Glucose-6-phosphate Dehydrogenase Levels Research Articles

Rate of glucose-6-phosphate dehydrogenase deficiency in neonatal indirect hyperbilirubinemia at a private tertiary centre.

To investigate the rate of hospitalized neonates with glucose-6-phosphate dehydrogenase (G6PD) deficiency presented with indirect hyperbilirubinemia at a private tertiary center in Al-Ahsa, Saudi Arabia, over 4 years and to compare the characteristics of G6PD-deficient and normal neonates admitted for indirect hyperbilirubinemia. The retrospective case control study was carried out at Almoosa Specialist Hospital, Al-Ahsa, Saudi Arabia. Data were collected from Yassasi Medical System from 2018-2021 and finalized in 2024. The study included 2 groups: G6PD-normal and G6PD-deficient neonates with indirect hyperbilirubinemia not having recognizable triggers of hemolysis. The analysis focused on serum bilirubin levels, direct bilirubin levels, hematocrit levels, hemoglobin levels, reticulocyte percentage, G6PD levels, duration of phototherapy, and the need for exchange transfusion. The study enrolled 3200 neonates with hyperbilirubinemia, of whom 274 met inclusion criteria. A total of 103 (37.6%) neonates were G6PD-deficient, with 77 (74.8%) being male and 26 (25.2%) female. Glucose-6-phosphate dehydrogenase-deficient neonates exhibited significantly higher initial total bilirubin levels and earlier sampling times. There was no significant correlation between G6PD deficiency and hematocrit or hemoglobin levels in hyperbilirubinemic neonates, but 4 neonates required exchange transfusion, demonstrating statistical significance (p=0.009). High rate of G6PD deficiency in neonates with indirect hyperbilirubinemia, requiring close monitoring to prevent exchange transfusions, with no significant differences in hematocrit or hemoglobin levels.

Alterations of urine microRNA-7977/G6PD level in patients with diabetic kidney disease and its association with dysfunction of albumin-induced autophagy in proximal epithelial tubular cells.

Diabetic kidney disease (DKD) remains as one of the leading long-term complications of type 2 diabetic mellitus (T2DM). Studies have shown that decreased expression of glucose-6-phosphate dehydrogenase (G6PD) plays an important role in DKD. However, the upstream and downstream pathways of G6PD downregulation leading to DKD have not been elucidated. We conducted a series of studies including clinical study, animal studies, and in vitro studies to explore this. First, a total of 90 subjects were evaluated including 30 healthy subjects, 30 patients with T2DM, and 30 patients with DKD. The urinary G6PD activity and its association with the clinical markers were analyzed. Multivariate linear regression analysis was used to analyze the risk factors of urinary G6PD in these patients. Then, microRNAs that were differentially expressed in urine and could bind and degrade G6PD were screened and verified in patients with DKD. After that, high glucose (HG)-cultured human kidney cells (HK-2) and Zucker diabetic fatty (ZDF) rats were used to test the roles of miR-7977/G6PD/albumin-induced autophagy in DKD. Beclin and P62 were used as markers of kidney autophagy indicators. A dual-luciferase reporter assay system was used to test the binding of G6PD by mir-7977. The plasma and urinary G6PD activity were decreased significantly in patients with DKD, accompanied by increased urinary mir-7977 level. The fasting plasma glucose (FPG), triglyceride (TG), low-density lipoprotein cholesterol (LDL-C), and urinary albumin excretion were independent predictors of urinary G6PD activity, according to multiple linear regression analysis. The increased expression of miR-7977 and decreased expression of G6PD were also found in the kidney of ZDF rats with early renal tubular damage. The correlation analysis showed that beclin protein expression levels were positively correlated with kidney G6PD activity, whereas P62 protein expression was negatively correlated with kidney G6PD activity in rats. In HK-2 cells cultured with normal situation, a low level of albumin could induce autophagy along with the stimulation of G6PD, although this was impaired under high glucose. Overexpression of G6PD reversed albumin-induced autophagy in HK-2 cells under high glucose. Further study revealed that G6PD was a downstream target of miR-7977. Inhibition of miR-7977 expression led to significantly increased expression of G6PD and reversed the effects of high glucose on albumin-induced autophagy. In conclusion, our study supports a new mechanism of G6PD downregulation in DKD. Therapeutic measures targeting the miR-7977/G6PD/autophagy signaling pathway may help in the prevention and treatment of DKD.NEW & NOTEWORTHY This study provides new evidence that reduced glucose-6-phosphate dehydrogenase (G6PD) may damage the endocytosis of renal tubular epithelial cells by reducing albumin-induced autophagy. More importantly, for the first time, our study has provided evidence from humans that the decrease in urinary G6PD activity is positively associated with renal injury, and abnormal glucose and lipid metabolism may be important reasons for reduced G6PD levels. Increased miR-7977 may at least in part explain the downregulation of G6PD.

