1,909 results match your criteria Lymphangiectasia


Risk stratification of patients with hypoplastic left heart syndrome and intact atrial septum using fetal MRI and echocardiography.

Cardiol Young 2020 May 14:1-9. Epub 2020 May 14.

Pediatric Cardiology, Riley Hospital for Children, Indianapolis, IN, USA.

Despite prenatal diagnosis, prenatal intervention, and immediate postnatal intervention, patients with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have the highest risk for mortality. Charts for all infants diagnosed with hypoplastic left heart syndrome from 2009 to 2017 were retrospectively reviewed and compared, including pulmonary vein Doppler patterns on fetal echocardiogram and evidence of pulmonary lymphangiectasia on fetal MRI. Of the 81 newborns with hypoplastic left heart syndrome, we defined two groups. Read More

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http://dx.doi.org/10.1017/S1047951120001006DOI Listing

A familial case of alveolar capillary dysplasia with misalignment of the pulmonary veins: the clinicopathological features and unusual glomeruloid endothelial proliferation.

Diagn Pathol 2020 May 9;15(1):48. Epub 2020 May 9.

Department of Diagnostic Pathology, Kyoto University Hospital, 54 Kawahara-cho, Sakyo-ku, Kyoto, 606-8507, Japan.

Background: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare disorder of pulmonary vascular abnormality with persistent pulmonary hypertension of the newborn. The symptom usually presents within hours after birth, leading to an early demise. Heterozygous de novo point mutations and genomic deletions of the FOXF1 (forkhead box F1) gene or its upstream enhancer have been identified in most patients with ACD/MPV. Read More

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http://dx.doi.org/10.1186/s13000-020-00972-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7211333PMC
May 2020
2.411 Impact Factor

Gastrointestinal Cryptococcosis Associated with Intestinal Lymphangiectasia.

Case Rep Med 2020 20;2020:7870154. Epub 2020 Apr 20.

Internal Medicine Department, Hospital de Especialidades Eugenio Espejo, Quito, Ecuador.

Intestinal lymphangiectasia is a pathological dilation of enteric lymphatic vessels resulting in lymph leakage to the intestinal lumen. This chronic lymph leakage leads to a state of immunosuppression secondary to the loss of humoral and cellular components of the immune system and represents a potential risk factor for opportunistic infections. We report a case of protein-losing enteropathy in a seemingly immunocompetent patient. Read More

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http://dx.doi.org/10.1155/2020/7870154DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7191395PMC

Des vésicules cutanées.

Rev Med Interne 2020 Apr 28. Epub 2020 Apr 28.

Unité de Lymphologie, site constitutif du Centre national de référence des maladies vasculaires rares (lymphœdèmes primaires), Hôpital Cognacq-Jay, 15 rue Eugène Millon, 75015 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.revmed.2020.03.007DOI Listing

Chronic diarrhea with white colon: Primary Intestinal lymphangiectasia.

QJM 2020 Apr 24. Epub 2020 Apr 24.

Department of Gastroenterology, Post-Graduate institute of medical education and research, Chandigarh, India.

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http://dx.doi.org/10.1093/qjmed/hcaa139DOI Listing

Refractory hypertension due to unilateral renal lymphangiectasia: An uncommon case with a surgical solution.

Urol Case Rep 2020 Sep 8;32:101177. Epub 2020 Apr 8.

Department of Surgery, Division of Urology, Fox Chase Cancer Center - Temple University Health System, USA.

We describe a case of unilateral renal lymphangiectasia (RLM) in a 30-year-old male with severe, refractory hypertension (HTN) and end-organ effects despite five anti-hypertensives. After diagnostic testing, the patient ultimately underwent a successful right laparoscopic nephrectomy with significant improvement of HTN. We review the literature regarding the pathophysiology and management strategies of HTN in patients with renal lymphangiectasia. Read More

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http://dx.doi.org/10.1016/j.eucr.2020.101177DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7171455PMC
September 2020

Updates on bone health in children with gastrointestinal diseases.

