Cirrhosis and portal hypertension in a patient with adult Niemann-Pick disease
| Autor: | Joseph P. Tassoni, David E. Johnston, Karim A. Fawaz |
|---|---|
| Rok vydání: | 1991 |
| Předmět: |
Adult
Liver Cirrhosis congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Cirrhosis medicine.medical_treatment Splenectomy Peripheral edema Hepatosplenomegaly Gastroenterology hemic and lymphatic diseases Internal medicine Hypertension Portal Ascites Humans Medicine Niemann-Pick Diseases Hepatology business.industry nutritional and metabolic diseases medicine.disease Portal hypertension Female Upper gastrointestinal bleeding medicine.symptom business Niemann–Pick disease |
| Zdroj: | Gastroenterology. 100:567-569 |
| ISSN: | 0016-5085 |
| DOI: | 10.1016/0016-5085(91)90233-b |
| Popis: | A woman with known Niemann-Pick disease, type B, presented at age 33 with upper gastrointestinal bleeding, ascites, and peripheral edema. Evaluation showed massive hepatosplenomegaly, infiltration of the liver with Niemann-Pick cells, cirrhosis, and evidence of portal hypertension. Chronic gastrointestinal bleeding, thrombocyctopenia, and platelet dysfunction were treated successfully by splenectomy. Cirrhosis and portal hypertension have not been reported previously in adult Niemann-Pick disease in the absence of some other cause. |
| Databáze: | OpenAIRE |
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