bluuebunny/crossref_metadata_2025_split huggingface dataset:
Newborn Screening for Pompe Disease: Synthesis of the Evidence and Development of Screening Recommendations
Authors: Kemper Alex R. , Hwu Wuh-Liang , Lloyd-Puryear Michele , Kishnani Priya S. 
Published: November 2007

BACKGROUND. Pompe disease is a lysosomal storage disorder that leads to the accumulation of glycogen and subsequently to muscle weakness, organ damage, and death. Pompe disease is detectable through newborn screening, and treatment has become available recently.
OBJECTIVE. Our goal was to review systematically all available evidence regarding screening for infantile Pompe disease to help policy makers determine whether Pompe disease should be added to their state's newborn screening battery.
METHODS. We searched online databases, including Medline, clinicaltrials.gov, and the Computer Retrieval of Information on Scientific Projects database, as well as Web sites maintained by federal organizations (eg, the Food and Drug Administration) and other nonprofit or private organizations (eg, the March of Dimes and Genzyme Corp), by using the terms “glycogen storage disease type II,” “Pompe disease,” and “Pompe's disease.” We also obtained preliminary findings from a screening program in Taiwan. Data were critically appraised and extracted by 2 investigators, one who is an expert in systematic review methods and the other who is an expert in Pompe disease.
RESULTS. The prevalence of Pompe disease has been estimated to be ∼1 case per 40000. Small studies suggest that enzyme therapy is highly efficacious in infantile Pompe disease and that earlier intervention leads to improved outcomes. Screening cannot distinguish between infantile and late-onset Pompe disease. The current screening program in Taiwan has a high false-positive rate; however, the threshold was purposely set low to ensure that no case would be missed.
CONCLUSIONS. Pilot studies of screening are needed to identify the most efficacious strategy for screening and determine how to manage cases of late-onset Pompe disease before screening for Pompe disease is adopted widely by newborn screening programs.
Impact of Singular Excessive Computer Game and Television Exposure on Sleep Patterns and Memory Performance of School-aged Children
Authors: Dworak Markus , Schierl Thomas , Bruns Thomas , Strüder Heiko Klaus 
Published: November 2007

OBJECTIVE. Television and computer game consumption are a powerful influence in the lives of most children. Previous evidence has supported the notion that media exposure could impair a variety of behavioral characteristics. Excessive television viewing and computer game playing have been associated with many psychiatric symptoms, especially emotional and behavioral symptoms, somatic complaints, attention problems such as hyperactivity, and family interaction problems. Nevertheless, there is insufficient knowledge about the relationship between singular excessive media consumption on sleep patterns and linked implications on children. The aim of this study was to investigate the effects of singular excessive television and computer game consumption on sleep patterns and memory performance of children.
METHODS. Eleven school-aged children were recruited for this polysomnographic study. Children were exposed to voluntary excessive television and computer game consumption. In the subsequent night, polysomnographic measurements were conducted to measure sleep-architecture and sleep-continuity parameters. In addition, a visual and verbal memory test was conducted before media stimulation and after the subsequent sleeping period to determine visuospatial and verbal memory performance.
RESULTS. Only computer game playing resulted in significant reduced amounts of slow-wave sleep as well as significant declines in verbal memory performance. Prolonged sleep-onset latency and more stage 2 sleep were also detected after previous computer game consumption. No effects on rapid eye movement sleep were observed. Television viewing reduced sleep efficiency significantly but did not affect sleep patterns.
CONCLUSIONS. The results suggest that television and computer game exposure affect children's sleep and deteriorate verbal cognitive performance, which supports the hypothesis of the negative influence of media consumption on children's sleep, learning, and memory.
Association Between Iron-Deficiency Anemia and Stroke in Young Children
Authors: Maguire Jonathon L. , deVeber Gabrielle , Parkin Patricia C. 
Published: November 2007

