Oral Health Behaviour and its Influence on Oral Health Status and Related Quality of Life among Dental Outpatients - A Cross-Sectional Study.

OBJECTIVES: This study aimed to predict oral health behaviour (OHB) using the theory of planned behaviour (TPB) and determine its influence on oral health status and oral health-related quality of life (OHRQoL) among subjects attending the outpatient department of a tertiary dental hospital in India. METHODS: A pre-validated questionnaire was used among 240 randomly selected study subjects to record their demographic details, attitudes (Att), subjective norms (SN) and perceived behaviour control (PBC) with regard to dietary habits (DH), oral hygiene habits (OH) and dental attendance (DA) behaviours. OHRQoL and oral health status of study participants were recorded using Oral Health Impact Profile-14 (OHIP-14) and World Health Organization (WHO) Oral Health Assessment Form (2013), respectively. Multivariate analysis was performed after the necessary bivariate comparisons. RESULTS: Among demographic characteristics, the Socioeconomic status (SES) of the study subjects highly influenced their DH and OH (P < 0.05). DA was largely affected by the age of the study subjects (P < 0.05). While attitude of the study participants greatly affected their DH, PBC largely influenced their OH and DA behaviours (P < 0.05). The OHRQoL and Decayed Missing Filled Teeth (DMFT) levels were strongly influenced by the participants' DA behaviours (P < 0.05). CONCLUSIONS: DMFT scores and OHRQoL were highly influenced by DA behaviour besides others. DA instead was influenced by PBC. Hence, there needs to be a conscious shift towards strengthening the skills of the population to promote oral health.

Growth patterns in individuals with CDKL5 deficiency disorder.

AIM: To compare growth in individuals with cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder with population norms and to investigate the effect of gastrostomy on growth. METHOD: The longitudinal study included 353 individuals from the International CDKL5 Disorder Database with any anthropometric measurement in baseline and/or follow-up questionnaires. The British 1990 growth reference was used to determine the age- and sex-standardized z-score. Repeated cross-sectional data were fitted using a Gaussian linear regression model with generalized estimating equations. RESULTS: All growth parameters were below the general population norm (mean z-scores: weight -0.97, height -0.65, body mass index [BMI] -0.81, head circumference -2.12). The disparity was particularly pronounced for all anthropometric measurements after 4 years of age except for BMI. Moreover, individuals with gastrostomy placement were shown to have a larger decrease than those without. INTERPRETATION: In addition to weight, height, and BMI, head circumference was also compromised in this disorder. Microcephaly could be considered a helpful diagnostic feature, especially in adults. Any benefit of gastrostomy on weight and BMI was mainly seen in the early years.

Hospitalizations from Birth to 28 Years in a Population Cohort of Individuals Born with Five Rare Craniofacial Anomalies in Western Australia.

OBJECTIVE: To describe trends, age-specific patterns, and factors influencing hospitalizations for 5 rare craniofacial anomalies (CFAs). METHODS: Data on livebirths (1983-2010; n = 721 019) including rare CFA (craniofacial microsomia, mandibulofacial dysostosis, Pierre Robin sequence, Van der Woude syndrome, and frontonasal dysplasia), episodes of death, and demographic and perinatal factors were identified from the Western Australian Register of Developmental Anomalies, Death Registrations and Midwives Notification System. Information on incident craniofacial and noncraniofacial related admissions, length of hospital stay, and intensive care and emergency-related admissions were identified using principal diagnosis and procedural codes were extracted from the Hospital Morbidity Data Collection and linked to other data sources. Associations of hospitalizations by age groups as well as demographic and perinatal factors were expressed as incidence rate ratio (IRR). RESULTS: The incident hospitalizations were 3 times as high for rare CFA (IRR 3.22-3.72) throughout childhood into adolescence than those without. Children with rare CFA had 3-4 times as many potentially preventable hospitalizations until 18 years of age than those without. Specifically, respiratory infections (IRR 2.13-2.35), ear infections (IRR 7.92-26.28), and oral health-related conditions contributed for most noncraniofacial admissions until the adolescence period. A greater incidence of noncraniofacial related hospitalizations was observed among Indigenous children, births with intrauterine growth restrictions, and families with high socioeconomic disadvantage. CONCLUSIONS: Throughout childhood, individuals with rare CFA had greater hospital service use, specifically for potentially preventable conditions, than those without. These population-level findings can inform new preventive strategies and early disease management targeted toward reducing preventable hospitalizations.

