Myeloperoxidase is high

In our lives, many people suffer from immune diseases. Immune diseases are generally caused by the lack of oxidase in our body, and this disease is also hereditary, which generally causes anemia and hyperlipidemia in our body. Therefore, if the patient has high water peroxidase in life, it must be dealt with in time. So what is the high myeloperoxidase?

Myeloperoxidase deficiency is a rare hereditary immunodeficiency disorder characterized by a deficiency of the enzyme myeloperoxidase in phagocytes. A familial disease characterized by proteinuria, anemia, hyperlipidemia, and corneal opacities. Further studies found that the patients' plasma cholesterol and triglyceride levels were increased, the lysolecithin level was decreased, the free cholesterol phosphatidylcholine level was increased, but the plasma phosphatidylcholine-cholesterol acyltransferase activity was not detected. These findings are the main manifestations of phosphatidylcholine-cholesterol acyltransferase deficiency, a familial metabolic disease. The disease often affects the kidneys. The patients initially reported were all Scandinavians, but it was later confirmed that the disease is widespread throughout the world, with uniform histopathological features and a constant pattern of lesions.

Symptoms and signs

MPO is a hereditary disease, so newborns have myeloperoxidase deficiency. Because they have not eaten, the concentration of harmful metabolites is not high, so there are no clinical manifestations at birth. The main clinical manifestations are:

Myeloperoxisome-like

1. Growth and development retardation In addition to physical growth and development retardation, the main manifestation is intellectual retardation. It manifests itself in an IQ lower than that of normal babies of the same age, and it can appear 4 to 9 months after birth. In severe cases, the IQ is below 50, and more than 14% of children are idiots, with language development disorders being particularly obvious. These findings suggest a developmental disorder of the brain. Limiting the intake of phenylalanine in newborns can prevent intellectual development disorders. Children with severe intellectual development disorders have higher blood phenylalanine concentrations than those with mild intellectual development disorders. Based on this, it can be considered that intellectual development disorders are related to phenylalanine toxicity. The more detailed pathophysiological mechanism is still unclear.

2. Neuropsychiatric manifestations include cerebellar malformation due to brain atrophy and recurrent convulsions, which tend to improve with age. Increased muscle tone and hyperreflexia. There is often restlessness, hyperactivity and abnormal behavior.

3. Skin and hair manifestations: The skin is often dry and prone to eczema and skin scratches. Because tyrosinase is inhibited, melanin synthesis is reduced, so the hair of the affected child is light brown.

4. Others Due to the lack of phenylalanine hydroxylase, phenylalanine produces increased phenyllactic acid and phenylacetic acid from another pathway, which are excreted in sweat and urine and have a musty odor (or rat odor). The clinical manifestations are related to the type of gene mutation and the severity of the clinical phenotype. The clinical phenotype of cofactor deficiency is milder than that of PAH protein abnormality.