Having just read this article I am concerned about the terminology used as I am not sure it truly reflects the clinical problem posed. The article refers to "maintenance" fluids but the question asked relates more to “resuscitation” fluids.
It is important to be clear as to the aim of treatment in the individual patient when prescribing fluids rather than just following a guideline. The paper debates the relative merits of 0.9% sodium chloride and balanced fluids as “maintenance” fluids. To my mind “maintenance” fluids are administered to patients who have a replete extracellular fluid (ECF) volume. If ECF volume is low then “resuscitation” fluids are required. “Maintenance” and “resuscitation” fluids have different roles and therefore might be expected to have different characteristics.
As the article refers to “maintenance” fluids I will deal with these first. This fluid is needed to replicate the fluid that the patient would normally be drinking but for a variety of reasons may not be able to ingest. It should be differentiated in turn from "replacement" fluid which is the fluid given on top of the "maintenance" fluid when patients have fluid losses in excess of those normally anticipated. This includes diarrhoea, vomiting and fluid from surgical drains. The fluid used for "replacement" needs to match the composition and volume of the fluid being lost. Once "resuscitation" and "replacement" fluid...
As the article refers to “maintenance” fluids I will deal with these first. This fluid is needed to replicate the fluid that the patient would normally be drinking but for a variety of reasons may not be able to ingest. It should be differentiated in turn from "replacement" fluid which is the fluid given on top of the "maintenance" fluid when patients have fluid losses in excess of those normally anticipated. This includes diarrhoea, vomiting and fluid from surgical drains. The fluid used for "replacement" needs to match the composition and volume of the fluid being lost. Once "resuscitation" and "replacement" fluids have been taken care of and the ECF compartment is full, "maintenance" fluid needs to be prescribed. This equates to insensible water losses and urine output and will be the same as the fluid and electrolytes we take in orally each day. Current guidelines propose the use of a fluid containing 154 mmol/l of sodium. This article questions whether this should be accompanied by chloride or a mix of chloride and potential bicarbonate. However none of us would dream of drinking such a fluid and the recommended sodium intake for infants is around 3 mmol/kg/day which for a 10 kg child given the standard 1 litre of maintenance fluid equates to a solution containing 30 mmol/l or 0.18% sodium chloride. This is the sodium concentration you will find in total parenteral nutrition (TPN). Using 0.9% sodium chloride gives 154 mmol of sodium or 15.4 mmol/kg! The concern about using hypotonic solutions as “maintenance” fluid centres around the notion that the syndrome of inappropriate anti-diuretic hormone secretion (SIADH) is a common phenomenon and administration of hypotonic solutions will cause hyponatraemia. However SIADH is overdiagnosed and when ADH levels are raised it is almost always appropriate i.e. ADH is being produced in response to ECF volume contraction. In that situation an isotonic solution is required. Once ECF volume has been restored ADH production will almost always be switched off and continuing use of isotonic solutions will risk causing hypernatraemia.
The question that should have been asked was which fluid was more appropriate for “resuscitation” purposes? In the scenario described it is the administration of a 20ml/kg bolus of 0.9% sodium chloride that has led to a hyperchloraemic metabolic acidosis. We have recently had this discussion in our department and as in this article, have had to look primarily at evidence from adult studies. At this meeting the use of isotonic crystalloid as "maintenance" fluid was questioned and subsequently we have seen three cases of hypernatraemia as a result of 0.9% sodium chloride “maintenance” therapy.
"Resuscitation" fluid is given to restore ECF volume. To achieve this it would make sense to use a fluid that matches the composition of ECF and unfortunately 0.9% saline only goes part of the way to achieving this. ECF contains 140 mmol/l of sodium, 4 mmol/l of potassium, 113 mmol/l of chloride and 26 mmol/l of bicarbonate and as a result, when using 0.9% sodium chloride to correct a deficit there is an excess of chloride and a lack of bicarbonate. This creates a metabolic acidosis. It therefore makes sense to use balanced fluids containing potential bicarbonate. The studies quoted in this article are primarily related to severely ill patients many with sepsis in whom ongoing problems with ECF volume will be an issue and cannot be extrapolated to children on a general paediatric ward.
