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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thin glomerular basement membrane (GBM) nephropathy, also called familial benign hematuria, is characterized by chronic hematuria and uniform thinning of the lamina densa of the glomerular basement membrane. It generally holds an excellent renal prognosis. Alport syndrome in early stages can also show attenuation of the GBM; conversely, renal insufficiency has been reported in familial benign hematuria. To discern early Alport syndrome from thin GBM nephropathy, we carried out a prospective epidemiological study in which 19 normotensive and non-azotemic adult patients with chronic microscopic (18 of 19) and macroscopic (1 of 19) hematuria and biopsy-proven thin GBM nephropathy were followed for a median of 12 years (range 9 to 15 years). Renal biopsies of thin GBM patients at entry showed an increased incidence of focal global glomerulosclerosis when compared to disease controls as IgA nephropathy (P = 0.047) and normal renal tissue (P = 0.0075). All renal biopsies showed the presence of the Goodpasture antigen when tested immunohistochemically. Presence of Alport syndrome was excluded clinically as none of the patients had complaints of hearing loss or abnormalities by audiography and ophthalmology. At the end of follow-up, the incidence of hypertension in thin GBM nephropathy (35%) exceeded that of healthy clinical controls (P = 0.048), and one hypertensive patient developed mild renal failure. In the normotensive patients, the glomerular filtration rate at follow-up as measured by inulin clearance was reduced in three out of seven; these were over 50 years of age. Although no family members were known to have renal disease at inclusion, within four families six elderly first degree relatives had developed unexplained renal insufficiency at the end of follow-up. Thus, thin GBM nephropathy predisposes to premature glomerular obsolescence, leading in time to increased incidences of hypertension and late onset renal insufficiency.
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PMID:Thin GBM nephropathy: premature glomerular obsolescence is associated with hypertension and late onset renal failure. 915 Apr 78

It has been reported that circumferential mesangial interposition (CMI) is an important morphological feature suggesting the progression of glomerulosclerosis in glomerular disease. The relation between CMI and its associated lesions was investigated in various renal diseases by electron microscopy. In 276 patients, of whom the glomeruli were observed by electron microscopy, CMI was observed non-specifically in 48 patients with various glomerular diseases (IgA nephropathy, 11; non-IgA glomerulonephritis, 1; membranoproliferative glomerulonephritis, 8; membranous nephropathy, 5; lupus glomerulonephritis, 12; toxemia of pregnancy, 2; diabetic nephropathy, 7; mitomycin nephropathy, 1; and Seckel's dwarfism patients, 1). The glomeruli with CMI showed a marked increase in mesangial matrix, as well as various grades of mesangial cell proliferation. Mesangiolysis associated with subendothelial widening was observed in a lesion of CMI in most cases. This phenomenon appears to be an initial alteration that conducts proliferated cells to the peripheral portion of a capillary loop. Localized severe thinning of the glomerular basement membrane was frequently combined with CMI, particularly in IgA nephropathy patients. Endothelial cells were occasionally interposed into the widened subendothelial space. Subendothelial deposits were noticed in the CMI lesion, particularly in MPGN patients. In conclusion, in the process of glomerulosclerosis progression in various glomerular diseases, lytic and edematous changes initially occur in the mesangio-subendothelial system (mesangiolysis and subendothelial widening), then proliferating mesangial cells extend into the widened space (between GBM and endothelial cells), and reach the peripheral portion of a capillary loop.
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PMID:[An electron microscopic study of circumferential mesangial interposition in various renal diseases]. 965 10

IgA nephropathy associated with thin basement membrane disease is reported in a 9-year-old female. The diagnosis of IgA nephropathy was made by means of an immunofluorescence investigation, which showed generalized diffuse mesangial deposits. Thin basement membrane disease was identified by electron-microscopic investigations, which disclosed thinning of the basement membrane of several capillary loops and prominence of the lamina densa. Her father, elder sister and younger sister were also found to have hematuria and her sisters were diagnosed as having thin basement membrane disease by renal biopsy. Patients with IgA nephropathy have focal thinning of the glomerular basement membrane, but we consider that urinalysis of the family needs to be done for the diagnosis of familial thin basement membrane disease, when diffuse thinning of the glomerular basement membrane is detected in such patients.
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PMID:A case of IgA nephropathy in three sisters with thin basement membrane disease. 973 May 67

Sixteen renal biopsy specimens from adult patients with IgA nephropathy (IgAN) for whom both light and electron microscopy as well as immunofluorescence microscopy and full clinical data were available were examined quantitatively and compared with six cases of normal controls. The electron micrographs of the glomeruli were scanned in a Primax flatbed A4 scanner and then morphometric investigations were performed by means of a computer image analysis system to evaluate glomerular basement membrane (GBM) thickness and to study whether this parameter could correlate with the degree of the haematuria, which is thought to be a main renal symptom in this glomerulopathy. The study revealed that the mean value of the GBM thickness was in IgAN patients significantly lower in comparison with normal controls (268.6 nm versus 338.8 nm). The authors also noted the tendency to negative correlation between GBM thinning and haematuria, although this relationship was not significant. It is suggested that deposits located in the vicinity of the capillary loops may play a role in this process.
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PMID:Glomerular basement membrane thickness in primary diffuse IgA nephropathy: ultrastructural morphometric analysis. 982 Oct 57

Membranous nephropathy, mesangial proliferative glomerulonephritis and renal amyloidosis are common renal pathology in RA patients. However, IgA nephropathy and diffuse thinning of glomerular basement membrane are described as common and characteristic renal lesions in Japanese RA patients. Glomerular filtration rate may decrease significantly in active lupus nephritis, but renal plasma flow does not change or even increase. These findings seem to be characteristic of SLE patients with active renal disorders. Therefore, filtration fraction may be a useful clinical parameter to evaluate SLE patients. Scleroderma renal crisis(SRC) has been believed to be the most serious renal disorder in systemic sclerosis (SSc). Recently, the presence of an antibody to RNA polymerase has been associated with a high prevalence of SRC.
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PMID:[Renal disorders in patients with collagen vascular diseases]. 1007 13

The term thin glomerular basement membrane disease (TBMD) refers to a condition characterised by thinning of the GBM at electron microscopy examination and, clinically, by isolated hematuria, frequently occurring in other family members, with no extra-renal manifestations. Progression towards chronic renal failure (CRF), although rare, has been reported and blood pressure is high in 30-35% of cases during follow-up. TBMD is generally considered different from Alport syndrome since immunohistological investigation does not show abnormalities of type IV collagen alpha chains in the GBM, as frequently observed in Alport patients; moreover, in familial cases, the disease is transmitted as autosomal dominant trait, rarely observed in Alport syndrome. Genetic studies suggest that TBMD is a heterogeneous disease, but some cases may be related to mutations of COL4A3/COL4A4 genes, thus belonging to the spectrum of type IV collagen diseases. TBMD may arise with other glomerular diseases, most frequently IgA nephropathy, and it remains to be established whether these cases are a casual occurrence or whether a thinner than normal GBM predisposes to immune complex deposition.
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PMID:Thin glomerular basement membrane disease. 1072 Feb 10


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