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Orthopaedic Surgery


Answer 5
  1. McCune-Albright syndrome. McCune-Albright syndrome is a condition with the classic triad of polyostotic fibrous dysplasia, cutaneous café au lait spots, and endocrine dysfunction.5 Precocious puberty and vaginal bleeding are also common in young female patients with McCune-Albright syndrome. Bone lesions typically have a ground-glass appearance on radiography and occur most commonly in the femur (91%) and tibia (81%).5 Letterer-Siwe disease (fulminant fatal form of histiocytosis X) and Hand-Schüller- Christian disease (classic triad of exophthalmus, diabetes insipidus, and lytic skull lesions) are associated with eosinophilic granulomatous lesions, not fibrous dysplasia. Paget’s disease is a form of abnormal bone remodeling found in adults. Gardner’s syndrome is an autosomal dominant disorder manifested in childhood by multiple neoplasms (ie, bone and mesenteric tumors, fatty and fibrous skin, and intestinal polyps).

    REFERENCE
    5.
     Parekh SG, Donthineni-Rao R, Ricchetti E, Lackman RD. Fibrous dysplasia. J Am Acad Orthop Surg 2004;12:305-13

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