bluuebunny/crossref_metadata_2025_split huggingface dataset:
Children with early refusal to eat: Follow‐up in adolescence
Authors: Rydell Ann‐Margret , Dahl Margareta 
Published: August 2010

Abstract Aim: To describe the adolescent outcome of infant refusal to eat (RTE) and to investigate early predictors of adolescent outcomes. Methods: 19 of 25 children with RTE and 35 of 42 controls were assessed at age 16. The participants rated eating attitudes and self‐perceptions with a Swedish version of the Children's Eating Attitudes Test (ChEAT) and a Swedish self‐perception measure. Weight and height were assessed through self‐reports, and body mass index (BMI) was computed. Child and family variables measured from infancy through age 4 were used as predictors. Results: RTE and controls did not differ with regard to disturbed eating attitudes, BMI or self‐perceptions. There were no relations between early predictors and adolescent disturbed eating for the whole sample, but breastfeeding problems and social adversity in the early years predicted negative self‐perceptions, and weight at 4 y predicted adolescent BMI. For the RTE group, low birthweight predicted higher levels of disturbed eating attitudes and less positive self‐perceptions. For the control group, social adversity predicted high BMI. Conclusion: RTE does not seem to constitute a risk factor for adolescent disturbed eating. Further, there may be different risk factors for adolescent disturbed eating for children with and without RTE.
Behavioural effects of phenylalanine‐free amino acid tablet supplementation in intellectually disabled adults with untreated phenylketonuria
Authors: Kalkanoğlu H. Serap , Ahring Kirsten K. , Sertkaya Durdu , Birk Møller Lisbeth , Romstad Anne , Mikkelsen Ingrid , Guldberg Per , Lou Hans C. , Güttler Flemming 
Published: August 2010

Abstract Aim: To evaluate the effects of phenylalanine (Phe)‐free essential amino acid (AA) tablets enriched in tyrosine and tryptophan on the performance of intellectually disabled adult patients with untreated phenylketonuria (PKU). Methods: Phe‐free AA tablets and placebo tablets were administered to 19 untreated PKU subjects on a normal diet for 6 mo in a prospective double‐blinded crossover study. The adaptive behaviour of the patients was tested prior to the study and at 6 and 12 mo after the start, using a simplified version of the Vineland Adaptive Behaviour Scale. For each sub‐domain, the patients were rated either “0” (for poor performance) or “1” (for good performance). Neurological signs and symptoms and specific behavioural characteristics were recorded monthly by caretakers. Every 6 mo, neurological examination of the patients was performed, and the caretakers were interviewed. The statistical significance of the results was tested by means of the Fisher's exact and Wilcoxon tests. Results: The most significant changes were an improved concentration and the development of a meaningful smile, which were observed in 44% and 43% of the patients on AA tablet treatment, respectively, but not patients on placebo. Other important but less significant changes included increased awareness of external stimuli (63%) and less self‐injury (43%), and 40% were smiling and laughing occasionally. The mean overall rating increased from an initial value of 6.3 to 10.1 in patients when on AA tablet treatment (p=0.002), and to 7.0 in patients when on placebo (p=0.068). The difference between active AA treatment and placebo was statistically significant (p=0.027). Conclusions: This pilot study suggests that Phe‐free AA tablets enriched in tyrosine and tryptophan may improve the quality of life in some intellectually disabled adults with untreated PKU.
Autonomic nervous system functions in children with breath‐holding spells and effects of iron deficiency
Authors: Kolkiran Abdülkerim , Tutar Ercan , Atalay Semra , Deda Gülhis , Cin Şükrü 
Published: August 2010

