Pregnancy in women with hemoglobin H disease in the Guangxi Zhuang
Autonomous Region of southern China
Objective To investigate the fertility status and pregnancy outcome in
women with hemoglobin H disease in southern China. Design Retrospective
analysis. Setting The First Affiliated Hospital of Guangxi Medical
University, Nanning, Guangxi Population 28 young women with Hb H
disease. Methods Clinic characteristics were included during their
pregnancies over a 12-year period and their babies’ outcomes were
assessed.. Main outcome measures Genotype, methods of conception and
delivery, complications during pregnancies, and frequency of
transfusion. Hb and serum ferritin levels. Babies’ birth weight, Apgar
scores at 1 minute. Results Four genotypes, namely, - -/-α3.7, -
-/-α4.2, - -/αWSα, - -/αQSα, and - -/αCSα, were found among these women.
The most frequent ethnic background was Zhuang (57.14%). 59 pregnancies
occurred. 56 conceptions were spontaneous. Six pregnancies were
terminated because of Hb Bart’s hydrops fetalis. 44 resulted in
successful deliveries of 46 neonates, and 17 were via cesarean section.
The mean gestational period of delivery was 38 weeks, the rate of
pregnancy complications was 50%. The majority of these women were never
transfused before or during their pregnancy, except those with genotype
–/αCSα. and the mean birth weight of the 46 neonates was
2815.00±503.26g. The mean hemoglobin concentration and median serum
ferritin level were significantly decreased during the pregnancy.
Conclusions Despite the good pregnancy outcome for women with Hb H
disease, the pregnancy course is still considered high risk. Close
monitoring of maternal and fetal conditions should be performed by
multidisciplinary team to ensure the best outcome.