Perinatal case report of unexpected thalassemia Hb Bart.
Homozygous alpha-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 preterm labour, pre-eclampsia 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 alpha-thalassaemia Hb Bart and respiratory distress 5 days post partum. The parents had both an alpha-zero-thalassaemia. alpha-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 alpha-zero-thalassaemia.
Hofstaetter
C
C
University Department of Obstetrics Charlottenburg, Berlin, FRG.
Gonser
M
M
Goelz
R
R
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D005858:Germany; D006331:Heart Diseases / Q000209:etiology; D006340:Heart Rate, Fetal; D006455:Hemoglobins, Abnormal; D006801:Humans; D007231:Infant, Newborn; D007812:Laos / Q000208:ethnology; D011247:Pregnancy; D012131:Respiratory Insufficiency / Q000209:etiology; D017085:alpha-Thalassemia / Q000097:blood / Q000175:diagnosis* / Q000235:genetics
D006455:Hemoglobins, Abnormal; C030448:hemoglobin Bart's