956 results match your criteria Intestinal Lymphangiectasia


Hypomagnesemia in intestinal lymphangiectasia: a case report and review of the literature.

BMC Gastroenterol 2022 May 15;22(1):246. Epub 2022 May 15.

Department of Gastroenterology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.

Background: Intestinal lymphangiectasia (IL) is a rare disease characterized by dilation of lymphatic vessels and leakage of lymphatic fluids into the intestinal lumen, causing depletion of lymphocytes, protein, lipids, fat-soluble vitamins, and electrolytes. Hypomagnesemia can occur in IL patients but is seldom discussed.

Case Presentation: A 30-year-old Tibetan woman who had chronic diarrhea, edema, tetany, and tingling was diagnosed with IL. Read More

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Evaluation of the degree and distribution of lymphangiectasia in full-thickness canine small intestinal specimens diagnosed with lymphoplasmacytic enteritis and granulomatous lymphangitis.

J Vet Med Sci 2022 Apr 14;84(4):566-573. Epub 2022 Mar 14.

Department of Veterinary Internal Medicine, Graduate School of Agricultural and Life Sciences, The University of Tokyo, Tokyo, Japan.

Intestinal lymphangiectasia (IL) is often observed in dogs with chronic small intestinal diseases. Hypoplasia of the lymphatic vessel due to decreased lymphangiogenesis, which has been suggested in human idiopathic IL, may contribute to the pathogenesis of canine IL. This study aimed to evaluate the diameter and number of lymphatic vessels in full-thickness small intestinal specimens of dogs with IL. Read More

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Primary intestinal lymphangiectasia: a rare disease as a cause of protein-losing enteropathy.

Rev Esp Enferm Dig 2022 Mar 4. Epub 2022 Mar 4.

Aparato Digestivo, Complejo Asistencial Universitario de León, España.

Primary intestinal lymphangiectasia is a rare disorder associated with protein-losing enteropathy. The main manifestations are those resulting from hypoalbuminemia. Diagnosis requires the typical endoscopic image of intestinal lymphangiectasia and increased 24-hour fecal alpha-1-antitrypsin clearance. Read More

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A rare case of intestinal lymphangiectasia induced by pazopanib.

Int Cancer Conf J 2022 Jan 1;11(1):87-90. Epub 2021 Dec 1.

Department of Urology, Kyoto University Graduate School of Medicine, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto, 606-8507 Japan.

A 62-year-old man underwent left radical nephrectomy for left renal cell carcinoma at our hospital in 1999. At the age of 79 years, he was diagnosed with intra-abdominal disseminations, lung metastases, pancreas metastases, and bilateral femoral muscle metastases during a routine follow-up computed tomography scan. The patient began treatment with pazopanib. Read More

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January 2022

Protein-losing enteropathy caused by a jejunal ulcer after an internal hernia in Petersen's space: A case report.

World J Clin Cases 2022 Jan;10(1):323-330

Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Nippon Medical School, Tokyo 113-8603, Japan.

Background: The incidence of internal hernias has recently increased in concordance with the popularization of laparoscopic surgery. Of particular concern are internal hernias occurring in Petersen's space, a space that is surgically created after treatment for gastric cancer and obesity. These hernias cause devastating sequelae, such as massive intestinal necrosis, fatal Roux limb necrosis, and superior mesenteric vein thrombus. Read More

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January 2022

Redefining WILD syndrome: a primary lymphatic dysplasia with congenital multisegmental lymphoedema, cutaneous lymphovascular malformation, CD4 lymphopaenia and warts.

J Med Genet 2021 Dec 16. Epub 2021 Dec 16.

Infection Care Group, St George's University Hospitals NHS Foundation Trust, London, UK.

Background: Primary lymphoedema (PL) syndromes are increasingly recognised as presentations of complex genetic disease, with at least 20 identified causative genes. Recognition of clinical patterns is key to diagnosis, research and therapeutics. The defining criteria for one such clinical syndrome, 'WILD syndrome' (arts, mmunodeficiency, ymphoedema and anogenital ysplasia), have previously depended on a single case report. Read More

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December 2021

A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma.

