The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: a prospective cohort study
Summary: Background: Sickle cell disease is the most common severe monogenic disorder in humans. In Africa, 50–90% of children born with sickle cell disease die before they reach their fifth birthday. In this study, we aimed to describe the comparative incidence of specific clinical outcomes among...
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2019-10-01
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| Series: | The Lancet Global Health |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2214109X19303286 |
| _version_ | 1818196039885127680 |
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| author | Sophie Uyoga, PhD Alex W Macharia, MSc George Mochamah, MSc Carolyne M Ndila, PhD Gideon Nyutu, BSc Johnstone Makale, BSc Metrine Tendwa, BSc Emily Nyatichi, BSc John Ojal, PhD Mark Otiende, MSc Mohammed Shebe, DipMed Kennedy O Awuondo, BSc Neema Mturi, MMed Norbert Peshu, MMed Benjamin Tsofa, MMed Kathryn Maitland, ProfFMedSci J Anthony G Scott, ProfFMedSci Thomas N Williams, ProfFMedSci |
| author_facet | Sophie Uyoga, PhD Alex W Macharia, MSc George Mochamah, MSc Carolyne M Ndila, PhD Gideon Nyutu, BSc Johnstone Makale, BSc Metrine Tendwa, BSc Emily Nyatichi, BSc John Ojal, PhD Mark Otiende, MSc Mohammed Shebe, DipMed Kennedy O Awuondo, BSc Neema Mturi, MMed Norbert Peshu, MMed Benjamin Tsofa, MMed Kathryn Maitland, ProfFMedSci J Anthony G Scott, ProfFMedSci Thomas N Williams, ProfFMedSci |
| author_sort | Sophie Uyoga, PhD |
| collection | DOAJ |
| description | Summary: Background: Sickle cell disease is the most common severe monogenic disorder in humans. In Africa, 50–90% of children born with sickle cell disease die before they reach their fifth birthday. In this study, we aimed to describe the comparative incidence of specific clinical outcomes among children aged between birth and 5 years with and without sickle cell disease, who were resident within the Kilifi area of Kenya. Methods: This prospective cohort study was done on members of the Kilifi Genetic Birth Cohort Study (KGBCS) on the Indian Ocean coast of Kenya. Recruitment to the study was facilitated through the Kilifi Health and Demographic Surveillance System (KHDSS), which covers a resident population of 260 000 people, and was undertaken between Jan 1, 2006, and April 30, 2011. All children who were born within the KHDSS area and who were aged 3–12 months during the recruitment period were eligible for inclusion. Participants were tested for sickle cell disease and followed up for survival status and disease-specific admission to Kilifi County Hospital by passive surveillance until their fifth birthday. Children with sickle cell disease were offered confirmatory testing and care at a dedicated outpatient clinic. Findings: 15 737 infants were recruited successfully to the KGBCS, and 128 (0·8%) of these infants had sickle cell disease, of whom 70 (54·7%) enrolled at the outpatient clinic within 12 months of recruitment. Mortality was higher in children with sickle cell disease (58 per 1000 person-years of observation, 95% CI 40–86) than in those without sickle cell disease (2·4 per 1000 person-years of observation, 2·0–2·8; adjusted incidence rate ratio [IRR] 23·1, 95% CI 15·1–35·3). Among children with sickle cell disease, mortality was lower in those who enrolled at the clinic (adjusted IRR 0·26, 95% CI 0·11–0·62) and in those with higher levels of haemoglobin F (HbF; adjusted IRR 0·40, 0·17–0·94). The incidence of admission to hospital was also higher in children with sickle cell disease than in children without sickle cell disease (210 per 1000 person-years of observation, 95% CI 174–253, vs 43 per 1000 person-years of observation, 42–45; adjusted IRR 4·80, 95% CI 3·84–6·15). The most common reason for admission to hospital among those with sickle cell disease was severe anaemia (incidence 48 per 1000 person-years of observation, 95% CI 32–71). Admission to hospital was lower in those with a recruitment HbF level above the median (IRR 0·43, 95% CI 0·24–0·78; p=0·005) and those who were homozygous for α-thalassaemia (0·07, 0·01–0·83; p=0·035). Interpretation: Although morbidity and mortality were high in young children with sickle cell disease in this Kenyan cohort, both were reduced by early diagnosis and supportive care. The emphasis must now move towards early detection and prevention of long-term complications of sickle cell disease. Funding: Wellcome Trust. |
| first_indexed | 2024-12-12T01:27:45Z |
| format | Article |
| id | doaj.art-f1875ddea719475f8f263dac2dd214fb |
| institution | Directory Open Access Journal |
| issn | 2214-109X |
| language | English |
| last_indexed | 2024-12-12T01:27:45Z |
| publishDate | 2019-10-01 |
| publisher | Elsevier |
| record_format | Article |
| series | The Lancet Global Health |
