensity in bm/bm mice, and the cells of the superficial layer were unstained. The extracellular matrix of bm/bm articular cartilage was completely free of staining. Brachymorphic epiphyseal growth plate and articular chondrocytes, and osteoblasts in the primary spongiosa, express reduced amounts of immunoreactive fibroblast growth factor-2. This phenotypical characteristic may be associated with abnormal endochondral ossification and development of bone in brachymorphic mice.

  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. Preventive role of zinc chloride against toxicity of ciprofloxacin on the growing cartilage of Wistar albino rat litter.

    PubMed

    Channa, Haji Muhammad Aslam; Ashfaq, Muhammad; Bangash, Rahim; Abbasi, Abdullah; Qureshi, Muhammad Azam

    2008-01-01

    To assess the preventive role of zinc chloride on toxicity of ciprofloxacin administration in Wistar albino rat litter. It was a Prospective experimental study. The study was carried out in the Department of Anatomy, Basic Medical Sciences Institute, Jinnah Postgraduate Medical Centre, Karachi, Pakistan during March 2002 to February 2003 one year study. Ciprofloxacin and zinc chloride were administered to newly born albino rat litters separately and simultaneously at a dose of 20 mg/kg body weight and 1200 microg/Kg body weight respectively, intraperitonealy twice daily from 1 - 14 day after birth. The animals were sacrificed by deep ether anaesthesia. The fore and hind limbs were dis-articulated from the axial skeleton, soft tissue was removed and bones were fixed in 10% buffered formalin. Decalcification was done in 10% nitric acid and 10% formic acid changes. After paraplast embedding, 4 microm thick longitudinal sections of proximal & distal ends of long bones were cut by a rotary microtome. Routine staining with haemotoxylin and eosin was performed. Histomorphometery was done to measure the thickness of epiphyseal cartilage and was compared with similar values of the control animals. The results were statistically analyzed to evaluate the significance. Our study revealed that ciprofloxacin administration in new born albino rat litter decreased the width of epiphyseal growth plate cartilage by 13.7 +/- 0.42 microm, 10.43% in humerus and 6.6 +/- 1.2 microm 4.72% in femur as compared to control, whereas, simultaneous zinc chloride administration restricted the decrease to 1.27 microm +/- SD in humerus and 2.05 microm +/- SD in femur. Simultanous zinc chloride administration minimized the epiphseal cartilage damage induced by ciprofloxacin in Wistar albino rat litter.

  11. 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.

  12. Novel Application of Magnetic Resonance Imaging Demonstrates Characteristic Differences in Vasculature at Predilection Sites of Osteochondritis Dissecans

    PubMed Central

    Tóth, Ferenc; Nissi, Mikko J.; Ellermann, Jutta M.; Wang, Luning; Shea, Kevin G.; Polousky, John; Carlson, Cathy S.

    2016-01-01

    Background Understanding the pathogenesis of osteochondrosis/osteochondritis dissecans and other developmental orthopaedic diseases that are thought to occur secondary to defects in vascular supply to growth/epiphyseal cartilage has been hampered by the inability to image the vasculature in this tissue. This is particularly true in human beings due to limitations of current imaging techniques and the lack of availability of appropriate cadaveric samples for histological studies. Hypothesis Susceptibility weighted imaging, an MRI sequence, allows identification of characteristic differences in the vascular architecture in species that are affected by osteochondrosis/osteochondritis dissecans on the femoral condyle (humans and pigs) versus one that is free of the disease (goat). Study design Descriptive laboratory study Materials Distal femora from cadavers of juvenile humans (n=5), pigs (n=3), and goats (n=3) were scanned in a 9.4T MRI scanner using susceptibility weighted imaging. Three-dimensional reconstructions were created and minimum intensity projections were calculated in three planes to enhance visualization of the vascular architecture. Results Susceptibility weighted imaging allowed clear visualization of the epiphyseal vasculature in all species. Vascular architecture, with vessels primarily arising from the perichondrium, was similar in humans and pigs, which are predisposed to osteochondrosis/osteochondritis dissecans, and was starkly different from that present in goats, a species in which there are no reports of osteochondrosis/osteochondritis dissecans. Furthermore, vessels in the distal femoral predilection site disappeared with age in a similar pattern in humans as has been reported previously in pigs. Conclusion Nearly identical vascular architecture at the shared primary predilection site of osteochondrosis/osteochondritis dissecans in the femoral condyles in human beings and pigs suggests that vascular failure, which is known to be central to

  13. Salter-Harris Type III and IV medial malleolar fractures: growth arrest: is it a fate? A retrospective study of 48 cases with open reduction.

