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)

A four-month-old child with non-ketotic hypoglycemia and rapidly progressive cirrhosis excreted in her urine large amounts of two unidentified organic acids in addition to a spectrum of saturated, unsaturated, and 3-hydroxy dicarboxylic acids in her urine. Gas chromatography/mass spectrometry of the trimethylsilyl derivative of one of the unknown compounds suggested the structure of 3-hydroxyoctanoic acid, which was confirmed by similar analysis of the authentic compound. The same organic acid was found in the child's plasma. The significance of 3-hydroxyoctanoic aciduria as a possible marker for a primary defect of 3-hydroxy fatty acid metabolism is discussed.
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PMID:3-Hydroxyoctanoic aciduria: identification of a new organic acid in the urine of a patient with non-ketotic hypoglycemia. 316 81

We report two sisters with neonatal hemochromatosis (NHC), including the first documented survivor. Characterized by excessive parenchymal iron in liver, pancreas, heart, and other organs, but little iron in the spleen, bone marrow, or other sites of the reticuloendothelial system, NHC is rarely reported and has been uniformly fatal. The first infant (case 1) presented with neonatal hypoglycemia, coagulopathy, and mild hyperbilirubinemia; she rapidly deteriorated and died of multisystem failure. Autopsy showed cirrhosis. Her sister (case 2) presented similarly; liver biopsy showed giant cell hepatitis, which is consistent with idiopathic neonatal hepatitis (INHP). However, iron staining revealed that case 1 had extensive iron deposits in the liver, pancreas, heart, thymus, and bone, but none in bone marrow or spleen. Case 2 had grade 4 liver iron staining, normal bone marrow iron, elevated serum ferritin and transferrin saturation, and HLA-A3 haplotype. At 16 months of age, the growth, development, and serum measures of iron status in case 2 were normal; liver biopsy showed fibrosis, negative iron staining, and normal tissue iron concentration. NHC is compatible with survival, has clinicopathologic features that overlap with INHP, and may frequently be misdiagnosed as INHP. A prospective study is needed to determine the incidence and natural history of NHC--a disorder that may be more common than is currently recognized.
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PMID:Familial neonatal hemochromatosis with survival. 333 84

In the past 10 years we have examined 20 children with inflammatory liver disease associated with high serum titers of anti-liver-kidney microsome antibody (anti-LKM). The first hepatic symptoms were progressive fatigue and jaundice, the fortuitous finding of hepatomegaly or splenomegaly with raised transaminase activity, or an acute hepatitis-like illness. At the time of diagnosis, hepatomegaly was present in 18 children, splenomegaly in 16, jaundice in nine, and ascites in two. Serum alanine transferase activities were elevated in all but two, who had already received steroids. Serum total gammaglobulin values were greater than 2.0 gm/dl in 16 children, prothrombin activity less than or equal to 60% in six, and serum titer of anti-LKM between 1:100 and 1:100,000. All children but one had cirrhosis, and histologic signs of aggressivity were present in 14. In 11 children one or more extrahepatic diseases were present, including type 1 diabetes, vitiligo, glomerulonephritis, autoimmune hemolytic anemia, hypoglycemia with hyperinsulinism, autoimmune thyroiditis, chronic mucocutaneous candidiasis with hypoparathyroidism, and multiple cutaneous and visceral telangiectasias. Treatment with prednisone and azathioprine improved the liver condition in 16 of the 18 patients given treatment. In eight of them discontinuation of treatment resulted in rapid relapse; 14 are still receiving treatment and have stable hepatic function with follow-up from 8 months to 6 1/2 years. Only two are free of treatment. Four children died, two in spite of immunosuppressive therapy, one during a relapse, and one of extrahepatic disease. These results indicate that this autoimmune inflammatory liver disease may have onset early in life, with several clinical patterns; is frequently associated with certain types of extrahepatic manifestations of autoimmune origin; and is a potentially fatal disease for which immunosuppressive treatment must be started early.
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PMID:Liver disease associated with anti-liver-kidney microsome antibody in children. 395 Aug 19