The effect of single low-dose primaquine treatment for uncomplicated Plasmodium falciparum malaria on haemoglobin levels in Ethiopia: a longitudinal cohort study

BackgroundTo interrupt residual malaria transmission and achieve successful elimination of Plasmodium falciparum in low-transmission settings, the World Health Organization (WHO) recommends the administration of a single dose of 0.25 mg/kg (or 15 mg/kg for adults) primaquine (PQ) combined with artemisinin-based combination therapy (ACT), without glucose-6-phosphate dehydrogenase (G6PD) testing. However, due to the risk of haemolysis in patients with G6PD deficiency (G6PDd), PQ use is uncommon. Thus, this study aimed to assess the safety of a single low dose of PQ administered to patients with G6PD deficiency.MethodsAn observational cohort study was conducted with patients treated for uncomplicated P. falciparum malaria with either single-dose PQ (0.25 mg/kg) (SLD PQ) + ACT or ACT alone. Microscopy-confirmed uncomplicated P. falciparum malaria patients visiting public health facilities in Arjo Didessa, Southwest Ethiopia, were enrolled in the study from September 2019 to November 2022. Patients with uncomplicated P. falciparum malaria were followed up for 28 days through clinical and laboratory diagnosis, such as measurements of G6PD levels and haemoglobin (Hb) concentrations. G6PD levels were measured by a quantiative CareSTART™ POCT S1 biosensor machine. Patient interviews were also conducted, and the type and frequency of clinical complaints were recorded. Hb data were taken on days (D) 7, 14, 21, and 28 following treatment with SLD-PQ + ACT or ACT alone.ResultsA total of 249 patients with uncomplicated P. falciparum malaria were enrolled in this study. Of these, 83 (33.3%) patients received ACT alone, and 166 (66.7%) received ACT combined with SLD-PQ treatment. The median age of the patients was 20 (IQR 28–15) years. G6PD deficiency was found in 17 (6.8%) patients, 14 males and 3 females. There were 6 (7.2%) and 11 (6.6%) phenotypic G6PD-deficient patients in the ACT alone and ACT + SLD-PQ arms, respectively. The mean Hb levels in patients treated with ACT + SLD-PQ were reduced by an average of 0.45 g/dl (95% CI = 0.39 to 0.52) in the posttreatment phase (D7) compared to a reduction of 0.30 g/dl (95% CI = 0.14 to − 0.47) in patients treated with ACT alone (P = 0.157). A greater mean Hb reduction was observed on day 7 in the G6PDd ACT + SLD-PQ group (− 0.60 g/dL) than in the G6PDd ACT alone group (− 0.48 g/dL); however, there was no statistically significant difference (P = 0.465). Overall, D14 losses were 0.10 g/dl (95% CI = − 0.00 to 0.20) and 0.05 g/dl (95% CI = − 0.123 to 0.22) in patients with and without SLD-PQ, respectively (P = 0.412).ConclusionsThis study’s findings indicate that using SLD-PQ in combination with ACT is safe for uncomplicated P. falciparum malaria regardless of the patient's G6PD status in Ethiopian settings. Caution should be taken in extrapolating this finding in other settings with diverse G6DP phenotypes.

Associations between COVID-19 and Glucose-6-Phosphate Dehydrogenase Activity in Brazil.

Glucose-6 phosphate dehydrogenase deficiency (G6PDd) was suggested as a risk factor for severe disease in patients with COVID-19. We evaluated clinical outcomes and glucose-6 phosphate dehydrogenase (G6PD) activity during and after illness in patients with COVID-19. This prospective cohort study included adult participants (≥18 years old) who had clinical and/or radiological COVID-19 findings or positive reverse transcription-polymerase chain reaction results. Epidemiological and clinical data were extracted from electronic medical records. Glucose-6 phosphate dehydrogenase activity was measured using SD Biosensor STANDARD G6PD® equipment on admission and 1 year after discharge. Samples were genotyped for the three most common single nucleotide polymorphisms for G6PDd in the Brazilian Amazon. Seven hundred fifty-three patients were included, of whom 123 (16.3%) were G6PD deficient. There was no difference between groups regarding the risks of hospitalization (P = 0.740) or invasive mechanical ventilation (P = 0.31), but the risk of death was greater in patients with normal G6PD levels (P = 0.022). Only 29 of 116 participants (25%) carried the African G6PDd genotype. Of 30 participants tested as G6PD deficient during disease, only 11 (36.7%) results agreed 1 year after discharge. In conclusion, this study does not demonstrate an association of G6PDd with severity of COVID-19. Limitations of the test for detecting enzyme levels during COVID-19 illness were demonstrated by genotyping and retesting after the disease period. Care must be taken when screening for G6PDd in patients with acute COVID-19.

