Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

2-Deoxy-D-galactose, in a dose of 3 mmol/kg, was administered intraperitoneally twice daily to young rats for periods up to 12 weeks. This dosage schedule resulted in recurrent phosphate trapping predominantly in liver. UTP deficiency was excluded by simultaneous uridine injections. Phosphate trapping was caused by the rapid accumulation of 2-deoxy-D-galactose 1-phosphate and was most pronounced in liver but also demonstrated in small intestine, brain, spleen, and thymus. The marked, although transient, drop in the hepatic content of inorganic phosphate triggered the catabolism of adenine nucleotides and a loss of ATP. Other metabolic pathways affected by phosphate deficiency include glycogenolysis and glycolysis. Increasing with time, repeated doses of the galactose analog led to retardation and arrest of growth, hepatomegaly, and splenomegaly. The average relative liver and spleen weights were elevated 2.5- and 4.5-fold, respectively, after 12 weeks of treatment. Liver damage was indicated by hyperbilirubinaemia and a progressive rise in the activity in plasma of sorbitol dehydrogenase, alkaline phosphatase, and gamma-glutamyltransferase. Examination by light and electron microscopy showed increasing numbers of vacuoles, surrounded by a single membrane, in hepatocytes, sinusoidal endothelial cells, and Kupffer cells. Focal cytoplasmic degeneration in hepatocytes was occasionally indicated by formation of autophagic vacuoles and finger print lysosomes. Hepatocytes of 2-deoxy-D-galactose-treated rats showed a dissociation and fragmentation of the rough endoplasmic reticulum. Sinusoidal endothelial cells and Kupffer cells were markedly enlarged, the latter contained a PAS-positive but amylase resistant substance. Extrahepatic changes included an increased occurrence of vacuolated cells in thymus. Phosphate trapping and its metabolic consequences are common phenomena in the experimental injury induced b 2-deoxy-D-galactose and in some hereditary diseases such as uridylyltransferase deficiency galactosaemia, fructose intolerance and glucose-6-phosphatase deficiency.
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PMID:Consequences of recurrent phosphate trapping induced by repeated injections of 2-deoxy-D-galactose. Biochemical and morphological studies in rats. 4 10

Nodular regenerative hyperplasia (NRH) is a condition characterized by hepatocytic nodules distributed throughout the liver without perinodular fibrosis. The etiology is unknown, but it has been reported in patients with a variety of diseases, including autoimmune diseases and primary biliary cirrhosis (PBC). In this study, the liver biopsies of 64 patients with PBC were reviewed. Thirty-five biopsies in histological stages I or II belonging to 30 patients were suitable for study. NRH was found in 43% of biopsies (47% of patients). In 80% of these biopsies, nodular transformation was focal, while it was diffuse in the remaining 20%. Splenomegaly was more common in patients with NRH. Laparoscopy displayed evidence of portal hypertension in two of these patients and endoscopy showed esophageal varices in one patient. Serum levels of gamma-glutamyl transpeptidase were also higher in these patients. Lesions in small intrahepatic vessels, florid bile duct lesions, and portal granulomas were found more frequently in biopsies with NRH. We conclude that nodular hyperplastic changes are very common in early histological stages of PBC, and consequently, this disease should be considered in the etiology of NRH. These changes may contribute to early development of portal hypertension in these patients.
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PMID:Nodular regenerative hyperplasia of the liver in early histological stages of primary biliary cirrhosis. 155 37

A 51-year-old man had for 5 years been known to have erythropoietic protoporphyria. GPT levels were raised up to 40 U/l, gamma-GT up to 120 U/l. After lengthy exposure to sun radiation an erythema with blisters, abdominal discomfort and jaundice developed (total bilirubin 7.3 mg/dl) and biliary liver cirrhosis with portal hypertension and splenomegaly were diagnosed. Because the acute hepatobiliary complications were not improved by conservative treatment (daily 750 mg ursodeoxycholic acid and 12 g colestyramine), an orthotopic liver transplantation was performed without complication. The excised liver showed small nodular parenchymal transformation and contained reddish brown protoporphyrin pigment in the hepatocellular cytoplasm, the Kupffer cells, the canaliculi and in some biliary ducts. Bilirubin and transaminase levels in blood became normal after the transplantation, as did the urinary excretion of coproporphyrin. However, isomer I was still dominant. The protoporphyrin level in erythrocytes and plasma remained elevated. After a symptom-free interval of one year biochemical and histological tests demonstrated protoporphyrin-induced damage in the transplanted liver.
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PMID:[Liver transplantation in erythrohepatic protoporphyria]. 162 35

