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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intraperitoneal deferoxamine is a well established treatment for aluminum accumulation syndrome in patients with end-stage renal disease receiving peritoneal dialysis, but the use of intraperitoneal deferoxamine has not been described outside of the setting of chronic renal failure. We present here a case of secondary hemochromatosis, complicated by cirrhosis and cardiomyopathy, in which a chronic peritoneal dialysis catheter was used both to treat ascites and to deliver parenteral deferoxamine for iron overload. Daily urinary iron excretion was similar to that achieved when using standard routes of deferoxamine administration. Over a 2-year period, reversal of both the biochemical indicators and the clinical manifestations of iron overload was accomplished.
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PMID:Long-term intraperitoneal deferoxamine for hemochromatosis. 862 76

Hemochromatosis is an autosomal-recessive disease which causes iron-overload of various organs including liver, pancreas and heart. This report analyzes the course of hemochromatosis in two patients (a 28-year-old man and a 57-year-old woman) in whom hemochromatosis was detected because of severe cardiomyopathy. Initial symptoms were edema, anasarca and dyspnea. Further examinations showed pleural effusion, decreased left-ventricular-function, skin pigmentation, diabetes mellitus and liver cirrhosis. Although phlebotomy treatment and iron-chelation therapy with deferoxamine initially resulted in some improvement, both patients died from cardiomyopathy three months after diagnosis. The reports of these two cases underline that hemochromatosis-associated cardiomyopathy is often irreversible if severe congestive heart failure is present. In cardiac decompensation heart transplantation has to be considered as early as possible.
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PMID:-Cardiomyopathy as the cause of death in genetic hemochromatosis-. 865 Sep 71

Administration of vitamins or metals may cause severe side effects. Retinoids (derivatives of vitamin A) used for the treatment of various skin disorders are teratogenic, hepatotoxic and may induce a substantial increase in serum lipids. A case report demonstrates that vitamin D supplementation in a patient under total parenteral nutrition can cause hypercalcemia. The isolated administration of vitamin B1, without concomitant vitamin B6 and nicotinamide may precipitate potentially life-threatening pellagra encephalopathy. Repeat blood transfusions may produce clinically overt organ hemosiderosis, e.g. cirrhosis of the liver, diabetes mellitus or myocardiopathy. The literature contains reports on a few cases of sarcoma associated with orthopedic metal implants. The controversial issue of the potential dangers of dental amalgams is briefly mentioned.
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PMID:[Vitamins and metals: possible hazards for humans]. 866 74

The aim of this study was to identify the risk factors of alcohol-related peripheral neuropathies. A case-control study was performed to compare two groups of alcoholic patients, one with peripheral neuropathy and the other without, but with alcohol-related cirrhosis, pancreatitis or cardiomyopathy. Ninety patients were recruited in four in-patient units of a French hospital: 32 patients had a peripheral neuropathy and 58 patients did not. Univariate analysis showed no differences between the two groups for sex, age, body mass index and duration of the alcoholic disease. Peripheral neuropathies were associated with a higher frequency of parental history of alcoholism, severity of alcohol dependence, heavier alcohol consumption and more alcohol-related somatic diseases. Multivariate analysis showed a strong relationship between a parental history of alcoholism and the presence of a neuropathy, when the severity of the alcoholic disease was taken into account (adjusted OR = 6.8, IC95% [2.2-21.6], P < 0.001). The hypothesis that neuropathy may be a marker of an inherited susceptibility to alcoholism is discussed.
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PMID:Parental history of alcoholism: a risk factor for alcohol-related peripheral neuropathies. 867 15

In cirrhosis, cardiac contractile function has been extensively documented to be abnormal. At baseline, cardiac output is increased, and this is one of the characteristics of hyperdynamic circulation. However, when cirrhotic patients are challenged by pharmacological or physiological stress, ventricular hyporesponsiveness is revealed. Similar patterns have been noted in cirrhotic animal models. This phenomenon has been termed "cirrhotic cardiomyopathy." Although alcohol abuse may contribute to some cases of cirrhotic cardiomyopathy, it has been clearly documented to occur even in the absence of alcohol ingestion. Diminished myocardial beta-adrenergic receptor signal transduction function, possibly caused by a persistent elevation in norepinephrine content, has been shown to play an important role. Alternation in cardiac plasma membrane properties due to impaired lipid metabolism is also crucial. Other possible pathogenic factors are reviewed, including accumulation of cardiodepressant substances caused by hepatocellular insufficiency, and ventricular overload secondary to increased blood volume and hyperdynamic circulation. Because the cardiac reserve function is borderline in patients with cirrhosis, cardiovascular status should be carefully monitored, especially when patients undergo stresses such as liver transplantation or portosystemic shunting procedures.
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PMID:Cirrhotic cardiomyopathy: getting to the heart of the matter. 869 Apr 19