Authors:
Hye Ran Yang

Ann Pediatr Endocrinol Metab 2020 Mar 31;25(1):10-14. Epub 2020 Mar 31.

Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.

Chronic gastrointestinal diseases such as inflammatory bowel disease, malabsorption syndromes (e.g., intestinal lymphangiectasia, celiac disease, congenital chloride diarrhea, cystic fibrosis), and postsubtotal gastrectomy state or short-bowel syndrome after extensive bowel resection are related to poor bone health in pediatric patients due to increased risks of low bone mineral density, osteoporosis, and fractures. Read More

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http://dx.doi.org/10.6065/apem.2020.25.1.10DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7136502PMC

Atypical cadherin FAT4 orchestrates lymphatic endothelial cell polarity in response to flow.

J Clin Invest 2020 May 18. Epub 2020 May 18.

Centre for Cancer Biology, University of South Australia and SA Pathology, Adelaide, South Australia, Australia.

The atypical cadherin FAT4 has established roles in the regulation of planar cell polarity and Hippo pathway signaling that are cell context dependent. The recent identification of FAT4 mutations in Hennekam syndrome, features of which include lymphedema, lymphangiectasia, and mental retardation, uncovered an important role for FAT4 in the lymphatic vasculature. Hennekam syndrome is also caused by mutations in collagen and calcium binding EGF domains 1 (CCBE1) and ADAM metallopeptidase with thrombospondin type 1 motif 3 (ADAMTS3), encoding a matrix protein and protease, respectively, that regulate activity of the key prolymphangiogenic VEGF-C/VEGFR3 signaling axis by facilitating the proteolytic cleavage and activation of VEGF-C. Read More

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http://dx.doi.org/10.1172/JCI99027DOI Listing

Primary Chylous Lymphangiectasia of Female Genitalia.

Indian J Dermatol 2020 Mar-Apr;65(2):133-135

Department of Nuclear Medicine, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India.

We report a case of a 50-year-old female who presented with multiple thick-walled vesicles involving the labia majora and minora which started developing at the age of 10 year. The vesicles extruded milky white fluid when punctured. Biochemical analysis of the fluid was consistent with chyle. Read More

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http://dx.doi.org/10.4103/ijd.IJD_76_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7059483PMC

First Report of Treatment with Pancreas-Sparing Duodenectomy in a Child with Primary Intestinal Lymphangiectasia.

J Indian Assoc Pediatr Surg 2020 Mar-Apr;25(2):106-109. Epub 2020 Jan 28.

Department of Pediatric Gastroenterology, Hepatology and Nutrition, Faculty of Medicine, Baskent University, Ankara, Turkey.

Pancreas-sparing duodenectomy (PSD) is a known surgical technique used in patients with duodenal pathologies in the adult age group. We present a 3-year-old female patient with intestinal lymphangiectasia who underwent PSD. This is the first case in which this surgical technique was used in childhood. Read More

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http://dx.doi.org/10.4103/jiaps.JIAPS_48_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7020686PMC
January 2020

A girl with protein-losing enteropathy during a ketogenic diet: a case report.

BMC Pediatr 2020 Mar 3;20(1):102. Epub 2020 Mar 3.

Department of Neurology, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders, China International Science and Technology Cooperation base of Child development and Critical Disorders, Chongqing Key Laboratory of Translational Medical Research in Cognitive Development and Learning and Memory Disorders, Children's Hospital of Chongqing Medical University, No.136, zhongshan 2nd road, yuzhong district, chongqing, 400014, China.

Background: A ketogenic diet (KD) is an effective treatment for intractable epilepsy in children. Protein-losing enteropathy (PLE) is a rarely reported but serious complication of KDs.

Case Presentation: A 3-month-old female patient presented with PLE while following a KD as treatment for intractable epilepsy. Read More

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http://dx.doi.org/10.1186/s12887-020-1991-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7053130PMC

Successful thoracic duct embolisation in a child with recurrent massive pericardial effusion diagnosed as a lymphatic anomaly.

