MRCP UK EXAMINATIONS

ABDOMINAL

THALASSEMIA

Question [CLICK ON ANY CHOICE TO KNOW THE RIGHT ANSWER]
Why is hydrops fetalis incompatible with life?
A
Life cannot exist without HbA
B
Lack of embryoinc hemoglobins precludes fetal development
C
All three normal adult hemoglobins contain alpha chains
D
Fetal hgb is essential to sustain life after birth
Explanation: 

Detailed explanation-1: -Hemoglobin Barts hydrops fetalis syndrome, the most severe and generally fatal clinical phenotype of a-thalassemia, is due to the deletion of all four functional -globin genes of the hemoglobin, resulting in no -globin chain production.

Detailed explanation-2: -Bart’s hydrops fetalis is caused by deletion or inactivation of all four alpha-globin alleles leading to a severe deficiency in alpha-globin chains of Hb, and to the production of gamma-4 tetramers (Hb Bart’s) during fetal life, and beta-4 tetramers (HbH) postnatally.

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