Factors Associated with Stroke Coding Quality: A Comparison of Registry and Administrative Data.

BACKGROUND: The International Statistical Classification of Diseases and Related Health Problems, 10th Revision, Australian Modification (ICD-10-AM) codes are commonly used to identify patients with diseases or clinical conditions for epidemiological research. We aimed to determine the diagnostic agreement and factors associated with a clinician-assigned stroke diagnosis in a national registry and the ICD-10-AM codes recorded in government-held administrative data. MATERIALS AND METHODS: Data from 39 hospitals (2009-2013) participating in the Australian Stroke Clinical Registry (AuSCR) were linked and merged with person-level administrative data. The AuSCR clinician-assigned stroke diagnosis was the reference standard. Concordance was defined as agreement between the clinician-assigned diagnosis and the ICD-10-AM codes for acute stroke or transient ischemic attack (TIA) (ICD-10-AM codes: I61-I64, G45.9). Multivariable logistic regression was undertaken to assess factors associated with coded diagnostic concordance. RESULTS: A total of 14,716 patient admissions were included (46% female, 63% ischemic, 14% intracerebral hemorrhage [ICH], 18% TIA and 5% unspecified stroke based on the reference standard). Principal ICD-10-AM code concordance was ICH: 76.7%; ischemic stroke: 72.2%; TIA: 80.2%; unspecified stroke: 50.8%. Factors associated with a greater odds of ischemic stroke concordance included: treatment in a stroke unit (adjusted Odds Ratio, aOR:1.58; 95% confidence interval (CI) 1.37, 1.82); length of stay >4 days (aOR:1.30; 95% CI 1.17, 1.45); and discharge destination other than home (Residential care aOR:1.57; 95% CI 1.24, 1.96; Inpatient rehabilitation aOR:1.63; 95% CI 1.43, 1.86). CONCLUSIONS: Diagnostic concordance varied based on stroke type. Future research to improve the quality of coding for stroke should focus on patients not treated in stroke units or with shorter lengths of stay where documentation in medical records may be limited.

Genetics and pediatric hospital admissions, 1985 to 2017.

PURPOSE: To determine the prevalence and sociodemographic and hospitalization history of genetic conditions in a sample of inpatients in a pediatric hospital in 2017, and to compare results with unpublished studies from 1985, 1995, and 2007. METHODS: Two weeks of admissions were classified according to a pre-existing categorization, based on genetic etiology, encompassing chromosomal and monogenic conditions, multifactorial (MF) conditions, and no known genetic cause. RESULTS: In 2017, 299 (16%) patients had chromosomal or monogenic conditions, 6-7% more than 2007 and 1995, but similar to 1985. Autosomal dominant (AD) conditions increased from <2% previously to 6% in 2017 (p < 0.001). MF conditions comprised the majority throughout, increasing from 45% to 54%. Age at admission was highest in autosomal recessive (AR) and X-linked categories in 1995, 2007, and 2017, reflected in their high number of previous admissions, while the AD, MF, and nongenetic categories were the youngest with similar lengths of stay and previous admissions. CONCLUSION: Conditions with a genetic contribution account for over half of pediatric inpatients. Since 1985, there have been many changes in age at admission and length of stay, but it is the increasing prevalence of AR, AD, and MF conditions that is important when considering future service provision.

Work-integrated learning for health information management students: Lessons learned from literature, and experiences of supervisors and students on virtual or remote placements.

