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    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

    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 Jun 27:1-3. 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 Jun 19. 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

    Primary Intestinal Lymphangiectasia as a First Manifestation of Tuberous Sclerosis Complex.
    J Pediatr Gastroenterol Nutr 2017 Jun 13. Epub 2017 Jun 13.
    *Victorian Clinical Genetics Services, Murdoch Children's Research Institute, Parkville Victoria Australia †The Royal Children's Hospital, Parkville Victoria Australia ‡Department of Paediatrics, Monash University, Clayton Victoria Australia.

    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 Mar 27. 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 Mar 13:1-5. 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

    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

    A Quick Reference on Hypocalcemia.
    Vet Clin North Am Small Anim Pract 2017 Mar 22;47(2):249-256. Epub 2016 Dec 22.
    The Ohio State University, 317 West 6th Avenue, Columbus, OH 43201, USA. Electronic address:
    Primary hypoparathyroidism should be considered in dogs with vague signs, including tremors, facial rubbing, and seizures. Ionized hypocalcemia should be considered in dogs with protein-losing enteropathy, especially lymphangiectasia caused by hypovitaminosis D. Ionized hypocalcemia typically occurs only in advanced chronic kidney disease. Read More

    Histopathology of Conjunctivochalasis Compared to Normal Conjunctiva.
    J Ophthalmic Vis Res 2016 Oct-Dec;11(4):345-349
    Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Massachusetts, Boston, USA.
    Purpose: To evaluate the histopathologic changes in the conjunctiva of patients with conjunctivochalasis (CCh) compared to age-matched controls.

    Methods: This cross-sectional, controlled study included 27 eyes of 27 patients with CCh and 16 eyes of 16 age-matched controls. A biopsy of the bulbar conjunctiva was performed along the temporal lower lid margin before cataract surgery in both groups. Read More

    Interventional Treatment of Pulmonary Lymphatic Anomalies.
    Tech Vasc Interv Radiol 2016 Dec 8;19(4):299-304. Epub 2016 Oct 8.
    CHOP/HUP Center for Lymphatic Imaging and Interventions, Children's Hospital of Philadelphia, Penn Medicine: Hospital of University of Pennsylvania, Philadelphia, PA. Electronic address:
    Pulmonary lymphatic diseases have been recognized for many years and have been referred as pulmonary lymphangiectasia, pulmonary lymphangiomatosis, plastic bronchitis, and idiopathic chylothorax or chylopericardium. The lymphatic etiology of these conditions has been determined by detection of cystic lymphatic structures on biopsy or postmortem examination. Development of new imaging techniques such as dynamic contrast-enhanced magnetic resonance lymphangiography has allowed better understanding of pathophysiology of these conditions. Read More

    Deep Vein Thrombosis as Initial Manifestation of Whipple Disease.
    Case Rep Gastroenterol 2016 Sep-Dec;10(3):640-645. Epub 2016 Nov 7.
    Universidade Federal da Paraíba, João Pessoa, Brazil.
    Introduction: Wipple disease (WD) is a rare chronic disease caused by the bacillus Tropheryma whipplei. Constitutive, rheumatologic, gastrointestinal, cardiac, cerebral, lymphatic, cutaneous, and ophthalmological signs are possible systemic symptoms. However, thrombotic manifestations are rarely described as "stroke-like syndrome" or arterial thrombosis. Read More

    Point-of-Care Thoracic Ultrasonography in the Diagnosis and Management of Kaposiform Lymphangiomatosis.
    Pediatr Emerg Care 2016 Dec;32(12):888-891
    From the *Pediatric Residency Program and †Division of Emergency Medicine and Transport, Children's Hospital Los Angeles, Los Angeles, CA.
    Kaposiform lymphangiomatosis is a generalized lymphatic disorder complicated by consumptive coagulopathy and pericardial and pleural effusions. We present the case of a 13-year-old female adolescent given a diagnosis of a large pleural effusion by point-of-care thoracic ultrasonography, which led to further evaluation and diagnosis of this rare disorder. We review the use of point-of-care thoracic ultrasonography for the diagnosis of pleural effusion. Read More

    Marginal Zone Lymphoma Complicated by Protein Losing Enteropathy.
    Case Rep Hematol 2016 9;2016:9351408. Epub 2016 Nov 9.
    Department of Gastroenterology and Hepatology, University Hospital Zurich, Zurich University, Zurich, Switzerland.
    Protein losing enteropathy (PLE) refers to excessive intestinal protein loss, resulting in hypoalbuminemia. Underlying pathologies include conditions leading to either reduced intestinal barrier or lymphatic congestion. We describe the case of a patient with long-lasting diffuse abdominal problems and PLE. Read More

    [Nephrotic syndrome complicated by chylous ascites in a girl of 2 years and 8 months old].
    Arch Pediatr 2017 Jan 21;24(1):24-27. Epub 2016 Nov 21.
    Service de pédiatrie nourrissons, CHU de Brazzaville, Brazzaville, Congo; Faculté des sciences de la santé, université Marien Ngouabi, Brazzaville, Congo.
    We report on a case of nephrotic syndrome with focal and segmental hyalinosis complicated by chylous ascites in a girl of 2 years and 8 months old. This pure nephrotic syndrome in its early stage was initially treated with intensive steroid treatment at 2mg/kg/day orally for 2 months, followed by a bolus of methylprednisolone. The persistence of proteinuria meant corticosteroid resistance. Read More

