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1.
J Clin Lab Anal ; 35(6): e23802, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33938598

RESUMO

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: < 170 cm/s) and 69 children with abnormal TCD (TAMMV ≥ 200 cm/s). Alpha-thalassemia (the α-3.7 globin gene deletion) was determined by multiplex gap-PCR, while G6PD polymorphisms (202G > 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.


Assuntos
Anemia Falciforme/fisiopatologia , Deficiência de Glucosefosfato Desidrogenase/complicações , Acidente Vascular Cerebral/patologia , Talassemia alfa/complicações , Adolescente , Velocidade do Fluxo Sanguíneo , Estudos de Casos e Controles , Circulação Cerebrovascular , Criança , Pré-Escolar , Feminino , Seguimentos , Deficiência de Glucosefosfato Desidrogenase/diagnóstico por imagem , Deficiência de Glucosefosfato Desidrogenase/patologia , Humanos , Masculino , Nigéria/epidemiologia , Prognóstico , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/etiologia , Ultrassonografia Doppler Transcraniana , Talassemia alfa/diagnóstico por imagem , Talassemia alfa/patologia
2.
Paediatr Int Child Health ; 38(1): 34-39, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-28388354

RESUMO

BACKGROUND: Transcranial Doppler ultrasound (TCD) scan, which measures blood flow velocity through the time-averaged mean of maximum velocities (TAMMVs) in the internal carotid arteries and middle cerebral arteries, is a useful screening tool for predicting stroke risk in children with sickle cell anaemia (SCA). AIM: To investigate which clinical and laboratory indices predict abnormal TCD velocity in children with SCA. METHODS: Fifty-four SCA patients with normal TCD (TAMMV < 170 cm/s), classified as negative TCD (NTCD), and 93 patients with conditional and abnormal TCD velocities (TAMMV ≥ 170 cm/s) classified as positive TCD were recruited. The haemoglobin oxygen saturation, haematological variables, nitric oxide metabolites and lactate dehydrogenase activity of the patients were analysed. RESULTS: The mean (SD) age was 7.16 (3.84) years (range 2-16). The median SpO2 of the patients in the positive TCD group was significantly lower than that of the negative TCD group (p = 0.002). Multivariate logistic regression analysis indicated that the MCV [odds ratio (OR) 1.12, 95% confidence interval (CI) 1.04-1.22, p = 0.01)], MCH (OR 1.34, 95% CI 1.02-1.77, p = 0.04), leucocyte count (OR 1.26, 95% CI 1.07-1.49, p = 0.01) and lactate dehydrogenase (LDH) level (OR 1.00, 95% CI 1.00-1.01, p = 0.01) were independent predictors of high cerebral blood flow velocities. CONCLUSIONS: These clinical and laboratory indices are characteristic of chronic hypoxia and severe anaemia and are predictors of abnormal cerebral blood flow velocity. They can be used to predict stroke risk in children with SCA when access to TCD screening is limited.


Assuntos
Anemia Falciforme/complicações , Circulação Cerebrovascular , Hipóxia Encefálica/diagnóstico , L-Lactato Desidrogenase/análise , Contagem de Leucócitos , Oxiemoglobinas/análise , Acidente Vascular Cerebral/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Nigéria , Prognóstico , Medição de Risco
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