Digital clubbing in primary intestinal lymphangiectasia: a case report.
Identifieur interne : 005950 ( Main/Exploration ); précédent : 005949; suivant : 005951Digital clubbing in primary intestinal lymphangiectasia: a case report.
Auteurs : Christian J. Wiedermann [Italie] ; Michael Kob ; Stefano Benvenuti ; Rodolfo Carella ; Lucio Lucchin ; Lucia Piazzi ; Fausto Chilovi ; Guido MazzoleniSource :
- Wiener medizinische Wochenschrift (1946) [ 1563-258X ] ; 2010.
Descripteurs français
- KwdFr :
- Adulte d'âge moyen, Aliment formulé, Association thérapeutique, Biopsie, Entéropathie exsudative (anatomopathologie), Entéropathie exsudative (diagnostic), Entéropathie exsudative (diétothérapie), Entéroscopie double ballon, Humains, Intestin grêle (anatomopathologie), Lymphangiectasie intestinale (anatomopathologie), Lymphangiectasie intestinale (diagnostic), Lymphangiectasie intestinale (diétothérapie), Lymphoedème (anatomopathologie), Lymphoedème (diagnostic), Lymphoedème (diétothérapie), Mâle, Nutrition entérale, Ostéoarthropathie hypertrophiante secondaire (diétothérapie), Ostéoarthropathie hypertrophiante secondaire (étiologie), Régime pauvre en graisses.
- MESH :
- anatomopathologie : Entéropathie exsudative, Intestin grêle, Lymphangiectasie intestinale, Lymphoedème.
- diagnostic : Entéropathie exsudative, Lymphangiectasie intestinale, Lymphoedème.
- diétothérapie : Entéropathie exsudative, Lymphangiectasie intestinale, Lymphoedème, Ostéoarthropathie hypertrophiante secondaire.
- étiologie : Ostéoarthropathie hypertrophiante secondaire.
- Adulte d'âge moyen, Aliment formulé, Association thérapeutique, Biopsie, Entéroscopie double ballon, Humains, Mâle, Nutrition entérale, Régime pauvre en graisses.
English descriptors
- KwdEn :
- Biopsy, Combined Modality Therapy, Diet, Fat-Restricted, Double-Balloon Enteroscopy, Enteral Nutrition, Food, Formulated, Humans, Intestine, Small (pathology), Lymphangiectasis, Intestinal (diagnosis), Lymphangiectasis, Intestinal (diet therapy), Lymphangiectasis, Intestinal (pathology), Lymphedema (diagnosis), Lymphedema (diet therapy), Lymphedema (pathology), Male, Middle Aged, Osteoarthropathy, Secondary Hypertrophic (diet therapy), Osteoarthropathy, Secondary Hypertrophic (etiology), Protein-Losing Enteropathies (diagnosis), Protein-Losing Enteropathies (diet therapy), Protein-Losing Enteropathies (pathology).
- MESH :
- diagnosis : Lymphangiectasis, Intestinal, Lymphedema, Protein-Losing Enteropathies.
- diet therapy : Lymphangiectasis, Intestinal, Lymphedema, Osteoarthropathy, Secondary Hypertrophic, Protein-Losing Enteropathies.
- etiology : Osteoarthropathy, Secondary Hypertrophic.
- pathology : Intestine, Small, Lymphangiectasis, Intestinal, Lymphedema, Protein-Losing Enteropathies.
- Biopsy, Combined Modality Therapy, Diet, Fat-Restricted, Double-Balloon Enteroscopy, Enteral Nutrition, Food, Formulated, Humans, Male, Middle Aged.
Abstract
Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. The symptoms usually start in early infancy. We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea. Malabsorption with inability to gain weight, and finally weight loss and formation of leg edema were associated with protein-losing enteropathy. A low-fat diet associated with medium-chain triglyceride supplementation was clinically effective as medical management in reducing diarrhea and leg edema, and promoting weight gain. Double-balloon enteroscopy and small bowel biopsy histopathology confirmed dilated intestinal lacteals. Digital clubbing associated with primary intestinal lymphangiectasia which may causally be related to chronic platelet excess has not been reported before.
