bluuebunny/crossref_metadata_2025 huggingface dataset:
Developmental Outcomes With Early Orthotopic Liver Transplantation for Infants With Neonatal-Onset Urea Cycle Defects and a Female Patient With Late-Onset Ornithine Transcarbamylase Deficiency
Authors: McBride Kim L. , Miller Geoffrey , Carter Susan , Karpen Saul , Goss John , Lee Brendan
Published: October 2004

Urea cycle defects (UCDs) typically present with hyperammonemia, the duration and peak levels of which are directly related to the neurologic outcome. Liver transplantation can cure the underlying defect for some conditions, but the preexisting neurologic status is a major factor in the final outcome. Multicenter data indicate that most of the children who receive transplants remain significantly neurologically impaired. We wanted to determine whether aggressive metabolic management of ammonia levels after early referral/transfer to a metabolism center and early liver transplantation would result in better neurologic outcomes. We report on 5 children with UCDs, ie, 2 male patients with X-linked ornithine transcarbamylase deficiency and 2 male patients with carbamoyl phosphate synthase deficiency, all of whom had neonatal presentations and underwent orthotopic liver transplantation before 1 year of age, and 1 female patient with partial X-linked ornithine transcarbamylase deficiency that was intractable to medical therapy, who underwent transplantation at 35 months of age. Developmental testing with the Griffiths scale was performed on 3 occasions each, 12 months apart, up to 45 months after transplantation. Full-scale indices for 3 children who underwent early transplantation showed average developmental quotients of 67. All 5 children had metabolic cures. There were no deaths (30-month survival rate: 100%). One child is currently listed for repeat transplantation because of bile duct stenosis and cirrhosis. We conclude that early liver transplantation and aggressive metabolic management improve early neurologic outcomes for children with UCDs, but longer follow-up monitoring is needed.
Diffusion-Weighted Magnetic Resonance Imaging in Term Perinatal Brain Injury: A Comparison With Site of Lesion and Time From Birth
Authors: Rutherford Mary , Counsell Serena , Allsop Joanna , Boardman James , Kapellou Olga , Larkman David , Hajnal Jo , Edwards David , Cowan Frances
Published: October 2004

Objective. The aim of this study was to establish a more objective method for confirming tissue injury in term neonates who present with early seizures that are believed to be hypoxic-ischemic in origin.

Methods. We studied the relationship between contemporaneous diffusion-weighted magnetic resonance imaging and conventional magnetic resonance imaging in 63 symptomatic term-born neonates and 15 control term infants performed in the neonatal period. Apparent diffusion coefficients (ADC) were obtained for multiple regions of the brain.

Results. ADC values in the 15 control infants were 1 (1–1.15) (median [range]) × 10−3/mm2/second in the thalami and 1.1 (1–1.3) × 10−3/mm2/second in the lentiform nuclei, 1.5 (1.3–1.7) × 10−3/mm2/second in the centrum semiovale, 1.6 (1.46–1.7) × 10−3/mm2/second in the anterior white matter (WM), and 1.55 (1.35–1.85) × 10−3/mm2/second in the posterior WM with little variation over time. ADC values were significantly reduced in the first week after severe injury to either WM or basal ganglia and thalami (BGT), but values normalized at the end of the first week and then increased during week 2. ADC values were either normal or increased in moderate BGT and WM lesions when compared with controls. ADC values < 1.1 × 10−3/mm2/second were always associated with WM infarction and values <0.8 × 10−3/mm2/second with thalamic infarction.

Conclusion. A reduced ADC soon after delivery allows the presence of tissue infarction to be confirmed at a time when conventional imaging changes may be subtle. However, as both moderate WM and BGT lesions may have normal or increased ADC values, a normal ADC value during the first week does not signify normal tissue. ADC values should always be measured in combination with visual analysis of both conventional and diffusion-weighed images for maximum detection of pathologic tissue, and the timing of the scan needs to be taken into account when interpreting the results.
Life-Threatening Human Metapneumovirus Pneumonia Requiring Extracorporeal Membrane Oxygenation in a Preterm Infant
Authors: Ulloa-Gutierrez Rolando , Skippen Peter , Synnes Anne , Seear Michael , Bastien Nathalie , Li Yan , Forbes John C
Published: October 2004

We present the first report in the literature of a child with human metapneumovirus pneumonia who required extracorporeal membrane oxygenation for survival. This was a 3-month-old premature boy from British Columbia, Canada, who developed severe respiratory failure, experienced failure of high-frequency oscillatory mechanical ventilation, and required extracorporeal membrane oxygenation support for 10 days. This case illustrates the importance of including this newly discovered pathogen among the causes of childhood pneumonia.
Summary of the Presentations at the Conference on Preventing Childhood Obesity, December 8, 2003
Authors: Lederman Sally Ann , Akabas Sharon R. , Moore Barbara J. , Bentley Margaret E. , Devaney Barbara , Gillman Matthew W. , Kramer Michael S. , Mennella Julie A. , Ness Andrew , Wardle Jane
Published: October 2004

Objective. Because of the rising rates of childhood obesity, we set out to determine what is known about its causes and what could be done to prevent additional increases.

Methodology. A meeting was convened of experts in areas that bear on prevention of obesity development during intrauterine life, infancy, and very early childhood. They presented recent data and their interpretations of the stage of our current knowledge in related areas. They also proposed possible useful interventions and future directions for research.

