Liver transplantation in children: Experience with the development of an Australian pilot programme. The Queensland Liver Transplantation Programme

Children with end-stage liver disease now form a major sub-group of patients considered suitable for liver transplantation (ltp), and enjoy better survival statistics after transplantation than do adults. Since June 1984, a paediatric ltp programme has been developed in Brisbane with an initial working relationship and ongoing close links with two USA centres (Pittsburgh, and the UCLA Medical Center). Fourteen children with end-stage liver disease have been referred to the Queensland Liver Transplantation Programme for formal assessment. Following frank, informed discussion with their parents, 10 of these children were offered the option of ltp. During the transition stage, two infants with biliary atresia were referred to UCLA at their parents' request and, subsequently, eight children aged from 9 months to 6 years have been placed on a transplant candidacy list in Brisbane. A donor procurement team with access to a Queensland Government jet has been available to cover all mainland States except Western Australia. Six of the children have now had orthotopic ltp (two children at the UCLA Medical Center; four children at the Royal Children's Hospital, Brisbane). One UCLA patient died with a non-functioning graft, and one Brisbane patient died 5 weeks post-transplant with rejection, hepatic artery thrombosis and sepsis. The other four children are alive and well, three with normal liver function and one with unexplained intrahepatic cholestasis, during the 1-20 month follow-up to date. Three further children have died of their liver disease without a donor of an appropriate blood group and size being found, and one patient still awaits a suitable donor. The experience of these authors suggests that ltp is a major advance in the treatment of paediatric liver disease, and that the procedure can be carried out successfully in Australia with initial results comparable with leading overseas centres. The procedure requires the full array of services of a major paediatric tertiary care facility, an intensive team effort with awareness of the special needs of children, and a widespread procurement capability. A major problem for Australia is the procurement of sufficient numbers of optimal paediatric donor livers.

Relative peripheral hyperopia does not predict development and progression of myopia in children

Purpose To test the hypothesis that relative peripheral hyperopia predicts development and progression of myopia. Methods Refraction along the horizontal visual field was measured under cycloplegia at visual field angles of 0°, ±15°, and ±30° at baseline, 1 and 2 years in over 1700 initially 7-year-old Chinese children, and at baseline and 1 year in over 1000 initially 14-year olds. One refraction classification for central refraction was “nonmyopia, myopia” (nM, M), consisting of nM greater than −0.50 diopters (D; spherical equivalent) and M less than or equal to −0.50 D. A second classification was “hyperopia, emmetropia, low myopia, and moderate/high myopia” (H, E, LM, MM) with H greater than or equal to +1.00 D, E, −0.49 to +0.99 D, LM, −2.99 to −0.50 D, and MM less than or equal to −3.00 D. Subclassifications were made on the basis of development and progression of myopia over the 2 years. Changes in central refraction over time were determined for different groups, and relative peripheral refraction over time was compared between different subgroups. Results Simple linear regression of central refraction as a function of relative peripheral refraction did not predict myopia progression as relative peripheral refraction became more hyperopic: relative peripheral hyperopia and relative peripheral myopia predicted significant myopia progression for 0% and 35% of group/visual field angle combinations, respectively. Subgroups who developed myopia did not have more initial relative peripheral hyperopia than subgroups who did not develop myopia. Conclusions Relative peripheral hyperopia does not predict development nor progression of myopia in children. This calls into question the efficacy of treatments that aim to slow progression of myopia in children by “treating” relative peripheral hyperopia.

A systematic review of instruments for assessment of capacity in activities of daily living in children with developmental co-ordination disorder

Children with developmental co-ordination disorder (DCD) face evident motor difficulties in activities of daily living (ADL). Assessment of their capacity in ADL is essential for diagnosis and intervention, in order to limit the daily consequences of the disorder. The aim of this study is to systematically review potential instruments for standardized and objective assessment of children's capacity in ADL, suited for children with DCD. As a first step, databases of MEDLINE, EMBASE, CINAHL and PsycINFO were searched to identify studies that described instruments with potential for assessment of capacity in ADL. Second, instruments were included for review when two independent reviewers agreed that the instruments: (1) are standardized and objective; (2) assess at activity level and comprise items that reflect ADL, and; (3) are applicable to school-aged children that can move independently. Out of 1507 publications, 66 publications were selected, describing 39 instruments. Seven of these instruments were found to fulfil the criteria and were included for review: the Bruininks-Oseretsky Test of Motor Performance-2 (BOT2); the Do-Eat (Do-Eat); the Movement Assessment Battery for Children-2 (MABC2); the school-Assessment of Motor and Process Skills (schoolAMPS); the Tuffts Assessment of Motor Performance (TAMP); the Test of Gross Motor Development (TGMD); and the Functional Independence Measure for Children (WeeFIM). As a third step, for the included instruments, suitability for children with DCD was discussed based on the ADL comprised, ecological validity and other psychometric properties. We concluded that current instruments do not provide comprehensive and ecologically valid assessment of capacity in ADL as required for children with DCD.

