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BACKGROUND: Dermabond (Dermabond; Ethicon, Sommerville, NJ), is a skin adhesive commonly utilized in pediatric orthopedic surgery for postoperative wound care. Few studies have examined outcomes of Dermabond exposure in children. The purpose of this study is to estimate the incidence of skin reactions following Dermabond exposure in pediatric orthopedic surgery and investigate potential risk factors associated with Dermabond sensitivity. METHODS: This was a retrospective study of a level-one pediatric trauma center. All orthopaedic surgeries in 2019 were screened for Dermabond application. Three surgeons with the highest rates of Dermabond application defined our cohort. Out of 2990 surgeries in 2019, the 3 surgeons performed 234 surgeries with Dermabond. Postoperative reactions and repeat Dermabond exposures were collected for these 234 patients. Subjects with known allergies to Dermabond were excluded. Reactions were defined. as discoloration, irritation, and wound dehiscence. Significant differences between patients with repeat Dermabond exposures and those without were determined using χ 2 analysis. Associations between patient characteristics and sensitivity were determined using logistic regression analysis. P values less than 0.05 were considered significant. RESULTS: In all, 234 patients were included for analysis. The mean age at surgery was 12.5 years (SD 6.1), and 39% (92/234) of the cohort was male. Thirty-two patients (14%) experienced skin reactions during the study period (95% CI=7%-19%). Reactions most frequently included. erythema (10/32; 31%) and itchiness (10/32; 31%). Reactions were most frequently treated with oral antibiotics, Benadryl, or a dressing change. Of 144 patients with 1 Dermabond exposure, 17 (12%) experienced reactions (95% CI=7%-18%). Of 128 patients experiencing a repeat Dermabond exposure, 27 (21%) experienced reactions (95% CI=19%-34%, P =0.03). Age, surgical procedure, and surgical location were not, associated with a variable rate of sensitivity. CONCLUSIONS: Sensitivity to Dermabond after pediatric orthopedic surgery occurred at a higher rate than seen in adults, and patients with multiple Dermabond exposures experienced significantly higher sensitivity than patients with a single exposure. Increased awareness of this potential complication is needed to help inform decisions regarding Dermabond's application in pediatric orthopedics.
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Procedimentos Ortopédicos , Adesivos Teciduais , Adulto , Humanos , Masculino , Criança , Adesivos Teciduais/efeitos adversos , Estudos Retrospectivos , Incidência , Procedimentos Ortopédicos/efeitos adversosRESUMO
BACKGROUND: In adults, the incidence of ipsilateral femoral neck fractures in the setting of femoral shaft fractures is reported to be as high as 9%; however, scant literature exists on the same clinical scenario in pediatric/adolescent populations. Therefore, the purpose of this study was to investigate the incidence of ipsilateral femoral neck fracture in the setting of femoral shaft fractures in children and adolescents treated in pediatric hospitals across the United States. METHODS: The Pediatric Health Information System database was queried for patients aged 18 years or younger who were treated for a femoral neck, femoral shaft, and pertrochanteric femur fractures through an emergency department, inpatient, ambulatory surgery, or observation visit. Patients were identified using ICD-9 and ICD-10 diagnosis codes. Data from 49 pediatric hospitals between the years 2002 and 2020 were included. Incidence was calculated as the number of cases including the event divided by the total number of cases. RESULTS: A total of 90,146 records were identified from a cohort of 55,733,855 (0.16%). Distal femur fractures, pathologic fractures, and periprosthetic fractures were excluded, resulting in 65,651 unique cases. Of the 65,651 cases, 7104 (11%) were identified as isolated neck fractures. The combined incidence of femoral neck or pertrochanteric femur fractures in the setting of a femoral shaft fracture was 82.3 per 10,000 cases (0.82%). Only 283 cases of concomitant femoral neck and shaft fractures were found among 55,169 femoral shaft fractures (0.5%). CONCLUSIONS: The incidence of ipsilateral femoral neck or pertrochanteric femur fractures in the setting of a femoral shaft fracture is 82.3 per 10,000 patients (0.82%) based on data from Pediatric Health Information System-participating institutions. The incidence of femoral neck/pertrochanteric femur fractures and femoral shaft fractures in children and adolescents is more than 10 times lower than reported for adults; therefore, the routine use of advanced diagnostic imaging in pediatric patients with femoral shaft fractures should be considered cautiously. LEVEL OF EVIDENCE: Level IV; cross-sectional analysis.
