bluuebunny/crossref_metadata_2025_split huggingface dataset:
Identification and use of biomarkers in Gaucher disease and other lysosomal storage diseases
Authors: Aerts J M F G , Hollak C E M , van Breemen M , Maas M , Groener J E M , Boot RG 
Published: August 2010

AbstractThe value of biomarkers in the clinical management of lysosomal storage diseases is best illustrated by the present use of plasma chitotriosidase levels in the diagnosis and monitoring of Gaucher disease. The enzyme chitotriosidase is specifically produced and secreted by the pathological storage macrophages (Gaucher cells). Plasma chitotriosidase levels are elevated on average 1000‐fold in symptomatic patients with Gaucher disease and reflect the body burden on storage cells. Changes in plasma chitotriosidase reflect changes in clinical symptoms. Monitoring of plasma chitotriosidase levels is nowadays commonly used in decision making regarding initiation and optimization of costly therapeutic interventions (enzyme replacement therapy or substrate reduction therapy). A novel substrate has been developed that further facilitates the measurement of chitotriosidase in plasma samples. Moreover, an alternative Gaucher‐cell marker, CCL18, has been very recently identified and can also be employed to monitor the disease, particularly in those patients lacking chitotriosidase due to a genetic mutation. There is a need for comparable surrogate markers for other lysosomal storage diseases and the search for such molecules is an area of intense investigation. Conclusion: The use of biomarkers can provide valuable insight into the molecular pathogenesis of LSDs, such as Gaucher disease and Fabry disease.
Prebiotic carbohydrates in human milk and formulas
Authors: Boehm Günther , Stahl Bernd , Jelinek Jürgen , Knol Jan , Miniello Vito , Moro Guido E 
Published: August 2010

AbstractHuman milk oligosaccharides play an important role, as prebiotic soluble fibres, in the postnatal development of the intestnial flora. Infant formulas are virtually free of prebiotic oligosaccharides. As a consequence, formula‐fed infants develop an intestinal flora significantly different to the flora of breastfed infants. Due to the complexity of human milk oligosaccharides, it is necessary to use alternative sources of prebiotic ingredients as components of infant formulas. The present review summarizes the data of experimental research and clinical studies with a prebiotic mixture containing 90% short‐chain galacto‐oligosaccharides and 10% long‐chain fructo‐oligosacchrides are summarized. The data demonstrate that, with this prebiotic mixture, the growth of bifidobacteria and lactobacilli can be stimulated, the faecal pH can be decreased, and the presence of pathogens can be reduced to levels similar to those of breastfed infants. Thus, prebiotic oligosaccharides such as the studied mixture provide beneficial effects for formula‐fed infants.
Galacto‐oligosaccharides and long‐chain fructo‐oligosaccharides as prebiotics in infant formulas: A review
Authors: Fanaro Silvia , Boehm Günther , Garssen Johan , Knol Jan , Mosca Fabio , Stahl Bernd , Vigi Vittorio 
Published: August 2010

AbstractThe present review summarizes clinical and experimental data concerning the possible effects of a prebiotic mixture of short‐chain galacto‐oligosaccharides and long‐chain fructo‐oligosaccharides. The results from several studies, made up of over 400 preterm and term infants, clearly demonstrate that the prebiotic mixture under examination specifically stimulates the growth of bifidobacteria and lactobacilli and reduces the growth of pathogens. As a consequence of the changed intestinal flora by the dietary galacto‐oligosaccharides and fructo‐oligosaccharides, the faecal pH values and the short‐chain fatty acid pattern were similar to those found in breastfed infants. In addition, the stool consistency was the same as in breastfed infants. In vitro experiments have demonstrated that the specific short‐chain fatty acid pattern, at a pH similar to that found in faecal samples of breastfed infants, reduces the growth of pathogens in a dose‐dependent manner but does not influence the growth of bifidobacteria and lactobacilli. In an animal vaccination model, the prebiotic mixture improved the response to vaccination. In an allergy model (sensitization by ovalbumin), the allergic reaction was reduced by the prebiotic mixture. The data obtained from animal experiments are in agreement with preliminary data from clinical trials which indicate a reduced allergic response (reduced plasma IgE/IgG4 ratio) and reduced episodes of upper airway infection during the first year of life. Conclusion: Experimental evidence demonstrates that the prebiotic mixture employed in these studies modulates the intestinal flora and modulates the immune system as human milk does. There are sufficient experimental data to put forward the hypothesis that substances like the prebiotic mixture under study will substantially contribute to the improvement of the protective properties of infant formulas.
Hydrolysed milk in preterm infants: An open problem
Authors: Zuppa Antonio A , Visintini Federica , Cota Francesco , Maggio Luca , Romagnoli Costantino , Tortorolo Giuseppe 
Published: August 2010

