Metabolic disorders management

The authors reviewed the available literature and proposed a detailed surveillance and management plan for this metabolic disorder in patients with melanoma treated with interferon. 

For the first time in history there was clear, unambiguous clinical evidence, in humans, that symptoms of diabetes mellitus could be controlled with the exogenous administration of the active factor of the pancreas—insulin. Thus, replacement therapy with the newly discovered hormone, insulin, had arrested what was clearly an otherwise fatal metabolic disorder. From that point forward, diabetes mellitus (type 1) became a manageable disease by pharmacological intervention. 

The cost beneflt ratio associated with more aggressive management of metabolic disorders (e.g., hyperphosphatemia and hypercalcemia) and sHPT has not been formally evaluated. If the associated complications such as vascular and soft tissue calcifications that may increase morbidity and hospitalizations can be significantly reduced, the additional medication costs may ultimately be of minimal consequence. 

A number of physicians are using a commonsense approach to the management of these disorders. Logic tells us that if a person cannot harvest energy from carbohydrates in the diet, perhaps a diet high in protein and lipids might be beneficial. As with any condition of this sort, it is important to consult a physician who understands the metabolic disorder and who will design and supervise a customized diet. 

Acosta PB. Nutrition management of patients with inherited metabolic disorders. Acosta PB, editor. Jones and Bartlett Publishers, LLC, Sudbury, Massachusetts 2010. p. 476. 

The goals of nutrition management are to prevent the accumulation of ammonia, normalize plasma amino acids, and promote normal growth and development (Box 15.2). The treatment of UCD differs from other metabolic disorders with respect to protein intake. In UCD total protein is... 

Although there is limited experience in managing pregnancies in these disorders, it has become apparent that the risk for metabolic decompensation increases for the mother, especially during the postpartum period when protein catabolism is greatest. However, unlike in PKU, it appears that the infant may not be at increased risk of adverse outcomes in these other protein metabolism disorders. This chapter will review what has been learned about managing pregnancies in these intoxication disorders. 

Nutrition Management of Inherited Metabolic Disorders Lessons from Metabolic University http //www.imd-nutrition-management.com (online diet calculations)... 

This book contains only subject matter covered at MU, and the chapters are authored by the experts who presented the material. Therefore, it is not a comprehensive treatise on IMD but rather a textbook on the most frequently encountered challenges in IMD nutrition. The book contains introductory chapters on nutrition and metabolism principles common to many metabolic disorders and disease-specific chapters on disorders of amino acid, fat, and carbohydrate metabolism. Appendix M contains an overview of nutrition management of IMD including those disorders for which there is not a specific chapter. 

James WP, Avenell A, Broom J, Whitehead J. A one-year trial to assess the value of orlistat in the management of obesity. Int. J. Obesity Relat. Metabol. Disord. 1997 21 (suppl 3) S24-30. 

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