Gilber-Meilengracht Syndrome

Gilbert-Meilengracht syndrome, first described in 1901, is a common type of congenital non-hemolytic unconjugated hyperbilirubinemia, detected in 5-11% of the population, with predominance in males. The study of many families with typical cases of the disease shows an autosomal dominant character of inheritance with a relatively low frequency of manifestation( penetrance) and a variable development of the defect.

As a rule, this pathology is noted in children during puberty. With a careful study of the history of life of this patient, only occasionally one can find evidence of prolonged jaundice in the period of newborn. In mild cases, the pathology remains unrecognized for a long time. The main manifestation of this disease is often mild jaundice with its periodic increase due to physical stress, other diseases such as angina, acute respiratory viral infection, exacerbation of chronic tonsillitis, as well as dietary errors, starvation, mental stress. Some of these patients have neurotic complaints, as well as complaints of violations on the part of the digestive system. Perhaps a moderate increase in the liver, complaints of heaviness in the right upper quadrant, abdominal pain. As a rule, the pathology develops after the transferred viral hepatitis, which is the factor triggering the genetically caused defect of one of the liver systems.

In biochemical blood tests of children with Gilbert-Meylengracht syndrome, an increased content of free bilirubin fraction is noted, the indices of other functional liver samples do not change. Ultrasonic, scintigraphic and laparoscopic liver pattern is usually normal. The gallbladder is well defined in cholecystography.

The course of the Gilbert-Meilengracht syndrome, as a rule, is quite long, wavy. When treating phenobarbital, the level of bilirubin decreases usually to normal, at rest, jaundice in most cases disappears after 5-7 days even without treatment.

Prognosis favorable, progressive changes in the liver are not described, however, monitoring patients is necessary throughout life.