867 results match your criteria Intestinal Lymphangiectasia


Crohn's disease initiated with extraintestinal features.

BMJ Case Rep 2019 Apr 20;12(4). Epub 2019 Apr 20.

Department of Oral Health Policy and Epidemiology, Harvard School of Dental Medicine, Boston, Massachusetts, USA.

Crohn's disease (CD) is a multifactorial, chronic immune-mediated disorder. The oral cavity is involved in 0.5% to 20% of the patients with CD. Read More

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http://dx.doi.org/10.1136/bcr-2019-229916DOI Listing
April 2019
2 Reads

Mesenteric Lymphatic Alterations Observed During DSS Induced Intestinal Inflammation Are Driven in a TLR4-PAMP/DAMP Discriminative Manner.

Front Immunol 2019 26;10:557. Epub 2019 Mar 26.

Department of Physiology and Pharmacology, Inflammation Research Network, Snyder Institute for Chronic Diseases, University of Calgary, Calgary, AB, Canada.

Inflammatory bowel disease (IBD) is characterized by both acute and chronic phase inflammation of the gastro-intestinal (GI) tract that affect a large and growing number of people worldwide with little to no effective treatments. This is in part due to the lack of understanding of the disease pathogenesis and also the currently poorly described involvement of other systems such as the lymphatics. During DSS induced colitis, mice also develop a severe inflammation of terminal ileum with many features similar to IBD. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2019.00557
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http://dx.doi.org/10.3389/fimmu.2019.00557DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443629PMC
March 2019
3 Reads

Reversal of secondary protein-losing enteropathy after surgical revision of a jejunal Roux-en-Y loop in a patient after liver transplantation.

Am J Transplant 2019 Mar 14. Epub 2019 Mar 14.

Department of Pediatric Gastroenterology and Hepatology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Secondary protein-losing enteropathy (PLE) is a rare complication following pediatric liver transplantation (LT), mostly related to venous outflow obstruction of the liver. Here, we discuss a thus far unknown cause of secondary PLE following pediatric LT. A 7-month-old boy underwent LT with biliary anastomosis using a Roux-en-Y jejunal loop. Read More

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http://dx.doi.org/10.1111/ajt.15354DOI Listing
March 2019
1 Read

Phenotype and Genotype of a Cohort of Chinese Children with Early-Onset Protein-Losing Enteropathy.

J Pediatr 2019 Mar 7. Epub 2019 Mar 7.

Department of Gastroenterology, Children's Hospital of Fudan University, Shanghai, China.

Objectives: To examine the phenotypes and perform next-generation sequencing in children with early-onset protein-losing enteropathy.

Study Design: We performed a retrospective review of 27 children with early-onset protein-losing enteropathy. Patients were characterized on clinical, immunologic, and systemic involvements. Read More

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http://dx.doi.org/10.1016/j.jpeds.2018.12.003DOI Listing
March 2019
1 Read

Pediatric localized intestinal lymphangiectasia treated with resection.

Int Med Case Rep J 2019 11;12:23-27. Epub 2019 Feb 11.

University of Szeged, Department of Pediatrics, Szeged, Hungary,

Introduction: Primary intestinal lymphangiectasia (PIL) is a very rare disorder usually diagnosed before the third year of life or later in adulthood, presenting with pitting edema, hypoproteinemia and low immunoglobulin levels. The location and the extent of the affected bowel greatly influence the clinical manifestation. The localized or segmental form of PIL is extremely rare with only five pediatric cases reported worldwide. Read More

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https://www.dovepress.com/pediatric-localized-intestinal-lym
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http://dx.doi.org/10.2147/IMCRJ.S192940DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375111PMC
February 2019
3 Reads

Association of chronic enteropathy activity index, blood urea concentration, and risk of death in dogs with protein-losing enteropathy.

J Vet Intern Med 2019 Mar 19;33(2):536-543. Epub 2019 Feb 19.

Bristol Veterinary School, University of Bristol, Bristol, United Kingdom.

Background: Malnutrition is associated with increased risk of premature death in humans with inflammatory bowel disease.

Hypothesis/objective: To determine if historical, clinical, and laboratory markers of malnutrition in dogs at the time of histologic diagnosis of protein-losing enteropathy (PLE) caused by chronic enteropathy (CE) or lymphangiectasia are associated with increased risk of death.

Animals: Seventy-one client-owned dogs diagnosed with PLE. Read More

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http://dx.doi.org/10.1111/jvim.15448DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430906PMC
March 2019
1 Read

Comparative pathophysiology and management of protein-losing enteropathy.

J Vet Intern Med 2019 Mar 14;33(2):383-402. Epub 2019 Feb 14.

Department of Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota.

Protein-losing enteropathy, or PLE, is not a disease but a syndrome that develops in numerous disease states of differing etiologies and often involving the lymphatic system, such as lymphangiectasia and lymphangitis in dogs. The pathophysiology of lymphatic disease is incompletely understood, and the disease is challenging to manage. Understanding of PLE mechanisms requires knowledge of lymphatic system structure and function, which are reviewed here. Read More

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http://dx.doi.org/10.1111/jvim.15406DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430879PMC
March 2019
1 Read

Primary lymphangiectasia of the gastrointestinal tract.

