Table 3.

Medical treatment and interventions during the follow-up of adult patients with primary intestinal lymphangiectasia

Case number	Age/sex	Albumin	Octreotide	Tranexamic acid	Steroid	Sirolimus	Everolimus	Outcome of treatment	Lng mediastinum MRI (reason)	Lymphangiography	Lymphatic embolization
		Periodic injection intravenously	Subcutaneous; Dose; Frequency; Duration	Oral; Dose; Frequency; Duration	Oral; Dose; Frequency; Duration	Oral; Dose; Frequency; Duration	Oral; Dose; Frequency; Duration		No/yes (reason)	No/yes (reason)	No/yes (reason)
1	30/M	Yes	(5) 100-200 mcg qd-bid for 6 month	(4) 500 mg tid for 3 month	(2) 40 mg tapered for 2 month	(3)-(4) 1-2 mg qd for 6 month	(1) 2.5 mg qd for 2 month	Improvement and observation after stopping medical treatment
2	70/M	Yes	(1)-(3) 50-100 mcg qd-bid for continuously	(2) 500 mg tid for 3 month		(3) 1 mg qd for 6 month		Octreotide continuously
3	63/F	Yes						Unable to treat appropriately due to underlying colon cancer
4	54/M	Yes				(1) 2-3 mg qd for continuously		Sirolimus continuously
5	28/F	Yes	(1)-(2) 100 mcg qd for 6 month			(2) 1-2 mg qd for continuously		Sirolimus continuously
6	67/F	Yes	(1) 100 mcg bid for 3 month	(2) 250 mg tid for continuously		(2) 2 mg qd for continuously		Tranexamic acid and sirolimus continuously	Yes (pleural effusion)	Yes (pleural effusion)	Yes (pleural effusion)
7	32/M	Yes						Regular albumin replacement	No	No	No
8	65/M	Yes				(1) 1-2 mg qd for continuously		Sirolimus continuously	No	No	No
9	41/F	Yes			(1) 40 mg tapered for 4 wk	(2) 1-2 mg qd for 1.5 yr		Improvement and observation after stopping medical treatment	Yes (pleural effusion)	No	No
10	35/M	Yes	(1)-(2) 50-200 mcg qd-bid for 2 yr	(4) 250 mg tid for 6 month	(3) 30 mg tapered for 1 month	(2)-(4) 1 mg qd for continuously		Sirolimus continuously	Yes (pleural effusion)	Yes (pleural effusion)	No
11	32/M	Yes	(2) 100 mcg qd-bid for continuously			(1)-(2) 1-4 mg qd for continuously		Octreotide and sirolimus continuously	No	No	No
12	76/F	Yes	(2) 100 mcg qd-bid for continuously			(1) 1 mg qd-bid for 6 month		Octreotide continuously	No	No	No

bid: Bis in die (two times a day); F: Female; M: Male; tid: Ter in die (three times a day); qd: Quaque die (once a day); qid: Quarter in die (four times a day).