Implementing early rehabilitation and mobilisation for children in UK paediatric intensive care units: the PERMIT feasibility study.

Background: Early rehabilitation and mobilisation encompass patient-tailored interventions, delivered within intensive care, but there are few studies in children and young people within paediatric intensive care units. Objectives: To explore how healthcare professionals currently practise early rehabilitation and mobilisation using qualitative and quantitative approaches; co-design the Paediatric Early Rehabilitation and Mobilisation during InTensive care manual of early rehabilitation and mobilisation interventions, with primary and secondary patient-centred outcomes; explore feasibility and acceptability of implementing the Paediatric Early Rehabilitation and Mobilisation during InTensive care manual within three paediatric intensive care units. Design: Mixed-methods feasibility with five interlinked studies (scoping review, survey, observational study, codesign workshops, feasibility study) in three phases. Setting: United Kingdom paediatric intensive care units. Participants: Children and young people aged 0-16 years remaining within paediatric intensive care on day 3, their parents/guardians and healthcare professionals. Interventions: In Phase 3, unit-wide implementation of manualised early rehabilitation and mobilisation. Main outcome measures: Phase 1 observational study: prevalence of any early rehabilitation and mobilisation on day 3. Phase 3 feasibility study: acceptability of early rehabilitation and mobilisation intervention; adverse events; acceptability of study design; acceptability of outcome measures. Data sources: Searched Excerpta Medica Database, Cumulative Index to Nursing and Allied Health Literature, MEDLINE, PEDro, Open grey and Cochrane CENTRAL databases. Review methods: Narrative synthesis. Results: In the scoping review we identified 36 full-text reports evaluating rehabilitation initiated within 7 days of paediatric intensive care unit admission, outlining non-mobility and mobility early rehabilitation and mobilisation interventions from 24 to 72 hours and delivered twice daily. With the survey, 124/191 (65%) responded from 26/29 (90%) United Kingdom paediatric intensive care units; the majority considered early rehabilitation and mobilisation a priority. The observational study followed 169 patients from 15 units; prevalence of any early rehabilitation and mobilisation on day 3 was 95.3%. We then developed a manualised early rehabilitation and mobilisation intervention informed by current evidence, experience and theory. All three sites implemented the Paediatric Early Rehabilitation and Mobilisation during InTensive care manual successfully, recruited to target (30 patients recruited) and followed up the patients until day 30 or discharge; 21/30 parents consented to complete additional outcome measures. Limitations: The findings represent the views of National Health Service staff but may not be generalisable. We were unable to conduct workshops and interviews with children, young people and parents to support the Paediatric Early Rehabilitation and Mobilisation during InTensive care manual development due to pandemic restrictions. Conclusions: A randomised controlled trial is recommended to assess the effectiveness of the manualised early rehabilitation and mobilisation intervention. Future work: A definitive cluster randomised trial of early rehabilitation and mobilisation in paediatric intensive care requires selection of outcome measure and health economic evaluation. Study registration: The study is registered as PROSPERO CRD42019151050. The Phase 1 observational study is registered Clinicaltrials.gov NCT04110938 (Phase 1) (registered 1 October 2019) and the Phase 3 feasibility study is registered NCT04909762 (Phase 3) (registered 2 June 2021). Funding: This award was funded by the National Institute for Health and Care Research (NIHR) Health Technology Assessment programme (NIHR award ref: 17/21/06) and is published in full in Health Technology Assessment; Vol. 27, No. 27. See the NIHR Funding and Awards website for further award information.

Early rehabilitation and mobilisation, within the first week of intensive care admission, can improve the speed of recovery from illness or injury in adults. However, there is a lack of evidence about whether critically unwell children benefit from early rehabilitation and mobilisation. We aimed to identify which patients may benefit from early rehabilitation and mobilisation. Also, to develop and test a manual of early rehabilitation and mobilisation using the best evidence and expertise ­ called the Paediatric Early Rehabilitation and Mobilisation during InTensive care manual. Then evaluate whether the manual could be implemented safely in paediatric intensive care units and was acceptable to staff and families. We undertook in respect of early rehabilitation and mobilisation: review of existing research; national survey of practice (124 staff); gathered information about current conduct (15 paediatric intensive care units, 169 patients); spoke to experts (18 people); developed the Paediatric Early Rehabilitation and Mobilisation during InTensive care manual to guide paediatric intensive care unit staff; Tested the Paediatric Early Rehabilitation and Mobilisation during InTensive care manual in three paediatric intensive care units with 30 patients; gathered feedback from healthcare professionals via weekly 'debriefs' (47), interviews (13) and surveys (118), and from parents via parent-completed questionnaires (21) and interviews (14). Despite being regarded as important, currently early rehabilitation and mobilisation practice is inconsistent, not considered 'early' enough and often focuses on low-risk activities conducted on the bed. Introducing the Paediatric Early Rehabilitation and Mobilisation during InTensive care manual as part of a trial was acceptable and feasible and helps standardise delivery to unwell children. Measuring child and parent reported outcomes was acceptable but follow-up at 30 days was incomplete. A larger trial of early rehabilitation and mobilisation, involving more paediatric intensive care units, is feasible and required to demonstrate benefit to children.