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Fraturas do Fêmur , Fraturas do Colo Femoral , Adulto , Adolescente , Humanos , Criança , Colo do Fêmur/diagnóstico por imagem , Incidência , Estudos Transversais , Fraturas do Fêmur/diagnóstico por imagem , Fraturas do Fêmur/epidemiologia , Tomografia Computadorizada por Raios X , Fraturas do Colo Femoral/diagnóstico por imagem , Fraturas do Colo Femoral/epidemiologia , Fraturas do Colo Femoral/cirurgiaRESUMO
BACKGROUND: Classifications describing forearm lesions in patients with Hereditary Multiple Osteochondromatosis (HMO) have been used to recommend surgical intervention and stratify outcomes; however, there is no consensus on which classification offers greater reliability. The purpose of this study was to determine the reliability of the Masada classification and newer classifications among pediatric hand surgeons. METHODS: One hundred one patients with HMO between June 2014 and October 2019 were enrolled in the Congenital Upper Limb Differences (CoULD) Registry. Of those, 67 patients with 101 forearms were included. Four pediatric hand surgeons from the CoULD study group undertook an online evaluation. Each rater classified radiographs according to the Masada classification. Six weeks later, raters were asked to reclassify images according to the Masada, Gottschalk, and Jo classifications. Rater agreement for these classifications was assessed by estimating Fleiss kappa along with a 95% CI. RESULTS: Interrater agreement for Masada classification after the first reading was poor (κ=0.35; 95% CI=0.30-0.41) across all raters. Interrater agreement across the 4 raters decreased for the Masada classification from the first to the second reading (κ=0.35 vs 0.21; P <0.001). Intrarater agreement for the Masada classification ranged from 0.32 to 0.63 from the first to the second study reading. Gottschalk and Jo classifications yielded significantly better interrater agreement compared with Masada (κ=0.43 vs 0.21; P <0.001). Unclassifiable cases were highest in the Masada classification (34% to 44%) and lower in the Jo (17%) and Gottschalk (14%) classifications. CONCLUSION: Despite wide use, the Masada classification was found to have low reliability when classifying forearm deformities in HMO. Gottschalk offered more options for location, yet lacked deformity description including radial head dislocation. Jo classification offered more locations than Masada and incorporated radial head dislocation in some patterns. Based on the shortcomings in all 3 classification systems, the development of a more inclusive and reliable classification is warranted. LEVEL OF EVIDENCE: Level II; Diagnostic.
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Exostose Múltipla Hereditária , Luxações Articulares , Deformidades Congênitas das Extremidades Superiores , Humanos , Criança , Exostose Múltipla Hereditária/diagnóstico por imagem , Exostose Múltipla Hereditária/cirurgia , Reprodutibilidade dos Testes , Antebraço/anormalidades , Variações Dependentes do ObservadorRESUMO
PURPOSE: To advance the understanding of the epidemiology and treatment outcomes of congenital upper limb differences, a multicenter registry for Congenital Upper Limb Differences (CoULD) was established. After 4 years of recruitment, we sought to examine whether the relative frequency of congenital conditions compares with prior cross-sectional research and how the data have matured over time by (1) comparing our registry population with previous studies in similar populations and (2) evaluating the change over time of relative frequencies of selected conditions within the CoULD registry cohort, specifically to investigate for registry inclusion effects. METHODS: Data from the 2 founding centers in the CoULD registry were analyzed over a 4-year period. We compared patients included in the CoULD registry against 2 prior studies by matching each condition according to the Oberg-Manske-Tonkin classification system. The relative frequency of 4 representative conditions was calculated to evaluate change over time and to determine when the inception cohort effect diminished. RESULTS: The CoULD cohort of 1,381 patients was found to have notable differences compared with a 1-year cross-sectional cohort from the U.S. Midwest and a Swedish birth registry. Each of these registries had differences from the CoULD population in prevalence for approximately 33% of the diagnosis categories. The CoULD registry identified and included more pathologies of late presentation and those that do not commonly require surgical care. Changes in relative frequencies of incident and prevalent conditions, the registry inclusion effect, occurred early and stabilized by the third year. CONCLUSIONS: The CoULD registry captures a different relative frequency of conditions than prior studies in similar populations. The findings highlight the CoULD registry may be a more accurate representation of clinical practice in tertiary referral centers; however, it is important to note that there was a registry inclusion effect identified. CLINICAL RELEVANCE: Inclusion criteria are an important consideration with any longitudinal data collection method and data should display stability prior to registry reporting.
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Deformidades Congênitas das Extremidades Superiores , Estudos Transversais , Humanos , Sistema de Registros , Suécia , Extremidade SuperiorRESUMO
PURPOSE: Pollicization is a well-accepted treatment for thumb hypoplasia, yet little is known about the reasons why patients return early in the postoperative period and complications that occur after surgery. The purpose of this investigation was to describe 30-day returns, readmission, and complication rates after pollicization in the United States. METHODS: A total of 459 pollicization procedures performed in 408 patients at 38 U.S. pediatric hospitals from 2003 to 2014 were identified using the Pediatric Health Information System database. A stepwise search strategy identified returns and readmissions within 30 days after pollicization to quantify complications and/or additional procedures. Risk factors for readmission and complications were compared across groups using uni- and multivariable general linear modeling. RESULTS: There were 61 patients who returned to the hospital (emergency department, ambulatory surgery, or inpatient hospitalization) within 30 days of their pollicization. Of those, 39 patients returned for suture removal, cast changes, or other expected aspects of postoperative care. The remaining 22 patients had a total of 26 complications, accounting for a 4.8% complication rate. The majority had vascular complications and wound problems. Single complications ranging from wound infection to hemorrhage were found in 20 cases. Overall, 35 of the 61 returns were readmitted to the hospital for treatment of complications or additional procedures. There was no effect of age group, diagnosis, geographical region, or physician subspecialty on the likelihood of complication or readmission. CONCLUSIONS: Sixty-one patients returned within 30 days of their pollicization, and 22 presented with a complication (4.8%), most commonly vascular in nature. These baseline data are informative because they identify opportunities for future preventative measures and quality improvement. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.
