Leukocyte Glucose-6-Phosphate Dehydrogenase Deficiency
Introduction
Introduction to leukocyte glucose-6-phosphate dehydrogenase deficiency G-6-PDdeficiency inleukocyte is reported in 1972 by Cooper et al., which is characterized by significant activity of glucose-6-phosphate dehydrogenase (G-6-PD) in patients with neutrophils. Reduced, repeated bacterial infections from childhood. basic knowledge The proportion of illness: 0.03% Susceptible people: no specific people Mode of infection: non-infectious Complications: urinary tract fungal infection
Cause
Causes of leukocyte glucose-6-phosphate dehydrogenase deficiency
(1) Causes of the disease
Bacterial infection caused by decreased 6-phosphate deactivating activity in leukocytes.
(two) pathogenesis
According to a study by Bachner et al. in 1971, G-6-PD is required for the catabolism of intracellular Hexase-monophosphate, which catalyzes the dehydrogenation of hexose monophosphate to produce NADPH, which is oxidized in NADPH. Under the action of enzyme, H202 is produced to exert bactericidal action in the cells. The activity of G-6-PDase in neutrophils of patients with this disease is lower than that of normal people (25%) (Congenital leukocyte G-6PD deficiency is confirmed) The neutrophil content of this enzyme is often low, but in most patients with erythrocyte G-6PD deficiency, the neutrophil G-6PD level is equivalent to 25% to 50% of normal people, and the H202 content in the cells. It is also lower than 25% of normal people. Even though the phagocytic function of neutrophils is normal, it does not have a bactericidal effect, so it does not increase oxygen consumption and hexose monophosphate bypass activity after phagocytosis of pathogens. Hydrogen peroxide.
Prevention
Leukocyte glucose-6-phosphate dehydrogenase deficiency prevention
prevention:
Promote eugenics and conduct pre-marital and prenatal checkups. Pay attention to adjusting the diet structure, pay attention to eating more fresh vegetables, supplement various vitamins, should not simply eat light foods, should be combined with vegetarians, eat plenty of food, drink plenty of water, maintain the body's nutritional balance, in order to enhance the body's immunity .
Complication
Leukocyte glucose-6-phosphate dehydrogenase deficiency complications Complications, urinary tract fungal infections
A urinary tract fungal infection can occur.
Symptom
Leukocyte glucose-6-phosphate dehydrogenase deficiency symptoms Common symptoms Fungal infection granulomatous infection induces coma
Mainly susceptible to H202-positive bacteria susceptibility, prone to staphylococcus, Escherichia coli, Serratia marcescens and many fungal infections, in addition to chronic granuloma symptoms, combined with mild non-spherical cell hemolysis.
Examine
Examination of leukocyte glucose-6-phosphate dehydrogenase deficiency
1. The NBT reduction experiment is normal or reduced, and the bactericidal power is low in most cases.
2. The addition of methylene blue to the neutrophil culture medium stimulated HMP, and the neutrophil HMP activity of CGD increased, but this case did not respond.
3. The neutrophil G-6PD activity decreased below normal 5%.
Diagnosis
Diagnosis and differentiation of leukocyte glucose-6-phosphate dehydrogenase deficiency
According to clinical manifestations combined with laboratory tests can be diagnosed.
The disease is generally not confused with other diseases.
