Multiple neonatal endocrinopathies in McCune-Albright syndrome

Citation
P. Bareille et al., Multiple neonatal endocrinopathies in McCune-Albright syndrome, J PAEDIAT C, 35(3), 1999, pp. 315-318
Citations number
13
Categorie Soggetti
Pediatrics
Journal title
JOURNAL OF PAEDIATRICS AND CHILD HEALTH
ISSN journal
10344810 → ACNP
Volume
35
Issue
3
Year of publication
1999
Pages
315 - 318
Database
ISI
SICI code
1034-4810(199906)35:3<315:MNEIMS>2.0.ZU;2-L
Abstract
Two Gases of McCune-Albright syndrome (MAS) are reported who presented in t he neonatal period with profound failure to thrive, cardio-respiratory dist ress, precocious puberty and Gushing's syndrome for which both underwent bi lateral adrenalectomy. Both girls had also bilateral nephrocalcinosis; in o ne case that may have been attributed to Gushing's syndrome, but in the sec ond case the cause remained obscure with no obvious abnormality of calcium metabolism. The first girl had hydrocephalus which is uncommon in this cond ition and the second girl still failed to thrive at the age of 6 years, des pite adequate caloric intake and hormonal manipulation. A constellation of other abnormal features are described. These cases illustrate the complexit y of MAS which can become a life-threatening or a debilitating disorder.