ISSN:0250-8095    

DOI:10.1159/000167599

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A variety of histologic changes have been observed in the kidneys of patients with light-chain deposition disease. We report 3 cases with kappa light chain deposition who presented with diverse clinical, histological, and ultrastructural features. All the cases were diagnosed by the presence of monoclonal kappa light chain deposits by immunohistochemical methods. Based on the present study and review of literature, four basic patterns of glomerular lesions are identified: (1) minimal glomerular changes without glomerular deposits, but with extensive tubular involvement; (2) mild glomerular changes with glomerular deposits; (3) focal or diffuse proliferative glomerulonephritis with or without crescents, and (4) nodular glomerulosclerosis resembling diabetic glomerulosclerosis. This pathological and clinical variability can cause diagnostic problems at initial presentation. A high index of suspicion is necessary along with appropriate laboratory data and routine staining for light chains.

ISSN:0250-8095    

DOI:10.1159/000167600

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

β2-Microglobulin (β2m) has been identified as the major constituent of dialysis-related amyloid. Although there is no clear correlation between absolute 62m levels and amyloidosis-related symptoms, elevated serum levels are thought to be the basis for tissue deposition of β 2m. Besides diminished renal excretion and insufficient removal during hemodialysis, a dialysis-related induction of β 2m production is discussed as the major cause of elevated serum 62m levels. In order to evaluate the influence of hemodialysis membranes and the hemodialysis procedure on 62m levels we determined serum β 2m levels in patients on chronic intermittent hemodialysis. Polymethylmethacrylate 2.0 m2, cuprophane and cellulose acetate dialyzers led to increasing β 2m levels during dialysis, which was in excess of what could be accounted for by hemoconcentration. The polymethylmethacrylate 1.6 m2 dialyzer did not result in a significant rise of β 2m levels during dialysis. This indicates that production of 62m is not only dependent on the membrane material but also on the surface area of the dialyzer. The use of polysulfone and hemophane low-flux dialyzers did not induce an increase in β 2m levels during dialysis but a significant clearance of β 2m was not demonstrable either. Volume-controlled dialysis with high-flux membranes (polysulfone 0.65 m2 and polysulfone 1.25 m2) lowered β 2m; clearance values, however, were significantly higher when these dialyzers were used in a hemodiafiltration procedure. We conclude from our study that some dialysis membranes appear to induce β 2m production, whereas others do not. Significant removal of β2m, however, can be obtained by hemodiafiltration and a significant decrease of predialytic β 2m levels was obtained after only 2 months of application of this treat

ISSN:0250-8095    

DOI:10.1159/000167601

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

Patients with stable renal insufficiency were randomized into two groups: (1) patients given the channel blocker nisoldipine (n = 17) and (2) placebo-treated patients (n = 17) also taking their regular antihypertensive therapy which did not include calcium blockers. Patients were already on low protein diet with a protein intake of 0.80 ± 0.2 in the nisoldipine group versus 0.85 ± 0.25 g/kg body weight in the placebo-treated group. The monthly progression of their renal failure was assessed by the reciprocal of serum creatinine versus time in months. After a mean follow-up of 17.4 ± 8.2 (range 6–30) months, the nisoldipine-treated group had a significant decrease in their slope of progression, whereas the placebo-treated patients, after 16.94 ± 7.2 (range 6–30) months of follow-up, had no significant change in their slope. The protein intake during follow-up was similar, being 0.85 ± 0.2 g/kg actual body weight in the nisoldipine-treated group and 0.88 ± 0.26 g/kg in the placebo group. The changes in slope did not correlate with the changes in blood

ISSN:0250-8095    

DOI:10.1159/000167602

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

We describe 40 adults with idiopathic minimal-change glomerulopathy. They consisted of 27 females and 13 males, mean age 40.7 ± (SD) 19.8 years (range 15–78 years). Twenty patients were < 40 years of age at presentation. They presented with significantly ( p 1.3 mg/dl). All patients had nephrotic-range proteinuria at the time of presentation or biopsy. There was no significant difference in presenting proteinuria (8.7 ± 5.7 g/24 h) or length of follow-up (mean 63.5, range 4–176 months) between the two age groups. Microscopic hematuria and hypertension were each present in 21 % of the patients. Thirty-four patients received therapy with prednisone. A complete remission was obtained in 91 % of the patients treated with prednisone. The response occurred within 16 weeks in 77% of the patients. The response to prednisone therapy was similar for patients 40 years, with a complete remission being obtained in 88 and 94%, respectively. The rate of response, however, differed significantly with 73% of patients 40 years achieving a complete remission by 8 weeks. Twenty patients initially responding to prednisone therapy (64.5%) relapsed. A relapse occurred within 3 months of attaining a complete remission in 70% of the patients. No difference was recognized between patients 40 years with respect to initial relapse time or total relapses. Eight patients (26%) were steroid dependent. A spontaneous complete remission occurred in 2 patients. At last follow-up, 29 patients (72.5%) were in complete remission, 3 patients were in a partial remission (7.5%), and 8 patients (20%) were nephrotic. Our experience shows that adults with minimal-change glomerulopathy have a clinical presentation and course similar to that of children with the same lesion. A major difference was that the rate of response in our patients was slower than that reported in children. Once in remission, relapse rate and prognosis of our adults mirrored that of the childhood experience with minimal-change glome

