bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pheochromocytoma and Paraganglioma in Children: A Review of Medical and Surgical Management at a Tertiary Care Center
Authors: Pham Tuan H. , Moir Christopher , Thompson Geoffrey B. , Zarroug Abdalla E. , Hamner Chad E. , Farley David , van Heerden Jon , Lteif Aida N. , Young William F. 
Published: September 2006

OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children.METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients &lt;18 years of age with histologically confirmed pheochromocytoma or paraganglioma.RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of &gt;6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 ± 32 months.CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of &gt;6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.
Child Maltreatment in the United States: Prevalence, Risk Factors, and Adolescent Health Consequences
Authors: Hussey Jon M. , Chang Jen Jen , Kotch Jonathan B. 
Published: September 2006

OBJECTIVES. The purpose of this study was to estimate the prevalence of child maltreatment in the United States and examine its relationship to sociodemographic factors and major adolescent health risks.METHODS. The National Longitudinal Study of Adolescent Health is a prospective cohort study following a national sample of adolescents into adulthood. The wave III interview, completed by 15 197 young adults in 2001–2002 (77.4% response rate), included retrospective measures of child maltreatment. We used these measures to estimate the prevalence of self-reported supervision neglect, physical neglect, physical assault, and contact sexual abuse during childhood. Next, we investigated the relationship between sociodemographic characteristics and maltreatment. Finally, we examined the association between child maltreatment and adolescent self-rated health; overweight status; depression; cigarette, alcohol, marijuana, and inhalant use; and violent behavior.RESULTS. Having been left home alone as a child, indicating possible supervision neglect, was most prevalent (reported by 41.5% of respondents), followed by physical assault (28.4%), physical neglect (11.8%), and contact sexual abuse (4.5%). Each sociodemographic characteristic was associated with ≥1 type of maltreatment, and race/ethnicity was associated with all 4. Each type of maltreatment was associated with no fewer than 8 of the 10 adolescent health risks examined.CONCLUSIONS. Self-reported childhood maltreatment was common. The likelihood of maltreatment varied across many sociodemographic characteristics. Each type of maltreatment was associated with multiple adolescent health risks.
Simultaneous Multigene Mutation Detection in Patients With Sensorineural Hearing Loss Through a Novel Diagnostic Microarray: A New Approach for Newborn Screening Follow-up
Authors: Gardner Phyllis , Oitmaa Eneli , Messner Anna , Hoefsloot Lies , Metspalu Andres , Schrijver Iris 
Published: September 2006

OBJECTIVE. The advent of universal newborn hearing screening in the United States and other countries, together with the identification of genes involved in the process of hearing, have led to an increase in both the need and opportunity for accurate molecular diagnosis of patients with hearing loss. Deafness and hearing impairment have a genetic cause in at least half the cases. The molecular genetic basis for the majority of these patients remains obscure, however, because of the absence of associated clinical features in ∼70% (ie, nonsyndromic hearing loss) of patients, genetic heterogeneity, and the lack of molecular genetic tests that can evaluate a large number of mutations across multiple genes.
DESIGN. We report on the development of a diagnostic panel with 198 mutations underlying sensorineural (mostly nonsyndromic) hearing loss. This panel, developed on a microarray, is capable of simultaneous evaluation of multiple mutations in 8 genes (GJB2, GJB6, GJB3, GJA1, SLC26A4, SLC26A5 and the mitochondrial genes encoding 12S rRNA and tRNA-Ser[UCN]).
RESULTS. The arrayed primer extension array for sensorineural hearing loss is based on a versatile platform technology and is a robust, cost-effective, and easily modifiable assay. Because hearing loss is a major public health concern and common at all ages, this test is suitable for follow-up after newborn hearing screening and for the detection of a genetic etiology in older children and adults.
CONCLUSIONS. Comprehensive and relatively inexpensive genetic testing for sensorineural hearing loss will improve medical management for affected individuals and genetic counseling for their families.
Changing Their Minds With Time: A Comparison of Hypothetical and Actual Reproductive Behaviors in Parents of Children With Cystic Fibrosis
Authors: Sawyer Susan M. , Cerritelli Belinda , Carter Lucy S. , Cooke Mary , Glazner Judith A. , Massie John 
Published: September 2006

