Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia
Identifieur interne : 005339 ( Ncbi/Merge ); précédent : 005338; suivant : 005340Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia
Auteurs : Siham Al Sinani ; Yusria Al Rawahi ; Hamed AbdoonSource :
- World Journal of Gastroenterology : WJG [ 1007-9327 ] ; 2012.
Descripteurs français
- KwdFr :
- Entéropathie exsudative (), Entéropathie exsudative (étiologie), Humains, Hypoalbuminémie (), Hypoalbuminémie (étiologie), Hérédité, Lymphangiectasie intestinale (diagnostic), Lymphangiectasie intestinale (génétique), Lymphangiectasie intestinale (traitement médicamenteux), Lymphoedème (diagnostic), Lymphoedème (génétique), Lymphoedème (traitement médicamenteux), Maladies de l'appareil génital mâle (diagnostic), Maladies de l'appareil génital mâle (génétique), Maladies de l'appareil génital mâle (traitement médicamenteux), Malformations crâniofaciales (diagnostic), Malformations crâniofaciales (génétique), Malformations crâniofaciales (traitement médicamenteux), Mâle, Nouveau-né, Octréotide (usage thérapeutique), Pedigree, Phénotype, Prédisposition génétique à une maladie, Résultat thérapeutique.
- MESH :
- diagnostic : Lymphangiectasie intestinale, Lymphoedème, Maladies de l'appareil génital mâle, Malformations crâniofaciales.
- génétique : Lymphangiectasie intestinale, Lymphoedème, Maladies de l'appareil génital mâle, Malformations crâniofaciales.
- traitement médicamenteux : Lymphangiectasie intestinale, Lymphoedème, Maladies de l'appareil génital mâle, Malformations crâniofaciales.
- usage thérapeutique : Octréotide.
- étiologie : Entéropathie exsudative, Hypoalbuminémie.
- Entéropathie exsudative, Humains, Hypoalbuminémie, Hérédité, Mâle, Nouveau-né, Pedigree, Phénotype, Prédisposition génétique à une maladie, Résultat thérapeutique.
English descriptors
- KwdEn :
- Craniofacial Abnormalities (diagnosis), Craniofacial Abnormalities (drug therapy), Craniofacial Abnormalities (genetics), Genetic Predisposition to Disease, Genital Diseases, Male (diagnosis), Genital Diseases, Male (drug therapy), Genital Diseases, Male (genetics), Heredity, Humans, Hypoalbuminemia (etiology), Hypoalbuminemia (prevention & control), Infant, Newborn, Lymphangiectasis, Intestinal (diagnosis), Lymphangiectasis, Intestinal (drug therapy), Lymphangiectasis, Intestinal (genetics), Lymphedema (diagnosis), Lymphedema (drug therapy), Lymphedema (genetics), Male, Octreotide (therapeutic use), Pedigree, Phenotype, Protein-Losing Enteropathies (etiology), Protein-Losing Enteropathies (prevention & control), Treatment Outcome.
- MESH :
- chemical , therapeutic use : Octreotide.
- diagnosis : Craniofacial Abnormalities, Genital Diseases, Male, Lymphangiectasis, Intestinal, Lymphedema.
- drug therapy : Craniofacial Abnormalities, Genital Diseases, Male, Lymphangiectasis, Intestinal, Lymphedema.
- etiology : Hypoalbuminemia, Protein-Losing Enteropathies.
- genetics : Craniofacial Abnormalities, Genital Diseases, Male, Lymphangiectasis, Intestinal, Lymphedema.
- prevention & control : Hypoalbuminemia, Protein-Losing Enteropathies.
- Genetic Predisposition to Disease, Heredity, Humans, Infant, Newborn, Male, Pedigree, Phenotype, Treatment Outcome.
Abstract
A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.
