|Year : 2016 | Volume
| Issue : 1 | Page : 74-77
Treatment of pediatric burn patient having glucose-6-phosphate dehydrogenase deficiency
Vijay Y Bhatia, Sankit D Shah, Harshil Y Ravalji, Deepa Banker
Department of Burns, Plastic and Reconstructive Surgery, V.S. General Hospital and N.H.L. Medical College, Ahmedabad, Gujarat, India
|Date of Web Publication||12-Dec-2016|
Vijay Y Bhatia
V.S. General Hospital and N.H.L. Medical College, Ahmedabad, Gujarat
Source of Support: None, Conflict of Interest: None
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common red cell enzymopathy found in humans. It clearly has an X-linked recessive inheritance with its prevalence varying from 0% to 27% in a different caste, ethnic, and linguistic groups. This deficiency may result in hemolytic anemia during stress, infection, and use of certain drugs. The use of topical silver sulfadiazine can produce hemolysis in patients having G6PD deficiency. Here, we describe one case successfully treated of pediatric burn of 25% of body surface area who was a known case of G6PD deficiency.
Keywords: Burn, glucose-6-phosphate dehydrogenase deficiency, hemolysis
|How to cite this article:|
Bhatia VY, Shah SD, Ravalji HY, Banker D. Treatment of pediatric burn patient having glucose-6-phosphate dehydrogenase deficiency. Indian J Burns 2016;24:74-7
|How to cite this URL:|
Bhatia VY, Shah SD, Ravalji HY, Banker D. Treatment of pediatric burn patient having glucose-6-phosphate dehydrogenase deficiency. Indian J Burns [serial online] 2016 [cited 2019 Sep 18];24:74-7. Available from: http://www.ijburns.com/text.asp?2016/24/1/74/195538
| Introduction|| |
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common of the red cell enzymopathy in humans and has an X-linked recessive disorder.,, It has been reported from India more than 30 years ago, and the prevalence varies from 0% to 27% in a different caste, ethnic and linguistic groups. The activity of the G6PD enzyme is reduced in red cells in these patients. This reduction in activity may result in hemolytic anemia during stress, infection, and particularly after exposure to oxidizing drugs or contact with any such chemicals., Many drugs and chemicals are described as being capable of inducing hemolysis in this population. Among these are acetanilide, methylene blue, nalidixic acid, naphthalene, niridazole, pyridium, phenylhydrazine, toluidine blue, trinitrotoluene, nitrofurans (Furadantin, Furoxone), antimalarial drugs (primaquine, quinine), the group of sulfa (sulfacetamide, sulfamethoxazole, sulfanilamide, sulfapyridine, and thiazosulfones), and urate oxidase.
The severity of G6PD deficiency depends on the magnitude of the enzyme deficiency and the severity of hemolysis.
- Class I variants, which are rare, have severe enzyme deficiency (<10% of normal) and have chronic hemolytic anemia
- Class II variants also have severe enzyme deficiency, but there is usually only intermittent hemolysis associated with infection, drugs, or chemicals
- Class III variants have moderate enzyme deficiency (10–60% of normal) with intermittent hemolysis usually associated with infection, drugs, or chemicals
- Classes IV and V are of no clinical significance.
Many cases reported as acute hemolysis in G6PD deficiency are due to drug interaction, few cases reported of acute hemolysis in burns patient due to stress or use of topical antimicrobial like silver sulfadiazine or mefenamic acid., We describe one case successfully treated of pediatric burn that was a known case of G6PD deficiency.
| Case Report|| |
A 4-year-male child sustained accidental flame burn due to fall into burning campfire while playing. His parents brought him to our hospital. After examining the child, he was diagnosed with 25% superficial with patchy second degree deep burns involving the face, chest, abdomen, and right upper limb with respiratory involvement [Figure 1]. He was admitted in burns ward under plastic surgery department for further management. His parents informed that child is suffering from a disease called G6PD deficiency. The mother had delivered twins by normal delivery. At birth both of them were healthy. They developed Jaundice on the 5th day and were serious. At that time, doctor performed various blood tests and diagnosed G6PD deficiency in them. The patient was treated in the form of initial resuscitation and dressing with paraffin gauze. The patient was also referred to a pediatrician, for G6PD deficiency.
We avoided locally acting anti-microbial agents causing hemolysis in this patient. We treated him as regular dressing with paraffin gauze on alternate day in operation theater under all aseptic precautions. Anesthesia was not required during dressings. Essential drugs and nutrition were provided which were safe for him. Injectable ceftazidime and metronidazole were given for 7 days as antibiotic coverage. For next 7 days, he was given oral cefadroxil and metrogyl as syrup. Then later, the antibiotics were withdrawn. For analgesia, paracetamol was used. The patient was monitored clinically. Complete blood count, reticulocyte counts, and urine analysis were also done on regular basis to rule out hemolysis.