OBJECTIVE. Newborn screening for cystic fibrosis, with appropriate counseling, enables carrier parents to be informed early about future reproductive choices. Previous studies have assessed attitudes toward reproductive decisions in a hypothetical pregnancy or have measured reproductive behaviors. We aimed to measure parent attitudes to reproductive technologies and to compare prospectively these attitudes with later reproductive behaviors.METHODS. Parents of children who had cystic fibrosis and were aged 2 to 7 years were surveyed at baseline using a written questionnaire that explored attitudes to prenatal testing and termination of pregnancy in a hypothetical pregnancy. Parent knowledge and access to genetic counseling services also were assessed. Five years later, we compared attitudes with actual reproductive behaviors.RESULTS. Fifty-six mothers participated at baseline, and 43 were resurveyed 5 years later. Parent knowledge of cystic fibrosis and genetics was very good. A total of 93% had met a genetic counselor at the time of diagnosis, and more than half had on at least 1 subsequent occasion. At baseline, 82% reported that they would be likely to have prenatal diagnosis in a subsequent pregnancy, and 56% reported that they would be likely to terminate an affected pregnancy. Twenty-seven mothers since had been pregnant, with prenatal diagnosis used in 33 of the 55 pregnancies. In 67%, the hypothetically reported behavior regarding use of prenatal testing was the same as their actual behavior. Five of the 33 tested pregnancies were affected; all ended in termination. Reproductive choices in relationship to the number of children wanted, together with attitudes toward prenatal diagnosis and termination of pregnancy, were dynamic over time, with decisions having changed in both directions.CONCLUSIONS. This cohort of parents has actively used reproductive technologies since the birth of a child who has cystic fibrosis that was diagnosed by newborn screening. The dynamic aspect of reproductive choices highlights the importance of ongoing access to genetic counseling beyond the initial period of diagnosis and education, regardless of whether parents report that they expect to use reproductive technologies.
Role of Pediatric Health Care Professionals in the Provision of Parenting Advice: A Qualitative Study With Mothers From 4 Minority Ethnocultural Groups
Authors: Dumont-Mathieu Thyde M. , Bernstein Bruce A. , Dworkin Paul H. , Pachter Lee M. 
Published: September 2006

OBJECTIVE. This study's aim was to elicit the perspectives of minority parents on their expectations of pediatric health care providers as a source of advice on “raising their child” and whether they would seek advice from these providers. A secondary aim was to demonstrate the value of qualitative methods for assessing parental attitudes in pediatric research.METHODS. Mothers with children between 3 and 12 years of age who identified themselves as African American, Jamaican, Haitian, or Puerto Rican were recruited from community sites. Audiotaped focus groups were conducted by trained moderators using an interview guide, to obtain the perspectives of the participants regarding the role of pediatric providers in the provision of parenting advice.RESULTS. Ninety-one mothers participated in a total of 20 focus groups, with 4 to 6 discussions per ethnocultural group. The focus groups revealed that, in general, parents do not look to child health care providers for advice on raising their children. The identified themes emphasized the importance of the relationship between providers and families. A few parents had the type of relationship within which the pediatrician already functioned as a provider of parenting advice. Physicians were considered skilled in the maintenance of physical health. The parents expressed a desire to receive more anticipatory guidance on developmental and behavioral stages and milestones. Pediatricians also served specific administrative functions valued by parents.CONCLUSIONS. Minority parents of preschool-aged and school-aged children do not view the primary care provider's role as including the provision of parenting advice. Expectations must be modified to enable health care professionals to function effectively in the role of advisor regarding parenting issues.
Surveillance for Influenza Admissions Among Children Hospitalized in Canadian Immunization Monitoring Program Active Centers, 2003–2004
Authors: Moore Dorothy L. , Vaudry Wendy , Scheifele David W. , Halperin Scott A. , Déry Pierre , Ford-Jones Elizabeth , Arishi Haider M. , Law Barbara J. , Lebel Marc , Le Saux Nicole , Grimsrud Karen , Tam Theresa 
Published: September 2006

OBJECTIVES. Influenza is a common childhood infection that may result in hospitalization. Our objectives were to (1) determine characteristics of children hospitalized for influenza and disease manifestations and (2) obtain baseline data before implementation of new recommendations for routine immunization of young children and their caretakers against influenza.METHODS. All of the children hospitalized with laboratory-confirmed influenza at 9 Canadian tertiary care hospitals during the 2003–2004 influenza season were identified from virology laboratory reports, and their charts were reviewed.RESULTS. There were 505 children admitted because of influenza. Fifty-seven percent were &lt;2 years old. Previously healthy children accounted for 58% of all of the cases. Pulmonary and neurologic disorders were the most common underlying chronic conditions. Fever and cough were the most frequent manifestations. Seizures occurred in 9% of cases. Serious complications included myocarditis (2), encephalopathy (6), and meningitis (1). There were 3 influenza-related deaths. Mean duration of hospitalization was 5.3 days. Twelve percent of children required ICU admission, and 6% required mechanical ventilation. Antibiotic therapy was administered in 77% of cases, and 7% received anti-influenza drugs. Information on influenza vaccination was available for 84 of 154 children identified as vaccine candidates. Twenty two had received vaccine, but only 7 children had been fully immunized &gt;14 days before the onset of illness.CONCLUSIONS. Healthy young children and children with chronic conditions are at risk for serious illness with influenza. Ongoing surveillance is needed to evaluate the impact of changing immunization recommendations on the burden of influenza illness in children.
Immunogenicity and Safety of an Inactivated Hepatitis A Vaccine Administered Concomitantly With Diphtheria-Tetanus-Acellular Pertussis and Haemophilus influenzae Type B Vaccines to Children Less Than 2 Years of Age
Authors: Nolan Terry , Bernstein Henry , Blatter Mark M. , Bromberg Kenneth , Guerra Fernando , Kennedy William , Pichichero Michael , Senders Shelly D. , Trofa Andrew , Collard Alix , Sullivan Diane C. , Descamps Dominique 
Published: September 2006

