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Erschienen in: Clinical and Experimental Nephrology 5/2019

05.02.2019 | Original Article

The long-term outcome of childhood nephrotic syndrome in Germany: a cross-sectional study

verfasst von: Malik Aydin, Ingo Franke, Lisa Kurylowicz, Rainer Ganschow, Michael Lentze, Mark Born, Rebekka Hagemann

Erschienen in: Clinical and Experimental Nephrology | Ausgabe 5/2019

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Abstract

Background

Long-term outcomes of children with nephrotic syndrome have not been well described in the literature.

Methods

Cross-sectional study data analysis of n = 43 patients with steroid-sensitive (SSNS) and n = 7 patients with steroid-resistant (SRNS) nephrotic syndrome were retrospectively collected; patients were clinically examined at a follow-up visit (FUV), on average 30 years after onset, there was the longest follow-up period to date.

Results

The mean age at FUV was 33.6 years (14.4–50.8 years, n = 41). The mean age of patients with SSNS at onset was 4.7 years (median 3.8 years (1.2–14.5 years), the mean number of relapses was 5.8 (0 to 29 relapses). Seven patients (16.3%) had no relapses. Eleven patients were “frequent relapsers” (25.6%) and four patients still had relapses beyond the age of 18 years. Except of cataracts and arterial hypertension, there were no negative long-term outcomes and only one patient was using immunosuppressant therapy at FUV. 55% of patients suffered from allergies and 47.5% had hypercholesterolemia. Two patients suffered a heart attack in adulthood. A younger age at onset (< 4 years) was a risk factor for frequent relapses. An early relapse (within 6 months after onset) was a risk factor and a low birth weight was not a significant risk factor for a complicated NS course. The mean age of patients with SRNS at onset was 4.6 ± 4.4 years and 27.5 ± 9.9 years at FUV. Three patients received kidney transplantations.

