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Even after the First World War, the British and German armies remained strongly influenced by Clausewitz, for whom personality rather than mass was the best means to reduce friction. This article explores how this was reflected in their military doctrine between the two world wars. The German regulations showed a clear alignment with Clausewitz's thinking. The British tended to focus on the characteristics Clausewitz had argued were necessary for the troops, rather than for their commanders. The campaigns of 1939/40 caused the Germans to place even greater emphasis on boldness, while the British focused on steadiness and caution.

During the second half of the nineteenth century, the introduction of new weaponry dramatically changed the balance between moral factors and technology on the battlefield. Yet, this shift was widely met by a renewed emphasis on the importance of the human element. This article explores the development of thinking on this issue in the British Army during the period from 1856 to 1899. This reveals three phases, representing the struggle between the conservative Duke of Cambridge and the modernizing Lord Wolseley, with their view explored through the writings of key theorists and in the official manuals. This reveals that the Duke remained focused on a mechanical model, centred on the teachings of Jomini, where the troops were simply tools in the hands of their commanders, whereas Wolseley emphasized the need to protect the ‘moral strength’ of the troops and saw undermining that of the enemy as the key to victory, yet always recognizing that bravery and resilience could never overcome modern weaponry.

Social, political and industrial upheavals swept across Europe after November 1918. These affected the victors too, with some social groups seeking to consolidate gains made during the war, while others attempted to restore pre-war norms. In Italy, industrial unrest in Vincenza added a distinctive gendered character. An overlooked factor is the role of British troops, who became involved with local women factory workers, and hence drawn into direct conflict with the Italian authorities as these attempted to suppress strikes. This confrontation, part of the wider falling out between the wartime allies, was only resolved through the hasty removal of British forces.

Guidelines for air passengers with respiratory disease focus on primary lung pathology. Little evidence exists to guide professionals advising children needing ventilatory support because of neuromuscular or central hypoventilation conditions; these children might risk hypoxia and hypercapnia if unable to mount an adequate hyperventilation response.This study assessed the response to low ambient oxygen using a modified hypoxic challenge test. In addition to measuring pulse oximetry and response to supplementary oxygen, we also measured transcutaneous carbon dioxide and response to ventilatory support.Twenty children on nocturnal ventilatory support aged 1.6-18 years were recruited in a pragmatic sample from outpatient clinics; 10 with neuromuscular weakness and 10 with central hypoventilation. Participants underwent a two-stage, modified hypoxic challenge test; a conventional stage, where oxygen alone was titrated according to SpOThirteen participants needed supplemental oxygen during the conventional stage, but only two did when using ventilatory support. Transcutaneous carbon dioxide remained within normal range for all participants, on or off ventilatory support. Whilst some participants found testing challenging, participants generally reported both testing and air travel to be valuable.Evaluating response to patients' usual ventilation through "fitness-to-fly" assessment aids decision making when considering whether children who receive nocturnal ventilation can travel by air, since for some using a ventilator reduces or avoids the need for supplemental oxygen.

Given the backdrop of multiple concurring crises—a global pandemic, political instability and violence, and multiple structural inequalities—we see the problem of now as this: How do educational developers continue to address the wicked problems in teaching and learning when we are simply so exhausted? Our article presents the importance of communities of practice for educational developers, inviting us to witness and name the communities in which we belong; the important functions they engage; who they nurture and how; and what care is undertaken to sustain these groups and ourselves. To help educational developers understand and appreciate the ways that communities of practice support our work (emotionally, professionally, and socially), we share a framework from the literature of organizational management and apply it to communities in educational development. We include narratives to demonstrate this framework in action to amplify the particularly important role these groups have played in our professional and personal lives. We end with actions we can take to care for our communities of practice that build upon the presented theoretical foundation. As these groups are fragile, maintaining our communities is important so that they will provide us support and shelter into the post-pandemic future.

Patients with syndromic craniosynostosis have a molecularly identified genetic cause for the premature closure of their cranial sutures and associated facial and extra-cranial features. Their clinical complexity demands comprehensive management by an extensive multidisciplinary team. This review aims to marry genotypic and phenotypic knowledge with clinical presentation and management of the craniofacial syndromes presenting most frequently to the craniofacial unit at Great Ormond Street Hospital for Children NHS Foundation Trust.

