Screening Cord Bloods for Detection of Sickle Cell Disease in Jamaica

Serjeant, B. E.; Forbes, M.; Williams, Leslie L; Serjeant, G. R.

doi:10.1093/clinchem/20.6.666

Cited by 128 publications

(40 citation statements)

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“…The cord blood survey of newborn infants at the Victoria Jubilee Hospital in Kingston which was used to identify the SS and AA infants examined in this study and the follow-up procedures have been described in detail previously (Serjeant et al, 1974(Serjeant et al, , 1978(Serjeant et al, , 1980(Serjeant et al, , 1981. By April 1980, 266 infants (139 male, 127 female) were entered into the cohort study together with 243 age and sex matched (1 1 8 male, 12 5 female) AA controls.…”

Section: Kurlene P Muson Et Ul Materials and Methodsmentioning

confidence: 99%

Post‐natal decline of fetal haemoglobin in homozygous sickle cell disease: relationship to parental Hb F levels

et al. 1982

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The decline of fetal haemoglobin (Hb F) from birth to 6 years has been compared in a cohort of 266 Jamaican children with homozygous sickle cel (SS) disease and in 243 matched controls with a normal haemoglobin (AA) genotype. Hb F levels were significantly higher in the SS cases from 1 month onward but, unlike the normal controls, no sex difference was apparent. The Hb F levels in SS disease were significantly correlated with parental Hb F levels, suggesting that genetic factors regulating adult Hb F levels are active at earlier stages in development. Furthermore, some of these genetic determinants of Hb F production may be linked to the beta-like globin gene complex and be in linkage disequilibrium with the beta s allele.

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Section: Kurlene P Muson Et Ul Materials and Methodsmentioning

confidence: 99%

Post‐natal decline of fetal haemoglobin in homozygous sickle cell disease: relationship to parental Hb F levels

et al. 1982

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“…Haemoglobin electrophoresis. Cellulose acetate electrophoresis was used to identify the different subtypes of haemoglobin and citrate agar electrophoresis was used to confirm and differentiate the abnormal haemoglobins (Serjeant et al, 1974). Participants with haemoglobin electrophoresis patterns indicating a carrier state (HbAS, HbAC) were not excluded from the study.…”

Section: Phenotypingmentioning

confidence: 99%

Ethnic differences in F cell levels in Jamaica: a potential tool for identifying new genetic loci controlling fetal haemoglobin

et al. 2009

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Summary High levels of fetal haemoglobin (HbF) are protective in β‐haemoglobinopathies. The proportion of erythrocytes containing HbF (F‐cells, FC) was measured in healthy adults of African and Caucasian ancestry to assess the feasibility of localizing genes for the FC trait using admixture mapping. Participants were Afro‐Caribbean (AC) blood donors and residents of a rural enclave with a history of recent German admixture (Afro‐German, AG) recruited in Jamaica, and Caucasian Europeans recruited in Jamaica and the UK. FC levels were significantly different between groups (P < 0·001); the geometric mean FC level in the AC sample (n = 176) was 3·75% [95% confidence interval (CI) 3·36–4·18], AG sample (n = 631) was 2·77% (95% CI 2·63–2·92), and among Caucasians (n = 1099) was 3·26% (95% CI 3·13–3·39). After adjustment for age, sex, haemoglobin electrophoresis pattern, and HBG2 genotype, FC levels in the AC group remained significantly different (P < 0·001) from those in the Caucasian and the AG group but the difference between the Caucasian and AG groups became non‐significant (P = 0·46) despite substantial differences in average ancestry. The data confirm ethnic differences in FC levels and indicate the potential usefulness of these populations for admixture mapping of genes for FC levels.

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“…The patients attended the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica and participated in a Cohort Study from birth (Serjeant et al , 1974, 1986). From June 1973 to December 1981, the screening of 100 000 consecutive deliveries at the main Government maternity hospital (Victoria Jubilee Hospital) identified 315 children with SS disease of whom 311 have been followed prospectively from birth and at the study date (July 2000) were aged 18·5–27·0 years.…”

Section: Patientsmentioning

confidence: 99%

Are there clinical phenotypes of homozygous sickle cell disease?

et al. 2004

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Summary The distribution of clinical features was examined in subjects with homozygous sickle cell (SS) disease in the Jamaican Cohort Study to determine whether there is evidence of distinct clustering of symptoms or clinical phenotypes. A twofold model yielded groups that could be interpreted as painful crisis or leg ulcer phenotypes and 78% of patients were classified with 95% confidence into one of these. The painful crisis phenotype also manifested higher frequencies of dactylitis, meningitis/septicaemia, acute chest syndrome and stroke. Attempts to define a three‐group model were less convincing although 43% of patients could be allocated with 95% confidence. The three‐group model essentially divided subjects with the leg ulcer phenotype into subgroups with higher and lower frequencies of painful crisis, dactylitis, meningitis/septicaemia and acute chest syndrome. In the three‐group model, the painful crisis phenotype had lower total haemoglobin, fetal haemoglobin, mean cell volume and higher reticulocytes but there was no apparent influence of alpha thalassaemia or beta globin haplotype. Both environmental and genetic factors are likely to contribute to most manifestations of SS disease and the evidence for different clinical phenotypes suggests that a search for associated genetic polymorphisms may provide insights into the mechanisms of clinical variability in SS disease.

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Screening Cord Bloods for Detection of Sickle Cell Disease in Jamaica

Cited by 128 publications

References 0 publications

Post‐natal decline of fetal haemoglobin in homozygous sickle cell disease: relationship to parental Hb F levels

Post‐natal decline of fetal haemoglobin in homozygous sickle cell disease: relationship to parental Hb F levels

Ethnic differences in F cell levels in Jamaica: a potential tool for identifying new genetic loci controlling fetal haemoglobin

Are there clinical phenotypes of homozygous sickle cell disease?

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