Abstract Aim: To review knowledge about the role, indications and nutritional adequacy of protein hydrolysate formulas (HF) in the preterm newborn. Methods: A review of the available literature was carried out. Results: No data support the use of HF for atopy prevention. HF could have a positive role in the early feeding of very‐low‐birthweight infants, but several concerns exist about their nutritional adequacy. Conclusion: Further investigations addressing protein and mineral metabolism as well as long‐term effects, including neurodevelopment, are needed.
Feeding intolerance in very‐low‐birthweight infants: What is it and what can we do about it?
Authors: Neu Josef , Zhang Liyan 
Published: August 2010

AbstractThe increased survival of very‐low‐birth‐weight infants has resulted in the need to better understand the immaturities that challenge optimal nutrition for these infants and how to surmount them. This is critical if we are to prevent short and long term morbidity associated with poor nutrition. Here we describe several of these immaturities including those related to digestion and absorption, suck‐swallow incoordination, delayed gastric emptying, and intestinal motility and how they lead to the common problem of feeding intolerance. Scientifically based strategies for introducing, stopping and continuing enteral feedings in association with parenteral nutrition are presented.
Risk factors associated with the need for additional intravenous gamma‐globulin therapy for Kawasaki disease
Authors: Muta Hiromi , Ishii Masahiro , Furui Jun , Nakamura Yosikazu , Matsuishi Toyojiro 
Published: August 2010

Abstract Aim: To investigate the characteristics of patients with Kawasaki disease who needed intravenous gamma‐globulin (IVGG) re‐treatment. Methods: Using the database of the 17th nationwide survey in Japan, a total 11 366 patients were identified and analysed (1855 re‐treatment patients and 9511 responders). Results: Multivariate logistic regression analysis showed that male sex (odds ratio (OR) 1.26; 95% CI 1.14–1.40), complete cases (OR 1.39; 95% CI 1.07–1.80), recurrence (OR 1.47; 95% CI 1.15–1.88), IVGG treatment within 4 d of illness (OR 2.05; 95% CI 1.84–2.27), daily dose of initial IVGG less than 1000 mg/Kg (OR 0.54; 95% CI 0.48–0.61), exanthema (OR 2.03; 95% CI 1.62–2.56), lips and oral lesions (OR 1.57; 95% CI 1.24–1.98), peripheral extremities changes (OR 1.85; 95% CI 1.54–2.22), and cervical lymphadenopathy (OR 1.89; 95% CI 1.66–2.16) were independent risk factors associated with the need for IVGG re‐treatment. Conclusion: Male sex, recurrence, and treatment with IVGG at a dose of 1000 mg/d or less within 4 d of illness onset are independent risk factors associated with the need for IVGG re‐treatment.
Increased levels of BAL cysteinyl leukotrienes in acute RSV bronchiolitis
Authors: Keun Kim Chang , Yeon Koh Ji , Hee Han Tae , Kyun Kim Do , Il Kim Beyong , Yull Koh Young 
Published: August 2010

Abstract Background: Cysteinyl leukotrienes (CysLTs), including LTC4, LTD4 and LTE4, are pivotal mediators in the pathophysiology of asthma. Aim: To determine whether CysLT levels are increased in the lower airways of children with respiratory syncytial virus (RSV) bronchiolitis, as they are in asthmatic children, and to investigate a possible heterogeneity in CysLT levels in children with RSV bronchiolitis. Methods:Bronchoalveolar lavage (BAL) fluids were obtained from children with acute RSV bronchiolitis (n=20), from children with acute asthma who had no identifiable virus infection (n=16) and from control subjects (n=14). BAL cell counts and differentials were determined, and the concentrations of CysLTs were measured by ELISA. Results: CysLT levels in the asthma (70.6±52.7 pg/ml, p < 0.001) and bronchiolitis groups (21.9±23.3 pg/ml, p < 0.05) were significantly higher than in the control group (8.7±5.2 pg/ml). Among bronchiolitis subjects, the eosinophil‐positive subgroup (n=6) showed significantly higher CysLT levels (49.0±26.7 pg/ml, p = 0.001) than the control group, but this was not observed in the eosinophil‐negative subgroup (n=14, 10.3±6.3 pg/ml, p = 0.47). Conclusion: CysLT levels are increased in the lower airways during RSV bronchiolitis, although their intensities are lower than those in acute asthma. Among bronchiolitis subjects, high CysLT producers could be distinguished from low CysLT producers by the presence of eosinophilia in BAL fluids, suggesting a pathophysiological heterogeneity in RSV bronchiolitis.
Rapid control of norovirus gastroenteritis outbreak in an acute paediatric ward
Authors: Cheng F. W. T. , Leung T. F. , Lai R. W. M. , Chan P. K. S. , Hon E. K. L. , Ng P. C. 
Published: August 2010

