bluuebunny/crossref_metadata_2025_split huggingface dataset:
Expert consensus in the development of a European health‐related quality of life measure for children and adolescents: a Delphi study
Authors: Herdman M , Rajmil L , Ravens‐Sieberer U , Bullinger M , Power M , Alonso J ,   The European Kidscreen and Disabkids groups
Published: August 2010

Aim: To determine the level of consensus among experts regarding content, structure and sources of content for a new European measure of health‐related quality of life in children and adolescents. Methods: A three‐round Delphi questionnaire was sent by e‐mail to 24 experts in quality of life measurement in 9 European countries. Consensus was considered reached when ≫90% of experts either agreed or disagreed with a given statement, or where median scores were over 6 on a scale of 1–10, and score dispersion was within predefined limits. Results: Completed questionnaires were received from 20 panellists in each round. It was agreed that the new instrument should be a multidimensional, profile measure with 30–49 items covering 5–8 dimensions, which should take no more than 10–15 min to complete. Agreement was also reached on 8 specific dimensions to include in the questionnaire (psychological well‐being, self‐esteem, body image, cognitive functioning, mobility, energy/vitality, social relations, family/home function). Consensus was against the use of individualized questionnaires. Focus groups with children, parents and workers in the field, literature and instrument reviews were considered appropriate sources for content. Conclusion: Using a Delphi method in this way for the first time showed it to be a feasible and useful method for establishing a conceptual and operational framework for the Kidscreen questionnaire.
Homocysteine and other vascular risk factors in patients with phenylketonuria on a diet
Authors: Schulpis KH , Karikas GA , Papakonstantinou E 
Published: August 2010

The aim of this study was to investigate the known risk factors, such as lipids, homocysteine and endothelin, for the development of coronary artery disease (CAD) in phenylketonuria (PKU) patients, depending on their diet. The PKU patients (n= 74) were divided into two groups. Group A (n= 34; mean age 6.78 ± 1.5 y) adhered strictly to a diet and group B (n= 40; mean age 8.0 ± 3.2 y) did not comply with the diet. The control group comprised 50 healthy non‐PKU children. All groups were evaluated for blood levels of homocysteine and vitamin B6 by high‐performance liquid chromatography, vitamin B12 and folate in serum by a radioassay, lipids by a routine method, and lipoprotein(a) and endothelin‐1 with an immunoassay. Homocysteine levels (28.65 ± 3.3 μmol 1‐1) were increased in group A compared with group B (6.86 ± 1.6 μmol 1‐1) and the controls (6.9 ± 2.0 μmol 1‐1) (p > 0.001). Vitamin B6 (10.7 ± 10.9 nmol 1‐1), vitamin B12 (98.5 ± 22.3 pmol 1‐1), folate (2.35 ± 1.3 nmol 1‐1) and lipids were decreased in group A. The other vascular risk factors, which were not dependent on diet [lipoprotein(a) and endothelin‐1], did not differ among the three groups. Conclusion: PKU patients on a strict diet had low vitamin B6, vitamin B12 and folate levels resulting in moderate hyperhomocysteinaemia. The evaluation of these vitamins at short intervals and their supplementation could be an early measure in the prevention of CAD.
Neonatal hip instability: results and experiences from ten years of screening with the anterior‐dynamic ultrasound method
Authors: Andersson JE 
Published: August 2010

Aim: To record the results and experiences from a 10‐y screening period with the anterior‐dynamic ultrasound method for detecting neonatal hip instability. Methods: An ultrasonographic improvement of the Palmen/Barlow test was used. The screening programme included 22 047 newborns. Decisions about treatment were made solely on the ultrasound result. Results: It was found that 175 infants (7.9/1000) had at least one unstable hip—dislocated or dislocatable. Dislocated hips were found in 1.1/1000. Dislocatable hips were found in 6.8/1000 but only 1.1/1000 needed treatment. The total frequency of treatment was 2.2/1000. All cases but one were diagnosed before discharge from the maternity ward. The rate of surgery was 0.1/1000 newborns. Girls were more affected than boys, by a ratio of 3:1. Among the affected hips 64.4% were a left hip. Conclusion: Neonatal hip instability is always present at birth and can be diagnosed immediately after birth. We have no indications that instability can appear at a later stage. The anterior‐dynamic ultrasound screening programme is an efficient tool to diagnose neonatal hip instability and to decide when to begin treatment.
Low birth weight children: coping in school?
Authors: Elgen I , Sommerfelt K 
Published: August 2010

