bluuebunny/crossref_metadata_2025_split huggingface dataset:
Recurrent gastroesophageal reflux in neurologically impaired patients after fundoplication
Authors: Goessler A , Huber‐Zeyringer A , Hoellwarth ME 
Published: April 2008

AbstractGoessler A, Huber‐Zeyringer A, Hoellwarth M.E. “Recurrent gastroesophageal reflux in neurologically impaired patients after fundoplication.” Acta Paediatr 00, Stockholm. ISSN 0803‐5253.Aim: To evaluate the outcome and analyse the main causes of complications and failures of antireflux surgery for gastroesophageal reflux disease (GERD) in neurologically impaired patients (NIP).Methods: From 1985 to 1999 44 NIP (mean age 12 years) underwent surgery for GERD. Type of surgery, complications and recurrent reflux were analysed.Results: Twenty‐seven patients (61%) showed preoperatively severe failure to thrive. All patients showed pathologic results in 24‐h pH monitoring. Surgical treatment consisted of ventral (n = 25, Thal) or dorsal (n = 4, Toupet) semifundoplication or a Nissen fundoplication (n = 15). Postoperatively, all patients showed an impressive growth, regress of symptoms and improvement of results of diagnostic investigations. Late complications and recurrence of reflux were significantly related to preoperative extreme dystrophy (p < 0.0025). In six patients (15%) severity of symptoms related to recurrent reflux required a reoperation 17.8 month postoperatively (range 8–35 month). Recurrent reflux was found in 40% after ventral semifundoplication and in 46% after Nissen fundoplication (8 and 47 months postoperatively, respectively).Conclusion: Symptoms improved impressively after fundoplication in mentally retarded children. The incidence of recurrent reflux is not related to the type of surgery, however, it occurs significantly earlier with ventral semifundoplication when compared with Nissen fundoplication. Both late complications as well as recurrent reflux are related significantly to preoperative dystrophy.
Psychosocial problems in children with cystic fibrosis
Authors: Bregnballe V , Thastum M , Schiøtz PO 
Published: April 2008

AbstractAim: To compare the well‐being of children (7–14 years) with cystic fibrosis (CF) (n = 43) with the well‐being of healthy controls (n = 1121).Methods: The self‐report questionnaire Beck Youth Inventories (BYI) was used to study depression, anxiety, anger, disruptive behaviour and self‐concept in children with CF. A measure of social desirability was included as well as body mass index (BMI) and percentage of predicted forced expiratory volume in one second (FEV1) as measures of health status.Results: The children with CF did not differ from the norm group concerning depression, disruptive behaviour and self‐concept. Young children with CF (7–10 years) and boys with CF scored significantly higher on anxiety. Girls with CF scored significantly lower on anger than controls. BMI was not associated with any of the BYI subscales. In patients aged 11–14 years, there was a significant correlation between FEV1 and self‐concept as well as a significant inverse correlation between FEV1 and anxiety.Conclusions: Younger children with CF and boys with CF were more anxious than the healthy controls, and girls with CF expressed less anger than their healthy peers. Effects sizes however were small. Low FEV1 was associated with low self‐concept and high anxiety in adolescent patients.
Validating and updating a prediction rule for serious bacterial infection in patients with fever without source
Authors: Bleeker SE , Derksen‐Lubsen G , Grobbee DE , Donders ART , Moons KGM , Moll HA 
Published: April 2008

AbstractAim: To externally validate and update a previously developed rule for predicting the presence of serious bacterial infections in children with fever without apparent source.Methods: Patients, 1–36 mo, presenting with fever without source, were prospectively enrolled. Serious bacterial infection included bacterial meningitis, sepsis, bacteraemia, pneumonia, urinary tract infection, bacterial gastroenteritis, osteomyelitis/ethmoiditis. The generalizability of the original rule was determined. Subsequently, the prediction rule was updated using all available data of the patients with fever without source (1996–1998 and 2000–2001, n = 381) using multivariable logistic regression.Results: the generalizability of the rule appeared insufficient in the new patients (n = 150). In the updated rule, independent predictors from history and examination were duration of fever, vomiting, ill clinical appearance, chest‐wall retractions and poor peripheral circulation (ROC area (95%CI): 0.69 (0.63–0.75)). Additional independent predictors from laboratory were serum white blood cell count and C‐reactive protein, and in urinalysis ≥70 white bloods (ROC area (95%CI): 0.83 (0.78–0.88).Conclusions: A previously developed prediction rule for predicting the presence of serious bacterial infection in children with fever without apparent source was updated. Its clinical score can be used as a first screening tool. Additional laboratory testing may specify the individual risk estimate (range: 4–54%) further.
Developing a social brain
Authors: Johnson Mark H 
Published: April 2008

