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1.
J Orthop ; 37: 75-80, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36974094

RESUMO

Aims and objectives: The prevalence and treatment of severe scoliosis and other spinal anomalies in patients with Turner's syndrome (TS) is not well reported. This is the largest case series to date regarding the treatment course and outcomes of severely scoliotic TS patients. Methods: A retrospective chart review was performed to identify all patients with TS seen at a single center academic pediatric institution from 2007 to 2021. Of these, the presence of concomitant severe scoliosis or other spinal anomalies was determined, defined by a major coronal curve measuring 45° or greater. Demographic, clinical, surgical, and radiologic data was collected at both pre- and post-intervention time points. Results: A retrospective chart review identified 306 patients with TS. Of those, six were identified to have severe scoliosis or other severe spinal anomalies requiring fusion. All four posterior spinal fusion (PSF) patients demonstrated improvement of their spinal curvature. One patient who electively pursued only bracing demonstrated minimal improvement and surgery was subsequently recommended, but not pursued. One patient expired from a pre-existing heart condition prior to intervention. All postoperative complications resolved with no further complications. The only brace-related complication was an allergic rash related to the brace material. Conclusion: All four patients who underwent PSF demonstrated significant improvement of their spinal curvature with few post-surgical complications. None of the patients in the bracing cohort demonstrated stabilization of their spinal curvature. Therefore, these data corroborate with prior studies, suggesting that operative management consisting of spinal fusion with instrumentation provides optimal clinical outcomes, compared to bracing only.

2.
J Pediatr Orthop ; 43(5): 299-302, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36791404

RESUMO

BACKGROUND: The prevalence of major coronal and sagittal spinal curves (scoliosis and kyphosis) in Turner syndrome (TS) is not well established due to limited reporting. The relationship between growth hormone (GH) therapy and its effect on TS spinal curve incidence is also not well established. METHODS: A retrospective chart review of 306 TS patients from 2007 to 2021 evaluated major coronal and sagittal spinal curves, progression of the curve, and treatment with GH. Statistical significance (defined as P <0.05) between curvature rates and curve progression was compared between GH-treated patients and non-GH-treated patients using a χ 2 or Fisher exact test when appropriate. RESULTS: Thirty-seven of 306 (12%) TS patients had a radiographically relevant spinal deformity. Twenty-seven of 37 (73%) had mild; 4 of 37 (11%) had moderate, and 6 of 37 (16%) had severe curves. Of those with severe, 4 underwent spinal fusion, 1 was treated with bracing, and 1 was braced before a cardiovascular-related death. Regarding GH use among TS patients, 190 of 306 (62%) used GH versus 116 of 306 (38%) who did not. Of those with a spinal curve, 24 of 37 (65%) used GH compared with 13 of 37 (35%) who did not. On univariate analysis, GH therapy was not a risk factor for the diagnosis of a major spinal curve, a more severe degree of the curve at the time of diagnosis, or spinal curve progression ( P >0.05 for all). CONCLUSIONS: This is the largest single institution retrospective review of a TS cohort known to the authors assessing spinal curve prevalence and relation to GH treatment and demonstrates a TS spinal curve rate of 12% (37/306). Four of six (11%) TS patients with a severe curve underwent corrective spine fusion. There was no relationship between the use of GH and the presence of a spinal curve or curve progression. Further study is warranted to determine risk factors for curve progression. LEVEL OF EVIDENCE: Level III. CLINICAL RELEVANCE: This retrospective case series serves to review and address the prevalence of spinal deformity in TS patients and whether GH impacts worsening deformity.


Assuntos
Cifose , Escoliose , Fusão Vertebral , Síndrome de Turner , Humanos , Criança , Escoliose/epidemiologia , Escoliose/etiologia , Escoliose/terapia , Estudos Retrospectivos , Prevalência , Atenção Terciária à Saúde , Síndrome de Turner/complicações , Síndrome de Turner/epidemiologia , Cifose/epidemiologia , Cifose/etiologia , Fusão Vertebral/efeitos adversos , Resultado do Tratamento
3.
J Clin Anesth ; 75: 110493, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34482261

