The use of adrenaline autoinjectors by children and teenagers.

BACKGROUND: Although adrenaline is recommended as first line treatment for anaphylaxis, it is often not utilized. There has been a debate about when adrenaline autoinjectors should be prescribed and how many should be dispensed. OBJECTIVES: To see how many adrenaline autoinjectors were used during anaphylactic reactions and to determine why they were not used in situations where they were clinically indicated. METHODS: Patients were recruited prospectively at 14 paediatric allergy clinics throughout UK. Participants completed a questionnaire covering demographic data, atopic status and details of allergic reactions in the previous year and reasons for using more than one device. RESULTS: A total of 969 patients were recruited of whom 466 (48.1%, 95% CI: 37.9-58.2) had had at least one reaction in the previous year; 245 (25.3%, 95% CI: 16.2-34.4) of these reactions were anaphylaxis. An adrenaline autoinjector was used by 41 (16.7%, 95% CI: 11.7-21.3) participants experiencing anaphylaxis. Thirteen participants received more than one dose of adrenaline, for nine of these a health professional gave at least one. The commonest reasons for using more than one were severe breathing difficulties (40%), lack of improvement with first dose (20%) and miss-firing (13.3%). The commonest reasons for not using adrenaline in anaphylaxis were 'thought adrenaline unnecessary' (54.4%) and 'unsure adrenaline necessary' (19.1%). Many with wheeze did not use their autoinjector. CONCLUSIONS AND CLINICAL RELEVANCE: Adrenaline is used by only a minority of patients experiencing anaphylaxis in the community. Thirteen of the 41 patients with anaphylaxis who used their autoinjector needed another dose of adrenaline. Further research is needed to consider how to best encourage the usage of adrenaline when clinically indicated in anaphylaxis.

An evaluation of the completeness of reporting of childhood tuberculosis.

The sensitivity of the Enhanced Tuberculosis Surveillance (ETS) scheme for monitoring tuberculosis in children is unknown. We used the British Paediatric Surveillance Unit (BPSU) reporting scheme to conduct a prospective observational study of tuberculosis in children aged <16 yrs in the UK. Reported cases were then matched with records from the ETS database. A total of 320 cases were reported to the BPSU between January and December 2004. We estimated that there were 557 paediatric cases in England, Wales and Northern Ireland in 2004: 222 (40%) cases reported to both BPSU and ETS, 98 (18%) reported to BPSU but not ETS and 237 (42%) reported to ETS but not BPSU. Children aged <5 yrs were significantly less likely to be reported to ETS compared with older children (p<0.01). There is substantial under-reporting of childhood tuberculosis, especially of children aged <5 yrs. ETS provides a representative picture of the demographics but may miss approximately 20% of cases. This should be taken into account when planning training and resource requirements for tuberculosis. Increased efforts are needed to ensure that all paediatric cases are reported to ETS.

Neonatal screening for cystic fibrosis in Wales and the West Midlands: 1. Evaluation of immunoreactive trypsin test.

A study programme was set up in Wales and the West Midlands to evaluate serum immunoreactive trypsin screening for cystic fibrosis in neonates using blood spots collected for metabolic screening. By screening half the blood spots from each area, it was hoped to generate two comparable groups of fibrocystic children; those detected by screening and those not screened who would be diagnosed clinically. Over almost three years, more than 120,000 specimens were screened and 37 infants detected with cystic fibrosis. Four additional fibrocystic patients were missed on screening: two had negative immunoreactive trypsin values, of which one had meconium ileus, and two, although giving initial positive tests, were negative on follow up. Excluding infants known to be at risk, comparison of the numbers of children detected in the screened and unscreened groups showed more than a two-fold difference in favour of the screened group. There may be a large number of undiagnosed fibrocystic patients in the general population.

Increased susceptibility to peroxide-induced haemolysis with normal vitamin E concentrations in cystic fibrosis.

Vitamin E status was assessed in 22 patients with cystic fibrosis and 9 controls by measuring concentrations of the vitamin, vitamin E:lipid ratios and peroxide-induced haemolysis in plasma and erythrocytes. For a given concentration of plasma or erythrocyte alpha-tocopherol, erythrocytes of patients with cystic fibrosis were more susceptible to peroxide-induced haemolysis than controls. This susceptibility should be countered by supplementation with vitamin E to maintain higher than normal concentrations of circulating alpha-tocopherol-greater than 4.8 mmol alpha-tocopherol/mol cholesterol.

Tuberculosis in the United Kingdom and Republic of Ireland.

