Ivacaftor and sinonasal pathology in a cystic fibrosis patient with genotype deltaF508/S1215N.

In patients with Cystic Fibrosis and a type III mutation, ivacaftor (Kalydeco(®), Vertex) can increase the opening time of the CFTR channel and improve chloride transport. Research showed significant improvement of lung function and increase in weight following ivacaftor use. However, ivacaftor showed to have adverse events on the sinonasal system as well, such as upper respiratory tract infections, nasal congestion and headaches. This case report showed a positive effect of ivacaftor on the sinonasal pathology in a 17 year old patient with CF. After 5 months of ivacaftor use, the CT-sinus showed complete resolution of the opacification of the paranasal sinuses and a decrease in symptoms of sinonasal disease. This positive effect of ivacaftor on sinonasal pathology seems promising, therefore more research is needed to evaluate the effect of ivacaftor on the upper airways in CF.

[Clinical characteristics and management of adolescents admitted to the emergency ward for alcohol intoxication in the region of The Hague during the period 1999-2001]. / Kliniek en beleid bij jongeren met alcoholintoxicatie op de afdelingen voor spoedeisende hulp in de regio Den Haag, 1999-2000.

OBJECTIVE: To analyse the findings in adolescents between the ages of 12 and 17 who were admitted to the emergency ward as a result of excessive alcohol consumption. DESIGN: Retrospective. METHOD: The clinical characteristics, laboratory findings and treatment policy were studied in adolescents between the ages of 12 and 17 who were admitted for alcohol intoxication (> or = 1/1000) to the emergency wards of Leiden University Medical Centre, Juliana Children's Hospital, Haaglanden Medical Centre (Westeinde location) or the Leyenburg Hospital (all hospitals in the area of The Hague, The Netherlands) during the period 1999-2001. RESULTS: In the three successive years, the number of patients was 19, 18 and 51, respectively; there were a total of 58 boys and 30 girls. Laboratory determinations and other investigations were carried out and/or recorded in varying numbers of patients. The cause of the intoxication was usually the consumption of spirits together with friends; 60% of the patients had a Glasgow coma score < 15 and 30% were admitted to hospital. In 25% the visit to the emergency ward was preceded by an accident in which alcohol played a part. Little attention seems to have been paid to aftercare in the form of a follow-up visit.

[Acute respiratory insufficiency in children]. / Acute respiratoire benauwdheid bij kinderen.

Acute respiratory distress in children is often a consequence of asthma. Other causes are subglottic laryngitis, epiglottitis, aspiration of a foreign body, acute bacterial pneumonia or pneumothorax. History and physical examination should differentiate between the various diseases. Asthma is characterized by recurrent symptoms and signs, while this is not the case with the other causes of acute breathlessness described. An asthma exacerbation is often preceded by one or more prodromes. In case of aspiration of a foreign body, like a peanut, immediate action is needed to prevent irreversible damage to the airways. Subglottic laryngitis and epiglottitis are both characterized by an inspiratory stridor; in case of epiglottitis immediate action is needed, while in case of subglottic laryngitis observation time is available in most cases. Pneumothorax as a cause of acute breathlessness is rare in childhood; it should be considered in male smoking leptosomic asthmatic adolescents.

The discrepancy between daily practice and the policy of a decision-analytic model: the management of fever of unknown origin.

The optimal treatment of children with fever of unknown origin is controversial, in spite of two decision analyses that advise treatment with antibiotics for all such children. The aim of this study was to analyze the differences between pediatricians' diagnostic and therapeutic decisions and the outcomes of the decision-analytic models. Thirty-six pediatricians were asked to evaluate 30 patient cases and to give their diagnostic and therapeutic judgments. In addition, the pediatricians were asked questions about the epidemiology of fever of unknown origin. Analyses showed that the differences in policy between pediatricians and the models could not be explained by the reasons mentioned in the literature, i.e., 1) differences in epidemiologic data used, 2) differences in the weighting of clinical information, and 3) differences in the evaluation of outcomes. The differences in policy might be due to a difference between the objective of the models and pediatricians' aim. In a curative setting, pediatricians are not trying to prevent meningitis (or another serious disease) by treating possible occult bacteremia, but rather aim to detect meningitis in an early stage. A decision analysis determining the most cost-effective strategy for early detection of meningitis might therefore be more easily accepted by pediatricians.

Sleeping position for infants and cot death in The Netherlands 1985-91.

Until the early 1970s the traditional sleeping position for Dutch infants was not prone. After a much publicised lecture in October 1987 on the possible relation between sleeping prone and cot death, the fairly new habit of placing infants prone is being replaced by more traditional positions. The decrease in the prevalence of the prone sleeping position has been documented in six studies. Since 1987 the incidence of registered cot deaths has decreased from 1.04/1000 live births in 1986 to 0.44 in 1991; the real decrease of sudden unexpected death in infancy, however, is greater.