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    1 OF 36

    Transmission electron microscopy helpfulness in Whipple's disease masked by immunosuppressant therapy for arthritis.
    APMIS 2017 Nov 20. Epub 2017 Nov 20.
    Section of Pathology, Department of Emergency and Organ Transplantation, University 'Aldo Moro' of Bari, Bari, Italy.
    A 61-year-old woman received a diagnosis of undifferentiated non-erosive arthritis in 2010 and assumed methotrexate and steroids in 2014. After 1 year, she experienced watery diarrhea, vomiting, fever, weight loss, and severe hypoalbuminemia, thus being admitted into our Unit. Esophagogastroduodenoscopy showed duodenal lymphangiectasia and duodenal biopsy samples several foamy PAS-positive macrophages and villous subtotal atrophy. Read More

    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

    Evaluation of lymphatic vessel dilatations by anterior segment swept-source optical coherence tomography: case report.
    BMC Ophthalmol 2017 Oct 23;17(1):194. Epub 2017 Oct 23.
    Faculty of Medicine Department of Ophthalmology, Semmelweis University, Budapest, Hungary.
    Background: Conjunctival lymphangiectasia is a rare condition presumably caused by the obstruction of lymphatic channels or by an abnormal connection between conjunctival lymphatic and blood vessels. Diagnosis is based on clinical appearance and histology. We report a case of conjunctival lymphangiectasia in which anterior segment optical coherence tomography (OCT) was used to assist the diagnosis and the planning of the biopsy location. Read More

    Loss of ADAMTS3 activity causes Hennekam lymphangiectasia-lymphedema syndrome 3.
    Hum Mol Genet 2017 Nov;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

    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

    Cutaneous alternariosis in an immunocompromised dog successfully treated with cold plasma and cessation of immunosuppressive medication.
    Tierarztl Prax Ausg K Kleintiere Heimtiere 2017 Oct 13;45(5):337-343. Epub 2017 Sep 13.
    Janine Classen, Clinic of Small Animal Medicine, Centre for Clinical Veterinary Medicine, Ludwig-Maximilians University, Veterinärstraße 13, 80539 Munich, Germany, Email:
    A cutaneous infection with Alternaria spp. was diagnosed in a 2-year-old male intact Irish setter dog, presenting with multifocal papules, plaques and ulcerations involving all four distal limbs, shoulder blades, scrotum, pinnae and nasal mucous membranes. The dog had been treated for inflammatory bowel disease and lymphangiectasia with immunosuppressive doses of cyclosporine and prednisolone for approximately 3 months. Read More

    [Primary intestinal lymphangiectasia (Waldmann's disease)].
    Rev Med Interne 2017 Aug 31. Epub 2017 Aug 31.
    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

    Transient Reactive Eruptive Lymphangiectasia Following Partial Vulvectomy for Chronic Acquired Lymphangioma.
    Skinmed 2017 1;15(4):311-313. Epub 2017 Aug 1.
    Division of Dermatologic Surgery and Dermatopathology, Department of Dermatology, Buffalo Medical Group, Buffalo, NY.
    A 64-year-old white woman was originally diagnosed with histiocytic lymphoma in 1977. She had bilateral lymph node biopsies of the groin, chemotherapy, and radiation therapy after her diagnosis had been confirmed pathologically. She was treated with prednisone and vincristine. Read More

    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

    Rapamycin reversal of VEGF-C-driven lymphatic anomalies in the respiratory tract.
    JCI Insight 2017 Aug 17;2(16). Epub 2017 Aug 17.
    Cardiovascular Research Institute, Department of Anatomy, and Helen Diller Family Comprehensive Cancer Center, University of California, San Francisco, San Francisco, California, USA.
    Lymphatic malformations are serious but poorly understood conditions that present therapeutic challenges. The goal of this study was to compare strategies for inducing regression of abnormal lymphatics and explore underlying mechanisms. CCSP-rtTA/tetO-VEGF-C mice, in which doxycycline regulates VEGF-C expression in the airway epithelium, were used as a model of pulmonary lymphangiectasia. Read More