    Young patient with generalized lymphangiomatosis: Differentiating the differential.
    Indian J Radiol Imaging 2016 Jul-Sep;26(3):411-415
    Department of Radiotherapy, Christian Medical College, Vellore, Tamil Nadu, India.
    We present the case of a 19-year-old man who was extensively evaluated in multiple centres for long-standing cough, dyspnea, and hemoptysis without a definitive diagnosis. His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. Computed tomography of the thorax showed a confluent, fluid-density mediastinal lesion enveloping the mediastinal viscera without any mass effect. Read More

    Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography.
    AJR Am J Roentgenol 2017 Feb 15;208(2):300-305. Epub 2016 Nov 15.
    1 Department of CT, Beijing Shijitan Hospital, Capital Medical University, Yangfangdian Tieyiyuan Rd No. 10, Haidian District, Beijing 100038, China.
    Objective: The aim of this study was to analyze the findings of MDCT performed after direct lymphangiography in patients with diffuse pulmonary lymphangiomatosis.

    Materials And Methods: Twenty-three patients (13 male and 10 female patients) diagnosed with diffuse pulmonary lymphangiomatosis on the basis of clinical features and findings from imaging, bronchoscopy, and pathologic analysis were retrospectively evaluated. All patients underwent pulmonary MDCT after direct lymphangiography, surgical operation or open lung biopsy, and histopathologic examination. Read More

    Bilateral Renal Lymphangiectasia.
    J Clin Diagn Res 2016 Sep 1;10(9):TD01-TD02. Epub 2016 Sep 1.
    Professor, Department of Nephrology, G.R. Doshi and K.M. Mehta Institute of Kidney Diseases & Research Centre (IKDRC) & Dr.H.L. Trivedi Institute of Transplantation Sciences (ITS) , Gujarat, India .
    Renal Lymphangiectasia (RLM) is very rare benign lymphatic malformation. It can be misdiagnosed for other cystic renal masses, most commonly polycystic kidneys. Though incidentally found in most cases, it may be the cause for hypertension and renal failure in undiagnosed patients. Read More

    Gastrointestinal Lymphorrhea Diagnosed by Exercise Lymphoscintigraphy.
    Clin Nucl Med 2016 Dec;41(12):e520-e521
    From the *Department of Nuclear Medicine, and †Paediatric Department, University Hospital Split, Split, Croatia.
    Lymphoscintigraphy is not considered as a first line diagnostic procedure in abdominal or thoracic lymphorrhea of various origin. We report a patient with lymphangiectasia in whom posttraumatic lymphorrhea was diagnosed by lymphoscintigraphy only after third attempt when we applied pushups exercise with the aim to raise venous pressure and thus provoke lymph backflow in ductus thoracicus and enteric lymphytics. Lymphorrhea was clearly visible in colon ascendens and colon transversum on 9h planar scintigram. Read More

    Surgical Drainage of Lymphangiectasia Haemorrhagica Conjunctivae.
    Cornea 2017 Jan;36(1):116-118
    *Cole Eye Institute, Cleveland Clinic, Cleveland, OH; and †Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH.
    Purpose: To report a case of nonresolving lymphangiectasia haemorrhagica conjunctivae (LHC) successfully treated with surgical drainage.

    Methods: Retrospective case report.

    Results: A 17-year-old white girl presented with a history of a large red lesion affecting her right eye. Read More

    [Malabsorption is a leading clinical sign of small bowel disease].
    Ter Arkh 2016;88(8):4-9
    Moscow Clinical Research and Practical Center, Moscow Healthcare Department, Moscow, Russia.
    The paper presents a variety of clinical manifestations of malabsorption syndrome (MAS) in celiac disease, collagenous sprue, Whipple's disease, Crohn's disease, intestinal lymphangiectasia, amyloidosis, common variable immune deficiency, and treatment of short bowel syndrome. It shows the specific features of the pathophysiology, diagnosis, and treatment of MAS in small bowel diseases. Read More

    Lymphovenous Anastomosis and Secondary Resection for Noonan Syndrome with Vulvar Lymphangiectasia.
    Plast Reconstr Surg Glob Open 2016 Aug 29;4(8):e1007. Epub 2016 Aug 29.
    Department of Plastic and Reconstructive Surgery, Radboud University Medical Center, Nijmegen, the Netherlands.
    In this case report we describe the use of a 2-stage approach to treat severe recurrent vulvar lymphangiectasia in a patient with Noonan syndrome. First, 3 functional lymphatic vessels were identified and anastomosed to venules in an end-to-end fashion. Then, in a second surgical procedure, the vulvar lesions were resected as much as possible and the vulva was reconstructed. Read More

    Pathological Investigation of Acquired Lymphangiectasia Accompanied by Lower Limb Lymphedema: Lymphocyte Infiltration in the Dermis and Epidermis.
    Lymphat Res Biol 2016 Sep 6;14(3):172-80. Epub 2016 Sep 6.
    2 Department of Plastic and Reconstructive Surgery, The University of Tokyo Hospital , Tokyo, Japan .
    Background: Sometimes acquired lymphangiectasia (lymphangioma circumscriptum), the pathological mechanism of which is unknown, accompanies lymphedema. The purpose of this study was to better understand the pathological changes present in acquired lymphangiectasia.

    Methods And Results: We examined the pathological characteristics of acquired lymphangiectasia with lymphedema among patients treated at the University of Tokyo Hospital from March 2008 to December 2015. Read More

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