Correction to: Real-World Outcomes with Lomitapide Use in Paediatric Patients with Homozygous Familial Hypercholesterolaemia

Correction to: Adv Ther (2019) 36:1786–1811 10.1007/s12325-019-00985-8

In this article, the drug name in figure 2 is given as Atorvastatin 20 mg incorrectly. The correct Fig. 2 is given below.

Fig. 2.

Evolution of LDL-C values in case 2 with lomitapide therapy. Upper panel shows mean interval LDL-C levels for patient 2. Middle panel shows lomitapide dose changes over time. Lower panel shows corresponding ALT (closed circles) and AST (open squares) levels over the same period. Dotted line on upper panel shows EAS targets for LDL-C levels in children with HoFH. Dotted line on lower panel indicates 3× upper limit of normal for LFTs; ALT alanine aminotransferase, AST aspartate aminotransferase, EAS European Atherosclerosis Society, HoFH homozygous familial hypercholesterolaemia, LDL-C low-density lipoprotein cholesterol, LFTs liver function tests, Q2W every 2 weeks, ULN upper limit of normal

The Tables 1 and 2 consists few errors in published article. The correct Tables 1 and 2 are given below.

Table 1.

Individual data for the 11 patients

Parameter	Patient
	1	2	3	4	5	6	7	8	9	10	11
Sex	Female	Male	Male	Male	Female	Male	Female	Male	Male	Female	Male
Age, years	13	12	16	7	11	16	4	14	15	11	9
Genetic variant	LDLR c.313+5 G>A	LDLR c.682G>T	LDLR c.119_1207del	LDLR c.666C>A, c.1646C>A	c.-187-? 940+? Dup	LDLR c.131G>A, c.2043C>A	LDLR c.2043C>A	LDLR c.1846-? 2311+?del, c.1895A>T	LDLR c.313+1 G>A, del exon 1–6	LDLR c.1731G>T	LDLR c.1731G>T
LDL-C at diagnosis, mg/dL	799	672	981	1008	1009	901	739	474	982	1002	824
LLT prior to lomitapide	Statins, ezetimibe, LA	Statins, ezetimibe, LA	Statins, ezetimibe, LA, EV	Statins, ezetimibe, bile acid sequestrant	Statins, ezetimibe	Statins, ezetimibe, LA, EV	Statins, ezetimibe	Statins, ezetimibe	Statins, ezetimibe, LA, EV	Statins, ezetimibe, bile acid sequestrant	Statins, ezetimibe, bile acid sequestrant
Duration of therapy prior to lomitapide, years	11	2	14	3	8	6	< 1	6	11	8	8
LDL-C prior to lomitapide, mg/dL	299	326	187	833	443	274	649	223	81	630	705
LDL-C at nadir, mg/dL	56	98	73	360	231	23	236	75	62	441	460
Concomitant LLT	Atv 40mg Ez 10mg LA Q2W	Ro 20mg Ez 10mg LA Q15D	Ro 20mg Ez 10mg Ev 420mg QW Co 3250mg LA Q1W	Ro 20mg Ez 10mg Co 625mg	Atv 10mg Ez 10mg	Ro 20mg Ez 10mg LA Q2W	Atv 10mg Ez 5mg	Ro 30mg Ez 10mg	Atv 40mg Ez 10mg LA 2xW	Atv 40mg Ez 10mg Cholestyramine 4g	Atv 40mg Ez 10mg Cholestyramine 4g
Maximal reduction with lomitapide, %	81	70	61	57	48	92	64	66	24	27	34
Maximum dose of lomitapide, mg/day	20	40	60	30	20**	30	15	15***	15	20	20
Length of lomitapide exposure, months	17	15	20	15	48	15	12	22	18	19	19
Change in concomitant LLT	Ev stopped§ LA stopped	Ev stopped§ LA reduced to Q4W	Ev stopped§ Ro stopped LA reduced to Q2W	None	Atv 40mg† Atv 60mg†	Ev stopped§ Ro 30mg Ro 40mg**** LA stopped	None	Ez stopped§ LA stopped	LA reduced 75% Ev stopped§	None	None
Liver status	Liver enzymes normal	Liver enzymes normal	Elevated liver enzymes resolved after Ro stopped	Liver enzymes normal	Liver enzymes normal	Minimal ALT increase resolved without intervention	Liver enzymes normal	ALT increases managed with lomitapide dose reduction	Liver enzymes and liver imaging normal	Liver enzymes normal	Liver enzymes normal
Adverse events¶	Nausea, vomiting, Diarrhoea, frequent bowel move-ments	Diarrhoea, vomiting	Flatulence, hypertransaminasaemia	None	Diarrhoea	Gastrointestinal pain, Hypertransaminasaemia	Diarrhoea	Hypertransaminasaemia	None	None	None

