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G6PD deficiency (favism)
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Glucose 6-phosphate dehydrogenase is an enzyme that converts glucose 6-phosphate to 6-phosphogluconic acid. 6 phosphogluconic acid produces NADPH, which ultimately preserves GSH (glutathione). Glutathione is an antioxidant.
This disease is the most common RBC enzyme disorder that manifests itself in two ways:
Episodic acute hemolytic anemia
Chronic nonspherocytic hemolytic anemia
This disease is X-linked.
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Symptoms:
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Jaundice
Indirect hyperbilirubinemia
restlessness
tachycardia
weakness
dark urine
Splenomegaly
Thunder in RUQ
Most people have no symptoms. unless exposed to oxidative substances.
Typically, helitic anemia in these people begins 24 to 48 hours after exposure to oxidative substances.
The factors that cause the acute phase include: (refer to the table of predisposing factors for the acute phase of favism)
Sulfonamides
Ciprofloxacin
Cotrimoxazole
Chloramphenicol
Primaquine
Naphthalene
Fava bean
DKA
Phenazopyridine
hepatitis
Sepsis
The severity of the symptoms depends on the type of triggering substance and the severity of the mutation in the patient.
In cases where the mother’s fetus has G6PD deficiency and the mother consumes an oxidative agent, the fetus may be born with G6PD deficiency and in the acute phase of the attack.
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Treatment:
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The basis of treatment is supportive and the patient should avoid oxidative factors that cause symptoms.
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Suggested order for treatment of acute episode of G6PD deficiency:
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Imp: G6PD deficiency Acute episode
Condition: Not Good / Not Bad / Fair / Emergent / Urgent
Diet: NPO
Please:
1. IV line fixed
2. Check V/S as routine + T chart
3. Check CBC-diff-BUN-Cr-Na-K-VBG-ESR-CRP-PBS-Retic count-Coombs- Bill (T,D)-AST-ALT-ALP – U/A- BS Glucometery- BG- Rh
CBC should show evidence of a helitic anemia.
Bite cell, blister cell, Heinz body are usually evident in the PBS of these patients.
The Retic Count of these patients has increased.
LDH of these patients has increased.
Bilirubin: There should be evidence of indirect hyperbilirubinemia.
Hemoglobin should be increased in U/A.
Very important point: due to the fact that in these patients, the enzyme defects that exist in old RBCs are more severe, when these people are in the acute phase, old RBCs are destroyed earlier and young RBCs They are replaced. The same thing causes the G6PD enzyme defect to be compensated to a large extent. So, in the acute phase of this disease, G6PD Level cannot be evaluated and judged.
It is better to check the G6PD level after two to three weeks.
4. Serum DW5%:
In these patients, fluid therapy should be done with 1.5 times the amount of maintenance. Also, the serum used for these patients must be potassium-free. Due to the lysis process that exists in these patients, they are at risk of hyperkalemia.
5. Collection of serial urine under the bed
6. Packed cell 10cc/kg Iso group, Iso Rh, Cross match
Indications for P/C in these patients include the following:
Hemoglobin less than 7
Hemoglobin between 7 and 9 to have evidence of lysis.
The prescribed dose of Pack Cell is 10 cc/kg at a rate of 3-5 cc/kg/hour, which increases the amount of hemoglobin by 2 to 3 mg/dl.
7. Check CBC
6 hours after administration of TB pack
#children #order #order #plan #emergency #children #blood #favism #G6PD #hemolysis #jaundice
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This post is written by Phxadmin