83:
214:: The most common endocrinopathy is precocious puberty, which presents in girls (~85%) with recurrent estrogen-producing cysts leading to episodic breast development, growth acceleration, and vaginal bleeding. Precocious puberty may also occur in boys with McCune–Albright syndrome, but is much less common (~10–15%). In children of both sexes, growth acceleration may lead to tall stature in childhood, however premature bone maturation may lead to early growth plate fusion and short stature in adulthood.
39:
929:
Cutler, Carolee M.; Lee, Janice S.; Butman, John A.; FitzGibbon, Edmond J.; Kelly, Marilyn H.; Brillante, Beth A.; Feuillan, Penelope; Robey, Pamela G.; DuFresne, Craig R.; Collins, Michael T. (November 2006). "Long-term outcome of optic nerve encasement and optic nerve decompression in patients with
364:
has been found successful in reducing bone pain and decreasing tumor growth, however there is limited safety data available in patients with fibrous dysplasia. Muscle strengthening exercises are important for preventing bone fractures; cycling and swimming are recommended in order to reduce the risk
289:
McCune–Albright syndrome has different levels of severity. For example, one child with McCune–Albright syndrome may be entirely healthy, with no outward evidence of bone or endocrine problems, enter puberty at close to the normal age, and have no unusual skin pigmentation. Diagnosis may be made only
276:
The mutation that causes McCune–Albright syndrome arises very early during embryogenesis. Because all cases of the syndrome are sporadic, it is believed that the mutation would be lethal if it affected all cells in the embryo. Mutant cells can only survive when they are intermixed with normal cells.
368:
Screening and management of endocrinopathies is an important part of managing fibrous dysplasia. For example, untreated growth hormone excess increases the risk of craniofacial fibrous dysplasia expansion and may lead to vision loss. Untreated hyperthyroidism and hypophosphatemia increases the risk
333:
Growth hormone excess is diagnosed using blood tests, such as insulin-like growth factor-1 levels. Monitoring growth rates alone is not sufficient to screen for growth hormone excess, because linear growth in children with McCune–Albright syndrome may be affected by skeletal deformities and other
305:
of the skull is the most useful test to evaluate craniofacial fibrous dysplasia. Regular hearing and vision screening is recommended. X-rays are usually sufficient to reveal fibrous dysplasia of the appendicular skeleton, but CT and/or MRI scans can reveal microfractures. Regular screening for
475:
Boyce, Alison M.; Collins, Michael T. (1993). "Fibrous
Dysplasia/McCune-Albright Syndrome". In Adam, Margaret P.; Ardinger, Holly H.; Pagon, Roberta A.; Wallace, Stephanie E.; Bean, Lora J.H.; Mefford, Heather C.; Stephens, Karen; Amemiya, Anne; Ledbetter, Nikki (eds.).
1931:
280:
There are no known risk factors for acquiring McCune–Albright syndrome, and no exposures during pregnancy that are known to either cause or prevent the mutation from occurring. The disease cannot be inherited and occurs equally among all ethnic groups.
298:
All patients with known or suspected McCune–Albright syndrome should undergo a screening evaluation for fibrous dysplasia. Nuclear medicine tests such as technetium-99 scintigraphy are the most sensitive way to detect fibrous dysplasia lesions.
1787:
1772:
51:
A typical lesion on the face, chest, and arm of a 5-year-old girl with McCune-Albright syndrome which demonstrates jagged "coast of Maine" borders, and the tendency for the lesions to both respect the midline and follow the developmental
1924:
414:
may be effective. Surgery may be an option, but may be complicated by the cranial abnormalities associated with the disorder. Excessive prolactin secretion may also occur; this is treated with dopamine agonists such as
1917:
224:: Hyperthyroidism occurs in approximately one-third of patients with McCune Albright syndrome. Patients have characteristic abnormalities on thyroid ultrasound, and may have a slight increased risk for thyroid cancer.
545:
Albright F, Butler AM, Hampton AO, Smith P (1937). "Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females: report of five cases".
217:
Testicular abnormalities: Testicular abnormalities are seen in a majority (~85%) of boys with McCune–Albright syndrome. These typically present with macro-orchidism. On pathology lesions show Leydig and
Sertoli cell
396:. However, because hyperthyroidism in McCune–Albright syndrome does not resolve, surgery or radiation are more definitive treatments. Periodic thyroid examination should be performed to screen for thyroid cancer.
203:
Patients may have one or many of these features, which may occur in any combination. As such, the clinical presentation of patients with McCune
Albright syndrome varies greatly depending on the disease features.
1311:
Javaid, Muhammad Kassim; Boyce, Alison; Appelman-Dijkstra, Natasha; Ong, Juling; Defabianis, Patrizia; Offiah, Amaka; Arundel, Paul; Shaw, Nick; Pos, Valter Dal; Underhil, Ann; Portero, Deanna (13 June 2019).
