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Background: No studies have been performed in which therapeutic regimens have been compared between mild and moderate-to-severe pediatric Crohn's disease (CD) at diagnosis. The aim was to analyze pediatric CD activity at diagnosis, its influence on pediatrician's prescribing behavior, and clinical outcome 5 years later., Methods: In a retrospective multicenter study we divided pediatric CD patients at diagnosis into mild or moderate-severe disease. We compared initial therapies, duration of first remission, number of exacerbations, height-for-age and weight-for-height evolvement, and cumulative duration of systemic steroid use in a 5-year follow-up period., Results: Forty-three children were included (25 with mild and 18 with moderate-severe disease). Aminosalicylate monotherapy was more frequently prescribed in the mild group (40% versus 17%; P < 0.01). The median duration of systemic steroid use was 18.3 months in the mild group and 10.4 months in the moderate-severe group (P = 0.09). Duration of first remission was 15.0 months in the mild group and 23.4 months in the moderate-severe group (P = 0.16). The mean number of exacerbations was 2.2 in the mild group and 1.8 in the moderate-severe group (P = 0.28)., Conclusions: CD patients with mild disease were treated with aminosalicylate monotherapy more frequently. These patients, however, tend to have more exacerbations, shorter duration of first remission, and longer total duration of systemic steroid use. Our data support the concept that severity of disease at diagnosis does not reliably predict subsequent clinical course. This study suggests that there is no indication that children with mild CD should be treated differently compared to children with moderate-severe disease.

Anaemia was diagnosed in four adopted children during a standard screening examination 1-4 weeks after arrival. Further investigation revealed a number of causes which could then be specifically treated. The children were a girl aged 14 months from China with iron-deficiency anaemia, a boy aged 16 months from Nigeria with sickle cell anaemia, a girl aged 5 from Haiti who had alpha-thalassaemia, and a boy aged 7 from Brazil with spherocytosis. Iron deficiency is the most common cause of anaemia in childhood. However, in adopted children from sub-tropical areas other causes of anaemia like haemoglobinopathies or erythrocyte membrane defects should be borne in mind, particularly as a history of disease and family history are often lacking. Additional investigations may be necessary. An incorrect diagnosis of iron deficiency may result in ongoing and unjustified iron supplementation leading to harmful iron accumulation in thalassaemia and a delay in the correct treatment in sickle cell anemia or spherocytosis which could carry considerable risk.

A 17-year-old, previously healthy boy was admitted with complaints of fever, malaise and pain in the pubic region and groin. His left elbow was also warm and swollen and could not be extended fully. Cultures of fluid drained from both hips and the elbow, blood and inflamed tissue from the nail bed of the right big toe yielded Staphylococcus aureus. The patient was treated with intravenous antibiotics for septic arthritis of both hips, the left elbow and possibly the pubic symphysis. The infected nail bed, which was the most likely port of entry, was also treated. One year later, the original painful symptoms had disappeared but the right hip was almost immobile. In a patient with indications of septic arthritis, timely diagnosis and adequate therapy reduce the risk of permanent damage to the joint.

It is unclear whether cyclosporine A (CsA) can be withdrawn safely during follow-up after pediatric liver transplantation. In our transplant program we have been using a strict protocol to withdraw CsA. The aim of this study was to retrospectively assess the effects of CsA withdrawal after pediatric liver transplantation on the incidence of rejection and renal function. Between 1986 and 2001, 91 children received CsA for at least 2 yr after liver transplantation. Specific criteria for eligibility to withdraw CsA were set. In 53 of the 91 children CsA was withdrawn. In 35 patients (66%) withdrawal of CsA did not cause rejection. In these patients the renal function improved compared with baseline values (glomerular filtration rate (GFR) at 1 yr, +16 mL/minute/1.73 m3, P < 0.001; at 2 yr, +10 mL/minute/1.73 m3, P < 0.05). After CsA withdrawal, 18 patients developed rejection (34%), which could be effectively treated by methylprednisolone and restarting CsA. Failure to withdraw CsA was not associated with increased incidence of graft loss. A body weight below 10 kg at the time of transplantation correlated significantly with successful withdrawal of CsA (<10 kg, 85% vs. > 10 kg, 60%; P < 0.05). In conclusion CsA can successfully be withdrawn in a major proportion of selected pediatric liver transplantation patients during follow-up. The success rate is the highest in children with a body weight below 10 kg at the time of transplantation. Successful withdrawal improves renal function, whereas failure to withdraw is not associated with graft loss or persisting morbidity., (Copyright 2006 AASLD)