RESUMO

STUDY OBJECTIVE: This study assessed whether implementation of an enhanced recovery-based pathway decreased length of stay without increasing readmissions among patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion. DESIGN: Retrospective observational before-and-after study. SETTING: A tertiary children's hospital. PATIENTS: A total of 117 patients were studied, 78 in the pre-intervention group and 39 in the post-intervention group. All patients underwent posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) in the same institution with one of two spine surgeons. Age, sex, American Society of Anesthesiologists physical status, and Cobb angle were comparable between the two groups. INTERVENTIONS: Between the pre- and post-intervention groups an enhanced recovery protocol was developed. The pathway included standardized use of nonopioid analgesics, proactive transition to oral analgesics, scheduled antiemetics, plans for diet advancement, and specific physical therapy goals. MEASUREMENTS: Outcome measurements included hospital length of stay, cumulative opioid doses in the first two postoperative days, and time to discontinuation of urinary catheter and patient-controlled analgesia. Postoperative emergency department visits, hospital readmissions and chronic pain management referrals were also measured. Pain scores on postoperative days one through four were recorded. MAIN RESULTS: Hospital length of stay decreased from 4.6 days to 3.8 days. Patient-controlled analgesia (PCA) was discontinued one day earlier on average following pathway implementation. Average cumulative postoperative opioid use, in morphine equivalents, decreased in the first two postoperative days from 2.5 to 2.2 mg/kg. There was no change in hospital readmission rate or postoperative chronic pain referral. CONCLUSIONS: Patients undergoing PSF for AIS experienced shorter hospital stays without increased readmissions following the implementation of an enhanced recovery pathway. Development of this pathway required buy-in from multiple stakeholders and significant coordination among services. The principles used to develop this pathway may be applied in other institutions and to other patient populations using the model outlined here.


Assuntos
Escoliose , Fusão Vertebral , Adolescente , Analgesia Controlada pelo Paciente , Criança , Humanos , Tempo de Internação , Dor Pós-Operatória/tratamento farmacológico , Dor Pós-Operatória/epidemiologia , Estudos Retrospectivos , Escoliose/cirurgia , Fusão Vertebral/efeitos adversos
4.
J Orthop ; 26: 126-129, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34404968

RESUMO

Idiopathic Scoliosis (IS) is a relatively common condition and is estimated to affect as many as 3 % of youth aged 10-17 years (in the United States an estimated approximately 1.4 million otherwise healthy individuals). A clear understanding of the etiology will better direct optimization of evaluation, treatments and therapies, especially early treatments with less invasive methods. A mechanistic explanation of factors combining to initiate and then cause progression of this common condition-- in otherwise healthy pre-teenage and teenage patients--will be discussed. A recent well-designed structured systematic review states that 'strong evidence is lacking for a consistent pattern of occurrence and any abnormality', in other words there is no strong evidence for 'other associated diagnoses' in IS. And so, certain important inherent factors of IS merit greater discussion. Inherent, or intrinsic factors include: a natural susceptibility to develop a lateral and rotational deformity in the immature rapidly growing erect human spine, inherent torsion associated at the induction of deformity, biomechanics related to curve progression, and anthropology/bipedal gait. We know more today about factors related to the condition and its etiology than we have previously. Across multiple disciplines, a mechanistic approach to understanding the etiopathogenesis of IS, allows a reasonable 'theory' for IS etiology and its progression. We will discuss these inherent intrinsic factors in order to further add to our understanding of the theoretical etiopathogenesis. A better understanding of the etiology (and progression) may better direct ways to optimize evaluation, treatments and therapies, especially early treatments with less invasive methods.

