Von Hippel-Lindau disease: an evaluation of natural history and functional disability.

ABSTRACT:

Background

Although many studies have been published about specific lesions characterizing von Hippel-Lindau(VHL) disease, none have dealt with the natural history of the whole disease and the consequent disabilities. We aim to define the comprehensive natural history of VHL disease and to describe the functional disabilities and their impact upon patients' quality of life, thereby tailoring the follow-up schedule accordingly.

Methods

We performed a prospective analysis on 128 VHL-affected patients beginning in 1996. For each affected organ, we defined intervals between the first and subsequent VHL-related manifestations and compared them with current VHL surveillance protocols. We looked for any association of the number of involved organs with age, sex, type of VHL gene mu

SUBMITTER: Feletti A 

PROVIDER: S-EPMC4896541 | biostudies-literature | 2016 Jul

REPOSITORIES: biostudies-literature