Parental reports of the oral health-related quality of life of children with cerebral palsy

Background: The severity of physical and mental impairments and oral problems, as well as socioeconomic factors, may have an impact on quality of life of children with cerebral palsy (CP). The aim of this research was to assess the impact of impairments and oral health conditions, adjusted by socioeconomic factors, on the Oral Health-Related Quality of Life (OHRQoL) of children with CP using their parents as proxies. Methods: Sixty children, between 6-14 years of age were selected. Their parents answered a children's OHRQoL instrument (5 domains) which combines the Parental-Caregivers Perception Questionnaire (P-CPQ) and Family Impact Scale (FIS). The severity of dental caries, type of CP, communication ability, gross motor function, seizures and socioeconomic conditions were assessed. Results: Considering the total score of the OHRQoL instrument, only the reduction of communication ability and dental caries severity had a negative impact on the OHRQoL (p < 0.05). Considering each domain of the instrument, the severity of the type of CP and its reduction of communication ability showed a negative impact on oral symptoms and functional limitations domains (p < 0.05). Seizures have a negative impact on oral symptoms domain (p = 0.006). The multivariate fitted model showed that the severity of dental caries, communication ability and low family income were negatively associated with the impact on OHRQoL (p = 0.001). Conclusions: The severity of dental caries, communication ability, and family income are conditions strongly associated with a negative impact on OHRQoL of children with CP.

Pharmacokinetics of cyclosporin - a microemulsion in children with idiopathic nephrotic syndrome

OBJECTIVE: We present a prospective study of a microemulsion of cyclosporin to treat idiopathic nephrotic syndrome in ten children with normal renal function who presented cyclosporin trough levels between 50 and 150 ng/ml and achieved complete remission with cyclosporin. To compare the pharmacokinetic parameters of cyclosporin in idiopathic nephrotic syndrome during remission and relapse of the nephrotic state. METHOD: The pharmacokinetic profile of cyclosporin was evaluated with the 12-hour area under the time-concentration curve (auc0-12) using seven time-point samples. This procedure was performed on each patient during remission and relapse with the same cyclosporin dose in mg/kg/day. The 12-hour area under the time-concentration curve was calculated using the trapezoidal rule. All of the pharmacokinetic parameters and the resumed 4-hour area under the time-concentration curve were correlated with the 12-hour area under the time-concentration curve. ClinicalTrials.gov: NCT01616446. RESULTS: There were no significant differences in any parameters of the pharmacokinetic of cyclosporin during remission and relapse, even when the data were normalized by dose. The best correlation with the 12-hour area under the time-concentration curve was the 4-hour area under the time-concentration curve on remission and relapse of the disease, followed by the 2-hour level after cyclosporin (c2) dosing in both disease states. CONCLUSIONS: These data indicate that the same parameters used for cyclosporin therapeutic monitoring estimated during the nephrotic state can also be used during remission. Larger controlled studies are needed to confirm these findings.

Isokinetic analysis of the elbow joint in children with cerebral palsy

The joint torque is an important variable related to children with cerebral palsy. The present study analyzed kinetic parameters during elbow flexion and extension movements in healthy and cerebral palsy children. Ten healthy and 10 cerebral palsy children participated of the study. An isokinetic dynamometer was used to measure the elbow mean peak torque, mean angle peak torque, coefficient of variation and acceleration during flexion and extension movements at different angular speeds. The mean peak torque on extension movement in healthy children group was significant higher compared to the cerebral palsy group. The coefficient of variation on both flexion and extension movements was significantly higher in cerebral palsy group. However there were significantly difference on both groups compared the lowest and highest velocities. Although the results showed no difference in flexor peak torque, the acceleration is significantly lower in lowest and highest angular velocity.

