Oostenbrink, R.

Can urgency classification of the Manchester triage system predict serious bacterial infections in febrile children? (Article)

2011-08-01T00:00:00Z

Objective: To evaluate the discriminative ability of the Manchester triage system (MTS) to identify serious bacterial infections (SBIs) in children with fever in the emergency department (ED) and to study the association between predictors of SBI and discriminators of MTS urgency of care. Methods: This prospective observational study included 1255 children with fever (1 month-16 years) attending the ED of the Erasmus MC - Sophia Children's Hospital, Rotterdam, The Netherlands in 2008-9. Triage urgency was determined with the MTS (urgency (U) level 1-5). The relationship between triage urgency and SBI was assessed with multivariable logistic regression, including effects of age, sex and temperature. Discriminative ability was assessed by receiver operating characteristic curve analysis. Results: SBI prevalence was 11% (n = 131, 95% CI 9% to 12%). The discriminative value of the MTS for predicting SBI was 0.57 (95% CI 0.52 to 0.62), and the MTS did not contribute to a model including age, sex and temperature. The sensitivity of the MTS (U1-2 vs U3-5) to detect SBI was 0.42 (95% CI 0.33 to 0.51) and specificity was 0.69 (95% CI 0.66 to 0.72). MTS high urgency discriminators include several known predictors of SBI, such as fever, work of breathing, meningism and oxygen saturation, but apply to non-SBI children as well. Conclusion: The MTS has poor discriminative ability to predict the presence of SBIs in children presenting with fever to the paediatric ED. Important predictors of SBI are represented within the MTS, but are used in a different way to classify urgency.

Health-related quality of life in preschool children in five health conditions (Article)

2011-06-01T00:00:00Z

Objective: To test the responsiveness of the Infant/Toddler Quality of Life Questionnaire (ITQOL) to five health conditions. In addition, to evaluate the impact of the child's age and gender on the ITQOL domain scores. Methods: Observational study of 494 Dutch preschool-aged children with five clinical conditions and 410 healthy preschool children randomly sampled from the general population. The clinical conditions included neurofibromatosis type 1, wheezing illness, bronchiolitis, functional abdominal complaints, and burns. Health-related quality of life (HRQoL) was assessed by a mailed parent-completed ITQOL. Mean ITQOL scale scores for all conditions were compared with scores obtained from the reference sample. The effect of patient's age and gender on ITQOL scores was assessed using multi-variable regression analysis. Results: In all health conditions, substantially lower scores were found for several ITQOL scales. The conditions had a variable effect on the type of ITQOL domains and a different magnitude of effect. Scores for 'physical functioning', 'bodily pain', and 'general health perceptions' showed the greatest range. Parental impact scales were equally affected by all conditions. In addition to disease type, the child's age and gender had an impact on HRQoL. Conclusions: The five health conditions (each with a distinct clinical profile) affected the ITQOL scales differently. These results indicate that the ITQOL is sensitive to specific characteristics and symptom expression of the childhood health conditions investigated. This insight into the sensitivity of the ITQOL to health conditions with different symptom expression may help in the interpretation of HRQoL results in future applications.

Impact of von Willebrand disease on health-related quality of life in a pediatric population (Article)

