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)

13 patients with extrahepatic bile duct carcinoma treated in our institute from 1960 to 1986 are reported. All were proven by pathology. There were 10 moderately differentiated or mucin adenocarcinomas, 2 poorly differentiated and 1 undifferentiated cancers. There were 9 males and 4 females with an average age of 60.6 years. Progressive obstructive jaundice was the most common presenting symptom (11/13). Hepatomegaly was found in 7 patients, distended gallbladder in 4 and gallstone in 2. Before operation, 10 patients were misdiagnosed as hepatitis, cholecystitis or cholelithiasis. During operation, regional lymph node metastasis was observed in the majority of patients. Palliative operation was performed in 10 patients and radical surgery in 3. Three received operation plus postoperative radiotherapy. None survived more than two years. The lesions occurred frequently in the upper bile duct (8 patients). The middle bile duct and diffuse type carcinomas comprised 2 each. One was not recorded clearly. The prognosis is related to the gross type of the tumor and differentiation degree. Finally, carcinogenesis is discussed briefly.
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PMID:[Carcinoma of the extrahepatic bile duct--report of 13 patients]. 285 Jan 47

Hepatic morphology and immunocytology were evaluated in 4 children with clinical and immunologic characteristics of the acquired immune deficiency syndrome or acquired immune deficiency syndrome related complex. All 4 children had hepatomegaly and increased serum alanine and aspartate aminotransferase activity. Both lobular and portal changes were noted. Lymphocytic infiltration, piecemeal necrosis, hepatocellular and bile duct damage, sinusoidal cell hyperplasia, and endothelialitis were prominent. Vesicular rosettes in sinusoidal lymphocytes and tubuloreticular structures in sinusoidal endothelial cells were demonstrated by electron microscopy. The lymphocytic infiltrate in both the lobular and portal spaces was characterized by a relative increase of cytotoxic/suppressor (T8) cells. Hepatitis may be a common feature of pediatric acquired immune deficiency syndrome and acquired immune deficiency syndrome-related complex. Although the histopathologic changes are consistent with chronic active hepatitis, the specific pathogenesis remains to be determined.
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PMID:Hepatitis in children with acquired immune deficiency syndrome. Histopathologic and immunocytologic features. 293 90

Clinical data and histologic sections of the liver, including immunohistochemical studies for hepatitis B surface and core antigens, were reviewed in 42 autopsy cases of the acquired immune deficiency syndrome (AIDS). Hepatomegaly, elevation of serum transaminases, and mild elevation of alkaline phosphatase were commonly observed clinical and biochemical abnormalities. Mildly elevated alkaline phosphatase and normal bilirubin levels were present in patients with Mycobacterium avium-intracellulare (MAI) infection, cytomegalovirus (CMV) infection, and Kaposi's sarcoma (KS). Histologic sections demonstrated liver involvement by MAI in eight cases; KS in six cases; cryptococcus in three cases; and CMV in two cases. One case of MAI infection was associated with marked central vein sclerosis, a finding previously unreported. Thirty-two (76%) of 42 cases had serologic or pathologic evidence of hepatitis exposure. Two patients had histologic evidence of chronic active hepatitis. The pathologic processes involving the liver appeared to be secondary to the infections and neoplasms for which this population is susceptible and did not significantly contribute to morbidity or mortality. No findings specific or pathognomic for AIDS were identified in the liver.
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PMID:Clinical and pathologic findings of the liver in the acquired immune deficiency syndrome (AIDS). 298 50

The clinical and pathologic features of Epstein-Barr virus (EBV) hepatitis in 3 children are described. Manifestations included fever, hepatomegaly, disseminated intravascular coagulation, and failure of uptake of technetium by the reticuloendothelial system of the liver. Histologic features may mimic chronic active hepatitis and lymphoid malignancy. Two patients underwent exploratory laparotomy because of suspected tumor. Recognition of the wide spectrum of hepatic involvement in infectious mononucleosis is important in the differential diagnosis of hepatomegaly. Diagnosis should be made by measurement of IgM-specific EBV antibodies.
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PMID:The spectrum of Epstein-Barr virus hepatitis in children. 302 40

A 3-month-old girl presented with anorexia, failure to thrive and drowsiness. She was mildly icteric with hepatomegaly and peripheral oedema. Disordered liver function tests were associated with the biopsy appearances of a giant cell hepatitis and with a Fanconi syndrome. At the age of 16 weeks she collapsed with profound hypoglycaemia. Fasting also provoked hypoglycaemia with lactic acidaemia. She became increasingly irritable and hypotonic and, although initially liver and renal function improved, she deteriorated and died of hepatocellular failure and septicaemia. A post-mortem revealed massive fatty degeneration of the liver. The activity of phosphoenolpyruvate carboxykinase in her cultured skin fibroblasts was 16% of controls. Her brother died at the age of 4 weeks of sudden infant death syndrome.
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PMID:Mitochondrial phosphoenolpyruvate carboxykinase deficiency. 308 95

