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 10-month-old male infant had keratosis follicularis spinulosa decalvans, an X-linked dominant disorder. His cutaneous abnormalities consisted of generalized hyperkeratosis, spiny follicular papular lesions, universal alopecia, and hypoplastic nails. Ocular changes characteristic of the disease were also present. Unusual findings included deafness, failure to thrive, predisposition to bacterial infections without demonstrable immune defect, and transient hepatomegaly with abnormal liver function studies.
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PMID:Keratosis follicularis spinulosa decalvans. An infant with failure to thrive, deafness, and recurrent infections. 64 99

Supplementation of a closed formula, cereal based stock diet with excess L-histidine at a 5% or 8% level for 4 days reduced growth and induced hepatomegaly and an increase in plasma cholesterole levels in weanling rats. The enlargement of the liver was in part due to glycogen accumulation; plasma glucose concentration was unchanged. Feeding four different amino acids (L-phenylalanine, L-glutamic acid, glycine and L-tryptophan), at levels which caused reduction of growth comparable to the 5% and 8% L-histidine supplementation, did not effect liver weight or plasma cholesterol levels. L-Threonine added, at a 2% level, to the 8% L-histidine diet did not alleviate any of the histidine effects. Rats fed a diet containing 5% urocanic acid, the first metabolite of the histidine degradative pathway, grew at a normal rate but had higher plasma cholesterol levels compared to rats fed stock diet. When rats fed L-histidine-or urocanic acid-supplemented diets were returned to stock diet, a normal growth rate was resumed immediately and plasma cholesterol levels returned to normal within 6 days. These results suggest that L-histidine and/or urocanic acid induce a hypercholesterolemia which disappears several days after the supplementation ceases.
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PMID:Effect of excess dietary L-histidine on plasma cholesterol levels in weanling rats. 65 Feb 95

Amiodarone, a commonly used antiarrhythmic agent, has numerous adverse effects. The purpose of this case report is to highlight its hepatotoxicity, an unusual complication of long term amiodarone therapy. Our patient is a 76-year-old man with underlying ischaemic heart disease and recurrent ventricular tachycardia. Eleven months after commencing amiodarone, he developed asymptomatic raised aminotransferases which resolved following drug withdrawal. Amiodarone was then reintroduced and four years later, the patient developed hepatomegaly, worsening liver biochemistry and histopathological changes consistent with early cirrhosis. His symptoms improved following discontinuation of amiodarone. However, hepatomegaly and a low serum albumin still persist four years later.
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PMID:Hepatotoxicity of amiodarone. 129 29

Three patients with hepatic fascioliasis are presented. (case 1) A 36-year-old female was admitted in January, 1988, because of intermittent high-grade fever. Laboratory findings included white blood cell count 8,050/mm3 with 29% eosinophils, and CRP 9.5 mg/dl. There was a positive intradermal reaction to Fasciola hepatica antigen. Liver biopsy revealed eosinophilic abscess, granulation and many Charcot-Leyden crystals. (case 2) A 54-year-old male was admitted in June, 1985, because of abdominal fullness. Marked hepatomegaly was noticed. Laboratory findings included white blood cell count 10,200/mm3 with 26% eosinophils, and CRP 2.2 mg/dl. (case 3) A 48-year-old male was admitted in March, 1989, because of intermittent high-grade fever. Hepatomegaly was noticed. Laboratory findings included white blood cell count 7,310/mm3 with 30.3% eosinophils, and CRP 3.5 mg/dl. His condition was complicated by pleuritis with invasion of Fasciola hepatica. In all patients, stool specimens were negative for ova and immunoelectrophoresis was positive for F. hepatica antigen. Computed tomography demonstrated multiple low-density areas in the periphery of the liver in cases 1 and 3, and disseminated areas in case 2. After treatment with bithionol 40 mg/kg every other day for 40 days, all three patients became asymptomatic.
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PMID:[Three cases of human fascioliasis]. 178 14