AIMS: To describe the clinical features, diagnosis and management of children with tuberculosis in the United Kingdom and Republic of Ireland. METHODS: Cases of culture-confirmed and clinically diagnosed tuberculosis were reported to the British Paediatric Surveillance Unit from December 2003 to January 2005. RESULTS: 385 eligible cases were reported. Pulmonary disease was present in 154 (40%) children. Just over half (197, 51%) of children presented clinically and most of the remainder (166, 43%) at contact tracing. A probable source case was identified for 73/197 (36%) of the children presenting clinically. The majority (253, 66%) of children had a microbiological and/or histological investigation, and culture results were available for 240 (62%), of whom 102 (26%) were culture positive. Drug resistance was reported in 15 (0.4%) cases. 44% (128/292) of non-white children did not receive the recommended quadruple drug therapy. Seven children died. Only 57% (217) of children were managed by a paediatric subspecialist in respiratory or infectious diseases or a general paediatrician with a special interest in one of these areas. Fewer than five cases were reported from 119/143 (83%) respondents and 72 of 96 (75%) centres. CONCLUSIONS: Many paediatricians and centres see few children with tuberculosis. This may affect adherence to national guidelines. Managed clinical networks for children with tuberculosis may improve management and should be the standard of care.

Ethnic differences in selective neonatal BCG immunisation: white British children miss out.

BACKGROUND: Tuberculosis (TB) is a re-emerging problem, especially in the larger cities of Western Europe. Selective neonatal BCG vaccination is recommended for infants at risk of TB in the UK. Neonatal BCG is safe and effective, with an overall protective value of 75%. This study aimed to assess BCG rates among at risk infants in Cardiff and the Vale of Glamorgan, South Wales in the year 2003. METHODS: A cohort of infants at risk for TB was identified from demographic data stored on a computerised maternity activity database. A manual search of immunisation records determined overall rates and the rates for infants belonging to various ethnic groups. RESULTS: Of 5308 infants born in 2003, 514 (9.6%) were at risk of TB; 423 (82.2%) of these infants were referred postnatally for BCG vaccination and 391 received it. Twenty six of the 41 at risk white British infants missed having a BCG vaccination compared with 47 of 288 Asian infants and seven of 39 black African babies. The rate of BCG vaccination among white British infants was 36.5% compared with 83.6% for Asian infants from the Indian subcontinent (chi(2) = 7.25, p<0.01) and 82% for black African infants (chi(2) = 4.48, p<0.05). CONCLUSIONS: The overall BCG rate among at risk infants in Cardiff was 76% during the study period. The vaccination rate was poor among white British infants compared with other ethnic groups. Enhanced awareness of health professionals to recognise the need for vaccinating certain white children at risk of TB is essential to improve BCG coverage in an increasingly multiethnic population.

Active surveillance for tuberculosis in Wales: 1996-2003.

AIMS: To estimate the incidence of active tuberculosis (TB) and study the use of chemoprophylaxis for latent TB in children in Wales, and to identify potential areas for improving prevention and management. METHODS: Active surveillance for TB in children aged 0-15 years from July 1996 to December 2003, using the Welsh Paediatric Surveillance Scheme. RESULTS: A total of 232 children, 102 with active TB (2.3 per 100 000) and 130 with latent TB (2.9 per 100 000), were identified. Nearly half (45%) belonged to ethnic minorities (19% were of black African origin), a much higher proportion than the base population. Pulmonary disease was the most common presentation (47%), including six (9%) children who were sputum smear positive. There were 10 cases of disseminated TB, nearly all in white children under 10 years of age. Less than two thirds of eligible children (27/46, 59%) were known to have received BCG immunisation. The source of infection was an adult household contact in most cases, but was not known in 44 cases, particularly among teenagers. Four community outbreaks occurred during the surveillance period, including three in high schools. CONCLUSION: TB incidence in children in Wales remains low, but the epidemiology is changing with an increasing proportion of cases in black African children. The high proportion of patients with disseminated TB is of particular concern. TB in teenagers was often associated with school outbreaks. Many eligible children do not receive BCG immunisation, indicating further scope for prevention.

Fractional measurements of sweat osmolality in patients with cystic fibrosis.

After pilocarpine iontophoresis the change of sweat concentration during collection was studied by vapour pressure osmometry in 24 patients with cystic fibrosis and 24 healthy controls. There was a continuous but proportionate fall in sweat concentrations during the collection period. Mean (SD) initial sweat concentration in the control group was 154.4 (32.6) mmol/kg falling, after 50 microliters of sweat produced, to 92.9 (15.8) mmol/kg. In the cystic fibrosis group it was 315.9 (35.8) mmol/kg falling to 247.4 (24.9) mmol/kg. Despite different rates of fall in concentrations, separation of the two groups was maintained throughout. We conclude that there are implications for the potential improvement of the predictive value of the sweat test.

Antiphospholipid syndrome presenting as episodic limb ischaemia.

A child presented with ischaemic episodes of his left leg from the age of 2 months. He was found to have raised anticardiolipin antibodies so was started on low dose aspirin. At a three year follow up he was asymptomatic with a normal anticardiolipin antibody level while taking aspirin daily, probably for life.

Student-initiated revision in child health.

Most teaching of child health in Cardiff takes place in block attachments of 8 weeks. There is an introductory seminar of 2 days followed by a 6-week clinical attachment in a district general hospital in Wales, and then a revision period of one week designed to help students formalize and structure their basic knowledge and to clarify aspects of child health which they may have had difficulty in understanding. The revision programme has to take into account: the short time available, the small number of teaching staff, the most relevant basic knowledge and active participation by the student. This paper describes how this week has been improved through the use of student-initiated revision (SIR). The students' appraisal of this revision and in particular SIR is presented.

