Document Detail


The use of parenteral nutrition for the management of PKU patient undergoing chemotherapy for lymphoma: A case report.
MedLine Citation:
PMID:  22305856     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
The metabolic control of phenylalanine levels is a challenge during illness. We present the metabolic management of a 6year old boy with classical PKU who was diagnosed with stage III intraabdominal Burkit's lymphoma and underwent surgical resection and chemotherapy. The metabolic control during chemotherapy was achieved by the use of parenteral custom made amino acid solution and pro-active adjustment of intake. From the 94 obtained plasma phenylalanine (Phe) levels, 18.4% were above our clinic's recommended upper limit (360μmol/L, 6mg/dL) while 52.7% of Phe levels were below the recommended lower limit (120μmol/L, 2mg/dL). Phe levels above recommended range were associated with low caloric/protein intake, while levels below recommended range reflected the difficulty in achieving the full prescribed Phe intake. We recommend early institution of custom made amino acid solution with maximum amino acid content and caloric intake to provide optimal phenylalanine control. Administration of phenylalanine via regular intravenous amino acid solution may assist in avoiding low Phe levels when prescribed intake is compromised due to vomiting and other disease related illnesses. Use of custom made, phenylalanine free amino acid solution proved beneficial in the management of blood phenylalanine levels in a PKU patient during chemotherapy for Burkitt lymphoma.
Authors:
R Salvarinova-Zivkovic; C Hartnett; G Sinclair; D Dix; G Horvath; Y Lillquist; S Stockler-Ipsiroglu
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-1-16
Journal Detail:
Title:  Molecular genetics and metabolism     Volume:  -     ISSN:  1096-7206     ISO Abbreviation:  -     Publication Date:  2012 Jan 
Date Detail:
Created Date:  2012-2-6     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9805456     Medline TA:  Mol Genet Metab     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Elsevier Inc. All rights reserved.
Affiliation:
Division of Biochemical Diseases, Department of Pediatrics, UBC, Vancouver, Canada.
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