Cardiol Young 2020 Apr 24;30(4):571-573. Epub 2020 Feb 24.

Department of Radiology, Seoul National University Hospital, Seoul, South Korea.

A 29-month-old girl had idiopathic massive pericardial effusion for over 6 months. Lymphangiography was performed for chronic and recurrent pericardial effusion and pulmonary lymphangiectasia, suspected based on CT findings. Magnetic resonance lymphangiography revealed chylolymphatic reflux from a tortuously dilated thoracic duct in the mediastinum to the pericardial space, suggesting primary chylopericardium with lymphangiectasia. Read More

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http://dx.doi.org/10.1017/S1047951120000323DOI Listing

Clinical characteristics of dogs with food-responsive protein-losing enteropathy.

J Vet Intern Med 2020 Mar 15;34(2):659-668. Epub 2020 Feb 15.

Laboratory of Veterinary Internal Medicine, Department of Clinical Sciences, Graduate School of Veterinary Medicine, Hokkaido University, Sapporo, Hokkaido, Japan.

Background: In dogs with protein-losing enteropathy (PLE), data on the clinical characteristics of food-responsive PLE (FR-PLE) remain scarce.

Objective: To determine the clinical characteristics of FR-PLE in dogs responsive to ultralow-fat diet (ULFD) management.

Animals: Thirty-three dogs diagnosed with PLE based on standard diagnostic criteria. Read More

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http://dx.doi.org/10.1111/jvim.15720DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7096654PMC

A PTPN11 mutation in a woman with Noonan syndrome and protein-losing enteropathy.

BMC Gastroenterol 2020 Feb 13;20(1):34. Epub 2020 Feb 13.

Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1, Shuaifuyuan, Wangfujing St. Beijing, Beijing, 100730, China.

Background: Noonan syndrome is an autosomal dominant, variably expressed multisystem disorder characterized by specific facial and cardiac defects, delayed growth, ectodermal abnormalities, and lymphatic dysplasias. Lymphedema and chylous pleural effusions are common in Noonan syndrome, but protein-losing enteropathy (PLE) has only rarely been described in the condition and little is known about its genetic associations.

Case Presentation: We report the case of a 30-year-old Chinese woman who developed severe recurrent edema and hypoproteinemia. Read More

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http://dx.doi.org/10.1186/s12876-020-01187-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7017519PMC
February 2020
2.365 Impact Factor

Vaginal discharge caused by lymphatic malformation identified by lymphoscintigraphy combined with T2-weighted magnetic resonance imaging.

Authors:
Gongshun Tang

J Vasc Surg Cases Innov Tech 2020 Mar 1;6(1):1-5. Epub 2020 Feb 1.

West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Prepubertal vaginal discharge is most commonly caused by vulvovaginitis and is rarely caused by lymphatic malformations, resulting in chylous vaginal discharge. The diagnosis of chylous vaginal discharge remains a challenge because of a knowledge gap. We describe a 12-year-old girl with intermittent vaginal discharge for 10 years. Read More

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http://dx.doi.org/10.1016/j.jvscit.2019.10.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6997504PMC

Primary Intestinal Lymphangiectasia (Waldmann's Disease) A Rare Case of Protein Losing Enteropathy.

J Assoc Physicians India 2020 Jan;68(1):89

MGM Medical College and MY Hospital, Indore, MP.

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January 2020

Conjunctival lymphangiectasia as a biomarker of severe systemic disease in Ser77Tyr hereditary transthyretin amyloidosis.

Br J Ophthalmol 2020 Jan 16. Epub 2020 Jan 16.

Ophhalmology, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Paris-Sud University, French Reference Center for Familial Amyloid Polyneuropathies (NNERF), Le Kremlin-Bicêtre, France

Aims: To investigate the relationship between the ophthalmic and systemic phenotypes in patients with hereditary transthyretin amyloidosis with the S77Y mutation (ATTRS77Y).