G6PD maintains the VSMC synthetic phenotype and accelerates vascular neointimal hyperplasia by inhibiting the VDAC1-Bax-mediated mitochondrial apoptosis pathway.

Glucose-6-phosphate dehydrogenase (G6PD) plays an important role in vascular smooth muscle cell (VSMC) phenotypic switching, which is an early pathogenic event in various vascular remodeling diseases (VRDs). However, the underlying mechanism is not fully understood. An IP‒LC‒MS/MS assay was conducted to identify new binding partners of G6PD involved in the regulation of VSMC phenotypic switching under platelet-derived growth factor-BB (PDGF-BB) stimulation. Co-IP, GST pull-down, and immunofluorescence colocalization were employed to clarify the interaction between G6PD and voltage-dependent anion-selective channel protein 1 (VDAC1). The molecular mechanisms involved were elucidated by examining the interaction between VDAC1 and apoptosis-related biomarkers, as well as the oligomerization state of VDAC1. The G6PD level was significantly elevated and positively correlated with the synthetic characteristics of VSMCs induced by PDGF-BB. We identified VDAC1 as a novel G6PD-interacting molecule essential for apoptosis. Specifically, the G6PD-NTD region was found to predominantly contribute to this interaction. G6PD promotes VSMC survival and accelerates vascular neointimal hyperplasia by inhibiting VSMC apoptosis. Mechanistically, G6PD interacts with VDAC1 upon stimulation with PDGF-BB. By competing with Bax for VDAC1 binding, G6PD reduces VDAC1 oligomerization and counteracts VDAC1-Bax-mediated apoptosis, thereby accelerating neointimal hyperplasia. Our study showed that the G6PD-VDAC1-Bax axis is a vital switch in VSMC apoptosis and is essential for VSMC phenotypic switching and neointimal hyperplasia, providing mechanistic insight into early VRDs.

Hemolytic Anemia Due to Glucose-6-Phospate Dehydrogenase Deficiency in Neonate

Glucose-6-phosphate dehydrogenase (G6PD) is an enzyme in erythrocytes that functions to maintain glycolysis, form an erythrocyte membrane, and protect hemoglobin from oxidative denaturation. Clinical manifestations of anemia and severe jaundice are risk factors for acute hyperbilirubinemia encephalopathy, which can progress to kernicterus. G6PD deficiency is X-linked. The incidence is most common in males with an incidence in Indonesia of 1-14%. A 3-day-old male baby appeared yellowish and had a history of being pale since 1 day of age. There was a history of phototherapy and packed red blood cell transfusion from a previous hospital. The baby was born via caesarean section, term baby, with a birth weight of 2700 grams. The Physical examination revealed Kramer V, tachypnea, but no hepatosplenomegaly. A previous laboratory examination at AM Hospital showed total bilirubin of 23.3 mg/dL, indirect bilirubin 23 mg/dL, and pre-transfusion hemoglobin 9.8 g/dL. Chest X-rays revealed grade I–II hyaline membrane disease. Meanwhile, the laboratory test results at the Dr Wahidin Sudirohusodo Hospital showed a total bilirubin of 38.5 mg/dL, indirect bilirubin 37.17 mg/dL, reticulocytes 9.95%, hemoglobin 11.8 g/dL, CRP 5.2 mg/L, Procalcitonin 0.5 ng/mL, and a negative Coombs test. The peripheral blood smear showed fragmented cells of erythrocytes. The G6PD level was 17.2 U/dL (reference value >20.5 U/dL). The patient received low-flow oxygenation, antibiotics, and 72 hours of intensive phototherapy. On the 7th day of treatment, the condition improved, and the baby was discharged. Therefore, Rapid diagnosis and treatment of G6PD deficiency are important to prevent other potential complication such as acute hyperbilirubinemia encephalopathy.

Comparative effects of pravastatin and rosuvastatin on carbohydrate metabolism in an experimental diabetic rat model.

