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Sample records for 3c adolescent idiopathic

  1. Genetics Home Reference: adolescent idiopathic scoliosis

    MedlinePlus

    ... Understand Genetics Home Health Conditions adolescent idiopathic scoliosis adolescent idiopathic scoliosis Enable Javascript to view the expand/ ... boxes. Download PDF Open All Close All Description Adolescent idiopathic scoliosis is an abnormal curvature of the ...

  2. Adolescent idiopathic scoliosis.

    PubMed

    Cheng, Jack C; Castelein, René M; Chu, Winnie C; Danielsson, Aina J; Dobbs, Matthew B; Grivas, Theodoros B; Gurnett, Christina A; Luk, Keith D; Moreau, Alain; Newton, Peter O; Stokes, Ian A; Weinstein, Stuart L; Burwell, R Geoffrey

    2015-01-01

    Adolescent idiopathic scoliosis (AIS) is the most common form of structural spinal deformities that have a radiological lateral Cobb angle - a measure of spinal curvature - of ≥10(°). AIS affects between 1% and 4% of adolescents in the early stages of puberty and is more common in young women than in young men. The condition occurs in otherwise healthy individuals and currently has no recognizable cause. In the past few decades, considerable progress has been made towards understanding the clinical patterns and the three-dimensional pathoanatomy of AIS. Advances in biomechanics and technology and their clinical application, supported by limited evidence-based research, have led to improvements in the safety and outcomes of surgical and non-surgical treatments. However, the definite aetiology and aetiopathogenetic mechanisms that underlie AIS are still unclear. Thus, at present, both the prevention of AIS and the treatment of its direct underlying cause are not possible. PMID:27188385

  3. Adolescent Idiopathic Scoliosis

    PubMed Central

    Choudhry, Muhammad Naghman; Ahmad, Zafar; Verma, Rajat

    2016-01-01

    Background: Scoliosis refers to deviation of spine greater than 10 degrees in the coronal plane. Idiopathic Scoliosis is the most common spinal deformity that develops in otherwise healthy children. The sub types of scoliosis are based on the age of the child at presentation. Adolescent idiopathic scoliosis (AIS) by definition occurs in children over the age of 10 years until skeletal maturity. Objective: The objective of this review is to outline the features of AIS to allow the physician to recognise this condition and commence early treatment, thereby optimizing patient outcome. Method: A thorough literature search was performed using available databases, including Pubmed and Embase, to cover important research published covering AIS. Conclusion: AIS results in higher incidence of back pain and discontent with body image. Curves greater than 50 degrees in thoracic region and greater than 30 degrees in lumbar region progress at a rate of 0.5 to 1 degree per year into adulthood. Curves greater than 60 degrees can lead to pulmonary functional deficit. Therefore once the disease is recognized, effective treatment should be instituted to address the deformity and prevention of its long-term sequelae. PMID:27347243

  4. Nonsurgical Management of Adolescent Idiopathic Scoliosis.

    PubMed

    Gomez, Jaime A; Hresko, M Timothy; Glotzbecker, Michael P

    2016-08-01

    Pediatric patient visits for spinal deformity are common. Most of these visits are for nonsurgical management of scoliosis, with approximately 600,000 visits for adolescent idiopathic scoliosis (AIS) annually. Appropriate management of scoliotic curves that do not meet surgical indication parameters is essential. Renewed enthusiasm for nonsurgical management of AIS (eg, bracing, physical therapy) exists in part because of the results of the Bracing in Adolescent Idiopathic Scoliosis Trial, which is the only randomized controlled trial available on the use of bracing for AIS. Bracing is appropriate for idiopathic curves between 20° and 40°, with successful control of these curves reported in >70% of patients. Patient adherence to the prescribed duration of wear is essential to maximize the effectiveness of the brace. The choice of brace type must be individualized according to the deformity and the patient's personality as well as the practice setting and brace availability. PMID:27388720

  5. Optimal management of idiopathic scoliosis in adolescence

    PubMed Central

    Kotwicki, Tomasz; Chowanska, Joanna; Kinel, Edyta; Czaprowski, Dariusz; Tomaszewski, Marek; Janusz, Piotr

    2013-01-01

    Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%–3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid the need for surgical treatment. Surgery is the treatment of choice for severe idiopathic scoliosis which is rapidly progressive, with early onset, late diagnosis, and neglected or failed conservative treatment. The psychologic impact of idiopathic scoliosis, a chronic disease occurring in the psychologically fragile period of adolescence, is important because of its body distorting character and the onerous treatment required, either conservative or surgical

  6. Optimal management of idiopathic scoliosis in adolescence.

    PubMed

    Kotwicki, Tomasz; Chowanska, Joanna; Kinel, Edyta; Czaprowski, Dariusz; Tomaszewski, Marek; Janusz, Piotr

    2013-01-01

    Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%-3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid the need for surgical treatment. Surgery is the treatment of choice for severe idiopathic scoliosis which is rapidly progressive, with early onset, late diagnosis, and neglected or failed conservative treatment. The psychologic impact of idiopathic scoliosis, a chronic disease occurring in the psychologically fragile period of adolescence, is important because of its body distorting character and the onerous treatment required, either conservative or surgical

  7. [Adolescent idiopathic scoliosis : Guideline for practical application].

    PubMed

    Seifert, J; Thielemann, F; Bernstein, P

    2016-06-01

    Juvenile or adolescent idiopathic scoliosis is a relatively common spinal deformity, with an incidence of more than 1 %. Early diagnosis can lead to successful therapy. In the case of pathological clinical findings, the anteroposterior X‑ray of the whole spine leads the way to the correct grading, according to Cobb angle measurement. Depending on the individual risk of progression, brace treatment will be started with a Cobb angle range of 20-25°. Important predictors of therapeutic success are sufficient primary corrective power and patient compliance. COBB angles of 40-50° usually lead to the recommendation for surgery, which is performed as either anterior or posterior spinal fusion in skeletally mature adolescents, depending on the grade of the deformity according to Lenke's classification. To achieve the best possible results, it is recommended that both conservative and surgical treatments are carried out by scoliosis specialists. PMID:27241514

  8. [Idiopathic headache in childhood and adolescence].

    PubMed

    Karwautz, A; Wöber-Bingöl, C; Wöber, C

    1993-12-01

    This review of the literature covers classification, epidemiology and clinical aspects of idiopathic headache in childhood and adolescence. In addition, pathogenetic models taking into account the complex involvement of organic, psychological and psychosocial factors are critically reviewed. A general pathogenetic model of migraine may be characterized by a given predisposition, various co-factors which enhance the tendency, and finally, trigger mechanisms which induce an attack. No generally accepted model exists for tension-type headache. In assessing the importance of various factors thought to be related to idiopathic headache, it is necessary to differentiate between causal relation, unspecific association, and coincidence. The aim of this review is to present potential factors influencing headache in childhood and adolescence and to discuss these factors critically with regard to their etiopathogenetic importance. Organic factors seem to be most important in migraine, whereas psychological and (psycho)social factors may influence any type of headache. Briefly, migraine in childhood and adolescence seems to be definitively associated with vegetative dysfunction, abdominal symptoms and hormonal factors and possibly with allergic reactions, whereas a relation to epilepsy can be excluded. There is absolutely no evidence for a typical personality of migraine patients. Various psychic reactions, however, are important in all types of headache. Depression and anxiety in young headache patients seem to be related generally to pain, but not specifically to headache. However, school problems, learning disabilities and stress coping behaviour seem to be related directly to recurrent headache. Additionally, there is evidence that the prevalence of headache is associated with low economic status. PMID:8114976

  9. Adolescent idiopathic scoliosis in athletes: is there a connection?

    PubMed

    Kenanidis, Eustathios I; Potoupnis, Michael E; Papavasiliou, Kyriakos A; Sayegh, Fares E; Kapetanos, George A

    2010-06-01

    The potential relationship between adolescent idiopathic scoliosis (AIS) and sports is rather vague. Sports have often been considered to be a causative factor of, or a treatment option for the former, particularly among adolescent athletes who are engaged in certain athletic activities. The highly repetitive nature of sports, amenorrhea, exercise-related exerted stress on the immature spine of professional adolescent athletes, and the joint laxity that may coexist during adolescence, have also been associated with an increased incidence of AIS. The purpose of this article is to discuss the potential connection between sports and AIS by reviewing the existing literature. PMID:20631476

  10. Genetic Risk for Aortic Aneurysm in Adolescent Idiopathic Scoliosis

    PubMed Central

    Haller, Gabe; Alvarado, David M.; Willing, Marcia C.; Braverman, Alan C.; Bridwell, Keith H.; Kelly, Michael; Lenke, Lawrence G.; Luhmann, Scott J.; Gurnett, Christina A.; Dobbs, Matthew B.

    2015-01-01

    Background: Scoliosis is a feature of several genetic disorders that are also associated with aortic aneurysm, including Marfan syndrome, Loeys-Dietz syndrome, and type-IV Ehlers-Danlos syndrome. Life-threatening complications of aortic aneurysm can be decreased through early diagnosis. Genetic screening for mutations in populations at risk, such as patients with adolescent idiopathic scoliosis, may improve recognition of these disorders. Methods: The coding regions of five clinically actionable genes associated with scoliosis (COL3A1, FBN1, TGFBR1, TGFBR2, and SMAD3) and aortic aneurysm were sequenced in 343 adolescent idiopathic scoliosis cases. Gene variants that had minor allele frequencies of <0.0001 or were present in human disease mutation databases were identified. Variants were classified as pathogenic, likely pathogenic, or variants of unknown significance. Results: Pathogenic or likely pathogenic mutations were identified in 0.9% (three) of 343 adolescent idiopathic scoliosis cases. Two patients had pathogenic SMAD3 nonsense mutations consistent with type-III Loeys-Dietz syndrome and one patient had a pathogenic FBN1 mutation with subsequent confirmation of Marfan syndrome. Variants of unknown significance in COL3A1 and FBN1 were identified in 5.0% (seventeen) of 343 adolescent idiopathic scoliosis cases. Six FBN1 variants were previously reported in patients with Marfan syndrome, yet were considered variants of unknown significance based on the level of evidence. Variants of unknown significance occurred most frequently in FBN1 and were associated with greater curve severity, systemic features of Marfan syndrome, and joint hypermobility. Conclusions: Clinically actionable pathogenic mutations in genes associated with adolescent idiopathic scoliosis and aortic aneurysm are rare in patients with adolescent idiopathic scoliosis who are not suspected of having these disorders, although variants of unknown significance are relatively common. Clinical

  11. Adolescent idiopathic scoliosis: Indications and efficacy of nonoperative treatment

    PubMed Central

    Canavese, Federico; Kaelin, André

    2011-01-01

    The strategy for the treatment of idiopathic scoliosis depends essentially upon the magnitude and pattern of the deformity, and its potential for progression. Treatment options include observation, bracing and/or surgery. During the past decade, several studies have demonstrated that the natural history of adolescent idiopathic scoliosis can be positively affected by nonoperative treatment, especially bracing. Other forms of conservative treatment, such as chiropractic or osteopathic manipulation, acupuncture, exercise or other manual treatments, or diet and nutrition, have not yet been proven to be effective in controlling spinal deformity progression, and those with a natural history that is favorable at the completion of growth. Observation is appropriate treatment for small curves, curves that are at low risk of progression, and those with a natural history that is favorable at the completion of growth. Indications for brace treatment are a growing child presenting with a curve of 25°–40° or a curve less than 25° with documented progression. Curves of 20°–25° in patients with pronounced skeletal immaturity should also be treated. The purpose of this review is to provide information about conservative treatment of adolescent idiopathic scoliosis. Indications for conservative treatment, hours daily wear and complications of brace treatment as well as brace types are discussed. PMID:21221217

  12. [Genome-wide association study for adolescent idiopathic scoliosis].

    PubMed

    Ogura, Yoji; Kou, Ikuyo; Scoliosis, Japan; Matsumoto, Morio; Watanabe, Kota; Ikegawa, Shiro

    2016-04-01

    Adolescent idiopathic scoliosis(AIS)is a polygenic disease. Genome-wide association studies(GWASs)have been performed for a lot of polygenic diseases. For AIS, we conducted GWAS and identified the first AIS locus near LBX1. After the discovery, we have extended our study by increasing the numbers of subjects and SNPs. In total, our Japanese GWAS has identified four susceptibility genes. GWASs for AIS have also been performed in the USA and China, which identified one and three susceptibility genes, respectively. Here we review GWASs in Japan and abroad and functional analysis to clarify the pathomechanism of AIS. PMID:27013625

  13. Cardiac and respiratory responses to exercise in adolescent idiopathic scoliosis.

    PubMed Central

    Shneerson, J M

    1980-01-01

    Twenty girls, aged 11-15 years, with adolescent idiopathic scoliosis were studied before spinal fusion was performed. Each underwent a range of lung function tests and a standardised progressive exercise test on a bicycle ergometer. The resting lung function tests showed reduced peak flow rates, lung volumes, and maximum voluntary ventilation. The maximum oxygen uptake was slightly diminished but maximum exercise ventilation was normal. The latter was achieved by using a greater than normal fraction of the vital capacity in tidal breathing while exercising. Mild hyperventilation during submaximal exercise and a trend towards an exercise tachycardia with increasing body weight were observed. PMID:7434284

  14. Patients' experience of adolescent idiopathic scoliosis surgery: a phenomenological analysis.

    PubMed

    Honeyman, Cheryl; Davison, Jean

    2016-09-12

    Background Adolescent idiopathic scoliosis is a three-dimensional curvature of the spine of unknown cause that occurs in often otherwise fit young people. A complex surgical procedure is required for the most severe curves. Quantitative literature suggests scoliosis surgery improves patients' lives, while qualitative literature focuses on patients' concerns rather than their experience. Aims To explore how adolescents interpret their perioperative experience. Method Six participants, aged 15-18, were interviewed and transcripts were analysed. Findings Four themes were identified: shock, fears and worries; parental interaction; coping; and motivation and positivity. Conclusion Participants were reluctant to share concerns, however those they shared related more to fear of the unknown and lack of control than specific issues such as pain. Participants depended on their parents, especially their mothers, during the perioperative period, and they recognised their parents' stress. Participants coped well, were motivated and had a positive outlook. PMID:27615585

  15. Altered sensory-weighting mechanisms is observed in adolescents with idiopathic scoliosis

    PubMed Central

    Simoneau, Martin; Mercier, Pierre; Blouin, Jean; Allard, Paul; Teasdale, Normand

    2006-01-01

    Background Scoliosis is the most common type of spinal deformity. In North American children, adolescent idiopathic scoliosis (AIS) makes up about 90% of all cases of scoliosis. While its prevalence is about 2% to 3% in children aged between 10 to 16 years, girls are more at risk than boys for severe progression with a ratio of 3.6 to 1. The aim of the present study was to test the hypothesis that idiopathic scoliosis interferes with the mechanisms responsible for sensory-reweighting during balance control. Methods Eight scoliosis patients (seven female and one male; mean age: 16.4 years) and nine healthy adolescents (average age 16.5 years) participated in the experiment. Visual and ankle proprioceptive information was perturbed (eyes closed and/or tendon vibration) suddenly and then returned to normal (eyes open and/or no tendon vibration). An AMTI force platform was used to compute centre of pressure root mean squared velocity and sway density curve. Results For the control condition (eyes open and no tendon vibration), adolescent idiopathic scoliosis patients had a greater centre of pressure root mean squared velocity (variability) than control participants. Reintegration of ankle proprioception, when vision was either available or removed, led to an increased centre of pressure velocity variability for the adolescent idiopathic scoliosis patients whereas the control participants reduced their centre of pressure velocity variability. Moreover, in the absence of vision, adolescent idiopathic scoliosis exhibited an increased centre of pressure velocity variability when ankle proprioception was returned to normal (i.e. tendon vibration stopped). The analysis of the sway density plot suggests that adolescent idiopathic scoliosis patients, during sensory reintegration, do not scale appropriately their balance control commands. Conclusion Altogether, the present results demonstrate that idiopathic scoliosis adolescents have difficulty in reweighting sensory inputs

  16. AKAP2 identified as a novel gene mutated in a Chinese family with adolescent idiopathic scoliosis

    PubMed Central

    Li, Wei; Li, YaWei; Zhang, Lusi; Guo, Hui; Tian, Di; Li, Ying; Peng, Yu; Zheng, Yu; Dai, Yuliang; Xia, Kun; Lan, Xinqiang; Wang, Bing; Hu, Zhengmao

    2016-01-01

    Background Adolescent idiopathic scoliosis exhibits high heritability and is one of the most common spinal deformities found in adolescent populations. However, little is known about the disease-causing genes in families with adolescent idiopathic scoliosis exhibiting Mendelian inheritance. Objective The aim of this study was to identify the causative gene in a family with adolescent idiopathic scoliosis. Methods Whole-exome sequencing was performed on this family to identify the candidate gene. Sanger sequencing was conducted to validate the candidate mutations and familial segregation. Real-time QPCR was used to measure the expression level of the possible causative gene. Results We identified the mutation c.2645A>C (p.E882A) within the AKAP2 gene, which cosegregated with the adolescent idiopathic scoliosis phenotypes. AKAP2 is located in a previously reported linkage locus (IS4) on chromosome 9q31.2–q34.2 and has been implicated in skeletal development. The mutation was absent in dbSNP144, ESP6500 and 503 ethnicity-matched controls. Real-time QPCR revealed that the mRNA expression level in the patients was increased significantly compared with the family controls (p<0.0001). Conclusions AKAP2 was therefore implicated as a novel gene mutated in a Chinese family with adolescent idiopathic scoliosis. Further studies should be conducted to validate the results from the perspective of both the genetics and pathogenesis of this disease. PMID:26989089

  17. Postural Rehabilitation for Adolescent Idiopathic Scoliosis during Growth

    PubMed Central

    Weiss, Hans-Rudolf; Moramarco, Marc Michael; Borysov, Maksym; Lee, Sang Gil; Nan, Xiaofeng; Moramarco, Kathryn Ann

    2016-01-01

    Long-term follow-up of untreated patients with adolescent idiopathic scoliosis (AIS) indicates that, with the exception of some extremely severe cases, AIS does not have a significant impact on quality of life and does not result in dire consequences. In view of the relatively benign nature of AIS and the long-term complications of surgery, the indications for treatment should be reviewed. Furthermore, recent studies have shown that scoliosis-specific exercises focusing on postural rehabilitation can positively influence the spinal curvatures in growing adolescents. Experiential postural re-education is a conservative, non-invasive approach, and its role in the management of AIS warrants further study. This article reviews current evidence for the inclusion of various forms of postural reeducation in the management of AIS. Recent comprehensive reviews have been researched including a manual and PubMed search for evidence regarding the effectiveness of physical/postural re-education/physiotherapy programs in growing AIS patients. This search revealed that there were few studies on the application of postural re-education in the management of AIS. These studies revealed that postural re-education in the form of exercise rehabilitation programs may have a positive influence on scoliosis; however, the various programs were difficult to compare. More research is necessary. There is at present Level 1 evidence for the effectiveness of Schroth scoliosis exercises in the management of AIS. Whether this evidence can be extrapolated to include other forms of scoliosis- pattern-specific exercises requires further investigation. Because corrective postures theoretically reduce the asymmetric loading of the spinal deformities and reverse the vicious cycle of spinal curvature progression, their integration into AIS programs may be beneficial and should be further examined. PMID:27340540

  18. Postural Rehabilitation for Adolescent Idiopathic Scoliosis during Growth.

    PubMed

    Weiss, Hans-Rudolf; Moramarco, Marc Michael; Borysov, Maksym; Ng, Shu Yan; Lee, Sang Gil; Nan, Xiaofeng; Moramarco, Kathryn Ann

    2016-06-01

    Long-term follow-up of untreated patients with adolescent idiopathic scoliosis (AIS) indicates that, with the exception of some extremely severe cases, AIS does not have a significant impact on quality of life and does not result in dire consequences. In view of the relatively benign nature of AIS and the long-term complications of surgery, the indications for treatment should be reviewed. Furthermore, recent studies have shown that scoliosis-specific exercises focusing on postural rehabilitation can positively influence the spinal curvatures in growing adolescents. Experiential postural re-education is a conservative, non-invasive approach, and its role in the management of AIS warrants further study. This article reviews current evidence for the inclusion of various forms of postural reeducation in the management of AIS. Recent comprehensive reviews have been researched including a manual and PubMed search for evidence regarding the effectiveness of physical/postural re-education/physiotherapy programs in growing AIS patients. This search revealed that there were few studies on the application of postural re-education in the management of AIS. These studies revealed that postural re-education in the form of exercise rehabilitation programs may have a positive influence on scoliosis; however, the various programs were difficult to compare. More research is necessary. There is at present Level 1 evidence for the effectiveness of Schroth scoliosis exercises in the management of AIS. Whether this evidence can be extrapolated to include other forms of scoliosis- pattern-specific exercises requires further investigation. Because corrective postures theoretically reduce the asymmetric loading of the spinal deformities and reverse the vicious cycle of spinal curvature progression, their integration into AIS programs may be beneficial and should be further examined. PMID:27340540

  19. Current concepts and controversies on adolescent idiopathic scoliosis: Part I

    PubMed Central

    Sud, Alok; Tsirikos, Athanasios I

    2013-01-01

    Adolescent idiopathic scoliosis is the most common spinal deformity encountered by General Orthopaedic Surgeons. Etiology remains unclear and current research focuses on genetic factors that may influence scoliosis development and risk of progression. Delayed diagnosis can result in severe deformities which affect the coronal and sagittal planes, as well as the rib cage, waistline symmetry, and shoulder balance. Patient's dissatisfaction in terms of physical appearance and mechanical back pain, as well as the risk for curve deterioration are usually the reasons for treatment. Conservative management involves mainly bracing with the aim to stop or slow down scoliosis progression during growth and if possible prevent the need for surgical treatment. This is mainly indicated in young compliant patients with a large amount of remaining growth and progressive curvatures. Scoliosis correction is indicated for severe or progressive curves which produce significant cosmetic deformity, muscular pain, and patient discontent. Posterior spinal arthrodesis with Harrington instrumentation and bone grafting was the first attempt to correct the coronal deformity and replace in situ fusion. This was associated with high pseudarthrosis rates, need for postoperative immobilization, and flattening of sagittal spinal contour. Segmental correction techniques were introduced along with the Luque rods, Harri-Luque, and Wisconsin systems. Correction in both coronal and sagittal planes was not satisfactory and high rates of nonunion persisted until Cotrel and Dubousset introduced the concept of global spinal derotation. Development of pedicle screws provided a powerful tool to correct three-dimensional vertebral deformity and opened a new era in the treatment of scoliosis. PMID:23682172

  20. Expression Signatures of Long Noncoding RNAs in Adolescent Idiopathic Scoliosis

    PubMed Central

    Liu, Xiao-Yang; Wang, Liang; Yu, Bin; Zhuang, Qian-yu; Wang, Yi-Peng

    2015-01-01

    Purpose. Adolescent idiopathic scoliosis (AIS), the most common pediatric spinal deformity, is considered a complex genetic disease. Causing genes and pathogenesis of AIS are still unclear. This study was designed to identify differentially expressed long noncoding RNAs (lncRNAs) involving the pathogenesis of AIS. Methods. We first performed comprehensive screening of lncRNA and mRNA in AIS patients and healthy children using Agilent human lncRNA + mRNA Array V3.0 microarray. LncRNAs expression in different AIS patients was further evaluated using quantitative PCR. Results. A total of 139 lncRNAs and 546 mRNAs were differentially expressed between AIS patients and healthy control. GO and Pathway analysis showed that these mRNAs might be involved in bone mineralization, neuromuscular junction, skeletal system morphogenesis, nucleotide and nucleic acid metabolism, and regulation of signal pathway. Four lncRNAs (ENST00000440778.1, ENST00000602322.1, ENST00000414894.1, and TCONS_00028768) were differentially expressed between different patients when grouped according to age, height, classification, severity of scoliosis, and Risser grade. Conclusions. This study demonstrates the abnormal expression of lncRNAs and mRNAs in AIS, and the expression of some lncRNAs was related to clinical features. This study is helpful for further understanding of lncRNAs in pathogenesis, treatment, and prognosis of AIS. PMID:26421281

  1. The Vestibular-Evoked Postural Response of Adolescents with Idiopathic Scoliosis Is Altered

    PubMed Central

    Pialasse, Jean-Philippe; Descarreaux, Martin; Mercier, Pierre; Blouin, Jean; Simoneau, Martin

    2015-01-01

    Adolescent idiopathic scoliosis is a multifactorial disorder including neurological factors. A dysfunction of the sensorimotor networks processing vestibular information could be related to spine deformation. This study investigates whether feed-forward vestibulomotor control or sensory reweighting mechanisms are impaired in adolescent scoliosis patients. Vestibular evoked postural responses were obtained using galvanic vestibular stimulation while participants stood with their eyes closed and head facing forward. Lateral forces under each foot and lateral displacement of the upper body of adolescents with mild (n = 20) or severe (n = 16) spine deformation were compared to those of healthy control adolescents (n = 16). Adolescent idiopathic scoliosis patients demonstrated greater lateral displacement and net lateral forces than controls both during and immediately after vestibular stimulation. Altered sensory reweighting of vestibular and proprioceptive information changed balance control of AIS patients during and after vestibular stimulation. Therefore, scoliosis onset could be related to abnormal sensory reweighting, leading to altered sensorimotor processes. PMID:26580068

  2. The Vestibular-Evoked Postural Response of Adolescents with Idiopathic Scoliosis Is Altered.

    PubMed

    Pialasse, Jean-Philippe; Descarreaux, Martin; Mercier, Pierre; Blouin, Jean; Simoneau, Martin

    2015-01-01

    Adolescent idiopathic scoliosis is a multifactorial disorder including neurological factors. A dysfunction of the sensorimotor networks processing vestibular information could be related to spine deformation. This study investigates whether feed-forward vestibulomotor control or sensory reweighting mechanisms are impaired in adolescent scoliosis patients. Vestibular evoked postural responses were obtained using galvanic vestibular stimulation while participants stood with their eyes closed and head facing forward. Lateral forces under each foot and lateral displacement of the upper body of adolescents with mild (n = 20) or severe (n = 16) spine deformation were compared to those of healthy control adolescents (n = 16). Adolescent idiopathic scoliosis patients demonstrated greater lateral displacement and net lateral forces than controls both during and immediately after vestibular stimulation. Altered sensory reweighting of vestibular and proprioceptive information changed balance control of AIS patients during and after vestibular stimulation. Therefore, scoliosis onset could be related to abnormal sensory reweighting, leading to altered sensorimotor processes. PMID:26580068

  3. Low bone mineral status in adolescent idiopathic scoliosis

    PubMed Central

    Li, Xin-Feng; Li, Hai

    2008-01-01

    Adolescent idiopathic scoliosis (AIS) is a pathological entity of unknown etiology. The causes of osteoporosis or osteopenia in AIS remain undetermined. Whether poor bone quality is an etiologic factor remains controversial. To determine the correlation between low bone mineral status and AIS, a review of literature was performed. After a literature search from 1966 to June 2007 (using Medline, EMBASE, Cochrane DSR, ACP Journal Club, DARE, CCTR, CINAHL and hand searches of references) for studies regarding low bone mineral status and AIS, 20 studies meeting the inclusion criteria were reviewed in terms of the appropriateness of valuation technique, the validity of descriptive system, the number and type of respondents, and overall quality of the studies. Nearly all investigations demonstrated that low bone mineral density (BMD) was a generalized phenomenon and a systematic disorder in AIS. The prevalence of AIS with osteoporosis is approximately 20–38%. The follow-up studies indicated that osteopenia in patients with AIS may be a persistent phenomenon. BMD could be affected by the mechanical loading and lower bone mineral mass is always associated with lower bone strength. The spinal architecture associated with the osteopenia may aggravate the spinal deformity. However, with regard to the concave and convex femoral neck BMD values, and the correlation of BMD to scoliosis parameters, the results remain inconsistent. Bracing may not result in permanent loss of bone mineral mass. The effect of the eccentric tension–compression environments on BMD, the correlation of BMD with scoliosis parameters and the effect of bracing on BMD should be investigated further in prospective, randomized and longitudinal follow-up studies. PMID:18751741

  4. Current concepts and controversies on adolescent idiopathic scoliosis: Part II

    PubMed Central

    Sud, Alok; Tsirikos, Athanasios I

    2013-01-01

    A new era in the surgical treatment of adolescent idiopathic scoliosis (AIS) opened with the introduction of pedicle screw instrumentation, which provides 3-column vertebral fixation and allows major deformity correction on the coronal, sagittal, and axial planes. A steep learning curve can be expected for spinal surgeons to become familiar with pedicle screw placement and correction techniques. Potential complications including injury to adjacent neural, vascular, and visceral structures can occur due to screw misplacement or pull-out during correction maneuvers. These major complications are better recognized as pedicle screw techniques become more popular and may result in serious morbidity and mortality. Extensive laboratory and clinical training is mandatory before pedicle screw techniques in scoliosis surgery are put to practice. Wider application, especially in developing countries, is limited by the high cost of implants. Refined correction techniques are currently developed and these utilize a lesser number of pedicle anchors which are strategically positioned to allow optimum deformity correction while reducing the neurological risk, surgical time, and blood loss, as well as instrumentation cost. Such techniques can be particularly attractive at a time when cost has major implications on provision of health care as they can make scoliosis treatment available to a wider population of patients. Pedicle screw techniques are currently considered the gold standard for scoliosis correction due to their documented superior biomechanical properties and ability to produce improved clinical outcomes as reflected by health-related quality-of-life questionnaires. Ongoing research promises further advances with the future of AIS treatment incorporating genetic counseling and possibly fusionless techniques. PMID:23798750

  5. Gait in adolescent idiopathic scoliosis: kinematics and electromyographic analysis.

    PubMed

    Mahaudens, P; Banse, X; Mousny, M; Detrembleur, C

    2009-04-01

    Adolescent idiopathic scoliosis (AIS) is a progressive growth disease that affects spinal anatomy, mobility, and left-right trunk symmetry. Consequently, AIS can modify human locomotion. Very few studies have investigated a simple activity like walking in a cohort of well-defined untreated patients with scoliosis. The first goal of this study is to evaluate the effects of scoliosis and scoliosis severity on kinematic and electromyographic (EMG) gait variables compared to an able-bodied population. The second goal is to look for any asymmetry in these parameters during walking. Thirteen healthy girls and 41 females with untreated AIS, with left thoracolumbar or lumbar primary structural curves were assessed. AIS patients were divided into three clinical subgroups (group 1 < 20 degrees, group 2 between 20 and 40 degrees, and group 3 > 40 degrees). Gait analysis included synchronous bilateral kinematic and EMG measurements. The subjects walked on a treadmill at 4 km/h (comfortable speed). The tridimensional (3D) shoulder, pelvis, and lower limb motions were measured using 22 reflective markers tracked by four infrared cameras. The EMG timing activity was measured using bipolar surface electrodes on quadratus lumborum, erector spinae, gluteus medius, rectus femoris, semitendinosus, tibialis anterior, and gastrocnemius muscles. Statistical comparisons (ANOVA) were performed across groups and sides for kinematic and EMG parameters. The step length was reduced in AIS compared to normal subjects (7% less). Frontal shoulder, pelvis, and hip motion and transversal hip motion were reduced in scoliosis patients (respectively, 21, 27, 28, and 22% less). The EMG recording during walking showed that the quadratus lumborum, erector spinae, gluteus medius, and semitendinosus muscles contracted during a longer part of the stride in scoliotic patients (46% of the stride) compared with normal subjects (35% of the stride). There was no significant difference between scoliosis groups 1

  6. Hirschsprung's disease and idiopathic megacolon in adults and adolescents.

    PubMed Central

    Barnes, P R; Lennard-Jones, J E; Hawley, P R; Todd, I P

    1986-01-01

    The distinction between Hirschsprung's disease and idiopathic megacolon in childhood dates from the classic clinical, radiological, and histological studies of Bodian, Stephens, and Ward. This article describes clinical experience over 15 years of 94 patients in whom megacolon of these two types was recognised for the first time after the age of 10, to illustrate the problems of diagnosis and treatment in later years. Just as it is now recognised that patients with the clinical characteristics of Hirschsprung's disease may have one of several abnormalities of the myenteric plexus, including not only absence of ganglion cells, but also patchy or zonal loss, abnormal neurones or neuronal dysplasia, so idiopathic megacolon may also be a heterogeneous group of cases. This paper suggests on clinical grounds that those patients with idiopathic megacolon whose symptoms start in childhood differ from those whose symptoms develop in later years. Images Fig. 1 Fig. 2 Fig. 6 PMID:3699562

  7. Sleep, stress, neurocognitive profile and health-related quality of life in adolescents with idiopathic musculoskeletal pain

    PubMed Central

    Molina, Juliana; Dos Santos, Flávia Heloísa; Terreri, Maria Teresa R. A.; Fraga, Melissa Mariti; Silva, Simone Guerra; Hilário, Maria Odete E.; Len, Claudio A.

    2012-01-01

    OBJECTIVES: The aims of this study were to measure levels of sleep, stress, and depression, as well as health-related quality of life, and to assess the neurocognitive profiles in a sample of adolescents with idiopathic musculoskeletal pain. METHODS: Nineteen adolescents with idiopathic musculoskeletal pain and 20 age-matched healthy control subjects were evaluated regarding their levels of sleep and stress, as well as quality of life, and underwent neurocognitive testing. RESULTS: The sample groups consisted predominantly of females (84%), and the socioeconomic status did not differ between the two groups. In addition, the occurrence of depressive symptoms was similar between the two groups; specifically, 26% of the idiopathic musculoskeletal pain patients and 30% of the control subjects had scores indicative of depression. Teenagers in the group with idiopathic musculoskeletal pain reported poorer quality of life and sleep scores than those in the control group. Regarding stress, patients had worse scores than the control group; whereas 79% of the adolescents with idiopathic musculoskeletal pain met the criteria for a diagnosis of stress, only 35% of the adolescents in the control group met the criteria. In both groups, we observed scores that classified adolescents as being in the resistance phase (intermediate) and exhaustion phase (pathological) of distress. However, the idiopathic musculoskeletal pain group more frequently reported symptomatic complaints of physical and emotional distress. The neurocognitive assessment showed no significant impairments in either group. CONCLUSION: Adolescents with idiopathic musculoskeletal pain did not exhibit cognitive impairments. However, adolescents with idiopathic musculoskeletal pain did experience intermediate to advanced psychological distress and lower health-related quality of life, which may increase their risk of cognitive dysfunction in the future. PMID:23070339

  8. Non-Surgical Interventions for Adolescents with Idiopathic Scoliosis: An Overview of Systematic Reviews

    PubMed Central

    Płaszewski, Maciej; Bettany-Saltikov, Josette

    2014-01-01

    Background Non-surgical interventions for adolescents with idiopathic scoliosis remain highly controversial. Despite the publication of numerous reviews no explicit methodological evaluation of papers labeled as, or having a layout of, a systematic review, addressing this subject matter, is available. Objectives Analysis and comparison of the content, methodology, and evidence-base from systematic reviews regarding non-surgical interventions for adolescents with idiopathic scoliosis. Design Systematic overview of systematic reviews. Methods Articles meeting the minimal criteria for a systematic review, regarding any non-surgical intervention for adolescent idiopathic scoliosis, with any outcomes measured, were included. Multiple general and systematic review specific databases, guideline registries, reference lists and websites of institutions were searched. The AMSTAR tool was used to critically appraise the methodology, and the Oxford Centre for Evidence Based Medicine and the Joanna Briggs Institute’s hierarchies were applied to analyze the levels of evidence from included reviews. Results From 469 citations, twenty one papers were included for analysis. Five reviews assessed the effectiveness of scoliosis-specific exercise treatments, four assessed manual therapies, five evaluated bracing, four assessed different combinations of interventions, and one evaluated usual physical activity. Two reviews addressed the adverse effects of bracing. Two papers were high quality Cochrane reviews, Three were of moderate, and the remaining sixteen were of low or very low methodological quality. The level of evidence of these reviews ranged from 1 or 1+ to 4, and in some reviews, due to their low methodological quality and/or poor reporting, this could not be established. Conclusions Higher quality reviews indicate that generally there is insufficient evidence to make a judgment on whether non-surgical interventions in adolescent idiopathic scoliosis are effective. Papers

  9. BENDING RADIOGRAPHS AS A PREDICTIVE FACTOR IN SURGICAL CORRECTION OF ADOLESCENT IDIOPATHIC SCOLIOSIS

    PubMed Central

    Gotfryd, Alberto Ofenhejm; Franzin, Fernando José; Poletto, Patrícia Rios; de Laura, Alexandre Spertini; da Silva, Luis Carlos Ferreira

    2015-01-01

    Objective: To evaluate the use of x-rays in dorsal decubitus, as a predictive factor for surgical correction of the main thoracic curve using pedicle screws, on patients with idiopathic adolescent scoliosis. Method: Twenty patients with idiopathic adolescent scoliosis of Lenke types 1A and 1B who were operated using a technique only involving pedicle screws by means of the posterior route were evaluated clinically and radiographically. The curve flexibility was calculated by means of active supine lateral oblique radiographs. The postoperative values for the main thoracic curve were included in a mathematical equation proposed by Cheung et al., with the aim of predicting the expected angular result from the surgical correction. The difference between the expected and actual postoperative results was then investigated regarding its statistical significance. Results: There was statistical significance for all the cases studied, between the values predicted before the operation and the radiographic findings immediately after the operation (p < 0.005). Conclusions: It is possible to predict the percentage surgical correction of the main thoracic curve that will be achieved using pedicle screws in patients with idiopathic adolescent scoliosis of Lenke types 1A and 1B, by means of preoperative supine oblique radiographs. PMID:27027056

  10. Gait in adolescent idiopathic scoliosis: energy cost analysis.

    PubMed

    Mahaudens, P; Detrembleur, C; Mousny, M; Banse, X

    2009-08-01

    Walking is a very common activity for the human body. It is so common that the musculoskeletal and cardiovascular systems are optimized to have the minimum energetic cost at 4 km/h (spontaneous speed). A previous study showed that lumbar and thoracolumbar adolescent idiopathic scoliosis (AIS) patients exhibit a reduction of shoulder, pelvic, and hip frontal mobility during gait. A longer contraction duration of the spinal and pelvic muscles was also noted. The energetic cost (C) of walking is normally linked to the actual mechanical work muscles have to perform. This total mechanical work (W(tot)) can be divided in two parts: the work needed to move the shoulders and lower limbs relative to the center of mass of the body (COM(b)) is known as the internal work (W(int)), whereas additional work, known as external work (W(ext)), is needed to accelerate and lift up the COM(b) relative to the ground. Normally, the COM(b) goes up and down by 3 cm with every step. Pathological walking usually leads to an increase in W (tot) (often because of increased vertical displacement of the COM(b)), and consequently, it increases the energetic cost. The goal of this study is to investigate the effects of scoliosis and scoliosis severity on the mechanical work and energetic cost of walking. Fifty-four female subjects aged 12 to 17 were used in this study. Thirteen healthy girls were in the control group, 12 were in scoliosis group 1 (Cobb angle [Cb] < or = 20 degrees), 13 were in scoliosis group 2 (20 degrees < Cb < 40 degrees), and 16 were in scoliosis group 3 (Cb > or = 40 degrees). They were assessed by physical examination and gait analysis. The 41 scoliotic patients had an untreated progressive left thoracolumbar or lumbar AIS. During gait analysis, the subject was asked to walk on a treadmill at 4 km h(-1). Movements of the limbs were followed by six infrared cameras, which tracked markers fixed on the body. W(int) was calculated from the kinematics. The movements of the COM

  11. Adolescent Idiopathic Scoliosis and Eating Disorders: Is There a Relation? Results of a Cross-Sectional Study

    ERIC Educational Resources Information Center

    Zaina, Fabio; Donzelli, Sabrina; Lusini, Monia; Vismara, Luca; Capodaglio, Paolo; Neri, Laura; Negrini, Stefano

    2013-01-01

    A recent study suggests a correlation between idiopathic scoliosis in adolescence and eating disorders. However, this does not correspond with our clinical experience in the same population. The aim of this study was to verify the correlation between scoliosis and eating disorders in adolescence. A cross-sectional study was designed including 187…

  12. Torsional deformity of apical vertebra in adolescent idiopathic scoliosis.

    PubMed

    Kotwicki, Tomasz; Napiontek, Marek

    2002-01-01

    CT scans of structural thoracic idiopathic scoliosis were reviewed in nine patients admitted to our department for scoliosis surgery. The apical vertebra scans were chosen and the following parameters were evaluated: 1) alpha angle formed by the axis of vertebra and the axis of spinous process 2) beta concave and beta convex angle between the spinous process and the left and right transverse process, respectively, 3) gamma concave and gamma convex angle between the axis of vertebra and the left and right transverse process, respectively, 4) the rotation angle to the sagittal plane. The constant deviation of the spinous process towards the convex side of the curve was observed. The vertebral body itself was distorted towards the concavity of the curve. The angle between the spinous process and the transverse process was smaller on the convex side of the curve. The torsional, intravertebral deformity of the apical vertebra was a factor acting in the direction opposite to the rotation, in the sense to reduce the deformity of the spine in idiopathic scoliosis. PMID:15456062

  13. Health and identity: Self-positioning in adolescent chronic fatigue syndrome and juvenile idiopathic arthritis.

    PubMed

    Fuchs, Coralie E; van Geelen, Stefan M; van Geel, Rolf; Sinnema, Gerben; van de Putte, Elise M; Hermans, Hubert J M; Kuis, Wietse

    2013-07-01

    The aim of this study is to gain more insight into basic aspects of identity, in relation to adolescent chronic fatigue syndrome (CFS) and juvenile idiopathic arthritis (JIA). In dialogical self theory, identity is regarded as incorporating multiple self-positions, such as 'I as tired', 'I as pessimistic', or 'I as decisive'. Physical and psychosocial impairment might alter the organization of these self-positions. The Personal Position Repertoire procedure, a quantitative method to analyse the prominence of self-positions, the Child Health Questionnaire, assessing health-related functioning, and the Checklist Individual Strength, measuring fatigue, were completed by 42 adolescents with CFS, 37 adolescents with JIA and 23 healthy teenagers. Adolescents with JIA report impaired physical functioning and general health. However, they position themselves very similar to healthy teenagers - i.e. as strong and healthy. While this self-positioning approach might be adequate and sustainable in adolescence, it could prove too strenuous to maintain throughout adult life. Adolescents with CFS, besides indicating severe physical difficulties, also report more psychosocial problems. They position themselves as significantly less strong and more unwell. With this emphasis on positions relating to their illness, there seems to be little room left for stronger positions. It is regarded of clinical importance to address these issues in this crucial developmental period. PMID:23060600

  14. Adolescent idiopathic scoliosis – to operate or not? A debate article

    PubMed Central

    Weiss, Hans-Rudolf; Bess, Shay; Wong, Man Sang; Patel, Vikas; Goodall, Deborah; Burger, Evalina

    2008-01-01

    Adolescent idiopathic scoliosis (AIS) represents a rare condition with a potentially detrimental impact on young patients. Despite vast clinical research and published treatment guidelines and algorithms, the optimal therapeutic choice for these patients remains highly controversial. While advocates of early surgery emphasize the benefits of surgical deformity correction with regard to physical and psychological outcome, the opponents base their arguments on the high risk of complications and a lack of documented subjective long-term outcome. In the present paper, the authors were invited to debate the opposite positions of "pro" versus "contra" surgical treatment of AIS, based on the currently available evidence and published guidelines. PMID:18826571

  15. Predictors of curve flexibility in adolescent idiopathic scoliosis: a retrospective study of 100 patients.

    PubMed

    Ameri, Ebrahim; Behtash, Hamid; Mobini, Bahram; Daraie, Ariasb

    2015-01-01

    Curve flexibility in adolescent idiopathic scoliosis (AIS) was one of the major concerns of spinal surgeons since the evolution of surgical correction techniques. In this respect, many tried to identify which criteria denote more rigid curve. In the present study, we aimed toward determining important factors influencing AIS curve flexibility on supine bending films. We assessed radiographs of 100 patients with AIS for direction of curves, number of involved vertebrae, apical vertebral translation and rotation, magnitude of main thoracic curve and T5-T12 kyphosis. Statistical analysis performed via stepwise linear regression model with these variables plus age and sex against flexibility index. According to regression analysis, there was a clear relationship between flexibility indexes (FI) and magnitude of main thoracic curve at all (P<0.001). When we consider flexible curves (FI>50%) against rigid curves, apical vertebral rotation was a major determinant of curve flexibility also (P<0.001). Adolescent idiopathic scoliosis curves with larger Cobb's angle and apical vertebral rotation show less flexibility on supine bending films. PMID:25796026

  16. Ultrasound evaluation of the symmetry of abdominal muscles in mild adolescent idiopathic scoliosis

    PubMed Central

    Linek, Paweł; Saulicz, Edward; Wolny, Tomasz; Myśliwiec, Andrzej; Gogola, Anna

    2015-01-01

    [Purpose] The objective of the study was to evaluate the symmetry of the thickness of the abdominal muscles at rest and while standing in patients with adolescent idiopathic scoliosis. [Subjects and Methods] An ultrasound assessment was performed of the side-to-side differences of the external oblique (EO), internal oblique (IO), and transversus abdominalis (TrA) muscles in the supine and standing positions in adolescent idiopathic scoliosis (AIS) and control groups. [Results] In the AIS group, 64.3% of the patients had left scoliosis with a mean Cobb angle of 10.7°, and 35.7% of the patients had right scoliosis with a mean Cobb angle of 10°. In the supine position, the thickness asymmetry of the TrA was greater in the AIS compared with the control group by an average of 14% (95% CI 3.9–24.2). [Conclusion] Among the abdominal muscles examined, patients with AIS exhibited more asymmetry only for the TrA. In the standing position, the TrA was as symmetric in the patients as in the control group. Mild scoliosis has no impact on the symmetry of the thickness of the OE and OI in the supine and standing positions. The direction of curvature had no effect on the symmetry of the abdominal muscles studied. PMID:25729192

  17. Ultrasound evaluation of the symmetry of abdominal muscles in mild adolescent idiopathic scoliosis.

    PubMed

    Linek, Paweł; Saulicz, Edward; Wolny, Tomasz; Myśliwiec, Andrzej; Gogola, Anna

    2015-02-01

    [Purpose] The objective of the study was to evaluate the symmetry of the thickness of the abdominal muscles at rest and while standing in patients with adolescent idiopathic scoliosis. [Subjects and Methods] An ultrasound assessment was performed of the side-to-side differences of the external oblique (EO), internal oblique (IO), and transversus abdominalis (TrA) muscles in the supine and standing positions in adolescent idiopathic scoliosis (AIS) and control groups. [Results] In the AIS group, 64.3% of the patients had left scoliosis with a mean Cobb angle of 10.7°, and 35.7% of the patients had right scoliosis with a mean Cobb angle of 10°. In the supine position, the thickness asymmetry of the TrA was greater in the AIS compared with the control group by an average of 14% (95% CI 3.9-24.2). [Conclusion] Among the abdominal muscles examined, patients with AIS exhibited more asymmetry only for the TrA. In the standing position, the TrA was as symmetric in the patients as in the control group. Mild scoliosis has no impact on the symmetry of the thickness of the OE and OI in the supine and standing positions. The direction of curvature had no effect on the symmetry of the abdominal muscles studied. PMID:25729192

  18. An intraoperative irrigation regimen to reduce the surgical site infection rate following adolescent idiopathic scoliosis surgery.

    PubMed

    Herwijnen, B van; Evans, N R; Dare, C J; Davies, E M

    2016-05-01

    Introduction The aim of this study was to compare the efficacy of a gentamicin antibiotic intraoperative irrigation regimen (regimen A) with a povidone-iodine intraoperative irrigation regimen (regimen B) and to evaluate the ability of adjunctive local vancomycin powder (regimen C) to reduce the surgical site infection (SSI) rate following idiopathic scoliosis correction. Methods This was a retrospective, single centre, two-surgeon cohort study of paediatric scoliosis procedures involving 118 patients under the age of 18 years who underwent correction for idiopathic scoliosis over a period of 42 months. Patients' baseline characteristics, pseudarthrosis and rates of SSI were compared. Results Baseline characteristics were comparable in all three groups, with the exception of sex distribution. Over a quarter (27%) of patients with regimen B were male compared with 13% and 6% for regimens A and C respectively. Patients were mostly followed up for a minimum of 12 months. The SSI rate for both superficial and deep infections was higher with regimen A (26.7%) than with regimens B and C (7.0% and 6.3% respectively). The SSI rates for regimens B and C were comparable. No patients developed complications related to vancomycin toxicity, metalwork failure or pseudarthrosis. Conclusions Wound irrigation with a povidone-iodine solution reduces SSIs following adolescent idiopathic scoliosis surgery. The direct application of vancomycin powder to the wound is safe but does not reduce the SSI rate further in low risk patients. Additional studies are needed to elucidate whether it is effective at higher doses and in high risk patient groups. PMID:27087324

  19. Electromyographic Assessment of Functional Symmetry of Paraspinal Muscles during Static Exercises in Adolescents with Idiopathic Scoliosis

    PubMed Central

    Chwała, Wiesław; Koziana, Agnieszka; Kasperczyk, Tadeusz; Płaszewski, Maciej

    2014-01-01

    Background. The question of how to correct and rehabilitate scoliosis remains one of the most difficult problems of orthopaedics. Controversies continue to arise regarding various types of both symmetric and asymmetric scoliosis-specific therapeutic exercises. Objective. The aim of the present paper was to conduct an electromyographic assessment of functional symmetry of paraspinal muscles during symmetric and asymmetric exercises in adolescents with idiopathic scoliosis. Materials and Methods. The study was conducted in a group of 82 girls, mean age 12.4 ± 2.3 years with single- or double-major-idiopathic scoliosis, Cobb angle 24 ± 9.4°. The functional biopotentials during isometric work of paraspinal muscles in “at rest” position and during two symmetric and four asymmetric exercises were measured with the use of the Muscle Tester ME 6000 electromyograph. Results. In general, asymmetric exercises were characterised by larger differences in bioelectrical activity of paraspinal muscles, in comparison with symmetric exercises, both in the groups of patients with single-curve and double-curve scoliosis. Conclusion. During symmetric and asymmetric exercises, muscle tension patterns differed significantly in both groups, in comparison with the examination at rest, in most cases generating positive corrective patterns. Asymmetric exercises generated divergent muscle tension patterns on the convex and concave sides of the deformity. PMID:25258713

  20. Idiopathic Bilateral Gluteus Maximus Contracture in Adolescent Female: A Case Report

    PubMed Central

    Pathak, Abhishek; Shukla, Jiten

    2013-01-01

    Introduction: Bilateral gluteus muscle contracture is a rare entity. “Idiopathic contracture of gluteus maximus” a term proposed by Gao is a rare condition. It present as typical complain of inability to squat with patient assuming a typical frog leg position. Case Report: We report a case of idiopathic gluteal muscle contracture in adolescent female presenting with inability to squat, sit cross legged and painless awkward gait. The patient could not sit comfortably except in a high chair position. No history of repeated intramuscular injections in the buttocks or signs suggestive of post-polio residual paralysis. All her investigations were within normal limit. Our patient belonged to type III according to GMC classification and underwent surgical release of fibrotic band of gluteal maximus muscle. Patient had remarkable recovery with patient able to squat and sit cross-legged within 4 weeks of surgery. Conclusion: Gluteal maximus contracture, though rare, present with characteristic clinical symptom and should not be missed. Our patient though presented late showed excellent result after surgical release. PMID:27298891

  1. Developmental dyscalculia in children and adolescents with idiopathic epilepsies in a Brazilian sample.

    PubMed

    Thomé, Ursula; Paixão Alves, Sandra Regina da; Guerreiro, Sabrina Mendonça; Machado da Costa, Célia Regina Carvalho; Souza Moreira, Fernanda de; Bandeira Lima, Andrea; Ferreira Tavares, Maria Rita; Souza Maia Filho, Heber

    2014-04-01

    Epilepsy is one of the most prevalent chronic disorders of childhood which can threaten child development and mental health. Among cognitive disorders, dyscalculia is one of the most important. In this study, 39 children and adolescents with idiopathic epilepsy underwent clinical and neuropsychological assessment to determine the intellectual level, math skills, reading and writing performance and neuropsychological profile. It was observed that the mathematical ability was below schooling expectations in a higher frequency than expected. There were no significant differences in mathematical performance among groups divided by number of antiepileptic drugs used, duration of disease and types and frequency of seizures. There was a positive correlation with intelligence quotient and attentional and reading level. These results suggest the existence not only of dyscalculia, but the concurrence of attentional and reading problems for the poor mathematical performance in this population. PMID:24760092

  2. Accuracy of robot-assisted pedicle screw placement for adolescent idiopathic scoliosis in the pediatric population.

    PubMed

    Macke, Jeremy J; Woo, Raymund; Varich, Laura

    2016-06-01

    This is a retrospective review of pedicle screw placement in adolescent idiopathic scoliosis (AIS) patients under 18 years of age who underwent robot-assisted corrective surgery. Our primary objective was to characterize the accuracy of pedicle screw placement with evaluation by computed tomography (CT) after robot-assisted surgery in AIS patients. Screw malposition is the most frequent complication of pedicle screw placement and is more frequent in AIS. Given the potential for serious complications, the need for improved accuracy of screw placement has spurred multiple innovations including robot-assisted guidance devices. No studies to date have evaluated this robot-assisted technique using CT exclusively within the AIS population. Fifty patients were included in the study. All operative procedures were performed at a single institution by a single pediatric orthopedic surgeon. We evaluated the grade of screw breach, the direction of screw breach, and the positioning of the patient for preoperative scan (supine versus prone). Of 662 screws evaluated, 48 screws (7.2 %) demonstrated a breach of greater than 2 mm. With preoperative prone position CT scanning, only 2.4 % of screws were found to have this degree of breach. Medial malposition was found in 3 % of screws, a rate which decreased to 0 % with preoperative prone position scanning. Based on our results, we conclude that the proper use of image-guided robot-assisted surgery can improve the accuracy and safety of thoracic pedicle screw placement in patients with adolescent idiopathic scoliosis. This is the first study to evaluate the accuracy of pedicle screw placement using CT assessment in robot-assisted surgical correction of patients with AIS. In our study, the robot-assisted screw misplacement rate was lower than similarly constructed studies evaluating conventional (non-robot-assisted) procedures. If patients are preoperatively scanned in the prone position, the misplacement rate is further

  3. Heated indoor swimming pools, infants, and the pathogenesis of adolescent idiopathic scoliosis: a neurogenic hypothesis

    PubMed Central

    2011-01-01

    Background In a case-control study a statistically significant association was recorded between the introduction of infants to heated indoor swimming pools and the development of adolescent idiopathic scoliosis (AIS). In this paper, a neurogenic hypothesis is formulated to explain how toxins produced by chlorine in such pools may act deleteriously on the infant's immature central nervous system, comprising brain and spinal cord, to produce the deformity of AIS. Presentation of the hypothesis Through vulnerability of the developing central nervous system to circulating toxins, and because of delayed epigenetic effects, the trunk deformity of AIS does not become evident until adolescence. In mature healthy swimmers using such pools, the circulating neurotoxins detected are chloroform, bromodichloromethane, dibromochloromethane, and bromoform. Cyanogen chloride and dichloroacetonitrile have also been detected. Testing the hypothesis In infants, the putative portals of entry to the blood could be dermal, oral, or respiratory; and entry of such circulating small molecules to the brain are via the blood-brain barrier, blood-cerebrospinal fluid barrier, and circumventricular organs. Barrier mechanisms of the developing brain differ from those of adult brain and have been linked to brain development. During the first 6 months of life cerebrospinal fluid contains higher concentrations of specific proteins relative to plasma, attributed to mechanisms continued from fetal brain development rather than immaturity. Implications of the hypothesis The hypothesis can be tested. If confirmed, there is potential to prevent some children from developing AIS. PMID:21975145

  4. Predicting success or failure of brace treatment for adolescents with idiopathic scoliosis.

    PubMed

    Chalmers, Eric; Westover, Lindsey; Jacob, Johith; Donauer, Andreas; Zhao, Vicky H; Parent, Eric C; Moreau, Marc J; Mahood, James K; Hedden, Douglas M; Lou, Edmond H M

    2015-10-01

    Adolescent idiopathic scoliosis (AIS) is a three-dimensional spinal deformity. Brace treatment is a common non-surgical treatment, intended to prevent progression (worsening) of the condition during adolescence. Estimating a braced patient's risk of progression is an essential part of planning treatment, so method for predicting this risk would be a useful decision support tool for practitioners. This work attempts to discover whether failure of brace treatment (progression) can be predicted at the start of treatment. Records were obtained for 62 AIS patients who had completed brace treatment. Subjects were labeled as "progressive" if their condition had progressed despite brace treatment and "non-progressive" otherwise. Wrapper-based feature selection selected two useful predictor variables from a list of 14 clinical measurements taken from the records. A logistic regression model was trained to classify patients as "progressive" or "non-progressive" using these two variables. The logistic regression model's simplicity and interpretability should facilitate its clinical acceptance. The model was tested on data from an additional 28 patients and found to be 75 % accurate. This accuracy is sufficient to make the predictions clinically useful. It can be used online: http://www.ece.ualberta.ca/~dchalmer/SimpleBracePredictor.html . PMID:26002592

  5. The Prevalence of Idiopathic Scoliosis in Eleven Year-Old Korean Adolescents: A 3 Year Epidemiological Study

    PubMed Central

    Lee, Jin-Young; Moon, Seong-Hwan; Kim, Han Jo; Suh, Bo-Kyung; Nam, Ji Hoon; Jung, Jae Kyun; Lee, Hwan-Mo

    2014-01-01

    Purpose School screening allows for early detection and early treatment of scoliosis, with the purpose of reducing the number of patients requiring surgical treatment. Children between 10 and 14 years old are considered as good candidates for school screening tests of scoliosis. The purpose of the present study was to assess the epidemiological findings of idiopathic scoliosis in 11-year-old Korean adolescents. Materials and Methods A total of 37856 11-year-old adolescents were screened for scoliosis. There were 17110 girls and 20746 boys. Adolescents who were abnormal by Moiré topography were subsequently assessed by standardized clinical and radiological examinations. A scoliotic curve was defined as 10° or more. Results The prevalence of scoliosis was 0.19% and most of the curves were small (10° to 19°). The ratio of boys to girls was 1:5.5 overall. Sixty adolescents (84.5%) exhibited single curvature. Thoracolumbar curves were the most common type of curve identified, followed by thoracic and lumbar curves. Conclusion The prevalence of idiopathic scoliosis among 11-year-old Korean adolescents was 0.19%. PMID:24719147

  6. FUNCTIONAL AND RADIOGRAPHIC COMPARISON OF ANTERIOR AND POSTERIOR INSTRUMENTATION FOR THE CORRECTION OF ADOLESCENT IDIOPATHIC SCOLIOSIS

    PubMed Central

    Vieira, Juliano Silveira; da Silva Herrero, Carlos Fernando Pereira; Porto, Maximiliano Aguiar; Arlet, Vincent; Defino, Helton Luiz Aparecido

    2015-01-01

    Objective: This was a retrospective study to compare the anterior instrumentation (AI) and posterior instrumentation (PI) results among patients diagnosed with adolescent idiopathic scoliosis (Lenke type I) who were treated surgically. Methods: The results from 24 patients aged 11 to 18 years with Lenke type I idiopathic scoliosis who underwent surgery with AI (12 patients) or PI (12 patients) were compared. All the patients were operated by the same surgeon and were followed up for a minimum period of five years. The variables for comparison included: coronal and sagittal correction, distance from apical vertebra to midline, apical vertebral rotation, number of instrumented vertebrae and functional variables (by means of the SRS-22 questionnaire). The data obtained were analyzed using the SAS software, version 9. The two groups were compared using Student's t-test with a significance level of 5% (0.05). Results: The correction of the curve in the frontal plane was higher in the group of patients with the anterior approach, in the immediate (p=0.031) and late (p=0.043) postoperative periods, as was the apical vertebral rotation during the immediate (p=0.002) and late (p=0.019) evaluations. The number of instrumented vertebrae was 7.69 ± 1.38 in the AI group and 11.38 ± 2.92 in the PI group (p = 0.021). Functional assessment (SRS-22) did not show any significant difference (p > 0.05) between the groups. Conclusion: The patients who underwent scoliosis correction with AI presented greater correction in the frontal plane, greater derotation of apical vertebrae and a smaller number of fused vertebrae. PMID:27026964

  7. Three-Dimensional Vertebral Wedging in Mild and Moderate Adolescent Idiopathic Scoliosis

    PubMed Central

    Scherrer, Sophie-Anne; Begon, Mickaël; Leardini, Alberto; Coillard, Christine; Rivard, Charles-Hilaire; Allard, Paul

    2013-01-01

    Background Vertebral wedging is associated with spinal deformity progression in adolescent idiopathic scoliosis. Reporting frontal and sagittal wedging separately could be misleading since these are projected values of a single three-dimensional deformation of the vertebral body. The objectives of this study were to determine if three-dimensional vertebral body wedging is present in mild scoliosis and if there are a preferential vertebral level, position and plane of deformation with increasing scoliotic severity. Methodology Twenty-seven adolescent idiopathic scoliotic girls with mild to moderate Cobb angles (10° to 50°) participated in this study. All subjects had at least one set of bi-planar radiographs taken with the EOS® X-ray imaging system prior to any treatment. Subjects were divided into two groups, separating the mild (under 20°) from the moderate (20° and over) spinal scoliotic deformities. Wedging was calculated in three different geometric planes with respect to the smallest edge of the vertebral body. Results Factorial analyses of variance revealed a main effect for the scoliosis severity but no main effect of vertebral Levels (apex and each of the three vertebrae above and below it) (F = 1.78, p = 0.101). Main effects of vertebral Positions (apex and above or below it) (F = 4.20, p = 0.015) and wedging Planes (F = 34.36, p<0.001) were also noted. Post-hoc analysis demonstrated a greater wedging in the inferior group of vertebrae (3.6°) than the superior group (2.9°, p = 0.019) and a significantly greater wedging (p≤0.03) along the sagittal plane (4.3°). Conclusions Vertebral wedging was present in mild scoliosis and increased as the scoliosis progressed. The greater wedging of the inferior group of vertebrae could be important in estimating the most distal vertebral segment to be restrained by bracing or to be fused in surgery. Largest vertebral body wedging values obtained in the sagittal plane support the claim

  8. Idiopathic scoliosis.

    PubMed

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

    Scoliosis refers to curves exceeding 10 degrees observed through posterioanterior direct radiography. In fact, the diagnosis for idiopathic scoliosis is accepted to exclude already available causes. The aim of this paper was to review the etiopathogenesis, classification systems and the treatment management of idiopathic scoliosis. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' was performed. For the literature review, papers concerning the etiopathogenesis, classification and treatment were selected among these articles. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' yielded 4518 articles published between 1947 and 2013. The main hypothesis put forward included genetic factors, hormonal factors, bone and connective tissue anomalies. King, Lenke, Coonrad and Peking Union Medical College (PUMC) classifications were the main classification systems for idiopathic scoliosis. Exercise, bracing and anterior, posterior or combined surgery when indicated are the choices for the treatment. Every idiopathic scoliosis case has to be managed to its own characteristics. It is the post-operative appearance that the surgeons are perhaps the least interested but the adolescent patients the most interested in. The aim of scoliosis surgery is to restore the spine without neurological deficit. PMID:25269032

  9. Idiopathic hypersomnia

    MedlinePlus

    Hypersomnia - idiopathic; Drowsiness - idiopathic; Somnolence - idiopathic ... extremely sleepy. It is different from narcolepsy because idiopathic hypersomnia does not usually involve suddenly falling asleep (sleep ...

  10. Complementary and alternative medicine use in adolescents with inflammatory bowel disease and juvenile idiopathic arthritis

    PubMed Central

    2014-01-01

    Background The use of complementary alternative medicine (CAM) is potentially prevalent among paediatric patients with chronic diseases but with variable rates among different age groups, diseases and countries. There are no recent reports on CAM use among paediatric patients with inflammatory bowel disease (IBD) and juvenile idiopathic arthritis (JIA) in Europe. We hypothesized that CAM use associates with a more severe disease in paediatric IBD and JIA. Methods A cross-sectional questionnaire study among adolescent outpatients with IBD and JIA addressing the frequency and type of CAM use during the past year. The patients were recruited at the Children’s Hospital, University of Helsinki, Finland. Results Of the 147 respondents, 97 had IBD (Crohn’s disease: n = 46; median age 15.5, disease duration 3.4 years) and 50 had JIA (median age 13.8, disease duration 6.9 years). During the past 12 months, 48% regularly used CAM while 81% reported occasional CAM use. Compared to patients with JIA, the use of CAM in IBD patients tended to be more frequent. The most commonly used CAM included probiotics, multivitamins, and mineral and trace element supplements. Self-imposed dietary restrictions were common, involving 27.6% of the non-CAM users but 64.8% of all CAM users. Disease activity was associated with CAM use in JIA but not in IBD. Conclusions CAM use is frequent among adolescents with IBD and JIA and associates with self-imposed dietary restrictions. Reassuringly, adherence to disease modifying drugs is good in young CAM users. In JIA, patients with active disease used more frequently CAM than patients with inactive disease. As CAM use is frequent, physicians should familiarise themselves with the basic concepts of CAM. The potential pharmacological interaction or the toxicity of certain CAM products warrants awareness and hence physicians should actively ask their patients about CAM use. PMID:24708564

  11. Adolescent idiopathic scoliosis (AIS): a multifactorial cascade concept for pathogenesis and embryonic origin.

    PubMed

    Burwell, R Geoffrey; Clark, Emma M; Dangerfield, Peter H; Moulton, Alan

    2016-01-01

    This paper formulates a novel multifactorial Cascade Concept for the pathogenesis of adolescent idiopathic scoliosis (AIS). This Concept stems from the longitudinal findings of Clark et al. (J Bone Miner Res 29(8):1729-36, 2014) who identified leptin body composition factors at 10 years of age associated with a scoliosis deformity found at 15 years. We interpret these findings in the light of some concepts for AIS pathogenesis. In particular, we speculate that the leptin body composition effect is linked to central nervous system development and the initiation of the asynchronous neuro-osseous growth mechanism that involves the creation of a neuraxis tether of relative anterior vertebral overgrowth. The latter mechanism in combination with age and gender-related anatomical variants of vertebral backward tilt (dorsal shear concept), human upright posture, adolescent growth factors, Hueter-Volkmann effect in vertebrae and vertebral bone mass abnormalities, lead to AIS, possibly both initiation and progression of scoliosis curvatures. Being multifactorial, while the Cascade Concept cannot be tested for all its components, some components should be testable by the method of numerical simulation. Clark et al. (J Bone Miner Res 29(8):1729-36, 2014) also suggested the origin of scoliosis was in the embryonic stages of life from cell types, including adipocytes and osteoblasts, derived from the same progenitor cells, and myoblasts from mesodermal somites. The involvement of cell types from different developmental origins suggests a process acting in embryonic life at a similar time, probably environmental, as previously proposed from anthropometric studies. As a Complex disease, AIS will involve genetic, environmental and life style factors operating in development and growth; this possibility needs evaluating in epidemiological studies. PMID:27252984

  12. Forces exerted during exercises by patients with adolescent idiopathic scoliosis wearing fiberglass braces

    PubMed Central

    Romano, Michele; Carabalona, Roberta; Petrilli, Silvia; Sibilla, Paolo; Negrini, Stefano

    2006-01-01

    Objective To quantify and compare the forces exerted by scoliosis patients in fiberglass braces during exercises usually prescribed in departments where casts are made. The exercises are intended to increase corrective forces, activate muscles, stimulate ventilation and help the patient psychologically. Setting Outpatient care. Patients 17 consecutive adolescent patients wearing fiberglass brace for idiopathic scoliosis. Interventions Exercises (kyphotization, rotation, "escape from the pad") in different positions (sitting, supine, on all fours). Main outcome measure Pressure detected by the F-Socket System between the rib hump and the pad of the brace. Results In static and dynamic conditions, the position adopted did not alter the total pressure exerted by the brace, although the part of the sensor stimulated did vary. Kyphotization and rotation exercises produced a significant increase of pressure (+ 58.9% and +29.8%, respectively); however, the "escape from the pad" exercise, despite its name, did not produce any significant variation of pressure. Conclusion Exercises in the brace allow adjunctive forces to be applied on soft tissues and through them, presumably on the spine. Different exercises can be chosen to obtain different actions. Physical exercises and sporting activities are useful in mechanical terms, although other important actions should not be overlooked. PMID:16859544

  13. Intraoperative Computed Tomography versus Perdriolle and Scoliometer Evaluation of Spine Rotation in Adolescent Idiopathic Scoliosis

    PubMed Central

    Pankowski, Rafal; Wałejko, Szymon; Rocławski, Marek; Ceynowa, Marcin; Mazurek, Tomasz

    2015-01-01

    Numerous indirect methods for apical vertebral rotation (AVR) measurement have been reported and none of them seems to be as accurate as computed tomography evaluation. The aim of this study was to compare spinal rotation changes during innovative technique of intraoperative computed tomography (ICT) evaluation with indirect methods such as Perdriolle and clinical evaluation with scoliometer. We examined 42 adolescent idiopathic scoliosis (AIS) patients treated with posterior scoliosis surgery (PSS). The mean age at the time of surgery was 16 years. ICT evaluation was performed before and after scoliosis correction in prone position. Clinical rib hump measure with scoliometer and radiographic Perdriolle were performed before and after surgery. There was 71,5% of average rib hump correction with scoliometer but only 31% of correction with ICT (P=0,026) and there was no significant correlation between them (R=0,297, p=0,26). Mean postcorrectional Perdriolle AVR had a decrease of 16,5°. The average ICT AVR had a decrease of only 1,2° (P=0,003). There was no significant statistic correlation between ICT and Perdriolle AVR evaluation (R=0,297, p=0,2). There is a significant discrepancy in AVR and rib hump assessment between scoliometer and Perdriolle methods and ICT evaluation, which seems to be the most accurate tool for spinal derotation measurement. PMID:25861628

  14. Intraoperative computed tomography versus Perdriolle and scoliometer evaluation of spine rotation in adolescent idiopathic scoliosis.

    PubMed

    Pankowski, Rafal; Wałejko, Szymon; Rocławski, Marek; Ceynowa, Marcin; Mazurek, Tomasz

    2015-01-01

    Numerous indirect methods for apical vertebral rotation (AVR) measurement have been reported and none of them seems to be as accurate as computed tomography evaluation. The aim of this study was to compare spinal rotation changes during innovative technique of intraoperative computed tomography (ICT) evaluation with indirect methods such as Perdriolle and clinical evaluation with scoliometer. We examined 42 adolescent idiopathic scoliosis (AIS) patients treated with posterior scoliosis surgery (PSS). The mean age at the time of surgery was 16 years. ICT evaluation was performed before and after scoliosis correction in prone position. Clinical rib hump measure with scoliometer and radiographic Perdriolle were performed before and after surgery. There was 71,5% of average rib hump correction with scoliometer but only 31% of correction with ICT (P=0,026) and there was no significant correlation between them (R=0,297, p=0,26). Mean postcorrectional Perdriolle AVR had a decrease of 16,5°. The average ICT AVR had a decrease of only 1,2° (P=0,003). There was no significant statistic correlation between ICT and Perdriolle AVR evaluation (R=0,297, p=0,2). There is a significant discrepancy in AVR and rib hump assessment between scoliometer and Perdriolle methods and ICT evaluation, which seems to be the most accurate tool for spinal derotation measurement. PMID:25861628

  15. A Functional SNP in BNC2 Is Associated with Adolescent Idiopathic Scoliosis

    PubMed Central

    Ogura, Yoji; Kou, Ikuyo; Miura, Shigenori; Takahashi, Atsushi; Xu, Leilei; Takeda, Kazuki; Takahashi, Yohei; Kono, Katsuki; Kawakami, Noriaki; Uno, Koki; Ito, Manabu; Minami, Shohei; Yonezawa, Ikuho; Yanagida, Haruhisa; Taneichi, Hiroshi; Zhu, Zezhang; Tsuji, Taichi; Suzuki, Teppei; Sudo, Hideki; Kotani, Toshiaki; Watanabe, Kota; Hosogane, Naobumi; Okada, Eijiro; Iida, Aritoshi; Nakajima, Masahiro; Sudo, Akihiro; Chiba, Kazuhiro; Hiraki, Yuji; Toyama, Yoshiaki; Qiu, Yong; Shukunami, Chisa; Kamatani, Yoichiro; Kubo, Michiaki; Matsumoto, Morio; Ikegawa, Shiro

    2015-01-01

    Adolescent idiopathic scoliosis (AIS) is the most common spinal deformity. We previously conducted a genome-wide association study (GWAS) and detected two loci associated with AIS. To identify additional loci, we extended our GWAS by increasing the number of cohorts (2,109 affected subjects and 11,140 control subjects in total) and conducting a whole-genome imputation. Through the extended GWAS and replication studies using independent Japanese and Chinese populations, we identified a susceptibility locus on chromosome 9p22.2 (p = 2.46 × 10−13; odds ratio = 1.21). The most significantly associated SNPs were in intron 3 of BNC2, which encodes a zinc finger transcription factor, basonuclin-2. Expression quantitative trait loci data suggested that the associated SNPs have the potential to regulate the BNC2 transcriptional activity and that the susceptibility alleles increase BNC2 expression. We identified a functional SNP, rs10738445 in BNC2, whose susceptibility allele showed both higher binding to a transcription factor, YY1 (yin and yang 1), and higher BNC2 enhancer activity than the non-susceptibility allele. BNC2 overexpression produced body curvature in developing zebrafish in a gene-dosage-dependent manner. Our results suggest that increased BNC2 expression is implicated in the etiology of AIS. PMID:26211971

  16. Time-to-contact and multiscale entropy identify differences in postural control in adolescent idiopathic scoliosis.

    PubMed

    Gruber, Allison H; Busa, Michael A; Gorton Iii, George E; Van Emmerik, Richard E A; Masso, Peter D; Hamill, Joseph

    2011-05-01

    Previous reports on changes in postural control in adolescent idiopathic scoliosis (AIS) compared to healthy controls have been inconsistent. This may suggest center of pressure (COP) sway parameters are not sufficient for determining the ability to maintain quiet upright stance indicating more complex measures may be needed to examine postural control in AIS. The purpose of this investigation was to compare postural control between AIS of different severity levels and healthy controls using time-to-contact (TtC), the complexity index of multiscale entropy (C(r)), and COP sway parameters. Thirty-six AIS patients were classified as pre-bracing or pre-operative and compared to 10 healthy control subjects. Overall, the AIS patients showed significantly greater COP sway in mediolateral direction, but deficits with respect to the anteroposterior direction were only systematically identified with the time-to-contact and entropy measures. The multiscale entropy (C(r)) results indicate that those with AIS utilize a different control strategy from healthy controls in the mediolateral direction that is more constrained, less complex and less adaptable. AIS severity further reduced this adaptability in the anteroposterior direction. These results indicate it is necessary to examine both planes of motion when investigating postural control in AIS. Additionally, the application of the measures used to assess the nature of the postural control changes in AIS should also be considered. PMID:21478018

  17. MR Analysis of Regional Brain Volume in Adolescent Idiopathic Scoliosis: Neurological Manifestation of a Systemic Disease

    PubMed Central

    Liu, Tianming; Chu, Winnie C.W.; Young, Geoffrey; Li, Kaiming; Yeung, Benson H.Y.; Guo, Lei; Man, Gene C.W.; Lam, Wynnie W.M.; Wong, Stephen T.C.; Cheng, Jack C.Y.

