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INTRODUCTION/AIMS: Myxovirus resistance protein A (MxA) is a type I interferon (IFN1) pathway activation marker and MxA sarcoplasmic expression is currently recognized as a highly specific marker for dermatomyositis (DM). However, we have frequently observed endothelial tubuloreticular inclusions (TRI), another surrogate IFN1 activation marker, in a variety of overlap myositides. The aim of this study was to examine MxA expression in those myositides. METHODS: We retrospectively performed MxA immunostaining on a wide range of myositides. RESULTS: MxA sarcoplasmic expression was present in DM (94.4%, 17/18), active lupus myositis (LM, 80%,16/20), inactive LM (36%, 4/11), antisynthetase syndrome (ASyS, 20%, 2/10), systemic sclerosis (13%, 2/15), Sjogren's syndrome (7.7%, 1/13), and human immunodeficiency virus (HIV) myositis (5.6%, 1/18) and was absent in immune-mediated necrotizing myopathy (IMNM, 0/16) and hydroxychloroquine myopathy (0/5). The sensitivity and specificity of MxA sarcoplasmic expression for LM and DM combined compared with all other myositides were 84.6% (95% CI: 69.5-94.1) and 92.1 (95% CI: 83.6-97.0), respectively, and superior to TRIs. MxA capillary expression was nonspecific. Histologically, 35% of LM cases demonstrated a unique panfascicular necrotizing myopathy pattern. The remainder of the LM cases had significant morphological overlap with DM/ASyS (20%), IMNM (20%), or polymyositis (15%). DISCUSSION: MxA sarcoplasmic expression is highly prevalent in LM and DM and is a useful marker in differentiating DM and LM from other myositides. LM can manifest in various pathology patterns that need to be differentiated from DM, IMNM, ASyS, and polymyositis.
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Dermatomiosite , Doenças Musculares , Miosite , Orthomyxoviridae , Polimiosite , Humanos , Biomarcadores , Dermatomiosite/patologia , Miosite/patologia , Polimiosite/patologia , Estudos RetrospectivosRESUMO
BACKGROUND: Acute bronchiolitis (AB) is the most common lower respiratory tract infection in infants. Objective scoring tools and plain film radiography have limited application, thus diagnosis is clinical. The role of point-of-care lung ultrasound (LUS) is not well established. OBJECTIVE: We sought to characterize LUS findings in infants presenting to the pediatric ED diagnosed with AB, and to identify associations between LUS and respiratory support (RS) at 12 and 24 h, maximum RS during hospitalization, disposition, and hospital length of stay (LOS). METHODS: Infants ≤12 months presenting to the ED and diagnosed with AB were enrolled. LUS was performed at the bedside by a physician. Lungs were divided into 12 segments and scanned, then scored and summated (min. 0, max. 36) in real time accordingly: 0 - A lines with <3 B lines per lung segment. 1 - ≥3 B lines per lung segment, but not consolidated. 2 - consolidated B lines, but no subpleural consolidation. 3 - subpleural consolidation with any findings scoring 1 or 2. Chart review was performed for all patients after discharge. RS was categorized accordingly: RS (room air), low RS (wall O2 or heated high flow nasal cannula <1 L/kg), and high RS (heated high flow nasal cannula ≥1 L/kg or positive pressure). RESULTS: 82 subjects were enrolled. Regarding disposition, the mean (SD) LUS scores were: discharged 1.18 (1.33); admitted to the floor 4.34 (3.62); and admitted to the ICU was 10.84 (6.54). For RS, the mean (SD) LUS scores at 12 h were: no RS 1.56 (1.93), low RS 4.34 (3.51), and high RS 11.94 (6.17). At 24 h: no RS 2.11 (2.35), low RS 4.91 (3.86), and high RS 12.64 (6.48). Maximum RS: no RS 1.22 (1.31), low RS 4.11 (3.61), and high RS 10.45 (6.16). Mean differences for all dispositions and RS time points were statistically significant (p < 0.05, CI >95%). The mean (SD) hospital LOS was 84.5 h (SD 62.9). The Pearson correlation coefficient (r) comparing LOS and LUS was 0.489 (p < 0.0001). CONCLUSION: Higher LUS scores for AB were associated with increased respiratory support, longer LOS, and more acute disposition. The use of bedside LUS in the ED may assist the clinician in the management and disposition of patient's diagnosed with AB.