OBJECTIVE. Iron-deficiency anemia occurs with a peak prevalence of 4% to 8% in children between 1 and 3 years of age. Case reports have suggested an association between iron-deficiency anemia in healthy children and ischemic stroke. Our objective was to investigate whether iron-deficiency anemia is associated with stroke in young children.
METHODS. A case-control study was conducted of case patients who were selected from the stroke registry at the Hospital for Sick Children (Toronto, Ontario, Canada) and control subjects selected from a database of healthy children who were prospectively enrolled in an outpatient setting. Children were aged 12 to 38 months and were previously healthy with no identifiable risk factors for stroke. Age, gender, mean corpuscular volume, platelet count, and hemoglobin and ferritin levels were collected. Iron-deficiency anemia was defined as a hemoglobin level of &lt;110 g/L, mean corpuscular volume &lt;73 fL, and serum ferritin level &lt;12 μg/L. Stroke was defined according to clinical and radiologic criteria.
RESULTS. Case (n = 15) and control (n = 143) subjects were similar with respect to median age and percentage of boys. Case patients had a lower median hemoglobin level and mean corpuscular volume and a higher median platelet count. Iron-deficiency anemia was significantly more common among case patients (8 [53%] of 15) than control subjects (13 [9%] of 143).
CONCLUSIONS. Previously healthy children with stroke were 10 times more likely to have iron-deficiency anemia than healthy children without stroke. Furthermore, children with iron-deficiency anemia accounted for more than half of all stroke cases in children without an underlying medical illness, which suggests that iron-deficiency anemia is a significant risk factor for stroke in otherwise healthy young children. Primary prevention and early identification of iron-deficiency anemia must remain a priority.
Inhaled Nitric Oxide for Preterm Infants: A Systematic Review
Authors: Barrington Keith J. , Finer Neil N. 
Published: November 2007

OBJECTIVE. Our goal was to determine whether, for preterm newborn infants with respiratory disease, inhaled nitric oxide reduced the rates of death, bronchopulmonary dysplasia, intracranial hemorrhage, or neurodevelopmental disability.
METHODS. We searched Medline, Embase, Healthstar, and the Cochrane Central Register of Controlled Trials using the search terms “nitric oxide,” “clinical trial,” and “newborn” and covering 1985–2006. We also searched abstracts of the Pediatric Academic Societies.
RESULTS. Eleven randomized, controlled trials of inhaled nitric oxide therapy for preterm infants were found. The trials were grouped into 3 categories according to the entry criteria, that is, entry in the first 3 days of life on the basis of oxygenation criteria (early rescue), enrollment after 3 days on the basis of elevated risk of bronchopulmonary dysplasia, and routine use for intubated preterm infants. Early rescue treatment based on oxygenation criteria did not seem to affect mortality or bronchopulmonary dysplasia rates. Routine use for intubated preterm infants showed a barely significant reduction in the incidence of the combined outcome of death or bronchopulmonary dysplasia (relative risk [RR]: 0.91 [95% confidence limits (CLs): 0.84, 0.99]). Later treatment based on the risk of bronchopulmonary dysplasia showed no significant effect on this outcome. Early rescue treatment showed a trend toward increased incidence of severe intracranial hemorrhage, whereas routine use for intubated preterm infants seemed to show a reduction in the incidence of either severe intracranial hemorrhage or periventricular leukomalacia (RR: 0.70 [95% CLs: 0.53, 0.91]).
CONCLUSIONS. Inhaled nitric oxide as rescue therapy for very ill preterm infants undergoing ventilation does not seem to be effective and may increase severe intracranial hemorrhage. Later use of inhaled nitric oxide to prevent bronchopulmonary dysplasia does not seem to be effective. Early routine use of inhaled nitric oxide for mildly sick, preterm infants seems to decrease the risk of serious brain injury and may improve rates of survival without bronchopulmonary dysplasia.
Long-term Immunologic Effects of Broad-Spectrum Aeroallergen Immunotherapy
Authors: Maloney Jennifer M. 
Published: November 2007

