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Title: Anemia and hydrops in a fetus with homozygous hemoglobin constant spring
Authors: Pimlak Charoenkwan
Supatra Sirichotiyakul
Pharuhas Chanprapaph
Fuanglada Tongprasert
Rawee Taweephol
Rattika Sae-Tung
Torpong Sanguansermsri
Keywords: Medicine
Issue Date: 1-Dec-2006
Abstract: Hemoglobin Constant Spring (Hb CS) is an unstable hemoglobin (Hb) variant that results from a nucleotide substitution at the termination codon of the α2-globin gene. The compound heterozygosity of α-thalassemia and Hb CS (-/αα) results in a Hb H/CS disease which is clinically more severe than deletional Hb H disease. Homozygosity of Hb CS (αα/ αα) is generally characterized with mild hemolytic anemia, jaundice, and splenomegaly. The authors report 1 case with Hb CS homozygosity who presented with fetal anemia and hydrops. Intrauterine transfusions were given which rendered a favorable outcome. This report demonstrates an unusual and serious in utero complication in a fetus with Hb CS/CS. © 2006 Lippincott Williams & Wilkins, Inc.
ISSN: 15363678
Appears in Collections:CMUL: Journal Articles

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