New review highlights principles of nutrition management of inherited metabolic disorders

Thanks to screening of newborns and newly developed therapies, the population of patients with inherited metabolic disorders has expanded. As these patients age and enter adulthood, diet therapy will be the mainstay of treatment for their disorders.

A new review, published today in Nutrition in Clinical Practice (NCP), a peer-reviewed, interdisciplinary journal of the American Society for Parenteral and Enteral Nutrition (A.S.P.E.N.) that publishes articles about the scientific basis and clinical application of nutrition and nutrition support, highlights the basic principles of chronic nutrition management of inherited metabolic disorders.

"Failure to follow specific dietary treatment can lead to complications and even death in individuals with metabolic disorders," said Jeanette Hasse, PhD, RD, FADA, CNSC, Editor-in-Chief, NCP. "This review not only summarizes key concepts about several inborn errors of protein, carbohydrate, and fat metabolism, but also outlines the main principles of dietary treatment. This is a 'must-read' article for any nutrition professional who may provide care to individuals with a metabolic disorder."

Dietary therapy is critical for normal growth and development, to prevent acute complications, and/or to prevent long-term complications of the given disorder. Therapy is customized for the specific disorder and individualized for each patient to promote compliance.

Because nutrition therapy for patients with inherited metabolic disorders is lifelong, registered dietitians trained in the area of rare metabolic disorders are an essential part of the multi-disciplinary team including nurses, physicians, social workers, and other healthcare providers that delivers care for these patients.