NEUROFIBROMATOSIS COMPLICATED WITH XXX-SYNDROME AND RENOVASCULAR HYPERTENSION

A 25-year-old woman with neurofibromatosis was admitted to our hospita l for evaluation of hyper-tension, When she was 6 years old, she was d iagnosed as having neurofibromatosis and XXX syndrome because of multi ple cafe-au-lait spots, neurofibromas of the skin and mental retardati on. Chromosome analysis revealed that her karyotype was 46, XX/47, XXX . Renal arteriography disclosed aneurysmal change and stenosis of the right renal artery. After right-side nephrectomy and aneurysmectomy, t he kidney was autotransplanted in the left iliac fossa. Surgical proce dure resulted in marked amelioration of the hypertension without medic al treatment. Thus, aortorenal bypass and renal auto-transplantation h ave emerged as the preferred revascularization operations, This is the first report of a chromosomal linkage between neurofibromatosis which is thought to be an autosomal dominant disease and the XXX syndrome.