scholarly journals Glucose-6-phosphate Dehydrogenase Deficiency: A Case Report

2017 ◽  
Vol 12 (1) ◽  
pp. 47-49
Author(s):  
Md Kamrul Hassan ◽  
Aloke Kumar Saha ◽  
Lakshman Chandra Kundu ◽  
Poly Begum ◽  
Abu Yousuf
Keyword(s):  
G6pd Deficiency ◽  
Neonatal Jaundice ◽  
Dehydrogenase Deficiency ◽  
Cultural Factors ◽  
Enzyme Defect ◽  
Asian Populations ◽  
Hemolytic Crisis ◽  
Life Threatening ◽  
Fava Beans ◽  
Glucose 6 Phosphate Dehydrogenase

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary enzyme disorder and more than 200 million people have a deficiency in this enzyme. G6PD deficiency is an X-linked enzyme defect, and one of its main signs is the presence of hemolytic anemia. It is a worldwide important cause of neonatal jaundice and causes life threatening hemolytic crisis in childhood. At later ages, certain drugs such as anti-malarial drugs and fava beans cause hemolysis among G6PD deficiency patients. The frequency and severity is influenced by genetic and cultural factors. It is common in Mediterranean, African and some East Asian populations but rare in Bangladeshi peoples. Genetic counseling may be of benefit for patients and their families. Other treatment is symptomatic and supportive.Faridpur Med. Coll. J. Jan 2017;12(1): 47-49

scholarly journals Fava bean–induced hemolytic crisis in glucose-6-phosphate dehydrogenase deficiency

2016 ◽  
Vol 43 (6) ◽  
pp. 230
Author(s):  
IPG Karyana ◽  
IB Mudita
Keyword(s):  
G6pd Deficiency ◽  
Dehydrogenase Deficiency ◽  
Fava Bean ◽  
Hemolytic Crisis ◽  
Fava Beans ◽  
Glucose 6 Phosphate Dehydrogenase

This paper reports a case of hemolytic crisis dueto fava beans in a child with G6PD deficiency.

Clinical Spectrum of Primaquine-induced Hemolysis in Glucose-6-Phosphate Dehydrogenase Deficiency: A 9-Year Hospitalization-based Study From the Brazilian Amazon

2019 ◽  
Vol 69 (8) ◽  
pp. 1440-1442 ◽  
Author(s):  
Jose Diego Brito-Sousa ◽  
Thalie C Santos ◽  
Sara Avalos ◽  
Gustavo Fontecha ◽  
Gisely C Melo ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Plasmodium Vivax ◽  
G6pd Deficiency ◽  
Dehydrogenase Deficiency ◽  
Brazilian Amazon ◽  
Clinical Spectrum ◽  
Life Threatening ◽  
Endemic Areas ◽  
Glucose 6 Phosphate Dehydrogenase

Abstract Despite glucose-6-phosphate dehydrogenase (G6PD) deficiency prevalence of 5% in the Amazon, primaquine is administered without G6PD screening. This is an important cause of hospitalization among Plasmodium vivax–infected individuals, leading to life-threatening anemia and acute renal failure across endemic areas. In Manaus, the frequency of primaquine-induced hemolysis was 85.2 cases per 100 000 primaquine users.

Clinical Manifestations and Therapeutic Findings of the Children with Glucose-6-Phosphate Dehydrogenase Deficiency Presenting Favism

2020 ◽  
Vol 20 ◽  
Author(s):  
Fariba Tarhani ◽  
Alireza Nezami ◽  
Ghobad Heidari ◽  
Babak Abdolkarimi
Keyword(s):  
G6pd Deficiency ◽  
Dehydrogenase Deficiency ◽  
Clinical Manifestations ◽  
Descriptive Study ◽  
Cross Sectional ◽  
Common Complaint ◽  
The Mean ◽  
Fava Beans ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Hydration Therapy

