Prevention of large-vessel stenoses in atypical hemolytic uremic syndrome associated with complement dysregulation
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Jean-Claude Davin & Charles Majoie & Jaap Groothoff & Valentina Gracchi & Antonia Bouts & Timothy H. J. Goodship & Chantal Loirat
- چاپ و سال / کشور: 2011
Description
The oldest of three sisters, two of whom are monozygous twins, presenting all with atypical hemolytic uremic syndrome (aHUS) associated with a CFH mutation, was referred at the age of 3 years to the Emma Children’s Hospital/Academic Medical Centre of Amsterdam (Fig. 1) [1, 2]. She never recovered renal function and commenced peritoneal dialysis. Bilateral nephrectomy was performed 1 year later because of hypertension. She lost two transplants by recurrence, one immediately posttransplantation and the other one 2 months posttransplant despite preventive plasma exchanges (PEs) contemporary to frequency reduction of PE. From the age of 15 years, she began to experience transient sensory and motor symptoms on both sides of her body associated with low blood pressure (BP) during hemodialysis. Magnetic resonance angiogram (MRA) showed severe stenoses of both middle and both anterior cerebral arteries (Fig. 2a) (partial report in [3]). She did not present any additional risk factors for developing arteriosclerosis (such as long-duration hypertension, diabetes, uncontrolled hyperparathyroidism). No arterial calcification and no left ventricular hypertrophy were shown.
Pediatr Nephrol (2011) 26:155–157 DOI 10.1007/s00467-010-1608-9 Received: 1 June 2010 / Revised: 8 June 2010 / Accepted: 9 June 2010 / Published online: 24 July 2010
