bluuebunny/crossref_metadata_2025_split huggingface dataset:
Familial Mediterranean Fever: Clinical and Genetic Characterization in a Mixed Pediatric Population of Jewish and Arab Patients
Authors: Brik Riva , Shinawi Marwan , Kepten Ilana , Berant Moshe , Gershoni-Baruch Ruth 
Published: August 2004

Objective.
Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease which primarily affects non-Ashkenazi Jews, Armenians, Arabs, and Turks. The gene responsible for the disease (MEFV/FMF) has been recently identified. Four common mutations in exon 10 of theMEFV gene seem to account for 86% of the DNA variations identified in patients with FMF. We conducted a phenotype/genotype correlation study in a mixed population of Jewish and Arab children with FMF.

Study Design.
Seventy patients clinically diagnosed as having FMF underwent molecular genetic studies using polymerase chain reaction and restriction endonuclease digestion methods to detect the presence of the four mutations (M694V, M680I, V726A, M694I). We then correlated the presence of each mutation with ethnic origin, age of onset, clinical manifestations, disease severity, and occurrence of amyloidosis.

Results.
The M694V mutation, which is predominant in non-Ashkenazi Jews, was found in 92% of our Jewish patients and in only 30% of the Arab patients. All four mutations were identified among 94% of the Arab patients, but with no particular prevalence for any one of them. The presence of a homozygous M694V mutation was significantly associated with a more severe form of the disease: the clinical onset of the disease manifested at an earlier age; the number of attacks per month was higher; the global assessment by the treating physician and the severity of pain scored higher; and arthritis was more frequent. Only patients with the M694V mutation had a family history of amyloidosis. No association was found between the type of mutation and the predominance of fever, abdominal pain, pleuritis, skin eruption, or response to colchicine in the clinical picture.

Conclusions.
Homozygosity for the M694V mutation, predominant among North African Jews, is associated with a severe course and prognosis for FMF. This mutation is less common among Arabs and, when present, occurs almost only in heterozygous form. In Arab patients, the disease tends to run a milder course and seems to bear a better prognosis. The phenotype/genotype patterns that are evident from our study of a mixed series of Jewish and Arab children with FMF might provide a rational basis for counseling about the natural history of the disease and for clinical treatment of FMF patients and their families.
The Relation of Overweight to Cardiovascular Risk Factors Among Children and Adolescents: The Bogalusa Heart Study
Authors: Freedman David S. , Dietz William H. , Srinivasan Sathanur R. , Berenson Gerald S. 
Published: August 2004

Background.Although overweight and obesity in childhood are related to dyslipidemia, hyperinsulinemia, and hypertension, most studies have examined levels of these risk factors individually or have used internal cutpoints (eg, quintiles) to classify overweight and risk factors.Objective.We used cutpoints derived from several national studies to examine the relation of overweight (Quetelet index, &gt;95th percentile) to adverse risk factor levels and risk factor clustering.Design.The sample consisted of 9167 5- to 17-year-olds examined in seven cross-sectional studies conducted by the Bogalusa Heart Study between 1973 and 1994.Results.About 11% of examined schoolchildren were considered overweight. Although adverse lipid, insulin, and blood pressure levels did not vary substantially with the Quetelet index at levels &lt;85th percentile, risk factor prevalences increased greatly at higher levels of the Quetelet index. Overweight schoolchildren were 2.4 times as likely as children with a Quetelet index &lt;85th percentile to have an elevated level of total cholesterol. Odds ratios for other associations were 2.4 (diastolic blood pressure), 3.0 (low-density lipoprotein cholesterol), 3.4 (high-density lipoprotein cholesterol), 4.5 (systolic blood pressure), 7.1 (triglycerides), and 12.6 (fasting insulin). Several of these associations differed between whites and blacks, and by age. Of the 813 overweight schoolchildren, 475 (58%) were found to have at least one risk factor. Furthermore, the use of overweight as a screening tool could identify 50% of schoolchildren who had two or more risk factors.Conclusions.Because overweight is associated with various risk factors even among young children, it is possible that the successful prevention and treatment of obesity in childhood could reduce the adult incidence of cardiovascular disease.overweight, lipids, blood pressure, insulin, children, adolescents.
Sociodemographic Factors Identify US Infants at High Risk of Injury Mortality
Authors: Scholer Seth J. , Hickson Gerald B. , Ray Wayne A. 
Published: August 2004