Abstract Aim: To analyse the activity of the autonomic nervous system during breath‐holding spells, we assessed the ECG changes, including ventricular repolarization parameters before and during the spell. We also analysed the effects of iron deficiency on these ECG parameters. Methods: The study group consisted of 37 children with breath‐holding spells (30 cyanotic, 7 pallid) (mean age±SD: 12.9±10.8 mo). Twenty‐six healthy children (mean age±SD: 14.4±8.6 mo) served as a control group. All patients and controls had standard 12‐lead simultaneous surface ECG. All patients had ECG recordings during at least one severe breath‐holding spell obtained by “event recorder”. Traces obtained by “event recorder” were analysed in terms of mean heart rate and the frequency and duration of asystole during the spell. Results: Respiratory sinus arrhythmia on standard ECGs and asystole frequency during spells were higher in patients with pallid breath‐holding spells. Patients with iron deficiency had a lower frequency of respiratory sinus arrhythmia and prolonged asystole time during the spell. There was no difference in terms of ventricular repolarization parameters (QT/QTc intervals and QT/QTc dispersions) between patients and controls and between patient subgroups (cyanotic versus pallid). Conclusion: These results confirmed the presence of autonomic dysregulation in children with breath‐holding spells. Iron deficiency may have an impact on this autonomic dysregulation. Ventricular repolarization was unaffected in patients with breath‐holding spells.
Improved final height with long‐term growth hormone treatment in Noonan syndrome
Authors: Osio Deborah , Dahlgren Jovanna , Wikland Kerstin Albertsson , Westphal Otto 
Published: August 2010

Abstract Aim: To assess whether children with Noonan syndrome on long‐term growth hormone (GH) therapy improve their final height to near mid‐parental height. Methods: Twenty‐five prepubertal children (13 girls) with Noonan syndrome (NS) were studied. A single clinician made the diagnosis based on clinical criteria. GH treatment started at an age ranging from 3.1 to 13.8 y and was continued for at least 2 y. Improvement or “gain” in final height (FH) was defined as either the difference between adult height SD scores (SDS) and pre‐treatment height SDS (the childhood component of the Swedish reference) or height SDS compared to the Noonan reference. Results: Ten children received a GH dose of 33 μg/kg/d (mean age at start 7.7±2.1 y, mean age at stop 17.6±1.7 y) and 15 received a dose of 66 μg/kg/d (mean age at start 8.6±3.3 y, mean age at stop 18.4±2.1 y). Eighteen out of 25 patients reached FH. A substantial improvement in FH of 1.7 SDS, equivalent to 10.4 cm compared to pre‐treatment height, was observed. No significant difference was seen between the two GH doses. Females gained a mean height of 9.8 cm and males 1–13 cm (FH 174.5±7.8 cm vs mean adult height of 162.5±5.4 cm for males with NS) at final height. Moreover, 60% reached a mid‐parental height of±1 SD. Conclusion: GH treatment improves final height in patients with Noonan syndrome, with a mean gain of 1.7 SDS. The prepubertal height gain is maintained to final height and the children achieve a height close to their mid‐parental height.
Early post‐convulsive prolongation of QT time in children
Authors: Kändler Liane , Fiedler Andreas , Scheer Katrin , Wild Florentine , Frick Ulrich , Schneider Peter 
Published: August 2010

AbstractAim:An important differential diagnosis of seizures in childhood is the long QT syndrome. Childhood epilepsy occurs about 400 times more often than long QT syndrome. We had observed children with slight post‐convulsive prolongation of QT time more often than the reported incidence of long QT syndrome. We therefore conducted a prospective study to define the characteristics of post‐convulsive prolongation of QT time in children.Methods:We investigated 30 consecutive infants and children (3 mo to 14 y) within 2 h after seizures. A follow‐up ECG was obtained 1–9 d later. We also obtained ECGs from 30 healthy age‐ and gender‐matched controls. We calculated the QT interval corrected for heart rate (QTc) by Bazett's formula in leads II, V5, V6, QT dispersion and the number of notched T waves.Results:We found a QTc interval of more than 440 ms in one or more leads in the first ECG in seven of 30 infants and children compared to 1 of 30 in the follow‐up ECG (p=0.0003) and two of 30 in the healthy controls (p=0.14). Average QTc was higher for all leads in the first ECG. This was statistically significant in leads II (414 vs 402 ms,p=0.008), V5 (416 vs 404 ms,p=0.002) and V6 (415 vs 399 ms,p=0.001). Compared to healthy controls, QT dispersion was slightly larger in the early post‐convulsive ECG (36 vs 31 ms,p=0.03). Notched T waves occurred more frequently in the early compared to the late post‐convulsive ECGs (p=0.009).Conclusion:Slight to moderate post‐convulsive prolongation of the QT interval is not rare but transient in paediatric patients.
Use of supplementary equipment for resuscitation of newborn infants at tertiary perinatal centres in Australia and New Zealand
Authors: O'donnell Colm P. F. , Davis Peter G. , Morley Colin J. 
Published: August 2010

