Citations

Objective.To determine why people do or do not wear helmets while bicycling.Methods.A survey was conducted from August through October 1999. Two survey areas were chosen for this study: local public schools and paved bicycle trails. For the school arm of the study, 3 public elementary, middle, and high schools were selected from 3 different regions of Rochester, Minnesota, for participation in the study. For the bicycle arm of the study, 3 paved trails located in southeastern Minnesota were selected. A total of 2970 surveys were distributed to the public school system, and 463 surveys were collected from bicyclists on the paved bicycle trails. The survey population was split into 3 age categories for analysis: child (7–10), adolescent (11–19), and adult (older than 19).Results.Of the 2970 surveys distributed to Rochester public schools, 2039 (69%) were returned for analysis. Seventy-eight of the surveys that were completed in the public school system were discarded for the following reasons: age <10 years (35), insufficient completion (24), and selection of every reason for not wearing a bicycle helmet (19). A total of 463 surveys were completed on the 3 paved bicycle trails. One survey from the paved bicycle trail arm of the study was discarded because of insufficient completion. The total number of surveys used for statistical analysis was 2424. The distribution of male (52.7%) and female (47.3%) participants was similar. No significant difference in bicycle helmet use was found between genders.The age groups with the highest rate of bicycle helmet use were 50 to 59 years (62%) and older than 59 years (70%). The age groups with the lowest rate of bicycle helmet use were 11 to 19 years (31%) and 30 to 39 years (30%). The most common reasons given for not wearing a bicycle helmet were “uncomfortable,” “annoying,” “it's hot,” “don't need it,” and “don't own one.” Bicycle helmet use was significantly influenced by peer helmet use in all 3 age groups. Children also were more likely to wear a bicycle helmet when their parents wore bicycle helmets.A majority of respondents in all 3 age groups indicated that bicycle helmets provided either “moderate” or “great” protection from head injury, although significantly more adults (65.9%) than adolescents (43.9%) believed that the protection afforded by bicycle helmets was “great.” Despite this belief, a majority of adolescents and adults indicated that there was only a “slight risk” of head injury when bicycling without a helmet. Participants in all 3 categories were more likely to wear a bicycle helmet when they indicated either that there was a “great risk” of head injury when bicycling without a helmet or that helmets provided “great protection” from head injury. Adolescents and adults who believed that bicycling without a helmet put one at “great risk” for head injury also were more likely to indicate that helmets provided “great protection” from head injury.Conclusions.The prevalence of bicycle helmet use remains low despite research indicating the high level of head injury risk when bicycling without a helmet and the significant protection afforded by bicycle helmets. With the information provided by this survey, a well-designed intervention to increase the use of bicycle helmets can be implemented. Suggestions for a campaign to promote an increase in bicycle helmet use include focusing efforts on males and females between 11 and 19 years and 30 and 39 years of age; educating the public on new bicycle helmet designs that address comfort, ventilation, and fashion; educating adolescents on the significant protection from head injury afforded by bicycle helmets; and educating the public on the risk and severity of head injury associated with bicycling without a helmet. The influence of parents and peers on bicycle helmet use may be targeted through education and statements such as, “If you wear a bicycle helmet, you are not only protecting yourself, you are also helping to protect your friends and/or children.” bicycle, helmet, injury, accident, prevention.

