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Von Hippel-Lindau disease: an overview

Michele Inglese1

  • 1UMDNJ School of Nursing, Newark NJ, USA. j-minglese@mindspring.com

Nephrology Nursing Journal : Journal of the American Nephrology Nurses' Association
|September 26, 2007
PubMed

Insights

Von Hippel-Lindau disease is a rare genetic disorder causing tumors in various organs. This overview focuses on renal manifestations and essential nursing care for affected patients.

Area of Science:

  • Oncology
  • Genetics
  • Nephrology

Background:

  • Von Hippel-Lindau disease is a rare, inherited disorder characterized by the development of multiple tumors.
  • Tumors can affect various organs including the eyes, brain, adrenal glands, pancreas, liver, spinal cord, and kidneys.
  • Renal tumors in VHL disease are predominantly renal cell carcinoma, appearing as primary or secondary manifestations.

Purpose of the Study:

  • To provide a comprehensive overview of Von Hippel-Lindau disease.
  • To detail the renal manifestations of Von Hippel-Lindau disease.
  • To outline the nursing care strategies for patients with renal involvement in VHL disease.

Main Methods:

  • Literature review of Von Hippel-Lindau disease.
  • Analysis of renal tumor pathology in VHL.
  • Synthesis of nursing care principles for VHL patients with kidney issues.

Main Results:

  • Von Hippel-Lindau disease involves a spectrum of tumors across multiple organ systems.
  • Renal cell carcinoma is the most common renal tumor associated with VHL.
  • Renal involvement can be the initial presentation or a later development in the disease course.

Conclusions:

  • Understanding VHL disease is crucial for early diagnosis and management.
  • Renal involvement requires specific attention due to the high incidence of renal cell carcinoma.
  • Effective nursing care is vital for managing VHL patients with renal complications.

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