Clinical Nephrology, Vol. 81 – No. 3/2014 (166-173)
Long-term outcomes in idiopathic nephrotic syndrome: from childhood to adulthood Original ©2014 Dustri-Verlag Dr. K. Feistle ISSN 0301-0430 DOI 10.5414/CN108044 e-pub: December 23, 2013
Piotr Skrzypczyk1, Małgorzata Pańczyk-Tomaszewska1, Maria Roszkowska-Blaim1, Zofia Wawer1, Beata Bieniaś2, Małgorzata Zajączkowska2, Katarzyna Kiliś-Pstrusińska3, Anna Jakubowska3, Marta Szczepaniak4, Monika Pawlak-Bratkowska4, and Marcin Tkaczyk4 1Department
of Pediatrics and Nephrology, Medical University of Warsaw, Warsaw, of Pediatric Nephrology, Medical University of Lublin, Lublin, 3Department of Pediatric Nephrology, Medical University of Wroclaw, Wroclaw, and 4Nephrology Division, Department of Pediatrics and Immunology, Polish Mother’s Memorial Hospital-Research Institute, Lodz, Poland 2Department
Key words nephrotic syndrome – children – outcomes – health status – social status
Received March 11, 2013; accepted in revised form October 4, 2013 Correspondence to Piotr Skrzypczyk, MD Department of Pediatrics and Nephrology, Medical University of Warsaw, 24 Marszalkowska St, 00-576 Warsaw, Poland [email protected]
Abstract. Background: The aim of the study was to assess idiopathic nephrotic syndrome (INS) relapse rate, co-morbidities, and social status of adults diagnosed with INS in childhood. Material and methods: A written questionnaire was sent to 118 adults treated for INS in childhood. In 61 (51.7%) responders (aged 26.0 ± 6.2 years, range 18 – 51.5 years), we used available medical records to evaluate age at the onset of INS, number of INS relapses below 18 years of age, response to corticosteroids (CS), renal biopsy findings, and immunosuppressive treatment as well as questionnaire to evaluate the number and treatment of INS relapses above 18 years of age, co-morbidities, age at menarche, marital status, offspring, educational status, and occupation. Results: In the group of 61 responders, median age at the onset of INS was 3 (range 1.3 – 14.0) years, median number of INS relapses at 18 years of age had more (p 30 years (n = 12). The study was approved by the Bioethics Committee at the Medical University of War-
168
saw, and all participants gave their informed consent prior to their inclusion in the study. Statistical analyses were performed using the Statistica 9.0 software (Statsoft, Tulsa, OK, USA). Variable distribution was analyzed using the Shapiro-Wilk test. Normally distributed variables were presented as mean values with standard deviation and compared using the Student t-test for independent groups and the ANOVA test. Non-normally distributed variables were presented as median values with range and compared using the Mann Whitney U-test and the KruskalWallis analysis of variance. Differences in the frequency of analyzed variables between two groups were analyzed using the χ2-test or the Fisher exact test, when appropriate. pvalue of less than 0.05 was considered statistically significant.
