Biomedical subjects

C Hendriksz

Publications and source records attributed to C Hendriksz.

4 recordsLinked to original sources

PubMed2006

'Ready to drink' protein substitute is easier is for people with phenylketonuria.

UNLABELLED: In phenylketonuria (PKU), compliance with taking protein substitute is an issue in teenage and older patients. A 'ready to drink' protein substitute may overcome many of the practical issues associated with its administration. OBJECTIVE: To investigate the efficacy of a liquid protein substitute in a 6-week, three-part, randomized, crossover, controlled study. METHODS: 27 subjects (15 female; 12 male) with PKU with a median age of 30 years (range 8-49 years) were recruited. One subject withdrew from the study. Their median daily dose of protein equivalent was 60 g (range 45-75 g). In parts 1 and 2, subjects were randomized to either a liquid or a powder protein substitute with the same nutritional composition per unit (each 130 ml liquid pouch or 25 g powder sachet contained 15 g protein equivalent). In part 3, subjects chose liquid, powder or a combination of both. Weekly blood phenylalanine (Phe) concentrations were estimated, and during weeks 2, 4 and 6 subjects completed a daily questionnaire on administration issues. RESULTS: All but one of 26 subjects chose the liquid in part 3 as either their sole (69%, n = 18) or partial source (28%, n = 7) of protein substitute. Blood Phe concentrations were significantly better on the liquid (p = 0.03). With the liquid protein substitute, subjects were less self-consciousness (p = 0.003) and found it easier to take away from home (p = 0.001). Overall, the liquid was easier (p < 0.0001), more convenient (p = 0.002) and resulted in less wastage of protein substitute (p = 0.001). CONCLUSION: Liquid protein substitute was popular and efficacious, reduced self-consciousness and overall improved compliance of teenagers and adults with PKU.

Adolescent↗

PubMed2006

Protein substitute dosage in PKU: how much do young patients need?

BACKGROUND: The optimal dose of protein substitute has not been determined in children with phenylketonuria (PKU). AIM: To determine if a lower dose of protein substitute could achieve the same or better degree of blood phenylalanine control when compared to the dosage recommended by the UK MRC.(1) METHODS: In a six week randomised, crossover study, two doses of protein substitute (Protocol A: 2 g/kg/day of protein equivalent; Protocol B: 1.2 g/kg/day protein equivalent) were compared in 25 children with well controlled PKU aged 2-10 years (median 6 years). Each dose of protein substitute was taken for 14 days, with a 14 day washout period in between. Twice daily blood samples (fasting pre-breakfast and evening, at standard times) for plasma phenylalanine were taken on day 8-14 of each protocol. The median usual dose of protein substitute was 2.2 g/kg/day (range 1.5-3.1 g/kg/day). RESULTS: When compared with control values, median plasma phenylalanine on the low dose of protein substitute increased at pre-breakfast by 301 mumol/l (95% CI 215 to 386) and in the evening by 337 micromol/l (95% CI 248 to 431). On the high dose of protein substitute, plasma phenylalanine concentrations remained unchanged when compared to control values. However, wide variability was seen between subjects. CONCLUSIONS: A higher dosage of protein substitute appeared to contribute to lower blood phenylalanine concentrations in PKU, but it did have a variable and individual impact and may have been influenced by the carbohydrate (+/- fat) content of the protein substitute.

Child↗

PubMed2006

Home delivery of dietary products in inherited metabolic disorders reduces prescription and dispensing errors.

UNLABELLED: In the UK, for patients with inherited metabolic disorders (IMD) the traditional system for acquiring essential dietary products [patient prompted prescriptions generated by a medical general practitioner (GP) and dispensed by a chemist] is problematic. OBJECTIVE: To investigate the efficacy of a home delivery service (HDS) for essential dietary products (EDP) (i.e. protein substitutes, milk replacements, energy and vitamin and mineral supplements) for subjects with IMD, particularly examining any prescription and dispensing errors, metabolic control and consumer satisfaction. METHODS: A prospective, controlled, home delivery trial for EDP was conducted in patients with IMD for 12 months. Sixty-two patients with IMD [50 with phenylketonuria (PKU); 12 with other IMD: aged 6 months-30 years] were recruited. Thirty subjects used a monthly HDS (Homeward: Nutricia) to receive EDP, 32 remained on the traditional system. Each month, the HDS checked home stock levels of EDP, obtained their prescriptions directly from GP's, and then delivered them to the subjects' homes. An independent researcher completed monthly telephone interviews with patients/parents about any EDP prescription errors or delay in receipt. RESULTS: Incorrect protein substitute was dispensed once by the HDS compared with nine subjects who had 12 errors in the control group (P = 0.01); incorrect flavours of protein substitute were dispensed to the home delivery group once compared with eight subjects getting 11 errors via the chemist (P = 0.03). The HDS delayed delivery of protein substitute for one subject on three occasions compared with 39 occasions in 16 subjects via the chemist (P = 0.001). In patients with PKU, plasma phenylalanine control deteriorated in the control group (P < 0.05) but not in the HDS group. CONCLUSIONS: The long-term use of a HDS for EDP in IMD is safer, effective and more reliable than conventional systems.

Adolescent↗

PubMedDate not supplied

Breast feeding in IMD.

Breast feeding has proven benefits for many infants with inherited metabolic disorders (IMDs) but, with the exception of phenylketonuria, there are few reports in other conditions. A questionnaire, completed by dietitians and clinicians from 27 IMD centres from 15 countries (caring for a total of over 8000 patients with IMDs on diet) identified breast feeding experience in IMD. Successful, demand breast feeding (in combination with an infant amino acid formula free of precursor amino acids) was reported in 17 infants with MSUD, 14 with tyrosinaemia type I, and 5 with homocystinuria. Eighty-nine per cent were still breast fed at 16 weeks. Fewer infants with organic acidaemias were demand breast fed (7 with propionic acidaemia; 6 with methylmalonic acidaemia and 13 with isovaleric acidaemia) (usually preceded by complementary feeds of a protein-free infant formula or infant amino acid formula free of precursor amino acids). Only 12 infants with urea cycle disorders were given demand breast feeds, but this was unsuccessful beyond 8 days in CPS deficiency. Further work is needed in developing guidelines for feeding and for clinical and biochemical monitoring for breast-fed infants with IMDs.

Amino Acid Metabolism, Inborn Errors↗