BMC Gastroenterol 2021 Dec 11;21(1):461. Epub 2021 Dec 11.

Department of Gastroenterology, Qingdao Municipal Hospital, Jiaozhou Road 1#, Qingdao, 266071, People's Republic of China.

Background: Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. Increasing evidence has demonstrated an association between PIL and lymphoma.

Case Presentation: A 54-year-old man with a 20-year history of abdominal distension and bilateral lower limb edema was admitted. Read More

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December 2021

Histopathology of intestinal villi in neonatal and paediatric age: main features with clinical correlation - Part I.

Pathologica 2022 Feb 2;114(1):12-21. Epub 2021 Dec 2.

Institute of Pathology, Spedali Civili di Brescia, Brescia, Italy.

The neonatal and paediatric spectrum of small bowel disorders encompass a wide variety of conditions, ranging from food allergies to life-threatening surgical emergencies or life-long medical conditions and, as such, it comes with a whole set of diagnostic challenges for the non-paediatric pathologist. Histologic examination is a cornerstone of diagnosis in a large number of diseases and may still provide important diagnostic clues in the appropriate clinical context. In this review, divided in two sections, we aim to provide a comprehensive histopathological summary of paediatric small bowel alteration and their differential diagnoses with a reference to the main clinical aspects required for appropriate interpretation. Read More

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February 2022

Association between intestinal lymphangiectasia and expression of inducible nitric oxide synthase in dogs with lymphoplasmacytic enteritis.

J Vet Med Sci 2022 Jan 1;84(1):20-24. Epub 2021 Dec 1.

Department of Veterinary Internal Medicine, Graduate School of Agricultural and Life Sciences, The University of Tokyo, 1-1-1 Yayoi, Bunkyo-ku, Tokyo 113-8657, Japan.

Intestinal lymphangiectasia (IL) is a common complication in dogs. Since nitric oxide (NO) is known to relax the lymphatic vessel, we evaluated inducible NO synthase (iNOS) expression using immunohistochemistry in 13 dogs with lymphoplasmacytic enteritis (LPE) with or without IL. The duodenal iNOS expressing cells were significantly increased in dogs with IL-negative or IL-positive LPE dogs (P=0. Read More

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January 2022

Protein-losing enteropathy caused by disseminated Mycobacterium avium complex infection in a patient receiving antiretroviral therapy: an autopsy case report.

AIDS Res Ther 2021 11 29;18(1):90. Epub 2021 Nov 29.

Department of Infectious Diseases, Osaka City General Hospital, 2-13-22 Miyakojimahondori, Miyakojima-ku, Osaka, Osaka, 534-0021, Japan.

Background: Disseminated Mycobacterium avium complex infection is an important indicator of acquired immunodeficiency syndrome (AIDS) in patients with advanced human immunodeficiency virus (HIV) infection. Effective antiretroviral therapy has dramatically reduced the incidence of and mortality due to HIV infection, although drug resistance and poor medication adherence continue to increase the risk of disseminated M. avium complex infection. Read More

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November 2021

Clinicopathological and Fecal Proteome Evaluations in 16 Dogs Presenting Chronic Diarrhea Associated with Lymphangiectasia.

Vet Sci 2021 Oct 19;8(10). Epub 2021 Oct 19.

School of Biosciences and Veterinary Medicine, University of Camerino, Via Circonvallazione 93/95, 62024 Matelica, Italy.

Canine intestinal lymphangiectasia (IL) is a condition characterized by variably severe gastrointestinal signs, frequently associated with laboratory abnormalities; the research for markers allowing a better understanding of the severity degree and/or obtaining an early diagnosis and/or monitoring is continuously progressing. In the present study, we investigated possible new diagnostic/follow-up markers in IL dogs, namely, serum C-reactive protein, serum bacterial lipopolysaccharide, serum cleaved cytokeratin 18, serum citrulline, and zonulin (in both serum and feces). A fecal proteomic study looking for possible confirmation and/or new marker candidates was also performed. Read More

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October 2021

Primary Intestinal Lymphangiectasia in a Middle-Aged Female.