    2008-01-01

    Purpose To investigate whether regional brain volumes in adolescent idiopathic scoliosis (AIS) patients differ from matched control subjects as AIS subjects are reported to have poor performance on combined visual and proprioceptive testing and impaired postural balance in previous studies. Materials and Methods Twenty AIS female patients with typical right-convex thoracic curve (age range,11−18 years; mean, 14.1 years) and 26 female controls (mean age, 14.8 years) underwent three-dimensional magnetization prepared rapid acquisition gradient echo (3D-MPRAGE) MR imaging. Volumes of 99 preselected neuroanatomical regions were compared by statistical parametric mapping and atlas-based hybrid warping. Results Analysis of variance statistics revealed significant mean volumetric differences in 22 brain regions between AIS and controls. Ten regions were larger in AIS including the left frontal gyri and white matter in left frontal, parietal, and temporal regions, corpus callosum and brainstem. Twelve regions were smaller in AIS, including right-sided descending white matter tracts (anterior and posterior limbs of the right internal capsule and the cerebral peduncle) and deep nucleus (caudate), bilateral perirhinal cortices, left hippocampus and amygdala, bilateral precuneus gyri, and left middle and inferior occipital gyri. Conclusion Regional brain volume difference in AIS subjects may help to explain neurological abnormalities in this group. PMID:18302230

  18. Genome-wide association study identifies new susceptibility loci for adolescent idiopathic scoliosis in Chinese girls

    PubMed Central

    Zhu, Zezhang; Tang, Nelson Leung-Sang; Xu, Leilei; Qin, Xiaodong; Mao, Saihu; Song, Yueming; Liu, Limin; Li, Fangcai; Liu, Peng; Yi, Long; Chang, Jiang; Jiang, Long; Ng, Bobby Kin-Wah; Shi, Benlong; Zhang, Wen; Qiao, Jun; Sun, Xu; Qiu, Xusheng; Wang, Zhou; Wang, Fei; Xie, Dingding; Chen, Ling; Chen, Zhonghui; Jin, Mengran; Han, Xiao; Hu, Zongshan; Zhang, Zhen; Liu, Zhen; Zhu, Feng; Qian, Bang-ping; Yu, Yang; Wang, Bing; Lee, K. M.; Lee, Wayne Y.W.; Lam, T. P.; Qiu, Yong; Cheng, Jack Chun-Yiu

    2015-01-01

    Adolescent idiopathic scoliosis (AIS) is a structural deformity of the spine affecting millions of children. As a complex disease, the genetic aetiology of AIS remains obscure. Here we report the results of a four-stage genome-wide association study (GWAS) conducted in a sample of 4,317 AIS patients and 6,016 controls. Overall, we identify three new susceptibility loci at 1p36.32 near AJAP1 (rs241215, Pcombined=2.95 × 10−9), 2q36.1 between PAX3 and EPHA4 (rs13398147, Pcombined=7.59 × 10−13) and 18q21.33 near BCL-2 (rs4940576, Pcombined=2.22 × 10−12). In addition, we refine a previously reported region associated with AIS at 10q24.32 (rs678741, Pcombined=9.68 × 10−37), which suggests LBX1AS1, encoding an antisense transcript of LBX1, might be a functional variant of AIS. This is the first GWAS investigating genetic variants associated with AIS in Chinese population, and the findings provide new insight into the multiple aetiological mechanisms of AIS. PMID:26394188

  19. Validity Study of Vertebral Rotation Measurement Using 3-D Ultrasound in Adolescent Idiopathic Scoliosis.

    PubMed

    Wang, Qian; Li, Meng; Lou, Edmond H M; Chu, Winnie C W; Lam, Tsz-Ping; Cheng, Jack C Y; Wong, Man-Sang

    2016-07-01

    This study aimed to assess the validity of 3-D ultrasound measurements on the vertebral rotation of adolescent idiopathic scoliosis (AIS) under clinical settings. Thirty curves (mean Cobb angle: 21.7° ± 15.9°) from 16 patients with AIS were recruited. 3-D ultrasound and magnetic resonance imaging scans were performed at the supine position. Each of the two raters measured the apical vertebral rotation using the center of laminae (COL) method in the 3-D ultrasound images and the Aaro-Dahlborn method in the magnetic resonance images. The intra- and inter-reliability of the COL method was demonstrated by the intra-class correlation coefficient (ICC) (both [2, K] >0.9, p < 0.05). The COL method showed no significant difference (p < 0.05) compared with the Aaro-Dahlborn method. Furthermore, the agreement between these two methods was demonstrated by the Bland-Altman method, and high correlation was found (r > 0.9, p < 0.05). These results validated the proposed 3-D ultrasound method in the measurements of vertebral rotation in the patients with AIS. PMID:27083978

  20. A neglected point in surgical treatment of adolescent idiopathic scoliosis: Variations in the number of vertebrae.

    PubMed

    Hu, Zongshan; Zhang, Zhen; Zhao, Zhihui; Zhu, Zezhang; Liu, Zhen; Qiu, Yong

    2016-08-01

    Inaccurate identification of vertebral levels is the main cause of wrong-site spine surgery which is performed by nearly half of the spine surgeons. Unusual anatomy and failure to verify the surgical level on radiographs have been commonly reported. We aimed at investigating the variations in vertebral number in adolescent idiopathic scoliosis (AIS) patients and thus to raise awareness of the possibility for wrong-level spinal surgery and to make a comparison with normal adolescents. A cohort of 657 AIS patients and 248 normal adolescents, presented to our center from June 2008 to February 2013, who met the inclusion criteria, were recruited. Radiographs were reviewed to identify the number of thoracic or lumbar vertebrae and the presence of a lumbosacral transitional vertebra. In the AIS group, 70 (10.6%) patients had variations in the number of thoracic and/or lumbar vertebrae. Remarkably, the prevalence of variations in male subjects was significantly higher than that in female subjects (P < 0.05). Thirty-seven patients (5.6%) had an atypical number of thoracic vertebrae, with 33 having 11 thoracic vertebrae and 4 patients having 13. Forty-eight patients (7.3%) had an atypical number of lumbar vertebrae, with 14 having 4 lumbar vertebrae and 34 patients having 6. Multilevel vertebral anomalies were present in 2.3% of the patients (15 of 657). A variation in the number of vertebrae had been identified in 1.7% (11) of the reports by the radiologist. In the normal group, 27 (10.9%) subjects showed variations in the vertebral number. There was no significant difference in the prevalence of atypical numbers of vertebral number between the AIS and normal groups (P > 0.05). Therefore, we concluded that variations in the number of thoracic-lumbar vertebrae were found in up to10.6% of AIS patients. Identification of variations in the number of vertebrae is crucial to serve to decrease the risk of wrong-level surgery. PMID:27559975

  1. Physical activity, functional ability, and disease activity in children and adolescents with juvenile idiopathic arthritis.

    PubMed

    Gueddari, S; Amine, B; Rostom, S; Badri, D; Mawani, N; Ezzahri, M; Moussa, F; Shyen, S; Abouqal, R; Chkirat, B; Hajjaj-Hassouni, N

    2014-09-01

    Juvenile idiopathic arthritis (JIA) is a chronic condition known to cause pain-related complications in youth and affect children's physical functioning. There is no data in Arabic children with JIA about the impact of illness upon their physical activity. The objective of this study was to explore physical activity (PA) in children and adolescents with JIA compared with a healthy population and to examine associations between PA, functional ability, and disease activity. Our study included patients with JIA and group control aged between 8 and 17 years. The diagnosis was used according to the International League of Association of Rheumatology (ILAR) criteria 2001. Sociodemographic data and clinical features were collected. Physical activity level and energy expenditure were assessed with a 1-day activity diary and the metabolic equivalent (MET), respectively. Functional ability was assessed with the Moroccan version of the Childhood Health Assessment Questionnaire (CHAQ). Disease activity was measured using the Juvenile Arthritis Disease Activity Score (JADAS). Fifty patients and 50 controls were included (mean ± SD age 11.5 ± 3.3 and 10.5 ± 3.8 years, respectively; p = 0.49) with masculine predominance n = 30 (59.6 %) and n = 29 (58 %), respectively (p = 0.26). The median disease duration was 4.3 years (2-5). The median analog scale (VAS) pain was 20 (10-40). Fourteen patients (28 %) had an active disease. Patient population consisted in majority of oligoarticular arthritis (28 %), 14 patients. The mean of energy expenditure and physical activity were significantly higher in the JIA group. The JIA group spent more time in bed and less time on moderate to vigorous PA than the control group. There is no significant relationship between PA, functional ability, and disease activity. Our study suggests that children and adolescents with JIA have low PA levels and are at risk of losing the benefits of PA. Low PA is not related to

  2. Preoperative Factors Predicting Intraoperative Blood Loss in Female Patients With Adolescent Idiopathic Scoliosis

    PubMed Central

    Li, Chao; Yang, Mingyuan; Wang, Chao; Wang, Chuanfeng; Fan, Jianping; Chen, Ziqiang; Wei, Xianzhao; Zhang, Guoyou; Bai, Yushu; Zhu, Xiaodong; Xie, Yang; Li, Ming

    2015-01-01

    Abstract In this article, a retrospective analysis of 161 female patients with adolescent idiopathic scoliosis (AIS) is performed who underwent posterior correction and fusion using all-pedicle screw instrument. The aim of this article is to find out preoperative factors that influence intraoperative blood loss (IOBL) in female patients with AIS. The IOBL in posterior correction and fusion surgery for patients with idiopathic scoliosis greatly varies. The variables affecting the IOBL also greatly vary among different studies. Medical records of all female patients with AIS who underwent posterior correction and fusion operations using the all-pedicle screw system in our hospital from January 2012 to January 2014 were reviewed. Patients with irregular menstruation, who underwent osteotomy, and using coagulants were excluded. Preoperative clinical data, including patient age, height, weight, Risser sign, day after last menstruation, major curve Cobb angle, fulcrum-bending Cobb angle, curve flexibility index, sagittal thoracic Cobb angle, sagittal lumbar Cobb angle, albumin, hemoglobin, platelet, activated partial thromboplastic time (APTT), prothrombin time, thrombin time, fibrinogen, fusion level, menstrual phase, and blood type, were collected. Data were further analyzed using multiple linear regression with forward elimination. A total of 161 patients were included in this study. The mean IOBL was 933.98 ± 158.10 mL (500–2000 mL). Forward selection showed that fulcrum-bending Cobb angle, fusion level, Risser sign, APTT, fibrinogen, and menstrual phase were the preoperative factors that influenced the IOBL in female patients with AIS. Equation of IOBL was built by multiple linear regression: IOBL = −966.228 + 54.738 Risser sign + 18.910 fulcrum-bending Cobb angle + 114.737 fibrinogen + 21.386 APTT − 71.312 team 2 − 177.985 team 3 − 165.082 team 4 + 53.470 fusion level. R = 0.782. Operation for patients with AIS was featured by large IOBL

  3. Anterior Overgrowth in Primary Curves, Compensatory Curves and Junctional Segments in Adolescent Idiopathic Scoliosis

    PubMed Central

    van Stralen, Marijn; Chu, Winnie C. W.; Lam, Tsz-Ping; Ng, Bobby K. W.; Vincken, Koen L.; Cheng, Jack C. Y.; Castelein, René M.

    2016-01-01

    Introduction Although much attention has been given to the global three-dimensional aspect of adolescent idiopathic scoliosis (AIS), the accurate three-dimensional morphology of the primary and compensatory curves, as well as the intervening junctional segments, in the scoliotic spine has not been described before. Methods A unique series of 77 AIS patients with high-resolution CT scans of the spine, acquired for surgical planning purposes, were included and compared to 22 healthy controls. Non-idiopathic curves were excluded. Endplate segmentation and local longitudinal axis in endplate plane enabled semi-automatic geometric analysis of the complete three-dimensional morphology of the spine, taking inter-vertebral rotation, intra-vertebral torsion and coronal and sagittal tilt into account. Intraclass correlation coefficients for interobserver reliability were 0.98–1.00. Coronal deviation, axial rotation and the exact length discrepancies in the reconstructed sagittal plane, as defined per vertebra and disc, were analyzed for each primary and compensatory curve as well as for the junctional segments in-between. Results The anterior-posterior difference of spinal length, based on “true” anterior and posterior points on endplates, was +3.8% for thoracic and +9.4% for (thoraco)lumbar curves, while the junctional segments were almost straight. This differed significantly from control group thoracic kyphosis (-4.1%; P<0.001) and lumbar lordosis (+7.8%; P<0.001). For all primary as well as compensatory curves, we observed linear correlations between the coronal Cobb angle, axial rotation and the anterior-posterior length difference (r≥0.729 for thoracic curves; r≥0.485 for (thoraco)lumbar curves). Conclusions Excess anterior length of the spine in AIS has been described as a generalized growth disturbance, causing relative anterior spinal overgrowth. This study is the first to demonstrate that this anterior overgrowth is not a generalized phenomenon. It is

  4. Transforming Growth Factor-Beta (TGF-β) Signaling in Paravertebral Muscles in Juvenile and Adolescent Idiopathic Scoliosis

    PubMed Central

    Kwiecien, Magdalena

    2014-01-01

    Most researchers agree that idiopathic scoliosis (IS) is a multifactorial disease influenced by complex genetic and environmental factors. The onset of the spinal deformity that determines the natural course of the disease, usually occurs in the juvenile or adolescent period. Transforming growth factors β (TGF-βs) and their receptors, TGFBRs, may be considered as candidate genes related to IS susceptibility and natural history. This study explores the transcriptional profile of TGF-βs, TGFBRs, and TGF-β responsive genes in the paravertebral muscles of patients with juvenile and adolescent idiopathic scoliosis (JIS and AIS, resp.). Muscle specimens were harvested intraoperatively and grouped according to the side of the curve and the age of scoliosis onset. The results of microarray and qRT-PCR analysis confirmed significantly higher transcript abundances of TGF-β2, TGF-β3, and TGFBR2 in samples from the curve concavity of AIS patients, suggesting a difference in TGF-β signaling in the pathogenesis of juvenile and adolescent curves. Analysis of TGF-β responsive genes in the transcriptomes of patients with AIS suggested overrepresentation of the genes localized in the extracellular region of curve concavity: LTBP3, LTBP4, ITGB4, and ITGB5. This finding suggests the extracellular region of paravertebral muscles as an interesting target for future molecular research into AIS pathogenesis. PMID:25313366

  5. Usability Testing of an Online Self-management Program for Adolescents With Juvenile Idiopathic Arthritis

    PubMed Central

    McGrath, Patrick; Hodnett, Ellen; Feldman, Brian; Duffy, Ciaran; Huber, Adam; Tucker, Lori; Hetherington, Ross; Tse, Shirley; Spiegel, Lynn; Campillo, Sarah; Gill, Navreet; White, Meghan

    2010-01-01

    Background A new bilingual (English and French) Internet-based self-management program, Teens Taking Charge: Managing Arthritis Online, for adolescents with arthritis and their parents was developed following a needs assessment. Objectives This study explored the usability (user performance and satisfaction) of the self-management program for youth with juvenile idiopathic arthritis (JIA) and their parents to refine the health portal prototype. Methods A qualitative study design with semi-structured, audio taped interviews and observation by a trained observer was undertaken with two iterative cycles to determine the usability (ease of use, efficiency, errors, and user satisfaction) of the user interface and content areas of the intervention. A purposive sample of English-speaking (n = 11; mean age = 15.4, standard deviation [SD] 1.7) and French-speaking (n = 8; mean age = 16.0, SD 1.2) adolescents with JIA and one of their respective parents/caregivers were recruited from 2 Canadian tertiary care centers. Descriptive statistics and simple content analyses were used to organize data into categories that reflected the emerging usability themes. Results All of the participants had access to a computer/Internet at home; however, adolescents were more comfortable using the computer/Internet than their parents. Adolescents and parents provided similar as well as differing suggestions on how the website user interface could be improved in terms of its usability (navigation; presentation and control usage errors; format and layout; as well as areas for further content development). There were no major differences in usability issues between English- and French-speaking participants. Minor changes to the website user interface were made and tested in a second cycle of participants. No further usability problems were identified in the second iterative cycle of testing. Teens and parents responded positively to the appearance and theme of the website (ie, promoting self

  6. Active Treatment for Idiopathic Adolescent Scoliosis (ACTIvATeS): a feasibility study.

    PubMed Central

    Williams, Mark A; Heine, Peter J; Williamson, Esther M; Toye, Francine; Dritsaki, Melina; Petrou, Stavros; Crossman, Richard; Lall, Ranjit; Barker, Karen L; Fairbank, Jeremy; Harding, Ian; Gardner, Adrian; Slowther, Anne-Marie; Coulson, Neil; Lamb, Sarah E

    2015-01-01

    BACKGROUND The feasibility of conducting a definitive randomised controlled trial (RCT) evaluating the clinical effectiveness and cost-effectiveness of scoliosis-specific exercises (SSEs) for adolescent idiopathic scoliosis (AIS) is uncertain. OBJECTIVES The aim of this study was to assess the feasibility of conducting a large, multicentre trial of SSE treatment for patients with AIS, in comparison with standard care, and to refine elements of the study design. The objectives were to (1) update a systematic review of controlled trials evaluating the efficacy of SSE in AIS; (2) survey UK orthopaedic surgeons and physiotherapists to determine current practice, patient populations and equipoise; (3) randomise 50 adolescents to a feasibility trial of either usual care or SSE interventions across a range of sites; (4) develop, document and assess acceptability and adherence of interventions; (5) assess and describe training requirements of physiotherapists; and (6) gain user input in all relevant stages of treatment and protocol design. DESIGN Multicomponent feasibility study including UK clinician survey, systematic literature review and a randomised feasibility trial. SETTING The randomised feasibility study involved four secondary care NHS trusts providing specialist care for patients with AIS. PARTICIPANTS The randomised feasibility study recruited people aged 10-16 years with mild AIS (Cobb angle of < 50°). INTERVENTIONS The randomised study allocated participants to standard practice of advice and education or a physiotherapy SSE programme supported by a home exercise plan. Our choice of intervention was informed by a systematic review of exercise interventions for AIS. MAIN OUTCOME MEASURES The main outcome was feasibility of recruitment to the randomised study. Other elements were to inform choice of outcomes for a definitive trial and included curve severity, quality of life, requirement for surgery/brace, adverse events, psychological symptoms, costs and

  7. Leptin Receptor Metabolism Disorder in Primary Chondrocytes from Adolescent Idiopathic Scoliosis Girls

    PubMed Central

    Wang, Yun-Jia; Yu, Hong-Gui; Zhou, Zhen-Hai; Guo, Qiang; Wang, Long-Jie; Zhang, Hong-Qi

    2016-01-01

    To investigate the underlying mechanisms of low metabolic activity of primary chondrocytes obtained from girls with adolescent idiopathic scoliosis (AIS); AIS is a spine-deforming disease that often occurs in girls. AIS is associated with a lower bone mass than that of healthy individuals and osteopenia. Leptin was shown to play an important role in bone growth. It can also regulate the function of chondrocytes. Changes in leptin and Ob-R levels in AIS patients have been reported in several studies. The underlying mechanisms between the dysfunction of peripheral leptin signaling and abnormal chondrocytes remain unclear; The following parameters were evaluated in AIS patients and the control groups: total serum leptin levels; Ob-R expression in the plasma membrane of primary chondrocytes; JAK2 and STAT3 phosphorylation status. Then, we inhibited the lysosome and proteasome and knocked down clathrin heavy chain (CHC) expression in primary chondrocytes isolated from girls with AIS and evaluated Ob-R expression. We investigated the effects of leptin combined with a lysosome inhibitor or CHC knockdown in primary chondrocytes obtained from AIS patients; Compared with the controls, AIS patients showed similar total serum leptin levels, reduced JAK2 and STAT3 phosphorylation, and decreased cartilage matrix synthesis in the facet joint. Lower metabolic activity and lower membrane expression of Ob-R were observed in primary chondrocytes from the AIS group than in the controls. Lysosome inhibition increased the total Ob-R content but had no effect on the membrane expression of Ob-R or leptin’s effects on AIS primary chondrocytes. CHC knockdown upregulated the membrane Ob-R levels and enhanced leptin’s effects on AIS primary chondrocytes; The underlying mechanism of chondrocytes that are hyposensitive to leptin in some girls with AIS is low plasma membrane Ob-R expression that results from an imbalance between the rate of receptor endocytosis and the insertion of newly

  8. Effects of Exercise on Spinal Deformities and Quality of Life in Patients with Adolescent Idiopathic Scoliosis

    PubMed Central

    Anwer, Shahnawaz; Alghadir, Ahmad; Abu Shaphe, Md.; Anwar, Dilshad

    2015-01-01

    Objectives. This systematic review was conducted to examine the effects of exercise on spinal deformities and quality of life in patients with adolescent idiopathic scoliosis (AIS). Data Sources. Electronic databases, including PubMed, CINAHL, Embase, Scopus, Cochrane Register of Controlled Trials, PEDro, and Web of Science, were searched for research articles published from the earliest available dates up to May 31, 2015, using the key words “exercise,” “postural correction,” “posture,” “postural curve,” “Cobb's angle,” “quality of life,” and “spinal deformities,” combined with the Medical Subject Heading “scoliosis.” Study Selection. This systematic review was restricted to randomized and nonrandomized controlled trials on AIS published in English language. The quality of selected studies was assessed by the PEDro scale, the Cochrane Collaboration's tool, and the Grading of Recommendations Assessment, Development, and Evaluation System (GRADE). Data Extraction. Descriptive data were collected from each study. The outcome measures of interest were Cobb angle, trunk rotation, thoracic kyphosis, lumbar kyphosis, vertebral rotation, and quality of life. Data Synthesis. A total of 30 studies were assessed for eligibility. Six of the 9 selected studies reached high methodological quality on the PEDro scale. Meta-analysis revealed moderate-quality evidence that exercise interventions reduce the Cobb angle, angle of trunk rotation, thoracic kyphosis, and lumbar lordosis and low-quality evidence that exercise interventions reduce average lateral deviation. Meta-analysis revealed moderate-quality evidence that exercise interventions improve the quality of life. Conclusions. A supervised exercise program was superior to controls in reducing spinal deformities and improving the quality of life in patients with AIS. PMID:26583083

  9. Video-Assisted Thoracoscopic Surgery Plus Lumbar Mini-Open Surgery for Adolescent Idiopathic Scoliosis

    PubMed Central

    Chong, Hyon Su; Kim, Hak Sun; Ankur, Nanda; Kho, Phillip Anthony; Kim, Sung Jun; Kim, Do Yeon; Park, Jin Oh; Moon, Seong Hwan; Lee, Hwan Mo

    2011-01-01

    Purpose The objectives of this study are to describe the outcome of adolescent idiopathic scoliosis (AIS) patients treated with Video Assisted Thoracoscopic Surgery (VATS) plus supplementary minimal incision in the lumbar region for thoracic and lumbar deformity correction and fusion. Materials and Methods This is a case series of 13 patients treated with VATS plus lumbar mini-open surgery for AIS. A total of 13 patients requiring fusions of both the thoracic and lumbar regions were included in this study: 5 of these patients were classified as Lenke type 1A and 8 as Lenke type 5C. Fusion was performed using VATS up to T12 or L1 vertebral level. Lower levels were accessed via a small mini-incision in the lumbar area to gain access to the lumbar spine via the retroperitoneal space. All patients had a minimum follow-up of 1 year. Results The average number of fused vertebrae was 7.1 levels. A significant correction in the Cobb angle was obtained at the final follow-up (p = 0.001). The instrumented segmental angle in the sagittal plane was relatively well-maintained following surgery, albeit with a slight increase. Scoliosis Research Society-22 (SRS-22) scores were noted have significantly improved at the final follow-up (p < 0.05). Conclusion Indications for the use of VATS may be extended from patients with localized thoracic scoliosis to those with thoracolumbar scoliosis. By utilizing a supplementary minimal incision in the lumbar region, a satisfactory deformity correction may be accomplished with minimal post-operative scarring. PMID:21155045

  10. Genetic Linkage Localizes an Adolescent Idiopathic Scoliosis and Pectus Excavatum Gene to the 18q Chromosome

    PubMed Central

    Gurnett, Christina A; Alaee, Farhang; Bowcock, Anne; Kruse, Lisa; Lenke, Lawrence G.; Bridwell, Keith H.; Kuklo, Timothy; Luhmann, Scott J.; Dobbs, Matthew B.

    2009-01-01

    Study Design A single large family, in which adolescent idiopathic scoliosis (AIS) and pectus excavatum (PE) segregate as an autosomal dominant condition, was evaluated. Genome-wide linkage analysis and candidate gene sequencing were performed. Objective To map the disease-causing locus in a large Caucasian family in which AIS and PE co-segregate. Summary of Background Data AIS and PE are common musculoskeletal conditions known to have a genetic component, though few genes have been identified for either. Genetic studies have been confounded by a lack of large families in which the disorders segregate. Methods Clinical examinations were performed on the proband, who underwent posterior spinal fusion, and twelve additional affected family members. To map a gene causing AIS and PE, a genome-wide linkage analysis was performed with the Affymetrix Mapping 10K XbaI array on thirteen affected and ten unaffected family members. Candidate genes were sequenced. Results AIS was present in thirteen female family members and PE was present in three males and one female. Genome-wide linkage analysis resulted in a linkage peak on chromosome 18q with a maximum parametric multipoint logarithm of the odds (LOD) score of 3.86. Recombinants delineated the critical genetic region to an interval of 6.4 cM between SNP_A-1519369 and SNP_A-1507702, corresponding to a 7.06 Mb region (hg18: chr18:26342508-34395660). The chromosome 18q linkage region contains more than 30 genes. Re-sequencing of the coding regions of 21 candidate genes in the region did not reveal any causative mutation. Conclusion Linkage analysis in this large family demonstrated a novel locus for AIS and PE on chromosome 18q. Because of the increased frequency of PE in family members of AIS patients, consideration of family members with PE as affected may increase the power of AIS genetic linkage studies. PMID:19139660

  11. A polygenic burden of rare variants across extracellular matrix genes among individuals with adolescent idiopathic scoliosis.

    PubMed

    Haller, Gabe; Alvarado, David; Mccall, Kevin; Yang, Ping; Cruchaga, Carlos; Harms, Matthew; Goate, Alison; Willing, Marcia; Morcuende, Jose A; Baschal, Erin; Miller, Nancy H; Wise, Carol; Dobbs, Matthew B; Gurnett, Christina A

    2016-01-01

    Adolescent idiopathic scoliosis (AIS) is a complex inherited spinal deformity whose etiology has been elusive. While common genetic variants are associated with AIS, they explain only a small portion of disease risk. To explore the role of rare variants in AIS susceptibility, exome sequence data of 391 severe AIS cases and 843 controls of European ancestry were analyzed using a pathway burden analysis in which variants are first collapsed at the gene level then by Gene Ontology terms. Novel non-synonymous/splice-site variants in extracellular matrix genes were significantly enriched in AIS cases compared with controls (P = 6 × 10(-9), OR = 1.7, CI = 1.4-2.0). Specifically, novel variants in musculoskeletal collagen genes were present in 32% (126/391) of AIS cases compared with 17% (146/843) of in-house controls and 18% (780/4300) of EVS controls (P = 1 × 10(-9), OR = 1.9, CI = 1.6-2.4). Targeted resequencing of six collagen genes replicated this association in combined 919 AIS cases (P = 3 × 10(-12), OR = 2.2, CI = 1.8-2.7) and revealed a highly significant single-gene association with COL11A2 (P = 6 × 10(-9), OR = 3.8, CI = 2.6-7.2). Importantly, AIS cases harbor mainly non-glycine missense mutations and lack the clinical features of monogenic musculoskeletal collagenopathies. Overall, our study reveals a complex genetic architecture of AIS in which a polygenic burden of rare variants across extracellular matrix genes contributes strongly to risk. PMID:26566670

  12. Rare variants in FBN1 and FBN2 are associated with severe adolescent idiopathic scoliosis

    PubMed Central

    Buchan, Jillian G.; Alvarado, David M.; Haller, Gabe E.; Cruchaga, Carlos; Harms, Matthew B.; Zhang, Tianxiao; Willing, Marcia C.; Grange, Dorothy K.; Braverman, Alan C.; Miller, Nancy H.; Morcuende, Jose A.; Tang, Nelson Leung-Sang; Lam, Tsz-Ping; Ng, Bobby Kin-Wah; Cheng, Jack Chun-Yiu; Dobbs, Matthew B.; Gurnett, Christina A.

    2014-01-01

    Adolescent idiopathic scoliosis (AIS) causes spinal deformity in 3% of children. Despite a strong genetic basis, few genes have been associated with AIS and the pathogenesis remains poorly understood. In a genome-wide rare variant burden analysis using exome sequence data, we identified fibrillin-1 (FBN1) as the most significantly associated gene with AIS. Based on these results, FBN1 and a related gene, fibrillin-2 (FBN2), were sequenced in a total of 852 AIS cases and 669 controls. In individuals of European ancestry, rare variants in FBN1 and FBN2 were enriched in severely affected AIS cases (7.6%) compared with in-house controls (2.4%) (OR = 3.5, P = 5.46 × 10−4) and Exome Sequencing Project controls (2.3%) (OR = 3.5, P = 1.48 × 10−6). Scoliosis severity in AIS cases was associated with FBN1 and FBN2 rare variants (P = 0.0012) and replicated in an independent Han Chinese cohort (P = 0.0376), suggesting that rare variants may be useful as predictors of curve progression. Clinical evaluations revealed that the majority of AIS cases with rare FBN1 variants do not meet diagnostic criteria for Marfan syndrome, though variants are associated with tall stature (P = 0.0035) and upregulation of the transforming growth factor beta pathway. Overall, these results expand our definition of fibrillin-related disorders to include AIS and open up new strategies for diagnosing and treating severe AIS. PMID:24833718

  13. Experience with an online prospective database on adolescent idiopathic scoliosis: development and implementation.

    PubMed

    Arlet, Vincent; Shilt, Jeffrey; Bersusky, Ernesto; Abel, Mark; Ouellet, Jean Albert; Evans, Davis; Menon, K V; Kandziora, Frank; Shen, Frank; Lamartina, Claudio; Adams, Marc; Reddi, Vasantha

    2008-11-01

    Considerable variability exists in the surgical treatment and outcomes of adolescent idiopathic scoliosis (AIS). This is due to the lack of evidence-based treatment guidelines and outcome measures. Although clinical trials have been extolled as the highest form of evidence for evaluating treatment efficacy, the disadvantage of cost, time, lack of feasibility, and ethical considerations indicate a need for a new paradigm for evidence based research in this spinal deformity. High quality clinical databases offer an alternative approach for evidence-based research in medicine. So, we developed and established Scolisoft, an international, multidimensional and relational database designed to be a repository of surgical cases for AIS, and an active vehicle for standardized surgical information in a format that would permit qualitative and quantitative research and analysis. Here, we describe and discuss the utility of Scolisoft as a new paradigm for evidence-based research on AIS. Scolisoft was developed using dot.net platform and SQL server from Microsoft. All data is deidentified to protect patient privacy. Scolisoft can be accessed at (www.scolisoft.org). Collection of high quality data on surgical cases of AIS is a priority and processes continue to improve the database quality. The database currently has 67 registered users from 21 countries. To date, Scolisoft has 200 detailed surgical cases with pre, post, and follow up data. Scolisoft provides a structured process and practical information for surgeons to benchmark their treatment methods against other like treatments. Scolisoft is multifaceted and its use extends to education of health care providers in training, patients, ability to mine important data to stimulate research and quality improvement initiatives of healthcare organizations. PMID:18830720

  14. Predictors of spine deformity progression in adolescent idiopathic scoliosis: A systematic review with meta-analysis

    PubMed Central

    Noshchenko, Andriy; Hoffecker, Lilian; Lindley, Emily M; Burger, Evalina L; Cain, Christopher MJ; Patel, Vikas V; Bradford, Andrew P

    2015-01-01

    AIM: To evaluate published data on the predictors of progressive adolescent idiopathic scoliosis (AIS) in order to evaluate their efficacy and level of evidence. METHODS: Selection criteria: (1) study design: randomized controlled clinical trials, prospective cohort studies and case series, retrospective comparative and none comparative studies; (2) participants: adolescents with AIS aged from 10 to 20 years; and (3) treatment: observation, bracing, and other. Search method: Ovid MEDLINE, Embase, the Cochrane Library, PubMed and patent data bases. All years through August 2014 were included. Data were collected that showed an association between the studied characteristics and the progression of AIS or the severity of the spine deformity. Odds ratio (OR), sensitivity, specificity, positive and negative predictive values were also collected. A meta-analysis was performed to evaluate the pooled OR and predictive values, if more than 1 study presented a result. The GRADE approach was applied to evaluate the level of evidence. RESULTS: The review included 25 studies. All studies showed statistically significant or borderline association between severity or progression of AIS with the following characteristics: (1) An increase of the Cobb angle or axial rotation during brace treatment; (2) decrease of the rib-vertebral angle at the apical level of the convex side during brace treatment; (3) initial Cobb angle severity (> 25o); (4) osteopenia; (5) patient age < 13 years at diagnosis; (6) premenarche status; (7) skeletal immaturity; (8) thoracic deformity; (9) brain stem vestibular dysfunction; and (10) multiple indices combining radiographic, demographic, and physiologic characteristics. Single nucleotide polymorphisms of the following genes: (1) calmodulin 1; (2) estrogen receptor 1; (3) tryptophan hydroxylase 1; (3) insulin-like growth factor 1; (5) neurotrophin 3; (6) interleukin-17 receptor C; (7) melatonin receptor 1B, and (8) ScoliScore test. Other predictors

  15. Oral health and quality of life of children and adolescents with juvenile idiopathic arthritis according to their caregivers' perceptions.

    PubMed

    Santos, Débora; Silva, Carlos; Silva, Marlete

    2015-01-01

    The aim of this study was to assess the correlation between oral health indicators and oral health-related quality of life (OHRQoL) of children and adolescents with juvenile idiopathic arthritis (JIA) according to their caregivers' perceptions. Parents or guardians (mean age, 40.6 years; standard deviation [SD] = 10.97 years) of children and adolescents with JIA (n = 17; mean age, 9.8 years; SD = 2.86) and parents or guardians of healthy children and adolescents (n = 15; mean age, 10.7 years; SD = 2.16) filled the short form of the Brazilian Parental-Caregiver Questionnaire (SF: 13 - B-PCPQ). Dental evaluations were performed on all children. There was no significant difference in SF: 13 - B-PCPQ scores of the two groups. Children and adolescents with JIA had fewer caries in their primary dentition and more gingival bleeding after probing than those without JIA. The frequency of temporomandibular disorders was 50.0% for JIA patients and 46.7% for their healthy counterparts. There was no correlation between oral health indicators and SF: 13 - B-PCPQ scores. As perceived by caregivers, JIA did not negatively impact the well-being of their children and adolescents as related to oral health, and their OHRQoL did not correlate with oral health status. PMID:26255878

  16. Role of adalimumab in the management of children and adolescents with juvenile idiopathic arthritis and other rheumatic conditions

    PubMed Central

    Marzan, Katherine Anne B

    2012-01-01

    Treatment of children and adolescents with juvenile idiopathic arthritis and other pediatric rheumatic diseases has evolved. Where once there was only a limited arsenal of medications, with significant side effects and inadequate efficacy, today, with an increased understanding of the pathogenesis of these diseases, there is a wider variety of more targeted and effective treatments. TNF-α is a cytokine involved in a number of inflammatory pathways in pediatric rheumatic diseases. The emergence of biologic modifiers that target TNF-α has been pivotal in providing the ability to deliver early and aggressive treatment. Adalimumab, a recombinant monoclonal antibody to TNF-α, is an important therapeutic option, which affords children and adolescents with chronic illnesses an improved quality of life. PMID:24600289

  17. In favour of the definition "adolescents with idiopathic scoliosis": juvenile and adolescent idiopathic scoliosis braced after ten years of age, do not show different end results. SOSORT award winner 2014

    PubMed Central

    2014-01-01

    Background The most important factor discriminating juvenile (JIS) from adolescent idiopathic scoliosis (AIS) is the risk of deformity progression. Brace treatment can change natural history, even when risk of progression is high. The aim of this study was to compare the end of growth results of JIS subjects, treated after 10 years of age, with final results of AIS. Methods Design: prospective observational controlled cohort study nested in a prospective database. Setting: outpatient tertiary referral clinic specialized in conservative treatment of spinal deformities. Inclusion criteria: idiopathic scoliosis; European Risser 0–2; 25 degrees to 45 degrees Cobb; start treatment age: 10 years or more, never treated before. Exclusion criteria: secondary scoliosis, neurological etiology, prior treatment for scoliosis (brace or surgery). Groups: 27 patients met the inclusion criteria for the AJIS, (Juvenile Idiopathic Scoliosis treated in adolescence), demonstrated by an x-ray before 10 year of age, and treatment start after 10 years of age. AIS group included 45 adolescents with a diagnostic x-ray made after the threshold of age 10 years. Results at the end of growth were analysed; the threshold of 5 Cobb degree to define worsened, improved and stabilized curves was considered. Statistics: Mean and SD were used for descriptive statistics of clinical and radiographic changes. Relative Risk of failure (RR), Chi-square and T-test of all data was calculated to find differences among the two groups. 95% Confidence Interval (CI) , and of radiographic changes have been calculated. Results We did not find any Cobb angle significant differences among groups at baseline and at the end of treatment. The only difference was in the number of patients progressed above 45 degrees, found in the JIS group. The RR of progression of AJIS was, 1.35 (IC95% 0.57-3.17) versus AIS, and it wasn't statistically significant in the AJIS group, in respect to AIS group (p = 0.5338). Conclusion

  18. Assessment of spontaneous correction of lumbar curve after fusion of the main thoracic in Lenke 1 adolescent idiopathic scoliosis☆

    PubMed Central

    Mizusaki, Danilo; Gotfryd, Alberto Ofenhejm

    2016-01-01

    Objective To evaluate the clinical and radiographic response of the lumbar curve after fusion of the main thoracic, in patients with adolescent idiopathic scoliosis of Lenke type 1. Methods Forty-two patients with Lenke 1 adolescent idiopathic scoliosis who underwent operations via the posterior route with pedicle screws were prospectively evaluated. Clinical measurements (size of the hump and translation of the trunk in the coronal plane, by means of a plumb line) and radiographic measurements (Cobb angle, distal level of arthrodesis, translation of the lumbar apical vertebral and Risser) were made. The evaluations were performed preoperatively, immediately postoperatively and two years after surgery. Results The mean Cobb angle of the main thoracic curve was found to have been corrected by 68.9% and the lumbar curve by 57.1%. Eighty percent of the patients presented improved coronal trunk balance two years after surgery. In four patients, worsening of the plumb line measurements was observed, but there was no need for surgical intervention. Less satisfactory results were observed in patients with lumbar modifier B. Conclusions In Lenke 1 patients, fusion of the thoracic curve alone provided spontaneous correction of the lumbar curve and led to trunk balance. Less satisfactory results were observed in curves with lumbar modifier B, and this may be related to overcorrection of the main thoracic curve. PMID:26962505

  19. Etiologic theories of idiopathic scoliosis. Somatic nervous system and the NOTOM escalator concept as one component in the pathogenesis of adolescent idiopathic scoliosis.

    PubMed

    Burwell, R G; Dangerfield, P H; Freeman, B J C

    2008-01-01

    There is no generally accepted scientific theory for the causes of adolescent idiopathic scoliosis (AIS). In recent years encouraging advances thought to be related to the pathogenesis of AIS have been made in several fields. After reviewing concepts of AIS pathogenesis we formulated a collective model of pathogenesis. The central concept of this collective model is a normal neuro-osseous timing of maturation (NOTOM) system operating in a child's internal world during growth and maturation; this provides a dynamic physiological balance of postural equilibrium continuously renewed between two synchronous, polarized processes (NOTOM escalator) linked through sensory input and motor output, namely: 1) osseous escalator-increasing skeletal size and relative segmental mass, and 2) neural escalator - including the CNS body schema. The latter is recalibrated continuously as the body adjusts to biomechanical and kinematic changes resulting from skeletal enlargement, enabling it to coordinate motor actions. We suggest that AIS progression results from abnormality of the neural and/or osseous components of these normal escalator in time and/or space - as asynchrony and/or asymmetries - which cause a failure of neural systems to control asymmetric growth of a rapidly enlarging and moving adolescent spine. This putative initiating asymmetric growth in the spine is explained in separate papers as resulting from dysfunction of the hypothalamus expressed through the sympathetic nervous system (leptin-sympathetic nervous system concept for AIS pathogenesis). In girls, the expression of AIS may result from disharmony between the somatic and autonomic nervous systems - relative postural maturational delay in the somatic nervous system and hypothalamic dysfunction in the autonomic nervous system, with the conflict being fought out in the spine and trunk of the girl and compounded by biomechanical spinal growth modulation. PMID:18810026

  20. Postoperative pain relief using intermittent intrapleural analgesia following thoracoscopic anterior correction for progressive adolescent idiopathic scoliosis

    PubMed Central

    2013-01-01

    Background Thoracoscopic anterior scoliosis instrumentation is a safe and viable surgical option for corrective fusion of progressive adolescent idiopathic scoliosis (AIS) and has been performed at our centre on 205 patients since 2000. However, there is a paucity of literature reporting on or examining optimum methods of analgesia following this type of surgery. A retrospective study was designed to present the authors’ technique for delivering intermittent local anaesthetic boluses via an intrapleural catheter following thoracoscopic scoliosis surgery; report the pain levels that may be expected and any adverse effects associated with the use of intrapleural analgesia, as part of a combined postoperative analgesia regime. Methods Records for 32 patients who underwent thoracoscopic anterior correction for AIS were reviewed. All patients received an intrapleural catheter inserted during surgery, in addition to patient-controlled opiate analgesia and oral analgesia. After surgery, patients received a bolus of 0.25% bupivacaine every four hours via the intrapleural catheter. Patient’s perceptions of their pain control was measured using the visual analogue pain scale scores which were recorded before and after local anaesthetic administration and the quantity and time of day that any other analgesia was taken, were also recorded. Results 28 female and four male patients (mean age 14.5 ± 1.5 years) had a total of 230 boluses of local anaesthetic administered in the 96 hour period following surgery. Pain scores significantly decreased following the administration of a bolus (p < 0.0001), with the mean pain score decreasing from 3.66 to 1.83. The quantity of opiates via patient-controlled analgesia after surgery decreased steadily between successive 24 hours intervals after an initial increase in the second 24 hour period when patients were mobilised. One intrapleural catheter required early removal due to leakage; there were no other associated

  1. Reliability and Validity Study of Clinical Ultrasound Imaging on Lateral Curvature of Adolescent Idiopathic Scoliosis

    PubMed Central

    Wang, Q.; Li, M.; Lou, Edmond H. M.; Wong, M. S.

    2015-01-01

    Background Non-ionizing radiation imaging assessment has been advocated for the patients with adolescent idiopathic scoliosis (AIS). As one of the radiation-free methods, ultrasound imaging has gained growing attention in scoliosis assessment over the past decade. The center of laminae (COL) method has been proposed to measure the spinal curvature in the coronal plane of ultrasound image. However, the reliability and validity of this ultrasound method have not been validated in the clinical setting. Objectives To evaluate the reliability and validity of clinical ultrasound imaging on lateral curvature measurements of AIS with their corresponding magnetic resonance imaging (MRI) measurements. Methods Thirty curves (ranged 10.2°–68.2°) from sixteen patients with AIS were eligible for this study. The ultrasound scan was performed using a 3-D ultrasound unit within the same morning of MRI examination. Two researchers were involved in data collection of these two examinations. The COL method was used to measure the coronal curvature in ultrasound image, compared with the Cobb method in MRI. The intra- and inter-rater reliability of the COL method was evaluated by intra-class correlation coefficient (ICC). The validity of this method was analyzed by paired Student’s t-test, Bland–Altman statistics and Pearson correlation coefficient. The level of significance was set as 0.05. Results The COL method showed high intra- and inter-rater reliabilities (both with ICC (2, K) >0.9, p<0.05) to measure the coronal curvature. Compared with Cobb method, COL method showed no significant difference (p<0.05) when measuring coronal curvature. Furthermore, Bland-Altman method demonstrated an agreement between these two methods, and Pearson’s correlation coefficient (r) was high (r>0.9, p<0.05). Conclusion The ultrasound imaging could provide a reliable and valid measurement of spinal curvature in the coronal plane using the COL method. Further research is needed to validate the

  2. Gait in thoracolumbar/lumbar adolescent idiopathic scoliosis: effect of surgery on gait mechanisms.

    PubMed

    Mahaudens, Philippe; Detrembleur, C; Mousny, M; Banse, X

    2010-07-01

    For patients whose scoliosis progresses, surgery remains the ultimate way to correct and stabilise the deformity while maintaining as many mobile spinal segments as possible. In thoracolumbar/lumbar adolescent idiopathic scoliosis (AIS), the spinal fusion has to be extended to the lumbar spine. The use of anterior spinal fusion (ASF) instead of the classic posterior fusion (PSF) may preserve more distal spinal levels in attempt to limit the consequences of surgery on trunk mobility. The effects of surgery on body shape, pain and the decompensation phenomenon have all been well evaluated. Very few studies have addressed the effect of ASF or PSF on basic activities, such as walking. Before any treatment, AIS patients already have reduced pelvis, hip and shoulder motion when walking at a normal speed compared with adolescents without scoliosis (control group). Additionally, they have longer contraction time of the lumbar and pelvic muscles leading to an excessive energy cost and reduced muscle efficiency. In addition, if these changes are associated with spinal stiffness, spinal fusion could further negatively affect this pre-surgical inefficient walk. The goals of this study were (a) to compare pre- and 1-year post-surgery conditions in order to assess the effects of spinal arthrodesis on gait parameters and (b) to compare the anterior versus the posterior surgical approaches. Nineteen young females with thoracolumbar/lumbar AIS were assessed by radiological and clinical examination and by conventional gait analysis before surgery and at almost 12 months after surgery. Seven subjects underwent surgery using ASF and 12 using PSF. Three-dimensional gait analysis was performed on a motor-driven treadmill at spontaneous self-selected speed to record kinematic, electromyographic (EMG), mechanical and energetic measurements synchronously. Although it was expected that the instrumentation would modify the characteristics of normal walking, this study showed that surgery

  3. Gait in thoracolumbar/lumbar adolescent idiopathic scoliosis: effect of surgery on gait mechanisms

    PubMed Central

    Detrembleur, C.; Mousny, M.; Banse, X.

    2010-01-01

    For patients whose scoliosis progresses, surgery remains the ultimate way to correct and stabilise the deformity while maintaining as many mobile spinal segments as possible. In thoracolumbar/lumbar adolescent idiopathic scoliosis (AIS), the spinal fusion has to be extended to the lumbar spine. The use of anterior spinal fusion (ASF) instead of the classic posterior fusion (PSF) may preserve more distal spinal levels in attempt to limit the consequences of surgery on trunk mobility. The effects of surgery on body shape, pain and the decompensation phenomenon have all been well evaluated. Very few studies have addressed the effect of ASF or PSF on basic activities, such as walking. Before any treatment, AIS patients already have reduced pelvis, hip and shoulder motion when walking at a normal speed compared with adolescents without scoliosis (control group). Additionally, they have longer contraction time of the lumbar and pelvic muscles leading to an excessive energy cost and reduced muscle efficiency. In addition, if these changes are associated with spinal stiffness, spinal fusion could further negatively affect this pre-surgical inefficient walk. The goals of this study were (a) to compare pre- and 1-year post-surgery conditions in order to assess the effects of spinal arthrodesis on gait parameters and (b) to compare the anterior versus the posterior surgical approaches. Nineteen young females with thoracolumbar/lumbar AIS were assessed by radiological and clinical examination and by conventional gait analysis before surgery and at almost 12 months after surgery. Seven subjects underwent surgery using ASF and 12 using PSF. Three-dimensional gait analysis was performed on a motor-driven treadmill at spontaneous self-selected speed to record kinematic, electromyographic (EMG), mechanical and energetic measurements synchronously. Although it was expected that the instrumentation would modify the characteristics of normal walking, this study showed that surgery

  4. Long-term three-dimensional changes of the spine after posterior spinal instrumentation and fusion in adolescent idiopathic scoliosis.

    PubMed

    Papin, P; Labelle, H; Delorme, S; Aubin, C E; de Guise, J A; Dansereau, J

    1999-01-01

    This is a prospective study comparing the short- and long-term three-dimensional (3D) changes in shape, length and balance of the spine after spinal instrumentation and fusion in a group of adolescents with idiopathic scoliosis. The objective of the study was to evaluate the stability over time of the postoperative changes of the spine after instrumentation with multi rod, hook and screw instrumentation systems. Thirty adolescents (average age: 14.5+/-1.6 years) undergoing surgery by a posterior approach had computerized 3D reconstructions of the spine done at an average of 3 days preoperatively (stage I), and 2 months (stage II) and 2,5 years (stage III) after surgery, using a digital multi-planar radiographic technique. Stages I, II and III were compared using various geometrical parameters of spinal length, curve severity, and orientation. Significant improvement of curve magnitude between stages I and II was documented in the frontal plane for thoracic and lumbar curves, as well as in the orientation of the plane of maximum deformity, which was significantly shifted towards the sagittal plane in thoracic curves. However, there was a significant loss of this correction between stages II and III. Slight changes were noted in apical vertebral rotation, in thoracic kyphosis and in lumbar lordosis. Spinal length and height were significantly increased at stage II, but at long-term follow-up spinal length continued to increase while spinal height remained similar. These results indicate that although a significant 3D correction can be obtained after posterior instrumentation and fusion, a significant loss of correction and an increase in spinal length occur in the years following surgery, suggesting that a crankshaft phenomenon may be an important factor altering the long-term 3D correction after posterior instrumentation of the spine for idiopathic scoliosis. PMID:10190849

  5. [Exercise therapy in the treatment of idiopathic adolescent scoliosis: Is it useful?].

    PubMed

    Porte, M; Patte, K; Dupeyron, A; Cottalorda, J

    2016-06-01

    Many practitioners, pediatricians, and general practitioners prescribe physical therapy when tracking scoliosis. However, has physical therapy alone proved its efficacy in the care of the scoliosis to slow down progression? Our purpose is to report the results of a literature review on the effectiveness of rehabilitation in idiopathic scoliosis. No current study presents sufficient scientific proof to validate the efficacy of isolated exercise therapy in scoliosis. Learned societies recognize, however, the efficacy of combining conservative therapy (brace+physiotherapy) in idiopathic scoliosis. Should we then still prescribe rehabilitation without brace treatment? Although physical therapy alone does not seem effective in treating scoliosis, it can limit potential painful phenomena and be beneficial for respiratory function. The physical therapist can also teach the teenager the classic principles of hygiene of the back. It may therefore be appropriate to prescribe physical therapy, but the principles and objectives must be explained to the patient and family in light of current evidence-based medicine. PMID:27117993

  6. Idiopathic hypersomnia.

    PubMed

    Billiard, Michel; Sonka, Karel

    2016-10-01

    Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood. A familial background is often present but rigorous studies are still lacking. The key manifestation is hypersomnolence. It is often accompanied by sleep of long duration and debilitating sleep inertia. Polysomnography (PSG) followed by a multiple sleep latency test (MSLT) is mandatory, as well as a 24 h PSG or a 2-wk actigraphy in association with a sleep log to ensure a total 24-h sleep time longer than or equal to 66O minutes, when the mean sleep latency on the MSLT is longer than 8 min. Yet, MSLT is neither sensitive nor specific and the polysomnographic diagnostic criteria require continuous readjustment and biologic markers are still lacking. Idiopathic hypersomnia is most often a chronic condition though spontaneous remission may occur. The condition is disabling, sometimes even more so than narcolepsy type 1 or 2. Based on neurochemical, genetic and immunological analyses as well as on exploration of the homeostatic and circadian processes of sleep, various pathophysiological hypotheses have been proposed. Differential diagnosis involves a number of diseases and it is not yet clear whether idiopathic hypersomnia and narcolepsy type 2 are not the same condition. Until now, the treatment of idiopathic hypersomnia has mirrored that of the sleepiness of narcolepsy type 1 or 2. The first randomized, double-blind, placebo-controlled trials of modafinil have just been published, as well as a double

  7. Influence of pelvic asymmetry and idiopathic scoliosis in adolescents on postural balance during sitting.

    PubMed

    Jung, Ji-Yong; Cha, Eun-Jong; Kim, Kyung-Ah; Won, Yonggwan; Bok, Soo-Kyung; Kim, Bong-Ok; Kim, Jung-Ja

    2015-01-01

    The effects of pelvic asymmetry and idiopathic scoliosis on postural balance during sitting were studied by measuring inclination angles, pressure distribution, and electromyography. Participants were classified into a control group, pelvic asymmetry group, scoliosis group, and scoliosis with pelvic asymmetry and then performed anterior, posterior, left, and right pelvic tilting while sitting on the unstable board for 5 seconds to assess their postural balance. Inclination and obliquity angles between the groups were measured by an accelerometer located on the unstable board. Pressure distribution (maximum force and peak pressure) was analyzed using a capacitive seat sensor. In addition, surface electrodes were attached to the abdominal and erector spinae muscles of each participant. Inclination and obliquity angles increased more asymmetrically in participants with both pelvic asymmetry and scoliosis than with pelvic asymmetry or scoliosis alone. Maximum forces and peak pressures of each group showed an asymmetrical pressure distribution caused by the difference in height between the left and right pelvis and curve type of the patients' spines when performing anterior, posterior, left, and right pelvic tilting while sitting. Muscle contraction patterns of external oblique, thoracic erector spinae, lumbar erector spinae, and lumbar multifidus muscles may be influenced by spine curve type and region of idiopathic scoliosis. Asymmetrical muscle activities were observed on the convex side of scoliotic patients and these muscle activity patterns were changed by the pelvic asymmetry. From these results, it was confirmed that pelvic asymmetry and idiopathic scoliosis cause postural asymmetry, unequal weight distribution, and muscular imbalance during sitting. PMID:26406054

  8. Electromyographic responses of erector spinae and lower limb's muscles to dynamic postural perturbations in patients with adolescent idiopathic scoliosis.

    PubMed

    Farahpour, Nader; Ghasemi, Safoura; Allard, Paul; Saba, Mohammad Sadegh

    2014-10-01

    The aim of this study was to evaluate electromyographic (EMG) responses of erector spinae (ES) and lower limbs' muscles to dynamic forward postural perturbation (FPP) and backward postural perturbation (BPP) in patients with adolescent idiopathic scoliosis (AIS) and in a healthy control group. Ten right thoracic AIS patients (Cobb=21.6±4.4°) and 10 control adolescents were studied. Using bipolar surface electrodes, EMG activities of ES muscle at T10 (EST10) and L3 (ESL3) levels, biceps femoris (BF), gastrocnemius lateralis (G) and rectus femoris (RF) muscles in the right and the left sides during FPP and BPP were evaluated. Muscle responses were measured over a 1s time window after the onset of perturbation. In FPP test, the EMG responses of right EST10, ESL3 and BF muscles in the scoliosis group were respectively about 1.40 (p=0.035), 1.43 (p=0.07) and 1.45 (p=0.01) times greater than those in control group. Also, in BPP test, at right ESL3 muscle of the scoliosis group the EMG activity was 1.64 times higher than that in the control group (p=0.01). The scoliosis group during FPP displayed asymmetrical muscle responses in EST10 and BF muscles. This asymmetrical muscle activity in response to FPP is hypothesized to be a possible compensatory strategy rather than an inherent characteristic of scoliosis. PMID:25008019

  9. Body Image and Quality-of-Life in Untreated Versus Brace-Treated Females With Adolescent Idiopathic Scoliosis

    PubMed Central

    Schwieger, Traci; Campo, Shelly; Weinstein, Stuart L.; Dolan, Lori A.; Ashida, Sato; Steuber, Keli R.

    2016-01-01

    Study Design. The Bracing in Adolescent Idiopathic Scoliosis Trial (BrAIST) included skeletally immature high-risk patients with adolescent idiopathic scoliosis (AIS) with moderate curve sizes (20°– 40°). BrAIST was a multicenter, controlled trial using both randomized and preference treatment arms into either an observation group or a brace treatment group. Objective. The aim of this study was to analyze and compare body image and quality-of-life (QOL) in female AIS patients who were observed or treated with a brace. Summary of Background Data. Brace treatment is an effective means for controlling progressive scoliosis and preventing the need for surgery, but there is no consensus regarding the effect of brace treatment on body image or on QOL in adolescents with AIS. Methods. Data from female BrAIST patients in the randomized (n = 132) or preference (n = 187) arms and were observed (n = 120) or brace treated (n = 199) were analyzed. Patients completed the Spinal Appearance Questionnaire (SAQ) and the Pediatric Quality of Life Inventory (PedsQOL) 4.0 Generic Scales at baseline and 6 month follow-up visits up to 2 years. Items on the SAQ measured three body image constructs (self, ideal, and overall). The PedsQOL measured health, activities, feelings, social factors, and school. Results. . In general, there were no significant differences within or between study arms or treatments regarding body image or QOL through 2 years of follow-up. Poorer body image was significantly correlated with poorer QOL during the first 2 years of follow-up regardless of study arm or treatment. Patients who crossed-over to a different treatment and patients with largest Cobb angles ≥ 40 degrees had significantly poorer body image, in particular self-body image, compared with those that did not. Conclusion. This study does not support findings from previous research indicating that wearing a brace has a negative impact on or is negatively impacted by body image or

  10. Posterior-only surgery with strong halo-femoral traction for the treatment of adolescent idiopathic scoliotic curves more than 100°.

    PubMed

    Zhang, Hong-qi; Wang, Yu-xiang; Guo, Chao-feng; Tang, Ming-xing; Chen, Ling-qiang; Liu, Shao-hua; Wang, Yong-fu; Chen, Jing

    2011-07-01

    The aim of this study was to investigate the feasibility and clinical efficacy of treatment of adolescent idiopathic scoliosis of >100° via posterior-only surgery with strong halo-femoral traction and posterior wide release. From December 2003 to August 2006, 121 patients with adolescent idiopathic scoliosis were treated in our hospital; among them, 29 patients with curves over 100° were included in this study. From December 2003 to June 2005, group A included the first 12 patients who underwent combined anterior release followed by two-week halo-femoral traction and then posterior instrumentation. From July 2005 to August 2006, 17 patients in group B underwent posterior surgery alone with strong halo-femoral traction and posterior wide release. All of the patients were followed-up for a minimum of 31 months (mean, 36 months; range, 31-41 months). There were no severe complications. All of the patients achieved bony fusion without instrumentation breakage or pseudarthrosis. There were no statistically significant differences between the two groups in gender, age, type of adolescent idiopathic scoliosis, preoperative coronal major curve values, major curve flexibility, or final follow-up major curve correction rate. The average operative time, blood loss and hospital stay in group B were less than those in group A. In adolescent idiopathic scoliosis with Cobb >100°, posterior-only surgery with strong halo-femoral traction and posterior wide release can provide comparable curve correction with shorter operative time, less blood loss and shorter hospital stay when compared to combined anteroposterior surgery. PMID:20703456

  11. Transient long thoracic nerve injury during posterior spinal fusion for adolescent idiopathic scoliosis: A report of two cases.

    PubMed

    Tsirikos, Athanasios I; Al-Hourani, Khalid

    2013-11-01

    We present the transient long thoracic nerve (LTN) injury during instrumented posterior spinal arthrodesis for idiopathic scoliosis. The suspected mechanism of injury, postoperative course and final outcome is discussed. The LTN is susceptible to injury due to its long and relatively superficial course across the thoracic wall through direct trauma or tension. Radical mastectomies with resection of axillary lymph nodes, first rib resection to treat thoracic outlet syndrome and cardiac surgery can be complicated with LTN injury. LTN injury producing scapular winging has not been reported in association with spinal deformity surgery. We reviewed the medical notes and spinal radiographs of two adolescent patients with idiopathic scoliosis who underwent posterior spinal arthrodesis and developed LTN neuropraxia. Scoliosis surgery was uneventful and intraoperative spinal cord monitoring was stable throughout the procedure. Postoperative neurological examination was otherwise normal, but both patients developed winging of the scapula at 4 and 6 days after spinal arthrodesis, which did not affect shoulder function. Both patients made a good recovery and the scapular winging resolved spontaneously 8 and 11 months following surgery with no residual morbidity. We believe that this LTN was due to positioning of our patients with their head flexed, tilted and rotated toward the contralateral side while the arm was abducted and extended. The use of heavy retractors may have also applied compression or tension to the nerve in one of our patients contributing to the development of neuropraxia. This is an important consideration during spinal deformity surgery to prevent potentially permanent injury to the nerve, which can produce severe shoulder dysfunction and persistent pain. PMID:24379470

  12. Whither the etiopathogenesis (and scoliogeny) of adolescent idiopathic scoliosis? Incorporating presentations on scoliogeny at the 2012 IRSSD and SRS meetings.

    PubMed

    Burwell, R Geoffrey; Dangerfield, Peter H; Moulton, Alan; Grivas, Theodoros B; Cheng, Jack Cy

    2013-01-01

    This paper aims to integrate into current understanding of AIS causation, etiopathogenetic information presented at two Meetings during 2012 namely, the International Research Society of Spinal Deformities (IRSSD) and the Scoliosis Research Society (SRS). The ultimate hope is to prevent the occurrence or progression of the spinal deformity of AIS with non-invasive treatment, possibly medical. This might be attained by personalised polymechanistic preventive therapy targeting the appropriate etiology and/or etiopathogenetic pathways, to avoid fusion and maintain spinal mobility. Although considerable progress had been made in the past two decades in understanding the etiopathogenesis of adolescent idiopathic scoliosis (AIS), it still lacks an agreed theory of etiopathogenesis. One problem may be that AIS results not from one cause, but several that interact with various genetic predisposing factors. There is a view there are two other pathogenic processes for idiopathic scoliosis namely, initiating (or inducing), and those that cause curve progression. Twin studies and observations of family aggregation have revealed significant genetic contributions to idiopathic scoliosis, that place AIS among other common disease or complex traits with a high heritability interpreted by the genetic variant hypothesis of disease. We summarize etiopathogenetic knowledge of AIS as theories of pathogenesis including recent multiple concepts, and blood tests for AIS based on predictive biomarkers and genetic variants that signify disease risk. There is increasing evidence for the possibility of an underlying neurological disorder for AIS, research which holds promise. Like brain research, most AIS workers focus on their own corner and there is a need for greater integration of research effort. Epigenetics, a relatively recent field, evaluates factors concerned with gene expression in relation to environment, disease, normal development and aging, with a complex regulation across the

  13. Whither the etiopathogenesis (and scoliogeny) of adolescent idiopathic scoliosis? Incorporating presentations on scoliogeny at the 2012 IRSSD and SRS meetings

    PubMed Central

    2013-01-01

    This paper aims to integrate into current understanding of AIS causation, etiopathogenetic information presented at two Meetings during 2012 namely, the International Research Society of Spinal Deformities (IRSSD) and the Scoliosis Research Society (SRS). The ultimate hope is to prevent the occurrence or progression of the spinal deformity of AIS with non-invasive treatment, possibly medical. This might be attained by personalised polymechanistic preventive therapy targeting the appropriate etiology and/or etiopathogenetic pathways, to avoid fusion and maintain spinal mobility. Although considerable progress had been made in the past two decades in understanding the etiopathogenesis of adolescent idiopathic scoliosis (AIS), it still lacks an agreed theory of etiopathogenesis. One problem may be that AIS results not from one cause, but several that interact with various genetic predisposing factors. There is a view there are two other pathogenic processes for idiopathic scoliosis namely, initiating (or inducing), and those that cause curve progression. Twin studies and observations of family aggregation have revealed significant genetic contributions to idiopathic scoliosis, that place AIS among other common disease or complex traits with a high heritability interpreted by the genetic variant hypothesis of disease. We summarize etiopathogenetic knowledge of AIS as theories of pathogenesis including recent multiple concepts, and blood tests for AIS based on predictive biomarkers and genetic variants that signify disease risk. There is increasing evidence for the possibility of an underlying neurological disorder for AIS, research which holds promise. Like brain research, most AIS workers focus on their own corner and there is a need for greater integration of research effort. Epigenetics, a relatively recent field, evaluates factors concerned with gene expression in relation to environment, disease, normal development and aging, with a complex regulation across the

  14. The effect of chiropractic techniques on the Cobb angle in idiopathic scoliosis arising in adolescence

    PubMed Central

    Byun, Sunghak; Han, Dongwook

    2016-01-01

    [Purpose] The purpose of this study was to examine whether chiropractic techniques would reduce the curvature of idiopathic scoliosis, which commonly occurs in elementary school children. [Subjects] The subjects of this study were 5 healthy elementary students who listened to an explanation of the study methods and purpose of the study and agreed to participate in the experiment. [Methods] The Cobb angle was measured by taking an X-ray (FCT-1, Dongmun, Goyangsi, Republic of Korea) taken from the rear, using X-ray film. The method of intervention this study used was application of chiropractic techniques. Spinal correction was carried out for 30 minutes per session, which included soft tissue massage, 3 times a week for 8 weeks. [Results] It was established that the Cobb angle was noticeably decreased after 4 weeks of the intervention. Post Hoc analysis revealed that the Cobb angle noticeably decreased after 4 weeks compared with the Cobb angle before the chiropractic techniques were applied. However, no significant difference in Cobb angle was evident after the fourth week. [Conclusion] This study demonstrated that chiropractic techniques can effectively reduce the Cobb angle within as little as 4 weeks. So, we can confirm that the chiropractic techniques were effective for reducing the curvature of idiopathic scoliosis. PMID:27190435

  15. The effect of chiropractic techniques on the Cobb angle in idiopathic scoliosis arising in adolescence.

    PubMed

    Byun, Sunghak; Han, Dongwook

    2016-04-01

    [Purpose] The purpose of this study was to examine whether chiropractic techniques would reduce the curvature of idiopathic scoliosis, which commonly occurs in elementary school children. [Subjects] The subjects of this study were 5 healthy elementary students who listened to an explanation of the study methods and purpose of the study and agreed to participate in the experiment. [Methods] The Cobb angle was measured by taking an X-ray (FCT-1, Dongmun, Goyangsi, Republic of Korea) taken from the rear, using X-ray film. The method of intervention this study used was application of chiropractic techniques. Spinal correction was carried out for 30 minutes per session, which included soft tissue massage, 3 times a week for 8 weeks. [Results] It was established that the Cobb angle was noticeably decreased after 4 weeks of the intervention. Post Hoc analysis revealed that the Cobb angle noticeably decreased after 4 weeks compared with the Cobb angle before the chiropractic techniques were applied. However, no significant difference in Cobb angle was evident after the fourth week. [Conclusion] This study demonstrated that chiropractic techniques can effectively reduce the Cobb angle within as little as 4 weeks. So, we can confirm that the chiropractic techniques were effective for reducing the curvature of idiopathic scoliosis. PMID:27190435

  16. Progression or not progression? How to deal with adolescent idiopathic scoliosis during puberty.

    PubMed

    Dimeglio, Alain; Canavese, Federico

    2013-02-01

    Idiopathic scoliosis is a growing spine disorder. Only a perfect knowledge of normal growth parameters such as bone age, stages of puberty, standing and sitting height, arm spam, Tanner stages, and weight allow the surgeon to plan the best treatment at the right moment. Measurements should be repeated and carefully recorded at regular intervals. Puberty is not a sudden event: it has a gradual onset and it is preceded by an incubation phase. It is not a point on a chart: it is a period that lasts two years beginning at a bone age of 11 and 13 years in girls and in boys, respectively. Two years of rapid growth ("acceleration phase") are followed by three years of steady reduction of growth rates ("deceleration phase"). Skeletal maturation needs to be evaluated carefully to evaluate the progression risk of scoliosis during the phase of accelerating growth velocity. This article explains how to identify pubertal growth spurt, both clinically and radiographically, in order to correctly monitor patients with idiopathic scoliosis. PMID:24432058

  17. Segmental correction of adolescent idiopathic scoliosis by all-screw fixation method in adolescents and young adults. minimum 5 years follow-up with SF-36 questionnaire

    PubMed Central

    2012-01-01

    Background In our institution, the fixation technique in treating idiopathic scoliosis was shifted from hybrid fixation to the all-screw method beginning in 2000. We conducted this study to assess the intermediate -term outcome of all-screw method in treating adolescent idiopathic scoliosis (AIS). Methods Forty-nine consecutive patients were retrospectively included with minimum of 5-year follow-up (mean, 6.1; range, 5.1-7.3 years). The average age of surgery was 18.5 ± 5.0 years. We assessed radiographic measurements at preoperative (Preop), postoperative (PO) and final follow-up (FFU) period. Curve correction rate, correction loss rate, complications, accuracy of pedicle screws and SF-36 scores were analyzed. Results The average major curve was corrected from 58.0 ± 13.0° Preop to 16.0 ± 9.0° PO(p < 0.0001), and increased to 18.4 ± 8.6°(p = 0.12) FFU. This revealed a 72.7% correction rate and a correction loss of 2.4° (3.92%). The thoracic kyphosis decreased little at FFU (22 ± 12° to 20 ± 6°, (p = 0.25)). Apical vertebral rotation decreased from 2.1 ± 0.8 PreOP to 0.8 ± 0.8 at FFU (Nash-Moe grading, p < 0.01). Among total 831 pedicle screws, 56 (6.7%) were found to be malpositioned. Compared with 2069 age-matched Taiwanese, SF-36 scores showed inferior result in 2 variables: physical function and role physical. Conclusion Follow-up more than 5 years, the authors suggest that all-screw method is an efficient and safe method. PMID:22340624

  18. Magnetic resonance imaging investigations of position of conus medullaris in adolescent idiopathic scoliosis as a peripheral neuropathy.

