How long does hereditary nephritis come on?

summary

Alport syndrome (AS), also known as hereditary nephritis, familial glomerulonephritis and hereditary progressive nephritis, is a common monogenic renal disease in children. The incidence rate of 1/5000-10000 is Alport. Children often progress to renal failure in adulthood, accompanied by hearing and vision abnormalities. There is no specific treatment for this disease. Finally, they can only rely on renal replacement therapy such as hemodialysis, peritoneal dialysis and even kidney transplantation. Now share with you how long does hereditary nephritis come on?.

How long does hereditary nephritis come on?

First, Alport syndrome is mainly inherited by X-linked dominant inheritance. The pathogenic gene is located in the middle of the long arm of the X chromosome, so the inheritance is related to gender. The mother's disease is transmitted to the daughter, and the father's disease is transmitted to the daughter but not to the son. Hematuria (deformable red blood cell hematuria) is prominent and the first manifestation. Intermittent or continuous hematuria under naked eye or microscope is the most common manifestation. It is often aggravated after specific upper respiratory tract infection, fatigue or pregnancy. Renal function showed chronic progressive damage, especially in men, often in 20-30 years old into end-stage renal failure. It is often accompanied by high frequency nerve deafness. 10% - 20% of the patients have eye diseases, including myopia, strabismus, nystagmus, keratoconus, corneal pigmentation, spherical lens, cataract and fundus diseases.

Second: hereditary nephritis will appear hematuria symptoms at the beginning, the probability of boys will be higher, the average age of 5 years old onset. More than half of the patients can have gross hematuria, later the disease will worsen, slowly appear proteinuria. In the later stage of the disease, hypertension and progressive decline of renal function were common; Girl's symptom is lighter, often have hematuria only, won't affect life expectancy commonly. Hereditary nephritis will also affect hearing, beginning not serious, to school began to find hearing impairment. In addition, vision will also have progressive decline, children with strabismus, nystagmus, cataract and so on.

Third, the coding genes of these six chains are located on three chromosomes α 1 (IV) and α 2 (Ⅳ) on chromosome 13, COL4A1 and col4a2 genes were used to cheat; α 3， α 4 is encoded by the genes Col4a3 and COL4a4 on chromosome 2, α 5， α 6 is encoded by COL4A5 and col4a6 on the long arm of X chromosome. It is believed that autosomal dominant and recessive Alport syndrome is caused by Col4a3 / COL4a4 gene mutation on chromosome 2.

matters needing attention

Therefore, parents of patients with kidney disease in their families should pay attention to observe whether their children have abnormal physical performance, especially when their children have unexplained hematuria. Parents should cooperate with doctors to carry out family investigation and accept genetic guidance. Here we warmly remind you that prevention of kidney disease is a problem that every normal person should pay attention to.