OBJECTIVE

To determine if a single application of a vapor rub (VR) or petrolatum is superior to no treatment for nocturnal cough, congestion, and sleep difficulty caused by upper respiratory tract infection.

METHODS

Surveys were administered to parents on 2 consecutive days—on the day of presentation when no medication had been given the previous evening, and the next day when VR ointment, petrolatum ointment, or no treatment had been applied to their child’s chest and neck before bedtime according to a partially double-blinded randomization scheme.

RESULTS

There were 138 children aged 2 to 11 years who completed the trial. Within each study group, symptoms were improved on the second night. Between treatment groups, significant differences in improvement were detected for outcomes related to cough, congestion, and sleep difficulty; VR consistently scored the best, and no treatment scored the worst. Pairwise comparisons demonstrated the superiority of VR over no treatment for all outcomes except rhinorrhea and over petrolatum for cough severity, child and parent sleep difficulty, and combined symptom score. Petrolatum was not significantly better than no treatment for any outcome. Irritant adverse effects were more common among VR-treated participants.

CONCLUSIONS

In a comparison of VR, petrolatum, and no treatment, parents rated VR most favorably for symptomatic relief of their child’s nocturnal cough, congestion, and sleep difficulty caused by upper respiratory tract infection. Despite mild irritant adverse effects, VR provided symptomatic relief for children and allowed them and their parents to have a more restful night than those in the other study groups.

OBJECTIVES

More than $55 million is spent on hospital-based child protection teams (CPTs) annually, but there is no consensus on what makes CPTs effective. The objective of this study was to create expert consensus on tasks that CPTs should perform and factors that contribute to effectiveness.

METHODS

A modified Delphi approach was used to create expert consensus among professionals with experience working on or with hospital-based CPTs. Three initial rounds of surveys were conducted; a first round of open-ended questions generated topics related to CPT tasks and factors related to team effectiveness. A Likert scale (range: 1–7) determined rank. In the fourth round, participants ranked the top 5 variables associated with effectiveness.

RESULTS

Twenty-six (90%) of 29 participants completed the first 3 rounds, and 20 (67%) completed the final ranking. Experts believed that CPTs should provide communication of findings to appropriate agencies (mean Likert score: 7.0), court testimony (7.0), medical consultations (6.9), multidisciplinary case review (6.6), and forensic interviews (6.0). CPT success should be determined by professionals who use CPT services (6.6) and CPT members (6.5). Variables that were ranked most often as critical to effectiveness included interdisciplinary collaboration (95% of participants), provision of resources (80%), and team collegiality (75%). Variables that were ranked as most detrimental included inadequate staffing (85%) and lack of collegiality (80%).

CONCLUSIONS

A multidisciplinary team working in a collegial atmosphere seems to be the major key to CPT effectiveness. In addition to providing services, CPTs should focus on improving collegiality and interdisciplinary collaboration and should seek performance feedback from referring professionals and CPT members.

OBJECTIVE

Relative adrenal insufficiency in extremely low birth weight infants may contribute to significant morbidity and death. Our objective was to evaluate the relationship between cortisol concentrations and short-term outcomes.

METHODS

Cortisol concentrations were obtained for 350 intubated, extremely low birth weight infants at postnatal age of 12 to 48 hours and at day 5 to 7, as part of a multicenter, randomized trial of hydrocortisone treatment for prophylaxis of relative adrenal insufficiency. Death and short-term morbidity were monitored prospectively. Cortisol levels at each time point were divided into quartiles. The incidence rates of outcomes were determined for each quartile and for infants with cortisol values of <10th percentile or >90th percentile.

RESULTS

Median cortisol values were 16.0 μg/dL at baseline and 13.1 μg/dL on day 5 to 7 in the placebo group. Outcomes did not differ in each quartile between treatment and placebo groups. Low cortisol values at baseline or day 5 to 7 were not associated with increased morbidity or mortality rates and were not predictive of open-label hydrocortisone use. In fact, vasopressor use was lower for infants with lower cortisol values at baseline. Severe intraventricular hemorrhage was more frequent in infants with cortisol levels in the upper quartile at baseline, and values of >90th percentile were significantly associated with higher rates of death, severe intraventricular hemorrhage, periventricular leukomalacia, gastrointestinal perforation, and severe retinopathy of prematurity.