Perinatal and early life factors associated with symptoms of depression in Brazilian children

Background: Few studies have been conducted on the association between perinatal and early life factors with childhood depression and results are conflicting. Our aim was to estimate the prevalence and perinatal and early life factors associated with symptoms of depression in children aged 7 to 11 years from two Brazilian birth cohorts. Methods: The study was conducted on 1444 children whose data were collected at birth and at school age, in 1994 and 2004/2005 in Ribeirao Preto, where they were aged 10-11 years and in 1997/98 and 2005/06 in Sao Luis, where children were aged 7-9 years. Depressive symptoms were investigated with the Child Depression Inventory (CDI), categorized as yes (score >= 20) and no (score < 20). Adjusted and non-adjusted prevalence ratios (PR) were estimated by Poisson regression with robust estimation of the standard errors. Results: The prevalence of depressive symptoms was 3.9% (95% CI = 2.5-5.4) in Ribeirao Preto and 13.7% (95% CI = 11.0-16.4) in Sao Luis. In the adjusted analysis, in Ribeirao Preto, low birth weight (PR = 3.98; 95% CI = 1.72-9.23), skilled and semi-skilled manual occupation (PR = 5.30; 95% CI = 1.14-24.76) and unskilled manual occupation and unemployment (PR = 6.65; 95% CI = 1.16-38.03) of the household head were risk factors for depressive symptoms. In Sao Luis, maternal schooling of 0-4 years (PR = 2.39; 95% CI = 1.31-4.34) and of 5 to 8 years (PR = 1.80; 95% CI = 1.08-3.01), and paternal age < 20 years (PR = 1.92; 95% CI = 1.02-3.61), were independent risk factors for depressive symptoms. Conclusions: The prevalence of depressive symptoms was much higher in the less developed city, Sao Luis, than in the more developed city, Ribeirao Preto, and than those reported in several international studies. Low socioeconomic level was associated with depressive symptoms in both cohorts. Low paternal age was a risk factor for depressive symptoms in the less developed city, Sao Luis, whereas low birth weight was a risk factor for depressive symptoms in the more developed city, Ribeirao Preto.

Quantitative comparison of mobility and gross motor function in Brazilian children with cerebral palsy

Background: Cerebral palsy (CP) presents changes in posture and movement as a core characteristic, which requires therapeutic monitoring during the habilitation or rehabilitation of children. Besides clinical treatment, it is fundamental that professionals use systems of evaluation to quantify the difficulties presented to the individual and assist in the organization of a therapeutic program. The aim of this study was to quantitatively verify the performance of children with spastic di-paresia type CP. Methods: The Pediatric Evaluation of Disability Inventory (PEDI) and Gross Motor Function Classification System (GMFM) tests were used and classification made through the GMFCS in the assessment of 7 patients with CP, 4 females and 3 males, average age of 9 years old. Results: According to GMFCS scales, 17% (n=1) were level II and 83% (n=6) were level III. The PEDI test and 88 GMFM items were used in the area of mobility. We observed that there was high correlation between mobility and gross motor function with Pearson's correlation coefficient =0.929) showing the likely impact of these areas in the functional skills and the quality of life of these patients. Conclusion: We suggest the impact of the limitation of the areas in functional skills and quality of life of these patients.

Do children with motor difficulties show low levels of physical fitness?

The aim of the present study was to examine whether children with motor difficulties would show levels of health-related components of physical fitness lower than children without such difficulties. Sixteen children with motor difficulties with approximately 8 years of age were age-and gender-matched with other 16 children without motor difficulties. Participants completed the test batteries for physical fitness (seat and reach, standing long jump, sit-up, modified pull-up and 9-minute run) and for motor coordination (MABC-2). The results indicated significant differences in standing long jump, sit-up, modified pull-up tests, but not for the seat and reach and the 9-minute run tests. Overall, the results are similar to those published in the international literature, with the exception of cardiorespiratory fitness. The idea that at to some degree coordination is required to perform physical fitness tasks and might impact on physical fitness performance is further discussed in the present work.

The therapeutic journey of families of children with respiratory diseases in the public health service

This study's purpose was to identify the therapeutic journey of families seeking health care for their children with respiratory diseases. This qualitative study had the participation of parents of children younger than five years old who were hospitalized with respiratory diseases. Path mapping was used as an instrument to collect data, which was analyzed through thematic analysis. The finding indicate that families sought the health services as soon as they perceived symptoms and had access to medical care, however such care was not decisive in resolving their health issues. Even though the families returned to the service at least another three times, the children had to be hospitalized. The attributes of primary health care were not observed in the public health services, while therapeutic encounters had no practical success.