455:
227:
Growth hormone excess: Excess growth hormone secretion and is found in approximately 10–15% of patients. This may lead to expansion of craniofacial fibrous dysplasia, increasing the risk of vision and hearing
1668:
Tessaris, D.; Corrias, A.; Matarazzo, P.; De
Sanctis, L.; Wasniewska, M.; Messina, M. F.; Vigone, M. C.; Lala, R. (2012). "Thyroid abnormalities in children and adolescents with McCune-Albright syndrome".
324:
is typically diagnosed based on clinical presentation. A bone age examination should be performed to evaluation for skeletal maturation. Boys and men should have a screening testicular ultrasound.
1611:
Boyce, Alison M.; Chong, William H.; Shawker, Thomas H.; Pinto, Peter A.; Linehan, W. Marsten; Bhattacharryya, Nisan; Merino, Maria J.; Singer, Frederick R.; Collins, Michael T. (September 2012).
170:
66:
A typical lesion on the lower back in an adult with McCune-Albright syndrome demonstrates jagged borders (white arrow). Note the spinal asymmetry due to fibrous dysplasia-related scoliosis.
189:
Hyperpigmented skin lesions with characteristic features, including jagged "coast of Maine" borders and tendency occur along the midline of the body. These lesions are historically termed
812:
Celi, Francesco S.; Coppotelli, Giuseppe; Chidakel, Aaron; Kelly, Marilyn; Brillante, Beth A.; Shawker, Thomas; Cherman, Natasha; Feuillan, Penelope P.; Collins, Michael T. (June 2008).
1428:
Florenzano, Pablo; Pan, Kristen S.; Brown, Sydney M.; Paul, Scott M.; Kushner, Harvey; Guthrie, Lori C.; de Castro, Luis
Fernandez; Collins, Michael T.; Boyce, Alison M. (April 2019).
2686:
1485:
Boyce AM, Chong WH, Yao J, Gafni RI, Kelly MH, Chamberlain CE, Bassim C, Cherman N, Ellsworth M, Kasa-Vubu JZ, Farley FA, Molinolo AA, Bhattacharyya N, Collins MT (2012).
1534:
1371:
Kushchayeva, Yevgeniya S.; Kushchayev, Sergiy V.; Glushko, Tetiana Y.; Tella, Sri Harsha; Teytelboym, Oleg M.; Collins, Michael T.; Boyce, Alison M. (December 2018).
290:
after decades. In other cases, children are diagnosed in early infancy, show obvious bone disease, and obvious increased endocrine secretions from several glands.
419:. Radiation therapy has been associated with malignant transformation of skull base fibrous dysplasia, and should be avoided in all but the most dire cases.
2464:
981:
Boyce, Alison M.; Brewer, Carmen; DeKlotz, Timothy R.; Zalewski, Christopher K.; King, Kelly A.; Collins, Michael T.; Kim, H. Jeffrey (1 February 2018).
2721:
2316:
1314:"Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium"
2643:
1138:"Identification of a mutation in the gene encoding the alpha subunit of the stimulatory G protein of adenylyl cyclase in McCune-Albright syndrome"
356:
Surgical management of skeletal abnormalities has evolved over the years. Surgical intervention may be necessary for some skeletal abnormalities.
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20:
19:
This article is about the genetic condition McCune–Albright syndrome. For the genetically-similar hypoparathyroid hormone type 1A condition, see
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McCune–Albright syndrome is estimated to occur at a frequency between 1 person in 100,000 to 1 person in 1,000,000 individuals worldwide.
814:"The Role of Type 1 and Type 2 5′-Deiodinase in the Pathophysiology of the 3,5,3′-Triiodothyronine Toxicosis of McCune-Albright Syndrome"
1802:
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2003:
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2008:
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Estrada, Andrea; Boyce, Alison M.; Brillante, Beth A.; Guthrie, Lori C.; Gafni, Rachel I.; Collins, Michael T. (November 2016).
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is effective at prevent bleeding episodes and preventing short stature. In boys, these should be combined with drugs such as
270:
2711:
2251:
2199:
2117:
1430:"Age-Related Changes and Effects of Bisphosphonates on Bone Turnover and Disease Progression in Fibrous Dysplasia of Bone"
340:
Cushing syndrome is very rare, and is typically diagnosed clinically in infants who present with signs of severe illness.
183:
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Patients with known or suspected McCune–Albright syndrome should undergo a screening evaluation for endocrine features.
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Typical lesions that are often found on the nape of the neck and crease of the buttocks are shown (black arrows).
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to treat androgen excess. Periodic ultrasounds of testicular lesions should be performed to screen for cancer.
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are helpful in relieving bone pain, but it is no longer believed that they prevent progression of the disease.
