Search

The objective of this study was to retrospectively evaluate the utility of serum neopterin as a diagnostic marker of hemophagocytic lymphohistiocytosis (HLH). The medical records of patients diagnosed with HLH (familial and secondary) between January 2000 and May 2009 were reviewed retrospectively, and clinical and laboratory information related to HLH criteria, in addition to neopterin levels, was recorded. A group of 50 patients with active juvenile dermatomyositis (JDM) (who routinely have neopterin levels assessed) served as controls for the assessment of the accuracy, sensitivity, and specificity of neopterin as a diagnostic test for HLH. The Pearson correlation was used to measure the association between serum neopterin levels and established HLH-related laboratory data. Serum neopterin levels were measured using a competitive enzyme immunoassay. During the time frame of the study, 3 patients with familial HLH and 18 patients with secondary HLH were identified as having had serum neopterin measured (all HLH patients were grouped together). The mean neopterin levels were 84.9 nmol/liter (standard deviation [SD], 83.4 nmol/liter) for patients with HLH and 21.5 nmol/liter (SD, 10.13 nmol/liter) for patients with JDM. A cutoff value of 38.9 nmol/liter was 70% sensitive and 95% specific for HLH. For HLH patients, neopterin levels correlated significantly with ferritin levels (r= 0.76,P= 0.0007). In comparison to the level in a control group of JDM patients, elevated serum neopterin was a sensitive and specific marker for HLH. Serum neopterin has value as a diagnostic marker of HLH, and prospective studies are under way to further evaluate its role as a marker for early diagnosis and management of patients.

Correlate adenoid size as determined by lateral neck radiographs and intra-operative mirror exam. Determine if a radiologist's assessment of the lateral neck X-ray correlates with adenoid size. Assess the correlation of endoscopic findings to the degree of adenoid hypertrophy seen on intra-operative mirror exam. To perform a cost analysis of radiographic and endoscopic evaluations of the adenoids.Retrospective study.Patients who underwent adenoidectomy were reviewed. The adenoid size as determined by the adenoid-to-nasopharyngeal (A/N) ratio, radiology report, and flexible nasal endoscopy were compared to the adenoid size as determined by intra-operative mirror nasopharyngeal exam. Compensation rates for each modality were compared.Sixty-one children had pre-operative airway radiography. Ninety-nine patients underwent flexible nasopharyngoscopy. When the A/N ratio was compared to the intra-operative mirror exam, the Pearson Correlation coefficient was 0.64 (p0.0001). The radiology reading was compared to intra-operative mirror exam and the Spearman Correlation coefficient was 0.29 (p=0.0258). When endoscopic nasopharyngoscopy was compared to intra-operative mirror exam, the Pearson Correlation coefficient was 0.62 (p0.0001). The cost of nasal endoscopy was $654. Lateral airway radiography plus radiology interpretation cost $605.Children who undergo lateral radiographs to assess adenoid size are younger than those who undergo awake flexible endoscopic nasopharyngoscopy. Both the A/N ratio and endoscopic nasopharyngoscopy correlate well with the findings of the intra-operative mirror exam. The radiologist interpretations that do not utilize the A/N ratio measurement do not correlate well with intra-operative mirror exam findings. Both modalities are comparable in cost.

Juvenile dermatomyositis (JDM) is a systemic vasculopathy characterized by skin and muscle involvement. Diagnostic criteria include muscle weakness and rash, electromyographic evidence of myopathy, muscle biopsy demonstrating inflammatory infiltrate with or without atrophy, and elevated muscle enzymes, although muscle enzymes may be normal (1). The etiology of JDM has not been established, but evidence suggests that environmental triggers such as an antecedent infection may play a role (2). Many children with JDM have a TNFα–308A allele which is associated with vascular occlusion(3) and increased TNF-α production (4). In addition, JDM disease activity has been associated with decreased absolute numbers of CD3−CD56+/16+ NK cells and elevated von Willebrand factor antigen (vWF:Ag) levels (5). Genes involved in immune responses, including those related to dendritic cell maturation and vasculature remodeling, are expressed at higher levels in muscle biopsies from children with greater duration of untreated disease (6). One particular manifestation of JDM is the presence of abnormal nailfold capillaries, evidenced by capillary dropout, capillary dilatation, and bushy loops (7). Nailfold capillaroscopy (NFC) is a noninvasive, reproducible technique that provides information about abnormalities in periungual microvasculature. It takes into account quantitative measurements of capillary density or end-row loop (ERL) loss as well as the presence of avascularity and abnormal capillaries represented by “bushy” or “bizarre” loops (7,8). ERL scores between 7.0 and 11.5 have been observed in healthy children without JDM or other recognized autoimmune diseases (9). Serial nailfold capillary observations have demonstrated progression of nailfold changes over time in children with JDM (6), 10- 12) and their degree of morphologic changes may correlate with the clinical course of the disease (10). While abnormal NFC are not part of the Bohan and Peter criteria for dermatomyositis, they may reflect systemic vasculopathy, and the data can be used to evaluate patients with JDM (13). ERL regeneration is associated with a shorter duration of untreated disease, a unicyclic disease course resulting in discontinuation of all immunosuppressive therapy before 36 months (versus non-unicyclic in which continuous or repeated immunosuppressive therapy is required), and a lower skin disease activity score (DAS), (11), (12). Lower numbers of ERL in children with JDM are also associated with decreased bioavailability of oral prednisolone compared with intravenous methylprednisolone, suggesting a relationship between systemic vasculopathy and decreased absorption of oral prednisolone (14). Another recent study also showed that six months after diagnosis, the presence of abnormal nailfold capillaries with Gottron’s papules predicted a longer time to remission (15). Given the potential uses of NFC in providing valuable clinical information with regard to JDM, we studied untreated children fulfilling criteria for definite/probable JDM for the following: 1) the association of normal ERL at the time of diagnosis with clinical and laboratory data, and 2) predictors of decreased number of ERL over time.

