Skip to main content

Table 2 Clinical characteristics associated with severity of CHI

From: Vineland adaptive behavior scales to identify neurodevelopmental problems in children with Congenital Hyperinsulinism (CHI)

  Late or Early Presenting Mutation Transient or Persistent Responsive to Medication Surgery Focal or Diffuse Total VABS II SDS
# 1 E Paternal ABCC8 P Unresponsive SP D -0.9
# 2 L Negative P Diazoxide No D -2.6
# 3 L Negative P Diazoxide No D -1.3
# 4 E Compound heterozygous ABCC8 P Unresponsive SP D -0.9
# 5 L Negative P Diazoxide No D -1.1
# 6 L GCK P Diazoxide No D -2.6
# 7 E Negative T Diazoxide No D -1.9
# 8 E Paternal KCNJ11 T Diazoxide No D -1.1
# 9 E Paternal ABCC8 P Unresponsive FL F -2.3
# 10 E Negative P Diazoxide No D -2.5
# 11 E Negative P Diazoxide No D -1.3
# 12 L Negative P Unresponsive SP D -3.6
# 13 E Homozygous ABCC8 P Unresponsive SP D -1.1
# 14 L Negative P Diazoxide No D 0.7
# 15 E Negative T Diazoxide No D 1.9
# 16 E Negative T Diazoxide No D -0.8
# 17 E Negative P Unresponsive SP D 1.1
# 18 E Maternal KCNJ11 T Diazoxide No D -0.4
# 19 L Negative T Diazoxide No D 0.2
# 20 E Negative T Diazoxide No D -0.5
# 21 E Maternal ABCC8 T Diazoxide No D 0.1
# 22 E Paternal ABCC8 T Diazoxide No D -2.1
# 23 E Maternal ABCC8 P Diazoxide No D -1.7
# 24 L Negative P Diazoxide No D -1.4
# 25 E Negative T Glucose No D 0.3
# 26 L Negative P Diazoxide No D -0.3
# 27 E Negative T Diazoxide No D -1.5
# 28 E de novo ABCC8 P Diazoxide No D 2.3
# 29 E Negative T Diazoxide No D -0.8
# 30 L Negative P Diazoxide No D -0.2
# 31 E Negative T Glucose No D -2.1
# 32 L Negative T Diazoxide No D -0.1
# 33 L Paternal ABCC8 T Octreotide No D -1.4
# 34 E Paternal ABCC8 T Octreotide No D -2.7
# 35 L Negative T Diazoxide No D 1.3
# 36 E Maternal ABCC8 T Diazoxide No D 0.0
# 37 L Paternal ABCC8 P Diazoxide FL F 0.9
# 38 E Negative T Diazoxide No D 0.7
# 39 E Negative T Glucose No D 1.4
# 40 E Negative T Diazoxide No D -1.3
# 41 E Negative P Diazoxide No D -1.3
# 42 E Negative T Diazoxide No D -1.4
# 43 E Negative T Diazoxide No D -1.3
# 44 E Negative P Diazoxide No D 1.3
# 45 E Negative T Diazoxide No D 4.0
# 46 L Negative T Diazoxide No D -1.5
# 47 L Negative T Diazoxide No D 1.3
# 48 E Negative T Diazoxide No D 0.5
# 49 E Paternal KCNJ11 T Octreotide No D -0.3
# 50 E Negative T Diazoxide No D -0.2
# 51 E Negative P Diazoxide No D 0.0
# 52 E Negative P Diazoxide No D -0.9
# 53 E Negative T Diazoxide No D 0.3
# 54 E Homozygous ABCC8 P Unresponsive SP D -2.6
# 55 E Negative T Diazoxide No D 0.9
# 56 E Homozygous ABCC8 P Octreotide No D -0.4
# 57 E Negative T Diazoxide No D -0.6
# 58 E Negative P Diazoxide No D 2.2
# 59 E Paternal KCNJ11 P Octreotide No D -1.9
# 60 E Negative T Diazoxide No D -0.4
# 61 E Compound heterozygous ABCC8 P Octreotide No D 0.9
# 62 E Negative T Diazoxide No D 0.5
# 63 E Negative T Diazoxide No D -0.5
# 64 E Negative T Diazoxide No D 0.1
  1. Table of clinical descriptors in patients with CHI with time of presentation, genetic status, resolution of hypoglycaemia, response to medication, requirement for pancreatic surgery and Total VABS-II scores
  2. E early, L late, T transient, P permanent, D diffuse, F focal, FL focal lesionectomy, SP subtotal pancreatectomy
  3. All patients on octreotide had previously failed to respond to diazoxide
  4. Outcomes of surgery: Patients with focal CHI were cured after focal lesionectomy. Patient # 1 and # 13 became diabetic post-pancreatectomy; patient # 12 and # 54 are euglycaemic post-pancreatectomy; patients # 4 and #17 have impaired glucose tolerance