bluuebunny/crossref_metadata_2025_split huggingface dataset:
Measurement of renal function in patients with Fabry disease
Authors: Kleinert J , Lorenz M , Hauser A ‐C , Becherer A , Staudenherz A , Födinger M , Sunder‐Plassmann G 
Published: August 2010

AbstractAppropriate measurement of the glomerular filtration rate (GFR) is important for the assessment of renal function. This paper reviews the methods used to assess GFR in clinical trials of enzyme replacement therapy (ERT) in patients with Fabry disease, which include inulin clearance, 24‐hour creatinine clearance, chromium ethylene diamine tetraacetate (51Cr‐EDTA) clearance and cystatin C concentrations. GFR has also been estimated using calculations based on creatinine clearance (the Cockcroft–Gault formula) and the Modification of Diet in Renal Disease (MDRD) equation. Analysis of the results of these studies shows that there are striking discrepancies between estimated and measured GFR. For example, the MDRD equation overestimates GFR in patients with Fabry disease who have normal renal function. In addition, cystatin C has been shown to be of limited use for measuring renal function during ERT, because it is influenced by other factors such as age, gender and weight. Conclusion: The use of exact methods, such as inulin clearance, 124I‐iothalamate, 99mTc‐DTPA, 51Cr‐EDTA and iohexol, appears to be mandatory for a robust evaluation of the effects of ERT on GFR in patients with Fabry disease.
What can cell biology tell us about heterogeneity in lysosomal storage diseases?
Authors: Gieselmann V 
Published: August 2010

AbstractLysosomal storage diseases are clinically heterogeneous with respect to their age of onset, progression of symptoms and the particular organs involved. Varying levels of residual enzyme activity, associated with different defective alleles that cause the respective diseases, are responsible in part for this clinical heterogeneity. In general, the higher the residual enzyme activity, the milder the phenotype. Enzyme activity in severe forms of disease is frequently zero, and in mild forms usually does not exceed approximately 5%. However, the correlation is not so strict as to allow prediction of the phenotype of individual patients. The molecular basis of the different levels of enzyme activity can only be revealed by biochemical investigations of the defective lysosomal proteins. Null alleles may be due to splice‐site mutations or deletions. In the case of missense mutations, enzymes frequently fold incorrectly and are retained in the endoplasmic reticulum and subsequently degraded. As these enzymes do not reach the lysosome, they do not provide any functional residual activity. Residual enzyme activity is only observed in cases where the defective enzyme reaches the lysosome and has retained enzymatic activity. Patients carrying the same mutant alleles still show considerable phenotypic variability due to modifying genes and epigenetic factors. None of these has so far been elucidated. However, there are some indications that differences in splicing‐factor machinery may influence the phenotypic expression of splice‐site mutations and that hormonal modulation of secondary microglial activation in lipidosis may also influence the disease course. Conclusion: Phenotypic variability is a frequent phenomenon in lysosomal storage diseases. Residual enzyme activity has been identified as one of the factors influencing the clinical outcome of disease; however, it is obvious that other genetic and epigenetic factors also affect phenotypic variability, particularly in patients with late onset disease.
Action, prevention and epidemiology of paediatric obesity
Authors: Lissau Inge 
Published: August 2010

AbstractThe overall aim of this paper is to describe important issues regarding paediatric obesity as a public health problem. This paper focuses on actions taken, and on the prevalence of obesity in children, teens and adults in Denmark. In addition, the paper describes some important prevention studies, all of which are performed outside Denmark. Thus, this paper is not a classical review but rather a highlight of some aspects that the author finds important. The latest Danish national figures show a marked increase in the prevalence of obesity, especially among young men—a sevenfold increase from 1987 to 2000 (0.7 to 4.9%). Among young women aged 16–24, the increase is threefold in the same period. Among teens, the prevalence has increased by 2–3 times in recent decades. Nevertheless, compared to other European countries and the US, Denmark has a relatively low prevalence of obesity in adolescents. The present paper also covers results from prevention studies performed in both preschool and school settings. Some of these focus on the reduced intake of carbonated drinks, whereas others focus on both diet and physical exercise. Finally, this paper demonstrates that Denmark is at the forefront regarding a national action plan against obesity. Conclusion: This paper highlights some important aspects of the epidemiology, prevention and actions in the field of paediatric obesity with special focus on Denmark.
PRESTO—Prevention Study of Obesity: A project to prevent obesity during childhood and adolescence
Authors: Dämon Sabine , Dietrich Sabine , Widhalm Kurt 
Published: August 2010