Objectives.To identify sociodemographic predictors of infant injury mortality and to compare trends in injury mortality rates for high- and low-risk US infants from 1985 to 1991.Design.Historical cohort.Setting/Study Participants.The National Center for Health Statistics linked US infants (&lt;1 year) born from 1985 to 1991 with death certificates.Main Outcome Measures.Multivariate regression was used to identify sociodemographic factors associated with injury mortality. The adjusted relative risks (RRs) of maternal age, education, marital status, number of other children, and infant birth weight were used to categorize infants into risk groups. We compared trends in injury rates for the highest and lowest risk groups.Results.There were 5963 injury deaths and 18.6 million infant years or 32.1 injury deaths per 100 000 infant years. Highest risk infants were born to mothers who were younger than 20 years compared with older than 30 years (RR, 3.25; 95% CI, 2.92–3.63), had less than a high school education compared with a college education (RR, 2.22; 95% CI, 1.95–2.53), had more than 2 other children compared with no other children (RR, 3.15; 95% CI, 2.88–3.45), were unmarried (RR, 1.67; 95% CI, 1.57–1.78), or had birth weights ≤1500 g compared with &gt;2500 g (RR, 3.36; 95% CI, 2.94–3.84). Infants in the highest risk group (21.0% of the population) had a &gt;10-fold increased risk of injury mortality compared with the lowest risk group (18.1% of the population) and there was no evidence that this disparity was narrowing.Conclusions.Sociodemographic predictors of infant injury mortality include maternal age, education, number of other children, marital status, and infant birth weight. Based on these factors, 1 in 5 infants in the United States can be identified at birth as having a &gt;10-fold increased risk of injury mortality compared with infants in lowest risk group. Programs to reduce injuries in these high-risk groups are urgently needed.
Hepatitis B Vaccine Administered to Children and Adolescents at Yearly Intervals
Authors: Halsey Neal A. , Moulton Lawrence H. , O'Donovan J. Crossan , Walcher J. Ronald , Thoms Mary Lou , Margolis Harold S. , Krause David S. 
Published: August 2004

Objective.
Hepatitis B vaccines are usually administered on a schedule of 0, 1 to 2, and 6 months. Longer intervals between the second and third doses have been studied, but the effectiveness of hepatitis B vaccine administered at intervals of &gt;2 months between the first and second doses have not been studied. Our objective was to compare the antibody response in recipients of Engerix-B hepatitis B vaccine administered at 12-month intervals to the response to vaccine administered at 0-, 1-, and 6-month intervals.

Methods.
A total of 389 children, 5 through 16 years of age, were randomized to receive Engerix-B (10 mg) at a schedule of either 0-, 1-, and 6-month intervals or 0-, 12-, and 24-month intervals. Blood was drawn before and 1 month after the third dose.

Results.
Immediately before the third dose of vaccine, 92.3% of children who received vaccine on the 0-, 1-, and 6-month schedule and 88.8% of children who received the 0-, 12-, and 24-month schedule had antibody to hepatitis B surface (anti-HBs) antigen concentrations ≥10 mIU/mL. Of the children in the 0-, 1-, and 6-month schedule, 95% received the third dose according to protocol versus 90% of those in the 0-, 12-, 24-month schedule. The geometric mean anti-HBs concentration just before the third dose for recipients of the 0-, 1-, and 6-month schedule (117.9 mIU/mL) was somewhat lower than that for the children who had received vaccine on the 0-, 12-, and 24-month schedule (162.1 mIU/mL). One month after the third dose, &gt;98% of all children had anti-HBs concentrations ≥10 mIU/mL and high geometric mean antibody concentrations were observed in both groups: 5687 mIU/mL for children on the 0-, 1-, and 6-month schedule and 3159 mIU/mL for children on the 0-, 12-, and 24-month schedule. Body mass index was correlated inversely with final antibody concentration, but age was not a factor after adjustment for body mass index.