CONCLUSIONS

Low cortisol concentrations were not predictive of adverse short-term outcomes, but high cortisol concentrations were associated with severe intraventricular hemorrhage, and extremely elevated values were associated with morbidity and death. Low cortisol concentrations alone at these 2 time points did not identify the infants at highest risk for adverse outcomes. In contrast, high cortisol values were associated with increased morbidity and mortality rates.

OBJECTIVE

We describe the first cohort-based analysis of the impact of genetic disorders in craniosynostosis. We aimed to refine understanding of prognosis and pathogenesis, and to provide rational criteria for clinical genetic testing.

METHODS

We undertook targeted molecular genetic and cytogenetic testing of 326 children requiring surgery for craniosynostosis, born in the years 1993-2002, presenting to a single craniofacial unit, and followed up until the end of 2007.

RESULTS

Eighty-four children (and 64 relatives) had a pathological genetic alteration, comprised of 86% single gene mutations and 14% chromosome abnormalities. The FGFR3 P250R mutation was the single largest contributor to the genetic cases (24%). Genetic diagnoses accounted for 21% of all craniosynostosis and were associated with increased rates of many complications. Children with an initial clinical diagnosis of non-syndromic craniosynostosis were more likely to have a causative mutation if the synostosis was unicoronal or bicoronal (10 of 48 cases) than if it was sagittal or metopic (none of 55; P=0.0003). Repeat craniofacial surgery was required in 58% of children with single gene mutations but only 17% of those with chromosome abnormalities (P=0.01).

CONCLUSIONS

Clinical genetic assessment is critical for the management of children with craniosynostosis. Genetic testing of non-syndromic patients (as a minimum, for FGFR3 P250R and FGFR2 exons IIIa/c), should be targeted to those with coronal or multisuture synostosis. Single gene disorders that disrupt physiological signalling in the cranial sutures often require re-operation, whereas chromosome abnormalities follow a more indolent course suggesting a different, secondary origin of the associated craniosynostosis.

OBJECTIVE

We tested the impact of the Fast Track conduct disorder prevention program on the use of pediatric, general health, and mental health services in adolescence.

PATIENTS AND METHODS

Participants were 891 public kindergarten boys and girls screened from a population of 9594 children and found to be at risk for conduct disorder. They were assigned randomly (by school) to intervention or control conditions and were followed for 12 years. Intervention lasted 10 years and included parent training, child social-cognitive skills training, reading tutoring, peer-relations enhancement, and classroom curricula and management. Service use was assessed through annual interviews of parents and youth.

RESULTS

Youth assigned to preventive intervention had significantly reduced use of professional general health, pediatric, and emergency department services relative to control youth on the basis of parent-report data. For control-group youth, the odds of greater use of general health services for any reason and general health services use for mental health purposes were roughly 30% higher and 56% higher, respectively. On the basis of self-report data, the intervention reduced the likelihood of outpatient mental health services among older adolescents for whom odds of services use were more than 90% higher among control-group youth. No differences were found between intervention and control youth on the use of inpatient mental health services. Statistical models controlled for key study characteristics, and potential moderation of the intervention effect was assessed.

CONCLUSIONS

Random assignment to the Fast Track prevention program is associated with reduced use of general health and outpatient mental health services in adolescents. Future studies should examine the mechanism of this impact and service use patterns as subjects reach young adulthood.

OBJECTIVE

We sought to establish the frequency of receiving >1 dose of epinephrine in children who present to the emergency department (ED) with food-related anaphylaxis.

PATIENTS AND METHODS

We performed a medical chart review at Boston hospitals of all children presenting to the ED for food-related acute allergic reactions between January 1, 2001, and December 31, 2006. We focused on causative foods, clinical presentations, and emergency treatments.

RESULTS

Through random sampling and appropriate weighting, the 605 reviewed cases represented a study cohort of 1255 patients. These patients had a median age of 5.8 years (95% confidence interval [CI]: 5.3– 6.3), and the cohort was 62% male. A variety of foods provoked the allergic reactions, including peanuts (23%), tree nuts (18%), and milk (15%). Approximately half (52% [95% CI: 48–57]) of the children met diagnostic criteria for food-related anaphylaxis. Among those with anaphylaxis, 31% received 1 dose and 3% received >1 dose of epinephrine before their arrival to the ED. In the ED, patients with anaphylaxis received antihistamines (59%), corticosteroids (57%), epinephrine (20%). Over the course of their reaction, 44% of patients with food-related anaphylaxis received epinephrine, and among this subset of patients, 12% (95% CI: 9–14) received >1 dose. Risk factors for repeat epinephrine use included older age and transfer from an outside hospital. Most patients (88%) were discharged from the hospital. On ED discharge, 43% were prescribed self-injectable epinephrine, and only 22% were referred to an allergist.

