SOD is characterized by optic nerve hypoplasia and a variety of endocrine deficiencies. In addition, forebrain malformations are present in most SOD patients. Hormonal disorders are present in some SOD patients which may be of hypothalamic origin and need to be investigated systematically.
The Radcli/fe I t~h a r v .OXFORD. SEXUALLY DlhlORPBIC CHANGES 1N LH SECRETION DURING CHILDHOOD AND PUBERTY.We have examined changes in the pulsatile pattern of L H secretion that occur during childhood and puherty, using spectral analysis and a novel distribution method. 24 hour profiles of LH concentr;ttion wcre taken from 78 children aged 4.2-15.6yrs and 6 adult men. Relativc Fourier transforms were derived, and a normalised estimate of the concentration below which each profile spent 58(OC5), 50%(OC50) and 95%(OC95) of the total time of the profile was calculated. In children aged 4.2-9.8yrs, LH periodicity was 140-200 minutes. T h e OC5 in children aged 4.2-6.9yrs was greater ( 0.05) than in children a g e d 7.1-9.8yrs. In prepubertal children aged l~.~-1 4 . 8 y r s , L H periodicity decreased to 100-120 mins (pt0.025), and OC50 and OC95 values increased (p=0.01, 0.003). LH eriodicity in puherty was 120-160 minutes. In early puberty girls had greater 6 C5, OC50 and OC95 (p=0.001) and boys had greater OG15 (p=O.O?), compared to prepubertal children. In late puberry O C values did not change for girls hut OC5 and OC95 were greater for boys (p=0.001) compar5d with early puherty. O C values for both sexes in late puberty did not dltfer from the adult men. CONCLUSIONS The decrease in LI-1 secretion that occurs after the neonatal period continues as late as 4 years of age. Before the onset of puberty there was a n increase in pulse frequency with an increase in peak but not baseline L H values. A mature pattern of LH secretion with elevated peak and baseline values was attained in early puberty in girls, but later in boys. This pattern may be important for fertility, and is the first time that sexual dimorphism has been described in the control of the onset of puberty. monthly intervals u p t o 3 years a f t e r surgery & radiotherapy + chemothe~;tpy. We have compared results with those obtained in 26 short normal controls (SN). T h e profiles were analysed by a distribution method. We obseried discrepancies between peaks of spontaneous and stimulated GI1 in B T hut not SN. T h e ratio was significantly greater in all B T groups compared to SN (p<0.05). 7:d x~ chem surg SN N 26 10 7 26 Mean sem) 2.62 (0.73) 2.61 (0.51) 3.03 (1.14) 3.47 (1.03) 0.99 (0.19) Peak AH t o 17T was not influenced either by the degree of hypoglycaemia (r=0.1, p=0.65) nor by days off dexamethasone therapy in those children assessed before radiotherapy (r=0.24, p=0.37). In 14 BT children in whom G H R H tests at 2 doses (I p g k g and 0.1 ~g l k g ) were performed, correlation with spontaneous edks wds grcater with the low dose (r=0.7, p=0.005) than the high dose ( r = L , p=b.26). A spontaneousistimulated ratio approaching 1.0 suggests intact hypothalamic G H R H and somatostatin (SS) tone. ITT results reflect SS tone which was abnormal In children with BT. 24hr profile results reflect G H R H secretion and low dose G H R H tests identified this.NA Bri&es. P C Hi~ldmarsl~, DR Matrlrews*, CGD Brook Departme111 ofErrdocri~~ology, Middlesex ...
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