Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We studied endocrine functions at baseline and after TRH and LHRH stimulation in a group of 7 young male patients with genetic hemochromatosis (HE) without liver damage (i.e. fibrosis and cirrhosis). In five patients endocrine re-evaluations after complete iron depletion was also performed. Mean basal testosterone (T), FSH, LH and PRL were significantly lower than in controls. Serum T increased normally after HCG stimulation. The normal or high increments of LH after LHRH stimulation suggest that secretion capacity of LH was intact and that hypothalamic dysfunction could be responsible for the preclinical gonadal deficiency found in our patients. The response of PRL to TRH indicates that secretion capacity of lactotrophs although present, was decreased and did not improve after phlebotomy therapy. After iron depletion the two patients with the lowest basal T levels showed the highest increments indicating that in the early stages of hypothalamic-pituitary damage gonadal dysfunction is still reversible in HE patients.
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PMID:Preclinical hypogonadism in genetic hemochromatosis in the early stage of the disease: evidence of hypothalamic dysfunction. 140 47

To study the effects of cirrhosis on serum inhibition of Escherichia coli, cirrhosis with ascites was induced in male Sprague-Dawley rats by intragastric administration of carbon tetrachloride. Heat-inactivated (56 degrees C for 30 minutes) serum from cirrhotic rats (CRS) or that from control rats (NRS) was inoculated with 1 x 10(5) colony-forming units per milliliter (CFU/ml) of E. coli, and growth was measured after 24 hours. The mean growth of E. coli in CRS was significantly higher than growth in NRS: 3.5 +/- 5.4 x 10(8) CFU/ml versus 1.2 +/- 2.0 x 10(6) CFU/ml, respectively (p < 0.01). Fifty-four percent of CRS samples (22/41) completely lacked bacteriostatic activity. These CRS samples were categorized as growth-supporting (G+CRS) because their growth exceeded the mean + 2 SD of NRS (5.2 x 10(6) CFU/ml). Serum bacteriostasis could be restored to G+CRS by adding purified rat apotransferrin (1 mg/ml), suggesting the presence of excess iron in G+CRS. However, serum iron concentration (SI) and total iron binding capacity (TIBC) were virtually the same in G+CRS (SI = 120 +/- 22 micrograms/dl; TIBC = 351 +/- 45 micrograms/dl) as in growth-inhibitory CRS (SI = 131 +/- 16 micrograms/dl; TIBC = 347 +/- 46 micrograms/dl) but were significantly less than NRS (SI = 208 +/- 29 micrograms/dl; TIBC = 533 +/- 57 micrograms/dl), p < 0.01. The percent transferrin saturation was similar in all groups: 34% +/- 6%; 38% +/- 5% and 39% +/- 9%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Cirrhosis impairs serum bacteriostasis for Escherichia coli. 140 39

We report a case of neonatal hemochromatosis in which the genetic counseling was initiated by, and based on, retrospective pathologic diagnosis. Perinatal or neonatal hemochromatosis is beginning to be recognized as a distinct clinical entity and one of the most common causes of perinatal cirrhosis. The exact mechanism of liver damage and the relationship to adult type hemochromatosis have not been fully clarified. The pattern of fibrosis in the liver plus the abundant iron deposition in the liver and other organs separate this entity pathologically from other causes of neonatal liver failure. We report on a case of neonatal hemochromatosis that was diagnosed on retrospective autopsy review of an infant with supposed hereditary tyrosinemia, when the family presented for genetic counseling. This case emphasizes to the genetic counselor and pathologist the need to consider the diagnosis prenatally, after birth, or at death, as failure to do so would result in the inability to identify families at genetic risk for neonatal hemochromatosis or in mislabeling a family with another inborn error.
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PMID:Neonatal hemochromatosis: genetic counseling based on retrospective pathologic diagnosis. 144 81

A retrospective study of 127 patients with untreated homozygous genetic hemochromatosis (HGH) was conducted to evaluate the respective roles of iron overload and non-iron-related factors in the development of hepatic fibrosis in HGH. Twenty-seven percent of the patients had cirrhosis, 21% had liver fibrosis and 52% had no fibrosis (prefibrotic group). The mean value of liver iron concentration was increased significantly (p < 0.001) in cirrhotic (378 +/- 144 mumol/g dry wt.) and in fibrotic (331 +/- 168) subjects compared to prefibrotic (237 +/- 108) patients. Of 13 patients with liver iron concentration > or = 500, 12 had liver fibrosis or cirrhosis, versus 48/134 with liver iron concentration < 500. Chronic alcoholic men exhibited hepatic fibrosis or cirrhosis more frequently than non-alcoholic men (p < 0.001). Non-alcoholic men had hepatic fibrosis or cirrhosis more often than non-alcoholic women (p < 0.05). Cirrhotic and fibrotic patients were significantly older than prefibrotic patients whilst a significant correlation between age and liver iron concentration was found in younger patients only. These results suggest that the iron overload threshold necessary to induce fibrosis is modulated by non-iron-related factors such as alcoholism, sex and age. The development of fibrosis in HGH with liver iron concentration < 500 mumol/g is frequent and must lead to a search for associated non-iron-related fibrogenic factors.
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PMID:Liver fibrosis in genetic hemochromatosis. Respective roles of iron and non-iron-related factors in 127 homozygous patients. 148 46