Findings. The speakers’ talks indicated that (1) breastfeeding as currently practiced seems to be significantly (albeit weakly) protective against obesity and should be encouraged as the preferred method of feeding infants for as long a duration as practical during the first year of life; (2) infant-feeding practices are changing in a way that may predispose to obesity (eg, soda and french fries are being fed to infants as young as 7 months of age), possibly altering taste preferences for foods and beverages that are energy dense and nutrient poor; (3) although little is known about parenting styles (eg, authoritative versus permissive), parenting style is likely to be a fruitful area of current research into childhood obesity etiology; and (4) the pattern of weight changes in the first few years of life may contribute to later risk of obesity.

Conclusions. Children’s obesity will continue to be a growing problem unless we improve understanding of the key factors likely to be operative during intrauterine life, infancy, and very early childhood, identify those in whom intervention would have the greatest effect, design and evaluate preventive interventions, and promote those that are successful.
Lead Poisoning From a Toy Necklace
Authors: VanArsdale Jessica L. , Leiker Richard D. , Kohn Mel , Merritt T. Allen , Horowitz B. Zane
Published: October 2004

A 4-year-old, previously healthy boy experienced intermittent abdominal pain for several weeks. He was diagnosed with probable gastroenteritis, but his symptoms worsened. Laboratory tests revealed normocytic anemia, and an abdominal radiograph showed a metallic foreign body in the stomach, without evidence of obstruction. Endoscopy resulted in the retrieval of a quarter and a medallion pendant from the stomach. A venous blood lead level measurement was extremely elevated, at 123 μg/dL (level of concern: ≥10 μg/dL). The medallion was tested by the state environmental quality laboratory and was found to contain 38.8% lead (388 000 mg/kg), 3.6% antimony, and 0.5% tin. Similar medallions purchased from toy vending machines were analyzed and were found to contain similarly high levels of lead. State health officials notified the US Consumer Product Safety Commission, which resulted in a national voluntary recall of >1.4 million metal toy necklaces.
Paraneoplastic Autoimmune Multiorgan Syndrome (Paraneoplastic Pemphigus) in a Child: Case Report and Review of the Literature
Authors: Lane Joshua E. , Woody Carol , Davis Loretta S. , Guill Margaret F. , Jerath Rita S.
Published: October 2004

Paraneoplastic autoimmune multiorgan syndrome, also known as paraneoplastic pemphigus, has been observed only rarely among children. We describe a 10-year-old boy with typical clinical and histologic findings of paraneoplastic pemphigus associated with Castleman’s disease. His disease was refractory to resection of the tumor and aggressive combination immunosuppressive therapies. The patient died 1 year after presentation, as a result of complications of bronchiolitis obliterans. This case is unusual because of the young age of the patient.
Newborn Screening for Lysosomal Storage Disorders: Clinical Evaluation of a Two-Tier Strategy
Authors: Meikle Peter J. , Ranieri Enzo , Simonsen Henrik , Rozaklis Tina , Ramsay Steve L. , Whitfield Phillip D. , Fuller Maria , Christensen Ernst , Skovby Flemming , Hopwood John J.
Published: October 2004

Objective. To evaluate the use of protein markers using immune-quantification assays and of metabolite markers using tandem mass spectrometry for the identification, at birth, of individuals who have a lysosomal storage disorder.

Methods. A retrospective analysis was conducted of Guthrie cards that were collected from newborns in Denmark during the period 1982–1997. Patients whose lysosomal storage disorder (LSD; 47 representing 12 disorders) was diagnosed in Denmark during the period 1982–1997 were selected, and their Guthrie cards were retrieved from storage. Control cards (227) were retrieved from the same period. Additional control cards (273) were collected from the South Australian Screening Centre (Australia).

Results. From 2 protein and 94 metabolite markers, 15 were selected and evaluated for their use in the identification of LSDs. Glycosphingolipid and oligosaccharide markers showed 100% sensitivity and specificity for the identification of Fabry disease, α-mannosidosis, mucopolysaccharidosis (MPS) IVA, MPS IIIA, Tay-Sachs disease, and I-cell disease. Lower sensitivities were observed for Gaucher disease and sialidosis. No useful markers were identified for Krabbe disease, MPS II, Pompe disease, and Sandhoff disease. The protein markers LAMP-1 and saposin C were not able to differentiate individuals who had an LSD from the control population.

Conclusions. Newborn screening for selected LSDs is possible with current technology. However, additional development is required to provide a broad coverage of disorders in a single, viable program.
Effects of a Primary Care-Based Intervention on Violent Behavior and Injury in Children
Authors: Borowsky Iris Wagman , Mozayeny Sara , Stuenkel Kristen , Ireland Marjorie
Published: October 2004

Objective. Although many major health care organizations have made recommendations regarding physicians' roles in preventing youth violence, the efficacy of violence prevention strategies in primary care settings remains to be empirically tested.

Methods. We conducted a randomized, controlled trial to evaluate the effects of an office-based intervention on children's violent behaviors and violence-related injuries. Children 7 to 15 years of age who presented at 8 pediatric practices and scored positive on a brief psychosocial screening test (n = 224) were randomly assigned to an intervention group (clinicians saw the screening test results during the visit and a telephone-based parenting education program was made available to clinicians as a referral resource for parents) or a control group (clinicians did not see the screening test results).

