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Read N.,Brighton and Sussex Medical School | Lim E.,Brighton and Sussex Medical School | Tarzi M.D.,Brighton and Sussex Medical School | Hildick-Smith P.,Royal Alexandra Childrens Hospital | And 2 more authors.
Clinical and Experimental Immunology | Year: 2014

Hereditary angioedema (HAE) is a rare disease characterized by episodes of potentially life-threatening angioedema. For affected children in the United Kingdom, there are relatively few data regarding disease prevalence, service organization and the humanistic burden of the disease. To improve knowledge in these areas, we surveyed major providers of care for children with HAE. A questionnaire was sent to major paediatric centres to determine patient numbers, symptoms, diagnostic difficulties, management and available services. In addition, all patients at a single centre were given a questionnaire to determine the experiences of children and their families. Sixteen of 28 centres responded, caring for a total of 111 UK children. Seven children had experienced life-threatening crises. One-third of patients were on long-term prophylactic medication, including C1 inhibitor prophylaxis in four children. Eight centres reported patients who were initially misdiagnosed. Broad differences in management were noted, particularly regarding indications for long-term prophylaxis and treatment monitoring. We also noted substantial variation in the organization of services between centres, including the number of consultants contributing to patient care, the availability of specialist nurses, the availability of home therapy training and the provision of patient information. Ten of 12 patient/carer questionnaires were returned, identifying three common themes: the need to access specialist knowledge, the importance of home therapy and concerns around the direct effect of angioedema on their life. To our knowledge, this study represents the first dedicated survey of paediatric HAE services in the United Kingdom and provides useful information to inform the optimization of services. © 2014 British Society for Immunology.

Smith C.,Royal Alexandra Childrens Hospital | Winn A.,University of Brighton | Seddon P.,Respiratory Medicine | Ranganathan S.,Respiratory Medicine
Journal of Cystic Fibrosis | Year: 2012

Background: The fundamental nutritional treatment of a high fat diet for cystic fibrosis (CF) is established and essentially unchanged in the last 25. years. However, recent concerns have emerged regarding the potential risks of such a diet. We investigated the diets of children with CF to determine the source of energy, energy imbalance, and changing trends of fat intake. Method: In a prospective longitudinal study over 8. years at a single paediatric CF clinic three-day food diaries that included supplementary nutrition (SN) either as enteral feeds or oral nutritional supplements (ONS), were analysed annually. Influence of year on percent energy by type (fat, carbohydrate and protein) and on fat component: saturated (SFA); monounsaturated (MUFA) and polyunsaturated fatty acids (PUFA) was examined. Results: 136 food diaries were analysed in 27 children (age range 1-18. years). 51 (37%) food diaries included SN (enteral feeds n = 15 and ONS n = 36). Mean energy intake was 1726. Kcals (oral diet alone) and 2245. Kcals (including SN). Percent energy from macronutrients did not change significantly over time (protein p = 0.06; carbohydrate p = 0.44; fat p = 0.07) and remained within recommended levels. Mean caloric contribution from fat was 38.7% from oral diet alone and 37.8% including SN. Percent energy derived from SFA remained statistically unchanged (SFA p = 0.57) but fell from MUFA (p = 0.05) and PUFA (p = 0.004). Mean SFA consistently contributed > 134% (mean 158%) of reference nutrient intake and mean PUFA intake < 100% (92%). Conclusion: Macronutrient intakes did not change significantly in our population of CF children, but there was a consistent imbalance of fat-sources with over-dependence on saturated fats which, in the context of increased survival in CF may potentially increase risk of cardiovascular disease. Further studies are needed to confirm our findings, investigate consequences of fat imbalance and guide clearer advice regarding appropriate proportions of sources of fat for CF patients. © 2011.