Limb SL, Brown KC, Wood RA, Eggleston PA, Hamilton RG, Adkinson NF Jr. Int Arch Allergy Immunol. 2006;140:245–251
PURPOSE OF THE STUDY. To evaluate the long-term clinical effects, skin tests, and specific immunoglobulin E (IgE) levels from subjects who had previously received broad-spectrum aeroallergen immunotherapy several years earlier.
STUDY POPULATION. Eighty-two polysensitized subjects who had previously been enrolled onto a randomized, double-blind, placebo-controlled trial of specific immunotherapy for treatment of childhood allergic asthma
METHODS. Eighty-two subjects (78% male; mean age: 23 years) previously enrolled onto a randomized, placebo-controlled trial of immunotherapy for the treatment of childhood allergic asthma were reevaluated in adulthood (mean follow-up interval: 10.8 years) by puncture skin tests and CAP-RAST levels for major aeroallergens. All subjects originally completed at least 18 months (median: 27 months) of maintenance active immunotherapy treatment or placebo injections without subsequent immunotherapy.
RESULTS. At adult follow-up, 36% of all skin tests to treatment allergens among subjects who received immunotherapy (n = 41) had significantly reduced intensity versus 26% of skin tests among placebo recipients (n = 41; P = .03). No significant differences were noted for individual treatment allergens. No significant differences were observed in the long-term changes of serum-specific IgE antibody levels for all or individual treatment allergens between immunotherapy treatment and placebo groups (P = .43). The treatment and placebo groups had a similar acquisition of new skin-test sensitivities from time of randomization in the original childhood trial to debriefing (15% vs 20%; P = .28) and to adult follow-up (30% vs 31%; P = .75).
CONCLUSIONS. Immunotherapy suppresses skin-test sensitivity 8 to 16 years after discontinuation of treatment, but long-term effects on specific IgE levels in serum are not observed. Broad-spectrum immunotherapy does not seem to affect the acquisition of new inhalant sensitivities.
REVIEWER COMMENTS. In this study, allergic-asthmatic children who participated in a double-blind, placebo-controlled immunotherapy trial were reevaluated as adults to determine if immunotherapy had long-lasting effects. This study revealed that asthmatic children who received immunotherapy to multiple allergens for a median of 27 months had limited long-term effects on the results of testing parameters routinely used in allergy practice. The long-term efficacy of immunotherapy and optimal duration of therapy are issues that remain open.
Use of Complementary and Alternative Medicine by Food-Allergic Patients
Authors: Goldsobel Alan B. 
Published: November 2007

Ko J, Lee JI, Muñoz-Furlong A, Li XM, Sicherer SH. Ann Allergy Asthma Immunol. 2006;97:365–369
PURPOSE OF THE STUDY. To determine the prevalence of complementary and alternative medicine (CAM) use, the types of CAM modalities used, and opinions regarding CAM in food-allergic patients.
STUDY POPULATION. A total of 442 individuals were polled, 95% of whom were parents of food-allergic children. Two groups were evaluated by using an anonymous questionnaire: attendees at a Food Allergy & Anaphylaxis Network conference in 2002 and a convenience sample from the Pediatric Allergy and Immunology practice of Mount Sinai Medical Center (New York, NY). Three hundred eighty individuals filled out questionnaires, equal numbers from both groups.
METHODS. A 19-item questionnaire was constructed to collect data on types, frequency, and opinions of CAM use, severity of food allergy, and demographic information.
RESULTS. Diagnostic modalities considered unproven or disproven (including immunoglobulin G4 testing, kinesiology, electrodermal skin testing, and provocation testing) were used by 22% of the respondents. Chiropractors (10%) were the most common CAM providers, followed by homeopaths (5%), acupuncturists (4%), and herbologists (4%). Of food-allergic CAM users, 33% reported using chiropractic, 33% homeopathy, 17% Nambudripad's allergy-elimination technique, 12% acupressure, 9% massage, 6% acupuncture, and 3% reflexology. Sources of information about CAM included friends (39%), family (28%), the Internet (8%), and television (6%). Only 49% of the participants reported CAM use to their regular physicians. On a scale from 0 (not effective) to 5 (very effective), patients found that CAM therapies were not particularly effective (mean score: 2.08). If available, an herbal therapy of equal efficacy, safety, and cost was preferred as compared with a pharmaceutical drug (37% vs 12%; P = .001).
CONCLUSIONS. Unproven or disproven diagnostic methods and CAM treatments were used by ∼20% of the respondents (most of whom were parents of food-allergic patients). Most of those who used CAM noted poor efficacy, but if given a choice, many would prefer herbal therapies to pharmaceutical drugs.
REVIEWER COMMENTS. Education regarding effective food-allergy tests and additional research on CAM therapies are needed.
Administration of Epinephrine for Life-Threatening Allergic Reactions in School Settings
Authors: Randolph Christopher 
Published: November 2007