Results. Compared with control subjects, at 9 months after study enrollment, children in the intervention group exhibited decreases in aggressive behavior (adjusted mean difference: −1.71; 95% confidence interval [CI]: −2.89 to −0.53), delinquent behavior (adjusted mean difference: −0.71; 95% CI: −1.28 to −0.13), and attention problems (adjusted mean difference: −1.02; 95% CI, −1.77 to −0.26) on the Child Behavior Checklist. Children in the intervention group had lower rates of parent-reported bullying (adjusted odds ratio: 4.43; 95% CI: 1.87-10.52), physical fighting (adjusted odds ratio: 1.79; 95% CI: 1.11-2.87), and fight-related injuries requiring medical care (adjusted odds ratio: 4.70; 95% CI: 1.33-16.59) and of child-reported victimization by bullying (adjusted odds ratio: 3.23; 95% CI: 1.96-5.31).

Conclusions. A primary care-based intervention that includes psychosocial screening and the availability of a parenting education resource can decrease violent behavior and injury among youths.
Clinical Spectrum, Morbidity, and Mortality in 113 Pediatric Patients With Mitochondrial Disease
Authors: Scaglia Fernando , Towbin Jeffrey A. , Craigen William J. , Belmont John W. , Smith E. O’Brian , Neish Stephen R. , Ware Stephanie M. , Hunter Jill V. , Fernbach Susan D. , Vladutiu Georgirene D. , Wong Lee-Jun C. , Vogel Hannes
Published: October 2004

Objectives. The aim of this study was to elucidate the frequency of major clinical manifestations in children with mitochondrial disease and establish their clinical course, prognosis, and rates of survival depending on their clinical features.

Methods. We performed a retrospective review of the medical records of 400 patients who were referred for evaluation of mitochondrial disease. By use of the modified Walker criteria, only patients who were assigned a definite diagnosis were included in the study.

Results. A total of 113 pediatric patients with mitochondrial disease were identified. A total of 102 (90%) patients underwent a muscle biopsy as part of the diagnostic workup. A significant respiratory chain (RC) defect, according to the diagnostic criteria, was found in 71% of the patients who were evaluated. In this cohort, complex I deficiency (32%) and combined complex I, III, and IV deficiencies (26%) were the most common causes of RC defects, followed by complex IV (19%), complex III (16%), and complex II deficiencies (7%). Pathogenic mitochondrial DNA abnormalities were found in 11.5% of the patients. A substantial fraction (40%) of patients with mitochondrial disorders exhibited cardiac disease, diagnosed by Doppler echocardiography; however, the majority (60%) of patients had predominant neuromuscular manifestations. No correlation between the type of RC defect and the clinical presentation was observed. Overall, the mean age at presentation was 40 months. However, the mean age at presentation was 33 months in the cardiac group and 44 months in the noncardiac group. Twenty-six (58%) patients in the cardiac group exhibited hypertrophic cardiomyopathy, 29% had dilated cardiomyopathy, and the remainder (13%) had left ventricular noncompaction. Patients with cardiomyopathy had an 18% survival rate at 16 years of age. Patients with neuromuscular features but no cardiomyopathy had a 95% survival at the same age.

Conclusions. This study gives strong support to the view that in patients with RC defects, cardiomyopathy is more common than previously thought and tends to follow a different and more severe clinical course. Although with a greater frequency than previously reported, mitochondrial DNA mutations were found in a minority of patients, emphasizing that most mitochondrial disorders of childhood follow a Mendelian pattern of inheritance.
Targeted Tuberculin Skin Testing and Treatment of Latent Tuberculosis Infection in Children and Adolescents
Authors:   Pediatric Tuberculosis Collaborative Group
Published: October 2004

Comprehensive new guidelines for screening, targeted testing, and treating latent tuberculosis infection (LTBI) in children and adolescents are presented. The recent epidemiology of TB and data on risk factors for LTBI are reviewed. The evidence-based recommendations provided emphasize the paradigm that children and adolescents should be screened for risk factors by using a risk-factor questionnaire for TB and LTBI and tested with the tuberculin skin test only if ≥1 risk factor is present. The use of administrative or mandated tuberculin skin tests for entry to day care, school, or summer camp is strongly discouraged. Treatment regimens, suggestions to improve adherence, and methods to monitor toxicities are summarized. Children and adolescents with LTBI represent the future reservoir for cases of TB. Thus, detecting and treating LTBI in children and adolescents will contribute to the elimination of TB in the United States.
Cost-Effectiveness of Strategies That Are Intended to Prevent Kernicterus in Newborn Infants
Authors: Suresh Gautham K. , Clark Robin E.
Published: October 2004

Objective. There is concern about an increasing incidence of kernicterus in healthy term neonates in the United States. Although the incidence of kernicterus is unknown, several potential strategies that are intended to prevent kernicterus have been proposed by experts. It is necessary to assess the costs, benefits, and risks of such strategies before widespread policy changes are made. The objective of this study was to determine the direct costs to prevent a case of kernicterus with the following 3 strategies: (1) universal follow-up in the office or at home within 1 to 2 days of early newborn discharge, (2) routine predischarge serum bilirubin with selective follow-up and laboratory testing, and (3) routine predischarge transcutaneous bilirubin with selective follow-up and laboratory testing.

