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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 6 (1992), S. 503-505 
    ISSN: 1432-198X
    Keywords: Nephrotic syndrome ; Methylprednisolone ; Alkylating agent
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Treatment with a combination of pulse methylprednisolone (MP) and an alkylating agent has been reported to induce long-term remission of proteinuria in patients with steroid-resistant nephrotic syndrome (SRNS). We have treated 13 patients with SRNS with a course of pulse MP. There were 8 black patients and 5 white; 10 had a biopsy diagnosis of focal segmental glomerulosclerosis (FSGS) and 3 nil lesion. Initially 5 patients responded and 2 partially responded. Of the responding patients, 5 relapsed while treated with alternate-week MP therapy. Of these relapsing patients, 3 received a second course of MP plus chlorambucil; 2 responded. The patients were observed for a mean of 47 months (range 4–64 months). When last seen only the 3 patients with a biopsy diagnosis of nil lesion were protein free. There were no complications of steroid therapy. Six patients currently have end-stage renal disease and 2 have renal insufficiency. All of the 6 patients with no response to treatment were black. These data suggest that a course of pulse MP therapy alone induces short-term remission of the nephrotic syndrome in some white patients with FSGS, but in almost no blacks. Patients who relapse may respond to retreatment, but addition of an alkylating agent does not appear to induce longterm remission in patients with FSGS.
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  • 2
    ISSN: 1432-198X
    Keywords: IgA nephropathy ; Treatment ; Prednisone
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have previously reported our experience with the use of alternate-day prednisone in the treatment of 6 patients with IgA nephropathy who have clinical or pathological risk factors for disease progression. We have now treated a total of 13 patients and followed them from 4 to 10 years. Patients received an alternate-morning dose of prednisone for 2–4 years. Dosage began at 60 mg/m2 for 3 month, was reduced to 30 mg/m2 by 1 year and 15 mg/m2 by 2 years. At last observation, urinary protein excretion was normal in 12 patients and no patient had hematuria. Twelve patients had normal estimated glomerular filtration rate (GFR) and one had renal insufficiency (GFR=38 ml/min per 1.73 m2). A renal biopsy was performed in 11 patients after 2 years of treatment. Activity score decreased from 5.2 to 4.3 (P=0.03) and chronicity score increased from 2.2 to 2.8 (P=0.12). There were no complications of treatment. When compared with a historical group, the treated patients had a significant improvement in urinalysis (P〈0.00001) and preservation of normal GFR (P=0.03). We conclude that alternate-day prednisone therapy may benefit patients with IgA nephropathy. A large prospective controlled trial is needed.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-198X
    Keywords: Recombinant human growth hormone ; Chronic renal insufficiency ; Children under 2.5 years
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Children with chronic renal insufficiency (CRI) secondary to congenital structural abnormalities frequently have significant growth retardation by 2 years of age. In a multicenter placebo-controlled study of the use of recombinant human growth hormone (rhGH), 30 of 125 (24%) participants were〈2.5 years of age at enrollment. Since the treatment arms of the study were balanced for age at randomization, data for these patients were examined for efficacy and safety. During the first 2 years of the study, approximately two-thirds of the patients (n=19) received rhGH 0.05 mg/kg per day subcutaneously and one-third (n=11) received placebo injections. At entry into the study, the mean (± SD) calculated creatinine clearance was 29.2±14.3 (range 12.0−63.7) ml/min per 1.73 m2 in the rhGH-treated group and 23.3±15.1 (range 8.0−59.4) ml/min per 1.73 m2 in the placebo-treated group. The 1st year growth rate was 14.1±2.6 cm/year for the rhGH-treated group and 9.3±1.5 cm/year in the placebo-treated group (P〈0.00005). During the 2nd year of the study, the growth rate was 8.6±1.2 cm/year in the rhGH-treated group compared with 6.9±1.0 in the placebo groupP=0.025). The Δ height standard deviation score was +2.0±0.7 for the rhGH-treated group compared with −0.2±1.1 in the placebo-treated group (P〈0.00005) during the 2 years of the study. Minor adverse events occurred with similar frequency in both groups. These data suggest that rhGH is efficacious and safe in children with CRI under age 2.5 years. rhGH therapy may correct significant loss of growth at this age when used in conjunction with optimal medical management.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-198X
    Keywords: Growth hormone ; Transplantation ; Mixed leukocyte culture
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Recombinant human growth hormone (rhGH) improves growth in children after renal transplantation, but may be associated with augmented immune responses. We previously demonstrated that rhGH augments proliferative and cytotoxic responses and interferon-gamma (IFN-γ) mRNA expression during a mixed leukocyte culture (MLC). In this study, we evaluated 12 pediatric patients after receiving a renal allograft from one of their parents. Peripheral blood mononuclear cells (PBMC) were isolated from patients and cultured with either donor or unrelated third-party PBMC in an MLC. Patients developed significant donor-specific hyporesponsiveness (DSH), however, no correlation was seen between the amount of DSH and graft function. Of the 12 patients, 2 developed augmented responses in the presence of rhGH. rhGH augments proliferation, cytotoxicity, and IFN-γ expression during an MLC. Some patients develop increased responses to donorspecific alloantigens after renal transplantation. Further study is needed to better determine the significance of this finding.