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693:"Long-term Outcomes of Letrozole Treatment for Precocious Puberty in Girls with McCune-Albright Syndrome"
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Various endocrine diseases may present in McCune–Albright syndrome due to increased hormone production.
1097:"Thyroid carcinoma in the McCune-Albright syndrome: contributory role of activating Gs alpha mutations"
234:: In McCune–Albright syndrome, Cushing's syndrome is a very rare feature that develops only in infancy.
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Treatment is dictated by both the tissues affected and the extent to which they are affected.
197:
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McCune–Albright syndrome is suspected when two or more of the following features are present:
123:
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518:
McCune, Donovan J.; Bruch, Hilde (1937). "Progress in
Pediatrics: Osteodystrophia Fibrosa".
422:
140:
127:
119:
76:
2507:
2474:
2401:
2112:
1968:
795:
757:
Boyce, Alison M; Collins, Michael T (1993). "Fibrous
Dysplasia/McCune-Albright Syndrome".
573:
Anderson, Sharon (January 2020). "Café au Lait
Macules and Associated Genetic Syndromes".
357:
327:
221:
158:
104:
38:
1195:
Happle, R. (1986). "The McCune-Alrbight syndrome: a lethal gene surviving by mosaicism".
531:
330:
is diagnosed based on blood tests. A screening thyroid ultrasound exam may be performed.
1551:
Salenave, Sylvie; Boyce, Alison M.; Collins, Michael T.; Chanson, Philippe (June 2014).
872:
Salenave, Sylvie; Boyce, Alison M.; Collins, Michael T.; Chanson, Philippe (June 2014).
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633:"Fibrous Dysplasia/McCune-Albright Syndrome: A Rare, Mosaic Disease of Gα s Activation"
632:
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is a rare but potentially fatal complication that can occur in the first year of life.
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state, leads to constitutive receptor signaling and inappropriate production of excess
2700:
1706:
1613:"Characterization and management of testicular pathology in McCune-Albright syndrome"
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261:, on the long (q) arm of chromosome 20 at position 13.3, which is involved in
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production may lead to rickets, osteomalacia, and worsening skeletal outcomes.
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983:"Association of Hearing Loss and Otologic Outcomes With Fibrous Dysplasia"
930:
fibrous dysplasia: risk factors for blindness and safety of observation".
1892:
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889:
829:
1038:
Brown, Rebecca J.; Kelly, Marilyn H.; Collins, Michael T. (April 2010).
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Mosaic genetic disorder affecting the bone, skin and endocrine systems
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2501:
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1776:
1373:"Fibrous dysplasia for radiologists: beyond ground glass bone matrix"
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For treatment of precocious puberty, the aromatase inhibitor such as
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2193:
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456:
List of radiographic findings associated with cutaneous conditions
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2173:
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1807:
1095:
Collins MT, Sarlis NJ, Merino MJ, et al. (September 2003).
255:
1913:
1940:
Deficiencies of intracellular signaling peptides and proteins
1136:
Schwindinger, W F; Francomano, C A; Levine, M A (June 1992).
1245:
Dumitrescu, Claudia E.; Collins, Michael T. (19 May 2008).
337:
Hypophosphatemia is diagnosed by blood phosphorus levels.
153:
It was first described in 1937 by
American pediatrician
122:
affecting the bone, skin and endocrine systems. It is a
760:
Fibrous
Dysplasia/McCune-Albright Syndrome Gene Reviews
171:
List of conditions associated with café au lait macules
1754:
1617:
The Journal of Clinical Endocrinology and Metabolism
1557:
The Journal of Clinical Endocrinology and Metabolism
1044:
The Journal of Clinical Endocrinology and Metabolism
878:
The Journal of Clinical Endocrinology and Metabolism
818:
The Journal of Clinical Endocrinology and Metabolism
631:
Boyce, Alison M.; Collins, Michael T. (2020-04-01).
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Hyperthyroidism is managed with medications such as
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2242:
2081:
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1982:
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482:. Seattle (WA): University of Washington, Seattle.
265:signaling. This mutation, which occurs only in the
90:
70:
31:
1040:"Cushing Syndrome in the McCune-Albright Syndrome"
520:Archives of Pediatrics & Adolescent Medicine
403:may be given for treatment of hypophosphatemia.
1142:Proceedings of the National Academy of Sciences
1751:for fibrous dysplasia/McCune–Albright Syndrome
752:
750:
748:
746:
744:
2687:intracellular signaling peptides and proteins
1925:
867:
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8:
1533:: CS1 maint: multiple names: authors list (
807:
805:
1487:"Denosumab treatment for fibrous dysplasia"
987:JAMA Otolaryngology–Head & Neck Surgery
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2027:
1951:
1932:
1918:
1910:
1755:
406:For growth hormone excess, treatment with
81:
37:
28:
2549:EDARADD Hypohidrotic ectodermal dysplasia
1644:
1584:
1553:"Acromegaly and McCune-Albright syndrome"
1510:
1461:
1404:
1347:
1329:
1272:
1262:
1171:
1153:
1112:
1071:
1014:
905:
874:"Acromegaly and McCune-Albright Syndrome"
845:
724:
664:
1240:
1238:
1236:
1234:
467:
369:of fractures and skeletal deformities.
