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Acta Medica Medianae |
INDIRECT HYPERBILIRUBINEMIA OF THE CHILD AGE AS A CONSEQUENCE OF SPHEROCYTOSIS
Gordana BJELAKOVIĆ, Dudica MILENOVIĆ, Saša ŽIVIĆ, Gordana KOSTIĆ and Bojko B. BJELAKOVIĆ
Biochemistry Institute of the Faculty of Medicine and the Children Internal Clinic of the Clinic Center, Niš
The hereditary spherocytosis is an inherited hemolytic anemia; it can be the cause of the hemolytic disease of newborns, infants and small children. The spherocytosis appears due to a defect in the structure of the specific plasma proteins of the erythrocyte membrane, that is, of the spectrin, and, as sometimes possible, of the ankyrin as well. As a consequence of the intrinsic ("intracorpuscular") defect in the membrane, the erythrocytes at the hereditary spherocytosis, accumulate natrium ions and water; thus, they become swollen and spherical two to three times than the normal erythrocytes. That is why they exhibit increased osmotic fragility and quickened antihemolysis. The spherocytosis is most often revealed by perceiving the symptoms of the hemolytic anemia, that is, by the presence of anemia, indirect hyperbilirubinemia, a greater presence of natrium in the erythrocytes along with the simultaneous reduction of the amount of kalium, the reduced osmotic resistance of the erythrocytes, mild hypersideremia and reticulocytosis in addition to the appearance of spherocytes and splenomegaly. The paper presents the cases of three patients who, after being subjected to splenectomy, have shown the improvement of the general state as well as mitigation of the hemolytic anemia symptoms.
Key words: Indirect hyperbilirubinemia, spherocytosis, natrium and kalium of erythrocytes |