Conclusions

The positive long-term prognosis of SSNS can reduce the concern of parents about the probability of the child developing a chronic renal disease during the clinical course after onset.
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Literatur
1.
Zurück zum Zitat International Study of Kidney Disease in Children/ISKDC. The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. J Pediatr. 1981;98:561–4.CrossRef International Study of Kidney Disease in Children/ISKDC. The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. J Pediatr. 1981;98:561–4.CrossRef
2.
Zurück zum Zitat Nash MA, Edelmann CM Jr, Bernstein J, Barnett HL. Minimal change nephrotic syndrome, diffuse mesangial hypercellularity and focal glomerulosclerosis. In: Edelmann CM Jr, editor. Pediatric kidney disease. 2nd ed. Boston: Little, Brown; 1992. pp. 1267–90. Nash MA, Edelmann CM Jr, Bernstein J, Barnett HL. Minimal change nephrotic syndrome, diffuse mesangial hypercellularity and focal glomerulosclerosis. In: Edelmann CM Jr, editor. Pediatric kidney disease. 2nd ed. Boston: Little, Brown; 1992. pp. 1267–90.
5.
Zurück zum Zitat Bagga A, Mantan M. Nephrotic syndrome in children. Indian J Med Res. 2005;122:13–28.PubMed Bagga A, Mantan M. Nephrotic syndrome in children. Indian J Med Res. 2005;122:13–28.PubMed
6.
Zurück zum Zitat International Study of Kidney Disease in Children/ISKDC. Early identification of frequent relapsers among children with minimal change nephrotic syndrome. J Pediatr. 1982;101:514–8.CrossRef International Study of Kidney Disease in Children/ISKDC. Early identification of frequent relapsers among children with minimal change nephrotic syndrome. J Pediatr. 1982;101:514–8.CrossRef
7.
Zurück zum Zitat Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. J Am Soc Nephrol. 1997;8:769–76.PubMed Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. J Am Soc Nephrol. 1997;8:769–76.PubMed
8.
Zurück zum Zitat Broyer M, Meyrier A, Niaudet P, Habib R. Minimal changes and focal segmental glomerular sclerosis. In: Davison AM, editor. Oxford textbook of clinical nephrology. 2nd ed. Vol. 1. Oxford: Oxford university Press; 1998. pp. 493–535. Broyer M, Meyrier A, Niaudet P, Habib R. Minimal changes and focal segmental glomerular sclerosis. In: Davison AM, editor. Oxford textbook of clinical nephrology. 2nd ed. Vol. 1. Oxford: Oxford university Press; 1998. pp. 493–535.
9.
Zurück zum Zitat Gipson DS, Chin H, Presler TP, et al. Differential risk of remission and ESRD in childhood FSGS. Pediatr Nephrol. 2006;21:344–9.CrossRefPubMed Gipson DS, Chin H, Presler TP, et al. Differential risk of remission and ESRD in childhood FSGS. Pediatr Nephrol. 2006;21:344–9.CrossRefPubMed
10.
Zurück zum Zitat Siegel NJ, Goldberg B, Krassner LS, Hayslett JP. Long-term follow-up of children with steroid-responsive nephrotic syndrome. J Pediatr. 1972;81:251–8.CrossRefPubMed Siegel NJ, Goldberg B, Krassner LS, Hayslett JP. Long-term follow-up of children with steroid-responsive nephrotic syndrome. J Pediatr. 1972;81:251–8.CrossRefPubMed
11.
Zurück zum Zitat Fakhouri F, Bocquet N, Taupin P, Presne C, Gagnadoux MF, Landais P, Lesavre P, Chauveau D, Knebelmann B, Broyer M, Grünfeld JP, Niaudet P. Steroid-sensitive nephrotic syndrome: from childhood to adulthood. Am J Kidney Dis. 2003;41:550–7.CrossRefPubMed Fakhouri F, Bocquet N, Taupin P, Presne C, Gagnadoux MF, Landais P, Lesavre P, Chauveau D, Knebelmann B, Broyer M, Grünfeld JP, Niaudet P. Steroid-sensitive nephrotic syndrome: from childhood to adulthood. Am J Kidney Dis. 2003;41:550–7.CrossRefPubMed