We report a patient with profound congenital hypotonia, central hypoventilation, poor visual behaviour with retinal hypopigmentation, and significantly decreased mitochondrial respiratory chain complex I activity in muscle, who died at 7 months of age having made minimal developmental progress. Biallelic predicted truncating P4HTM variants were identified following trio whole-genome sequencing, consistent with a diagnosis of hypotonia, hypoventilation, intellectual disability, dysautonomia, epilepsy and eye abnormalities (HIDEA) syndrome. Very few patients with HIDEA syndrome have been reported previously and mitochondrial abnormalities were observed in three of four previous cases who had a muscle biopsy, suggesting the possibility that HIDEA syndrome represents a primary mitochondrial disorder. P4HTM encodes a transmembrane prolyl 4-hydroxylase with putative targets including hypoxia inducible factors, RNA polymerase II and activating transcription factor 4, which has been implicated in the integrated stress response observed in cell and animal models of mitochondrial disease, and may explain the mitochondrial dysfunction observed in HIDEA syndrome.

As part of a feasibility study investigating a new protocol for hypoxic challenge testing (HCT) in children with neuromuscular weakness or central hypoventilation who use ventilation at home, we conducted interviews evaluating parents’ and children’s experiences of flying and this pre-flight testing. The new protocol may better advise patients who use ventilators about their ‘fitness to fly’. We have recruited 15 children, 7 with neuromuscular weakness, and 8 with central hypoventilation to date (mean age 8.5 years, range 1.7 to 16.7 years), all of whom have sat in an airtight chamber with an oxygen concentration 15% (normal 21%) to simulate the in-flight environment. Patients had monitoring of oxygen saturation, end tidal and transcutaneous carbon dioxide in air, 15% oxygen, and then with supplemental oxygen, ventilation and if needed, both. Semi-structured interviews immediately after the HCT and by telephone 3–4 months later identified the following factors from the preliminary analysis of interview transcripts. Parents identified the challenges they faced when planning and undertaking air-travel, including repeated negotiations with airlines, on provision of oxygen, accommodating essential equipment thereby requiring families to be proactive managers. Despite these challenges several families were frequent travellers, who valued opportunities to visit extended family and enjoy new experiences as a whole family. During interviews older children identified travel as an opportunity for normalisation of their lives. Some families remained cautious about air travel expressing concerns about their child’s underlying vulnerability. Parents and children provided valuable feedback on the experience of the HCT protocol, including challenges of space for wheelchairs and equipment in the chamber, the importance of considering school attendance in the context of multiple hospital appointments. Overall the testing process and results were seen positively by many, it built confidence for parents by providing more information about their child’s responses to flight to aid future decision making.

Background Congenital Central Hypoventilation Syndrome (CCHS) is a rare condition characterized by an alveolar hypoventilation due to a deficient autonomic central control of ventilation and a global autonomic dysfunction. Paired-like homeobox 2B (PHOX2B) mutations are found in most of the patients with CCHS. In recent years, the condition has evolved from a life-threatening neonatal onset disorder to include broader and milder clinical presentations, affecting children, adults and families. Genes other than PHOX2B have been found responsible for CCHS in rare cases and there are as yet other unknown genes that may account for the disease. At present, management relies on lifelong ventilatory support and close follow up of dysautonomic progression. Body This paper provides a state-of-the-art comprehensive description of CCHS and of the components of diagnostic evaluation and multi-disciplinary management, as well as considerations for future research. Conclusion Awareness and knowledge of the diagnosis and management of this rare disease should be brought to a large health community including adult physicians and health carers.