Discussion.
Engerix-B administered on a 0-, 12-, and 24-month schedule is highly immunogenic. Providers should consider this alternate immunization schedule for children who are at low risk of immediate exposure to hepatitis B infections. vaccine, hepatitis B, hepatitis B vaccine, antigen, dose, schedule, immunization, adolescent.
Prevalence of IgA-Antiendomysium and IgA-Antigliadin Autoantibodies at Diagnosis of Insulin-Dependent Diabetes Mellitus in Swedish Children and Adolescents
Authors: Carlsson Anneli K. , Axelsson Irene E. M. , Borulf Stefan K. , Bredberg Anders C. A. , Lindberg Bengt A. , Sjöberg Klas G. , Ivarsson Sten-A. 
Published: August 2004

Objective.
This study was conducted to investigate the prevalence of celiac disease (CD) in children and adolescents at diagnosis of insulin-dependent diabetes mellitus (IDDM) before insulin treatment was started.

Material and Methods.
At diagnosis of IDDM, and before treatment was started, 115 children and adolescents were screened for IgA- antiendomysium (EMA) and IgA-antigliadin antibodies (AGA). Those found to be EMA-positive and/or AGA-positive were investigated further with intestinal biopsy.

Results.
Of the 115 patients, 2 had known CD at diagnosis of IDDM; of the remainder of patients, 6% (7/113) were found to be EMA-positive and 9% (10/113) were found to have AGA levels above normal. Of the 6 patients who underwent biopsy, 5 manifested villous atrophy. In addition, 2 patients with high EMA and AGA antibody titers refused biopsy, and 4 patients with low EMA and/or AGA titers were found to have normal titers at control before biopsy decision.

Conclusion.
Because the prevalence of CD at diagnosis of IDDM would seem to be 6% to 8%, screening for CD seems to be justified among patients with newly diagnosed IDDM.
Language Barriers and Resource Utilization in a Pediatric Emergency Department
Authors: Hampers Louis C. , Cha Susie , Gutglass David J. , Binns Helen J. , Krug Steven E. 
Published: August 2004

Background.Although an inability to speak English is recognized as an obstacle to health care in the United States, it is unclear how clinicians alter their diagnostic approach when confronted with a language barrier (LB).Objective.To determine if a LB between families and their emergency department (ED) physician was associated with a difference in diagnostic testing and length of stay in the ED.Design.Prospective cohort study.Methods.This study prospectively assessed clinical status and care provided to patients who presented to a pediatric ED from September 1997 through December 1997. Patients included were 2 months to 10 years of age, not chronically ill, and had a presenting temperature ≥38.5°C or complained of vomiting, diarrhea, or decreased oral intake. Examining physicians determined study eligibility and recorded the Yale Observation Score if the patient was &lt;3 years old, and whether there was a LB between the physician and the family. Standard hospital charges were applied for each visit to any of the 22 commonly ordered tests. Comparisons of total charges were made among groups using Mann-Whitney U tests. Analysis of covariance was used to evaluate predictors of total charges and length of ED stay.Results.Data were obtained about 2467 patients. A total of 286 families (12%) did not speak English, resulting in a LB for the physician in 209 cases (8.5%). LB patients were much more likely to be Hispanic (88% vs 49%), and less likely to be commercially insured (19% vs 30%). These patients were slightly younger (mean 31 months vs 36 months), but had similar acuity, triage vital signs, and Yale Observation Score (when applicable). In cases in which a LB existed, mean test charges were significantly higher: $145 versus $104, and ED stays were significantly longer: 165 minutes versus 137 minutes. In an analysis of covariance model including race/ethnicity, insurance status, physician training level, attending physician, urgent care setting, triage category, age, and vital signs, the presence of a LB accounted for a $38 increase in charges for testing and a 20 minute longer ED stay.Conclusion.Despite controlling for multiple factors, the presence of a physician–family LB was associated with a higher rate of resource utilization for diagnostic studies and increased ED visit times. Additional study is recommended to explore the reasons for these differences and ways to provide care more efficiently to non-English-speaking patients. language barriers, resource utilization, test ordering.
Assessment of Neonatal Encephalopathy by Amplitude-integrated Electroencephalography
Authors: al Naqeeb Niran , Edwards A. David , Cowan Frances M. , Azzopardi Denis 
Published: August 2004