Patterns, trends, and factors influencing hospitalizations for craniosynostosis in Western Australia. A population-based study.

Understanding hospital service use among children with a diagnosis of craniosynostosis (CS) is important to improve services and outcomes. This study aimed to describe population-level trends, patterns, and factors influencing hospitalizations for craniosynostosis in Western Australia. Data on live births (1990-2010; n = 554,624) including craniosynostosis, episodes of death, demographic, and perinatal factors were identified from the midwives, birth defects, hospitalizations, and death datasets. Information on craniosynostosis and non-craniosynostosis-related admissions, cumulative length of hospital stay (cLoS), intensive care unit, and emergency department-related admissions were extracted from the hospitalization dataset and linked to other data sources. These associations were examined using negative binomial regression presented as annual percent change and associations of hospitalizations by age groups, demographic, and perinatal factors were expressed as incidence rate ratio (IRR). We found an increasing trend in incident hospitalizations but a marginal decline in cLoS for craniosynostosis over the observed study period. Perinatal conditions, feeding difficulties, nervous system anomalies, respiratory, and other infections contributed to majority of infant non-CS-related admissions.Respiratory infections accounted for about twice the number of admissions for individuals with CS (IRRs 1.94-2.34) across all observed age groups. Higher incidence of non-CS hospitalizations was observed among females, with associated anomalies, to families with highest socioeconomic disadvantage and living in remote areas of the state.   Conclusion: Marginal reduction in the cLoS for CS-related admissions observed over the 21-year period are potentially indicative of improved peri-operative care. However, higher incidence of respiratory infection-related admissions for syndromic synostosis is concerning and requires investigation.

Quantification of Helicobacter pylori and its oncoproteins in the oral cavity: A cross-sectional study.

OBJECTIVE: To identify Helicobacter pylori (H. pylori) and related oncogenic and virulent proteins (CagA and VacA) in patients with gingivitis, periodontitis, oral cancer and gastric cancer. METHODS: Subgingival plaque samples were collected from 90 individuals with either gingivitis/periodontitis (group A, n = 30), oral cancer (group B, n = 30) and gastric cancer (group C, n = 30). H. pylori was identified by real-time polymerase chain reaction (RT-PCR). The virulent organisms were detected by identification of proteins CagA and VacA through Enzyme Linked Immuno-Sorbent Assay (ELISA). RESULTS: We identified the presence of H. pylori in subgingival plaque samples among a large majority (76/90) of our study cohort. The proportions of CagA and VacA identified among H. pylori individuals with periodontal inflammation and oral cancer were lower than those diagnosed with gastric cancer. Furthermore, the relative risk of oral cancer based on the presence of the organism was no different to those with gingivitis/periodontitis. CONCLUSION: The findings of our study do not indicate significant association between the organism and oral cancer, but preludes that the oral cavity could act as a potential niche for H. pylori. The possibility for CagA and VacA proteins to be pathogenic in oral cavity is highly possible and must be researched extensively.

Epidemiology of Hospital Admissions for Craniosynostosis in Australia: A Population-Based Study.