BACKGROUND: Traditionally, health placements have required practical in-person learning, including placements completed by health information management (HIM) students. COVID-19 made in-person healthcare placements largely unviable. Alternative virtual/remote placements were required. AIMS: (1) Explore the experiences of virtual/remote placements for HIM students and their supervisors; (2) Compare these experiences to the literature on barriers and facilitators for virtual/remote placement and (3) Develop best practice guidelines for the delivery of virtual/remote placements for HIM students. METHOD: A cross-sectional survey asked final-year HIM placement students and their supervisors about their virtual/remote placement experiences in 2021. Survey findings were compared to 10 barriers and facilitators for remote/virtual placements identified in the literature. RESULTS: Students were challenged by autonomous virtual/remote placements but enjoyed their flexibility. A work schedule provides placement structure to students. The use of technology was embraced although unfamiliarity with video-conferencing software prior to placement was an issue for some students. The most common method of student-supervisor communication was email. However, students missed casual corridor conversations. The importance of maintaining a community of practice was reported in the literature and confirmed by students. Most students preferred undertaking a virtual/remote placement rather than delaying graduation. The majority of supervisors reported complete satisfaction with the placement students' performance. CONCLUSION: Virtual/remote placements were a viable option for HIM students when in-person placements were impossible. Students required a work-based schedule, appropriate information technology, a dedicated workspace, familiarity with communication technologies, good communication channels with their supervisors and a supportive 'community of practice'. HIM supervisors were satisfied with virtual/remote methods of placement delivery.

The applications of Australian-coded ICD-10 and ICD-10-AM data in research: A scoping review of the literature.

Background: Australia uses the International Classification of Diseases (ICD-10) for mortality coding and its Australian Modification, ICD-10-AM, for morbidity coding. The ICD underpins surveillance (population health, mortality), health planning and research (clinical, epidemiological and others). ICD-10-AM also supports activity-based funding, thereby propelling realignment of the foci of clinical coding and, potentially, coded data's research utility. Objective: To conduct a scoping review of the literature exploring the use of ICD-10 and ICD-10-AM Australian-coded data in research. Research questions addressed herein: (1) What were the applications of ICD-10(-AM) Australian-coded data in published peer-reviewed research, 2012-2022? (2) What were the purposes of ICD-10(-AM) coded data within this context, as classified per a taxonomy of data use framework? Method: Following systematic Medline, Scopus and Cumulative Index to Nursing and Allied Health Literature database searches, a scoping literature review was conducted using PRISMA Extension for Scoping Reviews guidelines. References of a random 5% sample of within-scope articles were searched manually. Results were summarised using descriptive analyses. Results: Multi-stage screening of 2103 imported articles produced 636, including 25 from the references, for extraction and analysis; 54% were published 2019-2022; 50% within the largest five categories were published post-2019; 22% fell within the "Mental health and behavioural" category; 60.3% relied upon an ICD-10 modification. Articles were grouped by: research foci; relevant ICD chapter; themes per the taxonomy; purposes of the coded data. Observational study designs predominated: descriptive (50.6%) and cohort (34.6%). Conclusion: Researchers' use of coded data is extensive, robust and growing. Increasing demand is foreshadowed for ICD-10(-AM) coded data, and HIM-Coders' and Clinical Coders' expert advice to medical researchers.

Researchers' perceptions of the trustworthiness, for reuse purposes, of government health data in Victoria, Australia: Implications for policy and practice.

In 2022 the Australian Data Availability and Transparency Act (DATA) commenced, enabling accredited "data users" to access data from "accredited data service providers." However, the DATA Scheme lacks guidance on "trustworthiness" of the data to be utilised for reuse purposes. Objectives: To determine: (i) Do researchers using government health datasets trust the data? (ii) What factors influence their perceptions of data trustworthiness? and (iii) What are the implications for government and data custodians? Method: Authors of published studies (2008-2020) that utilised Victorian government health datasets were surveyed via a case study approach. Twenty-eight trust constructs (identified via literature review) were grouped into data factors, management properties and provider factors. Results: Fifty experienced health researchers responded. Most (88%) believed that Victorian government health data were trustworthy. When grouped, data factors and management properties were more important than data provider factors in building trust. The most important individual trust constructs were: "compliant with ethical regulation" (100%) and "monitoring privacy and confidentiality" (98%). Constructs of least importance were knowledge of "participant consent" (56%) and "major focus of the data provider was research" (50%). Conclusion: Overall, the researchers trusted government health data, but data factors and data management properties were more important than data provider factors in building trust. Implications: Government should ensure the DATA Scheme incorporates mechanisms to validate those data utilised by accredited data users and data providers have sufficient quality (intrinsic and extrinsic) to meet the requirements of "trustworthiness," and that evidentiary documentation is provided to support these "accredited data."

