Nanoduct(R) sweat testing for rapid diagnosis in newborns, infants and children with cystic fibrosis

Determination of chloride concentration in sweat is the current diagnostic gold standard for Cystic Fibrosis (CF). Nanoduct(R) is a new analyzing system measuring conductivity which requires only 3 microliters of sweat and gives results within 30 minutes. The aim of the study was to evaluate the applicability of this system in a clinical setting of three children's hospitals and borderline results were compared with sweat chloride concentration. Over 3 years, 1,041 subjects were tested and in 946 diagnostic results were obtained. In 95 children, Nanoduct(R) failed (9.1% failure rate), mainly due to failures in preterm babies and newborns. Assuming 59 mmol/L as an upper limit of normal conductivity, all our 46 CF patients were correctly diagnosed (sensitivity 100%, 95% CI: 93.1-100; negative predicted value 100% (95% CI: 99.6-100) and only 39 non CF's were false positive (39/900, 4.3%; specificity 95.7%, 95%CI: 94.2-96.9, positive predicted value 54.1% with a 95%CI: 43.4-65.0). Increasing the diagnostic limit to 80 mmol/L, the rate fell to 0.3% (3/900). CF patients had a median conductivity of 115 mmol/L; the non-CF a median of 37 mmol/L. In conclusion, the Nanoduct(R) test is a reliable diagnostic tool for CF diagnosis: It has a failure rate comparable to other sweat tests and can be used as a simple bedside test for fast and reliable exclusion, diagnosis or suspicion of CF. In cases with borderline conductivity (60-80 mmol/L) other additional methods (determination of chloride and genotyping) are indicated.

[Localised rhabdomyosarcoma of the extremities in children and adolescents: results of the French experience]

PATIENTS AND METHODS: Forty-six patients with localised RMS of the limbs entered the MMT 89 and 95 study in France. We studied potential risk factors that were predictive of relapse and survival to propose a therapeutic approach of surgery and radiotherapy appropriate to the risk of relapse. RESULTS: Median age at diagnosis was 6.5 years [9 months to 15.5 years]. At time of diagnosis, 43% had marginal surgery and only 13% radical intervention. Primary re-excision was performed in 12% of the patients. All patients received chemotherapy, 43% had second look surgery and 37% received radiotherapy. Fifty-four percent of all tumors relapsed: local relapse 36%, nodes l8%, metastatic 40%, local and metastatic 16%. Estimated overall 5-year event-free survival (EFS) and overall survival (OS) were 40 and 57%, respectively. CONCLUSIONS: Prognosis of RMS of the limbs is bad but only 37% of the patients had radiotherapy. We could define patients with very high risk among those with limbs RMS as nodal involvement (5 years overall survival OS 22%), alveolar histology (OS 38%) and site of hand and foot (4 survivors out of 10 patients). In further studies, these patients should be treated even more aggressive with early surgery followed by re-excision if necessary, chemotherapy including alkylating agents and systematic radiotherapy.

Time required to obtain endobronchial biopsies in children during fiberoptic bronchoscopy

BACKGROUND: Endobronchial biopsies are an important tool for the study of airway remodeling in children. We aimed to evaluate the impact of performing endobronchial biopsies as a part of fiberoptic bronchoscopy on the length of the procedure. METHODS: Clinically indicated fiberoptic bronchoscopy at which endobronchial biopsy was attempted as a part of a research protocol was performed in 40 children (median age 6 years, range 2 months-16 years). Time needed for airway inspection, bronchoalveolar lavage (BAL) with three aliquots of 1 ml/kg of 0.9% saline, sampling of three macroscopically adequate biopsies, teaching, and other interventions (e.g., removal of plugs) was recorded. The bronchoscopist was not aware that the procedure was being timed. RESULTS: Median (range) duration (min) was 2.5 (1.0-8.2) for airway inspection, 2.8 (1.7-9.4) for BAL, 5.3 (2.5-16.6) for biopsy sampling, 2.4 (1.5-6.6) for teaching and 4.1 (0.8-18.5) for other interventions. Three adequate biopsies were obtained in 33 (83%) children. Use of 2.0 mm biopsy forceps (via 4.0 and 4.9 mm bronchoscopes) rather than 1.0 mm (via 2.8 and 3.6 mm bronchoscopes) significantly reduced biopsy time (4.6 min vs. 8.4 min, P < 0.001). CONCLUSIONS: It takes a median of just over 5 min to obtain three endobronchial biopsies in children, which we consider an acceptable increase in the duration of fiberoptic bronchoscopy for the purpose of research.

