The burden of rheumatologic disease in Aboriginal and Torres Strait Islander Australians.

The objective of this article is to summarise the current knowledge regarding the prevalence of six rheumatological conditions in indigenous Australians - rheumatoid arthritis (RA), osteoarthritis (OA), osteoporosis (OSP), systemic lupus erythematosus (SLE), gout and musculoskeletal (MSK) pain. Online medical literature databases were searched for 'indigenous', 'Aboriginal' and 'Torres Strait Islander', as well as the names of the six conditions. Other included search terms were 'crystal', 'urate', 'arthritis' and 'arthropathy'. No limitations were placed on publication data or language. Forty-five articles examining the prevalence of the six conditions were identified. Based on the published literature, SLE appears to have a higher prevalence, while RA appears to have a lower prevalence in indigenous Australians compared to the non-indigenous community. MSK pain is prevalent, has a significant impact on indigenous people and is perceived as an important area of need. There is a paucity of data regarding these conditions in indigenous Australians. This may be impacted by the uncertainty of case ascertainment by self-report, differences in disease phenotypes and prevalence between the metropolitan compared to the rural or remote indigenous population, and difficulty with access to healthcare. Further studies in conjunction with local indigenous communities are needed to accurately determine the burden of rheumatological disease in the indigenous population. This will assist with resource and workforce planning to deliver culturally appropriate interventions. Strategies for future clinical work and research include the development and dissemination of culturally safe rheumatology resources, rheumatology training of Aboriginal Health Workers and wider integration of rheumatology clinics into community-controlled Aboriginal Health Services.

Outcome of Absence Epilepsy With Onset at 8-11 Years of Age: Watershed Ages When Syndromes Overlap.

Introduction: Absence seizures occur in various epilepsy syndromes, including childhood and juvenile absence epilepsy and juvenile myoclonic epilepsy. When children present with absence seizures at ages when syndromes overlap, initial syndrome designation is not always possible, making early prognostication challenging. For these children, the study objective is to determine clinical and initial electroencephalograph (EEG) findings to predict the development of generalized tonic-clonic seizures, which is a factor that affects outcome. Methods: Children with new-onset absence seizures between 8 and 11 years of age with at least 5 years of follow-up data were studied through the review of medical records and initial EEG tracings. Results: Ninety-eight patients were included in the study. The median age of absence seizure onset was 9 years (interquartile range [IQR] = 8.00, 10.00) and follow-up was 15 years (IQR = 13.00, 18.00). Forty-six percent developed generalized tonic-clonic seizures and 20% developed myoclonic seizures. On multiple regression analysis, a history of myoclonic seizures, anxiety, as well as bifrontal slowing and mild background slowing on initial EEG (P < .05) were associated with generalized tonic-clonic seizures. Although not statistically significant, a shorter duration of shortest EEG burst on baseline EEG was also associated with generalized tonic-clonic seizures. Conclusion: On initial EEG, bifrontal and background slowing and myoclonic seizures and anxiety are associated with developing generalized tonic-clonic seizures, which is of prognostic significance when early syndrome designation is difficult.

The disproportionately large contribution of the Maori and Pacific Islander community to the healthcare burden of gout in Western Sydney.

BACKGROUND: Gout is a common chronic inflammatory disorder due to monosodium urate deposition, which results in severe inflammatory arthritis. It is particularly common in those of Maori or Pacific Islander heritage. There is a significant number of this at-risk ethnic group in western Sydney. AIMS: To determine the healthcare burden of gout in Western Sydney. METHODS: We characterised patients managed in the emergency departments (EDs) of the four Western Sydney Local Health District (WSLHD) hospitals and those admitted for gout as the primary or secondary diagnosis from 1 January 2017 to 31 December 2018. RESULTS: There were 472 patients managed in ED on 552 occasions at a direct cost to the LHD of A$367 835. Those of Maori or Pacific Islander ethnicity comprised 25.2% (n = 119/472), while half (n = 39/80) of those managed in ED for gout on two or more occasions were of Maori or Pacific Islander ethnicity. Overall, 310 patients were admitted with gout as the principal diagnosis on 413 occasions at a cost of A$1.73 million. Seventy-five (24.2%) of the 310 patients were of Maori or Pacific Islander heritage. A total of 584 WSLHD inpatients had gout as a secondary diagnosis. This was associated with 714 admissions. CONCLUSIONS: The disproportionately large healthcare burden of gout in Western Sydney from the relatively small Maori and Pacific Islander population needs attention. Urgent culturally appropriate interventions to address gout are required to address this inequality.