Activities of daily living in children with developmental coordination disorder: Performance, learning, and participation

Background Children with developmental coordination disorder (DCD) face evident motor difficulties in daily functioning. Little is known, however, about their difficulties in specific activities of daily living (ADL). Objective The purposes of this study were: (1) to investigate differences between children with DCD and their peers with typical development for ADL performance, learning, and participation, and (2) to explore the predictive values of these aspects. Design. This was a cross-sectional study. Methods In both a clinical sample of children diagnosed with DCD (n=25 [21 male, 4 female], age range=5-8 years) and a group of peers with typical development (25 matched controls), the children’s parents completed the DCDDaily-Q. Differences in scores between the groups were investigated using t tests for performance and participation and Pearson chi-square analysis for learning. Multiple regression analyses were performed to explore the predictive values of performance, learning, and participation. Results Compared with their peers, children with DCD showed poor performance of ADL and less frequent participation in some ADL. Children with DCD demonstrated heterogeneous patterns of performance (poor in 10%-80% of the items) and learning (delayed in 0%-100% of the items). In the DCD group, delays in learning of ADL were a predictor for poor performance of ADL, and poor performance of ADL was a predictor for less frequent participation in ADL compared with the control group. Limitations A limited number of children with DCD were addressed in this study. Conclusions This study highlights the impact of DCD on children’s daily lives and the need for tailored intervention.

Development and psychometric properties of the DCDDaily: a new test for clinical assessment of capacity in activities of daily living in children with developmental coordination disorder

Objective To develop the DCDDaily, an instrument for objective and standardized clinical assessment of capacity in activities of daily living (ADL) in children with developmental coordination disorder (DCD), and to investigate its usability, reliability, and validity. Subjects Five to eight-year-old children with and without DCD. Main measures The DCDDaily was developed based on thorough review of the literature and extensive expert involvement. To investigate the usability (assessment time and feasibility), reliability (internal consistency and repeatability), and validity (concurrent and discriminant validity) of the DCDDaily, children were assessed with the DCDDaily and the Movement Assessment Battery for Children-2 Test, and their parents filled in the Movement Assessment Battery for Children-2 Checklist and Developmental Coordination Disorder Questionnaire. Results 459 children were assessed (DCD group, n = 55; normative reference group, n = 404). Assessment was possible within 30 minutes and in any clinical setting. For internal consistency, Cronbach’s α = 0.83. Intraclass correlation = 0.87 for test–retest reliability and 0.89 for inter-rater reliability. Concurrent correlations with Movement Assessment Battery for Children-2 Test and questionnaires were ρ = −0.494, 0.239, and −0.284, p < 0.001. Discriminant validity measures showed significantly worse performance in the DCD group than in the control group (mean (SD) score 33 (5.6) versus 26 (4.3), p < 0.001). The area under curve characteristic = 0.872, sensitivity and specificity were 80%. Conclusions The DCDDaily is a valid and reliable instrument for clinical assessment of capacity in ADL, that is feasible for use in clinical practice.

Positive associations of serum Perfluoroalkyl substances with uric acid and hyperuricemia in children from Taiwan

To investigate the risk of hyperuricemia in relation to Perfluoroalkyl substances (PFASs) in children from Taiwan, 225 Taiwanese children aged 12-15 years were recruited from 2009 to 2010. Linear and logistic regression models were employed to examine the influence of PFASs on serum uric acid levels. Findings revealed that eight of ten PFASs analyses were detected in > 94% of the participants' serum samples. Multivariate linear regression models revealed that perfluorooctanic acid (PFOA) was positively associated with serum uric acid levels (β=0.1463, p<0.05). Of all the PFASs analyses, only PFOA showed a significant effect on elevated levels of hyperuricemia (aOR=2.16, 95%CI: 1.29-3.61). When stratified by gender, the association between serum PFOA and uric acid levels was only evident among boys (aOR=2.76, 95%CI: 1.37-5.56). In conclusion, PFOA was found to be associated with elevated serum levels of uric acid in Taiwanese children, especially boys. Further research is needed to elucidate these links.

