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Table 1 Clinical characteristics of 7 patients diagnosed with primary intestinal lymphangiectasia. wks; week-old, yr.; year-old

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

Patient number Age range at diagnosisa Time interval of 2nd-line therapyb Group Type of lymphangiectasia Intestinal involve 3rd space involved Response to diet therapy Type of 2nd-line therapy
1 Infant 1 Focal intestine Focal No Yes None
2 Child 0.5 yr 1 Focal intestine Focal No No Surgery
3 Child 8 yr 1 Focal intestine Focal No No Embolization
4 Adolescent 0.7 yr 3 Extensive Diffuse Yes No Octreotide➔Sirolimus
5 Neonate 3 yr 3 Extensive Diffuse Yes No Octreotide➔Sirolimus
6 Neonate 8.9 yr 2 Diffuse intestine Diffuse No No Sirolimus
7 Child 0.7 yr 2 Diffuse intestine Diffuse No No Sirolimus
  1. aThere is an accurate diagnosis age, but age is expressed as a range for anonymity
  2. bThe time when the second line therapy started from the diagnosis