The changing landscape in management of neuromuscular disorders (NMDs) is attributed to the use of antisense oligonucleotides, gene therapy, and other novel treatments. Children with spinal muscular atrophy (SMA) type 1 who were dying before the age of 2 years are living longer. Standard of care for specific diseases based on expert opinion has helped to improve the supportive management which has shown enhanced survival and good quality of life. Respiratory management is one of the most important aspects of care for these patients. Principles of respiratory care are to maintain a clear airway free from secretions and support effective cough and timely treatment of hypoventilation with assisted ventilation. The challenges faced by clinicians include managing recurrent chest infection, excess secretions, and aspiration-related multiple hospital admissions. There is paucity of randomised trials, and management is mainly based on expert opinion and clinical experience. Service planning and provision needs to take in context changing natural history of these conditions.

Background: Congenital Central Hypoventilation Syndrome (CCHS) is a rare condition characterized by an alveolar hypoventilation due to a deficient autonomic central control of ventilation and a global autonomic dysfunction. Paired-like homeobox 2B (PHOX2B) mutations are found in most of the patients with CCHS. In recent years, the condition has evolved from a life-threatening neonatal onset disorder to include broader and milder clinical presentations, affecting children, adults and families. Genes other than PHOX2B have been found responsible for CCHS in rare cases and there are as yet other unknown genes that may account for the disease. At present, management relies on lifelong ventilatory support and close follow up of dysautonomic progression. Body: This paper provides a state-of-the-art comprehensive description of CCHS and of the components of diagnostic evaluation and multi-disciplinary management, as well as considerations for future research. Conclusion: Awareness and knowledge of the diagnosis and management of this rare disease should be brought to a large health community including adult physicians and health carers.

Introduction Beckwith-Wiedemann Syndrome (BWS) is a congenital condition where patients have macroglossia, often requiring tongue reduction surgery as well as being a potential risk factor for obstructive sleep apnoea (OSA). Prevalence of OSA is high and therefore sleep studies (SS) pre- and post-surgery are useful to assess the impact of surgery. Method From 58 patients with BWS who underwent SS before or after tongue reduction surgery over a 20-year period, there were 9 patients who had tongue reduction surgery plus a SS pre- and post-operatively with sufficient data to calculate an Apnoea Hypopnoea Index (AHI). Results Figure 1 shows the change in Obstructive AHI (OAHI) before and after tongue reduction surgery in the 9 patients. Discussion In our cohort with pre- and post-surgery SS data the prevalence of OSA was 44% (mild 100%). 4 patients showed reductions in OAHI after surgery (mean change: 1evs/hr), 3 others showed a worsening (mean change: 5.5evs/hr), while 2 showed no change in OAHI. Of the 3 that worsened, 1 was restudied after two years and showed no evidence of OSA with no medical intervention. Another had grade 2 tonsils which have been surgically removed. The third patient is waiting for ENT surgical review. The small sample size of BWS patients with sufficient SS to qualify for this study may be related to the referral pathway with the majority of patients diagnosed with moderate to severe OSA pre- surgery were either treated (e.g. tracheostomy) or surgery delayed due to OSA. In conclusion, tongue reduction surgery does not appear to affect the level of OSA and patients who had worsening of OSA, had other factors in their history predominantly ENT related. An alternative method of assessment, such as a questionnaire, focussed on sleep disordered breathing, might be a better determinant of the impact of surgery.

Introduction Beckwith-Wiedemann Syndrome (BWS) is a congenital condition where patients have macroglossia, often requiring tongue reduction surgery as well as being a potential risk factor for obstructive sleep apnoea (OSA). Prevalence of OSA is high and therefore sleep studies (SS) pre- and post-surgery are useful to assess the impact of surgery. Method From 58 patients with BWS who underwent SS before or after tongue reduction surgery over a 20-year period, there were 9 patients who had tongue reduction surgery plus a SS pre- and post-operatively with sufficient data to calculate an Apnoea Hypopnoea Index (AHI). Results Figure 1 shows the change in Obstructive AHI (OAHI) before and after tongue reduction surgery in the 9 patients. Discussion In our cohort with pre- and post-surgery SS data the prevalence of OSA was 44% (mild: 100%). 4 patients showed reductions in OAHI after surgery (mean change: 1evs/hr), 3 others showed a worsening (mean change: 5.5evs/hr), while 2 showed no change in OAHI. Of the 3 that worsened, 1 was restudied after two years and showed no evidence of OSA with no medical intervention. Another had grade 2 tonsils which have been surgically removed. The third patient is waiting for ENT surgical review. The small sample size of BWS patients with sufficient SS to qualify for this study may be related to the referral pathway with the majority of patients diagnosed with moderate to severe OSA pre- surgery were either treated (e.g. tracheostomy) or surgery delayed due to OSA. In conclusion, tongue reduction surgery does not appear to affect the level of OSA and patients who had worsening of OSA, had other factors in their history predominantly ENT related. An alternative method of assessment, such as a questionnaire, focussed on sleep disordered breathing, might be a better determinant of the impact of surgery.