AbstractThe PRESTO project has been launched as a school‐oriented pilot project. The aims were to influence nutrition, physical activity and health. The intervention was performed by a multiprofessional team and covered 11 nutrition and health‐related sessions with 1 h per week in each class. The initial poor knowledge was improved significantly to the control group with better results in grammar schools compared to comprehensive schools. BMI was not improved in overweight children. However, this project could serve as a successful model in Austria.
Fetal nutrition: A review
Authors: Cetin Irene , Alvino Gioia , Radaelli Tatjana , Pardi Giorgio 
Published: August 2010

AbstractKnowledge of fetal nutrient supply has greatly increased in the last decade due to the availability of fetal blood samples obtained under relatively steady‐state conditions. These studies, together with studies utilizing stable isotope methodologies, have clarified some aspects of the supply of the major nutrients for the fetus such as glucose, amino acids and fatty acids. At the same time, the relevance of intrauterine growth has been recognized not only for the well‐being of the neonate and child, but also for later health in adulthood. The major determinants of fetal nutrient availability are maternal nutrition and metabolism together with placental function and metabolism. The regulation of the rate of intrauterine growth is the result of complex interactions between genetic inheritance, endocrine environment and availability of nutrients to the fetus.
The Nobel Prize in 2005 for the discovery of Helicobacter pylori: Implications for child health
Authors: Zetterström Rolf 
Published: August 2010

AbstractThe Nobel Prize in Physiology or Medicine in 2005 has been awarded to B. Marshall and R. Warren for their discovery that peptic ulcer disease is caused by an infection with Helicobacter pylori. This infection, which affects about half of the world's population and is already extremely prevalent in adolescents in developing countries, starts as an asymptomatic gastritis which, under certain conditions, is followed by gastric or duodenal ulcer disease. Conclusion: No proven benefit has yet been found by treating H. pylori‐infected children with gastritis unless they have a peptic ulcer. Vaccination against H. pylori infection during early childhood is considered a means of preventing peptic ulcer disease and also possibly adenocarcinoma.
Assessment of total body fat using the skinfold technique in full‐term and preterm infants
Authors: Olhager Elisabeth , Forsum Elisabet 
Published: August 2010

Abstract Background: Assessment of body composition may be of interest when the nutritional status of infants is evaluated but is often difficult since simple and valid methods are lacking. With appropriate validation, measurements based on skinfold thickness (SFT) may be useful for this purpose. Aims: To evaluate the potential of a published method, based on measurements of SFT, to assess total body fat (TBF) of infants; and to calculate the fat content of adipose tissue (AT) in infants using previously published information regarding AT volume and total body water. Subjects and methods: Forty‐five full‐term infants and eight infants born in gestational weeks 31–33 were studied at a postnatal age of 4–131 and 44–75 d, respectively. The body water dilution method was used to obtain reference estimates of TBF (TBF‐BWD). Results: In full‐term infants, TBF assessed using the skinfold method (TBF‐SFT) minus TBF‐BWD was 1.5±10.8% (mean±2 SD). Furthermore, TBF‐SFT minus TBF‐BWD (%, y) was correlated (p<0.0001) with the average of TBF‐SFT and TBF‐BWD (%, x), showing that TBF‐SFT was too high in lean infants and too low in infants with more TBF. In the full‐term infants, AT contained 0.68±0.14 g fat/ml. In the premature infants, TBF‐SFT (%), TBF‐BWD (%) and the AT fat content were similar to the corresponding figures in nine full‐term newborns. Conclusion: The results indicate that the SFT method produced inaccurate and biased estimates of TBF in infants. A considerable variation between infants regarding their AT fat content may be an important reason for these findings.
Increase of interleukin‐6 in tracheal aspirate at birth: A predictor of subsequent bronchopulmonary dysplasia in preterm infants
Authors: Won Choi Chang , Il Kim Beyong , Kim Han‐Suk , Dong Park June , Choi Jung‐Hwan , Woo Son Dong 
Published: August 2010

