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primary intestinal lymphangiectasia in an adult patient:A case report and review of literature

World Journal of Gastroenterology 2020年第48期26卷 7707-7718页

作者： Rudolf Huber Georg Semmler Alexander Mayr Felix Offner Christian Datz Department of Internal Medicine General Hospital OberndorfTeaching Hospital of the Paracelsus Medical University SalzburgOberndorf 5110Austria Department of Pathology General Hospital FeldkirchFeldkirch 6800Austria

BACKGROUND primary intestinal lymphangiectasia(PIL),first described in 1961,is a rare disorder of unknown etiology resulting in protein-losing *** disease is characterized by dilatation and leakage of intestinal lymph vessels leading to hypoalbuminemia,hypogammaglobulinemia,and *** the severity and location of lymph vessels being affected can vary considerably,the range of associated symptoms is wide from mild lower-limb edema to generalized edema,abdominal and/or pleural effusion,and recurrent diarrhea,among *** usually developing in early childhood,we present the case of a 34-yearold woman with ***,we performed a literature review systematically assessing clinical presentation,and provide a practical approach to facilitate diagnosis and therapy of PIL in *** SUMMARY Our patient presented with unspecific symptoms of abdominal discomfort,fatigue,nausea,and recurrent edema of the lower ***,a striking collinearity of clinical symptoms with female hormone status was ***,polyglobulia,hypoalbuminemia,hypogammaglobulinemia,and transient lymphocytopenia were *** to suspicion of a bone marrow disease,an extensive diagnostic investigation was carried out excluding secondary causes of polyglobulinemia and *** diagnosis of primary intestinal lymphangiectasia was established after 22 wk by histological analysis of biopsy samples obtained via ***,the patient was put on a high-protein and low-fat diet with medium-chain triglycerides supplementation leading to significant improvement of clinical symptoms until 2 years of *** PIL can be the reason for cryptogenic hypoalbuminemia,hypogammaglobulinemia,and lymphopenia in *** to difficulty in correct diagnosis,treatment initiation is often delayed despite being effective and *** leads to a significant disease burden in affected *** is increasingly been

关键词： primary intestinal lymphangiectasia Waldmann´s disease Protein losing enteropathy Hypoproteinemia Case report

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primary intestinal lymphangiectasia presenting as limb convulsions:A case report

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World Journal of Clinical Cases 2022年第18期10卷 6234-6240页

作者： Yun Cao Xiao-Hong Feng Hai-Xiang Ni Department of Endocrinology The First Affiliated Hospital of Zhejiang Chinese Medical University(Zhejiang Provincial Hospital of Traditional Chinese Medicine)Hangzhou 310006Zhejiang ProvinceChina

BACKGROUND primary intestinal lymphangiectasia(PIL)is a rare protein-losing enteropathy characterized by abnormally dilated lymphatic structures,resulting in leakage of lymph(rich in protein,lymphocytes,and fat)from the intestinal mucosal and submucosal layers and thus hypoproteinemia,lymphopenia,hypolipidemia,and pleural *** SUMMARY A 19-year-old Chinese male patient complained of recurrent limb convulsions for the last 1 *** investigations revealed low levels of calcium and magnesium along with hypoproteinemia and high parathyroid hormone levels,whereas gastroscopy exhibited chronic non-atrophic gastritis and duodenal lymphatic *** gastric biopsy showed moderate chronic inflammatory cell infiltration distributed around a small mucosal patch in the descending duodenum followed by lymphatic dilatation in the mucosal lamina propria,which was later diagnosed as *** following appropriate mediumchain triglycerides nutritional support significantly improved the patient’s *** Since several diseases mimic the clinical symptoms displayed by PIL,like limb convulsions,low calcium and magnesium,and loss of plasma proteins,it is imperative to conduct a detailed analysis to avoid any misdiagnosis while pinpointing the correct clinical diagnosis and simultaneously ruling out other clinical aspects in the reported cases without any past disease history.A careful assessment should always be made to ensure an accurate diagnosis in a timely manner so that the patient can be delivered quality health services for a positive health outcome.

关键词： Protein-losing enteropathy primary intestinal lymphangiectasia Limb convulsions Adult Case report

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How to treat an extensive form of primary intestinal lymphangiectasia?

