| Clinical Observation on isolated TRH deficient Congenital Hypothyroidism. |
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Heon Seok Han, Hyung Ro Moon |
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Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea |
| TRH 단독 결핍성 선천성 갑상선기능 저하증에 관한 연구 |
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한헌석, 문형로 |
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서울대학교 의과대학 소아과학교실 |
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Received: 27 March 1990 • Accepted: 25 May 1990 |
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| Abstract |
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Clinical features and endocrine function of 3 children with isolated TRH deficient congenital
hypothyroidism followed at Seoul National University Children’s Hospital from Aug, 1986 to
Aug. 1990 were reviewed.
During above period 262 congenital hypothyroid patients were followed at endocrine clinic, number
of congenital primary hypothyroidism was 218 cases (83.2%), and that of congenital secondary
hypothyroidism was 44 cases (16.8%). Of the congenital primary hypothyroidism, 194 cases(74%)
were thyroid dysgenesis and 24 cases(9.2%) were thyroid dyshormonogenesis. Of the congenital
secondary hypothyroidism, 4 cases(1.5%) were isolated form and 40 cases(15.3%) were combined
form. Of the 4 isolated congenital secondary hypothyroidism, 3 cases were isolated TRH deficient
form(1.2%).
They were all female and diagnosed between 7.5 to 15.9 year of age.
All complained short stature, but other clinical features of cretinism were not prominent. Associat-
ed congenital anomaly was detected in one case as Turner syndrome. TRH stimulation test
revealed normal response in 2 cases, exaggerated and prolonged response in 1 case. During OPD
follow up with thyroxine replacement, rapid growth velocity was observed in 2 cases. |
| Key Words:
Isolated TRH deficient congenital hypothyroidism |
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