Background: Guidelines for asthma management focus on treatment with inhaled corticosteroids and on home recording of peak expiratory flow (PEF). The effect of maintenance treatment with inhaled corticosteroids on PEF variation and its relation to other parameters of disease activity were examined in 102 asthmatic children aged 7-14 years., Methods: During 20 months of treatment with inhaled salbutamol, with or without inhaled budesonide (600 micrograms daily), forced expiratory volume in one second (FEV1), the dose of histamine required to provoke a fall in FEV1 of more than 20% (PD20), the percentage of symptom free days, and PEF variation were assessed bimonthly. PEF variation was computed as the lowest PEF as a percentage of the highest PEF occurring over 14 days, the usual way of expressing PEF variation in asthma self-management plans. For each patient using inhaled corticosteroids within subject correlation coefficients (rho) were computed of PEF variation to the percentage of symptom free days, FEV1, and PD20., Results: PEF variation decreased significantly during the first two months of treatment with inhaled corticosteroids and then remained stable. The same pattern was observed for symptoms and FEV1. In contrast, PD20 histamine continued to improve throughout the whole follow up period. In individual patients predominantly positive associations of PEF variation with symptoms, FEV1, and PD20 were found, but the ranges of these associations were wide., Conclusions: During treatment with inhaled corticosteroids the changes in PEF variation over time show poor concordance with changes in other parameters of asthma severity. When only PEF is monitored, clinically relevant deteriorations in symptoms, FEV1, or PD20 may be missed. This suggests that home recording of PEF alone may not be sufficient to monitor asthma severity reliably in children.

This study examined whether correction of peak expiratory flow (PEF) values for the inaccuracy of the meter would affect asthma management in 102 children (7-14 y old). PEF was recorded with a mini Wright meter twice daily for 2 weeks. As expected, measured PEF overestimated PEF level and asthma control in these children on many diary days. The actual numerical differences between measured and corrected PEF on these days were very small (>5% in only five patients, maximum 10%). It is unlikely that such small changes in PEF justify changes in asthma management, even if these changes cause PEF levels to cross arbitrary borders between various levels of asthma control used in self-management plans. The clinical importance of the inaccuracy of portable PEF meters is negligible.

Although home recording of peak expiratory flow (PEF) is considered useful in managing asthma, little is known about the relationship of PEF variation to other indicators of disease activity. We examined the relationship of PEF variation, expressed in various ways, to symptoms, atopy, level of lung function, and airways hyperresponsiveness in schoolchildren with asthma. One hundred and two asthmatic children (aged 7-14 yrs) recorded symptoms and PEF (twice daily) in a diary for 2 weeks after withdrawal of all anti-inflammatory maintenance medication. PEF variation was expressed as amplitude % mean, as standard deviation and coefficient of variation of all recordings, and as low % best (lowest PEF as percentage of the highest of all values). Atopy and level of forced expiratory volume in one second (FEV1) % predicted were not significantly related to PEF variation. The provocative dose of histamine causing a 20% fall in FEV1 (PD20) and symptom scores were significantly, but weakly, related to PEF variation. The index, low % best, proved easy to calculate and effective in identifying a short-term episode of reduced PEF. We conclude that peak expiratory flow variation in children with stable, moderately severe asthma is significantly, but weakly, related to symptoms and airways hyperresponsiveness. These three phenomena, therefore, all provide different information on the actual disease state. Expressing peak expiratory flow variation as low % best is easy to perform and appears to be clinically relevant.

