Science.gov

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. Diagnosis and treatment of adolescent idiopathic scoliosis.

    PubMed

    Burton, Monique S

    2013-11-01

    Scoliosis is defined as a lateral curvature of the spine greater than 10 degrees on radiography that is typically associated with trunk rotation. The three major types of scoliosis are congenital, idiopathic, and neuromuscular. Idiopathic scoliosis is divided into three subcategories based on the age of onset. Infantile idiopathic scoliosis affects patients younger than 3 years, juvenile idiopathic scoliosis appears in children between 3 and 10 years, and adolescent idiopathic scoliosis (AIS) occurs in skeletally immature patients older than 10 years. AIS is the most common form of idiopathic scoliosis. Approximately 2% to 4% of children aged 10 to 16 years have some degree of spinal curvature. Although some researchers view routine screening for AIS as controversial, well-child examinations and sports physicals are an optimal time to evaluate for AIS in the clinical setting. In 2008, the American Academy of Orthopaedic Surgeons, the Scoliosis Research Society, the Pediatric Orthopaedic Society of North America, and the American Academy of Pediatrics convened a task force to review the issues related to scoliosis screening and issued an information statement concluding that although screening has limitations, the potential benefits that patients with idiopathic scoliosis receive from early treatment can be substantial. Recommendations are now that females are screened twice, at age 10 and 12 years, and males once at age 13 or 14 years. Screening during routine well-child examinations and/or school-based evaluations will help identify patients who need ongoing monitoring. The evaluation of curvatures in conjunction with the level of skeletal maturity will help to guide the management of the curvature.

  6. 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

  7. Adolescent idiopathic scoliosis and back pain.

    PubMed

    Balagué, Federico; Pellisé, Ferran

    2016-01-01

    This broad narrative review addresses the relationship between adolescent idiopathic scoliosis (AIS) and back pain. AIS can be responsible for low back pain, particularly major cases. However, a linear relationship between back pain and the magnitude of the deformity cannot be expected for any individual patient. A large number of juvenile patients can remain pain-free. The long-term prognosis is rather benign for many cases and thus a tailored approach to the individual patient seems mandatory. The level of evidence available does not allow stringent recommendations for any of the disorders included in this review. PMID:27648474

  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. Sagittal Balance in Adolescent Idiopathic Scoliosis

    PubMed Central

    Xu, Xi-Ming; Wang, Fei; Zhou, Xiao-Yi; Liu, Zi-Xuan; Wei, Xian-Zhao; Bai, Yu-Shu; Li, Ming

    2015-01-01

    Abstract The relationship between spinal sagittal alignment and pelvic parameters is well known in adolescent idiopathic scoliosis. However, few studies have reported the sagittal spinopelvic relationship after selective posterior fusion of thoracolumbar/lumbar (TL/L) curves. We evaluated the relationship between spinal sagittal alignment and the pelvis, and analyzed how the pelvic sagittal state is adjusted in Lenke type 5C patients. We conducted a retrospective study of 36 patients with Lenke type 5C curves who received selective posterior TL/L curve fusion. Coronal and spinopelvic sagittal parameters were pre and postoperatively compared. Pearson coefficients were used to analyze the correlation between all spinopelvic sagittal parameters before and after surgery. We also evaluated 3 pelvic morphologies (anteverted, normal, and retroverted) before and after surgery. Preoperatively, the mean pelvic incidence was 46.0°, with a pelvic tilt and sacral slope (SS) of 8.2° and 37.8°, respectively, and 25% (9/36) of patients had an anteverted pelvis, whereas the other 75% had a normal pelvis. Postoperatively, 42% (15/36) of patients had a retroverted pelvis, 53% (19/36) had a normal pelvis, and 2 patients had an anteverted pelvis. Logistic regression analyses yielded 2 factors that were significantly associated with the risk for a postoperative unrecovered anteverted pelvis, including increased lumbar lordosis (LL) (odds ratio [OR] 4.8, P = 0.029) and increased SS (OR 5.6, P = 0.018). Four factors were significantly associated with the risk of a postoperative newly anteverted pelvis, including LL at the final follow-up (OR 6.9, P = 0.009), increased LL (OR 8.9, P = 0.003), LL below fusion (OR 9.4, P = 0.002), and increased SS (OR 11.5, P = 0.001). The pelvic state may be adjusted after selective posterior TL/L curve fusion in Lenke 5C adolescent idiopathic scoliosis patients. It is difficult to improve an anteverted pelvis in patients who have

  10. 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

  11. 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

  12. 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

  13. [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

  14. Current progress in genetic research of adolescent idiopathic scoliosis

    PubMed Central

    Xu, Leilei; Qiu, Yong

    2015-01-01

    Previous genetic linkage analysis and candidate gene association analysis have unveiled dozens of variants associated with the development of adolescent idiopathic scoliosis (AIS), which however can seldom be replicated in different ethnics. Recently, two genome-wide association studies of AIS performed in Japan revealed that ladybird homeobox 1 (LBX1) gene and G protein–coupled receptor 126 (GPR126) gene could play a role in the etiopathogenesis of the disease. Since the association between these two genes and AIS were successfully validated in the Caucasian and the Chinese population, LBX1 gene and GPR126 gene were the most reliable genetic variants underling the development of AIS. PMID:26046064

  15. [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.

  16. 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

  17. 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.

  18. 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

  19. 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

  20. 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

  1. 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

  2. Geographic latitude and prevalence of adolescent idiopathic scoliosis.

    PubMed

    Grivas, Theodoros B; Vasiliadis, Elias; Savvidou, Olga; Mouzakis, Vasilios; Koufopoulos, Georgios

    2006-01-01

    Adolescent Idiopathic Scoliosis (AIS) prevalence has been reported to be different in various geographic latitudes and demonstrates higher values in northern countries. A study on epidemiological reports from the literature was conducted to record the prevalence of AIS among the general population of boys and girls, aged 10-16 years old, in different geographic latitudes, in order to test the hypothesis that the prevalence of AIS among boys and girls is different in various geographic latitudes and to examine if there is a possible association between them. Seventeen peer-reviewed published papers reporting AIS prevalence in the general population of boys and girls from most geographic areas of the northern hemisphere were retrieved from the literature. The geographic latitude of each centre where a particular study was originated was documented. The statistical analysis included a linear regression forward modeling procedure of the AIS prevalence by latitude, weighted by sample size. According to the modelling of the data, a significant positive association between prevalence of AIS and latitude was found for girls (p<0.001), following a rather curvilinear trend, but not a significant positive association was found for boys (p<0.111). A positive association between prevalence of AIS and geographic latitude is reported only for girls in the present study. Prevalence of AIS in boys is not associated significantly with geographic latitude. This differing significant association implicates the possible role of environmental factors in the pathogenesis of AIS that may act in a different way between boys and girls.

  3. 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

  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. Magnetic resonance imaging of the brain stem in adolescent idiopathic scoliosis.

    PubMed

    Geissele, A E; Kransdorf, M J; Geyer, C A; Jelinek, J S; Van Dam, B E

    1991-07-01

    The cause of adolescent idiopathic scoliosis remains an enigma. Several studies have demonstrated abnormalities of posture, proprioception, and equilibrium control in patients with adolescent idiopathic scoliosis. These functions are integrated by structures in and around the brain stem. Twenty-seven patients with adolescent idiopathic scoliosis were studied with magnetic resonance imaging to delineate the anatomy of the brain stem in such patients. Imaging was conducted from the hypothalamus to the spinal cord at C3 in 26 patients; the remaining patient underwent an incomplete study because of a claustrophobic reaction. The study group consisted of 25 females and 2 males with an average age of 16 + 5 years. There were 19 right thoracic curves, 5 thoracolumbar curves, and 3 left lumbar curves. The mean primary curve size was 27 degrees at the most recent clinical evaluation. Seven patients were treated with observation, 14 with bracing, and 6 with surgery. The magnetic resonance imaging studies were read independently by three attending radiologists in a randomized, blinded fashion along with the magnetic resonance imaging studies of 11 controls. Asymmetry in the ventral pons or medulla in the area of the corticospinal tracts was noted in seven study patients and one control; one study patient had an enlarged cisterna magna and one an inconclusive (incomplete) study. These findings may support previous studies that have suggested a central nervous system abnormality as a cause of adolescent idiopathic scoliosis. PMID:1925751

  6. 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

  7. Weakness of the neck extensors, possible causes and relation to adolescent idiopathic cervical kyphosis.

    PubMed

    Xiaolong, Shen; Xuhui, Zhou; Jian, Chen; Ye, Tian; Wen, Yuan

    2011-09-01

    Cervical kyphosis may be congenital, or occur as a result of laminectomy, post-traumatic deformity, infection, neuromuscular disorders such as muscular dystrophies, motor neuron disorders such as amyotrophic lateral sclerosis, tumor, and inflammation such as ankylosing spondylitis. Furthermore, adolescent idiopathic cervical kyphosis was defined as cervical kyphotic deformity of adolescent patient without any cause such as those previously described. As no standard values for "cervical kyphosis" could be found in the literature, many reported studies only report a subjective classification, "kyphotic, straight or lordotic". But this method had proven to be unreliable. Grob et al. defined "straight" for the global curvature as +4° to -4°, and lordotic and kyphotic as <-4° and >+4°, respectively. The etiology and pathogenesis of adolescent idiopathic cervical kyphosis remain little understood. Weakness of the neck extensors can result in "dropped head syndrome", a rare disorder characterized by weakness of neck extensor muscles causing an inability to extend the neck and resulting in a chin-on-chest deformity. The purpose of this paper is to propose a possible mechanical cause leading to the kyphotic deformity. We hypothesize that weakness of the neck extensors could be the initiating factor for adolescent idiopathic cervical kyphosis.

  8. 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

  9. 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

  10. [Fusion in adolescent idiopathic scoliosis : Anterior, posterior or combined? One-stage or two-stage?].

    PubMed

    Quack, V; Rath, B; Schenker, H; Schulze, A; El Mansy, Y; Tingart, M; Betsch, M

    2015-11-01

    Scoliosis is a complex, three-dimensional spinal deformity with various causes. Adolescent idiopathic scoliosis (AIS) is the most common form. Surgical treatment is indicated for curves greater than 45-50° meaured using the Cobb method. We can distinguish among posterior, anterior or combined surgical procedures. Today, the posterior, transpedicular approach has revolutionized scoliosis surgery. This review gives an overview of current surgical options in scoliosis treatment.

  11. 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

  12. Comparative analysis of pedicle screw versus hybrid instrumentation in adolescent idiopathic scoliosis surgery

    PubMed Central

    Rafi, Sohail; Munshi, Naseem; Abbas, Asad; Shaikh, Rabia Hassan; Hashmi, Imtiaz

    2016-01-01

    Introduction: Adolescent idiopathic scoliosis is the most common type of scoliosis. A Cobb angle of 50° will progress beyond the age of spinal maturity. Surgery over bracing is advised at a Cobb angle above or equal to 50°. The aim of surgery is to bring the Cobb angle down below 50° to prevent reprogression as well as improve the quality of life. The objective of the study is to analyze the efficacy and significance in lifestyle improvement of pedicle screw-only fixation system versus the more common hybrid instrumentation system used for the surgical treatment of adolescent idiopathic scoliosis. Materials and Methods: A prospective cohort study was conducted involving two groups of patients were included in the study. One group was operated with pedicle screw-only method while the other with hybrid instrumentation system. The pre- and post-operative Cobb's angles were taken across a follow-up of 4 years. An SRS-30 questionnaire was given in a yearly follow-up to assess the lifestyle improvement of the patient. Results: Pedicle screw-only method was significantly more effective in reducing Cobb's angle (P = 0.0487). It was showed less loss of correction (P = 0.009) pedicle screw-only surgery was also better at reducing thoracic curves (P = 0.001). There seemed a better recovery time with pedicle screw surgery (P = 0.003). Conclusion: Pedicle screws are more effective and durable than hybrid systems at when treating adolescent idiopathic scoliosis.

  13. Comparative analysis of pedicle screw versus hybrid instrumentation in adolescent idiopathic scoliosis surgery

    PubMed Central

    Rafi, Sohail; Munshi, Naseem; Abbas, Asad; Shaikh, Rabia Hassan; Hashmi, Imtiaz

    2016-01-01

    Introduction: Adolescent idiopathic scoliosis is the most common type of scoliosis. A Cobb angle of 50° will progress beyond the age of spinal maturity. Surgery over bracing is advised at a Cobb angle above or equal to 50°. The aim of surgery is to bring the Cobb angle down below 50° to prevent reprogression as well as improve the quality of life. The objective of the study is to analyze the efficacy and significance in lifestyle improvement of pedicle screw-only fixation system versus the more common hybrid instrumentation system used for the surgical treatment of adolescent idiopathic scoliosis. Materials and Methods: A prospective cohort study was conducted involving two groups of patients were included in the study. One group was operated with pedicle screw-only method while the other with hybrid instrumentation system. The pre- and post-operative Cobb's angles were taken across a follow-up of 4 years. An SRS-30 questionnaire was given in a yearly follow-up to assess the lifestyle improvement of the patient. Results: Pedicle screw-only method was significantly more effective in reducing Cobb's angle (P = 0.0487). It was showed less loss of correction (P = 0.009) pedicle screw-only surgery was also better at reducing thoracic curves (P = 0.001). There seemed a better recovery time with pedicle screw surgery (P = 0.003). Conclusion: Pedicle screws are more effective and durable than hybrid systems at when treating adolescent idiopathic scoliosis. PMID:27695235

  14. Evidence for Conservative Treatment of Adolescent Idiopathic Scoliosis - Update 2015 (Mini-Review).

    PubMed

    Sy, Ng; Bettany-Saltikov, Josette; Moramarco, Marc

    2016-01-01

    Idiopathic scoliosis predominantly afflicts adolescents. Adolescents with mild curvatures do not generally have any symptoms. However spinal fusion is indicated when the deformity exceeds 45°. Treatment is thus necessary to prevent and/or reduce the progression of curvatures to that below which surgery is indicated. Conservative treatment of adolescent idiopathic scoliosis includes observation, scoliosis-specific exercises (SSE) and bracing. There is increasing evidence suggesting that SSE and brace treatment can significantly limit the progression of spinal curvatures. In growing adolescents with curvatures more than 20°, bracing is indicated and should be used in conjunction with SSE. The effectiveness of bracing varies according to the type of brace applied to the patient. In general rigid braces are preferable to soft flexible braces, as the latter falls short of halting curvatures progression. Also, preliminary evidence suggests that asymmetric braces which enable over-correction provide more correction when compared with symmetrical braces. Recently it has also been reported that high quality bracing can also reduce curvatures exceeding 45° in over 70% of growing adolescents. This new knowledge might possibly increase the threshold of surgical indications to beyond 50° or above in the near future.

  15. 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…

  16. Eigenspine: computing the correlation between measures describing vertebral pose for patients with adolescent idiopathic scoliosis.

    PubMed

    Forsberg, Daniel; Lundström, Claes; Knutsson, Hans

    2014-10-01

    This paper describes the concept of eigenspine, a concept applicable for determining the correlation between pair-wise combinations of measures useful for describing the three-dimensional spinal deformities associated with adolescent idiopathic scoliosis. The proposed data analysis scheme is based upon the use of principal component analysis (PCA) and canonical correlation analysis (CCA). PCA is employed to reduce the dimensionality of the data space, thereby providing a regularization of the measurements, and CCA is employed to determine the linear dependence between pair-wise combinations of different measures. The usefulness of the eigenspine concept is demonstrated by analyzing the position and the rotation of all lumbar and thoracic vertebrae as obtained from 46 patients suffering from adolescent idiopathic scoliosis. The analysis showed that the strongest linear relationship is found between the lateral displacement and the coronal rotation of the vertebrae, and that a somewhat weaker but still strong correlation is found between the coronal rotation and the axial rotation of the vertebrae. These results are well in-line with the general understanding of idiopathic scoliosis. Noteworthy though is that the correlation between the anterior-posterior displacement and the sagittal rotation was not as strong as expected and that the obtained results further indicate the need for including the axial vertebral rotation as a measure when characterizing different types of idiopathic scoliosis. Apart from analyzing pair-wise correlations between different measures, the method is believed to be suitable for finding a maximally descriptive low-dimensional combination of measures describing spinal deformities in idiopathic scoliosis.

  17. 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

  18. Scoliosis elasticity assessed by manual traction: 49 juvenile and adolescent idiopathic cases.

    PubMed

    Soucacos, P K; Soucacos, P N; Beris, A E

    1996-04-01

    We assessed preoperative curve elasticity in 49 consecutive patients with juvenile or adolescent idiopathic scoliosis who were operated on with Harrington distraction rods. Preoperatively, the curve was determined from posteroanterior radiographs taken in the standing position and in the supine position, with traction. In the latter, the radiographs were taken at the moment of maximal traction when one technician applied traction to the ankles and another to the wrists. The scoliotic curve in the 10 patients with juvenile scoliosis averaged 59 degrees and 32 degrees in the standing and supine positions with traction, respectively. Immediately postoperatively, the curve averaged 19 degrees. 39 patients with adolescent scoliosis had a scoliotic curve which averaged 58 degrees in the standing position and 32 degrees in the supine position with traction. The mean postoperative measurement was 21 degrees. These findings suggest that manual traction is a simple and reliable means of predicting the minimal correction of the scoliotic curve to be expected, using Harrington distraction rods.

  19. 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

  20. 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

  1. [Advance on genetic mechanism of adolescent idiopathic scoliosis and genetic relationship map].

    PubMed

    Wang, Wei; Ma, Jun; Li, Shu-yuan; Wu, Xian; Hu, Bin; Wang, Xiao-feng; Zhou, Xu-hui

    2015-09-01

    Identification of genetic risk factors is the hotspot of adolescent idiopathic scoliosis (AIS). Through candidate gene approach and genome-wide association studies (GWAS), some genes were preliminary identified. To review AIS related genes,and construct the gene network map of AIS gene. We searched on NCBI PubMed and Web of Science database using search terms "adolescent idiopathic scoliosis" and "gene", to classify induction genes. We then constructed gene diagram using string-db. We found 35 AIS genes relating to connective tissue, nervous system active substances, melatonin synthesis and metabolism, puberty and growth, and genes whose function is unknown. Gene diagram shows that a network relationship between gene and other genes,in which IL6, ESR1, ESR2, VDR, TGFB1, IGF1 gene may as the key gene about AIS' genetic mechanism. Two sites of 3 GWAS results outside the network, it is suggesting new pathway that need to be explored. The study about AIS susceptibility gene is still preliminary, requiring in-depth research to identify the new networks.

  2. Association of GPR126 gene polymorphism with adolescent idiopathic scoliosis in Chinese populations.

    PubMed

    Xu, Ji-Feng; Yang, Guang-hai; Pan, Xiao-Hong; Zhang, Shui-Jun; Zhao, Chen; Qiu, Bin-Song; Gu, Hai-Feng; Hong, Jian-Fei; Cao, Li; Chen, Yu; Xia, Bing; Bi, Qin; Wang, Ya-Ping

    2015-02-01

    Idiopathic scoliosis is the most common pediatric spinal deformity affecting 1% to 3% of the population, and adolescent idiopathic scoliosis (AIS) accounts for approximately 80% of these cases; however, the etiology and pathogenesis of AIS are still uncertain. The current study aims to identify the relationship between G protein-coupled receptor 126 (GPR126) gene and AIS predisposition, to identify the relationship between the genotypes of the GPR126 SNPs and the clinical phenotypes of AIS. We conducted a case-control study and genotyped twenty SNPs of GPR126 gene including ten exonic SNPs and ten intronic polymorphisms in 352 Chinese sporadic AIS patients and 149 healthy controls. We provided evidence for strong association of three intronic SNPs of the GPR126 gene with AIS susceptibility: rs6570507 A > G (p =0 .0035, OR = 1.729), rs7774095 A > C (p = 0.0078, OR = 1.687), and rs7755109 A > G (p = 0.0078, OR = 1.687). However, we did not identify any significant association between ten exonic SNPs of GPR126 and AIS. Linkage disequilibrium analysis indicated that rs7774095 A > C and rs7755109 A > G could be parsed into one block. The association between the intronic haplotype and AIS was further confirmed in an independent population with 110 AIS individuals and 130 healthy controls (p = 0.046, OR = 1.680). Furthermore, molecular mechanisms underlying intronic SNP regulation of GPR126 gene were studied. Although intronic SNPs associated with AIS didn't influence GPR126 mRNA alternative splicing, there was a strong association of rs7755109 A > G with decreased GPR126 mRNA level and protein levels. Our findings indicate that genetic variants of GPR126 gene are associated with AIS susceptibility in Chinese populations. The genetic association of GPR126 gene and AIS might provide valuable insights into the pathogenesis of adolescent idiopathic scoliosis.

  3. A review of pinealectomy-induced melatonin-deficient animal models for the study of etiopathogenesis of adolescent idiopathic scoliosis.

    PubMed

    Man, Gene Chi Wai; Wai, Man Gene Chi; Wang, William Wei Jun; Jun, Wang William Wei; Yim, Annie Po Yee; Yee, Yim Annie Po; Wong, Jack Ho; Ho, Wong Jack; Ng, Tzi Bun; Bun, Ng Tzi; Lam, Tsz Ping; Ping, Lam Tsz; Lee, Simon Kwong Man; Man, Lee Simon Kwong; Ng, Bobby Kin Wah; Wah, Ng Bobby Kin; Wang, Chi Chiu; Chiu, Wang Chi; Qiu, Yong; Yong, Qiu; Cheng, Chun Yiu; Yiu, Cheng Jack Chun

    2014-09-18

    Adolescent idiopathic scoliosis (AIS) is a common orthopedic disorder of unknown etiology and pathogenesis. Melatonin and melatonin pathway dysfunction has been widely suspected to play an important role in the pathogenesis. Many different types of animal models have been developed to induce experimental scoliosis mimicking the pathoanatomical features of idiopathic scoliosis in human. The scoliosis deformity was believed to be induced by pinealectomy and mediated through the resulting melatonin-deficiency. However, the lack of upright mechanical spinal loading and inherent rotational instability of the curvature render the similarity of these models to the human counterparts questionable. Different concerns have been raised challenging the scientific validity and limitations of each model. The objectives of this review follow the logical need to re-examine and compare the relevance and appropriateness of each of the animal models that have been used for studying the etiopathogenesis of adolescent idiopathic scoliosis in human in the past 15 to 20 years.

  4. Do Adolescent Idiopathic Scoliosis (AIS) Neglect Proprioceptive Information in Sensory Integration of Postural Control?

    PubMed Central

    Assaiante, Christine; Mallau, Sophie; Jouve, Jean-Luc; Bollini, Gérard; Vaugoyeau, Marianne

    2012-01-01

    Introduction It has been reported that AIS rely much more on ankle proprioception to control the amplitude of the balance control commands as compared to age-matched healthy adolescents. Our hypothesis was that AIS do not neglect proprioceptive information to control posture probably because of their vestibular deficits. We investigated the proprioceptive contribution to postural control in AIS which expresses spinal deformity during a crucial transitional period of ontogenesis. Methods 10 adolescents with idiopathic scoliosis (AIS) with moderate spinal deformity (10° < Cobb Angle >35°) and 10 control adolescents (CA) had to maintain vertical stance while very slow oscillations in the frontal plane (below the detection threshold of the semicircular canal system) were applied to the support with the eyes open and closed. Postural orientation and segmental stabilisation were analysed at head, shoulder, trunk and pelvis levels. Results Scoliosis did not affect vertical orientation control and segmental stabilization strategies. Vision improves postural control in both CA and AIS, which seem more dependent on visual cues than adults. Conclusions AIS as CA were unable to control efficiently their postural orientation on the basis of the proprioceptive cues, the only sensory information available in the EC situation, whereas in the same condition healthy young adults present no difficulty to achieve the postural control. This suggests that AIS as CA transitory neglect proprioceptive information to control their posture. These results and previous studies suggest the existence of different afferent pathways for proprioceptive information subserving different parts in sensory integration of postural control. We conclude that the static proprioceptive system is not affected by the idiopathic scoliosis, while the dynamic proprioceptive system would be mainly affected. PMID:22815779

  5. The Association Study of Calmodulin 1 Gene Polymorphisms with Susceptibility to Adolescent Idiopathic Scoliosis

    PubMed Central

    Zhang, Yu; Gu, Zuchao; Qiu, Guixing

    2014-01-01

    Objective. Idiopathic scoliosis is the most common pediatric spinal deformity affecting 1% to 3% of the population, and adolescent idiopathic scoliosis (AIS) accounts for approximately 80% of these cases; however, the etiology and pathogenesis of AIS are still uncertain. The current study aims to identify the relationship between calmodulin 1 (CALM1) gene and AIS predisposition, to identify the relationship between the genotypes of the SNPs and the clinical phenotypes of AIS. Methods. 146 AIS patients and 146 healthy controls were enrolled into this case-control study. 12 single nucleotide polymorphisms (SNPs) candidates in CALM1 gene were selected to determine the relationship between CALM1 gene and AIS predisposition. Case-only study was performed to determine the effects of these variants on the severity of the condition. Results. Three SNPs from 12 candidates were found to be associated with AIS predisposition. The ORs were observed as 0.549 (95% CI 0.3519–0.8579, P = 0.0079), 0.549 (95% CI 0.3519–0.8579, P = 0.0079), and 1.6139 (95% CI 1.0576–2.4634, P = 0.0257) for rs2300496, rs2300500, and rs3231718, respectively. There was no statistical difference between main curve, severity, and genotype distributions of all of 12 SNPs. Conclusion. Genetic variants of CALM1 gene are associated with AIS susceptibility. PMID:24551838

  6. 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

  7. The current status of bracing for patients with adolescent idiopathic scoliosis.

    PubMed

    Stokes, O M; Luk, K D K

    2013-10-01

    Adolescent idiopathic scoliosis affects about 3% of children. Non-operative measures are aimed at altering the natural history to maintain the size of the curve below 40° at skeletal maturity. The application of braces to treat spinal deformity pre-dates the era of evidence-based medicine, and there is a paucity of irrefutable prospective evidence in the literature to support their use and their effectiveness has been questioned. This review considers this evidence. The weight of the evidence is in favour of bracing over observation. The most recent literature has moved away from addressing this question, and instead focuses on developments in the design of braces and ways to improve compliance.

  8. Improving perioperative care for adolescent idiopathic scoliosis patients: the impact of a multidisciplinary care approach

    PubMed Central

    Borden, Timothy C; Bellaire, Laura L; Fletcher, Nicholas D

    2016-01-01

    The complex nature of the surgical treatment of adolescent idiopathic scoliosis (AIS) requires a wide variety of health care providers. A well-coordinated, multidisciplinary team approach to the care of these patients is essential for providing high-quality care. This review offers an up-to-date overview of the numerous interventions and safety measures for improving outcomes after AIS surgery throughout the perioperative phases of care. Reducing the risk of potentially devastating and costly complications after AIS surgery is the responsibility of every single member of the health care team. Specifically, this review will focus on the perioperative measures for preventing surgical site infections, reducing the risk of neurologic injury, minimizing surgical blood loss, and preventing postoperative complications. Also, the review will highlight the postoperative protocols that emphasize early mobilization and accelerated discharge.

  9. Improving perioperative care for adolescent idiopathic scoliosis patients: the impact of a multidisciplinary care approach

    PubMed Central

    Borden, Timothy C; Bellaire, Laura L; Fletcher, Nicholas D

    2016-01-01

    The complex nature of the surgical treatment of adolescent idiopathic scoliosis (AIS) requires a wide variety of health care providers. A well-coordinated, multidisciplinary team approach to the care of these patients is essential for providing high-quality care. This review offers an up-to-date overview of the numerous interventions and safety measures for improving outcomes after AIS surgery throughout the perioperative phases of care. Reducing the risk of potentially devastating and costly complications after AIS surgery is the responsibility of every single member of the health care team. Specifically, this review will focus on the perioperative measures for preventing surgical site infections, reducing the risk of neurologic injury, minimizing surgical blood loss, and preventing postoperative complications. Also, the review will highlight the postoperative protocols that emphasize early mobilization and accelerated discharge. PMID:27695340

  10. A novel evaluation index for adolescent idiopathic scoliosis progression measurement and diagnosis.

    PubMed

    Zheng, X; Tang, Y J; Ong, S K; Nee, A Y C

    2014-01-01

    Adolescent idiopathic scoliosis is a trunk deformity of the spine with lateral deviation and rotation in the transverse plane. The most traditional measurement index and parameter for scoliosis diagnosis is the Cobb's angle, which evaluates the curves of the scoliotic spine on the radiographic projection of the trunk. Although this method is widely accepted as the benchmark in scoliosis assessment, it has some limitations and restrictions in practical applications because it is measured on a PA (posterior-anterior or back to front) X-ray image, such as potential harmfulness from radiation exposure and high cost.In this paper, a novel evaluation index for adolescent idiopathic scoliosis measurement and diagnosis is introduced to complement the existing assessment index, such as the Cobb's angle, the differences of shoulder height, etc. The new evaluation index is based on the phenomenon of the tilt and deviation of the vertebras in a scoliotic spine, which forms the tilt angles between each pair of adjacent vertebras.A data sample of 30 X-ray images of scoliotic spines was used in this research to evaluate and examine the usability and validity of the new index. The Cobb's angle and the new index were calculated and compared using the same data sample. The correlation between the Cobb's angle and the index was also determined, and a high correlation is found which demonstrated the usefulness of this proposed index. In this paper, it has been shown that the newly-proposed index has the potential to be used as a tool to support the traditional scoliosis measurement methods.This method can also be generalized on the sagittal plane to define other evaluation indices for assessing the severity of kyphosis and lordosis. The idea of using angular separation to evaluate spinal deformity in multiple planes or in three-dimensional spaces will be discussed in future research.

  11. 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

  12. 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

  13. 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 .

  14. 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

  15. An alternative to a randomised control design for assessing the efficacy and effectiveness of bracing in adolescent idiopathic scoliosis.

    PubMed

    Fong, D Y T; Cheung, K M C; Wong, Y W; Cheung, W Y; Fu, I C Y; Kuong, E E; Mak, K C; To, M; Samartzis, D; Luk, K D K

    2015-07-01

    Randomised controlled trials (RCTs) that assessed the efficacy of bracing for adolescent idiopathic scoliosis have suffered from small sample sizes, low compliance and lack of willingness to participate. The aim of this study was to assess the feasibility of a comprehensive cohort study for evaluating both the efficacy and the effectiveness of bracing in patients with adolescent idiopathic scoliosis. Patients with curves at greater risk of progression were invited to join a randomised controlled trial. Those who declined were given the option to remain in the study and to choose whether they wished to be braced or observed. Of 87 eligible patients (5 boys and 63 girls) identified over one year, 68 (78%) with mean age of 12.5 years (10 to 15) consented to participate, with a mean follow-up of 168 weeks (0 to 290). Of these, 19 (28%) accepted randomisation. Of those who declined randomisation, 18 (37%) chose a brace. Patients who were more satisfied with their image were more likely to choose bracing (Odds Ratio 4.1; 95% confidence interval 1.1 to 15.0; p = 0.035). This comprehensive cohort study design facilitates the assessment of both efficacy and effectiveness of bracing in patients with adolescent idiopathic scoliosis, which is not feasible in a conventional randomised controlled trial.

  16. 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

  17. 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

  18. 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

  19. 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.

  20. 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

  1. 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

  2. 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

  3. Histochemical Analysis of Paraspinal Rotator Muscles From Patients With Adolescent Idiopathic Scoliosis

    PubMed Central

    Wajchenberg, Marcelo; Martins, Delio Eulalio; Luciano, Rafael de Paiva; Puertas, Eduardo Barros; Del Curto, David; Schmidt, Beny; Oliveira, Acary Bulle de Souza; Faloppa, Flavio

    2015-01-01

    Abstract Morphological, biochemical, and histopathological alterations in the paraspinal skeletal muscle of patients with adolescent idiopathic scoliosis (AIS) have been extensively reported. We evaluated rotator muscle fibers from the apex vertebra of AIS patients through histological and immunohistochemical analysis. A population of 21 female AIS patients who underwent corrective surgery between 2010 and 2013 had biopsies taken from the paraspinal muscle in the convex and concave sides of the thoracic curve apical vertebra. Serial sections were stained following routine protocols for hematoxylin and eosin (HE), Sudan red, Gomori trichrome, NADH, ATPase, and cytochrome oxidase. We assessed muscular atrophy and hypertrophy, fatty proliferation, endomysial and perimysial fibrosis, the presence of hyaline fibers, mitochondrial proliferation, muscular necrosis, nuclear centralization, and inflammation. Two independent professionals evaluated the slices. The thoracic curves had an average Cobb angle of 68 degree. Comparative analysis of the concave and convex sides was performed with McNemar test at a significance level of 5%. Results showed significant differences in both endomysial and perimysial fibrosis and fatty involution between the two sides of the apex vertebra. Paraspinal muscles in the concave side of the scoliosis apex had significantly more fibrosis and fatty involution. However, both sides showed signs of myopathy, muscular atrophy due to necrosis, presence of hyaline fibers, and mitochondrial proliferation. PMID:25715269

  4. 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

  5. Natural History of Adolescent Idiopathic Scoliosis in Skeletally Mature Patients: A Critical Review.

    PubMed

    Agabegi, Steven S; Kazemi, Namdar; Sturm, Peter F; Mehlman, Charles T

    2015-12-01

    The surgical treatment of adolescent idiopathic scoliosis is dependent on several factors, including curve type and magnitude, degree of curve progression, skeletal maturity, and other considerations, such as pain and cosmesis. The most common indication for surgery is curve progression. Most authors agree that surgical treatment should be considered in skeletally mature patients with curves > 50° because of the risk of progression into adulthood. Furthermore, most authors would agree that curves measuring < 40° to 45° in skeletally mature patients should be observed. When a skeletally mature patient with a curve measuring between 45° to 55° is presenting to an orthopaedic surgeon, it is not uncommon that the patient has no pain, no progression, and no imbalance. The generally accepted belief has been that curves that reach 50° are likely to progress into adulthood, progressing at a rate of 1° per year, based largely on the Iowa studies. However, the level of evidence for this is relatively weak, and the existing literature is equivocal in supporting the practice of performing surgery on these patients.

  6. 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

  7. Trunk rotation and hip joint range of rotation in adolescent girls with idiopathic scoliosis: does the "dinner plate" turn asymmetrically ?

    PubMed Central

    Kotwicki, Tomasz; Walczak, Agata; Szulc, Andrzej

    2008-01-01

    Background In patients with structural idiopathic scoliosis the body asymmetries involve the pelvis and the lower limbs; they are included in many theories debating the pathogenesis of idiopathic scoliosis. Methods Hip joint range of motion was studied in 158 adolescent girls, aged 10–18 years (mean 14.2 ± 2.0) with structural idiopathic scoliosis of 20–83° of Cobb angle (mean 43.0° ± 14.5°) and compared to 57 controls, sex and age matched. Hip range of rotation was examined in prone position, the pelvis level controlled with an inclinometer; hip adduction was tested in five different positions. Results In girls with structural scoliosis the symmetry of hip rotation was less frequent (p = 0.0047), the difference between left and right hip range of internal rotation was significantly higher (p = 0.0013), and the static rotational offset of the pelvis, calculated from the mid-points of rotation, revealed significantly greater (p = 0.0092) than in healthy controls. The detected asymmetries comprised no limitation of hip range of motion, but a transposition of the sector of motion, mainly towards internal rotation in one hip and external rotation in the opposite hip. The data failed to demonstrate the curve type, the Cobb angle, the angle of trunk rotation or the curve progression factor to be related to the hip joint asymmetrical range of motion. Conclusion Numerous asymmetries around the hip were detected, most of them were expressed equally in scoliotics and in controls. Pathogenic implications concern producing a "torsional offset" of muscles patterns of activation around the spine in adolescent girls with structural idiopathic scoliosis during gait. PMID:18205943

  8. 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

  9. 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

  10. 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

  11. The effects of load carriage and bracing on the balance of schoolgirls with adolescent idiopathic scoliosis

    PubMed Central

    Leung, Dawn S. S.; Holmes, Andrew D.

