Nomenclature and Databases — The Past, the Present, and the Future

This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for congenital heart disease. Five areas will be reviewed: (1) common language = nomenclature, (2) mechanism of data collection (database or registry) with an established uniform core data set, (3) mechanism of evaluating case complexity, (4) mechanism to ensure and verify data completeness and accuracy, and (5) collaboration between medical subspecialties. During the 1990s, both the Society of Thoracic Surgeons (STS) and the European Association for Cardiothoracic Surgery (EACTS) created congenital heart surgery outcomes databases. Beginning in 1998, the EACTS and STS collaborated in the work of the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common congenital heart surgery nomenclature, along with a common core minimal data set, were adopted by the EACTS and the STS and published in the Annals of Thoracic Surgery. In 2000, the International Nomenclature Committee for Pediatric and Congenital Heart Disease was established; this committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD). The working component of ISNPCHD is the International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group (NWG). By 2005, the NWG cross-mapped the EACTS–STS nomenclature with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology and created the International Paediatric and Congenital Cardiac Code (IPCCC) (). This common nomenclature (IPCCC), and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both EACTS and STS; since 1998, this nomenclature and database have been used by both the STS and EACTS to analyze outcomes of more than 75,000 patients. Two major multi-institutional efforts have attempted to measure case complexity; the Risk Adjustment in Congenital Heart Surgery-1 and the Aristotle Complexity Score. Efforts to unify these two scoring systems are in their early stages but are encouraging. Collaborative efforts involving the EACTS and STS are under way to develop mechanisms to verify data completeness and accuracy. Further collaborative efforts are also ongoing between pediatric and congenital heart surgeons and other subspecialties, including pediatric cardiac anesthesiologists (via the Congenital Cardiac Anesthesia Society), pediatric cardiac intensivists (via the Pediatric Cardiac Intensive Care Society), and pediatric cardiologists (via the Joint Council on Congenital Heart Disease). Clearly, methods of congenital heart disease outcomes analysis continue to evolve, with continued advances in five areas: nomenclature, database, complexity adjustment, data verification, and subspecialty collaboration.     

Clinical presentation of Crohn’s,ulcerative colitis,and indeterminate colitis: Symptoms,extraintestinal manifestations,and disease phenotypes

The incidence of inflammatory bowel disease (IBD) is rising with 25% of IBD diagnosed in children under 18 years of age. The clinical presentation of IBD in children is often vague leading to initial misdiagnosis as infectious colitis or irritable bowel syndrome. When IBD is identified, overlap in histologic and endoscopic features may lead to difficulty distinguishing Crohn’s disease from ulcerative colitis, resulting in a higher frequency of the diagnosis indeterminate colitis or IBD unspecified. Recognizing the common and the atypical presentation of pediatric IBD and extraintestinal manifestations will aid in expeditious referral and early diagnosis. Activity severity scoring tools and more specific classification systems for pediatric IBD direct therapeutic algorithms and allow for improved longitudinal assessment since disease severity and location have been shown to be associated with outcome.     

Food-induced anaphylaxis: who, what, why, and where?

Food-induced anaphylaxis is a leading cause of anaphylaxis treated in emergency departments and hospitals around the world. Peanuts, tree nuts, fish, and shellfish are the most commonly implicated foods. Food-induced anaphylaxis may occur in any age group and with any food. However, food-induced anaphylaxis fatalities disproportionately affect adolescents and young adults with peanut and tree nut allergy. Individuals who have both IgE-mediated food allergy and asthma are at a higher risk for food-induced anaphylaxis fatality. Delayed administration of epinephrine is also associated with fatal outcome. Often, in fatal reactions, the food allergen is unknowingly ingested away from home, in settings such as restaurants and schools. Although avoidance of food allergens is critical, timely administration of epinephrine is also of great importance in the treatment of food-induced anaphylaxis. Patients, families, and caregivers must be well educated regarding the signs, symptoms and risk factors for anaphylaxis. They must also be counseled on the importance of strict food avoidance of the implicated food allergens, compliance with having self-injectable epinephrine available at all times, and the importance of timely administration of epinephrine, even when cutaneous symptoms are lacking.     