The knowledge and reuse practices of researchers utilising government health information assets, Victoria, Australia, 2008-2020.

BACKGROUND: Using government health datasets for secondary purposes is widespread; however, little is known on researchers' knowledge and reuse practices within Australia. OBJECTIVES: To explore researchers' knowledge and experience of governance processes, and their data reuse practices, when using Victorian government health datasets for research between 2008-2020. METHOD: A cross-sectional quantitative survey was conducted with authors who utilised selected Victorian, Australia, government health datasets for peer-reviewed research published between 2008-2020. Information was collected on researchers': data reuse practices; knowledge of government health information assets; perceptions of data trustworthiness for reuse; and demographic characteristics. RESULTS: When researchers used government health datasets, 45% linked their data, 45% found the data access process easy and 27% found it difficult. Government-curated datasets were significantly more difficult to access compared to other-agency curated datasets (p = 0.009). Many respondents received their data in less than six months (58%), in aggregated or de-identified form (76%). Most reported performing their own data validation checks (70%). To assist in data reuse, almost 71% of researchers utilised (or created) contextual documentation, 69% a data dictionary, and 62% limitations documentation. Almost 20% of respondents were not aware if data quality information existed for the dataset they had accessed. Researchers reported data was managed by custodians with rigorous confidentiality/privacy processes (94%) and good data quality processes (76%), yet half lacked knowledge of what these processes entailed. Many respondents (78%) were unaware if dataset owners had obtained consent from the dataset subjects for research applications of the data. CONCLUSION: Confidentiality/privacy processes and quality control activities undertaken by data custodians were well-regarded. Many respondents included data linkage to additional government datasets in their research. Ease of data access was variable. Some documentation types were well provided and used, but improvement is required for the provision of data quality statements and limitations documentation. Provision of information on participants' informed consent in a dataset is required.

Defining career success: A cross-sectional analysis of health information managers' perceptions.

BACKGROUND: Career success can be defined as the accomplishment of desirable outcomes in an individual's work experiences. It can be divided into objective and subjective career success. Objective success refers to tangible and measurable outcomes such as promotions and position titles. Subjective career success relates to an individual's interpretations of their success or accomplishments. The career success of health information management professionals has not been explored in the literature. AIM: To determine the indicators of career success as reported by health information managers (HIMs) and identify whether there are any differences based on length of time in the profession. METHODS: Using a cross-sectional study design, an online survey was administered to a sample of La Trobe University and Lincoln Institute of Health Sciences Medical Record Administration and Health Information Management graduates from 1985, 1995, 2005 and 2015, which included the following question: "How would you define success in your career?" RESULTS: Almost 88% (n = 63) of overall participants in the study responded to this item. Subjective factors (n = 77) of career success, compared to objective factors (n = 22), were more common. The categories of recognition (feeling valued/appreciated), job satisfaction and feelings of accomplishment/sense of achievement were commonly reported. DISCUSSION: Subjective factors of an individual's career success were deemed to be more significant than objective factors among HIMs. CONCLUSION: Factors such as recognition and appreciation at work, job satisfaction, fostering high-quality work outputs and creating a sense of achievement should be the major foci for managers, organisations and individuals.

The suitability of government health information assets for secondary use in research: A fit-for-purpose analysis.

