Branched-chain amino acid-free parenteral nutrition in the treatment of acute metabolic decompensation in patients with maple syrup urine disease

Article Abstract:

Maple-syrup urine disease results from a deficiency in the alpha-keto acid dehydrogenase enzyme complex. Patients with this enzymatic defect cannot properly metabolize branched-chain amino acids (certain building-blocks of proteins) such as leucine, isoleucine, and valine, which results in the accumulation of these amino acids and the presence of keto acids in blood, tissues, and urine. The keto acids give the urine its characteristic maple syrup smell from which the disorder gets its name. The chronic metabolic imbalance results in growth failure and psychomotor retardation; acute decompensation resulting from fasting or stress may result in acidosis, vomiting, and potentially fatal encephalopathy (brain dysfunction). The disease can be treated by careful regulation of the diet. When the dietary branched-chain amino acids are kept to a minimum, virtually all are incorporated into proteins and cannot accumulate in the blood. For acutely ill patients, the goal of therapy is to reduce the blood levels of branched-chain amino acids as rapidly as possible. The authors report their experience using parenteral (intravenous) feeding in the treatment of children with acute metabolic decompensation of maple syrup urine disease. Five such patients were treated with intravenous nutrition especially formulated without branched-chain amino acids. This resulted in an immediate drop in the blood levels of leucine, which is the most common of the three branched-chain amino acids. Blood levels may be as high as 20 times normal, however, and five days may be required to bring the leucine levels close to the normal range. The authors recommend the widespread adoption of modified parenteral feeding for acutely ill patients who cannot tolerate conventional feeding. Considering the seriousness of the cerebral edema which can develop if the acute decompensation is not rapidly treated, the authors recommend keeping a liter of branched-chain amino acid-free solution on hand at the hospital pharmacy; the cost of this safeguard should only be about $200 per month. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Branched chain amino acids, Metabolism, Inborn errors of, Inborn errors of metabolism, Amino acid metabolism, Inborn errors of, Inborn errors of amino acid metabolism, Maple syrup urine disease

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Outpatient parenteral antimicrobial-drug therapy

Article Abstract:

Outpatient intravenous antimicrobial drug therapy can save on hospitalization costs. The patient must have an infectious disease that does not require hospitalization and there must be no safe oral drug. The drugs must be relatively stable and are delivered by catheters and infusion pumps. The catheters are placed in the arm or in a central vein such as the subclavian or internal jugular vein. Some can even be implanted subcutaneously so they are not visible. Complications of intravenous catheters include infection, hypersensitivity and blood clot formation.

Methods, Analysis, Equipment and supplies, Intravenous catheterization, Ambulatory medical care, Ambulatory care, Drug delivery devices

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Survival after treatment with phenylacetate and benzoate for urea-cycle disorders

Article Abstract:

A research proves that treatment with intravenous sodium phenylacetate and sodium benzoate lowers plasma ammonium levels and improves survival in patients suffering from urea-cycle disorders.

Health aspects, Sodium benzoate, Clinical report, Urea cycle

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