Changing trends in causes and patterns of facial fractures in children

OBJECTIVE: To review the epidemiology of facial fractures in children and to analyze whether it has changed over time. STUDY DESIGN: Retrospective review of records of children aged < or = 15 years diagnosed for fracture during 2 10-year periods. RESULTS: A total of 378 children were diagnosed with fractures, 187 in 1980-1989 and 191 in 1993-2002. The proportion of children with mandibular fractures decreased by 13.6 percentage-points from the first period to the second, whereas the proportion of patients with midfacial fractures increased by 18.7 percentage-points. Assault as a causative factor increased by 5.5 percentage-points, almost exclusively among children aged 13-15 years, with a high percentage (23.5%). CONCLUSIONS: Recognition of a change in fracture patterns over time is probably due to the increased use of computerized tomographic scanning.

Nasolabial esthetics in children with complete unilateral cleft lip and palate after 1- versus 3-stage treatment protocols

PURPOSE: Facial esthetics play an important role in social interactions. However, children with a repaired complete unilateral cleft lip and palate usually show some disfigurement of the nasolabial area. To date, few studies have assessed the nasolabial appearance after different treatment protocols. The aim of the present study was to compare the nasolabial esthetics after 1- and 3-stage treatment protocols. MATERIALS AND METHODS: Four components of the nasolabial appearance (nasal form, nasal deviation, mucocutaneous junction, and profile view) were assessed by 4 raters in 108 consecutively treated children who had undergone either 1-stage closure (Warsaw group, 41 boys and 19 girls, mean age 10.8 years, SD 2.0) or 3-stage (Nijmegen group, 30 boys and 18 girls, mean age 8.9 years, SD 0.7). A 5-grade esthetic index of Asher-McDade was used, in which grade 1 indicates the most esthetic and grade 5 the least esthetic outcome. RESULTS: The nasal form was judged the least esthetic in both groups and graded 3.1 (SD 1.1) and 3.2 (SD 1.1). The nasal deviation, mucocutaneous junction, and profile view were scored from 2.1 (SD 0.8) to 2.3 (SD 1.0) in both groups. The treatment outcome after the Warsaw and Nijmegen protocols was comparable. Neither overall nor any of the 4 components of the nasolabial appearance showed intercenter differences (P > .1). CONCLUSIONS: The nasolabial appearance after the Warsaw (1-stage) and Nijmegen (3-stage) protocols was comparable. The technique of lip repair (triangular flap in Warsaw and Millard rotation advancement in Nijmegen) gave comparable results for the esthetics of the nasolabial area.

Comparison of the immediate effects of gaseous ozone and chlorhexidine gel on bacteria in cavitated carious lesions in children in vivo

Clinical application of ozone gas has been shown to arrest the progression of dentinal caries in children. In this study, we compare the immediate effects of gaseous ozone and chlorhexidine gel on bacteria in cavitated carious lesions in children. Forty children, each with at least two open occlusal carious lesions, were enrolled in the study. Two teeth were chosen randomly. In one lesion, overlying soft biological material was removed, whilst the other lesion was not excavated. Cavities were rinsed with sterile water and dried with air. A standardised sample was taken from the mesial part of each lesion. Then, gaseous ozone (HealOzone) or 1% chlorhexidine gel (Corsodyl) was applied for 30 s on both lesions of 20 children each, and a second sample was taken from the distal part of each lesion. The anaerobic microbiota was cultivated; the number of colony forming units was calculated per milligram sample. The two-sided paired t test showed no significant (P > 0.05) differences in the reduction of total bacterial counts per milligram comparing samples before and after ozone or chlorhexidine application. The tests also showed no statistically significant difference whether the superficial decayed dentine had been removed before ozone or with chlorhexidine treatment or not. It can be concluded that gaseous ozone or chlorhexidine gel application for 30 s to deep occlusal carious cavities had no significant immediate antimicrobial effects whether the superficial decayed layers dentine were removed or not.