Objective.To define normal and abnormal patterns, test interobserver variability, and the prognostic accuracy of amplitude-integrated electroencephalography (aEEG) soon after the onset of neonatal encephalopathy.Methods.Consecutive cases of neonatal encephalopathy (n = 56; gestation median, 40; range, 35–42 weeks) and healthy infants (n = 14; gestation median, 40; range, 39–40 weeks) were studied. aEEG was recorded using a cerebral function monitor, at median, 0, range, 0–21 days of age. Of the infants, 24 of the 56 with encephalopathy and all of the normal infants were studied within 12 hours of birth (median, 5; range, 3–12 hours). Forty infants were suspected of having suffered birth asphyxia. Criteria for normal and abnormal patterns were defined and the interobserver variability of these classifications determined. Results were compared with neurodevelopmental outcome assessed at 18 to 24 months of age. aEEG also was compared with a standard EEG and with magnetic resonance imaging.Results.The median upper margin of the widest band of aEEG activity in the control infants was 37.5 μV (range, 30–48 μV), and median lower margin was 8 μV (range, 6.5–11 μV). We classified the aEEG background activity as normal amplitude, the upper margin of band of aEEG activity &gt;10 μV and the lower margin &gt;5 μV; moderately abnormal amplitude, the upper margin of band of aEEG activity &gt;10 μV and the lower margin ≤5 μV; and suppressed amplitude, the upper margin of the band of aEEG activity &lt;10 μV and lower margin &lt;5 μV. Recordings were analyzed further for the presence of seizures, defined as periods of sudden increase in voltage accompanied by a narrowing of the band of aEEG activity.Tests of interobserver variability showed excellent agreement both for assessment of amplitude (κ statistic = 0.85) and for identification of seizures (κ statistic = 0.76)There was a close relationship between the aEEG and subsequent outcome: 19 of 21 infants with a normal aEEG finding were normal on follow-up at 18 to 24 months of age, whereas 27 of 35 infants with a moderately abnormal or suppressed aEEG and/or seizures died or developed neurologic abnormalities. Thus, aEEG predicted outcome with a sensitivity of 0.93, a specificity of 0.70, positive predictive value of 0.77, negative predictive value of 0.90, and the likelihood ratio of a positive result of 3.1 and a negative result of 0.06. For the 24 infants studied within 12 hours of birth, the corresponding results were sensitivity, 1.0; specificity, 0.82; positive predictive value, 0.85; negative predictive value, 1; likelihood ratio of a positive result, 5.5; and likelihood ratio of a negative result, 0.18.Conclusion.The aEEG is a simple but accurate and reproducible clinical tool that could be useful in the assessment of infants with encephalopathy.
Risk Factors for Early-onset Group B Streptococcal Sepsis: Estimation of Odds Ratios by Critical Literature Review
Authors: Benitz William E. , Gould Jeffrey B. , Druzin Maurice L. 
Published: August 2004

Objective.To identify and to establish the prevalence of ORs for factors associated with increased risk for early-onset group B streptococcal (EOGBS) infection in neonates.Study Design.Literature review and reanalysis of published data.Results.Risk factors for EOGBS infection include group B streptococcal (GBS)-positive vaginal culture at delivery (OR: 204), GBS-positive rectovaginal culture at 28 (OR: 9.64) or 36 weeks gestation (OR: 26.7), vaginal Strep B OIA test positive at delivery (OR: 15.4), birth weight ≤ 2500 g (OR: 7.37), gestation &lt;37 weeks (OR: 4.83), gestation &lt;28 weeks (OR: 21.7), prolonged rupture of membranes (PROM) &gt;18 hours (OR: 7.28), intrapartum fever &gt;37.5°C (OR: 4.05), intrapartum fever, PROM, or prematurity (OR: 9.74), intrapartum fever or PROM at term (OR: 11.5), chorioamnionitis (OR: 6.43). Chorioamnionitis is reported in most (88%) cases in which neonatal infection occurred despite intrapartum maternal antibiotic therapy. ORs could not be estimated for maternal GBS bacteriuria during pregnancy, with preterm premature rupture of membranes, or with a sibling or twin with invasive GBS disease, but these findings seem to be associated with a very high risk. Multiple gestation is not an independent risk factor for GBS infection.Conclusions.Mothers with GBS bacteriuria during pregnancy, with another child with GBS disease, or with chorioamnionitis should receive empirical intrapartum antibiotic treatment. Their infants should have complete diagnostic evaluations and receive empirical treatment until infection is excluded by observation and negative cultures because of their particularly high risk for EOGBS infection. Either screening with cultures at 28 weeks gestation or identification of clinical risk factors, ie, PROM, intrapartum fever, or prematurity, may identify parturients whose infants include 65% of those with EOGBS infection. Intrapartum screening using the Strep B OIA rapid test identifies more at-risk infants (75%) than any other method. These risk identifiers may permit judicious selection of patients for prophylactic interventions.
Helicobacter pyloriInfection and Insulin Requirement Among Children With Type 1 Diabetes Mellitus
Authors: Begue Rodolfo E. , Mirza Ayesha , Compton Terry , Gomez Ricardo , Vargas Alfonso 
Published: August 2004

