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Sample records for idiopathic scoliosis patients

  1. 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

  2. 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

  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. Psychological Evaluations of Patients Operated for Idiopathic Scoliosis by the Harrington Method.

    ERIC Educational Resources Information Center

    Orvomaa, E.

    1998-01-01

    A study of 204 patients operated on for idiopathic scoliosis by the Harrington method between 1970 and 1975 found that patients were content with their lives, tended to form families later in life, and had fewer sexual relationships. The patients felt their illness had mostly influenced their participation in work and in physical activities.…

  5. 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 ...

  6. Association between Estrogen Receptor Alpha Gene Polymorphisms and Susceptibility to Idiopathic Scoliosis in Bulgarian Patients: A Case-Control Study

    PubMed Central

    Nikolova, Svetla; Yablanski, Vasil; Vlaev, Evgeni; Stokov, Luben; Savov, Alexey; Kremensky, Ivo

    2015-01-01

    BACKGROUND: The current consensus on idiopathic scoliosis maintains that it has a multifactorial etiology with genetic predisposing factors. AIM: Estrogen receptor alpha gene has been considered as candidate gene of idiopathic scoliosis. MATERIAL AND METHODS: We conducted a case-control study of Bulgarian population samples (eighty patients with idiopathic scoliosis and one hundred-sixty healthy unrelated gender-matched controls) trying to investigate the association between common genetic polymorphisms of estrogen receptor alpha and the susceptibility to idiopathic scoliosis. Molecular detection of the restriction polymorphisms XbaI and PvuII was performed by polymerase chain reaction following by restriction fragment length polymorphism. The statistical analysis was performed by Pearson’s chi-squared test. RESULTS: Our case-control study showed statistically significant association between the PvuII polymorphism and susceptibility to idiopathic scoliosis and curve progression. No genotype or allele of XbaI polymorphism was found to be correlated with the onset or severity of the disease. CONCLUSIONS: The identification of molecular markers with diagnostic and prognostic value could be useful for early detection of children at risk for the development of scoliosis and for prognosis of the risk for a rapid deformity progression. That would facilitate the therapy decisions and early stage treatment of the patient with the least invasive procedures. PMID:27275235

  7. 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

  8. 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

  9. 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

  10. Posterior spinal instrumented fusion for idiopathic scoliosis in patients with multisystemic neurodegenerative disorder: a report of two cases.

    PubMed

    Loh, K W; Chan, C Yw; Chiu, C K; Bin Hasan, M S; Kwan, M K

    2016-08-01

    Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke (MELAS) syndrome is a progressive multisystemic neurodegenerative disorder. MELAS syndrome impairs oxidative phosphorylation and predisposes patients to lactic acidosis, particularly under metabolic stress. We report 2 siblings with MELAS-associated idiopathic scoliosis who underwent posterior spinal instrumented fusion with measures taken to minimise anaesthetic and surgical stress, blood loss, and operating time. PMID:27574278

  11. Management of severe and rigid idiopathic scoliosis.

    PubMed

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

    2015-07-01

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

  12. 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

  13. 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

  14. 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

  15. Optimal management of idiopathic scoliosis in adolescence.

    PubMed

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

    2013-01-01

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

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

    PubMed

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

    2016-01-01

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

  17. Curve progression in Risser stage 0 or 1 patients after posterior spinal fusion for idiopathic scoliosis.

    PubMed

    Roberto, R F; Lonstein, J E; Winter, R B; Denis, F

    1997-01-01

    A retrospective review was performed to determine "crankshaft" prevalence in 86 immature patients who underwent posterior spinal fusion for idiopathic scoliosis. Tanner stage, chronologic age, bone age, and epiphyseal status were used as maturity indicators. Overall, 62 (72%) patients progressed < or = 10 degrees, 18 (21%) patients progressed 11-15 degrees, and six (7%) patients progressed > or = 16 degrees in the coronal plane. Tanner I patients with open triradiate cartilage had the highest rate of crankshaft occurrence; nine (75%) of 12 patients progressed >10 degrees (p < 0.05). Fifty-two percent of Tanner I, 26% of Tanner II, 11% of Tanner III, and no Tanner IV patients progressed >10 degrees (p < 0.05). Cobb angle increases of >10 degrees degrees occurred in 54% of patients with open triradiate cartilage (p < 0.05) and in 48% of patients with open capital femoral epiphyses (p < 0.05). Anterior and posterior spinal fusion should be considered in prepubertal (Tanner I) patients with open triradiate cartilage. PMID:9591972

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

    PubMed Central

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

    2015-01-01

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

  19. 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

  20. Vertebral Body Stapling versus Bracing for Patients with High-Risk Moderate Idiopathic Scoliosis

    PubMed Central

    Cuddihy, Laury; Danielsson, Aina J.; Cahill, Patrick J.; Samdani, Amer F.; Grewal, Harsh; Richmond, John M.; Mulcahey, M. J.; Gaughan, John P.; Antonacci, M. Darryl; Betz, Randal R.

    2015-01-01

    Purpose. We report a comparison study of vertebral body stapling (VBS) versus a matched bracing cohort for immature patients with moderate (25 to 44°) idiopathic scoliosis (IS). Methods. 42 of 49 consecutive patients (86%) with IS were treated with VBS and followed for a minimum of 2 years. They were compared to 121 braced patients meeting identical inclusion criteria. 52 patients (66 curves) were matched according to age at start of treatment (10.6 years versus 11.1 years, resp. [P = 0.07]) and gender. Results. For thoracic curves 25–34°, VBS had a success rate (defined as curve progression <10°) of 81% versus 61% for bracing (P = 0.16). In thoracic curves 35–44°, VBS and bracing both had a poor success rate. For lumbar curves, success rates were similar in both groups for curves measuring 25–34°. Conclusion. In this comparison of two cohorts of patients with high-risk (Risser 0-1) moderate IS (25–44°), in smaller thoracic curves (25–34°) VBS provided better results as a clinical trend as compared to bracing. VBS was found not to be effective for thoracic curves ≥35°. For lumbar curves measuring 25–34°, results appear to be similar for both VBS and bracing, at 80% success. PMID:26618169

  1. 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

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

    PubMed

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

    2014-10-01

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

  3. 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

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

    PubMed

    Seifert, J; Thielemann, F; Bernstein, P

    2016-06-01

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

  5. Infantile Idiopathic Scoliosis

    MedlinePlus

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  6. Adolescent Idiopathic Scoliosis

    MedlinePlus

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  7. 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

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

    PubMed Central

    2013-01-01

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

  9. Pulmonary function in children with idiopathic scoliosis

    PubMed Central

    2012-01-01

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

  10. Functional variants of POC5 identified in patients with idiopathic scoliosis

    PubMed Central

    Patten, Shunmoogum A.; Margaritte-Jeannin, Patricia; Bernard, Jean-Claude; Alix, Eudeline; Labalme, Audrey; Besson, Alicia; Girard, Simon L.; Fendri, Khaled; Fraisse, Nicolas; Biot, Bernard; Poizat, Coline; Campan-Fournier, Amandine; Abelin-Genevois, Kariman; Cunin, Vincent; Zaouter, Charlotte; Liao, Meijiang; Lamy, Raphaelle; Lesca, Gaetan; Menassa, Rita; Marcaillou, Charles; Letexier, Melanie; Sanlaville, Damien; Berard, Jerome; Rouleau, Guy A.; Clerget-Darpoux, Françoise; Drapeau, Pierre; Moldovan, Florina; Edery, Patrick

    2015-01-01

    Idiopathic scoliosis (IS) is a spine deformity that affects approximately 3% of the population. The underlying causes of IS are not well understood, although there is clear evidence that there is a genetic component to the disease. Genetic mapping studies suggest high genetic heterogeneity, but no IS disease-causing gene has yet been identified. Here, genetic linkage analyses combined with exome sequencing identified a rare missense variant (p.A446T) in the centriolar protein gene POC5 that cosegregated with the disease in a large family with multiple members affected with IS. Subsequently, the p.A446T variant was found in an additional set of families with IS and in an additional 3 cases of IS. Moreover, POC5 variant p.A455P was present and linked to IS in one family and another rare POC5 variant (p.A429V) was identified in an additional 5 cases of IS. In a zebrafish model, expression of any of the 3 human IS-associated POC5 variant mRNAs resulted in spine deformity, without affecting other skeletal structures. Together, these findings indicate that mutations in the POC5 gene contribute to the occurrence of IS. PMID:25642776

  11. 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

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

    PubMed Central

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

    2006-01-01

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

  13. 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

  14. Comparison of Functional Outcome and Quality of Life in Patients With Idiopathic Scoliosis Treated by Spinal Fusion.

    PubMed

    Fan, Hengwei; Wang, Qifei; Huang, Zifang; Sui, Wenyuan; Yang, Jingfan; Deng, Yaolong; Yang, Junlin

    2016-05-01

    Longer spinal fusions have been shown to result in improved deformity correction; however, loss of normal flexibility in the fusion area should not be ignored. Current consensus was to achieve a shorter fusion in primary surgery, with the goal of preserving as much of the distal motion segment as possible. However, the correlation between the length of fusion and functional outcome remains controversial. To the best of our knowledge, a previous study has demonstrated the function outcomes and the differences in HRQoL with specific fusion levels.In this cross-sectional study, 172 patients (mean age, 17.8 y) with idiopathic scoliosis treated by spinal fusion (mean time since surgery, 29.7 mo) were included to measure lumbar spine mobility and quality of life using validated outcome instruments in the study population. Patients were assigned to 5 groups according to the lower instrumented vertebra (LIV) level: group A (fusion above L2) 26 patients; group B (fusion to L2) 21 patients; group C (fusion to L3) 46 patients; group D (fusion to L4) 53 patients; and group E (fusion to L5) 26 patients. At each follow-up, patients were asked to complete the Scoliosis Research Society 22 (SRS-22) Questionnaire. Lumbar mobility was assessed using a dual digital inclinometer.Average spinal range of motion (ROM) was 41.4 degrees (SD, 20.7), forward flexion was 29.2 degrees (SD, 15.0), and backward extension was 12.2 degrees (SD, 9.5). The total spinal range of motion and forward flexion dropped noticeably as the LIV got more distal. Statistically significant between-group differences (1-way ANOVA) were found for ROM (P < 0.001), forward flexion (P < 0.001), or backward extension (P < 0.001). The motion segments preserved significantly correlated with ROM (r = 0.76, P < 0.001), ROMF (r = 0.76, P < 0.001), and ROME (r = 0.39, P < 0.001). However, no significant between-group differences was found for each domain of SRS-22 questionnaire.The motion

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

    PubMed

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

    2015-08-01

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

  16. Comparison of Functional Outcome and Quality of Life in Patients With Idiopathic Scoliosis Treated by Spinal Fusion

    PubMed Central

    Fan, Hengwei; Wang, Qifei; Huang, Zifang; Sui, Wenyuan; Yang, Jingfan; Deng, Yaolong; Yang, Junlin

    2016-01-01

    Abstract Longer spinal fusions have been shown to result in improved deformity correction; however, loss of normal flexibility in the fusion area should not be ignored. Current consensus was to achieve a shorter fusion in primary surgery, with the goal of preserving as much of the distal motion segment as possible. However, the correlation between the length of fusion and functional outcome remains controversial. To the best of our knowledge, a previous study has demonstrated the function outcomes and the differences in HRQoL with specific fusion levels. In this cross-sectional study, 172 patients (mean age, 17.8 y) with idiopathic scoliosis treated by spinal fusion (mean time since surgery, 29.7 mo) were included to measure lumbar spine mobility and quality of life using validated outcome instruments in the study population. Patients were assigned to 5 groups according to the lower instrumented vertebra (LIV) level: group A (fusion above L2) 26 patients; group B (fusion to L2) 21 patients; group C (fusion to L3) 46 patients; group D (fusion to L4) 53 patients; and group E (fusion to L5) 26 patients. At each follow-up, patients were asked to complete the Scoliosis Research Society 22 (SRS-22) Questionnaire. Lumbar mobility was assessed using a dual digital inclinometer. Average spinal range of motion (ROM) was 41.4 degrees (SD, 20.7), forward flexion was 29.2 degrees (SD, 15.0), and backward extension was 12.2 degrees (SD, 9.5). The total spinal range of motion and forward flexion dropped noticeably as the LIV got more distal. Statistically significant between-group differences (1-way ANOVA) were found for ROM (P < 0.001), forward flexion (P < 0.001), or backward extension (P < 0.001). The motion segments preserved significantly correlated with ROM (r = 0.76, P < 0.001), ROMF (r = 0.76, P < 0.001), and ROME (r = 0.39, P < 0.001). However, no significant between-group differences was found for each domain of SRS-22 questionnaire

  17. Effect of bracing on respiratory mechanics in mild idiopathic scoliosis.

    PubMed Central

    Kennedy, J D; Robertson, C F; Hudson, I; Phelan, P D

    1989-01-01

    The use of a corrective orthopaedic brace is an established form of management for patients with progressive idiopathic scoliosis. Thirteen patients with mild idiopathic scoliosis were studied with and without the corrective brace applied. Lung volumes and the pattern of chest wall and abdominal movement were measured during quiet breathing. Transdiaphragmatic pressures were measured in six of the patients and upper ribcage movement in seven patients. Application of the brace resulted in a significant reduction in vital capacity (14%), functional residual capacity (22%), and total lung capacity (12%). There was no effect on respiratory rate or minute volume. In the erect position the pattern of chest wall movement was altered with a reduction in lower ribcage movement of 46% and abdominal wall of 39% and an increase in upper ribcage movement of 43%. These changes were greater in the supine position. There was at least a twofold increase in end inspiratory and end expiratory gastric pressures during tidal breathing, but oesophageal pressures were not affected by the brace. Transdiaphragmatic pressures showed a similar twofold increase, which implies a substantial increase in the work of breathing. In view of the doubts concerning the influence of bracing on the natural history of idiopathic scoliosis and the substantial functional effect of bracing on the respiratory system, it is suggested that the current practice of bracing in this condition needs to be reviewed. PMID:2772855

  18. 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

  19. 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

  20. Infection after anterior spinal fusion for idiopathic scoliosis using the Cotrel-Dubousset-Hopf system: A clinical case series of three patients

    PubMed Central

    Willems, Paul C.; Punt, Ilona M.; van Rhijn, Lodewijk W.; van Ooij, André

    2016-01-01

    Background Three patients with late-onset infection after multilevel instrumented anterior spinal fusion for idiopathic scoliosis, using the Cotrel-Dubousset-Hopf (CDH) system, are presented. The CDH-system is an anterior instrumentation with high biomechanical stability and rigidity, ensuring a stable primary fixation. Unlike after posterior spinal fusion, infection after anterior spinal fusion (ASF) for idiopathic scoliosis has rarely been reported. Methods The files of three patients who developed an infection after ASF for scoliosis using the CDH-system, were reviewed. The clinical presentation and diagnostic and therapeutic options are discussed. Results All three patients had a late-onset infection of the CDH-system, which was difficult to diagnose because of nonspecific symptoms. Radiographs and technetium bone scan appeared to be of low value. When an abscess was present, this could accurately be diagnosed with MRI or CT imaging. Operative treatment with implant removal and antibiotic therapy was successful in all cases. Conclusion Late onset infections after ASF using the CDH-system presented with few and nonspecific symptoms. The clinical presentation was mainly characterized by vague abdominal- or back-pain after an interval of normal postoperative recovery, moderately raised infection parameters and inconclusive findings with imaging modalities. As treatment, implant removal, debridement and parenteral antibiotics are recommended. It should be noted though that implant removal poses serious risks for vascular and visceral structures. PMID:26913222

  1. 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

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

    PubMed

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

    2016-06-01

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

  3. [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

  4. Effects of the Schroth exercise on the Cobb's angle and vital capacity of patients with idiopathic scoliosis that is an operative indication.

    PubMed

    Kim, Kyoung-Don; Hwangbo, Pil-Neo

    2016-03-01

    [Purpose] The purpose of this study was to investigate the effects of the Schroth exercise on the Cobb's angle and vital capacity of patients with growing idiopathic scoliosis, an operative indication. [Subjects] Five idiopathic scoliosis patients with a Cobb's angle of the thoracic vertebra of 40 degrees or higher and Risser sign stage 3 or higher. [Methods] The Schroth exercise was applied 3 times a week for 12 weeks. We measured the thoracic trunk inclination, Cobb's angle, and vital capacity before and after the exercise program. [Results] The thoracic trunk rotation angle decreased from 11.86 ± 3.32° to 4.90 ± 1.91° on average, the thoracic Cobb's angle decreased from 42.40 ± 7.86° to 26.0 ± 3.65° on average, and the vital capacity also increased from 2.83 ± 1.23° to 4.04° ± 1.67° on average. All these effects were significant. [Conclusion] The 12-week Schroth exercise caused significant effects in the thoracic trunk inclination, Cobb's angle, and vital capacity. The conservative treatment method was found to be effective even at a 40 degree or higher Cobb's angle. In the future, universal exercise approach methods and preventive training for the treatment of scoliosis should be developed further. PMID:27134385

  5. 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

  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. Electromyography of the paravertebral muscles in idiopathic scoliosis. Measurements of amplitude and spectral changes under load.

    PubMed

    Zetterberg, C; Björk, R; Ortengren, R; Andersson, G B

    1984-06-01

    The myoelectric activity of the paraspinal muscles was recorded in girls with idiopathic scoliosis and in healthy controls. The muscles of the back were loaded isometrically and the signals recorded at the T8 and L3 levels were analysed as regards amplitude and frequency. A comparatively higher signal amplitude was found on the convex side of the scoliosis curve. This was found to be due to a lower amplitude on the concave side when the scoliosis group was compared to the controls. The amplitude difference was correlated to the degree of scoliosis. A shift in the myoelectric spectrum toward lower frequencies occurred during the loading period. There were no differences in this respect between the sides of the scoliosis, nor were there differences between the scoliosis patients and the controls. Secondary adaptation to the higher load demand by the muscles on the convex side in scoliosis is the most probable explanation for our observations. PMID:6741480

  8. 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

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

    PubMed Central

    ZHENG, XU; MA, ZHENGLIANG; GU, XIAOPING

    2015-01-01

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

  10. 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

  11. 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

  12. The relation between idiopathic scoliosis and the frontal and lateral facial form

    PubMed Central

    Kim, Tae-Hwan; Kim, Joo-Hwan; Kim, Yae-Jin; Cho, Il-Sik; Lim, Yong-Kyu

    2014-01-01

    Objective The purpose of this study was to evaluate the relation between idiopathic scoliosis and facial deformity in the horizontal, vertical, and anteroposterior planes. Methods A total of 123 female patients aged 14 years or older, who visited the Spine Clinic at the Department of Orthopedics, Korea University Guro Hospital for treatment of idiopathic scoliosis, were enrolled. Whole-spine anteroposterior and lateral radiographs were taken with the patient in a naturally erect position, and frontal and lateral cephalograms were taken in an erect position with the Frankfort horizontal line parallel to the floor. Scoliosis was classified according to the Cobb angle and Lenke classification of six curve types. Cephalometric tracing in all cases was carried out with V-Ceph 5.5 by the same orthodontist. The Kruskal-Wallis test was performed to determine whether any relation existed between each group of the idiopathic scoliosis classification and the cephalometric measurements of frontal and lateral cephalograms. Results The measurements did not reveal any significant association between the Cobb angle and cephalometric measurements and between the curve type based on the Lenke classification and cephalometric measurements. Conclusions Based on the results of this study, no apparent relation was observed between the severity of scoliosis and facial form variations in idiopathic scoliosis patients. PMID:25309865

  13. 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

  14. 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

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

    PubMed

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

    2015-01-01

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

  16. Idiopathic scoliosis: the tethered spine II: post-surgical pain.

    PubMed

    Whyte Ferguson, Lucy

    2014-10-01

    The treatment of severe chronic pain in young people following surgery for the correction of curvatures of idiopathic scoliosis (IS) is presented through two case histories. Effective treatment involved release of myofascial trigger points (TrPs) known to refer pain into the spine, and treatment of related fascia and joint dysfunction. The TrPs found to be contributing to spinal area pain were located in muscles at some distance from the spine rather than in the paraspinal muscles. Referred pain from these TrPs apparently accounted for pain throughout the base of the neck and thoracolumbar spine. Exploratory surgery was considered for one patient to address pain following rod placement but the second surgery became unnecessary when the pain was controlled with treatment of the myofascial pain and joint dysfunction. The other individual had both scoliosis and hyperkyphosis, had undergone primary scoliosis surgery, and subsequently underwent a second surgery to remove hardware in an attempt to address her persistent pain following the initial surgery (and because of dislodged screws). The second surgery did not, however, reduce her pain. In both cases these individuals, with severe chronic pain following scoliosis corrective surgery, experienced a marked decrease of pain after myofascial treatment. As will be discussed below, despite the fact that a significant minority of individuals who have scoliosis corrective surgery are thought to require a second surgery, and despite the fact that pain is the most common reason leading to such revision surgery, myofascial pain syndrome (MPS) had apparently not previously been considered as a possible factor in their pain. PMID:25440198

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

    PubMed

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

    2009-08-01

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

  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. 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

  20. 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

  1. Posterior Spine Fusion with Moss-Miami Instrumentation for Adolescent Idiopathic Scoliosis: Radiographic, Clinical and Patient-Centered Outcomes

    PubMed Central

    Maio, Fernando De; Dolan, Lori A; Luna, Vincenzo De; Weinstein, Stuart L

    2007-01-01

    This paper evaluates the efficacy the Moss-Miami System instrumentation for surgical treatment of spinal deformity. Eight-five patients with AIS underwent a posterior spinal fusion with using this system between 1994 and 1998. Radiographs of the spine were taken preoperatively, at discharge, one year after surgery, and at the latest follow-up (average of 2.5 year, range 2 to 6 years). All radiographs were assessed for curve magnitude, coronal balance, kyphosis, lordosis, junctional kyphosis, and sagittal balance. The scoliosis research society instrument was administered at the final follow-up. the average curve correction ranged from 53 to 65 percent. All patients showed solid fusion by final follow-up. three patients required a second operation for complications related to their scoliosis; two patients showed a rod fracture without evidence of pseudoarthrosis or curve progression. Two transient neurological complications related to the surgery were observed. SRS results were favorable with regard to function and cosmetic appearance. PMID:17907427

  2. 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

  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. Alternative methods of conservative treatment of idiopathic scoliosis.

    PubMed

    Zarzycka, Maja; Rozek, Karina; Zarzycki, Michał

    2009-01-01

    Scoliosis is a deformity of the spine known since Hippocrates times. The value of certain methods of conservative treatment remains controversial. Some of them have only a psychological value both for the physician and his or her caregivers. Based on current literature and the Scoliosis Research Society Report of Alternative Methods of Treatment of Idiopathic Scoliosis, we describe the effectiveness of various alternative methods, such as exercise, Dobosiewicz technique, Karski method, SEAS 02, acupuncture, Alexander technique, aromatherapy, ayurveda, ASCO treatment, biofeedback, chiropractic, Yoga, Feldenkrais method, Pilates method, massage therapy, rolfing, magnet therapy, surface electrical stimulation, PNF, Copes system, and bracing. PMID:19920282

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

    PubMed Central

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

    2015-01-01

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

  6. Low bone mineral status in adolescent idiopathic scoliosis

    PubMed Central

    Li, Xin-Feng; Li, Hai

    2008-01-01

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

  7. Cerebral glucose metabolic abnormality in patients with congenital scoliosis.

    PubMed

    Park, Weon Wook; Suh, Kuen Tak; Kim, Jeung Il; Ku, Ja Gyung; Lee, Hong Seok; Kim, Seong-Jang; Kim, In-Ju; Kim, Yong-Ki; Lee, Jung Sub

    2008-07-01

    A possible association between congenital scoliosis and low mental status has been recognized, but there are no reports describing the mental status or cerebral metabolism in patients with congenital scoliosis in detail. We investigated the mental status using a mini-mental status exam as well as the cerebral glucose metabolism using F-18 fluorodeoxyglucose brain positron emission tomography in 12 patients with congenital scoliosis and compared them with those of 14 age-matched patients with adolescent idiopathic scoliosis. The mean mini-mental status exam score in the congenital scoliosis group was significantly lower than that in the adolescent idiopathic scoliosis group. Group analysis found that various brain areas of patients with congenital scoliosis showed glucose hypometabolisms in the left prefrontal cortex (Brodmann area 10), right orbitofrontal cortex (Brodmann area 11), left dorsolateral prefrontal cortex (Brodmann area 9), left anterior cingulate gyrus (Brodmann area 24) and pulvinar of the left thalamus. From this study, we could find the metabolic abnormalities of brain in patients with congenital scoliosis and suggest the possible role of voxel-based analysis of brain fluorodeoxyglucose positron emission tomography. PMID:18446384

  8. 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

  9. 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

  10. 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

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

    PubMed

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

    2015-02-01

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

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

    PubMed Central

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

    2014-01-01

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

  13. Predictive factors for perioperative blood transfusion in surgeries for correction of idiopathic, neuromuscular or congenital scoliosis

    PubMed Central

    Cristante, Alexandre Fogaça; Borges, Paulo Alvim; Barbosa, Angelo Roberto; Letaif, Olavo Biraghi; Marcon, Raphael Martus; de Barros-Filho, Tarcisio Eloy Pessoa

    2014-01-01

    OBJECTIVE: To evaluate the association of clinical and demographic variables in patients requiring blood transfusion during elective surgery to treat scoliosis with the aim of identifying markers predictive of the need for blood transfusion. METHODS: Based on the review of medical charts at a public university hospital, this retrospective study evaluated whether the following variables were associated with the need for red blood cell transfusion (measured by the number of packs used) during scoliosis surgery: scoliotic angle, extent of arthrodesis (number of fused levels), sex of the patient, surgery duration and type of scoliosis (neuromuscular, congenital or idiopathic). RESULTS: Of the 94 patients evaluated in a 55-month period, none required a massive blood transfusion (most patients needed less than two red blood cell packs). The number of packs was not significantly associated with sex or type of scoliosis. The extent of arthrodesis (r = 0.103), surgery duration (r = 0.144) and scoliotic angle (r = 0.004) were weakly correlated with the need for blood transfusion. Linear regression analysis showed an association between the number of spine levels submitted to arthrodesis and the volume of blood used in transfusions (p = 0.001). CONCLUSION: This study did not reveal any evidence of a significant association between the need for red blood cell transfusion and scoliotic angle, sex or surgery duration in scoliosis correction surgery. Submission of more spinal levels to arthrodesis was associated with the use of a greater number of blood packs. PMID:25518018

  14. 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

  15. Scoliosis Research Society (SRS) Criteria and Society of Scoliosis Orthopaedic and Rehabilitation Treatment (SOSORT) 2008 Guidelines in Non-Operative Treatment of Idiopathic Scoliosis.

    PubMed

    Korbel, Krzysztof; Kozinoga, Mateusz; Stoliński, Łukasz; Kotwicki, Tomasz

    2014-01-01

    According to the Scoliosis Research Society (SRS), idiopathic scoliosis (IS) is a curvature of more than 10° Cobb angle, affecting 2-3% of pediatric population. Idiopathic scoliosis accounts for 80% of all scoliosis cases. Non-operative principles in the therapy of idiopathic scoliosis, including Scoliosis Research Society (SRS) criteria and guidelines proposed by the experts of the Society on Scoliosis Orthopedic and Rehabilitation Treatment (SOSORTS) were presented. The possibility to carry out quality of life assessments in a conservative procedure was also demonstrated. Based on the natural history of idiopathic scoliosis, SRS criteria, SOSORT 2008 experts' opinion and the knowledge of the possibilities of psychological assessment of conservative IS treatment, rules were proposed regarding nonsurgical IS therapy procedures, with special consideration being paid to the proper treatment start time (age, Risser test, biological maturity, Cobb angle), possibility of curvature progression, the importance of physiotherapy and psychological assessment. The knowledge of SRS criteria and SOSORT guidelines regarding the conservative treatment of IS are essential for proper treatment (the right time to start treatment), and supports establishment of interdisciplinary treatment teams, consisting of a physician, a physiotherapist, an orthopedic technician and a psychologist. PMID:25066033

  16. Knowledge about idiopathic scoliosis among students of physiotherapy.

    PubMed

    Ciazynski, D; Czernicki, K; Durmala, J

    2008-01-01

    The aim of the study was to determine the level of basic knowledge about idiopathic scoliosis (IS) among students of physiotherapy. The study included 37 students of Medical University of Silesia (17F and 20M aged 22-25, mean 22.6), attending the 3(rd) year of a 1(st) degree of physiotherapy. All students had credits in kinesiotherapy, including methods of conservative treatment of IS. Students were examined using a questionnaire, comprising general knowledge of IS, questions related to sagittal plane correction, influence of various physical activities on IS and known methods of conservative treatment. 81 students considered IS as 3-D deformity. 62.2% of those questioned would diagnose IS when the Cobb angle reaches 10 degrees . All students agreed that the aetiology of IS remains unknown. 54.1% considered forcible extensory exercises of back as favourable in IS. Questioned students mostly preferred swimming (94.6%), yoga (73.0%) and martial arts (32.4%) as beneficial to IS. The methods of conservative treatment which were known best were: Lehnert-Schroth-Weiss (94.6%), Klapp (91.9%), Majoch (89.2%) and Dobosiewicz (78.4%). The conclusions indicate that the average level of knowledge of idiopathic scoliosis among students of physiotherapy is unsatisfactory, despite the education programme including the SOSORT guidelines. Education in the field of scoliosis should be comprehensive and meet contemporary guidelines and standards. PMID:18810037

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

    PubMed

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

    2016-05-01

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

  18. Recommendations for research studies on treatment of idiopathic scoliosis: Consensus 2014 between SOSORT and SRS non-operative management committee.

    PubMed

    Negrini, Stefano; Hresko, Timothy M; O'Brien, Joseph P; Price, Nigel

    2015-01-01

    The two main societies clinically dealing with idiopathic scoliosis are the Scoliosis Research Society (SRS), founded in 1966, and the international Society on Scoliosis Orthopedic and Rehabilitation Treatment (SOSORT), started in 2004. Inside the SRS, the Non-Operative Management Committee (SRS-NOC) has the same clinical interest of SOSORT, that is the Orthopaedic and Rehabilitation (or Non-Operative, or conservative) Management of idiopathic scoliosis patients. The aim of this paper is to present the results of a Consensus among the best experts of non-operative treatment of Idiopathic Scoliosis, as represented by SOSORT and SRS, on the recommendation for research studies on treatment of Idiopathic Scoliosis. The goal of the consensus statement is to establish a framework for research with clearly delineated inclusion criteria, methodologies, and outcome measures so that future meta- analysis or comparative studies could occur. A Delphi method was used to generate a consensus to develop a set of recommendations for clinical studies on treatment of Idiopathic Scoliosis. It included the development of a reference scheme, which was judged during two Delphi Rounds; after this first phase, it was decided to develop the recommendations and 4 other Delphi Rounds followed. The process finished with a Consensus Meeting, that was held during the SOSORT Meeting in Wiesbaden, 8-10 May 2014, moderated by the Presidents of SOSORT (JP O'Brien) and SRS (SD Glassman) and by the Chairs of the involved Committees (SOSORT Consensus Committee: S Negrini; SRS Non-Operative Committee: MT Hresko). The Boards of the SRS and SOSORT formally accepted the final recommendations. The 18 Recommendations focused: Research needs (3), Clinically significant outcomes (4), Radiographic outcomes (3), Other key outcomes (Quality of Life, adherence to treatment) (2), Standardization of methods of non-operative research (6). PMID:25780381

  19. Recovery of dynamic balance following external posture disturbance in children with idiopathic scoliosis.

    PubMed

    Ostrowska, Bozena; Rozek-Piechura, Krystyna; Skolimowski, Tadeusz

    2006-06-30

    Background. The aim of this study was to attempt the implementation of the method of mathematical modelling for motor properties estimation in children with idiopathic scoliosis on the process of keeping the balance in a standing position in the presence of mild disturbances. Material and methods. 42 children with idiopathic scoliosis (13-18 yr) and 40 healthy children were included into the study. Stabilography method was used to estimate body balance and the COP time series were recorded for subsequent analysis. Motor parameters of the evaluated individuals were determined based on the coefficient values of equation assessed through mathematics model. Results. The analysis of obtained calculated results showed that there are significant differences in meandered reaction induced by balance disturbation in a standing posture in evaluated patients. In scoliosis group there was a bigger loss of balance than in healthy children.The speed of loosing of balance after its deviation was slower and was dependent on the magnitude of the curvature angle. The bigger the angle values of the curvature the slower is the process of balance loss and at a lower speed and acceleration. And the time required for regaining the posture is prolonged. Conclusions. Standing posture of children with lateral curvature of the spine is more susceptible to balance disturbances and is characterised by worse stability. The reaction of nervous system to balance disturbances in scoliosis is visibly delayed and is characterised by lower impulsiveness. PMID:17592411

  20. Idiopathic scoliosis: managing pain before and after spinal surgery.

    PubMed

    Bray, Lucy; Craske, Jennie

    2015-12-01

    Idiopathic scoliosis surgery is performed to correct a curvature of the spine. This is a painful surgical procedure which is carried out on otherwise healthy young people. This article reports on a small evaluation project which focused on young people's opinions and experiences of their pain information needs, pain management and pain assessment. Nine young people completed written activity sheets before and after scoliosis surgery. Most of the information provided on the activity sheets involved positive comments about the surgical experience although some indicated that the management of pain continued to be a challenge after spinal surgery. The project has highlighted that there is more work to be done to ensure that young people are prepared for surgery and they are helped to convey their experiences of pain to health professionals during their treatment and recovery. PMID:26654026

  1. A History of Bracing for Idiopathic Scoliosis in North America

    PubMed Central

    Cho, Robert H.; Herman, Martin J.

    2009-01-01

    The care of the patient with scoliosis has a history extending back over two millennia with cast and brace treatment being a relatively recent endeavor, the modern era comprising just over half a century. Much of the previous literature provides a modest overview with emphasis on the history of the operative management. To better understand the current concepts of brace treatment of scoliosis, an appreciation of the history of bracing would be helpful. As such, we review the history of the treatment of scoliosis with an emphasis on modern brace treatment, primarily from a North American perspective. Our review utilizes consideration of historical texts as well as current treatises on the history of scoliosis and includes discussion of brace development with their proponents’ rationale for why they work along with an appraisal of their clinical outcomes. We provide an overview of the current standards of care and the braces typically employed toward that standard including: the Milwaukee brace, the Wilmington brace, the Boston brace, the Charleston brace, the Providence brace and the SpineCor brace. Finally, we discuss future trends including improvements in methods of determining the critical period of peak growth velocity in children with scoliosis, the exciting promise of gene markers for progressive scoliosis and “internal bracing” options. PMID:19462214

  2. [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

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

    PubMed Central

    Shneerson, J M

    1980-01-01

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

  4. 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

  5. [Criteria for treatment of idiopathic scoliosis between 40 degrees and 50 degrees. Surgical vs. conservative therapy].

    PubMed

    Hopf, C

    2000-06-01

    The treatment of idiopathic scoliosis over 40 degrees (Cobb) during the growth period is under discussion concerning the indication for conservative or surgical treatment. Curve progression depends on the degree of the frontal and sagittal deformity, vertebral rotation, rigidity of the curve, the skeletal age, the age and sex of the patient, the familial frequency of scoliosis and the location of the curve. In scoliosis over 40 degrees progression is fast and the possibilities for successful conservative brace treatment are reduced during the growth period. Progression occurs more frequently in thoracic and double major scolioses, especially in young patients (Risser sign 0 and 1). Predictive factors of a successful brace treatment are the correction of scoliosis and rotation; deterioration of both during the brace treatment leads to poor results. Evaluating the flexibility of the sagittal profile is important, as is primary correction of 30-50% in the brace during the 3 months. Operative correction of small primary curves reduces the fusion length, operation time, and blood loss and is followed by a reduction in loading on the adjacent vertebral segments in comparison to the long fusions necessary in more structural and double major scolioses. So far it is not possible to make an equivalent judgment of the frequency of the "crankshaft" phenomenon and the treatment necessary in young patients (Rissersign 0 and 1) treated by dorsal instrumentation alone, but temporary brace treatment may be considered in those cases. PMID:10929330

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

    PubMed Central

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

    2014-01-01

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

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

    PubMed

    Dimeglio, Alain; Canavese, Federico

    2013-02-01

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

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

    PubMed Central

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

    2008-01-01

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

  9. [Successful treatment of idiopathic scoliosis in school children with Vojta's neuro-physiotherapy].

    PubMed

    Avalle, C; Duelund, M; Eder, K

    1981-09-17

    The article describes the treatment of two children with idiopathic scoliosis in the age of 10 and 14 years. After an unsuccessful orthopedic treatment, these children have been treated with the neurophysio-therapy according to Vojta. PMID:7274944

  10. Minimally Invasive Scoliosis Surgery: A Novel Technique in Patients with Neuromuscular Scoliosis

    PubMed Central

    Sarwahi, Vishal; Amaral, Terry; Wendolowski, Stephen; Gecelter, Rachel; Gambassi, Melanie; Plakas, Christos; Liao, Benita; Kalantre, Sarika; Katyal, Chhavi

    2015-01-01

    Minimally invasive surgery (MIS) has been described in the treatment of adolescent idiopathic scoliosis (AIS) and adult scoliosis. The advantages of this approach include less blood loss, shorter hospital stay, earlier mobilization, less tissue disruption, and relatively less pain. However, despite these significant benefits, MIS approach has not been reported in neuromuscular scoliosis patients. This is possibly due to concerns with longer surgery time, which is further increased due to more levels fused and instrumented, challenges of pelvic fixation, size and number of incisions, and prolonged anesthesia. We modified the MIS approach utilized in our AIS patients to be implemented in our neuromuscular patients. Our technique allows easy passage of contoured rods, placement of pedicle screws without image guidance, partial/complete facet resection, and all standard reduction maneuvers. Operative time needed to complete this surgery is comparable to the standard procedure and the majority of our patients have been extubated at the end of procedure, spending 1 day in the PICU and 5-6 days in the hospital. We feel that MIS is not only a feasible but also a superior option in patients with neuromuscular scoliosis. Long-term results are unavailable; however, short-term results have shown multiple benefits of this approach and fewer limitations. PMID:26649305

  11. 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

  12. 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

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

    PubMed Central

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

    2011-01-01

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

  14. Reversal of childhood idiopathic scoliosis in an adult, without surgery: a case report and literature review

    PubMed Central

    2009-01-01

    Background Some patients with mild or moderate thoracic scoliosis (Cobb angle <50-60 degrees) suffer disproportionate impairment of pulmonary function associated with deformities in the sagittal plane and reduced flexibility of the spine and chest cage. Long-term improvement in the clinical signs and symptoms of childhood onset scoliosis in an adult, without surgical intervention, has not been documented previously. Case presentation A diagnosis of thoracic scoliosis (Cobb angle 45 degrees) with pectus excavatum and thoracic hypokyphosis in a female patient (DOB 9/17/52) was made in June 1964. Immediate spinal fusion was strongly recommended, but the patient elected a daily home exercise program taught during a 6-week period of training by a physical therapist. This regime was carried out through 1992, with daily aerobic exercise added in 1974. The Cobb angle of the primary thoracic curvature remained unchanged. Ongoing clinical symptoms included dyspnea at rest and recurrent respiratory infections. A period of multimodal treatment with clinical monitoring and treatment by an osteopathic physician was initiated when the patient was 40 years old. This included deep tissue massage (1992-1996); outpatient psychological therapy (1992-1993); a daily home exercise program focused on mobilization of the chest wall (1992-2005); and manipulative medicine (1994-1995, 1999-2000). Progressive improvement in chest wall excursion, increased thoracic kyphosis, and resolution of long-standing respiratory symptoms occurred concomitant with a >10 degree decrease in Cobb angle magnitude of the primary thoracic curvature. Conclusion This report documents improved chest wall function and resolution of respiratory symptoms in response to nonsurgical approaches in an adult female, diagnosed at age eleven years with idiopathic scoliosis. PMID:20003501

  15. 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

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

    PubMed

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

    2015-01-01

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

  17. Fully automatic measurements of axial vertebral rotation for assessment of spinal deformity in idiopathic scoliosis

    NASA Astrophysics Data System (ADS)

    Forsberg, Daniel; Lundström, Claes; Andersson, Mats; Vavruch, Ludvig; Tropp, Hans; Knutsson, Hans

    2013-03-01

    Reliable measurements of spinal deformities in idiopathic scoliosis are vital, since they are used for assessing the degree of scoliosis, deciding upon treatment and monitoring the progression of the disease. However, commonly used two dimensional methods (e.g. the Cobb angle) do not fully capture the three dimensional deformity at hand in scoliosis, of which axial vertebral rotation (AVR) is considered to be of great importance. There are manual methods for measuring the AVR, but they are often time-consuming and related with a high intra- and inter-observer variability. In this paper, we present a fully automatic method for estimating the AVR in images from computed tomography. The proposed method is evaluated on four scoliotic patients with 17 vertebrae each and compared with manual measurements performed by three observers using the standard method by Aaro-Dahlborn. The comparison shows that the difference in measured AVR between automatic and manual measurements are on the same level as the inter-observer difference. This is further supported by a high intraclass correlation coefficient (0.971-0.979), obtained when comparing the automatic measurements with the manual measurements of each observer. Hence, the provided results and the computational performance, only requiring approximately 10 to 15 s for processing an entire volume, demonstrate the potential clinical value of the proposed method.

  18. [A myelographic study of idiopathic scoliosis--with special reference to its clinical significance].

    PubMed

    Isobe, K

    1988-04-01

    The purpose of this study was to investigate the concealed anomaly of the spinal cord in the so-called idiopathic scoliosis. Thirty-three patients with idiopathic scoliotic curvatures underwent metrizamide myelography before surgery from 1979 through 1985. Spinal cord anomalies were found in 8 of the 33 patients (abnormal group). They were 3 patients with Arnold-Chiari malformation with syringomyelia, 3 with syringomyelia and 2 with "narrowed dural tube". The remaining 25 patients (normal group) gave us the standard values for myelographic measurements with which we compared quantitatively the size of dural tube and spinal cord in the normal and abnormal groups. The abnormal group was classified into three types: type I was swollen spinal cord with normal dural tube; type II was swollen spinal cord with narrowed dural tube; and type III was normal spinal cord with narrowed dural tube. In conclusion myelography and myelographic measurements are essential for idiopathic scoliotic patients who will undergo spinal correction and fusion operations. PMID:3404008

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

    PubMed Central

    2013-01-01

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

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

    PubMed

    Burwell, R G; Dangerfield, P H

    2002-01-01

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

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

    PubMed

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

    2007-06-01

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

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

    PubMed

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

    2016-06-01

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

  3. 2011 SOSORT guidelines: Orthopaedic and Rehabilitation treatment of idiopathic scoliosis during growth

    PubMed Central

    2012-01-01

    Background The International Scientific Society on Scoliosis Orthopaedic and Rehabilitation Treatment (SOSORT), that produced its first Guidelines in 2005, felt the need to revise them and increase their scientific quality. The aim is to offer to all professionals and their patients an evidence-based updated review of the actual evidence on conservative treatment of idiopathic scoliosis (CTIS). Methods All types of professionals (specialty physicians, and allied health professionals) engaged in CTIS have been involved together with a methodologist and a patient representative. A review of all the relevant literature and of the existing Guidelines have been performed. Documents, recommendations, and practical approach flow charts have been developed according to a Delphi procedure. A methodological and practical review has been made, and a final Consensus Session was held during the 2011 Barcelona SOSORT Meeting. Results The contents of the document are: methodology; generalities on idiopathic scoliosis; approach to CTIS in different patients, with practical flow-charts; literature review and recommendations on assessment, bracing, physiotherapy, Physiotherapeutic Specific Exercises (PSE) and other CTIS. Sixty-five recommendations have been given, divided in the following topics: Bracing (20 recommendations), PSE to prevent scoliosis progression during growth (8), PSE during brace treatment and surgical therapy (5), Other conservative treatments (3), Respiratory function and exercises (3), Sports activities (6), Assessment (20). No recommendations reached a Strength of Evidence level I; 2 were level II; 7 level III; and 20 level IV; through the Consensus procedure 26 reached level V and 10 level VI. The Strength of Recommendations was Grade A for 13, B for 49 and C for 3; none had grade D. Conclusion These Guidelines have been a big effort of SOSORT to paint the actual situation of CTIS, starting from the evidence, and filling all the gray areas using a scientific

  4. Impact of Increasing Age on Outcomes of Spinal Fusion in Adult Idiopathic Scoliosis

    PubMed Central

    Verla, Terence; Adogwa, Owoicho; Toche, Ulysses; Farber, S. Harrison; Petraglia, Frank; Murphy, Kelly R.; Thomas, Steven; Fatemi, Parastou; Gottfried, Oren; Bagley, Carlos A.; Lad, Shivanand P.

    2016-01-01

    Objective To investigate the role of advancing age on postoperative complications and revision surgery after fusion for scoliosis. Methods A retrospective, cohort study was performed using the Thomson Reuters MarketScan database, examining patients with adult scoliosis who underwent spinal fusion from 2000 to 2009. Primary outcomes included infection, hemorrhage and pulmonary embolism (PE) within 90 days of surgery, and refusion. The effect of increasing age was estimated using the odds ratio (OR) of complications in a multivariate logistic regression analysis, and a Cox proportional hazard model estimated the hazard ratio of refusion. Results A total of 8432 patients were included in this study. Overall, the average age was 53.3 years, with 26.90% males and 39% with a Charlson Comorbidity Score of ≥1. Most patients had commercial insurance (66.81%), with 26.03% and 7.16% covered by Medicare and Medicaid, respectively. Increasing age (per 5-year increment) was a significant predictor of hemorrhagic complication (OR, 1.06; confidence interval [CI], 1.01–1.11; P = 0.0196), PE (OR, 1.09; CI, 1.03–1.16; P = 0.0031), infection (OR, 1.04; CI, 1.01–1.07; P = 0.0053), and refusion (hazard ratio, 1.07; CI, 1.02–1.13; P = 0.0103). Conclusions In this study, age was associated with increased risk of hemorrhage, PE, infection, and refusion. With the aging population, the role of patient age on postoperative healing and outcomes deserves deeper investigation after repair of adult idiopathic scoliosis. PMID:26546999

  5. Methodology of evaluation of morphology of the spine and the trunk in idiopathic scoliosis and other spinal deformities - 6th SOSORT consensus paper

    PubMed Central

    2009-01-01

    Background Comprehensive evaluation of the morphology of the spine and of the whole body is essential in order to correctly manage patients suffering from progressive idiopathic scoliosis. Although methodology of clinical and radiological examination is well described in manuals of orthopaedics, there is deficit of data which clinical and radiological parameters are considered in everyday practise. Recently, an increasing tendency to extend scoliosis examination beyond the measure of the Cobb angle can be observed, reflecting a more patient-oriented approach. Such evaluation often involves surface parameters, aesthetics, function and quality of life. Aim of the study To investigate current recommendations of experts on methodology of evaluation of the patient with spinal deformity, essentially idiopathic scoliosis. Methods Structured Delphi procedure for collecting and processing knowledge from a group of experts with a series of questionnaires and controlled opinion feedback was performed. Experience and opinions of the professionals - physicians and physiotherapists managing scoliosis patients - were studied. According to Delphi method a Meeting Questionnaire (MQ) has been developed, resulting from a preliminary Pre-Meeting Questionnaire (PMQ) which had been previously discussed and approved on line. The MQ was circulated among the SOSORT experts during Consensus Session on "Measurements" which took place at the Annual Meeting of the Society, totally 23 panellists being engaged. Clinical, radiological and surface topography parameters were checked for agreement. Results 90% agreement or more was reached in 35 items and superior than 75% agreement was reached in further 25 items. An evaluation form was proposed to be used by clinicians and researchers. Conclusion The consensus was reached on evaluation of the morphology of the patient with idiopathic scoliosis, comprising clinical, radiological and, to less extend, surface topography assessment. Considering the

  6. 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

  7. Zebrafish models of idiopathic scoliosis link cerebrospinal fluid flow defects to spine curvature.

    PubMed

    Grimes, D T; Boswell, C W; Morante, N F C; Henkelman, R M; Burdine, R D; Ciruna, B

    2016-06-10

    Idiopathic scoliosis (IS) affects 3% of children worldwide, yet the mechanisms underlying this spinal deformity remain unknown. Here we show that ptk7 mutant zebrafish, a faithful developmental model of IS, exhibit defects in ependymal cell cilia development and cerebrospinal fluid (CSF) flow. Transgenic reintroduction of Ptk7 in motile ciliated lineages prevents scoliosis in ptk7 mutants, and mutation of multiple independent cilia motility genes yields IS phenotypes. We define a finite developmental window for motile cilia in zebrafish spine morphogenesis. Notably, restoration of cilia motility after the onset of scoliosis blocks spinal curve progression. Together, our results indicate a critical role for cilia-driven CSF flow in spine development, implicate irregularities in CSF flow as an underlying biological cause of IS, and suggest that noninvasive therapeutic intervention may prevent severe scoliosis. PMID:27284198

  8. Conservative treatment of idiopathic scoliosis according to FITS concept: presentation of the method and preliminary, short term radiological and clinical results based on SOSORT and SRS criteria

    PubMed Central

    2011-01-01

    Background Conservative scoliosis therapy according to the FITS Concept is applied as a unique treatment or in combination with corrective bracing. The aim of the study was to present author's method of diagnosis and therapy for idiopathic scoliosis FITS-Functional Individual Therapy of Scoliosis and to analyze the early results of FITS therapy in a series of consecutive patients. Methods The analysis comprised separately: (1) single structural thoracic, thoracolumbar or lumbar curves and (2) double structural scoliosis-thoracic and thoracolumbar or lumbar curves. The Cobb angle and Risser sign were analyzed at the initial stage and at the 2.8-year follow-up. The percentage of patients improved (defined as decrease of Cobb angle of more than 5 degrees), stable (+/- 5 degrees), and progressed (increase of Cobb angle of more than 5 degrees) was calculated. The clinical assessment comprised: the Angle of Trunk Rotation (ATR) initial and follow-up value, the plumb line imbalance, the scapulae level and the distance from the apical spinous process of the primary curve to the plumb line. Results In the Group A: (1) in single structural scoliosis 50,0% of patients improved, 46,2% were stable and 3,8% progressed, while (2) in double scoliosis 50,0% of patients improved, 30,8% were stable and 19,2% progressed. In the Group B: (1) in single scoliosis 20,0% of patients improved, 80,0% were stable, no patient progressed, while (2) in double scoliosis 28,1% of patients improved, 46,9% were stable and 25,0% progressed. Conclusion Best results were obtained in 10-25 degrees scoliosis which is a good indication to start therapy before more structural changes within the spine establish. PMID:22122964

  9. The Use of Cotrel-Dubousset Instrumentation in the Treatment of Adult Idiopathic Scoliosis

    PubMed Central

    Skubic, John W.; Kostuik, John P.; Suh, Paul B.; Salo, Paul

    1991-01-01

    Between November 1985 and April 1988, forty-nine adults (forty-three females) underwent correction of their spinal deformity secondary to idiopathic scoliosis with Cotrel-Dubousset Instrumentation (CDI). Average age at the time of surgery was forty-three years (twenty-one to seventy-five years) with nineteen patients older than fifty years. Twelve patients had previous spinal fusions and nineteen patients required staged surgery. Follow up averaged thirty-four months (range twenty-four to fifty months). Average preoperative scoliosis was 60 degrees (range 26 to 115 degrees), and at final follow-up averaged 35 degrees (forty-two per cent correction). Previously unfused patients averaged 50 per cent correction. Sagittal curve correction was most dramatic in the hypokyphotic thoracic or hypolordotic lumbar spine, averaging 173 and 133 per cent improvement respectively. For lumbar curves especially, surgical technique depended on the flexibility of the curve, sagittal alignment, and on the need to fuse to the sacrum as determined by preoperative lumbosacral discography and pain reproduction. Twentyeight complications occurred in twenty patients. Loss of fixation due to lumbar lamina fracture and hook pull out occurred in four patients over age fifty who had a staged anterior release. Supplementing distal lumbar fixation with pedicle screws in this category of patients appears to be a viable solution. One nerve root neuropraxia attributable to the instrumentation resolved. Pain relief occurred in 82 per cent. ImagesFigures 1-A-B-C-DFigures 2-A, 2-B, 2-C and 2-DFigures 3-A, 3-B, 3-C and 3-DFigures 4-A, 4-B, 4-C and 4-DFigures 5-A, 5-B, 5-C and 5-DFigures 6-A, 6-B, 6-C and 6-DFigures 7-A, 7-B and 7-C

  10. 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…

  11. 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

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

    PubMed Central

    2014-01-01

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

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

    PubMed

    Tsirikos, Athanasios I; Al-Hourani, Khalid

    2013-11-01

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

  14. 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

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

    PubMed Central

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

    2015-01-01

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

  16. [Preliminary study on the action of hypopressive gymnastics in the treatment of idiopathic scoliosis].