2011-03-01T00:00:00Z

Background: Von Willebrand disease (VWD) is the most frequent inherited bleeding disorder. Whether VWD is associated with health-related quality of life (HR-QoL) in children is unknown. Objectives: This nationwide cross-sectional study measured HR-QoL in children with moderate or severe VWD. Our primary aim was to compare HR-QoL of VWD patients with that of reference populations. Additionally, we studied the impact of bleeding phenotype and VWD type on HR-QoL. Methods: HR-QoL was assessed with the Infant/Toddler QoL Questionnaire (0-5 years) and Child Health Questionnaire (6-15 years), and compared with reference population scores. Multivariate analysis was used to evaluate the influence of type of VWD and bleeding phenotype on HR-QoL scores. Results: Preschool children (0-5 years, n=46) with VWD had lower HR-QoL scores for general health perceptions and parental time than reference populations. School children (6-15 years, n=87) with VWD had lower scores for physical functioning, role functioning - emotional/behavioral, general health perceptions, and physical summary. Type of VWD was associated with HR-QoL in school children for bodily pain, general health perceptions, parental emotion, family activities, and physical summary. Scores of children with type 3 VWD were, on average, 15 points lower than those of the reference population on the above-mentioned scales. A more severe bleeding phenotype was associated with a lower score on 11/15 physical, emotional and social scales. Conclusion: HR-QoL is lower in VWD children than in reference populations, in particular in school children. The negative impact of VWD is sensitive to type of VWD and bleeding phenotype; as well as physical scales, emotional and social scales are affected.

Legius syndrome in fourteen families (Article)

2011-01-01T00:00:00Z

Legius syndrome presents as an autosomal dominant condition characterized by café-au-lait macules with or without freckling and sometimes a Noonan-like appearance and/or learning difficulties. It is caused by germline loss-of-function SPRED1 mutations and is a member of the RAS-MAPK pathway syndromes. Most mutations result in a truncated protein and only a few inactivating missense mutations have been reported. Since only a limited number of patients has been reported up until now, the full clinical and mutational spectrum is still unknown. We report mutation data and clinical details in fourteen new families with Legius syndrome. Six novel germline mutations are described. The Trp31Cys mutation is a new pathogenic SPRED1 missense mutation. Clinical details in the 14 families confirmed the absence of neurofibromas, and Lisch nodules, and the absence of a high prevalence of central nervous system tumors. We report white matter T2 hyperintensities on brain MRI scans in 2 patients and a potential association between postaxial polydactyly and Legius syndrome.

KBG syndrome associated with periventricular nodular heterotopia (Article)

2010-07-01T00:00:00Z

Parental fever attitude and management: Influence of parental ethnicity and child's age (Article)

2010-05-01T00:00:00Z

Objective: The objective was to study parental fever management and attitude toward fever from the perspective of the child's ethnicity and age. Patients and setting:: Children with fever presenting at the pediatric emergency department (PED) of the Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands, in the period from February 2002 to March 2004. DESIGN:: Prospective observational. MAIN OUTCOME MEASURES:: Parental fever attitude and management assessed by a questionnaire. Results: Two hundred eleven children with fever (median age, 1.2 years; interquartile range, 0.7-2.0 years) were included, of whom 108 (55%) were boys. One hundred fourteen children (54%) were self-referrals at the PED. Accompanying symptoms were reported in 95% (50% had ≥3); median temperature measured at PED was 39.5°C (interquartile range, 38.9°C-40.8°C). One hundred fifty-five parents (74%) had used antipyretics to reduce fever, and 155 parents (74%) were worried about fever and its possible complications. Differences between Dutch and non-Dutch ethnicities were seen in temperature-reducing techniques, self-referral, and parental anxiety of fever and its complications. Age did not influence parental fever attitude and management. Conclusions: For most children in our population, the use of antipyretics was justified, as the majority of our children visiting the PED for an acute febrile episode are young infants, in particular with a high degree of fever and accompanying symptoms. We confirm and extend previous findings of ethnicity influencing parental fever management.

Functional abdominal complaints in pre-school children: Parental reports of health-related quality of life (Article)

