Perinatal Case Report of Unexpected Thalassemia Hb Bart
作者:
C. Hofstaetter,
M. Gonser,
R. Goelz,
期刊:
Fetal Diagnosis and Therapy
(Karger Available online 1993)
卷期:
Volume 8,
issue 6
页码: 418-422
ISSN:1015-3837
年代: 1993
DOI:10.1159/000263861
出版商: S. Karger AG
关键词: Hb Bart thalassaemia;Hydrops fetalis;Prenatal diagnosis
数据来源: Karger
摘要:
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-thalassa
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