AbstractA new account of human postnatal functional brain development, interactive specialization (IS), is introduced and applied to one important domain of human development: the social brain. Behavioural, ERP and fMRI evidence from newborns, infants and children in face processing tasks is briefly reviewed, and is shown to be consistent with predictions from the IS account.Conclusion: It is suggested that this new view of functional brain development can also be applied to other domains of typical and atypical human development.
Preliminary evidence of a sensitive period for olfactory learning by human newborns
Authors: Romantshik O , Porter RH , Tillmann V , Varendi H 
Published: April 2008

AbstractAim: To test the hypothesis that a brief window of time immediately after delivery may be a particularly sensitive period for olfactory learning by human neonates.Methods: Fifty‐five vaginally delivered newborns were exposed to an odorant for 30 min beginning 4–37 min after birth (Early exposure) or 12‐h post‐partum (Late exposure). Several days later, newborns' head orientation responses to the exposure odour versus an unfamiliar odour or an odourless control stimulus were tested.Results: Infants in the Early exposure group spent significantly more time oriented towards the familiar scent rather than a novel odour (Z = 2.869; n = 28; p < 0.01), or an odourless stimulus (Z = 2.550; n = 28; p < 0.01). Infants in the Late exposure condition did not respond differentially to the exposure odour versus a novel odour (Z = 1.105; n = 27, p = 0.27), and spent more time oriented towards an odourless stimulus than to the exposure odour (Z = 2.042; n = 27, p < 0.05).Conclusion: Infants in the Early exposure group, but not in the Late exposure group, became familiar with the exposure odour and retained a memory trace of it during the test trials.
Females born large for gestational age have a doubled risk of giving birth to large for gestational age infants
Authors: Ahlsson F , Gustafsson J , Tuvemo T , Lundgren M 
Published: April 2008

AbstractAim: To analyse if females born large for gestational age (LGA) have an increased risk to give birth to LGA infants and to study anthropometric characteristics in macrosomic infants of females born LGA.Methods: The investigation was performed as an intergenerational retrospective study of women born between 1973 and 1983, who delivered their first infant between 1989 and 1999. Birth characteristics of 47 783 females, included in the Swedish Birth Register both as newborns and mothers were analysed. LGA was defined as >2 SD in either birth weight or length for gestational age. The infants were divided into three subgroups: born tall only, born heavy only and born both tall and heavy for gestational age. Multiple logistic and linear regression analyses were performed.Results: Females, born LGA with regard to length or weight, had a two‐fold (adjusted OR 1.96, 95% Cl 1.54–2.48) increased risk to give birth to an LGA infant. Females, born LGA concerning weight only, had a 2.6 (adjusted OR 2.63, 95%, 1.85–3.75) fold increased risk of having an LGA offspring heavy only and no elevated risk of giving birth to an offspring that was tall only, compared to females born not LGA. In addition, maternal obesity was associated with a 2.5 (adjusted OR 2.56, 95%, 2.20–2.98) fold increased risk of having an LGA newborn, compared to mothers with normal weight.Conclusion: Females, born LGA, have an increased risk to give birth to LGA infants, compared to mothers born not LGA. Maternal overweight increases this risk even further.
Early cardiac involvement in children carrying the A3243G mtDNA mutation
Authors: Wortmann S B , Rodenburg R J , Backx A P , Schmitt E , Smeitink J A M , Morava E 
Published: April 2008

