Severe axillary lymphadenitis after BCG vaccination:alert for primary immunodeficiencies

The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a partial recessive interferon-γ receptor 1 deficiency, who developed BCG dissemination; and two relatives with ZAP70 deficiency, a severe combined immunodeficiency, both of whom presented with regional and distant BCG disease. All had severe axillary lymphadenitis. These clinical cases underline the importance of considering the diagnosis of immunodeficiency in a child with severe axillary lymphadenitis after BCG vaccination and of disseminated BCG disease in an immunodeficient child in the appropriate clinical setting. Moreover, BCG vaccination should be delayed in every newborn with a family history of primary immunodeficiency until the condition has been ruled out

Válvulas da Uretra Posterior: Experiência de um Hospital Nível III

Introduction: The posterior urethral valves (PUV) are an important cause of congenital urinary tract obstruction affecting male children. Our aim in this study was to characterize the evolution of a children group with PUV with identification of possible risk factors to chronic kidney disease (CKD). Methods: Retrospective analysis of PUV children diagnosed between January 1999 and January 2017 in a level III hospital. Data collected included: clinical, analytical and imagiological findings, treatment and evolution. Statistical tests were realized using: Excel 2016® e Epi Info 7®, with p < 0.05 considered significant. Results: During the studied period, we identified 23 cases of VUP. The diagnosis was prenatal in 16 and four of seven were diagnosed before age one. The clinical presentation was: acute renal failure (n= 11), isolated urinary tract infections (UTI) (n= 4) and poor urinary stream (n= 3). Median creatinine was 151 μmol/L. All 23 children presented renal and upper urinary tract dilatation, (bilateral in 22), 21 had parenchymal changes and 19 had thickened wall bladder. Cistouretrography confirmed the diagnosis and revealed vesicoureteral reflux (VUR) in 15 cases, bilateral in 10. The main treatment was valve ablation by endoscopic fulguration, performed in all cases, one of them with a previous vesicostomy. The mean follow-up was 7.5 years. During that time, 14 presented recurrent UTI’s, seven remained with VUR and 11 with bladder dysfunction. Some needed other surgical procedures, like VUR correction (n= 3) and unilateral nephrectomy (n= 2). Five cases (21.5%) presented CKD stage ≥ 3, with four cases needing dialysis around 9.3 years of mean age. The recurrent UTI’s and bilateral VUR were associated with evolution to that stage (p 0.03 and p 0.049 respectively). Conclusion: PUV remains a frequent aetiology of CKD, despite timely and accurate diagnosis and treatment.Introdução: As válvulas da uretra posterior (VUP) são uma causa importante de obstrução congénita do trato urinário no sexo masculino, com repercussão na função vesical e renal. Neste estudo pretendeu-se caracterizar a clínica, tratamento, evolução e identificação de possíveis fatores de risco para doença renal crónica (DRC) de um grupo de crianças com VUP. Métodos: Análise retrospetiva dos processos clínicos de crianças seguidas na Consulta de Nefrologia num hospital nível III entre janeiro 1999 e janeiro 2017. Variáveis analisadas: diagnóstico pré-natal, apresentação clínica, avaliação analítica e imagiológica, tratamento e evolução. Para análise estatística utilizou-se: Excel 2016® e Epi Info 7®, considerando-se significativo p < 0,05. Resultados: Dos 23 casos de VUP incluídos no estudo, o diagnóstico foi sugerido por ecografia pré-natal em 16 e confirmado após nascimento, quatro tiveram diagnóstico no primeiro ano de vida e três depois dessa idade. As manifestações clínicas iniciais foram: lesão renal aguda (LRA) (n= 11), pielonefrite aguda (PNA) (n= 4) e alterações do jato urinário (n= 3). A mediana da creatinina (Cr) inicial foi 151 μmol/L. Todos apresentavam hidronefrose na ecografia pós-natal (bilateral em 22) 21 tinham alterações do parênquima renal e 19 espessamento parietal vesical. O diagnóstico foi realizado por cistografia radiológica, que também mostrou refluxo vesico-uretral (RVU) em 15 casos, bilateral em 10. Todas as crianças foram submetidas a fulguração, uma com vesicostomia prévia. O tempo médio de seguimento foi 7,5 anos, durante o qual 14 mantiveram PNA de repetição, sete RVU e 11 cursaram com disfunção vesical. Alguns casos necessitaram de outras intervenções cirúrgicas, que incluíram correção de RVU (n= 3) e nefrectomia unilateral (n= 2). Cinco (21,7%) evoluíram para DRC estadio ≥ 3, dos quais quatro iniciaram diálise com idade média de 9,3 anos. As PNA de repetição e o RVU bilateral associaram-se a evolução para o referido estadio (p 0,03 e p 0,049 respetivamente). Conclusão: As VUP continuam a ser uma etiologia frequente da DRC na criança, apesar do diagnóstico e tratamento cada vez mais precoces

Disparities in treatment and outcome of kidney replacement therapy in children with comorbidities : an ESPN/ERA Registry study

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