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OBJETIVO: correlacionar os dados clínicos do óbito com achados de necropsia em pacientes internados em uma unidade de reumatologia pediátrica. MÉTODOS: o estudo é uma coorte histórica. Em dez anos, no período compreendido entre janeiro de 1994 e dezembro de 2003, ocorreram 57.159 internações com 1.907 (3%) óbitos no Instituto da Criança. Destas internações, 548 (1%) apresentaram doenças reumáticas ou pediátricas e foram acompanhadas pela Unidade de Reumatologia Pediátrica, incluindo 348 pacientes. Os óbitos e as necropsias foram analisados e a classificação de Goldman foi utilizada para detectar discordância entre o diagnóstico clínico do óbito e a necropsia. RESULTADOS: no período do estudo, 34 (10%) pacientes evoluíram para óbito. As principais doenças associadas ao óbito foram: lúpus eritematoso sistêmico juvenil (LESJ) em 18 pacientes (53%) e artrite idiopática juvenil (AIJ) em sete casos (21%). Necropsias foram realizadas em 21 pacientes (64% dos óbitos). Nos 18 casos de LESJ, a atividade da doença esteve presente em 16 casos, sendo associada à septicemia em 15 deles. Discordância entre o diagnóstico clínico e necropsia foi evidenciada em seis casos com LESJ: três com infecções fúngicas, um com tuberculose, um com nefrite proliferativa difusa e outro com aterosclerose. Sete pacientes com AIJ evoluíram para óbito: septicemia em quatro e síndrome de ativação macrofágica em três. Em um caso de AIJ foi diagnosticado, exclusivamente na necropsia: linfoma de Hodgkin, aterosclerose e infarto anterior do miocárdio. CONCLUSÕES: a freqüência de óbitos foi 10% e necropsia evidenciou doença infecciosa, aterosclerose ou neoplasia não diagnosticadas previamente. A necropsia é importante para determinar eventos não esclarecidos ou duvidosos no óbito e deve ser sempre solicitada.OBJECTIVE: to correlate the clinical data of necropsies in hospitalized patients of a Pediatric Rheumatology Unit. METHODS: this study is a historic cohort. In ten years, from January 1994 to December 2003, there were 57,159 hospitalizations with 1,907 (3%) deaths in Instituto da Criança. From these hospitalizations, 548 (1%) presented rheumatic or pediatric diseases and were followed by the Pediatric Rheumatology Unit, involving 348 patients. The deaths and necropsies were analyzed and the Goldman Classification was used to detect any disagreement between the clinic diagnosis and the necropsy. RESULTS: over the period of this study, 34 (10%) of patients died. The main diseases that caused deaths were: juvenile systemic erithematosus lupus (JSLE) in 18 patients (53%) and juvenile idiopathic arthritis (JIA) in 7 cases (21%). Necropsies were done in 21 patients (64% of deaths). In 18 cases of JSLE, disease activity was present in 16 cases, being associated to septicemia in 15. Differences between the clinical diagnosis and necropsy were observed in 6 cases of JSLE: 3 with fungal infection, 1 with tuberculosis, 1 with diffuse proliferative nephritis and 1 with atherosclerosis. Seven patients with JIA died: septicemia in 4 and macrophage activation syndrome in 3. In 1 case of JIA were detected only by necropsy: Hodgkin lymphoma, atherosclerosis and anterior myocardial infarction. CONCLUSIONS: the frequency of deaths was 10% and necropsy showed infection disease, atherosclerosis or malignancy not detected previously. Necropsy is important to determine events not detected or doubtful in death patients and must always be requested.

Objective: To evaluate liver and spleen dimensions in childhood-onset systemic lupus erythematosus (c-SLE) patients and healthy controls., Methods: 30 c-SLE patients and 30 healthy control volunteers underwent abdominal ultrasound. The following two liver measurements were performed in left hepatic lobe: craniocaudal and anteroposterior and three in right hepatic lobe (RHL): posterior craniocaudal (PCC-RHL), anterior craniocaudal and anteroposterior. Three spleen dimension measurements were also evaluated: longitudinal, transverse and anteroposterior. Demographic, clinical and laboratorial data, SLEDAI-2K, ECLAM, SLAM and treatment were assessed., Results: Mean current age was similar in c-SLE and controls (170.31 ± 27.81 vs. 164.15 ± 39.25 months; p = 0.486). The mean of PCC-RHL dimension was significantly higher in c-SLE compared to controls (13.30 ± 1.85 vs. 12.52 ± 0.93, p = 0.044). There were no differences between the other hepatic biometrics and splenic parameters (p > 0.05). Further analysis in c-SLE patients according to PCC-RHL dimension ≥ 13.3cm versus < 13.3 cm showed that the median of SLEDAI-2K [8(0-18) vs. 2(0-8), p=0.004], ECLAM [4(0-9) vs. 2(0-5), p = 0.019] and SLAM [5(1-13) vs. 2(0-14), p = 0.016] were significantly higher in patients with higher PCC-RHL dimension, likewise the frequencie of nephritis (77% vs. 29%, p = 0.010). Liver enzymes were similar in both groups (p > 0.05). Positive correlation was observed between SLEDAI-2K and PCC-RHL (p = 0.001, r = +0.595). Negative correlation was evidenced between disease duration and longitudinal dimension of spleen (p = 0.031, r = -0.394)., Conclusion: Our data raises the possibility that disease activity could lead to a subclinical and localized hepatomegaly during the disease course. Long disease duration resulted to spleen atrophy in c-SLE patients., (Copyright © 2015 Elsevier Editora Ltda. All rights reserved.)