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Objective: To determine the inter-rater reliability and validity of the Netherlands Triage Standard (NTS) for paediatric triage., Design: A cross-sectional study using fictional cases for telephone and physical triage., Method: An expert panel established in advance the urgency of 40 cases concerning emergency help requests from non-referred children (the reference standard). These requests were presented in an online survey to triagists from three general practitioner (GP) out-of-hours practices, three ambulance dispatching centres and three hospital emergency departments. Triagists assessed all cases, using the NTS. We determined the agreement on degrees of urgency between different triagists and compared them with the reference standard. The outcome measure for inter-rater reliability was the intraclass correlation coefficient (ICC). The outcome measures for validity were the degree of agreement with the reference standard, under-triage and over-triage, and sensitivity and specificity in identifying high-urgency (U0-U2) versus low-urgency cases (U30U5)., Results: In total, 116 triagists participated in the study (response: 86%). The ICC was 0.73 among all triagists, and was highest in the out-of-hours GP cooperatives. There was 62.3% agreement with the reference standard, 17.4% under-triage and 20.2% over-triage. Of the divergent urgencies, 77% differed by only one urgency category. The sensitivity was 85.2% and the specificity 89.7%. The sensitivity and specificity of triage by the GP out-of-hours practices (82.7% and 92.7%, respectively) were almost the same as that by the hospital emergency departments (79.6% and 92.5%, respectively). Triage by the ambulance dispatching centres had relatively high sensitivity (93%), but relatively low specificity (82.4%)., Conclusion: The results of the study contribute to the evidence that the NTS is a reliable and valid triage standard for paediatric patients. The urgency assessments by triagists in the GP out-of-hours practices, ambulance dispatching centres and hospital emergency departments were broadly in agreement. Results were limited by cases being on paper and triage only on anamnestic characteristics.

In this case series, we describe four children and adolescents with tall stature or growth acceleration to illustrate the diagnostic evaluation of tall stature according to the new Paediatric Association of the Netherlands (NVK) Guideline on growth disorders. A 14-year-old girl with tall stature and a relatively late onset of puberty was diagnosed with idiopathic familial tall stature, and the patient decided not to opt for epiphysiodesis. A 14-year-old boy with prepubertal growth acceleration and a history of behavioural problems was diagnosed with Klinefelter syndrome. A 7-year-old boy with tall stature, arachnodactyly, pectus excavatum and lumbar scoliosis was diagnosed with Marfan syndrome. Finally, a 16-year-old girl with isolated progressive tall stature was diagnosed with growth hormone excess caused by a pituitary somatotroph adenoma. The most clinically relevant conditions associated with tall stature are Klinefelter and Marfan syndrome, and secondary growth disorders such as precocious puberty and growth hormone excess.

In children, a malignant lymphoma can present with life-threatening complications arising from progressive tumour growth, warranting swift diagnosis and treatment. Early recognition can be challenging if a child with paediatric lymphoma presents with abdominal and throat symptoms because of the frequency, and frequently benign nature, of these symptoms in the general paediatric population. In these cases, it is essential for the physician to recognize the alarm signals for suspicion of malignant lymphoma, to allow timely diagnosis and treatment. We present two cases (children age 8 and 16 years) demonstrating the symptoms of malignant lymphoma that should raise alarm in children presenting with unilateral tonsil enlargement or intussusception, and illustrating the importance of timely recognition of these alarm signals, to prevent life-threatening complications due to tumour progression. In patients presenting with unilateral tonsil enlargement, malignant lymphoma should be considered if atypical macroscopic tonsil morphology, cervical lymphadenopathy, dysphagia, snoring, fever or weight loss is present. The presence of a lead point as the cause of intussusception should be considered in children aged > 2 years of age, in a child with an ileo-iliac intussusception and in patients with longstanding abdominal symptoms. In patients presenting with simultaneous symptoms and alarm signals for malignant lymphoma, the relationship between the presenting symptoms should be recognized. Interdisciplinary and intradisciplinary consultation with colleagues can help in the search for an overarching diagnosis, and it is crucial that this should happen without delay to prevent acute life-threatening situations.

