Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each lesion, both antenatally and postnatally, is described and illustrated. A number of quandaries exist in the prediction of prognosis and subsequent treatment of BPFM. We discuss the radiological features that may help to elucidate an individual prognosis and aid in the planning of treatment. The treatment options available for BPFM are briefly discussed. Finally, the link between BPFM, in particular cystic adenomatoid malformations and malignancy, is discussed. We aim to provide a comprehensive overview of the embryology, radiology, prognosis and treatment highlighting contentious issues of BPFM.
Takayasu Arteritis (TA) is a rare, debilitating large vessel vasculitis occurring in patients of all ages, including infants, but the disease most commonly presents in the third decade. Diagnosis is often delayed and consequently TA is associated with significant morbidity and mortality. Accurate methods of monitoring disease activity or damage are lacking and currently rely on a combination of clinical features, blood inflammatory markers, and imaging modalities. In this report we describe a case of a 14-year-old boy with childhood-onset TA who, despite extensive negative investigations, did indeed have on-going active large vessel vasculitis with fatal outcome. Postmortem analysis demonstrated more extensive and active disease than originally identified. This report illustrates and discusses the limitations of current modalities for the detection and monitoring of disease activity and damage in large vessel vasculitis. Clinicians must be aware of these limitations and challenges if we are to strive for better outcomes in TA.
ObjectiveDosing errors can cause significant harm in paediatric healthcare settings. Our objective was to investigate the effects of paediatric dose range checking (DRC) clinical decision support (CDS) software on overdosing-related outcomes.MethodsA before-after study and a semistructured survey of prescribers was conducted across inpatient wards (excluding intensive care) in a regional children’s hospital. DRC CDS software linked to a paediatric drug formulary was integrated into an existing electronic prescribing system. The main outcome measures were; the proportion of prescriptions with overdosing errors; overdosing-related clinical incidents; severity of clinical incidents; and acceptability of the intervention.ResultsThe prescription overdosing error rate did not change significantly following the introduction of DRC CDS software: in the preintervention period 12/847 (1.4%) prescriptions resulted in prescription errors and in the postintervention period there were 9/684 (1.3%) prescription overdosing errors (n=21, Pearson χ2 value=0.028, p=0.868). However, there was a significant trend towards a reduction in the severity of harm associated with reported overdosing incidents (n=60, Mann-Whitney U value=301.0, p=0.012). Prescribers reported that the intervention was beneficial and they were also able to identify factors that may have contributed to the persistence of overdosing errors.ConclusionDRC CDS software did not reduce the incidence of prescription overdosing errors in a paediatric hospital setting but the level of harm associated with the overdosing errors may have been reduced. Use of the software seemed to be safe and it was perceived to be beneficial by prescribers.
Objectives Despite medical advances, life-changing articular damage may still occur in patients with JIA. We report a cohort with destructive arthropathy of the ankle treated by surgical arthrodiastasis. Methods Eight patients (nine ankles) received arthrodiastasis by means of an Ilizarov frame between 2009 and 2013. Patient- and clinician-reported outcome measures were collated prospectively, with retrospective analysis of demographics, disease and pre-surgical treatment. Results Pre-surgery, all patients received IA CS (mean 0.8 injections/year) and MTX (mean diagnosis to treatment 3.8 years; two of eight started within 3 months). Seven of eight patients received biologic drugs. Pain scores improved by 56 and 29% (P < 0.005) at 6 and 12 months post-frame removal. American Academy Orthopaedic Foot and Ankle Society ankle–hindfoot scale, Oxford Ankle Foot Questionnaire-Child and Oxford Ankle Foot Questionnaire-Parent scores improved by 171, 62 and 80%, respectively (P < 0.005) at 12 months post-frame removal. Patients remained satisfied with surgical treatment for a mean of 13.3 months. There was transient pin site infection in three patients, and all patients had radiological improvement in joint space. Conclusion Arthrodiastasis with an Ilizarov frame is a safe, well-tolerated technique that should be considered as a short-term joint-preserving procedure to improve pain and function when damage has occurred. Delays to systemic medical treatment in this cohort would be considered out-with standard modern practice but, although less prevalent, destructive ankle arthropathy continues to occur in JIA, and we believe this study to be relevant. The ankle is particularly susceptible to damage and, even if localized, should be treated early and aggressively with DMARDs and rapid progression to biologic therapies. Levelof evidence Level IV.