    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

    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

    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

    Congenital Pulmonary Lymphangiectasia: A Disorder not only of Fetoneonates.
    Klin Padiatr 2017 Jul 17;229(4):205-208. Epub 2017 Jul 17.
    Department of Cardiothoracic Surgery, The First Hospital of Putian, Teaching Hospital, Fujian Medical University, Putian, China.
    Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder of the lung, characterized by dilation of pulmonary subpleural, interlobar, perivascular and peribronchial lymphatics. The incidence of CPL among stillborn and neonates was estimated to be <1%. The etiology of CPL is unknown. Read More

    Newcomers in paediatric GI pathology: childhood enteropathies including very early onset monogenic IBD.
    Virchows Arch 2017 Jul 17. 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

    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

    Dachsous1-Fat4 Signaling Controls Endothelial Cell Polarization During Lymphatic Valve Morphogenesis-Brief Report.
    Arterioscler Thromb Vasc Biol 2017 Sep 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 Fat4 or Dachsous1. The overall architecture of lymphatic vasculature is unaltered, yet both genes are specifically required for lymphatic valve morphogenesis. Read More

    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

    A case of protein-losing enteropathy caused by sclerosing mesenteritis diagnosed with capsule endoscopy and double-balloon endoscopy.
    Clin J Gastroenterol 2017 Aug 29;10(4):351-356. Epub 2017 Jun 29.
    Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuoka Shimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan.
    A 75-year-old man presented with abdominal distension, hypoproteinemia, ascites and a 35-mm mass in the small bowel mesentery. Laparotomy was performed, and he was diagnosed with sclerosing mesenteritis. His clinical condition improved, with computed tomography (CT) showing tumor shrinkage and decreasing ascites after administration of prednisolone; however, on drug withdrawal, abdominal fullness recurred and CT revealed an enlarging tumor and increasing ascites. Read More

    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

    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

    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

    Role of CT scan in diagnosis of renal lymphangiectasia: our single-center experience.
    Ren Fail 2017 Nov;39(1):533-539
    c Department of Nephrology , G.R. Doshi and K.M. Mehta Institute of Kidney Diseases and Research Centre (IKDRC) and Dr. H.L. Trivedi Institute of Transplantation Sciences (ITS) , Civil Hospital Campus , Asarwa, Ahmedabad , Gujarat , India.
    Background: Renal lymphangiectasia is rarely reported benign renal disorder of lymphatic malformation. Though found incidentally; it presents with nonspecific symptoms and shows characteristic findings in radiological imaging studies.

    Aim: Here, we report eight patients with symptoms, laboratory and imaging findings compatible with renal lymphangiectasia. Read More

    Gottron Papules Show Histopathologic Features of Localized Lymphedema.
    Am J Dermatopathol 2017 Jul;39(7):518-523
    *Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain; and †Department of Pathology, Southern California Kaiser Permanente, Los Angeles Medical Center (LAMC), Kaiser Permanente, Los Angeles, CA.
    Although Gottron papules are a relevant feature of dermatomyositis, they are reported in very few papers in the literature, and the largest series on the subject includes 11 cases. We intend to study the main histopathologic features of Gottron papules in a series of 12 biopsies. We evaluated hematoxylin-eosin stains on all cases. Read More

    Diagnosis of secondary pulmonary lymphangiectasia in congenital heart disease: a novel role for chest ultrasound and prognostic implications.
    Pediatr Radiol 2017 Oct 19;47(11):1441-1451. Epub 2017 Jun 19.
    Department of Diagnostic Imaging, The Hospital for Sick Children, Department of Medical Imaging, University of Toronto, 555 University Ave., M644, Toronto, ON, M5G 1X8, Canada.
    Background: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction.

    Objectives: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia.

    Materials And Methods: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Read More

    Management of refractory chylous ascites with peritoneovenous shunts.
    J Vasc Surg Venous Lymphat Disord 2017 Jul 9;5(4):538-546. Epub 2017 May 9.
    Division of Vascular and Endovascular Surgery, Mayo Clinic, Rochester, Minn.
    Objective: The purpose of this study was to define outcome of treatments of refractory chylous ascites using peritoneovenous shunts (PVSs).