Pediatricians’ Knowledge,Attitudes, and Practice Patterns Regarding Special Education and Individualized Education Programs

ObjectiveThe medical community has called upon pediatricians to be knowledgeable about an individualized education program (IEP). We sought to: 1) evaluate pediatricians’ knowledge and attitudes regarding special education; 2) examine the relationship between perceived responsibilities and practice patterns; and 3) identify barriers that impact pediatricians’ ability to provide comprehensive care to children with educational difficulties.MethodsSurveys were mailed to a national sample of 1000 randomly selected general pediatricians and pediatric residents from October 2010 to February 2011.ResultsThe response rate was 47%. Of the knowledge items, respondents answered an average of 59% correctly. The majority of respondents thought pediatricians should be responsible for identifying children who may benefit from special education services and assist families in obtaining services, but less than 50% thought they should assist in the development of an IEP. The majority of pediatricians inquired whether a child is having difficulty at school, but far fewer conducted screening tests or asked parents if they needed assistance obtaining services. Overall, the prevalence of considering a practice a pediatrician’s responsibility is significantly higher than examples of such a practice pattern being reported. Financial reimbursement and insufficient training were among the most significant barriers affecting a pediatrician’s ability to provide care to children with educational difficulties.ConclusionsIn order to provide a comprehensive medical home, pediatricians must be informed about the special education process. This study demonstrates that there are gaps in pediatricians’ knowledge and practice patterns regarding special education that must be addressed.     

The Relationship between Adolescents’ Social Capital and Individualism-Collectivism in Estonia,Germany, and Russia

The present study examines the relationship between adolescents’ social capital and individualism-collectivism using data from the Value of Children Study (Trommsdorff and Nauck 2005) from Estonia (N?=?228), Germany (N?=?278), and Russia (N?=?280). Two social capital indexes for adolescents (measuring parental social capital and peer-group social capital) were developed for the analysis. The COLINDEX Scale (Chan 1994) was used to measure individualistic and collectivistic values. In all three countries collectivistic values predicted parental social capital whereas individualistic values predicted peer-group social capital. There were also a few country-specific relationships between the constructs, with collectivism and peer-group social capital being positively related in Estonia and individualism and parental social capital significantly negatively correlated in Russia. The current analysis suggests that during the adolescence, collectivistic values are more likely to be related to higher levels of parental social capital and individualistic values to higher levels of peer-group social capital. Therefore, it seems that at the individual level and for adolescents the individualism and collectivism are related to different forms of social capital in the different manner.     

Asthma and Medicines – Long-Term Side-Effects,Monitoring and Dose Titration

Asthma is a major pediatric respiratory morbidity requiring long-term management. A thorough knowledge of long-term medication side-effects in children is, thus, essential for every physician dealing with childhood asthma. Establishing diagnosis and initiating treatment is just a beginning of the journey. Ongoing monitoring is an essential component of comprehensive asthma management programme. Monitoring includes not only assessment of asthma control but also checking for adherence to treatment, technique of inhaler device use, associated co-morbities, if any, and potential environmental exposure. Various tools – both subjective and objective - are available for assessment of asthma control. However, evidence for their optimum use in different settings and patient groups is lacking and monitoring has to be customized depending on available resources and individual patient characteristics. Patient education is an important component of long-term asthma therapy. The ultimate aim is to achieve optimum asthma control i.e., achieve and maintain control of clinical symptoms, decrease future risk to patients (risk of exacerbations, progressive loss of lung function and development of fixed airflow obstruction, adverse effects of medications) and enabling the child to lead a life without restrictions, at lowest possible dose of drugs. This article reviews the side-effects of medications used for long-term management of asthma and discusses current literature on asthma monitoring and dose titration in pediatric population to help the asthma therapist not only prescribe the drugs rationally but also help the family make right choices for treatment.     

Overweight and obesity and their associated factors in adolescents in Tehran, Iran, 2004–2005

Overview Obesity is a significant health crisis around the world. Of great concern are the data pointing to the recent increase in the prevalence of obesity irregardless of age group and country. Overweight and obesity in adolescence are markers of overweight and obesity in adults, respectively. Very little data are currently available on the prevalence of childhood obesity in Iran, and more research on the risk factors is required before preventive public health programs can be formulated and put into practice.Objective The objective of this study was to quantify the prevalence of overweight and obesity and their associated factors in adolescent children living in Tehran.Materials and methods During a multistage stratified cluster sampling, 2900 students (1200 males and 1700 females) aged 11–17 years were selected from 20 secondary schools in the school year of 2004–2005. A questionnaire was filled, and weight and height were measured.Discussions and conclusions The body mass index (BMI) was calculated and adjusted for age and sex. Prevalences of overweight and obesity were 17.9 and 7.1%, respectively. BMI increased with age, and it was higher in those who had lower levels of physical activity. Age at menarche was negatively associated with BMI. There was no relationship between macro- and micronutrient intake and overweight and obesity. This study highlights the high prevalence of overweight and obesity in adolescent children in Tehran.     

