Sickle cell disease clinical phenotypes in Nigeria: A preliminary analysis of the Sickle Pan Africa Research Consortium Nigeria database

Isa, Hezekiah; Adegoke, Samuel Ademola; Madu, Anazoeze Jude; Hassan, Abdulaziz; Ohiaeri, Chinatu; Chianumba, Reuben; Brown, Biobele J.; Okocha, Emmanuel; Ugwu, Ngozi Immaculata; Diaku-Akinwumi, Ijeoma; Adeyemo, Titilope A.; Kuliya‐Gwarzo, Aisha; Dogara, Livingstone Gayus; Haliru, Lawal; Tanko, Yohanna; Ladu, Adama Isah; Kangiwa, Umar; Ekwem, Lilian; Oniyangi, Seyi; Wakama, Tamunomieibi; Umoru, Domic; Olanrewaju, Olaniyi; Akinola, N. O.; Nnebe‐Agumadu, Uche; Asala, Samuel; Adekile, Adekunle; Olaniyi, John Ayodele; Sangeda, Raphael Zozimus; Nnodu, Obiageli

doi:10.1016/j.bcmd.2020.102438

Cited by 16 publications

(17 citation statements)

References 37 publications

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“…The observed regional differences may be attributable to the already suggested genetic and environmental contributions to the aetiopathogenesis of SLU [13]. Isa et al also reported a lower prevalence of 6.5% in their study, which included children [7]. While it is rare in people below the age of 10 years [14], the frequency of SLU increases with increasing age after the third decade of life [13].…”

Section: Discussionmentioning

confidence: 77%

“…The basic pathology in SCD includes de-oxygenation and polymerization of red cells, causing them to deform and haemolyse, leading to a myriad manifestations from vaso-occlusive crises to more chronic complications like leg ulcers, osteonecrosis, pulmonary hypertension, and renal disease [5][6][7]. An attempt has been made by some investigators to compartmentalize the complications of SCD in either being haemolytic or vaso-occlusive in origin, though in most cases, a significant overlap seems to be present [8,9].…”

Section: Introductionmentioning

confidence: 99%

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Variations and characteristics of the various clinical phenotypes in a cohort of Nigerian sickle cell patients

et al. 2021

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Background: Sickle cell anaemia affects about 4 million people across the globe, making it an inherited disorder of public health importance. Red cell lysis consequent upon haemoglobin crystallization and repeated sickling leads to anaemia and a baseline strain on haemopoiesis. Vaso-occlusion and haemolysis underlies majority of the chronic complications of sickle cell. We evaluated the clinical and laboratory features observed across the various clinical phenotypes in adult sickle cell disease patients. Methods: Steady state data collected prospectively in a cohort of adult sickle cell disease patients as out-patients between July 2010 and July 2020. The information included epidemiological, clinical and laboratory data. Results: About 270 patients were captured in this study (165 males and 105 females). Their ages ranged from 16 to 55 years, with a median age of 25 years. Sixty-eight had leg ulcers, 43 of the males had priapism (erectile dysfunction in 8), 42 had AVN, 31 had nephropathy, 23 had osteomyelitis, 15 had osteoarthritis, 12 had cholelithiasis, 10 had stroke or other neurological impairment, 5 had pulmonary hypertension, while 23 had other complications. Frequency of crisis ranged from 0 to >10/year median of 2. Of the 219 recorded, 148 of the patients had been transfused in the past, while 71 had not. Conclusion:The prevalence of SLU, AVN, priapism, nephropathy and the other complications of SCD show some variations from other studies. This variation in the clinical parameters across different clinical phenotypes indicates an interplay between age, genetic and environmental factors.