BACKGROUND: Governments have responsibility for ensuring the quality and fitness-for-purpose of personal health data provided to them. While these health information assets are used widely for research, this secondary usage has received minimal research attention. OBJECTIVE: This study aimed to investigate the secondary uses, in research, of population health and administrative datasets (information assets) of the Department of Health (DoH), Victoria, Australia. The objectives were to (i) identify research based on these datasets published between 2008 and 2020; (ii) describe the data quality studies published between 2008 and 2020 for each dataset and (iii) evaluate "fitness-for-purpose" of the published research. METHOD: Using a modified scoping review, research publications from 2008 to 2020 based on information assets related to health service provision and containing person-level data were reviewed. Publications were summarised by data quality and purpose-categories based on a taxonomy of data use. Fitness-for-purpose was evaluated by comparing the publicly stated purpose(s) for which each information asset was collected, with the purpose(s) assigned to the published research. RESULTS: Of the >1000 information assets, 28 were utilised in 756 publications: 54% were utilised for general research purposes, 14% for patient safety, 10% for quality of care and 39% included data quality-related publications. Almost 85% of publications used information assets that were fit-for-purpose. CONCLUSION: The DoH information assets were used widely for secondary purposes, with the majority identified as fit-for-purpose. We recommend that data custodians, including governments, provide information on data quality and transparency on data use of their health information assets.

A documentary analysis of Victorian Government health information assets' websites to identify availability of documentation for data sharing and reuse in Australia.

BACKGROUND: Health data sharing is important for monitoring diseases, policy and practice, and planning health services. If health data are used for secondary purposes, information needs to be provided to assist in reuse. OBJECTIVES: To review government health information asset websites to ascertain the extent of readily available, explanatory documentation for researcher sharing and reuse of these data. METHOD: Documentary analysis was undertaken on selected Victorian Government health information assets' websites in Australia. Data were obtained on nine information-categories: data custodian; data context; data dictionary; quality controls; data quality; limitations; access process; privacy/confidentiality/security and research requests/outputs. Information-categories were compared by dataset type (administrative or population-health) and by curating organisation (government or other agency). Descriptive statistics were used. RESULTS: The majority of the 25 websites examined provided information on data custodian (96%) and data context (92%). Two-thirds reported access process (68%) and privacy/confidentiality/security information (64%). Compared with population-health websites, administrative dataset websites were more likely to provide access to a data dictionary (67% vs 50%) and information on quality controls (56% vs 44%), but less likely to provide information on the access process (56% vs 75%) and on research requests/outputs (0% vs 56%, p = 0.024). Compared with government-curated websites, other agency websites were more likely to provide information on research requests/outputs (80% vs 7%, p < 0.001). CONCLUSION: There is inconsistent explanatory documentation available for researchers for reuse of Victorian Government health datasets. Importantly, there is insufficient information on data quality or dataset limitations. Research-curated dataset websites are significantly more transparent in displaying research requests or outputs.

Workforce survey of Australian health information management graduates, 2017-2021: A 5-year follow-on study.

Background: Employment outcomes of La Trobe University's 2012-2016 health information manager (HIM) graduate cohort were reported previously. Objectives: To identify the 2017-2021 Australia-based, graduate HIMs' early career employment experiences; identify employment roles and destinations; investigate knowledge and skill sets utilised in professional performance; and compare outcomes with the previous study. Method: A cross-sectional design was utilised. An online survey elicited: demographic data, position-related details and knowledge-skills applied in the workplace. Inter- and intra-cohort comparisons were calculated. Results: Of contactable graduates, 75% (n = 150) completed the survey; 90% (n = 132) had held at least one profession-related position postgraduation; 51% gained employment before final examinations and 92% within 6 months. In their first role, 87% joined the public healthcare sector, 47% had worked in two or more positions and 12.3% in three or more positions. Categorisation of position titles showed that 40% had undertaken "health information management" roles, 14.9% "health classification," 16.6% "data management and analytics," 17.4% "health ICT" and 11.1% "other," roles. Almost two-thirds (64.1%) had utilised three or four of the four professional knowledge-skill domains. There was an increase, from the 2012 to 2016 cohort, in those undertaking "data management and analytics" and "health ICT" roles, and a decrease in "health classification" role uptake. Conclusion: Early-career HIMs have very high employability. They engage throughout health care, predominately in the public health sector. Their mobility reflects national workforce trends. The majority utilise all or most of the professional knowledge-skill domains studied at university.

