Von Hippel-Lindau disease in an adolescent

Von Hippel-Lindau disease is a hereditary neoplastic syndrome that generally manifests in early adulthood but does present occasionally in adolescence. In the past, diagnosis and management of this disorder fell within the domain of internists and surgeons. Because pediatricians are now seeing older...

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Published inPediatrics (Evanston) Vol. 79; no. 4; p. 632
Main Authors Seitz, M L, Shenker, I R, Leonidas, J C, Nussbaum, M P, Wind, E S
Format Journal Article
LanguageEnglish
Published United States 01.04.1987
Subjects
Abdomen
Adolescent
Angiomatosis - diagnosis
Angiomatosis - physiopathology
Angiomatosis - surgery
Cerebellar Neoplasms - complications
Female
Hemangiosarcoma - complications
Humans
Pain - etiology
Pancreatic Cyst - complications
von Hippel-Lindau Disease - diagnosis
von Hippel-Lindau Disease - physiopathology
von Hippel-Lindau Disease - surgery
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Summary:Von Hippel-Lindau disease is a hereditary neoplastic syndrome that generally manifests in early adulthood but does present occasionally in adolescence. In the past, diagnosis and management of this disorder fell within the domain of internists and surgeons. Because pediatricians are now seeing older patients, they must learn to recognize the various components of this disorder. Additionally, they must be able to assure appropriate medical evaluation and follow-up and also arrange for genetic counseling. Pediatricians already attuned to the benefits of preventive medicine should find themselves uniquely qualified to provide the level of care that this disease requires.
ISSN:0031-4005