Methods: In this cross-sectional study, patients with genetically confirmed ATTRS77Y amyloidosis were enrolled. All patients underwent complete neurological examination, including staging with the Neuropathy Impairment Score (NIS), Polyneuropathy Disability (PND) score; complete cardiological evaluation, including echocardiography, cardiac MRI and/or cardiac scintigraphy and complete ophthalmic evaluation, including slit lamp examination and fundus examination. Read More

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http://dx.doi.org/10.1136/bjophthalmol-2019-315381DOI Listing
January 2020

Treating and preventing recurrence of recurrent genital acquired lymphangiectasia using lymphaticovenous anastomosis at genital area: A case report.

Microsurgery 2020 Mar 6;40(3):399-403. Epub 2020 Jan 6.

Department of Lymphatic and Reconstructive Surgery, JR Tokyo General Hospital, Tokyo, Japan.

After the simple resection of genital acquired lymphangiectasia, a high recurrence rate is reported, as lymphatic fluid from the legs to the genital region continues. Here we report a case in which we treated and prevented recurrence of recurrent genital acquired lymphangiectasia with genital lymphaticovenous anastomosis (LVA). A 48-year-old woman underwent hysterectomy and pelvic lymph node dissection for uterine cervical cancer at 30 years of age. Read More

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http://dx.doi.org/10.1002/micr.30552DOI Listing

Predictors of iron-deficiency anemia in primary care older adults: a real-world European multi-country longitudinal study.

Aging Clin Exp Res 2020 Jan 1. Epub 2020 Jan 1.

Italian College of General Practitioners and Primary Care, Health Search, Via Del Sansovino 179, 50141, Florence, Italy.

Background: Iron deficiency is a major cause of anemia in older people. Increasing the knowledge on the predictors of iron-deficiency anemia (IDA) may facilitate its timely diagnosis.

Aim: To investigate the predictors of IDA in older people in four European countries. Read More

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http://dx.doi.org/10.1007/s40520-019-01454-6DOI Listing
January 2020

Microscopic features of small bowel mucosa of patients with Crohn's disease.

BMC Gastroenterol 2019 Dec 30;19(1):232. Epub 2019 Dec 30.

State Key Laboratory for Oncogenes and Related Genes, Key Laboratory of Gastroenterology and Hepatology, Ministry of Health, Division of Gastroenterology and Hepatology, Shanghai Institute of Digestive Disease, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, 145 Middle Shandong Road, Shanghai, 200001, China.

Background: Double-balloon enteroscopy enables performing numerous small bowel biopsies for pathologic analysis. However, most histopathological characteristics of Crohn's disease are non-specific characteristics. We aimed to explore the small bowel mucosal histopathologic characters of Crohn's disease and identify some disease-specific changes. Read More

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http://dx.doi.org/10.1186/s12876-019-1138-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6937864PMC
December 2019

Carbohydrate Intolerance and Disaccharidase Measurement - a Mini-Review.

Authors:
Matthew Burke

Clin Biochem Rev 2019 Nov;40(4):167-174

Chemical Pathology, Pathology Queensland, Herston, Qld 4029, Australia.

Carbohydrate intolerance is one of several syndromes and diseases which together are known as malabsorption syndromes. These include small intestinal bacterial overgrowth (SIBO), coeliac disease, intestinal lymphangiectasia, short bowel syndrome, tropical sprue and some inherited metabolic disorders such as galactosaemia and pyruvate kinase deficiency. Specifically, the malabsorption of sugars affects morbidity for millions of sufferers across the world. Read More

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http://dx.doi.org/10.33176/AACB-19-00025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6892707PMC
November 2019

Topical sirolimus 0.1% for treating cutaneous microcystic lymphatic malformations in children and adults (TOPICAL): protocol for a multicenter phase 2, within-person, randomized, double-blind, vehicle-controlled clinical trial.

Trials 2019 Dec 17;20(1):739. Epub 2019 Dec 17.

INSERM U1246 -SPHERE « MethodS in Patients-centered outcomes and HEalth REsearch », University of Nantes, University of Tours, 37000, Tours, France.