    2007-01-01

    The balance function of children is known to be affected by carriage of a school backpack. Children with adolescent idiopathic scoliosis (AIS) tend to show poorer balance performance, and are typically treated by bracing, which further affects balance. The objective of this study is to examine the combined effects of school backpack carriage and bracing on girls with AIS. A force platform was used to record center of pressure (COP) motion in 20 schoolgirls undergoing thoraco-lumbar-sacral orthosis (TLSO brace) treatment for AIS. COP data were recorded with and without brace while carrying a backpack loaded at 0, 7.5, 10, 12.5 and 15% of the participant’s bodyweight (BW). Ten participants stood on a solid base and ten stood on a foam base, while all participants kept their eyes closed throughout. Sway parameters were analyzed by repeated measures ANOVA. No effect of bracing was found for the participants standing on the solid base, but wearing the brace significantly increased the sway area, displacement and medio-lateral amplitude in the participants standing on the foam base. The medio-lateral sway amplitude of participants standing on the solid base significantly increased with backpack load, whereas significant increases in antero-posterior sway amplitude, sway path length, sway area per second and short term diffusion coefficient were found in participants standing on the foam base. The poorer balance performance exhibited by participants with AIS when visual and somatosensory input is challenged appears to be exacerbated by wearing a TLSO brace, but no interactive effect between bracing and backpack loading was found. PMID:17340156

  12. 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

  13. Usefulness of Simple Rod Rotation to Correct Curve of Adolescent Idiopathic Scoliosis

    PubMed Central

    Kim, Ji Yong; Song, Kyungchul; Kim, Kyung Hyun; Rim, Dae Cheol

    2015-01-01

    Objective To correct apical vertebral rotation for adolescent idiopathic scoliosis (AIS), direct vertebral derotation (DVD) or simple rod rotation (SRR) might be considered. The aim of the present study is to introduce the surgical experiences of AIS by a Korean neurosurgeon and to evaluate the effectiveness of SRR for apical vertebral rotation. Methods A total of 9 patients (1 male and 8 females) underwent scoliosis surgery by a neurosurgeon of our hospital. The Lenke classifications of the patients were 1 of 1B, 2 of 1C, 1 of 2A, 1 of 2C, 3 of 5C and 1 of 6C. Surgery was done by manner of simple rod rotation on the concave side and in situ coronal bending. Coronal Cobb's angles, vertebral rotation angles and SRS-22 were measured on a plain standing X-ray and CT before and after surgery. Results The mean follow up period was 25.7 months (range : 5–52). The mean number of screw positioning level was nine (6–12). The mean age was 16.4 years (range : 13–25) at surgery. The mean Risser grade was 3.7±0.9. The apical vertebral rotation measured from the CT scans was 25.8±8.5° vs. 9.3±6.7° (p<0.001) and the Coronal Cobb's angle was 53.7±10.4° vs. 15.4±6.5° (p<0.001) preoperatively and postoperative, respectively. The SRS-22 improved from 71.9 preoperatively to 90.3 postoperatively. There were no complications related with the operations. Conclusion SRR with pedicle screw instrumentation could be corrected successfully by axial rotation without complications. SRR might serve as a good option to correct AIS deformed curves of AIS. PMID:26819688

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

    PubMed

    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

  15. 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

  16. Surgical treatment of Lenke 5 adolescent idiopathic scoliosis: Comparison of anterior vs posterior approach

    PubMed Central

    Abel, Mark F; Singla, Anuj; Feger, Mark A; Sauer, Lindsay D; Novicoff, Wendy

    2016-01-01

    AIM To compare the posterior vs anterior approaches for fusion of Lenke 5 adolescent idiopathic scoliosis curves, matched for curve magnitude and for the distal level of fixation (dLOF) standardized to the third lumbar vertebrae (L3). METHODS A prospectively collected multicenter database was used for this retrospective comparative study. Our dependent variables included sagittal and coronal radiographic measurements, number of fused vertebrae, estimated blood loss, length of hospitalization and SRS total and individual domain scores at the two-year follow-up. Subject demographics were similar for all group comparisons. Independent t-test was used to compare groups for all analyses at P < 0.01. RESULTS For all matched cases of Lenke 5 curves, a selective approach was used only 50% of the time in cases undergoing a posterior fusion. When comparing a posterior selective approach to an anterior selective approach, surgeons utilizing a posterior approach fused significantly more levels than surgeons using an anterior approach with no other significant differences in radiographic or SRS outcomes (Ant = 4.8 ± 1.0 levels vs post = 6.1 ± 1.0 levels, P < 0.0001). When the dLOF was standardized to L3, the anterior approached provided significantly greater lumbar Cobb percent correction than the posterior approach (Ant = 69.1% ± 12.6% vs post = 54.6% ± 16.4%, P = 0.004), with no other significant radiographic or SRS score differences between approaches. CONCLUSION Surgeons treating Lenke 5c curves with a posterior instrumentation and fusion vs an anterior approach include more motion segments, even with a selective fusion. When controlled for the distal level of fixation, the anterior approach provides greater correction of the thoracolumbar curve.

  17. 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

  18. Surgical treatment of Lenke 5 adolescent idiopathic scoliosis: Comparison of anterior vs posterior approach

    PubMed Central

    Abel, Mark F; Singla, Anuj; Feger, Mark A; Sauer, Lindsay D; Novicoff, Wendy

    2016-01-01

    AIM To compare the posterior vs anterior approaches for fusion of Lenke 5 adolescent idiopathic scoliosis curves, matched for curve magnitude and for the distal level of fixation (dLOF) standardized to the third lumbar vertebrae (L3). METHODS A prospectively collected multicenter database was used for this retrospective comparative study. Our dependent variables included sagittal and coronal radiographic measurements, number of fused vertebrae, estimated blood loss, length of hospitalization and SRS total and individual domain scores at the two-year follow-up. Subject demographics were similar for all group comparisons. Independent t-test was used to compare groups for all analyses at P < 0.01. RESULTS For all matched cases of Lenke 5 curves, a selective approach was used only 50% of the time in cases undergoing a posterior fusion. When comparing a posterior selective approach to an anterior selective approach, surgeons utilizing a posterior approach fused significantly more levels than surgeons using an anterior approach with no other significant differences in radiographic or SRS outcomes (Ant = 4.8 ± 1.0 levels vs post = 6.1 ± 1.0 levels, P < 0.0001). When the dLOF was standardized to L3, the anterior approached provided significantly greater lumbar Cobb percent correction than the posterior approach (Ant = 69.1% ± 12.6% vs post = 54.6% ± 16.4%, P = 0.004), with no other significant radiographic or SRS score differences between approaches. CONCLUSION Surgeons treating Lenke 5c curves with a posterior instrumentation and fusion vs an anterior approach include more motion segments, even with a selective fusion. When controlled for the distal level of fixation, the anterior approach provides greater correction of the thoracolumbar curve. PMID:27672568

  19. Impact of Rotation Correction after Brace Treatment on Prognosis in Adolescent Idiopathic Scoliosis

    PubMed Central

    Takigawa, Tomoyuki; Tanaka, Masato; Sugimoto, Yoshihisa; Arataki, Shinya; Ozaki, Toshifumi

    2016-01-01

    Study Design Level 4 retrospective review. Purpose Brace treatment is the standard nonoperative treatment for adolescent idiopathic scoliosis (AIS). Rotation correction is also important, because AIS involves a rotation deformity. The purpose of this study was to evaluate the impact of rotation correction after Osaka Medical College (OMC) brace treatment on clinical outcomes in AIS. Overview of Literature Brace treatment has a significant effect on the progression of AIS. However, few reports have examined rotation correction after brace treatment. Methods A total of 46 patients who wore the OMC brace were retrospectively reviewed. The curve magnitude was determined according to the Cobb method, and the rotation angle of the apical vertebrae was measured by the modified Nash-Moe method. Based on the difference in the rotation angle before and after the initial brace treatment, patients were divided into two groups. Group A (n=33) was defined as no change or improvement of the rotation angle; group B (n=13) was defined as deterioration of the rotation angle. If the patients had curve or rotation progression of 5° or more at skeletal maturity, or had undergone surgery, the treatment was considered a failure. Results Differences of rotation angle between before and after the initial brace treatment were 2°±2° in group A and –3°±2° in group B (p<0.001). The rates of treatment failure were 42% in group A and 77% in group B (p<0.05). This study included 25 patients with Lenke type 1 (54%). Group A (24%) with Lenke type 1 also had a significantly better success rate of brace treatment than group B (75%) (p<0.05). Conclusions Insufficient rotation correction increased brace treatment failure. Better rotation correction resulted in a higher success rate of brace treatment in patients with Lenke type 1. PMID:27790317

  20. 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

  1. 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

  2. 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

  3. The effect of an exercise program on change in curve in adolescents with minimal idiopathic scoliosis. A preliminary study.

    PubMed

    Stone, B; Beekman, C; Hall, V; Guess, V; Brooks, H L

    1979-06-01

    Forty-two adolescents with minimal idiopathic scoliosis, who had been on an exercise program for 9 to 15 months, were evaluated to determine the influence of exercise on change in their curvatures. A difference of 4 degrees or greater between initial and final curve measurements was considered to be a change. Five percent of the curves increased, 74% remained the same, and 21% decreased. Change in curvature for these patients was also compared with that of a matched retrospective group of adolescents with scoliosis who had not had the exercise program. No significant difference in change in curve between the two groups was found. For patients who had been on the exercise program, there was no significant relationship between change in curve and extent of physical activity or between change in curve and exercise recall, correct performance, or frequency. Limitations in the study design and possible explanations for the results are discussed.

  4. Natural history of adolescent idiopathic scoliosis: a tool for guidance in decision of surgery of curves above 50°.

    PubMed

    Danielsson, Aina J

    2013-02-01

    The purpose of this lecture was to give an overview of the natural history of adolescent idiopathic scoliosis (AIS), in order to serve as guidance in the decision of performing surgery or not for the specific patient with AIS. A literature review was performed. Studies concerning long-term outcome in patients with adolescent idiopathic scoliosis that had received no treatment were used. Outcome in terms of curve size, pulmonary function, back function and quality or life/social life was compared. The literature review showed that single thoracic curves of 50°-75° progress 0.73°/year over a 40-year period. AIS do not result in increased mortality, but pulmonary symptoms may be associated with larger curves. Back pain is more frequent among patients with AIS. No study using modern quality of life questionnaires exists, but for social function, childbearing, and marriage no apparent disadvantageous effects were reported compared to the healthy population. The conclusion is that most individuals with AIS and moderate curve size around maturity function well and lead an acceptable life in terms of work and family. Some patients with larger curves have pulmonary problems, but not to the extent that this affects the life span. This needs to be taken into account when discussing surgery with the individual patient. PMID:24432057

  5. 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

  6. Natural history of adolescent idiopathic scoliosis: a tool for guidance in decision of surgery of curves above 50°.

    PubMed

    Danielsson, Aina J

    2013-02-01

    The purpose of this lecture was to give an overview of the natural history of adolescent idiopathic scoliosis (AIS), in order to serve as guidance in the decision of performing surgery or not for the specific patient with AIS. A literature review was performed. Studies concerning long-term outcome in patients with adolescent idiopathic scoliosis that had received no treatment were used. Outcome in terms of curve size, pulmonary function, back function and quality or life/social life was compared. The literature review showed that single thoracic curves of 50°-75° progress 0.73°/year over a 40-year period. AIS do not result in increased mortality, but pulmonary symptoms may be associated with larger curves. Back pain is more frequent among patients with AIS. No study using modern quality of life questionnaires exists, but for social function, childbearing, and marriage no apparent disadvantageous effects were reported compared to the healthy population. The conclusion is that most individuals with AIS and moderate curve size around maturity function well and lead an acceptable life in terms of work and family. Some patients with larger curves have pulmonary problems, but not to the extent that this affects the life span. This needs to be taken into account when discussing surgery with the individual patient.

  7. Comparative multifactorial analysis of the effects of idiopathic adolescent scoliosis and Scheuermann kyphosis on the self-perceived health status of adolescents treated with brace.

    PubMed

    Korovessis, Panagiotis; Zacharatos, Spyridon; Koureas, Georgios; Megas, Panagiotis

    2007-04-01

    Bracing is the most effective non-operative treatment for mild progressive spinal deformities in adolescence but it has shown a considerable impact on several aspects of adolescents' functioning. This cross-sectional study investigated the self-perceived health status of adolescents with the two most common deformities, treated with body orthosis. Seventy-nine adolescents with spinal deformities (idiopathic adolescent scoliosis, thoracic Scheuermann kyphosis) and 62 adolescents without spinal deformities were asked to complete the Quality of Life profile for Spine Deformities Instrument. This study showed that adolescents with deformities are significantly less likely to have back pain in training than controls, but more likely to have difficulty in forward bending, and in the most common daily activities while in brace. These individuals claim they wake up because of back pain and feel quite nervous with the external appearance of their body. These patients face often problems with their relations with friends, while they reported difficulties in getting up from bed and sleep at night more often than their counterparts without deformities. As they grow older, patients feel increasing ashamed of their body, as they are more concerned about the future effect of the deformity on their body. As the bracing time increases, patients have much more probability than controls to get low back pain. Girls with deformity have a higher probability than boys to get low back pain while working in the house and while training. Individuals with larger spinal curvatures have more difficulties in bending and increased incidence of back pain than their counterparts with smaller curvatures. Psychological reasons associated mainly with relations at school and back pain are the main causes for low compliance in adolescents with spinal deformities treated with body orthosis. Careful instructions for all individuals who will undergo brace therapy, psychological support for all patients

  8. 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

  9. 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

  10. [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

  11. 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

  12. 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.

  13. 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.

  14. Timing of menarche in Chinese girls with and without adolescent idiopathic scoliosis: current results and review of the literature.

    PubMed

    Mao, Sai-Hu; Jiang, Jun; Sun, Xu; Zhao, Qinghua; Qian, Bang-Ping; Liu, Zhen; Shu, Hao; Qiu, Yong

    2011-02-01

    Age at menarche is closely related to scoliosis progression during adolescence. Current data concerning the timing of menarche between scoliotic and non-scoliotic girls in the literature are conflicting, with inconclusive results. The aim of this study was to investigate the distribution difference of age at menarche for adolescent idiopathic scoliosis (AIS) girls and normal control population and to subsequently elucidate the menarche age difference through literature reviewing. Moreover, menarche age of AIS girls with Cobb angle <40°, 40-60°, >60° were compared to estimate its association with curve severity. Menstrual status data were available for 6,376 healthy female adolescents and 2,196 AIS girls. We notice that less than 10% of healthy Chinese girls experienced onset of menses before 11.38 years, and approximately 90% of healthy Chinese girls were menstruating by 13.88 years, with a median age of 12.63 years. As for AIS girls, less than 10% started to menstruate before 11.27 years, and approximately 90% were menstruating by 14.38 years, with a median age of 12.83 years. Average menarche age in AIS (12.83 ± 1.22 years) was significantly later than that of normal control girls (12.63 ± 0.98 years) (p < 0.001). Age at menarche for AIS affected girls was significantly greater than that of normal control girls at 75%, 90% of whom had attained menarche (p = 0.001, p < 0.001). Proportion of girls starting to menstruate after 14 years was significantly higher in AIS population compared with normal controls (16.3 vs. 8.1%, p < 0.001). In addition, AIS girls with Cobb angle >60° experienced onset of menses at an average age of 13.25 years, which was significantly later than AIS girls with Cobb angle <40° (12.81 years, p < 0.05) and marginally significantly later than AIS girls with Cobb angle between 40 and 60° (12.86 years, p = 0.053). In conclusion, a tendency of delayed onset of menarche was observed in Chinese idiopathic scoliotic girls in this large

  15. 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

  16. 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

  17. 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

  18. Gait stability improvement after fusion surgery for adolescent idiopathic scoliosis is influenced by corrective measures in coronal and sagittal planes.

    PubMed

    Paul, Justin C; Patel, Ashish; Bianco, Kristina; Godwin, Ellen; Naziri, Qais; Maier, Stephen; Lafage, Virginie; Paulino, Carl; Errico, Thomas J

    2014-09-01

    To achieve optimal results after fusion for adolescent idiopathic scoliosis (AIS), radiographic parameters must be aligned with motion and performance. The effects of fusion on balance are poorly understood. Center of mass (COM) excursion and instantaneous interaction with center of pressure (COP) provides information about patients' balancing ability during gait. This study investigates the interaction between COM and COP (COM-COP) in AIS patients before and one year after spine fusion and determines what radiographic goals predict restoration of harmonious COM-COP. This was a prospective study that investigated sixteen adolescents with AIS curvature >30˚ requiring surgical correction. Clinical outcomes measures, X-rays, and 3D motion-capture gait analysis were collected. Sagittal and coronal COM and COP offsets and inclination angles were calculated from positional data. COM excursion was calculated as peak COM displacement based on mediolateral and vertical deviation from a line fitted to the patient's path. Radiographic parameters were measured to determine variables predictive of change in COM excursion. Post-operatively, average COM peak displacement decreased (42.6 to 13.1 mm, p=0.001) and COM peak vertical displacement remained unchanged (17.0 to 16.3 mm, p=0.472). COM-COP inclination angles reduced in the coronal, but not sagittal plane. Coronal lower extremity peak inclination angles reduced (8.8˚ to 7.5˚, p=0.025), correlating with C7 plumb-line offset (R=0.581, p=0.018). Thoracic Cobb, thoracic kyphosis, and C7 plumb-line were predictors of change in COM excursion. Mediolateral COM excursion post-surgery may reflect an attempt to reduce kinetic demands with improved spinal alignment. Although AIS correction has historically focused on the coronal plane, sagittal parameters may be more important for motion than previously theorized.

  19. 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.

  20. Association between Common Variants near LBX1 and Adolescent Idiopathic Scoliosis Replicated in the Chinese Han Population

    PubMed Central

    Liang, Guoyan; Liang, Anjing; Ye, Wei; Zhang, Liangming; Sharma, Swarkar; Su, Peiqiang; Huang, Dongsheng

    2013-01-01

    Background Adolescent idiopathic scoliosis (AIS) is one of the most common spinal deformities found in adolescent populations. Recently, a genome-wide association study (GWAS) in a Japanese population indicated that three single nucleotide polymorphisms (SNPs), rs11190870, rs625039 and rs11598564, all located near the LBX1 gene, may be associated with AIS susceptibility [1]. This study suggests a novel AIS predisposition candidate gene and supports the hypothesis that somatosensory functional disorders could contribute to the pathogenesis of AIS. These findings warrant replication in other populations. Methodology/Principal Findings First, we conducted a case-control study consisting of 953 Chinese Han individuals from southern China (513 patients and 440 healthy controls), and the three SNPs were all found to be associated with AIS predisposition. The ORs were observed as 1.49 (95% CI 1.23–1.80, P = 5.09E-5), 1.70 (95% CI 1.42–2.04, P = 1.17E-8) and 1.52 (95% CI 1.27–1.83, P = 5.54E-6) for rs625039, rs11190870 and rs11598564, respectively. Second, a case-only study including a subgroup of AIS patients (N = 234) was performed to determine the effects of these variants on the severity of the condition. However, we did not find any association between these variants and the severity of curvature. Conclusion This study shows that the genetic variants near the LBX1 gene are associated with AIS susceptibility in Chinese Han population. It successfully replicates the results of the GWAS, which was performed in a Japanese population. PMID:23308168

  1. 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

  2. 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

  3. 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

  4. Are adolescent idiopathic scoliosis and ankylosing spondylitis counter-opposing conditions? A hypothesis on biomechanical contributions predisposing to these spinal disorders.

    PubMed

    Masi, A T; Dorsch, J L; Cholewicki, J

    2003-01-01

    Human spinal biomechanics are profoundly complex and not well understood, especially in terms of the dynamic spine function. Translation of biomechanics to disease is difficult, particularly since cause must be separated from effect. Primary dynamics predisposing to the onset of chronic spinal disorders, e.g., adolescent idiopathic scoliosis (AIS) or ankylosing spondylitis (AS), must clearly be differentiated from secondary alterations. This commentary addresses primary biomechanics that may predispose to these idiopathic diseases. A novel hypothesis is proposed, based upon inferences regarding their contrasting muscular dynamics. The hypothesis postulates opposing inherent muscle tonicity in AIS versus AS. Converse degrees of spinal stability may predispose to the respective curvature deformities of AIS and the enthesopathy lesions of AS. One condition is suspected to counter-oppose the other, within a polymorphic spectrum of spinal stability.

  5. Adolescent Idiopathic Scoliosis Treatment Using Pettibon Corrective Procedures: A Case Report

    PubMed Central

    Morningstar, Mark W; Strauchman, Megan N; Gilmour, Greg

    2004-01-01

    Abstract Objective To investigate the possible benefits of using Pettibon corrective procedures to reduce the curvature associated with idiopathic scoliosis. These procedures were tested to determine potential effectiveness in a single patient. Clinical Features A patient with a 35° left convex thoracolumbar scoliosis was treated using Pettibon corrective procedures. Initial and follow-up outcome measures included a Borg pain scale, a Functional Rating Index, a balance test, and radiographic analysis. Intervention and Outcome The patient was treated using a combination of manipulative and rehabilitative procedures designed to restore normal sagittal curves and reduce the severity of the coronal curvatures. After six weeks of treatment, the post treatment radiograph revealed a 20° left convex thoracolumbar scoliosis, as well as decreases in the Borg pain scale from six to two, and Functional Rating Index score from 18/40 to 7/40 after the trial period. Her balance time increased from 18 seconds to 56 seconds. Conclusion Pettibon corrective procedures seemed to be effective at reducing the thoracolumbar scoliosis 15° (43%) after six weeks. The subjective and objective results of this case study warrant further such investigations. PMID:19674630

  6. 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

  7. 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.

  8. 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

  9. 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

  10. Relationship between pulmonary function and degree of spinal deformity, location of apical vertebrae and age among adolescent idiopathic scoliosis patients

    PubMed Central

    Johari, Joehaimey; Sharifudin, Mohd Ariff; Rahman, Azriani Ab; Omar, Ahmad Sabri; Abdullah, Ahmad Tajudin; Nor, Sobri; Lam, Weii Cheak; Yusof, Mohd Imran

    2016-01-01

    INTRODUCTION This retrospective review aimed to examine the relationship between preoperative pulmonary function and the Cobb angle, location of apical vertebrae and age in adolescent idiopathic scoliosis (AIS). To our knowledge, there have been no detailed analyses of preoperative pulmonary function in relation to these three factors in AIS. METHODS A total of 38 patients with thoracic or thoracolumbar scoliosis were included. Curvature of spinal deformity was measured using the Cobb method. Forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) were used to evaluate preoperative pulmonary function. Statistical methods were used to analyse the relationship between preoperative pulmonary function and the factors that may contribute to poor pulmonary function. RESULTS The mean age of the patients was 16.68 ± 6.04 years. An inverse relationship was found between the degree of the Cobb angle and FVC as well as FEV1; however, the relationships were not statistically significant (p = 0.057 and p = 0.072, respectively). There was also a trend towards a significant negative correlation between the thoracic curve and FVC (p = 0.014). Patients with larger thoracic curves had lower pulmonary function. A one-year increase in age significantly decreased FVC by 1.092 units (p = 0.044). No significant relationship between age and preoperative FEV1 was found. The median FVC was significantly higher in patients with affected apical vertebrae located at levels L1–L3 than at T6–T8 or T9–T12 (p = 0.006). CONCLUSION Lung function impairment was seen in more severe spinal deformities, proximally-located curvature and older patients. PMID:26831315

  11. Association of IL-6 and MMP-3 gene polymorphisms with susceptibility to adolescent idiopathic scoliosis: a meta-analysis.

    PubMed

    Zhao, Jian; Yang, Mingyuan; Li, Ming

    2016-09-01

    Recently, several institutions have investigated the associations of MMP-3-1171 5A/6A and IL-6-174-G/C gene polymorphisms with adolescent idiopathic scoliosis (AIS), while reports from different institutions are not consistent. Therefore, we, comprehensively and systematically performed this meta-analysis to detect whether the two gene polymorphisms are correlated with AIS. From January 1994 to October 2015, all case-control studies focussed on the relationship between the two aforementioned gene polymorphisms and the susceptibility to AIS were retrieved from bibliographic databases. A total of 16 articles were found, of which five consisted of 944 cases and 1177 controls, were finally included after being assessed by two reviewers. We calculated the pooled odds ratio (OR) with 95% confidence interval (95% CI) to assess the associations. The pooled data analyses were based on allele contrast, homozygote, heterozygote, dominant and recessive models. Overall, there was no significant association of IL-6-174-G/C gene polymorphism with AIS risk. Significant association was observed in homozygote model of MMP-3-1171-5A/6A gene polymorphism (5A5A versus 6A6A: OR = 1.69, 95% CI = 1.11-2.58, P = 0.02). When stratified into Caucasian and Asian populations, positive association was found in Caucasian population (5A versus 6A: OR = 1.43, 95% CI = 1.11-1.84, P = 0.006; 5A5A versus 6A6A: OR = 1.90, 95% CI = 1.13-3.19, P = 0.015); however, there was no significant association in Asian population. The present study concluded that 5A5A genotype of MMP-3-1171 5A/6A gene polymorphism was associated with AIS, especially in Caucasian population. However, no significant association was detected between IL-6-174-G/C gene polymorphism and AIS. PMID:27659327

  12. 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

  13. 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.

  14. 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

  15. Adolescent idiopathic scoliosis screening for school, community, and clinical health promotion practice utilizing the PRECEDE-PROCEED model

    PubMed Central

    Mirtz, Timothy A; Thompson, Mark A; Greene, Leon; Wyatt, Lawrence A; Akagi, Cynthia G

    2005-01-01

    Background Screening for adolescent idiopathic scoliosis (AIS) is a commonly performed procedure for school children during the high risk years. The PRECEDE-PROCEDE (PP) model is a health promotion planning model that has not been utilized for the clinical diagnosis of AIS. The purpose of this research is to study AIS in the school age population using the PP model and its relevance for community, school, and clinical health promotion. Methods MEDLINE was utilized to locate AIS data. Studies were screened for relevance and applicability under the auspices of the PP model. Where data was unavailable, expert opinion was utilized based on consensus. Results The social assessment of quality of life is limited with few studies approaching the long-term effects of AIS. Epidemiologically, AIS is the most common form of scoliosis and leading orthopedic problem in children. Behavioral/environmental studies focus on discovering etiologic relationships yet this data is confounded because AIS is not a behavioral. Illness and parenting health behaviors can be appreciated. The educational diagnosis is confounded because AIS is an orthopedic disorder and not behavioral. The administration/policy diagnosis is hindered in that scoliosis screening programs are not considered cost-effective. Policies are determined in some schools because 26 states mandate school scoliosis screening. There exists potential error with the Adam's test. The most widely used measure in the PP model, the Health Belief Model, has not been utilized in any AIS research. Conclusion The PP model is a useful tool for a comprehensive study of a particular health concern. This research showed where gaps in AIS research exist suggesting that there may be problems to the implementation of school screening. Until research disparities are filled, implementation of AIS screening by school, community, and clinical health promotion will be compromised. Lack of data and perceived importance by school/community health

  16. 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

  17. 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

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

    PubMed Central

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

    2015-01-01

    Abstract 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

  19. 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

  20. 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

  1. [Orthopedic and surgical treatment of idiopathic scoliosis in childhood and adolescence].

    PubMed

    Vialle, Raphaël

    2006-01-31

    Any evolutive scoliosis must be treated actively. The orthopaedic treatment by brace makes it possible to slow down the spinal deformity evolution. In many cases, physiotherapy could be of great help during the conservative treatment phase. Thus, it is possible to contain the scoliosis progression during the rapid growth phase of the spine, in adolescence. In some patients, spinal deformity can remain moderate, making it possible to keep a satisfying function and balance of the spine in adulthood. Sometimes, the orthopaedic treatment alone is not sufficient and surgery is necessary to provide a well-balanced spine with an acceptable residual spinal deformity. The aims of surgical treatment are to correct spinal deformity by means of vertebral osteosynthesis and to stabilize the final correction by means of bone grafting. These surgical procedures made according to strict rules, give excellent functional results and make it possible to carry out a strictly normal adult life.

  2. "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

  3. Progression of Idiopathic Eruptive Macular Pigmentation in a Girl from Childhood to Adolescence: Case Report and Literature Review.

    PubMed

    Tsai, Wen-Chien; Lan, Jui; Lee, Chih-Hung

    2016-09-01

    A 14-year-old girl developed brownish round macules and patches over the face, trunk, and proximal limbs with extensive progression since she was 7 years old. Electron microscopy examination revealed an increase in the number and maturity of melanosomes in basal and suprabasal keratinocytes, although the number of melanocytes was within the normal range. A diagnosis of idiopathic eruptive macular pigmentation was made. We describe the unusual disease progression of this case of idiopathic eruptive macular pigmentation, which was thought to be self-limited.

  4. Computer-aided design and computer-aided manufacture (CAD/CAM) system for construction of spinal orthosis for patients with adolescent idiopathic scoliosis.

    PubMed

    Wong, M S

    2011-01-01

    ABSTRACT Spinal orthoses are commonly prescribed to patients with moderate adolescent idiopathic scoliosis (AIS) for prevention of further curve deterioration. In conventional manufacturing method, plaster bandages are used to obtain the patient's body contour and then the plaster cast is rectified manually. With computer-aided design and computer-aided manufacture (CAD/CAM) system, a series of automated processes from body scanning to digital rectification and milling of the positive model can be performed in a fast and accurate fashion. The purpose of this manuscript is to introduce the application of CAD/CAM system to the construction of spinal orthosis for patients with AIS. Based on evidence within the literature, CAD/CAM method can achieve similar clinical outcomes but with higher efficiency than the conventional fabrication method. Therefore, CAD/CAM method should be considered a substitute to the conventional method in fabrication of spinal orthoses for patients with AIS.

  5. Posterior spinal fusion for adolescent idiopathic thoracolumbar/lumbar scoliosis: clinical outcomes and predictive radiological factors for extension of fusion distal to caudal end vertebra.

    PubMed

    Roberts, S B; Tsirikos, A I; Subramanian, A S

    2014-08-01

    Clinical, radiological, and Scoliosis Research Society-22 questionnaire data were reviewed pre-operatively and two years post-operatively for patients with thoracolumbar/lumbar adolescent idiopathic scoliosis treated by posterior spinal fusion using a unilateral convex segmental pedicle screw technique. A total of 72 patients were included (67 female, 5 male; mean age at surgery 16.7 years (13 to 23)) and divided into groups: group 1 included 53 patients who underwent fusion between the vertebrae at the limit of the curve (proximal and distal end vertebrae); group 2 included 19 patients who underwent extension of the fusion distally beyond the caudal end vertebra. A mean scoliosis correction of 80% (45% to 100%) was achieved. The mean post-operative lowest instrumented vertebra angle, apical vertebra translation and trunk shift were less than in previous studies. A total of five pre-operative radiological parameters differed significantly between the groups and correlated with the extension of the fusion distally: the size of the thoracolumbar/lumbar curve, the lowest instrumented vertebra angle, apical vertebra translation, the Cobb angle on lumbar convex bending and the size of the compensatory thoracic curve. Regression analysis allowed an equation incorporating these parameters to be developed which had a positive predictive value of 81% in determining whether the lowest instrumented vertebra should be at the caudal end vertebra or one or two levels more distal. There were no differences in the Scoliosis Research Society-22 outcome scores between the two groups (p = 0.17). In conclusion, thoracolumbar/lumbar curves in patients with adolescent idiopathic scoliosis may be effectively treated by posterior spinal fusion using a unilateral segmental pedicle screw technique. Five radiological parameters correlate with the need for distal extension of the fusion, and an equation incorporating these parameters reliably informs selection of the lowest instrumented

  6. Application of 3D rapid prototyping technology in posterior corrective surgery for Lenke 1 adolescent idiopathic scoliosis patients.

    PubMed

    Yang, Mingyuan; Li, Chao; Li, Yanming; Zhao, Yingchuan; Wei, Xianzhao; Zhang, Guoyou; Fan, Jianping; Ni, Haijian; Chen, Ziqiang; Bai, Yushu; Li, Ming

    2015-02-01

    A retrospective study to evaluate the effectiveness of 3-dimensional rapid prototyping (3DRP) technology in corrective surgery for Lenke 1 adolescent idiopathic scoliosis (AIS) patients. 3DRP technology has been widely used in medical field; however, no study has been performed on the effectiveness of 3DRP technology in corrective surgery for Lenke 1 AIS patients. Lenke 1 AIS patients who were preparing to undergo posterior corrective surgery from a single center between January 2010 and January 2012 were included in this analysis. Patients were divided into 2 groups. In group A, 3-dimensional (3D) printing technology was used to create subject-specific spine models in the preoperative planning process. Group B underwent posterior corrective surgery as usual (by free hand without image guidance). Perioperative and postoperative clinical outcomes were compared between 2 groups, including operation time, perioperative blood loss, transfusion volume, postoperative hemoglobin (Hb), postoperative complications, and length of hospital stay. Radiological outcomes were also compared, including the assessment of screw placement, postoperative Cobb angle, coronal balance, sagittal vertical axis, thoracic kyphosis, and lumbar lordosis. Subgroup was also performed according to the preoperative Cobb angle: mean Cobb angle <50° and mean Cobb angle >50°. Besides, economic evaluation was also compared between 2 groups. A total of 126 patients were included in this study (group A, 50 and group B, 76). Group A had significantly shorter operation time, significantly less blood loss and transfusion volume, and higher postoperative Hb (all, P < 0.001). However, no significant differences were observed in complication rate, length of hospital stay, and postoperative radiological outcomes between 2 groups (all, P>0.05). There was also no significant difference in misplacement of screws in total populations (16.90% vs 18.82%, P = 0.305), whereas a low misplacement rate of

  7. Application of 3D Rapid Prototyping Technology in Posterior Corrective Surgery for Lenke 1 Adolescent Idiopathic Scoliosis Patients

    PubMed Central

    Yang, Mingyuan; Li, Chao; Li, Yanming; Zhao, Yingchuan; Wei, Xianzhao; Zhang, Guoyou; Fan, Jianping; Ni, Haijian; Chen, Ziqiang; Bai, Yushu; Li, Ming

    2015-01-01

    Abstract A retrospective study to evaluate the effectiveness of 3-dimensional rapid prototyping (3DRP) technology in corrective surgery for Lenke 1 adolescent idiopathic scoliosis (AIS) patients. 3DRP technology has been widely used in medical field; however, no study has been performed on the effectiveness of 3DRP technology in corrective surgery for Lenke 1 AIS patients. Lenke 1 AIS patients who were preparing to undergo posterior corrective surgery from a single center between January 2010 and January 2012 were included in this analysis. Patients were divided into 2 groups. In group A, 3-dimensional (3D) printing technology was used to create subject-specific spine models in the preoperative planning process. Group B underwent posterior corrective surgery as usual (by free hand without image guidance). Perioperative and postoperative clinical outcomes were compared between 2 groups, including operation time, perioperative blood loss, transfusion volume, postoperative hemoglobin (Hb), postoperative complications, and length of hospital stay. Radiological outcomes were also compared, including the assessment of screw placement, postoperative Cobb angle, coronal balance, sagittal vertical axis, thoracic kyphosis, and lumbar lordosis. Subgroup was also performed according to the preoperative Cobb angle: mean Cobb angle <50° and mean Cobb angle >50°. Besides, economic evaluation was also compared between 2 groups. A total of 126 patients were included in this study (group A, 50 and group B, 76). Group A had significantly shorter operation time, significantly less blood loss and transfusion volume, and higher postoperative Hb (all, P < 0.001). However, no significant differences were observed in complication rate, length of hospital stay, and postoperative radiological outcomes between 2 groups (all, P>0.05). There was also no significant difference in misplacement of screws in total populations (16.90% vs 18.82%, P = 0.305), whereas a low misplacement rate

  8. 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 ...

  9. Idiopathic Gastroparesis

    PubMed Central

    Parkman, Henry P.

    2015-01-01

    Gastroparesis is a chronic symptomatic disorder of the stomach characterized by delayed emptying without evidence of mechanical obstruction. The three main causes of gastroparesis are diabetic, postsurgical, and idiopathic. Idiopathic gastroparesis refers to gastroparesis of unknown cause, that is, not from diabetes, not from prior gastric surgery, and not related to other endocrine, neurologic, rheumatologic causes of gastroparesis. The gastroparesis should not be related to medications that can delay gastric emptying, such as narcotic analgesic or anticholinergic medications. There is overlap in the symptoms of idiopathic gastroparesis and functional dyspepsia. A substantial minority of patients with functional dyspepsia can have delayed gastric emptying, blurring the distinction between idiopathic gastroparesis and functional dyspepsia. Patients with idiopathic gastroparesis often have a constellation of symptoms including nausea, vomiting, early satiety, postprandial fullness, and upper abdominal pain. Although the presentation of idiopathic gastroparesis is relatively similar to diabetic gastroparesis, abdominal pain occurs more often in idiopathic gastroparesis, whereas nausea and vomiting are more severe in diabetic gastroparesis. Treatment may employ agents used for diabetic gastroparesis and functional dyspepsia, including dietary management, prokinetics agents, antiemetic agents, and symptom modulators. Current treatment options do not adequately address clinical need for idiopathic gastroparesis. PMID:25667023

  10. 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

  11. 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

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

    PubMed Central

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

    2016-01-01

    Abstract 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

  13. 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

  14. 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

  15. Health-related quality-of-life scores, spine-related symptoms, and reoperations in young adults 7 to 17 years after surgical treatment of adolescent idiopathic scoliosis.