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World Journal of Gastroenterology 2015年第23期21卷 7320-7325页

作者： Rosana Troskot Dragan Jurcic Ante Bilic Marija Gomercic Palcic Stanko Tezak Ivana Brajkovic Faculty of Medicine J.J.Strossmayer University of OsijekDivision of Gastroenterology and HepatologyDepartment of Internal MedicineClinical Hospital“Sveti Duh” Division of Gastroenterology and Hepatology Department of Internal Medicine“Sestre milosrdnice”University Hospital Center University of Zagreb School of Medicine Clinical Department of Nuclear Medicine and Radiation Protection University Hospital Centre Zagreb Dialysis clinic Polyclinic“Sveti Duh”II

We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia,which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal *** numerous therapeutic strategies are available,only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.

关键词： primary intestinal lymphangiectasia Malabsorption Seizure Treatment Diet Octreotide

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Tuberous sclerosis complex presenting as primary intestinal lymphangiectasia: A case report

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World Journal of Clinical Cases 2020年第10期8卷 1995-2000页

作者： Wen-Hao Lin Zu-Han Zhang Hong-Li Wang Lu Ren Lan-Lan Geng Department of Gastroenterology Guangzhou Women and Children's Medical CenterGuangzhou Medical UniversityGuangzhou 510623Guangdong ProvinceChina

BACKGROUND primary intestinal lymphangiectasia(PIL)is a rare congenital protein-losing enteropathy caused by dysplasia of the small intestinal *** cause of the disease is *** a literature review,we found that PIL and tuberous sclerosis complex(TSC)have some common symptoms and molecular *** SUMMARY Here,we present the case of a patient with a three-year history of primary intestinal *** patient most recently visited the hospital with abdominal distension and swelling of the left *** mother told us that she was diagnosed with TSC one year previously,which alerted us because the patient had multiple regions of *** evaluate the condition of the child and make a definite diagnosis,multiple imaging examinations were performed,as was TSC gene *** results met the diagnostic criteria for *** patient was discharged after symptomatic *** a review of the literature,it can be seen that changes at the molecular gene level of TSC can lead to abnormal lymphatic *** In summary,when patients with hypomelanotic macules or enamel hypoplasia are diagnosed with PIL,TSC gene screening may be important for further diagnosis.

关键词： primary intestinal lymphangiectasia Tuberous sclerosis complex Lymphedema Hypoproteinemia Children Case report

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Endoscopic classification and pathological features of primary intestinal lymphangiectasia

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World Journal of Gastroenterology 2022年第22期28卷 2482-2493页

作者： Ming-Ming Meng Kui-Liang Liu Xin-Ying Xue Kun Hao Jian Dong Chun-Kai Yu Hong Liu Cang-Hai Wang Hui Su Wu Lin Guo-Jun Jiang Nan Wei Ren-Gui Wang Wen-Bin Shen Jing Wu Department of Gastroenterology Beijing Shijitan HospitalCapital Medical UniversityBeijing 100038China Department of Respiratory and Critical Care Medicine Beijing Shijitan HospitalCapital Medical UniversityBeijing 100038China Departments of Lymphatic Surgery Beijing Shijitan HospitalCapital Medical UniversityBeijing 100038China Departments of Radiology Beijing Shijitan HospitalCapital Medical UniversityBeijing 100038China Departments of Pathology Beijing Shijitan HospitalCapital Medical UniversityBeijing 100038China

BACKGROUND The appearance of the intestinal mucosa during endoscopy varies among patients with primary intestinal lymphangiectasia(PIL).AIM To classify the endoscopic features of the intestinal mucosa in PIL under endoscopy,combine the patients’imaging and pathological characteristics of the patients,and explain their *** We retrospectively analyzed the endoscopic images of 123 patients with PIL who were treated at the hospital between January 1,2007 and December 31,*** compared and analyzed all endoscopic images,classified them into four types according to the endoscopic features of the intestinal mucosa,and analyzed the post-lymphographic computed tomography(PLCT)and pathological characteristics of each *** According to the endoscopic features of PIL in 123 patients observed during endoscopy,they were classified into four types:nodular-type,granular-type,vesicular-type,and *** showed diffuse thickening of the small intestinal wall,and no contrast agent was seen in the small intestinal wall and mesentery in the patients with nodular and granular *** agent was scattered in the small intestinal wall and mesentery in the patients with vesicular and edematous *** of the small intestinal mucosal pathology revealed that nodular-type and granulartype lymphangiectasia involved the small intestine mucosa in four layers,whereas ectasia of the vesicular-and edematous-type lymphatic vessels largely involved the lamina propria mucosae,submucosae,and muscular *** Endoscopic classification,combined with the patients’clinical manifestations and pathological examination results,is significant and very useful to clinicians when scoping patients with suspected PIL.

关键词： primary intestinal lymphangiectasia Endoscopic features Post-lymphangiographic computed tomography Pathology

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