Von Hippel-Lindau: Current Evidence in Diagnosis, Treatment, and Nursing Implications.

BACKGROUND: Von Hippel-Lindau (VHL) is a rare autosomal dominant hereditary disorder that predisposes individuals to benign and malignant tumors in the brain, eyes, kidneys, pancreas, genital tract, or other body parts. The VHL gene, which is located on the short arm of chromosome 3, prevents cells from dividing too rapidly. Mutations in the VHL gene result in uncontrollable cell growth and tumor formation. OBJECTIVES: The purpose of this article is to summarize the current research literature describing diagnosis, treatment, and nursing implications of VHL. METHODS: Three electronic databases, relevant journals, and relevant websites were searched. FINDINGS: The majority of patients affected with VHL have an affected parent, but a small percentage develop VHL from a new mutation that takes place in a single egg or sperm during conception or from a post-conception mutation. Genetic testing, either through sequence analysis, Southern blot analysis, or quantitative polymerase chain reaction, is considered standard in evaluating patients suspected of having VHL. A diagnosis of VHL can be made by identifying one VHL tumor for a patient who has a confirmed family history of VHL. The presence of at least two tumors is required to make a diagnosis of VHL in a patient without a positive family history. The nursing role includes providing resources on VHL genetic counseling, genetic testing, and palliative care.

Von Hippel-Lindau disease: an overview.

Von Hippel-Lindau disease is a rare, familial disease consisting of multiple tumors, which can present in the eyes, brain, adrenal gland, pancreas, liver, spinal cord, kidneys, or other areas of the body. The renal tumors are primarily renal cell carcinoma. Renal involvement may be the primary manifestation, but most frequently it is a secondary manifestation. This article presents an overview of von Hippel-Lindau disease and the nursing care of the patient with renal involvement.

von Hippel-Lindau disease: a genetic condition predisposing tumor formation.

Von Hippel-Lindau disease (VHL) is a rare, autosomal, dominantly transmitted disease that genetically predisposes affected people to tumor development. The most common manifestations of the disease and those associated with the greatest morbidity and mortality are cerebellar, spinal, and medullary hemangioblastomas, retinal angiomas, renal cell carcinomas, and pheochromocytomas. In this paper the genetic transmission, tumorigenesis, diagnosis, treatment, surveillance methods, and nursing implications associated with VHL are reviewed. The importance of early detection and the need for long-term follow-up are emphasized. Oncology nurses will need to stay abreast of the genetic aspects of cancer as information in this area increases and as expanded roles bring them in closer contact with patients and families affected by these conditions.

A patient with Lindau-von Hippel disease--a nursing challenge.

After six weeks participation in the rehabilitation program, D.K. was ready for discharge. He was receiving medications for symptomatic treatment: fluorocortisone acetate, cortisone acetate, and cimetidine. Rehabilitation goals were met to some degree; his strength was increased significantly and both motor control and balance were slightly better than at admission. He and his family recognized the need for ongoing care and agreed to be seen for follow-up treatment at the rehabilitation center.