Congenital forms of anemia in children

Among them are the most important Fanconi anemia, aplastic family Oestrich-Dameshek, partial hypoplastic Josephs-Diamond-Black fan. Fanconi Anemia usually occurs during the first years of life. The boys are sick 2 times more often girls. Children lag behind in physical and mental development. Malformations observed eyes, kidneys, sky, hands, microcephaly (reduced size of the head and thereby cerebral hypoplasia), increasing the content of pigment in the skin and, therefore, its darkening. At the age of 5 years and older usually appears a lack of red blood cells and platelets (pancytopenia), in which there is bleeding, liver enlargement without an increase in the spleen and lymph nodes, inflammatory changes of many organs and tissues. In anemia Oestrich-Dameshek observed similar changes in the blood, but no malformations. Anemia Josephs-Diamond-Black fan is shown in the first year of life, often a benign. The disease develops gradually - appears pale skin and mucous membranes, lethargy, decreased appetite; erythrocytes and hemoglobin in the blood is reduced.
Treatment: transfusions of red cells, splenectomy or embolisation (see above.), Hemostatic agent for bleeding.