Inhaled corticosteroid has been shown to be effective in the management of asthma. However, there is a lack of studies that assess the effect of cessation after long-term treatment with inhaled corticosteroid. This question was addressed in 28 children with stable asthma, aged 11 to 18 yr of age, who had completed 28 to 36 months of treatment with inhaled corticosteroid (budesonide 200 micrograms 3 times/day) and inhaled beta-2-agonist (salbutamol 200 micrograms 3 times/day). The children were randomized in a 1:2 ratio in a double-blind study either to continue budesonide (n = 8) during a period of 6 months or to decrease the dose of budesonide (n = 20) within 2 months, followed by placebo for 4 months. Treatment with salbutamol 600 micrograms daily was continued in both groups. Eight children from the tapering-off group withdrew, mainly due to symptoms of asthma, compared with none in the continuous treatment group. Five patients in the tapering-off group experienced exacerbations for which prednisolone was given, compared with none in the continuous treatment group. After tapering-off, symptoms of asthma and additional bronchodilator use increased, and both FEV1% predicted and PD20 histamine (provocation dose of histamine causing a 20% fall in FEV1) decreased, whereas these all remained unchanged in the group that continued treatment with inhaled corticosteroid. We conclude that in this study long-term treatment with 600 micrograms budesonide daily suppressed underlying mechanisms of asthma, but did not cure the disease.

The bronchodilator response (BDR) in forced expiratory volume in one second (FEV1) is routinely assessed to estimate the reversibility of airways obstruction. However, there is no consensus on how the BDR should be expressed, and recommendations applying to children are lacking. Similarly, the relationship between BDR and nonspecific bronchial hyperresponsiveness to histamine (BHR) has not been elucidated. These questions were addressed in 116 children, 7-16 yrs of age, with stable asthma after withdrawal of all pulmonary maintenance medication. Inclusion criteria were an initial FEV1 between 55-90% predicted, and/or FEV1/forced vital capacity (FVC) between 50-75%, as well as a fall in FEV1 of 20% or more when challenged with up to 150 micrograms histamine. The change in FEV1 (delta FEV1) 20 min after inhalation of 800 micrograms salbutamol was expressed in four ways: as an absolute difference (delta FEV1(l)), as a percentage of predicted FEV1 (delta FEV1%pred) or initial FEV1 (delta FEV1%init), and as a percentage of the deficit in FEV1 (delta FEV1%(pred-init)). delta FEV1%init and delta FEV1%pred were not related to age and stature of the children; delta FEV1%(pred-init) was related to stature, whilst delta FEV1(l) was related to both age and stature. All indices correlated with initial FEV1. However, this is an artefact introduced by relating change to initial value, rather than to the mean of initial and final value. In fact, BDR, expressed as delta FEV1%pred, was only slightly greater in children with the lowest initial airway calibre (p = 0.08), unlike delta FEV1%init. BDR was weakly related to BHR.(ABSTRACT TRUNCATED AT 250 WORDS)

In a randomized double-blind multicenter clinical study, 116 children with asthma were randomly assigned to treatment with an inhaled beta-2-agonist (salbutamol 0.2 mg) plus an inhaled corticosteroid (budesonide 0.2 mg) three times a day (BA+CS) or to an inhaled beta-2-agonist (salbutamol 0.2 mg) plus a placebo three times a day (BA+PL). After a median follow-up time of 22 months, 26 patients receiving BA+PL (45%) had withdrawn from randomized treatment, mainly because of asthma symptoms, compared with three withdrawals in the patients receiving BA+CS (p less than 0.0001). The FEV1, expressed as a percentage of the predicted value for age, sex, and height, showed an absolute increase of 7.0% after 2 months of BA+CS compared with a decrease of 4.0% after 2 months of BA+PL. This 11% difference in percent predicted FEV1 (95% confidence interval, 7 to 15%; p less than 0.0001) was then maintained after a median follow-up period of 22 months. Postbronchodilator FEV1 showed an absolute increase of 3.7% predicted within 2 months in patients receiving BA+CS and an absolute decrease of 1.1% predicted in children receiving BA+PL (p = 0.0005). Thereafter, this difference between the two treatment groups was maintained. Average peak expiratory flow rate (PEFR) increased from baseline by 36.6 L/min in the BA+CS group compared with 3.7 L/min in the BA+PL group (p = 0.003). This difference then remained for the median follow-up time of 22 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Smoking habits and attitudes towards smoking of medical students (n = 725), house officers (n = 126) and consultants (n = 236) of the University Hospital of Groningen were studied, in 1989 by means of a World Health Organization (WHO) questionnaire. Overall response rate was 84%. Twenty seven percent of the medical students are current smokers, 28% of the house officers and 34% of the consultants. There is a remarkable difference among medical specialists i.e: smoking prevalence is highest among psychiatrists and lowest among paediatricians. The prevalence of smoking in medical students and house officers is lower than in the Dutch population. Smoking habits of the consultants are similar to those of the general population. About 75% of the doctors reported having no experience with smoking cessation programmes. Doctors report a need for more skills and knowledge on smoking cessation programmes.

