Dietary practices in propionic acidemia: A European survey

Area of Science:

• Biochemistry
• Metabolic Disorders
• Nutritional Science

Background:

• The optimal dietary management for propionic acidemia (PA) is not definitively established.
• Natural protein restriction and adequate energy intake are crucial for managing PA.

Purpose of the Study:

• To investigate and describe the dietary practices employed in managing PA patients across Europe before the official European PA guidelines were published.

Main Methods:

• A cross-sectional survey was conducted in 2014, involving 27 questions.
• The survey targeted European metabolic disorder (IMD) dietitians and health professionals.
• Data were collected from 47 centers across 14 European countries, representing 186 PA patients.

Main Results:

• Most centers (77%) provided total protein intake meeting WHO/FAO/UNU (2007) safe levels through a combination of protein precursor-free L-amino acid supplements (PFAA) and natural protein.
• PFAA were utilized in 81% of centers to supplement protein intake, contributing a median of 44% to the total requirement.
• A significant proportion of patients (74%) received natural protein intakes below recommended safe levels for their age groups, and 63% were tube-fed, primarily via gastrostomy.

Conclusions:

• European centers frequently use PFAA, but natural protein intake is often below recommended safe levels.
• Further longitudinal, multi-center, prospective case-controlled studies are essential to determine optimal dietary management strategies for PA.
• The metabolic complexity of PA and limited patient numbers per center present significant challenges for such research.