منابع مشابه
Familial Cholestatic Syndromes
The term ‘familial’ is an unfortunate one. Although the definition includes ‘hereditary’, the inference is that it will usually have occurred in other family members. For many of the conditions described in this article this is often not the case, and for this reason, ‘familial’ conditions may be overlooked when reaching a diagnosis. Cholestasis is defined differently by clinicians, pathologist...
متن کاملFamilial Intrahepatic Cholestatic Jaundice
Recurrent jaundice due to intrahepatic cholestasis is reported most often in adults. Some varieties are familial and are associated with failure of excretion of conjugated bilirubin by the liver cell. The original descriptions of these types given by Rotor, Manahan, and Florentin (1948) and Dubin and Johnson (1954) concerned patients with a benign recurrent jaundice. The diseases described by t...
متن کاملNutritional Management of Cholestatic Syndromes in Childhood
Cholestatic liver disease causes severe risk of malnutrition which includes protein-energy malnutrition and specific nutritional deficiencies. The nutritional status can be assessed based on anthropometric measurements, which can be misleading because of ascitis and peripheral edema. Biochemical determinations of lipid-soluble vitamin status are important to evaluate requirements. Based on nutr...
متن کاملPathogenesis of familial periodic fever syndromes or hereditary autoinflammatory syndromes.
Familial periodic fever syndromes, otherwise known as hereditary autoinflammatory syndromes, are inherited disorders characterized by recurrent episodes of fever and inflammation. The general hypothesis is that the innate immune response in these patients is wrongly tuned, being either too sensitive to very minor stimuli or turned off too late. The genetic background of the major familial perio...
متن کاملFamilial Syndromes Coupling with Small Renal Masses
During the past two decades, several new hereditary renal cancers have been discovered but are not yet widely known. Hereditary renal cancer syndromes can lead to multiple bilateral kidney tumors that occur at a younger age than that at which the nonhereditary renal cancers occur. The aim of our work is to review the features of hereditary renal cancers, the basic principles of genetic relevant...
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ژورنال
عنوان ژورنال: Annales Nestlé (English ed.)
سال: 2008
ISSN: 1661-4011,0517-8606
DOI: 10.1159/000147409