Proteins patients with metabolic disorders

In patients with metabolic disorders, adequate protein and indispensable amino acid intakes are needed to promote anabolism, prevent protein and amino acid insufficiency, and promote normal growth and development. Risk of protein over-restriction is a serious concern and can lead to protein-energy malnutrition and poor growth... 

More protein from medical food may be needed. Given the rapid oxidation of amino acids compared to intact protein sources, protein requirements for patients with metabolic disorders are often higher than recommended for the general population (Chap. 7). If excess energy intake is a concern, adding a medical food concentrated in protein can inaease total pro-... 

Fatal hereditary disorder that typically presents in the neonatal period. Clinical features include an array of hepatic, renal and neurological dysfunctions. Patients with Zellweger syndrome rarely survive the first year of life. The disease is caused by mutations in the Pex proteins leading to an defective import of peroxisomal matrix proteins and consequently to a loss of most peroxisomal metabolic pathways. 

Shinohara E, Yamashita S, Kihara S, Hirano K, Ishigami M, Arai T, Nozaki S, Kameda-Takemura K, Kawata S, Matsuzawa Y. Interferon alpha induces disorder of lipid metabolism by lowering postheparin lipases and cholesteryl ester transfer protein activities in patients with chronic hepatitis C. Hepatology 1997 25(6) 1502-6. 

Isoniazid (INH, Laniazid, Nydrazid, other names) is one of the primary drugs used to treat tuberculosis.24,61 Although the exact mechanism of action is unknown, this drug appears to interfere with several enzymatic pathways involving protein, lipid, carbohydrate, and nucleic acid metabolism in susceptible bacteria. Adverse reactions to isoniazid are common, and patients may develop disorders such as hepatitis and peripheral neuropathies. 

Bone and mineral disorders are common in patients with renal disease. Therapy and calcium metabolism of these patients are best evaluated with the determination of free calcium because of alterations in protein, pH, protein binding of calcium, and calcium complexes with organic and inorganic anions. Total and adjusted calcium concentrations are often invalid when compared with free calcium measurements... 

In dementia, as in any other CNS disorders, CYP genomics is a very important issue since in practice over 90 % of patients with dementia are daily consumers of psychotropics. Furthermore, some acetylcholinesterase inhibitors (the most prescribed antidementia drugs worldwide) are metabolized via CYP enzymes see Table 3). Most CYP enzymes display highly significant ethnic differences, indicating that the enzymatic capacity of these proteins... 

Mortality secondary to cardiovascular disease is 10 to 30 times greater in dialysis patients than in the general population. In addition to traditional cardiac risk factors such as diabetes, hypertension, hyperlipidemia, tobacco use, and physical inactivity, patients with kidney disease have other unique risk factors. Among these are hyper-homocysteinemia, elevated levels of C-reactive protein, increased oxidant stress, and hemodynamic overload. Complications previously discussed such as anemia and metabolic disorders of CKD are also contributory. In particular, arterial vascular disease (i.e., atherosclerosis) and cardiomyopathy are the primary types of cardiovascular disorders present in the CKD population. These disorders lead to development of ischemic heart disease and its manifestations including myocardial infarction. As a predominant comorbidity, cardiovascular disorders and their sequela are the leading cause of death in the ESKD population. ... 

Paddon-Jones et al. reported optimal protein synthesis when dietary protein was provided three times per day compared with the same total amount of protein given in various amounts [26, 29]. In patients with PKU, providing amino acid-based medical food throughout the day compared to a single dose of similar protein equivalents had a positive effect on protein synthesis [30]. A positive effect on plasma phenylalanine concentrations was also reported [30]. For patients with a metabolic disorder, providing medical food along with limited whole protein foods is most beneficial to optimize synthesis. 

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