    Methods: Clinical data of patients with refractory chylous ascites treated with PVSs between 1992 and 2015 were retrospectively reviewed. The primary end point was clinical benefit, defined as cured, improved, or poor results; secondary end points were complications and reinterventions. Read More

    Renal lymphangiectasia treated with percutaneous drainage and sclerotherapy.
    Int J Adolesc Med Health 2017 Jun 9. Epub 2017 Jun 9.
    .
    Renal lymphangiectasia is a rare benign condition of the lymphatic system. Primarily, the diagnosis is based upon characteristic imaging findings along with biochemical analysis of aspirated fluid. No definitive algorithm exists for treating this condition owing to its rarity. Read More

    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

    Conjunctival lymphangiectasia associated with classic Fabry disease.
    Br J Ophthalmol 2017 May 12. Epub 2017 May 12.
    Department of Optometry and Vision Science, University of Alabama at Birmingham, Birmingham, Alabama, USA.
    Background: Fabry disease (FD) is a treatable multisystem disease caused by a defect in the alpha-galactosidase gene. Ocular signs of FD, including corneal verticillata, are among the earliest diagnostic findings. Conjunctival lymphangiectasia (CL) has not previously been associated with FD. Read More

    A primary intestinal lymphangiectasia hiding the diagnosis of pleural and pericardial tuberculosis: a clinical observation.
    Pan Afr Med J 2017 23;26:89. Epub 2017 Feb 23.
    Tuberculosis Department, Moulay Youssef University Hospital, Rabat, Maroc.
    Primary intestinal lymphangiectasia (Waldmann's disease) is an exudative enteropathy characterized by lymph leakage into the small bowel lumen leading to hypoalbuminemia, hypogammaglobulinemia and lymphopenia (particularly T-cell). The diagnosis is based on viewing the duodenal lymphangiectasia. A 20 years old female patient, treated for a primary intestinal lymphangiectasia, has consulted for anasarca. Read More

    Neuron-specific knockdown of the Drosophila fat induces reduction of life span, deficient locomotive ability, shortening of motoneuron terminal branches and defects in axonal targeting.
    Genes Cells 2017 Jul 9;22(7):662-669. Epub 2017 May 9.
    Department of Applied Biology, Kyoto Institute of Technology, Matsugasaki, Sakyo-ku, Kyoto, 606-8585, Japan.
    Mutations in FAT4 gene, one of the human FAT family genes, have been identified in Van Maldergem syndrome (VMS) and Hennekam lymphangiectasia-lymphedema syndrome (HS). The FAT4 gene encodes a large protein with extracellular cadherin repeats, EGF-like domains and Laminin G-like domains. FAT4 plays a role in tumor suppression and planar cell polarity. Read More

    Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases.
    Intern Med 2017 15;56(8):943-948. Epub 2017 Apr 15.
    Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, Japan.
    This report describes two patients with sporadic lymphangioleiomyomatosis complicated by protein-losing enteropathy (PLE). Imaging studies indicated retroperitoneal lymphangioleiomyomas and abnormalities of the adjacent digestive tract. Endoscopic mucosal biopsy revealed colonic lymphangiectasia in one patient; whereas the site in the other patient was intestinal. Read More

    Waldmann's disease: a rare cause of protein losing enteropathy in an adult patient.
    Rev Esp Enferm Dig 2017 May;109(5):385-388
    Centre Hospitalier Universitaire de Dijon.
    Primary intestinal lymphangiectasia or Waldmann's disease is an uncommon cause of protein losing enteropathy with an unknown etiology and is usually diagnosed during childhood. It is characterized by dilation and leakage of intestinal lymph vessels leading to hypoalbuminemia, hypogammaglobulinemia and lymphopenia. Differential diagnosis should include erosive and non-erosive gastrointestinal disorders, conditions involving mesenteric lymphatic obstruction and cardiovascular disorders that increase central venous pressure. Read More

