myeloperoxidase deficiency
Introduction
Introduction to myeloperoxidase deficiency Myeloperoxidase deficiency (MPO) is an immunodeficiency disease of myeloperoxidase deficiency in hereditary phagocytic cells, which is rare. basic knowledge The proportion of illness: 0.0035% Susceptible people: no special people Mode of infection: non-infectious Complications: septic shock
Cause
Cause of myeloperoxidase deficiency
Cause:
Autosomal recessive inheritance with a family history.
Pathogenesis
MPO is another enzyme system in the phagocytic bactericidal system. When the enzyme activity is very low, the neutrophils completely lack the bactericidal activity of the MPO-H2O2-halide system, and the susceptibility to purulent bacteria and fungi increases. The gene is located at 17q22. -q23.
Prevention
Myeloperoxidase deficiency prevention
The cause of this disease is hereditary disease, there is no effective measure for the occurrence of this disease, but for those who have already had symptoms, they should actively treat the disease to prevent the development of the disease and complications. Strengthen nutrition and use foods that are easy to digest, contain enough calories, protein and vitamins. Such as porridge, milk, soft rice, soy milk, eggs, lean meat, fresh vegetables and fruits rich in vitamins A, B, C. These foods can enhance the body's resistance. In daily life, we should pay attention to nutrition and rationality. Try to diversify foods as much as possible. Eat high-protein, multi-vitamins, low-fat animal fats, digestible foods, fresh fruits and vegetables, and do not eat old-fashioned or irritating things. Grilled, marinated, fried, salty foods, staple foods and coarse grains to ensure nutritional balance.
Complication
Myeloperoxidase deficiency complications Complications septic shock
Various infections occur repeatedly. MPO activity is reduced or absent.
Symptom
Myeloperoxidase deficiency symptoms common symptoms immunodeficiency hypothermia fungal infection
There is a family history, and bacterial or fungal infections occur repeatedly from childhood. M2, M3 and MDS in AML can stimulate MPO deficiency.
Examine
Examination of myeloperoxidase deficiency
1. Neutrophil peroxidase staining is shown to be reduced or absent in MPO activity.
2. The tetrazolium blue reduction experiment and the oxygen consumption and glucose HMP metabolism were normal, and the H2O2 yield was not reduced.
According to the clinical manifestations, symptoms, signs can choose X-ray, B-ultrasound, electrocardiogram, biochemical and blood urinary bacteria culture.
Diagnosis
Diagnosis and differentiation of myeloperoxidase deficiency
There is often a family history of infection, and various infections occur repeatedly from childhood, and MPO activity is reduced or lacked, and the disease should be considered.
Must be identified with chronic granulomatous disease.
