Abstract
Two neonates (M.B. & J.C.) with typical features and histological evidence of Finnish-type CNS had low serum T4 (<6μg/dl) and normal serum TSH (<20μIU/dl) detected by the New England Regional Hypothyroid Screening Program. At 6 and 4 weeks of age serum TSH was elevated (M.B.>300μIU/dl;J.C. 71μIU/dl), T4 was low and FT4 was normal (M.B.3.1 ng/dl;J.C. 4.4 ng/dl). Technetium scan showed normally located thyroid gland in both. The half-life of parenterally administered sodium levothyroxine (150μg/IM) was accelerated (M.B.19.8 hrs; J.C. 22.6 hrs) compared to reported T4 half-life of 6-8 days. Urinary thyroxine was elevated in both patients (M.B.114-203μg/gm creat;J.C. 293μg/gm creat; normal <10μg/gm creat). Administration of 20-25μg/Kg/day of oral sodium levothyroxine suppressed TSH values in both. We conclude that despite low serum T4, which may be related to elevated urinary loss of hormonal iodine, normal free T4 is maintained by compensatory hypersecretion of TSH.
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Rasoulpour, M., Mclean, R., Siegel, N. et al. 1096 COMPENSATED HYPOTHYROIDISM IN CONGENITAL NEPHROTIC SYNDROME (CNS). Pediatr Res 12 (Suppl 4), 546 (1978). https://doi.org/10.1203/00006450-197804001-01102
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DOI: https://doi.org/10.1203/00006450-197804001-01102