Aim: Favism is characterized as acute anemia, due to Glucose-6-phosphate dehydrogenase (G6PD) deficiency as a result of fava beans intake. It is associated with paleness, jaundice, and hemoglobinuria. In this study, signs, symptoms and therapeutic findings of the patients with hemolysis due to G6PD deficiency were investigated in Shahid Madani Hospital of Khorramabad, Lorestan. Methods: This is a single-center cross-sectional descriptive study that was conducted on all children with G6PD deficiencyinduced hemolysis. Results: 308 children (64.3% male and 35.7% female) were included in this study. The most common complaint was jaundice (82.5%) and the most common cause of hemolysis was the intake of fava bean (85.7%). 68% of the children were treated with hydration/fluid therapy. Blood transfusion was conducted in 36.36% of the cases and the mean of blood administered was 18.9 cc/kg. Conclusion: In this study, hydration therapy was performed in most of the children presenting favism. Also, the incorrect calculation of the amount of blood needed for transfusion has increased the frequency of blood transfusions and prolonged hospitalization time.

scholarly journals FREQUENCY OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY IN NEONATAL JAUNDICE IN DISTRICT SWAT, PAKISTAN

1969 ◽  
Vol 3 (1) ◽  
pp. 242-247
Author(s):  
AMREEK LAL ◽  
S.M.NAEEM ◽  
SHAHJEHAN ◽  
ASHFAQ AHMAD ◽  
ISRARUL HAQ
Keyword(s):  
G6pd Deficiency ◽  
Neonatal Jaundice ◽  
Dehydrogenase Deficiency ◽  
Reduction Method ◽  
Serum Bilirubin ◽  
Newborn Infants ◽  
Dye Reduction ◽  
Complete Blood Counts ◽  
Deficiency Screening ◽  
Glucose 6 Phosphate Dehydrogenase

Background: Hyperbilirubinemia is the most common cause of neonatal jaundice. Severe neonatalhyperbilirubinemia and kemicterus continue to be reported worldwide in otherwise healthy term infants.We conducted this study to estimate the incidence of severe neonatal hyperbilirubinemia and to determineunderlying causes, improved knowledge of which would be valuable to help identify strategies for riskreduction.Methods: 100 infants of both sexes, of the age between 1-28 days, with no infection and Rh incompatibilitywere included in the study. The activity of G6PD was determined by a dye-reduction method screening testdevised by Sigma Diagnostics USA. Complete blood counts (CBC) with reticulocyte count, serum bilirubin(total and indirect) were performed and the results were recorded for further analyses.Results: Out of the total 100 subjects, classified in two groups on the basis of presence of jaundice, 14 (14%)were G6PD deficient. The frequency of G6PD deficiency was markedly prominent, in neonates withjaundice.Discussion: G6PD deficiency is one of the major causes of jaundice in neonates. The study area also have aconsiderable number of G6PD deficient individuals. This indicates a need for a more thorough assessmentof newborn infants and consideration of strategies to identify at-risk newborns, such as pre-dischargemeasurement of serum bilirubin levels and G6PD deficiency screening.

scholarly journals Neonatal jaundice and glucose-6-phosphate dehydrogenase deficiency

2016 ◽  
Vol 11 (4) ◽  
Author(s):  
Sohail Rasheed ◽  
Abdul Hayee ◽  
Yasmeen Lodhi ◽  
Rafi Ahmed
Keyword(s):  
Bilirubin Level ◽  
G6pd Deficiency ◽  
Neonatal Jaundice ◽  
Dehydrogenase Deficiency ◽  
Total Bilirubin ◽  
Total Bilirubin Level ◽  
Normal Subjects ◽  
Group I ◽  
Level Of Significance ◽  
Glucose 6 Phosphate Dehydrogenase

One hundred new born males babies aged 7 days and above were included in this study. These were divided into two groups — Group I included G6PD normal subjects and Group II included G6PD deficient subjects. Total bilirubin and G6PD enzyme levels were done by commercially available kits. Results were analysed by using students `t` test and level of significance was done. A significant increase in total bilirubin level was observed in infants of G6PD deficiency, Erythrocyte G6PD level is significantly decreased in 06% of infants born with neonatal jaundice.

