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)

Among patients with minor glomerular abnormality detected by light microscopy, but without significant finding by immunofluorescent study, fifteen cases showing global thinning of the glomerular basement membrane (GBM) when examined by electron microscopy were investigated as thin basement membrane disease (TBMD). Fourteen cases with a normal GBM thickness were selected as the control group. In the TBMD group, the mean width of the GBM, the distance between the cell membranes of the epithelial and endothelial cells, was 225 +/- 20 nm, whereas it was 354 +/- 42 nm in the control group. Some cases in the control group revealed a thinner GBM than other cases, which suggested the existence of an intermediate type between the TBMD and normal groups. The mean thickness of GBM in the intermediate and other control cases were 299 +/- 6 nm and 388 +/- 9 nm, respectively. In addition to global thinning of the GBM, the characteristic lesions of TBMD, a lucent appearance of the mesangial area (6 cases), widening of the subendothelial space (8 cases), splitting of the GBM (2 cases) and rupture of the GBM (one case) were observed in the TBMD cases, although these changes were mild in degree. A lucent appearance of the mesangium and widening of the subendothelial space were noticed more frequently in the TBMD group, compared with the control group. A common urinary abnormality in the TBMD patients was microscopic hematuria, which was occasionally associated with mild to moderate proteinuria. The renal function remained within the normal range in thirteen cases. Five cases of the TBMD group had members with renal abnormality in their family, of whom two showed renal failure and were undergoing hemodialysis.
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PMID:[A clinical and morphological study of thin basement membrane disease (TBMD)]. 796 72

A case of Ask-Upmark kidney is presented. An 18-year-old male patient referred to this facility presented with symptoms of hypertension, microscopic hematuria and proteinuria. A hormonal study revealed a high plasma renin activity level. Intravenous pyelography and abdominal computed tomography revealed thinning of the cortex with calyceal dilatation. Arteriography revealed a deep cortical groove in the middle portion of the kidney without renal arterial stenosis. Plasma renin activity of the left renal vein was significantly higher than that of the right renal vein. A left simple nephrectomy was performed under the diagnosis of Ask-Upmark kidney. Postoperatively, plasma renin activity returned to the normal range and a decrease in blood pressure was noted. Recent reports have suggested Ask-Upmark kidney to be a consequence of vesicoureteral reflux rather than a true congenital malformation. Our case indicated no evidence of vesicoureteral reflux and suggests that the lesion was congenital rather than acquired.
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PMID:[Ask-Upmark kidney: a case report]. 807 59

Six boys and six girls aged under 3 years with asymptomatic hematuria and/or proteinuria were found and renal biopsies were performed from 1 month to 10 years after the discovery. Light microscopy uncovered 4 cases of focal segmental glomerulosclerosis (FSGS), 3 cases of diffuse mild mesangial proliferation, and 5 cases of minor glomerular abnormalities. Immunofluorescent studies did not show any significant depositions. Electron microscopy revealed diffuse thinning of the glomerular basement membrane (GBM) in 2 cases, and segmental thinning of the GBM in 8 cases, including 2 cases of FSGS found in the light microscopy. Four cases, including 2 cases of FSGS, had irregular thickening of the GBM and splitting of the lamina densa. Two cases of FSGS did not have GBM abnormalities. The significance of GBM abnormalities in children with asymptomatic hematuria and/or proteinuria under 3 years of age needs to further evaluated.
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PMID:[Asymptomatic hematuria and/or proteinuria in children under 3 years of age: clinicopathological evaluation]. 808 72

A 59-year-old woman has had persistent microscopic hematuria for 10 years. A few days after the onset of upper respiratory tract infection, edema and severe proteinuria appeared. In renal biopsy, an electron micrograph revealed electron dense deposits in the subepithelial regions with diffuse thinning of the glomerular basement membrane. These glomerular findings were compatible with thin basement membrane syndrome associated with membranous nephropathy. On the basis of our review of the literature, the association of these two diseases seems to be very rare.
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PMID:A case of thin basement membrane syndrome associated with idiopathic membranous nephropathy. 810 10

