J.P. chanoinex, J.J. ~ody~,G. Van Vliet, F. Delange. Depts. of Pediatrics and Radioisotopes, St Pierre Hos- Departments of Pediatrics, Uppsala pital, Dept.of Medicine, Inst. J Bordet, Children Hos- University, and Pediatric Radiology,, 23 pital,. Free University of Brussels, Belgium. 26 Karolinska Institutet, S: t Garan's MONOMERIC CALCITONIN (CT) DEFICIENCY IN PATIENTS WITH Children's Hospital, Stockholm, Sweden. CONGENITAL NON GOITROUS HYPOTHYROIDISM (CH) DURING METHODS FOR BONE MATURITY ASSESSMENT TO EARLY INFANCY. EVALUATE PRENATAC HYPOTHYROIDISM. CT deficiency has been reported in children and adolescents with Delayed skeletal maturation in newborn infants wit Cll;bbt whether it is also deficient in CH patients during infancy congenital hypothyroidism (CH) is considered t has not been investigated. Plasma extraction of CT has much impro- reflect the degree of intrauterine hormone deficien ved the sensitivity and specificity of CT radioimmunoassays for cy. Bone development was assessed by X-ray during th the measurement of the monomeric CT form, the active form of this flrst month of life in 63 infants who had positiv hypocalcemic hormone. To further analyze CT secretion in early in- screening tests for CH. Serum hormone analysis con fancy, we measured CT and Calcium (Cn) values in I) normal infants firmed the diagnosis in 46 infants while the remainin aged 0-2 yrs (gr. NL-I, n=39) and 2-5 yrs (gr. NL-2, n=12) and 2) 17 infants were euthyroid at follow-up. Therapy wa CH infants aged 0-2 yrs (gr. CH-I, n=6) and 2-5 yrs (gr. CH-2,114) initiated in CH patients at the same age as radiogram Results are shown in the table (Mean !: SEM):( XX pC0,OI as compa- were taken: 14.5i5.7 (x*SD) vs 15.0'5.5 days. Fiv red to groups NL) radiographic methods to assess skeletal, developm gr.NL-l gr.CH-l gr.NL-2 gr .CH-2 (including Seneca1 et al. (Arch Fr Ped. 3 (1977) 4 CT(pglm1) 12.86+1.12 3.23t0.63~~ 6.48+1 .I6 1.25+0.06~~ were tested for their capacity to distinguish Ca(mgId1) 9.73+0.09 9.73t0.20 I 9.65+0.08 9.57+0.11 patients from euthyroid infante. The various meth The data show that CT values I) decrease during the first years of involved assessment of the shape and size of ossifi lifc in both normal and CH patients, and 2) are lower in CH than tion centers in the knee and ankle regions. All f in normal subjects. In conclusion: I) low but detectable values of methods revealed statistically significant retar CT are present in CH patients, indicating that functional calcito- nin secreting cells do exist in these patients; 2) the low CT levels in infants with CH as compared with normal subjects suggest that CT deficiency could have an important pathogenic role in the hypercalcemia occasionally observed in CH patients during the neonatal period.

