Browsing by Author "Lavrador, V."
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- Eritroblastopenia transitória da infância: atitude expectante face a uma anemia gravePublication . Pereira, M.; Bergantim, R.; Costa, E.; Morais, L.; Lavrador, V.; Barbot, J.Introdução: A eritroblastopenia transitória da infância (ETI) é uma condição aguda caracterizada por anemia moderada a grave e reticulocitopenia, secundárias a uma supressão temporária da eritropoiese. A etiologia é desconhecida, embora ocorra associação com uma infecção vírica em cerca de metade dos casos. Tipicamente, apresenta um curso benigno e auto-limitado, necessitando apenas de vigilância da evolução clínica. Caso clínico: Descreve-se o caso clínico de uma menina com três anos de idade, internada por infecção respiratória alta e ETI. O seu quadro hematológico era caracterizado por anemia com hemoglobina de 7.1 g/dl e reticulocitopenia, sem alterações nas outras linhas celulares. A evolução foi favorável, com recuperação espontânea ao fim de três semanas. Conclusão: O reconhecimento desta entidade hematológica benigna, distinguindo-a de outras formas de anemia arregenerativa, pode evitar o recurso a procedimentos diagnósticos e terapêuticos desnecessários. ABSTRACT Introduction: Transient erythroblastopenia of childhood (TEC) is an acquired, acute disorder, characterized by moderate to severe anemia and reticulocytopenia, secondary to a temporary suppression of red blood cell production. The etiology of TEC remains unknown, although an association with viral infections has been proposed. It is self-resolving and careful observation is the only medical support needed in most cases. Case report: The authors describe the case of a three-year-old girl who was admitted with an upper respiratory tract infection and TEC. Initial signs were anemia, with a 7.1 g/dl hemoglobin level and reticulocytopenia, without another hematologic abnormality. Spontaneous recovery occurred after three weeks. Conclusion: A better knowledge of this benign hematologic disorder of childhood, distinguishing TEC from other causes of non-regenerative anemia, can prevent unnecessary diagnostic procedures and treatment.
- Membranoproliferative glomerulonephritis and x-linked agammaglobulinemia: an uncommon associationPublication . Lavrador, V.; Correia, F.; Sampaio, R.; Candido, C.; Sameiro-Faria, M.; Marques, L.; Mota, C.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