Bulletin of Emergency Medicine and Research

Research Article | Open Access

Volume 2023 - 1 | Article ID 237 | http://dx.doi.org/10.51521/BEMR.2024.11101

Effects of Fetal Hemoglobin on Disease Severity in sickle cell disease patients managed at UNIMEDTH, Southwestern Region of Nigeria

Academic Editor: John Bose

  • Received 2023-10-16
  • Revised 2023-11-20
  • Accepted 2023-12-18
  • Published 2024-01-05

PATRICK OLAREWAJU OSHO1, OLAWALE OJO-ROWLAND2, ROSENA OLUBANKE OLUWAFEMI3, OWOJUYIGBE, TEMILOLA OLUDUNMOMI4, PRECIOUS GBAJUOLA JOY5

 

1Department of Haematology and Immunology, University of Medical Sciences, Ondo city, Ondo state, Nigeria

2Department of Microbiology, University of Ibadan, Ibadan, Oyo state, Nigeria

3Department of Paediatrics and Child Health, University of Medical Sciences, Ondo city, Ondo state, Nigeria

4Department of Haematology and Immunology, Obafemi Awolowo University, Ile-Ife, Osun state, Nigeria

5Department of Medical Laboratory Science, University of Medical Sciences, Ondo city, Ondo state, Nigeria

 

Corresponding Author: Dr P.O. Osho, Department of Haematology and Immunology, University of Medical Sciences, Ondo city, Ondo state, Nigeria.

 

Citation: Patrick Olarewaju Osho, Olawale Ojo-Rowland, Rosena Olubanke Oluwafemi, Owojuyigbe, Temilola Oludunmomi, Precious Gbajuola Joy (2024). Hysterosalphingography in Women with Infertility in Ondo, what are the Recent Findings?. Bull Emer Med Res, 1(1),1-7.

Copyright: © 2024, Dr P.O. Osho. This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.

 

ABSTRACT

 

Introduction: Sickle cell disease (SCD) is the most prevalent monogenic complex condition that is clinically diverse. It characterized is by an aberrant haemoglobin S (Hb S) in which glutamic acid at position 6 of the hemoglobin β-globin chain is replaced by valine. The globin gene (HBB; c.20A>T, p.Glu7Val), which produces sickle haemoglobin (HbS) in a tetramer with alpha globin chains, is the mutation that causes it. When HbS is deoxygenated, it possesses the unusual ability to form lengthy, branching polymers. This aberration results in a cascade of reactions in red blood cells that leads to formation of long branching polymers in hypoxic conditions, called tactoids. A strong relationship exists between fetal hemoglobin (Hb F) and disease severity among patients living with SCD. The fetal haemoglobin, (Hb F) is known to be a genetic modulator of SCD. Hb F alleviates the clinical presentations of the disease and its concentration in red blood cells varies greatly in different individuals depending on the genotype and extent of the disease presentation.

Aim of the study: The research aimed to determine and evaluate the influence of fetal haemoglobin on disease severity of the homozygous form of sickle cell disease (SCD) patients at the University of Medical Sciences Teaching Hospital, Akure Complex, Ondo State, Nigeria. The research also looks into the association of Hb F with the socio – demographics and clinical variables of people living with SCD.

Methods: The research was conducted at the University of Medical Sciences Teaching Hospital, Akure Complex, Ondo state, Nigeria. The data were collected and managed using IBM SPSS software version 23.0. Patients were enrolled in the study from the clinics of the hospital department and underwent testing at the Department of Hematology, Faculty of Basic Medical Sciences. The levels of fetal hemoglobin were determined using automated BIO-RAD® D10 high-performance liquid chromatography (HPLC) machine at a private diagnostic centre (POSE Specialist Diagnostic Centre) in Akure, Ondo state, Nigeria.

Results: A total of 88 SCD patients were included in the study. When considering the socioeconomic class, 44.3% of the patients belonged to class I (lower class), 31.8% belonged to class II (middle class), 12.5% belonged to class III (upper class), and 11.4% belonged to class IV (upper upper class). Concerning socio-demographic factors, when considering age, there was a significant association between age groups and HbF levels- 9.1% of the patients were in the 0-10 age group, 18.2% were in the 11-20 age group, 43.2% were in the 21-30 age group, and 29.5% were in the 31-40 age group. Patients in the 0-10 age group with low HbF levels were more likely to have higher pain episodes compared to patients with high HbF levels. In terms of sex, statistically, 55.7% of the patients were male, while 44.3% were female and there was a significant association between sex and HbF levels. Male patients with low HbF levels had a higher likelihood of experiencing more pain episodes compared to male patients with high HbF levels.

Conclusion: The most prevalent monogenic complex condition that is clinically diverse is sickle cell disease and it has haemoglobin F as a genetic modulator. Hydroxyurea is used as a treatment of choice although it is under-prescribed and under-used in our environment. Hydroxyurea is known to increase the level of fetal hemoglobin. Although, larger research is required to advance the topic, which necessarily should include international cooperation between nations and continents

 

Keywords: Haemoglobin S (HbS), Haemoglobin F (HbF), Pain Episodes, Demographic Pattern, UNIMEDTH.

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