scholarly journals Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge

2018 ◽  
Vol 75 (1) ◽  
pp. 95-99
Author(s):  
Tatiana Jocic ◽  
Olgica Latinovic-Bosnjak ◽  
Mirjana Zivojinov ◽  
Mirjana Stojsic ◽  
Jelena Ilic-Sabo
Keyword(s):  
Small Intestine ◽  
Celiac Disease ◽  
Plasma Proteins ◽  
Histological Analysis ◽  
Inflammatory Diseases ◽  
Lymphatic Vessels ◽  
Intestinal Lymphangiectasia ◽  
Double Balloon Enteroscopy ◽  
Endoscopy Capsule ◽  
Secondary Causes

Introduction. Primary intestinal lymphangiectasia is a rare disorder, characterized by abnormal dilation of intestinal lymphatic vessels and extensive enteric loss of lymph rich in plasma proteins, lymphocytes and chylomicrons. The main characteristics of the disease are hypoalbuminemia, hypogammaglobulinemia, lymphocytopenia, and more rarely, the deficit of liposoluble vitamins and anemia. Except for primary, there are secondary lymphangiectasia, associated with celiac disease, malignant, infective and inflammatory diseases of the small intestine, fibrosis, liver and cardiovascular diseases. Case report. A male, 33 years of age, presented for his medical examination suffering from diarrhea and edema. The diagnosis was established upon the histological examination of a small intestine biopsy during double balloon enteroscopy, which revealed changes only in one segment of the intestine examined. Such a finding was later confirmed by the video endoscopy capsule. Conclusion. The diagnosis of intestinal lymphangiectasia is usually established before the age of 3, but it can also be diagnosed in adults. The diagnosis is based on the histological analysis of the intestinal mucosa biopsy, obtained by endoscopic procedures. The diagnosis of primary intestinal lymphangiectasia is also made upon the exclusion of secondary causes.

scholarly journals Wait-and-See Approach or Gluten-Free Diet Administration—The Rational Management of Potential Coeliac Disease

Nutrients ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 947
Author(s):  
Anna Szaflarska-Popławska
Keyword(s):  
Small Intestine ◽  
Celiac Disease ◽  
Prognostic Factors ◽  
Coeliac Disease ◽  
Gluten Free Diet ◽  
Gluten Free ◽  
Individual Basis ◽  
Therapeutic Decisions ◽  
Rational Management ◽  
Wait And See

Potential celiac disease (PCD) is a heterogeneous disease; only some patients develop full celiac disease (CD), characterised by advanced atrophic changes in the small intestine. Few accurate prognostic factors exist for the progression of PCD; therefore, therapeutic decisions should be made on an individual basis in each case. Patients with clinical gastroenterological or parenteral symptoms often benefit from a gluten-free diet, and those left on a diet containing gluten should receive clinical, serological and histopathological supervision.

scholarly journals Congenital intestinal lymphangiectasia

2011 ◽  
Vol 68 (3) ◽  
pp. 270-273
Author(s):  
Dusan Popovic ◽  
Milan Spuran ◽  
Tamara Alempijevic ◽  
Miodrag Krstic ◽  
Srdjan Djuranovic ◽  
...  
Keyword(s):  
Early Recognition ◽  
Clinical Manifestations ◽  
Lymphatic Vessels ◽  
High Protein Diet ◽  
Intestinal Wall ◽  
Intestinal Biopsy ◽  
Intestinal Lymphangiectasia ◽  
Laboratory Assessment ◽  
Protein Losing Enteropathy ◽  
Adequate Treatment

Background. Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. Case report. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and suportive therapy. Conclusion. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

An Endoscopic Analysis of Ulcers of Small Intestine Detected By Double Balloon Enteroscopy

2009 ◽  
Vol 69 (5) ◽  
pp. AB203
Author(s):  
Tae Hee Lee ◽  
Jin-Oh Kim ◽  
Wan Jung Kim ◽  
Hyun Gun Kim ◽  
Won Young Cho ◽  
...  
Keyword(s):  
Small Intestine ◽  
Double Balloon Enteroscopy ◽  
Balloon Enteroscopy ◽  
Double Balloon