A big drop in the number of severe infections has resulted in less experience in recognising a seriously ill child. The challenge is finding a safe and effective balance between high-quality expertise and quickly accessible care, while avoiding over-diagnosis. There are a number of tools available to aid recognition of a seriously ill child and to avoid delay in diagnostic procedures and treatment: the use of a validated paediatric triage system, validated decision rules and guidelines, listening carefully to the parents ('my child's illness is different this time'), the clinical intuition of the experienced paediatrician and the provision of good 'safety net' advice to parents concerning the alarm signals and when they should contact a care provider. Experienced paediatricians should be at the forefront in the evaluation of the acutely ill child in order to teach their younger colleagues the importance of various alarm signals and the role played by clinical intuition.

Objective: To give an overview of disciplinary cases regarding action taken by paediatricians and paediatric residents in cases of (suspected) child abuse and to discuss the considerations of the disciplinary board in these cases., Design: Retrospective, descriptive study., Method: We considered all disciplinary cases instigated from 2001 to 2013 against paediatricians or paediatric residents and selected complaints regarding action taken in cases of (suspected) child abuse. We divided these complaints into six categories and studied the considerations of the disciplinary board in these cases., Results: From 33 disciplinary cases instigated from 2001 to 2013, we selected 76 complaints regarding action taken by paediatricians or paediatric residents in cases of (suspected) child abuse. The majority of these complaints concerned the reporting or requesting of information in the context of (suspected) child abuse. All of the complaints in the category 'unwarranted reporting of child abuse' were declared unfounded by the disciplinary judge., Conclusion: The disciplinary board declared all complaints unfounded in cases where the paediatrician or paediatric resident had followed the Dutch national protocol regarding reporting of child abuse and domestic violence. The disciplinary board examines whether action was taken in accordance with reasonable standards of professional competence and considers that paediatricians have an important role in identifying child abuse.

Health information technology (eHealth) can be cost-effective if it replaces existing care services. The introduction of eHealth has provided the opportunity to directly support and improve care for children with chronic diseases. We show an example of this in children with diabetes mellitus. Giving patients direct feedback on their glucose levels results in better outcomes of patient care. Accessing data from electronic patient files and using them effectively, however, is not yet possible in a number of health care institutions.

A more prominent role for the youth healthcare physician: Youth Healthcare physicians reflect on the Dutch Paediatric Association (NVK) guideline entitled 'Gastro-oesophageal reflux (disease) in children aged 0-18 years'. This guideline states that medicinal treatment is given to these children too often. Dutch Youth Healthcare physicians see a large number of children with gastro-oesophageal reflux, with or without additional symptoms. The most common symptoms (crying and diminished weight gain) might be present even in the absence of reflux. Parents should be given advice and support when they are worried about reflux, crying or low weight gain. A lower weight gain curve on the growth chart is normal in breastfed children, and crying might be due to factors such as lack of routine or stimulus reduction. Overfeeding might also be the cause. Parents should be supported and followed up, with or without treatment, as necessary. Youth Healthcare professionals could perform this task.

Febrile children pose the diagnostic dilemma of distinguishing those with serious infections from the vast majority with self-limiting diseases at an early stage. Alarm symptoms can aid in assessing the probability of serious infections in febrile children. The combined absence of alarm symptoms is useful in ruling out serious infections. CRP and PCT values are important diagnostic markers in febrile children in hospital settings. Children without any alarm symptoms and low inflammatory markers can be treated conservatively, provided there are good instructions on reassessment. Children with amber alarm symptoms or mildly elevated inflammatory markers in whom a serious infection cannot be ruled out require clinical observation or ambulant follow-up; empirical parenteral antibiotic treatment should also be considered and easily accessible. Children with red alarm symptoms or highly elevated inflammatory markers deserve clinical observation. In children < 3 months, empirical parenteral antibiotic treatment is also indicated.

