bluuebunny/crossref_metadata_2025_split huggingface dataset:
Molecular Epidemiology of Childhood Mitochondrial Encephalomyopathies in a Finnish Population: Sequence Analysis of Entire mtDNA of 17 Children Reveals Heteroplasmic Mutations in tRNAArg, tRNAGlu, and tRNALeu(UUR) Genes
Authors: Uusimaa Johanna , Finnilä Saara , Remes Anne M. , Rantala Heikki , Vainionpää Leena , Hassinen Ilmo E. , Majamaa Kari 
Published: August 2004

Objectives. Many heteroplasmic point mutations in tRNA genes of mitochondrial DNA (mtDNA) have been associated with human diseases. We recently reported on a prospective 7-year study in which we enrolled 116 consecutive children with undefined encephalomyopathy. Seventeen of them were found to have both a defect in the mitochondrial respiratory chain and abnormal ultrastructure of muscle mitochondria, suggesting a clinically probable mitochondrial encephalopathy.
Methods. We determined the frequency of mtDNA mutations in these 17 children by analyzing the entire sequence of mtDNA by conformation-sensitive gel electrophoresis and sequencing.
Results. Three heteroplasmic tRNA mutations that were considered to be pathogenic were detected. Two of the mutations were novel transitions, 10438A&gt;G in the tRNAArg gene and 14696A&gt;G in the tRNAGlu gene, whereas the third one was 3243A&gt;G, the common MELAS mutation. The mutant load was very high in the blood and skeletal muscle of the patients and markedly lower in the blood of asymptomatic maternal relatives. The 10438A&gt;G mutation changes the nucleotide flanking the anticodon, whereas 14696A&gt;G changes a nucleotide in the stem of the pseudouridine loop, creating a novel base pair and reducing the wobble.
Conclusions. Our results emphasize that the analysis of the entire sequence of mtDNA is worthwhile in the diagnostic evaluation of patients with clinically probable mitochondrial encephalomyopathy. The frequency of pathogenic mtDNA mutations was found to be 18% among children with biochemically and histologically defined mitochondrial disease, suggesting that the likelihood of nuclear DNA mutations in such a group is several times higher than that of mtDNA mutations.
Complications Associated With Image-Guided Gastrostomy and Gastrojejunostomy Tubes in Children
Authors: Friedman Jeremy N. , Ahmed Sabrina , Connolly Bairbre , Chait Peter , Mahant Sanjay 
Published: August 2004

Objective. To evaluate the complications associated with the image-guided insertion of gastrostomy (G) and gastrojejunostomy (GJ) tubes in children, performed by the retrograde percutaneous route.
Methods. A convenience sample of 208 charts of 840 patients recorded as having G and/or GJ tubes placed by the interventional radiology service in a 4-year period (1995–1999) at the Hospital for Sick Children in Toronto, Canada, were selected for review. Complications were categorized as major (including subcutaneous abscess, peritonitis, septicemia, gastrointestinal bleeding, and death) or minor.
Results. In total, 253 tubes (208 G tubes, 41 GJ tubes, 4 G and GJ tubes) were placed in the 208 patients reviewed. The median age at the time of insertion was 15 months (range: 7 days–18 years). The most common diagnostic category was neurologic disease (47%). The main indications for tube insertion were recorded as failure to thrive (57%) and risk of aspiration (47%). Major complications were seen in 5% of patients. Peritonitis was noted in 3%, and there was 1 death related to tube insertion (0.4%). Minor complications were found in 73% of patients, including tube dislodgement (37%), tube leakage (25%), and G-tube site skin infection (25%). GJ tubes had a higher rate than G tubes of obstruction, migration, dislodgement, leakage, and intussusception. Site infection, gastroesophageal reflux, and bleeding from the site were seen less frequently in patients with GJ tubes compared with G tubes.
Conclusion. G and GJ tubes placed by the image-guided retrograde percutaneous method are associated with a wide range of complications. The majority of these are minor and are predominantly related to tube maintenance, but major complications, including death, do occur.
Gastrointestinal Involvement in Chronic Granulomatous Disease
Authors: Marciano Beatriz E. , Rosenzweig Sergio D. , Kleiner David E. , Anderson Victoria L. , Darnell Dirk N. , Anaya-O'Brien Sandra , Hilligoss Dianne M. , Malech Harry L. , Gallin John I. , Holland Steven M. 
Published: August 2004