    PubMed

    Hesarikia, Hamid; Azma, Kamran; Kousari, Aliasghar; Nikouei, Farshad

    2015-01-01

    Adolescent idiopathic scoliosis (AIS) is a structural 3-dimensional deformity the spine, which is occurring between 10 years of age and skeletal maturity and it mostly affects prepuberbal girls. The etiology of AIS remains unknown and seems should be multifactorial. According to the theories, there could be a shorter spinal cord or a higher location of the conus medullaris and disproportionate growth in neuro-osseous system. This study wants to investigate the position of the conus medullaris in AIS patients with a large curve magnitude in comparison with healthy adolescents. 94 AIS patients consisting of 25 males and 69 females between 11 and 25 years old, based on physical examination and standing posteroanterior roentgenography of the total spine with a Cobb angle more than 40 degrees was chosen. The main curve magnitude of every AIS patient was measured by the Cobb method. Apex of deformity was determined based on SRS definition. Patients' deformity were calcified based on Lenke classification. Magnetic resonance imaging examinations of the total spine were performed in the AIS group, by means of a 1.5-T magnetic resonance imaging system. The position of conus medullaris was defined according to the method by saffiudin et al and was assessed based on the age, sex, type of deformity, severity of deformity, kyphosis, lordosis, flexibility, apical vertebra, stable vertebra. The mean age of patients were 16.34 with 6.77 of conus medullaris position in mean, which was lower one-third of L1. Our study showed no significant correlation between the position of conus medullaris with age, weight, preoperative curve, flexibility, types of deformity based on Lenke classification and degree of kyphosis and lordisis. In conclusion, there is the same mean and the distribution of the conus medullaris locations for AIS patients and normal populations. PMID:26131185

  19. Determinants of health-related quality of life impairment in Egyptian children and adolescents with juvenile idiopathic arthritis: Sharkia Governorate.

    PubMed

    Abdul-Sattar, Amal B; Elewa, Enass A; El-Shahawy, Eman El-Dessoky; Waly, Eman H

    2014-08-01

    The aim of this study was to identify the possible determinants of impaired health-related quality of life (HRQOL) in Egyptian children and adolescents with juvenile idiopathic arthritis (JIA). Fifty-eight consecutive patients of JIA aged from 8 to 18 years underwent assessment of socio-economic and demographic characteristics; HRQOL using Pediatric Quality of Life Inventory 4.0 Generic Core Scale, disease activity using the Juvenile Arthritis Disease Activity Score based on 27 joints (JADAS-27), functional ability using the childhood health assessment questionnaire (CHAQ), pain score on visual analog scale and psychological symptoms using the Children's Depression Inventory (CDI) score. Multivariate modeling was applied to determine the factors that associated with HRQOL impairment. A total of 55 % of the patients (32 of 58) had impaired HRQOL (<78.6). In multiple regression analyses, high CHAQ scores (OR 6.0, 95 % CI 2.0-17.5, P = 0.001), pain (OR 3.1, 95 % CI 1.9-6.3, P = 0.01), stop going to school (OR 3.9, 95 % CI 2.0-7.3, P = 0.01), low socioeconomic status (OR 2.3, 95 % CI 1.09-4.7, P = 0.04) and high psychological symptoms (OR 4.2, 95 % CI 2.0-12.6, P = 0.001) were determinants for HRQOL impairment. HRQOL impairment is a significant problem in Egyptian children and adolescents with JIA. These findings underscore the critical need for monitoring of HRQOL in these patients. More attention should be given to JIA patients who stop going to school and who has low socioeconomic status. PMID:24469640

  20. Adolescent Idiopathic Scoliosis: A 71 Cases Study Ascertaining that Straightening Is Possible, and a New Etiological Hypothesis

    PubMed Central

    2013-01-01

    Study Design Seventy-one children (23 boys and 48 girls, aged 6 to 18 year-old) with adolescent idiopathic scoliosis (AIS) between 11° and 62°, without braces, have been treated manually, only at the level of the neck. Purpose To ascertain that non-surgical straightening of AIS is possible (without brace). Overview of Literature So far no disease modifying treatment for AIS existed. Braces can only slow down worsening (and this can only be achieved if they are worn 23 hours a day). Surgery is not without important risks. Methods All patients have been treated exclusively with a manual therapy called Brachy-Myotherapy. This method treats spasmed (contractured) muscles by placing them in a shortening position according to a specific protocol. Results An average straightening of 8° of AIS was observed, with a maximum of 25°. 94% of cases improved, 67 out of 71. The worst prognosis was, the better results. The more advanced AIS was, the better the results. Conclusions A simple and reliable treatment of AIS is possible. AIS seems to be a compensation mechanism of the body, with the aim of keeping the ears, and thus the labyrinths, at a horizontal level for correct equilibrium. When lasting post-traumatic neck muscle contractures causing a permanent side-bending of the skull have been treated, this compensation mechanism becomes irrelevant and scoliosis tends to subside. PMID:24353844

  1. Reliability Analysis of the Distal Radius and Ulna Classification for Assessing Skeletal Maturity for Patients with Adolescent Idiopathic Scoliosis

    PubMed Central

    Cheung, Jason Pui Yin; Samartzis, Dino; Cheung, Prudence Wing Hang; Cheung, Kenneth M. C.; Luk, Keith D. K.

    2015-01-01

    Study Design Prospective radiographic study. Objective To test the reliability of the Distal Radius and Ulna Classification (DRU). Methods This single-center study included prospectively recruited subjects with adolescent idiopathic scoliosis managed with bracing. The left-hand radiographs were measured using the DRU classification by two examiners. Intra- and interobserver reliability analysis were performed using intraclass correlation (ICC) analysis. Results From these clinics, 161 patients (124 females and 37 males) with left-hand radiographs were included in the study. The mean age was 13.3 years (standard deviation: 1.5). There was excellent intra- (ICC: 0.93 to 0.95) and interobserver (ICC: 0.97) reliability. Conclusions The DRU classification scheme has been shown to be accurate in determining the peak growth phase and growth cessation. It has now been confirmed to be a reliable tool. Future prospective studies should be performed to investigate its application in deciding when to apply bracing or operative treatment. PMID:26933618

  2. Experiments with a novel content-based image retrieval software: can we eliminate classification systems in adolescent idiopathic scoliosis?

    PubMed

    Menon, K Venugopal; Kumar, Dinesh; Thomas, Tessamma

    2014-02-01

    Study Design Preliminary evaluation of new tool. Objective To ascertain whether the newly developed content-based image retrieval (CBIR) software can be used successfully to retrieve images of similar cases of adolescent idiopathic scoliosis (AIS) from a database to help plan treatment without adhering to a classification scheme. Methods Sixty-two operated cases of AIS were entered into the newly developed CBIR database. Five new cases of different curve patterns were used as query images. The images were fed into the CBIR database that retrieved similar images from the existing cases. These were analyzed by a senior surgeon for conformity to the query image. Results Within the limits of variability set for the query system, all the resultant images conformed to the query image. One case had no similar match in the series. The other four retrieved several images that were matching with the query. No matching case was left out in the series. The postoperative images were then analyzed to check for surgical strategies. Broad guidelines for treatment could be derived from the results. More precise query settings, inclusion of bending films, and a larger database will enhance accurate retrieval and better decision making. Conclusion The CBIR system is an effective tool for accurate documentation and retrieval of scoliosis images. Broad guidelines for surgical strategies can be made from the postoperative images of the existing cases without adhering to any classification scheme. PMID:24494177

  3. Effects of Multilevel Facetectomy and Screw Density on Postoperative Changes in Spinal Rod Contour in Thoracic Adolescent Idiopathic Scoliosis Surgery

    PubMed Central

    Kokabu, Terufumi; Sudo, Hideki; Abe, Yuichiro; Ito, Manabu; Ito, Yoichi M.; Iwasaki, Norimasa

    2016-01-01

    Flattening of the preimplantation rod contour in the sagittal plane influences thoracic kyphosis (TK) restoration in adolescent idiopathic scoliosis (AIS) surgery. The effects of multilevel facetectomy and screw density on postoperative changes in spinal rod contour have not been documented. This study aimed to evaluate the effects of multilevel facetectomy and screw density on changes in spinal rod contour from before implantation to after surgical correction of thoracic curves in patients with AIS prospectively. The concave and convex rod shapes from patients with thoracic AIS (n = 49) were traced prior to insertion. Postoperative sagittal rod shape was determined by computed tomography. The angle of intersection of the tangents to the rod end points was measured. Multiple stepwise linear regression analysis was used to identify variables independently predictive of change in rod contour (Δθ). Average Δθ at the concave and convex side were 13.6° ± 7.5° and 4.3° ± 4.8°, respectively. The Δθ at the concave side was significantly greater than that of the convex side (P < 0.0001) and significantly correlated with Risser sign (P = 0.032), the preoperative main thoracic Cobb angle (P = 0.031), the preoperative TK angle (P = 0.012), and the number of facetectomy levels (P = 0.007). Furthermore, a Δθ at the concave side ≥14° significantly correlated with the postoperative TK angle (P = 0.003), the number of facetectomy levels (P = 0.021), and screw density at the concave side (P = 0.008). Rod deformation at the concave side suggests that corrective forces acting on that side are greater than on the convex side. Multilevel facetectomy and/or screw density at the concave side have positive effects on reducing the rod deformation that can lead to a loss of TK angle postoperatively. PMID:27564683

  4. Spot14/Spot14R expression may be involved in MSC adipogenic differentiation in patients with adolescent idiopathic scoliosis

    PubMed Central

    WANG, QIFEI; YANG, JUNLIN; LIN, XIANG; HUANG, ZIFANG; XIE, CHAOFAN; FAN, HENGWEI

    2016-01-01

    The aim of the present study was to evaluate the different expression levels of thyroid hormone responsive (THRSP; Spot14)/S14 related, Mig12 (S14R) during bone marrow mesenchymal stem cell (BM-MSC) adipogenesis in adolescent idiopathic scoliosis (AIS) patients. MSCs were retrospectively isolated from AIS patients and controls, and adipogenic differentiation was induced. Total RNA was extracted for Affymetrix 3′-IVT expression profiling microarrays and compared with the results from healthy controls. The results were confirmed by semiquantitative reverse transcription-polymerase chain reaction (RT-PCR) validation and the protein expression levels of Spot14 and its paralogous gene S14R by western blotting and immunohistochemistry. A total of 300 significantly altered mRNAs were detected (111 upregulated and 189 downregulated) and confirmed by RT-qPCR. The mRNA expression levels of seven genes, including Spot14, were altered by >2-fold in AIS patients. Spot14/S14R was selected for further investigation. The results of the western blotting demonstrated that mRNA and protein expression levels of Spot14/S14R were significantly higher in AIS patients than the controls (P<0.05). Immunohistochemistry demonstrated Spot14 was expressed in 85% (17/20 cases) in adipose tissue samples from AIS patients and 23.1% (3/13 cases) of adipose tissue samples from controls. The positive ratio of Spot14 in adipose tissue samples from AIS was significantly higher than the controls (P<0.001). The results of the present study indicated that Spot14/S14R were differently expressed in MSC adipogenesis in AIS patients, and they may be important in the abnormal adipogenic differentiation in AIS. PMID:27082501

  5. Spot14/Spot14R expression may be involved in MSC adipogenic differentiation in patients with adolescent idiopathic scoliosis.

    PubMed

    Wang, Qifei; Yang, Junlin; Lin, Xiang; Huang, Zifang; Xie, Chaofan; Fan, Hengwei

    2016-06-01

    The aim of the present study was to evaluate the different expression levels of thyroid hormone responsive (THRSP; Spot14)/S14 related, Mig12 (S14R) during bone marrow mesenchymal stem cell (BM-MSC) adipogenesis in adolescent idiopathic scoliosis (AIS) patients. MSCs were retrospectively isolated from AIS patients and controls, and adipogenic differentiation was induced. Total RNA was extracted for Affymetrix 3'‑IVT expression profiling microarrays and compared with the results from healthy controls. The results were confirmed by semiquantitative reverse transcription-polymerase chain reaction (RT-PCR) validation and the protein expression levels of Spot14 and its paralogous gene S14R by western blotting and immunohistochemistry. A total of 300 significantly altered mRNAs were detected (111 upregulated and 189 downregulated) and confirmed by RT‑qPCR. The mRNA expression levels of seven genes, including Spot14, were altered by >2‑fold in AIS patients. Spot14/S14R was selected for further investigation. The results of the western blotting demonstrated that mRNA and protein expression levels of Spot14/S14R were significantly higher in AIS patients than the controls (P<0.05). Immunohistochemistry demonstrated Spot14 was expressed in 85% (17/20 cases) in adipose tissue samples from AIS patients and 23.1% (3/13 cases) of adipose tissue samples from controls. The positive ratio of Spot14 in adipose tissue samples from AIS was significantly higher than the controls (P<0.001). The results of the present study indicated that Spot14/S14R were differently expressed in MSC adipogenesis in AIS patients, and they may be important in the abnormal adipogenic differentiation in AIS. PMID:27082501

  6. Understanding the role of the immune system in adolescent idiopathic scoliosis: Immunometabolic CONnections to Scoliosis (ICONS) study protocol

    PubMed Central

    Samaan, M Constantine; Missiuna, Paul; Peterson, Devin; Thabane, Lehana

    2016-01-01

    Introduction Adolescent idiopathic scoliosis (AIS) affects up to 3% of children around the world. There is limited knowledge of AIS aetiopathogenesis, and this evidence is needed to develop new management strategies. Paraspinal muscle in AIS demonstrates evidence of differential fibrosis based on curve sidedness. Fibrosis is the hallmark of macrophage-driven inflammation and tissue remodelling, yet the mechanisms of fibrosis in paraspinal muscle in AIS are poorly understood. Objectives The primary objective of this study is to determine the influence of curve sidedness on paraspinal muscle inflammation. Secondary objectives include defining the mechanisms of macrophage homing to muscle, and determining muscle–macrophage crosstalk in muscle fibrosis in AIS. Methods and analysis This is a cross-sectional study conducted in a tertiary paediatric centre in Hamilton, Ontario, Canada. We will recruit boys and girls, 10–17 years of age, who are having surgery to correct AIS. We will exclude children who have an active infection or are on immunosuppressive therapies within 2 weeks of surgery, smokers and pregnant girls. Paraspinal muscle biopsies will be obtained at the start of surgery. Also, blood and urine samples will be collected from participants, who will fill questionnaires about their lifestyle. Anthropometric measures will also be collected including height, weight, waist and hip circumferences. Ethics and dissemination This study has received ethics authorisation by the institutional review board. This work will be published in peer-reviewed journals and will be presented in oral and poster formats at scientific meetings. Discussion This study will explore the mechanisms of paraspinal muscle inflammation, remodelling and fibrosis in AIS. This will help identify pathways and molecules as potential therapeutic targets to treat and prevent AIS. It may also yield markers that predict scoliosis progression and response to treatment in these children. PMID

  7. Factors favoring regain of the lost vertical spinal height through posterior spinal fusion in adolescent idiopathic scoliosis.

    PubMed

    Shi, Benlong; Mao, Saihu; Xu, Leilei; Sun, Xu; Liu, Zhen; Zhu, Zezhang; Lam, Tsz Ping; Cheng, Jack Cy; Ng, Bobby; Qiu, Yong

    2016-01-01

    Height gain is a common beneficial consequence following correction surgery in adolescent idiopathic scoliosis (AIS), yet little is known concerning factors favoring regain of the lost vertical spinal height (SH) through posterior spinal fusion. A consecutive series of AIS patients from February 2013 to August 2015 were reviewed. Surgical changes in SH (ΔSH), as well as the multiple coronal and sagittal deformity parameters were measured and correlated. Factors associated with ΔSH were identified through Pearson correlation analysis and multivariate regression analysis. A total of 172 single curve and 104 double curve patients were reviewed. The ΔSH averaged 2.5 ± 0.9 cm in single curve group and 2.9 ± 1.0 cm in double curve group. The multivariate regression analysis revealed the following pre-operative variables contributed significantly to ΔSH: pre-op Cobb angle, pre-op TK (single curve group only), pre-op GK (double curve group only) and pre-op LL (double curve group only) (p < 0.05). Thus change in height (in cm) = 0.044 × (pre-op Cobb angle) + 0.012 × (pre-op TK) (Single curve, adjusted R(2) = 0.549) or 0.923 + 0.021 × (pre-op Cobb angle1) + 0.028 × (pre-op Cobb angle2) + 0.015 × (pre-op GK)-0.012 × (pre-op LL) (Double curve, adjusted R(2) = 0.563). Severer pre-operative coronal Cobb angle and greater sagittal curves were beneficial factors favoring more contribution to the surgical lengthening effect in vertical spinal height in AIS. PMID:27373798

  8. Abnormal Bone Mechanical and Structural Properties in Adolescent Idiopathic Scoliosis: A Study with Finite Element Analysis and Structural Model Index.

    PubMed

    Cheuk, K Y; Zhu, T Y; Yu, F W P; Hung, V W Y; Lee, K M; Qin, L; Cheng, J C Y; Lam, T P

    2015-10-01

    Previous studies found adolescent idiopathic scoliosis (AIS) is associated with low bone mineral density (BMD) and abnormal bone quality, whilst the association between AIS and their bone strength is unknown. From high-resolution peripheral quantitative computed tomography-generated images, bone mechanical properties can be evaluated with finite element analysis (FEA), and trabecular rod-plate configuration related to trabecular bone strength can be quantified by structure model index (SMI). This study aimed to compare trabecular configuration and bone mechanical properties between AIS and the controls. 95 AIS girls aged 12-14 years and 97 age- and gender-matched normal controls were recruited. Bilateral femoral necks and non-dominant distal radius were scanned by dual-energy X-ray absorptiometry for areal BMD and HR-pQCT for SMI and FEA, respectively. Subjects were further classified into osteopenic and non-osteopenic group based on their areal BMD. Bone mechanical properties (stiffness, failure load and apparent modulus) were calculated using FEA. Linear regression model was used for controlling age, physical activity and calcium intake. AIS was associated with lower failure load and apparent modulus after adjusting for age, whereas AIS was associated with lower apparent modulus after adjusting for all confounders. Osteopenic AIS was associated with more rod-like trabeculae when compared with non-osteopenic AIS, whereas no difference was detected between osteopenic and non-osteopenic controls. This might be the result of abnormal regulation and modulation of bone metabolism and bone modelling and remodelling in AIS which will warrant future studies with a longitudinal design to determine the significance of micro-architectural abnormalities in AIS. PMID:26100651

  9. Pedicle Screw Instrumentation for Adolescent Idiopathic Scoliosis: The Insertion Technique, the Fusion Levels and Direct Vertebral Rotation

    PubMed Central

    2011-01-01

    The pedicle is a power nucleus of the vertebra and offers a secure grip of all 3 columns. Pedicle screw instrumentation has advantages of rigid fixation with improved three-dimensional (3D) correction and it is accepted as a reliable method with a high margin of safety. Accurate placement of the pedicle screws is important to reduce possible irreversible complications. Many methods of screw insertion have been reported. The author has been using the K-wire method coupled with the intraoperative single posteroanterior and lateral radiographs, which is the most safe, accurate and fast method. Identification of the curve patterns and determining the fusion levels are very important. The ideal classification of adolescent idiopathic scoliosis should address the all patterns, predict the extent of accurate fusion and have good inter/intraobserver reliability. My classification system matches with the ideal classification system, and it is simple and easy to learn; and my classification system has only 4 structural curve patterns and each curve has 2 types. Scoliosis is a 3D deformity; the coronal and sagittal curves can be corrected with rod rotation, and rotational deformity has to be corrected with direct vertebral rotation (DVR). Rod derotation and DVR are true methods of 3D deformity correction with shorter fusion and improved correction of both the fused and unfused curves, and this is accomplished using pedicle screw fixation. The direction of DVR is very important and it should be opposite to the direction of the rotational deformity of the vertebra. A rigid rod has to be used to prevent rod bend-out during the derotation and DVR. PMID:21629468

  10. Determination of the Distal Fusion Level in the Management of Thoracolumbar and Lumbar Adolescent Idiopathic Scoliosis Using Pedicle Screw Instrumentation

    PubMed Central

    Kim, Sung-Soo; Kim, Jin-Hyok; Kim, Jong-Woo; Um, Kyu-Sub; Ahn, Soo-Hyung; Suk, Se-Il

    2014-01-01

    Study Design A retrospective study. Purpose To determine the exact distal fusion level in the management of thoracolumbar/lumbar adolescent idiopathic scoliosis (TL/L AIS) using pedicle screw instrumentation (PSI). Overview of Literature The selection of distal fusion level remains controversial in TL/L AIS. Methods Radiographic parameters of 66 TL/L AIS patients were analyzed. The patients were grouped according to the distal fusion level; L3 group (fusion to L3, n=58) and L4 group (fusion to L4, n=8). The L3 group was subdivided into L3A (L3 crosses the mid-sacral line with rotation of less than grade II, n=33) and L3B (L3 does not cross the mid-sacral line or rotation is grade II or more, n=25) based on both bending radiographs. All of the patients in the L4 group had the same location and rotation of L3 in bending films as that of patients in the L3B group. An unsatisfactory result was defined as a lowest instrumented vertebral tilt (LIVT) of more than 10° or coronal balance of more than 15 mm. Results Among the 3 groups, there was a significantly lesser correction in the TL/L curve and LIVT in the L3B group. Unsatisfactory results were obtained in 3 patients (9.1%) of the L3A group, in 15 patients (68.2%) of the L3B group, and in 1 patient (12.5%) of the L4 group with a significant difference. Conclusions In TL/L AIS treatment with PSI, the curve can be fused to L3 with favorable radiographic outcomes when L3 crosses the mid-sacral line with rotation of less than grade II in bending films. Otherwise, fusion has to be extended to L4. PMID:25558324

  11. Abnormal osteogenic and chondrogenic differentiation of human mesenchymal stem cells from patients with adolescent idiopathic scoliosis in response to melatonin

    PubMed Central

    Chen, Chong; Xu, Caixia; Zhou, Taifeng; Gao, Bo; Zhou, Hang; Chen, Changhua; Zhang, Changli; Huang, Dongsheng; Su, Peiqiang

    2016-01-01

    Abnormalities of membranous and endochondral ossification in patients with adolescent idiopathic scoliosis (AIS) remain incompletely understood. To investigate abnormalities in the melatonin signaling pathway and cellular response to melatonin in AIS, a case-control study of osteogenic and chondrogenic differentiation was performed using human mesenchymal stem cells (hMSCs). AIS was diagnosed by physical and radiographic examination. hMSCs were isolated from the bone marrow of patients with AIS and control subjects (n=12 each), and purified by density gradient centrifugation. The expression levels of melatonin receptors (MTs) 1 and 2 were detected by western blotting. Osteogenic and chondrogenic differentiation was induced by culturing hMSCs in osteogenic and chondrogenic media containing vehicle or 50 nM melatonin. Alkaline phosphatase (ALP) activity assays, quantitative glycosaminoglycan (GAG) analysis, and reverse transcription-quantitative polymerase chain reaction analysis were performed. Compared with controls, MT2 demonstrated low expression in the AIS group. Melatonin increased ALP activity, GAG synthesis and upregulated the expression of genes involved in osteogenic and chondrogenic differentiation including, ALP, osteopontin, osteocalcin, runt-related transcription factor 2, collagen type II, collagen type X, aggrecan and sex-determining region Y-box 9 in the normal control hMSCs, but did not affect the AIS groups. Thus, AIS hMSCs exhibit abnormal cellular responses to melatonin during osteogenic and chondrogenic differentiation, which may be associated with abnormal membranous and endochondral ossification, and skeletal growth. These results indicate a potential modulating role of melatonin via the MT2 receptor on abnormal osteogenic and chondrogenic differentiaation in patients with AIS. PMID:27314307

  12. Radiological and clinical outcome of screw placement in adolescent idiopathic scoliosis: evaluation with low-dose computed tomography

    PubMed Central

    Ohlin, Acke; Strömbeck, Anita; Maly, Pavel; Sundgren, Pia C.

    2009-01-01

    Posterior corrective surgery using “all pedicle screw construct” carries risk of neurovascular complications. The study aims were to assess the screw placement in patients with adolescent idiopathic scoliosis using CT with low-radiation dose, and to evaluate the clinical outcome in patients with misplaced pedicle screws. CTs of 49 consecutive patients (873 screws, 79% thoracic) were retrospectively evaluated by two independent radiologists. A new grading system was developed to distinguish between lateral, medial and anterior cortical perforations, endplate perforation and foraminal perforation. The grading system is based on whether the cortical violation is partial or total rather than on mm-basis. The overall rate of screw misplacement was 17% (n = 149): 8% were laterally placed and 6.1% were medially placed. The rates of anterior cortical, endplate and foraminal perforation were 1.5, 0.9, and 0.5%, respectively. Lateral cortical perforation was more frequent in the thoracic spine (P = 0.005), whereas other types of misplacement including medial cortical perforation were more frequent on the left and the concave side of scoliotic curves (P = 0.002 and 0.003). No neurovascular complications were reported. The association between the occurrence of screw misplacement and the Cobb angle was statistically significant (P = 0.037). Misplacements exceeding half screw diameter should be classified as unacceptable. Low-dose CT implies exposing these young individuals to a significantly lower radiation dose than do other protocols used in daily clinical practice. We recommend using low-dose CT and the grading system proposed here in the postoperative assessment of screw placement. PMID:19888607

  13. Factors favoring regain of the lost vertical spinal height through posterior spinal fusion in adolescent idiopathic scoliosis

    PubMed Central

    Shi, Benlong; Mao, Saihu; Xu, Leilei; Sun, Xu; Liu, Zhen; Zhu, Zezhang; Lam, Tsz Ping; Cheng, Jack CY; Ng, Bobby; Qiu, Yong

    2016-01-01

    Height gain is a common beneficial consequence following correction surgery in adolescent idiopathic scoliosis (AIS), yet little is known concerning factors favoring regain of the lost vertical spinal height (SH) through posterior spinal fusion. A consecutive series of AIS patients from February 2013 to August 2015 were reviewed. Surgical changes in SH (ΔSH), as well as the multiple coronal and sagittal deformity parameters were measured and correlated. Factors associated with ΔSH were identified through Pearson correlation analysis and multivariate regression analysis. A total of 172 single curve and 104 double curve patients were reviewed. The ΔSH averaged 2.5 ± 0.9 cm in single curve group and 2.9 ± 1.0 cm in double curve group. The multivariate regression analysis revealed the following pre-operative variables contributed significantly to ΔSH: pre-op Cobb angle, pre-op TK (single curve group only), pre-op GK (double curve group only) and pre-op LL (double curve group only) (p < 0.05). Thus change in height (in cm) = 0.044 × (pre-op Cobb angle) + 0.012 × (pre-op TK) (Single curve, adjusted R2 = 0.549) or 0.923 + 0.021 × (pre-op Cobb angle1) + 0.028 × (pre-op Cobb angle2) + 0.015 × (pre-op GK)-0.012 × (pre-op LL) (Double curve, adjusted R2 = 0.563). Severer pre-operative coronal Cobb angle and greater sagittal curves were beneficial factors favoring more contribution to the surgical lengthening effect in vertical spinal height in AIS. PMID:27373798

  14. Foot function is well preserved in children and adolescents with juvenile idiopathic arthritis who are optimally managed

    PubMed Central

    Hendry, Gordon J.; Rafferty, Danny; Barn, Ruth; Gardner-Medwin, Janet; Turner, Debbie E.; Woodburn, James

    2013-01-01

    Purpose The objective of this study was to compare disease activity, impairments, disability, foot function and gait characteristics between a well described cohort of juvenile idiopathic arthritis (JIA) patients and normal healthy controls using a 7-segment foot model and three-dimensional gait analysis. Methods Fourteen patients with JIA (mean (standard deviation) age of 12.4 years (3.2)) and a history of foot disease and 10 healthy children (mean (standard deviation) age of 12.5 years (3.4)) underwent three-dimensional gait analysis and plantar pressure analysis to measure biomechanical foot function. Localised disease impact and foot-specific disease activity were determined using the juvenile arthritis foot disability index, rear- and forefoot deformity scores, and clinical and musculoskeletal ultrasound examinations respectively. Mean differences between groups with associated 95% confidence intervals were calculated using the t distribution. Results Mild-to-moderate foot impairments and disability but low levels of disease activity were detected in the JIA group. In comparison with healthy subjects, minor trends towards increased midfoot dorsiflexion and reduced lateral forefoot abduction within a 3–5° range were observed in patients with JIA. The magnitude and timing of remaining kinematic, kinetic and plantar pressure distribution variables during the stance phase were similar for both groups. Conclusion In children and adolescents with JIA, foot function as determined by a multi-segment foot model did not differ from that of normal age- and gender-matched subjects despite moderate foot impairments and disability scores. These findings may indicate that tight control of active foot disease may prevent joint destruction and associated structural and functional impairments. PMID:23142184

  15. Effects of Multilevel Facetectomy and Screw Density on Postoperative Changes in Spinal Rod Contour in Thoracic Adolescent Idiopathic Scoliosis Surgery.

    PubMed

    Kokabu, Terufumi; Sudo, Hideki; Abe, Yuichiro; Ito, Manabu; Ito, Yoichi M; Iwasaki, Norimasa

    2016-01-01

    Flattening of the preimplantation rod contour in the sagittal plane influences thoracic kyphosis (TK) restoration in adolescent idiopathic scoliosis (AIS) surgery. The effects of multilevel facetectomy and screw density on postoperative changes in spinal rod contour have not been documented. This study aimed to evaluate the effects of multilevel facetectomy and screw density on changes in spinal rod contour from before implantation to after surgical correction of thoracic curves in patients with AIS prospectively. The concave and convex rod shapes from patients with thoracic AIS (n = 49) were traced prior to insertion. Postoperative sagittal rod shape was determined by computed tomography. The angle of intersection of the tangents to the rod end points was measured. Multiple stepwise linear regression analysis was used to identify variables independently predictive of change in rod contour (Δθ). Average Δθ at the concave and convex side were 13.6° ± 7.5° and 4.3° ± 4.8°, respectively. The Δθ at the concave side was significantly greater than that of the convex side (P < 0.0001) and significantly correlated with Risser sign (P = 0.032), the preoperative main thoracic Cobb angle (P = 0.031), the preoperative TK angle (P = 0.012), and the number of facetectomy levels (P = 0.007). Furthermore, a Δθ at the concave side ≥14° significantly correlated with the postoperative TK angle (P = 0.003), the number of facetectomy levels (P = 0.021), and screw density at the concave side (P = 0.008). Rod deformation at the concave side suggests that corrective forces acting on that side are greater than on the convex side. Multilevel facetectomy and/or screw density at the concave side have positive effects on reducing the rod deformation that can lead to a loss of TK angle postoperatively. PMID:27564683

  16. Chinese Adaptation of the Bad Sobernheim Stress Questionnaire for Patients With Adolescent Idiopathic Scoliosis Under Brace Treatment.

    PubMed

    Xu, Ximing; Wang, Fei; Yang, Mingyuan; Huang, Qikai; Chang, Yifan; Wei, Xianzhao; Bai, Yushu; Li, Ming

    2015-08-01

    Bad Sobernheim Stress Questionnaire (BSSQ)-Deformity and BSSQ-Brace are the most widely used instruments for evaluating stress levels in adolescent idiopathic scoliosis (AIS) patients under brace treatment, and good reliability and validity have been demonstrated across different cultures. Great stress has been found among many adolescents, becoming a major concern for professionals. However, no previous research has addressed the cultural adaptations and psychometric testing of BSSQ-Deformity and BSSQ-Brace in China or the stress levels in AIS patients. The purposes of our study were to evaluate the cross-cultural adaptation and validation of the BSSQ-Deformity and BSSQ-Brace and to investigate stress levels in Chinese (AIS) patients under brace treatment.The original (German) versions of BSSQ-Deformity and BSSQ-Brace were cross-culturally translated according to international guidelines. Psychometric properties such as reliability and construct validity were tested. Eighty-six AIS patients were included in our study, and 50 patients paid a second visit 3 to 7 days later to test reproducibility. Cronbach α and the intraclass coefficient were determined to assess internal consistency and reproducibility. Scoliosis Research Society patient questionnaire-22 (SRS-22) was applied to evaluate construct validity.The mean BSSQ-Deformity and BSSQ-Brace scores were 15.3 and 13.4 points, respectively. Severe stress was observed in 12% of patients due to brace treatment. Item analysis demonstrated that each item was scored under a normal distribution with no redundancy. Psychometric analysis revealed excellent internal consistency (Cronbach α = 0.85 and 0.80, respectively) and reproducibility (intraclass correlation coefficient = 0.85 and 0.90, respectively) for BSSQ-Deformity and BSSQ-Brace. The correlation coefficients of BSSQ-Deformity, BSSQ-Brace and SRS-22 were 0.48 and 0.63, respectively.In conclusion, BSSQ-Deformity and BSSQ-Brace have been successfully

  17. Association of Body Composition with Curve Severity in Children and Adolescents with Idiopathic Scoliosis (IS)

    PubMed Central

    Matusik, Edyta; Durmala, Jacek; Matusik, Pawel

    2016-01-01

    The link between scoliotic deformity and body composition assessed with bioimpedance (BIA) has not been well researched. The objective of this study was to correlate the extent of scoliotic-curve severity with the anthropometrical status of patients with idiopathic scoliosis (IS) based on standard anthropometric measurements and BIA. The study encompassed 279 IS patients (224 girls/55 boys), aged 14.21 ± 2.75 years. Scoliotic curve severity assessed by Cobb’s angle was categorized as moderate (10°–39°) or severe (≥40°). Corrected height, weight, waist and hip circumferences were measured and body mass index (BMI), corrected height z-score, BMI Z-score, waist/height ratio (WHtR) and waist/hip ratio (WHR) were calculated for the entire group. Body composition parameters: fat mass (FAT), fat-free mass (FFM) and predicted muscle mass (PMM) were determined using a bioelectrical impedance analyzer. The mean Cobb angle was 19.96° ± 7.92° in the moderate group and 52.36° ± 12.54° in the severe group. The corrected body heights, body weights and BMIs were significantly higher in the severe IS group than in the moderate group (p < 0.05). Significantly higher FAT and lower FFM and PMM were observed in the severe IS group (p < 0.05). The corrected heights and weights were significantly higher in patients with severe IS and normal weight (p < 0.01). Normal and overweight patients with a severe IS had significantly higher adiposity levels assessed by FAT, FFM and PMM for normal and BMI, BMI z-score, WHtR, FAT and PMM for overweight, respectively. Overweight IS patients were significantly younger and taller than underweight and normal weight patients. The scoliotic curve severity is significantly related to the degree of adiposity in IS patients. BMI z-score, WHtR and BIA seem to be useful tools for determining baseline anthropometric characteristics of IS children. PMID:26828519

  18. Association of Body Composition with Curve Severity in Children and Adolescents with Idiopathic Scoliosis (IS).

    PubMed

    Matusik, Edyta; Durmala, Jacek; Matusik, Pawel

    2016-02-01

    The link between scoliotic deformity and body composition assessed with bioimpedance (BIA) has not been well researched. The objective of this study was to correlate the extent of scoliotic-curve severity with the anthropometrical status of patients with idiopathic scoliosis (IS) based on standard anthropometric measurements and BIA. The study encompassed 279 IS patients (224 girls/55 boys), aged 14.21 ± 2.75 years. Scoliotic curve severity assessed by Cobb's angle was categorized as moderate (10°-39°) or severe (≥40°). Corrected height, weight, waist and hip circumferences were measured and body mass index (BMI), corrected height z-score, BMI Z-score, waist/height ratio (WHtR) and waist/hip ratio (WHR) were calculated for the entire group. Body composition parameters: fat mass (FAT), fat-free mass (FFM) and predicted muscle mass (PMM) were determined using a bioelectrical impedance analyzer. The mean Cobb angle was 19.96° ± 7.92° in the moderate group and 52.36° ± 12.54° in the severe group. The corrected body heights, body weights and BMIs were significantly higher in the severe IS group than in the moderate group (p < 0.05). Significantly higher FAT and lower FFM and PMM were observed in the severe IS group (p < 0.05). The corrected heights and weights were significantly higher in patients with severe IS and normal weight (p < 0.01). Normal and overweight patients with a severe IS had significantly higher adiposity levels assessed by FAT, FFM and PMM for normal and BMI, BMI z-score, WHtR, FAT and PMM for overweight, respectively. Overweight IS patients were significantly younger and taller than underweight and normal weight patients. The scoliotic curve severity is significantly related to the degree of adiposity in IS patients. BMI z-score, WHtR and BIA seem to be useful tools for determining baseline anthropometric characteristics of IS children. PMID:26828519

  19. Leisure in Children and Adolescents with Juvenile Idiopathic Arthritis: A Systematic Review

    PubMed Central

    Cavallo, Sabrina; April, Karine Toupin; Grandpierre, Viviane; Majnemer, Annette; Feldman, Debbie Ehrmann

    2014-01-01

    The aim of this systematic review is to describe participation in social and physical leisure activities among children and adolescents with JIA, as well as identify potential determinants of leisure participation. Methods Electronic databases were systematically searched for articles published up until June 2013 pertaining to participation in leisure activities among youth with JIA and other rheumatic diseases. Studies were included if they measured involvement in either social or physical leisure activities. Selection and quality appraisal of articles were completed independently by two authors. Results Eight hundred and ninety-three articles were found through electronic and reference search. One hundred and nine full articles were reviewed to assess for eligibility. Twelve articles met inclusion criteria and findings were reviewed. Most focused on describing participation in physical rather than social activities. Results suggest that youth with JIA participated less in both social and physical leisure activities as compared to healthy peers, and those with JIA did not meet national recommendations for physical activity. Potential determinants of leisure participation were socio-demographic (age, sex), anthropometric (height, weight) and disease-related (JIA subtype, disease duration, pain, number of swollen or painful joints, stiffness, fatigue, well-being) factors. Conclusion Characterization of leisure activity remains limited and mostly focused on physical activity in JIA. Assessment of more comprehensive outcome measures is warranted to obtain a better description of leisure in this population. Evidence of the influence of contextual factors as potential determinants of involvement in leisure among children with pediatric rheumatologic diseases is needed. PMID:25329390

  20. "Bone-οn-Bone" surgical reconstruction of moderate severity, flexible single curve adolescent idiopathic scoliosis: continuing improvements of the technique and results in three scoliosis centers after almost twenty years of use.

    PubMed

    Gaines, Robert W; Min, Kan; Zarzycki, Daniel

    2015-01-01

    The "bone-on-bone" reconstruction for adolescent idiopathic scoliosis is reviewed in this article. Extensive use over the past 18 years has identified it's functional benefits outstanding clinical results, and very limited complications. This is an extensive update of it's application, since it's introduction, 18 years ago. PMID:26000031

  1. Plasma levels of tumor necrosis factor-α in adolescent idiopathic scoliosis patients serve as a predictor for the incidence of early postoperative cognitive dysfunction following orthopedic surgery

    PubMed Central

    ZHENG, XU; MA, ZHENGLIANG; GU, XIAOPING

    2015-01-01

    The aim of the present study was to evaluate whether the levels of the plasma biomarkers, cortisol, interleukin (IL)-1β, IL-6, IL-10 and tumor necrosis factor (TNF)-α, change in adolescent idiopathic scoliosis patients with postoperative cognitive dysfunction (POCD); thus, may serve as predictive factors for POCD. In total, 75 adolescent scoliosis patients, aged between 11 and 18 years and categorized as American Society of Anesthesiologists classification I or II, were scheduled for orthopedic surgery with general anesthesia. Blood samples were collected on the day of admission and at day 2 following surgery. The plasma levels of IL-1β, IL-6, IL-10 and TNF-α were measured using an enzyme-linked immunosorbent assay, while the concentrations of cortisol were determined using a radioimmunoassay. Cognitive function was assessed one day prior to and at day 7 following the surgery in a quiet room with the guidance of a Chinese version protocol. In total, 66 patients completed the sample collection and neurocognitive tests. According to the criteria for the diagnosis of POCD, 19 patients (28.8%) developed POCD within seven days of surgery. No statistically significant differences were observed in the baseline concentrations of cortisol, TNF-α, IL-1β, IL-10 or IL-6 between the POCD and non-POCD groups. However, the baseline plasma level of TNF-α on day 2 in the POCD group was found to be higher compared with the non-POCD group. In addition, at day 2 after surgery, the concentration of cortisol in the non-POCD group was higher compared with the POCD group. Therefore, the plasma level of TNF-α in adolescent idiopathic scoliosis patients at day 2 following orthopedic surgery may be a predictor for the incidence of early POCD. PMID:25780449

  2. Effectiveness of the SpineCor brace based on the new standardized criteria proposed by the scoliosis research society for adolescent idiopathic scoliosis.

    PubMed

    Coillard, Christine; Vachon, Valerie; Circo, Alin B; Beauséjour, Marie; Rivard, Charles H

    2007-06-01

    The purpose of this prospective observational study was to evaluate the effectiveness of the Dynamic SpineCor brace for adolescent idiopathic scoliosis in accordance with the standardized criteria proposed by the Scoliosis Research Society Committee on Bracing and Nonoperative Management. They proposed these guidelines to make the comparison among studies more valid and reliable. From 1993 to 2006, 493 patients were treated using the SpineCor brace. Two hundred forty-nine patients met the criteria for inclusion, and 79 patients were still actively being treated. Overall, 170 patients have a definitive outcome. All girls were premenarchal or less than 1 year postmenarchal. Assessment of brace effectiveness included (1) percentage of patients who have 5 degrees or less curve progression, and percentage of patients who have 6 degrees or more progression; (2) percentage of patients who have been recommended/undergone surgery before skeletal maturity; (3) percentage of patients with curves exceeding 45 degrees at maturity (end of treatment); and (4) Two-year follow-up beyond maturity to determine the percentage of patients who subsequently underwent surgery. Successful treatment (correction, >5 degrees, or stabilization, +/-5 degrees) was achieved in 101 (59.4%) of the 170 patients from the time of the fitting of the SpineCor brace to the point in which it was discontinued. Thirty-nine immature patients (22.9%) required surgical fusion while receiving treatment. Two (1.2%) of 170 patients had curves exceeding 45 degrees at maturity. One mature patient (2.1%) required surgery within 2 years of follow-up beyond skeletal maturity. The conclusion drawn from these findings is that the SpineCor brace is effective for the treatment of adolescent idiopathic scoliosis. Moreover, positive outcomes are maintained after 2 years because 45 (95.7%) of 47 patients stabilized or corrected their end of bracing Cobb angle up to 2 years after bracing. Therapeutic study-investigating the

  3. Adolescent Idiopathic Scoliosis

    MedlinePlus

    ... 中文 فارسی français deutsche Ελληνικά Italiano 日本語 한국어 português español Türkçe Member Login Become a Member Find ... Patients and Families Professionals About SRS Türkçe español português 한국어 日本語 Ελληνικά deutsche Italiano français فارسی 中文 ...

  4. Idiopathic anaphylaxis.

    PubMed

    Greenberger, Paul A

    2007-05-01

    Idiopathic anaphylaxis is a prednisone-responsive condition without external cause, but it can coexist with food-, medication-, or exercise-induced anaphylaxis. Mast cell activation may occur at night or after foods that have been eaten with impunity many times previously. Idiopathic anaphylaxis can be classified into frequent (if there are six or more episodes per year or two episodes in the last 2 months) or infrequent (if episodes occur less often). Idiopathic anaphylaxis-generalized consists of urticaria or angioedema associated with severe respiratory distress, syncope or hypotension, and gastrointestinal symptoms. Idiopathic anaphylaxis-angioedema consists of massive tongue enlargement or severe pharyngeal or laryngeal swelling with urticaria or peripheral angioedema. The differential diagnosis of idiopathic anaphylaxis is reviewed, and treatment approaches are presented. PMID:17493503

  5. Identification of candidate diagnostic biomarkers for adolescent idiopathic scoliosis using UPLC/QTOF-MS analysis: a first report of lipid metabolism profiles

    PubMed Central

    Sun, Zhi-jian; Jia, Hong-mei; Qiu, Gui-xing; Zhou, Chao; Guo, Shigong; Zhang, Jian-guo; Shen, Jian-xiong; Zhao, Yu; Zou, Zhong-mei

    2016-01-01

    Adolescent idiopathic scoliosis (AIS) is a complex spine deformity, affecting approximately 1–3% adolescents. Earlier diagnosis could increase the likelihood of successful conservative treatment and hence reduce the need for surgical intervention. We conducted a serum metabonomic study to explore the potential biomarkers of AIS for early diagnosis. Serum metabolic profiles were firstly explored between 30 AIS patients and 31 healthy controls by ultra high-performance liquid chromatography coupled with quadrupole time-of-flight mass spectrometry. Then, the candidate metabolites were validated in an independent cohort including 31 AIS patients and 44 controls. The results showed that metabolic profiles of AIS patients generally deviated from healthy controls in both the discovery set and replication set. Seven differential metabolites were identified as candidate diagnostic biomarkers, including PC(20:4), 2-hexenoylcarnitine, beta-D-glucopyranuronicacid, DG(38:9), MG(20:3), LysoPC(18:2) and LysoPC(16:0). These candidate metabolites indicated disrupted lipid metabolism in AIS, including glycerophospholipid, glycerolipid and fatty acid metabolism. Elevated expressions of adipose triglyceride lipase and hormone sensitive lipase in adipose tissue further corroborated our findings of increased lipid metabolism in AIS. Our findings suggest that differential metabolites discovered in AIS could be used as potential diagnostic biomarkers and that lipid metabolism plays a role in the pathogenesis of AIS. PMID:26928931

  6. Idiopathic hypersomnia

    MedlinePlus

    ... page, please enable JavaScript. Idiopathic hypersomnia is a sleep disorder in which a person is excessively sleepy ( hypersomnia ) ... other potential causes of excessive daytime sleepiness. Other sleep disorders that may cause daytime sleepiness include: Narcolepsy Obstructive ...

  7. Lack of association between the CHL1 gene and adolescent idiopathic scoliosis susceptibility in Han Chinese: a case-control study

    PubMed Central

    2014-01-01

    Background A previous genome-wide association study (GWAS) suggested a strong association between the single nucleotide polymorphism (SNP) rs10510181 in the proximity of the gene encoding a cell adhesion molecule with homology to L1CAM (CHL1) and adolescent idiopathic scoliosis (AIS) in Caucasians. To clarify the role of CHL1 in the etiopathogenesis of AIS, we performed a case-control replication study in a Han Chinese population. Methods Five hundred female AIS patients between 10 and 18 years of age, as well as 500 age- and sex-matched controls were included. This study was conducted as a 2-stage case-control analysis: initial screening for the association between AIS and SNPs in and around the CHL1 gene (186 cases and 169 controls) followed by a confirmation test (314 cases and 331 controls). rs10510181 and 4 SNPs (rs2055314, rs331894, rs2272522, and rs2272524) in the CHL1 gene were selected for genotyping. Results Putative associations were shown between AIS and rs10510181, rs2055314, and rs2272522 in stage I. However, the associations were not confirmed in stage II. For rs10510181, the genotype frequencies were GG 28.8%, GA 46.2%, and AA 25.0% in AIS patients and GG 29.8%, GA 48.8%, and AA 21.4% in controls. No significant difference was found in genotype distribution between cases and controls (P = 0.39). Similarly, the genotype and allele distribution were comparable between case and control for rs2055314 and rs2272522. Conclusions There was no statistical association between polymorphisms of the CHL1 gene and idiopathic scoliosis in a Chinese population. PMID:24512353

  8. Comparative Analysis of Interval, Skipped, and Key-vertebral Pedicle Screw Strategies for Correction in Patients With Lenke Type 1 Adolescent Idiopathic Scoliosis.

    PubMed

    Wang, Fei; Xu, Xi-Ming; Lu, Yanghu; Wei, Xian-Zhao; Zhu, Xiao-Dong; Li, Ming

    2016-03-01

    Pedicle screw constructs have become the mainstay for surgical correction in patients with spinal deformities. To reduce or avoid the risk of pedicle screw-based complications and to decrease the costs associated with pedicle screw instrumentation, some authors have introduced interval, skipped, and key-vertebral pedicle screw strategies for correction. However, there have been no comparisons of outcomes among these 3 pedicle screw-placement strategies.The aim of this study was to compare the correlative clinical outcomes of posterior correction and fusion with pedicle screw fixation using these 3 surgical strategies.Fifty-six consecutive patients with Lenke type 1 adolescent idiopathic scoliosis were included in this study. Twenty patients were treated with the interval pedicle screw strategy (IPSS), 20 with the skipped pedicle screw strategy (SPSS), and 16 with the key-vertebral pedicle screw strategy (KVPSS). Coronal and sagittal radiographs were analyzed before surgery, at 1 week after surgery, and at the last follow-up after surgery.There were no significant differences among the 3 groups regarding preoperative radiographic parameters. No significant difference was found between the IPSS and SPSS groups in correction of the main thoracic curve (70.8% vs 70.0%; P = 0.524). However, there were statistically significant differences between the IPSS and KVPSS groups (70.8% vs 64.9%) and between the SPSS and KVPSS groups (70.0% vs 64.9%) in correction of the main thoracic curve (P < 0.001 for both). Additionally, there were no significant differences among the 3 strategies for sagittal parameters at the immediate postoperative and last postoperative follow-up periods, though there were significant differences in the Cobb angle between the preoperative and immediate postoperative periods among the 3 groups, but not between the immediate postoperative and last follow-up periods. The amount of hospital charges in the SPSS group was significantly higher than

  9. [Juvenile idiopathic epiretinal membrane].

    PubMed

    Kontopoulou, K; Krause, S; Fili, S; Hayvazov, S; Schilling, H; Kohlhaas, M

    2016-07-01

    Idiopathic epiretinal membrane (iERM) is very rare in adolescent patients. The pathogenesis remains unclear although the role of hyalocytes is of major importance. The clinical features in young patients are different from those in older patients. We describe a case of iERM in a 15-year-old girl who presented with metamorphopsia of the right eye. This case report presents the basis for the decision for surgical treatment as well as the clinical features at follow-up examination 9 months after surgery. PMID:26458892

  10. Indication for surgical treatment in patients with adolescent idiopathic scoliosis – a critical appraisal and counter-point

    PubMed Central

    2013-01-01

    In a recent letter to the editor (Patient Saf. Surg. 2013, 7:17), Weiss and Moramarco made the claim that surgical correction of idiopathic scoliosis is not supported based on the available literature citing no medical indication and high complication rates as compared to non-operative management. In this letter we show that there is a role for surgical treatment as the only predictable option to obtain correction of a curve and that the risk of complications with newer instrumentation does not approach 50% as cited by Weiss and Moramarco. We share the opinion with Weiss and Moramarco that a decision for surgery is not one to be made lightly, and should include options of purely observation, bracing, as well as surgery depending on the potential progression profile and conversations with the child and parents. PMID:23819754

  11. Changes of concave and convex rib-vertebral angle, angle difference and angle ratio in patients with right thoracic adolescent idiopathic scoliosis.

    PubMed

    Canavese, Federico; Turcot, Katia; Holveck, Jerôme; Farhoumand, Agnés Dahl; Kaelin, André

    2011-01-01

    The aim of this study is to describe the radiological changes in rib-vertebral angles (RVAs), rib-vertebral angle differences (RVADs), and rib-vertebral angle ratios (RVARas) in patients with untreated right thoracic adolescent idiopathic scoliosis and to compare with the normal subjects. The concave and convex RVA from T1 to T12, the RVADs and the RVARas were measured on AP digital radiographs of 44 female patients with right convex idiopathic scoliosis and 14 normal females. Patients were divided into three groups: normal subjects (group 1), scoliotic patients with Cobb's angle equal or <30° (group 2) and scoliotic patients with Cobb's angle over 30° (group 3). Overall values (mean ± SD) of the RVAs on the concave side were 90.5° ± 17° in group 1, 90.3° ± 15.8° in group 2 and 88.8° ± 15.4° in group 3. On the convex side, values were 90.0° ± 17.3° in group 1, 86.3° ± 13.7° in group 2 and 80.7° ± 14.4° in group 3. Overall values (mean ± SD) of the RVADs at all levels were 0.5° ± 0.7° in group 1, 4.0° ± 4.8° in group 2 and 8.0° ± 4.0° in group 3. The RVARa values (mean ± SD) at all levels was 1.008° ± 0.012° in group 1, 1.041° ± 0.061° in group 2 and 1.102° ± 0.151° in group 3. RVAD and RVARa values in the scoliotic segment were greater in patients with untreated scoliosis over 30° than in patients with an untreated deformity of <30° or normal subjects. A significant effect between groups was observed for the RVA, RVAD and RVARa variables. Measurement of RVA, RVAD and RVARa should not only be performed at and around the apex of a thoracic spinal deformity, but also extended to the whole thoracic spine. PMID:20811755

  12. Are Volumetric Bone Mineral Density and Bone Micro-Architecture Associated with Leptin and Soluble Leptin Receptor Levels in Adolescent Idiopathic Scoliosis? – A Case-Control Study

    PubMed Central

    Tam, Elisa M. S.; Yu, Fiona W. P.; Hung, Vivian W. Y.; Liu, Zhen; Liu, King Lok; Ng, Bobby K. W.; Lee, Simon K. M.; Qiu, Yong; Cheng, Jack C. Y.; Lam, Tsz-Ping

    2014-01-01

    Background Adolescent idiopathic scoliosis (AIS) is associated with low bone mineral density (BMD). The underlying etiology and how it may relate to the development of osteopenia remains unknown. Leptin has been postulated as one of the etiologic factors of AIS because of its profound effects on bone metabolism and pubertal growth. Its modulator, soluble leptin receptor (sOB-R), may affect leptin bioavailability and signaling. This study aimed to investigate whether serum leptin and sOB-R levels may be associated with bone quality, and whether these relationships may differ between young adolescent girls with and without AIS. Methods This was a case-control study involving 94 newly diagnosed AIS girls (Cobb angle 12–48°) aged 12 to 14 years old and 87 age and gender-matched normal controls. Subjects with BMI>23.0 Kg/m2 were excluded. Anthropometric measurements including body weight, height, arm span and sitting height were taken. Serum total leptin and sOB-R were assayed with ELISA. Non-dominant distal radius was scanned with High Resolution pQCT for assessing bone quality in terms of bone morphometry, volumetric BMD (vBMD) and trabecular bone micro-architecture. Results Compared with normal controls, AIS girls had numerically higher sOB-R (p = 0.006), lower average vBMD (p = 0.048), lower cortical vBMD (p = 0.029), higher cortical bone perimeter (p = 0.014) and higher trabecular area (p = 0.027), but none remained statistically significant after the Hochberg-Benjamini procedure. Correlation analysis on serum leptin level indicated that distinctive correlations with trabecular bone parameters occurred only in AIS. Conclusion This study showed that bone quality in AIS girls was deranged as compared with controls. In addition, the distinct differences in correlation pattern between leptin and trabecular bone parameters indicated possible abnormalities in bone metabolism and dysfunction of the leptin signaling pathway in AIS. PMID:24516571

  13. Tuberculin skin test and ELISPOT/T. SPOT.TB in children and adolescents with juvenile idiopathic arthritis

    PubMed Central

    2014-01-01

    Background There are controversies regarding the accuracy of the tuberculin skin test (TST) and methods based on the production of interferon gamma by sensitized T cells for the diagnosis of latent tuberculosis infection (LTBI) in pediatrics and immunosuppressed patients. Our objectives are to study TST and ELISPOT/T. SPOT.TB in the diagnosis of LTBI in children and adolescents with JIA undergoing methotrexate, the correlation between both and the sensitivity and specificity of T. SPOT.TB. Methods This is an observational prospective longitudinal study in which children and adolescents with JIA undergoing methotrexate therapy were assessed for clinical and epidemiological data for LTBI, in addition to performing TST and T. SPOT.TB at baseline and after 3 and 12months. Results There were 24 patients. The prevalence of LTBI at inclusion was 20.8%, the incidence after initiation of immunosuppressions 26.3% and the prevalence at the end of the study 41.6%. Epidemiological history positive for TB showed a relative risk of 2.0 for the development of LTBI. Only 2 patients had positive T. SPOT.TB but only in one it was useful for detecting early LTBI. T. SPOT.TB presented a sensitivity of 10%, specificity of 92.8%, and low correlation with TST. No patient developed TB disease at a mean follow-up of 47months. Conclusions We found a high prevalence of ILTB that doubled with immunosuppression and that epidemiological history was an important relative risk. T. SPOT.TB showed low sensitivity and high specificity, and no superiority over TST. There was low agreement and little influence of immunosuppression on the results of both tests. PMID:24904240

  14. Evaluation of implant loosening following segmental pedicle screw fixation in adolescent idiopathic scoliosis: a 2 year follow-up with low-dose CT

    PubMed Central

    2014-01-01

    Background The long term radiological status of screw fixation following scoliosis surgery with all pedicle screw construct is not previously studied. Aim To evaluate the incidence of loosening (implant failure) evaluated with low-dose CT two years following scoliosis surgery. Study design Retrospective study. Methods 81 consecutive patients with adolescent idiopathic scoliosis (AIS), aged 18 ± 3 years at 2 years follow-up (83% were female), subjected for scoliosis corrective surgery with all pedicle screw construct (total of 1666 screws) has been examined with plain radiography and with low dose CT 6 weeks and 2 years postoperatively. Results In 26 out of 81 (32%) patients there were signs of loosening of one or more screws, a maximum 3 screws. 47 out of 1666 (2.8%) screws showed evidence of loosening. Preoperative Cobb angle was 56° among patients with loosening compared with 53° among patients with no evidence of loosening (P = 0.288). In males there were signs of loosening in 8 out of 14 (57%) and in females 18 out of 67 (27%), (P = 0.027). Among cases with loosening, 14% had suboptimal screw placement at the first postoperative CT compared with 11% among patients with no evidence of loosening (P = 0.254). One patient with a loosened L4 screw had neurological deficit and subjected for revision of the construct. Out of 26 patients with evidence of loosening, 5 patients reported minor pain or discomfort, 1 patient had a minor proximal junctional kyphosis of about 15° and 3 patients showed evidence of pull-out of 3–5 mm at the upper end of the construct but no clinical complaint. With plain radiography loosening could be observed only in 11 out of 26 cases, 5 were in the lumbar region. Conclusions In a consecutive series of 81 cases with AIS who had underwent scoliosis surgery, one third showed, 2 years after the intervention, minor screw loosening. Males were more prone to develop screw loosening. In CT system that enables low

  15. Functional Investigation of a Non-coding Variant Associated with Adolescent Idiopathic Scoliosis in Zebrafish: Elevated Expression of the Ladybird Homeobox Gene Causes Body Axis Deformation

    PubMed Central

    Guo, Long; Yamashita, Hiroshi; Kou, Ikuyo; Takimoto, Aki; Meguro-Horike, Makiko; Horike, Shin-ichi; Sakuma, Tetsushi; Miura, Shigenori; Adachi, Taiji; Yamamoto, Takashi; Ikegawa, Shiro; Hiraki, Yuji; Shukunami, Chisa

    2016-01-01

    Previously, we identified an adolescent idiopathic scoliosis susceptibility locus near human ladybird homeobox 1 (LBX1) and FLJ41350 by a genome-wide association study. Here, we characterized the associated non-coding variant and investigated the function of these genes. A chromosome conformation capture assay revealed that the genome region with the most significantly associated single nucleotide polymorphism (rs11190870) physically interacted with the promoter region of LBX1-FLJ41350. The promoter in the direction of LBX1, combined with a 590-bp region including rs11190870, had higher transcriptional activity with the risk allele than that with the non-risk allele in HEK 293T cells. The ubiquitous overexpression of human LBX1 or either of the zebrafish lbx genes (lbx1a, lbx1b, and lbx2), but not FLJ41350, in zebrafish embryos caused body curvature followed by death prior to vertebral column formation. Such body axis deformation was not observed in transcription activator-like effector nucleases mediated knockout zebrafish of lbx1b or lbx2. Mosaic expression of lbx1b driven by the GATA2 minimal promoter and the lbx1b enhancer in zebrafish significantly alleviated the embryonic lethal phenotype to allow observation of the later onset of the spinal curvature with or without vertebral malformation. Deformation of the embryonic body axis by lbx1b overexpression was associated with defects in convergent extension, which is a component of the main axis-elongation machinery in gastrulating embryos. In embryos overexpressing lbx1b, wnt5b, a ligand of the non-canonical Wnt/planar cell polarity (PCP) pathway, was significantly downregulated. Injection of mRNA for wnt5b or RhoA, a key downstream effector of Wnt/PCP signaling, rescued the defective convergent extension phenotype and attenuated the lbx1b-induced curvature of the body axis. Thus, our study presents a novel pathological feature of LBX1 and its zebrafish homologs in body axis deformation at various stages of

  16. Accuracy of free-hand placement of thoracic pedicle screws in adolescent idiopathic scoliosis: how much of a difference does surgeon experience make?

    PubMed

    Samdani, Amer F; Ranade, Ashish; Sciubba, Daniel M; Cahill, Patrick J; Antonacci, M Darryl; Clements, David H; Betz, Randal R

    2010-01-01

    The use of thoracic pedicle screws for the treatment of adolescent idiopathic scoliosis (AIS) has gained widespread popularity. However, the placement of pedicle screws in the deformed spine poses unique challenges, and surgeons experience a learning curve. The in vivo accuracy as determined by computed tomography (CT) of placement of thoracic pedicle screws in the deformed spine as a function of surgeon experience is unknown. We undertook a retrospective review to determine the effect of surgeon experience on the accuracy of thoracic pedicle screw placement in AIS. In 2005, we started to obtain routine postoperative CT scans on patients undergoing a spinal fusion. From a database of these patients, we selected AIS patients, who underwent a posterior spinal fusion. Fifteen consecutive patients for each of the following three groups stratified by attending surgeon experience were selected (N = 45): A) less than 20 cases of all pedicle screw constructs for AIS (surgeons <2 years of practice), B) 20-50 cases (surgeons 2-5 years of practice), and C) greater than 50 cases (surgeons greater than 5 years of practice). Intraoperative evaluation of all screws included probing of the pedicle screw tract, neurophysiologic monitoring, and fluoroscopic confirmation. A total of 856 thoracic pedicle screws were studied. Postoperative CT scans were evaluated by two spine surgeons and a consensus read established as follows: (1) In: intraosseous placement or 2-mm breach, either medial or lateral. Of the 856 screws, 104 demonstrated a >2-mm breach, for an overall rate of 12.1% (medial = 55, lateral = 49, P = 0.67). When the breach rates were stratified by surgeon experience, there was a trend toward decreased rate of breach for the most experienced surgeons, although this did not attain statistical significance (Group A: 12.7%, Group B: 12.9%, Group C: 10.8%, P = 0.58). However, the most experienced group (C) had a markedly decreased rate of medial

  17. Uncoupled neuro-osseous growth in adolescent idiopathic scoliosis? A preliminary study of 90 adolescents with whole-spine three-dimensional magnetic resonance imaging.

    PubMed

    Lao, Li-Feng; Shen, Jian-Xiong; Chen, Zheng-Guang; Wang, Yi-Peng; Wen, Xi-Sheng; Qiu, Gui-Xing

    2011-07-01

    Previous literatures revealed abnormal cross-sectional morphology of spinal cord in AIS, suggesting the presence of disproportional growth between the neural and skeletal system. No accurate measurement of whole spine by MRI multiplanar reconstruction and their correlation with Cobb angle were studied. In this study, MRI three-dimensional reconstruction of the whole spine was performed in 90 adolescents (49 AIS with thoracic/thoracolumbar curve, and 41 age-matched healthy controls). Measurements of the ratio of anteroposterior (AP) and transverse (TS) diameter of the cord, the concave and convex lateral cord space (LCS) were obtained at the apical level in AIS patients. Cerebellar tonsillar level related to the basion-opsithion line, location of conus medullaris, cord length, vertebral column length, cord/vertebral column length ratio were obtained. All of the same parameters were also measured in healthy controls at matched vertebral levels and their correlations with Cobb angle were made. We notice that AP, TS, AP/TS and LCS ratio were increased in AIS subjects with low-lying position of cerebellar tonsillar level and elevating position of conus medullary when compared with healthy controls (P < 0.01). AP, AP/TS and LCS ratio were correlated significantly with Cobb angle (P < 0.05). Cord length and vertebral column length were not significantly different between AIS and control group. However, cord/vertebral column length ratio was significantly smaller in AIS group (P < 0.01). Cord length, vertebral column length and cord/vertebral column length ratio were not related with age or Cobb angle (P > 0.05). These data suggest the presence of uncoupled neuro-osseous growth along the longitudinal axis of spinal cord with associated morphologic changes of cross-sectional configuration and relative position of the cord. Some changes are significantly relevant with Cobb angle, which may indicate pathogenesis of AIS. PMID:20552379

  18. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... Is Juvenile Idiopathic Arthritis the same as Juvenile Rheumatoid Arthritis? Yes, Juvenile Idiopathic Arthritis (JIA) is a new ... of chronic inflammatory diseases that affect children. Juvenile Rheumatoid Arthritis (JRA) is the older term that was used ...

  19. Idiopathic cardiomegaly*

    PubMed Central

    1968-01-01

    Cardiomyopathies are certain heart diseases of unknown etiology and pathogenesis, occurring mostly in tropical and subtropical areas, where they constitute a major clinical problem and sometimes a public health problem. The need for international co-operation in the study of such forms of heart disease has long been recognized and WHO convened informal meetings of investigators on various aspects of the subject in 1964, 1965 and 1966. Out of these have arisen co-operative studies co-ordinated by WHO. In November 1967 a fourth informal meeting was held in Kingston, Jamaica, to review the following topics: the progress reports from all co-operating laboratories; the different types of cardiomyopathies; past experience with cardiac registries, and the diagnostic importance of coronary angiography. Steps were taken towards the formulation of a standard terminology, since too many confusing names are currently employed to mean “cardiomegaly of unknown origin”. A common name, “idiopathic cardiomegaly”, was therefore suggested for future use. The account presented here was prepared by Dr Z. Fejfar, Chief Medical Officer, Cardiovascular Diseases, World Health Organization, Geneva, on behalf of the other participants and is a précis of some of the information that was exchanged, some of the views that were expressed and of the suggestions that were made. PMID:4235740

  20. Clinical and radiographic outcomes of the treatment of adolescent idiopathic scoliosis with segmental pedicle screws and combined local autograft and allograft bone for spinal fusion: a retrospective case series

    PubMed Central

    2010-01-01

    Background High morbidity has been reported with iliac crest bone graft harvesting; however, donor bone is typically necessary for posterior spinal fusion. Autograft bone combined with allograft may reduce the morbidity associated with iliac crest bone harvesting and improve the fusion rate. Our aim in this study was to determine the presence of complications, pseudarthrosis, non-union, and infection using combined in situ local autograft bone and freeze-dried cancellous allograft bone in patients undergoing posterior spinal fusion for the treatment of adolescent idiopathic scoliosis. Methods A combination of in situ local autograft bone and freeze-dried cancellous allograft blocks were used in 50 consecutive patients with adolescent idiopathic scoliosis treated by posterior fusion and Moss Miami pedicle screw instrumentation. Results were assessed clinically and radiographically and quality of life and functional outcome was evaluated by administration of a Chinese version of the SRS-22 survey. Results There were 41 female and 9 male patients included for analysis with an average age of 14.7 years (range, 12-17). All patients had a minimum follow-up of 18 months (range, 18 to 40 months). The average preoperative Cobb angle was 49.8° (range, 40° to 86°). The average number of levels fused was 9.8 (range, 6-15). Patients had a minimum postoperative follow-up of 18 months. At final follow-up, the average Cobb angle correction was 77.8% (range, 43.4 to 92.5%). There was no obvious loss in the correction, and the average loss of correction was 1.1° (range, 0° to 4°). There was no pseudarthrosis and no major complications. Conclusions In situ autograft bone combined with allograft bone may be a promising method enhances spinal fusion in AIS treated with pedicle screw placement. By eliminating the need for iliac crest bone harvesting, significant morbidity may be avoided. PMID:20630050

  1. The Superiority of Intraoperative O-arm Navigation-assisted Surgery in Instrumenting Extremely Small Thoracic Pedicles of Adolescent Idiopathic Scoliosis

    PubMed Central

    Liu, Zhen; Jin, Mengran; Qiu, Yong; Yan, Huang; Han, Xiao; Zhu, Zezhang

    2016-01-01

    Abstract To investigate the accuracy of O-arm navigation-assisted screw insertion in extremely small thoracic pedicles and to compare it with free-hand pedicle screw insertion in adolescent idiopathic scoliosis (AIS). A total of 344 pedicle screws were inserted in apical region (defined as 2 vertebrae above and below the apex each) of 46 AIS patients (age range 13–18 years) with O-arm navigation and 712 screws were inserted in 92 AIS patients (age range 11–17 years) with free-hand technique. According to the narrowest diameter orthogonal to the long axis of the pedicle on a trajectory entering the vertebral body on preoperative computed tomography, the pedicles were classified into large (>3 mm) and small (≤3 mm) subgroups. Furthermore, a subset of extremely small pedicles (≤2 mm in the narrowest diameter) was specifically discussed. Screw accuracy was categorized as grade 0: no perforation, grade 1: perforation by less than 2 mm, grade 2: perforation by 2 to 4 mm, grade 3: perforation over 4 mm. In the O-arm group, the mean thoracic pedicle diameters were 2.23 mm (range 0.7–2.9 mm) and 3.48 mm (3.1–7.1 mm) for small and large pedicles, respectively. In the free-hand group, the small and large thoracic pedicle diameters were 2.42 mm (range 0.6–2.9 mm) and 3.75 mm (3.1–6.9 mm), respectively. The overall accuracies of screw insertion in large and small thoracic pedicles (grade 0, 1) were significantly higher in O-arm group (large: 93.8%, 210/224, small: 91.7%, 110/120) than those of free-hand group (large: 84.9%, 353/416, small: 78.4%, 232/296) (P < 0.05). Importantly, the overall accuracy of screw placement in extremely small pedicles was significantly higher in the O-arm group (84.3%, 48/57) compared with 62.7% (79/126) in free-hand group (P < 0.05), and the incidence of medial perforation was significantly lower in O-arm group (11.1%, 1/9) compared with 17.0% (8/47) in free-hand group (P < 0.05). The O

  2. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

    MedlinePlus

    ... Asked Questions Español Condiciones Chinese Conditions Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) En Español Read in Chinese What is idiopathic intracranial hypertension? Idiopathic intracranial hypertension (IIH) is a disorder that ...

  3. INTEGRATED POLARIZATION PROPERTIES OF 3C48, 3C138, 3C147, AND 3C286

    SciTech Connect

    Perley, R. A.; Butler, B. J. E-mail: BButler@nrao.edu

    2013-06-01

    We present the integrated polarization properties of the four compact radio sources 3C48, 3C138, 3C147, and 3C286, from 1 to 50 GHz, over a 30 yr time frame spanning 1982-2012. Using the polarized emission of Mars, we have determined that the position angle of the linearly polarized emission of 3C286 rises from 33 Degree-Sign at 8 GHz to 36 Degree-Sign at 45 GHz. There is no evidence for a change in the position angle over time. Using these values, the position angles of the integrated polarized emission from the other three sources are determined as a function of frequency and time. The fractional polarization of 3C286 is found to be slowly rising, at all frequencies, at a rate of {approx}0.015% yr{sup -1}. The fractional polarizations of 3C48, 3C138, and 3C147 are all slowly variable, with the variations correlated with changes in the total flux densities of these sources.

  4. Expression of Estrogen Receptor Coactivator Proline-, Glutamic Acid- and Leucine-Rich Protein 1 within Paraspinal Muscles in Adolescents with Idiopathic Scoliosis

    PubMed Central

    Skibinska, Izabela; Tomaszewski, Marek; Andrusiewicz, Miroslaw; Urbaniak, Paulina; Czarnecka-Klos, Roza; Shadi, Milud; Kotwicki, Tomasz; Kotwicka, Malgorzata

    2016-01-01

    Purpose The aim of this study was to detect and assess the estrogen receptor (ESR) coactivator PELP1 expression within human paraspinal skeletal muscles in patients suffering from idiopathic scoliosis. Methods During surgical correction of scoliosis the muscle biopsies harvested in 29 females. Presence of PELP1, ESR1 and ESR2 genes transcripts was studied using RT-qPCR technique while immunohistochemistry and western blot methods were used to detect the PEPL1 protein presence. Results PELP1 expression in deep paraspinal muscles revealed higher than in superficial back muscles (p = 0.005). Positive immunohistochemical staining for PELP1 was observed in the nuclei of the paraspinal muscle cells. Western blot revealed PELP1 protein in all samples. No significant difference in PELP1 expression between the convex and the concave scoliosis side (p>0.05) was found. In deep paraspinal back muscles, a significant correlation between the PELP1 expression level on the concave side and the Cobb angle (r = 0.4; p<0.05) was noted as well as between the PELP1 and ESR1 expression level (r = 0.7; p<0.05) while no correlation between PELP1 and ESR2 expression level was found. Conclusion To our knowledge, three techniques for the first time demonstrated the presence of the PELP1 in paraspinal muscles of patients with idiopathic scoliosis. The PELP1 potential regulatory impact on back muscle function is to be further investigated. PMID:27045366

  5. Body mass index in relation to truncal asymmetry of healthy adolescents, a physiopathogenetic concept in common with idiopathic scoliosis: summary of an electronic focus group debate of the IBSE

    PubMed Central

    2013-01-01

    There is no generally accepted scientific theory for the cause of adolescent idiopathic scoliosis (AIS). As part of its mission to widen understanding of scoliosis etiology, the International Federated Body on Scoliosis Etiology (IBSE).introduced the electronic focus group (EFG) as a means of increasing debate on knowledge of important topics. This has been designated as an on-line Delphi discussion. The text for this debate was written by Dr TB Grivas. It is based on published research from Athens, Greece evaluating schoolchildren age 11–17 years for the relation of body mass index (BMI) to each of truncal asymmetry (TA) and menarcheal status. Girls with relatively lower BMI were found to have a significant excess of severe TAs and significantly later menarche confirming the well-known relation of BMI to menarche. Together with other evidence linking nutritional status to skeletal growth, the observations suggest energy balance via the hypothalamus is related to trunk asymmetry. As with a recent speculative hypothesis for the pathogenesis of AIS in girls, Grivas et al. suggest that the severe TAs involve a genetically-determined selectively increased sensitivity (up-regulation) of the hypothalamus to circulating leptin with asymmetry as an adverse response to stress (hormesis). The TA is expressed bilaterally via the sympathetic nervous system to produce left-right asymmetry in ribs and/or vertebrae leading to severe TAs when beyond the capacity of postural mechanisms of the somatic nervous system to control the shape distortion in the trunk. This EFG discusses the findings and interpretations of the paper by Grivas and colleagues as research at the borderland between the genesis of TA (physiogenesis) and AIS (pathogenesis). It is suggested that TAs, here regarded in common with AIS, result from the combination of secondary sexual development affecting body composition, adolescent skeletal growth velocity, and an asymmetry process. The possible involvement of

  6. Improving the measurement of health-related quality of life in adolescent with idiopathic scoliosis: the SRS-7, a Rasch-developed short form of the SRS-22 questionnaire.