Professional identity and workplace motivation: A case study of health information managers.

BACKGROUND: The professional identity and motivation of qualified health information managers (HIMs) is largely unexplored. OBJECTIVES: A larger study has investigated the motivators of HIMs in the construction of their professional identity and associated relationships to job satisfaction and engagement with their profession. The aims of this component of the study were to: (i) identify and analyse the characteristics of members of the profession who have different motivation profiles; (ii) obtain HIMs' perspectives on their professional identity; and (iii) measure correlation between HIMs' professional identity and different motivating factors. Method: A cross-sectional study design, with a convergent mixed-methods approach to data collection was employed. An online survey was administered to the 1985, 1995, 2005 and 2015 Australian health information management and medical record administration graduate cohorts from one university in Victoria. RESULTS: Response rate: 72.7% (n = 72). There were no statistically significant correlations between the HIMs' motivation profile and professional identity. The HIMs were largely motivated by a need for achievement (striving for excellence) and continuous improvement; maintained high standards of work quality (95.8%); valued their work (94.4%) and work collaborations (84.7%); satisfactorily applied skills-knowledge (94%); demonstrated a very strong professional association (92% were proud to belong to the profession). Key factors in motivation that were consistently reported by members of all cohorts in the open-ended questions were as follows: intrinsic motivation; colleagues and teamwork; the variety of work performed; and contribution to the bigger picture. Overall, and notwithstanding between-cohort differences: 65.3% confidently directed others, 45.8% aspired to leadership and 38% actively networked. They related difficulty in explaining the profession to outsiders. CONCLUSION: There was no correlation between motivation profile and professional identity. Significantly, the HIMs demonstrated exceptionally strong positive professional identity, reflected particularly in pride in membership of the profession and their belief in the importance of their professional work.

An investigation of the status and maturity of hospitals' health information governance in Victoria, Australia.

BACKGROUND: Health information governance (IG) in Australian hospitals was hitherto unexplored. OBJECTIVES: To determine hospitals' health IG status and maturity in Victoria, Australia, identify drivers and barriers affecting IG adoption, examine electronic health data breach response plan usage and assess employees' electronic data breach awareness. METHOD: Mixed-methods descriptive study utilising an online survey of directors - clinical/health information services and chief health information managers (HIMs) in Victorian hospitals, ≥50 beds. RESULTS: Response rate: 42.9% (n = 36). Fifty percent (n = 17) of respondent-hospitals had an IG program. IG equally supported decision-making and risk identification and prevention. The greatest potential organisational damages from system disruption or failure were information loss (66.7%) and clinical risks (63.9%). HIMs in 15 (55.6%) hospitals had knowledge to monitor and detect electronic data breaches. Staff in 19 (70.4%) hospitals knew who to inform about a suspected breach. Most hospitals had mature health information-related IG practices, most (88.9%, n = 24) provided IG-related education, 77.8% (n = 21) regularly reviewed data breach response plans. The strongest IG drivers were privacy-security compliance and changes to data capture or documentation practices (82.8%, n = 24); the greatest barriers were implementation complexity (57.1%, n = 16) and cost (55.6%, n = 15). CONCLUSION: These baseline Australian data show 50% of respondent-hospitals had no formal health IG program. Privacy-security compliance, and audits, needed improvement; however, most hospitals had well-developed medical record/health information IG-relevant schedules, policies and practices. HIMs, the professionals most engaged in IG, required upskilling in electronic data breach detection.

Workforce survey of Australian graduate health information managers: Employability, employment, and knowledge and skills used in the workplace.

