INBORN ERRORS OF METABOLISM IN AYURVEDA WITH SPECIAL REFERENCE TO PRAMEHA: A COMPREHENSIVE REVIEW

Inborn errors of metabolism (IEM) are a group of genetic disorders characterized by defects in enzymes, transporters, or cofactors that disrupt normal biochemical pathways. These disorders lead to the accumulation of toxic metabolites or deficiencies of essential biomolecules, resulting in a wide range of clinical manifestations. Ayurveda, the ancient Indian system of medicine, describes similar metabolic disturbances under the broad concept of Prameha, a disorder primarily characterized by abnormal urine output and systemic metabolic dysfunction. The process of Ahara Pachana (food metabolism) in Ayurveda is divided into Avastha Paka (digestive transformation) and Nistha Paka (tissue assimilation), which closely resemble enzymatic digestion and intracellular metabolism, respectively. Disruptions in these processes lead to the formation of Ama (partially digested toxic substances) and Sama (pathologically altered tissue metabolites), which manifest as Prameha. The three major subtypes of Prameha—Kaphaja, Pittaja, and Vataja—are correlated with protein, carbohydrate, and lipid metabolic errors, respectively. Kaphaja Prameha resembles disorders of amino acid metabolism (e.g., phenylketonuria), Pittaja Prameha aligns with glucose metabolism defects (e.g., diabetes mellitus), and Vataja Prameha corresponds to severe lipid metabolism derangements (e.g., diabetic ketoacidosis). Ayurvedic management strategies, including Deepana-Pachana (digestive correction), Shamana Chikitsa (palliative therapy), and personalized dietary modifications, offer a holistic approach to managing metabolic disorders. This review highlights the potential of Ayurveda in addressing both acquired and inborn metabolic errors through a combination of traditional wisdom and modern scientific understanding.