Objective. Chronic granulomatous disease (CGD) is a rare disorder of phagocyte oxidative metabolism. In addition to infectious complications, granulomatous lesions often involve hollow viscera, especially the gastrointestinal (GI) tract. The objective of this study was to evaluate the clinical presentation, prevalence, and consequences of GI involvement in patients with CGD.Methods. The medical records of 140 patients with CGD (67% X-linked) followed at the National Institutes of Health were reviewed and abstracted for GI manifestations. All available GI pathology was reviewed.Results. GI involvement was recorded in 46 (32.8%) of 140 patients with CGD, 89% of whom had X-linked inheritance. The median age at the time of initial GI manifestations was 5 years (range: 0.8–30 years); 70% of the affected patients presented with GI involvement in the first decade of life. Abdominal pain was the most frequent symptom (100%), and hypoalbuminemia was the most frequent sign (70%). Prednisone controlled symptoms and signs in the majority of affected patients, but relapse of symptoms occurred in 71%. GI involvement had no effect on mortality and was unassociated with interferon-γ use.Conclusion. GI involvement is a common and recurring problem in CGD, especially in those with X-linked inheritance. Currently, there is no clear evidence for an infectious cause. The frequency of GI involvement is unaffected by the use of interferon-γ and does not affect mortality. GI involvement should be sought in patients who have CGD with abdominal pain, growth delay, or hypoalbuminemia.
Inhaled Corticosteroids and the Risk of Fractures in Children and Adolescents
Authors: Schlienger Raymond G. , Jick Susan S. , Meier Christoph R. 
Published: August 2004

Objective. To determine whether children or adolescents who are exposed to inhaled corticosteroids (ie, beclomethasone, budesonide, fluticasone) are at a higher risk of having bone fractures compared with nonexposed individuals.
Methods. We performed a population-based nested case-control analysis using data from the United Kingdom–based General Practice Research Database. Within a base population of 273 456 individuals aged 5 to 79 years, we identified by International Classification of Diseases codes children or adolescents who were aged 5 to 17 years with a fracture diagnosis and up to 6 control subjects per case matched to cases on age, gender, general practice attended, calendar time, and years of history in the GPRD. We compared use of inhaled steroids before the index date between fracture cases and control patients.
Results. We identified 3744 cases and 21 757 matched control subjects aged 5 to 17 years. Current exposure to inhaled steroids did not reveal a substantially altered fracture risk compared with nonusers, even in individuals with current longer term exposure (ie, ≥20 prescriptions; adjusted odds ratio 1.15; 95% confidence interval: 0.89–1.48). In individuals with current or previous exposure to oral steroids, the adjusted odds ratio for current long-term inhaled steroid use compared with nonusers was 1.21 (95% confidence interval: 0.99–1.49).
Conclusions. Exposure to inhaled steroids does not materially increase the fracture risk in children or adolescents compared with nonexposed individuals.
Clinical Outcomes and Secondary Diagnoses for Infants Born With Hypoplastic Left Heart Syndrome
Authors: Connor Jean Anne , Arons Raymond R. , Figueroa Mayte , Gebbie Kristine M. 
Published: August 2004

Objective. To explore clinical outcomes and secondary diagnoses present at discharge for infants born with hypoplastic left heart syndrome (HLHS), from a national perspective.
Methods. We examined hospitalizations for infants ≤30 days of age who were born with HLHS, using hospital discharge data from the 1997 Kids Inpatient Database. To explore treatment choices, clinical outcomes, and resource use, we used International Classification of Diseases, 9th Revision, Clinical Modification diagnostic and procedure codes to classify discharges according to type of surgical intervention versus no surgical intervention. To investigate outcomes in more detail, we identified secondary diagnoses noted at discharge, using International Classification of Diseases, 9th Revision, Clinical Modification codes, and stratified results according to type of surgical intervention.
Results. Of a total of 550 patients with HLHS, 234 underwent the Norwood procedure, 17 underwent orthotopic heart transplantation, and 106 died in the hospital with no reported surgical intervention. Although we found no demographic variables to be significantly associated with the type of treatment received, discharged patients who died without surgical intervention were significantly more likely to have received care in hospitals identified as small (odds ratio [OR]: 1.5; 95% confidence interval [CI]: 1.03–3.1) or not children’s hospitals (OR: 2.02; 95% CI: 1.13–3.6). Secondary diagnoses of cardiac arrest (OR: 2.0; 95% CI: 1.1–3.4) and seizures (OR: 2.6; 95% CI: 1.2–5.5) occurred more frequently in orthotopic heart transplantation cases than in Norwood procedure cases.
Conclusions. These data from a national perspective reflect outcomes of infants with HLHS during a time when rates of initial survival after surgical intervention were considered to be improved. These findings may be useful to clinicians when they are considering and recommending initial medical and surgical strategies currently being proposed for the treatment of HLHS.
Body Mass Index, Waist Circumference, and Clustering of Cardiovascular Disease Risk Factors in a Biracial Sample of Children and Adolescents
Authors: Katzmarzyk Peter T. , Srinivasan Sathanur R. , Chen Wei , Malina Robert M. , Bouchard Claude , Berenson Gerald S. 
Published: August 2004

