Hyperbilirubinemia functional

Hyperbilirubinemia functionalHYPERBILIRUBINEMIA FUNCTIONAL (benign hyperbilirubinemia, jaundice functional) is a group of diseases and syndromes, characterized by a yellowish staining of the skin and mucous membranes, hyperbilirubinemia with normal other indicators of liver function and (basic forms) the absence of morphological changes in the liver, benign course. These include hyperbilirubinemia post-hepatitis and hyperbilirubinemia functional congenital. Hyperbilirubinemia functional congenital group of hereditary transmitted (genetically determined) non-haemolytic Hyperbilirubinemia. Diseases caused by disorders of the processes of entrainment hepatocytes free bilirubin from the blood, associating it with glucuronic acid to form bilirubin-glucuronide (conjugated bilirubin) and then highlight it with bile. In all cases, hyperbilirubinemia and jaundice detected in early childhood, in most cases (except syndrome criglernajjar H. Nayyar) is insignificant, can be interspersed in nature (to increase under the influence of errors in diet, alcohol intake, intercurrent diseases, physical fatigue and other reasons). Frequent soft expressed dyspeptic symptoms, mild asthenia, weakness, fatigue. The liver is usually not enlarged, soft, painless, functional liver samples (except Hyperbilirubinemia) are not changed. Radioisotope hepatocyte changes reveals. The spleen is not enlarged. Osmotic resistantly of erythrocytes, their life expectancy is normal. Needle biopsy in all forms (except syndrome Dubin-Johnson) does not detect changes. Gilbert's syndrome Gilbert's disease, Gilbert - Lerolle symptom, birth family cholemia, congenital hyperbilirubinemia, simple family cholemia, idiopathic hyperbilirubinemia, constitutional hyperbilirubinemia, idiopathic unconjugated hyperbilirubinemia) is characterized by moderate intermittently increasing the content of unbound (indirect) bilirubin in the blood, lowering the degree of Hyperbilirubinemia under the action of phenobarbital, no other functional or morphological changes of the liver and autosomal dominant (?) the type of inheritance. Criglernajjar Najjar syndrome syndrome (congenital phenolation-Kai jaundice type 1, congenital non-haemolytic jaundice with jaundice staining nerve nuclei, idiopathic hyperbilirubinemia) is characterized by extremely high blood levels of free (indirect) bilirubin due to the lack of hepatocytes, glucuronyltransferase translating free bilirubin in the associated toxic effects of bilirubin on basal and stem nuclei of the brain resulting encefalopatia, often leading patients to die in childhood, and autosomal recessive type of inheritance. Dubin - Johnson syndrome (jaundice Dubin-Johnson, jaundice constitutional non-haemolytic with lipochromis hepatosis) is characterized by a moderate increase in blood conjugated bilirubin - direct bilirubin-glucuronide) as a result of violations of the mechanisms of transport from microsomes of hepatocytes into the bile, perverted test results delay bromsulphalein with a secondary increase in its content in the blood after 90 min after intravenous, contractarianism gallbladder oral x-ray study, black liver due to the accumulation in hepatocytes brown lipochrome pigment, increased excretion of coproporphyrin I and autosomal recessive type of inheritance. Rotor syndrome (idiopathic hyperbilirubinemia type Rotor) is characterized by a moderate increase in blood bound bilirubin (bilirubin-glucuronide), the delay of excretion by the liver is introduced intravenous bromsulphalein, increased allocation of kidneys of coproporphyrin (mostly 1 isomer), absence of other functional and morphological changes of the liver, an autosomal recessive type of inheritance. For in all forms (except syndrome criglernajjar H. Nayyar) benign, the prognosis is good; the ability to work, usually saved. Syndrome criglernajjar Najjar syndrome patients usually die in early childhood..

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