ArticleNutrients2021
Food Preferences of Patients with Citrin Deficiency.
Article in Nutrients, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed.
- Challenges in dietary management: a qualitative study on caregivers of preschool and school-age children with citrin deficiency.Orphanet journal of rare diseases · 2025Article
- Current Understanding of Pathogenic Mechanisms and Disease Models of Citrin Deficiency.Journal of inherited metabolic disease · 2025Review
- Citrin Deficiency: Clinical and Nutritional Features.Nutrients · 2023Review
- Pathogenic variants of the mitochondrial aspartate/glutamate carrier causing citrin deficiency.Trends in endocrinology and metabolism: TEM · 2022Review
- Obstacles to home-based dietary management for caregivers of children with citrin deficiency: a qualitative study.Orphanet journal of rare diseases · 2022Article
- Characteristics of patients with neonatal intrahepatic cholestasis caused by citrin deficiency in China: long-term follow-up outcomes.Jornal de pediatriaArticle
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Authors and funding
10 authors.
Funding
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Abstract
Citrin deficiency is characterized by a wide range of symptoms from infancy through adulthood and presents a distinct preference for a diet composed of high protein, high fat, and low carbohydrate. The present study elucidates the important criteria by patients with citrin deficiency for food selection through detailed analysis of their food preferences. The survey was conducted in 70 citrin-deficient patients aged 2-63 years and 55 control subjects aged 2-74 years and inquired about their preference for 435 food items using a scale of 1-4 (the higher, the more favored). The results showed that the foods marked as "dislike" accounted for 36.5% in the patient group, significantly higher than the 16.0% in the controls. The results also showed that patients clearly disliked foods with 20-24 (% of energy) or less protein, 45-54% (of energy) or less fat, and 30-39% (of energy) or more carbohydrate. Multiple regression analysis showed carbohydrates had the strongest influence on patients' food preference (β = -0.503). It also showed female patients had a stronger aversion to foods with high carbohydrates than males. The protein, fat, and carbohydrate energy ratio (PFC) of highly favored foods among patients was almost the same as the average PFC ratio of their daily diet (protein 20-22: fat 47-51: carbohydrates 28-32). The data strongly suggest that from early infancy, patients start aspiring to a nutritional balance that can compensate for the metabolism dissonance caused by citrin deficiency in every food.
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