5.
J Orthop ; 21: 421-426, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32943828

RESUMO

OBJECTIVE: IS (idiopathic scoliosis) is a common spinal condition occurring in otherwise completely healthy adolescents. The root cause of IS remains unclear. This systematic review will focus on an update of genetic factors and IS etiology. Though it is generally accepted that the condition is not due to a single gene effect, etiology studies continue looking for a root cause including genetic variants. Though susceptibility from multiple genetic components is plausible based on known family history data, the literature remains unclear regarding multifactorial genetic influences. The objective of this study was to critically evaluate the evidence behind genetic causes (not single gene) of IS through a systematic review and strength-of-study analysis of existing genetic and genome-wide association studies (GWAS). We used the protocol of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). METHODS: PubMed was searched for the terms IS, scoliotic, spinal curve, genetic, gene, etiology, polymorphisms. Articles were assessed for risk-of-bias. Level-of-evidence grading was completed via Oxford Centre for Evidence-Based Medicine criteria. The assessment scores factor strength of a study in determining a positive or negative association to a gene etiology. RESULTS: After screening of 36 eligible papers, 8 relevant studies met inclusion criteria at this time, 3 were in favor of a genetic factor for IS, whereas 5 studies were against it. CONCLUSION: Based on the literature analyzed, there is moderate evidence with a low risk-of-bias that does not clarify a genetic cause of IS. The 2 studies in favor of a genetic etiology were completed in homogeneous populations, limiting their generalizability. Relying on a genetic etiology alone for IS may over simplify its multifactorial nature and limit appreciation of other influences.

6.
Muscle Nerve ; 61(6): 740-744, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32108365

RESUMO

Limb contractures are debilitating complications associated with various muscle and nervous system disorders. This report summarizes presentations at a conference at the Shirley Ryan AbilityLab in Chicago, Illinois, on April 19-20, 2018, involving researchers and physicians from diverse disciplines who convened to discuss current clinical and preclinical understanding of contractures in Duchenne muscular dystrophy, stroke, cerebral palsy, and other conditions. Presenters described changes in muscle architecture, activation, extracellular matrix, satellite cells, and muscle fiber sarcomeric structure that accompany or predispose muscles to contracture. Participants identified ongoing and future research directions that may lead to understanding of the intersecting factors that trigger contractures. These include additional studies of changes in muscle, tendon, joint, and neuronal tissues during contracture development with imaging, molecular, and physiologic approaches. Participants identified the requirement for improved biomarkers and outcome measures to identify patients likely to develop contractures and to accurately measure efficacy of treatments currently available and under development.


Assuntos
Contratura/fisiopatologia , Educação/tendências , Doenças Musculoesqueléticas/fisiopatologia , Doenças do Sistema Nervoso/fisiopatologia , Relatório de Pesquisa/tendências , Paralisia Cerebral/diagnóstico , Paralisia Cerebral/fisiopatologia , Paralisia Cerebral/terapia , Chicago , Contratura/diagnóstico , Contratura/terapia , Humanos , Distrofia Muscular de Duchenne/diagnóstico , Distrofia Muscular de Duchenne/fisiopatologia , Distrofia Muscular de Duchenne/terapia , Doenças Musculoesqueléticas/diagnóstico , Doenças Musculoesqueléticas/terapia , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/terapia
7.
J Orthop ; 19: 174-177, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32025128

RESUMO

Adolescent idiopathic scoliosis (AIS) a common spinal condition affecting adolescents. Though the etiology is still unknown, it is widely thought to have a multifactorial etiology and early diagnosis remains a significant challenge. The purpose of this study is to identify early vertebral morphological changes and patterns of spinal asymmetry in these at-risk individuals who later progress to adolescent idiopathic scoliosis. This was a retrospective study of patients treated for AIS between 1997 and 2017. We utilized two study groups, a group with immature onset of spinal asymmetry and a control group. Inclusion criteria for the immature onset group was defined by a Cobb angle between 10 and 40° diagnosed prior to the age of 12 with MRI scans and XRs available for review. Qualitative assessments observed for sagittal vertebral wedging, analysis of vertebral corner anatomy, spinal harmony, and sagittal balance. These findings were then qualitatively compared between groups. Twenty patients were included in this study, ten each in the immature onset and control groups. In the immature onset group, two patients had sagittal wedging, five had abnormal vertebral corners, nine did not have spinal harmony, and nine had negative sagittal balance, compared to none of the control patients having sagittal wedging, none having abnormal vertebral corners, all having spinal harmony, and nine having positive spinal balance. This pilot MRI study identifies qualitative vertebral morphological changes in patients who progress to AIS. Our findings suggest abnormal vertebral corner anatomy, sagittal wedging, and negative sagittal balance as potential early findings in patients who develop AIS.