CONCLUSIONS

Among children with food-related anaphylaxis who received epinephrine, 12% received a second dose. Results of this study support the recommendation that children at risk for food-related anaphylaxis carry 2 doses of epinephrine.

OBJECTIVE

No consensus exists among general pediatricians or pediatric rheumatologists regarding whether corticosteroid therapy ameliorates the acute manifestations of Henoch-Schönlein purpura or mitigates renal injury. Therefore, we sought to synthesize the reported experimental and observational data regarding corticosteroid use.

METHODS

We performed a meta-analysis based on a comprehensive review of the literature in the Medline database (1956 to January 2007) and the Cochrane Controlled Trials Register. On the basis of reported outcomes among patients with Henoch-Schönlein purpura who were treated at diagnosis with corticosteroids compared with patients treated with supportive care only, we calculated odds ratios for the resolution of abdominal pain, the need for surgical intervention secondary to severe pain or intussusception, the likelihood of Henoch-Schönlein purpura recurrence, and the development of transient or persistent renal disease.

RESULTS

Of 201 articles retrieved from the initial literature search, 15 were eligible for inclusion. Corticosteroid treatment did not reduce the median time to resolution of abdominal pain but did significantly reduce the mean resolution time and increased the odds of resolution within 24 hours. Early corticosteroid treatment significantly reduced the odds of developing persistent renal disease. In addition, although the results were not statistically significant, the prospective data suggest reduced odds of both surgical intervention and recurrence.

CONCLUSIONS

Corticosteroids, given early in the course of illness, seem to produce consistent benefits for several major clinically relevant Henoch-Schönlein purpura outcomes.

OBJECTIVE

To characterize the effect of corticosteroid exposure on clinical outcomes in children hospitalized with new-onset Henoch-Schönlein purpura (HSP).

PATIENTS AND METHODS

We conducted a retrospective cohort study of children discharged with an International Classification of Diseases, Clinical Modification code of HSP between 2000 and 2007 by using inpatient administrative data from 36 tertiary care children’s hospitals. We used stratified Cox proportional hazards regression models to estimate the relative effect of time-varying corticosteroid exposure on the risks of clinical outcomes that occur during hospitalization for acute HSP.

RESULTS

During the 8-year study period, there were 1895 hospitalizations for new-onset HSP. After multivariable regression modeling adjustment, early corticosteroid exposure significantly reduced the hazard ratios for abdominal surgery (0.39 [95% confidence interval (CI): 0.17– 0.91]), endoscopy (0.27 [95% CI: 0.13– 0.55]), and abdominal imaging (0.50 [95% CI: 0.29 – 0.88]) during hospitalization.

CONCLUSIONS

In the hospital setting, early corticosteroid exposure was associated with benefits for several clinically relevant HSP outcomes, specifically those related to the gastrointestinal manifestations of the disease.

BACKGROUND

Prevalence estimates of illicit drug use by teens are typically generated from confidential or anonymous self-report. While data comparing teen self-report with biological measures are limited, adult studies identify varying degrees of under-reporting.

METHODS

Hair analyses for cocaine, opiates and marijuana were compared to confidential teen self- and parent-reported teen drug use in a longitudinal cohort of >400 high-risk urban teens and parents.

RESULTS

Both teens and parents substantially underreported recent teen cocaine and opiate use. However, compared with parents, teens were more likely to deny biomarker-verified cocaine use. Teen specimens (hair) were 52 times more likely to identify cocaine use compared with self-report. Parent hair analyses for cocaine and opiate use were 6.5 times and 5.5 times, respectively, more likely to indicate drug use than were parental self-report. The lack of concordance between self-report and bioassay occurred despite participant’s knowledge that a “certificate of confidentiality” protected both teen and adult participants, and that the biological specimens would be tested for drugs.

CONCLUSIONS

These findings confirm prior reports of adult under-reporting of their own drug use while extending our understanding of teen’s self-admitted drug use. The lack of concordance between teen self- or parent-reported teen drug use and biomarkers confirm our concerns that both teen- and parent-reported teen drug use is limited, at least for youth in high-risk urban settings. Methods of ascertainment other than self- or parent-report must be considered when health care providers, researchers and public health agencies attempt to estimate teen drug-use prevalence.