Ter Arkh 2018 Apr;90(3):96-98

FSBEI HE "Dagestan State Medical University" Ministry of Health, Makhachkala, Russia.

Intestinal lymphangiectasia is a very rare pathology, characterized by the presence of enlarged lymphatic vessels in all layers of the intestinal wall and in the mesentery. As a result, "lymphatic lakes" are formed, through which lymph exudates into the lumen of the intestine. The main manifestation is hypoproteinemic edema. Read More

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http://dx.doi.org/10.26442/terarkh201890396-98DOI Listing
April 2018
3 Reads

Prevalence of Mycobacterium avium subspecies paratuberculosis IS 900 DNA in biopsy tissues from patients with Crohn's disease: histopathological and molecular comparison with Johne's disease in Fars province of Iran.

BMC Infect Dis 2019 Jan 7;19(1):23. Epub 2019 Jan 7.

Department of Pathology, School of Veterinary Medicine, Shiraz University, PO Box 71345-1731, Shiraz, Iran.

Background: Crohn's disease is a chronic enteritis of humans that affects the gastrointestinal tract, especially the terminal ileum, cecum and colon. The etiology of this disease is still unknown but seems to be multifactorial. There are reports about the potential link between Crohn's disease in humans and the causative agent of Johne's disease in ruminants. Read More

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http://dx.doi.org/10.1186/s12879-018-3619-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322312PMC
January 2019
4 Reads

CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease.

Authors:
Ahmet Ozen

Immunol Rev 2019 Jan;287(1):20-32

Division of Allergy and Immunology, Marmara University School of Medicine, Istanbul, Turkey.

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease was described in 1961 as an important cause of protein-losing enteropathy (PLE). PIL can be the sole finding in rare individuals or occur as part of a multisystemic genetic syndrome. Although genetic etiologies of many lymphatic dysplasia syndromes associated with PIL have been identified, the pathogenesis of isolated PIL (with no associated syndromic features) remains unknown. Read More

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http://dx.doi.org/10.1111/imr.12715DOI Listing
January 2019
3 Reads

A story of oedemas.

Ann Biol Clin (Paris) 2018 Dec;76(6):681-685

Laboratoire de biochimie, Hôpital Saint-Philibert, Lomme, France.

The Waldmann's disease is a primitive intestinal lymphangectasia. This exsudative enteropathy initiates a protein leakage by the digestive tract. Clinically, this syndrome is characterised by oedemas and biologically by hypoprotidemia and loss of lymphocytes T CD4+, which increases a risk for infections. Read More

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http://dx.doi.org/10.1684/abc.2018.1398DOI Listing
December 2018

[Primary intestinal lymphangiectasia: Could propranolol be an effective alternative treatment?]

An Pediatr (Barc) 2018 Oct 25. Epub 2018 Oct 25.

Servicio de Gastroenterología y Nutrición, Hospital Materno Infantil, Badajoz, España.

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http://dx.doi.org/10.1016/j.anpedi.2018.09.006DOI Listing
October 2018
1 Read

Novel Magnified Single-Balloon Enteroscopy Enables Observation of Jejunal White Spots Associated with Lymphangiectasia.

Dig Dis 2019 22;37(2):170-174. Epub 2018 Nov 22.

Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan.

A 59-year-old woman was diagnosed with primary intestinal lymphangiectasia (PIL), with characteristic findings on capsule enteroscopy and confirmation by histopathological examination of biopsy specimens. We viewed the abnormal jejunal mucosa using a newly developed magnifying single-balloon enteroscope (SIF-Y0007). Conventional observation showed leakage of chyle. Read More

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http://dx.doi.org/10.1159/000493578DOI Listing
February 2019
3 Reads

An additional case of Hennekam lymphangiectasia-lymphedema syndrome caused by loss-of-function mutation in ADAMTS3.

Am J Med Genet A 2018 Dec 18;176(12):2858-2861. Epub 2018 Nov 18.

Ambry Genetics, Aliso Viejo, California, USA.

Hennekam lymphangiectasia-lymphedema syndrome (HKLLS) is a genetically heterogeneous lymphatic dysplasia with characteristic of facial dysmorphism, neurocognitive impairments, and abnormalities of the pericardium, intestinal tract, and extremities. It is an autosomal recessive condition caused by biallelic mutations in CCBE1 (collagen- and calcium-binding epidermal growth factor domain-containing protein 1) (HKLLS1; OMIM 235510) or FAT4 (HKLLS2; OMIM 616006). CCBE1 acts via ADAMTS3 (a disintegrin and metalloprotease with thrombospondin motifs-3 protease) to enhance vascular endothelial growth factor C signaling. Read More

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http://doi.wiley.com/10.1002/ajmg.a.40633
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http://dx.doi.org/10.1002/ajmg.a.40633DOI Listing
December 2018
15 Reads

Milky Mesentery: Acute Abdomen with Chylous Ascites.