Very long chain fatty acid dehydrogenase (VLCAD) deficiency is a rare but treatable cause of cardiomyopathy, fatty liver, skeletal myopathy, pericardial effusions, ventricular arrhythmias, and sudden death. Unrecognized, VLCAD deficiency may be rapidly progressive and fatal, secondary to its cardiac involvement. Because early diagnosis improves outcome, we present a neonate with VLCAD deficiency in whom retrospective analysis of the newborn screening card revealed that a correct diagnosis could have been made by newborn screening using tandem mass spectrometry. Our patient demonstrated a classic neonatal course with transient hypoglycemia at birth, interpreted as culture-negative sepsis, followed by a quiescent period notable only for hypotonia and poor feeding. At 3 months, he presented with cardiorespiratory failure and pericardial effusions, requiring pericardiocentesis, tracheostomy, and prolonged mechanical ventilation. Plasma free-fatty acid and acylcarnitine profiles demonstrated small but significant elevations of C14:2, C14:1, C16, and C18:1 acylcarnitine species, findings consistent with a biochemical diagnosis of VLCAD deficiency. Enteral feeds were changed to Portagen formula with marked improvement in cardiac symptoms over several weeks. To confirm the biochemical diagnosis, molecular analysis was performed by analysis of genomic DNA on a blood sample of the patient. Sequencing analysis and delineation of VLCAD mutations were performed using polymerase chain reaction and genomic sequencing. The patient was heterozygous for 2 different disease-causing mutations at the VLCAD locus. The maternal mutation was a deletion of bp 842–3 in exon 8, causing a shift in the reading frame. The paternal mutation was G+1A in the consensus donor splice site after exon 1; this splice-site mutation would likely result in decreased mRNA. The likely consequence of these mutations is essentially a null phenotype. To determine whether this case could have been picked up by tandem mass spectrometry analysis at birth when the patient was asymptomatic, acylcarnitine analysis was performed on the patient's original newborn card (after obtaining parental consent, the original specimen was provided courtesy of Dr Kenneth Pass, Director, New York State Newborn Screening Program). The blood sample had been obtained at 1 week of age and stored at room temperature for 6 months and at 70°C thereafter for 18 months. Electrospray tandem mass spectrometry used a LC-MS/MS API 2000 operated in ion evaporation mode with the TurboIonSpray ionization probe source. The acylcarnitine profile obtained from the patient's original newborn card was analyzed 2 years after it was obtained. In comparison with a normal control, there was a significant accumulation of long chain acylcarnitine species, with a prominent peak of tetradecenoylcarnitine (C14:1), the most characteristic metabolic marker of VLCAD deficiency. This profile would have likely been even more significant if it had been analyzed at the time of collection, yet 2 years later is sufficient to provide strong biochemical evidence of the underlying disorder. Discussion. VLCAD was first discovered in 1992, and clinical experience with VLCAD deficiency has been accumulating rapidly. Indeed, the patients originally diagnosed with long chain acyl-CoA deficiency suffer instead from VLCAD deficiency. The phenotype of VLCAD deficiency is heterogeneous, ranging from catastrophic metabolic and cardiac failure in infancy to mild hypoketotic, hypoglycemia, and exertional rhabdomyolysis in adults. This case demonstrates that VLCAD deficiency could have been detected from the patient's own neonatal heel-stick sample. Most likely, a presymptomatic diagnosis would have avoided at least part of a lengthy and intensive prediagnosis hospitalization that had an estimated cost of $400 000. Although VLCAD is relatively rare, timely and correct diagnosis leads to dramatic recovery, so that detection by newborn screening could prevent the onset of arrhythmias, heart failure, metabolic insufficiency, and death. Fatty acid oxidation defects, including VLCAD deficiency, may account for as many as 5% of sudden infant death patients. Recent instrumentation advances have made automated tandem mass spectrometry of routine neonatal heel-stick samples technically feasible. Pilot studies have demonstrated an incidence of fatty acid oxidation defects, including short chain, medium chain, and very long chain acyl-CoA dehydrogenase deficiencies, of approximately 1/12 000. As a result, cost-benefit ratios for this approach should be systematically examined.