ObjectiveTo test the hypothesis that children with Prader-Willi syndrome (PWS) and obstructive sleep apnoea syndrome (OSAS) have hypercapnia for higher proportion of total sleep time (TST) than non-syndromic children with similar obstructive apnoea–hypopnoea index (OAHI).DesignCross-sectional study.SettingTwo tertiary care hospitals.PatientsPatients with PWS and non-syndromic children with snoring who underwent polygraphy and were of similar age, body mass index (BMI) z-score and OAHI.Main outcome measureThe two groups were compared regarding %TST with transcutaneous CO2 (PtcCO2) >50 mm Hg. The interaction between PWS diagnosis and OSAS severity (OAHI 5 episodes/h) regarding %TST with PtcCO2 >50 mm Hg was tested using multiple linear regression.Results48 children with PWS and 92 controls were included (median age 2.3 (range 0.2–14.1) years vs 2.2 (0.3–15.1) years; BMI z-score 0.7±1.9 vs 0.8±1.7; median OAHI 0.5 (0–29.5) episodes/h vs 0.5 (0–33.9) episodes/h; p>0.05). The two groups did not differ in %TST with PtcCO2 >50 mm Hg (median 0% (0–100%) vs 0% (0–81.3%), respectively; p>0.05). However, the interaction between PWS and OSAS severity with respect to duration of hypoventilation was significant (p2 >50 mm Hg between children with PWS and controls for OAHI 5 episodes/h were +0.2%, +1% and +33%, respectively.ConclusionIncreasing severity of upper airway obstruction during sleep in children with PWS is accompanied by disproportionately longer periods of hypoventilation when compared with non-syndromic children with similar frequency of obstructive events.

Rommel’s approach to command and his relationship with the German army’s doctrine remain subjects of lively debate. Analysis of his career prior to 1940 shows he was a highly respected figure at the centre of the army’s officer training system. Consideration of his approach during the crossing of the Meuse in May 1940 demonstrates consistency with his actions at Caporetto in 1917. Far from proving his unorthodoxy, however, comparison of this approach with the army’s official manuals suggests Rommel acted fully in accordance with doctrine. This highlights a tension within that doctrine between independence of action and wilfulness.

BackgroundNusinersen has been used to treat spinal muscular atrophy type 1 (SMA1) in the UK since 2017. While initial trials showed neuromuscular benefit from treating SMA1, there is little information on the respiratory effects of nusinersen. We aimed to look at the respiratory care, hospital utilisation and associated costs in newly treated SMA1.MethodsWe reviewed the medical records of all children within the West Midlands with SMA1 treated with nusinersen at Royal Stoke University Hospital. Baseline demographics and hospital admission data were collected including: the reason for admission, total hospital days, days of critical care, days intubated, discharge diagnosis, doses of nusinersen and treatment complications.Results11 children (six girls) received nusinersen between May 2017 and April 2019. Their median (range) age was 29 (7–97) months. The median (range) number of nusinersen doses per child was 6 (4–8). All children were receiving long-term ventilatory support; this was mask ventilation in nine and tracheostomy ventilation in two. The total number of hospital days since diagnosis was 1101 with a median (range) of 118 (7–235) days per child. This included general paediatric ward days 0 (0–63), High Dependency Unit 79 (7–173) days and Paediatric Intensive Care Unit 13 (0–109) days per child. This equated to a median (range) of 20 (2–72) % of their life in hospital. The estimated cost of this care was £2.2M.ConclusionPatients with SMA1 treated with nusinersen initially spend a considerable proportion of their early life in hospital. Parents should be counselled accordingly. These data suggest that for every 10 children started on nusinersen an extra HDU bed is required. This has a significant cost implication.