BACKGROUND

Fibrodysplasia ossificans progressiva is a rare and disabling genetic condition characterized by congenital malformation of the great toes and by progressive heterotopic ossification in specific anatomic patterns. Most patients with fibrodys-plasia ossificans progressiva are misdiagnosed early in life before the appearance of heterotopic ossification and undergo diagnostic procedures that can cause lifelong disability. Recently, the genetic cause of fibrodysplasia ossificans progressiva was identified, and definitive genetic testing for fibrodysplasia ossificans progressiva is now available before the appearance of heterotopic ossification.

METHODS

We recently evaluated 7 children for diagnosis of fibrodysplasia ossificans progressiva before the onset of heterotopic ossification. A medical history, physical examination, and skeletal survey were obtained on all of the patients, as well as clinical genetic testing for the canonical fibrodysplasia ossificans progressiva mutation.

RESULTS

All 7 of the children (4 girls and 3 boys; ages 3 months to 6 years) had congenital malformations of the great toes, but none had radiographic evidence of heterotopic ossification at the time of evaluation. Five of the 7 children had soft tissue lesions of the neck and back, suggestive of early fibrodysplasia ossificans progressiva flare-ups, 3 of whom had undergone invasive diagnostic procedures that exacerbated their condition. Two children had no history or signs of soft tissue swelling or flare-ups. DNA sequence analysis found that all 7 of the children had the recurrent fibrodysplasia ossificans progressiva missense mutation, a single nucleotide substitution (c.617G>A) at codon 206 in the glycine-serine activation domain of activin receptor IA, a bone morphogenetic protein type 1 receptor.

CONCLUSION

Clinical suspicion of fibrodysplasia ossificans progressiva early in life on the basis of malformed great toes can lead to early clinical diagnosis, confirmatory diagnostic genetic testing, and the avoidance of additional harmful diagnostic and treatment procedures. This is the first report of genetic confirmation of fibrodysplasia ossificans progressiva before the appearance of heterotopic ossification. Pediatricians should be aware of the early diagnostic features of fibrodysplasia ossificans progressiva, even before the appearance of heterotopic ossification. This awareness should prompt early genetic consultation and testing and the institution of assiduous precautions to prevent iatrogenic harm.

OBJECTIVES

Recent evidence suggests higher prevalence of autism spectrum disorder (ASD) in NICU graduates. This aim of this study was to identify retrospectively early behaviors found more frequently in NICU infants who went on to develop ASD.

METHODS

Twenty-eight NICU graduates who later received a diagnosis of ASD were compared with 2169 other NICU graduates recruited from 1994 to 2005. They differed in gender, gestational age, and birth cohort. These characteristics were used to draw a matched control sample (n = 112) to determine which, if any, early behaviors discriminated subsequent ASD diagnosis. Behavioral testing at targeted ages (adjusted for gestation) included the Rapid Neonatal Neurobehavioral Assessment (hospital discharge, 1 month), Arousal-Modulated Attention (hospital discharge, 1 and 4 months), and Bayley Scales of Infant Development (multiple times, 4–25 months).

RESULTS

At 1 month, children with ASD but not control children had persistent neurobehavioral abnormalities and higher incidences of asymmetric visual tracking and arm tone deficits. At 4 months, children with ASD had continued visual preference for higher amounts of stimulation than did control children, behaving more like newborns. Unlike control children, children with ASD had declining mental and motor performance by 7 to 10 months, resembling infants with severe central nervous system involvement.

CONCLUSIONS

Differences in specific behavior domains between NICU graduates who later receive a diagnosis of ASD and matched NICU control children may be identified in early infancy. Studies with this cohort may provide insights to help understand and detect early disabilities, including ASD.

While asthma has emerged as a major contributor to disease and disability in American children, the burden of this disease is unevenly distributed within the population. This paper provides a brief overview of social status variables that predict variation in asthma risks and social exposures such as stress and violence that are emerging as important risk factors. However, the central focus of the paper is on the distal social variables that have given rise to unhealthy residential environments in which the risk factors for asthma and other diseases are clustered. Effective initiatives for the prevention and treatment of childhood asthma need to address these non-medical determinants of the prevalence of asthma.

Objective

We examined the prognostic significance of elevated albuminuria in youth with type 2 diabetes.

Patients and Methods

Cross-sectional and prospective studies were conducted in Pima Indian youth aged 5-19 years at baseline who were examined between July 1, 1982 and December 31, 2007. Prevalence and sequential changes in the level of microalbuminuria (30≤ albumin-to-creatinine ratio <300 mg/g) and macroalbuminuria (albumin-to-creatinine ratio ≥300 mg/g) and incidence of macroalbuminuria were computed according to the presence or absence of type 2 diabetes.

