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Journal of Veterinary Internal Medicine Mar 2019Protein-losing enteropathy, or PLE, is not a disease but a syndrome that develops in numerous disease states of differing etiologies and often involving the lymphatic... (Review)
Review
Protein-losing enteropathy, or PLE, is not a disease but a syndrome that develops in numerous disease states of differing etiologies and often involving the lymphatic system, such as lymphangiectasia and lymphangitis in dogs. The pathophysiology of lymphatic disease is incompletely understood, and the disease is challenging to manage. Understanding of PLE mechanisms requires knowledge of lymphatic system structure and function, which are reviewed here. The mechanisms of enteric protein loss in PLE are identical in dogs and people, irrespective of the underlying cause. In people, PLE is usually associated with primary intestinal lymphangiectasia, suspected to arise from genetic susceptibility, or "idiopathic" lymphatic vascular obstruction. In dogs, PLE is most often a feature of inflammatory bowel disease (IBD), and less frequently intestinal lymphangiectasia, although it is not proven which process is the true driving defect. In cats, PLE is relatively rare. Review of the veterinary literature (1977-2018) reveals that PLE was life-ending in 54.2% of dogs compared to published disease-associated deaths in IBD of <20%, implying that PLE is not merely a continuum of IBD spectrum pathophysiology. In people, diet is the cornerstone of management, whereas dogs are often treated with immunosuppression for causes of PLE including lymphangiectasia, lymphangitis, and crypt disease. Currently, however, there is no scientific, extrapolated, or evidence-based support for an autoimmune or immune-mediated mechanism. Moreover, people with PLE have disease-associated loss of immune function, including lymphopenia, severe CD4+ T-cell depletion, and negative vaccinal titers. Comparison of PLE in people and dogs is undertaken here, and theories in treatment of PLE are presented.
Topics: Animals; Dog Diseases; Dogs; Humans; Inflammatory Bowel Diseases; Lymphangiectasis, Intestinal; Lymphatic System; Protein-Losing Enteropathies
PubMed: 30762910
DOI: 10.1111/jvim.15406 -
Clinical & Experimental Optometry May 2018
Topics: Conjunctiva; Conjunctival Diseases; Diagnosis, Differential; Female; Humans; Lymphangiectasis; Slit Lamp Microscopy; Tomography, Optical Coherence; Young Adult
PubMed: 29232763
DOI: 10.1111/cxo.12647 -
Current Biology : CB Apr 2017Parpaite and Coste introduce Piezo channels and their role in mechanotransduction.
Parpaite and Coste introduce Piezo channels and their role in mechanotransduction.
Topics: Anemia, Hemolytic, Congenital; Animals; Craniofacial Abnormalities; Humans; Hydrops Fetalis; Ion Channels; Lymphangiectasis, Intestinal; Lymphedema; Mammals; Mechanotransduction, Cellular; Mutation
PubMed: 28376327
DOI: 10.1016/j.cub.2017.01.048 -
Clinical Medicine (London, England) Dec 2017Lymphoedema is the build-up of lymphatic fluid leading to swelling in the tissues. Most commonly it affects the peripheries. Diagnosis is based on clinical assessment... (Review)
Review
Lymphoedema is the build-up of lymphatic fluid leading to swelling in the tissues. Most commonly it affects the peripheries. Diagnosis is based on clinical assessment and imaging with lymphoscintigraphy. Treatment is supportive with compression garments, massage, good skin hygiene and prompt use of antibiotics to avoid the complication of cellulitis. Most commonly, lymphoedema occurs as a result of damage to the lymphatic system following surgery, trauma, radiation or infection. However, it can be primary, often associated with a genetic defect that causes disruption to the development of the lymphatic system. Common genetic conditions associated with lymphoedema include Turner syndrome and Noonan syndrome; however, there are numerous others that can be classified based on their clinical presentation and associated features. Herein we discuss how to diagnose and classify the known primary lymphoedema conditions and how best to investigate and manage this group of patients.
Topics: Age of Onset; Craniofacial Abnormalities; Genetic Testing; Humans; Ion Channels; Lymphangiectasis, Intestinal; Lymphedema; Noonan Syndrome; Receptor, EphB4; Turner Syndrome; Vascular Endothelial Growth Factor Receptor-3
PubMed: 29196357
DOI: 10.7861/clinmedicine.17-6-552 -
Lymphatic Research and Biology 2011Lymphangiomatosis is a rare disease characterized by diffuse infiltration of lymphangiomas in the lung, bone, and other tissues. Due to its rarity, the spectrum of...