Lessons Learned and New Challenges: Re-evaluation of End-User Assessment of a Skills-Based Training Program for Urology Trainees.

OBJECTIVES: To re-assess the perceived benefit and relevance of simulation sessions to Victorian urology trainees and to identify areas for potential improvement. SUBJECTS AND METHODS: All trainees attending skills training sessions between 2011 and 2016 were asked to complete a structured questionnaire at the completion of the session. The questionnaire included 11 topic areas ranging from the year of surgical training to degree of usefulness of the session, including several sections for free-text response to offer more detailed feedback. Sessions were examined both individually and collectively to assess end-user satisfaction with the structure and content of the program. RESULTS: In total, 24 individual skills sessions were held over the 6-year period, with a total of 355 attendees. Of these, 331 attendees completed the majority of the questionnaire, a response rate of over 93%. Overall 88% of the surveyed attendees stated that they had both the support of their supervising consultant and the flexibility of workload to attend the session; 90% of trainees felt that there was adequate reading material provided prior to the skills session, an improvement from 76% in the previous study period; and 97% of those surveyed felt that the existing session structure was appropriate and the same proportion found the sessions both useful and interesting, compared with just 63% in the previous study period. Analysis of individual topics demonstrates some variability in outcome measures, but for nearly every assessed parameter, greater than 90% of participants agreed that the session fulfilled the expected criteria. New topics developed since the 2011 analysis, including renal transplant and vascular repair, also had high levels of satisfaction. The practical models used have been refined and achieved higher scores than those in the previous assessment period. CONCLUSION: The urology skills-based training program has been well received by the surveyed trainees and is now embedded and accepted as part of the Victorian training program. The format of the sessions has matured and the overall rating, both individually and collectively, was high. There has been a clear increase in satisfaction across most areas assessed when compared with previous feedback. Despite this, there remain areas that can be improved, such as the amount and quality of available equipment and the inclusion of video demonstrations of operative techniques.

RARS2 mutations in a sibship with infantile spasms.

Pontocerebellar hypoplasia is a group of heterogeneous neurodevelopmental disorders characterized by reduced volume of the brainstem and cerebellum. We report two male siblings who presented with early infantile clonic seizures, and then developed infantile spasms associated with prominent isolated cerebellar hypoplasia/atrophy on magnetic resonance imaging (MRI). Using whole exome sequencing techniques, both were found to be compound heterozygotes for one previously reported and one novel mutation in the gene encoding mitochondrial arginyl-tRNA synthetase 2 (RARS2). Mutations in this gene have been classically described in pontocerebellar hypoplasia type six (PCH6), a phenotype characterized by early (often intractable) seizures, profound developmental delay, and progressive pontocerebellar atrophy. The electroclinical spectrum of PCH6 is broad and includes a number of seizure types: myoclonic, generalized tonic-clonic, and focal clonic seizures. Our report expands the characterization of the PCH6 disease spectrum and presents infantile spasms as an associated electroclinical phenotype.

SIU/ICUD Consultation on Urethral Strictures: Evaluation and follow-up.

For the 2010 International Consultation on Urethral Strictures, all available published data relating to the evaluation and follow-up of patients with anterior urethral stricture or posterior urethral stenosis were reviewed and evaluated. Selected manuscripts were classified by Level of Evidence using previously established criteria. Consensus was achieved through group discussion, and formal recommendations were established and graded on the basis of levels of evidence and expert opinion. Retrograde urethrography remains the de facto standard for the evaluation of patients with urethral stricture. It can readily be combined with voiding cystourethrography to achieve a synergistic evaluation of the entire urethra, and this approach is currently recommended as the optimal method for pretreatment staging. Cystoscopy is recommended as the most specific procedure for the diagnosis of urethral stricture and is a useful adjunct in the staging of anterior urethral stricture, particularly to confirm abnormal or equivocal findings on imaging studies. Cystoscopy is also an important modality for assessing the bladder neck and posterior urethra in the setting of a pelvic fracture-related urethral injury. Although urethrography and cystoscopy remain the principle forms of assessment of the patient with urethral stricture, additional adjuncts include uroflowmetry, symptom scores, quality of life assessments, ultrasonography, computed tomography, and magnetic resonance imaging. These modalities might be helpful to further evaluate patients in select circumstances or provide a less invasive approach to monitoring outcomes after surgical treatment. Further research is needed to establish consensus opinion as to the definition of success after urethroplasty and to develop standardized patient outcome measures.

Stomatin-deficient cryohydrocytosis results from mutations in SLC2A1: a novel form of GLUT1 deficiency syndrome.