8.
Anesth Analg ; 129(1): 184-191, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31210654

RESUMO

BACKGROUND: Preoperative pulmonary function tests are routinely obtained in children with scoliosis undergoing posterior spinal fusion despite unclear benefits as a perioperative risk assessment tool and frequent inability of patients to provide acceptable results. The goal of this study was to determine whether preoperative pulmonary function test results are associated with the need for postoperative intubation or intensive care unit admission after posterior spinal fusion. METHODS: The electronic medical records of patients who underwent posterior spinal fusion at a pediatric tertiary hospital between June 2012 and August 2017 were reviewed. Pulmonary function tests were consistently ordered for all patients, unless the patient was deemed unable to perform the test due to cognitive disability. Cases were categorized as primary or secondary scoliosis.Demographic data, preoperative bilevel positive airway pressure use, Cobb angle, intraoperative allogeneic blood transfusion, and ability to produce acceptable pulmonary function test results were collected for each patient. In patients with satisfactory pulmonary function test results, forced vital capacity and maximum inspiratory pressure were collected. Primary outcomes for analysis were postoperative intubation and intensive care unit admission. Univariable logistic regression models were used to assess the association between each variable of interest and the primary outcomes. RESULTS: The study sample included 433 patients, 288 with primary scoliosis and 145 with secondary scoliosis. Among patients with primary scoliosis, 90% were able to produce acceptable pulmonary function test results, zero remained intubated postoperatively, and 6 were admitted to the intensive care unit. Among patients with secondary scoliosis, 44% could not attempt pulmonary function tests. Among those who did attempt the test, 30% were unable to produce meaningful results. Forced vital capacity and maximum inspiratory pressure were not found to be associated with postoperative intubation or intensive care unit admission. Weight, Cobb angle, intraoperative blood transfusion, American Society of Anesthesiologists physical status classification, and preoperative bilevel positive airway pressure use were associated with patient outcomes. Among 357 total patients who attempted pulmonary function tests, 37 had high-risk results. Only 1 of these 37 patients remained intubated postoperatively. CONCLUSIONS: Patients undergoing posterior spinal fusion, especially those with secondary scoliosis, are frequently unable to adequately perform pulmonary function tests. Among patients with interpretable pulmonary function tests, there was no association between results and postoperative intubation or intensive care unit admission. Routine pulmonary function testing for all patients with scoliosis may not be indicated for purposes of risk assessment before posterior spinal fusion. Clinicians should consider a targeted approach and limit pulmonary function tests to patients for whom results may guide preoperative optimization as this may improve outcomes and reduce inefficiencies and costs.


Assuntos
Intubação Intratraqueal , Pulmão/fisiopatologia , Testes de Função Respiratória , Escoliose/cirurgia , Fusão Vertebral/efeitos adversos , Adolescente , Fatores Etários , Extubação , Criança , Cuidados Críticos , Registros Eletrônicos de Saúde , Feminino , Humanos , Masculino , Pressões Respiratórias Máximas , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/terapia , Valor Preditivo dos Testes , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Escoliose/diagnóstico por imagem , Escoliose/fisiopatologia , Resultado do Tratamento , Capacidade Vital
10.
J Orthop ; 16(1): 11-13, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30733624

RESUMO

PURPOSE: The overall benefits of ACL reconstructive surgery in young athletes has been previously established. Graft selection for ACL reconstruction, specifically in this population however, remains controversial. The literature is limited and long-term survival rate of allograft ACL reconstruction in the adolescent population remains poorly defined. Current evidence, none level I, appears to demonstrate increased failure and subsequent revision rates in allograft reconstruction of complete ACL tears compared to autograft; 7-35% compared to 3-13% respectively. The purpose of the present study was to evaluate revision rate and functional outcomes of allograft ACL reconstruction in the adolescent population at extended follow-up. METHODS: A retrospective chart review was performed. Forty patients who underwent transphyseal ACL reconstruction with either bone patellar tendon bone (BTB) or Achilles tendon bone (ATB) allograft performed by a single surgeon over a 12-year period were identified. Demographic and surgical details were analyzed. Enrolled patients completed a Lysholm Knee Scoring Scale and a Tegner Activity Level Scale during phone interviews. All secondary surgeries performed on the ipsilateral knee were recorded. RESULTS: Twenty-five patients were enrolled; fifteen were lost to follow-up. There were ten male and fifteen female patients included for analysis. Average age at index surgery was 16 years (range 13-18 years). BTB allograft was used for seven patients, and ATB allograft was used for the remaining eighteen patients. Average follow-up was 54 months (range 13-136 months). The average Lysholm score at follow-up was 87 (range 57-100). The average Tegner score at follow-up was 6.8 (range 3-10). Three patients underwent revision ACL surgery (12% study group, 7.5% all) for traumatic re-rupture. Re-rupture occurred 12, 13 and 38 months after index surgery. CONCLUSIONS: Autograft remains the standard for ACL reconstruction in the general pediatric population. In the adolescent population, however, the use of BTB or ATB allograft is a reasonable alternative with satisfactory outcomes, decreased harvest site morbidity, decreased post-operative pain and faster rehabilitation. The traumatic re-rupture rate in this series was similar to previously published traumatic failure rates in young adult athletes after reconstruction with autologous tissue (11-13%). Further prospective studies are needed to determine any true difference in the use of either allograft or autograft in the adolescent population.