OBJECTIVE. The availability of a hepatitis A virus vaccine for infant and early childhood immunization could reduce the transmission of hepatitis A virus in the United States. This study evaluated the immunogenicity and safety of a hepatitis A virus vaccine (Havrix, GlaxoSmithKline Biologicals, Rixensart, Belgium) administered concomitantly with diphtheria-tetanus-acellular pertussis and Haemophilus influenzae type b vaccines to children &lt;2 years.
METHODS. In this open, comparative, multicenter study, 1084 healthy children aged 11 to 25 months were allocated (4:4:3:3:4 ratio) to 5 treatment groups based on age and previous vaccination history. Subjects 11 to 13 months of age received 2 doses of hepatitis A virus vaccine 6 months apart (N = 243). Subjects aged 15 to 18 months received 2 doses of hepatitis A virus vaccine 6 months apart (N = 241); or hepatitis A virus vaccine, diphtheria-tetanus-acellular pertussis, and H influenzae type b at month 0 and the second dose of hepatitis A virus vaccine 6 months later (N = 183); or diphtheria-tetanus-acellular pertussis and H influenzae type b at month 0 and hepatitis A virus vaccine at months 1 and 7 (N = 175). Subjects 23 to 25 months of age received hepatitis A virus vaccine at months 0 and 6 (N = 242). Immune responses were measured at baseline and 30 days after vaccine doses, and solicited and unsolicited adverse events were collected.
RESULTS. After 2 doses of hepatitis A virus vaccine, all of the subjects in all of the groups were seropositive. Coadministration of hepatitis A virus vaccine with diphtheria-tetanus-acellular pertussis and H influenzae type b vaccines did not impact the immunogenicity of the 3 vaccines, except for the antipertussis toxoid vaccine response, which was slightly decreased. Hepatitis A virus vaccine was well tolerated in children 11 to 25 months of age.
CONCLUSION. The administration of 2 doses of hepatitis A virus vaccine on a 0- and 6-month schedule starting at 11 to 13 months of age or at 15 to 18 months of age was as immunogenic and well tolerated as the administration of 2 doses in children 2 years of age. Immune responses to diphtheria-tetanus-acellular pertussis and H influenzae type b either given alone or coadministered with hepatitis A virus vaccine were similar except for antipertussis toxoid response.
Cost-effectiveness of 4 Neonatal Screening Strategies for Cystic Fibrosis
Authors: van den Akker-van Marle M. Elske , Dankert Hinke M. , Verkerk Paul H. , Dankert-Roelse Jeannette E. 
Published: September 2006

