摘要:

The basic tubular alteration present in Bartter’s syndrome is still a subject of controversy. The possibility that a generalized defect in the transmembrane ion transport underlies the disease has been extensively investigated. Previous evaluations of cellular sodium metabolism in Bartter patients showed extremely variable findings. We have examined in the red blood cells of two patients with Bartter’s syndrome the intracellular Na+ and K+ concentrations, the activity of the ouabain-sensitive Na+/K+ pump, furosemide-sensitive Na+/K+ cotransport, Na+/Li+ countertransport and the rate constant of Na+ and K+ passive permeability. We have compared these values with those of healthy subjects and patients with chronic hypokalemia produced by conditions other than Bartter’s syndrome. Ouabain-sensitive Na+/K+ pump activity was decreased in both patients, whereas Na+/Li+ countertransport was activated. One of the patients also exhibited markedly decreased intraerythrocyte K+ concentration and decreased furosemide-sensitive Na+/K+ cotransport. The other had increased Na+/K+ cotransport activity and Na+ passive permeability. Intracellular Na+ and passive permeability to K+ were normal in both subjects. Neither oral potassium supplementation (100 mEq/day) nor indo-methacin treatment (150 mg/day) could correct these abnormalities. Our results are partially consistent with previous observations and indicate the existence of heterogenous abnormalities of erythrocyte sodium transport systems in patients with Bartter’s syndrome which are not a consequence of chronic hypokalemia.

ISSN:0250-8095    

DOI:10.1159/000168435

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Although the efficacy of hepatitis B vaccines in patients under chronic hemodialysis treatment has been well documented, the persistence of immunity in this population remains largely unknown. In this study we have followed 60 hemodiaysis patients up to 3 years after primary hepatitis B vaccination (four doses of recombinant hepatitis B vaccine; Engerix B, 20 mg/dose) to evaluate the persistence of immunity (as indicated by serum levels of antibody to hepatitis B surface antigen – anti-HBs – higher than or equal to 10 mlU/ml). Four-ty-four (73%) patients developed anti-HBs levels above 10 mlU/ml after vaccination; the remaining 16 (27%) vaccinees were considered nonresponders and were given a booster dose that again failed to elicit an immunoresponse. After 3 years of follow-up, 18 out of 44 (41 %) responders had no detectable anti-HBs levels in the serum (antibody loss occurring within 8 and 12 months in 3 cases, within 1 and 2 years in 13, and within 2 and 3 years in 2 other cases). When compared with the responders that lost their antibodies during the follow-up period, those who remained immunoreactive 3 years after vaccination was initiated were younger and had higher anti-HBs levels at 8 months of follow-up. Two hepatitis B virus infections were detected among nonresponders during the follow-up period. Based on these data, we conclude that patients undergoing chronic hemodialysis therapy not only have lower response rates to hepatitis B vaccination than healthy adults, but also that these are frequently transient. We then recommend for this population (1) anti-HBs testing soon after vaccination and (in case of response) every 6 months thereafter and (2) to give a booster dose to primary responders whenever their antibody levels decrease below 10 mlU

ISSN:0250-8095    

DOI:10.1159/000168436

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

The effect of triidothyronine (T3) on the responses to mitogens and on the production of prostaglandin E2 and interleukin 2 were studied in serum-free cultures of peripheral blood mononuclear cells (PBMC) in 20 patients undergoing hemodialysis and in 30 control subjects. T3 increased the growth of PMBC induced by phytohemagglutinin and pokeweed mitogen in both groups. PBMC reached growth maximum at 0.5 nM T3 when stimulated by phytohemagglutinin in both groups. At higher concentrations of T3 the effect declined in the control group, but the response of uremic PBMC was constant. The response to T3 of pokeweed mitogen stimulated PBMC was lower in the uremic patients. The production of prostaglandin E2 by PBMC was higher in the uremic patients than in the controls. T3 had no effect on prostaglandin E2 production. Indomethacin alone and in combination with T3 had a stimulatory effect on cell growth in the patient group. T3 had no effect on the release of interleukin 2 by PBMC. An additive effect of interleukin 2 and. T3 was observed in cultures stimulated by suboptimal concentrations of the mitogens. In conclusion, the impaired growth of PBMC in serum-free cultures from uremic patients was enhanced, however, not normalized, by external addition of T3, inhibition of prostaglandin E2 synthesis, and addition of interleukin 2.