11.
J Orthop ; 15(4): 971-973, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30224852

RESUMO

BACKGROUND: Pediatric femur fractures are frequently encountered injuries frequently treated with spica casting. Spica casting may, however, be expensive and burdensome to patients. A possible alternative is a long leg splint. METHODS: Patients aged 6 months to 5 years old who were treated for a femoral shaft fracture with a long leg splint extending above the waist were matched with a patient treated with a spica cast. RESULTS: At the time of healing, the alignment in the spica cast group was only significantly better than the alignment of the splint group with respect to coronal angulation.

12.
J Orthop ; 15(2): 563-565, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29881194

RESUMO

PURPOSE: The evaluation, management and follow-up of adolescent idiopathic scoliosis (AIS) occur frequently within a pediatric orthopedic surgery practice. Curve status can be assessed with Scoliometer measurements of angle trunk rotation (ATR), which are reliable and reproducible to within 3°. This study assessed the longitudinal efficacy, safety and cost savings of integrating ATR measurements to monitor curve status and progression in AIS, and suggests a quality-based management strategy. METHODS: A retrospective review of medical records between 2004 and 2014 included patients with AIS between 10-17 years, excluding those with Cobb angle >52° at presentation. Two cohorts were analyzed based on presentation prior to menarche (PRE) or after menarche (POST). The PRE groups was further classified based on whether the curve was Stable or Unstable. The cost of a single PA thoracolumbar radiograph was defined based on the 2015 CMS fee schedule ($36.27). Safety was defined based on the effective radiation dose avoided (0.14 millisieverts/radiograph). RESULTS: A total of 59 children were included with 45 in PRE and 14 in the POST cohort. The use of ATR measurements provided a cost benefit in both the PRE Stable and Unstable cohorts, by avoiding radiographs with an average savings of $161.76 and $147.50 respectively. Similarly in POST, there was an average cost savings of $105.18 per patient. The safety benefit of using ATR measurements included avoiding an average of 0.62, 0.56 and 0.4 millisieverts of radiation in the PRE Stable, PRE Unstable and POST groups respectively. CONCLUSIONS: An evaluation strategy with ATR measurements provides for a reliable, cost-effective and safety advantage in the monitoring of curve progression in both skeletally mature and immature patients with AIS. These findings suggest that stable ATR measurements are a safe and cost effective alternative to serial radiographs in the clinical monitoring of AIS. Recent evidence from 25 years of scoliosis treatment in Denmark noted a cancer rate 17 times that of an age-matched population. Thus, reducing radiation exposure during scoliosis monitoring using ATR measurements has important clinical significance for cancer risk reduction.

