Short stature and thyroxine-binding globulin excess: improvement with triiodothyronine treatment.
Thyroxine-binding globulin (TBG) excess with increased total thyroxine (T4) and triiodothyronine (T3) levels has not been thought to produce symptoms. We report on a white boy, initially seen at 4.3 years of age and observed for 4 years, who has short stature caused by the excess thyroxine binding. At his initial examination his thyroxine-binding globulin (TBG) levels were elevated (17 mg/dL), and he had a T4 level of 25.8 micrograms/dL, short stature, a bone age of 19 months, normal vital signs, and hyperthyroid-stimulating hormone (TSH) response to thyrotropin-releasing hormone (TRH) testing (maximal value 58 microIU/mL). Results of tests obtained during the next 6 months showed other abnormalities related to thyroid function. Tests showed the following values: T3 412 ng/dL, thyroid uptake 24%, and low T3 resin uptake. They also showed these values: an elevated basal TSH of 8.7 microIU/mL, a slightly low preejection period to left ventricular ejection time ratio of 0.29 (normal 0.35 +/- 0.04), and WISC-R IQ within normal limits. Because of the persistent short stature, T3 supplementation was started at age 7 years and gradually increased to 35 micrograms/d. The patient showed no thyrotoxic symptoms. Serum T4 level decreased from 25.8 to 4.2 micrograms/dL, T3 increased to 1,240 ng/dL, the TRH/TSH test result was suppressed (maximal level 1.8), and the preejection period to left ventricular ejection time ratio decreased to 0.24. Growth velocity increased by 65%. Both of the child's parents had normal thyroid test results. A younger brother also showed similar elevations of TBG level and even greater T4 values (36 micrograms/dL).(ABSTRACT TRUNCATED AT 250 WORDS)
D001827:Body Height / Q000187:drug effects*; D002675:Child, Preschool; D005808:Genes, Recessive; D006130:Growth Disorders / Q000188:drug therapy / Q000235:genetics; D006801:Humans; D008297:Male; D013972:Thyrotropin / Q000378:metabolism; D013973:Thyrotropin-Releasing Hormone; D013975:Thyroxine-Binding Proteins / Q000032:analysis*; D014284:Triiodothyronine / Q000627:therapeutic use*; D014960:X Chromosome
D013975:Thyroxine-Binding Proteins; D014284:Triiodothyronine; D013973:Thyrotropin-Releasing Hormone; D013972:Thyrotropin