To describe trends, age, and sex-specific patterns of population hospital admissions with a diagnosis of craniosynostosis (CS) in Australia.Population data for hospital separations (in-patient) from public and private hospitals (July 1996-June 2018) were obtained from the publicly available Australian Institute of Health and Welfare (AIHW) National Hospital Morbidity Database.The outcome variables were hospital separation rates (HSR) (number of hospital separations divided by the estimated resident population [ERP] per year) and average length of stay (aLOS) (patient days divided by the number of hospital separations) with a diagnosis of CS. Trends in HSR and aLOS adjusted for age, sex, and type of CS were investigated by negative binomial regression presented as annual percent change (APC).In 8057 admissions identified, we observed no significant change in the annual trend for HSR for the 22-year period. However, a marginal annual decrease of 1.6% (95% CI: -0.7, -2.4) in the aLOS was identified for the same time period. HSR were higher for males, infants, and single suture synostosis. aLOS was 3.8 days (95% CI: 3.8, 3.9) per visit, longer for syndromic conditions.There was a minor reduction in the average length of hospital stay for CS over the 22-year period potentially indicative of improved care. Population-level information on hospitalisations for rare craniofacial conditions can inform research, clinical, and surgical practice.

Factors influencing the attainment of major motor milestones in CDKL5 deficiency disorder.

This study investigated the influence of factors at birth and in infancy on the likelihood of achieving major motor milestones in CDKL5 Deficiency Disorder (CDD). Data on 350 individuals with a pathogenic CDKL5 variant was sourced from the International CDKL5 Disorder Database. A first model included factors available at birth (e.g., sex, variant group and mosaicism) and the second additionally included factors available during infancy (e.g., age at seizure onset, number of anti-seizure medications used, experience of a honeymoon period and formal therapy). Cox regression was used to model the time to achieve the milestones. The probability of attaining the outcomes at specific ages was estimated by evaluating the time-to-event function at specific covariate values. Independent sitting and walking were achieved by 177/350 and 57/325 children respectively. By seven years of age, 67.1% of females but only 37.3% of males could sit independently. About a quarter each of females and males achieved independent walking by eight and six years, respectively. When observed from birth, female gender, a late truncating variant and mosaicism impacted most positively on the likelihood of independent sitting. When observed from one year, later seizure onset and experiencing a honeymoon period also improved the likelihood of independent sitting. Factors that favoured sitting (except gender) also improved walking. Having a truncating variant between aa178 and aa781 reduced the likelihood of achieving independent sitting and walking. It is possible to utilise factors occurring early in life to inform the likelihood of future motor development in CDD.

Association between Parental Stress and Early Childhood Caries Experience among Preschool Children in Maduravoyal, Chennai: A Cross-sectional Study.

Background: In recent decades stress has emerged as a variable of significant interest in the examination of oral health. Objective: To study the association between parental stress and early childhood caries experience among 3-5 years old children. Methods: A cross-sectional study was conducted among 342 randomly selected mother-child dyads in Maduravoyal, Chennai. The mothers were stratified into four stress categories based on their parental stress scores from the Parental Stress Scale (Berry and Jones, 1995). The children were classified into three subgroups, no caries [dmft = 0], low caries [dmft = 1-2], and high caries [dmft≥3]. Chi-square test with Yates' continuity correction and Spearman's rank correlation were used as statistical test methods. Results: Among the mothers with no stress, 44.9% had children in the no caries group, compared to 21.6% in the low caries and 7.3% in the high caries group [p < 0.001]. And among the mothers with mild to moderate stress, 6.3% had children in the no caries group, compared to 5.4% in the low caries and 30% in the high caries group [p < 0.001]. A positive correlation between parental stress and early childhood caries experience was observed [rho = 0.461, p < 0.001]. Conclusion: A significant moderate positive correlation between parental stress and early childhood caries experience was observed. Stressful parents can be a probable risk factor for early childhood caries. Hence, providing prenatal counseling will be of added value to all expecting mothers. How to cite this article: Mahapatra S, Chaly PE, Junaid M, et al. Association between Parental Stress and Early Childhood Caries Experience among Preschool Children in Maduravoyal, Chennai: A Cross-sectional Study. Int J Clin Pediatr Dent 2022;15(S-2):S131-S134.

Association between craniofacial anomalies, intellectual disability and autism spectrum disorder: Western Australian population-based study.

