Pregnancy outcome in homozygous sickle cell disease: observations from the Jamaican Birth Cohort.

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Tytuł:: Pregnancy outcome in homozygous sickle cell disease: observations from the Jamaican Birth Cohort.
Autorzy:: Lewis G; Department of Child and Adolescent Health, University of the West Indies, Kingston, Jamaica.
Thame M; Department of Child and Adolescent Health, University of the West Indies, Kingston, Jamaica.
Howitt C; Sir George Alleyne Chronic Disease Research Centre, The University of the West Indies, Cave Hill, Bridgetown, Barbados.
Hambleton I; Sir George Alleyne Chronic Disease Research Centre, The University of the West Indies, Cave Hill, Bridgetown, Barbados.
Serjeant GR; The Sickle Cell Trust, Kingston, Jamaica.
Źródło:: BJOG : an international journal of obstetrics and gynaecology [BJOG] 2021 Sep; Vol. 128 (10), pp. 1703-1710. Date of Electronic Publication: 2021 Apr 06.
Typ publikacji:: Journal Article
Język:: English
Imprint Name(s):: Publication: : Oxford : Wiley-Blackwell
Original Publication: Oxford [England] : Blackwell Science, [2000]-
MeSH Terms:: Anemia, Sickle Cell/*epidemiology
Pregnancy Complications, Hematologic/*epidemiology
Adolescent ; Adult ; Anemia, Sickle Cell/mortality ; Cohort Studies ; Female ; Humans ; Infant, Newborn ; Jamaica/epidemiology ; Male ; Maternal Death ; Pregnancy ; Pregnancy Complications, Hematologic/mortality ; Pregnancy Outcome ; Socioeconomic Factors ; Stillbirth ; Young Adult
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Grant Information:: British Medical Research Council (MRC)
Contributed Indexing:: Keywords: Homozygous sickle cell disease; maternal mortality; pregnancy complications
Entry Date(s):: Date Created: 20210308 Date Completed: 20210831 Latest Revision: 20220425
Update Code:: 20240105
DOI:: 10.1111/1471-0528.16696
PMID:: 33683802
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Objective: To document pregnancy outcome in homozygous sickle cell (SS) disease and in age-matched controls with a normal haemoglobin genotype followed from birth for up to 45 years.
Methods: A total of 100 000 consecutive non-operative deliveries screened for sickle cell disease at the main Government maternity hospital in Kingston, Jamaica between 1973 and 1981 detected 311 (149 female) babies with SS disease who were matched by age and gender with 250 (129 female) controls with an AA haemoglobin phenotype. These individuals have been followed from birth with prospective assessment of menarche and detailed documentation of all pregnancies.
Results: There were 177 pregnancies in 71 SS patients and 226 pregnancies in 74 AA controls. Mothers with SS disease had more spontaneous abortions (adjusted relative risk [aRR] 3.2, 95% CI 1.6-6.1), fewer live births (aRR 0.7, 95% CI 0.6-0.9) and their offspring were more likely to have a gestational age <37 weeks (aRR 2.1, 95% CI 1.1-3.7) and low birthweight <2.5 kg (aRR 3.0, 95% CI 1.6-5.3). They were more prone to acute chest syndrome (aRR 13.7, 95% CI 4.1-45.5), urinary tract infection (aRR 12.8, 95% CI 1.3-125.9), pre-eclampsia/eclampsia (aRR 3.1, 95% CI 1.1-8.8), retained placenta (aRR 10.1, 95% CI 1.1-90.3), sepsis (Fisher's Exact test 0.04) and pregnancy-related deaths (Fisher's Exact test 0.02). Four of five deaths were attributable to acute chest syndrome. There was no genotypic difference in pregnancy-induced hypertension or postpartum haemorrhage.
Conclusion: Pregnancy in SS disease carries risks for both mother and child. The variable characteristics of pregnancy-related deaths complicate their prevention.
Tweetable Abstract: Pregnancy in SS disease compared with controls showed increased abortions and stillbirths, fewer live births and maternal deaths in 7% patients.
(© 2021 John Wiley & Sons Ltd.)

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