OVERVIEW
OVERVIEW
Leukocyte glucose 6-phosphate dehydrogenase deficiency is an inherited genetic defect in which the activity of glucose-6-phosphate dehydrogenase is significantly reduced in the patient’s neutrophils and is characterized by recurrent recurrent bacterial infections of various kinds since childhood.
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Department
Infection, Pediatrics
Synonyms
G-6-PD deficiency
Clinical Symptoms
Prone to staphylococcal, Escherichia coli, Serratia marcescens, and various fungal infections, chronic granulomatous symptoms, and mild non-spherocytic hemolysis. There may be systemic manifestations such as fever, headache, and malaise, as well as localized redness, swelling, heat, pain, and other purulent manifestations.
Hazards
Prone to a variety of fungal infections, resulting in decreased immunity, seriously affecting the health of the child.
Examination
Blood routine, NBT reduction test, neutrophil G-6-PD activity test, etc.
Diagnosis
Diagnosis is made on the basis of the child’s susceptibility to staphylococcal, Escherichia coli, Serratia marcescens, and many fungal infections, chronic granulomatous symptoms, and mild non-spherocytotic hemolysis, combined with the NBT reduction assay, and neutrophil G-6-PD activity test.
Treatment principle
Symptomatic supportive therapy, infection control.
Curability
Hemolytic episodes are generally self-limiting. Almost all patients with drug- or infection-induced hemolysis return to normal uneventfully. Jaundice in newborns with serosanguineous disease and bilirubin encephalopathy is relatively dangerous, but the mortality rate is very low with prompt treatment.
Dietary advice
Avoid fava beans and their products. High protein, high calorie, high vitamin diet, balanced nutrition, strengthen resistance.
Causes
Epidemiology
The age of onset is mostly under 5 years old.
Causes
Reduced 6-phosphate dehydrogenase deficiency in leukocytes, often to less than 5% of normal, with recurrent infections occurring if the level drops to 1%.
Symptoms and Diagnosis
Typical symptoms
Increased susceptibility to catalase-positive organisms, staphylococcal, Escherichia coli, Serratia marcescens, and fungal infections. In addition to chronic granulomatous symptoms, it is associated with mild non-spherocytic hemolysis.
Diagnostic basis
Children are susceptible to staphylococcal, Escherichia coli, Serratia marcescens, and various fungal infections, chronic granulomatous symptoms, and mild non-spherocytic hemolysis.The diagnosis is based on a normal or decreased NBT reduction assay, low bactericidal activity in most cases, and a decrease in neutrophil G-6-PD activity of less than 5% of normal.
Treatment
Treatment guidelines
Symptomatic supportive therapy, infection control.
Drug treatment
Define the pathogenic bacteria as early as possible and choose sensitive antibiotics. Take bactericidal drugs as the mainstay and in high doses. Continue the medication for 2~3 weeks after the condition is controlled to consolidate the efficacy and prevent recurrence.
Other treatments
Leukocyte infusion or bone marrow transplantation can be tried for severe infection.
Prognosis
Hemolytic episodes are generally self-limiting. Almost all patients with drug- or infection-induced hemolysis return to normal uneventfully. Jaundice in newborns with serosanguineous disease and bilirubin encephalopathy is relatively dangerous, but the mortality rate is very low with prompt treatment.
Nursing care
Daily care
1. Avoid taking medicines that can induce hemolytic attack. 2. Pay attention to rest, avoid exertion, keep happy, strengthen physical exercise and enhance physical fitness. 3.
Dietary management
1. Avoid consumption of fava beans and their products. 2. High protein, high calorie, high vitamin diet, balanced nutrition, strengthen resistance. 3.
Other Attention
Promote pre-marital checkup and eugenics.