Objective. Inherited long QT syndrome (LQTS) may present with syncope, seizures, and/or sudden death as a result of ventricular tachyarrhythmias. Identification of family members who are at risk because they harbor the genetic substrate for LQTS is critical. Presently, such identification relies on the 12-lead electrocardiogram (ECG). The purpose of this study was to evaluate the efficacy of the automated ECG as a screening tool for LQTS. Method. Molecular testing of a proband and 22 additional family members for the KVLQT1 mutation and symptomatic status facilitated the classification of each family member into the following patient groups: noncarriers (13), asymptomatic carriers (5), and symptomatic carriers (5). Each individual had a standard 12-lead ECG from which the computer and manual (lead II) corrected QT interval were determined. In addition, we determined the accuracy of the computer ECG diagnostic interpretation for each patient group. Results. With the use of a corrected QT interval of ≥460 ms as a diagnostic cutoff, the positive and negative predictive values for identifying at-risk individuals were 100%. Despite this, the computer-generated ECG diagnostic interpretation erroneously classified 6 of 23 family members. Moreover, half of the family members, proved to have the ion channel defect, received the diagnostic interpretation “normal ECG.” Conclusion. Reliance on the computer-generated ECG diagnostic interpretation alone will fail to identify many at-risk family members. It is suggested that all first-degree relatives of an identified LQTS proband have a 12-lead ECG that is reviewed independently by a physician who is familiar with LQTS in an effort to improve screening for this potentially lethal syndrome.electrocardiogram, long QT syndrome, QT interval, sudden death.

Objective.To gather clinicopathologic data on subcutaneous granuloma annulare (SGA), a subtype of granuloma annulare that occurs exclusively in children and is histologically similar to rheumatoid nodules.Design.Retrospective record review.Patients.Children <10 years old in whom SGA, deep granuloma annulare, or necrobiotic granuloma was diagnosed at the Mayo Clinic (Rochester, MN) from 1983 to 1998.Results.Thirty-four patients (21 girls and 13 boys; average age at diagnosis: 4.6 years) were found to have SGA. The lesions predominantly occurred in the lower extremity, especially in a pretibial location. Local recurrence within 1 month to 7 years was documented in 38.2%; recurrence at other locations was documented in 14.7%. Average follow-up was 60 months; during follow-up, no patients developed signs or symptoms of rheumatologic disease. Insulin-dependent diabetes mellitus was diagnosed in 2 patients, 1 before the development of SGA and 1 after it by 1 month.Conclusions.SGA is a lesion that presents as subcutaneous nodules on the lower extremities, hands, or scalp in young children. Recurrence is common but usually does not warrant additional biopsy.

Objective. To evaluate factors related to the development and persistence of adolescent sleep problems. Methods. In this longitudinal, population-based study, the Teenage Attitudes and Practices Survey was administered by telephone to 7960 adolescents (3921 girls and 4039 boys) 12 to 18 years old in 1989 and at follow-up in 1993. Sleep problems at both time points were assessed using a single item on the Teenage Attitudes and Practices Survey. Those who responded that they “often or sometimes” had trouble going to sleep or staying asleep during the past 12 months were categorized as reporting sleep problems, whereas those who responded “often” were categorized as having frequent sleep problems. Multiple logistic regression analyses were used to identify baseline characteristics predictive of the development and persistence of sleep problems or frequent sleep problems, respectively, from baseline to follow-up. Results. Of the 4866 adolescents without sleep problems at baseline, 28% developed sleep problems by 1993, and 9% developed frequent sleep problems. Of the 3094 adolescents who reported sleep problems at baseline, 52% reported sleep problems in 1993, and 21% reported frequent sleep problems. Female sex and notable depressive symptoms were associated with the development and persistence of sleep problems and frequent sleep problems at follow-up. Cigarette smoking status showed a dose–response relationship with development of sleep problems and frequent sleep problems, and with persistence of frequent sleep problems at follow-up. Conclusion. The reduction of depressive symptoms and cigarette smoking among adolescents are important factors to consider in prevention and treatment efforts focused on adolescent sleep problems.