McIntyre CL, Sheetz AH, Carroll CR, Young MC. Pediatrics. 2005;116:1134–1140
PURPOSE OF THE STUDY. To ascertain the incidence of anaphylaxis in schools, characterize the circumstances surrounding anaphylactic episodes, and evaluate practices that are used to manage students with life-threatening allergies.
STUDY POPULATION AND METHODS. School districts in Massachusetts (N = 109) that completed an epinephrine-administration form whenever epinephrine was injected at school. Data were obtained from September 2001 to August of 2003.
RESULTS. Forty-eight school districts noted a total of 159 administrations of epinephrine during the 2-year period of reporting. The individual was not known to have a life-threatening allergy in 24% of the cases. Thirty-one percent of the students who received epinephrine had allergy to multiple antigens, and 25% had allergy to tree nuts or peanuts only. Nineteen percent of the cases occurred outside the school building on a playground or when transporting them to or from school or on field trips. The registered school nurse in the health office administered the epinephrine in most cases. The average time from development of symptoms until epinephrine was delivered was 10 minutes. In 92% of the cases, the student involved was taken to a medical facility using the emergency medical system.
CONCLUSIONS. Anaphylactic reactions in schools, although not frequent, are not uncommon events. A systematic review of anaphylactic events that required epinephrine administration identified opportunities for improvement in the treatment of students with life-threatening allergies.
REVIEWER COMMENTS. The limitation of this study is that it was based on voluntary reporting. There are variations in reporting among regions in the state, but it is not possible to determine if the differences are attributable to reporting practices or actual difference in epinephrine administration. Because there were no unique identifiers for subjects in the study, there is no assurance that allergy events are not recurring repeatedly in the same student. In summary, a thorough program should be in place in the schools to evaluate, treat, and manage students with life-threatening anaphylaxis to foods.
Cardiopulmonary Exercise Testing in Children and Adolescents With Asthma Who Report Symptoms of Exercise-Induced Bronchoconstriction
Authors: Humayun Fawwaz , Leo Harvey L. 
Published: November 2007