Comparative study of the neuropeptide-Y sympathetic nerves in endometriotic involved and noninvolved sacrouterine ligaments in women with pelvic endometriosis

STUDY OBJECTIVE: To show the relationship between the neuropeptide-Y pelvic sympathetic nerves and neoangiogenesis in the development of endometriosis DESIGN: Prospective study. SETTING: Academic community teaching hospital. PATIENTS: Fifteen consecutive women with unilateral endometriotic infiltration of the sacrouterine ligaments. INTERVENTIONS: A laparoscopic excision/biopsy of involved and noninvolved parts of the sacrouterine ligaments were taken. The sections were incubated with the neuronal marker rabbit polyclonal anti-protein gene product 9.5 and rabbit polyclonal anti-neuropeptide-Y. We made a comparative study on the distribution of nerve fibers and their relationship to the vessels on intact and endometriotic involved tissue. MEASUREMENTS AND MAIN RESULTS: The results show that a large amount of nerves are present around the blood vessels in the endometriosis samples, and a large number of these nerves are neuropeptide-Y sympathetic nerves. Adrenergic fibers are also present in the intact control subjects, however, in significantly smaller amounts. CONCLUSION: This finding shows a strong relationship between the neuropeptide-Y sympathetic pelvic nerves and the neoangiogenesis required for the development of endometriosis.

Growth curves for cardio-metabolic risk factors in children and adolescents

OBJECTIVE: This study developed percentile curves for anthropometric (waist circumference) and cardiovascular (lipid profile) risk factors for US children and adolescents. STUDY DESIGN: A representative sample of US children and adolescents from the National Health and Nutrition Examination Survey from 1988 to 1994 (NHANES III) and the current national series (NHANES 1999-2006) were combined. Percentile curves were constructed, nationally weighted, and smoothed using the Lambda, Mu, and Sigma method. The percentile curves included age- and sex-specific percentile values that correspond with and transition into the adult abnormal cut-off values for each of these anthropometric and cardiovascular components. To increase the sample size, a second series of percentile curves was also created from the combination of the 2 NHANES databases, along with cross-sectional data from the Bogalusa Heart Study, the Muscatine Study, the Fels Longitudinal Study and the Princeton Lipid Research Clinics Study. RESULTS: These analyses resulted in a series of growth curves for waist circumference, total cholesterol, LDL cholesterol, triglycerides, and HDL cholesterol from a combination of pediatric data sets. The cut-off for abnormal waist circumference in adult males (102 cm) was equivalent to the 94(th) percentile line in 18-year-olds, and the cut-off in adult females (88 cm) was equivalent to the 84(th) percentile line in 18-year-olds. Triglycerides were found to have a bimodal pattern among females, with an initial peak at age 11 and a second at age 20; the curve for males increased steadily with age. The HDL curve for females was relatively flat, but the male curve declined starting at age 9 years. Similar curves for total and LDL cholesterol were constructed for both males and females. When data from the additional child studies were added to the national data, there was little difference in their patterns or rates of change from year to year. CONCLUSIONS: These curves represent waist and lipid percentiles for US children and adolescents, with identification of values that transition to adult abnormalities. They could be used conditionally for both epidemiological and possibly clinical applications, although they need to be validated against longitudinal data.

Anterior Cruciate Ligament Injuries in Children and Adolescents: A Review of the Recent Literature

In contrast to the treatment of avulsion lesions of the anterior cruciate ligament (ACL) the management of intrasubstance ACL tears in the skeletally immature patient remains controversial. Prospective studies could show that conservative treatment results in severe instability with concomitant intraarticular damage and poor function of the knee. Reconstruction of a torn ACL always carries the risk of damaging the open growth plates; with consecutively affecting the longitudinal or axial growth of the lower extremity either on the femoral or the tibial side. Thus, several surgical procedures are available to prevent adverse events mentioned above. The purpose of this study is to review the recent literature regarding the treatment algorithm for ACL injuries in skeletally immature patients. This review will (1) investigate the indications for ACL surgery in children; (2) determine if a surgical procedure is clinically superior in skeletally immature patients; and (3) correlate the adverse events with the surgical technique.

Facial esthetics in children with unilateral cleft lip and palate 3 years after alveolar bonegrafting combined with rhinoplasty between 2 and 4 years of age