Background: Cutaneous microcystic lymphatic malformations (CMLMs) are rare conditions in children and adults. They present as clusters of vesicles full of lymph and blood to various extents, inducing maceration, esthetic impairment, pain, and impaired quality of life. The treatment is challenging. Read More

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http://dx.doi.org/10.1186/s13063-019-3767-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6918625PMC
December 2019

Primary Intestinal Lymphangiectasia Causing Intussusception and Small Bowel Obstruction.

ACG Case Rep J 2019 Oct 15;6(10):e00233. Epub 2019 Oct 15.

Department of Gastroenterology, University of Missouri-Kansas City School of Medicine, Kansas City, MO.

Abdominal lymphangiectasia is a rare disease manifestation with a variety of anatomic locations and clinical presentations. The gastrointestinal tract may be affected, and lymphangiectasia originating in the wall of the intestine has rarely been described. We present a case of primary small bowel lymphangiectasia causing intussusception in a 30-year-old woman who presented with emesis and gastrointestinal bleeding. Read More

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http://dx.doi.org/10.14309/crj.0000000000000233DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6855551PMC
October 2019

[Clinical characteristics of Kaposiform lymphangiomatosis: a report of 8 cases].

Zhonghua Wai Ke Za Zhi 2019 Dec;57(12):921-926

Department of Pediatric Surgery, West China Hospital, Sichuan University, Chengdu 610041, China.

To investigate the clinical symptoms, treatment intervention and prognosis of Kaposiform lymphangiomatosis(KLA). Medical information and clinical characteristics data of 8 KLA patients who were admitted to Department of Pediatric Surgery of West China Hospital of Sichuan University from January 2016 to February 2019 were retrospectively reviewed and analyzed. There were 5 males and 3 females with age of 5. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-5815.2019.12.010DOI Listing
December 2019

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization.

Pediatr Gastroenterol Hepatol Nutr 2019 Nov 7;22(6):594-600. Epub 2019 Nov 7.

Department of Pediatrics, The University of Arizona College of Medicine, Tucson, AZ, USA.

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0. Read More

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http://dx.doi.org/10.5223/pghn.2019.22.6.594DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6856498PMC
November 2019

Orofacial granulomatosis presenting with the unusual sign of lymphangiectasia successfully treated with isotretinoin.

Int J Dermatol 2020 Apr 25;59(4):e109-e110. Epub 2019 Nov 25.

Department of Dermatology, Northampton General Hospital, Cliftonville, Northampton, UK.

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http://dx.doi.org/10.1111/ijd.14738DOI Listing

Obscure GI bleeding in a young man.

Gut 2019 Nov 19. Epub 2019 Nov 19.

Pathology and Laboratory Medicine, American University of Beirut, Beirut, Lebanon.

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http://dx.doi.org/10.1136/gutjnl-2019-319870DOI Listing
November 2019

Abdominal Pain, Fevers, and Intestinal Lymphangiectasia.

Clin Infect Dis 2019 11;69(11):2035-2036

Division of Infectious Diseases, Mayo Clinic, Rochester, Minnesota.

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http://dx.doi.org/10.1093/cid/ciz041DOI Listing
November 2019

Diffuse pulmonary lymphangiomatosis: A rare case report in an adult.

Medicine (Baltimore) 2019 Oct;98(43):e17349

Department of Respiratory Medicine.

Rationale: Diffuse pulmonary lymphangiomatos (DPL) is a rare aggressive lymphatic disorder characterized by proliferation of anastomozing lymphatic vessels and extremely rare in adult patients.

Patient Concerns: We report a case of diffuse pulmonary lymphangiomatosis in 59-year-old man presented with cough and sputum for 2 months.

Diagnoses: Combining clinical manifestations with results of radiological, bronchoscopy, and surgical lung biopsy, it was consistent with the diagnosis of DPL. Read More

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http://dx.doi.org/10.1097/MD.0000000000017349DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6824776PMC
October 2019

Biallelic mutation of FBXL7 suggests a novel form of Hennekam syndrome.

