bluuebunny/crossref_metadata_2025_split huggingface dataset:
A new type of Ehlers‐Danlos syndrome associated with tortuous systemic arteries in a large kindred from Qatar
Authors: Wahab A Abdul , Janahi IA , Eltohami A , Zeid A , Haque NF Ul , Teebi AS 
Published: August 2010

Aim: To describe the clinical spectrum of anomalies of a new type of Ehlers‐Danlos syndrome in 32 patients from a large inter‐related extended family in Qatar. Methods: Among the 32 patients (from 22 families), there were 6 affected pairs of siblings and 2 families with 3 affected siblings. The male to female ratio was 2:1, ages ranging from birth to 18 y (mean 7.4 y). Results: Anomalies included a variable degree of skin hyperextensibility, hypermobility of small and large joints, and tortuous systemic arteries. Peculiar facial features included epicanthic folds, flat saggy cheeks, elongated faces and micrognathia. The combination of an elongated aortic arch and tortuous brachiocephalic arteries was seen in 30 patients (93.8%), aneurysm of the ascending aorta in 3 patients (9.4%), bifid pulmonary artery in 27 patients (84.4%) and multiple severe peripheral stenosis of the right and/or left pulmonary artery in 7 patients (21.9%). A prominent aortic knuckle was observed on the chest roentgenograms of 30 patients (93.8%); inguinal hernia in 11 patients (34%), diaphragmatic hernia and/or hiatus hernia in 7 patients (21.9%); and laryngo‐tracheomalacia in 2 patients (6.3%). Generalized muscle hypotonia was found in 15 neonates (46.9%). Parental consanguinity involved in all the patients was traced to a common ancestor from a large Bedouin tribe in Qatar. These patients are at risk for potentially catastrophic arterial rupture. Linkage to the major loci involved in Ehlers‐Danlos syndrome and other connective tissue disorders, such as Cutis Laxa, Familial Aneurysm, and Osteogenesis imperfecta, was excluded by using specific DNA markers, confirming the uniqueness of this disorder. Conclusion: The study describes a large cohort of patients from the same closely related family, sharing peculiar dysmorphisim and consistent radiological and echocardiographic features different from known types of Ehlers‐Danlos syndrome. As known loci involved in Ehlers‐Danlos syndrome and other connective tissue disorders were excluded by specific DNA markers, this appears to be a new type of Ehlers‐Danlos syndrome or even a new syndrome.
Extemporaneous (magistral) preparation of oral medicines for children in European hospitals
Authors: Brion F , Nunn AJ , Rieutord A 
Published: August 2010

Aim: To evaluate methods of preparation of oral medicines in European children's hospitals when drugs prescribed are unlicensed or off‐label, and to determine whether such extemporaneously prepared medicines are available as suitable, authorized products in other countries. Methods: A questionnaire was distributed to 41 hospital pharmacists in 18 European countries, requesting information on the most frequent extemporaneously prepared oral liquid, powder and capsule medicines, and on their formulation and stability. Information was gathered on the availability of suitable authorized forms of these medicines in other European countries, the USA and Australia. Results: 21 questionnaires from 16 countries returned showed that the methods of extemporaneous preparation vary in different European countries with, for example, liquid preparations predominating in England and Sweden, capsules in France and Spain, and powders in Finland and Italy. The top 20 preparations are made in many different strengths and formulations. The same drug may be prepared in liquid, capsule or powder form, depending on the country. Many of the extemporaneous preparations were available as suitable authorized paediatric medicines in other countries. The quality of information available on formulation and stability was limited, and there was concern about the availability and quality of chemical ingredients. Conclusion: Preparation of children's oral medicines is subject to much variation in hospitals throughout Europe and there is little harmonization of formulations or information on stability of products. The European Union could be the focus for improving the availability of appropriate authorized medicines for children and ensuring that when extemporaneous preparation is necessary it is of a common high standard.
Neonatal ovarian cysts
Authors: Vogtländer MF , Rijntjes‐Jacobs EGJ , van den Hoonaard ThL , Versteegh FGA 
Published: August 2010