14.
J Bone Joint Surg Am ; 99(23): e128, 2017 Dec 06.
Artigo em Inglês | MEDLINE | ID: mdl-29206799

RESUMO

BACKGROUND: Simulation-based education has been integrated into many orthopaedic residency programs to augment traditional teaching models. Here we describe the development and implementation of a combined didactic and simulation-based course for teaching medical students and interns how to properly perform a closed reduction and percutaneous pinning of a pediatric supracondylar humeral fracture. METHODS: Subjects included in the study were either orthopaedic surgery interns or subinterns at our institution. Subjects all completed a combined didactic and simulation-based course on pediatric supracondylar humeral fractures. The first part of this course was an electronic (e)-learning module that the subjects could complete at home in approximately 40 minutes. The second part of the course was a 20-minute simulation-based skills learning session completed in the simulation center. Subject knowledge of closed reduction and percutaneous pinning of supracondylar humeral fractures was tested using a 30-question, multiple-choice, written test. Surgical skills were tested in the operating room or in a simulated operating room. Subject pre-intervention and post-intervention scores were compared to determine if and how much they had improved. RESULTS: A total of 21 subjects were tested. These subjects significantly improved their scores on both the written, multiple-choice test and skills test after completing the combined didactic and simulation module. Prior to the module, intern and subintern multiple-choice test scores were significantly worse than postgraduate year (PGY)-2 to PGY-5 resident scores (p < 0.01); after completion of the module, there was no significant difference in the multiple-choice test scores. After completing the module, there was no significant difference in skills test scores between interns and PGY-2 to PGY-5 residents. Both tests were validated using the scores obtained from PGY-2 to PGY-5 residents. CONCLUSIONS: Our combined didactic and simulation course significantly improved intern and subintern understanding of supracondylar humeral fractures and their ability to perform a closed reduction and percutaneous pinning of these fractures.


Assuntos
Pinos Ortopédicos , Fixação Interna de Fraturas/educação , Fraturas do Úmero/cirurgia , Ortopedia/educação , Treinamento por Simulação , Adulto , Competência Clínica , Currículo , Educação de Pós-Graduação em Medicina , Educação de Graduação em Medicina , Avaliação Educacional , Feminino , Humanos , Masculino
15.
J Rheumatol ; 43(1): 161-8, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26628598

RESUMO

OBJECTIVE: To identify differentially expressed microRNA (miRNA) in muscle biopsies (MBx) from 15 untreated children with juvenile dermatomyositis (JDM) compared with 5 controls. METHODS: Following MBx miRNA profiling, differentially expressed miRNA and their protein targets were validated by quantitative real-time PCR (qRT-PCR) and immunological assay. The association of miRNA-10a and miRNA-10b with clinical data was evaluated, including Disease Activity Score (DAS), von Willebrand factor antigen (vWF:Ag), nailfold capillary end row loops, duration of untreated disease, and tumor necrosis factor (TNF)-α-308A allele. RESULTS: In JDM, 16/362 miRNA were significantly differentially expressed [false discovery rate (FDR) < 0.05]. Among these, miRNA-10a was the most downregulated miRNA in both FDR and ranking of fold change: miRNA-10a = -2.27-fold, miRNA-10b = -1.80-fold. Decreased miRNA-10a and miRNA-10b expressions were confirmed using q RT-PCR: -4.16 and -2.59 fold, respectively. The qRT-PCR documented that decreased miRNA-10a expression was related to increased vascular cell adhesion molecule 1 in 13 of these JDM cases (correlation -0.67, p = 0.012), unlike miRNA-10b data (not significant). Concurrent JDM plasma contained increased levels of interleukin (IL) 6 (p = 0.0363), IL-8 (p = 0.0005), TNF-α (p = 0.0011), and monocyte chemoattractant proteins 1 (p = 0.0139). Decreased miRNA-10a, but not miRNA-10b, was associated with the TNF-α-308A allele (p = 0.015). In the 15 JDM, a trend of association of miRNA-10a (but not miRNA-10b) with vWF:Ag and DAS was observed. CONCLUSION: MiRNA-10a downregulation is an important element in untreated JDM muscle pathophysiology. We speculate that muscle miRNA expression in adult dermatomyositis differs from muscle miRNA expression in untreated childhood JDM.


Assuntos
Dermatomiosite/genética , Dermatomiosite/patologia , Regulação da Expressão Gênica/imunologia , Mediadores da Inflamação/metabolismo , MicroRNAs/genética , Biópsia por Agulha , Estudos de Casos e Controles , Criança , Pré-Escolar , Citocinas/metabolismo , Dermatomiosite/terapia , Regulação para Baixo , Feminino , Perfilação da Expressão Gênica , Humanos , Biópsia Guiada por Imagem , Imuno-Histoquímica , Imageamento por Ressonância Magnética/métodos , Masculino , Reação em Cadeia da Polimerase em Tempo Real/métodos , Valores de Referência , Estudos de Amostragem , Índice de Gravidade de Doença
17.
Am J Orthop (Belle Mead NJ) ; 43(9): E200-5, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25251533

