Orphanet Journal of Rare Diseases

Table 2 Summary of dietary treatment including tripheptanoin (C7) in 12 Austrian LC-FAOD patients

From: Long-term experience with triheptanoin in 12 Austrian patients with long-chain fatty acid oxidation disorders

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
Patient	Age at start of C7 therapy (years)	Total duration of C7 therapy (years)	Reported adverese events	g/day	g/kg/ day	C7 in diet [%] of total calories	MCT in diet [%]of total calories	Total amount fat including C7 [%]	Daily calorie intake (kcal)	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 meal^e	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	1.2^a	Abdominal pain^a	20	0.52	13.0	8.0	30	1900	22:00	Late meal	38.0	99	131	30	22.0	95
10	3.7	1.3^b	Abdominal pain^b	0^b	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	2.2^c	Abdominal pain^c	60	0.70	20	0.0	up to 30	2520	22:00	Late meal and 80 g uncooked corn starch	84.0	82^d	180	55^d	26.0	89^d
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]

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ISSN: 1750-1172

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