The role of lymphoscintigraphy in the diagnosis of lymphedema in Turner syndrome.
Identifieur interne : 005F79 ( Main/Exploration ); précédent : 005F78; suivant : 005F80The role of lymphoscintigraphy in the diagnosis of lymphedema in Turner syndrome.
Auteurs : C. Bellini [Italie] ; E. Di Battista ; F. Boccardo ; C. Campisi ; G. Villa ; G. Taddei ; C. Traggiai ; A. Amisano ; A. Amisamo ; P Polo Perucchin ; C S Benfenati ; E. Bonioli ; R. LoriniSource :
- Lymphology [ 0024-7766 ] ; 2009.
Descripteurs français
- KwdFr :
- MESH :
- imagerie diagnostique : Lymphoedème, Syndrome de Turner.
- Adolescent, Adulte, Agrégat d'albumine marquée au technétium (99mTc), Enfant, Enfant d'âge préscolaire, Femelle, Humains, Mâle, Radiopharmaceutiques, Scintigraphie.
English descriptors
- KwdEn :
- MESH :
- chemical : Radiopharmaceuticals, Technetium Tc 99m Aggregated Albumin.
- diagnostic imaging : Lymphedema, Turner Syndrome.
- Adolescent, Adult, Child, Child, Preschool, Female, Humans, Male, Radionuclide Imaging.
Abstract
Lymphedema can be present in patients affected by Turner syndrome (TS) with the dorsum of the hands and feet most commonly affected. This lymphedema results from underdevelopment of the lymphatic system before birth, and it usually decreases during childhood. The aim of our study was to evaluate the role of lymphoscintigraphy as a diagnostic tool in patients with TS to assess possible impairments in the lymphatic system. Eighteen patients with TS were karyotyped to confirm diagnosis and were evaluated by lymphoscintigraphy. Lymphatic dysfunction was demonstrated in 15/18 patients. Lymphoscintigraphic studies showed: 1) lymphatic channels, 2) collateral lymphatic channels, 3) interrupted lymphatic structures, and 4) lymph nodes of the deep lymphatic system. Our data demonstrate that lymphoscintigraphy should be mandatory not only in patients affected by Turner syndrome with signs of lymphatic dysplasia but also in those with minimal or absent signs of lymphatic impairment in order to obtain a very early diagnosis and to provide substantial information for possible medical or surgical treatment.
PubMed: 19927901
Affiliations:
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Boccardo</name></author><author><name sortKey="Campisi, C" sort="Campisi, C" uniqKey="Campisi C" first="C" last="Campisi">C. Campisi</name></author><author><name sortKey="Villa, G" sort="Villa, G" uniqKey="Villa G" first="G" last="Villa">G. Villa</name></author><author><name sortKey="Taddei, G" sort="Taddei, G" uniqKey="Taddei G" first="G" last="Taddei">G. Taddei</name></author><author><name sortKey="Traggiai, C" sort="Traggiai, C" uniqKey="Traggiai C" first="C" last="Traggiai">C. Traggiai</name></author><author><name sortKey="Amisano, A" sort="Amisano, A" uniqKey="Amisano A" first="A" last="Amisano">A. Amisano</name></author><author><name sortKey="Amisamo, A" sort="Amisamo, A" uniqKey="Amisamo A" first="A" last="Amisamo">A. 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Bellini</name><affiliation wicri:level="1"><nlm:affiliation>Neuromuscular Diseases Unit, Department of Pediatrics, University of Genoa, Genoa, Italy. carlobellini@ospedale-gaslini.ge.it</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Neuromuscular Diseases Unit, Department of Pediatrics, University of Genoa, Genoa</wicri:regionArea><wicri:noRegion>Genoa</wicri:noRegion></affiliation></author><author><name sortKey="Di Battista, E" sort="Di Battista, E" uniqKey="Di Battista E" first="E" last="Di Battista">E. Di Battista</name></author><author><name sortKey="Boccardo, F" sort="Boccardo, F" uniqKey="Boccardo F" first="F" last="Boccardo">F. Boccardo</name></author><author><name sortKey="Campisi, C" sort="Campisi, C" uniqKey="Campisi C" first="C" last="Campisi">C. Campisi</name></author><author><name sortKey="Villa, G" sort="Villa, G" uniqKey="Villa G" first="G" last="Villa">G. Villa</name></author><author><name sortKey="Taddei, G" sort="Taddei, G" uniqKey="Taddei G" first="G" last="Taddei">G. Taddei</name></author><author><name sortKey="Traggiai, C" sort="Traggiai, C" uniqKey="Traggiai C" first="C" last="Traggiai">C. Traggiai</name></author><author><name sortKey="Amisano, A" sort="Amisano, A" uniqKey="Amisano A" first="A" last="Amisano">A. Amisano</name></author><author><name sortKey="Amisamo, A" sort="Amisamo, A" uniqKey="Amisamo A" first="A" last="Amisamo">A. Amisamo</name></author><author><name sortKey="Perucchin, P Polo" sort="Perucchin, P Polo" uniqKey="Perucchin P" first="P Polo" last="Perucchin">P Polo Perucchin</name></author><author><name sortKey="Benfenati, C S" sort="Benfenati, C S" uniqKey="Benfenati C" first="C S" last="Benfenati">C S Benfenati</name></author><author><name sortKey="Bonioli, E" sort="Bonioli, E" uniqKey="Bonioli