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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.

Aims: To determine whether the use of negative pressure ventilation (NPV) was associated with a lower rate of endotracheal intubation in infants with recurrent apnoea secondary to acute bronchiolitis. Methods: Retrospective review of two paediatric intensive care units (PICU) databases and case notes; one PICU offered NPV. Results: Fifty two infants with bronchiolitis related apnoea were admitted to the two PICUs (31 to the NPV centre). There were no significant differences between infants in the two centres in age and weight on admission, gestational age at birth, birth weight, history of apnoea of prematurity or chronic lung disease, days ill before referral, respiratory syncytial virus status, oxygen requirement before support, and numbers retrieved from secondary care centres. Respiratory support was provided to all 31 infants in the NPV centre (23 NPV, 8 PPV), and 19/21 in the non-NPV centre (18 PPV, 1 CPAP); the NPV centre had lower rates of endotracheal intubation rates (8/31 v 18/21), shorter durations of stay (median 2 v 7 days), and less use of sedation (16/31 v 18/21). In the two years after the NPV centre discontinued use of NPV, 14/17 (82%) referred cases were intubated, with a median PICU stay of 7.5 days. Conclusions: The use of NPV was associated with a reduced rate of endotracheal intubation, and shorter PICU stay. A prospective randomised controlled trial of the use of NPV in the treatment of bronchiolitis related apnoea is warranted.

Imposed upper airway obstruction was diagnosed as the cause of recurrent and severe cyanotic episodes in 14 patients. Episodes started between 0.8 and 33 months of age (median 1.4) and occurred over a period of 0.8 to 20 months (median 3.5). Diagnosis was made by covert video surveillance, instituted after either (a) the observation that episodes began only in the presence of one person, or (b) characteristic findings on physiological recordings, lasting between 12 hours and three weeks, performed in hospital or at home. Surveillance was undertaken for between 15 minutes and 12 days (median 24 hours) and resulted in safety for the patient and psychiatric assessment of the parent: mother (n = 12), father (n = 1), and grandmother (n = 1). These revealed histories of sexual, physical, or emotional abuse (n = 11), self harm (n = 9), factitious illness (n = 7), eating disorder (n = 10), and previous involvement with a psychiatrist (n = 7). Management of the abusing parents is complex, but recognition of their psychosocial characteristics may allow earlier diagnosis. Imposed upper airway obstruction should be considered and excluded by physiological recordings in any infant or young child with recurrent cyanotic episodes. If physiological recordings fail to substantiate a natural cause for episodes, covert video surveillance may be essential to protect the child from further injury or death.

Background Guidance from the British Thoracic Society, National Patient Safety Agency and British National Formulary advises that oxygen should be treated like other drugs in terms of appropriate prescribing and monitoring. Whilst guidelines exist for the prescription of long-term oxygen [1], there is little available for its short-term use in children, similar to that for adults [2] Aims and Methods Paediatric in-patient units in England were contacted to evaluate their practise regarding the use of oxygen based on the above guidelines. Information was obtained from the paediatric pharmacists and senior nursing staff in 68 units using a telephone questionnaire in 2010 and from paediatric pharmacists in 105 units using survey monkey in 2012. Results Of the units contacted, 51.4% and 42% units respectively completed the survey in 2010 and 2012. The proportion of hospitals that reported they routinely prescribed oxygen increased from 11% to 42% between the two survey points. In 71% of units, prescribing was done on a drug chart. The oxygen was prescribed in percentage terms in 79% of units, and as flow rates in 81%. These were adjusted for the targeted saturations in 73%. Hospitals that have a protocol or guideline for prescribing oxygen have marginally improved from 40 to 43%. Conclusion The results suggest that although there is an increase in the routine use of oxygen prescriptions, the majority of units neither have any guidelines for it nor use oxygen prescriptions. There is a need for the multidisciplinary development of guidelines and educational programmes to aid in the appropriate prescribing and delivery of oxygen in acute units. References NPSA Oxygen safety in hospitals | Rapid Response Report. http://www.nrls.npsa.nhs.uk/resources/?entryid45 = 62811 [Accessed 12th Dec, 2012] Balfour-Lynn IM, Field DJ, Gringras P, et al BTS guidelines for home oxygen in children. Thorax 2009; 64 Suppl 2ii1–26 O’Driscoll B R, Howard L S, Davison A G. BTS guideline for emergency oxygen use in adult patients. Thorax 2008; 63: Supplement VI.

