Childhood Idiopathic Nephrotic Syndrome: Does the Initial Steroid Treatment Modify the Outcome? A Multicentre, Prospective Cohort Study

Background: A great majority of children with idiopathic nephrotic syndrome will relapse after successful treatment of the initial episode. The possibility that different steroid dosing regimens at onset, adjusted for risk factors, can reduce the rate of relapse represents an interesting option to investigate.Objectives: To evaluate the effect of the initial steroid regimen, adjusted for time to remission (TTR), on the frequency of relapses and steroid dependence, and to verify the influence of prognostic factors on disease course.Methods: A multicentre, prospective, cohort study. Children with nephrotic syndrome, with TTR ≤ 10 days (Group A), were given a 20-week prednisone regimen (2,828 mg/m2) and those with a TTR >10 days, a 22-week regimen (3,668 mg/m2) (Group B). Previously published retrospective data from the same centers were also evaluated. Main outcomes were: relapse rate, number of frequent relapsers + steroid dependent children and total prednisone dose after induction.Results: 143 children were enrolled. Rate of relapsed subjects (77 vs. 79%) and frequent relapsers + steroid dependent subjects (40 vs. 53%) did not differ between Groups A and B, or between the retrospective and prospective cohorts. The cumulative prednisone dose taken after the induction treatment was similar in both groups and in the retrospective and prospective cohorts. TTR was not associated with relapse risk. Age at onset and total serum protein were significantly lower in relapsing patients. At ROC analysis, the best cut-off was 5.3 years for age at onset and 4.2 g/dL for total serum protein. According to these cut-offs, older children with higher total serum protein had a higher relapse free survival rate (58%) than younger children with lower total serum protein (17%).Conclusions: TTR was not found to be a prognostic factor of relapse; because of this, different steroid regimens, adjusted for TTR, did not modify the relapse rate in any relevant measure. Conversely, younger age and low total serum protein were independent predictors of relapse risk, however this outcome was not modified by higher prednisone regimens.Clinical Trial Registration:https://www.ClinicalTrials.gov/, identifier: NCT01386957 (www.nefrokid.it).

TO STUDY THE PREVALENCE OF VITAMIN D3 DEFICIENCY IN NEPHROTIC SYNDROME

Background: Cross-sectional studies of children with Nephrotic Syndrome have shown deciency of Vitamin D due to its loss in urine, bound to Vitamin D binding protein and through various other mechanisms. Therefore, this study was taken up to study the prevalence of Vitamin D deciency in Children with Nephrotic syndrome. A prospective study of children with Methods: Nephrotic syndrome at the time of hospital admission to Department of Paediatrics, MMIMSR, Mullana, Ambala, was undertaken. 2ml venous blood of the child was collected for assessment of 25(OH)D. A total of 50 chil Results: dren were enrolled in this study. Mean age was 6.4 years. Male to female ratio was 16:9. Vitamin D levels < 20ng/ml were seen in 76% children in the study population; with 28% patients having Vitamin D deciency while 48% had insufciency. Also, frequent relapsers and children with initial episode had lower levels of vitamin D as compared to infrequent relapsers. Children with Nephrotic Syndrome should have routine measurement of Conclusion: 25(OH)D and they will benet from vitamin D supplementation, if having decient/ insufcient levels. Individualized Vitamin D treatment strategy can be devised for each child.

Study of risk factors for relapse in frequently versus infrequently relapsing nephrotic syndrome in 1-18 year age group: a combined prospective retrospective cohort analytical observational study

Background: Early prediction and prevention of risk factors is the key to successful management of childhood nephrotic syndrome. This study was carried out to find the risk factors of relapse which will help in early prediction and reduce the risk of relapse in childhood nephrotic syndrome.Methods: It was a combined prospective-retrospective cohort analytical observational study of duration 18 months with sample size of 80 patients of age group 1-18 years who fulfilled the inclusion and exclusion criteria. The variables taken into account for the present study were demographic and disease related.Results: In the present study, 67.7% of patients with ≤6 year of age at first onset were frequent relapsers while 60% of patients with >6 year of age at first onset were infrequent relapsers. A 77.1% of patients belonging to lower socioeconomic strata and 60% of patients belonging to lower-middle socioeconomic strata were frequent relapsers. 100% of patients having ≥ 4 relapses within the 1st year after diagnosis were frequent relapsers while 73.2% of patients having ≤3 relapses within the same period were infrequent relapsers. In present study, out of 38 patients who had received 8 weeks of steroid therapy 92.1% were frequently relapsing while out of 42 patients who received 12 weeks of steroid therapy 64.3% were found to be infrequently relapsing.Conclusions: Younger age at first onset, higher number of relapses in first year and lower socio-economic strata is associated with frequently relapsing nephrotic syndrome. Longer duration of steroid therapy (12 weeks) lowers the chance of frequent relapses.

Cyclosporin-A Treatment in Steroid Nonresponsive Nephrotic Syndrome

Fifteen patients with steroid nonresponsive nephrotic syndrome (NS) aged 4-16 years received oral cyclosporin-A (CyA) for 12 weeks. Nine of the patients were boys. Out of the 15 patients, 7 were frequent relapsers, 3 were steroid dependents, 4 were steroid resistants and one with toxic steroid. After 12 weeks of CyA treatment;, 6 patients showed complete remission, 7 showed partial remission, and 2 patients did not respond at all. Side effects observed were slight renal function impairment, gingival hyperplasia, and a hump on the breast; all disappeared gradually after stopping CyA. Patients with total remission experienced relapse 2 to 12 months after discontinuation of CyA, while patients with partial remission experienced relapse 2 weeks to 3 months after CyA was discontinued A tentative conclusion can be drawn that CyA is a good alternative in the treatment of idiopathic NS, especially in steroid dependent patients who are at risk of developing steroid toxicity. CyA represent a major advance in the treatment selected SN patients who have failed with the conventional modes of therapy.

Minimal Change Nephropathy in the West of Scotland: A Review of Sixty-Four Patients

Sixty-four patients with a histological diagnosis of minimal change nephropathy have been followed for a median of 110 months. Patients transferred from paediatric units (11%) had a worse prognosis in that all became frequent relapsers. Patients who relapsed within three months or who went on to become frequent relapsers had a higher 24 hour urine protein excretion at presentation than patients who did not relapse. After fifteen months of remission relapse was rare; 97% of those who relapsed did so within 36 months. Patients who have been off steroids and proteinuria free for 36 months might therefore be considered cured.

CYCLOPHOSPHAMIDE IN THE TREATMENT OF THE NEPHROTIC SYNDROME IN CHILDHOOD

To what extent, if any, adrenocortical steroid drugs have changed the ultimate outcome of children with the nephrotic syndrome is, and probably will remain, a moot question. This mode of therapy, however, has modified the natural course of this syndrome so dramatically that it has resulted in a reclassification of patients according to their response to steroid treatment. Two categories of problem patients thus have emerged: those who fail to attain remission (nonresponders), and those who do respond but relapse frequently, becoming steroid-dependent and steroid-toxic (frequent relapsers). A new hope for these patients has arisen from the increasing body of anecdotal evidence suggesting that a favorable effect can be obtained by treatment with various antimetabolites and alkylating agents.