Methods. We performed an incremental cost-effectiveness analysis of the 3 strategies compared with current practice. We used a decision analytic model and a spreadsheet to estimate the direct costs and outcomes, including the savings resulting from prevented kernicterus, for an annual cohort of 2 800 000 healthy term newborns who are eligible for early discharge. We used a modified societal perspective and 2002 US dollars. With each strategy, the test and treatment thresholds for hyperbilirubinemia are lowered compared with current practice.

Results. With the base-case assumptions (current incidence of kernicterus 1:100 000 and a relative risk reduction [RRR] of 0.7 with each strategy), the cost to prevent 1 case of kernicterus was $10 321 463, $5 743 905, and $9 191 352 respectively for strategies 1, 2, and 3 listed above. The total annual incremental costs for the cohort were, respectively, $202 300 671, $112 580 535, and $180 150 494. Sensitivity analyses showed that the cost per case is highly dependent on the population incidence of kernicterus and the RRR with each strategy, both of which are currently unknown. In our model, annual cost savings of $46 179 465 for the cohort would result with strategy 2, if the incidence of kernicterus is high (1:10 000 births or higher) and the RRR is high (≥0.7). If the incidence is lower or the RRR is lower, then the cost per case prevented ranged from $4 145 676 to as high as $77 650 240.

Conclusions. Widespread implementation of these strategies is likely to increase health care costs significantly with uncertain benefits. It is premature to implement routine predischarge serum or transcutaneous bilirubin screening on a large scale. However, universal follow-up may have benefits beyond kernicterus prevention, which we did not include in our model. Research is required to determine the epidemiology, risk factors, and causes of kernicterus; to evaluate the effectiveness of strategies intended to prevent kernicterus; and to determine the cost per quality-adjusted life year with any proposed preventive strategy.
Counseling Parents and Children on Sun Protection: A National Survey of Pediatricians
Authors: Balk Sophie J. , O’Connor Karen G. , Saraiya Mona
Published: October 2004

Objective. To describe pediatricians’ attitudes toward skin cancer (SC), sun protection (SP) counseling, and the quantity and content of such counseling and to identify barriers to counseling.

Methods. An American Academy of Pediatrics Periodic Survey was mailed to 1616 randomly selected US members between October 2001 and February 2002. The response rate was 54.6%.

Results. More than 90% of pediatricians agreed that SC is a significant public health problem and that preventing episodic high exposures to the sun during childhood will reduce the risk of adult melanoma. However, only 22.3% of respondents reported counseling most patients in all age groups. Female pediatricians were more likely to counsel most patients; pediatricians located in the South and West and those who practice in hospital/clinic settings were least likely to counsel compared with those in other regions. Approximately half (53%) of pediatricians reported selectively counseling on the basis of patient characteristics The most important SP recommendation named was using a sunscreen with a sun protection factor ≥15. Only 38% of pediatricians rated SP as very important to their patients’ health compared with other topics such as use of car seats (86%), nutrition (79%), immunization issues (76%), and smoking/avoidance of environmental tobacco smoke (74%). The most frequently named barrier to SP counseling was lack of time (58% reporting).

Conclusions. Although the majority of pediatricians believe that SC prevention is a worthy issue, only a minority reported providing routine SP counseling to most patients in every age group, and most ranked SP lower in importance than other issues. Interventions might include programs and materials to educate patients and pediatricians alike. To have an effect on increasing rates of SC and SC mortality, a broader public health approach is needed as a complement to pediatricians’ counseling efforts.
The Pediatrician’s Role in the Prevention of Missing Children
Authors: Howard Barbara J. , Broughton Daniel D. ,   Committee on Psychosocial Aspects of Child and Family Health
Published: October 2004

This Clinical Report was retired December 2020.

In 2002, the Second National Incidence Studies of Missing, Abducted, Runaway, and Thrownaway Children report was released by the US Department of Justice, providing new data on a problem that our nation continues to face. This clinical report describes the categories of missing children, the prevalence of each, and prevention strategies that primary care pediatricians can share with parents to increase awareness and education about the safety of their children.
Providing a Primary Care Medical Home for Children and Youth With Cerebral Palsy
Authors: Cooley W. Carl ,   and Committee on Children with Disabilities
Published: October 2004

Children and youth with cerebral palsy present pediatricians with complex diagnostic and therapeutic challenges. In most instances, care also requires communication and comanagement with pediatric subspecialists and pediatric surgical specialists, therapists, and community developmental and educational teams. The importance of family resilience to the patient's well-being broadens the ecologic scope of care, which highlights the value of a primary care medical home from which care is initiated, coordinated, and monitored and with which families can form a reliable alliance for information, support, and advocacy from the time of diagnosis through the transition to adulthood. This report reviews the aspects of care specific to cerebral palsy that a medical home should provide beyond the routine health maintenance, preventive care, and anticipatory guidance needed by all children.
Guidelines and Levels of Care for Pediatric Intensive Care Units
Authors: Rosenberg David I. , Moss M. Michele ,   Section on Critical Care,   Committee on Hospital Care
Published: October 2004

This Policy Statement was retired January 2013.

