Aιμοσφαιρινοπάθειες και κύηση

Postgraduate Thesis uoadl:1314643 303 Read counter

Unit:
Κατεύθυνση Παθολογία της Κύησης
Library of the School of Health Sciences
Deposit date:
2015-03-30
Year:
2015
Author:
Αλχαζίδου Έλενα
Supervisors info:
Κ.Κασσάνος,Κ.Σαλαμαλέκης,Κ.Χρέλιας
Original Title:
Aιμοσφαιρινοπάθειες και κύηση
Languages:
Greek
Summary:
INTRODUCTION: Hemoglobinopathies are common autosomal
recessive disorders. During the last decades large immigration movement
coupled with gene interaction have changed the geographical pattern of these
diseases, As a result, countries of Northen Europe were forced to adopt
screening programmes of the South.
SCOPE OF THE CURRENT STUDY: Improvements in the overall
management of hemoglobinopathies have allowed affected persons to enjoy
increased life expectancy. Pregnancy poses a unique challenge for patients
with hemoglobinopathies and obstetricians and gynecologists. The current
study aims to summarise data of recent bibliorgaphy regarding the optimal
obstetric management of such patients.
OUTCOMES: Based on pathophysiological mechanisms
hemoglobinopathies can be divided into two large categories: thalassemia
(TM), where there is a decrese in the production of a normal globulin and
sickle cell disease (SCD), where an abnormal globulin is synthesused. In b-
TM, the basic disorder consists of ineffective hematopoiesis, while the
mainstay of treatment includes transfusion theraphy which causes iron
accumulation. On the other hand, in SCD deformed red blood cells become
entraped in microcirculation resulting in vasoocclusion.
Family planning is considered the cornerstone for women of reproductive
age. Women should remain on constant contraception and become pregnant
only when a total remission of the disease has been achieved and well
documented. Prenatally, evaluation of possible overt cardiac impairement and
genetic counseling should be offered to affected individuals at minimum.
Furthermore, aggressive pre- pregnancy iron chelation and prophylactic
vaccination of spleenectomised patients is highly recommended. Concerning
TM, clinicians should be alert for presentation of gestational diabetes,
hypothyreoidism or osteoporosis related to pregnancy. As regards SCD,
96
clinicians should try to avert sickle cell crisis at all costs, predominately by
preventing infection. Fetal well being requires increased monitoring with
successive ultrasonographic examinations, in order to assess fetal
development and possible anemia attributed to alloimunisation. Delivery
should be planned, so that blood products can become immediately available.
Cesarean section is recommended only for obstetrical reasons. Breastfeeding
should be encouraged but its duration should be tailored according to the
need to resume medication. Anticoagulation should be considered during the
puepertum.
CONCLUSION: With improved life expectancy in the
hemoglobinopathies, many women with these disorders are now choosing to
become pregnant. Although numerous complications can occur, vigilant
monitoring by both experienced obstetricians and hematologists can lead to
successful pregnancy outcomes.
Keywords:
Hemoglobinopathies, Pathophysiology, Thalassemia, Sickle cell disease, Family planning
Index:
No
Number of index pages:
0
Contains images:
Yes
Number of references:
71
Number of pages:
108
File:
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