Aim. To describe and compare school performance and IQ at 11 y of age in a population of 130 children weighing less than 2000 g at birth without any major handicaps (low birthweight) and a random control sample of 131 children born at term weighing over 3000 g (normal birthweight); and to evaluate the relative strength of parental factors versus child birthweight in predicting IQ. Methods. The mothers and teachers completed validated questionnaires addressing school performance and the child's IQ was evaluated by WISC‐R, prorated. In addition, socioeconomic status was investigated using different questionnaires. Results. According to maternal reports, twice as many low birthweight children had school problems and three times as many of these children were referred to the School Psychological Service. Mean prorated IQ was 5 points lower in the low birthweight group. No statistically significant difference was found for mean IQ between the groups with birthweights of less than 1500 g vs 1500–2000 g. In a multivariate linear regression analysis, parental factors accounted for 13% of the variance in child IQ compared with only 3% accounted for by child birthweight. Conclusion. Low birthweight significantly increases the risk of school problems.
Leptin levels in breast‐fed and formula‐fed infants
Authors: Savino F , Costamagna M , Prino A , Oggero R , Silvestro L 
Published: August 2010

Aim: Leptin, a hormone that regulates food intake and energy metabolism, is present in breast milk and thus may be involved in body composition differences between breastfed and formula‐fed infants. The aim of this study was to evaluate whether diet and gender affect plasma leptin concentration in breastfed and formula‐fed infants during the first months of life. Methods: Anthropometric and bioelectrical impedance measurements [total body water (TBW) calculated with the Fjeld equation] were made and venous blood plasma samples were analysed for leptin concentration in healthy, exclusively breastfed or formula‐fed Italian infants in the first year of life. Infants were subdivided in two ways: three groups (periods) in relation to age, and five groups in relation to weight. Results: The average serum concentration of leptin was 7.35 ng ml‐1. Serum leptin values were higher in breastfed than in formula‐fed infants. Breastfed infants in group 1 had a statistically higher serum leptin concentration (2500–3749 g). There were no significant differences in anthropometric measurements, body mass index or skinfold thickness between breastfed and formula‐fed infants. In the periods I and II, breastfed infants had a significantly higher TBW than formula‐fed infants. Males had a significantly higher TBW than females in periods I and II. Breastfed infants in group 2 (3750–4999 g) had a significantly higher TBW than formula‐fed infants. Conclusion: The data on TBW, weight and skinfold thickness suggest that the higher leptin concentration observed in breastfed infants in the first months of life may be due not only to adipose tissue production but also to human milk.
Postnatal adaptation after Caesarean section or vaginal delivery, studied with the static‐charge‐sensitive bed
Authors: Nikkola EM , Kirjavainen TT , Ekblad UU , Kero PO , Salonen MAO 
Published: August 2010

Aim: To compare postnatal adaptation between Caesarean and vaginal deliveries, by studying sleep states, oxygenation, heart rate and body movements. Another aim was to follow the adaptation of healthy, term, vaginally born babies. Methods: Ten vaginally born and 12 neonates born by elective Caesarean section were recorded with a movement sensor (SCSB, static‐charge‐sensitive bed), electrocardiogram and oximeter. The recordings started 1.5 h after birth and lasted for 12 h. For the vaginal group, another 12 h recording was performed during the third night postpartum. Results: Delivery mode did not affect sleep state distribution. The vaginal group had more oxyhaemoglobin desaturation episodes >95% than the Caesarean section group (mean ± SD: 59 ± 10% vs 42 ± 22% of epochs, p= 0.03), especially in active sleep, but baseline saturation was similar (96 ± 1% vs 95 ± 3%, p= 0.93). The vaginal group had fewer movements during sleep than the Caesarean section group (movements of 5–10 s: 5 ± 1 h‐1 vs 10 ± 3 h‐1p= 0.0001). During the first 3 d, the amount of sleeping and active sleep increased, whereas wakefulness and quiet sleep decreased. Baseline oxyhaemoglobin saturation and the number of movements of over 5 s increased. Conclusion: Delivery mode did not affect sleep state distribution but, unexpectedly, the vaginal group had more oxyhaemoglobin desaturation events and fewer body movements than the Caesarean section group. These differences during the first postnatal day remain unexplained, but they may reflect stress and pain during labour. After a few days, changes in sleep organization, and increases in oxyhaemoglobin saturation and frequency of body movements were noted in the vaginal group, which may represent recovery and adaptation to extrauterine life.
Effect of blood transfusions on cerebral haemodynamics in preterm infants
Authors: Dani C , Pezzati M , Martelli E , Prussi C , Bertini G , Rubaltelli FF 
Published: August 2010