ISSN:0250-8095    

DOI:10.1159/000168437

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Forty-three children with crescentic glomerulonephritis (GN), having large crescents in more than 50% of the glomeruli, were observed during a period of 22 years. There were 17 boys and 26 girls between the ages of 3.5 and 14 years (mean 8.7 ± 2.6). Thirty-one patients (72%) presented with acute nephritic features and increasing renal insufficiency (rapidly progressive GN) whereas 12 had an insidious onset with nephrotic syndrome, or rarely with nonspecific symptoms. Eleven patients had evidence of poststreptococcal GN and 6 an underlying systemic disorder. Renal biopsy showed large crescents in > 80% of the glomeruli in 38 cases (100% in 28) which were predominantly fibrocel-lular or fibrous in 80% of the patients. Nineteen patients (44%) were treated with prednisolone, cyclophosphamide and dipyridamole; in addition, 8 were also given anticoagulants. Six patients received pulse doses of corticosteroids. In 23 patients, there was inexorable progression of renal failure, 14 showed partial improvement but subsequently had varying degrees of renal insufficiency and in 6, there was recovery of renal function with normal levels of serum creatinine. Of the latter, 4 had received immunosuppressive anticoagulant therapy and 2 only supportive care. Of 11 patients with poststreptococcal crescentic GN, 7 progressed to end-stage renal disease and 2 developed chronic renal insufficiency. Our findings confirm the poor outcome of crescentic GN in children, irrespective of the underlying etiology. In a small proportion of cases, the disorder may have an insidious onset and a slowly progressive course, but an equally grave prognosis

ISSN:0250-8095    

DOI:10.1159/000168438

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

The quality of life of 12 hemodialysis (HD) patients was assessed in a prospective, blinded, cross-over fashion before treatment with recombinant human erythropoietin (r-HuEPO) and at two different levels of hemoglobin (Hb, 9 and 12 g/dl) by means of an interviewer-based questionnaire, the sickness impact profile (SIP). Patients were matched into two groups with no significant difference for age, weight, Hb (6.3 ± 0.5, x ± SEM, group A, vs. 6.4 ± 0.9 group B), length of hemodialysis or number of years of prior transplantation. SIP was assessed prior to treatment, after reaching the first target Hb (Hb 9 g/dl group A, 12 g/dl group B), after 4 months at that target Hb and after 4 months at the alternative target Hb for each group. For all patients, there was a highly significant improvement in quality of life as assessed by lower SIP scores between the initial and second assessments. This was evident for the physical(8.9 ± 1.4 vs. 2.8 ± 1.0; p < 0.001) and psychosocial (14.9 ± 3.9vs. 4.4 ± l.l;p < 0.01) dimensions. Total scores (16.3 ± 2.4 vs. 5.7 ± 0.9; p < 0.001) showed similar changes, reflecting significant improvement in 10 of 12 possible categories between the first two assessments (p < 0.05 to p < 0.001). Improved scores were maintained but did not change appreciably after the 2nd assessment. There was no significant difference in any score (category, dimensional or total) obtained after 4 months at Hb 9 g/dl compared to those after the same period at Hb 12 g/dl. There was also no significant difference in scores between the two groups at any assessment. Results support an enhanced quality of life from r-HuEPO in HD patients which might not improve beyond a certain level of hemoglobin, as most benefits were evident at a Hb o

ISSN:0250-8095    

DOI:10.1159/000168439

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

From 1983 to 1990, 32 patients with hemorrhagic fever with renal syndrome (HFRS) were admitted to our hospital. The diagnosis was confirmed by high IgM type titers of antibodies to Hantaan virus. All patients presented with serum and urine abnormalities suggesting renal involvement. Serum creatinine was elevated and ranged between 1.8 and 14.3 mg/dl. Proteinuria ranged between 0.5 and 6.4 g/24 h. Seven patients died due to shock or hemorrhage, while 6 patients were supported by hemodialysis or peritoneal dialysis. Five of them had a complete recovery. Two patients were discharged with some degree of renal impairment which remained stable 12-15 months later. Kidney biopsy in the first patient performed 1 year after his discharge revealed some degree of interstitial fibrosis and tubular atrophy as well as an area with ischemic and sclerosed glomeruli. We conclude that HFRS in Greece is a severe disease with a high mortality rate. The disease may cause chronic renal failure in a limited number of patients.

ISSN:0250-8095    

DOI:10.1159/000168440

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

We studied the effect of repeated heavy physical activity on glomerular filtration rate (GFR) in healthy, uninephrectomized and experimentally uremic mice. Exercise consisted of running uphill in the inner surface of a rotating cylinder in ideal environmental temperature. In the control groups, no extra physical activity was imposed. In sham-operated and nephrectomized mice, GFR rose significantly following training. By contrast, GFR did not change significantly in the exercised mice with experimental renal failure 24 h following the last exercise session. During the same period, no significant change was observed in GFR of any of the control groups. Following training in each experimental group, mean aortic blood pressure as well as fractional kidney weight (kidney weight/body weight) were not different from the respective controls. Our results indicate that the capacity to augment GFR by physical training is dependent upon the amount of remaining functional renal tissue.

