No abstract

Federal Universitv of P a r a d . Curitiba. Brasll.Enciocrinol. Sheba Med. Ctr . . Tel-Hashomer. I s r a e l . Tel Aviv Univ. Chronic e m s u r e -t o sex steroids d&ing childhccd accelerates s k e l e t a l maturation and may corn-crromise f i n a l height. The growth of 26 children (18 g i r l s , 8 Soys) disease-free of ACC f o r maze than 1 yr were reviewed. The i r i t i a l c l i n i c a l siw appeared a t ~. 9 2 2.8 y r and t h e diap.osis wa.; made a t 3.6 2 2.8 yr. Time of follow-up a f t e r surgery was 6.2 2 3 . 5 yr (range 1 . 5 -1 3 . 0 ) . 50% had adrenogenital sp-drome (AS), 38% mixed syndrome (US: Gushing's plus AS), 8% no endocrine s m t o m s and 4% C~s h l n g syndrow. A t t h e time of d i a g o s i s t h e mean H-SDS was higher than t a r g e t height-SDS (W-SDS; p < 0.0001). H-SDS was not d i f f e r e n t between AS and M S groups ( p > 0 . 1 ) . Bone age 18.9; 6.2 53.6) was grearer than helght age (HA; 4.2 2 2 . 7 ) and chronological age (CA; 3 . 8 2 2.9) ( p < 0.05), whereas :iA m d CA were not d i f f e r e n t . B A advcnced more than CA i n t h e 1st year followino tumor removal: i n the 2nd year and t h e r e a f t e r we observed catch-doh?l arohTh t h a t was m r e intense i n B A +Am i n H-SDS. Only one patient of t k e s e r i e s , with no B A catch-down, developed t r u e precocious puberty. I n l t i a l predicted adult height (?.AH; Bayley & Tinneau) of 10 patients was lower than tar-t height (TH; p < 0:Ol); however, i n t h e l a s t evaluation (16 p t s ) PAH and W were not d i f f e r e z t ( p > 0 . 1 ) . I n conclssion: a ) children exposed t o with/without glucocorticoid for a l i q i t e d w r i @ of time usuallv increase B A more than H-SDS: b ) a f t e r tumor exclsion both 5.4Medical Schcol, I s r a e l . Insulin l i k e growth factor-1 (1Gr"-1) increases during normal adolescence and in CPP secondary t o t h e r i s e in sex s t e r o i d s and GH.Treatment of CP? using GnRH malog suppresses GH a s well a s gonadotropins. W e e x z~i n e d IGF-1 and its m j o r bmnding protein-IGF3P-3 i n sera of t e n g i r l s d i a g o s e d with CPP, before and during the f i r s t 3 months of GnRH analog therapy. Serum IGF-1 w a s increased i n ~a t i e n t s with CPP a s comwared with controls (48.8 + 6 . 5 vs 23.1 + 41 9 m l / L , p < 0.01 ) . G& analog therapy ca&ed serum E2 levels t o return t o p r e p b e r t a l l e v e l s i n a l l 10 p a t i e n t s , whereas semi IGF-1 l e v e l s decreased minimally a f t e r one (43.2 ?: 5.6 ~m l / L ) , two (42.3 t 6.4 m l / L ) , m d three ( 4 4 . 1 2 7.2 m l / L ) months of therapy. Serum IGFBP-3 concentratiors measured using IRW were a l s o higher i n CPP c o m p~e d with controls (4.7 + 0.37 vs 3.7 t 0.42 mg/L w < 0.01). These d~f f e r e n c e s were a l s o evident when a l l IGF bincino proteins' were measwed by Western ligand blotting. GnXa therap? cacsed a small md insignificant decrease i n serum IGFBP-3 levels a f t e r one (4.57 2 0.33 mg/L), two (4.48 t 0.4 mg/L) and three (4.42 t 0 . 3 mo/L) months of therawv. This...