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Table 1 Summary of all four patients with medical conditions, treatment plan and observed side effects

From: Treatment with long-acting lanreotide autogel in early infancy in patients with severe neonatal hyperinsulinism

Clinical characteristics Patient #1 Patient #2 Patient #3 Patient #4
Age at presentation hypoglycaemia DOL 1 hypoglycaemia DOL 1 hypoglycaemia at birth hypoglycaemia DOL1
Mutation homozygous c.563A > G
(ABCC8)
homozygous c.1176G > C
(ABCC8)
mosaic paternal UPD 11 mosaic paternal UPD 11
Associated syndromic condition no no yes, BWS yes, BWS
Treatment prior to LAN-ATG DZ p.o. 10 mg/kg/d
OCT sc. 43 μg/kg/d
GLUC sc. max. 50 μg/kg/d
carbohydrates 154 g/d p.o.
DZ p.o. 14 mg/kg/d
OCT sc. 36 μg/kg/d
GLUC sc. max. 84 μg/kg/d
carbohydrates 100 g/d p.o.
DZ p.o. 10 mg/kg/d
carbohydrates 69 g/d p.o.
DZ p.o. 10 mg/kg/d
OCT sc. 40 μg/kg/d
carbohydrates 78 g/d
p.o. and i.v.
LAN-ATG treatment 30–90 mg/month
(4.8–6.9 mg/kg)
30–90 mg/month
(5–7.5 mg/kg)
1× 30 mg
(5.3 mg/kg)
60 mg/14d-90 mg/month
(9.5–13.6 mg/kg)
Carbohydrates after LAN-ATG 64.2 g/d p.o. 63.6 g/d p.o. 63 g/d p.o. 78 g/d p.o.
Duration of LAN-ATG treatment 25 months 23 months single injection 3 months
Additional medication none none none DZ 8 mg/kg/d
Side effects of LAN-ATG nodules at injection site
pre-existing cholelithiasis
nodules at injection site
pre-existing biliary sludge
none nodules at injection site
  1. DOL days of life, UPD uniparental disomy, BWS Beckwith Wiedemann syndrome, DZ Diazoxide, OCT octreotide, GLUC glucagon, and LAN-ATG lanreotide autogel