[Acid sphingomyelinase deficiency and spleen trauma: splenectomy or not splenectomy?]. / Déficit en sphingomyélinase acide et rupture splénique : splénectomie ou traitement conservateur ?
Rev Med Interne
; 36(9): 619-22, 2015 Sep.
Article
em Fr
| MEDLINE
| ID: mdl-25479761
ABSTRACT
INTRODUCTION:
Acid sphingomyelinase deficiency leads to a severe infantile disease (Niemann-Pick disease type A) or an attenuated form of the disease encountered in adults (Niemann-Pick type B), including pulmonary fibrosis and splenomegaly. CASE REPORT A 52-year-old man with Niemann-Pick disease type B was admitted with splenic rupture. Embolization of the splenic artery was initially performed. Three months later, the splenic volume had increased and functional asplenia was diagnosed. Splenic scintigraphy showed 20% of functional splenic tissue. Splenectomy was finally performed because of complete necrosis of the spleen.CONCLUSION:
Despite its theoretical contra-indication in Niemann-Pick disease due to a risk of respiratory insufficiency, splenectomy must sometimes be considered.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Baço
/
Esplenectomia
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Ruptura Esplênica
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Doença de Niemann-Pick Tipo B
Idioma:
Fr
Ano de publicação:
2015
Tipo de documento:
Article