Results

The prevalence of micro- and macroalbuminuria was 6.5% and 0.6% in the 3,856 nondiabetic youth and 18.5% and 2.9% in the 103 youth with diabetes. One-hundred-forty-one (75.4%) of 187 nondiabetic youth, but only one (7.1%) of 14 diabetic youth with elevated albumin-to-creatinine ratio (≥30 mg/g) regressed to undetectable or normal albumin-to-creatinine ratio (<30 mg/g) on subsequent examination. In a subset of 2,666 youth with a median follow-up of 8.1 years, 36 nondiabetic and 30 diabetic youth with baseline albumin-to-creatinine ratio <300 mg/g developed macroalbuminuria. For a given albumin-to-creatinine ratio level, the incidence of macroalbuminuria was 15.9-fold (95% CI = 11.1 to 22.6) higher in the diabetic than in the nondiabetic youth.

Conclusions

Elevated albuminuria is infrequent and largely transient in nondiabetic youth, but is relatively frequent and largely persistent in those with diabetes. Microalbuminuria in youth with type 2 diabetes strongly predicts progression to macroalbuminuria, supporting annual screening for albuminuria.

Objective

To determine whether elementary school-aged children with unilateral hearing loss (UHL) demonstrate significantly worse language skills than their sibling controls with normal hearing, and whether they are more likely to receive extra assistance or resources at school.

Patients and Methods

Case-control study of age 6-12 year old children with UHL compared with sibling controls (74 matched pairs, total n=148), all with normal cognition by parental report. Scores on the oral portion of the Oral and Written Language Scales (OWLS) were the primary outcome measure. Potential confounders were evaluated for their effect on the OWLS scores. Multivariable analysis was used to determine whether UHL independently predicted OWLS scores.

Results

Children with UHL had significantly worse language comprehension (91 vs. 98, P = 0.003), oral expression (94 vs. 101, P = 0.007), and oral composite (90 vs. 99, P <0.001) scores than their siblings with normal hearing. Multivariable regression models demonstrated that UHL was an independent predictor of these OWLS scores, with moderate effect sizes of 0.3 to 0.7. Family income and maternal education level were also independent predictors of oral expression and oral composite scores. No differences were found between children with right or left UHL, nor with varying severity of hearing loss. Children with UHL were more likely to have an Individualized Education Plan (OR 4.4, 95% CI 2.0-9.5) and to have received speech-language therapy (OR 2.6, 95% CI 1.3-5.4).

Conclusions

School-aged children with UHL demonstrated worse oral language scores compared with siblings with normal hearing. These findings suggest that the common practice of withholding hearing-related accommodations from children with UHL should be reconsidered and studied, and that parents, pediatricians, and educators be informed about the deleterious effects of UHL on oral language skills.

OBJECTIVE

The purpose of this multicenter study was to confirm the validity and reliability of the Pediatric Cardiac Quality of Life Inventory (PCQLI).

METHODS

Seven centers recruited pediatric patients (8–18 years of age) with heart disease (HD) and their parents to complete the PCQLI and generic health-related quality of life (Pediatric Quality of Life Inventory [PedsQL]) and non–quality of life (Self-Perception Profile for Children [SPPC]/Self-Perception Profile for Adolescents [SPPA] and Youth Self-Report [YSR]/Child Behavior Checklist [CBCL]) tools. PCQLI construct validity was assessed through correlations of PCQLI scores between patients and parents and with severity of congenital HD, medical care utilization, and PedsQL, SPPC/SPPA, and YSR/CBCL scores. PCQLI test-retest reliability was evaluated.

RESULTS

The study enrolled 1605 patient-parent pairs. Construct validity was substantiated by the association of lower PCQLI scores with Fontan palliation and increased numbers of cardiac operations, hospital admissions, and physician visits (P < .001); moderate to good correlations between patient and parent PCQLI scores (r = 0.41–0.61; P <.001); and fair to good correlations between PCQLI total scores and PedsQL total (r = 0.70–0.76), SPPC/SPPA global self-worth (r = 0.43–0.46), YSR/CBCL total competency (r = 0.28–0.37), and syndrome and Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition-oriented scale (r = −0.58 to −0.30; P < .001) scores. Test-retest reliability correlations were excellent (r = 0.78–0.90; P < .001).

