A case of Turner syndrome with congenital hypothyroidism untreated until age 38 years.
OBJECTIVE
The effect of thyroid hormone on human growth and maturation is considered 'permissive'. To evaluate the effect of a prolonged thyroid hormone defect, especially in the pubertal period, a woman with untreated congenital hypothyroidism underwent studies of thyroid function and bone maturation for the first time at age 38 years 10 months and received thyroid hormone replacement.


RESULTS
The karyotype was 45,X/46,XX. Menstruation had occurred for 10 years, from menarche until she was about 31 years old. Epiphyseal closure of the left hand was incomplete. The serum thyroid hormone level was virtually undetectable, and her thyroid gland was not detectable in the normal position by ultrasonography. Her height increased by 3.5 cm in the first 9 months after starting thyroid hormone replacement; after 11 months, closure of the epiphysis was complete.


CONCLUSIONS
Thyroid hormone is necessary to achieve bone maturation and epiphyseal closure, but its role is only permissive.
Ishikawa
Nori
N
Department of Pediatrics, Niigata Prefectural Koide Hospital, Niigata, Japan. yamato_it@mail.tiara.or.jp
Tanaka
Toshiaki
T
Hashimoto
Kinya
K
Wada
Masaki
M
D000328:Adult; D001827:Body Height / Q000187:drug effects; D003409:Congenital Hypothyroidism; D005260:Female; D006132:Growth Plate / Q000000981:diagnostic imaging / Q000503:physiopathology; D006225:Hand / Q000000981:diagnostic imaging; D006801:Humans; D007037:Hypothyroidism / Q000150:complications* / Q000188:drug therapy; D007621:Karyotyping; D008572:Menarche; D011859:Radiography; D013961:Thyroid Gland / Q000000981:diagnostic imaging; D013974:Thyroxine / Q000097:blood / Q000627:therapeutic use; D014424:Turner Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology*; D014463:Ultrasonography
D013974:Thyroxine