    PubMed

    Cottalorda, Jérôme; Béranger, Vincent; Louahem, Djamel; Camilleri, Jean Philippe; Launay, Franck; Diméglio, Alain; Bourelle, Sophie; Jouve, Jean-Luc; Bollini, Gérard

    2008-09-01

    Salter-Harris type III and IV medial malleolar fractures (MacFarland fracture) is a joint fracture of the ankle in children. The fracture line passes through the medial part of the lower epiphyseal disk of the tibia. Prognosis is dominated by later risk of misalignment and osteoarthritis. The aim of this study was to evaluate the functional and radiological outcome of these fractures. We retrospectively analyzed the cases of 48 children with MacFarland fractures (31 boys and 17 girls), mean age at the time of trauma 11 years 6 months (range, 8-15 years). The fractures were classed into two groups according to the Salter and Harris classification for epiphyseal detachment: Salter III (30 cases) and Salter IV (18 cases). Surgical treatment was given in all cases (46 screw fixations, 2 pin fixations). Three outcome categories were used: good (no pain, no stiffness, no limp, no misalignment, no surgical complication, no healing problem), fair (pain and/or stiffness and/or limp and/or healing problem without misalignment, no surgical complication), and poor (misalignment or surgical complication). Mean follow-up was 3 years and 3 months (24-94 months). Twenty-eight children were skeletally mature at the longest follow-up. The three-month postoperative assessment showed 35 patients with good results and 13 children with fair results. Ankle stiffness was noted in 6 cases, ankle pain in 4 cases, wound healing complications in 4 cases, limp in 1 case, and snapping in 1 case. The long-term outcome was considered good for 45 patients, fair for 2 patients (1 wound adherence and 1 hypertrophic scar tissue), and poor for 1 patient (6-degree varus deformity). We did not note leg-length discrepancy or malunion at the longest follow-up. Our results show that growth arrest after MacFarland fracture is no fate. We used surgery more than is generally reported by other teams, opting for surgery as soon as the displacement was >or=1 mm. Surgical treatment was arthrotomy in all cases to

  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. 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

  16. 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

  17. 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

  18. Functional results of displaced proximal humerus fractures in children treated by elastic stable intramedullary nail.

    PubMed

    Khan, A; Athlani, L; Rousset, M; Samba, A; Canavese, F

    2014-02-01

    Displaced proximal humerus fractures within the pediatric population can be treated by elastic stable intramedullary nailing (ESIN). The main objective of our study is to evaluate functional outcome of the displaced proximal humeral fractures treated by ESIN within the pediatric group using a standardized evaluation scale. The secondary goal is to compare functional outcome of epiphyseal and metaphyseal injuries and functional outcome of children younger and older than 10 years of age. From March 2010 to December 2011, 27 children had been treated surgically using ESIN for displaced fractures at the proximal extremity of the humerus. These fractures were displaced and closed without neurovascular insult. Patients were followed radiographically and clinically on a regular basis. After hardware removal, the functional outcomes had been homogeneously assessed by using the French edition of the QuickDash(®) evaluation scale. The descriptive statistics including mean, standard deviation, and confidence interval have been realized. We included 27 children who were operated on consecutively (10 boys, 17 girls). The mean age at the time of operation is 11.2 ± 2.7 years (range 7.1-15.9). The mean angulation is 55.9° ± 20.3° (range 20-90). The mean apposition is 52.8 % ± 33.1 (range 10-100). The mean follow-up period is 15.2 ± 5.6 months (range 6.1-28.5). Results were considered good for children between 7.1 and 15.9 years old with epiphyseal and meataphyseal fractures. The mean QuickDash(®) score is 2.0 (range 0-6.5), with 14 cases showing a score of 0 (58.3 %), 2 cases with 4.3 (8.3 %), 4 cases with 4.5 (16.7 %), and 4 cases with a score of 6.5 (16.7 %). Ranges of movement were preserved. The patients regained their daily and sportive activity over the time without pain or discomfort. Our study showed a good outcome of functional results within a pediatric population who had a sustained displaced proximal humeral fracture and treated by ESIN. Using a standardized

  19. Bone loss from the human distal tibia epiphysis during 24 days of unilateral lower limb suspension

    PubMed Central

    Rittweger, Jörn; Winwood, Keith; Seynnes, Olivier; de Boer, Maarten; Wilks, Desirée; Lea, Rosalind; Rennie, Michael; Narici, Marco

    2006-01-01

    Bone loss during immobilization is well documented. Currently, the only means of studying this in human beings is bed rest, which is resource intensive and inconvenient for the subjects. Unilateral lower limb suspension (ULLS) has been suggested as an alternative, but has not previously been demonstrated to cause bone loss. The main aim of our study was to test the hypothesis that ULLS would cause bone loss determined by peripheral quantitative computed tomography (pQCT). We investigated eight young healthy volunteers (19.1 ± 0.7 years; body mass index, 22.4 ± 2.6 kg m−2), who underwent ULLS for 24 days; their right foot was suspended with a strap attached to the shoulder so the knee angle was 10 deg and they wore a left shoe with a 7.5 cm sole to allow clearance of the right foot and used bilateral crutches to perambulate. Bone scans were obtained by pQCT from the distal epiphyses and from the diaphyses of the tibia in each leg twice before suspension, at days 7, 14 and 21 of the ULLS, and at days 4, 9, 35 and 90 of recovery. After 21 days of ULLS, bone mineral content of the peripheral portion of the epiphysis of the suspended tibia was reduced by 0.89 ± 0.48% (from 280.9 ± 34.5 to 278.4 ± 34.2 mg mm−1, P < 0.001) but no changes were observed in its central portion or in the unsuspended tibia. In the peripheral epiphyseal portion, significant bone loss (by 0.32 ± 0.54%, P = 0.045) occurred as early as day 7 of ULLS. We have demonstrated, for the first time, that in humans bone is lost during ULLS at rates comparable to those seen with bed rest, without alteration in limb fluid volumes thus validating the technique and raising important questions about the mechanisms involved. PMID:17023509

  20. 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.