Thomson S D, Chmura A M, Slapak M
Wessex Regional Transplant Unit, St Mary's Hospital, Portsmouth, England, UK.
J R Soc Med. 1995 Sep;88(9):532P-533P.
Marfan's syndrome is an autosomal dominant condition with a prevalence of 4 to 6:100,000, and classically comprises skeletal changes, ectopia lentis and cystic medial necrosis of the largest arteries. The latter leads to aneurysm formation, most often commencing at the aortic root, and is responsible for an average age at death of 32 years. We describe successful preservation of threatened renal function by renal autotransplantation, without concurrent aortic replacement, following dissection of the descending aorta in a patient with Marfan's syndrome.
马凡综合征是一种常染色体显性疾病,发病率为4至6/10万,典型表现包括骨骼改变、晶状体异位以及最大动脉的中层囊性坏死。后者会导致动脉瘤形成,最常见于主动脉根部,这也是导致患者平均死亡年龄为32岁的原因。我们描述了1例马凡综合征患者在降主动脉夹层分离后,未同时进行主动脉置换,而是通过自体肾移植成功保留了受到威胁的肾功能。