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Bronquiolite , Sistemas Automatizados de Assistência Junto ao Leito , Lactente , Humanos , Criança , Pulmão/diagnóstico por imagem , Bronquiolite/diagnóstico por imagem , Bronquiolite/terapia , Ultrassonografia , Serviço Hospitalar de EmergênciaRESUMO
BACKGROUND: In facial reanimation, dual-innervated gracilis free functional muscle transfers (FFMTs) may have amalgamated increases in tone, excursion, synchroneity, and potentially spontaneity when compared with single innervation. The ideal staging of dual-innervated gracilis FFMTs has not been investigated. We aim to compare objective long-term outcomes following one- and two-stage dual-innervated gracilis FFMTs. METHODS: Included were adult patients with facial paralysis who underwent either one- (one-stage group) or two-stage (two-stage group) dual-innervated gracilis FFMT with ≥1 year of postoperative follow-up. Facial measurements were obtained from standardized photographs of patients in repose, closed-mouth smile, and open-mouth smile taken preoperatively, 1 year postoperatively, and 3 years postoperatively. Symmetry was calculated from the absolute difference between the paralyzed and healthy hemiface; a lower value indicates greater symmetry. RESULTS: Of 553 facial paralysis patients, 14 were included. Five and nine patients were in the one- and two-stage groups, with mean follow-up time, respectively, being 2.5 and 2.6 years. Within-group analysis of both groups, most paralyzed-side and symmetry measurements significantly improved over time with maintained significance at 3 years postoperatively in closed and open-mouth smile (all p ≤ 0.05). However, only the two-stage group had maintained significance in improvements at 3 years postoperatively in paralyzed-side and symmetry measurements in repose with commissure position (median change [interquartile range, IQR], 7.62 [6.00-10.56] mm), commissure angle (median change [IQR], 8.92 [6.18-13.69] degrees), commissure position symmetry (median change [IQR], -5.18 [-10.48 to -1.80] mm), commissure angle symmetry (median change [IQR], -9.78 [-11.73 to -7.32] degrees), and commissure height deviation (median change [IQR], -5.70 [-7.19 to -1.64] mm; all p ≤ 0.05). In the between-group analysis, all measurements were comparable in repose, closed-mouth smile, and open-mouth smile (all p > 0.05). CONCLUSION: Long-term outcomes demonstrate that both one- and two-stage dual-innervated gracilis FFMTs significantly improve excursion, but only two-stage reconstruction significantly improves resting tone.
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Paralisia Facial , Retalhos de Tecido Biológico , Músculo Grácil , Humanos , Paralisia Facial/cirurgia , Paralisia Facial/fisiopatologia , Masculino , Feminino , Músculo Grácil/transplante , Músculo Grácil/inervação , Adulto , Pessoa de Meia-Idade , Resultado do Tratamento , Retalhos de Tecido Biológico/inervação , Procedimentos de Cirurgia Plástica/métodos , Sorriso/fisiologia , Seguimentos , Estudos Retrospectivos , Adulto Jovem , IdosoRESUMO
To aid in the standardization of evaluating patients with multiple keloids, a Keloid Area and Severity Index (KASI) was developed using patient feedback, previous literature, and clinical expertise. The system was validated using intrarater and interrater reliability assessments. Here, we present a verified, reliable method of assessing keloid area and severity in clinical and research settings.
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Queloide , Humanos , Queloide/diagnóstico , Queloide/patologia , Reprodutibilidade dos TestesRESUMO
ABSTRACT: Women and minorities are underrepresented in leadership positions in academic specialties. We investigated representation of women and minorities in selected leadership positions and in fellowships in North American academic pediatric gastroenterology programs (PGP) via voluntary surveys. We also assessed for factors influencing diversity. We found that 45.8% of Program Directors (PD), 75% of division chiefs (DC), and 71% of Pediatrics department chairs were men. Sixty-three percentage of the PG fellows were women. Most DCs were male professors. Most PDs, DCs, and department chairs were White (70%, 80%, and 88.3%), with Blacks being the least represented group in leadership and also among fellows. We found a higher likelihood of having a White PD if the department chair was White. We found gender and racial disparities in all PGP leadership positions. This data can serve as a guide in efforts to support diversity for both gender and race at all positions and academic ranks.