In summary, prescription of intravenous fluids should only be made after a careful assessment of the patient, in particular their ECF volume status. If that is reduced an isotonic solution should be administered. A balanced solution makes more sense physiologically but the evidence supporting its use over 0.9% saline is limited, particularly in the paediatric population. If there are ongoing losses they should be replaced by a solution best matching the fluid being lost. If it is not possible to establish the electrolyte content of the fluid losses an isotonic solution is advised. Once ECF volume is replete then a hypotonic solution should be given and on our ward we would use 0.45% saline + 2.5% dextrose. This is still providing more sodium than is normally recommended if giving fluids orally or in TPN. Unfortunately ECF volume status is notoriously difficult to assess and if there is doubt isotonic solutions will be safer. However if 0.9% sodium chloride continues to be administered as "maintenance" fluid we will see more cases of hypernatraemia. Whatever strategy is adopted it is essential to monitor serum electrolytes at least daily in children receiving a significant (>50%) proportion of their fluids intravenously.
I have not mentioned the ongoing debate about the volume of "maintenance" fluid we should be giving children. Currently this is based on recommendations made by Holliday and Segar in the 1950’s, matching fluid requirement to energy expenditure. It is now recognised that these do not reflect the fluid requirements of unwell children lying in bed and as a result “maintenance” fluid volume prescriptions may be twice what they should be. The prescription of more water than is needed is potentially a contributing factor to hypotonic solutions producing hyponatraemia - the prescribing of too much water rather than too little sodium. It is also likely that the cases of hyponatraemia reported in the literature were the result of the prescribing of hypotonic solutions to patients who were deplete of ECF in which case isotonic solutions should have been given.
Thank you to the authors for bringing the concept of ACPs and their importance to the forefront for consideration in practice. Thankfully, I have never had to utilise one in my professional practice, but having recently been encouraged to use an antenatal ACP for our daughter, I would offer personal support to the intentions that they should be used in practice, when appropriate. Our daughter was given a terminal diagnosis antenatally, and the decisions that followed to prepare for her birth and her death would have been overwhelming and emotionally charged in the immediate aftermath and I can only assume that our journey would have looked very different without one. Instead, the decisions and plans were considered, they were controlled and they were based on our true wishes for our daughter and our family. As the authors relate too in their article, without the ACP there would have been significant likelihood that the decisions we made for our daughter would have been made ad-hoc, under emotional pressure, and the outcome of her peaceful and comfortable death, surrounded by her family and in our arms, would likely have not been as achievable. Furthermore, the authors comment that it guides healthcare providers and parents, in a structured manner to ensure needs and wishes are met and I could not agree more. I think much of the conversations that took place, would have taken place without the ACP document, but the concept as a whole encouraged timely discussions, e...