Abstract Aim: Neonatal resuscitation is a common and important intervention. International consensus statements advise how newborns should be resuscitated and suggest equipment to be used. Use of equipment not specifically recommended in these guidelines has been advocated. We wished to determine how widely this supplementary equipment is used in a geographically defined region. Methods: Each of the 25 tertiary perinatal centres with on‐site deliveries in Australia and New Zealand was surveyed. The questionnaire asked about the use of the following items during delivery room resuscitation: pulse oximetry, exhaled carbon dioxide detection, polyethylene wrapping, oxygen blenders, laryngeal mask and oropharyngeal airways. Results: Data were obtained from all centres. Pulse oximetry is used at 12 (48%) centres. Exhaled CO2detection is used to confirm endotracheal tube placement at three (12%) of the centres. Polyethylene wrapping is used to prevent heat loss in very‐low‐birthweight infants at delivery at 11 (44%) centres. Oxygen blenders are used to modify the amount of oxygen delivered at nine (36%) centres. Laryngeal mask airways are infrequently used at two (8%) centres. Oropharyngeal airways are infrequently used at five (20%) centres. Conclusion: There is considerable variation in the equipment and techniques used to resuscitate newly born infants. Use of equipment not specifically recommended in international consensus statements is widespread. These are potentially effective tools to improve resuscitation. The evidence supporting their use is, however, limited. Urgent evaluation of their efficacy and safety is required before even more widespread use occurs.
Outcome in neonates with congenital heart disease referred for respiratory extracorporeal membrane oxygenation
Authors: Brown Kate L. , Miles Fiona , Sullivan Ian D. , Hoskote Aparna , Verhulst Linda , Ridout Deborah A. , Goldman Allan P. 
Published: August 2010

Abstract Aim: To evaluate the proportion of neonates referred for extracorporeal membrane oxygenation (ECMO) support in the modern era of advanced conventional treatments for respiratory failure who actually had congenital heart disease (CHD), and to assess the impact of this diagnostic route on patient condition and outcome. Methods: A retrospective case‐note review of neonatal ECMO and cardiac admissions to a single, tertiary ECMO and cardiac intensive care unit (ICU) between March 1999 and February 2002. Results: 287 symptomatic neonates presented to the ICU with previously undiagnosed cardiac or respiratory disease. Eighty‐two with presumed respiratory failure were referred for ECMO, and 205 with suspected CHD were referred for cardiac evaluation. Eight (10%) ECMO referrals, all with presumed persistent pulmonary hypertension of the newborn (PPHN), were found to have CHD (transposition: 3; total anomalous pulmonary venous connection: 3; left heart obstructive lesions: 2). Mortality in this group was 50%, compared with 11% for correctly identified CHD patients (odds ratio 8.2, 95% CI 1.92, 35.4, p<0.01). For all neonates with CHD, the risk of death was increased by the presence of cardiovascular collapse and end‐organ dysfunction at presentation to the ICU (p<0.01 for both). Conclusion: Neonates with CHD may present as severe “PPHN” via the ECMO service. Poor outcome in these patients relates to the high incidence of cardiovascular collapse and end‐organ dysfunction. Early echocardiography is recommended for neonates with presumed PPHN. Neonatal ECMO support should be based in centres with cardiac surgical services.
Mothers' experiences of child health clinic services in Sweden
Authors: Örtenstrand Annica , Waldenström Ulla 
Published: August 2010

Abstract Aim: To investigate women's experiences of child health clinic (CHC) care and risk factors for being dissatisfied. Experiences of specific aspects of care were further explored in subgroups of women who were not generally satisfied. Methods: All Swedish‐speaking women admitted to any of the approximately 600 antenatal clinics in Sweden during 3 wk evenly spread over 1 y in 1999 and 2000 were invited to participate in the study. Altogether, 3113 women agreed to participate. Data were collected by questionnaires: in early pregnancy, 2 mo and 1 y postpartum, and from the Swedish Medical Birth Register. For the purpose of this study, data from the 2415 women who answered the question used as the principal outcome in this study, i.e. overall satisfaction with CHC care, were analysed. Results: Psychological factors, such as maternal depressive symptoms and worry about caring for the newborn, and serious infant feeding problems were predictors of less satisfied or mixed feelings about CHC care. About one in three women were dissatisfied with the attention paid to their own needs, and a similar proportion said information about vaccinations was insufficient. Of the four subgroups—mothers whose babies had feeding problems and mothers with depressive symptoms at 2 mo, 1 y, and on both occasions—the latter group was the least satisfied with the nurse and the time allocated to various issues. Conclusions: Swedish mothers appear to be satisfied with the attention given to their infants at CHCs, but many feel that their own needs as new mothers are neglected. This study supports current development towards screening for maternal depression at CHCs.
Socio‐economic determinants, maternal smoking and coffee consumption, and exclusive breastfeeding in 10 205 children
Authors: Ludvigsson Jonas F. , Ludvigsson Johnny 
Published: August 2010