Development of a Novel Prospective Model to Predict Unplanned 90-Day Readmissions After Total Hip Arthroplasty.

BACKGROUND: For hospitals participating in bundled payment programs, unplanned readmissions after surgery are often termed "bundle busters." The aim of this study was to develop the framework for a prospective model to predict 90-day unplanned readmissions after elective primary total hip arthroplasty (THA) at a macroscopic hospital-based level. METHODS: A national, all-payer, inpatient claims and cost accounting database was used. A mixed-effect logistic regression model measuring the association of unplanned 90-day readmissions with a number of patient-level and hospital-level characteristics was constructed. RESULTS: Using 427,809 unique inpatient THA encounters, 77 significant risk factors across 5 domains (ie, comorbidities, demographics, surgical history, active medications, and intraoperative factors) were identified. The highest frequency domain was comorbidities (64/100) with malignancies (odds ratio [OR] 2.26), disorders of the respiratory system (OR 1.75), epilepsy (OR 1.5), and psychotic disorders (OR 1.5), being the most predictive. Other notable risk factors identified by the model were the use of opioid analgesics (OR 7.3), Medicaid coverage (OR 1.8), antidepressants (OR 1.6), and blood-related medications (OR 1.6). The model produced an area under the curve of 0.715. CONCLUSION: We developed a novel model to predict unplanned 90-day readmissions after elective primary THA. Fifteen percent of the risk factors are potentially modifiable such as use of tranexamic acid, spinal anesthesia, and opioid medications. Given the complexity of the factors involved, hospital systems with vested interest should consider incorporating some of the findings from this study in the form of electronic medical records predictive analytics tools to offer clinicians with real-time actionable data.

Bone health assessment with dual energy X-ray absorptiometry in men with high-risk prostate carcinoma commencing adjuvant androgen deprivation therapy.

Background: Androgen deprivation therapy (ADT) is a key component of therapy for patients with high-risk prostate carcinoma, but it may be deleterious for bone health. We sought to determine the frequency of dual energy x-ray absorptiometry (DXA) scanning in patients commencing adjuvant ADT for treatment of high-risk prostate cancer at a large integrated regional cancer centre. Material and methods: The electronic medical records (EMR) of all patients with high-risk prostate carcinoma commenced on adjuvant ADT between January 1, 2016 and December 31, 2017 at the Mid-North Coast Cancer Institute, Coffs Harbour, Australia were reviewed. Patients commenced on neoadjuvant ADT and long-term suppressive ADT for metastatic disease were excluded. The following data were obtained: socio-demographic information, prostate cancer data, ADT details and DXA results. Results: 188 men (mean age ± SD, 75.4 ± 7 years) were commenced on adjuvant ADT for a total duration (mean ± SD) of 23.4 ± 7 months. Most (n = 155/188, 82%) were commenced on leuprorelin acetate. While only 26/188 (14%) had a DXA scan performed prior to ADT, another 133 (71%) had a DXA scan at a median of 20 days (interquartile range 7-98), later. Of the 159 men with DXA readings, 76 (48%) were osteopaenic and 38 (24%) were osteoporotic by DXA criteria. Conclusion: A high level (85%) of DXA scanning in men commencing ADT for prostate cancer can be achieved at a regional centre. The high prevalence (72%) of low bone mass in our unselected cohort underscores the importance of routine DXA scanning to guide bone health management during ADT.

The exercise in pulmonary arterial hypertension (ExPAH) study: A randomized controlled pilot of exercise training and multidisciplinary rehabilitation in pulmonary arterial hypertension.