    PubMed

    Caufriez, Marcel; Fernández-Domínguez, Juan Carlos; Brynhildsvoll, Nils

    2011-01-01

    Our goal is to describe the results of a hypopressive gymnastics (HG) program applied to 3 children with idiopathic scoliosis. Three children (ages ranging from 8 to 15 years) suffering from idiopathic scoliosis were recruited for this study. Thoracic or thoracolumbar curves showed between 15° and 40° Cobb degrees. The evolutionary character of the curves had been confirmed. A study of different clinical and radiological parameters was carried out to compare the measurements before and after (3 months later) the application of a 5 HG daily exercises programme: A radiological study of the spine was carried out to compare the measurements of the vertebral rotation. A radiological study, a plumb line and a measuring tape were used to assess the vertebral tilt in this study. A Scoliometer was used to measure the deformation of the rib cage (gibbosity). The most significant results were: a trend to reach stabilisation in the vertebral tilt and rotation, and stabilisation of gibbosity, which probably might improve the respiratory function of these subjects. Finally, the performance of an ordinary HG exercise program shows a trend to control and stabilise dorsal idiopathic scoliosis progression. PMID:22019528

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

    PubMed

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

    2016-05-01

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

  18. Scoliosis

    MedlinePlus

    Scoliosis causes a sideways curve of your backbone, or spine. These curves are often S- or C-shaped. Scoliosis is most common in late childhood and the ... a physical exam, and imaging tests to diagnose scoliosis. Treatment depends on your age, how much more ...

  19. Assessment of the paraspinal muscles of subjects presenting an idiopathic scoliosis: an EMG pilot study

    PubMed Central

    Gaudreault, Nathaly; Arsenault, A Bertrand; Larivière, Christian; DeSerres, Sophie J; Rivard, Charles-Hilaire

    2005-01-01

    Background It is known that the back muscles of scoliotic subjects present abnormalities in their fiber type composition. Some researchers have hypothesized that abnormal fiber composition can lead to paraspinal muscle dysfunction such as poor neuromuscular efficiency and muscle fatigue. EMG parameters were used to evaluate these impairments. The purpose of the present study was to examine the clinical potential of different EMG parameters such as amplitude (RMS) and median frequency (MF) of the power spectrum in order to assess the back muscles of patients presenting idiopathic scoliosis in terms of their neuromuscular efficiency and their muscular fatigue. Methods L5/S1 moments during isometric efforts in extension were measured in six subjects with idiopathic scoliosis and ten healthy controls. The subjects performed three 7 s ramp contractions ranging from 0 to 100% maximum voluntary contraction (MVC) and one 30 s sustained contraction at 75% MVC. Surface EMG activity was recorded bilaterally from the paraspinal muscles at L5, L3, L1 and T10. The slope of the EMG RMS/force (neuromuscular efficiency) and MF/force (muscle composition) relationships were computed during the ramp contractions while the slope of the EMG RMS/time and MF/time relationships (muscle fatigue) were computed during the sustained contraction. Comparisons were performed between the two groups and between the left and right sides for the EMG parameters. Results No significant group or side differences between the slopes of the different measures used were found at the level of the apex (around T10) of the major curve of the spine. However, a significant side difference was seen at a lower level (L3, p = 0.01) for the MF/time parameter. Conclusion The EMG parameters used in this study could not discriminate between the back muscles of scoliotic subjects and those of control subject regarding fiber type composition, neuromuscular efficiency and muscle fatigue at the level of the apex. The

  20. Association Study between Promoter Polymorphism of TPH1 and Progression of Idiopathic Scoliosis

    PubMed Central

    Yablanski, Vasil; Nikolova, Svetla; Vlaev, Evgeni; Savov, Alexey; Kremensky, Ivo

    2016-01-01

    The concept of disease-modifier genes as an element of genetic heterogeneity has been widely accepted and reported. The aim of the current study is to investigate the association between the promoter polymorphism TPH1 (rs10488682) and progression of idiopathic scoliosis (IS) in Eastern European population sample. A total of 105 patients and 210 healthy gender-matched controls were enrolled in this study. The TPH1 promoter polymorphism was genotyped by amplification followed by restriction. The statistical analysis was performed by Fisher's Exact Test. The results indicated that the genotypes and alleles of TPH1 (rs10488682) are not correlated with curve severity, curve pattern, or bracing. Therefore, the examined polymorphic variant could not be considered as a genetic factor with modifying effect of IS. In conclusion, this case-control study revealed no statistically significant association between TPH1 (rs10488682) and progression of IS in Eastern European population sample. These preliminary results should be replicated in extended population studies including larger sample sizes. The identification of molecular markers for IS could be useful for a more accurate prognosis of the risk for a rapid progression of the curve. That would permit early stage treatment of the patient with the least invasive procedures. PMID:27293961

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

    PubMed

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

    2011-05-01

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

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

    PubMed Central

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

    2008-01-01

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

  3. 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

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

    PubMed Central

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

    2015-01-01

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

  5. 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

  6. In Search of Biomarkers for Idiopathic Scoliosis: Leptin and BMP4 Functional Polymorphisms

    PubMed Central

    Nikolova, Svetla; Yablanski, Vasil; Vlaev, Evgeni; Getova, Gergana; Atanasov, Ventseslav; Stokov, Luben; Savov, Alexey Slavkov; Kremensky, Ivo Marinov

    2015-01-01

    Idiopathic scoliosis (IS) is the most common spinal disorder in children and adolescents. The current consensus on IS maintains that it has a multifactorial etiology with genetic predisposition factors. In the present study the association of two functional polymorphisms of leptin (rs7799039) and BMP4 (rs4898820) with susceptibility to IS and curve severity was investigated in a Bulgarian population sample. The molecular detection of the genotypes was performed by amplification followed by restriction technology. The statistical analysis was performed by Pearson's chi-squared test. This case-control study revealed no statistically significant association between the functional polymorphisms of leptin and BMP4 and susceptibility to IS or curve progression (p > 0.05). On the basis of these results the examined polymorphic variants of leptin and BMP4 could not be considered as genetic variants with predisposition effect or as risk factors for the progression of the curve. In addition, these results do not exclude a synergistic effect of the promoter polymorphisms of leptin and BMP4 in the etiology and pathogenesis of IS. The identification of molecular markers for IS could be useful for early detection and prognosis of the risk for a rapid progression of the curve. That would permit early stage treatment of the patient with the least invasive procedures. PMID:26317037

  7. 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

  8. 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

  9. 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

  10. 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

  11. 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

  12. Migraine vestibulopathy in three families with ıdiopathic scoliosis: a case series

    PubMed Central

    2009-01-01

    Introduction We assessed clinical and etiological association between vestibular pathology and idiopathic scoliosis concerning seven members of three families with idiopathic scoliosis. Case presentation The families were referred to neurotology center for evaluation of balance problems. Patients were evaluated thorough anamnesis to relevant vestibular and audiological studies in addition to idiopathic scoliosis assessment. All evaluated scoliotic patients had clinical manifestations of vestibular dysfunctions and migrainous headache. All of the scoliotic patients (seven patients) in these three families were diagnosed as migraine vestibulopathy. Conclusion With the presentation of these three families, we discussed the probable role of the vestibular dysfunctions including migraine vestibulopathy in the development and progression of idiopathic scoliosis. PMID:20062612

  13. 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

  14. 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

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

    PubMed

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

    2010-07-01

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

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

    PubMed Central

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

    2010-01-01

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

  17. 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

  18. Model-based registration for assessment of spinal deformities in idiopathic scoliosis

    NASA Astrophysics Data System (ADS)

    Forsberg, Daniel; Lundström, Claes; Andersson, Mats; Knutsson, Hans

    2014-01-01

    Detailed analysis of spinal deformity is important within orthopaedic healthcare, in particular for assessment of idiopathic scoliosis. This paper addresses this challenge by proposing an image analysis method, capable of providing a full three-dimensional spine characterization. The proposed method is based on the registration of a highly detailed spine model to image data from computed tomography. The registration process provides an accurate segmentation of each individual vertebra and the ability to derive various measures describing the spinal deformity. The derived measures are estimated from landmarks attached to the spine model and transferred to the patient data according to the registration result. Evaluation of the method provides an average point-to-surface error of 0.9 mm ± 0.9 (comparing segmentations), and an average target registration error of 2.3 mm ± 1.7 (comparing landmarks). Comparing automatic and manual measurements of axial vertebral rotation provides a mean absolute difference of 2.5° ± 1.8, which is on a par with other computerized methods for assessing axial vertebral rotation. A significant advantage of our method, compared to other computerized methods for rotational measurements, is that it does not rely on vertebral symmetry for computing the rotational measures. The proposed method is fully automatic and computationally efficient, only requiring three to four minutes to process an entire image volume covering vertebrae L5 to T1. Given the use of landmarks, the method can be readily adapted to estimate other measures describing a spinal deformity by changing the set of employed landmarks. In addition, the method has the potential to be utilized for accurate segmentations of the vertebrae in routine computed tomography examinations, given the relatively low point-to-surface error.

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

    PubMed Central

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

    2009-01-01

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

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

    PubMed

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

    2016-08-01

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

  1. 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

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

    PubMed

    Byun, Sunghak; Han, Dongwook

    2016-04-01

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

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

    PubMed Central

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

    2016-01-01

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

  4. Optimization Correction Strength Using Contra Bending Technique without Anterior Release Procedure to Achieve Maximum Correction on Severe Adult Idiopathic Scoliosis

    PubMed Central

    Rahyussalim, Ahmad Jabir; Saleh, Ifran; Purnaning, Dyah; Kurniawati, Tri

    2016-01-01

    Adult scoliosis is defined as a spinal deformity in a skeletally mature patient with a Cobb angle of more than 10 degrees in the coronal plain. Posterior-only approach with rod and screw corrective manipulation to add strength of contra bending manipulation has correction achievement similar to that obtained by conventional combined anterior release and posterior approach. It also avoids the complications related to the thoracic approach. We reported a case of 25-year-old male adult idiopathic scoliosis with double curve. It consists of main thoracic curve of 150 degrees and lumbar curve of 89 degrees. His curve underwent direct contra bending posterior approach using rod and screw corrective manipulation technique to achieve optimal correction. After surgery the main thoracic Cobb angle becomes 83 degrees and lumbar Cobb angle becomes 40 degrees, with 5 days length of stay and less than 800 mL blood loss during surgery. There is no complaint at two months after surgery; he has already come back to normal activity with good functional activity. PMID:27064801

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

    PubMed Central

    2013-01-01

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

  6. Could CCI or FBCI Fully Eliminate the Impact of Curve Flexibility When Evaluating the Surgery Outcome for Thoracic Curve Idiopathic Scoliosis Patient? A Retrospective Study

    PubMed Central

    Ni, Haijian; Zhu, Xiaodong; Li, Ming

    2015-01-01

    Purpose To clarify if CCI or FBCI could fully eliminate the influence of curve flexibility on the coronal correction rate. Methods We reviewed medical record of all thoracic curve AIS cases undergoing posterior spinal fusion with all pedicle screw systems from June 2011 to July 2013. Radiographical data was collected and calculated. Student t test, Pearson correlation analysis and linear regression analysis were used to analyze the data. Results 60 were included in this study. The mean age was 14.7y (10-18y) with 10 males (17%) and 50 females (83%). The average Risser sign was 2.7. The mean thoracic Cobb angle before operation was 51.9°. The mean bending Cobb angle was 27.6° and the mean fulcrum bending Cobb angle was 17.4°. The mean Cobb angle at 2 week after surgery was 16.3°. The Pearson correlation coefficient r between CCI and BFR was -0.856(P<0.001), and between FBCI and FFR was -0.728 (P<0.001). A modified FBCI (M-FBCI) = (CR-0.513)/BFR or a modified CCI (M-CCI) = (CR-0.279)/FFR was generated by curve estimation has no significant correlation with FFR (r=-0.08, p=0.950) or with BFR (r=0.123, p=0.349). Conclusions Fulcrum-bending radiographs may better predict the outcome of AIS coronal correction than bending radiographs in thoracic curveAIS patients. Neither CCI nor FBCI can fully eliminate the impact of curve flexibility on the outcome of correction. A modified CCI or FBCI can better evaluating the corrective effects of different surgical techniques or instruments. PMID:25984945

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

    PubMed

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

    2016-01-01

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

  8. 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

  9. 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

  10. Sensory reweighting is altered in adolescent patients with scoliosis: Evidence from a neuromechanical model.

    PubMed

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

    2015-10-01

    Idiopathic scoliosis is the most frequent spinal deformity in adolescence. While its aetiology remains unclear, impairments in balance control suggest a dysfunction of the sensorimotor control mechanisms. The objective of this paper is to evaluate the ability of patients with idiopathic scoliosis to reweigh sensory information. Using a neuromechanical model, the relative sensory weighting of vestibular and proprioceptive information was assessed. Sixteen healthy adolescents and respectively 20 and 16 adolescents with mild or severe scoliosis were recruited. Binaural bipolar galvanic vestibular stimulation was delivered to elicit postural movement along the coronal plane. The kinematics of the upper body, using normalized horizontal displacement of the 7th cervical vertebra, was recorded 1s before, 2s during, and 1s following vestibular stimulation. The neuromechanical model included active feedback mechanisms that generated corrective torque from the vestibular and proprioceptive error signals. The model successfully predicted the normalized horizontal displacement of the 7th cervical vertebra. All groups showed similar balance control before vestibular stimulation; however, the amplitude (i.e., peak horizontal displacement) of the body sway during and immediately following vestibular stimulation was approximately 3 times larger in patients compared to control adolescents. The outcome of the model revealed that patients assigned a larger weight to vestibular information compared to controls; vestibular weight was 6.03% for controls, whereas it was 13.09% and 13.26% for the mild and severe scoliosis groups, respectively. These results suggest that despite the amplitude of spine deformation, the sensory reweighting mechanism is altered similarly in adolescent patients with scoliosis. PMID:26371828

  11. The Level of Self-Esteem and Sexual Functioning in Women with Idiopathic Scoliosis: A Preliminary Study

    PubMed Central

    Durmała, Jacek; Blicharska, Irmina; Drosdzol-Cop, Agnieszka; Skrzypulec-Plinta, Violetta

    2015-01-01

    A person’s image, which is determined through physical appearance, considerably affects self-esteem developed from early childhood. Scoliosis causes multiple trunk deformations that can affect a person’s perception of the body. The aim of the study was to analyze the impact of scoliosis dimension and the degree of trunk deformation on the level of self-esteem and sexual functioning in women with idiopathic scoliosis. Thirty-six women diagnosed with idiopathic scoliosis were recruited to a prospective, double-blind, randomized controlled trial. The subjects were divided into two groups depending on the value of the Cobb angle. The level of self-esteem was determined by means of the Rosenberg Self-Esteem Scale (SES), whereas the sexual functioning was assessed via the Female Sexual Function Index (FSFI). The trunk deformations were specified with the Posterior Trunk Symmetry Index (POTSI). A statistically significant correlation was proved between the amount of points received in the Rosenberg scale evaluation and the POTSI index in Group A (R = −0.56, p = 0.04). Subjects with smaller deformations within the coronal plane had a higher level of self-confidence. The trunk asymmetries in the coronal plane may have a negative effect on women with scoliosis and their self-appraisal. PMID:26274967

  12. The Level of Self-Esteem and Sexual Functioning in Women with Idiopathic Scoliosis: A Preliminary Study.

    PubMed

    Durmała, Jacek; Blicharska, Irmina; Drosdzol-Cop, Agnieszka; Skrzypulec-Plinta, Violetta

    2015-08-01

    A person's image, which is determined through physical appearance, considerably affects self-esteem developed from early childhood. Scoliosis causes multiple trunk deformations that can affect a person's perception of the body. The aim of the study was to analyze the impact of scoliosis dimension and the degree of trunk deformation on the level of self-esteem and sexual functioning in women with idiopathic scoliosis. Thirty-six women diagnosed with idiopathic scoliosis were recruited to a prospective, double-blind, randomized controlled trial. The subjects were divided into two groups depending on the value of the Cobb angle. The level of self-esteem was determined by means of the Rosenberg Self-Esteem Scale (SES), whereas the sexual functioning was assessed via the Female Sexual Function Index (FSFI). The trunk deformations were specified with the Posterior Trunk Symmetry Index (POTSI). A statistically significant correlation was proved between the amount of points received in the Rosenberg scale evaluation and the POTSI index in Group A (R = -0.56, p = 0.04). Subjects with smaller deformations within the coronal plane had a higher level of self-confidence. The trunk asymmetries in the coronal plane may have a negative effect on women with scoliosis and their self-appraisal. PMID:26274967

  13. Spinal Fusion for Scoliosis in Rett Syndrome With an Emphasis on Respiratory Failure and Opioid Usage.

    PubMed

    Rumbak, Dania M; Mowrey, Wenzhu; W Schwartz, Skai; Sarwahi, Vishal; Djukic, Aleksandra; Killinger, James S; Katyal, Chhavi

    2016-02-01

    Our objective was to characterize our experience with 8 patients with Rett syndrome undergoing scoliosis surgery in regard to rates of respiratory failure and rates of ventilator-acquired pneumonia in comparison to patients with neurologic scoliosis and adolescent idiopathic scoliosis. This study was a retrospective chart review of patients undergoing scoliosis surgery at a tertiary children's hospital. Patients were divided into 3 groups: (1) adolescent idiopathic scoliosis, (2) neurologic scoliosis, and (3) Rett syndrome. There were 133 patients with adolescent idiopathic scoliosis, 48 patients with neurologic scoliosis, and 8 patients with Rett syndrome. We found that patients with Rett syndrome undergoing scoliosis surgery have higher rates of respiratory failure and longer ventilation times in the postoperative period when compared with both adolescent idiopathic scoliosis and neurologic scoliosis patients. There is insufficient evidence to suggest a difference in the incidence of ventilator-acquired pneumonia between the Rett syndrome and the neurologic scoliosis group. We believe our findings are the first in the literature to show a statistically significant difference between these 3 groups in regard to incidence of respiratory failure. PMID:25991642

  14. Scoliosis

    MedlinePlus

    ... or an uneven waist with a tendency to lean to one side. Because scoliosis can develop very ... orthopedist, he or she will examine you and study X-rays of your spine. You ... deep under the spine muscles. In most cases they can't be felt and do not ...

  15. Secondary scoliosis after thoracotomy in patients with aortic coarctation and patent ductus arteriosus.

    PubMed

    Roclawski, Marek; Pankowski, Rafal; Smoczynski, Andrzej; Ceynowa, Marcin; Kloc, Wojciech; Wasilewski, Wojciech; Jende, Piotr; Liczbik, Wieslaw; Beldzinski, Piotr; Libionka, Witold; Pierzak, Olaf; Adamski, Stanislaw; Niedbala, Miroslaw

    2012-01-01

    The aim of this study was to determine the influence of lateral thoracotomy on the development of scoliosis in subjects undergoing repair of coarctation of the aorta (CoAo) and patent ductus arteriosus (PDA). A group of 133 patients with CoAo and PDA was evaluated. Forty-five patients with CoAo and 38 with PDA were operated on using lateral thoracotomy (operative group) while 12 patients with CoAo and 31 with PDA were treated using balloon dilatation and stent or coil implantation (non-operative group). Clinical examination and the evaluation of spinal roentgenograms were performed. Among the operated patients 46.6% of those with CoAo and 39.5% of those with PDA had clinical scoliosis. In the non-operated patients scoliosis was present in only 16.6% of those with CoAo and 12.9% of those with PDA. Scoliosis ranged between 10° and 42° and it was mild in the majority of cases. In 90.4% of the operated scoliotic patients with CoAo and 73.3% of those with PDA the curve was thoracic and in 47.6% of the CoAo group and 53,3% of the PDA group the curve was left sided. All curves were right sided in non-operated subjects. Scoliosis in the operated group was higher in males than in females (63.3% versus 60% in CoAo and 68.2% versus 37.5% in PDA). The prevalence of scoliosis after thoracotomy was significantly higher than after non-surgical methods of treatment of both CoAo and PDA as well as in the general population. The rate of single thoracic and the rate of left thoracic curves in patients after thoracotomy is higher than in patients treated non-surgically or in idiopathic scoliosis. The rate of scoliosis after thoracotomy is higher in males than females especially following thoracotomy for PDA. PMID:22744454

  16. Surgical approaches for the management of idiopathic thoracic scoliosis and the indications for combined anterior-posterior technique.

    PubMed

    Rauzzino, M J; Shaffrey, C I; Wagner, J; Nockels, R; Abel, M

    1999-05-15

    The indications for surgical intervention in patients with idiopathic scoliosis have been well defined. The goals of surgery are to achieve fusion and arrest progressive curvature while restoring normal coronal and sagittal balance. As first introduced by Harrington, posterior fusion, the gold standard of treatment, has a proven record of success. More recently, anterior techniques for performing fusion procedures via either a thoracotomy or a retroperitoneal approach have been popularized in attempts to achieve better correction of curvature, preserve motion segments, and avoid some of the complications of posterior fusion such as the development of the flat-back syndrome. Anterior instrumentation alone, although effective, can be kyphogenic and has been shown to be associated with complications such as pseudarthrosis and instrumentation failure. Performing a combined approach in patients with scoliosis and other deformities has become an increasingly popular procedure to achieve superior correction of deformity and to minimize later complications. Indications for a combined approach (usually consisting of anterior release, arthrodesis with or without use of instrumentation, and posterior segmental fusion) include: prevention of crankshaft phenomenon in juvenile or skeletally immature adolescents; correction of large curves (75 degrees ) or excessively rigid curves in skeletally mature or immature patients; correction of curves with large sagittal-plane deformities such as thoracic kyphosis (> 90 degrees ) or thoracic lordosis (> 20 degrees ); and correction of thoracolumbar curves that need to be fused to the sacrum. Surgery may be performed either in a staged proceedure or, more commonly, in a single sitting. The authors discuss techniques for combined surgery and complication avoidance. PMID:17031912

  17. 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

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

    PubMed

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

    2008-01-01

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

  19. Guidelines on "Standards of management of idiopathic scoliosis with corrective braces in everyday clinics and in clinical research": SOSORT Consensus 2008

    PubMed Central

    Negrini, Stefano; Grivas, Theodoros B; Kotwicki, Tomasz; Rigo, Manuel; Zaina, Fabio

    2009-01-01

    Background Reported failure rates,(defined based on percentage of cases progressing to surgery) of corrective bracing for idiopathic scoliosis are highly variable. This may be due to the quality of the brace itself, but also of the patient care during treatment. The latter is sometimes neglected, even though it is considered a main determinant of good results among conservative experts of SOSORT. The aim of this paper was to develop and verify the Consensus on management of scoliosis patients treated with braces Methods We followed a Delphi process in four steps, distributing and gradually changing according to the results a set of recommendations: we involved the SOSORT Board twice, then all SOSORT members twice, with a Pre-Meeting Questionnaire (PMQ), and during a Consensus Session at the SOSORT Athens Meeting with a Meeting Questionnaire (MQ). We set a 90% agreement as the minimum to be reached. Results We had a 71% response rate to PMQ, and 66.7% to MQ. Since the PMQ we had a good agreement (no answers below 72% – 70.2% over 90%). With the MQ the agreement consistently increased for all the answers previously below 90% (no answers below 83%, 75% over 90%). With increasing experience in bracing all numerical criteria tended to become more strict. We finally produced a set of 14 recommendations, grouped in 6 Domains (Experience/competence, Behaviours, Prescription, Construction, Brace Check, Follow-up). Conclusion The Consensus permits establishment of recommendations concerning the standards of management of idiopathic scoliosis with bracing, with the aim to increase efficacy and compliance to treatment. The SOSORT recommends to professionals engaged in patient care to follow the guidelines of this Consensus in their clinical practice. The SOSORT criteria should also be followed in clinical research studies to achieve a minimum quality of care. If the aim is to verify the efficacy of bracing these criteria should be companions of the methodological research

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

    PubMed Central

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

    2014-01-01

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

  1. 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

  2. Characteristics of lateral electrical surface stimulation (LESS) and its effect on the degree of spinal deformity in idiopathic scoliosis

    NASA Astrophysics Data System (ADS)

    Kowalski, Ireneusz M.; Palko, Tadeusz; Pasniczek, Roman; Szarek, Jozef

    2009-01-01

    Clinical studies were carried out in the period of 2003-2006 at the Provincial Children's Rehabilitation Hospital in Ameryka near Olsztyn (Poland). The study involved a group of children and youth exhibiting spinal deformity progression in idiopathic scoliosis (IS) of more than 5° per year according to the Cobb scale. Four hundred and fifty patients between 4 and 15 years of age were divided into three groups (n = 150). Group I and group II received 2-hour and 9-hour lateral electrical surface stimulation (LESS), respectively, whereas group III (control) was treated only with corrective exercises for 30 minutes twice a day. LESS was performed with the use of a battery-operated SCOL-2 stimulator manufactured by Elmech, Warsaw, Poland. The effectiveness of this method was confirmed in the treatment of spinal IS in children and youth, especially when the initial spinal deformity did not exceed 20° according to the Cobb scale. A short-duration electrostimulation (2 hours daily) was found to produce results similar to those obtained after overnight (9 h) electrostimulation. Moreover, the analysis of the Harrington prognostic index F confirms the positive effect of LESS in both groups of patients (2 h and 9 h of LESS).

  3. A Comparison of Cobb Angle: Standing Versus Supine Images of Late-Onset Idiopathic Scoliosis

    PubMed Central

    Vavruch, Ludvig; Tropp, Hans

    2016-01-01

    Summary Background Scoliosis is traditionally evaluated by measuring the Cobb angle in radiograph images taken while the patient is standing. However, low-dose computed tomography (CT) images, which are taken while the patient is in a supine position, provide new opportunities to evaluate scoliosis. Few studies have investigated how the patient’s position, standing or supine, affects measurements. The purpose of this study was to compare the Cobb angle in images from patients while standing versus supine. Material/Methods A total of 128 consecutive patients (97 females and 21 males; mean age 15.5 [11–26] years) with late-onset scoliosis requiring corrective surgery were enrolled. One observer evaluated the type of curve (Lenke classification) and measured the Cobb angle in whole-spine radiography (standing) and scout images from low-dose CT (supine) were taken on the same day. Results For all primary curves, the mean Cobb angle was 59° (SD 12°) while standing and 48° (SD 12°) while in the supine position, with a mean difference of 11° (SD 5°). The correlation between primary standing and supine images had an r value of 0.899 (95% CI 0.860–0.928) and an intra-class correlation coefficient value of 0.969. The correlation between the difference in standing and supine images from primary and secondary curves had an r value of 0.340 (95% CI 0.177–0.484). Conclusions We found a strong correlation between the Cobb angle in images obtained while the patient was standing versus supine for primary and secondary curves. This study is only applicable for patients with severe curves requiring surgical treatment. It enables additional studies based on low-dose CT. PMID:27354881

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

    PubMed Central

    2014-01-01

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

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

    PubMed Central

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

    2014-01-01

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

  6. [Treatment of severe scoliosis].

    PubMed

    Helenius, Ilkka; Pajulo, Olli

    2015-01-01

    Untreated severe scoliosis results in a serious malposition of the back, restrictive lung disease and increases mortality compared with the normal population. Idiopathic scoliosis rarely reaches a severe degree. In the treatment of severe scoliosis, preoperative head traction of several weeks' duration has proven a safe and effective method of correcting the malposition, and will also make scoliosis surgery technically easier. Most cases of severe scoliosis can be treated with anterior pedicle screw instrumentation, and vertebrectomy is only seldom required. PMID:26638663

  7. CHD7 Gene Polymorphisms Are Associated with Susceptibility to Idiopathic Scoliosis

    PubMed Central

    Gao, Xiaochong; Gordon, Derek; Zhang, Dongping; Browne, Richard; Helms, Cynthia; Gillum, Joseph; Weber, Samuel; Devroy, Shonn; Swaney, Saralove; Dobbs, Matthew; Morcuende, Jose; Sheffield, Val; Lovett, Michael; Bowcock, Anne; Herring, John; Wise, Carol

    2007-01-01

    Idiopathic scoliosis (IS) is the most common spinal deformity in children, and its etiology is unknown. To refine the search for genes underlying IS susceptibility, we ascertained a new cohort of 52 families and conducted a follow-up study of genomewide scans that produced evidence of linkage and association with 8q12 loci (multipoint LOD 2.77; P=.0028). Further fine mapping in the region revealed significant evidence of disease-associated haplotypes (P<1.0×10-4) centering over exons 2–4 of the CHD7 gene associated with the CHARGE (coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and/or development, genital and/or urinary abnormalities, and ear abnormalities and deafness) syndrome of multiple developmental anomalies. Resequencing CHD7 exons and conserved intronic sequence blocks excluded coding changes but revealed at least one potentially functional polymorphism that is overtransmitted (P=.005) to affected offspring and predicts disruption of a caudal-type (cdx) transcription-factor binding site. Our results identify the first gene associated with IS susceptibility and suggest etiological overlap between the rare, early-onset CHARGE syndrome and common, later-onset IS. PMID:17436250

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

    PubMed Central

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

    2015-01-01

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

  9. A PAX1 enhancer locus is associated with susceptibility to idiopathic scoliosis in females

    PubMed Central

    Sharma, Swarkar; Londono, Douglas; Eckalbar, Walter L.; Gao, Xiaochong; Zhang, Dongping; Mauldin, Kristen; Kou, Ikuyo; Takahashi, Atsushi; Matsumoto, Morio; Kamiya, Nobuhiro; Murphy, Karl K.; Cornelia, Reuel; Karol, L.; Rathjen, K.; Sucato, D.; Birch, J.; Johnston, C.; Richards, B. S.; Milbrandt, T.; Talwakar, V.; Iwinski, H.; Muchow, R.; Tassone, J. C.; Liu, X. -C.; Shindell, R.; Schrader, W.; Eberson, C.; Lapinsky, A.; Loder, R.; Davey, J.; Hosogane, Naobumi; Ogura, Yoji; Takahashi, Yohei; Miyake, Atushi; Watanabe, Kota; Chiba, Kazuhiro; Toyama, Yoshiaki; Kono, Katsuki; Kawakami, Noriaki; Tsuji, Taichi; Uno, Koki; Suzuki, Teppei; Ito, Manabu; Sudo, Hideki; Minami, Shohei; Kotani, Toshiaki; Yanagida, Haruhisa; Taneichi, Hiroshi; Yonezawa, Ikuho; Kaneko, Kazuo; Herring, John A.; Burns, Dennis; Ahituv, Nadav; Ikegawa, Shiro; Gordon, Derek; Wise, Carol A.

    2015-01-01

    Idiopathic scoliosis (IS) is a common paediatric musculoskeletal disease that displays a strong female bias. By performing a genome-wide association study (GWAS) of 3,102 individuals, we identify significant associations with 20p11.22 SNPs for females (P=6.89 × 10−9) but not males (P=0.71). This association with IS is also found in independent female cohorts from the United States of America and Japan (overall P=2.15 × 10−10, OR=1.30 (rs6137473)). Unexpectedly, the 20p11.22 IS risk alleles were previously associated with protection from early-onset alopecia, another sexually dimorphic condition. The 174-kb associated locus is distal to PAX1, which encodes paired box 1, a transcription factor involved in spine development. We identify a sequence in the associated locus with enhancer activity in zebrafish somitic muscle and spinal cord, an activity that is abolished by IS-associated SNPs. We thus identify a sexually dimorphic IS susceptibility locus, and propose the first functionally defined candidate mutations in an enhancer that may regulate expression in specific spinal cells. PMID:25784220

  10. 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

  11. 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

  12. Effects of consecutive application of stretching, Schroth, and strengthening exercises on Cobb’s angle and the rib hump in an adult with idiopathic scoliosis

    PubMed Central

    Yang, Jae-Man; Lee, Jung-Hoon; Lee, Dae-Hee

    2015-01-01

    [Purpose] To report the effects of consecutive application of stretching, Schroth, and strengthening exercises in an adult with idiopathic scoliosis. [Subject] A 26-year-old woman with idiopathic scoliosis, Cobb’s angle of 20.51°, and back pain. [Methods] The exercise program consisted of 3 sessions: 10 minutes of stretching exercises, 20 minutes of Schroth exercises, and 10 minutes of strengthening exercises. This program was implemented 3 times a week, for 8 weeks. [Results] The thoracic Cobb’s angle decreased from 20.51° to 16.35°, and the rib hump decreased from 15° to 9°. [Conclusion] Consecutive application of stretching, Schroth, and strengthening exercises may help reduce Cobb’s angle and the rib hump in adults with idiopathic scoliosis. PMID:26355577

  13. The correlation between calcaneal valgus angle and asymmetrical thoracic-lumbar rotation angles in patients with adolescent scoliosis.

    PubMed

    Park, Jaeyong; Lee, Sang Gil; Bae, Jongjin; Lee, Jung Chul

    2015-12-01

    [Purpose] This study aimed to provide a predictable evaluation method for the progression of scoliosis in adolescents based on quick and reliable measurements using the naked eye, such as the calcaneal valgus angle of the foot, which can be performed at public facilities such as schools. [Subjects and Methods] Idiopathic scoliosis patients with a Cobb's angle of 10° or more (96 females, 22 males) were included in this study. To identify relationships between factors, Pearson's product-moment correlation coefficient was computed. The degree of scoliosis was set as a dependent variable to predict thoracic and lumbar scoliosis using ankle angle and physique factors. Height, weight, and left and right calcaneal valgus angles were set as independent variables; thereafter, multiple regression analysis was performed. This study extracted variables at a significance level (α) of 0.05 by applying a stepwise method, and calculated a regression equation. [Results] Negative correlation (R=-0.266) was shown between lumbar lordosis and asymmetrical lumbar rotation angles. A correlation (R=0.281) was also demonstrated between left calcaneal valgus angles and asymmetrical thoracic rotation angles. [Conclusion] Prediction of scoliosis progress was revealed to be possible through ocular inspection of the calcaneus and Adams forward bending test and the use of a scoliometer. PMID:26834376

  14. "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

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

    PubMed Central

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

    2009-01-01

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

  16. 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

  17. 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

  18. 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

  19. 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

  20. Scoliosis in patients with aortic coarctation and patent ductus arteriosus: does standard posterolateral thoracotomy play a role in the development of the lateral curve of the spine?

    PubMed

    Roclawski, Marek; Sabiniewicz, Robert; Potaz, Piotr; Smoczynski, Andrzej; Pankowski, Rafal; Mazurek, Tomasz; Daibo, Bawo

    2009-10-01

    The aim of this study was to determine the influence of lateral thoracotomy on the development of scoliosis in subjects undergoing repair of coarctation of the aorta (CoA) and patent ductus arteriosus (PDA). A group of 133 patients with CoA and PDA was evaluated. Forty-five patients with CoA and 38 with PDA underwent surgery using standard posterolateral thoracotomy (operative group), whereas 12 patients with CoA and 31 with PDA were treated using balloon dilatation and stent or coil implantation (nonoperative group). A spinal examination, together with the evaluation of chest and spinal roentgenograms, was conducted. Among the operated patients, 62% of those with CoA and 55% of those with PDA had clinical scoliosis. In the nonoperated patients, scoliosis was present in only 25% of those with CoA and 16% of those with PDA. Scoliosis ranged between 10 degrees and 42 degrees . In 89% of the operated patients with CoA and 76% of those with PDA the curve was thoracic; in 46% of the CoA group and 57% of the PDA group the curve was left-sided. All curves were right-sided in nonoperated subjects. Scoliosis in the operated group was higher in male than in female subjects (63% vs. 60% in CoA and 86% vs. 37% in PDA). The prevalence of scoliosis after standard posterolateral thoracotomy was significantly higher than after nonsurgical treatment methods in the CoA and PDA groups as well as in the general population. The rate of single thoracic and the rate of left-sided thoracic curves in patients after thoracotomy is higher than in nonoperated patients or in those with idiopathic scoliosis. The rate of scoliosis after thoracotomy is higher in male than female patients, especially after thoracotomy for PDA. PMID:19597861

  1. 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

  2. Correlation of idiopathic scoliosis assessments between newly developed Milwaukee Topographic Scanner and Quantec.

    PubMed

    Lim, C H; Tassone, C; Liu, X C; Thometz, J G; Lyon, R

    2012-01-01

    The Milwaukee Topographic Scanner (MTS) is a newly developed system, which utilizes laser technology to obtain three-dimensional topographic evaluation of the spine. The goal of this study was to determine the correlation of topographic measurements between MTS and Quantec Spinal Imaging System. Twelve parameters generated by the MTS and Quantec Spinal Imaging System was compared to each other using the Pearson Coefficient. Twenty patients between the ages 16-18 with scoliosis were evaluated with the MTS and Quantec. There were several parameters, which showed high correlations especially back height (0.972), coronal curve (0.952), and left trunk volume (0.905). MTS is a reliable three-dimensional topographic alternative to radiographs without the exposure to radiation. PMID:22744503

  3. 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

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

    PubMed

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

    2013-01-01

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

  5. 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

  6. A Retrospective Study of Congenital Cardiac Abnormality Associated with Scoliosis

    PubMed Central

    Ucpunar, Hanifi; Sevencan, Ahmet; Balioglu, Mehmet Bulent; Albayrak, Akif; Polat, Veli

    2016-01-01

    Study Design Retrospective study. Purpose To identify the incidence of congenital cardiac abnormalities in patients who had scoliosis and underwent surgical treatment for scoliosis. Overview of Literature Congenital and idiopathic scoliosis (IS) are associated with cardiac abnormalities. We sought to establish and compare the incidence of congenital cardiac abnormalities in patients with idiopathic and congenital scoliosis (CS) who underwent surgical treatment for scoliosis. Methods Ninety consecutive scoliosis patients, who underwent surgical correction of scoliosis, were classified as CS (55 patients, 28 female [51%]) and IS (35 patients, 21 female [60%]). The complete data of the patients, including medical records, plain radiograph and transthoracic echocardiography were retrospectively assessed. Results We found that mitral valve prolapse was the most common cardiac abnormality in both patients with IS (nine patients, 26%) and CS (13 patients, 24%). Other congenital cardiac abnormalities were atrial septal aneurysm (23% of IS patients, 18% of CS patients), pulmonary insufficiency (20% of IS patients, 4% of CS patients), aortic insufficiency (17% of IS patients), atrial septal defect (11% of IS patients, 13% of CS patients), patent foramen ovale (15% of CS patients), dextrocardia (4% of CS patients), bicuspid aortic valve (3% of IS patients), aortic stenosis (2% of CS patients), ventricular septal defect (2% of CS patients), and cardiomyopathy (2% of CS patients). Conclusions We determined the increased incidence of congenital cardiac abnormalities among patients with congenital and IS. Mitral valve prolapse appeared to be the most prevalent congenital cardiac abnormality in both groups. PMID:27114761

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

    PubMed Central

    2012-01-01

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

  8. Severe scoliosis in a patient with severe methylenetetrahydrofolate reductase deficiency.

    PubMed

    Munoz, Tatiana; Patel, Jinesh; Badilla-Porras, Ramses; Kronick, Jonathan; Mercimek-Mahmutoglu, Saadet

    2015-01-01

    Severe methylenetetrahydrofolate reductase (MTHFR) deficiency is a rare autosomal recessively inherited inborn error of folate metabolism. We report a new patient with severe MTHFR deficiency who presented at age 4 months with early onset severe scoliosis associated with severe hypotonia. Markedly decreased MTHFR enzyme activity (0.3 nmoles CHO/mg protein/h; reference range>9) and compound heterozygous mutations (c. 1304T>C; p.Phe435Ser and c.1539dup; p.Glu514Argfs∗24) in the MTHFR gene confirmed the diagnosis. She was treated with vitamin B12, folic acid and betaine supplementation and showed improvements in her developmental milestones and hypotonia. To the best of our knowledge, this is the first patient with MTHFR deficiency reported with severe early onset scoliosis. Despite the late diagnosis and treatment initiation, she showed favorable short-term neurodevelopmental outcome. This case suggests that homocysteine measurement should be included in the investigations of patients with developmental delay, hypotonia and scoliosis within first year of life prior to organizing genetic investigations. PMID:24726568

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

    PubMed

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

    2014-02-01

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

  10. Thoracolumbar Scoliosis in a Patient With Proteus Syndrome

    PubMed Central

    Li, Zheng; Shen, Jianxiong; Liang, Jinqian

    2015-01-01

    Abstract The Proteus syndrome (PS) is a complex and rare congenital hamartomatous condition with a wide range of malformations. Little is reported about spinal deformity associated with this syndrome. This study presents a case of scoliosis occurring in the setting of PS and explores the possible mechanisms between the 2 diseases. The patient is a 17-year-old Chinese female with scoliosis and hemihypertrophy of the right upper and lower extremity as well as exostosis of the right lower leg joint including the hip, knee, ankle, and toes. These manifestations were suggestive of PS. She underwent a posterior correction at thoracic 2-lumbar 4 (T5–L4) levels, using the Moss-SI spinal system. At 3-month follow-ups, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. The severity of scoliosis in PS is progressively aggravated and the correction of the extensive spinal deformities is generally difficult. Therefore, early diagnosis is required for adequate interdisciplinary treatment. PMID:25654373

  11. 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

  12. Neuroimaging of scoliosis in childhood.

    PubMed

    Kim, F M; Poussaint, T Y; Barnes, P D

    1999-02-01

    A curvature abnormality may be the initial or major presenting feature in a child with disease of the spinal column or spinal neuraxis. A simplified classification of common spinal curvature abnormalities of childhood include idiopathic, congenital/dysraphic, skeletal dysplasia, neurofibromatosis, and painful. The great majority of childhood scoliosis falls into the idiopathic category. Atypical clinical or radiographic features in a presumed idiopathic scoliosis may indicate an otherwise occult tumor or hydrosyringomyelia, or may be a consequence of increasing curvature with disk protrusion, nerve impingement, or cord attenuation. Neuroimaging beyond plain films is commonly necessary for atypical idiopathic scoliosis and for the other categories of scoliosis listed. PMID:9974506

  13. Radiation exposure to patients receiving routine scoliosis radiography measured at depth in an anthropomorphic phantom

    SciTech Connect

    Dutkowsky, J.P.; Shearer, D.; Schepps, B.; Orton, C.; Scola, F. )

    1990-07-01

    Concern about the amount of radiation received during scoliosis evaluation and treatment led us to measure radiation exposure in an anthropomorphic phantom to determine the increased risk of breast cancer in young women with scoliosis. Assuming that 22 radiographic examinations were performed over the course of scoliosis treatment, the increased relative risk of breast cancer was determined to be 0.22% in these patients.

  14. Effects of Schroth and Pilates exercises on the Cobb angle and weight distribution of patients with scoliosis.

    PubMed

    Kim, Gichul; HwangBo, Pil-Neo

    2016-03-01

    [Purpose] The purpose of this study was to compare the effect of Schroth and Pilates exercises on the Cobb angle and body weight distribution of patients with idiopathic scoliosis. [Subjects] Twenty-four scoliosis patients with a Cobb angle of ≥20° were divided into the Schroth exercise group (SEG, n = 12) and the Pilates exercise group (PEG, n = 12). [Methods] The SEG and PEG performed Schroth and Pilates exercises, respectively, three times a week for 12 weeks. The Cobb angle was measured in the standing position with a radiography apparatus, and weight load was measured with Gait View Pro 1.0. [Results] In the intragroup comparison, both groups showed significant changes in the Cobb angle. For weight distribution, the SEG showed significant differences in the total weight between the concave and convex sides, but the PEG did not show significant differences. Furthermore, in the intragroup comparison, the SEG showed significant differences in the changes in the Cobb angle and weight distribution compared with the PEG. [Conclusion] Both Schroth and Pilates exercises were effective in changing the Cobb angle and weight distribution of scoliosis patients; however, the intergroup comparison showed that the Schroth exercise was more effective than the Pilates exercise. PMID:27134403

  15. Effects of Schroth and Pilates exercises on the Cobb angle and weight distribution of patients with scoliosis

    PubMed Central

    Kim, Gichul; HwangBo, Pil-neo

    2016-01-01

    [Purpose] The purpose of this study was to compare the effect of Schroth and Pilates exercises on the Cobb angle and body weight distribution of patients with idiopathic scoliosis. [Subjects] Twenty-four scoliosis patients with a Cobb angle of ≥20° were divided into the Schroth exercise group (SEG, n = 12) and the Pilates exercise group (PEG, n = 12). [Methods] The SEG and PEG performed Schroth and Pilates exercises, respectively, three times a week for 12 weeks. The Cobb angle was measured in the standing position with a radiography apparatus, and weight load was measured with Gait View Pro 1.0. [Results] In the intragroup comparison, both groups showed significant changes in the Cobb angle. For weight distribution, the SEG showed significant differences in the total weight between the concave and convex sides, but the PEG did not show significant differences. Furthermore, in the intragroup comparison, the SEG showed significant differences in the changes in the Cobb angle and weight distribution compared with the PEG. [Conclusion] Both Schroth and Pilates exercises were effective in changing the Cobb angle and weight distribution of scoliosis patients; however, the intergroup comparison showed that the Schroth exercise was more effective than the Pilates exercise. PMID:27134403

  16. Idiopathic scoliosis. Mechanical properties of the respiratory system and the ventilatory response to carbon dioxide.

    PubMed

    Kafer, E R

    1975-06-01

    The aims were to examine the effects of scoliosis (angle), and age on lung volumes, elastic properties of the respiratory system, and the ventilatory response to CO2. The mean age of the 55 patients was 25.4 plus or minus SEM 2.5 yr, and the mean angle was 80 plus or minus SEM 4.2. The mean plus or minus SEM percent predicted lung volumes were vital capacity (VC), 60.5 plus or minus 2.7; total lung capacity (TLC), 70,2 plus or minus 2.6; functional residual capacity (frc), 79.3 plus or minus 3.2; and residual volume (RV), 99.7 plus or minus 5.2. The correlation coefficients between the angle of scoliosis and each of the following were significant: TLC (-0.548), percent predicted TLC (-0.547), VC (-0.485), percent predicted VC (-0.523), FRC (-0.533), percent predicted FRC (-0.338), RV (-0.438), and percent predicted RV (-0.318). The mean compliance of the total respiratory system (Crs) was 0.049 litter/cm H2O plus or minus SEM 0.004, and the mean compliance of the chest wall (Ccw) was 0.080 liter/cm H2O plus or minus SEM 0.012. The Crs and Ccw were inversely proportional to the angle (r-0.620 and -0.721) and directly proportional to the height and the weight. The mean deltaV/deltaPco2 was 1.32 liter/min per mm Hg (SEM 0.171), and the mean deltaVt/deltaPco2 was 28.9 ml/mm Hg (SEM 3.64). The correlation coefficients between deltaV/deltaPco2 and the following were height, 0.499; VC, 0.792; TLC, 0.632; AND Crs, 0.520; and between the deltaTt/deltaPco2 and the following were height, 0.500; VC, 0.878; TLC, 0.802; and Crs, 0.590. We conclude that body size and the deformity were the determinants of the lung volumes and the mechanical properties of the respiratory system, and that these variables were the major factors in both the magnitude and pattern of the ventilatory response to CO2. The correlations between age and the mechanical properties of the respiratory sytem, deltaV/deltaPco2, and deltaVt/deltaPco2, were not significant, but the correlation coefficients between

  17. Present day explanation of the clinical signs in the biomechanical aetiology of the so-called idiopathic scoliosis (1995-2011). The relationship between the "model of hips movement" and the character of scoliosis; three groups and four types. The causative role of "gait" and "standing "at ease" on the right leg".

