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Chapter-06 Thalassemia in Pregnancy

BOOK TITLE: Anemia in Pregnancy

Author
1. Puri Manju
2. Taneja Anjali
ISBN
9788184482997
DOI
10.5005/jp/books/10044_6
Edition
1/e
Publishing Year
2008
Pages
26
Author Affiliations
1. Lady Hardinge Medical College and Smt SK Hospital, New Delhi, India, Lady Hardinge Medical College and SSK Hospital, New Delhi, India, Lady Hardinge Medical College and SSK Hospital, New Delhi, Lady Hardinge Medical College and SSK Hospital, New Delhi., Lady Hardinge Medical College, New Delhi., Lady Hardinge Medical College and Hospital, New Delhi 110 001, India, LMMC and SSKH, Lady Hardinge Medical College, New Delhi, Lady Hardinge Medical College, New Delhi 110001, India, New Delhi, Lady Hardinge Medical College, New Delhi, India, Lady Hardinge Medical College and Associated Smt Sucheta Kriplani Hospital, New Delhi, India
2. Lady Hardinge Medical College and SSK Hospital, New Delhi., New Delhi
Chapter keywords

Abstract

Thalassemias are the commonest single gene disorders of hemoglobin synthesis, characterized by lack of or decreased synthesis of one or more globin chains. Two most common types are: beta thalassemia (Mutation disorder), diagnosed by Hb electrophoresis and beta-thalassemia (deletion disorder), diagnosed by genetic testing. In beta-thalassemia minor, obstetric outcome is not affected. However folic acid supplementation is recommended. Screening of partner is done and genetic counseling and prenatal diagnosis is offered if found to be positive. Beta thalassemia major can cause hypogonadism consequent to multiple endocrinopathies due to iron deposition. Evaluation of end organ damage and folic acid supplementation are essential preconceptionally. During pregnancy chelation should be stopped and vitamin C supplemented along with continued high dose folic acid supplementation. Repeated blood transfusions would be needed with the aim to maintain Hb at 10 gm%. Prenatal diagnosis should be offered if required. Fetal monitoring for IUGR should be done. Lactation is not contraindicated. Chelation therapy should be restarted at 3 weeks postpartum. Hormonal contraceptive pills are avoided. In beta thalassemia, adverse effects in pregnancy are due to accumulation of abnormal hemoglobins like HbH and Bart’s Hb. Silent carrier state is asymptomatic while beta thalassemia trait can present with mild anemia. Pregnancy is usually well tolerated. FA supplementation is recommended. In HbH disease anemia is exaggerated and there is an increased incidence of hemolytic crises. Blood transfusion is required for severe anemia. Screening of partner and genetic counseling is recommended for women with beta thalassemia trait/ HbH disease. Prenatal diagnosis should be offered to detect affected fetus.

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