2010-04-01T00:00:00Z

Purpose The aim of this study is to assess the influence of functional abdominal complaints (FAC) on health-related quality of life in a group of Dutch pre-school children. Methods Parents of children aged up to 6.0 visiting the outpatient pediatric department, Erasmus MC-Sophia, Rotterdam, The Netherlands in the period January 2005-December 2006 for functional abdominal complaints during at least 3 months were asked to complete the Infant/Toddler Quality of life Questionnaire (ITQOL), and questions of the abdominal pain index for use by parents to report pain symptoms in pre-school children. ITQOL scale scores of children with FAC were compared against with Dutch reference values. The abdominal pain index was tested for internal consistency and test-retest reliability. Correlations between ITQOL scale scores and abdominal pain index were assessed by Spearman's rank test. Results Results are based on 81 questionnaires completed by parents of children with FAC (response rate 61%). Children had a median age of 46 months (interquartile range 27-59), 48% girls. A significant impact was observed on most aspects of quality of life, particularly for physical functioning, general development, bodily pain, temperament and moods, general health perceptions and parental emotional impact. Parents of children with functional constipation tended to report lower scores than those of children with other FAC. The abdominal pain index appeared to be valid and was significantly correlated with ITQOL scales bodily pain and general health perceptions. Conclusions A substantial lower health-related quality of life is reported in pre-school children with functional abdominal complaints, with effects on physical, emotional and parental domains. The 5-question severity index of abdominal pain appeared a valid tool and may be helpful to quickly assess the severity of abdominal pain in clinical practice.

Reply (Article)

2009-09-01T00:00:00Z

Health-Related Quality of Life in Children with Neurofibromatosis Type 1: Contribution of Demographic Factors, Disease-Related Factors, and Behavior (Article)

2009-03-01T00:00:00Z

Objective: To investigate health-related quality of life (HR-QOL) in children with neurofibromatosis type 1 (NF1) with parental reports and children's self-reports, and to investigate the potential contribution of demographic factors, disease-specific factors, and problems in school performance or behavior. Study design: In a prospective observational study, parents of 58 children with NF1 (32 boys, 26 girls, age 12.2 ± 2.5 years) visiting a university clinic, and their 43 children 10 years or older were assessed with the Child Health Questionnaire (CHQ). Potential determinants of domain scores were assessed in 3 explorative regression models. Results: Parents reported a significant impact of NF1 on 9/13 CHQ scales, with moderate effect sizes on 8 (general health perceptions, physical functioning, general behavior, mental health, self esteem, family activities, role functioning emotional/behavioral, and parent emotional impact). Children report an impact on bodily pain, and an above average general behavior. Multiple CHQ scales were sensitive to demographic factors and behavioral problems, and 1 to NF1 severity. NF1 visibility and school problems did not influence HR-QOL. Conclusions: Parents, but not the children with NF1, report a profound impact of NF1 on physical, social, behavioral, and emotional aspects of HR-QOL. Multiple HR-QOL domains were most sensitive to behavioral problems, which points to an exciting potential opportunity to improve HR-QOL in children with NF1 by addressing these behavioral problems.

Parental Reports of Health-Related Quality Of Life in Young Children with Neurofibromatosis Type 1: Influence of Condition Specific Determinants (Article)

2007-08-01T00:00:00Z

Objective: To assess the health-related quality of life of 34 Dutch children 12 to 72 months of age with neurofibromatosis type 1 (NF1) using the Infant/Toddler Quality of Life Questionnaire (ITQOL) and to investigate the potential impact of clinical factors on parental reports of health-related quality of life. Study design: A parent-completed form including the ITQOL, NF1-specific questions, and sociodemographic questions was sent. ITQOL scale scores were compared for the study population against Dutch reference values. The influence of general and clinical characteristics on ITQOL scale scores was evaluated with multivariate analysis. Results: A significant impact was observed on most aspects of quality of life, particularly for growth and development, general health perceptions and parental impact. The lowest scores were observed in children with complications because of NF1. ITQOL scale scores were affected by parental educational level, familial NF1, and parental reports of complications of NF1 and perceived disease severity. Conclusions: Important aspects of health-related quality of life were observed to be negatively affected in children with NF1, as measured by the ITQOL. Family-related and disease-related variables appeared to influence the quality of life in children with NF1.

