What is Alport syndrome

Hereditary nephritis (ie Alport's syndrome, AS) is one of the main manifestations of hematuria and renal function loss, sensorineural deafness and eye abnormalities inherited glomerular basement membrane disease, is due to encoding the glomerular the main collagen IV basement membrane collagen gene Mutations produce disease. Gene Mutations in occar Approximately 1/10000 to 1/5000.

Alport syndrome is Essentially a hereditary kidney disease-based clinical syndrome, and THEREFORE their performance in clinical practice is Necessary to pay attention to the Characteristics of kidney abnormalities, but Also pay attention to "renal" performance, but Also through family history try to infer the genetic type, because the clinical performance of Characteristics of different genetic type of Alport syndrome, and the prognosis varies.

Kidney performance

Hematuria most common, most of glomerular hematuria, the data from our Patients with Previously Reported 68% of Alport syndrome glomerular hematuria. Performance in Patients with X-linked dominant hereditary male persistent microscopic hematuria, hematuria or even within a few days after birth; outside microscopic hematuria was 100%. About 67% of the male Alport syndrome Patients episodes of gross hematuria, mostly in the 10 to 15 years ago, gross hematuria may, after upper respiratory tract infection or exertion. There are authors believe that the dominant X-linked Alport syndrome pedigrees hereditary aspect to 10 years of age have not yet found if hematuria, the boy most likely not Involved. Female Patients with autosomal dominant X-linked Alport syndrome type more than 90% had microscopic hematuria, a small number of female Patients with gross hematuria. Almost all autosomal genotype negative Hasta (both men and women) showed hematuria; autosomal recessive type and heterozygous relatives, hematuria incidence of 50 to 60 percent, not to exceed 80%.

Patients with X-linked Alport syndrome type will be the dominant male will appea proteinuria, hematuria and proteinuria showed age or sustained, even nephrotic range proteinuria, nephrotic syndrome incidence of 30 to 40 percent. First Hospital of Peking University, China Reported level of proteinuria of nephrotic syndrome accounted for 31.8%, and prompt poor prognosis. Similarly, the incidence and severity of hypertension increases with age, and mostly Occurs in Patients male.

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