[Long-term study in congenital refractory anemia and secondary siderosis].
It is reported on the course of the disease in a female patient with congenital therapy-refractory anaemia, in whom a transfusion siderosis of high degree with functional disturbances of the myocardium and the liver, with endocrine disturbances (primary hypothyroidism and normogonadotropic hypogonadism) and with small growth became clinically manifest. A reduction of the iron overload by a continuous desferrioxamine therapy in form of long-term subcutaneous infusions over two years could be proved. Possibilities and problems of treatment and conclusions relevant to practice are described.
D000293:Adolescent; D000741:Anemia, Aplastic / Q000150:complications / Q000151:congenital* / Q000628:therapy; D003676:Deferoxamine / Q000627:therapeutic use; D004392:Dwarfism / Q000209:etiology; D005260:Female; D006486:Hemosiderosis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D007006:Hypogonadism / Q000209:etiology; D007037:Hypothyroidism / Q000209:etiology; D011239:Prednisolone / Q000627:therapeutic use; D065227:Transfusion Reaction
D011239:Prednisolone; D003676:Deferoxamine
Langzeitbeobachtung bei kongenitaler therapierefraktärer Anämie mit sekundärer Siderose.