title: [Acid sphingomyelinase deficiency and spleen trauma: splenectomy or not splenectomy?].
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  Despite its theoretical contra-indication in Niemann-Pick disease due to a risk of respiratory insufficiency, splenectomy must sometimes be considered.
authors.affiliation: Service de médecine interne-rhumatologie, hôpital de la Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France. Electronic address: olidove@hopital-dcss.org.
authors.forename: O
authors.initials: O
authors.lastname: Lidove
authors.affiliation: Service de médecine interne-rhumatologie, hôpital de la Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Le Fèvre
authors.affiliation: Service de chirurgie viscérale, hôpital de la Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.
authors.forename: N
authors.initials: N
authors.lastname: Goasguen
authors.affiliation: Service d'anatomopathologie, hôpital Saint-Antoine, 184, rue du Faubourg-Saint-Antoine, 75571 Paris cedex 12, France.
authors.forename: M
authors.initials: M
authors.lastname: Jamali
authors.affiliation: Service de médecine nucléaire, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Vercellino
authors.affiliation: Service de réanimation, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France.
authors.forename: M
authors.initials: M
authors.lastname: Garnier
authors.affiliation: Service de médecine interne, hôpital Henri-Mondor, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94000 Créteil, France.
authors.forename: M
authors.initials: M
authors.lastname: Khellaf
authors.affiliation: Service de médecine interne, hôpital Beaujon, 100, boulevard du Général-Leclerc, 92110 Clichy, France.
authors.forename: N
authors.initials: N
authors.lastname: Belmatoug
authors.affiliation: Service de médecine interne-rhumatologie, hôpital de la Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.
authors.forename: J-M
authors.initials: JM
authors.lastname: Ziza
mesh_terms: D004621:Embolization, Therapeutic; D006801:Humans; D008297:Male; D008875:Middle Aged; D052537:Niemann-Pick Disease, Type B / Q000150:complications* / Q000628:therapy*; D013154:Spleen / Q000293:injuries* / Q000601:surgery; D013156:Splenectomy / Q000706:statistics & numerical data*; D013161:Splenic Rupture / Q000150:complications / Q000628:therapy*; D013163:Splenomegaly / Q000150:complications / Q000601:surgery
keywords: Acid sphingomyelinase deficiency; Maladie de Niemann-Pick B; Sphingomyélinase acide; Splenectomy; Splénectomie