Abstract Aim: We tested whether interleukin‐6 (IL‐6) in tracheal aspirate (TA) at birth, as a marker of fetal pulmonary inflammation, can be a predictor of bronchopulmonary dysplasia (BPD) in preterm infants. Methods: A total of 75 preterm (≤32 wk) infants who were intubated in the delivery room were prospectively enrolled. Multivariate logistic regression analysis was done to determine whether IL‐6 in TA at birth is an independent risk factor for BPD, and a receiver‐operating characteristic curve was constructed to determine the accuracy of IL‐6 in TA for predicting the risk of BPD. Results: IL‐6 in TA at birth was an independent risk factor for BPD. Fetal pulmonary inflammation defined as IL‐6 in TA at birth ≥316 pg/ml together with patent ductus arteriosus (PDA) additively predicted the risk of BPD. The sensitivity, specificity, and positive and negative predictive values of fetal pulmonary inflammation for the identification of BPD were 73%, 71%, 58% and 83%, respectively. Conclusion: IL‐6 in TA at birth can be used as a predictor of BPD in combination with the presence of PDA.
Umbilical artery catheter blood sampling volume and velocity: Impact on cerebral blood volume and oxygenation in very‐low‐birthweight infants
Authors: Roll Claudia , Hüning Britta , Käunicke Matthias , Krug Jens , Horsch Sandra 
Published: August 2010

Abstract Aim: Blood sampling from umbilical artery catheters decreases cerebral blood volume and cerebral oxygenation. The aim of this study was to assess the impact of sampling volume and velocity. Methods: Forty‐eight infants, median birthweight 965 g (480–1500 g), median gestational age 27 wk (23–34 wk), were studied during routine blood sampling from umbilical artery catheters. The sampling procedure was performed following a strict protocol for draw‐up volume (1.6 ml), sampling volume (1.7 ml or 0.2 ml), re‐injection volume (1.6 ml) and flushing volume (0.6 ml), time of aspiration (40 s or 80 s), re‐injection (30 s) and flushing (6 s). In each infant, sampling volume and aspiration time were subject to sequential variation in a randomized fashion (1.7 ml/40 s, 1.7 ml/80 s, 0.2 ml/30 s). Using near‐infrared spectroscopy, changes in concentrations of cerebral oxygenated and deoxygenated haemoglobin were measured, and changes in cerebral blood volume and cerebral oxygenation were calculated. Results: During all three sampling procedures, oxygenated haemoglobin decreased significantly from baseline, whereas deoxygenated haemoglobin did not change. Correspondingly, a decrease in cerebral blood volume and cerebral oxygenation occurred. This decrease was not affected significantly by extending the sampling time from 40 s to 80 s, whereas it was blunted by reducing the amount of blood withdrawn. Conclusion: Blood sampling from umbilical artery catheters induces a decrease in cerebral blood volume and cerebral oxygenation. The magnitude of the decrease depends on the blood volume withdrawn but not on sampling velocity.
The relationship between socio‐economic status, parental support and adolescent physical activity
Authors: Raudsepp Lennart 
Published: August 2010

Abstract Aim: To examine: 1) the association between socio‐economic status and adolescent physical activity; 2) to assess links between mothers’ and fathers’ social support and adolescents’ physical activity. Methods: Participants were 326 urban adolescents and their parents. Physical activity was measured using a 7‐d physical activity recall. Socio‐economic status and parents’ social support was assessed using questionnaires. Results: Boys were significantly more active than girls, and fathers’ explicit modelling towards boys was higher compared to girls. In contrast, fathers’ and mothers’ logistic support was significantly higher towards their daughters. Social class and fathers’ and mothers’ social support were significantly related to adolescent self‐reported physical activity. Fathers’ explicit modelling was the strongest predictor of adolescent physical activity, predicting 13.5% of the total variance. Social class, fathers’ logistic support and mothers’ explicit modelling were also significant predictors, accounting for additional 14% of the variance in adolescents’ weekly physical activity. Conclusion: Parental support and social class are positively related to physical activity in Estonian adolescents.
Effects of brief exposure to water, breast‐milk substitutes, or other liquids on the success and duration of breastfeeding: A systematic review
Authors: Szajewska Hania , Horvath Andrea , Koletzko Berthold , Kalisz Marcin 
Published: August 2010

