Establishing sickle cell diagnostics and characterizing a paediatric sickle cell disease cohort in Malawi

Objectives. The prevalence of stroke among children with sickle cell disease (SCD) in sub-Saharan Africa was systematically reviewed. Methods. Comprehensive searches of PubMed, Embase, and Web of Science were performed for articles published between 1980 and 2016 (English or French) reporting stroke prevalence. Using preselected inclusion criteria, titles and abstracts were screened and full-text articles were reviewed. Results. Ten full-text articles met selection criteria. Cross-sectional clinic-based data reported 2.9% to 16.9% stroke prevalence among children with SCD. Using available sickle gene frequencies by country, estimated pediatric mortality, and fixed- and random-effects model, the number of affected individuals is projected as 29 800 (95% confidence interval = 25 571-34 027) and 59 732 (37 004-82 460), respectively. Conclusion. Systematic review enabled the estimation of the number of children with SCD stroke in sub-Saharan Africa. High disease mortality, inaccurate diagnosis, and regional variability of risk hamper more precise estimates. Adopting standardized stroke assessments may provide more accurate determination of numbers affected to inform preventive interventions.

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“…Of the sample of 117 (median age was 7.3 years [interquartile range = 2.7-10.4]), 10 (8.5%) had a history of stroke. 18 …”

Section: By Countrymentioning

confidence: 99%

Stroke Prevalence in Children With Sickle Cell Disease in Sub-Saharan Africa: A Systematic Review and Meta-Analysis

Marks

Munube

Kasirye

et al. 2018

Global Pediatric Health

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“…The Chichewa PROMIS‐25 pediatric short form has an incredibly broad scope for implementation throughout Malawi, since it can be used to measure HRQoL for all pediatric patients and is not limited to patients with cancer. For example, there is high prevalence of sickle cell disease among children in Malawi with significantly high associated morbidity and mortality . The pediatric PROMIS measure has recently been validated for use in pediatric patients with sickle cell disease in the United States .…”

Section: Discussionmentioning

confidence: 99%

“…For example, there is high prevalence of sickle cell disease among children in Malawi with significantly high associated morbidity and mortality. 23 The pediatric PROMIS measure has recently been validated for use in pediatric patients with sickle cell disease in the United States. 24,25 This suggests that pediatric PROMIS measures may also be useful for both clinical trials and clinical practice to improve the treatment, management, and care of patients in Malawi, and across SSA, with sickle cell disease.…”

Section: Discussionmentioning

confidence: 99%

Translation, psychometric validation, and baseline results of the Patient‐Reported Outcomes Measurement Information System (PROMIS) pediatric measures to assess health‐related quality of life of patients with pediatric lymphoma in Malawi

Westmoreland

Reeve

Amuquandoh³

et al. 2018

Pediatric Blood & Cancer

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“…Pain was the most common presenting complaints among patients presenting to ED. Vaso-occlusive painful crisis phenomena is a well documented reason for ED visit among sickle cell patients in different settings [ 7 , 8 ], and it has been shown to be a potential marker of serious illness, which may be associated with increased morbidity and mortality [ 7 , 15 ]. Respiratory compromise, denoted by tachypnea and hypoxia (oxygen saturation < 95%), was the most common physical examination finding at presentation.…”

Section: Discussionmentioning

confidence: 99%

The clinical presentation, utilization, and outcome of individuals with sickle cell anaemia presenting to urban emergency department of a tertiary hospital in Tanzania

et al. 2018

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BackgroundSickle cell anaemia (SCA) is prevalent in sub-Saharan Africa, with high risk of complications requiring emergency care. There is limited information about presentation of patients with SCA to hospitals for emergency care. We describe the clinical presentation, resource utilization, and outcomes of SCA patients presenting to the emergency department (ED) at Muhimbili National Hospital (MNH) in Dar es Salaam, Tanzania.MethodsThis was a prospective cohort study of consecutive patients with SCA presenting to ED between December 2014 and July 2015. Informed consent was obtained from all patients or patients’ proxies prior to being enrolled in the study. A standardized case report form was used to record study information, including demographics, relevant clinical characteristics and overall patients outcomes. Categorical variables were compared with chi-square test or Fisher’s exact test; continuous variables were compared with two-sample t-test or Mann-Whitney U-test.ResultsWe enrolled 752 (2.7%) people with SCA from 28,322 patients who presented to the MNH-ED. The median age was 14 years (Interquartile range [IQR]: 6–23 years), and 395 (52.8%) were female. Pain 614 (81.6%), fever 289 (38.4%) were the most frequent presenting complaint. Patients with fever, hypoxia, altered mental status and bradycardia had statistically significant relative risk of mortality of 10.4, 153, 50 and 12.1 (p < 0.0001) respectively, compared to patients with normal vitals. Overall, 656 (87.2%) patients received Complete Blood Cell counts test, of these 342 (52.1%) had severe anaemia (haemoglobin < 7 g/dl), and a 30.3 (p = 0.02) relative risk of relative risk of mortality compare to patients with higher haemoglobin. Patients who had malaria, elevated renal function test and hypoglycemia, had relative risk of mortality of 22.9, 10.4 and 45.2 (p < 0.0001) respectively, compared to patient with normal values. Most 534 (71.0%) patients were hospitalized for in patients care, and the overall morality rate was 16 (2.1%).ConclusionsWe described the clinical presentation, management, and outcomes of patients with SCA presenting to the largest public ED in Tanzania, as well as information on resource utilization. This information can inform development of treatment guidelines, clinical staff education, and clinical research aimed at optimizing care for SCA patients.

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Establishing sickle cell diagnostics and characterizing a paediatric sickle cell disease cohort in Malawi

Cited by 15 publications

References 10 publications

Stroke Prevalence in Children With Sickle Cell Disease in Sub-Saharan Africa: A Systematic Review and Meta-Analysis

Stroke Prevalence in Children With Sickle Cell Disease in Sub-Saharan Africa: A Systematic Review and Meta-Analysis

Translation, psychometric validation, and baseline results of the Patient‐Reported Outcomes Measurement Information System (PROMIS) pediatric measures to assess health‐related quality of life of patients with pediatric lymphoma in Malawi

The clinical presentation, utilization, and outcome of individuals with sickle cell anaemia presenting to urban emergency department of a tertiary hospital in Tanzania

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