Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A long-term study of 17 patients with paroxysmal nocturnal hemoglobinuria revealed an unexpectedly high incidence of functional and anatomic renal abormalities. All patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. All patients also had granular casts in multiple urinalyses. Evaluation of renal function revealed hyposthenuria, abnormal tubular function and declining creatinine clearance. Radiologically, one or more of these demonstrated enlarged kidneys, renal cortical infarcts and thinning, papillary necrosis, acute renal atrophy, retroperitoneal hematoma and ureteral infarction, which were confirmed by autopsy studies. Hypertension developed in 7 patients. Urinary tract infection was uncommon and no patient had a clinical history compatible with chronic or acute pyelonephritis. Contrary to usual opinion our compatible clearly showed evidence of frequent and widespread renal pathology in paroxysmal nocturnal hemoglobinuria most likely due to repeated microvascular thromboses similar to the venous trombosis involving other organs in this disorder. Since most of these patients present initially to urologists knowledge of this entity is mandatory.
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PMID:Urologic manifestations of paroxysmal nocturnal hemoglobinuria. 114 29

Graded dextrans were used as tracers to study glomerular permeability in nephrotic rats. Two narrow range fractions were used, one which was approximately the same size as albumin (62,000 mol wt) and one which was considerably larger (125,000 mol wt). Nephrosis was induced with daily injections of an aminonucleoside of puromycin, and the animals examined after 7 days, when proteinuria is minimal, or after 10 days, when proteinuria has almost reached a maximum. At both stages and with both dextran fractions the following results were obtained: (a) dextran was retained for up to 3 h (the longest interval studied) in the plasma at high concentration; (b) there was a sharp drop in the concentration of tracer between the inner, looser portions of the basement membrane (lamina rara interna) and its outer denser portions (lamina densa), (c) accumulation of dextran was seen in the mesangial areas with time; and (d) no accumulation of dextran was seen in the slits at any time. These results are the same as those reported earlier in normal animals, and they demonstrate that in nephrotics the basement membrane still behaves as the main filtration barrier retaining most of the plasma proteins. Certain differences from the findings in normals were also noted in that increased amounts of the tracer were present on the epithelial side of the basement membrane: (a) in the urinary spaces; (b) in the subepithelial portions of the basement membrane; and (c) within lysosomes (protein absorption droplets) in the epithelial cytoplasm. In addition areas of thinning of the dense portions of the basement membrane (lamina densa) were seen which were accompanied by a corresponding widening of the less dense, subendothelial and subepithelial layers (lamina rara interna and externa, respectively). The presence of increased quantities of dextran on the epithelial side of the basement membrane indicates that the filter, i.e. the basement membrane, is leaky and allows increased passage of dextrans and therefore plasma proteins.
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PMID:The permeability of glomerular capillaries of aminonuceoside nephrotic rats to graded dextrans. 115 Dec 87

The visceral glomerular epithelium of rats made nephrotic by daily injections of puromycin aminonucleoside was examined from the time of onset of proteinuria (day 6) to the time when many animals die (day 15) in order to establish the chronology of the pathologic alterations which occur during the course of the disease. In addition, the structure of the residual epithelial slits was examined using special fixatives and freeze-fracture. Changes seen early in the disease (7 to 9 days) are: (1) a reduction in the number of foot processes and filtration slits; (2) occurrence of occluding junctions in many of the residual slits coupled with displacement of the slit diaphragms; (3) thinning of the dense central portion of the basement membrane (lamina densa) with a corresponding widening of the space (lamina rara externa) between it and the epithelium; (4) heightened epithelial pinocytosis with increased numbers of protein absorption droplets or lysosomes. In freeze-fracture preparations the occluding junctions were seen to be limited in extent and made up of only a few strands, indicating they are incomplete and represent occluding maculae or fasciae rather than zonulae. Later on in the disease (10 to 15 days) no further changes in the number or arrangement of slits is evident, but other alterations occur: (1) denuded regions of basement membrane are seen where there is initially partial and eventually complete detachment of the epithelium from the basement membrane; (2) increasing numbers of large vacuoles or phagosomes and decreasing numbers of fully condensed lysosomes are present; and (3) basement membrane-like material is seen in the spaces between the partially detached epithelium and basement membrane. The new findings in this study are: (1) the clarification of early (reversible) versus late (probably irreversible) changes in the glomerular epithelium in a acute aminonucleoside nephrosis; (2) delineation of the structure of the residual epithelial slits; (3) the description of progressive loosening of the attachment between the epithelium and the basement membrane leading to focal or complete epithelial cell detachment; (4) the presentation of evidence indicating that exhaustion of the lysosomal system of the glomerular epithelium (in protein absorption and concentration) occurs late in the disease. The available evidence is summarized and indicates that the glomerular basement membrane, the main glomerular filter, is defective in aminonucleoside nephrosis and allows increased protein leakage. However, it seems likely that the main site of action of aminonucleoside is on the epithelium leading to the production of defective basement membrane.
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PMID:Alterations of the glomerular epithelium in acute aminonucleoside nephrosis. Evidence for formation of occluding junctions and epithelial cell detachment. 124 24