Thank you to the authors for bringing the concept of ACPs and their importance to the forefront for consideration in practice. Thankfully, I have never had to utilise one in my professional practice, but having recently been encouraged to use an antenatal ACP for our daughter, I would offer personal support to the intentions that they should be used in practice, when appropriate. Our daughter was given a terminal diagnosis antenatally, and the decisions that followed to prepare for her birth and her death would have been overwhelming and emotionally charged in the immediate aftermath and I can only assume that our journey would have looked very different without one. Instead, the decisions and plans were considered, they were controlled and they were based on our true wishes for our daughter and our family. As the authors relate too in their article, without the ACP there would have been significant likelihood that the decisions we made for our daughter would have been made ad-hoc, under emotional pressure, and the outcome of her peaceful and comfortable death, surrounded by her family and in our arms, would likely have not been as achievable. Furthermore, the authors comment that it guides healthcare providers and parents, in a structured manner to ensure needs and wishes are met and I could not agree more. I think much of the conversations that took place, would have taken place without the ACP document, but the concept as a whole encouraged timely discussions, ensured all parties were included, and offered a prompt that the conversations we would be having would be difficult, but that they were incredibly important. I, obviously can not be sure of how our team of professionals found the logistical use of our ACP at the time, but I can only assume it was of benefit, as they provided our daughters care, as requested, without any additional need for questions or decisions at an incredibly difficult time. From a parents viewpoint, the conversations that occurred whilst completing the ACP with our team, ensured that we had considered all aspects of her care, that we understood options and that everyone in the team, including us as parents, were all in agreement around ceiling of care. Our healthcare team knew her name from before she was born and therefore referred to her as such from the minute she arrived; they knew we wished for her sibling to join us as soon as possible to meet her, and they arranged this to prevent us wasting our fleeting moments together with texts and phone-calls or battling to allow relatives access to the busy the ward; they knew we wished to have photos taken and as such they did not fear snapping every moment for us (as a result we have the most beautiful and natural photos that we will cherish forever); they knew we wanted time alone so did not feel awkward at leaving us to just be together, and they knew that we wanted her not to leave our side, and that was never questioned. The ACP (and all the staff who made it happen!) brought us a beautiful short life for our daughter and a calm and peaceful death, which is really all you can hope for in such tragic circumstances.
I welcome Himmelmann’s editorial concerning the prevention of respiratory problems for individuals with cerebral palsy1. As a speech and language therapist working within a multi-disciplinary nutrition team, I recognise the need to increase our understanding of the complex interactions between risk factors through collaboration across stakeholders. It is of particular concern that solids or liquids in the lungs or windpipe have been identified as the cause of death for almost a quarter of people with cerebral palsy2.
With this in mind, we developed the Eating and Drinking Ability Classification System (EDACS) for people with cerebral palsy from age 3 years. EDACS classifies limitations to eating and drinking ability in 1 of 5 levels, replacing frequently used terms “mild”, “moderate” and “severe” which lack shared definition. Key features of “safety” and “efficiency” are used to determine 5 distinct levels of ability: from Level I Eats and drinks safely and efficiently through to Level V Unable to eat or drink safely – tube feeding may be considered to provide nutrition. EDACS demonstrated strong content validity and excellent inter-observer reliability when used by speech and language therapists3. EDACS is free to download from www.edacs.org along with sixteen completed translations. Ten other language translations are currently in process.
Himmelmann1 points out associations between limitations to gross motor function and...
Himmelmann1 points out associations between limitations to gross motor function and someone’s eating and drinking abilities. We found that there was a statistically significant but only moderate positive correlation between EDACS and the widely used GMFCS4 for a group of children with CP (n=128 Kendall’s tau 0.5)3. The clinical relevance of this is that some children with limited gross motor function will show greater ability when eating and drinking; conversely, other children who walk with or without assistance may eat and drink with increased risk of choking or aspiration.
Someone’s eating and drinking ability is not readily observed in most clinical contexts. Whilst video-fluoroscopic swallowing examinations will support identification of “silent aspiration”, it provides a useful but partial view of the overall clinical picture. Some health professionals may rely on parent or carer report. Parents were involved in the development of EDACS and found it easy to use to describe their children’s eating and drinking abilities. However, questions remain about parents’ use of EDACS to describe their children’s eating and drinking abilities. Direct comparisons of parents' and speech and language therapists’ classifications using EDACS revealed more disagreements than between pairs of speech and language therapists, although these were consistent: parents either agreed with speech and language therapists or parents systematically rated their children as more able than speech and language therapists, by one level3.
EDACS provides a framework which makes explicit the extent of the disagreement between parents and professionals including implications for safety and efficiency of children's eating/drinking. There is uncertainty whether children experience adverse health outcomes because of these disagreements. Disagreements between parents and healthcare teams regularly occur in this emotionally charged area of function. It can be challenging for the multi-disciplinary team to provide children with cerebral palsy and their parents with person-centred healthcare with consequent negative impacts on the well-being and quality of life of their child and family5.
