Idiopathic hypothalamic dysfunction with precocious puberty and adipsic hypernatremia first presenting in adolescence

M. S. Cauble, Lynn R Mack, G. B. Schaefer, S. Balakrishnan, Jennifer Lynn Larsen

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Background: Idiopathic hypothalamic dysfunction is a rare syndrome that has been described in young children. This syndrome is characterized by a disturbance of thirst regulation with hypernatremia, hyperosmolarity, and altered hypothalamic function. Patient report: At age 6 years the reported patients presented with precocious puberty, by age 12 years she had hypernatremia presumed secondary to central diabetes insipidus and was treated with DDAVP, and at age 14 was identified to have hyperprolactinemia. At age 19 she presented with serum sodium of 185 mg/dl during an episode of illness associated with dehydration. After hydration, her sodium remained elevated. Arginine vasopressin was measurable but inappropriate to serum sodium, while urinary cyclic AMP response to vasopressin was appropriate. Conclusions: This is the first case of precocious puberty identified in association with the more classic features of idiopathic hypothalamic dysfunction, including later-onset hypernatremia, poor thermoregulatory function, obesity, and hyperprolactinemia.

Original languageEnglish (US)
Pages (from-to)1163-1167
Number of pages5
JournalJournal of Pediatric Endocrinology and Metabolism
Volume14
Issue number8
StatePublished - Oct 10 2001

Fingerprint

Hypernatremia
Precocious Puberty
Hyperprolactinemia
Sodium
Neurogenic Diabetes Insipidus
Thirst
Deamino Arginine Vasopressin
Arginine Vasopressin
Serum
Vasopressins
Dehydration
Cyclic AMP
Obesity

Keywords

  • Diabetes insipidus
  • Hypernatremia
  • Hyperprolactinemia
  • Puberty

ASJC Scopus subject areas

  • Endocrinology
  • Pediatrics, Perinatology, and Child Health

Cite this

Idiopathic hypothalamic dysfunction with precocious puberty and adipsic hypernatremia first presenting in adolescence. / Cauble, M. S.; Mack, Lynn R; Schaefer, G. B.; Balakrishnan, S.; Larsen, Jennifer Lynn.

In: Journal of Pediatric Endocrinology and Metabolism, Vol. 14, No. 8, 10.10.2001, p. 1163-1167.

Research output: Contribution to journalArticle

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