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)

We report on three children aged 1 1/2, 2 and 9 1/2 years with Wilms' tumor, who developed a tender hepatomegaly and ascites associated with elevated liver enzymes, anemia and thrombocytopenia during chemotherapy. This clinical picture and liver sonography abnormality are best explained by veno-occlusive disease (VOD) of the liver, while other causes of liver disease could not be identified. Actinomycin D dosage was 0.045 mg/kg as bolus injection in two patients and 0.075 mg/kg split over five days in a third patient. Presumable, this drug was the causative agent. VOD was observed after preoperative and postoperative chemotherapy. No child had received abdominal irradiation. The authors comment on the influence of Actinomycin D as the cause for this unusual liver toxicity.
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PMID:[Veno-occlusive disease of the liver as a treatment complication in children with Wilm's tumor]. 131 36

We have observed hepatopathy, associated with thrombocytopenia, in children receiving chemotherapy for Wilms' tumor. We have studied this hepatopathy-thrombocytopenia syndrome (HTS) in patients enrolled in the United Kingdom Childrens' Cancer Study Group (UKCCSG) Wilms' tumor trials (UKW1 and UKW2). At the time of this study, 501 patients had completed therapy. Treatment flow sheets were examined for evidence of hepatopathy (hepatomegaly with abnormal liver function tests) and severe thrombocytopenia (platelet count less than 25 x 10(9)/L). No child who developed the syndrome had received irradiation. HTS was seen in five of 355 (1.4%) of patients treated with combination chemotherapy but in none of the 146 patients treated with vincristine alone. In each instance, the onset was less than 10 weeks after diagnosis. In two children, hepatopathy was severe with jaundice, ascites, transaminases greater than 1,000 IU/L, and prolongation of prothrombin time. On average, HTS lasted 12 days, and resolved with supportive treatment. After recovery, the children tolerated chemotherapy, mostly at reduced dosage, without recurrence. There was no evident long-term morbidity. Dactinomycin is the probable cause of this syndrome. We conclude that the HTS is a rare but important complication of dactinomycin-containing combination chemotherapy for Wilms' tumor. Children developing "isolated" thrombocytopenia following dactinomycin are "at risk" of developing the full-blown syndrome and should have their treatment modified accordingly.
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PMID:Hepatopathy-thrombocytopenia syndrome--a complication of dactinomycin therapy for Wilms' tumor: a report from the United Kingdom Childrens Cancer Study Group. 184 5

Metastatic malignant teratoma of the testis is associated with early death for most afflicted patients. Chemotherapy has limited success with this disease. Medroxyprogesterone acetate (MPA) has been known to exert an antitumor effect in cancers of the endometrium, breast and kidney. This paper documents the objective improvement in 3 of 16 patients with metastatic testicular teratoma treated with MPA. The patients were aged 49, 34 and 28 years. 2 of these patients had multiple lung metastases which were unsuccessfully managed by actinomycin D therapy. The 34-year old patient exhibited well-marked tumor regression for several months following MPA therapy. However, his general condition deteriorated with hepatomegaly until death occured. The 49-year old exhibited no sign of tumor occurence and is alive and well 7 years after beginning MPA therapy. The 28-year old patient is currently clinically tumor-free after 2 years of MPA treatment. Actinomycin D will be stopped but MPA will be continued for another 12 months in gradually decreasing doses. The 3 cases demonstrate good response to treatment with MPA. Evidence suggests that progesterone, including MPA, inhibits the release of pituitary gonadotropins causing impairment of spermatogenesis and accessory gland function associated with pronounced degenerative testicular changes. 9 rare cases of spontaneous regression of testicular tumor were documented by Everson and Cale in a literature review covering the period 1900 and 1965. It is unlikely however that the improvement in the 2 cases presented in this report can be attributed to spontaneous regression. In both patients, metastases continued to progress until shortly after MPA treatment was initiated. Prospective multicenter clinical trials should be done to evaluate the therapeutic value of hormones, alone or in combination with chemotherapy and irradiation in the management of malignant testicular teratoma.
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PMID:Possible role of hormones in treatment of metastatic testicular teratomas: tumour regression with medroxyprogesterone acetate. 472 28

Invasive surgical procedures will be avoided by recognition of veno-occlusive disease (VOD) as a clinical syndrome which sometimes leads to serious complications in children receiving Actinomycin D for Wilms' tumor. In order to document the unusually frequent occurrence of VOD far beyond the observations of others, a prospective study was undertaken in 13 consecutive Actinomycin D-treated children. There were 9 children experiencing 27 events of mostly mild VOD. Six of them were below 3 years of age having in 5 cases a unilateral kidney tumor on the right side. The criteria used for VOD were painful hepatomegaly and abdominal distension accompanied by weight gain, ascites, hemoglobin and platelet drop, with or without elevated bilirubin level found in all patients developing VOD. Supportive management of these patients should attempt to preserve respiratory and renal function, generally resulting in a favorable outcome.
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PMID:Hepatic veno-occlusive disease in Wilms' tumor. 900 66