Foetal and neonatal ovarian cysts have previously been considered uncommon. With the development of sonography, however, the detection of cysts has increased. As ovarian cyst formation in the perinatal period is a self‐limiting process, treatment options depend on the risk of complications and on the ability to differentiate these benign cysts from other pathology. The history of two neonates with an ovarian cyst detected antenatally with ultrasound techniques is described. Conclusion: It is important that paediatricians have knowledge about the incidence, treatment and prognosis of foetal neonatal ovarian cysts.
Intravenous immunoglobulin therapy for acute cerebellar ataxia
Authors: Go T 
Published: August 2010

A case of acute cerebellar ataxia without any prodromal illness showed cerebellar hypoperfusion on 123I‐iodoamphetamine single photon emission computed tomography. The symptoms did not resolve spontaneously or with methylprednisolone pulse therapy but disappeared rapidly with intravenous immunoglobulin therapy. Conclusion: Intravenous immunoglobulin therapy is worth considering in acute cerebellar ataxia that does not respond to high‐dose steroid therapy.
Paraneoplastic limbic encephalitis masquerading as chronic behavioural disturbance in an adolescent girl
Authors: Lee ACW , Ou Y , Lee WK , Wong YC 
Published: August 2010

Aim: To describe an unusual but treatable cause of behavioural disturbance in adolescence. Methods: The case is reported of a 15‐y‐old girl presenting with acute confusion, memory problems and psychotic symptoms following an 18‐mo history of change in personality, school failure and running away from home. A review of the literature is also presented. Results: Microbiology, toxicology, computed tomography and magnetic resonance imaging did not show any pathology of the central nervous system. Bilateral ovarian immature teratomas were eventually diagnosed and removed. The rapid improvement in the patient's mental and cognitive functions after corticosteroid treatment and the abnormality shown on the single photon emission computed tomography suggested a diagnosis of paraneoplastic limbic encephalitis. Conclusion: Paediatricians and neurologists should be aware of this rare disease entity among the more common conditions of behavioural problems and substance abuse in adolescents.
Reference values for change in body mass index from birth to 18 years of age
Authors: Karlberg J , Kwan CW , Albertsson‐Wikland K 
Published: August 2010

Body mass index (BMI) has become the measure of choice for determination of nutritional status during the paediatric years, as in adults. Recently, several cross‐sectional BMI childhood reference values standards have been published. In order precisely to evaluate childhood nutritional interventions, reference values allowing for the evaluation of changes in BMI values are also needed. For the first time, such reference values can be presented based on 3650 longitudinally followed healthy Swedish children born full term. The reference values for the change in BMI are given as the change in BMI standard deviation scores. The reference values are given as means of mathematical functions adjusting for gender, age of the child and the length of the interval between two measurements for interval lengths of 0.25 to 1.0 y before 2y of age and of 1 to 5 y between birth and 18 y. The usefulness of the reference values is proved by a graph that forms a part of a clinical computer program; the ‐2 to +2 standard deviation range of the predicted change in BMI can be computed for an individual child and drawn in the graph as an extended support for clinical decision‐making. Conclusion: For the first time this communication gives access to BMI growth rate values that can be used both in research and in the clinic to evaluate various interventions, be they nutritional, surgical or therapeutic.
Thymic size in uninfected infants born to HIV‐positive mothers and fed with pasteurized human milk
Authors: Jeppesen D , Hasselbalch H , Ersbøll AK , Heilmann C , Valerius NH 
Published: August 2010

Aim: To examine the size of the thymus in uninfected infants born to HIV‐positive mothers and to study the effects of feeding by human donor milk on the size of the thymus in these infants. Methods: The absolute and relative thymic size was assessed by sonography as thymic index (Ti), and the Ti/weight‐ratio (Ti/w) at birth and at 4 mo of age in 12 healthy uninfected infants born to HIV‐infected mothers. All infants were exclusively fed pasteurized donor milk. The results were compared with those obtained from a previous cohort of exclusively breastfed, partially breastfed and exclusively formula‐fed infants. Results: At birth the Ti was reduced in infants born to HIV‐infected mothers in comparison with that in control infants but this difference disappeared when their birthweights were taken into consideration (Ti/w‐ratio). At 4 mo of age the geometric mean Ti of infants fed donor milk was 23.8 and the mean Ti/w‐ratio was 4.2. Compared with those of exclusively breastfed infants, the Ti and Ti/w‐ratio of infants fed donor milk were significantly reduced (p < 0.01). The Ti/w‐ratio increased in donor‐milk‐fed infants compared with that in the formula‐fed infants (p= 0.02). Conclusion: At birth the size of the thymus was smaller in uninfected infants of HIV‐positive mothers compared with infants of HIV‐negative mothers but when birthweight was taken into account this difference disappeared. Feeding by human donor milk seemed to result in an increased size of the thymus at 4 mo of age compared with thymic size in infants that were exclusively formula fed.
Conscious or deep sedation: a questionnaire regarding the experience of parents, children and staff during small bowel biopsy
Authors: Skoglösa J , Fälth‐Magnusson K , Stenhammar L 
Published: August 2010