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Gastroenterologia , Pediatria , Criança , Docentes de Medicina , Bolsas de Estudo , Feminino , Humanos , Liderança , Masculino , Estados UnidosRESUMO
OBJECTIVE: To address the current lack of specified data existing regarding the perioperative characteristics and outcomes in a novel patient population, which may bridge the current understanding of how patient characteristics and perioperative management may influence the postoperative hospital course before cardiac transplantation. DESIGN: A retrospective electronic chart review included all patients with failing single- ventricle (SV) physiology receiving ventricular assist device (VAD) support at a high-volume pediatric VAD center between April 5, 2010, and December 1, 2020, using institution-based electronic medical records for retrospective analysis. SETTING: At a single pediatric hospital. PARTICIPANTS: Fourteen pediatric patients with failing SV physiology receiving ventricular assist device therapy (SVAD). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Preoperative, intraoperative, and postoperative patient demographic and medical data were obtained from prior inpatient progress notes, laboratory values, anesthetic records, cardiac catheterization reports, echocardiography reports, and postoperative surgical notes entered during inpatient encounters at the time of SVAD placement. Between April 5, 2010, and December 1, 2020, 16 VAD device implants supported 14 pediatric patients with failing SV physiology. Most patients presented with a preoperative diagnosis of hypoplastic left heart syndrome (N = 9, 64.3%). A total of 6 patients expired on VAD therapy (43%), 7 (50%) survived to receive a cardiac transplant, and 1 patient currently remains on device therapy. CONCLUSION: Although our institutional approach represents a single perspective, we anticipate that our experience institutional experience may prove helpful to others caring for peditric patients with single ventricle physiology undergoing ventricular assist device placement and promote collaborative efforts to improve their care.
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Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Síndrome do Coração Esquerdo Hipoplásico , Humanos , Criança , Estudos Retrospectivos , Ecocardiografia , Insuficiência Cardíaca/cirurgia , Resultado do TratamentoRESUMO
BACKGROUND: Postparalytic synkinesis presents with a combination of hypo- and hypertonic muscles, leading to facial asynchrony with animation and at rest. One ubiquitous finding is a hypertonic depressor anguli oris (DAO) muscle and a weak depressor labii inferioris (DLI) muscle. The goal of this study was to evaluate the utility of DAO myectomy with or without its transfer to the weakened DLI in improving critical components of the dynamic smile. METHODS: From 2018 to 2020, this single-center, prospective study included of postparetic facial synkinetic patients with evidence of DAO hypertonicity who underwent DAO myectomy with or without transfer to DLI. Objective facial measurements were used to compare the effectiveness of DAO to DLI transfer to pure DAO myectomy in improving asymmetry of the synkinetic hemiface. RESULTS: Twenty-one patients with unilateral postparetic facial synkinesis with DAO hypertonicity were included; 11 underwent DAO myectomy, while 10 underwent DAO to DLI transfer. Baseline demographics and facial measurements were similar between the groups. DAO myectomy resulted in increased modiolus resting position, closed-mouth smile modiolus angle and excursion, open-mouth smile modiolus angle, excursion, dental show, and decreased lower lip height deviation. DAO to DLI transfer demonstrated similar findings but lacked significant increase in excursion and resulted in worsened lower lip height deviation. CONCLUSION: These findings illustrate the utility of DAO myectomy in improving imbalance in the synkinetic patient and necessitate further technical refinements for DAO transfers or a different approach for improving lower lip depression in this subgroup of patients.