AbstractThe phenotypic spectrum of the mitochondrial A3243G DKA mutation is highly variable, particularly when occuring in childhood. In contrast to the classical presentation in adulthood (MELAS syndrome; mitochondria! myopathy, encephalopathy, lactic acidosis and stroke‐like episodes) children show a different pattern of symptoms, often without the typical encephalopathy or psychomotor regression. We present six children carrying the A3243G mtDNA mutation with a heteroplasmy above 50 % in muscle tissue. The age of diagnosis ranged from 2 weeks up to 14.5 years. The clinical presentation was rather non‐specific including muscle weakness, developmental delay and epilepsy. In this small pediatric group we detected presymptomatic cardiac involvement in five out of six children already at an early stage of disease. The cardiac pathology included cardiomyopathy and biventricular hypertrophy with rhythm disturbances (for example long QT‐syndrome). The observed cardiac changes do not always increase the risk of cardiac deterioration; however, two of our patients died early on.Conclusion: We hypothesize that the A3243G mutation might be underdiagnosed, as patients could suffer from an unexplained cardiac death before the diagnosis is made. We advise performing regular repeated ECGs and echocardiography in all children carrying a A3243G mtDNA mutation independently from the presence of cardiac symptoms.
New guidelines for newborn resuscitation
Authors: Saugstad Ola Didrik 
Published: April 2008

AbstractThe new guidelines from the International Liaison Committee on Resuscitation and American Heart Association/American Academy of Pediatrics for newborn resuscitation underline that efficient ventilation is the key to a successful resuscitation of the newly born infant. Compared with the former guidelines published in 1999, the major changes are (i) less emphasis on using supplemental oxygen when initiating resuscitation, (ii) no need for routine intrapartum oropharyngeal and nasopharyngeal suctioning for vigorous infants born to mothers with meconium staining of amniotic fluid, (iii) occlusive wrapping of very low birth weight infants <28 weeks to reduce heat loss is recommended, (iv) preference for the intravenous versus endotracheal route for adrenaline and (v) more emphasis on parental autonomy at the threshold of viability. A number of gaps in newborn resuscitation have been identified and discussed.Conclusion: The new guidelines for newborn resuscitation are more evidence‐based than previously ones. However, still there is a need for further research and modifications.
Working mothers breastfeed babies more than housewives
Authors: Bakoula Chryssa , Veltsista Alexandra , Prezerakou Anargyroula , Moustaki Maria , Fretzayas Andreas , Nicolaidou Polyxeni 
Published: April 2008

AbstractAim: To examine the prevalence and determinants of breastfeeding and to identify perinatal, sociodemographic, psychosocial and environmental factors associated with maternal infant feeding intention.Methods: A sample of 3734 Greek mothers that delivered their infants in 2000 was recruited in a longitudinal cohort study. Data on duration of breastfeeding was based on a questionnaire answered by 76.6% of the participants at 8–12 months postpartum.Results: The rates of any breastfeeding at 3 and 6 months were 52% and 24%, respectively. The corresponding rates of exclusive breastfeeding were 37% and 17%. From Cox regression analysis mother's intention to breastfeed for a short time and upbringing in the area of Athens were significantly associated with early termination of any breastfeeding and exclusive breastfeeding. A positive intention to breastfeed was influenced by maternal entitlement more than 6 months, delivery in autumn or winter, mother's upbringing abroad and previous breastfeeding experience.Conclusions: The rates of any breastfeeding and exclusive breastfeeding during the first year of life were fairly satisfactory. Programs to support breastfeeding are necessary and should encourage women to feel positive to breastfeed, focusing particularly on non‐working mothers, mothers with a maternity entitlement less than 6 months and mothers who lack previous breastfeeding experience.
A major deletion in the surfactant protein‐B gene causing lethal respiratory distress
Authors: Wegner Daniel J , Hertzberg Torbjörn , Heins Hillary B , Elmberger Göran , MacCoss Michael J , Carlson Christopher S , Nogee Lawrence M , Cole F Sessions , Hamvas Aaron 
Published: April 2008

