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)

The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS.
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PMID:Early bone marrow transplantation in an infant with Wiskott-Aldrich syndrome. 179 57

In 5 years, 64 solid hepatic lesions have been referred for diagnosis and management which have been found unexpectedly on organ imaging in well patients. We have called this lesion a "dystychoma". Patients have undergone a two phase investigation programme which allows a diagnosis without admission to hospital in about 50% of cases. About three quarters of patients (47/64) have had non-neoplastic lesions, and about half (33/64) have had haemangiomas. About one patient in four (17/64) has had a neoplasm, and the neoplasm has been malignant in about one in six (11/64) of all patients. We stress the need to pursue the diagnosis in these patients. There were no reliable clinical, biochemical or imaging characteristics which individually distinguished benign from malignant lesions. Age over 55 years, an enlarged liver or a palpable liver mass and a raised serum alkaline phosphatase were all significantly more frequent with malignant tumours. The risk of malignancy rose with the number of risk factors, and all patients with all three risk factors had malignant tumours. Only 11 of the 64 patients were judged to have benefited by significant increase in quality or quantity of life as a result of what was frequently inappropriate organ imaging. There is no strong argument for replacing history taking and physical examination by CT scanning, ultrasound examination or other organ imaging.
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PMID:Hepatic dystychoma: a five year experience. 181 Mar 71

The association of several drugs with different target specificities has long been proven to be more efficient than one single agent in the treatment of cancer. This strategy might also be of benefit in the cure of retrovirus-induced diseases. The effectiveness of several drug combinations was evaluated on DBA/2 mice injected with Friend leukaemia virus (FLV). Elliptinium (ELP), a known chemotherapeutic agent with possible antiviral activity, was given in association with norcantharidin (NCTD) (shown previously to increase the cytotoxic potential of human lymphocytes) and/or in association with tetrachlordecaoxide (TCDO), which augments both humoral and cellular immune responses. ELP alone at a dose of 0.2 mg/kg in long-term treatment significantly increased the survival of mice infected by FLV. When ELP, TCDO (2 micrograms/kg) and NCTD (2 mg/kg) were given together, the survival time was prolonged 1.6 times and 2 times as compared to the group treated by ELP alone or to non-treated controls, respectively. Moreover, the combined treatment gave more effective inhibition of hepatomegaly than ELP alone, suggesting that this protocol might have an organ-specific effect and might suppress the leukaemogenesis induced by FLV in some erythropoietic organs. These results indicate that a chemotherapeutic agent (ELP) associated with two immunomodulatory substances (NCTD and TCDO) is more effective than chemotherapy alone in controlling retroviral infection and in the prolongation of survival. These data taken together suggest that the role of the two immunomodulatory agents might be to suppress the retroviral infection synergistically with ELP and enhance immune functions. Possible modes of action are discussed and are under investigation.
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PMID:Elliptinium, norcantharidin and tetrachlordecaoxide in combined chemo-immunotherapy prolong the survival of Friend-virus-infected mice. 182 34

48 patients with colorectal cancer metastatic to the liver were implanted with a subcutaneous access system allowing hepatic intra-arterial perfusion. Regional chemotherapy used 5-fluorouracil, while 17 patients also received low-dose mitomycin at the beginning of the study. Responses to the treatment occurred in 29 patients (60%) and median survival was 14.4 months. Toxicity included gastroduodenal erosions in 12.5% of the patients, leucopenia in 20.8%, catheter thrombosis in 42% and arterial thrombosis in 50%. 2 patients died of digestive haemorrhage probably related to treatment. When individually analysed, four factors were found to significantly affect survival: presence of hepatomegaly (defined as palpable liver edge exceeding the right costal margin by more than 5 cm) (P = 0.006), percentage of hepatic replacement superior to 50% (P = 0.003), more than four metastases (P = 0.025) and hypovascularised metastases at radionuclide liver scan with 99m technetium-labelled macroaggregate albumin (MAA) (P = 0.04). The effect of the four variables on the observed survival time was analysed using a Cox regression model. Two variables were found to have simultaneously influenced survival. Presence of hepatomegaly emerged as the more significant (P = 0.0001), the other being hypovascularised metastases at 99mTc-MAA.
Eur J Cancer 1991
PMID:Prognostic factors in patients with liver metastases from colorectal carcinoma treated with discontinuous intra-arterial hepatic chemotherapy. 183 91