1526:
938:(5): 1011–1017, discussion 1017–1018.
791:
781:
1606:
1604:
1546:
1544:
1306:
1304:
1302:
1300:
1298:
1296:
1294:
1292:
763:. University of Washington, Seattle.
7:
2180:Neutrophil immunodeficiency syndrome
2063:Albright's hereditary osteodystrophy
1434:Journal of Bone and Mineral Research
686:
684:
626:
624:
622:
620:
532:10.1001/archpedi.1937.01980040110009
250:Genetically, there is a spontaneous
21:Albright's hereditary osteodystrophy
2534:Signal transducing adaptor proteins
2118:KRAS Cardiofaciocutaneous syndrome
2009:X-linked intellectual disability 1
2004:Juvenile primary lateral sclerosis
1209:10.1111/j.1399-0004.1986.tb01261.x
944:10.1227/01.NEU.0000254440.02736.E3
237:Hypophosphatemia due to increased
14:
2465:Bannayan–Riley–Ruvalcaba syndrome
1318:Orphanet Journal of Rare Diseases
1251:Orphanet Journal of Rare Diseases
697:European Journal of Endocrinology
2722:Diseases named after discoverers
2053:Progressive osseous heteroplasia
575:Journal of Pediatric Health Care
433:may also be used for treatment.
2658:PRKCSH Polycystic liver disease
1671:Hormone Research in Paediatrics
2644:Wolff–Parkinson–White syndrome
2048:Pseudopseudohypoparathyroidism
1947:GTP-binding protein regulators
1:
2252:Cardiofaciocutaneous syndrome
2200:Chylomicron retention disease
365:of fracture during exercise.
184:polyostotic fibrous dysplasia
157:and American endocrinologist
2494:X-linked myotubular myopathy
451:List of cutaneous conditions
429:is the treatment of choice.
2291:X-linked agammaglobulinemia
2135:Charcot–Marie–Tooth disease
587:10.1016/j.pedhc.2019.05.001
560:10.1056/NEJM193704292161701
310:should also be undertaken.
239:fibroblast growth factor 23
2738:
1994:Marinesco–Sjögren syndrome
1721:"McCune-Albright syndrome"
1247:"McCune-Albright syndrome"
1101:J. Clin. Endocrinol. Metab
168:
18:
2682:
2602:Neurofibromatosis type II
2436:Pseudohypoaldosteronism 2
2163:Griscelli syndrome type 2
1956:GTPase-activating protein
1389:10.1007/s13244-018-0666-6
1331:10.1186/s13023-019-1102-9
999:10.1001/jamaoto.2017.2407
45:
36:
2470:Lhermitte–Duclos disease
2068:McCune–Albright syndrome
2058:Pseudohypoparathyroidism
1964:Neurofibromatosis type I
1887:McCune–Albright syndrome
148:heterotrimeric G protein
116:McCune–Albright syndrome
32:McCune-Albright syndrome
2228:Bardet–Biedl syndrome 3
1155:10.1073/pnas.89.11.5152
373:Endocrine abnormalities
314:Endocrine abnormalities
46:Skin hyperpigmentation.
2376:Peutz–Jeghers syndrome
2362:Incontinentia pigmenti
2348:Li–Fraumeni syndrome 2
1999:Aarskog–Scott syndrome
1264:10.1186/1750-1172-3-12
1114:10.1210/jc.2002-021642
352:Skeletal abnormalities
294:Skeletal abnormalities
2480:Proteus-like syndrome
2334:Coffin-Lowry syndrome
1377:Insights into Imaging
649:10.1210/endrev/bnz011
126:disease arising from
2712:Congenital disorders
2388:Myotonic dystrophy 1
1629:10.1210/jc.2012-1791
1569:10.1210/jc.2013-3826
1056:10.1210/jc.2009-2321
890:10.1210/jc.2013-3826
830:10.1210/jc.2007-2237
252:postzygotic mutation
191:café au lait macules
155:Donovan James McCune
131:activating mutations
100:Donovan James McCune
709:10.1530/EJE-16-0526
399:Oral phosphate and
2518:Metachondromatosis
2214:Joubert syndrome 8
2149:Carpenter syndrome
1984:Guanine nucleotide
1974:Tuberous sclerosis
1851:External resources
334:endocrinopathies.
322:Precocious puberty
232:Cushing's syndrome
212:Precocious puberty
165:Signs and symptoms
2694:
2693:
2528:
2527:
2508:Noonan syndrome 1
2402:Seckel syndrome 1
2238:
2237:
2113:Noonan syndrome 3
2099:Costello syndrome
2017:
2016:
1907:
1906:
1749:GeneReviews entry
1683:10.1159/000342641
1623:(9): E1782–1790.
