¨A síndrome de McCune Albright é uma doença rara, clinicamente definida pela combinação de displasia fibrosa poliostótica – caracterizada por múltiplas lesões císticas nos ossos, manchas café-com-leite e endocrinopatias com hiperfunção, como puberdade precoce, hipertireoidismo, acromegalia e outras. A fisiopatologia biomolecular baseia-se em uma mutação ativadora do gene para a subunidade alfa da proteína de membrana Gs, que estimula a produção intracelular de AMPc, conferindo secreção autônoma à glândula em particular. A glândula tireóide é freqüentemente envolvida nesta doença. O hipertireoidismo pode causar puberdade precoce. Esta síndrome é tratada com testolactona, um inibidor da aromatase. Os tumores adrenais produtores de esteroides são raramente encontrados são tratados cirurgicamente.¨
■ Severe neonatal cholestasis as an early presentation of McCune-Albright syndrome
Nicole Coles, Ian Comeau, Tatiana Munoz et al
J Clin Res Pediatr Endocrinol. 2019 Mar; 11(1): 100–103.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6398189/
■ Polyostotit fibrous dysplasia with and without McCune-Albright syndrome – clinical features in a Nordic pediatric cohort
Pauliina Utriainen, Helena Valta, Sigridur Björnsdottir et al
Front Endocrinol (Lausanne) 2018; 9: 96
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5863549/
■ Peculiarities of precocious puberty in boys and girls with McCune-Albrigh syndrome
Domenico Corica, Tommaso Aversa, Giorgia Pepe et al
Front Endocrinol (Lausanne) 2018; 9: 337
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6023984/
■ Secondary aneurysmal bone cyst in McCune-Albright syndrom
S Tournis, A Balanika, P D Megaloikonomos, A F Mavrogenis
Clin Cases Miner Bone Metab. 2017 Sep-Dec;14(3):332-335
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5762225/
■ Gigantism in a McCune-Albright syndrome with calcified GH-releasing pituitary adenoma: case report and literature review
Miguel Vega-Arroyo, Martha Lilia Tena-Suck, Celia Teresa de Jesús Álvarez-Gamiño et al
Int J Surg Case Rep. 2018; 53: 61–65
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6215971/
■ McCune-Albright syndrome associated bone marrow failure and extramedullary haematopoisis secondary to fibrous
A J Mahdi, P Connor, I Thakur
British Journal of Haematology 2017, 178,179
https://onlinelibrary.wiley.com/doi/epdf/10.1111/bjh.14690
■ Fibrous dysplasia/McCune-Albrigh syndrome: clinical and translational perspectives
Cemre Robinson, Michael T Collins, Alison M Boyce
Curr Osteoporos Rep. 2016 Oct; 14(5): 178–186
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5035212/
■ McCune-Albright syndrome with craniofacial dysplasia: clinical review and surgical management
T A B Blsuzarri, J F M Araujo, C A M Melro et al
Surg Neurol Int 2016, vol 7 (Suppl 6): S165-S169
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4804399/
■ Carney complex and McCune Albright syndrome: an overview of clinical manifestations and human molecular genetics
Paraskevi Salpea, Constantine A. Stratakis
Mol Cell Endocrinol. 2014 Apr 5; 386(0): 85–91
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3943598/
■ Oral pigmentation in McCune-Albright syndrome
Dominique C. Pichard, Alison M. Boyce, Michael T. Collins, Edward W. Cowen
JAMA Dermatol. 2014 Jul; 150(7): 760–76
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4933654/
■ Acromegaly and McCune-Albright syndrome
Sylvie Salenave, Alison M. Boyce, Michael T. Collins, Philippe Chanson
J Clin Endocrinol Metab. 2014 Jun; 99(6): 1955–1969
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4037730/
■ Dental perspectives in fibrous dysplasia and McCune-Albright syndrome
Sunday O. Akintoye, Alison M. Boyce, Michael T. Collins
Oral Surg Oral Med Oral Pathol Oral Radiol. 2013 Sep; 116(3):
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3851571/
■ Sinonasal disease in polyostotic fibrous dysplasia and McCune Albrigh syndrome
Timothy R. DeKlotz, Hung Jeffrey Kim, Marilyn Kelly, Michael T. Collins
Laryngoscope. 2013 Apr; 123(4): 823–828
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3609909/
■ Palmidronic acid and cabergoline as effective long-term therapy in a 12-year-old girl with extended facial polyostotic fibrous dysplasia, prolactinona and acromegaly in McCune-Albright syndrome: a case report
Carl Friedrich Classen, Monika Mix, Ulrike Kyank,et al
J Med Case Reports. 2012; 6: 32.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3277484/
■ Primary bimorphic adrenocortical disease: cause of hypercolrtisolism in McCune-Albright syndrome
- Aidan Carney, William F. Young, Jr, Constantine A. Stratakis
Am J Surg Pathol. 2011 Sep; 35(9): 1311–1326.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4140081/
■ Oral alendronate treatment for severe polyostotic fibrous dysplasia due to McCune-Albright syndrome in a child: a case report
Ana Luiza Andrade Aragão, Ivani Novato Silva
Int J Pediatr Endocrinol. 2010; 2010: 432060
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2952807/
■ McCune-albright syndrome and acromegaly: hormonal control with use of cabergoline and long-acting somatostatin – case report.
Almeida, João Paulo C. et al.
Arq Bras Endocrinol Metab, Feb 2009, vol.53, no.1, p.102-106
■ Hipertireoidismo relacionado à síndrome de McCune Albright: relato de dois casos e revisão da literatura
Sallum, Ana Carolina R. et al.
Arq Bras Endocrinol Metab, Abr 2008, vol.52, no.3, p.556-561
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302008000300017&lng=en&nrm=iso
■ Clinical, microscopic and imaging findings associated to Mccune-Albright syndrome: report of two cases
Xavier, Samuel Porfírio et al.
Braz. Dent. J., 2008, vol.19, no.2, p.165-17
■ Envolvimento orbitário difuso por displasia fibrosa na síndrome de McCune Albright: relato de caso
Costanzi, Márcio Alberto and Cruz, Antonio Augusto Velasco e
Arq. Bras. Oftalmol., Dez 2007, vol.70, no.6, p.1021-1023
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27492007000600026&lng=en&nrm=iso
■ A case of McCune-Albright syndrome with associated multiple endocrinopathies
S H Sung, H D Yoon, H S Shon et al
Korean J Inter Med 2007, vol 22 (1)
http://www.kjim.org/journal/view.php?number=15960
■ Estudio clínico-molecular de pacientes chilenas con síndrome de McCune-Albright.
Román R, Rossana et al.
Rev. méd. Chile, Dic 2001, vol.129, no.12, p.1365-137
https://scielo.conicyt.cl/scielo.php?script=sci_arttext&pid=S0034-98872001001200001&lng=es&nrm=iso
■ Dr Paulo Fernando Leite
Cardiologia/Prevenção Cardiovascular
Estratificação de Risco Cardiovascular
Consultório: Rua Padre Rolim 815/sala 815
Tel: 33245518 (Consulta Particular/Unimed)
– Centro Médico Unimed BH/Contagem/Pedro I
Belo Horizonte/MG/Brasil
CRMMG: 7026
Email: pfleite1873@gmail.com