Objectives To report the 12-month prevalence of joint bleeds from the National Haemophilia Database (NHD) and Haemtrack, a patient-reported online treatment diary and concurrent joint disease status using the haemophilia joint health score (HJHS) at individual joint level, in children and adults with severe haemophilia A and B (HA/HB) without a current inhibitor. Design A 2018 retrospective database study of NHD from which 2238 cases were identified, 463 patients had fully itemised HJHS of whom 273 were compliant in recording treatment using Haemtrack. Setting England, Wales and Scotland, UK. Participants Children (<18 years) and adults (≥18 years) with severe HA and HB (factor VIII/factor IX, <0.01 iu/mL) without a current inhibitor. Primary and secondary outcomes Prevalence of joint haemarthrosis and concurrent joint health measured using the HJHS. Results The median (IQR) age of children was 10 (6–13) and adults 40 (29–50) years. Haemarthrosis prevalence in HA/HB children was 33% and 47%, respectively, and 60% and 42%, respectively, in adults. The most common site of haemarthrosis in children was the knee in HA and ankle in HB. In adults, the incidence of haemarthrosis at the ankles and elbows was equal. The median total HJHS in HA/HB children was 0 and in adults with HA/HB, were 18 and 11, respectively. In adults with HA/HB, the median ankle HJHS of 4.0 was higher than the median HJHS of 1.0 for both the knee and elbow. Conclusion Despite therapeutic advances, only two-thirds of children and one-third of adults were bleed-free, even in a UK cohort selected for high compliance with prophylaxis. The median HJHS of zero in children suggests joint health is relatively unaffected during childhood. In adults, bleed rates were highest in ankles and elbows, but the ankles led to substantially worse joint health scores.

Introduction In good risk patients (historic inhibitor peak < 200BU), the International Immune Tolerance Study demonstrated equal efficacy to induce tolerance between high (200iu/kg/day) and low dose (50iu/kg x3 times/week) immune tolerance induction (ITI) regimens. However, the trial stopped early on account of the excessive bleed rate in the low dose ITI arm. Methods United Kingdom Haemophilia Centre Doctors' Organization (UKHCDO) Paediatric and Inhibitor working parties considered available ITI data alongside the bi-phenotypic antibody emicizumab (Hemlibra (R)) efficacy and safety data to develop a consensus guideline for the future UK ITI guideline. Results This revision of UKHCDO ITI guidance incorporates the recommendation to use emicizumab as a prophylaxis haemostatic agent to reduce bleeding rates and to facilitate low dose and reduced frequency of FVIII CFC for ITI in the majority of children. Conclusion This consensus protocol will facilitate future evaluation of ITI outcomes in the evolving landscape of haemophilia therapeutics and ITI strategies.

Correction to: British Journal of Cancer (2010) 103, 1448–1452. doi:10.1038/sj.bjc.6605903 After publication of this article in 2010, it was noted that there was an error in the data format used for the analyses of incidence rates throughout the paper, which led to overestimation of the population denominators.

Increasing cancer incidence together with improved survival rates are contributing to the growing number of cancer survivors. Survivors may encounter a range of potential effects as a result of the cancer itself or cancer treatments. Traditionally, the major focus of follow-up care has been on detection of cancer recurrence; however, the efficacy of such strategies is questionable. Traditional follow-up frequently fails to identify or adequately address many survivors' concerns. Aftercare needs to be planned to enable better outcomes for survivors, while using scarce health-care resources efficiently. This review focuses on provision of survivorship care, rather than on research. England's National Cancer Survivorship Initiative has developed principles for improved care of those living with and beyond cancer. These include risk-stratified pathways of care, the use of treatment summaries and care plans, information and education to enable choice and the confidence to self manage, rapid re-access to specialist care, remote monitoring and well-coordinated care. Many of these principles are relevant internationally, though preferred models of care will depend on local circumstances.