We experienced a 68 years-old male who had frequent hypoglycemic attacks. The diagnosis of a giant hepatoma associated with hypoglycemia was established by oral glucose tolerance test (O-GTT), angiography and computed tomography. The cavography demonstrated the invasion or depression of the tumor. It was ascertained by an operative exploration that the tumor occupied most of the right trisegments of the liver and infiltrated into a part of the left lateral segment. The right hepatic trisegmentectomy along 1.5cm left side line apart from the falciform ligament was performed. Resected tumor showed 11 X 13 X 10cm in size and 1200g in weight. Microscopic findings demonstrated hepatocellular carcinoma as Edmondson's Type II. After the operation, this patient became free from the hypoglycemic attack. A fasting blood sugar became within normal limits and O-GTT demonstrated a normal blood sugar level and insulin response. No concentration of immuno-reactive insulin was found in the resected tumor, however, the blood sugar in rats which received the intravenous injection of the tumor extracts was remarkably decreased in 20 minutes. From these observation, hypoglycemia in this patient seemed to depend mainly on the factor of insulin-like activity of the tumor. In the literature, the hepatocellular carcinoma associated with paraneoplastic phenomenon, e.g. hypoglycemia, has rarely been resected because of accompanied liver cirrhosis or giant size of the tumor. This is the second patient in our country in whom the tumor was fortunately resected and the paraneoplastic syndrome disappeared postoperatively.
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PMID:[Successful resection of giant hepatocellular carcinoma with hypoglycemic attack]. 609 7

Problems raised by major hepatobiliary surgery affect the total economy of the human body. The liver is implicated in all body metabolism processes and possible problems during partial or total hepatectomy can only be solved by a knowledge of liver physiology. The liver plays a major role in the metabolism of products of digestion, whether these are carbohydrates or amino acids arriving in the portal blood. The most important activity of the liver is the preservation of a constant level of glucose by ensuring a fixed and predictable concentration to extrahepatic tissues. It ensures a very precise control of plasma and tissue flow of amino acids and thus protein synthesis and neoglycogenesis. Synthesis and degradation of non-esterified fatty acids, ketogenesis, cholesterol synthesis and triglyceride production result from the action of the liver on lipid metabolism. Free cholesterol is the precursor of bile acids and steroid hormones, but esterified cholesterol is not synthetized in the liver. Apart from its role in bilirubin metabolism, it has a key role to play in correct functioning of most endocrine systems: many are catabolized in the liver. But one of the most interesting properties of the hepatic tissue, as far as the surgeon is concerned, is liver regeneration, which combines hypertrophy with hyperplasia. This is dependent on age, hepatotrophic factors of portal blood, and extraportal factors. A study of hepatic metabolic processes allows assessment of the consequences of partial hepatectomy. Postoperative hypoglycemia, in the absence of a continuous infusion of glucose, is easily explained by the weakness of hepatic reserves in glycogen. Albumin levels fall during the first 7-10 days after liver resection, but this hypo-albuminemia is often marked by the need to infuse large quantities of frozen fresh plasma to try to avoid lesions of the other specific proteins, which are coagulation factors. Lipid metabolism disorders are of little clinical consequence. Hepatic resection is being alarming when it involves 80 to 90% of the hepatic mass and menaces the life of the patient. The existence of a previous liver alteration worsens the consequences of major hepatobiliary surgery. Indications for hepatic resection must be weighed carefully in patients with cirrhosis, liver regeneration being totally absent after resection. Metabolic consequences of total hepatectomy followed by transplantation are identical in kind to those of partial hepatectomy but are increased in frequency and start during operation. Postoperative surveillance must be strict to avoid marked variations in glycemia with the risk of hypoglycemia, and variations in kaliemia.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Hepatic insufficiency and nutritional problems after major hepatobiliary surgery]. 643 25

Analyses of 23 fatal instances of hepatic injury in patients taking valproic acid reveals that all but three were less than 20 years old, and all but four had been taking the drugs for more than 1 month. Convulsions, facial edema, lassitude, and vomiting were prominent clinical features. Hypoglycemia was recorded in six patients. Rash and eosinophilia were not seen. Values for transaminases were modestly elevated in most patients. Most levels of SGOT were below 500 IU, and SGPT levels were below 200 IU. Livers showed microvesicular steatosis in most patients, usually accompanies by necrosis. Four patients had cirrhosis. Overt valproic acid-induced hepatic injury appears to be rare and hence, by definition, idiosyncratic. That it may be an idiosyncratic exaggeration of a much more frequent phenomenon is suggested by the higher incidence of seemingly trivial injury. The idiosyncrasy appears to be metabolic rather than immunologic, and the available information leads to the plausible hypothesis that a metabolite is responsible for the microvesicular steatosis seen in most fatal cases. The steatosis resembles that of Reye's syndrome and Jamaican vomiting sickness, and there is reason to believe that the metabolite responsible for the steatosis resembles the agent responsible for Jamaican vomiting sickness. A different metabolite is presumably responsible for the necrosis seen in many of the cases.
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PMID:Valproate-induced hepatic injury: analyses of 23 fatal cases. 681 94