Randomised controlled trial of three burns dressings for partial thickness burns in children

BACKGROUND This study compared the effects of three silver dressing combinations on small to medium size acute partial thickness burns in children, focusing on re-epithelialization time, pain and distress during dressing changes. METHOD Children (0-15 years) with clean, ≤ 10% total body surface area (TBSA) partial thickness burns who met the inclusion criteria were included in the study. Children received either (1) Acticoat™; (2) Acticoat™ with Mepitel™; or (3) Mepilex Ag™ dressings. Measures of burn re-epithelialization, pain, and distress were recorded at dressing changes every 3-5 days until full re-epithelialization occurred. RESULTS One hundred and three children were recruited with 96 children included for analysis. No infections were detected for the course of the study. When adjusted for burn depth, Acticoat™ significantly increased the expected days to full re-epithelialization by 40% (IRR = 1.40; 95% CI: 1.14-1.73, p < 0.01) and Acticoat™ with Mepitel™ significantly increased the expected days to full re-epithelialization by 33% (IRR = 1.33; 95% CI: 1.08-1.63, p ≤ 0.01) when compared to Mepilex Ag™. Expected FLACC scores in the Mepilex Ag™ group were 32% lower at dressing removal (p = 0.01) and 37% lower at new dressing application (p = 0.04); and scores in the Acticoat™ with Mepitel™ group were 23% lower at dressing removal (p = 0.04) and 40% lower at new dressing application (p < 0.01), in comparison to the Acticoat™ group. Expected Visual Analog Scale-Pain (VAS-P) scores were 25% lower in the Mepilex Ag™ group at dressing removal (p = 0.04) and 34% lower in the Acticoat™ with Mepitel™ group (p = 0.02) at new dressing application in comparison to the Acticoat™ group. There was no significant difference between the Mepilex Ag™ and the Acticoat™ with Mepitel™ groups at all timepoints and with any pain measure. CONCLUSION Mepilex Ag™ is an effective silver dressing, in terms of accelerated wound re-epithelialization time (compared to Acticoat™ and Acticoat™ with Mepitel™) and decreased pain during dressing changes (compared to Acticoat™), for clean, < 10% TBSA partial thickness burns in children.

Associations of serum perfluoroalkyl acid levels with T-helper cell-specific cytokines in children: By gender and asthma status

Perfluoroalkyl acids (PFAAs) are a group of common chemicals that ubiquitously exist in wildlife and humans. Experimental data suggest that they may alter T-lymphocyte functioning in situ by preferentially enhancing the development of T-helper 2 (TH2)- and inhibiting TH1-lymphocyte development and might increase allergic inflammation, but few human studies have been conducted. To evaluate the association between serum PFAAs concentrations and T-lymphocyte-related immunological markers of asthma in children, and further to assess whether gender modified this association, 231 asthmatic children and 225 non-asthmatic control children from Northern Taiwan were recruited into the Genetic and Biomarker study for Childhood Asthma. Serum concentrations of ten PFAAs and levels of TH1 [interferon (IFN)-γ, interleukin (IL)-2] and TH2 (IL-4 and IL-5) cytokines were measured. The results showed that asthmatics had significantly higher serum PFAAs concentrations compared with the healthy controls. When stratified by gender, a greater number of significant associations between PFAAs and asthma outcomeswere found in males than in females. Among males, adjusted odds ratios for asthma among those with the highest versus lowest quartile of PFAAs exposure ranged from 2.59 (95% CI: 1.14, 5.87) for the perfluorobutanesulfonate (PFBS) to 4.38 (95% CI: 2.02, 9.50) for perfluorooctanesulfonate (PFOS); and serum PFAAs were associated positively with TH2 cytokines and inversely with TH1 cytokines among male asthmatics. Among females, no significant associations between PFAAs and TH2 cytokines could be detected. In conclusion, increased serum PFAAs levels may promote TH cell dysregulation and alter the availability of key TH1 and TH2 cytokines, ultimately contributing to the development of asthma that may differentially impact males to a greater degree than females. These results have potential relevance in asthma prevention.

Protocol for assessing maternal, environmental and epigenetic risk factors for dental caries in children

Background Expenditure on dental and oral health services in Australia is $3.4 billion AUD annually. This is the sixth highest health cost and accounts for 7 % of total national health expenditure. Approximately 49 % of Australian children aged 6 years have caries experience in their deciduous teeth and this is rising. The aetiology of dental caries involves a complex interplay of individual, behavioural, social, economic, political and environmental conditions, and there is increasing interest in genetic predisposition and epigenetic modification. Methods The Oral Health Sub-study; a cross sectional study of a birth cohort began in November 2012 by examining mothers and their children who were six years old by the time of initiation of the study, which is ongoing. Data from detailed questionnaires of families from birth onwards and data on mothers’ knowledge, attitudes and practices towards oral health collected at the time of clinical examination are used. Subjects’ height, weight and mid-waist circumference are taken and Body Mass Index (BMI) computed, using an electronic Bio-Impedance balance. Dental caries experience is scored using the International Caries Detection and Assessment System (ICDAS). Saliva is collected for physiological measures. Salivary Deoxyribose Nucleic Acid (DNA) is extracted for genetic studies including epigenetics using the SeqCap Epi Enrichment Kit. Targets of interest are being confirmed by pyrosequencing to identify potential epigenetic markers of caries risk. Discussion This study will examine a wide range of potential determinants for childhood dental caries and evaluate inter-relationships amongst them. The findings will provide an evidence base to plan and implement improved preventive strategies.

