Diethylcarbamazine (DEC) in Relapse Cases of Nephrotic Syndrome in Filarial Endemic Region: A Case Series

Introduction: Incidence of nephrotic syndrome (NS) and filariasis both is high in China, Japan & India. Studies have shown the association of filariasis in NS. In the coastal belt of Gujarat, Filariasis occurs as the mosquito responsible is still prevalent. Therefore, filariasis association may be causing persistence of oedema in NS relapse cases. After a 7th relapse patient was treated successfully with diethylcarbamazine citrate (DEC), we decided to observe the effect of DEC on weight loss and urine protein, in relapse patients of NS. Case Details: In relapse patients of NS, DEC was given by oral route in the dose of 72 mg/kg/cycle for 7 days. Weight record and urine protein were measured daily. Steroid as Tab prednisolone was administered at 2 mg/k/d. Outcome: The 1st case was a steroid-dependent nephrotic syndrome with the 7th relapse; she had been on prednisolone and Levamisol for 3 years. DEC was started on day 3 of admission and response was seen on the 5th day. Urinary protein became nil on day 10, and the patient has been relapse free for 1 year. In each of the other 4 cases with 1st, 2nd, 2nd and 4th relapse respectively, the response of DEC was seen within 2 days. Thus, after starting DEC weight and urine protein reduced within 2 days in 5 relapse cases. Filaria was not detected in blood film of any patient and Elisa tests done in 2 were negative. Conclusion: Randomized studies with controls and better filarial detection methods are required for DEC to be considered as an add-on drug in relapse cases of NS in, Filarial endemic regions, as it is faster acting, effective, similar to Levamisol and safe.

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Lipoprotein (a) as a Predictor of Steroid Dependence in Paediatric Steroid Sensitive Nephrotic Syndrome

JOURNAL FOR CLINICAL AND DIAGNOSTIC RESEARCH ◽

10.7860/jcdr/2021/46262.14409 ◽

2021 ◽

Author(s):

Surupa Basu ◽

Sushmita Banerjee ◽

Pranab Roy ◽

Apurba Ghosh

Keyword(s):

Nephrotic Syndrome ◽

First Episode ◽

Urine Protein ◽

Lipoprotein A ◽

Steroid Dependence ◽

Steroid Dependency ◽

Steroid Dependent ◽

Steroid Sensitive ◽

One Year ◽

Lipid Parameters

Introduction: Lipoprotein a {Lp(a)} increases in Nephrotic Syndrome (NS). Although the majority of paediatric NS are steroid sensitive, relpase and steroid dependence are commonly seen in this cases. Lp(a) is an LDL-like lipoprotein that consists of an LDL particle to which the glycoprotein apolipoprotein(a) {apo(a)} is attached. Aim: To evaluate the potential of Lp(a), measured on admission, for the prediction of relapse/steroid dependency. Materials and Methods: Children (n=36) with first episode NS were recruited in this prospective observational case-control study and followed up for one year. They were tested at presentation for Lp(a) (mg/dL) and standard tests such as haemoglobin, albumin, protein, cholesterol, triglyceride, and urine protein. Children received standard therapy for NS, and were followed for a period of one year from diagnosis to record days to initial remission, relapse episodes, steroid dependence etc. Patients were categorised as: no relapse (NR), Infrequent Relapse (IFR), frequent relapse (FR) and Steroid Dependent (SD) as per standard definitions. Fifteen healthy volunteers were also tested for lipid profile and Lp(a) levels. Results: Of 36 cases (median age 3 years, 19 males), there were 15NR, 7IFR, 2FR and 12SD. The mean Lp(a) of the NS group (165.2±120.4 mg/dL) was higher than controls (30.52±21.9 mg/dL) (p<0.0001). All the lipid parameters except HDL-cholesterol were significantly higher in the NS group. Within the NS group, Lp(a) showed significant correlation (Spearman-rho) with albumin (p=0.0062,r=0.47), but no correlation with lipid parameters or urine protein. Comparison of Lp(a)levels in the NS groups revealed that the SD patients had a high Lp(a)(222.0±115.7 mg/dL) compared to NR (129.7±120.1 mg/dL) (p=0.02). Conclusion: Concentration of plasma Lp(a) in patients with SDNS was higher compared to patients who did not suffer any relapse, and this concentration may serve as a marker for prediction of SDNS.