Renal biopsy specimens of 29 Japanese non-insulin dependent diabetes mellitus (NIDDM) patients were examined by quantitative electron microscopic morphometry. In NIDDM the relative increase of percent total mesangium and mesangial capillary surface density (S/Vb) and the relative decrease of peripheral capillary surface density (S/Va) were compared with disease controls. However, mesangial-GBM-epithelial surface density (S/Vc) was not different between both groups. These results suggest that the increased mesangial matrix expands directly towards the capillary lumen as well as along the inner surface of GBM, and narrows the capillary lumen and filtration surface. The duration of diabetes mellitus (DM) did not correlate with all morphological parameters. The mesangial expansion correlated with urinary protein excretion and decreased creatinine clearance (CCr). GBM thickening correlated with proteinuria, but not with CCr. The degree of these morphological changes could be the indicators of hypertension of NIDDM patients. Areas of thin GBM were occasionally noticed in glomeruli which revealed thick GBM extensively, although the mechanism of GBM thinning is not known at the present time.
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PMID:An electron microscopic study of glomeruli in Japanese patients with non-insulin dependent diabetes mellitus. 151 97

Glomerulonephritis has been believed to be a rare complication in rheumatoid arthritis (RA). However, recent studies have revealed a focal segmental increase in mesangial cells and matrix in RA patients with hematuria. In our series, proteinuria, hematuria or both abnormalities were recognized in 74 (22%) out of 336 RA cases. Among 119 patients examined by renal biopsy, mild mesangial proliferative glomerulonephritis (GN) was found in 25 patients, of which 22 demonstrated mesangial IgA deposits, by immunofluorescent microscopy. Membranous nephropathy was noticed in 26 cases. Three cases of membranous nephropathy had no history of gold or D-penicillamine treatment. Electron microscopy revealed diffuse thinning of the glomerular basement membrane in 12 cases. The average thickness of the glomerular basement membrane was significantly thinner in RA patients than in normal subjects. The immunological processes associated with rheumatoid factor do not seem to be related to the renal lesions in RA patients.
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PMID:[Renal disorders in rheumatoid arthritis]. 158 51

To clarify the incidence of thin basement membrane disease (TMD) among the patients with idiopathic asymptomatic hematuria and/or proteinuria, in 217 serious renal biopsies (children 85, adults 132) were studied with clinical and morphometric analysis. TMD used is defined as follows: 1) Glomerulus in minor abnormalities; 2) GBM less than or equal to 200 nm in width, with more than 20% in total glomerular capillary surface; 3) Absence of significant immunoglobulins or complement components. Out of 217 patients 93% had either IgA nephropathy (55%), normal glomeruli (21%) or TMD (17%). TMD consisted of 22% in children and 14% in adults. Remained 15 consisted of non-IgA mesangial proliferative glomerulonephritis (6 cases), incomplete foot process disease (5), membranous nephropathy (2), membranoproliferative glomerulonephritis (1), and unclassified (1). Patients with TMD were found mostly (71%) in younger age less than 20 years old. Out of the patients with TMD, 38% had renal abnormality in the family history, but remainders were sporadic. The common urinary abnormality in TMD was microscopic hematuria occasionally with mild proteinuria (95%), while proteinuria only was rare (5%). Outcome of TMD was favorable prognosis with normal renal function. TMD was histologically divided into 2 groups; diffuse type (GBM thinning was more than 50% in the capillary surface) (19 cases; 51%), and focal type (it was less than 50%) (18 cases; 49%). The incidence of those family history was 64% and 15%, respectively (p less than 0.05). It was concluded that TMD was a popular disorder in patients with asymptomatic hematuria and/or proteinuria and it may expect 17% in incidence.
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PMID:[Thin basement membrane disease in patients with asymptomatic hematuria and/or proteinuria: a clinicopathological study]. 187 54

