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Fatty gut needs low-fat formula

Hasosah, Mohammed

Section Editor(s): Cui, Yi

doi: 10.1097/CM9.0000000000000258
Correspondence
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Department of Pediatric Gastroenterology, King Saud Bin Abdulaziz University for Health Sciences, National Guard Health Affairs, Jeddah, Kingdom of Saudi Arabia.

Correspondence to: Dr. Mohammed Hasosah, Department of Pediatric Gastroenterology, King Saud Bin Abdulaziz University for Health Sciences Jeddah, PO Box: 8202, Jeddah 21482, Saudi ArabiaE-Mail: hasosah2007@yahoo.com

How to cite this article: Hasosah M. Fatty gut needs low-fat formula. Chin Med J 2019;00:00–00. doi: 10.1097/CM9.0000000000000258

Received 24 October, 2018

This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0

To the Editor: Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia, and hypogammaglobulinemia.[1] Etiology and prevalence of the PIL are unknown. PIL affects males and females equally, and there is no racial predisposition. There is no specific treatment for PIL and fat restriction forms the cornerstone of treatment.[1] There is very little case reported in Asia.

In May 2016, a 5-month-old baby, who presented with chronic diarrhea and severe failure to thrive since the age of 1 month. The mother describes diarrhea as cheesy and foully smelling. Diarrhea (ten loose bowel movements per day) is associated with significant weight loss. Physical examination reveals that her weight and height are both below the fifth percentile for age. He was wasted and there were no subcutaneous fat tissues [Figure 1]. The laboratory study findings include a hemoglobin level of 102 g/L (reference range, 122–153 g/L) and serum albumin of 19 g/L (reference range, 35–55 g/L). White blood cell, platelet, C-reactive protein, bilirubin, alkaline phosphatase, alanine transaminase, and aspartate aminotransferase are normal. The stool sample contains many fat droplets, but his stool culture results are negative. Random stool for alpha-1-antitrypsin is 3.7 mg/g (reference range, 0–0.5 mg/g). Esophagogastroscopy reveals edematous duodenal mucosa with scattered whitish spots. The histopathologic biopsy of the duodenum reveals dilated lymphatics [Figure 2]. A diagnosis of PIL is made based on history, physical examination, hypoalbuminemia, positive stool alpha-1 antitrypsin, and corroboration on histopathology findings.

Figure 1

Figure 1

Figure 2

Figure 2

We started our patient on medium-chain triglycerides (MCT) based diet (MonogenR formula) and fat restriction. Monogen is a nutritionally complete, low fat, whole whey protein powdered feed, low in long-chain triglycerides and high in MCT and supplemented with docosahexaenoic acid and arachidonic acid. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. After 3 months he gained weight, and diarrhea improved [Figure 3]. On office follow-up after hospital discharge, laboratory findings (serum albumin and stool alpha-1-antitrypsin) were normalized.

Figure 3

Figure 3

PIL is an extremely rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel. Depending on the cause of the disease, it can be classified into primary PIL, which can occur as an isolated disorder (from birth) or as a part of syndromes such as Noonan, Klippel-Trennay-Weber, von Recklinghausen, Hennekam, and yellow nail syndrome.[2] Secondary intestinal lymphangiectasia occurs as a complication to disease states, such as constrictive pericarditis, lymphoma, sarcoidosis, scleroderma, Whipple's disease, and Crohn's disease.[3]

There is no specific treatment for PIL and fat restriction forms the cornerstone of treatment.[4] It is likely that the absence of fat in the diet prevents engorgement of the intestinal lymphatics with chyle, thereby preventing their rupture that results in protein and T-cell loss. Thus, a low-fat diet with supplemental MCT forms the cornerstone of management in PIL. MCT are directly absorbed into the portal venous system, which prevents lacteal engorgement. Small bowel resection may be helpful if the disease involves a localized segment of bowel.[5] Octreotide, antiplasmin (tranexamic acid), and corticosteroids are treatment options described in the literature, but there is insufficient data for their use.[5]

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Declaration of patient consent

The patient was a participant of our registered clinical trial which was approved by Institutional Ethical of King Saud Bin Abdulaziz University for Health Sciences, and informed consent was achieved from the patient.

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Conflicts of interest

None.

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References

1. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5doi: 10.1186/1750-1172-3-5.
2. Wen J, Tang Q, Wu J, Wang Y, Cai W. Primary intestinal lymphangiectasia: four case reports and a review of the literature. Dig Dis Sci 2010; 55:3466–3472. doi: 10.1007/s10620-010-1161-1.
3. Ingle SB, Chitra RH. Primary intestinal lymphangiectasia: minireview. World J Clin Cases 2014; 2:528–533. doi: 10.12998/wjcc.v2.i10.528.
4. Xinias I, Mavroudi A, Sapountzi E, Thomaidou A, Fotoulaki M, Kalambakas A, et al. Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. Case Rep Gastroenterol 2013; 7:153–163. doi: 10.1159/000348763.
5. Troskot R, Jurčić D, Bilić A, Palčić MG, Težak S, Brajković I. How to treat an extensive form of primary intestinal lymphangiectasia? World J Gastroenterol 2015; 21:7320–7325. doi: 10.3748/wjg.v21. i23.7320.
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