Indian Pediatr 2018 Oct;55(10):909-910

Department of Surgery, University College of Medical Sciences and Guru Teg Bahadur Hospital, Shahdara, Delhi, India.

Background: Clinical presentations of intestinal lymphangiectasia include pitting edema, chylous ascites, pleural effusion, diarrhea, malabsorption and intestinal obstruction.

Case Characteristics: An 8-year-old male child presented to the emergency department with clinical features of peritonitis, raising suspicion of appendicular or small bowel perforation.

Intervention/outcome: Diagnosis of chylous ascites with primary intestinal lymphangiectasia made on laparotomy. Read More

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October 2018
14 Reads

Eculizumab Is Safe and Effective as a Long-term Treatment for Protein-losing Enteropathy Due to CD55 Deficiency.

J Pediatr Gastroenterol Nutr 2019 Mar;68(3):325-333

The Genetics Institute, Rambam Health Care Campus.

Objectives: Loss of the complement inhibitor CD55 leads to a syndrome of early-onset protein-losing enteropathy (PLE), associated with intestinal lymphangiectasia and susceptibility to large-vein thrombosis. The in vitro and short-term treatment benefits of eculizumab (C5-inhibitor) therapy for CD55-deficiency have been previously demonstrated. Here we present the 18-months treatment outcomes for 3 CD55-deficiency patients with sustained therapeutic response. Read More

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http://Insights.ovid.com/crossref?an=00005176-900000000-9663
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http://dx.doi.org/10.1097/MPG.0000000000002198DOI Listing
March 2019
5 Reads

Primary Intestinal Lymphangiectasia (Waldmann's Disease).

Am J Gastroenterol 2019 Feb;114(2):197

Department of Pediatric Gastroenterology and Nutrition, School of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.

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http://dx.doi.org/10.1038/s41395-018-0413-0DOI Listing
February 2019
23 Reads

Primary intestinal lymphangiectasia and a review of the current literature.

Turk J Gastroenterol 2018 11;29(6):714-716

Department of Internal Medicine, Health Sciences University, İzmir Tepecik Health Practice and Research Center, İzmir, Turkey.

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http://www.turkjgastroenterol.org/eng/makale/5407/310/Full-T
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http://dx.doi.org/10.5152/tjg.2018.18596DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6284676PMC
November 2018
2 Reads

Intestinal lymphangiectasia: a rare cause of intussusception in an adolescent.

Ann R Coll Surg Engl 2019 Feb 16;101(2):e43-e44. Epub 2018 Oct 16.

Department of Surgical Gastroenterology, Jawaharlal Institute of Post Graduate Medical Education and Research, Pondicherry , Puducherry , India.

Intussusception in adolescents is usually idiopathic in nature. A 17-year-old woman with diffuse large B cell lymphoma presented with signs of intestinal obstruction after initiation of induction chemotherapy. On evaluation, the patient was diagnosed to have ileoileal intussusception with intestinal lymphangiectasia as the lead point. Read More

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https://publishing.rcseng.ac.uk/doi/10.1308/rcsann.2018.0182
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http://dx.doi.org/10.1308/rcsann.2018.0182DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351867PMC
February 2019
19 Reads

Magnified single-balloon enteroscopy in the diagnosis of intestinal follicular lymphoma: a case series.

Intest Res 2018 Oct 10;16(4):628-634. Epub 2018 Oct 10.

Department of Medicine, Shiga University of Medical Science, Otsu, Japan.

The objective of this study was to evaluate the magnified endoscopic findings in the diagnosis of follicular lymphoma in the small intestine in comparison with those of intestinal follicular lymphoma and lymphangiectasia. Four patients with follicular lymphoma and 3 with lymphangiectasia in the small intestine were retrospectively analyzed. A prototype magnifying singleballoon enteroscope was used. Read More

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http://irjournal.org/journal/view.php?doi=10.5217/ir.2018.00
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http://dx.doi.org/10.5217/ir.2018.00003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6223446PMC
October 2018
17 Reads

Atypical Clinical Presentation of Crohn's Disease with Superior Mesenteric Vein Obstruction and Protein-losing Enteropathy.

Intern Med 2019 Feb 12;58(3):369-374. Epub 2018 Sep 12.

Department of Internal Medicine, National Defense Medical College, Japan.

We herein report a 44-year-old man suffering from systemic edema due to protein-losing enteropathy (PLE) with superior mesenteric vein (SMV) obstruction and development of collateral veins, which subsequently proved to be a chronic result of thrombosis and a complication of Crohn's disease (CD). PLE was supposedly induced by both intestinal erosion and thrombosis-related lymphangiectasia, which was histologically proven in his surgically-resected ileal stenosis. Elemental diet and anti-TNFα agent improved his hypoalbuminemia after surgery. Read More

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http://dx.doi.org/10.2169/internalmedicine.1192-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395114PMC
February 2019
5 Reads

CT Lymphangiography (CTL) in Primary Intestinal Lymphangiectasia (PIL): A Comparative Study with Intraoperative Enteroscopy (IOE).