We have studied a Portuguese family with a dominant beta-thalassaemia trait that was present in one member of each of three generations. It was characterized by a moderate anaemia, microcytosis and hypochromia, anisopoikilocytosis, Heinz body formation in peripheral red cells, splenomegaly, and a blood transfusion requirement during pregnancy. Sequence analyses of amplified DNA detected a deletion of (G) TG.GCT.GGT.GT(G) at codons 134-137 (Val.Ala.Gly.Val) and the insertion of (G)GC.AG(G) (Gly.Arg) at the same location. Thus, the resulting beta chain has an abnormal structure only at codons 134-137 and is two residues shorter than the normal 146 residues. This chain could not be detected in circulating red cells and must be degraded rapidly by proteolysis because the Heinz bodies consisted mainly of alpha chains.
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PMID:Dominant beta-thalassaemia trait in a Portuguese family is caused by a deletion of (G)TGGCTGGTGT(G) and an insertion of (G)GCAG(G) in codons 134, 135, 136 and 137 of the beta-globin gene. 165 62

Seven women, mean age 47.7 years, with primary biliary cirrhosis (6 patients in the II-III stage and I patient in IV stage of the disease) were treated in the course of 16 months with ursodeoxycholic acid (Ursofalk) 500 mg daily. At the end of the 3-d month of treatment the itching had passed in 3 of the patients and in the remaining 4 patients it had substantially decreased. In all patients the subjective complaints, dyspeptic syndrome, appetite and sleep improved. The serum concentrations of bilirubin, copper and cholesterol started to decrease and the serum activity of the enzymes alkaline phosphatase, ALAT and ASAT also decreased. In one patient the treatment was discontinued in the 6-th month because of allergic reaction. After 16 month treatment in the 6 patients who completed the treatment the itching passed and the working capacity improved. The serum concentrations of bilirubin, cholesterol, copper and IgG significantly fell (p less than 0.01), the serum activity of alkaline phosphatase, gamma glutamyl transpeptidase, ALAT and ASAT fell near the upper normal range. The hepatomegaly, splenomegaly, McLagan's flocculation test, serum concentration of IgM and the titer of the specific antimitochondrial antibodies (M2) did not change in spite of the treatment. The results show the ursodeoxycholic acid as a perspective therapeutic means for primary biliary cirrhosis which lowers or overcomes the syndrome of intrahepatic cholestasis and limits the activity of the cirrhotic process in the liver. Ursodeoxycholic acid is well tolerated.
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PMID:[The treatment of primary biliary liver cirrhosis with ursodeoxycholic acid (preliminary report)]. 177 66

Among 203 patients with high altitude erythrocythemia (HAE), 89% had various kinds of alimentary canal symptoms, while these were present only in 31% of a control group (P less than 0.01). 49% of the HAE group had stomach, while only 8% of the control group had it. Endoscopic examination revealed 44% of the HAE group had peptic ulcer, being much higher than 6% of the control group. The ratio of gastric to duodenal ulcer in the HAE group was 2 to 1. The HAE patients had higher values of ALT, total bilirubin, LDH activity, LDH1 and LDHs, but lower values of AKP, LDH2, LDH4 and gamma-GT than the controls. The difference was, however, of no statistical significance. B mode ultrasonography showed that the incidence of hepatomegaly and splenomegaly was 24% and 11% respectively in the HAE group, while it was only 4% and 2% in the control group. HAE patients usually had symptoms of digestive system two years after the onset and the incidence at that time was 67%. With the extended course of the disease, the incidence gradually came down. The mechanism of digestive system impairment in HAE was probed and it was shown that treatment of digestive system impairment would promote the healing of HAE.
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PMID:[Impairment of the digestive system in high altitude erythrocythemia]. 179 46