The proband was a 14-year-old girl with hematuria and proteinuria. Many members of her maternal pedigree had hematuria and proteinuria. Her mother, younger brother (age 12 years) and younger sister (age 9 years) had microscopic hematuria and proteinuria with normal renal function. Her mother had nephrotic syndrome during pregnancy and a renal biopsy was performed. Light microscopic findings of the renal biopsy specimen revealed mild mesangial proliferation and irregularity of glomerular basement membrane. The pedigree showed no chronic renal failure and no deafness. The proband had experienced microscopic hematuria and occasionally macroscopic hematuria since 3 years of age. Proteinuria increased steadily and at the age of 14 years, she had nephrotic syndrome and renal dysfunction (creatinine clearance of 57.9 ml/min/1.48 m2). Renal biopsy was performed and light microscopic findings showed segmental mesangial cell proliferation and numerous interstitial foam cells without significant findings by immunofluorescent study. Electron microscopic examination showed splitting into many layers and thinning of the glomerular basement membrane. She had no complaint of hearing. However, audiological studies detected bilateral low-tone (from 125 Hz to 1000 Hz) sensorineural hearing difficulty, ranging from 30 to 40 dB. High scores on the short increment sensitivity index (SISI) test suggested inner ear hearing difficulty. Audiogram of her brother revealed also low-tone sensorineural hearing loss. Hereditary nephritis with the characteristic lesion of the glomerular basement membrane and sensorineural hearing difficulty has been known as Alport syndrome. Alport syndrome associated with familial low-tone hearing difficulty has not been reported previously.
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PMID:Hereditary nephritis associated with low-tone sensorineural hearing difficulty: a case report. 869 14

A mouse model for the autosomal form of Alport syndrome was produced. These mice develop a progressive glomerulonephritis with microhematuria and proteinuria, consistent with the human disease. End-stage renal disease develops at approximately 14 weeks of age. TEM analysis of the glomerular basement membranes (GBM) during development of renal pathology revealed focal multilaminated thickening and thinning beginning in the external capillary loops at 4 weeks and spreading throughout the GBM by 8 weeks. By 14 weeks, half of the glomeruli were fibrotic with collapsed capillaries. Immunofluorescence analysis of the GBM showed the absence of type IV collagen alpha-3, alpha-4, and alpha-5 chains and a persistence of alpha-1 and alpha-2 chains (these chains normally localize to the mesangial matrix). Northern blot analysis using probes specific for the collagen chains illustrate the absence of COL4A3 in the knockout, whereas mRNAs for the remaining chains are unchanged. An accumulation of fibronectin, heparan sulfate proteoglycan, laminin-1, and entactin was observed in the GBM of the affected animals. The temporal and spatial pattern of accumulation was consistent with that for thickening of the GBM as observed by TEM. Thus, expression of these basement membrane-associated proteins may be involved in the progression of Alport renal disease pathogenesis. The levels of mRNAs encoding the basement membrane-associated proteins at 7 weeks were unchanged.
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PMID:Collagen COL4A3 knockout: a mouse model for autosomal Alport syndrome. 895 99

The nail-patella syndrome is a hereditary disorder showing an autosomal dominant trait. It is characterized by a series of skeletal disorders and nephropathy. The skeletal defects and the renal involvement might occur separately. The usual clinical presenting syndromes of the nephropathy are asymptomatic proteinuria, microscopic haematuria and sometimes nephrotic syndrome. In a considerable proportion of patients renal failure develops. We summarise the clinico-pathological features of the disease presenting in two children and in a young man. The two children showed heavy microscopic occasionally, macroscopic haematuria, asymptomatic proteinuria and the adult patient had nephrotic syndrome. Nail-patella abnormalities were observed in one child without the involvement of family members. Except for the mother of the other child no urine abnormalities could be demonstrated in the patient's families. The kidney biopsy revealed the characteristic signs of the nail-patella syndrome in different extent: bundles of collagen fibrils in the glomerular basement membrane (GBM). Segmental and thinning of the GBM also occurred in the two children. This defect predisposes to the clinically dominant micro- and macroscopic haematuria. These children's reual function remained stable during the follow-up period of 4-7 years. In the GBM of the third patient small subepithelial electron dense deposits-corresponding to stage I. membranous glomerulonephritis- and extensive collagen deposition was found. After two years follow-up persistent nephrotic syndrome and gradual decline in renal function could be observed.
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PMID:[Nail-patella syndrome: clinico-pathologic characteristics]. 899 23