    PubMed

    Spanyer, Jonathan M; Crawford, Charles H; Canan, Chelsea E; Burke, Lauren O; Heintzman, Sara E; Carreon, Leah Y

    2015-01-01

    The goal of surgical treatment of adolescent idiopathic scoliosis (AIS) is to prevent disability associated with curve progression. Few investigators have considered whether the function of patients with AIS becomes adversely affected by major spine fusion surgery. Tertiary referral center patients (age, 10-17 years) who underwent spinal deformity correction a minimum of 5 years earlier were identified. Scoliosis Research Society-22R (SRS-22R) and Short Form-12 (SF-12) were administered. Data were available for 118 patients. Mean age was 14.1 years at surgery and 26.8 years at follow-up. Mean outcome scores were 50.9 (SF-12 physical composite summary), 49.4 (SF-12 mental composite summary), and 4.0 (SRS-22R total). One hundred patients (85%) were working. Common symptoms included occasional back pain (90, 76%), limited range of motion (52, 44%), activity limitations (54, 46%), waistline imbalance (41, 35%), rib prominence (28, 24%), wound/scar problems (18, 15%), and shortness of breath (18, 15%). Prominent implants were reported by 11 patients (9%). Seven of 14 reoperations were for instrumentation removal. There was a high incidence of occasional back pain and activity complaints after surgery for AIS in our cohort. However, normal SF-12 scores suggested that these symptoms did not lower the patients' general health.

  16. 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

  17. Medically Significant Infections Are Increased in Patients With Juvenile Idiopathic Arthritis Treated With Etanercept: Results From the British Society for Paediatric and Adolescent Rheumatology Etanercept Cohort Study

    PubMed Central

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

    2015-01-01

    Objective The association between anti–tumor necrosis factor therapy and increased rates of infection is widely documented in adults with rheumatoid arthritis. Findings in children with juvenile idiopathic arthritis (JIA) have been less well documented. The aims of this analysis were to compare the rates of medically significant infections (MSIs) in children with JIA treated with etanercept (ETN) versus methotrexate (MTX) and to compare the rates between combination therapy with ETN plus MTX and monotherapy with ETN. Methods A total of 852 ETN‐treated children and 260 MTX‐treated children had been recruited to the British Society for Paediatric and Adolescent Rheumatology Etanercept Cohort Study (BSPAR‐ETN). MSIs included infections that resulted in death or hospitalization or were deemed medically significant by the clinician. This on‐drug analysis followed the patients until the first MSI, treatment discontinuation, the last followup, or death. Cox proportional hazards models, which were adjusted using propensity deciles, were used to compare rates of MSI between cohorts. Sensitivity analyses were conducted specifically with regard to serious infections (SIs), which were defined as those requiring hospitalization or treatment with intravenous antibiotics/antivirals. Results The ETN‐treated cohort was older and had a longer disease duration, but the disease activity was similar between the cohorts. A total of 133 first MSIs were reported (109 with ETN and 24 with MTX). Patients receiving ETN had higher rates of MSI than did the controls (propensity decile adjusted hazard ratio 2.13 [95% confidence interval 1.22–3.74]). The risk of MSI was higher whether patients were receiving combination or monotherapy. Sensitivity analysis showed no between‐group difference in the rate of SIs, which were much less common. Conclusion ETN therapy is associated with an increased risk of MSI; however, this increased risk disappears when considering only SIs, which

  18. Physical activities of Patients with adolescent idiopathic scoliosis (AIS): preliminary longitudinal case-control study historical evaluation of possible risk factors.

    PubMed

    McMaster, Marianne E; Lee, Amanda Jane; Burwell, R Geoffrey

    2015-01-01

    To our knowledge there are no publications that have evaluated physical activities in relation to the etiopathogenesis of adolescent idiopathic scoliosis (AIS) other than sports scolioses. In a preliminary longitudinal case-control study, mother and child were questioned and the children examined by one observer. The aim of the study was to examine possible risk factors for AIS. Two study groups were assessed for physical activities: 79 children diagnosed as having progressive AIS at one spinal deformity centre (66 girls, 13 boys) and a Control Group of 77 school children (66 girls, 11 boys), the selection involving six criteria. A structured history of physical activities was obtained, every child allocated to a socioeconomic group and examined for toe touching. Unlike the Patients, the Controls were not X-rayed and were examined for surface vertical spinous process asymmetry (VSPA). Statistical analyses showed progressive AIS to be positively associated with social deprivation, early introduction to indoor heated swimming pools and ability to toe touch. AIS is negatively associated with participation in dance, skating, gymnastics or karate and football or hockey classes, which might suggest preventive possibilities. There is a significantly increased independent odds of AIS in children who went to an indoor heated swimming pool within the first year of life (odds ratio 3.88, 95% CI 1.77-8.48; p = 0·001). Furthermore fourteen (61%) Controls with VSPA compared with 9 (17%) Controls without VSPA had been introduced to the swimming pool within their first year of life (P < 0.001). Early exposure to indoor heated swimming pools for both AIS and VSPA, suggests that the AIS findings do not result from sample selection. PMID:25866554

  19. Three-Dimensional Spinal Morphology can Differentiate Between Progressive and Non-Progressive Patients With Adolescent Idiopathic Scoliosis at the Initial Presentation.

    PubMed

    Nault, M-L; Mac-Thiong, J-M; Roy-Beaudry, M; Turgeon, I; de Guise, J; Labelle, H; Parent, S

    2014-02-27

    Study Design. This is a prospective case-control study.Objective. The objective of this study was to compare 3D morphologic parameters of the spine at the first visit between a non progressive (NP) and a progressive (P) group of immature adolescent idiopathic scoliosis (AIS).Summary of Background Data. Prediction of curve progression remains challenging in AIS at the first visit. Prediction of progression is based on curve type, curve magnitude and skeletal or chronological age.Methods. A prospective cohort of 134 AIS was followed from skeletal immaturity to maturity (mean 37 months). The first group was made up of AIS patients with a minimum 6° progression of the major curve between the first and last follow up (P) (n=53) and the second group was composed of NP patients that reached maturity with less than 6° progression (n=81). Computerized measurements were undertaken on reconstructed 3D spine radiographs of the first visit. There were 6 categories of measurements: angle of plane of maximum curvature, Cobb angles (kyphosis, lordosis), 3D wedging (apical vertebra, apical disks), rotation (upper and lower junctional vertebra, apical vertebra, thoracolumbar junction), torsion and slenderness (height/width ratio). t tests were also undertaken.Results. There was no statistical difference between the two groups for age and initial Cobb angle. NP presented significant hypokyphosis, and parameters related to rotation presented significant statistical differences between NP and P (plane of maximal curvature, torsion, apical axial rotation). Depth slenderness also presented statistical differences.Conclusion. This study confirms that even at the initial visit, 3D morphologic differences exist between P and NP AIS. It supports the use of 3D reconstructions of the spine in the initial evaluation of AIS to help predict outcome.

  20. 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

  1. Chronic idiopathic nausea.

    PubMed

    Kovacic, Katja; Chelimsky, Gisela

    2014-04-01

    Chronic nausea is a prevalent but poorly described symptom in adolescents. It often co-occurs with other functional gastrointestinal disorders (FGIDs) but may also present in isolation. A multitude of triggers and complex neural pathways underlie the sensation of nausea. These include gastrointestinal and blood-borne insults, dysmotility, vestibular or central nervous system pathways, an aberrant autonomic nervous system, and psychosocial factors. Although clinical algorithms are lacking, diagnosis is typically made on the basis of a thorough clinical history and without extensive testing. Treatment is mainly empiric and may be directed at comorbid symptoms such as migraine, delayed gastric emptying, orthostatic intolerance, and visceral hypersensitivity. Chronic idiopathic nausea is an increasingly prevalent symptom that needs careful clinical assessment and individualized treatment plans.

  2. 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

  3. Pre-Operative Autologous Blood Donation Does Not Affect Pre-Incision Hematocrit in Adolescent Idiopathic Scoliosis Patients. A Retrospective Cohort of a Prospective Randomized Trial

    PubMed Central

    Verma, Kushagra; Peters, Austin; Lonner, Baron S.; Errico, Thomas

    2016-01-01

    Background Pre-donation of autologous blood prior to spine fusion for adolescent idiopathic scoliosis (AIS) has been used in deformity surgery. The effect of pre-donation on pre-operative hematocrit (Hct) remains debated. Multiple factors may influence pre-operative Hct including intravascular volume status, patient factors, and timing of pre-operative blood donation. The purpose of this study was to determine if pre-donation significantly lowers pre-incision Hct in AIS patients. Methods A retrospective cohort study of a Level-1 prospective randomized trial was conducted. 125 patients from the homogeneous population were included. AIS patients undergoing a posterior only spinal fusion for AIS were separated into two groups based on their pre-operative blood donation history. Demographic variables, pre-incision Hct, and transfusion rates were compared between the two groups using the Student's T-test. Results Pre-donation and non pre-donation groups had 28 and 97 patients, respectively. Pre-donation group was 75% female (21F, 7M) and non pre-donation group was 78% female (76F, 21M). There was no difference between pre-donation and non pre-donation groups in mean age (15.6 ± 2.2 vs 14.8 ± 2.2, p = 0.081), BMI (23.1 ± 4.2 vs 21.7 ± 5.3, p = 0.219), and pre-incision Hct (32.8 ± 3.4 vs 33.8 ± 3.1, p = 0.628). The overall transfusion rates were equivalent (32.1± 48.0% vs 25.8 ± 44.0%, p = 0.509), however, the rate of allogenic transfusion for the pre-donation group was significantly lower (3.6 ± 18.9% vs 25.8 ± 44.0%, p = 0.011). Conclusions This study supports the use of pre-donation for AIS, without a significant drop in pre-incision Hct. Patients that donate are also much less likely to be exposed to allogenic blood. There may be a surgeon bias to recommend pre-donation in patients with a larger BMI and older age. Future studies are needed from a larger population of patients including those with non-AIS pathology. Level of evidence: Level III. PMID:27652198

  4. Pre-Operative Autologous Blood Donation Does Not Affect Pre-Incision Hematocrit in Adolescent Idiopathic Scoliosis Patients. A Retrospective Cohort of a Prospective Randomized Trial

    PubMed Central

    Verma, Kushagra; Peters, Austin; Lonner, Baron S.; Errico, Thomas

    2016-01-01

    Background Pre-donation of autologous blood prior to spine fusion for adolescent idiopathic scoliosis (AIS) has been used in deformity surgery. The effect of pre-donation on pre-operative hematocrit (Hct) remains debated. Multiple factors may influence pre-operative Hct including intravascular volume status, patient factors, and timing of pre-operative blood donation. The purpose of this study was to determine if pre-donation significantly lowers pre-incision Hct in AIS patients. Methods A retrospective cohort study of a Level-1 prospective randomized trial was conducted. 125 patients from the homogeneous population were included. AIS patients undergoing a posterior only spinal fusion for AIS were separated into two groups based on their pre-operative blood donation history. Demographic variables, pre-incision Hct, and transfusion rates were compared between the two groups using the Student's T-test. Results Pre-donation and non pre-donation groups had 28 and 97 patients, respectively. Pre-donation group was 75% female (21F, 7M) and non pre-donation group was 78% female (76F, 21M). There was no difference between pre-donation and non pre-donation groups in mean age (15.6 ± 2.2 vs 14.8 ± 2.2, p = 0.081), BMI (23.1 ± 4.2 vs 21.7 ± 5.3, p = 0.219), and pre-incision Hct (32.8 ± 3.4 vs 33.8 ± 3.1, p = 0.628). The overall transfusion rates were equivalent (32.1± 48.0% vs 25.8 ± 44.0%, p = 0.509), however, the rate of allogenic transfusion for the pre-donation group was significantly lower (3.6 ± 18.9% vs 25.8 ± 44.0%, p = 0.011). Conclusions This study supports the use of pre-donation for AIS, without a significant drop in pre-incision Hct. Patients that donate are also much less likely to be exposed to allogenic blood. There may be a surgeon bias to recommend pre-donation in patients with a larger BMI and older age. Future studies are needed from a larger population of patients including those with non-AIS pathology. Level of evidence: Level III.

  5. Natural evolution from idiopathic photosensitive occipital lobe epilepsy to idiopathic generalized epilepsy in an untreated young patient.

    PubMed

    Bonini, Francesca; Egeo, Gabriella; Fattouch, Jinan; Fanella, Martina; Morano, Alessandra; Giallonardo, Anna Teresa; di Bonaventura, Carlo

    2014-04-01

    Idiopathic photosensitive occipital lobe epilepsy (IPOE) is an idiopathic localization-related epilepsy characterized by age-related onset, specific mode of precipitation, occipital photic-induced seizures--frequently consisting of visual symptoms--and good prognosis. This uncommon epilepsy, which usually starts in childhood or adolescence, has rarely been observed in families in which idiopathic generalized epilepsy also affects other members. We describe a nuclear family in which the proband showed electro-clinical features of idiopathic photosensitive occipital lobe epilepsy in childhood, which subsequently evolved into absences and a single generalized tonico-clonic seizure in early adolescence. His mother had features suggestive of juvenile myoclonic epilepsy. This case illustrates a continuum between focal and generalized entities in the spectrum of the so-called idiopathic (genetically determined) epileptic syndromes. PMID:23815968

  6. Natural evolution from idiopathic photosensitive occipital lobe epilepsy to idiopathic generalized epilepsy in an untreated young patient.

    PubMed

    Bonini, Francesca; Egeo, Gabriella; Fattouch, Jinan; Fanella, Martina; Morano, Alessandra; Giallonardo, Anna Teresa; di Bonaventura, Carlo

    2014-04-01

    Idiopathic photosensitive occipital lobe epilepsy (IPOE) is an idiopathic localization-related epilepsy characterized by age-related onset, specific mode of precipitation, occipital photic-induced seizures--frequently consisting of visual symptoms--and good prognosis. This uncommon epilepsy, which usually starts in childhood or adolescence, has rarely been observed in families in which idiopathic generalized epilepsy also affects other members. We describe a nuclear family in which the proband showed electro-clinical features of idiopathic photosensitive occipital lobe epilepsy in childhood, which subsequently evolved into absences and a single generalized tonico-clonic seizure in early adolescence. His mother had features suggestive of juvenile myoclonic epilepsy. This case illustrates a continuum between focal and generalized entities in the spectrum of the so-called idiopathic (genetically determined) epileptic syndromes.

  7. 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

  8. 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.

  9. 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 ...

  10. 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

  11. 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.

  12. [Physiotherapy for juvenile idiopathic arthritis].

    PubMed

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  13. 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

  14. 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' ...

  15. Review of idiopathic pancreatitis

    PubMed Central

    Lee, Jason Kihyuk; Enns, Robert

    2007-01-01

    Recent advances in understanding of pancreatitis and advances in technology have uncovered the veils of idiopathic pancreatitis to a point where a thorough history and judicious use of diagnostic techniques elucidate the cause in over 80% of cases. This review examines the multitude of etiologies of what were once labeled idiopathic pancreatitis and provides the current evidence on each. This review begins with a background review of the current epidemiology of idiopathic pancreatitis prior to discussion of various etiologies. Etiologies of medications, infections, toxins, autoimmune disorders, vascular causes, and anatomic and functional causes are explored in detail. We conclude with management of true idiopathic pancreatitis and a summary of the various etiologic agents. Throughout this review, areas of controversies are highlighted. PMID:18081217

  16. 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 is thought to be an autoimmune illness . This means the body attacks ...

  17. Concepts on the pathogenesis of adolescent idiopathic scoliosis. Bone growth and mass, vertebral column, spinal cord, brain, skull, extra-spinal left-right skeletal length asymmetries, disproportions and molecular pathogenesis.

    PubMed

    Burwell, R Geoffrey; Dangerfield, Peter H; Freeman, Brian J C

    2008-01-01

    There is no generally accepted scientific theory for the causes of adolescent idiopathic scoliosis (AIS). Encouraging advances thought to be related to AIS pathogenesis have recently been made in several fields including anthropometry of bone growth, bone mass, spinal growth modulation, extra-spinal left-right skeletal length asymmetries and disproportions, magnetic resonance imaging of vertebral column, spinal cord, brain, skull, and molecular pathogenesis. These advances are leading to the evaluation of new treatments including attempts at minimally invasive surgery on the spine and peri-apical ribs. Several concepts of AIS are outlined indicating their clinical applications but not their research potential. The concepts, by derivation morphological, molecular and mathematical, are addressed in 15 sections: 1) initiating and progressive factors; 2) relative anterior spinal overgrowth; 3) dorsal shear forces that create axial rotational instability; 4) rotational preconstraint; 5) uncoupled, or asynchronous, spinal neuro-osseous growth; 6) brain, nervous system and skull; 7) a novel neuro-osseous escalator concept based on a putative abnormality of two normal polarized processes namely, a) increasing skeletal dimensions, and b) the CNS body schema - both contained within a neuro-osseous timing of maturation (NOTOM) concept; 8) transverse plane pelvic rotation, skeletal asymmetries and developmental theory; 9) thoraco-spinal concept; 10) origin in contracture at the hips; 11) osteopenia; 12) melatonin deficiency; 13) systemic melatonin-signaling pathway dysfunction; 14) platelet calmodulin dysfunction; and 15) biomechanical spinal growth modulation. From these concepts, a collective model for AIS pathogenesis is formulated. The central concept of this model includes the body schema of the neural systems, widely-studied in adults, that control normal posture and coordinated movements with frames of reference in the posterior parietal cortex. The escalator concept

  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 epilepsy in dogs.

    PubMed

    Thomas, W B

    2000-01-01

    Idiopathic epilepsy is a chronic condition characterized by recurrent seizures for which there is no identifiable cause. It is the most common neurologic disorder in the dog. This article discusses the diagnostic evaluation and rational treatment of dogs with recurrent seizures. Types of seizures, client education, choice of therapy, use of specific drugs, therapeutic monitoring, and nondrug treatments are reviewed.

  20. 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

  1. Idiopathic inflammatory myopathies.

    PubMed

    Dimachkie, Mazen M; Barohn, Richard J

    2012-07-01

    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.

  2. [Idiopathic renal arteriovenous fistula].

    PubMed

    Bennani, S; Ait Bolbarod, A; el Mrini, M; Kadiri, R; Benjelloun, S

    1996-06-01

    The authors report a case of idiopathic renal arteriovenous fistula. The diagnosis was established angiographically in a 24 year old man presenting gross hematuria. Embolization of the fistula was performed. Efficiency of this treatment was appreciated clinically and by duplex renal ultrasonography. The characteristics of renal arteriovenous fistulas are reviewed. PMID:8763700

  3. 18 CFR 3c.2 - Nonpublic information.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 18 Conservation of Power and Water Resources 1 2010-04-01 2010-04-01 false Nonpublic information. 3c.2 Section 3c.2 Conservation of Power and Water Resources FEDERAL ENERGY REGULATORY COMMISSION, DEPARTMENT OF ENERGY GENERAL RULES STANDARDS OF CONDUCT § 3c.2 Nonpublic information. (a) Section 1264(d)...

  4. 18 CFR 3c.2 - Nonpublic information.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 18 Conservation of Power and Water Resources 1 2014-04-01 2014-04-01 false Nonpublic information. 3c.2 Section 3c.2 Conservation of Power and Water Resources FEDERAL ENERGY REGULATORY COMMISSION, DEPARTMENT OF ENERGY GENERAL RULES STANDARDS OF CONDUCT § 3c.2 Nonpublic information. (a) Section 1264(d)...

  5. 18 CFR 3c.2 - Nonpublic information.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 18 Conservation of Power and Water Resources 1 2011-04-01 2011-04-01 false Nonpublic information. 3c.2 Section 3c.2 Conservation of Power and Water Resources FEDERAL ENERGY REGULATORY COMMISSION, DEPARTMENT OF ENERGY GENERAL RULES STANDARDS OF CONDUCT § 3c.2 Nonpublic information. (a) Section 1264(d)...

  6. 18 CFR 3c.2 - Nonpublic information.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 18 Conservation of Power and Water Resources 1 2012-04-01 2012-04-01 false Nonpublic information. 3c.2 Section 3c.2 Conservation of Power and Water Resources FEDERAL ENERGY REGULATORY COMMISSION, DEPARTMENT OF ENERGY GENERAL RULES STANDARDS OF CONDUCT § 3c.2 Nonpublic information. (a) Section 1264(d)...

  7. 18 CFR 3c.2 - Nonpublic information.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 18 Conservation of Power and Water Resources 1 2013-04-01 2013-04-01 false Nonpublic information. 3c.2 Section 3c.2 Conservation of Power and Water Resources FEDERAL ENERGY REGULATORY COMMISSION, DEPARTMENT OF ENERGY GENERAL RULES STANDARDS OF CONDUCT § 3c.2 Nonpublic information. (a) Section 1264(d)...

  8. Idiopathic toe walking.

    PubMed

    Oetgen, Matthew E; Peden, Sean

    2012-05-01

    Toe walking is a bilateral gait abnormality in which a normal heel strike is absent and most weight bearing occurs through the forefoot. This abnormality may not be pathologic in patients aged <2 years, but it is a common reason for referral to an orthopaedic surgeon. Toe walking can be caused by several neurologic and developmental abnormalities and may be the first sign of a global developmental problem. Cases that lack a definitive etiology are categorized as idiopathic. A detailed history, with careful documentation of the developmental history, and a thorough physical examination are required in the child with a primary report of toe walking. Treatment is based on age and the severity of the abnormality. Management includes observation, stretching, casting, bracing, chemodenervation, and surgical lengthening of the gastrocnemius-soleus complex and/or Achilles tendon. An understanding of idiopathic toe walking as well as treatment options and their outcomes can help the physician individualize treatment to achieve optimal results.

  9. [Idiopathic hypercalciuria. Differential diagnosis].

    PubMed

    Cano, F; Rodríguez, E; Delucchi, M A; Wolff, E

    1990-01-01

    In 50 children with hematuria or urolithiasis and idiopathic hypercalciuria, and in 15 control children, urinary calcium/creatinine concentration rates were measured after fasting and after calcium loading. Patients were classified into two groups depending on the results of an orally administered calcium loading test. Children were considered to have absorptive hypercalciuria (42%) when they had low fasting urinary calcium/creatinine concentration ratio (less than 0.21), and a large increase of this index after calcium administration (greater than 0.28). Patients were labeled as renal hypercalciuria (32%) if they had high fasting urinary calcium/creatinine concentration ratio (greater than 0.21), and variable increases of it after calcium overload. A third group of children (26%), were not classifiable by means of this test. Our data support the contention that this simple ambulatory test is very useful in the diagnostic workup of idiopathic hypercalciuria. PMID:2087593

  10. [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.

  11. Juvenile idiopathic arthritis.

    PubMed

    Gowdie, Peter J; Tse, Shirley M L

    2012-04-01

    Juvenile idiopathic arthritis (JIA) encompasses a complex group of disorders with arthritis as a common feature. This article provides the pediatrician with a review of the epidemiology, classification, clinical manifestations, and complications of JIA. It also provides an update on the current understanding of the cause of JIA and recent developments in management and a recent review of the long-term outcome in JIA.

  12. The high energy source 3C 273

    NASA Technical Reports Server (NTRS)

    Vonmontigny, Corinna

    1990-01-01

    The properties of 3C 273 are reviewed and an attempt is made to find an answer to the question why 3C 273 is the only extragalactic source so far, which was detected at energies greater than or equal to 50 MeV.

  13. 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

  14. Idiopathic Gingival Fibromatosis

    PubMed Central

    Nayak, Ullal Anand; Khandelwal, Vishal; Ninave, Nupur

    2011-01-01

    ABSTRACT Idiopathic gingival fibromatosis is a rare heriditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements. This case report gives an overview of gingival fibromatosis in a 11-year-old female patient who presented with generalized gingival enlargement. Based on the history and clinical examination, the diagnosis was made and the enlarged tissue was surgically removed. The patient was being regularly monitored clinically for improvement in her periodontal condition as well as for any recurrence of gingival overgrowth. PMID:27616864

  15. [Idiopathic progressive subglottic stenosis].

    PubMed

    Sittel, C

    2014-07-01

    Idiopathic subglottic stenosis is causing a narrowing of the central airway at the laryngotracheal junction. Etiology is remaining unclear at large. There is a marked preponderance for women in the fertile age, an association to estrogene or progesterone metabolism remains doubtful. Suggested treatment varies from repeated endoscopic interventions to primary open resection. Therapy selection in this heterogeneous condition should be based on the individual patient situation as well as surgeon's expertise. This complex entity is prone to complications and should preferably be managed in a referral center.

  16. Congenital idiopathic clubfoot deformities.

    PubMed

    Kyzer, S P; Stark, S L

    1995-03-01

    Clubfoot is a birth defect that is marked primarily by a deformed talus (ie, ankle) and calcaneous (ie, heel) that give the foot a characteristic "club-like" appearance. In congenital idiopathic clubfoot (ie, talipes equinovarus), the infant's foot points downward (ie, equinus) and turns inward (ie, varus), while the forefoot curls toward the heel (ie, adduction). This congenital disorder has an incidence of 1 in 400 live births, with boys affected twice as often as girls. Unilateral clubfoot is somewhat more common than bilateral clubfoot and may occur as an isolated defect or in association with other disorders (eg, chromosomal aberrations, cerebral palsy, spina bifida, arthrogryposis). Infantile clubfoot deformity is painless and is correctable with early diagnosis and prompt treatment. PMID:7778903

  17. Juvenile idiopathic arthritis.

    PubMed

    Espinosa, Maria; Gottlieb, Beth S

    2012-07-01

    Juvenile idiopathic arthrithis (JIA) is the most common rheumatic disease of childhood.JIA is a chronic disease that is associated with periods of disease flares and periods of disease inactivity.Early, aggressive treatment with nonsteroidal anti-inflammatory drugs, intra-articular corticosteroid injections, or methotrexate, has significantly improved the outcome of most children who have JIA. Biologics have been shown to be both safe and effective for the treatment of more aggressive forms of arthritis and for uveitis. Long-term safety data of biologics is still uncertain. In the near future, it is hoped that genetic testing will allow earlier diagnosis of JIA as well as help predict the disease course of children who have JIA. Genetic analysis also may allow physicians to target therapies more effectively. It is hoped that development of more specific therapies will decrease overall immunosuppression and other associated toxicities.

  18. 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

  19. 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

  20. [Idiopathic intracranial hypertension].

    PubMed

    Sergeev, A V

    2016-01-01

    Idiopathic intracranial hypertension (IIH) is a condition due to high intracranial pressure in the absence of an intracranial mass lesion, venous thrombosis or brain infection. It mostly occurs in young obese females. Currently, the incidence of IIH in obese women is estimated to be 12 per 100,000 people per year. Epidemiological data demonstrate the increase in incidence in this group: 323 cases per 100,000. IIH can cause visual loss in 1-2% of the patients during the year before the diagnosis and beginning of treatment. IIH treatment is a complex multidisciplinary problem that includes a body-mass reduction program, conservative pharmacological treatment, prolonged ophthalmological study and, if necessary, timely neurosurgical treatment.

  1. 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.

  2. 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

  3. Idiopathic pulmonary fibrosis.

    PubMed

    Meltzer, Eric B; Noble, Paul W

    2008-01-01

    Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median

  4. 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 ...

  5. 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

  6. 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

  7. 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

  8. Aetiology of idiopathic granulomatous mastitis

    PubMed Central

    Altintoprak, Fatih; Kivilcim, Taner; Ozkan, Orhan Veli

    2014-01-01

    Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail. PMID:25516860

  9. 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

  10. 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.

  11. 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

  12. Idiopathic necrotising enteritis cases continue.

    PubMed

    2014-09-27

    Cases of idiopathic necrotising enteritis in calves continue Polioencephalitis of unknown cause in lambs Rare types of deformities seen in piglets Colibacillosis in postweaned pigs Rotavirus in gamebirds These are among matters discussed in the Animal Health and Veterinary Laboratories Agency's (AHVLA's) disease surveillance report for June 2014. PMID:25256728

  13. 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

  14. 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

  15. 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).

  16. 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.

  17. 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.

  18. 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

  19. 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

  20. 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.

  1. 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.

  2. 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

  3. Glucocorticoids in juvenile idiopathic arthritis.

    PubMed

    Malattia, Clara; Martini, Alberto

    2014-05-01

    Although the use of corticosteroids in juvenile idiopathic arthritis (JIA) is now much more limited owing to the availability of methotrexate and biological agents, there are clinical scenarios where it is still indicated. For example, corticosteroids may be indicated for intraarticular injections to prevent joint deformities, as a "bridge" drug to relieve symptoms in polyarticular disease while waiting for methotrexate and biologics to exert their full therapeutic effects, and in the treatment of chronic iridocyclitis, macrophage activation syndrome, and systemic JIA, although the advent of interleukin (IL)-1 and IL-6 blockers has greatly reduced the latter indication.

  4. Idiopathic internal mammary artery aneurysm

    PubMed Central

    Heyn, Jens; Zimmermann, Hanna; Klose, Alexander; Luchting, Benjamin; Hinske, Christian; Sadeghi-Azandaryani, Mojtaba

    2014-01-01

    Aneurysms of the internal mammary artery are extremely rare, and their presentation and treatment are variable. Since these aneurysms often tend to rupture and cause haemothorax and life-threatening conditions, the knowledge of secure treatment options is indispensable. We here report the case of an idiopathic internal mammary aneurysm in a 46-year-old man. Open surgical resection of the aneurysm was performed in this case without any complications. The postoperative course was uneventful and the patient was in a good physical condition without any vascular or neurological abnormalities during follow-up. PMID:25452261

  5. Chronic idiopathic pulmonary hilar fibrosis

    PubMed Central

    Yacoub, Magdi H.; Thompson, Vernon C.

    1971-01-01

    Idiopathic pulmonary hilar fibrosis is a condition related to mediastinal fibrosis, characterized by localization of the fibrosing process to one or both pulmonary hila. This results in pulmonary hypertension and bronchial narrowing. Three patients suffering from this disease, in whom the diagnosis has been confirmed by thoracotomy, are reported. The clinical and pathological features are described and previously reported cases are reviewed. The syndrome is classified into two types, according to whether the obstruction affects mainly the pulmonary artery or veins. The disease is a self-limiting one but may lead to organic changes in the lungs causing severe disability. Images PMID:5565782

  6. Vitamin D deficiency in chronic idiopathic urticaria.

    PubMed

    Movahedi, Masoud; Tavakol, Marzieh; Hirbod-Mobarakeh, Armin; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Tavakol, Zahra; Momenzadeh, Kaveh; Nabavi, Mohammad; Dabbaghzade, Abbas; Mosallanejad, Asieh; Rezaei, Nima

    2015-04-01

    Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urticaria. One hundred and fourteen patients with chronic idiopathic urticaria were recruited in this study along with one hundred and eighty seven sex-matched and age-matched healthy volunteers as the control group. For each patient, urticaria activity score was calculated and autologous serum skin test was done. Vitamin D metabolic statue was measured in serum as 25 hydroxyvitamin D using enzyme immunoassay method. Patients with chronic idiopathic urticaria significantly showed lower levels of vitamin D. Vitamin D deficiency was significantly associated with increased susceptibility to chronic idiopathic urticaria. There was a significant positive correlation between vitamin D levels and urticaria activity score. This study showed that patients with chronic idiopathic urticaria had reduced levels of vitamin D, while vitamin D deficiency could increase susceptibility to chronic idiopathic urticaria.

  7. 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

  8. Observation and Early Intervention in Mild Idiopathic Scoliosis via Corrective Exercises in Growing Children.

    PubMed

    Sy, Ng

    2016-01-01

    Idiopathic scoliosis afflicts 2-3% of the population. For mild curvatures, observation is the treatment of choice. Though this passive "wait and see" approach has been used for many years, the practice is inconsistent among different countries. In Anglo-Saxon countries where scoliosis specific exercises are not practised, observation is indicated for curvatures below 25° in growing children and adolescents. In countries, such as France, Germany, Italy and Poland where scoliosis specific corrective exercises are employed, only patients with no signs of maturity and with curvatures below 15° are treated by observation. Patients with curvatures between 15 - 25° are treated by scoliosis specific exercises. In view of the unpredictability of the progression of scoliosis curvatures in immature patients and the lack of knowledge of long term biomechanical repercussions of mild idiopathic scoliosis on lumbar spine and lower extremities, it is proposed that active intervention through scoliosis specific exercises rather than passive observation be employed in the treatment of mild adolescent idiopathic scoliosis.

  9. Idiopathic subglottic stenosis: a familial predisposition.

    PubMed

    Dumoulin, Elaine; Stather, David R; Gelfand, Gary; Maranda, Bruno; Maceachern, Paul; Tremblay, Alain

    2013-03-01

    Idiopathic subglottic stenosis is a narrowing of the trachea at the level of the cricoid cartilage of unknown etiology. It is a rare condition for which the real incidence has never been established owing to the difficulty of making the diagnosis. Although there is a female preponderance, no familial cases have been reported in the literature. We describe two pairs of sisters as well as a mother and daughter presenting with idiopathic subglottic stenosis. All known causes of tracheal stenosis were excluded, including prolonged intubation, surgery, autoimmune and inflammatory disorders, infection and gastroesophageal reflux disease. These are the first cases reported in the literature that suggest a genetic predisposition for idiopathic subglottic stenosis.

  10. 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

  11. Poliovirus protease 3C(pro) kills cells by apoptosis.

    PubMed

    Barco, A; Feduchi, E; Carrasco, L

    2000-01-20

    The tetracycline-based Tet-Off expression system has been used to analyze the effects of poliovirus protease 3C(pro) on human cells. Stable HeLa cell clones that express this poliovirus protease under the control of an inducible, tightly regulated promoter were obtained. Tetracycline removal induces synthesis of 3C protease, followed by drastic morphological alterations and cellular death. Degradation of cellular DNA in nucleosomes and generation of apoptotic bodies are observed from the second day after 3C(pro) induction. The cleavage of poly(ADP-ribose) polymerase, an enzyme involved in DNA repair, occurs after induction of 3C(pro), indicating caspase activation by this poliovirus protease. The 3C(pro)-induced apoptosis is blocked by the caspase inhibitor z-VAD-fmk. Our findings suggest that the protease 3C is responsible for triggering apoptosis in poliovirus-infected cells by a mechanism that involves caspase activation.

  12. 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

  13. Body mass index of girls in health influences menarche and skeletal maturation: a leptin-sympathetic nervous system focus on the trunk with hypothalamic asymmetric dysfunction in the pathogenesis of adolescent idiopathic scoliosis?

    PubMed

    Burwell, R G; Aujla, R K; Kirby, A S; Dangerfield, P H; Moulton, A; Cole, A A; Polak, F J; Pratt, R K; Webb, J K

    2008-01-01

    Lower body mass index (BMI) and lower circulating leptin levels have been reported in girls with AIS. In this paper we evaluate skeletal sizes and asymmetries by higher and lower BMI subsets about the means for each of three groups of girls age 11-18 years: 1) normals, 2) school screening referrals, and 3) preoperative girls. Higher and lower BMI subsets, likely to have separated subjects with higher from those with lower circulating leptin levels, identify: 1) girls with relatively earlier and later menarche; 2) trunk width size greater in the higher than in the lower BMI subset, of all three groups; 3) abnormal upper arm length (UAL) asymmetries (right minus left) in the lower BMI subset of the preoperative girls; and 4) in thoracic AIS of screened and preoperative girls, Cobb angle and apical vertebral rotation each significantly and positively correlate with UAL asymmetry in the lower BMI subset but not in the higher BMI subset. In preoperative girls, the lower BMI subset shows the combination of relatively reduced pelvic width and abnormal UAL asymmetry, suggesting that both are linked to lower circulating leptin levels. An earlier puberty with hormonal changes provides a plausible explanation for the larger trunk width at the shoulders and pelvis especially at the younger ages in the higher BMI subsets. At the shoulders, this widening is driven by the ribcage which, in human evolution was acquired with decoupling of head and trunk movements required for efficient bipedal gait. The UAL asymmetry patterns within the groups and BMI subsets are not explained by hormonal mechanisms. It is hypothesized that 1) normal trunk widening of the thoracic cage by hormones in human adolescence is supplemented via the sympathetic nervous system under leptin-hypothalamic control influenced by energy stores (metabolic fuel); and 2) hypothalamic dysfunction with altered hypothalamic sensitivity to leptin through a SNS-driven asymmetric effect may create skeletal length

  14. Hodgkin's disease presenting as idiopathic thrombocytopenic purpura.

    PubMed Central

    Murphy, W. G.; Allan, N. C.; Perry, D. J.; Stockdill, G.

    1984-01-01

    A case of Hodgkin's disease presenting as idiopathic thrombocytopenic purpura in a 23-year-old male is reported. This is a rare presentation of Hodgkin's disease having been previously described in only two cases. PMID:6541338

  15. 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): ...

  16. Epigenomics of idiopathic pulmonary fibrosis.

    PubMed

    Yang, Ivana V

    2012-04-01

    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 nd future directions in this field.