Url:
DOI: 10.3748/wjg.v18.i43.6333
PubMed: 23180957
PubMed Central: 3501785
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PMC:3501785Le document en format XML
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Disease</term><term>Heredity</term><term>Humans</term><term>Infant, Newborn</term><term>Male</term><term>Pedigree</term><term>Phenotype</term><term>Treatment Outcome</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Entéropathie exsudative</term><term>Humains</term><term>Hypoalbuminémie</term><term>Hérédité</term><term>Mâle</term><term>Nouveau-né</term><term>Pedigree</term><term>Phénotype</term><term>Prédisposition génétique à une maladie</term><term>Résultat thérapeutique</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.</p></div></front></TEI><double pmid="23180957"><pmc><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia</title><author><name sortKey="Al Sinani, Siham" sort="Al Sinani, Siham" uniqKey="Al Sinani S" first="Siham" last="Al Sinani">Siham Al Sinani</name></author><author><name sortKey="Rawahi, Yusria Al" sort="Rawahi, Yusria Al" uniqKey="Rawahi Y" first="Yusria Al" last="Rawahi">Yusria Al Rawahi</name></author><author><name sortKey="Abdoon, Hamed" sort="Abdoon, Hamed" uniqKey="Abdoon H" first="Hamed" last="Abdoon">Hamed Abdoon</name></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">23180957</idno><idno type="pmc">3501785</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3501785</idno><idno type="RBID">PMC:3501785</idno><idno type="doi">10.3748/wjg.v18.i43.6333</idno><date when="2012">2012</date><idno type="wicri:Area/Pmc/Corpus">003221</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">003221</idno><idno type="wicri:Area/Pmc/Curation">003220</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">003220</idno><idno type="wicri:Area/Pmc/Checkpoint">002851</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">002851</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia</title><author><name sortKey="Al Sinani, Siham" sort="Al Sinani, Siham" uniqKey="Al Sinani S" first="Siham" last="Al Sinani">Siham Al Sinani</name></author><author><name sortKey="Rawahi, Yusria Al" sort="Rawahi, Yusria Al" uniqKey="Rawahi Y" first="Yusria Al" last="Rawahi">Yusria Al Rawahi</name></author><author><name sortKey="Abdoon, Hamed" sort="Abdoon, Hamed" uniqKey="Abdoon H" first="Hamed" last="Abdoon">Hamed Abdoon</name></author></analytic><series><title level="j">World Journal of Gastroenterology : WJG</title><idno type="ISSN">1007-9327</idno><imprint><date when="2012">2012</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.</p></div></front></TEI></pmc><pubmed><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.</title><author><name sortKey="Al Sinani, Siham" sort="Al Sinani, Siham" uniqKey="Al Sinani S" first="Siham" last="Al Sinani">Siham Al Sinani</name><affiliation><nlm:affiliation>Child Health Department, Sultan Qaboos University Hospital, Muscat 123, Sultanate of Oman. siham_ss@hotmail.com</nlm:affiliation><wicri:noCountry code="subField">Sultanate of Oman</wicri:noCountry></affiliation></author><author><name sortKey="Rawahi, Yusria Al" sort="Rawahi, Yusria Al" uniqKey="Rawahi Y" first="Yusria Al" last="Rawahi">Yusria Al Rawahi</name></author><author><name sortKey="Abdoon, Hamed" sort="Abdoon, Hamed" uniqKey="Abdoon H" first="Hamed" last="Abdoon">Hamed Abdoon</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2012">2012</date><idno type="RBID">pubmed:23180957</idno><idno type="pmid">23180957</idno><idno type="doi">10.3748/wjg.v18.i43.6333</idno><idno type="wicri:Area/PubMed/Corpus">001E54</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">001E54</idno><idno type="wicri:Area/PubMed/Curation">001E54</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">001E54</idno><idno type="wicri:Area/PubMed/Checkpoint">001E54</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">001E54</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.</title><author><name sortKey="Al Sinani, Siham" sort="Al Sinani, Siham" uniqKey="Al Sinani S" first="Siham" last="Al Sinani">Siham Al Sinani</name><affiliation><nlm:affiliation>Child Health Department, Sultan Qaboos University Hospital, Muscat 123, Sultanate of Oman. siham_ss@hotmail.com</nlm:affiliation><wicri:noCountry code="subField">Sultanate of Oman</wicri:noCountry></affiliation></author><author><name sortKey="Rawahi, Yusria Al" sort="Rawahi, Yusria Al" uniqKey="Rawahi Y" first="Yusria Al" last="Rawahi">Yusria Al Rawahi</name></author><author><name sortKey="Abdoon, Hamed" sort="Abdoon, Hamed" uniqKey="Abdoon H" first="Hamed" last="Abdoon">Hamed Abdoon</name></author></analytic><series><title level="j">World journal of