AbstractAim:To examine socio‐economic factors, smoking, coffee consumption and exclusive breastfeeding duration.Methods:This study was part of a prospective cohort study of children born between 1 October 1997 and 1 October 1999 (the All Babies in Southeast Sweden (ABIS) study). Eleven socio‐economic characteristics (parental employment, civil status, whether parents were born in Sweden, parental education, residence at birth and during child's first year, crowded living), maternal smoking, coffee consumption, infant sex, siblings, parental age, and maternal alcohol consumption during pregnancy were analysed using logistic regression and Cox's proportional hazards method. All data were obtained through questionnaires distributed at infant birth and at 1 y of age. Exclusive breastfeeding duration <4 mo and actual breastfeeding duration were our main outcome measures.Results:Out of 10&!hairsp;205 infants, 2&!hairsp;206 (21.6%) were exclusively breastfed for less than 4 mo (“short exclusive breastfeeding”; SEBF). Backward stepwise regression analysis identified the following risk factors for SEBF: maternal smoking (95% confidence interval for adjusted odds ratio, 95% CI AOR 2.00–2.82), low maternal education (95% CI AOR 1.45–2.19), maternal employment less than 3 mo during pregnancy (95% CI AOR 1.17–1.54), paternal age 29 y (95% CI AOR 1.14–1.47), maternal age 29 y (95% CI AOR 1.08–1.39) and low paternal education (95% CI AOR 1.08–1.48). The odds ratio for SEBF increased with the number of cigarettes smoked. Coffee consumption was not associated with duration of exclusive breastfeeding.Conclusion:This study indicates that socio‐economic factors and smoking may be of importance to the risk of breastfeeding exclusively for less than 4 mo, while coffee consumption is not.
Gender affects self‐evaluation in children with cystic fibrosis and their healthy siblings
Authors: Wennström Inga‐lill , Berg Ulla , Kornfält Ragnhild , Rydén Olof 
Published: August 2010

Abstract Aim: To determine whether self‐esteem among children with cystic fibrosis (CF) and their healthy siblings differs from that of a healthy reference group and whether there are differences within and between sibling pairs. Methods: All Swedish CF children 6–14 y old with a healthy sibling in the same age range (n=65) were invited to participate, 55 sibling pairs and their parents taking part in the study. Five aspects of the children's self‐concept—physical characteristics, skills and talents, mental well‐being, relations to parents and family, and relations to others—were assessed by the “I think I am” self‐evaluation questionnaire. Severity of illness was assessed by means of the Shwachman Clinical Evaluation System. Results: Whereas self‐evaluation did not differ between groups at a general level, healthy girls as well as those with CF scored lower than girls in the reference group on the “mental well‐being” and “relations to parents and family” subscales. Comparison of gender combinations (sick girl/healthy boy, sick girl/healthy girl, sick boy/healthy boy, sick boy/healthy girl) suggested that girls pay a cost of a lesser sense of psychological well‐being and feelings of inadequacy in relation to their parents and family. The Shwachman score of the sick child was not related to the level of self‐esteem. Conclusion: When CF is present among siblings, girls seem to carry more of the family pain than boys, a finding that calls for an increased awareness of the girls' situation by members of care teams.
Ages and Stages Questionnaire used to measure cognitive deficit in children born extremely preterm
Authors: Klamer Anja , Lando Ane , Pinborg Anja , Greisen Gorm 
Published: August 2010