DOI: 10.1007/s10354-010-0815-0
PubMed: 20812055
Affiliations:
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Wiedermann</name><affiliation wicri:level="1"><nlm:affiliation>Division of Internal Medicine, Central Hospital, Bolzano, Italy. christian.wiedermann@asbz.it</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Division of Internal Medicine, Central Hospital, Bolzano</wicri:regionArea><wicri:noRegion>Bolzano</wicri:noRegion></affiliation></author><author><name sortKey="Kob, Michael" sort="Kob, Michael" uniqKey="Kob M" first="Michael" last="Kob">Michael Kob</name></author><author><name sortKey="Benvenuti, Stefano" sort="Benvenuti, Stefano" uniqKey="Benvenuti S" first="Stefano" last="Benvenuti">Stefano Benvenuti</name></author><author><name sortKey="Carella, Rodolfo" sort="Carella, Rodolfo" uniqKey="Carella R" first="Rodolfo" last="Carella">Rodolfo Carella</name></author><author><name sortKey="Lucchin, Lucio" sort="Lucchin, Lucio" uniqKey="Lucchin L" first="Lucio" last="Lucchin">Lucio Lucchin</name></author><author><name sortKey="Piazzi, Lucia" sort="Piazzi, Lucia" uniqKey="Piazzi L" first="Lucia" last="Piazzi">Lucia Piazzi</name></author><author><name sortKey="Chilovi, Fausto" sort="Chilovi, Fausto" uniqKey="Chilovi F" first="Fausto" last="Chilovi">Fausto Chilovi</name></author><author><name sortKey="Mazzoleni, Guido" sort="Mazzoleni, Guido" uniqKey="Mazzoleni G" first="Guido" last="Mazzoleni">Guido Mazzoleni</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2010">2010</date><idno type="RBID">pubmed:20812055</idno><idno type="pmid">20812055</idno><idno type="doi">10.1007/s10354-010-0815-0</idno><idno type="wicri:Area/PubMed/Corpus">002935</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">002935</idno><idno type="wicri:Area/PubMed/Curation">002935</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">002935</idno><idno type="wicri:Area/PubMed/Checkpoint">002935</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">002935</idno><idno type="wicri:Area/Ncbi/Merge">003D73</idno><idno type="wicri:Area/Ncbi/Curation">003D73</idno><idno type="wicri:Area/Ncbi/Checkpoint">003D73</idno><idno type="wicri:Area/Main/Merge">005A02</idno><idno type="wicri:Area/Main/Curation">005950</idno><idno type="wicri:Area/Main/Exploration">005950</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Digital clubbing in primary intestinal lymphangiectasia: a case report.</title><author><name sortKey="Wiedermann, Christian J" sort="Wiedermann, Christian J" uniqKey="Wiedermann C" first="Christian J" last="Wiedermann">Christian J. Wiedermann</name><affiliation wicri:level="1"><nlm:affiliation>Division of Internal Medicine, Central Hospital, Bolzano, Italy. christian.wiedermann@asbz.it</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Division of Internal Medicine, Central Hospital, Bolzano</wicri:regionArea><wicri:noRegion>Bolzano</wicri:noRegion></affiliation></author><author><name sortKey="Kob, Michael" sort="Kob, Michael" uniqKey="Kob M" first="Michael" last="Kob">Michael Kob</name></author><author><name sortKey="Benvenuti, Stefano" sort="Benvenuti, Stefano" uniqKey="Benvenuti S" first="Stefano" last="Benvenuti">Stefano Benvenuti</name></author><author><name sortKey="Carella, Rodolfo" sort="Carella, Rodolfo" uniqKey="Carella R" first="Rodolfo" last="Carella">Rodolfo Carella</name></author><author><name sortKey="Lucchin, Lucio" sort="Lucchin, Lucio" uniqKey="Lucchin L" first="Lucio" last="Lucchin">Lucio Lucchin</name></author><author><name sortKey="Piazzi, Lucia" sort="Piazzi, Lucia" uniqKey="Piazzi L" first="Lucia" last="Piazzi">Lucia Piazzi</name></author><author><name sortKey="Chilovi, Fausto" sort="Chilovi, Fausto" uniqKey="Chilovi F" first="Fausto" last="Chilovi">Fausto Chilovi</name></author><author><name sortKey="Mazzoleni, Guido" sort="Mazzoleni, Guido" uniqKey="Mazzoleni G" first="Guido" last="Mazzoleni">Guido Mazzoleni</name></author></analytic><series><title level="j">Wiener medizinische Wochenschrift (1946)</title><idno type="eISSN">1563-258X</idno><imprint><date when="2010" type="published">2010</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Biopsy</term><term>Combined Modality Therapy</term><term>Diet, Fat-Restricted</term><term>Double-Balloon Enteroscopy</term><term>Enteral Nutrition</term><term>Food, Formulated</term><term>Humans</term><term>Intestine, Small (pathology)</term><term>Lymphangiectasis, Intestinal (diagnosis)</term><term>Lymphangiectasis, Intestinal (diet therapy)</term><term>Lymphangiectasis, Intestinal (pathology)</term><term>Lymphedema (diagnosis)</term><term>Lymphedema (diet therapy)</term><term>Lymphedema (pathology)</term><term>Male</term><term>Middle Aged</term><term>Osteoarthropathy, Secondary Hypertrophic (diet therapy)</term><term>Osteoarthropathy, Secondary Hypertrophic (etiology)</term><term>Protein-Losing Enteropathies (diagnosis)</term><term>Protein-Losing