Primary intestinal lymphangiectasia and a review of the current literature

Altın, Zeynep; Atabay, Yusuf; Özer, Serhat; Karakoyun, Miray; Ekmekçi, Sümeyye; Akar, Harun

doi:10.5152/tjg.2018.18596

Cited by 11 publications

(21 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Although the mechanism of action of octreotide in diminishing protein loss through the gastrointestinal tract is unclear, theorized mechanisms of octreotide’s action in PIL include decreased intestinal fat absorption, inhibition of gastrointestinal vasoactive peptides, and stimulation of the autonomic nervous system[ 57 - 59 ]. Octreotide is usually given at doses of 150-200 µg subcutaneously twice daily[ 14 ]. From all 29 cases that reported efficacy of therapy, octreotide was added to MCT in 6 patients and started as initial treatment in one patient, with 2 patients having an insufficient response and 2 patients report in recurrence of symptoms after discontinuation of octreotide with otherwise good response.…”

Section: Discussionmentioning

confidence: 99%

Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature

Huber¹,

Semmler²,

Mayr³

et al. 2020

WJG

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature

Huber¹,

Semmler²,

Mayr³

et al. 2020

WJG

View full text Add to dashboard Cite

“…Having response to dietary therapy is ideal but some patients are non-responsive to dietary therapy. For that reason, more than 10 reports have introduced secondary therapy like surgery, octreotide, or sirolimus, but there is no consensus on how to choose and apply these therapies to patients who are refractory to dietary therapy [2,[20][21][22][23][24][25][26][27][28][29][30][31][32]. We tried to suggest a reasonable choice of second-line therapy because we had several experiences with therapeutic challenges and success with multimodal treatment options.…”

Section: Discussionmentioning

confidence: 99%

“…Clinical symptoms are induced by the excessive loss of lymphatic contents including protein, fat, and lymphocytes, resulting in hypoproteinemia and edema. Depending on the location of the injured lymphatic channel, pleural effusion, pericardial effusion, and ascites can develop as clinical features [ 1 , 2 ].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

View full text Add to dashboard Cite

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.

show abstract

“…Its symptoms include generalized edema, general weakness, and diarrhea. Serum albumin levels, immunoglobulin levels, and lymphocyte counts of patients with protein-losing enteropathy are decreased under normal limits, 1 while alpha-1 antitrypsin clearance is elevated in stool because of enteral protein loss. 2 …”

Section: Introductionmentioning

confidence: 99%

Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia

et al. 2021

View full text Add to dashboard Cite

Primary intestinal lymphangiectasia and a review of the current literature

Cited by 11 publications

References 4 publications

Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature

Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature

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

Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia

Contact Info

Product

Resources

About