bluuebunny/crossref_metadata_2025_split huggingface dataset:
Bone mineral content and collagen metabolites in children receiving steroid treatment for nephrotic syndrome
Authors: Esbjörner E , Arvidsson B , Jones IL , Palmér M 
Published: August 2010

Bone mineral density (BMD) and content (BMC) were measured in nine children treated with corticosteroids for nephrotic syndrome and in age‐matched controls, using dual‐energy X‐ray absorptiometry (DEXA). The urinary excretion of cross‐linked N‐telopeptide (NTx) released from collagen type I as a specific marker of bone resorption was also measured. There were no significant differences in body size, BMD results or NTx urinary concentrations between patients and controls, nor could any significant differences be found when the six patients given a cumulative corticosteroid dose of < 15 g were analysed separately. The lack of significant differences could be due to the small number of patients included in the study. But when the measured BMD and BMC were analysed according to methods that corrected for body size and puberty stage, values well within the normal range were found in patients as well as in controls. There was, however, a significant, negative correlation between the urinary excretion of NTx and the cumulative dose of corticosteroids. Conclusion: Despite treatment for long periods with high, cumulative doses of corticosteroids, the skeletons of the patients had a normal mineral content, which is encouraging for all those in need of steroids for nephrotic syndrome. A negative correlation between urinary collagen degradation products and the cumulative steroid dose might point to a reduced growth velocity in patients on high doses of steroids earlier than an effect on bone mineralization.
Bibliometric data: a disaster for many non‐American biomedical journals
Authors: Zetterström R 
Published: August 2010

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Pœdiatrica, has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American‐oriented research projects at the expense of those that are based on concepts of predominantly European relevance. Conclusion: For the future of non‐American research, it is important that no single super‐power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.
Exhaled and nasal nitric oxide in mechanically ventilated preterm and term newborns
Authors: Aikio O , Pokela ML , Hallman M 
Published: August 2010

Aim: Nitric oxide (NO) is an important mediator required for neonatal pulmonary circulatory adaptation and for pulmonary defence. Both deficient and excessive NO production have been proposed to play a role in neonatal lung disease. This study aimed to establish a method that allows direct measurement of exhaled and nasal NO concentrations in newborn infants who require intubation and ventilation.Methods: A rapid‐response chemiluminescence NO analyser was used. Gas was sampled from the endotracheal intubation tube, and tidal volumes and flow rates were measured. The nasal NO was sampled from the non‐intubated nostril. The accuracy of the method was validated using a lung model. NO levels from six preterm and six term/near‐term newborns were studied. Measurements were performed on a daily basis during the first week.Results: An expiration >0.2 s in duration with a flow rate >1.7mls−1could be accurately analysed for the presence of >1 parts per billion of NO. The very preterm infants with neonatal lung disease had a different postnatal NO output pattern from the lower and upper airways compared with the ventilated term/near‐term infants.Conclusion: A novel method for measurement of exhaled NO of an intubated newborn is presented. The possible association of exhaled NO concentration with the development of chronic lung disease remains to be studied.
Effects of gender, apolipoprotein E phenotype and cholesterol‐lowering by plant stanol esters in children: The STRIP study
Authors: Tammi A , Rönnemaa T , Miettinen TA , Gylling H , Rask‐Nissilä L , Viikari J , Tuominen J , Marniemi J , Simell O 
Published: August 2010

