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Albrights Syndrome

Discussion

  • consists of polyostotic fibrous dysplasia, cafe'-au-lait spots of "coast of Maine" variety, and premature menses;
  • it is more common in girls than boys and accompanies approx 1 in 30 cases of polyostotic fibrous dysplasia;
  • there is appreciable coincidence of other endocrinologic dz including hyperthyroidism & Cushing's dz as well as hyperparathyroidism and hypophosphatemic rickets;
  • in polyostotic cases of fibrous dysplasia, dx is usually not difficult;
  • in monostotic cases, the radiographs are frequently not diagnostic and biopsy may be required;
  • malignant transformation is seen in less than 0.5%, with osteogenic sarcoma being the most common neoplasm
  • ref: Images in Clinical Medicine. Albright's Hereditary Osteodystrophy