  17. 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

  18. 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

  19. 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

  20. 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

  1. 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

  2. Idiopathic Adult Growth Hormone Deficiency

    PubMed Central

    2013-01-01

    GH secretion is controlled by hypothalamic as well as intrapituitary and peripheral signals, all of which converge upon the somatotroph, resulting in integrated GH synthesis and secretion. Enabling an accurate diagnosis of idiopathic adult GH deficiency (IAGHD) is challenged by the pulsatility of GH secretion, provocative test result variability, and suboptimal GH assay standardization. The spectrum between attenuated GH secretion associated with the normal aging process and with obesity and truly well-defined IAGHD is not distinct and may mislead the diagnosis. Adult-onset GHD is mainly caused by an acquired pituitary deficiency, commonly including prior head/neck irradiation, or an expanding pituitary mass causing functional somatotroph compression. To what extent rare cryptic causes account for those patients seemingly classified as IAGHD is unclear. About 15% of patients with adult GHD and receiving GH replacement in open-label surveillance studies are reported as being due to an idiopathic cause. These patients may also reflect a pool of subjects with an as yet to be determined occult defect, or those with unclear or incomplete medical histories (including forgotten past sports head injury or motor vehicle accident). Therefore, submaximal diagnostic evaluation likely leads to an inadvertent diagnosis of IAGHD. In these latter cases, adherence to rigorous biochemical diagnostic criteria and etiology exclusion may result in reclassification of a subset of these patients to a distinct known acquired etiology, or as GH-replete. Accordingly, rigorously verified IAGHD likely comprises less than 10% of adult GHD patients, an already rare disorder. Regardless of etiology, patients with adult GHD, including those with IAGHD, exhibit a well-defined clinical phenotype including increased fat mass, loss of lean muscle mass, decreased bone mass, and enhanced cardiac morbidity. Definition of unique efficacy and dosing parameters for GH replacement and resultant therapeutic

  3. Ultrastructural differences between diabetic and idiopathic gastroparesis

    PubMed Central

    Faussone-Pellegrini, Maria Simonetta; Grover, Madhusudan; Pasricha, Pankaj J; Bernard, Cheryl E; Lurken, Matthew S; Smyrk, Thomas C; Parkman, Henry P; Abell, Thomas L; Snape, William J; Hasler, William L; Ünalp-Arida, Aynur; Nguyen, Linda; Koch, Kenneth L; Calles, Jorges; Lee, Linda; Tonascia, James; Hamilton, Frank A; Farrugia, Gianrico

    2012-01-01

    Abstract The ultrastructural changes in diabetic and idiopathic gastroparesis are not well studied and it is not known whether there are different defects in the two disorders. As part of the Gastroparesis Clinical Research Consortium, full thickness gastric body biopsies from 20 diabetic and 20 idiopathic gastroparetics were studied by light microscopy. Abnormalities were found in many (83%) but not all patients. Among the common defects were loss of interstitial cells of Cajal (ICC) and neural abnormalities. No distinguishing features were seen between diabetic and idiopathic gastroparesis. Our aim was to provide a detailed description of the ultrastructural abnormalities, compare findings between diabetic and idiopathic gastroparesis and determine if patients with apparently normal immunohistological features have ultrastructural abnormalities. Tissues from 40 gastroparetic patients and 24 age- and sex-matched controls were examined by transmission electron microscopy (TEM). Interstitial cells of Cajal showing changes suggestive of injury, large and empty nerve endings, presence of lipofuscin and lamellar bodies in the smooth muscle cells were found in all patients. However, the ultrastructural changes in ICC and nerves differed between diabetic and idiopathic gastroparesis and were more severe in idiopathic gastroparesis. A thickened basal lamina around smooth muscle cells and nerves was characteristic of diabetic gastroparesis whereas idiopathic gastroparetics had fibrosis, especially around the nerves. In conclusion, in all the patients TEM showed abnormalities in ICC, nerves and smooth muscle consistent with the delay in gastric emptying. The significant differences found between diabetic and idiopathic gastroparesis offers insight into pathophysiology as well as into potential targeted therapies. PMID:21914127

  4. 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.…

  5. Clinical features of idiopathic megarectum and idiopathic megacolon.

    PubMed Central

    Gattuso, J M; Kamm, M A

    1997-01-01

    BACKGROUND: Dilatation of the rectum and/or colon, in the absence of demonstrable organic disease, is an uncommon and poorly characterised condition. AIMS: To characterise the clinical and diagnostic features, and response to treatment, of patients with idiopathic megarectum (IMR) and idiopathic megacolon (IMC). METHODS: A retrospective review was undertaken of all patients operated on for these conditions over a 23 year period. In addition all patients treated over a three year period were prospectively studied by means of a questionnaire, contrast studies of the upper and lower intestine, spine x rays to exclude spinal dysraphism, anorectal physiological studies, and assessment of clinical outcome. Patients with Hirschsprung's disease and other known causes of gut dilatation were excluded. RESULTS: (i) Retrospective study: Of 63 operated patients, 22 had IMR, 23 had IMR and IMC, and 18 had IMC only. Five patients with IMC had previous sigmoid volvulus, and three had associated non-gastrointestinal congenital abnormalities. Faecal incontinence was always associated with rectal impaction and 14 patients (82%) with IMR alone had had manual disimpaction. (ii) Prospective study: Twenty two patients had IMR, with a median rectal diameter of 10 cm (normal < 6.5 cm). Six patients had IMC and one patient had IMR and IMC. Patients with IMR were significantly (p = 0.0007) younger than patients with IMC. All patients with IMR became symptomatic in childhood, compared with half the patients with IMC who developed symptoms as adults. Patients with IMR all presented with soiling and impaction, compared with patients with IMC whose symptoms were variable and included constipation or increased bowel frequency, pain, and variable need for laxatives. No upper gut dilatation was seen in either group of patients. Spinal dysraphism was seen in two of 18 patients with IMR and two of four with IMC, suggesting extrinsic denervation as a possible cause in a minority. Twelve of 22 patients

  6. Why Are C3-C4 Intermediate Species Rare?

    NASA Astrophysics Data System (ADS)

    Johnson, J. E.; Field, C. B.; Berry, J. A.

    2014-12-01

    While C3-C4 intermediate photosynthesis is thought to represent the evolutionary bridge between C3 and C4 photosynthesis, C3-C4 intermediate species are ecologically rare in comparison to both C3 and C4 species. Here, we report results from a laboratory experiment, field observations, and model simulations that suggest a new explanation for the ecological rarity of C3-C4 intermediate species. In the laboratory experiment, we combined gas exchange and fluorescence to characterize the temperature response of photosynthesis in three closely-related species in the genus Flaveria that are representatives of the C3, C3-C4 intermediate, and C4 photosynthetic pathways. The leaf temperature that maximized the quantum yield for CO2 assimilation (Topt(ΦCO2)) was 24.9 ± 0.7°C in Flaveria robusta (C3), 29.8 ± 1.0°C in F. chloraefolia (C3-C4), and 35.7 ± 0.8°C in F. bidentis (C4), and was linearly related to the temperature sensitivity of the coupling between CO2 assimilation and electron transport (d(ΦCO2/ ΦPSII)/dT)). While F. chloraefolia does not simultaneously occur with F. robusta and F. bidentis in naturally-assembled communities, this C3-C4 intermediate species does occur with other C3 and C4 species. During the growing season in two of these mixed-photosynthetic-type communities, leaf temperatures for F. chloraefolia were similar to the Topt(ΦCO2) determined in the laboratory. A model of maximum potential carbon gain suggests that competitive coexistence of C3, C3-C4 intermediate, and C4 species could be dependent on a temperature regime that highlights the distinct relative advantages of the C3-C4 intermediate pathway. In combination, these results suggest that the relative temperature sensitivity of the C3, C3-C4 intermediate, and C4 photosynthetic pathways combined with environmental variation in temperature may help to explain why C3-C4 intermediate species are generally rare.

  7. 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

  8. Idiopathic Gingival Fibromatosis: Case Report and Its Management

    PubMed Central

    Jaju, Prashant P.; Desai, Ankit; Desai, Rajiv S.; Jaju, Sushma P.

    2009-01-01

    Idiopathic gingival fibromatosis is a rare condition. We present a case of idiopathic gingival fibromatosis with its multidisciplinary approach of management. The clinical, radiographic, and histopathological features have been described in detail. PMID:20339448

  9. Towards Large Area Growth of 3C-SiC

    SciTech Connect

    Vasiliauskas, Remigijus; Liljedahl, Rickard; Syvaejaervi, Mikael; Yakimova, Rositza

    2010-11-01

    In this work we have analyzed the possibility of upscaling the growth of 3C-SiC. The growth was done at different temperatures to find limiting mechanisms of the growth rate and to examine the morphology of grown layers. Coverage by 3C-SiC increases when increasing temperature, however more twins appeared. Activation energy of the growth is 130 kcal/mol--showing that growth rate limiting mechanism is sublimation of the source. We discuss the influence of large area 6H-SiC wafers on the formation of 3C-SiC, in which the change in basal plane orientation could also influence the growth of 3C-SiC.

  10. 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

  11. 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

  12. Diagnosis and classification of idiopathic pulmonary fibrosis.

    PubMed

    Kekevian, Alana; Gershwin, M Eric; Chang, Christopher

    2014-01-01

    Idiopathic pulmonary fibrosis is a difficult disease to diagnose. Idiopathic pulmonary fibrosis is a member of a class of diseases known as idiopathic interstitial pneumonias. Other members include nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. Usual interstitial pneumonia (UIP) is the pathological equivalent of idiopathic pulmonary fibrosis. Prior to 2011, the diagnosis was based on major and minor criteria, but because these criteria were not evidence based, the criteria were modified by consensus from the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. These new criteria now include satisfying three core requirements, including exclusion of other possible cause of interstitial lung disease, specific findings of usual interstitial pneumonia on high resolution computed tomography, and a combination of "possible UIP" findings on high resolution computed tomography and UIP findings on lung biopsy. Idiopathic pulmonary fibrosis is a severe, progressive disease with limited treatment options, and exacerbations are associated with a high degree of morbidity and mortality.

  13. 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 ...

  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. [Helicobacter pylori infection and idiopathic thrombocytopenic purpura].

    PubMed

    Franchini, Massimo; Veneri, Dino

    2005-03-01

    There are increasing data on the association between Helicobacter pylori infection and idiopathic thrombocytopenic purpura and the significant increase in platelet count after bacterial eradication. The aim of this review was to consider the studies so far published on Helicobacter pylori infection and idiopathic thrombocytopenic purpura in order to evaluate a possible correlation between these two conditions. A review of the literature showed that 306 out of the 524 patients investigated (54.8%) were positive for Helicobacter pylori infection and that the bacterium was eradicated in 83% of cases. Eradication therapy was accompanied by a complete or partial platelet response in approximately half the cases. Overall, these data show that Helicobacter pylori eradication in patients with idiopathic thrombocytopenic purpura is effective in increasing platelet count. However, since the studies so far published are few, sometimes controversial and involve small series of patients, further studies on larger numbers of patients with longer followup are needed to confirm these preliminary findings.

  16. Urinary Calcium Excretion After Immobilization and Spinal Fusion in Adolescents

    PubMed Central

    Millard, F. J. C.; Nassim, J. R.; Woollen, J. W.

    1970-01-01

    The effect of prolonged immobilization and spinal fusion on the urinary calcium excretion of adolescents is described. Some patients developed severe hypercalcuria, but there was considerable individual variation. No significant difference was found between the sexes nor between patients with scoliosis due to muscle weakness and idiopathic scoliosis. PMID:5427856

  17. 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.

  18. 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

  19. Idiopathic congenital chylothorax treated with octreotide

    PubMed Central

    Sousa, Paulo Rego; Leitão, Henrique; Camacho, Maria Carmo; Nunes, José Luis

    2010-01-01

    Idiopathic congenital chylothorax is defined as an abnormal accumulation of lymphatic fluid within the pleural space and is a relatively rare condition. It is a cause of progressive respiratory distress with nutritional and immunological consequences. Treatment of congenital chylothorax has been conservative management and cases unresponsive usually require surgery. We report a case of idiopathic congenital chylothorax treated with octreotide (a somatostatin analogue), avoiding surgery after failed conservative medical treatment. The patient promptly improved after initiation of endovenous octreotide treatment (10 µg/kg/h) with no observed side effects. PMID:22750919

  20. 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

  1. 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

  2. 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.

  3. A Case of Idiopathic Thyrotropin (TSH) Deficiency

    PubMed Central

    Ichida, Tatsuya; Kajita, Yoshihiro

    1997-01-01

    The first case of idiopathic thyrotropin (TSH) deficiency in an old woman with thyroid functioning adenoma was reported. She got subtotal thyroidectomy before about four years of her admission to our hospital because of fatigability, puffy face and leg edema. At that time, she had low TSH and free T4 levels despite replacement therapy with desiccated thyroid. No response of only serum TSH after adminstration of combined stimulant containing TRH and repeated TRH suggested the failure of TSH secretion. CT MRI did not show any abnormality. These results indicated that her hypothyroidism was due to acquired idiopathic TSH deficiency. PMID:9159047

  4. A surgical challenge: Idiopathic scrotal elephantiasis

    PubMed Central

    Brotherhood, Hilary Laurel; Metcalfe, Michael; Goldenberg, Larry; Pommerville, Peter; Bowman, Cameran; Naysmith, David

    2014-01-01

    Scrotal elephantiasis is a condition rarely encountered in developed nations. It is endemic in tropical regions due to the presence of filariasis (Wucheria bancrofti). We report 2 cases of idiopathic scrotal elephantiasis in Canadian citizens with no history of travel to endemic filariasis regions, malignancy, surgery or radiation. Both patients underwent complete excision of the involved tissue with reconstruction. We found that for advanced cases of scrotal lymphedema, surgery is currently the only solution. In our cases of advanced idiopathic disease, surgical treatment combining the expertise of a plastic surgeon and a urologist provided a successful functional and cosmetic result. PMID:25132898

  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. Optical variability of PHL 1811 and 3C 273

    NASA Astrophysics Data System (ADS)

    Fan, J. H.; Kurtanidze, O.; Liu, Y.; Yuan, Y. H.; Hao, J. M.; Cai, W.; Xiao, H. B.; Pei, Z. Y.

    2015-03-01

    In this work, we reported the optical photometry monitoring results for two brightest nearby quasars, PHL 1811 and 3C 273 using the ST-6 camera at Abastumani Observatory, Georgia. For PHL 1811, we found 3 microvariability events with time scale of ΔT = 6.0 min. For 3C273, we found that the largest variations are ΔV = 0.369 +/- 0.028 mag, ΔR = 0.495 +/- 0.076 mag, and ΔI = 0.355 +/- 0.009 mag. When periodicity analysis methods are adopted to the available data, a period of p = 5.80 +/- 1.12 years is obtained for PHL 1811, and p = 21.10 +/- 0.14, 10.00 +/- 0.14, 7.30 +/- 0.09, 13.20 +/- 0.09, 2.10 +/- 0.06, and 0.68 +/- 0.05 years are obtained for 3C 273.

  7. 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.

  8. 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

  9. Idiopathic segmental sclerosis of vertebral bodies

    SciTech Connect

    McCarthy, E.F.; Dorfman, H.D.

    1982-12-01

    Five cases of idiopathic vetebral sclerosis are presented. The features of this condition are segmental vertebral sclerosis of a single lumbar vertebra in a young adult without disc space narrowing or alteration of vertebral contour. The differential diagnosis is discussed. Lumbar vertebra biopsies of three patients showed reactive nonspecific osteosclerosis.

  10. 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.

  11. 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

  12. [Idiopathic hypereosinophilic syndrome presenting with oculomotor paresis].

    PubMed

    Rafik, R; Bourazza, A; Karouache, A; Mounach, J; Boutaleb, N; Ouhabi, H; Mossadeq, R

    2004-12-01

    A 42-year-old patient with a known depressive syndrome developed diplopia. The neurological examination revealed a peripheral neurogenic syndrome with incoordination of the left arm. Biological and radiological findings were in agreement with the diagnosis of idiopathic hypereosinophilic syndrome. Treatment with corticosteroids was effective.

  13. 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

  14. 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.

  15. 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.

  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. Connection Between X-Ray Emission and Relativistic Jets in the Radio Galaxies 3C 111 and 3C 120

    NASA Technical Reports Server (NTRS)

    Aller, Margo F.

    2005-01-01

    This work represents a part of a longterm study of the X-ray flux variability in radio galaxies and its relation to flux and structural changes in the associated radio jet. The work described here included: 1) continued study of the emission properties of the FR I radio galaxy 3C 120 known to exhibit a jet/disk connection from our past work; and 2) the commencement of monitoring of a second radio galaxy, the FR I1 object 3C 111 which was selected because of similar radio and X-ray properties to 3C 120, including the presence of Fe K a emission. The association between X-ray dips and new superluminal components, suggesting a picture in which the radio jet is fed by accretion events near the black hole, was identified in 3C 120 using combined RXTE and radio flux monitoring data and bi-monthly to monthly imaging data from the VLBA at 43 GHz. Such data were also obtained for both targets during the period described here. Specific goals were to more broadly investigate the X-ray dip/superluminal connection in 3C 120, thereby determining the epochs of X-ray minima and superluminal ejections more accurately (and hence more precisely determining the distance between the accretion disk and the core of the radio jet), and to determine whether a similar pattern is present in the data for a second radio galaxy. In 3C 111 a different time scale (longer time delays between X-ray dips and superluminal ejections) was expected due to the higher black hole mass implied by its higher radio luminosity: no black hole mass is published for this object but one can be determined from a PDS analysis of the RXTE data. The addition of the second source to the study would identify whether a similar connection was present in other sources and, if found, would provide important information on how time scale (and hence size scale) of accretion disk/jet systems depends on black hole mass. The grant included funding for the reduction and analysis of data obtained during the time period of Rossi

  18. Idiopathic childhood occipital epilepsy of Gastaut: report of 12 patients.

    PubMed

    Wakamoto, Hiroyuki; Nagao, Hideo; Fukuda, Mitsumasa; Watanabe, Shohei; Motoki, Takahiro; Ohmori, Hiromitsu; Ishii, Eiichi

    2011-03-01

    This study sought to present clinical and outcome data of patients with idiopathic childhood occipital epilepsy of Gastaut, to validate previously reported characteristics of this epilepsy. The study group was comprised of 12 affected children (three boys and nine girls), with a median age of onset at 10.3 years. Common ictal manifestations included elementary visual hallucinations (75.0%), blindness or blurring of vision (50.0%), headache (50.0%), and secondarily generalized tonic-clonic seizures (58.3%). Interictal electroencephalography revealed occipital spike-wave paroxysms reactive to eye closure and opening in all patients, accompanied by spike-wave activity in the extra-occipital areas in four (33.3%), and by generalized spike-wave discharges in two (16.7%). One patient exhibited the onset of occipital lobe seizures 1 year after manifesting absence epilepsy. Seizure remission occurred in 81.8% of cases, in half of which medication was discontinued by late adolescence. This study confirmed the previously delineated electroclinical features of epilepsy syndrome, with additional aspects including the frequent association of generalized tonic-clonic seizures and atypical evolution from childhood absence epilepsy.

  19. X-Ray Monitoring of 3C120

    NASA Astrophysics Data System (ADS)

    Miller, Hugh

    The goal of this program is to characterize the variability at both X- ray and optical wavelengths simultaneously for the high luminosity, radio-loud AGN(RLAGN), 3C 120. The key question that is most central to the study of the variability of both RLAGN and radio-quiet AGN(RQAGN) is to identify a ''characteristic timescale", e.g. breaks (''knees") or spikes in the power density spectrum. There are now several RQAGN (e.g., NGC 3516 and Akn 120) for which characteristic timescales (i.e., knee frequencies) have been detected. However there is no RLAGN for which a knee frequency has been observed. The Seyfert-like RLAGN, 3C 120 offers a special opportunity for a possible first detection of a knee frequency in RLAGN.

  20. The double-lobed blazar 3C 371

    NASA Astrophysics Data System (ADS)

    Wrobel, J. M.; Lind, K. R.

    1990-01-01

    High dynamic range VLA imaging reveals, for the first time, that the blazar 3C 371 exhibits very low surface brightness twin radio lobes. These lobes straddle the dominant compact core and have a projected extent of about 42/h kpc. A weak component located in the western lobe shares the morphology, size, and inferred magnetic field configuration of radio source hot spots. A continuous wiggling jet connects this hot spot with the compact core. Double lobes, hot spots, and one-sided jets are traits of extended, edge-brightened radio sources. The discovery of corresponding components in 3C 371's extended emission suggests that this emission is an edge-brightened double, viewed at a large enough angle to the line of sight that the radio lobes do not overlap. A moderate viewing angle is consistent with 3C 371's low bolometric luminosity for a blazar. The diffuse radio halo that surrounds the twin lobes may be related to the fat bridges or bridge distortions shown by edge-brightened doubles with similar radio powers.

  1. Voltammetry of Sc{sub 3}@C{sub 82}

    SciTech Connect

    Anderson, M.R.; Dorn, H.C.; Stevenson, S.; Burbank, P.M.; Gibson, J.R.

    1997-01-15

    This paper describes the first electrochemical data for a polymetallic endohedral metallofullerene. The voltammetric behavior of Sc{sub 3}@C{sub 82} resembles that for La@C{sub 82} and Y@C{sub 82}, indicating that the identity of the metal does not influence dramatically the energies of the metallofullerene molecular orbitals. The three Sc atoms, however, transfer more electrons from the encapsulated metals to the cage than the monometallic counterparts, accounting for the differences in the voltammetry for Sc{sub 3}@C{sub 82}. The similarity of the voltammetry of Sc{sub 3}@C{sub 82} with that of the monometallic fullerenes suggests that Sc{sub 3}{sup +4}@C{sub 82}{sup 4-} may describe the formal charges on the Sc trimer and the C{sub 82} cage of these species. Metallofullerene electronic structure and physical properties should be better understood as more metallofullerenes become available for investigation. 31 refs., 1 fig., 1 tab.

  2. AGN feedback on the ISM of 3C 236

    NASA Astrophysics Data System (ADS)

    Labiano, A.; García-Burillo, S.; Combes, F.; Usero, A.; Soria-Ruiz, R.; Tremblay, G.; Neri, R.; Fuente, A.; Morganti, R.; Oosterloo, T.

    2013-03-01

    We have carried out 1mm/3mm continuum and 12CO(2-1) line high resolution observations to identify the footprints of AGN feedback on 3C 236. The CO emission comes from a spatially resolved disk characterized by a regular rotating pattern. Within the limits imposed by the sensitivity and velocity coverage of our data, we do not detect any outflow signatures in the cold molecular gas. Re-inspection of optical and IR spectra, shows the presence of a previously unknown ionized gas outflow. The star-formation efficiency in 3C 236, is consistent with the value measured in normal galaxies, which follow the canonical Kennicutt-Schmidt law. This result, confirmed to hold in other young radio sources examined in this work, is in stark contrast with the factor of 10-50 lower SFE that has been claimed to characterize evolved powerful radio galaxies. The recent reactivation of the AGN in 3C 236 is a likely explanation for the early evolutionary status of its molecular disk.

  3. The milliarcsecond structure of 3C 273 at 22 GHz

    SciTech Connect

    Zensus, J.A.; Biretta, J.A.; Unwin, S.C.; Cohen, M.H. Owens Valley Radio Observatory, Pasadena, CA )

    1990-12-01

    The first VLBI images at 22 GHz of the jet in the quasar 3C 273 are presented. In addition to the compact core region, two emission regions can be identified with features seen at lower frequencies; they separate from the core with constant speeds of 0.65 + or - 0.09 and 0.92 + or - 0.11 mas/yr, corresponding to apparent superluminal motion of 4.3 + or - 0.3c and 6.1 + or - 0.3c (for Ho = 100 km/s Mpc, qo = 0.5). The core region brightened at about the estimated epoch of zero separation for the latest superluminal component, suggesting a causal relationship. The curved ridge line of the jet smoothly extends inward towards the core, although no pronounced bends in the range of core distance 0.5-2.5 mas are seen. No significant evidence is found against a common path of subsequent superluminal features. An apparent frequency dependence in the position of one superluminal feature tentatively suggests that opacity effects across the jet direction are present. The results are consistent with an interpretation of the superluminal features as shocks in an underlying relativistic flow, although alternative explanations cannot be ruled out. 43 refs.

  4. 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.

  5. 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

  6. 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

  7. 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

  8. 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

  9. Dental manifestation of primary idiopathic hypoparathyroidism.

    PubMed

    Srirangarajan, Sridharan; Satyanarayan, Aparna; Ravindra, Shivamurthy; Thakur, Srinath

    2014-07-01

    Idiopathic hypoparathyroidism (IHP) is a rare endocrinopathic disorder, of idiopathic nature, characterized by a deficiency of parathyroid hormone causing low serum calcium (Ca) and high serum phosphorus concentrations. We present a case report ofa 40-year-old woman with complaint of bleeding gums and tingling sensation in fingers and toes reported to our department in March 2005 with no tangible diagnosis from previous medical examinations. Oral findings included abnormally short roots, cemental hyperplasia, widening of the periodontal ligament space and root resorption. Laboratory investigations revealed a low serum Ca level and along with the clinical findings, a diagnosis of IHP was reached. Oral prophylaxis was performed and Ca supplements were prescribed. We believe that this case report is a first of its kind reporting cemental hyperplasia, altered alveolar bone patterns and periodontal ligament widening in a patient with IHP.

  10. [Pathogenesis of the idiopathic inflammatory myopathies].

    PubMed

    Riebeling-Navarro, Carlos; Nava, Arnulfo

    2009-11-01

    The inflammatory myopathies, commonly described as idiopathic, are a group of acquired diseases characterized by an inflammatory infiltrate of the skeletal muscle. On the basis of clinical and immuno-pathological features, three major diseases can be identified: dermatomiositis (DM), polymyositis (PM) and inclusion body myositis (IBM). Immunopathogenesis mechanisms are crucial for discriminating between the three different subsets of inflammatory myopathies. DM is a complement-mediated microangiopathy affecting skin and muscle. PM and IBM are T cell-mediated disorders, where CD8-positive cytotoxic T cells invade muscle fibres expressing MHC class I antigens. This article summarizes the main immunopathological markers. The impact of this new knowledge must be defined in relation to potential therapeutic targets for idiopathic inflammatory myopathies.

  11. Idiopathic gingival enlargement and its management

    PubMed Central

    Shetty, Arvind K.; Shah, Hardik J.; Patil, Mallika A.; Jhota, Komal N.

    2010-01-01

    Idiopathic gingival enlargement is a proliferative fibrous lesion of the gingival tissue that causes esthetic and functional problems. Both genetically and pharmacologically induced forms of gingival enlargement exist. This case report addresses the diagnosis and treatment of a case of idiopathic gingival enlargement in a 13-year-old female. The patient presented with generalized diffuse gingival enlargement involving the maxillary and mandibular arches extending on buccal and lingual/palatal surfaces and covering incisal / occlusal third of the tooth resulting in difficulty in speech and mastication since last three years. Patient also gave a history of surgical treatment being carried out four years back in upper anterior region suggesting of recurrence. Biopsy report confirmed the diagnosis of gingival hyperplasia. Gingivectomy was carried out in all four quadrants by using four different methods. PMID:21731254

  12. Spontaneous Idiopathic Unilateral Adrenal Haemorrhage (SIAH).

    PubMed

    Naqvi, Syed Ali; Zaman, Shamas; Ahmed, Irfan

    2015-04-01

    Spontaneous Idiopathic Adrenal Haemorrhage (SIAH) is an unusual surgical emergency which can present with life threatening massive retroperitoneal bleeding. Most of the cases reported in the literature are associated with use of anticoagulation or underlying adrenal pathology such as tumors or cysts. Since this clinical entity is uncommon and clinical presentation is very indistinct, the diagnosis can be easily missed and can be challenging for the treating physicians. Nevertheless a raised clinical suspicion coupled with advances in radiological imaging have considerably improved the detection of SIAH in recent times. We report an unusual case of a 20 years old healthy female student who presented to our hospital with sudden onset of abdominal pain and shock. She was diagnosed as a case of massive spontaneous idiopathic unilateral adrenal haemorrhage, unaccompanied by any hematologic disorder, trauma or underlying pathology. Although patient was hemodynamically unstable at presentation, she was resuscitated promptly, investigated appropriately, hence recovered uneventfully with conservative management alone.

  13. "Idiopathic" mental retardation and new chromosomal abnormalities

    PubMed Central

    2010-01-01

    Mental retardation is a heterogeneous condition, affecting 1-3% of general population. In the last few years, several emerging clinical entities have been described, due to the advent of newest genetic techniques, such as array Comparative Genomic Hybridization. The detection of cryptic microdeletion/microduplication abnormalities has allowed genotype-phenotype correlations, delineating recognizable syndromic conditions that are herein reviewed. With the aim to provide to Paediatricians a combined clinical and genetic approach to the child with cognitive impairment, a practical diagnostic algorithm is also illustrated. The use of microarray platforms has further reduced the percentage of "idiopathic" forms of mental retardation, previously accounted for about half of total cases. We discussed the putative pathways at the basis of remaining "pure idiopathic" forms of mental retardation, highlighting possible environmental and epigenetic mechanisms as causes of altered cognition. PMID:20152051

  14. [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 6 weeks duration in patients aged less than 16 years 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

  15. Cognitive function of idiopathic childhood epilepsy

    PubMed Central

    2012-01-01

    Most children with epilepsy are of normal intelligence. However, a significant subset will have temporary or permanent cognitive impairment. Factors that affect cognitive function are myriad and include the underlying neuropathology of the epilepsy, seizures, epileptiform discharges, psychosocial problems, age at seizure onset, duration of epilepsy, and side effects associated with antiepileptic drugs. This review article discusses cognitive function in children with idiopathic epilepsy and the effects of antiepileptic drugs on cognitive function in children. PMID:22670150

  16. Treatment strategies for pediatric idiopathic hypercalciuria.

    PubMed

    Lau, Keith Kwong; Butani, Lavjay

    2009-06-01

    Idiopathic hypercalciuria (IH) is a common metabolic disorder in children and is associated with the development of renal calculi, nephrocalcinosis, hematuria and osteopenia. The effect of various dietary modifications and available pharmacologic therapies on reducing urinary calcium excretion and/or urinary supersaturation is discussed in this article. The importance of a multidisciplinary approach involving the patient, their families, and health-care professionals is also addressed.

  17. Cellular Changes in Diabetic and Idiopathic Gastroparesis

    PubMed Central

    2011-01-01

    Background Cellular changes associated with diabetic and idiopathic gastroparesis are not well described. Aim Describe histologic abnormalities in gastroparesis and compare findings in idiopathic versus diabetic gastroparesis. Methods Full thickness gastric body biopsies were obtained from 40 gastroparetics (20 diabetic) and matched controls. Sections were stained for H&E and trichrome, and immunolabeled with antibodies against PGP 9.5, nNOS, VIP, substance P and tyrosine hydroxylase to quantify nerves, S100β for glia, Kit for interstitial cells of Cajal (ICC), CD45 and CD68, for immune cells and smoothelin for smooth muscle cells. Tissue was also examined by transmission electron microscopy (TEM). Results Histological abnormalities were found in 83% of patients. Most common defects were loss of ICC with remaining ICC showing injury, an abnormal immune infiltrate containing macrophages, and decreased nerve fibers. On light microscopy, no significant differences were found between diabetic and idiopathic gastroparesis with the exception of nNOS expression which was decreased in more idiopathic gastroparetics (40%) compared to diabetic (20%) patients by visual grading. On electron microscopy, a markedly increased connective tissue stroma was present in both disorders. Conclusion This study suggests that on full thickness biopsies, cellular abnormalities are found in the majority of patients with gastroparesis. Most common findings were loss of Kit expression suggesting loss of ICC and an increase in CD45 and CD68 immunoreactivity. These findings suggest that examination of tissue can lead to valuable insights into the pathophysiology of these disorders and offers hope that new therapeutic targets can be found. PMID:21300066

  18. 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

  19. 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.

  20. 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.

  1. SUZAKU OBSERVATION OF THE GIANT RADIO GALAXY 3C 326

    SciTech Connect

    Isobe, Naoki; Tashiro, Makoto S.; Seta, Hiromi; Gandhi, Poshak; Hayato, Asami; Nagai, Hiroshi; Hada, Kazuhiro; Matsuta, Keiko

    2009-11-20

    A Suzaku observation of a giant radio galaxy, 3C 326, which has a physical size of about 2 Mpc, was conducted on 2008 January 19-21. In addition to several X-ray sources, diffuse emission was significantly detected and associated with its west lobe, but the east lobe was contaminated by an unidentified X-ray source WARP J1552.4+2007. After careful evaluation of the X-ray and non-X-ray background, the 0.4-7 keV X-ray spectrum of the west lobe is described by a power-law model modified with the Galactic absorption. The photon index and 1 keV flux density were derived as GAMMA = 1.82{sup +0.26}{sub -0.24} +- 0.04 and S {sub X} = 19.4{sup +3.3}{sub -3.2} +- 3.0 nJy, respectively, where the first and second errors represent the statistical and systematic ones. The diffuse X-rays were attributed to be inverse Compton (IC) radiation by the synchrotron radio electrons scattering off the cosmic microwave background photons. This radio galaxy is the largest among those with lobes detected through IC X-ray emission. A comparison of the radio to X-ray fluxes yields the energy densities of electron and magnetic field as u{sub e} = (2.3 +- 0.3 +- 0.3) x 10{sup -13} erg cm{sup -3} and u{sub m} = (1.2{sup +0.2}{sub -0.1} +- 0.2) x 10{sup -14} erg cm{sup -3}, respectively. The galaxy is suggested to host a low-luminosity nucleus with an absorption-corrected 2-10 keV luminosity of <2 x 10{sup 42} erg s{sup -1}, together with a relatively weak radio core. The energetics in the west lobe of 3C 326 were compared with those of moderate radio galaxies with a size of approx100 kpc. The west lobe of 3C 326 is confirmed to agree with the correlations for the moderate radio galaxies, u{sub e} propor to D {sup -2.2+}-{sup 0.4} and u{sub m} propor to D {sup -2.4+}-{sup 0.4}, where D is their total physical size. This implies that the lobes of 3C 326 are still being energized by the jet, despite the current weakness of the nuclear activity.

  2. Multiwavelength variability analysis of the FSRQ 3C 279

    NASA Astrophysics Data System (ADS)

    Patiño-Álvarez, V.; Chavushyan, V.; León-Tavares, J.; Carramiñana, A.; Carrasco, L.; Fernandes, S.; Schlegel, E. M.; López-Rodríguez, E.

    2015-03-01

    We present a multifrequency analysis of the variability in the flat-spectrum radio quasar 3C 279 from 2008 to 2014. Our multiwavelength dataset includes gamma-ray data from Fermi/LAT (Abdo et al. 2009), observations in 1mm from SMA (Gurwell et al. 2007), Near Infrared from OAGH (Carramiñana & Carrasco 2009) and SMARTS (Bonning et al. 2012); optical V band from the Steward Observatory (Smith et al. 2009) and SMARTS; optical spectra from OAGH (Patiño-Álvarez et al. 2013) and the Steward Observatory; and polarization spectra from the Steward Observatory. The light curves are shown in Fig. 1. Six out of seven optical activity periods identified within our dataset show clear counterparts in mm, NIR and gamma-rays, however, the late 2011 - early 2012 optical flare does not have a counterpart in the GeV regime. In this contribution, we discuss the flaring evolution of 3C 279 and speculate about the production of the anomalous activity period.

  3. Shocked Molecular Gas in the SNR 3C391

    NASA Astrophysics Data System (ADS)

    Bock, D. C.-J.; Wright, M. C. H.; Frail, D. A.; Gaensler, B. M.; Wilner, D. J.

    1999-05-01

    The discovery of OH (1720 MHz) masers in supernova remnants (SNRs) provides us with a convenient method for identifying promising candidates for studies of SNR/molecular cloud interactions. One remnant which is an ideal subject is 3C391. Here, single dish observations reveal a myriad of molecular lines from a giant shocked cloud at the blast-wave of the remnant. We present recent millimeter-wave observations of 3C391 with the Berkeley-Illinois-Maryland Association (BIMA) array. We have imaged the region towards the OH maser in HCN (J=1--0) and HCO+ (J=1--0) at a resolution of 4 arcsec. The morphology and brightness of the emission in HCO+ and HCN are similar; we resolve the shocked cloud seen by Frail and Mitchell (1998) into several clumps of characteristic size 10 arcsec. The maser is midway between two of these clumps, rather than being coincident with the peak column density. The clumps all have similar spectra with line-widths of ~ 30 km s(-1) , although there is evidence for a velocity gradient between the clumps. We examine the physical conditions of the shocked environment and discuss the relative abundances of the molecules. A companion study of the interaction between the SNR W51C and an associated molecular cloud is underway.