Recipient vessels from the head and neck region were histologically examined in 30 patients who had undergone extensive tumor resection necessitating microvascular tissue reconstruction. Past illnesses considered to be risk factors included hypertension, myocardial disease, lung disease, cirrhosis, diabetes, nephritis, as well as extensive nicotine and alcohol abuse. Blood vessels chosen for microsurgical anastomosis were exclusively examined histologically in this study. Patients undergoing microvascular surgery demonstrated vessel abnormalities in 93%. The frequency of dysplasia was higher in the arteries than in the veins (73% and 26%, respectively). Marked thickening of the blood vessel wall and severe exfoliation of the endothelial cells were observed in most arteries. Fibrodysplasia and exfoliated endothelial cells were more frequently observed in the recipient arteries than the graft arteries. Only slight thickening of the vessel wall and mild fibrodysplasia were seen in the veins. Two graft failures were correlated to technical errors rather than pre-existing vessel lesions. This study revealed that most patients undergoing microsurgery in the head and neck region demonstrate pre-existing damage in vessels, which generally hinders anastomosis. Although the study tried to identify fully the interrelationships between the extent of dysplasia, past medical history, preoperative therapy, risk factors and factors that cause free-graft failure, pre-existing changes in the recipient and graft vessels may cause technical difficulties and must be regarded as additional factors contributing to graft failure.
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PMID:Histological changes in vessels used for microvascular reconstruction in the head and neck. 870 38

12 autopsy cases of primary hemochromatosis were studied: 11 males (aged between 29 and 69 years, mean age 51 years) and 1 57-year-old female. Clinical course in 6 patients ran with predominant liver damage to the small nodule or was of biliary cirrhosis type, in 3 cases main manifestation was diabetes mellitus and in 3 cases cardiomyopathy. Generalized pattern of hemosiderine deposits with involvement of the liver, pancreas, heart, adrenals, pituitary, epiphysis, thyroid and other organs was established by the Pearl's test. Apart from pigment degeneration, atrophy and fibrosis, compensatory and regenerative changes were found. These changes should be taken into account when the disease is analyzed clinically and morphologically.
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PMID:[Clinico-morphologic characteristics of primary hemochromatosis]. 874 87

Alcoholism may lead to a great many physical and mental problems in individuals of any age. Elderly alcoholics often have additional problems resulting from the interaction of age related changes in physiology and "heavy" alcohol intake. Some of the more important problems are: Impairment of the immune system with decreased ability to deal with infection or cancer. Increased incidence of hypertension, cardiac arrhythmia, myocardial infarction, and cardiomyopathy. Increased incidence of stroke. Alcohol dementia. Increased incidence of esophageal and other cancers. Cirrhosis and other liver disease. Malnutrition. There seems to be no area in which even moderate alcohol intake is of definite benefit, and some areas in which even small amounts are detrimental.
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PMID:Medical manifestations of alcoholism in the elderly. 875 18

Type III glycogen storage disease (GSD) is a disorder of carbohydrate metabolism caused by a deficiency of debranching enzyme. Different subtypes with different clinical pictures have been recognized. During childhood and early adulthood, the symptoms generally regress, and normal adulthood appears possible in most patients without symptoms or signs of cirrhosis. We report on an adult patient with GSD who developed endstage cirrhosis and a small hepatocellular carcinoma. She had GSD subtype IIIb, i.e., there were no signs of cardiomyopathy, myopathy, or neuropathy. She underwent a successful transplantation, representing the first case treated this way for this indication to our knowledge, and she is doing well after 1 year. Debranching enzyme activity was absent both in the liver and in the leukocytes before transplantation. The debranching enzyme activity remained absent in the leukocytes after transplantation. We conclude that patients with GSD type III may develop end-stage cirrhosis and hepatocellular carcinoma and therefore need hepatological follow-up during adulthood.
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PMID:Type IIIb glycogen storage disease associated with end-stage cirrhosis and hepatocellular carcinoma. The Liver Transplant Group. 904 94

Hereditary hemochromatosis (HHC) is an inherited disease transmitted in an autosomal recessive pattern. With homozygosity occurring in up to 0.5% of the population, HHC is the most prevalent genetic disease among the white population worldwide and has the same prevalence as the sickle cell trait in the African-American population. An asymptomatic 50-year-old white man presented at the family practice clinic and stated that HHC had been diagnosed in his mother. Laboratory findings showed markedly elevated transferrin saturation and ferritin levels. The diagnosis of HHC was made on the basis of the laboratory results and family history, and therapy was begun. Clinical manifestations of HHC occur late and include diabetes mellitus, cirrhosis, and cardiomyopathy. As end-organ damage is preventable, optimal management involves early diagnosis and lifelong phlebotomy. Diagnosis is made by an elevated transferrin saturation level and an increased serum ferritin value. Hereditary hemochromatosis is a genetic disorder of iron metabolism that has an excellent prognosis if diagnosed early.
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PMID:Hereditary hemochromatosis. 907 Dec 52


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