Thin Basement Membrane

(Benign familial hematuria)

Under the syndrome of thin basement membrane ( “thin basement membrane disease”) is defined as a diffuse thinning in healthy people 300-400 nm thick glomerular basement membrane at about 150-225 nm.

The thin basement membrane is a kind of nephritic syndrome. It is hereditary and is usually transmitted as an autosomal dominant. Not all genetic mutations have been characterized so far. In some affected families but mutations in type IV collage-?4 gene were identified. The prevalence is estimated to be 5-9%.

From the syndrome of thin basement membrane ( “thin basement membrane disease”) refers to a thinning of the diffuse in healthy people 300-400 nm thick glomerular basement membrane at about 150-225 nm. The thin basement membrane is a kind of the nephritic syndrome. It is hereditary and is usually transmitted as an autosomal dominant. Not all genetic mutations have been characterized so far. In some affected families but mutations in type IV collage-?4 gene were identified. The prevalence is estimated to be 5-9%. Symptoms and signs Most patients remain asymptomatic. Microscopic hematuria is rather accidentally discovered during routine urine tests; occasionally a slight proteinuria and severe hematuria occur. Renal function is typically normal. but some patients develop kidney failure progredientes for unknown reasons. Recurring flank pain, similar to IgA nephropathy, are a rare manifestation. Diagnosis Clinical Investigation Sometimes renal biopsy The diagnosis is based on the family history and the findings of hematuria without other symptoms or pathology, especially if asymptomatic family members have hematuria. A kidney biopsy is not necessary, but is still often performed as part of a Hämaturieuntersuchung. In the initial stage, the delineation of this syndrome of a hereditary nephritis can be difficult due to the histologic similarities. Thin Basalmembranläsion figure provided by Agnes Fogo, M.D., and the American Journal of Kidney Disease, Atlas of Renal Pathology (see www.ajkd.org). var model = {thumbnailUrl: ‘/-/media/manual/professional/images/thin_basement_membrane_lesion_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/-/media/manual/professional/images/thin_basement_membrane_lesion_high_de.jpg?la = en & thn = 0 ‘, title:’ Thin Basalmembranläsion ‘description:’ u003Ca id = “v38396905 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eDiffuse attenuation of the glomerular basement membrane (thickness u003c 250 nm) in the transmission electron microscope ( u003cspan class = “”symbol “” u003e × u003c / span u003e3000). u003c / p u003e u003c / div u003e ‘credits’ figure provided by Agnes Fogo

Health Life Media Team

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