Statin treatment may increase the risk of diabetes; there is insufficient data on how statins affect glucose regulation and glycemic control and the effects of statins on liver enzymes related to carbohydrate metabolism have not been fully studied. Therefore, we aimed to compare the effects of the statin derivatives, pravastatin, and rosuvastatin, on carbohydrate metabolism in an experimental diabetic rat model. Female Wistar albino rats were used and diabetes was induced by intraperitoneal injection of streptozotocin. Thereafter, 10 and 20 mg kg-1 day-1 doses of both pravastatin and rosuvastatin were administered by oral gavage to the diabetic rats for 8 weeks. At the end of the experiment, body masses, the levels of fasting blood glucose, serum insulin, insulin resistance (HOMA-IR), liver glycogen, and liver enzymes related to carbohydrate metabolism were measured. Both doses of pravastatin significantly in creased the body mass in diabetic rats, however, rosuvastatin, especially at the dose of 20 mg kg-1 day-1 reduced the body mass signi ficantly. Pravastatin, especially at a dose of 20 mg kg-1 day-1, caused significant increases in liver glycogen synthase and glucose 6-phosphate dehydrogenase levels but significant decreases in the levels of glycogen phosphorylase, lactate dehydrogenase, and glucose-6-phosphatase. Hence, pravastatin partially ameliorated the adverse changes in liver enzymes caused by diabetes and, especially at the dose of 20 mg kg-1 day-1, reduced the fasting blood glucose level and increased the liver glycogen content. However, rosuvastatin, especially at the dose of 20 mg kg-1 day-1, significantly reduced the liver glycogen synthase and pyruvate kinase levels, but increased the glycogen phosphorylase level in diabetic rats. Rosuvastatin, 20 mg kg-1 day-1 dose, caused significant decreases in the body mass and the liver glycogen content of diabetic rats. It can be concluded that pravastatin, especially at the dose of 20 mg kg-1 day-1 is more effective in ameliorating the negative effects of diabetes by modulating carbohydrate metabolism.

Prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency among the fulminant hepatitis A virus infection patients.

Hepatitis A is a widespread viral infection with significant public health implications. Assessing glucose 6-phosphate dehydrogenase (G6PD) deficiency in hepatitis A patients is essential for various reasons, including prognosis, disease severity evaluation, encephalopathy risk identification, tailored management, and advancing scientific understanding. This study aimed to investigate the prevalence and clinical implications of G6PD impairment in individuals with fulminant hepatitis A. A cross-sectional descriptive analysis was conducted, involving hospitalized patients with fulminant hepatitis A. Demographic data, prevalence rates, and clinical findings were recorded in a database. The diagnosis of hepatitis A infection was confirmed using an anti-HAV IgM antibody test, and G6PD enzyme activity was measured with a fluorescent spot assay. Out of 81 patients with hepatitis A, 57 (70.4%) were males, and 24 (29.5%) were females, with an average age of 24.6 years. Dark yellow urine and anorexia were the most common clinical symptoms. Notably, 30 (37%) patients lacked G6PD. The group with G6PD deficiency showed significantly higher rates of encephalopathy and mortality (P<0.01), along with elevated bilirubin (P=0.00), abnormal coagulation parameters, and low hemoglobin levels (P=0.00). In light of these findings, the present study proposes the implementation of routine G6PD level assessments and the evaluation of other relevant markers in regions where hepatitis A is endemic. Furthermore, the study underscores the need for vigilant monitoring of hemolysis and encephalopathy in affected patients to optimize clinical management and reduce morbidity and mortality associated with this condition.

Mardin İli ve Çevresinde Serum Glukoz-6-Fosfat Dehidrogenaz Enzim Düzeyi Eksikliğinin Araştırılması

Introduction: Glucose-6-phosphate dehydrogenase (G6PD) is a cytosolic enzyme, and the first enzyme to catalyze the pentose phosphate pathway. Our aim is to investigate the G6PD enzyme activity in Mardin province.&#x0D; Methods: Our study was divided into subgroups according to gender, age, and G6PD levels. A total of 439 individuals, 168 females and 271 males, between the ages of 2 and 80 were included in the study. G6PD enzyme activity was measured by the photometric method using the AU5800 Beckman device.&#x0D; Results: G6PD enzyme activity in the Mardin region was found to be 13.95±7.29 U/gHb. The mean G6PD enzyme activity in female patients was 14.22±6.98 U/gHb, and the mean in male patients was 13.78±7.48 U/gHb (p&gt;0.05). When evaluated in terms of age, the G6PD enzyme activity was 14.93 (0.03-38.47) in patients aged 2-19, 13.76 (0.01-30.79) between 20-35 years old, 17.05 between 36-50 years old (0.13-27.21), 13.75 (0.05-26.99) U/gHb between 51-80 years old (p=0.301).&#x0D; Conclusion: In our study, no significant difference was found between G6PD enzyme activity in terms of age and gender. G6PD enzyme deficiency was shown as 6% relative to the reference range. A deficiency of 6.5% in male and 3.5% in female was observed. This study is valuable in that it is the first study conducted in Mardin. There is a need for larger studies to support this study locally.