Aim: The paediatric clinics of Linköping and Norrköping, Sweden, have different procedures regarding premedication and sedation during small bowel biopsy in children with suspected or diagnosed coeliac disease. In Linköping deep sedation using intravenous propofol is the method of sedation being used and parents are not present during the biopsy procedure. In Norrköping conscious sedation using intravenous midazolam is the routine and parents stay with their child throughout the whole biopsy procedure. The aim of this study was to find out whether the preprocedural and procedural differences between the clinics affected the way in which the parents and children experienced the time before and during the biopsy procedure. Methods: A questionnaire was used to ask the parents of 102 children who had undergone small bowel capsule biopsy for their opinion regarding the discomfort experienced by their children. The parents’and children's experience was also compared with that of the paediatric nurse caring for the family during the biopsy procedure, and the paediatric gastroenterologist performing the biopsy. Results: The differences regarding premedication and sedation between the two groups did not seem to affect the parents’or the children's total experience of the biopsy procedure, nor did the presence or absence of the parents throughout the biopsy procedure. As regards the sedation given, 95% of the parents did not think that their children suffered any discomfort at all. The total experience of the biopsy procedure on a five‐grade scale (5 being very good, 1 being very bad) was 5 for the parents and 4 for the children in both centres. Parents and children in both centres were very satisfied with the way in which they were taken care of during their visit to the hospital. In both units there was an obvious correlation between how the paediatric nurse experienced the biopsy procedure and how the paediatric gastroenterologist did, but only a weak correlation between the experience of the parents and that of the paediatric gastroenterologist and paediatric nurse. The anxiety of the parents was similarly estimated by the paediatric gastroenterologist and the paediatric nurse in both centres. There was no correlation between their assessment and the experience reported by the parents. Conclusion: The children undergoing small bowel biopsy and their parents felt well taken care of during their visit to the two hospitals. The differences between the clinics regarding method of sedation and presence or absence of the parents did not seem to affect how the parents and children experienced the biopsy procedure.
Treatment of extremely preterm infants: parents’attitudes
Authors: Hansen B Mølholm , Hoff B , Greisen G 
Published: August 2010

Aim: To conduct a survey of the attitude towards treatment of extremely preterm infants by comparing the attitude towards life‐saving treatment between a group of parents of extremely preterm children and parents in the general population. The importance the two groups of respondents assigned to parental preferences was also investigated. Methods: A Danish national cohort of children born from 1994 to 1995 with a birthweight below 1000 g or a gestational age below 28 wk were assessed in a 5‐y follow‐up study including a reference group of children born at term. The parents of the children were given a sequence of case vignettes presenting different clinical situations, which formed part of a questionnaire. Results: More than 80% of 222 possible index respondents and 76 possible reference respondents fulfilled the vignettes. Both groups of respondents were positive towards life‐saving treatment of extremely preterm infants. The recommendations given by both groups were significantly influenced by the hypothetical child's risk of having a serious handicap and the parents’preferences. The results suggest that parents of extremely preterm children do not have a more conservative attitude towards life‐saving treatment of extremely preterm infants than parents in the general population, and also support the view that parents’preferences should influence treatment decisions; although many believe that these should not be decisive. Conclusion: Compared to the general population, parents of children born extremely preterm did not express a more conservative attitude towards life‐saving treatment of extremely preterm infants.
Morbidity in children born to women infected with human immunodeficiency virus in South Africa: does mode of feeding matter?
Authors: Coutsoudis A , Pillay K , Spooner E , Coovadia HM , Pembrey L , Newell M‐L 
Published: August 2010