AbstractBackground: Loss of function mutations in the surfactant protein‐B gene (SFTPB) cause lethal neonatal respiratory distress due to reduced or absent expression of mature surfactant protein B (SP‐B, encoded in exons 6 and 7). No large deletions in SFTPB have been previously identified.Aim: Genomic, proteomic and immunohistochemical characterization of a 3 kb deletion in SFTPB.Methods: A full‐term newborn presented with refractory respiratory failure. We amplified and sequenced SFTPB from the infant and both parents, determined SP‐B protein expression in tracheal aspirate samples using Western‐blot analysis, and performed immunohistochemical staining and electron microscopy of lung biopsy tissue.Results: The infant was homozygous for a 2958 bp deletion in SFTPB that included exons 7 and 8. Both asymptomatic parents were heterozygous for the deletion. A truncated mature SP‐B peptide was detected on Western blotting of tracheal aspirate. Amino acid sequence specific to that encoded in exon 5 was present, but that encoded by exon 7 was absent. ProSP‐B expression was robust within alveolar type II cells and lamellar body structure was disrupted.Conclusions: This deletion in SFTPB resulted in SP‐B deficiency due to absence of elements in mature SP‐B that are critical for appropriate peptide folding, trafficking and processing.
Rapunzel syndrome: a diagnosis overlooked
Authors: Mathai John , Chacko Jacob , Kumar T. Sathish , Scott Julius Xavier , Agarwal Indira , Varkki Sneha 
Published: April 2008

AbstractWe report two children who presented with a painless abdominal mass masquerading as a splenic enlargement. The paucity of symptoms resulted in delayed diagnosis. At laparotomy the trichobezoars were found to have a tail extending into the small intestine.Conclusion: This condition eludes diagnosis unless sought for particularly in the paediatric age group. Hence we present our experience of two consecutive cases of paediatric Rapunzel syndrome, along with the relevant literature and in retrospect, how an early diagnosis could have been made.
Parental stress and toddler behaviour at age 18 months after pre‐term birth
Authors: Jackson Karin , Ternestedt Britt‐Marie , Magnuson Anders , Schollin Jens 
Published: April 2008

AbstractAim: To describe the parent's judgement of their own stress and the child's behaviour at 18 months after pre‐term birth and if there are any correlations between these assessments, the morbidity in the neonatal period, the gestational age at birth and the occurrence of twin/triplet births.Method: Twenty‐one mothers and 19 fathers of pre‐term infants answered two questionnaires, The Swedish Parenthood Stress Questionnaire (SPSQ) and The Toddler Behaviour Questionnaire (TBQ).Results: Mothers scored somewhat higher than fathers concerning parental stress. Parents with twins/triplets and of children born in gestational week 25–30 felt more stress, though the differences were not statistically significant. High‐risk diagnoses did not correlate to any of the dimensions. The parents judged the behaviour of the children similar except that parents of children born in gestational week 25–30 scored significantly higher for intensity/activity (p = 0.002). The correlation between parental stress and judgement of their children's behaviour did not show any significant association except for the dimension regularity in TBQ (p = 0.016).Conclusions: The mothers' and fathers' assessments of their own stress and of the children's behaviour were similar. Parents of very pre‐term children felt more stress and judged the children somewhat delayed in their social behaviours, probably due to their low gestational age.
Novel mutations in the gene encoding ATP binding cassette protein member A3 (ABCA3) resulting in fatal neonatal lung disease
Authors: Saugstad Ola D. , Hansen Thor Willy Ruud , Rønnestad Arild , Nakstad Britt , Tølløfsrud Per Arne , Reinholt Finn , Hamvas Aaron , Cole F. Sessions , Dean Michael , Wert Susan E. , Whitsett Jeffrey A. , Nogee Lawrence M. 
Published: April 2008

