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von Hippel-Lindau disease.

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Title: von Hippel-Lindau disease.
Authors: Lonser RR (AUTHOR), Glenn GM (AUTHOR), Walther M (AUTHOR), Chew EY (AUTHOR), Libutti SK (AUTHOR), Linehan WM (AUTHOR), Oldfield EH (AUTHOR), Lonser, Russell R (AUTHOR), Glenn, Gladys M (AUTHOR), Walther, McClellan (AUTHOR), Chew, Emily Y (AUTHOR), Libutti, Steven K (AUTHOR), Linehan, W Marston (AUTHOR), Oldfield, Edward H (AUTHOR)
Source: Lancet. 6/14/2003, Vol. 361 Issue 9374, p2059-2067. 9p.
Abstract: von Hippel-Lindau disease is a heritable multisystem cancer syndrome that is associated with a germline mutation of the VHL tumour suppressor gene on the short arm of chromosome 3. This disorder is not rare (about one in 36000 livebirths) and is inherited as a highly penetrant autosomal dominant trait (ie, with a high individual risk of disease). Affected individuals are at risk of developing various benign and malignant tumours of the central nervous system, kidneys, adrenal glands, pancreas, and reproductive adnexal organs. Because of the complexities associated with management of the various types of tumours in this disease, treatment is multidisciplinary. We present an overview of the clinical aspects, management, and treatment options for von Hippel-Lindau disease. [ABSTRACT FROM AUTHOR]
Copyright of Lancet is the property of Lancet and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Psychology and Behavioral Sciences Collection
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  Data: von Hippel-Lindau disease.
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  Data: <searchLink fieldCode="AR" term="%22Lonser+RR%22">Lonser RR</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Glenn+GM%22">Glenn GM</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Walther+M%22">Walther M</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Chew+EY%22">Chew EY</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Libutti+SK%22">Libutti SK</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Linehan+WM%22">Linehan WM</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Oldfield+EH%22">Oldfield EH</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Lonser%2C+Russell+R%22">Lonser, Russell R</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Glenn%2C+Gladys+M%22">Glenn, Gladys M</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Walther%2C+McClellan%22">Walther, McClellan</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Chew%2C+Emily+Y%22">Chew, Emily Y</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Libutti%2C+Steven+K%22">Libutti, Steven K</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Linehan%2C+W+Marston%22">Linehan, W Marston</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Oldfield%2C+Edward+H%22">Oldfield, Edward H</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22Lancet%22">Lancet</searchLink>. 6/14/2003, Vol. 361 Issue 9374, p2059-2067. 9p.
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  Data: von Hippel-Lindau disease is a heritable multisystem cancer syndrome that is associated with a germline mutation of the VHL tumour suppressor gene on the short arm of chromosome 3. This disorder is not rare (about one in 36000 livebirths) and is inherited as a highly penetrant autosomal dominant trait (ie, with a high individual risk of disease). Affected individuals are at risk of developing various benign and malignant tumours of the central nervous system, kidneys, adrenal glands, pancreas, and reproductive adnexal organs. Because of the complexities associated with management of the various types of tumours in this disease, treatment is multidisciplinary. We present an overview of the clinical aspects, management, and treatment options for von Hippel-Lindau disease. [ABSTRACT FROM AUTHOR]
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  Data: <i>Copyright of Lancet is the property of Lancet and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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        Value: 10.1016/s0140-6736(03)13643-4
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              Text: 6/14/2003
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