AE adverse events, ALT alanine aminotransferase, At atorvastatin, Co colesevalem, Ev evolocumab (all Ev stopped prior to lomitapide), Ez ezetimibe, GI gastrointestinal, LA lipoprotein apheresis, LDL-C low-density lipoprotein cholesterol, LLT lipid-lowering therapies

All oral drug doses are daily

*Q2W

**patient briefly received 30mg/day before back-titration to 20mg/day

***patient briefly received 20mg/day before back-titration to 15mg/day

****subsequent, post-hoc reduction to Ro 35mg

^†atorvastatin dose changes – Atv dose increased to 60mg near end of observation period

^§Patient had also received evolocumab (no response), which had been stopped before commencement on lomitapide

^¶MedDRA preferred term

Table 2.

Summary data for the 11 patients

Parameter	Age	Baseline LDL-C, mg/dL	Nadir LDL-C, mg/dL	Percentage reduction in LDL-C from baseline to nadir, %	Lomitapide dose, mg/day	Lomitapide exposure, months
Mean	11.6	422.7	192.2	56.7	25.0	20.0
Median	12.0	325.5	98.0	61.2	20.0	18.2
SD	3.8	245.4	163.2	21.7	13.8	9.5

LDL-C low-density lipoprotein cholesterol, SD standard deviation

In the result section of abstract, text has been updated. The incorrect text is “In the 11 cases, mean baseline LDL-C was 419 ± 74.6 mg/dL and was markedly reduced by lomitapide to a nadir of 176.7 ± 46.3 mg/dL (58.4 ± 6.8% decrease). Six patients achieved recommended target levels for children below 135 mg/dL, five of whom had LA frequency reduced.”

The correct text is “In the 11 cases, mean baseline LDL-C was 422 ± 245.4 mg/dL and was markedly reduced by lomitapide to a nadir of 192.2 ± 163.2 mg/dL (56.7 ± 21.7% decrease). Six patients achieved recommended target levels for children below 135 mg/dL, three of whom had LA frequency reduced and a further three stopped LA.”

In the section of Summary of the Case Series, text has been updated. The incorrect text is “Table 2 provides summary descriptive statistics for all 11 patients. Baseline LDL-C was 419.9 ± 74.6 mg/dL. The mean at nadir was 176.7 ± 46.3 mg/dL, representing a 58.4 ± 6.8% reduction in LDL-C. Note that patients 9–11 had modest decreases in LDL-C levels (patient 9 was treated to reduce LA frequency, and patients 10 and 11 had compliance issues). These LDL-C reductions were achieved with a mean dose of lomitapide 24.5 ± 4.3 mg/day over a mean period of 20.0 ± 2.9 months.”

The correct text is “Table 2 provides summary descriptive statistics for all 11 patients. Baseline LDL-C was 422.7 ± 245.4 mg/dL. The mean at nadir was 192.2 ± 163.2 mg/dL, representing a 56.7 ± 21.7% reduction in LDL-C. Note that patients 9–11 had modest decreases in LDL-C levels (patient 9 was treated to reduce LA frequency, and patients 10 and 11 had compliance issues). These LDL-C reductions were achieved with a mean dose of lomitapide 25.0 ± 13.8 mg/day over a mean period of 20.0 ± 2.9 months.”