Pulmonary hypertension among 5 to 18 year old children with sickle cell anaemia in Nigeria.

Ogochukwu J Sokunbi,Roosevelt Anyanwu,Ekanem N Ekure,Edamisan O Temiye,Christy A N Okoromah,Bamidele O Tayo

doi:10.1371/journal.pone.0184287

Ogochukwu J Sokunbi, Roosevelt Anyanwu + Show 4 more

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https://doi.org/10.1371/journal.pone.0184287

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Journal: PloS one	Publication Date: Sep 14, 2017
Citations: 18	License type: CC BY 4.0

Affiliation: University of Lagos, Lagos University Teaching Hospital

Abstract

BackgroundPulmonary hypertension (PHT) is a significant cause of mortality in patients with sickle cell disease (SCD). Few studies on PHT in SCD have been carried out in children. This study aimed to estimate the prevalence of PHT in children with sickle cell anaemia (SCA) and determine its clinical and laboratory correlates.MethodsIn this cross sectional study, evaluation involved obtaining bio-data, history and physical examination findings in 175 SCA subjects with haemoglobin genotype SS aged 5 to 18 years and 175 age and sex matched controls with haemoglobin genotype AA. PHT was determined using peak Tricuspid Regurgitant Velocity (TRV) obtained from echocardiography as a marker. Complete blood count (CBC), lactate dehydrogenase (LDH) assay, reticulocyte count, foetal haemoglobin (HbF) estimation as well as Human Immunodeficiency Virus (HIV) I and II, Hepatitis B Virus (HBV) and Hepatitis C Virus (HCV) screening were done for patients with SCA.ResultsThe mean peak TRV of subjects with SCA and controls was 2.2 ± 0.4 m/s and 1.9 ± 0.3 m/s respectively and prevalence of PHT among children with SCA and controls was 22.9% and 2.3% respectively. PHT in SCA correlated negatively with body mass index, haematocrit and haemoglobin.ConclusionThis study affirms that PHT prevalence is high in children with SCA in Nigeria. Cardiovascular examination for signs of PHT is recommended for children with SCA and if required, further echocardiographic assessment from as early as five years.

Highlights

Sickle cell disease (SCD) affects about 20–25 million individuals worldwide mostly of African, South and Central American, Caribbean, Saudi Arabian, Indian and Mediterranean ancestry. [1] It is most prevalent in sub Saharan Africa with an estimated 12–15 million cases in the region and affects two to three percent of Nigerians.[1]
Complete blood count (CBC), lactate dehydrogenase (LDH) assay, reticulocyte count, foetal haemoglobin (HbF) estimation as well as Human Immunodeficiency Virus (HIV) I and II, Hepatitis B Virus (HBV) and Hepatitis C Virus (HCV) screening were done for patients with sickle cell anaemia (SCA)
This study affirms that Pulmonary hypertension (PHT) prevalence is high in children with SCA in Nigeria

Summary

Introduction

Sickle cell disease (SCD) affects about 20–25 million individuals worldwide mostly of African, South and Central American, Caribbean, Saudi Arabian, Indian and Mediterranean ancestry. [1] It is most prevalent in sub Saharan Africa with an estimated 12–15 million cases in the region and affects two to three percent of Nigerians.[1]. Pulmonary hypertension (PHT) is defined as elevated mean pulmonary artery pressures measured during right sided cardiac catheterization.[5, 6] Measurement of TRV by echocardiography provides a non-invasive screening tool for the assessment of PHT and has been recommended as the initial non-invasive modality in the screening and evaluation of PHT. In SCD, this estimated pulmonary systolic pressure correlates well with measurements obtained by cardiac catheterization.[9] A value of 2.5 m /s or more corresponds to an estimated pulmonary artery systolic pressure of 35 mm Hg, which is approximately 2 SD above the normal mean value; for patients less than 40 years of age. Pulmonary hypertension (PHT) is a significant cause of mortality in patients with sickle cell disease (SCD). This study aimed to estimate the prevalence of PHT in children with sickle cell anaemia (SCA) and determine its clinical and laboratory correlates.

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