Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions
Identifieur interne : 008009 ( Ncbi/Curation ); précédent : 008008; suivant : 008010Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions
Auteurs : Xuefeng Wang ; Hong Jin ; Weilu WuSource :
- Medicine [ 0025-7974 ] ; 2016.
Descripteurs français
- KwdFr :
- Adolescent, Biopsie, Diagnostic différentiel, Endoscopie digestive (), Femelle, Humains, Hypoprotéinémie (physiopathologie), Hypoprotéinémie (étiologie), Jéjunum (anatomopathologie), Lymphangiectasie intestinale (), Lymphangiectasie intestinale (diagnostic), Lymphangiectasie intestinale (diétothérapie), Lymphangiectasie intestinale (physiopathologie), Membres (anatomopathologie), Oedème (diagnostic), Oedème (étiologie), Protéines alimentaires (administration et posologie), Régime pauvre en graisses (), Tomodensitométrie (), Triglycéride (administration et posologie), Épanchement pleural (diagnostic), Épanchement pleural (étiologie), Épanchement péricardique (diagnostic), Épanchement péricardique (étiologie).
- MESH :
- administration et posologie : Protéines alimentaires, Triglycéride.
- anatomopathologie : Jéjunum, Membres.
- diagnostic : Lymphangiectasie intestinale, Oedème, Épanchement pleural, Épanchement péricardique.
- diétothérapie : Lymphangiectasie intestinale.
- physiopathologie : Hypoprotéinémie, Lymphangiectasie intestinale.
- étiologie : Hypoprotéinémie, Oedème, Épanchement pleural, Épanchement péricardique.
- Adolescent, Biopsie, Diagnostic différentiel, Endoscopie digestive, Femelle, Humains, Lymphangiectasie intestinale, Régime pauvre en graisses, Tomodensitométrie.
English descriptors
- KwdEn :
- Adolescent, Biopsy, Diagnosis, Differential, Diet, Fat-Restricted (methods), Dietary Proteins (administration & dosage), Edema (diagnosis), Edema (etiology), Endoscopy, Digestive System (methods), Extremities (pathology), Female, Humans, Hypoproteinemia (etiology), Hypoproteinemia (physiopathology), Jejunum (pathology), Lymphangiectasis, Intestinal (complications), Lymphangiectasis, Intestinal (diagnosis), Lymphangiectasis, Intestinal (diet therapy), Lymphangiectasis, Intestinal (physiopathology), Pericardial Effusion (diagnosis), Pericardial Effusion (etiology), Pleural Effusion (diagnosis), Pleural Effusion (etiology), Tomography, X-Ray Computed (methods), Triglycerides (administration & dosage).
- MESH :
- chemical , administration & dosage : Dietary Proteins, Triglycerides.
- complications : Lymphangiectasis, Intestinal.
- diagnosis : Edema, Lymphangiectasis, Intestinal, Pericardial Effusion, Pleural Effusion.
- diet therapy : Lymphangiectasis, Intestinal.
- etiology : Edema, Hypoproteinemia, Pericardial Effusion, Pleural Effusion.
- methods : Diet, Fat-Restricted, Endoscopy, Digestive System, Tomography, X-Ray Computed.
- pathology : Extremities, Jejunum.
- physiopathology : Hypoproteinemia, Lymphangiectasis, Intestinal.
- Adolescent, Biopsy, Diagnosis, Differential, Female, Humans.
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder of unknown etiology characterized by diffuse or localized dilation and eventual rupture of the enteric lymphatic vessels in mucosa, submucosa, and/or subserosa. Lymph, rich in all kinds of proteins and lymphocytes, leaks into the gastrointestinal tract via the affected lymphatic vessels causing hypoproteinemia and lymphopenia. The main symptom is variable degrees of pitting edemas of bilateral lower limbs. But edemas of any other parts of body, and mild serous effusions may also occur sometimes. PIL occurs in conjunction with a right hemifacial edema, a right upper limb lymphedema, asymmetric bilateral calves edemas, and a unilateral massive pleural effusion seems never to be reported before. In addition, increased enteric protein loss that may cause severe hypoproteinemia usually get overlooked, and the lymphatic system disorders always put the diagnoses in a dilemma.
We described a case of a 17-year-old Chinese girl with a history of gradually progressive swellings of right-sided face, right upper limb, and bilateral calves since 3 to 4 months of age. A right-sided massive pleural effusion, a moderate pericardial effusion, and a mild ascites have been proved unchanged by a series of computerized tomography (CT) scans since 5 years ago. The diagnosis of PIL was finally confirmed by severe hypoproteinemia, endoscopic changes, and histology of jejunum biopsy. Further lymphoscintigraphy and lymphangiography also identified lymph leakage in her bowel and several abnormal lymphatic vessels. A high-protein, low-fat diet supplemented with medium-chain triglycerides (MCT) showed some benefit.
This case suggested that PIL was a rare but important etiology of hypoproteinemia, effusions, and edemas. PIL, effusions, and lymphedema can be the features of multisegmental generalized lymphatic dysplasia. In addition, both lymphoscintigraphy and intranodal lymphangiography could be considered when lymphatic system disorders are suspected.
