title: Haemorrhagic pericardial effusion in type I Gaucher's disease.
abstract: Gaucher's disease is a rare lysosomal storage disorder. Excess accumulation of glucosylceramide predominantly occurs in abdominal viscera. Cardiac involvement is rare, though they generally represent as restrictive cardiomyopathy and pericarditis. Our case, a 19-year old boy initially presented with hepatosplenomegaly but later diagnosed with haemorrhagic pericardial effusion. Enzyme supplementation gives successful outcome but our case was managed symptomatically with pericardiocentesis.
authors: R N
authors: RN
authors: Sarkar
authors: Sibaji
authors: S
authors: Phaujdar
authors: Kuntal
authors: K
authors: Bhattacharyya
authors: Dibyendu
authors: D
authors: De
authors: Hare Krishna
authors: HK
authors: Pal
authors: Omar Sharif
authors: OS
authors: Mullick
authors: Santosh Kumar
authors: SK
authors: Dey
mesh_terms: D005776:Gaucher Disease / Q000150:complications* / Q000175:diagnosis; D006470:Hemorrhage / Q000209:etiology* / Q000628:therapy; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D008297:Male; D010490:Pericardial Effusion / Q000209:etiology* / Q000628:therapy; D020519:Pericardiocentesis; D013163:Splenomegaly / Q000209:etiology; D055815:Young Adult