Haemorrhagic pericardial effusion in type I Gaucher's disease

Autor: R N, Sarkar, Sibaji, Phaujdar, Kuntal, Bhattacharyya, Dibyendu, De, Hare Krishna, Pal, Omar Sharif, Mullick, Santosh Kumar, Dey
Rok vydání: 2014
Předmět:
Zdroj: The Journal of the Association of Physicians of India. 61(11)
ISSN: 0004-5772
Popis: 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.
Databáze: OpenAIRE