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Dedos/transplante , Polegar/anormalidades , Polegar/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Hospitais Pediátricos , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Readmissão do Paciente/estatística & dados numéricos , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Estados Unidos/epidemiologiaRESUMO
PURPOSE: To characterize the functional, emotional, and social impact of congenital upper limb differences on affected children and families before treatment, using validated functional outcome instruments. METHODS: From June 2014 to March 2016, 586 children with congenital upper limb differences from 2 pediatric hospitals were enrolled in the Congenital Upper Limb Differences registry. Demographic, clinical, and radiographic data were collected, and diagnoses categorized according to the Oberg-Manske-Tonkin classification. Functional outcomes were assessed in 301 patients using the Pediatric Outcomes Data Collection Instrument (PODCI) and Patient-Reported Outcomes Measurement Information System (PROMIS) upper extremity (UE) function, pain, anxiety, depression, and peer relationships modules. RESULTS: The cohort had high median PODCI scores in all domains, ranging from 83 to 100 in children and adolescents. Patients had decreased PROMIS UE scores compared with population norms; however, they showed low scores for pain, anxiety, depression and higher scores in the peer relationship domain, respectively. Patients with entire limb involvement had higher PROMIS pain scores and lower PODCI UE and global functioning than those with differences limited only to the hand. Compared with those with bilateral involvement, patients with unilateral differences reported higher scores for PODCI sports global functioning, better PROMIS UE function, and lower pain scores. Additional orthopedic conditions and medical comorbidities negatively influenced all PODCI scores and PROMIS pain and UE function domains. CONCLUSIONS: Children with congenital hand differences report decreased upper limb function but better peer relationships and positive emotional states compared with population norms. CLINICAL RELEVANCE: The Congenital Upper Limb Differences registry is a valid source of information related to congenital upper limb differences in clinical practice. With continuous enrollment and longitudinal follow-up, the registry will increase the understanding of UE function and psychosocial aspects of health in pediatric population.
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Medidas de Resultados Relatados pelo Paciente , Deformidades Congênitas das Extremidades Superiores/fisiopatologia , Deformidades Congênitas das Extremidades Superiores/psicologia , Adolescente , Ansiedade/psicologia , Desempenho Atlético/fisiologia , Criança , Pré-Escolar , Comorbidade , Depressão/psicologia , Feminino , Humanos , Lactente , Relações Interpessoais , Estudos Longitudinais , Masculino , Medição da Dor , Grupo Associado , Qualidade de Vida , Sistema de Registros , Estados UnidosRESUMO
BACKGROUND: The purpose of this investigation was to determine the reliability of the Oberg-Manske-Tonkin (OMT) classification system applied to patients enrolled in a prospective, multicenter cohort study of congenital upper limb differences. Our hypothesis was that the OMT classification would exhibit high intraobserver and interobserver reliability and thus would be an appropriate tool for the new Congenital Upper Limb Differences registry. METHODS: Four pediatric hand surgeons independently reviewed the medical records, clinical photographs, and radiographs of 60 randomly selected patients previously enrolled in the Congenital Upper Limb Differences registry. Patient's information was summarized and shared electronically (PowerPoint). Each rater classified each congenital anomaly according to the OMT classification system. Responses were recorded using a web-based data capture tool (REDCap). Three weeks later, the surgeons repeated the process. Intraobserver reliability for each rater was assessed using Fleiss' κ. Interobserver reliability was assessed using percent exact agreement (when all 4 raters were in agreement) as well as Fleiss' κ. RESULTS: Using the OMT classification, intraobserver reliability for the 4 pediatric hand surgeons showed almost perfect agreement, with κ values in the range of 0.89 to 0.93. Interobserver reliability demonstrated substantial agreement, with κ value of 0.79 (95% confidence interval, 0.77-0.82) in the first reading and 0.80 (95% confidence interval, 0.77-0.83) in the second reading. The highest possible agreement (κ=1) was seen for the following diagnoses: congenital dislocation of radial head (OMT I.A.2.v), Madelung deformity (I.A.2.vii), radial polydactyly (I.B.2.iii), triphalangeal thumb (I.B.2.iv), Kirner deformity (I.B.4.vi), and osteochondromatosis (3.B.4.i). CONCLUSIONS: The OMT classification of congenital upper limb differences exhibits substantial to almost perfect intraobserver and interobserver reliability among pediatric hand surgeons at different institutions. The OMT is therefore well-suited for classification of congenital upper limb anomalies in patients enrolled in a multicenter registry. LEVEL OF EVIDENCE: Level II-diagnostic.