Objective. To derive optimal body mass index (BMI) and waist circumference thresholds for children and adolescents, to predict risk factor clustering.
Design. Cross-sectional receiver operating characteristic curve analysis.
Setting. The Bogalusa Heart Study, a community-based study of cardiovascular disease risk factors in early life.
Participants. A total of 2597 black and white children and adolescents, 5 to 18 years of age, who were examined between 1992 and 1994.
Main Outcome Measures. The presence or absence of ≥3 age-adjusted risk factors (low high-density lipoprotein cholesterol level, high low-density lipoprotein cholesterol level, high triglyceride level, high glucose level, high insulin level, and high blood pressure) was predicted from age-adjusted BMI and waist circumference values.
Results. The areas under the receiver operating characteristic curves were significantly different from 0.5 for both BMI and waist circumference for all gender/race groups, ranging from 0.73 to 0.82. The optimal BMI thresholds were at the 53rd and 50th percentiles for white and black male subjects, respectively, and at the 57th and 51st percentiles for white and black female subjects, respectively. Similarly, the optimal waist circumference thresholds were at the 56th and 50th percentiles for white and black male subjects, respectively, and at the 57th and 52nd percentiles for white and black female subjects, respectively. The sensitivity and specificity at the thresholds were similar for all gender/race groups, ranging from 67% to 75%.
Conclusions. The use of BMI and waist circumference for the prediction of risk factor clustering among children and adolescents has significant clinical utility. In this sample, race and gender differences in the optimal thresholds were minimal.
Hyperbilirubinemia Among African American, Glucose-6-Phosphate Dehydrogenase-Deficient Neonates
Authors: Kaplan Michael , Herschel Marguerite , Hammerman Cathy , Hoyer James D. , Stevenson David K. 
Published: August 2004

Background. Although glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is prevalent in African Americans, their risk of associated neonatal hyperbilirubinemia has not been prospectively studied.Objective. To compare hemolysis and the risk of hyperbilirubinemia among African American, G-6-PD-deficient neonates (study group) and G-6-PD-normal control subjects.Methods. Consecutive, healthy, term and near-term, male neonates born to African American mothers comprised the patient cohort. G-6-PD testing was performed with umbilical cord blood samples. Routine management included measurement of the end tidal carbon monoxide level corrected for ambient carbon monoxide level (ETCOc) within 4 hours after delivery (assessment of hemolysis), ≥1 predischarge bilirubin determination, and additional bilirubin testing as clinically indicated. Indications for phototherapy were identical for study patients and control subjects. Neonates were monitored for the first 1 week of life. ETCOc results, the incidence of hyperbilirubinemia (defined as a transcutaneous or plasma total bilirubin concentration of ≥95th percentile for the hour of life), and the need for phototherapy were compared between the G-6-PD-deficient and G-6-PD-normal groups.Results. Five hundred male patients were enrolled, of whom 64 (12.8%) were G-6-PD-deficient. ETCOc values (median and interquartile range) were higher among G-6-PD-deficient neonates than among control neonates (2.4 ppm [2.0–2.9 ppm] vs 2.1 ppm [1.7–2.5 ppm]). More G-6-PD-deficient neonates developed hyperbilirubinemia than did control subjects (14 of 64, 21.9%, vs 29 of 436, 6.7%; relative risk: 3.27; 95% confidence interval: 1.83-5.86), whereas 13 (20.3%) met the criteria for phototherapy, compared with 25 control subjects (5.7%) (relative risk: 3.53; 95% confidence interval: 1.91-6.56). No cases of kernicterus were observed.Conclusions. Within the African American neonatal population, there is a subgroup of G-6-PD-deficient infants with elevated rates of hemolysis, a higher incidence of hyperbilirubinemia, and a greater requirement for phototherapy, compared with G-6-PD-normal control subjects. These newborns should be monitored vigilantly for the development of hyperbilirubinemia.
Dating Violence and Associated Sexual Risk and Pregnancy Among Adolescent Girls in the United States
Authors: Silverman Jay G. , Raj Anita , Clements Karen 
Published: August 2004

