| The Clinical Characteristics of Idiopathic Hypopituitarism in Children and Adolescents. |
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Ji Hyun Kim, Seong Yong Lee, Hye Rim Chung, Sun Hee LEE, Jae Hyun Kim, Young Ah Lee, Choong Ho Shin, Sei Won Yang |
1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. chshinpd@snu.ac.kr
2Department of Pediatrics, Seoul National University Boramae Hospital, Seoul, Korea.
3Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea. |
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| Abstract |
PURPOSE
The purpose of this study was to evaluate the clinical evolution and endocrine features of idiopathic hypopituitarism.
METHODS: We reviewed the medical records of 33 patients with idiopathic hypopituitarism who had been followed up from 1982 to 2008, attained their adult final height, and received appropriate hormonal replacement therapy.
RESULTS
28 of these patients (85%) were male, 26 patients (79%) were delivered by breech presentation, and 18 (55%) were firstborns. The mean age at diagnosis was 10.1 +/- 4.6 years, and bone age was delayed a mean of 4.9 +/- 3.0 years.
The mean standard deviation score (SDS) for height at diagnosis was -3.6 +/- 2.9. Deficiencies of pituitary hormones were observed in the following order of frequency: growth hormone (100%), thyroid-stimulating hormone (97.0%), luteinizing hormone/follicle - stimulating hormone (81.8%), adrenocorticotropic hormone (45.5%), and antidiuretic hormone (12.1%). Deficiencies of 4 hormones were observed in 14 (42.4%); 3 hormones, in 11 (33.3%); 2 hormones, in 5 (18.2%); and 5 hormones, in 2 (6.1%). Spontaneous onset of puberty occurred in 6 boys (21.4%), and there were no significant differences in the clinical presentations of pituitary hormone deficiency between these boys and those with gonadotropin-releasing hormone deficiency.
CONCLUSION
Idiopathic hypopituitarism should be considered in patients of short stature who are delivered by breech presentation, and their hormone levels should be monitored periodically. |
| Keywords:
Hypopituitarism;Congenital;Pituitary hormones;Puberty |
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