Abstract Aim: To systematically evaluate the effect of supplemental fluids or feedings during the first days of life on the overall breastfeeding duration and rate of exclusive breastfeeding among healthy infants. Methods: Medical subject headings and free‐language terms were used to search the following electronic databases for studies relevant to breastfeeding: MEDLINE, EMBASE, Cumulative Index to Nursing and Allied Health (CINAHL), the Cochrane Library, and La Leche League. Only randomized, controlled trials (RCT) were considered for study inclusion. Results: Of 56 potentially relevant clinical trials identified, only one RCT (170 infants) met the inclusion criteria for this systematic review. In this study, formula feeding was significantly more frequent at 4 wk in the experimental group in which breastfeeding had been supplemented with 5% glucose ad libitum during the first 3 d of life (n=83) than in the exclusively breastfed control group (n=87) (p<0.05). At 16 wk (5 mo postpartum), the percentage of mothers who continued breastfeeding, either exclusively or partially, was significantly lower in the experimental group than in the control group (p<0.01). Conclusion: There remains considerable uncertainty about the effect of brief exposure to water, breast‐milk substitutes, or other liquids on the success and duration of breastfeeding.
Influence of early postnatal nutritional management on oxidative stress and antioxidant defence in extreme prematurity
Authors: Yeung Melinda Y. 
Published: August 2010

AbstractimageThe increased survival of infants born at mid‐gestation in the last decade is associated with significant oxygen free radical‐mediated morbidities. Resuscitation with 100% oxygen, oxidant load from parenteral nutrition fluids, and oxidant stress inherent to the systemic inflammatory state subsequent to infection and tissue injury are all contributory. Conclusion: Improving early postnatal protein nutrition and the formulation of parenteral nutrition fluids would potentially reduce the oxidative stress and enhance the antioxidant defence of extremely premature newborns.
Nontuberculous mycobacterial adenitis: Effectiveness of chemotherapy following incomplete excision
Authors: Coulter J. Brian S. , Lloyd David A. , Jones Matthew , Cooper John C. , McCormick Maxwell S. , Clarke Raymond W. , Tawil Mohommed I 
Published: August 2010

Abstract Background: Management of lymphadenopathy caused by nontuberculous mycobacteria (NTM) is primarily surgical. Where this cannot achieve sufficient clearance of infected nodes, chemotherapy is often given. Aim: This study compared results of surgery alone with surgery followed by chemotherapy in instances where there was incomplete surgical removal of diseased tissue. Methods: Chemotherapy comprised azithromycin 10 mg/kg and rifabutin 6 mg/kg both given once daily for 6 mo. Ninety‐eight children with NTM infection were seen in the period 1990–2004. Sixty‐eight cases with adenopathy where “time to healing” (discharge stopped and inflammation settled) was known were available to compare response to treatment. Results: The median (range) “time to healing” in weeks for 43 patients who had surgery alone was: incision and drainage (I&D)/curettage 6 (1–72) (n=10); excision 3 (1–28) (n=22); and from the last operation of multiple (repeat) surgery 3 (1–40) (n=11). For 25 patients who required chemotherapy in addition to surgery, the median (range) “time to healing” in weeks was I&D/curettage 10 (1–40) (n=17), excision 14 (8–20) (n=2) and multiple surgery 29 (2–88) (n=6). Conclusion: In children with adenitis due to NTM, where surgical resection is followed by continued discharge and inflammation, chemotherapy should be considered before further surgery is undertaken.
Oral aphthous ulcers and dental enamel defects in children with coeliac disease
Authors: Bucci Paolo , Carile Francesco , Sangianantoni Anna , D'Angiò Franca , Santarelli Andrea , Lo Muzio Lorenzo 
Published: August 2010

