McCune-Albright Syndrome: Long-term Follow-up

Peter A. Lee, Cornelis Van Dop, Claude J. Migeon

Research output: Contribution to journalArticle

Abstract

This article describes clinical follow-up of 15 patients—13 females and two males—with McCune-Albright syndrome. Osseous fractures occurred only during childhood, while hearing impairment due to temporal bone involvement occurred in four of six adults. Four females with precocious puberty had final heights that were not different from the mean for normal females; they eventually developed regular menses, and two had children. Persistent hyperthyroidism requiring ablative therapy occurred in three subjects, while hypophosphatemia occurred in three subjects. The protean manifestations of this disorder suggest that it results from a basic defect of cellular regulation. We postulate that its varied endocrine abnormalities result from altered regulation of intracellular cyclic adenosine monophosphate effects.

Original languageEnglish (US)
Pages (from-to)2980-2984
Number of pages5
JournalJournal of the American Medical Association
Volume256
Issue number21
DOIs
StatePublished - Dec 5 1986

Fingerprint

Polyostotic Fibrous Dysplasia
Hypophosphatemia
Precocious Puberty
Menstruation
Temporal Bone
Hyperthyroidism
Hearing Loss
Cyclic AMP
Therapeutics

ASJC Scopus subject areas

  • Medicine(all)

Cite this

McCune-Albright Syndrome : Long-term Follow-up. / Lee, Peter A.; Van Dop, Cornelis; Migeon, Claude J.

In: Journal of the American Medical Association, Vol. 256, No. 21, 05.12.1986, p. 2980-2984.

Research output: Contribution to journalArticle

Lee, Peter A. ; Van Dop, Cornelis ; Migeon, Claude J. / McCune-Albright Syndrome : Long-term Follow-up. In: Journal of the American Medical Association. 1986 ; Vol. 256, No. 21. pp. 2980-2984.
@article{b8afcab73e414b3bb4f9e9de61f1a0ad,
title = "McCune-Albright Syndrome: Long-term Follow-up",
abstract = "This article describes clinical follow-up of 15 patients—13 females and two males—with McCune-Albright syndrome. Osseous fractures occurred only during childhood, while hearing impairment due to temporal bone involvement occurred in four of six adults. Four females with precocious puberty had final heights that were not different from the mean for normal females; they eventually developed regular menses, and two had children. Persistent hyperthyroidism requiring ablative therapy occurred in three subjects, while hypophosphatemia occurred in three subjects. The protean manifestations of this disorder suggest that it results from a basic defect of cellular regulation. We postulate that its varied endocrine abnormalities result from altered regulation of intracellular cyclic adenosine monophosphate effects.",
author = "Lee, {Peter A.} and {Van Dop}, Cornelis and Migeon, {Claude J.}",
year = "1986",
month = "12",
day = "5",
doi = "10.1001/jama.1986.03380210076028",
language = "English (US)",
volume = "256",
pages = "2980--2984",
journal = "JAMA - Journal of the American Medical Association",
issn = "0098-7484",
publisher = "American Medical Association",
number = "21",

}

TY - JOUR

T1 - McCune-Albright Syndrome

T2 - Long-term Follow-up

AU - Lee, Peter A.

AU - Van Dop, Cornelis

AU - Migeon, Claude J.

PY - 1986/12/5

Y1 - 1986/12/5

N2 - This article describes clinical follow-up of 15 patients—13 females and two males—with McCune-Albright syndrome. Osseous fractures occurred only during childhood, while hearing impairment due to temporal bone involvement occurred in four of six adults. Four females with precocious puberty had final heights that were not different from the mean for normal females; they eventually developed regular menses, and two had children. Persistent hyperthyroidism requiring ablative therapy occurred in three subjects, while hypophosphatemia occurred in three subjects. The protean manifestations of this disorder suggest that it results from a basic defect of cellular regulation. We postulate that its varied endocrine abnormalities result from altered regulation of intracellular cyclic adenosine monophosphate effects.

AB - This article describes clinical follow-up of 15 patients—13 females and two males—with McCune-Albright syndrome. Osseous fractures occurred only during childhood, while hearing impairment due to temporal bone involvement occurred in four of six adults. Four females with precocious puberty had final heights that were not different from the mean for normal females; they eventually developed regular menses, and two had children. Persistent hyperthyroidism requiring ablative therapy occurred in three subjects, while hypophosphatemia occurred in three subjects. The protean manifestations of this disorder suggest that it results from a basic defect of cellular regulation. We postulate that its varied endocrine abnormalities result from altered regulation of intracellular cyclic adenosine monophosphate effects.

UR - http://www.scopus.com/inward/record.url?scp=0022892512&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0022892512&partnerID=8YFLogxK

U2 - 10.1001/jama.1986.03380210076028

DO - 10.1001/jama.1986.03380210076028

M3 - Article

C2 - 3773215

AN - SCOPUS:0022892512

VL - 256

SP - 2980

EP - 2984

JO - JAMA - Journal of the American Medical Association

JF - JAMA - Journal of the American Medical Association

SN - 0098-7484

IS - 21

ER -