    PubMed

    Karski, Tomasz

    2012-01-01

    The observations from 1985-1995 and till 2012 clarify that the development of so-called idiopathic scoliosis is connected with "gait" and habitual permanent "standing at ease" on the right leg. The scoliosis is "a result" of asymmetry of "function" - "changed" loading during gait and asymmetry in time during 'at ease' standing, more prevalent on the right leg. Every types of scoliosis is connected with the adequate "model of hips movements" [MHM] (Karski et al., 2006 [1]). This new classification clarifies the therapeutic approach to each types of scoliosis and provides the possibility to introduce causative prophylaxis. PMID:22744476

  18. Scoliosis Related Information on the Internet in China: Can Patients Benefit from This Information?

    PubMed Central

    Bao, Hongda; Zhu, Feng; Wang, Fei; Liu, Zhen; Bao, Mike H.; He, Shouyu; Zhu, Zezhang; Qiu, Yong

    2015-01-01

    Background There has been an increasing popularity of searching health related information online in recent years. Despite that considerable amount of scoliosis patients have shown interest in obtaining scoliosis information through Internet, previous studies have demonstrated poor quality of online information. However, this conclusion may vary depending on region and culture. Since China has a restricted Internet access outside of its borders, the aim of this study is to evaluate the quality of scoliosis information available online using recognized scoring systems and to analyze the Internet as a source of health information in China. Methods A survey-based questionnaire was distributed to 280 respondents at outpatient clinics. Information on demographics and Internet use was collected. Binary logistic analysis was performed to identify possible predictors for the use of Internet. In addition, the top 60 scoliosis related websites assessed through 4 search engines were reviewed by a surgeon and the quality of online information was evaluated using DISCERN score and JAMA benchmark. Results Use of the Internet as a source for scoliosis related information was confirmed in 87.8% of the respondents. College education, Internet access at home and urban residence were identified as potential predictors for Internet use. However, the quality of online scoliosis related information was poor with an average DISCERN score of 27.9±11.7 and may be misleading for scoliosis patients. Conclusion The study outlines the profile of scoliosis patients who use the Internet as a source of health information. It was shown that 87.8% of the scoliosis patients in outpatient clinics have searched for scoliosis related information on Internet. Urban patients, higher education and Internet access at home were identified as potential predictors for Internet search. However, the overall quality of online scoliosis related information was poor and confusing. Physician based websites seemed

  19. 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

  20. Low expression of microRNA-143 is related to degenerative scoliosis possibly by regulation of cyclooxygenase-2 expression

    PubMed Central

    Zheng, Jie; Yang, Yonghong; Zhao, Kefeng; Wang, Ran

    2015-01-01

    Aims: This study is to determine if expression level of microRNA-143 (miR-143) and cyclooxygenase-2 (COX-2) are related to the occurrence and development of degenerative scoliosis. Methods: A total of 30 patients with degenerative scoliosis, 30 patients with adolescent idiopathic scoliosis were enrolled in this study. For control, 30 patients with spinal burst fractures were also enrolled in this study. Real-time PCR and western blotting was performed to measure the expression levels of COX-2 in intervertebral disc tissues, peripheral blood and cerebrospinal. Expression levels of miR-143 in intervertebral disc tissues, peripheral blood and cerebrospinal were detected by real-time PCR. Results: The expression levels of COX-2 were increased in intervertebral disc tissues, peripheral blood and cerebrospinal of patients with degenerative scoliosis when compared with those of patients with adolescent idiopathic scoliosis and spinal burst fractures (P < 0.05). However, the expression levels of miR-143 were decreased in intervertebral disc tissues, peripheral blood and cerebrospinal of patients with degenerative scoliosis when compared with those of patients with adolescent idiopathic scoliosis and spinal burst fractures (P < 0.05). Conclusions: COX-2 is highly expressed whereas miR-143 is lowly expressed in patients with degenerative scoliosis. Decreased expression of miR-143 may be related to the aggravation of degenerative scoliosis by regulation of COX-2. PMID:26064322

  1. The effect of compliance to a Rigo System Cheneau brace and a specific exercise programme on idiopathic scoliosis curvature: a comparative study: SOSORT 2014 award winner

    PubMed Central

    2014-01-01

    Background There is controversy as to whether conservative management that includes wearing a brace and exercises is effective in stabilising idiopathic scoliosis curves. A brace only prevents progression of the curve and has been shown to have favourable outcomes when patients are compliant. So the aim of this study was to: determine the effect of compliance to the Rigo System Cheneau (RSC) brace and a specific exercise programme on Idiopathic Scoliosis curvature; and to compare the Quality of Life (QoL) and psychological traits of compliant and non compliant subjects. Methods A pre/post test study design was used with a post study comparison between subjects who complied with the management and those who did not. Fifty one subjects, girls aged 12-16 years, Cobb angles 20-50 degrees participated in the study. Subjects were divided into two groups, according to their compliance, at the end of the study. The compliant group wore the brace 20 or more hours a day and exercised three or more times per week. The non-compliant group wore the brace less than 20 hours a day and exercised less than three times per week. Cobb angles, vertebral rotation, scoliometer readings, peak flow, quality of life and personality traits were compared between groups, using the student’s two sample t-test and an analysis of covariance. Results The compliant group, wore the brace 21.5 hours per day and exercised four times a week, and significantly improved in all measures compared to non compliant subjects, who wore the brace 12 hours per day, exercised 1.7 times a week and significantly deteriorated (p < 0.0001). The major Cobb angles in the compliant group improved 10.19°(±5.5) and deteriorated 5.52°(±4.3) in the non compliant group (p < 0.0001). Compliant subjects had a significantly better QoL than the non compliant subjects (p = 0.001). The compliant group were significantly more emotionally mature, stable and realistic than the non compliant group (p = 0

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

    PubMed Central

    2011-01-01

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

  3. Raster-stereographic evaluation of the effects of biomechanical foot orthoses in patients with scoliosis

    PubMed Central

    Park, So Min; Ahn, Sang-Ho; Lee, A-Young; Park, In-Sik; Cho, Yun-Woo

    2016-01-01

    [Purpose] Little is known about the effects of biomechanical foot orthoses in scoliosis, as determined by raster stereography. The objective of this study was to investigate the effect of individually manufactured biomechanical foot orthoses on scoliosis angle, trunk imbalance, and pelvic obliquity by comparing them with general insoles by using DIERS formetric 4 dimensional in patients with scoliosis. [Subjects and Methods] Twenty-six patients with scoliosis were recruited at Yeungnam University Hospital and allocated equally to one of two groups, the biomechanical foot orthoses group or the control group. Parameters, such as, trunk rotation, imbalance, and scoliosis angle, were obtained using a DIERS formetric 4D. [Results] Scoliosis angle, pelvic obliquity, and trunk imbalance were significantly different between the two groups and improved in the biomechanical foot orthoses group with time, but no significant improvement in any parameter was observed in the control group. [Conclusion] Biomechanical foot orthoses could be effective in patients with scoliosis, and DIERS formetric 4D provides a useful method for evaluating scoliosis parameters. PMID:27512245

  4. Raster-stereographic evaluation of the effects of biomechanical foot orthoses in patients with scoliosis.

    PubMed

    Park, So Min; Ahn, Sang-Ho; Lee, A-Young; Park, In-Sik; Cho, Yun-Woo

    2016-07-01

    [Purpose] Little is known about the effects of biomechanical foot orthoses in scoliosis, as determined by raster stereography. The objective of this study was to investigate the effect of individually manufactured biomechanical foot orthoses on scoliosis angle, trunk imbalance, and pelvic obliquity by comparing them with general insoles by using DIERS formetric 4 dimensional in patients with scoliosis. [Subjects and Methods] Twenty-six patients with scoliosis were recruited at Yeungnam University Hospital and allocated equally to one of two groups, the biomechanical foot orthoses group or the control group. Parameters, such as, trunk rotation, imbalance, and scoliosis angle, were obtained using a DIERS formetric 4D. [Results] Scoliosis angle, pelvic obliquity, and trunk imbalance were significantly different between the two groups and improved in the biomechanical foot orthoses group with time, but no significant improvement in any parameter was observed in the control group. [Conclusion] Biomechanical foot orthoses could be effective in patients with scoliosis, and DIERS formetric 4D provides a useful method for evaluating scoliosis parameters. PMID:27512245

  5. Evaluation of IRX Genes and Conserved Noncoding Elements in a Region on 5p13.3 Linked to Families with Familial Idiopathic Scoliosis and Kyphosis

    PubMed Central

    Justice, Cristina M.; Bishop, Kevin; Carrington, Blake; Mullikin, Jim C.; Swindle, Kandice; Marosy, Beth; Sood, Raman; Miller, Nancy H.; Wilson, Alexander F.

    2016-01-01

    Because of genetic heterogeneity present in idiopathic scoliosis, we previously defined clinical subsets (a priori) from a sample of families with idiopathic scoliosis to find genes involved with spinal curvature. Previous genome-wide linkage analysis of seven families with at least two individuals with kyphoscoliosis found linkage (P-value = 0.002) in a 3.5-Mb region on 5p13.3 containing only three known genes, IRX1, IRX2, and IRX4. In this study, the exons of IRX1, IRX2, and IRX4, the conserved noncoding elements in the region, and the exons of a nonprotein coding RNA, LOC285577, were sequenced. No functional sequence variants were identified. An intrafamilial test of association found several associated noncoding single nucleotide variants. The strongest association was with rs12517904 (P = 0.00004), located 6.5 kb downstream from IRX1. In one family, the genotypes of nine variants differed from the reference allele in all individuals with kyphoscoliosis, and two of three individuals with scoliosis, but did not differ from the reference allele in all other genotyped individuals. One of these variants, rs117273909, was located in a conserved noncoding region that functions as an enhancer in mice. To test whether the variant allele at rs117273909 had an effect on enhancer activity, zebrafish transgenesis was performed with overlapping fragments of 198 and 687 bp containing either the wild type or the variant allele. Our data suggests that this region acts as a regulatory element; however, its size and target gene(s) need to be identified to determine its role in idiopathic scoliosis. PMID:27172222

  6. Evaluation of IRX Genes and Conserved Noncoding Elements in a Region on 5p13.3 Linked to Families with Familial Idiopathic Scoliosis and Kyphosis.

    PubMed

    Justice, Cristina M; Bishop, Kevin; Carrington, Blake; Mullikin, Jim C; Swindle, Kandice; Marosy, Beth; Sood, Raman; Miller, Nancy H; Wilson, Alexander F

    2016-01-01

    Because of genetic heterogeneity present in idiopathic scoliosis, we previously defined clinical subsets (a priori) from a sample of families with idiopathic scoliosis to find genes involved with spinal curvature. Previous genome-wide linkage analysis of seven families with at least two individuals with kyphoscoliosis found linkage (P-value = 0.002) in a 3.5-Mb region on 5p13.3 containing only three known genes, IRX1, IRX2, and IRX4 In this study, the exons of IRX1, IRX2, and IRX4, the conserved noncoding elements in the region, and the exons of a nonprotein coding RNA, LOC285577, were sequenced. No functional sequence variants were identified. An intrafamilial test of association found several associated noncoding single nucleotide variants. The strongest association was with rs12517904 (P = 0.00004), located 6.5 kb downstream from IRX1 In one family, the genotypes of nine variants differed from the reference allele in all individuals with kyphoscoliosis, and two of three individuals with scoliosis, but did not differ from the reference allele in all other genotyped individuals. One of these variants, rs117273909, was located in a conserved noncoding region that functions as an enhancer in mice. To test whether the variant allele at rs117273909 had an effect on enhancer activity, zebrafish transgenesis was performed with overlapping fragments of 198 and 687 bp containing either the wild type or the variant allele. Our data suggests that this region acts as a regulatory element; however, its size and target gene(s) need to be identified to determine its role in idiopathic scoliosis. PMID:27172222

  7. What Is Scoliosis?

    MedlinePlus

    ... causes of idiopathic scoliosis. They have studied genetics, growth, and changes in the spine, muscles, and nerves. They are studying how different treatments can help to straighten the spine or keep curves from getting worse. They are also studying the ...

  8. Conventional metrizamide myelography (MM) and computed tomographic metrizamide myelography (CTMM) in scoliosis: a comparative study

    SciTech Connect

    Pettersson, H.; Harwood-Nash, D.C.; Fitz, C.R.; Chuang, H.S.; Armstrong, E.

    1982-01-01

    A retrospective examination was performed to assess the accuracy of metrizamide myelography (MM) and computed tomographic metrizamide myelography (CTMM) in scoliosis. Of 81 consecutive scoliotic children studied by myelography, 30 had only MM while the remaining 51 had CTMM immediately afterward. CTMM added esential diagnostic information in 13 cases of dysraphism and 4 cases, both methods gave the same imformation. The outhors conclude that in patients with severe scoliosis, dysraphism, and scoliosis with localized neurological disturbances, CTMM should always be added to MM or be the only examination; while in idiopathic scoliosis with vague neurological disturbances a survey of the entire spine is essential, preferably with MM.

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

    PubMed Central

    2010-01-01

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

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

    PubMed

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

    2015-10-01

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

  11. Synthetic porous ceramic compared with autograft in scoliosis surgery. A prospective, randomized study of 341 patients.

    PubMed

    Ransford, A O; Morley, T; Edgar, M A; Webb, P; Passuti, N; Chopin, D; Morin, C; Michel, F; Garin, C; Pries, D

    1998-01-01

    We have evaluated the use of a synthetic porous ceramic (Triosite) as a substitute for bone graft in posterior spinal fusion for idiopathic scoliosis. In a prospective, randomised study 341 patients at five hospitals in the UK and France were randomly allocated either to autograft from the iliac crest or rib segments (171) or to receive Triosite blocks (170). All patients were assessed after operation and at 3, 6, 12 and 18 months. The two groups were similar with regard to all demographic and baseline variables, but the 184 treated in France (54%) had Cotrel-Dubouset instrumentation and the 157 treated in the UK usually had Harrington-Luque implants. In the Triosite group the average Cobb angle of the upper curve was 56 degrees, corrected to 24 degrees (57%). At 18 months, the average was 26 degrees (3% loss). In the autograft group the average preoperative upper curve of 53 degrees was corrected to 21 degrees (60%). At 18 months the mean curve was 25 degrees (8% loss). Pain levels after operation were similar in the two groups, being mild in most cases. In the Triosite group only three patients had problems of wound healing, but in the autograft group, 14 patients had delayed healing, infection or haematoma in the spinal wound. In addition, 15 autograft patients had pain at the donor site at three months. Seven had infections, two had haematoma and four had delayed healing. The haematological and serum biochemistry results showed no abnormal trends and no significant differences between the groups. There were no adverse events related to the graft material and no evidence of allergenicity. Our results suggest that Triosite synthetic porous ceramic is a safe and effective substitute for autograft in these patients. Histological findings on biopsy indicate that Triosite provides a favourable scaffolding for the formation of new bone and is gradually incorporated into the fusion mass. PMID:9460945

  12. Simple methods to reduce patient exposure during scoliosis radiography

    SciTech Connect

    Butler, P.F.; Thomas, A.W.; Thompson, W.E.; Wollerton, M.A.; Rachlin, J.A.

    1986-05-01

    Radiation exposure to the breasts of adolescent females can be reduced significantly through the use of one or all of the following methods: fast, rare-earth screen-film combinations; specially designed compensating filters; and breast shielding. The importance of exposure reduction during scoliosis radiography as well as further details on the above described methods are discussed. In addition, the early results of a Center for Devices and Radiological Health study, which recorded exposure and technique data for scoliosis radiography, is presented.

  13. Prevalence of scoliosis in Williams-Beuren syndrome patients treated at a regional reference center

    PubMed Central

    Damasceno, Marcelo Loquette; Cristante, Alexandre Fogaça; Marcon, Raphael Martus; de Barros Filho, Tarcísio Eloy Pessoa

    2014-01-01

    OBJECTIVE: This study assessed the prevalence of scoliosis and the patterns of scoliotic curves in patients with Williams-Beuren syndrome. Williams-Beuren syndrome is caused by a chromosome 7q11.23 deletion in a region containing 28 genes, with the gene encoding elastin situated approximately at the midpoint of the deletion. Mutation of the elastin gene leads to phenotypic changes in patients, including neurodevelopmental impairment of varying degrees, characteristic facies, cardiovascular abnormalities, hypercalcemia, urological dysfunctions, and bone and joint dysfunctions. METHODS: A total of 41 patients diagnosed with Williams-Beuren syndrome, who were followed up at the genetics ambulatory center of a large referral hospital, were included in the study. There were 25 male subjects. The patients were examined and submitted to radiographic investigation for Cobb angle calculation. RESULTS: It was observed that 14 patients had scoliosis; of these 14 patients, 10 were male. The pattern of deformity in younger patients was that of flexible and simple curves, although adults presented with double and triple curves. Statistical analysis showed no relationships between scoliosis and age or sex. CONCLUSION: This study revealed a prevalence of scoliosis in patients with Williams-Beuren syndrome of 34.1%; however, age and sex were not significantly associated with scoliosis or with the severity of the curves. PMID:25029575

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

    PubMed

    Caronni, Antonio; Zaina, Fabio; Negrini, Stefano

    2014-04-01

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

  15. Spinal curves and scoliosis.

    PubMed

    Anderson, Susan M

    2007-01-01

    Scoliosis, an abnormal side-to-side curve of the spine with associated vertebral rotation, affects as many as 4% of all adolescents. Several different categories of scoliosis exist, and treatment can range from observation and follow-up to bracing and surgical correction. This article discusses special imaging series for scoliosis and emphasizes the need for proper radiation protection techniques for patients with scoliosis, most of whom are girls in their early to mid-teens. PMID:17848532

  16. [Optimization of radiological scoliosis assessment].

    PubMed

    Enríquez, Goya; Piqueras, Joaquim; Catalá, Ana; Oliva, Glòria; Ruiz, Agustí; Ribas, Montserrat; Duran, Carmina; Rodrigo, Carlos; Rodríguez, Eugenia; Garriga, Victoria; Maristany, Teresa; García-Fontecha, César; Baños, Joan; Muchart, Jordi; Alava, Fernando

    2014-07-01

    Most scoliosis are idiopathic (80%) and occur more frequently in adolescent girls. Plain radiography is the imaging method of choice, both for the initial study and follow-up studies but has the disadvantage of using ionizing radiation. The breasts are exposed to x-ray along these repeated examinations. The authors present a range of recommendations in order to optimize radiographic exam technique for both conventional and digital x-ray settings to prevent unnecessary patients' radiation exposure and to reduce the risk of breast cancer in patients with scoliosis. With analogue systems, leaded breast protectors should always be used, and with any radiographic equipment, analog or digital radiography, the examination should be performed in postero-anterior projection and optimized low-dose techniques. The ALARA (as low as reasonable achievable) rule should always be followed to achieve diagnostic quality images with the lowest feasible dose. PMID:25128362

  17. Osteoporosis in unstable adult scoliosis

    SciTech Connect

    Velis, K.P.; Healey, J.H.; Schneider, R.

    1988-12-01

    New noninvasive techniques as well as conventional methods were used to evaluate skeletal mass in the following three populations of adult white women as follows: (1) 79 subjects with preexisting idiopathic scoliosis designated as unstable (US) because of the associated presence in the lumbar spine of lateral spondylolisthesis with segmental instability; (2) 67 subjects with preexisting idiopathic scoliosis without lateral spondylolisthesis designated as stable (SS); and (3) 248 age-matched nonscoliotic controls. Ages in all three groups were categorized into premenopausal (25-44 years), perimenopausal (45-54 years), and postmenopausal (55-84 years). The results showed higher scoliosis morbidity in the US compared to the SS populations. The prevalence and severity of osteoporosis were markedly increased in US versus SS populations. Femoral neck density determined by dual-photon absorptiometry techniques averaged 26% to 48% lower in all age categories of US patients compared to controls. These changes were found in the youngest age groups, indicating reductions in bone mineral content earlier in the adult life of white women with a specific type of high-morbidity US characterized by the marker of lateral spondylolisthesis.

  18. Bone mineral density evaluation among patients with neuromuscular scoliosis secondary to cerebral palsy☆

    PubMed Central

    Rezende, Rodrigo; Cardoso, Igor Machado; Leonel, Rayana Bomfim; Perim, Larissa Grobério Lopes; Oliveira, Tarcísio Guimarães Silva; Jacob Júnior, Charbel; Júnior, José Lucas Batista; Lourenço, Rafael Burgomeister

    2014-01-01

    Objective To evaluate bone mineral density among patients with neuromuscular scoliosis secondary to quadriplegic cerebral palsy. Methods This was a descriptive prospective study in which both bone densitometric and anthropometric data were evaluated. The inclusion criteria used were that the patients should present quadriplegic cerebral palsy, be confined to a wheelchair, be between 10 and 20 years of age and present neuromuscular scoliosis. Results We evaluated 31 patients (20 females) with a mean age of 14.2 years. Their mean biceps circumference, calf circumference and body mass index were 19.4 cm, 18.6 cm and 16.9 kg/m2, respectively. The mean standard deviation from bone densitometry was −3.2 (z-score), which characterizes osteoporosis. Conclusion There is high incidence of osteoporosis in patients with neuromuscular scoliosis secondary to quadriplegic cerebral palsy. PMID:26229882

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

    PubMed

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

    2010-01-01

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

  20. 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

  1. Surgical Correction of Scoliosis in Children with Spastic Quadriplegia: Benefits, Adverse Effects, and Patient Selection

    PubMed Central

    Legg, Julian; Davies, Evan; Raich, Annie L.; Dettori, Joseph R.; Sherry, Ned

    2014-01-01

    Study Rationale Cerebral palsy (CP) is a group of nonprogressive syndromes of posture and motor impairment associated with lesions of the immature brain. Spastic quadriplegia is the most severe form with a high incidence of scoliosis, back pain, respiratory compromise, pelvic obliquity, and poor sitting balance. Surgical stabilization of the spine is an effective technique for correcting deformity and restoring sitting posture. The decision to operate in this group of patients is challenging. Objectives The aim of this study is to determine the benefits of surgical correction of scoliosis in children with spastic quadriplegia, the adverse effects of this treatment, and what preoperative factors affect patient outcome after surgical correction. Materials and Methods A systematic review was undertaken to identify studies describing benefits and adverse effects of surgery in spastic quadriplegia. Factors affecting patient outcome following surgical correction of scoliosis were assessed. Studies involving adults and nonspastic quadriplegia were excluded. Results A total of 10 case series and 1 prospective and 3 retrospective cohort studies met inclusion criteria. There was significant variation in the overall risk of complications (range, 10.9−70.9%), mortality (range, 2.8−19%), respiratory/pulmonary complications (range, 26.9−57.1%), and infection (range, 2.5−56.8%). Factors associated with a worse outcome were a significant degree of thoracic kyphosis, days in the intensive care unit, and poor nutritional status. Conclusion Caregivers report a high degree of satisfaction with scoliosis surgery for children with spastic quadriplegia. There is limited evidence of preoperative factors that can predict patient outcome after scoliosis. There is a need for well-designed prospective studies of scoliosis surgery in spastic quadriplegia. PMID:24715871

  2. Congenital scoliosis: an up-to-date

    PubMed Central

    Burnei, G; Gavriliu, S; Vlad, C; Georgescu, I; Ghita, RA; Dughilă, C; Japie, EM; Onilă, A

    2015-01-01

    Congenital scoliosis represents a spinal malformation due to defects of formation, segmentation or mixed ones. It is characterized by a longitudinal and rotational imbalance. 54 patients were analyzed and 39 out of them were operated by various approaches with anterior and posterior instrumentations during 2000 and 2012. The impossibility to appoint some patients encountered in the daily practice into the known classifications, allowed us to purpose two categories of congenital scoliosis related to the predominance of spinal deviances in the coronal and transversal views. No certain etiology of congenital scoliosis has been identified until today. The susceptibility of some polygenic defects is obvious due to the presence of a sum of defects associated to most of the congenital scoliosis cases and the rarity of the presence of a unique defect. The diagnosis requires a thorough clinical and imaging examination in order to establish an individualized therapeutic strategy. The treatment of congenital scoliosis is different from the adolescent idiopathic one. Therapeutic criteria are significantly different. It is essential to assess the difference in growth of the concavity related to the convexity when choosing a particular procedure. The magnitude of the curve and the progressive rate are fundamental issues to the surgeon PMID:26351546

  3. [Conservative treatment of idiopathic scoliosis with effective braces: early response to trunk asymmetry may avoid curvature progress].

    PubMed

    Matussek, J; Dingeldey, E; Wagner, F; Rezai, G; Nahr, K

    2014-07-01

    Vertical posture of the growing child requires minute central nervous control mechanisms in order to maintain symmetry of the torso in its various activities. Scoliosis describes a constant deviation in the frontal, transverse and sagittal planes from the dynamic symmetry of the trunk. Early intervention with effective bracing, physiotherapy and sports can reverse curve progression during growth spurts, once these are identified in screening. Modern braces have a derotating and reducing effect (mirror effect) on asymmetric body volumes, thus influencing the growing torso and restoring lasting symmetry. Recent data support the use of braces to reverse progressing scoliosis. PMID:25028282

  4. The top view for analysis of scoliosis progression

    SciTech Connect

    De Smet, A.A.; Tarlton, M.A.; Cook, L.T.; Berridge, A.S.; Asher, M.A.

    1983-05-01

    Using computerized spinal analysis, a new top view was developed that displays the spine as if the observer were above and looking down on the patient. Serial top views were obtained of 12 patients with idiopathic scoliosis. In five patients with clinically stable curves, the top views showed no change. One patient with an enlaring rib hump was seen on the top view to have progressive kyphosis but stable scoliosis. Six patients with progressive scoliosis all demonstrated collapse of the thoracic curve in the anteroposterior direction. Five of these six patients had associated lumbar curves. Three lumbar curves demonstrated collapse in the anteroposterior direction similar to the collapse of the thoracic curves, and the other two curves appeared elongated in the anteroposterior direction.

  5. Surgical and conservative treatment of patients with congenital scoliosis: α search for long-term results

    PubMed Central

    2011-01-01

    Background In view of the limited data available on the conservative treatment of patients with congenital scoliosis (CS), early surgery is suggested in mild cases with formation failures. Patients with segmentation failures will not benefit from conservative treatment. The purpose of this review is to identify the mid- or long-term results of spinal fusion surgery in patients with congenital scoliosis. Methods Retrospective and prospective studies were included, reporting on the outcome of surgery in patients with congenital scoliosis. Studies concerning a small numbers of cases treated conservatively were included too. We analyzed mid-term (5 to 7 years) and long-term results (7 years or more), both as regards the maintenance of the correction of scoliosis and the safety of instrumentation, the early and late complications of surgery and their effect on quality of life. Results A small number of studies of surgically treated patients were found, contained follow-up periods of 4-6 years that in the most cases, skeletal maturity was not yet reached, and few with follow-up of 36-44 years. The results of bracing in children with congenital scoliosis, mainly in cases with failure of formation, were also studied. Discussion Spinal surgery in patients with congenital scoliosis is regarded in short as a safe procedure and should be performed. On the other hand, early and late complications are also described, concerning not only intraoperative and immediate postoperative problems, but also the safety and efficacy of the spinal instrumentation and the possibility of developing neurological disorders and the long-term effect these may have on both lung function and the quality of life of children. Conclusions Few cases indicate the long-term results of surgical techniques, in the natural progression of scoliosis. Similarly, few cases have been reported on the influence of conservative treatment. In conclusion, patients with segmentation failures should be treated

  6. Vertebral and Intraspinal Anomalies in Indian Population with Congenital Scoliosis: A Study of 119 Consecutive Patients

    PubMed Central

    S, Rajasekaran; G, Balamurali; Shetty, Ajoy

    2016-01-01

    Study Design Retrospective case study by clinical and radiological data analysis. Purpose To analyze different types of vertebral anomalies and the incidence of associated intraspinal anomalies in the Indian population. Overview of Literature This is the largest study of congenital scoliosis and associated intraspinal anomalies in Indian population. Incidence of intraspinal anomaly in this series is 47% which is higher than previous literature. Hemivertebra was the most common anomaly as seen in previous studies. Methods A total of 119 patients with congenital scoliosis who underwent surgery between December 2006 and December 2012 were studied. Data was reviewed with medical records, plain radiographs, and magnetic resonance imaging (MRI) scans. Results Thoracolumbar curve was most common, seen in 43.6% of patients. In addition to scoliotic deformity, kyphosis was seen in 26% of patients. Failure of formation, the most common vertebral anomaly, was seen in 51.2% of patients, failure of segmentation was seen in 19.3% of patients, and there were 29.4% patients having both formation and segmentation anomalies. Hemivertebra was the most common vertebral anomaly seen in 66.3% of patients and for whom 63.2% were in thoracic spine. Intraspinal anomalies were associated with 47% of patients with congenital scoliosis. Tethered cord was the most common intraspinal abnormality and was found in 48.2% patients with intraspinal anomalies. The patients with failure of segmentation and mixed deformities were found to have a significantly higher incidence of intraspinal anomalies (65% and 57%, respectively) than those with failure of formation (34%). Out of 31 patients with kyphotic deformity 29% had intraspinal anomalies, and amongst them tethered cord was the most common anomaly seen in 66% patients. Out of 12 patients with neurocutaneous markers, 83% patients had intraspinal anomaly. Conclusions Intraspinal anomalies were seen in 47% of patients with congenital scoliosis in the

  7. Scoliosis bracing and exercise for pain management in adults—a case report

    PubMed Central

    Weiss, Hans-Rudolf; Moramarco, Kathryn; Moramarco, Marc

    2016-01-01

    [Purpose] For adult patients with late-onset idiopathic scoliosis, cosmetic concerns and pain are the main reasons for seeking treatment at a physician’s office. The purpose of this paper was to describe the mid-term effect of physical rehabilitation and part-time bracing on an adult scoliosis patient who had been suffering from chronic low back pain for fourteen years. [Subject and Methods] Case description: A 37-year-old female patient with late-onset idiopathic scoliosis presented in the office of the first author in January 2014. She reported having chronic pain (low back pain) since the age of 23 and reported daily pain at a level of 5–7 on average on a Visual Analogue Scale of 0 to 10. She received a short scoliosis-specific Schroth exercise program and was also fitted with a Gensingen brace for part-time wear. [Results] At a 16 month follow-up, the patient no longer suffered from daily low back pain (with heavy lifting only) and was fully active. Additionally, her lumbar Cobb angle and angle of trunk rotation improved. [Conclusion] Patients with late-onset idiopathic scoliosis and pain may benefit from a pattern-specific conservative treatment approach. In this population, surgical intervention should be regarded as the last resort, since there are many long-term unknowns with surgery.

  8. Early-onset scoliosis: current treatment.

    PubMed

    Cunin, V

    2015-02-01

    Early-onset scoliosis, which appears before the age of 10, can be due to congenital vertebral anomalies, neuromuscular diseases, scoliosis-associated syndromes, or idiopathic causes. It can have serious consequences for lung development and significantly reduce the life expectancy compared to adolescent scoliosis. Extended posterior fusion must be avoided to prevent the crankshaft phenomenon, uneven growth of the trunk and especially restrictive lung disease. Conservative (non-surgical) treatment is used first. If this fails, fusionless surgery can be performed to delay the final fusion procedure until the patient is older. The gold standard delaying surgical treatment is the implantation of growing rods as described by Moe and colleagues in the mid-1980s. These rods, which are lengthened during short surgical procedures at regular intervals, curb the scoliosis progression until the patient reaches an age where fusion can be performed. Knowledge of this technique and its complications has led to several mechanical improvements being made, namely use of rods that can be distracted magnetically on an outpatient basis, without the need for anesthesia. Devices based on the same principle have been designed that preferentially attach to the ribs to specifically address chest wall and spine dysplasia. The second category of surgical devices consists of rods used to guide spinal growth that do not require repeated surgical procedures. The third type of fusionless surgical treatment involves slowing the growth of the scoliosis convexity to help reduce the Cobb angle. The indications are constantly changing. Improvements in surgical techniques and greater surgeon experience may help to reduce the number of complications and make this lengthy treatment acceptable to patients and their family. Long-term effects of surgery on the Cobb angle have not been compared to those involving conservative "delaying" treatments. Because the latter has fewer complications associated with

  9. Computer-aided optimal design of custom scoliosis braces considering clinical and patient evaluations.

    PubMed

    Visser, Daniel; Xue, Deyi; Ronsky, Janet L; Harder, James; Zernicke, Ronald F

    2012-09-01

    Scoliosis causes an abnormal three dimensional curvature of the spine that is often treated by an orthotic device called brace. The objective of this research was to develop a new approach to automatically identify the optimal design of custom-built brace, based on clinical and patient evaluations. In this approach, torso geometry of the scoliosis patient was achieved using a 3-D imaging system that generated a 3-D torso surface model, which was modified using a custom CAD system to design the 3-D brace surface model. Two design parameters, a translational correction factor and a rotational correction factor, were selected to design the brace geometry from the torso geometry. The 3-D digital brace was evaluated by three clinical evaluation measures (imbalance, rib hump and principal axis angle reduction) and one patient evaluation measure (discomfort). A multi-objective optimization method was employed to identify the optimal design parameters considering both clinical and patient evaluations. PMID:21257222

  10. The impact of patient self assessment of deformity on HRQL in adults with scoliosis

    PubMed Central

    Tones, Megan J; Moss, Nathan D

    2007-01-01

    Background Body image and HRQL are significant issues for patients with scoliosis due to cosmetic deformity, physical and psychological symptoms, and treatment factors. A selective review of scoliosis literature revealed that self report measures of body image and HRQL share unreliable correlations with radiographic measures and clinician recommendations for surgery. However, current body image and HRQL measures do not indicate which aspects of scoliosis deformity are the most distressing for patients. The WRVAS is an instrument designed to evaluate patient self assessment of deformity, and may show some promise in identifying aspects of deformity most troubling to patients. Previous research on adolescents with scoliosis supports the use of the WRVAS as a clinical tool, as the instrument shares strong correlations with radiographic measures and quality of life instruments. There has been limited use of this instrument on adult populations. Methods The WRVAS and the SF-36v2, a HRQL measure, were administered to 71 adults with scoliosis, along with a form to report age and gender. Preliminary validation analyses were performed on the WRVAS (floor and ceiling effects, internal consistency and collinearity, correlations with the SF-36v2, and multiple regression with the WRVAS total score as the predictor, and SF-36v2 scores as outcomes). Results The psychometric properties of the WRVAS were acceptable. Older participants perceived their deformities as more severe than younger participants. More severe deformities were associated with lower scores on the Physical Component Summary Score of the SF-36v2. Total WRVAS score also predicted Physical Component Summary scores. Conclusion The results of the current study indicate that the WRVAS is a reliable tool to use with adult patients, and that patient self assessment of deformity shared a relationship with physical rather than psychological aspects of HRQL. The current and previous studies concur that revision of the

  11. Gastric dysmotility following orthopaedic scoliosis surgery in patients with cerebral palsy: a case series.

    PubMed

    Vande Velde, S; Van Biervliet, S; De Bruyne, R; Van Renterghem, K; Plasschaert, F; Van Winckel, M

    2010-08-01

    Scoliosis is a common complication in children with cerebral palsy (CP). In these patients, surgical correction carries a high risk of complications. CP is also associated with gastrointestinal dysmotility such as delayed gastric emptying and gastro-oesophageal reflux. We describe 5 patients with CP in whom symptoms of gastric dysmotility clearly exacerbated after orthopaedic scoliosis surgery. They all showed persisting vomiting, nausea, bloating, weight loss, and anorexia necessitating total parental nutrition and/or jejunal feeding. This intensified nutritional support resulted in weight gain. Symptoms, however, persisted in half of the patients. The aetiology of these gastro-intestinal motility problems following scoliosis surgery remains unclear. Mechanical obstruction needs to be ruled out. Delayed gastric emptying may be due to postprandial antral hypomotility as a consequence of sympathic stimulation. Malnutrition could further aggravate gastrointestinal dysmotility. This complication should be taken into account when surgery for spinal deformities in CP patients is planned, especially in patients with pre-existing gastrointestinal motility problems. PMID:21086223

  12. Drug Fever Induced by Piperacillin/Tazobactam in a Scoliosis Patient: A Case Report.

    PubMed

    Li, Zheng; Shen, Jianxiong; Li, Qiyi; Chan, Matthew Tak Vai; Wu, William Ka Kei

    2015-11-01

    Drug fever is frequently underrecognized by clinicians despite its common occurrence. Fever induced by piperacillin/tazobactam has not been reported in scoliosis correction surgery.Drug fever caused by piperacillin/tazobactam in a scoliosis patient was described.A 36-year-old woman with adult scoliosis undergoing correction surgery was reported. She developed a fever after an intake of piperacillin/tazobactam for 3 days. Eosinophil count, erythrocyte sedimentation rate, and C-reactive proteins were increased in her blood examination. Thorough history, chest radiography, blood cultures, physical examination, and urinalysis revealed no evidences of fever. A drug fever is therefore considered. The fever lasted for 2 weeks and her body temperature come back to normal 4 days after piperacillin/tazobactam cessation.Fever could be caused by piperacillin/tazobactam. The drug fever's diagnosis is easily confounded by a co-occurring infection. Therefore, it is crucial for clinicians to doubt drugs as a reason when no other origin of fever could be identified in a patient. PMID:26579799

  13. Nonsurgical Management of Early-onset Scoliosis.

    PubMed

    Thorsness, Robert J; Faust, John R; Behrend, Caleb J; Sanders, James O

    2015-09-01

    Early-onset scoliosis is potentially fatal if left untreated. Although surgical management with growing instrumentation may be necessary, this is not a panacea and is associated with high complication rates. Recent evidence has demonstrated that nonsurgical treatment can be an effective early management strategy in delaying or even precluding the need for surgery, especially surgery with growing instrumentation. The goal of both nonsurgical and surgical management is to control or correct the spinal curve to allow appropriate pulmonary development while delaying definitive fusion until an appropriate skeletal age. Although more commonly used to delay surgery, serial cast correction using the Cotrel and Morel elongation-derotation-flexion technique may result in complete correction in patients with infantile idiopathic scoliosis and smaller curve magnitudes. PMID:26306805

  14. Physiotherapy scoliosis-specific exercises - a comprehensive review of seven major schools.

    PubMed

    Berdishevsky, Hagit; Lebel, Victoria Ashley; Bettany-Saltikov, Josette; Rigo, Manuel; Lebel, Andrea; Hennes, Axel; Romano, Michele; Białek, Marianna; M'hango, Andrzej; Betts, Tony; de Mauroy, Jean Claude; Durmala, Jacek

    2016-01-01

    In recent decades, there has been a call for change among all stakeholders involved in scoliosis management. Parents of children with scoliosis have complained about the so-called "wait and see" approach that far too many doctors use when evaluating children's scoliosis curves between 10° and 25°. Observation, Physiotherapy Scoliosis Specific Exercises (PSSE) and bracing for idiopathic scoliosis during growth are all therapeutic interventions accepted by the 2011 International Society on Scoliosis Orthopaedic and Rehabilitation Treatment (SOSORT). The standard features of these interventions are: 1) 3-dimension self-correction; 2) Training activities of daily living (ADL); and 3) Stabilization of the corrected posture. PSSE is part of a scoliosis care model that includes scoliosis specific education, scoliosis specific physical therapy exercises, observation or surveillance, psychological support and intervention, bracing and surgery. The model is oriented to the patient. Diagnosis and patient evaluation is essential in this model looking at a patient-oriented decision according to clinical experience, scientific evidence and patient's preference. Thus, specific exercises are not considered as an alternative to bracing or surgery but as a therapeutic intervention, which can be used alone or in combination with bracing or surgery according to individual indication. In the PSSE model it is recommended that the physical therapist work as part of a multidisciplinary team including the orthopeadic doctor, the orthotist, and the mental health care provider - all are according to the SOSORT guidelines and Scoliosis Research Society (SRS) philosophy. From clinical experiences, PSSE can temporarily stabilize progressive scoliosis curves during the secondary period of progression, more than a year after passing the peak of growth. In non-progressive scoliosis, the regular practice of PSSE could produce a temporary and significant reduction of the Cobb angle. PSSE can also

  15. Scoliosis (image)

    MedlinePlus

    ... the period of rapid growth. Most junior high schools routinely screen for scoliosis because, if caught early, progressive spine curvature can be prevented. Scoliosis affects girls much more frequently than boys.

  16. Scoliosis - resources

    MedlinePlus

    Resources - scoliosis ... The following organizations are good resources for information on scoliosis : American Academy of Orthopedic Surgeons -- orthoinfo.aaos.org/topic.cfm?topic=A00626 National Institute of Arthritis and ...

  17. Scoliosis (image)

    MedlinePlus

    Abnormal curvature in the spine is known as scoliosis, and generally begins just at the onset of ... growth. Most junior high schools routinely screen for scoliosis because, if caught early, progressive spine curvature can ...

  18. Intraoperative Neurophysiologic Monitoring (INM) in scoliosis surgery.

    PubMed

    Pankowski, Rafal; Dziegiel, Krzysztof; Roclawski, Marek; Smoczynski, Andrzej; Ceynowa, Marcin; Kloc, Wojciech; Wasilewski, Wojciech; Jende, Piotr; Liczbik, Wieslaw; Beldzinski, Piotr; Libionka, Witold; Pierzak, Olaf; Adamski, Stanislaw; Niedbala, Miroslaw

    2012-01-01

    Even among skilled spinal deformity surgeons, neurologic deficits are inherent potential complications of spine surgery. The aim was to assess the meaning of changes and to evaluate the critical rates of Somatosensory Evoked Potentials (SEP) and Motor Evoked Potentials (MEP) for Neurologic Deficit (ND) occurrence associated with scoliosis surgery. A Group of 30 patients with idiopathic scoliosis treated surgically by posterior correction and stabilisation were included. Patients were matched by age, sex, aetiology, Cobb angle, and surgical criteria. Data on three planar scoliosis correction and concomitant (INM) alarms were compared. Radiographic assessment was performed from radiographs taken before surgery and just after it. The (INM) was performed with the use of ISSIS (Inomed) in every patients the same fashion. The average thoracic curve correction was 69.7% and lumbar 69.8%. The average preoperative Apical Vertebral Rotation was 23.5° for thoracic and 27.9° for lumbar curves and postoperatively 10.9° and 14.3° respectively. There was a significant variability of SEP during surgery with only 7 (23%) patients with stable SEP. 15(50%) patients had a decrease of SEP below 50% and 8(27%) had severe decrease of SEP below 50% what caused us to stop surgery or to decrease correction of curves. There was a MEP decrease in 11(37%) patients and in 6 (20%) directly after correction up to 50% of normal value. In 5 of 30 (17%) patients there was a significant decrease of MEP below 50% and we immediately released the implant. The SEP decrease up to 50% without any MEP change did not influenced the outcome. There was no correlation between flexibility and correction of the curve and SEP and MEP decrease. The safe level for MEP was not determined but its meaning for the outcome was more important than SEP value. The need of (INM) during scoliosis surgery to avoid (ND) was confirmed. PMID:22744519

  19. [Transient tracheal obstruction during surgical correction of scoliosis in a patient with Marfan's syndrome].

    PubMed

    Kai, Y; Yamaoka, A; Irita, K; Zaitsu, A; Takahashi, S

    1995-06-01

    A 13-yr-old male with Marfan's syndrome underwent surgical correction of severe scoliosis. He had not manifested dyspnea previously in any position. Under anesthesia with thiamylal and vecuronium, his trachea was intubated with a reinforced endotracheal tube without any difficulty. Anesthesia was maintained with nitrous oxide and fentanyl, 8 micrograms.kg-1. The patient was placed in a prone position. Thirty min after the start of operation, when orthopedists compressed the thoracic vertebrae vertically, positive pressure ventilation became impossible abruptly, even with a high airway pressure. Three min later, ventilation became possible after cessation of compression and by mouth-to-tube insufflation. SpO2 monitored with a pulse oximeter recovered immediately from 61% to 99%. A capnogram showed a lengthy retardation of an inspiratory phase. Emergency fibreoptic bronchoscopy revealed that the trachea had been compressed vertically; the compression was reduced by moving the chest supporters laterally. After the apneic episode, the operation continued uneventfully, and he was discharged a month later. A severe deformity of the thorax due to severe scoliosis and weak tracheal tissue due to connective tissue defect caused partial tracheal compression before the surgery, and made his trachea susceptible to complete obstruction by vertical external compression on the thorax. Patients with Marfan's syndrome and scoliosis should have careful preoperative airway evaluation. The selection and positioning of endotracheal tubes should be done with care. During surgery, the patient's body position and the condition of the trachea should be checked frequently. Capnography and fiberoptic bronchoscopy seem to be mandatory for early detection of tracheal stenosis and prevention of tracheal obstruction. PMID:7637168

  20. Scoliosis curve type classification using kernel machine from 3D trunk image

    NASA Astrophysics Data System (ADS)

    Adankon, Mathias M.; Dansereau, Jean; Parent, Stefan; Labelle, Hubert; Cheriet, Farida

    2012-03-01

    Adolescent idiopathic scoliosis (AIS) is a deformity of the spine manifested by asymmetry and deformities of the external surface of the trunk. Classification of scoliosis deformities according to curve type is used to plan management of scoliosis patients. Currently, scoliosis curve type is determined based on X-ray exam. However, cumulative exposure to X-rays radiation significantly increases the risk for certain cancer. In this paper, we propose a robust system that can classify the scoliosis curve type from non invasive acquisition of 3D trunk surface of the patients. The 3D image of the trunk is divided into patches and local geometric descriptors characterizing the surface of the back are computed from each patch and forming the features. We perform the reduction of the dimensionality by using Principal Component Analysis and 53 components were retained. In this work a multi-class classifier is built with Least-squares support vector machine (LS-SVM) which is a kernel classifier. For this study, a new kernel was designed in order to achieve a robust classifier in comparison with polynomial and Gaussian kernel. The proposed system was validated using data of 103 patients with different scoliosis curve types diagnosed and classified by an orthopedic surgeon from the X-ray images. The average rate of successful classification was 93.3% with a better rate of prediction for the major thoracic and lumbar/thoracolumbar types.

  1. The effect of Swiss ball exercise and resistance exercise on balancing ability of scoliosis patients

    PubMed Central

    Song, Gui Bin; Kim, Jwa Jun; Park, Eun Cho

    2015-01-01

    [Purpose] The purpose of the study was to closely examine the efficiency of Swiss ball exercise and resistance exercise in improving the on the static balancing ability by applying them to patients with scoliosis and to compare the effects of the interventions. [Subjects] Forty scoliosis patients were divided into a Swiss ball exercise group (SEG, N = 20) and a Resistance exercise group (REG, N = 20) randomly. [Methods] SEG conducted chest stretching, trunk exercise using the Swiss ball. REG conducted chest stretching, trunk exercise with therapist’s resistance. Both groups received training 30 min per day, five times per week, for eight weeks. [Results] Both SEG and REG showed significant differences between pre- and post-mediation in terms of weight distribution, sway area, sway length, sway speed, and limit of stability. Sway speed and limit of stability had increased more significantly in REG than in SEG. [Conclusion] According to the result of this study, both Swiss ball exercise and chest Resistance exercise were effective for improving on the static balancing ability. But we suggest resistance exercise is more efficient to increase of sway speed, limit of stability. PMID:26834372

  2. The effect of Swiss ball exercise and resistance exercise on balancing ability of scoliosis patients.

    PubMed

    Song, Gui Bin; Kim, Jwa Jun; Park, Eun Cho

    2015-12-01

    [Purpose] The purpose of the study was to closely examine the efficiency of Swiss ball exercise and resistance exercise in improving the on the static balancing ability by applying them to patients with scoliosis and to compare the effects of the interventions. [Subjects] Forty scoliosis patients were divided into a Swiss ball exercise group (SEG, N = 20) and a Resistance exercise group (REG, N = 20) randomly. [Methods] SEG conducted chest stretching, trunk exercise using the Swiss ball. REG conducted chest stretching, trunk exercise with therapist's resistance. Both groups received training 30 min per day, five times per week, for eight weeks. [Results] Both SEG and REG showed significant differences between pre- and post-mediation in terms of weight distribution, sway area, sway length, sway speed, and limit of stability. Sway speed and limit of stability had increased more significantly in REG than in SEG. [Conclusion] According to the result of this study, both Swiss ball exercise and chest Resistance exercise were effective for improving on the static balancing ability. But we suggest resistance exercise is more efficient to increase of sway speed, limit of stability. PMID:26834372

  3. 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

  4. Scoliosis may be the first symptom of the tethered spinal cord

    PubMed Central

    Barutçuoğlu, Mustafa; Selçuki, Mehmet; Umur, Ahmet Sukru; Mete, Mesut; Gurgen, Seren Gulsen; Selcuki, Deniz

    2016-01-01

    Background: Tethered cord syndrome (TCS) is a progressive clinical entity that arises from abnormal spinal cord tension. Scoliosis may be a unique symptom in TCS. The aim of this study is to investigate prognosis after releasing the filum terminale in scoliosis due to TCS with/without findings in magnetic resonance imaging (MRI) and to draw attention to the importance of somatosensorial evoked potentials (SSEP) on the differential diagnosis of idiopathic scoliosis versus scoliosis due to TCS with normal appearance of filum terminale and conus medullaris. Materials and Methods: Eleven female and seven male patients with progressive scoliosis were included in the study. They were evaluated radiologically, SSEP and urodynamical studies. Preoperative and postoperative anteroposterior full spine X-rays were obtained for measuring the Cobb's angle. MRI was performed in all cases for probable additional spinal abnormalities. All patients underwent filum terminale sectioning through a L5 hemilaminectomy. The resected filum terminale were subjected to histopathological examination. Results: The mean Cobb angle was 31.6° (range 18°–45°). Eight patients (44.45%) had a normal appearance of filum terminale and normal level conus medullaris in MRI, but conduction delay and/or block was seen on SSEP. In the histopathological examination of filum terminale dense collagen fibers, hyaline degeneration and loss of elastic fibers were observed. Postoperatively none of the patients showed worsening of the Cobb angle. Three patients showed improvement of scoliosis. Conclusion: In TCS presented with scoliosis, untethering must be performed prior to the corrective spinal surgery. Absence of MRI findings does not definitely exclude TCS. SSEP is an important additional guidance in the diagnosis of TCS. After untethering, a followup period of 6 months is essential to show it untethering helps in stopping the progress of the scoliotic curve. In spite of non progression (curve stopped

  5. Trends of Posterior Long Segment Fusion with and without Recombinant Human Bone Morphogenetic Protein 2 in Patients with Scoliosis

    PubMed Central

    Ruofeng, Yin; Cohen, Jeremiah R.; Buser, Zorica; Yoon, S. Tim; Meisel, Hans-Joerg; Youssef, Jim A.; Park, Jong-Beom; Wang, Jeffrey C.; Brodke, Darrel S.

    2015-01-01

    Study Design  Retrospective study. Objective  Symptomatic scoliosis can be a source of severe pain and disability. When nonoperative treatments fail, spine fusion is considered as an effective procedure in scoliosis management. The purpose of this study was to evaluate the trends of patients with scoliosis undergoing posterior long segment fusion (PLSF) with and without recombinant human bone morphogenetic protein 2 (rhBMP-2). Methods  Patients within the orthopedic subset of Medicare database undergoing PLSF from 2005 to 2011 were identified using the PearlDiver Patient Records Database. Both diagnosis and procedural International Classification of Diseases, ninth edition and Current Procedural Terminology codes were used. The year of procedure, age, sex, region, and rhBMP-2 use were recorded. Results  In total, 1,265,591 patients with scoliosis were identified with 29,787 PLSF surgeries between 2005 and 2011. The incidence of PLSF procedures increased gradually from 2005 to 2009, decreased in 2010 (p < 0 0.01), and grew again in 2011. Patients over age 84 years had the highest incidence of PLSF. The lowest incidence of the procedures was in the Northeast, 5.96 per 100,000 patients. Sex differences were observed with a male-to-female ratio of 0.40 (p < 0.01). The use of rhBMP-2 for PLSF increased steadily from 2005 to 2009; the numbers dropped dramatically in 2010 and returned by 2011. Conclusions  According to our study, patients with scoliosis demonstrated a 0.6575 average incidence increase of PLSF treatments annually. There were significant differences in incidence of PLSF procedure and patient demographics. Additionally, rhBMP-2 consumption significantly changed when we stratified it by sex, age, and region respectively. PMID:27433425

  6. 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

  7. 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

  8. Effect of growing rod on sagittal and spinopelvic parameters in early-onset scoliosis patients.

    PubMed

    Sariyilmaz, Kerim; Akgul, Turgut; Ozkunt, Okan; Dikici, Fatih; Korkmaz, Murat; Sar, Cuneyt; Domanic, Unsal

    2016-05-01

    Growing rod is a commonly used surgery for early-onset scoliosis (EOS). However, the effect of growing-rod lengthening on the spinopelvic alignment is unclear. In this study, 21 EOS patients treated by growing rod were evaluated retrospectively and thoracic kyphosis (TK), lumbar lordosis (LL), pelvic incidence (PI) , sacral slope (SS), pelvic tilt (PT), and sagittal vertical axis (SVA) were measured. Preoperatively, the mean TK, LL, PI, PT, SS, and SVA were 27.4°, 35.2°, 43.8°, 7.5°, 33.8°, and 47.7 mm respectively. After the last lengthening, TK, LL, PI, PT, SS, and SVA were 28.3°, 28.06°, 41.4°, 7°, 5.2°, and 42.6 mm, respectively. The sagittal plane parameters in our EOS patients were not significantly altered during the lengthening period. PMID:27007546

  9. Circulating antimyenteric autoantibodies in Tunisian patients with idiopathic achalasia.

    PubMed

    Kallel-Sellami, M; Karoui, S; Romdhane, H; Laadhar, L; Serghini, M; Boubaker, J; Lahmar, H; Filali, A; Makni, S

    2013-01-01

    The physiopathology of idiopathic achalasia is still unknown. The description of circulating antimyenteric autoantibodies (CAA), directed against enteric neurons in sera of patients, suggests an autoimmune process. Recent data showed controversies according to the existence and the significance of CAA. The aims of this study were to investigate whether CAA are detected in Tunisian patients with idiopathic achalasia and to look for associated clinical or manometrical factors with CAA positivity. Twenty-seven patients with idiopathic achalasia and 57 healthy controls were prospectively studied. CAA were assessed by indirect immunofluorescence on intestinal monkey tissue sections. Western blot on primate cerebellum protein extract and dot technique with highly purified recombinant neuronal antigens (Hu, Ri, and Yo) were further used to analyze target antigens of CAA. CAA were significantly increased in achalasia patients compared with controls when considering nuclear or cytoplasmic fluorescence patterns. (33% vs. 12%, P = 0.03 and 48% vs. 23%, P = 0.001 respectively). By immunoblot analysis, CAA did not target neuronal antigens, however 52/53 and 49 kDa bands were consistently detected. CAA positivity was not correlated to specific clinical features. The results are along with previous studies demonstrating high CAA prevalence in achalasia patients. When reviewing technical protocols and interpretation criteria, several discrepancies which could explain controversies between studies were noted. PMID:22947106

  10. Resting energy expenditure and nutritional state of patients with increased oxygen cost of breathing due to emphysema, scoliosis and thoracoplasty.

    PubMed Central

    Sridhar, M. K.; Carter, R.; Lean, M. E.; Banham, S. W.