    PubMed

    Caronni, Antonio; Zaina, Fabio; Negrini, Stefano

    2014-04-01

    Scoliosis Research Society-22 (SRS-22) questionnaire was developed to evaluate health-related quality of life (HRQL) in adolescent idiopathic scoliosis (AIS) patients. Rasch analysis (RA) is a statistical procedure which turns questionnaire ordinal scores into interval measures. Measures from Rasch-compatible questionnaires can be used, similar to body temperature or blood pressure, to quantify disease severity progression and treatment efficacy. Purpose of the current work is to present Rasch analysis (RA) of the SRS-22 questionnaire and to develop an SRS-22 Rasch-approved short form. 300 SRS-22 were randomly collected from 2447 consecutive IS adolescents at their first evaluation (229 females; 13.9 ± 1.9 years; 26.9 ± 14.7 Cobb°) in a scoliosis outpatient clinic. RA showed both disordered thresholds and overall misfit of the SRS-22. Sixteen items were re-scored and two misfitting items (6 and 14) removed to obtain a Rasch-compatible questionnaire. Participants HRQL measured too high with the rearranged questionnaire, indicating a severe SRS-22 ceiling effect. RA also highlighted SRS-22 multidimensionality, with pain/function not merging with self-image/mental health items. Item 3 showed differential item functioning (DIF) for both curve and hump amplitude. A 7-item questionnaire (SRS-7) was prepared by selecting single items from the original SRS-22. SRS-7 showed fit to the model, unidimensionality and no DIF. Compared with the SRS-22, the short form scale shows better targeting of the participants' population. RA shows that SRS-22 has poor clinimetric properties; moreover, when used with AIS at first evaluation, SRS-22 is affected by a severe ceiling effect. SRS-7, an SRS-22 7-item short form questionnaire, provides an HRQL interval measure better tailored to these participants. PMID:24521663

  7. Idiopathic headshaking: is it still idiopathic?

    PubMed

    Pickles, Kirstie; Madigan, John; Aleman, Monica

    2014-07-01

    The clinical syndrome of equine idiopathic headshaking (HSK) was first described in the veterinary literature over 100 years ago, and the disorder continues to be a cause of substantial distress for the horse, frustration for the owner and therapeutic challenge for the veterinarian. This review presents a summary of the current knowledge of clinical signs, signalment, aetiopathogenesis, anatomy, diagnosis and treatment of idiopathic HSK. Recent advances in understanding the pathogenesis of the disease will be discussed with reference to human trigeminal neuralgia, along with the implications this may have for potential therapies. PMID:24821361

  8. [Idiopathic Pulmonary Fibrosis].

    PubMed

    Prasse, A

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF. PMID:26444136

  9. [Idiopathic pulmonary trunk aneurysm].

    PubMed

    Uehara, Mayuko; Kuroda, Yosuke; Ohori, Syunsuke; Mawatari, Toru; Morishita, Kiyofumi

    2010-07-01

    Pulmonary trunk aneurysm is generally associated with congenital cardiac defects, pulmonary hypertension, or infection. Idiopathic pulmonary trunk aneurysm without any associated diseases is a rare lesion and has seldom been reported. Here, we report a case of a 68-year-old woman with idiopathic pulmonary trunk aneurysm. The maximum diameter of the aneurysm was 53 mm while she was 142 cm in height. We successfully performed aneurysmorrhaphy and her postoperative course was uneventful. Aneurysmorrhaphy was an effective technique for idiopathic pulmonary trunk aneurysm without pulmonary hypertention. PMID:20662238

  10. Adolescent idiopathic scoliosis (AIS), environment, exposome and epigenetics: a molecular perspective of postnatal normal spinal growth and the etiopathogenesis of AIS with consideration of a network approach and possible implications for medical therapy

    PubMed Central

    2011-01-01

    Genetic factors are believed to play an important role in the etiology of adolescent idiopathic scoliosis (AIS). Discordant findings for monozygotic (MZ) twins with AIS show that environmental factors including different intrauterine environments are important in etiology, but what these environmental factors may be is unknown. Recent evidence for common chronic non-communicable diseases suggests epigenetic differences may underlie MZ twin discordance, and be the link between environmental factors and phenotypic differences. DNA methylation is one important epigenetic mechanism operating at the interface between genome and environment to regulate phenotypic plasticity with a complex regulation across the genome during the first decade of life. The word exposome refers to the totality of environmental exposures from conception onwards, comprising factors in external and internal environments. The word exposome is used here also in relation to physiologic and etiopathogenetic factors that affect normal spinal growth and may induce the deformity of AIS. In normal postnatal spinal growth we propose a new term and concept, physiologic growth-plate exposome for the normal processes particularly of the internal environments that may have epigenetic effects on growth plates of vertebrae. In AIS, we propose a new term and concept pathophysiologic scoliogenic exposome for the abnormal processes in molecular pathways particularly of the internal environment currently expressed as etiopathogenetic hypotheses; these are suggested to have deforming effects on the growth plates of vertebrae at cell, tissue, structure and/or organ levels that are considered to be epigenetic. New research is required for chromatin modifications including DNA methylation in AIS subjects and vertebral growth plates excised at surgery. In addition, consideration is needed for a possible network approach to etiopathogenesis by constructing AIS diseasomes. These approaches may lead through screening

  11. Idiopathic cardiomegaly in Africa.

    PubMed

    Ikeme, A C

    1976-01-01

    Idiopathic cardiomegaly is probably the commonest single diagnosis other than hypertension made in tropical and subtropical African cardiovascular practice. Understanding of the nature of this disease has been hampered by failure to recognize the possibility that the term "idiopathic cardiomegaly" may embrace several disease entities. Evidence suggests that many factors, sometimes acting singly, but often acting in combination, may be responsible for the genesis of so-called idiopathic myocardial failure. The future attitude to research should not be one of excluding well-defined forms from the concept of idiopathic cardiomegaly, but one of clinicopathological classification, which should be a prelude to the search, within each moiety of this group of disorders, for a specific or dominant etiological factor. PMID:829042

  12. Idiopathic Pulmonary Fibrosis

    MedlinePlus

    ... the NHLBI on Twitter. What Is Idiopathic Pulmonary Fibrosis? Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a ... time. The formation of scar tissue is called fibrosis. As the lung tissue thickens, your lungs can' ...

  13. Juvenile idiopathic arthritis

    MedlinePlus

    Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ... The cause of juvenile idiopathic arthritis (JIA) is not known. It ... illness . This means the body attacks and destroys healthy body ...

  14. Idiopathic Scrotal Calcinosis.

    PubMed

    Killedar, Madhura Milind; Shivani, Aslam A; Shinde, Usha

    2016-08-01

    Idiopathic scrotal calcinosis (ISC) is a rare benign condition which presents with multiple, asymptomatic, and painless nodules on the scrotal skin wall. The lesions have been attributed as sebaceous cysts, calcified steatocystoma, fibroma, atheroma, and xanthoma. Shapiro et al. reviewed the histologic data and found no evidence of an epithelial lining, residual cysts, and lipid or organisms, and concluded that the calcification was idiopathic introducing the term "idiopathic scrotal calcinosis." We have studied four cases of idiopathic scrotal calcinosis, one of which had scrotal calcinosis involving the whole of the scrotum. He presented with painless multiple nodules over the scrotum. He was subjected for surgery with SOS skin grafting, but as the scrotal skin is so lax, primary closure is easily possible. In all our four cases, primary closure was easily possible. PMID:27574356

  15. Idiopathic calcified myocardial mass

    PubMed Central

    Patterson, David; Gibson, Derek; Gomes, Ricardo; McDonald, Lawson; Olsen, Eckhardt; Parker, John; Ross, Donald

    1974-01-01

    Patterson, D., Gibson, D., Gomes, R., McDonald, L., Olsen, E., Parker, J., and Ross, D. (1974).Thorax,29, 589-594. Idiopathic calcified myocardial mass. Myocardial calcification can be subdivided into three groups—metastatic, dystrophic or an extension inwards from the pericardium. This case in which the calcified myocardial mass was initially delineated by radiography and by echocardiography and subsequently removed does not fit into any subdivision and has been termed idiopathic. Images PMID:4279467

  16. Idiopathic infantile hypercalcaemia--a continuing enigma.

    PubMed Central

    Martin, N D; Snodgrass, G J; Cohen, R D

    1984-01-01

    Seventy six children with documented Fanconi-type idiopathic infantile hypercalcaemia were studied and compared with 41 with the Williams-Beuren syndrome. Clinical comparison showed, as expected, very close similarities but also considerable differences, particularly in the severity of feeding problems and the degree of failure to thrive. The estimated incidence of idiopathic infantile hypercalcaemia alone has remained constant for the past 20 years, at approximately 18 cases per year in the United Kingdom (1 per 47 000 total live births). Long term morbidity in these children is mainly due to mental handicap and arteriopathy, but hypertension (29%), kyphoscoliosis (19%), hyperacusis (75%), and obesity (50%) may be added complications. In one child, hypercalcaemia recurred during adolescence but this seems to be excessively rare. More detailed investigation before treatment is required to discover the aetiology of hypercalcaemia in this condition. Images Fig. 2 PMID:6465928

  17. Idiopathic Juvenile Osteoporosis: A Case Report.

    PubMed

    Altan, Halenur; Tosun, Gül; Şen, Yaşar

    2015-08-01

    Idiopathic Juvenile Osteoporosis (IJO) is a very rare disease, self restrictive and shows marked, spontaneous improvement during adolescence. The major clinical features were pain with difficulty walking, growth retardation, oral and dental abnormalities with radiographically porous bone structure. A 13-year-old male referred to paediatric dentistry clinic for toothache. The observations made with extra-intraoral clinic examination that one revealed short and skinny stature, diffuse caries in deciduous teeth, abraded lower incisor, deep bite and dysmorphic appearance in permanent incisor. This report emphasizes the recognized features of IJO as well as describes facio-dental findings that could aid in the diagnosis and management of these patients. PMID:26436063

  18. Idiopathic Juvenile Osteoporosis: A Case Report

    PubMed Central

    Tosun, Gül; Şen, Yaşar

    2015-01-01

    Idiopathic Juvenile Osteoporosis (IJO) is a very rare disease, self restrictive and shows marked, spontaneous improvement during adolescence. The major clinical features were pain with difficulty walking, growth retardation, oral and dental abnormalities with radiographically porous bone structure. A 13-year-old male referred to paediatric dentistry clinic for toothache. The observations made with extra-intraoral clinic examination that one revealed short and skinny stature, diffuse caries in deciduous teeth, abraded lower incisor, deep bite and dysmorphic appearance in permanent incisor. This report emphasizes the recognized features of IJO as well as describes facio-dental findings that could aid in the diagnosis and management of these patients. PMID:26436063

  19. Idiopathic Inflammatory Myopathies

    PubMed Central

    Dimachkie, Mazen M.; Barohn, Richard J.

    2012-01-01

    The idiopathic inflammatory myopathies are a group of rare disorders including polymyositis (PM), dermatomyositis (DM), and autoimmune necrotizing myopathies (NMs). The idiopathic inflammatory myopathies share many similarities. They present acutely, subacutely, or chronically with marked proximal and symmetric muscle weakness, except for associated distal and asymmetric weakness in inclusion body myositis. The idiopathic inflammatory myopathies also share a variable degree of creatine kinase (CK) elevation and a nonspecifically abnormal electromyogram demonstrating an irritative myopathy. The muscle pathology demonstrates inflammatory exudates of variable distribution within the muscle fascicle. Despite these similarities, the idiopathic inflammatory myopathies are a heterogeneous group. The overlap syndrome (OS) refers to the association of PM, DM, or NM with connective tissue disease, such as scleroderma or systemic lupus erythematosus. In addition to elevated antinuclear antibodies (ANA), patients with OS may be weaker in the proximal arms than the legs mimicking the pattern seen in some muscular dystrophies. In this review, we focus on DM, PM, and NM and examine current and promising therapies. PMID:23117947

  20. Selection of fusion levels using the fulcrum bending radiograph for the management of adolescent idiopathic scoliosis patients with alternate level pedicle screw strategy: clinical decision-making and outcomes

    PubMed Central

    Samartzis, Dino; Leung, Yee; Shigematsu, Hideki; Natarajan, Deepa; Stokes, Oliver; Mak, Kin-Cheung; Yao, Guanfeng; Luk, Keith D. K.; Cheung, Kenneth M. C.

    2015-01-01

    Objective Selecting fusion levels based on the Luk et al criteria for operative management of thoracic adolescent idiopathic scoliosis (AIS) with hook and hybrid systems yields acceptable curve correction and balance parameters; however, it is unknown whether utilizing a purely pedicle screw strategy is effective. Utilizing the fulcrum bending radiographic (FBR) to assess curve flexibility to select fusion levels, the following study assessed the efficacy of pedicle screw fixation with alternate level screw strategy (ALSS) for thoracic AIS. Methods A retrospective study with prospective radiographic data collection/analyses (preoperative, postoperative 1-week and minimum 2-year follow-up) of 28 operative thoracic AIS patients undergoing ALSS was performed. Standing coronal/sagittal and FBR Cobb angles, FBR flexibility, fulcrum bending correction index (FBCI), trunkal shift, radiographic shoulder height (RSH), and list were assessed on x-rays. Fusion level selection was based on the Luk et al criteria and compared to conventional techniques. Results In the primary curve, the mean preoperative and postoperative 1 week and last follow-up standing coronal Cobb angles were 59.9, 17.2 and 20.0 degrees, respectively. Eighteen patients (64.3%) had distal levels saved (mean: 1.6 levels) in comparison to conventional techniques. Mean immediate and last follow-up FBCIs were 122.6% and 115.0%, respectively. Sagittal alignment did not statistically differ between any assessment intervals (p>0.05). A decrease in trunkal shift was noted from preoperative to last follow-up (p = 0.003). No statistically significant difference from preoperative to last follow-up was noted in RSH and list (p>0.05). No "add-on" of other vertebra or decompensation was noted and all patients achieved fusion. Conclusions This is the first report to note that using the FBR for decision-making in selecting fusion levels in thoracic AIS patients undergoing management with pedicle screw constructs (e.g. ALSS

  1. An idiopathic gigantomastia.

    PubMed

    Cho, Min Jeng; Yang, Jung-Hyun; Choi, Hyeon-Gon; Kim, Wan Seop; Yu, Yeong-Beom; Park, Kyoung Sik

    2015-03-01

    Gigantomastia is a rare condition characterized by excessive breast growth. It has been reported that the majority of gigantomastia cases occur during either pregnancy or puberty. We were presented with a rare case of gigantomastia associated with neither pregnancy nor puberty, and successfully treated it with reduction mammaplasty and free nipple graft. This idiopathic gigantomastia is the very first case in Korea, and adds to the worldwide total of 9 reported cases. PMID:25741497

  2. An idiopathic gigantomastia

    PubMed Central

    Cho, Min Jeng; Choi, Hyeon-Gon; Kim, Wan Seop; Yu, Yeong-Beom; Park, Kyoung Sik

    2015-01-01

    Gigantomastia is a rare condition characterized by excessive breast growth. It has been reported that the majority of gigantomastia cases occur during either pregnancy or puberty. We were presented with a rare case of gigantomastia associated with neither pregnancy nor puberty, and successfully treated it with reduction mammaplasty and free nipple graft. This idiopathic gigantomastia is the very first case in Korea, and adds to the worldwide total of 9 reported cases. PMID:25741497

  3. Idiopathic Polydactylous Longitudinal Erythronychia

    PubMed Central

    2011-01-01

    Objective: To describe the clinical features of idiopathic polydactylous longitudinal erythronychia. Introduction: Longitudinal erythronychia presents as a linear red band on the nail plate. Idiopathic polydactylous longitudinal erythronychia is a rarely described manifestation of longitudinal erythronychia in which one or more linear red bands present on the nails of multiple digits without any associated subungual malignant tumor, dermatological condition, or systemic disease. Methods: As part of a total body skin examination, the fingernails and toenails were evaluated for linear red bands. Results: One or more asymptomatic linear red bands (longitudinal erythronychia) was observed on multiple digits of the hands in one percent (3 men of 134 men and 112 women) of patients examined during a period of 75 days. The author also noted similar changes of his own nails. Between 3 to 10 digits were affected. Multiple linear red bands per nail were usually narrow (less than 1mm wide), whereas a single band on a nail often ranged from 4 to 6mm wide. The intensity of an individual wider linear red band was position-dependent in three individuals in whom the distal portion appeared less prominent when the affected digit was held upward above the level of the patient's heart—pseudolongitudinal erythronychia. Other nail changes in these patients included distal subungual hyperkeratosis, fissuring at the free end of the nail, leukonychia, red lunula, and splinter hemorrhages. Discussion: Idiopathic polydactylous longitudinal erythronychia is a benign, usually asymptomatic, condition of undetermined etiology characterized by one or more linear red bands originating at the proximal nail fold or distal lunula and extending to the free edge of the nail. It appears to be more prevalent in men over 50 years of age and its incidence was noted to be one percent of adults attending a dermatology clinic. Patients are either unaware of the nail changes or seek medical attention because

  4. Efficacy of Postoperative Pain Management Using Continuous Local Anesthetic Infusion at the Iliac Crest Bone Graft Site in Patients with Adolescent Idiopathic Scoliosis: A Parallel, Double-Blinded, Randomized Controlled Pilot Trial.

    PubMed

    Samartzis, Dino; Bow, Cora; Cheung, Jason Pui Yin; Sham, Phoebe; Mak, Kin-Cheung; Cheung, Wai-Yuen; Wong, Yat-Wa; Luk, Keith D K; Cheung, Kenneth M C; Lawmin, Jean-Claude

    2016-05-01

    Study Design Randomized controlled trial. Objective Adolescent idiopathic scoliosis (AIS) is a common spinal deformity that affects every population. In severe deformity, surgical intervention is performed. Autogenous iliac crest bone graft (ICBG) harvesting remains a common procedure worldwide for scoliosis surgery. Postoperative pain at the ICBG donor site is a major concern in patients undergoing spine surgery that affects postoperative functional outcome and consumes health care resources. Previous studies have noted a decrease in pain and postoperative analgesic use with the application of continuous infusion of anesthetic at the ICBG site in comparison with placebo. However, there is lack of evidence addressing the efficacy of continuous anesthetic infusion at the ICBG site in young patients and in particular those with spinal deformity, such as AIS. As such, this parallel, double-blinded, randomized controlled trial addressed the pain management efficacy of continuous anesthetic infusion versus saline at the ICBG site in patients with AIS during the immediate postoperative period. Methods Participants were randomized into two groups. Group A (control subjects) received 3 mL per hour of saline locally at the ICBG site, and group B (treatment subjects) received a constant rate of infusion of 3 mL per hour of 0.25% levobupivacaine. Both groups received their postoperative intervention for 47 hours. All subjects and outcome assessors were blinded to the type of intervention. Utilizing the visual analog pain scale, pain was assessed at the primary spine surgical site, ICBG site, and contralateral ICBG site. Overall physical pain was assessed by the McGill Pain Questionnaire. The degree of analgesic use and complications were also evaluated. All outcomes were assessed up until the fourth day of the patients' hospitalization following surgery. Results Twelve subjects were recruited (five in group A; seven in group B). No difference was noted at baseline

  5. Idiopathic gingival fibromatosis

    PubMed Central

    Dani, Nitin Hemchandra; Khanna, Dinkar Parveen; Bhatt, Vaibhavi Hitesh; Joshi, Chaitanya Pradeep

    2015-01-01

    Idiopathic gingival fibromatosis (IGF) is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth. PMID:26941525

  6. [Idiopathic granulomatous mastitis].

    PubMed

    Hello, M; Néel, A; Graveleau, J; Masseau, A; Agard, C; Caillon, J; Hamidou, M

    2013-06-01

    Idiopathic granulomatous mastitis (IGM) is a rare localized granulomatosis of unknown aetiology that usually affects women of childbearing age. It often mimics breast carcinoma or abscess. Histopathologic evaluation and elimination of the others aetiologies of granuloma play a crucial role in the diagnosis. Its etiopathogeny remains poorly understood, but Corynebacteria might be involved. The disease course is usually protracted, with a significant impact on quality of life. The management of IGM remains controversial, but corticosteroids are usually the first-line treatment. PMID:22981187

  7. [Genetics of idiopathic epilepsies].

    PubMed

    Weber, Y G; Lerche, H

    2013-02-01

    Idiopathic epilepsies are genetically determined. They are characterized by the observed seizure types, an age-dependent onset, electroencephalographic criteria and concomitant symptoms, such as movement disorders or developmental delay. The main subtypes are the idiopathic (i) generalized, (ii) the focal epilepsies including the benign syndromes of early childhood and (iii) the epileptic encephalopathies as well as the fever-associated syndromes. In recent years, an increasing number of mutations have been identified in genes encoding ion channels, proteins associated to the vesical synaptic cycle or proteins involved in energy metabolism. These mechanisms are pathophysiologically plausible as they influence neuronal excitability. The large number of genetic defects in epilepsy complicates the genetic diagnostic analysis but novel genetic methods are available covering all known genes at a reasonable price. The proof of a genetic defect leads to a definitive diagnosis, is important for the prognostic and genetic counselling and may influence therapeutic decisions in some cases, so that genetic diagnostic testing is becoming increasingly more important and meaningful in many cases in daily clinical practice. PMID:23392265

  8. Idiopathic Retroperitoneal Fibrosis.

    PubMed

    Vaglio, Augusto; Maritati, Federica

    2016-07-01

    Idiopathic retroperitoneal fibrosis (RPF), reviewed herein, is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retroperitoneum, where it frequently causes ureteral obstruction and renal failure. The clinical phenotype of RPF is complex, because it can be associated with fibro-inflammatory disorders involving other organs, is considered part of the spectrum of IgG4-related disease, and often arises in patients with other autoimmune conditions. Obstructive uropathy is the most common complication, although other types of renal involvement may occur, including stenosis of the renal arteries and veins, renal atrophy, and different types of associated GN. Environmental and genetic factors contribute to disease susceptibility, whereas the immunopathogenesis of RPF is mediated by different immune cell types that eventually promote fibroblast activation. The diagnosis is made on the basis of computed tomography or magnetic resonance imaging, and positron emission tomography is a useful tool in disease staging and follow-up. Treatment of idiopathic RPF aims at relieving ureteral obstruction and inducing disease regression, and includes the use of glucocorticoids, combined or not with other traditional immunosuppressants. However, biologic therapies such as the B cell-depleting agent rituximab are emerging as potentially efficacious agents in difficult-to-treat cases. PMID:26860343

  9. Idiopathic laryngotracheal stenosis.

    PubMed

    Costantino, Christina L; Mathisen, Douglas J

    2016-03-01

    Idiopathic laryngotracheal stenosis (ILTS) is a rare inflammatory disease of unknown etiology. Infectious, traumatic and immunologic processes must first be excluded. The majority of patients affected are female who present with progressive symptoms of upper airway obstruction, which can extend over a number of years. ILTS is characterized by short segment, circumferential stenotic lesions, located particularly at the level of the cricoid. Bronchoscopic evaluation is essential for establishing the diagnosis and operative planning. Various temporizing interventions have historically been utilized, including dilation and laser ablation, for symptomatic management. However these interventions have demonstrated diminishing returns and poor long-term outcomes. Patients with ILTS should be considered early for definitive surgical intervention to minimize complications and optimize outcomes. Laryngotracheal resection and reconstruction is a viable intervention, which has demonstrated good long-term results and low recurrence rates for this patient population. PMID:26981272

  10. Chronic Idiopathic Thrombocytogenic Purpura

    PubMed Central

    Pineo, G. F.

    1984-01-01

    Chronic idiopathic thrombocytopenic purpura (ITP) is a relatively common cause of an acquired hemostatic defect. It is important for family physicians to recognize this disorder, because of its insidious onset and the fact that it most commonly affects women of childbearing age. Chronic ITP is due to an antibody in the plasma which attaches to platelets and leads to their destruction in the reticuloendothelial system. The antibody can cross the placenta and affect the fetus. Although the condition may not disappear, in the vast majority of patients it can be controlled with current therapy, including prednisone, splenectomy and immunosuppressive agents. Although the mortality rate is low, patients with severe thrombocytopenia may have significant bleeding problems requiring special measures such as platelet transfusions, intravenous gammaglobulin, plasmapheresis and emergency splenectomy. Upon diagnosis, these patients should be referred to a large, specialized centre. PMID:21279099

  11. Idiopathic laryngotracheal stenosis

    PubMed Central

    Costantino, Christina L.

    2016-01-01

    Idiopathic laryngotracheal stenosis (ILTS) is a rare inflammatory disease of unknown etiology. Infectious, traumatic and immunologic processes must first be excluded. The majority of patients affected are female who present with progressive symptoms of upper airway obstruction, which can extend over a number of years. ILTS is characterized by short segment, circumferential stenotic lesions, located particularly at the level of the cricoid. Bronchoscopic evaluation is essential for establishing the diagnosis and operative planning. Various temporizing interventions have historically been utilized, including dilation and laser ablation, for symptomatic management. However these interventions have demonstrated diminishing returns and poor long-term outcomes. Patients with ILTS should be considered early for definitive surgical intervention to minimize complications and optimize outcomes. Laryngotracheal resection and reconstruction is a viable intervention, which has demonstrated good long-term results and low recurrence rates for this patient population. PMID:26981272

  12. Idiopathic Pulmonary Fibrosis.

    PubMed

    Martin, Maria D; Chung, Jonathan H; Kanne, Jeffrey P

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease and is associated with a very poor prognosis. IPF manifests histopathologically as usual interstitial pneumonia (UIP) and as subpleural and basal predominant reticulation with honeycombing on high-resolution computed tomography (HRCT) of the chest. When a high-confidence radiologic diagnosis of UIP is made on HRCT, surgical biopsy is rarely required. Therefore, radiologists should recognize a UIP pattern on HRCT as well as recognize other patterns of fibrosing lung disease such as nonspecific interstitial pneumonia or chronic hypersensitivity pneumonitis, both of which can be mistaken for UIP. This article reviews the clinical, CT, and histopathologic features of IPF, discusses the impact of CT findings on prognosis, and describes complications associated with IPF. PMID:27043425

  13. The idiopathic hypereosinophilic syndrome.

    PubMed Central

    Alfaham, M A; Ferguson, S D; Sihra, B; Davies, J

    1987-01-01

    A 14 year old girl with idiopathic hypereosinophilic syndrome is described. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the cardiovascular system as the major complication. She responded well to treatment. After a comprehensive search of the published reports 18 cases of this syndrome were identified in children under 16 years. Fifteen of these children had involvement of the cardiovascular system as the major source of their morbidity and mortality. Summary of the clinical details and laboratory, biopsy, and necropsy findings of the involvement of the various organ systems of the 18 children is presented. PMID:3619478

  14. Juvenile idiopathic arthritis

    PubMed Central

    Bhatt, Krupa H; Karjodkar, Freny R; Sansare, Kaustubh; Patil, Darshana

    2014-01-01

    Juvenile Idiopathic Arthritis (JIA) is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential. PMID:24808703

  15. Idiopathic Flushing with Dysesthesia

    PubMed Central

    Fogelman, Joshua P.; Ashinoff, Robin; Soter, Nicholas A.

    2015-01-01

    Objective: The purpose of this study was to analyze the efficacy and safety of the 585nm pulsed dye laser for the treatment of idiopathic flushing with dysesthesia. Design: This was a retrospective study of patients treated with a 585nm pulsed dye laser with fluences ranging from 3.5 to 7.5J/cm2 (purpura threshold fluences), a pulse duration of 450μsec, and a spot size of 5 or 10mm. Setting: The Ronald 0. Perelman Department of Dermatology at New York University Medical Center. Participants: Ten adult subjects who presented with flushing with dysesthesia. Measurements: Participants subjectively evaluated the decrease in dysesthesia and the number of flushing episodes. The objective response to treatment was evaluated by a single physician using pre- and postoperative photographs. The severity of postoperative erythema was compared with baseline using an ordinal scale ranging from zero (resolution of erythema) to four (76-100% of baseline erythema). Results: The mean number of treatments received by the subjects was seven. The mean fluence was 6.66J/cm2. Subjectively, 100 percent of subjects reported a decrease in dysethesia and the number of flushing episodes. Objectively, subjects demonstrated at least a 62.5-percent reduction in erythema. Conclusion: Laser surgery provided subjective relief of dysesthesia and decreased the number of flushing episodes with a greater than 62-percent objective reduction in the severity of erythema. The 585nm pulsed dye laser is a safe, efficacious treatment for the signs and symptoms of idiopathic flushing with dysesthesia. PMID:26345489

  16. [Idiopathic calcium nephrolithiasis: therapeutic aspects].

    PubMed

    Jaeger, P; Portmann, L; Burckhardt, P

    1983-11-26

    The 75% of the renal stone formers have a so-called idiopathic calcium urolithiasis. The majority of these patients, however, do have a detectable biochemical disorder such as hypercalciuria, hyperuricosuria or hyperoxaluria. A high fluid intake unequivocally represents the first step in the therapeutic approach to these patients. Nevertheless, the detection of any type of biochemical disturbance is of great importance since the addition of a specific therapy will then become possible. Patients with absorptive idiopathic hypercalciuria will be advised to decrease their intake of dairy products as a function of the degree of calcium hyperabsorption, and simultaneously the major dietary sources of oxalate such as chocolate, spinach, rhubarb and asparagus will be eliminated; neutral orthophosphates (3-4 times 500 mg/d) or a thiazide, resp. an analogue as chlorthalidone (50 mg/d) are reasonable alternatives. Renal idiopathic hypercalciuria should be treated, according to the authors, with chlorthalidone (50 mg/d), with or without allopurinol (300 mg/d) depending on the presence of concomitant hyperuricosuria. Patients with dietary idiopathic hypercalciuria should be advised to better equilibrate the various components of their dietary intake. Finally, patients with isolated idiopathic hyperuricosuria whose disease would remain active despite a high fluid intake should receive allopurinol (300 mg/d). The treatment of isolated idiopathic hyperoxaluria is not yet well established. Two main arguments favor this so to say "tailored" approach to the idiopathic stone former: first, some metabolic disturbances are causally related to a particularly active and severe urolithiasis, whereas others are less so; second, the lack of efficacy of some types of treatment appears more and more to be due to insufficient screening of the patients before starting a given treatment. PMID:6658421

  17. How Is Idiopathic Pulmonary Fibrosis Treated?

    MedlinePlus

    ... the NHLBI on Twitter. How Is Idiopathic Pulmonary Fibrosis Treated? Doctors may prescribe medicines, oxygen therapy , pulmonary ... PR), and lung transplant to treat idiopathic pulmonary fibrosis (IPF). Medicines Currently, no medicines are proven to ...

  18. Idiopathic pulmonary arterial hypertension.

    PubMed

    Souza, Rogerio; Jardim, Carlos; Humbert, Marc

    2013-10-01

    Idiopathic pulmonary arterial hypertension (IPAH), formerly called primary pulmonary hypertension, is a rare disease (incidence and prevalence rates of approximately one and six cases per million inhabitants, respectively) with different clinical phenotypes. A group of diverse conditions manifest pulmonary arterial hypertension (PAH) and share similar pathological and/or clinical findings with IPAH. By definition, IPAH is diagnosed only after alternative diagnoses have been ruled out. Extensive investigation is needed to determine if PAH is associated with thyroid diseases, infectious diseases, autoimmune conditions, exposure to certain drugs (particularly anorexigens), certain genetic mutations, and so on. The presence of genetic abnormalities and risk factors (such as specific drug exposures) reinforces the "multiple hit" concept for the development of pulmonary hypertension. Fortunately, within the past two decades, therapeutic options have become available for IPAH, resulting in improved survival and clinical outcomes. At least seven different compounds have been registered for PAH treatment. However, even with aggressive PAH-specific therapy, mortality rates remain high (∼40% at 5 years). Given the high mortality rates, the use of combinations of agents that work by different pathways has been advocated (either as "add-on" therapy or initial "up front" therapy). Further, new therapeutic agents and treatment strategies are on the near horizon, aiming to further improve survival from the remarkable progress already seen. PMID:24037625

  19. Idiopathic Inflammatory Myopathies

    PubMed Central

    Barohn, Richard J.; Amato, Anthony

    2014-01-01

    The idiopathic inflammatory myopathies (IIM) consist of rare heterogenous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis (IBM). Besides frequent creatine kinase (CK) elevation, the electromyogram confirms the presence of an irritative myopathy. Extramuscular involvement affects a significant number of cases with interstitial lung disease (ILD), cutaneous in dermatomyositis (DM), systemic or joint manifestations and increased risk of malignancy especially in DM. Myositis specific autoantibodies influence phenotype of the IIM. Jo-1 antibodies are frequently associated with ILD and the newly described HMG-CoA reductase antibodies are characteristic of autoimmune necrotizing myopathy (NM). Muscle pathology ranges from inflammatory exudates of variable distribution, to intact muscle fiber invasion, necrosis, phagocytosis and in the case of IBM rimmed vacuoles and protein deposits. Despite many similarities, the IIM are a quite heterogeneous from the histopathological and pathogenetic standpoints in addition to some clinical and treatment-response difference. The field has witnessed significant advances in our understanding of pathophysiology and treatment of these rare disorders. In this review, we focus on DM, polymyositis (PM) and NM and examine current and promising therapies. The reader interested in more details on IBM is referred to the corresponding chapter in this issue. PMID:25037081

  20. Idiopathic inflammatory myositis.

    PubMed

    Tieu, Joanna; Lundberg, Ingrid E; Limaye, Vidya

    2016-02-01

    Knowledge on idiopathic inflammatory myopathy (IIM) has evolved with the identification of myositis-associated and myositis-specific antibodies, development of histopathological classification and the recognition of how these correlate with clinical phenotype and response to therapy. In this paper, we outline key advances in diagnosis and histopathology, including the more recent identification of antibodies associated with immune-mediated necrotising myopathy (IMNM) and inclusion body myositis (IBM). Ongoing longitudinal observational cohorts allow further classification of these patients with IIM, their predicted clinical course and response to specific therapies. Registries have been developed worldwide for this purpose. A challenging aspect in IIM, a multisystem disease with multiple clinical subtypes, has been defining disease status and clinically relevant improvement. Tools for assessing activity and damage are now recognised to be important in determining disease activity and guiding therapeutic decision-making. The International Myositis Assessment and Clinical Studies (IMACS) group has developed such tools for use in research and clinical settings. There is limited evidence for specific treatment strategies in IIM. With significant development in the understanding of IIM and improved classification, longitudinal observational cohorts and trials using validated outcome measures are necessary, to provide important information for evidence-based care in the clinical setting. PMID:27421222

  1. Pathogenesis of adolescent idiopathic scoliosis in girls - a double neuro-osseous theory involving disharmony between two nervous systems, somatic and autonomic expressed in the spine and trunk: possible dependency on sympathetic nervous system and hormones with implications for medical therapy.

    PubMed

    Burwell, R Geoffrey; Aujla, Ranjit K; Grevitt, Michael P; Dangerfield, Peter H; Moulton, Alan; Randell, Tabitha L; Anderson, Susan I

    2009-01-01

    Anthropometric data from three groups of adolescent girls - preoperative adolescent idiopathic scoliosis (AIS), screened for scoliosis and normals were analysed by comparing skeletal data between higher and lower body mass index subsets. Unexpected findings for each of skeletal maturation, asymmetries and overgrowth are not explained by prevailing theories of AIS pathogenesis. A speculative pathogenetic theory for girls is formulated after surveying evidence including: (1) the thoracospinal concept for right thoracic AIS in girls; (2) the new neuroskeletal biology relating the sympathetic nervous system to bone formation/resorption and bone growth; (3) white adipose tissue storing triglycerides and the adiposity hormone leptin which functions as satiety hormone and sentinel of energy balance to the hypothalamus for long-term adiposity; and (4) central leptin resistance in obesity and possibly in healthy females. The new theory states that AIS in girls results from developmental disharmony expressed in spine and trunk between autonomic and somatic nervous systems. The autonomic component of this double neuro-osseous theory for AIS pathogenesis in girls involves selectively increased sensitivity of the hypothalamus to circulating leptin (genetically-determined up-regulation possibly involving inhibitory or sensitizing intracellular molecules, such as SOC3, PTP-1B and SH2B1 respectively), with asymmetry as an adverse response (hormesis); this asymmetry is routed bilaterally via the sympathetic nervous system to the growing axial skeleton where it may initiate the scoliosis deformity (leptin-hypothalamic-sympathetic nervous system concept = LHS concept). In some younger preoperative AIS girls, the hypothalamic up-regulation to circulating leptin also involves the somatotropic (growth hormone/IGF) axis which exaggerates the sympathetically-induced asymmetric skeletal effects and contributes to curve progression, a concept with therapeutic implications. In the somatic

  2. Pathogenesis of adolescent idiopathic scoliosis in girls - a double neuro-osseous theory involving disharmony between two nervous systems, somatic and autonomic expressed in the spine and trunk: possible dependency on sympathetic nervous system and hormones with implications for medical therapy

    PubMed Central

    2009-01-01

    Anthropometric data from three groups of adolescent girls - preoperative adolescent idiopathic scoliosis (AIS), screened for scoliosis and normals were analysed by comparing skeletal data between higher and lower body mass index subsets. Unexpected findings for each of skeletal maturation, asymmetries and overgrowth are not explained by prevailing theories of AIS pathogenesis. A speculative pathogenetic theory for girls is formulated after surveying evidence including: (1) the thoracospinal concept for right thoracic AIS in girls; (2) the new neuroskeletal biology relating the sympathetic nervous system to bone formation/resorption and bone growth; (3) white adipose tissue storing triglycerides and the adiposity hormone leptin which functions as satiety hormone and sentinel of energy balance to the hypothalamus for long-term adiposity; and (4) central leptin resistance in obesity and possibly in healthy females. The new theory states that AIS in girls results from developmental disharmony expressed in spine and trunk between autonomic and somatic nervous systems. The autonomic component of this double neuro-osseous theory for AIS pathogenesis in girls involves selectively increased sensitivity of the hypothalamus to circulating leptin (genetically-determined up-regulation possibly involving inhibitory or sensitizing intracellular molecules, such as SOC3, PTP-1B and SH2B1 respectively), with asymmetry as an adverse response (hormesis); this asymmetry is routed bilaterally via the sympathetic nervous system to the growing axial skeleton where it may initiate the scoliosis deformity (leptin-hypothalamic-sympathetic nervous system concept = LHS concept). In some younger preoperative AIS girls, the hypothalamic up-regulation to circulating leptin also involves the somatotropic (growth hormone/IGF) axis which exaggerates the sympathetically-induced asymmetric skeletal effects and contributes to curve progression, a concept with therapeutic implications. In the somatic

  3. Physiotherapy in pauciarticular juvenile idiopathic arthritis. Case study.

    PubMed

    Zuk, Beata; Kaczor, Zofia; Zuk-Drążyk, Berenika; Księżopolska-Orłowska, Krystyna

    2014-01-01

    Juvenile idiopathic arthritis (JIA) is the most common arthropathy of childhood and adolescence. This term encompasses a group of chronic systemic inflammatory diseases of the connective tissue which cause arthritis in patients under 16 years of age lasting at least 6 weeks. The authors presented the characteristic features of physiotherapy based on functional examination results on the basis of two cases of girls with pauciarticular JIA treated according to an established pharmacological regimen. Physiotherapy should be introduced at an early stage of the disease. Kinesiotherapy preceded by history-taking and a functional examination of the patient, has to focus on both primary and secondary joint lesions. PMID:25041889

  4. Idiopathic Interstitial Pneumonia

    PubMed Central

    Flaherty, Kevin R.; Andrei, Adin-Cristian; King, Talmadge E.; Raghu, Ganesh; Colby, Thomas V.; Wells, Athol; Bassily, Nadir; Brown, Kevin; du Bois, Roland; Flint, Andrew; Gay, Steven E.; Gross, Barry H.; Kazerooni, Ella A.; Knapp, Robert; Louvar, Edmund; Lynch, David; Nicholson, Andrew G.; Quick, John; Thannickal, Victor J.; Travis, William D.; Vyskocil, James; Wadenstorer, Frazer A.; Wilt, Jeffrey; Toews, Galen B.; Murray, Susan; Martinez, Fernando J.

    2007-01-01

    Rationale: Treatment and prognoses of diffuse parenchymal lung diseases (DPLDs) varies by diagnosis. Obtaining a uniform diagnosis among observers is difficult. Objectives: Evaluate diagnostic agreement between academic and community-based physicians for patients with DPLDs, and determine if an interactive approach between clinicians, radiologists, and pathologists improved diagnostic agreement in community and academic centers. Methods: Retrospective review of 39 patients with DPLD. A total of 19 participants reviewed cases at 2 community locations and 1 academic location. Information from the history, physical examination, pulmonary function testing, high-resolution computed tomography, and surgical lung biopsy was collected. Data were presented in the same sequential fashion to three groups of physicians on separate days. Measurements and Main Results: Each observer's diagnosis was coded into one of eight categories. A κ statistic allowing for multiple raters was used to assess agreement in diagnosis. Interactions between clinicians, radiologists, and pathologists improved interobserver agreement at both community and academic sites; however, final agreement was better within academic centers (κ = 0.55–0.71) than within community centers (κ = 0.32–0.44). Clinically significant disagreement was present between academic and community-based physicians (κ = 0.11–0.56). Community physicians were more likely to assign a final diagnosis of idiopathic pulmonary fibrosis compared with academic physicians. Conclusions: Significant disagreement exists in the diagnosis of DPLD between physicians based in communities compared with those in academic centers. Wherever possible, patients should be referred to centers with expertise in diffuse parenchymal lung disorders to help clarify the diagnosis and provide suggestions regarding treatment options. PMID:17255566

  5. Idiopathic Sporadic Onychomadesis of Toenails

    PubMed Central

    Nitayavardhana, Sunatra

    2016-01-01

    Onychomadesis is a clinical sign of nail plate separation due to transient or permanent arrest of nail matrix activities. Onychomadesis can be considered as a severe form of Beau's line. This condition usually occurs after trauma, causal diseases, or medications, yet it rarely occurs as an idiopathic condition. We report a case of a 38-year-old Thai female who developed recurrence onychomadesis in several toenails in the absence of predisposing factors or associated conditions. To the best of our knowledge, our patient is the first reported case of idiopathic onychomadesis limited to toenails. PMID:27437152

  6. Treatment of Idiopathic Intracranial Hypotension With Tea: A Case Report

    PubMed Central

    Petramfar, Peyman; Mohammadi, S. Saeed; Hosseinzadeh, Farideh

    2016-01-01

    Introduction The syndrome of spontaneous intracranial hypotension has been increasingly diagnosed since its discovery through magnetic resonance imaging (MRI). It is a rare syndrome that is due to the leakage of cerebrospinal fluid (CSF) from a tear in the dura and can occur at any age, even among adolescents, but is most frequently seen among females in late middle age. Case Presentation Here, we describe a 32-year-old woman with a two-month history of headaches and occasional nausea and vomiting (N/V). MRI without gadolinium was normal, but meningeal enhancement was seen in MRI with gadolinium. The lumbar puncture revealed a low opening pressure. Computed tomography myelography (CT myelography) showed no leakage; Therefore, idiopathic intracranial hypotension was diagnosed. Treatment was started using tea, and the patient’s headache got significantly better in about a day. Conclusions Conservative therapy, such as bed rest and caffeine treatment with eight cups of tea daily, yielded a significant improvement in our patient. Effectively, the patient constitutes a case of idiopathic intracranial hypotension due to undetectable CSF leakage or hyper-absorption, with good response to conservative management through tea-drinking. Further investigations with an appropriate sample size are needed in order to confirm this intervention in the treatment of idiopathic intracranial hypotension. PMID:27621920

  7. NATO-3C/Delta launch

    NASA Technical Reports Server (NTRS)

    1978-01-01

    NATO-3C, the third in a series of NATO defense-related communication satellites, is scheduled to be launched on a delta vehicle from the Eastern Test Range no earlier than November 15, 1978. NATO-3A and -3B were successfully launched by Delta vehicles in April 1976 and January 1977, respectively. The NATO-3C spacecraft will be capable of transmitting voice, data, facsimile, and telex messages among military ground stations. The launch vehicle for the NATO-3C mission will be the Delta 2914 configuration. The launch vehicle is to place the spacecraft in a synchronous transfer orbit. The spacecraft Apogee Kick motor is to be fired at fifth transfer orbit apogee to circularize its orbit at geosynchronous altitude of 35,900 km(22,260 miles) above the equator over the Atlantic Ocean somewhere between 45 and 50 degrees W longitude.

  8. Idiopathic CD4 Lymphocytopenia

    PubMed Central

    Régent, Alexis; Autran, Brigitte; Carcelain, Guislaine; Cheynier, Rémi; Terrier, Benjamin; Charmeteau-De Muylder, Bénédicte; Krivitzky, Alain; Oksenhendler, Eric; Costedoat-Chalumeau, Nathalie; Hubert, Pascale; Lortholary, Olivier; Dupin, Nicolas; Debré, Patrice; Guillevin, Loïc; Mouthon, Luc

    2014-01-01

    Abstract Idiopathic CD4 T lymphocytopenia (ICL) is a rare and severe condition with limited available data. We conducted a French multicenter study to analyze the clinical and immunologic characteristics of a cohort of patients with ICL according to the Centers for Disease Control criteria. We recruited 40 patients (24 female) of mean age 44.2 ± 12.2 (19–70) years. Patients underwent T-lymphocyte phenotyping and lymphoproliferation assay at diagnosis, and experiments related to thymic function and interferon (IFN)-γ release by natural killer (NK) cell were performed. Mean follow-up was 6.9 ± 6.7 (0.14–24.3) years. Infectious, autoimmune, and neoplastic events were recorded, as were outcomes of interleukin 2 therapy. In all, 25 patients had opportunistic infections (12 with human papillomavirus infection), 14 had autoimmune symptoms, 5 had malignancies, and 8 had mild or no symptoms. At the time of diagnosis, the mean cell counts were as follows: mean CD4 cell count: 127/mm3 (range, 4–294); mean CD8: 236/mm3 (range, 1–1293); mean CD19: 113/mm3 (range, 3–547); and mean NK cell count: 122/mm3 (range, 5–416). Most patients had deficiency in CD8, CD19, and/or NK cells. Cytotoxic function of NK cells was normal, and patients with infections had a significantly lower NK cell count than those without (p = 0.01). Patients with autoimmune manifestations had increased CD8 T-cell count. Proliferation of thymic precursors, as assessed by T-cell rearrangement excision circles, was increased. Six patients died (15%). CD4 T-cell count <150/mm3 and NK cell count <100/mm3 were predictors of death. In conclusion, ICL is a heterogeneous disorder often associated with deficiencies in CD8, CD19, and/or NK cells. Long-term prognosis may be related to initial CD4 and NK cell deficiency. PMID:24646462

  9. Periodicity Analysis of the Spectral Index in 3c 273 and 3c 446

    NASA Astrophysics Data System (ADS)

    Yuan, Yu-Hai; Fan, Jun-Hui

    In this work, we used the preliminary data of University of Michigan Radio Astronomy Observatory (UMRAO) for the spectral index calculation for two blazars, 3C 273 (1226+023) and 3C 446 (2223-052), and found that the spectral indices are variable. Therefore, we used three methods (Jurkevich method (J), the discrete correlation analysis (D), and the Periodogram method (P)) to investigate the period in the spectral index variation curves. The results show that 3C 273 has a quasi-period of 8.8 ± 1.3 yr, and 3C 446 has a period of 5.8 ± 1.2 yr.

  10. Idiopathic encapsulating peritonitis: report of two cases.

    PubMed

    Da Luz, Magda Maria Profeta; Barral, Sumara Marques; Barral, Carlyle Marques; Bechara, Cristiane De Souza; Lacerda-Filho, Antônio

    2011-12-01

    This report presents two cases of young males who developed the rare idiopathic form of sclerosing encapsulating peritonitis (SEP) presented as partial bowel obstruction, both diagnosed during surgical treatment, with satisfactory outcomes. Sclerosing encapsulating peritonitis is a rare and enigmatic condition, characterized by intraperitoneal fibrosclerosis, which causes intestinal obstruction. It is a chronic entity with a poorly elucidated pathophysiology, leading to the constitution of a thick white nacreous fibrosis membrane that wraps the bowel in a concertina-like fashion with some adhesions configuring an intra-abdominal cocoon. Sclerosing encapsulating peritonitis is reported in a wide variety of patients, including those who have undergone peritoneal dialysis, young adolescent girls, cirrhotic patients after peritoneal-venous shunting, and patients treated with β-blockers. Nevertheless, the etiology of SEP remains obscure. This entity presents many difficulties in preoperative diagnosis because of its peculiar characteristics. Recognition of the SEP results in proper management and prevents unnecessary bowel resection. Regardless of cause, the treatment of the obstruction is surgical, with dissection of the encasing membrane from the intestine and separation of adherent loops of small bowel until they are laid free and returned to their normal configuration. The prognosis after appropriate surgical therapy is good, but depends on coexisting diseases. PMID:21969199

  11. The optical variability of 3C 345

    NASA Astrophysics Data System (ADS)

    Kidger, M. R.

    1989-12-01

    The behavior of the light curve of 3C 345 is analyzed using all B magnitudes available in the literature, thus extending the number of points in the compiled light curve of this object by a factor of more than 50 percent and the coverage by about 7 yrs, in comparison with the light curve compiled by Kidger and Beckman (1986). In addition, a new and better m(B) - m(pg) correction is applied to all the data. Results of the analysis demonstrate that the light curve of 3C 345 is effectively aperiodic and that the power spectrum is not continuous, in the sense that even overlapping sections of light curve have very distinct power spectra. This behavior indicates that the curve variations are random. This conclusion is supported by the use of the Jurkevich (1971) V(m)-sq statistic.

  12. The Optical Variability of 3C273

    NASA Astrophysics Data System (ADS)

    Lin, Rui-Guang

    2001-06-01

    B-band measurements of 3C273 over some 110 years are compiled and used in a search for periodicities using the Jurkevich method. Periods of 2.0, 13.65±0.20 and 22.5±0.2 yr are found. If the long-term periodicity is from the instability of a slim disk, then the periodicity (~ 13-yr or ~ 22-yr) suggests masses of 107 Msun for the central black holes.

  13. Idiopathic focal epilepsies: the "lost tribe".

    PubMed

    Pal, Deb K; Ferrie, Colin; Addis, Laura; Akiyama, Tomoyuki; Capovilla, Giuseppe; Caraballo, Roberto; de Saint-Martin, Anne; Fejerman, Natalio; Guerrini, Renzo; Hamandi, Khalid; Helbig, Ingo; Ioannides, Andreas A; Kobayashi, Katsuhiro; Lal, Dennis; Lesca, Gaetan; Muhle, Hiltrud; Neubauer, Bernd A; Pisano, Tiziana; Rudolf, Gabrielle; Seegmuller, Caroline; Shibata, Takashi; Smith, Anna; Striano, Pasquale; Strug, Lisa J; Szepetowski, Pierre; Valeta, Thalia; Yoshinaga, Harumi; Koutroumanidis, Michalis

    2016-09-01

    The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community. Most neurologists would recognise the core syndromes of IFE to comprise: benign epilepsy of childhood with centro-temporal spikes or Rolandic epilepsy (BECTS/RE); Panayiotopoulos syndrome; and the idiopathic occipital epilepsies (Gastaut and photosensitive types). The Landau-Kleffner syndrome and the related (idiopathic) epilepsy with continuous spikes and waves in sleep (CSWS or ESES) are also often included, both as a consequence of the shared morphology of the interictal discharges and their potential evolution from core syndromes, for example, CSWS from BECTS. Atypical benign focal epilepsy of childhood also has shared electro-clinical features warranting inclusion. In addition, a number of less well-defined syndromes of IFE have been proposed, including benign childhood seizures with affective symptoms, benign childhood epilepsy with parietal spikes, benign childhood seizures with frontal or midline spikes, and benign focal seizures of adolescence. The

  14. Genetics and Idiopathic Interstitial Pneumonias.

    PubMed

    Chu, Sarah G; El-Chemaly, Souheil; Rosas, Ivan O

    2016-06-01

    Significant progress has been made in elucidating the genetics of parenchymal lung diseases, particularly idiopathic interstitial pneumonias (IIPs). IIPs are a heterogeneous group of diffuse interstitial lung diseases of uncertain etiology, diagnosed only after known causes of interstitial lung disease have been excluded. Idiopathic pulmonary fibrosis is the most common IIP. Through candidate gene approaches and genome wide association studies, much light has been shed on the genetic origins of IIPs, enhancing our understanding of risk factors and pathogenesis. However, significant work remains to be accomplished in identifying novel genetic variants and characterizing the function of validated candidate genes in lung pathobiology, their interplay with environmental factors, and ultimately translating these discoveries to patient care. PMID:27231858

  15. Treatment of Idiopathic Membranous Nephropathy

    PubMed Central

    Austin, Howard A.

    2012-01-01

    Exciting progress recently has been made in our understanding of idiopathic membranous nephropathy, as well as treatment of this disease. Here, we review important advances regarding the pathogenesis of membranous nephropathy. We will also review the current approach to treatment and its limitations and will highlight new therapies that are currently being explored for this disease including Rituximab, mycophenolate mofetil, and adrenocorticotropic hormone, with an emphasis on results of the most recent clinical trials. PMID:22859855

  16. The peculiar radio galaxy 3C 433

    NASA Technical Reports Server (NTRS)

    Van Breugel, W.; Helfand, D.; Balick, B.; Heckman, T.; Miley, G.

    1983-01-01

    Radio, optical and X-ray observations are presented of the peculiar radio galaxy 3C 433, a Seyfert 2 object with luminosity an order of magnitude greater than that expected from its complex, shell-type morphology. Observations conducted at 6 and 12 cm with the VLA and at 21 cm with the Westerbork telescope show a striking asymmetry between the northern and southern radio emissions, and an overall X-shaped morphology. Optical observations using the Video Camera and High Gain Video Spectrometer on the 4-m telescope and the Intensified Image Dissector Scanner on the 2.1-m telescope at Kitt Peak confirm the identification of the source with a pair of bright galaxies. Observations in the X-ray from the Einstein Observatory IPC reveal an unresolved source at the position of 3C 433, as well as two serendipitous X-ray sources. The observations may be used to explain the overall structure of the source either in terms of tidal torquing or precessing models of double galaxies; however, it is argued that the tidal torquing model requires fewer assumptions to account for the brightness asymmetry.

  17. Laparoscopic detorsion of isolated idiopathic fallopian tube torsion: conservative treatment in a 13-year-old girl.

    PubMed

    Ardıçlı, Burak; Ekinci, Saniye; Oğuz, Berna; Haliloğlu, Mithat; Tanyel, F Cahit; Karnak, İbrahim

    2013-01-01

    Isolated fallopian tube torsion (IFTT) is a rare entity in adolescent girls. Invasive surgery, salpingectomy, was used during the initial surgery or at the time of recurrent torsion in 87% of previously reported cases. Herein, the authors present an adolescent premenarcheal girl with idiopathic IFTT, which was treated by laparoscopic detorsion, to emphasize that tubal torsion should be included in the list of differential diagnoses of abdominal pain in adolescent girls. Minimally invasive, organ-saving surgery should be done, and the child should be followed up for recurrent torsion. PMID:24292044

  18. Two Active Nuclei in 3C 294

    NASA Astrophysics Data System (ADS)

    Stockton, Alan; Canalizo, Gabriela; Nelan, E. P.; Ridgway, Susan E.

    2004-01-01

    The z=1.786 radio galaxy 3C 294 lies < 10" from a 12 mag star and has been the target of at least three previous investigations using adaptive optics (AO) imaging. A major problem in interpreting these results is the uncertainty in the precise alignment of the radio structure with the H- or K-band AO imaging. Here we report observations of the position of the AO guide star with the Hubble Space Telescope Fine Guidance Sensor, which, together with positions from the second United States Naval Observatory's CCD Astrograph Catalog (UCAC2), allow us to register the infrared and radio frames to an accuracy of better than 0.1". The result is that the nuclear compact radio source is not coincident with the brightest discrete object in the AO image, an essentially unresolved source on the eastern side of the light distribution, as Quirrenbach and coworkers had suggested. Instead, the radio source is centered about 0.9" to the west of this object, on one of the two apparently real peaks in a region of diffuse emission. Nevertheless, the conclusion of Quirrenbach and coworkers that 3C 294 involves an ongoing merger appears to be correct: analysis of a recent deep Chandra image of 3C 294 obtained from the archive shows that the nucleus comprises two X-ray sources, which are coincident with the radio nucleus and the eastern stellar object. The X-ray/optical flux ratio of the latter makes it extremely unlikely that it is a foreground Galactic star. Based in part on observations made with the NASA/ESA Hubble Space Telescope, obtained at the Space Telescope Science Institute, which is operated by the Association of Universities for Research in Astronomy (AURA), Inc., under NASA contract NAS5-26555. These observations are associated with proposal 08315. Based in part on data collected at Subaru Telescope, which is operated by the National Astronomical Observatory of Japan. Some of the data presented herein were obtained at the W. M. Keck Observatory, which is operated as a

  19. Double Faraday rotation toward 3C 27

    NASA Astrophysics Data System (ADS)

    Goldstein, S. J., Jr.; Reed, J. A.

    1984-08-01

    From observations of the integrated flux of 3C 27 with the NRAO 140 foot (43 m) telescope at 40 frequencies between 1250 and 1445 MHz, the authors deduce rotation measures of 165±15 and -104±4 rad m-2. Since the source (assumed to be a radio galaxy) has components 45arcsec apart, it is concluded that the net magnetic field reverses between these directions. One explanation is that a large magnetic field surrounding the central galaxy of the distant source covers one component but not the other. Another explanation is that our Galaxy contains a dipole field with a scale of order 1 pc. One component of the distant source is seen inside the current loop associated with the dipole field, while the other is seen outside the loop.

  20. The NOTOM hypothesis for idiopathic scoliosis: is it nullified by the delayed puberty of female rhythmic gymnasts and ballet dancers with scoliosis?

    PubMed

    Burwell, R G; Dangerfield, P H

    2002-01-01

    Nachemson suggested that there are more girls than boys with progressive adolescent idiopathic because of a different timing between skeletal maturation and postural maturation in the sexes during adolescence. We termed Nachemson's concept the neuro-osseous timing of maturation (NOTOM) hypothesis and used it to propose a possible medical treatment for idiopathic scoliosis by delaying puberty through the pituitary using gonadorelin analogues as in idiopathic precocious puberty. The prevalence of scoliosis is reported to be increased in rhythmic gymnasts (RGs) in Bulgaria and in ballet dancers (BDs) in the USA. Both groups exhibit delayed puberty, which, at first sight, nullifies the NOTOM hypothesis for idiopathic scoliosis. While constitutional and environmental factors may determine these scolioses, the different curve types in RGs and BDs suggest that the exercise pattern over many years determines which type of scoliosis develops, although not the curve severity. We support the view that scoliotic RGs should be included in a group of sports-associated scoliosis separate from idiopathic scoliosis. Hence the delayed puberty of RGs and BDs with scoliosis does not nullify the NOTOM hypothesis as their scolioses are not idiopathic. There is a need to focus research on such subjects who have defined constitutional and environmental factors related to their scolioses. PMID:15457686

  1. Nintedanib in idiopathic pulmonary fibrosis.

    PubMed

    Woodcock, H V; Maher, T M

    2015-06-01

    Idiopathic pulmonary fibrosis (IPF) conveys a median survival of 3 years and until recently has lacked effective therapies. Nintedanib, an orally available, small-molecule tyrosine kinase inhibitor with selectivity for vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF) and fibroblast growth factor (FGF) receptors has recently been shown, in two pivotal phase III studies, to effectively slow IPF disease progression. Consequently, nintedanib was given accelerated approval by the FDA in October 2014 for the treatment of IPF. This monograph explores the preclinical rationale for the antifibrotic role of nintedanib and provides an overview of the available data on pharmacokinetics, efficacy and safety. PMID:26261848

  2. Multiwavelength observations of giant radio galaxy 3C 35 and 3C 284

    NASA Astrophysics Data System (ADS)

    Pal, Sabyasachi; Chakrabarti, Sandip Kumar; Patra, Dusmanta; Konar, Chiranjib

    2016-07-01

    We report multi wavelength observations of large radio galaxy 3C35 and 3C284. The low frequency observations were done with the Giant Metrewave Radio Telescope (GMRT) starting from 150 MHz. The high frequency observations were done with Jansky Very Large Array (JVLA). Our main motivation for these observations is to estimate the spectral ages of these galaxies and to examine any proof of extended emission at low radio frequencies due to an earlier cycle of activity. The spectral age is measured by fitting the spectra with different spectral ageing models e.g. Kardashev-Pacholczyk (KP), Jaffe-Perola (JP) and Continuous Injection (CI).

  3. THE SUZAKU VIEW OF 3C 382

    SciTech Connect

    Sambruna, R. M.; Gliozzi, M.; Tombesi, F.; Braito, V.; Ballo, L.; Reynolds, C. S.

    2011-06-20

    We present a long (116 ks) Suzaku observation of the broad-line radio galaxy (BLRG) 3C 382 acquired in 2007 April. A Swift BAT spectrum in 15-200 keV from the 58 month survey is also analyzed, together with an archival XMM-Newton EPIC exposure of 20 ks obtained one year after Suzaku. Our main result is the finding with Suzaku of a broad Fe K line with a relativistic profile consistent with emission from an accretion disk at tens of gravitational radii from the central black hole. The XIS data indicate emission from highly ionized iron and allow us to set tight, albeit model-dependent, constraints on the inner and outer radii of the disk reflecting region, r{sub in} {approx_equal} 10 r{sub g} and r{sub out} {approx_equal} 20 r{sub g} , respectively, and on the disk inclination, i {approx_equal} 30{sup 0}. Two ionized reflection components are possibly observed, with similar contributions of {approx}10% to the total continuum-a highly ionized one, with log{xi} {approx_equal} 3 erg s{sup -1} cm, which successfully models the relativistic line, and a mildly ionized one, with log{xi} {approx_equal} 1.5 erg s{sup -1} cm, which models the narrow Fe K{alpha} and high energy hump. When both these components are included, there is no further requirement for an additional blackbody soft excess below 2 keV. The Suzaku data confirm the presence of a warm absorber previously known from grating studies. After accounting for all the spectral features, the intrinsic photon index of the X-ray continuum is {Gamma}{sub x} {approx_equal} 1.8 with a cutoff energy at {approx}200 keV, consistent with Comptonization models and excluding jet-related emission up to these energies. Comparison of the X-ray properties of 3C 382 and other BLRGs to Seyferts recently observed with Suzaku and BAT confirms the idea that the distinction between radio-loud and radio-quiet active galactic nucleus at X-rays is blurred. The two classes form a continuum distribution in terms of X-ray photon index

  4. Multifrequency Monitoring of 3C 120, 3C 279, and PKS 1510--089

    NASA Technical Reports Server (NTRS)

    Jorstad, S. G.; Marscher, A. P.; Aller, M. F.; Balonek, T. J.; Gomez, J.-L.; McHardy, I. M.; Terasranta, H.; Raiteri, C.; Tosti, G.

    2001-01-01

    We analyze contemporaneous X-ray, optical, and radio light curves of 3C 120, ABC 279, and PKS 1510-089 on timescales from a few to hundreds of days over a 3-5 year period. The results show the diverse connections between variability properties at different frequencies for different blazers.

  5. Idiopathic epilepsy and school achievement.

    PubMed

    Sturniolo, M G; Galletti, F

    1994-05-01

    Forty one children (20 boys, 21 girls) aged 6-10.8 years (mean age 8.6 years) who were affected with idiopathic epilepsy underwent neuropsychological (Wechsler Intelligence Scale for Children, Bender test) and behavioural assessment (Personality Inventory for Children; this was also used in a matched control group). Further information was obtained by teachers' reports. School underachievement occurred in 25 children (61%). Statistical analysis showed no influence of sex, social background, age of onset, seizure type, duration of illness, features seen on electroencephalography, and treatment. School failure was due to poor performance in almost all academic fields, and was associated with higher visuomotor impairment; children showing good school performance had a higher mean IQ and less visuomotor impairment. The behaviour of children with epilepsy who had a good academic performance did not differ from that of their healthy peers. Emotional maladjustment (social skill impairment, depression, poor motivation, and low self esteem) was associated with poor school performance. Such problems, that may complicate the course of idiopathic epilepsy and require an appropriate educational programme, should be carefully considered by the clinician. PMID:8017966

  6. The resolved outflow from 3C 48

    SciTech Connect

    Shih, Hsin-Yi; Stockton, Alan E-mail: stockton@ifa.hawaii.edu

    2014-10-20

    We investigate the properties of the high-velocity outflow driven by the young radio jet of 3C 48, a compact-steep-spectrum source. We use the Space Telescope Imaging Spectrograph on board the Hubble Space Telecope to obtain (1) low-resolution UV and optical spectra and (2) multi-slit medium-resolution spectra of the ionized outflow. With supporting data from ground-based spectrographs, we are able to accurately measure the ratios of diagnostic emission lines such as [O III] λ5007, [O III] λ3727, [N II] λ6548, Hα, Hβ, [Ne V] λ3425, and [Ne III] λ3869. We fit the observed emission-line ratios using a range of ionization models, powered by active galactic nucleus (AGN) radiation and shocks, produced by the MAPPINGS code. We have determined that AGN radiation is likely the dominant ionization source. The outflow's density is estimated to be in the range n = 10{sup 3}-10{sup 4} cm{sup –3}, the mass is ∼6 × 10{sup 6} M {sub ☉}, and the metallicity is likely equal to or higher than solar. Compared with the typical outflows associated with more evolved radio jets, this young outflow is denser, less massive, and more metal rich. Multi-slit observations allow us to construct a two-dimensional velocity map of the outflow that shows a wide range of velocities with distinct velocity components, suggesting a wide-angle clumpy outflow.

  7. Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis.

    PubMed

    Siu, K K; Li, Rever; Lam, S Y

    2015-04-01

    This report demonstrates pulmonary haemorrhage as a differential cause of anaemia. Idiopathic pulmonary hemosiderosis is a rare disease in children; it is classically described as a triad of haemoptysis, pulmonary infiltrates on chest radiograph, and iron-deficiency anaemia. However, anaemia may be the only presenting feature of idiopathic pulmonary hemosiderosis in children due to occult pulmonary haemorrhage. In addition, the serum ferritin is falsely high in idiopathic pulmonary hemosiderosis which increases the diagnostic difficulty. We recommend that pulmonary haemorrhage be suspected in any child presenting with iron-deficiency anaemia and persistent bilateral pulmonary infiltrates. PMID:25904566

  8. Photosynthesis of C3, C3-C4, and C4 grasses at glacial CO2.

    PubMed

    Pinto, Harshini; Sharwood, Robert E; Tissue, David T; Ghannoum, Oula

    2014-07-01

    Most physiology comparisons of C3 and C4 plants are made under current or elevated concentrations of atmospheric CO2 which do not reflect the low CO2 environment under which C4 photosynthesis has evolved. Accordingly, photosynthetic nitrogen (PNUE) and water (PWUE) use efficiency, and the activity of the photosynthetic carboxylases [Rubisco and phosphoenolpyruvate carboxylase (PEPC)] and decarboxylases [NADP-malic enzyme (NADP-ME) and phosphoenolpyruvate carboxykinase (PEP-CK)] were compared in eight C4 grasses with NAD-ME, PCK, and NADP-ME subtypes, one C3 grass, and one C3-C4 grass grown under ambient (400 μl l(-1)) and glacial (180 μl l(-1)) CO2. Glacial CO2 caused a smaller reduction of photosynthesis and a greater increase of stomatal conductance in C4 relative to C3 and C3-C4 species. Panicum bisulcatum (C3) acclimated to glacial [CO2] by doubling Rubisco activity, while Rubisco was unchanged in Panicum milioides (C3-C4), possibly due to its high leaf N and Rubisco contents. Glacial CO2 up-regulated Rubisco and PEPC activities in concert for several C4 grasses, while NADP-ME and PEP-CK activities were unchanged, reflecting the high control exerted by the carboxylases relative to the decarboxylases on the efficiency of C4 metabolism. Despite having larger stomatal conductance at glacial CO2, C4 species maintained greater PWUE and PNUE relative to C3-C4 and C3 species due to higher photosynthetic rates. Relative to other C4 subtypes, NAD-ME and PEP-CK grasses had the highest PWUE and PNUE, respectively; relative to C3, the C3-C4 grass had higher PWUE and similar PNUE at glacial CO2. Biomass accumulation was reduced by glacial CO2 in the C3 grass relative to the C3-C4 grass, while biomass was less reduced in NAD-ME grasses compared with NADP-ME and PCK grasses. Under glacial CO2, high resource use efficiency offers a key evolutionary advantage for the transition from C3 to C4 photosynthesis in water- and nutrient-limited environments. PMID:24723409

  9. Factors associated with improvement in disease activity following initiation of etanercept in children and young people with Juvenile Idiopathic Arthritis: results from the British Society for Paediatric and Adolescent Rheumatology Etanercept Cohort Study

    PubMed Central

    Kearsley-Fleet, Lianne; Davies, Rebecca; Lunt, Mark; Southwood, Taunton R.

    2016-01-01

    Objectives. The objectives of this study were to investigate change in disease activity, and explore factors associated with response, in children with JIA over the initial year of etanercept treatment. Methods. This analysis included children with JIA starting etanercept in the British Society for Paediatric and Adolescent Rheumatology Etanercept Cohort Study. Response was assessed using change in juvenile arthritis disease activity score-71 (JADAS-71), an excellent response (ACR Pedi 90), and achieving minimal disease activity (MDA) at 1 year. Change in JADAS-71 was evaluated over time. Multivariable backward stepwise logistic regression was performed to identify factors associated with ACR Pedi 90 and MDA. Results. A total of 496 children were included. Over the first year, 17 stopped due to inefficacy, 9 due to adverse events and 7 for other reasons. One child stopped for remission. At 1 year, 74, 69 and 38% reached ACR Pedi 30, 50 and 90, respectively, and 48% had achieved MDA. Independent predictors of achieving ACR Pedi 90 at 1 year included shorter disease duration [odds ratio (OR) 0.91; 95% CI: 0.85, 0.97)], no concurrent oral corticosteroid use (OR 0.48; 95% CI: 0.29, 0.80) and history of uveitis (OR 2.26; 95% CI: 1.08, 4.71). Independent predictors of achieving MDA at 1 year included younger patients (OR 0.60; 95% CI: 0.38, 0.95), and disease not treated with concurrent oral corticosteroids (OR 0.57; 95% CI: 0.35, 0.93). Conclusion. Among this real-world cohort of children with severe JIA, a significant proportion of children achieved an excellent ACR Pedi response and MDA within 1 year of starting etanercept, although few clinical factors could predict this outcome. PMID:26721878

  10. Lateral Semicircular Canal Asymmetry in Idiopathic Scoliosis: An Early Link between Biomechanical, Hormonal and Neurosensory Theories?