Lim M.T.C.,Royal Brompton and Harefield NHS Trust | Wallis C.,Great Ormond Street Hospital | Price J.F.,Kings College | Carr S.B.,Barts and the London Hospital | And 4 more authors.
Archives of Disease in Childhood | Year: 2014

Introduction Newborn screening (NBS) for cystic fibrosis (CF) was introduced to London and South East England in 2007. We wished to assess the details of missed cases, and to compare the age at diagnosis and other clinical parameters, prescreening and postscreening. Methods Retrospective and prospective case notes and database review of all newly diagnosed CF patients in our 7 CF centres, for 18 months before and 4 years after NBS started. Results 347 patients were diagnosed with CF. 126 patients were not screened (born before or abroad), and had a median age at diagnosis of 2.4 years, excluding those with meconium ileus (MI). Their median time to diagnosis from initial symptoms was 1 year, and in 10% it was >6 years. After NBS started, 170 were diagnosed by NBS (48% were already symptomatic); 7 moved into the region after NBS elsewhere; 34 presented with MI (6 were negative on NBS); and 10 screened children were missed (false negative cases). Median age of diagnosis was 3 weeks. Prevalence was 1 in 3991 live births. By 2 years of age (with data on 104 patients), 49 children (47%) had their first isolation of Pseudomonas aeruginosa, while 37 (36%) had their first growth of Staphylococcus aureus from respiratory cultures. Conclusions NBS has significantly reduced the age of diagnosis, although many were symptomatic even at 3 weeks of age. A small number of patients with CF can still be missed by the screening programme, and the diagnosis should be considered even with a negative screen result.

Islam S.,Royal Alexandra Childrens Hospital | Adams S.D.,Royal Alexandra Childrens Hospital | Mahomed A.A.,Royal Alexandra Childrens Hospital
Minimally Invasive Surgery | Year: 2012

The aim of the study was to review our experience with single-incision laparoscopic surgery (SILS) and to compare costs and operative time to standard laparoscopic surgery (SLS). A prospectively collected database of operative times and costs was analysed for the years 2008-2011. SILS cases were compared to standard laparoscopy on a procedure-matched basis. Patient demographics, on-table time and consumable costs were collated. Descriptive statistics and Mann-Whitney U-test were utilized with SPSS for windows. Analysis of the data demonstrate that neither consumable costs nor operative time were significantly different in each group. Comparing operative costs, SILS appendicectomy, nephrectomy/heminephrectomy, and ovarian cystectomy/oophorectomy showed cost benefit over SLS (£397 versus £467; £942 versus £1127; £394 versus £495). A trend toward higher cost for SILS Palomo procedure is noted (£734 versus £400). Operative time for SILS appendicectomy, nephrectomy/heminephrectomy, and Palomo was lower compared to SLS (60 versus 103 minutes[mins.]; 130 versus 60 mins.; 60 versus 80 mins.). In conclusion, SILS appears to be cost-effective for the common pediatric surgical operations. There is no significant difference in operating time in this series, but small sample size is a limiting factor. Studies with larger numbers will be necessary to validate these initial observations. © 2012 Saidul Islam et al.

Lipworth B.J.,University of Dundee | Basu K.,Royal Alexandra Childrens Hospital | Basu K.,Royal Infirmary | Donald H.P.,Royal Infirmary | And 6 more authors.
Clinical Science | Year: 2013

The Arg16 β2 receptor genotype confers increased susceptibility to exacerbations in asthmatic children taking regular LABA (long-acting β2 agonists). We therefore evaluated using montelukast as an alternative to salmeterol as tailored second-line asthma controller therapy in children expressing this susceptible genotype. A total of 62 persistent asthmatic children with the homozygous Arg16 genotype were randomized to receive salmeterol (50 μg, b.i.d.) or montelukast (5 or 10 mg, once daily) as an add-on to inhaled fluticasone for 1 year. School absences (the primary outcome) were reduced with montelukast compared with salmeterol {difference in score= -0.40 [95% CI (confidence interval), -0.22 to -0.58]; P=0.005}. Salbutamol use was also reduced with montelukast compared with salmeterol [difference in score= -0.47 (95% CI, -0.16 to -0.79); P<0.0001]. Greater improvements occurred in both symptom and quality of life scores with montelukast against salmeterol, whereas there was no difference in FEV1 (forced expiratory volume in 1 s). In conclusion, montelukast may be suitable as tailored second-line controller therapy instead of salmeterol in asthmatic children expressing the susceptible Arg16 genotype, a move towards a personalized medicine approach to management. © The Authors Journal compilation © 2013 Biochemical Society.

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