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Biomedical subjects

D E Francis

Publications and source records attributed to D E Francis.

At least 19 recordsLinked to original sources
PubMed1995

Challenge confirmation of late-onset reactions to extensively hydrolyzed formulas in infants with multiple food protein intolerance.

BACKGROUND: Many infants with cow's milk protein intolerance have adverse reactions to soy, casein and whey hydrolysate formula and to other foods. The recent development of Neocate, a hypoallergenic, nutritionally complete infant formula composed of individual amino acids and other nutrients, has enabled these infants to be stabilized. OBJECTIVE: We observed the effect of food challenges in infants with reported hypersensitivity to hypoallergenic formulas. METHODS: Eighteen infants (median age, 7 1/2 months) were given Neocate formula for 2 months and then underwent a 7-day double-blind placebo-controlled challenge with the formula previously best tolerated. RESULTS: In 12 of the 18 infants irritability, vomiting, diarrhea, and/or eczema flares developed during the formula challenge. In two patients symptoms developed immediately, but in the remainder adverse reactions evolved within 7 days (range, 4 to 7 days). Adverse reactions were to soy formula (six patients), whey hydrolysate (two), and casein hydrolysate (four). When infants were 12 months of age, parents reported adverse reactions after the ingestion of other low allergen foods (median, six; from a panel of 10 such foods). CONCLUSION: A group of infants with late-onset adverse reactions to soy, extensively hydrolyzed casein, and whey formulas and to other foods has been identified. Neocate formula proved to be an effective substitute formula for these patients.

Amino Acids↗
PubMed1992

Maternal tyrosinaemia II: management and successful outcome.

A 25-year-old woman with tyrosinaemia type II was treated from the 5th week of pregnancy with a protein-restricted diet supplemented with a tyrosine/phenylalanine-free amino acid mixture. Tyrosine concentrations were maintained in the range 100-200 mumol/l by restricting natural protein intake to 0.16 g/kg per 24 h in early pregnancy, with increases up to 0.38 g/kg per 24 h in the last trimester. This treatment maintained plasma phenylalanine concentrations in the range 20-40 mumol/l. Maternal weight gain and fetal growth were normal, and the mother remained asymptomatic throughout the pregnancy. A normal infant was born at term with length, weight and head circumference between the 25-50th per centiles.

Adult↗
PubMed1991

Acute illness in maple syrup urine disease: dynamics of protein metabolism and implications for management.

Acute metabolic decompensation in maple syrup urine disease (MSUD) during otherwise minor illnesses has generally been presumed to result from massive release of leucine from protein catabolism. A stable isotope method based on the continuous infusion of (2H5)phenylalanine was used to measure protein metabolism in vivo in two children with MSUD during acute illness and when well. Net protein catabolism was greater in the unwell state (0.51 and 0.40 gm/kg per 24 hours in each child, respectively) than in the basal state (0.34 and 0.32). This rate of release of leucine from protein is compatible only with a slow (several days) rather than a dramatic rise in plasma leucine levels during acute illness in MSUD. Poor oral intake leading to a relative increase in time spent in the fasting state appears to be a more important determinant of increasing leucine levels than the catabolic effect of infection in itself. These factors suggested that branched-chain amino acid restriction should be commenced at the start of minor illness in children with MSUD, and that intake of other nutrients should be maintained or increased throughout the illness. A regimen based on these concepts was used during nine episodes of minor illness in two children with MSUD. Plasma branched-chain amino acid levels remained acceptable (less than 700 mumol/L) throughout each of these episodes. Dietary supplementation of this type may reduce the risk of metabolic decompensation during acute illnesses in children with MSUD.

Acute Disease↗
PubMed1991

Pregnancy in phenylketonuria: dietary treatment aimed at normalising maternal plasma phenylalanine concentration.

The transport characteristics of the placenta, which favour higher phenylalanine concentrations in the fetus than in the mother, and regression data of head circumference at birth against phenylalanine concentration at conception in maternal phenylketonuria (PKU), suggest that treatment of maternal PKU should ideally aim to maintain plasma phenylalanine concentration within the normal range throughout pregnancy. A patient with classical PKU was treated from before conception by aiming to maintain plasma phenylalanine concentration within the range 50-150 mumol/l and tyrosine within the range 60-90 mumol/l. The diet was supplemented with phenylalanine-free amino acids (100-180 g/day) and tyrosine (0-5 g/day). Plasma amino acid concentrations were monitored weekly by amino acid analyser. Dietary phenylalanine intake ranged from 6 mg/kg/day at conception to 30 mg/kg/day at delivery. Normal weight gain and fetal growth were maintained throughout the pregnancy. A normal baby was born at term with a head circumference of 35.5 cm; at 1 year of age no abnormality is detectable. These results show that with careful monitoring and compliance it is possible, and may be advisable, to maintain plasma phenylalanine concentration within the normal range in the management of PKU pregnancy.

Adult↗
PubMed1985

Amino acid and protein requirements in a preterm infant with classic phenylketonuria.