| spelling | doaj.art-f1875ddea719475f8f263dac2dd214fb2022-12-22T00:43:04ZengElsevierThe Lancet Global Health2214-109X2019-10-01710e1458e1466The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: a prospective cohort studySophie Uyoga, PhD0Alex W Macharia, MSc1George Mochamah, MSc2Carolyne M Ndila, PhD3Gideon Nyutu, BSc4Johnstone Makale, BSc5Metrine Tendwa, BSc6Emily Nyatichi, BSc7John Ojal, PhD8Mark Otiende, MSc9Mohammed Shebe, DipMed10Kennedy O Awuondo, BSc11Neema Mturi, MMed12Norbert Peshu, MMed13Benjamin Tsofa, MMed14Kathryn Maitland, ProfFMedSci15J Anthony G Scott, ProfFMedSci16Thomas N Williams, ProfFMedSci17KEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, KenyaKEMRI-Wellcome Trust Research Programme, Kilifi, Kenya; Faculty of Medicine, Imperial College, St Mary's Hospital, London, UKKEMRI-Wellcome Trust Research Programme, Kilifi, Kenya; London School of Hygiene & Tropical Medicine, London, UK; INDEPTH Network, Accra, GhanaKEMRI-Wellcome Trust Research Programme, Kilifi, Kenya; London School of Hygiene & Tropical Medicine, London, UK; INDEPTH Network, Accra, Ghana; Correspondence to: Prof Thomas N Williams, KEMRI-Wellcome Trust Research Programme, Centre for Geographic Medicine Research-Coast, Kilifi 80108, KenyaSummary: Background: Sickle cell disease is the most common severe monogenic disorder in humans. In Africa, 50–90% of children born with sickle cell disease die before they reach their fifth birthday. In this study, we aimed to describe the comparative incidence of specific clinical outcomes among children aged between birth and 5 years with and without sickle cell disease, who were resident within the Kilifi area of Kenya. Methods: This prospective cohort study was done on members of the Kilifi Genetic Birth Cohort Study (KGBCS) on the Indian Ocean coast of Kenya. Recruitment to the study was facilitated through the Kilifi Health and Demographic Surveillance System (KHDSS), which covers a resident population of 260 000 people, and was undertaken between Jan 1, 2006, and April 30, 2011. All children who were born within the KHDSS area and who were aged 3–12 months during the recruitment period were eligible for inclusion. Participants were tested for sickle cell disease and followed up for survival status and disease-specific admission to Kilifi County Hospital by passive surveillance until their fifth birthday. Children with sickle cell disease were offered confirmatory testing and care at a dedicated outpatient clinic. Findings: 15 737 infants were recruited successfully to the KGBCS, and 128 (0·8%) of these infants had sickle cell disease, of whom 70 (54·7%) enrolled at the outpatient clinic within 12 months of recruitment. Mortality was higher in children with sickle cell disease (58 per 1000 person-years of observation, 95% CI 40–86) than in those without sickle cell disease (2·4 per 1000 person-years of observation, 2·0–2·8; adjusted incidence rate ratio [IRR] 23·1, 95% CI 15·1–35·3). Among children with sickle cell disease, mortality was lower in those who enrolled at the clinic (adjusted IRR 0·26, 95% CI 0·11–0·62) and in those with higher levels of haemoglobin F (HbF; adjusted IRR 0·40, 0·17–0·94). The incidence of admission to hospital was also higher in children with sickle cell disease than in children without sickle cell disease (210 per 1000 person-years of observation, 95% CI 174–253, vs 43 per 1000 person-years of observation, 42–45; adjusted IRR 4·80, 95% CI 3·84–6·15). The most common reason for admission to hospital among those with sickle cell disease was severe anaemia (incidence 48 per 1000 person-years of observation, 95% CI 32–71). Admission to hospital was lower in those with a recruitment HbF level above the median (IRR 0·43, 95% CI 0·24–0·78; p=0·005) and those who were homozygous for α-thalassaemia (0·07, 0·01–0·83; p=0·035). Interpretation: Although morbidity and mortality were high in young children with sickle cell disease in this Kenyan cohort, both were reduced by early diagnosis and supportive care. The emphasis must now move towards early detection and prevention of long-term complications of sickle cell disease. Funding: Wellcome Trust.http://www.sciencedirect.com/science/article/pii/S2214109X19303286 |
| spellingShingle | Sophie Uyoga, PhD Alex W Macharia, MSc George Mochamah, MSc Carolyne M Ndila, PhD Gideon Nyutu, BSc Johnstone Makale, BSc Metrine Tendwa, BSc Emily Nyatichi, BSc John Ojal, PhD Mark Otiende, MSc Mohammed Shebe, DipMed Kennedy O Awuondo, BSc Neema Mturi, MMed Norbert Peshu, MMed Benjamin Tsofa, MMed Kathryn Maitland, ProfFMedSci J Anthony G Scott, ProfFMedSci Thomas N Williams, ProfFMedSci The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: a prospective cohort study The Lancet Global Health |
| title | The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: a prospective cohort study |
| title_full | The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: a prospective cohort study |
| title_fullStr | The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: a prospective cohort study |
| title_full_unstemmed | The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: a prospective cohort study |
| title_short | The epidemiology of sickle cell disease in children recruited in infancy in Kilifi, Kenya: a prospective cohort study |
| title_sort | epidemiology of sickle cell disease in children recruited in infancy in kilifi kenya a prospective cohort study |
| url | http://www.sciencedirect.com/science/article/pii/S2214109X19303286 |
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