Causes of death and early life determinants of survival in homozygous sickle cell disease: The Jamaican cohort study from birth

PloS One
G SerjeantJ Knight-Madden

Abstract

Globally, the majority of persons born with sickle cell disease do not have access to hydroxyurea or more expensive interventions. The objectives were to estimate the survival in homozygous sickle cell disease, unbiased by symptomatic selection and to ascertain the causes of death in a pre-hydroxyurea population. The utility of early life biomarkers and genetically determined phenotypes to predict survival was assessed. A cohort study based on neonatal diagnosis was undertaken at the Sickle Cell Unit, a specialist clinic delivering care to persons with sickle cell disease in Jamaica. Screening of 100,000 deliveries detected 315 babies with homozygous sickle cell disease of whom 311 have been followed from birth for periods up to 43 years. Pneumococcal prophylaxis and teaching mothers splenic palpation were important, inexpensive interventions. Anticipatory guidance, routine care and out-patient acute care were provided. Each participant was classified as alive, dead, or defaulted (usually emigration). Causes of death were ascertained from clinical records and/or post-mortem reports. Survival was assessed using the Kaplan-Meier function. Sex-adjusted Cox semi-parametric proportional hazards and Weibull modelling were used to ass...Continue Reading

References

Jun 10, 1978·British Medical Journal·D W RogersG R Serjeant
Mar 1, 1992·The Journal of Pediatrics·B BalkaranG R Serjeant
Oct 1, 1986·British Journal of Haematology·G R SerjeantH Lehmann
Jan 1, 1984·Annual Review of Genetics·S H Orkin, H H Kazazian
May 26, 1984·British Medical Journal·A B JohnG R Serjeant
Aug 28, 1982·British Medical Journal·A N ThomasG R Serjeant
Jun 9, 1994·The New England Journal of Medicine·O S PlattP P Klug
Dec 16, 1995·BMJ : British Medical Journal·A LeeG Serjeant
Jan 13, 2000·The New England Journal of Medicine·S T MillerT R Kinney
Dec 12, 1959·Nature·K BETKEI SCHLICHT
Aug 4, 2004·British Journal of Haematology·Neal AlexanderGraham R Serjeant
Feb 9, 2007·The New England Journal of Medicine·Graham R SerjeantIan R Hambleton
Oct 11, 2007·Journal of Medical Screening·L KingM Reid
Oct 4, 2011·Archives of Disease in Childhood·Lesley KingMarvin Reid
Mar 2, 2013·Public Health Reports·Sophie LanzkronCarlton Haywood
May 3, 2013·Pediatric Blood & Cancer·Dima Hamideh, Ofelia Alvarez
Jan 31, 2014·American Journal of Hematology·Hany ElmariahMarilyn J Telen
Sep 26, 2014·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Ying WangScott D Grosse
Jul 22, 2016·Blood·Kate GardnerSwee Lay Thein

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Citations

Oct 20, 2018·British Journal of Haematology·Stephan LobitzUNKNOWN with the endorsement of EuroBloodNet, the European Reference Network in Rare Haematological Diseases
Sep 13, 2018·Blood·Swee Lay Thein, Jo Howard
Mar 28, 2019·International Wound Journal·Esaie SoyaSophie Blaise
Jun 25, 2020·Clinical Pharmacology in Drug Development·Brinda K TammaraJoan M Korth-Bradley
Jan 15, 2019·Journal of Medical Case Reports·Angela E Rankine-MullingsMonika Asnani
Jun 9, 2020·Frontiers in Physiology·Gabriel Salinas Cisneros, Swee L Thein
May 29, 2020·The Indian Journal of Medical Research·Dipty JainGraham Roger Serjeant
Feb 12, 2021·Blood Advances·Caterina P MinnitiElizabeth S Klings
Jun 27, 2020·The Journal of Pediatrics·Monica AttiaRobert Sheppard Nickel
Jul 3, 2021·Hematology, Transfusion and Cell Therapy·Alessandra Palhoni SabarenseMarcos Borato Viana
Dec 28, 2021·Psychology, Health & Medicine·Dominique BulginPaula Tanabe

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Methods Mentioned

BETA
electrophoresis
genotyping

Software Mentioned

Stata

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