  4. 17 CFR 270.3c-4 - Definition of “common trust fund” as used in section 3(c)(3) of the Act.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 17 Commodity and Securities Exchanges 3 2010-04-01 2010-04-01 false Definition of âcommon trust... 1940 § 270.3c-4 Definition of “common trust fund” as used in section 3(c)(3) of the Act. The term common trust fund as used in section 3(c)(3) of the Act (15 U.S.C. 80a-3(c)(3)) shall include a...

  5. 17 CFR 270.3c-3 - Definition of certain terms used in section 3(c)(1) of the Act with respect to certain debt...

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... investment companies. 270.3c-3 Section 270.3c-3 Commodity and Securities Exchanges SECURITIES AND EXCHANGE COMMISSION (CONTINUED) RULES AND REGULATIONS, INVESTMENT COMPANY ACT OF 1940 § 270.3c-3 Definition of certain... 17 Commodity and Securities Exchanges 3 2010-04-01 2010-04-01 false Definition of certain...

  6. 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.

  7. 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.

  8. 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

  9. 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

  10. 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

  11. Anisotropies in the HI gas distribution toward 3C 196

    NASA Astrophysics Data System (ADS)

    Kalberla, P. M. W.; Kerp, J.

    2016-10-01

    Context. The local Galactic Hi gas was found to contain cold neutral medium (CNM) filaments that are aligned with polarized dust emission. These filaments appear to be dominated by the magnetic field and in this case turbulence is expected to show distinct anisotropies. Aims: We use the Galactic Effelsberg-Bonn Hi Survey (EBHIS) to derive 2D turbulence spectra for the Hi distribution in direction to 3C 196 and two more comparison fields. Methods: Prior to Fourier transform we apply a rotational symmetric 50% Tukey window to apodize the data. We derive average as well as position angle dependent power spectra. Anisotropies in the power distribution are defined as the ratio of the spectral power in orthogonal directions. Results: We find strong anisotropies. For a narrow range in position angle, in direction perpendicular to the filaments and the magnetic field, the spectral power is on average more than an order of magnitude larger than parallel. In the most extreme case the anisotropy reaches locally a factor of 130. Anisotropies increase on average with spatial frequency as predicted by Goldreich & Sridhar (1995, ApJ, 438, 763), at the same time the Kolmogorov spectral index remains almost unchanged. The strongest anisotropies are observable for a narrow range in velocity and decay with a power law index close to -8/3, almost identical to the average isotropic spectral index of -2.9 <γ< -2.6. Conclusions: Hi filaments, associated with linear polarization structures in LOFAR observations in direction to 3C 196, show turbulence spectra with marked anisotropies. Decaying anisotropies appear to indicate that we witness an ongoing shock passing the Hi and affecting the observed Faraday depth.

  12. Idiopathic toe walking and sensory processing dysfunction

    PubMed Central

    2010-01-01

    Background It is generally understood that toe walking involves the absence or limitation of heel strike in the contact phase of the gait cycle. Toe walking has been identified as a symptom of disease processes, trauma and/or neurogenic influences. When there is no obvious cause of the gait pattern, a diagnosis of idiopathic toe walking (ITW) is made. Although there has been limited research into the pathophysiology of ITW, there has been an increasing number of contemporary texts and practitioner debates proposing that this gait pattern is linked to a sensory processing dysfunction (SPD). The purpose of this paper is to examine the literature and provide a summary of what is known about the relationship between toe walking and SPD. Method Forty-nine articles were reviewed, predominantly sourced from peer reviewed journals. Five contemporary texts were also reviewed. The literature styles consisted of author opinion pieces, letters to the editor, clinical trials, case studies, classification studies, poster/conference abstracts and narrative literature reviews. Literature was assessed and graded according to level of evidence. Results Only one small prospective, descriptive study without control has been conducted in relation to idiopathic toe walking and sensory processing. A cross-sectional study into the prevalence of idiopathic toe walking proposed sensory processing as being a reason for the difference. A proposed link between ITW and sensory processing was found within four contemporary texts and one conference abstract. Conclusion Based on the limited conclusive evidence available, the relationship between ITW and sensory processing has not been confirmed. Given the limited number and types of studies together with the growing body of anecdotal evidence it is proposed that further investigation of this relationship would be advantageous. PMID:20712877

  13. 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

  14. 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

  15. Adolescent images of adolescence.

    PubMed

    Falchikov, N

    1989-06-01

    This study examines the extent to which a group of Scottish adolescents are influenced by negative images of adolescence present in our culture, and investigates their self-image by means of a Q sort. Forty 15- and 16-year-old school students took part in the study, half of whom were female. Half of the sample were staying on at school to take higher examinations, the other half being school leavers. Eleven factors emerged from the analysis, the first six of which met the criterion that distinguishes common factors. Participants defining common factors were re-interviewed, and their responses to factor interpretations noted. Little evidence was found to suggest that adolescents are influenced by newspaper images. Some descriptions hint at conflict, while others do not. If the results of the present study were to be replicated, a more pluralistic view of adolescence may be required.

  16. Effectiveness of Chêneau brace treatment for idiopathic scoliosis: prospective study in 79 patients followed to skeletal maturity

    PubMed Central

    2011-01-01

    Background Progressive idiopathic scoliosis can negatively influence the development and functioning of 2-3% of adolescents, with health consequences and economic costs, placing the disease in the centre of interest of the developmental medicine. The aim of this study was to evaluate the effectiveness of Chêneau brace in the management of idiopathic scoliosis. Methods A prospective observational study according to SOSORT and SRS recommendations comprised 79 patients (58 girls and 21 boys) with progressive idiopathic scoliosis, treated with Chêneau brace and physiotherapy, with initial Cobb angle between 20 and 45 degrees, no previous brace treatment, Risser 4 or more at the final evaluation and minimum one year follow-up after weaning the brace. Achieving 50° of Cobb angle was considered surgical recommendation. Results At follow-up 20 patients (25.3%) improved, 18 patients (22.8%) were stable, 31 patients (39.2%) progressed below 50 degrees and 10 patients (12.7%) progressed beyond 50 degrees (2 of these 10 patients progressed beyond 60 degrees). Progression concerned the younger and less skeletally mature patients. Conclusion Conservative treatment with Chêneau orthosis and physiotherapy was effective in halting scoliosis progression in 48.1% of patients. The results of this study suggest that bracing is effective in reducing the incidence of surgery in comparison with natural history. PMID:21266084

  17. Idiopathic granulomatous vasculitis: response to immunosuppressive therapy.

    PubMed Central

    Alguacil-Garcia, G F; Moreno-Requena, J; Martinez-Albadalejo, M; Hallal-Hachem, H; Gonzalez-Pina, B; de Paco-Moya, M

    1995-01-01

    A case of idiopathic granulomatous vasculitis (disseminated visceral giant cell arteritis) is described in an old woman, the seventh case of this rare disorder reported to date. The main organ affected was the liver and, to our knowledge, this is the first patient to be diagnosed while still alive and the only case to have received medical treatment. It is also the first time that muscular involvement has been documented in this condition. Cyclophosphamide treatment resulted in disappearance of symptoms and increase in weight. The patient died of an unrelated condition. Images PMID:7665707

  18. 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. PMID:27625576

  19. Idiopathic epilepsy in dogs and cats.

    PubMed

    Thomas, William B

    2010-01-01

    Idiopathic epilepsy is the most common brain disease in dogs and also occurs in cats. Optimal management entails an accurate diagnosis and appropriate drug therapy. In dogs, either phenobarbital or bromide is appropriate as initial therapy. Phenobarbital is the drug of choice for cats. Several other drugs including zonisamide and levetiracetam have the advantage of fewer side effects and are being increasingly used in veterinary medicine. Treatment is successful in most cases, allowing the pet and client to enjoy a good quality of life.

  20. Idiopathic thoracic aortic aneurysm at pediatric age.

    PubMed

    Marín-Manzano, E; González-de-Olano, D; Haurie-Girelli, J; Herráiz-Sarachaga, J I; Bermúdez-Cañete, R; Tamariz-Martel, A; Cuesta-Gimeno, C; Pérez-de-León, J

    2009-03-01

    A 6-year-old-boy presented with epigastric pain and vomiting over 1 year. Chest X-ray and esophagogastric transit showed a mediastinal mass. A chest computerized tomography angiogram demonstrated a descending thoracic aortic aneurysm. Analytical determinations carried out were all negative. The aneurysm was surgically repaired using a Dacron patch. The anatomopathological study described atherosclerotic lesions with calcifications, compatible with an atherosclerotic aneurysm wall. Aneurysms are uncommon in the pediatric population. Usually, no pathogenesis can be determined, and thus, such cases are grouped as idiopathic. Direct repair with or without patch is a therapeutic alternative in pediatric aneurysms and can allow the growth of the aortic circumference.

  1. Idiopathic Hypertrophic Pachymeningitis in the Craniocervical Junction

    PubMed Central

    Bang, Jin Hyuk; Kim, Eo Jin

    2015-01-01

    Idiopathic hypertrophic pachymeningitis (IHP) is a rare disease, and it is characterized by chronic progressive inflammatory fibrosis and thickening of the dura mater with resultant compression of the spinal cord or neural structure without any identifiable cause. It can occur in the intracranial or spinal dura mater alone or as a craniospinal form. The spinal form is rarer than the cranial form and the craniospinal form is extremely rare. We report a rare case of IHP in the craniocervical junction involving both the cranial and spinal dura mater and discuss the diagnosis and management of the disease. PMID:26512276

  2. [Early surgical therapy for idiopathic varicocele].

    PubMed

    Bolla, G; Sartore, G; Cumba, D

    2007-01-01

    The recurring questions about idiopathic varicocele are: a) timing of treatment; b) choice of therapeutical kind; c) influence of treatment on physiopathology of varicocele. In our experience, even if limited, the high ligation of spermatic vessels is simple, safe and effective, even at long term. Fifty-two boys under went surgical therapy at diagnosis--average age 12,9 y. We did not find recurrences of varicocele. Four boys showed postoperative hydrocele; no one required surgical treatment. The postoperative functional valuation, demonstrated a decreased quality of spermatograms in 9.1%. In conclusion, we suggest to early treatment of varicocele by means of a technique effective and devoid of complications.

  3. 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.

  4. Similarities in speech and white matter characteristics in idiopathic developmental stuttering and adult-onset stuttering

    PubMed Central

    Chang, Soo-Eun; Synnestvedt, Anna; Ostuni, John

    2009-01-01

    Adult-onset stuttering (AS) typically occurs following neurological and/or psychological trauma, considered different from developmental stuttering (DS), which starts during early childhood with few if any new cases reported after adolescence. Here we report four cases of AS, two with apparent psychological trigger and two without, none with evidence of neurological injury, and none conforming to previously reported characteristics of psychogenic stuttering. We asked whether this group of AS would have similar speech and neuroanatomical characteristics to those with DS. We conducted blinded analyses of speech samples in both AS cases and 14 cases of DS on type, frequency, and loci of disfluencies. Diffusion tensor imaging (DTI) was conducted to compare white matter tracts using fractional anisotropy (FA). We found that AS did not differ significantly from DS in any of the speech characteristics measured. On DTI, DS had significantly increased FA relative to controls in the right superior longitudinal tract. AS cases showed a similar trend for increases in these regions when compared to controls. The results of this study suggest that symptoms of idiopathic stuttering can begin during adulthood, and that similar neuroanatomical differences from controls may be associated with both developmental and adult onset idiopathic stuttering. PMID:20640049

  5. A Case of Idiopathic Hypereosinophilic Syndrome Causing Mitral Valve Papillary Muscle Rupture

    PubMed Central

    Tamse, Tiffany; Rampersad, Avind; Jordan-Villegas, Alejandro; Ireland, Jill

    2015-01-01

    Idiopathic Hypereosinophilic Syndrome (IHES) is a rare disease that can be difficult to diagnose as the differential is broad. This disease can cause significant morbidity and mortality if left untreated. Our patient is a 17-year-old adolescent female who presented with nonspecific symptoms of abdominal pain and malaise. She was incidentally found to have hypereosinophilia of 16,000 on complete blood count and nonspecific colitis and pulmonary edema on computed tomography. She went into cardiogenic shock due to papillary rupture of her mitral valve requiring extreme life support measures including intubation and extracorporal membrane oxygenation (ECMO) as well as mitral valve replacement. Pathology of the valve showed eosinophilic infiltration as the underlying etiology. The patient was diagnosed with IHES after the exclusion of infectious, rheumatologic, and oncologic causes. She was treated with steroids with improvement of her symptoms and scheduled for close follow-up. In general patients with IHES that have cardiac involvement have poorer prognoses. PMID:26640733

  6. 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

  7. The Aligned z~1 Radio Galaxy 3C 280

    NASA Astrophysics Data System (ADS)

    Ridgway, Susan E.; Stockton, Alan; Lacy, Mark

    2004-01-01

    The z~1 radio galaxy 3C 280 has a particularly striking rest-frame UV morphology, with multiple line and continuum components precisely aligned with the radio structure, including an obvious semicircular arc. Here we explore the nature of these various components by bringing together Hubble Space Telescope and ground-based imaging, ground-based spectroscopy, and radio mapping. From plausible decompositions of the spectra, we show that the continuum of the nuclear component is likely dominated by a combination of nebular thermal continuum, quasar light, and light from old stars. A component that falls directly on the probable path of the radio jet shows mostly nebular thermal continuum and includes contributions from a relatively young stellar population with age around 100 Myr. The arc appears to be completely dominated by line emission and nebular thermal continuum, with no evidence for a significant stellar contribution. Although much of the aligned light is in UV components, the underlying old elliptical galaxy is also well aligned with the radio axis. The elliptical galaxy is well fitted by a de Vaucouleurs profile, probably has a moderately old stellar population (~3 Gyr), and is a massive system with a velocity dispersion of σ~270 km s-1 that implies that it contains a supermassive black hole. Although the arc and the extended emission surrounding the eastern lobe suggest that interactions between the radio lobe and jet must have been important in creating the UV morphology, the ionization and kinematic properties in these components are more consistent with photoionization than shock excitation. 3C 280 may be a transition object between the compact steep-spectrum radio galaxies, which seem to be shock-dominated, and the extended radio sources, which may have evolved past this phase and rarely show shock signatures. Based on observations made with the NASA/ESA Hubble Space Telescope, obtained at the Space Telescope Science Institute, which is operated by the

  8. Clinical course and seizure outcome of idiopathic childhood epilepsy: determinants of early and long-term prognosis

    PubMed Central

    2013-01-01

    Background Idiopathic epilepsies and epileptic syndromes predominate childhood and adolescence epilepsy. The aim of the present study was to investigate the clinical course and outcome of idiopathic childhood epilepsy and identify variables determining both early and long-term prognosis. Methods We followed 303 children with newly diagnosed idiopathic epilepsy aged 1–14 years old, both prospectively and retrospectively. Outcome was defined at one, 2 and 4 years of follow-up, as well as at the end of the study period for all patients. Based on the data collected, patients were classified in four patterns of clinical course: “excellent”, “improving”, “relapsing” and “poor”. Variables defined at intake and after the initial year of treatment were analyzed for their prognostic relevance towards the clinical course and outcome of the patients. Results The mean age at seizure onset was 6,7 years and the mean duration of follow-up was 8,3 years (range 2,0-22,0,SD 4,24). During the initial year of treatment, 70,3% of patients were seizure-free. The course of epilepsy was “excellent” in 53,1% of the subjects, “improving” in 22,8%, “relapsing” in 22,1% whereas only 6 children with idiopathic epilepsy (2%) had a “poor” clinical course exhibiting drug-resistance. After multivariate analysis, variables predictive of a poor initial response to therapy were early seizure onset, multiple seizure types and history of status epilepticus. At the end of follow-up, early response to treatment was of significant positive predictive value, while the presence of multiple seizure types and the history of migraine had a negative impact on prognosis. Conclusions In the vast majority of children, the long-term prognosis of idiopathic epilepsy is favorable. More than half of the patients attain seizure freedom immediately and their clinical course is considered “excellent”. About one fifth exhibit either an improving or a fluctuating course. Early

  9. 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

  10. 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

  11. Idiopathic Fatal Pancytopenia: A Case Report.

    PubMed

    Goyal, Hema; Tilak, Vijai

    2016-06-01

    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

  12. Managing juvenile idiopathic arthritis-associated uveitis.

    PubMed

    Hawkins, Madeleine J; Dick, Andrew D; Lee, Richard J W; Ramanan, Athimalaipet V; Carreño, Ester; Guly, Catherine M; Ross, Adam H

    2016-01-01

    Bilateral chronic anterior uveitis is an extra-articular feature of juvenile idiopathic arthritis. Although figures vary, uveitis occurs in approximately 11%-13% of patients with this disease and is most commonly associated with the female gender, oligoarthritis, and presence of antinuclear antibodies. The disease has an insidious onset and is often asymptomatic. Managing patients with juvenile idiopathic arthritis-associated uveitis remains challenging as the disease may prove to be refractory to traditional treatment regimens. Stepwise immunomodulatory therapy is indicated, with new biologic drugs being used last in cases of refractory uveitis. Small scale studies and practice have provided the evidence to undertake randomized control trials to evaluate the efficacy, safety, and cost-effectiveness of anti-tumor necrosis factor-α therapies, such as infliximab and adalimumab. These have demonstrated promising results, with further data awaited from ongoing trials for adalimumab (as SYCAMORE and ADJUVITE trials). Lower grade evidence is supporting the use of newer biologics such as rituximab, daclizumab, tocilizumab, and abatacept in those cases refractory to anti-tumor necrosis factor-α therapy.

  13. 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.

  14. 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

  15. 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.

  16. 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.

  17. Multispectral Imaging of the Jet in 3C346

    NASA Astrophysics Data System (ADS)

    Smith, E. P.; Gardner, J. P.; Heap, S. R.; Baum, S. A.; O'Dea, C. P.

    1998-12-01

    Much progress has been made towards understanding the physics of extra-galactic jet flow in the past 10 years, through numerical modeling and detailed multi-frequency radio mapping. However, we still admit that basic parameters such as the magnetic field strength, the particle density, the bulk Lorentz factor and jet configuration (e.g., fast inner jet and slower outer sheath), and the nature and need for shocks remain unknown. However, by resolving the jets spectrally and spatially and pushing the observations to the higher energies with sufficient spatial resolution to resolve the cooling scale lengths, we can place unique constraints on the physical composition of, and acceleration mechanisms in, extra-galactic jets. The tightest constraints on the physics of jets come not from radio observations, but from observations in the optical, UV, and x-ray, where the lifetimes of the synchrotron emitting particles are measured in hundreds of years and the particles travel distances of hundreds of parsecs. To this end, we present radio through vacuum ultraviolet (STIS FUV-MAMA) spatially resolved images of the radio jet in 3C346 and use them examine the spatial variation of the spectral indices. Because the lifetime of the synchrotron radiating particles is inversely proportional to the observed frequency we can use the vastly different timescales (and therefore length scales) over which the radio (t ~ 10(5-7) yr.) and ultraviolet (t ~ 10(2-3) yr.) emitting particles cool to begin to place constraints on the magnetic field strengths. Also, the curvature of the spectrum as a function of position can be compared with aging models to constrain the magnetic field strength and to test the hypothesis that the magnetic field in radio jets may be substantially below equipartition (Heinz and Begelman 1997).

  18. Multiwavelength Observations of 3C66A in 2003

    NASA Astrophysics Data System (ADS)

    Boettcher, M.; Joshi, M.; Fossati, G.; Smith, I. A.; Mukherjee, R.; Bramel, D.; Cui, W.; WEBT Collaboration

    2004-08-01

    The radio-selected BL Lac object 3C66A was the target of an intensive multiwavelength observing campaign in the last quarter of 2003 and early 2004. It was monitored by the Whole Earth Blazar Telescope (WEBT) collaboration of optical observers, 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 high-spatial-resolution observations using the VLA are being carried out during the campaign and throughout the year 2004 to follow possible structural changes of the source. 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 hardness - intensity anti-correlation on intraday time scales. The X-ray spectrum is consistent with a power-law with a photon spectral index of ˜ 2.1, indicating that the RXTE energy band might be located right at the intersection of the synchrotron and the high-energy emission components of the broadband spectral energy distribution. No significant flux or spectral variability at X-ray energies was detected. We extracted snapshot spectral energy distributions at various times throughout the campaign, and present first spectral fits to those SEDs. This work was partially supported by NASA RXTE GO grant no. NNG 04GB13G.

  19. Bromide therapy in refractory canine idiopathic epilepsy.

    PubMed

    Podell, M; Fenner, W R

    1993-01-01

    On a retrospective basis, the response to adding chronic oral bromide (BR) to phenobarbital (PB) administration in 23 refractory canine idiopathic epileptics between 1986 and 1991 was studied. The mean age for an observed first seizure was 24 months (range 7 to 72) for all dogs. Thirteen (57%) dogs were males with no breed predisposition observed. All dogs were diagnosed as having idiopathic epilepsy based on normal metabolic and neurologic diagnostic evaluations. Dogs were evaluated before BR therapy for a mean time of 22 months (range 5 to 75 months). Seventeen dogs (74%) received multiple antiepileptic drugs (AEDs) before BR therapy. All animals were maintained on PB at least 4 months before the onset of BR therapy, with a mean trough serum concentration of 37.8 mcg/mL and no improvement in seizure severity or recurrence. Twelve dogs presented with generalized isolated seizures and 11 with generalized cluster seizures (two or more seizures within 24 hours) as their first seizure. The effects of BR therapy were evaluated for a mean time of 15 months (range 4 to 33), with 17 dogs (74%) followed for 12 or more months. The mean BR serum concentration for the 0 to 4 months time period was 117 mg/dL compared with 161 mg/dL for the greater than 4 months period. Overall, response to BR therapy was associated with a reduction in the total number of seizures in 83% of the dogs when compared with their respective pre-BR period. For those followed for 1 year after BR, there was a 53% reduction in the number of seizures compared with the previous 12 months. Furthermore, owners reported a decrease in seizure intensity (65% of dogs) and change to a less severe seizure type (22% of dogs) in those dogs that continued to have seizures. Seizure-free status was obtained in 26% of the dogs with protection continuing up to 31 months in one dog. No correlations could be determined between response to BR and either age of onset of the first seizure or interval from the first AED

  20. 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

  1. [Histopathological classification of idiopathic interstitial pneumonias].

    PubMed

    Povýsil, Ctibor

    2010-01-01

    The classification scheme of interstitial lung diseases has undergone numerous revisions. The criteria for distinguishing seven distinct subtypes of idiopathic interstitial pneumonias are now well defined by consensus in the recently published ATS/ERS classification of these lung diseases. In our present review the histological patterns of the different types are described and the differential diagnosis of idiopathic interstitial pneumonias is discussed. Surgical lung biopsy remains the gold standard for the diagnosis of interstitial pneumonias, and sampling from at least 2 sites is recommended. Video-assisted thoracoscopic surgical biopsy is the preferred method for obtaining lung tissue as this procedure offers a similar yield as an open thoracotomy The most common histological subtype of chronic interstitial lung disease is the usual interstitial pneumonia [UIP] which makes up 47-71% of cases. The key histologic features include patchy subpleural and paraseptal distribution of remodeling lung architecture with dense fibrosis, frequent honeycombing, and large fibroblastic foci. Temporal and spatial heterogeneity are the hallmarks. Nonspecific interstitial pneumonia [NSIP] occurs primarily in middle-aged women who have never smoked, with more than 5-years survival rate in 80% of patients. The major feature of NSIP is a uniform interstitial thickening of alveolar septa by a fibrosing or cellular process. The cardinal histological feature in respiratory bronchiolitis and desquamative pneumonia is an excess of intraalveolar histiocytes. In both patterns, there is variable interstitial fibrosis and chronic inflammation, and a strong association with a history of smoking. Organizing pneumonia (idiopathic bronchiolitis obliterans-organizing pneumonia [BOOP]) is not strictly an interstitial process, because the alveoli and bronchioles are filled by intraluminal polyps of fibroblastic tissue and the expansion of the interstitium is mild. Lymphocytic interstitial pneumonia

  2. 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

  3. 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- ...

  4. Tocilizumab in the treatment of systemic juvenile idiopathic arthritis

    PubMed Central

    Murakami, Miho; Tomiita, Minako; Nishimoto, Norihiro

    2012-01-01

    Systemic juvenile idiopathic arthritis is one of the common rheumatic diseases in childhood and characterized by spiking fever, evanescent skin rash, lymphadenopathy, hepatosplenomegaly, and serositis, in addition to arthritis. Children with systemic juvenile idiopathic arthritis often show growth retardation and developmental abnormality, as well as macrophage activation syndrome, a life-threatening complication. Overproduction of interleukin-6 is pathologically responsible for the systemic inflammatory manifestations and abnormal laboratory results with systemic juvenile idiopathic arthritis. Thus, tocilizumab, a humanized antihuman interleukin-6 receptor antibody, has been developed as a therapeutic agent for the disease. A series of clinical studies have demonstrated the excellent efficacy and safety of tocilizumab for patients with active disease. Tocilizumab was approved for systemic juvenile idiopathic arthritis in Japan in 2008 and in the European Union and the United States in 2011.

  5. 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)

  6. Idiopathic chylothorax and lymphedema in 2 whippet littermates

    PubMed Central

    Schuller, Simone; Le Garrérès, Alain; Remy, Isabelle; Peeters, Dominique

    2011-01-01

    Idiopathic chylothorax and limb edema was diagnosed in two 2-year-old male whippet siblings. The fact that the 2 related animals developed similar clinical signs at a young age may suggest a congenital or hereditary etiology. PMID:22547848

  7. Chronic idiopathic thrombocytopenia treated with immunoglobulin.

    PubMed Central

    Mori, P G; Mancuso, G; del Principe, D; Duse, M; Miniero, R; Tovo, R; Bardare, M; Carnelli, V; de Mattia, D

    1983-01-01

    Twenty five children with chronic idiopathic thrombocytopenic purpura followed from 6-96 months in 7 Italian paediatric departments were treated with high dose immunoglobulin according to a multicentre protocol. Positive responses were observed in 20 of 25 patients (80%) and negative responses in 5 of 25 (20%). On previous steroid treatment 7 of 10 positive responders were steroid resistant and 13 of 15 were steroid dependent. Within four weeks of beginning treatment 16 of 20 patients (80%) relapsed, while 4 of 20 (20%) maintained normal platelet values after 4-12 months' follow up. Statistical analysis of the platelet count on day five of treatment enabled us to divide positive responders into three groups: good, intermediate, and poor. The possible mode of action and clinical application of high dose immunoglobulin are discussed. PMID:6685997

  8. Idiopathic palmar fasciitis with polyarthritis syndrome.

    PubMed

    Sung, Yoon-Kyoung; Park, Moon-Hyang; Yoo, Dae-Hyun

    2006-12-01

    A 31-yr-old Korean woman was presented with 4-month history of bilateral hand swelling and stiffness. On clinical examination, she had a painful synovitis of both hands, wrists, knees and ankles. The radiologic and histological examinations confirmed it with palmar fasciitis and polyarthritis syndrome (PFPAS). PFPAS is an uncommon disorder characterized by progressive flexion contractures of both hands, inflammatory fasciitiis, fibrosis, and a generalized inflammatory arthritis. Although most reported cases of PFPAS have been associated with various malignancies, our patient have not been associated with malignancy during 24 months follow up period from her first symptom onset. Her symptoms were improved with moderate dose of corticosteroid and she is currently taking prednisone 5 mg daily without any evidence for internal malignancy. We present here in a young Korean patient with idiopathic PFPAS who was successfully treated with administration of corticosteroid.

  9. 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

  10. Interstitial Lung Disease in Idiopathic Inflammatory Myopathy

    PubMed Central

    Saketkoo, Lesley Ann; Ascherman, Dana P.; Cottin, Vincent; Christopher-Stine, Lisa; Danoff, Sonye K.; Oddis, Chester V.

    2011-01-01

    The lung is one of the most common extra-muscular targets in idiopathic inflammatory myopathies (IIM) and interstitial lung disease (ILD) is a prevalent and often devastating manifestation of IIM. IIM-associated ILD (IIM-ILD) contributes to nearly 80% of the mortality in IIM with a reported prevalence of 65% of newly diagnosed IIM cases. Although ILD frequently accompanies clinical and laboratory findings of myositis, overt signs of muscle disease may be absent in the setting of significant lung disease. Understanding the varied scope of presentation of these diseases is essential to providing optimal patient care. This review will provide an in depth examination of ILD in IIM both from a rheumatologic and pulmonary perspective and will discuss the scope of disease, presenting features, genetic associations, pathogenesis, diagnosis, radiographic and histopathologic findings, along with biomarker assessment and a rationale for therapeutic intervention. PMID:21941374

  11. 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

  12. 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

  13. Idiopathic focal segmental glomerulosclerosis and HLA antigens.

    PubMed

    Gerbase-DeLima, M; Pereira-Santos, A; Sesso, R; Temin, J; Aragão, E S; Ajzen, H

    1998-03-01

    The objective of the present study was to investigate a possible association between HLA class II antigens and idiopathic focal segmental glomerulosclerosis (FSGS). HLA-A, -B, -DR and -DQ antigens were determined in 19 Brazilian patients (16 white subjects and three subjects of Japanese origin) with biopsy-proven FSGS. Comparison of the HLA antigen frequencies between white patients and white local controls showed a significant increase in HLA-DR4 frequency among FSGS patients (37.7 vs 17.2%, P < 0.05). In addition, the three patients of Japanese extraction, not included in the statistical analysis, also presented HLA-DR4. In conclusion, our data confirm the association of FSGS with HLA-DR4 previously reported by others, thus providing further evidence for a role of genes of the HLA complex in the susceptibility to this disease. PMID:9698788

  14. The prognosis of idiopathic generalized epilepsy.

    PubMed

    Seneviratne, Udaya; Cook, Mark; D'Souza, Wendyl

    2012-12-01

    Prognosis describes the trajectory and long-term outcome of a condition. Most studies indicate a better prognosis in idiopathic generalized epilepsy (IGE) in comparison with other epilepsy syndromes. Studies looking at the long-term outcome of different IGE syndromes are relatively scant. Childhood absence epilepsy appears to have a higher rate of remission compared to juvenile absence epilepsy. In absence epilepsies, development of myoclonus and generalized tonic-clonic seizures predicts lower likelihood of remission. Although most patients with juvenile myoclonic epilepsy (JME) achieve remission on antiepileptic drug therapy, <20% appear to remain in remission without treatment. Data on the prognosis of other IGE syndromes are scarce. There are contradictory findings reported on the value of electroencephalography as a predictor of prognosis. Comparisons are made difficult by study heterogeneity, particularly in methodology and diagnostic criteria.

  15. [Pregnancy and labor in idiopathic thrombocytopenic purpura].

    PubMed

    Tampakoudis, P; Billi, H; Tantanassis, T; Kalachanis, I; Garipidou, B; Sinakos, Z; Mantalenakis, S

    1995-10-01

    Clinical data from eight pregnant women with idiopathic thrombocytopenic purpura (ITP) were retrospectively analyses. The mean age of the women was 28.2 years. Five women underwent splenectomy during childhood. The lowest maternal platelet count observed ranged from 8000 to 88000/mm3. Genital bleeding occurred in only one case. Treatment was based on administration of corticosteroids with or without human-pooled immunoglobulins. Caesarian section was performed in all cases. Six newborns were healthy and had a successful subsequent course. Two infants died, one in utero because of abruptio placentae and the other one 1 month post partum because of a cerebral haematoma. After a mean follow-up of eighteen months, thrombocytopenia is still present in two women, despite the continuous treatment. In conclusion, ITP rather rarely coincides with pregnancy. Treatment is usually successful for the mother but the risk for the fetus remains considerably high.

  16. 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

  17. 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

  18. Current management of juvenile idiopathic arthritis.

    PubMed

    Wallace, Carol A

    2006-04-01

    The goal of juvenile idiopathic arthritis (JIA) treatment is to achieve remission of disease. The absence of a full understanding of the disease pathogenesis for JIA hinders the development of truly effective treatment approaches. Further, the lack of clear knowledge regarding the mechanisms of action of rheumatologic medications and the existence of few randomized controlled trials leaves clinicians with very little evidence upon which to base decisions regarding the best timing, dosages or combinations of medications to be used for fully effective treatment of JIA. There is now a shift in treatment focus from that of chasing failure (gradual add-on approach to the use of medications) to one of early aggressive combination treatment. This chapter will discuss the current approaches to medical management of JIA and the medications currently available for use. JIA treatment is a vast, rich area in need of research. PMID:16546057

  19. Clinical manifestations of pediatric idiopathic hypercalciuria.

    PubMed

    Lau, Keith K

    2009-01-01

    Idiopathic hypercalciuria (IH) is a common metabolic disorder in children with protean manifestations, many of which can mimic other common pediatric diseases. Reports in the medical literature describe children with IH presenting with a wide array of calculi and non-calculi related clinical symptoms such as hematuria, urinary tract infections (UTIs), urgency, urinary incontinence and recurrent abdominal pain. Many of these symptom complexes have been only loosely associated with IH with no definite established causal relationship. Due to the common nature of IH and the varied clinical features attributed to it, it is of utmost importance for health-care professionals to be aware of these; this will facilitate early and appropriate investigations and prompt institution of therapy to avoid long-term morbidity.

  20. 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

  1. 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.

  2. [Unusual presentation of juvenile idiopathic arthritis and autoimmune hepatitis].

    PubMed

    Moreno Prieto, M; Carbonero Celis, M J; Cuadrado Caballero, M C

    2015-01-01

    The coexistence of autoimmune hepatitis and juvenile idiopathic arthritis is very rare. This is the case of an 18 month old female patient whose first sign of disease was torticollis due to an underlying atlanto-axial subluxation. Three months later, bilateral knee arthritis developed and she was diagnosed with Juvenile Idiopathic Arthritis. Throughout the disease a persistent elevation of liver enzymes was noted, combined with positive antinuclear antibodies and hypergammaglobulinemia, reaching the diagnosis of concomitant autoimmune hepatitis.

  3. 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

  4. 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.

  5. 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.

  6. A Two-Dimensional Zirconium Carbide by Selective Etching of Al3C3 from Nanolaminated Zr3Al3C5.

    PubMed

    Zhou, Jie; Zha, Xianhu; Chen, Fan Y; Ye, Qun; Eklund, Per; Du, Shiyu; Huang, Qing

    2016-04-11

    The room-temperature synthesis of a new two-dimensional (2D) zirconium-containing carbide, Zr3C2T(z) MXene is presented. In contrast to traditional preparation of MXene, the layered ternary Zr3Al3C5 material instead of MAX phases is used as source under hydrofluoric acid treatment. The structural, mechanical, and electronic properties of the synthesized 2D carbide are investigated, combined with first-principles density functional calculations. A comparative study on the structrual stability of our obtained 2D Zr3C2T(z) and Ti3C2T(z) MXenes at elevated temperatures is performed. The obtained 2D Zr3C2T(z) exhibits relatively better ability to maintain 2D nature and strucural integrity compared to Ti-based Mxene. The difference in structural stability under high temperature condition is explained by a theoretical investigation on binding energy.

  7. 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…

  8. 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…

  9. 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...

  10. 18 CFR 3c.3 - Reporting fraud, waste, abuse, and corruption and cooperation with official inquiries.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 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...

  11. 40 CFR 152.112 - Approval of registration under FIFRA sec. 3(c)(5).

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... sec. 3(c)(5). 152.112 Section 152.112 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY... Applications § 152.112 Approval of registration under FIFRA sec. 3(c)(5). EPA will approve an application under the criteria of FIFRA sec. 3(c)(5) only if: (a) The Agency has determined that the application...

  12. 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

  13. Learning effect on the dynamical strategies in sitting position on seesaw motion for idiopathic scoliosis patients.

    PubMed

    Bruyneel, Anne-Violette; Mesure, Serge

    2013-02-01

    Our study meant to determine, for idiopathic scoliosis patients, the biomechanical processes involved in postural regulation when self-imposed disturbances occur in seated position in both directions during successive trials. 12 female adolescents with right thoracic scoliosis (SG) and 15 control adolescents (CG) were included in this study. Ground reaction forces were studied using a force platform while the subjects were maintaining their balance in sitting position on a seesaw. Every test is recorded with eyes opened, arms on shoulders and legs free. The force platform data (AP and ML forces data) obtained were processed to determine the following normalized force parameters: delta value (difference between maxima and minima), maximal and minimal force values (peak and occurrence), and the variability of AP and ML forces. We used a variance analysis (ANOVA test) to analyze and compare 3 trials and groups. Our results show that, whatever the directions of destabilization (AP versus ML), SG was always in a learning situation. Indeed, the first test is always less stable than the second and third trials. However, for CG, adaptability between the tests is only highlighted during ML imbalance. Significant differences of strategies between the groups are only visible for the AP force component. For all conditions imposed, scoliotic patients perform specific trunk balance strategies. Clinical tests and rehabilitation methods should include the learning effect within the spatio-temporal adaptation to ground reaction forces.