    PubMed Central

    Hitier, Martin; Hamon, Michèle; Denise, Pierre; Lacoudre, Julien; Thenint, Marie-Aude; Mallet, Jean-François; Moreau, Sylvain; Quarck, Gaëlle

    2015-01-01

    Introduction Despite its high incidence and severe morbidity, the physiopathogenesis of adolescent idiopathic scoliosis (AIS) is still unknown. Here, we looked for early anomalies in AIS which are likely to be the cause of spinal deformity and could also be targeted by early treatments. We focused on the vestibular system, which is suspected of acting in AIS pathogenesis and which exhibits an end organ with size and shape fixed before birth. We hypothesize that, in adolescents with idiopathic scoliosis, vestibular morphological anomalies were already present at birth and could possibly have caused other abnormalities. Materials and Methods The vestibular organ of 18 adolescents with AIS and 9 controls were evaluated with MRI in a prospective case controlled study. We studied lateral semicircular canal orientation and the three semicircular canal positions relative to the midline. Lateral semicircular canal function was also evaluated by vestibulonystagmography after bithermal caloric stimulation. Results The left lateral semicircular canal was more vertical and further from the midline in AIS (p = 0.01) and these two parameters were highly correlated (r = -0.6; p = 0.02). These morphological anomalies were associated with functional anomalies in AIS (lower excitability, higher canal paresis), but were not significantly different from controls (p>0.05). Conclusion Adolescents with idiopathic scoliosis exhibit morphological vestibular asymmetry, probably determined well before birth. Since the vestibular system influences the vestibulospinal pathway, the hypothalamus, and the cerebellum, this indicates that the vestibular system is a possible cause of later morphological, hormonal and neurosensory anomalies observed in AIS. Moreover, the simple lateral SCC MRI measurement demonstrated here could be used for early detection of AIS, selection of children for close follow-up, and initiation of preventive treatment before spinal deformity occurs. PMID:26186348

  11. The Resolved Outflow from 3C 48

    NASA Astrophysics Data System (ADS)

    Shih, Hsin-Yi; Stockton, Alan

    2014-10-01

    We investigate the properties of the high-velocity outflow driven by the young radio jet of 3C 48, a compact-steep-spectrum source. We use the Space Telescope Imaging Spectrograph on board the Hubble Space Telecope to obtain (1) low-resolution UV and optical spectra and (2) multi-slit medium-resolution spectra of the ionized outflow. With supporting data from ground-based spectrographs, we are able to accurately measure the ratios of diagnostic emission lines such as [O III] λ5007, [O III] λ3727, [N II] λ6548, Hα, Hβ, [Ne V] λ3425, and [Ne III] λ3869. We fit the observed emission-line ratios using a range of ionization models, powered by active galactic nucleus (AGN) radiation and shocks, produced by the MAPPINGS code. We have determined that AGN radiation is likely the dominant ionization source. The outflow's density is estimated to be in the range n = 103-104 cm-3, the mass is ~6 × 106 M ⊙, and the metallicity is likely equal to or higher than solar. Compared with the typical outflows associated with more evolved radio jets, this young outflow is denser, less massive, and more metal rich. Multi-slit observations allow us to construct a two-dimensional velocity map of the outflow that shows a wide range of velocities with distinct velocity components, suggesting a wide-angle clumpy outflow. Based in part on observations made with the NASA/ESA Hubble Space Telescope, obtained at the Space Telescope Science Institute, which is operated by the Association of Universities for Research in Astronomy, Inc., under NASA contract NAS 5-26555. These observations are associated with program GO-11574. Some of the data presented herein were obtained at the W. M. Keck Observatory, which is operated as a scientific partnership among the California Institute of Technology, the University of California and the National Aeronautics and Space Administration. The Observatory was made possible by the generous financial support of the W. M. Keck Foundation. Some of the

  12. THE ACCELERATING JET OF 3C 279

    SciTech Connect

    Bloom, S. D.; Fromm, C. M.; Ros, E.

    2013-01-01

    Analysis of the proper motions of the subparsec scale jet of the quasar 3C 279 at 15 GHz with the Very Long Baseline Array shows significant accelerations in four of nine superluminal features. Analysis of these motions is combined with the analysis of flux density light curves to constrain values of Lorentz factor and viewing angle (and their derivatives) for each component. The data for each of these components are consistent with significant changes to the Lorentz factor, viewing angle, and azimuthal angle, suggesting jet bending with changes in speed. We see that for these observed components Lorentz factors are in the range {Gamma} = 10-41, viewing angles are in the range thetav = 0. Degree-Sign 1-5. Degree-Sign 0, and intrinsic (source frame) flux density is in the range, F{sub {nu},int} 1.5 Multiplication-Sign 10{sup -9}-1.5 Multiplication-Sign 10{sup -5} Jy. Considering individual components, the Lorentz factors vary from {Gamma} = 11-16 for C1, {Gamma} = 31-41 for C5, {Gamma} = 29-41 for C6, and {Gamma} = 9-12 for C8, indicating that there is no single underlying flow speed to the jet and likely we are seeing pattern speeds from shocks in the jet. The viewing angles vary in time from 0. Degree-Sign 6 to 1. Degree-Sign 5 in the case of C1 (the least extreme example), from 0. Degree-Sign 5 to 5. Degree-Sign 0 in the case of C8, and from 0. Degree-Sign 1 to 0. Degree-Sign 9 for C5 (the last two being the most extreme examples). The intrinsic flux density varies by factors from 1.4 for C8 and 430 for C5. Theoretical analysis of the accelerations also indicates potential jet bending. In addition, for one component, C5, polarization measurements also set limits to the trajectory of the jet.

  13. Cough in idiopathic pulmonary fibrosis.

    PubMed

    van Manen, Mirjam J G; Birring, Surinder S; Vancheri, Carlo; Cottin, Vincent; Renzoni, Elisabetta A; Russell, Anne-Marie; Wijsenbeek, Marlies S

    2016-09-01

    Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably "multifactorial" and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF. PMID:27581827

  14. Antidepressants in chronic idiopathic urticaria.

    PubMed

    Yasharpour, Michelle R; Randhawa, Inderpal

    2011-01-01

    Chronic idiopathic urticaria (CIU) is a common disease estimated to affect 0.1% of the population and can be very difficult to treat. Many psychotropic medications have been reported to be successful in treating refractory CIU. The purpose of this article was to discuss the pathophysiology of chronic urticaria and provide practicing allergists and dermatologists alternative treatment options in the management of refractory CIU, especially in those who have concurrent psychiatric comorbidity. A review was performed of pertinent literature pertaining to the pathophysiology of CIU and the many psychotropic medications reportedly successful in disease management. Although more research is needed, this article serves to broaden the mind of the physician treating CIU. PMID:22221435

  15. Pathology of Idiopathic Interstitial Pneumonias

    PubMed Central

    Hashisako, Mikiko; Fukuoka, Junya

    2015-01-01

    The updated classification of idiopathic interstitial pneumonias (IIPs) in 2013 by American Thoracic Society/European Respiratory Society included several important revisions to the categories described in the 2002 classification. In the updated classification, lymphoid interstitial pneumonia (LIP) was moved from major to rare IIPs, pleuroparenchymal fibroelastosis (PPFE) was newly included in the rare IIPs, acute fibrinous and organizing pneumonia (AFOP) and interstitial pneumonias with a bronchiolocentric distribution are recognized as rare histologic patterns, and unclassifiable IIP (UCIP) was classified as an IIP. However, recent reports indicate the areas of concern that may require further evaluation. Here, we describe the histopathologic features of the updated IIPs and their rare histologic patterns and also point out some of the issues to be considered in this context. PMID:26949346

  16. Epidemiology of idiopathic pulmonary fibrosis

    PubMed Central

    Ley, Brett; Collard, Harold R

    2013-01-01

    Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence) and public health impact (ie, health care costs and resource utilization). Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. PMID:24348069

  17. Epigenomics of idiopathic pulmonary fibrosis

    PubMed Central

    Yang, Ivana V

    2012-01-01

    Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology. Development of IPF is influenced by both genetic and environmental factors. Gene-expression profiling studies have taught us quite a bit about the biology of this fatal disease, but epigenetic marks may be the missing link that connects the environmental exposure in genetically predisposed individuals to transcriptome changes associated with the development of IPF. This review will begin with an introduction to the disease, followed by brief summaries of studies of gene expression in IPF and epigenetic marks associated with exposures relevant to IPF. The majority of the discussion will focus on epigenetic studies conducted so far in IPF, the limitations, challenges and future directions in this field. PMID:22449190

  18. Molecular etiology of idiopathic cardiomyopathy

    PubMed Central

    Arimura, T; Hayashi, T; Kimura, A

    2007-01-01

    Summary Idiopathic cardiomyopathy (ICM) is a primary cardiac disorder associated with abnormalities of ventricular wall thickness, size of ventricular cavity, contraction, relaxation, conduction and rhythm. Over the past two decades, molecular genetic analyses have revealed that mutations in the various genes cause ICM and such information concerning the genetic basis of ICM enables us to speculate the pathogenesis of this heterogeous cardiac disease. This review focuses on the molecular pathogenesis, i.e., genetic abnormalities and functional alterations due to the mutations especially in sarcomere/cytoskeletal components, in three characteristic features of ICM, hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM) and restrictive cardiomyopathy (RCM). Understanding the functional abnormalities of the sarcomere/cytoskeletal components, in ICM, has unraveled the function of these components not only as a contractile unit but also as a pivot for transduction of biochemical signals. PMID:18646564

  19. Genetics Home Reference: idiopathic pulmonary fibrosis

    MedlinePlus

    ... However, the course of the disease is highly variable; some affected people become seriously ill within a ... idiopathic pulmonary fibrosis: an observational cohort study with independent validation. Lancet Respir Med. 2014 Jul;2(7): ...

  20. ptk7 mutant zebrafish models of congenital and idiopathic scoliosis implicate dysregulated Wnt signalling in disease

    PubMed Central

    Hayes, Madeline; Gao, Xiaochong; Yu, Lisa X; Paria, Nandina; Henkelman, R. Mark; Wise, Carol A.; Ciruna, Brian

    2014-01-01

    Scoliosis is a complex genetic disorder of the musculoskeletal system, characterized by three-dimensional rotation of the spine. Curvatures caused by malformed vertebrae (congenital scoliosis (CS)) are apparent at birth. Spinal curvatures with no underlying vertebral abnormality (idiopathic scoliosis (IS)) most commonly manifest during adolescence. The genetic and biological mechanisms responsible for IS remain poorly understood due largely to limited experimental models. Here we describe zygotic ptk7 (Zptk7) mutant zebrafish, deficient in a critical regulator of Wnt signalling, as the first genetically defined developmental model of IS. We identify a novel sequence variant within a single IS patient that disrupts PTK7 function, consistent with a role for dysregulated Wnt activity in disease pathogenesis. Furthermore, we demonstrate that embryonic loss-of-gene function in maternal-zygotic ptk7 mutants (MZptk7) leads to vertebral anomalies associated with CS. Our data suggest novel molecular origins of, and genetic links between, congenital and idiopathic forms of disease. PMID:25182715

  1. Eight Years Follow-up of a Case with Idiopathic Pulmonary Hemosiderosis After Corticosteroid Therapy.

    PubMed

    Kahraman, Hasan; Köksal, Nurhan; Ozkan, Fuat

    2012-01-01

    Idiopathic pulmonary hemosiderosis is characterized by recurrent episodes of alveolar hemorrhage, hemoptysis, and secondary iron deficiency anemia with unknown etiology. It generally emerges in childhood and adolescence periods but rarely found in adulthood. Definite diagnosis is established by appearing the hemosiderin-laden macrophages at sputum, bronchoalveolar lavage, or open lung biopsy. We reported a male patient who was born in 1975, expectorated blood since 1995, and was diagnosed in 1998. He received many blood replacements. He admitted to our clinic in 2003 with complaints of coughing up blood, shortness of breath, and tiredness. We gave the corticosteroid therapy to patient for 6-month period. After treatment, the patient did not have any complaints. Clinicians should keep in mind that idiopathic pulmonary hemosiderosis may differ in localization on chest X-ray and corticosteroid treatment should be started when diagnosis is established. PMID:22393549

  2. [The Details of Thermoesthesia-and-Algesthesia State in Patients with Idiopathic Scoliosis of III-IV Degree].

    PubMed

    Shchurova, E N; Riabykh, S O; Kobyzev, A E; Ochirova, P V

    2016-01-01

    Sensitivity in patients with adolescent idiopathic scoliosis remains insufficiently studied. The details of thermoesthesia-and-algesthesia state were studied in adolescent patients with III-IV Degree idiopathic scoliosis. Thermoesthesia-and-algesthesia was evaluated in Th1-S1 dermatomes on the right and on the left using an electric esthesiometer. Thermoesthesia-and-algesthesia disorders of different severity degree have been established to be observed in all patients in all the dermatomes studied, manifesting themselves as hypesthesia, hyperesthesia, and thermoesthesia absence. The disorders of thermoesthesia-and-algesthesia were not of local character; but they went beyond the level of the spine deformity. Maximum negative changes in thermoesthesia-and-algesthesia were registered in the dermatomes corresponding to the apex of thoracic spine deformity (Th8, Th9, Th10) where the lowest percentage of patients with normal algesthesia thresholds is observed being combined with the decreased number of patients with thermoesthesia thresholds within the norm. PMID:27188152

  3. Brief Report: Cognitive Emotion Regulation Strategies and Psychological Adjustment in Adolescents with a Chronic Disease

    ERIC Educational Resources Information Center

    Garnefski, Nadia; Koopman, Hendrik; Kraaij, Vivian; ten Cate, Rebecca

    2009-01-01

    Objective of the study was to examine how cognitive emotion regulation strategies were related to psychological maladjustment in adolescents with a chronic disease. The sample consisted of adolescents with a diagnosis of Juvenile Idiopathic Arthritis (JIA). A self-report questionnaire was used to assess Internalizing problems and Quality of Life.…

  4. Single Nucleotide Polymorphisms in Pediatric Idiopathic Nephrotic Syndrome

    PubMed Central

    Suvanto, Maija; Jahnukainen, Timo; Kestilä, Marjo; Jalanko, Hannu

    2016-01-01

    Polymorphic variants in several molecules involved in the glomerular function and drug metabolism have been implicated in the pathophysiology of pediatric idiopathic nephrotic syndrome (INS), but the results remain inconsistent. We analyzed the association of eleven allelic variants in eight genes (angiopoietin-like 4 (ANGPTL4), glypican 5 (GPC5), interleukin-13 (IL-13), macrophage migration inhibitory factor (MIF), neural nitric oxide synthetase (nNOS), multidrug resistance-1 (MDR1), glucocorticoid-induced transcript-1 (GLCCI1), and nuclear receptor subfamily-3 (NR3C1)) in 100 INS patients followed up till adulthood. We genotyped variants using PCR and direct sequencing and evaluated estimated haplotypes of MDR1 variants. The analysis revealed few differences in SNP genotype frequencies between patients and controls, or in clinical parameters among the patients. Genotype distribution of MDR1 SNPs rs1236, rs2677, and rs3435 showed significant (p < 0.05) association with different medication regimes (glucocorticoids only versus glucocorticoids plus additional immunosuppressives). Some marginal association was detected between ANGPTL4, GPC5, GLCCI1, and NR3C1 variants and different medication regimes, number of relapses, and age of onset. Conclusion. While MDR1 variant genotype distribution associated with different medication regimes, the other analyzed gene variants showed only little or marginal clinical relevance in INS. PMID:27247801

  5. Perspective: Update on Idiopathic Intracranial Hypertension

    PubMed Central

    Bruce, Beau B.; Biousse, Valérie; Newman, Nancy J.

    2011-01-01

    Purpose Provide an update on various features of idiopathic intracranial hypertension. Design Perspective. Methods Selected articles on the epidemiology, clinical and imaging features, natural history, pathophysiology, and treatment of idiopathic intracranial hypertension were reviewed and interpreted in the context of the authors’ clinical and research experience. Results Idiopathic intracranial hypertension is primarily a disease of obese women of childbearing age, but it can affect patients of any weight, sex, and age. Although a relatively rare disorder, idiopathic intracranial hypertension’s associated costs in the U.S. entail hundreds of millions of dollars. Even following treatment, headaches are frequently persistent and may require the continued involvement of a neurologist. Quality of life reductions and depression are common among idiopathic intracranial hypertension patients. However, visual dysfunction, especially visual field abnormalities, represents the major morbidity of this disorder, and serial automated perimetry remains the primary mode of patient monitoring. Patients who are men, black, very obese, or anemic are at higher risk of visual loss. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding idiopathic intracranial hypertension’s pathophysiology. Treatment studies show that lumbar puncture is a valuable treatment (in addition to its crucial diagnostic role) and that weight management is critical. However, open questions remain regarding the efficacy of acetazolamide, CSF diversion procedures, and cerebral venous stenting. Conclusions Many questions remain unanswered about idiopathic intracranial hypertension. Ongoing studies, especially an ongoing NIH-funded clinical trial of acetazolamide, should provide more insight into this important, yet poorly understood syndrome of isolated intracranial hypertension. PMID:21696699

  6. Genetics of idiopathic generalized epilepsies.

    PubMed

    Gardiner, Mark

    2005-01-01

    The idiopathic generalized epilepsies (IGEs) are considered to be primarily genetic in origin. They encompass a number of rare mendelian or monogenic epilepsies and more common forms which are familial but manifest as complex, non-mendelian traits. Recent advances have demonstrated that many monogenic IGEs are ion channelopathies. These include benign familial neonatal convulsions due to mutations in KCNQ2 or KCNQ3, generalized epilepsy with febrile seizures plus due to mutations in SCN1A, SCN2A, SCN1B, and GABRG2, autosomal-dominant juvenile myoclonic epilepsy (JME) due to a mutation in GABRA1 and mutations in CLCN2 associated with several IGE sub-types. There has also been progress in understanding the non-mendelian IGEs. A haplotype in the Malic Enzyme 2 gene, ME2, increases the risk for IGE in the homozygous state. Five missense mutations have been identified in EFHC1 in 6 of 44 families with JME. Rare sequence variants have been identified in CACNA1H in sporadic patients with childhood absence epilepsy in the Chinese Han population. These advances should lead to new approaches to diagnosis and treatment. PMID:16302872

  7. Seizures of idiopathic generalized epilepsies.

    PubMed

    Durón, Reyna M; Medina, Marco T; Martínez-Juárez, Iris E; Bailey, Julia N; Perez-Gosiengfiao, Katerina Tanya; Ramos-Ramírez, Ricardo; López-Ruiz, Minerva; Alonso, María Elisa; Ortega, Ramón H Castro; Pascual-Castroviejo, Ignacio; Machado-Salas, Jesús; Mija, Lizardo; Delgado-Escueta, Antonio V

    2005-01-01

    Idiopathic generalized epilepsies (IGEs) comprise at least 40% of epilepsies in the United States, 20% in Mexico, and 8% in Central America. Here, we review seizure phenotypes across IGE syndromes, their response to treatment and advances in molecular genetics that influence nosology. Our review included the Medline database from 1945 to 2005 and our prospectively collected Genetic Epilepsy Studies (GENESS) Consortium database. Generalized seizures occur with different and similar semiologies, frequencies, and patterns, ages at onset, and outcomes in different IGEs, suggesting common neuroanatomical pathways for seizure phenotypes. However, the same seizure phenotypes respond differently to the same treatments in different IGEs, suggesting different molecular defects across syndromes. De novo mutations in SCN1A in sporadic Dravet syndrome and germline mutations in SCN1A, SCN1B, and SCN2A in generalized epilepsies with febrile seizures plus have unraveled the heterogenous myoclonic epilepsies of infancy and early childhood. Mutations in GABRA1, GABRG2, and GABRB3 are associated with absence seizures, while mutations in CLCN2 and myoclonin/EFHC1 substantiate juvenile myoclonic epilepsy as a clinical entity. Refined understanding of seizure phenotypes, their semiology, frequencies, and patterns together with the identification of molecular lesions in IGEs continue to accelerate the development of molecular epileptology. PMID:16302874

  8. Scrotal calcinosis: idiopathic or dystrophic?

    PubMed

    Dubey, Suparna; Sharma, Rajeev; Maheshwari, Veena

    2010-01-01

    Scrotal calcinosis is a rare benign local process characterized by multiple, painless, hard scrotal nodules in the absence of any systemic metabolic disorder. Histological examination reveals extensive deposition of calcium in the dermis, which may be surrounded by histiocytes and an inflammatory giant cell reaction. Numerous theories have been propounded to explain the pathogenesis of this condition, but the principal debate revolves around whether the calcium is deposited at the site of previous epithelial cysts or the calcified nodules are purely idiopathic. This is the largest study of scrotal calcinosis to date with 100 cases, on which clinical, biochemical, radiological, cytopathological, and histopathological examinations were conducted. The histological picture shows a continuous spectrum of changes ranging from intact epithelial cysts (41.0%) - both normal and inflamed; through inflamed cysts containing calcific material in the lumen but with intact cyst wall (53.0%); calcified inflamed cysts with partial epithelial lining (11.0%); to 'naked' calcium deposits lying in the dermis (100%), sometimes compressing surrounding collagen fibres to form a pseudocyst (56.0%). The presence of normal values of calcium and phosphorus along with this spectrum of changes in histology both support the theory that these form by dystrophic calcification of epithelial cysts in a progression that involves inflammation, rupture, calcification and obliteration of the cyst wall. PMID:20178701

  9. Familial idiopathic normal pressure hydrocephalus.

    PubMed

    Huovinen, Joel; Kastinen, Sami; Komulainen, Simo; Oinas, Minna; Avellan, Cecilia; Frantzen, Janek; Rinne, Jaakko; Ronkainen, Antti; Kauppinen, Mikko; Lönnrot, Kimmo; Perola, Markus; Pyykkö, Okko T; Koivisto, Anne M; Remes, Anne M; Soininen, Hilkka; Hiltunen, Mikko; Helisalmi, Seppo; Kurki, Mitja; Jääskeläinen, Juha E; Leinonen, Ville

    2016-09-15

    Idiopathic normal pressure hydrocephalus (iNPH) is a late-onset surgically alleviated, progressive disease. We characterize a potential familial subgroup of iNPH in a nation-wide Finnish cohort of 375 shunt-operated iNPH-patients. The patients were questionnaired and phone-interviewed, whether they have relatives with either diagnosed iNPH or disease-related symptomatology. Then pedigrees of all families with more than one iNPH-case were drawn. Eighteen patients (4.8%) from 12 separate pedigrees had at least one shunt-operated relative whereas 42 patients (11%) had relatives with two or more triad symptoms. According to multivariate logistic regression analysis, familial iNPH-patients had up to 3-fold risk of clinical dementia compared to sporadic iNPH patients. This risk was independent from diagnosed Alzheimer's disease and APOE ε4 genotype. This study describes a familial entity of iNPH offering a novel approach to discover the potential genetic characteristics of iNPH. Discovered pedigrees offer an intriguing opportunity to conduct longitudinal studies targeting potential preclinical signs of iNPH. PMID:27538594

  10. Glycine decarboxylase in C3, C4 and C3-C4 intermediate species.

    PubMed

    Schulze, Stefanie; Westhoff, Peter; Gowik, Udo

    2016-06-01

    The glycine decarboxylase complex (GDC) plays a central role in photorespiration. GDC is localized in the mitochondria and together with serine hydroxymethyltransferase it converts two molecules of glycine to one molecule of serine, CO2 and NH3. Overexpression of GDC subunits in the C3 species Arabidopsis thaliana can increase the metabolic flux through the photorespiratory pathway leading to enhanced photosynthetic efficiency and consequently to an enhanced biomass production of the transgenic plants. Changing the spatial expression patterns of GDC subunits was an important step during the evolution of C3-C4 intermediate and likely also C4 plants. Restriction of the GDC activity to the bundle sheath cells led to the establishment of a photorespiratory CO2 pump. PMID:27038285

  11. Hyperplastic Breast Anomalies in the Female Adolescent Breast

    PubMed Central

    Wolfswinkel, Erik M.; Lemaine, Valerie; Weathers, William M.; Chike-Obi, Chuma J.; Xue, Amy S.; Heller, Lior

    2013-01-01

    Macromastia in adolescents is multifactorial and usually idiopathic, associated with obesity or hormonal imbalances. Less commonly, it can result from virginal or juvenile breast hypertrophy, a rare condition of unknown etiology, where an alarmingly rapid breast enlargement occurs during puberty. Breast hypertrophy in the adolescent population can have significant long-term medical and psychological impacts. Although symptoms can be severe, many plastic surgeons, pediatricians, and parents are often reluctant to surgically treat adolescent macromastia. However, reduction mammoplasty is a safe and effective treatment and may be the only way to alleviate the increased social, psychological, and physical strain caused by macromastia in adolescents. PMID:24872740

  12. On the Y-chromosome haplogroup C3c classification.

    PubMed

    Malyarchuk, Boris A; Derenko, Miroslava; Denisova, Galina

    2012-10-01

    As there are ambiguities in classification of the Y-chromosome haplogroup C3c, relatively frequent in populations of Northern Asia, we analyzed all three haplogroup-defining markers M48, M77 and M86 in C3-M217-individuals from Siberia, Eastern Asia and Eastern Europe. We have found that haplogroup C3c is characterized by the derived state at M48, whereas mutations at both M77 and M86 define subhaplogroup C3c1. The branch defined by M48 alone would belong to subhaplogroup C3c*, characteristic for some populations of Central and Eastern Siberia, such as Koryaks, Evens, Evenks and Yukaghirs. Subhaplogroup C3c* individuals could be considered as remnants of the Neolithic population of Siberia, based on the age of C3c*-short tandem repeat variation amounting to 4.5 ± 2.4 thousand years. PMID:22810113

  13. Odor-associated idiopathic anaphylaxis. A case report.

    PubMed

    Saunders, R L; Halpern, G M; Gershwin, M E

    1995-01-01

    A 44 year old woman is described who appears to have idiopathic anaphylaxis triggered by chemical odors. Her case and a general discussion of anaphylaxis are presented. The known causes of anaphylaxis and a discourse on idiopathic anaphylaxis are given. The treatment of idiopathic anaphylaxis is discussed. PMID:7631593

  14. Idiopathic Nonviral Cryoglobulinemia Treated Successfully With Rituximab.

    PubMed

    Kamel, Mahmoud; Thajudeen, Bijin; Bracamonte, Erika; Madhrira, Machaiah

    2016-01-01

    Cryoglobulinemia is a systemic inflammatory syndrome that generally involves small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes. The therapeutic management of idiopathic cryoglobulinemic vasculitis has yet to be defined because no study has evaluated the best strategies. However, treatment of severe vasculitis is traditionally based on a combination of corticosteroids and immunosuppressants or plasmapheresis, and more recently rituximab. We report a case of 77-year-old female patient diagnosed with idiopathic cryoglobulinemia, treated successfully with 6 months prednisone tapering and 2 doses of rituximab (1 g each dose). After receiving the above-mentioned treatment, her creatinine went back to normal with resolution of proteinuria and hematuria, normalization of serum complements, and significant improvement in her clinical picture. We conclude that rituximab could be an effective treatment for idiopathic cryoglobulnemia. PMID:24914502

  15. Genetic polymorphisms and idiopathic generalized epilepsies.

    PubMed

    Lucarini, Nazzareno; Verrotti, Alberto; Napolioni, Valerio; Bosco, Guido; Curatolo, Paolo

    2007-09-01

    In recent years, progress in understanding the genetic basis of idiopathic generalized epilepsies has proven challenging because of their complex inheritance patterns and genetic heterogeneity. Genetic polymorphisms offer a convenient avenue for a better understanding of the genetic basis of idiopathic generalized epilepsy by providing evidence for the involvement of a given gene in these disorders, and by clarifying its pathogenetic mechanisms. Many of these genes encode for some important central nervous system ion channels (KCNJ10, KCNJ3, KCNQ2/KCNQ3, CLCN2, GABRG2, GABRA1, SCN1B, and SCN1A), while many others encode for ubiquitary enzymes that play crucial roles in various metabolic pathways (HP, ACP1, ME2, LGI4, OPRM1, GRIK1, BRD2, EFHC1, and EFHC2). We review the main genetic polymorphisms reported in idiopathic generalized epilepsy, and discusses their possible functional significance in the pathogenesis of seizures. PMID:17765802

  16. Idiopathic non-specific interstitial pneumonia.

    PubMed

    Belloli, Elizabeth A; Beckford, Rosemarie; Hadley, Ryan; Flaherty, Kevin R

    2016-02-01

    Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi-disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP. PMID:26564810

  17. Idiopathic pulmonary fibrosis: an Australian perspective.

    PubMed

    Prasad, J; Holland, A E; Glaspole, I; Westall, G

    2016-06-01

    Idiopathic pulmonary fibrosis is a progressive interstitial lung disease of unknown aetiology with a dismal median survival of 3 years. Patients typically develop progressive dyspnoea and increasing exercise limitation. With a rising incidence and prevalence, an unpredictable disease course and limited treatment options, it is rapidly becoming an important public health concern. To date, lung transplantation has been the sole viable hope for treatment for those who qualify. However, the landscape of idiopathic pulmonary fibrosis management is changing, with the recent emergence of novel pharmacotherapy shown to have a favourable influence on the natural history of this disease. PMID:27257148

  18. Idiopathic Parkinson's disease: epidemiology, diagnosis and management.

    PubMed Central

    Ben-Shlomo, Y; Sieradzan, K

    1995-01-01

    Since the introduction of levodopa therapy for idiopathic Parkinson's disease over 20 years ago, there has been an awakening of research interest in this chronic neuro-degenerative disorder. This paper describes current understanding of the role of genetic and environmental factors in the aetiology of idiopathic Parkinson's disease and problems associated with both diagnosis and management. It briefly outlines both pharmacological and non-pharmacological options for treatment. Despite an increasing armoury of available treatments, the optimum management for this condition remains controversial. PMID:7619574

  19. Adolescent development

    MedlinePlus

    Development - adolescent; Growth and development - adolescent ... During adolescence, children develop the ability to: Understand abstract ideas. These include grasping higher math concepts, and developing moral ...

  20. Adolescent development

    MedlinePlus

    Development - adolescent; Growth and development - adolescent ... rights and privileges. Establish and maintain satisfying relationships. Adolescents will learn to share intimacy without feeling worried ...

  1. Evidence-Based of Nonoperative Treatment in Adolescent Idiopathic Scoliosis

    PubMed Central

    2014-01-01

    Until now because there are many published journals with a variety of opinions so I will stratify these articles by giving weighted value on grade evaluation which depend on each institution (written author and co-authors) and external evaluate status (SCI, SCIE, impact factor) rather than the outcomes provided by each article. Consequently, before evaluating publicized papers, study quality assessment of each interesting paper should be performed by mean of gauging the quality of evidence. Reviewing these articles, a grade of medical literature was divided into the following 5 levels as level I (randomized controlled study), level II (non-randomized controlled study), level III (case-control study), level IV (case series), and level V (expert opinions). However, in present article I concluded only involved medical literatures with weighted value of level I and II evidence. PMID:25346826

  2. Resistin in idiopathic inflammatory myopathies

    PubMed Central

    2012-01-01

    Introduction The purpose of this study was to evaluate and compare the serum levels and local expression of resistin in patients with idiopathic inflammatory myopathies to controls, and to determine the relationship between resistin levels, inflammation and disease activity. Methods Serum resistin levels were determined in 42 patients with inflammatory myopathies and 27 healthy controls. The association among resistin levels, inflammation, global disease activity and muscle strength was examined. The expression of resistin in muscle tissues from patients with inflammatory myopathies and healthy controls was evaluated. Gene expression and protein release from resistin-stimulated muscle and mononuclear cells were assessed. Results In patients with inflammatory myopathies, the serum levels of resistin were significantly higher than those observed in controls (8.53 ± 6.84 vs. 4.54 ± 1.08 ng/ml, P < 0.0001) and correlated with C-reactive protein (CRP) levels (r = 0.328, P = 0.044) and myositis disease activity assessment visual analogue scales (MYOACT) (r = 0.382, P = 0.026). Stronger association was observed between the levels of serum resistin and CRP levels (r = 0.717, P = 0.037) as well as MYOACT (r = 0.798, P = 0.007), and there was a trend towards correlation between serum resistin and myoglobin levels (r = 0.650, P = 0.067) in anti-Jo-1 positive patients. Furthermore, in patients with dermatomyositis, serum resistin levels significantly correlated with MYOACT (r = 0.667, P = 0.001), creatine kinase (r = 0.739, P = 0.001) and myoglobin levels (r = 0.791, P = 0.0003) and showed a trend towards correlation with CRP levels (r = 0.447, P = 0.067). Resistin expression in muscle tissue was significantly higher in patients with inflammatory myopathies compared to controls, and resistin induced the expression of interleukins (IL)-1β and IL-6 and monocyte chemoattractant protein (MCP)-1 in mononuclear cells but not in myocytes. Conclusions The results of this study

  3. Selective Arterial Embolization of Idiopathic Priapism

    SciTech Connect

    Cohen, Gary S.; Braunstein, Larry; Ball, David S.; Roberto, Paul J.; Reich, Jeffrey; Hanno, Phillip

    1996-11-15

    We report a case of idiopathic priapism that was only identified as high-flow or arterial priapism after drainage of the corpora cavernosa. Following failure of conservative and surgical treatment attempts, two consecutive embolizations of a unilateral penile artery were performed with gelgoam particles.

  4. Idiopathic Arterial Calcification of Infancy: Case Report

    PubMed Central

    Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa

    2015-01-01

    Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding. PMID:27252793

  5. Idiopathic Aortic Root to Right Atrial Fistula.

    PubMed

    Campisi, Salvatore; Cluzel, Armand; Vola, Marco; Fuzellier, Jean Francois

    2016-06-01

    An aorta to right atrium fistula is rare. We report a case of idiopathic aortic root to right atrial fistula with right heart failure and review the literature. doi: 10.1111/jocs.12751 (J Card Surg 2016;31:373-375). PMID:27109166

  6. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis

    PubMed Central

    Nelson, Andrew D.; Fischer, Philip R.; Reed, Ann M.; Wylam, Mark E.

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis. PMID:26171269

  7. Mineral Oil Aspiration Related Juvenile Idiopathic Arthritis.

    PubMed

    Nelson, Andrew D; Fischer, Philip R; Reed, Ann M; Wylam, Mark E

    2015-01-01

    We describe the development of rheumatoid factor-positive migratory polyarthritis in a 5-year-old male who had been administered bidaily oral mineral oil as a laxative since birth. Minor respiratory symptoms, radiographic and bronchoscopic findings were consistent with chronic lipoid pneumonia. We speculate that immune sensitization to mineral oil promoted the clinical syndrome of juvenile idiopathic arthritis. PMID:26171269

  8. Idiopathic Arterial Calcification of Infancy: Case Report.

    PubMed

    Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa

    2015-11-01

    Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding. PMID:27252793

  9. Acute surgical management in idiopathic intracranial hypertension

    PubMed Central

    Zakaria, Zaitun; Fenton, Eoin; Sattar, Muhammad Taufiq

    2012-01-01

    Idiopathic intracranial hypertension is a headache syndrome with progressive symptoms of raised intracranial pressure. Most commonly, it is a slow process where surveillance and medical management are the main treatment modalities. We describe herein an acute presentation with bilateral sixth nerve palsies, papilloedema and visual deterioration, where acute surgical intervention was a vision-saving operation. PMID:23239783

  10. A precessing relativistic jet model for 3C 449

    NASA Technical Reports Server (NTRS)

    Gower, A. C.; Hutchings, J. B.

    1982-01-01

    It is shown that the radio structure of 3C 449 can be matched with a model in which the jets are precessing and have relativistic (beta greater-than or equal to 0.4) velocities. The best-fit model implies a precession period of about 100,000 yr and a cone angle which increases with time. A similar model may be relevant for the radio structure of 3C 31. A brief discussion of the implications for 3C 449 is given.

  11. 27 CFR 21.37 - Formula No. 3-C.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 1 2010-04-01 2010-04-01 false Formula No. 3-C. 21.37 Section 21.37 Alcohol, Tobacco Products and Firearms ALCOHOL AND TOBACCO TAX AND TRADE BUREAU, DEPARTMENT OF THE TREASURY LIQUORS FORMULAS FOR DENATURED ALCOHOL AND RUM Specially Denatured Spirits Formulas and Authorized Uses § 21.37 Formula No. 3-C....

  12. 27 CFR 21.37 - Formula No. 3-C.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 1 2011-04-01 2011-04-01 false Formula No. 3-C. 21.37 Section 21.37 Alcohol, Tobacco Products and Firearms ALCOHOL AND TOBACCO TAX AND TRADE BUREAU, DEPARTMENT OF THE TREASURY LIQUORS FORMULAS FOR DENATURED ALCOHOL AND RUM Specially Denatured Spirits Formulas and Authorized Uses § 21.37 Formula No. 3-C....

  13. Idiopathic Calcinosis Cutis of the Penis

    PubMed Central

    Tschen, Jaime A.

    2012-01-01

    Background: Calcinosis cutis—the deposition of insoluble calcium salts in the skin and the soft tissue—occurs in the following five settings: calciphylaxis, dystrophic, iatrogenic, idiopathic, and metastatic. Idiopathic calcinosis cutis of the penis is rare. Purpose: This paper describes a man with idiopathic calcinosis cutis of the penis, summarizes the clinical features of previously reported men with this condition, and also reviews dystrophic, iatrogenic, and metastatic penile calcinosis. Methods: A 27-year-old Pakistan man presented with concurrent, asymptomatic, individual nodules on the right mid-ventral penile shaft and left side of scrotum and two additional papules on the right side of the scrotum. Evaluation and treatment included the excision of all lesions. Reports of patients with penile calcinosis were identified using a medical search engine (PubMed Central) and referenced citations from the published papers on this subject. Results: Microscopic examination of the patient's nodules showed idiopathic and dystrophic calcinosis cutis of the penis and scrotum, respectively; the scrotal papules were fibroepithelial polyps. Including this individual, idiopathic calcinosis cutis of the penis has only been reported in 11 men. It presents as either an asymptomatic nodule (5 patients) or multiple lesions (6 patients) of less than one-year duration, on either the penile shaft (distal in 4 patients, mid in 2 patients, both in 1 patient, and site unspecified in 1 patient) or the prepuce (3 patients) of uncircumcised men less than 30 years of age. Concurrent scrotal calcification was noted in two patients. Dermal deposits of calcium are found in the dermis—often with surrounding histiocytes and multinucleated giant cells; concurrent features of dystrophic penile shaft calcification, such as calcium within syringomas or transepidermal elimination of calcium through eccrine sweat ducts, was only noted in two men. The nodules do not recur following excision

  14. Fine Structure in 3C 120 and 3C 84. Ph.D. Thesis - Maryland Univ., 24 Aug. 1976

    NASA Technical Reports Server (NTRS)

    Hutton, L. K.

    1976-01-01

    Seven epochs of very long baseline radio interferometric observations of the Seyfert galaxies 3C 120 and 3C 84, at 3.8-cm wave length using stations at Westford, Massachusetts, Goldstone, California, Green Bank, West Virginia, and Onsala, Sweden, have been analyzed for source structure. An algorithm for reconstructing the brightness distribution of a spatially confined source from fringe amplitude and so called closure phase data has been developed and successfully applied to artificially generated test data and to data on the above mentioned sources. Over the two year time period of observation, 3C 120 was observed to consist of a double source showing apparent super relativistic expansion and separation velocities. The total flux changes comprising one outburst can be attributed to one of these components. 3C 84 showed much slower changes, evidently involving flux density changes in individual stationary components rather than relative motion.

  15. Kvn Source-Frequency Phase-Referencing Observation of 3c 66A and 3c 66B

    NASA Astrophysics Data System (ADS)

    Zhao, Guang-Yao; Jung, Taehyun; Dodson, Richard; Rioja, Maria; Sohn, Bong Won

    2015-09-01

    In this proceedings, preliminary results of the KVN Source-Frequency Phase-Referencing (SFPR) observation of 3C 66A and 3C 66B are presented. The motivation of this work is to measure the core-shift of these 2 sources and study the temporal evolution of the jet opacity. Two more sources were observed as secondary reference calibrators and each source was observed at 22, 43, and 86 GHz simultaneously. Our preliminary results show that after using the observations at the lower frequency to calibrate those at the higher frequency of the same source, the residual visibility phases for each source at the higher frequencies became more aligned, and the coherence time became much longer; also, the residual phases for different sources, within 10 degrees angular separations, follow similar trends. After reference to the nearby calibrator, the SFPRed maps were obtained as well as the astrometric measurements, i.e. the combined coreshift. The measurements were found to be affected by structural blending effects because of the large beamsize of KVN, but this can be corrected with higher resolution maps (e.g. KAVA maps). *%K Astrometry, radio continuum: galaxies, galaxies: active, galaxy: individual(3C 66A, 3C 66B), techniques: interferometric *%O 3C 66A, 3C 66B

  16. RXTE, VLBA, Optical, and Radio Monitoring of the Quasars 3C 279, PKS 1510--089, and 3C 273

    NASA Technical Reports Server (NTRS)

    Marscher, A. P.; Jorstad, S. G.; Aller, M. F.; McHardy, I. M.; Balonek, T. J.

    2001-01-01

    We are continuing our combined RXTE X-ray, VLBA imaging (at 43 GHz), optical (several observatories), and radio (University of Michigan Radio Astronomy Observatory) monitoring of the quasars 3C 279 and PKS 1510-089, and have started similar monitoring of 3C 273. X-ray flares in 3C 279 and PKS 1510-089 are associated with ejections of superluminal components. In addition, there is a close connection between the optical and X-ray variability of 3C 279. There is a strong correlation between the 14.5 GHz and X-ray variability of PKS 1510-089 in 1997 and 1998 (with the radio leading the X-ray) that becomes weaker in subsequent years. X-ray fluctuations occur on a variety of timescales in 3C 273, with a major prolonged outburst in mid-2001. The lead author will discuss the correlations in terms of inverse Compton models for the X-ray emission coupled with synchrotron models for the lower-frequency radiation. Synchrotron self-Compton models can explain the "reverse" time lag in PKS 1510-089 is well as the variable correlation between the X-ray variations and those at lower frequencies in this object and in 3C 279.

  17. Idiopathic noncirrhotic portal hypertension: current perspectives.

    PubMed

    Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d'Amati, Giulia

    2016-01-01

    The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800

  18. Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome.

    PubMed

    Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana

    2015-01-01

    Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome. PMID:26419298

  19. Genetic Aspects of Congenital and Idiopathic Scoliosis

    PubMed Central

    Giampietro, Philip F.

    2012-01-01

    Congenital and idiopathic scoliosis represent disabling conditions of the spine. While congenital scoliosis (CS) is caused by morphogenic abnormalities in vertebral development, the cause(s) for idiopathic scoliosis is (are) likely to be varied, representing alterations in skeletal growth, neuromuscular imbalances, disturbances involving communication between the brain and spine, and others. Both conditions are characterized by phenotypic and genetic heterogeneities, which contribute to the difficulties in understanding their genetic basis that investigators face. Despite the differences between these two conditions there is observational and experimental evidence supporting common genetic mechanisms. This paper focuses on the clinical features of both CS and IS and highlights genetic and environmental factors which contribute to their occurrence. It is anticipated that emerging genetic technologies and improvements in phenotypic stratification of both conditions will facilitate improved understanding of the genetic basis for these conditions and enable targeted prevention and treatment strategies. PMID:24278672

  20. Acute idiopathic polyneuropathy in the dog.

    PubMed

    Northington, J W; Brown, M J; Farnbach, G C; Steinberg, S A

    1981-08-15

    From among a large group of dogs with acute tetraparesis, we identified 10 dogs with a distinct peripheral nerve disorder. Prior to the onset of signs, all of the dogs had been healthy, and none was known to have been exposed to a neurotoxin or raccoon bite. Weakness, with hypoactive or absent segmental reflexes, became progressively worse for 1 to 21 days. Results of electromyography and nerve conduction studies invariably were compatible with a diagnosis of polyneuropathy that predominantly affected proximal nerve segments. Appearance of nerve biopsy specimens and the short time course for functional recovery suggested a demyelinative component to the disorder. The extent of recovery was variable but often rapid and complete in dogs that did not succumb to complications in the early period. Corticosteroid therapy did not demonstrably influence the outcome. This acute idiopathic polyneuropathy in the dog shares many clinical and pathologic features with idiopathic polyradiculoneuritis (Coonhound paralysis). PMID:6270046

  1. Management of severe and rigid idiopathic scoliosis.

    PubMed

    Teixeira da Silva, Luis Eduardo Carelli; de Barros, Alderico Girão Campos; de Azevedo, Gustavo Borges Laurindo

    2015-07-01

    Frequently, severe idiopathic scoliosis patients are first seen in a spine centre after years of deformity evolution, presenting with large curves, severe rib hump, shoulder and trunk imbalance and cardiorespiratory complications related to neglected scoliosis. Severe rigid idiopathic scoliosis has <25% of correction on bending films and major curve over 90°. Adequate mobilization of this type of deformity is necessary to achieve maximal correction, often requiring more extensive surgical intervention, with care taken to avoid clinical and neurological complications. Halo traction, internal temporary distraction, releases, osteotomies and apical vertebral resection are often used in combination to achieve optimal results. Indications must be tailored by surgeons considering resources, deformity characteristics and patient's profile. Vertebral resection procedures may have potential neurological and clinical risks and should be one of the last treatment options performed by experienced surgical team. Neuromonitoring is essential during these procedures. PMID:26033753

  2. Idiopathic head tremor in English bulldogs.

    PubMed

    Guevar, Julien; De Decker, Steven; Van Ham, Luc M L; Fischer, Andrea; Volk, Holger A

    2014-02-01

    Idiopathic head tremor (IHT) syndrome is a recognized but poorly characterized movement disorder in English bulldogs (EBs). The data analyzed were collected via a detailed online questionnaire and video recordings. Thirty-eight percent of the population demonstrated IHT. The first presentation was early in life. There was no sex or neutered status predisposition. The condition disappeared with time in 50% of the cases. The direction of the head movement was vertical or horizontal. The number of episodes per day and the duration of the episodes were greatly variable. The majority of episodes occurred at rest. Most of the episodes were unpredictable. And there was no alteration of the mental status for most dogs during the episodes. Stress has been reported as a suspected trigger factor. IHT in EBs can be considered an idiopathic paroxysmal movement disorder. PMID:24375621

  3. [Juvenile idiopathic arthritis: Definition and classification].

    PubMed

    Deslandre, C

    2016-04-01

    Juvenile idiopathic arthritis (JIA) is a group of diseases defined by the presence of arthritis of more than 6weeks duration in patients aged less than 16years and with unknown etiology. The international classification based on clinical and biological criteria define each type of JIA: systemic, oligoarticular, polyarticular with and without rheumatoid factor, enthesitis-related arthritis, and psoriatic arthritis. However, some discussions persist concerning systemic-onset juvenile idiopathic arthritis, whose clinical symptoms and pathogenic mechanisms are quite similar to those observed in autoinflammatory diseases, arthritis with antinuclear factors (poly- and oligoarticular) that could be considered as a homogenous group, and a family history of psoriasis that frequently led to unclassified arthritis. Better knowledge of the pathogenic mechanisms should improve the initial clinical classification with more homogeneous groups of patients and reduce the number of unclassified cases of arthritis. PMID:26968301

  4. Idiopathic noncirrhotic portal hypertension: current perspectives

    PubMed Central

    Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d’Amati, Giulia

    2016-01-01

    The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800

  5. Polarization-maintaining amplifier based on 3C fiber structures

    NASA Astrophysics Data System (ADS)

    Enokidani, Jun; Ito, Rumi; Sakurai, Tsutomu; Shin, Sumida; Tei, Kazuyoku

    2015-03-01

    Chirally-Coupled-Core (3C) fiber structure can preserve a single mode quality and even a linear polarization for a large core size. A principal advantage of fiber laser is its compatibility with monolithic integration and robust system. But so far, devices such as a combiner using the 3C fibers have not been reported. Here we report the first demonstration of such monolithic amplifier structure which contains an active fiber and a combiner based on 3C fibers. A single-stage amplifier is seeded by an EO Q-switched micro-laser and pumped by two high power fiber pigtailed 976-nm laser diodes via an in-house fabricated (2 + 1) × 1 pump signal combiner. The active fiber is based on a 3-m-long, 3C Yb-doped fiber (33 μm/250 μm core/cladding diameter with 0.06/0.46 NA). The amplifier demonstrates scaling up to 30W average power and 150 kW peak power in 0.3mJ, 2ns pulses. The beam profiles and beam qualities were characterized as its output power was varied up to 30W. The beam profile was maintained at a high beam quality of around M2=1.2. The spectral properties of the 3C fiber were also characterized as its output peak power was varied.

  6. Levocetirizine for chronic idiopathic urticaria: a review.

    PubMed

    Ducharme, Erin E; Weinberg, Jeffrey M

    2009-03-01

    Only recently available on the US market, levocetirizine has emerged as an effective new option in the treatment of chronic idiopathic urticaria (CIU). Levocetirizine is the active isomer of cetirizine and demonstrates twice the affinity for the H1 receptor compared with the parent compound. In the treatment of CIU, levocetirizine has consistently demonstrated high response rates, fast onset, and a favorable side effect profile. PMID:19271371

  7. [Persistent idiopathic facial pain and atypical odontalgia].

    PubMed

    Gaul, Charly; Ettlin, Dominik; Pfau, Doreen B

    2013-01-01

    The terms 'persistent idiopathic facial pain' (PIFP) and 'atypical odontalgia' (AO) are currently used as exclusion diagnoses for chronic toothache and chronic facial pain. Knowledge about these pain conditions in medical and dental practices is of crucial importance for the prevention of iatrogenic tissue damage by not-indicated invasive interventions, such as endodontic treatment and tooth extraction. In the present paper, etiology and pathogenesis, differential diagnostic criteria, and diagnostic approaches will be explained and relevant therapeutic principles will be outlined. PMID:23916270

  8. Invasive diagnostic techniques in idiopathic interstitial pneumonias.

    PubMed

    Poletti, Venerino; Ravaglia, Claudia; Gurioli, Carlo; Piciucchi, Sara; Dubini, Alessandra; Cavazza, Alberto; Chilosi, Marco; Rossi, Andrea; Tomassetti, Sara

    2016-01-01

    Fibrosing interstitial lung diseases (f-ILDs) represent a heterogeneous group of disorders in which the aetiology may be identified or, not infrequently, remain unknown. Establishing a correct diagnosis of a distinct f-ILD requires a multidisciplinary approach, integrating clinical profile, physiological and laboratory data, radiological appearance and, when appropriate, histological findings. Surgical lung biopsy is still considered the most important diagnostic tool as it is able to provide lung samples large enough for identification of complex patterns such as usual interstitial pneumonitis (UIP) and nonspecific interstitial pneumonitis. However, this procedure is accompanied by significant morbidity and mortality. Bronchoalveolar lavage is still a popular diagnostic tool allowing identification of alternative diagnoses in patients with suspected idiopathic pulmonary fibrosis (IPF) when an increase in lymphocytes is detected. Conventional transbronchial lung biopsy has a very low sensitivity in detecting the UIP pattern and its role in this clinical-radiological context is marginal. The introduction of less invasive methods such as transbronchial cryobiopsy show great promise to clinical practice as they can be used to obtain samples large enough to morphologically support a diagnosis of IPF or other idiopathic interstitial pneumonias, along with fewer complications. Recent advances in the field suggest that less invasive methods of lung sampling, without significant side effects, in combination with other diagnostic methods could replace the need for surgical lung biopsy in the future. Indeed, these new multidisciplinary procedures may become the main diagnostic work-up method for patients with suspected idiopathic interstitial pneumonia. PMID:26682637

  9. Clomiphene Effects on Idiopathic Premature Ejaculation

    PubMed Central

    Ketabchi, Ali Asghar

    2015-01-01

    Background: Premature ejaculation (PE) is the inability to delay ejaculation, occurring sooner than they or their partner would like during sexual activities. PE is a challenging problem that can affect sexual enjoyment and may harm relationships of couples and affect their quality of life. In idiopathic PE, several helpful techniques and medicines are recommended, but none of them has yielded satisfactory results. Objectives: Our objective in this study was to evaluate the efficacy and safety of clomiphene as a selective estrogen receptor modulator on the treatment of idiopathic PE. Patients and Methods: In a randomized clinical trial, 178 married men with idiopathic PE defined according to the Diagnostic and Statistical Manual of Mental Disorders Third Revised Version (DSM-III-R) who referred to urology clinics over a 10-month period in 2012 were randomized into two groups, namely the study (clomiphene) and control (placebo) groups. They completed self-administered questionnaires that included intravaginal ejaculatory latency time (IELT), erectile dysfunction indexes, quality of life (QOL), sociodemographic characteristics, lifestyle, and medical illness. After 6 months of intervention, all data were compared with the baseline data and between the groups. Results: Within the 10-month study course, 126 patients (70.8%) completed this study. After intervention and comparison of the results between the two groups, IELT, sexual indexes, and QOL improved in the study group, but significant differences were observed only in the IELT and QOL findings. Conclusions: Clomiphene seems to be useful in the pharmacological treatment of PE compared to the placebo. PMID:26543830

  10. Interventional Radiologic Treatment for Idiopathic Portal Hypertension

    SciTech Connect

    Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi; Motohara, Tomofumi; Fukuda, Tetsuya; Yoshikawa, Takeshi

    1999-07-15

    Purpose: To evaluate the usefulness of interventional radiological treatment for idiopathic portal hypertension. Methods: Between 1995 and 1998, we performed an interventional radiological treatment in five patients with idiopathic portal hypertension, four of whom had refused surgery and one of whom had undergone surgery. Three patients with gastroesophageal varices (GEV) were treated by partial splenic embolization (PSE), one patient with esophageal varices (EV) and massive ascites by transjugular intrahepatic portosytemic shunt (TIPS) and PSE, and one patient with GEV by percutaneous transhepatic obliteration (PTO). Midterm results were analyzed in terms of the effect on esophageal and/or gastric varices. Results: In one woman with severe GEV who underwent three sessions of PSE, there was endoscopic confirmation that the GEV had disappeared. In one man his EV shrunk markedly after two sessions of PSE. In two patients slight reduction of the EV was obtained with one application of PSE combined with endoscopic variceal ligation therapy. PTO for GV in one patient resulted in good control of the varices. All patients have survived for 16-42 months since the first interventional treatment, and varices are well controlled. Conclusion: Interventional radiological treatment is effective for patients with idiopathic portal hypertension, whether or not they have undergone surgery.

  11. Pulmonary function in children with idiopathic scoliosis

    PubMed Central

    2012-01-01

    Idiopathic scoliosis, a common disorder of lateral displacement and rotation of vertebral bodies during periods of rapid somatic growth, has many effects on respiratory function. Scoliosis results in a restrictive lung disease with a multifactorial decrease in lung volumes, displaces the intrathoracic organs, impedes on the movement of ribs and affects the mechanics of the respiratory muscles. Scoliosis decreases the chest wall as well as the lung compliance and results in increased work of breathing at rest, during exercise and sleep. Pulmonary hypertension and respiratory failure may develop in severe disease. In this review the epidemiological and anatomical aspects of idiopathic scoliosis are noted, the pathophysiology and effects of idiopathic scoliosis on respiratory function are described, the pulmonary function testing including lung volumes, respiratory flow rates and airway resistance, chest wall movements, regional ventilation and perfusion, blood gases, response to exercise and sleep studies are presented. Preoperative pulmonary function testing required, as well as the effects of various surgical approaches on respiratory function are also discussed. PMID:22445133

  12. Energetics and structural stability of Cs3C60

    SciTech Connect

    Saito, Susumu; Umemoto, Koichiro; Louie, Steven G.; Cohen, MarvinL.

    2003-12-15

    Using the ab initio pseudo potential total-energy method and the density-functional theory, we study the energetics of face-centered-cubic Cs3C60 which is a material of great interest as a possible high transition-temperature superconductor. At the optimized lattice constant the volume per C60 is found to be smaller than the most stable hexagon-coordination A15 phase, while the total energy of the fcc phase is about 0.9 eV higher than the A15 phase. These results indicate that a low-temperature and high-pressure synthesis method might be a possible way to produce the fcc Cs3C60 phase. In addition, it is also found that the A15 Cs3C60 should show a phase transformation from a hexagon-coordination phase to a pentagon-coordination phase under hydrostatic pressure.

  13. Graphene/3C-SiC Hybrid Nanolaminate.

    PubMed

    Zhuang, Hao; Yang, Bing; Heuser, Steffen; Huang, Nan; Fu, Haiyuan; Jiang, Xin

    2015-12-30

    In this work, we demonstrate a one-step approach to create graphene/3C-SiC nanolaminate structure using microwave plasma chemical vapor deposition technique. Layer-by-layer arrangement of thin 3C-SiC layers and graphene sheets is obtained with the thicknesses of the individual 3C-SiC layers and graphene sheets being 5-10 nm and 2-5 nm, respectively. An intimate contact between 3C-SiC and the graphene sheets is achieved and the nanolaminate film shows a high room temperature conductivity of 96.1 S/cm. A dedicated structural analysis of the nanolaminates by means of high-resolution transmission electron microscopy (HRTEM) reveals that the growth of the nanolaminates follows an iterative process: preferential graphene nucleation around the planar defects at the central region of the SiC layer, leading to the "splitting" of the SiC layer; and the thickening of the SiC layer after being "split". A growth mechanism based on both kinetics and thermodynamics is proposed. Following the proposed mechanism, it is possible to control the layer thickness of the graphene/3C-SiC hybrid nanolaminate by manipulating the carbon concentration in the gas phase, which is further experimentally verified. The high electrical conductivity, large surface area porous structure, feasible integration on different substrates (metal, Mo; semiconductor, Si and 2H-SiC; insulator, diamond) of the graphene/3C-SiC hybrid nanolaminate as well as other unprecedented advantages of the nanolaminate structure make it very promising for applications in mechanical, energy, and sensor-related areas. PMID:26650041

  14. Antibacterial Activity of Ti3C2Tx MXene.

    PubMed

    Rasool, Kashif; Helal, Mohamed; Ali, Adnan; Ren, Chang E; Gogotsi, Yury; Mahmoud, Khaled A

    2016-03-22

    MXenes are a family of atomically thin, two-dimensional (2D) transition metal carbides and carbonitrides with many attractive properties. Two-dimensional Ti3C2Tx (MXene) has been recently explored for applications in water desalination/purification membranes. A major success indicator for any water treatment membrane is the resistance to biofouling. To validate this and to understand better the health and environmental impacts of the new 2D carbides, we investigated the antibacterial properties of single- and few-layer Ti3C2Tx MXene flakes in colloidal solution. The antibacterial properties of Ti3C2Tx were tested against Escherichia coli (E. coli) and Bacillus subtilis (B. subtilis) by using bacterial growth curves based on optical densities (OD) and colonies growth on agar nutritive plates. Ti3C2Tx shows a higher antibacterial efficiency toward both Gram-negative E. coli and Gram-positive B. subtilis compared with graphene oxide (GO), which has been widely reported as an antibacterial agent. Concentration dependent antibacterial activity was observed and more than 98% bacterial cell viability loss was found at 200 μg/mL Ti3C2Tx for both bacterial cells within 4 h of exposure, as confirmed by colony forming unit (CFU) and regrowth curve. Antibacterial mechanism investigation by scanning electron microscopy (SEM) and transmission electron microscopy (TEM) coupled with lactate dehydrogenase (LDH) release assay indicated the damage to the cell membrane, which resulted in release of cytoplasmic materials from the bacterial cells. Reactive oxygen species (ROS) dependent and independent stress induction by Ti3C2Tx was investigated in two separate abiotic assays. MXenes are expected to be resistant to biofouling and offer bactericidal properties. PMID:26909865

  15. Biglycan is a novel binding partner of fibroblast growth factor receptor 3c (FGFR3c) in the human testis.

    PubMed

    Winge, S B; Nielsen, J; Jørgensen, A; Owczarek, S; Ewen, K A; Nielsen, J E; Juul, A; Berezin, V; Rajpert-De Meyts, E

    2015-01-01

    Regulation of spermatogonial maintenance in the human testis is currently not well understood. One pathway suggested to be involved is activated by fibroblast growth factor receptor 3 (FGFR3), which is expressed in a subset of spermatogonia. FGFR3-activating mutations have been identified in spermatocytic seminoma, thought to originate from clonal expansion of spermatogonia. In this study we aimed to characterize potential binding partners of FGFR3, and specifically its mesenchymal "c" splice isoform, in human spermatogonia. Based on expression patterns and homology to the binding site, we identified FGF1, FGF2, and FGF9 as the best candidates for natural ligands of FGFR3c in the testis. In addition, we screened non-FGF proteins and found that a proteoglycan biglycan (BGN) contains a sequence homologous to the FGFR3c binding site on FGF1, and is expressed in peritubular cells adjacent to FGFR3-expressing spermatogonia. Experiments in a cell-free system confirmed that BGN binds to FGFR3c and FGF1. In conclusion, our findings further clarify the complex regulation of FGFR3c in the human testis. We postulate that BGN is a factor secreted by peritubular cells to modulate FGFR3c signaling and thus contributes to the regulation of spermatogonial maintenance. PMID:25260943

  16. Revisiting correlations between broad-line and jet emission variations for AGNs: 3C 120 and 3C 273

    NASA Astrophysics Data System (ADS)

    Liu, H. T.; Bai, J. M.; Feng, H. C.; Li, S. K.

    2015-06-01

    We restudy the issue of cross-correlations between broad-line and jet emission variations, and aim to locate the position of a radio (and gamma-ray) emitting region in a jet of active galactic nuclei. Considering the radial profiles of the radius and number density of clouds in a spherical broad-line region (BLR), we derive new formulae connecting the jet-emitting position Rjet to the time lag τob between broad-line and jet emission variations, and the BLR radius. Also, formulae are derived for a disc-like BLR and a spherical shell BLR. The model-independent flux randomization/random subset selection method is used to estimate τob. For 3C 120, positive lags of about 0.3 yr are found between the 15 GHz emission and the Hβ, Hγ and He II λ4686 lines, including broad-line data in a newly published paper, indicating that the line variations lead the 15 GHz ones. Each of the broad-line light curves corresponds to a radio outburst. Rjet = 1.1-1.5 parsec (pc) is obtained for 3C 120. For 3C 273, a common feature of negative time lags is found in the cross-correlation functions between light curves of radio emission and the Balmer lines, as well as Lyα λ1216 and C IV λ1549 lines. Rjet = 1.0-2.6 pc is obtained for 3C 273. The estimated Rjet is comparable for 3C 120 and 3C 273, and the gamma-ray-emitting positions will be within ˜1-3 pc from the central engines. Comparisons show that the cloud number density and radius radial distributions and the BLR structures have only negligible effects on Rjet.

  17. Microwave Radiometer – 3 Channel (MWR3C) Handbook

    SciTech Connect

    Cadeddu, MP

    2012-05-04

    The microwave radiometer 3-channel (MWR3C) provides time-series measurements of brightness temperatures from three channels centered at 23.834, 30, and 89 GHz. These three channels are sensitive to the presence of liquid water and precipitable water vapor.

  18. The 3C support: A survivable alternative to wood cribbing

    SciTech Connect

    Frederick, J.

    1995-10-01

    Wood cribbing has historically been a somewhat dependable and low cost method of providing mine roof support. In high stress conditions, such as longwall tailgates, the wood crib does not always survive. Failure of tailgate cribs can block travelways, restrict ventilation and force costly time-consuming rehabilitation. At least in the Western United States, wood cribbing is no longer the answer to many roof support problems. Western mines are being forced to find alternatives to wood cribbing. This is due to the escalating cost, questionable availability and dubious quality of available wood supplies. The Corrugated Confined Core mine roof Support (3C Support) was developed to survive the extreme ground control conditions of a longwall tailgate. The 3C Support testing has shown ultimate strengths exceeding 2,000,000 lbs and a yield range over 48-inches. Standard wood cribs, constructed from Western United States softwood, were also tested. The wood cribs had ultimate strengths up to 237,000 lbs and a yield range up to 27-inches. Underground testing of the 3C Support in longwall tailgates at Southern Utah Fuel Company (SUFCO) was also conducted. This testing and installation of over 5000 3C Supports have demonstrated the following advantages: (1) lower installed cost; (2) 55 percent reduction in cribbing manpower requirements; (3) improved yield and ultimate strength characteristics; (4) much improved tailgate roof support survivability; (5) virtually eliminates blocked tailgates; (6) improved safety; (7) reduced flammable material; (8) improved ventilation; and (9) environmentally friendly.

  19. Low Frequency Radio Observations of 3C 129

    NASA Astrophysics Data System (ADS)

    Lane, W. M.; Harris, D. E.; Ensslin, T. A.; Kassim, N. E.; Perley, R. A.

    2001-12-01

    We present a wide-field map of the radio galaxy 3C 129 and its companion galaxy 3C 129.1 at λ = 90 cm. Both galaxies are part of an X-ray identified cluster at z=0.021, which has been excluded from most optical studies because it lies in the galactic plane. 3C 129 is a narrow-angle-tail (NAT) source with a plume-like double-tail extending nearly 30' at a wavelength of 90cm. We see a distinct steep-spectrum feature near its head, extending in a direction perpendicular to the radio tails. We propose is that this `crosspiece' might consist of fossil radio plasma, which has been re-energized by the compression of the bow shock wave of the supersonically moving galaxy 3C 129. One possible origin of the fossil radio plasma could be the tail of a nearby head-tail radio galaxy, and we discuss the implications of this scenario. WML is a National Research Council Postdoctoral Fellow. Basic research in astronomy at the Naval Research Laboratory is funded by the Office of Naval Research. DEH acknowledges support from NASA grant GO1-2135A.

  20. The linear polarization of 3C 345 in the ultraviolet

    NASA Technical Reports Server (NTRS)

    Dolan, Joseph F.; Boyd, Patricia T.; Wolinski, Karen G.; Smith, Paul S.; Impey, C. D.; Bless, Robert C.; Nelson, M. J.; Percival, J. W.; Taylor, M. J.; Elliot, J. L.

    1994-01-01

    The linear polarization of 3C 345, a superluminal radio source and OVV quasar, was observed in two bandpasses in the ultraviolet (centered at 2160 A and 2770 A) in 1993 April using the High Speed Photometer on the Hubble Space Telescope. The quasar is significantly polarized in the UV (p greater than 5%). Ground-based polarimetry was obtained 11 days later, but a difference in the position angle between the observations in the visible and those in the UV indicate that the magnitude of the polarization of 3C 345 may have changed over that time. If the two observation sets represent the same state of spectral polarization, then the large UV flux implies that either the polarization of the synchrotron continuum must stop decreasing in the UV, or that there is an additional source of polarized flux in the ultraviolet. Only if the UV observations represent a spectral polarization state with the same position angle in the visible seen previously in 3C 345 can the polarized flux be represented by a single power law consistent with the three-component model of Smith et al. This model consists of a polarized synchrotron component, an unpolarized component from the broad-line region, and an unpolarized component attributed to thermal radiation from an optically thick accretion disk. Additional simultaneous polarimetry in the UV and visible will be required to further constrain models of the continuum emission processes in 3C 345 and determine if the UV polarized flux is synchrotron in origin.

  1. [Physiological adolescence, pathological adolescence].

    PubMed

    Olié, Jean-Pierre; Gourion, David; Canceil, Olivier; Lôo, Henri

    2006-11-01

    The uncertainties of looming adulthood, nostalgia for childhood, and a general malaise explain the crisis of adolescence. Rebellion, conflict, occasional failure at school or in society, and at-risk behaviors are not always signs of future psychiatric illness. In contrast, the physician must be in a position to identify tell-tale signs such as dysmorphophobia, existential anxiety, a feeling of emptiness, and school or social breakdown. Most psychiatric disorders that begin in adolescence are only diagnosed several years after onset. Yet early diagnosis is of utmost importance, as treatment becomes less effective and the long-term prognosis worsens with time. Suicide is the second cause of death during adolescence. All signs of suicidal behavior require hospitalization and evaluation in a psychiatric unit. Antidepressants may be necessary in adolescence. The recent controversy concerning a possible increase in the suicidal risk during antidepressant treatment should not mask the fact that the real public health issue is depression, and not antidepressants. Eating disorders are especially frequent among adolescent girls; it is important to identify psychiatric comorbidities such as schizophrenia, depression and obsessive-compulsive disorders, and to assess the vital risk. Illicit drug and alcohol consumption are frequent during adolescence; for example, close to half of all French adolescents have tried cannabis at least once. Once again, it is important to detect psychiatric comorbidities in substance-abusing adolescents. Phobia is an underdiagnosed anxiety disorder among adolescents; it may become chronic if proper treatment is not implemented, leading to suffering and disability. Finally, two major psychiatric disorders--schizophrenia and bipolar disorder--generally begin in adolescence. Treatment efficacy and the long-term prognosis both depend on early diagnosis. Treatment must be tailored to the individual patient. "Borderline" states are over

  2. Headache arising from idiopathic changes in CSF pressure.

    PubMed

    Ducros, Anne; Biousse, Valérie

    2015-06-01

    New onset of sudden or progressive headache can have various causes, including disorders of intracranial pressure (ICP). Headache is the most common-and often the presenting-symptom of both intracranial hypertension and intracranial hypotension syndromes, which can be symptomatic or idiopathic. Despite the widespread availability of diagnostic tests, including ocular ophthalmoscopy, neuroimaging, and measurement of CSF pressure, delays in diagnosis or misdiagnosis of idiopathic intracranial hypertension and spontaneous intracranial hypotension remain common. If left untreated, idiopathic intracranial hypertension and spontaneous intracranial hypotension produce highly disabling headaches, and threaten vision, hearing, and in rare cases, brain function and life. To improve the diagnosis of idiopathic intracranial hypertension and spontaneous intracranial hypotension, changes in the overall diagnostic strategy for headaches will be necessary in most care centres. Improved understanding of CSF physiology and the mechanisms of idiopathic intracranial hypertension and spontaneous intracranial hypotension will guide the development of new treatments. PMID:25987284

  3. Pulmonary function after less invasive anterior instrumentation and fusion for idiopathic thoracic scoliosis

    PubMed Central

    2013-01-01

    Purpose Standard thoracotomy for anterior instrumentation and fusion of the thoracic spine in idiopathic scoliosis may have detrimental effects on pulmonary function. In this study we describe a less invasive anterior surgical technique and show the pre- and postoperative pulmonary function with a minimum follow-up of 2 years. Methods Twenty patients with Lenke type 1 adolescent thoracic idiopathic scoliosis were treated with anterior spinal fusion and instrumentation. The mean preoperative Cobb angle of the thoracic curve was 53° ± 5.8. Pulmonary function tests (PFT) and radiographic evaluation was performed. Results The mean postoperative correction in Cobb angle of the thoracic curve was 27° ± 8.2 (49%). The mean preoperative FEV1 was 2.81 ± 0.43 L, which increased to 3.14 ± 0.50 L at 2 years postoperatively (P = 0.000). The mean FEV1% did not change (89.60 ± 7.49% preoperatively, versus 90.53 ± 5.95% at 2 years follow-up, P = 0.467). The TLC increased from 4.62 ± 0.62 L preoperatively to 5.17 ± 0.63 L at 2 years follow-up (P = 0.000). The FEV1% at two years of follow-up improved to 104% of the FEV1% predicted value. The FEV1 improved to 97% of the FEV1 predicted value. Conclusion Anterior spinal fusion for idiopathic scoliosis by means of a minimal open thoracotomy proved to be a safe surgical technique that resulted in an improvement of pulmonary function. Our results are similar to those of thoracoscopic procedures reported in literature. PMID:23965278

  4. Effect of body composition on bone mineral density in Moroccan patients with juvenile idiopathic arthritis

    PubMed Central

    El Badri, Dalal; Rostom, Samira; Bouaddi, Ilham; Hassani, Asmae; Chkirate, Bouchra; Amine, Bouchra; Hajjaj-Hassouni, Najia

    2014-01-01

    Introduction The link between bone mass and body composition is widely recognized, but only few works were selectively performed on subjects with juvenile idiopathic arthritis. The aim of our study was to investigate the effect of body composition on bone mineral density (BMD) in Moroccan patients with juvenile idiopathic arthritis. Methods Thirty three children with juvenile idiopathic arthritis (JIA) were included in a cross-sectional study. The diagnosis of JIA was made according to the criteria of the International League of Association of Rheumatology (ILAR). Body mass index (BMI) was calculated from the ratio of weight/height2(kg/m2). Pubertal status was determined according to the Tanner criteria. Bone status, body composition and bone mineral content (BMC) were analyzed by using dual-energy X-ray absorptiometry (DXA). BMD was assessed at the lumbar spine (L1-L4) and at total body in (g/cm2). Total body fat tissue mass (FTM) and lean tissue mass (LTM) were also analyzed by DXA and expressed in kilograms. In children, low BMD was defined as a Z-score less than -2 and osteoporosis was defined as a Z-score less than -2 with a fracture history. Results A cross-sectional study was conducted in 33 Moroccan patients with JIA aged between 4 and 16 years, Fat mass was not related to bone density; in contrast, BMD was positively associated to LTM in total body(r = =0.41, p= 0.04) but not in lumbar spine (r = 0.29, p= 0.17). There exist significant correlation between BMC and BMD in total body (r = 0.51, p = 0.01). Conclusion This study suggests that the LTM is a determining factor of the BMD during adolescence. Other studies with a broader sample would be useful to confirm this relation. PMID:25120859

  5. Idiopathic Bilateral External Jugular Vein Thrombosis

    PubMed Central

    Hindi, Zakaria; Fadel, Ehab

    2015-01-01

    Patient: Male, 21 Final Diagnosis: Idiopathic bilateral external jugular vein thrombosis Symptoms: Face engorgement • neck swelling Medication: — Clinical Procedure: None Specialty: Hematology Objective: Unknown ethiology Background: Vein thrombosis is mainly determined by 3 factors, which constitute a triad called Virchow’s triad: hypercoagulability, stasis, and endothelial injury. Venous thrombosis commonly occurs in the lower extremities since most of the blood resides there and flows against gravity. The veins of the lower extremities are dependent on intact valves and fully functional leg muscles. However, in case of valvular incompetency or muscular weakness, thrombosis and blood stasis will occur as a result. In contrast, the veins of the neck, specially the jugulars, have distensible walls which allow flexibility during respiration. In addition, the blood directly flows downward towards the heart. Nevertheless, many case reports mentioned the thrombosis of internal jugular veins and external jugular veins with identified risk factors. Jugular vein thrombosis has previously been associated in the literature with a variety of medical conditions, including malignancy. Case Report: This report is of a case of idiopathic bilateral external jugular vein thrombosis in a 21 year-old male construction worker of Southeast Asian origin with no previous medical history who presented with bilateral facial puffiness of gradual onset over 1 month. Doppler ultrasound and computed tomography were used in the diagnosis. Further work-up showed no evidence of infection or neoplasia. The patient was eventually discharged on warfarin. The patient was assessed after 6 months and his symptoms had resolved completely. Conclusions: Bilateral idiopathic external jugular veins thrombosis is extremely rare and can be an indicator of early malignancy or hidden infection. While previous reports in the literature have associated jugular vein thrombosis with malignancy, the present

  6. Idiopathic Polyhydramnios: Severity and Perinatal Morbidity.

    PubMed

    Wiegand, Samantha L; Beamon, Carmen J; Chescheir, Nancy C; Stamilio, David

    2016-06-01

    Objective To estimate the association between the severity of idiopathic polyhydramnios and adverse outcomes. Study Design Retrospective cohort study of deliveries at one hospital from 2000 to 2012 with an amniotic fluid index (AFI) measurement ≥24 + 0 weeks' gestation. Pregnancies complicated by diabetes, multiples, or fetal anomalies were excluded. Exposure was the degree of polyhydramnios: normal (AFI 5-24 cm), mild (≥ 24-30 cm), and moderate-severe (> 30 cm). Primary outcomes were perinatal mortality, neonatal intensive care unit (NICU) admission, and postpartum hemorrhage. Results There were 10,536 pregnancies: 10,188 with a normal AFI, 274 mild (78.74%), and 74 moderate-severe polyhydramnios (21.26%). Adverse outcomes were increased with idiopathic polyhydramnios: NICU admission (adjusted odds ratio [AOR] 3.71, 95% confidence interval [CI] 2.77-4.99), postpartum hemorrhage (AOR 15.81, 95% CI 7.82-31.96), macrosomia (AOR 3.41, 95% CI 2.61-4.47), low 5-minute Apgar score (AOR 2.60, 95% CI 1.57-4.30), and cesarean (AOR 2.16, 95% CI 1.74-2.69). There were increasing odds of macrosomia (mild: AOR 3.19, 95% CI 2.36-4.32; moderate-severe: AOR 4.44, 95% CI 2.53-7.79) and low 5-minute Apgar score (mild: AOR 2.24, 95% CI 1.23-4.08; moderate-severe: AOR 3.93, 95% CI 1.62-9.55) with increasing severity of polyhydramnios. Conclusion Idiopathic polyhydramnios is independently associated with increased risks of morbidity. There appears to be a dose-response relationship for neonatal macrosomia and low 5-minute Apgar score risks. PMID:26862725

  7. Idiopathic and mild subclinical hypothyroidism in childhood: clinical management.

    PubMed

    De Luca, F; Corica, D; Pitrolo, E; Santucci, S; Romeo, M

    2014-02-01

    Subclinical hypothyroidism (SH) is a common clinical problem in children and adolescents, for which there are many controversial issues regarding natural course and management. Aim of this review is to report on the state of the art regarding these controversial points of idiopathic SH in pediatric age. We included in our search only full-length articles on natural history and treatment of SH in children with no associated diseases or genetic syndromes, thus identifying only 13 papers that were suitable for our analysis. According to the results of these retrospective or longitudinal studies a persistently mild TSH elevation (between 5 and 10 mIU/L) in SH children with no underlying disease is unable to affect growth and bone maturation or body mass index (two studies) or cognitive function (one study). Moreover, the risk of a deterioration over time of thyroid function in the untreated cases with mild and idiophatic SH is only 12% (one study). Progression odds increase with increasing baseline TSH, with goiter presence and with positive anti-thyroid antibodies (two studies in children). Although no broad consensus exists on whether children with mild SH require L-T4 therapy, nevertheless, on the basis of the results of the majority of included studies, it can be concluded that L-T4 treatment is not indicated when TSH is repeatedly between 5 and 10 mIU/L and an underlying disease has been excluded. By contrast, treatment is suitable in the cases with TSH>10 mIU/L and/or an underlying disease. PMID:24608582

  8. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    PubMed Central

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF.