In 2102, a multidiscplinary guideline was developed on behalf of the Dutch Association of Pediatrics entitled 'Gastroesophageal reflux disease in children from 0-18 years'. The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) guideline from 2009 served for guidance. History and physical examination are usually sufficient for diagnosing gastroesophageal reflux disease. It is important to make a distinction between gastroesophageal reflux, a physiological phenomenon in children, and gastroesophageal reflux disease. In practice, medications are provided too often, especially in infants. Only children suffering from gastroesophageal reflux disease should be treated according to the therapeutical action plan.

Recent publications on the long-term effects of treatment for childhood cancer underline the need for a larger role of primary care in the guidance given to these patients regarding risks and lifestyle. This group is growing and there are a large number of potential long-term risks. Practice guidelines on registration in the electronic patient file are needed. Sometimes active screening is required, sometimes potential risk factors become manifest. The replacement of secondary care (often specialised outpatient clinics) with primary care should be an active process, which is supported by personalised and up-to-date information in the form of a letter from the specialist to the primary care physician. Patients can play an important role in drawing up the primary care follow-up plan.

It is not known if many existing medications can safely be used in children. For this reason the EU Paediatric Regulation came into force in 2007. When new medications are developed, pharmaceutical companies are obliged to produce a plan to research their use in children which must be approved by the regulatory authorities. Existing data on medications also had to be submitted. Review took place five years after the introduction of this regulation: of 682 medications, 476 plans were approved. In 206 of these medications this was unnecessary. By the end of 2011, research into 29 medications had been completed. The use of 10 of the 113 new, previously unregistered medications in children was approved, as was the case with 30 registered medications. Examination of existing data shows that results are often limited to changes in the 'Summary of Product Characteristics'. Many planned studies still have to be wound up, but in time the amount of information on safety and efficacy of new medications in children will increase greatly.

In 2012, at the request of the Dutch Society of Paediatrics, a multidisciplinary working group developed a guideline entitled 'Helicobacter pylori infection in children aged 0-18 years'. The 2011 international guideline served as the guiding principle for this. The guideline includes recommendations on which children should be tested for H. pylori infection, the diagnostic tests to be used, the type of treatment to be given, and how follow-up should be conducted. Children can be tested and treated in both primary and secondary care. A gastroscopy is indicated if the presence of H. pylori in a child has been confirmed by a non-invasive test and two attempts at eradication have failed, and/or there are alarm symptoms that may point to the symptoms having a different underlying cause. A seven-day course of triple eradication therapy can be prescribed. As the level of resistance is low in the Netherlands, this therapy can be used in both the first and second instance without the pattern of resistance being known.

The '5th National Growth Study' indicates that the percentage of overweight children in the Netherlands has risen from 9-12% in 1997 to 13-15% in 2009. Child Health Care is a unique setting for promotion of development, growth and behaviour of children, in which tailored prevention can be offered. Detection of overweight in children and intervention by Child Health Care takes place in a multidisciplinary setting linking general practitioners, paediatricians, dieticians, teachers, physiotherapists, pedagogues and psychologists. For overweight children, a change plan is created based on exercise, playing outside, having breakfast every day, as little as possible sweetened beverages and fast-food, and less time spent in front of the television or computer, with fewer energy-rich snacks. As recommended in the Dutch CBO guideline 'Obesity', obese children are referred to a general practitioner or paediatrician.

