Ataxia telangiectasia (A-T) is a rare, progressive, multisystem disease that has a large number of complex and diverse manifestations which vary with age. Patients with A-T die prematurely with the leading causes of death being respiratory diseases and cancer. Respiratory manifestations include immune dysfunction leading to recurrent upper and lower respiratory infections; aspiration resulting from dysfunctional swallowing due to neurodegenerative deficits; inefficient cough; and interstitial lung disease/pulmonary fibrosis. Malnutrition is a significant comorbidity. The increased radiosensitivity and increased risk of cancer should be borne in mind when requesting radiological investigations. Aggressive proactive monitoring and treatment of these various aspects of lung disease under multidisciplinary expertise in the experience of national multidisciplinary clinics internationally forms the basis of this statement on the management of lung disease in A-T. Neurological management is outwith the scope of this document.

A note on versions:The version presented here may differ from the published version or from the version of record. If you wish to cite this item you are advised to consult the publisher's version. Please see the repository url above for details on accessing the published version and note that access may require a subscription.For more information, please contact eprints@nottingham.ac.uk Financial disclosure: The other authors have no financial relationships relevant to this article to disclose. Outcomes of nosocomial viral respiratory infections in high-risk neonates Conflicts of interest:The other authors have no potential conflicts of interest to disclose. Abbreviations:VRTIs -viral respiratory tract infections BPD -bronchopulmonary dysplasia NICU -neonatal intensive care unit NEC -necrotising enterocolitis CPAP -continuous positive airway pressure Bi-PAP -bi-level positive airway pressure HFOV -high frequency ventilation ECMO -extracorporeal membrane oxygenation RT-PCR -real time polymerase chain reaction RVP -respiratory viral panel NHS -National Health Service What's Known on This SubjectViral respiratory tract infections cause severe respiratory morbidity in ex-preterm infants after NICU discharge. They are now recognized to be more prevalent in the NICU but their longterm impact, prior to discharge, during this early period of life is unclear. What This Study AddsThis study identifies the adverse impact viral respiratory infections, particularly rhinovirus, have on newborn infants during their initial NICU admission. Identification of the associated significant respiratory morbidity and healthcare costs should focus efforts on reducing these nosocomially acquired infections. Contributors' Statements:Dr Don Sharkey conceptualized and designed the study, drafted the initial manuscript, and approved the final manuscript as submitted.Drs Shairbanu Zinna, Arthi Lakshmanan, Shin Tan, Shiu Soo, Lisa Szatkowski and Miss Rebecca McClaughry and Mr Martin Clarkson carried out the data collection, initial analyses, reviewed and revised the manuscript, and approved the final manuscript as submitted.All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work. ABSTRACTBackground Neonatal respiratory disease, particularly bronchopulmonary dysplasia, remains one of the leading causes of morbidity and mortality in newborn infants. There is increasing evidence that nosocomially acquired viral respiratory tract infections (VRTIs) are not uncommon in the neonatal intensive care unit but there are few studies on their impact on neonatal respiratory outcomes and the associated healthcare costs. This study aimed to assess the association between nosocomial VRTI and neonatal respiratory disease.

Neurological and immunological contributions to lung disease in A-T require proactive and multidisciplinary management http://ow.ly/TqT44 Why indeed should we care? The answer is that ataxia telangiectasia (A-T) patients will present to respiratory paediatricians for diagnosis, when standard testing, if the diagnosis is not made, may do irreparable harm; and both adult and paediatric respiratory physicians will be involved in managing the respiratory disease, which is a major cause of morbidity and mortality. So there is a real “need to know” which is why the ERS convened a task force on A-T, whose findings are published in the European Respiratory Review [1]. A-T is an autosomal recessive, progressive, multisystem disease caused by mutations in the gene ATM (Ataxia-Telangiectasia Mutated) (11q22.3). This gene is expressed ubiquitously and encodes ATM kinase, a serine/threonine protein kinase, which is involved in signalling following cellular stress. It activates over a hundred proteins involved in the DNA damage response, cell cycle regulation and other pathways. ATM has important roles in neuroprotection, both adaptive and innate immunity, inflammatory responses, metabolism (e.g. insulin signalling), longevity and fertility. It has been estimated that a human cell is confronted with one million DNA lesions every day, placing DNA damage response mechanisms in a position of paramount importance. This has to be a very precise and efficient system to prevent cells with damaged DNA from dividing further or being passed on through germline mutation. The DNA double strand break (DSB) represents one of the most cytotoxic DNA lesions. DSBs can be generated by exposure to ionising radiation or various chemical compounds [2]. The Orphanet registry estimates the average prevalence of A-T to be 1 per 100 000 children [3]. A-T has a large number of other complex and diverse manifestations that vary with age. Neurological symptoms, particularly progressive cerebellar ataxia and abnormalities of the eye movements appear from an early age, and malignant disease is common. Some children first come to medical attention because of recurrent sino-pulmonary infections (which affect ∼50% of children with A-T). Oculocutaneous telangiectasia may not occur until 4 or 5 years of age, so this clue is not available in early life. Laboratory tests may show an elevated alpha-fetoprotein level, immunological deficiencies and chromosomal instability; one lesson is to have a low threshold for the measurement of alpha-fetoprotein as a screening test, especially in young children with infections plus another sign, especially drooling or ataxia. Patients with A-T die prematurely with the leading causes of death being respiratory diseases and cancer, hence the importance of receiving respiratory management right from the start. Also, it is good to consider the diagnosis before rather than after embarking on radiological investigations such as high-resolution computed tomography (HRCT), given the sensitivity of the patients to radiation. There is n...

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outcome/survival is not significantly different between the two groups. Using a SAD may not be worth doing as it takes time to insert, meaning there is no ventilation in that time. However, in children with difficult airways who intubation poses a problem, it is worth bearing in mind the use of a SAD. Over time the effectiveness of BVM decreases, hence a more definitive airway should always be planned. Clinical bottom line A bag valve mask with oropharyngeal airway should be used initially to oxygenate and ventilate a child in cardiopulmonary arrest. A supraglottic airway should be considered in children with a difficult airway or if there is going to be delay in establishing a definitive airway (endotracheal intubation). SADsupraglottic airway device. LMAlaryngeal mask airway. OPAoropharyngeal airway. BVMbag-valve-mask. ALSadvanced life support.