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 1 (1987), S. 150-153 
    ISSN: 1432-198X
    Keywords: Infants ; Nutritional management ; CAPD ; Transplantation ; Neurological development ; Growth
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Nine infants, who presented with renal failure within the first 3 months of life, were treated with continuous ambulatory peritoneal dialysis (CAPD). Seven infants survived to an age of 12–15 months, when they received transplants. Two patients died while on CAPD. Six infants are alive with a functioning renal allograft, at an average age of 35.5 months and an average of 22 months post-transplant. Neurological development is normal in four of the six infants tested. The mean current height of the six transplant recipients is just below 2 SD from the mean.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 1 (1987), S. 180-182 
    ISSN: 1432-198X
    Keywords: Cyclosporine A ; Focal sclerosis ; Nephrotic syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Six patients with biopsy-documented focal segmental glomerulosclerosis were treated with cyclosporine A (CyA) a new and potent T-cell suppressant. Dosage was adjusted to maintain trough levels between 100 and 300 ng/ml (whole blood radioimmunoassay). Patients were treated for 6 weeks or until unacceptable drug toxicity occurred. In one patient proteinuria was significantly reduced. This same individual was the only patient treated within 6 months of diagnosis. Therapy was discontinued in two patients at week 4, one because of worsening renal failure and one because of hypertension. These complications occurred despite trough CyA blood levels of less than 300 ng/ml. The results suggest that further and controlled studies of CyA are warranted in the therapy of nephrotic syndrome associated with focal segmental glomerulosclerosis, especially when used early in the course of disease. The data also suggest enhanced toxicity of CyA in patients with active nephrotic syndrome.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-198X
    Keywords: Key words: Growth hormone ; Transplantation ; Mixed leukocyte culture
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Recombinant human growth hormone (rhGH) improves growth in children after renal transplantation, but may be associated with augmented immune responses. We previously demonstrated that rhGH augments proliferative and cytotoxic responses and interferon-gamma (IFN-γ) mRNA expression during a mixed leukocyte culture (MLC). In this study, we evaluated 12 pediatric patients after receiving a renal allograft from one of their parents. Peripheral blood mononuclear cells (PBMC) were isolated from patients and cultured with either donor or unrelated third-party PBMC in an MLC. Patients developed significant donor-specific hyporesponsiveness (DSH), however, no correlation was seen between the amount of DSH and graft function. Of the 12 patients, 2 developed augmented responses in the presence of rhGH. rhGH augments proliferation, cytotoxicity, and IFN-γ expression during an MLC. Some patients develop increased responses to donor-specific alloantigens after renal transplantation. Further study is needed to better determine the significance of this finding.
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 7 (1993), S. 505-505 
    ISSN: 1432-198X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 10 (1996), S. 422-434 
    ISSN: 1432-198X
    Keywords: Key words: North American Pediatric Renal Transplant Cooperative Study   ;   Transplantation   ;   Dialysis   ;   Growth
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) is a research effort that was organized and initiated in 1987. The following manuscript is the 1994 NAPRTCS annual report which has summarized data that has been voluntarily contributed by 83 centers. The report includes data on 3,183 patients who have undergone a total of 3,445 renal transplants between 1 January 1987 and 18 February 1994 when the data set was closed. The report also contains data on 1,611 independent courses of dialysis which were initiated between 1 January 1992 and 18 February 1994. This report is meant to update the previous NAPRTCS annual reports as well as demonstrate how the NAPRTCS database has changed clinical practice since its inception. There have been 855 graft failures among the 3,438 transplants. Due to the maturing of the database, chronic rejection now accounts for 34% of graft failures which have occurred over the last year. Graft failure was increased in recipients if the recipients were 〈2 years of age, of the black race, or had received five or more prior transfusions. Early treatment with antithymocyte globulin/antilymphocyte globulin/OKT3 was associated with increased graft survival. Catch-up growth post transplant was only seen in the youngest patients (〈6 years of age). Those patients 〉6 years did not have catch-up growth post transplant. Overall graft survival has improved markedly since the inception of this study. The dialysis database is just maturing and the data confirm that growth on dialysis continues to be very poor. The 1994 annual report of NAPRTCS extends previous findings of this valuable database. It is gratifying to know that early findings of NAPRTCS have led to changes in therapy which have led to improvement in the care of these very special children.
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  • 10
    ISSN: 1432-198X
    Keywords: Key words: Nephrotic syndrome ; Focal segmental glomerulosclerosis ; Treatment ; Prednisone ; Cyclosporine A
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Patients with steroid-resistant focal and segmental glomerulosclerosis (FSGS) have a poor prognosis but may benefit from high-dose methylprednisolone or cyclosporine A therapy. Ten patients were treated with a protocol of methylprednisolone infusions for 8 weeks followed by a combination of cyclosporine A and alternate-day prednisone for maintenance of remission for 2 weeks. Eight of ten patients remitted the nephrotic syndrome within 8 weeks of beginning treatment. One patient remitted edema but remained proteinuric, and one did not respond. After observation for 12 – 24 months, seven patients maintained remission with normal glomerular filtration rate. One non-responder had renal insufficiency and one patient had secondary non-response and end-stage renal disease. No patients developed hypertension. One patient had the diagnosis of Hodgkin disease made after 10 months of therapy. Follow-up renal biopsy in four patients showed no evidence of progressive interstitial disease. There were no other major side effects. Steroid-resistant FSGS may be successfully treated with the described protocol. Additional studies will be needed to determine if this approach prevents progression of renal disease.
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