OBJECTIVES To evaluate facial esthetics in patients with unilateral cleft lip and palate (UCLP) after alveolar bone grafting combined with rhinoplasty between 2 and 4 years of age. DESIGN Retrospective case-control study. SETTING The Department of Pediatric Surgery, Institute of Mother and Child, Warsaw, Poland. MATERIAL AND METHODS Photographs of full faces and cropped images of five nasolabial components: nasal deviation, nasal form, nasal profile, vermillion border, and inferior view were assessed by 5 professional and 14 layraters in 29 children (23 boys and 6 girls; mean age = 5.3 years, SD 0.5; Early-grafted group) and 30 children (20 boys and 10 girls; mean age = 5.5 years, SD 1.0; Non-grafted group) with complete unilateral cleft lip and palate repaired with a one-stage closure. The groups differed regarding the timing of alveolar bone grafting: in the Early-grafted group, alveolar bone grafting in combination with rhinoplasty (ABG-R) was performed between 2 and 4 years of age (mean age = 2.3 years; SD 0.6); in the Non-grafted group, the alveolar defect was grafted after 9 years of age. No primary nose correction was carried out in any group. To rate esthetics, a modified five-grade esthetic index of Asher-McDade was used, where grade 1 means the most esthetic and grade 5 - the least esthetic outcome. RESULTS Esthetics of full faces and of all nasolabial elements in the Early-grafted group was significantly better than in Non-grafted group. The scores in the Early-grafted group ranged from 2.30 to 2.66 points, whereas in the Non-grafted group ranged from 2.66 to 3.17 points. All intergroup differences were statistically significant (p < 0.05). CONCLUSIONS Three years post-operatively, early alveolar bone grafting combined with rhinoplasty is favorable for facial esthetics in children with UCLP, but a longer follow-up is needed to assess whether the improvement was permanent.

Mid-term results of transphyseal anterior cruciate ligament reconstruction in children and adolescents

BACKGROUND Optimal therapy for anterior cruciate ligament (ACL) rupture in the paediatric population still provokes controversy. Although conservative and operative treatments are both applied, operative therapy is slightly favored. Among available surgical techniques are physeal-sparing reconstruction and transphyseal graft fixation. The aim of this study was to present our mid-term results after transphyseal ACL reconstruction. METHODS Fifteen young patients (mean age=12.8±2.6, range=6.2-15.8years, Tanner stage=2-4) with open physis and traumatic anterior cruciate rupture who had undergone transphyseal ACL reconstruction with unilateral quadriceps tendon graft were prospectively analyzed. All children were submitted to radiological evaluation to determine the presence of clearly open growth plates in both the distal femur and proximal tibia. Postoperatively, all patients were treated according to a standardized rehabilitation protocol and evaluated by radiographic analysis and the Lysholm-Gillquist and IKDC 2000 scores. Their health-related quality of life was measured using the SF-12 PCS (physical component summary) and MCS (mental component summary) questionnaires. RESULTS Mean postoperative follow-up was 4.1years. Mean Lysholm-Gillquist score was 94.0. Thirteen of the 15 knees were considered nearly normal on the IKDC 2000 score. The mean SF-12 questionnaire score was 54.0±4.8 for SF-12 PCS and 59.1±3.7 for SF-12 MCS. No reruptures were observed. Radiological analysis detected one knee with valgus deformity. All patients had a normal gait pattern without restrictions. CONCLUSION Transphyseal reconstruction of the anterior cruciate ligament shows satisfactory mid-term results in the immature patient.

Association of congenital, diffuse electrical disease in children with normal heart: sick sinus syndrome, intraventricular conduction block, and monomorphic ventricular tachycardia.

INTRODUCTION Rhythm disturbances in children with structurally normal hearts are usually associated with abnormalities in cardiac ion channels. The phenotypic expression of these abnormalities ("channelopathies") includes: long and short QT syndromes, Brugada syndrome, congenital sick sinus syndrome, catecholaminergic polymorphic ventricular tachycardia, Lènegre-Lev disease, and/or different degrees of cardiac conduction disease. METHODS The study group consisted of three male patients with sick sinus syndrome, intraventricular conduction disease, and monomorphic sustained ventricular tachycardia. Clinical data and results of electrocardiography, Holter monitoring, electrophysiology, and echocardiography are described. RESULTS In all patients, the ECG during sinus rhythm showed right bundle branch block and long QT intervals. First-degree AV block was documented in two subjects, and J point elevation in one. A pacemaker was implanted in all cases due to symptomatic bradycardia (sick sinus syndrome). Atrial tachyarryhthmias were observed in two patients. The common characteristic ventricular arrhythmia was a monomorphic sustained ventricular tachycardia, inducible with ventricular stimulation and sensitive to lidocaine. In one patient, radiofrequency catheter ablation was successfully performed. No structural abnormalities were found in echocardiography in the study group. CONCLUSION Common clinical and ECG features suggest a common pathophysiology in this group of patients with congenital severe electrical disease.

Insulin increases serum leptin concentrations in children and adolescents with newly diagnosed type I diabetes mellitus with and without ketoacidosis.