Our objective was to examine clinical/electrocardiogram (ECG) predictors and outcomes of arrhythmias beyond 1 year after pediatric heart transplantation (HTx). We performed a retrospective chart review of 94 1-year HTx survivors, 1988–2006. Clinical records identified patients with arrhythmias occurring >1 year after HTx requiring pharmacotherapy, excluding acute rejection. We reviewed preoperative diagnosis, gender, age at HTx, operative details, transplant coronary artery disease (TCAD), and mortality. We analyzed serial ECGs after HTx for HR, PR, QRS, QT, and QTc intervals. Our results found complete data in 58 patients, 14 (24%) with arrhythmia and 44 controls. Arrhythmias occurred 1.1–17.9 years after HTx (mean = 6.8): 11 focal atrial tachycardia, 1 atrial fibrillation/flutter, 1 atrioventricular node reentry tachycardia; only 1 patient had ventricular tachycardia (VT). Serial ECG intervals were similar between groups, as well as surgical technique, ischemic time, and rejection history. Seven patients (50%) with arrhythmias had death or graft death versus 11% of the controls (P = 0.006). Patients with arrhythmias were more likely to be diagnosed with TCAD (P = 0.007). The patient with VT had no TCAD. In conclusion, supraventricular arrhythmias were frequent (22%) in 1-year survivors of pediatric HTx. These patients were more likely to develop TCAD and/or graft loss/mortality.

Hispanic ethnicity is an independent risk factor for increased morbidity and mortality in cystic fibrosis (CF) patients. In order to compare the prevalence of risk factors for morbidity and mortality between the Hispanic CF population and the non-Hispanic CF population, we performed a cross-sectional study of patients in the 2004 Cystic Fibrosis Foundation Patient Registry. Among 22,714 CF patients, 1,511 were identified as ethnic Hispanic. Hispanic patients were diagnosed earlier (2.8 vs. 3.3 years, P = 0.005) and acquired Pseudomonas aeruginosa at a younger age (6.6 years vs. 10 years, P < 0.001). FEV(1) was lower for Hispanic patients (81.5% vs. 87% predicted for those under 18 years old [P < 0.001] and 2.1 L vs. 2.3 L for those 18 years and older [P = 0.01]). Hispanic patients had similar or better nutritional status. Hispanic patients were more likely to be diagnosed with liver disease (OR 1.31 [1.1, 1.56]) but less likely to be diagnosed with depression (OR 0.53 [0.39, 0.68]), bone and joint disease (OR 0.55 [0.41, 0.71]), or CF-related diabetes (OR 0.53 [0.43, 0.62]). Hispanic patients had lower median income by zip code ($41,930 vs. $47,341; P < 0.001), a higher rate of government insurance (55.2% vs. 32.0%; P < 0.001), and greater percentage of mothers with less than a high school education (26.7% vs. 6.5%; P < 0.001). We conclude that there is an increased prevalence of important risk factors for morbidity and mortality in the Hispanic CF population.