Objective. The development of this review article evolved from a National Kidney Foundation consensus conference on recent advances in the importance of evaluating and treating proteinuria. From this conference, a series of recommendations for the evaluation of adults with proteinuria was published. Because specific pediatric aspects of the problem were outside the scope of the original National Kidney Foundation publication, an ad hoc committee of 6 pediatric nephrologists who were active participants in the National Kidney Foundation conference was established to provide primary care physicians with a concise, up-to-date reference on this subject. Methods. The recommendations that are given represent the consensus opinions of the authors. These are based on data from controlled studies in children when available, but many of the opinions are, by necessity, based on uncontrolled series in children or controlled trials performed in adults, because controlled trials in children have not been performed to evaluate many of the treatments described. Results and Conclusions. These recommendations are intended to provide primary care physicians with a useful reference when they are faced with a young child or teenager who presents with proteinuria, whether this is mild and asymptomatic or more severe, leading to nephrotic syndrome.

Objectives. To describe normative sexual behavior in Dutch-speaking children; to assess the frequencies of different types of sexual behaviors reported in children by their parents; to analyze the relation of these sexual behaviors to demographic, personal, familial, and general behavioral variables; and to compare the Dutch-speaking sample with American samples. Method. Nine hundred seventeen children (2–12 years of age), screened to exclude sexual abuse, were rated by their caregiver via parent report consisting of the translated Dutch version of the Child Sexual Behavior Inventory, the Child Behavior Checklist, a life event checklist, and a questionnaire assessing family nudity and parental attitudes regarding sexuality. Results. Frequencies of a wide variety of sexual behaviors for 2- to 5-, 6- to 9-, and 10- to 12-year-old children are presented. Sexual behavior was found to be related to the child's age, maternal education, family nudity, and parental attitudes toward sexuality. The positive relation to general behavioral problems was confirmed. Findings were primarily similar to previously published American studies. Conclusion. Our study confirms that sexual behavior in children is varied and related to developmental, personal, and familial factors. The relative frequency of the wide variety of sexual behaviors in a Dutch-speaking normative sample is comparable to American samples.

Objective. To determine parents' knowledge and attitudes of medicolegal issues affecting adolescent medical care. Design. An anonymous, mailed survey with 16 questions concerning Minnesota consent and confidentiality laws that affect adolescents' medical care. Setting. A community of >70 000 in rural, southeastern Minnesota. Participants. Parents of 600 randomly selected 7th- through 12th-grade public school students. Results. Two hundred eighty-eight (48%) parents returned the surveys. Parents achieved a median score of 18.8% (range, 0%–93.8%) correct on a test of knowledge. Opinion was a median of +0.3 on a scale where −1 signified “a bad law,” 0 signified “neither a good nor bad law,” and +1 signified “a good law.” Four questions, however, generated a more intense response. Seventy-seven percent of parents reported that, as a whole, the laws in the survey had no effect on them and/or their children. Conclusions. These results suggest that parents are not knowledgeable of Minnesota laws that affect adolescent medical care. Overall opinion of these laws was mildly positive, with notable exceptions. Parents also lack a sense of impact of laws affecting adolescent medical care. Lack of knowledge and the presence of certain attitudes allow for identification of issues that clinicians should address by incorporating medicolegal education into the care of adolescents and their families. These results are especially timely in light of the Parental Rights and Responsibilities Act under consideration in Congress, which would provide parents greater authority over their children's medical care.

Objective. Sexual behavior in children can cause uncertainty in the clinician because of the relationship between sexual abuse and sexual behavior. Consequently, it is important to understand normative childhood sexual behavior. Design. Sexual behavior in 1114 2- to 12-year-old children was rated by primary female caregivers. These children were screened for the absence of sexual abuse. A 38-item scale assessing a broad range of sexual behavior (Child Sexual Behavior Inventory, Third Version) was administered along with the Child Behavior Checklist and a questionnaire assessing family stress, family sexuality, social maturity of the child, maternal attitudes regarding child sexuality, and hours in day care. Results. Sexual behavior was related to the child's age, maternal education, family sexuality, family stress, family violence, and hours/week in day care. Frequencies of sexual behaviors for 2- to 5-, 6- to 9-, and 10- to 12-year-old boys and girls are presented. Conclusions. A broad range of sexual behaviors are exhibited by children who there is no reason to believe have been sexually abused. Their relative frequency is similar to two earlier studies, and this reinforces the validity of these results.