High Rates of Detection of Respiratory Viruses in Tonsillar Tissues from Children with Chronic Adenotonsillar Disease

Chronic tonsillar diseases are an important health problem, leading to large numbers of surgical procedures worldwide. Little is known about pathogenesis of these diseases. In order to investigate the role of respiratory viruses in chronic adenotonsillar diseases, we developed a cross-sectional study to determine the rates of viral detections of common respiratory viruses detected by TaqMan real time PCR (qPCR) in nasopharyngeal secretions, tonsillar tissues and peripheral blood from 121 children with chronic tonsillar diseases, without symptoms of acute respiratory infections. At least one respiratory virus was detected in 97.5% of patients. The viral co-infection rate was 69.5%. The most frequently detected viruses were human adenovirus in 47.1%, human enterovirus in 40.5%, human rhinovirus in 38%, human bocavirus in 29.8%, human metapneumovirus in 17.4% and human respiratory syncytial virus in 15.7%. Results of qPCR varied widely between sample sites: human adenovirus, human bocavirus and human enterovirus were predominantly detected in tissues, while human rhinovirus was more frequently detected in secretions. Rates of virus detection were remarkably high in tonsil tissues: over 85% in adenoids and close to 70% in palatine tonsils. In addition, overall virus detection rates were higher in more hypertrophic than in smaller adenoids (p = 0.05), and in the particular case of human enteroviruses, they were detected more frequently (p = 0.05) in larger palatine tonsils than in smaller ones. While persistence/latency of DNA viruses in tonsillar tissues has been documented, such is not the case of RNA viruses. Respiratory viruses are highly prevalent in adenoids and palatine tonsils of patients with chronic tonsillar diseases, and persistence of these viruses in tonsils may stimulate chronic inflammation and play a role in the pathogenesis of these diseases.

Immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus

Objectives The aim of the present paper is to evaluate the immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus. Methods We performed a prospective and controlled study on a group of 54 SLE patients that were chosen at random to be or not to be vaccinated (28 were vaccinated and 26 were not). Twenty-eight healthy controls, of matching age and sex were also vaccinated. All were submitted to a questionnaire, physical evaluation and laboratory assays: lymphocyte immuno-phenotyping by flow cytometry, plasma varicella zoster virus (VZV) serology by ELISA and in vitro interferon gamma (IFN gamma) production by T-cells after stimulus with VZV antigen. They were evaluated before vaccination and at 30, 45, 180 and 360 days afterwards. Results We did not observe any differences in the frequency of adverse events in both vaccinated groups. At study entry, all individuals were seropositive for VZV antibodies. The serum VZV antibody titres similarly increased after vaccination. The frequency of flares and the SLEDAI score were also similar among the patients. Thirty days after vaccination the production of IFN gamma specific to VZV was lower in the SLE group compared to healthy, controls. In the follow-up we observed 4 cases of herpes zoster in the SLE unvaccinated group, but no zoster in the vaccinated group. Conclusion The varicella vaccine was well tolerated in SLE group, who had pre-existing immunity to varicella. The varicella vaccine immunogenicity measurement by serum antibody titres was appropriate. The incidence of HZ was lower in the vaccinated lupus group.

Prediction of tolerance in children with IgE mediated cow's milk allergy by microarray profiling and chemometric approach

The sera of a retrospective cohort (n = 41) composed of children with well characterized cow's milk allergy collected from multiple visits were analyzed using a protein microarray system measuring four classes of immunoglobulins. The frequency of the visits, age and gender distribution reflected real situation faced by the clinicians at a pediatric reference center for food allergy in 530 Paulo, Brazil. The profiling array results have shown that total IgG and IgA share similar specificity whilst IgM and in particular IgE are distantly related. The correlation of specificity of IgE and IgA is variable amongst the patients and this relationship cannot be used to predict atopy or the onset of tolerance to milk. The array profiling technique has corroborated the clinical selection criteria for this cohort albeit it clearly suggested that 4 out of the 41 patients might have allergies other than milk origin. There was also a good correlation between the array data and ImmunoCAP results, casein in particular. By using qualitative and quantitative multivariate analysis routines it was possible to produce validated statistical models to predict with reasonable accuracy the onset of tolerance to milk proteins. If expanded to larger study groups, the array profiling in combination with the multivariate techniques show potential to improve the prognostic of milk allergic patients. (C) 2012 Elsevier B.V. All rights reserved.