The practice of pediatric critical care medicine has matured dramatically during the past decade. These guidelines are presented to update the existing guidelines published in 1993. Pediatric critical care services are provided in level I and level II units. Within these guidelines, the scope of pediatric critical care services is discussed, including organizational and administrative structure, hospital facilities and services, personnel, drugs and equipment, quality monitoring, and training and continuing education.
Fluoroethers and Fluoroamines
Authors: Costello Michael G. , Flynn Richard M. , Owens John G.
Published: October 2004

Perfluoroaliphatic ethers and tertiary amines are members of a class of unreactive compounds known in the industry as inert fluids. These compounds are prepared by electrochemical fluorination, direct fluorination using elemental fluorine or cobalt trifluoride fluorination. They are nontoxic, nonflammable, have densities of 1.7–1.9 g/cm
3
, surface tensions of 9–19 dyn/cm and lower kinematic viscosities than hydrocarbons. Their thermal stabilities are comparable to the perfluoroalkanes. Their chemical properties are characterized by their extreme unreactivity. The lower molecular weight compounds are used in the electronics industry as heat‐transfer fluids and electronic testing fluids. The higher molecular weight polymers are used as hazardous duty vacuum pump oils, specialty greases, and lubricants.

Hydrofluoroethers and hydrofluoropolyethers are new classes of highly fluorinated ethers having properties intermediate between the perfluoroethers and hydrocarbon ethers. They are low in toxicity, nonflammable, with densities of 1.4–1.7 g/cm
3
, surface tensions of 13–16 dyn/cm and low kinematic viscosity. The hydrofluoropolyethers are used as heat‐transfer fluids. The hydrofluoroethers are used as heat‐transfer fluids as well as precision cleaning solvents and solvents for specialty applications such as coating deposition.
Geothermal Energy
Authors: Mink Roy
Published: October 2004

Heat emanating from within the earth is one source of geothermal energy. This vast repository of energy is generated from the decay of natural radioisotopes and heat from the molten core of the earth.

Natural sources of geothermal fluids for heating and bathing have been utilized since prehistoric times. The useful applications of hydrothermal resources depend on the temperature of the extracted fluid. Relatively low temperature fluids can be effectively applied for purposes such as greenhouse heating, fish farming, and especially space heating. Waters at higher temperatures can be used for a variety of industrial processes and the generation of electricity. Transportation of hot fluid over more than a few kilometers is often economically impractical. It is then possible to apply the power generated by hydrothermal energy in a variety of ways and at distant locations. The effeciency of electrical generation is directly related to the thermal quality of the resource. To successfully compete with the multitude of energy sources available, geothermal energy must be available and retrievable in both a convenient and an economical manner. Conditions have only been met using hydrothermal geothermal resources in the form of hot water and steam but these particular hydrothermal resources are limited. Most of the world's accessible geothermal energy is found in rock that is hot but contains limited fluid. Although research and development have demonstrated success in extracting thermal energy on a limited basis, the vast deeper hot rock resource has not yet been shown to be an economically feasible source of energy on a scale large enough for practical use.
Hazardous Waste Incinerators
Authors: Silcox Geoffrey D. , Lighty Joann S. , Keener Melvin E.
Published: October 2004

Many of the hazardous wastes that are generated by industrial, municipal, and governmental facilities are safely destroyed by incineration. This article provides a summary of the amount of hazardous waste generated, the amount destroyed by incineration, an overview of the incineration process, the types of incinerators, air pollution control devices and emissions, challenges in operation of incinerators, and regulations. The incinerators that are considered include rotary kilns, fluidized beds, moving and fixed grates (hearths), liquid waste incinerators, cement kilns, light aggregate kilns, and halogen acid production furnaces.
Fluid Catalytic Cracking (FCC), Catalysts and Additives
Authors: Nee J.R.D. , Harding R.H. , Yaluris G. , Cheng W.C. , Zhao X. , Dougan T.J. , Riley J.R.
Published: October 2004

The Fluid Catalytic Cracking (FCC) unit remains the primary hydrocarbon conversion unit in the modern petroleum refinery. The ability to convert a variety of feed types (eg, gas oils, cracked gas oils, deasphalted gas oils, atmospheric/vacuum resids) into lighter, more valuable products makes the FCC process extremely versatile and more importantly, profitable.

The profitability of an FCC unit depends largely on the type of feed being processed and the FCC catalyst employed. Up until the early 1960s, amorphous silica/alumina catalysts were used to crack vacuum gas oils. As feedstocks have become heavier and the level of contaminant metals has increased, more sophisticated catalysts have been introduced that contain special metals trapping technologies. Zeolite and matrix technologies have evolved to maximize conversion of difficult feeds to valuable transportation fuels and light olefins.

Increasingly stringent environmental regulations have also changed the objective function of the modern refinery. In addition to the classical goals of optimizing FCC yields for high gasoline selectivity, minimum dry gas production, high octane number, or good bottoms cracking, today's refiner must also reduce gaseous pollutants that originate in the FCCU regenerator as well as produce gasoline with low levels of contaminants, notably sulfur. This has presented opportunities for catalyst manufacturers to develop additives that enable the refiner to reduce emissions of SO

x

and NO

x

from the FCCU regenerator and catalysts and additives that produce cleaner transportation fuels.
Cerebellar hypoplasia, zonular cataract, and peripheral neuropathy in trisomy 17 mosaicism
Authors: Terhal Paulien , Sakkers Ralph , Hochstenbach Ron , Madan Kamlesh , Rabelink Gwenda , Sinke Richard , Giltay Jacques
Published: October 2004