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

A 47 years old male with POEMS syndrome is presented. His illness started seven months before admission. He was in poor general conditions and had malnutrition. He had cutaneous hyperpigmentation, skin changes, hepatomegaly, edema of the limbs, quadriplegia, myotatic reflexes abolished, hypotrophy of muscular masses and bilateral papilledema. Immunoglobulins IgA and IgM were abnormal. Bone scanning showed multiple lytic lesions. The study of bone marrow showed megaloblastic changes. Electromyography revealed severe neuropathy and biopsy from sural nerve showed severe demyelinization. At first, he showed improvement with steroids; nevertheless he died two months afterwards from possible bronchoaspiration. The autopsy revealed changes compatible with the clinical diagnosis and in addition an adenoma of the hypophysis. We emphasize the importance of POEMS syndrome in the differential diagnosis of polyneuropathies.
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PMID:[POEMS syndrome. Report of a case and review of the literature]. 148 88

It has been generally accepted that congestive heart failure does not lead to fulminant hepatic failure, unless it is associated with cardiac shock or low cardiac output. Only three cases have been reported, in which liver congestion is followed by fulminant hepatic failure without a history of shock or low cardiac output. Here we present a case of a 48-year-old man with dilated cardiomyopathy and pulmonary infarction, who developed fulminant hepatic failure from congestion. When he was admitted for the control of diabetes mellitus, hepatomegaly of 3-finger breadth and marked cardiomegaly without pulmonary congestion was noted. Diabetes was controlled using insulin. But 3 weeks after admission, he sometimes complained of back dullness because of pulmonary infarction. His heart gradually increased in size, and Jugular venous dilatation and pretibial pitting edema also worsened. Jaundice was noted and serum GOT and GPT increased. A large liver of 6-finger breath below the right costal margin was able to be felt. But within one week, the size of the liver markedly decreased and the signs of hepatic failure such as jaundice, hepatic encephalopathy and numerous petechiae appeared. Blood pressure was maintained and no hypotension or cardiac shock was noted. The patient died of fulminant hepatic failure on the 20th days after onset of the hepatic failure. The autopsy revealed liver atrophy with severe central lobular necrosis, and thrombus in the right main pulmonary artery which caused severe pulmonary infarction. The mechanism of fulminant hepatic failure not accompanied with low cardiac output is discussed.
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PMID:[A case of fulminant hepatic failure secondary to congestive heart failure]. 187 44

A 69-year-old male, who had been found (during a routine roentgenogram of the chest at an annual check-up) to have cardiomegaly, had been asymptomatic until 63 years of age, when DOE and swelling of the legs appeared. He visited our hospital because these symptoms has become more severe. Physical examination revealed JVD, third heart sound and a grade 3 holosystolic murmur, hepatomegaly and edema in the lower legs. His ECG showed atrial fibrillation. His chest X-P showed marked cardiomegaly and rt pleural effusion. His echocardiography and MRI revealed a marked enlargement of the right atrium and a slight enlargement of the right ventricle. The latter also showed persistent left superior vena cava. The cardiac catheterization, angiocardiography and intracavitary electrocardiography revealed no organic cardiac disease which induced enlargement of the right atrium. The idiopathic enlargement of the right atrium is a rare disease. Patients suffering from this disease are asymptomatic in most cases. We reported the idiopathic enlargement of the right atrium with persistent left superior vena cava in this paper.
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PMID:[Idiopathic enlargement of the right atrium with persistent superior vena cava]. 214 15

Two children are reported with hemangioendothelioma of the hepatobiliary system. One patient, a 7-month-old girl, presented with classic symptomatology of hepatomegaly and congestive heart failure, but did not have any cutaneous lesions. She was treated with systemic steroids and supportive therapy and did very well. The other patient, a 22-month-old boy, is the first patient reported with exclusive involvement of the extrahepatic biliary tree. His symptoms included hepatomegaly and obstructive jaundice. He was evaluated with an endoscopic retrograde cholangiopancreatogram and a transhepatic cholangiogram. He did very well after resection of the lesion and Roux-en-Y drainage of the proximal biliary tree and remains asymptomatic 2 years after surgery.
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PMID:Hemangioendothelioma of the hepatobiliary system: the classic and the unusual. 218 12

A case of malignant histiocytosis in a two year old boy is reported. His main clinical features were fever, lymphadenopathy, hepatomegaly and splenomegaly. Lymph node biopsy showed a sinusoidal type of lymph node infiltration, histiocytes of malignant aspect and erythrophagocytosis. Liver infiltration with tumoral cells was demonstrated by needle biopsy. The clinical evolution was rapidly progressive and after six months of chemotherapy he died of intercurrent respiratory infection.
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PMID:[Malignant histiocytosis in children]. 248 97


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