1527 e-Letters

  • The risk of ibuprofen-related acute kidney injury is just as important

    The question of the safety of ibuprofen in lower respiratory tract infection(LRTI)(1) should be part of a bigger question. That question is the issue of the safety of ibuprofen in a child who is at risk of dehydration. A febrile child with LRTI is at risk of dehydration because of increased insensible fluid loss via the skin. Furthermore, in the presence of LRTI-related tachypnoea, there will be increased insensible fluid loss via the upper respiratory tract . These fluid losses are compounded when the child is too ill to maintain a good oral fluid intake.
    In volume depleted states, such as the scenario depicted above, vasodilatory prostaglandins maintain adequate renal blood flow(RBF) and adequate glomerular filtration rate(GFR)(2). Nonsteroidal anti inflammatory drugs(NSAIDs) undermine those compensatory mechanisms by inhibiting prostaglandin synthesis(2). The consequence is the onset of NSAID-related acute kidney injury(AKI), as postulated by Misurac et al(3). These investigators postulated that NSAID-related inhibition of prostaglandin synthesis was the underlying cause of AKI in 21 of their 27 cases of NSAID-related AKI. In the remaining 6 children with AKI, acute interstitial nephritis(also attributable to NSAIDs) was the underlying cause.. Fifteen of the 20 children for whom dosing data were available took NSAID doses in the recommended range. Ibuprofen was the culprit NSAID in 67% of cases. Misurac et al also identified 54 other cases...

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  • Response letter to Nutritional rickets under 16 years: UK surveillance results

    Dear Editor

    Infant vitamin D supplementation prevents not just rickets but also hypocalcaemic seizures and cardiomyopathy (CMP). The BPSU survey (1) captures rickets incidence which, we feel compelled to highlight, represents only the tip of the iceberg of widespread vitamin D deficiency (VDD) in the population.

    In the UK, child surveillance checks are led by general practitioners (GPs). Most GPs do not receive postgraduate paediatric training and have inadequate undergraduate paediatric exposure, as acknowledged by the RCPCH president: “by any stretch of the imagination, GP training in the UK in paediatrics is woefully inadequate” (2). Recognising rickets requires paediatric experience as exemplified by recent cases of VDD induced CMP- one child’s death was preceded by multiple unfruitful visits to GPs and casualty (3). As the BPSU survey reached out only to paediatricians and not GPs, the extent of underreporting and under diagnosis is likely huge, limiting comparison with countries where paediatricians oversee primary care. The conclusion that rickets incidence in the UK is lower than expected downplays the extent of the underlying public health crisis, particularly when a significant number of cases were excluded [table 2 of (1)]. The true disease burden is unravelled when family members of affected children are investigated (3).

    Similar to previous studies, rickets incidence here is 90 to 166 fold higher in Asian and Black children compared to wh...

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  • Letter to Editor Nebulised hypertonic saline in moderate-to-severe bronchiolitis: a randomised clinical trial. Raphaelle Jaquet-Pilloud, Marie-Elise Verga, Michel Russo, Mario Gehri, Jean-Yves Pauchard

    Dear Editor

    We enjoyed reading the study by Jaquet-Pilloud et al. 1 examining the role of nebulised 3% hypertonic saline (HS) and bronchiolitis. We thank the authors for this excellent pragmatic non-blinded, randomised controlled trial. Their conclusions support the UK evidence from the SABRE study 2 and their systematic review3 which provides evidence of the futility of adding hypertonic saline to the management of bronchiolitis when compared to standard care alone with length of stay (LOS) or ready for discharge as the primary outcome. The article was well written and easy to follow. The study examined 121 infants with bronchiolitis recruited over three years from one tertiary centre in Switzerland. We felt it illustrated the very important problem of underpowered studies concluding no differences between two treatment regimens. The authors used the Korppi et al 3 study to assume that the mean LOS for infants admitted to hospital with bronchiolitis was 5 days [120 hours] with a standard deviation of 1.2 days [28.8 hours]. Reduction in hospital stay by 1 day was considered clinically significant and based on this a minimum sample size of 120 (with 60 in each group) was arrived. However the data from this paper by Jaquet-Pilloud et al show that the mean LOS was 47 hours (± 8.5) for nebulised hypertonic saline group and 50.4 hours (±11) for standard care group. The LOS at the authors’ hospital was less than half of the assumption used for their power calculation....

