Remission of congenital diabetes insipidus after eight years.
J Pediatr Endocrinol Metab
; 20(6): 733-7, 2007 Jun.
Article
em En
| MEDLINE
| ID: mdl-17663299
ABSTRACT
Septo-optic dysplasia (SOD) (De Morsier's syndrome) is a complex developmental disorder marked by variable and often incomplete formation of cranial midline structures, resulting in absence of the septum pellucidum, optic nerve hypoplasia, and hypothalamic-pituitary dysfunction. We describe a patient with SOD who manifested symptoms in the early neonatal period with severe deficiencies of multiple pituitary hormones including anti-diuretic hormone (ADH). Her congenital diabetic insipidus (DI), consequence of an anatomic defect, can be argued to be of the most severe type. Our patient resolved her severe DI 8 years after her initial presentation, suddenly requiring no further medical treatment for DI following longstanding pharmacological replacement of ADH. This is the first report of a patient with SOD with spontaneous resolution of congenital DI.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Vasopressinas
/
Displasia Septo-Óptica
/
Diabetes Insípido
Limite:
Child
/
Female
/
Humans
/
Newborn
Idioma:
En
Revista:
J Pediatr Endocrinol Metab
Assunto da revista:
ENDOCRINOLOGIA
/
PEDIATRIA
Ano de publicação:
2007
Tipo de documento:
Article
País de afiliação:
Estados Unidos