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Aims: To evaluate therapy and dose adjustments in patients with congenital hypothyroidism (CH), longitudinally followed up until 16 years old, according to aetiology, Beclard's nuclei presence, and thyroxine (T4) level at diagnosis., Methods: L-T4/kg/day and dose change ratio (CR) were assessed in 74 CH patients., Results: The dose was statistically larger in athyreosis than in dyshormonogenesis (1-10 and beyond 14 years) and in ectopy (2, 15, 16 years). The ectopic children required statistically larger L-T4/kg than the dyshormonogenetic ones (3-7 years). The L-T4/kg/day was increased, not statistically, in patients or with T4 <30 nmol/l or without Beclard's nuclei at diagnosis. The CR progressively dropped after the 6th month at each attendance, without any difference in terms of aetiology, T4 level at diagnosis, or Beclard's nuclei. The total CR was greater (significantly) in patients without Beclard's nuclei, and (not significantly) in those with T4 <30 nmol/l at diagnosis or with agenesia., Conclusion: The L-T4 dose in CH is highly affected by the aetiology. The CR is higher in patients with delayed bone maturation at diagnosis. We suggest that these latter patients need blood tests more frequently to obtain a proper titration of the therapy., (Copyright 2007 S. Karger AG, Basel.)

The aim of this retrospective study was to ascertain, whether an early growth hormone (GH) treatment can normalize height prognosis of children with congenital GH deficiency (GHD). The study covers 23 children with early onset GHD who received GH 0.1 U/kg/day from the beginning of therapy (0.4-4.9 years). This dose was corrected for weight every 3 months during the whole duration of treatment (mean 7.9 +/- 2.4 years). As a consequence of the significant growth acceleration induced by GH treatment, the patients' height deficiency at the last check had changed from a range of between -8.0 and -1.8 SDS to between +0.5 and -4.3 SDS; on the average, it was significantly less severe than before treatment. Satisfactory growth acceleration was achieved in most patients concomitantly with accelerated bone maturation, as was shown by the stable height age/bone age ratio observed during the follow-up period. The predicted ultimate height was significantly greater than the pretreatment height and it did not differ from the target height. It is concluded that catch-up growth to the target percentile in GHD patients is possible, provided that substitutive treatment is begun during the first years of life and that GH doses are adjusted periodically for weight changes.

The aim of this study, performed in 10 children, was to verify if a treatment schedule, better reproducing the growth hormone (GH) physiological pattern, could achieve a greater growth response than daily s.c. administration in previously successfully treated children with an isolated idiopathic GH deficiency. A 1-month washout period was observed between the previous regimen and this study. GH (0.6 U/kg/week, the same as given previously) was administered weekly in 6 equal doses. Three children received the daily dose by a single s.c. administration (at 20.00 h) and the other 7 through a 27-gauge infusion needle inserted s.c., using a mini infusion pump permitting a constant delivery of 50% of the daily dose (20.00-08.00 h) and the administration of the other 50% through 3 equal pulses (at 20.00, 24.00, and 04.00 h). After 6 months each child changed regimen undergoing the other one with a 1-month washout interval. During the 12 months of therapy, the mean height increased from -3.2 (SE 0.45) to -2.8 (SE 0.37) SDS. When evaluating the circulating insulin-like growth factor-I pattern as well as the growth velocity and the ratio bone age increase/height age increase, no differences were noted between the two treatment regimens.