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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.