Aims To survey trends in the number of sleep studies (SS) performed at paediatric sleep centres in the UK and Republic of Ireland (ROI) and to identify the reasons for performing the investigation. Methods A cross-sectional survey design was used with a newly developed questionnaire. Sleep centres were recruited via the British Paediatric Sleep Association (BPSA) and centre leads were sent a link to the online questionnaire. This was completed between May and June 2017. Results Of the 25 centres identified, 20 (80%) completed the online questionnaire. Centres represented England (n=16,80%), Scotland (n=2,10%) and ROI (n=2,10%). Fifteen (75%) centres provided data on the number of SS performed in their centre from 2007 to 2016 and 13 (65%) centres provided the reasons for referral. The linear mixed model test demonstrates that there was a significant main effect of time [F(9,49.7)=4.537, p≤0.001] and over this 10 year time span, there was a 6.5-fold rise in sleep studies. The main reason for referral was for the diagnosis of OSA (obstructive sleep apnoea) in otherwise healthy children (median=40%) with diagnosis of OSA in children with underlying medical problems (median=20%), assessment of children on mechanical ventilation (median=12%) and assessment of sleep-related hypoventilation in neuromuscular disorders (median=10%) contributing to paediatric sleep centre caseload. Conclusion The UK and ROI’s sleep study activity has been reported previously as significantly lower than in other countries (Flemons et al., 2004), but this is increasing as shown in this study. It is highly likely that the referral rates and numbers of studies performed in paediatric sleep centres will continue to increase. As expected the main reason for referral was for the diagnosis of OSA in otherwise healthy children and this referral group has contributed to the rise in the past 5 years. It is imperative to assess current diagnostic practices including triaging and ensuring guidelines/policies are relevant and updated. This will ensure adequate and timely diagnosis and follow-up and ensure resources are used efficiently.

Introduction Raised ICP is a recognised complication of craniosynostosis in children and may reduce cerebral blood flow and consciousness, as well as causing papilledema and other neurological symptoms. Obstructive sleep apnoea (OSA) has a reported prevalence of between 40% and 85% in children with syndromic craniosynostosis (SC) and it is known that SDB can further raise ICP. Since 2015 we have routinely carried out simultaneous sleep studies (SS) for children undergoing ICP monitoring and we wanted to examine further the relationship between ICP and SDB in this group. Methods We reviewed a new clinical service combining ICP measurement with respiratory polygraphy (RP). Patients with relevant symptoms were selected by the neurosurgery team and underwent placement of subdural ICP bolt under general anaesthetic followed by placement of respiratory polygraphy (RP) sensors in the late afternoon on the 1 st or 2nd night of a 48 hour ICP recording. Results A total of 27 patients (19 M: 8 F) were identified, with a mean age of 5.7 years (range 1.3 years to 18.2 years). The indications for ICP insertion and the clinical syndrome are shown in table 1(a) and 1(b) respectively. All studies were successful with no signal loss. The sleep study (SS) and ICP outcome, as well as the neurosurgical outcome, can be seen in table 1(c). Discussion Some studies showed important relationships between ICP and SDB, although the severity of OSA did not predict those who had high ICP or required surgery. Importantly, ICP in these patients may be significantly affected by sleep stage and in operated patients, by transient physiological rises in CO2.

The assault at Amiens was ‘the black day of the German Army’. British accounts treat the Germans as passive, of no more importance than the terrain, and victory as inevitable. Using German sources, and drawing on statistical ‘historical analysis’ of behaviour in battle, the article argues the frontline defenders panicked when thick fog left them defenceless against the British tanks. By contrast, when the fog lifted, the defence recovered. Although senior German commanders on the spot reacted well, Ludendorff fell into a state of shock, such that he convinced the Kaiser the war must be ended.

