Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Repeated blood cultures were negative in a six-year-old boy with high septic temperature. Rheumatoid arthritis, typhoid and brucellosis were excluded. There was no evidence of leukaemia (bone-marrow tests). There was hepatomegaly with increased transaminase activity. Cortisone treatment was begun for suspected collagen disease. Several liver needle biopsies at first revelaed increasingly severe necrotising changes, predominantly in the centres of the acini. There were no parasites demonstrable histologically. The spleen was increased in the isotope scan but, covered by the liver, not definitely palpable. Diagnosis of mediterranean Kala-Azar disease was then suspected (the parents reporting a camping holiday in Yugoslavia) and finally proven serologically. The previously treatment-resistant disease improved dramatically after administration of trivalent antimony (Fuadin).
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PMID:[Visceral kala-azar disease in a child (author's transl)]. 56 81

A fourteen month old infant was admitted for evaluation because of continuous high fever and an indurated nodular lesion at the left thigh of one month course. After admittance painful inflammatory subcutaneous nodules appeared in the face and trunk, these were accompanied by enlarged cervical lymph nodes and hepatomegaly. Histological evaluation of the skin biopsy showed destruction of subcutaneous tissue, foamy cells, vasculitis and polymorphonuclear leukocyte infiltration; histiocytic proliferation in the lymph nodes and steatosis in the liver biopsy. Osteoarticular infection, cellulitis, sepsis, tuberculosis, collagen disease, and malignancies of hematologic origin were all ruled out. Response to treatment with prednisone was excellent and the patient has been asymptomatic along a one year follow up period.
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PMID:[Weber-Christian disease]. 184 29

A case of cardiac amyloidosis in a 46-year-old male is reported. He was admitted for dyspnea. Physical examination revealed third and forth heart sound and hepatomegaly. Radiographic heart-thoracic ratio was 53%. Electrocardiogram showed first degree A-V block, rS pattern in V1-V4 leads, and ambulatory electrocardiogram showed ventricular tachycardia. Echocardiogram revealed hypertrophy and highly refractile echoes of the left ventricular wall. Endomyocardial biopsy was performed and it demonstrated amyloid fibrils, which were characterized immunohistochemically as Amyloid A (AA) protein, which is generally a constituent in secondary amyloidosis. Urine protein electrophoresis showed lambda type Bence-Jones protein, but bone marrow biopsy was normal. There was no evidence of malignancy, chronic inflammatory disease, or collagen disease. This case was diagnosed as primary amyloidosis with AA protein. It is rare that, in spite of its being a case of primary amyloidosis, its constituent protein is AA protein.
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PMID:[A case of primary cardiac amyloidosis with amyloid A protein]. 843 66