Pulmonary hypertension (PH) is characterized by progressive dyspnea, fatigue, and reduced exercise capacity. Despite medical treatment, outcomes remain poor. While exercise training is well established in patients with heart failure, it is less established in patients with PH. This single-blind, randomized controlled pilot study examined the feasibility and effect of 12-week outpatient exercise (multidisciplinary rehabilitation or home walking program) on hemodynamics using cardiac magnetic resonance imaging (cMRI) and right heart catheterization (RHC) in patients with pulmonary arterial hypertension (PAH), a subset of PH. Sixteen participants were randomized to either multidisciplinary outpatient rehabilitation or a home walking program for 12 weeks. Primary outcome measures were changes in right ventricular ejection fraction and stroke volume index on cMRI. Secondary outcome measures included hemodynamics on RHC, quality of life (QOL), muscle strength (handgrip and vital capacity) and 6-min walk test. This preliminary, pilot study suggests that outpatient exercise interventions may be associated with improved hemodynamic function (mean pulmonary artery wedge pressure, stroke volume, and stroke volume index), QOL (PH symptoms, depression, and anxiety), and muscular strength (vital capacity and handgrip strength) for people with PAH, but was not adequately powered to make any formal conclusions. However, our outpatient programs were feasible, safe, and acceptable to participants. Future studies are required to further explore the potential hemodynamic benefits of exercise in PAH.

School performance in children at the time of new-onset seizures and at long-term follow-up: A retrospective cohort study.

OBJECTIVE: School-performance difficulties (SPD) are common in children with epilepsy. The objectives of this study were to determine if the rate of SPD in children with seizures change from seizure-onset to follow-up and differ from children with psychiatric disorders. METHODS: School-aged children who required an initial electroencephalography (EEG) test in 2016 were reviewed and separated into two groups based on the presence or absence of seizures. Developmental delay and SPD were compared between groups at initial assessment and SPD was assessed after 2-4 years of follow-up. Analysis was also performed on a sub-set of patients with psychiatric disorders. RESULTS: At baseline, the rate of SPD was similar between the seizure (n = 146) and non-seizure (n = 332) groups [26% vs. 27%]. At follow-up, the seizure (n = 119) group had a significantly higher rate of SPD than the non-seizure (n = 215) group (54% vs. 43%). There was no difference in the rate of SPD between the seizure (n = 119) and psychiatric (n = 69) groups at baseline (31% vs. 43%) or follow-up (54% vs. 55%). CONCLUSION: Over time, children with recurrent seizures experience more SPD than children without seizures, but similar SPD to children with psychiatric disorders.

The diagnostic accuracy of temporal artery ultrasound and temporal artery biopsy in giant cell arteritis: A single center Australian experience over 10 years.

AIM: To assess the diagnostic accuracy of temporal artery ultrasound compared with temporal artery biopsy and clinical diagnosis in patients with suspected giant cell arteritis (GCA) over 10 years in an Australian center. METHOD: Patients presenting to Westmead Hospital with possible GCA from March 2011 to December 2020 were retrospectively identified. The following parameters were obtained from the medical record: clinical presentation, inflammatory markers, temporal artery ultrasound findings, and temporal artery biopsy report. Data were assembled in a 2 × 2 table; sensitivity and specificity of temporal artery ultrasound compared with temporal artery biopsy and clinical diagnosis were calculated. RESULTS: Over the 10-year study period, 65 temporal artery ultrasounds were performed in 63 patients (n = 65; 61.9% female) with a mean ± standard deviation age of 69.6 ± 12.3 years. Thirteen out of 65 (20%) temporal artery ultrasounds had findings suggestive of GCA. Twenty patients (31.7%) had a clinical diagnosis of GCA irrespective of sonographic or biopsy findings. Compared with temporal artery biopsy, temporal artery ultrasound had a sensitivity of 71.4% and specificity of 93.3%. Compared with clinical diagnosis made by the treating rheumatologist, temporal artery ultrasound had a sensitivity of 55% and specificity of 95.3%. CONCLUSION: Temporal artery ultrasound is a useful non-invasive investigation in the assessment of suspected GCA. If positive in the setting of a suggestive clinical presentation, a temporal artery ultrasound probably avoids the need for a temporal artery biopsy. Temporal artery ultrasound could be more widely used in the clinical management of GCA.

Pediatric Occipital Spikes at a Single Center Over 26 Years and the Significance of Tangential Dipole.