A preterm infant with classic phenylketonuria required rather less than 90 mg/kg of phenylalanine and between 270 and 290 mg/kg tyrosine daily to achieve a rate of weight gain of around 20 g/kg per day. Using Lofenalac as the low phenylalanine food, the intake of tyrosine, an essential amino acid for patients with phenylketonuria seemed to be limiting in respect of growth.

Amino Acids↗
PubMed1982

Outcome of maple syrup urine disease.

The outcome of 12 children with classical maple syrup urine disease is reviewed. All patients presented in the neonatal period at ages varying from 5 to 21 (median 8) days. The time taken to make the diagnosis ranged from 1 day to longer than 9 months (median 7 days). Each survived his initial illness but 3 died later after apparently mild infections. Three of the 12 patients had a spastic quadriplegia and 6 others abnormal neurological signs without clear cerebral palsy. The single most important factor determining the outcome appears to be the time taken to make the diagnosis after the first symptoms. Two patients were diagnosed within 24 hours of the first symptoms and one is of above average ability. The other is mildly retarded but control of the disease was poor in his first 4 years of life. Outcome is unpredictable if the delay is between 3 and 14 days. Two children are of normal ability but 6 others are retarded. A delay longer than 14 days is invariably associated with mental retardation and cerebral palsy. We conclude that early diagnosis is essential to improve the outcome of this condition.

Humans↗
PubMed1981

Hyperphenylalaninaemia of various types among three-quarters of a million neonates tested in a screening programme.

A total of 795 382 infants born in north London was screened for phenylketonuria using the Guthrie test between October 1969 and December 1978. During this period it became recognised that phenylketonuria is not a single disease entity but one that encompasses a number of disorders of differing clinical and biochemical severity. The overall incidence of persistent hyperphenylalaninaemia was of the order of 7 per 100 000 births (or 1 in 15 000) and all the early treated patients made normal developmental progress. During the study there was an appreciable fall in the incidence of uncomplicated transient hyperphenylalaninaemia with or without tyrosinaemia. This reduction coincided with the change in infant feeding practice in the UK which led to lower intakes of protein and phenylalanine. It was concluded that any infant found to have a persistent blood phenylalanine concentration of 240 mumol/1 (4 mg/100 ml) or greater should be followed closely.

Amino Acids↗
PubMed1977

Protracted diarrhoea in infancy. Analysis of 82 cases with particular reference to diagnosis and management.

Eighty-two cases of protracted diarrhoea in infancy presenting over a 6-year period have been analysed, with particular reference to diagnosis and management. The patients fell into 1 of 2 categories according to whether a specific diagnosis was established or not. A diagnosis (category 1) was established in 59 (72%), the commonest diagnoses being coeliac disease (33-2%), secondary disaccharide intolerance (12-2%), and cows' milk protein intolerance (12-3%). Other diagnoses included primary sucrase-isomaltase deficiency, Shwachman's syndrome, ulcerative colitis, ganglioneuroma, defective opsonization, staphylococcal pneumonia, and Hirschsprung's disease. Despite intensive investigation in diagnosis could not be established in 23 (28%) infants (category 2). Age of onset of symptoms in this group tended to be earlier than in category 1 patients, and 6 (7%) presented with diarrhoea dating from birth. Of particular interest in these 6 patients was the high incidence of associated extraintestinal anomalies, and of sibs who had died after protracted diarrhoea dating from birth. 4 of these 6 infants died, accounting for a mortality of 5% for the whole series. The remaining 17 (21%) patients in category 2 presented at a mean age of 4-9 weeks with a range of 1-18 weeks. All these 17 patients made an excellent response after institution of a chicken-based dietary formula, the details of which are presented. The pathophysiological mechanisms which may be operating in infants with protracted diarrhoea are discussed.

Animals↗
PubMed1975

Comparison of an amino acid mixture and protein hydrolysates in treatment of infants with phenylketonuria.

This study compares three feeding regimens for infants with phenylketonuria diagnosed by neonatal screening. Group 1 (five children) received Minafen (Cow & Gate) until they weighed twice their birthweights; Aminogran (Allen & Hanbury) was then added to the feeds in increasing amounts and replaced Minafen at between 8 and 10 months of age. Group 2 (five children) received Aminogran from the neonatal period. Group 3 (five children) received Minafen until they weighed twice their birthweights; Cymogran (Allen & Hanbury) was then added in increasing amounts and replaced Minafen at between 8 and 10 months of age. In all three groups growth was normal and control of phenylalanine levels satisfactory. During the first few months of life the Aminogran regimen proved more complicated and caused more practical difficulties than the regimens starting with Minafen. Later in the first year, when mixed feeding was introduced, and particularly when the bottle was exchanged for the cup, Aminogran had advantages over Cymogran because of its low calorie content, small bulk, and less unpleasant taste. At this age feeding problems were fewer and easier to manage with Aminogran than with Cymogran. A method of using Aminogran in the management of such problems is described. For these reasons, the regimen fed group 1, in which Minafen is used initially and then replaced by Aminogran, is preferred to the other two.

Alkaline Phosphatase↗