Aim: To assess the possible cerebral haemodynamic changes occurring in preterm infants after blood transfusions. Methods: Preterm infants who had undergone blood transfusions were prospectively studied using both near infrared spectroscopy and cerebral Doppler ultrasonography. Results: Fourteen infants (mean gestational age 29.6 wk, SD 2.6; mean birthweight 1430 g, SD 332) were studied at the mean age of 29 (SD 14) d. A significant increase in oxygenated haemoglobin (O2Hb), deoxygenated haemoglobin (HHb), mixed cerebral oxygen saturation (SmO2) and change in cerebral blood volume occurred after transfusion. Between ultrasound parameters, we found a decrease in diastolic velocity and an increase in resistance index. Conclusion: Blood transfusions improve cerebral oxygen supply and induce a decrease in cerebral blood volume, probably due to an increase in cerebral vessel resistance.
Hyperphagia, weight gain and neonatal drug withdrawal
Authors: Shephard R , Greenough A , Johnson K , Gerada C 
Published: August 2010

Hyperphagia, a classical feature of neonatal drug withdrawal, has been reported not to lead to excessive weight gain, but this is contrary to our clinical experience. The aim of this study was to determine whether infants with neonatal drug withdrawal suffered excessive weight gain because of hyperphagia and, if so, to determine the risk factors. The study population comprised 48 infants consecutively admitted to the neonatal intensive care unit, 11 of whom gained weight by more than 20 g kg‐1 d‐1 for at least 10 d (excessive weight gain). All 11 infants were hyperphagic (>200 ml/kg) for at least part of the excessive weight gain period. During the perinatal period, the 11 infants had a greater fluid intake (p > 0.01) but similar weight gain to gestational‐age‐matched, neonatal drug‐withdrawal infants who did not suffer any excessive weight gain. Compared to the rest of the cohort, the infants with excessive weight gain were more likely to require treatment with morphine/chlorpromzaine (p > 0.05) and had a higher maximum withdrawal score (p > 0.01). Conclusion: Hyperphagia can lead to excessive weight gain in infants with neonatal drug withdrawal. Our results suggest that hyperphagia occurs in those who require treatment for severe withdrawal.
Final height in psychosocial short stature: is there complete catch‐up?
Authors: Gohlke BC , Stanhope R 
Published: August 2010

Aim: To determine whether children with psychosocial short stature attain their genetic height potential. Methods: We report on 18 children (10 girls, 8 boys) diagnosed by a multidisciplinary team as having psychosocial short stature. All the children had had some kind of change in their environment (9 were separated from their families), and increased their mean height velocity standard deviation score (SDS) from −0.7 (1.3) to +3.6 (4.8) (p > 0.005) as well as their height SDS from −3.0 (0.3) to −2.6 (0.9) in the first year after the change. All the patients were post‐pubertal and had reached their near final height (mean age, 20.0 y; range, 16.0–23.3). Results: Only 3 out of 18 had a greater final height than the mid‐parental target height, 14 out of 18 had a near final height within the mid‐parental target range (95% tolerance limits of the mid‐parental height (±2 SD =±10 cm). Nevertheless, mean final height expressed in height SDS for the whole group was significantly shorter with −2.4 SDS compared with the mean of the mid‐parental target height of −1.5 SDS (p > 0.001). Surprisingly, initial catch‐up growth did not correlate with final height attainment. Conclusion: The majority of patients will attain a stature within the range of mid‐parental target height, although towards the lower limit of this range.
Reversible brain lesions in childhood hypertension
Authors: Singhi P , Subramanian C , Jain V , Singhi S , Ray M 
Published: August 2010