BACKGROUND: Employability, employment destinations and utilisation of knowledge-skill domains of new graduate health information managers (HIMs) have not been explored. OBJECTIVES: To capture the timing from course completion to employment and employment locations of a 5-year cohort of health information management graduates of La Trobe University, Australia, in 2017-2018; identify professional knowledge and skills used by the graduates in executing their roles; and map these to four domains of the health information management curriculum. METHOD: A mixed-methods descriptive study utilising a survey investigated early career pathways of new graduates of health information management courses from 2012 to 2016. Demographic data included age, year of graduation, lead time from course completion to employment, position title, number of positions held post-graduation and knowledge-skills used in the workplace. RESULTS: Eighty percent (n = 167) of graduates working in Australia with known contact details responded to the survey. Of these, 96.4% (n = 161) worked in at least one "health information management-related" position since graduation. Forty-five percent (n = 72) of graduates obtained a position before course completion, and over 94% (n = 150) were employed in the profession within 6 months of completion. Sixty percent (n = 97) of graduates had worked in two or more positions from 2012 to 2016. The large majority of new graduate HIMs (82.4%) were employed in the public healthcare sector in "health information management" (44%), or "health classification" (28.1%) roles. Most graduates (61%) had utilised at least three or four domains of professional knowledge-skills in the workplace. Whereas 16% (n = 26) of graduates used, solely, their health classification knowledge-skill set, almost 74% (n = 117) undertook some health classification-related activities. Only 16% (n = 26) of graduates were over 40 years of age, and there were no statistically significant differences between Bachelor and Master (Combined Degree Programs) graduates and Graduate-entry Master degree graduates in terms of lead time to employment, number of positions held, type of employing agency and professional knowledge-skills utilised in the workplace. CONCLUSION: Graduate HIMs have very high employability, demonstrate job mobility consistent with the national trends, are largely represented in the public sector but have presence throughout the healthcare system and utilise most or all of the specialised domains of professional knowledge and skills studied at university.

Is Down syndrome a disappearing birth defect?

OBJECTIVE: To assess trends in the prevalence of Down syndrome (DS) from 1986 to 2004 in Victoria, Australia (population approximately 5 million). STUDY DESIGN: The Victorian Birth Defects Register and the Prenatal Diagnosis Database were linked to ascertain all cases of DS. Total and birth prevalence estimates were calculated per year and presented as 3-year moving averages. RESULTS: The total number of cases of DS increased from 113 in 1986 to 188 in 2004. The number of births declined over the first decade of the study, particularly in younger women, but total numbers have fluctuated between 45 and 60 births since 1996. In women under age 35 years, total prevalence was 10/10,000 until 1997 and then increased to 12.5/10,000. In older women, total prevalence increased from 70/10,000 to 90/10,000 in this time frame. Birth prevalence declined at first but remained relatively stable in the later years of the study. The proportion of cases diagnosed prenatally increased from 3% to 60% in younger women. CONCLUSIONS: Our findings demonstrate the continuing need to devote resources to support individuals with DS and their families.

Frequency of holoprosencephaly in the International Clearinghouse Birth Defects Surveillance Systems: searching for population variations.

BACKGROUND: Holoprosencephaly (HPE) is a developmental field defect of the brain that results in incomplete separation of the cerebral hemispheres that includes less severe phenotypes, such as arhinencephaly and single median maxillary central incisor. Information on the epidemiology of HPE is limited, both because few population-based studies have been reported, and because small studies must observe a greater number of years in order to accumulate sufficient numbers of births for a reliable estimate. METHODS: We collected data from 2000 through 2004 from 24 of the 46 Birth Defects Registry Members of the International Clearinghouse for Birth Defects Surveillance and Research. This study is based on more than 7 million births in various areas from North and South America, Europe, and Australia. RESULTS: A total of 963 HPE cases were registered, yielding an overall prevalence of 1.31 per 10,000 births. Because the estimate was heterogeneous, possible causes of variations among populations were analyzed: random variation, under-reporting and over-reporting bias, variation in proportion of termination of pregnancies among all registered cases and real differences among populations. CONCLUSIONS: The data do not suggest large differences in total prevalence of HPE among the studied populations that would be useful to generate etiological hypotheses.