Hepatitis-Induced Hemolysis in Pediatrics: A Rare Case Report

This article presents a case study of a 6-year-old boy diagnosed with acute Hepatitis A virus (HAV) infection compounded by glucose-6-phosphate dehydrogenase (G6PD) deficiency. The patient exhibited a constellation of symptoms, including low-grade fever, upper abdominal pain, fatigue, and anorexia, over an 8 to 10-day period. Notably, jaundice, dark-colored urine, and hepatomegaly were prominent clinical features. Laboratory investigations revealed severe intravascular hemolysis, evidenced by a decline in hemoglobin, elevated reticulocytes, and unconjugated hyperbilirubinemia. The diagnosis of HAV hepatitis was confirmed through positive IgM anti-hepatitis A virus. Over the subsequent two weeks, the patient experienced peak levels of serum bilirubin, AST, and ALT. Conservative management focused on avoiding hepatotoxic drugs, ensuring adequate urine output, and monitoring metabolic parameters. Gradual improvement was observed over the subsequent month, with a significant reduction in bilirubin levels, restoration of hemoglobin, and a decline in liver enzymes. The patient was discharged for outpatient follow-up, and four weeks post-onset, G6PD levels approached normal, emphasizing the importance of comprehensive care in such complex cases. This case underscores the intricate interplay between viral hepatitis and G6PD deficiency, contributing valuable insights into the clinical presentation and management of severe hemolysis in this specific population

Serum levels of oxidative stress biomarkers is changed in pharmacoresistant mesial temporal lobe epilepsy patients with or without psychiatric disorders

The objective of the present study was to assess the levels of oxidative stress biomarkers (OSB) in patients with pharmacoresistant temporal lobe epilepsy and mesial temporal sclerosis (TLE-MTS) in order to investigate a possible role of oxidative stress (OS) in the pathophysiology of such disease. Ninety-eight participants were included and distributed in three groups: group 1 (G1) - 25 patients with pharmacoresistant TLE-MTS, without comorbid PD; Group 2 (G2) - 21 patients with pharmacoresistant TLE-MTS with PD; Group 3 (G3) - 52 healthy control subjects. Serum levels of catalase (CAT), glutathione peroxidase (GPx), glutathione reductase (GR), reduced glutathione plus oxidated glutathione (total GSH), glucose-6-phosphate dehydrogenase (G6PD), Trolox-equivalent antioxidant capacity (TEAC), and thiobarbituric acid reactive substances (TBARS) were analyzed through spectrometry. The significance level was set at P<0.05. Sociodemographic data were similar in all groups (P>0.05). A family history of epilepsy was more frequent among G1 and G2 groups when compared to G3 (P = 0.0002). Compared to controls, G1 and G2 presented higher CAT (P<0.0001) and TBARS levels (P = 0.001), as well as lower GPx (P<0.0001), G6PD (P = 0.005) and total GSH levels (P = 0.04). The presence of psychiatric disorders (PD), analyzed both separately and together, was not associated with significant differences in OSB levels (P>0.05). The lower levels of GPx, G6PD, and total GSH, as well as the higher CAT and TBARS levels observed in pharmaco-resistant TLE-MTS patients can indicate an imbalance between oxidizing agent production and elimination, supporting the hypothesis of a possible role of OS in the pathogenesis of this condition.

CANLI DONÖR KARACİĞER NAKLİNİN ÖNGÖRÜSEL BİYOBELİRTEÇLERİ

Objective: Liver transplantation using a living donor (LDLT) is currently the most popular method used in the worldwide. Appropriate biomarkers that predict graft status should be used to detect early post-transplant complications that may lead to a rejection reaction. Material and Method: The study involved a total of 44 liver recipients and 44 liver donors, from whom preoperative blood samples were taken and immunoassay and spectrophotometric studies were carried out. The levels of serum neopterin, interferon-gamma (IFN- y), indoleamine-2,3 dioxygenase (IDO), and -glutathione S transferase (a-GST) were assessed using an enzyme-linked immunosorbent test. The activity of glutathione reductase (GR) and glucose-6-phosphate dehydrogenase (G6PD) in erythrocytes was measured using spectrophotometry. Result and Discussion: “Neopterin, IDO, and G6PD levels were significantly higher in the recipient group than in the donor group. The differences in gender and blood groups were statistically insignificant. The rejection reaction developed in 25% of patients and none survived. These findings may facilitate the identification of novel predictive biomarkers for the diagnosis of acute rejection reactions after LDLT. The clinical use of novel non-invasive biomarkers may provide time and cost advantages.

Pan-cancer analysis reveals that G6PD is a prognostic biomarker and therapeutic target for a variety of cancers.

