نتایج جستجو برای: juvenile hemochromatosis

تعداد نتایج: 48167  

Journal: :Canadian Journal of Gastroenterology 1990

2017
Maciej Szemraj Katarzyna Oszajca Janusz Szemraj Piotr Jurowski

BACKGROUND Congenital hemochromatosis is a disorder caused by mutations of genes involved in iron metabolism, leading to increased levels of iron concentration in tissues and serum. High concentrations of iron can lead to the development of AMD. The aim of this study was to analyze circulating miRNAs in the serum of congenital hemochromatosis patients with AMD and their correlation with the exp...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2000
S P Hussain K Raja P A Amstad M Sawyer L J Trudel G N Wogan L J Hofseth P G Shields T R Billiar C Trautwein T Hohler P R Galle D H Phillips R Markin A J Marrogi C C Harris

Hemochromatosis and Wilson disease (WD), characterized by the excess hepatic deposition of iron and copper, respectively, produce oxidative stress and increase the risk of liver cancer. Because the frequency of p53 mutated alleles in nontumorous human tissue may be a biomarker of oxyradical damage and identify individuals at increased cancer risk, we have determined the frequency of p53 mutated...

Journal: :Blood 2005
Pierre-Alexandre Krayenbuehl Thomas Walczyk Ronny Schoenberg Friedhelm von Blanckenburg Georg Schulthess

It has recently been shown that the iron isotopic composition of blood differs between individuals and sexes, which is supposed to reflect individual differences in iron metabolism. We hypothesized that patients suffering from hereditary hemochromatosis would demonstrate alterations in the iron isotopic composition of blood due to persistent up-regulation of intestinal iron absorption. Blood fr...

2005
LEON M. PTASZEK ERIK T. PRICE MARY Y. HU PHILLIP C. YANG

The hallmark of hemochromatosis is the deposition of iron in multiple tissue types, most notably the skin, liver, pancreas, thyroid, and heart. Definitive diagnosis of iron deposition generally requires invasive methods, such as direct tissue biopsy. We describe a 40 year-old woman with end-stage liver disease secondary to hereditary hemochromatosis and alcohol abuse, who was referred to the ca...

Journal: :Blood 2008
Jill Waalen Vincent J Felitti Terri Gelbart Ernest Beutler

Because the penetrance of HFE hemochromatosis is low, traditional population screening measuring the transferrin saturation is unlikely to be cost-effective because the majority of subjects detected neither have clinical disease nor are likely to develop it. Three independent studies show that only patients with serum ferritin concentrations more than 1000 microg/L are at risk for cirrhosis, on...

2000
Robert B. Hash

Background: The understanding of hereditary hemochromatosis, along with the availability of genetic testing, is changing the approach to diagnosis of the disease. Methods: A MEDLINE search was performed using multiple key words related to hemochromatosis and iron metabolism. Results: Most cases of hereditary hemochromatosis are caused by a single mutation to the HFE gene, resulting in unregulat...

2017
Manuel A Lescano Letícia C Tavares Paulo C J L Santos

Juvenile hemochromatosis (JH) is a rare condition classified as an autosomal recessive disorder that leads to severe iron absorption. JH usually affects people under the age of 30 and presents symptoms such as chronic liver damage, hypogonadotropic hypogonadism, cardiac diseases and endocrine dysfunctions. The present case reports a 29-year-old Brazilian woman with JH condition due to HAMP muta...

Journal: :Bulletin of the Club of Pancreatologists 2017

Journal: :Hepatology 2004

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