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Primary intestinal lymphangiectasia: Minireview

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World Journal of Clinical Cases 2014年第10期2卷 528-533页

作者： Sachin B Ingle Chitra R Hinge(Ingle) Department of Pathology MIMSR Medical College Latur Maharashtra 4132512 India Department of Physiology MIMSR Medical College Latur Maharashtra 4132512 India

Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing *** often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.

关键词： Primary idiopathic intestinal lymphangiectasia Mucosa-submucosa Protein loosing enteropathy Double balloon endoscopy Biopsy

Intestinal lymphangiectasia in adults

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World Journal of Gastrointestinal Oncology 2011年第2期3卷 19-23页

作者： Hugh James Freeman Michael Nimmo Department of Medicine (Gastroen-terology) University of British Columbia Department of Pathology University of British Columbia

Intestinal lymphangiectasia in the adult may be characterized as a disorder with dilated intestinal lacteals causing loss of lymph into the lumen of the small intestine and resultant hypoproteinemia, hypogamma-globulinemia, hypoalbuminemia and reduced number of circulating lymphocytes or lymphopenia. Most often, intestinal lymphangiectasia has been recorded in children, often in neonates, usually with other congenital abnormalities but initial def inition in adults including the elderly has become increasingly more common. Shared clinical features with the pediatric population such as bilateral lower limb edema, sometimes with lymphedema, pleural effusion and chylous ascites may occur but these reflect the severe end of the clinical spectrum. In some, diarrhea occurs with steatorrhea along with increased fecal loss of protein, reflected in increased fe- cal alpha-1-antitrypsin levels, while others may present with iron def iciency anemia, sometimes associated with occult small intestinal bleeding. Most lymphangiectasia in adults detected in recent years, however, appears to have few or no clinical features of malabsorption. Diagnosis remains dependent on endoscopic changes confirmed by small bowel biopsy showing histological evidence of intestinal lymphangiectasia. In some, video capsule endoscopy and enteroscopy have revealed moreextensive changes along the length of the small intes- tine. A critical diagnostic element in adults with lym- phangiectasia is the exclusion of entities (e.g. malignancies including lymphoma) that might lead to obstruction of the lymphatic system and "secondary" changes in the small bowel biopsy. In addition, occult infectious (e.g. Whipple's disease from Tropheryma whipplei) or in? am- matory disorders (e.g. Crohn's disease) may also present with profound changes in intestinal permeability and protein-losing enteropathy that also require exclusion. Conversely, rare B-cell type lymphomas have also been described even decades following ini

关键词： Intestinal lymphangiectasia Adults Submucosa

Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic

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World Journal of Hepatology 2014年第9期6卷 685-687页

作者： Raffaele Licinio Mariabeatrice Principi Enzo Ierardi Alfredo Di Leo Gastroenterology Unit Department of Emergency and Organ TransplantationUniversity of Bari

The relationship between primary intestinal lymphangiectasia(PIL) and liver fibrosis is an emerging topic with many obscure aspects due to the rarity of the disorder.A recent paper reported that a six-month lowfat diet improved liver *** report the case of a 17-year-old girl affected by PIL whose hepatic fibrosis progressively worsened within one year,despite dietetic *** and the previous case report describe extraordinary events,which do not allow clear-cut clinical aspects to be ***,both cases suggest that in patients with PIL,it is necessary to closely monitor liver morphology with in-depth investigations including not only ultrasonography,but also elastography.

关键词： Hepatic transient elastography Liver fibrosis Low-fat diet Primary intestinal lymphangiectasia

Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia

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World Journal of Gastroenterology 2012年第43期18卷 6333-6337页

作者： Siham Al Sinani Yusria Al Rawahi Hamed Abdoon Child Health Department Sultan Qaboos University HospitalMuscat 123Sultanate of Oman

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.

关键词： Hennekam syndrome lymphangiectasia Octreotide Protein losing enteropathy

Intestinal lymphangiectasia:Understanding the bigger picture

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World Journal of Clinical Cases 2024年第18期12卷 3298-3303页

作者： Sudheer Marrapu Ramesh Kumar Department of Gastroenterology All India Institute of Medical SciencesPatna 801507India

Intestinal lymphangiectasia(IL)is characterized by the dilation of intestinal lymphatic vessels,which can rupture and cause loss of lymph into the *** to the high content of proteins,lipoproteins,and lymphocytes in the intestinal lymph,loss of lymph might result in hypoproteinemia,hypoalbuminemia,hypogammaglobulinemia,and *** addition,there may be a depletion of minerals,lipids,and fat-soluble *** can be primary due to inherent malfunctioning of the lymphatic system,or secondly,a result of various factors that may hinder lymphatic drainage either directly or *** condition has emerged as a subject of significant clinical *** that the intestinal lymphatic system plays an important role in the body’s fluid homeostasis,adaptive immunity,nutrient and drug absorption,intestinal transport,and systemic metabolism,its dysfunction may have wider *** primary IL is rare,with varied clinical features,complications,treatment response,and outcomes,secondary IL is more common than previously *** definitive diagnosis of IL requires endoscopic demonstration of whitish villi(which frequently resemble snowflakes)and histological confirmation of dilated lacteals in the small intestinal *** of IL is challenging and involves dietary modifications,managing underlying medical conditions,and using medications such as sirolimus and *** its prevalence and diverse etiology is crucial for targeted management of this challenging medical *** article provides a comprehensive exploration of the clinical implications associated with *** addition,it offers valuable insights into critical knowledge gaps in the existing diagnostic and management landscape.