Using indirect immunofluorescence, cryostat sections from renal biopsy specimens of 14 adult patients showing marked diffuse thinning of the glomerular basement membrane (GBM) on ultrastructural analysis were examined for the presence of the Goodpasture (GP) epitope M2 using anti-M2 antiserum. In no case was a total absence of M2 noted. The fluorescence pattern was fine but homogeneously linear along the GBM in 12 cases, intensity varying from +-++, as compared with for the control specimen GBM. A faint, broken line of stain, intensity+, was observed in biopsy specimens of two patients, one of whom had family members with progressive hereditary nephritis, type Alport's syndrome. Clinical presentation was dominated by hematuria (10/14 patients) but also included three patients with isolated proteinuria. Two patients had nephrotic range proteinuria. Other than the GBM changes, histological findings were sparse, with either no abnormalities or only slight mesangial increase in most. One case of focal segmental sclerosis and hyalinosis was also found. The findings from this study suggest that the abnormally thin GBM does not lack the GP epitope, but it may be reduced.
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PMID:Abnormally thin glomerular basement membrane and the Goodpasture epitope. 218 29

Electron microscopic examination of glomerular basement membrane (GBM) was performed in 19 patients whose morphological changes as well as clinical features indicated the diagnosis of progressive hereditary nephritis (Alport's syndrome). The percentage of characteristically thickened and split and of thin GBM portions was determined in all the cases. The clinical course was more severe in males, which corresponded to higher rate of GBM alterations. In males, 58% of GBM was thickened and split and 24% was thin, while in females, the reverse was true, 28% was split and 48% of GBM was thin. There was a positive correlation of the split lesions and age in males, but not in females. The degree of splitting was directly proportional to the grade of proteinuria, while GBM thinning did not significantly correlate with proteinuria.
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PMID:Correlation of glomerular basement membrane alterations with clinical data in progressive hereditary nephritis (Alport's syndrome). 219 78

Persistent microscopic haematuria, especially when present in young individuals and associated with proteinuria, is generally of glomerular origin. Important causes include glomerulonephritis and Alport's syndrome. A new type of benign familial haematuria has now been reported characterised by little or no proteinuria, no deafness or hypertension and no deterioration of renal function. Examination by electron microscopy of renal biopsy material shows thinning or attenuation of the glomerular basement membrane. A family with thin basement membrane nephropathy is reported to illustrate this newly recognised entity. This disorder should be considered in any patient with persistent microscopic haematuria. Pathologists who assess renal biopsy material should pay greater attention to an ultrastructural morphometric analysis of the glomerular basement membranes of at least two glomeruli.
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PMID:Familial haematuria due to thin basement membrane nephropathy. 237 Oct 4

A prospective multicenter study was designed to determine the frequency and prognostic importance of hypercalciuria in children with hematuria. Urinary calcium excretion was examined in 215 patients with unexplained isolated hematuria (no proteinuria, urolithiasis, infection or systemic disorder). Hypercalciuria (urinary calcium excretion greater than 4 mg/kg/day) was identified in 76 patients (35%). Compared to patients with normal urinary calcium excretion, children with hematuria and hypercalciuria were characterized by male preponderance, white race, family history of urolithiasis, gross hematuria and calcium oxalate crystals. Renal biopsies were performed in 10 patients with urinary calcium excretion 0.4 to 2.5 mg/kg/day; three had IgA glomerulonephritis, three had glomerular basement membrane thinning, one had proliferative glomerulonephritis and three were normal. Renal biopsies in three patients with hypercalciuria showed focal segmental glomerulosclerosis, hereditary nephritis or no abnormalities. Oral calcium loading tests showed renal hypercalciuria in 26 patients, absorptive hypercalciuria in 15 patients and were not diagnostic in 35 patients. Serum parathyroid hormone, bicarbonate and phosphorus and urinary cyclic adenosine monophosphate concentrations were similar in the three groups of hypercalciuric patients. Urinary calcium excretion after one week of dietary calcium restriction was higher (5.8 mg/kg/day) in renal hypercalciuria than in other hypercalciuric patients (3.4 mg/kg/day), P less than 0.01. One to four years follow-up was available for 184 patients. Eight of 60 hypercalciuric patients developed urolithiasis or renal colic compared to 2 of 124 patients with normal urinary calcium excretion (P less than 0.001). Hypercalciuria is commonly associated with isolated hematuria and represents a risk factor for future urolithiasis in children with hematuria.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Idiopathic hypercalciuria: association with isolated hematuria and risk for urolithiasis in children. The Southwest Pediatric Nephrology Study Group. 240 91


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