Acad Radiol 2019 Feb 6;26(2):275-281. Epub 2018 Jun 6.

Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi St, Haidian District, Beijing 100038, China. Electronic address:

Rationale And Objectives: To investigate the clinical feasibility of CT lymphangiography (CTL) in primary intestinal lymphangiectasia (PIL) by comparison with intraoperative enteroscopy (IOE) during exploratory laparotomy.

Materials And Methods: Eleven PIL patients (F/M, two/nine, age range 10-37 years) were recruited in this study, and they were performed IOE during exploratory laparotomy for suspected serious lymphatic-intestinal leakages. All the patients were performed CTL before surgery, and the imaging data were reviewed by two radiologists separately. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10766332183023
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http://dx.doi.org/10.1016/j.acra.2018.04.023DOI Listing
February 2019
22 Reads

Removal of Peritnoeo-venous-atrial shunt thrombus without cardiopulmonary bypass.

Asian Cardiovasc Thorac Ann 2018 Jun 7;26(5):387-389. Epub 2018 May 7.

5 Department of Interventional Radiology, Sir Charles Gairdner Hospital, Nedlands, Australia.

Thrombus formation is not uncommon in longstanding intracardiac catheters, but formation of a thrombus at the tip of a Peritnoeo-venous-atrial shunt, causing obstruction of the tricuspid valve, is a rare complication and frequently unrecognized. A large intracardiac thrombus causing valve obstruction requires surgical removal with the support of cardiopulmonary bypass which is associated with significant morbidity. We successfully removed a thrombus attached to the tip of peritoneovenous shunt without cardiopulmonary bypass in a 25-year-old man. Read More

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http://journals.sagepub.com/doi/10.1177/0218492318776878
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http://dx.doi.org/10.1177/0218492318776878DOI Listing
June 2018
11 Reads

The application value of capsule endoscopy in diagnosing small intestinal carcinoma.

J Cancer Res Ther 2018 Jan;14(1):57-60

Department of Endoscopy, Xinxiang Central Hospital, Henan Province, PR China.

Objective: The aim of this study was to explore the clinical value of capsule endoscopy in the diagnosis of small intestine neoplastic lesions.

Materials And Methods: A retrospective analysis was conducted on the clinical data of 108 patients who underwent capsule endoscopic examination in the Endoscopy Center of Xinxiang Central Hospital from February 2010 to January 2014. The characteristics of different small bowel diseases were observed, and the prevalence rates of different small bowel lesions were calculated. Read More

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http://dx.doi.org/10.4103/jcrt.JCRT_584_17DOI Listing
January 2018
4 Reads

Case report of primary intestinal lymphangiectasia diagnosed in an octogenarian by ileal intubation and by push enteroscopy after missed diagnosis by standard colonoscopy and EGD.

Medicine (Baltimore) 2018 Jan;97(3):e9649

Department of Pathology, William Beaumont Hospital.

Rationale: Primary intestinal lymphangiectasia (PIL) is a rare, presumably congenital lesion that is usually diagnosed in patients < 3 years old, is rarely first diagnosed in adulthood, and when first diagnosed in adulthood typically presents with symptoms for many years. Although PIL is often identified by endoscopic abnormalities, it must be emphasized that the jejunoileum/distal duodenum must be intubated for diagnosis because the lesions are present in these regions. This work demonstrates that 1)-PIL can occur in an octogenarian; 2)-shows that the characteristic endoscopic findings are not found at colonoscopy without terminal ileal intubation; and 3)-may be missed at standard EGD without distal duodenal intubation. Read More

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http://Insights.ovid.com/crossref?an=00005792-201801190-0003
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http://dx.doi.org/10.1097/MD.0000000000009649DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5779771PMC
January 2018
18 Reads

Nutritional therapy and effect assessment of infants with primary intestinal lymphangiectasia: Case reports.

Medicine (Baltimore) 2017 Dec;96(51):e9240

Department of Nuclear Medicine, Beijing Shijitan Hospital, Capital Medical University, Beijing, China.

Rationale: Intestinal lymphangiectasia (IL) is a rare enteropathy involving the expansion and rupture of intestinal lymphatic channels. Although several reports have studied cases of primary IL (PIL), this condition is very rare, and is even less commonly encountered in infants. This study aimed to investigate the nutritional therapy and effect assessment of chylous reflux disorder caused by PIL in infants. Read More

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http://dx.doi.org/10.1097/MD.0000000000009240DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5758182PMC
December 2017
10 Reads

Acute small intestinal inflammation results in persistent lymphatic alterations.

Am J Physiol Gastrointest Liver Physiol 2018 03 14;314(3):G408-G417. Epub 2017 Dec 14.

Inflammation Research Network and Smooth Muscle Research Group, Snyder Institute for Chronic Diseases, Department of Physiology and Pharmacology, Cumming School of Medicine, University of Calgary , Calgary, Alberta , Canada.