BACKGROUND: Accurate knowledge of the relationship between craniofacial anomalies (CFA), intellectual disability (ID) and autism spectrum disorder (ASD) is essential to improve services and outcomes. The aim is to describe the association between CFA, ID and ASD using linked population data. METHODS: All births (1983-2005; n = 566,225) including CFA births (comprising orofacial clefts, craniosynostosis, craniofacial microsomia and mandibulofacial dysostosis) surviving to 5 years were identified from the birth, death, birth defects and midwives population data sets. Linked data from these data sets were followed for a minimum of 5 years from birth until 2010 in the intellectual disability database to identify ID and ASD. These associations were examined using a modified Poisson regression. RESULTS: Prevalence of ID and ASD was higher among CFA (especially with additional anomalies) than those without [prevalence ratio 5.27, 95% CI 4.44, 6.25]. It was higher among CFA than those with other gastrointestinal and urogenital anomalies but lower than nervous system and chromosomal anomalies. Children with CFA and severe ID had a higher proportion of nervous system anomalies. CONCLUSIONS: Findings indicate increased ID and ASD among CFA but lower than nervous system and chromosomal anomalies. This population evidence can improve early identification of ID/ASD among CFA and support service planning. IMPACT: Our study found about one in ten children born with craniofacial anomalies (CFA) are later identified with intellectual disability (ID). Prevalence of ID among CFA was higher than those with other gastrointestinal, urogenital, and musculoskeletal birth defects but lower than those with the nervous system and chromosomal abnormalities. Most children with craniofacial anomalies have a mild-to-moderate intellectual disability with an unknown aetiology. On average, intellectual disability is identified 2 years later for children born with non-syndromic craniofacial anomalies than those with syndromic conditions. Our findings can improve the early identification of ID/ASD among CFA and support service planning.

The Lived Experience of Parents' Receiving the Diagnosis of CDKL5 Deficiency Disorder for Their Child.

CDKL5 deficiency disorder (CDD), a severe developmental and epileptic encephalopathy, is being diagnosed earlier with improved access to genetic testing, but this may also have unanticipated impacts on parents' experience receiving the diagnosis. This study explores the lived experience of parents receiving a diagnosis of CDD for their child using mixed methods. Thirty-seven semistructured interviews were conducted with parents of children with a diagnosis of CDD, which were coded and analyzed to identify themes. Grief was a nearly universal theme expressed among participants. Parents of younger children discussed grief in the context of receiving the diagnosis, whereas parents of older children indicated they were at different stages along the grieving journey when they received the diagnosis. Parents with less understanding of their child's prognosis (poorer prognostic awareness) connected their grief to receiving the diagnosis as this brought a clear understanding of the prognosis. Several themes suggested what providers did well to improve the diagnostic experience for parents, much of which aligns with existing literature around how to provide serious news. Additionally, parents identified long-term benefits of having a diagnosis for their child's medical problems. Although interview data were concordant with a survey of parents' diagnostic experience from a large international cohort, most participants in this study were relatively affluent, white mothers and further research is needed to better understand if other groups of parents have a different diagnostic experience. This study gives context of parental experience that providers should be aware of when conveying new genetic diagnoses to families.

Epidemiology of Rare Craniofacial Anomalies: Retrospective Western Australian Population Data Linkage Study.

OBJECTIVE: To describe birth prevalence of rare craniofacial anomalies and associations with antenatal and perinatal factors. STUDY DESIGN: All live and stillbirths in Western Australia between 1980 and 2010 were identified from the Western Australian Birth Registrations and the Midwives Notification System (also provides information on antenatal and perinatal factors). Rare craniofacial anomalies (craniosynostosis, craniofacial microsomia, and others [Pierre Robin, Van der Woude, and Treacher Collins syndrome]) were ascertained from the Western Australian Register of Developmental Anomalies and linked to other data sources. Trends in prevalence, adjusted for sex and Indigenous status, were investigated by Poisson regression and presented as annual percent change (APC). Strengths of association of related factors were assessed using multivariable log-binomial regression adjusted for sex, Indigenous status, birth year, socioeconomic disadvantage, and remoteness and reported as risk ratios with 95% CIs. RESULTS: There was a temporal increase in prevalence of metopic synostosis (APC 5.59 [2.32-8.96]) and craniofacial microsomia (Goldenhar syndrome) (APC 4.43 [1.94-6.98]). Rare craniofacial anomalies were more likely among infants born preterm, as twins or greater-order multiples, with growth restriction, to older parents, to mothers undertaking fertility treatments, and with pre-existing medical conditions, specifically epilepsy, diabetes, or hypothyroidism. Prenatal identification of rare craniofacial anomalies was uncommon (0.6%). CONCLUSIONS: Our findings indicate a steady increase over time in prevalence of metopic synostosis and craniofacial microsomia (Goldenhar syndrome). Possible associations of fertility treatments and pre-existing maternal medical conditions with rare craniofacial anomalies require further investigation.