EDACS provides a framework with potential to support person-centred healthcare. Further work needs to be carried out to implement use of EDACS across community and acute healthcare settings to fully realise its preventative potential.
1. Himmelmann K. Putting prevention into practice for the benefit of children and young people with cerebral palsy Archives of Disease in Childhood Published Online First: 18 July 2018. doi: 10.1136/archdischild-2018-315134
2. Glover G and Ayub M (2010). How people with learning disabilities die. Published by Improving Health and Lives: Learning Disabilities Observatory. http://www.improvinghealthandlives.org.uk/uploads/doc/vid_9033_IHAL2010-... accessed 21 September 2013.
3. Sellers D, Mandy A, Pennington L, Hankins M and Morris C (2014). Development and reliability of a system to classify the eating and drinking ability of people with cerebral palsy. Developmental Medicine & Child Neurology 56(3):245-251
4. Palisano R, Rosenbaum P, Walter S, Russell D, Wood E and Galuppi B (1997). Development and Reliability of a System to Classify Gross Motor Function of Children with Cerebral Palsy. Developmental Medicine &Child Neurology 39:214 -223.
5. Cowpe E, Hanson B and Smith C (2014). What do parents of children with dysphagia think about their MDT? A qualitative study. BMJ Open 4 e005934.
Thank you for the opportunity to reply, here is our response.
We would like to thank the authors for their valuable comments, we believe that these comments add to and complement our article. Our article aimed to cover a wide breadth of common gynaecological conditions that can affect children and unfortunately we were therefore not able to go in to great detail for each condition covered. We would certainly agree on the importance of an early diagnosis for lichen sclerosus and collaboration with a dermatologist for treatment if available. The British Association of Dermatologists guidelines for the management of lichen sclerosus was not published when we wrote our article, we can see that this is a very valuable resource.
We read with interest the review on ‘The paediatrician and the management of common gynaecological conditions’ (1). This is an important topic but we have concerns about incorrect information in this paper with respect to vulval disorders. Most vulval conditions are dermatological rather than gynaecological and the involvement of a dermatologist in the management of these children is vital. The breadth of paediatric vulval disease is not reflected in this paper including the concept of vulval presentation of various skin conditions (eg. psoriasis, chronic bullous dermatosis of childhood, erythema multiforme) and the need to examine the rest of the skin including the hair, nails and mucosa.
There appears to be some confusion within the article, for example, a vaginal discharge is not a vulval disorder. While a discharge can lead to a vulvitis, the two problems of vaginitis and vulvitis should be distinguished as the investigation and management of each is very different.
The commonest condition seen in children presenting with vulval symptoms is an irritant dermatitis which is not specifically mentioned in the section on vulval irritation. This is often seen in those with a background of atopy and requires emollients and a mild topical steroid application intermittently with good hygiene measures. Other important disorders such as Lipschutz ulcers and genital warts have been omitted.
However, our major concerns relate to the section o...
However, our major concerns relate to the section on lichen sclerosus (LS). This is an important condition which is often under-recognized or with significant delay in diagnosis. The references used are old and not original. The major symptom is itch which is not always synonymous with irritation. One of the frequent presenting symptoms of LS in children is constipation which again is not mentioned. LS should always be treated, even if asymptomatic as it is a disease that can lead to permanent anatomical change with functional sequelae. Detailed guidelines for the management of LS have been published recently (2) and were agreed by the Royal College of Paediatrics and Child Health in the consultation process. All the evidence shows that a 3 month induction regimen of an ultra-potent topical steroid is the first line treatment (3). Thereafter, the management is individualized to maintain control of symptoms and signs (4). The management outlined in this review is incorrect and gives the wrong message. It should be corrected as otherwise it will lead to undertreatment and its potential consequences. As with all vulval disorders, these children should be under the care of those experienced in the management of this condition and should receive dermatological input as part of the multi-disciplinary team.
