scholarly journals Glusoce-6-phosphate dehydrogenase- History and diagnosis

2016 ◽  
Vol 6 (12) ◽  
pp. 1034-1039 ◽  
Author(s):  
K Gautam
Keyword(s):  
Oxidative Stress ◽  
Blood Cells ◽  
Dehydrogenase Deficiency ◽  
Laboratory Diagnosis ◽  
Diagnostic Methods ◽  
Noncommunicable Diseases ◽  
Vast Number ◽  
History Of ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Enzymatic Defect

Glucose-6-phosphate dehydrogenase deficiency is the most common enzymatic defect of red blood cells, which increases the vulnerability of erythrocytes to oxidative stress leading to hemolytic anemia. Since its identification more than 60 years ago, much has been done with respect to its clinical diagnosis, laboratory diagnosis and treatment. Association of G6PD is not just limited to anti malarial drugs, but a vast number of other diseases. In this article, we aimed to review the history of Glucose-6-phosphate dehydrogenase, the diagnostic methods available along with its association with other noncommunicable diseases. 

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 Sulfhemoglobinemia and Acute Hemolytic Anemia with Heinz Bodies Following Contact with a Fungicide— Zinc Ethylene Bisdithiocarbamate—in a Subject with Glucose-6-Phosphate Dehydrogenase Deficiency and Hypocatalasemia

Blood ◽  
1963 ◽  
Vol 21 (4) ◽  
pp. 484-494 ◽  
Author(s):  
JACK PINKHAS ◽  
MEIR DJALDETTI ◽  
HENRY JOSHUA ◽  
CHAIM RESNICK ◽  
ANDRÉ DE VRIES
Keyword(s):  
Red Blood Cells ◽  
Hemolytic Anemia ◽  
Blood Cells ◽  
Dehydrogenase Deficiency ◽  
Reduced Glutathione ◽  
Body Formation ◽  
Low Glucose ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Enzymatic Defect

Abstract Sulfhemoglobinemia associated with Heinz body formation and acute hemolytic anemia following contact with a fungicide, zinc ethylene bisdithiocarbamate, is described in a Persian Jew whose red blood cells had low glucose-6-phosphate dehydrogenase activity with low and unstable reduced glutathione and low catalase activity. The fungicide, similarly to acetylphenylhydrazine, was capable of decreasing in vitro the reduced glutathione of the patient’s red blood cells, as well as of those of other subjects with the same enzymatic defect. The sulfhemoglobinemia and the hemolytic anemia are considered to have been produced independently by the fungicide, the glucose-6-phosphate dehydrogenase deficiency having played a role only in the latter. The possibility that the hypocatalasemia was a factor in rendering the patient’s red blood cells sensitive to the hemolysis- and sulfhemoglobin-producing action of the fungicide is discussed. The importance of zinc ethylene bisdithiocarbamate as a sulfhemoglobin-producing and hemolytic agent is stressed, in view of the widespread use of this fungicide.

Glucose-6-phosphate dehydrogenase deficiency and cardiac surgery

Perfusion ◽  
2010 ◽  
Vol 25 (6) ◽  
pp. 417-421 ◽  
Author(s):  
N. Dogra ◽  
GD Puri ◽  
SS Rana
Keyword(s):  
Cardiac Surgery ◽  
Blood Cells ◽  
Perioperative Management ◽  
Dehydrogenase Deficiency ◽  
Ischemia Reperfusion ◽  
Anaesthetic Management ◽  
Whole Body ◽  
Free Radical Production ◽  
Radical Production ◽  
Glucose 6 Phosphate Dehydrogenase

Cardiac surgery involving cardiopulmonary bypass (CPB) in its conventional form involves many processes leading to free radical production, such as perioperative ischemia, reperfusion, circulation of whole body blood through the CPB circuit, hypothermia and acidosis. The red blood cells of a glucose-6-phosphate dehydrogenase (G6PD)-deficient person are unable to scavenge these free radicals, resulting in haemolysis. Here, we describe the successful anaesthetic management of two G6PD-deficient children who underwent cardiac surgery, on and off CPB, without any obvious haemolytic reaction, followed by a discussion of the disorder, with specific consideration of perioperative management of such cases.

scholarly journals Paroxysmal Nocturnal Hemoglobinuria with Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report and Review of the Literature

2019 ◽  
Vol 12 (3) ◽  
pp. 838-844
Author(s):  
Mahmoud S. Eisa ◽  
Shehab F. Mohamed ◽  
Firyal Ibrahim ◽  
Khalid Shariff ◽  
Nagham Sadik ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
G6pd Deficiency ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Dehydrogenase Deficiency ◽  
Response To Treatment ◽  
X Chromosomes ◽  
Financial Status ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Real Challenge ◽  
And Oxidative Stress

In this study, we are describing a female patient with paroxysmal nocturnal hemoglobinuria (PNH) and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Both diseases are known to cause hemolytic anemia that mediates the hemolysis of RBCs through several mechanisms. In PNH the hemolysis is mediated through complement activation and oxidative stress. G6PD enzyme is crucial in preventing damage to cellular structures caused by oxygen-free radicles. In G6PD deficiency the hemolysis is mediated through the oxidative stress created by oxygen-free radicles. Since both diseases mediate hemolysis through the oxidative stress, we hypothesize that both conditions have facilitated an effect on each other and this will reflect on the response to treatment, and this response to treatment could vary based on whether the two mutations occurred in the same gene or in two different X chromosomes. Having diagnosed PNH, the management is very expensive and not all the patients can afford it, especially our patient who is a maid by occupation. So, the real challenge in our case is to monitor her in subsequent visits and to plan the treatment keeping in mind her financial status.

scholarly journals Anaesthesia for a child with glucose-6-phosphate-dehydrogenase deficiency: a case report

2020 ◽  
Vol 6 (12) ◽  
pp. 526
Author(s):  
Baththirange Malaka Munasinghe ◽  
Srisothinathan Nimalan ◽  
Nishanthan Subramaniam ◽  
Sonali Goonetilleke
Keyword(s):  
Oxidative Stress ◽  
Case Report ◽  
General Anaesthesia ◽  
G6pd Deficiency ◽  
Dehydrogenase Deficiency ◽  
Perioperative Period ◽  
Postoperative Monitoring ◽  
Successful Case ◽  
Operative Planning ◽  
Glucose 6 Phosphate Dehydrogenase

<p class="abstract">Patients with glucose-6-phosphate-dehydrogenase (G6PD) deficiency are prone to acute haemolytic crisis during perioperative period, precipitated by oxidative stress and certain medications, which are common concurrences during this period. Careful peri-operative planning, judicious use of medication and postoperative monitoring plays a pivotal role in preventing complications in these patients. Here, we report a successful case of general anaesthesia in a child with G6PD deficiency.  </p>

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