  14. Multifrequency VLBI follow up study of strong γ-ray flares in the blazars 3C273 and 3C279

    NASA Astrophysics Data System (ADS)

    Lisakov, Mikhail M.; Kovalev, Yuri Y.

    2015-03-01

    We present multifrequency VLBI observations of the blazars 3C273 and 3C279 after detecting strong γ-ray flares in both of them. 3C273 exhibited a prominent flare in γ-rays in September 2009 which was followed by a strong flare in the 7 mm VLBI core and emergence of a new feature in the parsec scale jet. We have used time delay between flares in different wavebands together with kinematic analysis to determine that the γ-ray emission zone in 3C273 is located 3.6-5.3 pc upstream from the apparent 7 mm core. We have also analyzed frequency dependent core position to measure a deprojected distance between 7 mm core and the true base of the jet: 1-6 pc for 3C273 and 1-3 pc for 3C279, depending on observing epoch. For 3C279 light curve analysis did not give a robust γ-radio delay because there were too many overlapping flares in this source during considered period.

  15. 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

  16. Epstein-Barr virus nuclear antigen 3C regulated genes in lymphoblastoid cell lines.

    PubMed

    Zhao, Bo; Mar, Jessica C; Maruo, Seiji; Lee, Sungwook; Gewurz, Benjamin E; Johannsen, Eric; Holton, Kristina; Rubio, Renee; Takada, Kenzo; Quackenbush, John; Kieff, Elliott

    2011-01-01

    EBV nuclear antigen 3C (EBNA3C) is an essential transcription factor for EBV transformed lymphoblast cell line (LCL) growth. To identify EBNA3C-regulated genes in LCLs, microarrays were used to measure RNA abundances in each of three different LCLs that conditionally express EBNA3C fused to a 4-OH-Tamoxifen-dependent estrogen receptor hormone binding domain (EBNA3CHT). At least three RNAs were assayed for each EBNA3CHT LCL under nonpermissive conditions, permissive conditions, and nonpermissive conditions with wild-type EBNA3C transcomplementation. Using a two-way ANOVA model of EBNA3C levels, we identified 550 regulated genes that were at least 1.5-fold up- or down-regulated with false discovery rates < 0.01. EBNA3C-regulated genes overlapped significantly with genes regulated by EBNA2 and EBNA3A consistent with coordinated effects on cell gene transcription. Of the 550 EBNA3C-regulated genes, 106 could be placed in protein networks. A seeded Bayesian network analysis of the 80 most significant EBNA3C-regulated genes suggests that RAC1, LYN, and TNF are upstream of other EBNA3C-regulated genes. Gene set enrichment analysis found enrichment for MAP kinase signaling, cytokine-cytokine receptor interactions, JAK-STAT signaling, and cell adhesion molecules, implicating these pathways in EBNA3C effects on LCL growth or survival. EBNA3C significantly up-regulated the CXCL12 ligand and its CXCR4 receptor and increased LCL migration. CXCL12 up-regulation depended on EBNA3C's interaction with the cell transcription factor, RBPJ, which is essential for LCL growth. EBNA3C also up-regulated MYC 1.3-fold and down-regulated CDKN2A exons 2 and 3, shared by p16 and p14, 1.4-fold, with false discovery rates < 5 × 10(-4).

  17. Idiopathic Inflammatory Myopathies: Clinical Approach and Management.

    PubMed

    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

  18. 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.

  19. 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.

  20. [Optic neuritis in juvenile idiopathic arthritis patient].

    PubMed

    Lourenço, Daniela M R; Buscatti, Izabel M; Lourenço, Benito; Monti, Fernanda C; Paz, José Albino; Silva, Clovis A

    2014-01-01

    Optic neuritis (ON) was rarely reported in juvenile idiopathic arthritis (JIA) patients, particularly in those under anti-tumor necrosis factor alpha blockage. However, to our knowledge, the prevalence of ON in JIA population has not been studied. Therefore, 5,793 patients were followed up at our University Hospital and 630 (11%) had JIA. One patient (0.15%) had ON and was reported herein. A 6-year-old male was diagnosed with extended oligoarticular JIA, and received naproxen and methotrexate subsequently replaced by leflunomide. At 11 years old, he was diagnosed with aseptic meningitis, followed by a partial motor seizure with secondary generalization. Brain magnetic resonance imaging (MRI) and electroencephalogram showed diffuse disorganization of the brain electric activity and leflunomide was suspended. Seven days later, the patient presented acute ocular pain, loss of acuity for color, blurred vision, photophobia, redness and short progressive visual loss in the right eye. A fundoscopic exam detected unilateral papilledema without retinal exudates. Orbital MRI suggested right ON. The anti-aquaporin 4 (anti-AQP4) antibody was negative. Pulse therapy with methylprednisolone was administered for five days, and subsequently with prednisone, he had clinical and laboratory improvement. In conclusion, a low prevalence of ON was observed in our JIA population. The absence of anti-AQP4 antibody and the normal brain MRI do not exclude the possibility of demyelinating disease associated with chronic arthritis. Therefore, rigorous follow up is required.

  1. Diagnosis and classification of juvenile idiopathic arthritis.

    PubMed

    Eisenstein, Eli M; Berkun, Yackov

    2014-01-01

    In recent years, it has become increasingly clear that the term Juvenile Idiopathic Arthritis (JIA) comprises not one disease but several. Moreover, recent studies strongly suggest that some of these clinico-pathophysiologic entities appear to cross current diagnostic categories. The ultimate goal of the JIA classification is to facilitate development of better, more specific therapy for different forms of disease though improved understanding of pathophysiology. The past two decades have witnessed significant advances in treatment and improved outcomes for many children with chronic arthritis. However, understanding of the basic biologic processes underlying these diseases remains far from complete. As a result, even the best biologic agents of today represent "halfway technologies". Because they do not treat fundamental biologic processes, they are inherently expensive, need to be given for a long time in order to ameliorate the adverse effects of chronic inflammation, and do not cure the disease. Pediatric rheumatology is now entering an era in which diagnostic categories may need to change to keep up with discovery. A more precise, biologically based classification is likely to contribute to development of more specific and improved treatments for the various forms of childhood arthritis. In this review, we discuss how genetic, gene expression, and immunologic findings have begun to influence how these diseases are understood and classified.

  2. [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

  3. Micturition disturbance in acute idiopathic autonomic neuropathy

    PubMed Central

    Sakakibara, R; Uchiyama, T; Asahina, M; Suzuki, A; Yamanishi, T; Hattori, T

    2004-01-01

    Objective: To define the nature of micturition disturbance in patients with acute idiopathic autonomic neuropathy (AIAN). Methods: Micturitional symptoms were observed during hospital admissions and the in outpatient clinics in six patients with clinically definite AIAN (typical form in four, cholinergic variant in one, autonomic-sensory variant in one). Urodynamic studies included medium-fill water cystometry, external sphincter electromyography, and a bethanechol test. Results: Four patients had urinary retention and two had voiding difficulty as the initial presentation. Patients with retention became able to urinate within a week (two to seven days). The major symptoms at the time of urodynamic studies (three weeks to four months after disease onset in most cases) were voiding difficulty and nocturnal frequency. None had urinary incontinence. Complete recovery from the micturition disturbance took from three months to >18 years. The recovery period was shorter in a patient with cholinergic variant, and it was longer in two patients who had a longer duration of initial urinary retention. Micturition disturbance tended to improve earlier than orthostatic hypotension. The major urodynamic abnormalities were detrusor areflexia on voiding (5), denervation supersensitivity to bethanechol (3); low compliance detrusor (1); and impaired bladder sensation (2). None had neurogenic motor unit potentials of the external sphincter muscles. Conclusions: In patients with AIAN, urinary retention and voiding difficulty are common initial presentations. The underlying mechanisms seem to be pre- and postganglionic cholinergic dysfunction with preservation of somatic sphincter function. The bladder problems tend to improve earlier than orthostatic hypotension. PMID:14742606

  4. 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

  5. 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

  6. 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

  7. 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

  8. The human microbiome and juvenile idiopathic arthritis.

    PubMed

    Verwoerd, Anouk; Ter Haar, Nienke M; de Roock, Sytze; Vastert, Sebastiaan J; Bogaert, Debby

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The microbiome has received increasing attention as a potential contributing factor to the development of a wide array of immune-mediated diseases, including inflammatory bowel disease, type 1 diabetes and rheumatoid arthritis. Also in JIA, there is accumulating evidence that the composition of the microbiome is different from healthy individuals. A growing body of evidence indeed suggests that, among others, the microbiome may influence the development of the immune system, the integrity of the intestinal mucosal barrier, and the differentiation of T cell subsets. In turn, this might lead to dysregulation of the immune system, thereby possibly playing a role in the development of JIA. The potential to manipulate the microbiome, for example by faecal microbial transplantation, might then offer perspectives for future therapeutic interventions. Before we can think of such interventions, we need to first obtain a deeper understanding of the cause and effect relationship between JIA and the microbiome. In this review, we discuss the existing evidence for the involvement of the microbiome in JIA pathogenesis and explore the potential mechanisms through which the microbiome may influence the development of autoimmunity in general and JIA specifically. PMID:27650128

  9. 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.

  10. Macrophages - silent enemies in juvenile idiopathic arthritis.

    PubMed

    Świdrowska-Jaros, Joanna; Orczyk, Krzysztof; Smolewska, Elżbieta

    2016-07-06

    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.

  11. 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

  12. Idiopathic pulmonary fibrosis and coronary artery disease.

    PubMed

    Cicchitto, Gaetano; Musella, Valentina; Acitorio, Maria; Capuano, Nicola; Fiorenzano, Giuseppe; Owen, Caroline A; Polverino, Mario; Polverino, Francesca

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is defined as a chronic fibrosing interstitial disease of unknown cause, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP); it generally progresses into respiratory failure and death. Although progression of the disease is the most common cause of death, there are increasing reports of its association with other pathologies has been reported: e.g., IPF patients seem more susceptible to cardiovascular diseases. Therefore, other pathologies might also influence the natural course. In this paper, we describe a case of IPF and coronary artery disease (CAD). We emphasize the importance of cardiopulmonary exercise test (CPET) as a useful procedure to monitor disease progression in IPF patients. We also stress the importance of a careful analysis of variables measured for an accurate interpretation of the clinical picture and an improvement of the clinical management of patients. Moreover, we suggest that a careful assessment of CPET parameters may additionally help in the early detection of high cardiovascular ischemic risk.

  13. Idiopathic learning disability and genome imbalance.

    PubMed

    Knight, S J L; Regan, R

    2006-01-01

    Learning disability (LD) is a very common, lifelong and disabling condition, affecting about 3% of the population. Despite this, it is only over the past 10-15 years that major progress has been made towards understanding the origins of LD. In particular, genetics driven advances in technology have led to the unequivocal demonstration of the importance of genome imbalance in the aetiology of idiopathic LD (ILD). In this review we provide an overview of these advances, discussing technologies such as multi-telomere FISH and array CGH that have already emerged as well as new approaches that show diagnostic potential for the future. The advances to date have highlighted new considerations such as copy number polymorphisms (CNPs) that can complicate the interpretation of genome imbalance and its relevance to ILD. More importantly though, they have provided a remarkable approximately 15-20% improvement in diagnostic capability as well as facilitating genotype/phenotype correlations and providing new avenues for the identification and understanding of genes involved in neurocognitive function.

  14. Endothelin-1 in idiopathic pulmonary fibrosis.

    PubMed Central

    Uguccioni, M; Pulsatelli, L; Grigolo, B; Facchini, A; Fasano, L; Cinti, C; Fabbri, M; Gasbarrini, G; Meliconi, R

    1995-01-01

    AIMS--To evaluate whether endothelin-1 is involved in the pathology of idiopathic pulmonary fibrosis (IPF). METHODS--Plasma endothelin-1 concentrations were evaluated in 37 patients with IPF and 27 normal controls by radioimmunoassay. In addition, expression of endothelin-1 in lung tissue was evaluated in biopsy specimens obtained from four patients with IPF. Three biopsy specimens of normal lung were used as controls. Endothelin-1 immunoreactivity was detected using immunohistochemistry. RESULTS--Elevated endothelin-1 plasma concentrations were found in patients with IPF compared with controls and a positive correlation was found with duration of disease. No significant difference was observed between treated and untreated patients with IPF. Increased endothelin-1 immunoreactivity was found in lungs of three of four patients with IPF. Endothelin-1 positive consisted mainly of small vessel endothelial cells. Some scattered macrophages were also positive. CONCLUSIONS--Elevated plasma concentrations and expression of endothelin-1 in lung tissue are suggestive of increased production of endothelin-1 in at least a proportion of patients with IPF. Consequently, endothelin-1 activity could play a role in the fibrogenic process of the disease. Images PMID:7615852

  15. [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.

  16. 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

  17. 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.

  18. 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.

  19. Antigens of monoclonal antibody NB3C4 are novel markers for oligodendrocytes.

    PubMed

    Yoshimura, K; Kametani, F; Shimoda, Y; Fujimaki, K; Sakurai, Y; Kitamura, K; Asou, H; Nomura, M

    2001-02-12

    We produced NB3C4, a novel monoclonal antibody specific for oligodendrocytes, using human neuroblastoma IMR-32 cells. NB3C4 specifically recognized oligodendrocytes in the CNS, although it bound to neuroblastoma IMR-32 cells and oligodendrocytes in vitro. Double immunofluorescence staining of rat brain using NB3C4 and anti-GST-pi, anti-glial fibrillary acidic protein (GFAP), or anti-neurofilament 200 (NF) antibody revealed that anti-GST-pi antibody identified an oligodendrocyte marker recognizing NB3C4-positive cells, while both anti-GFAP and anti-NF antibody did not. Western blotting of rat brain homogenates showed that NB3C4 bound three proteins of 22-28 kDa, while the anti-GST-pi recognized a 27 kDa protein. Therefore, antigens recognized by NB3C4 could be novel markers for oligodendrocytes.

  20. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity.

    PubMed

    Frankel, Stephen K; Cool, Carlyne D; Lynch, David A; Brown, Kevin K

    2004-12-01

    Between 1996 and 2001, we identified five cases of a unique idiopathic pleuroparenchymal lung disease characterized by a clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, marked pleural and parenchymal radiographic involvement with an upper lobe predominance, and surgical lung biopsy findings that did not fit with any of the currently defined interstitial pneumonias. The pathologic findings included the following: (1) intense fibrosis of the visceral pleura; (2) prominent, homogenous, subpleural fibroelastosis; (3) sparing of the parenchyma distant from the pleura; (4) mild, patchy lymphoplasmacytic infiltrates; and (5) small numbers of fibroblastic foci present at the leading edge of the fibrosis. In this report, we characterize the clinical, radiographic, physiologic, and pathologic findings of this entity, which we term idiopathic pleuroparenchymal fibroelastosis. PMID:15596706

  1. AA amyloidosis associated with systemic-onset juvenile idiopathic arthritis.

    PubMed

    Saha, Abhijeet; Chopra, Yogiraj; Theis, Jason D; Vrana, Julie A; Sethi, Sanjeev

    2013-10-01

    We report a 12-year-old boy with nephrotic syndrome due to renal AA amyloidosis. The AA amyloidosis was associated with a 3-year history of systemic-onset juvenile idiopathic arthritis. The presence of serum amyloid A protein was confirmed by laser microdissection of Congo Red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry; this analysis excluded hereditary and familial amyloidosis. Aggressive management of the systemic-onset juvenile idiopathic arthritis resulted in improvement in clinical and laboratory parameters. The case represents an unusual cause of nephrotic syndrome in children. Early diagnosis of renal amyloidosis and management of systemic-onset juvenile idiopathic arthritis is paramount to preventing progression of kidney disease.

  2. Multiple pulmonary hyalinizing granulomas associated with systemic idiopathic fibrosis.

    PubMed

    Kuramochi, S; Kawai, T; Yakumaru, K; Mikata, A; Torikata, C; Kasuga, Y; Fujiwara, T

    1991-05-01

    A 41-year-old man with progressive nodular infiltration of the lung of about 2 years' duration died of cardiac and respiratory failure. Autopsy revealed bilateral multiple pulmonary hyalinizing granulomas (PHGs) diagnosed on the basis of the characteristic dense hyaline collagen bundles with nonspecific inflammatory infiltration. Constrictive pericarditis, retroperitoneal fibrosis, mediastinal fibrosis, fibrous thickening of the peritoneal and pleural surfaces, and fibrosis of soft tissue of the neck, flank, and hepatic hilar region were present, therefore, a diagnosis of systemic idiopathic fibrosis was made. The patient had anti-thyroglobulin and anti-thyroid microsomal antibodies and lymphocytic thyroiditis. The inflammatory process of PHG of the present case was active and the clinical course was progressive. PHG seems to be a lesion belonging to the systemic idiopathic fibrosis complex. Immunologic abnormalities may be related to PHG and systemic idiopathic fibrosis. PMID:1714226

  3. 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.

  4. Pathogenesis, newly recognized etiologies, and management of idiopathic anaphylaxis.

    PubMed

    Kuhlen, James L; Virkud, Yamini V

    2015-02-01

    Idiopathic anaphylaxis (IA) is a life-threatening allergic disease and the most common diagnosis given to patients following an anaphylactic event. The inability of the healthcare provider and the patient to identify the trigger for anaphylaxis makes standard allergen avoidance measures ineffectual. IA is diagnosed after other causes of anaphylaxis have been excluded. Mast cell activation syndromes (MCAS), mastocytosis, IgE to galactose-alpha-1,3-galactose (α-gal), and certain medications have recently been recognized as causes of anaphylaxis that were previously labeled idiopathic. This review will describe the epidemiology and proposed theories of pathogenesis for IA, its diagnostic approach, its clinical management, and examine newly recognized disorders that were previously labeled as idiopathic anaphylaxis.

  5. 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

  6. Lone or idiopathic atrial fibrillation, messenger of misery in sight.

    PubMed

    Weijs, B; Crijns, H J G M

    2014-12-20

    This editorial refers to 'Gender-related differences in risk of cardiovascular morbidity and all-cause mortality in patients hospitalized with incident atrial fibrillation without concomitant diseases: a nationwide cohort study of 9519 patients' by T. Andersson et al. In order to adequately describe root causes and adverse consequences of apparently idiopathic AF, the requested study population has to be large and be followed for a very long time. Andersson et al. adequately deployed the excellent national Swedish health registries in order to cover the hiatus of aforementioned studies in the current idiopathic AF literature. Considering the notion that patients with idiopathic or lone AF have comparable prospects as AF patients overall but are only caught early in their 'arrhythmia and vascular career', the study by Andersson et al. should trigger physicians to give high priority to exposing predisposing factors or early stages of underlying cardiovascular disease in such a way that preventative measures can be accurately deployed in these patients.

  7. Idiopathic myenteric ganglionitis underlying intractable vomiting in a young adult.

    PubMed

    De Giorgio, R; Barbara, G; Stanghellini, V; Cogliandro, R F; Arrigoni, A; Santini, D; Ceccarelli, C; Salvioli, B; Rossini, F P; Corinaldesi, R

    2000-06-01

    Inflammatory infiltration of intestinal myenteric plexuses (i.e. myenteric ganglionitis), along with severe intestinal motor abnormalities, may accompany paraneoplastic syndromes, neurological disorders and gastrointestinal infections, although rare cases can be idiopathic. In this report, we describe the case of a patient who presented with chronic intractable vomiting and weight loss associated with idiopathic myenteric ganglionitis mainly involving the stomach. Tissue analysis showed that the inflammatory infiltrate comprised T lymphocytes (CD4+ and CD8+), and peptide immunolabelling revealed a marked decrease of substance P/tachykinin immunoreactive staining in nerve fibres and myenteric neurones. Following systemic steroid therapy, the patient's symptoms dramatically improved, and after one year of follow-up his general condition remains satisfactory. The possible mechanisms leading to symptom generation and gastric dysmotility in the context of an idiopathic myenteric ganglionitis are discussed.

  8. 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.

  9. Synthesis and X-ray studies of novel 3-C-nitromethyl-hexofuranoses.

    PubMed

    Turks, Māris; Vēze, Krista; Kiseļovs, Gļebs; Mackeviča, Jevgeņija; Lugiņina, Jevgeņija; Mishnev, Anatoly; Marković, Dean

    2014-06-01

    A practical method for the synthesis of three novel 3-C-nitromethyl-hexofuranoses is reported. The Henry reaction on a 1,2:5,6-di-O-isopropylidene-α-d-gulofuranose-derived ketone provided a 3-C-branched gulo-isomer as the sole reaction product. The dehydration-rehydration of the latter yielded an isopropylidene-protected 3-C-nitromethyl-galactofuranose. The reaction sequence can be also used for the synthesis of a 3-deoxy-3-C-nitromethyl-hexofuranose derivative with a gulo-configuration. Two of the newly obtained carbohydrate derivatives were characterized by X-ray crystallography.

  10. The nature of the optical variations of Seyfert galaxy 3C 120

    SciTech Connect

    Webb, J.R. Austin State Univ., TX )

    1990-01-01

    Results are presented from 61 years of optical observations of the Seyfert galaxy 3C 120. A previously published model of the 3C 120 light curve, derived from power spectrum analysis, is found to be valid for historical as well as current data. It is concluded that the optical variations of 3C 120 can be separated into a linear component, a sinusoidal component, and rapid, high-amplitude flares. Possible sources of the regular variations observed in 3C 120 are also suggested in the context of accretion models and other theoretical models. 15 refs.

  11. 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

  12. Complex genetics in idiopathic hypogonadotropic hypogonadism.

    PubMed

    Pitteloud, Nelly; Durrani, Sadia; Raivio, Taneli; Sykiotis, Gerasimos P

    2010-01-01

    Idiopathic hypogonadotropic hypogonadism (IHH) is an important human disease model. Investigations of the genetics of IHH have facilitated insights into critical pathways regulating sexual maturation and fertility. IHH has been traditionally considered a monogenic disorder. This model holds that a single gene defect is responsible for the disease in each patient. In the case of IHH, 30% of cases are explained by mutations in one of eleven genes. In recent years, several lines of evidence have challenged the monogenic paradigm in IHH. First, disease-associated mutations display striking incomplete penetrance and variable expressivity within and across IHH families. Second, each locus is responsible for only a small percentage of cases. Third, more than one disease-associated mutation seems to be segregating in some families with IHH, and their combined or separate presence in individuals accounts for the variability in disease severity. Finally, IHH is not strictly a congenital and life-long disorder; occasionally it manifests itself during adulthood (adult-onset IHH); in other cases, the disease is not permanent, as evidenced by normal activity of the hypothalamic-pituitary-gonadal axis after discontinuation of treatment in adulthood (IHH reversal). Together, these observations suggest that IHH is not strictly a monogenic mendelian disease, as previously thought. Rather, it is emerging as a digenic, and potentially oligogenic disease, in which hormonal and/or environmental factors may critically influence genetic predisposition and clinical course. Future investigations of IHH should characterize the extent of the involvement of multiple genes in disease pathogenesis, and elucidate the contributions of epigenetic factors.

  13. 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

  14. Smoking-related idiopathic interstitial pneumonia.

    PubMed

    Flaherty, Kevin R; Fell, Charlene; Aubry, Marie-Christine; Brown, Kevin; Colby, Thomas; Costabel, Ulrich; Franks, Teri J; Gross, Barry H; Hansell, David M; Kazerooni, Ella; Kim, Dong Soon; King, Talmadge E; Kitachi, Masanori; Lynch, David; Myers, Jeff; Nagai, Sonoko; Nicholson, Andrew G; Poletti, Venerino; Raghu, Ganesh; Selman, Moises; Toews, Galen; Travis, William; Wells, Athol U; Vassallo, Robert; Martinez, Fernando J

    2014-09-01

    Cigarette smoking is a key factor in the development of numerous pulmonary diseases. An international group of clinicians, radiologists and pathologists evaluated patients with previously identified idiopathic interstitial pneumonia (IIP) to determine unique features of cigarette smoking. Phase 1 (derivation group) identified smoking-related features in patients with a history of smoking (n=41). Phase 2 (validation group) determined if these features correctly predicted the smoking status of IIP patients (n=100) to participants blinded to smoking history. Finally, the investigators sought to determine if a new smoking-related interstitial lung disease phenotype could be defined. Phase 1 suggested that preserved forced vital capacity with disproportionately reduced diffusing capacity of the lung for carbon monoxide, and various radiographic and histopathological findings were smoking-related features. In phase 2, the kappa coefficient among clinicians was 0.16 (95% CI 0.11-0.21), among the pathologists 0.36 (95% CI 0.32-0.40) and among the radiologists 0.43 (95% CI 0.35-0.52) for smoking-related features. Eight of the 100 cases were felt to represent a potential smoking-related interstitial lung disease. Smoking-related features of interstitial lung disease were identified in a minority of smokers and were not specific for smoking. This study is limited by its retrospective design, the potential for recall bias in smoking history and lack of information on second-hand smoke exposure. Further research is needed to understand the relationship between smoking and interstitial lung disease. PMID:25063244

  15. 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

  16. 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

  17. 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

  18. Epistatic interactions in idiopathic pulmonary arterial hypertension

    PubMed Central

    Vadapalli, Shivani; Satyanarayana, M. L.; Chaitra, K. L.; Rani, H. Surekh; Sastry, B.K.S.; Nallari, Pratibha

    2012-01-01

    BACKGROUND: Idiopathic pulmonary arterial hypertension (IPAH) is a poorly understood complex disorder, which results in progressive remodeling of the pulmonary artery that ultimately leads to right ventricular failure. A two-hit hypothesis has been implicated in pathogenesis of IPAH, according to which the vascular abnormalities characteristic of PAH are triggered by the accumulation of genetic and/or environmental insults in an already existing genetic background. The multifactor dimensionality reduction (MDR) analysis is a statistical method used to identify gene–gene interaction or epistasis and gene–environment interactions that are associated with a particular disease. The MDR method collapses high-dimensional genetic data into a single dimension, thus permitting interactions to be detected in relatively small sample sizes. AIM: To identify and characterize polymorphisms/genes that increases the susceptibility to IPAH using MDR analysis. MATERIALS AND METHODS: A total of 77 IPAH patients and 100 controls were genotyped for eight polymorphisms of five genes (5HTT, EDN1, NOS3, ALK-1, and PPAR-γ2). MDR method was adopted to determine gene–gene interactions that increase the risk of IPAH. RESULTS: With MDR method, the single-locus model of 5HTT (L/S) polymorphism and the combination of 5HTT(L/S), EDN1(K198N), and NOS3(G894T) polymorphisms in the three-locus model were attributed to be the best models for predicting susceptibility to IPAH, with a P value of 0.05. CONCLUSION: MDR method can be useful in understanding the role of epistatic and gene–environmental interactions in pathogenesis of IPAH. PMID:22754222

  19. 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

  20. Optimal management of idiopathic macular holes.

    PubMed

    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.

  1. 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

  2. Multiple idiopathic external apical root resorption: A rare case report

    PubMed Central

    Bansal, Parul; Nikhil, Vineeta; Kapur, Sonali

    2015-01-01

    Multiple idiopathic external apical root resorption (MIEARR) is a relatively rare condition affecting multiple teeth in a dentition. As the condition is nonsymptomatic, a case is usually detected as an incidental radiographic finding. However, it may cause pain and mobility in severe cases. It is sometimes self-limiting or sometimes may progress to tooth loss. This paper presents a case of external apical root resorption involving multiple teeth in which etiology was not identified, so idiopathic root resorption was considered as a diagnosis of exclusion. PMID:25657532

  3. Risperidone in idiopathic and symptomatic dystonia: preliminary experience.

    PubMed

    Grassi, E; Latorraca, S; Piacentini, S; Marini, P; Sorbi, S

    2000-04-01

    Risperidone is a heterocyclic neuroleptic with prominent antiserotoninergic (5HT2) as well as antidopaminergic (D2) activity. We studied the efficacy of risperidone in the treatment of idiopathic and symptomatic dystonias in seven patients using the Fahn and Marsden rating scale for torsion dystonia before and after four weeks of treatment (2-6 mg/day). The twisting and involuntary movements with abnormal postures decreased in all the patients treated, with a statistically significant mean improvement (41%; p = 0.009, CI 95%). Our results suggest that risperidone is useful in idiopathic and symptomatic dystonia. PMID:10938193

  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. 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

  6. Clinical findings in unilateral acute idiopathic maculopathy: new findings in acute idiopathic maculopathy.

    PubMed

    Haruta, Hiroshi; Sawa, Miki; Saishin, Yoshitsugu; Ohguro, Nobuyuki; Tano, Yasuo

    2010-04-01

    We report a case of unilateral acute idiopathic maculopathy (UAIM) with new clinical findings. A 34-year-old Japanese man had a neurosensory retinal detachment (approximately 5 disk diameters) with yellowish-white exudates at the macula in the left eye (visual acuity (VA) 0.4). Fluorescein angiography (FA) showed early hypofluorescent spots and late pooling in the subretinal space. Three weeks after onset, indocyanine green angiography (IA) showed numerous hypofluorescent spots at the lesion. Optical coherence tomography (OCT) showed subretinal fluids and an elevated choroidal lesion with low reflectivity, suggesting choroidal edema. The VA and fundus appearance spontaneously resolved without treatment three months after onset. The VA was 1.0 six months after onset. Irregular pigmentation remained at the macular lesion. The main UAIM pathology may be outer retinal layer and retinal pigment epithelial inflammation. FA, IA, and OCT suggested that choroidal inflammation may be involved in the pathogenesis of UAIM.

  7. 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…

  8. 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

  9. Positively Adolescent!

    ERIC Educational Resources Information Center

    Williamson, Sue

    2000-01-01

    Believes that music teachers should reassess their views toward adolescent behavior in the music classroom by learning to see their behavior in a positive light. Describes teaching strategies that build on four adolescent behaviors: (1) desire for peer acceptance; (2) abundant energy; (3) love of fun; and (4) limited time-managing skills. (CMK)

  10. 17 CFR 270.3c-2 - Definition of beneficial ownership in small business investment companies.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... 17 Commodity and Securities Exchanges 3 2010-04-01 2010-04-01 false Definition of beneficial ownership in small business investment companies. 270.3c-2 Section 270.3c-2 Commodity and Securities Exchanges SECURITIES AND EXCHANGE COMMISSION (CONTINUED) RULES AND REGULATIONS, INVESTMENT COMPANY ACT...

  11. 17 CFR 270.3c-2 - Definition of beneficial ownership in small business investment companies.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... 17 Commodity and Securities Exchanges 3 2011-04-01 2011-04-01 false Definition of beneficial ownership in small business investment companies. 270.3c-2 Section 270.3c-2 Commodity and Securities Exchanges SECURITIES AND EXCHANGE COMMISSION (CONTINUED) RULES AND REGULATIONS, INVESTMENT COMPANY ACT...

  12. 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... EXCLUSIVE ECONOMIC ZONE OFF ALASKA Pt. 680, Table 3c Table 3c to Part 680—Crab Product Codes for Economic Data Reports Code Description 01 Whole crab. 80 Sections. 81 Meats. 97 Other (specify)....

  13. 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... EXCLUSIVE ECONOMIC ZONE OFF ALASKA Pt. 680, Table 3c Table 3c to Part 680—Crab Product Codes for Economic Data Reports Code Description 01 Whole crab. 80 Sections. 81 Meats. 97 Other (specify)....

  14. 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... EXCLUSIVE ECONOMIC ZONE OFF ALASKA Pt. 680, Table 3c Table 3c to Part 680—Crab Product Codes for Economic Data Reports Code Description 01 Whole crab. 80 Sections. 81 Meats. 97 Other (specify)....

  15. Eukaryotic initiation factor 3C silencing inhibits cell proliferation and promotes apoptosis in human glioma.

    PubMed

    Hao, Jinmin; Wang, Zhiming; Wang, Yaowu; Liang, Zhaohui; Zhang, Xin; Zhao, Zongmao; Jiao, Baohua

    2015-06-01

    Eukaryotic initiation factor 3, subunit c (eIF3c), an oncogene overexpressed in human cancers, plays an important role in cell tumorigenesis and proliferation. However, studies assessing its function in gliomas are scarce. The present study evaluated for the first time, the role of eIF3c in gliomas. Immunohistochemistry was carried out to assess eIF3c expression in 95 human glioma samples and normal brain tissues. Then, the eIF3c mRNA levels were detected in tumor and normal brain specimens by quantitative RT-PCR. In addition, eIF3c mRNA levels were assessed in four glioma cell lines (U87, U251, A172 and U373) by semi-quantitative RT-PCR. The RNA interference (RNAi) technology was employed to knock down the eIF3c gene in the U251 cells. Western blot analysis, BrdU assay and flow cytometry were used to measure eIF3c protein levels, cell proliferation, cell apoptosis and cell cycle, respectively. The eIF3c protein was overexpressed in the human glioma specimens. In agreement, the eIF3c mRNA expression levels were significantly higher in the human glioma tissues compared with the normal brain samples (P<0.0001). In addition, eIF3c mRNA was detected in all the glioma cell lines. Silencing the eIF3c gene in the U251 cells by RNAi significantly suppressed cell proliferation (P<0.01) and increased apoptosis (P<0.01). Finally, a stark decrease was observed in the G1 phase cell number (P<0.01), while the S and G2 phase cells were significantly increased (P<0.01) after eIF3c knockdown. These findings suggest that eIF3c is overexpressed in human gliomas and essential for their proliferation and survival. Therefore, inhibiting eIF3c expression may constitute an effective therapy for human glioma.

  16. 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.

  17. CVD growth and properties of boron phosphide on 3C-SiC

    NASA Astrophysics Data System (ADS)

    Padavala, Balabalaji; Frye, C. D.; Wang, Xuejing; Raghothamachar, Balaji; Edgar, J. H.

    2016-09-01

    Improving the crystalline quality of boron phosphide (BP) is essential for realizing its full potential in semiconductor device applications. In this study, 3C-SiC was tested as a substrate for BP epitaxy. BP films were grown on 3C-SiC(100)/Si, 3C-SiC(111)/Si, and 3C-SiC(111)/4H-SiC(0001) substrates in a horizontal chemical vapor deposition (CVD) system. Films were produced with good crystalline orientation and morphological features in the temperature range of 1000-1200 °C using a PH3+B2H6+H2 mixture. Rotational twinning was absent in the BP due to the crystal symmetry-matching with 3C-SiC. Confocal 3D Raman imaging of BP films revealed primarily uniform peak shift and peak widths across the scanned area, except at defects on the surface. Synchrotron white beam X-ray topography showed the epitaxial relationship between BP and 3C-SiC was (100) < 011 > BP||(100) < 011 > 3C-SiC and (111) < 11 2 ̅ > BP||(111) < 11 2 ̅ > 3C-SiC. Scanning electron microscopy, Raman spectroscopy and X-ray diffraction analysis indicated residual tensile strain in the films and improved crystalline quality at temperatures below 1200 °C. These results indicated that BP properties could be further enhanced by employing high quality bulk 3C-SiC or 3C-SiC epilayers on 4H-SiC substrates.

  18. Bone morphogenetic protein signalling in heritable versus idiopathic pulmonary hypertension

    PubMed Central

    Dewachter, Laurence; Adnot, Serge; Guignabert, Christophe; Tu, Ly; Marcos, Elisabeth; Fadel, Elie; Humbert, Marc; Dartevelle, Philippe; Simonneau, Gérald; Naeije, Robert; Eddahibi, Saadia

    2009-01-01

    Mutations in gene encoding for bone morphogenetic protein type 2 receptor (BMPR-2) have been reported in pulmonary arterial hypertension (PAH), but their functional relevance remains incompletely understood. BMP receptors expression was evaluated in human lungs and in cultured pulmonary artery smooth muscle cells (PASMCs) isolated from 19 idiopathic PAH patients and 9 heritable PAH patients with demonstrated BMPR-2 mutations. BMP4-treated PASMCs were assessed for Smad and p38MAPK signaling associated to mitosis and apoptosis. Lung tissue and PASMCs from heritable PAH patients presented with decreased BMPR-2 expression and variable increases in BMPR-1A and BMPR-1B expressions, while a less important decreased BMPR-2 expression was observed in PASMCs from idiopathic PAH patients. Heritable PAH PASMCs showed no increased phosphorylation of Smad1/5/8 in the presence of BMP4, which actually activated the p38MAPK pathway. Individual responses varied from one mutation to another. PASMCs from PAH patients presented with an in vitro proliferative pattern, which could be inhibited by BMP4 in idiopathic PAH, not in heritable PAH. PASMCs from idiopathic PAH and more so from heritable presented an inhibition of BMP4-induced apoptosis. Most heterogenous BMPR-2 mutations are associated with defective Smad signaling compensed for by an activation of p38MAPK signaling, accounting for PASMC proliferation and deficient apoptosis. PMID:19324947

  19. Loss of sensory function in patients with idiopathic hand dystonia.

    PubMed

    Suttrup, Inga; Oberdiek, Denise; Suttrup, Judith; Osada, Nani; Evers, Stefan; Marziniak, Martin

    2011-01-01

    Former studies suggest an additional involvement of the sensory nervous system, beside the involuntary contractions of antagonist muscles, in idiopathic hand dystonia. We studied contact heat-evoked potentials and quantitative sensory testing (QST) in 10 patients suffering from idiopathic hand dystonia and 10 age-matched healthy controls. Cortical potentials recorded from the vertex (Pz) after contact heat stimulation of the volar forearm and the dorsum of the hand at a temperature of 51°C showed significantly reduced A-δ-amplitudes. Numerical pain ratings on the affected side in comparison to the unaffected side and to healthy controls were significantly reduced. QST results showed an impairment of the thermal detection thresholds, the mechanical pain sensitivity and the mechanical pain threshold at the affected body side of the patients. Our results suggest a loss of distinct sensory functions of the affected hand in comparison with the contralateral hand and to matched healthy subjects in patients suffering from idiopathic hand dystonia. For the first time, an extended loss of sensory function could be shown in patients suffering from idiopathic hand dystonia.

