نتایج جستجو برای: maccune albright syndrome
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McCune Albright syndrome is diagnosed by a constellation of Café-au-lait spots, multiostosis fibrous dysplasia and precocious puberty. Other endocrine dysfunctions which are often associated are growth hormone excess, hyperthyroidism, Cushings syndrome, hyperprolactinemia and phosphate wasting. Its prevalence is 1:100000–1:1000000 in different ethnicity [1]. It is a rare cause of precocious pub...
Fibrous dysplasia is a benign bone lesion of unknown etiology. Bone involvement usually is solitary (monostotic). Multiple forms (polyostotic) associated with extra skeletal symptoms, particularly cutaneous pigmentation, endocrine dysfunction and precocious puberty is called McCune-Albright syndrome (MAS). We report the case of a 40-year-old man who presented with left mandibular body expansion...
The first three disorders discussed are abnormalities of bone: too little bone in cleidocranial dysplasia caused by mutations in RUNX2; too much bone in fibrodysplasia ossificans progressiva with overexpression of BMP4; and abnormal bone in McCune-Albright syndrome and fibrous dysplasia caused by mutations in GNAS1. Disorders of the sonic hedgehog signaling network are discussed next, including...
Objectives: The use of drug therapy based on cabergoline, octreotide and long-acting release (LAR) octreotide has presented varying results in the treatment of GH excessive production in patients with McCune-Albright Syndrome. Methods: We report the case of a 29 year-old female patient presenting McCune-Albright Syndrome and complaint of excessive bone growth. Results: The patient presented a p...
the classical triad of mccune-albright syndrome (mas) consists of polyostotic fibrous dysplasia (fd), skin hyperpigmentation (café-au-lait spots), and endocrine dysfunction, frequently seen in females as precocious puberty. etiology is genetically based and is explained by mosaicism of activating somatic mutations of the alpha-subunit of gs protein. clinical presentation is varied and is depend...
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