Results In the study group of 61 responders, median age of the onset of INS was 3.0 years (range 1.33 – 14.0 years), and median number of INS relapses in childhood was 5 (range 1 – 20). SSNS was diagnosed in 37 patients (60.7%), SDNS in 18 patients (29.5%), and SRNS in 6 patients (9.8%). At the time of current evaluation, mean age in these groups was 26.9 ± 7.1 (range 18.0 – 51.5), 24.5 ± 4.5 (range 18.9 – 34.0), and 24.6 ± 4.5 (range 18.5 – 32.4) years, respectively (p = 0.35). Duration of follow-up in the SSNS group ranged from 6 to 38 years (mean 21.4 ± 7.40 years), in the SDNS group from 15.5 to 32.7 years (mean 21.7 ± 4.8 years), and in the SRNS group from 13.4 to 29.8 years (mean 21.4 ± 5.3 years) (p = 0.88). Renal biopsies were performed in childhood in 28 patients (45.6%), with the following morphological patterns found: minimal change in 10 patients (35.7%), mesangial proliferation in 17 patients (60.7%), and focal and segmental glomerulosclerosis in 1 patient (3.6%). All patients were initially treated with oral CS. Patients with frequently relapsing SSNS were also treated with MPP (n = 4, 10.8%), LEV (n = 6, 16.2%), CHL (n = 3, 8.1%), and CYP (n = 2, 5.4%); SDNS patients were treated with MPP (n = 8, 44.4%), LEV (n = 13, 72.2%), CYP (n = 6, 33.3%), CHL (n = 5, 27.8%), and CsA (n = 2, 11.1%);
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Long-term outcome in idiopathic nephrotic syndrome
Table 2. Selected clinical parameters in patients with or without relapses of nephrotic syndrome in adulthood. Parameter Patients (n, %) Males/females (n/n) Current age (years) Nephrotic syndrome in childhood Age at disease onset (years) Response to CS (n, %) SSNS n = 37 (60.7%) SDNS n = 18 (29.5%) SRNS n = 6 (9.8%) Number of nephrotic syndrome relapses in childhood
Nephrotic syndrome in adulthood Relapse No relapse 10 (16.4%) 51 (83.6%) 5/5 30/21 28.2 ± 5.1 25.6 ± 6.4
p < 0.0005 NS NS
2.6 (1.3 – 13.5)
3.1 (1.5 – 14.0)
NS
5 (50.0%) 4 (40.0%) 1 (10.0%) 10 (4 – 20)
32 (62.7%) 14 (27.5%) 5 (9.8%) 4 (1 – 16)
NS NS NS < 0.005
CS = corticosteroids; n = number of patients; NS = nonsignificant; SDNS = steroid-dependent nephrotic syndrome; SRNS = steroid-resistant nephrotic syndrome; SSNS = steroid-sensitive nephrotic syndrome.
and SRNS patients were treated with MPP (n = 3, 50.0%), CYP (n = 5, 83.3%), and CHL (n = 3, 50.0%). All patients achieved complete remission in childhood and had normal renal function at the age of 18. Clinical data of responders and non-responders are shown in Table 1. The groups did not differ significantly in terms of current age, sex, age at onset of INS, response to CS, or the number of INS relapses. Renal biopsy was more frequently (p
Long-term outcomes in idiopathic nephrotic syndrome: from childhood to adulthood Original ©2014 Dustri-Verlag Dr. K. Feistle ISSN 0301-0430 DOI 10.5414/CN108044 e-pub: December 23, 2013
Piotr Skrzypczyk1, Małgorzata Pańczyk-Tomaszewska1, Maria Roszkowska-Blaim1, Zofia Wawer1, Beata Bieniaś2, Małgorzata Zajączkowska2, Katarzyna Kiliś-Pstrusińska3, Anna Jakubowska3, Marta Szczepaniak4, Monika Pawlak-Bratkowska4, and Marcin Tkaczyk4 1Department
of Pediatrics and Nephrology, Medical University of Warsaw, Warsaw, of Pediatric Nephrology, Medical University of Lublin, Lublin, 3Department of Pediatric Nephrology, Medical University of Wroclaw, Wroclaw, and 4Nephrology Division, Department of Pediatrics and Immunology, Polish Mother’s Memorial Hospital-Research Institute, Lodz, Poland 2Department
Key words nephrotic syndrome – children – outcomes – health status – social status
Received March 11, 2013; accepted in revised form October 4, 2013 Correspondence to Piotr Skrzypczyk, MD Department of Pediatrics and Nephrology, Medical University of Warsaw, 24 Marszalkowska St, 00-576 Warsaw, Poland [email protected]
Abstract. Background: The aim of the study was to assess idiopathic nephrotic syndrome (INS) relapse rate, co-morbidities, and social status of adults diagnosed with INS in childhood. Material and methods: A written questionnaire was sent to 118 adults treated for INS in childhood. In 61 (51.7%) responders (aged 26.0 ± 6.2 years, range 18 – 51.5 years), we used available medical records to evaluate age at the onset of INS, number of INS relapses below 18 years of age, response to corticosteroids (CS), renal biopsy findings, and immunosuppressive treatment as well as questionnaire to evaluate the number and treatment of INS relapses above 18 years of age, co-morbidities, age at menarche, marital status, offspring, educational status, and occupation. Results: In the group of 61 responders, median age at the onset of INS was 3 (range 1.3 – 14.0) years, median number of INS relapses at 18 years of age had more (p 30 years (n = 12). The study was approved by the Bioethics Committee at the Medical University of War-
168
saw, and all participants gave their informed consent prior to their inclusion in the study. Statistical analyses were performed using the Statistica 9.0 software (Statsoft, Tulsa, OK, USA). Variable distribution was analyzed using the Shapiro-Wilk test. Normally distributed variables were presented as mean values with standard deviation and compared using the Student t-test for independent groups and the ANOVA test. Non-normally distributed variables were presented as median values with range and compared using the Mann Whitney U-test and the KruskalWallis analysis of variance. Differences in the frequency of analyzed variables between two groups were analyzed using the χ2-test or the Fisher exact test, when appropriate. pvalue of less than 0.05 was considered statistically significant.