Recently the Paediatric Association of the Netherlands (NVK) published a new guideline on the treatment of diabetic ketoacidosis (DKA) and hyperglycaemic hyperosmolar syndrome (HHS) in children and adolescents. DKA comprises hyperglycaemia, ketosis and acidosis. Cerebral oedema is a feared, life-threatening complication of DKA. HHS is characterized by hyperglycaemia, hyperosmolarity, severe dehydration, and little or no ketone production. Multi-organ failure, rhabdomyolysis and thrombosis are the most common complications. The NVK guideline distinguishes between treatment of DKA and treatment of HHS, in contrast with the draft version of the Netherlands Association of Internal Medicine guideline on diabetes. To prevent cerebral oedema in children with DKA, it is necessary that both rehydration and metabolic correction are done slowly and carefully. Use of hypotonic fluids is not recommended. Correction of hyperglycaemia is of secondary importance and insulin should be started at a low dosage. Correction of intravascular hypovolaemia is the most important treatment in children with HHS. If adequate fluid replacement does not cause serum glucose levels to drop sufficiently, then administration of insulin should be considered. Fluid replacement is the initial treatment of HHS. Insulin administration should be considered when serum glucose concentrations are no longer declining adequately with fluid administration alone.

Continence problems can occur during childhood. This guideline is for the Dutch Youth Health Care (JGZ) and gives recommendations for the prevention, early detection and treatment of these problems. As a preventative measure advices for potty training should be started in children aged 18-24 months. If incontinence is present, it is important to take a history and carry out physical examination. In children over the age of 5 who are incontinent of urine the following are recommended: taking child out of bed, calendar with reward system, bedwetting alarm or voiding diary; children over the age of 8 can follow dry bed training. Faecal incontinence is often associated with constipation. Incontinent children with constipation are given advice about normal eating and exercise patterns. If this is not successful then laxatives are prescribed. The JGZ should refer further if there are indications of an underlying condition; if children over the age of 5 are wet during the day; if children are incontinent of faeces at night; if children are incontinent of faeces but not constipated; if children persistently wet the bed; if there is faecal incontinence despite counselling, and if medication needs to be prescribed.

There is a high incidence of skin disorders; these are also frequently encountered within Youth Healthcare (YHC). Some skin disorders are caused by an underlying disease, syndrome or child abuse. Therefore, detection of these causes in an early stage is important. Skin disorders can have a huge psychosocial impact on both child and parents. This is one of the reasons why prevention, detection, diagnosis, treatment, referral, and uniform advice and guidance are of great importance. The YHC Guideline examines counselling and advice, criteria for referral to primary or secondary healthcare, and skincare in general. It also describes the disorders that should be actively detected. The Guideline also looks at specific aspects of dark skins and ethnic diversity, and the impact of skin disorders on general wellbeing. The accompanying web-based tool includes argumentation and opinions from experts on more than 75 skin disorders, including illustrations and decision trees, to aid the drawing up of a treatment plan.

The objective of the evidence-based guidelines 'Asthma in children (ages 0-19)' for youth healthcare (CHC) is the prevention and reduction of asthma symptoms. The guidelines contain a lot of recommendations that apply to all disciplines in healthcare that deal with children. Primary prevention (preventing asthma): the main proven effective intervention is no smoking, neither passive nor active. Breastfeeding has a small protective effect. Starting at the first home visit at two weeks, the CHC nurse advises no smoking and to breastfeed. Secondary prevention (early detection): at all routine check-ups, the professional at CHC should inquire about signs of dyspnoea and wheezing. Patients suspected of having asthma should be referred to the GP. Tertiary prevention (reducing symptoms in children with asthma): the main advice is no smoking (neither passive nor active) and to follow the advice of the treating physician.

