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Extracorporeal Enzyme Reactors for Depletion of Phenylalanine in Phenylketonuria

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.; and PHILIP LI, Ph.D.
[+] Article and Author Information

Grant support: in part by grant 6-355 from the March of Dimes, grant FD-R000051 from the Orphan Grant Division of the Food and Drug Administration, and a grant from the Rich Foundation, Buffalo, New York.

▸Requests for reprints should be addressed to Clara M. Ambrus, M.D.; Department of Gynecology-Obstetrics, Children's Hospital of Buffalo, 140 Hodge Ave.; Buffalo, NY 14222.


Buffalo and West Seneca, New York; and New Haven, Connecticut


© 1987 American College of PhysiciansAmerican College of Physicians


Ann Intern Med. 1987;106(4):531-537. doi:10.7326/0003-4819-106-4-531
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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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