Patient's hemoglobin dropped to 7.5 g%, at that time we performed reticulocyte count which was 2% (normal range 0.2%–2.0%). The patient was given one unit of blood transfusion (10 ml/kg). After 26 days of treatment, he was discharged as the wounds were completely healed without any complication [Figure 2] and [Figure 3]. Although there was a limitation of use of certain drugs, the patient was treated successfully.
| Discussion|| |
The distribution of G6PD deficiency among ethnic groups varies widely, from only 1 in 1000 among the northern European population to about 50% among the Kurdish Jews in Israel. The prevalence of G6PD Deficiency in India varies between 0% and 27% in different geographical regions.
The likelihood of developing hemolysis and the severity of disease are determined by the biochemical characteristics of the G6PD variant. In normal subjects, the activity of G6PD falls exponentially as red cells age, as the normal enzyme (G6PD B) has an in vivo half-life of 62 days. However, normal old red blood cells still contain sufficient G6PD activity to maintain glutathione (GSH) levels in the face of oxidant stress. In contrast, the half-life is much shorter in the G6PD variants associated with hemolysis. As an example, the enzymatic activity of G6PD-A is normal in reticulocytes, but declines rapidly thereafter with a half-life of 13 days., G6PD Mediterranean is even more unstable, with a half-life measured in hours.
G6PD-deficient erythrocytes that are exposed to oxidants (e.g., drugs, infection) become depleted of GSH; this change is followed by oxidation of other sulfhydryl-containing proteins with the following consequences:
- Oxidation of the sulfhydryl groups on hemoglobin leads to the formation of methemoglobin and then denatured globin or sulfhemoglobin, which form insoluble masses that attach to the red cell membrane (called Heinz bodies). This oxidative denaturation of hemoglobin leads to its cross bonding; as a result, hemoglobin is no longer free to flow in the cytosol, producing the puddling of hemoglobin and bite or hemiblister cells in the peripheral smear
- Oxidation of membrane sulfhydryl groups leads to the accumulation of membrane polypeptide aggregates, presumably due to disulfide bond formation between spectrin dimers and between spectrin and other membrane proteins.,, The number of aggregates increases with the fall in red cell GSH.
The net effect is that the deficient red cells become rigid and nondeformable, making them susceptible to stagnation and destruction by reticuloendothelial macrophages in the marrow, spleen, and liver., This mechanism is shown in [Figure 4].
|Figure 4: Glucose-6-phosphate dehydrogenase protects red blood cells against oxidant injury. Oxidants generate hydrogen peroxide which is converted to water in a reaction catalyzed by glutathione peroxidase and associated with the conversion of reduced glutathione to oxidized glutathione. Glutathione is regenerated in a reaction catalyzed by glutathione reductase and associated with the conversion of NADPH to NADP. NADPH is regenerated in a reaction catalyzed by glucose-6-phosphate dehydrogenase in which glucose-6-phosphate is converted to 6-phosphogluconate. The last reaction (e.g., production of 6-phosphogluconate from glucose-6-phosphate) is the initial step in the hexose monophosphate shunt|
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Oxygen radicals play an important inflammatory role in all types of shock, including burn. These short-lived elements are highly unstable reactive metabolites of oxygen; each one has an unpaired electron, making them strong oxidizing agents. Infection during the burn is a major factor to produce oxidative stress. To prevent or treat it use of antimicrobials are must. The use of intravenous or oral antibiotics containing sulfa group also generates oxidative stress. The dressing of burn wounds plays major role in its treatment. Routinely, most of the burn center use silver sulfadiazine cream for dressing of this type of flame burn wounds. Use of topical silver sulfadiazine or mefenamic acid can produce hemolysis in patients having G6PD deficiency.,
We used these measures to treat the patient. Initial proper resuscitation and maintenance fluid supplements to this patient prevent shock. Vitamin C supplementation helped to reduce the free radical load. We had given 200 mg of Vitamin C per day till the patient was discharged (for antioxidant effects, dosage of Vitamin C is 200–1000 mg daily). Other than Vitamin C, folic acid can be used as antioxidant Vitamin in those having chronic hemolysis.
We used the measures mentioned ahead. Use of safe antibiotic to control infection, dressing was done with paraffin gauze in operation theater. Regular clinical evaluation of patient's condition by checking parameters (pulse, blood pressure), maintaining temperature and input–output chart was performed. Complete blood count with reticulocyte counts and urine test were regularly performed to check hemolysis. We regularly performed liver function tests; they were within normal limits. Lactate dehydrogenase was not monitored. Culture sensitivity of burn wounds was also done on regular basis. We gave blood transfusion once as per advised by a pediatrician. All the burn wounds healed without the need of skin grafting. We discharged the patient on the 26th day of treatment with regular follow-up till date.
| Conclusion|| |
Although the treatment of burn patient having G6PD deficiency is difficult, due to awareness of parents about the disease and taking proper measures not to trigger hemolysis, the patient was salvaged successfully without complication.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]