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

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

The preceding discussions outline the various forms of cirrhosis that may be encountered in the elderly population. Cirrhosis is not uncommon in older patients. Although it has been stated that most cirrhosis in the elderly is due to alcohol, these assumptions are perhaps overestimations. In the authors' experience, many older patients are inappropriately labeled with alcoholic liver disease--presumed guilty until proven otherwise--and have subsequently been shown to have nonalcoholic liver disease. Careful investigation is required. Hepatotoxic drug exposure (e.g., to alpha methyldopa, nitrofurantoin, or isoniazid) should be ruled out, and hepatitis B and hepatitis C serology obtained. Primary biliary cirrhosis may occur in both sexes, and thus antimitochondrial antibody should be assayed. Severe heart disease may result in cardiac cirrhosis in the elderly, with ascites and hepatomegaly. Alpha 1-antitrypsin deficiency, primary sclerosing cholangitis, idiopathic hemochromatosis, and chronic autoimmune hepatitis may result in advanced cirrhosis in the elderly; appropriate serum studies should be obtained. If questions remain and if therapy may be changed, liver biopsy can be performed. A recent study suggested, however, that the risk of hemorrhage from liver biopsy in the elderly may be increased, especially if malignancy is present. The era of treatment for liver diseases has arrived. Colchicine, methotrexate, ursodeoxycholic acid, and others have shown promise in the treatment of PBC, primary sclerosing cholangitis, and alcoholic liver disease. Corticosteroids may be lifesaving in autoimmune liver disease. Phlebotomy remains the treatment of choice for hemochromatosis in any age group. Interferons and other antiviral agents are being used in chronic type B and type C hepatitis. Treatment of the complications of cirrhosis in the elderly may be safely accomplished. Advanced age is not a contraindication to variceal sclerotherapy. Vasopressin, however, may be contraindicated in the elderly patient if there is an underlying history of atherosclerotic coronary or peripheral vascular disease. Large-volume paracentesis and peritoneal venous shunting can afford symptomatic relief of ascites, even in the geriatric population. Finally, as noted previously, advanced age is no longer to be considered an absolute contraindication for liver transplantation. The evaluation of liver disease in the elderly may be diagnostically challenging, and its treatment rewarding.
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PMID:Liver diseases in the elderly. 185 64

The purpose of our study was to assess the pattern of liver diseases diagnosed by ultrasonography (US) in Yaounde (Cameroon). From a total of 1,007 consecutive US studies performed over a period of 14 months in subjects over the age of 15 years, we found 322 patients with a sonographic diagnosis of liver disease. 8 diagnoses comprised 83% of the patients. Apart from non specific homogeneous hepatomegaly these diagnoses consisted of: cirrhosis, primary liver malignancies, liver abscess, congestive hepatomegaly, secondary liver malignancies, diffuse steatosis and nodular calcifications. 30 liver abscess drainages were performed with sonographic guidance. We conclude that in our environment where Computed Tomography is scarce and expensive, US is a useful tool in the study of some of the most frequent liver diseases.
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PMID:[Pattern of hepatic pathology diagnosed with echography in Yaounde (Cameroon)]. 192 48

Clinical and morphological findings of lymph nodes in 150 consecutive untreated non-Hodgkin lymphoma (NHL) patients were retrospectively studied. One hundred and fifteen (77%) patients had B-NHL and 35 (23%) T-NHL, 96 (64%) patients had NHL low grade malignancy and 54 (36%) NHL of high grade malignancy according to the Kiel classification. Lymph nodes exceeding 2 cm in diameter (p less than 0.05), hepatomegaly (p less than 0.05), splenomegaly (p less than 0.05), and the duration of lymphadenopathy for more than 6 months preceding diagnosis (p less than 0.01) were significantly more common in low than high grade malignancy of NHL patients. Febrile episodes at the diagnosis were significantly more common in high than in low grade malignancy of NHL patients (p greater than 0.05). Lymph nodes exceeding 2 cm in diameter (p less than 0.05) in B-NHL, and lymph nodes above the diaphragm (p less than 0.05) and skin infiltration (p less than 0.001) were more common in T-NHL than in B-NHL patients. At the diagnosis low grade NHL patients have significantly more often splenomegaly, hepatomegaly, large palpable lymph nodes, and long lasting lymphadenopathy before diagnosis. High grade malignancy NHL patients have more often general symptoms, B-NHL patients have more often large palpable lymph nodes, T-NHL patients have more often skin infiltration and lymph nodes above the diaphragm. Precise clinical characterization of patients in addition to pathohistological diagnosis are very important in this highly variable disease.
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PMID:[Relation between clinical parameters and histologic findings in patients with non-Hodgkin's lymphoma]. 195 Jun 42

Twenty-three cases of congenital malignant melanoma have previously been reported. Here the authors report the first case of a congenital malignant melanoma arising in the eye. A newborn girl had a large pigmented ocular tumor, hepatomegaly, and multiple pigmented skin and choroidal lesions. The histopathologic diagnosis was of a malignant melanoma with hepatic metastases. The skin and choroidal lesions were considered to be congenital melanocytic nevi. The most plausible pathogenetic link between these two conditions was that the malignancy had arisen as a second-hit mutation within a choroidal congenital melanocytic nevus. Despite widespread metastases the baby, treated by surgery and chemotherapy, survives in good health, aged 2 years, 10 months.
Cancer 1991 May 15
PMID:Congenital malignant melanoma of the eye. 201 65


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