Abstract
Although steroid-free remission can usually be achieved with cyclosporin A (CsA) in patients with steroid-dependent nephrotic syndrome (SDNS), some CsA-treated patients require long-term steroid therapy. Data on growth in these patients are scarce. Sixty-four boys with SDNS receiving long-term CsA and steroid therapy were retrospectively analyzed. During the 10-year follow-up period, height standard deviation score (HSDS) remained in the normal range in 47 patients but was below −2 SD in 17 patients. The occurrence of growth retardation was influenced by height at diagnosis and the number of relapses. Thirty patients were followed for at least 3 years before and after age 12. The decrease in HSDS per year of disease in patients older than 12 years was twice that observed in children younger than 12. However, adult height was ≤ −2 SD in only two of the 14 patients reaching adult height, reflecting potential catch-up growth during late puberty. Careful monitoring of growth is recommended, given than up to 25% of patients experienced severe growth retardation during the course of their disease.
Similar content being viewed by others
References
Niaudet P, Habib R (1994) Cyclosporine in the treatment of idiopathic nephrosis. J Am Soc Nephrol 5:1049–1056
Hulton SA, Neuhaus TJ, Dillon MJ, Barratt TM (1994) Long-term cyclosporin A treatment of minimal-change nephrotic syndrome of childhood. Pediatr Nephrol 8:401–403
Niaudet P, Broyer M, Habib R (1991) Treatment of idiopathic nephrotic syndrome with cyclosporin A in children. Clin Nephrol 35(Suppl 1):S31–S36
Kemper MJ, Kuwertz-Broeking E, Bulla M, Mueller-Wiefel DE, Neuhaus TJ (2004) Recurrence of severe steroid dependency in cyclosporin A-treated childhood idiopathic nephrotic syndrome. Nephrol Dial Transplant 19:1136–1141
Blodgett FM, Burgin L, Iezzoni D, Gribetz D, Talbot NB (1956) Effects of prolonged cortisone therapy on the statural growth, skeletal maturation and metabolic status of children. N Engl J Med 254:636–641
Emma F, Sesto A, Rizzoni G (2003) Long-term linear growth of children with severe steroid-responsive nephrotic syndrome. Pediatr Nephrol 18:783–788
Foote KD, Brocklebank JT, Meadow SR (1985) Height attainment in children with steroid-responsive nephrotic syndrome. Lancet 2:917–919
Lai HC, FitzSimmons SC, Allen DB, Kosorok MR, Rosenstein BJ, Campbell PW, Farrell PM (2000) Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. N Engl J Med 342:851–859
Lam CN, Arneil GC (1968) Long-term dwarfing effects of corticosteroid treatment for childhood nephrosis. Arch Dis Child 43:589–594
Simon D, Fernando C, Czernichow P, Prieur AM (2002) Linear growth and final height in patients with systemic juvenile idiopathic arthritis treated with long-term glucocorticoids. J Rheumatol 29:1296–1300
Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr (1997) 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 8:769–776
Saha MT, Laippala P, Lenko HL (1998) Normal growth of prepubertal nephrotic children during long-term treatment with repeated courses of prednisone. Acta Paediatr 87:545–548
Matsukura H, Inaba S, Shinozaki K, Yanagihara T, Hara M, Higuchi A, Takada T, Tanizawa T, Miyawaki T (2001) Influence of prolonged corticosteroid therapy on the outcome of steroid-responsive nephrotic syndrome. Am J Nephrol 21:362–367
Motoyama O, Iitaka K (2007) Final height in children with steroid-sensitive nephrotic syndrome. Pediatr Int 49:623–625
Rüth EM, Kemper MJ, Leumann EP, Laube GF, Neuhaus TJ (2005) Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr 147:202–207
Rees L, Greene SA, Adlard P, Jones J, Haycock GB, Rigden SP, Preece M, Chantler C (1988) Growth and endocrine function in steroid sensitive nephrotic syndrome. Arch Dis Child 63:484–490
Broyer M, Niaudet P, Habib R (1992) Treatment of idiopathic nephrotic syndrome with cyclosporin in children. Preliminary results of growth data in long-term use. J Autoimmun 5:325–331
Kano K, Nishikura K, Hoshi M, Yamada Y, Arisaka O (2001) Linear growth of low-dose cyclosporin a therapy in children with steroid-dependent nephrotic syndrome. Nephron 87:293–294
