Experimental Protein-Losing Gastroenteropathy

Kondo, Motoharu; Nakanishi, Kazuo; Bamba, Tadao; Hosokawa, Keimei; Masuda, Masasuke

doi:10.1016/s0016-5085(76)80555-0

Cited by 20 publications

(5 citation statements)

References 6 publications

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“…1 In rats, protein-losing enteropathy also occurs after ligation of the thoracic duct. 7 Blalock and his colleagues 8 demonstrated that there were multiple routes of lymphatic drainage to the venous system other than just through the thoracic duct. Because of these numerous sites of drainage, he found it difficult completely to disrupt lymphatic drainage simply by ligating the thoracic duct in dogs.…”

Section: Discussionmentioning

confidence: 99%

“…In sheep, elevation of venous pressure in the neck caused by infusion of saline leads to increased lymphatic pressure in the gut 1 . In rats, protein-losing enteropathy also occurs after ligation of the thoracic duct 7 . Blalock and his colleagues 8 demonstrated that there were multiple routes of lymphatic drainage to the venous system other than just through the thoracic duct.…”

Section: Discussionmentioning

confidence: 99%

“…scribed in animal models and humans following ligation of the thoracic duct, or with elevated pressure in the brachiocephalic vein. [1][2][3][4][5][6][7][8] In humans, this problem has been associated with constrictive pericarditis, restrictive cardiomyopathy, severe tricuspid insufficiency or stenosis, the Fontan operation [9][10][11][12] and, obstruction of the superior caval vein, the latter occurring with obstruction of the superior caval venous limb of the interatrial rerouting that is part of the Mustard or Senning procedures. 13 We describe our experience with 3 patients having protein-losing enteropathy, 2 with superior caval venous obstruction, and a third with stenosis in the Fontan circuit.…”

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confidence: 99%

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Role of caval venous manipulation in treatment of protein-losing enteropathy

et al. 2008

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Protein-losing enteropathy has been associated with the Fontan operation, restrictive cardiomyopathy, constrictive pericarditis, tricuspid valvar stenosis and insufficiency, and superior caval venous obstruction. The mechanism of development of this complication of cardiac disease likely is multifactorial. We report here our experience with 3 patients with protein-losing enteropathy. Two had superior caval venous occlusion. The third patient had undergone an extracardiac Fontan operation, with stenosis of the extracardiac conduit placed to the right pulmonary artery. In all three cases, the complication was resolved by restoring unobstructed flow of blood in the superior or inferior caval vein. Since the aetiology of protein-losing enteropathy associated with cardiac disease is multifactorial, reduction of pressure in the tributaries of the superior caval vein may, in some cases, be curative. It is more difficult to cure the problem when it is associated with the Fontan operation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Role of caval venous manipulation in treatment of protein-losing enteropathy

et al. 2008

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“…Tranexamic acid 25 mg/kg/dose three times a day was used orally (maximum 1000 mg) for 5 days [ 29 ], and patients showed clinical improvement after 1 month of treatment [ 27 ]. The mechanism of antiplasmin therapy is the normalization of tissue fibrinolytic activity [ 43 ]. Increased fibrinolytic activity, which causes intestinal protein loss, has been proposed as a mechanism.…”

Section: Discussionmentioning

confidence: 99%

Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature

et al. 2021

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Background Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a review of the literature on second-line therapy for primary intestinal lymphangiectasia. Method Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included and retrospectively reviewed in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved. Results Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for 3 months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3–4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after 1 month of sirolimus treatment. Conclusion Surgery or embolization is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.

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“…Tranexamic acid 25 mg/kg/dose three times a day was used orally (maximum 1,000 mg) for ve days [29], and patients showed clinical improvement after one month of treatment [27]. The mechanism of antiplasmin therapy is the normalization of tissue brinolytic activity [37]. Increased brinolytic activity, which causes intestinal protein loss, has been proposed as a mechanism.…”

Section: Discussionmentioning

confidence: 99%

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Systemic Review

Kwon

Kim

et al. 2020

Preprint

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Background: Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We experienced seven patients with primary intestinal lymphangiectasia who showed clinical improvement with different treatments. Aim: We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a systemic review of second-line therapy for primary intestinal lymphangiectasia.Method: Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved.Results: Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for three months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3 – 4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after one month of sirolimus treatment.Conclusion: Intervention is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.

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Experimental Protein-Losing Gastroenteropathy

Cited by 20 publications

References 6 publications

Role of caval venous manipulation in treatment of protein-losing enteropathy

Role of caval venous manipulation in treatment of protein-losing enteropathy

Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature

Individual Approach for Treatment of Primary Intestinal Lymphangiectasia in Children: Single-center Experience and Systemic Review

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