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Deformidades Congênitas da Mão/classificação , Criança , Feminino , Deformidades Congênitas da Mão/diagnóstico por imagem , Humanos , Masculino , Variações Dependentes do Observador , Estudos Prospectivos , Radiografia , Sistema de Registros , Reprodutibilidade dos Testes , Deformidades Congênitas das Extremidades Superiores/classificação , Deformidades Congênitas das Extremidades Superiores/diagnóstico por imagemRESUMO
BACKGROUND AND IMPORTANCE: Giant cavernous malformations (GCM) are low flow, angiographically occult vascular lesions, with a diameter >4 cm. Cerebellar GCMs are extremely rare, with only seven cases reported based on English literature. These lesions are most commonly seen in the pediatric age group, which is known to have an increased risk of hemorrhage, being surgery clearly recommended. CLINICAL PRESENTATION: An 18-month-old girl presented with a 6-month history of cervical torticollis and upper extremities clumsiness. An MRI revealed a 57 × 46 × 42 mm multi-cystic, left cerebellar hemisphere mass, showing areas of hemorrhages and cysts with various stages of thrombus. There was no enhancement with contrast. Cerebral angiography ruled out an arteriovenous malformation. She underwent a left paramedian occipital craniotomy, and macroscopic gross total resection was accomplished. Histopathologic examination was consistent with a cavernous malformation. After surgery, the patient had no new neurological deficit and an uneventful postoperative recovery. Follow-up MRI confirmed total removal of the lesion. CONCLUSION: Cerebellar GCMs in children are symptomatic lesions, which prompt immediate surgical treatment. These are rare lesions, which can radiologically and clinically mimic a tumor with bleed, having to be considered in the differential diagnosis of neoplastic lesions. Cerebellar GCMs might be suspected in the presence of large hemorrhagic intra-axial mass with "bubbles of blood," multi-cystic appearance, surrounded by hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect. Careful radiological study provides a preoperative diagnosis, but its confirmation requires histopathological examination. Complete surgical removal should be attempted when possible.
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Neoplasias Cerebelares/diagnóstico por imagem , Neoplasias Cerebelares/cirurgia , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico por imagem , Hemangioma Cavernoso do Sistema Nervoso Central/cirurgia , Feminino , Humanos , LactenteRESUMO
HSV-1 establishes life-long latency that can result in clinical relapses or in asymptomatic virus shedding. Although virtually all adults have been exposed to HSV-1, the clinical course varies remarkably. Genetic host variability could be related to this clinical diversity. In this study, we analyzed the contribution of gene families in chromosomes 1, 6, 12, and 19, which encode key regulators of the innate and adaptive immunity, in a cohort of 302 individuals. Class I and class II alleles of the HLA system, the copy-number variation of NK cell receptor genes (KIR and NKG2C), the combinations of killer cell Ig-like receptor and their HLA ligands, and CD16A and CD32A allotypes of variable affinity for IgG subclasses were all studied. Although no major susceptibility locus for HSV-1 was identified, our results show that the risk of suffering clinical HSV-1 infection is modified by MHC class I allotypes (B*18, C*15, and the group of alleles encoding A19), the high-affinity receptor/ligand pair KIR2DL2/HLA-C1, and the CD16A-158V/F dimorphism. Conversely, HLA class II and CD32A polymorphisms and NKG2C deletion did not seem to influence the clinical course of herpetic infection. Collectively, these findings support an important role in host defense against herpetic infection for several polymorphic genes implicated in adaptive immunity and in surveillance of its subversion. They confirm the crucial role of cytotoxic cells (CTL and NK) and the contribution of genetic diversity to the clinical course of HSV-1 infection.
