Case presentation: persistent adenovirus B3 infections associated with bronchiolitis obliterans treated with cidofovir in a child with mosaic tetrasomy 9p.

BACKGROUND: Adenoviruses (AdV) are non-enveloped, double-stranded DNA viruses with multiple serotypes, which cause a variety of end-organ disease in both immunocompetent and immunocompromised individuals. Some adenoviruses can become latent in the mucosa-associated lymphoid tissue (e.g. adenoids and tonsils), with the potential to reactivate sporadically, leading to upper or lower respiratory tract infection and disease. Bronchiolitis Obliterans (BO) is a rare chronic lung disorder which usually follows a severe insult to the respiratory tract. In children, it is a complication of severe infections (as post-infectious BO), typically manifesting after a severe respiratory infection, in previously healthy pre-school children. Symptoms and signs of air trapping (hyperinflated chest, expiratory wheeze) with persistent oxygen requirement are characteristic. The presence of the unusual mosaic tetrasomy 9p genotype in this case, despite standard cidofovir therapy for persistent or chronic adenovirus infection, may have impacted on the child's long-term clinical outcomes. CASE PRESENTATION: We present a case of persistent AdV B3 infection in a 14-month old boy with mosaic tetrasomy 9p, which persisted for 10 weeks, resulting in radiologically-confirmed BO, requiring cidofovir to control the persistent AdV B3 infection and standard therapy with pulsed steroids. We argue that in the presence of the mosaic tetrasomy 9p, earlier antiviral therapy may have decreased the severity of BO, as this mutation is known to be associated with some degree of immune dysregulation. CONCLUSIONS: Adenovirus infections are common in children and may persist as latent infections, with subsequent reactivations during loss of immune control, related to systemic illness arising from other causes. In chronic, reactivated AdV infection with pneumonia, BO is a recognised complication. However, in this case, with the presence of the mosaic tetrasomy 9p mutation, earlier antiviral therapy may have reduced such longer term complications, due to the immune dysregulatory nature of this mutation.

Cluster of human parechovirus infections as the predominant cause of sepsis in neonates and infants, Leicester, United Kingdom, 8 May to 2 August 2016.

We report an unusually high number of cases (n = 26) of parechovirus infections in the cerebrospinal fluid (CSF) of neonates and infants admitted with sepsis in the United Kingdom during 8 May to 2 August 2016. Although such infections in neonates and infants are well-documented, parechovirus has not been routinely included in many in-house and commercial PCR assays for CSF testing. Clinicians should consider routine parechovirus testing in young children presenting with sepsis.

Food protein-induced enterocolitis syndrome in the British Isles.

BACKGROUND: Food protein-induced enterocolitis syndrome (FPIES) is a delayed type of food allergy, most often seen in infancy. We aimed to estimate its incidence, to describe common food triggers and the patient journeys of this rare but serious condition. DESIGN: We undertook a prospective epidemiological survey of FPIES using the British Paediatric Surveillance Unit. SETTING: UK and Ireland. PARTICIPANTS: A survey of all paediatricians over 13 months between January 2019 and February 2020. MAIN OUTCOME MEASURES: 204 cases were reported, 98 (48%) meeting case definition, giving an incidence of 0.006% for England based on 93 cases. RESULTS: 98 patients reported 135 trigger foods, 27% (26 of 98) had multiple food triggers. Common food triggers included cow's milk (24%, 33 of 135), fruits and vegetables (19%, 26 of 135), hen's egg (16%, 22 of 135) and fish (14%, 19 of 135). In 46% (41 of 90), the initial trigger food had been ingested three or more times before diagnosis, with a median diagnostic delay of 7.9 months (3.0, 17.3). Half (50 of 98) were admitted, yet only 5% (5 of 98) received appropriate acute treatment with ondansetron. Most cases were diagnosed by an allergy specialist (74 of 98, 76%), within a median of 7.5 (3.0, 13.3) miles from home. CONCLUSION: The incidence of FPIES was significantly lower than expected across the whole of the British Isles. Most reports were of cases local to specialist allergy centres, with delays in diagnosis. This suggests under-recognition of FPIES in frontline clinical setting where education of healthcare professionals is required to improve recognition, earlier diagnosis and treatment.

Evaluation of AllergiSense Smartphone Tools for Adrenaline Injection Training.

