Anaemia neonatorum

Ohne Zusammenfassung     

Sideroblastic Anaemia

Sideroblastic Anaemias are characterised by a) chronic hypochromic anaemia, b) ringed sideroblasts in the bone marrow, c) an increase in total body iron, d) ineffective erythropoiesis and e) often abnormal concentrations of F.E.P. A classification of Sideroblastic Anaemia is given and the pathophysiology of Sideroblastic Anaemia is discussed. A series of seven paediatric cases with Sideroblastic Anaemia is presented and the results of studies of the iron, vitamin B₆ and porphyrin metabolism are discussed. In two cases arguments for an ALA-synthetase deficiency are given. All five males were diagnosed as hereditary X-linked Sideroblastic Anaemia, one female as I.R.S.A. and the other female, who showed the features of the X-linked type, as congenital Sideroblastic Anaemia.     

Über Anaemia neonatorum

Zusammenfassung Auf Grund der bisher in der Literatur niedergelegten Fälle und zweier eigener Beobachtungen wird versucht, die Symptomatologie, Ätiologie, Differentialdiagnose und Therapie der Anaemia neonatorum zu schildern.Bei dem einen Fall bestand ein aregeneratorisches Blutbild, d. h. keine Linksverschiebung, kein Auftreten von embryonalen Formen von roten und weißen Blutkörperchen, bei dem zweiten eine mäßige Regeneration, Normoblasten bis 9%, Reticulocyten bis 52:1000.Beide Fälle heilten nach 6 bzw. 22wöchentlicher Behandlung auf Bluttransfusion und Lebertherapie völlig aus.     

Cyclosporine in Steroid-Resistant Diamond-Blackfan Anaemia

ABSTRACT. Two siblings with Diamond-Blackfan anaemia (DBA) and several congenital malformations were first treated with corticosteroids and blood transfusions. High steroid doses were needed, but in spite of this haemoglobin values periodically fell below acceptable levels. Cyclosporine was then given in addition to prednisolone. A slow increase in haemoglobin levels was observed over 2–3 months, and the prednisolone doses could be reduced gradually. Two short communications in the literature report similar experiences (1, 2). Cyclosporine could be tried in DBA when reasonable corticosteroid doses do not give a satisfactory response. Since the main effect of cyclosporine is a specific inhibition of T-lymphocytes, the observed therapeutic effect in DBA indicates that T-lymphocytes may play an important role in the pathogenesis of this disease.     

Hypothyreoidie mit Rachitis und Anaemia pseudoleukaemica

Ohne Zusammenfassung