Aims We describe successful arthrodiastasis by the means of an Ilizarov frame in two children with juvenile idiopathic arthritis (JIA) with localised destructive disease despite medical therapy. Methods Case 1 - A 2 year old girl was diagnosed with polyarticular JIA (rheumatoid factor (RF) negative, intermittent antinuclear antibody (ANA) positive). Active symptomatic disease progressed despite combinations of intra-articular steroids, methotrexate (MTX), and biologic agents. Magnetic resonance imaging (MRI) showed erosive change of the left ankle with complete loss of joint space and subtalar joints, and by the age of 13 her mobility was increasingly limited. In order to control pain and prevent permanent arthrodesis, Ilizarov distraction of the left ankle was performed. Case 2 - A 13 year old girl with 10 year history of polyarticular JIA (ANA positive RF negative) developed destructive arthropathy in her right ankle with bone on bone crepitus despite treatment with MTX and etanercept. Serial MR scans showed progressive destructive disease. The patient was treated with cross ankle distracting Ilizarov frame with a perioperative course of intravenous methyprednisolone (IVMP) to control synovitis. She had minor skin sepsis at pin sites. Results In both cases, an improvement has been shown post-operatively in terms of reduced pain and improved function. There is radiological evidence of improvement in both patients. Conclusion JIA is a potentially destructive inflammatory disease. Treatment by a skilled multidisciplinary team with access to disease modifying drugs including biologic therapies usually prevents articular damage and disabling symptoms. Joint destruction is rare, but can significantly impact on the quality of life through pain, deformity, and loss of function. Arthrodiastasis, or joint distraction using an orthopaedic frame, can be used as rescue procedure to avoid arthrodesis in severely painful damaged joints unresponsive to medical therapies.
Aim Reported paediatric prescribing error rates are up to 13% and in our trust a baseline audit found our local error rate was 5.4%. A number of these errors are made by non paediatricians prescribing for paediatric patients. We wanted to increase their confidence in paediatric prescribing to try and reduce paediatric prescribing errors. Method We organised interactive case based teaching sessions for both the emergency medicine doctors and surgical doctors who commonly prescribe for our paediatric patients. We asked doctors to self-rate their confidence from strongly agree to strongly disagree with regards to prescribing in paediatrics. We then asked them to self-rate their confidence after the teaching session. Results Prior to the teaching sessions the majority of doctors said they either strongly disagreed or disagreed about their confidence in paediatric prescribing (25 out of 34 doctors). After the teaching sessions all doctors said they either agreed or strongly agreed that the session had increased their confidence in prescribing. Furthermore, we received many positive comments, in particular doctors appreciating the opportunity to practice prescribing for paediatric patients in an interactive environment. A repeat audit has shown a reduction in local error rate to an average of 3.4% from January to September 2018. We feel that the teaching sessions for non-paediatricians have helped to play a role with this. Conclusion Increasing confidence in paediatric prescribing for non-paediatricians is extremely important. We used interactive case studies in our teaching sessions, although we know this does not replicate seeing an actual patient, giving doctors a safe environment in which to learn in and ask questions helped improved confidence in prescribing. We feel that these teaching sessions have also helped to reduce our local error rate.Background Gastrostomy skin related stoma complications in children and young people (CYP) have negative effects and can be managed by a range of people including families, carers, special school staff, nurses and doctors. Within one educational institution we noted variations in practice, knowledge and leadership in gastrostomy problems management, this project was to explore reasons for this.No local or nationally recognised guidance were identified to help consistent management. Aims and objectives To evaluate current knowledge around gastrostomy complications and confidence of staff involved in managing these.
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