Trisomy 17 mosaicism in liveborns is an extremely rare chromosomal abnormality, with only three cases reported in the literature. Here we describe a 7‐year‐old boy with trisomy 17 mosaicism. The chromosome abnormality was detected by amniocentesis and was confirmed postnatally in cultured skin fibroblasts. The main clinical features were mental retardation and growth reduction, peripheral motor and sensory neuropathy, hypoplastic cerebellar vermis, zonular cataract, and body asymmetry. In our patient, and in the three earlier described cases, the additional chromosome 17 was detected in skin fibroblasts, not in peripheral lymphocytes. Molecular investigations excluded uniparental disomy of chromosome 17 in our patient. The extra chromosome 17 probably originated from a postzygotic mitotic nondisjunction of the maternal chromosome 17. In most cases of trisomy 17 mosaicism detected in amniocytes the chromosome abnormality seems to be confined to extra‐embryonic tissues and clinically normal children are born. If, however, there are also ultrasound abnormalities, the possibility of fetal trisomy 17 mosaicism should certainly be considered. If postnatal karyotyping is limited to blood the diagnosis of trisomy 17 mosaicism could easily be missed. Therefore, we recommend chromosome analysis to be based on cultured skin fibroblasts in all cases where mental retardation is accompanied by postnatal growth retardation, body asymmetry, peripheral neuropathy, and cerebellar hypoplasia or zonular cataract. © 2004 Wiley‐Liss, Inc.
Is SHORT syndrome another phenotypic variation of PITX2?
Authors: Karadeniz Nadide Nilüfer , Kocak‐Midillioglu Inci , Erdogan Derya , Bökesoy Isık
Published: October 2004

Even though responsible genetic loci and mode of inheritance for the Rieger syndrome have been well established, the mode of inheritance and the genetic basis for SHORT syndrome are still uncertain. The purpose of this paper is to document a familial translocation of t(1;4)(q31.2;q25), in a mother and her son manifesting Rieger syndrome with polycystic ovaries and SHORT syndrome, respectively. It is suggested that these two syndromes may be different expressions of the same gene, PITX2, localized at 4q25. Our patient is the second with the association of Rieger syndrome and polycystic ovaries, and thus this may not be coincidental, moreover insulin resistance‐related phenotypes, such as lipodystrophy and polycystic ovaries, can be major component of syndromes with Rieger eye malformation. © 2004 Wiley‐Liss, Inc.
Three patients with 9p deletions including DMRT1 and DMRT2: A girl with XY complement, bilateral ovotestes, and extreme growth retardation, and two XX females with normal pubertal development
Authors: Õunap K. , Uibo O. , Zordania R. , Kiho L. , Ilus T. , Õiglane‐Shlik E. , Bartsch O.
Published: October 2004

It is well documented that distal 9p monosomy can be associated with XY sex reversal. Recently, the possibility of DMRT1 and/or DMRT2 (the genes for doublesex and mab‐3 related transcription factor 1 and 2) being the sex determining genes(s) at 9p has been raised. DMRT1 and DMRT2 map near the 9p telomere, distal of marker D9S1779. We describe here three unrelated females with distal 9p monosomy, one with XY complement and two with XX complements. In each individual, fluorescent in situ hybridization predicted the loss of the DMRT genes. Patient 1, an XY individual with monosomy 9pter → p24.1 ∼ 24.2 and trisomy 7q32 → qter had normal female external genitalia, a blind ending vagina, no uterus, a Fallopian tube on the right, and bilateral ovotestes with primitive ovarian tissue. She also had extreme growth retardation. Around 80 cases of distal 9p monosomy have been reported previously, but there has been no report of ovotestes or gonads comprising ovarian tissue in a XY patient with 9p deletion. Findings in Patient 1 suggest that the phenotypic spectrum of the heterozygous DMRT deletion may include true hermaphroditism. Patients 2 and 3 were 12‐ and 14‐year‐old females with XX complements, normal external genitalia, and normal pubertal development. Patient 2 had pure monosomy 9pter → p23 and Patient 3 had monosomy 9pter → p22.3 ∼ 23 combined with trisomy 3pter → p23 ∼ 24. To date, detailed reports on the gonadal status of XX 9p‐females have been limited to prepubertal girls. Patients 2 and 3 are the first females reported to have distal 9p monosomy and a normal puberty. © 2004 Wiley‐Liss, Inc.
Titelbild: Molekulare Gyroskope: {Fe(CO)3}‐ und {Fe(CO)2(NO)}+‐Rotatoren in ei­nem dreispeichigen Stator; einfache Synthese durch Alkenmetathese (Angew. Chem. 41/2004)
Authors: Shima Takanori , Hampel Frank , Gladysz John A.
Published: October 2004

Dreifache Ringschluss‐Metathesen …︁…︁ liefern eine Klasse von molekularen Gyroskopen, die, wie J. A. Gladysz et al. in ihrer Zuschrift auf S. 5653 ff. erläutern, aus einer P‐Fe‐P‐Achse, einem {Fe(CO)2(L)}m+‐Rotator und drei über terminale Phosphoratome verbrückenden (CH2)n‐Speichen bestehen. Es sind wahrscheinlich die ersten Systeme, die die Konnektivität und Symmetrie eines Spielzeugkreisels genau wiedergeben.
Apolipophorin‐III‐like protein expressed in the antenna of the red imported fire ant, Solenopsis invicta Buren (Hymenoptera: Formicidae)
Authors: Guntur Kalyani V.P. , Velasquez Daniel , Chadwell Linda , Carroll Christopher , Weintraub Susan , Cassill J. Aaron , Renthal Robert
Published: October 2004

