Hereditary Nephritis

(Alport syndrome)

is a hereditary nephritis is a genetically heterogeneous disorder characterized by a nephritic syndrome characterized, often with hearing loss and (d h hematuria, proteinuria, hypertension, renal failure possible..) – rarely – ophthalmological symptoms. The cause is a genetic mutation, the type IV collagen in question. Diagnosis is made by medical history, including family history, urinalysis, and biopsy (kidney or skin). The treatment is the same as that in chronic kidney disease, and sometimes a kidney transplant.

Hereditary nephritis is a nephritic syndrome, which is caused by a mutation in COL4A5 gene encoding the alpha-5-chain of type IV collagen and leads to modified type IV collagen strands. The mechanism by which developed by collagen change in glomerular disorder is unknown. However, it is believed a disturbed structure and function. In most families, there is a thickening or thinning of the glomerular basement membrane and tubular with focal or locally circumscribed fragmentation of the lamina densa ( “basket-weave pattern”). It can ultimately lead to glomerular scarring and interstitial fibrosis.

is a hereditary nephritis is a genetically heterogeneous disorder characterized by a nephritic syndrome characterized, often with hearing loss and (d h hematuria, proteinuria, hypertension, renal failure possible..) – rarely – ophthalmological symptoms. The cause is a genetic mutation, the type IV collagen in question. Diagnosis is made by medical history, including family history, urinalysis, and biopsy (kidney or skin). The treatment is the same as that in chronic kidney disease, and sometimes a kidney transplant. Hereditary nephritis is a nephritic syndrome, which is caused by a mutation in COL4A5 gene encoding the alpha-5-chain of type IV collagen and leads to modified type IV collagen strands. The mechanism by which developed by collagen change in glomerular disorder is unknown. However, it is believed a disturbed structure and function. In most families, there is a thickening or thinning of the glomerular basement membrane and tubular with focal or locally circumscribed fragmentation of the lamina densa ( “basket-weave pattern”). It can ultimately lead to glomerular scarring and interstitial fibrosis. The disease is most common X-linked inherited, although autosomal recessive and – rarely – autosomal dominant forms exist. Cases with X-linked inheritance can be clinically categorized as juvenile form: renal failure develops between 20 and 30 years Adults Form: renal failure develops in people> 30 years hereditary nephritis figure provided by Agnes Fogo, MD, and the American Journal of Kidney (see www.ajkd.org) Disease, Atlas of Renal Pathology. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/hereditary_nephritis_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/-/media/manual/professional/images/hereditary_nephritis_high_de.jpg?la = en & thn = 0 ‘, title:’ Hereditary nephritis ‘description:’ u003Ca id = “v38396884 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eGlomeruläre basement membranes have an irregular thinning and thickening on a leafy basket weave-like appearance in the transmission electron microscope. The changes are injury and remodeling caused ( u003cspan class = “”symbol “” u003e x u003c / span u003e6000) u003c / p u003e u003c / div u003e ‘credits’. Made illustration available Agnes Fogo

Health Life Media Team

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