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Table 1 Clinical data in seven individuals with bi-allelic WNT10A mutations

From: Abnormal primary and permanent dentitions with ectodermal symptoms predict WNT10A deficiency

Patients

Ia

Ib

II

III

IV

V

VI

Total

Age at radiographic examination

8.5

7.5

8.0

6.0

16.5

6.5

7.0

 

Tooth agenesis in primary dentition

3

0

2

2

0

0

0

7

Tooth agenesis in permanent dentition

28

23

26

16

15

19

22

149

Tapered maxillary central incisors

-

2

2

2

1

0

2

9

Molars available for analysis of taurodontisma

75

75

75

-

36

75

75

 

85

85

85

85

46

85

85

13

Molars with taurodontisma

1

2

2

1

2

1

2

11

Hyperhidrosis of palms and soles

+

+

-

+

-

+

+

5

Coarse hair structure and/or light/scarce/brittle hair

+

+

+

+

+

+

+

7

Nail abnormalities

-

-

-

-

+

-

+

2

Dry skin

+

+

+

+

-

+

+

6

No. of affected ectodermal appendages

3

3

2

3

2

3

4

mean 2.9

  1. aAll were primary molars except for individual IV who had resorbed primary second molars and erupted permanent first molars