Hyperbilirubinemia (excessive production of bilirubin)
Predominantly unconjugated hiprbilirubinemija
Increased formation of bilirubin cause hemolytic processes and inadequate erythropoiesis. The circulation of adult adult (average 5l) should be about 150 g / l of hemoglobin (about 750 g of hemoglobin). Daily breaks down about 0.8% of the total amount of red blood cells, which means that the catabolism releases about 6.3 grams of hemoglobin / 24h.
The hemolytic process can free up to 45 grams hemoglobina/24h, the creation of an hemoglobin increase from the usual 300 mg and the amount of up to 8000 mg/24h. Thus, a large amount of bilirubin hepatocytes can not be accepted and processed, and it accumulates in tissues and blood. Increased formation of bilirubin may occur at extravascular myocardial tissue and the large collection of blood in the tissues. Clinically manifested by hemolysis: indirect hyperbilirubinemia with reticulocytosis, increased fecal Urobilinogen and shortened life of erythrocytes.
Increased amounts of bilirubin in seum is very dangerous in cases of neonatal jaundice (jaundice-kem) when it deposited in the basal ganglia to form bilirubinska encephalopathy.
Increased formation of bilirubin encountered in cases of inadequate eritrpeoeze (pernicious anemia, thalassemia and congenital erythropoietic porphyria), and sepsis and the effects of some drugs in long-term use. Predominantly unconjugated hyperbilirubinemia occurs with certain diseases, such as:
a) Sy. Gilbert. This form of the disease is quite similar to the previous form of hyperbilirubinemia, is caused by a reduction in opportunities to receive a liver bilirubin, decreased conjugation of bilirubin, as well as reducing the transport capacity of bilirubin. It has 7% of the healthy population. Clinical sea usually manifested in the twenties with a slight povišenimvrijednostima bilirubin (up to 30 mmol / l), usually after the infectious process, fasting, surgical flag, drinking alcohol and the like. Diagnosis is usually called. fasting test. The positive effect in reducing the value of a primary treatment of diseases with phenobarbital at a maximum dose of 3x360 mg/24h. Treatment of Gilbert's syndrome is not necessary.
b) Sy. Crigler-Najjar type in both its type I is a rare form of bilirubin which is a disorder caused by a complete lack of glucuronyl-transferase. It manifests itself immediately after birth the high values of serum bilirubin, with a mortality rate visokiom but the first year of life. The therapy is applied phototherapy and liver transplantation.Type II is characterized by a partial lack of glucuronyl-transferase. Clinically manifest at puberty with elevated serum bilirubin, but with a very rare neurological disorders. The therapy uses other than phototherapy and phenobarbital, which affects the effective reduction of serum bilirubin concentrations.
c) Acquired absence of glucuronyl-transferase. This form of the disease usually becomes apparent in novorođenačnoj age during lactation, mothers suffering from hypothyroidism, and treatment with chloramphenicol. Hyperbilirubinemia stop breastfeeding cessation. It can occur in cases of diseases such as sepsis, and in hepatocellular disease.
Predominantly conjugated hyperbilirubinemia
a) Sy. Dubin-Johnson. In this disease it is the innate benign disease that is clinically manifested by hyperbilirubinemia with the appearance of dark pigment in liver centrolubularnim parts. The cause of this disease is reduced transport of organic anions from the liver into the bile. Occurs between 10-40 years of age. Clinically it is manifested by mild gastroinstestinalnim problems with hepatomegaly in 50% of patients.
b) Sy. Rotor. The basic disorder in this form of congenital hyperbilirubinemia is reduced accommodation capacity of hepatocytes to bilirubin. Clinically manifest jaundice with bilirubin increase in infections and a reduction in the pregnancy.
c) Benign familial recurrent cholestasis. This extremely rare form of the disease is clinically manifested by the occurrence of jaundice, pruritus, stetoreje and weight loss.
d) Rekturna jaundice in pregnancy. It occurs most often in the last trimester of pregnancy, and is manifested by hyperbilirubinemia, pruritus with an increase in cholesterol and alkaline phosphatase seum. Resolved spontaneously ten days after birth. It can be repeated in the following pregnancies.
e) Cholestasis Cholestasis caused by the drug most commonly causes oral contraceptives and anabolic.
f) extrahepatic biliary obstruction
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