Reliability and validity of the Infant and Toddler Quality of Life Questionnaire (ITQOL) in a general population and respiratory disease sample (Article)

2007-04-01T00:00:00Z

Objective: To evaluate feasibility, internal consistency, test-retest reliability, and concurrent and discriminative validity of the Infant and Toddler Quality of Life Questionnaire (ITQOL) for parents of pre-school children with 12 scales (103-items) covering physical and psychosocial domains and impact of child health on parents, in comparison with the TNO-AZL Pre-school Children Quality of Life Questionnaire (TAPQOL). Methods: Parents of children from a random general population sample (2 months-4 years old; n = 500) and of an outpatient clinic sample of children with respiratory disease (5 months-51/2 years old; n = 217) were mailed ITQOL and TAPQOL questionnaires; a retest was sent after two weeks. Results: Feasibility: The response was ≥80% with few missing and non-unique ITQOL-answers (<2%) in both study populations. Some ITQOL-scales (3-4 scales) showed a ceiling effect (>25% at maximum score). Internal consistency: All Cronbach's α >0.70. Test-retest Intraclass Correlation Coefficients (ICCs) were moderate or adequate (≥0.50; p < 0.01) for 10 ITQOL-scales. Validity: ITQOL-scales, with a few exceptions, correlated better with predefined parallel TAPQOL scales than with non-parallel scales. Five to eight ITQOL-scales discriminated clearly between children with few and with many parent-reported chronic conditions, between children with and without doctor-diagnosed respiratory disease and with a low and a high parent-reported medical consumption (p < 0.05). Conclusions: This study supported the evidence that the ITQOL is a feasible instrument with adequate psychometric properties. The study provided reference ITQOL scores for gender/age subgroups. We recommend repeated evaluations of the ITQOL in varied populations, especially among very young children, including repeated assessments of test-retest characteristics and evaluations of responsiveness to change. We recommend developing and evaluating a shortened ITQOL version.

Children with meningeal signs: predicting who needs empiric antibiotic treatment (Article)

2002-01-01T00:00:00Z

BACKGROUND: Since delayed diagnosis and treatment of bacterial meningitis worsens patient prognosis, clinicians have a low threshold to perform a lumbar puncture or to start empiric antibiotic treatment in patients suspected of having meningitis. OBJECTIVE: To develop a decision rule, including cerebrospinal fluid (CSF) indices and clinical characteristics, to determine whether empiric antibiotic treatment should be started in children with meningeal signs. DESIGN: Multivariable logistic regression analysis of retrospectively collected data. Bacterial meningitis was defined as a CSF leukocyte count of more than 5/ micro L with positive bacterial culture findings from CSF or blood specimens. SETTING: Pediatric emergency department of a pediatric university hospital. PATIENTS: A total of 227 children (aged 1 month to 15 years) with meningeal signs. MAIN OUTCOME MEASURE: The diagnostic value of adding early obtainable CSF indices to clinical characteristics to predict bacterial meningitis. RESULTS: Independent predictors of bacterial meningitis from early obtainable CSF indices were the CSF polymorphonuclear leukocyte count and the CSF-blood glucose ratio. The diagnostic value (area under the receiver operating characteristic curve) of this CSF model was 0.93. Application of the model together with clinical characteristics could predict early the absence of bacterial meningitis in 69 (30%) of the 227 patients so that empiric antibiotic treatment could be safely withheld. CONCLUSION: A diagnostic decision rule that uses clinical characteristics at admission, the CSF polymorphonuclear leukocyte count, and the CSF-blood glucose ratio is a useful tool for deciding whether to start empiric antibiotics in children with meningeal signs.

Diagnostic and prognostic research in paediatrics: Children with meningeal signs (Doctoral Thesis)

2001-06-13T00:00:00Z

Meningeal signs are indicative of bacterial meningitis and therefore necessitate a full diagnostic work-up, although these signs are not pathognomonic. Meningeal signs are present in 50 - 70% of children older than one with bacterial meningitis, as well as in 25 % of the children without meningitis in whom a lumbar puncture is performed. In about 60% of children who have meningeal signs, another diagnosis may be assessed.