title: Fetal outcome and childhood mortality in offspring of mothers with sickle cell trait and disease.
abstract: The sickle cell hemoglobinopathy is a major public health problem which causes high morbidity and mortality in India. Although the hematological and clinical profile of the patients is extensively studies. The reproductive outcome of mothers afflicted with sickle cell trait and disease is still unknown in India. In a retrospective study, we have examined the reproductive profile of 190 mothers afflicted with sickle cell, attending Medical Out-Patient Department at V.S.S. Medical College Hospital, Burla in Western Orissa, India during the year 1991-1992. Seventy-three mothers who were found normal after medical examination and were free from hemoglobinopathic disorders, anemia, jaundice, iron deficiency, etc. constituted the control group and 66 mothers with sickle cell trait and 51 with sickle cell disease formed the study group. The reproductive history was recorded for number of conceptions, fate of offspring, live birth, surviving children and childhood mortality. Hematological investigations and hemoglobin electrophoresis were done as per the standard procedure. There was no difference in mean number of livebirths per mother between controls and sickle cell trait mothers. But between the controls and sickle cell homozygotes (p < 0.01), and sickle cell trait and disease (p < 0.01) mothers, this mean number was significant. For abortions/miscarriages, the difference between controls and sickle cell homozygotes (p < 0.001), and sickle cell trait and disease (p < 0.01) mothers was highly significant. The number of stillbirths per mother in homozygous sickle cell mothers was higher (p < 0.01) as compared to controls. There were significantly higher childhood deaths in sickle cell trait (p < 0.05) and disease (p < 0.05) mothers than in the controls. It seems that the sickle cell heterozygote and hemoglobin E heterozygote mothers are genetically better fit than the sickle cell homozygotes. Further, the sickle cell disease is clinically severer than the hemoglobin E disease in India probably due to molecular diversity.
authors.affiliation: Division of Human Genetics, Regional Medical Research Centre (ICMR), Chandrasekharpur, Bhabaneswar, Orissa.
authors.forename: R S
authors.initials: RS
authors.lastname: Balgir
authors.forename: B P
authors.initials: BP
authors.lastname: Dash
authors.forename: R K
authors.initials: RK
authors.lastname: Das
mesh_terms: D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D005260:Female; D006450:Hemoglobin SC Disease / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality; D006801:Humans; D007194:India / Q000453:epidemiology; D007226:Infant Mortality / Q000639:trends*; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis* / Q000401:mortality; D011256:Pregnancy Outcome; D012016:Reference Values; D012189:Retrospective Studies; D012805:Sickle Cell Trait / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality