Diversion hyperbilirubinemia syndrome, also known as Israel syndrome, is caused by excessive production of bilirubin by bone marrow erythrocytes or precursors, or directly by the destruction and generation of ferriheme or tetrapyrrole precursors.

bubble_chart Pathological Changes

The pathological changes include slight iron pigment deposition in the spleen, severe infiltration of irregularly sized and shaped brown granules in the liver parenchymal cells, an increase in stellate cells with pigment phagocytosis, and normal erythroid hyperplasia in the bone marrow.

bubble_chart Clinical Manifestations

Both males and females can be affected, with onset between the ages of 10 and 20. Symptoms include jaundice and splenomegaly. Blood tests reveal spherocytes and increased erythrocyte fragility, reticulocytosis grade II, indirect hyperbilirubinemia, and increased urobilinogen excretion. Free hemolysis and mechanical fragility are normal, and liver function tests are normal.

bubble_chart Diagnosis

The diagnosis can be made based on clinical features and laboratory tests, but confirmation requires liver biopsy to exclude other types of congenital jaundice.

bubble_chart Treatment Measures

Hemolysis occurs during seasonal epidemics. Splenectomy can correct postoperative hemolysis, but hyperbilirubinemia persists.