Due fratelli con i globuli rossi stressati

2021 ◽  
Vol 40 (3) ◽  
pp. 193-194
Author(s):  
Simone Ceratto ◽  
Eleonora Tognato ◽  
Lorenzo Fiorica ◽  
Giorgia Enrico ◽  
Lina Cimminelli ◽  
...  
Keyword(s):  
Neonatal Jaundice ◽  
Dehydrogenase Deficiency ◽  
Fava Beans ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
First Time

The paper describes the case of two Caucasian brothers (11 and 12 years old resepctively) who for the first time presented with acute haemolysis due to glucose-6-phosphate dehydrogenase deficiency. The two brothers showed different severity of symptoms, even though the elder one had already eaten fava beans before. Of note, both of them were affected by neonatal jaundice and needed phototherapy in their first days of life.

scholarly journals From Prejudice to Evidence: The Case of Rhizoma Coptidis in Singapore

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Chin Ee Ho ◽  
You Li Goh ◽  
Chang Zhang
Keyword(s):  
Chinese Medicine ◽  
Traditional Chinese Medicine ◽  
G6pd Deficiency ◽  
Neonatal Jaundice ◽  
Therapeutic Effects ◽  
Evidence Based ◽  
Rhizoma Coptidis ◽  
History Of ◽  
Hepatoprotective Effects ◽  
Glucose 6 Phosphate Dehydrogenase

Rhizoma Coptidis (RC), commonly known ashuanglian, is a herb frequently used in Traditional Chinese Medicine (TCM) prescriptions. Known to have “clearing damp-heat, quenching fire and counteracting poison” properties, it was widely used in the Chinese community in Singapore. Berberine, an alkaloid isolated from RC, is known to have a wide array of therapeutic effects including antimicrobial, antineoplastic, and hepatoprotective effects. In 1978, RC was implicated in causing neonatal jaundice (NNJ) and kernicterus in neonates suffering from glucose-6-phosphate dehydrogenase (G6PD) deficiency, leading to the banning of RC and berberine in Singapore. More than three decades later, accumulating evidence-based studies pointing to the safety of RC for general public and better understanding of G6PD deficiency, the Health Sciences Authority (HSA) in Singapore reviewed and lifted the prohibition on RC and berberine, turning a brand new chapter in the history of TCM in Singapore. This paper aims to review the safety of RC and berberine, using the prohibition of use and subsequent lifting of ban on RC and berberine in Singapore as an illustration to highlight the importance of evidence-based studies in Traditional Chinese Medicine (TCM).

scholarly journals A Rare Case of Coexistence of Homozygous β-Thalassaemia and Glucose 6 Phosphate Dehydrogenase Deficiency

2020 ◽  
Author(s):  
Jitendar Mohan Khunger ◽  
Monika Gupta ◽  
Ankur Jain ◽  
Monica Khunger Malhotra
Keyword(s):  
Oxidative Stress ◽  
Blood Cells ◽  
G6pd Deficiency ◽  
Rare Case ◽  
Dehydrogenase Deficiency ◽  
Globin Gene ◽  
Genetic Abnormality ◽  
Wide Range ◽  
Symptomatic Anaemia ◽  
Glucose 6 Phosphate Dehydrogenase

β-thalassaemia is one of the most prevalent autosomal disorders worldwide. Mutations/deletions in globin gene underlie deficiencies in Haemoglobin (Hb) production, which can interfere with oxygen delivery by Hb, resulting in thalassaemias causing anaemias with a wide range of disease severity. Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is a genetic abnormality resulting in inadequate amount of G6PD in the Red Blood Cells (RBCs). In patients with G6PD deficiency, the reduced or absent activity of the enzyme in RBCs causes premature haemolysis and symptomatic anaemia. The marked oxidative stress caused by homozygous β-thalassaemia is apparently incompatible with G6PD deficiency. Here, a rare case of six-month-old male child is described who presented with severe pallor hepato-splenomegaly and these two conditions co-existed in this patient.