1503:10.1002/jbmr.1603
1446:10.1002/jbmr.3649
1197:Clinical Genetics
1148:(11): 5152–5156.
637:Endocrine Reviews
198:endocrine disease
196:Hyperfunctioning
180:Fibrous dysplasia
141:the alpha-subunit
113:
112:
54:lines of Blaschko
26:Medical condition
2729:
2513:LEOPARD syndrome
2416:Oguchi disease 2
2317:Serine/threonine
2305:ZAP70 deficiency
2272:
2028:
1952:
1934:
1927:
1920:
1911:
1756:
1736:
1735:
1733:
1731:
1717:
1711:
1710:
1665:
1659:
1658:
1648:
1608:
1599:
1598:
1588:
1563:(6): 1955–1969.
1548:
1539:
1538:
1532:
1524:
1514:
1491:J Bone Miner Res
1482:
1476:
1475:
1465:
1425:
1419:
1418:
1408:
1383:(6): 1035–1056.
1368:
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1333:
1308:
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1286:
1276:
1266:
1242:
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1175:
1157:
1133:
1127:
1126:
1116:
1092:
1086:
1085:
1075:
1050:(4): 1508–1515.
1035:
1029:
1028:
1018:
978:
972:
971:
926:
920:
919:
909:
884:(6): 1955–1969.
869:
860:
859:
849:
824:(6): 2383–2389.
809:
800:
799:
793:
789:
787:
779:
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728:
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615:
614:
570:
564:
563:
542:
536:
535:
515:
509:
498:
492:
491:
472:
423:Cushing syndrome
139:, which encodes
120:genetic disorder
86:
85:
77:Medical genetics
41:
29:
2737:
2736:
2732:
2731:
2730:
2728:
2727:
2726:
2697:
2696:
2695:
2690:
2678:
2583:
2524:
2475:Cowden syndrome
2449:
2442:
2318:
2311:
2276:Tyrosine kinase
2256:
2234:
2077:
2013:
1986:exchange factor
1985:
1978:
1969:Watson syndrome
1941:
1938:
1908:
1903:
1902:
1846:
1845:
1767:
1745:
1740:
1739:
1729:
1727:
1719:
1718:
1714:
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1610:
1609:
1602:
1550:
1549:
1542:
1525:
1484:
1483:
1479:
1427:
1426:
1422:
1370:
1369:
1365:
1310:
1309:
1290:
1244:
1243:
1232:
1194:
1193:
1189:
1135:
1134:
1130:
1094:
1093:
1089:
1037:
1036:
1032:
980:
979:
975:
928:
927:
923:
871:
870:
863:
811:
810:
803:
790:
780:
773:
771:
756:
755:
742:
690:
689:
682:
630:
629:
618:
572:
571:
567:
554:(17): 727–746.
548:N. Engl. J. Med
544:
543:
539:
517:
516:
512:
499:
495:
474:
473:
469:
464:
447:
439:
375:
358:Bisphosphonates
354:
346:
328:Hyperthyroidism
316:
296:
287:
248:
222:Hyperthyroidism
182:(specifically,
173:
167:
159:Fuller Albright
146:
109:
105:Fuller Albright
80:
62:
57:
47:
27:
24:
17:
12:
11:
5:
2735:
2733:
2725:
2724:
2719:
2714:
2709:
2707:Genodermatoses
2699:
2698:
2692:
2691:
2683:
2680:
2679:
2677:
2676:
2675:
2674:
2662:
2661:
2660:
2648:
2647:
2646:
2634:
2633:
2632:
2630:Carney complex
2620:
2619:
2618:
2606:
2605:
2604:
2591:
2589:
2585:
2584:
2582:
2581:
2580:
2579:
2567:
2566:
2565:
2553:
2552:
2551:
2538:
2536:
2530:
2529:
2526:
2525:
2523:
2522:
2521:
2520:
2515:
2510:
2498:
2497:
2496:
2484:
2483:
2482:
2477:
2472:
2467:
2454:
2452:
2444:
2443:
2441:
2440:
2439:
2438:
2420:
2419:
2418:
2406:
2405:
2404:
2392:
2391:
2390:
2380:
2379:
2378:
2366:
2365:
2364:
2352:
2351:
2350:
2338:
2337:
2336:
2323:
2321:
2313:
2312:
2310:
2309:
2308:
2307:
2295:
2294:
2293:
2280:
2278:
2269:
2258:
2257:
2255:
2254:
2248:
2246:
2240:
2239:
2236:
2235:
2233:
2232:
2231:
2230:
2218:
2217:
2216:
2204:
2203:
2202:
2185:
2184:
2183:
2182:
2168:
2167:
2166:
2165:
2153:
2152:
2151:
2139:
2138:
2137:
2123:
2122:
2121:
2120:
2115:
2103:
2102:
2101:
2087:
2085:
2079:
2078:
2076:
2075:
2070:
2065:
2060:
2055:
2050:
2036:
2034:
2025:
2019:
2018:
2015:
2014:
2012:
2011:
2006:
2001:
1996:
1990:
1988:
1980:
1979:
1977:
1976:
1971:
1966:
1960:
1958:
1949:
1943:
1942:
1939:
1937:
1936:
1929:
1922:
1914:
1905:
1904:
1901:
1900:
1889:
1878:
1867:
1855:
1854:
1852:
1848:
1847:
1844:
1843:
1832:
1821:
1810:
1799:
1784:
1768:
1763:
1762:
1760:
1759:Classification
1753:
1752:
1744:
1743:External links
1741:
1738:
1737:
1712:
1677:(3): 151–157.