A patient with biopsy-proved biliary cirrhosis and previous gastrojejunostomy and portacaval anastomosis experienced episodes of severe hypoglycemia. She was found to have hyperinsulinemia and hyperglucagonemia. An oral glucose tolerance test showed postgastrectomy hypoglycemia. Results of the intravenous tolbutamide test were diagnostic for insulinoma, but results of the intravenous glucagon test and prolonged fast (96 hours) were not. Failure, on two occasions, to suppress C-peptide normally during insulin-induced hypoglycemia led to a diagnosis of pancreatogenous hyperinsulinemia. The pancreas showed a 10-fold increase in islet volume, with intensely positive staining with anti-insulin and anti-glucagon antiserums in addition to anti-somatostatin and anti-pancreatic polypeptide antiserums. Incidental findings at pancreatic exploration were a mesothelioma, which did not stain with anti-insulin antiserum, and, at autopsy one year later, a hepatoma.
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PMID:Diagnosis of pancreatic islet hyperplasia causing hypoglycemia in a patient with portacaval anastomosis. 699 72

Asymptomatic hypoglycemia was demonstrated in 15 of 30 cirrhotic patients with septicemia. Blood glucose levels were measured daily in these patients. Severe circulatory failure was present in the 15 patients with hypoglycemia and was absent in the 15 patients with normal blood glucose levels. Hypoglycemia is a common complication of septic shock in patients with cirrhosis, and blood glucose levels should be systematically measured in cirrhotic patients with septicemia or shock. Septicemia should be considered in any cirrhosis patient with a low blood glucose level.
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PMID:Hypoglycemia. A common complication of septicemia in cirrhosis. 728 59

Four patients had resection for primary hepatic sarcoma: one with malignant fibrous histiocytoma (MFH), two with poorly differentiated fibrosarcoma, and one with leiomyosarcoma. Age ranged from 40 to 69 years. One patient had a cousin and a grandmother who had died of hepatic tumors. At presentation, all patients had pain; one had tumor rupture, and one had mental changes and hypoglycemia. None had hepatitis or cirrhosis. Results of laboratory evaluation were nonspecific, including normal carcinoembryonic antigen and alpha-fetoprotein levels. Computed tomography showed hypodense masses with enhancement. Angiography showed a hypervascular mass in three patients and an avascular mass in the patient with MFH. Despite large tumors (8 to 32 cm), portal and hepatic veins were not invaded. The pattern of vascularization and lack of venous invasion helps differentiate primary hepatic sarcomas from hepatocellular carcinoma, especially in noncirrhotic patients. All patients had extensive hepatic resections, with one operative death. Immunohistochemical stains of the tumors were positive for vimentin but negative for epithelial markers, differentiating these lesions from other hepatic tumors. The patient with MFH died with recurrence at 10 1/2 months. The patient with the ruptured fibrosarcoma had a second resection and chemotherapy, but died with recurrence at 3 years. The patient with the leiomyosarcoma had a second resection and was disease free at 4 years. Resection of primary hepatic sarcoma is warranted, with potential survival measured in years.
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PMID:Resection of primary hepatic malignant fibrous histiocytoma, fibrosarcoma, and leiomyosarcoma. 751 Sep 7

Hypoglycemia in fulminant hepatic failure and hyperinsulinemia in cirrhosis are well-described phenomena. A patient with alcoholic cirrhosis who developed fasting hypoglycemia with an extremely high immunoreactive insulin level and a mildly elevated C-peptide level is reported. An insulinoma was excluded by detailed radiological imaging of the pancreas and by endoscopic ultrasonography. Detection of very high levels of insulin autoantibodies with no prior exposure to exogenous insulin confirmed the diagnosis of insulin autoimmune syndrome. During his hospital course, the patient developed another rare syndrome, acquired inhibitors to factor V, which led to the fatal coagulopathy that resulted in his death. Insulin autoimmune syndrome is the third leading cause of spontaneous hypoglycemia in Japan, where it has been associated with a variety of diseases and drugs. Outside of Japan, only approximately 20 cases have been reported and usually have been found in the context of an underlying autoimmune disorder or prior exposure to sulfhydryl drugs. It is believed that this is the first case reported outside Japan occurring in association with alcoholic liver disease, and the first in the world with coexisting acquired inhibitors to factor V.
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PMID:Insulin autoimmune syndrome as a cause of spontaneous hypoglycemia in alcoholic cirrhosis. 755 52


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