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MO291RITUXIMAB IS EFFECTIVE AND SAFE IN ADULTS WITH STEROID-DEPENDENT NEPHROTIC SYNDROME: A LONG-TERM, SINGLE-CENTER EXPERIENCE

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab104.0049 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Gemma Patella ◽

Alessandro Comi ◽

Giuseppe Coppolino ◽

Nicolino Comi ◽

Giorgio Fuiano ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Case Series ◽

Adult Patients ◽

Single Center ◽

Single Center Experience ◽

Steroid Dependent ◽

The Mean ◽

Steroid Dependent Nephrotic Syndrome

Abstract Background and Aims Steroid-dependent nephrotic syndrome (SDNS) may require a prolonged multi-drug therapy with risk of drug toxicity and renal failure. Rituximab (RTX) treatment has been found to be helpful in reducing the steroid dosage and the need for immunosuppressants (ISs), but little data are currently available regarding very long-term outcomes in adults. We herein describe a long-term, single-center experience of RTX use in a large series of adults with SDNS. Method We studied 23 adult patients with SDNS (mean age 54.2±17.1 y; 65% male; BMI 28.5±4.7), mostly consequent to membranous (47.8%) or focal glomerulonephritis (30.2 %) who were eligible to start a RTX regimen. Before entering the RTX protocol, proteinuria and eGFR were 7.06±3.87 g/24h and 65.9±28.2 ml/min/1.73 m2, respectively; albumin and CD19/CD20 ratio were 2.9±0.9 g/L and 0.99±0.01 respectively; the mean number of ISs was 2.39±0.89 and the mean annual rate of relapses was 2.2±0.9. Results Patients were followed over a mean follow-up of 64 months (range: 12-144). After RTX (mean dose: 1202.1±372.4 mg) the rate of relapses was virtually nullified (p<0.001). eGFR remained roughly stable (62.1±19.8 ml/min/1.73 m2, p=NS), while proteinuria, albumin, CD19/CD20 and BMI all significantly improved (p ranging from 0.01 to 0.001). The mean number of additional ISs was also reduced (0.44±0.12; p<0.001) and RTX enabled discontinuation of steroids in 13/23 (56.5%) patients. No major adverse events related to therapy were recorded. Conclusion Findings from this large case-series with a remarkable very long follow-up reinforce the role of RTX as an efficient and safe weapon to improve outcomes in adult patients suffering from SDNS.

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Efficacy of Semiannual Single Fixed Low-Dose Rituximab Therapy in Steroid-Dependent Minimal Change Nephrotic Syndrome: A Case Series

Case Reports in Nephrology and Dialysis ◽

10.1159/000493189 ◽

2018 ◽

Vol 8 (3) ◽

pp. 230-237 ◽

Cited By ~ 1

Author(s):

Ryosuke Usui ◽

Yohei Tsuchiya ◽

Kosaku Nitta ◽

Minako Koike

Keyword(s):

Nephrotic Syndrome ◽

Low Dose ◽

Chemotherapy Regimen ◽

Case Series ◽

Minimal Change Nephrotic Syndrome ◽

Minimal Change ◽

Cell Hyperplasia ◽

Conventional Dose ◽

Steroid Dependent ◽

Refractory Nephrotic Syndrome

The frequency of using rituximab to treat refractory nephrotic syndrome has recently been increasing, and the conventional dose of rituximab used to treat it, 375 mg/m2 body surface area once weekly for 4 weeks, has been modelled on the chemotherapy regimen for B-cell non-Hodgkin’s lymphoma. The dose and intervals of rituximab in refractory nephrotic syndrome remain controversial. Clear lymphoma cell hyperplasia is seen in lymphoma patients, but not in nephrotic syndrome patients. Since we thought that it might be possible to reduce the dose of rituximab if only used for the purpose of depleting CD20-positive B cells in nephrotic patients’ peripheral blood, we tried semiannually with a single fixed rituximab dose of 100 mg/body, and a complete remission was attained in 3 cases without treatment with prednisolone or cyclosporine. Our report strongly suggests considering appropriate dose and interval of rituximab therapy in the treatment of steroid-dependent nephrotic syndrome.