Am J Med Genet A 2020 01 21;182(1):189-194. Epub 2019 Oct 21.

Division of Genetics and Genomics, Boston Children's Hospital, Boston, Massachusetts.

Hennekam lymphangiectasia-lymphedema syndrome is an autosomal recessive disorder characterized by congenital lymphedema, intestinal lymphangiectasia, facial dysmorphism, and variable intellectual disability. Known disease genes include CCBE1, FAT4, and ADAMTS3. In a patient with clinically diagnosed Hennekam syndrome but without mutations or copy-number changes in the three known disease genes, we identified a homozygous single-exon deletion affecting FBXL7. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.61392
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http://dx.doi.org/10.1002/ajmg.a.61392DOI Listing
January 2020
7 Reads

Vulvar lymphangiectasia secondary to gastrointestinal tuberculosis infection in a teenager.

Pediatr Dermatol 2020 Jan 16;37(1):215-216. Epub 2019 Oct 16.

Dermatology Service, KK Women's and Children's Hospital, Singapore City, Singapore.

We report a rare presentation of vulvar lymphangiectasia that developed secondary to gastrointestinal tuberculosis in a teenager, and its spontaneous resolution after anti-tuberculous treatment was completed. Read More

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http://dx.doi.org/10.1111/pde.13920DOI Listing
January 2020
1 Read

Reversal of protein-losing enteropathy following surgical revision of a jejunal Roux-en-Y loop after liver transplantation: Look for lymphangiectasia!

Am J Transplant 2019 12 23;19(12):3440-3441. Epub 2019 Oct 23.

Department of Gastroenterology and Hepatology, University Hospitals Leuven, Leuven, Belgium.

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http://dx.doi.org/10.1111/ajt.15630DOI Listing
December 2019
1 Read

Localized acquired lymphangiectasias after breast surgery: Enhanced non-invasive diagnosis using dermoscopy and reflectance confocal microscopy.

Skin Res Technol 2020 Mar 24;26(2):205-208. Epub 2019 Sep 24.

Dermatology Clinic, University of Catania, Catania, Italy.

Background: Acquired lymphangiectasias represent cystic dilatations of the cutaneous lymphatic vessels resulting from damage and/or obstruction of previously normal lymphatics, usually secondary to surgery, radiotherapy, and/or infections.

Materials And Methods: The clinical, dermoscopic, and confocal microscopy features of three cases of acquired lymphangiectasias occurring after breast surgery are described along with histopathological correlations.

Results: Polarized dermoscopy revealed in all lesions the presence of well-circumscribed, white-yellowish lacunae surrounded by pale septa. Read More

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http://dx.doi.org/10.1111/srt.12780DOI Listing
March 2020
5 Reads

Diffuse pulmonary lymphangiomatosis.

J Bras Pneumol 2019 09 16;45(5):e20180412. Epub 2019 Sep 16.

. Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ) Brasil.

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http://dx.doi.org/10.1590/1806-3713/e20180412DOI Listing
September 2019

Orofacial Granulomatosis Presenting With Acquired Lymphangiectasia.

JAMA Dermatol 2019 Sep 11. Epub 2019 Sep 11.

Department of Dermatology, Royal Children's Hospital, Melbourne, Australia.

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https://jamanetwork.com/journals/jamadermatology/fullarticle
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http://dx.doi.org/10.1001/jamadermatol.2019.2689DOI Listing
September 2019
4 Reads

Novel clinical phenotype of generalised lymphatic dysplasia in a neonate: a missed diagnosis.

BMJ Case Rep 2019 Aug 30;12(8). Epub 2019 Aug 30.

Neonatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India.

We report a preterm neonate who had a large cervical cystic hygroma and right chylothorax. She was operated on day-21 and a near-complete resection of cystic hygroma was done. She developed refractory hypoxemia and shock post surgery and died after 24 hours. Read More

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http://dx.doi.org/10.1136/bcr-2019-229260DOI Listing
August 2019
3 Reads

Angeborene Lungenerkrankungen – welche Diagnostik ist wann sinnvoll?

