Hereditary nephritis with a characteristic renal lesion

Sherman, Raymond L.; Churg, Jacob; Yudis, Melvin

doi:10.1016/0002-9343(74)90749-9

Cited by 27 publications

(6 citation statements)

References 15 publications

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“…Four patients with the diagnosis of children. Most frequently the renal disorder is a glomerulone-benign familial hematuria presented with asymptomatic recurrent phritis (2,5,8,9,17). Asymptomatic hematuria is an early and hematuria without proteinuria.…”

Section: Methodsmentioning

confidence: 99%

Alteration of Collagen Metabolism in Hereditary Nephritis

et al. 1979

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SummaryHereditary nephritis (HN) is a progressive renal disease not uncommonly seen in children. Asymptomatic hematuria is an early and consistent finding'with or without proteinuria. Distinctive abnormalities frequently associated with this condition involve basement membranes primarily (glomerular basement membrane, lens capsule, and possibly, tectorial membrane of the organ of Corti). These basement membranes contain collagen with similar biochemical composition suggesting that this association may be pathogenetically related. On the basis of this observation, we studied collagen metabolism in patients with HN by utilizing urinary hydroxylysine glycosides (hyl glyc) as an index of collagen metabolism. The hyl glyc hydroxylysine galactose glucose and hydroxylysine are basic units in basement membrane as well as a large number of other collagen. We studied 25 normal children, seven patients with HN, and six HN siblings with hematuria. The mean excretion of total hyl glyc are 64 + 5.5,93 + 14, and 123 +. 15.6 umoles/g creatinine, respectively. The difference in the mean excretion level of total hyl glyc between patients with HN and normal controls is statistically different (P < 0.05).The siblings of HN patients differed significantly from normal (P > 0.005), but was comparable to the HN patients. Two of 16 additional patients studied had the Nail Patella syndrome and showed increased urinary hyl glyc. The elevated urinary hyl glyc in HN patients and their siblings strongly suggests an altered rate of collagen degradation during the natural course of the disease. The origin of collagen is presently unknown because no data exists describing the biochemical composition of basement membrane collagen in HN. To elucidate further the pathogenesis of this important disease, studies should investigate the biosynthesis and metabolism of basement membrane collagen of involved structures.sibly, tectorial membrane of the organ of Corti). Collagen recently shown to be present in these basement membranes have very similar biochemical composition (10). Collagen changes have been reported in the organ of Corti in HN. (12).On the basis of these observations we studied collagen metabolism in patients with HN by measuring urinary hydroxylysine glycosides. The hyl glyc are hydroxylysyl galactose glucose (HGG) and hydroxylysyl galactose (HG). These two glycosides are basic units in basement membrane collagen as well as a large number of other collagens. They are metabolized only to a minor degree before excretion and have a high threshold of clearance. MATERIALS AND METHODSEarly morning specimens were collected and stored without preservative at -70°C pending analysis. Informed consent for the study was obtained from the parents. Seven patients (M-4, F-3) ages 6-13 with HN were studied. All patients had positive family and clinical history. Two of four affected males were brothers with neurosensory hearing loss and were both azotemic. One girl was uremic without hearing loss at the time of the study. No other abnormalities were obse...

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Section: Methodsmentioning

confidence: 99%

Alteration of Collagen Metabolism in Hereditary Nephritis

et al. 1979

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“…The diagnostic finding of hereditary nephritis is seen on the ultrastructural examination of glomeruli. In males, there is diffuse thickening, lamellation, and reticulation of the GBM, imparting a characteristic bbasket weaveQ appearance [2][3][4]. Electron-dense particles of 20 to 60 nm in diameter are frequently seen in the lucent areas between the GBM lamellations [5].…”

Section: Introductionmentioning

confidence: 99%

Hereditary nephritis mimicking immune complex–mediated glomerulonephritis

et al. 2006

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“…Histologic and light microscopic findings are similar to those described in people. 19 These canine models have made significant contributions to the understanding of the pathogenesis and pathology of Alport syndrome in people.…”

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confidence: 99%

Histologic and Ultrastructural Studies of Juvenile Onset Renal Disease in Four Rottweiler Dogs

Wakamatsu¹,

Surdyk²,

Carmichael³

et al. 2007

Vet Pathol

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Abstract. Juvenile onset renal disease is described in 2 male and 2 female young Rottweiler dogs. Histologic changes in all dogs were cystic dilatation of Bowman's space, mesangial hypercellularity, and glomerulosclerosis. Three dogs also had glomerular crescents and moderate to severe interstitial fibrosis. Electron microscopy revealed glomerular basement membranes of variable thickness, with extensive splitting or lamellation of the lamina densa. These ultrastructural findings are similar to those found in people and in other breeds of dogs with inherited defects in type IV collagen.

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Hereditary nephritis with a characteristic renal lesion

Cited by 27 publications

References 15 publications

Alteration of Collagen Metabolism in Hereditary Nephritis

Alteration of Collagen Metabolism in Hereditary Nephritis

Hereditary nephritis mimicking immune complex–mediated glomerulonephritis

Histologic and Ultrastructural Studies of Juvenile Onset Renal Disease in Four Rottweiler Dogs

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