지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
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Osteopetrosis is a rare genetic bone disease characterized by increased bone densitybut prone to breakage due to defective osteoclastic function. Among two primary typesof autosomal dominant osteopetrosis (ADO), osteopetrosis type II is characterized bysclerosis of bones, predominantly involving the spine, the pelvis, and the skull base. Fra-gility of bones and dental abscess are leading complications. This report presents a caseof osteopetrosis in a 52-years-old female, which was complicated by the developmentof cavernous sinus thrombophlebitis and meningitis. She was suffered from multiplefractures since one year ago. Laboratory data revealed elevated serum levels of tartrateresistant acid phosphatase (TRAP) without carbonic anhydrase II DNA mutation. A tho-racolumbar spine X-ray showed, typical findings of ADO type II (ADO II; Albers-Schön-berg disease), prominent vertebral endplates so called the ‘rugger jersey spine’. Her oldersister also showed same typical spine appearance. We report a case of ADO II with cav-ernous sinus thrombophlebitis and meningitis that was successfully treated with long-term antibiotics with right sphenoidotomy.
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참고문헌 (16)
참고문헌 신청
Albers-Schonberg HE. / 1904 / Rontgenbilder einer seltenen knochenerkrankung / Munch Med Wochenschr. 51 : 365 ~ 368
Benichou OD. / 2000 / Type II autosomal dominant osteopetrosis (Albers-Schonberg disease): clinical and radiological manifestations in 42 patients / Bone. 26 : 87 ~ 93
Bollerslev J. / 1988 / Radiological, biochemical and hereditary evidence of two types of autosomal dominant osteopetrosis / Bone. 9 : 7 ~ 13
Campos-Xavier AB. / 2003 / Chloride channel 7 (CLCN7) gene mutations in intermediate autosomal recessive osteopetrosis / Hum Genet. 112 : 186 ~ 189
Cannon ML. / 2004 / Cavernous sinus thrombosis complicating sinusitis / Pediatr Crit Care Med. 5 : 86 ~ 88
Benichou OD. / 2000 / Type II autosomal dominant osteopetrosis (Albers-Schonberg disease): clinical and radiological manifestations in 42 patients / Bone. 26 : 87 ~ 93
Bollerslev J. / 1988 / Radiological, biochemical and hereditary evidence of two types of autosomal dominant osteopetrosis / Bone. 9 : 7 ~ 13
Campos-Xavier AB. / 2003 / Chloride channel 7 (CLCN7) gene mutations in intermediate autosomal recessive osteopetrosis / Hum Genet. 112 : 186 ~ 189
Cannon ML. / 2004 / Cavernous sinus thrombosis complicating sinusitis / Pediatr Crit Care Med. 5 : 86 ~ 88
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