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Biomedical subjects

J Walker-Smith

Publications and source records attributed to J Walker-Smith.

At least 19 recordsLinked to original sources
PubMed1999

Contribution of the MHC region to the familial risk of coeliac disease.

Susceptibility to coeliac disease is genetically determined by possession of specific HLA-DQ alleles, acting in concert with one or more non-HLA linked genes. The pattern of risk seen in sibs and twins in coeliac disease is most parsimonious with a multiplicative model for the interaction between the two classes of genes. Based on a sib recurrence risk for coeliac disease of 10% and a population prevalence of 0.0033, the sib relative risk is 30. To evaluate the contribution of the MHC region to the familial risk of coeliac disease, we have examined haplotype sharing probabilities across this region in 55 coeliac disease families. Based on these probabilities the sib relative risk of coeliac disease associated with the MHC region is 3.7. Combining these results with published data on allele sharing at HLA, the estimated sib relative risk associated with the MHC region is 3.3. Therefore, the MHC genes contribute no more than 40% of the sib familial risk of coeliac disease and the non-HLA linked gene (or genes) are likely to be the stronger determinant of coeliac disease susceptibility.

Celiac Disease↗
PubMed1998

Dyspepsia in infants and children.

Pathological processes and diseases of the upper gastrointestinal tract have become increasingly recognized over recent years as childhood entities responsible for a variety of upper gastrointestinal symptoms previously labelled as functional or non-organic. The term 'dyspepsia' is an adult one whose definition requires clarification before use in the paediatric context, but it encompasses age-dependent symptoms such as feed-associated irritability in the infant, peri-umbilical pain in the younger child, and heart-burn, nausea, and indigestion in the older child as in adults. The possible organic conditions giving rise to such symptoms are multiple and multiorgan and include: gastro-oesophageal reflux; peptic ulcer disease; upper gastrointestinal Crohn's disease; antroduodenal motility disorders; pancreatitis; cholecystitis; cholelithiasis; biliary dyskinesia; and abdominal migraine. However, Munchausen syndrome by proxy must not be forgotten. Non-ulcer dyspepsia, it is now clear, has a basis in altered gastroduodenal motility and may be amenable to propulsion agents. In many individuals the dyspeptic symptoms of recurrent abdominal pain may be altered by psychotherapeutic intervention. Indeed there remains a proportion of children who undoubtedly have a behavioural or psychological base to their complaint. Nevertheless, with the recent increase in diagnostic yield from improved technical investigative aids available to paediatrics in the last 5-10 years, it is clear that the responsibility of the paediatrician to the child to find a cause of their symptoms is paramount. The variety of presenting features, possible causes of these symptoms, and appropriate investigation and treatment will be discussed, and management algorithms based on published literature and personal practice will be offered.

Abdominal Pain↗
PubMed1994

Food sensitive enteropathy: overview and update.

There are two types of food sensitive enteropathy; permanent and temporary. Celiac disease belongs to the former, the temporary food sensitive enteropathies of early childhood to the latter. A food sensitive enteropathy is characterized by an abnormal small intestinal mucosa while having the offending food in the diet; the abnormality is reversed by an elimination diet, only to recur once more on challenge with the relevant food. These disorders are temporary and may follow gastroenteritis. Cow's milk sensitive enteropathy is the most frequent and best known example but soy protein, egg, fish, chicken meat, ground rice and probably gluten may also temporarily damage the small intestinal mucosa in infancy. Treatment is with an elimination diet and protein hydrolysates as a cow's milk substitute. The reason why these enteropathies are temporary has not yet been established.

Child↗
PubMed1994

Impact of rotavirus infection on a paediatric hospital in the east end of London.

