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)

A child with hemihypertrophy presented with Wilm's tumor at 3 years of age. History included neonatal hypoglycemia and the discovery of hepatomegaly and renal dysplasia. This case report emphasizes the close relation of body hemihypertrophy to Beckwith's syndrome, as well as the high degree to which both predispose to certain tumors.
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PMID:[Nephroblastoma and whole-body hemihypertrophy. Relation to Wiedemann-Beckwith's syndrome]. 23 62

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

Four weeks following autologous bone marrow transplantation for Wilms' tumor, a patient developed fever, hepatomegaly, coagulation disorders and pancytopenia. Bone marrow studies showed progressively increased hemophagocytosis of normal hematopoietic progenitors by histiocytes resulting in aplasia. Adenovirus type 11 was consistently isolated from urine and stool cultures, and one of the marrow aspirates. At autopsy, adenovirus was isolated from the lungs, liver, heart, intestine and spleen. These findings are consistent with the previously described virus-associated hemophagocytic syndrome, which have not been associated with bone marrow transplantation. This case suggests that this diagnosis should be considered in any bone marrow transplant patient who has evidence of secondary graft failure.
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PMID:Adenovirus-related hemophagocytic syndrome after bone marrow transplantation. 229 98

Veno-occlusive disease (VOD) of the liver was diagnosed in 8 patients with Wilms' tumour and peliosis hepatis (PH) in one. Fever of obscure origin, vague abdominal pain, hepatomegaly or hepatosplenomegaly, severe anaemia or sudden, unexplained drop in haemoglobin, thrombocytopenia, increasing serum transaminase levels, jaundice and ascites recorded within the first weeks or months of tumour diagnosis should arise suspicion of non-metastatic vascular hepatopathy. General or focal decreased accumulation of isotope at liver scintigraphy belong to the early radiologic findings. Sonography and CT may show a generalized irregular echogenicity or attenuation but no unequivocal metastases. One patient with PH had multiple low attenuating foci in both liver lobes and angiographically abnormal pooling of contrast medium in the liver. It is important to recognize these conditions as alternatives to suspected liver metastases, which as a rule develop much later yet on occasions may have very similar radiologic appearances. Therefore the relation in time between tumour diagnosis, initial operation and development of obscure hepatic manifestations is of critical significance for the recognition of VOD or PH. In these patients chemotherapy and irradiation must be discontinued without delay. If the disorders are adequately treated the prognosis may be considered fair.
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PMID:Veno-occlusive disease and peliosis of the liver complicating the course of Wilms' tumour. 300 Jan 41

In 1973, Perlman et al described a familial syndrome of bilateral renal hamartomas with or without nephroblastomatosis, macrosomia, islet cell hypertrophy, unusual facies, and early lethality. Two additional sibs were recently reported by Neri et al [1984]. We report on two sibs with polyhydramnios, fetal ascites, and abdominal muscular hypoplasia, visceromegaly, and subsequent development of Wilms tumor in one of them. Delineated features of this syndrome include visceromegaly, macrosomia, renal hamartomas, nephroblastomatosis, cryptorchidism in males, unusual facial appearance, polyhydramnios, fetal ascites, and Wilms tumor but do not include hemihypertrophy, omphalocele or umbilical abnormalities, aniridia, or other conditions known to be associated with Wilms tumor. This condition should be considered primarily in the differential diagnosis of fetal ascites without hydrops and possibly in the differential diagnosis of familial Wilms tumor, polyhydramnios, congenital hepatomegaly, or nephromegaly.
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PMID:The Perlman familial nephroblastomatosis syndrome. 301 Jul 22

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

The use of radiocolloid liver scanning as a routine diagnostic procedure to detect liver metastases has been declining as the most effective uses of this test have become better appreciated. Liver scanning to detect hepatic metastases appears to have the greatest efficacy in two circumstances. The first is as a staging procedure in malignancies that metastasize to the liver early, before being suspected clinically or liver function parameters alter. Such malignancies include gastric carcinoma, Wilms' tumor, small cell carcinoma of the lung, and rhabdomyosarcoma. The second effective use of liver scanning is as a confirmatory test in patients with known malignancy who develop abnormal levels of serum liver enzymes, carcinoembryonic antigen titer, hepatomegaly, ascites, or jaundice.
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PMID:Efficacy of liver scanning in malignant diseases. 609 64

Two children being treated with combination chemotherapy and irradiation for localized, rightsided Wilms' tumor developed sudden enlargement of the liver with defects on liver scintigram resembling liver metastases. One child also developed pancytopenia. When chemotherapy was temporarily withheld in both children, hepatomegaly and scintigram abnormalities resolved. The planned courses of chemotherapy were subsequently completed without complications. The clinical course in our patients is compared to previously published experiences. Awareness of this complication could prevent the mistaken diagnosis of metastatic disease and emphasizes the care necessary when administering cytotoxic drugs to children receiving irradiation to all or a portion of the liver.
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PMID:Hepatopathy following irradiation and chemotherapy for Wilms' tumor. 628 75

Three children, treated with dactinomycin and vincristine without radiotherapy showed hepatic toxicity consistent with diagnostic criteria for hepatic veno-occlusive disease (VOD). Two patients were affected with Wilms' tumor and the third with malignant fibromatosis. The clinical manifestations of VOD were mild and regressed in all patients after supportive therapy. Serial ultrasonography (US) was performed in all cases and was useful in confirming the diagnosis and in evaluating the severity of the disease. US features of VOD were hepatomegaly, gallbladder wall thickening, ill-defined borders of the hepatic vessels, and ascites. Parenchymal heterogeneity was still present after several months as a result of the hepatic injury. The hazy appearance of the portal vessels and the gallbladder wall thickening seemed directly correlated with the degree of hepatic involvement in the early phase of VOD.
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PMID:Ultrasound in the management of hepatic veno-occlusive disease in three children treated with dactinomycin and vincristine. 894 Jul 35


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