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  • Response to Dr Whitaker's letter

    Dr Whitaker, in a letter in response to our Archimedes review of whether waveform capnography reliably approximates paCO2 in neonates, highlights two important questions which capnography seeks to address: Firstly, whether or not the endotracheal tube (ETT) is patent and correctly positioned in the trachea and secondly, whether the current ventilation strategy provides optimal CO2 clearance for the patient. The two questions are, of course, interlinked.
    To date, in our field of neonatal medicine, the ETCO2 provides a valuable adjunct to clinical examination in determining ETT position and patency both at the point of intubation and during ongoing mechanical ventilation. However, for reasons explained in the paper, the numerical approximations to alveolar pCO2 provided by the currently available techniques of wave form capnography in neonates are not accurate enough to guide ventilatory changes. Thus, to guide ventilator changes, many neonatal intensive care units currently use transcutaneous capnometry.
    In addition to the physiological properties, the waveform capnography sensors add extra weight and dead space to an infant’s ventilator circuit. This adds further complexity, like their still not fully assessed effect on volume-guarantee ventilation and potential for auto-triggering of ventilators. As volume guarantee is now considered the gold standard for ventilating preterm infants with respiratory distress syndrome, the value of waveform capnography, in addi...

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  • Prof Tim Cook

    Dear Editor
    We read with interest Dr Scrivens et al’s commentary [1]. The mother’s question - ‘should capnography be used for breathing tube monitoring?’ – captures the subject addressed in our ‘PICNIC survey’ [2]. Conversely, the authors examine a completely different question - ‘is capnography an optimum respiratory monitor in ventilated neonates?’
    A respiratory monitor detects whether the end-tidal CO2 value usefully measures pulmonary ventilation or PaCO2. Although not our focus here, we are surprised the review omitted Kugelman’s study which reported waveform capnography monitoring in neonatal ICU (NICU) improved ventilation accuracy and neurological outcomes [3].
    An airway monitor assesses ‘whether lung ventilation is taking place via a tracheal tube that is in the airway and is patent’. High rates of neonatal failed intubation, oesophageal intubation, accidental extubation and reports of associated patient harm all suggest the value of a reliable airway monitor in NICU. Waveform capnography rapidly detects correct intubation with few false positives and immediately detects displacement or disconnection, the evidence for which we have previously set out [4-6].
    Some neonatologists argue that continuous waveform capnography cannot be used in neonates. It is used routinely in neonatal anaesthesia. Others use it routinely during transfer of small neonates (eg 400g) (personal communication Dr James Tooley, Consultant, Bristol) sometimes only for it...

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  • Multiple café-au-lait macules and Legius syndrome

    Dear authors,
    I read with great interest your article on multiple café-au-lait macules and movement disorder (1). I want to point out that probably an error happened during formatting of table 1. The term RASopathies is used to refer to a group of diseases caused by a mutation in a gene coding for a key component of the RAS-pathway and resulting in hyperactivation of the pathway. This group commonly includes Noonan syndrome, Neurofibromatosis type 1, Legius syndrome, LEOPARD syndrome (now referred to as Noonan syndrome with multiple lentigines), Costello syndrome, CFC syndrome, hereditary gingival fibromatosis, capillary malformation-arteriovenous malformation and Loh syndrome (2). It is confusing to find RASopathies next to Noonan, Legius and LEOPARD syndrome in the same column of table 1. In the same column Legius syndrome is listed as well as NF-like syndrome. Legius syndrome is listed followed by (PTPN11) and NF-like syndrome is followed by the gene (SPRED1). PTPN11 should be listed after Noonan syndrome because it is the most frequent cause of Noonan syndrome and it is not related to Legius syndrome. Legius syndrome is the same as NF1-like syndrome and only one of the two should be listed followed by (SPRED1). In our first publication (3) we named the condition neurofibromatosis 1-like syndrome but later is was renamed Legius syndrome (4).
    Congenital mismatch repair deficiency (CMMRD) is another autosomal recessive condition with café-au-lait macules and...

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  • Waveform capnography is reliable to ensure oxygenation

    I was interested to read the Archimedes article reviewing the structured question ‘in neonates who require ventilation, does waveform capnography give an accurate approximation of PaCO2?’ The findings such as the accuracy of ETCO2 decreases with the severity of lung disease (Grade B) adds to similar knowledge about waveform capnography when it was introduced in adults.