Drawing on theories of problems in warfare being ‘tame’ or ‘wicked’, this article explores continuity and changes in British and German doctrine through examination of wording, emphasis, and approach in field service manuals. This reveals significant continuities in German doctrine, especially the emphasis on initiative, but growing focus on rapid decision-making, coupled with forward command, to achieve surprise. British doctrine also displayed continuity, focused on controlling the battle and reluctance to allow subordinates to exercise initiative. A shift in British doctrine, from one similar to the German model towards a more restrictive approach, is identified between 1905 and 1909.

We read with interest the article by Leong et al on the use of polysomnography (PSG) in children,1 covering indications for PSG, along with limitations of oximetry, and clearly outlining how to undertake and interpret PSG in paediatric patients. It briefly discusses limited channel recordings (respiratory polygraphy, RP) and concludes that this ‘is not standard practice’. In many paediatric centres RP is standard practice, and routinely used for assessment of sleep-disordered breathing (SDB) in children, with the most common diagnosis being obstructive sleep apnoea (OSA). In a recent survey …

Background: Increased numbers of referrals for cardiorespiratory sleep studies are being received for Ear, Nose and Throat (ENT) patients (without accompanying co-morbidities); these studies help diagnose Obstructive Sleep Apnoea (OSA). Increased patient numbers have made quick diagnosis difficult. The aim was to assess if Oxygen Desaturation Index (ODI) could be used as a surrogate for Apnoea-Hypopnoea Index (AHI) to diagnose OSA, in patients with no comorbidities. Methods: The laboratory database (Microsoft Access) was used to retrieve sleep study data (recorded using Embla S4500 and analysed by physiologists) for ENT patients from the two most frequent general hospital referrers over the past 8 months. Data retrieved included: department sleep questionnaire, AHI, ODI and outcome. Both ODI and AHI were ranked for severity using AASM (Berry R.B. et al, American Academy of Sleep Medicine, 2017) criteria: Normal 10; the patients were grouped accordingly. Results: Data was collected from 154 patients (104 male, 50 female) aged from 10 months to 15 years (Median 4.29yrs). Spearman9s Rho test showed positive correlation between AHI & ODI [rs=0.862 (p= Conclusions: This data indicates that should the AASM criteria for scoring OSA severity by AHI, be applied to ODI, this could be a good indicator for the correct AHI, particularly in the severe category of patients (99.3%). There may be other reasons why at milder levels, the sensitivity is poorer, such as patient age, or inter-scorer variation affecting the AHI.

Introduction: Our attended paediatric cardiorespiratory sleep study measurements include respiratory bands (thorax/abdomen), SpO2 and pulse rate (pulse oximeter), transcutaneous CO2 (TcCO2), position and nasal flow (cannula). Aims: To review our system for assessing sleep study signal integrity. Methods: Studies were analysed (Embla REMlogic) to AASM paediatric guidelines. Minimal channels required for diagnosis were specified as respiratory bands, SpO2 and TcCO2. Respiratory bands were graded 1-5 for the time artefact-free signals were present (5 being present for entire study), with a pass considered >3. The time with acceptable and artefact-free SpO2 and TcCO2 signal was calculated as a percentage of total sleep time (TST), with >50% considered a pass. Failed studies were reviewed for staff feedback. A secondary audit looked at the time with acceptable nasal flow from nasal cannula as a percentage of TST, although this was not considered part of the signal audit pass/fail criteria. Results: 2787 cardiorespiratory sleep studies were assessed (n=2149 – nasal cannula present) and 98.3% passed. Nasal flow recording was less successful (present for 65.8%TST) than other sensors. A reduced time of TcCO2 present was the primary reason for failure in the 1.7% of failed studies. Conclusions: A sleep sensor scoring system was developed to identify if signal quality was sufficient for analysis. Most studies passed overall, with nasal flow recording most likely to fail. The system is now incorporated into our daily practice.

