Early identification of steroid dependency in Japanese children with steroid-sensitive nephrotic syndrome undergoing short-term initial steroid therapy

Introduction: Nephrotic syndrome is a clinical picture characterized by severe proteinuria, hypoalbuminemia, edema and hypercholesterolemia. A retrospective study was carried out in order to describe disease pattern in newly diagnosed nephrotic syndrome of children admitted to Tripoli children hospital during the year 2014.Methods: The medical data of 56 patients aged between 1 year and 11 years diagnosed with idiopathic nephrotic syndrome were analysed using SPSS software. The data included gender differences, sensitivity to steroid therapy, relapses during six months of follow up and the effect of variable factors such as family history, hypertension, hematuria, serum urea on the degree of relapse.Results: Out of 56 patients with newly diagnosed nephrotic syndrome (NS), 60.7% were boys and 39.3% were girls, with a mean age 4.2±2.2 years. Age was related significantly to the response to steroid therapy, where 79.5% of patients aged between 2-8 years (group 1) had steroid sensitive nephrotic syndrome (SSNS) compared with only 41.7% of patients aged less than 2 years or more than 8 years (group 2) (P<0.001). Although girls relapsed more than boys (70.5% versus 57.1%) during six months of therapy, this difference was not statistically significant. Similarly, no other factors measured such as family history of NS, hypertension, hematuria, serum complement and urea had any effect on the percentage of relapse in patients with newly diagnosed NS. Conclusion: NS is one of the commonest reasons for admission to nephrology ward. It is more common in boys than girls. The age at presentation related significantly to the response to steroidal therapy. Regarding relapses, girls seems to relapse more frequent than boys and relapses was seen more in age group 1 than group 2, however, these differences were not significant. Other factors studied seems to have no effect on the relapse rate of children with newly diagnosed NS.Key-words: Idiopathic nephrotic syndrome, Steroid sensitive nephrotic syndrome, Steroid resistant nephrotic syndrome, Proteinuria.

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Steroid sensitive nephrotic syndrome in children: Frequency of relapse during the first twelve months after completion of six months steroid therapy.

The Professional Medical Journal ◽

10.29309/tpmj/2020.27.03.3571 ◽

2020 ◽

Vol 27 (03) ◽

pp. 558-562

Author(s):

Irum Jabeen ◽

Asim Khurshid ◽

Tariq Aziz

Keyword(s):

Nephrotic Syndrome ◽

Steroid Therapy ◽

Case Series ◽

Outcome Variable ◽

First Episode ◽

Case Series Study ◽

Steroid Sensitive Nephrotic Syndrome ◽

Steroid Sensitive ◽

Set Up ◽

Nephrotic Children

Objectives: Nephrotic syndrome (NS) is described as the existence of nephrotic-range proteinuria along with edema, hyperlipidemia and hypoalbuminemia. NS is estimated to be 15 time more frequent in children as compared to adults. Relapse is a major problem while managing nephrotic children. This study was aimed to find out the frequency of relapse in children with first episode of steroid sensitive nephrotic syndrome (SSNS) during the first 12 months, after completion of 6 months steroid therapy. Study Design: Descriptive case series study. Setting: Department of Paediatric Nephrology, The Children’s Hospital & the Institute of Child Health, Multan. Period: From February 27, 2018 to February 27, 2019. Material and Methods: A total of 55 children, aged 1 to 10 years, diagnosed with SSNS, 1st presentation of NS (based on history) and who successfully completed 6 months steroid therapy, were enrolled. They were taught to check proteinuria at home by dipstick method and enter daily results on the follow up card provided from the Nephrology department of the hospital. The outcome variable, that is relapse, was noted on the Proforma. Results: Amongst 55 children, gender distribution showed 38 (69.1%) male and 17 (30.9%) females. Children with body weight <20 kg were 33 (60%) and those having ≥20kg were 22 (40%). Patients with age <6 years were 54.5% and patients with age ≥6 years were 45.5%. Mean age was 5.93± 3.36 years. Frequency of relapse was noted to be 78.2% and patients who did not relapse within 1 year of completion of treatment were 21.8%. Conclusion: Nephrotic syndrome is a common presentation of childhood renal problems and is major cause of morbidity in our set up. Relapses are frequently associated with SSNS and most of the patients relapse within 1 year of completion of treatment. Relapses are more common in male children as compared to female children.

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Study protocol: high-dose mizoribine with prednisolone therapy in short-term relapsing steroid-sensitive nephrotic syndrome to prevent frequent relapse (JSKDC05 trial)

BMC Nephrology ◽

10.1186/s12882-018-1033-z ◽

2018 ◽

Vol 19 (1) ◽

Cited By ~ 2

Author(s):

Taketsugu Hama ◽

◽

Koichi Nakanishi ◽

Kenji Ishikura ◽

Shuichi Ito ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Study Protocol ◽

High Dose ◽

Short Term ◽

Prednisolone Therapy ◽

Steroid Sensitive Nephrotic Syndrome ◽

Steroid Sensitive ◽

Frequent Relapse

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Analysis of Predictors of Relapse in Children with Steroid Sensitive Nephrotic Syndrome

Journal of Nepal Paediatric Society ◽

10.3126/jnps.v37i1.17432 ◽

2017 ◽

Vol 37 (1) ◽

pp. 1-4

Author(s):

Mukesh Bhatta ◽

Gauri Shankar Shah ◽

Om Prakash Mishra

Keyword(s):

