Page 209 - Critical Maternity & Newborn Health Nursing
P. 209

(II) β-Thalassemia:


                   a) β - thalassemia major (homozygotes):



                   The disorder starts in childhood leading to   death of the patient mostly in

                   the 2nd or 3rd decade.


                   b) β -thalassemia minor (heterozygotes): As a - thalassemia minor.










                   Effect on pregnancy:


                       •  (β -Thalassemia major is rarely encountered in pregnant women, but

                          if this happened the prognosis is poor.


                       •  Anemia becomes severe in   mid-pregnancy and may result in

                       •  heart failure.

                   Sickle Cell Anemia


                       •  An autosomal inherited disorder in  which glutamic  acid in

                          position 6 of the (b - chain of the hemoglobin molecule is HbS

                          on exposure to hypoxia forms insoluble  aggregations and

                          RBCs become sickle-shaped and are subsequently

                          fragmented.

                       •  In addition, these sickle-shaped   cells   increase    the blood viscosity

                          and occlude blood vessels of various organs.


                       •  The manifestations  appear usually in homozygous  not in
                          heterozygous.



                   Clinical picture:



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