Immunoglobulin-helicase-μ-binding protein 2 (IGHMBP2) mutations are associated with partial continuum between two extremes of rapidly lethal disorder of spinal muscular atrophy with respiratory distress type 1 (SMARD1), with infantile axonal neuropathy, diaphragmatic weakness and commonly death before 1 year of age, and Charcot-Marie-Tooth disease (CMT) type 2S with slowly progressive weakness and sensory loss but no significant respiratory compromise. We present an atypical case of CMT2S. A 9 month old boy presented with bilateral feet deformities and axonal neuropathy. Genetic testing revealed two heterozygous variants in the IGHMBP2 gene: c.1156 T > C p.(Trp386Arg) in exon 8 and c.2747G > A p.(Cys916Tyr) in exon 14, that were inherited from his father and mother respectively. At 9 years, he developed diaphragmatic weakness, following which he was established on non-invasive ventilation. Our case emphasizes the importance of life long respiratory surveillance for patients with CMT2S and expands the phenotype of this condition.

The Pediatric Sleep Questionnaire described by Chervin et al. (Sleep Medicine, 2000, 1, 21-32) was originally validated for children with obstructive sleep apnoea syndrome but without other disorders. The aim of our study was to check the applicability of this questionnaire in children with underlying chronic medical conditions. Children aged 2-18 years who underwent a diagnostic sleep study at Great Ormond Street Hospital were recruited over a 10-month period. The Pediatric Sleep Questionnaire completed by their parents and cardiorespiratory polygraphy were scored. Sensitivities and specificities of the Pediatric Sleep Questionnaire were calculated using a Pediatric Sleep Questionnaire score of 0.33 as being indicative of sleep-disordered breathing. A total of 561 patients were reviewed. Neuromuscular disorders (n = 108), craniofacial anomalies (n = 58) and the obstructive sleep apnea syndrome control group (n = 155) were best represented. The sensitivity for patients with isolated obstructive sleep apnoea syndrome was 76.5% when using an apnoea-hypopnoea index ≥ 5, but this was much lower when looking at specific sub-groups such as neuromuscular patients (25%) or patients with Trisomy 21 (36.7%). Sensitivities remained unchanged for patients with obstructive sleep apnoea syndrome (77.3%) when an apnoea-hypopnoea index of ≥ 1 was used, but improved for neuromuscular disorders sub-groups (36.7%) and Trisomy 21 (84%). In conclusion, the Pediatric Sleep Questionnaire is not a good screening tool for obstructive sleep apnoea syndrome in children with complex underlying disorders when a cut-off apnoea-hypopnoea index of ≥ 5 is used, and it cannot replace cardiorespiratory polygraphy recording.

Background Some cases of sudden unexpected death in infants and children (SUDIC) occur from fulminant sepsis. There is no agreed definition for accepting this mechanism of death within SUDICs. Aims To characterise the history, Emergency Department (ED) and post-mortem (PM) findings in children who died unexpectedly and in whom the likely diagnosis was sepsis and to make a working definition for Sudden Unexpected Death (SUDIC) due to Sepsis. Methods We reviewed all SUDIC cases between 2008 and 2016 listed on the database for the local Child Death Overview Panel, and identified those thought to be due to natural causes. We then collected clinical details alongside the ED and PM findings to characterise which deaths were likely to be due to severe and overwhelming infection. Our early management of SUDIC includes the collection of samples as soon as possible after death for investigation of infection. Results Over 8 years, there were 160 cases designated as SUDIC due to natural causes, of which 17 (11%) were from infection-related causes. We examined complete records for 12 children (7 girls), aged 4 months to 12 years (median 26 months). Three (25%) had preceding signs suggestive of infection, but insufficient to diagnose sepsis. Five of the other 9 children died from beta haemolytic group A streptococcus sepsis, one from beta haemolytic group B streptococcus sepsis, one from staphylococcus aureus sepsis, and one from Haemophilus influenza sepsis. In the remaining child, the cause of death was unascertained but ‘likely sepsis secondary to streptococcal infection’. Eight children were unwell for 48 hours, with a median duration of symptoms of 24 hours. Six had medical review before death. Symptoms included: fever, cough, coryza, unsettled, lethargy, vomiting and diarrhoea and all but one were managed as viral illnesses. Conclusion Lethal sepsis can progress rapidly, but be difficult to distinguish from viral infection at onset. A working definition for SUDIC-sepsis includes: sudden death with i) prodromal symptoms; ii) a likely pathogen in a normally sterile site; and, iii) evidence of inflammatory reaction (such as raised CRP, WCC and histological changes).