Estimating the prevalence of fetal alcohol syndrome in Victoria using routinely collected administrative data.

OBJECTIVE: To establish the prevalence of fetal alcohol syndrome (FAS) in Victoria through the Victorian Birth Defects Register (VBDR). METHODS: A sample of live births from 1995-2002 was selected from the Victorian Perinatal Data Collection and VBDR based on reported microcephaly, FAS or maternal use of alcohol during pregnancy. Following ethics approval, medical records of mother and child were requested for 117 births. One hundred and nine of these were accessed and examined for factors related to FAS. Records were categorised as FAS, possible FAS, unable to categorise, or not FAS. RESULTS: From the VBDR the prevalence was calculated at 0.006 per 1,000 live births. Four additional possible cases of FAS increased this to 0.014 per 1,000 live births. Six cases were defined as 'unable to categorise' as alcohol use was unknown but other features of FAS were evident. Including these cases, plus five where some low-level alcohol use was reported, increased the prevalence to 0.03 per 1,000 live births. Twenty-eight per cent of the audit population and 39% of the microcephalic cases had no information about maternal alcohol use recorded in the antenatal or babies' records. CONCLUSION AND IMPLICATIONS: The audit of medical records provided additional information regarding FAS prevalence in Victoria. This prevalence ranges from 0.01 to 0.03 per 1,000 live births. To accurately assess the extent of the problem, there needs to be improved reporting of alcohol use in pregnancy and a system in place to report cases diagnosed during and beyond the perinatal period to the VBDR.

Methodological processes in validating and analysing the quality of population-based data: a case study using the Victorian Perinatal Data Collection.

This paper describes methods used and results obtained from a study that measured the accuracy of a routinely collected population-based data set. Data on a random sample of births were extracted from the 2003 Victorian Perinatal Data Collection (VPDC) and compared with information in the original medical record. Accuracy was calculated for 111 items related to diverse aspects of maternity and neonatal health and care. Sensitivity, specificity, positive predictive value and negative predictive value were calculated for dichotomous items. Seventynine items were accurate in at least 97% of cases, 45 of them in at least 99% of cases, and accuracy was below 90% for five items. Very high specificities demonstrate that conditions were rarely reported in error. Lower sensitivities indicate that some events that occurred went unreported on the perinatal form. The excellent results for specifi city indicated that the dataset is appropriate for a conservative analysis of relationships between factors. The lower sensitivities could result in true relationships between factors remaining unidentified. Reasons for discrepancies between the VPDC and the original medical record are described.

Has prenatal screening influenced the prevalence of comorbidities associated with Down syndrome and subsequent survival rates?

OBJECTIVES: With this study we aimed to compare survival rates for children with Down syndrome in 2 time periods, 1 before prenatal screening (1988-1990) and 1 contemporaneous with screening (1998-2000), and to examine the frequency of comorbidities and their influence on survival rates. METHODS: Record-linkage was performed between the population-based Victorian Birth Defects Register and records of deaths in children up to 15 years of age collected under the auspice of the Consultative Council on Obstetric and Pediatric Mortality and Morbidity. Cases of Down syndrome were coded according to the presence or absence of comorbidities by using the International Classification of Diseases, Ninth Revision classification of birth defects. Kaplan-Meier survival functions and log rank tests for equality of survival distributions were performed. RESULTS: Of infants liveborn with Down syndrome in 1998-2000, 90% survived to 5 years of age, compared with 86% in the earlier cohort. With fetal deaths excluded, the proportion of isolated Down syndrome cases in the earlier cohort was 48.7% compared with 46.1% in the most recent cohort. In 1988-1990 there was at least 1 cardiac defect in 41.1% of cases and in 45.4% in 1998-2000. There was significant variation in survival rates for the different comorbidity groupings in the 1988-1990 cohort, but this was not so evident in the 1998-2000 cohort. CONCLUSIONS: Survival of children with Down syndrome continues to improve, and there is an overall survival figure of 90% to at least 5 years of age. It is clear from this study that prenatal screening technologies are not differentially ascertaining fetuses with Down syndrome and additional defects, because there has been no proportional increase in births of isolated cases with Down syndrome.