Abstract Aim: To validate the Ages and Stages Questionnaire (ASQ) and to measure average cognitive deficit in children born extremely preterm. Methods: Parents of 30 term children aged 36–42 mo completed the ASQ and the children underwent the Wechsler Preschool and Primary Scales of Intelligence—Revised. In a second study, the ASQ was obtained in 22 children born extremely preterm and 19 term children at the age of 35–44 mo. Results: The overall ASQ score correlated significantly with IQ (p=0.007). The children born extremely preterm had an ASQ score of −1.06 SD below the score of the term children (p=0.048). Conclusion: The ASQ identified a developmental deficit of the expected magnitude.
Neurodevelopmental outcome and haematological course of a long‐time survivor with homozygous alpha‐thalassaemia: Case report and review of the literature
Authors: Lücke Thomas , Pfister Stefan , Dürken Matthias 
Published: August 2010

Abstract Aim: Homozygous α‐thalassaemia, also called haemoglobin (Hb) Bart's hydrops fetalis, has been thought to be a lethal condition. Due to prenatal diagnosis and intrauterine blood transfusions, a few patients with Hb Bart's hydrops fetalis have survived. This fact raises the urgent questions of clinical management and appropriate follow‐up of these patients, both of which are addressed in this article. Methods: We report on a 6.5‐y‐old patient with homozygous α‐thalassaemia and review the literature of 13 other survivors published to date. Transfusion requirements were evaluated and the rate of liver iron accumulation was assessed by biomagnetic liver susceptometry before and after institution of iron‐chelating therapy. Psychometric evaluation was carried out using Munich's Functional Development Test, the Columbia Mental Maturity Scale, the Kaufman Assessment Battery for Children, and the Peabody Picture Vocabulary Test. Results: Our patient had significant delay of psychomotor development. Psychometric evaluation at the age of 5 y revealed an IQ of 85 and an intellectual level of a 4‐y‐old child. Early tissue iron overload was seen, but a negative iron balance was achieved after institution of desferrioxamine treatment at dosages used for β‐thalassaemia. Conclusion: Homozygous α‐thalassaemia should no longer be regarded as a lethal condition. Early intervention during pregnancy and careful haematological as well as neuropsychological follow‐up was able to provide long‐term survival and good life quality in our patient.
The right ventricle in Fabry disease
Authors: Kampmann C , Baehner FA , Whybra C , Bajbouj M , Baron K , Knuf M , Wiethoff CM , Trübel H , Beck M 
Published: August 2010

Abstract Aim: Left ventricular (LV) hypertrophy is a common feature in Fabry disease‐related progressive infiltrative hypertrophic cardiomyopathy and affects both men and women, but at different ages. To date, however, little is known about the role of right ventricular (RV) function in Fabry disease. Therefore, this study aimed to investigate the extent of RV involvement in patients with Fabry disease. Methods: Echocardiographic examination of the right and left ventricle was carried out in 129 patients (80 women and 49 men) with Fabry disease. Results: RV hypertrophy was present in 46 patients (35.7%). Of these patients, 13 showed signs of severely depressed right systolic function (tricuspid annulus movement <10 mm and a prolonged RV pre‐ejection period/pulmonary ejection time ratio) and six patients showed additional severe depression of parameters of diastolic function (pseudo‐normal or restrictive RV filling patterns). Those patients with RV hypertrophy and severely compromised systolic and diastolic function had the highest LV masses (92±11.7 g/m2.7). Conclusion: RV involvement is common in Fabry disease and ultimately progresses to severe systolic and diastolic RV dysfunction. These findings might explain why patients with preserved LV function can develop clinical features such as reduced exercise capacity, organomegaly and lymphoedema.
Vascular complications of Fabry disease: enzyme replacement and other therapies
Authors: Hughes DA , Mehta AB 
Published: August 2010

AbstractFabry disease is an X‐linked glycosphingolipid storage disorder resulting from deficiency of α‐galactosidase A. Storage of globotriaosylceramide ultimately results in multiorgan pathology, including cerebrovascular, cardiovascular and renal disease. Vascular involvement is evident throughout the body but the mechanisms by which storage on a cellular level leads to end‐organ pathology are unknown. Here the evidence for abnormal blood flow, vessel architecture and endothelial function will be reviewed and possible models of vascular pathology discussed. The effects of reversal of storage within vessels by enzyme replacement therapy (ERT) and the possibilities for intervention with additional agents will be considered. Conclusion: The pathology of Fabry disease has an important vascular component, although the underlying pathophysiology is unclear. Preliminary evidence suggests that ERT may have beneficial effects on the vascular component of this multisystem disease.