Background Resuscitation Council Guidelines (RC-UK, 2008) for treating anaphylaxis advocate intramuscular adrenaline in doses of 150, 300 or 500 micrograms, according to age bands [1]. However, ALS guidelines recommend weight-based calculations of 10 micrograms/kg IM, leading to administration of a range of volumes [2]. Aims A survey was conducted to evaluate the availability of fixed dose Epipens versus adrenaline vials in paediatric wards and radiology departments in England. Methods The questionnaire was sent to 105 paediatric pharmacists at the various paediatric units in UK. Results 60% responding hospitals had adrenaline available, half of them in vials and 85% with prefilled variable-dose syringes. In 53% units, wards stocked adrenaline 1:10,000 and 64% also had 1:1000. 19% hospitals stocked Epipens on crash trolleys and it was available in 48% wards. Adrenaline was given according to a weight-based dose in 57% wards. For contrast studies, adrenaline was available in 68% departments, 9.5% of which stocked Epipen. Conclusion Most units still use weight-based doses of adrenaline from vials or pre-filled syringes, with Epipens being available in less than half of units. Adrenaline must always be available on wards and in radiology departments, as most arrests from anaphylaxis occur within 10 minutes. To ensure compliance with RC-UK guidelines, either all wards should stock Epipens or the guidelines should reflect practise and recommend weight-based calculated doses. References Working Group of the Resuscitation Council (UK). Emergency treatment of anaphylactic reactions: Guidelines for healthcare providers: Resuscitation Council, 2008. Available at: http://www.resus.org.uk/pages/reaction.pdf (accessed 12 December 2012). Samuels M, Wieteska S. Advanced Paediatric Life Support, 5th edition, Wiley-Blackwell Publishing, Oxford, 2011.

Background and aim of the study Aim of the study was to identify the European centers for diagnosis and treatment of patients with CHS and to collect information on type of ventilatory support currently used for this group of patients. Method The survey was performed in countries participating in the EU-CHS network project. Using a special template following information were collected: total number of already diagnosed patients, number of currently treated patients, type of ventilatory support (invasive ventilation via tracheostomy, mask ventilation, diaphragm pacing, negative ventilation). Results The survey was performed during 2011. Replies were received from 15 centers located in 10 European countries. Number of CHS patients already diagnosed and currently treated varies significantly between centers (from 2 to 112 and from 1 to 70 respectively). Total number of already diagnosed CHS patients were 305 and 199 of them are currently treated. Most frequently used type of ventilator support is ventilation via tracheostomy (52% of patients). Diaphragm pacing alone or diaphragm pacing and ventilation is currently used in 42 patients. Only one CHS patient is currently treated with negative pressure ventilation. Conclusions Results of this study have shown existing significant differences (mainly number of patients) but also some similarities (type of ventilatory support) between centers for CHS diagnosis and treatment located in 10 different European countries. All of the collected data should be used for further work on the European CHS network of reference centers. The study was supported by the EU-CHS Network (grant number 2008 12 06).

Editor,—Intentional suffocation is a difficult diagnosis. Children often appear well in between such episodes and cannot tell their paediatrician that they are being intermittently assaulted. The evidence for intentional suffocation is often circumstantial, which means that doctors may have difficulty substantiating the diagnosis in court without evidence from covert video surveillance. We are surprised, therefore, that Davis et al state that a high proportion of suffocation victims were protected effectively without covert video surveillance.1 It may be that these cases were not contested or had evidence of other harm to the child in addition to the suffocation. Davis et al reinforced the British Paediatric Association working party guidelines that “when there is a very high risk, children should not be exposed to danger simply to achieve a criminal standard of proof.”2 Covert surveillance has been used in our knowledge primarily to confirm the diagnosis and to ensure effective protection for the child and siblings. Even in civil proceedings, which rely on a balance of probabilities, the more serious the allegations of abuse, the higher the level of evidence the court requires. In a number of cases of suspected suffocation, we have seen children inadequately protected …