Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0344329 (collapse)
 28,634 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Using semiquantitative morphometric methods, the clinical picture of decompensated benign nephrosclerosis is distinguished from that of secondary malignant nephrosclerosis, designated as the consequence of high pressure. It is shown that hypertensive glomerulopathy triggered by high pressure and postglomerular interstitial fibrosis with tubular atrophy are in the foreground of pathologic changes in decompensated benign nephrosclerosis, whereas the preglomerular vessel network is most often affected in secondary malignant nephrosclerosis. The preglomerular vascular lesions in secondary malignant nephrosclerosis lead to such heavy stenosis of the afferent vessels that the clinical picture of hypertensive glomerulopathy is rarely observed, while that of ischemic glomerular capillary collapse is frequent. The preferred affliction of the glomeruli and the postglomerular vessel network leads in decompensated benign nephrosclerosis to severe interstitial fibrosis, which has a pyramidal form, decreasing from the base of the pyramid at the corticomedullary boundary to the outer renal cortex. In secondary malignant nephrosclerosis fibrosis of the renal cortical interstitium is homogeneous in all layers of the renal cortex. Clinically, decompensated benign nephrosclerosis and secondary malignant nephrosclerosis, which occur predominantly in young to middle-aged males, manifest malignant hypertension. They are also accompanied by progressive renal insufficiency.
 ...
PMID:Decompensated benign nephrosclerosis and secondary malignant nephrosclerosis. 372 32

The occurrence of glomerular capillary aneurysms in light-chain nephropathy and a morphologic pattern of development as determined by electron microscopy are described. Renal biopsies from 4 patients with nodular glomerulopathy were evaluated. Light-chain deposition in mesangium and capillary walls was associated with monocyte accumulation in capillaries. Loss of endothelial cell lining and disruption of mesangial anchoring points of basement membranes ensued, resulting in aneurysmal dilatation of the capillaries. The walls collapsed, and the aneurysms healed as a result of mesangial interposition, a process which, in combination with enlarging nodules, led to thickening and collapse of the walls and narrowing of the lumens. This study, in conjunction with review of photographs from previously published reports, indicates that glomerular aneurysms are a common feature of nodular light-chain glomerulopathy. It also emphasizes additional light-microscopic similarities between this glomerulopathy and nodular diabetic glomerulosclerosis.
 ...
PMID:Glomerular capillary aneurysms in light-chain nephropathy. An ultrastructural proposal of pathogenesis. 391 54

An unusual presentation of glomerular disease occurring in a 30 year old black female is described. The case was characterized by a rapid deterioration of renal function. Associated glomerular manifestations were diffuse collapse of the glomerular capillary lumina, extracapillary hypercellularity without obliteration of the urinary space and without proliferation of parietal epithelial cells, electron-dense deposits, and deposition of IgG and C3. There was little loss of tubules and minor interstitial fibrosis. This case demonstrated a collapsing form of glomerulopathy with immunoglobulin and electron-dense deposits.
 ...
PMID:A collapsing form of glomerulopathy. 755 Oct 13

Sixteen patients with renal biopsy findings of extensive focal glomerular capillary collapse, visceral epithelial cell hypertrophy and hyperplasia, and variable degrees of tubulointerstitial injury in the absence of evidence for human immunodeficiency virus (HIV) infection or intravenous drug abuse were prospectively identified by renal biopsy. The pathologic process was designated collapsing glomerulopathy to distinguish it from other patterns of focal glomerular sclerosis. The clinical and pathologic characteristics of these 16 patients were analyzed and compared to a group of 25 patients with noncollapsing focal segmental glomerulosclerosis (FSGS). Thirteen of 16 patients with collapsing glomerulopathy were black as compared with 11 of 25 with FSGS (P = 0.018). The most common findings at presentation were hypertension and manifestations of the nephrotic syndrome. Although the duration of symptoms prior to presentation was no longer in the collapsing glomerulopathy group, the presenting mean serum creatinine was higher in patients with collapsing glomerulopathy than in those with noncollapsing FSGS (3.5 +/- 3.4 mg/dl vs. 1.3 0.6 mg/dl, P = 0.001). Twenty-four-hour urine protein excretion was also higher in the collapsing glomerulopathy group (13.2 +/- 7.7 g/day vs. 4.6 +/- 4.5 g/day FSGS, P = 0.005). The collapsing glomerulopathy patients had a mean age of 41.4 +/- 19.1 (range 19 to 81), a male-to-female ratio of 11:5 and a black-to-white ratio of 13:3. Renal survival, evaluated by life-table analysis, was markedly worse in collapsing glomerulopathy patients than in FSGS patients (P = 0.0004). It is proposed that collapsing glomerulopathy is a distinct entity characterized by black racial predominance, massive proteinuria, relatively rapidly progressive renal insufficiency, and distinctive pathologic findings.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Collapsing glomerulopathy: a clinically and pathologically distinct variant of focal segmental glomerulosclerosis. 807 54

