polyostotic fibrous dysplasia
Noun | 1. | polyostotic fibrous dysplasia - fibrous dysplasia of bone affecting multiple bones |
单词 | polyostotic fibrous dysplasia | |||
释义 | polyostotic fibrous dysplasia
polyostotic fibrous dysplasiapol·y·os·tot·ic fi·brous dys·pla·si·aMcCune-Albright syndromeAn autosomal dominant condition (OMIM:174800) due to altered regulation of cAMP, endocrinopathy (e.g., hyperthyroidism) and hypophosphatemia.Clinical findings Precocious puberty, polyostotic (cystic fibrous dysplasia) spontaneous fractures at young age, café-au-lait spots on skin, ovarian cysts. Lab Cyclical 4–6-week fluctuations of plasma oestrogen; afflicted young girls have decreased gonadotropins, decreased response to LH-RH; increased testosterone, increased alkaline phosphatase. Molecular pathology Defects in GNAS, which encodes a G protein that modulates various membrane signalling cascasdes, cause McCune-Albright syndrome. Management Aromatase inhibitor testolactone. polyostotic fibrous dysplasiapolyostotic fibrous dysplasia
Synonyms for polyostotic fibrous dysplasia
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