12.
Zurück zum Zitat Rüth EM, Kemper MJ, Leumann EP, Laube GF, Neuhaus TJ. Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr. 2005;147:202–7.CrossRefPubMed Rüth EM, Kemper MJ, Leumann EP, Laube GF, Neuhaus TJ. Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr. 2005;147:202–7.CrossRefPubMed
13.
Zurück zum Zitat Trompeter RS, Lloyd BW, Hicks J, White RH, Cameron JS. Long-term outcome for children with minimal-change nephrotic syndrome. Lancet. 1985;1:368–70.CrossRefPubMed Trompeter RS, Lloyd BW, Hicks J, White RH, Cameron JS. Long-term outcome for children with minimal-change nephrotic syndrome. Lancet. 1985;1:368–70.CrossRefPubMed
14.
Zurück zum Zitat Lewis MA, Baildom EM, Davis N, Houston IB, Postlethwaite RJ. Nephrotic syndrome: From toddlers to twenties. Lancet. 1989;1:255–9.CrossRefPubMed Lewis MA, Baildom EM, Davis N, Houston IB, Postlethwaite RJ. Nephrotic syndrome: From toddlers to twenties. Lancet. 1989;1:255–9.CrossRefPubMed
15.
Zurück zum Zitat Schwartz MW, Schwartz GJ, Cornfeld D. A 16 year follow up study of 163 children with nephrotic syndrome. Pediatrics. 1974;54:548–52. Schwartz MW, Schwartz GJ, Cornfeld D. A 16 year follow up study of 163 children with nephrotic syndrome. Pediatrics. 1974;54:548–52.
16.
Zurück zum Zitat Zidar N, Cavic MA, Kenda RB, Ferluga D. Unfavorable course of minimal change nephrotic syndrome in children with intrauterine growth retardation. Kidney Int. 1998;54:1320–3.CrossRefPubMed Zidar N, Cavic MA, Kenda RB, Ferluga D. Unfavorable course of minimal change nephrotic syndrome in children with intrauterine growth retardation. Kidney Int. 1998;54:1320–3.CrossRefPubMed
17.
Zurück zum Zitat Sheu JN, Jeun-Horng C. Minimal Change Nephrotic Syndrome in Children with Intrauterine Growth Retardation. Am J Kidney Dis. 2001;37:909–14.CrossRefPubMed Sheu JN, Jeun-Horng C. Minimal Change Nephrotic Syndrome in Children with Intrauterine Growth Retardation. Am J Kidney Dis. 2001;37:909–14.CrossRefPubMed
19.
Zurück zum Zitat Barker DJP, Winter PD, Osmond C, Simmons SJ. Weight in infancy and death from ischemic heart disease. Lancet. 1989;2:577–80.CrossRefPubMed Barker DJP, Winter PD, Osmond C, Simmons SJ. Weight in infancy and death from ischemic heart disease. Lancet. 1989;2:577–80.CrossRefPubMed
20.
Zurück zum Zitat Hales CN, Barker DJP, Clark PMS, Cox LJ, Fall C, Osmond C, Winter PD. Fetal and infant growth and impaired glucose tolerance at age 64. BMJ. 1991;303:1019–22.CrossRefPubMedPubMedCentral Hales CN, Barker DJP, Clark PMS, Cox LJ, Fall C, Osmond C, Winter PD. Fetal and infant growth and impaired glucose tolerance at age 64. BMJ. 1991;303:1019–22.CrossRefPubMedPubMedCentral
21.
Zurück zum Zitat Hinchliffe SA, Lynch MR, Sargent PH, Howard CV, Van Velzen D. The effect of intrauterine growth retardation on the development of renal nephrons. Br J Obstet Gynaecol. 1992;99:296–301.CrossRefPubMed Hinchliffe SA, Lynch MR, Sargent PH, Howard CV, Van Velzen D. The effect of intrauterine growth retardation on the development of renal nephrons. Br J Obstet Gynaecol. 1992;99:296–301.CrossRefPubMed
22.
Zurück zum Zitat Hughson M, Farris AB 3rd, Douglas-Denton R, Hoy WE, Bertram JF. Glomerular number and size in autopsy kidneys: the relationship to birth weight. Kidney Int. 2003;63:2113–22.CrossRefPubMed Hughson M, Farris AB 3rd, Douglas-Denton R, Hoy WE, Bertram JF. Glomerular number and size in autopsy kidneys: the relationship to birth weight. Kidney Int. 2003;63:2113–22.CrossRefPubMed
23.