AIMS/HYPOTHESIS The aims of this study were to analyse the changes of serum leptin in newly diagnosed children and adolescents with Type I (insulin-dependent) diabetes mellitus after insulin treatment and to examine the possible impact of ketoacidosis on these changes. METHODS Baseline serum leptin concentrations were measured in 28 newly diagnosed Type I diabetic patients [age 8.75 +/- 4.05 years (means +/- SD); BMI 15.79 +/- 2.47 kg/m(2); HbA(1 c) 11.3 +/- 1.9 %] with (n = 18) and without (n = 10) ketoacidosis before commencement of insulin treatment, at the time of diagnosis. Thereafter, during a 4-day course of continuous intravenous insulin injection to gain and maintain euglycaemia, serum leptin concentrations were assessed. RESULTS Baseline serum leptin concentrations, adjusted to age, BMI, sex and pubertal stage, differed among these patients. There was, however, an increase of leptin in all subjects from 1.37 +/- 0.56 ng/ml (mean +/- SD) up to 2.97 +/- 1.52 ng/ml by 117 % (p < 0.0001) after insulin therapy. On average, peak serum leptin concentration was obtained after 42 h of insulin treatment. Further, there was no difference in the mean increase of serum leptin concentrations in the two groups, namely with and without ketoadicosis, of insulin-dependent diabetic children and adolescents. In addition, there was no correlation between serum leptin concentrations and correction of ketoacidosis during insulin treatment. CONCLUSIONS/INTERPRETATION Insulin increases serum leptin, within 1 day, in children and adolescents with newly diagnosed Type I diabetes. Ketoacidosis does not influence this interaction between insulin and leptin.

Children's and adolescent's self - assessment of metabolic control versus professional judgment: a cross-sectional retrospective and prospective cohort study

BACKGROUND Morbidity and mortality in T1DM depend on metabolic control, which is assessed by HbA1c measurements every 3-4 months. Patients' self-perception of glycemic control depends on daily blood glucose monitoring. Little is known about the congruence of patients' and professionals' perception of metabolic control in T1DM. OBJECTIVE To assess the actual patients' self-perception and objective assessment (HbA1c) of metabolic control in T1DM children and adolescents and to investigate the possible factors involved in any difference. METHODS Patients with T1DM aged 8 - 18 years were recruited in a cross-sectional, retrospective and prospective cohort study. Data collection consisted of clinical details, measured HbA1c, self-monitored blood glucose values and questionnaires assessing self and professionals' judgment of metabolic control. RESULTS 91 patients participated. Mean HbA1c was 8.03%. HbA1c was higher in patients with a diabetes duration > 2 years (p = 0.025) and in patients of lower socioeconomic level (p = 0.032). No significant correlation was found for self-perception of metabolic control in well and poorly controlled patients. We found a trend towards false-positive memory of the last HbA1c in patients with a HbA1c > 8.5% (p = 0.069) but no difference in patients' knowledge on target HbA1c between well and poorly controlled patients. CONCLUSIONS T1DM patients are aware of a target HbA1c representing good metabolic control. Ill controlled patients appear to have a poorer recollection of their HbA1c. Self-perception of actual metabolic control is similar in well and poorly controlled T1DM children and adolescents. Therefore, professionals should pay special attention that ill controlled T1DM patients perceive their HbA1c correctly.

Physical Activity in Latino Children: Research and Its Implications

Childhood obesity affects children across all ages and genders. However, Latino children and adolescents are at an increased risk, with one out of three Latino children (ages 2-19) being classified as overweight. Physical inactivity is deemed a major factor contributing to the energy imbalance that leads to excess adiposity. The aims of this study are twofold: 1) to present relevant research regarding Latino children’s physical patterns, influences on their physical activity, and interventions designed to promote physical activity and fitness in this population; and 2) to discuss implications derived from this research to help health educators, practitioners, and policy makers increase awareness, and to motivate and enable Latino children to adopt an active lifestyle. Research reveals that Latino children and adolescents are consistently less active than their white counterparts. Latino girls are, in particular, at an increased risk for inactivity. Few studies have investigated the factors that contribute to low levels of physical activity among Latino children. Moreover, few physical activity interventions have involved Latino children. Some of our recent research studies have filled some gaps, including providing information on what physical activities Latino children like, what they intend to do, what they are actually doing, and where and when they do physical activity. Based on our research and review of related literature, we made specific physical activity recommendations for researchers, practitioners, and policy makers. These individual points should be applied and integrated within a broad framework and used in combinations to develop multi-component, coordinated approaches to enhancing physical activity among Latino youth.