Abstract
Since TRH deficiency accounts for clinical and biochemical picture of IHH, the etiological therapeutic approach to this rare disease should be TRH administration. After informed consent from parents, we administered TRH p.o. to 3 patients with IHH, at the dose of 20 mg/day for 25 days and then 10 mg. every other day for 3 months in order to avoid the well-known phenomenon of pituitary desensitization. During the first period all patients showed a marked increase in TSH secretion, when monitored 3 hrs. after TRH administration (basal: 4.1, 5.9, and 27.2; peak: 16.4, 16.3 and 69.7 mcU/ml, respoctively). Consequently, FT3 and FT4 levels increased, reaching the normal range at different intervals; only in one patient FT3 and FT4 values were in the normal range over all the treatment period. After TRH withdrawal basal hormone levels fell to pretreatment values with the exception of TSH which was consistently lower (0.9, 1.1 and 6.3 mcU/ml, respectively), thus demonstrating a pituitary desensitization. During the second period all patients had an increase in TSH levels but only in one case a sustained normalization of FT4 levels was seen. No side effects were noted. In conclusion, the present data 1) confirm that TRH as daily therapy restores a transient euthyroid state in patients with IHH; 2) damonstrate that, at the dose and time schedule we used, long-term TRH administration does not seem effective in the treatment of IHH, because of pituitary desensitization; and 3) suggest that L-Thyroxine replacement therapy still remains the most reliable treatment of this disease.
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Rondanini, G., de Panizza, G., Bollati, A. et al. EFFECTS OF LONG-TERM TRH ADMINISTRATION IN PATIENTS WITH IDIOPATHIC HYPOTALAÎ HYPOTHYROIDISM (IHH). Pediatr Res 23, 134 (1988). https://doi.org/10.1203/00006450-198801000-00197
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DOI: https://doi.org/10.1203/00006450-198801000-00197