McCune-Albright syndrome: a case report.
نویسندگان
چکیده
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 and intermittent suppuration from the skin sinus tract formation since he was 18 years old. He had skeletal deformities, limping, and multiple skin pigmentation. X-ray revealed multiple fractures and radiolucent lesions in numerous bones. Laboratory analysis showed an increased serum alkaline phosphatase. Precocious puberty was determined upon taking the patient's history. The patient's height was 148 cm. Microscopic findings of the mandibular lesion, clinical presentation and X-ray findings were strongly diagnostic for MAS.
منابع مشابه
McCune-Albright Syndrome: A Case Report and Literature Review
McCune-Albright syndrome (MAS) is a rare, heterogenous, clinical condition caused by a rare genetic mutation. The disorder is more common in females and is characterized by a triad of cutaneous, bone and endocrine abnormalities. We describe a girl patient with MAS having precocious puberty and multiple cafe-au-lait macules and deforming polyostotic fibrous dysplasia of bone. Clinical presentat...
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McCune-Albright Syndrome (MAS) is a rare sporadic disease characterized by bone fibrous dysplasia, Café au lait spots and a variable association of hyperfunction endocrine disorders. There is not any certain treatment available for this syndrome, and both physical and emotional disability in these patients is still a major concern for physicians. In present report we have described a 10- year-o...
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A 29- year old female with bone pain and history of precocious puberty was referred for bone scintigraphy. On physical examination café au lait macular spots were noted on her neck, buttocks and left leg. Bone scan showed multiple areas of intense increased activity which was in favour of polyostotic fibrous dysplasia. Considering the presence of polyostotic fibrous...
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Four year old girl was evaluated for precocious puberty. Breast development and pubic hair growth had started at age 2½ year, and had been followed by vaginal bleeding resembling periods. Gaf-au-lait spots were present in most of her skin surface. Bone x-rays showed multiple cysts in pelvis and the metaphysis of femur. Diagnosis of McCune-Albright syndrome was made. Concentrations of all hormon...
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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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The McCune Albright syndrome is seldom encountered and rarely reported in dental literature. It represents a special category of polyostic fibrous displasia associated with skin pigmentation and endocrine disturbances. We describe the case of a 9-year-old boy with the McCune Albright syndrome, who was treated for a mandibular osteolytic lesion.
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ورودعنوان ژورنال:
- Archives of Iranian medicine
دوره 13 3 شماره
صفحات -
تاریخ انتشار 2010