Page 208 - Critical Maternity & Newborn Health Nursing

Clinical picture:

1 - Features of anemia.

2- Features of haemolytic jaundice:

- Lemon yellow skin. - Ting of jaundice in the sclera.

- Dark stool and normal urine which darkens on standing.

3-Hepatosplenomegaly: are common.

The condition is inherited by 50% of the mother offspring. In the infant,

jaundice develops within 48 hours of birth and exchange transfusion

may be required.

Thalassaemia

An autosomal inherited disorder resulted from failure of production of

either a chain (α - thalassaemia) or b chain (b -thalassaemia) of the

hemoglobin molecule and their replacement with other polypeptide chains.

(I) α- thalassaemia:

a) α - thalassaemia major (homozygotes):

The fetus with this disorder is affected in utero showing polyhydramnios.

Erythroblastosis, anemia and hydrops resembling Rh incompatibility.

This fetus does not survive due to inability of oxygen transfer as the α-

chain is responsible for O2 carrying capacity.

b) α - thalassemia minor (heterozygotes):

& Patient develops mild progressive anemia during pregnancy.

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