AIMS: To study the impact of confirmed rotavirus infection at a paediatric hospital; to use the data to obtain a minimum estimate of the cost of treating reported cases of rotavirus in England and Wales. METHODS: Data were obtained on all patients with rotavirus over a two year period. Information was collected on 386 patients with rotavirus infection who were treated at the 120 bed Queen Elizabeth Hospital for Children in East London. This included the virus serotype, the patient's age, whether they required intravenous infusion, duration of hospital stay, numbers of patients treated in the casualty department, and numbers who had to be admitted. Treatment costs were obtained from the Finance Department of the Hospitals for Sick Children. RESULTS: The minimum cost of treating patients, excluding the cost of medical staff at the hospital, was estimated to be 95,400 pounds a year. One hundred and forty eight (38%) patients were admitted to the wards and a further 49 patients developed symptoms while in hospital. Intravenous infusion was required by 18 patients. The mean duration of hospital stay was 5.5 days. One hundred and eighty nine (49%) patients were treated with oral rehydration solution in casualty, given advice, and sent home. Ninety four per cent of the patients were aged under 2 years. The findings were comparable with those obtained in a study at Texas Children's Hospital, USA. The G serotype (VP7) of rotavirus did not influence the severity of infection. CONCLUSION: Rotavirus infections accounted for a significant number of patients treated in casualty, admissions to hospital, and bed occupancy in a paediatric hospital. The estimated cost of treating reported cases of rotavirus in England and Wales is in excess of 6.3 pounds million a year.

Age Factors↗
PubMed1990

Barium studies and ileoscopy compared in children with suspected Crohn's disease.

The findings on barium follow through, ileoscopy and biopsy of the terminal ileum in 46 children with suspected chronic inflammatory bowel disease have been compared to the final clinical diagnosis. Ileoscopy and barium follow through agreed in 91%, barium follow through and biopsy in 80% and barium follow through, ileoscopy and biopsy in 76% of cases. A pronounced lymphoid hyperplasia pattern was present radiologically in 24%, and was a source of error in two cases. Barium follow through compared favourably to ileoscopy or biopsy individually, detecting 18 of 20 and 17 of 20 cases respectively; however, ileoscopy combined with biopsy diagnosed every case. The sensitivity of the barium follow through was 0.90 with a specificity 0.96 for the diagnosis of Crohn's disease in the terminal ileum.

Barium Sulfate↗
PubMed1990

Down's syndrome and coeliac disease.

The association of congenital abnormalities and gastrointestinal tract diseases has been described. The coincidence of Down's syndrome and coeliac disease has been occasionally reported, but a clear relation is not definitely established. Reviewing 190 patients with coeliac disease diagnosed at Queen Elizabeth Hospital, London, from 1960 to 1985 an increased incidence of Down's syndrome compared to the natural incidence of this abnormality in the general population was found. The age of the mothers was well below that where a reverse in the incidence of Down's syndrome is expected, and it appears therefore that this anomaly and coeliac disease may be linked. This possibility should be bourne in mind when evaluating children with Down's syndrome and gastrointestinal symptoms.

Autoimmune Diseases↗
PubMed1989

Evaluation of infant feeding in acute gastroenteritis.

Sixty-eight bottle-fed babies under 9 months of age with mild acute gastroenteritis were observed to evaluate current feeding regimens following acute gastroenteritis in infancy. All babies were fed for 24 h with a glucose-electrolyte mixture (GEM) and then randomly assigned to either a gradual reintroduction to their normal milk, i.e., slow regrade; immediate return to full-strength formula; or a rapid regrade to a hypoallergenic whey hydrolysate formula. All groups were well matched for age, sex, ethnic origin, nutritional state, and degree of hydration. There was no significance difference in stool frequency or reducing substances, vomiting, and duration of hospital stay between the three groups. Many infants (6/24) refused to take the whey hydrolysate formula, presumably because of unpalatability. Weight gain was more rapid when full-strength milk was given. Clinical relapse developed in 12 (17%) of patients. An enteric pathogen was detected in eight of this group and cow's milk protein intolerance in three (one from each feeding group). No infant had clinically significant lactose intolerance, in marked contrast to previous experience at Queen Elizabeth Hospital. In this group of previously healthy, well-nourished babies with mild acute gastroenteritis, there was no advantage in regrading slowly to milk or a hypoallergenic formula. An immediate return to normal formula 24 h after GEM feeding was well tolerated and simpler for parents.

