Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome.

OBJECTIVE Long-term outcome of steroid-sensitive idiopathic nephrotic syndrome (SSNS) in children is usually considered benign, although data on follow-up into adulthood are scarce. The aim of this study was to investigate adults who had childhood SSNS regarding their relapse rate, growth, and renal and extrarenal morbidity. STUDY DESIGN Adult patients (n=42, 26 males) were evaluated at a median age of 28.0 (18.1 to 46.9) years and a median follow-up of 22.0 (2.9 to 39.0) years since diagnosis. RESULTS Fourteen of 42 (33%) patients relapsed in adulthood. The number of relapses during childhood and adolescence and a complicated course-administration of steroid-sparing medication such as cyclophosphamide, chlorambucil, and cyclosporin A-were identified as risk factors. Final adult height (median SD score -0.4, range -3.3 to +1.3) and body mass index (BMI) were normal. Renal function was normal in all patients, and overall morbidity was low. Only eight patients (three males) had children. Cytotoxic therapy was identified as a major factor contributing to childlessness. CONCLUSION Relapses in adulthood were common in pediatric patients with SSNS. Growth and renal function were normal, and overall morbidity was low. Yet, transition to an adult nephrologist is recommended for all children with relapsing SSNS.

[1]  M. Shearn,et al.  VIRUS-LIKE STRUCTURES IN SJÖGREN'S SYNDROME , 1970 .

[2]  J. Wilkinson,et al.  Folic acid in the treatment of pernicious anaemia. , 1946, Lancet.

[3]  J W Overstreet,et al.  Sperm morphology, motility, and concentration in fertile and infertile men. , 2001, The New England journal of medicine.

[4]  T. Barratt,et al.  Alternative treatment to corticosteroids in steroid sensitive idiopathic nephrotic syndrome. , 1994, Archives of disease in childhood.

[5]  R. Bogdanović,et al.  Pituitary-gonadal function in women following cyclophosphamide treatment for childhood nephrotic syndrome: long-term follow-up study , 1990, Pediatric Nephrology.

[6]  R. Coppo,et al.  Dose-dependent effects of deflazacort and prednisone on growth and skeletal maturation. , 1993, British journal of rheumatology.

[7]  P. Laippala,et al.  Normal growth of prepubertal nephrotic children during long‐term treatment with repeated courses of prednisone , 1998, Acta paediatrica.

[8]  L. Molinari,et al.  Physical growth of Swiss children from birth to 20 years of age. First Zurich longitudinal study of growth and development. , 1989, Helvetica paediatrica acta. Supplementum.

[9]  K. Fairley,et al.  RETURN OF SPERMATOGENESIS AFTER STOPPING CYCLOPHOSPHAMIDE THERAPY , 1975, The Lancet.

[10]  Neville C. Davis,et al.  NEPHROTIC SYNDROME: FROM TODDLERS TO TWENTIES , 1989, The Lancet.

[11]  M. Preece,et al.  Growth and endocrine function in steroid sensitive nephrotic syndrome. , 1988, Archives of disease in childhood.

[12]  R. White,et al.  LONG-TERM OUTCOME FOR CHILDREN WITH MINIMAL-CHANGE NEPHROTIC SYNDROME , 1985, The Lancet.

[13]  L. Strong,et al.  Effects of treatment on fertility in long-term survivors of childhood or adolescent cancer. , 1987 .

[14]  Paul Landais,et al.  Steroid-sensitive nephrotic syndrome: from childhood to adulthood. , 2003, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[15]  A. Elzouki,et al.  Long-term, Small Dose Prednisone Therapy in Frequently Relapsing Nephrotic Syndrome of Childhood , 1988, Clinical pediatrics.

[16]  R. Mazess,et al.  Effect of prednisone on growth and bone mineral content in childhood glomerular disease. , 1978, American journal of diseases of children.

[17]  M. Kemper,et al.  Health-related quality of life and psychosocial adjustment in steroid-sensitive nephrotic syndrome. , 2004, The Journal of pediatrics.

[18]  J. Tobin,et al.  Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. , 1997, Journal of the American Society of Nephrology : JASN.

[19]  U. Nieminen,et al.  Letter: Cervical screening. , 1974, Lancet.

[20]  J. Berns,et al.  Steroid-responsive nephrotic syndrome of childhood: a long-term study of clinical course, histopathology, efficacy of cyclophosphamide therapy, and effects on growth. , 1987, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[21]  M. Kiss,et al.  Gonadal function in male adolescents and young males with juvenile onset systemic lupus erythematosus. , 2002, The Journal of rheumatology.

[22]  M. Brandis,et al.  Acute leukemia after cytotoxic treatment for nonmalignant disease in childhood , 1981, European Journal of Pediatrics.

[23]  J. Ehrich,et al.  A meta-analysis of cytotoxic treatment for frequently relapsing nephrotic syndrome in children , 2001, Pediatric Nephrology.

[24]  J. Ehrich,et al.  Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children , 1993, European Journal of Pediatrics.

[25]  K. Fairley,et al.  Sterility and testicular atrophy related to cyclophosphamide therapy. , 1972, Lancet.

[26]  V. Koch,et al.  Growth in steroid-responsive nephrotic syndrome: a study of 85 pediatric patients , 2003, Pediatric Nephrology.

[27]  G. Rizzoni,et al.  Long-term linear growth of children with severe steroid-responsive nephrotic syndrome , 2003, Pediatric Nephrology.

[28]  See Appendix Early identification of frequent relapsers among children with minimal change nephrotic syndrome. A report of the International Study of Kidney Disease in Children. , 1982, The Journal of pediatrics.

[29]  D. Bockenhauer,et al.  The risk of cardiovascular disease in adults who have had childhood nephrotic syndrome , 2004, Pediatric Nephrology.

[30]  B. Ramot,et al.  POSSIBLE T-LYMPHOCYTE ORIGIN OF REED-STERNBERG CELLS , 1974 .

[31]  R. Bogdanović,et al.  Testicular function following cyclophosphamide treatment for childhood nephrotic syndrome: long-term follow-up study , 1990, Pediatric Nephrology.

[32]  J. Rapola,et al.  Long-term outcome of primary nephrotic syndrome. , 1982, Archives of disease in childhood.

[33]  H. Yap,et al.  Efficacy and safety of one year of growth hormone therapy in steroid-dependent nephrotic syndrome. , 1997, The Journal of pediatrics.

[34]  E. Kuwertz-Broeking,et al.  Recurrence of severe steroid dependency in cyclosporin A-treated childhood idiopathic nephrotic syndrome. , 2004, Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.

[35]  M. Preece,et al.  Oxandrolone for delayed puberty in boys taking long-term steroid therapy for renal disease , 1990, Pediatric nephrology (Berlin, West).

[36]  T. Neuhaus,et al.  Long-term low-dose cyclosporin A in steroid dependent nephrotic syndrome of childhood , 1992, European Journal of Pediatrics.

[37]  Miles Be,et al.  Letter: Serum-phenytoin. , 1976 .

[38]  J. Shults,et al.  Long-term, high-dose glucocorticoids and bone mineral content in childhood glucocorticoid-sensitive nephrotic syndrome. , 2004, The New England journal of medicine.

[39]  J M Tanner,et al.  Standards for Children's Height at Ages 2-9 Years Allowing for Height of Parents , 1970, Archives of disease in childhood.