Abstract Aim: To provide a practical action plan for effective infection control of norovirus outbreak in acute paediatric wards. Methods: We report the infection control measures that were implemented to terminate and to prevent nosocomial spread of norovirus gastroenteritis in an open‐designed paediatric ward. Results: Nine children, one visitor, and one medical student were affected in a norovirus gastroenteritis outbreak in an acute paediatric ward. Vomiting was the main presenting symptom. The outbreak was rapidly terminated three days after implementation of stringent infection control measures and there was no second wave of attack. These measures included strict contact precautions, prompt isolation and cohorting of symptomatic patients, vigorous environmental cleansing with concentrated disinfectant (hypochlorite solution 1000 ppm), meticulous handling of waste products, and efficient contact tracing of exposed patients, family members, and medical students. Conclusion: Prompt implementation of stringent infection control measures and contact tracing can rapidly terminate the norovirus outbreak and prevent a second wave of infection. Children with unexplained vomiting and those with contact history of gastroenteritis should be properly triaged, isolated, and investigated for possible infective causes, including norovirus‐induced gastroenteritis.
Respiratory Tract Malacia: Possible cause of sudden death in infancy and early childhood
Authors: Rohde Marianne , Banner Jytte 
Published: August 2010

Abstract Aim: To stress the importance of considering Laryngo‐tracheo‐bronchomalacia (LTBM) as a cause of death in infancy and early childhood. Methods: A case of Tracheo‐bronchomalacia diagnosed at autopsy is presented, with a short review of the literature. Results: The condition causes functional weakness of the airways due to congenital or acquired abnormalities of airway cartilage. The etiology of the congenital variant is uncertain; the acquired may be caused by localized external pressure. Morphologically, cartilage rings are shorter and softer than normal, causing collapse, especially during forced expirations. The defect can be an isolated finding, but there are a number of associations with other malformations. LTBM may be misdiagnosed as asthma. Symptoms include wheezing, barking cough, frequent respiratory infections and cyanotic episodes. Treatment might be conservative or include surgery, depending on etiology and severity. Conclusion: Laryngo‐tracheo‐bronchomalacia (LTBM) is usually not considered a sufficient cause of death in infancy and early childhood, but airway malacia may be an unrecognized cause of sudden death in infancy and early childhood, and should be considered, especially in cases where there is a history of respiratory distress.
Hypoglycaemia and seizures in large‐for‐gestational‐age (LGA) full‐term neonates
Authors: Groenendaal Floris , Elferink‐Stinkens Patty M. ,   The Netherlands Perinatal Registry
Published: August 2010

Abstract Aim/methods: To assess the risk of hypoglycaemia‐associated seizures in large‐for‐gestational‐age (LGA) full‐term neonates data from the Netherlands Perinatal Registry were analysed. Results: From 1997 to 2002 hypoglycaemia was recorded in 1513 of 9318 (16.2%) admitted LGA (defined as birthweight ≥97.7 percentile) full‐term neonates without maternal diabetes, of whom 20 (1.3%) had seizures. In six of these 20, hypoglycaemia was the single cause of seizures. Conclusion: Symptomatic hypoglycaemia occurs in healthy LGA full‐term neonates.
Breastfeeding in the WHO Multicentre Growth Reference Study
Authors:   WHO MULTICENTRE GROWTH REFERENCE STUDY GROUP, de Onis Mercedes 
Published: August 2010