Joyner BL, Fiorino EK, Matta-Arroyo E, Needleman JP. J Asthma. 2006;43:675–678
PURPOSE OF THE STUDY. To use cardiopulmonary exercise testing (CPET) to establish the cause of exercise limitation in a population of children with asthma who were reporting symptoms of exercise-induced bronchoconstriction (EIB).
STUDY POPULATION. A total of 42 children (aged 7 to 19 years) who continued to report exercise-associated symptoms attributed to asthma despite daily controller therapy were included in the study. There were 22 boys and 20 girls. All patients were receiving daily inhaled corticosteroids and had normal pulmonary function at the time of the study. Patients were excluded if they had underlying cardiac disease, had another chronic lung disease, or were unable to ride the cycle ergometer.
METHODS. Each patient's BMI was calculated, and baseline spirometry was performed. Then, the patients performed cycle ergometry with a ramp protocol to voluntary exhaustion to determine maximal oxygen consumption (V̇o2). Spirometry was repeated at intervals of 5 and 20 minutes. A decrease of 10% in forced expiratory volume in 1 second was considered a positive finding of EIB.
RESULTS. Ten patients (24%) developed EIB after CPET. There were no significant differences in BMI, BMI z score, V̇o2, or initial pulmonary function between the subjects who developed EIB and those who did not.
CONCLUSIONS. Exercise limitation without EIB was found in both obese and nonobese patients, suggesting that poor fitness is a problem independent of body habitus. Including CPET in the management of children with suspected EIB would provide a better understanding of the etiology of their symptoms and facilitate more appropriate treatment.
REVIEWER COMMENTS. This study illustrates how a patient's BMI does not directly correlate with fitness levels. Nonobese individuals can be just as out of shape as obese individuals. Given the trend toward more obesity in this country, it is important to not let any generalizations based on body habitus affect our judgment as physicians. Therefore, when a nonobese individual presents with symptoms similar to EIB, it may be prudent to have them undergo CPET before labeling them as asthmatic.
Effect of Albendazole Treatments on the Prevalence of Atopy in Children Living in Communities Endemic for Geohelminth Parasites: A Cluster-Randomized Trial
Authors: Simons Elinor 
Published: November 2007

Cooper PJ, Chico ME, Vaca MG, et al. Lancet. 2006;367:1598–1603
PURPOSE OF THE STUDY. To determine if repeated antihelminthic treatments with albendazole affect the prevalence of atopy and clinical indices of allergy among children who live in parasite-endemic communities.
STUDY POPULATION. Children (N = 2373) attending the second to seventh year of primary education at 1 of 68 rural schools in a tropical and subtropical region of Ecuador.
METHODS. Children were cluster randomized by school to receive albendazole treatment every 2 months for 12 months or no treatment. The primary outcome was the proportion of children with at least 1 positive skin-test result to an environmental allergen after 12 months; secondary outcomes included the proportion of children with reported allergy symptoms, flexural dermatitis, and exercise-induced bronchospasm. No placebo was used, but the investigators who evaluated the children were blinded to their treatment-group assignment.
RESULTS. At baseline, there was an inverse association between geohelminth infection and skin-test reactivity (odds ratio: 0.78; 95% confidence interval: 0.65–0.95). Of children who attended albendazole-allocated schools, 91.5% received all 7 albendazole doses; albendazole treatment by parents was reported for 29.4% of the children allocated to no-treatment schools. After 12 months, the prevalence of geohelminth infection declined from 69.3% to 20.5% in the treatment group and from 74.6% to 65.7% in the no-treatment group. There was no evidence of an increase in the prevalence of atopy among children who received albendazole (17%) compared with those in the no-treatment group (17%; age- and gender-adjusted odds ratio: 0.97; 95% CI: 0.68–1.39). Both groups had similar reported allergy symptoms, flexural dermatitis, and exercise-induced bronchospasm after 12 months.
CONCLUSIONS. After 12 months, the prevalence of atopy did not increase among school-aged children who were treated with albendazole, compared with children who were not treated.
REVIEWER COMMENTS. Management of allergic conditions has increasingly focused on prevention and reduction of risk factors. Small intervention studies have shown increased atopy associated with regular antihelminthic treatment. Although this study was larger, it had a high dropout rate (31%), and antihelminthic treatment of children in the no-treatment group may have biased the results toward the null. It is important to note that the protective effect of geohelminth exposure against atopy may be greater during the first few years of life than by school age. However, these results suggest that treatment of school-aged children with antihelminthics over 12 months does not put them at greater risk of atopy.