Couri Carlos Eduardo Barra, da Silva Geruza Alves, Martinez José Antônio Baddini, Pereira Francisco de Assis, de Paula Francisco José Albuquerque
Department of Internal Medicine, School of Medicine of Ribeirão Preto, University of São Paulo, Ribeirão Preto, Brazil.
BMC Cardiovasc Disord. 2005 Dec 12;5:34. doi: 10.1186/1471-2261-5-34.
Since its first description, Mönckeberg's sclerosis has only been related to arterial media calcification, being listed among the primary diseases of the vessels.
We report here a clinically and histologically confirmed case of Mönckeberg's sclerosis in which the patient presented with massive areas of soft tissue calcifications in the pharynx and larynx. Polysomnographic parameters showed severe obstructive apnea refractory to nasal continuous positive airway pressure. Clinical and laboratory findings excluded concomitant endocrine or rheumatological diseases.
Our data provide a new insight about Mönckeberg's sclerosis, i.e., the fact that the etiopathogenic process involved in the phenomenon of calcification may not be restricted only to the arteries, but may occur in the entire organism. Further studies of the etiopathogenesis of this disease are needed.
自首次被描述以来,门克贝格硬化症仅与动脉中层钙化有关,被列为血管原发性疾病之一。
我们在此报告一例经临床和组织学确诊的门克贝格硬化症病例,该患者咽部和喉部出现大面积软组织钙化。多导睡眠图参数显示,患者对经鼻持续气道正压通气治疗难治的严重阻塞性呼吸暂停。临床和实验室检查结果排除了合并内分泌或风湿性疾病。
我们的数据为门克贝格硬化症提供了新的见解,即钙化现象所涉及的病因发病过程可能不仅局限于动脉,而是可能发生在整个机体。需要对该疾病的病因发病机制进行进一步研究。