Evidence for Medium Chain Triglycerides in the Treatment of Primary Intestinal Lymphangiectasia

Desai, Ashish; Güvenç, Birol; Carachi, Robert

doi:10.1055/s-0029-1216389

Cited by 50 publications

(36 citation statements)

References 37 publications

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“…The principles of the treatment of PIL are different from those of other forms of protein-losing enteropathy, with no exact recommendations on the dosage and duration of therapy. A high-protein, lowfat diet, supplemented with medium-chain triglycerides, is currently the most effective and widely prescribed treatment, with minimal side effects [12]. In the present case, a low-fat diet, supplemented with medium-chain triglycerides, vitamins, and electrolyte supplements was introduced, with subsequent improvements in her clinical (abdominal pain and diarrhea, cured; lower leg edema, relieved) and laboratory data (hemoglobin [14.7 g/dL, total protein [5.1 g/L], albumin [2.6 g/dL], serum calcium [8.7 mg/ dL]).…”

Section: Case Reportmentioning

confidence: 99%

Novel Magnified Single-Balloon Enteroscopy Enables Observation of Jejunal White Spots Associated with Lymphangiectasia

Tominaga

Tsuchiya

Kawata

et al. 2018

Dig Dis

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A 59-year-old woman was diagnosed with primary intestinal lymphangiectasia (PIL), with characteristic findings on capsule enteroscopy and confirmation by histopathological examination of biopsy specimens. We viewed the abnormal jejunal mucosa using a newly developed magnifying single-balloon enteroscope (SIF-Y0007). Conventional observation showed leakage of chyle. However, using this new scope, we could see scattered white villi, representing dilated lymphatic vessels within the intestinal villi protruding from the dilated submucosal lymphoid vessels (D2–40 positive) within an edematous jejunal lesion. This report is the first to describe the white villi in a patient with PIL observed clearly using a newly developed magnifying enteroscope. Technological advancements and the accumulation of reported pathological data would further improve our understanding of the pathophysiological aspects of this disease entity, even in the jejunum.

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Section: Case Reportmentioning

confidence: 99%

Novel Magnified Single-Balloon Enteroscopy Enables Observation of Jejunal White Spots Associated with Lymphangiectasia

Tominaga

Tsuchiya

Kawata

et al. 2018

Dig Dis

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“…Consistent with these results, three of the patients in this study with IL had hypoalbumenia, lymphopenia, or chronic diarrhoea. Short-and medium-chain fatty acids are absorbed directly into the portal system without contributing to the formation of chylomicrons, thus providing energy and lessening lacteal engorgement and lymph loss [17]. A low-fat diet reduces lymphatic ow and pressure, preventing the lacteal dilatation and lymph leakage resulting from their rupture.…”

Section: Discussionmentioning

confidence: 99%

What Is the Clinical Importance of White Spots in the Duodenum?

2017

Ann Clin Anal Med

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“…The benefit of this treatment is accomplished through several mechanisms: the exclusion of long-chain fatty acids prevents congestion and rupture of dilated lymphatics; and MCTs directly absorb into the portal venous circulation, which avoids lymphatic vessel overload [4] . Desai et al [5] showed that this type of diet not only improves symptoms of the disease, but also reduces mortality. Based on the published study results, it was suggested that diet supplemented with MCTs should be continued as a lifelong therapy, regardless of the inadequate patient response.…”

Section: Discussionmentioning

confidence: 99%

How to treat an extensive form of primary intestinal lymphangiectasia?

Troskot

Jurčić

Bilić

et al. 2015

WJG

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We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia, which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa. Although numerous therapeutic strategies are available, only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.

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Evidence for Medium Chain Triglycerides in the Treatment of Primary Intestinal Lymphangiectasia

Abstract: We conclude that, although an MCT diet is not completely curative in all cases, it does improve the symptoms of primary intestinal lymphangiectasia and reduces mortality. Hence it is a valid option in the paediatric age group.

Cited by 50 publications

References 37 publications

Novel Magnified Single-Balloon Enteroscopy Enables Observation of Jejunal White Spots Associated with Lymphangiectasia

Novel Magnified Single-Balloon Enteroscopy Enables Observation of Jejunal White Spots Associated with Lymphangiectasia

What Is the Clinical Importance of White Spots in the Duodenum?

How to treat an extensive form of primary intestinal lymphangiectasia?

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