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. Author manuscript; available in PMC: 2017 Jan 1.
Published in final edited form as: J Med Genet. 2015 Nov 6;53(1):53–61. doi: 10.1136/jmedgenet-2015-103394

Table 2.

Hyperinsulinism clinical features and management

Patient # Diazoxide
responsiveness
HI management GIR Surgery Postsurgery
management
Age off treatment
(months)
Current age
(months)
Surgery
  1 Unknown Deceased 15 ND NA NA NA
  2 Surgery 25 98% Oct+continuous feeds NA Deceased
  3 Surgery 44 2% None 1 22
  4 Surgery 22 90% Diazoxide 7 15
  5 Surgery 8 75% none 9 78
  6 Surgery 13 60% Continuous D20 5 23
  7 Deceased 50 ND NA NA NA
  8 Surgery 5 biopsies Continuous D20 21 52
  9 Surgery 32 99% None 2.25
  10 Surgery 14.5 91% None 5 68
  11 Surgery 22 95% Continuous feeds 28 44
  12 Surgery 18 85% None 18 27
  13 Unknown Deceased 32 ND NA NA NA
  14 Deceased 24 ND NA NA NA
Octreotide
  15 Oct 11 NA 12 62
  16 Oct 5.5 NA 7 17
  17 Oct 7.7 NA 24 60
  18 Oct NK NA 48 107
  19 Oct 6.1 NA 17 45
  20 Oct 13 NA NA Deceased
Diazoxide
  21 + Diaz NK NA 24 177
  22 + Diaz 11.5 NA 24 53
  23 + Diaz NK NA 1 75
  24 + Diaz 21 NA 16
  25 + Diaz 10 NA 12 63
  26 + Diaz 10 NA NK 36
IC2
  27 + Low-dose diaz 12 NA 15
  28 NT Frequent feeds NK NA 13 22

D20, intravenous dextrose; Diaz, diazoxide; GIR, glucose infusion rate in mg/kg/min-; NA, not applicable; ND, patient died prior to surgery; NK, not known; NT, diazoxide not trialled; Oct, octreotide.