Inflammatory bowel disease (IBD) has a complex pathophysiology with limited treatments. Structural and functional changes in the intestinal lymphatic system have been associated with the disease, with increased risk of IBD occurrence linked to a history of acute intestinal injury. To examine the potential role of the lymphatic system in inflammation recurrence, we evaluated morphological and functional changes in mouse mucosal and mesenteric lymphatic vessels, and within the mesenteric lymph nodes during acute ileitis caused by a 7-day treatment with dextran sodium sulfate (DSS). Read More

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http://dx.doi.org/10.1152/ajpgi.00340.2017DOI Listing
March 2018
8 Reads

[Clinical analysis of intestinal lymphangiectasia in 47 children].

Zhonghua Er Ke Za Zhi 2017 Dec;55(12):937-941

Department of Gastroenterology, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.

To analyze the clinical manifestations, diagnosis, treatment and prognosis of intestinal lymphangiectasia (IL) in children in order to improve the skills of diagnosis and treatment of IL. Clinical manifestations, laboratory findings, gastroscopic findings, histopathological examinations and lymphatic radionuclide imaging assessments were analyzed retrospectively among 47 IL patients who were hospitalized in the Gastroenterology Department of Beijing Children's Hospital Affiliated to Capital Medical University from June 2007 to December 2015. All patients were followed up by telephone. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2017.12.014DOI Listing
December 2017
37 Reads

An approach to familial lymphoedema.

Clin Med (Lond) 2017 Dec;17(6):552-557

Department of Clinical Genetics, St Georges Hospital and St George's, University of London, London, UK.

Lymphoedema is the build-up of lymphatic fluid leading to swelling in the tissues. Most commonly it affects the peripheries. Diagnosis is based on clinical assessment and imaging with lymphoscintigraphy. Read More

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http://www.clinmed.rcpjournal.org/lookup/doi/10.7861/clinmed
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http://dx.doi.org/10.7861/clinmedicine.17-6-552DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297692PMC
December 2017
12 Reads

[Primary intestinal lymphangiectasia associated with follicular lymphoma: one case report].

Authors:
Y Y Wu X Lai

Zhonghua Xue Ye Xue Za Zhi 2017 Oct;38(10):900-901

Department of Hematology. The First People's Hospital of Yunnan, Kunming 650032, China.

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2017.10.016DOI Listing
October 2017
1 Read

Correlation between the FCEAI and diagnostic parameters in chronic enteropathies in 147 cats (2006-2012).

Tierarztl Prax Ausg K Kleintiere Heimtiere 2017 Nov 3;45(6). Epub 2017 Nov 3.

Dr. Stefanie Mitze, Tierklinik Haar, Keferloher Strasse 25, 85540 Haar, Email:

Objective: The Feline Chronic Enteropathy Activity Index (FCEAI) has been established as a quantitative index for disease activity in chronic enteropathies in cats. A definite diagnosis is aimed at histology with initial exclusion of extraintestinal causes by laboratory examinations, diagnostic imaging and endoscopy. The study aimed to examine diagnostic parameters and FCEAI in chronic gastroenteropathies. Read More

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http://dx.doi.org/10.15654/TPK-170089DOI Listing
November 2017
21 Reads

Loss of ADAMTS3 activity causes Hennekam lymphangiectasia-lymphedema syndrome 3.

Hum Mol Genet 2017 11;26(21):4095-4104

Human Molecular Genetics, de Duve Institute, University of Louvain, 1200 Brussels, Belgium.

Primary lymphedema is due to developmental and/or functional defects in the lymphatic system. It may affect any part of the body, with predominance for the lower extremities. Twenty-seven genes have already been linked to primary lymphedema, either isolated, or as part of a syndrome. Read More

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http://dx.doi.org/10.1093/hmg/ddx297DOI Listing
November 2017
49 Reads

Primary Intestinal Lymphangiectasia (Waldmann's Disease) Presenting with Chylous Effusions in a 15-Year-Old.

J Clin Diagn Res 2017 Aug 1;11(8):OD17-OD18. Epub 2017 Aug 1.

Professor, Department of Medicine, JIPMER, Puducherry, India.

Primary Intestinal Lymphangiectasia (PIL) is a rare disease of unknown aetiology which presents in the paediatric age group with anasarca, diarrhoea, hypoproteinaemia, lymphoedema and chylous effusions. Tuberculosis, filariasis, chest trauma, malignancies and haematological disorders usually contribute to most cases of secondary lymphangiectasia and chylous effusions. We hereby describe a case of PIL presenting with chylous effusions which remained undiagnosed for eight years. Read More

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http://dx.doi.org/10.7860/JCDR/2017/29055.10522DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5620829PMC
August 2017
18 Reads

[Diagnostic and therapeutic approach to chronic inflammatory enteropathies in dogs].

Tierarztl Prax Ausg K Kleintiere Heimtiere 2017 10 21;45(5):317-327. Epub 2017 Sep 21.