  9. Idiopathic lipoid pneumonia successfully treated with prednisolone.

    PubMed

    Lococo, Filippo; Cesario, Alfredo; Porziella, Venanzio; Mulè, Antonino; Petrone, Gianluigi; Margaritora, Stefano; Granone, Pierluigi

    2012-01-01

    Lipoid pneumonia (LP) is a rare type of pneumonia that is radiologically characterized by lung infiltrates, although imaging alone may not be diagnostic. We describe an unusual 61-year-old patient with idiopathic LP presenting as a solitary pulmonary nodule mimicking lung cancer because of its rapid growth. After treatment with oral prednisone, a control chest radiogram indicated complete normalization of the radiologic features. This case shows that LP should be considered in the diagnostic assessment of any undefined pulmonary mass, after malignancy has been pathologically excluded. PMID:21419490

  10. Renal involvement in idiopathic hypereosinophic syndrome

    PubMed Central

    Shehwaro, Nathalie; Langlois, Anne Lyse; Gueutin, Victor; Izzedine, Hassane

    2013-01-01

    The hypereosinophilic syndromes (HESs) are a group of disorders marked by the sustained overproduction of eosinophils, in which eosinophilic infiltration and mediator release cause damage to multiple organs. In idiopathic HES, the underlying cause of hypereosinophilia (HE) remains unknown despite thorough aetiological work-up. Kidney disease is thought to be rare in HES. Renal manifestations described include eosinophilic interstitial nephritis, various types of glomerulopathies, thrombotic microangiopathy (TMA) and electrolyte disturbances. The diagnosis must be made in time, because a recovery of renal function can be obtained if treatment is initiated promptly. PMID:26064485

  11. "Nocturnal seizures" in idiopathic pulmonary arterial hypertension.

    PubMed

    Izzo, Anthony; McSweeney, Julia; Kulik, Thomas; Khatwa, Umakanth; Kothare, Sanjeev V

    2013-10-15

    The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made. Sleep physicians should consider IPAH in the differential diagnosis of nocturnal events in children. PMID:24127156

  12. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    PubMed Central

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting. PMID:26819784

  13. Dystonia Associated with Idiopathic Slow Orthostatic Tremor

    PubMed Central

    Kobylecki, Christopher; Silverdale, Monty A.; Dick, Jeremy P. R.; Kellett, Mark W.; Marshall, Andrew G.

    2015-01-01

    Background We aimed to characterize the clinical and electrophysiological features of patients with slow orthostatic tremor. Case Report The clinical and neurophysiological data of patients referred for lower limb tremor on standing were reviewed. Patients with symptomatic or primary orthostatic tremor were excluded. Eight patients were identified with idiopathic slow 4–8 Hz orthostatic tremor, which was associated with tremor and dystonia in cervical and upper limb musculature. Coherence analysis in two patients showed findings different to those seen in primary orthostatic tremor. Discussion Slow orthostatic tremor may be associated with dystonia and dystonic tremor. PMID:26877891

  14. Idiopathic pulmonary hemosiderosis complicated by Down syndrome.

    PubMed

    Watanabe, Hirofumi; Ayusawa, Mamoru; Kato, Masataka; Chou, Ami; Komori, Akiko; Abe, Yuriko; Matsumura, Masaharu; Kamiyama, Hiroshi; Izumi, Hiroyuki; Takahashi, Shori

    2015-10-01

    We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH. PMID:26508184

  15. Systemic Juvenile Idiopathic Arthritis: Diagnosis and Management.

    PubMed

    Kumar, Sathish

    2016-04-01

    Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. In sJIA, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of sJIA and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. Recent data suggests that early cytokine blockage might abrogate chronic, destructive, therapy resistant arthritis phase, reflecting a potential "window of opportunity" in the care of children with sJIA. PMID:26916892

  16. Body experiences, emotional competence, and psychosocial functioning in juvenile idiopathic arthritis.

    PubMed

    Bomba, Monica; Meini, Antonella; Molinaro, Anna; Cattalini, Marco; Oggiano, Silvia; Fazzi, Elisa; Neri, Francesca; Plebani, Alessandro; Nacinovich, Renata

    2013-08-01

    We investigated self-image, psychological functioning, and quality of life in children and adolescents with juvenile idiopathic arthritis (JIA). Thirty-nine children with JIA were compared with 80 healthy peers. We first administered the Human Figure Drawing Test (HFDT) to all subjects; children also completed standardized questionnaires evaluating health-related quality of life (PEDSQL 4.0 Generic Core Scales) and the main aspects of psychological functioning: anxiety (SAFA-A) and depression (CDI). Parents were asked to complete the Child Behaviour Checklist (CBCL) and the PEDSQL 4.0. For each patient with JIA, clinical notes were gathered and a global disease assessment (visual analog scale--VAS) was performed. Compared to healthy peers, patients with JIA reported reduced maturity quotients at HFDT, more depressive traits, greater anxiety, and lower health-related quality of life. Among the subjects with JIA, HFDT revealed that adolescents had a greater impairment in all areas investigated. Furthermore, there was a significant correlation between the physical well-being rated by VAS and the perception of poorer quality of life in patients, mostly in the psychosocial domains. Children and adolescents with JIA exhibit emotional difficulties and a delay of psychological development leading to low self-esteem, a distorted self-image, more anxiety and depression traits, and a worse quality of life, when compared to healthy subjects. PMID:23392772

  17. Altered erythrocyte Na/sup +/ + K/sup +/ pump in adolescent obesity

    SciTech Connect

    DeLuise, M.; Rappaport, E.; Flier, J.S.

    1982-11-01

    The number of Na/K pump units and the cation transport activity of the pump were measured in erythrocytes from two etiologically different groups of obese adolescents and a group of normal controls. There was a significant reduction in the number of pump units, as measured by saturation ouabain binding, in erythrocytes from adolescents with idiopathic, early onset obesity. Individuals whose obesity developed subsequent to the appearance of a variety of hypothalamic lesions showed no reduction in the red cell complement of Na/K pump when compared to controls and the cation transport activity of their cells was higher than both the controls and the subjects with idiopathic obesity. These results support data obtained in adults that reduced red cell Na/K pump levels are seen in a group of individuals with idiopathic obesity. They further suggest that such reductions are not likely to be secondary to the obese state per se.

  18. The Trails of Superluminal Jet Components in 3C 111

    NASA Technical Reports Server (NTRS)

    Kadler, M.; Ros, E.; Perucho, M.; Kovalev, Y. Y.; Homan, D. C.; Agudo, I.; Kellermann, K. I.; Aller, M. F.; Aller, H. D.; Lister, M. L.; Zensus, J. A.

    2007-01-01

    The parsec-scale radio jet of the broad-line radio galaxy 3C 111 has been monitored since 1995 as part of the 2cm Survey and MOJAVE monitoring observations conducted with the VLBA. Here, we present results from 18 epochs of VLBA observations of 3C 111 and from 18 years of radio flux density monitoring observations conducted at the University of Michigan. A major radio flux-density outburst of 3C 111 occurred in 1996 and was followed by a particularly bright plasma ejection associated with a superluminal jet component. This major event allows us to study a variety of processes associated with outbursts of radio-loud AGN in much greater detail than possible in other cases: the primary perturbation gives rise to the formation of a forward and a backward-shock, which both evolve in characteristically different ways and allow us to draw conclusions about the workflow of jet-production events; the expansion, acceleration and recollimation of the ejected jet plasma in an environment with steep pressure and density gradients are revealed; trailing components are formed in the wake of the primary perturbation as a result of Kelvin- Helmholtz instabilities from the interaction of the jet with the external medium. The jet-medium interaction is further scrutinized by the linear-polarization signature of jet components traveling along the jet and passing a region of steep pressure/density gradients.

  19. Optimal High-TC Superconductivity in Cs3C60

    NASA Astrophysics Data System (ADS)

    Harshman, Dale; Fiory, Anthony

    The highest superconducting transition temperatures in the (A1-xBx)3C60 superconducting family are seen in the A15 and FCC structural phases of Cs3C60 (optimized under hydrostatic pressure), exhibiting measured values for near-stoichiometric samples of TC0 meas . = 37.8 K and 35.7 K, respectively. It is argued these two Cs-intercalated C60 compounds represent the optimal materials of their respective structures, with superconductivity originating from Coulombic e- h interactions between the C60 molecules, which host the n-type superconductivity, and mediating holes associated with the Cs cations. A variation of the interlayer Coulombic pairing model [Harshman and Fiory, J. Supercond. Nov. Magn. 28 ̲, 2967 (2015), and references therein] is introduced in which TC0 calc . ~ 1 / lζ , where l relates to the mean spacing between interacting charges on surfaces of the C60 molecules, and ζ is the average radial distance between the surface of the C60 molecules and the neighboring Cs cations. For stoichiometric Cs3C60, TC0 calc . = 38.08 K and 35.67 K for the A15 and FCC macrostructures, respectively; the dichotomy is attributable to differences in ζ.

  20. Quality of life in patients submitted to surgical treatment of idiopathic scoliosis

    PubMed Central

    Rodrigues, João Bernardo Sancio Rocha; Saleme, Nathália Ambrozim Santos; Batista, José Lucas; Cardoso, Igor Machado; Jacob, Charbel

    2015-01-01

    ABSTRACT OBJECTIVE : To evaluate quality of life, using the SF-36, in patients with adolescent idiopathic scoliosis (AIS) who un-derwent surgery for deformity correction, comparing the results in the pre-and post-operative period. METHODS : We evaluated 29 patients, 24 female, mean age 14.5 years, all patients had measurement of Cobb angle greater than 50º, and responded to the SF-36 questionnaire preope-ratively and on average two years after surgery. RESULTS : There was improvement in all eight domains studied by the SF-36 after surgical treatment, with statistically significant improvement of the domains functional capacity physical aspects, pain and general state. Vitality and mental heal-th were those with the lowest percentage of improvement postoperatively. CONCLUSION : Surgical treatment of defor-mity in all AIS improved the functional aspects assessed by the SF-36, representing, in practice, better quality of life for these patients. Evidence Level II, Prospective Study. PMID:27057138

  1. Systemic-onset juvenile idiopathic arthritis.

    PubMed

    Cimaz, Rolando

    2016-09-01

    Systemic-onset juvenile idiopathic arthritis (SoJIA) is a systemic inflammatory disease which has up to now been classified as a category of juvenile idiopathic arthritis. However, in this context, systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that it may rather be part of the spectrum of autoinflammatory disorders. The disease is in fact unique with regard to the other JIA categories, in terms of clinical manifestations, prognosis, and response to conventional immunosuppressant therapies. It is characterized clinically by fever, lymphadenopathy, arthritis, rash, and serositis. IL-1 and IL-6 play a major role in the pathogenesis of SoJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SoJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation continue to be a major issue in patients' care. Recent advances on the pathogenesis and treatment have revolutionized the care and prognosis of this potentially life-threatening pediatric condition. PMID:27392503

  2. Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

    PubMed Central

    Lee, Hwajeong; Rehman, Aseeb Ur; Fiel, M. Isabel

    2016-01-01

    Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity. PMID:26563701

  3. Idiopathic incus necrosis: Analysis of 4 cases.

    PubMed

    Kansu, Leyla; Yilmaz, Ismail; Akdogan, Volkan; Avci, Suat; Ozluoglu, Levent

    2013-02-01

    We evaluated ossicular chain reconstruction in patients with idiopathic incus necrosis who have conductive hearing loss and an intact ear drum. The study included four patients (3 women and 1 man; the ages of the patients were 22, 31, 35, and 56 years, respectively) with unilateral conductive hearing loss, no history of chronic serous otitis media, an intact ear drum, normal middle ear mucosa, and necrosis of the long processes of the incus. On preoperative pure tone audiometry, air-bone gaps were 24, 25, 38, and 33 dB. Bilateral tympanometry and temporal bone computed tomography results were normal. All 4 patients underwent an exploratory tympanotomy. During the operation, the mucosa of the middle ear was normal, with a mobile stapes foot plate and malleus. No evidence of any granulation tissue was found; however, necrosis of the incus long processes was seen. For ossicular reconstruction, we used tragal cartilage between the incus and the stapes in 1 patient; in the other 3 patients, glass ionomer bone cement was used (an interposition cartilage graft also was used in the patients who received the glass ionomer bone cement). In all patients, air-bone gaps under 20 dB were established in the first year after surgery. In the ossicular disorders within the middle ear, the incus is the most commonly affected ossicle. While, the most common cause of these disorders is chronic otitis media, it may be idiopathic rarely. Several ossicular reconstruction techniques have been used to repair incudostapedial discontinuity. PMID:23460219

  4. Possible development of idiopathic sclerosing encapsulating peritonitis.

    PubMed

    Yanagi, H; Kusunoki, M; Yamamura, T

    1999-01-01

    We report a rare case of idiopathic sclerosing encapsulating peritonitis (SEP). During a laparotomy before undergoing a distal gastrectomy with Billroth II reconstruction for early gastric cancer, the patient was found to have a membranous encapsulation wrapping each small bowel loop, unlike peritoneal encapsulation or typical SEP. He had complained of persistent heartburn, distension and diarrhea for 2 months in the post-operative course. The second laparotomy, which was performed to improve prolonged transit, revealed typical SEP with a thick and fibrotic membrane that encased the small bowel entirely. Stripping of the sclerosing encasing membrane, separation of the adherent loops of the proximal small bowel, and Braun's anastomosis were performed. The patient complained of epigastric fullness and diarrhea after he was relieved from the complete bowel obstruction for 45 days post-operatively. Trimebutine maleate was administrated 5 months after the second operation and this markedly improved his symptoms. This case might reflect the developmental process of idiopathic SEP. In addition, the use of a motility regulator may improve symptoms related to the abnormal intestinal motility by this disease. PMID:10228820

  5. Medical Therapy in Idiopathic Pulmonary Fibrosis.

    PubMed

    Antoniou, Katerina M; Wuyts, Wim; Wijsenbeek, Marlies; Wells, Athol U

    2016-06-01

    Medical therapy for idiopathic fibrosis remains controversial. Idiopathic pulmonary fibrosis (IPF) was uniformly a disease that progressed inexorably, typically leading to death within 3 to 5 years from onset of symptoms. Until recently, lung transplantation was the only effective transplant option. Within the past decade, several placebo-controlled trials failed to show benefit in patients with IPF. However, within the past 2 years, two novel antifibrotic agents (pirfenidone and nintedanib) were approved by the Food and Drug Administration (FDA) in the United States and European Medicines Agency (EMA) based upon pivotal studies that showed benefit (specifically slowing of the rate of disease progression) with both agents. Short-term outcomes (12 months) showed less deterioration of physiological parameters (e.g., change in forced vital capacity), although survival benefit has not convincingly been established with either agent. Nonetheless, these agents bring a glimmer of hope to patients with this deadly disease. The appropriate indications for initiating therapy, best candidates for therapy, and possible role for combination therapy remain controversial. Additional studies using agents that attenuate or abrogate profibrotic cytokines and chemokines may provide even further improvement in the future. PMID:27231861

  6. Idiopathic osteoarthritis and contracture: causal implications

    PubMed Central

    Jones, P; Alexander, C; Stewart, J; Lynskey, N

    2005-01-01

    Objective: To use the known association of idiopathic osteoarthritis with contracture as a means of searching for its cause. There are currently two theories concerning this association, one assuming that the contracture is a consequence of the osteoarthritis and the other that it precedes and causes the osteoarthritis. This study tested both theories. Methods: Flexion ranges in the 12 finger joints were obtained by goniometric measurement in two samples of normal female subjects, one group with a mean age of 22 years (25 subjects) and one with a mean age of 45 years (50 subjects). The results were compared with the known regional prevalence of osteoarthritis in the finger joints of women. Results: The older group showed evidence of reduced flexion range consistent with development of contracture in the extensor mechanism of the fingers. The distribution of the contracture showed a strong negative correlation with the regional prevalence of osteoarthritis. Conclusions: An early dorsal contracture develops in the fingers of normal subjects, but it is neither a consequence of nor the cause of digital osteoarthritis. The most parsimonious explanation for the association is that both contracture and idiopathic osteoarthritis are independent consequences of failure to use the full movement range. If this hypothesis is correct, the disease could be preventable. PMID:15647431

  7. Idiopathic Fatal Pancytopenia: A Case Report

    PubMed Central

    Tilak, Vijai

    2016-01-01

    Pancytopenia is defined as decrease in red blood cells, white blood cells and platelets. Many disease processes involve the bone marrow primarily or secondarily resulting in pancytopenia. A 55-year-old male presented with generalized body weakness and few episodes of malena for last one year. Physical and systemic examination was unremarkable. CBC report revealed pancytopenia. Other haematological parameters were within normal limit. Stool for occult blood was positive. USG and CECT abdomen showed no abnormality. The patient was evaluated for any evidence of malignancy but no clue was found. Bone marrow examination was done as patient was having pancytopenia. Bone marrow smears, clot sections and bone marrow biopsy was normal. Immunohistochemistry and cytogenetics study was unremarkable. Patient was admitted in hospital for 1 month and his condition rapidly deteriorated. The cause of pancytopenia remained unexplained and therefore it was named as Idiopathic fatal pancytopenia. “Idiopathic Fatal Pancytopenia (IFP)” is an emerging new entity with a grave prognosis. We wish to sensitize the medical community and the scientists to this rapidly fatal condition. PMID:27504300

  8. Acute and subacute idiopathic interstitial pneumonias.

    PubMed

    Taniguchi, Hiroyuki; Kondoh, Yasuhiro

    2016-07-01

    Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia. PMID:27123874

  9. Assimilatory Sulfate Reduction in C3, C3-C4, and C4 Species of Flaveria1

    PubMed Central

    Koprivova, Anna; Melzer, Michael; von Ballmoos, Peter; Mandel, Therese; Brunold, Christian; Kopriva, Stanislav

    2001-01-01

    The activity of the enzymes catalyzing the first two steps of sulfate assimilation, ATP sulfurylase and adenosine 5′-phosphosulfate reductase (APR), are confined to bundle sheath cells in several C4 monocot species. With the aim to analyze the molecular basis of this distribution and to determine whether it was a prerequisite or a consequence of the C4 photosynthetic mechanism, we compared the intercellular distribution of the activity and the mRNA of APR in C3, C3-C4, C4-like, and C4 species of the dicot genus Flaveria. Measurements of APR activity, mRNA level, and protein accumulation in six Flaveria species revealed that APR activity, cysteine, and glutathione levels were significantly higher in C4-like and C4 species than in C3 and C3-C4 species. ATP sulfurylase and APR mRNA were present at comparable levels in both mesophyll and bundle sheath cells of C4 species Flaveria trinervia. Immunogold electron microscopy demonstrated the presence of APR protein in chloroplasts of both cell types. These findings, taken together with results from the literature, show that the localization of assimilatory sulfate reduction in the bundle sheath cells is not ubiquitous among C4 plants and therefore is neither a prerequisite nor a consequence of C4 photosynthesis. PMID:11598228

  10. Idiopathic interstitial pneumonias: progress in classification, diagnosis, pathogenesis and management.

    PubMed Central

    King, Talmadge E.

    2004-01-01

    The idiopathic interstitial pneumonias are a heterogeneous group of poorly understood diseases with often devastating consequences for those afflicted. Subclassification of the idiopathic interstitial pneumonia based on clinical-radiological-pathological criteria has highlighted important pathogenic, therapeutic and prognostic implications. The most critical distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a worse response to therapy and prognosis. New insight into the pathophysiology of usual interstitial pneumonia suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. While the clinical and radiographic diagnosis of idiopathic interstitial pneumonias can be made confidently in some cases, many patients require surgical lung biopsy to determine their underlying histopathology. A structured, clinical-radiological-pathological approach to the diagnosis of the idiopathic interstitial pneumonias, with particular attention to the identification of idiopathic pulmonary fibrosis, insures proper therapy, enhances prognostication, and allows for further investigation of therapies aimed at distinct pathophysiology. Images Fig. 2 Fig. 4 Fig. 5 Fig. 6 PMID:17060957

  11. Comparative Characterization of Phosphoenolpyruvate Carboxylase in C3, C4, and C3-C4 Intermediate Panicum Species 1

    PubMed Central

    Holaday, A. Scott; Black, Clanton C.

    1981-01-01

    Various properties of phosphoenolpyruvate carboxylases were compared in leaf preparations from C3-C4 intermediate, C3, and C4Panicum species. Values of Vmax in micromoles per milligram chlorophyll per hour at pH 8.3 were 57 to 75 for the enzyme from Panicum milioides, Panicum schenckii, and Panicum decipiens (all C3-C4). The values for Panicum laxum (C3) and Panicum prionitis (C4) were 20 to 40 and 952 to 1374, respectively. The Vmax values did not change at pH 7.3 except for the C4 value, which increased about 24%. At pH 8.3, the phosphoenolpyruvate carboxylases from C3 and C3-C4 species had slightly higher Km HCO3− and lower K′ phosphoenolpyruvate values than did the C4 enzyme. With each species at pH 7.3, all K′ phosphoenolpyruvate values were 2- to 4-fold greater. The enzyme from all species was inhibited 85 to 90% by 1 millimolar malate at rate-limiting phosphoenolpyruvate and Mg2+ levels. With low levels of malate, 0.2 millimolar, the rate curve with respect to phosphoenolpyruvate was distinctly sigmoidal, and the inhibition was not eliminated at 5 millimolar phosphoenolpyruvate. Malate at 10 millimolar protected all phosphoenolpyruvate carboxylases from inactivation at 55 C at pH 5.5, but not at pH 8.3. Aspartate did not protect well. When incubated at 37 C at pH 8.3 without phosphoenolpyruvate, but with HCO3−, the enzyme from several C4 grasses lost 92 to 98% of the initial activity after 4 minutes, whereas the enzymes from C3 and C3-C4Panicum species retained 60 to 70% of their activities. In contrast, 5 millimolar phosphoenolpyruvate stabilized the enzyme at 37 C in all plant extracts. The phosphoenolpyruvate carboxylase from C3-C4 intermediate Panicum species has properties most similar to the enzyme from C3Panicum species. The higher leaf activity of the enzyme from the intermediate plants than from C3 species is not due to any unusual property assayed other than a higher Vmax. PMID:16661669

  12. Idiopathic Calcium Nephrolithiasis And Hypercalciuria: The Role Of Genes

    NASA Astrophysics Data System (ADS)

    Gambaro, Giovanni; Abaterusso, Cataldo

    2007-04-01

    Idiopathic calcium nephrolithiasis and hypercalciuria are multifactorial disease conditions, the pathogenesis of which involves the interaction of environmental and individual factors. Data support a strong role of genes in the pathogenesis of these two conditions. Findings obtained in monogenic disorders characterized by renal calcium stones, and/or hypercalciuria, and/or nephrocalcinosis have proposed a number of genes as candidate genes in the pathogenesis of the common idiopathic calcium nephrolithiasis and hypercalciuria. The physiological role of these genes, and findings in monogenic disorders and idiopathic, multifactorial disorders will be presented.

  13. Idiopathic granulomatous interstitial nephritis responsive to mycophenolate mofetil therapy.

    PubMed

    Leeaphorn, Napat; Stokes, Michael B; Ungprasert, Patompong; Lecates, William

    2014-04-01

    Granulomatous interstitial nephritis (GIN) is a rare histologic disease. Various causes have been reported in the literature, including drugs, sarcoidosis, and infections. Other incidents have no discernible cause and are identified as idiopathic. We report a 68-year-old white man who presented with acute kidney injury and was given a diagnosis of idiopathic GIN. Mycophenolate mofetil treatment was elected because of steroid toxicity. He responded well to mycophenolate mofetil and has been in remission for more than 3 years. To our knowledge, this is the first report of successful treatment with mycophenolate mofetil of an adult patient with idiopathic GIN. PMID:24315767

  14. X-rays from the SNR 3C 391

    NASA Technical Reports Server (NTRS)

    Seward, F. D.; Wang, Z. R.

    1984-01-01

    An X-ray map and spectral information were obtained from a short Einstein observation of 3C 391 (G31.9 + 0.0). Both X-ray and radio emission appear to come from an irregular shell 5 arcmin in diameter. For a distance of 11 kpc the X-ray luminosity is at least 10 to the 35th ergs/sec. The luminosity, the radius, and the temperature are about as expected from a middle-aged SNR expanding into a medium with density a few tenths of an atom per cu cm.

  15. Discovery of an optical synchrotron jet in 3C 264

    NASA Technical Reports Server (NTRS)

    Crane, P.; Peletier, R.; Baxter, D.; Sparks, W. B.; Albrecht, R.; Barbieri, C.; Blades, J. C.; Boksenberg, A.; Deharveng, J. M.; Disney, M. J.

    1993-01-01

    Observations with the Faint Object Camera on board the Hubble Space Telescope have revealed a new optical jet in the core of the elliptical galaxy NGC 3862 (3C 264). Morphologically, this jet is similar to the synchrotron jets seen in other galaxies, as it shows knots and bifurcations. The optical spectral index is also similar to that found in other jets. Thus, the nucleus of NGC 3862 appears to contain the fifth known example of an optical synchrotron jet. Since NGC 3862 is a typical radio-loud elliptical galaxy, it seems likely that many nonthermal jets found in the radio continuum may also have optical counterparts.

  16. The optical variability periodicity analysis of 3C273

    NASA Astrophysics Data System (ADS)

    Fan, J. H.; Romero, G. E.; Lin, R. G.

    2001-02-01

    The authors have compiled measurements of ~ 110 years in the B-band of the quasar 3C273 and used this database to search for periodicity signals in the optical light curve. Two different methods were applied: the Jurkevich technique and the discrete correlation function (DCF) method. They revealed the existence of periods of 2.0, (13.65+/-0.20) and (22.50+/-0.20) years in the source variability. The possible origin of such a behavior is also discussed.

  17. Infrared variability properties of the blazar 3C 279

    NASA Astrophysics Data System (ADS)

    Fan, J. H.

    1999-10-01

    The long-term (about 27 years) near-infrared K light curve is constructed from the published literature for the blazar 3C 279. The Jurkevich method is adopted to analyse the periodicity, and a strong 7.1+/-0.44yr period is found, suggesting that the next near-infrared outburst will occur in 2002/03. The correlation between colour index (spectral index) and magnitude is discussed, and a significant correlation between (J-H) and K is found with a correlation coefficient r=0.72 (p=2.0x10^-10), which is consistent with Brown et al.'s proposal.

  18. Photopolarimetry of Blazar 3C454.3 from MIRO

    NASA Astrophysics Data System (ADS)

    Baliyan, Ks; Ganesh, S.; Chandra, Sunil; Joshi, Uc

    2009-12-01

    The Blazar 3C 454.3 has been active in Gamma-rays, optical and X- rays since Sept. 2009 ( Atel #2181, #2200, #2201). Very recently, it has been reported to be flaring up in the optical, X-ray and gamma-ray energy regimes(ATel #2322; #2325; #2326; #2328; #2329; #2330; #2332). In Atel #2333, Sasada et al report optical behaviour of this source on Dec 1.9 with brightness (V=14.06+/-0.02 and degree of polarization 6.0+/-0.1% on the same epoch.

  19. The optical variability of the quasar 3C 446

    SciTech Connect

    Barbieri, C.; Vio, R.; Cappellaro, E; Turatto, M Padova Osservatorio Astronomico, Padua )

    1990-08-01

    The optical variability of the quasar 3C 446 is investigated using power spectrum and structure function analysis along with a new set of observations that extend the available data till 1989. No contradiction is found between the PS and SF analyses. The presence of the 1540-day periodicity is strengthened by the occurrence of the 1988 luminosity peak, suggesting that the next burst will occur in the northern spring of 1992. The time series of the quasar is nonstationary. The light variations are determined by a sequence of luminosity bursts, mostly regularly spaced in time and lasting up to 2 yr. 25 refs.

  20. Idiopathic pleuroparenchymal fibroelastosis, a new idiopathic interstitial pneumonia: A case report.

    PubMed

    Hurtado, Enrique Javier Soto; González, Maria Luisa Amaya; Soto, Maria Del Mar Elena; Rueda, Francisco Jose Cabello; Nadal, Francisco Javier Pérez; Cantero, Alberto Ruíz

    2016-08-01

    Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease, idiopathic in most of the cases described in the literature. We report the case of a 55-year-old patient, non-smoker with tuberculosis treated in his youth, starting with progressive dyspnoea and cough, with radiographic abnormalities consisting of pleural thickening, bronchiectasis and structural distortion predominantly in the upper lobes. Due to functional impairment and persistent radiographic abnormalities, biopsy by video-assisted thoracoscopic surgical was decided. The presence of striking elastosis, absence of interstitial damage and abrupt boundary of the fibrous lesion with healthy lung allowed the diagnosis of IPPFE. Currently, the patient has no specific treatment and is in follow-up in the Transplant Unit. PMID:26810762

  1. The Idiopathic Intracranial Hypertension Treatment Trial

    PubMed Central

    Wall, Michael; Kupersmith, Mark J.; Kieburtz, Karl D.; Corbett, James J.; Feldon, Steven E.; Friedman, Deborah I.; Katz, David M.; Keltner, John L.; Schron, Eleanor B.; McDermott, Michael P.

    2015-01-01

    IMPORTANCE To our knowledge, there are no large prospective cohorts of untreated patients with idiopathic intracranial hypertension (IIH) to characterize the disease. OBJECTIVE To report the baseline clinical and laboratory features of patients enrolled in the Idiopathic Intracranial Hypertension Treatment Trial. DESIGN, SETTING, AND PARTICIPANTS We collected data at baseline from questionnaires, examinations, automated perimetry, and fundus photography grading. Patients (n = 165) were enrolled from March 17, 2010, to November 27, 2012, at 38 academic and private practice sites in North America. All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation between −2 dB and −7 dB. All but 4 participants were women. MAIN OUTCOMES AND MEASURES Baseline and laboratory characteristics. RESULTS The mean (SD) age of our patients was 29.0 (7.4) years and 4 (2.4%) were men. The average (SD) body mass index (calculated as weight in kilograms divided by height in meters squared) was 39.9 (8.3). Headache was the most common symptom (84%). Transient visual obscurations occurred in 68% of patients, back pain in 53%, and pulse synchronous tinnitus in 52%. Only 32% reported visual loss. The average (SD) perimetric mean deviation in the worst eye was −3.5 (1.1) dB, (range, −2.0 to −6.4 dB) and in the best eye was −2.3 (1.1) dB (range, −5.2 to 0.8 dB). A partial arcuate visual field defect with an enlarged blind spot was the most common perimetric finding. Visual acuity was 85 letters or better (20/20) in 71% of the worst eyes and 77% of the best eyes. Quality of life measures, including the National Eye Institute Visual Function Questionnaire–25 and the Short Form–36 physical and mental health summary scales, were lower compared with population norms. CONCLUSIONS AND RELEVANCE The Idiopathic Intracranial Hypertension Treatment Trial represents the largest prospectively analyzed cohort of untreated patients with IIH. Our data show

  2. Photonic Crystal Cavities in Cubic (3C) Silicon Carbide

    NASA Astrophysics Data System (ADS)

    Radulaski, Marina; Babinec, Thomas; Buckley, Sonia; Rundquist, Armand; Provine, J.; Alassaad, Kassem; Ferro, Gabriel; Vuckovic, Jelena

    2014-03-01

    Silicon carbide (SiC) combines many of the outstanding material properties of other well-known optical and quantum optical materials, including strong optical nonlinearity, high Young's modulus, and a host of optically-active crystalline defects, in a single CMOS-compatible platform. For many applications in classical and quantum information processing, the material properties of the cubic silicon carbide polytype (3C-SiC) in particular are advantageous. We therefore present the design, fabrication, and characterization of high quality factor and small mode volume planar photonic crystal cavities in cubic 3C-SiC thin films (200 nm). We demonstrate cavity resonances across the infrared telecommunications band, with wavelengths from 1.25 - 1.6 μm. Finally, we highlight our progress developing higher Q/V nanobeam cavities, as well as extending this optical cavity platform towards integration with SiC color centers. PECASE Grant ECCS-10 25811, NSF Grant ECS-9731293, Stanford Graduate Fellowship, National Science Graduate Fellowship.

  3. Chronic idiopathic urticaria and anxiety symptoms.

    PubMed

    Barbosa, Filipe; Freitas, João; Barbosa, António

    2011-10-01

    Chronic idiopathic urticaria (CIU) is a frequently disabling disease with a negative influence on the quality of life, and can cause psychopathological symptoms, such as anxiety. Our aim is to study further anxiety symptoms on CIU patients. Both CIU patients and the control group were studied by means of validated scales for psychopathology symptoms, psychological variables and quality of life. In this study, we reported high levels of anxiety symptoms. We found statistically significant correlations between anxiety symptoms, some personality dimensions, insecure attachment styles, alexithymia and with some quality of life dimensions. CIU patients exhibit high levels of psychological distress that could potentiate difficulties at several domains, namely social, emotional, general health perception and interpersonal relationships. PMID:21459916

  4. Chronic idiopathic urticaria: treatment with omalizumab.

    PubMed

    Naaman, Sandra; Sussman, Gordon

    2014-01-01

    Chronic idiopathic urticaria (CIU) is a common autoimmune skin condition characterized by spontaneously recurring hives for 6 weeks or longer. The new terminology used for CIU in most countries including Canada is chronic spontaneous urticaria (CSU). CSU is associated with significant psychosocial morbidity with a markedly negative impact on overall quality of life. Conventional approaches with antihistamines, even at high doses, is effective in about 50% of patients suffering from CSU. A new treatment option, omalizumab, a humanized monoclonal antibody against the Fc domain of IgE, has undergone the scrutiny of randomized research studies evaluating the efficacy in CSU. This editorial reviews mechanisms of action of omalizumab, efficacy, cost and potential side effect profile. Omalizumab has emerged as a very promising treatment option for patients with CSU. Future research is necessary to establish standardized protocols related to dosing as well as monitoring possible adverse effects of long-term treatment. PMID:25807072

  5. Idiopathic granulomatous mastitis: presentation, investigation and management.

    PubMed

    Benson, John R; Dumitru, Dorin

    2016-06-01

    Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory condition of the breast which although benign can mimic carcinoma. Establishing a diagnosis can be challenging and requires a high index of suspicion with exclusion of infective and autoimmune breast diseases. IGM is characterized histologically by noncaseating granulomas which are of a lobulo-centric pattern and often associated with microabscess formation. Management of confirmed cases remains controversial with proponents of initial surgical or medical therapies - each has its associated problems which can be worse than the original symptoms of IGM. However, many patients require more than one modality of treatment to completely resolve IGM lesions and careful judgment is necessary to ensure optimal type and sequencing of treatments. PMID:27067146

  6. An Update on Idiopathic Intracranial Hypertension

    PubMed Central

    Thurtell, Matthew J.; Bruce, Beau B.; Newman, Nancy J.; Biousse, Valérie

    2013-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology often encountered in neurologic practice. It produces non-localizing symptoms and signs of raised intracranial pressure and, when left untreated, can result in severe irreversible visual loss. It most commonly occurs in obese women of childbearing age, but it can also occur in children, men, non-obese adults, and older adults. While it is frequently associated with obesity, it can be associated with other conditions, such as obstructive sleep apnea and transverse cerebral venous sinus stenoses. Recent identification of subgroups at high risk for irreversible visual loss, including black patients, men, and patients with fulminant forms of IIH, help guide the optimal management and follow-up. Ongoing studies of venous anatomy and physiology in IIH patients, as well as a recently begun randomized clinical treatment trial, should provide more insights into this common yet poorly understood syndrome of isolated intracranial hypertension. PMID:20944524

  7. Idiopathic intracranial hypertension presenting as postpartum headache.

    PubMed

    Mathew, Mariam; Salahuddin, Ayesha; Mathew, Namitha R; Nandhagopal, Ramachandiran

    2016-01-01

    Postpartum headache is described as headache and neck or shoulder pain during the first 6 weeks after delivery. Common causes of headache in the puerperium are migraine headache and tension headache; other causes include pre-eclampsia/eclampsia, post-dural puncture headache, cortical vein thrombosis, subarachnoid hemorrhage, posterior reversible leukoencephalopathy syndrome, brain tumor, cerebral ischemia, meningitis, and so forth. Idiopathic intracranial hypertension (IIH) is a rare cause of postpartum headache. It is usually associated with papilledema, headache, and elevated intracranial pressure without any focal neurologic abnormality in an otherwise healthy person. It is more commonly seen in obese women of reproductive age group, but rare during pregnancy and postpartum. We present a case of IIH who presented to us 18 days after cesarean section with severe headache and was successfully managed. PMID:26818168

  8. Acute Exacerbations of Idiopathic Pulmonary Fibrosis

    PubMed Central

    Collard, Harold R.; Moore, Bethany B.; Flaherty, Kevin R.; Brown, Kevin K.; Kaner, Robert J.; King, Talmadge E.; Lasky, Joseph A.; Loyd, James E.; Noth, Imre; Olman, Mitchell A.; Raghu, Ganesh; Roman, Jesse; Ryu, Jay H.; Zisman, David A.; Hunninghake, Gary W.; Colby, Thomas V.; Egan, Jim J.; Hansell, David M.; Johkoh, Takeshi; Kaminski, Naftali; Kim, Dong Soon; Kondoh, Yasuhiro; Lynch, David A.; Müller-Quernheim, Joachim; Myers, Jeffrey L.; Nicholson, Andrew G.; Selman, Moisés; Toews, Galen B.; Wells, Athol U.; Martinez, Fernando J.

    2007-01-01

    The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed. PMID:17585107

  9. Malignant Hyperthermia and Idiopathic HyperCKemia.

    PubMed

    Kasi, Pashtoon Murtaza

    2011-01-01

    Malignant hyperthermia (MH) is a rare but life-threatening condition that is more frequently encountered and discussed within the anesthesia literature. Here we through a case specifically discuss the susceptibility of individuals and/or families with asymptomatic unexplained elevations of creatine kinase (CK), also frequently referred to as hyperCKemia or idiopathic hyperCKemia (IHCK) in recent reports. The clinical implications would be to underscore the importance of this as a susceptibility to developing MH and highlight the importance of genetic susceptibility testing in such cases. Anesthesiologists and critical care intensivists as well as primary care physicians should keep this in mind when seeing patients with asymptomatic hyperCKemia and potentially inform them about the possibility of developing MH if exposed to triggering agents. Genetic susceptibility testing should be considered if available and family members should also receive nontriggering agents when undergoing anesthesia and wear Medic Alert tags. PMID:22162697

  10. A Mathematical Model of Idiopathic Pulmonary Fibrosis

    PubMed Central

    Hao, Wenrui; Marsh, Clay; Friedman, Avner

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology, and life expectancy of 3-5 years after diagnosis. The incidence rate in the United States is estimated as high as 15 per 100,000 persons per year. The disease is characterized by repeated injury to the alveolar epithelium, resulting in inflammation and deregulated repair, leading to scarring of the lung tissue, resulting in progressive dyspnea and hypoxemia. The disease has no cure, although new drugs are in clinical trials and two agents have been approved for use by the FDA. In the present paper we develop a mathematical model based on the interactions among cells and proteins that are involved in the progression of the disease. The model simulations are shown to be in agreement with available lung tissue data of human patients. The model can be used to explore the efficacy of potential drugs. PMID:26348490

  11. MUC5B and Idiopathic Pulmonary Fibrosis.

    PubMed

    Yang, Ivana V; Fingerlin, Tasha E; Evans, Christopher M; Schwarz, Marvin I; Schwartz, David A

    2015-11-01

    Idiopathic pulmonary fibrosis (IPF), a fatal disease that is a result of complex interactions between genetics and the environment, has limited treatment options. We have identified the MUC5B promoter polymorphism and other common genetic variants that in aggregate explain roughly one-third of disease risk. The MUC5B promoter polymorphism is the strongest and the most replicated genetic risk factor for IPF, appears to be protective and predictive in this disease, and is likely involved in disease pathogenesis through an increase in MUC5B expression in terminal bronchi and honeycombed cysts. Expression of MUC5B is also highly correlated with expression of cilium genes in IPF lung. Our work suggests that mucociliary dysfunction in the distal airway may play a role in the development of progressive fibroproliferative lung disease. In addition, our work has important implications for secondary prevention, early detection, and future early and personalized treatment based on genetic profiles. PMID:26595739

  12. Cardiac manifestations of idiopathic pulmonary fibrosis

    PubMed Central

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-01-01

    Summary Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14–43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality. PMID:27195188

  13. Cardiac manifestations of idiopathic pulmonary fibrosis.

    PubMed

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14-43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality. PMID:27195188

  14. Isolated unilateral idiopathic transient hypoglossal nerve palsy

    PubMed Central

    Ahmed, Syed Viqar; Akram, Muhammad Saqub

    2014-01-01

    A 52-year-old Caucasian man presented with sudden onset of difficulty in moving his tongue to the left with preceding left-sided headache with no neck pain. Earlier, he had self-limiting chest infection without rashes or tonsillar enlargement. His medical and surgical history was unremarkable with no recent trauma. Oral examination revealed difficulty in protruding his tongue to the left with muscle bulk loss and fasciculation on the same side, suggesting left hypoglossal nerve palsy. Examination of the rest of the cranial nerves and nervous system was normal. The patient's oropharyngeal and laryngeal examination was unremarkable with no cervical lymphadenopathy. He had normal laboratory investigations and cerebrospinal fluid examination. Extensive imaging of the head, neck and chest failed to reveal any pathology. Further review by an otorhinologist and rheumatologist ruled out any other underlying pathology. He made a good recovery without treatment. English literature search revealed very few cases of idiopathic, transient, unilateral hypoglossal nerve palsy. PMID:24969070

  15. [Idiopathic pulmonary fibrosis and lung cancer].

    PubMed

    Yoshimura, Akinobu; Kudoh, Syoji

    2003-02-01

    It is widely known that patients with idiopathic pulmonary fibrosis (IPF) are frequently associated with lung cancer. Although a complication with lung cancer is an important prognostic factor for IPF, standard treatments for lung cancer cannot be given because of IPF. Especially, the administration of many anticancer agents is limited by a complication with IPF, which is recognized as a risk factor for the development of fatal lung injury in cancer chemotherapy. Epidemiological studies reveal that cigarette smoking and occupational and environmental exposure to toxic substances are common risk factors for both IPF and lung cancer. It has been assumed that metaplasia in fibrous lesions is pathologically a precancerous lesion, but it is necessary to prove several genetic abnormalities in the process of carcinogenesis in order to clarify that. Currently, several genetic abnormalities in IPF, including in p53, K-ras, FHIT and transforming growth factor (TGF)-beta 1 type II receptor, have been reported. PMID:12610869

  16. White Matter Microstructure in Idiopathic Craniocervical Dystonia

    PubMed Central

    Pinheiro, Giordanna L. S.; Guimarães, Rachel P.; Piovesana, Luiza G.; Campos, Brunno M.; Campos, Lidiane S.; Azevedo, Paula C.; Torres, Fabio R.; Amato-Filho, Augusto C.; França, Marcondes C.; Lopes-Cendes, Iscia; Cendes, Fernando; D’Abreu, Anelyssa

    2015-01-01

    Background Dystonias are hyperkinetic movement disorders characterized by involuntary muscle contractions resulting in abnormal torsional movements and postures. Recent neuroimaging studies in idiopathic craniocervical dystonia (CCD) have uncovered the involvement of multiple areas, including cortical ones. Our goal was to evaluate white matter (WM) microstructure in subjects with CCD using diffusion tensor imaging (DTI) analysis. Methods We compared 40 patients with 40 healthy controls. Patients were then divided into subgroups: cervical dystonia, blepharospasm, blepharospasm + oromandibular dystonia, blepharospasm + oromandibular dystonia + cervical dystonia, using tract-based spatial statistics. We performed a region of interest-based analysis and tractography as confirmatory tests. Results There was no significant difference in the mean fractional anisotropy (FA) and mean diffusivity (MD) between the groups in any analysis. Discussion The lack of DTI changes in CCD suggests that the WM tracts are not primarily affected. PMID:26056610

  17. Dobosiewicz method physiotherapy for idiopathic scoliosis.

    PubMed

    Dobosiewicz, Krystyna; Durmala, Jacek; Kotwicki, Tomasz

    2008-01-01

    The method developed since 1979, comprises active 3-dimensional auto-correction, concerning the primary curve mobilization towards the correction of the curvature, with special emphasis on the kyphotization of the thoracic spine, carried on in closed kinematic chains, and developed on a symmetrically positioned pelvis and shoulder girdle, followed by active stabilization of the corrected position, and endured as postural habit. The positions for exercising and the movements involved are described in details. Small, moderate and important curves can be managed with DoboMed, however the effectiveness of the therapy depends on the curve flexibility and patient's compliance. DoboMed has been used as a single therapy or together with bracing, as well as preparation for scoliosis surgery. The published results demonstrated that the DoboMed has a positive influence on inhibition of the curve progression in idiopathic scoliosis, the improvement of respiratory functions, assessed by the spirometric values, and the general exercise efficiency evaluated using ergospirometry. PMID:18401093

  18. Managing comorbidities in idiopathic pulmonary fibrosis

    PubMed Central

    Fulton, Blair G; Ryerson, Christopher J

    2015-01-01

    Major risk factors for idiopathic pulmonary fibrosis (IPF) include older age and a history of smoking, which predispose to several pulmonary and extra-pulmonary diseases. IPF can be associated with additional comorbidities through other mechanisms as either a cause or a consequence of these diseases. We review the literature regarding the management of common pulmonary and extra-pulmonary comorbidities, including chronic obstructive pulmonary disease, lung cancer, pulmonary hypertension, venous thromboembolism, sleep-disordered breathing, gastroesophageal reflux disease, coronary artery disease, depression and anxiety, and deconditioning. Recent studies have provided some guidance on the management of these diseases in IPF; however, most treatment recommendations are extrapolated from studies of non-IPF patients. Additional studies are required to more accurately determine the clinical features of these comorbidities in patients with IPF and to evaluate conventional treatments and management strategies that are beneficial in non-IPF populations. PMID:26451121

  19. Idiopathic pulmonary hemosiderosis - a diagnostic challenge.

    PubMed

    Bakalli, Ilirjana; Kota, Luljeta; Sala, Durim; Celaj, Ermela; Kola, Elmira; Lluka, Robert; Sallabanda, Sashenka

    2014-01-01

    Idiopathic pulmonary hemosiderosis is a rare disorder that can occur at any age and is characterized by the triad of hemoptysis, iron deficiency anemia and diffuse pulmonary infiltrates. The clinical course is exceedingly variable especially in children and a substantial proportion of this age group is undiagnosed. It is probably due to the fact that iron deficiency anemia may be the first and the only manifestation of IPH, preceding other symptoms and signs by several months and IPH is not considered as a rare cause of anemia, unless the typical triad is present. We present a case of IPH in a 13-year-old girl, treated for several months with persistent iron deficiency anemia, without response to therapy. PMID:24708654

  20. Genetics Home Reference: familial idiopathic basal ganglia calcification

    MedlinePlus

    ... in regulating phosphate levels within the body (phosphate homeostasis) by transporting phosphate across cell membranes. The SLC20A2 ... link familial idiopathic basal ganglia calcification with phosphate homeostasis. Nat Genet. 2012 Feb 12;44(3):254- ...

  1. [Idiopathic facial pain--Part 1: Definition, classification and etiology].

    PubMed

    Reher, P; Harris, M

    1998-01-01

    The present paper introduces a new concept in the physiopathology and treatment of the called 'temporomandibular dysfunction'. This paper was divided into two parts, this first explaining its definition, aetiology and hypothesis of the idiopathic facial pain. The second part, will mention its treatment, prognosis and recommendations. Under this new view, this 'dysfunction' is part of the called Idiopathic Facial Pain. The idiopathic facial pain is didactically subdivided in (1) facial artromialgia, (2) atypical facial pain, (3) atypical odontalgia and (4) oral dysestesia. Occlusal factors are less important in its aetiology, and psychological and biochemical factors are more recognised. Correct diagnosis is essential, and a full history, including psychological and social aspects is very important. The present management is based on a working model developed in this department and in other world centres, and is revolutionising the clinical management of the patients with idiopathic facial pain. PMID:9922499

  2. Long term combination treatment for severe idiopathic pulmonary arterial hypertension

    PubMed Central

    Affuso, Flora; Cirillo, Plinio; Ruvolo, Antonio; Carlomagno, Guido; Fazio, Serafino

    2010-01-01

    We report the long-term follow-up of 3 cases of severe idiopathic pulmonary arterial hypertension, in whom tadalafil plus sitaxentan combination therapy improved the clinical condition and exercise performance without any relevant adverse event. PMID:21160759

  3. Idiopathic Paroxysmal Ventricular Tachycardia in Infants and Children

    ERIC Educational Resources Information Center

    Hernandez, Antonio; And Others

    1975-01-01

    Laboratory tests including blood count serum electrolyte measures, and electroencephalograms were performed on seven children ages 1 day to 18 years with recurrent attacks of rapid heart action known as idiopathic paroxysmal ventricular tachycardia. (CL)

  4. Microcatheter Embolization of Intractable Idiopathic Epistaxis

    SciTech Connect

    Leppaenen, Martti; Seppaenen, Seppo; Laranne, Jussi; Kuoppala, Katriina

    1999-11-15

    Purpose: To assess the efficacy and safety of microcatheter embolization in the treatment of intractable idiopathic epistaxis. Methods: Thirty-seven patients underwent microcatheter embolization in 1991-1998. We evaluated retrospectively the technical and clinical outcome, the number of complications, the duration of embolization in each case, and the number of blood transfusions needed. All embolizations were done with biplane digital subtraction angiography (DSA) equipment. The procedure was carried out under local anesthesia using transfemoral catheterization, except in one case where the translumbar route was used. Tracker 18 or 10 microcatheters were advanced as far as possible to the distal branches of the sphenopalatine artery. Polyvinyl alcohol (PVA) particles were used for embolization in most cases, while platinum coils or a combination of these two materials were occasionally used. The primary outcome was always assessed immediately by angiography. Follow-up data were obtained from patient records, by interviewing patients on the telephone or by postal questionnaires when necessary. The mean follow-up time was 21 months. Results: The embolization was technically successful in all 37 cases. A curative outcome was achieved in 33 cases (89%). The mean duration of the procedure was 110 min. Four patients (8%) had mild transient complications, but no severe or persistent complications were encountered. Twenty-three patients needed a blood transfusion. Slight rebleeding occurred in three patients during the follow-up; all responded to conservative treatment. One patient suffered two episodes of rebleeding within 2 months after primary embolization. Re-embolizations successfully stopped the bleeding. Conclusion: Embolization is the primary invasive modality for treating intractable idiopathic epistaxis. It proved both safe and effective over a relatively long follow-up.

  5. Impact of juvenile idiopathic arthritis on schooling

    PubMed Central

    2013-01-01

    Background Juvenile idiopathic arthritis (JIA) is the most common arthropathy of childhood. Different diseases affect school attendance to varying degrees. The aim of this study was to assess the impact of juvenile idiopathic arthritis (JIA) on Moroccan children’s schooling. Methods Thirty-three children with JIA were included in this study, having been previously diagnosed according to the classification criteria of the International League of Associations for Rheumatology (ILAR). Seventy-four healthy children were recruited to serve as controls. Data was obtained for all children on their school level, educational performance, and attendance. The rate of absenteeism due to health complications was noted. Results All healthy children were able to attend school (p<0.0001), while 33% of children with JIA were unable to attend school due to their condition. The students with JIA who were able to attend school were absent much more often than controls (63% compared to 20%), with a highly significant p value (p<0.0001). Slightly less than half of the JIA patients (48.5%) failed in their schooling. In univariate analysis, there was an association between absenteeism and tender joints (p=0.02), disease activity score (DAS28) (p=0.007), Childhood Health Assessment Questionnaire (CHAQ) (p=0.01), and erythrocyte sedimentation rate (ESR) (p=0.03). In multivariate analysis, the only association persisted between DAS28 and absenteeism. Conclusions Our study suggested that the schooling of children with JIA was negatively impacted due to the disorder. More studies, with a larger sample of children, are needed to confirm our findings. PMID:23289498

  6. Idiopathic corporeal hemihypertrophy associated with hemihypertrichosis.

    PubMed

    Maniar, S; Azzi, K; Iraqi, H; El Hassan Garbi, M; Chraibi, A; Gaouzi, A

    2011-02-01

    The hemihypertrophy or hemihyperplasy is a rare congenital abnormality, characterized by an asymmetric growth of the limbs, the trunk, and the face or half of the entire body. It may be isolated or be part of several syndromes including Beckwith-Wiedemann syndrome, Klippel-Trenaunay-Weber syndrome, Silver-Russell syndrome and Proteus syndrome. In its isolated form, it is called idiopathic. The latter may be associated with several anomalies including dermatological and urogenital abnormalities with increased risk of developing embryonal tumors. We report the case of a 22-month-old infant, who was referred by his pediatrician at the age of 15 months for a corporeal hemihypertrophy associated with hemihypertrichosis. In his medical history, a second degree parental consanguinity and a hypospadias in the father and a paternal uncle were found. Clinical examination found a weight and a size greater than chronological age (3 standard deviations), a hemihypertrophy of entire left side with a difference of length and diameter between the left and right limbs of 2 cm. The hemihypertrichosis was widespread in the left body and the genital examination found a hypospadias. Biological and radiological assessments did not show any abnormality, with the exception of an initially high plasma testosterone level, which gradually normalized. Thus, the diagnosis of idiopathic hemihypertrophy with congenital hemihypertrichosis was retained. This is the fourth case reported in the literature. Its management is similar to all hemihypertrophies, which consists of an initial assessment to detect an embryonic tumor, followed by a monitoring protocol including an abdominal and renal ultrasound every 6 months until the age of 8, determination of alpha-feto-protein every 6 to 12 weeks until the age of 4 years to track the development of the two most frequent tumors: Wilms tumor and hepatoblastoma. The hemihypertrophy associated with hemihypertrichosis has been exceptionally reported and the

  7. Sleepiness in Idiopathic REM Sleep Behavior Disorder and Parkinson Disease

    PubMed Central

    Arnulf, Isabelle; Neutel, Dulce; Herlin, Bastien; Golmard, Jean-Louis; Leu-Semenescu, Smaranda; Cochen de Cock, Valérie; Vidailhet, Marie

    2015-01-01

    Objective: To determine whether patients with idiopathic and symptomatic RBD were sleepier than controls, and if sleepiness in idiopathic RBD predicted earlier conversion to Parkinson disease. Methods: The Epworth Sleepiness Scale (ESS) and its determinants were compared at the time of a video-polysomnography for an RBD diagnosis in patients with idiopathic RBD, in patients with Parkinson disease, and in controls. Whether sleepiness at time of RBD diagnosis predicted an earlier conversion to neurodegenerative diseases was retrospectively analyzed in the followed-up patients. Results: The 75 patients with idiopathic RBD were sleepier (ESS: 7.8 ± 4.6) at the time of RBD diagnosis than 74 age- and sex-matched controls (ESS: 5.0 ± 3.6, P < 0.0001). They reached the levels of 114 patients with Parkinson disease (ESS: 8.7 ± 4.8), whether they had (n = 78) or did not have (n = 36) concomitant RBD. The severity of sleepiness in idiopathic RBD correlated with younger age, but not with sleep measures. Among the 69 patients with idiopathic RBD who were followed up for a median 3 years (1–15 years), 16 (23.2%) developed parkinsonism (n = 6), dementia (n = 6), dementia plus parkinsonism (n = 2), and multiple system atrophy (n = 2). An ESS greater than 8 at time of RBD diagnosis predicted a shorter time to phenoconversion to parkinsonism and dementia, from RBD onset, and from RBD diagnosis (when adjusted for age and time between RBD onset and diagnosis). Conclusions: Sleepiness is associated with idiopathic REM sleep behavior disorder and predicts more rapid conversion to parkinsonism and dementia, suggesting it is an early marker of neuronal loss in brainstem arousal systems. Citation: Arnulf I, Neutel D, Herlin B, Golmard JL, Leu-Semenescu S, Cochen de Cock V, Vidailhet M. Sleepiness in idiopathic REM sleep behavior disorder and Parkinson disease. SLEEP 2015;38(10):1529–1535. PMID:26085299

  8. Substitutional Ge in 3C-SiC

    NASA Astrophysics Data System (ADS)

    Guedj, C.; Kolodzey, J.

    1999-02-01

    The incorporation of substitutional Ge into 3C-SiC alloys is studied theoretically with an anharmonic Keating model specifically adapted to the computation of the structural properties and the lattice dynamics of Si1-x-yGexCy alloys. Basic energy calculations show that the substitution of Si by Ge is more probable than the substitution of C by Ge in the zinc-blende silicon carbide crystal. If Ge replaces only Si, then the lattice parameter equals (0.43593±0.00002)+(0.000337±0.000002)y, where y stands for the Ge content. Hence, Vegard's law is not applicable. The alloy is characterized by a distinct phonon spectrum whose maximum peak position in cm-1 is best described by the exponential decay (243±1)+(27±2)exp[-y/(7.5±1.2)] up to the zinc-blende GeC compound.

  9. Behavior of inversion layers in 3C silicon carbide

    NASA Technical Reports Server (NTRS)

    Avila, R. E.; Kopanski, J. J.; Fung, C. D.

    1986-01-01

    A study on the field-induced surface-charge region in 3C silicon carbide (SiC) using 1 MHz capacitance-voltage (C-V) measurements at room temperature is here reported. A double column mercury probe was used on oxidized SiC substrates to form metal-oxide-semiconductor (MOS) structures. These structures were characterized in terms of the substrate doping profile, effective fixed oxide charge, and interface trap density. A distinctive feature of the MOS C-V curves from accumulation to inversion is that after going into deep depletion the capacitance rises to its equilibrium inversion level during the voltage sweep. Capacitance transient measurements indicate that the minority-carrier generation occurs at the SiO2/SiC interface.

  10. Behavior of inversion layers in 3C silicon carbide

    NASA Astrophysics Data System (ADS)

    Avila, R. E.; Kopanski, J. J.; Fung, C. D.

    1986-08-01

    A study on the field-induced surface-charge region in 3C silicon carbide (SiC) using 1 MHz capacitance-voltage (C-V) measurements at room temperature is here reported. A double column mercury probe was used on oxidized SiC substrates to form metal-oxide-semiconductor (MOS) structures. These structures were characterized in terms of the substrate doping profile, effective fixed oxide charge, and interface trap density. A distinctive feature of the MOS C-V curves from accumulation to inversion is that after going into deep depletion the capacitance rises to its equilibrium inversion level during the voltage sweep. Capacitance transient measurements indicate that the minority-carrier generation occurs at the SiO2/SiC interface.

  11. Structural Variability of 3C 111 on Parsec Scales

    NASA Technical Reports Server (NTRS)

    Grossberger, C.; Kadler, M.; Wilms, J.; Muller, C.; Beuchert, T.; Ros, E.; Ojha, R.; Aller, M.; Aller, H.; Angelakis, E.; Fuhrmann, L.; Nestoras, I.; Schmidt, R.; Zensus, J. A.; Krichbaum, T. P.; Ungerechts, H.; Sievers, A.; Riquelme, D.

    2011-01-01

    We discuss the parsec-scale structural variability of the extragalactic jet 3C 111 related to a major radio flux density outburst in 2007, The data analyzed were taken within the scope of the MOJAVE, UMRAO, and F-GAMMA programs, which monitor a large sample of the radio brightest compact extragalactic jets with the VLBA, the University of Michigan 26 m, the Effelsberg 100 m, and the IRAM 30 m radio telescopes. The analysis of the VLBA data is performed by fitting Gaussian model components in the visibility domain, We associate the ejection of bright features in the radio jet with a major flux-density outburst in 2007, The evolution of these features suggests the formation of a leading component and multiple trailing components

  12. Trisodium citrate, Na3(C6H5O7)

    PubMed Central

    Rammohan, Alagappa; Kaduk, James A.

    2016-01-01

    The crystal structure of anhydrous tris­odium citrate, Na3(C6H5O7), has been solved and refined using synchrotron X-ray powder diffraction data, and optimized using density functional theory (DFT). There are two independent five-coordinate Na+ and one six-coordinate Na+ cations in the asymmetric unit. The [NaO5] and [NaO6] polyhedra share edges and corners to form a three-dimensional framework. There are channels parallel to the a and b axes in which the remainder of the citrate anions reside. The only hydrogen bonds are an intra­molecular one between the hy­droxy group and one of the terminal carboxyl­ate O atoms and an intermolecular one between a methylene group and the hydroxyl O atom. PMID:27308044

  13. Milliarcsecond polarization structure of the superluminal quasar 3C 273

    NASA Astrophysics Data System (ADS)

    Roberts, David H.; Kollgaard, Ronald I.; Brown, Leslie F.; Gabuzda, Denise C.; Wardle, John F. C.

    1990-09-01

    A 2 x 10 marcsec-resolution determination is presented for the total intensity and linear polarization structures of the superluminal quasar 3C 273 at 5 GHz. Substantial polarized flux was detected from several superluminal components of the jet, whose fractional polarization increased symmetrically with distance from the core; the most distant component is highly polarized and exhibits a highly ordered magnetic field. Within a few marcsec of the core, the inferred magnetic field orientation varies rapidly with position along the jet. The primarily longitudinal magnetic field orientation is concluded to become established within 20 marcsec of the core. A highly disorganized magnetic field is the most plausible explanation for the low degree of polarization in the innermost regions of the jet.

  14. The Parsec-Scale Jet of Quasar 3C345

    NASA Astrophysics Data System (ADS)

    Zensus, J. A.; Rabaca, C. R.

    1993-12-01

    We discuss the parsec-scale structure of the superluminal quasar 3C345. Monitoring of the structure with VLBI at cm and mm wavelengths has shown apparent superluminal motion of at least four distinct emission features, over a distance of more than 40 pc from the stationary core (Zensus, Cohen, and Unwin, submitted to APJ). Near the core, the projected trajectories are curved and different for individual components, indicative of more complicated flow patterns than previously suspected, and consistent with motion along helical paths. The motion accelerates with increasing separation from the core, as the jet curves towards the extended kiloparsec structure. The flux evolution of individual components can be described using a generalized shock model. We apply this to component C4 and discuss the impact of orientation effects and implications for specific shock models.

  15. Extended Ly-alpha emission associated with 3C 294

    NASA Technical Reports Server (NTRS)

    Mccarthy, Patrick J.; Spinrad, Hyron; Dickinson, Mark; Van Breugel, Wil; Liebert, James; Djorgovski, S.; Eisenhardt, Peter

    1990-01-01

    Optical, IR, and radio observations of the powerful radio source 3C 294, which is surrounded by a large cloud of ionized gas, are presented. The galaxy is faint in the rest-frame UV, yet has a near-IR luminosity that is typical of radio galaxies at redshifts of order two. In contrast to the large extent of the ionized gas, the K-band image is quite compact. The emission-line cloud is closely aligned with the radio source axis and has an ionization state indicative of ionization by a nonstellar source. The velocity field of the gas has both large ordered motions and large turbulent components. The total mass required to keep the gas bound to the system is comparable to present-day massive galaxies and their halos. The velocity fields of the high-ionization lines are systematically different from Ly-alpha in a manner that is not easily understood.

  16. NIR Flaring of the Quasar 3C454.3

    NASA Astrophysics Data System (ADS)

    Carrasco, L.; Carramiñana, A.; Recillas, E.; Escobedo, G.; Porras, A.; Mayya, Y. D.; Valdes, J. R.

    2010-10-01

    We report the ongoing NIR flare of the quasar 3C454.3, also known as [HB89]2251+158. It is likely associated with a gamma ray source CGRaBSJ2253+1608 and the radio source WMAP055. It is an intermediate redshift FRSQSO Z=0.859 (RA=22:53:57.75, Dec=+16:08:53.6(J2000). On October 31th,2010 (JD 2455500.781451), we determined the NIR flux from this object to correspond to H = 11.190 +/- 0.03, 0.63mag brighter than it was two days earlier(JD 2455498.821166) when we determined it to have H = 11.820 +/- 0.03.

  17. Development of potent inhibitors of the coxsackievirus 3C protease

    SciTech Connect

    Lee, Eui Seung; Lee, Won Gil; Yun, Soo-Hyeon; Rho, Seong Hwan; Im, Isak; Yang, Sung Tae; Sellamuthu, Saravanan; Lee, Yong Jae; Kwon, Sun Jae; Park, Ohkmae K.; Jeon, Eun-Seok; Park, Woo Jin . E-mail: wjpark@gist.ac.kr; Kim, Yong-Chul . E-mail: yongchul@gist.ac.kr

    2007-06-22

    Coxsackievirus B3 (CVB3) 3C protease (3CP) plays essential roles in the viral replication cycle, and therefore, provides an attractive therapeutic target for treatment of human diseases caused by CVB3 infection. CVB3 3CP and human rhinovirus (HRV) 3CP have a high degree of amino acid sequence similarity. Comparative modeling of these two 3CPs revealed one prominent distinction; an Asn residue delineating the S2' pocket in HRV 3CP is replaced by a Tyr residue in CVB3 3CP. AG7088, a potent inhibitor of HRV 3CP, was modified by substitution of the ethyl group at the P2' position with various hydrophobic aromatic rings that are predicted to interact preferentially with the Tyr residue in the S2' pocket of CVB3 3CP. The resulting derivatives showed dramatically increased inhibitory activities against CVB3 3CP. In addition, one of the derivatives effectively inhibited the CVB3 proliferation in vitro.

  18. Association of exposure to phenols and idiopathic male infertility.

    PubMed

    Chen, Minjian; Tang, Rong; Fu, Guangbo; Xu, Bin; Zhu, Pengfei; Qiao, Shanlei; Chen, Xiaojiao; Xu, Bo; Qin, Yufeng; Lu, Chuncheng; Hang, Bo; Xia, Yankai; Wang, Xinru

    2013-04-15

    Widespread human exposure to phenols has been documented recently, and some phenols which are potential endocrine disruptors have demonstrated adverse effects on male reproduction in animal and in vitro studies. However, implications about exposure to phenols and male infertility are scarce in humans. Case-control study of 877 idiopathic infertile men and 713 fertile controls was conducted. Urinary levels of bisphenol A, benzophenone-3, pentachlorophenol, triclosan, 4-tert-octylphenol (4-t-OP), 4-n-octylphenol (4-n-OP) and 4-n-nonylphenol (4-n-NP) and semen parameters were measured. After multivariate adjustment, we found 4-t-OP, 4-n-OP and 4-n-NP exposure was associated with idiopathic male infertility (p-value for trend: <0.0001, 0.014 and 0.001, respectively). Aside from these associations, 4-t-OP and 4-n-NP exposure was also associated with idiopathic male infertility with abnormal semen parameters. Moreover, we observed significant associations between sum alkylphenols (APs) exposure and idiopathic male infertility. There were no relationships between exposure to other phenols and idiopathic male infertility in the present study. Our study provides the first evidence that exposure to APs (4-t-OP, 4-n-OP and 4-n-NP) is associated with idiopathic male infertility. PMID:23435201

  19. 50 CFR Table 3c to Part 680 - Crab Product Codes for Economic Data Reports

    Code of Federal Regulations, 2011 CFR

    2011-10-01

    ... 50 Wildlife and Fisheries 11 2011-10-01 2011-10-01 false Crab Product Codes for Economic Data Reports 3c Table 3c to Part 680 Wildlife and Fisheries FISHERY CONSERVATION AND MANAGEMENT, NATIONAL... EXCLUSIVE ECONOMIC ZONE OFF ALASKA Pt. 680, Table 3c Table 3c to Part 680—Crab Product Codes for...

  20. 50 CFR Table 3c to Part 680 - Crab Product Codes for Economic Data Reports

    Code of Federal Regulations, 2012 CFR

    2012-10-01

    ... 50 Wildlife and Fisheries 13 2012-10-01 2012-10-01 false Crab Product Codes for Economic Data Reports 3c Table 3c to Part 680 Wildlife and Fisheries FISHERY CONSERVATION AND MANAGEMENT, NATIONAL... EXCLUSIVE ECONOMIC ZONE OFF ALASKA Pt. 680, Table 3c Table 3c to Part 680—Crab Product Codes for...