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Paralisia Facial , Sincinesia , Músculos Faciais/cirurgia , Paralisia Facial/cirurgia , Humanos , Lábio , Estudos Prospectivos , Sincinesia/cirurgiaRESUMO
BACKGROUND/OBJECTIVE: We have limited knowledge regarding characteristics of patients with interstitial pneumonia with autoimmune features (IPAF) that are associated with response to immunosuppression. In this study, we used published IPAF criteria to characterize features associated with response to treatment. METHODS: We conducted a single-center medical records review study of 63 IPAF patients to evaluate for serological, clinical, and morphological characteristics that are associated with response to immunosuppression. Response was defined as % relative functional vital capacity decline of less than 10% and absence of death or lung transplant within the first year of continuous immunosuppressive therapy. Nonparametric measures of association and multivariate logistic regression were used to evaluate the relationship between baseline characteristics and immunosuppressive response. RESULTS: There was a trend of greater progression among men, ever smokers, those negative for antisynthetase antibodies, and those with usual interstitial pneumonia radiographic pattern, but no statistically significant relationship was found between baseline serological, clinical, or morphological features and response to immunosuppression. Patients on combination therapy with mycophenolate mofetil and prednisone had less disease progression (p = 0.018) than those on regimens that did not include both of these medications. CONCLUSIONS: In our cohort, baseline clinical assessment did not identify which patients with IPAF will respond to immunosuppressive therapy. Combination therapy with mycophenolate mofetil and prednisone was associated with lack of disease progression in our IPAF patients, including in IPAF-usual interstitial pneumonia. Further studies are needed to evaluate which IPAF patients would benefit from immunosuppressive therapy, antifibrotic therapy, or a combination of both.
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Doenças Autoimunes , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Humanos , Fibrose Pulmonar Idiopática/complicações , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Masculino , Ácido Micofenólico/uso terapêutico , Estudos RetrospectivosRESUMO
BACKGROUND: Inguinal hernia repair is the most common general surgery operation in the United States. Nearly 80% of inguinal hernia operations are performed under general anesthesia versus 15%-20% using local anesthesia, despite the absence of evidence for the superiority of the former. Although patients aged 65 y and older are expected to benefit from avoiding general anesthesia, this presumed benefit has not been adequately studied. We hypothesized that the benefits of local over general anesthesia for inguinal hernia repair would increase with age. MATERIALS AND METHODS: We analyzed 87,794 patients in the American College of Surgeons National Surgical Quality Improvement Project who had elective inguinal hernia repair under local or general anesthesia from 2014 to 2018, and we used propensity scores to adjust for known confounding. We compared postoperative complications, 30-day readmissions, and operative time for patients aged <55 y, 55-64 y, 65-74 y, and ≥75 y. RESULTS: Using local rather than general anesthesia was associated with a 0.6% reduction in postoperative complications in patients aged 75+ y (95% CI -0.11 to -1.13) but not in younger patients. Local anesthesia was associated with faster operative time (2.5 min - 4.7 min) in patients <75 y but not in patients aged 75+ y. Readmissions did not differ by anesthesia modality in any age group. Projected national cost savings for greater use of local anesthesia ranged from $9 million to $45 million annually. CONCLUSIONS: Surgeons should strongly consider using local anesthesia for inguinal hernia repair in older patients and in younger patients because it is associated with significantly reduced complications and substantial cost savings.
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Anestesia Geral/estatística & dados numéricos , Anestesia Local/estatística & dados numéricos , Hérnia Inguinal/cirurgia , Herniorrafia/estatística & dados numéricos , Complicações Pós-Operatórias/epidemiologia , Idoso , Anestesia Geral/efeitos adversos , Feminino , Herniorrafia/efeitos adversos , Herniorrafia/economia , Humanos , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Readmissão do Paciente/estatística & dados numéricos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Estudos Retrospectivos , Estados Unidos/epidemiologiaRESUMO
BACKGROUND: The optimal anesthesia modality for umbilical hernia repair is unclear. We hypothesized that using local rather than general anesthesia would be associated with improved outcomes, especially for frail patients. METHODS: We utilized the 1998-2018 Veterans Affairs Surgical Quality Improvement Program to identify patients who underwent elective, open umbilical hernia repair under general or local anesthesia. We used the Risk Analysis Index to measure frailty. Outcomes included complications and operative time. RESULTS: There were 4958 Veterans (13%) whose hernias were repaired under local anesthesia. Compared to general anesthesia, local was associated with a 12%-24% faster operative time for all patients, and an 86% lower (OR 0.14, 95%CI 0.03-0.72) complication rate for frail patients. CONCLUSIONS: Local anesthesia may reduce the operative time for all patients and complications for frail patients having umbilical hernia repair.