AbstractAim: To investigate whether intractable respiratory distress syndrome in three Norwegian term infants was due to mutations in the ABCA3 gene.Methods: The genes encoding SP‐B (SFTPB), SP‐C (SFTPC), and ABCA3 (ABCA3) were sequenced from the parents of one infant and two unrelated infants with fatal neonatal lung disease. Lung tissue was examined by histology, immunohistochemistry and electron microscopy.Results: Novel ABCA3 mutations were identified in each family. One patient had a phenotype differing from previous descriptions of this disease with an initial uneventful period. The diagnosis was established 19 years after death by analysing DNA material from the parents, with an ABCA3 mutation identified on one allele in each parent. The other two infants had more typical clinical courses with the onset of respiratory symptoms immediately after birth. ABCA3 mutations were identified on both alleles from these two infants, and electron microscopy of alveolar type 2 cells demonstrated abnormal lamellar body formation characteristic of this disorder.Conclusion: ABCA3 mutations were the basis for lung disease in all three patients. Children with lung disease due to ABCA3 deficiency may not have symptoms at birth. The finding of five novel mutations indicates allelic heterogeneity for ABCA3 mutations within the Norwegian population.
Lack of recognition and complexity of foetal alcohol neuroimpairments
Authors: Elgen I , Bruaroy S , Laegreid L M 
Published: April 2008

AbstractAims: To obtain the recorded prevalence of foetal alcohol syndrome (FAS) and foetal alcohol spectrum disorders (FASD) in Norway, and evaluate the effect of a general information program to increase the recognition of FAS/FASD for health care and social workers.Methods: A questionnaire regarding prevalence of FAS/FASD was sent to all Norwegian paediatric and child psychiatry departments. In the region Hordaland county, an information program was carried out to educate health‐care and social workers on symptoms and signs of FAS/FASD, and referral was encouraged for suspected cases. Referred children received a neuropaediatric evaluation, and the effect of the information program on recorded FAS/FASD was recorded.Results: Based on the national survey, a prevalence of 0.3 per 1000 was calculated. After the information program, the estimated prevalence in Hordaland County increased to 1.5 per 1000. In 5 years, 25 children were diagnosed with FAS and 22 with FASD. One‐third of all children were mentally retarded. Microcephaly and neuroimpairments were more common among FAS children. Almost all children met the criteria of ADHD.Conclusion: The rate of FAS/FASD may be greatly underestimated because of lack of knowledge. An information program aimed at health‐care and social workers is effective.
Is there a connection between severe cerebral palsy and increased gluten sensitivity?
Authors: Stenberg R , Schollin J 
Published: April 2008

AbstractIn nine children with severe cerebral palsy (CP), feeding difficulties and poor development of weight and height, laboratory markers for metabolic and enteral dysfunction were studied.Conclusion: Four of the nine patients with CP had increased levels of antigliadin antibodies AGA (IgA), a finding which calls for further studies concerning the possible connection between increased celiac markers and CP.
Enzyme replacement therapy with agalsidase alfa in children with Fabry disease
Authors: Ramaswami U , Wendt S , Pintos‐Morell G , Parini R , Whybra C , Leon Leal J A , Santus F , Beck M 
Published: April 2008

AbstractAim: To assess the effects of enzyme replacement therapy (ERT) in children with Fabry disease.Methods: Safety and efficacy of ERT with agalsidase alfa, 0.2 mg/kg infused over 40 minutes every 2 weeks for 23 weeks, were studied in a multicentre open‐label trial in nine boys and four girls. Median age at the start of the study was 11.0 years (range 3.5–18 years).Results: Fifty‐four adverse events were reported in 11 patients. No serious adverse events related to ERT were reported. Twelve of the 54 adverse events were considered possibly or probably related to ERT. Infusion reactions (8 mild, 3 moderate) occurred in four boys, in seven infusions. One boy developed IgG antibodies, although he continued to make good clinical progress. At the end of the study, two of the four boys and the one girl on regular pain medication at baseline had stopped taking analgesics. Brief Pain Inventory (BPI) scores decreased in most patients by week 12 and were sustained until the end of the study. This change was greater in the boys, who had higher (worse) BPI scores at baseline. Pain‐related quality of life (QoL) scores also decreased during the study. Plasma globotriaosylceramide concentrations and urinary globotriaosylceramide:sphingomyelin ratios decreased after 12 and 23 weeks of therapy, particularly in the boys. Increases in sweat volume were recorded in three out of five of the boys and in one of two girls tested after 23 weeks of treatment.Conclusion: ERT with agalsidase alfa in children with Fabry disease is well tolerated and, in the short term, appears to decrease pain and to improve pain‐related QoL.