Rofo 2019 Sep 20;191(S 02):S119-S121. Epub 2019 Aug 20.

Universitätsklinikum Jena, Sektion Pädiatrische Radiologie, Institut für Diagnostische und Interventionelle Radiologie.

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http://dx.doi.org/10.1055/a-0943-1293DOI Listing
September 2019

Sex-limited penetrance of lymphedema to females with CELSR1 haploinsufficiency: A second family.

Clin Genet 2019 11 23;96(5):478-482. Epub 2019 Aug 23.

Department of Surgery, University of Arizona College of Medicine, Tucson, Arizona.

A second multigeneration family with hereditary lymphedema (LE) secondary to a variant in the planar polarity gene, CELSR1, is described. Dominant inheritance of the variant was discovered using whole-exome sequencing and confirmed by Sanger sequencing. In contrast to heterozygous males, all heterozygous females showed LE during physical examination albeit variable in severity and age of onset. Read More

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http://dx.doi.org/10.1111/cge.13622DOI Listing
November 2019
4 Reads

Image Gallery: Acquired cutaneous lymphangiectasia secondary to rectal cancer treatment.

Authors:
X Wang J Sun

Br J Dermatol 2019 08 25;181(2):e36. Epub 2019 Jun 25.

Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College - Department of Pathology, 12 Jiangwangmiao Street, Nanjing, Jiangsu, 210042, China.

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http://dx.doi.org/10.1111/bjd.18010DOI Listing
August 2019
3 Reads

Primary intestinal lymphangiectasia in a 23-month- old girl.

Oxf Med Case Reports 2019 Jul 17;2019(7):omz065. Epub 2019 Jul 17.

Department of Pediatrics, Tishreen University Hospital, Lattakia, Syria.

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy which is defined as dilation of existing mucosal, submucosal, or subserosal lymphatics within the gastrointestinal tract. That causes loss of lymph fluid into the gastrointestinal tract, leading to the development of hypoproteinemia, edema, lymphocytopenia, hypogammaglobinemia, and immunologic anomalies. It is usually diagnosed in patients younger than 3 years old and is rarely first diagnosed in adulthood. Read More

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http://dx.doi.org/10.1093/omcr/omz065DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6637462PMC
July 2019
6 Reads

AS-OCT in diffuse conjunctival lymphangiectasia.

Authors:
Nikhil S Gokhale

Indian J Ophthalmol 2019 08;67(8):1338

Cornea and Ocular Surface, Gokhale Eye Hospital, Mumbai, Maharashtra, India.

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http://dx.doi.org/10.4103/ijo.IJO_2080_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6677058PMC

Hennekam syndrome: an uncommon cause of chylous ascites and intestinal lymphangiectasia in the tropics.

BMJ Case Rep 2019 Jul 19;12(7). Epub 2019 Jul 19.

Division of Paediatric Gastroenterology, Hepatology & Nutrition, Post Graduate Institute of Medical Education & Research, Chandigarh, India.

Paediatric chylous ascites in tropics is commonly caused by infections and trauma. We describe the clinical characteristics of an uncommon inherited cause of chylous ascites, Hennekam syndrome, treated by nutritional modification. Read More

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http://dx.doi.org/10.1136/bcr-2019-229419DOI Listing
July 2019
12 Reads

Protein-losing enteropathy and primary intestinal lymphangiectasia.

QJM 2020 Mar;113(3):224-225

Department of Digestive Diseases, Huashan Hospital, Fudan University, 12 Wulumuqi Middle Road, Shanghai, China.

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http://dx.doi.org/10.1093/qjmed/hcz177DOI Listing
March 2020
5 Reads

[A large acquired lymphangiectasia of the vulva: report of a case].