1660:
1600:
1540:
1497:(7): 1462–70.
1477:
1440:(4): 653–660.
1420:
1363:
1288:
1230:
1203:(4): 321–324.
1187:
1128:
1087:
1030:
993:(2): 102–107.
973:
921:
861:
801:
792:|website=
740:
703:(5): 477–483.
680:
643:(2): 345–370.
616:
565:
537:
510:
493:
466:
465:
463:
460:
459:
458:
453:
446:
443:
438:
435:
383:spironolactone
374:
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353:
350:
345:
342:
315:
312:
295:
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286:
283:
247:
244:
243:
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144:
111:
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96:
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74:
68:
67:
43:
42:
34:
33:
25:
15:
13:
10:
9:
6:
4:
3:
2:
2734:
2723:
2720:
2718:
2715:
2713:
2710:
2708:
2705:
2704:
2702:
2689:
2688:
2681:
2673:
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2667:
2663:
2659:
2656:
2655:
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2649:
2645:
2642:
2641:
2640:
2639:
2635:
2631:
2628:
2627:
2626:
2625:
2621:
2617:
2614:
2613:
2612:
2611:
2607:
2603:
2600:
2599:
2598:
2597:
2593:
2592:
2590:
2586:
2578:
2575:
2574:
2573:
2572:
2568:
2564:
2561:
2560:
2559:
2558:
2554:
2550:
2547:
2546:
2545:
2544:
2540:
2539:
2537:
2535:
2531:
2519:
2516:
2514:
2511:
2509:
2506:
2505:
2504:
2503:
2499:
2495:
2492:
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2481:
2478:
2476:
2473:
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2468:
2466:
2463:
2462:
2461:
2460:
2456:
2455:
2453:
2451:
2445:
2437:
2434:
2433:
2432:
2431:
2426:
2425:
2421:
2417:
2414:
2413:
2412:
2411:
2407:
2403:
2400:
2399:
2398:
2397:
2393:
2389:
2386:
2385:
2384:
2381:
2377:
2374:
2373:
2372:
2371:
2367:
2363:
2360:
2359:
2358:
2357:
2353:
2349:
2346:
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2344:
2343:
2339:
2335:
2332:
2331:
2330:
2329:
2325:
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2322:
2320:
2314:
2306:
2303:
2302:
2301:
2300:
2296:
2292:
2289:
2288:
2287:
2286:
2282:
2281:
2279:
2277:
2273:
2270:
2268:
2264:
2259:
2253:
2250:
2249:
2247:
2245:
2241:
2229:
2226:
2225:
2224:
2223:
2219:
2215:
2212:
2211:
2210:
2209:
2205:
2201:
2198:
2197:
2196:
2195:
2190:
2187:
2186:
2181:
2178:
2177:
2176:
2175:
2170:
2169:
2164:
2161:
2160:
2159:
2158:
2154:
2150:
2147:
2146:
2145:
2144:
2140:
2136:
2133:
2132:
2131:
2130:
2125:
2124:
2119:
2116:
2114:
2111:
2110:
2109:
2108:
2104:
2100:
2097:
2096:
2095:
2094:
2089:
2088:
2086:
2084:
2080:
2074:
2071:
2069:
2066:
2064:
2061:
2059:
2056:
2054:
2051:
2049:
2045:
2041:
2038:
2037:
2035:
2033:
2032:Heterotrimeic
2029:
2026:
2024:
2020:
2010:
2007:
2005:
2002:
2000:
1997:
1995:
1992:
1991:
1989:
1987:
1981:
1975:
1972:
1970:
1967:
1965:
1962:
1961:
1959:
1957:
1953:
1950:
1948:
1944:
1935:
1930:
1928:
1923:
1921:
1916:
1915:
1912:
1899:
1895:
1894:
1890:
1888:
1884:
1883:
1879:
1877:
1873:
1872:
1868:
1866:
1862:
1861:
1857:
1856:
1853:
1849:
1842:
1838:
1837:
1833:
1831:
1827:
1826:
1822:
1820:
1816:
1815:
1811:
1809:
1805:
1804:
1800:
1798:
1794:
1793:
1789:
1785:
1783:
1779:
1778:
1774:
1770:
1769:
1766:
1761:
1757:
1750:
1747:
1746:
1742:
1726:
1722:
1716:
1713:
1708:
1704:
1700:
1696:
1692:
1688:
1684:
1680:
1676:
1672:
1664:
1661:
1656:
1652:
1647:
1642:
1638:
1634:
1630:
1626:
1622:
1618:
1614:
1607:
1605:
1601:
1596:
1592:
1587:
1582:
1578:
1574:
1570:
1566:
1562:
1558:
1554:
1547:
1545:
1541:
1536:
1530:
1522:
1518:
1513:
1508:
1504:
1500:
1496:
1492:
1488:
1481:
1478:
1473:
1469:
1464:
1459:
1455:
1451:
1447:
1443:
1439:
1435:
1431:
1424:
1421:
1416:
1412:
1407:
1402:
1398:
1394:
1390:
1386:
1382:
1378:
1374:
1367:
1364:
1359:
1355:
1350:
1345:
1341:
1337:
1332:
1327:
1323:
1319:
1315:
1307:
1305:
1303:
1301:
1299:
1297:
1295:
1293:
1289:
1284:
1280:
1275:
1270:
1265:
1260:
1256:
1252:
1248:
1241:
1239:
1237:
1235:
1231:
1226:
1222:
1218:
1214:
1210:
1206:
1202:
1198:
1191:
1188:
1183:
1179:
1174:
1169:
1165:
1161:
1156:
1151:
1147:
1143:
1139:
1132:
1129:
1124:
1120:
1115:
1110:
1107:(9): 4413–7.
1106:
1102:
1098:
1091:
1088:
1083:
1079:
1074:
1069:
1065:
1061:
1057:
1053:
1049:
1045:
1041:
1034:
1031:
1026:
1022:
1017:
1012:
1008:
1004:
1000:
996:
992:
988:
984:
977:
974:
969:
965:
961:
957:
953:
949:
945:
941:
937:
933:
925:
922:
917:
913:
908:
903:
899:
895:
891:
887:
883:
879:
875:
868:
866:
862:
857:
853:
848:
843:
839:
835:
831:
827:
823:
819:
815:
808:
806:
802:
797:
785:
770:
766:
762:
761:
753:
751:
749:
747:
745:
741:
736:
732:
727:
722:
718:
714:
710:
706:
702:
698:
694:
687:
685:
681:
676:
672:
667:
662:
658:
654:
650:
646:
642:
638:
634:
627:
625:
623:
621:
617:
612:
608:
604:
600:
596:
592:
588:
584:
580:
576:
569:
566:
561:
557:
553:
549:
541:
538:
533:
529:
525:
521:
514:
511:
508:
507:Who Named It?
504:
503:
497:
494:
489:
485:
481:
480:
471:
468:
461:
457:
454:
452:
449:
448:
444:
442:
436:
434:
432:
428:
427:Adrenalectomy
424:
420:
418:
413:
410:analogues or
409:
404:
402:
397:
395:
390:
388:
384:
380:
372:
370:
366:
363:
359:
351:
349:
343:
341:
338:
335:
331:
329:
325:
323:
319:
313:
311:
309:
304:
300:
293:
291:
284:
282:
278:
274:
272:
268:
264:
260:
257:
253:
245:
240:
236:
233:
230:
226:
223:
220:
216:
213:
210:
209:
208:
205:
199:
195:
192:
188:
185:
181:
178:
177:
176:
172:
164:
162:
160:
156:
151:
149:
142:
138:
137:
132:
129:
125:
121:
118:is a complex
117:
106:
103:
101:
98:
97:
95:
93:
89:
84:
78:
75:
73:
69:
65:
60:
55:
50:
44:
40:
35:
30:
22:
2684:
2664:
2650:
2636:
2622:
2608:
2594:
2569:
2555:
2541:
2500:
2486:
2457:
2428:
2422:
2408:
2394:
2368:
2354:
2340:
2326:
2297:
2283:
2220:
2206:
2192:
2172:
2155:
2141:
2127:
2105:
2091:
2067:
1891:
1880:
1869:
1858:
1834:
1823:
1812:
1801:
1786:
1771:
1728:. Retrieved
1724:
1715:
1674:
1670:
1663:
1620:
1616:
1560:
1556:
1529:cite journal
1494:
1490:
1480:
1437:
1433:
1423:
1380:
1376:
1366:
1321:
1317:
1254:
1250:
1200:
1196:
1190:
1145:
1141:
1131:
1104:
1100:
1090:
1047:
1043:
1033:
990:
986:
976:
935:
932:Neurosurgery
931:
924:
881:
877:
821:
817:
772:. Retrieved
759:
700:
696:
640:
636:
581:(1): 71–81.