Aim: To evaluate the effects of gender, apolipoprotein E phenotype and cholesterol absorption and synthesis (estimated as serum plant sterol and cholesterol precursor sterol concentrations) on the cholesterol‐lowering effect of plant stanol esters in children. Methods: Eighty‐one healthy, normocholesterolaemic 6‐y‐old children (45 boys) were recruited from the Special Turku Coronary Risk Factor Intervention Project (STRIP), a randomized prospective trial aiming at atherosclerosis prevention in childhood. This placebo‐controlled, double‐blind, cross‐over study comprised two 3‐mo study periods and a 6‐wk wash‐out period. During the study periods, 20 g of the children's daily dietary fat intake was replaced with plant stanol ester margarine or control margarine. Results: In boys, plant stanol esters reduced serum total and low‐density lipoprotein cholesterol concentrations by 6% (0.09 to 0.42mmol/L) and 9% (0.09 to 0.36mmol/L), respectively (p > 0.01 for both). In girls, the decreases in concentrations were 4% (0.03 to 0.38 mmol/L) and 6% (0.02 to 0.32 mmol/1) (p > 0.05 for both). The response rate did not differ between the genders. Serum total and low‐density lipoprotein cholesterol concentrations decreased by 6% and 8% (p > 0.01 for both), respectively, in both children with the apolipoprotein E 3/4 or 4/4 (apoE4+) phenotype and the apolipoprotein E 2/3 or 3/3 (apoE4—) phenotype. Cholesterol absorption decreased both in the apoE4+ children and in the apoE4— children, but cholesterol synthesis consistently increased in the apoE4+ children only. Conclusion: Plant stanol esters reduce serum cholesterol concentration in healthy children irrespective of their gender or apoE4 phenotype.
Pathogenesis of retinopathy of prematurity
Authors: Smith LEH 
Published: August 2010

Retinopathy of prematurity (ROP) is a blinding disease, initiated by delayed retinal vascular growth after premature birth. There are both oxygen‐regulated and non‐oxygen‐regulated factors, which contribute to both normal vascular development and retinal neovascularization. One important oxygen‐regulated factor, critical to both phases of ROP, is vascular endothelial growth factor (VEGF). A critical non oxygen‐regulated growth factor is insulin‐like growth factor (IGF‐1). In knockout mice, lack of IGF‐1 prevents normal retinal vascular growth, despite the presence of VEGF, important to vessel development. In vitro, low IGF‐1 prevents vascular endothelial growth factor‐induced activation of Akt, a kinase critical for vascular endothelial cell survival. Premature infants who develop ROP have lower levels of serum IGF‐1 than age‐matched infants without disease. Conclusion: IGF‐1 is critical to normal vascular development. Low IGF‐1 predicts ROP and restoration of IGF‐1 to normal levels may prevent ROP.
Craniofacial morphology in preschool children with sleep‐related breathing disorder and hypertrophy of tonsils
Authors: Kawashima S , Peltomäki T , Sakata H , Mori K , Happonen R‐P , Rönning O 
Published: August 2010

The purpose of this study was to examine craniofacial morphology, pharyngeal airway space and hyoid bone position in preschool children with sleep‐related breathing disorder associated with hypertrophy of tonsils (SBDT). Thirty‐eight preschool children, mean age 4.7 y, with SBDT and with an apnoea index (AI) of 0 < AI <5, were divided into two groups. One consisted of 15 children with sleep‐related breathing disorder (SBD) and more than 75% of the tonsils visible (GUI) and the other of 23 children with SBD and 25–75% of the tonsils visible (Gil). The control group consisted of 31 children without ear, nose and throat disease and with GI (barely visible) tonsils. Compared with the controls, GUI children had a retrognathic mandible, a large posterior facial height, a large interincisal angle with retroclined lower incisors, a narrow pharyngeal airway space, an anterior tongue base position and a long soft palate. Compared with the controls, Gil children had a large anterior lower facial height and a short nasal floor. However, like the controls, Gil children did not have a retrognathic mandible. Conclusion: The findings show that children with SBDT display a characteristic facial appearance at an early age. Since the condition has an effect on growth, it needs to be prevented by controlling morphology and function at the preschool age.
Alteration of biochemical profiles after high‐dose intravenous immunoglobulin administration in Kawasaki disease
Authors: Lee KY , Han JW , Lee JS , Whang KT 
Published: August 2010

Intravenous immunoglobulin (IVIG) has been used as an immunomodulatory treatment for several immune‐mediated diseases. Early effects of high‐dose IVIG treatment on biochemical profiles including lipids and proteins were evaluated in patients with Kawasaki disease (KD). Twelve children with KD (9 boys) were treated with IVIG at 2 g/kg over a period of 12 h. Serial sera samples were collected from the patients four times: before IVIG treatment and 2 h, 24 h and 7 d after IVIG treatment. The samples were frozen at –20°C before biochemical analysis. A significant decrease in albumin concentration was found 2 h and 24 h after IVIG treatment, but this recovered to the pretreatment level after 7 d. Total cholesterol and triglyceride increased slightly after 7 d. A significant decrease in HDL‐cholesterol and C‐reactive protein was seen 2 h and 24 h after IVIG treatment. Conclusion: High‐dose IVIG affects immediate changes in protein profiles and HDL‐cholesterol in KD. Changes in HDL‐cholesterol induced by IVIG may be the result of changes in systemic protein metabolism.
Factor V 1691 G‐A mutation in children with intracardiac thrombosis: a prospective study
Authors: Atalay S , Akar N , Tutar HE , Ylmaz E 
Published: August 2010