The effects of treatment with budesonide (200 micrograms twice daily) and terbutaline (500 micrograms four times daily) has been compared with the effects of placebo and terbutaline in 27 children with mild asthma, aged 7-14 years, in a double blind, randomised placebo controlled study over eight weeks. Bronchial responsiveness (PC20 histamine), lung function, the amplitude of diurnal variation in peak expiratory flow (PEF), and symptom scores were measured. Baseline FEV1 was over 70% predicted and PC20 histamine less than 8 mg/ml. Twelve children were treated with budesonide and terbutaline and 15 with placebo and terbutaline. After four and eight weeks of treatment the change in PC20 was significantly greater after budesonide and terbutaline than after terbutaline alone by 2.1 (95% CI 0.5-3.8) and 1.3 (95% CI 0.1-2.5) doubling doses respectively. Mean FEV1 did not change in either group. The change in afternoon and nocturnal PEF was significantly greater after budesonide and terbutaline than after terbutaline alone. The amplitude of diurnal variation in PEF did not change significantly in either group. Peak flow reversibility decreased in the budesonide group. There were no differences between treatments for cough and dyspnoea, but wheeze improved in the budesonide group. The children with mild asthma treated with budesonide and terbutaline showed improvement in bronchial responsiveness, afternoon and nocturnal PEF, and symptoms of wheeze and a fall in peak flow reversibility by comparison with those who received terbutaline alone.

Smoking habits and attitudes towards smoking of medical students (n = 725), house officers (n = 126) and consultants (n = 236) of the University Hospital of Groningen were studied in 1989 using a WHO questionnaire. Overall response was 84%. Twenty-seven percent of the medical students, 28% of the house officers and 34% of the consultants are current smokers. The prevalence of smoking in medical students and house officers is lower than that in the Dutch population. Smoking habits of consultants are similar to the general population. The prevalence of smoking Dutch physicians which was 56% in 1983 is decreasing rapidly. Doctors report they want more information about smoking cessation programs.

Nocturnal airflow obstruction is a common symptom in children with asthma. The increase in airflow obstruction overnight is underassessed by children as well as their parents. Worsening of the early morning peak flow values may be an indication for an increase in airflow obstruction overnight. An increase in vagal activity does not, in contrast with the observations in adult patients, contribute to the development of nocturnal airflow obstruction in asthmatic children. However, a nocturnal increase in inflammatory mediators seems to play a role of importance. Treatment of nocturnal airflow obstruction should therefore be focussed on inflammatory processes in the lungs.

Stridor is the presenting symptom of abnormalities of larynx and trachea in children. The anatomical differences in size and consistency of the larynx play a crucial role in stridor in children as compared with adults. From 1983 to 1988, 81 children (65 males, 16 females) with severe or prolonged stridor were admitted to our hospital. Thorough examination, including endoscopy of larynx and trachea and radiology revealed various congenital abnormalities and acquired conditions as the cause of stridor. Congenital laryngeal anomalies accounted for stridor in 26 children, there were 12 children with congenital tracheal or vascular anomalies, 28 with infectious diseases and 15 with other diseases. Intubation and (or) tracheotomy were performed in one-third of the patients: 5 of them died of severe complications. For proper treatment of children with stridor it is essential to investigate its aetiology carefully.