Abstract Aim: Coeliac disease is characterized by oral manifestations, such as dental enamel defects and recurrent oral aphthae. In this study we compared the prevalence of enamel defects and recurrent aphthous stomatitis (RAS) between patients diagnosed with coeliac disease and healthy controls. Methods: A total of 72 patients with coeliac disease were studied together with 162 normal healthy subjects as controls to individualize the prevalence of enamel defects and recurrent aphthous stomatitis (RAS) in this disease. Results: Dental enamel defects were found in 14/70 (20%) coeliac patients and in 9/159 (5.6%) controls. In particular, 13/53 (24.5%) coeliac patients with mixed or permanent dentition and 1/17 (5.8%) coeliac children with deciduous dentition had enamel defects. Altogether, 9/145 (6.2%) control subjects with mixed or permanent dentition had dental enamel defects. None of the controls with deciduous dentition had enamel defects. Thus, the enamel defects occurred more frequently in coeliac patients (p<0.001). Regarding RAS, 24/72 (33.3%) coeliac patients and 38/162 (23.4%) control subjects had aphthous ulcers. Statistically significant differences were not observed between the two groups (p > 0.05). One in three coeliac subjects suffering from RAS received benefit from a gluten‐free diet. Conclusion: In the present study, the prevalence of enamel defects was found to be greater in coeliac patients than healthy controls. Even though the prevalence of RAS in coeliac subjects varied from healthy controls, the difference was not statistically significant. However, more than 1/3 coeliac subjects suffering from RAS benefited from a gluten‐free diet.
Children with chronic conditions in a paediatric emergency department
Authors: Massin Martial M. , Montesanti Jessica , Gérard Paul , Lepage Philippe 
Published: August 2010

Abstract Aim: To describe the use of a paediatric emergency department by children with chronic conditions. Material and methods: We prospectively indexed all the children seen in our emergency department during 1 y to describe the population of children with chronic conditions, and to compare it with the general paediatric population. Results: There were 11 483 patient visits, of which 11% were by chronically ill children. Six subspeciality areas accounted for the vast majority of the chronic conditions seen: neurology (30%), cardiology (18%), pulmonology (18%), nephro‐urology (16%), haemato‐oncology (9%) and endocrinology (4%). Admission rate to the hospital was significantly higher for chronically ill patients (38% vs 23%). Among chief complaints, fever, respiratory distress, headache and seizures were overrepresented in chronically ill children. The chief complaint was related to the child's chronic condition in 51% of the visits. In both chronically and non‐chronically ill patients, the majority of final diagnoses were related to infection (57% and 65%, respectively). Among diagnoses, bacterial infections, seizures, vasovagal syncopes and asthma were overrepresented in chronically ill children. Conclusion: From the analysis of our data, we conclude that children with chronic conditions account for a tenth of all visits to a paediatric emergency department. The paediatric emergency physicians provide an important service to both the children with chronic conditions and the subspecialists who care for them.
Elevation of serum creatine kinase in response to medical treatment of Graves’ disease in children
Authors: Mizuno Haruo , Sugiyama Yukari , Nishi Yoshikazu , Ueda Noriko , Ohro Yoichiro , Togari Hajime 
Published: August 2010

AbstractWe describe two cases of increases in serum creatine kinase (CK) concentrations in children undergoing treatment of Graves’ disease with antithyroid medications. Presenting complaints consisted of myalgias and muscle cramping in both patients, and increases in serum CK levels were noted 1 mo after initiation of antithyroid drugs. Both patients were euthyroid at the time of CK elevation. While the mechanisms for this process are not clear, it is likely that the acute decrease of thyroid hormones in tissues following a state of chronic hyperthyroidism may result in relative hypothyroid states and subsequent alterations in CK concentrations. Conclusion: Although this side effect has been reported in adults, it is a novel finding in children. Clinicians should be aware of the rare potential for elevations in serum CK when initiating treatment for Graves’ disease in children.
Intracranial germinoma: A rare but important differential diagnosis in children with growth retardation
Authors: Gottschling Sven , Graf Norbert , Meyer Sascha , Reinhard Harald , Krenn Thomas , Rohrer Tilman 
Published: August 2010

Abstract Aim: Intracranial germinoma is a rare malignant tumour in childhood with an excellent prognosis under adequate therapy. Finding the right diagnosis at an early stage is difficult because of the slow‐growing tumour and the resulting lack of clinical symptoms. Methods: Our patients with histologically secured germinoma were retrospectively assessed concerning growth retardation, pituitary hormone status, magnetic resonance imaging scan results and clinical symptoms to find out whether there is a leading parameter. Results: In all our patients, the leading symptom was growth retardation of at least 2 y before being diagnosed. Conclusion: Growth retardation seems to be a very early sign of germinoma in the suprasellar region. Therefore, early neuroradiological imaging combined with pituitary hormone status should be considered in every paediatric patient with a history of secondary growth retardation.