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Anestesia Geral/efeitos adversos , Anestesia Local , Fragilidade/complicações , Hérnia Umbilical/cirurgia , Herniorrafia/métodos , Saúde dos Veteranos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Bases de Dados Factuais , Procedimentos Cirúrgicos Eletivos/métodos , Feminino , Idoso Fragilizado , Hérnia Umbilical/complicações , Humanos , Modelos Lineares , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Many studies have identified racial disparities in healthcare, but few have described disparities in the use of anesthesia modalities. We examined racial disparities in the use of local versus general anesthesia for inguinal hernia repair. We hypothesized that African American and Hispanic patients would be less likely than Caucasians to receive local anesthesia for inguinal hernia repair. MATERIALS AND METHODS: We included 78,766 patients aged ≥ 18 years in the Veterans Affairs Surgical Quality Improvement Program database who underwent elective, unilateral, open inguinal hernia repair under general or local anesthesia from 1998-2018. We used multiple logistic regression to compare use of local versus general anesthesia and 30-day postoperative complications by race/ethnicity. RESULTS: In total, 17,892 (23%) patients received local anesthesia. Caucasian patients more frequently received local anesthesia (15,009; 24%), compared to African Americans (2353; 17%) and Hispanics (530; 19%), P < 0.05. After adjusting for covariates, we found that African Americans (OR 0.82, 95% CI 0.77-0.86) and Hispanics (OR 0.77, 95% CI 0.69-0.87) were significantly less likely to have hernia surgery under local anesthesia compared to Caucasians. Additionally, local anesthesia was associated with fewer postoperative complications for African American patients (OR 0.46, 95% CI 0.27-0.77). CONCLUSIONS: Although local anesthesia was associated with enhanced recovery for African American patients, they were less likely to have inguinal hernias repaired under local than Caucasians. Addressing this disparity requires a better understanding of how surgeons, anesthesiologists, and patient-related factors may affect the choice of anesthesia modality for hernia repair.
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Anestesia Local/estatística & dados numéricos , Etnicidade/estatística & dados numéricos , Disparidades em Assistência à Saúde/etnologia , Herniorrafia/estatística & dados numéricos , Complicações Pós-Operatórias/etnologia , Idoso , Feminino , Hérnia Inguinal/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Estudos Retrospectivos , Estados Unidos/epidemiologia , Veteranos/estatística & dados numéricosRESUMO
Increased circulating catecholamines are associated with worse exercise performance in adult heart failure patients. Patients with Fontan physiology have increased circulating catecholamines and theoretically could benefit from beta blockade. We hypothesized that carvedilol would improve exercise performance in Fontan patients. A double-blind, placebo-controlled, crossover trial of carvedilol was performed. Single ventricle patients between the ages of 10 and 35 years with a previous Fontan operation who were able to complete a maximal exercise test (respiratory exchange ratio > 1.0) were included. Two 12-week treatment arms were separated by a 6-week washout period. Exercise testing was performed at beginning and end of each treatment arm. Primary endpoint was improvement in peak oxygen consumption/kg (pVO2) from baseline. Of the 26 subjects enrolled, 23 completed the study. Four subjects did not reach goal maximum carvedilol dose, vs. 1 for placebo (p = 0.14). The mean change in pVO2 between treatments was not different (carvedilol = - 2.1 mL/kg/min v. placebo = - 1.42, p = 0.28). Carvedilol therapy decreased peak heart rate by 4.2 ± 20.2 bpm, (p < 0.01) leading to an increase in peak oxygen pulse (p < 0.01). Serum N-terminal-proBNP increased with carvedilol therapy (mean change of + 23.77 pg/mL) compared to placebo (mean change of - 5.37 pg/mL, p = 0.03). There were no serious adverse events related to study drug. Carvedilol was not associated with improved exercise performance and was associated with mildly increased N-terminal-proBNP. This study does not support the routine administration of carvedilol to healthy Fontan patients.Clinical Trials Registration ClinicalTrials.gov Identifier: NCT02946892. Registered October 27, 2016. Retrospectively Registered. https://clinicaltrials.gov/ct2/show/NCT02946892.
