Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy.
Identifieur interne : 001092 ( Main/Exploration ); précédent : 001091; suivant : 001093Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy.
Auteurs : Michio Ozeki [Japon] ; Tomohiro Hori ; Kaori Kanda ; Norio Kawamoto ; Takashi Ibuka ; Tatsuhiko Miyazaki [Japon] ; Toshiyuki FukaoSource :
- Pediatrics [ 1098-4275 ] ; 2016.
Descripteurs français
- KwdFr :
- Enfant, Entéropathie exsudative (traitement médicamenteux), Entéropathie exsudative (étiologie), Humains, Immunosuppresseurs (administration et posologie), Lymphangiectasie intestinale (), Lymphangiectasie intestinale (diétothérapie), Lymphangiectasie intestinale (traitement médicamenteux), Lymphoedème (), Lymphoedème (diétothérapie), Lymphoedème (traitement médicamenteux), Mâle, Relation dose-effet des médicaments, Régime pauvre en graisses, Études de suivi, Évérolimus (administration et posologie).
- MESH :
- administration et posologie : Immunosuppresseurs, Évérolimus.
- diétothérapie : Lymphangiectasie intestinale, Lymphoedème.
- traitement médicamenteux : Entéropathie exsudative, Lymphangiectasie intestinale, Lymphoedème.
- étiologie : Entéropathie exsudative.
- Enfant, Humains, Lymphangiectasie intestinale, Lymphoedème, Mâle, Relation dose-effet des médicaments, Régime pauvre en graisses, Études de suivi.
English descriptors
- KwdEn :
- Child, Diet, Fat-Restricted, Dose-Response Relationship, Drug, Everolimus (administration & dosage), Follow-Up Studies, Humans, Immunosuppressive Agents (administration & dosage), Lymphangiectasis, Intestinal (complications), Lymphangiectasis, Intestinal (diet therapy), Lymphangiectasis, Intestinal (drug therapy), Lymphedema (complications), Lymphedema (diet therapy), Lymphedema (drug therapy), Male, Protein-Losing Enteropathies (drug therapy), Protein-Losing Enteropathies (etiology).
- MESH :
- chemical , administration & dosage : Everolimus, Immunosuppressive Agents.
- complications : Lymphangiectasis, Intestinal, Lymphedema.
- diet therapy : Lymphangiectasis, Intestinal, Lymphedema.
- drug therapy : Lymphangiectasis, Intestinal, Lymphedema, Protein-Losing Enteropathies.
- etiology : Protein-Losing Enteropathies.
- Child, Diet, Fat-Restricted, Dose-Response Relationship, Drug, Follow-Up Studies, Humans, Male.
Abstract
Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is an exudative enteropathy resulting from morphologic abnormalities in the intestinal lymphatics. In this article, we describe a 12-year-old boy with PIL that led to protein-losing enteropathy characterized by diarrhea, hypoalbuminemia associated with edema (serum albumin level: 1.0 g/dL), and hypogammaglobulinemia (serum IgG level: 144 mg/dL). Severe hypoalbuminemia, electrolyte abnormalities, and tetany persisted despite a low-fat diet and propranolol. Everolimus (1.6 mg/m(2)/day) was added to his treatment as an antiangiogenic agent. With everolimus treatment, the patient's diarrhea resolved and replacement therapy for hypoproteinemia was less frequent. Hematologic and scintigraphy findings also improved (serum albumin level: 2.5 g/dL). There were no adverse reactions during the 12-month follow-up. To the best of our knowledge, this is the first report of everolimus use in a patient with PIL.
DOI: 10.1542/peds.2015-2562
PubMed: 26908672
Affiliations:
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Le document en format XML
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<term>Follow-Up Studies</term>
<term>Humans</term>
<term>Immunosuppressive Agents (administration & dosage)</term>
<term>Lymphangiectasis, Intestinal (complications)</term>
<term>Lymphangiectasis, Intestinal (diet therapy)</term>
<term>Lymphangiectasis, Intestinal (drug therapy)</term>
<term>Lymphedema (complications)</term>
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<term>Entéropathie exsudative (étiologie)</term>
<term>Humains</term>
<term>Immunosuppresseurs (administration et posologie)</term>
<term>Lymphangiectasie intestinale ()</term>
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<term>Relation dose-effet des médicaments</term>
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<term>Lymphedema</term>
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<front><div type="abstract" xml:lang="en">Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is an exudative enteropathy resulting from morphologic abnormalities in the intestinal lymphatics. In this article, we describe a 12-year-old boy with PIL that led to protein-losing enteropathy characterized by diarrhea, hypoalbuminemia associated with edema (serum albumin level: 1.0 g/dL), and hypogammaglobulinemia (serum IgG level: 144 mg/dL). Severe hypoalbuminemia, electrolyte abnormalities, and tetany persisted despite a low-fat diet and propranolol. Everolimus (1.6 mg/m(2)/day) was added to his treatment as an antiangiogenic agent. With everolimus treatment, the patient's diarrhea resolved and replacement therapy for hypoproteinemia was less frequent. Hematologic and scintigraphy findings also improved (serum albumin level: 2.5 g/dL). There were no adverse reactions during the 12-month follow-up. To the best of our knowledge, this is the first report of everolimus use in a patient with PIL.</div>
</front>
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<tree><noCountry><name sortKey="Fukao, Toshiyuki" sort="Fukao, Toshiyuki" uniqKey="Fukao T" first="Toshiyuki" last="Fukao">Toshiyuki Fukao</name>
<name sortKey="Hori, Tomohiro" sort="Hori, Tomohiro" uniqKey="Hori T" first="Tomohiro" last="Hori">Tomohiro Hori</name>
<name sortKey="Ibuka, Takashi" sort="Ibuka, Takashi" uniqKey="Ibuka T" first="Takashi" last="Ibuka">Takashi Ibuka</name>
<name sortKey="Kanda, Kaori" sort="Kanda, Kaori" uniqKey="Kanda K" first="Kaori" last="Kanda">Kaori Kanda</name>
<name sortKey="Kawamoto, Norio" sort="Kawamoto, Norio" uniqKey="Kawamoto N" first="Norio" last="Kawamoto">Norio Kawamoto</name>
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<name sortKey="Miyazaki, Tatsuhiko" sort="Miyazaki, Tatsuhiko" uniqKey="Miyazaki T" first="Tatsuhiko" last="Miyazaki">Tatsuhiko Miyazaki</name>
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