HaemophiliaVolume 17, Issue 4 p. 711-712 LETTERS TO THE EDITORS A case report of a premature infant with haemophilia A and factor VIII inhibitor P. CARTLEDGE, P. CARTLEDGE Neonatal Unit, St James’s Hospital, Leeds, UKSearch for more papers by this authorK. DEAKIN, K. DEAKIN Neonatal Unit, St James’s Hospital, Leeds, UKSearch for more papers by this authorL. McKECKNIE, L. McKECKNIE Neonatal Unit, St James’s Hospital, Leeds, UKSearch for more papers by this authorM. RICHARDS, M. RICHARDS Department of Paediatric Haematology, Children’s Day Hospital, St. James’s University, Hospital, Leeds, UKSearch for more papers by this author P. CARTLEDGE, P. CARTLEDGE Neonatal Unit, St James’s Hospital, Leeds, UKSearch for more papers by this authorK. DEAKIN, K. DEAKIN Neonatal Unit, St James’s Hospital, Leeds, UKSearch for more papers by this authorL. McKECKNIE, L. McKECKNIE Neonatal Unit, St James’s Hospital, Leeds, UKSearch for more papers by this authorM. RICHARDS, M. RICHARDS Department of Paediatric Haematology, Children’s Day Hospital, St. James’s University, Hospital, Leeds, UKSearch for more papers by this author First published: 04 March 2011 https://doi.org/10.1111/j.1365-2516.2010.02455.xCitations: 6 Dr Michael Richards, Consultant Paediatric Haematologist, Department of Paediatric Haematology, Children’s Day Hospital, St. James’s University Hospital, Leeds LS9 7TF, UK.Tel: +44 0113 2066295; fax: +44 0113 2470248;e-mail: michael.richards@leedsth.nhs.uk Dr Peter Cartledge: SpR in General Paediatrics. Department of General Paediatrics, Leeds Children’s Hospital, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK Dr Kathryn Deakin: Consultant paediatrician, Pinderfields General Hospital, Aberford Road, Wakefield WF1 4DG, UK Dr Liz McKecknie: Consultant Neonatologist. Neonatal unit, Leeds Children’s Hospital, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume17, Issue4July 2011Pages 711-712 RelatedInformation

Evidence-based guidelines are presented for the management of haemophilia in the fetus and neonate. This includes information regarding the management of pregnancy and delivery as well as aspects of management during the early neonatal period. Specific issues regarding the mode of delivery and the risk of intra-cranial and extra-cranial haemorrhage are discussed.

Objective Few studies have examined differences in the epidemiology of cancer between ethnic groups for children and young adults in the UK. We investigated incidence rates, associated trends and survival by ethnicity (south Asian vs all other ethnic groups) across childhood (0–14) and young adult (15–29) ages using a unique specialist cancer register. Methods The data used for this study were extracted from the Yorkshire Specialist Register of Cancer in Children and Young People. Patients diagnosed from 1990 to 2005 in the former Yorkshire Regional Health Authority were included in the analysis. Ethnicity was assigned using name analysis programs and Hospital Episode Statistics data. Incidence rates (per 1 000 000 person-years) by ethnic group were derived using mid-year population estimates. Poisson regression was used to examine trends in incidence by ethnicity and diagnostic sub-group, adjusting for sex and age. An interaction term between year and ethnicity was added to the model and likelihood-ratio test used to determine whether incidence trends differed for south and non south Asians. Survival rates were assessed using Kaplan-Meier estimates and log-rank tests. Cox regression was used to assess the effect of ethnicity on survival, adjusting for age, sex, year and deprivation. Results Overall cancer incidence was similar for south Asians (12.1; 95% CI 10.7 to to 13.5, n=275) and non-south Asians (12.6; 95% CI 12.2 to to 13.1, n=3259). For non-south Asians, incidence rates increased on average by 1.5% per year (95% CI 0.8 to to 2.3); the rate of increase for south Asians was significantly higher (7.0%; 95% CI 4.2 to to 9.9). Survival rates were significantly poorer for 15–29 vs 0–14 year olds (HR 1.25; 95% CI 1.09 to to 1.43). A significant increased risk of death was seen for south Asians compared to non-south Asians with leukaemia (HR 1.76; 95% CI 1.10 to to 2.81) and lymphoma (HR 3.11; 95% CI 1.61 to to 5.99). South Asians with other solid tumours had a significantly reduced risk of death (HR 0.43 95% CI 0.23 to to 0.81) compared to non-south Asians. Conclusion If present trends continue, the higher rate of increase seen among the Asian population in Yorkshire will result in 3-times higher incidence than non-south Asians by 2020. Lower survival rates seen for south Asians with leukaemia and lymphoma and for 15–29 year olds warrant further detailed investigation.