    1994-01-01

    BACKGROUND--Weight loss is a well recognised feature of patients with emphysematous chronic obstructive pulmonary disease (COPD). It has been suggested that this weight loss could be due to a hypermetabolic state resulting from the increased oxygen cost of breathing (OCB). To clarify the relation between resting energy expenditure (REE), nutritional state, and OCB these indices were measured in patients with respiratory impairment and an increased OCB due to COPD, scoliosis, and thoracoplasty. METHODS--Eighteen patients (six COPD, six scoliosis, six thoracoplasty) of mean (SD) age 59.9 (8.6) years (8M, 10F) and six controls (45.5 (9.9) years; 2M, 4F) were studied. OCB was estimated by the addition of dead space to the breathing circuit and REE was measured by indirect calorimetry using a ventilated canopy system. Height, arm span, weight, triceps skin fold thickness (TSF), mid-arm muscle circumference (MAMC), forced expiratory volume in one second (FEV1), and vital capacity (VC) were measured in all study subjects. RESULTS--OCB was elevated in all patient groups (mean 7.0 ml/l) compared with controls (1.9 ml/l). All patients with COPD, four with scoliosis, three with thoracoplasty, and none of the controls were < 90% ideal body weight. Mean (SD) measured REE as % predicted (Harris-Benedict equation) was 103.8 (7.6) in patients with COPD, 105.5 (10.9) in those with scoliosis, 106.3 (6.9) in the thoracoplasty patients, and 103.3 (3.4) in controls. One patient with COPD, two with scoliosis, two with thoracoplasty, but no controls were hypermetabolic (REE > 110% predicted). In all groups there was a negative relation between OCB and lung function (OCB v FEV1 r = -0.83 in COPD, -0.62 in scoliosis, -0.67 in thoracoplasty, and -0.76 in controls). There was no correlation between REE and OCB or MAMC. CONCLUSIONS--In patients with respiratory disease OCB (augmented ventilation) is related to lung function but not to REE. This is evidence against the hypothesis that

  11. Contemporary Outcome in Patients With Idiopathic Dilated Cardiomyopathy.

    PubMed

    Broch, Kaspar; Murbræch, Klaus; Andreassen, Arne Kristian; Hopp, Einar; Aakhus, Svend; Gullestad, Lars

    2015-09-15

    Outcome is better in patients with idiopathic dilated cardiomyopathy (IDC) than in ischemic heart failure (HF), but morbidity and mortality are nevertheless presumed to be substantial. Most data on the prognosis in IDC stem from research performed before the widespread use of current evidence-based treatment, including implantable devices. We report outcome data from a cohort of patients with IDC treated according to current HF guidelines and compare our results with previous figures: 102 consecutive patients referred to our tertiary care hospital with idiopathic IDC and a left ventricular ejection fraction <40% were included in a prospective cohort study. After extensive baseline work-up, follow-up was performed after 6 and 13 months. Vital status and heart transplantation were recorded. Over the first year of follow-up, the patients were on optimal pharmacological treatment, and 24 patients received implantable devices. Left ventricular ejection fraction increased from 26 ± 10% to 41 ± 11%, peak oxygen consumption increased from 19.5 ± 7.1 to 23.4 ± 7.8 ml/kg/min, and functional class improved substantially (all p values <0.001). After a median follow-up of 3.6 years, 4 patients were dead, and heart transplantation had been performed in 9 patients. According to our literature search, survival in patients with IDC has improved substantially over the last decades. In conclusion, patients with IDC have a better outcome than previously reported when treated according to current guidelines. PMID:26233575

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

    PubMed Central

    2013-01-01

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

  13. SU-E-I-15: Comparison of Radiation Dose for Radiography and EOS in Adolescent Scoliosis Patients

    SciTech Connect

    Schueler, B; Walz-Flannigan, A

    2014-06-01

    Purpose: To estimate patient radiation dose for whole spine imaging using EOS, a new biplanar slot-scanning radiographic system and compare with standard scoliosis radiography. Methods: The EOS imaging system (EOS Imaging, Paris, France) consists of two orthogonal x-ray fan beams which simultaneously acquire frontal and lateral projection images of a standing patient. The patient entrance skin air kerma was measured for each projection image using manufacturer-recommended exposure parameters for spine imaging. Organ and effective doses were estimated using a commercially-available Monte Carlo simulation program (PCXMC, STUK, Radiation and Nuclear Safety Authority, Helsinki, Finland) for a 15 year old mathematical phantom model. These results were compared to organ and effective dose estimated for scoliosis radiography using computed radiography (CR) with standard exposure parameters obtained from a survey of pediatric radiographic projections. Results: The entrance skin air kerma for EOS was found to be 0.18 mGy and 0.33 mGy for posterior-anterior (PA) and lateral projections, respectively. This compares to 0.76 mGy and 1.4 mGy for CR, PA and lateral projections. Effective dose for EOS (PA and lateral projections combined) is 0.19 mSv compared to 0.51 mSv for CR. Conclusion: The EOS slot-scanning radiographic system allows for reduced patient radiation dose in scoliosis patients as compared to standard CR radiography.

  14. Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

    PubMed Central

    Hoeper, Marius M.; Behr, Juergen; Held, Matthias; Grunig, Ekkehard; Vizza, C. Dario; Vonk-Noordegraaf, Anton; Lange, Tobias J.; Claussen, Martin; Grohé, Christian; Klose, Hans; Olsson, Karen M.; Zelniker, Thomas; Neurohr, Claus; Distler, Oliver; Wirtz, Hubert; Opitz, Christian; Huscher, Doerte; Pittrow, David; Gibbs, J. Simon R.

    2015-01-01

    Background Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies. Methods We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP). Results Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP. Conclusions Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival. Trial Registration clinicaltrials.gov NCT01347216 PMID:26630396

  15. Effects of Swiss ball exercise and resistance exercise on respiratory function and trunk control ability in patients with scoliosis

    PubMed Central

    Kim, Jwa Jun; Song, Gui Bin; Park, Eun Cho

    2015-01-01

    [Purpose] This study compared the effects of Swiss ball exercise and resistance exercise on the respiratory function and trunk control ability of patients with scoliosis. [Subjects] Forty scoliosis patients were randomly divided into the Swiss ball exercise group (n= 20) and resistance exercise group (n = 20). [Methods] The Swiss ball and resistance exercise groups performed chest expansion and breathing exercises with a Swiss ball and a therapist’s resistance, respectively. Both groups received training 30 min per day, 5 times per week for 8 weeks. [Results] Both groups exhibited significant changes in forced vital capacity, forced expiratory volume in one second, and trunk impairment scale after the intervention. However, there was no significant change in the forced expiratory volume in one second/forced vital capacity ratio after the intervention in either group. Meanwhile, forced expiratory volume in one second and trunk impairment scale were significantly greater in the resistance exercise group after the intervention. [Conclusion] Both Swiss ball exercise and resistance exercise are effective for improving the respiratory function and trunk control ability of patients with scoliosis. However, resistance exercise is more effective for increasing the forced expiratory volume in one second and trunk control ability. PMID:26180318

  16. Effects of Swiss ball exercise and resistance exercise on respiratory function and trunk control ability in patients with scoliosis.

    PubMed

    Kim, Jwa Jun; Song, Gui Bin; Park, Eun Cho

    2015-06-01

    [Purpose] This study compared the effects of Swiss ball exercise and resistance exercise on the respiratory function and trunk control ability of patients with scoliosis. [Subjects] Forty scoliosis patients were randomly divided into the Swiss ball exercise group (n= 20) and resistance exercise group (n = 20). [Methods] The Swiss ball and resistance exercise groups performed chest expansion and breathing exercises with a Swiss ball and a therapist's resistance, respectively. Both groups received training 30 min per day, 5 times per week for 8 weeks. [Results] Both groups exhibited significant changes in forced vital capacity, forced expiratory volume in one second, and trunk impairment scale after the intervention. However, there was no significant change in the forced expiratory volume in one second/forced vital capacity ratio after the intervention in either group. Meanwhile, forced expiratory volume in one second and trunk impairment scale were significantly greater in the resistance exercise group after the intervention. [Conclusion] Both Swiss ball exercise and resistance exercise are effective for improving the respiratory function and trunk control ability of patients with scoliosis. However, resistance exercise is more effective for increasing the forced expiratory volume in one second and trunk control ability. PMID:26180318

  17. Neuromuscular Scoliosis

    MedlinePlus

    ... degree of neuromuscular involvement. Diagnosis Incidence of Scoliosis Cerebral palsy (2 limbs involved) 25% Myelodysplasia (lower lumbar) 60% Spinal muscle atrophy 67% Friedreich ataxia 80% Cerebral palsy (4 limbs involved) 80% Duchenne muscular dystrophy 90% ...

  18. Surgical management of severe scoliosis with high risk pulmonary dysfunction in Duchenne muscular dystrophy: patient function, quality of life and satisfaction

    PubMed Central

    Nakazawa, Toshiyuki; Imura, Takayuki; Okada, Takamitsu; Fukushima, Kensuke; Ueno, Masaki; Takahira, Naonobu; Takahashi, Kazuhisa; Yamazaki, Masashi; Ohtori, Seiji; Okamoto, Hirotsugu; Okutomi, Toshiyuki; Okamoto, Makihito; Masaki, Takashi; Uchinuma, Eijyu; Sakagami, Hiroyuki

    2010-01-01

    In a previous study, the authors reported the clinical and radiological results of Duchenne muscular dystrophy (DMD) scoliosis surgery in 14 patients with a low FVC of <30%. The purpose of this study was to determine if surgery improved function and QOL in these patients. Furthermore, the authors assessed the patients’ and parents’ satisfaction. %FVC increased in all patients after preoperative inspiratory muscle training. Scoliosis surgery in this group of patients presented no increased risk of major complications. All-screw constructions and fusion offered the ability to correct spinal deformity in the coronal and pelvic obliquity initially, intermediate and long-term. All patients were encouraged to continue inspiratory muscle training after surgery. The mean rate of %FVC decline after surgery was 3.6% per year. Most patients and parents believed scoliosis surgery improved their function, sitting balance and quality of life even though patients were at high risk for major complications. Their satisfaction was also high. PMID:20155495

  19. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients

    PubMed Central

    Nagasaki, Joji; Manabe, Masahiro; Ido, Kentaro; Ichihara, Hiroyoshi; Aoyama, Yasutaka; Ohta, Tadanobu; Furukawa, Yoshio; Mugitani, Atsuko

    2016-01-01

    The etiologies of secondary idiopathic thrombocytopenic purpura (ITP) include infection, autoimmune disease, and immunodeficiency. We report the cases of three elderly patients who developed ITP after receiving influenza vaccinations. The platelet count of an 81-year-old woman fell to 27,000/μL after she received an influenza vaccination. A 75-year-old woman developed thrombocytopenia (5,000 platelets/μL) after receiving an influenza vaccination. An 87-year-old woman whose laboratory test values included a platelet count of 2,000/μL experienced genital bleeding after receiving an influenza vaccination. After Helicobacter pylori (HP) eradication or corticosteroid treatment, all of the patients' platelet counts increased. Influenza vaccination is an underlying etiology of ITP in elderly patients. HP eradication or corticosteroid treatment is effective for these patients. Clinicians should be aware of the association between ITP and influenza vaccinations. PMID:26998369

  20. Temperament and character profiles of patients with chronic idiopathic urticaria

    PubMed Central

    Canan, Fatih; Karakaş, Ayşe Akman; Geçici, Nihal

    2015-01-01

    Introduction Psychosocial factors have been implicated as being important in the onset and/or exacerbation of urticaria. Aim To examine both personality factors of patients with chronic idiopathic urticaria (CIU) and the correlations between illness duration, severity of itching, urticaria activity score (UAS) and temperament-character dimensions. Material and methods A total number of 70 CIU patients and 60 healthy individuals were included in the study. The Temperament and Character Inventory (TCI) was administered individually. The relationship between UAS, illness duration and severity of pruritus and TCI subscales were evaluated. Results The CIU group had significantly higher scores of novelty seeking and lower scores of cooperativeness, reward dependence and self-directedness than the control group. Conclusions The current study shows that CIU patients have distinctive temperament and character dimensions when compared with the control group. We suggest that evaluation and treatment of CIU should also include psychosomatic approaches in clinical practice. PMID:26161056

  1. Portosystemic Shunt Surgery in Patients with Idiopathic Noncirrhotic Portal Hypertension.

    PubMed

    Karagul, Servet; Yagci, Mehmet Ali; Tardu, Ali; Ertugrul, Ismail; Kirmizi, Serdar; Sumer, Fatih; Isik, Burak; Kayaalp, Cuneyt; Yilmaz, Sezai

    2016-01-01

    BACKGROUND Idiopathic noncirrhotic portal hypertension (INCPH) is a rare disease characterized by increased portal venous pressure in the absence of cirrhosis and other causes of liver diseases. The aim of the present study was to present our results in using portosystemic shunt surgery in patients with INCPH. MATERIAL AND METHODS Patients who had been referred to our Liver Transplantation Institute for liver transplantation and who had undergone surgery from January 2010 to December 2015 were retrospectively analyzed. Patients with INCPH who had undergone portosystemic shunt procedure were included in the study. Age, sex, symptoms and findings, type of portosystemic shunt, and postoperative complications were assessed. RESULTS A total of 1307 patients underwent liver transplantation from January 2010 to December 2015. Eleven patients with INCPH who did not require liver transplantation were successfully operated on with a portosystemic shunt procedure. The mean follow-up was 30.1±19 months (range 7-69 months). There was no mortality in the perioperative period or during the follow-up. Two patients underwent surgery again due to intra-abdominal hemorrhage; one had bleeding from the surgical site except the portacaval anastomosis and the other had bleeding from the h-graft anastomosis. No patient developed encephalopathy and no patient presented with esophageal variceal bleeding after portosystemic shunt surgery. Shunt thrombosis occurred in 1 patient (9.9%). Only 1 patient developed ascites, which was controlled medically. CONCLUSIONS Portosystemic shunt surgery is a safe and effective procedure for the treatment of patients with INCPH. PMID:27194018

  2. Effect of medical x-ray exposure on subsequent reproductive outcomes in scoliosis patients

    SciTech Connect

    Visscher, W.A.

    1987-01-01

    A retrospective cohort study was done which was designed to assess the effects of medical x-ray exposure on cancer incidence among scoliosis patients. Although the primary purpose of the study was to assess cancer incidence, a secondary goal was to investigate whether diagnostic x-ray exposure is related to adverse reproductive events in the female subjects. A series of case-control analyses were done which were designed to assess these effects. Radiation exposure was measured both by total films received and by an estimate of the number of films received and by an estimate of the number of films which involved ovarian irradiation. Radiation appeared to increase a woman's risk of any adverse event in the overall analysis and her risk of a premature or low birth weight infant in the separate analyses. Radiation did not appear to be related to spontaneous abortion, complications of pregnancy or delivery or birth defects, although the results of the pregnancy complications analysis was suggestive.

  3. Increased CD40+ fibrocytes in patients with idiopathic orbital inflammation

    PubMed Central

    Lee, Brian J; Atkins, Stephen; Ginter, Anna; Elner, Victor M; Nelson, Christine C; Douglas, Raymond S

    2014-01-01

    Objective To investigate the phenotypic and functional characteristics of peripheral and tissue-infiltrating stem cells, called fibrocytes in patients with idiopathic orbital inflammation (IOI). Methods Seven patients with IOI were studied. In the three patients requiring orbital biopsy, fibrocytes were identified in orbital tissue from patients with IOI compared to healthy controls using immunohistochemistry. Fibrocytes from the peripheral blood of all seven patients and controls were quantified and phenotyped by flow cytometry and immunofluorescence for expression of CD34, alpha smooth muscle actin, CD40 and Collagen 1. Quantitation of CD40-mediated IL-6 production was measured using ELISA. Results Orbital biopsy specimens from patients with IOI demonstrate tissue infiltration by fibrocytes (n=3). Fibrocytes are present in the peripheral blood of IOI patients (n= 7) but are scarce in healthy donors (n=19). Fibrocytes from IOI patients express substantial levels of CD40 and ligation of CD40 increases IL-6 expression. Conclusions Fibrocytes are present in the peripheral blood and orbital tissues of patients with IOI and constitutively express CD40 and express IL-6 in response to ligation. This site-specific predilection of CD34+ fibrocytes to sites of orbital inflammation and fibrosis may suggest a role in IOI. Moreover CD40-mediated activation cytokine production may contribute to the proinflammatory and profibrotic features of IOI and may provide a mechanism for future targeted therapy. PMID:25098443

  4. Quantifying torso deformity in scoliosis

    NASA Astrophysics Data System (ADS)

    Ajemba, Peter O.; Kumar, Anish; Durdle, Nelson G.; Raso, V. James

    2006-03-01

    Scoliosis affects the alignment of the spine and the shape of the torso. Most scoliosis patients and their families are more concerned about the effect of scoliosis on the torso than its effect on the spine. There is a need to develop robust techniques for quantifying torso deformity based on full torso scans. In this paper, deformation indices obtained from orthogonal maps of full torso scans are used to quantify torso deformity in scoliosis. 'Orthogonal maps' are obtained by applying orthogonal transforms to 3D surface maps. (An 'orthogonal transform' maps a cylindrical coordinate system to a Cartesian coordinate system.) The technique was tested on 361 deformed computer models of the human torso and on 22 scans of volunteers (8 normal and 14 scoliosis). Deformation indices from the orthogonal maps correctly classified up to 95% of the volunteers with a specificity of 1.00 and a sensitivity of 0.91. In addition to classifying scoliosis, the system gives a visual representation of the entire torso in one view and is viable for use in a clinical environment for managing scoliosis.

  5. 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

  6. Incidence of herpes zoster infections in juvenile idiopathic arthritis patients.

    PubMed

    Nimmrich, S; Horneff, G

    2015-03-01

    The risk of herpes zoster among patients with juvenile idiopathic arthritis (JIA) exposed to biologics has not been evaluated. We determined incidence rates of herpes zoster among children with JIA in correlation with medication at time of occurrence and total drug exposure. The German biologics register database was used to identify patients with herpes zoster. Crude infection rates and incidence ratios (IRR) were compared to published rates. Demographics and overall exposure and particular exposure time to corticosteroids, immunosuppressive drugs and biologics were analyzed. The JIA cohort included 3,042 patients with 5,557.9 person-years of follow-up; 1,628 have used corticosteroids, 2,930 methotrexate and 1,685 etanercept. In total, 17 herpes zoster events have been documented [6/1,000 patients (3.5-9.0); 3.1/1,000 patient-years (1.9-4.9)]. Thus, the incidence rate in JIA patients was higher than expected [IRR 2.9 (1.8-4.5), p < 0.001]. In all patients, the event resolved completely. There were two complications, one patient developed intercostal neuralgia, and one had a recurrent herpes zoster. Compared to the healthy population, a significant higher IRR is observed in JIA patients who received a monotherapy with etanercept or in combination with steroids and methotrexate, but not in JIA patients exposed to methotrexate without biologics. In comparison with our control group of patients treated with methotrexate, the IRR was higher for exposure to etanercept monotherapy and combination of etanercept and corticosteroids irrespective of methotrexate use. A generally higher incidence rate in JIA patients treated with etanercept was observed. No serious or refractory manifestations occurred. PMID:25583050

  7. Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review.

    PubMed

    Raghu, Ganesh; Amatto, Valeria C; Behr, Jürgen; Stowasser, Susanne

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is associated with a fatal prognosis and manifests in patients over 60 years old who may have comorbidities. The prevalence and impact of comorbidities on the clinical course of IPF is unclear.This systematic literature review examined the prevalence of comorbidities and mortality associated with comorbidities in IPF patients. Relevant observational studies published in English from January 1990 to January 2015 identified via MEDLINE and EMBASE were included; bibliographies of articles were also searched.Among the 126 studies included, prevalence of pulmonary hypertension (PH) was 3-86%, 6-91% for obstructive sleep apnoea, 3-48% for lung cancer and 6-67% for chronic obstructive pulmonary disease (COPD). Nonrespiratory comorbidities included ischaemic heart disease (IHD) (3-68%) and gastro-oesophageal reflux (GER) (0-94%). Mortality was highest among patients with IPF and lung cancer. Most studies assessed relatively small samples of patients with IPF.PH, COPD, lung cancer, GER and IHD are significant comorbidities; differences in IPF severity, case definitions and patient characteristics limited the comparability of findings. The identification and prompt treatment of comorbidities may have a clinically significant impact on overall outcome that is meaningful for patients with IPF. PMID:26424523

  8. Scoliosis secondary to ganglioneuroma: a case report and up to date literature review.

    PubMed

    D'Eufemia, Patrizia; Properzi, Enrico; Palombaro, Marta; Lodato, Valentina; Mellino, Loretta; Tetti, Martina; Martini, Lorena; Persiani, Pietro

    2014-07-01

    Idiopathic scoliosis is the most common form of spinal deformity in children. However, secondary causes of scoliosis, such as ganglioneuroma, should be always considered to avoid wrong diagnosis, and further investigations are required when there are atypical signs. We report a case of ganglioneuroma misdiagnosed as idiopathic scoliosis and review the literature to identify the red flags useful for physicians during the evaluation of a child with scoliosis. On the basis of both clinical and radiographic criteria that emerged from this study, we propose an algorithm that could help in the differential diagnosis, suggesting when to perform an MRI. PMID:24681491

  9. Voxel-based morphometry in patients with idiopathic generalized epilepsies.

    PubMed

    Betting, Luiz Eduardo; Mory, Susana Barreto; Li, Li Min; Lopes-Cendes, Iscia; Guerreiro, Marilisa M; Guerreiro, Carlos A M; Cendes, Fernando

    2006-08-15

    Idiopathic generalized epilepsies (IGE) are a group of frequent age-related epilepsy syndromes. IGE are clinically characterized by generalized tonic-clonic, myoclonic and absence seizures. According to predominant seizure type and age of onset, IGE are divided in subsyndromes: childhood absence and juvenile absence epilepsy (AE), juvenile myoclonic epilepsy (JME) and generalized tonic-clonic seizures on awakening (GTCS). The limits between these subsyndromes are not well defined, supporting the existence of only one major syndrome. Visual assessment of routine magnetic resonance imaging (MRI) in patients with IGE is normal. MRI voxel-based morphometry (VBM) uses automatically segmented gray and white matter for comparisons, eliminating the investigator bias. We used VBM to study 120 individuals (47 controls, 44 with JME, 24 with AE and 15 with GTCS) to investigate the presence of subtle structural abnormalities in IGE subsyndromes. VBM was performed searching for abnormalities on gray matter concentration (GMC) between patients groups and controls. Compared to controls, JME presented increased GMC in frontobasal region and AE showed increased GMC in the superior mesiofrontal region. The GTCS group did not differ from controls. There were no areas of reduced GMC with the statistical level selected. Region of interest analysis showed increased GMC in the anterior portion of the thalamus in patients with absence seizures. Our results support subtle GMC abnormalities in patients with JME and AE when compared to controls. These findings suggest the existence of different patterns of cortical abnormalities in IGE subsyndromes. PMID:16702001

  10. Idiopathic pancreatitis in a patient with a STAT3 mutation

    PubMed Central

    Peppers, Brian; Frith, John; Tcheurekdjian, Haig; Hostoffer, Robert

    2016-01-01

    Background: Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent skin infections with abscesses, recurrent pneumonias with pneumatoceles, and immunoglobulin E levels of >10 times the upper limit of normal. Case: The patient described herein had a classic case of signal transducer and activator of transcription 3 (STAT3) deficiency associated with HIES diagnosed several years before this particular presentation. He demonstrated extraimmune manifestations of the disease as well, including characteristic facies and a history of skeletal fractures. In addition, the patient had several distinct episodes of idiopathic pancreatitis for which a full gastrointestinal workup had been performed. STAT3 mutation was confirmed by genotyping at the time of diagnosis of HIES. Conclusions: STAT3, a mammalian protein that regulates cell growth, survival, and differentiation, has been linked to human pancreatic carcinogenesis as well as the above-mentioned immune deficiency. Mouse studies demonstrated that genetic ablation of STAT3 exacerbates the course of acute pancreatitis, whereas normal pancreatic STAT3 seems to have a protective effect against necrotizing pancreatitis. An association between STAT3 mutations and pancreatitis has not yet been revealed in humans. Here we describe a case of acute pancreatitis that presented in a patient with STAT3 mutation. PMID:27103560

  11. Decompression alone versus decompression with limited fusion for treatment of degenerative lumbar scoliosis in the elderly patient

    PubMed Central

    Daubs, Michael D.; Lenke, Lawrence G.; Bridwell, Keith H.; Cheh, Gene; Kim, Yongjung J.; Stobbs, Georgia

    2012-01-01

    Study design: Retrospective cohort study. Objective: To analyze the surgical results of a group of patients older than 65 years treated for mild degenerative lumbar scoliosis (<30°) with stenosis, treated with decompression alone or decompression and limited fusion. Methods: We evaluated 55 patients, all older than 65 years from our prospectively collected database with mild degenerative scoliosis (<30°) and stenosis who underwent surgery. Laminectomy alone was performed in 16 patients, and laminectomy and limited fusion in 39 patients. Mean follow-up was 4.6 years in the decompression group and 5.0 years in the fusion group. Clinical results were graded by patients' self-reported satisfaction and length of symptom-free period to recurrence. Results: In the decompression alone group, 6 (37%) of 16 patients developed recurrent stenosis at the previously decompressed level and five developed recurrence within 6 months postoperatively versus the decompression and fusion group where 3 (8%) of 39 (P = .0476) developed symptomatic stenosis supra adjacent to the fusion. Of 16 patients in the decompression alone group, 12 (75%) had recurrence of symptoms by the 5-year follow-up period versus only 14 (36%) patients in the decompression and fusion group (P = .016). Adjacent segment degenerative changes were common in the fusion group, but only 7% developed symptomatic stenosis. Conclusions: Decompression with limited fusion prevents early return of stenotic symptoms compared with decompression alone in the setting of mild degenerative scoliosis (<30°) and symptomatic stenosis in patients 65 years and older. Final class of evidence–prognosis Study design  RCT  Cohort •  Case control  Case series Methods  Concealed allocation (RCT)  Intention to treat (RCT)  Blinded/independent evaluation of primary outcome  F/U ≥ 85% •  Adequate sample size  Control for confounding Overall class of evidence III The definiton of the different classes of

  12. Vitreous estrogen levels in patients with an idiopathic macular hole

    PubMed Central

    Inokuchi, Naoki; Ikeda, Tsunehiko; Nakamura, Kimitoshi; Morishita, Seita; Fukumoto, Masanori; Kida, Teruyo; Oku, Hidehiro

    2015-01-01

    Purpose Estrogen, a female hormone, activates collagenase and might be associated with the pathogenesis of vitreoretinal collagen fiber disease. The purpose of the present study was to investigate the vitreous levels of estrone (E1) and estradiol (E2) in subjects with an idiopathic macular hole (IMH). Methods Vitreous samples were obtained from ten female patients with an IMH and from nine female patients with other retinal diseases (six with rhegmatogenous retinal detachment and three with age-related macular degeneration) as a control at the time of vitreous surgery. E1 and E2 levels in the vitreous samples were then determined using the Coat-A-Count® Estradiol Radioimmunoassay (RIA) Kit and the DSL-70 Estrone RIA Kit, respectively. Results The mean vitreous levels of E1 and E2 in the subjects with IMH were 1.83±2.00 pg/mL and 7.03±2.97 pg/mL, respectively, whereas in the control subjects they were 2.42±1.25 pg/mL and 4.90±2.90 pg/mL, respectively. Thus, the vitreous E2 levels in the subjects with IMH were significantly higher than in the controls (P<0.05). Conclusion The findings of this study suggest that E2 might be associated with the pathogenesis of IMH, but further investigation is needed to elucidate that association. PMID:25848205

  13. [Anesthetic Management of a Patient with Central Core Disease for Scoliosis Surgery].

    PubMed

    Jimi, Nobuo; Izumi, Kaoru; Sumiyoshi, Rieko; Mizuno, Keiichiro

    2016-06-01

    Central core disease (CCD) is a dominantly inherited congenital myopathy. CCD is also associated with muscular and skeletal abnormalities such as abnormal curvature of the spine (scoliosis), hip dislocation, and joint deformities. CCD and malignant hyperthermia (MH) are both associated with mutations in the ryanodine receptor on chromosome 19q13.1. An 11-year-old boy with CCD complicated with severe scoliosis was scheduled for spinal fusion surgery under general anesthesia. Furthermore, he had trismus caused by temporomandibular contracture. He was considered as MH susceptible. Anesthesia was managed with remifentanil and propofol without using muscle relaxtants and volatile anesthetics. We could intubate the trachea with Airtraq laryngoscope without any complications. The perioperative course was uneventful. PMID:27483668

  14. Orthodontic treatment in patient with idiopathic root resorption: a case report.

    PubMed

    Rey, Diego; Smit, Rosana Martínez; Gamboa, Liliana

    2015-01-01

    Multiple idiopathic external root resorption is a rare pathological condition usually detected as an incidental radiographic finding. External root resorption of permanent teeth is a multifactorial process related to several local and systemic factors. If an etiological factor cannot be identified for root resorption, the term "idiopathic" is applied. This report presents a case of multiple idiopathic apical root resorption. The condition was found in a young female patient seeking orthodontic treatment due to malocclusion. This kind of resorption starts apically and progresses coronally, causing a gradual shortening and rounding of the remaining root. Patients with this condition are not the ideal candidates for orthodontic treatment; however, the aim of this report is to describe an unusual case of idiopathic root resorption involving the entire dentition, and to present the orthodontic treatment of this patient. It describes the progress and completion of orthodontic therapy with satisfactory end results. PMID:25741832

  15. [Successful pregnancy in a patient with idiopathic pulmonary arterial hypertension. Case report].

    PubMed

    Szenczi, Orsolya; Karlócai, Kristóf; Bucsek, László; Rigó, János

    2016-04-10

    Idiopathic pulmonary arterial hypertension is characterized by progressive increase in pulmonary arterial pressure and pulmonary vascular resistance which lead to right ventricular failure and death. Pregnancy in patients with idiopathic pulmonary arterial hypertension is contraindicated because of the high maternal and fetal mortality. The authors present a case of successful pregnancy and delivery of a patient with idiopathic pulmonary arterial hypertension in Hungary for the first time. The aim of the report was to demonstrate that management and treatment of idiopathic pulmonary arterial hypertension in a pregnant woman is a complex and multidisciplinary task that should involve obstetrician, cardiologist and anesthesiologist. Those patients who become pregnant and do not wish to terminate the pregnancy must be referred to obstetric centers where a multidiciplinary approach is taken. PMID:27039998

  16. Orthodontic treatment in patient with idiopathic root resorption: A case report

    PubMed Central

    Rey, Diego; Smit, Rosana Martínez; Gamboa, Liliana

    2015-01-01

    Multiple idiopathic external root resorption is a rare pathological condition usually detected as an incidental radiographic finding. External root resorption of permanent teeth is a multifactorial process related to several local and systemic factors. If an etiological factor cannot be identified for root resorption, the term "idiopathic" is applied. This report presents a case of multiple idiopathic apical root resorption. The condition was found in a young female patient seeking orthodontic treatment due to malocclusion. This kind of resorption starts apically and progresses coronally, causing a gradual shortening and rounding of the remaining root. Patients with this condition are not the ideal candidates for orthodontic treatment; however, the aim of this report is to describe an unusual case of idiopathic root resorption involving the entire dentition, and to present the orthodontic treatment of this patient. It describes the progress and completion of orthodontic therapy with satisfactory end results. PMID:25741832

  17. SOSORT 2012 consensus paper: reducing x-ray exposure in pediatric patients with scoliosis

    PubMed Central

    2014-01-01

    This 2012 Consensus paper reviews the literature on side effects of x-ray exposure in the pediatric population as it relates to scoliosis evaluation and treatment. Alternative methods of spinal assessment and imaging are reviewed, and strategies for reducing the number of radiographs are developed. Using the Delphi technique, SOSORT members developed consensus statements that describe how often radiographs should be taken in each of the pediatric and adolescent sub-populations. PMID:24782912

  18. Radiographic Parameters in Adult Degenerative Scoliosis and Different Parameters Between Sagittal Balanced and Imbalanced ADS Patients

    PubMed Central

    Yang, Changwei; Yang, Mingyuan; Chen, Yuanyuan; Wei, Xianzhao; Ni, Haijian; Chen, Ziqiang; Li, Jingfeng; Bai, Yushu; Zhu, Xiaodong; Li, Ming

    2015-01-01

    Abstract A retrospective study. To summarize and describe the radiographic parameters of adult degenerative scoliosis (ADS) and explore the radiological parameters which are significantly different in sagittal balanced and imbalanced ADS patients. ADS is the most common type of adult spinal deformity. However, no comprehensive description of radiographic parameters in ADS patients has been made, and few studies have been performed to explore which radiological parameters are significantly different between sagittal balanced and imbalanced ADS patients. Medical records of ADS patients in our outpatient clinic from January 2012 to January 2014 were reviewed. Demographic data including age and sex, and radiographic data including the coronal Cobb angle, location of apical vertebra/disc, convexity of the curve, degree of apical vertebra rotation, curve segments, thoracic kyphosis (TK), lumbar lordosis (LL), thoracolumbar kyphosis (TL), sacral slope (SS), pelvic tilt (PT), pelvic incidence (PI), sagittal vertical axis (SVA), and PI minus LL (PI − LL) were reviewed to make comprehensive description of radiographic parameters of ADS. Furthermore, patients were divided into 2 groups according to whether the patients’ sagittal plane was balanced: Group A (imbalanced, SVA > 5 cm) and Group B (balanced, SVA ≤ 5 cm). Demographic and radiological parameters were compared between these 2 groups. A total of 99 patients were included in this study (Group A = 33 and Group B = 66; female = 83 and male = 16; sex ratio = 5:1). The median of age were 67 years (range: 41–92 years). The median of coronal Cobb angle and length of curve was 23 (range: 10–75°) and 5 segments (range: 3–7), respectively. The most common location of apical vertebra was at L2 to L3 (81%) and the median of degree of apical vertebra rotation was 2° (range: 1–3). Our study also showed significant correlations between coronal Cobb angle and curve segments (r

  19. Scoliosis in Herlyn–Werner–Wunderlich Syndrome

    PubMed Central

    Li, Zheng; Yu, Xin; Shen, Jianxiong; Liang, Jinqian

    2014-01-01

    Abstract Herlyn–Werner–Wunderlich syndrome (HWWS) is a congenital Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Little is reported about spinal deformity associated with this syndrome. This study presents a case of scoliosis occurring in the setting of HWWS and explores the possible association between the 2 diseases. A previously unreported scoliosis in HWWS is described. The patient is a 12-year-old Chinese female with scoliosis that underwent a posterior correction at thoracic 5–thoracic 12 (T5–T12) levels, using the Moss-SI (Johnson & Johnson, American) spinal system. At 24-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. Six months after scoliosis correction surgery, the patient went to our clinics for the treatment of HWWS. She was performed a vaginal septum resection and detected with pyocolpos. Her follow-up was symptom free at the fourth postoperative month. The prevalence of scoliosis among patients with HWWS was 8.57% that is much higher than the incidence of congential scoliosis among general population (1/1000). To the best of our knowledge, this is the first report of HWWS with thoracic scoliosis. During surgery, surgeons and anesthesiologists must pay particular attention to the Müllerian duct anomaly and renal agenesis associated with HWWS. There is a potential association between congenital scoliosis and HWWS. PMID:25526433

  20. Exercise therapy for patients with diffuse idiopathic skeletal hyperostosis.

    PubMed

    Al-Herz, Adeeba; Snip, Jan Paul; Clark, Bruce; Esdaile, John M

    2008-02-01

    We evaluated the effect of exercise therapy on back pain, spinal range of motion (ROM), and disability in persons with diffuse idiopathic skeletal hyperostosis (DISH). Persons with symptomatic DISH received a daily exercise program for 24 weeks consisting of mobility, stretching, and strengthening exercises for the cervical, thoracic, and lumbar spine. It included 14 supervised sessions over 8 weeks. Outcomes included visual analogue scales (VAS) for pain, stiffness, and fatigue, 13 spinal measurements, the neck pain and disability scale, the Quebec back pain disability scale, the Bath Spondylitis Functional Index, and the MACTAR patient preference scale. Assessments were made at baseline, 8 weeks, and 24 weeks. Fifteen of 17 completed the study. Comparing week 24 with baseline, Schober's test improved significantly (p = 0.02), and VAS stiffness and left finger-to-floor test demonstrated a trend to improvement (p = 0.07 each). The physical measures, which were expected to improve with the exercise program, all moved in the direction expected, but had p values > 0.10. At 24 weeks, eight (53.3%) participants rated their status as improved, three (20%) as unchanged, and four (27%) were unsure about the benefit. The exercise program designed for DISH and tested in this study led to small improvements in physical measures which achieved significance only for lumbosacral flexion. PMID:17885726

  1. Congenital Scoliosis (Mini-review).

    PubMed

    Weiss, Hans-Rudolf; Moramarco, Marc

    2016-01-01

    Congenital scoliosis is a lateral deformity of the spine with a disturbance of the sagittal profile caused by malformations of vertebra and ribs. Typically, early surgical intervention is the suggested treatment (before three-years-old) for young patients with congenital scoliosis. While a previous study was conducted in 2011 to investigate long-term studies supporting the necessity for this recommendation and no evidence was found, this current review, is an updated search for evidence published from 2011 through March 2015. This also failed to find any prospective or randomized controlled studies to support the hypothesis that spinal fusion surgery in patients with congenital scoliosis should be considered as evidence-based treatment. Contradictory results exist on the safety of hemivertebra resection and segmental fusion using pedicle screw fixation. When using the VEPTR (vertical expandable prosthetic titanium rib) device, studies show a high rate of complications exist. It is difficult to predict the final outcome for patients with congenital scoliosis. However, it is possible that many patients with congenital scoliosis may be able to avoid spinal surgery with the application of advanced bracing technology. Therefore, it is only prudent to advocate for conservative management first before spinal surgery is considered. PMID:26769614

  2. Congenital scoliosis treated with posterior vertebral column resection in patients younger than 18 years: longer than 10-year follow-up.

    PubMed

    Chang, Dong-Gune; Yang, Jae Hyuk; Lee, Jung-Hee; Kim, Jin-Hyok; Suh, Seung-Woo; Ha, Kee-Yong; Suk, Se-Il

    2016-08-01

    OBJECTIVE There have been no reports on the long-term radiographic outcomes of posterior vertebral column resection (PVCR) in patients with congenital scoliosis. The purpose of this study was to evaluate the surgical outcomes and complications after PVCR and its long-term effects on correcting this deformity in children with congenital scoliosis. METHODS The authors retrospectively analyzed the medical records of 45 patients with congenital scoliosis who were younger than 18 years at the time of surgery and who underwent PVCR and fusion with pedicle screw fixation (PSF). The mean age of the patients at the time of surgery was 11.3 years (range 2.4-18.0 years), and the mean length of follow-up was 12.8 years (range 10.1-18.2 years). RESULTS The mean Cobb angle of the main curve was 46.5° before PVCR, 13.7° immediately after PVCR, and 17.6° at the last follow-up. For the compensatory cranial curve, PVCR corrected the preoperative Cobb angle of 21.2° to 9.1° postoperatively and maintained it at 10.9° at the last follow-up. For the compensatory caudal curve, the preoperative Cobb angle of 23.8° improved to 7.7° postoperatively and was 9.8° at the last follow-up. The authors noted 22 complications, and the overall incidence of complications was 48.9%. CONCLUSIONS Posterior vertebral column resection is an effective procedure for managing congenital scoliosis in patients younger than 18 years. Use of PVCR and fusion with PSF for congenital scoliosis achieved rigid fixation and satisfactory deformity correction that was maintained over the long term. However, the authors note that PVCR is a technically demanding procedure and entails risks for major complications and excessive blood loss. PMID:26967991

  3. Development of CAD/CAM Based Brace Models for the Treatment of Patients with Scoliosis-Classification Based Approach versus Finite Element Modelling

    PubMed Central

    Kleban, Alexander

    2015-01-01

    Study Design Retrospective controlled cohort study comparing the in-brace correction of two samples of scoliosis patients with braces of different computer aided design (CAD). Purpose In-brace correction and compliance correlate with outcome. The more standardized CAD braces that are available should enable improved in-brace correction and outcome. This study compared recent CAD brace developments with respect to in-brace corrections. Overview of Literature A 2013 randomized controlled trial demonstrated that 72% of a population complying to Scoliosis Research Society inclusion criteria on bracing did not progress using braces (mainly Boston braces) used in the United States and Canada with moderate corrective effect. Methods In-brace corrections achieved in a sample of patients fulfilling the inclusion criteria for studies on bracing using the classification based approach (CBA) were compared to the recent individual CAD/computer aided manufacturing bracing based on finite element modelling approach (FEMA). Results In-brace corrections using the different approaches differed widely. CBA in-brace corrections were 66% of the initial value. FEMA in-brace correction was 42% of the initial value. Conclusions Considering the fact that in-brace correction (and compliance) determines the end result of bracing in the treatment of scoliosis, scoliosis braces based on CBA are superior to the FEMA and the standard plaster based brace applications. PMID:26435781

  4. Sexual maturation in Moroccan patients with juvenile idiopathic arthritis.

    PubMed

    El Badri, D; Rostom, S; Bouaddi, I; Hassani, A; Chkirate, B; Amine, B; Hajjaj-Hassouni, N

    2014-05-01

    Abnormal puberty is often reported in children suffering from many chronic diseases. Juvenile idiopathic arthritis (JIA) is the most common joint disorder in developing children. The aim of this study was to assess sexual maturation of Moroccan children with JIA and to compare the development of secondary sexual characteristics in children with JIA to children in the general population. Forty children with JIA and 74 healthy controls 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. Every child was examined for the development of genitalia as per criteria given by Tanner. The children with JIA were also divided into 3 groups: pre-puberty (stage 1), puberty (stages 2-3) and post-puberty (stage 4-5), and the association between puberty and cumulative dose of steroids, disease duration, disease activity, height, weight and age was investigated. Forty children with JIA were included (22 male, 18 female); the mean of age of the patients was 11 ± 4.23 years. Puberty in the patients (mean of tanner 2.43 ± 1.36) was lower than controls (2.55 ± 1.36). The prevalence of the children in prepuberty was of 15 (37.5 %) and 8 (20 %) in postpuberty. The prevalence of the children having a delayed puberty was of 6 (15 %) versus 1(1.4 %) in healthy controls (p = 0.005). There was an association between dose of corticosteroids, age at the administration of corticosteroids and the delayed puberty in boys (p = 0.009). In addition, there was no significant association in both sex between this poor puberty and duration of JIA (p = 0.45 in boys and p = 1.99 in girls) and its activity calculated by the DAS28 (p = 0.73 in boys and p = 1). Our study suggests that the puberty is retarded in Moroccan patients with JIA comparing to healthy children and that the dose of corticosteroid and the age at its administration may contribute to delayed puberty in boys

  5. Determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis undergoing lung transplant evaluation.

    PubMed

    Porteous, Mary K; Rivera-Lebron, Belinda N; Kreider, Maryl; Lee, James; Kawut, Steven M

    2016-03-01

    Little is known about the physiologic determinants of 6-minute walk distance in idiopathic pulmonary fibrosis. We investigated the demographic, pulmonary function, echocardiographic, and hemodynamic determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis evaluated for lung transplantation. We performed a cross-sectional analysis of 130 patients with idiopathic pulmonary fibrosis who completed a lung transplantation evaluation at the Hospital of the University of Pennsylvania between 2005 and 2010. Multivariable linear regression analysis was used to generate an explanatory model for 6-minute walk distance. After adjustment for age, sex, race, height, and weight, the presence of right ventricular dilation was associated with a decrease of 50.9 m (95% confidence interval [CI], 8.4-93.3) in 6-minute walk distance ([Formula: see text]). For each 200-mL reduction in forced vital capacity, the walk distance decreased by 15.0 m (95% CI, 9.0-21.1; [Formula: see text]). For every increase of 1 Wood unit in pulmonary vascular resistance, the walk distance decreased by 17.3 m (95% CI, 5.1-29.5; [Formula: see text]). Six-minute walk distance in idiopathic pulmonary fibrosis depends in part on circulatory impairment and the degree of restrictive lung disease. Future trials that target right ventricular morphology, pulmonary vascular resistance, and forced vital capacity may potentially improve exercise capacity in patients with idiopathic pulmonary fibrosis. PMID:27076905

  6. Determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis undergoing lung transplant evaluation

    PubMed Central

    Rivera-Lebron, Belinda N.; Kreider, Maryl; Lee, James; Kawut, Steven M.

    2016-01-01

    Abstract Little is known about the physiologic determinants of 6-minute walk distance in idiopathic pulmonary fibrosis. We investigated the demographic, pulmonary function, echocardiographic, and hemodynamic determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis evaluated for lung transplantation. We performed a cross-sectional analysis of 130 patients with idiopathic pulmonary fibrosis who completed a lung transplantation evaluation at the Hospital of the University of Pennsylvania between 2005 and 2010. Multivariable linear regression analysis was used to generate an explanatory model for 6-minute walk distance. After adjustment for age, sex, race, height, and weight, the presence of right ventricular dilation was associated with a decrease of 50.9 m (95% confidence interval [CI], 8.4–93.3) in 6-minute walk distance (P=0.02). For each 200-mL reduction in forced vital capacity, the walk distance decreased by 15.0 m (95% CI, 9.0–21.1; P<0.001). For every increase of 1 Wood unit in pulmonary vascular resistance, the walk distance decreased by 17.3 m (95% CI, 5.1–29.5; P=0.006). Six-minute walk distance in idiopathic pulmonary fibrosis depends in part on circulatory impairment and the degree of restrictive lung disease. Future trials that target right ventricular morphology, pulmonary vascular resistance, and forced vital capacity may potentially improve exercise capacity in patients with idiopathic pulmonary fibrosis. PMID:27076905

  7. Risk of Idiopathic Dilated Cardiomyopathy in 29 000 Patients With Celiac Disease

    PubMed Central

    Emilsson, Louise; Andersson, Bert; Elfström, Peter; Green, Peter H.R.; Ludvigsson, Jonas F.

    2012-01-01

    Background Dilated cardiomyopathy (DCM) is a rare disease of largely unknown origin. Previous studies have suggested an increased prevalence of celiac disease (CD) in patients with DCM. These studies, however, were based on a maximum of 5 patients with both CD and DCM. In the present large Swedish population-based cohort study, we examined the risk of idiopathic DCM in patients with CD determined by small-intestinal histopathology. Methods and Results From 2006 to 2008, we collected duodenal/jejunal biopsy data on CD (equal to villous atrophy, Marsh stage 3, n=29 071 unique individuals) from (all) 28 pathology departments in Sweden. These individuals were compared with 144 429 reference individuals matched for age, sex, calendar year, and county. Data on DCM were obtained through the National Patient Register and confirmed by patient charts and echocardiography data. During follow-up, 17 patients with CD and 52 reference individuals developed idiopathic DCM. Thus, patients with CD were at an increased risk of idiopathic DCM (hazard ratio, 1.73; 95% confidence interval, 1.00 to 3.00), although the risk estimate failed to attain statistical significance (P=0.052). Conclusion This nationwide study found a moderately but not statistically significantly increased risk of idiopathic DCM in patients with biopsy-verified CD. (J Am Heart Assoc. 2012;1:e001594 doi: 10.1161/JAHA.112.001594.) PMID:23130142

  8. Imaging in childhood scoliosis: a pictorial review.

    PubMed

    Musson, Rachel E; Warren, Daniel J; Bickle, Ian; Connolly, Daniel J; Griffiths, Paul D

    2010-07-01

    Childhood scoliosis is a common clinical entity with a number of different causes. In the majority of cases, the scoliosis is idiopathic, but it may be the manifestation of an occult spinal pathology. The clinical history and examination may elicit certain worrying features such as pain, neurological symptoms or an atypical curve pattern. These findings should prompt advanced imaging, as early and accurate detection of an underlying cause allows optimal planning and timing of surgery and helps reduce associated risks. The most common occult pathologies detected by advanced imaging are Arnold Chiari malformations, syringohydromyelia and closed spinal dysraphism such as diastematomyelia. Advanced imaging techniques, in particular multiplanar MRI, are also increasingly requested in children with known congenital scoliosis associated with spinal dysraphism and developmental causes of scoliosis such as neurofibromatosis and Klippel-Feil syndrome, as it allows superior delineation of the spinal column without the radiation risk. This review aims to examine the different imaging techniques currently used in the evaluation of scoliosis and provide a pictorial summary of the more common causes and associations. PMID:20634252

  9. Scoliosis (For Parents)

    MedlinePlus

    ... avoiding surgery. If an operation is necessary, the orthopedic surgeon performs a procedure that will correct the ... Surgery Bones, Muscles, and Joints Kyphosis Common Childhood Orthopedic Conditions X-Ray Exam: Scoliosis Word! Scoliosis Back ...

  10. Idiopathic hypersomnia

    MedlinePlus

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

  11. Using Patient-Reported Outcome Measures to Capture the Patient's Voice in Research and Care of Juvenile Idiopathic Arthritis.

    PubMed

    Hersh, Aimee O; Salimian, Parissa K; Weitzman, Elissa R

    2016-05-01

    Patient-reported outcome (PRO) measures provide a valuable window into how patients with juvenile idiopathic arthritis and their parents perceive their functioning, quality of life, and medication side effects in the context of their disease and treatment. Momentum behind adoption of PRO measures is increasing as these patient-relevant tools capture information pertinent to taking a patient-centered approach to health care and research. This article reviews the clinical and research utility of obtaining PROs across domains applicable to the experience of juvenile idiopathic arthritis and summarizes available self-report and parent-proxy PRO measures. Current challenges and limitations of PRO usage are discussed. PMID:27133493

  12. Immunohistochemical changes in morphologically involved and uninvolved colonic mucosa of patients with idiopathic proctitis.

    PubMed Central

    Das, K M; Erber, W F; Rubinstein, A

    1977-01-01

    Alterations in secretory component, IgA, IgG, and IgM were studied by immunofluorescent techniques in mucosal biopsy specimens obtained at colonoscopy from inflamed and grossly uninvolved colonic mucosa from 12 patients with idiopathic proctitis. Parotid-salivary secretory component and IgA and serum immunoglobulins were also investigated. Decreased secretory IgA was observed in the epithelium of all grossly involved rectal mucosa and in 40% of proximal normal mucosa. Salivary secretory IgA was not diminished. These observations suggest that a local immune defect may be pathogenetically related to idiopathic proctitis. Images PMID:320226

  13. Improving care for patients with idiopathic pulmonary fibrosis (IPF) in the UK: a round table discussion.

    PubMed

    Thickett, David R; Kendall, Clare; Spencer, Lisa G; Screaton, Nick; Wallace, William A; Pinnock, Hilary; Bott, Julia; Pigram, Lucy; Watson, Sarah; Millar, Ann B

    2014-12-01

    This article is the result of a round table discussion held at BMA House. Its purpose is to outline the challenges facing both patients and doctors in managing idiopathic pulmonary fibrosis. A number of recommendations for service development and research have been identified. PMID:25335486

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

    PubMed

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

    2009-01-01

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

  15. 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

  16. Is anterior release necessary in severe scoliosis treated by posterior segmental pedicle screw fixation?

    PubMed Central

    Kim, Jin-Hyok; Cho, Kyu-Jung; Kim, Sung-Soo; Lee, Jeong-Joon; Han, Yong-Taek

    2007-01-01

    With the advent of segmental pedicle screw fixation that enables more powerful corrective forces, it is postulated that an additional anterior procedure may be unnecessary even in severe deformities. The purpose of this paper is to evaluate the results of a posterior procedure alone using segmental pedicle screw fixation in severe scoliotic curves over 70°. Thirty-five scoliosis patients treated by pedicle screw fixation and rod derotation were retrospectively analyzed after a minimum follow-up of 2 years (range 2–10.4). The mean age of patients was 15.3 years (range 9.8–34.2). Diagnoses were idiopathic scoliosis in 29, neuromuscular scoliosis in 3 and scoliosis associated with Marfan syndrome in 3. Scoliosis consisted of single thoracic curve in 18, double thoracic in 5 and double major in 12. Twenty-five patients showed a major thoracic curve greater than 70° (range 70–100), and different ten patients showed a major lumbar curve greater than 70° (range 70–105), pre-operatively. The deformity angle, lowest instrumented vertebral tilt (LIVT) and spinal balance were measured. Pre-operatively there were nine patients with coronal decompensation. The pre-operative thoracic curve of 80 ± 9° with the flexibility of 45 ± 11% (45 ± 11° in side-bending film) was corrected to 27 ± 10° at the most recent follow-up, showing a correction of 66% (53°) and loss of correction of 3.0% (3.7°). The pre-operative lumbar curve of 79 ± 12° with the flexibility of 62 ± 14% (30 ± 11° in side-bending film) was corrected to 33 ± 14° at the most recent follow-up [59% (46°) curve correction, 3.5% (3.0°) loss of curve correction]. The pre-operative LIVT of 30 ± 8° was corrected to 11 ± 6°, showing a correction of 62% (19°). Residual coronal decompensation was observed in three patients postoperatively. Pre-operative thoracic kyphosis of 27° (range 0–82) improved postoperatively to 31° (range 14–53). In conclusion, posterior

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

    PubMed

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

    2011-07-01

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

  18. Effects of standing on cerebrovascular resistance in patients with idiopathic orthostatic intolerance

    NASA Technical Reports Server (NTRS)

    Jacob, G.; Atkinson, D.; Jordan, J.; Shannon, J. R.; Furlan, R.; Black, B. K.; Robertson, D.

