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Objectives: To describe the epidemiology of childhood tuberculosis in Wales and to assess the standard of management of patients with tuberculosis.Design: Retrospective study of data retrieved from case notes and review of radiographs of all identified patients.Subjects: Forty eight cases of tuberculosis and 10 of tuberculosis chemoprophylaxis in children under 15 years of age, in Wales, between January 1986 and December 1992.Main Outcome Measures: Management of childhood tuberculosis in Wales compared with the published recommendations of the Joint Tuberculosis Committee of the British Thoracic Society.Results: Documentation was poor in most of the 48 cases of tuberculosis and only 31 (65%) were formally notified. One third of patients were asymptomatic and were detected by contact tracing; only eight (17%) were culture positive. Only 20% of patients from ethnic minorities had previously been immunised with BCG. Management and chemotherapy varied widely. Few patients were managed jointly by paediatricians and chest doctors. Only 10% completed treatment with a recommended chemotherapy regimen. In 37% of patients treatment was inadequate, and in the remainder either the choice of drugs or the duration of treatment was inappropriate. No patient died, nor had any relapsed by June 1995.Conclusions: Very few cases of childhood tuberculosis were managed according to the recommendations of the British Thoracic Society. [ABSTRACT FROM AUTHOR]

Immunogens from Aspergillus fumigatus were fractionated on the basis of molecular weight. Nine fractions ranging from 900 to 10 kD 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 >900 kD and 170 kD whereas the profile for control subjects had a peak at >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 IgF lo all fractions were seen during an episode of ABPA. Although there was an exaggerated IgG increase lo 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 Aspergillus 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM PUBLISHER]

Breast cancer is a most common malignancy especially in Iraqi women accounting for high morbidity and mortality. Mutations in BRCA1 gene is one of the important genetic predisposing factors inbreast cancer. Similarly ERBB2 and TP53 are also key prognostic markers in breast cancer treatment.We were interested to explore the gene expression profiles of BRCA1, ERBB2 and TP53 in breast cancer women patients from Iraq so as to assess the potential of such markers in breast cancer treatment. The mRNA levels were significantly over-expressed in tumor tissues in comparison to normal ones with p values (p<0.005) observed between malignant BRCA1 and control tissue samples. Similarly significant difference (p<0.001) was observed between malignant ERBB2 in comparison to control, and malignant TP53 and benign tissue samples as well. However in blood samples, no considerable expression of these markers was observed. Out of three selected genes, ERBB2 expression was significantly expressed in comparison to BRCA1 and TP53 in cancer tissue. Mutation analysis of BRCA1, ERBB2 and TP53 has been made to find out the region most susceptible to mutations in these genes The BRCA1 exon 11, ERBB2 16 and TP53 exon 5 displayed increased chances of having mutations. We can conclude from the study that differential gene expression of BRCA1, ERBB2 and TP53 at mRNA levels may act as a diagnostic marker of circulating tumor cells having important prognostic value in breast cancer patients.

Background: Intramuscular epinephrine (adrenaline) is the first-line therapy for anaphylaxis and prompt administration improves outcome. In 2011, two epinephrine autoinjectors existed in the United Kingdom, differing in their users' administration method: EpiPen(®) and Anapen(®) . We routinely train all families who receive these devices., Aim: To evaluate: Maternal competence in using epinephrine autoinjectors following our standard anaphylaxis training package. Which device mothers found easier to use., Methods: Mothers with no previous epinephrine autoinjector experience were approached to participate. One clinician provided a standardized demonstration on using a randomly assigned autoinjector device. She immediately evaluated the mothers' performance using ten predetermined criteria. Four criteria were device specific and six were common criteria to both devices., Results: One hundred mothers participated: 50 EpiPen(®) and 50 Anapen(®) . A substantial proportion of mothers (15% overall) were not able to successfully 'fire' these training devices: Anapen(®) 4% and EpiPen(®) 26% (OR 8.43, p = 0.005). Only 22% of mothers overall were able to perform all ten procedures completely successfully: Anapen(®) 32% and EpiPen(®) 12%. Chi-squared analysis showed a significantly higher proportion of mothers correctly performing all Anapen(®) specific procedures than EpiPen(®) (OR 14.24, p < 0.0001)., Conclusion: It is concerning that 15% of mothers overall could not 'fire' these devices correctly despite a one-to-one demonstration, identifying a need for more user friendly devices and training. Mothers found the Anapen(®) device significantly easier to use, which may have implications for future prescribing. Evaluation of the next generation of autoinjectors and their training packages needs to be performed as important practical differences may be found., (© 2013 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd.)