Aim: To examine infant morbidity risks associated with refraining from breastfeeding where it is used in an attempt to prevent mother‐to‐child transmission (MTCT) of human immunodeficiency virus (HIV). Methods: The population consisted of infants born to HIV‐infected women in South Africa who were participating in a vitamin A intervention trial to prevent MTCT of HIV. Women chose to breastfeed or formula feed their infants according to UNAIDS guidelines. Actual feeding practices and morbidity were recorded at clinic follow‐up visits at 1 wk, 6 wk, 3 mo and every 3 mo thereafter until 15 mo of age or cessation of breastfeeding. The infant's HIV status was assessed according to a predetermined algorithm. Results: HIV‐infected infants who were never breastfed had a poorer outcome than those who were breastfed; 9 (60%) of those who were never breastfed had 3 or more morbidity episodes compared with 15 (32%) of breastfed children [odds ratio (OR) 4.05, 95% confidence interval (95% CI) 0.91–20.63, p = 0.05]. During the first 2 mo of life, never‐breastfed infants (regardless of HIV status) were nearly twice as likely to have had an illness episode than breastfed infants (OR 1.91, 95% CI 1.17‐3.13, p = 0.006). Conclusion: The significant extra morbidity experienced in the first few months by all never‐breastfed infants and at all times by HIV‐infected infants who are not breastfed needs to be considered in all decisions by mothers, health workers and policy makers so as not to offset any gains achieved by decreasing HIV transmission through avoiding breastfeeding.
Controlled trial of dexamethasone in neonatal chronic lung disease: an 8‐year follow‐up of cardiopulmonary function and growth
Authors: Mieskonen S , Eronen M , Malmberg LP , Turpeinen M , Kari MA , Hallman M 
Published: August 2010

Aim: To evaluate the long‐term effects of postnatal dexamethasone treatment in high‐risk infants of very low birthweight. Methods The study included 16 children aged 7.8 to 9.2 y who had been born very prematurely at gestational ages of 24–29 wk and with birthweights of < 1500 g and who had participated in a randomized study of dexamethasone or placebo treatment in ventilator‐dependent infants at 10 d of age. Flow‐volume spirometry, impulse oscillometry, skin‐prick tests and Doppler echocardiography were carried out at school age, and respiratory morbidity and overall neurological outcome evaluated. Controls were 18 non‐atopic children born at term, tested for lung function. Results: No significant differences were found in respiratory morbidity at school age between the dexamethasone (n= 8) and placebo (n= 8) groups. Six of the 16 children had moderate to severe neurosensory impairments, but all were able to walk without support and attended primary school. In prematurely born children, standardized height was significantly less than that in controls, but between the two study groups, no significant differences existed in somatic growth. Atopy was uncommon: skin‐prick tests were positive in only one child in the placebo group. In the dexamethasone group, forced vital capacity adjusted to height was significantly higher than that in the placebo group, but impairment of basic lung function and bronchial obstruction was evident in both study groups. No hypertrophic cardiomyopathy was apparent, and non‐invasive measurements of pulmonary arterial pressure did not reveal any significant difference between the study groups. Conclusion: In very low‐birthweight children, high respiratory morbidity and neurological impairment continued until school age. Neonatal dexamethasone treatment at school age was not associated with any detectable adverse effects on somatic growth, or pulmonary or cardiac function.
Baby swimming increases the risk of recurrent respiratory tract infections and otitis media
Authors: Nystad W , Njå F , Magnus P , Nafstad P 
Published: August 2010