Saudi J Med Med Sci 2021 Sep-Dec;9(3):280-281. Epub 2021 Sep 4.

Department of Pathology, Bhopal Memorial Hospital and Research Centre, Bhopal, Madhya Pradesh, India.

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September 2021

Primary intestinal lymphangiectasia in children: Twelve years of experience in the diagnosis and management.

Asia Pac J Clin Nutr 2021 Sep;30(3):358-364

Department of Clinical Nutrition, Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China. Email:

Background And Objectives: Primary Intestinal Lymphangiectasia (PIL) is a rare congenital and digestive disease, which could present through a broad spectrum of clinical manifestations, diagnostic and treatment management. The aim of this study was to introduce the diagnosis and nutrition treatment of children with PIL through the twelve years of experience.

Methods And Study Design: The patients diagnosed with PIL admitted to the Department of Gastroenterology and Nutrition in Xinhua Hospital from June 2006 to September 2017 were included in the study. Read More

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September 2021

The Update of Treatment for Primary Intestinal Lymphangiectasia.

Pediatr Gastroenterol Hepatol Nutr 2021 Sep 8;24(5):413-422. Epub 2021 Sep 8.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy. Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. Read More

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September 2021

Drugs in Focus: Octreotide Use in Children With Gastrointestinal Disorders.

J Pediatr Gastroenterol Nutr 2022 01;74(1):1-6

Department of Paediatric Gastroenterology and Nutrition, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Abstract: Octreotide, a somatostatin analogue, has been used for more than 20 years in children with gastrointestinal bleeding, chylothorax or chylous ascites, intestinal lymphangiectasia, pancreatitis, intestinal dysmotility, and severe diarrhoea; however, until now, there is a lack of randomised clinical trials evaluating the efficacy of this compound in childhood. Hence, we aimed to review the literature in order to determine the evidence of its use and safety in children, using PubMed from 2000 to 2021 with the search terms "octreotide" and "children" and "bleeding or chylous ascites or chylothorax or acute pancreatitis or lymphangiectasia or diarrhoea or intestinal dysmotility". Read More

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January 2022

Dynamic Contrast Magnetic Resonance Lymphangiography Localizes Lymphatic Leak to the Duodenum in Protein-Losing Enteropathy.

J Pediatr Gastroenterol Nutr 2022 01;74(1):38-45

Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA.

Objectives: Protein-losing enteropathy (PLE) is a disorder of intestinal lymphatic flow resulting in leakage of protein-rich lymph into the gut lumen. Our primary aim was to report the imaging findings of dynamic contrast magnetic resonance lymphangiography (DCMRL) in patients with PLE. Our secondary objective was to use these imaging findings to characterize lymphatic phenotypes. Read More

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January 2022

Complicated primary intestinal lymphangiectasia (Waldmann's disease) in a child successfully treated with octreotide: A case report from a low-resource setting.

Ann Med Surg (Lond) 2021 Aug 29;68:102588. Epub 2021 Jul 29.

Department of Pediatrics, Mohammed VI University Hospital, Oujda, Morocco.

Introduction And Importance: The primary intestinal lymphangiectasia is a rare exudative enteropathy of unknown etiology that affects the lymphatic system. It causes lymphedema and malabsorption syndrome by the escape of the lymph and its elements into the intestinal lumen.

Case Presentation: A female patient, diagnosed at the age of 11 with Waldmann's disease, has initially manifested chronic diarrhea with a stature-ponderal delay at the age of 6 months old; she was treated for a long time as celiac disease patient. Read More

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Extended phenotypes of PIEZO1-related lymphatic dysplasia caused by two novel compound heterozygous variants.