Despite accumulating evidence revealing that Glucose-6-phosphate dehydrogenase (G6PD) is highly expressed in many tumor tissues and plays a remarkable role in cancer tumorigenesis and progression, there is still a lack of G6PD pan-cancer analysis. This study was designed to analyze the expression status and prognostic significance of G6PD in pan-cancer. G6PD expression data were obtained from multiple data resources including the Genotype-Tissue Expression, the Cancer Genome Atlas, and the Tumor Immunity Estimation Resource. These data were used to assess the G6PD expression, prognostic value, and clinical characteristics. The ESTIMATE algorithms were used to analyze the association between G6PD expression and immune-infiltrating cells and the tumor microenvironment. The functional enrichment analysis was also performed across pan-cancer. In addition, the GDSC1 database containing 403 drugs was utilized to explore the relationship between drug sensitivity and G6PD expression levels. Furthermore, we also performed clinical validation and in vitro experiments to further validate the role of G6PD in hepatocellular carcinoma (HCC) cells and its correlation with prognosis. The R software was used for statistical analysis and data visualization. G6PD expression was upregulated in most cancers compared to their normal counterparts. The study also revealed that G6PD expression was a prognostic indicator and high levels of G6PD expression were correlated with worse clinical prognosis including overall survival, disease-specific survival, and progression-free interval in multiple cancers. Furthermore, the G6PD level was also related to cancer immunity infiltration in most of the cancers, especially in KIRC, LGG, and LIHC. In addition to this, G6PD expression was positively related to pathological stages of KIRP, BRCA, KIRC, and LIHC. Functional analysis and protein-protein interactions network results revealed that G6PD was involved in metabolism-related activities, immune responses, proliferation, and apoptosis. Drug sensitivity analysis showed that IC50 values of most identified anti-cancer drugs were positively correlated with the G6PD expression. Notably, in vitro functional validation showed that G6PD knockdown attenuated the phenotypes of proliferation in HCC. G6PD may serve as a potential prognostic biomarker for cancers and may be a potential therapeutic target gene for tumor therapy.

Performance Comparison Between Conventional Fluorescent Spot Test and Quantitative Assay in Detecting G6PD Deficiency in Neonates.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzymopathy worldwide. The fluorescent spot test (FST) is the conventional method for screening neonates for G6PD. However, it has limitations and quantitative assays such as the CareStart Biosensor 1 are being increasingly recommended. This study aimed to compare FST and CareStart Bioensor 1 in their ability to detect G6PD levels in neonates. This cross-sectional study involved 455 neonates between June and December 2020. Two milliliters of cord blood were analyzed with CareStart Biosensor 1 and dried cord blood spots with FST. Data was recorded and statistically analyzed. Sensitivity, specificity, positive predictive value, and negative predictive value were calculated to determine the performance of FST at specific G6PD cut-off values; Cohen's kappa analysis assessed the agreement between the two methods. The sensitivity of FST at 30% cut-off G6PD activity level was 91.0%, (95% CI: 57.0-100) and specificity of 97.0% (95% CI: 95.0-98.0). At 60% cut-off, the FST sensitivity sharply declined to 29.0% (95% CI: 19.0-40.0) with a specificity of 100% (95% CI: 98.0-100). The overall prevalence of G6PD deficiency was 5.1% as measured by FST and 17.8% by Biosensor 1 (p < 0.001). In this study, FST missed a significant proportion of cases of intermediate G6PD levels. FST also misclassified several G6PD intermediate individuals as normal, rendering them susceptible to oxidative stress. Biosensor 1 reported a significantly higher prevalence of G6PD deficiency.

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency in Neonates Presenting with Indirect Hyperbilirubinemia

Objective: To determine the frequency of glucose-6-phosphate dehydrogenase (G6PD) deficiency in neonates presenting with indirect hyperbilirubinemia.Methods: A prospective cross-sectional study was conducted at Neonatal intensive care unit (NICU) of National Institute of Child Health, Karachi from August 2020 to July 2021. All neonates with either gender born with indirect hyperbilirubinemia &gt;5mg/dl admitted in NICU were consecutively enrolled. G6PD deficiency along with baseline and laboratory characteristics were observed. The presence of G6PD level of &lt;1.3 U/g Hb was labeled as G6PD deficiency.Results: Of 228 neonates, the mean age was 8.58 ±3.68 days. There were 153 (67.1%) males and 75 (32.9%) females. The frequency of G6PD deficiency was found to be 72 (31.6%). The mean weight was significantly higher among neonates with G6PD deficiency as compared to the patients with no G6PD deficiency, i.e., 2.53 ±0.48 vs. 2.37 ±0.56 (p-value 0.035). A significant difference of hemoglobin level (p-value &lt;0.001), serum total bilirubin level (p-value &lt;0.001), and serum indirect bilirubin level (p-value0.001) was observed in between G6PD deficient and non-deficient neonates. Increased reticulocyte count was also found significantly higher in neonates with G6PD deficiency as compared to the neonates without G6PD deficiency, i.e., 69 (95.8%) and 34 (21.8%) respectively (p-value &lt;0.001). Conclusion: G6PD deficiency was considerably prevalent in neonates with indirect hyperbilirubinemia. Moreover, hemoglobin level, serum total bilirubin level, serum indirect bilirubin level, and increased reticulocyte count was also observed significant contributing factors for G6PD deficiency.