    1999-01-01

    PURPOSE: Patients with idiopathic orthostatic intolerance often have debilitating symptoms on standing that are suggestive of cerebral hypoperfusion despite the absence of orthostatic hypotension. SUBJECTS AND METHODS: We evaluated the effects of graded head-up tilt on cerebral blood flow as determined by transcranial Doppler measurements in 10 patients with idiopathic orthostatic intolerance (nine women, one man, 22 to 47 years) and nine age- and sex-matched control subjects. RESULTS: In patients, mean (+/- SD) arterial pressure at 0 degrees head-up tilt was 90 +/- 11 mm Hg and was well maintained at all tilt angles (90 +/- 11 mm Hg at 75 degrees). In controls, mean arterial pressure was 85 +/- 7 mm Hg at 0 degrees and 82 +/- 11 mm Hg at 75 degrees head-up tilt. There was a substantial decrease in peak velocity with increasing tilt angle in patients (28% +/- 10%) but not in controls (10% +/- 10% at 75 degrees, P <0.001). Similarly, mean velocity decreased 26% +/- 13% in patients and 12% +/- 11% in controls (P = 0.01). With increasing head-up tilt, patients had a significantly greater increase in regional cerebrovascular resistance than controls. CONCLUSIONS: In patients with idiopathic orthostatic intolerance, peak and mean middle cerebral artery blood flow velocity decreased in response to head-up tilt despite well sustained arterial blood pressure. These observations indicate that in this group of patients, regulation of cerebrovascular tone may be impaired and might therefore be a target for therapeutic interventions.

  19. Diagnostic accuracy of somatosensory evoked potential monitoring during scoliosis fusion.

    PubMed

    Thirumala, Parthasarathy D; Cheng, Hannah L; Loke, Yoon K; Kojo Hamilton, D; Balzer, Jeffrey; Crammond, Donald J

    2016-08-01

    The goal of this review was to ascertain the diagnostic accuracy of intraoperative somatosensory evoked potential (SSEP) changes to predict perioperative neurological outcome in patients undergoing spinal deformity surgery to correct adolescent idiopathic scoliosis (AIS). The authors searched PubMed/MEDLINE and World Science databases to retrieve reports and/or experiments from January 1950 through January 2014 for studies on SSEP use during AIS surgery. All motor and sensory deficits were noted in the neurological examination administered after the procedure which was used to determine the effectiveness of SSEP as an intraoperative monitoring technique. Fifteen studies identified a total of 4763 procedures on idiopathic patients. The observed incidence of neurological deficits was 1.11% (53/4763) of the sample population. Of the patients with new postoperative neurological deficits 75.5% (40/53) showed significant SSEP changes, and 24.5% (13/53) did not show significant change. Pooled analysis using the bivariate model showed SSEP change with pooled sensitivity (average 84%, 95% confidence interval 59-95%) and specificity (average 98%, 95% confidence interval 97-99%). The diagnostic odds ratio of a patient who had a new neurological deficit with SSEP changes was a diagnostic odds ratio of 340 (95% confidence interval 125-926). Overall, detection of SSEP changes had excellent discriminant ability with an area under the curve of 0.99. Our meta-analysis covering 4763 operations on idiopathic patients showed that it is a highly sensitive and specific test and that iatrogenic spinal cord injury resulting in new neurological deficits was 340 times more likely to have changes in SSEP compared to those without any new deficits. PMID:27021224

  20. Fellowship and Practice Composition Affect Surgical Decision Making in Patients with Adult Degenerative Scoliosis: Spinal Deformity versus Degenerative Spinal Surgeons

    PubMed Central

    Protopsaltis, Themistocles; Patel, Ashish; Yoo, Andrew; Lonner, Baron

    2015-01-01

    Background For the Adult Degenerative Scoliosis (ADS) patient with radiculopathy, there is no clear data in the literature to guide the spine surgeon's decision making in choosing between limited decompression alone, short segment fusion, or longer arthrodesis of the deformity. This study investigates the differences in operative planning, for patients with ADS and radiculopathy, between two groups of spine surgeons based on fellowship experience and practice composition. Methods Six Degenerative Spine surgeons (Group 1) and 6 Spinal Deformity surgeons (Group 2) were shown 7 cases of patients with ADS and radiculopathy. Surgeons completed a questionnaire detailing their planned operative intervention including the number of fusion levels, if any, approach, choice of bone graft, and interbody device. Pearson Correlation was used to investigate the association between fellowship training, practice composition, number of levels fused, and other variables. Intraclass correlation (ICC) analysis was used to investigate the internal consistency among the groups. Results There was a direct correlation between fellowship deformity experience and practice composition (r=0.75, p<0.01), and between deformity practice composition and the number of planned fusion levels (r=0.90, p<0.001). Group 1 surgeons fused a mean 3.7 vertebral levels (range 0-6.7), while Group 2 surgeons fused a mean 10.8 levels (range 4-16.5). Group 2 surgeons fused a significantly greater number of levels for each case than degenerative surgeons on paired student t-test (p=0.002). Group 1 surgeons chose decompression alone more commonly than deformity surgeons (p<0.05). Group 2 surgeons had significantly higher group consistency by ICC analysis (p=0.004). Conclusions Fellowship and practice composition influence the physician's surgical planning in ADS. There is a lack of standardized treatment paradigms for the management of radiculopathy in patients with ADS. PMID:26114090

  1. Incidental (malignancy) and coincidental (idiopathic polydactylous longitudinal erythronychia) conditions in patients with segmental neurofibromatosis.

    PubMed

    Cohen, Philip R

    2013-04-01

    Segmental neurofibromatosis (SNF) is an uncommon presentation of neurofibromatosis type 1 (NF-1). Although patients with SNF are at a lower risk for developing NF-l-associated complications, the estimated occurrence of related malignancies may be approaching the frequency observed in patients with NF-1. Idiopathic polydactylous longitudinal erythronychia also may be associated with SNF, though the frequency of this association remains to be determined. PMID:23763076

  2. Comparison of endogenous and radiolabeled bile acid excretion in patients with idiopathic chronic diarrhea

    SciTech Connect

    Schiller, L.R.; Bilhartz, L.E.; Santa Ana, C.A. )

    1990-04-01

    Fecal recovery of radioactivity after ingestion of a bolus of radiolabeled bile acid is abnormally high in most patients with idiopathic chronic diarrhea. To evaluate the significance of this malabsorption, concurrent fecal excretion of both exogenous radiolabeled bile acid and endogenous (unlabeled) bile acid were measured in patients with idiopathic chronic diarrhea. Subjects received a 2.5-microCi oral dose of taurocholic acid labeled with 14C in the 24th position of the steroid moiety. Endogenous bile acid excretion was measured by a hydroxysteroid dehydrogenase assay on a concurrent 72-h stool collection. Both radiolabeled and endogenous bile acid excretion were abnormally high in most patients with chronic diarrhea compared with normal subjects, even when equivoluminous diarrhea was induced in normal subjects by ingestion of osmotically active solutions. The correlation between radiolabeled and endogenous bile acid excretion was good. However, neither radiolabeled nor endogenous bile acid excretion was as abnormal as is typically seen in patients with ileal resection, and none of these diarrhea patients responded to treatment with cholestyramine with stool weights less than 200 g. These results suggest (a) that this radiolabeled bile acid excretion test accurately reflects excess endogenous bile acid excretion; (b) that excess endogenous bile acid excretion is not caused by diarrhea per se; (c) that spontaneously occurring idiopathic chronic diarrhea is often associated with increased endogenous bile acid excretion; and (d) that bile acid malabsorption is not likely to be the primary cause of diarrhea in most of these patients.

  3. Radiation hazards in scoliosis management

    SciTech Connect

    Drummond, D.; Ranallo, F.; Lonstein, J.; Brooks, H.L.; Cameron, J.

    1983-10-01

    Safe radiography in scoliosis management is based on a sound knowledge of 1) the radiographic imaging process, 2) the degree of risk to the patient from radiation exposure, and 3) the radiographic requirements to both evaluate and follow patients with spine deformity. This paper is a current review of the subject and work done at the authors' centers. It includes recommendations for reducing the radiation risk while maintaining necessary diagnostic information.

  4. Immunosuppressive therapy for patients with Down syndrome and idiopathic aplastic anemia.

    PubMed

    Suzuki, Kyogo; Muramatsu, Hideki; Okuno, Yusuke; Narita, Atsushi; Hama, Asahito; Takahashi, Yoshiyuki; Yoshida, Makoto; Horikoshi, Yasuo; Watanabe, Ken-Ichiro; Kudo, Kazuko; Kojima, Seiji

    2016-07-01

    Idiopathic aplastic anemia (AA) is a rare hematological complication of Down syndrome (DS). The safety and efficacy of immunosuppressive therapy (IST) in individuals with DS remain unknown. We used a standard regimen of IST, comprising antithymocyte globulin and cyclosporine A, to treat three children with DS and idiopathic acquired AA. Two patients achieved a hematological (complete or partial) response and became transfusion independent at the final follow-up. The third patient failed to respond to IST and underwent bone marrow transplantation from a human leukocyte antigen (HLA)-mismatched unrelated donor. None of the patients experienced severe or unexpected adverse events during IST. Our experience suggests that IST is a safe and reasonable treatment, even in individuals with DS who suffer from AA and lack an HLA-matched sibling donor. PMID:27107757

  5. Stress, psychiatric co-morbidity and coping in patients with chronic idiopathic urticaria.

    PubMed

    Chung, Man Cheung; Symons, Christine; Gilliam, Jane; Kaminski, Edward R

    2010-04-01

    This study examined life event stress, perceived stress and psychiatric co-morbidity among patients with Chronic Idiopathic Urticaria (CIU). It also investigated the relationship between coping, stress, the severity of CIU and psychiatric co-morbidity. Total of 100 CIU patients and 60 allergy patients participated in the study. They completed the General Health Questionnaire, the Social Readjustment Rating Scale, the Perceived Stress Scale, and the Ways of Coping Checklist. Compared with allergy patients, CIU patients had worse co-morbidity and higher levels of life event stress and perceived stress. Emotion-focussed coping was associated with the severity of CIU; perceived stress was associated with co-morbidity. PMID:20204926

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

    PubMed

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

    2011-07-01

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

  7. Caffeine restriction has no role in the management of patients with symptomatic idiopathic ventricular premature beats.

    PubMed Central

    Newby, D. E.; Neilson, J. M.; Jarvie, D. R.; Boon, N. A.

    1996-01-01

    OBJECTIVE: To assess the role of caffeine restriction in the management of patients with symptomatic idiopathic ventricular premature beats. DESIGN: A randomised, double blind, 6 week intervention trial incorporating dietary caffeine restriction, caffeinated coffee, and decaffeinated coffee. SETTING: Cardiac outpatient clinic. PATIENTS: 13 patients with symptomatic frequent idiopathic ventricular premature beats. MAIN OUTCOME MEASURES: Weekly measures of serum caffeine concentration, coffee consumption, visual analogue score of palpitations, and 24 hour ventricular premature beat frequency. RESULTS: The interventions achieved significant alterations in serum caffeine concentrations (P < 0.001) which correlated with coffee consumption (r = 0.70; P < 0.001). Visual analogue palpitation scores showed a small, but significant correlation with ventricular premature beat frequencies (r = 0.34; P = 0.003). However, there were no significant changes in palpitation scores or ventricular premature beat frequencies during the intervention weeks and no significant correlations were found between these variables and serum caffeine concentrations. CONCLUSIONS: Caffeine restriction has no role in the management of patients referred with symptomatic idiopathic ventricular premature beats. PMID:8983684

  8. Linaclotide in Chronic Idiopathic Constipation Patients with Moderate to Severe Abdominal Bloating: A Randomized, Controlled Trial

    PubMed Central

    Lacy, Brian E.; Schey, Ron; Shiff, Steven J.; Lavins, Bernard J.; Fox, Susan M.; Jia, Xinwei D.; Blakesley, Rick E.; Hao, Xinming; Cronin, Jacquelyn A.; Currie, Mark G.; Kurtz, Caroline B.; Johnston, Jeffrey M.; Lembo, Anthony J.

    2015-01-01

    Background Abdominal bloating is a common and bothersome symptom of chronic idiopathic constipation. The objective of this trial was to evaluate the efficacy and safety of linaclotide in patients with chronic idiopathic constipation and concomitant moderate-to-severe abdominal bloating. Methods This Phase 3b, randomized, double-blind, placebo-controlled clinical trial randomized patients to oral linaclotide (145 or 290 μg) or placebo once daily for 12 weeks. Eligible patients met Rome II criteria for chronic constipation upon entry with an average abdominal bloating score ≥5 (self-assessment: 0 10-point numerical rating scale) during the 14-day baseline period. Patients reported abdominal symptoms (including bloating) and bowel symptoms daily; adverse events were monitored. The primary responder endpoint required patients to have ≥3 complete spontaneous bowel movements/week with an increase of ≥1 from baseline, for ≥9 of 12 weeks. The primary endpoint compared linaclotide 145 μg vs. placebo. Results The intent-to-treat population included 483 patients (mean age=47.3 years, female=91.5%, white=67.7%). The primary endpoint was met by 15.7% of linaclotide 145 μg patients vs. 7.6% of placebo patients (P<0.05). Both linaclotide doses significantly improved abdominal bloating vs. placebo (P<0.05 for all secondary endpoints, controlling for multiplicity). Approximately one-third of linaclotide patients (each group) had ≥50% mean decrease from baseline in abdominal bloating vs. 18% of placebo patients (P<0.01). Diarrhea was reported in 6% and 17% of linaclotide 145 and 290 μg patients, respectively, and 2% of placebo patients. AEs resulted in premature discontinuation of 5% and 9% of linaclotide 145 μg and 290 μg patients, respectively, and 6% of placebo patients. Conclusions Once-daily linaclotide (145 and 290 μg) significantly improved bowel and abdominal symptoms in chronic idiopathic constipation patients with moderate-to-severe baseline abdominal

  9. [Lung transplantation in patients with interstitial lung disease/idiopathic pulmonary fibrosis].

    PubMed

    Murer, Christian; Benden, Christian

    2016-01-01

    Lung transplantation is an established therapy for advanced lung disease. Among the common disease indications for lung transplantation, patients with interstitial lung disease, in particular, idiopathic pulmonary fibrosis (IPF), have the worst prognosis. Thus referral to a transplant center should ideally be realised at the time of diagnosis of usual interstitial pneumonitis (UIP), regardless of lung function, in order to carry out a through initial assessment and evaluation. PMID:26884220

  10. Inter-rater reliability of the EEG reading in patients with childhood idiopathic epilepsy.

    PubMed

    Piccinelli, Paolo; Viri, Maurizio; Zucca, Claudio; Borgatti, Renato; Romeo, Antonino; Giordano, Laura; Balottin, Umberto; Beghi, Ettore

    2005-01-01

    The level of agreement in the interpretation of EEG records by different experienced readers working in three child neurology tertiary centers has been evaluated. EEG recordings randomly chosen from patients with idiopathic epilepsy were included. Optimal or suboptimal agreement was found for presence of ictal and interictal discharges. Contrary to ictal discharges, the distribution and location of interictal discharges was not unanimously interpreted and agreement was unsatisfactory when assessing the background activity. PMID:16118044

  11. Synthetic ACTH in High Risk Patients with Idiopathic Membranous Nephropathy: A Prospective, Open Label Cohort Study

    PubMed Central

    van de Logt, Anne-Els; Beerenhout, Charles H.; Brink, Hans S.; van de Kerkhof, Jos J.; Wetzels, Jack F.; Hofstra, Julia M.

    2015-01-01

    New therapeutic agents are warranted in idiopathic membranous nephropathy. Synthetic ACTH may be advantageous with reported remission rates up to 85% and few side effects. We conducted a prospective open label cohort study from 2008 till 2010 (NCT00694863). We prospectively selected patients with idiopathic membranous nephropathy and high risk for progression (defined as βeta-2-microglobulin (β2m) excretion of >500 ng/min). For comparison, we selected matched historical controls treated with cyclophosphamide. The prospectively selected patients received intramuscular injections of synthetic ACTH during 9 months (maximal dose 1 mg twice a week). The primary endpoints concerned the feasibility and incidence of remissions as a primary event. Secondary endpoints included side effects of treatment and the incidence of remissions and relapses at long-term follow-up. Twenty patients (15 men) were included (age 54±14 years, serum creatinine 104 μmol/l [IQR 90–113], urine protein:creatinine ratio 8.7 g/10 mmol creatinine [IQR 4.3–11.1]). Seventeen patients (85%) completed treatment. 97% of injections were administered correctly. Cumulative remission rate was 55% (complete remission in 4 patients, partial remission 7 patients). In a group of historical controls treated with cyclophosphamide and steroids, 19 of 20 patients (95%) developed a remission (complete remission in 13 patients, partial remission in 6 patients) (p<0.01). The main limitation of our study is its small size and the use of a historical control group. We show that treatment with intramuscular injections of synthetic ACTH is feasible. Our data suggest that synthetic ACTH is less effective than cyclophosphamide in inducing a remission in high risk patients with idiopathic membranous nephropathy. The use of synthetic ACTH was also associated with many adverse events. Therefore, we advise against synthetic ACTH as standard treatment in membranous nephropathy. Trial Registration ClinicalTrials.gov NCT

  12. Radiation enteritis and radiation scoliosis

    SciTech Connect

    Shah, M.; Eng, K.; Engler, G.L.

    1980-09-01

    Any patient with radiation scoliosis should be suspected of having a visceral lesion as well. Chronic radiation enteritis may be manifested by intestinal obstruction, fistulas, perforation, and hemorrhage. Intestinal obstruction is the most common complication, and must be differentiated from postoperative cast or from spinal-traction syndrome. Obstruction that does not respond promptly to conservative measures must be treated surgically. Irradiated bowel is ischemic, and necrosis with spontaneous perforation can only be avoided with early diagnosis and surgical intervention.

  13. Transient Monoplegia as a Result of Unilateral Femoral Artery Ischemia Detected by Multimodal Intraoperative Neuromonitoring in Posterior Scoliosis Surgery

    PubMed Central

    Pankowski, Rafal; Roclawski, Marek; Dziegiel, Krzysztof; Ceynowa, Marcin; Mikulicz, Marcin; Mazurek, Tomasz; Kloc, Wojciech

    2016-01-01

    Abstract This is to report a case of 16-year-old girl with transient right lower limb monoplegia as a result of femoral artery ischemia detected by multimodal intraoperative spinal cord neuromonitoring (MISNM) during posterior correction surgery of adolescent idiopathic scoliosis. A patient with a marfanoid body habitus and LENKE IA type scoliosis with the right thoracic curve of 48° of Cobb angle was admitted for posterior spinal fusion from Th6 to L2. After selective pedicle screws instrumentation and corrective maneuvers motor evoked potentials (MEP) began to decrease with no concomitant changes in somato-sensory evoked potentials recordings. The instrumentation was released first partially than completely with rod removal but the patient demonstrated constantly increasing serious neurological motor deficit of the whole right lower limb. Every technical cause of the MEP changes was eliminated and during the wake-up test the right foot was found to be pale and cold with no popliteal and dorsalis pedis pulses palpable. The patient was repositioned and the pelvic pad was placed more cranially. Instantly, the pulse and color returned to the patient's foot. Following MEP recordings showed gradual return of motor function up to the baseline at the end of the surgery, whereas somato-sensory evoked potentials were within normal range through the whole procedure. This case emphasizes the importance of the proper pelvic pad positioning during the complex spine surgeries performed in prone position of the patient. A few cases of neurological complications have been described which were the result of vascular occlusion after prolonged pressure in the inguinal area during posterior scoliosis surgery when the patient was in prone position. If incorrectly interpreted, they would have a significant impact on the course of scoliosis surgery. PMID:26871822

  14. The distribution of immunomodulatory cells in the lungs of patients with idiopathic pulmonary fibrosis.

    PubMed

    Nuovo, Gerard J; Hagood, James S; Magro, Cynthia M; Chin, Nena; Kapil, Rubina; Davis, Luke; Marsh, Clay B; Folcik, Virginia A

    2012-03-01

    We have characterized the immune system involvement in the disease processes of idiopathic pulmonary fibrosis in novel ways. To do so, we analyzed lung tissue from 21 cases of idiopathic pulmonary fibrosis and 21 (non-fibrotic, non-cancerous) controls for immune cell and inflammation-related markers. The immunohistochemical analysis of the tissue was grouped by patterns of severity in disease pathology. There were significantly greater numbers of CD68(+) and CD80(+) cells and significantly fewer CD3(+), CD4(+), and CD45RO(+) cells in areas of relatively (histologically) normal lung in biopsy samples from idiopathic pulmonary fibrosis patients compared with controls. In zones of active disease, characterized by epithelial cell regeneration and fibrosis, there were significantly more cells expressing CD4, CD8, CD20, CD68, CD80, chemokine receptor 6 (CCR6), S100, IL-17, tumor necrosis factor-α, and retinoic acid-related orphan receptors compared with histologically normal lung areas from idiopathic pulmonary fibrosis patients. Inflammation was implicated in these active regions by the cells that expressed retinoid orphan receptor-α, -β, and -γ, CCR6, and IL-17. The regenerating epithelial cells predominantly expressed these pro-inflammatory molecules, as evidenced by co-expression analyses with epithelial cytokeratins. Macrophages in pseudo-alveoli and CD3(+) T cells in the fibrotic interstitium also expressed IL-17. Co-expression of IL-17 with retinoid orphan receptors and epithelial cytoskeletal proteins, CD68, and CD3 in epithelial cells, macrophages, and T-cells, respectively, confirmed the production of IL-17 by these cell types. There was little staining for forkhead box p3, CD56, or CD34 in any idiopathic pulmonary fibrosis lung regions. The fibrotic regions had fewer immune cells overall. In summary, our study shows participation of innate and adaptive mononuclear cells in active-disease regions of idiopathic pulmonary fibrosis lung, where the regenerating

  15. Idiopathic Intracranial Hypertension in a Prepubertal Pediatric Japanese Patient Complicated by Severe Papilledema

    PubMed Central

    Oka, Akiko; Ikesugi, Kengo; Kondo, Mineo

    2016-01-01

    Purpose To report a rare case of idiopathic intracranial hypertension (IIH) in a prepubertal pediatric patient. Case Report The patient was an 11-year-old Japanese boy. Initially, an ophthalmologist found severe papilledema, and the patient was diagnosed with IIH. He was unresponsive to conservative therapy, and a ventriculoperitoneal shunt was inserted in the Neurosurgery Department because the visual impairment was severe. Twelve months after the shunt, the improvement in vision was limited due to optic disc atrophy. Conclusion Ophthalmologists need to be more aware of II, especially in cases with severe papilledema that can lead to permanent reduction of vision. PMID:27065853

  16. Scoliosis corrective force estimation from the implanted rod deformation using 3D-FEM analysis

    PubMed Central

    2015-01-01

    Background Improvement of material property in spinal instrumentation has brought better deformity correction in scoliosis surgery in recent years. The increase of mechanical strength in instruments directly means the increase of force, which acts on bone-implant interface during scoliosis surgery. However, the actual correction force during the correction maneuver and safety margin of pull out force on each screw were not well known. In the present study, estimated corrective forces and pull out forces were analyzed using a novel method based on Finite Element Analysis (FEA). Methods Twenty adolescent idiopathic scoliosis patients (1 boy and 19 girls) who underwent reconstructive scoliosis surgery between June 2009 and Jun 2011 were included in this study. Scoliosis correction was performed with 6mm diameter titanium rod (Ti6Al7Nb) using the simultaneous double rod rotation technique (SDRRT) in all cases. The pre-maneuver and post-maneuver rod geometry was collected from intraoperative tracing and postoperative 3D-CT images, and 3D-FEA was performed with ANSYS. Cobb angle of major curve, correction rate and thoracic kyphosis were measured on X-ray images. Results Average age at surgery was 14.8, and average fusion length was 8.9 segments. Major curve was corrected from 63.1 to 18.1 degrees in average and correction rate was 71.4%. Rod geometry showed significant change on the concave side. Curvature of the rod on concave and convex sides decreased from 33.6 to 17.8 degrees, and from 25.9 to 23.8 degrees, respectively. Estimated pull out forces at apical vertebrae were 160.0N in the concave side screw and 35.6N in the convex side screw. Estimated push in force at LIV and UIV were 305.1N in the concave side screw and 86.4N in the convex side screw. Conclusions Corrective force during scoliosis surgery was demonstrated to be about four times greater in the concave side than in convex side. Averaged pull out and push in force fell below previously reported safety

  17. Relationship between cardioscopic images and histological changes in the left ventricle of patients with idiopathic myocarditis†

    PubMed Central

    Uchida, Yasumi; Uchida, Yasuto; Sakurai, Takeshi; Kanai, Masahito; Shirai, Seiichiro; Nakagawa, Osamu; Hiruta, Nobuyuki

    2011-01-01

    Aims Endomyocardial biopsy is essential for definite diagnosis of idiopathic myocarditis. However, since endomyocardial biopsy is guided by fluoroscopy, whether or not the diseased myocardium is biopsied depends on chance, and this may lead to misdiagnosis. If the endocardial surface represents changes indicative of stages of myocarditis, staging of myocarditis and targeted cardioscope-guided biopsy could be used for accurate histological diagnosis. Methods and results The relationship between left ventricular endocardial surface colour observed by cardioscopy and biopsy findings were examined in 78 patients with suspected idiopathic myocarditis. Of these, 59 patients were diagnosed histologically as idiopathic myocarditis. Endocardial colour was classified into red, milky white, purple, yellowish brown, or white. Biopsied specimens with red and milky white wall segments exhibited histological changes compatible with acute myocarditis; purple segments, active chronic myocarditis; and yellowish brown and white segments, inactive chronic myocarditis. The sensitivity, specificity, and predictive value of red and milky white colours for detecting acute myocarditis were 100, 100, and 100%, respectively; of purple for detecting active chronic myocarditis were 83, 92, and 78%, respectively; and yellowish brown and white for detecting inactive chronic myocarditis were 82, 74, and 53, respectively. Conclusion Red and milky white endocardial surface colours predicted histological acute myocarditis, and purple predicted active chronic myocarditis. However, yellowish brown and white colours did not predict inactive chronic myocarditis. PMID:21257727

  18. Rotenone Susceptibility Phenotype in Olfactory Derived Patient Cells as a Model of Idiopathic Parkinson's Disease.

    PubMed

    Murtaza, M; Shan, J; Matigian, N; Todorovic, M; Cook, A L; Ravishankar, S; Dong, L F; Neuzil, J; Silburn, P; Mackay-Sim, A; Mellick, G D; Wood, S A

    2016-01-01

    Parkinson's disease is a complex age-related neurodegenerative disorder. Approximately 90% of Parkinson's disease cases are idiopathic, of unknown origin. The aetiology of Parkinson's disease is not fully understood but increasing evidence implies a failure in fundamental cellular processes including mitochondrial dysfunction and increased oxidative stress. To dissect the cellular events underlying idiopathic Parkinson's disease, we use primary cell lines established from the olfactory mucosa of Parkinson's disease patients. Previous metabolic and transcriptomic analyses identified deficiencies in stress response pathways in patient-derived cell lines. The aim of this study was to investigate whether these deficiencies manifested as increased susceptibility, as measured by cell viability, to a range of extrinsic stressors. We identified that patient-derived cells are more sensitive to mitochondrial complex I inhibition and hydrogen peroxide induced oxidative stress, than controls. Exposure to low levels (50 nM) of rotenone led to increased apoptosis in patient-derived cells. We identified an endogenous deficit in mitochondrial complex I in patient-derived cells, but this did not directly correlate with rotenone-sensitivity. We further characterized the sensitivity to rotenone and identified that it was partly associated with heat shock protein 27 levels. Finally, transcriptomic analysis following rotenone exposure revealed that patient-derived cells express a diminished response to rotenone-induced stress compared with cells from healthy controls. Our cellular model of idiopathic Parkinson's disease displays a clear susceptibility phenotype to mitochondrial stress. The determination of molecular mechanisms underpinning this susceptibility may lead to the identification of biomarkers for either disease onset or progression. PMID:27123847

  19. Congenital Scoliosis in Smith–Magenis Syndrome

    PubMed Central

    Li, Zheng; Shen, Jianxiong; Liang, Jinqian; Sheng, Lin

    2015-01-01

    Abstract The Smith–Magenis syndrome (SMS) is a complex and rare congenital condition that is characterized by minor craniofacial anomalies, short stature, sleep disturbances, behavioral, and neurocognitive abnormalities, as well as variable multisystemic manifestations. Little is reported about spinal deformity associated with this syndrome. This study is to present a case of scoliosis occurring in the setting of SMS and explore the possible mechanisms between the 2 diseases. The patient is a 13-year-old Chinese female with congenital scoliosis and Tetralogy of Fallot, mental retardation, obstructive sleep apnea, hypertrophy of tonsil, conductive hearing loss, and agenesis of the epiglottis. An interphase fluorescent in situ hybridization at chromosome 17p11.2 revealed a heterozygous deletion, confirming a molecular diagnosis of SMS. She underwent a posterior correction at thoracic 1-lumbar 1 (T1-L1) levels, using the Moss-SI spinal system. At 6-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of correction. Congenital cardiac disease, immunodeficiency, and severe behavioral problems can affect the surgical outcome following spine fusion and need to be taken into consideration for the surgeon and anesthesiologist. Scoliosis is not uncommon among patients with SMS, and there is a potential association between congenital scoliosis and SMS. The potential mechanisms in the pathogenesis of congenital scoliosis of SMS included retinoic acid-induced 1 (RAI1) microdeletion and RAI1 gene point mutation. PMID:25929900

  20. Pulmonary artery aneurysm in an adult patient with idiopathic dilatation of the pulmonary artery

    PubMed Central

    Betkier-Lipińska, Katarzyna; Czarkowski, Sebastian; Hendzel, Piotr; Cwetsch, Andrzej

    2015-01-01

    Idiopathic dilatation of the pulmonary artery (IDPA) is a rare congenital heart disease. It has been described for almost one hundred years, and numerous definitions have been proposed. The IDPA diagnostic criteria have not been updated for years. Secondary to primary disease, pulmonary artery aneurism was recognised as a lethal defect; however, long-term follow-up of patients with IDPA has not been well researched. Thus, indications to medical or surgical treatment are not evidence based. Here, we present a rare case of a 54-year-old patient with IDPA, who remained under observation for 36 years without surgical intervention. PMID:26855651

  1. A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone.

    PubMed

    Sato, Seidai; Hanibuchi, Masaki; Takahashi, Mikiko; Fukuda, Yuh; Morizumi, Shun; Toyoda, Yuko; Goto, Hisatsugu; Nishioka, Yasuhiko

    2016-01-01

    The patient was a 68-year-old man presenting with body weight loss and exertional dyspnea. High-resolution computed tomography of the chest showed dense subpleural consolidation with traction bronchiectasis and volume loss predominantly in bilateral apical lesions and upper lobes. A histopathological analysis of a specimen of the right upper lobe showed histological patterns which were consistent with idiopathic pleuroparenchymal fibroelastotis (IPPFE). Treatment with pirfenidone was introduced with the expectation of its potential benefit. The effect of pirfenidone was satisfactory, and a decline in forced vital capacity was inhibited during treatment. This is the first case report suggesting the efficacy of pirfenidone for patients with IPPFE. PMID:26935370

  2. Macrophage activation syndrome in a patient with systemic onset of the juvenile idiopathic arthritis

    PubMed Central

    Aggarwal, Hari K.; Rao, Avinash; Mittal, Anshul; Jain, Promil

    2016-01-01

    Systemic onset juvenile idiopathic arthritis (sJIA) is defined as arthritis affecting one or more joint usually in the juvenile age group (< 16 years of age) with or preceded by fever of at least 2 weeks duration that is documented to be daily (“quotidian”) for at least 3 days which may be associated with evanescent (non-fixed) erythematous rash or generalized lymph node enlargement or hepatomegaly/splenomegaly/both or serositis. Macrophage activation syndrome (MAS) is a life-threatening complication of sJIA marked by sudden onset of non-remitting high fever, profound depression in all three blood cell lines (i.e. leukopenia, anemia, and thrombocytopenia), hepatosplenomegaly, lymphadenopathy, and elevated serum liver enzyme levels. In children with systemic juvenile idiopathic arthritis, the clinical picture may mimic sepsis or an exacerbation of the underlying disease. We report a case of a 16-year-old female patient presenting with high grade fever with joint pains and generalized weakness which proved to be systemic onset juvenile idiopathic arthritis with macrophage activation syndrome after ruling out all other differential diagnoses and responded well to intravenous steroids. PMID:27407277

  3. [Treatment standards of the oral anticoagulant in patients with idiopathic pulmonary embolism].

    PubMed

    Kowalski, Zbigniew; Kowalski, Piotr; Grzegorek, Damian

    2016-07-01

    The optimal and the most effective treatment of pulmonary embolism is still a matter of concern and each day sees a new set of challenges for the world of medicine. The progress, has been made in recent years, improved quality of life and caused much better treatment results. This is difficult issue in patients, receiving anticoagulant therapy, because they require an individual approach and adjustability to the therapeutic possibilities. The benefits of long-term anticoagulant therapy, which decreases relapses of idiopathic venous thromboembolism and diminishes risk of thromboembolic complications, should be taking under consideration. It is still a matter of dispute the time of carrying out of treatment, especially after the first life idiopathic episode of pulmonary embolism. The purpose of this paper is an overview and a summary of the foregoing achievements concerned the standards of idiopathic pulmonary embolism treatment, expecting benefits flowing with using new oral anticoagulants, as an alternative to known for decades Vitamin K antagonist drugs. A lot of information about new oral anticoagulants speaks in favor of their use, but unknown safety of the drugs caused searching the best strategy of pulmonary embolism treatment all the time. PMID:27590653

  4. [Treatment standards of the oral anticoagulant in patients with idiopathic pulmonary embolism].

    PubMed

    Kowalski, Zbigniew; Kowalski, Piotr; Grzegorek, Damian

    2016-08-01

    The optimal and the most effective treatment of pulmonary embolism is still a matter of concern and each day sees a new set of challenges for the world of medicine. The progress, has been made in recent years, improved quality of life and caused much better treatment results. This is difficult issue in patients, receiving anticoagulant therapy, because they require an individual approach and adjustability to the therapeutic possibilities. The benefits of long-term anticoagulant therapy, which decreases relapses of idiopathic venous thromboembolism and diminishes risk of thromboembolic complications, should be taking under consideration. It is still a matter of dispute the time of carrying out of treatment, especially after the first life idiopathic episode of pulmonary embolism. The purpose of this paper is an overview and a summary of the foregoing achievements concerned the standards of idiopathic pulmonary embolism treatment, expecting benefits flowing with using new oral anticoagulants, as an alternative to known for decades Vitamin K antagonist drugs. A lot of information about new oral anticoagulants speaks in favor of their use, but unknown safety of the drugs caused searching the best strategy of pulmonary embolism treatment all the time. PMID:27591448

  5. Associations between neuropsychiatric symptoms and cognition in Chinese idiopathic Parkinson's disease patients.

    PubMed

    Guo, Xiaoyan; Song, Wei; Chen, Ke; Chen, XuePing; Zheng, Zhenzhen; Cao, Bei; Huang, Rui; Zhao, Bi; Wu, Ying; Shang, Hui-Fang

    2015-03-01

    The associations between neuropsychiatric symptoms and cognition, frontal lobe function and frontal behavioral changes in the Chinese idiopathic Parkinson's disease (PD) population are largely unknown. This study included 348 idiopathic PD patients from southwest China. Neuropsychiatric symptoms were investigated using the Neuropsychiatric Inventory Questionnaire (NPI), and cognition was assessed using Addenbrooke's Cognitive Examination-Revised (ACE-R). The Frontal Assessment Battery (FAB) was used to evaluate frontal function and the Frontal Behavior Inventory (FBI) was used to assess frontal behavioral changes. The mean (± standard deviation) age of the PD patients was 60.24 ± 12.07 years, and the mean disease duration was 3.88 ± 3.34 years. The mean NPI score was 3.49 ± 4.00. The mean score of ACE-R was 76.82 ± 16.73. The mean score of FAB was 15.27 ± 2.90, and the mean score of FBI was 3.18 ± 5.17. Weak negative correlations between the NPI and ACE-R scores as well as FAB score were found in the total sample, the male patient subgroup, the early onset PD subgroup and the late onset PD subgroup. Strong positive correlations were found between the NPI and FBI scores in the total sample (r=0.661, p<0.001), the male patient subgroup (r=0.789, p<0.001) and the late onset PD subgroup (r=0.749, p<0.001). Moderate positive correlations were found between the NPI and FBI scores in the female patient subgroup (r=0.536, p<0.001) and the early onset PD subgroup (r=0.462, p<0.001). Neuropsychiatric symptoms were closely associated with frontal behavioral changes but were not closely related with worse cognition and frontal lobe function in the Chinese idiopathic PD population. PMID:25582976

  6. GASTRIC MOTOR DISTURBANCES IN PATIENTS WITH IDIOPATHIC RAPID GASTRIC EMPTYING

    PubMed Central

    Bharucha, Adil E.; Manduca, Armando; Lake, David S.; Fidler, Jeff; Edwards, Phillip; Grimm, Roger C.; Zinsmeister, Alan R.; Riederer, Stephen J.

    2011-01-01

    Background and Aims The mechanisms of “idiopathic” rapid gastric emptying, which is associated with functional dyspepsia and functional diarrhea, are not understood. Our hypotheses were that increased gastric motility and reduced postprandial gastric accommodation contribute to rapid gastric emptying. Methods Fasting and postprandial (300kCal nutrient meal) gastric volumes were measured by magnetic resonance imaging (MRI) in 20 healthy people and 17 with functional dyspepsia; 7 had normal and 10 had rapid gastric emptying. In 17 healthy people and patients, contractility was analyzed by spectral analysis of a time-series of gastric cross-sectional areas. Logistic regression models analyzed whether contractile parameters, fasting volume, and postprandial volume change could discriminate between health and patients with normal or rapid gastric emptying. Results While upper gastrointestinal symptoms were comparable, patients with rapid emptying had a higher (p = 0.002) body mass index (BMI) than normal gastric emptying. MRI visualized propagating contractions at ~ 3 cpm in healthy people and patients. Compared to controls (0.16 ± 0.02, Mean ± SEM), the amplitude of gastric contractions in the entire stomach was higher (OR 4.1, 95% CI 1.2–14.0) in patients with rapid (0.24 ± 0.03) but not normal gastric emptying (0.10 ± 0.03). Similar differences were observed in the distal stomach. However, the propagation velocity, fasting gastric volume, and the postprandial volume change were not significantly different between patients and controls. Conclusions MRI provides a noninvasive and refined assessment of gastric volumes and contractility in humans. Increased gastric contractility may contribute to rapid gastric emptying in functional dyspepsia. PMID:21470342

  7. Craniofacial surgical management of a patient with systematic juvenile idiopathic arthritis and Crohn's disease.

    PubMed

    Kasfikis, Georgios; Georgios, Kasfikis; Antoniades, Helias; Helias, Antoniades; Kyrgidis, Athanassios; Athanassios, Kyrgidis; Markovitsi, Eleni; Eleni, Markovitsi; Antoniades, Konstantinos; Konstantinos, Antoniades

    2009-05-01

    Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory disease in early age. It affects one or more joints, lasts more than 3 weeks, and appears in patients younger than 16 years. Juvenile idiopathic arthritis is classified according to the International League of Associations for Rheumatology consensus depending on the number of affected joints in the beginning of the disease. When JIA affects the temporomandibular joint, the development of the mandible is constrained. Patients show a tendency toward retrognathism and a vertical facial development pattern. The purpose of this study was to present a rare case of a young teenager who experienced JIA and Crohn's disease at the same time. The patient was referred to the hospital for aesthetic and functional problems, mainly convex facial profile and obstructive sleep apnea caused by the craniofacial abnormality. The patient was treated by sagittal split mandibular advancement osteotomy and advancement genioplasty. The mechanisms of obstructive sleep apnea development and the surgical treatment through osteotomies are commentated on. The surgical outcome is functionally and aesthetically favorable and solid 2 years after the operation. Surgical management of the craniofacial region can be a problem-solving treatment modality for patients with juvenile arthritis. PMID:19461338

  8. Clinical Response of Levodopa Carbidopa Combination in Patients with Idiopathic Parkinsonism

    PubMed Central

    Pillai, Pradeep Sadasivan; Iype, Thomas

    2016-01-01

    Introduction Parkinson’s disease is the most common form of a group of progressive neurodegenerative disorders. The use of levodopa as dopamine – replacement therapy is highly effective in ameliorating the symptoms of the disease and remains the standard drug with which other therapies are compared. Aim To study the change in Unified Parkinson’s Disease Rating scale (UPDRS) scores in patients receiving levodopa and carbidopa treatment (levodopa- carbidopa combination). Materials and Methods Study was conducted in Department of Neurology, Government Medical College, Trivandrum, India on 75 patients. All patients diagnosed with Idiopathic Parkinson’s disease (PD) satisfying inclusion criteria were enrolled into the study. Informed written consent was taken from all patients. Baseline UPDRS scores were recorded followed by reassessment at the end of six month. Data was analysed using paired t-test with help of SPSS-16 statistical software. Results Baseline UPDRS was collected and after 6 months of treatment, it was reassessed. Baseline total score was 49.8; the follow-up score was 39.5. A decrease in score was seen in various components of UPDRS. Conclusion Upon statistical analysis this difference was found to be significant, which implies that, there is improvement in patient’s condition. Improvement was noted in Mentation, behaviour, mood, activities of daily living and motor functions. Hence there is positive treatment response for levodopa carbidopa therapy in patients with idiopathic PD. PMID:27437241

  9. [Early postoperative complications after scoliosis surgery].

    PubMed

    Pérez-Caballero Macarrón, C; Burgos Flores, J; Martos Sánchez, I; Pérez Palomino, A; Vázquez Martínez, J L; Alvarez Rojas, E; Fernández Pineda, L; Vellibre Vargas, D

    2006-03-01

    Several medical complications can occur after scoliosis surgery in children and adolescents. New surgical techniques have allowed greater degrees of scoliosis correction but have also increased the possibility of postsurgical deficit due to their greater aggressivity. We analyzed the early postsurgical complications of scoliosis surgery in a pediatric intensive care unit over a 10-year period. Seventy-six surgical procedures were performed. Of these, no complications occurred in 55 (73%). Chest X-ray revealed pulmonary atelectasia in 8 patients (10%) and pleural effusion in 7 patients (9%). Symptoms and signs of infection related to surgery were observed in only 6 children (8%). The absence of severe medical complications may be related to new surgical techniques and an experienced team. PMID:16527092

  10. Reduction of radiation dose in radiologic examination of patients with scoliosis.

    PubMed

    Hellström, G; Irstam, L; Nachemson, A

    1983-01-01

    In an attempt to reduce the radiation dose during the examination of scoliotic patients, several screen-film combinations have been compared with a conventional system used at present. Kodak's Lanex Regular screen with Kodak Ortho H film enables the dose to be reduced eight times without significant deterioration of the image quality. The dose to the mammary glands can be reduced further by a factor of five if posterior--anterior instead of anterior--posterior projection is used. PMID:6867854

  11. Idiopathic histaminergic angioedema without wheals: a case series of 31 patients.

    PubMed

    Faisant, C; Boccon-Gibod, I; Mansard, C; Dumestre Perard, C; Pralong, P; Chatain, C; Deroux, A; Bouillet, L

    2016-07-01

    Idiopathic histaminergic acquired angioedema (IH-AAE) is a common cause of recurrent angioedema without wheals. It is a mast cell-mediated disease thought to belong to the same clinical entity as chronic urticaria (CU). The objective of this study was to describe the clinical and epidemiological characteristics of IH-AAE patients. From 2014 to 2015, 534 patients were seen at our national reference centre for angioedema and/or urticaria. Among them, we identified 31 patients with idiopathic histaminergic acquired angioedema without wheals (IH-AAE). Thirty-one patients (15 men and 16 women) with a mean age of 50 years met the criteria for IH-AAE. The average delay in diagnosis was 6·3 years. A history of allergy was found in 12 patients (38·7%), nine suffering from allergic rhinitis. The mean duration of attacks was 28·1 h. The AE attack was located in the upper respiratory tract in 54·8% of cases (17 patients). A lingual location was found in 29% of patients. Men were more likely than women to have an upper airway involvement. No intubations or admissions to intensive care units were reported. The dosage of anti-histamines to control the symptoms was onefold the recommended dose in 51·6% of patients (16 patients), twofold in 32% (10 patients) and three-fourfold in 16·1% (five patients). IH-AAE is characterized by an important delay in diagnosis, a frequent involvement of the upper airway and a benign course during attacks. As in CU, a trial of up to fourfold dose of H1-anti-histamines may be necessary to control symptoms. PMID:26969870

  12. Microtubule Destabilization Is Shared by Genetic and Idiopathic Parkinson’s Disease Patient Fibroblasts

    PubMed Central

    Cartelli, Daniele; Goldwurm, Stefano; Casagrande, Francesca; Pezzoli, Gianni; Cappelletti, Graziella

    2012-01-01

    Data from both toxin-based and gene-based models suggest that dysfunction of the microtubule system contributes to the pathogenesis of Parkinson’s disease, even if, at present, no evidence of alterations of microtubules in vivo or in patients is available. Here we analyze cytoskeleton organization in primary fibroblasts deriving from patients with idiopathic or genetic Parkinson’s disease, focusing on mutations in parkin and leucine-rich repeat kinase 2. Our analyses reveal that genetic and likely idiopathic pathology affects cytoskeletal organization and stability, without any activation of autophagy or apoptosis. All parkinsonian fibroblasts have a reduced microtubule mass, represented by a higher fraction of unpolymerized tubulin in respect to control cells, and display significant changes in microtubule stability-related signaling pathways. Furthermore, we show that the reduction of microtubule mass is so closely related to the alteration of cell morphology and behavior that both pharmacological treatment with microtubule-targeted drugs, and genetic approaches, by transfecting the wild type parkin or leucine-rich repeat kinase 2, restore the proper microtubule stability and are able to rescue cell architecture. Taken together, our results suggest that microtubule destabilization is a point of convergence of genetic and idiopathic forms of parkinsonism and highlight, for the first time, that microtubule dysfunction occurs in patients and not only in experimental models of Parkinson’s disease. Therefore, these data contribute to the knowledge on molecular and cellular events underlying Parkinson’s disease and, revealing that correction of microtubule defects restores control phenotype, may offer a new therapeutic target for the management of the disease. PMID:22666358

  13. Clinical characteristics of idiopathic pulmonary fibrosis patients according to their smoking status

    PubMed Central

    Nagano, Hiroaki; Nei, Yuichiro; Yamashiro, Shin

    2016-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonias (IIPs) of unknown etiology that often affects male, elderly smokers. However, it is sometimes observed in never smokers. This study aimed to clarify the clinical characteristics of IPF in never-smoking patients compared with those in smoking patients. Methods We retrospectively reviewed medical records, pulmonary function tests, and chest high-resolution computed tomography (HRCT) scan of never-smoking and smoking IPF patients from July 1, 2008 to June 30, 2013 at our hospital. Results We identified 32 never-smoking IPF patients and 66 smoking IPF patients. Never-smoking IPF patients developed more acute exacerbation (AE) than smoking IPF patients (50% vs. 18.2%, P<0.0001). The strongest predictor of AE in never-smoking IPF was modified Medical Research Council (mMRC) breathlessness scale [Hazards ratio (HR), 2.84, P=0.006]. The median survival time of never-smoking and smoking were 18.5 (0.1–138) and 26.3 (0.1–98.4) months, P<0.0001, respectively. The Cox proportional hazard model showed that 1-year mMRC breathlessness scale (HR, 3.24, P=0.001) and gender, age, and physiology (GAP) score (HR, 1.59, P=0.029) were strong predictors of mortality in never-smoking IPF patients at our hospital. Conclusions In conclusion, never-smoking IPF patients developed AE more often and showed poor prognosis compared with smoking IPF patients. The 1-year mMRC breathlessness scale was an important predictor of mortality at our hospital. PMID:27293827

  14. Syndromic 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 ...

  15. Adult 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 ...

  16. Congenital 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 ...

  17. Comparison of the efficacy of olopatadine and fexofenadine in chronic idiopathic urticaria patients: a crossover study.

    PubMed

    Tanizaki, Hideaki; Yamamoto, Yosuke; Nakamizo, Satoshi; Otsuka, Atsushi; Miyachi, Yoshiki; Kabashima, Kenji

    2015-01-01

    Olopatadine is one of the second-generation H1 antihistamines that were used for treating allergic disorders initially in Asia, and now also in Western countries. Whereas several trials compared the efficacy on chronic urticaria among second-generation H1 antihistamines, no study has directly compared the clinical efficacy between olopatadine and other H1 antihistamines in patients with chronic idiopathic urticaria (CIU). In this study, we address this issue for the first time and conclude that olopatadine is a good candidate for the treatment of CIU. PMID:25592003

  18. Invasive pulmonary aspergillosis in a patient presenting with idiopathic systemic capillary leak syndrome

    PubMed Central

    Hayama, Manabu; Shime, Nobuaki; Mio, Tadashi

    2014-01-01

    A 54-year-old man presented to our emergency department with fever and dyspnoea. He required vigorous haemodynamic support and mechanical ventilation for hypotensive distributive shock with hypoalbuminaemia, haemoconcentration, rhabdomyolysis and acute renal failure, consistent with idiopathic systemic capillary leak syndrome. Left lung consolidation and hypoxaemia were observed 6 days after admission. Sputum smear revealed the presence of acute angled branching hyphae, consistent with a diagnosis of invasive pulmonary aspergillosis. Antifungal therapy was administered and mechanical ventilation discontinued on day 66. The patient recovered and was discharged from the hospital on day 185. PMID:24859554

  19. Idiopathic pseudoaneurysm in a patient with breast implants.

    PubMed

    Khorasani, Hoda; Eiberg, Jonas; Bigaard, Janne

    2016-01-01

    Aneurysms and pseudoaneurysms of the internal mammary artery (IMA) are rare and the etiology uncertain although iatrogenic causes have been described. A 64-year-old woman suffered hematoma and bleeding from a pseudoaneurysm probably originating from a branch of the right IMA 16 years after breast implant surgery. After clinical assessment, the patient underwent surgery with resection of the aneurysm and ligation of the arterial branch. No early postoperative complications were reported. Pseudoaneurysms in small vessels such as the IMA or its branches are not well described in the literature. Proper diagnosis with imaging is of importance in order to determine treatment as each case is unique. Some cases can be managed conservatively. PMID:27470014

  20. Idiopathic pseudoaneurysm in a patient with breast implants

    PubMed Central

    Khorasani, Hoda; Eiberg, Jonas; Bigaard, Janne

    2016-01-01

    Aneurysms and pseudoaneurysms of the internal mammary artery (IMA) are rare and the etiology uncertain although iatrogenic causes have been described. A 64-year-old woman suffered hematoma and bleeding from a pseudoaneurysm probably originating from a branch of the right IMA 16 years after breast implant surgery. After clinical assessment, the patient underwent surgery with resection of the aneurysm and ligation of the arterial branch. No early postoperative complications were reported. Pseudoaneurysms in small vessels such as the IMA or its branches are not well described in the literature. Proper diagnosis with imaging is of importance in order to determine treatment as each case is unique. Some cases can be managed conservatively. PMID:27470014

  1. Scoliosis quantification: an overview

    PubMed Central

    Kawchuk, Greg; McArthur, Ross

    1997-01-01

    Scoliotic curvatures have long been a focus of attention for clinicians and research scientists alike. The study, treatment and ultimately, the prevention of this prevalent health condition are impeded by the absence of an accurate, reliable, convenient and safe method of scoliosis quantification. The purpose of this paper is to provide an overview of the current methods of scoliosis quantification for clinicians who address this condition in their practices.