ADV has emerged as an important pathogen in children undergoing allogeneic HPCT. A prospective study of the epidemiology of ADV infection and preemptive therapy of high risk ADV infections in children undergoing HPCT was undertaken. Cultures of throat, urine, and stool for viral pathogens and plasma for ADV PCR were obtained prior to transplantation, weekly for the first 100 days, and then monthly for one yr. Children developing high-risk ADV infections were treated preemptively with cidofovir 1 mg/kg/day given three times weekly for three wk. A case-controlled study was performed to identify risk factors for high-risk ADV infections. Seven (18%) of the 38 subjects developed high-risk ADV infections usually within 100 days of HPCT and were preemptively treated with i.v. cidofovir at a dose of 1 mg/kg/dose three times weekly for nine doses. High-risk ADV infections resolved in all seven patients without renal toxicity. CMV viremia occurred in two of seven patients during or shortly after therapy with cidofovir. A case-control study did not identify any risk factors that achieved statistical significance. Treatment with a modified dosing regimen of cidofovir was well-tolerated and high-risk ADV infections resolved in all patients.

Although HIV risk in male-to-female (MTF) transgender adults is well documented, limited data exists on the experiences of MTF transgender youth. This cross-sectional study examines environmental, psychosocial, and individual correlates of risky sex among 51 MTF ethnie minority transgender youth age 16-25. Fifty-nine percent of participants reported high-risk sex defined as either unprotected insertive or receptive anal intercourse in the past year. Multiple factors that included higher depression scores, lower self-esteem, less social support, poorer safer sex communication skills, being non-African American, use of injection silicone, history of arrest, history of forced sex and having sex while high on drugs/alcohol, were significantly associated with high-risk sex among participants. Multivariate logistic regression indicated that among possible models with two independent variables, poorer communication skills and non-African American race/ethnicity were the most significant predictors of hi...

Nosocomial transmission of respiratory syncytial virus (RSV) is a serious, preventable cause of RSV infection. Passive RSV prophylaxis became available in 1996. We compared the RSV nosocomial infection rate in our neonatal intensive care unit before and after RSV prophylaxis, using nosocomial rotavirus infections as a comparator. There were no significant differences between nosocomial RSV infection rates before and after institution of prophylaxis.

Previous research indicates a role of pheromones in novel male-induced early pregnancy disruptions. Although some reports suggest that urine alone is sufficient to produce this effect, others raise procedural concerns and fail to replicate such effects. On Days 1 to 5 after insemination, female CF-1 mice had their nasal regions repeatedly painted with water, urine from males housed in isolation, or urine from males housed in proximity to females. Almost all (87.5%) of the control females delivered litters. There was a small nonsignificant reduction in proportion parturient (78.5%) among females exposed to urine of males housed without social contact. The proportion of females parturient (57.1%) after treatment with urine from males housed in proximity to females was significantly different from controls. The magnitude of the effect of socially stimulated male urine is substantially less than that recorded when males are housed directly above inseminated females separated by a wire-mesh grid. These data suggest that production of pregnancy-disrupting male pheromones is stimulated by contact with conspecifics.

It has recently been demonstrated that variability in blood phenylalanine levels is inversely correlated with IQ and is a better predictor of IQ in early and continuously treated patients with phenylketonuria (PKU) than mean blood phenylalanine levels. This suggests that stability of blood phenylalanine should be a therapeutic goal in patients with PKU. The purpose of this study was to determine if treatment with sapropterin in patients with BH4-responsive PKU would increase the stability of blood phenylalanine levels. The records of all patients treated with sapropterin in the PKU Clinic at Children's Memorial Hospital in Chicago were examined retrospectively. Patients were included in the study if they were responsive to sapropterin during a 2- to 4-week challenge (reduction of blood phenylalanine level of at least 25% after 2weeks of therapy or, in the case of patients with well-controlled blood phenylalanine at the time of testing, increased dietary phenylalanine tolerance by 4weeks of treatment). A total of 37 subjects were eligible for inclusion (16male; 21 female); the mean age was 12.6years (range, 1.5–32.0). The total number of observations (phenylalanine levels) for all subjects was 1391 with a mean of 39 per subject (range, 13–96). Linear mixed modeling was utilized to estimate variances of the blood phenylalanine before (pre) and after (post) starting sapropterin. Likelihood ratio test was performed using SAS 9.1. Means and standard deviations for phenylalanine as estimated by the model were 6.67mg/dl (4.20) and post 5.16 (3.78). The mean level post-sapropterin was significantly lower ( p =.0002). The within-subject variances (mean and SD) of phenylalanine were: pre 6.897 (2.62) and post 4.799 (2.19). These two variances are significantly different with a p =.0017. We conclude that sapropterin therapy results in increased stability of blood phenylalanine levels. This effect is likely to improve cognitive outcome in BH4-responsive patients with PKU.

