Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twelve of 43 patients with chronic active hepatitis (CAH) (28%) manifested clinical and laboratory features of cholestasis. The criteria for selection of these patients included at least two of the following: chronic or recurrent pruritus, serum alkaline phosphatase levels of 300 mU./ml. and cholesterol of 300 mg./dl. or more. When compared with 31 control cases these patients were found to have a preponderance of Ashkenazi Jews of Roumanian origin, a higher prevalence of joint and thyroid involvement and higher serum Ig-M Levels. Mortality was similar in both groups but patients with cholestatic features tended to die earlier in the course of the disease. Retrospectively, it was found that they had been treated more intensively, attained complete remissions less frequently and developed cirrhosis more readily. There were no significant differences in the frequency of HBsAg and anti-HBs, the mode of onset, the frequency of hepatosplenomegaly and jaundice, the hematologic findings and the prevalence of autoantibodies. Like acute cholangiolitic viral hepatitis, CAH with cholestatic features emerges as a more serious disease than the classical form of CAH.
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PMID:Chronic active hepatitis with cholestatic features. I. A clinical and immunological study. 53 97

Hyperbilirubinemia may be of several etiologies in the individual patient. An 18-year-old man presented with extreme hyperbilirubinemia (direct bilirubin 23.0 mg/dl, total bilirubin 60.0 mg/dl), hepatosplenomegaly, and anemia. Hematologic studies prelaparotomy documented the presence of hereditary spherocytosis. Intraoperative liver biopsy revealed moderately reduced levels of glucuronyl transferase activity (Gilbert's syndrome). Common bile duct obstruction secondary to choledocholithiasis was found, and a cholecystectomy and splenectomy were performed. This case stresses the potential confusion among several diseases which may present with hyperbilirubinemia.
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PMID:Extreme hyperbilirubinemia in a patient with hereditary spherocytosis, Gilbert's syndrome, and obstructive jaundice. 68 44

A 15-year old Black teenager came to a clinic at the University of Alabama's School of Medicine in Tuscaloosa requesting oral contraceptives (OCs). The physical examination indicated that she was in good health and the physician prescribed an OC (1 mg norethindrone and .035 mg ethinyl estradiol). 21 months later she returned complaining of yellow eyes for 3 weeks. The oral mucosa was also jaundiced. She had considerably high levels of bilirubin and alkaline phosphatase. She had no hepatitis virus antibodies. 5 months later she returned for the physical examination required to renew the OC prescription. She did not have jaundice at this time. 10 months later she complained of malaise and muscular pain. Her alkaline phosphatase level was high, but her bilirubin level was normal. She had mild hepatosplenomegaly without focal defects. After reviewing her medical records, the physician diagnosed intrahepatic cholestasis and discontinued her OC prescription. Liver function tests were normal within 3 months. 14 months later, she returned complaining of malaise and reported taking OCs obtained at another clinic 3 months earlier. The physician advised her about the complications of OCs and about other contraceptive methods. The same physician also examined a 32-year-old Black woman who had intermittent epigastric and right-upper quadrant abdominal pain for 2 weeks. Eating worsened the pain, which lasted for up to 15 minutes. She had used an OC for 12 years. Ultrasound revealed a 4.2 cm hypoechoic mass in the left upper lobe of the liver. The physician discontinued the OCs. The tumor regressed over 12 months. Active liver disease is a contraindication to OC use. Women who had cholestatic jaundice while pregnant or have first degree relatives with cholestatic jaundice of pregnancy should not use OCs. Physicians may introduce OCs to closely monitored women with a history of liver disease whose liver function tests are normal. Women with a family history of biliary excretion defects should not use OCs.
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PMID:Hepatobiliary complications of oral contraceptives. 133 97

Diphenylhydantoin-induced hepatitis and mononucleosis are uncommon in children. The occurrence of these two diseases in the same individual, with progression to hepatic failure is rare and has not been reported in infants. This report represents a 6-month-old male infant who developed an infectious mononucleosis-like syndrome and hepatic failure 16 days after diphenylhydantoin administration. He took this anticonvulsant for controlling seizures after a head injury. Fever, skin rash, hepatosplenomegaly, lymphadenopathy, and atypical lymphocytosis led to the initial diagnosis of infectious mononucleosis. However, negative heterophil antibody did not support the diagnosis. Jaundice ensued in the following course and became more and more profound. Meanwhile, physical examination showed shrinking in liver size. Negative virology studies, including Epstein-Barr virus, cytomegalovirus, and hepatitis B virus, excluded them as causative agents. The patient lapsed into a stage I hepatic coma, but gradually recovered clinically and biochemically after eight successive exchange transfusions and supportive care. Two liver biopsies were performed 20 and 50 days after the onset of disease, respectively. Remarkable hepatic parenchymal loss, cholestasis, and fatty change were found on histologic examination of the first biopsy specimen, and portal fibrosis was noted on the second.
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PMID:Mononucleosis and hepatic failure associated with diphenylhydantoin treatment in an infant. 167 17