Antennal proteins of the male fire ant (Solenopsis invicta) were analyzed by two‐dimensional gel electrophoresis, with the objective of identifying pheromone‐binding proteins, which have not previously been found in ant antennae. The major low‐molecular weight protein found in the male fire ant antenna was subjected to Edman degradation to determine the N‐terminal amino acid sequence. Degenerate PCR primers based on this sequence were used to obtain a cDNA sequence corresponding to the full‐length protein sequence. In‐gel trypsin digestion followed by MALDI‐TOF mass spectrometry and HPLC‐ESI/MS/MS demonstrated that the protein gel spot contained only the protein corresponding to the cDNA sequence obtained by PCR. The sequence is similar to apolipophorin‐III, an exchangeable lipid‐binding protein. Fire ant apolipophorin‐III is expressed in the antenna as well as the head, thorax and abdomen. Arch. Insect Biochem. Physiol. 57:101–110, 2004. © 2004 Wiley‐Liss, Inc.
The angiotensin converting enzyme inhibitor captopril reduces oviposition and ecdysteroid levels in Lepidoptera
Authors: Vercruysse L. , Gelman D. , Raes E. , Hooghe B. , Vermeirssen V. , Van Camp J. , Smagghe G.
Published: October 2004

The role of angiotensin converting enzyme (ACE, peptidyl dipeptidase A) in metamorphic‐ and reproductive‐related events in the Egyptian cotton leafworm, Spodoptera littoralis (Lepidoptera, Noctuidae) was studied by using the selective ACE inhibitor captopril. Although oral administration of captopril had no effect on larval growth, topical administration to new pupae resulted in a large decrease of successful adult formation. Oviposition and overall appearance of adults emerging from treated larvae did not differ significantly from those emerging from non‐treated larvae. In contrast, topical or oral administration of captopril to newly emerged adults caused a reduction in oviposition. By evaluating the effect of captopril on ecdysteroid titers and trypsin activity, we revealed an additional physiological role for ACE. Captopril exerted an inhibitory effect on ecdysteroid levels in female but not in male adults. Larvae fed a diet containing captopril exhibited increased trypsin activity. A similar captopril‐induced increase in trypsin activity was observed in female adults. In male adults, however, captopril elicited reduced levels of trypsin activity. Our results suggest that captopril downregulates oviposition by two independent pathways, one through ecdysteroid biosynthesis regulation, and the other through regulation of trypsin activity. Apparently, fecundity is influenced by a complex interaction of ACE, trypsin activity, and ecdysteroid levels. Arch. Insect Biochem. Physiol. 57:123–132, 2004. © 2004 Wiley‐Liss, Inc.
Robotic Astronomy – Proceedings of the 3rd Potsdam Thinkshop on Robotic Astronomy
Authors: Strassmeier K. G. , Hessman F. V.
Published: October 2004

From July 12–15, 2004, the third Potsdam Thinkshop was held in the Hotel Dorint “Sanssouci” in Potsdam, Germany on the topic of ROBOTIC ASTRONOMY. Its aim was to bring together astronomers and technicians from any field in astronomy to review the current status of robotic telescope projects and to discuss the future science cases for such installations. Part of the “Thinkshop” concept of this meeting was to jointly think about new scientific projects tailored to the unique capabilities of modern robotic telescopes, small and large. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Radial velocities from automated telescopes
Authors: Pepe F. , Mayor M.
Published: October 2004

During the past years, precise radial‐velocity measurements have been the fundamental tool for the discovery and characterization of almost all the presently known extra‐solar planets. The increasing precision and efficiency of this technique has also opened new possibilities for the follow‐up of planetary transit candidates, as well as for other fields of astronomy, such as asteroseismology and stellar physics. The example of extra‐solar planets illustrates quite clearly the strong need for large observational surveys: 1) In order to get a complete view of the planet “zoo”, many stars of various types must be measured and followed up. 2) The detection efficiency increases enormously with the quality and the quantity of data points. Time sampling is a critical parameter. 3) The great diversity of known planetary systems requires covering observation time‐scales from one night to several years and even decades. A direct consequence of these factors is that survey programmes require a large amount of telescope time and a continuous follow‐up of the observations. In a context of limited resources and large amounts of data, automated telescopes will be of great help, or even required, to carry out these programmes. Based on our experience with CORALIE and HARPS, we shall try to define a series of “requirements” towards automated telescopes for precise radial‐velocity measurements. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Robotic telescopes and the public
Authors: Baruch J. E. F. , Hedges D. G. , Machell J. , Tallon C. J.
Published: October 2004

The development of robotic telescopes for access by the public is discussed. The importance of astronomy in providing easily accessible science and stimulating young minds with the beauty and mysteries of the cosmos is outlined in the light of the continuing need to inspire young people with science and make possible careers in science and engineering. The growing problems of seeing the stars in our cities due to light pollution and the increasing attraction of TV, computer games and the Internet are all reducing the levels of familiarity of young people with the night sky and their potential interest in astronomy. The ways in which robotic telescopes can reintroduce young people to the heritage of the night sky are outlined and various initiatives discussed around the returns that can be gained in bringing astronomy to the public. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Stellar activity and the long‐term use of robotic telescopes
Authors: Rodonò M. , Messina S. , Lanza A. F. , Cutispoto G.
Published: October 2004