Nephrotic Syndrome ◽

Steroid Therapy ◽

Age Of Onset ◽

Total Leukocyte Count ◽

Late Response ◽

Female Ratio ◽

Younger Age ◽

Steroid Sensitive Nephrotic Syndrome ◽

Steroid Sensitive ◽

One Year

Introduction: Children with idiopathic nephrotic syndrome (INS) are steroid responsive but have relapses in subsequent non-treatment period. The objective of the present study was to analyze the factors which could predict relapses in these children.Material and Methods: Forty patients of INS aged 1-14 years of both gender were enrolled over one year period and followed for six months after treatment of initial episode of Nephrotic Syndrome.Results: The median age of children was 4.5 years and male to female ratio 1.9:1. There were 24(60%) relapses and 16(40%) non-relapses. The relapses had significantly higher mean total leukocyte count, serum urea, potassium and cholesterol than non-relapses. It was also observed that the median age of onset in relapses was significantly lower than non-relapses (p<0.001). Also, the median time to response to steroid therapy was longer in relapses than non-relapses (p<0.001). Children who relapsed had infections at the time of relapse.Conclusion: Thus, onset of disease in younger age group, late response to steroid therapy and presence of infections were found to be associated with relapses in these children.

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Levamisole in Children with Idiopathic Nephrotic Syndrome: Clinical Efficacy and Pathophysiological Aspects

Journal of Clinical Medicine ◽

10.3390/jcm8060860 ◽

2019 ◽

Vol 8 (6) ◽

pp. 860 ◽

Cited By ~ 7

Author(s):

Anne K. Mühlig ◽

Jun Young Lee ◽

Markus J. Kemper ◽

Andreas Kronbichler ◽

Jae Won Yang ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Idiopathic Nephrotic Syndrome ◽

Low Frequency ◽

Glomerular Diseases ◽

Steroid Dependency ◽

Randomized Controlled ◽

Steroid Dependent ◽

Frequent Relapses ◽

Steroid Sensitive Nephrotic Syndrome ◽

Steroid Sensitive

Steroid sensitive nephrotic syndrome is one of the most common pediatric glomerular diseases. Unfortunately, it follows a relapsing and remitting course in the majority of cases, with 50% of all cases relapsing once or even more often. Most children with idiopathic nephrotic syndrome respond initially to steroid therapy, nevertheless repeated courses for patients with relapses induce significant steroid toxicity. Patients with frequent relapses or steroid dependency thus require alternative treatment, such as cyclophosphamide, cyclosporine, tacrolimus, mycophenolate mofetil, levamisole, or rituximab. To reduce the relapse rate, several drugs have been used. Among these, levamisole has been considered the least toxic and least expensive therapy. Several randomized controlled trials (RCT) showed that levamisole is effective in reducing the relapse risk in steroid sensitive forms of nephrotic syndrome with a low frequency of side effects. Levamisole is a synthetic imidazothiazole derivative with immune-modulatory properties. In this article, we review recent data from randomized trials and observational studies to assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome.

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Steroid-induced diabetic ketoacidosis in a 14-year-old boy with steroid-sensitive nephrotic syndrome: Case report and literature review.

Nigerian Journal of Paediatrics ◽

10.4314/njp.v42i2.17 ◽

1970 ◽

Vol 42 (2) ◽

pp. 154-157

Author(s):

AN Onyiriuka ◽

NJ Iduoriyekemwen ◽

RY Esin

Keyword(s):

Nephrotic Syndrome ◽

Blood Glucose ◽

Diabetic Ketoacidosis ◽

Steroid Therapy ◽

Postprandial Blood Glucose ◽

High Dose ◽

Premixed Insulin ◽

Glucose Levels ◽

Steroid Sensitive Nephrotic Syndrome ◽

Steroid Sensitive

In this report, we described the case of a 14-year-old boy with steroid-sensitive nephrotic syndrome who developed hyperglycaemia and ultimately, diabetic ketoacidosis, following high-dose steroid therapy for a primary renal disease. The nephrotic syndrome was diagnosed based on generalized oedema, massive proteinuria, hypoalbuminaemia and hypercholesterolaemia. Serum creatinine and random blood glucose levels were normal and there was no glycosuria. He was commenced on high dose prednisolone 40 mg 12 hourly and by the 8th day on prednisolone, he achieved remission and was discharged. However, four weeks later, he developed features of diabetic ketoacidosis (DKA) which was confirmed by the presence of hyperglycaemia (random blood glucose19.4 mmol/L), acidosis (serum bicarbonate 10 mmol/L) and ketonuria (2+). The DKA was managed with intravenous fluid (0.9% sodium chloride), continuous insulin infusion and antibiotics. After resolution of the DKA, he was switched to subcutaneous soluble insulin and thereafter, premixed insulin twice daily with a reduction in the dose of prednisolone and was discharged home after 30 days on admission. Blood glucose level has remained within normal range one year after discontinuing insulin and he is still in remission with regard to the nephrotic syndrome at follow up.Conclusion: The risk of diabetic ketoacidosis should be considered in the course of steroid therapy for nephrotic syndrome. To avoid missing of cases of steroid-induced diabetes mellitus, and ultimately DKA, both fasting and postprandial blood glucose values should be monitored.Key words: Adolescence, diabetes, ketoacidosis, nephrotic syndrome, steroid therapy.

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