ISSN:0250-8095    

DOI:10.1159/000168441

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Glucose-induced insulin secretion is impaired in chronic renal failure (CRF), and this abnormality is due to the elevation of cytosolic calcium [Ca2+]i and other derangements in pancreatic islet metabolism. Verapamil given to rats from day 1 of CRF prevented the rise in [Ca2+]i of islets and the impairment in insulin secretion. However, it is not known whether verapamil can reverse the abnormalities of islet function and metabolism in animals with preexisting renal failure. Such a documentation has important clinical implications for the treatment of carbohydrate intolerance in patients with CRF. The present study examined this question. After 6 weeks of CRF, rats were randomized into two subgroups and maintained for additional 6 weeks. One subgroup received intraperitoneal injections of verapamil (0.1 µg/kg body weight twice daily) and the other received vehicle only. At the time of randomization, there were no significant differences between the two subgroups in their body weight, plasma levels of calcium, phosphorus and creatinine, serum parathyroid hormone and creatinine clearance. Similarly, at the time of sacrifice (12 weeks), there were no significant differences in these parameters except for a modestly lower plasma level of creatinine and modestly higher creatinine clearance. The treatment of rats with preexisting CRF with verapamil (CRF-V) normalized glucose intolerance and reversed to normal or near normal the derangements in islet metabolism and function including [Ca2+]i (normal: 85 ± 1.5 nM; CRF: 135 ± 1.9 nM; CRF-V: 94 ± 2.9 nM), basal levels of ATP content (normal: 12.8 ± 1.11 pmol/islet; CRF: 7.8 ± 0.31 pmol/islet; CRF-V: 10.6 + 0.60 pmol/islet) and stimulated ATP content and ATP/ADP ratio, glucose-induced insulin secretion (normal: 2,380 ± 116.9 pg/islet-28 min; CRF: 842 ± 75.0 pg/islet-28 min; CRF-V: 1,929 ± 65.3 pg/islet-28 min) and the glucose-induced calcium signal (normal: 167 ± 11.1 nM;CRF: 136 ± 12.1 nM; CRF-V: 180 ± 13.5 nM). Vmax of Ca2+ ATPase and Na+-K+ ATPase of islets of CRF were also markedly and significantly improved by treatment with verapamil. These data demonstrate that the calcium channel blocker verapamil can reverse the functional and metabolic derangements of pancreatic islets that occur in CRF. The results provide the experimental basis for the clinical use of calcium channel blockers in the treatment of carbohydrate intoler

ISSN:0250-8095    

DOI:10.1159/000168442

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

We describe the first reported case of resistance to human recombinant erythropoietin (rhEPO) treatment caused by vitamin B12 deficiency in a chronic hemodialysis patient. Despite a normal B12 level before rhEPO treatment, resistant anemia together with a low B12 level and a megaloblastic bone marrow developed after only 8 months of rhEPO. There was a rapid reticulocyte response to B12 supplements, and transfusion requirements dropped from 2 units monthly to nothing. Atrophic gastritis was diagnosed through endoscopy and biopsy. Because of the fall in B12 level after 8 months of rhEPO treatment, we analyzed the results of routinely measured B12 levels in 30 hemodialysis patients treated with rhEPO, and found the mean B12 levels to be unchanged before and after rhEPO treatment. Although we found screening for B12 deficiency of little benefit, any patient with rhEPO resistance should have B12 levels tested, given the potentially serious extrahematological effects of B12 deficiency.

ISSN:0250-8095    

DOI:10.1159/000168443

出版商:S. Karger AG    

年代:1992

数据来源: Karger

摘要:

Hemolytic-uremic syndrome following quinine ingestion is a newly described phenomenon, with just two previous descriptions of 4 cases in the literature. We describe a 5th case. The reaction may be mediated by the presence of antibodies reactive against platelets in the presence of quinine. Treatment has included use of plasma exchange, prednisone, aspirin, and dipyridamole. The patients have all regained some degree of renal function. However, it is unclear whether pharmacological treatment or spontaneous resolution is responsible for the improvement. Quinine-associated hemolytic-uremic syndrome probably occurs more often than is recognized. It is important to recognize this reaction when it occurs and to avoid further quinine exposure, since the reaction seems to be recurrent.

ISSN:0250-8095    

DOI:10.1159/000168444

出版商:S. Karger AG    

年代:1992

数据来源: Karger