ISSN:0250-8095    

DOI:10.1159/000167603

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

Animals and humans undergoing treatment with ciclosporin (CS) show a reversible increase in renal vascular resistance and a decrease in glomerular filtration rate. The causes of these abnormalities have not yet been established. We evaluated the effects of a 1-week treatment with CS on creatinine clearance, renal arachidonic acid metabolites, plasma renin activity (PRA), plasma aldosterone levels, urinary excretion and plasma levels of catecholamines in 7 patients with idiopathic uveitis. We show that CS treatment induces a significant (p < 0.05) decrease in creatinine clearance (from 132 ± 0.5 to 108 ± 8 ml/min); urinary 6-keto-PGF1 excretion (from 17.8 ± 4.9 to 10.9 ± 3.3 ng/mmol creatinine), urinary thromboxane B2 excretion (from 7.0 ± 1.0 to 3.6 ± 0.9 ng/mmol creatinine), upright PRA (from 4.2 ± 0.9 to 2.3 ± 0.8) and supine PRA (from 2.0 ± 0.5 to 1.1 ± 0.3). We found no change in plasma aldosterone levels and plasma levels and urinary excretion of catecholamines. We suggest that the reversible renal vasoconstriction observed in patients treated with CS may be induced by inhibition of renal prostacyclin synthesis. In this setting inhibition of PRA and angiotensin II formation may impair autoregulation of effective filtration pressure and therefore glomerular filtra

ISSN:0250-8095    

DOI:10.1159/000167604

出版商:S. Karger AG    

年代:1988

数据来源: Karger

ISSN:0250-8095    

DOI:10.1159/000167605

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A woman with a history of urethral stenosis, not causing significant urinary tract obstruction but requiring dilatation, presented at the age of 49 with renal failure (creatinine clearance of 16–20 ml/min). Investigations (including pyelography) directed to finding the cause of her renal failure were negative with the exception of renal biopsy which revealed granulomata, one of which showed caseation. Treatment with antituberculous drugs arrested the decline in glomerular filtration rate and was later associated with sustained improvement in renal function. Tuberculosis should always be considered as a possible cause of unexplained renal failure even if the classical pyelographic features of tuberculosis, i.e. calyceal distortion and calcification, are absent. Appropriate treatment may improve renal function and avert the need for renal replacement therap

ISSN:0250-8095    

DOI:10.1159/000167606

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

The pathogenesis of Bartter’s syndrome remains uncertain. The prevailing theory postulates a defect in salt reabsorption, more frequently described in the thick ascending limb of Henle’s loop. The patient we studied presents a normal urinary concentration capacity associated with impaired dilution, a free water clearance at the lower end of normal (5.4 ml/min/lOOml glomerular filtrate), a decreased distal fractional chloride reabsorption (54%) when studied during hypotonic saline diuresis, and a normal decrease in free water clearance after furosemide (2.1 ml/min/100 ml glomerular filtrate), suggesting a defect in the cortical part of Henle’s loop. When studied during oral water diuresis, the fractional chloride reabsorption was normal (82%). This could be explained by a relative inability of the cortical diluting segment to reach maximal absorptive rates for NaCl. An inappropriate kaliuria related to an excessive delivery of salt load to the distal tubule is suggested by the correlation between urinary potassium and chloride excretion (r = 0.84; p < 0.001). Aldosterone secretion participates also partially in the urinary potassium

ISSN:0250-8095    

DOI:10.1159/000167607

出版商:S. Karger AG    

年代:1988

数据来源: Karger

摘要:

A 57-year-old Caucasian male presented with severe nephrotic syndrome and diffuse organomegaly; he subsequently developed renal failure and died. Intracellular, crystalloid material was identified by light and electron microscopy in bone marrow, liver, spleen, mesenteric lymph nodes, and kidneys. Tissue extraction analysis identified the material as glucocerebroside and its immediate precursor, ceramide lactoside. Although Gaucher’s disease cannot be completely excluded, glycolipid profiles do not conform to those of known storage disorders. Additionally, electron-microscopic studies indicate that the structural features of the glycolipid deposits are different from those of previously described storage diseases. These findings suggest a unique crystalloid deposition as the probable cause of a multisystem process, which was associated with renal insufficiency and deat

ISSN:0250-8095    

DOI:10.1159/000167608

出版商:S. Karger AG    

年代:1988

数据来源: Karger