# Assessing the nutritional value and health risks of special low‑protein foods: narrative review

## TL;DR

This paper reviews low-protein foods used for metabolic disorders, highlighting their benefits and potential health risks, and suggests natural alternatives to improve nutrition.

## Contribution

The paper proposes exploring natural plant-based alternatives to enhance the nutritional quality and health outcomes of low-protein foods.

## Key findings

- SLPFs are crucial for managing inherited amino acid disorders but may cause gut dysbiosis and cardio-metabolic risks.
- Natural plant sources could improve SLPFs' nutritional quality and reduce adverse effects.
- Hydrocolloid fibers in SLPFs may suppress gut microbiota and contribute to obesity.

## Abstract

Special low-protein foods (SLPFs) are essential for patients with disorders of inherited amino acid metabolism that require lifelong dietary protein restriction to prevent severe neurocognitive effects and even death. Conditions such as phenylketonuria (PKU), tyrosinemia (TYR), maple syrup urine disease (MSUD), homocystinuria (HCU), and urea cycle disorders (UCD) depend on these foods to support metabolic control and dietary adherence. SLPFs provide satiety, energy, and help prevent catabolism, but their nutritional composition poses challenges. Most SLPFs are formulated using isolated starches as the primary macronutrient base. Hydrocolloid fibers are commonly added to improve texture, consistency, shelf life, and water or gas retention. These ingredients form the backbone of SLPFs production and are consistently used across different regions worldwide, reflecting a standardized approach to their formulation. However, their potential adverse effects include suppression of gut microbiota, gut dysbiosis, increased inflammatory markers, overweight, and obesity, all of which raise cardio‑metabolic risks. Strengthening the nutritional quality of SLPFs through natural plant sources may help mitigate their potential adverse outcomes while ensuring patients’ dietary needs are met. Therefore, it is important to explore natural low‑protein alternatives that can both support sustainable food production and promote long‑term health benefits.