CLARA M. AMBRUS, M.D., Ph.D.; SIDNEY ANTHONE, M.D.; CSABA HORVATH, Ph.D.; KRISHNA KALGHATGI, Ph.D.; AMOL S. LELE, M.D.; GIORGINA EAPEN, M.D.; JULIAN L. AMBRUS, M.D., Ph.D.; A. JOHN RYAN, M.D.; PHILIP LI, Ph.D.
AMBRUS CM, ANTHONE S, HORVATH C, KALGHATGI K, LELE AS, EAPEN G, et al. Extracorporeal Enzyme Reactors for Depletion of Phenylalanine in Phenylketonuria. Ann Intern Med. 1987;106:531-537. doi: 10.7326/0003-4819-106-4-531
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Published: Ann Intern Med. 1987;106(4):531-537.
Multitubular enzyme reactors with immobilized enzymes were developed to achieve depletion of circulating substrate by extracorporeal means. To act as prototypes, reactors were prepared with immobilized L-phenylalanine ammonia-lyase, an enzyme that metabolizes phenylalanine to trans-cinnamic acid and ammonia without the need for a coenzyme. We report the first application of phenylalanine ammonia-lyase reactors in an extracorporeal circulation system in a patient with phenylketonuria. A phenylalanine level of 1.82 mmol/L (for the last 6 years) decreased to 1.24 mmol/L after 5.5. hours of treatment, without the enzyme entering the circulation. Total phenylalanine depletion from blood and tissue stores was estimated at 1800 mg. The hemodialysis-like procedure proved to be without side effects, specific for phenylalanine, and suitable in the management of pregnant women with phenylketonuria and late-onset hyperphenylalaninemia. The extracorporeal use of enzyme reactors for temporary enzyme replacement represents a new, safe, and effective therapeutic modality.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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