1/45. Late manifestation of Indian childhood cirrhosis in a 3-year-old German girl.We report on a 3.8-year-old German girl who presented with signs of subacute liver failure based on a 4-month history. Investigations done before admission excluded infectious, metabolic and immunological diseases. Laboratory findings were increased values of aminotransferases, hyperbilirubinaemia, hyperammonaemia and deteriorated plasmatic coagulation. Caeruloplasmin and serum copper concentrations were normal; however, urinary copper excretion was elevated (80 microg/l). Liver biopsy showed a micronodular liver cirrhosis and an extremely high liver copper concentration (1400 microg/g dry weight). Epidemiological investigations revealed an elevated copper concentration (8645 microg/l, normal: <2000) and a low pH value (6.3) of the drinking water supplied by copper pipes. The girl had been exposed to copper-contaminated drinking water since the age of 2 years. CONCLUSION: Laboratory, histopathological findings and a proven chronic copper intoxication lead to the diagnosis of Indian childhood cirrhosis in a German girl. Whereas this disease is mostly described in patients with increased copper intake in infancy, our patient developed toxic liver cirrhosis with relatively late copper exposure. Indian childhood cirrhosis should be considered in the differential diagnosis of early childhood liver cirrhosis.- - - - - - - - - - ranking = 1keywords = water, intake (Clic here for more details about this article) |
2/45. copper and the liver: late lessons from the Camperdown experience.In 1973 copper-associated liver disease was first described in an Australian boy of non-Indian origin who died at the age of 15 months with liver cirrhosis. At necropsy the hepatic copper concentration was excessively high (> 300 mg/g dry weight). It was shown that the boy s disease was caused by consumption of copper contaminated drinking water.- - - - - - - - - - ranking = 4.7260787945714keywords = consumption, water (Clic here for more details about this article) |
3/45. copper toxicosis in an Australian child.A 20 month old Caucasian child living in rural australia presented with liver failure after exposure from birth to milk formula made from acidic bore water containing excess copper leached from the copper piping. The liver pathology was identical to that seen in Indian childhood cirrhosis and similar disease in non-Indian children now termed idiopathic copper toxicosis (ICT). The only other Australian case, reported more than 20 years previously, had identical presentation, pathology and circumstances of occurrence. The rarity of ICT in australia, despite a significant population at risk, and the implications of these cases are discussed.- - - - - - - - - - ranking = 0.33573062623088keywords = water (Clic here for more details about this article) |
4/45. Transjugular intrahepatic portosystemic shunt for recurrent hepatic hydrothorax.For many years, pleural effusions have been recognized as a complication of cirrhosis, occurring in approximately 5.5% of patients. Recent studies have confirmed that small defects in the diaphragm allow for passage of ascitic fluid into the pleural space. Successful management of these patients is challenging, as many of the treatment options can be associated with increased morbidity. The initial treatment should focus on eliminating and preventing the recurrence of ascites with diuretics and water and salt restriction. For those patients who do not respond medically, more invasive techniques have been used including serial thoracentesis, chest tube placement, chemical pleurodesis, and peritoneovenous shunts. We present a patient with recurrent pleural effusions secondary to hepatic cirrhosis who was unsuccessfully treated medically, and subsequently treated with thoracentesis, chest tube drainage and pleurodesis, with ultimate resolution after transjugular intrahepatic portosystemic shunt placement.- - - - - - - - - - ranking = 0.33573062623088keywords = water (Clic here for more details about this article) |
5/45. vitamin e deficiency and platelet functional defect in a jaundiced infant.A 16-month-old infant with hepatic fibrosis, cholestasis, and chronic jaundice had signs of vitamin e deficiency, including mild acanthocytosis, thrombocytosis, increased peroxide haemolysis, and absent serum vitamin E. Abormal prothrombin consumption and platelet restocetin aggregation suggested the presence of defective platelet function, and correction studies indicated that this was due to a plasma defect. The abnormality was corrected by treatment with vitamin E, and the findings suggest a possible role of this vitamin in platelet reactions.- - - - - - - - - - ranking = 4.3903481683406keywords = consumption (Clic here for more details about this article) |
6/45. Postmortem diagnosis of "occult" klinefelter syndrome in a patient with chronic renal disease and liver cirrhosis.This report describes a patient not suspected of having klinefelter syndrome during life but diagnosed with it following postmortem examination using fluorescent in situ hybridization (FISH) for sex chromosomes and hormone serum analysis. A 49-year-old Japanese man had a history of nephrosis, heavy alcohol consumption, diabetes mellitus, and liver cirrhosis and had been undergoing dialysis for 10 years. He died of ruptured esophageal varices. autopsy revealed hypogonadism, suggesting klinefelter syndrome. This was confirmed by FISH, which showed a mosaic 46XY, 47XXY karyotype, and by serum analysis, which revealed high luteinizing hormone and follicle-stimulating hormone and low testosterone levels. autopsy also revealed a nodular, bilateral, testicular Leydig cell hyperplasia. This report illustrates the value of postmortem laboratory investigations, particularly FISH for sex chromosomes and serum hormone analysis, for the demonstration of clinically uncertain or "occult" klinefelter syndrome.- - - - - - - - - - ranking = 4.3903481683406keywords = consumption (Clic here for more details about this article) |