The hereditary stomatocytoses are a series of dominantly inherited hemolytic anemias in which the permeability of the erythrocyte membrane to monovalent cations is pathologically increased. The causative mutations for some forms of hereditary stomatocytosis have been found in the transporter protein genes, RHAG and SLC4A1. Glucose transporter 1 (glut1) deficiency syndromes (glut1DSs) result from mutations in SLC2A1, encoding glut1. Glut1 is the main glucose transporter in the mammalian blood-brain barrier, and glut1DSs are manifested by an array of neurologic symptoms. We have previously reported 2 cases of stomatin-deficient cryohydrocytosis (sdCHC), a rare form of stomatocytosis associated with a cold-induced cation leak, hemolytic anemia, and hepatosplenomegaly but also with cataracts, seizures, mental retardation, and movement disorder. We now show that sdCHC is associated with mutations in SLC2A1 that cause both loss of glucose transport and a cation leak, as shown by expression studies in Xenopus oocytes. On the basis of a 3-dimensional model of glut1, we propose potential mechanisms underlying the phenotypes of the 2 mutations found. We investigated the loss of stomatin during erythropoiesis and find this occurs during reticulocyte maturation and involves endocytosis. The molecular basis of the glut1DS, paroxysmal exercise-induced dyskinesia, and sdCHC phenotypes are compared and discussed.

Treatment of primary angiitis of the central nervous system in childhood with mycophenolate mofetil.

OBJECTIVE: To assess the use of mycophenolate mofetil (MMF) in the treatment of refractory primary angiitis of the CNS in childhood (cPACNS). METHODS: A retrospective chart review was performed in patients with cPACNS who were treated with MMF following failure of a combination of corticosteroids and another immunosuppressant. RESULTS: Three patients from two centres were included in this study. The age of onset of disease was 5, 6 and 9 years. All the patients improved when treated with MMF, such that the dose of corticosteroids could be weaned or stopped. CONCLUSIONS: MMF should be considered for maintenance treatment in the management of patients with cPACNS refractory to the combination of corticosteroids and first-line immunosuppressive agents.

Intracranial pressure complicating severe traumatic brain injury in children: monitoring and management.

OBJECTIVE: To identify factors associated with the use of intracranial pressure (ICP) monitoring and to establish which ICP-targetted therapies are being used in children with severe traumatic brain injury (TBI) in the United Kingdom. To evaluate current practice against recently published guidelines. DESIGN AND SETTING: Prospective data collection of clinical and demographic information from paediatric and adult intensive care units in the UK and Ireland admitting children (< 16 years) with TBI between February 2001 and August 2003. RESULTS: Detailed clinical information was obtained for 501 children, with information on the use of ICP monitoring available in 445. ICP monitoring was used in only 59% (75/127) of children presenting with an emergency room Glasgow Coma Scale of 8 or below. Large between centre variation was seen in the use of ICP monitoring, independent of severity of injury. There were 86 children who received ICP-targetted therapies without ICP monitoring. Wide between centre variation was found in the use of ICP-targetted therapies and in general aspects of management, such as fluid restriction, the use of muscle relaxants and prophylactic anticonvulsants. Intra-ventricular catheters are rarely placed (6% of cases); therefore cerebrospinal fluid drainage is seldom used as a first-line therapy for raised ICP. Jugular venous bulb oximetry (4%), brain microdialysis (< 1%) and brain tissue oxygen monitoring (< 1%) are rarely used in current practice. Contrary to published guidelines, moderate to severe hyperventilation is being used without monitoring for cerebral ischaemia. CONCLUSIONS: There is an urgent need for greater standardisation of practice across UK centres admitting children with severe TBI.

Influence of control variables on mannequin temperature in a paediatric operating theatre.

BACKGROUND: Core temperature drops in all children having general anaesthesia. Convection heating may be useful, but its effectiveness in the paediatric setting is not established. Additionally, its utility in many paediatric situations is limited by blanket design. METHODS: Using a mannequin model in a sham operation, we assessed the likely safety and effectiveness of a draping technique in association with a 'Bair Hugger' and a heat dissipation unit (HDU). In Part 1 of the study, the influence of ambient temperature was assessed. In Part 2, a simulated laparotomy was set up and a more detailed assessment of air temperatures around the mannequin was made. In addition, the effect of a change in the HDU design was assessed. RESULTS: Part 1: the technique achieved 'near-plateau' temperature within 5-10 min. A difference of 8 degrees C in ambient temperature (between 18 and 26 degrees C) translated only to a 2-3 degrees C difference under the drapes. Part 2: the technique produced sidestream cooler zones at the head and shoulders. Air temperature at these sites was 28-34 degrees C, whereas at other points (irrespective of their distance from the heat source), it was 37-40 degrees C. Warm air reached sufficient skin sites to anticipate adequate heat transfer in the clinical situation. Air temperature at 'skin' surface stayed below 40 degrees C over the 90-min study period. CONCLUSIONS: A customized HDU used in association with a 'Bair Hugger' unit and a careful surgical draping technique provides stable, safe and consistent air temperatures around a mannequin. Net heat gain by a child's body should occur with this arrangement. Further evaluation in a clinical study is underway.