INDIRECT HYPERBILIRUBINEMIA OF THE CHILD AGE AS A CONSEQUENCE OF SPHEROCYTOSIS

Abstract

The hereditary spherocytosis is an inherited hemolytic anemia; it can be thecause of the hemolytic disease of nevvborns, infants and small children. The spherocytosisappears due to a defect in the structure of the specific plasma proteins ofthe erythrocyte membrane, that is, of the spectrin, and, as sometimes possible, of theankvrin as well. As a consequence of the intrinsic ("intracorpuscular") defect in themembrane, the erythrocytes at the hereditary spherocytosis, accumulate natrium ionsand water; thus, they become swollen and spherical two to three times than the normalerythrocytes. That is why they exhibit increased osmotic fragility and quickenedantihemolysis. The spherocytosis is most often revealed by perceiving the symptomsof the hemolytic anemia, that is, by the presence of anemia, indirect hyperbilirubinemia,a greater presence of natrium in the erythrocytes along with the simu-Itaneous reduction of the amount of kalium, the reduced osmotic resistance of theerythrocytes, mild hypersideremia and reticulocytosis in addition to the appearanceof spherocytes and splenomegaly. The paper presents the cases of three patients who,after being subjected to splenectomy, have shown the improvement of the generalstate as well as mitigation of the hemolytic anemia symptoms.

Keywords

• Indirect hyperbilirubinemia
• spherocytosis
• natrium and kalium of erythrocytes