  2. Bone morphogenetic protein receptor 2 in patients with idiopathic portal hypertension

    PubMed Central

    De Gottardi, Andrea; Seijo, Susana; Milá, Montserrat; Alvarez, M Isabel; Bruguera, Miquel; Abraldes, Juan G; Bosch, Jaime; García-Pagán, Juan-Carlos

    2012-01-01

    In idiopathic portal hypertension (IPH) typical vascular lesions are present in the branches of the portal vein or in the perisinusoidal area of the liver. Similar histological alterations have been reported in the pulmonary vasculature of patients with idiopathic pulmonary artery hypertension (IPAH). As IPAH is associated with mutations of the bone morphogenetic protein receptor 2 (BMPR2) gene, the aim of this study was to investigate whether this association might also be found in patients with IPH. Twenty-three samples belonging to 21 unrelated caucasian patients with IPH followed in the hepatic haemodynamic laboratory of the Hospital Clinic in Barcelona were included in the study. All patients were studied for the entire open reading frame and splice site of the BMPR2 gene by direct sequencing and multiple ligation probe amplification (MLPA) in order to detect large deletions/duplications. None of the 23 patients had pulmonary artery hypertension. Four patients presented one single nucleotide polymorphism (SNP) in intron 5, four patients had a SNP in exon 12 and a SNP in exon 1 was found in two cases. Two patients had both intron 5 and exon 12 polymorphisms. All SNPs were previously described. Except for these three SNPs, neither mutations nor rearrangements have been identified in the BMPR2 gene in this population. We did not detect mutations or rearrangements in the coding region of the BMPR2 gene in our patients with IPH. These findings suggest that, in contrast to IPAH, mutations in BMPR2 are not involved in the pathogenesis of IPH. PMID:22129439

  3. 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

  4. Depletion of B Lymphocytes in Idiopathic Membranous Glomerulopathy: Results from Patients with Extended Follow-Up

    PubMed Central

    Lionaki, Sophia; Marinaki, Smaragdi; Nakopoulou, Lydia; Skalioti, Chrysanthi; Iniotaki, Aliki; Sfikakis, Petros P.; Siamopoulos, Costas; Boletis, John

    2013-01-01

    Aims To assess the long-term therapeutic benefit of temporary depletion of B lymphocytes in patients with idiopathic membranous glomerulopathy (MGN) and search for potential predictors of response. Patients and Methods The patients included had been diagnosed with biopsy-proven MGN in the absence of secondary causes. Estimated glomerular filtration rate should be above 30 ml/min/1.73 m2 and 24-hour proteinuria 3 g/day or more. Patients who had been treated with cyclosporine or cytotoxic agents the year prior to study entry were excluded. Depletion of B cells was achieved with rituximab, which was administered intravenously for 4 consecutive weeks. Partial remission was defined as a >50% decrease in proteinuria with absolute proteinuria <3 g/day, while complete remission was defined as a >50% decrease in proteinuria and an absolute protein excretion <0.3 g/day. Results Twelve patients were studied (4 females/8 males) with a mean age of 51.3 years. No major adverse effects were observed. During a median follow-up time of 48 months, 11/12 (91.6%) patients achieved remission [7/12 (58.3%) complete remission and 4/12 (33.3%) partial remission], while 1 patient did not respond to therapy. Twelve months after therapy, 68.8% (p = 0.003) of cases had achieved partial and 28.4% complete remission. Measurements of lymphocyte subpopulations did not reveal any changes except for the B cell depletion. B cell infiltrates captured per mm3 of renal tissue in the diagnostic biopsy did not correlate with subsequent response. Conclusion Depletion of B cells in idiopathic MGN was well tolerated and resulted in significant and long-lasting response rates in a series of 12 patients. PMID:23573072

  5. Paraspinous hemolymphangioma associated with adolescent scoliosis.

    PubMed

    Swarup, Ishaan; Bjerke-Kroll, Benjamin T; Cunningham, Matthew E

    2015-06-16

    Lymphangiomas are a group of benign malformations of the lymphatic system, and hemolymphangioma (or hemangiolymphangioma) is a rare congenital malformation of the lymphatic system with vascular involvement. These benign malformations are most commonly diagnosed at an early age, and may be present as a part of an associated syndrome. In this case report, we describe the first case of adolescent scoliosis associated with a large, paraspinous hemolymphangioma. A 15-year-old girl with an incidental finding of a paraspinous hemolymphangioma is presented along with her history, physical exam, radiographic findings, and operative course. The possible pathogenesis, treatment approach, and clinical dilemmas are also discussed. Given the well-known relationship between tumors and scoliosis, a benign paraspinous vascular and lymphatic tumor may be responsible for the presence of scoliosis in a small number of patients. PMID:26090372

  6. Scoliosis in Children and Adolescents

    MedlinePlus

    ... Questions and Answers about Scoliosis in Children and Adolescents This publication defines scoliosis and provides information about ... it is diagnosed and treated in children and adolescents. You may be interested in contacting one or ...

  7. Carcinoembryonic antigen in bronchoalveolar lavage fluid in patients with idiopathic pulmonary fibrosis.

    PubMed

    Takahashi, H; Nukiwa, T; Matsuoka, R; Danbara, T; Natori, H; Arai, T; Kira, S

    1985-08-01

    An increased incidence of lung cancer and epithelial metaplasia or hyperplasia which is felt to be as a precursor of cancer, has been reported in patients with idiopathic pulmonary fibrosis (IPF). In this study, carcinoembryonic antigen (CEA) in bronchoalveolar lavage (BAL) fluid was measured in 53 control patients, 31 patients with sarcoidosis, 10 patients with hypersensitivity pneumonitis, 16 patients with primary lung cancer and 26 patients with histologically confirmed IPF. High ratio of CEA to albumin (Alb), exceeding mean + 2SD of nonsmoking control patients, were found in 8 (25%) out of 32 smoking control patients, 4 (44%) out of 9 nonsmoking patients with IPF, 8 (62%) out of 13 smoking patients with IPF, 3 (75%) out of 4 smoking patients with IPF and lung cancer and 13 (81%) out of 16 patients with primary lung cancer, although BAL was performed at the noncancerous parts of the lung in the cases of lung cancer. Furthermore, it was confirmed that CEA increased in BAL fluid in these subjects were different from nonspecific cross-reacting antigen (NCA) which was detectable in the normal lung. Thus we consider that the increase of CEA/Alb ratio in BAL fluid is a possible marker of these early histological disorders in the lung, and also suggests a greater risk of malignant change in the clinical course of IPF. PMID:4068359

  8. Dental Management of a Patient with Multiple Idiopathic Cervical Root Resorption.

    PubMed

    Ali, Rahat; Fayle, Stephen; Langley, David; Altaie, Asmaa; Nattress, Brian

    2015-09-01

    Multiple Idiopathic Cervical Root Resorption (MICRR) is a rare condition. It initiates at the cemento-enamel junction of multiple teeth. The lesions continue to grow until they unite, thereby undermining the entire coronal structure of affected teeth. Its distribution can vary from a single region to the entire dentition and the number of teeth affected by resorption tends to increase as the condition is followed over time. The teeth themselves appear clinically normal. The aetiology of MICRR is unknown and it is considered to be a diagnosis of exclusion. The condition tends to be progressive. Consequently, root treatments/surgical curettage and restoration of the lesions have been unsuccessful at arresting the condition. Affected teeth are often extracted in anticipation of catastrophic fracture and have been replaced with partial or complete dentures. In this case report, we describe how a young female patient was dentally managed over 10 years and ultimately rehabilitated with dental implants. CPD/CLINICAL RELEVANCE: Patients suspected of having multiple idiopathic cervical root resorption may require specialist, multidisciplinary care and require referral to an appropriate secondary care unit for treatment planning and potential oral rehabilitation. PMID:26630864

  9. Peliosis hepatis presenting with massive hepatomegaly in a patient with idiopathic thrombocytopenic purpura.

    PubMed

    Kim, Sun Bean; Kim, Do Kyung; Byun, Sun Jeong; Park, Ji Hye; Choi, Jin Young; Park, Young Nyun; Kim, Do Young

    2015-12-01

    Peliosis hepatis is a rare condition that can cause hepatic hemorrhage, rupture, and ultimately liver failure. Several authors have reported that peliosis hepatis develops in association with chronic wasting disease or prolonged use of anabolic steroids or oral contraceptives. In this report we describe a case in which discontinuation of steroid therapy improved the condition of a patient with peliosis hepatis. Our patient was a 64-year-old woman with a history of long-term steroid treatment for idiopathic thrombocytopenic purpura . Her symptoms included abdominal pain and weight loss; the only finding of a physical examination was hepatomegaly. We performed computed tomography (CT) and magnetic resonance imaging (MRI) of the liver and a liver biopsy. Based on these findings plus clinical observations, she was diagnosed with peliosis hepatis and her steroid treatment was terminated. The patient recovered completely 3 months after steroid discontinuation, and remained stable over the following 6 months. PMID:26770928

  10. Idiopathic Hyperammonemia That Developed During Initial Treatment With Steroid in a Patient With Newly Diagnosed Leukemia.

    PubMed

    Kobayashi, Shogo; Ito, Masaki; Sano, Hideki; Mochizuki, Kazuhiro; Akaihata, Mitsuko; Waragai, Tomoko; Ohara, Yoshihiro; Hosoya, Mitsuaki; Kikuta, Atsushi

    2015-08-01

    Idiopathic hyperammonemia (IHA) has been described as a complication of intensive chemotherapy for the treatment of hematologic malignancy but has subsequently been found in patients undergoing bone marrow transplantation and in those with solid tumors treated with 5-fluorouracil. Although IHA is a rare complication, it is sometimes associated with high mortality in hematologic malignancies. Here we report the case of a 15-year-old boy in whom hyperammonemia developed during the initial treatment with prednisolone for newly diagnosed acute lymphoblastic leukemia and who survived after early detection and oral lactulose therapy. To the best of our knowledge, this is the first report of IHA that was not induced by intensive chemotherapy, stem cell transplantation, or asparaginase therapy in a patient with newly diagnosed leukemia, but developed during an initial treatment with a steroid. Early detection of IHA by measuring the plasma ammonia level in patients with neurological symptoms may improve the outcome. PMID:25222063

  11. Human experts' and a fuzzy model's predictions of outcomes of scoliosis treatment: a comparative analysis.

    PubMed

    Chalmers, Eric; Pedrycz, Witold; Lou, Edmond

    2015-03-01

    Brace treatment is the most commonly used nonsurgical treatment for adolescents with idiopathic scoliosis. However, brace treatment is not always successful and the factors influencing its success are not completely clear. This makes treatment outcome difficult to predict. A computer model which can accurately predict treatment outcomes could potentially provide valuable treatment recommendations. This paper describes a fuzzy system that includes a prediction model and a decision support engine. The model was constructed using conditional fuzzy c-means clustering to discover patterns in retrospective patient data. The model's ability to predict treatment outcome was compared to the ability of eight Scoliosis experts. The model and experts each predicted treatment outcome retrospectively for 28 braced patients, and these predictions were compared to the actual outcomes. The model outperformed all but one expert individually and performed similarly to the experts as a group. These results suggest that the fuzzy model is capable of providing meaningful treatment recommendations. This study offers the first model for this application whose performance has been shown to be at or above the human expert level. PMID:25494498

  12. Probable disseminated Mycobacterium abscessus subspecies bolletii infection in a patient with idiopathic CD4+ T lymphocytopenia: a case report

    PubMed Central

    2012-01-01

    Introduction Rapidly growing mycobacteria are opportunistic pathogens in patients with underlying risk factors. Mycobacterium abscessus subsp. bolletii is a newly recognized member of rapidly growing mycobacteria, isolated from respiratory tract and cutaneous infections. Case presentation We describe a case of chronic disseminated infection caused by M. abscessus subsp. bolletii in a 38-year-old Sri Lankan man with idiopathic CD4+ T lymphocytopenia. Idiopathic CD4+ T lymphocytopenia is a rare cause of immunodysfunction that, similar to human immunodeficiency virus infection, causes a depletion of CD4+ T lymphocytes. M. abscessus subsp. bolletii infection was diagnosed by culture isolation from two sputum samples. Conclusions To the best of our knowledge this is the first report of M. abscessus subsp. bolletii disseminated infection in a patient affected by idiopathic CD4+ T lymphocytopenia. In contrast to previous reports, the isolate of M. abscessus subsp. bolletii presented intermediate resistance to clarithromycin and was susceptible to cefoxitin and imipenem. PMID:22947084

  13. Impact of Helicobacter pylori Eradication Therapy on Platelet Counts in Patients With Chronic Idiopathic Thrombocytopenic Purpura

    PubMed Central

    Amiri, Mohamadreza

    2016-01-01

    This study was a before and after clinical evaluation of Helicobacter pylori eradication on platelet counts in a group of 23 patients with chronic Idiopathic (Autoimmune) thrombocytopenic purpura (CITP). H. pylori infection was identified in patients by a 13C-urea breath test and confirmed by an H. pylori stool antigen test. Eradication was conducted in patients testing positive. Infected (n = 10) and uninfected (n = 13) patient groups did not differ with respect to age, gender, history of previous splenectomy, treatment with anti-D, current treatment with corticosteroids, or initial platelet counts. H. pylori eradication was successful in eight infected CITP patients, with two patients not responsive to treatment. Compared to the uninfected group, patients in the infected group who responded to eradication therapy had significantly increased platelet counts after six months (56.2 ± 22.2 vs. 233 ± 85.6 ×103 million cells/L; P < 0.01), whereas platelet counts in the non-responding patients and uninfected group did not differ after this period of time. H. pylori eradication promotes significant platelet count improvement in patients with CITP. Thus, all patients with CITP should be tested and treated for H. pylori infections. PMID:26925898

  14. Impact of Helicobacter pylori Eradication Therapy on Platelet Counts in Patients With Chronic Idiopathic Thrombocytopenic Purpura.

    PubMed

    Amiri, Mohamadreza

    2016-01-01

    This study was a before and after clinical evaluation of Helicobacter pylori eradication on platelet counts in a group of 23 patients with chronic Idiopathic (Autoimmune) thrombocytopenic purpura (CITP). H. pylori infection was identified in patients by a (13)C-urea breath test and confirmed by an H. pylori stool antigen test. Eradication was conducted in patients testing positive. Infected (n = 10) and uninfected (n = 13) patient groups did not differ with respect to age, gender, history of previous splenectomy, treatment with anti-D, current treatment with corticosteroids, or initial platelet counts. H pylori eradication was successful in eight infected CITP patients, with two patients not responsive to treatment. Compared to the uninfected group, patients in the infected group who responded to eradication therapy had significantly increased platelet counts after six months (56.2 ± 22.2 vs. 233 ± 85.6 ×10(3) million cells/L; P < 0.01), whereas platelet counts in the non-responding patients and uninfected group did not differ after this period of time. H. pylori eradication promotes significant platelet count improvement in patients with CITP. Thus, all patients with CITP should be tested and treated for H. pylori infections. PMID:26925898

  15. Transient Monoplegia as a Result of Unilateral Femoral Artery Ischemia Detected by Multimodal Intraoperative Neuromonitoring in Posterior Scoliosis Surgery: A Case Report.

    PubMed

    Pankowski, Rafal; Roclawski, Marek; Dziegiel, Krzysztof; Ceynowa, Marcin; Mikulicz, Marcin; Mazurek, Tomasz; Kloc, Wojciech

    2016-02-01

    This is to report a case of 16-year-old girl with transient right lower limb monoplegia as a result of femoral artery ischemia detected by multimodal intraoperative spinal cord neuromonitoring (MISNM) during posterior correction surgery of adolescent idiopathic scoliosis.A patient with a marfanoid body habitus and LENKE IA type scoliosis with the right thoracic curve of 48° of Cobb angle was admitted for posterior spinal fusion from Th6 to L2. After selective pedicle screws instrumentation and corrective maneuvers motor evoked potentials (MEP) began to decrease with no concomitant changes in somato-sensory evoked potentials recordings.The instrumentation was released first partially than completely with rod removal but the patient demonstrated constantly increasing serious neurological motor deficit of the whole right lower limb. Every technical cause of the MEP changes was eliminated and during the wake-up test the right foot was found to be pale and cold with no popliteal and dorsalis pedis pulses palpable. The patient was repositioned and the pelvic pad was placed more cranially. Instantly, the pulse and color returned to the patient's foot. Following MEP recordings showed gradual return of motor function up to the baseline at the end of the surgery, whereas somato-sensory evoked potentials were within normal range through the whole procedure.This case emphasizes the importance of the proper pelvic pad positioning during the complex spine surgeries performed in prone position of the patient. A few cases of neurological complications have been described which were the result of vascular occlusion after prolonged pressure in the inguinal area during posterior scoliosis surgery when the patient was in prone position. If incorrectly interpreted, they would have a significant impact on the course of scoliosis surgery. PMID:26871822

  16. Chromosome abnormality rate among Iranian patients with idiopathic mental retardation from consanguineous marriages

    PubMed Central

    Behjati, Farkhondeh; Ghasemi Firouzabadi, Saghar; Kahrizi, Kimia; Kariminejad, Roxana; Bagherizadeh, Iman; Ansari, Javad; Fallah, Masoumeh; Mojtahedi, Forough; Darvish, Hossein; Bahrami Monajemi, Gholamreza; Abedini, S. Sedigheh; Jamali, Payman; Mojahedi, Faezeh; Zadeh-Vakili, Azita; Najmabadi, Hossein

    2011-01-01

    Introduction Mental retardation (MR) has heterogeneous aetiology mostly with genetic causes. Chromosomal aberrations are one of the most common causes of MR. Reports on chromosome abnormality rate among consanguineous families are sparse. In order to identify the chromosome abnormality rate in idiopathic mental retardation from consanguineous marriages, a total of 322 Iranian families with positive family history for MR were investigated in the Genetics Research Center. Material and methods In the majority of families (92%) at least two sibs were affected with MR and none had specific chromosomal syndromes such as Down syndrome. Standard cytogenetic techniques using high resolution GTG banding were carried out on all the patients. Results The overall chromosome abnormality rate contributing to mental retardation was 1.24% (4 cases), which comprised 46,XY,der(18)t(4;18)(q31.1;q23)mat; 45,XY,-21,-22,+der(22)t(21;22)(q21.1;q13.33)mat; 46,XY,rec(2)dup(2p)inv(2)(p25.1q37.3)pat, and 46,XY,der(11)t(10;11)(q25.2;q25)pat. Conclusions Although the most likely genetic cause of mental retardation in patients with consanguineous parents is autosomal recessive, the fact that 1.24% of our patients had chromosomal abnormalities emphasizes the importance of cytogenetic investigation as the first laboratory genetic tests for all MR patients. To our knowledge, this is the first report on the rate of chromosome abnormality among patients with idiopathic mental retardation from consanguineous marriages. PMID:22291774

  17. Factors influencing the quality of life of Moroccan patients with juvenile idiopathic arthritis.

    PubMed

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

    2014-11-01

    The aim of our study is to investigate the factors influencing the quality of life, assessed by the Pediatric Quality of Life Inventory 4.0 (PedsQL4) Generic Score Scales, in Moroccan patients with juvenile idiopathic arthritis. This is a cross-sectional study conducted between January and June 2012, covering children with juvenile idiopathic arthritis (JIA) seen at the consultations of El Ayachi Hospital and Children's Hospital of the University Hospital of Rabat. Quality of life is assessed by the PedsQL4 which is a questionnaire composed of 23 items, completed by the child and the parent; the response to each item ranges from 0 to 100, so that higher scores indicate a better quality of life. The functional impact is assessed by the Childhood Health Assessment Questionnaire (CHAQ), and the disease activity by the number of tender and swollen joints, visual analogue scale (VAS) activity, erythrocyte sedimentation rate (ESR), and C-reactive protein. Forty-seven patients are included; the average age of the patients is 11 ± 3.35 years, and 40.4 % are females, with a median disease duration of 4 (2; 6) years. The oligoarticular form presents 26.7 %, the systemic form 24.4 %, and the enthesic form 22.2 %. The median of PedsQL4 is 80.43 (63.19; 92.93), and the median of the CHAQ is 0 (0; 1). Our study shows that some clinical and biological characteristics have significant effects on PedsQL by both parent and child reports. This study suggests that the achievement of the quality of life of our patients with JIA depends on the disease activity measured by swollen joints, the number of awakenings, parent VAS, physician VAS, patient VAS, and the ESR. PMID:24445385

  18. Animal models of scoliosis.

    PubMed

    Bobyn, Justin D; Little, David G; Gray, Randolph; Schindeler, Aaron

    2015-04-01

    Multiple techniques designed to induce scoliotic deformity have been applied across many animal species. We have undertaken a review of the literature regarding experimental models of scoliosis in animals to discuss their utility in comprehending disease aetiology and treatment. Models of scoliosis in animals can be broadly divided into quadrupedal and bipedal experiments. Quadrupedal models, in the absence of axial gravitation force, depend upon development of a mechanical asymmetry along the spine to initiate a scoliotic deformity. Bipedal models more accurately mimic human posture and consequently are subject to similar forces due to gravity, which have been long appreciated to be a contributing factor to the development of scoliosis. Many effective models of scoliosis in smaller animals have not been successfully translated to primates and humans. Though these models may not clarify the aetiology of human scoliosis, by providing a reliable and reproducible deformity in the spine they are a useful means with which to test interventions designed to correct and prevent deformity. PMID:25492698

  19. Successful use of rituximab in refractory idiopathic thrombocytopenic purpura in a patient with common variable immunodeficiency.

    PubMed

    Al-Ahmad, M; Al-Rasheed, M; Al-Muhani, A

    2010-01-01

    Idiopathic thrombocytopenic purpura (ITP) is a common autoimmune disease in patients with common variable immunodeficiency (CVID). We describe a 36-year-old woman with CVID. The clinical course of her disease was complicated by bronchiectasis, antiphospholipid antibody syndrome, and portal vein thrombosis. She developed recurrent attacks of ITP refractory to high doses of corticosteroid, intravenous immunoglobulin (IVIG), and splenectomy. She received a total of 5 doses of rituximab (375 mg/m2) and achieved an immediate and persistent response. Therapy was well tolerated. Her platelet count remained above 370,000/microL for 8 months of follow-up, despite repeated infections. During this period the patient remained off corticosteroids and on continuous IVIG replacement therapy. PMID:20635793

  20. [Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].

    PubMed

    Tavares, Anna Carolina Faria Moreira Gomes; Ferreira, Gilda Aparecida; Guimarães, Luciano Junqueira; Guimarães, Raquel Rosa; Santos, Flávia Patrícia Sena Teixeira

    2015-01-01

    Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol. PMID:25575650

  1. Imaging in scoliosis from the orthopaedic surgeon's point of view.

    PubMed

    Thomsen, Marc; Abel, Rainer

    2006-04-01

    For treating patients with scoliosis orthopaedic surgeons need diagnostic imaging procedures in order to provide answers about a possible underlying disease, choice of treatment, and prognosis. Once treatment is instituted, imaging is also critical for monitoring changes of the deformity so as to optimize therapy. The combined effort of orthopaedic surgeons and radiologists helps detect treatable causes of scoliosis at an early stage, define the need and timing for surgery, and ensure that every precaution is taken to minimize the risks of surgery. Neurosurgical causes, with particular reference to spinal cord tumours and syringomyelia, need to be addressed before scoliosis surgery can be contemplated. PMID:16439089

  2. Presence of Idiopathic Thrombocytopenic Purpura and autoimmune hemolytic anemia in the patients with common variable immunodeficiency.

    PubMed

    Ramyar, Asghar; Aghamohammadi, Asghar; Moazzami, Kasra; Rezaei, Nima; Yeganeh, Mehdi; Cheraghi, Taher; Pouladi, Nima; Heydari, Golnaz; Abolhassani, Hassan; Amirzargar, Ali Akbar; Parvaneh, Nima; Moin, Mostafa

    2008-09-01

    Common Variable Immunodeficiency (CVID) is a heterogeneous group of disorders characterized by hypogammaglobulinemia and an increased susceptibility to recurrent infections as well as autoimmunity and malignancies. Idiopathic Thrombocytopenic Purpura (ITP) and Autoimmune Hemolytic Anemia (AIHA) are two autoimmune disorders which may be seen in association with CVID. Among 85 CVID patients, seven cases had ITP and/or AIHA (8%). Four of these patients had one or more episodes of ITP, one patient had AIHA, and two patients had both ITP and AIHA (Evans syndrome). Almost, all patients experienced chronic and recurrent infections mostly in respiratory and gastrointestinal systems during the course of the disease. Among the seven patients, five presented their underlying disease with recurrent respiratory and/or gastrointestinal tract infections, while in two remaining patients, CVID was presented with ITP. Three patients died until now; two because of hepatic failure and one due to pulmonary hemorrhage. As CVID is prone to autoimmune disorders, it should be considered as a differential diagnosis of adult-onset ITP and possibly in children. Chronic and recurrent ITP, especially in the presence of propensity to respiratory and gastrointestinal infections mandate the evaluation for an underlying immune dysregulation such as CVID. PMID:18780952

  3. Juvenile Idiopathic Inflammatory Myopathy in a Patient With Dyskeratosis Congenita Due to C16orf57 Mutation.

    PubMed

    Kilic, Sara S; Cekic, Sukru

    2016-03-01

    Dyskeratosis congenita (DC) is a rare inherited disorder characterized by reticular skin pigmentation, oral cavity leukoplakia, and nail dystrophy. A variety of noncutaneous (dental, pulmonary, gastrointestinal, neurological, genitourinary, ophthalmic, and skeletal) abnormalities also have been reported. An 8-year-old boy with DC developed juvenile idiopathic inflammatory myopathy. C16orf57 mutation was identified as a genetic cause of DC. Treatment with methylprednisolone was initiated, followed with methotrexate, prednisolone, and high-dose intravenous immunoglobulin treatment. This is the first report on a patient with juvenile idiopathic inflammatory myopathy and DC. PMID:26535771

  4. Arthroscopic correction for concomitant cam impingement in a patient with idiopathic osteonecrosis of the femoral head: A case report

    PubMed Central

    Fukui, Kiyokazu; Kaneuji, Ayumi; Matsumoto, Tadami

    2015-01-01

    A 53-year-old man presented with pain in the right hip. Radiological examination showed idiopathic osteonecrosis of the femoral head (ONFH) combined with a cam lesion. Findings on physical examination were consistent for femoroacetabular impingement. At surgery, we performed isolated arthroscopic correction for the cam lesion but did not use other treatment options such as hip arthroplasty or osteotomies for the ONFH. At the latest follow-up evaluation 3 years after surgery, findings indicted a satisfactory outcome, with a Harris hip score of 93.2 (compared with 76.4 before surgery), no joint-space narrowing, and no collapse of the femoral head. It is important to accurately diagnose the status of idiopathic ONFH and to consider another possible pathogenesis when a patient with idiopathic ONFH has hip pain even without femoral-head collapse. PMID:26773875

  5. Screening for Scoliosis: Different Countries' Perspectives and Evidence-Based Health Care

    ERIC Educational Resources Information Center

    Plaszewski, Maciej; Nowobilski, Roman; Kowalski, Pawel; Cieslinski, Maciej

    2012-01-01

    Idiopathic scoliosis affects 2-3% of adolescents. Large, progressing deformities, mostly present in girls, may lead to pulmonary complications, pain symptoms, the feeling of social isolation, and even mental disorders. The correlation of screening programs with surgery rate reduction and the clinical effectiveness of bracing remain a matter of…

  6. Vitamin D Receptor Gene Polymorphisms and Haplotypes in Hungarian Patients with Idiopathic Inflammatory Myopathy

    PubMed Central

    Griger, Zoltán; Dankó, Katalin

    2015-01-01

    Idiopathic inflammatory myopathies are autoimmune diseases characterized by symmetrical proximal muscle weakness. Our aim was to identify a correlation between VDR polymorphisms or haplotypes and myositis. We studied VDR-BsmI, VDR-ApaI, VDR-TaqI, and VDR-FokI polymorphisms and haplotypes in 89 Hungarian poly-/dermatomyositis patients (69 females) and 93 controls (52 females). We did not obtain any significant differences for VDR-FokI, BsmI, ApaI, and TaqI genotypes and allele frequencies between patients with myositis and healthy individuals. There was no association of VDR polymorphisms with clinical manifestations and laboratory profiles in myositis patients. Men with myositis had a significantly different distribution of BB, Bb, and bb genotypes than female patients, control male individuals, and the entire control group. Distribution of TT, Tt, and tt genotypes was significantly different in males than in females in patient group. According to four-marker haplotype prevalence, frequencies of sixteen possible haplotypes showed significant differences between patient and control groups. The three most frequent haplotypes in patients were the fbAt, FBaT, and fbAT. Our findings may reveal that there is a significant association: Bb and Tt genotypes can be associated with myositis in the Hungarian population we studied. We underline the importance of our result in the estimated prevalence of four-marker haplotypes. PMID:25649962

  7. [Anesthetic Management Using Transesophageal Echocardiography and EV1000 in a Patient with Ebstein's Anomary Undergoing Scoliosis Surgery].

    PubMed

    Tanimura, Kazuki; Miura, Yukiko; Ishii, Hisanari

    2016-02-01

    An 18-year-old female patinet with Ebstein anomaly underwent surgical repair of scoliosis under total intravenous anesthesia. In addtition to normal monitors, we used transesophageal echocardiography (TEE) and EV1000 (Edwards Lifesciences, Irvine, USA), which show stroke volume variation and stroke volume index simultaneously in a rectangular coordinates. TEE detected reversal of intracardiac shunt which caused SpO2 decrease during fixing screws at thoracic vertebrae, then manual ventilation with oxygen unproved SpO2. Because of a high venous pressure due to Ebstein anomaly, surgical bleeding seemed to be larger than usual. By using EV1000, volume status and cardiac contractility were estimated and adequate volume loading and inoptrope injection were performed to stabilize circulatory condition. The operation was completed without any cardiac and respiratory complications. PMID:27017770

  8. Revisiting scoliosis in the KNM-WT 15000 Homo erectus skeleton.

    PubMed

    Schiess, Regula; Boeni, Thomas; Rühli, Frank; Haeusler, Martin

    2014-02-01

    Owing to its completeness, the 1.5 million year old Nariokotome boy skeleton KNM-WT 15000 is central for understanding the skeletal biology of Homo erectus. Nevertheless, since the reported asymmetries and distortions of Nariokotome boy's axial skeleton suggest adolescent idiopathic scoliosis, possibly associated with congenital skeletal dysplasia, it is questionable whether it still can be used as a reference for H. erectus. Recently, however, the presence of skeletal dysplasia has been refuted. Here, we present a morphological and morphometric reanalysis of the assertion of idiopathic scoliosis. We demonstrate that unarticulated vertebral columns of non-scoliotic and scoliotic individuals can be distinguished based on the lateral deviation of the spinous process, lateral and sagittal wedging, vertebral body torsion, pedicle thickness asymmetry, and asymmetry of superior and inferior articular facet areas. A principal component analysis of the overall asymmetry of all seven vertebral shape variables groups KNM-WT 15000 within non-scoliotic modern humans. There is, however, an anomaly of vertebrae T1-T2 that is compatible with a short left convex curve at the uppermost thoracic region, possibly due to injury or local growth dysbalance. Asymmetries of the facet joints L3-L5 suggest a local right convex curve in the lower lumbar region that probably resulted from juvenile traumatic disc herniation. This pattern is incompatible with adolescent idiopathic scoliosis or other types of scoliosis, including congenital, neuromuscular or syndromic scoliosis. It is, however, consistent with a recent reanalysis of the rib cage that did not reveal any asymmetry. Except for these possibly trauma-related anomalies, the Nariokotome boy fossil therefore seems to belong to a normal H. erectus youth without evidence for adolescent idiopathic scoliosis or other severe pathologies of the axial skeleton. PMID:24491377

  9. Widefield OCT Findings of a Patient With Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis.

    PubMed

    Mandell, Jacqueline B; Kim, Alice Y; Shahidzadeh, Anoush; Ameri, Hossein; Puliafito, Carmen A; Moshfeghi, Andrew A

    2016-08-01

    The authors report extensive peripheral retinoschisis in a patient with stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) detected by widefield optical coherence tomography (OCT). A 64-year-old woman diagnosed with foveomacular retinoschisis 3 years prior presented for evaluation after being seen by multiple other retina specialists. Standard macular spectral-domain OCT (6 mm) revealed typical foveomacular schisis involving only the outer retina. However, widefield OCT (12 mm) revealed diffuse bilateral retinoschisis involving both inner and outer retinal layers in the macula and midperiphery. Widefield imaging is important to evaluate and monitor complex peripheral retinoschisis that may be otherwise undetectable using conventional techniques. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:774-777.]. PMID:27548456

  10. Nocardia transvalensis Disseminated Infection in an Immunocompromised Patient with Idiopathic Thrombocytopenic Purpura

    PubMed Central

    García-Méndez, Jorge; Carrillo-Casas, Erika M.; Rangel-Cordero, Andrea; Leyva-Leyva, Margarita; Xicohtencatl-Cortes, Juan; Arenas, Roberto; Hernández-Castro, Rigoberto

    2016-01-01

    Nocardia transvalensis complex includes a wide range of microorganisms with specific antimicrobial resistance patterns. N. transvalensis is an unusual Nocardia species. However, it must be differentiated due to its natural resistance to aminoglycosides while other Nocardia species are susceptible. The present report describes a Nocardia species involved in an uncommon clinical case of a patient with idiopathic thrombocytopenic purpura and pulmonary nocardiosis. Microbiological and molecular techniques based on the sequencing of the 16S rRNA gene allowed diagnosis of Nocardia transvalensis sensu stricto. The successful treatment was based on trimethoprim-sulfamethoxazole and other drugs. We conclude that molecular identification of Nocardia species is a valuable technique to guide good treatment and prognosis and recommend its use for daily bases diagnosis. PMID:27313917

  11. Nocardia transvalensis Disseminated Infection in an Immunocompromised Patient with Idiopathic Thrombocytopenic Purpura.

    PubMed

    García-Méndez, Jorge; Carrillo-Casas, Erika M; Rangel-Cordero, Andrea; Leyva-Leyva, Margarita; Xicohtencatl-Cortes, Juan; Arenas, Roberto; Hernández-Castro, Rigoberto

    2016-01-01

    Nocardia transvalensis complex includes a wide range of microorganisms with specific antimicrobial resistance patterns. N. transvalensis is an unusual Nocardia species. However, it must be differentiated due to its natural resistance to aminoglycosides while other Nocardia species are susceptible. The present report describes a Nocardia species involved in an uncommon clinical case of a patient with idiopathic thrombocytopenic purpura and pulmonary nocardiosis. Microbiological and molecular techniques based on the sequencing of the 16S rRNA gene allowed diagnosis of Nocardia transvalensis sensu stricto. The successful treatment was based on trimethoprim-sulfamethoxazole and other drugs. We conclude that molecular identification of Nocardia species is a valuable technique to guide good treatment and prognosis and recommend its use for daily bases diagnosis. PMID:27313917

  12. 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

  13. Treatment pattern in patients with idiopathic membranous nephropathy—practices in Sweden at the start of the millennium

    PubMed Central

    Lönnbro-Widgren, Jennie; Mölne, Johan; Haraldsson, Börje; Nyström, Jenny

    2016-01-01

    Background Idiopathic membranous nephropathy (MN) is one of the leading causes of nephrotic syndrome in adults and may result in end-stage renal disease (ESRD). In this retrospective study, we describe the outcomes and treatment patterns of patients with idiopathic MN in six nephrology clinics in the western part of Sweden. Methods Seventy-three consecutive patients with biopsy-proven MN in the years 2000–12 were classified as idiopathic, i.e. secondary forms were excluded. The patients were followed retrospectively for a mean period of 83 months and clinical data were collected through the medical files. Results A high proportion (88%) of the patients received supportive treatment with angiotensin-converting enzyme inhibition, angiotensin receptor blockade and/or statins. At the end of follow-up, 43 patients were in complete remission, 12 in partial remission, 10 patients had developed ESRD and 8 patients had on-going proteinuria. Fifty-one per cent of the patients received immunosuppressive therapy and the choice of therapy varied between and within the clinics. There was a tendency to initiate specific treatment at an early point instead of awaiting a possible spontaneous remission (21% of the patients), and non-recommended therapy such as corticosteroids only was used in a high proportion of these cases (47%). Conclusions Even though the treatment recommendations in idiopathic MN have not changed the last decade, the question of whom and when to treat seems to lead to uncertainty. Recent studies have presented promising results supporting the PLA2R antibody the predictive marker needed for this patient group. The diverse treatment approach presented in this study might have resulted in a worse outcome than expected. Hopefully, unnecessary exposure to immunosuppressive therapy or delayed treatment can be avoided through better support, education and treatment forums, and thus result in an improved outcome. PMID:26985373

  14. Exercise related ventilation dynamics and clinical correlates in patients with fibrotic idiopathic interstitial pneumonias.

    PubMed

    De Martino, Marina; Cobuccio, Raffaele; Bruzzese, Dario; Rea, Gaetano; Meoli, Ilernando; Stefanelli, Francesco; Canora, Angelo; Capaccio, Annalisa; Sanduzzi, Alessandro; Matarese, Alessandro; Bocchino, Marialuisa

    2016-01-01

    Assessment of exercise performance is a key component in the management of interstitial lung diseases, as its limitation may occur very early. Aim of the present study was to assess ventilation dynamics in combination with pulse-oximetry changes in 54 clinically stable patients affected by idiopathic pulmonary fibrosis or idiopathic fibrotic nonspecific interstitial pneumonia. Testing was successfully performed with the Spiropalm 6-MWT Hand-held spirometer by the majority of cases (94%). End test oxygen saturation (SpO2) values <88% were common in most of patients (76%), with a mean distance walked of 403 meters. Ventilation significantly increased due to the contribution of the tidal volume and the respiratory frequency (RF). This finding was associated with a decrease of the end of test respiratory reserve (RR), that was <20% in 9 cases (17.6%). Lung function was inversely related to the end of test RF, while a positive correlation occurred with the end of test RR and the estimated maximal voluntary ventilation (MVV). RR was also a predictive factor of declining forced vital capacity and lung diffusion capacity for carbon monoxide (DLCO) over a 6-month period. Further factors of DLCO impairment were low SpO2 and MVV. Comparison with the cardio-pulmonary exercise test (CPET) showed that the 6-MWT end of test RR was inversely related to the CPET-derived peak RF and VE/VCO2 suggesting RR as pivotal in exercise limitation assessment. Our results open challenging perspectives in an unexplored field. Future research will include management of latent respiratory failure and monitoring of disease progression and therapy response. PMID:27537719

  15. Survival benefits of warfarin in Korean patients with idiopathic pulmonary arterial hypertension

    PubMed Central

    Kang, Byung Ju; Oh, Yeon-Mok; Lee, Sang-Do; Lee, Jae Seung

    2015-01-01

    Background/Aims: Idiopathic pulmonary arterial hypertension (IPAH) is an incurable disease with high mortality. Although most studies recommend anticoagulation treatment for IPAH, the benefits are uncertain, particularly in Korea, where it has not been studied. The purpose of this study was to evaluate survival outcomes of Korean patients with IPAH treated with warfarin. Methods: We performed a retrospective cohort study of patients diagnosed previously with pulmonary arterial hypertension (PAH) at the Asan Medical Center in Korea, between January 1994 and February 2013. We excluded patients with associated PAH, patients who did not undergo right heart catheterization (RHC), and patients with a positive vasoreactivity test. Patients in the study cohort were classified into a “warfarin group” and a “non-warfarin group,” according to the treatment they received during the first year after diagnosis. Results: We identified 31 patients with IPAH and a negative vasoreactivity test on RHC. Median patient age was 36.0 years, and 23 patients (74.2%) were female. The median time from the onset of symptoms to diagnosis was 19.0 months, and the most common presenting symptom was dyspnea. Survival rates of the patients at 1, 3, 5, and 10 years were 90.2%, 79.5%, 62.7%, and 34.8%, respectively. The mean survival period was 12.0 years in the warfarin group and 6.1 years in the non-warfarin group. Warfarin treatment had significant survival benefits in patients with IPAH (p = 0.023). Conclusions: Warfarin treatment substantially improved survival outcomes in Korean cases of IPAH. PMID:26552459

  16. [Determination of activated platelets: evaluation of methodology and application for patients with idiopathic thrombocytopenic purpura].

    PubMed

    Hayashi, S; Oshida, M; Kiyokawa, T; Aochi, H; Honda, S; Tomiyama, Y; Kurata, Y

    2001-12-01

    Platelet activation causes a change in surface expression of several endogenous proteins, such as CD62P, CD63 and CD40L. Therefore, it is possible to analyze the functional in vivo status of the circulating platelet population directly by flow cytometry. In this study we developed the method to be suitable for use in clinical studies. We used EDTA-2K as anticoagulant since the sample anticoagulated with EDTA-2K, sodium citrate or ACD-A showed no difference in the data of activated platelets. We determined whether fixation of sample is necessary. The samples stained before or without fixation showed abnormally high level of activated platelets, indicating that fixation is necessary before staining. It is controversial whether activated platelets circulate in patients with idiopathic thrombocytopenic purpura(ITP). We measured activated platelets in patients with ITP using our optimised method. The percentages of CD62P, CD63 and CD40L positive platelets were significantly high in patients with ITP and 24%, 55% and 36% (respectively) of ITP patients showed elevated level of activated platelets. These data indicate that activated platelets circulate in ITP patients. PMID:11797399

  17. The Importance of the Neutrophil-Lymphocyte Ratio in Patients with Idiopathic Peripheral Facial Palsy

    PubMed Central

    Kiliçkaya, M. Mustafa; Tuz, Mustafa; Yariktaş, Murat; Yasan, Hasan; Aynalı, Giray; Bagci, Özkan

    2015-01-01

    Objective. The purpose of this study was to investigate whether or not there was a correlation between the neutrophil-to-lymphocyte ratio (NLR) value and the severity of idiopathic peripheral facial palsy (IPFP) and to determine whether or not NLR could be used as an early predictive parameter in the prognosis of IPFP patients. Material and Method. This retrospective study was conducted on 146 patients who were diagnosed with IPFP. The control group comprised 140 patients. Patients with IPFP were categorized according to the House-Brackmann grading system (HBS). The NLR value was obtained by dividing the neutrophil value by the lymphocyte value. Results. In the IPFP group, the mean NLR value was 3.63 ± 2.74 and, in the control group, 1.84 ± 0.78. The mean NLR value was significantly higher in IPFP patients than in the control subjects (p < 0.0001). The mean NLR value in group A (Grades I-II ) was 2.61 ± 2.28, in group B (Grades III-IV) 3.22 ± 2.65, and in group C (Grades V-VI) 10.69 ± 6.30. Conclusion. We determined that as the severity of IPFP increased, the NLR value increased. The NLR value can be used as a prognostic factor in the early prediction of IPFP prognosis. PMID:26713095

  18. Manual characterization of sleep spindle index in patients with narcolepsy and idiopathic hypersomnia.

    PubMed

    Delrosso, Lourdes M; Chesson, Andrew L; Hoque, Romy

    2014-01-01

    This is a retrospective review of PSG data from 8 narcolepsy patients and 8 idiopathic hypersomnia (IH) patients, evaluating electrophysiologic differences between these two central hypersomnias. Spindles were identified according to the AASM Manual for the Scoring of Sleep and Associated Events; and counted per epoch in the first 50 epochs of N2 sleep and the last 50 epochs of N2 sleep in each patient's PSG. Spindle count data (mean ± standard deviation) per 30 second-epoch (spindle index) in the 8 narcolepsy patients was as follows: 0.37 ± 0.73 for the first 50 epochs of N2; 0.65 ± 1.09 for the last 50 epochs of N2; and 0.51 ± 0.93 for all 100 epochs of N2. Spindle index data in the 8 IH patients was as follows: 2.31 ± 2.23 for the first 50 epochs of N2; 2.84 ± 2.43 for the last 50 epochs of N2; and 2.57 ± 2.35 for all 100 epochs of N2. Intergroup differences in spindle count in the first 50 N2 epochs, the last 50 N2 epochs, and all 100 epochs of scored N2 were significant (P < 0.01) as were the intragroup differences between the first 50 N2 epochs and the last 50 N2 epochs. PMID:24800086

  19. SHOX gene and conserved noncoding element deletions/duplications in Colombian patients with idiopathic short stature

    PubMed Central

    Sandoval, Gloria Tatiana Vinasco; Jaimes, Giovanna Carola; Barrios, Mauricio Coll; Cespedes, Camila; Velasco, Harvy Mauricio

    2014-01-01

    SHOX gene mutations or haploinsufficiency cause a wide range of phenotypes such as Leri Weill dyschondrosteosis (LWD), Turner syndrome, and disproportionate short stature (DSS). However, this gene has also been found to be mutated in cases of idiopathic short stature (ISS) with a 3–15% frequency. In this study, the multiplex ligation-dependent probe amplification (MLPA) technique was employed to determine the frequency of SHOX gene mutations and their conserved noncoding elements (CNE) in Colombian patients with ISS. Patients were referred from different centers around the county. From a sample of 62 patients, 8.1% deletions and insertions in the intragenic regions and in the CNE were found. This result is similar to others published in other countries. Moreover, an isolated case of CNE 9 duplication and a new intron 6b deletion in another patient, associated with ISS, are described. This is one of the first studies of a Latin American population in which deletions/duplications of the SHOX gene and its CNE are examined in patients with ISS. PMID:24689071

  20. Land-Jump Performance in Patients with Juvenile Idiopathic Arthritis (JIA): A Comparison to Matched Controls

    PubMed Central

    Ford, Kevin R.; Myer, Gregory D.; Melson, Paula G.; Darnell, Shannon C.; Brunner, Hermine I.; Hewett, Timothy E.

    2009-01-01

    Objective. The purpose of this study was to determine if high functioning children with Juvenile Idiopathic Arthritis (JIA) with minimal disease activity have different biomechanics during high loading tasks compared to controls. Patients were included if they had minimal inflammation documented in one or both knees. Methods. The subject groups consisted of eleven patients with JIA and eleven sex, age, height, and weight matched controls. Sagittal plane kinematic and kinetics were calculated during a drop vertical jump maneuver. The Child Health Assessment Questionnaire (CHAQ) was collected on each patient with JIA. Results. The subjects with JIA had increased knee (P = .011) and hip flexion (P < .001) compared to control subjects. Subjects with JIA also demonstrated decreased knee extensor moments during take-off (P = .028) and ankle plantar flexor moments during landing (P = .024) and take-off (P = .004). In the JIA group, increased hip extensor moments were predictive of increased disability (R2 = .477, SEE = .131). Conclusions. Patients with JIA may demonstrate underlying biomechanical deviations compared to controls. In addition, biomechanical assessment of hip extensor mechanics during dynamic tasks may provide an objective assessment tool to determine overall function in patients with JIA. PMID:20148070

  1. [Central hemodynamics and cerebrovascular disorders in patients with idiopathic arterial hypotension].

    PubMed

    Foniakin, A V; Mashin, V Vl; Ataian, A S; Saprygina, L V

    2012-01-01

    A total of 65 patients (mean age 40.2 +/- 8.1 yr) with neurologic and neuropsychological disorders associated with long-term idiopathic arterial hypotension (IAH) were studied to estimate the state of their central blood circulation. Neuropsychological conditions were estimated from the state of higher psychic functions, such as memory speech, gnosis, praxis, cognition, attention, counting, writing, and reading abilities. Central hemodynamics was studied by 24 hr monitoring arterial pressure and echocardiography. Group 1 included patients without neurologic problems (n = 19), group 2 consisted of patients with early manifestations of chronic cerebrovascular insufficiency (n = 46, 71%). They were older than patients of group 1 and had a long history of IAH. It was shown that most patients presented with stably reduced systolic and diastolic AP and non-dipper type of low 24-hour SAD index. The cardiac index was elevated due to increased left ventricular ejection fraction (group 1) or increased heart rate (group 2). The severity of neuropsychic disorder was negatively related to SAD and DAD indices and positively to systolic hypotension time. PMID:23516869

  2. The Prevalence and Characteristics of Primary Headache and Dream-Enacting Behaviour in Japanese Patients with Narcolepsy or Idiopathic Hypersomnia: A Multi-Centre Cross-Sectional Study

    PubMed Central

    Suzuki, Keisuke; Miyamoto, Masayuki; Miyamoto, Tomoyuki; Inoue, Yuichi; Matsui, Kentaro; Nishida, Shingo; Hayashida, Kenichi; Usui, Akira; Ueki, Yoichiro; Nakamura, Masaki; Murata, Momoyo; Numao, Ayaka; Watanabe, Yuji; Suzuki, Shiho; Hirata, Koichi

    2015-01-01

    Background Because the prevalence and characteristics of primary headache have yet to be thoroughly studied in patients with hypersomnia disorders, including narcolepsy and idiopathic hypersomnia, we examined these parameters in the Japanese population. Methods In a multicentre cross-sectional survey, among 576 consecutive outpatients with sleep disorders, 68 narcolepsy patients and 35 idiopathic hypersomnia patients were included. Additionally, 61 healthy control subjects participated. Semi-structured headache questionnaires were administered to all participants. Results The patients with narcolepsy (52.9%) and idiopathic hypersomnia (77.1%) more frequently experienced headache than the healthy controls (24.6%; p<0.0001). The prevalence rates were 23.5%, 41.2% and 4.9% for migraine (p<0.0001) and 16.2%, 23.5% and 14.8% (p = 0.58) for tension-type headache among the narcolepsy patients, the idiopathic hypersomnia patients and the control subjects, respectively. Those who experienced migraine more frequently experienced excessive daytime sleepiness, defined as an Epworth Sleepiness Scale score of ≥10, than those who did not experience headache among the patients with narcolepsy (93.8% vs. 65.6%, p = 0.040) and idiopathic hypersomnia (86.7% vs. 37.5%, p = 0.026). Dream-enacting behaviour (DEB), as evaluated by the rapid eye movement sleep disorders questionnaire, was more frequently observed in the narcolepsy patients than in the idiopathic hypersomnia patients and the control subjects. An increased DEB frequency was observed in the narcolepsy patients with migraines compared to those without headache. Conclusions Migraines were frequently observed in patients with narcolepsy and idiopathic hypersomnia. DEB is a characteristic of narcolepsy patients. Further studies are required to assess the factors that contribute to migraines in narcolepsy and idiopathic hypersomnia patients. PMID:26418536

  3. Changes in Follicular Helper T Cells in Idiopathic Thrombocytopenic Purpura Patients

    PubMed Central

    Xie, Jue; Cui, Dawei; Liu, Yan; Jin, Jie; Tong, Hongyan; Wang, Lei; Ruan, Guoxiang; Lu, Yun; Yuan, Huiming

    2015-01-01

    Background: Idiopathic thrombocytopenic purpura (ITP) is a primary autoimmune disease with a decreased platelet count caused by platelet destruction mediated mainly by platelet antibodies. T follicular helper (TFH) cells have demonstrated important roles in autoimmune diseases. The aim of this study is to explore the might role of TFH cells in the patients of ITP. Methods: Twenty-three ITP patients and 12 healthy controls (HC) were enrolled in this study. The frequency of circulating TFH cells in both the patients and HC was analyzed by flow cytometry. Serum interleukin (IL)-21 and IL-6 levels were measured using ELISA, and platelet antibodies were tested using a solid phase technique. Additionally, IL-21, IL-6, Bcl-6 and c-Maf mRNA expressions in peripheral blood mononuclear cells (PBMCs) were detected using real-time PCR. Results: The percentages of circulating CXCR5+ CD4+TFH cells with ICOShigh or PD-1high expression were significantly higher in the ITP patients than in the HC. Moreover, the frequencies of circulating CXCR5+ CD4+TFH cells with inducible costimulator (ICOS)high or programmed death-1 (PD-1)high expression were notably higher in ITP with platelet-antibody-positive ( ITP (+) ) patients than in ITP with platelet-antibody-negative ( ITP (-) ) patients and HC, as were the serum IL-21 and IL-6 levels (significant). Moreover, a positive correlation was found between the CXCR5+CD4+TFH cells with ICOShigh or PD-1high expression and the serum IL-21 levels of ITP (+) patients. Additionally, the mRNA expression levels of IL-21, IL-6, Bcl-6 and c-Maf were significantly increased in ITP patients, especially in ITP (+) patients. Conclusions: This study demonstrated TFH cells and effector molecules might play an important role in the pathogenesis of ITP, which are possible therapeutic targets in ITP patients. PMID:25561904

  4. Congenital scoliosis - Quo vadis?

    PubMed

    Debnath, Ujjwal K; Goel, Vivek; Harshavardhana, Nanjanduppa; Webb, John K

    2010-04-01

    Congenital spinal vertebral anomalies can present as scoliosis or kyphosis or both. The worldwide prevalence of the vertebral anomalies is 0.5-1 per 1000 live births. Vertebral anomalies can range from hemi vertebrae (HV) which may be single or multiple, vertebral bar with or without HV, block vertebrae, wedge shaped or butterfly vertebrae. Seventy per cent of congenital vertebral anomalies result in progressive deformities. The risk factors for progression include: type of defect, site of defect (junctional regions) and patient's age at the time of diagnosis. The key to success in managing these spinal deformities is early diagnosis and anticipation of progression. One must intervene surgically to halt the progression of deformity and prevent further complications associated with progressive deformity. Planning for surgery includes a preoperative MRI scan to rule out spinal anomalies such as diastematomyelia. The goals of surgical treatment for congenital spinal deformity are to achieve a straight growing spine, a normal standing sagittal profile, and a short fusion segment. The options of surgery include in situ fusion, convex hemi epiphysiodesis and hemi vertebra excision. These basic surgical procedures can be combined with curve correction, instrumentation and short segment fusion. Most surgeons prefer posterior (only) surgery for uncomplicated HV excision and short segment fusion. These surgical procedures can be performed through posterior, anterior or combined approaches. The advocates of combined approaches suggest greater deformity correction possibilities with reduced incidence of pseudoarthrosis and minimize crankshaft phenomenon. We recommend posterior surgery for curves involving only an element of kyphosis or modest deformity, whereas combined anterior and posterior approach is indicated for large or lordotic deformities. In the last decade, the use of growing rods and vertebral expandable prosthetic titanium rib has improved the armamentarium of the

  5. Scoliosis Screening in Schools.

    ERIC Educational Resources Information Center

    New York State Education Dept., Albany. Div. of Pupil Personnel Services.

