Effect of fenfluramine on autistic symptoms.
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Biomedical subjects
Publications and source records attributed to C J Rolles.
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An ACTH-producing thymic carcinoid tumour was diagnosed in a 10-year-old girl, 8 years after bilateral adrenalectomy for Cushing's syndrome. The peptides produced by the tumour were characterised thoroughly. High circulating levels of beta-endorphin and other peptides may have contributed to mood and behaviour disturbances.
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The diagnosis of cystic fibrosis is straight forward if the clinical findings and sweat sodium are considered together. We describe 3 patients where the diagnosis has proved difficult as both the clinical features and sweat sodium have varied between normal and abnormal.
We describe our technique of small bowel biopsy, which has been used on 190 occasions over a four year period. In 77%, the examination was completed within 10 minutes, and fluoroscopy times were less than 10 seconds in 75% of the cases. The technique is easily taught, and it has made the examination a minor procedure.
Bounty Services Ltd advertise commercial products on postnatal wards of hospitals throughout Britain by means of free gifts. Since the " Bounty lady" is apparently well known to nursing staff but may have gone unnoticed by paediatricians and obstetricians an investigation was conducted of the quality of the gifts and the nature of the company. With two exceptions the samples and promotional leaflets were sound, and mothers apparently enjoyed receiving them. Educational pamphlets distributed with the gifts were excellent, and cine films shown to the mothers were of good quality and informative about basic parental skills. Bounty , however, operates by exerting commercial pressure on new mothers at a time when they are most vulnerable and distributes materials among them without consultation with medical staff. Most of the mothers questioned assumed that the service was being provided by the NHS. The Bounty service may or may not be perceived as a satisfactory operation but details should be known to paediatricians and obstetricians; any action to be taken might be decided locally after discussion in individual hospitals or districts.
Resection of the terminal ileum for necrotizing enterocolitis is not uncommon in neonates requiring intensive care in the first weeks of life. They may therefore be at risk of vitamin B12 malabsorption, and later of vitamin B12 deficiency. A method of measuring B12 absorption is described and the results are given. This assessment should be part of the follow up for all these children.
The nutritional status and growth of infants in the pre-obese phase of the Prader-Willi Syndrome has not previously been investigated. In this study the daily energy and protein intake of a male infant with this syndrome was measured from the 21st to the 330th day of life, together with weekly weight and monthly height measurements. The primary source of food was expressed breast milk. Daily energy intake was 29-66 per cent below recommended amounts. Protein intake did not exceed 12 g per day. Body weight remained at or below the third centile with a marked downward trend at 6 months. The symptoms associated with the Prader-Willi Syndrome result in a lack of physical maturity; poor feeding exacerbates this situation.
The comparative nutritional merits of two infant milk formulae, Ready-to-Feed Premium Babyfood (Cow and Gate Ltd.) and Prematalac (Cow and Gate Ltd.) were examined--Prematalac is modified in an attempt to meet the specific requirements of the low birthweight infant. Nine premature infants were enrolled for two metabolic balance periods in a cross-over experimental design. At the onset the infants were randomly allocated one of two milks. Various anthropometric data were collected at the start and end of each 4 day balance. All infants received more energy and protein per kg body weight per day on Prematalac compared with Premium. Incremental weight, though satisfactory for both milks over the balance periods, was greater when Prematalac was fed compared with Premium (mean and (range) 46 (25-93), 19 (9-44) g per day respectively). No differences were detected for gain in length between the two milks. Mean percent digestibility of energy intake was similar for both milks (92%). Infants retained more nitrogen when fed Prematalac compared with Premium (mean (+SD) 477 (59) and 299 (30) mg per kg per day respectively). Neither milk was considered ideal, and we conclude that standard formulae are unable to support the potential for gain in lean body tissue of the premature infant.
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In an outbreak of benign myalgic encephalomyelitis in a girls' school all the residential pupils, both those affected and those unaffected, were investigated. Special virological tests were essentially negative, but it seemed that a few girls had had a viral infection. Psychological testing showed that among younger girls the patients were more neurotic than the others. Girls with various disorders were found to have been classified as having the same disorder, because of what has been called altered medical perception. The conclusions of an international symposium on this condition were not substantiated.
Subfractions of fraction 9, obtained from a peptic-tryptic-pancreatinic digest of wheat gliadin, were subjected to in vitro mucosal digestion and the filtrates examined for residual peptides. Small-intestinal mucosa from four groups of individuals were studied-eight patients with coeliac disease in remission; eight healthy controls; nine first degree relatives of patients with coeliac disease, and six children with recurrent diarrhoea investigated for possible coeliac disease, but in whom the diagnosis was excluded. The highest amounts of residual peptides (measured by scanning densitometer) were detected after digestion with mucosa from patients with coeliac disease and the lowest amounts with the control groups. The results obtained with the group of relatives fell between those of the coeliac disease and control groups, while the recurrent diarrhoea group overlapped the relatives and controls. The residual peptides were derived chiefly from the B-type subfractions of subfractions 1 and 2, obtained by ion-exchange chromatography of fraction 9. These subfractions are rich in glutamine/glutamic acid and proline and have a molecular weight (apparent) of not greater than 1500 Daltons. The results lend further support to the hypothesis of an enzyme deficiency in coeliac disease. A partial enzyme deficiency may exist in some first-degree relatives and in some children with recurrent diarrhoea but with histology of the small intestine within normal limits. HLA-B8 antigen is not correlated with this deficiency, but, when the two factors are associated, they could be related to the manifestation and severity of coeliac disease.
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Thirty-five children, in whom coeliac disease had been diagnosed on inadequate grounds and who had been on a gluten-free diet for one to 10 years, were challenged with gluten in accordance with a standardised procedure. All children were admitted to hospital for 48 hours for general assessment, two one-hour blood xylose tests, and the introduction of gluten. Thirty children underwent a pre-challenge peroral jejunal mucosal biopsy; the specimens were either normal or showed slight non-specific abnormalities. Gluten powder 20 g/day was given in addition to an otherwise gluten-free diet. The children were reassessed as outpatients every two weeks, when a one-hour blood xylose test was performed. Repeat biopsy was performed when xylose absorption fell or after three months. Seventeen children had abnormal post-challenge biopsy appearances compatible with coeliac disease in relapse; 14 of these children completed their challenge within eight weeks. Seventeen children had completely normal biopsy appearances at the end of three months and were returned to a normal diet. One to two years later eight underwent repeat biopsies, which showed nothing abnormal. In only one child, the oldest in the series, were the histological findings equivocal. In the 17 children in whom coeliac disease was confirmed the duration of gluten challenge was not related to age, duration of gluten-free diet, histological findings on the pre-challenge biopsy, or HLA status.
Chromatographically separated fractions of a proteolytic digest of wheat gliadin were assayed for cytotoxic properties using cultured human embryonic intestinal, lung, kidney, adrenal, and HEp-2 cells. In all cell types noxious effects were observed microscopically over a 24 h period. The most active fraction was that previously shown to produce xylose malabsorption in subjects with coeliac disease, disruption of lysosomes, and inhibition of morphological recovery of cultured mucosa from a patient on a gluten-free diet.