BACKGROUND: Pediatric occipital epileptiform discharges occur in various clinical settings, including self-limited and treatment-resistant epilepsies. The study objective is to determine electro-clinical predictors for prognosis in children with occipital epileptiform discharges. METHODS: 205 patients with occipital epileptiform discharges were classified into seizure groups: self-limited occipital (SLO) (n = 57), including Panayiotopoulos and Gastaut syndrome; non-self-limited occipital (non-SLO) (n = 98), including various seizure etiologies; genetic-generalized (n = 18); febrile (n = 5); and no-seizure (n = 27) groups. Electro-clinical features of the SLO and non-SLO were compared, as this is of most clinical relevance. RESULTS: The median age of seizure onset was 3 years (range: 0-19). Occipital epileptiform discharges with frontal/central positivity were present in both groups, but more common in the SLO than non-SLO groups; 21/57 (36.8%) and 19/98 (19.4%), respectively (P < .022). However, when occipital epileptiform discharges with tangential dipoles (P < .048) were accompanied by abnormal ictal eye movements (P < .037), they were predictive of SLO epilepsy. CONCLUSIONS: In our cohort, occipital epileptiform discharges with tangential dipole detected by visual analysis and abnormal ictal eye movements were predictive of SLO epilepsy.

Infantile Spasms and Trisomy 21: Unfavorable Outcomes with First-line Vigabatrin Therapy.

INTRODUCTION: Among children with infantile spasms (ISs), those with trisomy 21 (T21) and those with normal development at onset and no identifiable etiology (previously referred to as "idiopathic") are expected to have relatively favorable outcomes. The study objective is to determine if differences exist in treatment response, relapse, and subsequent epilepsy between these two groups when vigabatrin is used as first-line treatment. METHODS: In this retrospective study, patients were classified into the following groups and clinical features were compared: T21 (n = 24) and IS with normal development at onset and no identified etiology (n = 40; control group). RESULTS: There was no significant difference in the age of IS onset, sex distribution, or treatment lag between the groups. The T21 compared to the control group required a higher mean number of anti-seizure therapies (3.6 vs. 1.9, p < 0.001), had more relapses [10 (42%) vs. 4 (10%), p < 0.005)], and had higher risk of subsequent epilepsy [11 (46%) vs. 8 (20%), p < 0.003]. Relapses were often delayed in the T21 group, with a mean of 8 months after IS cessation. CONCLUSION: Our results differ from most studies using steroids as first-line treatment where the groups were shown to have similar treatment response and T21 patients had a low risk of relapse and subsequent epilepsy. Therefore, our results suggest that vigabatrin as first-line treatment in T21 with IS may be less favorable than steroids.

'Tired, afraid, breathless .' An international survey of the exercise experience for people living with pulmonary hypertension.

Patients with pulmonary hypertension are more sedentary than the general population, but attitudes and experiences that may influence their exercise behaviour remain poorly understood. This study identified patterns of behaviour, attitudes towards exercise, barriers and enablers of exercise for people living with pulmonary hypertension. Accessibility of rehabilitation services from a patient perspective was also explored. A voluntary, international survey of people living with pulmonary hypertension was conducted, with mixed quantitative and qualitative data collection. Data from 187 participants in 19 countries were included in the analyses. In total, 52% (95/183) of people with pulmonary hypertension reported that they attempted to engage in regular physical activity. This was less than the proportion who did so prior to diagnosis (61%, 112/184, p = 0.006) and was accompanied by uncertainty and anxiety about exercise. In total, 63% (113/180) of the cohort reported experiencing previous adverse events while exercising, which was associated with a greater likelihood of ongoing exercise concerns and anxiety. Fear, frustration and uncertainty about exercise were noted as common barriers to engaging in exercise with pulmonary hypertension. Other barriers to exercise included intrinsic factors such as debilitating breathlessness and fatigue, and external factors such as cost and access to appropriate services. Most respondents (76%, 128/169) did not have access to a multi-disciplinary rehabilitation service, although an overwhelming majority (92%, 159/172) reported that this would be helpful. Respondents rated education; a supervised, structured exercise programme; and psychology input as the most important components of a multi-disciplinary rehabilitation service for pulmonary hypertension. Health professionals must work together with consumers to co-design rehabilitation services that will facilitate exercise and increased activity for people living with pulmonary hypertension.

Patients discharged from a fracture liaison service still require follow-up and bone health advice.