A 2-year-old boy with Glucose-6-Phosphate Dehydrogenase Deficiency Following Naphthalene Ingestion: a Case Report

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary enzyme disorder in the world and more than 400 million people have a deficiency in this enzyme. G6PD deficiency is an X-linked disorder, and one of its important signs is the presence of hemolytic anemia. It is a worldwide important cause of neonatal jaundice and causes life threatening hemolytic crisis in childhood. Here a 2- year- old boy was admitted into department of pediatrics of Sylhet Women’s Medical College Hospital with fever for 2 days &amp; passage of dark color urine for 1 day. There was history of accidental naphthalene ingestion present prior to develop symptoms. The boy was clinically febrile, severely pale, anicteric. No organomegaly was present. It was confirmed by G6PD level assay. Symptomatic &amp; supportive treatment was given with packed cell volume transfusion and avoidance of triggering factors was advised. Genetic counseling was also done.

The effect of phototherapy on neonatal calcium and bilirubin levels: a comparison between patients with low and normal G6PD levels

Introduction: Iran is among countries that report a higher prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency, ranging from 2.28% to 30.80%. This study aimed to estimate the general prevalence of G6PD deficiency in newborns with jaundice who were hospitalised in southeast Iran. Methods: This was a prospective study. A total of 100 newborns (boys and girls) with hyperbilirubinaemia, presented to Amir Al-Mo’menin Hospital in 2006 and 2007, were selected for the study. Age, gender, weight, time of jaundice, the maximum level of direct bilirubin, and the total level of bilirubin among the newborns were recorded, and the data were analysed by SPSS. Results: A total of 100 jaundice neonates, including 46 girls and 54 boys, were enrolled. Twenty-two percent of these patients, including 18 (82%) boys and 4 (18%) girls, showed low levels of G6PD. The overall mean bilirubin level was 15.45 ± 3.43 mg/dL among neonates with low G6PD levels, while it was 19.21 ± 3.34 mg/dL in patients with normal G6PD levels, which showed a significant difference (p &lt; 0.05). Conclusion: The prevalence of G6PD deficiency in neonates with jaundice in our study was 22%, which was higher compared to other regions. We propose G6PD deficiency screening for the prevention of its complications. We also suggest assessing calcium levels following phototherapy, given the higher prevalence of hypocalcaemia.

DP17 A very rare presentation of linear IgA disease during pregnancy

Abstract We present a very rare immunobullous dermatosis, linear IgA disease (LAD), that developed during pregnancy, to highlight its clinical features, management and important safety considerations for the neonate. During her first pregnancy, a 32-year-old Japanese woman of 27 weeks’ gestation developed an intensely itchy blistering rash on her limbs, trunk and face without periumbilical predilection. The blisters were tense, clustering in an annular configuration, with subsequent development of blistering and erosions involving the oral and genital mucosae. Medications included a pregnancy multivitamin only. A lesional skin biopsy revealed a subepidermal blister containing neutrophils and eosinophils. Direct immunofluorescence of perilesional skin demonstrated linear deposition of IgA along the basement membrane zone. Linear IgA disease was diagnosed. Initial treatment consisted of erythromycin and daily clobetasol ointment. Owing to inadequate symptom control and blister progression, dapsone 50 mg daily was initiated with prompt improvement in her condition and cessation of new blistering. A postpartum flare necessitated a dose increase to 75 mg daily. Her baby’s glucose-6-phosphate dehydrogenase (G6PD) level was found to be normal at birth. She proceeded to breastfeed her son, and monitoring of his haemoglobin level was normal. Although her clinical presentation initially raised the possibility of pemphigoid gestationis (PG), there was no periumbilical involvement, and the morphology of blistering in conjunction with mucosal involvement was more suggestive of LAD. The immunofluorescence results also helped to distinguish LAD from PG. The new onset of LAD during pregnancy is very unusual and has been rarely reported (Ikkaku N, Tateishi N, Oda Y et al. Linear immunoglobulin A bullous dermatosis developing during late pregnancy. J Dermatol 2017; 44:e44–5). Pre-existing LAD typically improves during pregnancy, although postpartum flares are recognized. Pemphigoid gestationis has been associated with an increased risk of prematurity and intrauterine growth restriction, but this has not been reported with LAD during pregnancy, possibly due to its rarity. Both the diagnosis of LAD and its mainstay treatment—dapsone—have important potential implications for the neonate. As for other immunobullous diseases, transplacental transfer of pathogenic antibodies may result in transient, usually mild, neonatal blistering. Although dapsone can be safely used during pregnancy and breastfeeding, the risk of neonatal haemolysis and methemoglobinaemia necessitates the assessment of the G6PD level and haemoglobin monitoring in the neonate. Multidisciplinary input from the dermatology, obstetric and paediatric teams is crucial to ensure appropriate counselling and care of the affected mother and baby.