Posterior leukoencephalopathy syndrome is characterized by an acute, usually reversible, encephalopathy with transient occipital lobe abnormalities detected on MRI that occur mostly in association with acute hypertension. The clinical presentation includes seizures, headache, altered mental status and blindness. Disturbed autoregulation of cerebral blood flow and endothelial injury are central to the pathogenesis of this disorder. Prompt control of hypertension results in rapid and complete neurological recovery. In this report we discuss the cases of two children with acute onset hypertension of different aetiologies that presented with the characteristic features of posterior leukoencephalopathy syndrome. Conclusion: Early recognition of this readily treatable condition may obviate the need for extensive and invasive investigations. Despite the alarming lesions on the MRI, prompt control of hypertension carries a uniformly favourable prognosis.
Developmental biology of thrombopoietin in the human fetus and neonate
Authors: Dame C 
Published: August 2010

This review focuses on the biology of thrombopoietin (Tpo) during human development. It summarizes the current understanding on molecular biological aspects of Tpo, cellular effects of Tpo on fetal and neonatal megakaryopoiesis, regulation of Tpo production, and circulating Tpo concentrations in human fetuses and neonates. Some important aspects on the developmental biology of Tpo are highlighted. They include the finding of high Tpo gene expression in the bone marrow during the onset of medullary hematopoiesis, higher circulating Tpo concentrations in fetuses and neonates than in children or adults, and a higher sensitivity of neonatal megakaryocyte progenitor cells to Tpo. However, other aspects of the developmental biology of Tpo are incompletely understood. Conclusion: More carefully designed studies are needed to provide the necessary background for an optimal treatment of fetal and neonatal thrombocytopenia.
Evaluation and treatment of thrombocytopenia in the neonatal intensive care unit
Authors: Murray NA 
Published: August 2010

Phlebotomy‐induced anaemia excepted, thrombocytopenia is the most common haematological abnormality in neonatal intensive care unit (NICU) patients. Roughly one‐quarter of all NICU patients and half of all sick preterm neonates develop thrombocytopenia. Whereas a large number of varied precipitating conditions has been identified, early‐onset thrombocytopenia (>72 h) is most commonly associated with fetomaternal conditions complicated by placental insufficiency and/or fetal hypoxia, e.g. maternal pre‐eclampsia and fetal intrauterine growth restriction. The resulting neonatal thrombocytopenia is usually mild to moderate, resolves spontaneously and requires no specific therapy. Deviation from this pattern of thrombocytopenia suggests the presence of more significant precipitating conditions. The most important of these are the immune thrombocytopenias, and every NICU should develop investigation and treatment protocols to manage these cases promptly and avoid unnecessary risk of haemorrhage. In contrast, late‐onset thrombocytopenia (>72 h) is almost always associated with sepsis or necrotizing enterocolitis and the associated thrombocytopenia is severe, prolonged and often requires treatment by platelet transfusion. Unfortunately, evidence‐based guidelines for platelet transfusion therapy in NICU patients are currently unavailable, making it difficult to define widely accepted thresholds for transfusion and leading to a significant variation in transfusion practice between centres. Conclusion: While improving this situation remains a pressing need, the growing evidence that impaired megakaryocytopoiesis and platelet production are major contributors to many neonatal thrombocytopenias suggests that recombinant haemopoietic growth factors, including thrombopoietin and interleukin‐11, may be useful future therapies to ameliorate neonatal thrombocytopenia.
Inflammatory mediators in perinatal asphyxia and infection
Authors: Xanthou M , Fotopoulos S , Mouchtouri A , Lipsou N , Zika I , Sarafidou J 
Published: August 2010