Zurück zum Zitat Manalich R, Reyes L, Herrera M, Melendi C, Fundora I. Relationship between weight at birth and the number and size of renal glomeruli in humans: a histomorphometric study. Kidney Int. 2000;58:770–3.CrossRefPubMed Manalich R, Reyes L, Herrera M, Melendi C, Fundora I. Relationship between weight at birth and the number and size of renal glomeruli in humans: a histomorphometric study. Kidney Int. 2000;58:770–3.CrossRefPubMed
24.
Zurück zum Zitat Matsukura H, Inaba S, Shinozaki K, Yanagihara T, Hara M, Higuchi A, Takada T, Tanizawa T, Miyawaki T. Influence of prolonged corticosteroid therapy on the outcome of steroid-responsive nephrotic syndrome. Am J Nephrol. 2001;21:362–7.CrossRefPubMed Matsukura H, Inaba S, Shinozaki K, Yanagihara T, Hara M, Higuchi A, Takada T, Tanizawa T, Miyawaki T. Influence of prolonged corticosteroid therapy on the outcome of steroid-responsive nephrotic syndrome. Am J Nephrol. 2001;21:362–7.CrossRefPubMed
25.
Zurück zum Zitat Motoyama O, Iitaka K. Final height in children with steroid-sensitive nephrotic syndrome. Pediatrics Int. 2007;49:623–5.CrossRef Motoyama O, Iitaka K. Final height in children with steroid-sensitive nephrotic syndrome. Pediatrics Int. 2007;49:623–5.CrossRef
27.
Zurück zum Zitat Calderon-Margalit R, Golan E, Twig G, Leiba A, Tzur D, Afek A, et al. History of childhood kidney disease and risk of adult end-stage renal disease. N Engl J Med. 2018;378(5):428–38.CrossRefPubMed Calderon-Margalit R, Golan E, Twig G, Leiba A, Tzur D, Afek A, et al. History of childhood kidney disease and risk of adult end-stage renal disease. N Engl J Med. 2018;378(5):428–38.CrossRefPubMed
28.
Zurück zum Zitat Leonard MB, Feldman HI, Shults J, Zemel BS, Foster BJ, Stallings VA. Long-term, high-dose glucocorticoids and bone mineral content in childhood glucocorticoid sensitive nephrotic syndrome. N Engl J Med. 2004;351:868–75.CrossRefPubMed Leonard MB, Feldman HI, Shults J, Zemel BS, Foster BJ, Stallings VA. Long-term, high-dose glucocorticoids and bone mineral content in childhood glucocorticoid sensitive nephrotic syndrome. N Engl J Med. 2004;351:868–75.CrossRefPubMed
29.
Zurück zum Zitat Weng FL, Shults J, Herskovitz RM, Zemel BS, Leonard MB. Vitamin D insufficiency in steroid-sensitive nephrotic syndrome in remission. Pediatr Nephrol. 2005;20:56–63.CrossRefPubMed Weng FL, Shults J, Herskovitz RM, Zemel BS, Leonard MB. Vitamin D insufficiency in steroid-sensitive nephrotic syndrome in remission. Pediatr Nephrol. 2005;20:56–63.CrossRefPubMed
30.
Zurück zum Zitat Truthmann J, Mensink GBM, Bosy-Westphal A, Scheidt-Nave C, Schienkiewitz A. Metabolic Health in Relation to Body Size: Changes in Prevalence over Time between 1997-99 and 2008-11 in Germany. PLoS One. 2016;11(11):e0167159.CrossRefPubMedPubMedCentral Truthmann J, Mensink GBM, Bosy-Westphal A, Scheidt-Nave C, Schienkiewitz A. Metabolic Health in Relation to Body Size: Changes in Prevalence over Time between 1997-99 and 2008-11 in Germany. PLoS One. 2016;11(11):e0167159.CrossRefPubMedPubMedCentral
32.
Zurück zum Zitat Elmaoğulları S, Tepe D, Uçaktürk SA, Karaca Kara F, Demirel F. Prevalence of dyslipidemia and associated factors in obese children and adolescents. J Clin Res Pediatr Endocrinol. 2015;7(3):228–34.CrossRefPubMedPubMedCentral Elmaoğulları S, Tepe D, Uçaktürk SA, Karaca Kara F, Demirel F. Prevalence of dyslipidemia and associated factors in obese children and adolescents. J Clin Res Pediatr Endocrinol. 2015;7(3):228–34.CrossRefPubMedPubMedCentral
33.