Phytochemical screening and evaluation of glucose-6-phosphate dehydrogenase activity of fermented honey-garlic in hypothyroidism rats

Introduction: Hypercholesterolemia is an indicator of atherosclerosis in blood vessels and is currently a top priority in overcoming health problems, especially non-communicable diseases in developed and developing countries. Thyroid hormones regulate various metabolic processes in the body including synthesis, mobilization, and lipid degradation. A lack of thyroid hormone in the body or called hypothyroidism will decrease the number of LDL receptors in the liver. This causes an increase in plasma LDL levels which also increases total cholesterol levels. Despite the lack of study, Fermented Honey-Garlic (FHG) is now widely developed for its high antioxidant activity. This study aims to determine the content of bioactive compounds in FHG by qualitative secondary metabolite testing and GC-MS analysis and to evaluate the activity of the enzyme glucose-6-phosphate dehydrogenase (G6PD) in rats with hypothyroidism. Method: Rats were grouped into 6 groups: positive control group induced by propylthiouracil (PTU) with a dose of 20 mg/day, negative control treated with distilled water, treatment group with a dose of FHG 0.2 g/kg BW (P1), treatment group with a dose of FHG 0.5 g/kg BW (P2), treatment group with a dose of FHG 1 g/kg BW (P3) and standard drug Simvastatin group with the dose 0,18 mg/day/200 g BW. Results: Results showed that FHG contained flavonoid compounds, alkaloids, tannins, and saponins, and statistical results showed that the significance value for G6PD activity was 0.014 (P&lt;0.05). Conclusion: Therefore, it can be concluded that FHG administration yields a significant effect on G6PD levels.

Butyrate ameliorates lithium-induced cardiometabolic disorders in male Wistar rats

BackgroundCardiometabolic disease is becoming a main cause of death worldwide with over ten million fatalities annually. Lithium (Li) has been reported to be one of the major causes of cardiometabolic disorders due to its narrow therapeutic index. Sodium butyrate (NaB) which is a short chain fatty acid is implicated to possess an amazing therapeutic effect against cardiometabolic disorders. As a result, the current study aims to evaluate sodium butyrate impact on lithium-induced cardiometabolic disorders. MethodsTwenty male Wistar rats weighing between 230 and 250 g were used for the experiment. They were grouped into control and treated groups respectively as (CTR; normal chow), Lithium only (Li; 5 mg/kg, po), Sodium butyrate only (NaB) (200 mg/kg, po) and NaB +Lithium (Li; 5 mg/kg +NaB; 200 mg/kg, po) for six weeks, after a week of acclimation; n=5. The animals were anesthetized using sodium pentobarbital at a dose of 50 mg/kg intraperitoneally, blood samples were collected by cardiac puncture and cardiac tissue homogenates were measured for plasma Fasting Blood Sugar (FBS), plasma Insulin, Phosphatidylinositol 3 Kinase (PI3K), Nitric oxide (NO), Glucose-6-phosphate dehydrogenase (G6PDH), Malonyladehyde (MDA), Catalase (CAT), Superoxide Dismutase (SOD), Nicotinamide adenine dinucleotide phosphate (NADPH), Glutathione Peroxidase (GPx), and Adenosine deaminase /Xanthine oxidase /uric acid (ADA/XO/UA) using respective ELISA kits. ResultsThe results showed that lithium significantly increased fasting blood sugar (FBS), plasma insulin, GPx, CAT, SOD, and MDA while causing a statistical reduction in plasma and cardiac concentration of NADPH, G6PDH, PI3K, endothelial nitric oxide synthase (eNOS), and Nitric Oxide (NO) in comparison with the control. However, butyrate attenuated plasma GPx, CAT, SOD, MDA, FBS, plasma insulin and significantly elevated plasma and cardiac PI3K, eNOS, NO, plasma NADPH and G6PDH levels in relation to control. Also no change was observed in the plasma and cardiac ADA/XO/UA following sodium butyrate. ConclusionTherefore, butyrate has a remarkable therapeutic potential in ameliorating lithium induced cardiometabolic disorders through glucometabolic control.