Results In the study group of 61 responders, median age of the onset of INS was 3.0 years (range 1.33 – 14.0 years), and median number of INS relapses in childhood was 5 (range 1 – 20). SSNS was diagnosed in 37 patients (60.7%), SDNS in 18 patients (29.5%), and SRNS in 6 patients (9.8%). At the time of current evaluation, mean age in these groups was 26.9 ± 7.1 (range 18.0 – 51.5), 24.5 ± 4.5 (range 18.9 – 34.0), and 24.6 ± 4.5 (range 18.5 – 32.4) years, respectively (p = 0.35). Duration of follow-up in the SSNS group ranged from 6 to 38 years (mean 21.4 ± 7.40 years), in the SDNS group from 15.5 to 32.7 years (mean 21.7 ± 4.8 years), and in the SRNS group from 13.4 to 29.8 years (mean 21.4 ± 5.3 years) (p = 0.88). Renal biopsies were performed in childhood in 28 patients (45.6%), with the following morphological patterns found: minimal change in 10 patients (35.7%), mesangial proliferation in 17 patients (60.7%), and focal and segmental glomerulosclerosis in 1 patient (3.6%). All patients were initially treated with oral CS. Patients with frequently relapsing SSNS were also treated with MPP (n = 4, 10.8%), LEV (n = 6, 16.2%), CHL (n = 3, 8.1%), and CYP (n = 2, 5.4%); SDNS patients were treated with MPP (n = 8, 44.4%), LEV (n = 13, 72.2%), CYP (n = 6, 33.3%), CHL (n = 5, 27.8%), and CsA (n = 2, 11.1%);
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Long-term outcome in idiopathic nephrotic syndrome
Table 2. Selected clinical parameters in patients with or without relapses of nephrotic syndrome in adulthood. Parameter Patients (n, %) Males/females (n/n) Current age (years) Nephrotic syndrome in childhood Age at disease onset (years) Response to CS (n, %) SSNS n = 37 (60.7%) SDNS n = 18 (29.5%) SRNS n = 6 (9.8%) Number of nephrotic syndrome relapses in childhood
Nephrotic syndrome in adulthood Relapse No relapse 10 (16.4%) 51 (83.6%) 5/5 30/21 28.2 ± 5.1 25.6 ± 6.4
p < 0.0005 NS NS
2.6 (1.3 – 13.5)
3.1 (1.5 – 14.0)
NS
5 (50.0%) 4 (40.0%) 1 (10.0%) 10 (4 – 20)
32 (62.7%) 14 (27.5%) 5 (9.8%) 4 (1 – 16)
NS NS NS < 0.005
CS = corticosteroids; n = number of patients; NS = nonsignificant; SDNS = steroid-dependent nephrotic syndrome; SRNS = steroid-resistant nephrotic syndrome; SSNS = steroid-sensitive nephrotic syndrome.
and SRNS patients were treated with MPP (n = 3, 50.0%), CYP (n = 5, 83.3%), and CHL (n = 3, 50.0%). All patients achieved complete remission in childhood and had normal renal function at the age of 18. Clinical data of responders and non-responders are shown in Table 1. The groups did not differ significantly in terms of current age, sex, age at onset of INS, response to CS, or the number of INS relapses. Renal biopsy was more frequently (p