CONCLUSIONS

PCQLI scores are valid and reliable for children and adolescents with congenital and acquired HD and may be useful for future research and clinical management. Pediatrics 2010;126:498–508

OBJECTIVE

We sought to compare the tuberculin skin test (TST) to the QuantiFERON-TB Gold In-Tube assay (QFT-IT) and assess the effects of malnourishment and intestinal helminth infection on QFT-IT results.

METHODS

In this population-based cross-sectional study from Dhaka, Bangladesh, we screened children for latent tuberculosis infection with the QFT-IT and TST. We assess the agreement between the TST and QFT-IT, risk factors associated with indeterminate QFT-IT results, and magnitude of interferon γ (IFN-γ) production.

RESULTS

Three hundred and two children (aged 11–15.3 years) were enrolled, including 93 (30.8%) who were malnourished. Of 251 participants who provided stool samples, 117 (46.6%) were infected with Ascaris lumbricoides and/or Trichuris trichiura. TST results were positive (≥10 mm) for 101 (33.4%) children and negative for 201 (66.6%) children. QFT-IT results were positive for 107 (35.4%) children, negative for 121 (40.1%) children, and indeterminate for 74 (24.5%) children. Agreement between the tests was moderate (κ = 0.55 [95% confidence interval: 0.44–0.65]; P < .0001) when excluding indeterminate results. Children with indeterminate QFT-IT results were separately compared with children with positive and negative QFT-IT results; malnutrition (P = .0006 and .0003), and helminth infection (P = .05 and .02), and the statistical interaction between these 2 terms (P = .03 and .004) were associated with indeterminate results. Higher levels of IFN-γ in response to tuberculosis antigens were associated with positive TST results (P < .0001); lower levels were associated with malnutrition (P = .02).

CONCLUSIONS

Malnutrition and helminth infections were associated with indeterminate QFT-IT results. Therefore, the presence of such conditions may limit the interpretability of QFT-IT results in children.

OBJECTIVE

We assessed the neurodevelopment of infants with and without deformational plagiocephaly (DP), at an average age of 6 months.

METHODS

The Bayley Scales of Infant Development III (BSID-III) were administered to 235 case subjects and 237 demographically similar, control participants. Three-dimensional head photographs were randomized and rated for severity of deformation by 2 craniofacial dysmorphologists who were blinded to case status.

RESULTS

We excluded 2 case subjects with no photographic evidence of DP and 70 control subjects who were judged to have some degree of DP. With control for age, gender, and socioeconomic status, case subjects performed worse than control subjects on all BSID-III scales and subscales. Case subjects’ average scores on the motor composite scale were ~10 points lower than control subjects’ average scores (P < .001). Differences for the cognitive and language composite scales were ~5 points, on average (P < .001 for both scales). In subscale analyses, case subjects’ gross-motor deficits were greater than their fine-motor deficits. Among case subjects, there was no association between BSID-III performance and the presence of torticollis or infant age at diagnosis.

CONCLUSIONS

DP seems to be associated with early neurodevelopmental disadvantage, which is most evident in motor functions. After follow-up evaluations of this cohort at 18 and 36 months, we will assess the stability of this finding. These data do not necessarily imply that DP causes neurodevelopmental delay; they indicate only that DP is a marker of elevated risk for delays. Pediatricians should monitor closely the development of infants with this condition.

OBJECTIVE

The purpose of this study was to investigate the impact of prenatal exposure to chlorpyrifos on 3-year neurodevelopment and behavior in a sample of inner-city minority children.

METHODS

As part of an ongoing prospective cohort study in an inner-city minority population, neurotoxicant effects of prenatal exposure to chlorpyrifos were evaluated in 254 children through the first 3 years of life. This report examined cognitive and motor development at 12, 24, and 36 months (measured with the Bayley Scales of Infant Development II) and child behavior at 36 months (measured with the Child Behavior Checklist) as a function of chlorpyrifos levels in umbilical cord plasma.

RESULTS

Highly exposed children (chlorpyrifos levels of >6.17 pg/g plasma) scored, on average, 6.5 points lower on the Bayley Psychomotor Development Index and 3.3 points lower on the Bayley Mental Development Index at 3 years of age compared with those with lower levels of exposure. Children exposed to higher, compared with lower, chlorpyrifos levels were also significantly more likely to experience Psychomotor Development Index and Mental Development Index delays, attention problems, attention-deficit/hyperactivity disorder problems, and pervasive developmental disorder problems at 3 years of age.

