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[Interstitial pneumopathy revealing type B Niemann Pick disease in an adult].
- Source :
-
La Revue de medecine interne [Rev Med Interne] 1999 Jul; Vol. 20 (7), pp. 597-601. - Publication Year :
- 1999
-
Abstract
- Introduction: Niemann-Pick disease is an autosomal recessive disorder due to partial or total deficit in sphingomyelinase.<br />Exegesis: We report a case of type B Niemann-Pick disease revealed by pneumonia and splenomegaly associated with blue histiocyte syndrome. Investigations that were done 2 years prior to diagnosis had shown the existence of isolated chronic lipid pneumonia which is specific of overloading.<br />Conclusion: Though diagnosis is based on biochemistry, the morphology of alveolar histiocytes after simple bronchoalveolar lavage is of value.
Details
- Language :
- French
- ISSN :
- 0248-8663
- Volume :
- 20
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- La Revue de medecine interne
- Publication Type :
- Academic Journal
- Accession number :
- 10434350
- Full Text :
- https://doi.org/10.1016/s0248-8663(99)80109-x