scholarly journals Clinical spectrum and severity of hemolytic anemia in glucose 6-phosphate dehydrogenase–deficient children receiving dapsone

Blood ◽  
2012 ◽  
Vol 120 (20) ◽  
pp. 4123-4133 ◽  
Author(s):  
Allan Pamba ◽  
Naomi D. Richardson ◽  
Nick Carter ◽  
Stephan Duparc ◽  
Zul Premji ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Hemolytic Anemia ◽  
G6pd Deficiency ◽  
Case Reports ◽  
Drug Induced ◽  
Once Daily ◽  
Maximum Decrease ◽  
Life Threatening ◽  
The Mean ◽  
Glucose 6 Phosphate Dehydrogenase

AbstractDrug-induced acute hemolytic anemia led to the discovery of G6PD deficiency. However, most clinical data are from isolated case reports. In 2 clinical trials of antimalarial preparations containing dapsone (4,4′-diaminodiphenylsulfone; 2.5 mg/kg once daily for 3 days), 95 G6PD-deficient hemizygous boys, 24 G6PD-deficient homozygous girls, and 200 girls heterozygous for G6PD deficiency received this agent. In the first 2 groups, there was a maximum decrease in hemoglobin averaging −2.64 g/dL (range −6.70 to +0.30 g/dL), which was significantly greater than for the comparator group receiving artemether-lumefantrine (adjusted difference −1.46 g/dL; 95% confidence interval −1.76, −1.15). Hemoglobin concentrations were decreased by ≥ 40% versus pretreatment in 24/119 (20.2%) of the G6PD-deficient children; 13/119 (10.9%) required blood transfusion. In the heterozygous girls, the mean maximum decrease in hemoglobin was −1.83 g/dL (range +0.90 to −5.20 g/dL); 1 in 200 (0.5%) required blood transfusion. All children eventually recovered. All the G6PD-deficient children had the G6PD A− variant, ie, mutations V68M and N126D. Drug-induced acute hemolytic anemia in G6PD A− subjects can be life-threatening, depending on the nature and dosage of the drug trigger. Therefore, contrary to current perception, in clinical terms the A− type of G6PD deficiency cannot be regarded as mild. This study is registered at http://www.clinicaltrials.gov as NCT00344006 and NCT00371735.

scholarly journals Case report: Acute Hepatitis E Infection with Co-Existent Glucose-6-Phosphate Dehydrogenase Deficiency

2003 ◽  
Vol 14 (4) ◽  
pp. 230-231 ◽  
Author(s):  
Amitabh Monga ◽  
Ravinder PS Makkar ◽  
Anju Arora ◽  
Surabhi Mukhopadhyay ◽  
Ajay K Gupta
Keyword(s):  
Viral Hepatitis ◽  
G6pd Deficiency ◽  
Hepatitis E Virus ◽  
Dehydrogenase Deficiency ◽  
Acute Viral Hepatitis ◽  
Hepatitis E ◽  
Severe Anemia ◽  
Northern India ◽  
Acute Hepatitis E ◽  
Glucose 6 Phosphate Dehydrogenase

Hepatitis E virus is one of the leading causes of acute viral hepatitis in India but usually manifests as a mild self-limiting illness. Viral hepatitis in the presence of glucose-6-phosphate dehydrogenase (G6PD) deficiency may be associated with complications such as severe anemia, hemolysis, renal failure, hepatic encephalopathy and even death. The incidence of G6PD deficiency in the general population of northern India is reported to be between 2.2% and 14%. Despite both hepatitis E infection and G6PD deficiency being common, their impact on patient illness has only recently been reported. The present study reports a case of severe hemolysis in a patient with G6PD deficiency and hepatitis E infection.

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