Gorlin's syndrome, or nevoid basal cell carcinoma syndrome.
Identifieur interne : 002997 ( Pmc/Checkpoint ); précédent : 002996; suivant : 002998Gorlin's syndrome, or nevoid basal cell carcinoma syndrome.
Auteurs : P. J. Fitzpatrick ; G. A. ThompsonSource :
- Canadian Medical Association Journal [ 0008-4409 ] ; 1982.
Abstract
Gorlin's syndrome is a condition inherited in an autosomal dominant fashion. It involves many organs, but principally affects the skin, skeleton, and endocrine and nervous systems. The most common features are multiple nervi and basal cell carcinomas of the skin, benign jaw cysts, dyskeratotic pits in the palms and soles, rib and vertebral abnormalities, brachymetacarpalism, and calcification of the falx cerebri. In 14 patients, 4 of whom belonged to one family, the age at the time of diagnosis ranged from 11 to 63 years. Ten patients are alive, but five are severely disfigured by carcinomas. Two patients died of complications resulting from uncontrolled tumours, and two died of other cancers. New skin tumours constantly develop; small ones can be excised, but large ones require extensive surgery with or without radiotherapy.
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PubMed: 7116263
PubMed Central: 1862096
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Gorlin's syndrome, or nevoid basal cell carcinoma syndrome.</title><author><name sortKey="Fitzpatrick, P J" sort="Fitzpatrick, P J" uniqKey="Fitzpatrick P" first="P. J." last="Fitzpatrick">P. J. Fitzpatrick</name></author><author><name sortKey="Thompson, G A" sort="Thompson, G A" uniqKey="Thompson G" first="G. A." last="Thompson">G. A. Thompson</name></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">7116263</idno><idno type="pmc">1862096</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1862096</idno><idno type="RBID">PMC:1862096</idno><date when="1982">1982</date><idno type="wicri:Area/Pmc/Corpus">001498</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">001498</idno><idno type="wicri:Area/Pmc/Curation">001498</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">001498</idno><idno type="wicri:Area/Pmc/Checkpoint">002997</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">002997</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Gorlin's syndrome, or nevoid basal cell carcinoma syndrome.</title><author><name sortKey="Fitzpatrick, P J" sort="Fitzpatrick, P J" uniqKey="Fitzpatrick P" first="P. J." last="Fitzpatrick">P. J. Fitzpatrick</name></author><author><name sortKey="Thompson, G A" sort="Thompson, G A" uniqKey="Thompson G" first="G. A." last="Thompson">G. A. Thompson</name></author></analytic><series><title level="j">Canadian Medical Association Journal</title><idno type="ISSN">0008-4409</idno><imprint><date when="1982">1982</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Gorlin's syndrome is a condition inherited in an autosomal dominant fashion. It involves many organs, but principally affects the skin, skeleton, and endocrine and nervous systems. The most common features are multiple nervi and basal cell carcinomas of the skin, benign jaw cysts, dyskeratotic pits in the palms and soles, rib and vertebral abnormalities, brachymetacarpalism, and calcification of the falx cerebri. In 14 patients, 4 of whom belonged to one family, the age at the time of diagnosis ranged from 11 to 63 years. Ten patients are alive, but five are severely disfigured by carcinomas. Two patients died of complications resulting from uncontrolled tumours, and two died of other cancers. New skin tumours constantly develop; small ones can be excised, but large ones require extensive surgery with or without radiotherapy.