Objective.Helicobacter pylori induces gastric inflammation and the production of cytokines in infected individuals. Theoretically, this increased production of cytokines could be deleterious for the control of the glycemia of patients with diabetes. This study aimed to describe the insulin requirement among patients with type 1 diabetes and H pylori infection compared with uninfected counterparts.Methods.Cross-sectional design. Demographic information (age, gender, race, annual family income, and number of individuals per room in the household) and clinical information (age at diagnosis of diabetes, duration of illness, weight, height, compliance with clinical appointments, daily insulin units per kilogram of body weight [IU/kg/d], and glycosylated hemoglobin A level) was obtained from children and adolescents with diagnosis of type 1 diabetes mellitus who were seen at Children's Hospital in New Orleans. A total of 2 mL of blood was also collected and sera were tested forH pylori-specific immunoglobulin G antibodies using an enzyme immunoassay. The daily insulin requirement among infected and uninfected children was compared, and the effect of other variables was evaluated with multiple linear regression.Results.Of the 71 subjects who were evaluated (median age: 11 years), 11 (15.5%) were found to be infected. H pylori infection was more frequent among subjects who were older, who had a lower family income, and who were black. Infected children were found to require more insulin (1.2 vs 0.9 IU/Kg/d) and their glycosylated hemoglobin A level was higher (14.9 vs 11.8) than the level found in uninfected subjects. Multiple linear regression analysis identified H pylori infection duration of illness, race (black), body mass index, and gender (female), to be associated independently with increased daily insulin requirement (IU/kg/d).Conclusion.In our study population, children with type 1 diabetes and H pylori infection had an increased daily insulin requirement compared with the requirement of their uninfected peers. The reason for this association requires additional investigation.
Flexible Flat Feet in Children: A Real Problem?
Authors: Garcı́a-Rodrı́guez Antonio , Martı́n-Jiménez Felipe , Carnero-Varo Manuel , Gómez-Gracia Enrique , Gómez-Aracena Jorge , Fernández-Crehuet Joaquı́n 
Published: August 2004

Objectives
To estimate the prevalence of flexible flat feet in the provincial population of 4- to 13-year-old schoolchildren and the incidence of treatments considered unnecessary.

Setting.
Province of Málaga, Spain.

Methods.
We examined and graded by severity a sample of 1181 pupils taken from a total population of 198 858 primary schoolchildren (CI: 95%; margin of error: 5%). The sample group was separated into three 2-year age groups: 4 and 5 years, 8 and 9 years, and 12 and 13 years. The plantar footprint was classified according to Denis1 into three grades of flat feet: grade 1 in which support of the lateral edge of the foot is half that of the metatarsal support; grade 2 in which the support of the central zone and forefoot are equal; and grade 3 in which the support in the central zone of the foot is greater than the width of the metatarsal support.
The statistical analysis for the evaluation of the differences between the groups was performed with Student's t and χ2 tests as appropriate.

Results.
The prevalence of flat feet was 2.7%. Of the 1181 children sampled, 168 children (14.2%) were receiving orthopedic treatment, but only 2.7% had diagnostic criteria of flat feet. When we inspected the sample, we found that a number of children were being treated for flat feet with boots and arch supports. Most of them did not have a flat plantar footprint according to the criteria that we used for this work. Furthermore, in the group of children that we diagnosed as having flat feet, only 28.1% were being treated. We found no significant differences between the number of children receiving orthopedic treatments and the presence or absence of a flat plantar footprint. Children who were overweight in the 4- and 5-year-old group showed an increased prevalence for flat feet as diagnosed by us.

Conclusion.
The data suggest that an excessive number of orthopedic treatments had been prescribed in the province. When extrapolated to the 1997 population of schoolchildren within the age groups studied the figures suggested that ∼28 167 children in Málaga province probably would have been receiving orthopedic treatments with boots and insoles at the time of our study. Consequently, the total expenditure on orthopedic boots and insoles that year could be estimated as 676 008 000 pesetas ($4 447 422 in US currency).