Carolin Erdmann, Klinik für Kleintiere, der Universität Leipzig, An den Tierkliniken 23, 04103 Leipzig, E-Mail:

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http://dx.doi.org/10.15654/TPK-170366DOI Listing
October 2017
39 Reads

[Primary intestinal lymphangiectasia (Waldmann's disease)].

Rev Med Interne 2018 Jul 1;39(7):580-585. Epub 2017 Sep 1.

Service de gastro-entérologie et nutrition, hôpital Saint-Antoine, AP-HP, 184, rue du Faubourg Saint-Antoine, 75571 Paris cedex 12, France.

Primary intestinal lymphangiectasia (PIL), Waldmann's disease, is a rare disorder of unknown etiology characterized by dilated intestinal lacteals leading to lymph leakage into the small-bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. The main symptom is bilateral lower limb edema. Read More

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http://dx.doi.org/10.1016/j.revmed.2017.07.009DOI Listing
July 2018
5 Reads

Exacerbation of primary intestinal lymphangiectasia during late pregnancy and recovery after delivery: A case report and literature review.

Medicine (Baltimore) 2017 Sep;96(35):e7928

Department of Obstetrics, Hangzhou First People's Hospital, Nanjing, Medical University, Hangzhou, Zhejiang Province, People's Republic of China.

Rationale: Primary intestinal lymphangiectasia (PIL) is a rare disease characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen. Main clinical features include intermittent diarrhea, hypoproteinemia. Scattered case reports suggested that PIL is compatible to pregnancy, but with increased complications. Read More

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http://dx.doi.org/10.1097/MD.0000000000007928DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5585511PMC
September 2017
9 Reads

Deterioration of duodenal lymphangiectasia after radiotherapy for gastric MALT lymphoma.

Ecancermedicalscience 2017 11;11:752. Epub 2017 Jul 11.

Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan.

A 68-year-old Japanese woman underwent radiotherapy for gastric lymphoma. Although lymphangiectasia was sparsely observed in the second portion of the duodenum before radiotherapy, the number of pinpoint white spots obviously increased after the treatment. Although the duodenal lymphangiectasia gradually progressed, the patient had no features of protein-losing enteropathy. Read More

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http://dx.doi.org/10.3332/ecancer.2017.752DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5533599PMC
July 2017
7 Reads

Primary intestinal lymphangiectasia in an elderly female patient: A case report on a rare cause of secondary immunodeficiency.

Medicine (Baltimore) 2017 Aug;96(31):e7729

aDivision of Internal Medicine, University Hospital Basel, University of Basel, Basel, Switzerland bMedical Outpatient Department, University Hospital Basel, University of Basel, Basel, Switzerland cDepartment of Gastroenterology and Hepatology, University Hospital Basel, University of Basel, Basel, Switzerland dInstitute of Pathology, University Hospital Basel, Basel, Switzerland.

Protein loss via the gut can be caused by a number of gastrointestinal disorders, among which intestinal lymphangiectasia has been described to not only lead to a loss of proteins but also to a loss of lymphocytes, resembling secondary immunodeficiency. We are reporting on a 75-year-old female patient who came to our hospital because of a minor stroke. She had no history of serious infections. Read More

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http://dx.doi.org/10.1097/MD.0000000000007729DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5626168PMC
August 2017
32 Reads

Cutaneous lymphangiectasia in a patient with primary intestinal lymphangiectasia.

Int J Dermatol 2017 Sep 1;56(9):963-964. Epub 2017 Aug 1.

Division of Dermatology, University Medicine Cluster, National University Health Systems, Singapore, Singapore.

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http://dx.doi.org/10.1111/ijd.13719DOI Listing
September 2017
5 Reads

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states.

Clin Exp Gastroenterol 2017 17;10:147-168. Epub 2017 Jul 17.

Research Service, Veterans Affairs Medical Center, Minneapolis, MN, USA.

Protein losing enteropathy (PLE) has been associated with more than 60 different conditions, including nearly all gastrointestinal diseases (Crohn's disease, celiac, Whipple's, intestinal infections, and so on) and a large number of non-gut conditions (cardiac and liver disease, lupus, sarcoidosis, and so on). This review presents the first attempt to quantitatively understand the magnitude of the PLE in relation to the associated pathology for three different disease categories: 1) increased lymphatic pressure (e.g. Read More

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http://dx.doi.org/10.2147/CEG.S136803DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5522668PMC
July 2017
17 Reads

Newcomers in paediatric GI pathology: childhood enteropathies including very early onset monogenic IBD.

Virchows Arch 2018 Jan 17;472(1):111-123. Epub 2017 Jul 17.

Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, 3401 Civic Center Boulevard, 5 NW26, Philadelphia, PA, 19104, USA.

