Abstract
A 9 year old adopted girl was seen for a goitre. Her natural mother had a chronic goitre. She was clinically euthyroid. Height and weight were normal. Blood pressure was 95/55, pulse 80/min and temperature 36.8°C. Serum thyroid hormone levels (/100ml) were markedly elevated : T4 : 24.4μg, T3 : 620ng. Basal plasma TSH was normal:<2 μu/ml. There was no circulating LATS or thyroidal antibodies. Radioactive I 123 thyroidal uptake was 78% at 150 min. Serum thyroid binding globulin level was normal. TRH test (150μg IV) resulted in a subnormal increase in plasma TSH: 8.4μu/ml (N : 11–15) with a slight burst of serum T3 level (+105ng). Carbimazole (30mg/day) lowered serum T4(11.2μg) and T3 (320ng) while causing a disproportionate rise in plasma TSH (18μu/ml) ; addition of thyroxine (100 μg/day) raised serum T4 and T3 and decreased plasma TSH (9.2μu/ml). After treatment, biological data returned to pretreatment values. This child presented the characteristic findings of a partial peripheral resistance to thyroid hormones ; although review of the 5 reports of this syndrome shows that it is rather heterogeneous, our case appears to be almost identical to the one of Lamberg (Lancet, i, 855, 1973).
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