Lymphangiogenesis in development and disease

2007 ◽  
Vol 98 (08) ◽  
pp. 304-310 ◽  
Author(s):  
Ruediger Liersch ◽  
Michael Detmar
Keyword(s):  
Lymph Nodes ◽  
Cancer Metastasis ◽  
Lymphatic System ◽  
Vascular System ◽  
Malignant Tumors ◽  
Inflammatory Diseases ◽  
Transplant Rejection ◽  
Lymphatic Vessels ◽  
Lipid Uptake ◽  
Molecular Control

SummaryThe lymphatic vascular system plays an important role in the maintenance of fluid homeostasis, in the afferent immune response, in the intestinal lipid uptake and in the metastatic spread of malignant cells. The recent discovery of specific markers and growth factors for lymphatic endothelium and the establishment of genetic mouse models with impairment of lymphatic function have provided novel insights into the molecular control of the lymphatic system in physiology and in embryonic development. They have also identified molecular pathways whose mutational inactivation leads to human diseases associated with lymphedema. Moreover, the lymphatic system plays a major role in chronic inflammatory diseases and in transplant rejection. Importantly, malignant tumors can directly promote lymphangiogenesis within the primary tumor and in draining lymph nodes, leading to enhanced cancer metastasis to lymph nodes and beyond. Based upon these findings, novel therapeutic strategies are currently being developed that aim at inhibiting or promoting the formation and function of lymphatic vessels in disease.

Abstract 563: Mechanisms of VEGFR3-Dependent Pathological Lymphangiogenesis

2014 ◽  
Vol 34 (suppl_1) ◽  
Author(s):  
Wang Min
Keyword(s):  
Inflammatory Diseases ◽  
Active Form ◽  
Lymphatic Vessels ◽  
Master Regulators ◽  
Downstream Signaling ◽  
Neonatal Skin ◽  
Genetic Deletion ◽  
Vasculogenesis And Angiogenesis ◽  
Intracellular Mediators

Vascular remodeling, i.e., the growth and/or enlargement of both blood and lymphatic vessels, is tightly associated with inflammatory diseases, such as chronic airway inflammation, rheumatoid arthritis, inflammatory bowl disease, and chronic inflammatory skin psoriasis. The lymphatic system collects extravasated fluid, macromolecules, and immune cells from tissues and returns them to the blood circulation. While VEGFR2 and its ligand VEGF-A functions as master regulators of vasculogenesis and angiogenesis, VEGFR3 is critical for lymphangiogenesis. These conclusions are based on observations in genetically modified mice for VEGFR3 and its ligands. VEGFR3 is expressed on the blood and lymphatic endothelium during development, and is restricted to the lymphatics in the adult. Extensive studies have thus far focused on the role of VEGFR3 in lymphangiogenesis during development, however the function, regulation and intracellular mediators of the VEGFR3-dependent pathways in pathologic formation of lymphatic vessels during diseases remain poorly characterized. Our recent studies have identified two critical intracellular mediators, AIP1 and Bmx, in regulating VEGFR3 expression and activity. Genetic deletion of AIP1, a Ras-GAP protein, delays developmental lymphangiogenesis in neonatal skin and mesentery, and mounts weaker VEGF-C-induced cornea lymphangiogenesis. Deletion of Bmx, a non-receptor tyrosine kinase, has no effect on developmental lymphangiogenesis but blunts pathological lymphangiogenesis in mouse models. Mechanistically, AIP1 and Bmx sequentially regulate different steps during the VEGFR3 activation. AIP1 constitutively binds to VEGFR3 and enhances VEGFR3 endocytosis and stability. Bmx is not expressed in developmental lymphatics but is induced in response to pathological stimuli such as ischemia, and associates with an active form of VEGFR3 to regulate its downstream signaling without an effect on receptor phosphorylation. I will report the most recent progress as to how AIP1 and Bmx coordinately regulate VEGFR3-dependent lymphangiogenesis.

scholarly journals Molecular Biomarkers for Celiac Disease: Past, Present and Future