At present Non-A and Non-B hepatitis disseminated from the intestine in the world is believed to have a better prognosis and has no chronicity. From 1980-1986, this hepatitis has occurred in the south of Xinjiang. It was sporadic (1980-1985) and there was an outbreak (1986). Our study indicated that the results from 500 cases followed up for two years were different from the literature reported. 1. Patients with hepatomegaly were 11.2% at 7th months, 12.8% at 19th months and 45.3% at 28th months. At the same time there were 3 cases of splenomegaly and spider in each of the 19th month and 28th month. 2. Liver function test showed that gamma-GTP, BSP and gamma-globulins rose in different degrees among the 3-7 month cases. Reexamined at 19th months, 3.6% cases of both ZTT and SGPT were high. General proteins of 8% patients dropped. In 42% of the patients the globulins rose and the album in dropped. 3. Biopsy of the liver after 28th months demonstrated that it was in agreement with the pathologic changes found in chronic lobule hepatitis of CPH under the light microscope and electron microscope.
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PMID:[Chronic process with Non-A and Non-B hepatitis disseminated from the intestine (appended 500 cases followed up for two years)]. 190 14

Thirteen workers with persistent abnormalities in one or more liver function tests (LFT) at a vinyl chloride monomer (VCM) polymerization plant were investigated. Twelve workers were found to have VCM-induced liver dysfunction based on circumstantial evidence. They were employed between 1971 and 1982 when the VCM levels ranged from 1 to 21 p.p.m. After 1982 when the environmental VCM levels were controlled to below 1 p.p.m., no cases of VCM-induced liver dysfunction were detected. In most cases, glutamic pyruvic transaminase was the earliest parameter to be raised. The second most common parameter is serum gamma glutamyl transpeptidase. The latent period ranged from 1 to 13 years. An improvement in their LFT results was shown by 83.3 per cent of workers within 6 months to 2 years after removal from further VCM exposure. For workers who returned to VCM work, their LFT became abnormal again. Liver scans showed hepato and/or splenomegaly in most cases. Liver biopsies on 9 workers were reported as 'non-specific fatty changes' of varying degrees. These observations highlight the need for continual vigilance with environmental monitoring and medical surveillance of VCM-exposed workers.
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PMID:Persistent liver dysfunction among workers at a vinyl chloride monomer polymerization plant. 201 Oct

Subacute thyroiditis is generally thought to be a self-limited inflammatory disease of the thyroid gland. This paper describes serial observations on the clinical course of a typical patient with subacute thyroiditis. This patient showed specific features of destructive thyrotoxicosis with increases in the serum levels of acute phase reactants and in the erythrocyte sedimentation rate. She also showed signs of liver dysfunction [slightly increased alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (gamma-GTP), and leucine aminopeptidase (LAP)], slight anemia, glucose intolerance, increased pancreatic enzymes, splenomegaly, and an increase in peripheral Leu 7 positive (NK/K) cells. These abnormalities all improved with recovery from disease. These findings indicate that in this patient with subacute thyroiditis inflammation is not limited to the thyroid gland but also involves the liver, pancreas and spleen. Thus the subacute thyroiditis in this patient may be a systemic multi-organ disease.
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PMID:Subacute thyroiditis associated with systemic multi-organ disorders. 263 13

A retrospective study was designed to analyse the mode of presentation, clinical signs, haematological, biochemical and histological features in 46 Indian patients admitted with cirrhosis to R. K. Khan and King Edward VIII Hospitals, Durban, between 1977-1981. The commonest presenting feature was swelling of the body followed by pain in the right upper quadrant, most patients had hepatomegaly, jaundice and ascites, and splenomegaly was detected in one-third of cases. Biochemical investigations indicated that most patients had a high globulin and low albumin concentration. Liver function tests revealed raised bilirubin and gamma-glutamyltransferase values in most cases. On histological examination, micronodular cirrhosis predominated (95%) with a high incidence of fat and iron deposition. Changes consistent with alcoholic hepatitis were superimposed in one-third of cases while immunological and viral markers were absent. This study suggests that alcohol is the predominant cause of cirrhosis in Natal Indians.
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PMID:Patterns of cirrhosis in Natal Indians. 320 19


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