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Antagonistas Adrenérgicos beta/uso terapêutico , Carvedilol/uso terapêutico , Exercício Físico , Técnica de Fontan/métodos , Insuficiência Cardíaca/tratamento farmacológico , Adolescente , Adulto , Criança , Estudos Cross-Over , Método Duplo-Cego , Teste de Esforço/métodos , Feminino , Insuficiência Cardíaca/cirurgia , Frequência Cardíaca , Humanos , Masculino , Peptídeo Natriurético Encefálico/análise , Consumo de Oxigênio , Fragmentos de Peptídeos/análise , Resultado do Tratamento , Adulto JovemRESUMO
INTRODUCTION: Juvenile myasthenia gravis (JMG), a pediatric autoimmune neuromuscular junction disorder, includes generalized (GMG), and ocular (OMG) variants. We sought to determine whether differences existed between OMG and GMG children regarding demographics or treatment response. METHODS: We performed retrospective analysis of 60 children with JMG seen between 1990 and 2018. Osserman scores were used to define OMG and GMG. The myasthenia scale of Millichap and Dodge was used to assess treatment responses. RESULTS: There were no differences between GMG and OMG regarding time interval from disease onset to prednisone initiation (P = .42), or treatment response according to Millichap and Dodge (P = .12). Compared with GMG, OMG children showed younger age of disease onset and better outcomes after treatment. No OMG patients progressed to generalized disease during the follow-up period. DISCUSSION: Compared with GMG, OMG patients had earlier disease onset and improved outcomes after treatment.
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Miastenia Gravis/tratamento farmacológico , Adolescente , Idade de Início , Anti-Inflamatórios/uso terapêutico , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Lactente , Masculino , Miastenia Gravis/fisiopatologia , Músculos Oculomotores/fisiopatologia , Prednisona/uso terapêutico , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: To inform selective and efficient use of appendix ultrasound (US) beyond adult parameters of body mass index (BMI) of less than 25 kg/m, we correlate abdominal wall thickness (AWT) with age and BMI to generate parameters for male and female children. Information presented in chart format can aid in the decision to utilize US for the evaluation of appendicitis. METHODS: In this observational study, 1600 pediatric computed tomography scans of the abdomen and pelvis were analyzed to obtain measurements of AWT in the right lower quadrant. Measurements were correlated by patient age, BMI, and sex. Results and consensus-based recommendations were presented in chart format with color-coded groupings to allow for convenient referencing in the clinical setting. RESULTS: One thousand four hundred eighty-eight computed tomography scans and AWT measurements were included. All age groups with BMI of less than 25 kg/m and all male and female groups younger than 6 years regardless of BMI had median AWT of less than 4 cm resulting in strong recommendation for US. Males older than 6 years and all female age groups with BMI of greater than 30 kg/m and female older than 15 years and BMI of greater than 25 kg/m had AWT of more than 5 cm resulting in low recommendation for US. CONCLUSIONS: While the BMI cutoff standard of less than 25 kg/m for usefulness of appendix US holds in the adult population, our data expand the acceptable range in children younger than 9 years regardless of BMI and male children with BMI up to 30 kg/m. Female children younger than 15 years with a BMI up to 30 kg/m may also be amenable to right lower quadrant US based on AWT. These parameters inform selective and efficient use of US for appendix evaluation.
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Parede Abdominal/fisiologia , Apendicite/diagnóstico por imagem , Apêndice/diagnóstico por imagem , Adolescente , Índice de Massa Corporal , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Tomografia Computadorizada por Raios X , UltrassonografiaRESUMO
Background and Purpose- Predicting long-term functional outcomes after intracranial aneurysmal rupture can be challenging. We developed and validated a scoring system-the Southwestern Aneurysm Severity Index-that would predict functional outcomes at 1 year after clipping of ruptured aneurysms. Methods- Ruptured aneurysms treated microsurgically between 2000 and 2014 were included. Outcome was defined as Glasgow Outcome Score (ranging from 1, death, to 5, good recovery) at 1 year. The Southwestern Aneurysm Severity Index is composed of multiple prospectively recorded patient demographic, clinical, radiographic, and aneurysm-specific variables. Multivariable analyses were used to construct the best predictive models for patient outcomes in a random 50% of the cohort and validated in the remaining 50%. A scoring system was created using the best model. Results- We identified 527 eligible patients. The Glasgow Outcome Score at 1 year was 4 to 5 in 375 patients (71.2%). In the multivariable logistic regression, the best predictive model for unfavorable outcome included intracerebral hemorrhage (odds ratio [OR], 2.53; 95% CI, 1.55-4.13), aneurysmal size ≥20 mm (OR, 6.07; 95% CI, 1.92-19.2), intraventricular hemorrhage (OR, 2.56; 95% CI, 1.15-5.67), age >64 (OR, 3.53; 95% CI, 1.70-7.35), location (OR, 1.82; 95% CI, 1.10-3.03), and hydrocephalus (OR, 2.39; 95% CI, 1.07-5.35). The Southwestern Aneurysm Severity Index predicts Glasgow Outcome Score at 1 year with good discrimination (area under the receiver operating characteristic curve, derivation: 0.816, 95% CI, 0.759-0.873; validation: 0.803, 95% CI, 0.746-0.861) and accurate calibration ( R2=0.939). Conclusions- The Southwestern Aneurysm Severity Index has been internally validated to predict 1 year Glasgow Outcome Scores at initial presentation, thus optimizing patient or family counseling and possibly guiding therapeutic efforts.