FM Lewis MD FRCP, St John’s Institute of Dermatology, Guy’s & St Thomas’ Hospital, London
SS Velangi FRCP Queen Elizabeth University Hospital, Birmingham
SM Taibjee MB BCh BMedSci MRCPCH CCT Derm DipRCPath (Dermatopathology) Dorset County Hospital, Dorchester
1. Ritchie JK et al. The paediatrician and the management of common gynaecological conditions. Arch Dis Child 2018;108:703-6
2. Lewis FM Tatnall FM, Velangi SS et al. British Association of Dermatologists guidelines for the management of lichen sclerosus, 2018. Br J Dermatol 2018;178:839-53.
3. Mashayeki S, Flohr C, Lewis FM. The treatment of vulval lichen sclerosus in prepubertal girls: a critically appraised topic. Br J Dermatol 2017;176:307-16.
4. Ellis E, Fischer G. Prepubertal-onset vulvar lichen sclerosus: the importance of maintenance therapy in long-term outcomes. Ped Dermatol 2015:32:461-7.
This study was published online in Archives of Disease in Childhood after peer review in September 2017. The trial tested the effectiveness of a neurolinguistic programming intervention (used widely but never formally tested) in children and young people with chronic fatigue recruited between 2010 and 2013. Though the number of participants was small, analysis suggested a benefit in terms of physical function (measured by the standard SF 36 scale) at both 6 and 12 months after intervention.
Since publication, the study has been criticised for failing to meet ICMJE and BMJ policy on trial registration and for not fully adhering to CONSORT guidance on trial reporting. The journal has been criticised for not detecting these issues during editorial and peer review. We have acknowledged these comments and reviewed our processes in relation to this paper and relating to EQUATOR guidance in general. In addition, we have received clarifications from the authors which are under editorial consideration.
I was interested to read the articles in this month’s journal exploring the difficulties of end of life decisions when parents and their doctors cannot agree.(1–3) These articles reflect the global media attention focused upon several recent tragic cases in the UK, where differences in view between parents and the clinical team led to confrontation and an unfolding tragedy in the public arena. Whilst all these articles describe the complexity they offer little in terms of solutions. Is it possible to prevent future cases from degenerating into public dispute, or is it an inevitable consequence of modern medicine? Have we advanced to a point where children that would have succumbed now live, and so the focus of care has shifted towards how they live rather than if they live or die?
At least part of the solution should be a shift in focus shift toward prevention of conflict in these high stakes clinical areas rather than finding a remedy once conflict has occurred. This is not just about being better at communicating with families. Conflict prevention will require cultural change, the identification of early warning signs and the use of mediation to facilitate communication between parents and doctors at an early stage.
Communication is not just about what we say, but about how we act and the social networks that we live and work in. It was interesting that there was also an article on Family Integrated Care in the same issue of the journal (4). Patel and colle...
Communication is not just about what we say, but about how we act and the social networks that we live and work in. It was interesting that there was also an article on Family Integrated Care in the same issue of the journal (4). Patel and colleagues describe a new way of working in intensive care medicine, where parents are integrated into the clinical team as primary caregivers. They describe how cultural changes to clinical practice enhance the wellbeing of parents and reduce length of stay of their new-borns. Parental integration will surely improve communication between parents and staff. It reduces barriers to effective communication and creates a shared problem rather than adopting sides.
Despite everyone’s best efforts, there will be times when dissatisfaction and conflict arise. To prevent communication breakdown we should identify risk factors within the situation that would lead to early intervention. Forbat et al identified three distinct phases of escalation in paediatric conflicts from mild, through, moderate to severe.(5) The mild stage focussed on conflict triggers, for example, the inappropriate use of language, conflicting messages given to parents by clinical staff, staff making assumptions about parents and a history of previous unresolved conflict. Training clinical staff to recognise conflict early and use mediation skills to de-escalate and resolve it is another intervention. Six month follow up of a cohort of staff trained in one tertiary children’s hospital reported that 57% of respondents had experienced conflict in the six months following the training. Of these, 91% reported that the training had enabled them to de-escalate the conflict.(6) Formal testing of a framework for the early recognition and management of conflicts between families and health professionals is being undertaken by four tertiary hospitals in the UK later this year.
