Diet and fat in premature infants.
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Biomedical subjects
Publications and source records attributed to D Burman.
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Three cases of Huntington's chorea with onset before age 10 years are reported. Each child presented with rigidity and indistinct speech, and there was progressive deterioration. Necropsy examination confirmed the diagnosis in 2 of them. A review of reports showed a further 43 cases with onset before 10 years. The rigid variety of disease was seen most often, but isolated chorea and isolated progressive mental deterioration occurred. Fits were common but occurred late and were often difficult to control. Dysarthria was common and occurred early. The duration of illness was very variable and ranged from 2 to 38 years. Symptoms can occur in a child before appearing in the affected parent who is most likely to be the father. Affected siblings develop the disease early, often in the first decade. Siblings of patients with onset before age 10 years who are unaffected by age 25 years had only an 8% chance of developing the disease, compared with a 50% chance in unselected at risk individuals of the same age.
Low birthweight babies fed standard modified cows' milk formulae are at risk from the high fluid intake needed for adequate nutrition, and very preterm babies often develop late hyponatraemia if the sodium intake fails to match large renal losses. A new cows' milk formula (Cow and Gate Prematalac) provides 120 kcal, 3.6 g protein, 7.5 g fat, and 4 mmol sodium in 150 ml. Ten low birthweight babies were fed the new formula at 150 ml/kg a day and compared with 12 similar babies fed a standard modified cows' milk formula (Wyeth SMA Gold Cap) at 180 ml/kg a day. All the babies grew at intrauterine rates and there was no difference in clinical course. None fed the new formula developed hypernatraemia, oedema, or dehydration and none fed the standard formula developed hyponatraemia. The Prematalac group safely excreted the increased osmotic load and had a higher urinary sodium concentration which should protect less mature preterm infants from late hyponatraemia.
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Two groups of white, primiparous women and their babies were studied: one group in April 1979 and the other in September 1979. They were selected to be as near normal as possible. In each case maternal and cord blood samples were taken at delivery and analysed for serum 25-hydroxycholecalciferol (25-OHD), calcium, magnesium, phosphate, alkaline phosphatase, total protein, and albumin. Follow-up was by questionnaire at 6 weeks. The study showed a highly significant increase in maternal and cord serum 25-OHD levels in September. The few mothers who had taken vitamin D supplements had significantly higher serum 25-OHD values. Some of the unsupplemented women studied in April had low serum 25-OHD levels suggesting that oral vitamin D supplements should be given to pregnant white women in Britain, at least during the winter.
The clinical and radiological features of a patient with Kniest dysplasia, a form of metatropic dwarfism, are described. The patient excreted glycosaminoglycans (mucopolysaccharides) in normal amounts during infancy but subsequently showed abnormal keratan sulphate excretion. The significance of these findings and the possibility that Kniest dysplasia represent another mucopolysaccharidosis are discussed.
The protective effect of treating the skin of newborn infants with powders containing 1% chlorhexidine or 0.33% hexachlorophane was compared. Each was equally effective in preventing colonisation and infection by Staphylococcus aureus. In contrast, the skin became profusely colonised by coagulase-negative staphylococci, irrespective of the powder used. Venous blood concentrations of chlorhexidine were low or undetectable in the few infants whose blood was analysed.
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Low birth weight infants (246) entered a trial to folic acid supplementation from 3 weeks to 12 months of age. The folic acid group had significantly higher mean hemoglobin levels at 6 and 9 months of age but the differences were only about 0.5 gm/dl, there was no significant difference in hematocrit, and in both groups of infants the mean hemoglobin levels were higher than those of normal birth weight infants. The differences in hemoglobin, although statistically significant, are of uncertain clinical significance. Median red cell folate levels remained within the normal adult range in both groups of infants. A minority of infants in the untreated group had low red cell folate levels but this was usually temporary, corrected by dietary folate, and not associated with low hemoglobin. Weight gain was not affected by folic acid supplementation. The infants in this trial were fed with a milk preparation containing 3.5 microgram/100 ml of folic acid which is a similar concentration to that of human milk and we recommend that the folate content of milks fed to low birth weight infants should not fall below this level. We do not have sufficient grounds to recommend routine folic acid supplements for all low birth weight infants throughout the first year of life but there is a possibility that their folate intake may sometimes be suboptimal.
Details are given of all serologically confirmed Mycoplasma pneumoniae infections in children referred to Bristol hospitals during an epidemic lasting 18 months. 44 children, many below school age, had lower respiratory infections. The majority had cough and malaise which had failed to respond to antibiotics given before referral. Chest x-rays showed no pathognomonic features: segmental or patchy consolidation was common; 3 cases of lobar consolidation. Cold agglutinins were raised in 9 out of 12 cases. In the majority of cases the total leucocyte count was normal and the absolute neutrophil count raised. Mean duration of symptoms was 4.2 weeks (range 1-16). Treatment with erythromycin or tetracycline appeared to have little effect in most cases. Seven nonrespiratory manifestations were seen in 6 children. These were meningitis (2 cases), Stevens-Johnson syndrome (4 cases, 1 case complicated by toxic epidermal necrolysis), and acute haemolytic anaemia (1 case).
Two patients with phenylketonuria (PKU) requiring treatment were fed on low protein milks. Both had blood phenylalanine levels below 1200 micronmol/l (20mg/100 ml) until given a phenylalanine challenge. Phenylalanine content of mature breast milk may provide intakes similar to those used in treating PKU. Diagnosis of PKU is unlikely to be missed if screening is carried out on the sixth or seventh day of life because of higher phenylalanine in breast milk during the first week. Interpretation of screening tests requires knowledge of the infants' feeds and a blood phenylalanine above 360 micronmol/l (6 mg/100 ml) in the absence of tyrosinaemia requires careful investigation.
The effect of age, sex, birth weight and area of birth on blood phenylalanine has been studied in a group of 41 795 infants. The mean phenylalanine level in low birth weight infants was higher than in those of normal birth weight from six to over 43 days of age. There was a trend of increasing blood phenylalanine with age in both birth weight groups. No consistent difference was observed between the sexes. In several of the age and birth weight groups, infants living in urban areas had significantly higher blood phenylalanine levels than those in rural areas.
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