Editorial: Polygenic scores in child and adolescent psychiatry – strengths,weaknesses, opportunities and threats

Polygenic scores estimate an individual’s genetic liability for a particular disorder or trait. They are based on current knowledge of the trait’s genetic architecture and focus on common genetic variants. In this editorial, I will discuss some of the strengths, weaknesses, opportunities and threats (SWOT) to polygenic scores within the context of child and adolescent psychiatry. I consider how the potential application of polygenic scores in health settings has some parallels with existing practices, but that polygenic scores also undoubtedly raise unique challenges. This SWOT analysis is accompanied by discussion of some new findings using polygenic scores in this issue of Journal of Child Psychology and Psychiatry.     

Situs inversus,bronchiectasis, and sinusitis and its relation to immotile cilia: history of the diseases and their discoverers—Manes Kartagener and Bjorn Afzelius

The relationship of Kartageners syndrome to immobile cilia syndrome is a fascinating merging of clinical observations and basic science in Zurich, Stockholm, and Toronto. In 1933, Manes Kartagener, a Zurich pulmonary physician, reported four patients with the triad of sinusitis, bronchiectasis, and situs inversus. In the following decades, he reviewed reports of hundreds of cases, but the fact that the male patients with the condition never had offspring eluded his notice. In the 1970s, Bjorn Afzelius, a Ph.D. ultrastructuralist from Stockholm, reported cilia immotility in infertile males, some of the cases occurring in families. Half of the cases had Kartageners triad. The observation of Afzelius was soon applied to children by Jennifer Sturgess, a Ph.D. ultrastructuralist, and her medical colleagues in Toronto. With over 500 MEDLINE references since 1966 on Kartageners and over 1,000 references on immotile cilia, the causes of the pulmonary infections have become clearer as the patients demonstrate impaired clearance of mucus with resultant sinus and bronchial disease. The cause of the situs inversus remains elusive to this day. It is appropriate to call the condition Kartagener-Afzelius syndrome.     

Dopaminergic,serotonergic, and oxytonergic candidate genes associated with infant attachment security and disorganization? In search of main and interaction effects

Background and methods: In two birth cohort studies with genetic, sensitive parenting, and attachment data of more than 1,000 infants in total, we tested main and interaction effects of candidate genes involved in the dopamine, serotonin, and oxytocin systems (DRD4, DRD2, COMT, 5‐HTT, OXTR) on attachment security and disorganization. Parenting was assessed using observational rating scales for parental sensitivity ( Ainsworth, Bell, & Stayton, 1974 ), and infant attachment was assessed with the Strange Situation Procedure. Results: We found no consistent additive genetic associations for attachment security and attachment disorganization. However, specific tests revealed evidence for a codominant risk model for COMT Val158Met, consistent across both samples. Children with the Val/Met genotype showed higher disorganization scores (combined effect size d = .22, CI = .10–.34, p < .001). Gene‐by‐environment interaction effects were not replicable across the two samples. Conclusions: This unexpected finding might be explained by a broader range of plasticity in heterozygotes, which may increase susceptibility to environmental influences or to dysregulation of emotional arousal. This study is unique in combining the two largest attachment cohorts with molecular genetic and observed rearing environment data to date.     

Infant cardiac surgery and the father–infant relationship: Feelings of strength,strain, and caution

Objective

The purpose of this study was to examine the father–infant relationship in infants with congenital heart disease (CHD).

Method

Sixty-three fathers whose infants had cardiac surgery before 3 months of age reported on their attachment relationship with their infant within two months of hospital discharge using both qualitative and quantitative methods.

Results

Mean scores on the Paternal Postnatal Attachment Scale and scores for patience and tolerance were not different from previously published community norms, ps > .05. Scores for pleasure and interaction (t[50] = − 2.383, p = .021, CI: − 2.93, − .25) and affection and pride subscale (t[56] = − 2.935, p = .005, CI: − 1.20, − .23) were significantly lower than community norms. Additionally, 37% of fathers described feeling a strong relationship with their infant whereas 17% reported initial apprehension or condition-specific worry. Fathers with infants who spent fewer days at home prior to admission reported feelings of relationship strain as well as lower pleasure in interaction, affection and pride, patience and tolerance, and overall attachment quality.

Conclusion

Having an infant with CHD affects some father–infant relationships differently than others with some fathers feeling closer to their infant and other fathers feeling reservation about getting too close. One explanation for these differences may be that spending a great deal of time in hospital restricts the number and quality of interactions infants have with their fathers. Opportunities for intervention include clinical psychosocial services encouraging fathers to interact with and provide physical care of their infant, especially if fathers perceive their infant as medically fragile.