Zurück zum Zitat Korsten-Reck U, Kromeyer-Hauschild K, Korsten K, Baumstark MW, Dickhuth HH, Berg A. Frequency of secondary dyslipidemia in obese children. Vasc Health Risk Manag. 2008;4(5):1089–94.CrossRefPubMedPubMedCentral Korsten-Reck U, Kromeyer-Hauschild K, Korsten K, Baumstark MW, Dickhuth HH, Berg A. Frequency of secondary dyslipidemia in obese children. Vasc Health Risk Manag. 2008;4(5):1089–94.CrossRefPubMedPubMedCentral
34.
Zurück zum Zitat Mérouani A, Lévy E, Mongeau J-J, Robitaille P, Lambert M, Delvin EE. Hyperlipidemic profiles during remission in childhood idiopathic nephrotic syndrome. Clin Biochem. 2003;36:571–4.CrossRefPubMed Mérouani A, Lévy E, Mongeau J-J, Robitaille P, Lambert M, Delvin EE. Hyperlipidemic profiles during remission in childhood idiopathic nephrotic syndrome. Clin Biochem. 2003;36:571–4.CrossRefPubMed
35.
Zurück zum Zitat Kniazewska MH, Obuchowics AK, Wielkoszyński T, Zmudzińska-Kitczak J, Urban K, Marek M, Witanowska J, Sieroń-Stołtny K. Atherosclerosis risk factors in young patients formerly treated for idiopathic nephrotic syndrome. Pediatr Nephrol. 2009;24:549–54.CrossRefPubMed Kniazewska MH, Obuchowics AK, Wielkoszyński T, Zmudzińska-Kitczak J, Urban K, Marek M, Witanowska J, Sieroń-Stołtny K. Atherosclerosis risk factors in young patients formerly treated for idiopathic nephrotic syndrome. Pediatr Nephrol. 2009;24:549–54.CrossRefPubMed
36.
Zurück zum Zitat Emma F, Sesto A, Rizzoni G. Long-term linear growth of children with severe steroid responsive nephrotic syndrome. Pediatr Nephrol. 2003;18:783–8.CrossRefPubMed Emma F, Sesto A, Rizzoni G. Long-term linear growth of children with severe steroid responsive nephrotic syndrome. Pediatr Nephrol. 2003;18:783–8.CrossRefPubMed
37.
Zurück zum Zitat Foote KD, Brocklebank JT, Meadow SR. Height attainment in children with steroid responsive nephrotic syndrome. Lancet. 1985;2:917–9.CrossRefPubMed Foote KD, Brocklebank JT, Meadow SR. Height attainment in children with steroid responsive nephrotic syndrome. Lancet. 1985;2:917–9.CrossRefPubMed
38.
Zurück zum Zitat Foster BJ, Shults J, Zemel BS, Leonard MB. Risk factors for glucocorticoid-induced obesity in children with steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2006;21:973–80.CrossRefPubMed Foster BJ, Shults J, Zemel BS, Leonard MB. Risk factors for glucocorticoid-induced obesity in children with steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2006;21:973–80.CrossRefPubMed
39.
Zurück zum Zitat Salsano ME, Graziano L, Luongo I, Pilla P, Giordano M, Lama G. Atopy in childhood idiopathic nephrotic syndrome. Acta paediatrica. 2007;96:561–6.CrossRefPubMed Salsano ME, Graziano L, Luongo I, Pilla P, Giordano M, Lama G. Atopy in childhood idiopathic nephrotic syndrome. Acta paediatrica. 2007;96:561–6.CrossRefPubMed
40.
Zurück zum Zitat Meadow SR, Sarsfield JK, Scott DG, Rajah SM. Steroid-responsive nephrotic syndrome and allergy: immunological studies. Arch Dis Child. 1981;56:517–24.CrossRefPubMedPubMedCentral Meadow SR, Sarsfield JK, Scott DG, Rajah SM. Steroid-responsive nephrotic syndrome and allergy: immunological studies. Arch Dis Child. 1981;56:517–24.CrossRefPubMedPubMedCentral
41.
Zurück zum Zitat Hu JF, Liu YZ. Elevated serum IgE levels in children with nephrotic syndrome, a steroid-resistant sign? Nephron. 1990;54:275.CrossRefPubMed Hu JF, Liu YZ. Elevated serum IgE levels in children with nephrotic syndrome, a steroid-resistant sign? Nephron. 1990;54:275.CrossRefPubMed
42.
Zurück zum Zitat Abdel-Havez M, Shimada M, Lee PY, Johnson RJ, Garin EH. Idiopathic nephrotic syndrome and atopy. Is there a common link? Am J Kidney Dis. 2009;54(5):945–53.CrossRef Abdel-Havez M, Shimada M, Lee PY, Johnson RJ, Garin EH. Idiopathic nephrotic syndrome and atopy. Is there a common link? Am J Kidney Dis. 2009;54(5):945–53.CrossRef
