How to treat an extensive form of primary intestinal lymphangiectasia?

Troskot, Rosana; Jurčić, Dragan; Bilić, Ante; Palčić, Marija Gomerčić; Težak, Stanko; Brajković, Ivana

doi:10.3748/wjg.v21.i23.7320

Cited by 17 publications

(37 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A low‐fat diet with supplementary MCT is the cornerstone of PLE management in people, proven to be the most effective, most widely prescribed approach and with minimal adverse effects . Desai et al showed that within a few weeks, a high‐protein, low‐fat diet with MCT not only improves clinical signs but also reduces mortality in people .…”

Section: Management Of Plementioning

confidence: 99%

“…Octreotide is expensive and administered parenterally, and in responsive patients discontinuation usually causes PLE recurrence . Reported adverse effects in human clinical trials were diarrhea, abdominal pain, nausea, headache, cholelithiasis, hyperglycemia, and constipation . Octreotide has been used in dogs for the treatment of insulinoma …”

Section: Management Of Plementioning

confidence: 99%

See 1 more Smart Citation

Comparative pathophysiology and management of protein‐losing enteropathy

Craven

Washabau

2019

Veterinary Internal Medicne

116

View full text Add to dashboard Cite

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.

show abstract

Section: Management Of Plementioning

confidence: 99%

Section: Management Of Plementioning

confidence: 99%

Comparative pathophysiology and management of protein‐losing enteropathy

Craven

Washabau

2019

Veterinary Internal Medicne

116

View full text Add to dashboard Cite

show abstract

“…The lowest concentrations are found in serum proteins with long half-life and slow synthesis, so the levels of IgG, IgM and IgA will be significantly reduced, while insulin and IgE will be normal due to their short half-life. Lymphocytopenia is a finding that suggests IL and is not found in other protein-losing enteropathies 7,8 . PIL is not easily diagnosed due to localization of changes in the small intestine, which is the most difficult to access in endoscopic diagnostics.…”

Section: Disscusionmentioning

confidence: 99%

Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge

et al. 2018

View full text Add to dashboard Cite

Introduction. Primary intestinal lymphangiectasia is a rare disorder, characterized by abnormal dilation of intestinal lymphatic vessels and extensive enteric loss of lymph rich in plasma proteins, lymphocytes and chylomicrons. The main characteristics of the disease are hypoalbuminemia, hypogammaglobulinemia, lymphocytopenia, and more rarely, the deficit of liposoluble vitamins and anemia. Except for primary, there are secondary lymphangiectasia, associated with celiac disease, malignant, infective and inflammatory diseases of the small intestine, fibrosis, liver and cardiovascular diseases. Case report. A male, 33 years of age, presented for his medical examination suffering from diarrhea and edema. The diagnosis was established upon the histological examination of a small intestine biopsy during double balloon enteroscopy, which revealed changes only in one segment of the intestine examined. Such a finding was later confirmed by the video endoscopy capsule. Conclusion. The diagnosis of intestinal lymphangiectasia is usually established before the age of 3, but it can also be diagnosed in adults. The diagnosis is based on the histological analysis of the intestinal mucosa biopsy, obtained by endoscopic procedures. The diagnosis of primary intestinal lymphangiectasia is also made upon the exclusion of secondary causes.

show abstract

“…Intestinal lymphangiectasia (IL) is a rare 1 - 4 benign disease characterized by focal or diffuse dilation of the mucosal, submucosal, and subserosal lymphatics. 2 , 5 In addition to being an important cause of protein losing enteropathy (PLE), 6 IL is frequently associated with extraintestinal lymphatic abnormalities. 5 Depending on the underlying pathology IL can be classified as primary or secondary disease.…”

mentioning

confidence: 99%

“… 1 , 2 , 4 Palliative treatment with lifelong dietary modification is the most effective and widely prescribed therapy. 6 Limiting the dietary fat intake reduces chyle flow and therefore, protein loss. 1 Once protein level is within the normal range, recurrence of enteric protein loss can be prevented by total parenteral nutrition (TPN) and medium chain triglycerides (MCT).…”

mentioning

confidence: 99%

Intestinal lymphangiectasia in children

Isa¹,

Al-Arayedh²,

Mohamed³

2016

SMJ

View full text Add to dashboard Cite

Intestinal lymphangiectasia (IL) is a rare disease characterized by dilatation of intestinal lymphatics. It can be classified as primary or secondary according to the underlying etiology. The clinical presentations of IL are pitting edema, chylous ascites, pleural effusion, acute appendicitis, diarrhea, lymphocytopenia, malabsorption, and intestinal obstruction. The diagnosis is made by intestinal endoscopy and biopsies. Dietary modification is the mainstay in the management of IL with a variable response. Here we report 2 patients with IL in Bahrain who showed positive response to dietary modification.

show abstract

How to treat an extensive form of primary intestinal lymphangiectasia?

Cited by 17 publications

References 13 publications

Comparative pathophysiology and management of protein‐losing enteropathy

Comparative pathophysiology and management of protein‐losing enteropathy

Primary intestinal lymphangiectasia in adults - diagnostic and therapeutic challenge

Intestinal lymphangiectasia in children

Contact Info

Product

Resources

About