Table 4 Prevalence of visceral manifestations in HHT1, HHT2, and HHT? after exclusion of the proband of each family (first patient referred and ascertained) in order to correct for possible referral bias .
| HHT1 | HHT2 | HHT? | p | |||||
|---|---|---|---|---|---|---|---|---|
| PAVM | 133/300 (44.3%) | 3/88 (3.4%) | 16/42 (38.1%) | 1.5×10−12 (pc = 6×10−12) | ||||
| CAVM | 31/225 (13.8%) | 1/50 (2.0%) | 2/27 (7.4%) | 0.019 (pc = 0.094) | ||||
| CAVM + PAVM | 16/218 (7.3%) | 0/45 (0%) | 0/27 (0%) | 0.06 (NS) | ||||
| HAVM | 9/119 (7.6%) | 8/21 (38.1%) | 6/19 (31.6%) | 7.8×10−5 (pc = 0.0004) | ||||
| GI telangiectasia | 44/62 (71%) | 12/18 (66.7%) | 7/11 (63.3%) | NS (NS) |
Patients with a doubtful result were not included in the analysis. Denominators varied between categories because not all patients underwent all examinations for all visceral organs. The statistical analysis was performed comparing HHT1 and HHT2. The p values are shown, with p values after correction for multiple testing in brackets