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Levamisole in steroid-dependent nephrotic syndrome in children: A case series

Medical Journal of Dr D Y Patil Vidyapeeth ◽

10.4103/mjdrdypu.mjdrdypu_211_20 ◽

2021 ◽

Vol 0 (0) ◽

pp. 0

Author(s):

Muzamil Latief ◽

Obeid Shafi ◽

Zhahid Hassan ◽

Farhat Abbas ◽

Summyia Farooq

Keyword(s):

Nephrotic Syndrome ◽

Case Series ◽

Steroid Dependent ◽

Steroid Dependent Nephrotic Syndrome

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A case of a 7-year-old boy with steroid-dependent nephrotic syndrome who developed severe neutropenia three months after the administration of rituximab

Nihon Shoni Jinzobyo Gakkai Zasshi ◽

10.3165/jjpn.22.97 ◽

2009 ◽

Vol 22 (2) ◽

pp. 97-101 ◽

Cited By ~ 2

Author(s):

Ryugo Hiramoto ◽

Shinsuke Matsumoto ◽

Hironobu Eguchi ◽

Yoshitaka Miyoshi ◽

Isao Komori ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Severe Neutropenia ◽

Steroid Dependent ◽

Steroid Dependent Nephrotic Syndrome

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Worsening anasarca on a child with severe steroid-dependent nephrotic syndrome without proteinuria: Answers

Pediatric Nephrology ◽

10.1007/s00467-021-05124-6 ◽

2021 ◽

Author(s):

Nikita Tripathi ◽

Anup Singh ◽

Prachi Agarwal

Keyword(s):

Nephrotic Syndrome ◽

Steroid Dependent ◽

Steroid Dependent Nephrotic Syndrome

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Effect of single-dose rituximab on steroid-dependent minimal-change nephrotic syndrome in adults

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfs515 ◽

2012 ◽

Vol 28 (5) ◽

pp. 1225-1232 ◽

Cited By ~ 36

Author(s):

T. Takei ◽

M. Itabashi ◽

T. Moriyama ◽

C. Kojima ◽

S. Shiohira ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Single Dose ◽

Minimal Change Nephrotic Syndrome ◽

Minimal Change ◽

Steroid Dependent

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IGFs and IGF-binding proteins in short children with steroid-dependent nephrotic syndrome on chronic glucocorticoids: changes with 1 year exogenous GH

Acta Endocrinologica ◽

10.1530/eje.0.1440237 ◽

2001 ◽

pp. 237-243 ◽

Cited By ~ 13

Author(s):

X Zhou ◽

KY Loke ◽

CC Pillai ◽

HK How ◽

HK Yap ◽

...

Keyword(s):

Nephrotic Syndrome ◽

Growth Retardation ◽

Bone Age ◽

Gh Treatment ◽

Igf Binding Proteins ◽

Steroid Dependent ◽

Igf I ◽

Igf Binding ◽

Pre Treatment ◽

Igfbp 3

OBJECTIVE: Children with steroid-dependent nephrotic syndrome (SDNS), despite being in remission on glucocorticoids, continue to have growth retardation and short stature. The mechanism is uncertain as both chronic glucocorticosteroids and the nephrotic syndrome may independently affect growth. We investigated the changes in the IGFs and IGF-binding proteins (IGFBPs) in a group of short SDNS children, and studied the changes prospectively with 1 year's treatment with GH. DESIGN AND METHODS: Total and 'free' IGF-I, IGFBP-3 and acid-labile subunit (ALS) were studied in eight SDNS boys (mean age=12.6 years; mean bone age=9.1 years) on long term oral prednisolone (mean dose 0.46 mg/kg per day) before, during, and after, 1 year's treatment with GH (mean dose 0.32 mg/kg per week). Pretreatment comparisons were made with two control groups, one matched for bone age (CBA; mean bone age=9.2 years), and another for chronological age (CCA; mean chronological age=13 years). Subsequently, three monthly measurements of serum and urine IGFBPs were carried out in the GH-treated SDNS patients using Western ligand blot and Western immunoblot. RESULTS: Pre-treatment serum total IGF-I levels and the IGF-I/IGFBP-3 ratio were elevated significantly in SDNS compared with CBA, and were similar to CCA. Serum free IGF-I levels were elevated significantly compared with both control groups, but serum IGFBP-3 did not differ significantly. Urinary IGFBP-2, IGFBP-3 and ALS were detectable in the SDNS children only. With GH treatment, IGF-I and IGFBP-3, but not IGF-II, increased significantly compared with pre-treatment values, and returned to baseline after cessation of GH treatment. Urinary IGFBPs did not change significantly with GH treatment. CONCLUSIONS: There is persistent urinary loss of IGFBP-2, IGFBP-3 and ALS in children with SDNS in remission with growth retardation. However, the significant elevation in serum IGF-I suggests that glucocorticoid-induced resistance to IGF is the main factor responsible for the persistent growth retardation in these children. Exogenous GH was able to overcome this resistance by further increasing serum IGF-I.

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