Review

Authors: Melanie D Craven, Robert J Washabau

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

Authors: Thibaud Parpaite, Bertrand Coste

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

Authors: Hans-Joachim Mentzel

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

Authors: Melissa N Satria, Gustavo Pacheco-Rodriguez, Joel Moss...

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

Authors: Sara A Jablonski, Allison S W Mazepa, M Katherine Tolbert...

BACKGROUND

More than 50% of dogs with protein-losing enteropathy (PLE) fail to respond to standard therapies. Octreotide, a somatostatin analogue, is used in cases of intestinal lymphangiectasia (IL) in humans with some success.

OBJECTIVES

Describe the use of octreotide in dogs with PLE including reason for and details of prescription, adverse effects, and apparent response.

ANIMALS

Eighteen dogs with PLE, 13 with histopathology available. Ninety-two percent (12/13) had IL diagnosed on biopsy. All 13 dogs had intestinal inflammatory infiltrates noted.

METHODS

Multicenter, retrospective, descriptive study. Cases were volunteered for inclusion by individual attending veterinarians who reported the use of octreotide in cases of PLE.

RESULTS

In 16/18 (89%) cases octreotide was prescribed to PLE dogs with a clinical suspicion or confirmed diagnosis of IL that were refractory to standard therapies. Median serum albumin at the time of octreotide prescription was 1.7 g/dL (range, 1.0-3.1 g/dL). The median dose of octreotide prescribed was 20 μg/kg, SQ, daily with a range of 4-39 μg/kg, SQ, daily. Adverse effects were noted in 3/18 (17%, 95% CI [4%, 41%]) of dogs; discontinuation of the drug was necessary in 1 dog. Improvement in clinical signs was noted in 6/12 (50%, 95% CI [21%, 79%]).

CONCLUSIONS AND CLINICAL IMPORTANCE

Octreotide was most commonly prescribed to dogs with PLE and suspected or confirmed IL that had failed to respond to standard therapies. Though a benefit to PLE dogs cannot be confirmed, octreotide was well tolerated by the majority of dogs at the doses prescribed in this study.

Topics: Humans; Dogs; Animals; Retrospective Studies; Protein-Losing Enteropathies; Octreotide; Dog Diseases; Intestines; Lymphangiectasis, Intestinal

PubMed: 38038236
DOI: 10.1111/jvim.16966

Review

Authors: Carlo Bellini, Francesco Boccardo, Corradino Campisi...

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

Authors: Igor Biscotto, Rosana Souza Rodrigues, Danielle Nunes Forny...

Topics: Adolescent; Biopsy; Humans; Immunohistochemistry; Lung Diseases; Lymphangiectasis; Male; Pleural Effusion; Tomography, X-Ray Computed

PubMed: 31531617
DOI: 10.1590/1806-3713/e20180412

Review

Authors: Gabriela E Jones, Sahar Mansour

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

Authors: Peter G Swann, Tina Han

Topics: Conjunctiva; Conjunctival Diseases; Diagnosis, Differential; Female; Humans; Lymphangiectasis; Slit Lamp Microscopy; Tomography, Optical Coherence; Young Adult

PubMed: 29232763
DOI: 10.1111/cxo.12647

Authors: Roy Jung, Harish P Janardhan, Chinmay M Trivedi...

Topics: Cations; Channelopathies; Craniofacial Abnormalities; Humans; Lymphangiectasis, Intestinal; Lymphatic Vessels; Lymphedema

PubMed: 35861738
DOI: 10.1161/CIRCRESAHA.122.321400