The objective of this study was to determine the association between intracardiac thrombosis and hereditary causes of thrombophilia, including factor V 1691 G‐A (factor V Leiden, FVL) and prothrombin 20210 G‐A mutations. Over a period of 3 y, genetic risk factors were evaluated in 13 consecutive children (mean age 6.27 ± 5.44 y) with intracardiac thrombosis, diagnosed by cross‐sectional echocardiography. Thrombi were localized in the left heart in four patients and the right heart in nine patients. All children had predisposing factors for thrombus formation: ventriculoatrial shunt for hydrocephalus (n= 3), indwelling catheter for chemotherapy (n= 5), cardiomyopathy (n= 2), sepsis (n= 1), homocystinuria (n= 1) and tetralogy of Fallot (n= 1). Six of the 13 children with intracardiac thrombosis were heterozygotes for FVL mutation. Three of these six children with FVL mutation had ventriculoatrial shunt for hydrocephalus, two children had cardiomyopathy and one had sepsis. None of the patients carried the prothrombin 20210 G‐A mutation. Conclusion: It is recommended that FVL mutations should be investigated in all cases of intracardiac thrombosis irrespective of whether or not a predisposing factor is identified. When a predisposing factor is found antithrombotic prophylaxis may be considered in patients carrying the FVL mutation.
Liver involvement in alpha1‐antitrypsin‐deficient phenotypes PiSZ and PiMZ
Authors: Pittschieler K 
Published: August 2010

The long‐term outcome of 3 PiSZ and 57 PiMZ children affected by alpha1‐antitrypsin deficiency‐associated liver involvement during infancy was assessed. At 5 and 10 y of age all PiSZ and PiMZ children exhibited normal levels of liver enzymes. All families, except one, were deterred from smoking following counselling. Conclusion: Transient liver involvement observed during early infancy is likely to be a benign self‐limiting process.
Determinants of linear growth and predictors of severe stunting during infancy in rural Malawi
Authors: Espo M , Kulmala T , Maleta K , Cullinan T , Salin M‐L , Ashorn P 
Published: August 2010

Stunting is common among children under 5 y of age in sub‐Saharan Africa. Several risk factors have been associated with poor growth but few studies have prospectively addressed the development of linear growth faltering and stunting during the first year of life. The present study was designed to analyse typical growth among rural Malawian infants, focusing particularly on the impact of birth size, adherence to feeding guidelines and morbidity in the development of severe stunting during infancy. A community‐based cohort of 613 singleton newborns was prospectively followed by monthly home visits. Data were collected on the children's socioeconomic background, maternal size and weight gain during pregnancy, birth events, morbidity, breastfeeding and complementary feeding, growth and mortality. Univariate and multivariate analyses were used to determine associations between predictor variables and poor linear growth. The proportions of stunted infants (Height‐for‐age Z‐score > –2) at 3, 6 and 9 mo of age were 27%, 51%, and 63%, respectively. At 1 y of age, over two‐thirds (71%) of the infants were at least moderately (HAZ > –2) and 31% severely stunted (HAZ > –3).Conclusion: The strongest predictor of severe stunting at 12 mo of age was small birth size. Other variables independently associated with this outcome included inappropriate complementary feeding, high morbidity, maternal short stature, male gender, and home delivery. Faltering of linear growth started soon after birth and continued throughout infancy. Interventions increasing birth size could have a significant role in the prevention of early childhood stunting. The ideal strategy should also emphasize the importance of appropriate infant feeding and decreasing the number of illness episodes amongst the infants.
Early‐onset Group B streptococcal sepsis in a preterm infant with Kostmann syndrome
Authors: Fujiu T , Maruyama K , Koizumi T 
Published: August 2010