The Effects of Oxybutynin on Urinary Symptoms in Children with Williams-Beuren Syndrome

Purpose: Williams-Beuren syndrome is a genomic disorder caused by a hemizygous contiguous gene deletion on chromosome 7q11.23. Lower urinary tract symptoms are common in children with Williams-Beuren syndrome. However, there are few data on the management of voiding symptoms in this population. We report our experience using oxybutynin to treat urinary symptoms in children with Williams-Beuren syndrome. Materials and Methods: We prospectively analyzed 42 patients with Williams-Beuren syndrome and significant lower urinary tract symptoms due to detrusor overactivity diagnosed on urodynamics in a 12-week, open-label study. Urological assessment included symptomatic evaluation, the impact of lower urinary tract symptoms on quality of life, frequency-volume chart, urodynamics and urinary tract sonography. After 12 weeks of treatment with 0.6 mg/kg oxybutynin per day given in 3 daily doses, patients were assessed for treatment efficacy and side effects. Results: A total of 17 girls and 19 boys completed medical therapy and were assessed at 12 weeks. Mean +/- SD patient age was 9.2 +/- 4.3 years (range 3 to 18). The most common urinary complaint was urgency, which occurred in 31 patients (86.1%), followed by urge incontinence, which was seen in 29 (80.5%). Compared to baseline, urinary symptoms were substantially improved. The negative impact of storage symptoms on quality of life was significantly decreased from a mean +/- SD of 3.3 +/- 1.7 to 0.5 +/- 0.9 (p <0.001). Mean +/- SD maximum urinary flow improved from 14.2 +/- 15.0 to 20.5 +/- 6.4 ml per second (p <0.001). Conclusions: A total of 12 weeks of therapy with 0.6 mg/kg oxybutynin daily resulted in improvement of lower urinary tract symptoms, quality of life and maximum flow rate in most patients with Williams-Beuren syndrome.

The Differential Clinical Impact of Human Coronavirus Species in Children With Cystic Fibrosis

We investigated the clinical impact of human coronaviruses (HCoV) OC43, 229E, HKU1 and NL63 in pediatric patients with cystic fibrosis (CF) during routine and exacerbation visits. A total of 408 nasopharyngeal aspirate samples were obtained from 103 patients over a 1-year period. Samples positive for HCoV were submitted for nucleotide sequencing to determine the species. Nineteen samples (4.65%) were positive for HCoV, of which 8 were positive for NL63, 6 for OC43, 4 for HKU1, and 1 for 229E. Identification of HCoV was not associated with an increased rate of respiratory exacerbations, but NL63-positive patients had higher exacerbation rates than patients who were positive for other HCoV species.

Growth hormone pharmacogenetics: the interactive effect of a microsatellite in the IGF1 promoter region with the GHR-exon 3 and-202 A/C IGFBP3 variants on treatment outcomes of children with severe GH deficiency

Insulin-like growth factor type 1 (IGF1) is a mediator of growth hormone (GH) action, and therefore, IGF1 is a candidate gene for recombinant human GH (rhGH) pharmacogenetics. Lower serum IGF1 levels were found in adults homozygous for 19 cytosine-adenosine (CA) repeats in the IGF1 promoter. The aim of this study was to evaluate the influence of (CA)n IGF1 polymorphism, alone or in combination with GH receptor (GHR)-exon 3 and -202 A/C insulin-like growth factor binding protein-3 (IGFBP3) polymorphisms, on the growth response to rhGH therapy in GH-deficient (GHD) patients. Eighty-four severe GHD patients were genotyped for (CA) n IGF1, -202 A/C IGFBP3 and GHR-exon 3 polymorphisms. Multiple linear regressions were performed to estimate the effect of each genotype, after adjustment for other influential factors. We assessed the influence of genotypes on the first year growth velocity (1st y GV) (n = 84) and adult height standard deviation score (SDS) adjusted for target-height SDS (AH-TH SDS) after rhGH therapy (n = 37). Homozygosity for the IGF1 19CA repeat allele was negatively correlated with 1st y GV (P = 0.03) and AH-TH SDS (P = 0.002) in multiple linear regression analysis. In conjunction with clinical factors, IGF1 and IGFBP3 genotypes explain 29% of the 1st y GV variability, whereas IGF1 and GHR polymorphisms explain 59% of final height-target-height SDS variability. We conclude that homozygosity for IGF1 (CA) 19 allele is associated with less favorable short-and long-term growth outcomes after rhGH treatment in patients with severe GHD. Furthermore, this polymorphism exhibits a non-additive interaction with -202 A/C IGFBP3 genotype on the 1st y GV and with GHR-exon 3 genotype on adult height. The Pharmacogenomics Journal (2012) 12, 439-445; doi:10.1038/tpj.2011.13; published online 5 April 2011