Aims: Numerous studies have identified shortcomings in the management of children at risk of severe acute allergic reactions (anaphylaxis). The Science and Research Department at the Royal College of Paediatrics and Child Health (RCPCH) was commissioned by the Department of Health to develop competence based national care pathways for children with allergies. Anaphylaxis is the first completed pathway., Methods: The anaphylaxis pathway was developed by a multidisciplinary working group, reviewed by a broad group of stakeholders and approved by the Allergy Care Pathways Project Board and the RCPCH Clinical Standards Committee., Results: Pathway development is described under five headings: evidence review, mapping, external review, core knowledge documents and key recommendations. The full pathway can be downloaded from www.rcpch.ac.uk/allergy/anaphylaxis. This document describes the entry points and the ideal pathway of care from self-care through to follow-up. The five key recommendations focus on: (1) prompt administration of adrenaline by intramuscular injection; (2) referral to specialists with competence in paediatric allergies; (3) risk analysis; (4) provision of a self-management plan; and (5) suggested creation of a national anaphylaxis death register., Conclusions: We present the first national care pathway for anaphylaxis, which is based on a critique of published evidence, expert consensus and multi-stakeholder input including patient representation via the Anaphylaxis Campaign. The Project Board urges health professionals to work together across networks to improve care for children at risk of anaphylaxis, in particular during the period after an acute reaction. Additionally, the Project Board strongly recommends the funding of a national anaphylaxis register.

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.

Background: The handling of sick neonates may have detrimental effects such as hypoxia or bradycardia. Such handling is inevitable due to the frequent need for practical procedures; however, minimising handling reduces these adverse events and may improve outcome. Radiography is one of the commonest procedures performed on neonates. Usually the infant is lifted and placed onto the radiographic cassette; however, modern incubators often incorporate a tray beneath the mattress in which the radiographic cassette can be placed without the need to disturb the infant., Objective: To compare the quality of chest radiographs taken using the standard direct contact method, with those taken using the under-tray technique., Materials and Methods: A series of chest radiographs taken over a 21-month period were analysed independently by two consultant paediatric radiologists unaware of the radiographic details. The position of the radiograph, i.e. direct contact or under-tray, was determined by the radiographer. Radiographic quality was scored on the following features: exposure, blurring, rotation, cut-off or coning, and side markers. A subjective score was also included. The results from each radiologist were analysed separately., Results: Seventy chest radiographs were analysed-25 standard method, 45 under-tray. A statistically significant advantage for the under-tray method was seen on two analyses-radiologist 1 for exposure, and radiologist 2 for cut-off. No other significant differences were noted. There were no differences in the infants' weights or radiation exposure., Conclusions: The under-tray method for taking radiographs may produce films of at least equivalent quality to the standard method. Since the standard method involves handling with potential desaturation and bradycardia, this technique should cease.

Children with peanut allergy are almost always advised to avoid nuts for life. There have been recent reports from academic centres that in some cases the allergy might resolve and thus these dietary restrictions can be lifted. To evaluate resolution of peanut allergy in a selected group of children in a general paediatric setting. Children 4-16 yr old with a clear history of an allergic reaction to peanuts who had not had any reaction in the previous 2 yr were eligible. Specific immunoglobulin E (IgE) or skin prick test (SPT) at the time of diagnosis was sought. A SPT and specific IgE was then done and if this was

Objective: To determine the vitamin D status of pregnant women from non-European ethnic minorities in South Wales., Design: Prospective study., Setting: Llandough Hospital, Cardiff, South Wales., Sample: One hundred and sixty pregnant women from a non-European ethnic minority population in South Wales., Methods: Biochemical screening of vitamin D status was carried out at the first antenatal visit. Women found to be deficient in vitamin D were subsequently supplemented and vitamin D status was rechecked at delivery., Main Outcome Measure: Vitamin D status at delivery., Results: Eighty of 160 women had a vitamin D level below 8 ng/mL at their first antenatal visit and were treated with oral vitamin D. Factors that could influence vitamin D status such as religion, fluency in English and dressing habits did not appear to have any effect, although a higher proportion of women who had lived in Britain for longer than three years had subnormal vitamin D levels. In 58 of those checked at delivery, the mean plasma vitamin D level increased from 6 to 11 ng/mL although the mean parathyroid hormone level was unchanged., Conclusion: In view of the high incidence of subnormal vitamin D levels in women from ethnic minorities, we recommend biochemical screening of these women in early pregnancy, with subsequent supplementation where indicated.

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.