Table 1

Patient characteristics

Patient	Age (years)	Genotype	Serum IgE (IU/ml)	PA	SA	ABPA		No. of courses	FEV₁ (%)		FVC (%)		Prednisolone mg/kg/day		ICS μg/day		Adverse effects	Comments
Patient	Age (years)	Genotype	Serum IgE (IU/ml)	PA	SA	Past	Active	No. of courses	Start	End	Start	End	Start	End	Start	End	Adverse effects	Comments
Denotes patients still on IVIG. PA, Pseudomonas aeruginosa; SA, Staphylococcus aureus*.
1	13.8	ΔF508/ΔF508	7	Y	N	N	N	31	36	62	65	85	0.20	0	800	800	No	Low IgG pre-IVIG
2	15.4	ΔF508/ΔF508	271	Y	Y	N	N	9	48	69	60	84	1.20	0.20	1600	1600	No
3	12.7	ΔF508/621+IG>	2424	N	N	Y	Y	9	40	51	78	87	0.60	0	2400	1600	No
4	9.0	ΔF508/ΔF508	1326	N	N	Y	Y	4	70	70	81	87	0.80	0.80	3200	3200	No
5	11.4	ΔF508/ΔF508	1739	N	N	Y	Y	7	29	38	33	52	1.20	0	3200	1600	No
6	16.6	ΔF508/I507	29	Y	N	N	N	11	26	27	61	68	1.00	0	3000	1500	No
7	14.6	ΔF508/ΔF508	340	Y	N	Y	N	66	30	51	45	93	2.00	0	3200	0	No	Index case
8	11.9	ΔF508/ΔF508	424	N	N	Y	N	7	72	80	84	112	0	0	1000	1000	No
9	15.5	ΔF508/G551D	211	Y	N	N	N	1	36	31	40	56	0	0	1600	1600	Chest tightness, fever	Refused further dose. Died post-transplant
10	3.3	ΔF508/?	70	Y	N	N	N	6	*	*	*	*	1.50	0	1600	800	Aseptic meningitis	Marked symptomatic improvement sustained
11	13.3	ΔF508/ΔF508	27	N	Y	N	N	6	47	55	59	62	0	0.20	1500	1500	No	Died of invasive aspergillosis after 6th dose
12	13.4	ΔF508/ΔF508	2993	Y	N	Y	Y	13	59	78	72	97	0.60	0	6000	1000	No
13	5.3	ΔF508/G542X	8353	Y	N	Y	Y	8	90	61	90	74	0.10	0	2000	2000	Headache, hypotension
14	13.6	ΔF508/ΔF508	201	Y	N	Y	N	6	51	50	65	72	0.80	0.25	2000	2000	No
15	10.9	ΔF508/ΔF508	84	Y	N	Y	N	5	51	45	64	65	0.30	0.15	800	800	No
16	8.5	ΔF508/ΔF508	404	Y	N	Y	Y	9	56	52	88	82	0	0	800	800	No
Median	13.0		306	Y = 11	Y = 2	Y = 10	Y = 6	7.5	48	52	65	82	0.6	0	1800	1500	No = 13