Skip to main content

Table 1 Subject medical and family history

From: Phenotypic characteristics of early Wolfram syndrome

Subject

Age

Gender

DM onset

DI onset

Optic atrophy onset

Hearing loss

Diagnosed with WFS

FH WFS

FH DM*

FH hearing loss*

Other problems

WU.WOLF-01

13.1

M

3.5

9

5

9

N/A

N

Father

N

GERD, constipation, headaches, restless legs, hypothyroidism, hypogonadism

WU.WOLF-02

10.9

F

6

7.5

9

N

9.5

N

Mgaunt

Pgf

None

WU.WOLF-03

17.9

M

5.0

6

6

6

6

N

Mgf

mgm

GERD; restless legs; occasional myoclonus

WU.WOLF-04

23.8

F

2.3

12

5

5

12

N

Father, pgm, paunts

Father

GERD; OCD spectrum behaviors; constipation, loss of taste sensation

WU.WOLF-05

13.8

F

3.8

N

12

1.7

13

N

Mgf, mggm, mgaunt

mcousin

FTT at 1 year, feeding tube at age 2 1/2; Near-drowning age 6 ½; celiac sprue

WU.WOLF-07

7.3

M

2.7

7

N

7

3

Y†

Mgf, paunt, puncle, mggf, mguncle

Pgf, 3 pguncles

anxiety

WU.WOLF-09

14.3

M

10.8

14

11

N

N/A

Y‡

Distant cousin, pggf

Maunt, muncle, pguncle, mggf

Asthma, migraines, obstructive sleep apnea constipation,

WU.WOLF-10

11.7

F

7.0

11

9

N

8

Y‡

Distant cousin, pggf

Maunt, muncle, pguncle, mggf

GERD, headaches, night terrors, constipation

WU.WOLF-11

8.3

M

7.5

10

6

8

7

Y‡

Distant cousin, pggf

Maunt, muncle, pguncle, mggf

headaches, constipation, loss of normal taste—“things taste funny”

WU.WOLF-12

22.9

M

7.0

17

7

7

17

N

Father (after kidney cancer), pgf, puncle, pggf, mgf, pguncle

N

GERD, myoclonus, neurogenic bladder, chronic renal failure, hypogonadism, loss of taste sensation

WU.WOLF-13

5.9

F

4.8

7.5

5.2

6

5.4

N

first cousin

N

Constipation

WU.WOLF-14

13.6

F

6.3

11.3

7.9

10.1

8.8

N

Mgf, pggm, mggf

N

None

WU.WOLF-15

10.8

F

2.8

N

7

9

7

N

N

brother

Constipation

WU.WOLF-16

25.8

F

13.1

14.6

13.6

25.8

14.9

N

pgf

pgm

None

WU.WOLF-17

17.2

F

5

N

15.3

16.3

16.6

N

Mother, maunt, mgm, mgf, mggf, mgaunt, mguncle, mcousin

mgaunt

None

WU.WOLF-18

11.9

M

5.1

10.33

10.1

11.9

10.6

N

Mgf

Father

None

WU.WOLF-19

11.9

F

N

N

5

Prior to 5

11

No info

No info

No info

International adoption age 5, maternal half sibling is healthy.

Constipation

WU.WOLF-22

15.8

F

14

N

13

N

14.6

N

Mggf, pgm, pgf

N

None

MEAN

14.2

 

6.3

10.6

8.7

9.0

10.3

    

STD

5.7

 

3.5

3.3

3.3

6.0

4.2

    
  1. *Not in a person with Wolfram syndrome.
  2. †3 distant cousins with Wolfram, parents consanguinous.
  3. ‡Sibling with Wolfram (patients WU.WOLF-11, WU.WOLF-10,WU.WOLF-09 are siblings).
  4. Abbreviations: DI: diabetes insipidus; DM: diabetes mellitus; FH: family history; FTT: failure to thrive; GERD: gastroesophageal reflux disease; maunt: maternal aunt; mcousin: maternal cousin; mgaunt: maternal great aunt; mgf: maternal grandfather; mggf: maternal great grandfather; mggm:maternal great grandmother; mgm: maternal grandmother; mguncle: maternal great uncle; muncle: maternal uncle; OCD: obsessive compulsive disorder; paunt: paternal aunt; pgaunt: paternal great aunt; pgf: paternal grandfather; pgm: paternal grandmother; pguncle: paternal great uncle; puncle: paternal uncle; WFS: Wolfram Syndrome.