RESUMO

There are many surgical techniques for treating the chronically dislocated, painful hip in patients with neuromuscular spasticity, but each has significant complication rates. We conducted a study to examine the outcomes of a novel technique, an extreme varus femoral shortening osteotomy, used in nonambulatory patients with neuromuscular spasticity. Patients who underwent the procedure were identified retrospectively by surgical codes. Medical records were reviewed for range of motion, pain and functional assessment, surgical indications, complications, and results. In addition, preoperative and postoperative radiographs were assessed, and caretaker questionnaires reviewed. Between 2001 and 2010, 1 surgeon performed 6 femoral shortening osteotomies in 5 nonambulatory patients with neuromuscular spasticity. In all 5 cases, there were improvements in pain, sitting tolerance, ease of hygiene, and ease of transfers at a minimum follow-up of 2 years (mean, 3.4 years). Postoperative complications included asymptomatic heterotopic ossification and recurrent subluxation. Extreme femoral shortening is a reproducible surgical technique that alleviates pain and makes hygiene easier in nonambulatory children with symptomatic hip dislocations caused by neuromuscular spasticity. Our complication rate was comparable to that of other procedures.


Assuntos
Luxação do Quadril/cirurgia , Articulação do Quadril/cirurgia , Espasticidade Muscular/cirurgia , Osteotomia/métodos , Adolescente , Criança , Doença Crônica , Feminino , Seguimentos , Luxação do Quadril/complicações , Humanos , Masculino , Espasticidade Muscular/complicações , Amplitude de Movimento Articular , Resultado do Tratamento
18.
J Bone Joint Surg Am ; 95(17): e1261-7, 2013 Sep 04.
Artigo em Inglês | MEDLINE | ID: mdl-24005210

RESUMO

BACKGROUND: There is a need to provide more efficient surgical training methods for orthopaedic residents. E-learning could possibly increase resident surgical preparedness, confidence, and comfort for surgery. Using closed reduction and pinning of pediatric supracondylar humeral fractures as the index case, we hypothesized that e-learning could increase resident knowledge acquisition for case preparation in the operating room. METHODS: An e-learning surgical training module was created on the Computer Enhanced Visual Learning platform. The module provides a detailed and focused road map of the procedure utilizing a multimedia format. A multisite prospective randomized controlled study design compared residents who used a textbook for case preparation (control group) with residents who used the same textbook plus completed the e-learning module (test group). All subjects completed a sixty-question test on the theory and methods of the case. After completion of the test, the control group then completed the module as well. All subjects were surveyed on their opinion regarding the effectiveness of the module after performing an actual surgical case. RESULTS: Twenty-eight subjects with no previous experience in this surgery were enrolled at four academic centers. Subjects were randomized into two equal groups. The test group scored significantly better (p < 0.001) and demonstrated competence on the test compared with the control group; the mean correct test score (and standard deviation) was 90.9% ± 6.8% for the test group and 73.5% ± 6.4% for the control group. All residents surveyed (n = 27) agreed that the module is a useful supplement to traditional methods for case preparation and twenty-two of twenty-seven residents agreed that it reduced their anxiety during the case and improved their attention to surgical detail. CONCLUSIONS: E-learning using the Computer Enhanced Visual Learning platform significantly improved preparedness, confidence, and comfort with percutaneous closed reduction and pinning of a pediatric supracondylar humeral fracture. We believe that adapting such methods into residency training programs will improve efficiency in surgical training.


Assuntos
Competência Clínica , Instrução por Computador/métodos , Fraturas Ósseas/cirurgia , Internato e Residência , Procedimentos Ortopédicos/educação , Humanos , Ortopedia/educação , Pediatria/educação
19.
Proc Natl Acad Sci U S A ; 110(10): 4003-8, 2013 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-23431178

