Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report 2 cases of hepatocellular tumour in children treated with anabolic androgens for aplastic anemia. In both cases, the presentation was by a picture of acute abdomen due to hemoperitoneum caused by tumour rupture. In the first case, there was multiple hepatic adenomas necessitating right hepatic lobectomy. The second infant had a single tumour of segment IV treated by simple excision of the tumour. It was a hepatocellular-carcinoma. Follow-up for one year after the initial operation showed no signs of recurrence in both infants. The review of the literature permitted us to find 48 other cases of hepatocellular tumour secondary to androgen therapy. In order of frequency, the hepatocellular-carcinoma is the most frequent and it is usually single; followed by the adenoma which is usually multiple. The other types of tumours are rare: focal nodular hyperplasia, angiosarcoma and cholangiocarcinoma. The hepatocellular-carcinoma and adenoma have some characteristic features: spontaneous regression may occur after withdrawing of androgens; the risk of rupture is important; their evolution is almost always favorable despite of a severe histopathological picture; the alpha-foeto-protein is nearly always negative; and the metastasis are exceptional. The hepatocellular-carcinomas associated with androgen therapy are probably just adenomas with marked dysplasia, but their long term malignant potential remain unknown. Except in case of rupture, surgical intervention should be postponed until the effect of discontinuing the hormonal therapy is assessed, because of the potential for spontaneous regression. The administration of antineoplastic chemotherapeutic agents should be reserved for the tumours showing evidence of malignancy.
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PMID:[Tumors of the liver secondary to androgen therapy. Apropos of 2 cases in children]. 304 Feb 81

From 1976 through 1982, 35 children with leukemia (27), aplastic anemia (4), and solid tumors (4), were evaluated for symptoms and signs of acute abdominal disease. Twenty-six patients required 34 operations, 14 of them for life-threatening events. Sixteen patients received a bone marrow transplant. The most common surgical emergencies were for acute massive Gl hemorrhage (10), biliary tract disease (4), and typhlitis (3). Thirteen (37%) of the children are alive and free of disease, 22 (63%) have died, 13 of them as a direct result of the abdominal complication which occurred. Alterations in the immune system and hematopoietic system caused by the malignant disease and its treatment with chemotherapy and irradiation appears to be responsible for a number of abdominal complications which the surgeon is asked to see. When symptoms and signs of an acute abdomen develop in patients with leukemia or solid tumors, prompt, thorough evaluation and early aggressive surgical treatment is needed if the patient is to survive. The surgeon should be aware of the unusual array of abdominal complications which can occur in this population of patients.
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PMID:The acute abdomen in the immunologically compromised child. 636 69