Aim: To estimate the association between baby swimming and recurrent respiratory tract infections and otitis media in the first year of life in children of parents without and with atopy. Methods: Norwegian schoolchildren (n= 2862) was enrolled in a cross‐sectional study of asthma and allergy using the questionnaire of the International Study of Asthma and Allergies in Childhood (ISAAC). The outcomes were parental retrospective report of recurrent respiratory tract infections and otitis media diagnosed by a physician in the first year of life. The exposure was baby swimming during the same period. Parental atopy reflects a history of maternal or paternal asthma, hayfever or eczema. Results: The prevalence of recurrent respiratory tract infections was higher (12.3%) among children who took part in baby swimming than among those who did not (7.5%). The prevalence of recurrent respiratory tract infections during the first year of life was 5.6% and 10.5%, respectively, in children of parents without and with atopy, whereas the prevalence of baby swimming was 5.6% and 5.1%, respectively, in the two groups. Stratified analysis using parental atopy as strata showed that the increased risk of recurrent respiratory tract infections was only present among children of parents with atopy [adjusted odds ratio (aOR) 2.08, 95% confidence interval (95% CI) 1.08–4.03]. A similar trend was present for otitis media (aOR 1.77, 95% CI 0.96–3.25). Conclusion: The results of this study suggest that baby swimming and infant respiratory health may be linked. The findings need to be examined in a longitudinal study.
Nutritional status of mentally retarded children in northwest Spain: II. Biochemical indicators
Authors: Sánchez‐Lastres J , Eirís‐Puñal J , Otero‐Cepeda JL , Pavón‐Belinchón P , Castro‐Gago M 
Published: August 2010

Aim: To evaluate the nutritional status of mentally retarded children in the region of Galicia, northwest Spain, on the basis of serum biochemistry variables. Methods: The following serum biochemistry variables were determined in a sample of 128 mentally retarded children (81 boys and 47 girls): albumin, prealbumin, retinol‐binding protein, transferrin, ferritin, ceruloplasmin, iron, calcium, phosphorus, copper, total cholesterol, HDL cholesterol, LDL cholesterol, triglycerides, alkaline phosphatase, transaminases and carnitine. A preliminary statistical analysis indicated that most information content could be maintained taking into consideration only five of these variables. A factor analysis of the resulting 5 × 128 variables‐by‐subjects data matrix was then performed, identifying three factors (FB1, FB2 and FB3) that together explained 74% of total variance. Taking these factors as indicators of nutritional status, the data were then analysed for possible effects of age, gender, socioeconomic and family environment, IQ, presence/absence of cerebral palsy, quality of diet, appetite and use of antiepileptics. Results: The analysis suggests that most subjects were in the normal nutritional range, but that about 37% showed either borderline or definite malnutrition. The FB1 score showed a significant, positive correlation with age, while FB2 score was negatively correlated with age. The FB1 score showed a significant positive correlation with socioeconomic class, and was significantly higher among children from inland‐rural areas than among children from coastal or urban areas. The FB1 score was also positively correlated with parents’mean age. Finally, the results suggest that antiepileptic treatment with phenobarbital or diphenylhydantoin led to reductions in serum levels of calcium and phosphorus. Conclusion: Malnutrition as revealed by biochemical variables is highly prevalent among mentally retarded children, and the prevalence is affected by socioeconomic and family environment variables.
Recovery of metabolic acidosis in term infants with postasphyxial hypoxic‐ischemic encephalopathy*
Authors: Shah PS , Raju NV , Beyene J , Perlman M 
Published: August 2010

Aims: To describe the base deficit (BD) values and the rate of postnatal recovery of the BD of infants with hypoxic ischemic encephalopathy due to intrapartum asphyxia; and to explore the relationships between the rate of recovery of BD, severe adverse outcome and different time patterns (acute total vs prolonged partial) of asphyxia. Methods: Clinical and laboratory data were collected from the neonatal period (n= 244) and outcome data to a minimal age of 1 y (n= 218). Rates of recovery of BD were described in four 60 min blocks of time. The values of rate of recovery were compared between the outcome groups, ignoring correlation structure within subjects and with adjustment by the generalized estimating equations method. Results: The BD normalized within 4 h of birth in all but 9 of 244 infants. The rates of recovery of BD in infants with good and severe adverse outcome respectively were 0.11 [95% confidence interval (95% CI) 0.07, 0.14] and 0.09 (95% CI 0.07, 0.12) mmol l‐1 min‐1 over the first 4 h after birth. The rates of recovery were similar with or without buffer therapy, and after acute near‐total and prolonged partial asphyxia. Conclusion: The BD in the great majority of infants with severe intrapartum asphyxia normalizes within 4 h of birth. The BD recovery rate of infants with adverse outcomes was similar to those with relatively good outcome. The different time patterns of asphyxial episode were not associated with differential recovery profiles.