To examine risk factors for lack of immunization, we tested the impact of maternal, paternal, and household variables on child immunization status in childrenor =1 year in a rural area of Ethiopia. Data collected by face-to-face interview on maternal, paternal, household and child variables from cross-sectional random sample community-based study on health and well-being in rural Ethiopia was used to test hypotheses on immunization status of children (n = 924). Bivariate and multivariate logistic regression models were used for two immunization outcomes: record of at least one vaccination, and record of DPT3, indicating completion of the DPT series. Complete data were available for 924 childrenor =1 year of which 79% had at least one vaccination. Of those, 64% had DPT3/Polio3; below recommended coverage level. Children were more likely to be vaccinated if the mother reported antenatal care (ANC), and less likely to be vaccinated if the mother had a history of stillbirth, and no opinion of health center. Children were more likely to have DPT3 if: mother hador =1 year of education, mother reported ANC, or older paternal age. Children were less likely to have DPT3 in households with food insecurity and no maternal opinion of health center. The study had three findings with implications for immunization programming: (1) Mothers completing the recommended ANC visits is strongly associated with receiving at least one vaccination and with completing a vaccination series; (2) Maternal education is associated with a completed vaccination series; (3) Paternal characteristics may affect vaccination series completion.

Approximately 10% to 50% of competitive athletes experience asthma symptoms with exercise, due to either chronic asthma or exercise-induced bronchospasm. Early recognition and management of asthma symptoms may improve athletic performance and quality of life for athletes with asthma or exercise-induced bronchospasm. Athletic trainers may have frequent opportunities to identify asthma symptoms and assist athletes with management.To survey athletic trainers about their experience and comfort with evaluation and management of asthma symptoms in athletes and identify athletic trainer characteristics associated with higher comfort levels.A 2005 cross-sectional survey of National Athletic Trainers' Association and Illinois Athletic Trainers Association members.A total of 304 athletic trainers.Respondents completed a Web-based survey reporting years of experience, competitive level of athletes supervised, satisfaction with asthma education, experience evaluating asthma symptoms, and comfort managing asthma.Response rate was 13.9% (304 of 2,175). At least 23% of respondents evaluated asthma symptoms five or more times the previous year. Respondents working exclusively with junior high and/or high school athletes evaluated asthma symptoms more frequently than those working exclusively with college and/or professional athletes. Fifty-eight percent of respondents were unsatisfied with their asthma education. Only 25.3% were "very" comfortable managing asthma. Respondents with higher comfort levels evaluated asthma symptoms more frequently (p0.01, r = 0.18) and were more likely to be satisfied with their asthma education (p0.001). Over 95% of respondents endorsed more asthma education in athletic training curricula.Results of this pilot study indicate that athletic trainers have opportunities to help athletes manage asthma symptoms that can compromise athletic performance or limit sports participation. However, few athletic trainers are very comfortable managing asthma, and most are unsatisfied with their asthma education. Further study is needed to determine the effect of enhanced asthma education on athletic trainers' comfort and skills with asthma evaluation and management.

OBJECTIVES. The goals were to determine whether primary care provider identification of children as overweight was associated with additional screening or referrals and whether the types and numbers of visits to primary care differed for overweight and nonoverweight children. METHODS. Sequential parents/guardians at 13 diverse pediatric practices completed an in-office survey addressing health habits and demographic features. Medical records of each child from a sample of families were reviewed. Data were abstracted from the first visit and from all visits in the 14-month period before study enrollment. Analyses were limited to children ≥2 years of age for whom BMI percentile could be calculated. RESULTS. The analytic sample included 1216 children (mean age: 7.9 years; 51% male) from 777 families (parents were 43% white, 18% black, 34% Hispanic, and 5% other; 49% of families had a child receiving Medicaid/uninsured). Among overweight children (BMI of ≥95th percentile; n = 248), 28% had been identified as such in the record. Screening or referral for evaluation of comorbidities was more likely among overweight children who were identified in the record (54%) than among overweight children who were not identified (17%). Among children at risk of overweight (BMI of 85th to 94th percentile; n = 186), 5% had been identified as such in the record and overall 15% were screened/referred. In logistic regression modeling, the children identified as overweight/at risk of overweight had 6 times greater odds of receiving any management for overweight. CONCLUSIONS. Low rates of identification of overweight status and evaluation or referrals for comorbidities were found. Identification of overweight status was associated with a greatly increased rate of screening for comorbidities.