OBJECTIVES. The purpose of this work was to assess the costs of 4 neonatal screening strategies for cystic fibrosis in relation to health effects. In each strategy, the first test was the measurement of serum concentration of immunoreactive trypsin. The second step consisted of either a second immunoreactive trypsin test (strategy 1) or a multiple mutation analysis (strategy 2). In strategies 3 and 4, a third step was added to strategy 2: a second immunoreactive trypsin test (strategy 3) or an extended mutation analysis of the cystic fibrosis gene, that is, a denaturing gradient gel electrophoresis analysis (strategy 4).METHODS. We conducted an economic-modeling exercise in the Netherlands based on published data and expert opinions. Subjects were a hypothetical cohort of 200 000 neonates, the approximate number of children born annually in the Netherlands, and we assessed the costs and number of life-years gained as a result of neonatal screening for cystic fibrosis. The costs and effects of changes in reproductive decisions because of neonatal screening were also assessed.RESULTS. Immunoreactive trypsin + immunoreactive trypsin had the most favorable cost-effectiveness ratio of €24800 per life-year gained. Immunoreactive trypsin + DNA + denaturing gradient gel electrophoresis achieved more health effects than immunoreactive trypsin + DNA + immunoreactive trypsin at lower cost. The incremental costs per life-year gained of the immunoreactive trypsin + DNA + denaturing gradient gel electrophoresis strategy compared with the immunoreactive trypsin + immunoreactive trypsin strategy were €130700, whereas the incremental costs of the immunoreactive trypsin + DNA strategy compared with the immunoreactive trypsin + DNA + denaturing gradient gel electrophoresis strategy were €2154300. When changes in reproductive decisions as a result of neonatal screening are also taken into account, neonatal screening for cystic fibrosis may lead to financial savings of approximately €1.8 million annually, depending on the screening strategy used.CONCLUSIONS. Cystic fibrosis screening for neonates is a good economic option, and positive health effects can also be expected. Immunoreactive trypsin + immunoreactive trypsin and immunoreactive trypsin + DNA + denaturing gradient gel electrophoresis are the most cost-effective strategies.
Smoking Prevention and Cessation Intervention Delivery by Pediatric Providers, as Assessed With Patient Exit Interviews
Authors: Pbert Lori , Fletcher Kenneth E. , Flint Alan J. , Young Martin H. , Druker Susan , DiFranza Joseph 
Published: September 2006

OBJECTIVE. The goal was to evaluate the degree to which a smoking prevention and cessation intervention was delivered by providers to adolescents in the pediatric office setting.METHODS. Eight pediatric clinics in central Massachusetts were assigned randomly to either a special intervention (brief pediatric provider-delivered intervention plus peer counseling) or the usual care condition. Subjects (n = 2710) were adolescents 13 to 17 years of age, both smokers (smoked in the past 30 days) and nonsmokers/former smokers. The degree to which smoking prevention and treatment interventions were delivered by providers was assessed through patient exit interviews with adolescents after their clinic visits; interviews assessed the occurrence of 10 possible intervention steps.RESULTS. The percentage of providers engaging in the smoking interventions differed significantly between the special intervention and usual care conditions, according to adolescent reports in the patient exit interviews. For nonsmokers/former smokers, overall patient exit interview scores were 7.24 for the special intervention condition and 4.95 for the usual care condition. For current smokers, overall patient exit interview scores were 8.40 and 6.24 for the special intervention and usual care conditions, respectively. Intervention fidelity of special intervention providers was 72.2% and 84.0% for nonsmokers/former smokers and current smokers, respectively.CONCLUSIONS. Pediatric providers who receive training and reminders to deliver a brief smoking prevention and cessation intervention to adolescents in the context of routine pediatric primary care practice can do so feasibly and with a high degree of fidelity to the intervention protocol.
A Functional Magnetic Resonance Imaging Study of the Long-term Influences of Early Indomethacin Exposure on Language Processing in the Brains of Prematurely Born Children
Authors: Ment Laura R. , Peterson Bradley S. , Meltzer Jed A. , Vohr Betty , Allan Walter , Katz Karol H. , Lacadie Cheryl , Schneider Karen C. , Duncan Charles C. , Makuch Robert W. , Constable R. Todd 
Published: September 2006

BACKGROUND. Previous studies have demonstrated that indomethacin lowers the incidence and decreases the severity of intraventricular hemorrhage, as well as improves the cognitive outcome, in prematurely born male infants.OBJECTIVE. The purpose of this work was to use functional magnetic resonance imaging to test the hypothesis that neonatal indomethacin treatment would differentially affect brain activation across genders in school-aged, prematurely born children during performance of a language task.METHODS. Forty-seven prematurely born children (600–1250-g birth weight) and 24 matched term control subjects were evaluated using a functional magnetic resonance imaging passive language task and neurodevelopmental assessments that included the Wechsler Intelligence Scale for Children-III and the Peabody Picture Vocabulary Test-Revised. Neural activity was assessed during both phonologic and semantic processing in the functional magnetic resonance imaging protocol.RESULTS. Neurodevelopmental assessments demonstrated significant differences in full-scale, verbal, and performance intelligence quotient, as well as Peabody Picture Vocabulary Test scores, between the preterm and term control subjects. Rates of perinatal complications did not differ significantly across preterm treatment groups, but male preterm subjects randomly assigned to saline tended to have lower Peabody Picture Vocabulary Test-Revised scores than did all of the other preterm groups. During phonological processing, a significant treatment-by-gender effect was demonstrated in 3 brain regions: the left inferior parietal lobule, the left inferior frontal gyrus (Broca's area), and the right dorsolateral prefrontal cortex.CONCLUSIONS. These data demonstrate a differential effect of indomethacin administration early in postnatal life on the subsequent development of neural systems that subserve language functioning in these male and female preterm infants.