Childhood enteropathies are a group of diseases causing severe chronic (>2-3 weeks) diarrhoea often starting in the first week of life with the potential for fatal complications for the affected infant. Early identification and accurate classification of childhood enteropathies are, therefore, crucial for making treatment decisions to prevent life-threatening complications. Childhood enteropathies are classified into four groups based on the underlying pathology: (i) conditions related to defective digestion, absorption and transport of nutrients and electrolytes; (ii) disorders related to enterocyte differentiation and polarization; (iii) defects of enteroendocrine cell differentiation; and (iv) disorders associated with defective modulation of intestinal immune response. Read More

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http://dx.doi.org/10.1007/s00428-017-2197-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171108PMC
January 2018
2 Reads

Causes Of Chronic Non-Infectious Diarrhoea In Infants Less Than 6 Months Of Age: Rarely Recognized Entities.

J Ayub Med Coll Abbottabad 2017 Jan-Mar;29(1):78-82

Department of Paediatric Gastroenterology & Hepatology, The Children's Hospital and Institute of Child Health Lahore, Pakistan.

Background: Non-infectious causes of chronic diarrhoea are important and easily missed. The study was done with the objectives to identify different causes of chronic non-infectious diarrhoea in infants less than 6 months of age.

Methods: All patients less than 6 months of age presenting for the first time to a Paediatric Gastroenterology tertiary care centre with a history of chronic diarrhoea and negative stool cultures were enrolled over a period of 8 months. Read More

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April 2019
17 Reads

Dachsous1-Fat4 Signaling Controls Endothelial Cell Polarization During Lymphatic Valve Morphogenesis-Brief Report.

Arterioscler Thromb Vasc Biol 2017 09 13;37(9):1732-1735. Epub 2017 Jul 13.

From the I2MC INSERM UMR 1048, Toulouse Cedex, France (F.P., A.-C.P., B.G.-S., F.T.); Department Craniofacial Development and Stem Cell Biology, King's College London, United Kingdom (T.H., P.F.-W.); Rudbeck Laboratory, Department Immunology, Genetics and Pathology, Uppsala University, Sweden (I.M.-C., T.M.); Department of Molecular Biology, Princeton University, NJ (D.D.); Laboratory for Cell Adhesion and Tissue Patterning, RIKEN Center for Developmental Biology, Kobe, Japan (M.T.); and INSERM, Université de Bordeaux, France (E.G.).

Objective: The purpose of this study was to investigate the role of Fat4 and Dachsous1 signaling in the lymphatic vasculature.

Approach And Results: Phenotypic analysis of the lymphatic vasculature was performed in mice lacking functional or . The overall architecture of lymphatic vasculature is unaltered, yet both genes are specifically required for lymphatic valve morphogenesis. Read More

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http://dx.doi.org/10.1161/ATVBAHA.117.309818DOI Listing
September 2017
18 Reads

In search of albumin: An unusual case of lower limb edema.

Eur J Intern Med 2018 04 8;50:e3-e4. Epub 2017 Jul 8.

AZ Sint-Jan, Department of Gastroenterology, Kaïrostraat 84, 8400 Oostende, Belgium.

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https://linkinghub.elsevier.com/retrieve/pii/S09536205173027
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http://dx.doi.org/10.1016/j.ejim.2017.07.009DOI Listing
April 2018
2 Reads

In vivo characterization of abnormalities in small-bowel diseases using probe-based confocal laser endomicroscopy.

Endosc Int Open 2017 Jul 23;5(7):E547-E558. Epub 2017 Jun 23.

Department of Diagnostic Pathology I, Fujita Health University School of Medicine, Toyoake, Aichi, Japan.

Background And Study Aims:  Probe-based confocal laser endomicroscopy (pCLE) enables real-time optical biopsy. Little is known about pCLE imaging deep inside the small bowel, therefore the aim of this study was to determine its usefulness.

Patients And Methods:  Between April 2014 and January 2016, we performed 38 pCLE examinations during double-balloon enteroscopy with intravenous fluorescein in 37 patients with: tumors (n = 10), vascular disorders (n = 6), inflammatory diseases and drug injuries (n = 13), other disorders (n = 4), and normal findings (n = 4). Read More

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http://dx.doi.org/10.1055/s-0043-106184DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5482742PMC
July 2017
22 Reads

CD55 Deficiency, Early-Onset Protein-Losing Enteropathy, and Thrombosis.

N Engl J Med 2017 07 28;377(1):52-61. Epub 2017 Jun 28.