Description of Total Population Hospital Admissions for Treacher Collins Syndrome in Australia.

OBJECTIVE: To describe patterns and demographic characteristics of total-population hospital admissions with a diagnosis of Treacher Collins syndrome (TCS) in Australia. DATA SOURCE: Population summary data for inpatient hospitals admissions (public and private) with a principal diagnosis of TCS (ICD10-AM-Q87.04) were obtained from the Australian Institute of Health and Welfare National Hospital Morbidity Database for a 11-year period (2002-2013). MAIN OUTCOME MEASURES: The primary outcome was hospital separation rate (HSR), calculated by dividing the number of hospital separations by estimated resident population per year. Trends in HSR s adjusted for age and sex were investigated by negative binomial regression presented as annual percent change and the association of rates with age and sex was expressed as incidence rate ratio. RESULTS: In 244 admissions identified, we observed an increase of 4.55% (95% confidence interval [CI] -1.78, 11.29) in HSR's over the 11-year period. Rates were higher during infancy (1.87 [95% CI 1.42, 2.42]), declining markedly with increasing age. The average length of hospital stay was 6.09 days (95% CI 5.78, 6.40) per episode, but longer for females and infants. CONCLUSIONS: Findings indicate an increase in hospitalization rates, especially among infants and females which potentially relates to early airway intervention procedures possibly influenced by sex specific-disease severity and phenotypic variability of TCS. Awareness of the TCS phenotype and improved access to genetic testing may support more personalized and efficient care. Total-population administrative data offers a potential to better understand the health burden of rare craniofacial diseases.

Factors associated with dental hospitalisations in children with intellectual disability or autism spectrum disorder: a Western Australian population-based retrospective cohort study.

PURPOSE: This study investigated dental hospitalisations in Western Australian (WA) children with intellectual disability (ID) and/or autism spectrum disorder (ASD) aged up to 18 years. METHODS: Data on WA live births from 1983 to 2004 from the WA Midwives Notification System were linked to the Intellectual Disability Exploring Answers database, the WA Hospital Morbidity Data System, and the Western Australian Birth Defects Registry databases. Children were followed from birth to 2010 and the data grouped into three age-groups. Primary and secondary admissions for relevant dental diagnoses were identified and factors associated with having a dental hospitalisation investigated. RESULTS: There were 1366, 1596, and 780 dental hospitalisations amongst 1122, 1154, and 609 children with ID and/or ASD in the 0-6, >6-12, and >12-18 year age groups, respectively. Children with severe ID were much more likely to be hospitalised than those with mild/moderate ID. More socioeconomically disadvantaged children were less likely to be hospitalised than children whose parents were socially advantaged. CONCLUSIONS: There is concern that more vulnerable children in the WA community with ID or ASD are receiving an inadequate level of dental services compared with other groups resulting in potentially preventable hospitalisations, a situation in need of urgent remediation.Implications for rehabilitationLittle is known about why some children with intellectual disability (ID) or autism are being hospitalised for their dental care and others are not.Children with disability whose families are socioeconomically disadvantaged should have equivalent opportunity to receive optimal dental care.Dental practitioners at all levels need training and confidence in treating children with ID.

Influences on the trajectory and subsequent outcomes in CDKL5 deficiency disorder.

