Nephrotic Syndrome: From Toddlers to Twenties

Lewis, MalcolmA.; Davis, Naomi; Baildom, E. M.; Houston, I. B.; Postlethwaite, RobertJ.

doi:10.1016/s0140-6736(89)91266-x

Cited by 90 publications

(58 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The preponderance of males in our study differs from the male:female ratio of 1.5 in children with first episode nephrotic syndrome, but it is in concert with a higher relapse frequency found in boys [1,36,37].…”

Section: Discussioncontrasting

confidence: 79%

Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

et al. 2008

View full text Add to dashboard Cite

We performed a multi-centre randomized controlled trial to compare the efficacy of mycophenolate mofetil (MMF) to that of cyclosporine A (CsA) in treating children with frequently relapsing nephrotic syndrome and biopsy-proven minimal change disease. Of the 31 randomized initially selected patients, seven were excluded. The remaining 24 children received either MMF 1200 mg/m 2 per day (n=12) or CsA 4-5 mg/kg per day (n=12) during a 12-month period. Of the 12 patients in the MMF group, two discontinued the study medication. Evaluation of the changes from the baseline glomerular filtration rate showed an overall significant difference in favour of MMF over the treatment period (p=0.03). Seven of the 12 patients in the MMF group and 11 of the 12 patients in the CsA group remained in complete remission during the entire study period. Relapse rate in the MMF group was 0.83/year compared to 0.08/year in the CsA group (p=0.08). None of the patients reported diarrhea. Pharmacokinetic profiles of mycophenolic acid were performed in seven patients. The patient with the lowest area under the curve had three relapses within 6 months. In children with frequently relapsing minimal change nephrotic syndrome, MMF has a favourable side effect profile compared to CsA; however, there is a tendency towards a higher relapse risk in patients treated with MMF.

show abstract

Section: Discussioncontrasting

confidence: 79%

Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

et al. 2008

View full text Add to dashboard Cite

show abstract

“…In the few studies that observed an effect of sex on the clinical course of NS, males were at a disadvantage. 20,22 It would be interesting to further explore whether boys and girls benefit from different treatment regimens in studies with larger sample sizes. We found no effect of age at onset.…”

Section: Discussionmentioning

confidence: 99%

Extending Prednisolone Treatment Does Not Reduce Relapses in Childhood Nephrotic Syndrome

Teeninga

Holthe

Rijswijk

et al. 2013

Journal of the American Society of Nephrology

120

View full text Add to dashboard Cite

Prolonged prednisolone treatment for the initial episode of childhood nephrotic syndrome may reduce relapse rate, but whether this results from the increased duration of treatment or a higher cumulative dose remains unclear. We conducted a randomized, double-blind, placebo-controlled trial in 69 hospitals in The Netherlands. We randomly assigned 150 children (9 months to 17 years) presenting with nephrotic syndrome to either 3 months of prednisolone followed by 3 months of placebo (n=74) or 6 months of prednisolone (n=76), and median follow-up was 47 months. Both groups received equal cumulative doses of prednisolone (approximately 3360 mg/m 2 ). Among the 126 children who started trial medication, relapses occurred in 48 (77%) of 62 patients who received 3 months of prednisolone and 51 (80%) of 64 patients who received 6 months of prednisolone. Frequent relapses, according to international criteria, occurred with similar frequency between groups as well (45% versus 50%). In addition, there were no statistically significant differences between groups with respect to the eventual initiation of prednisolone maintenance and/or other immunosuppressive therapy (50% versus 59%), steroid dependence, or adverse effects. In conclusion, in this trial, extending initial prednisolone treatment from 3 to 6 months without increasing cumulative dose did not benefit clinical outcome in children with nephrotic syndrome. Previous findings indicating that prolonged treatment regimens reduce relapses most likely resulted from increased cumulative dose rather than the treatment duration.

show abstract

“…Two studies in the 1980s conducted in the pre-CsA era revealed a percentage of 5.5 and 26% of persistent relapses of biopsy-proven MCNS in adulthood (3,4).…”

Section: Discussionmentioning

confidence: 99%

“…If relapses persist afterward, then treatment with cyclosporin A (CsA) is given, which allows tapering of the steroid dosage but frequently leads to CsA dependence, necessitating long-term immunosuppressive treatment. The percentage of childhood MCNS that relapses in adulthood varied from 10 to 40% in the recent studies (2)(3)(4). There are few data about the long-term prognosis in this group of patients, especially concerning possible adverse effects of the immunosuppressive medication.…”

mentioning

confidence: 99%

Long-Term Outcome of Biopsy-Proven, Frequently Relapsing Minimal-Change Nephrotic Syndrome in Children

Kyrieleis¹,

Löwik²,

Pronk³

et al. 2009

Clinical Journal of the American Society of Nephrology

124

113

View full text Add to dashboard Cite

Background and objectives: Frequently relapsing and steroid-dependent minimal-change nephrotic syndrome (MCNS) that originates in childhood can persist after puberty in >20% of patients. These patients require immunosuppressive treatment during several decades of their life. We examined long-term adverse effects of persistent nephrotic syndrome and immunosuppressive medications, focusing on renal function, growth, obesity, osteoporosis, hypertension, ocular complications, and fertility in adult patients with biopsy-proven childhood-onset MCNS. Molecular analysis was performed to evaluate a possible association of a complicated course of MCNS with podocyte gene mutations.Design, setting, participants, & measurements: We performed a prospective clinical examination of 15 adult patients that included serum and urine analysis; dual-energy x-ray absorptiometry; ophthalmologic examination; semen examination; and molecular analysis of NPHS1, NPHS2, CD2AP, and ACTN4 genes.Results: All patients had normal GFR. Most frequent long-term complications were hypertension (in seven of 15 patients) and osteoporosis in one third of patients. Oligozoospermia was found in one patient, reduced sperm motility in four of eight patients, and teratozoospermia in six of eight patients. Ophthalmologic examination revealed myopia in 10 of 15 patients and cataract in three of 15 patients.Conclusions: Children with MCNS that persists after puberty are at risk for complications such as osteoporosis, hypertension, cataract, and sperm abnormalities. Our study underscores a need for more effective and less toxic therapies for relapsing MCNS.

show abstract

Nephrotic Syndrome: From Toddlers to Twenties

Cited by 90 publications

References 9 publications

Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

Extending Prednisolone Treatment Does Not Reduce Relapses in Childhood Nephrotic Syndrome

Long-Term Outcome of Biopsy-Proven, Frequently Relapsing Minimal-Change Nephrotic Syndrome in Children

Contact Info

Product

Resources

About