Hereditary disease, due to the violation of the intracellular transport of conjugated (related) bilirubin, and its excretion into bile. As a result, bilirubin through sinusoidal poles hepatocytes (liver cells) enters the general circulation.
The clinical picture is dominated by the phenomenon! želtuhi, It notes dark urine staining, enlargement of the liver.
The blood is determined by the high content of direct bilirubin; during the X-ray examination with contrast media, taken by mouth, determined by the "mute" the gallbladder, T. it is. the gall bladder is not contrasted and is not determined.
With an increase in symptoms of jaundice is a choleretic therapy.
Favorable prognosis.
Hereditary disease, ydentychnoe Johnson syndrome Dabyna-, but a defect in the excretion of bilirubin this pathological process less significant. In contrast, Dubin-Johnson syndrome, Rotor syndrome when the gallbladder during holetsistografii contrasted.
Favorable prognosis, Treatment consists in the appointment choleretic, sedatives, detoxification therapy, appropriate diet.
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