Only after these foundations are in place, where parents are empowered as part of the clinical team, and an open and safe environment for communication already exists, that local hospital review panels and clinical ethics committees and the courts may be able to help when complex clinical decisions need to be considered. These panels should be introduced as part of the wider clinical team. Doctors and parents should approach them together as a united front to listen to their deliberations.
It is vital that we learn how to prevent these high-profile cases from occurring again. There are no winners or losers in these situations, only victims and casualties: parents whose chance of coping with bereavement has been broken, and a career curtailing event for the doctors and nurses who cared for them. When parents and doctors are in conflict, once sides have been drawn the battle is lost. Changing the way that we work with families, mediation as routine practice, and the early identification of the warning signs of conflict is the only way to prevent this from happening again.
1. Wallis C. When paediatricians and families can’t agree. Arch Dis Child. 2018 May;103(5):413–4.
2. Wheeler R. Response to “When paediatricians and families can”t agree’. Arch Dis Child. 2018 May;103(5):410–1.
3. Lagercrantz H. Observations on the case of Charlie Gard. Arch Dis Child. 2018 May;103(5):409–10.
4. Patel N, Ballantyne A, Bowker G, Weightman J, Weightman S, Helping Us Grow Group (HUGG). Family Integrated Care: changing the culture in the neonatal unit. Arch Dis Child. 2018 May;103(5):415–9.
5. Forbat L, Teuten B, Barclay S. Conflict escalation in paediatric services: findings from a qualitative study. Arch Dis Child. 2015 Aug;100(8):769–73.
6. Forbat L, Simons J, Sayer C, Davies M, Barclay S. Training paediatric healthcare staff in recognising, understanding and managing conflict with patients and families: findings from a survey on immediate and. Arch Dis Child. 2017 Mar;102(3):250–4.
The story of Charlie, like that of the little Alfie, are events on which everything has been said, but without an adequate reflection on some basic principles that concern precisely the respect for life and the quality of care that daily thousands of health workers try to provide terminal patients. We can discuss for a long time what is best for the interest of the individual patient and family, but the value of the scientific method that constitutes the cornerstone of the medical profession can not be ignored.
Likewise it is the duty of the community to uphold the moral integrity of clinical practice by refusing to provide treatments that do not meet a reasonable scientific justification based on evidence of efficacy. Not thinking according to these principles are also betraying the dictates of the heart and not only those of a reasonable science, which should always be at the service of the patient's good, even in the face of death, in a society that should be defined as "civil".
If it is true that the heart has its reasons that reason does not know, it is the heart that, in the case of terminal children, makes the best choices.
He wrote anonymously one of the two hundred health care workers who followed Charlie: "We did not want to lose Charlie, but it was our legal and moral obligation, our job, to become his spokesman when it was time to say enough".
I read with great interest the article titled “Off-label use of tacrolimus in children with Henoch-Schönlein purpura nephritis: a pilot study” by Zhang et al.(1). To my great astonishment the authors did not discuss any of the studies published on the use of another calcineurin inhibitor Cyclosporine A (CyA) in the treatment of Henoch Schönlein purpura in children.
In the first chapter of the discussion section they refer to our report where we compared methylprednisolone pulse treatment (MP) and CyA in a randomized trial (2) stating that “Remission was achieved slowly and only in 53% of patients with methylprednisolone” (1).
I would like to draw the readers´s attention to the fact that in the same paper we showed that another calcineurin inhibitor i.e. CyA was by no means inferior to MP for the treatment of severe HSN (2). Indeed, CyA was even more efficacious than MP, since remission was achieved within 3 months in all CyA-treated patients (N=11) compared to 54% (7/13) in MP group (p=0.016). All the CyA treated patients responded to the treatment with no need for additional immunosuppressive therapy. In contrast, in MP group 6/13 (46%) needed additional immunosuppressive treatment. The remission rates in the MP treated patients were 85% (11/13) and 77% (10/13) after 1 and 2 years, respectively in contrast to 100 % in CyA-treated patients. The renal survival rate in the CyA group was 100%, as against 85% in the...
