Birmingham Childrens Hospital Birmingham UK

Birmingham Childrens Hospital Birmingham UK

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Alfred A.,Rotherham Foundation NHS Trust Rotherham UK | Taylor P.C.,Rotherham Foundation NHS Trust Rotherham UK | Dignan F.,Central Manchester NHS Foundation Trust Manchester UK | El-Ghariani K.,Therapeutics and Tissue Services NHS Blood and Transplant Sheffield UK | And 13 more authors.
British Journal of Haematology | Year: 2017

Extracorporeal photopheresis (ECP) has been used for over 35 years in the treatment of erythrodermic cutaneous T-cell lymphoma (CTCL) and over 20 years for chronic and acute graft-versus-host disease (GvHD) and solid organ transplant rejection. ECP for CTCL and GvHD is available at specialised centres across the UK. The lack of prospective randomised trials in ECP led to the development of UK Consensus Statements for patient selection, treatment schedules, monitoring protocols and patient assessment criteria for ECP. The recent literature has been reviewed and considered when writing this update. Most notably, the national transition from the UVAR XTS® machine to the new CELLEX machine for ECP with dual access and a shorter treatment time has led to relevant changes in these schedules. This consensus statement updates the previous statement from 2007 on the treatment of CTCL and GvHD with ECP using evidence based medicine and best medical practise and includes guidelines for both children and adults. © 2017 John Wiley & Sons Ltd.

Kerlin B.A.,Ohio State University | Inbal A.,Tel Aviv University | Will A.,Royal Manchester Childrens Hospital ManchesterUK | Williams M.,Birmingham Childrens Hospital Birmingham UK | And 3 more authors.
Journal of Thrombosis and Haemostasis | Year: 2017

Essentials: Prophylaxis is the standard of care for congenital factor XIII-A (FXIII-A) deficiency. Six children with FXIII-A deficiency received once-monthly prophylaxis with recombinant FXIII-A. Prophylaxis was well tolerated and no anti-FXIII antibodies were detected. Prophylaxis was effective with an annualized bleeding rate of zero. Summary: Background: Factor XIII deficiency is a rare, severe congenital bleeding disorder. Monthly prophylaxis with recombinant FXIII A-Subunit (rFXIII) has demonstrated favorable safety and efficacy in patients aged ≥ 6 years, and may similarly benefit younger children. Objective: To evaluate the long-term safety and efficacy of rFXIII in children aged < 6 years with congenital FXIII A-subunit deficiency. Patients/methods: Six children, who had previously completed a single-dose pharmacokinetic trial of rFXIII, received 35 IU kg-1 rFXIII every 28 days (± 2 days) for a minimum of 52 weeks, and were evaluated for bleeding and adverse events. The Berichrom FXIII activity assay was used to monitor FXIII activity. Results: The children, three girls and three boys, had an average age of 3.0 years (range: 1-4 years) at enrollment. The total treatment duration was 1.8-3.5 years, giving a total of 16.6 patient-years. No antibody development, thromboembolic events or allergic reactions occurred. There were 93 mild and seven moderate adverse events. Two adverse events (lymphopenia and gastroenteritis) were reported as probably or possibly related to rFXIII in two children. Two serious adverse events, unrelated to rFXIII, were reported in a single child, each related to head injury, and neither resulting in intracranial hemorrhage. The geometric mean FXIII activity trough was 0.19 IU mL-1. No bleeding episodes requiring treatment with an FXIII-containing hemostatic agent occurred during the trial; thus, the annualized bleeding rate was 0. Conclusions: Consistent with data from older age groups, prophylaxis with rFXIII appears to be safe and effective in young children with congenital FXIII A-subunit deficiency. © 2017 International Society on Thrombosis and Haemostasis.

Swallow V.,University of Manchester | Smith T.,Royal Manchester Childrens Hospital Manchester UK | Webb N.J.A.,Royal Manchester Childrens Hospital Manchester UK | Wirz L.,Royal Infirmary | And 12 more authors.
Child: Care, Health and Development | Year: 2014

Background: Long-term childhood conditions are often managed by hospital-based multidisciplinary teams (MDTs) of professionals with discipline specific expertise of a condition, in partnership with parents. However, little evidence exists on professional-parent interactions in this context. An exploration of professionals' accounts of the way they individually and collectively teach parents to manage their child's clinical care at home is, therefore, important for meeting parents' needs, informing policy and educating novice professionals. Using chronic kidney disease as an exemplar this paper reports on one aspect of a study of interactions between professionals and parents in a network of 12 children's kidney units in Britain. Methods: We conducted semi-structured, qualitative interviews with a convenience sample of 112 professionals (clinical-psychologists, dietitians, doctors, nurses, pharmacists, play-workers, therapists and social workers), exploring accounts of their parent-educative activity. We analysed data using framework and the concept of distributed expertise. Results: Four themes emerged that related to the way expertise was distributed within and across teams: (i) recognizing each other's' expertise, (ii) sharing expertise within the MDT, (iii) language interpretation, and (iv) acting as brokers. Two different professional identifications were also seen to co-exist within MDTs, with participants using the term 'we' both as the intra-professional 'we' (relating to the professional identity) when describing expertise within a disciplinary group (for example: 'As dietitians we aim to give tailored advice to optimize children's growth'), and the inter-professional 'we' (a 'team-identification'), when discussing expertise within the team (for example: 'We work as a team and make sure we're all happy with every aspect of their training before they go home'). Conclusions: This study highlights the dual identifications implicit in 'being professional' in this context (to the team and to one's profession) as well as the unique role that each member of a team contributes to children's care. Our methodology and results have the potential to be transferred to teams managing other conditions. © 2014 The Authors.

Evans S.,Birmingham Childrens Hospital Birmingham UK | Daly A.,Birmingham Childrens Hospital Birmingham UK | Chahal S.,Birmingham Childrens Hospital Birmingham UK | Macdonald J.,Birmingham Childrens Hospital Birmingham UK | Macdonald A.,Birmingham Childrens Hospital Birmingham UK
Journal of Human Nutrition and Dietetics | Year: 2015

Background: In phenylketonuria (PKU), little is known about the effect of bitter-tasting phenylalanine-free l-amino acid exposure on taste preference development. The present prospective study aimed to determine the flavour preferences of children with PKU versus healthy control children. Methods: Thirty-five children with PKU and 35 age/gender-matched controls, aged 4-13 years, tasted 10 blinded puree foods in random order. They were rated using a seven-point pictorial hedonic scale (super yummy to super yucky) and ranked in preferential order. Caregivers completed a neophobia and food frequency questionnaire on behalf of their children. Results: Both PKU and control groups rated sweet foods higher than savoury, bitter and sour foods. However, control children ranked fruits as a group higher than PKU children (mean 3.7 versus 4.6; P = 0.03), whereas PKU children ranked vegetables as a group higher than controls (mean 5.6 versus 6.3; P = 0.05). Children with PKU had more neophobia and were untrusting/fearful of new foods. Conclusions: Although there was some evidence to suggest that children with PKU aged ≥4 years prefer savoury foods (vegetables) more than control children, they did not prefer bitter-tasting foods, and so early and persistent administration of bitter-tasting l-amino acids was not associated with apparent taste imprinting. Neophobia appears to play significant part in food refusal in PKU, perhaps more so than taste preferences. © 2015 The British Dietetic Association Ltd.

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