We studied the risk of posttransfusion hepatitis in recipients of blood collected from volunteer donors who tested negative for HBsAg and had serum ALT levels less than 1.5 times the upper limit of the normal range. Between October, 1983 and September, 1984, 676 consecutive patients who needed blood or plasma transfusions during or after elective surgery, who had no history of liver disease and had never received blood previously, were studied. The patients were given a total of 4,813 (mean = 7) units. Ninety-six patients developed posttransfusion hepatitis, which yielded a hepatic incidence of 20 cases per 1,000 units of transfused blood. Ninety-two patients had non-A, non-B hepatitis, 3 had hepatitis B and 1 had cytomegalovirus infection. The incubation periods for non-A, non-B hepatitis ranged from 2 to 26 (mean = 9.5 +/- 4) weeks. In 68 (73%) patients, the hepatitis was completely asymptomatic; only 24 (27%) patients developed symptoms, including jaundice and hepatomegaly. There were no cases of fulminant hepatitis. Sixty per cent of the patients still had elevated serum ALT levels 1 year after the onset of hepatitis. The 96 patients with hepatitis had received a mean of 9.6 blood units, as compared to a mean of 6.7 units for the unaffected patients (p less than 0.001). This study demonstrated that non-A, non-B hepatitis remains a common and important complication of blood transfusion despite screening of blood donors for HBsAg and elevated serum ALT levels.
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PMID:A multicenter, prospective study of posttransfusion hepatitis in Milan. 311 67

Nine patients of leprosy, 5 BL and 4 LL who developed jaundice during the course of disease were investigated. Two LL patients developed jaundice during ENL reaction. There was slight hepatomegaly in 5 patients and moderate splenomegaly in 3 only. There was significant alterations in liver enzymes and serum bilirubin in all patients. The abnormalities of the enzymes levels persisted for abnormally long periods even when the serum bilirubin had come down and the patients had become asymptomatic. Blood for HBsAg and anti-HAV IgM was negative in all the patients except one in whom HBsAg was positive. Drugs could not be implicated as the cause of jaundice, all patients maintained recovery even after restarting antileprosy drugs. The possibility of non A, non B viruses producing hepatitis during the course of disease is brought out. Course of prolonged jaundice in leprosy is compared with other diseases which could result in a similar situation.
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PMID:Leprosy, liver and jaundice. 311 6

The presentation and 2 year treatment of a patient with argininosuccinic aciduria is reported. Erythrocyte argininosuccinate lyase activity was less than 2% of normal. Long-term management included protein restriction and arginine dietary supplementation. The child experienced three episodes of hyperammonaemia (greater than 100 microns), the first at birth, the second at 6.5 months and the third at 16 months. Neurological development deteriorated between 14 and 24 months. Hepatomegaly and biochemical hepatitis, a feature of this condition, was accompanied by enlarged mitochondria with tubular paracrystalline inclusions not previously recognized in this disorder.
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PMID:Argininosuccinic aciduria: long-term treatment with arginine. 311 34

Thirty six patients with culture-proven enteric fever and 15 patients of fever with etiology other than enteric fever as a control group were studied, with special reference to hepatic dysfunction and its relation to clinical features of the disease. Hepatomegaly was observed in 55% of enteric fever patients, and was slightly more common than splenomegaly (50%). Its incidence in typhoid fever (67%) was three times higher than in paratyphoid fever (22%). Hepatic dysfunction occurred in 55% of cases. Jaundice was noted in only 8% of the cases, whereas hyperbilirubinemia (serum bilirubin greater than 1.8 mg %) was present in 17%. Although hepatic manifestations of enteric fever were mild, a small but important group had sufficient hepatic involvement to mimick the clinical picture seen in viral hepatitis, amebic liver disease, and malaria with jaundice. It may be considered of clinical significance, since enteric hepatitis responds very well to specific therapy.
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PMID:The spectrum of hepatic injury in enteric fever. 312 48

A case report is presented of a 43-year-old woman with generalized peliosis hepatitis that developed during longterm use of oral contraceptives (OCs). The patient had been in good health until the last 2 years when she began to experience vague epigastric pains and a feeling of abdominal distension. Several months prior to admission, she had started to complain of itching and fatigue. There was no history of dark urine, white stools, or hepatitis. On physical examination, no jaundice or cutaneous stigmata of chronic liver disease were observed. Laboratory studies showed a normal erythrocyte sedimentation rate and hematological blood count. A radionuclide study of the liver showed hepatomegaly; especially the left lobe was enlarged. A computerized tomographic scan of the liver showed multiple areas of decreased density in both of the enlarged lobes. There was no evidence of a tumor. Selective transfemoral angiography of the celiac artery also showed hepatic enlargement but no signs of a space-occupying lesion. At laparoscopy, the liver was grossly enlarged and had a lumpy appearance, but again there were no signs of a tumor. No evidence of veno-occlusive disease or hepatocellular adenoma was found. The diagnosis was peliosis hepatitis. The OCs were withdrawn, and the patient was discharged. Regular follow-up in the outpatient department showed no decrease in the size of the liver. The alkaline phosphatase level rose. The fatigue became worse, and cholestyramine was prescribed for progressive itching. In September 1980, the patient was admitted for reevaluation. A repeated CT scan and angiography of the liver again yielded no evidence of a tumor. Esophagoscopy showed the presence of varices grade 2. The liver at laparoscopy had the same appearance as it had in 1976. Histological examination of a biopsy specimen showed occasional dilated sinusoids and locally marked periportal and intralobular fibrosis. No regeneration nodules were found. The diagnosis was liver fibrosis. The patient's condition deteriorated gradually in the following years. She experienced increasing fatigue. Steatorrhea developed, and the patient lost weight. She needed increasing doses of cholestyramine and oral supplementation of vitamins A, D, and K. She was admitted for a 3rd time in February 1985. Esophagoscopy revealed varices grade 4. A CT scan of the liver showed no change. The patient successfully underwent an orthotopic liver transplantation in January 1987. The diagnosis of peliosis hepatis was well documented in this patient.
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PMID:Generalized peliosis hepatis and cirrhosis after long-term use of oral contraceptives. 312 33


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