Pseudomonas aeruginosa is a frequent cause of respiratory exacerbations in individuals with cystic fibrosis. An important virulence determinant of this pathogen is its type III protein secretion system. In this study, the type III secretion properties of 435 P. aeruginosa respiratory isolates from 56 chronically infected individuals with cystic fibrosis were investigated. Although it had been previously reported that 75 to 90% of P. aeruginosa isolates from patients with hospital-acquired pneumonia secreted type III proteins, only 12% of isolates from cystic fibrosis patients did so, with nearly all of these isolates secreting ExoS and ExoT but not ExoU. Despite the low overall prevalence of type III protein-secreting isolates, at least one secreting isolate was cultured from one-third of cystic fibrosis patients. Interestingly, the fraction of cystic fibrosis patient isolates capable of secreting type III proteins decreased with duration of infection. Although 90% of isolates from the environment, the presumed reservoir for the majority of P. aeruginosa strains that infect patients with cystic fibrosis, secreted type III proteins, only 49% of isolates from newly infected children, 18% of isolates from chronically infected children, and 4% of isolates from chronically infected adults with cystic fibrosis secreted these proteins. Within individual patients, isolates of clonal origin differed in their secretion phenotypes, indicating that as strains persisted in cystic fibrosis patient airways, their type III protein secretion properties changed. Together, these findings indicate that following infection of cystic fibrosis patient airways, P. aeruginosa strains gradually change from a type III protein secretion-positive phenotype to a secretion-negative phenotype.

Objective: Length of time elapsed between recurrent laryngeal nerve (RLN) injury and surgery may affect voice result. The purpose of this study was to compare voice outcome after thyroplasty in patients with short- and long-term vocal fold immobility. Methods: Thirty-five patients with longstanding vocal fold immobility (8.3 years) and 35 patients more acutely denervated (10 months) underwent type I thyroplasty. Trained listeners before and after surgery rated perceptual judgments of voice. Acoustic and aerodynamic data were analyzed. Results: Voice outcome did not differentiate the two groups with respect to objective measures. Perceptual ratings of post-operative voice quality favored the short-term patients, with the long-term group having a mildly ‘constricted’ quality. Conclusion: Thyroplasty yields a functional voice result even when substantial time has elapsed. Patients with chronic immobility may be unable to release longstanding hyperfunctional voicing behaviors even after surgery has afforded them an improved mechanism. In five of 35 long-term patients, therapy enhanced voice quality.

The objective of this study was to retrospectively evaluate the utility of serum neopterin as a diagnostic marker of hemophagocytic lymphohistiocytosis (HLH). The medical records of patients diagnosed with HLH (familial and secondary) between January 2000 and May 2009 were reviewed retrospectively, and clinical and laboratory information related to HLH criteria, in addition to neopterin levels, was recorded. A group of 50 patients with active juvenile dermatomyositis (JDM) (who routinely have neopterin levels assessed) served as controls for the assessment of the accuracy, sensitivity, and specificity of neopterin as a diagnostic test for HLH. The Pearson correlation was used to measure the association between serum neopterin levels and established HLH-related laboratory data. Serum neopterin levels were measured using a competitive enzyme immunoassay. During the time frame of the study, 3 patients with familial HLH and 18 patients with secondary HLH were identified as having had serum neopterin measured (all HLH patients were grouped together). The mean neopterin levels were 84.9 nmol/liter (standard deviation [SD], 83.4 nmol/liter) for patients with HLH and 21.5 nmol/liter (SD, 10.13 nmol/liter) for patients with JDM. A cutoff value of 38.9 nmol/liter was 70% sensitive and 95% specific for HLH. For HLH patients, neopterin levels correlated significantly with ferritin levels (r = 0.76, P = 0.0007). In comparison to the level in a control group of JDM patients, elevated serum neopterin was a sensitive and specific marker for HLH. Serum neopterin has value as a diagnostic marker of HLH, and prospective studies are under way to further evaluate its role as a marker for early diagnosis and management of patients.

A 24-item tool, using a Likert attitudinal scale, was developed to measure labor and delivery nurses' attitudes toward fetal monitoring and was administered to 124 nurses. For each of the 24 attitude statements, cumulative responses were over 50% positive at least. Fifty-two percent of the nurses felt that routine continuous monitoring of all labor patients would be ideal, and 88% felt that the fetal surveillance achieved by monitoring cannot be matched by intermittent auscultation.

Nager's syndrome, which has the facial features of Treacher Collins syndrome, preaxial upper limb defects, short stature, and frequently subnormal intelligence, is very rare. Five new cases have been collected. In four, there was virtually total absence of the soft palate. This has been described in two patients in the past. Differing methods of correcting this are presented.

Prenatal education should be initiated as soon as any pregnancy is suspected. This article describes the development and improvement of a program of early prenatal education for a large obstetrical population initiated prior to the completion of the first trimester.