Aim: To determine serum levels of interleukin‐6 (IL‐6), IL‐1β, tumor necrosis factor‐α (TNF‐α), soluble intercellular adhesion molecule‐1 (sICAM‐1) and C‐reactive protein (CRP) in asphyxiated neonates and compare these inflammatory factors with those found in neonates with perinatal infection. Methods: 88 neonates were studied, of whom 36 were asphyxiated, 18 were infected and the remaining 34 were controls. Peripheral blood samples were obtained on the 1st, 3rd and 5th postnatal days. Results: Cytokines IL‐6 and IL‐1β as well as sICAM‐1 serum levels did not differ between asphyxiated and infected neonates; however, at most time periods, their values were significantly higher than controls. TNF‐α was similar in the three groups of neonates. CRP serum values were significantly higher in the infected neonates than in the asphyxiated or control subjects. Among the 54 asphyxiated and infected neonates, 15 were considered as severe cases and 39 as mild. The severe cases, at most time periods, had significantly higher IL‐6, IL‐1β and sICAM‐1 levels compared with the mild ones. Through receiver operating characteristic curves the cut‐off points, sensitivities, and specificities for distinguishing neonates at risk or at high risk for brain damage were established. Conclusion: Similar increases in serum levels of IL‐6, IL‐1β and sICAM‐1 were found in perinatally asphyxiated and infected neonates. As these increases correlated with the severity of the perinatal insults, neonates at high risk for brain damage might be detected.
Late‐onset neutropenia in very low birthweight infants
Authors: Chirico G , Motta M , Villani P , Cavazza A , Cardone ML 
Published: August 2010

Aim: To evaluate the incidence and duration of late‐onset neutropenia (defined as an absolute neutrophil count (ANC) <1500 mm−3 at a postnatal age of >3 wk) in a population of infants with birthweight <2000 g, and to determine whether copper deficiency, a possible cause of both anemia and neutropenia, may be associated with this complication. Methods: Complete blood cell count and differential were assessed in 247 low (LBW) and very low birthweight (VLBW) infants who were discharged after 3 wk of life. In neutropenic infants plasma copper and ceruloplasmin levels were also measured. Results: Late‐onset neutropenia was detected in 11 out of 147 VLBW infants (7.5%) and in 7 out of 127 LBW infants (5.5%). A neutrophil count of <1000 mm−3 was observed in 14 infants (5.1%). A significantly lower gestational age was found in neutropenic infants compared with non‐neutropenic infants. In neutropenic infants ANCs were significantly correlated with hemoglobin and hematocrit. In addition, a significant negative correlation was found between neutrophil and reticulocyte counts. Plasma copper concentration was significantly correlated with birthweight. Oral copper sulfate was administered to infants with plasma copper concentration <50 μg dl−1, and did not seem to affect ANC, hemoglobin, hematocrit or reticulocyte counts. Conclusion: Late‐onset neutropenia appears to be a benign condition that is not associated with any particular complication and does not require specific treatment. Reference ranges after the early neonatal period and during the first few months of life in LBW and VLBW infants should probably be set at lower values.
Aetiology and pathogenesis of hypertrophic cardiomyopathy
Authors: Lombardi R , Betocchi S 
Published: August 2010

The term hypertrophic cardiomyopathy is used to describe an autosomal dominant cardiac disorder, characterized by myocyte hypertrophy and disarray, interstitial fibrosis and small vessel disease, with or without macroscopic hypertrophy. More than 100 mutations in ten genes, all encoding sarcomeric proteins, have been identified as responsible for this disease. Mutations in the genes for β‐myosin heavy chain, myosin binding protein‐C, and cardiac troponin T are the most common. Other genes involved are α‐tropomyosin, cardiac troponin‐I, essential and regulatory light chains, α‐cardiac actin, titin, and α‐myosin heavy chain. Some mutations are more frequently associated with a given phenotype, but no particular phenotype is mutation specific; in fact, some mutations exhibit highly variable clinical, electrocardiographic and echocardiographic manifestations. This variability in the phenotypic manifestations is probably due to the influence of environmental factors and/or modifier genes. While the aetiology of hypertrophic cardiomyopathy has been extensively elucidated, its pathogenesis is not completely understood. Mutated proteins are incorporated in the sarcomere and impair myocyte contractility. This probably triggers the compensatory local release of trophic factors, which influence the development of the typical anatomical features of the disease, with a pathway similar to that observed in secondary, pressure overload hypertrophy. Conclusions: The various pathological cardiac changes seen in hypertrophic cardiomyopathy are probably due to a compensatory response to impaired myocyte function resulting from mutations in the genes encoding sarcomeric proteins.