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Aneurisma Roto/cirurgia , Aneurisma Intracraniano/cirurgia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Aneurisma Roto/diagnóstico por imagem , Aneurisma Roto/mortalidade , Ventrículos Cerebrais , Estudos de Coortes , Feminino , Escala de Resultado de Glasgow , Humanos , Hidrocefalia/complicações , Hidrocefalia/mortalidade , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/mortalidade , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: An operative biopsy is an important component in the diagnosis and treatment of neuromuscular disorders (NMDs). However, recent advances in molecular genetics suggest less invasive genetic testing should be the initial approach. The purpose of our study was to demonstrate the diagnostic value of muscle or nerve biopsy within the pediatric population at a pediatric academic center and offer recommendations for genetic testing in relation to biopsy to achieve the highest diagnostic yield. METHODS: Following institutional review board approval, we retrospectively reviewed the electronic medical record of 221 pediatric patients who underwent muscle and/or nerve biopsy for suspicion of NMD from January 2007 to March 2018. Demographics, family history, clinical presentations, genetic testing results, pathology results, anesthesia complications, clinical diagnoses, and clinic follow-up data were collected. Chi-square analysis was done for statistical significance. RESULTS: A total of 220 underwent muscle biopsy, and 15 underwent nerve biopsy. Not all patients received genetic testing. The average age at biopsy was 7.7 y. Biopsy revealed significant histologic abnormalities in 62.9% (139), directly leading to a specific clinical diagnosis in 33.9% (75). When genetic testing was done before biopsy, definite pathogenic variants were found in 7.6% (9). When genetic testing was done after biopsy, definite pathogenic variants were found in 45.0% (27). Genetic testing yield for pathogenic variants was higher when done after biopsy (P value < 0.00001). CONCLUSIONS: Muscle and nerve biopsies may provide significant diagnostic value. Biopsy helped to rule in or out NMD and guide genetic testing. Our data suggest NMD genetic testing yield was higher when done after biopsy.
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Testes Genéticos , Músculo Esquelético/patologia , Doenças Neuromusculares/diagnóstico , Nervos Periféricos/patologia , Biópsia , Criança , Feminino , Seguimentos , Humanos , Masculino , Doenças Neuromusculares/genética , Doenças Neuromusculares/patologia , Estudos RetrospectivosRESUMO
BACKGROUND: The purpose of this prospective, randomized, controlled trial was to determine whether multiple ports improve the analgesic efficacy of wire-reinforced flexible catheters used for labor epidural analgesia (LEA). METHODS: Six hundred fifty laboring patients were randomized to receive epidural analgesia using either a multiport or uniport wire-reinforced flexible catheter. The primary outcome was analgesic success, defined as the incidence of adequate analgesia following the initial bolus given to initiate LEA. Secondary outcomes included the number of patients requiring clinician interventions during maintenance of LEA; anesthetic success, defined as the incidence of adequate anesthesia following the initial bolus given to establish surgical anesthesia for cesarean delivery; and maternal satisfaction with the overall quality of LEA. RESULTS: There was no significant difference in analgesic success at initiation of LEA between the uniport and the multiport wire-reinforced flexible catheter (93.6% vs 89.5%, respectively; difference of 4.1% [95% confidence interval, -0.4% to 8.5%]; P = .077). There was also no difference in the number of patients requiring clinician interventions during maintenance of LEA and in anesthetic success at the establishment of surgical anesthesia for cesarean delivery between the 2 catheter types. CONCLUSIONS: Multiple ports do not appear to improve the analgesic efficacy of wire-reinforced flexible catheters used for LEA.