-- -*(Introd. by M.Ritz6n). J Le er*, C.Tau*, M.Garabedian*, P.Czernichow Department of Pediatrics.Karolinska Institute, St G6rans etric Endocrinology and Diabetes Children's Hospital. Stockholm, Sweden. lbpital des Wants blades, Paris, France. 27 THYROXIN (T4) EFFECTS ON GROWTH AND HEPATIC 24 HYPERCALCIMA IN INFANTS WITH CONGENITAL -01- EPIDERMAL GROWTH FACTOR RECEPTOR (EGF) DISM (CH) RECEIVING 400 iu/d Vit D. ONTOGENY IN MICE WITH CONGENITAL HYPO- THYROIDISM (CH). Yrpercalcemia has been described in infants with M The effects of thyroid hormones on growth and development before and after treatment with lhyroxine (T4). In a group of 25 are probably mediated by specific growth factors as EGF. We have studied the infants, hypercalcemia during T4 therapy was related to vit D effects of thyroxine on growth and hepatic EGF receptor ontogeny in a supplementation (in France 1200iuld). To study further adequate mouse strain with an inherited form of CH. vit D dosage to maintain normal Ca levels, 2 groups of infants Hypothyroid mice (h/h) were treated with T4 between day 20 and day 40 were studied during the first 6 months of life. Group I (11x5) postnatally, h/h nontreated mice and euthyroid (+I+) mice served as controls. without and Group I1 (n=10) with 400 iu/d of vit D. Senm Ca Animals were killed at day 20 or day 40 postnatally. Serum thyroxine was (mg/dl +SEM) and 250HD were measured at diagnosis (

J L4 er* and P.Czernichow C. Mengreli, E .Sarafidou*, S.Pantelakis>* P*ic rnd~~rin~l~gyand Diabetes Institute of Child Health, Athens, Greece Hopital des Wants blades, Paris, France. METABOLISM OF a-FETOPROTEIN IN CONGENITAL 28 ECIOPIC THYROID TISSUE IS STILL SECRFPING 25 HYPOTHYROIDISM AFPER 5 YEARS OF LT4 EUTNENT Old data seems to indicate that ectopic thyroids involuate after several years of TSH suppression by T4 a-Fetoprotein (a-FP) levels are increased in congenital hypothy- treatment. 'Ihis work was undertaken to study the secretion of roidism (CH) for unknown reasons. To explore this, we studied the thyroid hormone (M)and thyroglobulin (Tg) by remnant thyroid half-life of a-FP during the first month of life in neonates with tissue. In 16 children with congenital hypothyroidim (CH) due CH and neonates with transient hyperthyrotropinaemia (TH), which to ectopic gland, aged 7.lt0.5 yrs (range 5-11.5 yrs), sennu we considered as the control group. Between the 3rd and 7th day TSH, FT4, FT3 and Tg were measured under adequate LT4 therapy of life a-FP was measured in 60 cases with CH with a mean gesta- (phase I) and after 4 weeks of LT4 deprivation (phase 11). tionol age of 39.W-1.5 weeks ard in 184 cases with Ttl with a mean Informed consent was obtained from the parents. gestational age of 39.3~1.9weeks. The mean log a-FP levels in Phase TSH mull FT4 pool11 FT3 poll1 the two groups were 4.35+0.39ng/ml and 3.97+0.36ng/ml respective- I 5.6tl.5 26t1 7.4fl.3 Tg a!? ly (p <0.01). Levels of ~XPwere also measured in 61 cases with I1 90.46.6' 8fl* 3.7$.3* 25+3* CH at a mean age of 29.8i12.9 days and in 37 cases with TH at a (* p < 0.001 vs period I) mean age of 36.5t13.1 days. The respective mean values of log In phase I Tg was detectable in 8/16 patients which indicates a-FP were 3.84+0.46ng/ml and 2.71+0.55ng/ml,after adjustment for that some thyroid secretion occurs in treated patients. Tg conceptional age differences (p< 6,001). The half -life of a-FP levels increase in phase I1 (range 6 to 50 nglml) which was 15.7 days for the CH group and 7.7 days for the TH group. The demonstrates in all cases the presence of actively secreting half-life of a-FP was also calculated in each of 39 cases with renmant tissue responsive to TSH stimulation. The low levels of Ch and 8 cases with TH for which there were serial measurements FT4 and FT3 may indicate some thyroid hormone production in of a-FP. The half-life of a-FP in the two groups was 12 days and patients off therapy for one month. 4.9 days, respectively. We conclude that catabolism of a-FP is In conclusion: Ectopic thyroid doesn't involuate during the 5 delayed in CH group, resulting in inceased serum levels of a-FP. first years of life. Tg measurement is an efficient tool to The lack of thyroid hormones, and especially of T3, is probably evaluate remnant thyroid tissue if the patient are investigated responsible for that. under appropriate TSH stimulation.