The consequences of prenatal alcohol exposure were first described more than 40 years ago.1,2 The term "fetal alcohol syndrome" (FAS) was first used to describe the cluster of birth defects due to ...prenatal alcohol exposure (including growth restriction, craniofacial abnormalities and intellectual disabilities) with lifetime consequences.2 The term "fetal alcohol spectrum disorder" (FASD) has since been adopted to describe a broader spectrum of presentations and disabilities resulting from alcohol exposure in utero. The prevalence has been estimated at 1 in 100 people, which translates to more than 330 000 affected individuals in Canada.3 The development of clinical capacity for FASD diagnosis remains difficult,4 because the diagnosis requires a medical evaluation and neurodevelopmental assessment conducted by a multidisciplinary team. In 2005, an international, collaborative, evidence-based guideline for diagnoses related to prenatal alcohol exposure was published.5 Since then, the field has evolved, and additional evidence, expertise and experience have emerged to suggest that a revision was required to improve both diagnoses and outcomes. The literature has also shown that impairments in behaviour and function associated with FASD have been detected from exposure to binge drinking, even infrequently or early in pregnancy, which underscores the importance of pre-pregnancy counselling. Specific research involving infants, young children and adults with FASD, as well as further insight into the neurodevelopmental dysfunction and nomenclature, prompted the update and revision process. A literature review and broad consultation process was undertaken to revise the 2005 guideline for diagnosing FASD.5 The guideline was developed according to the Appraisal of Guidelines, Research and Evaluation (AGREE II) framework,6 which provides a rigorous, evidence-based approach. The steering committee identified the scope of the guidelines and developed the key questions. These tasks were accomplished during the first meetings, after which the scope and questions were further refined and approved by consensus. The literature review was then conducted by two committee members (J.L.C. and C.R.G.) using the following combinations of MeSH (medical subject heading) search terms to address the key questions: "fetal alcohol," "diagnosis*," "infant*," "prenatal alcohol" and "adult." Relevant reports published from 2005 to September 2014 were identified from the following databases: PubMed, PsychLIT, Medscape, MEDLINE, the Canadian Institutes of Health Research funding database and the Cochrane Library.
Social determinants of health (SDoH) influence overall health, although little is known about the SDoH for pediatric patients requiring surgical services. This study aims to describe SDoH for ...pediatric surgical patients attending out-patient, community, and outreach clinics, as well as demonstrate the feasibility of identifying and addressing SDoH and Adverse Childhood Experiences (ACEs) when appropriate.
A cross-sectional study using surveys evaluating SDoH that were distributed to families attending pediatric surgical clinics over a two-year period. The pilot survey used validated questions and was later refined to a shorter version with questions on: Barriers to care, Economic factors, Adversity, Resiliency and Social capital (BEARS). Data was analyzed with descriptive and inferential statistics.
851 families across 13 clinics participated. One third of families reported not having a primary health care provider or being unable to turn to them for additional support. One in four families were found to have a household income less than the Canadian after-tax low-income threshold (<$40,000 CAD). Two-thirds of families answered questions about ACEs, and those with more ACEs were more likely to report a low income. Forty percent of families rarely or only sometimes had adequate social support.
This survey tool enabled discussions between families and care providers, which allowed clinicians to appropriately follow-up with families and refer them to social work for further support when indicated. Addressing concerns around SDoH within a busy surgical clinical is feasible and may positively affect long-term health outcomes and equitable resource allocation.
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•What is currently known about this topic?
Healthcare providers recognize social determinants of health (SDoH) affect a child's overall health and access to healthcare. SDoH for pediatric surgical patients in a socialized healthcare system are not well known, and more understanding is needed on how surgical disciplines can discuss and support families with SDoH.•What new information is contained in this article?
SDoH for 851 families attending pediatric surgical clinics are summarized. A survey using validated questions, and later refined to a shorter version is described (Barriers to care, Economic factors, Adversity, Resiliency, Social capital). The questionnaire supported discussions with families and allowed for appropriate referrals to, and follow-up with social work.
Objective:
A 2016 review of the BC Children’s Hospital Cleft Palate - Craniofacial Program (CPP) revealed that one-third of patients met the program’s care recommendations and half met the American ...Cleft Palate-Craniofacial Association guidelines. This study reviews patients on the CPP waitlist and determines median wait times and missed clinical assessments as well as identifies how wait times are influenced by medical complexity, specialized speech service needs, vulnerability, and distance from clinic.
Design:
Cross-sectional.
Setting:
BC Children’s Hospital Cleft Palate—Craniofacial Program.
Patients:
Five hundred seventy-six waitlisted patients.
Main Outcome Measures:
Additional wait time after recommended appointment date. Correlation of additional wait time with diagnosis, number of specialists required, speech services needed, vulnerability, and distance from the clinic. Missed plastic surgery, speech, and orthodontic assessments according to CPP team recommendations and ACPA guidelines.
Results:
Patients had a median additional wait time of 11 months (interquartile range: 5-27). Longer additional wait times were associated with a craniofacial diagnosis (P = .019), a need for formal speech assessments or evaluations (P < .001), or a requirement to see multiple specialists (P < .001). Vulnerability and distance from clinic did not affect wait times. Plastic surgery assessments were not available at the preschool and preteen time points for 45 (8%) patients, 355 (62%) patients were unable to access speech assessments, and 120 (21%) were unable to complete an orthodontic assessment.