578:
574:
568:
551:
547:
540:
523:
519:
513:
500:
496:
478:
470:
440:
437:Epidemiology
421:
408:somatostatin
405:
398:
391:
376:
367:
355:
347:
339:
336:
332:
326:
320:
317:
301:
297:
288:
279:
275:
249:
218:hyperplasia.
206:
202:
174:
152:
134:
115:
114:
63:
58:
48:
2450:phosphatase
2267:phosphatase
1860:MedlinePlus
1725:NIH Library
479:GeneReviews
417:cabergoline
412:pegvisomant
92:Named after
2701:Categories
2577:Zaspopathy
2244:MAP kinase
1882:Patient UK
1825:DiseasesDB
1324:(1): 139.
526:(4): 806.
462:References
431:Metyrapone
401:calcitriol
394:thioamides
169:See also:
2717:Syndromes
2685:See also
2563:Cherubism
2083:Monomeric
2023:G protein
1871:eMedicine
1841:726029005
1836:SNOMED CT
1730:6 January
1707:207737684
1691:1663-2826
1637:1945-7197
1577:1945-7197
1454:1523-4681
1397:1869-4101
1340:1750-1172
1257:(1): 12.
1164:0027-8424
1064:0021-972X
1007:2168-619X
952:1524-4040
898:0021-972X
838:0021-972X
794:ignored (
784:cite book
774:6 January
717:0804-4643
657:1945-7189
611:209341168
595:1532-656X
502:synd/1844
387:flutamide
379:letrozole
362:Denosumab
344:Treatment
308:scoliosis
285:Diagnosis
263:G-protein
72:Specialty
2448:Tyrosine
1893:Orphanet
1876:ped/1386
1699:23006743
1655:22745241
1595:24517150
1521:22431375
1472:30645769
1415:30484079
1358:31196103
1283:18489744
1225:37078044
1123:12970318
1082:20157193
1025:29192304
968:19550908
960:17143235
916:24517150
856:18349068
769:25719192
735:27562402
675:31673695
603:31831114
488:25719192
445:See also
246:Genetics
143:of the G
2624:PRKAR1A
2616:CADASIL
2610:Notch 3
2543:EDARADD
2328:RPS6KA3
1819:D005359
1646:3431566
1586:4037730
1512:3377825
1463:6983318
1406:6269335
1349:6567644
1274:2459161
1217:3720010
1182:1594625
1073:2853983
1016:5839293
907:4037730
847:2435649
726:5066167
666:7127130
303:CT scan
254:of the
128:somatic
2652:PRKCSH
2638:PRKAG2
2557:SH3BP2
2502:PTPN11
2319:kinase
2263:kinase
2261:Other
2208:ARL13B
1865:001217
1808:174800
1797:756.54
1705:
1697:
1689:
1653:
1643:
1635:
1593:
1583:
1575:
1519:
1509:
1470:
1460:
1452:
1413:
1403:
1395:
1356:
1346:
1338:
1281:
1271:
1223:
1215:
1180:
1170:
1162:
1121:
1080:
1070:
1062:
1023:
1013:
1005:
966:
958:
950:
914:
904:
896:
854:
844:
836:
767:
733:
723:
715:
673:
663:
655:
609:
601:
593:
486:
267:mosaic
124:mosaic
79:
2672:XIAP2
2588:Other
2370:STK11
2356:IKBKG
2342:CHEK2
2299:ZAP70
2194:SAR1B
2171:RHO:
2157:RAB27
2143:RAB23
2126:RAB:
2090:RAS:
2073:CGL 2
2044:GNAS1
1782:Q78.1
1703:S2CID
1221:S2CID
1173:49247
964:S2CID
607:S2CID
228:loss.
2666:XIAP
2571:LDB3
2488:MTM1
2459:PTEN
2430:WNK1
2424:WNK4
2410:GRK1
2383:DMPK
2222:ARL6
2174:RAC2
2129:RAB7
2107:KRAS
2093:HRAS
2040:cAMP
1830:7880
1814:MeSH
1803:OMIM
1792:9-CM
1732:2018
1695:PMID
1687:ISSN
1651:PMID
1633:ISSN
1591:PMID
1573:ISSN
1535:link
1517:PMID
1468:PMID
1450:ISSN
1411:PMID
1393:ISSN
1354:PMID
1336:ISSN
1279:PMID
1213:PMID
1178:PMID
1160:ISSN
1119:PMID
1078:PMID
1060:ISSN
1021:PMID
1003:ISSN
956:PMID
948:ISSN
912:PMID
894:ISSN
852:PMID
834:ISSN
796:help
776:2018
765:PMID
731:PMID
713:ISSN
671:PMID
653:ISSN
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