gastroenterology</title><idno type="eISSN">2219-2840</idno><imprint><date when="2012" type="published">2012</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Craniofacial Abnormalities (diagnosis)</term><term>Craniofacial Abnormalities (drug therapy)</term><term>Craniofacial Abnormalities (genetics)</term><term>Genetic Predisposition to Disease</term><term>Genital Diseases, Male (diagnosis)</term><term>Genital Diseases, Male (drug therapy)</term><term>Genital Diseases, Male (genetics)</term><term>Heredity</term><term>Humans</term><term>Hypoalbuminemia (etiology)</term><term>Hypoalbuminemia (prevention & control)</term><term>Infant, Newborn</term><term>Lymphangiectasis, Intestinal (diagnosis)</term><term>Lymphangiectasis, Intestinal (drug therapy)</term><term>Lymphangiectasis, Intestinal (genetics)</term><term>Lymphedema (diagnosis)</term><term>Lymphedema (drug therapy)</term><term>Lymphedema (genetics)</term><term>Male</term><term>Octreotide (therapeutic use)</term><term>Pedigree</term><term>Phenotype</term><term>Protein-Losing Enteropathies (etiology)</term><term>Protein-Losing Enteropathies (prevention & control)</term><term>Treatment Outcome</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Entéropathie exsudative ()</term><term>Entéropathie exsudative (étiologie)</term><term>Humains</term><term>Hypoalbuminémie ()</term><term>Hypoalbuminémie (étiologie)</term><term>Hérédité</term><term>Lymphangiectasie intestinale (diagnostic)</term><term>Lymphangiectasie intestinale (génétique)</term><term>Lymphangiectasie intestinale (traitement médicamenteux)</term><term>Lymphoedème (diagnostic)</term><term>Lymphoedème (génétique)</term><term>Lymphoedème (traitement médicamenteux)</term><term>Maladies de l'appareil génital mâle (diagnostic)</term><term>Maladies de l'appareil génital mâle (génétique)</term><term>Maladies de l'appareil génital mâle (traitement médicamenteux)</term><term>Malformations crâniofaciales (diagnostic)</term><term>Malformations crâniofaciales (génétique)</term><term>Malformations crâniofaciales (traitement médicamenteux)</term><term>Mâle</term><term>Nouveau-né</term><term>Octréotide (usage thérapeutique)</term><term>Pedigree</term><term>Phénotype</term><term>Prédisposition génétique à une maladie</term><term>Résultat thérapeutique</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Octreotide</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Craniofacial Abnormalities</term><term>Genital Diseases, Male</term><term>Lymphangiectasis, Intestinal</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Lymphangiectasie intestinale</term><term>Lymphoedème</term><term>Maladies de l'appareil génital mâle</term><term>Malformations crâniofaciales</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Craniofacial Abnormalities</term><term>Genital Diseases, Male</term><term>Lymphangiectasis, Intestinal</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Hypoalbuminemia</term><term>Protein-Losing Enteropathies</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Craniofacial Abnormalities</term><term>Genital Diseases, Male</term><term>Lymphangiectasis, Intestinal</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Lymphangiectasie intestinale</term><term>Lymphoedème</term><term>Maladies de l'appareil génital mâle</term><term>Malformations crâniofaciales</term></keywords><keywords scheme="MESH" qualifier="prevention & control" xml:lang="en"><term>Hypoalbuminemia</term><term>Protein-Losing Enteropathies</term></keywords><keywords scheme="MESH" qualifier="traitement médicamenteux" xml:lang="fr"><term>Lymphangiectasie intestinale</term><term>Lymphoedème</term><term>Maladies de l'appareil génital mâle</term><term>Malformations crâniofaciales</term></keywords><keywords scheme="MESH" qualifier="usage thérapeutique" xml:lang="fr"><term>Octréotide</term></keywords><keywords scheme="MESH" qualifier="étiologie" xml:lang="fr"><term>Entéropathie exsudative</term><term>Hypoalbuminémie</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Genetic Predisposition to Disease</term><term>Heredity</term><term>Humans</term><term>Infant, Newborn</term><term>Male</term><term>Pedigree</term><term>Phenotype</term><term>Treatment Outcome</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Entéropathie exsudative</term><term>Humains</term><term>Hypoalbuminémie</term><term>Hérédité</term><term>Mâle</term><term>Nouveau-né</term><term>Pedigree</term><term>Phénotype</term><term>Prédisposition génétique à une maladie</term><term>Résultat thérapeutique</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.</div></front></TEI></pubmed></double></record>Pour manipuler ce document sous Unix (Dilib)
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