title: Inborn errors in metabolism and 4-boronophenylalanine-fructose-based boron neutron capture therapy.
abstract: Infusions of boronophenylalanine-fructose complex (BPA-F), at doses up to 900 mg/kg of BPA and 860 mg/kg of fructose, have been used to deliver boron to cancer tissue for boron neutron capture therapy (BNCT). In patients with phenylketonuria (PKU), phenylalanine accumulates, which is harmful in the long run. PKU has been an exclusion criterion for BPA-F-mediated BNCT. Fructose is harmful to individuals with hereditary fructose intolerance (HFI) in amounts currently used in BNCT. The harmful effects are mediated through induction of hypoglycemia and acidosis, which may lead to irreversible organ damage or even death. Consequently, HFI should be added as an exclusion criterion for BNCT if fructose-containing solutions are used in boron carriers. Non-HFI subjects may also develop symptoms, such as gastrointestinal pain, if the fructose infusion rate is high. We therefore recommend monitoring of glucose levels and correcting possible hypoglycemia promptly. Except for some populations with extremely low PKU prevalence, HFI and PKU prevalences are similar, approximately 1 or 2 per 20,000.
authors.affiliation: HUCH Institute, PL 105, FIN-00029 HUS, Helsinki, Finland.
authors.forename: Juha
authors.initials: J
authors.lastname: Laakso
authors.forename: Inkeri
authors.initials: I
authors.lastname: Ruokonen
authors.forename: Risto
authors.initials: R
authors.lastname: Lapatto
authors.forename: Merja
authors.initials: M
authors.lastname: Kallio
mesh_terms: D015746:Abdominal Pain / Q000209:etiology*; D000138:Acidosis / Q000209:etiology; D001896:Boron Compounds / Q000008:administration & dosage / Q000009:adverse effects*; D016754:Boron Neutron Capture Therapy / Q000009:adverse effects* / Q000379:methods; D005632:Fructose / Q000008:administration & dosage / Q000009:adverse effects* / Q000031:analogs & derivatives*; D005633:Fructose Intolerance / Q000150:complications* / Q000378:metabolism; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology / Q000517:prevention & control; D008661:Metabolism, Inborn Errors / Q000150:complications* / Q000378:metabolism; D009369:Neoplasms / Q000150:complications / Q000532:radiotherapy*; D010661:Phenylketonurias / Q000150:complications* / Q000378:metabolism
chemical_list: C089739:4-boronophenylalanine-fructose; D001896:Boron Compounds; D005632:Fructose