Table 1.

Information on patients and families, at the end of the study, stratified by genetic findings

	No PV	BMPR1A	SMAD4	PTEN	Total
Number of patients (male:female)	4* (2:2)	20 (10:10)	41 (21:20)	1 (1:0)	66 (34:32)
Number of families	4*	7	15	1	27
Mean age	34 years	48 years (11–73 years)	40 years (2–78 years)	59 years	40 years (2–78 years)
Deceased patients	0	4	9	0	13
Mean age at death	–	59 years (37–86 years)	54 years (25–77 years)	–	57 years
Family history: yes/no/unknown	0/4/0	15/2/4	30/7/4	0/1/0	45/13/8

*In one patient/family genetic analyses were not performed