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Table 2 Summary of articles/case reports of intestinal lymphangiectasia treated with second-line therapy. wks; week-old, yr.; year-old, mo.; month-old

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

Author Age Group Type of lymphangiectasia Intestinal involve 3rd space involve Response to diet therapy Type of 2nd-line therapy Results of 2nd-line therapy
CP Chen et al. (2003) [24] 49 yr. 1 Focal intestine Focal No No Surgical resection Remission
L Zhu et al. (2010) [32] 22 yr. 1 Focal intestine Focal No Limited data Surgical resection Remission
44 yr. 1 Focal intestine Focal No Limited data Surgical resection Remission
71 yr. 1 Focal intestine Focal No Limited data Surgical resection Remission
55 yr. 1 Focal intestine Focal No Limited data Surgical resection Remission
W Kneist et al. (2013) [25] 58 yr. 1 Focal intestine Focal No No Surgical resection Remission
J Mari et al. (2019) [28] 10 yr. 1 Focal intestine Focal No Limited data Surgical resection Remission
G Kuroiwa et al. (2001) [26] 21 yr. 1 Focal intestine Focal No No Octreotide Recurred after discontinuation
L Filk et al. (2004) [34] 25 yr Limited data Limited data Limited data Limited data No Octreotide Clinical improvement
S Sari et al. (2010) [30] N = 6, age 2–24 mo. Limited data Limited data Limited data Limited data No Octreotide Clinical improvement
Suehiro et al. (2012) [22] 63 yr. 1 Focal intestine Focal No No Octreotide Clinical improvement
MJ Alshikho et al.(2016) [35] 24 yr Limited data Limited data Limited data Limited data No Coconut oil + Octreotide Clinical improvement
Troskot et al. (2015) [31] 42 yr. 2 Diffuse intestine Diffuse No No Octreotide Clinical improvement
Z Altın et al. (2018) [2] 34 yr. 3 Extensive limited Yes No Octreotide Recurred after discontinuation
Acer-Demir et al. (2020) [23] 3 yr. 3 Extensive Diffuse Yes No Octreotide ➔
Surgical resection
Died d/t uncontrolled symptoms
MacLean et al. (2002) [27] 14 yr. 3 Extensive Diffuse Yes No Octreotide➔
Tranexamic acid
Clinical improvement
D Prasad et al. (2019) [29] N = 2, age 2–18 yr. 2 Diffuse intestine Diffuse No No Octreotide Clinical improvement
N = 4, age 1–10 yr. 3 Extensive Diffuse Yes No Octreotide + Tranexamic acid Clinical improvement
Ozeki et al. (2016) [20] 12 yr. 3 Extensive Diffuse Yes No Propranolol➔Everolimus Remission for 12 months