Split and extremely thin glomerular basement membranes in hereditary nephropathy (Alport's syndrome)

Rumpelt, H. J.; Langer, Karl; Schärer, K; Straub, É; Thoenes, W.

doi:10.1007/bf00433075

Cited by 59 publications

(19 citation statements)

References 8 publications

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“…It is still possible that X-linked TBMN exists with inheritance, as depicted in Figure 3B. For example, clinical studies that were performed before identification of the COL4A5 gene indicated the existence of such cases (95,96); similarly, a more recent study by Liapis et al (97) suggested that a connection between some cases of TBMN and COL4A5 could exist. It is important to solve this controversy by more extensive sequence analyses of the COL4A5 gene in individuals with TBMN that does not show association with chromosome 2.…”

Section: -Terminus (B) Three Chains Form Triple-helical Molecules Thmentioning

confidence: 98%

Thin Basement Membrane Nephropathy

Tryggvason¹,

Patrakka²

2006

Journal of the American Society of Nephrology

108

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Section: -Terminus (B) Three Chains Form Triple-helical Molecules Thmentioning

confidence: 98%

Thin Basement Membrane Nephropathy

Tryggvason¹,

Patrakka²

2006

Journal of the American Society of Nephrology

108

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“…Those studies show that 'benign familial hematuria' with a thin GBM [14,15], 'thin mem brane nephropathy' [16], 'type II basement membrane nephropathy' [17] and Alport's syndrome [18][19][20][21][22][23][24][25] cannot be sharply distinguished from one another on the basis of family histories, prognosis or ultrastructural changes. They appear to represent parts of a spectrum of heredi tary nephritides with 'benign familial hematuria' with a thin GBM at one end and Alport's syndrome at the other.…”

Section: Discussionmentioning

confidence: 99%

Familial Thin Basement Membrane Nephropathy in Children with Asymptomatic Microhematuria

Gauthier

Trachtman

Frank

et al. 1989

Nephron

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A review of 130 children with persistent microhematuria showed that the most common abnormality was a form of hereditary nephritis for wich we propose the term ‘familial thin basement membrane nephropathy’ (FTBMN). This lesion is not as severe as that of Alport’s syndrome but carries a guarded prognosis. Family histories, urinalyses and audiograms systematically done on parents and other relatives show that FTBMN may be present in as many as 39% of children with persistent microhematuria and that careful family studies identify children likely to have this lesion.

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“…The first case was described in 1973 [7], and since then several clinical and pathological studies have emphasized the benign prognosis in most instances [1,8,9,10,11,12,13,14,15,16,17,18,19,20]. However, the finding of basement membrane thinning does not guarantee a benign course, because in the early stages of Alport syndrome, isolated GM attenuation may be the sole finding [21,22,23,24]. Moreover, extensive literature on adults with thin GBM suggest that this condition is sometimes associated with late onset of hypertension and renal failure [13,25,26,27,28].…”

Section: Introductionmentioning

confidence: 95%

Childhood thin GBM disease: review of 22 children with family studies and long-term follow-up

et al. 2005

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Thin glomerular basement membrane (GBM) disease is generally known to have a good renal prognosis, although renal insufficiency has sometimes been reported and the overlap with Alport syndrome implies that a good prognosis cannot be guaranteed. In order to shed light on long-term prognosis of thin GBM disease we have retrospectively evaluated 22 children with persistent haematuria and biopsy-proven thin GBM. Mean follow up was 7 years (range 2-17 years), mean age at onset was 7 years (range 1.5-15). Biopsies were performed a mean of 3.8 years after detection of hematuria. The light microscopy (LM) and immunofluorescence (IF) findings were essentially unremarkable in all of the children, while electron microscopy (EM) showed thinning of the GBM in all cases and no changes characteristic of Alport syndrome. The family history was positive for renal disease in 17 (77.3%) patients with hematuria in 8 (36.3%) families, and hematuria with renal failure (RF) or deafness in 9 (40.9%). It was completely negative for renal disease in 4 (18.2%) and unavailable in 1 (4.5%). Four patients (18%) showed a decline in renal function after 6, 8, 9 and 12 years of follow-up, and 1 of these also developed hearing impairment. None developed hypertension. Our study suggests that thin GBM disease is not always benign and a child with thin GBM should never be assigned such a prognosis, especially if there is a family history of renal impairment or deafness, where careful follow-up is needed due to the risk of late onset renal failure.

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Split and extremely thin glomerular basement membranes in hereditary nephropathy (Alport's syndrome)

Cited by 59 publications

References 8 publications

Thin Basement Membrane Nephropathy

Thin Basement Membrane Nephropathy

Familial Thin Basement Membrane Nephropathy in Children with Asymptomatic Microhematuria

Childhood thin GBM disease: review of 22 children with family studies and long-term follow-up

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