DISEASES OF THE BLOOD SYSTEM : JAUNDICE CONGENITAL HEMOLYTIC (ICTERUS НАЕMOLYTICUS) - etiology, pathogenesis, symptoms and course of, recognition, prediction and prevention
Etiology. There's a risk of haemolytic substances contributing to failure of the bone marrow, producing defective erythrocytes (microcity - microporosity), characterized by a sharp decrease in the osmotic resistance due to the predominance among them young forms. There have been several degenerative disorders morphological structure from the side of the skull, palate (tower skull, high standing of the sky, and others). Onset in early childhood. Often it ill several members of the same family.
The pathogenesis. Of particular importance hemolysis, the destruction of red blood cells in the cells of the reticulo-endothelial system, mainly the spleen and liver.
Symptoms. The main symptoms are jaundice and splenomegaly. Sometimes there is the development of severe anemia. Gematologichesky for hemolytic jaundice is characterized microparasites, a sharp decrease in the osmotic stability of erythrocytes, as well as a marked reticulocytosis, often reaching 50% of all erythrocytes. Anemia often hypochromic type.
For chronic. Jaundice periodically increases and again diminished, but never disappears completely. Patients often develop biliary colic and pain in the spleen, which complicate the disease. Biliary colic due pleochroic, the formation of pigment stones; pain in the spleen due to a heart attack and subsequent perisplenitis.
The recognition is based on the triad:
1) microcytosis erythrocytes,
3) lowering the osmotic stability of erythrocytes and the presence of jaundice, anemia and splenomegaly.
The prediction is relatively favorable.
Spleen removal is recommended only when the exacerbation of course, the complication of anaemia. A blood transfusion is only possible after prior plasmacare. Intravenous infusion of plasma 50 - 100 - 200 ml - in the form of a rate of 10 to 15 injections (Vlados, Belousov). Sometimes the therapeutic effect achieved by the introduction of large doses of inorganic iron (Ferri hydrogenio reducti to 6,0 - 10,0 per day). Hygienic regimen.