Protein-Restricted Diet Guide for OTC Deficiency

A protein-restricted diet is often used in managing patients with OTC deficiency, a urea cycle disorder that impairs the body's ability to eliminate ammonia. Ammonia is a toxic byproduct of protein metabolism. In OTC deficiency, the body cannot effectively convert ammonia into urea for excretion, leading to hyperammonemia (high ammonia levels in the blood), which can cause severe neurological damage.

The dietary management typically involves restricting protein intake to reduce the production of ammonia while ensuring adequate calories from carbohydrates and fats to prevent catabolism (the breakdown of body proteins). The goal is to minimize ammonia production while maintaining essential amino acid intake for growth and metabolism. In some cases, supplements such as citrulline or arginine are provided to support the urea cycle, along with ammonia scavengers and careful monitoring.

Long-term management requires balancing protein restriction to prevent hyperammonemia without causing malnutrition or growth impairment. Regular monitoring of blood ammonia, amino acids, and nutritional status is essential.

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