Von Hippel-Lindau Disease: A Rare Familiar Multi-System Disorder and the Impactof the Clinical Nurse Specialist

摘要

Von Hippel-Lindau disease is a rare-familiar multi-system disorder thatpredisposes tumor formation. The lesions that most commonly cause morbidity and mortality are renal cell carcinoma, cerebellar and spinal cord hemangioblastomas, retinal angiomas and pheochromocytomas. The genetics, symptomatology, and medical treatments are reviewed. A case study is presented. Often the psychosocial aspects of a complex illness are ignored. The role of the clinical nurse specialist can be instrumental in a positive outcome for the patient and family affected by such rare multi-system disorders.