Berard E, Broyer M, Dehennault M, Dumas R, Eckart P, Fischbach M, Loirat C, Martinat L (2005) Corticosensitive nephrotic syndrome (or nephrosis) in children. Therapeutic guideline proposed by the Pediatric Society of Nephrology. Nephrol Ther 1:150–156
Letavernier B, Letavernier E, Leroy S, Baudet-Bonneville V, Bensman A, Ulinski T (2008) Prediction of high-degree steroid dependency in pediatric idiopathic nephrotic syndrome. Pediatr Nephrol 23:2221–2226
Broyer M, Meyrier, A, Niaudet, P, Habib R (1992) Minimal changes and focal and segmental glomerular sclerosis. In: Cameron J, Davison, MA, Grünfeld, JP, Kerr DN, Ritz E, Winearls CG (eds). Oxford Textbook of Clinical Nephrology, Oxford Medical, p 298
Tanner JM, Goldstein H, Whitehouse RH (1970) Standards for children's height at ages 2–9 years allowing for heights of parents. Arch Dis Child 45:755–762
Sempé M, Pedron G, Roy-Pernot MP (1979) Auxologie, méthode et séquences. Théraplix, Paris, p 205
Leroy B, Lefort F (1971) The weight and size of newborn infants at birth. Rev Fr Gynecol Obstet 66:391–396
Kashtan C, Melvin T, Kim Y (1988) Long-term follow-up of patients with steroid-dependent, minimal change nephrotic syndrome. Clin Nephrol 29:79–85
Chesney RW, Mazess RB, Rose P, Jax DK (1978) Effect of prednisone on growth and bone mineral content in childhood glomerular disease. Am J Dis Child 132:768–772
Adhikari M, Manikkam NE, Coovadia HM (1992) Effects of repeated courses of daily steroids and of persistent proteinuria on linear growth in children with nephrotic syndrome. Pediatr Nephrol 6:4–9
Cooper MS, Blumsohn A, Goddard PE, Bartlett WA, Shackleton CH, Eastell R, Hewison M, Stewart PM (2003) 11{beta}-hydroxysteroid dehydrogenase type 1 activity predicts the effects of glucocorticoids on bone. J Clin Endocrinol Metab 88:3874–3877
Zalewski G, Wasilewska A, Zoch-Zwierz W, Chyczewski L (2008) Response to prednisone in relation to NR3C1 intron B polymorphisms in childhood nephrotic syndrome. Pediatr Nephrol 23:1073–1078
Tanaka H, Ichikawa Y, Akama H, Homma M (1989) In vivo responsiveness to glucocorticoid correlated with glucocorticoid receptor content in peripheral blood leukocytes in normal humans. Acta Endocrinol (Copenh) 121:470–476
Russcher H, Smit P, van den Akker EL, van Rossum EF, Brinkmann AO, de Jong FH, Lamberts SW, Koper JW (2005) Two polymorphisms in the glucocorticoid receptor gene directly affect glucocorticoid-regulated gene expression. J Clin Endocrinol Metab 90:5804–5810
Westeel FP, Mazouz H, Ezaitouni F, Hottelart C, Ivan C, Fardellone P, Brazier M, El Esper I, Petit J, Achard JM, Pruna A, Fournier A (2000) Cyclosporine bone remodeling effect prevents steroid osteopenia after kidney transplantation. Kidney Int 58:1788–1796
Schärer K, Essigmann HC, Schaefer F (1999) Body growth of children with steroid-resistant nephrotic syndrome. Pediatr Nephrol 13:828–834
Pope E, Krafchik BR, Macarthur C, Stempak D, Stephens D, Weinstein M, Ho N, Baruchel S (2007) Oral versus high-dose pulse corticosteroids for problematic infantile hemangiomas: a randomized, controlled trial. Pediatrics 119:e1239–1247
Sogo T, Fujisawa T, Inui A, Komatsu H, Etani Y, Tajiri H, Waki K, Shimizu Y, Nakashima S, Imagawa T, Yokota S (2006) Intravenous methylprednisolone pulse therapy for children with autoimmune hepatitis. Hepatol Res 34:187–192
Schaefer F, Veldhuis JD, Stanhope R, Jones J, Scharer K (1994) Alterations in growth hormone secretion and clearance in peripubertal boys with chronic renal failure and after renal transplantation. Cooperative Study Group of Pubertal Development in Chronic Renal Failure. J Clin Endocrinol Metab 78:1298–1306
Morris HG (1975) Growth and skeletal maturation in asthmatic children: effect of corticosteroid treatment. Pediatr Res 9:579–583
Acknowledgments
The authors thank all the attending physicians for providing data, and J.F. Hartmann, M.D., for invaluable assistance with the manuscript preparation.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Leroy, V., Baudouin, V., Alberti, C. et al. Growth in boys with idiopathic nephrotic syndrome on long-term cyclosporin and steroid treatment. Pediatr Nephrol 24, 2393–2400 (2009). https://doi.org/10.1007/s00467-009-1266-y
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-009-1266-y