A preterm infant died of group B streptococcal sepsis 7 h after birth. The infant's complete blood count showed total agranulocytosis. Histopathology of the major organs showed significant bacterial invasion without infiltration of polymorphonuclear leucocytes. Examination of the bone marrow revealed normal cellularity of the granulocyte precursors with arrested maturation. These findings are consistent with Kostmann syndrome. Conclusion: It is suggested that in patients with deteriorating early‐onset infection, underlying congenital abnormalities in host defence, such as Kostmann syndrome, should be considered.
Neurobehavioral and developmental profile of very low birthweight preterm infants in early infancy
Authors: Wolf M‐J , Koldewijn K , Beelen A , Smit B , Hedlund R , de Groot IJM 
Published: August 2010

Aim: To describe the neurobehavioral and developmental profile of very low birthweight (VLBW) preterm infants in early infancy. Methods: Twenty VLBW infants and 10 term control infants were assessed at term, 3 and 6 mo of age. Neurobehavioral assessments included the Neonatal Behavioral Assessment Scale (NBAS) at term; the Infant Behavioral Assessment at term, 3 and 6 mo of age and the Behavioral Rating Scale of the Bayley Scales of Infant Development—II (BSID‐II) at 3 and 6 mo of age. Development was evaluated with the Bayley Motor and Mental Scale at 3 and 6 mo. Results: At term age VLBW infants differed from term infants on all the clusters and supplementary items of the NBAS. VLBW infants also showed more stress and less approach behavior at term and 6 mo of age and more problems with self‐regulation in all subsystems at 6 mo of age. Moreover, VLBW infants performed lower on the Bayley Motor, Mental and Behavioral Rating Scale: 12 VLBW infants scored questionable or non‐optimal on the Psychomotor Development Index and 18 questionable or non‐optimal on the Behavioral Rating Scale. These results support the need for neurobehavioral intervention of VLBW infants in the first 6 mo of life. Conclusion: Almost all VLBW infants showed non‐optimal motor quality behavior at 6 mo and encountered far more problems with self‐regulation compared with term infants.
Efficacy and safety of a home‐made non‐valved spacer for bronchodilator therapy in acute asthma
Authors: Duarte M , Camargos P 
Published: August 2010

The aim of this study was to assess the efficacy and safety of an alternative spacer to deliver salbutamol in acute asthma. A prospective randomized study was performed with 196 patients with acute asthma, aged 4–15 y. The clinical features, oxygen saturation (S aO2) and the best of three peak expiratory flow rate (PEFR) measurements were recorded in a standardized questionnaire. Each patient received up to three conventional doses of salbutamol through an alternative homemade non‐valved spacer (NVS) (500 ml mineral water plastic bottle) or an oxygen‐driven nebulizer (ODN). The clinical and functional respiratory parameters (PEFR and S aO2) were assessed 15 min after each administration of the bronchodilator. In the ODN (n= 99) and NVS (n= 97) groups, PEFR (p < 0.05) and S aO2 (p < 0.05) measurements improved in all stages of the treatment. During the asthma attacks, the ODN group needed a prolonged observation in the emergency room (p= 0.000000). Side effects of salbutamol, e.g. increased heart rate, were observed in 17.2% of the patients in the ODN group and 4.1% in the NVS group (p= 0.003). Conclusion: Both devices showed comparable efficacy in mild and moderate acute attacks, but the use of the alternative NVS resulted in a shorter observation period in the emergency room. The frequency of side effects was significantly higher in the ODN group than in the NVS group.
Hepatitis B immunization in low birthweight infants: do they need an additional dose?
Authors: Arora NK , Ganguly S , Agadi SN , Irshad M , Kohli R , Deo M , Paul VK , Deorari AK , Chellani H , Prasad MS , Sharma D 
Published: August 2010