A 47 year-old patient with an 8-year history of proteinuria was admitted to our hospital in 1989. His laboratory data were compatible with nephrotic syndrome: total serum protein 5.9g/dl (albumin 3.0g/dl), total serum cholesterol 280mg/dl and total urinary protein excretion 5.0g/day. Renal biopsy specimens contained 27 glomeruli associated with sclerosis and collapse of loops under light microscopical examination. In addition, mesangial proliferation and thickening of the basement membrane were visualized. With immunofluorescent study granular IgG deposits were detected in the peripheral region of the glomeruli. Staining for IgA, IgM, C3, Clq, light chain and Congo-red were all negative. On electron microscopy, microtubules apparently resembling cellular projections appeared to thicken the basement membrane. We are tempted to conclude that the current case is an atypical glomerulopathy accompanied by a glomerular microtubular-like structure.
 ...
PMID:[Nephrotic syndrome with microcellular projections into the thickened basement membrane]. 823 Aug 21

The characteristic pathological feature of collapsing glomerulopathy (CG) is marked cell hyperplasia and hypertrophy within the glomeruli. The present study investigated the phenotypic alteration of hyperplastic epithelial cells in CG to determine their origin. Renal biopsy specimens from two patients with CG were analyzed by immunohistochemical staining, using markers for podocytes (PHM-5), parietal epithelial cells (PECs; cytokeratin), and cell proliferation (Ki-67). In collapsed glomeruli, hyperplastic and hypertrophic epithelial cells were frequently connected to PECs and collapsed glomerular basement membranes (GBMs). These epithelial cells were more often Ki-67 positive and expressed cytokeratin, whereas PHM-5 was almost invariably negative. Serial section analysis showed that a small number of hyperplastic epithelial cells expressed both PHM-5 and cytokeratin, suggesting phenotypic conversion between podocytes and PECs. Moreover, cytokeratin-positive cells were associated with the sclerotic glomerular segments. Thus, we suggest that the majority of hyperplastic and hypertrophic epithelial cells in CG are of PEC origin. These epithelial features may participate in the development of characteristic tuft collapse and glomerulosclerosis in CG.
 ...
PMID:Origin and phenotypic features of hyperplastic epithelial cells in collapsing glomerulopathy. 985 11

In 1986, Weiss et al reported a group of patients with nephrotic syndrome, progressive chronic renal failure, and the histopathologic features of glomerular capillary collapse. Similar lesions are often described in human immunodeficiency virus (HIV) nephropathy. We evaluated 893 consecutive nontransplant renal biopsies performed in our department and the follow-up of the patients at our outpatient service. Sixteen specimens were identified with the pathological features of collapsing glomerulopathy (focal segmental or global glomerular capillary collapse and visceral epithelial cell hyperplasia), with no evidence of HIV infection and/or intravenous drug abuse. Their clinical characteristics were analyzed and compared with a group of 29 patients with noncollapsing focal segmental glomerulosclerosis (FSGS). The follow-up period of both patient groups was 5 +/- 1.46 years. The Kaplan-Meier life table method was used to present survival of the patients. The age of both groups was similar, 34 +/- 4 years (mean +/- standard error of the mean) for patients with collapsing glomerulopathy and 35 +/- 3 years for those with FSGS. The serum creatinine level was greater in patients with collapsing glomerulopathy (183 +/- 31 micromol/L) compared with those with FSGS (115 +/- 18 micromol/L), but the difference was not significant (P = 0.0504). The difference in proteinuria was not significant (P = 0.7668); it was 5.83 +/- 0.74 g/d in patients with collapsing glomerulopathy and 5.42 +/- 0.84 g/d in those with focal sclerosing glomerulonephritis. The difference in systolic (P = 0.4) and diastolic blood pressure (P = 0.556) was also not significant. Survival of the patients with collapsing glomerulopathy was worse than that of patients with FSGS (P = 0.025). Renal function survived 5 years in 40% of the patients with FSGS, but patients with collapsing glomerulopathy had no renal function survival. Our data suggest that idiopathic collapsing glomerulopathy is a distinct clinicopathologic entity with similar clinical features to focal sclerosing glomerulonephritis, but a worse prognosis and a rapidly progressive course toward end-stage renal disease.
 ...
PMID:Collapsing glomerulopathy: clinical characteristics and follow-up. 1019 29