We evaluated the prevalence of hepatitis B virus (HBV) and hepatitis C virus (HCV) infection in 78 Italian patients with hereditary hemochromatosis as well as the relation between HCV antibody (anti-HCV) status, hepatitis B surface antigen (HBsAg) and liver histology. None of the patients had been transfused or ever consumed more than 60 g of alcohol per day. Eighteen showed histological signs of chronic hepatitis, active cirrhosis was present in 12, chronic active hepatitis in 4 and chronic persistent hepatitis in 2. Liver fibrosis or cirrhosis without inflammatory activity was observed in 31 subjects, whereas liver histology was normal except for iron overload in 18. The prevalence of HBsAg in the whole series was 5% and of anti-HCV was 20.5%. The prevalence of HBsAg and anti-HCV was significantly higher in the chronic hepatitis group than in the fibrosis/cirrhosis (p = 0.01) and the normal groups (p < 0.01). Fourteen of 18 hereditary hemochromatosis patients with chronic hepatitis were HBsAg (4) or anti-HCV (10) positive and all the latter subgroup had HCV-RNA in their serum as shown by the polymerase chain reaction. Although most of the patients with associated chronic hepatitis had cirrhosis, their serum ferritin levels and amount of mobilizable iron were significantly lower than those of the fibrosis/cirrhosis group (p < 0.01). This indicates that hepatitis viral infection acts synergistically with iron in accelerating the development of liver damage.
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PMID:Liver damage in Italian patients with hereditary hemochromatosis is highly influenced by hepatitis B and C virus infection. 148 15

A 56-year-old male patient on chronic hemodialysis developed liver cirrhosis. He received a total of 20 liters of blood transfusion. Bronze pigmentation of the skin and iron deposition to the liver, spleen, pancreas and thyroid gland, which was demonstrated by computed tomography and magnetic resonance imaging studies, and histological demonstration of iron deposition to the thyroid gland, bone marrow and gastric mucosa established a diagnosis of secondary hemochromatosis. Endocrine work-up revealed the presence of diabetes mellitus with minimum insulin secretory response, primary (or thyroprivic) hypothyroidism, hypoparathyroidism and hypogonadotropic hypogonadism. A wide-spread endocrine involvement as seen in this patient is a rare clinical feature of hemochromatosis secondary to massive blood transfusion in hemodialysis patients. Particularly, primary hypothyroidism due to iron deposition to the thyroid gland was quite a rare feature of hemochromatosis.
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PMID:Primary hypothyroidism and multiple endocrine failure in association with hemochromatosis in a long-term hemodialysis patient. 151 78

Forty eight patients with liver cirrhosis and portal hypertension have been examined, most of them had hypochromic anemia. Serum iron and ferritin levels, total and latent iron binding capacity have been radioimmunoassayed. All the patients developed hyposiderosis, the study of liver and spleen bioptates showed tissue iron deficiency. It has been established that measurement of ferritin, total and latent iron binding capacity were not informative. It is iron concentration that should be determined. Intravenous administration of high doses of the drug Ferrum-lek seems most effective. It recovers red blood count, iron metabolism and iron tissue stores and reduces the incidence of pyogenic and cardiovascular complications.
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PMID:[Perioperative correction of hypochromic anemia and iron metabolic disorders in patients with liver cirrhosis and portal hypertension]. 152 42

Erythrocyte antioxidant enzymes (superoxide dismutase, catalase, glutathione peroxidase) and reduced glutathione, serum ceruloplasmin, and serum trace elements (copper, zinc, iron, and selenium) related to antioxidant enzymes were assayed in subjects with alcoholic liver disease of different degrees of severity. The erythrocytes of subjects with moderate and severe alcoholic liver cirrhosis had an unbalanced antioxidant system (normal superoxide dismutase, low catalase and glutathione peroxidase activities, and low glutathione content). Serum ceruloplasmin levels were in the normal range. Levels of the serum trace elements zinc and selenium were significantly low in subjects with moderate and severe cirrhosis, whose red cell half-life was also significantly short, as measured by radioactive chromium. These data suggest that the erythrocytes of subjects with moderate and severe alcoholic liver cirrhosis are less protected against oxidant stress. The particular erythrocyte antioxidant system and serum trace element pattern may play a role in the genesis of hemolytic disorders and of alcoholic hepatic damage.
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PMID:Erythrocyte antioxidant activity, serum ceruloplasmin, and trace element levels in subjects with alcoholic liver disease. 837 44

An MR study was performed in 73 clinically diagnosed cirrhotic patients to determine correlations among the demonstration of small low intensity nodules (SLINs), secondary changes due to cirrhosis, and serological data. In 32 patients, liver cirrhosis was proved histologically. SLINs were observed in 38 of the 73 patients on gradient echo (GRE) images and in 28 patients on T2-weighted SE images. Patho-MR correlation study of the liver in the 32 histologically proved cirrhotic patients revealed that SLINs were seen on GRE images only in patients with iron deposits in regenerating nodules (RNs). There was no significant correlation between secondary changes due to liver cirrhosis and the demonstration of SLINs. Serological data indicating liver cell injury and iron store in the body were significantly higher in patients with iron deposits in RNs than in those without iron deposits.
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PMID:[Regenerating nodules in liver cirrhosis--patho-MR-serological correlation study]. 157 25

Evaluation of chronic liver disease begins with a carefully taken history, thorough physical examination, and standard laboratory tests. Often, however, other studies are required, such as a viral hepatitis panel, serologic tests for autoimmune markers, tests for antimitochondrial antibodies, measurement of serum iron and ceruloplasmin levels, liver biopsy, and imaging studies of the extra-hepatic bile ducts. Medical treatment of chronic active hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis remains unsatisfactory. Early treatment of hemochromatosis and Wilson's disease can prevent cirrhosis and liver failure. Liver transplantation is now a viable procedure for patients with end-stage chronic liver disease.
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PMID:Chronic liver disease. The scope of causes and treatments. 158 71


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