Eur J Med Genet 2021 Oct 8;64(10):104295. Epub 2021 Aug 8.

Department of Pediatrics, Seoul National University College of Medicine, Seoul National University Children's Hospital, Seoul, South Korea; Rare Disease Center, Seoul National University Hospital, Seoul, South Korea. Electronic address:

Defects in the PIEZO1 gene cause lymphatic dysplasia in an autosomal recessive manner, mostly by loss-of-function variants. Moreover, since 2019, the role of PIEZO1 in bone formation has been established, but there have been no PIEZO1-related cases presenting definite skeletal involvement to date. A 21-year-old male with primary lymphatic dysplasia had some other distinctive clinical features, including multiple fracture history during infancy, thoracolumbar scoliosis, short stature, and left-sided facial bone hypoplasia. Read More

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October 2021

An unusual cause of heparin resistance - A case report.

Ann Card Anaesth 2021 Jul-Sep;24(3):375-377

Department of Cardiothoracic and Vascular Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals that result in leakage of excessive serum proteins and lymphocytes into the gastrointestinal (GI) tract culminating in protein-losing enteropathy. The GI loss of protein and possible antithrombin III (AT-III) loss creates a prothrombotic environment. The surgical management of congenital heart disease (CHD) in presence of PIL can present with altered heparin response and can impose problems in instituting cardiopulmonary bypass (CPB). Read More

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November 2021

Predicting the Most Deleterious Missense Nonsynonymous Single-Nucleotide Polymorphisms of Hennekam Syndrome-Causing CCBE1 Gene, In Silico Analysis.

ScientificWorldJournal 2021 10;2021:6642626. Epub 2021 Jun 10.

Department of Immunochemistry, Institute of Chemical Engineering, Ural Federal University, Yekaterinburg, Russia.

Hennekam lymphangiectasia-lymphedema syndrome has been linked to single-nucleotide polymorphisms in the CCBE1 (collagen and calcium-binding EGF domains 1) gene. Several bioinformatics methods were used to find the most dangerous nsSNPs that could affect CCBE1 structure and function. Using state-of-the-art in silico tools, this study examined the most pathogenic nonsynonymous single-nucleotide polymorphisms (nsSNPs) that disrupt the CCBE1 protein and extracellular matrix remodeling and migration. Read More

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January 2022

Intestinal lymphangiectasia in an adult: case report.

J Vasc Bras 2021 Jun 11;20:e20200160. Epub 2021 Jun 11.

Hospital Santa Paula, Vitória, ES, Brasil.

Intestinal lymphangiectasia is a group of rare diseases characterized by dilation of lymphatic channels. Its pathophysiology comprises obstruction of small bowel lymphatic drainage with secondary dilation of mucosal, submucosal, or subserous lymphatic vessels, distorting villous architecture and causing loss of lymph into the intestinal lumen, leading to malabsorption. The affected lymphatic vessels are primarily located in the small intestine, which is affected to a varying extent. Read More

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Exploration of Potential Immunodeficiency Unveils Hennekam Lymphangiectasia-Lymphedema Syndrome.

J Clin Immunol 2021 10 26;41(7):1674-1676. Epub 2021 Jun 26.

Department of Pediatrics, Leuven University Hospitals, Herestraat 49, 3000, Leuven, Belgium.

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October 2021

Home parenteral nutrition a life-saving therapy in a primary intestinal lymphangiectasia patient affecting the entire GI tract - 3 year follow-up case report.

Ann Med Surg (Lond) 2021 Jul 9;67:102483. Epub 2021 Jun 9.

Department of Gastroenterology, Dietetics and Internal Diseases, Poznan University of Medical Sciences, 49 Przybyszewskiego Street, 60-355, Poznan, PL, Poland.

