Introduction. X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by agammaglobulinemia requiring replacement treatment with immunoglobulin. The association of XLA and membranoproliferative glomerulonephritis (MPGN) is unexpected and, to our knowledge, only one case was previously published. Case Report. The authors report the case of a 10-year-old boy with family history and prenatal diagnosis of XLA, treated from birth with intravenous immunoglobulin replacement therapy. He presented with pneumonia, macroscopic hematuria, nephrotic proteinuria, hypoalbuminemia, and hypercholesterolemia with normal renal function and serum complement levels. Renal histology showed immune complex mediated MPGN. He was started on high dose prednisolone and ramipril and switched to weekly subcutaneous immunoglobulin. After a 4-month treatment, hematuria and proteinuria significantly improved and prednisolone was gradually tapered without relapse. Conclusion. The pathogenic process underlying MPGN development in this patient is unknown but residual humoral immunity might play an important role. Thus, this case highlights the risk of autoimmune disorders among patients with XLA.

Discussão: Na nossa revisão a taxa de sucesso terapêutico encontra-se dentro do esperado, com uma percentagem de falência inferior ao descrito na literatura. Apresentamos a instilação intrapleural de fibrinolítico e a toracoscopia videoassistida num mesmo protocolo de atuação, tendo como primeira linha terapêutica o fibrinolítico. Conclusão: A opção terapêutica apresentada teve uma baixa taxa de falência e permitiu evitar um procedimento cirúrgico mais agressivo. Consideramos que esta é uma opção de tratamento eficaz e que, na nossa amostra, cursou com uma baixa taxa de sequelas. Palavras-chave: Derrame Pleural; Empiema Pleural; Fibrinolíticos. ABSTRACT Introduction:The treatment of complicated pleural parapneumonic effusions with intrapleural instillation of fibrinolytics, has shown similar results as surgical treatment. The present study aimed to evaluate the results of the use of intrapleural instillation of fibrinolytics in the treatment of complicated pleural parapneumonic effusions, in patients followed in our hospital. Material and Methods:A retrospective review of all the patients (aged between one month and 18 years) diagnosed with complicated parapneumonic effusions, which had chest drain insertion with intrapleural instillation of fibrinolytic, between January 2005 and December 2013, was undertaken.Results: A total of 37 patients were identified. Mean duration of hospital stay was 17 ± 7.60 days. Chest drain was placed in the first 48 h of hospital admission in most of the patients (89.2%), with a mean of six days of drainage. Treatment failure was reported in 2.7% of cases and was related with effusion recurrence. This patient underwent video-assisted thoracoscopic surgery with the need to convert to open thoracotomy. A favorable outcome was achieved in 96.9 % of cases. Discussion: In our review, therapeutic success rate was as expected, with a failure rate below those reported in literature. We present intrapleural instillation of fibrinolytics and video-assisted thoracoscopic surgery as part of the same protocol, in which fibrinolytic therapy is the first-line treatment. Conclusion:The therapeutic option presented shows a low failure rate and avoids a more aggressive surgical procedure. We consider this an effective treatment option, with low sequelae rate.

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