Major haemorrhage in neonates with haemophilia - Frequency and risk factors – European cohort study

Although most surgical and invasive procedures can be performed safely in patients with haemophilia, the optimal level and duration of replacement therapy required to prevent bleeding complications have not been established conclusively. For providing more insight into optimal therapy during invasive procedures, a literature review of surgical procedures in patients with haemophilia was conducted. Concomitantly, current practice was surveyed in 26 European Haemophilia Comprehensive Care Centres, representing 15 different countries. The review identified 110 original papers published between 1965 and 2007. Of these, only two studies were randomized controlled trials. Target levels and the duration of replacement therapy in the published studies were as follows. For major orthopaedic surgery: preoperative targets were 80-90%; postoperative targets showed a high degree of variation, with trough levels ranging from 20% to 80%, duration 10-14 days; for liver biopsy, 70-100%, 1-7 days; tonsillectomy: 90-100%, 5-11 days; indwelling venous access device insertion: 100%, 3-10 days; circumcision: 50-60%, 2-4 days; dental surgery: 30-50%, single treatment. With the exception of dental surgery, current practice in Europe, as assessed by the survey, was largely in agreement with published data. In conclusion, this study provides both a comprehensive review and a large survey of replacement therapy in patients with haemophilia undergoing invasive procedures; these data have informed the consensus practical treatment recommendations made in this paper. This study highlights the need for better-designed studies in order to better define minimal haemostatic levels of replacement therapy and optimal treatment duration.

HaemophiliaVolume 13, Issue 6 p. 758-759 Venous thrombosis in Glanzmann’s thrombasthenia R. PHILLIPS, R. PHILLIPS Department of Paediatric Haematology, Children’s Day Hospital, St James’s Hospital, Leeds, UKSearch for more papers by this authorM. RICHARDS, M. RICHARDS Department of Paediatric Haematology, Children’s Day Hospital, St James’s Hospital, Leeds, UKSearch for more papers by this author R. PHILLIPS, R. PHILLIPS Department of Paediatric Haematology, Children’s Day Hospital, St James’s Hospital, Leeds, UKSearch for more papers by this authorM. RICHARDS, M. RICHARDS Department of Paediatric Haematology, Children’s Day Hospital, St James’s Hospital, Leeds, UKSearch for more papers by this author First published: 19 September 2007 https://doi.org/10.1111/j.1365-2516.2007.01555.xCitations: 17 Dr Bob Philips, BMBCh, MA, MRCPCH, Specialist Registrar in Paediatric Oncology, Department of Paediatric Haematology, Children’s Day Hospital, St James’s Hospital, Beckett Street, Leeds LS9 7TF, UK. Tel.: +44 113 2065710; fax: +44 113 247 0248;e-mail: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article.Citing Literature Volume13, Issue6November 2007Pages 758-759 RelatedInformation

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We identified five disease-causing mutations in six factor XIII deficient patients from four unrelated families: two novel nonsense mutations (nucleotide 979C-->T corresponding to Arg326Stop; and nucleotide 2075G-->A corresponding to Trp691 Stop), one novel deletion of a single nucleotide (nucleotide 708G or 709G), one previously reported missense mutation (nucleotide 888C-->G corresponding to Ser295Arg), and a previously reported splice site mutation (nucleotide 319G-->T at the last position of exon 3). The phenotypic consequences of these mutations are discussed.

The burden of childhood cancer for Primary Care Trusts (PCTs) is unknown. PCTs in Yorkshire are representative of England and Wales and show little heterogeneity in the incidence rates of childhood cancer. Each PCT will expect three to five newly diagnosed children per year. A single GP is likely to see an incident case once every 20 years.