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Imunidade Adaptativa/genética , Predisposição Genética para Doença , Herpes Simples/genética , Herpesvirus Humano 1 , Imunidade Inata/genética , Polimorfismo Genético , Adulto , Cromossomos Humanos/genética , Cromossomos Humanos/imunologia , Feminino , Antígenos HLA-C/genética , Antígenos HLA-C/imunologia , Herpes Simples/imunologia , Humanos , Vigilância Imunológica/genética , Vigilância Imunológica/imunologia , Células Matadoras Naturais/imunologia , Masculino , Pessoa de Meia-Idade , Receptores de IgG/genética , Receptores de IgG/imunologia , Receptores KIR2DL2/genética , Receptores KIR2DL2/imunologia , Linfócitos T Citotóxicos/imunologiaRESUMO
Background: There is growing evidence of increased venous thromboembolism (VTE) incidence in children with trauma or infection. The purpose of this study was to conduct a systematic review of existing literature related to VTE in the pediatric orthopaedic population, to estimate the overall incidence of VTE and identify risk factors associated with this condition. Methods: A systematic review of the available literature was performed to identify articles that described VTE in pediatric orthopaedic surgery or admission. Literature queries were performed to identify articles published from 1980 to 2021 that included patients ≤21 years of age. A stepwise search strategy of 5 electronic databases yielded 1,426 articles, which were filtered by 2 reviewers to identify 30 articles for full-text review. The primary aim was to determine the rate of VTE, and the secondary aim was to identify risk factors for VTE. The pooled incidence of VTE was estimated and reported in cases per 10,000. Studies were stratified by study size, by trauma versus elective surgery, and by orthopaedic subspecialty. Results: The 30 articles reported 3,113 VTE events in 2,467,764 pediatric patients (including those with non-orthopaedic conditions), for a pooled VTE incidence of 20 events (95% confidence interval [CI] = 10.8 to 37.2) per 10,000. Four of the studies were excluded for incomplete data or high heterogeneity. The remaining 26 studies had 850,268 orthopaedic patients with 1,108 cases of VTE, for a pooled VTE incidence of 16.6 events (95% CI = 9.1 to 30.5) per 10,000. Studies with <10,000 patients and those involving a diagnosis of trauma had the highest VTE incidence when stratification was performed. The most frequently analyzed risk factors in 15 available studies included age, sex, obesity/body mass index, type of surgery, and use of a central venous catheter. Conclusions: This systematic review indicated that the risk of VTE associated with pediatric orthopaedic surgery or admission remains low, at <17 events per 10,000 cases. However, orthopaedic surgeons should be aware of the most common risk factors associated with pediatric orthopaedic VTE and should pay special attention to traumatic etiologies, as these yielded the highest incidence. Levels of Evidence: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.
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Purpose: Risk factors for congenital upper limb differences (CoULDs) are often studied at the general population level. The CoULD registry provides a unique opportunity to study prenatal risk factors within a large patient sample. Methods: All patients enrolled between June 2014 and March 2020 in the prospective CoULD registry, a national multicenter database of patients diagnosed with a CoULD, were included in the analysis. We analyzed self-reported, prenatal risk factors, including maternal smoking, alcohol use, recreational drug use, prescription drug use, gestational diabetes mellitus (GDM), and gestational hypertension. The outcome measures included comorbid medical conditions, proximal involvement of limb difference, bilateral involvement, and additional orthopedic conditions. Multivariable logistic regression was used to analyze the effect of the risk factors, controlling for sex and the presence of a named syndrome. Results: In total, 2,410 patients were analyzed, of whom 72% (1,734) did not have a self-reported risk factor. Among the 29% (676) who did have at least 1 risk factor, prenatal maternal prescription drug use was the most frequent (376/2,410; 16%). Maternal prescription drug use was associated with increased odds of patient medical comorbidities (odds ratio [OR] = 1.43, P = .02). Gestational diabetes mellitus was associated with increased odds of comorbid medical conditions (OR = 1.58, P = .04), additional orthopedic conditions (OR = 1.51, P = .04), and proximal involvement (OR = 1.52, P = .04). Overall, reporting 1 or more risk factors increased the odds of patient comorbid medical conditions (OR = 1.42, P < .001) and additional orthopedic conditions (OR = 1.25, P = .03). Conclusions: Most caregivers (72%) did not report a risk factor during enrollment. However, reporting a risk factor was associated with patient medical and orthopedic comorbidities. Of note, GDM alone significantly increased the odds of both these outcome measures along with proximal limb differences. These findings highlight the ill-defined etiology of CoULDs but suggest that prenatal risk factors, especially GDM, are associated with a higher degree of morbidity. Type of study/level of evidence: Prognostic III.
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OBJECTIVE: Sarcopenia is a major cause of morbidity in rheumatoid arthritis patients. Our purpose was to determine whether polyautoimmunity is associated with sarcopenia and alterations in whole body composition in patients with rheumatoid arthritis (RA). METHODS: We performed a cross-sectional observational study of a series of cases of RA. All patients were recruited consecutively from a rheumatology clinic. Body composition by dual-energy x-ray absorptiometry (DEXA) was assessed. The variables of interest were polyautoimmunity (RA associated with other autoimmune diseases), sarcopenia, fat mass, and body mass index (BMI). Other variables included were clinical-analytical and inflammatory cytokines and adipokines. The relationship between sarcopenic obesity and the presence of polyautoimmunity was studied using multivariate analysis. RESULTS: Of the 94 patients with RA included in the study, 15 (16%) had polyautoimmunity. A total of 23 patients with RA (24.5%) had sarcopenia, which was more prevalent in patients with polyautoimmunity than in patients without polyautoimmunity (46.7% vs 20.3%; p = .029). Sarcopenia was not associated with body fat content (p = .870) or with BMI (p = .998). The multivariate analysis showed the factors associated with polyautoimmunity in RA to be sarcopenia (odds ratio [95% CI], 4.80 [1.49-13.95]), BMI (1.18 [1.04-1.35]), and resistin (1.249 [1.01-1.53]). CONCLUSION: Sarcopenia and obesity were more prevalent in patients with RA and polyautoimmunity. Resistin values were also higher in this group than in patients with RA without polyautoimmunity.
