Low-Fat Diet in Intestinal Lymphangiectasia

Jeffries, Graham H.; Chapman, A.; Sleisenger, Marvin H.

doi:10.1056/nejm196404092701503

Cited by 113 publications

(36 citation statements)

References 18 publications

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“…The rate of gastrointestinal protein loss is significantly reduced and lymphocyte level returns toward normal in some of the patients in this latter group when appropriate therapy is instituted. This is seen dramatically in constrictive pericarditis after pericardiectomy, as well as in tricuspid regurgitation after medical recompensation of cardiac failure.10 In addition, several patients with intestinal lymphangiectasia have obtained elevations of serum protein levels (55,56) as well as lymphocyte counts 11 when on a very low fat or middle chain triglyceride diet.…”

Section: Discussionmentioning

confidence: 99%

Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

Strober¹,

Wochner²,

Carbone³

et al. 1967

J. Clin. Invest.

188

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Abstract. Intestinal lymphangiectasia is a disease characterized by dilated intestinal lymphatics, protein-losing enteropathy, hypoalbuminemia, and edema. The immunologic status of 18 patients with intestinal lymphangiectasia was studied. Concentrations of IgG, IgA, and IgM were measured by immune precipitation and metabolism of these three immunoglobulins was studied using purified radioiodinated proteins. The serum concentration and total body pool of each immunoglobin were greatly reduced. The fraction of the intravascular protein pool catabolized per day was increased to 34% for IgG, 59% for IgA, and 66% for IgM; these are in contrast with control values of 7%, 28%, and 17%, respectively. Synthetic rates of the immunoglobulins were normal or slightly increased.Primary circulating antibody response was tested in five patients with Vi and tularemia antigens. Titers elicited in patients with the Vi antigen were significantly lower than those seen in a control group, whereas no difference was seen between patient and control responses to the tularemia antigen.Lymphocytopenia was noted in patients with intestinal lymphangiectasia. The mean circulating lymphocyte count was 710 + 340/mm8 in contrast to 2500 + 600/ mm3 in controls. Cellular hypersensitivity was studied with skin tests and skin grafts. 91% of normal individuals reacted to at least one of the four skin test antigens: purified protein derivative, mumps, Trichophyton, and Candida albicans; in contrast, only 17% of patients with intestinal lymphangiectasia had a positive reaction. Each of three patients tested with dinitrochlorobenzene had a negative reaction. Finally, all four patients who received skin homografts have retained these grafts for at least 12 months. The immunological disorders in patients with intestinal lymphangiectasia appear to result from loss of immunoglobulins and lymphocytes into the gastrointestinal tract secondary to disorders of lymphatic channels. Lymphocyte depletion then leads to skin anergy and impaired homograft rejection.

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Section: Discussionmentioning

confidence: 99%

Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

Strober¹,

Wochner²,

Carbone³

et al. 1967

J. Clin. Invest.

188

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“…However CT abdomen was not done in our case as the diagnosis was made on histology. Management consists of low-fat diet and supplementation with medium chain triglycerides to reduce ensuing intestinal lymph loss as medium chain triglycerides do not require bile salt activated lipase for absorption [4]. In those who do not respond to conservative line of management like dietary modification with medium chain triglycerides supplementation, enteral nutrition with elemental, semi elemental and polymeric diets may be required and even total parenteral nutrition in severe cases.…”

Section: Discussionmentioning

confidence: 99%

Primary Intestinal Lymphangieactasia in a Child

Bavanandam¹,

Dheivamani²

2016

J Gastrointest Dig Syst

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Primary intestinal lymphangiectasia is one of the causes of protein losing enteropathy in children. Three years old female child presented with anasarca, failure to thrive, anemia due to primary intestinal lymphangieactasia. Diagnosis was made by endoscopic finding with histopathology of small intestinal mucosa. She responded to diet therapy with medium chain triglycerides along with fat soluble vitamin supplementation. She is in clinical remission without loose stools, fluid retention with catch up growth on follow up period of three years till now.Keywords: Primary intestinal lymphangiectasia; Protein losing enteropathy; Chronic diarrhea; Children; Small intestinal biopsy Case ReportThree years old female child, third born of non-consanguineous parents presented with recurrent loose watery stools without blood, 6-8 episodes per day with pedal edema and puffiness of face since infancy. There was no history of cough, wheeze, rash or vomiting. Clinical examination revealed mild pallor, pedal edema, facial puffiness with weight and height less than 3SD as per WHO growth chart. Abdomen was distended with free fluid without organomegaly. Differential diagnosis of chronic diarrhea with hypoproteinemia in young children can be either primary or secondary. Some of the secondary causes include cow's milk protein allergy, intestinal tuberculosis, Strongyloidiasis, Crohn's disease, eosinphilic enteritis and hence investigations were done accordingly. Investigations showed Hb 10 gm/dl, TC 12,000 cells/cu mm. differential count polymorphs 70%, lymphocytes 30%, platelet count 4 lakhs and peripheral smear showed moderate hypochromic anemia. Blood sugar, renal function tests were normal. Motion for ova, cyst and occult blood were negative on three different occasions. Serum total protein was 4.5 gm/dl with albumin 2 gm/dl. Ascitic fluid protein was 134 mg/dl with elevated triglycerides. Chest X-ray was normal. Mantoux and retroviral tests were negative. USG abdomen showed thickened and edematous bowel with ascites. Upper GI endoscopy done using Olympus Video scope, under conscious sedation showed white fluffy flakes of duodenal mucosa due to oozing of lymph ( Figure 1) and duodenal biopsy was done. Histopathology of duodenum showed prominent dilatation of lymphatic vessels within lamina propria, blunting of villi, and mild increase in plasma cells, scattered neutrophils and eosinophils consistent with intestinal lymphangiectasia. Child is on nutritional rehabilitation along with low fat diet and medium chain triglycerides, regular fat soluble vitamin supplementation. She is on regular follow up for three years and she is asymptomatic without loose stools, fluid retention with weight of sixteen kg at six years (3 rd centile as per CDC growth chart).

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“…Low-fat diet associated with supplementary medium-chain triglycerides (MCT) is the cornerstone of PIL medical management [23]. It is likely that the absence of fat in the diet prevents engorgement of the intestinal lymphatics with chyle, thereby preventing their rupture with its ensuing protein and T-cell loss.…”

Section: Discussionmentioning

confidence: 99%

Digital clubbing in primary intestinal lymphangiectasia: a case report

Wiedermann

Kob

Benvenuti

et al. 2010

Wien Med Wochenschr

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Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, 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. The symptoms usually start in early infancy. We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea. Malabsorption with inability to gain weight, and finally weight loss and formation of leg edema were associated with protein-losing enteropathy. A low-fat diet associated with medium-chain triglyceride supplementation was clinically effective as medical management in reducing diarrhea and leg edema, and promoting weight gain. Double-balloon enteroscopy and small bowel biopsy histopathology confirmed dilated intestinal lacteals. Digital clubbing associated with primary intestinal lymphangiectasia which may causally be related to chronic platelet excess has not been reported before.

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Low-Fat Diet in Intestinal Lymphangiectasia

Cited by 113 publications

References 18 publications

Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

Primary Intestinal Lymphangieactasia in a Child

Digital clubbing in primary intestinal lymphangiectasia: a case report

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