  1. 50 CFR Table 3c to Part 680 - Crab Product Codes for Economic Data Reports

    Code of Federal Regulations, 2010 CFR

    2010-10-01

    ... 50 Wildlife and Fisheries 9 2010-10-01 2010-10-01 false Crab Product Codes for Economic Data Reports 3c Table 3c to Part 680 Wildlife and Fisheries FISHERY CONSERVATION AND MANAGEMENT, NATIONAL... EXCLUSIVE ECONOMIC ZONE OFF ALASKA Pt. 680, Table 3c Table 3c to Part 680—Crab Product Codes for...

  2. Recurrent seizures, mental retardation and extensive brain calcinosis related to delayed diagnosis of hypoparathyroidism in an adolescent boy.

    PubMed

    Eom, Tae-Hoon; Kim, Young-Hoon; Kim, Jung-Min

    2015-05-01

    Reports of adolescent patients presenting with intractable seizures and mental retardation secondary to idiopathic hypothyroidism are uncommon in the literature. In this case, we report a 17-year-old boy who developed recurrent seizures, mental retardation and extensive brain calcinosis related to delayed diagnosis of hypoparathyroidism. Hypoparathyroidism can be easily missed in children and adolescents, and may lead to irreversible neurologic sequelae. This case highlights the need to consider hypocalcemia in any patient with uncontrolled seizures. PMID:25835143

  3. An advanced compliance monitor for patients undergoing brace treatment for idiopathic scoliosis.

    PubMed

    Chalmers, Eric; Lou, Edmond; Hill, Doug; Zhao, H Vicky

    2015-02-01

    Adolescent idiopathic scoliosis is a spinal deformity affecting 2-3% of adolescents. Brace treatment, the most common non-surgical treatment, uses a hard plastic orthotic shell to prevent progression of the deformity. Previous studies have found association between treatment outcome and patients' compliance with the prescribed brace-wear regimen. However, the exact relationship between compliance and treatment outcome has yet to be elucidated. Current compliance monitoring techniques may not be providing enough information about patients' brace-wear habits. Building on previous work, we present a new compliance monitor which records both temperature and force applied to the patient's body. The combination of temperature and force readings shows both how often and how tightly the brace is worn. The new monitor is designed for minimal size and power consumption, measuring 5.2 cm × 2.5 cm × 0.8 cm, with a battery life of approximately one year. Seven patients wore the monitor in this pilot study. The temperature-based compliance estimate differed significantly from the force-based estimate in four out of seven cases. This suggests that some patients may wear their braces very loosely, and that existing temperature-only or force-only compliance monitors may not be giving a complete picture of brace-wear habits. PMID:25619614

  4. Desloratadine for chronic idiopathic urticaria: a review of clinical efficacy.

    PubMed

    DuBuske, Lawrence

    2007-01-01

    Chronic idiopathic urticaria (CIU) is a disabling affliction that considerably limits patients' daily activities and interferes with sleep. Clinical studies have shown that histamine H1-receptor antagonists (antihistamines) are highly effective for inhibiting the hives/wheals and pruritus associated with CIU, as well as improving patients' quality of life. Desloratadine is a rapid-acting, once-daily, nonsedating selective H1-receptor antagonist/inverse receptor agonist with proven clinical efficacy in patients with CIU. It has 10-20 times the in vivo H1 receptor-binding affinity of loratadine, its parent compound, and 52-194 times the H1 receptor-binding affinity of cetirizine, ebastine, loratadine, and fexofenadine. Desloratadine displays linear pharmacokinetics after oral administration. Age and sex have no apparent effect on the drug's metabolism and elimination, and food does not affect its bioavailability or absorption. Desloratadine also exerts anti-inflammatory effects via mechanisms that are independent of H1-receptor antagonism. Results from randomized, double-blind, placebo-controlled studies of 6 weeks' duration in adults and adolescents with moderate-to-severe CIU indicate that desloratadine significantly minimizes the severity of pruritus, reduces the number and size of hives, and improves disease-impaired sleep and daily activities. Improvements were noted after a single dose of desloratadine and were maintained over 6 weeks of treatment. Desloratadine was safe and well tolerated in clinical trials of patients with CIU. The adverse effect profile of desloratadine in adults, as well as in children aged from 6 months to 11 years, is comparable to that of placebo. Evaluations of cognitive and psychomotor performance in adults indicate no impairment of function with dosages of desloratadine 5 mg/day. In conclusion, desloratadine is an important therapeutic option for prompt and enduring symptom relief in patients with moderate-to-severe CIU. In addition

  5. Impairment and Coping in Children and Adolescents with Chronic Fatigue Syndrome: A Comparative Study with Other Paediatric Disorders

    ERIC Educational Resources Information Center

    Garralda, M. Elena; Rangel, Luiza

    2004-01-01

    Background: Functional impairment is a key feature of chronic fatigue syndrome (CFS) of childhood. Aim: To compare impairment, illness attitudes and coping mechanisms in childhood CFS and in other paediatric disorders. Method: Participants were 28 children and adolescents with CFS, 30 with juvenile idiopathic arthritis (JIA) and 27 with emotional…

  6. Using Acceptance and Commitment Therapy in the Rehabilitation of an Adolescent Female with Chronic Pain: A Case Example

    ERIC Educational Resources Information Center

    Wicksell, Rikard K.; Dahl, JoAnne; Magnusson, Bo; Olsson, Gunnar L.

    2005-01-01

    This case example illustrates how Acceptance and Commitment Therapy (ACT) within a behavior medicine approach was used in the rehabilitation of an adolescent with debilitating chronic pain. For chronic pain with unclear etiology (idiopathic pain), pharmacological therapy alone is often insufficient. Psychological treatment strategies have been…

  7. Surgical treatment analysis of idiopathic esophageal achalasia

    PubMed Central

    de AQUINO, José Luis Braga; SAID, Marcelo Manzano; PEREIRA, Douglas Rizzanti; do AMARAL, Paula Casals; LIMA, Juliana Carolina Alves; LEANDRO-MERHI, Vânia Aparecida

    2015-01-01

    Background Idiopathic esophageal achalasia is an inflammatory disease of unknown origin, characterized by aperistalsis of the esophageal body and failure of the lower esophageal sphincter in response to swallowing, with consequent dysphagia. Aim To demonstrate the results of surgical therapy in these patients, evaluating the occurred local and systemic complications. Methods Were studied retrospectively 32 patients, 22 of whom presented non-advanced stage of the disease (Stage I/II) and 10 with advanced disease (Stage III/IV). All of them had the clinical conditions to be submitted to surgery. The diagnoses were done by clinical, endoscopic, cardiological, radiological and esophageal manometry analysis. Pre-surgical evaluation was done with a questionnaire based on the most predisposing factors in the development of the disease and the surgical indication was based on the stage of the disease. Results The patients with non-advanced stages were submitted to cardiomyotomy with fundoplication, wherein in the post-surgical early assessment, only one (4,4%) presented pulmonary infection, but had a good outcome. In patients with advanced disease, seven were submitted to esophageal mucosectomy preserving the muscular layer, wherein one patient (14,2%) presented dehiscence of gastric cervical esophagus anastomosis as well as pulmonary infection; all of these complications were resolved with proper specific treatment; the other three patients with advanced stage were submitted to transmediastinal esophagectomy; two of them presented hydropneumothorax with good evolution, and one of them also presented fistula of the cervical esophagogastric anastomosis, but with spontaneous healing after conservative treatment and nutritional support. The two patients with fistula of the cervical anastomosis progressed to stenosis, with good results after endoscopic dilations. In the medium and long term assessment done in 23 patients, all of them reported improvement in life quality, with

  8. Organic functionalization of 3C-SiC surfaces.

    PubMed

    Schoell, Sebastian J; Sachsenhauser, Matthias; Oliveros, Alexandra; Howgate, John; Stutzmann, Martin; Brandt, Martin S; Frewin, Christopher L; Saddow, Stephen E; Sharp, Ian D

    2013-02-01

    We demonstrate the functionalization of n-type (100) and (111) 3C-SiC surfaces with organosilanes. Self-assembled monolayers (SAMs) of amino-propyldiethoxymethylsilane (APDEMS) and octadecyltrimethoxysilane (ODTMS) are formed via wet chemical processing techniques. Their structural, chemical, and electrical properties are investigated using static water contact angle measurements, atomic force microscopy, and X-ray photoelectron spectroscopy, revealing that the organic layers are smooth and densely packed. Furthermore, combined contact potential difference and surface photovoltage measurements demonstrate that the heterostructure functionality and surface potential can be tuned by utilizing different organosilane precursor molecules. Molecular dipoles are observed to significantly affect the work functions of the modified surfaces. Furthermore, the magnitude of the surface band bending is reduced following reaction of the hydroxylated surfaces with organosilanes, indicating that partial passivation of electrically active surface states is achieved. Micropatterning of organic layers is demonstrated by lithographically defined oxidation of organosilane-derived monolayers in an oxygen plasma, followed by visualization of resulting changes of the local wettability, as well as fluorescence microscopy following immobilization of fluorescently labeled BSA protein. PMID:23357505

  9. Effluent sampling of Titan 3 C vehicle exhaust

    NASA Technical Reports Server (NTRS)

    Gregory, G. L.; Storey, R. W., Jr.

    1975-01-01

    Downwind in situ ground-level measurements of the exhaust from a Titan 3 C launch vehicle were made during a normal launch. The measurement activity was conducted as part of an overall program to obtain field data for comparison with the multilayer dispersion model currently being used to predict the behavior of rocket vehicle exhaust clouds. All measurements were confined to land, ranging from the launch pad to approximately 2 kilometers downwind from the pad. Measurement systems included detectors for hydrogen chloride (HCl), carbon dioxide (CO2), and particulates (Al2O3). Airborne and ground-based optical systems were employed to monitor exhaust cloud rise, growth, and movement. These measurement systems, located along the ground track (45 deg azimuth from the launch pad) of the exhaust cloud, showed no effluents attributable to the launch. Some hydrogen chloride and aluminum oxide were detected in the surface wind direction (15 deg azimuth) from the pad. Comparisons with the model were made in three areas: (1) assumption of cloud geometry at stabilization; (2) prediction of cloud stabilization altitude; and (3) prediction of the path of cloud travel. In addition, the importance of elemental analyses of the particulate samples is illustrated.

  10. Not all chronic urticaria is "idiopathic"!

    PubMed

    Greaves, M W; O'Donnell, B F

    1998-02-01

    The aetiology of chronic urticaria in the majority of patients is elusive. The cause of physical urticarias (dermographism, delayed pressure urticaria, cold urticaria) is unknown. We have identified a subset of chronic "idiopathic" urticaria patients, representing approximately 30% of the total in which the disease is caused by the presence of IgG autoantibodies against the high affinity IgE receptor (FcepsilonRIalpha). This functional autoantibody stimulates normal (albeit vicarious) activation of mast cells and basophils via FcepsilonRI, causing whealing and angioedema. Anti-FcepsilonRI-positive patients are recognized by a combination of autologous serum skin testing and evoked histamine release from basophils of normal human donors. Autoantibody-positive and -negative patients are clinically indistinguishable and are treated routinely by combinations of H1 and occasionally H2-antihistamines in both cases. However, severely affected patients who are anti-FcepsilonRI-positive can also be treated by non-specific immunotherapy (plasma pheresis, intravenous immunoglobulin, cyclosporin. PMID:9517916

  11. Chronic idiopathic urticaria and Graves' disease.

    PubMed

    Ruggeri, R M; Imbesi, S; Saitta, S; Campennì, A; Cannavò, S; Trimarchi, F; Gangemi, S

    2013-01-01

    Chronic urticaria is a common condition characterized by recurrent episodes of mast cell-driven wheal and flare-type skin reactions lasting for more than 6 weeks. In about 75% of cases, the underlying causes remain unknown, and the term chronic idiopathic urticaria (CIU) is used to emphasize that wheals develop independently of identified external stimuli. Although CIU affects about 1.0% of the general population, its etiopathogenesis is not yet well understood. It is now widely accepted that in many cases CIU should be regarded as an autoimmune disorder caused by circulating and functionally active IgG autoantibodies specific for the IgE receptor (FceRI) present on mast cells and basophils or for IgE itself. The well-known association of CIU with other autoimmune processes/diseases represents further indirect evidence of its autoimmune origin. Autoimmune thyroid diseases, especially autoimmune thyroiditis, represent the most frequently investigated diseases in association with CIU. Here we review this topic with particular regard to the association between Graves' disease and CIU. The possible pathogenetic mechanisms and the clinical implications of such an association are discussed. PMID:23609949

  12. Idiopathic hypersomnia in an aircrew member.

    PubMed

    Withers, B G; Loube, D I; Husak, J P

    1999-08-01

    In aviation, it is essential that all aircrew members remain alert and contribute, by their observations and actions, to flight safety. Especially in helicopter operations, crewmembers riding in the rear of the aircraft play an integral role in many aspects of flight, such as take-offs, landings, turns, formation flights, hazard avoidance, situational awareness, military operations, and crew coordination. We present the case of a helicopter crew chief with idiopathic hypersomnia, briefly review the disorder, and give the recent U.S. military aviation experience with sleep disorders. Flight surgeons and aeromedical examiners should be active in considering and diagnosing sleep-related disorders as the aviator or crewmember may not be aware of the disease or may not volunteer the history. A directed history is important in making the diagnosis, as are reports from family and other aircrew members. Referral to a sleep specialist is required in performing objective sleep studies, establishing the diagnosis, recommending treatment, and providing a prognosis. Many sleep disorders are treatable and aeromedically waiverable. PMID:10447054

  13. [Glycosaminoglycans dysbolism in pathogenesis of idiopathic varicocele].

    PubMed

    Shenberger, A B; Bashkatov, S A; Parfenova, T I; Sufiiarova, R Sh

    2008-01-01

    Ultrasound dopplerography (UD) was used to examine 50 patients with idiopathic varicocele (IV) and 20 healthy subjects. Such parameters were assessed as age, severity of IV, its duration, scrotal vessels UD data, vein diameter of the pampiniform plexus on the left, reflux at the height of the Valsalva test, uronic acids (UA), glycosaminoglycanes (GAG) and their fractions (hialuronic acid--HA, chondroitin sulphates--CS, heparansulphates--HS). UA and GAG fraction composition was studied with ion exchange chromatography. Calculations were made of mean values, standard deviations, Student's t-criterion, Pirson's correlation coefficient. It was found that HA and UA concentrations were high. In young patients HA metabolism was disturbed more, in adult patients--disturbance of UA metabolism. Two equal negative moderately strong links were revealed between the patients' age, HS and total GAG: r1 = r2 = -0.56; CI of UA in the patients--21.97-59.09 mcg/ml, in the control group --23.70- 43.42 mcg/ml. With age, the organism adapts to the disease and spontaneous regress may occur. In UA concentration in the serum above 43.42 mcg/ml surgical treatment is indicated, if it is lower--conservative treatment and follow-up. PMID:19069496

  14. Diagnosis and classification of idiopathic inflammatory myopathies.

    PubMed

    Lundberg, I E; Miller, F W; Tjärnlund, A; Bottai, M

    2016-07-01

    The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of diseases, collectively termed myositis, sharing symptoms of muscle weakness, fatigue and inflammation. Other organs are frequently involved, supporting the notion that these are systemic inflammatory diseases. The IIMs can be subgrouped into dermatomyositis, polymyositis and inclusion body myositis. The myositis-specific autoantibodies (MSAs) identify other and often more distinct clinical phenotypes, such as the antisynthetase syndrome with antisynthetase autoantibodies and frequent interstitial lung disease and anti-SRP and anti-HMGCR autoantibodies that identify necrotizing myopathy. The MSAs are important both to support myositis diagnosis and to identify subgroups with different patterns of extramuscular organ involvement such as interstitial lung disease. Another cornerstone in the diagnostic procedure is muscle biopsy to identify inflammation and to exclude noninflammatory myopathies. Treatment effect and prognosis vary by subgroup. To develop new and better therapies, validated classification criteria that identify distinct subgroups of myositis are critical. The lack of such criteria was the main rationale for the development of new classification criteria for IIMs, which are summarized in this review; the historical background regarding previous diagnostic and classification criteria is also reviewed. As the IIMs are rare diseases with a prevalence of 10 in 100 000 individuals, an international collaboration was essential, as was the interdisciplinary effort including experts in adult and paediatric rheumatology, neurology, dermatology and epidemiology. The new criteria have been developed based on data from more than 1500 patients from 47 centres worldwide and are based on clinically easily available variables. PMID:27320359

  15. Lymphoedema caused by idiopathic lymphatic thrombus.

    PubMed

    Hara, Hisako; Mihara, Makoto; Seki, Yukio; Koshima, Isao

    2013-12-01

    Primary lymphoedema includes some diseases whose genetic anomaly is detected and others whose pathology is unknown. In this article, we report a lymphatic thrombus found in a limb with lymphoedema during lymphatico-venous anastomosis (LVA). A 32-year-old man was aware of oedema in his left calcar pedis 3 years previously, which appeared without any trigger. Indocyanine green lymphography indicated lymphatic stasis in the left calf and thigh region, and we performed LVA for the patient. During the operation, we found yellow vessels, which were thought to be lymphatic vessels filled with a yellow solid substance, just beneath the superficial fascia at the left ankle. Pathological examination of the thrombi revealed hyaline material mixed with cell components. The cells were categorised as lymphatic endothelial cells, as they were positive for podoplanin. There was no evidence of malignancy. Causes of idiopathic lymphatic thrombus such as this may be one of the causes of so-called primary lymphoedema, and evaluation of such cases may be the first step towards elucidating the mechanisms involved in the development of primary lymphoedema. PMID:23643778

  16. Idiopathic Granulomatous Hypophysitis Mimicking Pituitary Abscess

    PubMed Central

    Kong, Xiangyi; Wang, Renzhi; Yang, Yi; Wu, Huanwen; Su, Changbao; Ma, Wenbin; Li, Yongning; Xing, Bing; Lian, Wei; Xu, Zhiqin; Yao, Yong; Ren, Zuyuan

    2015-01-01

    Abstract Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary that commonly presents with enlargement of the pituitary gland. Clinically and radiologically, IGH is a rare sellar entity easily to be misdiagnosed as a pituitary adenoma. Through such a case, we aim to present this rarity and emphasize the importance to correctly diagnose confusing pituitary lesions comprehensively by clinical presentations, radiological signs, and biopsy. We present an uncommon case of IGH in a 19-year-old man. The patient was admitted to the hospital with severe headache, vomiting, and vision's sharp decline. Magnetic resonance imaging showed a sellar lesion with obvious cystic change and ring enhancement. The disease course including diagnosis and treatment was presented and analyzed in detail. The pertinent literature is reviewed regarding this uncommon entity. The patient underwent surgical exploration and partial resection via the transsphenoidal approach. The pathologic findings suggested IGH giving no significant evidences of systemic granulomatous disease and venereal disease. Large dose methylprednisolone was then used. The pituitary function recovered, but there was no apparent improvement of his vision. IGH is a rarely occurred inflammatory disease of unknown etiology. It is difficult to diagnose preoperatively and is often misdiagnosed. Although rare, IGH should be kept in mind in terms of differential diagnosis of sellar region lesions. PMID:26181544

  17. [Juvenile idiopathic arthritis and oral health].

    PubMed

    Kobus, Agnieszka; Kierklo, Anna; Sielicka, Danuta; Szajda, Sławomir Dariusz

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child's health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity. The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health. PMID:27180959

  18. The treatment of idiopathic pulmonary fibrosis.

    PubMed

    Woodcock, Hannah V; Maher, Toby M

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive and invariably fatal disease with a median survival of less than three years from diagnosis. The last decade has seen an exponential increase in clinical trial activity in IPF and this in turn has led to important developments in the treatment of this terrible disease. Previous therapeutic approaches based around regimens including corticosteroids and azathioprine have, when tested in randomized clinical trials, been shown to be harmful in IPF. By contrast, compounds with anti-fibrotic actions have been shown to be beneficial. Subsequently, the novel anti-fibrotic agent pirfenidone has, in many parts of the world, become the first treatment ever to be licensed for use in IPF. This exciting development, coupled with ongoing clinical trials of a range of other novel compounds, is bringing hope to patients and their clinicians and raises the prospect that, in the future, it may become possible to successfully arrest the development of progressive scarring in IPF. PMID:24669297

  19. Molecular mechanisms in progressive idiopathic pulmonary fibrosis.

    PubMed

    Steele, Mark P; Schwartz, David A

    2013-01-01

    There is clear evidence that environmental exposures and genetic predisposition contribute to the pathogenesis of idiopathic pulmonary fibrosis (IPF). Cigarette smoking increases the risk of developing IPF several-fold, as do other exposures such as metal-fume and wood-dust exposure. Occupations that increase the risk of IPF are agricultural work, hairdressing, and stone polishing, supporting the role of environmental exposure in disease pathogenesis. Genetic predisposition to IPF is evident from its familial aggregation and the fact that pulmonary fibrosis develops in several rare genetic disorders. Mutations in surfactant proteins lead to pulmonary fibrosis and are associated with endoplasmic reticulum stress in alveolar type II epithelial cells. Mutations in telomerase have been found in several families with IPF, and shortened telomeres are found in sporadic cases of IPF. A common variant in mucin 5B predisposes to both familial and sporadic IPF and is present in the majority of cases, indicating sporadic IPF occurs in those with genetic predisposition. PMID:23020878

  20. The laboratory profile in idiopathic intracranial hypertension.

    PubMed

    Pollak, Lea; Zohar, Efrat; Glovinsky, Yoseph; Huna-Baron, Ruth

    2015-07-01

    While overweight and female gender play an undisputable role in the pathogenesis of idiopathic intracranial hypertension (IIH), the contribution of other factors is still unclear. We have evaluated the laboratory findings of patients with IIH in an attempt to find the influence of abnormalities on the disease course. Included were 82 females after menarche and males older than 18 years who were followed up for at least 1 year. A wide range of laboratory parameters were examined at the time of presentation. The most frequent abnormal laboratory findings were elevated C reactive protein (CRP) (51 %), thrombophilia (31 %), increased plasma cortisol levels (29 %) and elevated lactate dehydrogenase (LDH) (20 %). Patients with elevated CRP and patients with thrombophilia had an unfavorable visual outcome. Increased cortisol levels and abnormal calcium correlated with a higher rate of recurrence. The visual outcome of patients with elevated LDH was better than those with normal LDH. It seems that certain metabolic, inflammatory and coagulation abnormalities may influence the course of IIH. If confirmed in further studies, these findings could contribute to elucidation of the etiology and prognosis of IIH. PMID:25596710

  1. 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.

  2. Trigeminal neuralgia and persistent idiopathic facial pain.

    PubMed

    Obermann, Mark; Holle, Dagny; Katsarava, Zaza

    2011-11-01

    Trigeminal neuralgia (TN) and persistent idiopathic facial pain (PIFP) are two of the most puzzling orofacial pain conditions and affected patients are often very difficult to treat. TN is characterized by paroxysms of brief but severe pain followed by asymptomatic periods without pain. In some patients a constant dull background pain may persist. This constant dull pain sometimes makes the distinction from PIFP difficult. PIFP is defined as continuous facial pain, typically localized in a circumscribed area of the face, which is not accompanied by any neurological or other lesion identified by clinical examination or clinical investigations. The pain usually does not stay within the usual anatomic boundaries of the trigeminal nerve distribution and is a diagnosis of exclusion. Epidemiologic evidence on TN, and even more so on PIFP, is quite scarce, but generally both conditions are considered to be rare diseases. The etiology and underlying pathophysiology of TN, and more so PIFP, remain unknown. Treatment is based on only few randomized controlled clinical trials and insufficiently evaluated surgical procedures. PMID:22014140

  3. Prucalopride: A Review in Chronic Idiopathic Constipation.

    PubMed

    Garnock-Jones, Karly P

    2016-01-01

    Prucalopride (Resolor®), a highly selective serotonin 5-HT4 receptor agonist, is indicated in the European Economic Area for the treatment of adults with chronic idiopathic constipation (CIC) in whom laxatives have failed to provide adequate relief. This article reviews the pharmacological properties of prucalopride and its clinical efficacy and tolerability in patients with CIC. In five well-designed, 12-week trials in patients with CIC, oral prucalopride 2 mg/day was significantly more effective than placebo at improving bowel function, including the number of bowel movements and a range of other constipation symptoms, as well as health-related quality of life and patient satisfaction; however, no significant differences in bowel function measures were observed between prucalopride and placebo in a 24-week trial. Oral PEG-3350 + electrolytes reconstituted powder was found to be noninferior but not superior to prucalopride according to primary endpoint data from a 4-week, controlled-environment trial. Prucalopride was generally well tolerated in clinical trials; the most common adverse events were headache, diarrhoea, nausea and abdominal pain. No cardiovascular safety issues have arisen with prucalopride treatment. Although further long-term and comparative data would be beneficial, prucalopride provides an additional treatment option for patients with CIC. PMID:26628294

  4. Macrophages - silent enemies in juvenile idiopathic arthritis.

    PubMed

    Świdrowska-Jaros, Joanna; Orczyk, Krzysztof; Smolewska, Elżbieta

    2016-01-01

    The inflammatory response by secretion of cytokines and other mediators is postulated as one of the most significant factors in the pathophysiology of juvenile idiopathic arthritis (JIA). The effect of macrophage action depends on the type of their activation. Classically activated macrophages (M1) are responsible for release of molecules crucial for joint inflammation. Alternatively activated macrophages (M2) may recognize self antigens by scavenger receptors and induce the immunological reaction leading to autoimmune diseases such as JIA. Molecules essential for JIA pathophysiology include: TNF-α, the production of which precedes synovial inflammation in rheumatoid arthritis; IL-1 as a key mediator of synovial damage; chemotactic factors for macrophages IL-8 and MCP-1; IL6, the level of which correlates with the radiological joint damage; MIF, promoting the secretion of TNF-α and IL-6; CCL20 and HIF, significant for the hypoxic synovial environment in JIA; GM-CSF, stimulating the production of macrophages; and IL-18, crucial for NK cell functions. Recognition of the role of macrophages creates the potential for a new therapeutic approach. PMID:27383571

  5. PROFILEing idiopathic pulmonary fibrosis: rethinking biomarker discovery.

    PubMed

    Maher, Toby M

    2013-06-01

    Despite major advances in the understanding of the pathogenesis of idiopathic pulmonary fibrosis (IPF), diagnosis and management of the condition continue to pose significant challenges. Clinical management of IPF remains unsatisfactory due to limited availability of effective drug therapies, a lack of accurate indicators of disease progression, and an absence of simple short-term measures of therapeutic response. The identification of more accurate predictors of prognosis and survival in IPF would facilitate counseling of patients and their families, aid communication among clinicians, and would guide optimal timing of referral for transplantation. Improvements in molecular techniques have led to the identification of new disease pathways and a more targeted approach to the development of novel anti-fibrotic agents. However, despite an increased interest in biomarkers of IPF disease progression there are a lack of measures that can be used in early phase clinical trials. Careful longitudinal phenotyping of individuals with IPF together with the application of novel omics-based technology should provide important insights into disease pathogenesis and should address some of the major issues holding back drug development in IPF. The PROFILE (Prospective Observation of Fibrosis in the Lung Clinical Endpoints) study is a currently enrolling, prospective cohort study designed to tackle these issues. PMID:23728868

  6. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome.

    PubMed

    Rossi, Giulio; Cavazza, Alberto; Spagnolo, Paolo; Sverzellati, Nicola; Longo, Lucia; Jukna, Agita; Montanari, Gloria; Carbonelli, Cristiano; Vincenzi, Giada; Bogina, Giuseppe; Franco, Renato; Tiseo, Marcello; Cottin, Vincent; Colby, Thomas V

    2016-06-01

    The term diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) may be used to describe a clinico-pathological syndrome, as well as an incidental finding on histological examination, although there are obvious differences between these two scenarios. According to the World Health Organization, the definition of DIPNECH is purely histological. However, DIPNECH encompasses symptomatic patients with airway disease, as well as asymptomatic patients with neuroendocrine cell hyperplasia associated with multiple tumourlets/carcinoid tumours. DIPNECH is also considered a pre-neoplastic lesion in the spectrum of pulmonary neuroendocrine tumours, because it is commonly found in patients with peripheral carcinoid tumours.In this review, we summarise clinical, physiological, radiological and histological features of DIPNECH and critically discuss recently proposed diagnostic criteria. In addition, we propose that the term "DIPNECH syndrome" be used to indicate a sufficiently distinct patient subgroup characterised by respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping on chest imaging and constrictive obliterative bronchiolitis, often with nodular proliferation of neuroendocrine cells with/without tumourlets/carcinoid tumours on histology. Surgical lung biopsy is the diagnostic gold standard. However, in the appropriate clinical and radiological setting, transbronchial lung biopsy may also allow a confident diagnosis of DIPNECH syndrome. PMID:27076588

  7. Mineralogical microanalysis of idiopathic pulmonary fibrosis

    SciTech Connect

    Monso, E.; Tura, J.M.; Marsal, M.; Morell, F.; Pujadas, J.; Morera, J. )

    1990-05-01

    A mineralogical analysis of lung tissue was conducted on 25 samples from patients who had been diagnosed as having idiopathic pulmonary fibrosis (IPF). Scanning electron microscopy (SEM) at low magnification and energy-dispersive x-ray analysis (EDXA) was used. In all samples, the surface silicon/sulfur (Si/S) ratio was calculated. The Si/S ratio for 25 samples of normal lung and 6 samples of pneumoconiotic lung was also determined (upper limit of normal Si/S ratio = 0.3). The difference between the Si/S ratio in the group with IPF and group with normal lung tissue was significantly significant (p less than .007, Wilcoxon test). Six of 12 patients with a previous diagnosis of IPF and a Si/S ratio greater than 0.3 had an exposure history that could imply inhalation of silica/silicates, and the correct diagnosis for these patients is most probably pneumoconiosis. The silica/silicate deposits detected in patients with IPF, and who had a ratio and no past exposure to dusts, could be either a cause or an effect of the disease.

  8. Management of Children with Juvenile Idiopathic Arthritis.

    PubMed

    Viswanathan, Vijay; Murray, Kevin J

    2016-01-01

    Juvenile idiopathic arthritis (JIA) comprises a group of heterogeneous disorders of chronic arthritis in childhood and remains the commonest pediatric rheumatic disease associated with significant long-term morbidity. Advances in understanding of the pathogenesis, better definition of disease control/remission measures, and the arrival of biological agents have improved the outcomes remarkably. Methotrexate (Mtx) remains the first-line disease modifying (DMARD) therapy for most children with JIA due to its proven efficacy and safety. Sulphosalazine (SSz) (especially for enthesitis) and leflunomide may also have a secondary role. Tumor necrosis factor inhibitors (TNF-I), alone or in combination with Mtx have shown tremendous benefit in children with polyarticular JIA, enthesitis related arthritis (ERA) and psoriatic arthritis. Tocilizumab appears very efficacious in systemic arthritis and abatacept and tocilizumab also appear to benefit polyarticular JIA; the role of rituximab remains unclear, though clearly beneficial in adult RA. TNF-I with Mtx is also effective in uveitis associated with JIA. Biologicals have demonstrated an impressive safety record in children with JIA, although close monitoring for rare but potentially dangerous adverse events, such as tuberculosis and other infections; paradoxical development of additional autoimmune diseases; and possibly an increased risk of cancers is warranted. PMID:26639461

  9. Urinary neopterin in idiopathic retinal vasculitis

    PubMed Central

    Palmer, H; Giovannoni, G; Stanford, M; Wallace, G; Graham, E

    2001-01-01

    AIMS—To determine whether urinary neopterin:creatinine (UNC) ratios relate to disease activity in idiopathic retinal vasculitis (RV).
METHODS—18 patients with RV were prospectively recruited into a year long longitudinal study. Patients collected first morning urine samples on a weekly basis and on the same day completed a diary which documented their subjective view of RV activity and any concurrent infection. They were examined in clinic on a 6-8 weekly basis and an objective assessment was made of RV disease activity. 14 healthy controls collected urine samples in the same way.
RESULTS—UNC ratios were significantly higher in patients than in controls (p=0.004, Mann-Whitney U test). UNC ratios were significantly higher when, according to their diaries, the patients had a subjective flare up of RV (p=0.001, Mann-Whitney U test). Subjective increased RV activity occurred more often when the patients had a concurrent infection (p<0.0001, χ2 test). There was no significant difference in the UNC ratio between objective clinical relapse and non-relapse of RV. There was moderate agreement between the clinical assessment and patients' subjective impression of RV activity (κ=0.48).
CONCLUSIONS—Higher neopterin levels reflect cell mediated disease that occurs in RV, but UNC ratios are not recommended as a means of monitoring clinical disease activity in RV.

 PMID:11133708

  10. Idiopathic Granulomatous Mastitis Associated with Erythema Nodosum

    PubMed Central

    Kalaycı, Tuğçe Özlem; Koruyucu, Melike Bedel; Apaydın, Melda; Etit, Demet; Varer, Makbule

    2016-01-01

    Background: Idiopathic granulomatous mastitis (IGM) is an uncommon benign chronic inflammatory breast disease, and erythema nodosum (EN) is an extremely rare systemic manifestation of IGM. Here, we report a rare case of IGM accompanied by EN. Case Report: A 32-year-old patient was admitted to our clinic with a history of a tender mass in the right breast. On physical examination, the right breast contained a hard, tender mass in the lower half with indrawing of the nipple. She had florid EN affecting both legs. She was evaluated with mammography, ultrasound, power Doppler ultrasound, non-enhancing magnetic resonance imaging (MRI), dynamic contrast-enhanced MRI, fine needle aspiration biopsy (FNAB) and excisional biopsy. Time-intensity curves showed a type II pattern on dynamic contrast-enhanced MRI, which has an intermediate probability for malignancy. The FNAB reported a benign cytology suggestive of a granulomatous inflammation, which was also supported by the histopathological findings. A partial mastectomy was performed following medical treatment. There was no recurrence at 1-year follow-up. Conclusion: IGM should be considered in the differential diagnosis of EN. Although histopathological examination remains the only method for the definite diagnosis of IGM, MRI can be helpful in the diagnosis or differentiation of benign lesions from malignant ones. PMID:27403395

  11. Idiopathic Inflammatory Myopathies: Clinical Approach and Management

    PubMed Central

    Malik, Asma; Hayat, Ghazala; Kalia, Junaid S.; Guzman, Miguel A.

    2016-01-01

    Idiopathic inflammatory myopathies (IIM) are a group of chronic, autoimmune conditions affecting primarily the proximal muscles. The most common types are dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with sub-acute to chronic onset of proximal weakness manifested by difficulty with rising from a chair, climbing stairs, lifting objects, and combing hair. They are uniquely identified by their clinical presentation consisting of muscular and extramuscular manifestations. Laboratory investigations, including increased serum creatine kinase (CK) and myositis specific antibodies (MSA) may help in differentiating clinical phenotype and to confirm the diagnosis. However, muscle biopsy remains the gold standard for diagnosis. These disorders are potentially treatable with proper diagnosis and initiation of therapy. Goals of treatment are to eliminate inflammation, restore muscle performance, reduce morbidity, and improve quality of life. This review aims to provide a basic diagnostic approach to patients with suspected IIM, summarize current therapeutic strategies, and provide an insight into future prospective therapies. PMID:27242652

  12. [Idiopathic Progressive Subglottic Stenosis: Surgical Techniques].

    PubMed

    Hoetzenecker, K; Schweiger, T; Klepetko, W

    2016-09-01

    Idiopathic subglottic stenosis is a disease characterized by slow, progressive scarring and constriction of the subglottic airway. It almost always occurs in females between the 3rd and 5th decade of life. Symptoms are frequently misinterpreted as asthma and patients are referred for endoscopic evaluation only when asthma medications fail to alleviate their symptoms. Treatment options can be divided into endoscopic and open surgical techniques. Microlaryngoscopic scar reduction by laser followed by balloon dilation usually delivers good short-term results. However, the majority of patients will experience restenosis within a short period of time. Open surgical correction techniques are based on a complete removal of the affected airway segment. This must be combined with various extended resection techniques in patients with advanced stenosis. Depending on the extent and severity of the stenosis the following surgical techniques are required: standard cricotracheal resection (Grillo's technique), cricoplasty with dorsal and lateral mucosaplasty, or a combination of resection and enlargement techniques using rib cartilage grafts. In experienced centres, success rates of over 95 % are reported with good functional outcome of voice and deglutition. PMID:27607884

  13. Psychological functioning in idiopathic short stature.

    PubMed

    Noeker, Meinolf

    2011-01-01

    Living with idiopathic short stature (ISS) may entail significant risks to psychological functioning and quality of life. Apparent inconsistency among study findings can be resolved if methodological differences among study designs are taken into account (i.e., definition of particular endpoints, sample selection from clinic or population, source of report, specific or generic assessment instruments, statistical control of confounders). Some individuals fail and others succeed in mastering the challenges of ISS. The principles of multifinality and equifinality may explain the emergence of a broad variation of individuals with ISS as a result of an interaction of the individual medical and auxological features on the one side, and psychosocial risk and protective factors on the other. As a result, patients may show heterogeneous developmental outcomes ranging from clinical psychopathology to development of resilience. A taxonomy of four distinct pathways of adaptation to ISS is delineated as a basis for case formulation and treatment planning. Psychological intervention in ISS includes counseling, cognitive-behavioral therapy and assertiveness training to improve psychological functioning via enhancement of target coping behaviors for critical situations. PMID:21912169

  14. Obesity in childhood and adolescence, genetic factors.

    PubMed

    Memedi, Rexhep; Tasic, Velibor; Nikolic, Erieta; Jancevska, Aleksandra; Gucev, Zoran

    2013-01-01

    Obesity and overweight are a pandemic phenomenon in the modern world. Childhood and adolescent obesity often ends up in obesity in adults. The costs of obesity and its consequences are staggering for any society, crippling for countries in development. The etiology is complex, but most often idiopathic. Hormonal, syndromic and medication-induced obesity are well investigated. Genetic causes are increasingly described. Novel technologies such as whole exome sequencing identify ever more candidate genes influencing or causing obesity. All insights into the complex problem of obesity in a team approach to treatment: diet, psychology, medications and surgery. We briefly review epidemiology, etiology, consequences and treatment approaches in childhood and adolescent obesity, with special emphasis on emerging knowledge of its genetics. PMID:24296635

  15. Observations of Titan 3C-4 Transtage Fragmentation Debris

    NASA Technical Reports Server (NTRS)

    Cowardin, Heather; Seitzer, P.; Abercromby, K.; Barker, E.; Cardona, T.; Krisko, P.; Lederer, S.

    2013-01-01

    The fragmentation of a Titan 3C-4 Transtage (1968-081) on 21 February 1992 is one of only two known break-ups in or near geosynchronous orbit. The original rocket body and 24 pieces of debris are currently being tracked by the US Space Surveillance Network (SSN). The rocket body (SSN# 3432) and several of the original fragments (SSN# 25000, 25001, 30000, and 33511) were observed in survey mode during 2004-2010 using the 0.6-m Michigan Orbital DEbris Survey Telescope (MODEST) in Chile using a broad R filter. This paper will present a size distribution for all calibrated magnitude data acquired on MODEST. Size distribution plots will also be shown using historical models for small fragmentation debris (down to 10 cm) believed to be associated with the Titan break-up. In November 2010, visible broadband photometry (Johnson/Kron-Cousins BVRI) was acquired with the 0.9-m Small and Moderate Aperture Research Telescope System (SMARTS) at the Cerro Tololo Inter-American Observatory (CTIO) in Chile on several Titan fragments (SSN# 25001, 33509, 33510) and the parent rocket body. Color index data will be used to determine the fragment brightness distribution and how the data compares to spacecraft materials measured in the laboratory using similar photometric measurement techniques. In 2012, the SSN added 16 additional fragments to the catalogue. MODEST acquired magnitude data on ten Titan fragments in late 2012 and early 2013. The magnitude distribution of all the observed fragments are analyzed as a function of time. In order to better characterize the breakup fragments spectral measurements were acquired on the original rocket body and five Titan fragments using the 6.5-m Magellan telescopes at Las Campanas Observatory in Chile. The telescopic spectra are compared with laboratory acquired spectra of materials (e.g., Aluminum and various paints) and categorized based on known absorption features for spacecraft materials.

  16. Factors associated with behavioral problems in children with idiopathic epilepsy.

    PubMed

    Dafoulis, Vaios; Kalyva, Efrosini

    2012-06-01

    The present study examined whether the perceived behavioral problems of children with idiopathic epilepsy differed from those of healthy controls according to parent proxy-reports and which factors are associated with these problems. The parents of 106 children with idiopathic epilepsy and 305 healthy controls aged 6-9 years old completed the Vanderbilt ADHD Diagnostic Parent Rating Scale and the Strengths and Difficulties Questionnaire. The 106 children with idiopathic epilepsy were also interviewed using the K-SADS-PL. The parents of children with idiopathic epilepsy reported more hyperactivity, emotional and conduct problems than the parents of healthy controls, as well as less prosocial behavior. Parents detected no differences in peer problems, inattention, oppositional/defiant disorder, and anxiety/depression. Age of onset of epilepsy (later), the number of administered antiepileptic drugs (polytherapy), and gender (male) predicted behavioral problems in children with idiopathic epilepsy. The frequency of seizures was associated with behavioral problems, while age was not. Finally, children with benign focal epilepsy were rated by their parents as having less behavioral problems than children with generalized epilepsy. PMID:22348790

  17. Prevalence of Celiac Disease in Turkish Children with Idiopathic Epilepsy

    PubMed Central

    Işikay, Sedat; Hizli, Şamil; Yilmaz, Kutluhan

    2014-01-01

    Objective: This study has examined the prevalence of celiac disease in Turkish children with idiopathic epilepsy. Methods: Children with idiopathic epilepsy were screened for celiac disease using the IgA anti-tissue transglutaminase antibody and compared with the healthy control group in order to find the association of celiac disease (CD) with idiopathic epilepsy. Upper gastrointestinal endoscopy and small intestinal biopsies were offered to all antibody-positive patients. Findings : A total of 214 children with the diagnosis of idiopathic epilepsy and 166 healthy children as control group were studied. Of the patients recruited, 55.1% had generalized epilepsy, and 44.9% had partial epilepsy. In 33 patients with partial epilepsy, electroclinical features were consistent with a diagnosis of childhood partial epilepsy with occipital paroxysms (CPEO). Two of 33 patients with CPEO had positive IgA anti-tissue transglutaminase antibodies in serology. Pathological examination of small intestinal biopsy specimens showed total villous atrophy in both of them. The prevalence of celiac disease among children with idiopathic epilepsy and CPEO was 0.9% and 6%, respectively. Conclusion: The results of the present study revealed that prevalence of CD is increased in children with epilepsy. On the other hand, as high as 6% prevalence of CD among patients with CPEO found in this study should be kept in mind and the clinicians should be aware of this association. PMID:25562021

  18. Idiopathic Nephrotic Syndrome and Atopy: Is There a Common Link?

    PubMed Central

    Abdel-Hafez, Maher; Shimada, Michiko; Lee, Pui Y.; Johnson, Richard J.; Garin, Eduardo H.

    2010-01-01

    Numerous reports during the last 60 years have reported a strong association between idiopathic nephrotic syndrome and atopic disorders. Idiopathic nephrotic syndrome can be precipitated by allergic reactions and has been associated with both aeroallergens (pollens, mold, and dust) and food allergies. Patients with idiopathic nephrotic syndrome also may show increased serum immunoglobulin E (IgE) levels. A review of the literature suggests that although some idiopathic nephrotic syndrome cases may be associated with allergies, evidence that it is a type of allergic disorder or can be induced by a specific allergen is weak. Rather, it is likely that the proteinuria and increased IgE levels in patients with idiopathic nephrotic syndrome are caused by increased levels of interleukin 13 observed in these patients. Recent studies suggest that interleukin 13, a known stimulator of IgE response, may mediate proteinuria in patients with minimal change disease because of its ability to directly induce CD80 expression on the podocyte. PMID:19556042

  19. Full-Length Semaphorin-3C Is an Inhibitor of Tumor Lymphangiogenesis and Metastasis.

    PubMed

    Mumblat, Yelena; Kessler, Ofra; Ilan, Neta; Neufeld, Gera

    2015-06-01

    Semaphorins play important regulatory roles in diverse processes such as axon guidance, angiogenesis, and immune responses. We find that semaphorin-3C (sema3C) induces the collapse of the cytoskeleton of lymphatic endothelial cells (LEC) in a neuropilin-2-, plexin-D1-, and plexin-A1-dependent manner, while most other semaphorins, including antiangiogenic semaphorins such as sema3A do not. Sema3C is cleaved, like other class-3 semaphorins, by furin-like pro-protein convertases (FPPC). Cleaved sema3C (p65-Sema3C) was unable to induce the collapse of the cytoskeleton of LEC. FPPC are strongly upregulated in tumor cells. In order to examine the effects of full-length sema3C on tumor progression, we therefore generated an active point mutated furin cleavage-resistant sema3C (FR-sema3C). FR-sema3C inhibited potently proliferation of LEC and to a lesser extent proliferation of human umbilical vein-derived endothelial cells. FR-sema3C also inhibited VEGF-C-induced phosphorylation of VEGFR-3, ERK1/2, and AKT. Expression of recombinant FR-sema3C in metastatic, triple-negative LM2-4 breast cancer cells did not affect their migration or proliferation in vitro. However, tumors derived from FR-sema3C-expressing LM2-4 cells implanted in mammary fat pads developed at a slower rate, contained a lower concentration of blood vessels and lymph vessels, and metastasized much less effectively to lymph nodes. Interestingly, p65-Sema3C, but not FR-sema3C, rendered A549 lung cancer cells resistant to serum deprivation, suggesting that previously reported protumorigenic activities of sema3C may be due to p65-Sema3C produced by tumor cells. Our observations suggest that FR-sema3C may be further developed into a novel antitumorigenic drug. PMID:25808871

  20. Simultaneous Multiwavelength Monitoring of 3C66A

    NASA Technical Reports Server (NTRS)

    Boettcher, M.

    2004-01-01

    The radio-selected BL Lac object 3C66A was the target of an intensive multiwavelength campaign from Sept. 2003 through Feb. 2004. It was monitored by the Whole Earth Blazar Telescope (WEBT) collaboration, in tandem with 20 X-ray monitoring observations by the Rossi X-Ray Timing Explorer (RXTE), VHE gamma-ray observations by STACEE and VERITAS, and long-term monitoring at radio frequencies. In addition. 9 observations using the VLBA are being carried out during the campaign and throughout the year 2004 to follow possible structural changes of the source. 21 pointings with RXTE during the period Sept. 15 - Dec. 27, 2003. All collected data have been fully analyzed, and first results have already been published at the 8th HEAD Meeting in New Orleans, LA, in Sept. 2004, and will also be presented at the 205th AAS Meeting in San Diego, CA, in Jan. 2005. A first Journal paper, to be submitted to the Astrophysical Journal, is currently in preparation, and we plan to have it ready for submission in January 2005. A gradual brightening of the source over the course of the campaign was observed at all optical frequencies, culminating in a very bright flare at the end of January 2004. Optical light curves indicate intraday microvariability on time scales down to about 1.3 hours. No significant color-magnitude correlation for the entire data set was evident, but there is a slight indication of a gradual spectral softening in the optical over the entire duration of multi-day outbursts (in both the rising and decaying phase). The X-ray spectrum is consistent with a power-law with a photon spectral index of approx. 2.1, indicating that the RXTE energy band might be located right at the intersection of the synchrotron and the high-energy emission components. No significant flux or spectral variability at X-ray energies was detected, though there seems to be a trend of very modest brightening in tandem with the optical flux. The first 4 VLBA epochs indicate a rather smooth jet with

  1. Effects of erythromycin in chronic idiopathic intestinal pseudo-obstruction.

    PubMed

    Minami, T; Nishibayashi, H; Shinomura, Y; Matsuzawa, Y

    1996-12-01

    The prokinetic effects of erythromycin, a macrolide antibiotic, on the gastrointestinal tract as a motilin receptor agonist and its potential value for the treatment of gastrointestinal motility disorders have recently attracted interest. The effects of erythromycin on the clinical symptoms and gastrointestinal motility of patients with chronic idiopathic pseudo-obstruction have not been investigated extensively. We presented a case of chronic idiopathic intestinal pseudo-obstruction, in a 67-year-old man in whom oral erythromycin (900 mg/day) dramatically improved postprandial abdominal distention, nausea, and vomiting. Other agents with prokinetic effects on intestinal motility, i.e., cisapride, domperidone, metoclopramide, and trimebutine maleate did not have a favorable effect. Gastric emptying, measured by the sulfamethizole method; and intestinal transit, evaluated using radio-opaque markers, were markedly improved by treatment with erythromycin. Our experience suggests that the prokinetic effects of erythromycin may be of therapeutic value in chronic idiopathic intestinal pseudo-obstruction. PMID:9027652

  2. The role of gastroesophageal reflux in idiopathic pulmonary fibrosis.

    PubMed

    Raghu, Ganesh

    2003-08-18

    Fibroblast foci are indicative of idiopathic pulmonary fibrosis and appear to be a cellular attempt to repair the damaged alveolus. Although this progressive, often fatal, clinical syndrome is thought to be dependent on alveolar injury of unknown origin, significant clinical and preclinical evidence points to gastric acid as a causative harmful agent. Graded instillation of various forms of acid in several animal models resulted in aspiration-induced lung injury, including pulmonary fibrosis in pigs. Moreover, compelling clinical data suggest that a high percentage of patients with idiopathic pulmonary fibrosis also experience abnormal esophageal acid exposure, without necessarily experiencing the typical symptoms of gastroesophageal reflux disease (GERD). Aggressive, long-term therapeutic trials of patients with GERD and evaluation of the therapeutic effects on pulmonary disease will allow determination of the real influences of abnormal esophageal acid exposure in the development of idiopathic pulmonary fibrosis. PMID:12928077

  3. Smoking-related idiopathic interstitial pneumonia: A review.

    PubMed

    Margaritopoulos, George A; Harari, Sergio; Caminati, Antonella; Antoniou, Katerina M

    2016-01-01

    For many years, cigarette smoking has been considered as the leading cause of chronic obstructive pulmonary disease and lung cancer. Recently, however, it has also been associated with the development of diffuse interstitial lung diseases. In the latest classification of the major idiopathic interstitial pneumonias (IIP), the term smoking-related IIP has been introduced, including two entities, namely desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-interstitial lung disease (RB-ILD). Other entities in which smoking has a definite or suggested role include pulmonary Langerhan's cell histiocytosis, smoking-related interstitial fibrosis, combined pulmonary fibrosis and emphysema syndrome and idiopathic pulmonary fibrosis. In this review, we will focus on the mechanisms of smoking-related lung damage and on the clinical aspects of these disorders with the exception of idiopathic pulmonary fibrosis, which will be reviewed elsewhere in this review series. PMID:26138798

  4. Gamma knife radiosurgery for medically refractory idiopathic trigeminal neuralgia.

    PubMed

    Kang, J H; Yoon, Y S; Kang, D W; Chung, S S; Chang, J W

    2008-01-01

    Gamma knife radiosurgery (GKS) has been generally considered as a viable therapeutic option for the management of medically refractory idiopathic trigeminal neuralgia (TN). We reviewed our experience with GKS in patients with TN. Between Feb 1996 and May 2006, 77 patients with medical refractory idiopathic TN were treated using GKS. Thirty-six patients who had undergone other previous procedures, previous GKS, or had brain stem lesion, atypical symptoms, were excluded from this study. Pain improvement was achieved in 38 of the patients with TN (pain response rate 92.7%). Twenty-three patients were pain free and 15 had reduced pain. There were no serious complications. We think that GKS is an effective treatment option for patients with medical refractory idiopathic TN. PMID:18642631

  5. Study on Treatment with Respect to Idiopathic Scoliosis

    NASA Astrophysics Data System (ADS)

    Takeuchi, Kenzen; Azegami, Hideyuki; Murachi, Shunji; Kitoh, Junzoh; Ishida, Yoshito; Kawakami, Noriaki; Makino, Mitsunori

    A hypothesis that the thoracic idiopathic scoliosis is buckling phenomenon of the fourth mode induced by the growth of thoracic vertebral bodies was presented in the previous work by the authors using numerical simulations with finite element model of the spine. If the hypothesis is acceptable, sensitivity function with respect to the critical growth of thoracic vertebrae on the maximization problem of buckling load with the fourth buckling mode gives us useful information to improve and develop treatments for the idiopathic scoliosis. The numerical results analyzed by the finite element method demonstrated that the sensitivity function is high at the articular capsules of the intervertebral joints, the intervertebral disks, the costotransverse joints and the constovertebral joints around the apex of the curvature in the case of the thoracic idiopathic scoliosis.

  6. [Adolescent pregnancy].

    PubMed

    Fatichi, B

    1991-10-01

    This exploration of adolescent pregnancy focuses on adolescents whose pregnancies are undesired. The physical and psychic transformations of puberty and adolescence may be experienced differently in different social contexts. The prolongation of school attendance in Western societies means that most adolescents remain financially dependent on their parents. But greater sexual freedom in the society at large has been reflected in an increase in early sexual activity among adolescents. Wider use of contraception has not completely eliminated prenatal pregnancy among adolescents. Adolescent pregnancies have actually declined in France as a proportion of all pregnancies carried to term, from 4% to 1.5-2% in the past 10 or 15 years. But in 1986, 42.5% of all induced abortions were performed on adolescents. Among causes of unwanted pregnancy in adolescents are their frequent inability to believe that they may be at risk of pregnancy, or that pregnancy can result from the 1st sexual intercourse. The episodic nature of sexual relations, the lack of ready availability of contraception, and specific shortcomings of different methods are factors in the frequent failure of adolescents to protect themselves against undesired pregnancy. Adolescents may become pregnant out of loneliness or to prove that they are women, or as a result of incest or prostitution. Adolescents who seek abortions are those who have discovered and acknowledged their pregnancies before the 12th week and had the courage to inform their parents and obtain legal permission for the abortion. Pregnancy terminations are more frequent in more advantaged societal sectors with more structured family life. The moral shock and sense of failure associated with abortion are often deeply felt by adolescents. Their experience is greatly influenced by the attitudes of those around them. Adolescents who carry their pregnancies to term are those who have not sought abortion in the 1st 12 weeks. Often they refuse to admit

  7. Optimal management of idiopathic macular holes

    PubMed Central

    Madi, Haifa A; Masri, Ibrahim; Steel, David H

    2016-01-01

    This review evaluates the current surgical options for the management of idiopathic macular holes (IMHs), including vitrectomy, ocriplasmin (OCP), and expansile gas use, and discusses key background information to inform the choice of treatment. An evidence-based approach to selecting the best treatment option for the individual patient based on IMH characteristics and patient-specific factors is suggested. For holes without vitreomacular attachment (VMA), vitrectomy is the only option with three key surgical variables: whether to peel the inner limiting membrane (ILM), the type of tamponade agent to be used, and the requirement for postoperative face-down posturing. There is a general consensus that ILM peeling improves primary anatomical hole closure rate; however, in small holes (<250 µm), it is uncertain whether peeling is always required. It has been increasingly recognized that long-acting gas and face-down positioning are not always necessary in patients with small- and medium-sized holes, but large (>400 µm) and chronic holes (>1-year history) are usually treated with long-acting gas and posturing. Several studies on posturing and gas choice were carried out in combination with ILM peeling, which may also influence the gas and posturing requirement. Combined phacovitrectomy appears to offer more rapid visual recovery without affecting the long-term outcomes of vitrectomy for IMH. OCP is licensed for use in patients with small- or medium-sized holes and VMA. A greater success rate in using OCP has been reported in smaller holes, but further predictive factors for its success are needed to refine its use. It is important to counsel patients realistically regarding the rates of success with intravitreal OCP and its potential complications. Expansile gas can be considered as a further option in small holes with VMA; however, larger studies are required to provide guidance on its use. PMID:26834454

  8. Chronic idiopathic urticaria: prevalence and clinical course.

    PubMed

    Kulthanan, Kanokvalai; Jiamton, Sukhum; Thumpimukvatana, Narumol; Pinkaew, Sumruay

    2007-05-01

    The purpose of our study was to assess the prevalence and clinical course of patients with chronic idiopathic urticaria (CIU), as well as possible causes or associated findings, laboratory findings and the duration of the disease in patients with chronic urticaria (CU). We retrospectively reviewed the 450 case record forms of patients with CU and/or angioedema who attended the Department of Dermatology, Siriraj Hospital, during the period 2000-2004. Of 450 patients with CU, 337 patients (75%) were diagnosed as CIU. Forty-three patients (9.5%) had physical urticaria, while 17 patients (3.8%) had infectious causes. Other possible causes were food, thyroid diseases, atopy, drugs, dyspepsia and collagen vascular diseases. In eighty-nine percent of patients, no abnormalities were detected at the time of physical examination. The most common abnormal laboratory finding was minimal elevation of the erythrocyte sedimentary rate (42%). In 61 patients, autologous serum skin tests had been done. Fifteen patients (24.5%) had positive results i.e. autoimmune urticaria. Anti-thyroglobulin and anti-microsomal antibodies were positive in 16 % and 12% of CIU patients respectively. After 1 year from the onset of the symptoms, 34.5% of CIU patients were free of symptoms and after 1.2 years from the onset of the symptoms, 56.5% of autoimmune urticaria patients were free of symptoms. The median disease duration of CIU and autoimmune urticaria were 390 days and 450 days respectively. Our study provided an overview of CU and CIU in a large series of Thai patients, based on etiological aspects and clinical courses. PMID:17408437

  9. The Etiology of Juvenile Idiopathic Arthritis.

    PubMed

    Rigante, Donato; Bosco, Annalisa; Esposito, Susanna

    2015-10-01

    Over the years, the commonly used term to describe juvenile idiopathic arthritis (JIA) has changed. By definition, JIA includes all types of arthritis with no apparent cause, lasting more than 6 weeks, in patients aged less than 16 years at onset. JIA pathogenesis is still poorly understood: the interaction between environmental factors and multiple genes has been proposed as the most relevant working mechanism to the development of JIA. The concept that various microbes that colonize or infect not only the mucosal surfaces, like the oral cavity, but also the airways and gut might trigger autoimmune processes, resulting in chronic arthritides, and JIA was first drafted at the outset of last century. JIA development might be initiated and sustained by the exposure to environmental factors, including infectious agents which affect people at a young age, depending on the underlying genetic predisposition to synovial inflammation. Many data from patients with JIA suggest a scenario in which different external antigens incite multiple antigen-specific pathways, cytotoxic T cell responses, activation of classical complement cascade, and production of proinflammatory cytokines. In this review, emphasis is paid not only to the potential role of parvovirus B19 and Epstein-Barr virus in primis but also to the general involvement of different bacteria as Salmonella spp., Shigella spp., Campylobacter spp., Mycoplasma pneumoniae, Chlamydophila pneumoniae, Bartonella henselae, and Streptococcus pyogenes for the development of immune-mediated arthritides during childhood. No unequivocal evidence favoring or refuting these associations has been clearly proved, and today, the strict definition of JIA etiology remains unknown. The infection can represent a random event in a susceptible individual, or it can be a necessary factor in JIA development, always in combination with a peculiar genetic background. Further studies are needed in order to address the unsolved questions

  10. Risk Factors for Idiopathic Optic Neuritis Recurrence

    PubMed Central

    Zhang, Yu-Jiao; Li, Kaijun; He, Jian-Feng

    2014-01-01

    Background Approximately 30–50% of idiopathic optic neuritis (ION) patients experience one or multiple episodes of recurrence. The aim of this study was to search for risk factors for ION recurrence. Methods Clinical data on hospitalized patients diagnosed with ION between January 2003 and January 2011 at the First Affiliated Hospital of Guangxi Medical University were retrospectively collected. Univariate and multivariate analyses were performed on factors that might cause ION recurrence. In total, 115 ION cases (32 recurrent and 83 non-recurrent cases) with complete data were analyzed. The length of the follow-up period ranged from 12 to 108 months (median: 42 months). Results The univariate analysis showed that the recurrence rate for unilateral ION was higher than that for bilateral ION (40% vs. 12%, p = 0.001). Underlying diseases had a significant impact on recurrence (p<0.001): the recurrence rates due to neuromyelitis optica (NMO), multiple sclerosis (MS), demyelinating lesions alone of the central nervous system, and unknown causes were 89%, 70%, 41%, and 8.7%, respectively. The multivariate analysis showed that the factors causing relatively high recurrence rates included NMO (odds ratio [OR], 73.5; 95% confidence interval [CI], 7.3 to 740.9), MS (OR, 33.9; 95% CI, 5.2 to 222.2), and demyelinating lesions alone (OR, 8.9; 95% CI, 2.3 to 34.4), unilateral involvement (OR, 5.7; 95% CI, 1.5 to 21.3), relatively low initial glucocorticoid dosage (equivalent to ≤100 mg prednisone/day) (OR, 4.3; 95% CI, 1.0 to 17.9). Conclusion Underlying diseases, laterality (unilateral or bilateral), and initial glucocorticoid dosage are important risk factors of ION recurrence. Clinical physicians are advised to treat ION patients with a sufficient dose of glucocorticoid in the initial treatment stage to reduce the recurrence risk. PMID:25255372

  11. Recent advances in understanding idiopathic pulmonary fibrosis

    PubMed Central

    Daccord, Cécile; Maher, Toby M.

    2016-01-01

    Despite major research efforts leading to the recent approval of pirfenidone and nintedanib, the dismal prognosis of idiopathic pulmonary fibrosis (IPF) remains unchanged. The elaboration of international diagnostic criteria and disease stratification models based on clinical, physiological, radiological, and histopathological features has improved the accuracy of IPF diagnosis and prediction of mortality risk. Nevertheless, given the marked heterogeneity in clinical phenotype and the considerable overlap of IPF with other fibrotic interstitial lung diseases (ILDs), about 10% of cases of pulmonary fibrosis remain unclassifiable. Moreover, currently available tools fail to detect early IPF, predict the highly variable course of the disease, and assess response to antifibrotic drugs. Recent advances in understanding the multiple interrelated pathogenic pathways underlying IPF have identified various molecular phenotypes resulting from complex interactions among genetic, epigenetic, transcriptional, post-transcriptional, metabolic, and environmental factors. These different disease endotypes appear to confer variable susceptibility to the condition, differing risks of rapid progression, and, possibly, altered responses to therapy. The development and validation of diagnostic and prognostic biomarkers are necessary to enable a more precise and earlier diagnosis of IPF and to improve prediction of future disease behaviour. The availability of approved antifibrotic therapies together with potential new drugs currently under evaluation also highlights the need for biomarkers able to predict and assess treatment responsiveness, thereby allowing individualised treatment based on risk of progression and drug response. This approach of disease stratification and personalised medicine is already used in the routine management of many cancers and provides a potential road map for guiding clinical care in IPF. PMID:27303645

  12. Adult idiopathic scoliosis: the tethered spine.

    PubMed

    Whyte Ferguson, Lucy

    2014-01-01

    This article reports on an observational and treatment study using three case histories to describe common patterns of muscle and fascial asymmetry in adults with idiopathic scoliosis (IS) who have significant scoliotic curvatures that were not surgically corrected and who have chronic pain. Rather than being located in the paraspinal muscles, the myofascial trigger points (TrPs) apparently responsible for the pain were located at some distance from the spine, yet referred pain to locations throughout the thoracolumbar spine. Asymmetries in these muscles appear to tether the spine in such a way that they contribute to scoliotic curvatures. Evaluation also showed that each of these individuals had major ligamentous laxity and this may also have contributed to development of scoliotic curvatures. Treatment focused on release of TrPs found to refer pain into the spine, release of related fascia, and correction of related joint dysfunction. Treatment resulted in substantial relief of longstanding chronic pain. Treatment thus validated the diagnostic hypothesis that myofascial and fascial asymmetries were to some extent responsible for pain in adults with significant scoliotic curvatures. Treatment of these patterns of TrPs and muscle and fascial asymmetries and related joint dysfunction was also effective in relieving pain in each of these individuals after they were injured in auto accidents. Treatment of myofascial TrPs and asymmetrical fascial tension along with treatment of accompanying joint dysfunction is proposed as an effective approach to treating both chronic and acute pain in adults with scoliosis that has not been surgically corrected. PMID:24411157

  13. Transitioning from Idiopathic to Explainable Autoimmune Hepatitis.

    PubMed

    Czaja, Albert J

    2015-10-01

    Autoimmune hepatitis lacks an identifiable cause, and its diagnosis requires the exclusion of etiologically defined diseases that resemble it. Insights into its pathogenesis are moving autoimmune hepatitis from an idiopathic to explainable disease, and the goal of this review is to describe the insights that are hastening this transition. Two types of autoimmune hepatitis are justified by serological markers, but they also have distinctive genetic associations (DRB1 and DQB1 genes) and autoantigens. DRB1 alleles are the principal susceptibility factors in white adults, and a six amino acid sequence encoded in the antigen-binding groove of class II molecules of the major histocompatibility complex can influence the selection of autoantigens. Polymorphisms, including variants of SH2B3 and CARD10 genes, may affect immune reactivity and disease severity. The cytochrome mono-oxygenase, CYP2D6, is the autoantigen associated with type 2 autoimmune hepatitis, and it shares homologies with multiple viruses that might promote self-intolerance by molecular mimicry. Chemokines, especially CXCL9 and CXCL10, orchestrate the migration of effector cells to sites of injury and are associated with disease severity. Cells of the innate and adaptive immune responses promote tissue damage, and possible deficiencies in the number and function of regulatory T cells may facilitate the injurious process. Receptor-mediated apoptosis is the principal mechanism of hepatocyte loss, and cell-mediated and antibody-dependent mechanisms of cytotoxicity also contribute. Insights that explain autoimmune hepatitis will allow triggering exogenous antigens to be characterized, risk management to be improved, prognostic indices to be refined, and site-specific therapeutic interventions to emerge. PMID:25999246

  14. Practical management of Idiopathic Pulmonary Fibrosis.

    PubMed

    Kishaba, Tomoo

    2015-01-01

    Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology. Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Majority of IPF patients have been elderly men with smokers. However, there are important differential diagnosis such as fibrotic non-specific interstitial pneumonia (NSIP), Connective Tissue Disease (CTD) associated ILD, chronic hypersensitivity pneumonia (CHP). Clinical point of view, non-productive cough and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing are key findings. Serum marker such as lactate deydrogenase (LDH), Krebs von den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test and 6 minute walk test (6MWT) are quite meaningful physiological examination. Serial change of forced vital capacity 6MWT distance predict mortality of IPF. International IPF guideline published recently and highlighted on the importance of high resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. IPF is chronic progressive disease. Therefore, tracing disease behavior is crucial and unifying clinical, physiological, imaging information over time provide useful information for physicians.In management, many candidate agent failed to have positive result. Pirfenidone which is anti-fibrotic agent showed to slow the decline of vital capacity and prevent of acute exacerbation. Molecular agent such as nintedanib is promising agent for prevention of progression of IPF. In this review, we review the clinical information of IPF and IPF guideline. Lastly, we show the clinical algorithm of this devastated disease. PMID:26278687

  15. Immune Complexes in Juvenile Idiopathic Arthritis

    PubMed Central

    Moore, Terry L.

    2016-01-01

    Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera. Immune complexes (ICs) have been detected in patients’ sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column, C1q solid-phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these ICs has shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF), isotypes of anti-cyclic citrullinated peptide (CCP) antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC) were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of the immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We will further address possible therapeutic modalities that could be instituted as a result of the information gathered by the presence of ICs in JIA. PMID:27242784

  16. Objective cough frequency in Idiopathic Pulmonary Fibrosis

    PubMed Central

    2010-01-01

    Background Cough is a common presenting symptom in patients with Idiopathic Pulmonary Fibrosis (IPF). This study measured cough rates in IPF patients and investigated the association between cough and measures of health related quality of life and subjective cough assessments. In addition, IPF cough rates were related to measures of physiological disease severity and compared to cough rates in health and other respiratory conditions. Methods Nineteen IPF patients, mean age 70.8 years ± 8.6, five female (26.3%) were studied. Subjects performed full pulmonary function testing, 24 hour ambulatory cough recordings, completed a cough related quality of life questionnaire (Leicester Cough Questionnaire) and subjectively scored cough severity with a visual analogue scale. Ambulatory cough recordings were manually counted and reported as number of coughs per hour. Results The 24hr cough rates were high (median 9.4, range 1.5-39.4), with day time rates much higher than night time (median 14.6, range 1.9-56.6 compared to 1.9, range 0-19.2, p = 0.003). Strong correlations were found between objective cough frequency and both the VAS (day r = 0.80, p < 0.001, night r = 0.71, p = 0.001) and LCQ (r = -0.80, p < 0.001), but not with measures of pulmonary function. Cough rates in IPF were higher than healthy subjects (p < 0.001) and asthma patients (p < 0.001) but similar to patients with chronic cough (p = 0.33). Conclusions This study confirms objectively that cough is a major, very distressing and disabling symptom in IPF patients. The strong correlations between objective cough counts and cough related quality of life measures suggest that in IPF patient's, perception of cough frequency is very accurate. PMID:20565979

  17. Bullous variant of idiopathic central serous chorioretinopathy

    PubMed Central

    Sahu, D.; Namperumalsamy, P; Hilton, G.; de Sousa, N. F

    2000-01-01

    BACKGROUND—Spontaneous bullous serous retinal detachment (RD) with subretinal exudation complicating idiopathic central serous chorioretinopathy (ICSC) is a rare and infrequently described clinical entity. Clinical observations are described on this variant form in 11 patients, the largest series reported to date.
METHODS—13 eyes of 11 Indian patients having this entity were followed up clinically and angiographically for 12-24 months (retrospective, longitudinal). None of the patients had any previous history of other diseases nor were they on any medications. Four eyes received laser treatment (group A); nine eyes were not treated (group B).
RESULTS—All 11 patients were male, aged 23-49 years (median 37 years). The clinical and photographic records revealed subretinal exudation and inferior bullous serous RD complicating ICSC with evidence of large, single or multiple, leaking retinal pigment epithelial detachments (PEDs) in all the cases. In group A, resolution of serous RD occurred in 12 weeks (median) with a visual recovery of ⩾20/30 in three out of four eyes while in group B resolution of serous retinal detachment was observed in 14 weeks (median) with eight out of nine eyes achieving a visual acuity of ⩾20/30. Subretinal fibrosis developed in two eyes in group A and none of the eyes in group B.
CONCLUSION—The disease is an exaggerated form of ICSC and can occur spontaneously without any history of corticosteroid therapy. Recognition of this atypical presentation is important to avoid inappropriate treatment. These observations suggest that with respect to the duration of the disease and the final visual outcome laser therapy offers no additional benefit over the natural course of this variant form of ICSC.

