Factors associated with blood pressure variation in sickle cell disease patients: a systematic review and meta-analyses

Figures & data

Figure 1. Flow chart of study selection.

Figure 2. BP indices in SCD patients versus controls, (a) = systolic BP, (b) = diastolic BP, (c) = mean arterial pressure; (d) = Pulse rate, (e) = Pulse pressure.

Table 2. Characteristics of included studies

Studies	Year	Countries	Study settings	Sample size	Methodology	Charateristics of study participants	Outcomes	Identified factors
Bhatnagar [26]	2013	United States (USA)	SCD cohorts of African American ancestry	1,617 patients	Genome-wide meta-analysis	This study includes two African American ancestry SCD cohorts. Gender: 843 males, 774 females. Average age was 8.96 from one cohort and 9.57 years from the second cohort	Systolic blood pressure (SBP)	Genetic factors
Campbell [13]	2007	Jamaica	SCD clinic	51 subjects with HbSC and 88 subjects with HbSS	Cross-sectional	51 subjects with HbSC (29 males, 22 females) and 88 subjects with HbSS (43 males, 45 females) Age: Age ranged from 23.1 to 31.6 years.	Blood pressure (BP) and BP by genotype	Anthropometric, hematological and renal functional factors
Desai [15]	2012	USA	SCD patients cohort at University of North Carolina (UNC) clinic	156 SCD patients in theUNC cohort	Cross-sectional	Age: Mean age was 36 years (range 27–47) Gender: 97 (62%) females Ethinicity: 154 (99%) african american	SBP and diastolic blood pressure (DBP)	Clinical and laboratory factors
Gordeuk [10]	2008	USA	Patients from Sickle Cell Pulmonary Hypertension Screening Study	163 SCD patients	Retrospective cohort cohort	Adults with hemoglobin SS or Sβ0 thalassemia patients	Relative systemic hypertension (RSH) (i.e. SBP 120–139 mm Hg or DBP 70–89 mm Hg)	Complications associated
Grell [14]	1981	Jamaica	SCDclinic of the University Hospital in Jamaica.	70 SCD patients	Retrospective cohort	Participants were aged over 30 years. Gender: 29 males and 41 females. Age: age ranged from 30 to 69 years.	SBP and DBP	Age and gender
Homi [21]	1993	Jamaica	SCD clinic of the University Hospital of the West Indies, Kingston, Jamaica.	342 SCD patients	Retrospective cohort	Adults aged 9.5 to 18.5 years.220 with homozygous SS and 122 with hemoglobin C	BP	Weight
Johnson [18]	1981	USA	SCD clinics at the Los Angeles County-University of Southern California Medical Center and The Martin Luther King, Jnr General Hospital.	187 SCD patients	Cross-sectional	Gender: 81 males, 106 females Age: mean age was 29 years (range 18–67). 130 sickle cell anemia, 43 sickle cell-hemoglobin C, 15 had sickle cell thalassemia	Prevalence and severity of hypertension in SCD	Hemoglobinopathy, age and advancing age
Strumph [28]	2021	USA	Children’s hospital	100 patients with SCD	Cross-sectional	100 SCD patients with age ranging from 5 to 21	Nocturnal hypertension	Complication associated
Kuma [16]	2018	Ghana	The Ghana Institute of Clinical Genetics	875 with homozygous or compound heterozygous SCD	Retrospective cohort	Participants were adults aged >18 years. Gender: 63% were females. Age: Median age was 31 (range 23–44)	Prevalence of RSH	Complications associated
Lamarre [20]	2013	(Guadeloupe, French West Indies)	SCD Center at the Academic Hospital of Pointe-a`-Pitre.	97 SCD patients	Cross-sectional	Adults aged ≥18 years. Gender: 43 male and 54 females	RSH and systematic hypertension	Biological factors
Lemonne [24]	2014	(Guadeloupe, French West Indies).	SCD Center at the Academic Hospital of Pointe-a`-Pitre.	89 SCD patients (sickle cell-hemoglobin C)	Cross-sectional	Gender: 40 males, 49 females Age: mean age was 38 ± 13 years	RSH	Blood Viscosity
Makubi [17]	2017	Tanzania	Patient were identified at pediatric or hematology clinics or during hospitalization	1013 SCD patients	Retrospective cohort	Gender: 56% were females Age: Median age was 17 (range15–22)	RSH and systematic hypertension	Any risk factors
Novelli [25]	2014	USA	Multicentre international sickle cell trial (USA and United Kingdom)	661 SCD patients	Retrospective cohort	Participants were adults: 500 HbSS and 161 HbSC, with mean age34 (range 25–45) and 41 (range 28–51)	Pulse pressure	Markers of hemolysis
Oguanobi [19]	2010	Nigeria	Outpatient sickle cell clinics and the cardiac center of the University of Nigeria Teaching Hospital, Enugu, Nigeria	62 SCD patients	Cross-sectional	Participants were adults. Age: Mean age was 28.27(range 18–44)	Arterial BP	Age, gender, and anthropometric data
Pegelow [23]	1997	USA	Cooperative Study of Sickle Cell Disease (CSSCD) at at 23 clinical centers, USA	3,317 SCD patients	Cross-sectional	Participants included were children and adults (2 years old or older)	BP	Age, gender, anthropometric, clinical and laboratory data
Pikilidou [22]	2015	Greece	SCD center	45 SCD patients	Cross-sectional	Participants were adults. Age: Mean age was 43 years (range 26–65) Body mass index (BMI):(24.1 ± 3.6 kg/m2 Gender: 30 males and 15 females	BP	Arterial stiffness

Table 1. Risk of bias in the included studies

Reference	Q1	Q2	Q3	Q4	Q5	Q6	Q7	Q8	Q9	Risk of bias score	Risk of bias
Genetic data
Bhatnagar	No	Yes	Yes	Yes	Yes	Yes	No	Yes	Yes	7	Moderate
Descriptive/Cross-sectional studies
Campbell	No	Yes	Yes	Yes	Yes	NA	No	Yes	Yes	6	Moderate
Desai	No	No	No	Yes	Yes	NA	No	Yes	Yes	4	High
Johnson	No	Yes	Yes	Yes	Yes	NA	No	Yes	Yes	6	Moderate
Strumph	No	Yes	Yes	Yes	Yes	NA	No	Yes	Yes	6	Moderate
Lamarre	No	Yes	Yes	Yes	Yes	NA	No	Yes	Yes	6	Moderate
Lemonne	No	Yes	Yes	Yes	Yes	NA	No	Yes	Yes	6	Moderate
Oguanobi	No	No	Yes	Yes	Yes	NA	No	Yes	Yes	5	High
Pegelow	No	Yes	Yes	Yes	Yes	NA	No	Yes	Yes	6	Moderate
Pikilidou	No	Yes	Yes	Yes	Yes	NA	No	Yes	Yes	6	Moderate
Cohort/Case control studies
Gordeuk	Yes	Yes	Yes	No	Yes	Yes	No	Yes	Yes	7	Moderate
Grell	No	Yes	Yes	Unclear	Yes	Yes	Unclear	Yes	Yes	6	Moderate
Homi	No	Yes	Yes	Yes	Yes	Yes	Unclear	Yes	Yes	7	Moderate
Kuma	Yes	Yes	Yes	Yes	Yes	Yes	Unclear	Yes	Yes	8	Low
Makubi	No	Yes	Yes	Yes	Yes	Yes	Unclear	Yes	Yes	7	Moderate
Novelli	Yes	Yes	Yes	Yes	Yes	Yes	Unclear	Yes	Yes	8	Low

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