43.
Zurück zum Zitat Mundy K, Nichols E, Lindsey J. Socioeconomic disparities in cataract prevalence, characteristics, and management. Semin Ophthalmol. 2016;31(4):358–63.CrossRefPubMed Mundy K, Nichols E, Lindsey J. Socioeconomic disparities in cataract prevalence, characteristics, and management. Semin Ophthalmol. 2016;31(4):358–63.CrossRefPubMed
44.
Zurück zum Zitat Brocklebank JT, Harcourt RB, Meadow SR. Corticosteroid-induced cataracts in idiopathic nephrotic syndrome. Arch Dis Childhood. 1982;53:30–4. Brocklebank JT, Harcourt RB, Meadow SR. Corticosteroid-induced cataracts in idiopathic nephrotic syndrome. Arch Dis Childhood. 1982;53:30–4.
45.
Zurück zum Zitat Bogdanovic R, Banicevic M, Cvoric A. Testicular function following cyclophosphamide treatment for childhood nephrotic syndrome: long-term follow-up study. Pediatr Nephrol. 1990;4:451–4.CrossRefPubMed Bogdanovic R, Banicevic M, Cvoric A. Testicular function following cyclophosphamide treatment for childhood nephrotic syndrome: long-term follow-up study. Pediatr Nephrol. 1990;4:451–4.CrossRefPubMed
46.
Zurück zum Zitat Callis L, Nieto J, Vila A, Rende J. Chlorambucil treatment in minimal lesion nephrotic syndrome: a reappraisal of its gonadal toxicity. J Pediatr. 1980;97:653–6.CrossRefPubMed Callis L, Nieto J, Vila A, Rende J. Chlorambucil treatment in minimal lesion nephrotic syndrome: a reappraisal of its gonadal toxicity. J Pediatr. 1980;97:653–6.CrossRefPubMed
47.
Zurück zum Zitat Guesry P, Lenoir G, Broyer M. Gonadal effects of chlorambucil given to prepubertal and pubertal boys for nephrotic syndrome. J Pediatr. 1978;92:299–303.CrossRefPubMed Guesry P, Lenoir G, Broyer M. Gonadal effects of chlorambucil given to prepubertal and pubertal boys for nephrotic syndrome. J Pediatr. 1978;92:299–303.CrossRefPubMed
48.
Zurück zum Zitat Penso J, Lippe B, Ehrlich R, Smith FG. Testicular function in prepubertal and pubertal male patients treated with cyclophosphamide for nephrotic syndrome. J Pediatr. 1974;84:831–6.CrossRefPubMed Penso J, Lippe B, Ehrlich R, Smith FG. Testicular function in prepubertal and pubertal male patients treated with cyclophosphamide for nephrotic syndrome. J Pediatr. 1974;84:831–6.CrossRefPubMed
49.
Zurück zum Zitat Rapola J, Koskimies O, Huttunen NP, Floman P, Vilska J, Hallmann N. Cyclophoshamide and the pubertal testis. Lancet. 1973;1:98–9.CrossRefPubMed Rapola J, Koskimies O, Huttunen NP, Floman P, Vilska J, Hallmann N. Cyclophoshamide and the pubertal testis. Lancet. 1973;1:98–9.CrossRefPubMed
50.
Zurück zum Zitat Basu B, Babu BG, Mahapatra TK. Long-term efficacy and safety of common steroid-sparing agents in idiopathic nephrotic syndrome. Clin Exp Nephrol. 2017;21(1):143–51.CrossRefPubMed Basu B, Babu BG, Mahapatra TK. Long-term efficacy and safety of common steroid-sparing agents in idiopathic nephrotic syndrome. Clin Exp Nephrol. 2017;21(1):143–51.CrossRefPubMed
Metadaten
Titel
The long-term outcome of childhood nephrotic syndrome in Germany: a cross-sectional study
verfasst von
Malik Aydin
Ingo Franke
Lisa Kurylowicz
Rainer Ganschow
Michael Lentze
Mark Born
Rebekka Hagemann
Publikationsdatum
05.02.2019
Verlag
Springer Singapore
Erschienen in
Clinical and Experimental Nephrology / Ausgabe 5/2019
Print ISSN: 1342-1751
Elektronische ISSN: 1437-7799
DOI
https://doi.org/10.1007/s10157-019-01696-8

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