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Bone Abstracts (2019) 7 P38 | DOI: 10.1530/boneabs.7.P38

ICCBH2019 Poster Presentations (1) (226 abstracts)

Clinical features and approach to treatment in pediatric patients with McCune-Albright syndrome: monocentric experience

Nadezhda Makazan , Elizaveta Orlova , Maria Kareva , Anna Kolodkina , Natalia Kalinchenko , Michael Petrov , Natalia Zubkova & Valentina Peterkova

Endocrinology Research Centre, Moscow, Russian Federation.

McCune-Albright-Syndrome (MAS) is a rare multisystem disorder presenting with polyostotic fibrous dysplasia accompanied by a list of extraskeletal features including café-aulait spots and various endocrine hyperfunctioning. There is no effective treatment for FD in MAS nowadays. Patients with MAS (n=60, 49 girls(G) and 11 boys (B) have been diagnosed and followed up during 20 years in the Institute of Pediatric Endocrinology. First clinical manifestations were peripheral precocious puberty (PPP) in 77%, fibrous dysplasia (FD) in 18% and Cushing’s syndrome (CS) in 5% of patients. PPP was seen as early as in 2 months-girl, fractures due to FD were seen since the age of 9 months, and CS was revealed in patients younger than 1-year. The way of treatment was determined for each patient according to the clinical signs. Girls with PPP with recurrent estrogen secreting ovary cysts were prescribed with antiestrogen treatment; long acting octreotide was given in case of growth hormone overproduction, methimazole was used for management of hyperthyroidism with 4 patients undergone thyroidectomy because of tissue overgrowth, oral phosphorus and calcitriol were given for management severe hypophosphatemia, adrenalectomy was the way of treatment for Cushing syndrome. Curettage and bone grafting was the most often used approach to the treatment of FD of low extremities. Unfortunately, surgery did not prevent recurrent fractures and progressive limb deformities in severe cases when the patients had total or subtotal FD involvement. Two girls underwent intramedullary nailing for treatment of femur fractures/In one case malposition occurred one year after the surgery and removal of the nail was performed. MAS can manifest in children at the age less than 1-year-old and involves the vast majority of tissues with different types of combinations of the features. Treatment of fibrous dysplasia is a challenging task with low efficacy, orthopedic surgery is controversial and requires more experience and further investigations.

Disclosure: The authors declared no competing interests.

Volume 7

9th International Conference on Children's Bone Health


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