Abstract
In a retrospective investigation growth and pubertal development were evaluated in 30 patients with nephropathic cystinosis. Growth was investigated during the stage of chronic renal insufficiency as well as after successful kidney transplantation and growth rates were related to kidney function. Pubertal development was evaluated in 17 patients between 12 ans 25 years of age. Prepubertal growth rates were stable in a range between −2 and −3 height velocity SDS as long as glomerular filtration rate was above 20ml/min per 1.73m2. A decrease in glomerular filtration rate below this threshold was followed by further decrease in height velocity. After kidney transplantation a significant catch-up growth was seen if immunosuppression was performed with cyclosporine A and lowdose prednisolone. This did not occur if conventional therapy with azathioprine and high-dose prednisolone was used. Onset of puberty was delayed in all patients. Gonadotropin and oestradiol levels in female patients showed normal fluctuations according to ovulatory cycles. In male patients after puberty there was an increase in gonadotropin levels above the normal range for adult men while testosterone levels remained in the low normal range. These results indicate that adult men with nephropathic cystinosis may develop hypergonadotropic hypogonadism.
Similar content being viewed by others
Abbreviations
- GFR:
-
glomerular filtration rate
- HVSDS:
-
height velocity standard deviation score
- HSDS:
-
height standard deviation score
References
Adamson MD, Anderson HC, Gahl WA (1989) Cystinosis. Seminars Nephrol 9:147–161
Aschendorff C, Offner G, Winkler L, Schirg E, Hoyer PF, Brodehl J (1990) Adult height achieved in children after kidney transplantation. Arch Dis Child 144:1138–1141
Balch CM, Morgan JM, Sterlin WA, Bradley EL, Diethelm AG (1975) Kidney transplantation in patients with end stage congenital renal disease. Am J Surg 130:303–308
Bidlingmaier F, Knorr D (1978) Oestrogens Pediatr Adolesc Endocrinol 4:43–119
Brodehl J, Ehrich JHH, Krohn HP, Offner G, Byrd D (1984) Kidney transplantation in nephropathic cystinosis. In: Brodehl J, Ehrich JHH (eds) Pediatric Nephrology, Springer, Berlin Heidelberg New York, pp 172–178
Brodehl J, Offner G, Hoyer PF, Schirg E, Pichlmayr R, Wonigeit K (1986) Cyclosporin A in pediatric kidney transplantation and its effect on posttransplantation growth. Nephron 44: 26–31
Broyer M (1981) Combined report on regular dialysis and transplantation of children in Europe 1980. Proc Eur Dial Transplant 18:60–87
Broyer M, Tete MJ, Gubler MS (1987) Late symptoms in infantile cystinosis. Pediatr Nephrol 1:519–524
Burke JR, El-Beshti MM, Maisey MN, Chantler C (1978) Hypothyreoidism in children with cystinosis. Arch Dis Child 53: 947–951
Cochat P, Drachman R, Gagnadoux MF, Pariente D, Broyer M (1986) Cerebral atrophy and nephropathic cystinosis. Arch Dis Child 61:401–403
Ehrich JHH, Stöppler L, Offner G, Brodehl J (1979) Evidence for cerebral involvement in nephropathic cystinosis. Neuropädiatrie 10:128–137
Ehrich JHH, Brodehl J, Byrd D, Hossfeld S, Hoyer PF, Leipert KP, Offner G, Wolff G (1991) Renal transplantation in 22 children with nephropathic cystinosis. Pediatr Nephrol 5:708–714
Gahl WA, Kaiser-Kupfer MJ (1987) Complications of nephropathic cystinosis after renal failure. Pediatr Nephrol 1:260–268
Gilli G, Mehls O, Wallstein B, Schärer K (1983) Prediction of adult height in children with renal insufficiency. Kidney Int 24 [Suppl 15]:48–52
Greulich WW, Pyle SJ (1957) Radiographic atlas of skeletal development of hand and wrist. 2nd edn. Stanford University Press, Stanford
Hossfeld S, Winkler L, Ehrich JHH, Offner G, Hoyer PF, Brodehl J (1989) Schilddrüsenfunktion bei Kindern mit nephropatischer Zystinose vor und nach Nierentransplantation. Monatsschr Kinderheilkd 187:186
Hoyer PF, Krohn HP, Offner G, Byrd D, Brodehl J, Wonigeit K, Pichlmayr R (1987) Renal function after kidney transplantation in children: a comparison of conventional immunosuppression with cyclosporin A. Transplantation 43:489–493
Kaiser-Kupfer MJ, Caruso RC, Minkler DS, Gahl WA (1986) Longerm ocular manifestation in nephropathic cystinosis. Arch Ophthalmol 104:706–711
Lippe B, Fine RN, Koch VH, Sherman BM (1988) Accelerated Growth following treatment of children with chronic renal failure with recombinant human growth hormone (Somatrem): a preliminary report. Acta Paediatr Scand [Suppl] 343:127–131
Mehls O, Ritz E, Hunziker EB, Tönshoff B, Heinrich U (1988) Role of growth hormone in growth failure of uraemia—Perspectives for application of recominant growth hormone. Acta Paediatrica Scand [Suppl] 343:118–126
Marshall WA, Tanner JM (1969) Percentiles for individual features of puberty. Arch Dis Child 44:291
Marshall WA, Tanner JM (1970) Percentiles for individual features of puberty. Arch Dis Child 45:13
Offner G, Hoyer PF, Jüppner H, Krohn HP, Brodehl J (1987) Somatic growth after kidney transplantation. Arch Dis Child 141:541–546
Offner G, Hoyer PF, Brodehl J, Pichlmayr R (1987) Cyclosporin A in paediatric kidney transplantion. Pediatr Nephrol 1:125–130
Offner G, Aschendorff C, Brodehl J (1991) Growth after renal transplantation — an update. Pediatr Nephrol 5:472–476
Schwartz GJ, Haycock GB, Edelmann CM (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259–263
Stolecke H (1982) Physiologie und klinischer Ablauf der Pubertät. In: Stolecke H (ed) Endokrinologie des Kindes- und Jugendalters. Springer, Berlin Heidelberg New York, pp 419–436
Tanner JM, Whitehouse RH (1976) Clinical longitudinal standards for height, weight, height velocity, weight velocity and stages of puberty. Arch Dis Child 51:170–179
Reiss RE, Kuwabara T, Schmith ML, Gahl WA (1988) Successful pregnancy despite placental cystine crystals in a woman with nephropathic cystinosis. N Engl J Med 319:223–226
Winter JSD (1974) Prepubertal and pubertal endokrinology. In: Falkner F, Tanner JM (eds) Postnatal growth. Plenum, New York, p 183
Wolff G, Ehrich JHH, Offner G, Brodehl J (1982) Psychosocial and intellectual development in 12 patients with infantile nephropatic cystinosis. Acta Paediatr Scand 71:1007–1011
Wolff G, Ehrich JHH, Offner G, Brodehl J (1984) Psychosocial problems in patients with infantile nephropatic cystinosis. In: Brodehl J, Ehrich JHH (eds) Pediatric nephrology. Springer, Berlin Heidelberg New York, pp 188–191
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Winkler, L., Offner, G., Krull, F. et al. Growth and pubertal development in nephropathic cystinosis. Eur J Pediatr 152, 244–249 (1993). https://doi.org/10.1007/BF01956154
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01956154