title: A case of familial thyroxine binding globulin excess associated with growth hormone deficiency.
abstract: A 7 years 3 months old Japanese boy with familial thyroxine binding globulin (TBG) excess associated with growth hormone (GH) deficiency is reported. The patients height was 106.4 cm (-2.86 s.d.) and his bone age was 5 years and 3 months. He had no goiter and his developmental milestones were normal. The serum thyroid stimulating hormone (TSH) was 2.8 microU/mL, triiodothyronine (T3) 3.1 ng/mL, thyroxine (T4) 23.4 micrograms/dL and free T4 1.8 ng/dL. The serum TBG level was beyond 80.0 micrograms/mL, with normal TSH response to the thyrotropin-releasing hormone (TRH) test. Familial study revealed that his grandmother, mother, uncle, younger sister and younger brother had high TBG and T3 levels, thus an X-linked co-dominant transmission was suggested. The peak GH responses to insulin and clonidine hydrochloride were 5.8 and 8.2 ng/mL, respectively. The mean nocturnal GH concentration was 2.5 ng/mL. His growth velocity increased from 4.8 to 8.4 cm/year and his serum TBG levels decreased gradually after human growth hormone (hGH) treatment.
authors.affiliation: Department of Pediatrics, Fukui Medical School, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Nakai
authors.forename: Y
authors.initials: Y
authors.lastname: Shigematsu
authors.forename: Y
authors.initials: Y
authors.lastname: Kikawa
authors.forename: M
authors.initials: M
authors.lastname: Sudo
mesh_terms: D001827:Body Height; D002648:Child; D013006:Growth Hormone / Q000097:blood / Q000172:deficiency* / Q000627:therapeutic use; D006801:Humans; D008297:Male; D013975:Thyroxine-Binding Proteins / Q000378:metabolism*; D014284:Triiodothyronine / Q000097:blood
chemical_list: D013975:Thyroxine-Binding Proteins; D014284:Triiodothyronine; D013006:Growth Hormone