CONCLUSIONS

The adjusted mean 36-month Psychomotor Development Index and Mental Development Index scores of the highly and lower exposed groups differed by only 7.1 and 3.0 points, respectively, but the proportion of delayed children in the high-exposure group, compared with the low-exposure group, was 5 times greater for the Psychomotor Development Index and 2.4 times greater for the Mental Development Index, increasing the number of children possibly needing early intervention services.

OBJECTIVES

Leukocyte Adhesion Deficiency (LAD) is a rare primary immune disorder caused by defects of the CD18 β-integrin molecule on immune cells. The condition usually presents in early infancy and is characterised by deep tissue infections, leukocytosis with impaired formation of pus and delayed wound healing. Allogeneic haematopoietic stem cell transplantation (HSCT) offers the possibility of curative therapy, and with patient numbers at any individual centre being limited, we surveyed the transplant experience at 14 centres worldwide.

PATIENTS & METHODS

The course of 36 children with a confirmed diagnosis of LAD who underwent HSCT between 1993 and 2007 was retrospectively analysed. Data was collected by the registries of the European Society for Immunodeficiencies (ESID)/European Group for Blood and Marrow Transplantation (EBMT), and the Center for International Blood and Marrow Transplant Research (CIBMTR)

RESULTS

At median followup of 62 months (extending to 14 years) overall survival was 75%. Myeloablative conditioning regimens were used in 28 patients, and reduced intensity conditioning (RIC) in 8 patients, with no deaths in this subgroup. Survival after matched family donor and unrelated donor transplants was similar, with 11/14 matched family donor and 12/14 unrelated donor recipients alive; mortality was greatest following haplo-identical transplants, where 4/8 children did not survive. Twenty seven transplant recipients are alive, with full donor engraftment in 17 cases, mixed multi-lineage chimerism in 7 patients, and mononuclear cell restricted chimerism in a further 3 cases.

CONCLUSIONS

HSCT offers long term benefit in LAD and should be considered as an early therapeutic option if a suitable HLA-matched stem cell donation is available. Reduced intensity conditioning was particularly safe, and mixed donor chimersim appears sufficient to prevent significant symptoms, although careful long term monitoring will be required for these patients.

OBJECTIVE

We measured the relative impact of influenza and respiratory syncytial virus (RSV) infections in young children in terms of emergency department (ED) visits, clinical care requirements, and overall resource use.

METHODS

Patients who were aged ≤7 years and treated in the ED of a tertiary care pediatric hospital for an acute respiratory infection were enrolled during 2 winter seasons between 2003 and 2005. We quantified health care resource use for children with influenza or RSV infections, and extrapolated results to estimate the national resource use associated with influenza and RSV infections.

RESULTS

Nationally, an estimated 10.2 ED visits per 1000 children were attributable to influenza and 21.5 visits per 1000 to RSV. Children who were aged 0 to 23 months and infected with RSV had the highest rate of ED visits with 64.4 visits per 1000 children. Significantly more children required hospitalization as a result of an RSV infection compared with influenza, with national hospitalization rates of 8.5 and 1.4 per 1000 children, respectively. The total number of workdays missed yearly by caregivers of children who required ED care was 246 965 days for influenza infections and 716 404 days for RSV infections.

CONCLUSION

For young children, RSV is associated with higher rates of ED visits, hospitalization, and caregiver resource use than is influenza. Our results provide data on the large number of children who receive outpatient care for influenza and RSV illnesses and serve to inform analyses of prevention programs and treatments for both influenza and RSV disease.

OBJECTIVES

To identify patterns of shared decision-making (SDM) among a nationally representative sample of US children with attention-deficit/hyperactivity disorder (ADHD) or asthma and determine if demographics, health status, or access to care are associated with SDM.

PATIENTS AND METHODS

We performed a cross-sectional study of the 2002–2006 Medical Expenditure Panel Survey, which represents 2 million children with ADHD and 4 million children with asthma. The outcome, high SDM, was defined by using latent class models based on 7 Medical Expenditure Panel Survey items addressing aspects of SDM. We entered factors potentially associated with SDM into logistic regression models with high SDM as the outcome. Marginal standardization then described the standardized proportion of children’s households with high SDM for each factor.

RESULTS

For both ADHD and asthma, 65% of children’s households had high SDM. Those who reported poor general health for their children were 13% less likely to have high SDM for ADHD (64 vs 77%) and 8% less likely for asthma (62 vs 70%) when adjusting for other factors. Results for behavioral impairment were similar. Respondent demographic characteristics were not associated with SDM. Those with difficulty contacting their clinician by telephone were 26% (ADHD: 55 vs 81%) and 29% (asthma: 48 vs 77%) less likely to have high SDM than those without difficulty.

