The use of parenteral nutrition for the management of PKU patient undergoing chemotherapy for lymphoma: a case report.
The metabolic control of phenylalanine levels is a challenge during illness. We present the metabolic management of a 6 year 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 (360 μmol/L, 6 mg/dL) while 52.7% of Phe levels were below the recommended lower limit (120 μmol/L, 2 mg/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.
Division of Biochemical Diseases, Department of Pediatrics, UBC, Vancouver, Canada. rsalvarinova@cw.bc.ca
R
R
Salvarinova-Zivkovic
C
C
Hartnett
G
G
Sinclair
D
D
Dix
G
G
Horvath
Y
Y
Lillquist
S
S
Stockler-Ipsiroglu
D000970:Antineoplastic Agents / Q000009:adverse effects; D002648:Child; D019468:Disease Management; D006760:Hospitalization; D006801:Humans; D008223:Lymphoma / Q000188:drug therapy*; D008297:Male; D010288:Parenteral Nutrition; D010649:Phenylalanine / Q000097:blood*; D010661:Phenylketonurias / Q000097:blood / Q000139:chemically induced / Q000628:therapy*
D000970:Antineoplastic Agents; D010649:Phenylalanine