Conclusion:
Patients wait up to an additional year to be seen by the CPP and miss speech, orthodontic, and surgical assessments at key developmental milestones. Additional resources are required to address these concerns.
This article describes the combined clinical experience of a multidisciplinary group of professionals on the sleep disturbances of children with fetal alcohol spectrum disorders (FASD) focusing on ...sleep hygiene interventions. Such practical and comprehensive information is not available in the literature. Severe, persistent sleep difficulties are frequently associated with this condition but few health professionals are familiar with both FASD and sleep disorders. The sleep promotion techniques used for typical children are less suitable for children with FASD who need individually designed interventions. The types, causes, and adverse effects of sleep disorders, the modification of environment, scheduling and preparation for sleep, and sleep health for their caregivers are discussed. It is our hope that parents and also researchers, who are interested in the sleep disorders of children with FASD, will benefit from this presentation and that this discussion will stimulate much needed evidence-based research.
A disproportionately large number of youth and adults with fetal alcohol syndrome (FAS) and fetal alcohol effects (FAE) seem to be coming into conflict with the legal system. Learning and behavioral ...difficulties associated with FAS/FAE may make them more susceptible to criminal behavior. This study determined the prevalence of FAS/FAE among youth who were remanded for a forensic psychiatric/psychological assessment. All youth remanded to a forensic psychiatric inpatient assessment unit over a 1-year period were evaluated for FAS/FAE. Of the 287 youth, 67 (23.3%) had an alcohol-related diagnosis: 3 (1.0%) had a diagnosis of FAS and 64 (22.3%) had a diagnosis of FAE. Thus, this group is disproportionately represented in the juvenile justice system, indicating the need for increased education and awareness among those in the criminal justice system involved with these youth.
Signal peptide peptidase (SPP) and the four homologous SPP-like proteases SPPL2a, SPPL2b, SPPL2c and SPPL3 are GxGD-type intramembrane-cleaving proteases (I-CLIPs). In addition to divergent ...subcellular localisations, distinct differences in the mechanistic properties and substrate requirements of individual family members have been unravelled. SPP/SPPL proteases employ a catalytic mechanism related to that of the γ-secretase complex. Nevertheless, differential targeting of SPP/SPPL proteases and γ-secretase by inhibitors has been demonstrated. Furthermore, also within the SPP/SPPL family significant differences in the sensitivity to currently available inhibitory compounds have been reported. Though far from complete, our knowledge on pathophysiological functions of SPP/SPPL proteases, in particular based on studies in mice, has been significantly increased over the last years. Based on this, inhibition of distinct SPP/SPPL proteases has been proposed as a novel therapeutic concept e.g. for the treatment of autoimmunity and viral or protozoal infections, as we will discuss in this review. This article is part of a Special Issue entitled: Proteolysis as a Regulatory Event in Pathophysiology edited by Stefan Rose-John.
•SPP/SPPL proteases are GxGD-type intramembrane proteases like presenilins.•Differential targeting of SPP/SPPLs and γ-secretase by inhibitors is possible.•Sensitivity of individual SPP/SPPL proteases to currently known inhibitors differs.•SPP inhibition may be beneficial to treat certain viral and protozoal infections.•SPPL2a may be a target to deplete B cells and dendritic cells upon autoimmunity.
Abstract
Background
Adverse Childhood Experiences (ACEs) are a group of early life events that lead to toxic stress and adverse adult health outcomes. Screening for ACEs can be challenging due to ...sensitivity and re-traumatization. There is a paucity of evidence regarding whether other social determinants of health (SDoH) might be independent predictors of an ACE score >=4. Likewise, no effective prediction rule exists for an elevated ACE score based on SDoH in children.
Objectives
1) Identify independent predictors of elevated ACE score from commonly screened SDoH.
2) Derive a clinical prediction rule based on the available data.
Design/Methods
Data were drawn from a longitudinal quality improvement SDoH study in pediatric surgical clinics at a provincial children’s hospital. Primary outcome of interest was an ACE score >=4. Multivariable logistic regression was utilized to identify independent predictors among other SDoH. Prediction methods and ROC analyses were completed to derive a prediction rule.
Results
515 respondents answered ACE screening; 63 (12.2%) reported >=4 ACEs. SDoH that were strong independent predictors of ACE score >=4 included poverty (OR 2.34, 95% CI 1.19-4.91), parental education (OR 2.76, 95% CI 1.17-6.54), and household income (OR 2.17, 95% CI 1.09-4.32). Housing status, Indigenous status, and disability status were not associated with elevated ACE score. A clinical prediction rule derived using four SDoH questions with a cut-off score of 1 had 96.67% sensitivity but only 21.54% specificity for an ACE score >=4 (AROC 0.75, 95% CI 0.69-0.81).
Conclusion
Several adverse SDoH were identified as independent predictors of an ACE score >=4 in children. A clinical prediction rule based on SDoH screening was sensitive but poorly specific for ACE >=4. Further research is required.
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