7/45. A patient with autoimmune hepatitis type I, Addison's disease, atrophic thyroiditis, atrophic gastritis, exocrine pancreatic insufficiency, and heterozygous alpha1-antitrypsin deficiency.This report describes a 60-yr-old white male presenting with decompensated liver cirrhosis. He had a history of Addison's disease for 36 yr, primary hypothyroidism for 5 yr, and moderate alcohol consumption. His laboratory studies and a liver biopsy supported the diagnosis of autoimmune hepatitis. Furthermore, he was found to be heterozygous for the piZ allele of the alpha1-antitrypsin gene with normal serum alpha1-antitrypsin levels and absence of pulmonary affection. Mucosal biopsies revealed moderately severe atrophic gastritis; however, signs of pernicious anemia were missing. An association of autoimmune hepatitis with endocrine disorders and atrophic gastritis has been described. Long term hydrocortisone therapy for his adrenal insufficiency may have prevented a faster course of the liver disease, whereas the heterozygous alpha1-antitrypsin deficiency and moderate alcohol consumption constituted additional risk factors ultimately leading to the development of cirrhosis.- - - - - - - - - - ranking = 8.7806963366811keywords = consumption (Clic here for more details about this article) |
8/45. Obese children with steatohepatitis can develop cirrhosis in childhood.Nonalcoholic steatohepatitis, in which fatty change and inflammation of the liver occur in the absence of excess alcohol intake, is increasingly recognized in obese children. Although fibrosis is common in pediatric nonalcoholic steatohepatitis, cirrhosis has been reported rarely. The two boys reported here developed cirrhosis from nonalcoholic steatohepatitis at ages 10 and 14 yr. One child progressed to cirrhosis with symptomatic portal hypertension within a 2-yr period.- - - - - - - - - - ranking = 0.32853874753823keywords = intake (Clic here for more details about this article) |
9/45. pathology related to chronic arsenic exposure.Millions now suffer the effects of chronic arseniasis related to environmental arsenic exposure. The biological mechanisms responsible for arsenic-induced toxicity and especially chronic effects, including cancer, are not well known. The U.S. Armed Forces Institute of pathology (AFIP) is participating in an international research effort to improve this understanding by the development of the International Tissue and Tumor Repository for Chronic Arsenosis (ITTRCA). The ITTRCA obtains, archives, and makes available for research purposes, tissues from subjects exposed to arsenic. We provide here a short overview of arsenic-induced pathology, briefly describe arsenic-induced lesions in the skin and liver, and present five case reports from the ITTRCA. arsenic-induced skin pathology includes hyperkeratosis, pigmentation changes, Bowen disease, squamous cell carcinoma, and basal cell carcinomas. A unique spectrum of skin lesions, known as arsenical keratosis, is rather characteristic of chronic arseniasis. Bowen disease, or squamous cell carcinoma in situ of the skin, has been well documented as a consequence of arsenical exposure. A spectrum of liver lesions has also been attributed to chronic arseniasis. Of these, hepatocellular carcinoma, angiosarcoma, cirrhosis, and hepatoportal sclerosis have been associated with arsenic exposure. We present case reports that relate to these health conditions, namely, squamous cell carcinoma, basal cell carcinoma, and Bowen disease of the skin and hepatocellular carcinoma and angiosarcoma of the liver. Four patients had been treated with arsenical medications for such conditions as asthma, psoriasis, and syphilis, and one case occurred in a boy chronically exposed to arsenic in drinking water.- - - - - - - - - - ranking = 0.33573062623088keywords = water (Clic here for more details about this article) |
10/45. Small dose of recombinant factor viia (rFVIIa) to perform percutaneous liver biopsies in cirrhotic patients.Low levels of vitamin k-dependent coagulation factors, synthesized in the liver, play a key role in the hypocoagulable state of end-stage liver disease patients. Recombinant factor VII (rFVIIa) has been developed for, and currently is used in the treatment of patients with hemophilia a and B with inhibitors. Some experience was gained with rFVIIa in liver diseases since 1995. We used a low dose of rFVIIa to perform percutaneous liver biopsy in three patients, all of them with abnormal coagulation, impeditive of the percutaneous liver biopsy. The first one was a 29 years old man with alcoholic cirrhosis and a liver nodule; the second was a 32 years old man with post hepatitis c cirrhosis and excessive alcohol intake; the third was a 53 years old man with chronic hepatitis c and a congenital deficit of factor VII. A single dose of 5 micrograms/Kg of rFVII administered before liver biopsies raised levels of factor VII to acceptable values during more than 5 hours in the first two patients. We conclude that a small dose of rFVIIa can be enough to correct the abnormal coagulation of cirrhotic patients, permits percutaneous liver biopsy, and is cost-effective, compared to transjugular access.- - - - - - - - - - ranking = 0.32853874753823keywords = intake (Clic here for more details about this article) |
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