Anaphylaxis is an increasingly prevalent life-threatening allergic condition that requires people with anaphylaxis and their caregivers to be trained in the avoidance of allergen triggers and in the administration of adrenaline autoinjectors. The prompt and correct administration of autoinjectors in the event of an anaphylactic reaction is a significant challenge in the management of anaphylaxis. Unfortunately, many people do not know how to use autoinjectors and either fail to use them or fail to use them correctly. This is due in part to deficiencies in training and also to the lack of a system encouraging continuous practice with feedback. Assistive smartphone healthcare technologies have demonstrated potential to support the management of chronic conditions such as diabetes and cardiovascular disease, but there have been deficiencies in their evaluation and there has been a lack of application to anaphylaxis. This paper describes AllergiSense, a smartphone app and sensing system for anaphylaxis management, and presents the results of a randomized, controlled, prepost evaluation of AllergiSense injection training and feedback tools with healthy participants. Participants whose training was supplemented with AllergiSense injection feedback achieved significantly better practiced injections with 90.5% performing correct injections compared to only 28.6% in the paper-only control group. In addition, the results provide insights into possible self-efficacy failings in traditional training and the benefits of embedding self-efficacy theory into the technology design process.

Active management of food allergy: an emerging concept.

IgE-mediated food allergies are common and currently there is no cure. Traditionally, management has relied upon patient education, food avoidance and the provision of an emergency medication plan. Despite this, food allergy can significantly impact on quality of life. Therefore, in recent years, evolving research has explored alternative management strategies. A more active approach to management is being adopted, which includes early introduction of potentially allergenic foods, anticipatory testing, active monitoring, desensitisation to food allergens and active risk management. This review will discuss these areas in turn.

What factors affect the carriage of epinephrine auto-injectors by teenagers?

BACKGROUND: Teenagers with allergies are at particular risk of severe and fatal reactions, but epinephrine auto-injectors are not always carried as prescribed. We investigated barriers to carriage. METHODS: Patients aged 12-18 years old under a specialist allergy clinic, who had previously been prescribed an auto-injector were invited to participate. Semi-structured interviews explored the factors that positively or negatively impacted on carriage. RESULTS: Twenty teenagers with food or venom allergies were interviewed. Only two patients had used their auto-injector in the community, although several had been treated for severe reactions in hospital. Most teenagers made complex risk assessments to determine whether to carry the auto-injector. Most but not all decisions were rational and were at least partially informed by knowledge. Factors affecting carriage included location, who else would be present, the attitudes of others and physical features of the auto-injector. Teenagers made frequent risk assessments when deciding whether to carry their auto-injectors, and generally wanted to remain safe. Their decisions were complex, multi-faceted and highly individualised. CONCLUSIONS: Rather than aiming for 100% carriage of auto-injectors, which remains an ambitious ideal, personalised education packages should aim to empower teenagers to make and act upon informed risk assessments.

Cardiovascular effects of methylphenidate, amphetamines and atomoxetine in the treatment of attention-deficit hyperactivity disorder.

Attention-deficit hyperactivity disorder (ADHD) is a very common condition in children and often extends into the adult years. Drugs such as methylphenidate, amphetamines and atomoxetine are frequently prescribed as part of management. The use of these drugs has been increasing and significant clinical benefit is achieved but safety has been questioned. In this review, the cardiovascular safety of these drugs is examined with regard to effects on blood pressure (BP), heart rate (HR), ECG parameters and the risk of sudden death. Methylphenidate appears to cause minor increases in BP and HR. There are no strong data to suggest that methylphenidate increases the corrected QT interval (QTc). Amphetamines appear to cause minor increases in HR and BP over the long term. There is growing evidence to suggest that amphetamines do not cause statistically or clinically significant increases in QTc. Sudden death remains an extremely rare event and there is no clear evidence to attribute this to methylphenidate. Some data even suggest that the risk of sudden death in treated children may be less common than in the background population. Limited data suggest that atomoxetine may increase BP and HR in the short term; in the long term it appears to increase BP. The effects of atomoxetine on QTc remain uncertain. Use of this drug does not appear to be associated with sudden death. Because the current evidence is based on research that has not been specifically designed to investigate the cardiovascular effects of these drugs it is difficult to draw firm conclusions, and further work is required specifically to address these questions.