Primary Intestinal and Thoracic Lymphangiectasia: A Response to Antiplasmin Therapy

MacLean, Joanna E.; Cohen, Eyal; Weinstein, Michael

doi:10.1542/peds.109.6.1177

Cited by 71 publications

(49 citation statements)

References 21 publications

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“…IL secondary to an inflammatory process has been successfully treated with corticosteroids [5,6]. Anecdotal reports have demonstrated the efficiency of octreotide and fibrinolysis therapy using antiplasmin or tranexamic acid [4,7,8]. Finally, in selected cases in which a localized area of severe abnormalities exists in the small intestine, surgery to remove this isolated segment has been attempted, although most IL cases present with diffuse small intestinal involvement, which is not amenable to resection [9].…”

Section: Introductionmentioning

confidence: 99%

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Ohmiya

Nakamura²,

Yamamura³

et al. 2014

Digestion

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Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy. Results: Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal E₁-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response. i 2014 S. Karger AG, Basel

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Section: Introductionmentioning

confidence: 99%

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Ohmiya

Nakamura²,

Yamamura³

et al. 2014

Digestion

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“…It has been postulated that octreotide inhibits gastrointestinal vasoconstrictive peptides or stimulates the autonomic nervous system [2], which alleviates the dilation of the lymphatic vessels and thus promotes healing of the locally affected intestinal mucosa [2]. D dimers, the unique breakdown products of fibrin, are elevated under conditions of increased fibrinolytic activity, which is another mechanism leading to intestinal protein loss [4]. Tranexamic acid, which inhibits the conversion of plasminogen to plasmin and decreases fibrin breakdown, is effective in some patients with both intestinal lymphangiectasia and elevated serum fibrinolytic activity, especially those with diffuse gastrointestinal bleeding [4].…”

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confidence: 99%

Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal

Lee

Kong

2007

Eur J Pediatr

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Primary intestinal lymphangiectasia is a protein-losing enteropathy caused by congenital malformation or obstruction of intestinal lymphatic vessels, which lead to excessive protein loss in the intestine and malabsorption of both chylomicrons and fat-soluble vitamins [3]. It affects males and females equally and occurs mainly in children and young adults. The management of primary intestinal lymphangiectasia is difficult for the paediatric gastroenterologist because of the rarity of the disease and its variable clinical manifestations. The diagnosis is based on the typical upper gastrointestinal endoscopic findings of diffuse scattered mucosal white blebs with characteristic histological findings of abnormal lymphatic dilatation [3] and the exclusion of other acquired causes of lymphatic damage [3]. Here, we report on a girl with primary intestinal lymphangiectasia in whom the initial diagnosis based on upper gastrointestinal endoscopy and histology was uncertain. Intestinal lymphangiectasia was diagnosed by upper gastrointestinal endoscopic biopsy sampling after a high-fat meal challenge.An 11-year-old female was admitted elsewhere with a 1-month history of watery diarrhoea, limb tetany, numbness and oedema. Laboratory studies showed low serum protein, albumin and calcium levels. She was diagnosed with eosinophilic gastroenteropathy after upper gastrointestinal endoscopy and transferred to our hospital because of a poor response to steroids. Her family and past histories were noncontributory. Physical examination revealed a body weight of 40.5 kg (50-75th percentile), body height of 158 cm (90-97th percentile) with distended abdomen and lower leg oedema. Her sexual maturity rating (SMR) was Tanner stage 2. Bone age was 12 years. Blood tests showed a low serum total protein of 3.1 g/dl (normal range: 5.5-8 g/dl), albumin of 1.6 g/dl (normal range: 2.8-5.4 g/dl) and total calcium <5 mg/dl (normal range: 8.4-10.8 mg/dl). Her serum cholesterol was 175 mg/dl (normal range: <170 mg/dl). Her haemogram showed a white blood cell count of 6.0×10 9 /l (normal range: 3.5-11×10 9 /l) with lymphocytopenia (0.78×10 9 /l). The urinalysis was normal and the stool analysis positive for occult blood with an elevated α-1-antitrypsin level of >43.2 mg/g faeces (normal: <2.957 mg/g). Other serum biochemical tests included a blood urea nitrogen of 8 mg/dl, creatinine of 0.6 mg/dl, aspartate aminotransferase of 26 U/l, alanine aminotransferase of 23 U/l and C-reactive protein of 1.62 mg/l (normal: <5 mg/l).The coagulation profile showed a serum D-dimer level of 529.2 ng/ml (normal: <500 ng/ml), prothrombin time of 11.7 s and partial thromboplastin time of 24.7 s. Her serum antinuclear antibody and a tuberculosis skin test were both negative.The patient underwent an upper gastrointestinal endoscopy after a high-fat meal challenge that revealed patchy areas of whitish dots scattered along the second portion of the duodenum (Fig. 1). Multiple biopsies were taken from the affected area. The histological examination disclosed intestinal Eur...

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“…The antiplasmin therapy affects fibrinolysis locally and improves lymphatic permeability for proteins [7] . Previous studies have confirmed its use (trans-4-aminothylcyclohexamine carboxylic acid (tranexamic acid, Cyklokapron, Transamin, Cyclo-F and Femstrual)) in partially improving serum albumin, immunoglobulins, endoscopically observed duodenal lesions, and gastrointestinal bleeding.…”

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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Primary Intestinal and Thoracic Lymphangiectasia: A Response to Antiplasmin Therapy

Cited by 71 publications

References 21 publications

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Primary intestinal lymphangiectasia diagnosed by endoscopy following the intake of a high-fat meal

How to treat an extensive form of primary intestinal lymphangiectasia?

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