Objectives. To assess the annual prevalence of physical violence from dating partners among a representative sample of sexually experienced adolescent girls attending US public and private high schools, as well as sexual risk behaviors and pregnancy among this population.Design, Setting, Participants, and Measures. Female students (9th through 12th grade) (N = 6864) participating in the 2001 National Youth Risk Behavior Survey completed measures of physical dating violence during the previous year, as well as assessments of health risk behaviors. Annual rates of physical dating violence were estimated for sexually experienced (n = 3085) and inexperienced girls. Multiple logistic regression models were constructed to assess whether physical dating violence in the previous year was associated with sexual health risks and pregnancy, after controlling for effects of potentially confounding demographic features and risk behaviors.Results. Slightly less than 1 of 5 sexually experienced US adolescent girls (17.7%) reported being intentionally physically hurt by a date in the previous year, and ∼1 of 25 girls (3.7%) who reported no sexual experience reported such violence. Dating violence among sexually experienced adolescent girls was related to increased risks for both sexual risk behaviors (eg, recent multiple sexual partners: odds ratio: 2.0; 95% confidence interval: 1.3–3.1) and pregnancy (odds ratio: 1.8; 95% confidence interval: 1.3–2.4).Conclusions. Dating violence is prevalent among US adolescent girls, especially those reporting having had sexual intercourse. Adolescent girls intentionally hurt by a date in the previous year are more likely to experience sexual health risks, including those increasing vulnerability to human immunodeficiency virus infection and other sexually transmitted infections, and to have been pregnant. Dating violence should be integrated into sexual health and pregnancy prevention programs, and greater efforts to identify girls experiencing dating violence are needed among those providing care related to adolescent sexual and reproductive health.
Natural History of Lipid Abnormalities and Fat Redistribution Among Human Immunodeficiency Virus-Infected Children Receiving Long-Term, Protease Inhibitor-Containing, Highly Active Antiretroviral Therapy Regimens
Authors: Taylor Perdita , Worrell Carol , Steinberg Seth M. , Hazra Rohan , Jankelevich Shirley , Wood Lauren V. , Zwerski Sheryl , Yarchoan Robert , Zeichner Steven 
Published: August 2004

Objective. To characterize the type and frequency of biochemical lipid abnormalities and physical changes in body composition associated with the use of protease inhibitor (PI)-containing antiretroviral therapy among human immunodeficiency virus-infected children treated for up to 6 years.
Methods. A retrospective study of human immunodeficiency virus-infected pediatric patients enrolled in research protocols between August 1995 and December 2001 was performed. All patients who had received a PI for ≥2 years as part of their investigational antiretroviral treatment regimens during the study period were eligible. Of the 110 patients identified as having received PI therapy, 94 met the study criteria.
Results. Of the 94 patients evaluated, 9 patients (10%) developed fat redistribution as well as dyslipidemia, 49 patients (52%) developed dyslipidemia without associated physical changes, and 36 patients (38%) exhibited no elevation of lipid levels or physical signs of fat redistribution. For all 9 patients with fat redistribution, the onset of the physical changes was closely associated with changes during pubertal development. Fat redistribution was also associated with lower viral loads and higher, more sustained levels of dyslipidemia. The onset of dyslipidemia and fat redistribution peaked between 10 and 15 years of age.
Conclusion. Among pediatric patients receiving PI therapy, there seems to be an age range in which children are at greater risk of developing hypercholesterolemia and subsequent fat redistribution, suggesting that unidentified physiologic changes associated with puberty may predispose pediatric patients treated with PI therapy to developing lipodystrophy.
Neonatal Genital Herpes Simplex Virus Type 1 Infection After Jewish Ritual Circumcision: Modern Medicine and Religious Tradition
Authors: Gesundheit Benjamin , Grisaru-Soen Galia , Greenberg David , Levtzion-Korach Osnat , Malkin David , Petric Martin , Koren Gideon , Tendler Moshe D. , Ben-Zeev Bruria , Vardi Amir , Dagan Ron , Engelhard Dan 
Published: August 2004