OBJECTIVE: The study investigated the effect of seizure and medication burden at initial contact with the International CDKL5 Disorder Database on subsequent development and clinical severity and compared quality of life among those whose development progressed, remained stable, or regressed between baseline and follow-up. METHODS: The effects of seizure and medication burden at baseline (high or low) on the CDKL5 Disorder Severity Scores and CDKL5 Developmental Score (CDS) at follow-up were assessed using linear and negative binomial regressions, respectively, with adjustment for age at baseline, gender, and follow-up duration with and without genotype. Seizure and medication burden were defined by average daily seizure count (high, ≥5/day; low, <5/day) and number of antiseizure medications (high, ≥3/day; low, <3/day), respectively. The effects of change in CDS over time (improved, stable, or deteriorated) on Quality of Life Inventory-Disability (QI-Disability) total and domain scores at follow-up were assessed in those aged at least 3 years at follow-up using linear regression models with adjustment for baseline CDS, gender, and follow-up duration. RESULTS: The expected follow-up CDS was lower for individuals with high compared to low seizure burden at baseline (ß = -.49, 95% confidence interval [CI] = -.84 to -.13). The average total QI-Disability score was 5.6 (95% CI = -.2 to 11.5) points higher among those with improved compared with stable or deteriorating CDS and 8.5 (95% CI = 3.1-13.8) points lower for those with deteriorating compared to stable or improved CDS. SIGNIFICANCE: Our finding that later development showed slight improvement in those with better earlier seizure control even after adjustment for genotype suggests that the trajectory for an individual child is not necessarily predetermined and could possibly be influenced by optimal seizure management. This has implications for children's quality of life.

Mouse periodontitis models using whole Porphyromonas gingivalis bacteria induction.

INTRODUCTION: Due to the increasing prevalence of periodontitis within the general population, it is important to study the progress and stages of periodontal disease and the efficacy of periodontal treatment through in vitro and in vivo experiments. Mouse periodontitis models are important in many in vivo studies. This study presents the findings from a scoping review of the current literature regarding the available method to produce mouse periodontitis models using whole Porphyromonas gingivalis (P. gingivalis) bacteria. METHODS: The scoping review was carried out based on the methodology described by Arskey and O'Malley. An electronic literature search was conducted in the PubMed database. Inclusion and exclusion criteria were established. The data were collected on a purpose-made data extraction table for descriptive analysis. RESULT: The researchers identified 11 articles that met the inclusion criteria for the review. Factors most considered in the literature relating to this topic are the vehicle to induce periodontitis, the type of strain for mice and P. gingivalis, the region of application, sacrifice day and the detection method used to measure the parameters. CONCLUSION: The most frequently used vehicle to induce a mouse periodontitis model is the combination of P. gingivalis with ligature. Future research on different types of vehicles and bacteria for inducing more effective and more time-efficient periodontitis models is needed to guide future researchers on this topic.

Association between ABO, Rh blood groups, lip and dermatoglyphic patterns, and nonsyndromic oral clefts: A case-control study.

OBJECTIVE: The objective of the study is to determine the association between nonsyndromic oral clefts (OC) in children and ABO, Rh blood groups, lip, and dermatoglyphic patterns of their unaffected parents. METHODS: This case-control study was conducted at a tertiary cleft center in Chennai, India, among 240 individuals comprising 80 units (40 cases and controls, respectively). Each unit (triad) was constituted by a child (0-12 years of age) either born with nonsyndromic OC (cases) or with no diagnosed congenital anomaly (control) and their unaffected parents (mother and father). ABO and Rh blood groups, specific lip print, fingerprint pattern, and palmar asymmetry were recorded for each individual. Strength of association of related factors was assessed by multivariable logistic regression reported as adjusted odds ratios and 95% confidence interval. RESULTS: A1-positive blood group was found to be considerably higher among case mothers (14.39 [1.57-32.27]). A higher odds of OCs were observed among case mothers with whorl lip pattern (1.51 [1.16-3.17]) and radial loop pattern in fingers (1.44 [1.09-2.31]) relative to controls. In addition, palmar asymmetry was distinctively higher among case parents compared to controls (P < 0.01). CONCLUSION: Findings indicate that A1-positive blood group, higher frequency of whorl lip, and radial loop finger patterns in mothers and higher ulnar loop pattern in fathers and palmar asymmetry in both parents increases odds of occurrence of OC among their offspring. These identifiable traits offer potential scope for better service planning among resource-constrained disadvantaged communities in India.