Abstract Aim: To document how children in the WHO Multicentre Growth Reference Study (MGRS) complied with feeding criteria and describe the breastfeeding practices of the compliant group. Methods: The MGRS longitudinal component followed 1743 mother–infant pairs from birth to 24 mo in six countries (Brazil, Ghana, India, Norway, Oman and the USA). The study included three criteria for compliance with recommended feeding practices that were monitored at each follow‐up visit through food frequency reports and 24‐h dietary recalls. Trained lactation counsellors visited participating mothers frequently in the first months after delivery to help with breastfeeding initiation and prevent and resolve lactation problems. Results: Of the 1743 enrolled newborns, 903 (51.8%) completed the follow‐up and complied with the three feeding criteria. Three quarters (74.7%) of the infants were exclusively/predominantly breastfed for at least 4 mo, 99.5% were started on complementary foods by 6 mo of age, and 68.3% were partially breastfed until at least age 12 mo. Compliance varied across sites (lowest in Brazil and highest in Ghana) based on their initial baseline breastfeeding levels and sociocultural characteristics. Median breastfeeding frequency among compliant infants was 10, 9, 7 and 5 feeds per day at 3, 6, 9 and 12 mo, respectively. Compliant mothers were less likely to be employed, more likely to have had a vaginal delivery, and fewer of them were primiparous. Pacifier use was more prevalent in the non‐compliant group. Conclusion: The MGRS lactation support teams were successful in enhancing breastfeeding practices and achieving high rates of compliance with the feeding criteria required for the construction of the new growth standards.
WHO Motor Development Study: Windows of achievement for six gross motor development milestones
Authors:   WHO MULTICENTRE GROWTH REFERENCE STUDY GROUP, de Onis Mercedes 
Published: August 2010

AbstractAim:To review the methods for generating windows of achievement for six gross motor developmental milestones and to compare the actual windows with commonly used motor development scales.Methods:As part of the WHO Multicentre Growth Reference Study, longitudinal data were collected to describe the attainment of six gross motor milestones by children aged 4 to 24 mo in Ghana, India, Norway, Oman and the USA. Trained fieldworkers assessed 816 children at scheduled visits (monthly in year 1, bimonthly in year 2). Caretakers also recorded ages of achievement independently. Failure time models were used to construct windows of achievement for each milestone, bound by the 1st and 99th percentiles, without internal demarcations.Results:About 90% of children achieved five of the milestones following a common sequence, and 4.3% did not exhibit hands‐and‐knees crawling. The six windows have age overlaps but vary in width; the narrowest is sitting without support (5.4 mo), and the widest are walking alone (9.4 mo) and standing alone (10.0 mo). The estimated 1st and 99th percentiles in months are: 3.8, 9.2 (sitting without support), 4.8, 11.4 (standing with assistance), 5.2, 13.5 (hands‐and‐knees crawling), 5.9, 13.7 (walking with assistance), 6.9, 16.9 (standing alone) and 8.2, 17.6 (walking alone). The 95% confidence interval widths varied among milestones between 0.2 and 0.4 mo for the 1st percentile, and 0.5 and 1.0 mo for the 99th.Conclusion:The windows represent normal variation in ages of milestone achievement among healthy children. They are recommended for descriptive comparisons among populations, to signal the need for appropriate screening when individual children appear to be late in achieving the milestones, and to raise awareness about the importance of overall development in child health.
Continuing Medical Education: Urogenital Complaints and Female Sexual Dysfunction (Part 2) (CME)
Authors: Wehbe Salim A. , Kellogg Susan , Whitmore Kristene 
Published: August 2010

ABSTRACT

Introduction
Female sexual dysfunction (FSD) is common in women with urogenital disorders that occur as a result of pelvic floor muscle and fascial laxity.

Aim
Provide a comprehensive review of FSD as it relates to common urogenital disorders including pelvic organ prolapse (POP) and stress urinary incontinence (SUI) and to discuss the impact of the surgical repair of these disorders on sexual function.

Methods
Systematic search of the medical literature using a number of related terms including FSD, POP, SUI, surgical repair, graft augmentation, complications, and dyspareunia.

Main Outcome Measures
Review of the medical literature to identify the relation between FSD and common urogenital disorders in women and to describe appropriate treatment strategies to improve quality of life (QOL) and sexual function.

Results
FSD is common in women with POP and SUI. Treatment options for POP and SUI include behavioral, pharmacological, and surgical methods which can also affect sexual function.