Assessment of in-line filters to prolong the life of intravenous cannulae in cystic fibrosis patients.

Twelve patients with cystic fibrosis received 12 courses of intravenous antibiotics, each over 10-14 days, both with and without the use of an extended-life disposable filter. The design of the trial was to replace the Venflon cannulae as they became non-patent and inserting a filter on alternate occasions. Thus each patient acted as her/his own control. Comparison of times during which cannulae remained patent showed a 50% improvement with use of a filter for 4 patients and no change for 7 patients. There was no significant difference associated with the use of a filter for the group as a whole but our small sample size excludes modest improvements.

Vitamin D deficiency: a concern in pregnant Asian women.

Vitamin D status of Asian mothers in Cardiff was investigated during early pregnancy and at the time of the birth of their babies, using serum parathyroid hormone (PTH). Median values in Asian (n 32) and Caucasian (n 63) mothers in early pregnancy were 1.56 and 0.81 pmol/l respectively. PTH levels from a separate sample of nineteen Asian and twenty-five Caucasian mothers at the time of birth were 3.0 and 2.20 pmol/l respectively. Altogether twelve Asian and two Caucasian women had elevated PTH. All Asian women who had high PTH values also had a very low serum 25-hydroxycholecalciferol level (25OHD). All samples were taken from women with no significant medical history and normal obstetric history. These findings suggest that subclinical vitamin D deficiency is still a cause for concern in Asian women. More active measures need to be taken to implement current recommendations to improve their vitamin D intake in pregnancy.

Collodion baby dehydration: the danger of high transepidermal water loss.

We describe transepidermal water loss (TEWL) measurements in a collodion baby suffering from severe hypernatraemic dehydration and hypothermia, who required intravenous fluid therapy in a special incubator. The TEWL values 4 days after birth were abnormally high compared with normal infants of the same age. The TEWL measurements returned towards normal within the first month, in parallel with the improvement of both the skin signs and the electrolyte and fluid balance.

Serum Tamm-Horsfall protein levels in childhood: relationship with age and glomerular filtration rate.

Serum Tamm-Horsfall protein (THP) levels were measured in 157 infants and children, 46 cord blood samples and 75 adults using an enzyme-linked immunosorbent assay. THP levels increased significantly across the age groups, suggesting a positive relationship with age. There was a strong positive relationship between serum THP and estimated glomerular filtration rate among children with chronic renal failure.

The idiopathic hypereosinophilic syndrome.

A 14 year old girl with idiopathic hypereosinophilic syndrome is described. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the cardiovascular system as the major complication. She responded well to treatment. After a comprehensive search of the published reports 18 cases of this syndrome were identified in children under 16 years. Fifteen of these children had involvement of the cardiovascular system as the major source of their morbidity and mortality. Summary of the clinical details and laboratory, biopsy, and necropsy findings of the involvement of the various organ systems of the 18 children is presented.

Fatty infiltration in the liver in medium chain acyl CoA dehydrogenase deficiency.

Fatty infiltration of the liver at postmortem examination has been recommended as a criterion for selection of infants who have died suddenly and unexpectedly for further biochemical investigation for disorders of fatty acid oxidation. We describe a boy with medium chain acyl CoA dehydrogenase deficiency who died four months after diagnosis and in whom only minimal hepatic fatty infiltration was found.

Sequential serological responses to Aspergillus fumigatus in patients with cystic fibrosis. Use of antigen 'stretching' to delineate IgG and IgE activity.

Immunogens from Aspergillus fumigatus were fractionated on the basis of molecular weight. Nine fractions ranging from 900 to 10 kDa were used in ELISA and in a radioallergosorbent test (RAST) with sera from cases of allergic bronchopulmonary aspergillosis (ABPA) and from cystic fibrosis (CF) patients with ABPA or other Aspergillus involvement and compared with control subjects. The profile of IgG reactivity to the nine fractions did not vary substantially for all Aspergillus-involved groups producing peaks at greater than 900 kD and 170 kD whereas the profile for control subjects had a peak at greater than 900 kD only. The IgE profile for CF patients with ABPA did not differ from the profile of the RAST-positive CF patients without ABPA and provided only one peak of activity at 24 kD. Recovery from an episode of ABPA in CF patients was accompanied by a fall in both IgG and IgE antibody levels to all nine fractions, whereas increases in IgG and IgE to all fractions were seen during an episode of ABPA. Although there was an exaggerated IgG increase to antigens in the 43-170 kD range during ABPA, a meaningful increase was also observed to unfractionated A. fumigatus antigen preparations. With IgE in one detailed study the 24-kD fraction provided a better indication of Aspergillus involvement than the unfractionated A. fumigatus antigens. Sequential studies of IgG and IgE levels were not able to predict an episode of ABPA but were useful in conjunction with clinical assessment in following the course of the illness.