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M Lilburn

Publications and source records attributed to M Lilburn.

8 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↗
PubMed2005

A new protein substitute for adolescents and adults with maple syrup urine disease (MSUD).

AIM: The aim was to study the efficacy and acceptability of MSUD Express in adolescent and adult patients with maple syrup urine disease (MSUD). BACKGROUND: There are difficulties associated with current protein substitutes designed for adolescents and adults with MSUD. This can affect their metabolic control. MSUD Express is a new low-volume, nutritionally complete protein substitute, free from leucine, isoleucine and valine and designed specifically for older patients with MSUD. METHODS: Four patients with MSUD were included in the trial (aged 16-41 years). Product dosage was 4 x 25 g sachets per day, providing 1264 kJ and 72 g amino acids: more than adequately meeting requirements for all micronutrients. RESULTS: At the start of the study, the mean (range) of the previous eight leucine concentrations (on MSUD Aid III) was 564 micromol/L (430-817 micromol/L) and during the study (on MSUD Express) was 382 micromol/L (181-603 micromol/L). The average percentage fall in mean leucine concentrations was 32%. In patients 2, 3 and 4, low leucine levels resulted in an increase in the natural protein allowance per day. MSUD Express was rated as 'excellent' or 'good' by all patients on the basis of appearance, taste, smell and texture. All patients found the product 'very easy' to prepare and found it 'easy' or 'very easy' to take outside the home. CONCLUSION: All patients thought the preparation of MSUD Express was 'very easy'. Metabolic control (mean leucine concentrations) improved in all of the patients. In three patients this meant that an increase in the amount of natural protein in the diet was possible. All patients elected to stay on MSUD Express after the trial period. MSUD Express appears to be an effective and acceptable product, offering an alternative to current protein substitutes available for older people with MSUD.

Adolescent↗
PubMed2004

A new, low-volume protein substitute for teenagers and adults with phenylketonuria.

Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low-volume (amino acids 72 g/100 g), low-carbohydrate, phenylalanine-free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty-three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8-37 years) took the substitute for 8 weeks. A 3-day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin-puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140-180) daily to 100 g (range 100-125) daily ( p <0.001). Median change in energy intake decreased by a median of 10% (95% CI 2.0 to 18.0) when compared to intake on original protein substitute. On PKU Express, the intakes of all nutrients exceeded the dietary reference values but none was excessively high. Blood phenylalanine decreased by a mean of 37 micromol/L (95% CI-27 to 102) during the trial. Body mass index decreased in 40% of subjects. Changes in blood phenylalanine or body mass index were not statistically significant. Most of the nutritional, haematological and biochemical indices stayed within normal reference ranges for the analytes studied. Sixteen (70%) of the subjects had low plasma selenium at the start, but only 13 (57%) at the study end. Plasma vitamin B12 was high in 8 subjects at the start of the study and 9 at the end. Twenty-one subjects (96%) stated that the product was convenient and easy to prepare. However, 7 (32%) described the smell and 9 (46%) the texture as the same as or worse than those of previous protein substitutes. Because of the use of the premeasured sachets, some subjects were able to prepare their own protein substitute for the first time. PKU Express is a safe, efficacious, protein substitute that significantly reduces the daily volume of prescribed protein substitute.

Adolescent↗
PubMed1996

Maternal phenylketonuria. A study from the United Kingdom.

By November 1994, 39 pregnancies had been completed in phenylketonuric mothers. Dietary control was post-conception in 6 and 2 of these offspring died of congenital heart disease and 1 other needed surgery for coarctation. There were no heart defects in the 34 offspring of the 33 pregnancies following preconception diet controlled by Guthrie assays of maternal phenylalanine three (Phe) weekly. These Phe results were analysed by trimester for the means, the number of days over 300 mumol/l or below 60 mumol/l. Generally good control was achieved suggesting the UK guidelines drawn up by the MRC Working Party are broadly achievable but excessively high and low values occur intermittently in many pregnancies both of which may adversely affect the fetus. Though developmental assessment scores at 1 year were over 100 in all but five, early outcome results suggest that intellectual development may still be impaired at 4 years. Until much more information is available caution is still needed in discussing outcome with phenylketonuria patients who wish to conceive.

Child Development↗
PubMed1996

Phenylketonuria: treatment in adolescence and adult life.

In our clinic the decision on whether to continue with dietary treatment of phenylketonuria or not is left to each adolescent and adult patient after the advantages and disadvantages, as discussed in this paper, of continuing diet have been presented to them. As a result 61 of 132 patients have stopped diet or declined to restart and only 4 of them have phenylalanine values below 1000 mumol/l. Seventy-one patients have remained on diet or started again with phenylalanine values below 1000 mumol/l in 58 of them. This series of 132 excludes women who returned to diet to conceive.

Adolescent↗
PubMed1977

25-Hydroxycholecalciferol absorption in steatorrhoea and postgastrectomy osteomalacia.

Post-absorption levels of 25-hydroxy vitamin D (25-OHD) after oral administration of 25-hydroxycholecalciferol (25-OHD3) were measured in 11 subjects. Five had presented with steatorrhoea of various causes while six had post-gastrectomy osteomalacia. Post-absorption levels of 25-OHD were low in four of the patients with steatorrhoea but normal in five of those with post-gastrectomy osteomalacia. There was a significant inverse correlation between peak post-absorption 25-OHD levels and faecal fat excretion. All patients with active post-gastrectomy osteomalacia had subnormal baseline plasma 25-OHD levels, which indicates that the condition is due to a deficiency of vitamin D. Only two of the patients with osteomalacia had estimated dietary vitamin D intakes ofer 1-75 microng/day. These findings suggest that an oral 25-OHD absorption test may be a valuable measure of small intestinal function and that poor dietary vitamin D intake rather than impaired absorption of the vitamin may be the major cause of post-gastrectomy osteomalacia.

Adult↗