 PMID:10781512

  18. Immune Complexes in Juvenile Idiopathic Arthritis.

    PubMed

    Moore, Terry L

    2016-01-01

    Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera. Immune complexes (ICs) have been detected in patients' sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column, C1q solid-phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these ICs has shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF), isotypes of anti-cyclic citrullinated peptide (CCP) antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC) were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of the immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We will further address possible therapeutic modalities that could be instituted as a result of the information gathered by the presence of ICs in JIA. PMID:27242784

  19. Counseling adolescents.

    PubMed

    Yamuna, Srinivasan

    2013-11-01

    Skills for counseling adolescents are acquired over a period of time by all practitioners of adolescent health. Though the principles of counseling remain the same the process of counseling an adolescent differs considerably from that of a child or an adult. Adolescents are in their transition between childhood and adulthood with physical, emotional and social challenges to face. The maturity level of each adolescent differs and that decides the pace and contents of each session. The counselor sets the context in a non judgmental manner so that the adolescent feels the ease and eagerness to self disclose. Privacy and confidentiality are two key issues that have to be taken care of during counseling. PMID:23888379

  20. Treatment of Idiopathic Clubfoot in the Ponseti Era and Beyond.

    PubMed

    Chu, Alice; Lehman, Wallace B

    2015-12-01

    The initial treatment of idiopathic clubfoot was mostly surgical for the 1980s/1990s. In the latter half of the 1990s, there was a surge of interest in the Ponseti method of casting after the publication of Dr. Ponseti's 30-year results. Many authors have since shown correction rates in the high 90th percentile, rendering posteromedial release surgery almost obsolete. The success of the Ponseti method has been brought internationally and extrapolated to more and more difficult cases, such as idiopathic or syndromic, primary or recurrence. This new trend will create a different subset of complications. PMID:26589078

  1. Update on diagnosis and treatment of idiopathic pulmonary fibrosis.

    PubMed

    Baddini-Martinez, José; Baldi, Bruno Guedes; Costa, Cláudia Henrique da; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério

    2015-01-01

    Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138

  2. Idiopathic Gingival Fibromatosis: Case Report and Review of the Literature.

    PubMed

    Ko, Yen Chen K; Farr, Jeffrey B; Yoon, Angela; Philipone, Elizabeth

    2016-06-01

    Gingival fibromatosis (GF) is a condition characterized by a progressive, normal colored enlargement of the gingiva caused by an increase in the size of submucosal connective tissue. Both familial and idiopathic variants of the condition exist. The authors present a case report of a 38-year-old African American man who presented with an impressive overgrowth of the maxillary and mandibular gingivae, subsequently diagnosed as idiopathic GF. In this report, the authors will review the etiologies, treatment, and clinical and histological findings of GF, review similar cases found in the literature, and discuss the differential diagnosis for diffuse gingival enlargements. PMID:27043333

  3. Disseminated eosinophilic disease resembling idiopathic hypereosinophilic syndrome in a dog.

    PubMed

    Aroch, I; Perl, S; Markovics, A

    2001-09-29

    True idiopathic hypereosinophilic syndrome has been described in human beings and cats, but not in dogs. The syndrome is characterised by prolonged unexplained peripheral mature eosinophilia, the infiltration of many organs by eosinophils, organ dysfunction and a fatal outcome. This paper describes an idiopathic disseminated eosinophilic disease in a dog involving various organs, manly the heart and the lungs, accompanied by a leukemoid eosinophilic response, and a fatal outcome. The histopathological findings included the infiltration of the myocardium, lung parenchyma, liver, spleen, lymph nodes and skeletal muscles with eosiniphils. PMID:11601516

  4. Update on diagnosis and treatment of idiopathic pulmonary fibrosis

    PubMed Central

    Baddini-Martinez, José; Baldi, Bruno Guedes; da Costa, Cláudia Henrique; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério

    2015-01-01

    Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138

  5. Clinical Analysis and Management of Acquired Idiopathic Generalized Anhidrosis.

    PubMed

    Satoh, Takahiro

    2016-01-01

    Acquired idiopathic generalized anhidrosis (AIGA) is a sweating disorder characterized by inadequate sweating in response to heat stimuli such as high temperature, humidity, and physical exercise. Patients exhibit widespread nonsegmental hypohidrosis/anhidrosis without any apparent cause, but the palms, soles, and axillae are rarely affected. Heat stroke readily develops due to increased body temperature. AIGA commonly affects young males. Approximately 30-60% of patients show complications of cholinergic urticaria, also known as idiopathic pure sudomotor failure or hypohidrotic cholinergic urticaria. Systemic corticosteroids are the most effective therapy, although recurrence is not uncommon. PMID:27584965

  6. 17 CFR 270.3c-5 - Beneficial ownership by knowledgeable employees and certain other persons.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 17 Commodity and Securities Exchanges 3 2010-04-01 2010-04-01 false Beneficial ownership by knowledgeable employees and certain other persons. 270.3c-5 Section 270.3c-5 Commodity and Securities Exchanges SECURITIES AND EXCHANGE COMMISSION (CONTINUED) RULES AND REGULATIONS, INVESTMENT COMPANY ACT OF 1940 § 270.3c-5 Beneficial ownership...

  7. 18 CFR 3c.3 - Reporting fraud, waste, abuse, and corruption and cooperation with official inquiries.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... OF CONDUCT § 3c.3 Reporting fraud, waste, abuse, and corruption and cooperation with official inquiries. (a) Employees shall, in fulfilling the obligation of 5 CFR 2635.101(b)(11), report fraud, waste..., abuse, and corruption and cooperation with official inquiries. 3c.3 Section 3c.3 Conservation of...

  8. 17 CFR 270.3c-2 - Definition of beneficial ownership in small business investment companies.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... ownership in small business investment companies. 270.3c-2 Section 270.3c-2 Commodity and Securities... 1940 § 270.3c-2 Definition of beneficial ownership in small business investment companies. For the... the outstanding voting securities of any issuer which is a small business investment company...

  9. 17 CFR 270.3c-2 - Definition of beneficial ownership in small business investment companies.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... ownership in small business investment companies. 270.3c-2 Section 270.3c-2 Commodity and Securities... 1940 § 270.3c-2 Definition of beneficial ownership in small business investment companies. For the... the outstanding voting securities of any issuer which is a small business investment company...

  10. 17 CFR 270.3c-2 - Definition of beneficial ownership in small business investment companies.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... ownership in small business investment companies. 270.3c-2 Section 270.3c-2 Commodity and Securities... 1940 § 270.3c-2 Definition of beneficial ownership in small business investment companies. For the... the outstanding voting securities of any issuer which is a small business investment company...

  11. 17 CFR 270.3c-2 - Definition of beneficial ownership in small business investment companies.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... ownership in small business investment companies. 270.3c-2 Section 270.3c-2 Commodity and Securities... 1940 § 270.3c-2 Definition of beneficial ownership in small business investment companies. For the... the outstanding voting securities of any issuer which is a small business investment company...

  12. Histochemical and biochemical observations of the spleen in atypical Niemann-Pick disease and in idiopathic thrombocytopenic purpura.

    PubMed

    Safanda, J; Fakan, F

    1981-01-01

    In a case of adolescent Niemann-Pick disease (NP) and in a case of idiopathic thrombocytopenic purpura (ITP), the histologic picture of the spleen showed appreciable similarity in localization of sparing cells and in a number of histochemical tests. The sphingomyelin, which was the main organ phospholipid in both conditions, contained substantially elevated content of C24 fatty acids. Detailed analysis of spleen lipids showed great relative increase of lysobisphosphatidic acid and of cholesterol which was in NP mainly in free form but in ITP surprisingly mainly esterified, mostly to oleic and palmitic acid. Possible molecular mechanism of sphingomyelin storage was enzymologically followed in model conditions using separated lipid fractions from NP's spleen. The activity of sphingomyelinase (Cl. perfringens exotoxin) was in comparison to phospholipase C relatively specifically inhibited by lysobisphosphatidic acid. PMID:6168156

  13. Diffuse idiopathic skeletal hyperostosis in ancient clergymen

    PubMed Central

    Oner, F. C.; Maat, G. J. R.

    2007-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a common but often unrecognized systemic disorder observed mainly in the elderly. DISH is diagnosed when the anterior longitudinal ligament of the spine is ossified on at least four contiguous spinal levels or when multiple peripheral enthesopathies are present. The etiology of DISH is unknown but previous studies have shown a strong association with obesity and insulin-independent diabetes mellitus. DISH can lead to back pain, dysphagia, myelopathy, musculoskeletal impairment and grossly unstable spine fractures after minor trauma. In archeological studies a high prevalence of DISH has been demonstrated in ancient clergymen. The present study describes the pathological changes of human remains excavated from the abbey court (Pandhof) in the city of Maastricht, The Netherlands. Human remains of 51 individuals buried between 275 and 1795 ce were excavated and examined. The remains were investigated according to a standardized physical anthropological report and individuals demonstrating ossification of spinal ligaments and/or multiple peripheral enthesopathies were included in the study group. The authors reviewed all available material and after reaching consensus, each abnormality found was given a diagnosis and subsequently recorded. After examination, 28 individuals were considered to be adult males; 11 adult females; three adults of indeterminate sex and nine individuals were of sub adult age. The mean age at death for adults was 36.8 years. Seventeen adult individuals (40.4% of all adults), displayed ossifications of at least four contiguous spinal levels and/or multiple enthesopathies of the appendicular skeleton and were therefore, assigned the diagnosis DISH. The mean age of these individuals was 49.5 ± 13.0 years. In at least three of these individuals, DISH had led to extensive ossification and subsequent ankylosis of axial and peripheral skeletal structures. In this population of (presumably

  14. Strontium oral load test in children with idiopathic hypercalciuria.

    PubMed

    Fernández, Porfirio; Santos, Fernando; Sotorrío, Pilar; Mayordomo, Juan; Ferrero, Luis

    2007-09-01

    Increased intestinal calcium absorption may play an important role in the pathogenesis of idiopathic hypercalciuria in children. Calcium absorption was assessed by an oral strontium load test in 22 prepubertal children (13 male) with idiopathic hypercalciuria, urinary calcium excretion 6.48 +/- 0.60 mg/kg per day (range 4.12-13.40 mg/kg per day), and ten healthy, young, normocalciuric controls (six male). After administration of 2.65 mg/kg of strontium chloride (SrCl(2)), the serum concentrations of strontium at 30 min, 60 min, 120 min, 240 min, and the fraction of the absorbed dose (FAD%) at 30 min, 60 min and 240 min, were similar in both groups. FAD% at 120 min was lower (P < 0.05) in hypercalciuric children than in controls (11.84 +/- 0.96% vs 15.87 +/- 1.77%). Values of the area under the curve were not different between both groups. In children with idiopathic hypercalciuria, serum basal intact parathyroid hormone (PTH) (r = -0.59, P = 0.004) and the 1,25-dihydroxyvitamin D/PTH ratio (r = 0.65; P = 0.001) were correlated with the serum concentration of strontium at 60 min. The study reported here provides, for the first time, the results of a strontium oral load test in children with idiopathic hypercalciuria. With this method no major alterations of intestinal calcium absorption were found in this disorder. PMID:17541793

  15. Idiopathic systemic AA-amyloidosis in a skunk (Mephitis mephitis).

    PubMed

    Elhensheri, Mohamed; Linke, Reinhold P; Blankenburg, Anja; Beineke, Andreas

    2012-03-01

    This report describes a case of systemic amyloidosis in a captive striped skunk. At necropsy, bilateral alopecia, as well as reno-, hepato-, and splenomegaly were present. Congo red staining and immunohistochemistry revealed depositions of AA-amyloid in different organs. The lack of a predisposing disease is suggestive of idiopathic systemic AA-amyloidosis. PMID:22448530

  16. Unified baseline and longitudinal mortality prediction in idiopathic pulmonary fibrosis.

    PubMed

    Ley, Brett; Bradford, Williamson Z; Weycker, Derek; Vittinghoff, Eric; du Bois, Roland M; Collard, Harold R

    2015-05-01

    The Gender-Age-Physiology (GAP) model is a validated, baseline-risk prediction model for mortality in idiopathic pulmonary fibrosis. Longitudinal variables have been shown to contribute to risk prediction in idiopathic pulmonary fibrosis and may improve the predictive performance of the baseline GAP model. Our aims were to further validate the GAP model and evaluate whether the addition of longitudinal variables improves its predictive performance. The study population was derived from a large clinical trials cohort of patients with idiopathic pulmonary fibrosis (n=1109). Model performance was determined by improvement in the C-statistic, net reclassification improvement, clinical net reclassification improvement, and a goodness-of-fit test. The GAP model had good discriminative performance with a C-statistic of 0.757 (95% CI 0.750-0.764). However, the original GAP model tended to overestimate risk in this cohort. A novel, easy to use model, consisting of the original GAP predictors plus history of respiratory hospitalisation and 24-week change in forced vital capacity (the longitudinal GAP model) improved model performance with a C-statistic of 0.785 (95% CI 0.780-0.790), net reclassification improvement of 8.5%, clinical net reclassification improvement of 25%, and a goodness-of-fit test of 0.929. The Longitudinal GAP model, along with the original GAP model, may unify baseline and longitudinal mortality risk prediction in idiopathic pulmonary fibrosis. PMID:25614172

  17. Pseudoangiomatous Stromal Hyperplasia: A Rare Cause of Idiopathic Gigantomastia

    PubMed Central

    Roy, Mélissa; Lee, James; Aldekhayel, Salah

    2015-01-01

    Summary: Gigantomastia remains a rare clinical diagnosis with significant physical and psychological impacts on patients. We present the case of a 40-year-old woman with idiopathic breast enlargement. Further histological analysis of the breast tissue revealed pseudoangiomatous stromal hyperplasia. This is the first reported case of diffuse breast enlargement resulting from pseudoangiomatous stromal hyperplasia. PMID:26495214

  18. Recurrent undifferentiated shock: Idiopathic Systemic Capillary Leak Syndrome

    PubMed Central

    Aroney, Nicholas; Ure, Stewart; White, Hayden; Sane, Sunil

    2015-01-01

    Key Clinical Message Idiopathic Systemic Capillary Leak Syndrome is a potentially fatal disorder that is under diagnosed. It commonly presents as recurrent undifferentiated shock with hypotension, hypoalbuminemia and hemoconcentration. There are three distinct phases that define the syndrome; Prodromal, Extravasation and Recovery. PMID:26273434

  19. Pseudoangiomatous Stromal Hyperplasia: A Rare Cause of Idiopathic Gigantomastia.

    PubMed

    Roy, Mélissa; Lee, James; Aldekhayel, Salah; Dionisopoulos, Tassos

    2015-09-01

    Gigantomastia remains a rare clinical diagnosis with significant physical and psychological impacts on patients. We present the case of a 40-year-old woman with idiopathic breast enlargement. Further histological analysis of the breast tissue revealed pseudoangiomatous stromal hyperplasia. This is the first reported case of diffuse breast enlargement resulting from pseudoangiomatous stromal hyperplasia. PMID:26495214

  20. Aberrant regeneration in idiopathic oculomotor nerve palsy: case report.

    PubMed

    Laguna, J F; Smith, M S

    1980-06-01

    Aberrant regeneration of the oculomotor nerve usually follows injury to the nerve by posterior communicating arty aneurysms or trauma. A case of idiopathic third nerve palsy with pupillary involvement occurred in an otherwise healthy 38-year-old man. Follow-up examination 32 months later showed evidence of oculomotor function with aberrant regeneration. PMID:7381547

  1. Kibbutz Adolescents.

    ERIC Educational Resources Information Center

    Mazor, Aviva, Ed.

    1993-01-01

    This special issue on adolescence in the Israeli kibbutz contains a series of seven papers that seek to advance thought and research regarding the relationship between a communal way of life and individual developmental processes in adolescence and early adulthood. The articles represent a transitional era as the kibbutz evolves. (SLD)

  2. Adolescent Literacy

    ERIC Educational Resources Information Center

    Ippolito, Jacy, Ed.; Steele, Jennifer L., Ed.; Samson, Jennifer F., Ed.

    2012-01-01

    "Adolescent Literacy" initially appeared as a special issue of the "Harvard Educational Review". It explores key issues and debates in the adolescent literacy crisis, the popular use of cognitive strategies, and disciplinary and content-area literacy. Also examined are alternative forms of literacy, afterschool interventions, new instruction…

  3. Adolescent Neurodevelopment

    PubMed Central

    Spear, Linda Patia

    2012-01-01

    Purpose The purpose of this paper is to outline notable alterations occurring in the adolescent brain, and consider potential ramifications of these developmental transformations for public policy and programs involving adolescents. Methods Developmental changes in the adolescent brain obtained from human imaging work are reviewed, along with results of basic science studies. Results Adolescent brain transformations include both progressive and regressive changes that are regionally specific and serve to refine brain functional connectivity. Along with still maturing inhibitory control systems that can be overcome under emotional circumstances, the adolescent brain is associated with sometimes elevated activation of reward-relevant brain regions, whereas sensitivity to aversive stimuli may be attenuated. At this time, the developmental shift from greater brain plasticity early in life to the relative stability of the mature brain is still tilted more towards plasticity than seen in adulthood, perhaps providing an opportunity for some experience-influenced sculpting of the adolescent brain. Conclusions Normal developmental transformations in brain reward/aversive systems, areas critical for inhibitory control, and regions activated by emotional, exciting and stressful stimuli may promote some normative degree of adolescent risk-taking. These findings have a number of potential implications for public policies and programs focused on adolescent health and well-being. PMID:23332574

  4. Adolescent suicide.

    PubMed

    1996-01-01

    In the introduction to this report our committee, with its focus on adolescent development, expressed its concern that adolescent suicidal behavior represented a grave crisis in the adolescent, a crisis not only in the development of the adolescent but one that endangers the existence of the adolescent. The possibility of a fatal outcome is abhorrent to us as physicians and psychiatrists, as it is to all those entrusted with the care and development of our fellow human beings. Consequently, we explored the ways in which developmental and other forces lead to adolescent suicide and the measures that can be taken to prevent it. We first considered the historical and cross-cultural aspects of suicidal behaviors. Societal and cultural stresses arise from parental attitudes, beliefs, expectations, and childrearing practices that evolve from the social and economic needs in each culture. If unbalanced by growth-sustaining supports, they may compromise or constrict the existential adaptive ability of the developing adolescent and place the adolescent at risk for suicide. Research into vulnerability in adolescence has revealed gender, ethnic, and geographic differences in the dimension of the problem and has indicated the social, psychological, and biological conditions that increase the likelihood that adolescents will resort to suicidal behaviors. Research is still needed to distinguish those adolescents who commit suicide from those adolescents with similar conditions who do not. Research has only begun to explore the ways in which the interaction of specific individual dynamics, precipitating events, and personal characteristics result in an adolescent's attempt of suicide. We discussed the strengths that adolescents acquire, but we emphasized the weaknesses that ensue as adolescents are faced with the impact of the thrust of their own biological, psychological, and social development with the forces inherent in their cultures. Adolescents progress through this period

  5. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis

    PubMed Central

    Duck, Annette; Spencer, Lisa G; Bailey, Simon; Leonard, Colm; Ormes, Jennifer; Caress, Ann-Louise

    2015-01-01

    Aims To understand the perceptions, needs and experiences of patients with Idiopathic Pulmonary Fibrosis. Background Idiopathic pulmonary fibrosis is a progressive interstitial lung disease, with a mean life expectancy similar to some forms of cancer of 2–4 years from diagnosis. Unlike the cancer literature, which is rich with studies exploring the needs of their disease group, few publications exist on patient needs with this severe fibrotic lung disease. Design A Qualitative study which took place between 2007–2012. Methods Seventeen patients with a multidisciplinary team confirmed diagnosis of Idiopathic Pulmonary Fibrosis, with moderate to advanced disease severity and six of their informal carers were interviewed. An interview topic guide was developed by the researchers and service user group. The interviews were audio-recorded, semi-structured and took place at a regional respiratory and lung transplant centre in North West England. Interviews were transcribed verbatim and data analysed using Framework Analysis. Findings Three main themes were identified: ‘Struggling to get a diagnosis’; ‘Loss of the life I previously had’; and ‘Living with Idiopathic Pulmonary Fibrosis’. Patients reported struggling to get a diagnosis and coping with a life-limiting, rapidly progressive illness with no good treatment and few support structures. Conclusions There is an urgent need for a better understanding of the difficulties faced by people with Idiopathic Pulmonary Fibrosis and their carers. This can be used to develop better supportive care in the United Kingdom and ultimately improve the quality of life of these patients. PMID:25533573

  6. CVD Growth of 3C-SiC on 4H/6H Mesas

    SciTech Connect

    Neudeck,P.; Trunek, A.; Spry, D.; Powell, J.; Du, H.; Skowronski, M.; Huang, X.; Dudley, M.

    2006-01-01

    This article describes growth and characterization of the highest quality reproducible 3C-SiC heteroepitaxial films ever reported. By properly nucleating 3C-SiC growth on top of perfectly on-axis (0001) 4H-SiC mesa surfaces completely free of atomic scale steps and extended defects, growth of 3C-SiC mesa heterofilms completely free of extended crystal defects can be achieved. In contrast, nucleation and growth of 3C-SiC mesa heterofilms on top of 4H-SiC mesas with atomic-scale steps always results in numerous observable dislocations threading through the 3C-SiC epilayer. High-resolution X-ray diffraction (HRXRD) and high resolution cross-sectional transmission electron microscopy (HRXTEM) measurements indicate non-trivial, in-plane, lattice mismatch between the 3C and 4H layers. This mismatch is somewhat relieved in the step-free mesa case via misfit dislocations confined to the 3C/4H interfacial region without dislocations threading into the overlying 3C-SiC layer. These results indicate that the presence or absence of steps at the 3C/4H heteroepitaxial interface critically impacts the quality, defect structure, and relaxation mechanisms of single-crystal heteroepitaxial 3C-SiC films.

  7. CFD Growth of 3C-SiC on 4H/6H Mesas

    NASA Technical Reports Server (NTRS)

    Neudeck, Philip G.; Trunek, Andrew J.; Spry, David J.; Powell, J. Anthony; Du, Hui; Skowronski, Marek; Huang, XianRong; Dudley, Michael

    2006-01-01

    This article describes growth and characterization of the highest quality reproducible 3C-SiC heteroepitaxial films ever reported. By properly nucleating 3C-SiC growth on top of perfectly on-axis (0001) 4H-SiC mesa surfaces completely free of atomic scale steps and extended defects, growth of 3C-SiC mesa heterofilms completely free of extended crystal defects can be achieved. In contrast, nucleation and growth of 3C-SiC mesa heterofilms on top of 4H-SiC mesas with atomic-scale steps always results in numerous observable dislocations threading through the 3C-SiC epilayer. High-resolution X-ray diffraction and transmission electron microscopy measurements indicate non-trivial in-plane lattice mismatch between the 3C and 4H layers. This mismatch is somewhat relieved in the step-free mesa case via misfit dislocations confined to the 3C/4H interfacial region without dislocations threading into the overlying 3C-SiC layer. These results indicate that the presence or absence of steps at the 3C/4H heteroepitaxial interface critically impacts the quality, defect structure, and relaxation mechanisms of single-crystal heteroepitaxial 3C-SiC films.

  8. Roles of the Picornaviral 3C Proteinase in the Viral Life Cycle and Host Cells.

    PubMed

    Sun, Di; Chen, Shun; Cheng, Anchun; Wang, Mingshu

    2016-01-01

    The Picornaviridae family comprises a large group of non-enveloped viruses that have a major impact on human and veterinary health. The viral genome contains one open reading frame encoding a single polyprotein that can be processed by viral proteinases. The crucial 3C proteinases (3C(pro)s) of picornaviruses share similar spatial structures and it is becoming apparent that 3C(pro) plays a significant role in the viral life cycle and virus host interaction. Importantly, the proteinase and RNA-binding activity of 3C(pro) are involved in viral polyprotein processing and the initiation of viral RNA synthesis. In addition, 3C(pro) can induce the cleavage of certain cellular factors required for transcription, translation and nucleocytoplasmic trafficking to modulate cell physiology for viral replication. Due to interactions between 3C(pro) and these essential factors, 3C(pro) is also involved in viral pathogenesis to support efficient infection. Furthermore, based on the structural conservation, the development of irreversible inhibitors and discovery of non-covalent inhibitors for 3C(pro) are ongoing and a better understanding of the roles played by 3C(pro) may provide insights into the development of potential antiviral treatments. In this review, the current knowledge regarding the structural features, multiple functions in the viral life cycle, pathogen host interaction, and development of antiviral compounds for 3C(pro) is summarized. PMID:26999188

  9. Transitional care in clinical networks for young people with juvenile idiopathic arthritis: current situation and challenges.

    PubMed

    Cruikshank, Mary; Foster, Helen E; Stewart, Jane; Davidson, Joyce E; Rapley, Tim

    2016-04-01

    Clinical networks for paediatric and adolescent rheumatology are evolving, and their effect and role in the transition process between paediatric and adult services are unknown. We therefore explored the experiences of those involved to try and understand this further. Health professionals, young people with juvenile idiopathic arthritis and their families were recruited via five national health service paediatric and adolescent rheumatology specialist centres and networks across the UK. Seventy participants took part in focus groups and one-to-one interviews. Data was analysed using coding, memoing and mapping techniques to identify features of transitional services across the sector. Variation and inequities in transitional care exist. Although transition services in networks are evolving, development has lagged behind other areas with network establishment focusing more on access to paediatric rheumatology multidisciplinary teams. Challenges include workforce shortfalls, differences in service priorities, standards and healthcare infrastructures, and managing the legacy of historic encounters. Providing equitable high-quality clinically effective services for transition across the UK has a long way to go. There is a call from within the sector for more protected time, staff and resources to develop transition roles and services, as well as streamlining of local referral pathways between paediatric and adult healthcare services. In addition, there is a need to support professionals in developing their understanding of transitional care in clinical networks, particularly around service design, organisational change and the interpersonal skills required for collaborative working. Key messages • Transitional care in clinical networks requires collaborative working and an effective interface with paediatric and adult rheumatology.• Professional centrism and historic encounters may affect collaborative relationships within clinical networks.• Education

  10. Motor cortical hyperexcitability in idiopathic scoliosis: could focal dystonia be a subclinical etiological factor?

    PubMed Central

    Tormos, José María; Barrios, Carlos; Pascual-Leone, Alvaro

    2009-01-01

    The aetiology of idiopathic scoliosis (IS) remains unknown; however, there is a growing body of evidence suggesting that the spine deformity could be the expression of a subclinical nervous system disorder. A defective sensory input or an anomalous sensorimotor integration may lead to an abnormal postural tone and therefore the development of a spine deformity. Inhibition of the motor cortico-cortical excitability is abnormal in dystonia. Therefore, the study of cortico-cortical inhibition may shed some insight into the dystonia hypothesis regarding the pathophysiology of IS. Paired pulse transcranial magnetic stimulation was used to study cortico-cortical inhibition and facilitation in nine adolescents with IS, five teenagers with congenital scoliosis (CS) and eight healthy age-matched controls. The effect of a previous conditioning stimulus (80% intensity of resting motor threshold) on the amplitude of the motor-evoked potential induced by the test stimulus (120% of resting motor threshold) was examined at various interstimulus intervals (ISIs) in both abductor pollicis brevis muscles. The results of healthy adolescents and those with CS showed a marked inhibitory effect of the conditioning stimulus on the response to the test stimulus at interstimulus intervals shorter than 6 ms. These findings do not differ from those reported for normal adults. However, children with IS revealed an abnormally reduced cortico-cortical inhibition at the short ISIs. Cortico-cortical inhibition was practically normal on the side of the scoliotic convexity while it was significantly reduced on the side of the scoliotic concavity. In conclusion, these findings support the hypothesis that a dystonic dysfunction underlies in IS. Asymmetrical cortical hyperexcitability may play an important role in the pathogenesis of IS and represents an objective neurophysiological finding that could be used clinically. PMID:20033462

  11. Stages of Adolescence

    MedlinePlus

    ... Español Text Size Email Print Share Stages of Adolescence Page Content Article Body Adolescence, these years from puberty to adulthood, may be roughly divided into three stages: early adolescence, generally ages eleven to fourteen; middle adolescence, ages ...

  12. In Search of Biomarkers for Idiopathic Scoliosis: Leptin and BMP4 Functional Polymorphisms

    PubMed Central

    Nikolova, Svetla; Yablanski, Vasil; Vlaev, Evgeni; Getova, Gergana; Atanasov, Ventseslav; Stokov, Luben; Savov, Alexey Slavkov; Kremensky, Ivo Marinov

    2015-01-01

    Idiopathic scoliosis (IS) is the most common spinal disorder in children and adolescents. The current consensus on IS maintains that it has a multifactorial etiology with genetic predisposition factors. In the present study the association of two functional polymorphisms of leptin (rs7799039) and BMP4 (rs4898820) with susceptibility to IS and curve severity was investigated in a Bulgarian population sample. The molecular detection of the genotypes was performed by amplification followed by restriction technology. The statistical analysis was performed by Pearson's chi-squared test. This case-control study revealed no statistically significant association between the functional polymorphisms of leptin and BMP4 and susceptibility to IS or curve progression (p > 0.05). On the basis of these results the examined polymorphic variants of leptin and BMP4 could not be considered as genetic variants with predisposition effect or as risk factors for the progression of the curve. In addition, these results do not exclude a synergistic effect of the promoter polymorphisms of leptin and BMP4 in the etiology and pathogenesis of IS. The identification of molecular markers for IS could be useful for early detection and prognosis of the risk for a rapid progression of the curve. That would permit early stage treatment of the patient with the least invasive procedures. PMID:26317037

  13. Laparoscopic suture repair of idiopathic gastric perforation in Duchenne muscular dystrophy

    PubMed Central

    Miyano, Go; Nouso, Hiroshi; Morita, Keiichi; Nakajima, Hideaki; Koyama, Mariko; Kaneshiro, Masakatsu; Miyake, Hiromu; Yamoto, Masaya; Fukumoto, Koji; Urushihara, Naoto

    2015-01-01

    We report herein an adolescent case of Duchenne muscular dystrophy (DMD) with idiopathic gastric perforation, in which emergency surgical repair was performed laparoscopically. A 14-year-old nonambulatory boy with DMD was brought to our emergency department with sudden onset of severe abdominal pain and distention. Plain radiograph and computed tomography confirmed the presence of free intraperitoneal air and intrapelvic effusion. The patient elected to undergo laparoscopic inspection with 4 trocars, revealing a focal perforation, 3-4 cm in diameter, on the upper gastric body near the diaphragm. The stomach was also found to have a thin wall without evidence of peptic ulcer disease or other abnormalities. An interrupted suture was placed using 4-0 PDS. The abdomen was extensively irrigated, and multiple J-Vac drains were left in situ. Total operation time was 90 min, and no intraoperative complications were encountered. Enteral feeding through a nasogastric tube was started on postoperative day 7. The postoperative course has been uneventful as of the 12-month follow-up. Pediatric surgeons should be aware of the increased risk of gastric perforation associated with DMD, and that laparoscopic repair can be safely performed even in emergency settings. PMID:26612127

  14. Genes and molecular mechanisms involved in the epileptogenesis of idiopathic absence epilepsies.

    PubMed

    Yalçın, Ozlem

    2012-03-01

    Idiopathic absence epilepsies (IAE), that have high prevalence particularly among children and adolescents, are complex disorders mainly caused by genetic factors. Childhood absence epilepsy and juvenile absence epilepsy are among the most common subtypes of IAEs. While the role of ion channels has been the primary focus of epilepsy research, the analysis of mutation and association in both patients with absence epilepsies and animal models revealed the involvement of GABA receptors and calcium channels, but also of novel non-ion channel proteins in inducing spike wave discharges (SWD). Functional studies on a mutated variant of these proteins also support their role in the epileptogenesis of absence seizures. Studies in animal models point to both the thalamus and cortex as the origin of SWDs: the abnormalities in the components of these circuits leading to seizure activity. This review examines the current research on mutations and susceptibility alleles determined in the genes that code for the subunits of GABA receptors (GABRG2, GABRA1, GABRB3, GABRA5, GABA(B1) and GABA(B2)), calcium channels (CACNA1A, CACNA1G, CACNA1H, CACNA1I, CACNAB4, CACNAG2 and CACNG3), and novel non-ion channel proteins, taking into account the results of functional studies on these variants. PMID:22206818

  15. Diffuse Large B-Cell Lymphoma in an Adolescent Male Presenting as Ureteral Stricture

    PubMed Central

    Jaeger, Christopher D.; McAlvany, Kelly L.; Zingula, Shannon N.; Kramer, Stephen A.; Granberg, Candace F.

    2014-01-01

    Lymphoma may affect the ureter in cases of retroperitoneal involvement. We present a case of an adolescent male found to have non-Hodgkin lymphoma initially presenting as ureteral stricture evident on imaging. He was treated and responded to multiagent chemotherapy with resolution of both the lymphoma and the ureteral stricture. Although rare, non-Hodgkin lymphoma should be included in the differential diagnosis of pediatric patients with noncalculous, idiopathic ureteral strictures. PMID:25093138

  16. 3C-SiC/ZnS heterostructured nanospheres with high photocatalytic activity and enhancement mechanism

    SciTech Connect

    Zhang, J.; Wu, X. L. E-mail: paul.chu@cityu.edu.hk; Liu, L. Z.; Yang, L.; Gan, Z. X.; Chu, Paul K. E-mail: paul.chu@cityu.edu.hk

    2015-03-15

    3C-SiC/n-type ZnS heterostructured nanospheres synthesized hydrothermally deliver enhanced photocatalytic performance under visible light excitation. The heterostructured catalysts consisting of 3C-SiC and ZnS nanocrystals with a mean size being less than 5 nm exhibit extended light absorption to the visible range. The proper band structure of the 3C-SiC and ZnS nanocrystals and intrinsic electric field induced by the heterojunction promote separation of photoexcited electrons and holes in the ZnS and 3C-SiC nanocrystals resulting in the increased photocatalytic efficiency. The associated mechanism is studied and proposed.

  17. 3C protein of feline coronavirus inhibits viral replication independently of the autophagy pathway.

    PubMed

    Hsieh, Li-En; Huang, Wei-Pang; Tang, Da-Jay; Wang, Ying-Ting; Chen, Ching-Tang; Chueh, Ling-Ling

    2013-12-01

    Feline coronavirus (FCoV) can cause either asymptomatic enteric infection or fatal peritonitis in cats. Although the mutation of FCoV accessory gene 3c has been suggested to be related to the occurrence of feline infectious peritonitis (FIP), how the 3C protein is involved in this phenomenon remains unknown. To investigate the role of the 3C protein, a full-length 3c gene was transiently expressed and the cytoplasmic distribution of the protein was found to be primarily in the perinuclear region. Using 3c-stable expression cells, the replication of a 3c-defective FCoV strain was titrated and a significant decrease in replication (p<0.05) was observed. The mechanism underlying the decreased FIPV replication caused by the 3C protein was further investigated; neither the induction nor inhibition of autophagy rescued the viral replication. Taken together, our data suggest that the 3C protein might have a virulence-suppressing effect in FCoV-infected cats. Deletion of the 3c gene could therefore cause more efficient viral replication, which leads to a fatal infection. PMID:24050534

  18. Mex3c mutation reduces adiposity partially through increasing physical activity.

    PubMed

    Han, Changjie; Jiao, Yan; Zhao, Qingguo; Lu, Baisong

    2014-06-01

    MEX3C is an RNA-binding protein with unknown physiological function. We have recently reported that a Mex3c mutation in mice causes growth retardation and reduced adiposity, but how adiposity is reduced remains unclear. Herein, we show that homozygous Mex3c gene trap mice have increased physical activity. The Mex3c mutation consistently conferred full protection from diet-induced obesity, hyperglycemia, insulin resistance, hyperlipidemia, and hepatic steatosis. In ob/ob mice with leptin deficiency, the Mex3c mutation also increased physical activity and improved glucose and lipid profiles. Expressing cre in the neurons of Mex3c gene trap mice, an attempt to partially restoring neuronal Mex3c expression, significantly increased white adipose tissue deposition, but had no effects on body length. Our data suggest that one way in which Mex3c regulates adiposity is through controlling physical activity, and that neuronal Mex3c expression could play an important role in this process. PMID:24741071

  19. Idiopath=ic Granulomatous Lobular Mastitis Masquerading as a Breast Tumor: A Case Report

    PubMed Central

    Raman R, Thulasi; Manimaran, D

    2016-01-01

    Introduction Idiopathic granulomatous lobular mastitis (IGLM) is an inflammatory disease of the breast with an obscure etiology. It occurs mainly in women of reproductive age, and the lesion mimics carcinoma of the breast both clinically and radiologically Case Presentation We present the case of a 29-year-old female who visited our hospital in Kancheepuram, Tamil Nadu, with a 4 × 3 cm lump in the upper outer quadrant of her left breast. The clinical and radiological findings were indicative of a malignant lesion; however, fine-needle aspiration cytology (FNAC) revealed features of granulomatous mastitis, and the subsequent histology of the excised lump confirmed the diagnosis of IGLM. Conclusions IGLM should be considered as one of the differential diagnoses when granulomas are encountered in breast FNAC and biopsy. A definitive diagnosis of IGLM can be made by identifying its characteristic histomorphology and ruling out other causes for granulomatous inflammation. An exact diagnosis is essential since the treatment for different granulomatous conditions of the breast varies. PMID:27437133

  20. Adolescent Prostitution.

    ERIC Educational Resources Information Center

    Schaffer, Bernie; DeBlassie, Richard R.

    1984-01-01

    Explores the conditions which lead to teenagers' becoming prostitutes, including alienation, abuse, lack of education and employment, and family problems. Discusses the role of the justice system and institutions and the need for improving adolescents' self-image. (JAC)

  1. Adolescent Sexuality.

    ERIC Educational Resources Information Center

    Sharpe, Thomasina H.

    2003-01-01

    This article offers a medical and psychosocial perspective of adolescent sexual development. Sub-types of sexual development are discussed as well as treatment implications for allied health providers. (Contains 38 references.) (Author)

  2. Relaxation treatment of pseudoinsomnia and idiopathic insomnia: an electroencephalographic evaluation.

    PubMed Central

    Borkovec, T D; Grayson, J B; O'Brien, G T; Weerts, T C

    1979-01-01

    Twenty-nine insomniacs underwent four consecutive sleep laboratory evaluations before and after receiving tension-release relaxation training, no-tension-release relaxation training, or no-treatment. On the basis of the discrepancy between subjective and EEG-defined measures of latency to sleep onset, subjects were classified as pseudoinsomniacs or idiopathic insomniacs. As predicted, tension-release relaxation was significancy more effective than the other two conditions on subjective sleep measures, regardless of insomnia subtype and on objective sleep measures only for idiopathic insomniacs. Subjective improvement was maintained at 12-month followup. Numerous differences between the two subtypes emerged on pretherapy and during-therapy measures distinct from the latency measures, but changes on those variables were unrelated to outcome improvement. PMID:381276

  3. RARE CASE OF IDIOPATHIC GINGIVAL FIBROMATOSIS AFFECTING PRIMARY DENTITION.

    PubMed

    Ahmed, Sanaa; Ali, Zahid

    2015-01-01

    Gingival fibromatosis (GF) is a rare condition with an estimated incidence of 1750,000 in autosomal dominant cases and is supposedly genetic in origin. Gingival fibromatosis (GF) may occur as an isolated finding (Idiopathic gingival fibromatosis or IGF) or in combination with additional clinical problems that is, giving rise to syndromic forms of the disease. It usually is triggered when permanent dentition starts to erupt and can cover crowns of the teeth. Gingival fibromatosis occasionally manifests at birth or affect primary dentition. This enlargement may cause mal-position and diastema. Surgical excision of excessive fibrous tissue is the only treatment but it recurs. We are presenting here a case of 5 year old patient presenting with severe idiopathic gingival fibromatosis covering crowns of primary teeth and causing functional impairment. PMID:27004356

  4. [Idiopathic granulomatous mastitis treated with steroids and methotrexate].

    PubMed

    Peña-Santos, Genaro; Ruiz-Moreno, José Luis

    2011-06-01

    Idiopathic granulomatous mastitis is a rare inflammatory breast disease of unknown etiology. It manifests as breast mass of 6 cm on average (range 2-10 cm), often in upper outer quadrant of left breast, in another quadrant, right or bilateral breast. Clinical diagnosis by ultrasound or mammography and fine needle aspiration confuses with carcinoma; histopathology (gold standard) confirm the diagnosis after ruling out causes of granulomatous inflammation, mainly tuberculosis. Steroid treatment achieve complete remission, but adverse reactions and relapses after the descent and suspension. Methotrexate or azathioprine is added from the start to maintain remission. We report three cases of idiopathic granulomatous mastitis diagnosis and treatment based on prednisone until clinical improvement and methotrexate as maintenance therapy. Complete remission was obtained in three patients. The rheumatologist knows and handles autoimmune/inflammatory with these drugs, therefore, is suggested the multidisciplinary treatment of this disease with oncologists and gynecologists. PMID:21966829

  5. Searching for Common Mammalian Retroviruses in Pediatric Idiopathic Diseases

    PubMed Central

    Jeziorski, Eric; Foulongne, Vincent; Ludwig, Catherine; Louhaem, Djamel; Rodiere, Michel; Sitbon, Marc; Courgnaud, Valérie

    2016-01-01

    Mammalian retroviruses cause a variety of diseases in their hosts, including hematological and immunodeficiency disorders. Both human T-cell leukemia (HTLV) and human immunodeficiency (HIV) viruses originated from several independent zoonotic transmissions, indicating that cross-species transmissions from animal to humans may still occur. Thus, as the risk for retroviral transmissions from animals to humans increase, we investigated whether mammalian retroviruses are involved in selected pediatric idiopathic diseases whose symptoms evoke retroviral infections. Blood samples, sera, and synovial fluids, or bone marrow cells were collected from pediatric patients under 18 years of age with different autoimmune idiopathic diseases. Overall, we screened clinical samples from 110 children using sensitive nested and semi-nested PCR strategies targeting env genes, and a C-type retrovirus reverse transcriptase (RT) activity kit. All clinical samples were free of retroviral signatures, indicating the unlikelihood of an etiological role of the retroviruses we assessed in the pediatric diseases we tested. PMID:27102168

  6. Familial Idiopathic Cranial Neuropathy in a Chinese Family.

    PubMed

    Zhang, Li; Liang, Jianfeng; Yu, Yanbing

    2016-01-01

    Cranial neuropathy is usually idiopathic and familial cases are uncommon. We describe a family with 5 members with cranial neuropathy over 3 generations. All affected patients were women, indicating an X-linked dominant or an autosomal dominant mode of inheritance. Our cases and a review of the literature suggest that familial idiopathic cranial neuropathy is a rare condition which may be related to autosomal dominant vascular disorders (e.g. vascular tortuosity, sclerosis, elongation or extension), small posterior cranial fossas, anatomical variations of the posterior circulation, hypersensitivity of cranial nerves and other abnormalities. Moreover, microvascular decompression is the treatment of choice because vascular compression is the main factor in the pathogenesis. To the best of our knowledge, this is the first report of familial cranial neuropathy in China. PMID:27161475

  7. Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide.

    PubMed

    Flanagan, Frances; Glackin, Louisa; Slattery, Dubhfeasa M

    2013-03-01

    Idiopathic pulmonary hemosiderosis (IPH), a subtype of diffuse alveolar hemorrhage is a rare condition, first described by Virchow in 1864. Historically, it manifests in children in the first decade of life with the combination of hemoptysis, iron deficiency anemia, and alveolar infiltrates on chest radiograph. More recently, diffuse alveolar hemorrhage has been classified by the absence or presence of pulmonary capillaritis (PC), the latter carrying a potential for a poorer outcome. While systemic corticosteroids remain the first line treatment option, other immune modulators have been trailed including hydroxychloroquine, azathioprine, 6-mercaptopurine, and cyclophosphamide with varying results. Our case demonstrates for the first time, the successful use of intravenous cyclophosphamide in the management of chronic idiopathic PC. PMID:22573417

  8. Precision Medicine: The New Frontier in Idiopathic Pulmonary Fibrosis.

    PubMed

    Brownell, Robert; Kaminski, Naftali; Woodruff, Prescott G; Bradford, Williamson Z; Richeldi, Luca; Martinez, Fernando J; Collard, Harold R

    2016-06-01

    Precision medicine is defined by the National Institute of Health's Precision Medicine Initiative Working Group as an approach to disease treatment that takes into account individual variability in genes, environment, and lifestyle. There has been increased interest in applying the concept of precision medicine to idiopathic pulmonary fibrosis, in particular to search for genetic and molecular biomarker-based profiles (so called endotypes) that identify mechanistically distinct disease subgroups. The relevance of precision medicine to idiopathic pulmonary fibrosis is yet to be established, but we believe that it holds great promise to provide targeted and highly effective therapies to patients. In this manuscript, we describe the field's nascent efforts in genetic/molecular endotype identification and how environmental and behavioral subgroups may also be relevant to disease management. PMID:26991475

  9. Searching for Common Mammalian Retroviruses in Pediatric Idiopathic Diseases.

    PubMed

    Jeziorski, Eric; Foulongne, Vincent; Ludwig, Catherine; Louhaem, Djamel; Rodiere, Michel; Sitbon, Marc; Courgnaud, Valérie

    2016-03-01

    Mammalian retroviruses cause a variety of diseases in their hosts, including hematological and immunodeficiency disorders. Both human T-cell leukemia (HTLV) and human immunodeficiency (HIV) viruses originated from several independent zoonotic transmissions, indicating that cross-species transmissions from animal to humans may still occur. Thus, as the risk for retroviral transmissions from animals to humans increase, we investigated whether mammalian retroviruses are involved in selected pediatric idiopathic diseases whose symptoms evoke retroviral infections. Blood samples, sera, and synovial fluids, or bone marrow cells were collected from pediatric patients under 18 years of age with different autoimmune idiopathic diseases. Overall, we screened clinical samples from 110 children using sensitive nested and semi-nested PCR strategies targeting env genes, and a C-type retrovirus reverse transcriptase (RT) activity kit. All clinical samples were free of retroviral signatures, indicating the unlikelihood of an etiological role of the retroviruses we assessed in the pediatric diseases we tested. PMID:27102168

  10. Advances on the genetics of mendelian idiopathic epilepsies.

    PubMed

    Baulac, Stéphanie; Baulac, Michel

    2009-11-01

    Genetic factors play an increasingly recognized role in idiopathic epilepsies. Since 1995, positional cloning strategies in multi-generational families with autosomal dominant transmission have revealed 11 genes (KCNQ2, KCNQ3, CHRNA4, CHRNA2, CHRNB2, SCN1B, SCN1A, SCN2A, GABRG2, GABRA1, and LGI1) and numerous loci for febrile seizures and epilepsies. To date, all genes with the exception of LGI1 (leucine-rich glioma inactivated 1), encode neuronal ion channel or neurotransmitter receptor subunits. Molecular approaches have revealed great genetic heterogeneity, with the vast majority of genes remaining to be identified. One of the major challenges is now to understand phenotype-genotype correlations. This review focuses on the current knowledge on the molecular basis of these rare Mendelian autosomal dominant forms of idiopathic epilepsies. PMID:19853223

  11. Advances on the genetics of Mendelian idiopathic epilepsies.

    PubMed

    Baulac, Stéphanie; Baulac, Michel

    2010-12-01

    Genetic factors play an increasingly recognized role in idiopathic epilepsies. Since 1995, positional cloning strategies in multigenerational families with autosomal dominant transmission have revealed 11 genes (KCNQ2, KCNQ3, CHRNA4, CHRNA2, CHRNB2, SCN1B, SCN1A, SCN2A, GABRG2, GABRA1, and LGI1) and numerous loci for febrile seizures and epilepsies. To date, all genes with the exception of LGI1, encode neuronal ion channel or neurotransmitter receptor subunits. Molecular approaches have revealed great genetic heterogeneity, with most genes remaining to be identified. One of the major challenges is now to understand phenotype-genotype correlations. This review focuses on the current knowledge on the molecular basis of these rare mendelian autosomal dominant forms of idiopathic epilepsies. PMID:20832659

  12. Finite Element Model for Hydrocephalus and Idiopathic Intracranial Hypertension.

    PubMed

    Kim, Dong-Joo; Kim, Hakseung; Park, Dae-Hyeon; Lee, Hack-Jin; Czosnyka, Zofia; Sutcliffe, Michael P F; Czosnyka, Marek

    2016-01-01

    Hydrocephalus and idiopathic intracranial hypertension (IIH) are neuropathies associated with disturbed cerebrospinal fluid dynamics. Several finite element (FE) brain models were suggested to simulate the pathological changes in hydrocephalus, but with overly simplified assumptions regarding the properties of the brain parenchyma. This study proposes a two-dimensional FE brain model, capable of simulating both hydrocephalus and IIH by incorporating poro-hyperelasticity of the brain and detailed structural information (i.e., sulci). PMID:27165898

  13. Long term gait outcomes of surgically treated idiopathic toe walkers.

    PubMed

    McMulkin, Mark L; Gordon, Andi B; Tompkins, Bryan J; Caskey, Paul M; Baird, Glen O

    2016-02-01

    Toe walking is a common gait deviation which in the absence of a known cause is termed idiopathic toe walking. Surgical treatment in the presence of a triceps surae contracture includes tendo-Achilles or gastrocnemius/soleus recession and has been shown to be effective in improving kinematic outcomes at a one year follow up. The purpose of this study was to assess longer term kinematic and kinetic outcomes of children with idiopathic toe walking treated surgically for gastrocnemius/soleus contractures. Eight subjects with a diagnosis of idiopathic toe walking who had surgical lengthening of the gastrocnemius/soleus and had previous motion analysis laboratory studies pre-operative and 1 year post-operative, returned for a motion analysis laboratory study greater than 5 years since surgery. Subjects completed lower extremity physical exam and 3-D computerized kinematics and kinetics. Significant improvements for mean pelvic tilt, peak dorsiflexion in stance and swing, and overall kinematics index at 1 year post-operative were maintained at 5 years post-operative. Kinetic variables of ankle moment and power were improved at 1 year and 5 years post-operative. On physical exam, dorsiflexion with knee extended was tighter from 1 to 5 year follow-up which did not correspond to the functional changes of gait. Idiopathic toe walkers who were treated surgically for triceps surae contractures showed significant improvements in key kinematic and kinetic gait analysis variables at 1 year post-operative that were maintained at 5 years post-operative. Overall, subjects were satisfied with outcomes of the surgery, unrestricted in activities, and reported minimal pain. PMID:27004661

  14. Primary cutaneous aspergillosis and idiopathic bone marrow aplasia.

    PubMed

    Furlan, Karina Colossi; Pires, Mario Cezar; Kakizaki, Priscila; Chartuni, Juliana Cabral Nunes; Valente, Neusa Yuriko Sakai

    2016-01-01

    We describe the case of a 9-year-old boy with idiopathic bone marrow aplasia and severe neutropenia, who developed skin ulcers under cardiac monitoring electrodes. The diagnosis of primary cutaneous aspergillosis was made after the second biopsy and culture. Imaging investigation did not reveal internal fungal infection. The child was treated, but did not improve and died 3 months after admission. The report highlights and discusses the preventable risk of aspergillus skin infection in immunocompromised patients. PMID:27438213

  15. Case Report: Elevated CPK, an indicator of idiopathic inflammatory myopathy?

    PubMed Central

    Khan, Hina N.; Jilani, Usman; Arora, Shitij

    2016-01-01

    Polymyositis is a rare disease with incidence rates at about 1 per 100,000 people annually. In this case report we will review a case of proximal muscle weakness with an elevated creatine phosphokinase that was initially misdiagnosed twice as rhabdomyolysis. Therefore, emphasizing that idiopathic inflammatory myopathy is a potential cause of myasthenia that must be considered in the differential. The case will also describe the current treatment and treatment response in polymyositis.

  16. Primary cutaneous aspergillosis and idiopathic bone marrow aplasia*

    PubMed Central

    Furlan, Karina Colossi; Pires, Mario Cezar; Kakizaki, Priscila; Chartuni, Juliana Cabral Nunes; Valente, Neusa Yuriko Sakai

    2016-01-01

    We describe the case of a 9-year-old boy with idiopathic bone marrow aplasia and severe neutropenia, who developed skin ulcers under cardiac monitoring electrodes. The diagnosis of primary cutaneous aspergillosis was made after the second biopsy and culture. Imaging investigation did not reveal internal fungal infection. The child was treated, but did not improve and died 3 months after admission. The report highlights and discusses the preventable risk of aspergillus skin infection in immunocompromised patients. PMID:27438213

  17. Case Report: Elevated CPK, an indicator of idiopathic inflammatory myopathy?

    PubMed

    Khan, Hina N; Jilani, Usman; Arora, Shitij

    2016-01-01

    Polymyositis is a rare disease with incidence rates at about 1 per 100,000 people annually. In this case report we will review a case of proximal muscle weakness with an elevated creatine phosphokinase that was initially misdiagnosed twice as rhabdomyolysis. Therefore, emphasizing that idiopathic inflammatory myopathy is a potential cause of myasthenia that must be considered in the differential. The case will also describe the current treatment and treatment response in polymyositis. PMID:27540467

  18. [Idiopathic non-cirrhotic portal hypertension: An update].

    PubMed

    Bissonnette, Julien; Rautou, Pierre-Emmanuel; Valla, Dominique-Charles

    2015-10-01

    Idiopathic non-cirrhotic portal hypertension is an under-estimated cause of portal hypertension. The diagnosis requires the exclusion of cirrhosis, common causes of chronic liver disease and venous obstruction of the portal and hepatic veins. It has been associated with various extra-hepatic conditions that are most frequently immunologic, prothrombotic, hematologic and toxic. The most frequent clinical complications are variceal hemorrhage and portal vein thrombosis. Complications of portal hypertension should be managed as in patients with cirrhosis. PMID:26362514

  19. Nerve sparing clitoroplasty in a rare case of idiopathic clitoromegaly

    PubMed Central

    Kujur, Abha Rani; Joseph, Vijay; Chandra, Praveen

    2016-01-01

    Clitoromegaly is an embarrassing condition causing psychological stress, requiring intervention. The goals of clitoroplasty are to achieve normal genital anatomy and to preserve tactile sensation with a satisfactory sexual response. We present a rare case of idiopathic clitoromegaly managed by reduction clitoroplasty, preserving the dorsal neurovascular bundle and extensive network of nerves around the corpora to the glans and the creation of labia minora. PMID:27274128

  20. Recent developments in the management of idiopathic cholestatic liver disease

    PubMed Central

    Imam, Mohamad H.; Weeding, Emma; Lindor, Keith D.

    2012-01-01

    In recent years, the clinical management of patients with idiopathic cholestatic liver disease has shown significant progress. Advancement of diagnostic and therapeutic approaches and better understanding of the pathophysiology underlying these diseases have all contributed considerably to this progress. In this review, we aim to touch briefly on several developments that have occurred in this regard and to discuss novel findings and interventions valuable to clinical practice. PMID:24714257

  1. Non-progressive familial idiopathic intracranial calcification: a family report.

    PubMed Central

    Callender, J S

    1995-01-01

    The clinical features and long term outcome of familial idiopathic intracranial calcification in three members of one family are described. The illness presented as psychiatric disorder in all patients, and in one patient, epilepsy and intellectual deterioration were later manifestations. Skull radiographs and CT were performed sequentially, in one patient, over a 22 year period and, in another, CT was carried out eight years apart. In neither patient was there any evidence of progression of calcification. Images PMID:7561925

  2. Premature subclinical atherosclerosis in children and young adults with juvenile idiopathic arthritis. A review considering preventive measures.

    PubMed

    Bohr, Anna-Helene; Fuhlbrigge, Robert C; Pedersen, Freddy Karup; de Ferranti, Sarah D; Müller, Klaus

    2016-01-01

    Many studies show that Juvenile Idiopathic Arthritis (JIA) is associated with early subclinical signs of atherosclerosis. Chronic inflammation per se may be an important driver but other known risk factors, such as dyslipidemia, hypertension, insulin insensitivity, a physically inactive lifestyle, obesity, and tobacco smoking may also contribute substantially. We performed a systematic review of studies through the last 20 years on early signs of subclinical atherosclerosis in children and adolescents with JIA with the purpose of investigating whether possible risk factors, other than inflammation, were considered.We found 13 descriptive cross sectional studies with healthy controls, one intervention study and two studies on adults diagnosed with JIA. Only one study addressed obesity, and physical activity (PA) has only been assessed in one study on adults with JIA and only by self-reporting. This is important as studies on PA in children with JIA have shown that most patients are less physically active than their healthy peers, and as physical inactivity in several large studies of normal schoolchildren is found to be associated with increased clustering of risk factors for cardiovascular disease. It is thus possible that an inactive lifestyle in patients with JIA is an important contributor to development of the subclinical signs of atherosclerosis seen in children with JIA, and that promotion of an active lifestyle in childhood and adolescence may diminish the risk for premature atherosclerotic events in adulthood. PMID:26738563

  3. Idiopathic interstitial pneumonia following bone marrow transplantation: the relationship with total body irradiation

    SciTech Connect

    Keane, T.J.; Van Dyk, J.; Rider, W.D.

    1981-10-01

    Interstitial pneumonia is a frequent and often fatal complication of allogenic bone marrow transplantation. Thirty to 40 percent of such cases are of unknown etiology and have been labelled as cases of idiopathic interstitial pneumonia. Idiopathic cases are more commonly associated with the use of total body irradiation; their occurrence appears to be independent of immunosupression or graft versus host disease. Evidence is presented from the literature suggesting that the development of idiopathic interstitial pneumonia is related to the absolute absorbed dose of radiation to lung. The similarity of idiopathic pneumonia to radiation pneumonitis seen in a different clinical setting is described.

  4. Helicobacter pylori-negative, non-steroidal anti-inflammatory drug: negative idiopathic ulcers in Asia.

    PubMed

    Iijima, Katsunori; Kanno, Takeshi; Koike, Tomoyuki; Shimosegawa, Tooru

    2014-01-21

    Since the discovery of Helicobacter pylori (H. pylori) infection in the stomach, the bacteria infection and non-steroidal anti-inflammatory drugs (NSAIDs) use had been considered to be the 2 main causes of peptic ulcers. However, there have been recent reports of an increase in the proportion of peptic ulcers without these known risk factors; these are termed idiopathic peptic ulcers. Such trend was firstly indicated in 1990s from some reports in North America. In Asia, numerous studies reported that idiopathic ulcers accounted for a small percentage of all ulcers in the 1990s, but in the 2000s, multiple studies reported that the proportion of idiopathic ulcers had reached 10%-30%, indicating that the incidence of idiopathic ulcers in Asia has also been rising in recent years. While a decline in H. pylori infection rates of general population in Asia is seen as the main reason for the increased incidence of idiopathic ulcers, it is also possible that the absolute number of idiopathic ulcer cases has increased. Advanced age, serious systemic complication, and psychological stress are considered to be the potential risk factors for idiopathic ulcers. Management of idiopathic ulcers is challenging, at present, because there is no effective preventative measure against recurrence in contrast with cases of H. pylori-positive ulcers and NSAIDs-induced ulcers. As it is expected that H. pylori infection rates in Asia will decline further in the future, measures to treat idiopathic ulcers will also likely become more important. PMID:24574744

  5. VizieR Online Data Catalog: Optical imaging and spectra of 3C 58 (Fesen+, 2008)

    NASA Astrophysics Data System (ADS)

    Fesen, R.; Rudie, G.; Hurford, A.; Soto, A.

    2009-09-01

    Interference filter images of 3C 58 were obtained in 1994 September using the McGraw-Hill 1.3m telescope at the MDM Observatory at Kitt Peak. Low-dispersion, long-slit optical spectra of 3C 58 were obtained on 1995 October 16-23 using the MDM 2.4m telescope. (1 data file).

  6. 40 CFR 59.1 - Final determinations under Section 183(e)(3)(C) of the CAA.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... furniture coatings; (b) Aerospace coatings; (c) Shipbuilding and repair coatings; (d) Lithographic printing...(e)(3)(C) of the CAA. 59.1 Section 59.1 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY... COMMERCIAL PRODUCTS General § 59.1 Final determinations under Section 183(e)(3)(C) of the CAA. This...

  7. 40 CFR 59.1 - Final determinations under Section 183(e)(3)(C) of the CAA.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... furniture coatings; (b) Aerospace coatings; (c) Shipbuilding and repair coatings; (d) Lithographic printing...(e)(3)(C) of the CAA. 59.1 Section 59.1 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY... COMMERCIAL PRODUCTS General § 59.1 Final determinations under Section 183(e)(3)(C) of the CAA. This...

  8. 40 CFR 59.1 - Final determinations under Section 183(e)(3)(C) of the CAA.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... furniture coatings; (b) Aerospace coatings; (c) Shipbuilding and repair coatings; (d) Lithographic printing...(e)(3)(C) of the CAA. 59.1 Section 59.1 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY... COMMERCIAL PRODUCTS General § 59.1 Final determinations under Section 183(e)(3)(C) of the CAA. This...

  9. 40 CFR 59.1 - Final determinations under Section 183(e)(3)(C) of the CAA.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ...(e)(3)(C) of the CAA. 59.1 Section 59.1 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY... COMMERCIAL PRODUCTS General § 59.1 Final determinations under Section 183(e)(3)(C) of the CAA. This section... materials; (e) Letterpress printing materials; (f) Flexible packaging printing materials; (g) Flat...

  10. [Case report: idiopathic ventricular tachycardia from the aortic sinus cusp].

    PubMed

    Tejera-Jurado, Luis Carlos; Nava, Santiago; Colín-Lizalde, Luis; Márquez, Manlio F; Gómez-Flores, Jorge; González-Hermosillo, Jesús Antonio; Iturralde-Torres, Pedro

    2009-01-01

    Idiopathic ventricular tachycardia is identified in 10% of the patients presenting with ventricular tachycardia, and they consist of various subtypes that can originate from different areas, including the aortic cusps which represent 0.7% of the total. Electrocardiographically, these ventricular tachycardias display a left bundle branch block pattern and inferior axis, and although rare, should be considered in the differential diagnosis with tachycardias originating from the septal region of the right ventricular outflow tract, which comprise 80% of the idiopathic ventricular tachycardias. Despite the vicinity of the left coronary artery ostium, radiofrequency catheter ablation can be curative in more than 90% of cases with a low risk (< 1% of serious complication). Therefore, it must be considered first-line therapy in patients who have failed or are intolerant to therapy with antiarrhythmic agents. The aim of this article is to describe the first case reported in Mexico of a successful ablation idiopathic ventricular tachycardia from the aortic sinus cusp in a patient with incessant ventricular tachycardia. PMID:19902669

  11. Correlation between quantitative EEG and MRI in idiopathic generalized epilepsy.

    PubMed

    Betting, Luiz E; Li, Li M; Lopes-Cendes, Iscia; Guerreiro, Marilisa M; Guerreiro, Carlos A M; Cendes, Fernando

    2010-09-01

    The objective of this study was to investigate the relationship between the focal discharges sometimes observed in the electroencephalogram of patients with idiopathic generalized epilepsies and subtle structural magnetic resonance imaging abnormalities. The main hypothesis to be assessed is that focal discharges may arise from areas of structural abnormality which can be detected by quantitative neuroimaging. Focal discharges were used for quantitative electroencephalogram source detection. Neuroimaging investigations consisted of voxel-based morphometry and region of interest volumetry. For voxel-based morphometry, volumetric MRI were acquired and processed. The images of each patient were individually compared with a control group. Statistical analysis was used to detect differences in gray matter volumes. Region of interest-based morphometry was automatically performed and used essentially to confirm voxel-based morphometry findings. The localization of the focal discharges on the electroencephalogram was compared to the neuroimaging results. Twenty-two patients with idiopathic generalized epilepsies were evaluated. Gray matter abnormalities were detected by voxel-based morphometry analysis in 77% of the patients. There was a good concordance between EEG source detection and voxel-based morphometry. On average, the nearest voxels detected by these methods were 19 mm (mm) apart and the most statistically significant voxels were 34 mm apart. This study suggests that in some cases subtle gray matter abnormalities are associated with focal epileptiform discharges observed in the electroencephalograms of patients with idiopathic generalized epilepsies. PMID:20082332

  12. Effect of bracing on respiratory mechanics in mild idiopathic scoliosis.

    PubMed Central

    Kennedy, J D; Robertson, C F; Hudson, I; Phelan, P D

    1989-01-01

    The use of a corrective orthopaedic brace is an established form of management for patients with progressive idiopathic scoliosis. Thirteen patients with mild idiopathic scoliosis were studied with and without the corrective brace applied. Lung volumes and the pattern of chest wall and abdominal movement were measured during quiet breathing. Transdiaphragmatic pressures were measured in six of the patients and upper ribcage movement in seven patients. Application of the brace resulted in a significant reduction in vital capacity (14%), functional residual capacity (22%), and total lung capacity (12%). There was no effect on respiratory rate or minute volume. In the erect position the pattern of chest wall movement was altered with a reduction in lower ribcage movement of 46% and abdominal wall of 39% and an increase in upper ribcage movement of 43%. These changes were greater in the supine position. There was at least a twofold increase in end inspiratory and end expiratory gastric pressures during tidal breathing, but oesophageal pressures were not affected by the brace. Transdiaphragmatic pressures showed a similar twofold increase, which implies a substantial increase in the work of breathing. In view of the doubts concerning the influence of bracing on the natural history of idiopathic scoliosis and the substantial functional effect of bracing on the respiratory system, it is suggested that the current practice of bracing in this condition needs to be reviewed. PMID:2772855

  13. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions.

    PubMed

    Markey, Keira A; Mollan, Susan P; Jensen, Rigmor H; Sinclair, Alexandra J

    2016-01-01

    Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus, and back and neck pain, but the clinical presentation is highly variable. Although few studies have been done to support evidence-based management, several recent advances have the potential to enhance understanding of the causes of the disease and to guide treatment decisions. Investigators of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide in patients with mild visual loss. Studies have also established weight loss as an effective disease-modifying treatment, and further clinical trials to investigate new treatments are underway. The incidence of idiopathic intracranial hypertension is expected to increase as rates of obesity increase; efforts to reduce diagnostic delays and identify new, effective approaches to treatment will be key to meeting the needs of a growing number of patients. PMID:26700907

  14. Towards a new taxonomy of idiopathic orofacial pain.

    PubMed

    Woda, Alain; Tubert-Jeannin, Stéphanie; Bouhassira, Didier; Attal, Nadine; Fleiter, Bernard; Goulet, Jean-Paul; Gremeau-Richard, Christelle; Navez, Marie Louise; Picard, Pascale; Pionchon, Paul; Albuisson, Eliane

    2005-08-01

    There is no current consensus on the taxonomy of the different forms of idiopathic orofacial pain (stomatodynia, atypical odontalgia, atypical facial pain, facial arthromyalgia), which are sometimes considered as separate entities and sometimes grouped together. In the present prospective multicentric study, we used a systematic approach to help to place these different painful syndromes in the general classification of chronic facial pain. This multicenter study was carried out on 245 consecutive patients presenting with chronic facial pain (>4 months duration). Each patient was seen by two experts who proposed a diagnosis, administered a 111-item questionnaire and filled out a standardized 68-item examination form. Statistical processing included univariate analysis and several forms of multidimensional analysis. Migraines (n=37), tension-type headache (n=26), post-traumatic neuralgia (n=20) and trigeminal neuralgia (n=13) tended to cluster independently. When signs and symptoms describing topographic features were not included in the list of variables, the idiopathic orofacial pain patients tended to cluster in a single group. Inside this large cluster, only stomatodynia (n=42) emerged as a distinct homogenous subgroup. In contrast, facial arthromyalgia (n=46) and an entity formed with atypical facial pain (n=25) and atypical odontalgia (n=13) could only be individualised by variables reflecting topographical characteristics. These data provide grounds for an evidence-based classification of idiopathic facial pain entities and indicate that the current sub-classification of these syndromes relies primarily on the topography of the symptoms. PMID:15979796

  15. Bosentan for pulmonary hypertension secondary to idiopathic pulmonary fibrosis

    PubMed Central

    Onda, Naomi; Tanaka, Yosuke; Hino, Mitsunori; Gemma, Akihiko

    2014-01-01

    Pulmonary hypertension is a poor prognostic factor in patients with interstitial lung disease. No established treatment exists for pulmonary hypertension secondary to interstitial pneumonia. We describe the case of an 81-year-old woman with idiopathic pulmonary fibrosis (IPF), who was admitted to our hospital due to aggravation of dyspnea and decreased oxygen saturation, as well as onset of orthopnea and rapidly progressing edema. The transthoracic echocardiography and right heart catheterization showed the mean pulmonary artery pressure was 39 mmHg and the mean pulmonary capillary wedge pressure was 9 mmHg. After various examinations, the diagnoses of pulmonary hypertension (PH) due to IPF and of congestive heart failure secondary to PH were established. Diuretic therapy was started, but the patient's condition showed poor improvement. Subsequent initiation of oral bosentan therapy led to improvement in symptoms and findings. At the follow-up assessment one year later her pulmonary function showed no significant changes and no apparent worsening of arterial blood gases, with evident improvement of PH, WHO functional class, maximum exercise tolerance on treadmill exercise testing, right heart catheterization, and transthoracic echocardiography. This report describes a case of successful treatment with bosentan for severe pulmonary hypertension in a patient with idiopathic pulmonary fibrosis. We also present a review of the literature on treatment of pulmonary hypertension in patients with chronic lung disease. Bosentan appears to be efficacious in some patients with pulmonary hypertension secondary to idiopathic interstitial pneumonitis. PMID:26029570

  16. Circulating antimyenteric autoantibodies in Tunisian patients with idiopathic achalasia.

    PubMed

    Kallel-Sellami, M; Karoui, S; Romdhane, H; Laadhar, L; Serghini, M; Boubaker, J; Lahmar, H; Filali, A; Makni, S

    2013-01-01

    The physiopathology of idiopathic achalasia is still unknown. The description of circulating antimyenteric autoantibodies (CAA), directed against enteric neurons in sera of patients, suggests an autoimmune process. Recent data showed controversies according to the existence and the significance of CAA. The aims of this study were to investigate whether CAA are detected in Tunisian patients with idiopathic achalasia and to look for associated clinical or manometrical factors with CAA positivity. Twenty-seven patients with idiopathic achalasia and 57 healthy controls were prospectively studied. CAA were assessed by indirect immunofluorescence on intestinal monkey tissue sections. Western blot on primate cerebellum protein extract and dot technique with highly purified recombinant neuronal antigens (Hu, Ri, and Yo) were further used to analyze target antigens of CAA. CAA were significantly increased in achalasia patients compared with controls when considering nuclear or cytoplasmic fluorescence patterns. (33% vs. 12%, P = 0.03 and 48% vs. 23%, P = 0.001 respectively). By immunoblot analysis, CAA did not target neuronal antigens, however 52/53 and 49 kDa bands were consistently detected. CAA positivity was not correlated to specific clinical features. The results are along with previous studies demonstrating high CAA prevalence in achalasia patients. When reviewing technical protocols and interpretation criteria, several discrepancies which could explain controversies between studies were noted. PMID:22947106

  17. Axial length in unilateral idiopathic central serous chorioretinopathy

    PubMed Central

    Moon, Hoseok; Lee, Dae Yeong; Nam, Dong Heun

    2016-01-01

    AIM To evaluate the axial length (AXL) in unilateral idiopathic central serous chorioretinopathy (CSC). METHODS This retrospective case-control study was comprised of a consecutive case series of 35 patients with acute unilateral idiopathic CSC, and age- and sex-matched 50 control eyes. AXL of both eyes of unilateral CSC patients and the control eyes were investigated. AXL was measured by ultrasonic biometry, and the adjusted AXL was calculated for CSC eyes as measured AXL plus differences of foveal thickness between CSC and normal fellow eyes in millimeters. The main outcome measures were comparison of AXL between CSC, fellow and control eyes. RESULTS The mean age of 35 CSC patients was 45.5y, and 31 males were included. The adjusted AXL of CSC eyes was 23.52 mm, and the AXL of fellow eyes was 23.46 mm, and of control eyes 23.94 mm. The AXL of both CSC and fellow eyes were significantly shorter than control eyes (CSC vs control, P=0.044; fellow vs control, P=0.026). There was no statistically significant difference in AXL between CSC and fellow eyes. CONCLUSION In unilateral idiopathic CSC, the AXL of CSC and fellow eyes are shorter than that of control eyes. Short AXL may be related with choroidal circulation abnormality in CSC. PMID:27275428

  18. Parameters of the turbulence of the interplanetary plasma derived from scintillation observations of the quasars 3C 48 and 3C 298 at the solar-activity maximum

    NASA Astrophysics Data System (ADS)

    Glubokova, S. K.; Glyantsev, A. V.; Tyul'bashev, S. A.; Chashei, I. V.; Shishov, V. I.

    2015-01-01

    An analysis of temporal spectra of interplanetary scintillations of the radio sources 3C 48 and 3C 298 observed at 111 MHz on the Big Scanning Antenna of the Lebedev Physical Institute at the maximum of the 24th solar-activity cycle is reported. The measured temporal spectra of the scintillations are used to estimate the velocity of the in homogeneities and the index of the spatial spectrum of the turbulence. The dependence of the spectral index of the turbulence on the solar-wind speed persists in periods of high solar activity, when the global spatial structure of the solar wind is strongly modulated by the activity cycle.

  19. Photoelectrochemical CO2 reduction on 3C-SiC photoanode in aqueous solution

    NASA Astrophysics Data System (ADS)

    Song, Jun Tae; Iwasaki, Takayuki; Hatano, Mutsuko

    2015-04-01

    Photoelectrochemical (PEC) carbon dioxide (CO2) reduction on a 3C-SiC photoanode is demonstrated in aqueous solution with Pt and Ag counter electrodes. It is demonstrated that 3C-SiC has sufficient potential for CO2 reduction by confirming the band-edge structure. Then, the CO2 reduction is realized by connecting the 3C-SiC photoanode with the counter electrode. As the products of the PEC reaction with an applied bias of 1 V (vs counter electrode) to the 3C-SiC photoanode, hydrogen (H2) and carbon monoxide (CO) were analyzed by highly sensitive micro-gas chromatography, by which the time dependence of the gas products can be analyzed. Under light illumination of the 3C-SiC photoanode, CO2 reduction occurred while producing 2.5 and 9 nmol of CO gas with the Pt and Ag counter electrodes, respectively, after the reaction for 3000 s.

  20. [Adolescence: viewpoints from adolescent psychiatry].

    PubMed

    Bürgin, D; von Klitzing, K

    1994-05-01

    Adolescence is a phase of human development which is marked by a high vulnerability due to the ongoing psycho-physiological transformations. The regulation of the self-esteem is especially in danger in youngsters who went into adolescence with a marked burden of conflicts or who lived in families with disturbed intrafamilial dynamics. To be present as a partner and not to find the solutions for the adolescents' conflicts, to accept their questioning of what is established and to recognize their movements of reconciliation are the quite complex demands put on to the world of the adults. Adolescents urge us to a review of our own adolescence, to a balancing of hate and love, openness and rigidity, and to dialectic movements between disintegration and reintegration as well as between the generations. Any help, be it on the physical, the social or the psychic level, should be directed toward a restitution of the intrapsychic, intrafamilial or intergenerational balance; sociocultural factors have also always to be respected. The helpers--especially in a culture with rapid change--are often confronted with their own adolescence, which took place a generation before and mostly under totally different conditions. PMID:8016759