Enteropathies (diet therapy)</term><term>Protein-Losing Enteropathies (pathology)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte d'âge moyen</term><term>Aliment formulé</term><term>Association thérapeutique</term><term>Biopsie</term><term>Entéropathie exsudative (anatomopathologie)</term><term>Entéropathie exsudative (diagnostic)</term><term>Entéropathie exsudative (diétothérapie)</term><term>Entéroscopie double ballon</term><term>Humains</term><term>Intestin grêle (anatomopathologie)</term><term>Lymphangiectasie intestinale (anatomopathologie)</term><term>Lymphangiectasie intestinale (diagnostic)</term><term>Lymphangiectasie intestinale (diétothérapie)</term><term>Lymphoedème (anatomopathologie)</term><term>Lymphoedème (diagnostic)</term><term>Lymphoedème (diétothérapie)</term><term>Mâle</term><term>Nutrition entérale</term><term>Ostéoarthropathie hypertrophiante secondaire (diétothérapie)</term><term>Ostéoarthropathie hypertrophiante secondaire (étiologie)</term><term>Régime pauvre en graisses</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Entéropathie exsudative</term><term>Intestin grêle</term><term>Lymphangiectasie intestinale</term><term>Lymphoedème</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Lymphangiectasis, Intestinal</term><term>Lymphedema</term><term>Protein-Losing Enteropathies</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Entéropathie exsudative</term><term>Lymphangiectasie intestinale</term><term>Lymphoedème</term></keywords><keywords scheme="MESH" qualifier="diet therapy" xml:lang="en"><term>Lymphangiectasis, Intestinal</term><term>Lymphedema</term><term>Osteoarthropathy, Secondary Hypertrophic</term><term>Protein-Losing Enteropathies</term></keywords><keywords scheme="MESH" qualifier="diétothérapie" xml:lang="fr"><term>Entéropathie exsudative</term><term>Lymphangiectasie intestinale</term><term>Lymphoedème</term><term>Ostéoarthropathie hypertrophiante secondaire</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Osteoarthropathy, Secondary Hypertrophic</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Intestine, Small</term><term>Lymphangiectasis, Intestinal</term><term>Lymphedema</term><term>Protein-Losing Enteropathies</term></keywords><keywords scheme="MESH" qualifier="étiologie" xml:lang="fr"><term>Ostéoarthropathie hypertrophiante secondaire</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Biopsy</term><term>Combined Modality Therapy</term><term>Diet, Fat-Restricted</term><term>Double-Balloon Enteroscopy</term><term>Enteral Nutrition</term><term>Food, Formulated</term><term>Humans</term><term>Male</term><term>Middle Aged</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte d'âge moyen</term><term>Aliment formulé</term><term>Association thérapeutique</term><term>Biopsie</term><term>Entéroscopie double ballon</term><term>Humains</term><term>Mâle</term><term>Nutrition entérale</term><term>Régime pauvre en graisses</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. The symptoms usually start in early infancy. We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea. Malabsorption with inability to gain weight, and finally weight loss and formation of leg edema were associated with protein-losing enteropathy. A low-fat diet associated with medium-chain triglyceride supplementation was clinically effective as medical management in reducing diarrhea and leg edema, and promoting weight gain. Double-balloon enteroscopy and small bowel biopsy histopathology confirmed dilated intestinal lacteals. Digital clubbing associated with primary intestinal lymphangiectasia which may causally be related to chronic platelet excess has not been reported before.</div></front></TEI><affiliations><list><country><li>Italie</li></country></list><tree><noCountry><name sortKey="Benvenuti, Stefano" sort="Benvenuti, Stefano" uniqKey="Benvenuti S" first="Stefano" last="Benvenuti">Stefano Benvenuti</name><name sortKey="Carella, Rodolfo" sort="Carella, Rodolfo" uniqKey="Carella R" first="Rodolfo" last="Carella">Rodolfo Carella</name><name sortKey="Chilovi, Fausto" sort="Chilovi, Fausto" uniqKey="Chilovi F" first="Fausto" last="Chilovi">Fausto Chilovi</name><name sortKey="Kob, Michael" sort="Kob, Michael" uniqKey="Kob M" first="Michael" last="Kob">Michael Kob</name><name sortKey="Lucchin, Lucio" sort="Lucchin, Lucio" uniqKey="Lucchin L" first="Lucio" last="Lucchin">Lucio Lucchin</name><name sortKey="Mazzoleni, Guido" sort="Mazzoleni, Guido" uniqKey="Mazzoleni G" first="Guido" last="Mazzoleni">Guido Mazzoleni</name><name sortKey="Piazzi, Lucia" sort="Piazzi, Lucia" uniqKey="Piazzi L" first="Lucia" last="Piazzi">Lucia Piazzi</name></noCountry><country name="Italie"><noRegion><name sortKey="Wiedermann, Christian J" sort="Wiedermann, Christian J" uniqKey="Wiedermann C" first="Christian J" last="Wiedermann">Christian J. 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