I would like to draw the readers´s attention to the fact that in the same paper we showed that another calcineurin inhibitor i.e. CyA was by no means inferior to MP for the treatment of severe HSN (2). Indeed, CyA was even more efficacious than MP, since remission was achieved within 3 months in all CyA-treated patients (N=11) compared to 54% (7/13) in MP group (p=0.016). All the CyA treated patients responded to the treatment with no need for additional immunosuppressive therapy. In contrast, in MP group 6/13 (46%) needed additional immunosuppressive treatment. The remission rates in the MP treated patients were 85% (11/13) and 77% (10/13) after 1 and 2 years, respectively in contrast to 100 % in CyA-treated patients. The renal survival rate in the CyA group was 100%, as against 85% in the MP group (2). Several reports have shown that CyA is effective also as a rescue therapy for Henoch Schönlein nephritis when other treatments have failed (3-5). Faul et al. have suggested that the beneficial effect of CyA on proteinuria is not dependent on T-cell function, but rather results from stabilization of the actin cytoskeleton in the kidney podocytes (6).
1. Zhang D-F, Hao G-X, Li C-Z et.al Off-label use of tacrolimus in children with Henoch-Schönlein purpura nephritis: a pilot study. e-pub http://dx.doi.org/10.1136/archdischild-2017-313788.
2. Jauhola O, Ronkainen J,Autio-Harmainen H, et al. . Cyclosporine A vs methylprednisolone for Henoch-Schönlein nephritis: a randomized trial. Pediatr Nephrol 2011;26:2159-66.
3. Ronkainen J, Autio-Harmainen J, Nuutinen M. Cyclosporin A for treatment of severe Henoch-Schönlein glomerulonephritis. Pediatr Nephrol 2002;18:1138-42.
4. Someya T, Kaneko K, Fujinaga S, Ohtaki R, Hira M, Yamashiro Y. Cyclosporine A for heavy proteinuria in a child with Henoch-Schönlein purpura nephritis. Pediatr Int 2004;46:111-3.
5. Park J, Won S, Shin J, Yim H, Pai K (2010) Cyclosporin A therapy for Henoch-Schönlein nephritis with nephrotic-range proteinuria. Pediatr Nephrol 2010;26:411-7.
6. Faul C, Donnelly M, Merscher-Gomez S et al. The actin cytoskeleton of kidney podocytes is a direct target of the antiproteinuric effect of cyclosporine A. Nat Med 2008;14:931-938.
The evidence that nasal mask continuous positive airway pressure (CPAP) is better than bilateral, short nasal prong CPAP is convincing.1 However the evidence of benefit is only for short-term outcomes. I have significant concerns about using nasal mask CPAP continuously over many days in extremely preterm or extremely low birth weight infants. These babies have soft malleable skulls. We saw that this was a problem decades ago when small babies were primarily nursed with their head on the side; and subsequent dolichocephaly was very common as a result.2 The hat and straps needed to keep a mask in place put pressure on a baby's soft, malleable skull in a different way to the hat used during nasal prong CPAP. The long term effects of this pressure on boney development, particularly of the midface, are unknown, but they could be considerable. Because of these concerns I do not use nasal mask CPAP continuously. I alternate the use of masks with nasal prongs.
1. Kieran EA, Twomey AR, Molloy EJ, et al. Randomized trial of prongs or mask for nasal continuous positive airway pressure in preterm infants. Pediatrics 2012;130:e1170–6.doi:10.1542/peds.2011-3548.
2. Ifflaender S, Rüdiger M, Konstantelos D, Wahls K, Burkhardt W. Prevalence of head deformities in preterm infants at term equivalent age. Early Human Development 2013;89(12):1041-1047.