Fifteen-minute consultation: An overview of major incidents.

Major incidents are rare but require a large amount of preparation, co-ordination and communication across different emergency services and specialities. This ensures that casualties are efficiently managed within the constraints of limited clinical resources. This article aims to provide a brief understanding of what constitutes as a major incident, how it is declared and the chain of command in communication and action, focusing specifically on the paediatric process. It also aims to highlight important considerations that could potentially be missed (eg, the mental health impact, forensic evidence and so on).

Fifteen-minute consultation: Recognition of sickle cell crises in the paediatric emergency department.

Children with sickle cell disease can develop life-threatening and painful crises that require prompt assessment and efficient management by healthcare professionals in the emergency or acute care setting. Due to migration patterns and improved survival rates in high-prevalence countries, there is an increased tendency to encounter these patients across the UK. These factors warrant regular revisions in sickle cell crisis management, along with education for medical personnel and patients to improve clinical care and patient management. The focus of this article is on the initial assessment and management of acute paediatric sickle cell complications in the emergency setting. Specific case studies, including acute pain crises, trauma, splenic sequestration, aplastic crises, acute chest syndrome, infection, avascular necrosis, osteomyelitis and stroke, are discussed. Due to the current COVID-19 pandemic, we have also reviewed specific concerns around this patient group.

Improving rates of appropriate skeletal survey use in a district general paediatric emergency department.

Improving rates of appropriate skeletal survey use in a district general paediatric emergency department.

Cancer immunotherapy with CAR-T cells - behold the future.

Cellular therapy is a key tool to treat haematological malignancies. Over 40,000 allogeneic and autologous haematopoietic stem cell transplants (HSCTs) are performed annually across Europe.1 Since 2017, a new T cell therapy, chimeric antigen receptor-T (CAR-T) cells have been licensed outside clinical trials. CAR-T cells have extremely potent antitumour activity, but also have a profile of toxic side effects not seen before. Cytokine release syndrome (CRS) and CAR-T cell-related encephalopathy syndrome (CRES) are common, predictable and potentially lethal side effects. Patients frequently require admission to intensive care, and management from a number of medical specialties. This exciting and powerful new therapy requires the formation of new multispecialty medical teams for safe delivery and to successfully manage the resultant complications.

Prospective evaluation of the Whitt Neonatal Trigger Score in an 'at-risk' neonatal population.

AIM: The aim of this study was to prospectively evaluate the Whitt Neonatal Trigger Score (W-NTS), determining optimum threshold scores for consideration of medical intervention and intensive care unit admission. METHODS: All neonates on the postnatal ward (PNW) with a set of pre-defined risk factors were scored on the W-NTS. Neonates were divided into three groups: 'unwell' admitted to neonatal intensive care unit (NICU); 'well', who remained on the PNW receiving standard care; and 'intervention', who received antibiotics but did not require admission to NICU. RESULTS: A total of 3315 scores from 455 neonates were collected. The W-NTS area under the receiver operating characteristic curve (AUC ROC) was 0.968, with a score of 2 or more predicting NICU admission, with 82.5% sensitivity and 95.0% specificity. Adopting a cut-off score of 2 for admission would significantly improve speed to admission (11.6 vs. 6.9 h, P 0.037). A score of 0 was strongly predictive of being well enough to remain on the PNW without intervention (odds ratio 565.6, P < 0.001), and a score of 1 or more predicted the need for intravenous antibiotics with 100.0% sensitivity and 86.1% specificity (AUC ROC 0.977). CONCLUSION: The W-NTS observation chart, previously shown to outperform existing early warning scores, acts well as an adjunct to clinical assessment on the PNW, with its simplicity allowing for the successful and safe use by non-paediatric specialists. We recommend that neonates scoring 1 should be reviewed, with a septic screen and commencement of antibiotic therapy considered, while those scoring 2 or more should be strongly considered for NICU admission for further management.

Cerebro-costo-mandibular syndrome: Clinical, radiological, and genetic findings.

Cerebro-Costo-Mandibular syndrome (CCMS) is a rare autosomal dominant condition comprising branchial arch-derivative malformations with striking rib-gaps. Affected patients often have respiratory difficulties, associated with upper airway obstruction, reduced thoracic capacity, and scoliosis. We describe a series of 12 sporadic and 4 familial patients including 13 infants/children and 3 adults. Severe micrognathia and reduced numbers of ribs with gaps are consistent findings. Cleft palate, feeding difficulties, respiratory distress, tracheostomy requirement, and scoliosis are common. Additional malformations such as horseshoe kidney, hypospadias, and septal heart defect may occur. Microcephaly and significant developmental delay are present in a small minority of patients. Key radiological findings are of a narrow thorax, multiple posterior rib gaps and abnormal costo-transverse articulation. A novel finding in 2 patients is bilateral accessory ossicles arising from the hyoid bone. Recently, specific mutations in SNRPB, which encodes components of the major spliceosome, have been found to cause CCMS. These mutations cluster in an alternatively spliced regulatory exon and result in altered SNRPB expression. DNA was available from 14 patients and SNRPB mutations were identified in 12 (4 previously reported). Eleven had recurrent mutations previously described in patients with CCMS and one had a novel mutation in the alternative exon. These results confirm the specificity of SNRPB mutations in CCMS and provide further evidence for the role of spliceosomal proteins in craniofacial and thoracic development.