Lymphangiomatosis is a rare disease characterized by diffuse infiltration of lymphangiomas in the lung, bone, and other tissues. Due to its rarity, the spectrum of lymphangiomatosis is beginning to be elucidated based on case reports. The limited pathological, radiological, and clinical studies have shed light on this disease. Treatments have been tested in unblinded manner with promising results; however, further understanding of the pathogenesis of disease, as well as its natural history, is needed to facilitate drug development.
Topics: Diagnosis, Differential; Diagnostic Imaging; Humans; Lung Diseases; Lymphangiectasis; Lymphangioleiomyomatosis; Respiratory Function Tests
PubMed: 22196284
DOI: 10.1089/lrb.2011.0023 -
RoFo : Fortschritte Auf Dem Gebiete Der... Sep 2019
Topics: Bronchi; Bronchopulmonary Sequestration; Cystic Adenomatoid Malformation of Lung, Congenital; Female; Humans; Infant; Infant, Newborn; Lung; Lung Diseases; Lymphangiectasis; Magnetic Resonance Imaging; Pregnancy; Prognosis; Pulmonary Emphysema; Trachea; Ultrasonography, Prenatal
PubMed: 31430779
DOI: 10.1055/a-0943-1293 -
Circulation Research Jul 2022
Topics: Cations; Channelopathies; Craniofacial Abnormalities; Humans; Lymphangiectasis, Intestinal; Lymphatic Vessels; Lymphedema
PubMed: 35861738
DOI: 10.1161/CIRCRESAHA.122.321400 -
Jornal Brasileiro de Pneumologia :... Sep 2019
Topics: Adolescent; Biopsy; Humans; Immunohistochemistry; Lung Diseases; Lymphangiectasis; Male; Pleural Effusion; Tomography, X-Ray Computed
PubMed: 31531617
DOI: 10.1590/1806-3713/e20180412 -
Orphanet Journal of Rare Diseases Oct 2006Congenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung, and characterized by pulmonary subpleural, interlobar, perivascular and... (Review)
Review
Congenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung, and characterized by pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth with severe respiratory distress, tachypnea and cyanosis, with a very high mortality rate at or within a few hours of birth. Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. Secondary PL may be caused by a cardiac lesion. The diagnostic approach includes complete family and obstetric history, conventional radiologic studies, ultrasound and magnetic resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy, and pleural effusion examination. During the prenatal period, all causes leading to hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation and pleural drainage are nearly always necessary to obtain a favorable outcome of respiratory distress. Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy, present medical problems which are characteristic of chronic lung disease.
Topics: Diagnosis, Differential; Female; Humans; Hydrops Fetalis; Infant, Newborn; Infant, Newborn, Diseases; Lung Diseases; Lymphangiectasis; Male; Pregnancy; Prenatal Diagnosis; Prognosis; Rare Diseases
PubMed: 17074089
DOI: 10.1186/1750-1172-1-43 -
Romanian Journal of Ophthalmology 2022Conjunctival lymphangiectasia is a rare pathology that represents the enlargement of the lymphatic vessels localized in the conjunctiva. Patients may be asymptomatic or...
Conjunctival lymphangiectasia is a rare pathology that represents the enlargement of the lymphatic vessels localized in the conjunctiva. Patients may be asymptomatic or experience symptoms such as foreign body sensation, congestion, irritation, dryness, and blurry vision. There are various methods of therapy for patients with severe and symptomatic conjunctival lymphangiectasia. Surgical excision has the lowest rates of recurrence. We present a case of a 24-year-old woman with conjunctival lymphangiectasia and a history of left lower limb enlargement and bilaterally enlarged submandibular and upper jugular lymph nodes without an identifiable cause, who presented to the ophthalmology clinic accusing ocular discomfort, foreign body sensation and transparent conjunctival cystic lesions in the left eye for the last five months. OD = right eye, OS = left eye, OCT = optical coherence tomography, VEGF = vascular endothelial growth factor.
Topics: Female; Humans; Young Adult; Adult; Lymphangiectasis; Vascular Endothelial Growth Factor A; Conjunctival Diseases; Lymphatic Vessels; Vascular Diseases; Foreign Bodies
PubMed: 36589329
DOI: 10.22336/rjo.2022.65