    The booklet outlines New York state school policy and procedures for screening students for scoliosis, lateral curvature of the spine. It is explained that screening is designed to discover spinal deformities early enough to prevent surgery. Planning aspects, including organizing a planning team for the school district, are discussed. Among…

  6. Serum thyroid autoantibodies in patients with idiopathic either acute or chronic urticaria.

    PubMed

    Gangemi, S; Saitta, S; Lombardo, G; Patafi, M; Benvenga, S

    2009-02-01

    In Italy, only one study was conducted on the detection of serum thyroid autoantibodies (ATA) in patients with urticaria. This northern-Italy study reported a 23% rate of ATA positiveness in 52 patients with chronic idiopathic urticaria (CIU). During the years 1998-2006, 688 patients with urticaria were hospitalized at our Division of Allergy and Clinical Immunology. Thyroglobulin and thyroperoxidase autoantibodies (TgAb and TPOAb) were assayed at admission in 144/688 patients. Of the 144 patients (mean age: 42.3+/-15.8 yr, range 17-84), 95 (72 women and 23 men) had an history of CIU (CIU group) and 49 (44 women and 5 men) did not [acute urticaria group or (AU)]. Of the 144 patients, 37 (25.7%) tested positive for at least one ATA: 31 with CIU (32.6%) and 6 with AU (12.2%, chi2=7.037, p=0.008). Positiveness for TPOAb or TgAb was 30/37 (81.1%) or 17/37 (45.9%); 10/37 (27.0%). Pre-hospitalization duration of CIU was longer in the 31 ATA positive patients compared to the 64 ATA negative patients (207.2+/-273.4 vs 81.6+/-106.3 weeks, p=0.015). Pre-hospitalization duration of CIU correlated positively with the log10-transformed serum concentration of TPOAb in the 25 CIU patients who tested TPOAb positive (r=0.42, p=0.039). We conclude that our rate of 31/95 (32.6%) positiveness for at least one type of ATA in CIU is greater than that of 6 representative international studies published between the years 2000 and 2006 (111/488 or 22.7%, range 15-29%, chi2=4.884, p=0.027). PMID:19411805

  7. Impact of Comorbidities on Mortality in Patients with Idiopathic Pulmonary Fibrosis

    PubMed Central

    Kreuter, Michael; Ehlers-Tenenbaum, Svenja; Palmowski, Karin; Bruhwyler, Jacques; Oltmanns, Ute; Muley, Thomas; Heussel, Claus Peter; Warth, Arne; Kolb, Martin; Herth, Felix J. F.

    2016-01-01

    Introduction Comorbidities significantly influence the clinical course of idiopathic pulmonary fibrosis (IPF). However, their prognostic impact is not fully understood. We therefore aimed to determine the impact of comorbidities, as individual and as whole, on survival in IPF. Methods The database of a tertiary referral centre for interstitial lung diseases was reviewed for comorbidities, their treatments, their frequency and survival in IPF patients. Results 272 patients were identified of which 12% had no, 58% 1–3 and 30% 4–7 comorbidities, mainly cardiovascular, pulmonary and oncologic comorbidities. Median survival according to the frequency of comorbidities differed significantly with 66 months for patients without comorbidities, 48 months when 1–3 comorbidities were reported and 35 months when 4–7 comorbidities were prevalent (p = 0.004). A multivariate Cox proportional hazard analyses identified other cardiac diseases and lung cancer as significant predictors of death, gastro-oesophageal reflux disease (GERD) and diastolic dysfunction had a significant positive impact on survival. A significant impact of comorbidities associated therapies on survival was not discovered. This included the use of proton pump inhibitors at baseline, which was not associated with a survival benefit (p = 0.718). We also established a predictive tool for highly prevalent comorbidities, termed IPF comorbidome which demonstrates a new relationship of IPF and comorbidities. Conclusion Comorbidities are frequent in IPF patients. Some comorbidities, especially lung cancer, mainly influence survival in IPF, while others such as GERD may inherit a more favourable effect. Moreover, their cumulative incidence impacts survival. PMID:27023440

  8. Pituitary suppression before frozen embryo transfer is beneficial for patients suffering from idiopathic repeated implantation failure.

    PubMed

    Yang, Xing; Huang, Rui; Wang, Yan-fang; Liang, Xiao-yan

    2016-02-01

    Long-term gonadotropin-releasing hormone agonist (GnRHa) administration before in vitro fertilization (IVF)/intracytoplasmic sperm injection (ICSI) in infertile women with endometriosis or adenomyosis significantly enhanced the chances of pregnancy in both fresh and frozen embryo transfer cycles. We hypothesized that long-term GnRHa treatment might also be beneficial for the idiopathic repeated implantation failure (RIF) patients. In the 21 patients receiving GnRHa and hormone replacement therapy (G-HRT) protocols for frozen embryo transfer, their data were compared with those of the 56 of frozen/fresh cycles they had previously undergone (previous protocols). Comparison showed that the finial results were significantly better with G-HRT protocols than with their previous protocols, with pregnancy rate, clinical pregnancy rate, implantation rate and on-going pregnancy rate being 70%, 60%, 40% and 38% respectively with G-HRT protocols, against 17%, 11%, 6.3% and 5% with previous protocols. The results showed that hormonally controlled endometrial preparation with prior GnRHa suppression could be used for patients who had experienced repeated failures of IVF treatment despite having morphologically optimal embryos, and the treatment may help increase the receptivity of the endometrium in these patients. PMID:26838753

  9. Efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis with more preserved lung function.

    PubMed

    Albera, Carlo; Costabel, Ulrich; Fagan, Elizabeth A; Glassberg, Marilyn K; Gorina, Eduard; Lancaster, Lisa; Lederer, David J; Nathan, Steven D; Spirig, Dominique; Swigris, Jeff J

    2016-09-01

    This post hoc analysis examined the differences in idiopathic pulmonary fibrosis disease progression and the effects of pirfenidone in patients stratified by more preserved versus less preserved baseline lung function status using forced vital capacity (FVC) or GAP (gender, age and physiology) index stage.Efficacy outcomes, i.e. FVC, 6-min walking distance (6MWD) and dyspnoea (University of California San Diego Shortness of Breath Questionnaire (UCSD SOBQ)), were analysed at 12 months in patients randomised to pirfenidone 2403 mg·day(-1) or placebo in the pooled phase 3 CAPACITY/ASCEND population (n=1247), with subgroups stratified by baseline FVC ≥80% versus <80% or GAP stage I versus II-III. Treatment-by-subgroup interaction was tested based on a rank ANCOVA model; factors in the model included study, region, treatment, subgroup and treatment-by-subgroup interaction term.Patients with both more preserved (FVC ≥80% or GAP stage I) and less preserved (FVC <80% or GAP stage II-III) lung function at baseline demonstrated clinically significant disease progression at 12 months in terms of categorical decline in FVC, 6MWD and UCSD SOBQ. The magnitude of pirfenidone treatment effect was comparable between subgroups, regardless of whether lung function was classified using FVC or GAP index stage.These findings support the initiation of treatment with pirfenidone, irrespective of stage of baseline lung function in this patient population. PMID:27471208

  10. [Recovery of hearing: results of delayed medical treatment in patients with idiopathic sudden hearing loss].

    PubMed

    Maassen, M M; Pfister, M; Plontke, S; Koitschev, A; Vögler, A; Löwenheim, H

    2002-12-01

    For the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL), a variety of studies about intravenous drug administration with the beginning of treatment in the early period of less then one week after the onset of hearing loss have been performed. In contrast, very little information is available about the efficacy of intravenous drug therapy for ISSNHL with the beginning of treatment later than four weeks after the onset of hearing loss. In a retrospective chart review we studied the treatment results of 57 patients with ISSNHL with beginning of treatment later than four weeks after the onset of hearing loss with no spontaneous recovery of hearing. Patients received a treatment with intravenous administration of Dextran (concentration 40 g/l with NaCl 0.9%) and Procain-HCl (a derivative of the local anaesthetic lidocaine,400-800 mg in a 500 ml rheologic infusion of Dextran 40). 25% of the patients showed a significant improvement of 10 dB or more in hearing threshold at 1000 Hz measured in bone-conducted pure tone audiometry. In a subjective evaluation 53% of the patients noticed a subjective improvement of their individual hearing thresholds. PMID:12474128

  11. Coronary artery bypass grafting in a patient with active idiopathic cryoglobulinemia: revisiting the issue

    PubMed Central

    Fakih, Hafiz Abdul Moiz; Elueze, Emmanuel; Vij, Rajiv

    2016-01-01

    Background Cryoglobulinemia is a cold-reactive autoimmune disease. It is of distinctive importance in cardiac surgery because of the use of hypothermic cardiopulmonary bypass (CPB). Cryoglobulins, which activate at variable levels of hypothermia, can cause precipitation during surgery leading to possibly severe leukocytoclastic or necrotizing vasculitis, clinically manifested as ischemic events, such as cutaneous ulcerations, glomerulonephritis, arthritis, or peripheral neuropathies among the most reported associated comorbidities. Management of CPB and systemic protection in this rare but unique scenario requires individualized planning. We report the case of a patient with active cryoglobulinemia who was preoperatively managed with plasmapheresis. He underwent hypothermic coronary bypass with no precipitation and flare during and after surgery. Case presentation We describe the case of a 59-year-old Caucasian male with clinically significant idiopathic cryoglobulinemia and history of recurrent skin lesions and toe amputations secondary to cold exposure. He presented with 2-h duration of chest pain and new onset atrial fibrillation. After cardiac catheterization, a diagnosis of three-vessel coronary artery disease was established and coronary artery bypass grafting (CABG) was scheduled. Because of a high risk of flare-up during surgery, the patient was preemptively treated with two sessions of plasmapheresis before bypass. He then underwent hypothermic CABG. The pre- and perioperative course was unremarkable without any clinical evidence of precipitation. The patient was discharged on day 6 postoperatively without any complications. Conclusion Preoperative plasmapheresis before hypothermic coronary bypass can prevent fatal cryoglobulinemia-related complications in patients with active disease. PMID:26908383

  12. Patient considerations and drug selection in the treatment of idiopathic pulmonary fibrosis

    PubMed Central

    Trawinska, Maria A; Rupesinghe, Ruwani D; Hart, Simon P

    2016-01-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease of unknown cause. Approximately 5,000 people are diagnosed with IPF in the UK every year. People with IPF suffer significant morbidity and, without any curative treatment at present, survival rates remain poor with a median survival of 3 years. While treatment remains largely supportive, many drug therapies have been trialed in IPF over the years. Pirfenidone and nintedanib are newly licensed treatments for IPF and the first drugs to have shown convincing evidence of slowing disease progression. In addition to evaluating clinical evidence, we also discuss elements affecting drug choice from the viewpoint of patients and health care professionals. We discuss pharmacological and nonpharmacological aspects of providing best supportive care for patients with IPF. However, few good quality studies exist focusing on controlling symptoms specifically in patients with IPF, and recommendations are often extrapolated from evidence in other chronic diseases. In covering these topics, we hope to provide readers with a comprehensive review of the available evidence pertaining to all aspects of care for patients suffering with IPF. PMID:27114711

  13. Quantitative MRI of the wrist and nerve conduction studies in patients with idiopathic carpal tunnel syndrome

    PubMed Central

    Uchiyama, S; Itsubo, T; Yasutomi, T; Nakagawa, H; Kamimura, M; Kato, H

    2005-01-01

    Objective: To correlate morphological findings of idiopathic carpal tunnel syndrome (CTS) with the function of the median nerve. Methods: In this study, 105 wrists of 105 women patients with idiopathic CTS, and 36 wrists of 36 female volunteers were subjected to nerve conduction studies and MRI. Cross sectional area, signal intensity ratio, and the flattening ratio of the median nerve, carpal tunnel area, flexor tendon area, synovial area, and intersynovial space, and the palmar bowing of the transverse carpal ligament (TCL) were quantified by MRI and correlated with the severity of the disease determined by nerve conduction studies. Results: Cross sectional areas of the median nerve, flexor tendons, and carpal tunnel, and the palmar bowing of the TCL of the CTS groups were greater than in the control group, but differences were not detected among the CTS groups for the area of the flexor tendons and the carpal tunnel. Enlargement, flattening, and high signal intensity of the median nerve at the distal radioulnar joint level were more significant in the advanced than in the earlier stages of the disease. Increase in palmar bowing of the TCL was less prominent in the most advanced group. Linear correlation between the area of the carpal tunnel and palmar bowing of the TCL was noted. Conclusion: Severity of the disease could be judged by evaluating not only longitudinal changes of signal intensity and configuration of the median nerve, but also palmar bowing of the TCL. Increased palmar bowing of the TCL was found to be associated with an increase in the area of the carpal tunnel. PMID:16024888

  14. Serum Amyloid A Circulating Levels and Disease Activity in Patients with Juvenile Idiopathic Arthritis

    PubMed Central

    Giani, Teresa; Fioravanti, Antonella; Iacoponi, Francesca; Simonini, Gabriele; Pagnini, Ilaria; Spreafico, Adriano; Chellini, Federico; Galeazzi, Mauro; Cimaz, Rolando

    2012-01-01

    The aim of our study was to evaluate the association between circulating levels of serum amyloid A protein (SAA) and disease activity in patients with juvenile idiopathic arthritis (JIA). Our study group included 41 JIA patients (9 male, 32 female), classified according to the International League of Associations for Rheumatology (ILAR) criteria (5); 16 had polyarticular onset disease and 25 had oligoarticular onset disease. Among 25 patients with oligoarticular disease, three had extended oligoarthritis. Serum amyloid A (SAA), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were measured in both patients and 26 healthy controls. SAA levels were higher in JIA patients versus healthy controls (p<0.001). Significant positive correlations were found between SAA and the presence of active joints (rho=0.363, p<0.05), the number of active joints (rho=0.418, p<0.05), ESR (R=0.702, p<0.05) and CRP (R=0.827, p<0.05). No significant correlations between ESR and the presence of active joints (rho=0.221, p=0.225) or between ESR and the number of active joints (rho=0.118, p=0.520) were demonstrated in JIA patients. No significant correlations were obtained between CRP and the presence of active joints (rho=0.034, p=0.855) or between CRP and the number of active joints (rho=0.033, p=0.859). We discovered a significant increase in SAA levels in JIA patients, compared to controls, and a strong positive correlation between SAA level and JIA disease activity. We also discerned SAA to be a more sensitive laboratory marker than ESR and CRP for evaluating the presence and number of active joints. We suggest that SAA can be used as an additional indicator of disease activity in JIA. PMID:22869491

  15. Functional improvement in patients with idiopathic pulmonary fibrosis undergoing single lung transplantation *

    PubMed Central

    Rubin, Adalberto Sperb; Nascimento, Douglas Zaione; Sanchez, Letícia; Watte, Guilherme; Holand, Arthur Rodrigo Ronconi; Fassbind, Derrick Alexandre; Camargo, José Jesus

    2015-01-01

    Abstract Objective: To evaluate the changes in lung function in the first year after single lung transplantation in patients with idiopathic pulmonary fibrosis (IPF). Methods: We retrospectively evaluated patients with IPF who underwent single lung transplantation between January of 2006 and December of 2012, reviewing the changes in the lung function occurring during the first year after the procedure. Results: Of the 218 patients undergoing lung transplantation during the study period, 79 (36.2%) had IPF. Of those 79 patients, 24 (30%) died, and 11 (14%) did not undergo spirometry at the end of the first year. Of the 44 patients included in the study, 29 (66%) were men. The mean age of the patients was 57 years. Before transplantation, mean FVC, FEV1, and FEV1/FVC ratio were 1.78 L (50% of predicted), 1.48 L (52% of predicted), and 83%, respectively. In the first month after transplantation, there was a mean increase of 12% in FVC (400 mL) and FEV1 (350 mL). In the third month after transplantation, there were additional increases, of 5% (170 mL) in FVC and 1% (50 mL) in FEV1. At the end of the first year, the functional improvement persisted, with a mean gain of 19% (620 mL) in FVC and 16% (430 mL) in FEV1. Conclusions: Single lung transplantation in IPF patients who survive for at least one year provides significant and progressive benefits in lung function during the first year. This procedure is an important therapeutic alternative in the management of IPF. PMID:26398749

  16. Immunophenotype characteristics of peripheral blood mononuclear leukocytes of chronic idiopathic urticaria patients.

    PubMed

    Garmendia, Jenny V; Zabaleta, Mercedes; Aldrey, Oscar; Rivera, Henry; De Sanctis, Juan B; Bianco, Nicolás E; Blanca, Isaac

    2006-12-01

    The pathogenesis of chronic idiopathic urticaria (CIU) is not completely understood although autoimmunity has been proposed. The aim of the study was to assess the expression of different leukocyte antigens, by flow cytometry, assaying total blood of 29 patients with CIU and of 20 sex and age matched controls. Moreover, we assessed soluble CD154 a marker of immune cell activation, predominantly memory T cells. When patients were divided depending an their response to the autologous serum skin test (ASST), three different groups were encountered: group 1 (n=11): with negative ASST-, group 2 (n=11): positive ASST (ASST+) with normal lymphocyte counts and group 3 (n=7): ASST+ with low lymphocyte counts (< 1500 cells/mm3). A significant increase in CD19+ percentage and not in the absolute count (P < 0.05) was observed in group 1 as compared to controls and to the other groups. In contrast, CD30+, CD45RO+ and CD4+/CD45RO+ percentages and biologically active soluble CD154 levels were significantly higher (P < 0.05) in group 3 as compared to group 1 or to controls. In ASST positive groups, CD45RO+ and CD4+/CD45RO+ positiveness correlates with wheal diameter. In conclusion, memory cells may play a role in these different types of patients and in understanding CIU pathogenesis. PMID:17176904

  17. Frequency and pattern of idiopathic osteosclerosis and condensing osteitis lesions in panoramic radiography of Iranian patients

    PubMed Central

    Farhadi, Farrokh; Ruhani, Mohammad Razavi; Zarandi, Ali

    2016-01-01

    Background: The purpose of this study was to investigate of radiographic pattern and relative frequency of idiopathic osteosclerosis (IO) and condensing osteitis (CO) in panoramic radiographs. Materials and Methods: Totally 411 panoramic radiographies were randomly selected from patients referred to Radiology Department of Faculty of Dentistry, Tabriz University of Medical Sciences. Descriptive characteristics of radiopacities, including shape, border, localization, and dental relationship, were recorded. The Chi-squared test was used. Results: IO was detected in 31 (7.5%) patients and 22 (68.8%) lesions had exact border while their shape was mostly irregular. About 17 (53.1%) lesions were apical, and 13 (40.6%) lesions had no relation to the teeth. The most involved teeth were the second premolar (28.1%) and first molar (25%). Moreover, CO was detected in 32 (7.8%) patients. 17 (53.1%) lesions had an ill-defined border, and their shape was mostly irregular (65.6%) with ill-defined border. Around 18 (56.3%) lesions were apical, and 11 (34.4) lesions were apical and interradicular. The most involved teeth were the second premolar (59.4%) and the first molar (21.9%). Conclusion: The results demonstrated that relative frequency of IO in the selected population was 7.5% and for CO, it was 7.8%.

  18. The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review.

    PubMed

    Tomassetti, Sara; Piciucchi, Sara; Tantalocco, Paola; Dubini, Alessandra; Poletti, Venerino

    2015-03-01

    Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressively fibrosing interstitial pneumonia that is associated with a significantly worse prognosis than other forms of chronic interstitial pneumonia. An early and accurate diagnosis of IPF is important to enable the initiation of disease-specific therapies, which have the potential to reduce disease progression, and the avoidance of inappropriate and potentially harmful drugs. Establishing an accurate diagnosis of IPF can be challenging. Recent studies and international guidelines advocate the importance of a multidisciplinary team (MDT) in the initial diagnostic assessment of patients with suspected IPF. Typical MDT members include a pulmonologist, a radiologist and a pathologist, with further input from a thoracic surgeon, a rheumatologist, a specialist nurse and an occupational physician where appropriate. Multidisciplinary diagnosis is considered the gold standard because it can improve the accuracy of diagnosis of IPF, avoid unnecessary testing (e.g. lung biopsy), and optimise patient management. Here we highlight the strengths and limitations of the multidisciplinary approach to IPF diagnosis through MDT discussion of two patient cases. PMID:25726558

  19. Cervical Scoliosis: Clinical and Radiographic Outcomes.

    PubMed

    Mesfin, Addisu; Bakhsh, Wajeeh R; Chuntarapas, Tapanut; Riew, K Daniel

    2016-02-01

    Study Design Retrospective study. Objective Cervical scoliosis is a rare condition that can arise from various etiologies. Few reports on the surgical management of cervical scoliosis exist. Our objective was to evaluate clinical and radiographic outcomes following surgical management of cervical scoliosis. Methods We evaluated our cervical spine surgical database for patients with cervical scoliosis (Cobb angle > 10 degrees) from 2005 to 2010. Demographic data including age, gender, diagnoses, and primary versus revision surgery was collected. Surgical data including procedure (anterior versus posterior), estimated blood loss (EBL), length of surgery, length of hospitalization, and complications was recorded. Preoperative and postoperative Cobb angle measurements and Neck Disability Index (NDI) scores were recorded. Results Cervical scoliosis was identified in 18 patients. We excluded 5, leaving 5 men and 8 women with an average age of 50.7 (median 52, range 25 to 65). The average follow-up was 40 months (median 36.5, range 5 to 87). An anterior-only approach was used in 6 cases (average 4 levels fused), 5 cases were posterior-only approach (average 8.7 levels fused), and 2 cases were combined anterior-posterior approach. The EBL was an average of 286 mL (median 150, range 50 to 900), the average surgical time was 266 minutes (median 239, range 136 to 508), and the average hospital stay was 2.7 days (median 2, range 1 to 7). Complications occurred in 7 patients, and 2 developed adjacent segment pathology. The average coronal Cobb angle preoperatively was 35.1 degrees (median 31, range 13 to 63) and corrected was 15.7 degrees (median 10.5, range 2 to 59) postoperatively (p < 0.005). The average NDI preoperatively was 24.9 (median 26, range 6 to 37) and was reduced to 17.8 (median 18, range 7 to 30) postoperatively (p < 0.02). Conclusion Surgical management of cervical scoliosis can result in deformity correction and improvement in patient

  20. Cervical Scoliosis: Clinical and Radiographic Outcomes

    PubMed Central

    Mesfin, Addisu; Bakhsh, Wajeeh R.; Chuntarapas, Tapanut; Riew, K. Daniel

    2015-01-01

    Study Design Retrospective study. Objective Cervical scoliosis is a rare condition that can arise from various etiologies. Few reports on the surgical management of cervical scoliosis exist. Our objective was to evaluate clinical and radiographic outcomes following surgical management of cervical scoliosis. Methods We evaluated our cervical spine surgical database for patients with cervical scoliosis (Cobb angle > 10 degrees) from 2005 to 2010. Demographic data including age, gender, diagnoses, and primary versus revision surgery was collected. Surgical data including procedure (anterior versus posterior), estimated blood loss (EBL), length of surgery, length of hospitalization, and complications was recorded. Preoperative and postoperative Cobb angle measurements and Neck Disability Index (NDI) scores were recorded. Results Cervical scoliosis was identified in 18 patients. We excluded 5, leaving 5 men and 8 women with an average age of 50.7 (median 52, range 25 to 65). The average follow-up was 40 months (median 36.5, range 5 to 87). An anterior-only approach was used in 6 cases (average 4 levels fused), 5 cases were posterior-only approach (average 8.7 levels fused), and 2 cases were combined anterior-posterior approach. The EBL was an average of 286 mL (median 150, range 50 to 900), the average surgical time was 266 minutes (median 239, range 136 to 508), and the average hospital stay was 2.7 days (median 2, range 1 to 7). Complications occurred in 7 patients, and 2 developed adjacent segment pathology. The average coronal Cobb angle preoperatively was 35.1 degrees (median 31, range 13 to 63) and corrected was 15.7 degrees (median 10.5, range 2 to 59) postoperatively (p < 0.005). The average NDI preoperatively was 24.9 (median 26, range 6 to 37) and was reduced to 17.8 (median 18, range 7 to 30) postoperatively (p < 0.02). Conclusion Surgical management of cervical scoliosis can result in deformity correction and improvement in patient

  1. Intravenous iron therapy in patients with idiopathic pulmonary arterial hypertension and iron deficiency

    PubMed Central

    Manders, Emmy; Happé, Chris M.; Schalij, Ingrid; Groepenhoff, Herman; Howard, Luke S.; Wilkins, Martin R.; Bogaard, Harm J.; Westerhof, Nico; van der Laarse, Willem J.; de Man, Frances S.; Vonk-Noordegraaf, Anton

    2015-01-01

    Abstract In patients with idiopathic pulmonary arterial hypertension (iPAH), iron deficiency is common and has been associated with reduced exercise capacity and worse survival. Previous studies have shown beneficial effects of intravenous iron administration. In this study, we investigated the use of intravenous iron therapy in iron-deficient iPAH patients in terms of safety and effects on exercise capacity, and we studied whether altered exercise capacity resulted from changes in right ventricular (RV) function and skeletal muscle oxygen handling. Fifteen patients with iPAH and iron deficiency were included. Patients underwent a 6-minute walk test, cardiopulmonary exercise tests, cardiac magnetic resonance imaging, and a quadriceps muscle biopsy and completed a quality-of-life questionnaire before and 12 weeks after receiving a high dose of intravenous iron. The primary end point, 6-minute walk distance, was not significantly changed after 12 weeks (409 ± 110 m before vs. 428 ± 94 m after; P = 0.07). Secondary end points showed that intravenous iron administration was well tolerated and increased body iron stores in all patients. In addition, exercise endurance time (P < 0.001) and aerobic capacity (P < 0.001) increased significantly after iron therapy. This coincided with improved oxygen handling in quadriceps muscle cells, although cardiac function at rest and maximal were unchanged. Furthermore, iron treatment was associated with improved quality of life (P < 0.05). In conclusion, intravenous iron therapy in iron-deficient iPAH patients improves exercise endurance capacity. This could not be explained by improved RV function; however, increased quadriceps muscle oxygen handling may play a role. (Trial registration: ClinicalTrials.gov identifier NCT01288651) PMID:26401247

  2. Intra-articular glucocorticoid injections in patients with juvenile idiopathic arthritis in a Singapore hospital

    PubMed Central

    Leow, Olivia Min Yi; Lim, Lee Kean; Ooi, Pei Ling; Shek, Lynette Pei Chi; Ang, Elizabeth You Ning; Son, Mary Beth

    2014-01-01

    INTRODUCTION This study aimed to evaluate the efficacy and safety of intra-articular glucocorticoid (IAG) injections in our institution in children with juvenile idiopathic arthritis (JIA). METHODS This is a retrospective assessment of IAG injections performed by the Department of Paediatrics, National University Hospital, Singapore, from October 2009 to October 2011. A total of 26 procedures were evaluated for efficacy, considering parameters such as clinical response, changes in systemic medication, length of time between repeat injections, safety, consent-taking, pre- and post-procedural advice, compliance with aseptic technique, and post-procedural complications. RESULTS A total of 26 IAG injections of triamcinolone hexacetonide were administered over 17 occasions (i.e. patient encounters) to ten patients with JIA during the study period. After the injections, clinical scoring by a paediatric rheumatologist showed overall improvement by an average of 2.62 points out of 15. Besides six patient encounters that had an increase in systemic medication on the day of the injection, five required an increase within six months post injection, two required no adjustments, and one resulted in a decrease in medications. In all, 21 injections did not require subsequent injections. The mean interval between repeat injections was 7.8 months. Cutaneous side effects were noted in three anatomically difficult joints. Medical documentation with regard to patient progress was found to be lacking. CONCLUSION As per the recommendations of the American College of Rheumatology, we safely used IAG injections as the first-line therapy in our group of patients with oligoarticular JIA, and/or as an adjunct to systemic therapy in our patients with JIA. PMID:24862747

  3. Increased frequency of monoclonal gammopathy of undetermined significance in patients with nonimmune chronic idiopathic neutropenia syndrome.

    PubMed

    Papadaki, H A; Eliopoulos, D G; Ponticoglou, C; Eliopoulos, G D

    2001-04-01

    This study describes the frequency of monoclonal gammopathy of undetermined significance (MGUS) and the changes in some inflammation-related serum proteins in 157 patients with nonimmune chronic idiopathic neutropenia syndrome (NI-CINS). Of these patients, 42 had pronounced neutropenia with neutrophil counts < 1500/microL, and 115 had mild neutropenia with neutrophil counts ranging from 1500 to 2499/microL. Sixty-six volunteers served as healthy control subjects and 157 age- and sex-matched patients hospitalized for nonmalignant diseases served as patient control subjects. We found that 28.6% of patients with pronounced neutropenia and 14.8% of patients with mild neutropenia had increased serum gamma globulins (above the 95% confidence limit of values of the control subjects). In the group of patients with pronounced neutropenia, 30.9% had increased immunoglobulin (Ig)G values and 23.8% had increased IgA values. In the group of patients with mild neutropenia, 17.4% had increased IgG values and 21.7% had increased IgA values. IgG and IgA values strongly correlated with the neutrophil count. No changes in serum IgM were found. Three of 42 patients with pronounced neutropenia (7.14%) and 3 of 115 patients with mild neutropenia (2.61%) had serum immunofixation tests which showed a small monoclonal spike--4 were IgG-kappa type, 1 was IgG-lambda type, and 1 was IgA-kappa type. None of the healthy or patient control subjects had any evidence of MGUS. No significant changes in the amount of monoclonal spikes were documented during an 18- to 143-month follow-up (median, 58 months). Except for significantly increased alpha1-antitrypsin levels, there were no significant differences in the levels of acute-phase proteins studied between the study patients and the control subjects. These findings are consistent with our previous report suggesting the possible existence of an unrecognized low-grade chronic inflammation in patients with NI-CINS, which may be involved in the

  4. Outcome in patients with idiopathic retroperitoneal fibrosis treated with corticosteroid or tamoxifen monotherapy

    PubMed Central

    van der Bilt, Floor E.; Hendriksz, Tadek R.; van der Meijden, Wilbert A.G.; Brilman, Lisette G.; van Bommel, Eric F.H.

    2016-01-01

    Background Although corticosteroids (CS) are used primarily in idiopathic retroperitoneal fibrosis (iRPF), tamoxifen (TMX) may be a suitable alternative. We compared outcome with CS or TMX monotherapy for first presentation in a large group of patients with iRPF disease. Methods Of all patients with iRPF disease who were seen at our tertiary care referral centre from February 1999 to December 2011, 118 patients were eligible for this retrospective study. Treatment success was defined as the composite of (i) amelioration of symptoms, (ii) computed tomography (CT)-documented mass regression and, if applicable, (iii) definitive removal of ureteral stent or nephrostomy tube. Recurrence was defined as recurrence of signs and symptoms and/or CT-documented mass increase after initial treatment success with primary treatment. Results Presenting signs and symptoms did not differ between patients treated with CS (n = 50) or TMX (n = 68). Time to amelioration of symptoms after treatment initiation was shorter in CS-treated patients [CS, 2.0 (0.8–3.8) weeks versus TMX, 4.0 (2.0–6.0) weeks; P < 0.01]. Short-term percentual decrease in acute-phase reactant levels (P < 0.001 for both erythrocyte sedimentation rate and C-reactive protein) and serum creatinine level (P < 0.01) following treatment initiation was greater in CS-treated patients compared with that in TMX-treated patients. Mass regression at first follow-up CT scan was observed more frequently in CS-treated patients (CS, 84.0% versus TMX, 68.3%; P = 0.05) with no difference in time interval from treatment initiation to first follow-up CT between groups [CS, 5 (2–7) months versus TMX, 4 (4–5) months; P = 0.34]. Definite treatment success was non-significantly higher in CS-treated patients (CS, 72.7% versus TMX, 58.3%; P = 0.15). In patients with initial treatment success with primary treatment, recurrence rate was lower in TMX-treated patients (CS, 62.5% versus TMX, 21.4%; P < 0.01). Conclusions CS are superior

  5. Osmolality of Cerebrospinal Fluid from Patients with Idiopathic Intracranial Hypertension (IIH)

    PubMed Central

    Wibroe, Elisabeth A.; Yri, Hanne M.; Jensen, Rigmor H.; Wibroe, Morten A.; Hamann, Steffen

    2016-01-01

    Introduction Idiopathic intracranial hypertension (IIH) is a disorder of increased intracranial fluid pressure (ICP) of unknown etiology. This study aims to investigate osmolality of cerebrospinal fluid (CSF) from patients with IIH. Methods We prospectively collected CSF from individuals referred on suspicion of IIH from 2011–2013. Subjects included as patients fulfilled Friedman and Jacobson’s diagnostic criteria for IIH. Individuals in whom intracranial hypertension was refuted were included as controls. Lumbar puncture with ICP measurement was performed at inclusion and repeated for patients after three months of treatment. Osmolality was measured with a Vapor Pressure Osmometer. Results We collected 90 CSF samples from 38 newly diagnosed patients and 28 controls. At baseline 27 IIH-samples and at 3 months follow-up 35 IIH-samples were collected from patients. We found no significant differences in osmolality between 1) patients at baseline and controls (p = 0. 86), 2) patients at baseline and after 3 months treatment (p = 0.97), and 3) patients with normalized pressure after 3 months and their baseline values (p = 0.79). Osmolality in individuals with normal ICP from 6–25 cmH2O (n = 41) did not differ significantly from patients with moderately elevated ICP from 26–45 cmH2O (n = 21) (p = 0.86) and patients with high ICP from 46–70 cmH2O (n = 4) (p = 0.32), respectively. There was no correlation between osmolality and ICP, BMI, age and body height, respectively. Mean CSF osmolality was 270 mmol/kg (± 1 SE, 95% confidence interval 267–272) for both patients and controls. Conclusions CSF osmolality was normal in patients with IIH, and there was no relation to treatment, ICP, BMI, age and body height. Mean CSF osmolality was 270 mmol/kg and constitutes a reference for future studies. Changes in CSF osmolality are not responsible for development of IIH. Other underlying pathophysiological mechanisms must be searched. PMID:26808050

  6. The use of cardioselective beta-blockers in a patient with idiopathic hypertrophic subaortic stenosis and chronic obstructive pulmonary disease.

    PubMed

    Bekker, Alex; Sorour, Khaled; Miller, Sanford

    2002-12-01

    The beta-adrenergic receptor blocking drugs are commonly used in the treatment of patients with idiopathic hypertropic subaortic stenosis (IHSS). These drugs, however, are contraindicated in patients with chronic obstructive pulmonary disease (COPD). We report the anesthetic management of a patient with IHSS complicated by severe COPD. We concluded that the beta(1) selective, ultra-short acting beta-blocker, esmolol, can be used intraoperatively when both conditions are present. The pathophysiology and the commonly used anesthetic drugs and practices for treatment of patients with IHSS are reviewed. PMID:12565117

  7. Unusual idiopathic normal pressure hydrocephalus patient with marked asymmetric and upper body parkinsonism

    PubMed Central

    Kang, Kyunghun; Choi, Dongho; Lee, Ho-Won

    2016-01-01

    Asymmetry of parkinsonian symptoms is strong evidence toward the diagnosis of Parkinson's disease (PD). Lower body parkinsonism is characteristic in idiopathic normal pressure hydrocephalus (INPH). We report an unusual INPH patient with marked asymmetric and upper body parkinsonism. An 83-year-old man presented with gait impairment and asymmetric clumsiness of movement. According to the Unified Parkinson's Disease Rating Scale (UPDRS), the motor subscore was 12 in the left limb and 8 in the right. The score was 14 for both the upper and lower body. After the cerebrospinal fluid tap test (CSFTT), he showed marked improvement in the upper body score. A loss of asymmetry of parkinsonian signs, with greater improvement in the left limb, was presented. Fluorinated N-3-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)-nortropane (F-18 FP-CIT) positron emission tomography (PET) imaging was normal. In the differential diagnosis of elderly patients presenting with parkinsonism compatible with PD, we might need to consider a diagnosis of INPH. PMID:27293338

  8. Unusual idiopathic normal pressure hydrocephalus patient with marked asymmetric and upper body parkinsonism.

    PubMed

    Kang, Kyunghun; Choi, Dongho; Lee, Ho-Won

    2016-01-01

    Asymmetry of parkinsonian symptoms is strong evidence toward the diagnosis of Parkinson's disease (PD). Lower body parkinsonism is characteristic in idiopathic normal pressure hydrocephalus (INPH). We report an unusual INPH patient with marked asymmetric and upper body parkinsonism. An 83-year-old man presented with gait impairment and asymmetric clumsiness of movement. According to the Unified Parkinson's Disease Rating Scale (UPDRS), the motor subscore was 12 in the left limb and 8 in the right. The score was 14 for both the upper and lower body. After the cerebrospinal fluid tap test (CSFTT), he showed marked improvement in the upper body score. A loss of asymmetry of parkinsonian signs, with greater improvement in the left limb, was presented. Fluorinated N-3-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)-nortropane (F-18 FP-CIT) positron emission tomography (PET) imaging was normal. In the differential diagnosis of elderly patients presenting with parkinsonism compatible with PD, we might need to consider a diagnosis of INPH. PMID:27293338

  9. Alveolar epithelial cells express mesenchymal proteins in patients with idiopathic pulmonary fibrosis.

    PubMed

    Marmai, Cecilia; Sutherland, Rachel E; Kim, Kevin K; Dolganov, Gregory M; Fang, Xiaohui; Kim, Sophia S; Jiang, Shuwei; Golden, Jeffery A; Hoopes, Charles W; Matthay, Michael A; Chapman, Harold A; Wolters, Paul J

    2011-07-01

    Prior work has shown that transforming growth factor-β (TGF-β) can mediate transition of alveolar type II cells into mesenchymal cells in mice. Evidence this occurs in humans is limited to immunohistochemical studies colocalizing epithelial and mesenchymal proteins in sections of fibrotic lungs. To acquire further evidence that epithelial-to-mesenchymal transition occurs in the lungs of patients with idiopathic pulmonary fibrosis (IPF), we studied alveolar type II cells isolated from fibrotic and normal human lung. Unlike normal type II cells, type II cells isolated from the lungs of patients with IPF express higher levels of mRNA for the mesenchymal proteins type I collagen, α-smooth muscle actin (α-SMA), and calponin. When cultured on Matrigel/collagen, human alveolar type II cells maintain a cellular morphology consistent with epithelial cells and expression of surfactant protein C (SPC) and E-cadherin. In contrast, when cultured on fibronectin, the human type II cells flatten, spread, lose expression of pro- SPC, and increase expression of vimentin, N-cadherin, and α-SMA; markers of mesenchymal cells. Addition of a TGF-β receptor kinase inhibitor (SB431542) to cells cultured on fibronectin inhibited vimentin expression and maintained pro-SPC expression, indicating persistence of an epithelial phenotype. These data suggest that alveolar type II cells can acquire features of mesenchymal cells in IPF lungs and that TGF-β can mediate this process. PMID:21498628

  10. Alveolar epithelial cells express mesenchymal proteins in patients with idiopathic pulmonary fibrosis

    PubMed Central

    Marmai, Cecilia; Sutherland, Rachel E.; Kim, Kevin K.; Dolganov, Gregory M.; Fang, Xiaohui; Kim, Sophia S.; Jiang, Shuwei; Golden, Jeffery A.; Hoopes, Charles W.; Matthay, Michael A.; Chapman, Harold A.

    2011-01-01

    Prior work has shown that transforming growth factor-β (TGF-β) can mediate transition of alveolar type II cells into mesenchymal cells in mice. Evidence this occurs in humans is limited to immunohistochemical studies colocalizing epithelial and mesenchymal proteins in sections of fibrotic lungs. To acquire further evidence that epithelial-to-mesenchymal transition occurs in the lungs of patients with idiopathic pulmonary fibrosis (IPF), we studied alveolar type II cells isolated from fibrotic and normal human lung. Unlike normal type II cells, type II cells isolated from the lungs of patients with IPF express higher levels of mRNA for the mesenchymal proteins type I collagen, α-smooth muscle actin (α-SMA), and calponin. When cultured on Matrigel/collagen, human alveolar type II cells maintain a cellular morphology consistent with epithelial cells and expression of surfactant protein C (SPC) and E-cadherin. In contrast, when cultured on fibronectin, the human type II cells flatten, spread, lose expression of pro- SPC, and increase expression of vimentin, N-cadherin, and α-SMA; markers of mesenchymal cells. Addition of a TGF-β receptor kinase inhibitor (SB431542) to cells cultured on fibronectin inhibited vimentin expression and maintained pro-SPC expression, indicating persistence of an epithelial phenotype. These data suggest that alveolar type II cells can acquire features of mesenchymal cells in IPF lungs and that TGF-β can mediate this process. PMID:21498628

  11. Reversal of idiopathic hypogonadotropic hypogonadism: a cohort study in Chinese patients.

    PubMed

    Mao, Jiang-Feng; Xu, Hong-Li; Duan, Jin; Chen, Rong-Rong; Li, Li; Li, Bin; Nie, Min; Min, Le; Zhang, Hong-Bing; Wu, Xue-Yan

    2015-01-01

    Although idiopathic hypogonadotropic hypogonadism (IHH) has traditionally been viewed as a life-long disease caused by a deficiency of gonadotropin-releasing hormone neurons, a portion of patients may gradually regain normal reproductive axis function during hormonal replacement therapy. The predictive factors for potential IHH reversal are largely unknown. The aim of our study was to investigate the incidence and clinical features of IHH male patients who had reversed reproductive axis function. In this retrospective cohort study, male IHH patients were classified into a reversal group (n = 18) and a nonreversal group (n = 336). Concentration of gonadotropins and testosterone, as well as testicle sizes and sperm counts, were determined. Of 354 IHH patients, 18 (5.1%) acquired normal reproductive function during treatment. The median age for reversal was 24 years old (range 21-34 years). Compared with the nonreversal group, the reversible group had higher basal luteinizing hormone (LH) (1.0 ± 0.7 IU l -[1] vs 0.4 ± 0.4 IU l-1 , P< 0.05) and stimulated LH (28.3 ± 22.6 IU l-1 vs 1.9 ± 1.1 IU l-1 , P< 0.01) levels, as well as larger testicle size (5.1 ± 2.6 ml vs 1.5 ± 0.3 ml, P< 0.01), at the initial visit. In summary, larger testicle size and higher stimulated LH concentrations are favorite parameters for reversal. Our finding suggests that reversible patients may retain partially active reproductive axis function at initial diagnosis. PMID:25578938

  12. Reversal of idiopathic hypogonadotropic hypogonadism: a cohort study in Chinese patients

    PubMed Central

    Mao, Jiang-Feng; Xu, Hong-Li; Duan, Jin; Chen, Rong-Rong; Li, Li; Li, Bin; Nie, Min; Min, Le; Zhang, Hong-Bing; Wu, Xue-Yan

    2015-01-01

    Although idiopathic hypogonadotropic hypogonadism (IHH) has traditionally been viewed as a life-long disease caused by a deficiency of gonadotropin-releasing hormone neurons, a portion of patients may gradually regain normal reproductive axis function during hormonal replacement therapy. The predictive factors for potential IHH reversal are largely unknown. The aim of our study was to investigate the incidence and clinical features of IHH male patients who had reversed reproductive axis function. In this retrospective cohort study, male IHH patients were classified into a reversal group (n = 18) and a nonreversal group (n = 336). Concentration of gonadotropins and testosterone, as well as testicle sizes and sperm counts, were determined. Of 354 IHH patients, 18 (5.1%) acquired normal reproductive function during treatment. The median age for reversal was 24 years old (range 21–34 years). Compared with the nonreversal group, the reversible group had higher basal luteinizing hormone (LH) (1.0 ± 0.7 IU l-1 vs 0.4 ± 0.4 IU l−1, P < 0.05) and stimulated LH (28.3 ± 22.6 IU l−1 vs 1.9 ± 1.1 IU l−1, P < 0.01) levels, as well as larger testicle size (5.1 ± 2.6 ml vs 1.5 ± 0.3 ml, P < 0.01), at the initial visit. In summary, larger testicle size and higher stimulated LH concentrations are favorite parameters for reversal. Our finding suggests that reversible patients may retain partially active reproductive axis function at initial diagnosis. PMID:25578938

  13. Polymorphisms of genes encoding interleukin-4 and its receptor in Iranian patients with juvenile idiopathic arthritis.

    PubMed

    Ziaee, Vahid; Rezaei, Arezou; Harsini, Sara; Maddah, Marzieh; Zoghi, Samaneh; Sadr, Maryam; Moradinejad, Mohammad Hassan; Rezaei, Nima

    2016-08-01

    As cytokines, including interleukin-4 (IL-4), seem to have a pivotal role in the pathogenesis of juvenile idiopathic arthritis (JIA), this study is aimed at investigating of association of polymorphisms in IL-4 and IL-4 receptor α (IL-4RA) genes with susceptibility to JIA. A case-control study was conducted on 53 patients with JIA and 139 healthy unrelated controls. Single nucleotide polymorphisms of IL-4 gene at positions -1098, -590, and -33, as well as IL-4RA gene at position +1902 were genotyped using polymerase chain reaction with sequence-specific primers method and compared between patients and healthy individuals. At the allelic level, C allele at IL-4 -33 was found to be more frequent in patients compared to control (P value <0.01). At the genotypic level, CC genotype at IL-4 -590 (P value <0.01), together with CC and TT genotypes at IL-4 -33 (P value <0.01), were significantly higher in patients with JIA, while TC genotypes at IL-4 -590 and -33 positions were found to be lower in case group (P value <0.01). At the haplotypic level, IL-4 (positions -1098, -509, -33) TTC, GCC, and TTT haplotypes were significantly lower than controls (P value <0.01, P value = 0.03, and P value = 0.04, respectively). Although, TCC haplotype at the same positions was found to be higher in patients (P value <0.01). Polymorphic site of +1902 IL-4RA gene did not differ between cases and controls. Polymorphisms in promoter region of IL-4 but not IL-4RA genes confer susceptibility to JIA and may predispose individuals to adaptive immune responses. PMID:26951255

  14. Scoliosis in cystic fibrosis - an appraisal

    SciTech Connect

    Paling, M.R.; Spasovsky-Chernick, M.

    1982-03-01

    An unusually high prevalence (10%) of scoliosis is described in a series of 151 patients aged four years and older with cystic fibrosis. The scolioses were of the late onset (juvenile and adolescent) type, being typically thoracic with the curve convex to the right, although there was no significant preference for either sex. No direct relationship was found between the spinal curvature and the severity or distribution of the lung disease, although the worse scolioses tended to occur in patients with relatively severe pulmonary involvement. There was no evidence of metabolic bone disease as a predisposing cause. Some indication of a familial tendency towards scoliosis was apparent, and a genetic or constitutional basis is postulated with an unknown precipitating factor.

  15. Bi-ventricular circulatory support with the Abiomed AB5000 system in a patient with idiopathic refractory ventricular fibrillation.