In context of the development of evidence-based guidelines by the Dutch Paediatric Society, the three most important controversies in asthma treatment in children were investigated by systematic literature review and assessed based on the quality of evidence according to GRADE (Grading of Recommendations Assessment, Development and Evaluation). Although the quality of evidence was low, this method did result in clear, although weak, recommendations, in which the considerations playing a role are made clear. In young children with severe and/or recurrent wheezing, maintenance treatment using an inhalation corticosteroid (ICS) is initially recommended. When inhalation technique is poor, a leukotriene receptor antagonist (LTRA) can be given instead. Inhalation corticosteroids with ultrafine particles are not specifically recommended as first line treatment in young children. If asthma is not controlled despite use of ICS, it is advised to double the dose of ICS after unfavourable environmental factors have been excluded. If the result is insufficient, a combination of ICS and a long acting bronchodilator is prescribed in children older than 4- 6 years of age.

Accumulating evidence shows that children are not simply little adults, and adapting adult evidence on medical care to children can result in ineffective or even unsafe medical care. The growing number of trials in children reflects this imperative to create a child-specific evidence base. Motivated by the increased focus on clinical research in children and the stark deficiencies in knowledge about the best ways to deal with methodological and practical challenges of research in children, the STaR Child Health initiative was created. It aims to enhance the design, conduct, and reporting of trials in children. The initiative brings together an international group of leading methodologists, clinicians, regulators, funders, and decision-makers to systematically identify what is known, create a research agenda where gaps exist and translate information into practical guidance for end-users. It is critical that funders, researchers, and editors work together for improved conduct and reporting of trials in the next 5-10 years to ensure that the investment in this area will produce the most reliable and relevant results. The first StaR Child Health summit will take place in Amsterdam on the 26th and 27th of October.

In order to reach a consensus concerning diagnosis, support and follow-up in children with an 'apparent life threatening event' (ALTE) multidisciplinary guidelines have been developed by the Dutch Paediatric Association and the Dutch Institute for Healthcare Improvement (CBO). All children presenting as an emergency with ALTE should undergo inpatient clinical observation for 24-72 h, with at least 24 h of cardio-respiratory monitoring. Observation does not need to be continued once an explanation for the incident has been established. The most common causes of ALTE are: gastro-oesophageal reflux (31%), neurological insult (11%) and lower respiratory tract infection (8%). Results from a targeted case history and from physical and neurological examination serve as a guideline for further investigations and support, in accordance with the formulated flowchart. A complete blood-count, CRP- and glucose concentration determination, blood-gas analysis and exploratory urine analysis should be carried out in all infants admitted with an ALTE, and an acute phase urine sample should be collected and frozen for possible metabolic investigation at a later date. Routine monitoring at home is not recommended following an ALTE.

Bilirubin encephalopathy and kernicterus are preventable conditions. Nevertheless cases continue to occur. It is difficult to identify those infants who may develop severe hyperbilirubinemia, because icterus neonatorum occurs in most newborns. The aim of this guideline is to reduce the incidence of severe neonatal hyperbilirubinemia and bilirubin encephalopathy, and at the same time to minimise the risk of unintended side effects. At the initiative of the Dutch Pediatric Association and with methodological support from the Dutch Institute for Healthcare Improvement (CBO), a multidisciplinary working group adapted the clinical practice guideline on hyperbilirubinemia of the American Academy of Pediatrics (AAP) to the Dutch situation. This guideline provides recommendations for the prevention, diagnosis and treatment of hyperbilirubinemia in neonates (>or= 35 weeks). For all newborns a risk assessment for the development of hyperbilirubinemia is made and they are to be systematically assessed during the first week of life. The guideline provides various intervention thresholds for risk groups, recommendations for the use of intravenous immunoglobulin in the event of severe hyperbilirubinemia on the basis of blood group antagonisms, and recommendations for conjugated hyperbilirubinemia. During the transfer of care, information about the risk factors in particular must be satisfactorily passed on.