Swallowing sounds in children: Establishing normative data

Limited data on cervical auscultation (CA) sounds in normal swallows of various food and fluid textures during the transitional feeding period of 4-36 months exists. This study documents the acoustic and perceptual parameters of swallowing sounds in healthy children aged 4–36 months over a range of food and fluid consistencies.

Is there a difference in sound acoustics between aspirating versus non-aspirating swallows in children with dysphagia?

Cervical auscultation (CA) may be used to complement the clinical feeding examination when assessing for oropharyngeal aspiration (OPA). Data exists on the acoustic properties of normal and abnormal swallowing sounds in adults and children. However, there are no published paediatric studies comparing the acoustic properties of sounds comparing OPA with non-OPA swallows. We aimed to determine if there is an acoustic difference between modified barium swallow (MBS)-identified OPA and non-OPA swallow sounds in children.

Prevalence, codetection and seasonal distribution of upper airway viruses and bacteria in children with acute respiratory illnesses with cough as a symptom

Most studies exploring the role of upper airway viruses and bacteria in paediatric acute respiratory infections (ARI) focus on specific clinicaldiagnoses and/or do not account for virus–bacteria interactions. We aimed to describe the frequency and predictors of virus and bacteria codetection in children with ARI and cough, irrespective of clinical diagnosis. Bilateral nasal swabs, demographic, clinical and risk factor data were collected at enrollment in children aged <15 years presenting to an emergency department with an ARI and where cough was a symptom. Swabs were tested by polymerase chain reaction for 17 respiratory viruses and seven respiratory bacteria. Logistic regression was used to investigate associations between child characteristics and codetection of the organisms of interest. Between December 2011 and August 2014, swabs were collected from 817 (93.3%) of 876 enrolled children, median age 27.7 months (interquartile range13.9–60.3 months). Overall, 740 (90.6%) of 817 specimens were positive for any organism. Both viruses and bacteria were detected in 423 specimens (51.8%). Factors associated with codetection were age (adjusted odds ratio (aOR) for age <12 months = 4.9, 95% confidence interval (CI) 3.0, 7.9; age 12 to <24 months = 6.0, 95% CI 3.7, 9.8; age 24 to <60 months = 2.4, 95% CI 1.5, 3.9), male gender (aOR 1.46; 95% CI 1.1, 2.0), child care attendance (aOR 2.0; 95% CI 1.4, 2.8) and winter enrollment (aOR 2.0; 95% CI 1.3, 3.0). Haemophilus influenzae dominated the virus–bacteria pairs. Virus–H. influenzae interactions in ARI should be investigated further, especially as the contribution of nontypeable H. influenzae to acute and chronic respiratory diseases is being increasingly recognized.

Appendiceal sonography in children: A retrospective analysis as a platform for potential implementation of diagnostic categories

- Background Sonography is an important diagnostic tool in children with suspected appendicitis. Reported accuracy and appendiceal visualisation rates vary significantly, as does the management of equivocal ultrasound findings. The aim of this study was to audit appendiceal sonography at a tertiary children's hospital, and provide baseline data for a future prospective study. - Summary of work Records of children who underwent ultrasound studies for possible appendicitis between January 2008 and December 2010 were reviewed. Variables included patient demographics, sonographic appendix characteristics, and secondary signs. Descriptive statistics and analysis using ANOVA, Mann-Whitney U test, and ROC curves were performed. Mater Human Research Ethic Committee approval was granted. - Summary of results There were 457 eligible children. Using a dichotomous diagnostic model (including equivocal results), sensitivity was 89.6%, specificity 91.6%, and diagnostic yield of 40.7%. ROC curve analysis of a 6mm diameter cut-off was 0.88 AUC (95% CI 0.80 to 0.95). - Discussion and conclusions Sonography is an accurate test for acute appendicitis in children, with a high sensitivity and negative predictive value. A diameter of 6mm as an absolute cut-off in a binary model can lead to false findings. Results were compared with available literature. Recent publications propose categorising diameter1 and integrating secondary signs2 to improve accuracy and provide more meaningful results to clinicians. This study will be a benchmark for future studies with multiple diagnostic categorisation.