The diagnosis of PSC in association with ulcerative colitis was delayed in this case by the interim development of papillary carcinoma of the thyroid that produced a rapidly enlarging neck mass. The presence of chronic cholestasis, pruritus, and hepatosplenomegaly, along with a liver biopsy with histologic changes consistent with PSC, were not immediately appreciated because of the concern for the patient's thyroid cancer. In all other respects, the clinical features of PSC in this case are characteristic of the disease, with the somewhat unusual feature of intra-abdominal lymphadenopathy. Whether or not the papillary thyroid carcinoma is a coincidental finding rather than an associated disease entity, such as ulcerative colitis, remains open to speculation. In any event, the finding of papillary thyroid carcinoma or any other extrahepatic malignancy in association with PSC is rare, but assumes an added dimension for patients with PSC because the presence of malignancy may affect their suitability for future liver transplant.
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PMID:A 22-year-old man with thyroid cancer and cholestatic liver disease. 204 92

We describe an infant with renal and liver disease who died at the age of 10 months from chronic renal failure. Hepatosplenomegaly was present along with biochemical findings of cholestasis and cytolysis. The diagnosis of tubulointerstitial nephropathy with cortical microcysts associated with hepatic fibrosis was based upon laboratory, radiological, and histological data. This rarely described disorder is characterized by an early clinical onset and unfavourable progression to end-stage renal failure before the age of 2.
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PMID:Progressive tubulointerstitial nephropathy with hepatic involvement in an infant. 217 36

Seventy eight patients with diffuse hepatic lesions were selected for this study. They were classified after liver biopsy histopathologically into four groups. 45 patients with pure bilharzial hepatomegaly (group 1). 12 patients with mixed bilharzial hepatosplenomegaly with hepatitis (group 2). 16 patients with chronic active hepatitis and cirrhosis (group 3) and 5 patients with extrahepatic cholestasis (group 4). Ultrasonography of gall bladder was done for all patients. Group 3 patients showed the highest incidence of gall stones (29.4%) as compared to other groups (P less than 0.05).
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PMID:Pattern of gall bladder sonography in chronic liver diseases. 267 Nov 76

Thirty-two patients of smear positive Plasmodium falciparum malaria having jaundice were analysed retrospectively. Majority of the cases were in the age group of 31-40 years. Serum bilirubin levels ranged from 2 mg to 40 mg%. Fourteen (42.6%) had serum bilirubin above 10 mg%. Conjugated bilirubinaemia was found in twenty one patients (65.5%), unconjugated in 4 (12.5%) while 7 patients (21.8%) had a mixed pattern. Serum transaminases were high in 21.8% patients. Twenty five patients (78%) had associated azotaemia, 11(34.3%) had intravascular haemolysis and 3(9.3%) had possible cerebral malaria. Hepatosplenomegaly was seen in all the 32 patients. Mortality was 21% but none died of hepatic encephalopathy. Histologically the most consistent finding in liver biopsies was reticulo-endothelial cell hyperplasia. Pigmentation in kupffer cells, fatty change, sinusoidal and portal infiltration and cholestasis were the other features seen.
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PMID:Jaundice in Plasmodium falciparum. 268 27

The first case of nonsyndromatic paucity of the intrahepatic bile ducts is reported in congenital syphilis. The patient, a 2-week-old female, was born at the 31st week of gestation, weighing 1,910 g. She had a high titer of IgM antibody to Treponema pallidum and sera from both parents also showed a positive reaction in the hemagglutination test for Treponema pallidum. The patient had hepatosplenomegaly and increasing jaundice, and died of respiratory failure on the 70th hospital day. Autopsy examination revealed paucity of the intrahepatic bile ducts, prominent giant cell transformation of hepatocytes, cholestasis and extramedullary hematopoiesis of the liver. The ratio of the number of intrahepatic bile ducts to that of the portal tracts was approximately 0.2:1. There was marked proliferation of atypical bile ductules at the margin of the portal tracts. The exact relation of this paucity to Treponema pallidum remains unknown.
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PMID:Nonsyndromatic paucity of intrahepatic bile ducts in congenital syphilis. A case report. 318 14

The results from the complex study on 26 patients with primary biliary cirrhosis (PBC), 20 females and 6 males, an average age of 46 years, are reported. The most frequent symptoms of PBC are itching, jaundice, hepatosplenomegaly; from the laboratory tests--most characteristic is the increase of serum 5'-nucleotidase, AP, LAP, gamma GTP, GOT, cholesterol, cholic acid and antimitochondrial antibodies and IgM (AP, 5'-nucleotidase and antimitochondrial antibodies, being most significant in making the early diagnosis). The laboratory results in PBC are compared with those of the chronic active hepatitis, cirrhosis of the liver, liver cancer, extrahepatic cholestasis, with outlining the characteristic differences, depending on the diagnosis. The diagnostic advantages of the various methods are discussed (mainly laparoscopy and liver biopsy) and the histologic and electron microscopic changes of percutaneous transhepatic cholangiography, via echography--81 per cent, laparoscopy--73 per cent, scintigraphy--61.53 per cent and liver biopsy--50 per cent. The results from the treatment with cholestrimine, corticosteroids and azathioprine and surgical treatment, observing a temporary improvement and progressing of PBC, are reported. With the follow-up care of 20 patients, it was established, that 9 had died 5 years, on the average, after making the diagnosis, 11 survived after the 5 years and they are still followed up. The longest survival was reported in two females--11 and 15 years after the onset of PBC.
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PMID:[Primary biliary cirrhosis]. 632 95


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