Vascular access for chronic hemodialysis in a patient with epidermolysis bullosa dystrophica Hallopeau-Siemens
Vascular access for chronic hemodialysis in a patient with epidermolysis bullosa dystrophica Hallopeau-Siemens
复制标题
DOI：
10.1111/j.1542-4758.2012.00699.x
复制
发表时间：
2013-01-01
影响因子：
1.3
通讯作者：
Peric, Mladen
中科院分区：
文献类型：
--
作者：
Sakan, Sanja;Basic-Jukic, Nikolina;Peric, Mladen
大疱性表皮病是一种罕见的遗传性疾病，以机械性大疱性皮肤病为特征。除皮肤外，这些患者有各种皮外表现，某些类型的大疱性表皮病几乎包括所有器官系统。由于寿命延长，慢性肾功能不全已成为这些患者发病和死亡的重要原因。功能性血管透析通路的建立对医生和患者都是一个巨大的挑战。多学科方法至关重要。我们介绍了一个成功建立透析通路，通过泰西欧导管在一个年轻女子患有大疱性表皮营养不良Hallopeau-Siemens和终末期肾病。从那时起，通过右颈内静脉插入的Tesio导管一直是透析的功能手段。尽管患有致残性疾病，患者仍有机会过上高质量和积极的生活。已报告了几例使用透析导管经中心静脉成功建立透析通路的病例。我们报告了通过Tesio导管成功建立长期透析通路，并建议这种方法是这些患者的理想选择。这是第一份报告，涉及血管通路在这组患者。
Epidermolysis bullosa is a rare genetic hereditary disease characterized with mechanobullous dermatosis. Except cutaneous, these patients have various extracutaneous manifestations and some types of epidermolysis bullosa comprise almost all organ systems. Because of prolonged life span, chronic renal insufficiency has become an important cause of morbidity and death in these patients. Establishment of functional vascular dialysis access is a great challenge for both the doctors and the patients. Multidisciplinary approach is essential. We present a case of successful establishment of dialysis access via Tesio catheter in a young woman suffering from epidermolysis bullosa dystrophica Hallopeau-Siemens and end-stage renal disease. Since then, the Tesio catheter inserted via the right internal jugular vein has been the functional mean of dialysis. The patient was given the opportunity to lead a quality and active life in spite of disabling disease. Several cases of successful dialysis access establishment with dialysis catheters via central veins have been reported. We report the successful establishment of long-term dialysis access via Tesio catheter and suggest this approach as ideal for these patients. This is the first report dealing with vascular access in this group of patients.