Table 2 First manifestation of HHT disease in the 233 patients, according to Sex, Education Level, Affected Gene, and Index/Non-Index status

SEX

Total

Males

Females

Onset Age, Mean ± SD (years)

14.1 ± 11.2

14.6 ± 12.6

13.6 ± 9.7

p = ns

[Range]

[3;60]

[3;60]

[3;50]

EDUCATION LEVEL

Low

Interm

High

Onset Age, Mean ± SD (years)

17.2 ± 13.4

12.2 ± 9.8

13.8 ± 10.7

p = 0.115

[Range]

[3;51]

[3;60]

[3;58]

AFFECTED GENE

HHT1

HHT2

HHT?

Onset Age, Mean ± SD (years)

9.9 ± 8.6

16.6 ± 12.0

17.4 ± 14.4

P < 0.0001

[Range]

[3;47]

[3;58]

[4;60]

INDEX/NON-INDEX STATUS

Index

Non-Index

Onset Age, Mean ± SD (years)

16.2 ± 13

12.1 ± 9.1

P < 0.02

[Range]

[3;60]

[3;49]

CLINICAL ONSET

Nr (%)

Epistaxis alone

202 (86.7%)

Epistaxis and Mucocut. Tel.

15 (6.4%)

Epistaxis and GI bleeding

2 (0.9%)

Epist., Mucocut. Tel, and GI bleeding

1 (0.5%)

Mucocut Tel. (bleeding + non-bleeding)

6 (2.6%)

GI bleeding

2 (0.9%)

PAVM-related Accidents

2 (0.9%)

CAVM-related Accidents

1 (0.5%)

Asymptomatic (only Genetic testing)

2 (0.9%)