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Section: Discussionmentioning

confidence: 77%

Section: Introductionmentioning

confidence: 99%

Variations and characteristics of the various clinical phenotypes in a cohort of Nigerian sickle cell patients

et al. 2021

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“…Africa bears the greatest burden of SCD in the world, as 75% of the >300,000 individuals born with SCD globally every year are born in sub-Saharan Africa 119 . SCD has been reported to increase stroke risk among children and adults in Africa 120 , 121 . There is a bimodal age distribution in the incidence of ischaemic stroke in individuals with SCD: most strokes occur before 20 years and after 30 years of age, with peak incidence at 10–15 years 122 , 123 .…”

Section: Stroke Epidemiology In Africamentioning

confidence: 99%

“…Stroke in individuals with SCD typically presents with transient ischaemic attacks, seizures and focal neurological deficits such as hemiparesis 124 . Cerebral ischaemia in SCD is thought to result from the occlusion of distal internal carotid arteries, middle cerebral arteries and anterior cerebral arteries 120 , 121 , 123 , 124 . Haemorrhagic strokes in SCD usually occur in the third decade of life 121 and are associated with low steady-state haemoglobin levels and high steady-state leukocyte counts 125 .…”

Section: Stroke Epidemiology In Africamentioning

confidence: 99%

“…Cerebral ischaemia in SCD is thought to result from the occlusion of distal internal carotid arteries, middle cerebral arteries and anterior cerebral arteries 120 , 121 , 123 , 124 . Haemorrhagic strokes in SCD usually occur in the third decade of life 121 and are associated with low steady-state haemoglobin levels and high steady-state leukocyte counts 125 . Emerging data from Kumasi, Ghana, indicate higher numbers of stroke admissions and stroke mortality in January–June 2020 than in January–June 2019, suggesting a link between stroke risk and SARS-CoV-2 infection 126 .…”

Section: Stroke Epidemiology In Africamentioning

confidence: 99%

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Stroke in Africa: profile, progress, prospects and priorities

et al. 2021

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Stroke is a leading cause of disability, dementia and death worldwide. Approximately 70% of deaths from stroke and 87% of stroke-related disability occur in low-income and middle-income countries. At the turn of the century, the most common diseases in Africa were communicable diseases, whereas non-communicable diseases, including stroke, were considered rare, particularly in sub-Saharan Africa. However, evidence indicates that, today, Africa could have up to 2–3-fold greater rates of stroke incidence and higher stroke prevalence than western Europe and the USA. In Africa, data published within the past decade show that stroke has an annual incidence rate of up to 316 per 100,000, a prevalence of up to 1,460 per 100,000 and a 3-year fatality rate greater than 80%. Moreover, many Africans have a stroke within the fourth to sixth decades of life, with serious implications for the individual, their family and society. This age profile is particularly important as strokes in younger people tend to result in a greater loss of self-worth and socioeconomic productivity than in older individuals. Emerging insights from research into stroke epidemiology, genetics, prevention, care and outcomes offer great prospects for tackling the growing burden of stroke on the continent. In this article, we review the unique profile of stroke in Africa and summarize current knowledge on stroke epidemiology, genetics, prevention, acute care, rehabilitation, outcomes, cost of care and awareness. We also discuss knowledge gaps, emerging priorities and future directions of stroke medicine for the more than 1 billion people who live in Africa.

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“SCULP” study: The benefits of skin graft pellets on the pain of sickle cell leg ulcers (SCLU)

Caboche-Salouhi,

Le Seac’h,

Lionnet

et al. 2023

JMV-Journal de Médecine Vasculaire

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Sickle cell disease clinical phenotypes in Nigeria: A preliminary analysis of the Sickle Pan Africa Research Consortium Nigeria database

Cited by 16 publications

References 37 publications

Variations and characteristics of the various clinical phenotypes in a cohort of Nigerian sickle cell patients

Variations and characteristics of the various clinical phenotypes in a cohort of Nigerian sickle cell patients

Stroke in Africa: profile, progress, prospects and priorities

“SCULP” study: The benefits of skin graft pellets on the pain of sickle cell leg ulcers (SCLU)

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