Homozygous α-thalassaemia Hb Bart is a very rare illness in North America and Western Europe, but is a common cause of NIHF especially in South East Asia. It causes severe hydrops fetalis with lethal outcome and maternal complications as pre-term labour, preeclampsia and retained placenta. A case report of an immigrant from Laos with preterm labour and fetal ascites, but without signs of pre-eclampsia is demonstrated. Fetal heart rate (FHR) trace and biophysical profile were pathological, while umbilical Doppler flow was normal due to hypoxaemia, but with hypervolumina and a high cardiac output. An amniocentesis and puncture of the ascites were performed, but FBS was not successful. Before receiving the results a caesarean section had to be done for deterioration of the FHR trace. A 1,370-g female infant was born who died of cardiovascular failure due to an α-thalassaemia Hb Bart and respiratory distress 5 days post partum. The parents had both an α-0-thalassaemia. α-Thalassaemia is still a very unknown illness in Germany, but with the increasing numbers of immigrants from South East Asia it should be considered at the differential diagnosis of hydrops fetalis. A prenatal diagnosis should be offered to parents with α-0-thalassaemia.

Keywords:
Hb Bart thalassaemia, Hydrops fetalis, Prenatal diagnosis
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© 1993 S. Karger AG, Basel
1993
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