Introduction And Importance: Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy of unknown etiology, characterized by impaired lymphatic vessels drainage. The pathological changes in PIL result in usually localized or diffuse dilatation of intestinal lacteals, leading to leakage of lymphatic fluid rich of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. PIL may be asymptomatic or mildly symptomatic in moderate forms of the disease. Read More

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Implications of lymphatic alterations in the pathogenesis and treatment of inflammatory bowel disease.

Biomed Pharmacother 2021 Aug 24;140:111752. Epub 2021 May 24.

Key Laboratory of Medical Science and Laboratory Medicine of Jiangsu Province, School of Medicine, Jiangsu University, Zhenjiang 212013, Jiangsu, PR China. Electronic address:

Inflammatory bowel disease (IBD) is characterized by intense immune dysregulation, gut microbiota imbalance, and intestinal epithelium destruction. Among the factors that contribute to the pathogenesis of IBD, lymphatics have received less attention, hence less studied, characterized, and explored. However, in recent years, the role of the lymphatic system in gastrointestinal pathophysiology continues to be highlighted. Read More

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Primary intestinal lymphangiectasia presenting as limb hemihyperplasia: a case report and literature review.

Authors:
Ammar A Khayat

BMC Gastroenterol 2021 May 18;21(1):225. Epub 2021 May 18.

Department of Pediatrics, Gastroenterology Unit, Department of Pediatrics, Faculty of Medicine, Umm AL Qura University, King Abdulaziz University, 24381, Al-Abdiyyah, Makkah, Saudi Arabia.

Background: Primary intestinal lymphangiectasia is an exceedingly rare disorder. Epidemiology is unknown. It usually presents with lower extremity swelling, diarrhea, ascites, and protein-losing enteropathy. Read More

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Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia.

Yonsei Med J 2021 May;62(5):470-473

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Primary intestinal lymphangiectasia (IL) can cause leakage of lymphatic fluids into the gastrointestinal tract, eventually leading to protein-losing enteropathy. A 15-year-old male patient, whose disease began at the age of 8 years, recently felt worsening general weakness. After diagnosing abnormal lymphatic lesions in the duodenum through endoscopy with biopsy and contrast-enhanced magnetic resonance lymphangiography, glue embolization of the leaking duodenal lymphatic channel was successfully performed. Read More

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Refractory primary intestinal lymphangiectasia effectively managed with subcutaneous octreotide.

BMJ Case Rep 2021 Apr 9;14(4). Epub 2021 Apr 9.

General Surgery, Highgate Private Hospital, London, Highgate, UK.

This case report describes a young man with a history of lymphoedema and long-standing gastrointestinal symptoms since childhood. After undergoing extensive investigations, he was diagnosed with primary intestinal lymphangiectasia (IL). The patient's condition was refractory to conventional medium-chain triglyceride diet but responded well to treatment with subcutaneous octreotide. Read More

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Lymphatic dysfunction in advanced cirrhosis: Contextual perspective and clinical implications.

World J Hepatol 2021 Mar;13(3):300-314

Department of Radiodiagnosis, All India Institute of Medical Sciences, Patna 801507, Bihar, India.

The lymphatic system plays a very important role in body fluid homeostasis, adaptive immunity, and the transportation of lipid and waste products. In patients with liver cirrhosis, capillary filtration markedly increases, primarily due to a rise in hydrostatic pressure, leading to enhanced production of lymph. Initially, lymphatic vasculature expansion helps to prevent fluid from accumulating by returning it back to the systemic circulation. Read More

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Hennekam lymphangiectasia syndrome: A rare case of primary lymphedema.

Indian J Dermatol Venereol Leprol 2021 Mar-Apr;87(2):240-244

Department of Dermatology and STD, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India.

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November 2021

[Intestinal lymphangiectasia in a patient with Sanfilippo B syndrome].

Arch Argent Pediatr 2021 04;119(2):e138-e141

Servicio de Anatomía Patológica, Hospital Clínico Universitario Lozano Blesa. Zaragoza, España.

Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with mediumchain triglyceride, our patient presents clinical improvement until today. Read More

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