Idiopathic collapsing glomerulopathy (ICG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis, characterized clinically by rapid progression of renal insufficiency, a male and African-American racial predominance, and pathologically by segmental glomerular collapse, visceral epithelial cell hypertrophy and hyperplasia, and the absence of endothelial tubuloreticular inclusions. Pathologically similar lesions have been reported in adult and pediatric patients with human immunodeficiency virus (HIV) infection and/or intravenous (IV) drug abuse. Most patients with ICG who have been reported in the literature are adults. Six children with ICG were retrospectively identified (two from East Carolina University, four from University of North Carolina-Chapel Hill). Clinical data and renal biopsy findings were reviewed for all patients. All six patients were male; five African-American and one Hispanic. Ages ranged from 2 to 17 years (mean 12 years). Steroid-resistant nephrotic syndrome was the presenting clinical finding. Average 24-h urine protein excretion was 6.3 g (range 3.2-15 g). Five patients were serologically negative for HIV infection (one patient not tested) and none had a history of IV drug abuse or known HIV risk factors. Progression to end-stage renal insufficiency in two patients within 1 year of biopsy required renal transplantation, and within 1 month of biopsy one patient required dialysis. We report a series of pediatric patients with ICG, an aggressive variant of focal segmental glomerulosclerosis. ICG in children is similar clinically and pathologically to this disease in adult patients.
 ...
PMID:Idiopathic collapsing glomerulopathy in children. 1068 63

Collapsing glomerulopathy is an aggressive form of glomerular disease defined for its histopathological features of glomerular collapse, visceral epithelial cell damage and tubulointerstitial changes that are characteristic. Patients with collapsing glomerulopathy present with severe nephrotic syndrome, marked proteinuria, generally more than 10 g/day and rapid progression to chronic renal failure, or death due to complications of nephrotic syndrome, despite any form of treatment. Collapsing glomerulopathy presents as de novo or recurrent disease in the renal allograft. There is slight predominance in males and strong predominance in blacks as renal diseases in general. Collapsing glomerulopathy shares several clinical and histopathological features with focal and segmental glomerulosclerosis and HIV-nephropathy; nevertheless, there is enough evidence to support collapsing glomerulopathy as a different entity. It must be mentioned that collapsing glomerulopathy, focal and segmental glomerulosclerosis and HIV-nephropathy may have a similar pathophysiological mechanism of damage to the visceral epithelial cell.
 ...
PMID:[Collapsing glomerulopathy: a new entity associated with nephrotic syndrome and end-stage renal failure]. 1097 63

Podocytes are well-differentiated postmitotic cells whose function is largely based on their complex cytoskeletal architecture. In diseases with proteinuria, podocytes undergo morphologic changes. Podocytes react to an injurious stimulus by a reorganization of their foot process architecture that is independent of the primary injury and the cause of the proteinuria. Collapsing glomerulopathies, including the idiopathic and secondary forms due to HIV infection, have been previously considered a part of the focal sclerosing glomerulosclerosis (FSGS) spectrum. However, in contrast to FSGS, both forms of collapsing glomerulopathy are characterized by segmental and global collapse of the glomerular basement membrane (GBM) and by characteristic ultrastructural alterations in podocytes. These alterations include loss of the actin-based cytoskeleton, a dysregulated/dedifferentiated phenotype, cellular hypertrophy, and cell proliferation. These observations raise the following questions: 1) What mechanism causes glomerular collapse and do podocytes have a role? We recently proposed that in collapsing glomerulopathies the composition of the GBM is altered and contains more immature forms of collagen IV. These observations suggest that dedifferentiated/dysregulated podocytes may participate in remodeling the GBM composition, producing fetal collagen isoforms. 2) What is the pathomechanism underlying podocyte dysregulation? Although it is still unclear which etiologic factors are responsible for the idiopathic forms of collapsing glomerulopathy, in situ hybridization studies in a transgenic mouse model of HIV-associated collapsing glomerulopathy and on renal biopsies of patients with HIV-associated collapsing glomerulopathy demonstrated the presence of the HIV-1 RNA in podocytes and tubular epithelial cells. These findings suggest a direct link between viral gene expression and the dysregulation of the podocyte phenotype. 3) Another open question is how podocytes become infected in HIV-associated collapsing glomerulopathy. HIV-1 typically uses CD4 and a co-receptor such as CCR5 or CXCR4 to enter cells. So far, there is no demonstration of the expression of these receptors in podocytes. These negative findings, however, do not exclude the possibility that in the kidney another, CD4 independent, co-receptor may be used for viral cell entry. Finally, is it important to mention that collapsing glomerulopathies have a high prevalence in black patients, suggesting a link between racial background and the virus-related podocyte injury.
 ...
PMID:Modulation of podocyte phenotype in collapsing glomerulopathies. 1201 94


1 2 3 4 Next >>