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Artrite Reumatoide , Sarcopenia , Humanos , Sarcopenia/complicações , Resistina , Estudos Transversais , Obesidade/complicações , Artrite Reumatoide/complicaçõesRESUMO
BACKGROUND: Deficiency of the radial aspect of the forearm and hand is the most common congenital longitudinal deficiency of the upper limb. Radial longitudinal deficiency is associated with several named syndromes. The purpose of the present study was to explore patterns of radial longitudinal deficiency and thumb hypoplasia in syndromes and to examine the severity of these differences across various syndromes. METHODS: Data were collected from the Congenital Upper Limb Differences (CoULD) registry. Congenital differences are classified in the registry with use of the Oberg-Manske-Tonkin (OMT) classification system. Diagnosis of a syndrome by a physician as noted in the CoULD registry was recorded. Thumb deficiency and radial deficiency were classified according to the modified versions of the Blauth criteria and the Bayne and Klug criteria, respectively. RESULTS: We identified 259 patients with 383 affected limbs with radial deficiency. Eighty-three of these patients had a diagnosed syndrome. The severity of radial deficiency was correlated with the severity of thumb deficiency. The Kendall tau coefficient indicated significant correlation between radial severity and thumb severity (tau = 0.49 [95% confidence interval = 0.40 to 0.57]; p < 0.05). Subjects with a syndrome were twice as likely to have bilateral deficiency and 2.5 times more likely to have both radial and thumb deficiency compared with subjects without a syndrome. Subjects with VACTERL syndrome (vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula, renal anomalies, and limb defects) had patterns of thumb and radial deficiency similar to the general cohort, whereas subjects with Holt-Oram syndrome, TAR (thrombocytopenia absent radius) syndrome, and Fanconi anemia demonstrated varied presentations of thumb and radial deficiency. CONCLUSIONS: The present study investigated the characteristics of patients with radial longitudinal deficiency and thumb hypoplasia. Our results support the findings of previous research correlating the severity of radial deficiency with the severity of thumb deficiency. Furthermore, we identified characteristic features of patients with radial longitudinal deficiency and associated syndromes.
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Rádio (Anatomia)/anormalidades , Polegar/anormalidades , Braço/anormalidades , Braço/patologia , Criança , Pré-Escolar , Feminino , Doenças Genéticas Inatas/complicações , Doenças Genéticas Inatas/genética , Doenças Genéticas Inatas/patologia , Loci Gênicos , Humanos , Lactente , Masculino , Rádio (Anatomia)/patologia , Sistema de Registros , Índice de Gravidade de Doença , Síndrome , Polegar/patologia , Estados UnidosRESUMO
Mycoviruses appear to be widespread in Fusarium species worldwide. The aim of this work was to identify mycoviral infections in Fusarium spp., isolated from maize and sorghum grown in Argentina, and to estimate their potential effects on the pathogenicity and toxigenesis of the host fungus towards maize. Mycoviruses were identified in 2 out of 105 isolates analyzed; Fusarium verticillioides strain Sec505 and Fusarium andiyazi strain 162. They were characterized as members of the genus Mitovirus by high-throughput sequencing and sequence analysis. The F. verticillioides mitovirus was a novel mycovirus whereas the F. andiyazi mitovirus was found to be a new strain of a previously identified mitovirus. We have named these mitoviruses, Fusarium verticillioides mitovirus 1 (FvMV1) and Fusarium andiyazi mitovirus 1 strain 162 (FaMV1-162). To our knowledge, FvMV1 is the first mycovirus reported as naturally infecting F. verticillioides, the major causal agent of ear rot and fumonisin producer in corn. Both mitoviruses exhibited 100% vertical transmission rate to microconidia. The Fa162 strain infected with FaMV1-162 did not show phenotypic alterations. In contract, F. verticillioides Sec505 infected with FvMV1 showed increased virulence as well as microconidia and fumonisin-B1 production, compared with two uninfected strains. These results suggest that FvMV1 could have a role in modulating F. verticillioides pathogenicity and toxin production worth further exploring.
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Micovírus/classificação , Fusarium/patogenicidade , Fusarium/virologia , Sorghum/microbiologia , Zea mays/microbiologia , Argentina , Micovírus/isolamento & purificação , Sequenciamento de Nucleotídeos em Larga Escala , Filogenia , Doenças das Plantas/microbiologia , Esporos Fúngicos/virologiaRESUMO
The variation of the color intensity of the chromatophores present in the wings of Schistocerca americana was analyzed by exposing 31 specimens to thermal transitions within the range of 45 - 5 °C. The adult specimens were collected using a mini-terrarium of dimensions 40x40x30 cm. As a substrate, a layer of soil, stones, and finally a layer of grass were used along with branches of bushes and leaves; hydroponic lettuce, cabbage and the grass were used as food for the specimens. Optical microscopy of the wings of the insects was used for live observation without coverslips or contrasting substances. At 45°C, degradation of color intensity was observed in the chromatophores present in the wings. At 5°C, chromatophores intensify their color to brownish-black. This temperature was the extreme minimum that S. americana could tolerate. We found negative correlation between the temperature and the degree of darkness (R2 = 0.8038). Our results are in accordance with a previously published study in which Phaulacridium vittatum was examined, as the decrease of temperature caused darkening color change in melanin-type chromatophores. The present investigation can be considered as the first initial study of its kind for S. americana, in terms of examining the changes in the color intensity of the chromatophores present in the wings caused by thermal transition under laboratory conditions.
