Electronic Resource
Springer
European journal of pediatrics
152 (1993), S. 244-249
ISSN:
1432-1076
Keywords:
Nephropathic cystinosis
;
Growth
;
Puberty
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01956154
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