A number of automated and robotic telescopes are nowadays devoted to the systematic monitoring of magnetically active stars and binary systems at several astronomical institutions, all over the world, and their number is steadily increasing. Standard equipments include wide– and narrow–band photometers and, more recently, spectroscopic capabilities. The long‐term time series that those telescopes are providing turn out to be of paramount importance in order to significantly progress in our understanding of solar‐like stellar activity of magnetic origin, that seemingly affect most of late‐type dwarfs and subgiants. Our principal aim is to illustrate which key parameters, that can be derived from such long‐term time series, determine the appearance and evolution of stellar activity phenomena in different astrophysical environments other than solar, and their role in determining the physical characteristics of starspots, their surface distribution, filling factor, migration in latitude and longitude, and evolution in time. By using spots as tracers of stellar rotation, reliable data on stellar differential rotation, the prime motor of magnetic activity, can be derived. Moreover, the activity cycle is the additional fundamental parameter that can be provided by long‐term time series. In order to properly address the study of stellar activity, an internationally coordinated network of 1‐2 m class robotic telescopes dedicated to multi‐wavelength systematic observations should be established. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Unsolved problems in observational astronomy. I. Focus on stellar spectroscopy
Authors: Pallavicini R. , Randich S.
Published: October 2004

We present the highlights of current observational programs in stellar optical spectroscopy carried out with 8‐10 m class telescopes as well as with smaller telescopes. Topics discussed include: 1. light elements abundances and their cosmological implications; 2. search for Population III stars and spectroscopy of extremely metal deficient stars; 3. abundances of different stellar populations in the Galaxy; 4. spectroscopy of resolved stars in Local Group galaxies; 5. Li and Be abundances and internal mixing in stars; 6. spectroscopy of very‐low mass stars and brown dwarfs; 7. radial velocity search of extrasolar planets; 8. stellar oscillations and asteroseismology; 9. stellar magnetic activity and Doppler imaging of stellar surface features. We also highlight the role that dedicated 1‐2 m automatic telescopes with spectroscopic capabilities can play in several fields of stellar optical spectroscopy. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
What makes an automated telescope robotic?
Authors: Granzer T.
Published: October 2004

Robotic telescopes are more and more commonly used in today's astronomical research. To optimize the scientific return the automation must not stop at a level, where a single observation can be performed by the robot. In fact it is necessary that target selection reflects the scientific needs of the different science programs, involving long‐term planing as well as shorttime optimization. In this paper, the approach pursued to solve this scheduling problem at the STELLA robotic observatory is reported. Hints for adopting the software package on other robotic facilities are outlined. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
The Liverpool telescope
Authors: Steele I. A.
Published: October 2004

The Liverpool Telescope is a 2.0 metre robotic telescope on La Palma. It achieved first light in July 2003, and began science operations in January 2004, with (supervised) robotic operation from April 2004. Unmanned operation is planned for October 2004. As a national, common‐user facility, the telescope must support over 50 science programmes and is equipped both with common user and experiment specific instruments. As an example, the SupIRCam instrument has been designed to obtain infrared light curves of Type Ia supernovae. This instrument achieved first light in July 2004. From August 2004 the telescope participates in the ROBONET‐1.0 network of 2.0 metre telescopes with the Faulkes Telescopes located in Hawaii and Australia, executing coordinated observations of GRB and exoplanet microlensing events. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
The MONET project and beyond
Authors: Hessman F. V.
Published: October 2004

The “MOnitoring NEtwork of Telescopes” (MONET) consists of two 1.2‐m imaging telescopes funded by the Alfried Krupp von Bohlen und Halbach Foundation and the Georg‐August‐Universität Göttingen and will be operated by the McDonald Observatory in West Texas and the South African Astronomical Observatory at Sutherland. Scheduled to go into full operation in 2005, it will be used to perform a variety of monitoring and survey observations over the whole sky, to aid observations by satellites and 10m‐class telescopes like the VLT, HET and SALT telescopes, and will be available to participating school classes all over the world. Through our development and use of Remote Telescope Markup Language (RTML), MONET should be one of the kernels of a growing international network of heterogeneous telescopes. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
MASTER: The Mobile Astronomical System of Telescope‐Robots
Authors: Lipunov V.M. , Krylov A. V. , Kornilov V. G. , Borisov G. V. , Kuvshinov D. A. , Belinsky A. A. , Kuznetsov M. V. , Potanin S. A. , Antipov G. A. , Tyurina N. V. , Gorbovskoy E. S. , Chilingaryan I.
Published: October 2004

We present the first Russian robot‐telescope designed to make prompt observations of gamma‐ray bursts (http://observ.pereplet.ru). The telescopes are near Moscow. The system of telescopes with prompt pointing rates connects to the internet. The main parameters are the following: Richter‐Slefogt system telescope (355 mm, f/d = 2.4); Richter‐Slefogt system telescope (200 mm, f/d = 2.4); Flugge system telescope (280 mm, f/d = 2.5); TV‐camera with 20 × 40 degree objective; two CCD cameras (Pictor 416); one CCD Apogee Camera AP16E. The type of mount is German with 8 grad/sec slew rate. MASTER images stars down to 19 magnitude in a 1 min exposure covering 6 square degrees. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)