关键词： Intestinal lymphatics lymphangiectasia Waldman's disease Lacteals Lymph

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

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World Journal of Gastrointestinal Endoscopy 2011年第11期3卷 235-240页

作者： Tak Geun Oh Joo Won Chung Hee Man Kim Seok-Joo Han Jin Sung Lee Jung Yeob Park Si Young Song Division of Gastroenterology Department of Internal MedicineYonsei Institute of GastroenterologyYonsei University College of MedicineSeoul 120-752South Korea epartment of Pediatric Surgery Yonsei University College of MedicineSeoul 120-752South Korea Department of Pediatrics Yonsei University College of MedicineSeoul 120-752South Korea Division of Gastroenterology Department of Internal MedicineBrain Korea 21 Project for Medical Science and Severance Biomedical Science InstituteYonsei University College of MedicineSeoul 120-752South Korea

Primary intestinal lymphangiectasia(PIL)is a rare disorder characterized by dilated intestinal lymphatics and the development of protein-losing *** with PIL develop hypoalbuminemia,hypocalcemia,lymphopenia and hypogammaglobulinemia,and present with bilateral lower limb edema,fatigue,abdominal pain and *** reveals diffusely elongated,circumferential and polypoid mucosae covered with whitish enlarged villi,all of which indicate intestinal *** is conf irmed by characteristic tissue pathology,which includes dilated intestinal lymphatics with diffusely swollen mucosa and enlarged *** prevalence of PIL has increased since the introduction of capsule *** etiology and prevalence of PIL remain *** studies have reported that several genes and regulatory molecules for lymphangiogenesis are related to *** report the case of a patient with PIL involving the entire small bowel that was confirmed by capsule endoscopy and double-balloon enteroscopy-guided tissue pathology who carried a deletion on chromosome *** relationship between this deletion on chromosome 4 and PIL remains to be investigated.

关键词： Capsule endoscopy Double balloon enteroscopy Chromosome deletion Chromosome 4q25 Primary intestinal lymphangiectasia

Lymphatic dysfunction in advanced cirrhosis:Contextual perspective and clinical implications

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World Journal of Hepatology 2021年第3期13卷 300-314页

作者： Ramesh Kumar Utpal Anand Rajeev Nayan Priyadarshi Department of Gastroenterology All India Institute of Medical SciencesPatna 801507BiharIndia Department of Surgical Gastroenterology All India Institute of Medical SciencesPatna 801507BiharIndia Department of Radiodiagnosis All India Institute of Medical SciencesPatna 801507BiharIndia

The lymphatic system plays a very important role in body fluid homeostasis,adaptive immunity,and the transportation of lipid and waste *** patients with liver cirrhosis,capillary filtration markedly increases,primarily due to a rise in hydrostatic pressure,leading to enhanced production of ***,lymphatic vasculature expansion helps to prevent fluid from accumulating by returning it back to the systemic ***,the lymphatic functions become compromised with the progression of cirrhosis and,consequently,the lymphatic compensatory mechanism gets overwhelmed,contributing to the development and eventual worsening of ascites and *** changes,low-grade chronic inflammation,and compounding effects of predisposing factors such as old age,obesity,and metabolic syndrome appear to play a significant role in the lymphatic dysfunction of *** portal hypertension can contribute to the development of intestinal lymphangiectasia,which may rupture into the intestinal lumen,resulting in the loss of protein,chylomicrons,and lymphocyte,with many clinical ***,due to high pressure,the rupture of the subserosal lymphatics into the abdomen results in the formation of chylous *** being highly significant,lymphatic dysfunctions in cirrhosis have largely been ignored;its mechanistic pathogenesis and clinical implications have not been studied in *** recommendation exists for the diagnostic evaluation and therapeutic strategies,with respect to lymphatic dysfunction in patients with *** article discusses the perspectives and clinical implications,and provides insights into the management strategies for lymphatic dysfunction in patients with cirrhosis.

关键词： Lymphatic dysfunction Cirrhosis Lymphedema lymphangiectasia Chylous ascites Refractory ascites

Anaemia in Waldmann's disease: A rare presentation of a rare disease

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World Journal of Gastrointestinal Endoscopy 2015年第5期7卷 567-572页

作者： Shahira A El-Etreby Ahmed Y Altonbary Mohamed El Sorogy Wagdi Elkashef Jehan A Mazroa Monir H Bahgat Divisions of Hepatology and Gastroenterology Pathology and Diagnostic Radiology Specialized Medical Hospital and Gastrointestinal Surgery Center

A 32-year-old female presented with 5-year history of iron deficiency anemia, marked pallor and edema of both lower limbs. Laboratory investigations including complete blood count, blood film, iron studies, lipid profile, ascitic fluid analysis, test of stool for occult blood and alpha 1 anti-trypsin. Upper, lower gastrointestinal(GIT) endoscopies, and enteroscopy were performed. Imaging techniques as abdominal ultrasonography and computed tomography were done. Echocardiography, lymph node biopsy and bone marrow examination were normal. The case was diagnosed as Waldmann's disease with protein losing enteropathy and recurrent GIT bleeding. Management started with low fat diet with medium chain triglyceride, octreotide 200 μg twice a day, tranexamic acid and blood transfusion. Then, exploratory laparotomy with pathological examination of resected segment was done when recurrent GIT bleeding occurred and to excluded malignant transformation.

关键词： Waldmann's disease lymphangiectasia Gastrointestinal bleeding Iron deficiency anemia