    PubMed

    Zhang, Li; Kapetanakis, Emmanouil I; Cooke, Richard H; Sweet, Leslie C; Boyce, Steven W

    2007-01-01

    A 45-year-old man had life-threatening recurrent idiopathic ventricular fibrillation and persistent cardiogenic shock develop. The episodes of ventricular fibrillation were refractory to aggressive medical management; therefore an Abiomed AB5000 bi-ventricular support system was implanted for arrhythmia control. The device was able to maintain hemodynamic stability during the following 2 weeks. The patient was discharged from the hospital with fully recovered cardiac function. PMID:17184687

  16. A Newfoundland cohort of familial and sporadic idiopathic pulmonary fibrosis patients: clinical and genetic features

    PubMed Central

    2012-01-01

    Background Idiopathic pulmonary fibrosis (IPF) is an adult-onset Idiopathic Interstitial Pneumonia (IIP) usually diagnosed between age 50 to 70 years. Individuals with Familial Pulmonary Fibrosis (FPF) have at least one affected first or second-degree relative and account for 0.5-20% of cases. Methods We ascertained and collected DNA samples from a large population-based cohort of IPF patients from Newfoundland, Canada. For each proband, a family history was documented and medical records were reviewed. Each proband was classified as familial (28 patients) or sporadic (50 patients) and all 78 probands were screened for variants in four highly penetrant, adult-onset PF genes (SFTPC, SFTPA2, TERT,TERC). Results Seventy-eight IPF probands were enrolled of whom 28 (35.9%) had a positive family history. These 28 familial patients led to the recruitment of an additional 49 affected relatives (total of 77 FPF patients). By age 60 years, 42% of the familial cohort had been diagnosed with PF compared with only 16% of the sporadic patient collection (χ2 = 8.77, p = 0.003). Mean age of diagnosis in the familial group was significantly younger than the sporadic group (61.4 years vs. 66.6 yrs, p = 0.012) with a wider age range of diagnosis (19–92 years compared with 47–82 years). Thirty-three of 77 (42.8%) FPF patients had a tissue diagnosis and all but five had usual interstitial pneumonia histology. Compared with other published case series, the familial IIP histologies were more homogeneous. Three of 28 familial probands (10.7%) and none of the 50 sporadic probands had pathogenic variants in the four genes tested. All three familial probands had mutations in TERT. Other phenotypes associated with telomerase deficiency were present in these families including cirrhosis, bone marrow hypoplasia and premature graying. Telomere length assays were performed on mutation carriers from two families and confirmed telomere-related deficiency. Conclusion The

  17. A Patient-Specific Foot Model for the Estimate of Ankle Joint Forces in Patients with Juvenile Idiopathic Arthritis.

    PubMed

    Prinold, Joe A I; Mazzà, Claudia; Di Marco, Roberto; Hannah, Iain; Malattia, Clara; Magni-Manzoni, Silvia; Petrarca, Maurizio; Ronchetti, Anna B; Tanturri de Horatio, Laura; van Dijkhuizen, E H Pieter; Wesarg, Stefan; Viceconti, Marco

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the leading cause of childhood disability from a musculoskeletal disorder. It generally affects large joints such as the knee and the ankle, often causing structural damage. Different factors contribute to the damage onset, including altered joint loading and other mechanical factors, associated with pain and inflammation. The prediction of patients' joint loading can hence be a valuable tool in understanding the disease mechanisms involved in structural damage progression. A number of lower-limb musculoskeletal models have been proposed to analyse the hip and knee joints, but juvenile models of the foot are still lacking. This paper presents a modelling pipeline that allows the creation of juvenile patient-specific models starting from lower limb kinematics and foot and ankle MRI data. This pipeline has been applied to data from three children with JIA and the importance of patient-specific parameters and modelling assumptions has been tested in a sensitivity analysis focused on the variation of the joint reaction forces. This analysis highlighted the criticality of patient-specific definition of the ankle joint axes and location of the Achilles tendon insertions. Patient-specific detection of the Tibialis Anterior, Tibialis Posterior, and Peroneus Longus origins and insertions were also shown to be important. PMID:26374518

  18. Interleukin-7 treatment of PML in a patient with idiopathic lymphocytopenia

    PubMed Central

    Miskin, Dhanashri P.; Chalkias, Spyridon G.; Dang, Xin; Bord, Evelyn; Batson, Stephanie

    2016-01-01

    Objective: To describe the compassionate use of interleukin-7 (IL-7) for treatment of progressive multifocal leukoencephalopathy (PML) in the setting of idiopathic CD8+ greater than CD4+ lymphocytopenia. Methods: A 66-year-old HIV-seronegative man presented with progressive language dysfunction. MRI showed hyperintense lesions in the left hemispheric white matter with mild contrast enhancement. A brain biopsy performed 4 months after symptom onset established the diagnosis of PML. The patient had profound lymphocytopenia with absolute lymphocyte count (ALC) at 168 cells/μL, 87 CD4+ T cells/μL, and 7 CD8+ T cells/μL. There was no evidence of hematologic malignancy or rheumatologic disease. Results: The patient received 3 intramuscular injections of IL-7 at a dose of 10 μg/kg per week with no adverse effects. ALC peaked at 595 cells/μL, CD4+ T cells at 301 cells/μL, and CD8+ T cells at 34 cells/μL 3 weeks after completion of treatment. His lesions on MRI stabilized and neurologic examination mildly improved. JCV-specific T-cell responses measured by intracellular cytokine staining were not altered after treatment with IL-7 but there was a marked increase in regulatory T cells. Conclusion: This case further supports the investigational use of IL-7 in patients who develop PML in the setting of ICL. Classification of evidence: This study provides Class IV evidence that for patients with ICL and PML, IL-7 improves PML-related-outcomes. The study is rated Class IV because it is a case report. PMID:27144212

  19. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry.

    PubMed

    Behr, Jürgen; Kreuter, Michael; Hoeper, Marius M; Wirtz, Hubert; Klotsche, Jens; Koschel, Dirk; Andreas, Stefan; Claussen, Martin; Grohé, Christian; Wilkens, Henrike; Randerath, Winfried; Skowasch, Dirk; Meyer, F Joachim; Kirschner, Joachim; Gläser, Sven; Herth, Felix J F; Welte, Tobias; Huber, Rudolf Maria; Neurohr, Claus; Schwaiblmair, Martin; Kohlhäufl, Martin; Höffken, Gert; Held, Matthias; Koch, Andrea; Bahmer, Thomas; Pittrow, David

    2015-07-01

    After introduction of the new international guidelines on idiopathic pulmonary fibrosis (IPF) in 2011, we investigated clinical management practices for patients with IPF according to physicians' diagnoses. A prospective, multicenter, noninterventional study with comprehensive quality measures including on-site source data verification was performed in Germany. 502 consecutive patients (171 newly diagnosed, 331 prevalent; mean±SD age 68.7±9.4 years, 77.9% males) with a mean disease duration of 2.3±3.5 years were enrolled. IPF diagnosis was based on clinical assessments and high-resolution computed tomography (HRCT) in 90.2%, and on surgical lung biopsy combined with histology in 34.1% (lavage in 61.8%). The median 6-min walk distance was 320 m (mean 268±200 m). The mean forced vital capacity was 72±20% pred and diffusing capacity of the lung for carbon monoxide was 35±15% pred. No drugs were administered in 17.9%, oral steroids in 23.7%, N-acetylcysteine in 33.7%, pirfenidone in 44.2% and other drugs in 4.6% of patients. Only 2.8% of the cohort was listed for lung transplantation. IPF patients were diagnosed in line with the new guidelines. They had more severe disease than those enrolled in recent randomised controlled trials. In addition to HRCT, the frequency of lung biopsies was surprisingly high. Treatment patterns varied substantially. PMID:25837040

  20. Neurodegenerative Disorder Risk in Idiopathic REM Sleep Behavior Disorder: Study in 174 Patients

    PubMed Central

    Iranzo, Alex; Fernández-Arcos, Ana; Tolosa, Eduard; Serradell, Mónica; Molinuevo, José Luis; Valldeoriola, Francesc; Gelpi, Ellen; Vilaseca, Isabel; Sánchez-Valle, Raquel; Lladó, Albert; Gaig, Carles; Santamaría, Joan

    2014-01-01

    Objective To estimate the risk for developing a defined neurodegenerative syndrome in a large cohort of idiopathic REM sleep behavior disorder (IRBD) patients with long follow-up. Methods Using the Kaplan-Meier method, we estimated the disease-free survival rate from defined neurodegenerative syndromes in all the consecutive IRBD patients diagnosed and followed-up in our tertiary referal sleep center between November 1991 and July 2013. Results The cohort comprises 174 patients with a median age at diagnosis of IRBD of 69 years and a median follow-up of four years. The risk of a defined neurodegenerative syndrome from the time of IRBD diagnosis was 33.1% at five years, 75.7% at ten years, and 90.9% at 14 years. The median conversion time was 7.5 years. Emerging diagnoses (37.4%) were dementia with Lewy bodies (DLB) in 29 subjects, Parkinson disease (PD) in 22, multiple system atrophy (MSA) in two, and mild cognitive impairment (MCI) in 12. In six cases, in whom postmortem was performed, neuropathological examination disclosed neuronal loss and widespread Lewy-type pathology in the brain in each case. Conclusions In a large IRBD cohort diagnosed in a tertiary referal sleep center, prolonged follow-up indicated that the majority of patients are eventually diagnosed with the synucleinopathies PD, DLB and less frequently MSA. IRBD represented the prodromal period of these conditions. Our findings in IRBD have important implications in clinical practice, in the investigation of the early pathological events occurring in the synucleinopathies, and for the design of interventions with potential disease-modifying agents. PMID:24587002

  1. Altered Responses to Homeostatic Cytokines in Patients with Idiopathic CD4 Lymphocytopenia

    PubMed Central

    Mouthon, Luc; Landires, Ivan; Rohrlich, Pierre; Pestre, Vincent; Thèze, Jacques; Lortholary, Olivier; Chakrabarti, Lisa A.

    2013-01-01

    Idiopathic CD4 lymphocytopenia (ICL) is a rare immune deficiency characterized by a protracted CD4+ T cell loss of unknown etiology and by the occurrence of opportunistic infections similar to those seen in AIDS. We investigated whether a defect in responses to cytokines that control CD4+ T cell homeostasis could play a role in ICL. Immunophenotype and signaling responses to interleukin-7 (IL-7), IL-2, and thymic stromal lymphopoietin (TSLP) were analyzed by flow cytometry in CD4+ T cells from 15 ICL patients and 15 healthy blood donors. The induction of phospho-STAT5 after IL-7 stimulation was decreased in memory CD4+ T cells of some ICL patients, which correlated with a decreased expression of the IL-7Rα receptor chain (R = 0.74, p<0.005) and with lower CD4+ T cell counts (R = 0.69, p<0.005). IL-2 responses were also impaired, both in the Treg and conventional memory subsets. Decreased IL-2 responses correlated with decreased IL-7 responses (R = 0.75, p<0.005), pointing to combined defects that may significantly perturb CD4+ T cell homeostasis in a subset of ICL patients. Unexpectedly, responses to the IL-7-related cytokine TSLP were increased in ICL patients, while they remained barely detectable in healthy controls. TSLP responses correlated inversely with IL-7 responses (R = −0.41; p<0.05), suggesting a cross-regulation between the two cytokine systems. In conclusion, IL-7 and IL-2 signaling are impaired in ICL, which may account for the loss of CD4+ T cell homeostasis. Increased TSLP responses point to a compensatory homeostatic mechanism that may mitigate defects in γc cytokine responses. PMID:23383227

  2. Plasma immunoreactive gamma melanotropin in patients with idiopathic hyperaldosteronism, aldosterone-producing adenomas, and essential hypertension.

    PubMed Central

    Griffing, G T; Berelowitz, B; Hudson, M; Salzman, R; Manson, J A; Aurrechia, S; Melby, J C; Pedersen, R C; Brownie, A C

    1985-01-01

    A non-ACTH aldosterone-stimulating factor(s) has been implicated in the pathogenesis of idiopathic hyperaldosteronism (IHA). Although this factor has not been fully characterized, some evidence suggests that it may be related to a pro-gamma-melanotropin (pro-gamma-MSH), derived from the NH2-terminal region of pro-opiomelanocortin. In the present study, plasma immunoreactive (IR-) gamma-MSH levels at 0800 h in patients with IHA were evaluated (90 +/- 17 fmol/ml; range: 13-173 fmol/ml) and found to be significantly higher (P less than 0.05) than those in subjects with aldosterone-producing adenomas (33 +/- 8 fmol/ml), essential hypertension (33 +/- 6 fmol/ml), and normotensive controls (19 +/- 2 fmol/ml). Seven of nine IHA subjects had circulating IR-gamma-MSH levels above the normal range (greater than 35 fmol/ml). In plasmas sampled at 1200 h, IR-gamma-MSH was significantly higher in patients with IHA (95 +/- 26 fmol/ml) and adenomas (63 +/- 23 fmol/ml), as compared with essential hypertensives (31 +/- 6 fmol/ml) and normotensives (19 +/- 3 fmol/ml). Mean plasma IR-ACTH, plasma cortisol, and urinary cortisol levels did not differ significantly between any of these groups. In order to evaluate the effect of a pro-gamma-MSH in vitro, adrenal adenoma tissue was obtained from two patients, one with elevated IR-gamma-MSH (61 fmol/ml) and a second with low IR-gamma-MSH (12 fmol/ml). Aldosterone secretion by dispersed adenoma cells from the former, but not the latter, underwent a fourfold dose-dependent (10(-14)-10(-9) M) increase in response to human Lys-gamma 3-MSH. These data suggest that a pro-gamma-MSH may be implicated as a pathogenic factor in a subset of patients with primary aldosteronism, particularly among those differentially diagnosed as having IHA. Images PMID:4019776

  3. Periostin promotes fibrosis and predicts progression in patients with idiopathic pulmonary fibrosis

    PubMed Central

    Naik, Payal K.; Bozyk, Paul D.; Bentley, J. Kelley; Popova, Antonia P.; Birch, Carolyn M.; Wilke, Carol A.; Fry, Christopher D.; White, Eric S.; Sisson, Thomas H.; Tayob, Nabihah; Carnemolla, Barbara; Orecchia, Paola; Flaherty, Kevin R.; Hershenson, Marc B.; Murray, Susan; Martinez, Fernando J.

    2012-01-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease without effective therapeutics. Periostin has been reported to be elevated in IPF patients relative to controls, but its sources and mechanisms of action remain unclear. We confirm excess periostin in lungs of IPF patients and show that IPF fibroblasts produce periostin. Blood was obtained from 54 IPF patients (all but 1 with 48 wk of follow-up). We show that periostin levels predict clinical progression at 48 wk (hazard ratio = 1.47, 95% confidence interval = 1.03–2.10, P < 0.05). Monocytes and fibrocytes are sources of periostin in circulation in IPF patients. Previous studies suggest that periostin may regulate the inflammatory phase of bleomycin-induced lung injury, but periostin effects during the fibroproliferative phase of the disease are unknown. Wild-type and periostin-deficient (periostin−/−) mice were anesthetized and challenged with bleomycin. Wild-type mice were injected with bleomycin and then treated with OC-20 Ab (which blocks periostin and integrin interactions) or control Ab during the fibroproliferative phase of disease, and fibrosis and survival were assessed. Periostin expression was upregulated quickly after treatment with bleomycin and remained elevated. Periostin−/− mice were protected from bleomycin-induced fibrosis. Instillation of OC-20 during the fibroproliferative phase improved survival and limited collagen deposition. Chimeric mouse studies suggest that hematopoietic and structural sources of periostin contribute to lung fibrogenesis. Periostin was upregulated by transforming growth factor-β in lung mesenchymal cells, and periostin promoted extracellular matrix deposition, mesenchymal cell proliferation, and wound closure. Thus periostin plays a vital role in late stages of pulmonary fibrosis and is a potential biomarker for disease progression and a target for therapeutic intervention. PMID:23043074

  4. Patterns of compensation of functional deficits of the knee joint in patients with juvenile idiopathic arthritis

    PubMed Central

    Księżopolska-Orłowska, Krystyna

    2015-01-01

    Objectives Juvenile idiopathic arthritis (JIA) is a group of pathological syndromes of unknown aetiology, observed at the developmental age. Their common feature is sustained chronic arthritis with flares and remissions. Clinical signs and symptoms include joint pain, periarticular tissue oedema or articular exudate, frequently associated with hypertrophy of the synovial membrane. The intra- and extra-articular structural damage impairs the motion range and smoothness. The disease process may involve any joint. The knee joint is the most frequently affected in oligo- and polyarthritis. The aim of the study was to determine a direct correlation between disorders of knee joint function and the change in the range of motion of the ankle and hip joints of both lower extremities, and the so-called indirect impact of these changes on patients’ posture. Material and methods The study included 36 JIA patients and 56 healthy controls aged 8–16 years. The evaluation was based on physical examination. Results The results showed differences in the values of quality and range of motion between patients and controls. In the patient group pes planovalgus was more frequently associated with knee joint dysfunction along with the inherent restriction of dorsal flexion of the foot. Shortening of the iliotibial band, increased outward rotation of the right lower extremity with enlarged joint contour and augmented inward rotation of the contralateral healthy extremity all proved significant. Changes in motion range in the joints below and over the knee were associated with alterations of antero-posterior spine curvatures and vertebral rotation along the long spinal axis. Based on the results, the mechanism of the compensation is outlined. Conclusions The observed differences in the range and quality of motion in the ankle, hip and spinal joints between patients and healthy children provide evidence that dysfunction of the knee joint affects the function of the other above

  5. Clinical outcomes of 114 patients who underwent γ-knife radiosurgery for medically refractory idiopathic trigeminal neuralgia.

    PubMed

    Li, Peng; Wang, Wei; Liu, Yi; Zhong, Qi; Mao, Boyong

    2012-01-01

    The optimal radiation dose and target of Gamma-knife radiosurgery (GKRS) for medically refractory idiopathic trigeminal neuralgia (TN) are contentious. We investigated the effects and trigeminal nerve deficits of GKRS using two isocenters to treat a great length of the trigeminal nerve. Between January 2005 and March 2010, 129 patients with idiopathic TN underwent GKRS at the West China Hospital of Sichuan University. A maximum central dose of 80-90 Gy was delivered to the trigeminal nerve root with two isocenters via a 4mm collimator helmet. One hundred and fourteen patients were followed-up periodically by telephone interview to determine the effects, trigeminal nerve deficits and time to the onset of pain relief. The mean follow-up duration was 29.6 months. One hundred and nine patients had complete or partial pain relief and the treatment failed in five patients. Nine patients experienced a recurrence after a mean time of 12.7 months, following an initial interval of pain relief. There were no significant differences between patients with different grades of pain relief with respect to central doses. The mean time to the onset of pain relief was 3.6 weeks. The time to the onset of complete pain relief was significantly shorter than that for partial pain relief. Forty-nine patients reported mild-to-moderate facial numbness and one patient experienced paroxysmal temporalis muscle spasms two weeks after the treatment. GKRS treatment for medically refractory idiopathic TN with two isocenters resulted in an initial pain improvement in 95.6% of patients. The early response to the treatment might suggest a good outcome but, given the high incidence of nerve deficits, GKRS for TN with two isocenters is not recommended as a routine treatment protocol. PMID:22154202

  6. Scoliosis, spinal fusion, and intrathecal baclofen pump implantation.

    PubMed

    Scannell, Brian; Yaszay, Burt

    2015-02-01

    This article reviews the incidence, management, and complications of scoliosis in patients with cerebral palsy. Treatment of scoliosis in patients with cerebral palsy includes both nonoperative and operative management and often the decision to proceed with surgery is a multidisciplinary decision. Because of severe spasticity, many of these patients undergo intrathecal baclofen pump placement before, during, or after posterior spinal fusion. The complication rates can be high with intrathecal baclofen pump placement, but many patients can have significant benefit with this therapy. PMID:25479781

  7. Scoliosis in Herlyn-Werner-Wunderlich syndrome: a case report and literature review.

    PubMed

    Li, Zheng; Yu, Xin; Shen, Jianxiong; Liang, Jinqian

    2014-12-01

    Herlyn-Werner-Wunderlich syndrome (HWWS) is a congenital Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Little is reported about spinal deformity associated with this syndrome. This study presents a case of scoliosis occurring in the setting of HWWS and explores the possible association between the 2 diseases. A previously unreported scoliosis in HWWS is described. The patient is a 12-year-old Chinese female with scoliosis that underwent a posterior correction at thoracic 5-thoracic 12 (T5-T12) levels, using the Moss-SI (Johnson & Johnson, American) spinal system. At 24-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. Six months after scoliosis correction surgery, the patient went to our clinics for the treatment of HWWS. She was performed a vaginal septum resection and detected with pyocolpos. Her follow-up was symptom free at the fourth postoperative month. The prevalence of scoliosis among patients with HWWS was 8.57% that is much higher than the incidence of congential scoliosis among general population (1/1000). To the best of our knowledge, this is the first report of HWWS with thoracic scoliosis. During surgery, surgeons and anesthesiologists must pay particular attention to the Müllerian duct anomaly and renal agenesis associated with HWWS. There is a potential association between congenital scoliosis and HWWS. PMID:25526433

  8. Pathological integrin signaling enhances proliferation of primary lung fibroblasts from patients with idiopathic pulmonary fibrosis.

    PubMed

    Xia, Hong; Diebold, Deanna; Nho, Richard; Perlman, David; Kleidon, Jill; Kahm, Judy; Avdulov, Svetlana; Peterson, Mark; Nerva, John; Bitterman, Peter; Henke, Craig

    2008-07-01

    Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive lung disease in which fibroblasts accumulate in the alveolar wall within a type I collagen-rich matrix. Although lung fibroblasts derived from patients with IPF display durable pathological alterations in proliferative function, the molecular mechanisms differentiating IPF fibroblasts from their normal counterparts remain unknown. Polymerized type I collagen normally inhibits fibroblast proliferation, providing a physiological mechanism to limit fibroproliferation after tissue injury. We demonstrate that beta1 integrin interaction with polymerized collagen inhibits normal fibroblast proliferation by suppression of the phosphoinositide 3-kinase (PI3K)-Akt-S6K1 signal pathway due to maintenance of high phosphatase activity of the tumor suppressor phosphatase and tensin homologue (PTEN). In contrast, IPF fibroblasts eluded this restraint, displaying a pathological pattern of beta1 integrin signaling in response to polymerized collagen that leads to aberrant activation of the PI3K-Akt-S6K1 signal pathway caused by inappropriately low PTEN activity. Mice deficient in PTEN showed a prolonged fibroproliferative response after tissue injury, and immunohistochemical analysis of IPF lung tissue demonstrates activation of Akt in cells within fibrotic foci. These results provide direct evidence for defective negative regulation of the proliferative pathway in IPF fibroblasts and support the theory that the pathogenesis of IPF involves an intrinsic fibroblast defect. PMID:18541712

  9. Pathological integrin signaling enhances proliferation of primary lung fibroblasts from patients with idiopathic pulmonary fibrosis

    PubMed Central

    Xia, Hong; Diebold, Deanna; Nho, Richard; Perlman, David; Kleidon, Jill; Kahm, Judy; Avdulov, Svetlana; Peterson, Mark; Nerva, John; Bitterman, Peter; Henke, Craig

    2008-01-01

    Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive lung disease in which fibroblasts accumulate in the alveolar wall within a type I collagen–rich matrix. Although lung fibroblasts derived from patients with IPF display durable pathological alterations in proliferative function, the molecular mechanisms differentiating IPF fibroblasts from their normal counterparts remain unknown. Polymerized type I collagen normally inhibits fibroblast proliferation, providing a physiological mechanism to limit fibroproliferation after tissue injury. We demonstrate that β1 integrin interaction with polymerized collagen inhibits normal fibroblast proliferation by suppression of the phosphoinositide 3-kinase (PI3K)–Akt–S6K1 signal pathway due to maintenance of high phosphatase activity of the tumor suppressor phosphatase and tensin homologue (PTEN). In contrast, IPF fibroblasts eluded this restraint, displaying a pathological pattern of β1 integrin signaling in response to polymerized collagen that leads to aberrant activation of the PI3K–Akt–S6K1 signal pathway caused by inappropriately low PTEN activity. Mice deficient in PTEN showed a prolonged fibroproliferative response after tissue injury, and immunohistochemical analysis of IPF lung tissue demonstrates activation of Akt in cells within fibrotic foci. These results provide direct evidence for defective negative regulation of the proliferative pathway in IPF fibroblasts and support the theory that the pathogenesis of IPF involves an intrinsic fibroblast defect. PMID:18541712

  10. Gene expression of muscular and neuronal pathways is cooperatively dysregulated in patients with idiopathic achalasia.

    PubMed

    Palmieri, Orazio; Mazza, Tommaso; Merla, Antonio; Fusilli, Caterina; Cuttitta, Antonello; Martino, Giuseppina; Latiano, Tiziana; Corritore, Giuseppe; Bossa, Fabrizio; Palumbo, Orazio; Muscarella, Lucia Anna; Carella, Massimo; Graziano, Paolo; Andriulli, Angelo; Latiano, Anna

    2016-01-01

    Idiopathic achalasia is characterized by the absence of peristalsis secondary to loss of neurons in the myenteric plexus that hampers proper relaxation of the lower esophageal sphincter. Achalasia can be considered a multifactorial disorder as it occurs in related individuals and is associated with HLA class II genes, thereby suggesting genetic influence. We used microarray technology and advanced in-silico functional analyses to perform the first genome-wide expression profiling of mRNA in tissue samples from 12 achalasia and 5 control patients. It revealed 1,728 differentially expressed genes, of these, 837 (48.4%) were up-regulated in cases. In particular, genes participating to the smooth muscle contraction biological function were mostly up-regulated. Functional analysis revealed a significant enrichment of neuronal/muscular and neuronal/immunity processes. Upstream regulatory analysis of 180 genes involved in these processes suggested TLR4 and IL18 as critical key-players. Two functional gene networks were significantly over-represented: one involved in organ morphology, skeletal muscle system development and function, and neurological diseases, and the other participating in cell morphology, humoral immune response and cellular movement. These results highlight on pivotal genes that may play critical roles in neuronal/muscular and neuronal/immunity processes, and that may contribute to the onset and development of achalasia. PMID:27511445

  11. Recombinant human pentraxin-2 therapy in patients with idiopathic pulmonary fibrosis: safety, pharmacokinetics and exploratory efficacy.

    PubMed

    van den Blink, Bernt; Dillingh, Marlous R; Ginns, Leo C; Morrison, Lake D; Moerland, Matthijs; Wijsenbeek, Marlies; Trehu, Elizabeth G; Bartholmai, Brian J; Burggraaf, Jacobus

    2016-03-01

    Abnormal fibrogenic repair response upon alveolar injury is believed to play an important role in the pathogenesis of idiopathic pulmonary fibrosis (IPF). PRM-151 (recombinant human pentraxin-2, also known as serum amyloid P), has been shown to reduce fibrosis in preclinical lung fibrosis models, and was well tolerated with a favourable pharmacokinetic profile in an earlier single-dose phase I study.A randomised, double-blind, placebo-controlled, multiple ascending dose trial was performed to assess the tolerability and pharmacokinetic and pharmacodynamic characteristics of multiple doses of PRM-151 in IPF patients. Subjects in three successive cohorts (1, 5, or 10 mg·kg(-1) versus placebo) received intravenous study drug on days 1, 3, 5, 8 and 15, and were followed-up to day 57.PRM-151 was well tolerated at all dose levels, with no serious adverse reactions. Administration of PRM-151 resulted in two- to eight-fold dose-dependent increases in circulating pentraxin-2 levels. Forced vital capacity and 6-min walk test showed trends towards improvement in the combined PRM-151 dose groups. On high-resolution computed tomography scans, stable or improved lung volume unoccupied by interstitial lung abnormality was noted in some PRM-151 subjects compared to placebo subjects on day 57.The efficacy of PRM-151 in IPF remains to be investigated in dedicated future trials. PMID:26869678

  12. Ureaplasma septic arthritis in an immunosuppressed patient with juvenile idiopathic arthritis.

    PubMed

    George, Michael David; Cardenas, Ana Maria; Birnbaum, Belinda K; Gluckman, Stephen J

    2015-06-01

    Mycoplasmas, including Ureaplasma and Mycoplasma species, are uncommon but important causes of septic arthritis, especially affecting immunosuppressed patients. Many of the reported cases have been associated with congenital immunodeficiency disorders, especially hypogammaglobulinemia. Mycoplasmas are difficult to grow in the laboratory, and these infections may be underdiagnosed using culture techniques. We report a case of a 21-year-old woman with juvenile idiopathic arthritis and hip arthroplasties treated with rituximab and adalimumab who developed urogenital infections and soft tissue abscesses followed by knee arthritis with negative routine cultures. Ureaplasma species was identified from synovial fluid on 2 separate occasions using a broad-range 16S ribosomal RNA gene polymerase chain reaction. Azithromycin led to rapid improvement in symptoms, but after completion of therapy, involvement of the hip prosthesis became apparent, and again, 16S rRNA gene polymerase chain reaction was positive for Ureaplasma species. The literature is reviewed with a discussion of risk factors for Mycoplasma septic arthritis, clinical presentation, methods of diagnosis, and treatment. PMID:26010188

  13. Gene expression of muscular and neuronal pathways is cooperatively dysregulated in patients with idiopathic achalasia

    PubMed Central

    Palmieri, Orazio; Mazza, Tommaso; Merla, Antonio; Fusilli, Caterina; Cuttitta, Antonello; Martino, Giuseppina; Latiano, Tiziana; Corritore, Giuseppe; Bossa, Fabrizio; Palumbo, Orazio; Muscarella, Lucia Anna; Carella, Massimo; Graziano, Paolo; Andriulli, Angelo; Latiano, Anna

    2016-01-01

    Idiopathic achalasia is characterized by the absence of peristalsis secondary to loss of neurons in the myenteric plexus that hampers proper relaxation of the lower esophageal sphincter. Achalasia can be considered a multifactorial disorder as it occurs in related individuals and is associated with HLA class II genes, thereby suggesting genetic influence. We used microarray technology and advanced in-silico functional analyses to perform the first genome-wide expression profiling of mRNA in tissue samples from 12 achalasia and 5 control patients. It revealed 1,728 differentially expressed genes, of these, 837 (48.4%) were up-regulated in cases. In particular, genes participating to the smooth muscle contraction biological function were mostly up-regulated. Functional analysis revealed a significant enrichment of neuronal/muscular and neuronal/immunity processes. Upstream regulatory analysis of 180 genes involved in these processes suggested TLR4 and IL18 as critical key-players. Two functional gene networks were significantly over-represented: one involved in organ morphology, skeletal muscle system development and function, and neurological diseases, and the other participating in cell morphology, humoral immune response and cellular movement. These results highlight on pivotal genes that may play critical roles in neuronal/muscular and neuronal/immunity processes, and that may contribute to the onset and development of achalasia. PMID:27511445

  14. Idiopathic pulmonary haemosiderosis: spectrum of thoracic imaging findings in the adult patient.

    PubMed

    Khorashadi, L; Wu, C C; Betancourt, S L; Carter, B W

    2015-05-01

    Idiopathic pulmonary haemosiderosis (IPH) is a rare disease characterized by alveolar capillary haemorrhage resulting in deposition and accumulation of haemosiderin in the lungs. Although its precise pathophysiology remains unclear, several hypotheses have been proposed to explain the aetiology of the disorder, including autoimmune, environmental, allergic, and genetic theories. IPH is typically diagnosed in childhood, usually before the age of 10 years; however, this entity may be encountered in older patients given the greater awareness of the diagnosis, availability and utilization of advanced imaging techniques, and improved treatment and survival. The classic presentation of IPH consists of the triad of haemoptysis, iron-deficiency anaemia, and pulmonary opacities on chest radiography. The diagnosis is usually confirmed via bronchoscopy with bronchoalveolar lavage (BAL), at which time haemosiderin-laden macrophages referred to as siderophages, considered pathognomonic for IPH, may be identified. However, lung biopsy may ultimately be necessary to exclude other disease processes. For children with IPH, the disease course is severe and the prognosis is poor. However, adults generally have a longer disease course with milder symptoms and the prognosis is more favourable. Specific imaging features, although non-specific in isolation, may be identified on thoracic imaging studies, principally chest radiography and CT, depending on the phase of disease (acute or chronic). Recognition of these findings is important to guide appropriate clinical management. PMID:25515792

  15. Studies of the prevalence and significance of radiolabeled bile acid malabsorption in a group of patients with idiopathic chronic diarrhea

    SciTech Connect

    Schiller, L.R.; Hogan, R.B.; Morawski, S.G.; Santa Ana, C.A.; Bern, M.J.; Norgaard, R.P.; Bo-Linn, G.W.; Fordtran, J.S.

    1987-01-01

    We studied radiolabeled fecal bile acid excretion in 11 normal subjects and 17 patients with idiopathic chronic diarrhea for three major purposes: to establish normal values for this test in the presence of increased stool volumes (induced in normal subjects by ingestion of poorly absorbable solutions); to test for bile acid malabsorption in the patients and to correlate this with an independent test of ileal function, the Schilling test; and to compare the results of the bile acid excretion test with the subsequent effect of a bile acid binding agent (cholestyramine) on stool weight. In normal subjects fecal excretion of the radiolabel was increased with increasing stool volumes. As a group, patients with idiopathic chronic diarrhea excreted radiolabeled bile acid more rapidly than normal subjects with induced diarrhea (t1/2 56 +/- 8 vs. 236 +/- 60 h, respectively, p less than 0.005). There was a statistically significant positive correlation between t1/2 of radiolabeled bile acid and Schilling test results in these patients. Although 14 of 17 patients absorbed labeled taurocholic acid less well than any of the normal subjects with comparable volumes of induced diarrhea, cholestyramine had no statistically significant effect on stool weight in the patient group, and in none of the patients was stool weight reduced to within the normal range. In summary, most patients with idiopathic chronic diarrhea have bile acid malabsorption (as measured by fecal excretion of labeled bile acid), but they do not respond to cholestyramine therapy with a significant reduction in stool weight. Although the significance of these findings was not clearly established, the most likely interpretation is that bile acid malabsorption is a manifestation of an underlying intestinal motility or absorptive defect rather than the primary cause of diarrhea.

  16. Default mode network connectivity in patients with idiopathic normal pressure hydrocephalus.

    PubMed

    Khoo, Hui Ming; Kishima, Haruhiko; Tani, Naoki; Oshino, Satoru; Maruo, Tomoyuki; Hosomi, Koichi; Yanagisawa, Takufumi; Kazui, Hiroaki; Watanabe, Yoshiyuki; Shimokawa, Toshio; Aso, Toshihiko; Kawaguchi, Atsushi; Yamashita, Fumio; Saitoh, Youichi; Yoshimine, Toshiki

    2016-02-01

    OBJECT Idiopathic normal pressure hydrocephalus (iNPH) is a neurological disorder characterized by gait disturbance, cognitive impairment, and incontinence. It is unclear whether the pathophysiology of iNPH is associated with alterations in the default mode network (DMN). The authors investigated alterations in the DMN of patients with iNPH and sought to determine whether a relationship exists between the resting-state functional connectivity of the DMN and a patient's clinical symptoms. METHODS Resting-state functional MRI (rs-fMRI) was performed in 16 preoperative patients with iNPH and 15 neurologically healthy control subjects of a similar age. Independent component and dual-regression analyses were used to quantify DMN connectivity. The patients' clinical symptoms were rated according to the iNPH grading scale (iNPHGS). Each of their specific clinical symptoms were rated according to the cognitive, gait, and urinary continence domains of iNPHGS, and neurocognitive status was assessed using the Mini-Mental State Examination, Frontal Assessment Battery (FAB), and Trail Making Test Part A. The strength of DMN connectivity was compared between patients and controls, and the correlation between DMN connectivity and iNPHGS was examined using both region of interest (ROI)-based analysis and voxel-based analysis. The correlation between DMN connectivity and each of the specific clinical symptoms, as well as neurocognitive status, was examined using voxel-based analysis. RESULTS Both ROI-based and voxel-based analyses revealed reduced DMN connectivity in patients with iNPH. ROI-based analysis showed increased DMN connectivity with worsening clinical symptoms of iNPH. Consistently, voxel-based analyses revealed that DMN connectivity correlated positively with the iNPHGS score, as well as the cognitive and urinary continence domain scores, and negatively with the FAB score. The significant peak in correlation in each case was localized to the precuneus. CONCLUSIONS This

  17. "Brace Technology" Thematic Series - The ScoliOlogiC® Chêneau light™ brace in the treatment of scoliosis

    PubMed Central

    2010-01-01

    Background Bracing concepts in use today for the treatment of scoliosis include symmetric and asymmetric hard braces usually made of polyethylene (PE) and soft braces. The plaster cast method worldwide seems to be the most practiced technique for the construction of hard braces at the moment. CAD (Computer Aided Design) systems are available which allow brace adjustments without plaster. Another possibility is the use of the ScoliOlogiC™ off the shelf system enabling the Certified Prosthetist and Orthotist (CPO) to construct a light brace for scoliosis correction from a variety of pattern specific shells to be connected to an anterior and a posterior upright. This Chêneau light™ brace, developed according to the Chêneau principles, promises a reduced impediment of quality of life in the brace. The correction effects of the first 81 patients (main diagnosis Adolescent Idiopathic Scoliosis (AIS) [n = 64] or Early Onset Scoliosis (EOS) [n = 15]), treated according to the principles of the Chêneau light™ brace have shown a satisfactory in-brace correction exceeding 50% of the initial Cobb angle. Brace description The ScoliOlogiC® off the shelf bracing system enables the CPO to construct a light brace for scoliosis correction from a variety of pattern specific shells to be connected to an anterior and a posterior upright. This brace, when finally adjusted is called Chêneau light™ brace. The advantage of this new bracing system is that the brace is available immediately, is easily adjustable and that it can also be easily modified. This avoids construction periods of sometimes more than 6 weeks, where the curve may drastically increase during periods of fast growth. The disadvantage of this bracing system is that there is a wide variability of possibilities to arrange the different shells during adjustment. Results The Cobb angle in the whole group was reduced by an average of 16,4°, which corresponds to a correction effect of 51%. The differences were

  18. Treatment of patients with juvenile idiopathic arthritis (JIA) in a population-based cohort.

    PubMed

    Zamora-Legoff, Jorge A; Krause, Megan L; Crowson, Cynthia S; Muskardin, Theresa Wampler; Mason, Thomas; Matteson, Eric L

    2016-06-01

    A population-based cohort was utilized to evaluate medications and intra-articular injection utilization for patients with juvenile idiopathic arthritis (JIA) to inform clinical practice and further research. In a geographically defined population, all incident cases of JIA cases were identified between January 1, 1994 and December 31, 2013 based first on diagnosis code followed by medical chart confirmation. Medications and intra-articular glucocorticoid injections were abstracted. Predictors of the first disease-modifying antirheumatic drug (DMARD)/biologic and injections were reported as a hazard ratio (HR) with 95 % confidence intervals (CIs) adjusted for age and sex. Kaplan-Meier methods evaluated therapy at 6 months and 1 year. Injections were reported per 100 person-years (py) with 95 % CI using the Poisson methods. Seventy-one incident cases were identified. Forty-two (59 %) were female with mean age (standard deviation) at diagnosis of 8.2 (5.3) years. Twenty-six (37 %) utilized at least one DMARD or biologic, in which 77 % of these were prescribed in the first 6 months. Subtype of JIA was significantly associated with DMARDs/biologics (p < 0.001). Intra-articular injections were performed in 48 %. The rate of intra-articular injections was 20.7 per 100 py (95 % CI 16.5, 25.6). The rate of joint injections was higher in the first year after diagnosis (p < 0.001) and more common in recent years (p < 0.001). The majority of patients with JIA in a modern population-based cohort do not require DMARDs or biologics. In those who do, the majority receives these within the first 6 months. Intra-articular injections were utilized in almost half of patients with JIA and were increasingly used. PMID:26825065

  19. Prevalence of HLA-B27 antigen in patients with juvenile idiopathic arthritis

    PubMed Central

    Turowska-Heydel, Dorota; Sobczyk, Małgorzata; Chudek, Jerzy

    2015-01-01

    Introduction Human leukocyte antigen B27 (HLA-B27) is considered as a risk factor for development of juvenile idiopathic arthritis (JIA). The aim of this study was to analyse the prevalence of HLA-B27 antigen in JIA categories and its influence on disease onset and response to conventional therapy. Material and methods The retrospective analysis included 461 unselected children with JIA hospitalized in a single reference rheumatology centre between July 2007 and June 2012. The diagnosis was based on criteria by the International League of Association for Rheumatology. HLA-B27 was determined in 387 of all patients (84%) by hybridization of the amplified, labelled product to immobilize it on the microarray probe. Results HLA-B27 antigen was found in 104 of 383 affected children (27.2%), 48 of 206 girls (23.3%), and 56 of 177 boys (31.6%) – most frequently in patients with enthesitis-related arthritis (71%), psoriatic arthritis (50%) and unclassified cases (86.7%). The age of JIA onset was slightly (by 1 year) but significantly different in patients with and without HLA-B27 antigen [11 (8.5–14) vs. 10 (5–13.5) years.; p < 0.001]. The use of disease-modifying antirheumatic drugs (DMARDs) and corticosteroids was more frequently clinically ineffective in HLA-B27 positive than negative patients (23.1% vs. 15.2%; p = 0.09). Patients with polyarthritis, systemic, and psoriatic arthritis more frequently received biological therapy. HLA-B27 positive patients with enthesitis-related arthritis received biological therapy more frequently than HLA-B27 negative ones (20.4% vs. 0, respectively; p = 0.09). Conclusions HLA-B27 antigen is a strong risk factor for the development of enthesitis-related arthritis, and to a lesser extent for psoriatic arthritis and extended course of oligoarthritis. The presence of this antigen does not affect the disease onset but seems to predict resistance to therapy with disease-modifying drugs and corticosteroids.

  20. Association Study for 26 Candidate Loci in Idiopathic Pulmonary Fibrosis Patients from Four European Populations

    PubMed Central

    Kishore, Amit; Žižková, Veronika; Kocourková, Lenka; Petrkova, Jana; Bouros, Evangelos; Nunes, Hilario; Loštáková, Vladimíra; Müller-Quernheim, Joachim; Zissel, Gernot; Kolek, Vitezslav; Bouros, Demosthenes; Valeyre, Dominique; Petrek, Martin

    2016-01-01

    Idiopathic pulmonary fibrosis (IPF) affects lung parenchyma with progressing fibrosis. In this study, we aimed to replicate MUC5B rs35705950 variants and determine new plausible candidate variants for IPF among four different European populations. We genotyped 26 IPF candidate loci in 165 IPF patients from four European countries, such as Czech Republic (n = 41), Germany (n = 33), Greece (n = 40), France (n = 51), and performed association study comparing observed variant distribution with that obtained in a genetically similar Czech healthy control population (n = 96) described in our earlier data report. A highly significant association for a promoter variant (rs35705950) of mucin encoding MUC5B gene was observed in all IPF populations, individually and combined [odds ratio (95% confidence interval); p-value as 5.23 (8.94–3.06); 1.80 × 10−11]. Another non-coding variant, rs7934606 in MUC2 was significant among German patients [2.85 (5.05–1.60); 4.03 × 10−4] and combined European IPF cases [2.18 (3.16–1.50); 3.73 × 10−5]. The network analysis for these variants indicated gene–gene and gene–phenotype interactions in IPF and lung biology. With replication of MUC5B rs35705950 previously reported in U.S. populations of European descent and indicating other plausible polymorphic variants relevant for IPF, we provide additional reference information for future extended functional and population studies aimed, ideally with inclusion of clinical parameters, at identification of IPF genetic markers. PMID:27462317

  1. Rotenone Susceptibility Phenotype in Olfactory Derived Patient Cells as a Model of Idiopathic Parkinson’s Disease

    PubMed Central

    Matigian, N.; Todorovic, M.; Cook, A. L.; Ravishankar, S.; Dong, L. F.; Neuzil, J.; Silburn, P.; Mackay-Sim, A.; Mellick, G. D.; Wood, S. A.

    2016-01-01

    Parkinson’s disease is a complex age-related neurodegenerative disorder. Approximately 90% of Parkinson’s disease cases are idiopathic, of unknown origin. The aetiology of Parkinson’s disease is not fully understood but increasing evidence implies a failure in fundamental cellular processes including mitochondrial dysfunction and increased oxidative stress. To dissect the cellular events underlying idiopathic Parkinson’s disease, we use primary cell lines established from the olfactory mucosa of Parkinson’s disease patients. Previous metabolic and transcriptomic analyses identified deficiencies in stress response pathways in patient-derived cell lines. The aim of this study was to investigate whether these deficiencies manifested as increased susceptibility, as measured by cell viability, to a range of extrinsic stressors. We identified that patient-derived cells are more sensitive to mitochondrial complex I inhibition and hydrogen peroxide induced oxidative stress, than controls. Exposure to low levels (50 nM) of rotenone led to increased apoptosis in patient-derived cells. We identified an endogenous deficit in mitochondrial complex I in patient-derived cells, but this did not directly correlate with rotenone-sensitivity. We further characterized the sensitivity to rotenone and identified that it was partly associated with heat shock protein 27 levels. Finally, transcriptomic analysis following rotenone exposure revealed that patient-derived cells express a diminished response to rotenone-induced stress compared with cells from healthy controls. Our cellular model of idiopathic Parkinson’s disease displays a clear susceptibility phenotype to mitochondrial stress. The determination of molecular mechanisms underpinning this susceptibility may lead to the identification of biomarkers for either disease onset or progression. PMID:27123847

  2. Patient-specific finite element model of the spine and spinal cord to assess the neurological impact of scoliosis correction: preliminary application on two cases with and without intraoperative neurological complications.

    PubMed

    Henao, Juan; Aubin, Carl-Éric; Labelle, Hubert; Arnoux, Pierre-Jean

    2016-06-01

    Scoliosis is a 3D deformation of the spine and rib cage. For severe cases, surgery with spine instrumentation is required to restore a balanced spine curvature. This surgical procedure may represent a neurological risk for the patient, especially during corrective maneuvers. This study aimed to computationally simulate the surgical instrumentation maneuvers on a patient-specific biomechanical model of the spine and spinal cord to assess and predict potential damage to the spinal cord and spinal nerves. A detailed finite element model (FEM) of the spine and spinal cord of a healthy subject was used as reference geometry. The FEM was personalized to the geometry of the patient using a 3D biplanar radiographic reconstruction technique and 3D dual kriging. Step by step surgical instrumentation maneuvers were simulated in order to assess the neurological risk associated to each maneuver. The surgical simulation methodology implemented was divided into two parts. First, a global multi-body simulation was used to extract the 3D displacement of six vertebral landmarks, which were then introduced as boundary conditions into the personalized FEM in order to reproduce the surgical procedure. The results of the FEM simulation for two cases were compared to published values on spinal cord neurological functional threshold. The efficiency of the reported method was checked considering one patient with neurological complications detected during surgery and one control patient. This comparison study showed that the patient-specific hybrid model reproduced successfully the biomechanics of neurological injury during scoliosis correction maneuvers. PMID:26324393

  3. Prognostic Significance of Frontal QRS-T Angle in Patients with Idiopathic Dilated Cardiomyopathy

    PubMed Central

    Li, Sheng-Na; Zhang, Xin-Lin; Cai, Guo-Long; Lin, Ruo-Wei; Jiang, He; Chen, Jian-Zhou; Xu, Biao; Huang, Wei

    2016-01-01

    Background: Current risk stratification of idiopathic dilated cardiomyopathy (IDC) lacks sufficient sensitivity and specificity. The objective of this study was to investigate the predictive role of frontal QRS-T angles in IDC. Methods: A prospective study with 509 IDC patients was performed from February 2008 to December 2013 in the Affiliated Drum Tower Hospital, Nanjing University School of Medicine. Baseline values and changes in QRS-T angles were recorded. Follow-up was conducted every 6 months. Analyses by Cox Proportional Hazards model were performed to evaluate the association between QRS-T angle and outcomes. The primary outcome of interest was all-cause mortality. Results: During a median follow-up of 34 months, 90 of 316 patients with QRS-T angles >90° died compared to 31 of 193 patients with QRS-T angles ≤90° (hazard ratio [HR] =2.4, P < 0.001). Cardiac death was more prevalent in patients with a wide QRS-T angle (HR = 2.4, P < 0.001), similar to heart failure rehospitalization (HR = 2.5, P < 0.001). After adjustment for potential prognostic factors, the QRS-T angle was independently associated with all-cause mortality (HR = 2.5, P < 0.05), cardiac mortality (HR = 1.9, P < 0. 05), and heart failure rehospitalization (HR = 2.3, P < 0.01). Optimized therapy significantly narrowed the frontal QRS-T angle (100.9 ± 53.4° vs. 107.2 ± 54.4°, P < 0.001). The frontal QRS-T angle correlated well with established risk factors, such as left ventricular ejection fraction, brain natriuretic peptide, and New York Heart Association functional class. Conclusions: The frontal QRS-T angle is a powerful predictor of all-cause mortality, cardiac mortality, and worsening heart failure in IDC patients, independent of well-established prognostic factors. Optimized therapy significantly narrows the QRS-T angle, which might be an indicator of medication compliance, but this requires further investigation. PMID:27503013

  4. Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura

    PubMed Central

    Zheng, X. Long; Wu, Haifeng M.; Shang, Dezhi; Falls, Erica; Skipwith, Christopher G.; Cataland, Spero R.; Bennett, Charles L.; Kwaan, Hau C.

    2010-01-01

    Background Type G immunoglobulins against ADAMTS13 are the primary cause of acquired (idiopathic) thrombotic thrombocytopenic purpura. However, the domains of ADAMTS13 which the type G anti-ADAMT13 immunoglobulins target have not been investigated in a large cohort of patients with thrombotic thrombocytopenic purpura. Design and Methods Sixty-seven patients with acquired idiopathic thrombotic thrombocytopenic purpura were prospectively collected from three major U.S. centers. An enzyme-linked immunosorbent assay determined plasma concentrations of anti-ADAMTS13 type G immunoglobulins, whereas immunoprecipitation plus western blotting determined the binding domains of these type G immunoglobulins. Results Plasma anti-ADAMTS13 type G immunoglobulins from 67 patients all bound full-length ADAMTS13 and a variant truncated after the eighth TSP1 repeat (delCUB). Approximately 97% (65/67) of patients harbored type G immunoglobulins targeted against a variant truncated after the spacer domain (MDTCS). However, only 12% of patients’ samples reacted with a variant lacking the Cys-rich and spacer domains (MDT). In addition, approximately 37%, 31%, and 46% of patients’ type G immunoglobulins interacted with the ADAMTS13 fragment containing TSP1 2-8 repeats (T2-8), CUB domains, and TSP1 5-8 repeats plus CUB domains (T5-8CUB), respectively. The presence of type G immunoglobulins targeted against the T2-8 and/or CUB domains was inversely correlated with the patients’ platelet counts on admission. Conclusions This multicenter study further demonstrated that the multiple domains of ADAMTS13, particularly the Cys-rich and spacer domains, are frequently targeted by anti-ADAMTS13 type G immunoglobulins in patients with acquired (idiopathic) thrombotic thrombocytopenic purpura. Our data shed more light on the pathogenesis of acquired thrombotic thrombocytopenic purpura and provide further rationales for adjunctive immunotherapy. PMID:20378566

  5. Scoliosis and tibiotarsal deformities in broiler chickens.

    PubMed

    Droual, R; Bickford, A A; Farver, T B

    1991-01-01

    The incidence and degree of scoliosis were investigated in broiler chickens with and without intertarsal deformities associated with slipped gastrocnemius tendons. In both groups, the incidence of scoliosis was similar and there was a significant tendency for scoliosis to be convex on the right side. However, scoliosis was significantly greater in birds with intertarsal deformities, and in a significant proportion of these the joint with a slipped tendon was on the convex side of scoliosis. In birds with deformities, inequalities between right and left tibiotarsi were significantly greater, and tibiotarsi with greater length, narrower condyles and trochleae, and shallower trochlear grooves were significantly more often on the convex side of scoliosis. Significant positive correlations were found between scoliosis and rotational and bending deformities of the distal tibiotarsus on the convex side of scoliosis. These findings suggest a cause-and-effect relationship between scoliosis and tibiotarsal deformities associated with slipped tendons. PMID:2029256

  6. Blueberry Improves the Therapeutic Effect of Etanercept on Patients with Juvenile Idiopathic Arthritis: Phase III Study.

    PubMed

    Zhong, Yingjie; Wang, Ye; Guo, Jun; Chu, Haifeng; Gao, Yong; Pang, Limin

    2015-01-01

    Juvenile idiopathic arthritis (JIA) is the most common arthritis in the adolescents under the age of 16. Etanercept, an inhibitor of tumor necrosis factor, is often used to treat JIA despite its significant side effects. Homeopathic remedies, such as blueberries, have anti-inflammatory properties with fewer unwanted effects and should be considered as a primary treatment. We aimed to explore the efficacy and safety of combination therapy of blueberry and etanercept for JIA. Two hundred and one JIA patients were selected, and randomly and evenly assigned to three groups: ETA (50 mg of etanercept twice weekly), ETABJ (matched etanercept and 50 ml blueberry juice daily) and ETAPJ (matched etanercept and placebo juice). The severity of JIA was measured using American College of Rheumatology scales (ACR) 20, 50 and 70. The levels of pro-inflammatory cytokines, interleukin-1 (IL1) alpha and IL1 beta, and interleukin-1 receptor antagonist (IL1RA) were measured by qRT-PCR and ELISA. After a 6-month follow-up, the ACR20, ACR50 and ACR70 in an ETABJ group were higher than those in other two groups (P < 0.05), suggesting clinically meaningful improvement in JIA. Meanwhile, the symptoms and side effects were reduced significantly or absent in an ETABJ group, including mental diseases, retrobulbar optic neuritis, gaining weight, infection, cutaneous vasculitis, diarrhea, uveitis and pancytopenia. Blueberries reduced the levels of IL1 alpha and beta, and increased the level of IL1RA. Thus, a combination therapy of blueberry and etanercept can reduce the severity of JIA and should be developed as a new method for JIA therapy. PMID:26477692

  7. [NERVE GROWTH FACTOR IN THE URINE OF PATIENTS WITH IDIOPATHIC DETRUSOR OVERACTIVITY AND OVERACTIVE BLADDER WITHOUT DETRUSOR OVERACTIVITY].

    PubMed

    Krivoborodov, G G; Kolesanova, E F; Tur, E I; Efremov, N S

    2015-01-01

    The purpose was to determine the concentration of the neurotrophin nerve growth factor in urine to assess its possible role as a marker in the diagnosis of various forms of overactive bladder. The study included