  20. 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

  1. [Association study of telomere length with idiopathic male infertility].

    PubMed

    Shuyuan, Liu; Changjun, Zhang; Haiying, Peng; Xiaoqin, Huang; Hao, Sun; Keqin, Lin; Kai, Huang; Jiayou, Chu; Zhaoqing, Yang

    2015-11-01

    Telomeres are evolutionary conserved, multifunctional DNA-protein complexes located at the ends of eukaryotic chromosomes. Telomeres maintain chromosome stability and genome integrity and also play an important role in meiosis which aid in synapsis, homologous recombination, and segregation. Sperm telomere has been reported to play an important role in fertilization and embryo development. Nowadays, the association between telomere and reproduction is one of the major areas of interest, however whether sperm telomere associated with male infertility is not clear. In this study, in order to find out the association between Chinese idiopathic infertility and sperm telomere length, we analyzed the difference of sperm telomere length between idiopathic infertile men and normal fertile men, as well as the correlations between sperm telomere length and human semen characteristics. We analyzed 126 Chinese idiopathic infertile men and 138 normal fertile men for sperm telomere length by using quantitative PCR. We found that the relative sperm mean telomere length of infertile men was significantly shorter than that of fertile men (2.894 ± 0.115 vs. 4.016 ± 0.603, P=5.097 x 10⁻⁵). Both sperm count and semen progressive motility are related with telomere length. Our results suggest that sperm telomere length is associated with idiopathic male infertility of China and we proposed the possibility that shorter telomeres in sperm chromosome will reduce spermatogenesis and sperm functions, which finally affected the fertility of male.

  2. Association between periodontal status and idiopathic male infertility.

    PubMed

    Pásztor, Norbert; Kárpáti, Krisztina; Szöllősi, János; Keresztúri, Márk; Kozinszky, Zoltan; Gorzó, István; Radnai, Márta

    2016-01-01

    About 30% of male infertility cases are idiopathic. Previous studies reported a positive correlation between deep periodontal pockets and sperm sub-motility, which suggests that periodontitis might have a role in idiopathic semen abnormality pathospermia. We evaluated correlations between periodontal infection parameters and the results of sperm analysis of men with idiopathic infertility. In this observational study, semen quality and periodontal status were analyzed for 95 otherwise healthy men attending an andrology unit for sperm analysis. Half the men in the sperm pathology and normozoospermia groups (50.8% and 50%, respectively) had poor periodontal status. Among the 95 participants, 38% had oligozoospermia, 28% had asthenozoospermia, 16% had cryptozoospermia, and 15% were classified as normozoospermic. Sperm pathology category was not associated with frequency of deep periodontal pockets or calculus. Bleeding on probing was significantly lower among men with asthenozoospermia than among those with normozoospermia. Poor periodontal status was not associated with any sperm pathology category or parameter. In contrast with previous findings, the present results indicate that pathospermia and poor semen quality are not associated with periodontal infection in men with idiopathic infertility. (J Oral Sci 58, 247-253, 2016). PMID:27349547

  3. Association between juvenile idiopathic arthritis and osteogenesis imperfecta: case report.

    PubMed

    Bica, Blanca Elena Rios Gomes; Ruiz, Danilo Garcia; Magalhães, Priscilla de Andrade; Barcellos, Marlúcia Guimarães; de Azevedo, Mário Newton Leitão

    2013-01-01

    The authors report a rare association case of juvenile idiopathic arthritis (JIA) and osteogenesis imperfecta (OI) in a 53 years-old female patient, present a literature review and discuss the radiological aspects of the temporo-mandibular joint involvement. To our knowledge, this is the first case report of JIA an OI association.

  4. 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.

  5. Idiopathic eruptive macular pigmentation with papillomatosis (IEMPP): A controversial entity.

    PubMed

    Pang, Y Z; Koh, W L; Ang, C C

    2016-01-01

    A 19-year-old man with a 6-month history of progressive development of hyperpigmented, velvety plaques on the face and body. A diagnosis of idiopathic eruptive macular pigmentation with papillomatosis (IEMPP) was determined. This entity is discussed. PMID:27617467

  6. 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 ...

  7. Schwann Cells Metabolize Extracellular 2',3'-cAMP to 2'-AMP.

    PubMed

    Verrier, Jonathan D; Kochanek, Patrick M; Jackson, Edwin K

    2015-08-01

    The 3',5'-cAMP-adenosine pathway (3',5'-cAMP→5'-AMP→adenosine) and the 2',3'-cAMP-adenosine pathway (2',3'-cAMP→2'-AMP/3'-AMP→adenosine) are active in the brain. Oligodendrocytes participate in the brain 2',3'-cAMP-adenosine pathway via their robust expression of 2',3'-cyclic nucleotide 3'-phosphodiesterase (CNPase; converts 2',3'-cAMP to 2'-AMP). Because Schwann cells also express CNPase, it is conceivable that the 2',3'-cAMP-adenosine pathway exists in the peripheral nervous system. To test this and to compare the 2',3'-cAMP-adenosine pathway to the 3',5'-cAMP-adenosine pathway in Schwann cells, we examined the metabolism of 2',3'-cAMP, 2'-AMP, 3'-AMP, 3',5'-cAMP, and 5'-AMP in primary rat Schwann cells in culture. Addition of 2',3'-cAMP (3, 10, and 30 µM) to Schwann cells increased levels of 2'-AMP in the medium from 0.006 ± 0.002 to 21 ± 2, 70 ± 3, and 187 ± 10 nM/µg protein, respectively; in contrast, Schwann cells had little ability to convert 2',3'-cAMP to 3'-AMP or 3',5'-cAMP to either 3'-AMP or 5'-AMP. Although Schwann cells slightly converted 2',3'-cAMP and 2'-AMP to adenosine, they did so at very modest rates (e.g., 5- and 3-fold, respectively, more slowly compared with our previously reported studies in oligodendrocytes). Using transected myelinated rat sciatic nerves in culture medium, we observed a time-related increase in endogenous intracellular 2',3'-cAMP and extracellular 2'-AMP. These findings indicate that Schwann cells do not have a robust 3',5'-cAMP-adenosine pathway but do have a 2',3'-cAMP-adenosine pathway; however, because the pathway mostly involves 2'-AMP formation rather than 3'-AMP, and because the conversion of 2'-AMP to adenosine is slow, metabolism of 2',3'-cAMP mostly results in the accumulation of 2'-AMP. Accumulation of 2'-AMP in peripheral nerves postinjury could have pathophysiological consequences. PMID:25998049

  8. 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

  9. Gait in 5-year-old children with idiopathic clubfoot

    PubMed Central

    Lööf, Elin; Andriesse, Hanneke; André, Marie; Böhm, Stephanie; Broström, Eva W

    2016-01-01

    Background and purpose Idiopathic clubfoot can be bilateral or unilateral; however, most studies of gait have assessed clubfoot cases as one uniform group. The contralateral foot in children with unilateral clubfoot has shown deviations in pedobarographic measurements, but it is seldom included in studies of gait. We evaluated gait in children with idiopathic clubfoot, concentrating on foot involvement. Patients and methods Three-dimensional gait analyses of 59 children, mean age 5.4 years, with bilateral (n = 30) or unilateral (n = 29) idiopathic clubfoot were stratified into groups of bilateral, unilateral, or contralateral feet. Age-matched controls (n = 28) were evaluated for comparison. Gait assessment included: (1) discrete kinematic and kinetic parameters, and (2) gait deviation index for kinematics (GDI) and kinetics (GDI-k). Results No differences in gait were found between bilateral and unilateral idiopathic clubfoot, but both groups deviated when compared to controls. Compared to control feet, contralateral feet showed no deviations in discrete gait parameters, but discrepancies were evident in relation to unilateral clubfoot, causing gait asymmetries in children with unilateral involvement. However, all groups deviated significantly from control feet according to GDI and GDI-k. Interpretation Bilateral and unilateral idiopathic clubfoot cases show the same persistent deviations in gait, mainly regarding reduced plantarflexion. Nevertheless, knowledge of foot involvement is important as children with unilateral clubfoot show gait asymmetries, which might give an impression of poorer deviations. The results of GDI/GDI-k indicate global gait adaptations of the contralateral foot, so the foot should preferably not be used as a reference for gait. PMID:27331243

  10. 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

  11. 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

  12. 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

  13. Sleep problems and associated factors in children with juvenile idiopathic arthritis: a systematic review

    PubMed Central

    2014-01-01

    Background Sleep problems are common among children with chronic illnesses such as Juvenile Idiopathic Arthritis (or JIA). However, little is known about the frequency and severity of sleep disturbance(s) and the factors that are associated with sleep problems in children with JIA. The mechanism(s) of the relationships characterizing the development or exacerbation of sleep problems in children with JIA are still unknown, however studies have reported an association. The purpose of this study was to synthesize existing research related to sleep problems in children with JIA. Methods The Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement guided the conduct and reporting of this review. An experienced librarian conducted searches in MEDLINE, EMBASE, PsychINFO, CINAHL, and the Cochrane Central Register of Controlled Trials from inception to January 2012, to identify potentially relevant citations. Two members independently selected, rated methodological quality using the QUIPS tool, and extracted data from included studies. Results Ten studies were included and findings varied across studies; studies were mostly cross-sectional, or case-controlled designs, with only one cohort study available. Four studies found that children and adolescents diagnosed with JIA had significantly more sleep disturbances when compared to healthy controls. Pain was most often associated with sleep disturbances. The heterogeneous findings highlight the complex relationships between JIA and sleep, and low methodological quality of studies in the field. Conclusions This review supports an association between poor sleep and increased symptoms related to JIA, specifically the experience of pain. However, results need to be interpreted cautiously given the inconsistent findings regarding factors associated with sleep problems in JIA, the limited evidence available, and its low quality. Furthermore it is not yet determined if the poor sleep patterns predate the

  14. 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

  15. Mengo virus 3C proteinase: recombinant expression, intergenus substrate cleavage and localization in vivo.

    PubMed

    Hall, D J; Palmenberg, A C

    1996-01-01

    Mengo virus 3C proteinase was cloned and expressed to high levels in a bacterial vector system. The protein was solubilized from inclusion bodies then purified to homogeneity (> 95%) by ion exchange chromatography. The recombinant enzyme was proteolytically active in cell-free processing assays with a Mengo capsid precursor substrate, L-P1-2A, correctly and proficiently cleaving it into L, 1AB, 1C, 1D and 2A protein products. Further analyses with synthetic peptide substrates encompassing the Mengo or rhinovirus-14 2C/3A cleavage sequences, showed the Mengo 3C could recognize and process specific glutamine-glycine sites within these peptides. The reactivity with the rhinovirus peptide was unexpected, because cross-reactivity between a picornavirus 3C enzyme and a protein substrate from different genus of this family has otherwise never been observed. In reciprocal reactions, a rhinovirus-14 3C preparation was unable to cleave the Mengo-derived synthetic peptide substrate. The recombinant Mengo 3C reactions were also characterized with regard to substrate Km, optimum pH and temperature. The protein was additionally used to raise monoclonal antibodies (mAbs) in mice, which in turn localized natural 3C, 3ABC, 3CD and P3 in immunoblots, immunoprecipitations and indirect immunofluorescence assays of Mengo-infected HeLa cells. The monoclonals showed cross-reactivity with 3C and 3C-containing precursors from encephalomyocarditis virus (EMCV), but did not react with 3C proteins from rhinovirus-14 or poliovirus-1M. PMID:8972564

  16. Renal failure after anti-D globulin treatment of idiopathic thrombocytopenic purpura.

    PubMed

    Kees-Folts, Deborah; Abt, Arthur B; Domen, Ronald E; Freiberg, Andrew S

    2002-02-01

    Idiopathic thrombocytopenic purpura (ITP) is a disorder of rapid destruction of antibody-coated platelets. Anti-D immune globulin has been used for treatment of ITP in the United States since 1995. Initial studies identified no significant side effects of treatment. However, a recent report highlighted occasional episodes of intravascular hemolysis after anti-D immune globulin. We describe two children with ITP who developed acute renal failure (ARF) after treatment with anti-D immune globulin and also analyze ten additional cases of ARF reported to the manufacturer, Cangene Corporation, through postmarketing surveillance. All episodes of ARF were associated with intravascular hemolysis. Four patients required dialysis. Patient age ranged from 1 to 82 years, but those requiring dialysis were all under age 15 years. Several patients with ARF had preexisting creatinine elevation. Three of the patients with ARF had serologic evidence of acute Epstein-Barr virus (EBV) infection. Renal biopsy in one patient showed acute tubular necrosis, with findings consistent with pigment nephropathy. Anti-D immune globulin, used to treat ITP, may be associated with intravascular hemolysis and resultant ARF. Renal function should be monitored in patients with evidence of intravascular hemolysis. Children and adolescents may have increased risk of ARF requiring dialysis.

  17. Gpr126/Adgrg6 deletion in cartilage models idiopathic scoliosis and pectus excavatum in mice

    PubMed Central

    Karner, Courtney M.; Long, Fanxin; Solnica-Krezel, Lilianna; Monk, Kelly R.; Gray, Ryan S.

    2015-01-01

    Adolescent idiopathic scoliosis (AIS) and pectus excavatum (PE) are common pediatric musculoskeletal disorders. Little is known about the tissue of origin for either condition, or about their genetic bases. Common variants near GPR126/ADGRG6 (encoding the adhesion G protein-coupled receptor 126/adhesion G protein-coupled receptor G6, hereafter referred to as GPR126) were recently shown to be associated with AIS in humans. Here, we provide genetic evidence that loss of Gpr126 in osteochondroprogenitor cells alters cartilage biology and spinal column development. Microtomographic and x-ray studies revealed several hallmarks of AIS, including postnatal onset of scoliosis without malformations of vertebral units. The mutants also displayed a dorsal-ward deflection of the sternum akin to human PE. At the cellular level, these defects were accompanied by failure of midline fusion within the developing annulus fibrosis of the intervertebral discs and increased apoptosis of chondrocytes in the ribs and vertebrae. Molecularly, we found that loss of Gpr126 upregulated the expression of Gal3st4, a gene implicated in human PE, encoding Galactose-3-O-sulfotransferase 4. Together, these data uncover Gpr126 as a genetic cause for the pathogenesis of AIS and PE in a mouse model. PMID:25954032

  18. Gout in a 15-year-old boy with juvenile idiopathic arthritis: a case study

    PubMed Central

    2014-01-01

    Joint pain is a common complaint in pediatrics and is most often attributed to overuse or injury. In the face of persistent, severe, or recurrent symptoms, the differential typically expands to include bony or structural causes versus rheumatologic conditions. Rarely, a child has two distinct causes for joint pain. In this case, an obese 15-year-old male was diagnosed with gout, a disease common in adults but virtually ignored in the field of pediatrics. The presence of juvenile idiopathic arthritis (JIA) complicated and delayed the consideration of this second diagnosis. Indeed, the absence of gout from this patient’s differential diagnosis resulted in a greater than two-year delay in receiving treatment. The patients’ BMI was 47.4, and he was also mis-diagnosed with osteochondritis dissecans and underwent medical treatment for JIA, assorted imaging studies, and multiple surgical procedures before the key history of increased pain with red meat ingestion, noticed by the patient, and a subsequent elevated uric acid confirmed his ultimate diagnosis. With the increased prevalence of obesity in the adolescent population, the diagnosis of gout should be an important consideration in the differential diagnosis for an arthritic joint in an overweight patient, regardless of age. PMID:24393408

  19. Laparoscopic suture repair of idiopathic gastric perforation in Duchenne muscular dystrophy.

    PubMed

    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.

  20. Gout in a 15-year-old boy with juvenile idiopathic arthritis: a case study.

    PubMed

    Morris, Hallie; Grant, Kristen; Khanna, Geetika; White, Andrew J

    2014-01-06

    Joint pain is a common complaint in pediatrics and is most often attributed to overuse or injury. In the face of persistent, severe, or recurrent symptoms, the differential typically expands to include bony or structural causes versus rheumatologic conditions. Rarely, a child has two distinct causes for joint pain. In this case, an obese 15-year-old male was diagnosed with gout, a disease common in adults but virtually ignored in the field of pediatrics. The presence of juvenile idiopathic arthritis (JIA) complicated and delayed the consideration of this second diagnosis. Indeed, the absence of gout from this patient's differential diagnosis resulted in a greater than two-year delay in receiving treatment. The patients' BMI was 47.4, and he was also mis-diagnosed with osteochondritis dissecans and underwent medical treatment for JIA, assorted imaging studies, and multiple surgical procedures before the key history of increased pain with red meat ingestion, noticed by the patient, and a subsequent elevated uric acid confirmed his ultimate diagnosis. With the increased prevalence of obesity in the adolescent population, the diagnosis of gout should be an important consideration in the differential diagnosis for an arthritic joint in an overweight patient, regardless of age.

  1. ScolioMedIS: web-oriented information system for idiopathic scoliosis visualization and monitoring.

    PubMed

    Devedžić, Goran; Cuković, Saša; Luković, Vanja; Milošević, Danijela; Subburaj, K; Luković, Tanja

    2012-11-01

    Adolescent idiopathic scoliosis is the most common type of abnormal curvature observed in spine and it progresses rapidly during the puberty period. The most followed clinical way of assessing the spinal deformity is subjective by measuring the characteristic angles of spinal curve from a set of radiographic images. This paper presents a web-based information system (called ScolioMedIS) based on parameterized 3D anatomical models of the spine to quantitatively assess the deformity and to minimize the amount of radiation exposure by reducing the number of radiographs required. The main components of the system are 3D parametric solid model of spine, back surfaces, relevant clinical information and scoliosis ontology. The patient-specific spine model is regenerated from the parametric model and surface data using anatomical information extracted from radiographic images. The system is designed to take inherent advantage of Web for facilitating multi-center data collection and collaborative clinical decisions. The preliminary analysis of patient data showed promising results, which involve improved documentation standard, clinical decision knowledge base record, facilitated exchange and retrieval of medical data between institutions in multi-center clinical studies, 3D visualization of spinal deformity, and permanent monitoring of treatments.

  2. Antiferromagnetic resonance in the Mott insulator fcc-Cs3C60.

    PubMed

    Suzuki, Yuta; Shibasaki, Seiji; Kubozono, Yoshihiro; Kambe, Takashi

    2013-09-11

    The magnetic ground state of the fcc phase of the Mott insulator Cs3C60 was studied using a low-temperature electron spin resonance technique, and antiferromagnetic resonance (AFMR) below 1.57 K was directly observed at ambient pressure. The AFMR modes for the fcc phase of Cs3C60 were investigated using a conventional two-sublattice model with uniaxial anisotropy, and the spin-flop field was determined to be 4.7 kOe at 1.57 K. The static magnetic exchange interactions and anisotropy field for fcc-Cs3C60 were also estimated.

  3. Adolescent immunization.

    PubMed

    Handal, G A

    2000-06-01

    The dramatic improvements achieved in the control of vaccine-preventable diseases in children have only been shared partially by adolescents and young adults, as today several million adolescents are not receiving the full complement of vaccines recommended by the Advisory Committee on Immunization Practices (ACIP). This article discusses the reasons for this problem and the tools to bridge this gap. In particular, medical societies and the Centers for Disease Control and Prevention (CDC) recommend a close assessment of the adolescentís immunization status between 11 and 12 years of age, inclusion of school immunization, and providing missing immunizations at any opportunity. The article also addresses other vaccines recommended for groups of adolescents with special needs, reporting information, and provides an update on the vaccines of the future.

  4. Structural Basis for Molecular Discrimination by a 3',3'-cGAMP Sensing Riboswitch

    SciTech Connect

    Ren, Aiming; Wang, Xin  C.; Kellenberger, Colleen  A.; Rajashankar, Kanagalaghatta  R.; Jones, Roger  A.; Hammond, Ming  C.; Patel, Dinshaw  J.

    2015-04-07

    Cyclic dinucleotides are second messengers that target the adaptor STING and stimulate the innate immune response in mammals. Besides protein receptors, there are bacterial riboswitches that selectively recognize cyclic dinucleotides. We recently discovered a natural riboswitch that targets 3',3'-cGAMP, which is distinguished from the endogenous mammalian signal 2',3'-cGAMP by its backbone connectivity. Here, we report on structures of the aptamer domain of the 3',3'-cGAMP riboswitch from Geobacter in the 3',3'-cGAMP and c-di-GMP bound states. The riboswitch adopts a tuning forklike architecture with a junctional ligand-binding pocket and different orientations of the arms are correlated with the identity of the bound cyclic dinucleotide. Subsequent biochemical experiments revealed that specificity of ligand recognition can be affected by point mutations outside of the binding pocket, which has implications for both the assignment and reengineering of riboswitches in this structural class.

  5. Are 3C 120 and Other Active Galactic Nuclei Overweight Microquasars?

    NASA Astrophysics Data System (ADS)

    Marscher, Alan P.

    2005-11-01

    The appearance of superluminal radio knots follows drops in the X-ray flux in the FR1 radio galaxy 3C 120 and possibly the FR2 source 3C 111. This corresponds in a very general way to the behavior of the X-ray binary GRS 1915 + 105, but the light curves of the microquasar are much richer in detail. Starting in 2003.7, the character of the radio and X-ray light curves of 3C 120 changed, perhaps signaling a new stage of activity. I discuss here what one might expect when a microquasar is scaled up to AGN dimensions, and compare this with what we see in 3C 120. There is a mismatch between expectations and observations.

  6. Adolescent sexuality.

    PubMed

    Grant, L M; Demetriou, E

    1988-12-01

    The consequences of adolescent sexual behavior are an enormous burden both for the adolescent and society. The problem is not that teens are sexually active but rather that they have little preparation and guidance in developing responsible sexual behavior. Developmentally, adolescents reach physical maturity before they are cognitively able to appreciate the consequences of their behavior. A teenager's primary source of information regarding sexuality is his or her peer group, all of whom are experiencing and reinforcing the same behaviors. The family, the major socializer of other behaviors, is not as powerful a force in shaping responsible sexual behavior because of parental discomfort with sex education and sexual discussions. This is the result of a social milieu in which sex is frequently portrayed but rarely linked with responsible behavior or accurate, nonjudgmental information. The pediatric practitioner is in an ideal position to intervene in these dynamics. In the office, the practitioner can provide accurate sexual information to both parents and adolescents, support parental-child communication on sexual issues, and provide appropriate services or referral. In the community, the practitioner can advocate for school-based sex education as well as act as an information resource. Finally, the practitioner can advocate for the health care needs for adolescents on a national level, supporting legislation that provides adolescents with information and access to services necessary to make responsible sexual decisions.

  7. Target Cell APOBEC3C Can Induce Limited G-to-A Mutation in HIV-1

    PubMed Central

    Bourara, Khaoula; Liegler, Teri J; Grant, Robert M

    2007-01-01

    The evolutionary success of primate lentiviruses reflects their high capacity to mutate and adapt to new host species, immune responses within individual hosts, and, in recent years, antiviral drugs. APOBEC3G (A3G) and APOBEC3F (A3F) are host cell DNA-editing enzymes that induce extensive HIV-1 mutation that severely attenuates viral replication. The HIV-1 virion infectivity factor (Vif), expressed in vivo, counteracts the antiviral activity of A3G and A3F by inducing their degradation. Other APOBECs may contribute more to viral diversity by inducing less extensive mutations allowing viral replication to persist. Here we show that in APOBEC3C (A3C)-expressing cells infected with the patient-derived HIV-1 molecular clones 210WW, 210WM, 210MW, and 210MM, and the lab-adapted molecular clone LAI, viral G-to-A mutations were detected in the presence of Vif expression. Mutations occurred primarily in the GA context and were relatively infrequent, thereby allowing for spreading infection. The mutations were absent in cells lacking A3C but were induced after transient expression of A3C in the infected target cell. Inhibiting endogenous A3C by RNA interference in Magi cells prevented the viral mutations. Thus, A3C is necessary and sufficient for G-to-A mutations in some HIV-1 strains. A3C-induced mutations occur at levels that allow replication to persist and may therefore contribute to viral diversity. Developing drugs that inhibit A3C may be a novel strategy for delaying viral escape from immune or antiretroviral inhibition. PMID:17967058

  8. AN ASYMMETRICAL SYNCHROTRON MODEL FOR KNOTS IN THE 3C 273 JET

    SciTech Connect

    Liu, Wen-Po; Chen, Y. J.; Wang, Chun-Cheng

    2015-06-20

    To interpret the emission of knots in the 3C 273 jet from radio to X-rays, we propose a synchrotron model in which, owing to the shock compression effect, the injection spectra from a shock into the upstream and downstream emission regions are asymmetric. Our model could well explain the spectral energy distributions of knots in the 3C 273 jet, and predictions regarding the knots’ spectra could be tested by future observations.

  9. Picornaviral 3C cysteine proteinases have a fold similar to the chymotrypsin-like serine proteinases

    SciTech Connect

    Allaire,M.; Chernaia, M.; Malcolm, B.; James, M.

    1994-01-01

    The picornavirus family includes several pathogens such as poliovirus, rhinovirus (the major cause of the common cold), hepatitis A virus and the foot-and-mouth disease virus. Picornaviral proteins are expressed by direct translation of the genomic RNA into a single, large polyprotein precursor. Proteolysis of the viral polyprotein into the mature proteins is assured by the viral 3C enzymes, which are cysteine proteinases. Here we report the X-ray crystal structure at 2.3 {angstrom} resolution of the 3C proteinase from hepatitis A virus (HAV-3C). The overall architecture of HAV-3C reveals a fold resembling that of the chymotrypsin family of serine proteinases, which is consistent with earlier predictions. Catalytic residues include Cys 172 as nucleophile and His 44 as general base. The 3C cleavage specificity for glutamine residues is defined primarily by His 191. The overall structure suggests that an inter-molecular (trans) cleavage releases 3C and that there is an active proteinase in the polyprotein.

  10. Diabetes mellitus secondary to pancreatic diseases (Type 3c)--are we neglecting an important disease?

    PubMed

    Ewald, Nils; Bretzel, Reinhard G

    2013-04-01

    Type 3c diabetes mellitus (T3cDM) is a clinically relevant condition with a prevalence of 5-10% among all diabetic subjects in Western populations. Its prevalence and clinical importance have been underestimated and underappreciated so far. In contrast to the management of type 1 or type 2 diabetes, the endocrinopathy in T3cDM is very complex and complicated by additional present comorbidities such as maldigestion and concommitant qualitative malnutrition. The failure to correctly diagnose T3cDM leads to failure to implement an appropriate medical therapy of these patients. Physicians should screen for important and easily reversable pathological conditions such as exocrine insufficiency, lack of fat-soluble vitamins (especially vitamin D) and impairment of fat hydrolysis and incretin secretion which are found very commonly in T3cDM. Since most patients with T3cDM suffer from chronic pancreatitis, physicians must additionally be aware of the elevated risk of pancreatic cancer in this subset of patients.

  11. Cleavage of interferon regulatory factor 7 by enterovirus 71 3C suppresses cellular responses.

    PubMed

    Lei, Xiaobo; Xiao, Xia; Xue, Qinghua; Jin, Qi; He, Bin; Wang, Jianwei

    2013-02-01

    Enterovirus 71 (EV71) is a positive-stranded RNA virus which is capable of inhibiting innate immunity. Among virus-encoded proteins, the 3C protein compromises the type I interferon (IFN-I) response mediated by retinoid acid-inducible gene-I (RIG-I) or Toll-like receptor 3 that activates interferon regulatory 3 (IRF3) and IRF7. In the present study, we report that enterovirus 71 downregulates IRF7 through the 3C protein, which inhibits the function of IRF7. When expressed in mammalian cells, the 3C protein mediates cleavage of IRF7 rather than that of IRF3. This process is insensitive to inhibitors of caspase, proteasome, lysosome, and autophagy. H40D substitution in the 3C active site abolishes its activity, whereas R84Q or V154S substitution in the RNA binding motif has no effect. Furthermore, 3C-mediated cleavage occurs at the Q189-S190 junction within the constitutive activation domain of IRF7, resulting in two cleaved IRF7 fragments that are incapable of activating IFN expression. Ectopic expression of wild-type IRF7 limits EV71 replication. On the other hand, expression of the amino-terminal domain of IRF7 enhances EV71 infection, which correlates with its ability to interact with and inhibit IRF3. These results suggest that control of IRF7 by the 3C protein may represent a viral mechanism to escape cellular responses. PMID:23175366

  12. Synthesis and electrochemical performance of Ti3C2Tx with hydrothermal process

    NASA Astrophysics Data System (ADS)

    Wang, Libo; Zhang, Heng; Wang, Bo; Shen, Changjie; Zhang, Chuanxiang; Hu, Qianku; Zhou, Aiguo; Liu, Baozhong

    2016-09-01

    In this study, a simple hydrothermal method has been developed to prepare Ti3C2Tx from Ti3AlC2 as a high-performance electrode material for supercapacitors. This method is environmentally friendly and has a low level of danger. The morphology and structure of the Ti3C2Tx can be controlled by hydrothermal reaction time, temperature and NH4F amounts. The prepared Ti3C2Tx was characterized by X-ray diffraction, field emission scanning electron microscopy, Raman spectroscopy, X-ray photoelectron spectroscopy and Brunauer-Emmet-Teller. The results show that the prepared Ti3C2Tx is terminated by O, OH, and F groups. The electrochemical properties of the Ti3C2Tx sample exhibit specific capacitance up to 141 Fcm-3 in 3 M KOH aqueous electrolyte, and even after 1000 cycles, no significant degradation of the volumetric capacitance was observed. These results indicate that the Ti3C2Tx material prepared by this hydrothermal method can be used in high performance supercapacitors.

  13. Binary WC- and Cr3C2-containing hardmetal compositions for thermally sprayed coatings

    NASA Astrophysics Data System (ADS)

    Berger, L.-M.

    2016-03-01

    Compositions of thermally sprayed hardmetal coatings for wear protection are based on the hard materials WC and Cr3C2 with Co and Ni as the most important binders, which are often alloyed with Cr. There are a few commercial compositions containing WC and Cr3C2 together, which have a high potential for the improvement of coating properties, in particular for service in corrosive environments and high temperature applications. However, the combined application of WC and Cr3C2 in the coating compositions leads to very complex reactions between these components both during feedstock powder preparation and the spray process. This contribution summarizes the knowledge about the interactions of WC and Cr3C2 for the most important commercially available compositions: WC-10Co-4Cr, WC-20‘CrC’-7Ni, 45Cr3C2- 37WC-18NiCo. These three compositions show remarkable differences of the interaction between WC and Cr3C2 and the coating properties.

  14. Synthesis and electrochemical performance of Ti3C2Tx with hydrothermal process

    NASA Astrophysics Data System (ADS)

    Wang, Libo; Zhang, Heng; Wang, Bo; Shen, Changjie; Zhang, Chuanxiang; Hu, Qianku; Zhou, Aiguo; Liu, Baozhong

    2016-08-01

    In this study, a simple hydrothermal method has been developed to prepare Ti3C2Tx from Ti3AlC2 as a high-performance electrode material for supercapacitors. This method is environmentally friendly and has a low level of danger. The morphology and structure of the Ti3C2Tx can be controlled by hydrothermal reaction time, temperature and NH4F amounts. The prepared Ti3C2Tx was characterized by X-ray diffraction, field emission scanning electron microscopy, Raman spectroscopy, X-ray photoelectron spectroscopy and Brunauer-Emmet-Teller. The results show that the prepared Ti3C2Tx is terminated by O, OH, and F groups. The electrochemical properties of the Ti3C2Tx sample exhibit specific capacitance up to 141 Fcm-3 in 3 M KOH aqueous electrolyte, and even after 1000 cycles, no significant degradation of the volumetric capacitance was observed. These results indicate that the Ti3C2Tx material prepared by this hydrothermal method can be used in high performance supercapacitors. [Figure not available: see fulltext.

  15. Atomic-scale recognition of surface structure and intercalation mechanism of Ti3C2X.

    PubMed

    Wang, Xuefeng; Shen, Xi; Gao, Yurui; Wang, Zhaoxiang; Yu, Richeng; Chen, Liquan

    2015-02-25

    MXenes represent a large family of functionalized two-dimensional (2D) transition-metal carbides and carbonitrides. However, most of the understanding on their unique structures and applications stops at the theoretical suggestion and lack of experimental support. Herein, the surface structure and intercalation chemistry of Ti3C2X are clarified at the atomic scale by aberration-corrected scanning transmission electron microscope (STEM) and density functional theory (DFT) calculations. The STEM studies show that the functional groups (e.g., OH(-), F(-), O(-)) and the intercalated sodium (Na) ions prefer to stay on the top sites of the centro-Ti atoms and the C atoms of the Ti3C2 monolayer, respectively. Double Na-atomic layers are found within the Ti3C2X interlayer upon extensive Na intercalation via two-phase transition and solid-solution reactions. In addition, aluminum (Al)-ion intercalation leads to horizontal sliding of the Ti3C2X monolayer. On the basis of these observations, the previous monolayer surface model of Ti3C2X is modified. DFT calculations using the new modeling help to understand more about their physical and chemical properties. These findings enrich the understanding of the MXenes and shed light on future material design and applications. Moreover, the Ti3C2X exhibits prominent rate performance and long-term cycling stability as an anode material for Na-ion batteries. PMID:25688582

  16. 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

  17. Image reconstruction algorithm for optically stimulated luminescence 2D dosimetry using laser-scanned Al2O3:C and Al2O3:C,Mg films

    NASA Astrophysics Data System (ADS)

    Ahmed, M. F.; Schnell, E.; Ahmad, S.; Yukihara, E. G.

    2016-10-01

    The objective of this work was to develop an image reconstruction algorithm for 2D dosimetry using Al2O3:C and Al2O3:C,Mg optically stimulated luminescence (OSL) films imaged using a laser scanning system. The algorithm takes into account parameters associated with detector properties and the readout system. Pieces of Al2O3:C films (~8 mm  ×  8 mm  ×  125 µm) were irradiated and used to simulate dose distributions with extreme dose gradients (zero and non-zero dose regions). The OSLD film pieces were scanned using a custom-built laser-scanning OSL reader and the data obtained were used to develop and demonstrate a dose reconstruction algorithm. The algorithm includes corrections for: (a) galvo hysteresis, (b) photomultiplier tube (PMT) linearity, (c) phosphorescence, (d) ‘pixel bleeding’ caused by the 35 ms luminescence lifetime of F-centers in Al2O3, (e) geometrical distortion inherent to Galvo scanning system, and (f) position dependence of the light collection efficiency. The algorithm was also applied to 6.0 cm  ×  6.0 cm  ×  125 μm or 10.0 cm  ×  10.0 cm  ×  125 µm Al2O3:C and Al2O3:C,Mg films exposed to megavoltage x-rays (6 MV) and 12C beams (430 MeV u‑1). The results obtained using pieces of irradiated films show the ability of the image reconstruction algorithm to correct for pixel bleeding even in the presence of extremely sharp dose gradients. Corrections for geometric distortion and position dependence of light collection efficiency were shown to minimize characteristic limitations of this system design. We also exemplify the application of the algorithm to more clinically relevant 6 MV x-ray beam and a 12C pencil beam, demonstrating the potential for small field dosimetry. The image reconstruction algorithm described here provides the foundation for laser-scanned OSL applied to 2D dosimetry.

