Polymorphism of Amyloid Fibrils In Vivo.

Polymorphism is a wide-spread feature of amyloid-like fibrils formed in vitro, but it has so far remained unclear whether the fibrils formed within a patient are also affected by this phenomenon. In this study we show that the amyloid fibrils within a diseased individual can vary considerably in their three-dimensional architecture. We demonstrate this heterogeneity with amyloid fibrils deposited within different organs, formed from sequentially non-homologous polypeptide chains and affecting human or animals.

[Hereditary hemochromatosis HFE: regulatory and economic aspects of diagnosis and medical management].

HFE hereditary hemochromatosis is a chronic illness (ALD n(o) 17 - Maladies métaboliques et héréditaires) which is the first genetic disease in France (60% of all genetic diseases). The list of medical acts and the services supported by the French national health insurance fund are fully codified by the French national authority for health (HAS) in order to reduce unnecessary health care spending. The search for the C282Y mutation of the HFE gene to confirm the diagnosis is supported by French national health insurance fund since 2007 (under certain conditions).

[Hereditary hemochromatosis, the clinician point of view].

Hereditary hemochromatosis (HH) is an autosomal recessive disease whose most common form is due to homozygosity for the C282Y mutation of the HFE gene. Its prevalence is estimated between 1/200 and 1/600 in France. This represents potentially several thousands of affected people.

Treatment of cirrhotic tense ascites with Dextran-40 versus albumin associated with large volume paracentesis: a randomized controlled trial.

Intravenous albumin infusion prevents complications after large-volume paracentesis (LVP), particularly paracentesis-induced circulatory dysfunction (PCD), and improves patient survival. However, albumin is expensive. We compared a low-molecular weight dextran (Dextran-40) with albumin in treating LVP in cirrhotic patients with tense ascites.

[Hereditary haemochromatosis].

EPIDEMIOLOGY ADN PHYSIOPATHOLOGY: Hereditary haemochromatosis is the most common genetic disease in France. Its frequency is on average 1 out of 300 French individuals. It is due to excessive dietary iron absorption, leading to accumulation of iron in the body.