title: A successful twin pregnancy in a patient with HbE-β-thalassemia in western India.
abstract: Improvements in medical facilities have helped a large number of clinically severe hemoglobin E (HbE)-β-thalassemia patients reach adulthood. Consequently, there is a new challenge, that of managing women with HbE-β-thalassemia during pregnancy. In particular, they have a high risk of abortion, preterm delivery, intrauterine growth restriction, and thromboembolism. A 27-year-old HbE-β-thalassemia patient on regular transfusion, who was splenectomized and heptatitis C (HCV)-positive, conceived for the first time without any infertility treatment. However, there was incomplete abortion with heavy bleeding at 3 months of gestation, which required bilateral uterine artery angiography. The angiogram showed the left uterine artery to be moderately hypertrophied. This was embolized with 300-500 micron polyvinyl alcohol (PVA) to stop the bleeding. Soon after, she conceived again with a twin pregnancy, and at 33.3 weeks of gestation, there was a normal delivery of twin girls without any postpartum hemorrhage or perineal tear. Both babies were given prematurity care. The mother and children were both normal up till the last follow-up 18 months after delivery, and both the girls are HbE heterozygous. Thorough monitoring of endocrine functions along with proper management of transfusions and iron overload can help in reducing the complications related to pregnancy in these patients.
authors.affiliation: Department of Paediatrics, Nanavati Hospital, Mumbai, Maharashtra, India.
authors.forename: R
authors.initials: R
authors.lastname: Merchant
authors.forename: K
authors.initials: K
authors.lastname: Italia
authors.forename: J
authors.initials: J
authors.lastname: Ahmed
authors.forename: K
authors.initials: K
authors.lastname: Ghosh
authors.forename: R B
authors.initials: RB
authors.lastname: Colah
mesh_terms: D000328:Adult; D001803:Blood Transfusion; D005260:Female; D006446:Hemoglobin E; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000097:blood; D011256:Pregnancy Outcome; D059285:Pregnancy, Twin; D013156:Splenectomy; D017086:beta-Thalassemia / Q000150:complications* / Q000175:diagnosis / Q000628:therapy
chemical_list: D006446:Hemoglobin E