From the Section of Molecular Development of the Immune System, Laboratory of Immunology (A.O., W.A.C., A.R.M., H.F.M., M.J.L.), the Clinical Genomics Program (A.O., W.A.C., A.R.M., Y.Z., H.F.M., H.C.S., M.J.L.), and the Human Immunological Diseases Section, Laboratory of Host Defenses (Y.Z., H.C.S.), National Institute of Allergy and Infectious Diseases, the Laboratory of Pathology, National Cancer Institute (S.P.), and Radiology and Imaging Sciences, Clinical Center (L.R.F.), National Institutes of Health, Bethesda, MD; the Department of Pediatrics, Division of Allergy and Immunology (A.O., E.K.-A., S.B., A. Kiykim, I.O.), and the Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology, and Nutrition (E.T., D.E.), Marmara University, Jeffrey Modell Diagnostic Center for Primary Immunodeficiency Diseases (A.O., E.K.-A., S.B., A. Kiykim, I.O.), and the Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, İstanbul University Cerrahpaşa Faculty of Medicine (Ö.F.B., T.E.), Istanbul, and the Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Gazi University (B.D., S.S.), the Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Faculty of Medicine, Başkent University (F.O., Z.B., M.G.), and the Pediatric Gastroenterology Clinic, Dr. Sami Ulus Children's Hospital (A.U.A.), Ankara - all in Turkey; Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases and the CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences (R.C.A., C.D.C., N.K.S., A. Krolo, K.B.), Clinical Institute of Pathology (R.K.), the Department of Pediatrics and Adolescent Medicine (K.B.), and St. Anna Kinderspital and Children's Cancer Research Institute, Department of Pediatrics (K.B.), Medical University of Vienna, Vienna; Merck Research Laboratories (J.J.M.), and the Division of Gastroenterology, Hepatology, and Nutrition, Boston Children's Hospital, Harvard Medical School (S.B.S.), Boston; and the Department of Pediatric Gastroenterology, University Medical Center-Wilhelmina Children's Hospital (R.H.J.H.), and the Department of Rheumatology and Clinical Immunology, University Medical Center (H.L.L.), Utrecht, the Netherlands.

Background: Studies of monogenic gastrointestinal diseases have revealed molecular pathways critical to gut homeostasis and enabled the development of targeted therapies.

Methods: We studied 11 patients with abdominal pain and diarrhea caused by early-onset protein-losing enteropathy with primary intestinal lymphangiectasia, edema due to hypoproteinemia, malabsorption, and less frequently, bowel inflammation, recurrent infections, and angiopathic thromboembolic disease; the disorder followed an autosomal recessive pattern of inheritance. Whole-exome sequencing was performed to identify gene variants. Read More

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http://dx.doi.org/10.1056/NEJMoa1615887DOI Listing
July 2017
36 Reads

Application of novel magnified single balloon enteroscopy for a patient with Cronkhite-Canada syndrome.

World J Gastroenterol 2017 Jun;23(22):4121-4126

Masaki Murata, Shigeki Bamba, Kenichiro Takahashi, Hirotsugu Imaeda, Atsushi Nishida, Osamu Inatomi, Akira Andoh, Department of Medicine, Shiga University of Medical Science, Seta-Tsukinowa, Otsu 520-2192, Japan.

We present a case of Cronkhite-Canada syndrome (CCS) in which the entire intestine was observed using a prototype of magnifying single-balloon enteroscope (SIF Y-0007, Olympus). CCS is a rare, non-familial gastrointestinal polyposis with ectodermal abnormalities. To our knowledge, this is the first report showing magnified intestinal lesions of CCS. Read More

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http://dx.doi.org/10.3748/wjg.v23.i22.4121DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5473131PMC
June 2017
8 Reads

Gastrointestinal haemorrhage due to lymphangiectasia caused by protein-losing enteropathy in the Fontan circulation.

Cardiol Young 2017 Oct 27;27(8):1641-1643. Epub 2017 Jun 27.

1Department of Pediatrics,Division of Pediatric Cardiology, Faculty of Medicine,Regional University Hospital Center of Lille,Lille,France.

We report the case of a 14-year-old boy with severe protein-losing enteropathy after Fontan surgery that led to lymphangiectasia, which caused gastrointestinal haemorrhage and required invasive treatment to stop the bleeding. Through this case and a review of the literature on protein-losing enteropathy after Fontan surgery, we highlight a rare and serious presentation of the disease and the difficulties of diagnosis and management. Read More

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http://dx.doi.org/10.1017/S1047951117000853DOI Listing
October 2017
16 Reads

Primary Intestinal Lymphangiectasia as a First Manifestation of Tuberous Sclerosis Complex.

J Pediatr Gastroenterol Nutr 2017 10;65(4):e96

*Victorian Clinical Genetics Services, Murdoch Children's Research Institute †The Royal Children's Hospital, Parkville Victoria ‡Department of Paediatrics, Monash University, Clayton, Victoria Australia.

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http://dx.doi.org/10.1097/MPG.0000000000001660DOI Listing
October 2017
2 Reads

Waldmann's Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect.

J Clin Diagn Res 2017 Apr 1;11(4):SD03-SD04. Epub 2017 Apr 1.

Assistant Professor, Department of Paediatrics, Kasturba Medical College, Manipal, Karnataka, India.

Waldmann's disease or Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of gastrointestinal tract characterized by dilated lymphatics and widened villi causing leakage of lymph into intestinal lumen. Loss of lymph leads to hypoalbuminemia, hyogammaglobulinemia and lymphopenia. Secondary lymphangiectasia occurs secondary to an elevated lymphatic pressure as in lymphoma, systemic lupus erythematosus, constrictive pericarditis, cardiac surgeries (Fontan's procedure), inflammatory bowel disease and malignancies. Read More

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http://dx.doi.org/10.7860/JCDR/2017/24989.9742DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5449870PMC
April 2017
92 Reads