Zhonghua Bing Li Xue Za Zhi 2019 07;48(7):566-567

Department of Surgical Pathology, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou 310058, China; Women's Reproductive Health Laboratory of Zhejiang Province, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou 310058, China.

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http://dx.doi.org/10.3760/cma.j.issn.0529-5807.2019.07.015DOI Listing
July 2019
2 Reads

Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.

Orphanet J Rare Dis 2019 07 5;14(1):165. Epub 2019 Jul 5.

Laboratory of Pathology, West China Hospital of Sichuan University, Chengdu, 610041, China.

Background: Kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) are rare and aggressive vascular disorders. The aim of this study was to examine the clinical features and prognosis of KLA and KHE involving the thorax.

Methods: The clinical features, imaging and pathological findings, treatments and outcome were retrospectively reviewed for 6 patients with KLA and 7 patients with KHE involving the thorax. Read More

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http://dx.doi.org/10.1186/s13023-019-1147-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6612206PMC
July 2019
4 Reads
3.358 Impact Factor

Duodenal focal lymphangiectasia.

Dig Liver Dis 2019 09 26;51(9):1344. Epub 2019 Jun 26.

Department of Internal Medicine, Division of Gastroenterology, Sahlgrenska University Hospital, Sweden.

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http://dx.doi.org/10.1016/j.dld.2019.05.037DOI Listing
September 2019
7 Reads

Gastroenterologist-Level Identification of Small-Bowel Diseases and Normal Variants by Capsule Endoscopy Using a Deep-Learning Model.

Gastroenterology 2019 10 25;157(4):1044-1054.e5. Epub 2019 Jun 25.

Department of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.

Background & Aims: Capsule endoscopy has revolutionized investigation of the small bowel. However, this technique produces a video that is 8-10 hours long, so analysis is time consuming for gastroenterologists. Deep convolutional neural networks (CNNs) can recognize specific images among a large variety. Read More

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http://dx.doi.org/10.1053/j.gastro.2019.06.025DOI Listing
October 2019
7 Reads
16.716 Impact Factor

Does surgery of lymphatic malformations lead to an increase in superficial lymphangiectasia? A retrospective study of 43 patients.

Br J Dermatol 2019 Dec 25;181(6):1324-1325. Epub 2019 Aug 25.

Dermatology and Reference Center for Genodermatoses and Rare Skin Diseases (MAGEC), Institut Imagine, Hôpital Necker-Enfants Malades, AP-HP, 149 Rue de Sèvres, 75015, Paris, France, Université Paris Descartes - Sorbonne Paris Cité, Paris, France.

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http://dx.doi.org/10.1111/bjd.18236DOI Listing
December 2019
9 Reads

Lymphatic endothelial cell immunohistochemical markers for evaluation of the intestinal lymphatic vasculature in dogs with chronic inflammatory enteropathy.

J Vet Intern Med 2019 Jul 6;33(4):1669-1676. Epub 2019 Jun 6.

Department of Clinical Sciences, College of Veterinary Medicine, Colorado State University, Fort Collins, Colorado.

Background: Lymphatic endothelial cell (LEC) immunohistochemical markers have identified intestinal lymphatic vasculature abnormalities in humans with inflammatory bowel disease, but have not been used to evaluate intestinal lymphatic vasculature in a group of dogs with chronic inflammatory enteropathy (CIE).

Objectives: To utilize LEC markers to identify and measure intestinal lymphatic vasculature in endoscopic biopsy samples of CIE dogs. To evaluate whether measured lymphatic vasculature variables correlate with serum albumin concentrations. Read More

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http://dx.doi.org/10.1111/jvim.15545DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6639479PMC
July 2019
9 Reads

Severe Pulmonary Lymphedema in a Patient with Diffuse Pulmonary Lymphangiomatosis.

Am J Respir Crit Care Med 2019 11;200(9):e91-e92

Pulmonology Scientific Research Institute, Federal Medical and Biological Agency of Russian Federation, Moscow, Russia.

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http://dx.doi.org/10.1164/rccm.201901-0062IMDOI Listing
November 2019
8 Reads