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GROWTH RETARDATION AND URINARY LOSS OF GROWTH HORMONE IN CYSTINOSIS

Cystinosis is characterized by intracellular accumulation of cystine, which causes nephropathy and severe growth retardation. We report on two prepubertal patients, adequately treated with oral administration of cystamine. We performed a 24 hour growth hormone (GH) profile and measured serum IGF-1 and urinary GH. One patient (#1) was subsequently placed on GH therapy (2 IU/m2/day).We found very high levels of urinary GH and low values of IGF-1. The 24h GH profiles showed a normal number of peaks and values of mean concentration and AUCb which were low compared to those of normal prepubertal children. We suggest that the growth retardation seen in cystinosis could be partly due to the excessive loss of GH in the urinary, amounting to nearly 15% of the daily production and urinary filtration of GH. This loss may contribute to the low AUCb of the GH profiles. Treatment with GH increases growth velocity but the effect on final height is yet to be determined.