Aim: To determine the influence of gestation and weight on the development of protective anti‐HB levels and geometric mean titres after three doses of HBV vaccine and to ascertain the need for a fourth dose in low birthweight infants. Methods: Hepatitis B vaccine (Enivac HB, Panacea Biotec Ltd., India) was given to 82 preterm (PT) and 60 term intrauterine growth‐retarded (T‐IUGR) infants at birth and at 6, 10 and 14 wk of life. Results: Protective anti‐HB levels (>10 mIU/ml) were reached in 86.6% (71/82) of PT infants and 96.7% (58/60) of T‐IUGR infants after three doses of HBV vaccine (p= 0.044). The odds of having a protective response after the third dose of HBV vaccine was 1.25 (95% CI 1.02–1.53) with every one‐week increase in gestation (p= 0.032). Birthweight was not associated with the development of a protective immune response. After the third dose, only 66.7% (8/12) of the PT infants whose mothers had anti‐HB antibodies, developed protective anti‐HB levels compared with 90% (63/70) of those with no maternal antibodies (p= 0.028). In PT infants after the fourth dose, there was a significant increase in the proportion of infants with protective antibody levels (8.6%, 95% CI 0.6–16.6%) among those with no maternal antibodies and 12.2% overall (95% CI 6.0–21.3) (p= 0.031 to 0.002) over that reached with the third dose. Administration of the fourth dose to T‐IUGR infants did not confer such a benefit. Conclusion: In HBV‐endemic areas, PT infants, irrespective of their birthweights, may benefit from an additional dose of hepatitis B vaccine in a schedule starting at birth. This approach will prevent vertical transmission and bring their immune response up to par with term infants. Term intrauterine growth‐retarded infants should be vaccinated as per the schedule recommended for normal term infants. However, studies in other settings with different vaccine formulations and a longer follow‐up period will be required before this strategy can be practised more widely.
Cardiac involvement in Fabry disease
Authors: Linhart A , Magage S , Palecek T , Bultas J 
Published: August 2010

Fabry disease is a rare X‐linked defect of the lysosomal enzyme α‐galactosidase A. The disease is characterized by progressive intracellular accumulation of neutral glycosphingolipids. The storage occurs within various tissues and cells, including cardiocytes, the cardiac conduction system, and valvular fibrocytes. Cardiac involvement may be the sole manifestation of the disease, particularly in individuals with residual enzyme activity. In general, hemizygous men are more seriously affected than heterozygous women. The main cardiac manifestations include myocardial hypertrophy, which, in some patients, mimics hypertrophic cardiomyopathy. Conduction system involvement leads to PR shortening or, in later stages, to AV blocks. Arrhythmias presenting with variable severity also appear to be common. Valvular involvement is frequently noted but generally mild and clinically non‐significant. Newly available enzyme replacement therapy has produced promising results in preventing further functional deterioration of affected organs and possibly also in reversing impaired function. Conclusions: With the advent of effective enzyme replacement therapy, early diagnosis of Fabry disease may be crucial for patient prognosis.
Elevated CNS average diffusion constant in Fabry disease
Authors: Moore DF , Schiffmann R , Ulug AM 
Published: August 2010

Aims: Evaluation of the average brain diffusion constant in Fabry disease. Introduction: Fabry disease is an X‐linked recessive lysosomal storage disorder secondary to deficiency of α‐galactosidase A and resulting in excess tissue globotriaosylceramide, particularly in cerebral blood vessels. This has been associated with cerebral hyperperfusion. Increased tissue perfusion should increase interstitial water by the Starling relationship. This hypothesis was examined by measuring the average CNS diffusion constant (Dav) in patients with Fabry disease using diffusion‐weighted magnetic resonance imaging (DWI). Methods: Axial DWI was performed at b= 1000 seconds/mm2 and b= 1000 seconds/mm2 (TR (pulse repetition time), 10 000; TE (time to echo), 100; FOV (field of view), 22 cm; 3 mm interleaved slices; image matrix, 128 × 128; GE Signa, 1.5T). Eight healthy male volunteers (age range, 21–47 years) and 17 hemizygous patients with Fabry disease (age range, 19–49 years) were examined. Following DWI acquisition, the trace image and the diffusion distribution map were calculated. The diffusion distribution curve was then fitted by a multi‐modal Gaussian curve, allowing estimation of Dav. Results: The Dav was 0.743 ±; 0.024 ±; 10‐5 cm2/second (mean ±; SD) for patients with Fabry disease and 0.726 ±; 0.014 ±; 10‐5 cm2/second for the control group. Dav was significantly increased in the patients with Fabry disease compared with the controls (p= 0.029) Conclusions: The elevated Dav indicates increased brain tissue water diffusivity in patients with Fabry disease, a finding consistent with increased extracellular water and increased cerebral blood flow.