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    Successful management of extremely high-output refractory congenital chylothorax with chemical pleurodesis using 4% povidone-iodine and propranolol: a case report.
    Clin Case Rep 2018 Apr 27;6(4):702-708. Epub 2018 Feb 27.
    PediatricsUniversity of Nebraska Medical CenterOmahaNebraska.
    First-line therapy for congenital chylothorax is conservative treatment. However, surgical intervention or chemical pleurodesis is required for refractory cases. With all the concerns regarding its complications, povidone-iodin provided a successful management for a high-output congenital chylothorax. Read More

    [The colonoscopic characteristics of colorectal endometriosis: a single-centered retrospective study].
    Zhonghua Nei Ke Za Zhi 2018 Apr;57(4):275-278
    Department of Gastroenterology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medicine Sciences, Beijing 100730, China.
    To reinforce the awareness of colorectal endometriosis (EM) in colonoscopy examination. Patients diagnosed as colorectal EM at Peking Union Medical College Hospital between February 2002 and February 2017 were enrolled in this study. The clinical characteristics and endoscopic features of EM lesions were summarized and compared between pathologically positive group and negative group. Read More

    Hennekam Syndrome: A Case Report.
    Ann Rehabil Med 2018 Feb 28;42(1):184-188. Epub 2018 Feb 28.
    Department of Rehabilitation Medicine, Hanyang University College of Medicine, Seoul, Korea.
    Hennekam syndrome is a rare autosomal recessive disorder resulting from malformation of the lymphatic system. The characteristic signs of Hennekam syndrome are lymphangiectasia, lymph edema, facial anomalies, and mental retardation. This is a case in which a patient presented with left-arm lymphedema, facial-feature anomalies, and multiple organ lymphangiectasia consistent with symptoms of Hennekam syndrome. Read More

    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

    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

    Anterior segment and external ocular disorders associated with HIV infections in the era of HAART in Chiang Mai University Hospital, a prospective descriptive cross sectional study.
    PLoS One 2018 21;13(2):e0193161. Epub 2018 Feb 21.
    Ophthalmology department, Chiang Mai University Hospital, Chiang Mai, Thailand.
    Human immunodeficiency virus (HIV) causes impairment to the human immune system which leads to immunocompromised conditions, including ocular complications. Several important HIV-associated disorders may involve the anterior segment, ocular surface, and adnexae organ such as dry eye, blepharitis which reduce quality of life of patients. In present, potent antiretroviral therapies HAART (highly active antiretroviral therapy) has improved the length and quality of life which may lead to an increased prevalence of anterior segment ocular disorders. Read More

    Hyperplasia of Lymphoid Follicles and Lymphangiectasia in the Parietal Pleura in Bucillamine-induced Yellow Nail Syndrome.
    Intern Med 2018 Feb 9. Epub 2018 Feb 9.
    Department of Pulmonary Medicine and Clinical Immunology, Dokkyo Medical University, Japan.
    Yellow nail syndrome (YNS) pleurisy is often difficult to control, and pathological examinations have rarely been reported. We herein report a case of bucillamine-induced YNS in which histopathology of the parietal pleura revealed hyperplasia of the lymphoid follicles and lymphangiectasia. Even after the discontinuation of bucillamine, the pleurisy and lymphedema showed no change. Read More

    Unilateral pedal lymphangiography plus computed tomography angiography for location of persistent idiopathic chyle leakage not detectable by ordinary contrast computed tomography.
    BMC Urol 2018 Feb 6;18(1). Epub 2018 Feb 6.
    Department of Urology, Shanghai Jiao Tong University Medical School Affiliated Ruijin Hospital, 197 Ruijin Er Road, Shanghai, 200025, People's Republic of China.
    Background: To identify the value of unilateral pedal lymphangiography (LPG) plus computed tomography angiography (CTA) in accurate depiction of persistent idiopathic chyluria undetectable by ordinary contrast CT.

    Methods: Eighteen patients 44-63 years of age with persistent idiopathic chyluria who failed conservative management were included. Ordinary CT had not revealed a chyle leak. Read More

    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

    Surgical Management of Chyloptysis.
    Ann Thorac Surg 2018 Feb;105(2):e79-e81
    Division of Thoracic Surgery, Mayo Clinic, Rochester, Minnesota.
    Chyloptysis is a rare clinical presentation. Diagnosis is challenging and requires recognition of milky-sputum or bronchial casts. We describe a case of chyloptysis secondary to thoracic lymphangiectasia that necessitated surgical ligation of the main thoracic duct and accessory branches. Read More

    Acute small intestinal inflammation results in persistent lymphatic alterations.
    Am J Physiol Gastrointest Liver Physiol 2018 Mar 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

    [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

    Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue: A rare case report.
    Medicine (Baltimore) 2017 Dec;96(49):e9032
    aDepartment of ENT, Children's Medical Center, Shanghai Jiao Tong University, ShanghaibDepartment of Pathology, Shanghai Tongji Hospital, Tongji Hospital Affiliated to Tongji University, ShanghaicDepartment of Pathology, Chongqing Medical University, ChongqingdDepartment of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
    Background: Diffuse pulmonary lymphangiomatosis (DPL) mainly affects the lung and pleura. There are very few pathological reports of lung damage accompanied by diffuse involvement of the extrapulmonary lymph nodes and surrounding soft tissue. The clinicopathological significance of coexistence of pulmonary and extrapulmonary lesions is unknown. Read More

    Transmission electron microscopy helpfulness in Whipple's disease masked by immunosuppressant therapy for arthritis.
    APMIS 2018 Jan 20;126(1):92-96. 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

    Lymphangiectatic Variant of Eccrine Spiradenoma - A Diagnostic Challenge.
    S D Med 2016 Aug;69(8):359-361
    Sanford Pathology Clinic, Sioux Falls, South Dakota.
    Conventional eccrine spiradenoma is a benign, slow growing and painful tumor of the skin. While the tumor does not usually present a diagnostic dilemma, a rare variant with marked stromal lymphedema can be a challenge to interpret. We present a case of lymphangiectatic variant of eccrine spiradenoma in an 82-year-old white male who presented with a persistent left flank lesion for several months. 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 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

    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 or . 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

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