2020 ◽  
Vol 21 (22) ◽  
pp. 8528
Author(s):  
Aarón D. Ramírez-Sánchez ◽  
Ineke L. Tan ◽  
B.C. Gonera-de Jong ◽  
Marijn C. Visschedijk ◽  
Iris Jonkers ◽  
...  
Keyword(s):  
Small Intestine ◽  
Celiac Disease ◽  
Early Stage ◽  
Villous Atrophy ◽  
Upper Endoscopy ◽  
Gluten Free ◽  
Non Invasive ◽  
Immune Mediated ◽  
Gastrointestinal Complaints ◽  
Small Intestinal

Celiac disease (CeD) is a complex immune-mediated disorder that is triggered by dietary gluten in genetically predisposed individuals. CeD is characterized by inflammation and villous atrophy of the small intestine, which can lead to gastrointestinal complaints, malnutrition, and malignancies. Currently, diagnosis of CeD relies on serology (antibodies against transglutaminase and endomysium) and small-intestinal biopsies. Since small-intestinal biopsies require invasive upper-endoscopy, and serology cannot predict CeD in an early stage or be used for monitoring disease after initiation of a gluten-free diet, the search for non-invasive biomarkers is ongoing. Here, we summarize current and up-and-coming non-invasive biomarkers that may be able to predict, diagnose, and monitor the progression of CeD. We further discuss how current and emerging techniques, such as (single-cell) transcriptomics and genomics, can be used to uncover the pathophysiology of CeD and identify non-invasive biomarkers.

scholarly journals IL-33 Alarmin and Its Active Proinflammatory Fragments Are Released in Small Intestine in Celiac Disease

2020 ◽  
Vol 11 ◽  
Author(s):  
Federico Perez ◽  
Carolina N. Ruera ◽  
Emanuel Miculan ◽  
Paula Carasi ◽  
Karen Dubois-Camacho ◽  
...  
Keyword(s):  
Small Intestine ◽  
Celiac Disease

The Value of Double-Balloon Enteroscopy in Patients With Refractory Celiac Disease

2007 ◽  
Vol 102 (5) ◽  
pp. 987-996 ◽  
Author(s):  
Muhammed Hadithi ◽  
Abdulbaqi Al-toma ◽  
Joost Oudejans ◽  
Adriaan A van Bodegraven ◽  
Chris J. Mulder ◽  
...  
Keyword(s):  
Celiac Disease ◽  
Double Balloon Enteroscopy ◽  
Balloon Enteroscopy ◽  
Refractory Celiac Disease ◽  
Double Balloon

Long-term and acute effects of gliadin on small intestine of patients on potentially pathogenic networks in celiac disease

Autoimmunity ◽  
2009 ◽  
Vol 43 (2) ◽  
pp. 131-139 ◽  
Author(s):  
Ainara Castellanos-Rubio ◽  
Izortze Santin ◽  
Ainhoa Martin-Pagola ◽  
Iñaki Irastorza ◽  
Luis Castaño ◽  
...  
Keyword(s):  
Small Intestine ◽  
Celiac Disease ◽  
Acute Effects

scholarly journals Gastrointestinal Cryptococcosis Associated with Intestinal Lymphangiectasia

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Fernando Naranjo-Saltos ◽  
Alejandro Hallo ◽  
Carlos Hallo ◽  
Andres Mayancela ◽  
Alejandra Rojas
Keyword(s):  
Opportunistic Infections ◽  
Lymphatic Vessels ◽  
Immunocompetent Patient ◽  
Intestinal Lumen ◽  
Histopathological Study ◽  
Intestinal Lymphangiectasia ◽  
Protein Losing Enteropathy ◽  
Unusual Association ◽  
Intestinal Involvement ◽  
Cellular Components

Intestinal lymphangiectasia is a pathological dilation of enteric lymphatic vessels resulting in lymph leakage to the intestinal lumen. This chronic lymph leakage leads to a state of immunosuppression secondary to the loss of humoral and cellular components of the immune system and represents a potential risk factor for opportunistic infections. We report a case of protein-losing enteropathy in a seemingly immunocompetent patient. An intestinal histopathological study revealed the unusual association of lymphangiectasia and intestinal cryptococcosis. Although cryptococcal infection is common in immunocompromised patients, intestinal involvement is rarely reported. We found no reports on the association of intestinal cryptococcosis in patients with lymphangiectasia. This case report is the first to describe intestinal cryptococcosis associated with intestinal lymphangiectasia.

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