Objective. Genital neonatal herpes simplex virus type 1 (HSV-1) infection was observed in a series of neonates after traditional Jewish ritual circumcision. The objective of this study was to describe neonate genital HSV-1 infection after ritual circumcision and investigate the association between genital HSV-1 after circumcision and the practice of the traditional circumcision.
Methods. Eight neonates with genital HSV-1 infection after ritual circumcision were identified.
Results. The average interval from circumcision to clinical manifestations was 7.25 ± 2.5 days. In all cases, the traditional circumciser (the mohel) had performed the ancient custom of orally suctioning the blood after cutting the foreskin (oral metzitzah), which is currently practiced by only a minority of mohels. Six infants received intravenous acyclovir therapy. Four infants had recurrent episodes of genital HSV infection, and 1 developed HSV encephalitis with neurologic sequelae. All four mohels tested for HSV antibodies were seropositive.
Conclusion. Ritual Jewish circumcision that includes metzitzah with direct oral–genital contact carries a serious risk for transmission of HSV from mohels to neonates, which can be complicated by protracted or severe infection. Oral metzitzah after ritual circumcision may be hazardous to the neonate.
Abrasives
Authors: Rue Charles V. 
Published: August 2004

Abstract
An abrasive is a substance used to abrade, smooth, or polish an object. Usually, abrasive connotes a very hard substance, ranging up to the hardest material known, diamond. There are three basic forms of abrasives: grit (loose particles); bonded materials (particles bonded into wheels, segments, or stick shapes); and coated materials (particles bonded to paper, plastic, cloth, or metal). Properties of abrasive materials include hardness, toughness, ie, ability of an abrasive grit to withstand impact without cracking, spalling, or shattering, melting temperature, chemical reactivity, thermal conductivity, and fracture characteristics. Naturally occurring abrasives remain an important item of commerce, though synthetic abrasives, eg, diamonds, corundum, emery, garnet, silica, sandstone, tripoli, pumice, and pumicite, fill many of their former uses. Manufactured abrasives include silicon carbide, fused aluminum oxide, synthetic diamond, cubic boron nitride, and grit. Coated abrasives consist of a flexible backing which holds a coating of abrasive grains. Grinding wheels are the most important bonded abrasive product. The latter are specified by a standard marking system used throughout most of the world, allowing the user to recognize the type and size of the abrasive. Both natural and manufactured abrasive grains are used loose in such operations as polishing, buffing, and lapping.
Acrylonitrile Polymers, ABS Resins
Authors: Kulich Donald M. , Pace John E. , Fritch Leroy W. , Brisimitzakis Angelo 
Published: August 2004

AbstractAcrylonitrile–butadiene–styrene (ABS) polymers are composed of elastomer dispersed as a grafted particulate phase in a thermoplastic matrix of styrene and acrylonitrile copolymer (SAN). The presence of SAN grafted onto the elastomeric component, usually polybutadiene or a butadiene copolymer, compatabilizes the rubber with the SAN component. Property advantages provided by this graft terpolymer include excellent toughness, good dimensional stability, good processibility, and chemical resistance. The system is structurally complex. This allows considerable versatility in the tailoring of properties to meet specific product requirements. Consequently, research and development in ABS systems is active. Numerous grades of ABS are available, including new alloys and specialty grades for high heat, flaming‐retardant, or static dissipative product requirements. Good chemical resistance combined with the relatively low water absorptivity\documentclass{article}\usepackage{amssymb}\pagestyle{empty}\begin{document}${{(}{\rm{<}}1{\%}{)}}$\end{document}results in high resistance to staining agents. Antioxidants substantially improve oxidative stability. Applications involving extended outdoor exposure require the use of stabilizing additives, pigments, and protective coatings. In manufacturing, grafting is achieved by the free‐radical copolymerization of styrene and acrylonitrile monomers. The three commercial processes for manufacturing ABS are emulsion, mass, and mass‐suspension. ABS is sold as an unpigmented product for on‐line coloring using color concentrates during molding, or as precolored pellets. ABS can be processed by compression and injection molding, extrusion, calendering, and blow‐molding. Post‐processing operations include cold forming, painting, and adhesive bonding. As a “bridge” polymer between commodity plastics and higher performance engineering thermoplastics, ABS has become the largest selling engineering thermoplastic.