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 |
Externí odkaz: |
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