We retrospectively collected data from 12 patients with LC-FAOD from three Austrian metabolic centers by reviewing their medical history. Nine patients were cared for in Innsbruck, one in Salzburg (Patient 10) and one in Vienna (Patient 11); outcome of one additional patient in Vienna (Patient 12) is reported separately. Data obtained from medical records cover the patients’ history until May 2020. Epidemiological data include sex, age, diagnosis, onset of disease, time to diagnosis, family history, newborn screening results, symptoms, dietary management, and initiation of triheptanoin treatment. Furthermore, number of hospital admissions (in days per year), number of episodes of rhabdomyolysis (defined as peak creatine kinase concentration above 500 U/I), as well as concomitant short- and long-term complications (cardiomyopathy, hepatopathy and retinopathy) were assessed. Total fat intake and quality of fats was obtained from dietary protocols. Additionally, late night feeds and nasogastric or PEG-tube feeding were assessed. Neurological outcome was documented according to self-reported school performance and need for physical support (i.e. wheelchair-bound). Weight, height and BMI percentiles were calculated according to reference data sets [12, 13]. Main outcome parameter was total hospitalization days per year before and after treatment with triheptanoin.

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