Quality of life in children with severe forms of idiopathic nephrotic syndrome in stable remission—A cross‐sectional study

Abstract

Aim: Severe forms of idiopathic nephrotic syndrome (INS) require immunosuppressive therapy: oral treatment or intravenous therapy (rituximab, RTX). The main objective was to describe quality of life (QOL) in these specific patients.Methods: Cross-sectional, multicentre, observational study analysed QOL using a standardised questionnaire in children from 7 to 17 years, with a steroid-dependent or steroid-resistant INS in stable remission. The questionnaire consisted of 30 questions concerning physical and emotional well-being, self-esteem, family, friends, school and disease resulting in a global score of 0-100. Results: A total of 110 patients with a mean age of 11.6 years from three French paediatric nephrology centres were included. A total of 71 patients had oral immunosuppressive treatment, 27 had RTX, and 12 had both. 13.6% of patients had a steroid-resistant INS. The mean number of relapses was 5.8. Seventy-eight patients answered the questionnaire. The global score in the whole study population was 74.7; 72.6 in the RTX group, 76.2 in the oral drugs group, (P = 0.49). The results of sub-dimension 'school' were statistically lower in RTX group (61.6 ± 19.5) compared with oral drugs group (71.4 ± 16; P = 0.02). Conclusion: Global QOL score was high in 'difficult-to-treat' patients with INS in stable remission on oral immunosuppressive or RTX treatment. K E Y W O R D S children and adolescents, immunosuppression, nephrotic syndrome, quality of life 2268 | ROUSSEL Et aL. S U PP O RTI N G I N FO R M ATI O N Additional supporting information may be found online in the Supporting Information section at the end of the article. How to cite this article: Roussel A, Delbet J-D, Micheland L, Deschênes G, Decramer S, Ulinski T. Quality of life in children with severe forms of idiopathic nephrotic syndrome in stable remission-A cross-sectional study. Acta Paediatr.

Reliability of generic quality-of-life instruments in assessing health-related quality of life among children and adolescents with idiopathic nephrotic syndrome: a systematic review

“…The ten selected studies consist of case–control studies (n = 5) [ 10 , 17 , 21 – 23 ], longitudinal cohort studies (n = 2) [ 24 , 25 ], and cross-sectional studies (n = 3) [ 15 , 16 , 26 ]. The countries of study were distributed among four continents as follows: North America [ 24 , 25 ], Europe [ 16 , 21 ], Asia [ 15 , 17 , 22 , 23 ], and Africa [ 10 , 26 ]. All the studies were hospital-based.…”

“…In five studies that reported sex distribution [ 15 , 17 , 22 , 24 , 26 ], the total number of males and females was 204 and 122, respectively, with an approximate ratio of 1.7: 1 (Table 1 ). Study methodological quality, using the NOS, shows a star-rating of < 7 (low quality) for three studies [ 15 , 16 , 26 ] and ≥ 7 (high quality) for seven studies [ 17 , 21 – 25 ]. (N/B: Rating indicates the range of quality [based on the adopted criteria from NOS] and does not translate to unreliable study findings for studies rated as low quality.…”

“…Prolonged disease duration and frequent relapses contributed significantly to low HRQoL scores. Secondly, Roussel et al described the HRQoL in 110 children with difficult-to-treat nephrotic syndrome on stable remission, on either oral immunosuppressive drugs or intravenous rituximab (RTX) [ 16 ]. Using a child-rated QoL evaluation, they found a high global HRQoL score on these parameters: physical and emotional well-being, self-esteem, family, friends, school, and disease.…”

“…The remaining three studies used the following instruments alone in evaluating the HRQoL of the patients: Netherlands Organization for Applied Scientific Research-Academical Medical Center (TNO-AZL) Child Quality of Life Questionnaire, Child Behavior checklist, and Teacher Report form [ 21 ]; 30-item standardized questionnaire with a global score of 0–100 [ 17 ]; and PROMIS instrument [ 25 ]. The PedsQL™ 4.0 Generic Core Scales was used to evaluate child-rated HRQoL in six studies [ 10 , 16 , 17 , 22 – 24 ], and both child- and parent-rated HRQoL in one study [ 26 ]. The PedsQL™ 4.0 Generic Core Scales specifically evaluates the HRQoL in five domains: physical functioning (eight items), psychosocial functioning, including emotional functioning (five items), social functioning (five items), and school functioning (five items).…”

“…We could access the data indicating the aggregate numerical indicators of QoL domains in four studies [ 16 , 17 , 22 , 24 ]. As shown in Table 3 and Fig.…”