Acute Disease↗
PubMed1988

The teenage coeliac: follow up study of 102 patients.

Over a 10 year period a total of 102 teenage patients with coeliac disease were assessed on transfer from paediatric hospitals to an adult clinic. Fifty seven patients said they were on a strict gluten free diet; 36 were semistrict, and nine admitted to eating a normal diet. Jejunal mucosal abnormalities, however, suggested that many patients on the 'strict' diet were actually consuming gluten. All patients were well with biochemical parameters within the normal range. Height percentiles were not significantly different from the normal population but patients, as a group, were significantly lighter.

Adolescent↗
PubMed1987

HLA class II alpha chain gene polymorphisms in patients with insulin-dependent diabetes mellitus, dermatitis herpetiformis, and celiac disease.

We have investigated DNA polymorphism of the class II alpha chain genes in HLA typed patients with insulin dependent diabetes mellitus (IDDM; n = 79), celiac disease (CD; n = 46), dermatitis herpetiformis (DH; n = 53), and controls (n = 86). Preferential allelic associations of HLA genes and gene products have thus been constructed for susceptibility to these diseases. DR alpha and DQ alpha gene polymorphisms indicated heterogeneity of HLA DR3, DRw6, and DR7, and HLA DR2 and DRw6, respectively. In DR7 positive CD patients a 3.8-kilobase (kb) DR alpha fragment, which correlated with DQw3, was found in only 11% of patients compared with 45% of corresponding controls (P less than 0.05). An increased frequency of a DX alpha genotype UU in all three diseases was found (IDDM 59%, DH 45%, CD 48%, compared to 21% in controls, P less than 0.001), which is not explained solely by the increased frequencies of DR3-DX alpha U. We therefore conclude part of the genetic susceptibility for these three conditions is encoded by genes within the DQ-DX subregion.

Celiac Disease↗
PubMed1982

Flat small intestinal mucosa and autoantibodies against the gut epithelium.

A male infant, aged 1 year 3 months, was admitted to the hospital with protracted diarrhoea, vomiting, and weight loss. The diarrhoea and vomiting coincided with an outbreak of acute diarrhoea and vomiting affecting other family members. Biopsy showed a flat small intestinal mucosa which did not respond to a diet free of gluten, cow's milk, and eggs, or during 8 weeks of intravenous alimentation. Steroids were given, and courses of nalcrom and later cimetidine, but these did not produce any significant improvement. A rare IgG autoantibody specific for gut epithelium was found, which, when present, was associated with a cytological abnormality of crypt enteroblasts. The autoantibody disappeared after treatment with cyclophosphamide, and the cytological abnormality subsequently diminished. However, the mucosa remained severely abnormal and has been so for 23 months. It is possible that an autoimmune reaction against the patient's small intestinal mucosa has led to persistence of the enteropathy.

Animals↗
PubMed1982

Traveller's diarrhoea with a vengeance in children of UK immigrants visiting their parental homeland.

Six healthy children, born in the UK, travelled to their parental homeland and developed a severe form of traveller's diarrhoea. This was characterised by rapid loss of weight and chronic diarrhoea. On return to this country, investigation in 5 of them showed an abnormal, small intestinal mucosa. There was a high incidence of pathogens and potential pathogens found in stools and duodenal juice. Immunodeficiency was found in 2 of them. Small intestinal mucosal damage related to gut infection in previously well children appears to be of cardinal importance in the interaction between chronic diarrhoea and malnutrition. This observation may point the way to future research into the primary role of gut infection in the initiation of the cycle of malnutrition and chronic diarrhoea in developing communities.

Body Weight↗