    Whenever capnography is discussed however it should always be remembered that the primary reason for its introduction into clinical practice was to reliably ensure patients oxygenation and reduce the incidence of hypoxic brain damage, which it did so dramatically. The presence of a capnography waveform is the gold standard to demonstrate the integrity and correct position of an airway and establish that the patient is being ventilated with the intended oxygen. This eureka moment discovering that waveform capnography is more about oxygenation than accuracy of PaCO2 estimation is crucial for patient safety. The exact value of PaCO2 is secondary.

    Unfortunately for over 20 years adult intensive care missed this eureka moment and consequently never started to use waveform capnography in adult ITUs when it was being universally introduced into operating theatres in the late 1980s [1].

    Despite waveform capnography continuing to save many patients lives in operating theatres the argument that the accuracy of ETCO2 decreased with the severity of lung disease predominated in intensive care and w...

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  • Paediatricians support national epidemiological surveillance.

    We welcome the comments made by Professor Andrew Williams, who has been a great supporter of our UK-wide study of children with progressive intellectual and neurological deterioration (PIND). The PIND Study uses the mechanism provided by the British Paediatric Surveillance Unit (BPSU), which is based in the Royal College of Paediatrics and Child Health. Since 1986 the BPSU has provided paediatricians in the United Kingdom with the means of investigating rare disorders of childhood. As Professor Williams points out there is a need to make research central to good paediatric practice and the BPSU continues to facilitate that.
    The PIND Study is funded by the National Institute for Health Research (NIHR) Policy Research Programme to look for cases of variant Creutzfeldt-Jakob disease (vCJD) among the many neurodegenerative diseases of childhood. Since the PIND Study started in 1997 we have identified children with more than 190 of these rare disorders - that number constantly increases as new diseases and new genetic variants of known diseases are discovered. Thus our study not only provides the sole means of systematically searching for vCJD in children but also gives a unique oversight of the changing pattern of childhood neurodegenerative disease in the UK. We work closely with the National Creutzfeldt-Jakob Disease Research and Surveillance Unit which carries out surveillance for vCJD in adults.
    Professor Williams highlights the fact that our work could not be...

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  • Response to e-letter regarding Primary Care screening tool

    We thank Luamar Dolfini and Gabriella Williamson for noting the sepsis screening tool that we developed in Leeds. Our tool was based on the NICE guidance, but used local early warning scores (PAWS) to simplify the assessment risk for sepsis. At Leeds Children's Hospital our tool is used on all acute paediatric admissions and in any child that deteriorates on the paediatric wards. Since our initial letter was published in 2018, our team have further amended our screening tool in response to human factors work, and have introduced the acronym LEEDS (Look for sepsis is all acute admissions or children who deteriorate: Evaluate the risk of sepsis by completing the sepsis screening tool; Escalate to a senior decision maker to consider the risk of sepsis; Decide whether there is a high/medium/low risk of sepsis using clinical assessment and investigations such as lactate; Start antibiotics in under 60 minutes if sepsis is a possibility). Our team have found the paper by Roland and Snelson ("So why didn't you think this baby was ill?" Decision-making in acute paediatrics, Arch Dis Educ Pract Ed 2019; 104:43-48) invaluable in educating our team about making decisions and assessing risk and this e-letter highlights that all parts of the puzzle (e.g. a full and comprehensive set of observations) are essential in being able to appropriately risk stratify patients, including for sepsis.

  • Supporting the PIND study as part of good paediatric practice.

    This most welcome paper by Verity et al relates the important longstanding work that the PIND Study produces and which all paediatricians should most strongly continue to support.[1]

    However, it is important for readers to understand that the PIND Study itself cannot in many cases be expected to be the full story when a child is referred to them.

    Indeed the relationship between the referring paediatrician and the PIND Study group can very helpfully continue long after the patient's death when new investigative technologies can finally provide a definitive diagnosis, so long as the appropriate samples have been appropriately taken. In this area, I have found guidance from the PIND Study can be very helpful.

    We in Northampton have always referred where appropriate to the PIND Study not only because we highly esteem its work, but also because it remains the only practical means of systemic surveillance of vCJD and other neurodegenerative conditions in the UK. Where inspite of every endeavour a diagnosis has not been found while the patient was alive, we in Northampton have continued to keep the PIND Study in the loop while working internationally with other groups to find an answer.

    For one such example, we have had children 2 brothers both referred to the PIND study in the early 2000's with a then undiagnosed condition. Both boys, having had post mortems and DNA storage and working with Professor Baas in the Netherlands were found to hav...

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