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Cromatóforos , Insetos , Asas de Animais , Animais , IncidênciaRESUMO
BACKGROUND: Syndactyly is one of the most common congenital differences of the upper extremity and offers an exceptional opportunity to evaluate value-based care in pediatric orthopedic surgery. We designed a study to characterize complications and cost associated to syndactyly surgery among US pediatric hospitals. METHODS: A total of 2047 patients were identified for syndactyly surgery at 38 pediatric hospitals from 2009 to 2012 using the Pediatric Health Information System (PHIS) database. We examined costs as well as complication rates across hospitals stratified by patient and hospital variables. RESULTS: The postoperative complication rate was 1.9% (95% confidence interval [CI]: 1.3%-2.5%). Postoperative infection rate was 1.6% and surgical complication rate was 0.3%. Median adjusted standardized cost was $4112.5 (interquartile range: $2979-$6049). Patients with more than 1 diagnosis had 19 times higher risk of complications and were associated with 13% more hospital cost than those with syndactyly as single diagnosis ( P < .001). Finally, there was a wide variation in cost across hospitals; 8 (21%) yielded confidence limits above the benchmarked value. CONCLUSIONS: In the United States, it is important to recognize variations in practice of syndactyly surgery in hopes of developing quality improvement strategies in pediatric orthopedic surgery.
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Custos Hospitalares/estatística & dados numéricos , Complicações Pós-Operatórias/epidemiologia , Sindactilia/economia , Sindactilia/cirurgia , Adolescente , Distribuição por Idade , Procedimentos Cirúrgicos Ambulatórios/economia , Procedimentos Cirúrgicos Ambulatórios/estatística & dados numéricos , Criança , Pré-Escolar , Estudos de Coortes , Bases de Dados Factuais , Feminino , Hospitais Pediátricos , Humanos , Lactente , Recém-Nascido , Seguro Saúde/estatística & dados numéricos , Tempo de Internação/estatística & dados numéricos , Masculino , Medicaid/estatística & dados numéricos , Grupos Raciais/estatística & dados numéricos , Estudos Retrospectivos , Distribuição por Sexo , Sindactilia/epidemiologia , Estados Unidos/epidemiologiaRESUMO
Congenital anomalies are prevalent in Nicaragua, and disability is estimated to be 10% in the general population. We studied children with congenital upper limb differences, as they are vulnerable to disability. This case study documents a collaborative effort between American and Nicaraguan orthopedic surgeons to determine unmet health needs of children with congenital upper limb differences at Hospital Manuel de Jesus Rivera ("La Mascota" Hospital) in Nicaragua, with the goal of developing programs that successfully address these needs within the context of the priorities of the community. Participants were recruited during one of the biannual pediatric hand specialty clinics held by a partnership of pediatric hand surgeons and occupational therapists under the auspices of Health Volunteers Overseas (La Brigada de las Manos, or "La Brigada") and Nicaraguan orthopedic surgeons. Structured interviews were performed with 34 parents or caregivers of patients with the diagnosis of a congenital upper limb difference. Parents were asked to rank the social, economic, environmental, and biological factors that determine health according to priority. Using the Hanlon Method for prioritizing health problems, in consultation with local providers and the program director of La Brigada, five needs were identified: (1) improvements in access to specialized care from hand surgeons and (2) rehabilitation specialists; (3) improvements in upper extremity function; (4) access to transportation; and (5) improvement in physical activity and sports participation. Based on the results of this needs assessment, we learned that some of the needs were already part of the ongoing work of the partnership, but in addition, more needs became evident; for that reason, local health care providers and members of La Brigada identified potential solutions to these needs and are currently working to translate these in future interventions.
RESUMO
BACKGROUND: Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid, is a serious, autoimmune, blistering disorder that can result in blindness and other complications as a result of scarring of the mucous membranes. Effective treatment modalities are often toxic. Herein, we describe a novel therapeutic approach that is based on 2 reports in the literature of the successful use of etanercept to treat MMP. OBSERVATIONS: Three patients with MMP were treated with subcutaneous injections of 25 mg of etanercept twice weekly. All 3 patients had oral mucosal involvement, and 1 had severe, recalcitrant, ocular disease. Oral mucosal disease improved in all 3 patients. The patient with ocular involvement experienced stabilization of progression. CONCLUSIONS: Effective treatment modalities for MMP are often toxic. Etanercept may be an effective treatment option for MMP of the oral and ocular mucous membranes. This therapy should be considered as an alternative treatment option for patients who would require other aggressive systemic treatments, such as cyclophosphamide, corticosteroids, azathioprine sodium, and intravenous immunoglobulin.
