Table 1 Characteristics of long-term conservative treatment in CHI: detailed description of different treatment regimen with focus on dose, combination of different drugs and duration of treatment. Patients receiving a combined therapy are listed twice

DZX

n _total 521

n _{previous surgery} 16 (3 %)

monotherapy

n = 380 (73 %)

combined therapy

n = 141 (27 %)

+ diet^a n = 113 (79 %)

+ STA n = 9 (6 %)

+ CCA n = 2 (1 %)

>2 combinations

+ STA + diet^a n = 15 (11 %)

+ CCA + diet^a n = 2 (1 %)

n _{dose given} 278 (53 %)

mean: 12.5 (±4.3) mg/kg • d

range: 2–60 mg/kg • d

_{2–3 single doses p.o. • d}

n _{treatment ongoing} 138 (26 %)

duration given for n = 113/138 (82 %)

mean duration: 62 months

n _{off medication} 69 (13 %)

duration given for n = 56/69 (81 %)

mean duration: 57 months

n _{switched to other therapy} 4 (1 %)

partial pancreatectomy: 3/4 (75 %)

STA: 1/4 (25 %)

n _{not mentioned; no follow-up} 309 (59 %)

n _dead 1 (0.2 %)

STA

n _total 100

OCT n = 79 (79 %)

LRT n = 21 (21 %)

n _{previous surgery} 17 (17 %)

monotherapy

n = 54 (54 %)

combined therapy

n = 46 (46 %)

+ diet^a n = 16 (35 %)

+ DZX n = 9 (20 %)

+ CCA n = 4 (9 %)

+ GLC n = 2 (4 %)

>2 combinations

+ DZX + diet^a n = 15 (11 %)

OCT

n _{dose given} 61 (77 %)

mean: 14.9 (±7.5) μg/kg • d

range: 2.3–50 μg/kg • d

_{continuously s.c. n = 28 (46 %)}

_{3–6 injections s.c. • d n = 17 (28 %)}

_{not specified n = 16 (26 %)}

LRT

n _{dose given} 11 (52 %)

mean: 67.3 (±39.8) mg • month

range: 10–120 mg • month

_{s.c. n = 8 (38 %)}

_{i.m. n = 13 (62 %)}

n _{treatment ongoing} 39 (39 %)

duration given for n = 39/39 (100 %)

mean duration: 35 months

n _{off medication} 22 (22 %)

duration given for n = 6/22 (27 %)

mean duration: 49 months

n _{switched to other therapy} 10 (10 %)

partial pancreatectomy: 3/10 (30 %)

DZX: 3/10 (30 %)

gastrostomy feeding: 2/10 (20 %)

DZX + CCA: 1/10 (10 %)

megestrol acetate: 1/10 (10 %)

n _{not mentioned; no follow-up} 29 (29 %)

CCA

n _total 25

NIF n = 19 (76 %)

VPM ° n = 2 (8 %)

AML n = 1 (4 %)

not specified n = 3 (12 %)

n _{previous surgery} 10 (40 %)

monotherapy

n = 15 (60 %)

combined therapy

n = 10 (40 %)

+ diet^a n = 2 (20 %)

+ DZX n = 2 (20 %)

+ STA n = 4 (40 %)

>2 combinations

+ DZX + diet^a n = 2 (20 %)

NIF

n _{dose given} 18 (95 %)

mean: 0.72 (±0.36) mg/kg • d

range: 0.1–2 mg/kg • d

VPM

n _{dose given} 2 (100 %)

dose: 240 mg • d

AML

n _{dose given} 1 (100 %)

dose: 0.1 mg/kg • d

_{2–4 single doses p.o. • d}

n _{treatment ongoing} 16 (64 %)

duration given for n = 16/16 (100 %)

mean duration: 25 months

n _{not mentioned; no follow-up} 9 (36 %)

GLC

n _total 7

n _{previous surgery} 6 (86 %)

monotherapy

n = 2 (29 %)

combined therapy

n = 5 (71 %)

+ diet^a n = 3 (60 %)

+ STA n = 2 (40 %)

n _{dose given} 7 (100 %)

given as mg/kg • d: n = 5 (71 %)

mean: 0.186 (±0.14) mg/kg • d

range: 0.03–0.2 mg/kg • d

_{continuously s.c.}

given as mg • d: n = 2 (29 %)

mean: 3.2 mg • d

_{s.c. or i.m.}

n _{treatment ongoing} 1 (14 %)

duration given for n = 1/1 (100 %)

mean duration: 12 months

n _{off medication} 3 (43 %)

duration given for n = 3/3 (100 %)

mean duration: 43 months

n _{switched to other therapy} 2 (29 %)

re-pancreatectomy: 1/2 (50 %)

diet : 1/2 (50 %)

n _dead 1 (14 %)