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

Table 1 Clinical presentation, laboratory findings and endoscopic diagnosis of all case reports describing adult patients)¹ with primary intestinal lymphangiectasia

Clinical presentation		n
Patient characteristics	Number of adult patients with PIL	49
	Age at diagnosis, yr	43 (20-83)
	Time to final diagnosis, yr	3 (0-40)
	Male, n (%)	21 (42.9)
	Female, n (%)	28 (57.1)
Symptoms, n (%)	Edema	40/48 (83.3)
	Recurrent diarrhea	20/48 (41.7)
	Abdominal effusion	13/48 (27.1)
	Pleural effusion	10/48 (20.8)
	Abdominal pain	9/48 (18.8)
Laboratory findings, n (%)	Hypogammaglobulinemia	35/48 (72.9)
	Hypoalbuminemia	35/48 (72.9)
	Lymphocytopenia	30/48 (62.5)
	Hypoproteinemia	26/48 (54.2)
	Hypocalcemia	12/48 (25.0)
	α1-antitrypsin (stool)	10/48 (20.8)2
CT-scan, n (%)	Normal	8/27 (29.6)
	Thickened wall of small bowel	11/27 (40.7)
Diagnosis possible, n (%)	Gastro-duodenoscopy	21/35 (60.0)
	Ileo-colonoscopy	5/21 (23.8)
	Enteroscopy	13/13 (100)

¹Diagnosis established after the 18^th birthday.

²All patients (100%) in which α1-antitrypsin levels or α1-antitrypsin clearance was measured in stool samples reported elevated results. CT: Computerized tomography.