Background: Glucose-6-phosphate dehydrogenase deficiency is an enzymopathy characterized by insufficient production of reduced glutathione (GSH), a molecule known for its antioxidant role. This lack of GSH leads to a ...Background: Glucose-6-phosphate dehydrogenase deficiency is an enzymopathy characterized by insufficient production of reduced glutathione (GSH), a molecule known for its antioxidant role. This lack of GSH leads to a deficit in the elimination of peroxide ions from the red blood cells, causing thereby hemolytic accidents, which can be fatal if not properly managed. In neonates, the clinical picture is most often that of neonatal jaundice. Objectives: This study aimed to determine the place of G6PD deficiency as a cause of neonatal jaundice at Essos Hospital Centre. Methods: We conducted a prospective descriptive study over three months. Blood samples taken from newborns aged 0 to 28 days were analyzed in the medical analysis laboratory of the Essos Hospital Centre in Yaoundé. We carried out a determination of the enzymatic activity of G6PD, a blood count and the determination of the bilirubin level. The results obtained were analysed using R statistical software version 4.1.1. Linear regression analyses were used to assess correlations between the variables of interest. Results: Sixty-nine icteric neonates constituted our study population, with a total of 40 boys (58%) and 29 girls (42%) with a sex ratio of 1.37 in favour of boys. The prevalence of G6PD deficiency in icteric children was 50.72%. The mean hemoglobin was 15.3 ± 3.08 g/dL and the mean red blood cell count was 4.52 ± 1.01 × 10<sup>6</sup>/mm<sup>3</sup>. The mean total bilirubin was 122 ± 48.3 mg/L with a maximum of 308 mg/L and the mean free bilirubin was 104 ± 46.6 mg/L with a maximum of 292 mg/L. Furthermore, after linear regression analysis, we obtained a positive and significant correlation between G6PD enzymatic activity and hemoglobin level (r = 0.33;p ≤ 0.001), G6PD red blood cell level (r = 0.26;p Conclusion: Neonatal jaundice in G6PD-deficient children is a real public health problem and the prevention of hemolysis in children requires an early diagnosis of the enzyme disorder and good follow-up of the children.展开更多
Introduction: Bilirubin encephalopathy is a debilitating complication of severe neonatal unconjugated bilirubin jaundice. The aim of this study was to determine the frequency of occurrence of this complication and to ...Introduction: Bilirubin encephalopathy is a debilitating complication of severe neonatal unconjugated bilirubin jaundice. The aim of this study was to determine the frequency of occurrence of this complication and to describe the diagnostic and prognostic aspects in Senegal. Materials and Methods: This was a multicenter, descriptive and analytical study conducted in 32 neonatal units of various levels, with retrospective (January to December 2020) and prospective (January to August 2021) collection. All neonates hospitalized for acute bilirubin encephalopathy were included. The diagnostic criteria were the presence of jaundice associated with neurological signs (muscle tone disorders, abnormal movements, convulsions, etc.), with no other obvious etiology found. The data were entered and analyzed using SPSS version 23 software. The significance threshold was retained for a p-value Results: We registered 151 patients. The mean age was 6 days and the sex ratio was 2.5 (clear male predominance). The majority of newborns were from Dakar (51%). Twenty-two (22%) were premature and 32% had low birth weight. Half of the newborns (50%) came from home and 87% were exclusively breastfed. The average time to consult was 3 days. Neurological signs were dominated by decreased primitive reflexes (74%), abnormal movements (59%) and lethargy (50%). Dehydration (30%) and anemia (26%) were often associated. The mean bilirubin level was 416 umol/l. Neonatal infections (19%) and Rhesus (16.7%) and ABO (8.7) incompatibility were the main causes. One third (33%) of patients had received intensive phototherapy and only 2% had received exchange blood transfusion. The case fatality was 48.9%. Conclusion: Bilirubin encephalopathy remains a major concern in Senegal. It is associated with high mortality and numerous neurological sequelae. Progress in terms of early detection and appropriate management is urgently needed on a national scale.展开更多
文摘Background: Glucose-6-phosphate dehydrogenase deficiency is an enzymopathy characterized by insufficient production of reduced glutathione (GSH), a molecule known for its antioxidant role. This lack of GSH leads to a deficit in the elimination of peroxide ions from the red blood cells, causing thereby hemolytic accidents, which can be fatal if not properly managed. In neonates, the clinical picture is most often that of neonatal jaundice. Objectives: This study aimed to determine the place of G6PD deficiency as a cause of neonatal jaundice at Essos Hospital Centre. Methods: We conducted a prospective descriptive study over three months. Blood samples taken from newborns aged 0 to 28 days were analyzed in the medical analysis laboratory of the Essos Hospital Centre in Yaoundé. We carried out a determination of the enzymatic activity of G6PD, a blood count and the determination of the bilirubin level. The results obtained were analysed using R statistical software version 4.1.1. Linear regression analyses were used to assess correlations between the variables of interest. Results: Sixty-nine icteric neonates constituted our study population, with a total of 40 boys (58%) and 29 girls (42%) with a sex ratio of 1.37 in favour of boys. The prevalence of G6PD deficiency in icteric children was 50.72%. The mean hemoglobin was 15.3 ± 3.08 g/dL and the mean red blood cell count was 4.52 ± 1.01 × 10<sup>6</sup>/mm<sup>3</sup>. The mean total bilirubin was 122 ± 48.3 mg/L with a maximum of 308 mg/L and the mean free bilirubin was 104 ± 46.6 mg/L with a maximum of 292 mg/L. Furthermore, after linear regression analysis, we obtained a positive and significant correlation between G6PD enzymatic activity and hemoglobin level (r = 0.33;p ≤ 0.001), G6PD red blood cell level (r = 0.26;p Conclusion: Neonatal jaundice in G6PD-deficient children is a real public health problem and the prevention of hemolysis in children requires an early diagnosis of the enzyme disorder and good follow-up of the children.
文摘Introduction: Bilirubin encephalopathy is a debilitating complication of severe neonatal unconjugated bilirubin jaundice. The aim of this study was to determine the frequency of occurrence of this complication and to describe the diagnostic and prognostic aspects in Senegal. Materials and Methods: This was a multicenter, descriptive and analytical study conducted in 32 neonatal units of various levels, with retrospective (January to December 2020) and prospective (January to August 2021) collection. All neonates hospitalized for acute bilirubin encephalopathy were included. The diagnostic criteria were the presence of jaundice associated with neurological signs (muscle tone disorders, abnormal movements, convulsions, etc.), with no other obvious etiology found. The data were entered and analyzed using SPSS version 23 software. The significance threshold was retained for a p-value Results: We registered 151 patients. The mean age was 6 days and the sex ratio was 2.5 (clear male predominance). The majority of newborns were from Dakar (51%). Twenty-two (22%) were premature and 32% had low birth weight. Half of the newborns (50%) came from home and 87% were exclusively breastfed. The average time to consult was 3 days. Neurological signs were dominated by decreased primitive reflexes (74%), abnormal movements (59%) and lethargy (50%). Dehydration (30%) and anemia (26%) were often associated. The mean bilirubin level was 416 umol/l. Neonatal infections (19%) and Rhesus (16.7%) and ABO (8.7) incompatibility were the main causes. One third (33%) of patients had received intensive phototherapy and only 2% had received exchange blood transfusion. The case fatality was 48.9%. Conclusion: Bilirubin encephalopathy remains a major concern in Senegal. It is associated with high mortality and numerous neurological sequelae. Progress in terms of early detection and appropriate management is urgently needed on a national scale.