</p><sec sec-type="scanned-figures"><title>Images</title><fig id="F1"><label>FIG. 1</label><graphic xlink:href="canmedaj01375-0021-a" xlink:role="467"></graphic></fig><fig id="F2"><label>FIG. 2</label><graphic xlink:href="canmedaj01375-0021-b" xlink:role="467"></graphic></fig><fig id="F3"><label>FIG. 3</label><graphic xlink:href="canmedaj01375-0021-c" xlink:role="467"></graphic></fig><fig id="F4"><label>FIG. 4</label><graphic xlink:href="canmedaj01375-0021-d" xlink:role="467"></graphic></fig><fig id="F5"><label>FIG. 5</label><graphic xlink:href="canmedaj01375-0022-a" xlink:role="468"></graphic></fig><fig id="F6"><label>FIG. 6</label><graphic xlink:href="canmedaj01375-0022-b" xlink:role="468"></graphic></fig><fig id="F7"><label>FIG. 7</label><graphic xlink:href="canmedaj01375-0022-c" xlink:role="468"></graphic></fig><fig id="F8"><label>FIG. 9</label><graphic xlink:href="canmedaj01375-0023-a" xlink:role="469"></graphic></fig><fig id="F9"><label>FIG. 10</label><graphic xlink:href="canmedaj01375-0023-b" xlink:role="469"></graphic></fig><fig id="F10"><label>FIG. 11</label><graphic xlink:href="canmedaj01375-0023-c" xlink:role="469"></graphic></fig></sec></div></front></TEI><pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Can Med Assoc J</journal-id><journal-title>Canadian Medical Association Journal</journal-title><issn pub-type="ppub">0008-4409</issn></journal-meta><article-meta><article-id pub-id-type="pmid">7116263</article-id><article-id pub-id-type="pmc">1862096</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title>Gorlin's syndrome, or nevoid basal cell carcinoma syndrome.</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Fitzpatrick</surname><given-names>P. J.</given-names></name></contrib><contrib contrib-type="author"><name><surname>Thompson</surname><given-names>G. A.</given-names></name></contrib></contrib-group><pub-date pub-type="ppub"><day>15</day><month>9</month><year>1982</year></pub-date><volume>127</volume><issue>6</issue><fpage>465</fpage><lpage>470</lpage><abstract><p>Gorlin's syndrome is a condition inherited in an autosomal dominant fashion. It involves many organs, but principally affects the skin, skeleton, and endocrine and nervous systems. The most common features are multiple nervi and basal cell carcinomas of the skin, benign jaw cysts, dyskeratotic pits in the palms and soles, rib and vertebral abnormalities, brachymetacarpalism, and calcification of the falx cerebri. In 14 patients, 4 of whom belonged to one family, the age at the time of diagnosis ranged from 11 to 63 years. Ten patients are alive, but five are severely disfigured by carcinomas. Two patients died of complications resulting from uncontrolled tumours, and two died of other cancers. New skin tumours constantly develop; small ones can be excised, but large ones require extensive surgery with or without radiotherapy.</p><sec sec-type="scanned-figures"><title>Images</title><fig id="F1"><label>FIG. 1</label><graphic xlink:href="canmedaj01375-0021-a" xlink:role="467"></graphic></fig><fig id="F2"><label>FIG. 2</label><graphic xlink:href="canmedaj01375-0021-b" xlink:role="467"></graphic></fig><fig id="F3"><label>FIG. 3</label><graphic xlink:href="canmedaj01375-0021-c" xlink:role="467"></graphic></fig><fig id="F4"><label>FIG. 4</label><graphic xlink:href="canmedaj01375-0021-d" xlink:role="467"></graphic></fig><fig id="F5"><label>FIG. 5</label><graphic xlink:href="canmedaj01375-0022-a" xlink:role="468"></graphic></fig><fig id="F6"><label>FIG. 6</label><graphic xlink:href="canmedaj01375-0022-b" xlink:role="468"></graphic></fig><fig id="F7"><label>FIG. 7</label><graphic xlink:href="canmedaj01375-0022-c" xlink:role="468"></graphic></fig><fig id="F8"><label>FIG. 9</label><graphic xlink:href="canmedaj01375-0023-a" xlink:role="469"></graphic></fig><fig id="F9"><label>FIG. 10</label><graphic xlink:href="canmedaj01375-0023-b" xlink:role="469"></graphic></fig><fig id="F10"><label>FIG. 11</label><graphic xlink:href="canmedaj01375-0023-c" xlink:role="469"></graphic></fig></sec></abstract></article-meta></front></pmc><affiliations><list></list><tree><noCountry><name sortKey="Fitzpatrick, P J" sort="Fitzpatrick, P J" uniqKey="Fitzpatrick P" first="P. J." last="Fitzpatrick">P. J. Fitzpatrick</name><name sortKey="Thompson, G A" sort="Thompson, G A" uniqKey="Thompson G" first="G. A." last="Thompson">G. A. Thompson</name></noCountry></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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