Url:
DOI: 10.1097/MD.0000000000002849
PubMed: 26962779
PubMed Central: 4998860
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PMC:4998860Le document en format XML
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<author><name sortKey="Jin, Hong" sort="Jin, Hong" uniqKey="Jin H" first="Hong" last="Jin">Hong Jin</name>
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<author><name sortKey="Wu, Weilu" sort="Wu, Weilu" uniqKey="Wu W" first="Weilu" last="Wu">Weilu Wu</name>
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<term>Biopsy</term>
<term>Diagnosis, Differential</term>
<term>Diet, Fat-Restricted (methods)</term>
<term>Dietary Proteins (administration & dosage)</term>
<term>Edema (diagnosis)</term>
<term>Edema (etiology)</term>
<term>Endoscopy, Digestive System (methods)</term>
<term>Extremities (pathology)</term>
<term>Female</term>
<term>Humans</term>
<term>Hypoproteinemia (etiology)</term>
<term>Hypoproteinemia (physiopathology)</term>
<term>Jejunum (pathology)</term>
<term>Lymphangiectasis, Intestinal (complications)</term>
<term>Lymphangiectasis, Intestinal (diagnosis)</term>
<term>Lymphangiectasis, Intestinal (diet therapy)</term>
<term>Lymphangiectasis, Intestinal (physiopathology)</term>
<term>Pericardial Effusion (diagnosis)</term>
<term>Pericardial Effusion (etiology)</term>
<term>Pleural Effusion (diagnosis)</term>
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<term>Tomography, X-Ray Computed (methods)</term>
<term>Triglycerides (administration & dosage)</term>
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<term>Diagnostic différentiel</term>
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<term>Femelle</term>
<term>Humains</term>
<term>Hypoprotéinémie (physiopathologie)</term>
<term>Hypoprotéinémie (étiologie)</term>
<term>Jéjunum (anatomopathologie)</term>
<term>Lymphangiectasie intestinale ()</term>
<term>Lymphangiectasie intestinale (diagnostic)</term>
<term>Lymphangiectasie intestinale (diétothérapie)</term>
<term>Lymphangiectasie intestinale (physiopathologie)</term>
<term>Membres (anatomopathologie)</term>
<term>Oedème (diagnostic)</term>
<term>Oedème (étiologie)</term>
<term>Protéines alimentaires (administration et posologie)</term>
<term>Régime pauvre en graisses ()</term>
<term>Tomodensitométrie ()</term>
<term>Triglycéride (administration et posologie)</term>
<term>Épanchement pleural (diagnostic)</term>
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<term>Épanchement péricardique (diagnostic)</term>
<term>Épanchement péricardique (étiologie)</term>
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<term>Triglycéride</term>
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<term>Lymphangiectasis, Intestinal</term>
<term>Pericardial Effusion</term>
<term>Pleural Effusion</term>
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<term>Épanchement péricardique</term>
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<term>Hypoproteinemia</term>
<term>Pericardial Effusion</term>
<term>Pleural Effusion</term>
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<term>Endoscopy, Digestive System</term>
<term>Tomography, X-Ray Computed</term>
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<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Extremities</term>
<term>Jejunum</term>
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<term>Lymphangiectasie intestinale</term>
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<term>Femelle</term>
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<front><div type="abstract" xml:lang="en"><title>Abstract</title>
<p>Primary intestinal lymphangiectasia (PIL) is a rare disorder of unknown etiology characterized by diffuse or localized dilation and eventual rupture of the enteric lymphatic vessels in mucosa, submucosa, and/or subserosa. Lymph, rich in all kinds of proteins and lymphocytes, leaks into the gastrointestinal tract via the affected lymphatic vessels causing hypoproteinemia and lymphopenia. The main symptom is variable degrees of pitting edemas of bilateral lower limbs. But edemas of any other parts of body, and mild serous effusions may also occur sometimes. PIL occurs in conjunction with a right hemifacial edema, a right upper limb lymphedema, asymmetric bilateral calves edemas, and a unilateral massive pleural effusion seems never to be reported before. In addition, increased enteric protein loss that may cause severe hypoproteinemia usually get overlooked, and the lymphatic system disorders always put the diagnoses in a dilemma.</p>
<p>We described a case of a 17-year-old Chinese girl with a history of gradually progressive swellings of right-sided face, right upper limb, and bilateral calves since 3 to 4 months of age. A right-sided massive pleural effusion, a moderate pericardial effusion, and a mild ascites have been proved unchanged by a series of computerized tomography (CT) scans since 5 years ago. The diagnosis of PIL was finally confirmed by severe hypoproteinemia, endoscopic changes, and histology of jejunum biopsy. Further lymphoscintigraphy and lymphangiography also identified lymph leakage in her bowel and several abnormal lymphatic vessels. A high-protein, low-fat diet supplemented with medium-chain triglycerides (MCT) showed some benefit.</p>
<p>This case suggested that PIL was a rare but important etiology of hypoproteinemia, effusions, and edemas. PIL, effusions, and lymphedema can be the features of multisegmental generalized lymphatic dysplasia. In addition, both lymphoscintigraphy and intranodal lymphangiography could be considered when lymphatic system disorders are suspected.</p>
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