We performed a detailed clinical review and pathologic analysis of the kidney biopsies of 134 children with nephrotic syndrome or asymptomatic proteinuria. This analysis challenges some of our concepts about the classification of conditions associated with these disorders. The presence of focal segmental sclerotic lesions does not define a unique disorder in childhood. Some children with such lesions will have unaffected glomeruli that are ultrastructurally completely normal. These patients, predominately black adolescents, present either with nephrotic syndrome or asymptomatic proteinuria. We classify this disorder as primary focal segmental glomerulosclerosis (FSGS) and have never found it to recur after transplantation. Most other children with FSGS have 1 of 2 specific glomerulopathies. Those with minimal change have generalized fusion of podocyte foot processes. Those with mesangial proliferation have similar foot process changes combined with mesangial expansion and proliferation and, frequently, thinning of the lamina densa and tubuloreticular inclusions. The presence of segmental lesions in these glomerulopathies appears to be nothing more than a marker of severity. Children with these glomerulopathies are generally younger white children, virtually all of whom have nephrotic syndrome. These disorders have a strong propensity to recur after transplantation. The presence of mesangial labeling of IgM or C1q has no significance in any of these 3 disorders. The classification of disorders associated with nephrotic syndrome or asymptomatic proteinuria must concentrate less on the presence or absence of focal sclerosis and more on the histologic appearance of the rest of the glomeruli.
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PMID:The nonspecificity of focal segmental glomerulosclerosis. The defining characteristics of primary focal glomerulosclerosis, mesangial proliferation, and minimal change. 906 87

Benign recurrent hematuria usually indicates a good prognosis. This condition is associated with abnormally thin glomerular basement membranes. Of 680 renal biopsy cases in which lower urinary tract disease had been excluded by careful study, 25 cases from seven children and eighteen adults met the criteria for thin glomerular basement membrane disease, placing the incidence of the disease at 3.7%. The mean patient age was 32.4 years and the male to female ratio was 1 to 5.3. The primary finding was microscopic hematuria in eighteen patients and gross hematuria in five patients. Among eighteen patients who had microscopic hematuria, one patient also exhibited proteinuria and one patient suffered from acute renal failure due to acute drug-induced interstitial nephritis. Proteinuria was only found in one patient. All of the patients had normal renal function, with the exception of one who suffered from acute renal failure. The duration of hematuria from the time of detection to the date of biopsy ranged from 3 months to 30 years with a mean interval of 56.6 months. No apparent evidence of familial hematuria in any patient was noted. Under light microscopy most glomeruli were normal. However, five cases showed focal global sclerosis. Under immunofluorescence microscopy seventeen cases were negative for all immunoglobulins, for complement, and for fibrinogen. Eight cases showed nonspecific mesangial deposition of fibrinogen and/or IgM. Ultrastructurally, extensive diffuse thinning of the GBM was a constant finding. The mean thickness of the GBM was 203.2 +/- 28.3 nm (n = 25); the thickness in adult (201.4 +/- 27.5 nm; n = 18) did not differ from that in children (208.1 +/- 32.0 nm; n = 7).
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PMID:Thin glomerular basement membrane disease: light microscopic and electron microscopic studies. 925 Sep 20

Fifteen renal biopsy specimens from adult patients with minimal change disease (MCD) 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 normal controls. The electron micrographs of the glomeruli were scanned in Primax flatbed A4 scanner and then morphometric investigations were performed by means of a computer image analysis system to compare glomerular basement membrane (GBM) thickness and to study whether this parameter could correlate with the degree of proteinuria. The study revealed that the mean GBM thickness was lower in MCD patients as compared with normal controls (299.2 nm versus 338.8 nm), but this difference did not reach statistical significance. Interestingly, the present investigations showed that the GBM thickness tended to be related directly to proteinuria and not inversely as it might be expected, but this relationship was not significant, either. In conclusion, we can confirm the tendency to GBM thinning in adult MCD patients. It should be also noted, that GBM thinning did not increase the degree of proteinuria in these cases.
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PMID:Glomerular basement membrane thickness in minimal change disease. The ultrastructural quantitative study. 964 Sep 71


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