RESUMO

Spina bifida (SB) patients afflicted with myelomeningocele typically possess a neurogenic urinary bladder and exhibit varying degrees of bladder dysfunction. Although surgical intervention in the form of enterocystoplasty is the current standard of care in which to remedy the neurogenic bladder, it is still a stop-gap measure and is associated with many complications due to the use of bowel as a source of replacement tissue. Contemporary bladder tissue engineering strategies lack the ability to reform bladder smooth muscle, vasculature, and promote peripheral nerve tissue growth when using autologous populations of cells. Within the context of this study, we demonstrate the role of two specific populations of bone marrow (BM) stem/progenitor cells used in combination with a synthetic elastomeric scaffold that provides a unique and alternative means to current bladder regeneration approaches. In vitro differentiation, gene expression, and proliferation are similar among donor mesenchymal stem cells (MSCs), whereas poly(1,8-octanediol-cocitrate) scaffolds seeded with SB BM MSCs perform analogously to control counterparts with regard to bladder smooth muscle wall formation in vivo. SB CD34(+) hematopoietic stem/progenitor cells cotransplanted with donor-matched MSCs cause a dramatic increase in tissue vascularization as well as an induction of peripheral nerve growth in grafted areas compared with samples not seeded with hematopoietic stem/progenitor cells. Finally, MSC/CD34(+) grafts provided the impetus for rapid urothelium regeneration. Data suggest that autologous BM stem/progenitor cells may be used as alternate, nonpathogenic cell sources for SB patient-specific bladder tissue regeneration in lieu of current enterocystoplasty procedures and have implications for other bladder regenerative therapies.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Transplante de Células-Tronco Mesenquimais , Regeneração/fisiologia , Disrafismo Espinal/fisiopatologia , Disrafismo Espinal/cirurgia , Bexiga Urinaria Neurogênica/fisiopatologia , Bexiga Urinaria Neurogênica/cirurgia , Bexiga Urinária/fisiopatologia , Bexiga Urinária/cirurgia , Adolescente , Animais , Criança , Citratos/química , Feminino , Humanos , Masculino , Neovascularização Fisiológica , Regeneração Nervosa/fisiologia , Polímeros/química , Ratos , Ratos Nus , Disrafismo Espinal/complicações , Engenharia Tecidual/métodos , Alicerces Teciduais/química , Bexiga Urinária/irrigação sanguínea , Bexiga Urinaria Neurogênica/etiologia
20.
Eur Spine J ; 21(1): 138-44, 2012 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21858726

RESUMO

PURPOSE: Fusionless growth modulation is an attractive alternative to conventional treatments of idiopathic scoliosis. To date, fusionless devices achieve unilateral growth modulation by compressing the intervertebral disc. This study explores a device to control spinal alignment and vertebral morphology via growth modulation while excluding the disc in a porcine model. METHODS: A device that locally encloses the vertebral growth plate exclusive of the disc was introduced anteriorly over T5-T8 in four immature pigs (experimental) while three underwent surgery without instrumentation (sham) and two were selected as controls. Bi-weekly coronal and lateral radiographs were taken over the 12-week follow-up to document vertebral morphology and spinal alignment modifications via an inverse approach (creation of deformity). RESULTS: All animals completed the experiment with no postoperative complications. Control and sham groups showed no significant changes in spinal alignment. Experimental group achieved a final coronal Cobb angle of 6.5° ± 3.5° (constrained to the four instrumented levels) and no alteration to the sagittal profile was observed. Solely the experimental group ended with consistent vertebral wedging of 4.1° ± 3.6° amounting to a cumulative wedging of up to 25° and a concurring difference in left/right vertebral height of 1.24 ± 1.86 mm in the coronal plane. CONCLUSIONS: The proposed intravertebral epiphyseal device, for the early treatment of progressive idiopathic scoliosis, demonstrated its feasibility by manipulating spinal alignment through the realization of local growth modulation exclusive of the intervertebral disc.


Assuntos
Epífises/cirurgia , Modelos Animais , Procedimentos Ortopédicos/instrumentação , Procedimentos Ortopédicos/métodos , Coluna Vertebral/crescimento & desenvolvimento , Coluna Vertebral/cirurgia , Animais , Epífises/anatomia & histologia , Epífises/fisiologia , Estudos de Viabilidade , Feminino , Disco Intervertebral/crescimento & desenvolvimento , Disco Intervertebral/cirurgia , Escoliose/fisiopatologia , Escoliose/cirurgia , Coluna Vertebral/anatomia & histologia , Sus scrofa
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