CONCLUSIONS

These findings indicate that households of children who report greater impairment or difficulty contacting their clinician by telephone are less likely to fully participate in SDM. Future research should examine how strategies to foster ongoing communication between families and clinicians affect SDM.

OBJECTIVE

A gluten-free camp allows children with celiac disease (CD) to enjoy a camp experience without concern and preoccupation with foods they eat or the stigma of their underlying disease. The objective of this study was to evaluate the impact of gluten-free camp on quality-of-life indicators for children and adolescents with CD.

METHODS

Children aged 7 to 17 years with CD were administered a 14-question survey at the beginning and the end of a 7-day gluten-free camp. Surveys used a Likert scale to examine general well-being, emotional outlook, and self-perception for the week before each survey. Differences between the time points were compared. Data were analyzed by paired t test.

RESULTS

Of the 104 campers who attended camp, 77 (21 male) completed the survey at both time points. Most (70%) had been on a glutenfree diet (GFD) for <4 years. All seemed to benefit from camp, no longer feeling different from other kids or feeling frustrated with a restricted diet. A more beneficial impact was found for campers who were on a GFD for <4 years. Overall, campers reported an improvement in 11 of 14 questions, statistically significant (P <.05) for 8 of those 11 questions. Improvement was observed in each of the 3 categories of questions: well-being, self-perception, and emotional outlook.

CONCLUSIONS

Children who had CD and attended a week-long glutenfree camp demonstrated improvement in well-being, self-perception, and emotional outlook. The positive effects of camp were more apparent among campers who had been on a GFD for <4 years compared with those who had been on a GFD for ≥4 years, suggesting an adaptation to CD with time. A gluten-free camp that provides an environment of unrestricted foods can at least temporarily alleviate stress and anxiety around food and social interactions. Durability of these observations on return to daily life requires additional study.

Objective

To determine whether N-carbamylglutamate reduces plasma levels of ammonia and glutamine and increases ureagenesis rate in patients with propionic acidemia

Patients and Methods

Identical four-hour studies were performed before and immediately after a 3-day trial of oral N-carbamylglutamate in 7 patients with propionic acidemia. An oral bolus of [13C]-sodium acetate was administered at the start of each study, and sequential blood samples were obtained to measure [13C]-urea, ammonia, urea and amino acids.

Results

With longitudinal mixed effects linear regression, peak [13C]urea increased following treatment with N-carbamylglutamate (from 2.2 μM to 3.8 μM; p < 0.0005). There were concomitant decreases in mean plasma ammonia (59 to 43 μM, p <0.0005)) and glutamine (552 to 331 μM, p <0.0005).

Conclusion

N-carbamylglutamate augments ureagenesis and decreases plasma ammonia and glutamine in patients with propionic acidemia. The drug may serve as an important therapeutic adjunct in the treatment of acute hyperammonemia in this disorder.

BACKGROUND

The lipid-laden macrophage index has been used to evaluate for gastroesophageal reflux-related respiratory disease, but the relationship between reflux detected by pH probe and the lipid-laden macrophage index is uncertain despite widespread use of the lipid-laden macrophage index in clinical decision-making. It was the aim of this study to correlate reflux as detected by multichannel intraluminal impedance with the lipid-laden macrophage index.

METHODS

Patients undergoing both pH multichannel intraluminal impedance testing and bronchoscopy between January 2002 and January 2006 were identified. Baseline characteristics were compared by using parametric and nonparametric testing. Reflux profiles were correlated with the lipid-laden macrophage index by using Spearman correlations.

RESULTS

There was no significant correlation between the lipid-laden macrophage index and the number of acid or nonacid reflux events. There also was no significant correlation between the lipid-laden macrophage index and the amount of full-column reflux. There was no significant difference between the mean lipid-laden macrophage index in patients with and without esophagitis. Finally, in patients who underwent fundoplication (n = 13) for intractable respiratory disease, there was no significant difference in any of the reflux parameters between patients who did and did not experience clinical improvement after fundoplication. There was, however, a higher lipid-laden macrophage index in patients with no symptomatic improvement compared with patients with symptomatic improvement.

CONCLUSIONS

Lipid-laden macrophage index lacks the specificity necessary to detect reflux-related respiratory disease.