Registry- and clinic-based analyses of birth defects and syndromes associated with cleft lip/palate in Victoria, Australia.

OBJECTIVE: To study the birth defects and syndromes associated with cleft lip and/or cleft palate in children born from 2000 through 2002 in Victoria, Australia, comparing data from the birth defects registry and detailed clinical assessment. DESIGN, SETTING, AND PARTICIPANTS: Data recorded in the Victorian Birth Defects Register were retrieved for all children with cleft lip and/or palate born from 2000 through 2002. In parallel, a cohort of children with clefts was recruited from the two cleft centers in Victoria. Clinical data were collected using structured parental interview, clinical/dysmorphologic examination, and file review. RESULTS: Victorian Birth Defects Register records of 312 children with cleft lip and/or palate were identified, and 53 children were recruited for the clinical study. The clinical study found a higher proportion of nonisolated clefts than were listed in the registry; this was largely due to the more detailed assessment, but some selection bias was possible. Poor growth and developmental delay were most likely to predict the presence of other birth defects or a syndrome diagnosis in a child with cleft lip and/or palate. The clinical study led to modifications to 16/53 (30.2%) of records in the Victorian Birth Defects Register. CONCLUSIONS: This study provides complementary registry- and clinic-based data on cleft lip and/or palate-associated malformations and syndrome diagnoses in Australian children and emphasizes the value of having a clinician experienced in dysmorphology involved in cleft services with ongoing reporting to the Victorian Birth Defects Register.

What has happened with neural tube defects and womens' understanding of folate in Victoria since 1998?

OBJECTIVE: To describe the prevalence of neural tube defects (NTDs) in Victoria, and to evaluate women's knowledge and awareness of the importance of folate after the introduction of voluntary food fortification. DESIGN AND SETTING: Descriptive study, set in Victoria, Australia, based on routinely collected data from the Victorian Birth Defects Register (VBDR) for 1998-2006, and responses by women aged 18-50 years to five questions relating to folate on the 2005 and 2006 Victorian Population Health Surveys (2314 and 2488 women, respectively). MAIN OUTCOME MEASURES: Prevalence of NTDs, and extent of women's knowledge of the importance of folate in NTD prevention, comparing the period before and since voluntary food fortification and a folate awareness campaign. RESULTS: The total prevalence of pregnancies affected by NTDs declined from approximately 17 to 14 per 10,000 births from 1997 to 1999 (coinciding with the period when voluntary food fortification was introduced, and a 1-year folate awareness campaign was held). It has since remained static. Over the 9-year study period, the termination of pregnancy rate was 79%, resulting in three NTD-affected babies per 10,000 livebirths. Compared with women aged 30-34 years (the reference group), those aged 20-24 years had the greatest likelihood of having a baby with an NTD (adjusted odds ratio, 1.70; 95% CI, 1.33-2.18; P < 0.001). Women aged 18-24 years had the lowest rate of folate supplement use (15.9% in 2006), while women aged 30-34 years had the highest rate (30.3% in 2006). CONCLUSIONS: There has been no further reduction in prevalence of NTDs in Victoria since 1999, and this prevalence remains well above that achievable through adequate folate intake. Accurate knowledge of folate consumption, population-based NTD prevalence data and folate awareness data are essential in monitoring the effectiveness of the mandatory fortification program to be implemented in Australia in the next 2 years.