E" first="E" last="Bonioli">E. Bonioli</name></author><author><name sortKey="Lorini, R" sort="Lorini, R" uniqKey="Lorini R" first="R" last="Lorini">R. Lorini</name></author></analytic><series><title level="j">Lymphology</title><idno type="ISSN">0024-7766</idno><imprint><date when="2009" type="published">2009</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Child</term><term>Child, Preschool</term><term>Female</term><term>Humans</term><term>Lymphedema (diagnostic imaging)</term><term>Male</term><term>Radionuclide Imaging</term><term>Radiopharmaceuticals</term><term>Technetium Tc 99m Aggregated Albumin</term><term>Turner Syndrome (diagnostic imaging)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adolescent</term><term>Adulte</term><term>Agrégat d'albumine marquée au technétium (99mTc)</term><term>Enfant</term><term>Enfant d'âge préscolaire</term><term>Femelle</term><term>Humains</term><term>Lymphoedème (imagerie diagnostique)</term><term>Mâle</term><term>Radiopharmaceutiques</term><term>Scintigraphie</term><term>Syndrome de Turner (imagerie diagnostique)</term></keywords><keywords scheme="MESH" type="chemical" xml:lang="en"><term>Radiopharmaceuticals</term><term>Technetium Tc 99m Aggregated Albumin</term></keywords><keywords scheme="MESH" qualifier="diagnostic imaging" xml:lang="en"><term>Lymphedema</term><term>Turner Syndrome</term></keywords><keywords scheme="MESH" qualifier="imagerie diagnostique" xml:lang="fr"><term>Lymphoedème</term><term>Syndrome de Turner</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Child</term><term>Child, Preschool</term><term>Female</term><term>Humans</term><term>Male</term><term>Radionuclide Imaging</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adolescent</term><term>Adulte</term><term>Agrégat d'albumine marquée au technétium (99mTc)</term><term>Enfant</term><term>Enfant d'âge préscolaire</term><term>Femelle</term><term>Humains</term><term>Mâle</term><term>Radiopharmaceutiques</term><term>Scintigraphie</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Lymphedema can be present in patients affected by Turner syndrome (TS) with the dorsum of the hands and feet most commonly affected. This lymphedema results from underdevelopment of the lymphatic system before birth, and it usually decreases during childhood. The aim of our study was to evaluate the role of lymphoscintigraphy as a diagnostic tool in patients with TS to assess possible impairments in the lymphatic system. Eighteen patients with TS were karyotyped to confirm diagnosis and were evaluated by lymphoscintigraphy. Lymphatic dysfunction was demonstrated in 15/18 patients. Lymphoscintigraphic studies showed: 1) lymphatic channels, 2) collateral lymphatic channels, 3) interrupted lymphatic structures, and 4) lymph nodes of the deep lymphatic system. Our data demonstrate that lymphoscintigraphy should be mandatory not only in patients affected by Turner syndrome with signs of lymphatic dysplasia but also in those with minimal or absent signs of lymphatic impairment in order to obtain a very early diagnosis and to provide substantial information for possible medical or surgical treatment.</div></front></TEI><affiliations><list><country><li>Italie</li></country></list><tree><noCountry><name sortKey="Amisamo, A" sort="Amisamo, A" uniqKey="Amisamo A" first="A" last="Amisamo">A. Amisamo</name><name sortKey="Amisano, A" sort="Amisano, A" uniqKey="Amisano A" first="A" last="Amisano">A. Amisano</name><name sortKey="Benfenati, C S" sort="Benfenati, C S" uniqKey="Benfenati C" first="C S" last="Benfenati">C S Benfenati</name><name sortKey="Boccardo, F" sort="Boccardo, F" uniqKey="Boccardo F" first="F" last="Boccardo">F. Boccardo</name><name sortKey="Bonioli, E" sort="Bonioli, E" uniqKey="Bonioli E" first="E" last="Bonioli">E. Bonioli</name><name sortKey="Campisi, C" sort="Campisi, C" uniqKey="Campisi C" first="C" last="Campisi">C. Campisi</name><name sortKey="Di Battista, E" sort="Di Battista, E" uniqKey="Di Battista E" first="E" last="Di Battista">E. Di Battista</name><name sortKey="Lorini, R" sort="Lorini, R" uniqKey="Lorini R" first="R" last="Lorini">R. Lorini</name><name sortKey="Perucchin, P Polo" sort="Perucchin, P Polo" uniqKey="Perucchin P" first="P Polo" last="Perucchin">P Polo Perucchin</name><name sortKey="Taddei, G" sort="Taddei, G" uniqKey="Taddei G" first="G" last="Taddei">G. Taddei</name><name sortKey="Traggiai, C" sort="Traggiai, C" uniqKey="Traggiai C" first="C" last="Traggiai">C. Traggiai</name><name sortKey="Villa, G" sort="Villa, G" uniqKey="Villa G" first="G" last="Villa">G. Villa</name></noCountry><country name="Italie"><noRegion><name sortKey="Bellini, C" sort="Bellini, C" uniqKey="Bellini C" first="C" last="Bellini">C. 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