Management of severe pulmonary Langerhans cell histiocytosis in children.

Patients with pulmonary Langerhans cell histiocytosis (LCH) typically have a benign course but may have extensive cystic lung disease with rare life-threatening complications including multiple and recurrent pneumothoraces and respiratory failure. We report seven severely affected pediatric patients treated with chemotherapy, aggressive chest tube management, and pleurodesis of whom five survived. Patients with extraordinary amounts of pulmonary cystic disease and multiple pneumothoraces due to LCH can have remarkable, curative outcomes with early recognition, optimal LCH-directed therapy, and supportive care.

Life-threatening hypersensitivity pneumonitis secondary to e-cigarettes.

We report a case of hypersensitivity pneumonitis (HP) in a young person secondary to vaping. He presented with a putative diagnosis of asthma and required extracorporeal membrane oxygenationbecause of intractable respiratory failure. He developed a critical illness and steroid myopathy and required prolonged rehabilitation. Our patient fulfils diagnostic criteria for HP secondary to e-cigarettes with a positive exposure history, deterioration after skin prick testing, specific serum IgM antibodies against the implicated liquid raising the possibility that the relevant antigen was present in that liquid and radiological and histopathological features compatible with acute HP. There are two learning points. The first is always to consider a reaction to e-cigarettes in someone presenting with an atypical respiratory illness. The second is that we consider e-cigarettes as 'much safer than tobacco' at our peril.

Blunt splenic injury in children: haemodynamic status key to guiding management, a 5-year review of practice in a UK major trauma centre.

PURPOSE: To review the management of children and adolescents (0-18 years), with blunt splenic injury treated at a single UK major trauma centre over a 5-year period, focusing upon efficacy of non-operative management and the use of haemodynamic stability as a guide to planning treatment strategy, rather than radiological injury grading. To produce a treatment pathway for management of blunt splenic injury in children. METHODS: Retrospective, cross-sectional study of all paediatric patients admitted with radiologically proven blunt splenic injury between January 2011 and March 2016. Penetrating injuries were excluded. Follow up was for at least 30 days. RESULTS: 30 Patients were included, mean age was 14.5 (SD 3.6), median injury severity score was 16 (IQR 10-31). 6 Patients (20%) had a splenectomy, whilst 22 patients (73%) were successfully treated non-operatively with 100% efficacy at index admission. 5/8 (63%) patients with radiological grade V injuries were managed non-operatively, injury grade was not associated with surgical intervention (p = 1.57). Haemodynamic instability was initially treated with fluid resuscitation leading to successful non-operative management in 5/11 (45%) patients. However, haemodynamic instability is a significant predictor of requirement for surgical intervention (p = 0.03), admission to critical care (p = 0.017), presence of additional injuries (p = 0.015) and increased length of stay (p = 0.038). No such relationships were found to be associated with increased radiological injury grade. CONCLUSIONS: Non-operative management should be first-line treatment in the haemodynamically stable child with a blunt splenic injury and may be carried out with a high degree of efficacy. It may also be successfully implemented in those initially showing signs of haemodynamic instability that respond to fluid resuscitation. Radiological injury grade does not predict definitive management, level of care, or length of stay; however, haemodynamic stability may be utilised to produce a treatment algorithm and is key to guiding management.

Electrical impedance tomography effectively used in a case of paediatric pulmonary Langerhans cell histiocytosis.

A 2-year-old boy with severe pulmonary Langerhans cell histiocytosis presented in extreme respiratory failure. He was intubated and ventilated. Despite maximal support, he deteriorated and needed extremely high ventilator pressures. An electrical impedance tomography monitor was used to inform management. This is a monitoring technique which is not used in children due to the lack of suitable interface devices and a lack of randomised clinical evidence. Despite technical difficulties, a good signal was achieved. This informed management and enabled the selection of a suitable ventilator strategy, facilitating weaning. Electrical impedance tomography is a viable technology for use in paediatric critical respiratory failure. This is a non-invasive and safe technology which adds individual patient information which is not available through any other modalities. We urge equipment manufacturers to develop belts which will allow routine application of this life-saving technology in children.

Oral sildenafil as a rescue therapy in presumed acute pulmonary hypertensive crisis.

A 23-week-old baby, born at 26(+2) weeks, presented to the hospital with critical respiratory failure, which was impossible to stabilize. She had unstable oxygen saturations between 35% and 95%. A presumptive diagnosis of bronchopulmonary dysplasia with associated pulmonary hypertensive crisis was made. In the absence of inhaled nitric oxide, 2 oral doses of 1 mg/kg sildenafil were given, with a dramatic improvement 30 to 45 minutes later. Her oxygenation index fell from 43 to 14. She made a full recovery and was discharged from the hospital 2 weeks later.