The new version of the practice guideline 'Children with fever' was recently published by the Dutch College of General Practitioners and is commented on from the perspective of paediatricians. Whether or not general practitioners should refer feverish children to the paediatrician depends on the age of the patient and the severity of the illness. All children younger than 1 month and children with warning symptoms should be referred to a paediatrician at once. One of the warning symptoms is that the child gives the impression of being seriously ill at the physical examination. As it can be difficult to ascertain the severity of the illness and to distinguish which children will develop a serious bacterial infection, observation scales can be of use, as can the general practitioner's intuitive feeling that 'something is wrong'. Emphasis is put on the fact that infections are dynamic processes and therefore good follow-up is important. Children under the age of 2 years with a fever with no apparent cause should be re-examined after 24-48 h. Parents should be properly instructed to come back for re-examination of their child if the clinical picture changes or worsens.

Hypovolaemia is the most common cause of circulatory failure in children. Treatment consists of volume suppletion with a crystalloid or colloid solution; which agent is the best in children is not clear. This evidence-based practice guideline formulates recommendations as to which fluid should be used for volume suppletion in critically-ill neonates and children up to the age of 18 years with hypovolaemia. Before the guideline development first-choice fluid for volume resuscitation was in 50% a colloid and in 50% a crystalloid solution for both neonatologists and paediatric intensivists. The neonatologists used human albumin as a priority, and the paeditric intensivists predominantly used a synthetic colloid. The guideline was developed on the basis of a comprehensive search and analysis of the literature according to the principles of evidence-based guideline development. The recommendations were formulated by a committee based on evidence from the literature and, when evidence from the literature was insufficient, on consensus after discussion in the committee. Since colloids are much more expensive than crystalloids and can give an anaphylactic reaction, their added value over crystalloids must be proven. In sick neonates and children, insufficient clinical trials have been done to reach the conclusion that colloids are more effective than crystalloids in hypovolaemia. A number of meta-analyses in adults revealed excess mortality in the group treated with albumin, but one recent, large, randomised study showed no difference in mortality. No added value could be demonstrated for the administration of synthetic colloids. On the basis of data from the literature and considerations regarding the applicability of evidence in adults to children and neonates, the side effects of resuscitation fluids, pathophysiology and costs, the first-choice fluid for neonates and children with hypovolaemia is isotonic saline. Albumin should not be used for the treatment of hypovolaemia. The volume to be administered and the infusion rate depend on the severity of the hypovolaemia and should be determined on an individual basis.

Three African children who migrated to the Netherlands developed serious infections after they arrived. The first patient, a girl of 3 years adopted from the Zaire, was discovered to be a chronic hepatitis B carrier. Advice to vaccinate her whole adoption family was not followed. Her adoptive mother became infected with hepatitis B. The second patient, an Ethiopian girl of 13 years, was not adequately screened for infections on arrival. She was a chronic hepatitis B carrier and infected her adoptive mother. Seven years later she developed pulmonary tuberculosis with cavity formation and infected four contacts. The third patient, a 15-year-old political refugee from Zaire, who developed paediatric aids and later died of it, was not screened according to the screening protocol advised for immigrating children. All immigrating children should be fully and adequately screened for infectious diseases upon arrival in the Netherlands.

Two hundred Dutch paediatricians were invited to give their description and interpretation of the concept of 'circumoral cyanosis'. Analysis of the results shows great inter-doctor variability: 63% considered the circumoral cyanosis as a pathologic sign and 70% as an expression of central cyanosis, although in textbooks and publications this sign is defined as an expression of peripheral cyanosis and is not considered to be pathological.

The yield of autopsies in a paediatric population. A retrospective analysis is presented of 91 autopsies on children, who died during the period January 1975 and December 1984 in St. Joseph Ziekenhuis, Eindhoven. In 21% of the cases (newborns 23% and older children 13%) the autopsy provided the definitive explanation for the cause of death. In 43% of the cases unexpected findings were obtained during autopsy. In 12% of the cases autopsy could not substantiate a suspected diagnosis and in 12% of the cases the findings at the autopsy influenced genetic counseling. This high rate of return stresses the importance of autopsy as a mean to improve the quality of medical care in a paediatric population.