Table 2.

Summary of dietary treatment including tripheptanoin (C7) in 12 Austrian LC-FAOD patients

Patient	Age at start of C7 therapy (years)	Total duration of C7 therapy (years)	Reported adverese events	Triheptanoin (C7) intake		Middle chain fat		Total amount fat including C7 [%]	Daily calorie intake (kcal)	Late evening meals or night feeds		Current weight		Current height		Current BMI
				g/day	g/kg/ day	C7 in diet [%] of total calories	MCT in diet [%]of total calories			Approx. time	Intake of	kg	percentile	cm	percentile	kg/m2	percentile
1	4.8	15.7	None	40	0.50	11.0	0	20	2200	22:00	Late meale	79.6	88	185	76	23.1	50
2	0.7	13.5	None	30	0.57	7.5	7.5	30	2200	22:00	Late meal	52.0	70	159	45	20.5	60
3	0.6	8.5	None	15	0.48	7.5	7.5	30	1900		No	31.0	50	148	97	14.2	10
4	1.0	3.8	None	14	0.80	7.5	7.5	30	1500	22:00	200 ml MCT based formula	17.4	50	111	77	14.1	20
5	1.0	4.0	None	40	1.00	7.5	7.5	30	1500	22:00	250 ml skimmed 0.1% milk	18.4	60	115	96	13.8	13
6	0.0	3.2	None	10	0.59	15.0	10.0	30	1000	22:00	200 ml MCT based formula	16.8	90	100	67	16.9	80
7	0.1	4.0	None	10	0.47	7.5	7.5	30	1000		No	21.0	99	116	99	15.6	50
8	0.1	4.0	None	10	0.58	7.5	7.5	30	1000		No	17.0	60	109	90	14.3	20
9	7.9	1.2a	Abdominal paina	20	0.52	13.0	8.0	30	1900	22:00	Late meal	38.0	99	131	30	22.0	95
10	3.7	1.3b	Abdominal painb	0b	1.00	0.0	30.0	40	2000	03:00	maltodextrin and MCT based formula	37.5	40	141	11	18.9	60
11	11.6	2.1	None	80	1.30	30	0.0	45	2200	03:00	70 ml MCT based formula	62.0	70	163	30	23.3	80
12	29.4	2.2c	Abdominal painc	60	0.70	20	0.0	up to 30	2520	22:00	Late meal and 80 g uncooked corn starch	84.0	82d	180	55d	26.0	89d
median	1.0	3.9			0.58	7.5	7.5	30				60	77		50

Patients 1, 2, 3 and 10 were previously reported: In: Orphanet J Rare Dis. (2015) 10:21, Karall et al. (https://doi.org/10.1186/s13023-015-0236-7), Patient 1 is Patient 2, Patient 2 is Patient 4, Patient 3 is Patient 6, Patient 10 is Patient 8

Patient 1 was previously reported in JIMD Reports (2014), Karall et al. (https://doi.org/10.1007/8904_2014_313

Patient 10 was previously reported in: Orphanet J Rare Dis. (2018) 13: 122, Lotz-Havla et. al. (https://doi.org/10.1186/s13023-018-0875-6), Patient 10 is Patient 3

^aPatient 9 discontinued C7 therapy (1 g/kg/day) for 1.5 months due to abdominal pain and recommenced at a reduced dosage (0.55 g/kg/day) with no further side-effects reported

^bIn 07/2013, at the age of 5 years, Patient 10 discontinued therapy after 15 months due to abdominal pain;

^cPatient 12 discontinued C7 therapy (1.2 g/kg/day) for seven months due to abdominal pain and recommenced at a reduced dosage (0.7 g/kg/day) with no further side-effects reported

^dAdult patient, percentiles at age 18; Kromeyer-Hauschild [13] and Zürcher Longitudinalstudien (1955–2009) [12]

^ePatient 1 together with the late meal takes: 30 g pegylated corn starch and 200 ml = 300 kcal drink with hydrolyzed protein and fiber, but fat, lactose and gluten free [14]