  18. Role of CNPase in the oligodendrocytic extracellular 2',3'-cAMP-adenosine pathway.

    PubMed

    Verrier, Jonathan D; Jackson, Travis C; Gillespie, Delbert G; Janesko-Feldman, Keri; Bansal, Rashmi; Goebbels, Sandra; Nave, Klaus-Armin; Kochanek, Patrick M; Jackson, Edwin K

    2013-10-01

    Extracellular adenosine 3',5'-cyclic monophosphate (3',5'-cAMP) is an endogenous source of localized adenosine production in many organs. Recent studies suggest that extracellular 2',3'-cAMP (positional isomer of 3',5'-cAMP) is also a source of adenosine, particularly in the brain in vivo post-injury. Moreover, in vitro studies show that both microglia and astrocytes can convert extracellular 2',3'-cAMP to adenosine. Here, we examined the ability of primary mouse oligodendrocytes and neurons to metabolize extracellular 2',3'-cAMP and their respective adenosine monophosphates (2'-AMP and 3'-AMP). Cells were also isolated from mice deficient in 2',3'-cyclic nucleotide-3'-phosphodiesterase (CNPase). Oligodendrocytes metabolized 2',3'-cAMP to 2'-AMP with 10-fold greater efficiency than did neurons (and also more than previously examined microglia and astrocytes); whereas, the production of 3'-AMP was minimal in both oligodendrocytes and neurons. The production of 2'-AMP from 2',3'-cAMP was reduced by 65% in CNPase -/- versus CNPase +/+ oligodendrocytes. Oligodendrocytes also converted 2'-AMP to adenosine, and this was also attenuated in CNPase -/- oligodendrocytes. Inhibition of classic 3',5'-cAMP-3'-phosphodiesterases with 3-isobutyl-1-methylxanthine did not block metabolism of 2',3'-cAMP to 2'-AMP and inhibition of classic ecto-5'-nucleotidase (CD73) with α,β-methylene-adenosine-5'-diphosphate did not attenuate the conversion of 2'-AMP to adenosine. These studies demonstrate that oligodendrocytes express the extracellular 2',3'-cAMP-adenosine pathway (2',3'-cAMP → 2'-AMP → adenosine). This pathway is more robustly expressed in oligodendrocytes than in all other CNS cell types because CNPase is the predominant enzyme that metabolizes 2',3'-cAMP to 2-AMP in CNS cells. By reducing levels of 2',3'-cAMP (a mitochondrial toxin) and increasing levels of adenosine (a neuroprotectant), oligodendrocytes may protect axons from injury. PMID:23922219

  19. Idiopathic acquired dacryocystocele treated with endonasal endoscopic dacryocystorhinostomy

    PubMed Central

    Koltsidopoulos, Petros; Papageorgiou, Elena; Konidaris, Vasileios Efstathios; Skoulakis, Charalambos

    2013-01-01

    A 64-year-old woman presented with a medial canthal mass in her left eye, which was accompanied only by mild epiphora. There was no history of dacryocystitis, bloody tears, midfacial trauma or surgery. Physical examination showed a non-inflammatory, subcutaneous, immobile mass below the level of the medial canthal tendon. Lacrimal irrigation demonstrated blockage at the nasolacrimal duct. A CT revealed a non-enhancing, low density, cystic lesion in the inferomedial aspect of the left orbit without bony erosion, which was compatible with an idiopathic acquired dacryocystocele. The patient underwent endonasal endoscopic dacryocystorhinostomy (DCR) and silicone intubation. Epiphora resolved immediately after surgery. Two years after surgery, the patient has had no recurrence of either the epiphora or the orbital. Idiopathic acquired dacryocystocele associated only with epiphora without accompanying dacryocystitis although rare should be considered in the differential diagnosis of acquired non-inflammatory medial canthal masses. Endonasal endoscopic DCR represents a safe and effective treatment. PMID:23960153

  20. 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.

  1. Juvenile idiopathic arthritis in the new world of biologics.

    PubMed

    Ostring, Genevieve Tyra; Singh-Grewal, Davinder

    2013-09-01

    Juvenile idiopathic arthritis results in significant pain and disability in both children and adults. Advances in treatment resulting in improved long-term outcomes have occurred; however, an emphasis on early and aggressive diagnosis and management hopes to improve outcomes further. Juvenile idiopathic arthritis remains a clinical diagnosis of exclusion, but further research may delineate biological markers associated with the disease and its subtypes. Therapy for patients includes intra-articular steroid injections, disease modifying agents such as methotrexate and biological agents. Biological agents have provided exciting new therapeutic options in the last decade; however, long-term side effects of modulating the immune system are not yet fully understood. Systemic steroids may also be required but their long-term use is avoided. Uveitis needs to be screened for in all of those with the diagnosis. Multidisciplinary team care is required in managing these young people.

  2. Canakinumab for the treatment of systemic juvenile idiopathic arthritis.

    PubMed

    Grom, Alexei A

    2014-11-01

    The introduction of methotrexate in the 1980s and of TNF-inhibiting agents and abatacept in the late 1990s led to a dramatic improvement in the outcomes of non-systemic categories of juvenile idiopathic arthritis. By contrast, the same treatment approaches had no strong impact on the outcome of systemic juvenile idiopathic arthritis (SJIA), and the main effective treatment in these patients remained glucocorticoids with their known side effects. Encouraging findings in small studies involving SJIA patients treated with IL-1 and IL-6 inhibitors led to large Phase III trials, and the results in these trials provide hope that substantial joint damage and disability seen in the majority of patients with persistent SJIA can be prevented. The purpose of this review is to discuss the safety and efficacy of the IL-1 and IL-6 inhibiting agents in SJIA with the main focus on canakinumab, a fully human monoclonal anti-IL-1β antibody.

  3. Hyperbaric oxygen therapy for idiopathic sudden sensorineural hearing loss.

    PubMed

    Murphy-Lavoie, H; Piper, S; Moon, R E; Legros, T

    2012-01-01

    Idiopathic sudden sensorineural hearing loss (ISSHL) is the newest indication approved by the Undersea and Hyperbaric Medical Society's Hyperbaric Oxygen Therapy Committee. Idiopathic sudden sensorineural hearing loss appears to be characterized by hypoxia in the perilymph and therefore the scala tympani and the organ of Corti. A review of the literature reveals more than 100 publications evaluating the use of hyperbaric oxygen (HBO2) for the treatment of ISSHL, including eight randomized controlled trials. The best and most consistent results are obtained when HBO2 is initiated within two weeks of symptom onset and combined with corticosteroid treatment. The average hearing gain is 19.3 dB for moderate hearing loss and 37.7 dB for severe cases. This improvement brings hearing deficits from the moderate/severe range into the slight/no impairment range. This is a significant gain that can markedly improve a patient's quality of life, both clinically and functionally.

  4. 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

  5. Idiopathic genital ulcers in women infected with human immunodeficiency virus.

    PubMed

    Anderson, J; Clark, R A; Watts, D H; Till, M; Arrastia, C; Schuman, P; Cohn, S E; Young, M; Bessen, L; Greenblatt, R; Vogler, M; Swindells, S; Boyer, P

    1996-12-01

    A national survey of investigators caring for human immunodeficiency virus (HIV)-infected women was undertaken to describe the clinical presentation of idiopathic genital ulcer disease. Patients with negative syphilis and herpes simplex testing and/or negative genital ulcer biopsy were included in this study. Study participants (n = 29) were generally severely immunocompromised (median CD4 cell count was 50/mm3, and 68% had an acquired immunodeficiency syndrome [AIDS]-defining opportunistic process). Thirty-seven percent had coexistent oral ulcers and 19% had their genital ulcer progress to fistula formation (four rectovaginal and one vaginal-perineal). There was generally a favorable response to topical, systemic, and intralesional steroid treatment. This study suggests that idiopathic or probable aphthous genital ulcers in women have similar clinical characteristics to aphthous oroesophageal ulcers. Although infrequent, these genital ulcers can cause severe morbidity. Further research is warranted to better define the pathophysiology and optimal management.

  6. A geriatric patient with diffuse idiopathic skeletal hyperostosis

    PubMed Central

    Karadag, Berrin; Cat, Huseyin; Aksoy, Selma; Ozulu, Banu; Ozturk, Ali Osman; Oguz, Sukru; Altuntas, Yuksel

    2010-01-01

    The most frequent health problems seen in senility are chronic and degenerative diseases. A 75-year-old male patient with the complaints of weight loss and difficulty in swallowing was admitted to our hospital from a nursing home. Upper system fiber-optic gastrointestinal endoscopy was performed and a mass at the junction of the hypopharynx and esophagus just below recessus piriformis obstructing almost the whole of the lumen and blocking the distal passage was detected. Computed tomography revealed marked narrowing secondary to osseous hypertrophy in the air column of the hypopharynx and proximal esophagus. Diffuse idiopathic skeletal hyperostosis or Forestier’s disease is an idiopathic disease characterized by the ossification of the anterior longitudinal ligament of vertebra and some of the extraspinal ligaments. In the present case we aim to discuss an elderly patient who suffered from dysphagia and weight loss and the diagnostic stages. PMID:20355249

  7. 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

  8. 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

  9. Magnetic resonance imaging in the idiopathic inflammatory myopathies.

    PubMed

    Fraser, D D; Frank, J A; Dalakas, M; Miller, F W; Hicks, J E; Plotz, P

    1991-11-01

    We examined the usefulness of magnetic resonance imaging (MRI) in detecting active muscle disease in 40 patients with idiopathic inflammatory myopathies (IIM). Ten patients without evidence of an inflammatory neuromuscular disease were also studied. The fat-suppressive (STIR) image signal intensity correlated with clinical disease activity and, in most cases, with the presence of inflammation on muscle biopsy. Increased STIR signal intensity paralleled disease activity in 3 patients followed serially. MRI provided a detailed anatomic view of the extent of muscle changes in these diseases. Because of inherent limitations of other measures of disease in these disorders, MRI may prove to be a useful complimentary test for assessing disease activity and guiding therapeutic decisions and biopsy in the idiopathic inflammatory myopathies.

  10. Behavioral Problems in Juvenile Idiopathic Arthritis: A Controlled Study to Examine the Risk of Psychopathology in a Chronic Pediatric Disorder

    PubMed Central

    Chamanara, Elham; Raeeskarami, Seyed-Reza

    2016-01-01

    Children with juvenile idiopathic arthritis (JIA) are prone to the problems that can delay their psychosocial development; however, the existing literature has not reached a consensus on the psychological problems related to JIA. A total of 51 children and adolescents with JIA and 75 healthy controls aged 6 to 18 years were examined using the Child Behavioral Checklist (CBCL). Our results represented that 70 percent of JIA group reached “borderline clinical” range or “clinical” range in internalizing problems, while this percentage in the control group was 18 percent. In addition, our results indicated that JIA group has gotten significantly higher scores (more than twofold) in externalizing behaviors compared to control group. Furthermore, children with JIA showed higher rate of anxiety/depression, withdrawal/depression, somatic complaints, rule breaking behaviors, and aggressive behaviors as well as thought and social problems compared to control group (p < 0.001). As a conclusion, children and adolescents with JIA compared to healthy controls may show higher rate of both internalizing and externalizing problems. Furthermore, our novel findings on externalizing, social, and thought problems in JIA warrant further investigation on affected children who may be at greater risk of future psychopathologies. PMID:27656678

  11. Behavioral Problems in Juvenile Idiopathic Arthritis: A Controlled Study to Examine the Risk of Psychopathology in a Chronic Pediatric Disorder

    PubMed Central

    Chamanara, Elham; Raeeskarami, Seyed-Reza

    2016-01-01

    Children with juvenile idiopathic arthritis (JIA) are prone to the problems that can delay their psychosocial development; however, the existing literature has not reached a consensus on the psychological problems related to JIA. A total of 51 children and adolescents with JIA and 75 healthy controls aged 6 to 18 years were examined using the Child Behavioral Checklist (CBCL). Our results represented that 70 percent of JIA group reached “borderline clinical” range or “clinical” range in internalizing problems, while this percentage in the control group was 18 percent. In addition, our results indicated that JIA group has gotten significantly higher scores (more than twofold) in externalizing behaviors compared to control group. Furthermore, children with JIA showed higher rate of anxiety/depression, withdrawal/depression, somatic complaints, rule breaking behaviors, and aggressive behaviors as well as thought and social problems compared to control group (p < 0.001). As a conclusion, children and adolescents with JIA compared to healthy controls may show higher rate of both internalizing and externalizing problems. Furthermore, our novel findings on externalizing, social, and thought problems in JIA warrant further investigation on affected children who may be at greater risk of future psychopathologies.

  12. 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

    2016-01-06

    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.

  13. Behavioral Problems in Juvenile Idiopathic Arthritis: A Controlled Study to Examine the Risk of Psychopathology in a Chronic Pediatric Disorder.

    PubMed

    Memari, Amir Hossein; Chamanara, Elham; Ziaee, Vahid; Kordi, Ramin; Raeeskarami, Seyed-Reza

    2016-01-01

    Children with juvenile idiopathic arthritis (JIA) are prone to the problems that can delay their psychosocial development; however, the existing literature has not reached a consensus on the psychological problems related to JIA. A total of 51 children and adolescents with JIA and 75 healthy controls aged 6 to 18 years were examined using the Child Behavioral Checklist (CBCL). Our results represented that 70 percent of JIA group reached "borderline clinical" range or "clinical" range in internalizing problems, while this percentage in the control group was 18 percent. In addition, our results indicated that JIA group has gotten significantly higher scores (more than twofold) in externalizing behaviors compared to control group. Furthermore, children with JIA showed higher rate of anxiety/depression, withdrawal/depression, somatic complaints, rule breaking behaviors, and aggressive behaviors as well as thought and social problems compared to control group (p < 0.001). As a conclusion, children and adolescents with JIA compared to healthy controls may show higher rate of both internalizing and externalizing problems. Furthermore, our novel findings on externalizing, social, and thought problems in JIA warrant further investigation on affected children who may be at greater risk of future psychopathologies. PMID:27656678

  14. 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.

  15. Helicobacter pylori-negative, non-steroidal anti-inflammatory drug: Negative idiopathic ulcers in Asia

    PubMed Central

    Iijima, Katsunori; Kanno, Takeshi; Koike, Tomoyuki; Shimosegawa, Tooru

    2014-01-01

    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

  16. 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.

  17. [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

  18. 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

  19. Simultaneous occurrence of idiopathic lipemic tears and massive seborrhea.

    PubMed

    Hebert, A A; Jorizzo, J L; Schoen, I; Graham, G; Weiss, G

    1985-01-01

    A 42-year-old man had idiopathic lipemic tears and massive seborrhea. Recent investigations have shown that cholesterol and other lipids occasionally found in tears are produced by meibomian rather than lacrimal glands. The finding in this patient of hypersecretion by both sebaceous glands and meibomian glands, a sebaceous gland variant, suggests that a common mechanism may regulate secretory control of both of these glands.

  20. 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

  1. Severe pulmonary hypertension in idiopathic nonspecific interstitial pneumonia

    PubMed Central

    Hallowell, Robert W.; Reed, Robert M.; Fraig, Mostafa; Horton, Maureen R.; Girgis, Reda E.

    2012-01-01

    Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD), particularly in idiopathic pulmonary fibrosis (IPF) and ILD associated with connective tissue disease, where the underlying pathology is often a nonspecific interstitial pneumonia (NSIP) pattern. The degree of PH in ILD is typically mild to moderate and radiographic changes of ILD are usually prominent. We describe four patients with idiopathic NSIP and severe PH (mPAP > 40 mmHg). The average mean pulmonary artery pressure was 51±7 mmHg and pulmonary vascular resistance was 13±4 Wood's units. Pulmonary function was characterized by mild restriction (total lung capacity 63–94% predicted) and profound reductions in DLCO (19–53% predicted). Computed tomographic imaging revealed minimal to moderate interstitial thickening without honeycombing. In two of the cases, an initial clinical diagnosis of idiopathic pulmonary arterial hypertension was made. Both were treated with intravenous epoprostenol, which was associated with worsening of hypoxemia. All four patients died or underwent lung transplant within 4 years of PH diagnonsis. Lung pathology in all four demonstrated fibrotic NSIP with medial thickening of the small and medium pulmonary arteries, and proliferative intimal lesions that stained negative for endothelial markers (CD31 and CD34) and positive for smooth muscle actin. There were no plexiform lesions. Severe pulmonary hypertension can therefore occur in idiopathic NSIP, even in the absence of advanced radiographic changes. Clinicians should suspect underlying ILD as the basis for PH when DLCO is severely reduced or gas exchange deteriorates with pulmonary vasodilator therapy. PMID:22558525

  2. Idiopathic hypertrophic cranial pachymenigitis - A long follow-up needed.

    PubMed

    Hashmi, M A; Gautam, G; Sengupta, P; Singh, H; Haque, N

    2011-07-01

    Idiopathic hypertrophic cranial pachymenigitis is a rare clinical condition caused by localized or diffuse inflammatory thickening of dura matter. Described here is a person having diffuse thickening of dura matter of base of skull and he was on follow-up treatment for 5 years with us. Diagnosis was done by excluding other conditions and with biopsy. The patient responded to steroid and the MRI picture, which is given serially, shows improvement. PMID:22347338

  3. [Angiodynography of idiopathic varicocele compared to conventional methods].

    PubMed

    Thomas, C; Dörnberger, V; Lenz, M

    1989-05-01

    21 patients with left-sided idiopathic varicoceles were examined by angiodynography, Doppler sonography and phlebography. Patients treated by sclerosis therapy were re-examined by angiodynography and Doppler sonography after six weeks. Both methods permitted classification into a "stop & shunt type". Angiodynography, particularly for small varicoceles, was quicker and more pleasant for the patient. Real time imaging by angiodynography makes it possible to evaluate pre- and posttherapeutic changes in the pampiniform plexus and in the scrotum.

  4. 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. PMID:27540467

  5. Antifungal effects of synthetic human β-defensin 3-C15 peptide

    PubMed Central

    Ahn, Ki-Bum; Kim, Christine; Kum, Jong-Won; Gu, Yu; Han, Seung Hyun; Shon, Won-Jun; Lee, Woocheol; Zhu, Qiang

    2016-01-01

    Objectives The purpose of this ex vivo study was to compare the antifungal activity of a synthetic peptide consisting of 15 amino acids at the C-terminus of human β-defensin 3 (HBD3-C15) with calcium hydroxide (CH) and Nystatin (Nys) against Candida albicans (C. albicans) biofilm. Materials and Methods C. albicans were grown on cover glass bottom dishes or human dentin disks for 48 hr, and then treated with HBD3-C15 (0, 12.5, 25, 50, 100, 150, 200, and 300 µg/mL), CH (100 µg/mL), and Nys (20 µg/mL) for 7 days at 37℃. On cover glass, live and dead cells in the biomass were measured by the FilmTracer Biofilm viability assay, and observed by confocal laser scanning microscopy (CLSM). On dentin, normal, diminished and ruptured cells were observed by field-emission scanning electron microscopy (FE-SEM). The results were subjected to a two-tailed t-test, a one way analysis variance and a post hoc test at a significance level of p = 0.05. Results C. albicans survival on dentin was inhibited by HBD3-C15 in a dose-dependent manner. There were fewer aggregations of C. albicans in the groups of Nys and HBD3-C15 (≥ 100 µg/mL). CLSM showed C. albicans survival was reduced by HBD3-C15 in a dose dependent manner. Nys and HBD3-C15 (≥ 100 µg/mL) showed significant fungicidal activity compared to CH group (p < 0.05). Conclusions Synthetic HBD3-C15 peptide (≥ 100 µg/mL) and Nys exhibited significantly higher antifungal activity than CH against C. albicans by inhibiting cell survival and biofilm. PMID:27200276

  6. Experimental Investigation of the Cr3C2 C Peritectic Fixed Point

    NASA Astrophysics Data System (ADS)

    Zheng, W.; Yamada, Y.; Wang, Y.

    2008-06-01

    The Cr3C2 C (1,826°C) peritectic point was investigated for its performance as a high-temperature fixed point. Dependence on the impurity content was observed, although it was less severe for the higher of the two equilibrium temperatures obtained with the same cell, the Cr3C2 C peritectic point, than for the lower, the Cr7C3 Cr3C2 eutectic point. Thermal history had an effect on the melting plateau duration, but not on the point-of-inflection temperature nor on the melting range. The melting rate had no apparent effect. The repeatability evaluated as the standard deviation of the repeated melting plateaux within a day was 20 mK for the Cr3C2 C peritectic point, while for the Cr7C3 Cr3C2 eutectic point, this was 210 mK. For both the Cr3C2 C peritectic and the Cr7C3 Cr3C2 eutectic, the freezing plateaux often showed deep supercools, which made them unsuitable for use. The observed good repeatability shows the peritectic-point performance to be comparable to the best MC-eutectic high-temperature fixed points investigated so far. The insensitivity to thermal history constitutes an important and practical advantage. The low price of chromium is a clear benefit as compared to Pt C (1,738°C) or Ru C (1,953°C) eutectic points, the M C eutectic points in this temperature range.

  7. 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

  8. Facts and controversies in the classification of idiopathic interstitial pneumonias.

    PubMed

    Poletti, V; Kitaichi, M

    2000-10-01

    Idiopathic interstitial pneumonias are defined from the pathological point of view as non granulomatous intralobular inflammatory and fibrotic processes involving the alveolar walls. More than thirty years ago Liebow and Carrington pioneered the notion that morphological characteristics could be used with benefit in separating the different entities found in this group, which present with typical, but not pathognomonic clinical features. In the mid-1980s some entities, including giant cell interstitial pneumonia (GIP) and lymphocytic interstitial pneumonia (LIP), were removed from this group and considered as peculiar forms. In the early 90s the concept of cellular or nonspecific interstitial pneumonia was reconsidered, leading to an in depth revision of various types of interstitial pneumonia of unknown etiology. The histological pattern observed in patients with idiopathic pulmonary fibrosis is now referred to as usual interstitial pneumonia (UIP). Other entities that have been revised during the last ten years are desquamative interstitial pneumonia/alveolar macrophage pneumonia (DIP/AMP), respiratory bronchiolitis-interstitial lung disease (RB-ILD), acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP) and nonspecific interstitial pneumonia (NSIP). This paper provides a detailed description of pulmonary disorders which have been included in the new classification systems of idiopathic interstitial pneumonias. In the second part of the paper we will discuss several doubts and controversies that this new classification schemes leave unresolved.

  9. Breast asymmetry pattern in women with idiopathic scoliosis.

    PubMed

    Cruz, Norma I; Korchin, Leo

    2013-01-01

    Breast asymmetry is frequent in women with idiopathic scoliosis. To understand the pattern of breast asymmetry in these women a clinical study was performed in which 54 female patients with idiopathic scoliosis were evaluated. The information recorded for each patient included: age, weight, height, scoliosis type, Cobb angle, breast measurements, and presence of rib cage asymmetry. Breast volume was calculated using anatomic measurements (anthropomorphic method). The mean age of the group was 25 +/- 7 years. A right convex thoracic curve occurred in 85%, with a mean Cobb angle of 32 +/- 15 degrees. Our study indicated that women with idiopathic scoliosis consistently presented breast asymmetry that followed a predictable pattern. The breast on the side of the convex thoracic scoliosis curve is always smaller in volume (mean difference 59 +/- 39 mi). The affected side also presents a smaller areola, a higher position of the nipple (mean difference 2.2 +/- 1.3 cm) and a higher position of the inframammary fold (mean difference 2.1 +/- 1.4 cm) when compared to the opposite breast. Though the asymmetry is predictable, the degree to which the patient presents these changes does not correlate with the severity of the scoliosis (Cobb angle). We believe that the severity of the asymmetry is a result of the difference between the hypoplastic breast and the normal breasts. In women with very large opposite breasts the asymmetry appears to be worse.

  10. Idiopathic hyposmia as a preclinical sign of Parkinson's disease.

    PubMed

    Ponsen, Mirthe M; Stoffers, Diederick; Booij, Jan; van Eck-Smit, Berthe L F; Wolters, Erik Ch; Berendse, Henk W

    2004-08-01

    Olfactory dysfunction is an early and common symptom in Parkinson's disease (PD). In an effort to determine whether otherwise unexplained (idiopathic) olfactory dysfunction is associated with an increased risk of developing PD, we designed a prospective study in a cohort of 361 asymptomatic relatives (parents, siblings, or children) of PD patients. A combination of olfactory detection, identification, and discrimination tasks was used to select groups of hyposmic (n = 40) and normosmic (n = 38) individuals for a 2-year clinical follow-up evaluation and sequential single-photon emission computed tomography (SPECT), using [123I]beta-CIT as a dopamine transporter ligand, to assess nigrostriatal dopaminergic function at baseline and 2 years from baseline. A validated questionnaire, sensitive to the presence of parkinsonism, was used in the follow-up of the remaining 283 relatives. Two years from baseline, 10% of the individuals with idiopathic hyposmia, who also had strongly reduced [123I]beta-CIT binding at baseline, had developed clinical PD as opposed to none of the other relatives in the cohort. In the remaining nonparkinsonian hyposmic relatives, the average rate of decline in dopamine transporter binding was significantly higher than in the normosmic relatives. These results indicate that idiopathic olfactory dysfunction is associated with an increased risk of developing PD of at least 10%. PMID:15293269

  11. Adolescent loneliness.

    PubMed

    Williams, E G

    1983-01-01

    The purpose of this study was to investigate the phenomenon of loneliness in delinquent adolescents with regard to types of delinquency offenses committed, demographic characteristics, and personality characteristics in the areas of interpersonal needs for inclusion, control, and affection. The types of delinquency offenses were categorized as burglary, runaway, drugs, assault, and incorrigible. The demographic variables examined were age, sex, race, family rank or birth order, family structure in terms of parental presence, family income level, religion, and geographic locale. A sample of 98 adolescents was obtained from juvenile detention facilities in three metropolitan areas in the United States. Subjects ranged in age from 12 to 18. Subjects were asked to complete the Loneliness Questionnaire as a self-reported measure of loneliness. Subjects also provided information about themselves relative to demographic characteristics and completed the FIRO-B Questionnaire, which measured interpersonal needs for inclusion, control, and affection. The results revealed no significant differences with regard to any of the demographic variables when the effect of each was considered alone. However, there was a significant effect on loneliness by income when considered jointly with other demographic variables. Adolescents from the middle income group expressed more loneliness than those from the upper and lower income groups. No significant differences were observed with regard to personality characteristics related to interpersonal needs for inclusion and affection, but delinquent adolescents with medium to high needs for control indicated significantly more feelings of loneliness than delinquent adolescents with low needs for control.

  12. Adaptation and validation of the Urticaria Patient Daily Diary for adolescents.

    PubMed

    Mathias, Susan D; Tschosik, Elizabeth A; Zazzali, James L

    2012-01-01

    The Urticaria Patient Daily Diary, including the Urticaria Activity Score, has recently been validated in adults with chronic idiopathic urticaria (CIU), but its validity in adolescents is unknown. This study was designed to (1) assess the content validity of the Adolescent Urticaria Patient Daily Diary and, (2) collect exploratory data on symptom experiences, sleep interference, and health-related quality of life (HRQOL) of adolescents with CIU. The Urticaria Patient Daily Diary was modified to increase its relevance with an adolescent population. A qualitative, cross-sectional, multicenter study was then conducted in the United States so that adolescent subjects could provide information on the impact of urticaria on their lives and comment on the diary. Data were collected via in-person semistructured interviews with subjects 12-17 years of age with moderate-to-severe CIU. The most bothersome symptom was itching (44%). The impact of CIU on HRQOL varied. The majority of subjects (78%) reported waking up at least once a night. Overall, subjects found the diary to be clear, easy to comprehend, and easy to complete. Revisions were made to the diary based on feedback from subjects. After nine interviews, no new information was received. The symptoms of CIU are bothersome to adolescents, particularly itch, and urticaria has a negative impact on the sleep of adolescent patients. The final Adolescent Urticaria Patient Daily Diary has evidence of content validity in patients with CIU ranging from 12 to 17 years of age.

  13. A Single Nucleotide Polymorphism in Human APOBEC3C Enhances Restriction of Lentiviruses

    PubMed Central

    Wittkopp, Cristina J.; Adolph, Madison B.; Wu, Lily I.; Chelico, Linda; Emerman, Michael

    2016-01-01

    Humans express seven human APOBEC3 proteins, which can inhibit viruses and endogenous retroelements through cytidine deaminase activity. The seven paralogs differ in the potency of their antiviral effects, as well as in their antiviral targets. One APOBEC3, APOBEC3C, is exceptional as it has been found to only weakly block viruses and endogenous retroelements compared to other APOBEC3s. However, our positive selection analyses suggest that APOBEC3C has played a role in pathogen defense during primate evolution. Here, we describe a single nucleotide polymorphism in human APOBEC3C, a change from serine to isoleucine at position 188 (I188) that confers potent antiviral activity against HIV-1. The gain-of-function APOBEC3C SNP results in increased enzymatic activity and hypermutation of target sequences when tested in vitro, and correlates with increased dimerization of the protein. The I188 is widely distributed in human African populations, and is the ancestral primate allele, but is not found in chimpanzees or gorillas. Thus, while other hominids have lost activity of this antiviral gene, it has been maintained, or re-acquired, as a more active antiviral gene in a subset of humans. Taken together, our results suggest that APOBEC3C is in fact involved in protecting hosts from lentiviruses. PMID:27732658

  14. Subcellular object quantification with Squassh3C and SquasshAnalyst.

    PubMed

    Rizk, Aurélien; Mansouri, Maysam; Ballmer-Hofer, Kurt; Berger, Philipp

    2015-11-01

    Quantitative image analysis plays an important role in contemporary biomedical research. Squassh is a method for automatic detection, segmentation, and quantification of subcellular structures and analysis of their colocalization. Here we present the applications Squassh3C and SquasshAnalyst. Squassh3C extends the functionality of Squassh to three fluorescence channels and live-cell movie analysis. SquasshAnalyst is an interactive web interface for the analysis of Squassh3C object data. It provides segmentation image overview and data exploration, figure generation, object and image filtering, and a statistical significance test in an easy-to-use interface. The overall procedure combines the Squassh3C plug-in for the free biological image processing program ImageJ and a web application working in conjunction with the free statistical environment R, and it is compatible with Linux, MacOS X, or Microsoft Windows. Squassh3C and SquasshAnalyst are available for download at www.psi.ch/lbr/SquasshAnalystEN/SquasshAnalyst.zip.

  15. Subcellular object quantification with Squassh3C and SquasshAnalyst.

    PubMed

    Rizk, Aurélien; Mansouri, Maysam; Ballmer-Hofer, Kurt; Berger, Philipp

    2015-11-01

    Quantitative image analysis plays an important role in contemporary biomedical research. Squassh is a method for automatic detection, segmentation, and quantification of subcellular structures and analysis of their colocalization. Here we present the applications Squassh3C and SquasshAnalyst. Squassh3C extends the functionality of Squassh to three fluorescence channels and live-cell movie analysis. SquasshAnalyst is an interactive web interface for the analysis of Squassh3C object data. It provides segmentation image overview and data exploration, figure generation, object and image filtering, and a statistical significance test in an easy-to-use interface. The overall procedure combines the Squassh3C plug-in for the free biological image processing program ImageJ and a web application working in conjunction with the free statistical environment R, and it is compatible with Linux, MacOS X, or Microsoft Windows. Squassh3C and SquasshAnalyst are available for download at www.psi.ch/lbr/SquasshAnalystEN/SquasshAnalyst.zip. PMID:26554508

  16. Capacitance of Ti3C2Tx MXene in ionic liquid electrolyte

    NASA Astrophysics Data System (ADS)

    Lin, Zifeng; Barbara, Daffos; Taberna, Pierre-Louis; Van Aken, Katherine L.; Anasori, Babak; Gogotsi, Yury; Simon, Patrice

    2016-09-01

    Ti3C2Tx MXene, a two-dimensional (2D) early transition metal carbide, has shown an extremely high volumetric capacitance in aqueous electrolytes, but in a narrow voltage window (less than 1.23 V). The utilization of MXene materials in ionic liquid electrolytes with a large voltage window has never been addressed. Here, we report the preparation of the Ti3C2Tx MXene ionogel film by vacuum filtration for use as supercapacitor electrodes operating in 1-Ethyl-3-methylimidazolium bis(trifluoromethylsulfonyl)imide (EMI-TFSI) neat ionic liquid electrolyte. Due to the disordered structure of the Ti3C2Tx hydrogel film and a stable spacing after vacuum drying, achieved through ionic liquid electrolyte immersion of the Ti3C2Tx hydrogel film, the Ti3C2Tx surface became accessible to EMI+ and TFSI- ions. A capacitance of 70 F g-1 together with a large voltage window of 3 V was obtained at a scan rate of 20 mV s-1 in neat EMI-TFSI electrolyte. The electrochemical signature indicates a capacitive behavior even at a high scan rate (500 mV s-1) and a high power performance. This work opens up the possibilities of using MXene materials with various ionic liquid electrolytes.

  17. Metagenomic chromosome conformation capture (meta3C) unveils the diversity of chromosome organization in microorganisms

    PubMed Central

    Marbouty, Martial; Cournac, Axel; Flot, Jean-François; Marie-Nelly, Hervé; Mozziconacci, Julien; Koszul, Romain

    2014-01-01

    Genomic analyses of microbial populations in their natural environment remain limited by the difficulty to assemble full genomes of individual species. Consequently, the chromosome organization of microorganisms has been investigated in a few model species, but the extent to which the features described can be generalized to other taxa remains unknown. Using controlled mixes of bacterial and yeast species, we developed meta3C, a metagenomic chromosome conformation capture approach that allows characterizing individual genomes and their average organization within a mix of organisms. Not only can meta3C be applied to species already sequenced, but a single meta3C library can be used for assembling, scaffolding and characterizing the tridimensional organization of unknown genomes. By applying meta3C to a semi-complex environmental sample, we confirmed its promising potential. Overall, this first meta3C study highlights the remarkable diversity of microorganisms chromosome organization, while providing an elegant and integrated approach to metagenomic analysis. DOI: http://dx.doi.org/10.7554/eLife.03318.001 PMID:25517076

  18. EBNA3C regulates p53 through induction of Aurora kinase B.

    PubMed

    Jha, Hem C; Yang, Karren; El-Naccache, Darine W; Sun, Zhiguo; Robertson, Erle S

    2015-03-20

    In multicellular organisms p53 maintains genomic integrity through activation of DNA repair, and apoptosis. EBNA3C can down regulate p53 transcriptional activity. Aurora kinase (AK) B phosphorylates p53, which leads to degradation of p53. Aberrant expression of AK-B is a hallmark of numerous human cancers. Therefore changes in the activities of p53 due to AK-B and EBNA3C expression is important for understanding EBV-mediated cell transformation. Here we show that the activities of p53 and its homolog p73 are dysregulated in EBV infected primary cells which can contribute to increased cell transformation. Further, we showed that the ETS-1 binding site is crucial for EBNA3C-mediated up-regulation of AK-B transcription. Further, we determined the Ser 215 residue of p53 is critical for functional regulation by AK-B and EBNA3C and that the kinase domain of AK-B which includes amino acid residues 106, 111 and 205 was important for p53 regulation. AK-B with a mutation at residue 207 was functionally similar to wild type AK-B in terms of its kinase activities and knockdown of AK-B led to enhanced p73 expression independent of p53. This study explores an additional mechanism by which p53 is regulated by AK-B and EBNA3C contributing to EBV-induced B-cell transformation.

  19. Structure, electronic, mechanical and optical properties of ternary YAl3C3 carbide

    NASA Astrophysics Data System (ADS)

    Hussain, Altaf; Javed, Athar; Mehmood, Salman; Rasool, M. Nasir; Khan, Muhammad Azhar; Iqbal, Faisal

    2016-05-01

    The electronic structure, mechanical and optical properties of ternary yttrium aluminum carbide (YAl3C3) has been studied by first principles approach. The crystal structure and elastic properties are studied by using Vienna ab initio simulation package (VASP). An orthogonalized linear combination of atomic orbitals (OLCAO) method based on the density functional theory (DFT) is implemented to elucidate the electronic structure and optical properties of ternary YAl3C3 carbide. The YAl3C3 carbide exhibits a narrow indirect band gap, Eg=0.12 eV which shows its poor metallic and/or semiconductor behavior. The effective charge (Q*) calculation reveals more charge transfer from Al-sites as compared to Y-sites which indicates dominant ionic character of Al-sites. The analysis of structure and bond order (BO) calculations show that the Al-C bonds in the basal plane are much stronger as compared to Al-C bonds along the c-axis. The Al-C bonds lying in the basal plane have main contribution into the overall stiffness of YAl3C3 carbide. The effective mass of charge carriers (electrons and holes) and inter-band optical properties (complex dielectric function and optical conductivity) are also studied which show high degree of anisotropy in YAl3C3.

  20. Contraceptive counseling for adolescents.

    PubMed

    Potter, Julia; Santelli, John S

    2015-11-01

    The majority of adolescents become sexually active during their teenage years, making contraceptive counseling an important aspect of routine adolescent healthcare. However, many healthcare providers express discomfort when it comes to counseling adolescents about contraceptive options. This Special Report highlights the evidence supporting age-appropriate contraceptive counseling for adolescents and focuses on best practices for addressing adolescents' questions and concerns about contraceptive methods.