    Lymphatic deletion of calcitonin receptor-like receptor exacerbates intestinal inflammation.
    JCI Insight 2017 Mar 23;2(6):e92465. Epub 2017 Mar 23.
    Department of Cell Biology and Physiology, University of North Carolina, Chapel Hill, North Carolina, USA.
    Lymphatics play a critical role in maintaining gastrointestinal homeostasis and in the absorption of dietary lipids, yet their roles in intestinal inflammation remain elusive. Given the increasing prevalence of inflammatory bowel disease, we investigated whether lymphatic vessels contribute to, or may be causative of, disease progression. We generated a mouse model with temporal and spatial deletion of the key lymphangiogenic receptor for the adrenomedullin peptide, calcitonin receptor-like receptor (Calcrl), and found that the loss of lymphatic Calcrl was sufficient to induce intestinal lymphangiectasia, characterized by dilated lacteals and protein-losing enteropathy. Read More

    Primary intestinal lymphangiectasia: Multiple detector computed tomography findings after direct lymphangiography.
    J Med Imaging Radiat Oncol 2017 Oct 27;61(5):607-613. Epub 2017 Mar 27.
    Department of CT, Beijing Shijitan Hospital, Capital Medical University, Haidian District, Beijing, China.
    Introduction: To analyse the findings of multiple detector computed tomography (MDCT) after direct lymphangiography in primary intestinal lymphangiectasia (PIL).

    Methods: Fifty-five patients with PIL were retrospectively reviewed. All patients underwent MDCT after direct lymphangiography. Read More

    Eculizumab in secondary atypical haemolytic uraemic syndrome.
    Nephrol Dial Transplant 2017 03;32(3):466-474
    Department of Nephrology, Instituto de Investigación Hospital 12 de Octubre (imas12), Madrid, Spain.
    Background: Complement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab.

    Methods: We identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Read More

    Congenital pulmonary lymphangiectasia and early mortality after stage 1 reconstruction procedures.
    Cardiol Young 2017 Sep 13;27(7):1356-1360. Epub 2017 Mar 13.
    4Department of Pathology and Laboratory Medicine,The Children's Hospital of Philadelphia,Philadelphia,Pennsylvania,United States of America.
    Objectives: Pulmonary lymphangiectasia associated with hypoplastic left heart syndrome with an intact or restrictive atrial septum may result from increased left atrial pressure, and is associated with worse outcomes following staged reconstruction due to lung dysfunction and significant hypoxaemia. Our objective was to characterise the incidence of pulmonary lymphangiectasia in cases of early mortality following stage 1 reconstructions.

    Methods: An institutional cardiac surgical database was retrospectively searched for patients who died within 30 days following a stage 1 reconstruction between 1 January, 1984 and 31 December, 2013. Read More

    Unsupervised abnormality detection using saliency and Retinex based color enhancement.
    Conf Proc IEEE Eng Med Biol Soc 2016 Aug;2016:3871-3874
    An efficient and automated abnormality detection method can significantly reduce the burden of screening of the enormous visual information resulting from capsule endoscopic procedure. As a pre-processing stage, color enhancement could be useful to improve the image quality and the detection performance. Therefore, in this paper, we have proposed a two-stage automated abnormality detection algorithm. Read More

    TNFΔARE Mice Display Abnormal Lymphatics and Develop Tertiary Lymphoid Organs in the Mesentery.
    Am J Pathol 2017 Apr 6;187(4):798-807. Epub 2017 Feb 6.
    Inflammation Research Network and Smooth Muscle Research Group, Department of Physiology & Pharmacology, Snyder Institute for Chronic Diseases, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada. Electronic address:
    Chronic inflammatory diseases are associated with a persistent and enhanced response to environmental antigens. As an adaptive response to this exaggerated immune state, affected tissue typically develops tertiary lymphoid organs. Studies of Crohn disease (CD), a chronic inflammatory disease of the intestinal tract, report tertiary lymphoid organs present within the mucosal wall, along with other lymphatic diseases, such as lymphangiogenesis and obstructed lymphatic vessels. Read More

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