title: A case of Gaucher's disease progressing to liver cirrhosis.
abstract: We are going to present a 17 year old female with Gaucher's disease. The patient presented with fever, cough, respiratory distress & abdominal heaviness. There was mild pallor, redness of palm of hands & raised temperature. Liver was hugely enlarged along with splenomegaly. X-ray chest showed non specific bronchiectatic change in both lungs. Ultrasonography of abdomen revealed marked hepatosplenomegaly with no ascites. Bone marrow examination showed cellular marrow with plenty of megakaryocytes. Most of the cells were smear cells & there was histiocytes proliferation & infiltration of bone marrow by small atypical cells. Histologically, lipid was found in hepatocytes in moderate amount. The portal areas showed high lipid contents in macrophages. Different clinical findings & incidental diagnosis of lipid storage disease submerged us in diagnostic dilemma. We give conservative treatment with antibiotic cefuroxime, syrup lactulose & vitamins and this patient was improved.
authors.affiliation: Department of Hepatology, Mymensingh Medical College, Mymensingh, Bangladesh.
authors.forename: C R
authors.initials: CR
authors.lastname: Debnath
authors.forename: M R
authors.initials: MR
authors.lastname: Debnath
authors.forename: N
authors.initials: N
authors.lastname: Nabi
authors.forename: N A
authors.initials: NA
authors.lastname: Khan
authors.forename: S
authors.initials: S
authors.lastname: Chakraborty
mesh_terms: D000293:Adolescent; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002444:Cefuroxime / Q000627:therapeutic use; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D005776:Gaucher Disease / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007792:Lactulose / Q000627:therapeutic use; D008103:Liver Cirrhosis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D014815:Vitamins / Q000627:therapeutic use
chemical_list: D000900:Anti-Bacterial Agents; D014815:Vitamins; D007792:Lactulose; D002444:Cefuroxime