A Review of Actuation and Sensing Mechanisms in MEMS-Based Sensor Devices.

Over the last couple of decades, the advancement in Microelectromechanical System (MEMS) devices is highly demanded for integrating the economically miniaturized sensors with fabricating technology. A sensor is a system that detects and responds to multiple physical inputs and converting them into analogue or digital forms. The sensor transforms these variations into a form which can be utilized as a marker to monitor the device variable. MEMS exhibits excellent feasibility in miniaturization sensors due to its small dimension, low power consumption, superior performance, and, batch-fabrication. This article presents the recent developments in standard actuation and sensing mechanisms that can serve MEMS-based devices, which is expected to revolutionize almost many product categories in the current era. The featured principles of actuating, sensing mechanisms and real-life applications have also been discussed. Proper understanding of the actuating and sensing mechanisms for the MEMS-based devices can play a vital role in effective selection for novel and complex application design.

Exploring quality of life in individuals with a severe developmental and epileptic encephalopathy, CDKL5 Deficiency Disorder.

BACKGROUND: CDKL5 Deficiency Disorder (CDD) is a rare genetic disorder caused by a mutation in the cyclin-dependent kinase-like 5 (CDKL5) gene. It is now considered to be a developmental and epileptic encephalopathy because of the early onset of seizures in association with severe global delay. Other features include cortical visual impairment, sleep and gastro-intestinal problems. Progress in clinical understanding, especially regarding the spectrum of functional ability, seizure patterns, and other comorbidities was initially slow but accelerated in 2012 with the establishment of the International CDKL5 Database (ICDD). Our aim was to use this data source to investigate quality of life (QOL) and associated factors in this disorder. METHOD: A follow-up questionnaire was administered in 2018 to parents of children registered with the ICDD who had a pathogenic CDKL5 variant. QOL was assessed using QI Disability, an instrument, specifically developed to measure total and specific domains of QOL (physical health, positive emotions, negative emotions, social interaction, leisure and the outdoors (leisure) and independence) in children with intellectual disability. Associations with functional abilities, physical health, mental health and family factors were investigated, initially using univariate analyses followed by multivariate analyses for each of these groups with a final composite model which included the important variables identified from previous models. RESULTS: Questionnaires were returned by 129/160 families with a child aged >3 years. Functional impairment, including lack of ability to sit, use hands and communicate had the greatest adverse impact on QOL. There were also some relationships with major genotype groupings. Individuals using three or more anti-epileptic medications had poorer QOL than those on one or no medication, particularly in the physical health domain. There was also variation by geographical region with those living in North America typically having the best QOL and those living in middle or lower income countries poorer QOL. CONCLUSION: Although lower functional abilities were associated with poorer quality of life further research is needed to understand how environmental supports might mitigate this deficit. Comprehensive care and support for both the child and family have important roles to play in helping families to thrive despite the severity of CDD.

Correlation between Academic Performance and Anxiety in Medical Students of Majmaah University - KSA.

OBJECTIVE: To study the prevalence of anxiety and its correlation with the academic performance among medical students.. METHODS: The analytical cross-sectional study was conducted over six months from March 2018 to August 2018, at the male and female campuses of the College of Medicine, Majmaah University, Majmaah, Saudi Arabia, and comprised medical students of either gender. Data on anxiety was collected using a selfreporting questionnaire that included the Beck Anxiety Inventory. Academic performance was taken as a measurable record from the cumulative grade point average. Data was analysed using SPSS 24. RESULTS: Of the 247 subjects, 170(68.8%) were males. Anxiety was found in 97(39.3%) of the students. The level of anxiety was significantly higher among females compared to males (p=0.001), among those in the final year (p=0.002), and in those with low academic grades (p=0.016). CONCLUSIONS: Anxiety was found to be common among medical students and it was associated with female gender, low grades and advanced year of studies.