Differences in Evolution of Children with Non-severe Acute Lower Respiratory Tract Infection With and Without Radiographically Diagnosed Pneumonia

Objective: To identify differences in the evolution of children with non-severe acute lower respiratory tract infection between those with and without radiographically diagnosed pneumonia. Design: Prospective cohort study. Setting: A public university pediatric hospital in Salvador, Northeast Brazil. Patients: Children aged 2-59 months. Methods: By active surveillance, the pneumonia cases were prospectively identified in a 2-year period. Each case was followed-up for changes in various clinical symptoms and signs. Demographic, clinical and radiographic data were recorded in standardized forms. Exclusion was due to antibiotic use in the previous 48 hours, signs of severe disease, refusal to give informed consent, underlying chronic illness, hospitalization in the previous 7 days or amoxicillin allergy. Chest X-ray (CXR) was later read by at least 2 independent pediatric radiologists. Main Outcome Measures: Radiographic diagnosed pneumonia based on agreed detection of pulmonary infiltrate or pleural effusion in 2 assessments. Results: A total of 382 patients receiving amoxicillin were studied, of whom, 372 (97.4%) had concordant radiographic diagnosis which was pneumonia (52%), normal CXR (41%). and others (7%). By multivariate analysis, age (OR=1.03; 95% CI: 1.02-1.05), disease >= 5days (OR = 1.04; 95% CI: 1.001-1.08), reduced pulmonary expansion (OR = 3.3; 95% CI: 1.4-8.0), absence of wheezing (OR = 0.5; 95% CI: 0.3-0.9), crackles on admission (OR = 2.0; 95% CI: 1.2-3.5), inability to drink on day 1 (OR = 4.2; 95% CI: 1.05-17.3), consolidation percussion sign (OR = 7.0; 95% CI: 1.5-32.3), tachypnea (OR = 2.0; 95% CI: 1.09-3.6) and fever (OR = 3.6; 95% CI: 1.4-9.4) on day 2 were independently associated with pneumonia. The highest positive predictive value was at the 2nd day of evolution for tachypnea (71.0%) and fever (81.1%). Conclusion: Persistence of fever or tachypnea up to the second day of amoxicillin treatment is predictive of radiographically diagnosed pneumonia among children with non-severe lower respiratory tract diseases.

Clinical correlates of social adjustment in patients with obsessive-compulsive disorder

Background: Patients with obsessive-compulsive disorder (OCD) frequently show poor social adjustment, which has been associated with OCD severity. Little is known about the effects that age at symptom onset, specific OCD symptoms, and psychiatric comorbidities have on social adjustment. The objective of this study was to investigate the clinical correlates of social functioning in OCD patients. Methods: Cross-sectional study involving 815 adults with a primary DSM-IV diagnosis of OCD participating in the Brazilian Research Consortium on Obsessive-Compulsive Spectrum Disorders. Patients were assessed with the Social Adjustment Scale, the Medical Outcomes Study 36-item Short-Form Health Survey, the Yale-Brown Obsessive-Compulsive Scale, the Dimensional Yale-Brown Obsessive-Compulsive Scale, and the Structured Clinical Interview for DSM-IV Axis I Disorders. Clinical correlates of social adjustment were assessed with generalized linear models with gamma distribution. Results: Poor overall social functioning was associated with greater OCD severity (p = 0.02); hoarding symptoms (p = 0.004); sexual/religious obsessions (p = 0.005); current major depressive disorder (p = 0.004); current post-traumatic stress disorder (p = 0.002); and current eating disorders (p = 0.02). Poor social adjustment was also associated with impaired quality of life. Conclusions: Patients with OCD have poor social functioning in domains related to personal relationships and professional performance. Hoarding symptoms and sexual/religious obsessions seem to have the strongest negative effects on social functioning. Early age at OCD symptom onset seems to be associated with professional and academic underachievement and impairment within the family unit, whereas current psychiatric comorbidity worsen overall social functioning. In comparison with quality of life, social adjustment measures seem to provide a more comprehensive overview of the OCD-related burden. (C) 2012 Elsevier Ltd. All rights reserved.