Conclusions
Sexual dysfunction is a common, underestimated complaint in women with POP and SUI. Treatment should be tailored toward improving sexual function and QOL. Surgical correction is generally beneficial but occasionally can result in negative alterations in sexual function. Patient selection and methods used for surgical repair are important factors in determining anatomical and functional success.
Sexual Complaints, Pelvic Floor Symptoms, and Sexual Distress in Women over Forty
Authors: Knoepp Leise R. , Shippey Stuart H. , Chen Chi Chiung Grace , Cundiff Geoffrey W. , Derogatis Leonard R. , Handa Victoria L. 
Published: August 2010

ABSTRACT

Introduction
The American Psychiatric Association recommends considering sexually related personal distress when assessing female sexual dysfunction. Currently, there is little data regarding the impact of sexual complaints on sexual distress.

Aim
To investigate the association between sexual complaints and perceived sexual distress in a population of ambulatory adult women.

Methods
Using the short forms of the Personal Experiences Questionnaire and Pelvic Organ Prolapse/Urinary Incontinence Sexual Function Questionnaire, we assessed sexual complaints among 305 women seeking outpatient gynecologic care. Depressive symptoms were quantified using the Center for Epidemiologic Studies Depression (CESD) score. Sexual distress was measured using the Female Sexual Distress Scale (FSDS). Using multivariable logistic regression, we compared sexual complaints between distressed and nondistressed women.

Main Outcome Measures
Sexual distress, defined by FSDS score ≥15.

Results
FSDS scores were available for 292/305 participants. Seventy-six (26%) scores reflected distress. Distressed women were more likely to be younger (55.2 ± 1.0 years vs. 56.7 ± 0.8 years, P = 0.017); have higher CESD scores (16.6 vs. 9.5, P = 0.001); and report decreased arousal (56.8% vs. 25.1%, P = 0.001), infrequent orgasm (54% vs. 28.8%, P = 0.001), and dyspareunia (39.7% vs. 10.6%, P = 0.001). Women with sexual distress were also more likely to report sexual difficulty related to pelvic floor symptoms, including urinary incontinence with sexual activity (9% vs. 1.3%, P = 0.005), sexual avoidance due to vaginal prolapse (13.9% vs. 1%, P = 0.001), or sexual activity restriction due to fear of urinary incontinence (14.9% vs. 0.5%, P = 0.001). After multivariate analysis, sexual distress was significantly associated with dyspareunia (odds ratio [OR] 3.11, P = 0.008) and depression score (OR 1.05, P = 0.006), and inversely associated with feelings of arousal during sex (OR 0.19, P = 0.001).

Conclusion
Our results indicate that sexually related personal distress is significantly associated with dyspareunia, depressive symptoms, and decreased arousal during sexual activity. This contributes to our understanding of how sexual complaints may adversely affect women’s quality of life.
Pulpal Temperature Changes During Desensitisation And Other Low‐Power Hard‐Tissue CO2 Laser Procedures
Authors: Sandford M.A. , Walsh L.J. 
Published: August 2010

AbstractThermal insult to pulpal tissue is recognised as a major limitation to the use of lasers for dental hard tissue procedures. This study examined thermal changes at the level of the dental pulp in human molar teeth irradiated with a CO2 dental laser using a pulsed mode of operation. Sectioned molar teeth were exposed, in vitro, to CO2 laser radiation. The laser parameters were those used clinically for laser desensitisation and laser‐enhanced fluoride treatment. Fissure regions and root surfaces were irradiated. For settings which might reasonably be used clinically, the temperature rise was not of a magnitude which would be expected to cause pulpal inflammation or necrosis. With regard to thermal properties of tooth structure, times taken to reach the maximum temperature reduced, and times taken to cool to baseline increased with increasing laser exposures.
Conservative Management Of A Large Periapical Lesion: Triumph And Tragedy
Authors: Cohn Steven A. 
Published: August 2010

AbstractTeeth that become devitalised before the completion of root maturation present many clinical challenges. These include the lack of an apical constriction, thin root walls that are prone to fracture and the presence of an associated large periapical lesion. The following case describes the treatment of such a case and its unfortunate outcome due to iatrogenic crown fracture.
Endodontic Treatment For A Molar Resorbed By A Dentigerous Cyst
Authors: Clarkson Roger 
Published: August 2010

AbstractA case is presented where an unerupted lower third molar in a 56‐year‐old woman developed a dentigerous cyst. The pressure of the enlarging cyst caused distal displacement of the third molar and resorption of the roots of the first molar. The first molar was treated endodontically prior to surgery on the cyst, and a thirteen year follow‐up shows successful healing.