Assuntos
Imunoglobulina G/uso terapêutico , Fatores Imunológicos/uso terapêutico , Penfigoide Mucomembranoso Benigno/tratamento farmacológico , Receptores do Fator de Necrose Tumoral/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Doenças da Túnica Conjuntiva/complicações , Doenças da Túnica Conjuntiva/diagnóstico , Doenças da Túnica Conjuntiva/tratamento farmacológico , Doenças da Túnica Conjuntiva/patologia , Diagnóstico Diferencial , Etanercepte , Feminino , Humanos , Imunoglobulina G/administração & dosagem , Fatores Imunológicos/administração & dosagem , Injeções Subcutâneas , Pessoa de Meia-Idade , Penfigoide Mucomembranoso Benigno/complicações , Penfigoide Mucomembranoso Benigno/diagnóstico , Penfigoide Mucomembranoso Benigno/patologia , Receptores do Fator de Necrose Tumoral/administração & dosagem , Índice de Gravidade de DoençaRESUMO
BACKGROUND: The accurate histologic diagnosis of germ cell tumors in the pineal region is a keystone for determining the best treatment strategy and prognosis. This situation poses a challenge for the neuropathologist, considering the lack of a standarized procedure to obtain biopsy samples, which results in few and small specimens, which are not suitable for diagnosis. CASE DESCRIPTION: We report a case in which a pineal region mixed germ cell tumor was accurately diagnosed by performing histologic mapping through a dual burr-hole endoscopic approach. The technical pitfalls and other considerations necessary for obtaining an accurate diagnosis in this tumor subgroup are specified. In addition, the histologic analysis regarding the sampling technique used is described. CONCLUSIONS: The supraorbital frontal endoscopic approach enables the surgeon to perform histologic mapping of pineal region tumors, allowing standarization of the procedure used to obtain the specimens. This approach could result in a more accurate diagnosis, especially in mixed germ cell neoplasms.
Assuntos
Tumor do Seio Endodérmico/patologia , Germinoma/patologia , Neoplasias Complexas Mistas/patologia , Pinealoma/patologia , Teratoma/patologia , Adolescente , Biópsia , Tumor do Seio Endodérmico/complicações , Tumor do Seio Endodérmico/diagnóstico por imagem , Tumor do Seio Endodérmico/cirurgia , Germinoma/complicações , Germinoma/diagnóstico por imagem , Germinoma/cirurgia , Humanos , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/etiologia , Imageamento por Ressonância Magnética , Masculino , Neoplasias Complexas Mistas/complicações , Neoplasias Complexas Mistas/diagnóstico por imagem , Neoplasias Complexas Mistas/cirurgia , Neoplasias Embrionárias de Células Germinativas/complicações , Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/cirurgia , Neuroendoscopia/métodos , Pinealoma/complicações , Pinealoma/diagnóstico por imagem , Pinealoma/cirurgia , Teratoma/complicações , Teratoma/diagnóstico por imagem , Teratoma/cirurgia , Tomografia Computadorizada por Raios XRESUMO
Se define como infección del sitio quirúrgico a toda aquella que ocurre en la incisión quirúrgica o cerca de la misma, pudiendo extenderse hacia estructuras adyacentes más profundas. Por lo general tienen baja incidencia y están asociadas con altas tasas de morbimortalidad, hospitalización prolongada, reintervención y mayores costos. Representan del 20 al 31% de todas las infecciones adquiridas en el hospital. Se identifican elementos de riesgo específicos del paciente y del entorno quirúrgico, los mismos que por lo general se superponen y coadyuvan. La etiología es multifactorial siendo la fuente más común de contaminación la flora endógena de la piel del paciente. Debido a que son varios los factores contribuyentes al desarrollo de infección del sitio quirúrgico, la prevención es compleja y requiere de la integración de medidas antes, durante y después de la cirugía. El objetivo de este trabajo es presentar un repaso actual de la epidemiología, incidencia y factores de riesgo asociados a infección del sitio quirúrgico posterior a procedimientos neuroquirúrgicos. Además, ofrecer recomendaciones basadas en la evidencia sobre estrategias y protocolos para el manejo y prevención de esta condición que con relativa frecuencia es vista en centros hospitalarios.
Surgical-site infections are infections that occur either in, or in close proximity to a surgical incision, and can spread to deeper, adjacent structures. Their incidence is considerable, and they are associated with high morbidity and mortality rates, prolonged hospitalization, the increased need for re-intervention, and higher costs. They represent 20% to 31% of all infections acquired in hospitals. Several risk factors specific to the patient and surgical environment have been identified, and these often overlap. The etiology of surgical-site infections is multi-factorial, the most common source of contamination being the endogenous flora of the patient's skin. Since several factors contribute to the development of surgical site infections, prevention is complex and requires integrating measures before, during and after surgery. The main objective of the current paper is to review the epidemiology, including the incidence of and risk factors for surgical-site infections after neurosurgical procedures. In addition, we provide evidence-based recommendations on strategies and protocols for the management and prevention of frequently-observed complications in hospital settings.