Association of alpha-thalassemia and Glucose-6-Phosphate Dehydrogenase deficiency with transcranial Doppler ultrasonography in Nigerian children with sickle cell anemia

Autor: Oyesola Oyewole Ojewunmi, Mfoniso Godwin Ekpo, Titilope A Adeyemo, Ajoke Idayat Oyetunji, Yewande Benn, Bamidele A. Iwalokun
Rok vydání: 2021
Předmět:
0301 basic medicine
Male
Ultrasonography
Doppler
Transcranial
Clinical Biochemistry
Alpha-thalassemia
Gastroenterology
0302 clinical medicine
hemic and lymphatic diseases
Immunology and Allergy
Child
alpha‐thalassemia
Stroke
Research Articles
Hematology
Prognosis
stroke
Sickle cell anemia
Medical Laboratory Technology
Cerebral blood flow
030220 oncology & carcinogenesis
Cerebrovascular Circulation
Child
Preschool
cardiovascular system
transcranial Doppler ultrasonography
Female
Blood Flow Velocity
Research Article
Microbiology (medical)
medicine.medical_specialty
Adolescent
Nigeria
Anemia
Sickle Cell
03 medical and health sciences
alpha-Thalassemia
sickle cell anemia
Internal medicine
medicine
Humans
glucose‐6‐phosphate dehydrogenase deficiency
business.industry
Biochemistry (medical)
Public Health
Environmental and Occupational Health
Odds ratio
medicine.disease
Confidence interval
030104 developmental biology
Glucosephosphate Dehydrogenase Deficiency
Case-Control Studies
Complication
business
Glucose-6-phosphate dehydrogenase deficiency
Follow-Up Studies
Zdroj: Journal of Clinical Laboratory Analysis
ISSN: 1098-2825
Popis: Background Stroke is a devastating complication of sickle cell anemia (SCA) and can be predicted through abnormally high cerebral blood flow velocity using transcranial Doppler Ultrasonography (TCD). The evidence on the role of alpha‐thalassemia and glucose‐6‐phosphate dehydrogenase (G6PD) deficiency in the development of stroke in children with SCA is conflicting. Thus, this study investigated the association of alpha‐thalassemia and G6PD(A−) variant with abnormal TCD velocities among Nigerian children with SCA. Methods One hundred and forty‐one children with SCA were recruited: 72 children presented with normal TCD (defined as the time‐averaged mean of the maximum velocity: A and 376A > G) were genotyped using restriction fragment length polymorphism—polymerase chain reaction. Results The frequency of α‐thalassemia trait in the children with normal TCD was higher than those with abnormal TCD: 38/72 (52.8%) [α‐/ α α: 41.7%, α ‐/ α ‐: 11.1%] versus 21/69 (30.4%) [α‐/ α α: 27.5%, α ‐/ α ‐: 2.9%], and the odds of abnormal TCD were reduced in the presence of the α‐thalassemia trait [Odds Ratio: 0.39, 95% confidence interval: 0.20–0.78, p = 0.007]. However, the frequencies of G6PDA− variant in children with abnormal and normal TCD were similar (11.6% vs. 15.3%, p = 0.522). Conclusion Our study reveals the protective role of α‐thalassemia against the risk of abnormal TCD in Nigerian children with SCA.
We investigated the association of alpha‐thalassemia deletion and G6PD deficiency [G6PDA‐] with stroke risk as stratified by TCD velocity status among Nigerian children with sickle cell anemia. Our results show that absence of α‐thalassemia increased risk of abnormal TCD and remains an independent predictor of abnormal TCD alongside lower hemoglobin oxygen saturation.
Databáze: OpenAIRE
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