Fracture liaison services (FLSs) were established to address the well-recognised gap in bone health management after a fragility fracture. However, it is unclear what happens to patients after discharge from an FLS. Our study suggests FLSs should include a patient bone health education session and a follow-up telephone call 12-18 months post-discharge to optimise management, in particular, to assess therapy adherence and to reinforce bone health advice. PURPOSE: While fracture liaison services (FLSs) have improved bone health management following fragility fracture, it is unclear what happens to patients following discharge from these services. We sought to determine patient self-reported medication adherence and the need for bone-specific health advice ≥ 12 months following discharge from one of the first FLSs in Australia. METHODS: Patients were contacted by telephone ≥ 12 months following discharge from the Coffs Fracture Prevention Clinic (CFPC)/FLS to determine if the patient was still taking prescribed bone protective therapy (BPT). Bone health advice was provided, if appropriate, during the telephone interview. RESULTS: Of the 516 consecutive patients seen in CFPC from July 2012-December 2018, 326 (63.2%) were assessed and discharged from the clinic. One hundred and two patients (19.8%) were lost to follow-up/uncontactable. Of 190 patients commenced on BPT at CFPC and who were discharged ≥ 12 months prior, 141 (74.2%) self-reported adherence with BPT. Bone health advice was required during the telephone call in 60/190 (31.6%) of these patients. Of the 141 adherent patients, 40 (28.4%) had attended a bone health education session, compared to 4/49 (8.2%) patients in the non-adherent group (p = 0.004). CONCLUSION: At 19 months following discharge from our FLS, self-reported adherence with treatment was 74%. One bone health education session at baseline was associated with increased treatment adherence. At time of telephone contact, one third of patients required further advice to optimise bone health.

Attitudes towards exercise among medical specialists who manage patients with pulmonary hypertension.

Exercise training was not traditionally recommended for patients with pulmonary hypertension. However, recent work has demonstrated that exercise improves endurance and quality-of-life in patients with pulmonary hypertension. Unfortunately, patients with pulmonary hypertension are often sedentary. While some studies have examined patient attitudes to exercise, none have investigated physician perspectives on exercise in patients with pulmonary hypertension. This multinational survey of physicians involved in treating patients with pulmonary hypertension sought to ascertain physician attitudes to exercise and physician-identified barriers and enablers of exercise in this patient population. We collected cross-sectional survey data from a cohort of 280 physicians, including rehabilitation physicians, cardiologists, respiratory physicians and rheumatologists. We found that overall, 86% physicians recommended exercise, in line with current guidelines, although there were differences in the rationale for prescribing exercise and in the type of exercise prescription. Barriers to exercise included patient-related factors, such as patient ill health preventing exercise; poor patient motivation and lack of understanding regarding the benefits of exercise. Systemic barriers included cost/funding issues and limited availability of appropriate services. Perceived enablers of exercise included access to appropriate programmes, provision of education and supportive treating clinicians. Further research is required to identify and implement interventions to promote physical activity in patients with pulmonary hypertension.

The utility of dual energy computed tomography in the management of axial gout: case reports and literature review.

BACKGROUND: Severe spinal pain is an unusual presentation of gout. Due to its rarity and the difficulty of obtaining joint fluid or tissue for crystal analysis, dual energy computed tomography (DECT) may be a useful imaging modality in the management of axial gout. CASE PRESENTATION: Two patients independently presented to a major teaching hospital with severe spinal pain subsequently shown to be due to gout. The first patient presented with back pain and fevers and was initially thought to have lumbar facet joint septic arthritis. The second case presented with severe back pain. In both cases, DECT suggested monosodium urate deposition in spinal tissues as the cause of their presentation. CONCLUSIONS: Axial gout should be considered in the differential diagnosis of severe spinal pain. A DECT study may be a useful diagnostic tool in the management of spinal gout.

Clinical Significance of Incidental Rolandic Spikes in Children With Absence Epilepsy.

PURPOSE: Absence epilepsy and benign epilepsy of childhood with central temporal spikes are common childhood epilepsy syndromes. Although 3-Hz generalized spike-wave discharges are almost always associated with absence seizures, rolandic spikes can be present in individuals without rolandic seizures. The co-occurrence of 3-Hz generalized spike-wave and rolandic spikes is very rare. Our objective was to compare clinical features of patients with absence epilepsy with and without rolandic spikes, to determine if the additional feature of rolandic spikes has any clinical significance. METHODS: Clinical information of 17 children with absence epilepsy and rolandic spikes was compared to an age-matched control group of 90 children with absence epilepsy. RESULTS: Although most patients had excellent seizure control at follow-up, epilepsy comorbidities (cognitive and emotional problems) were observed. Comparing study vs control groups, there was no difference with anxiety (2 [11.8%] vs 8 [9%]), behavioral issues (4 [23.5%] vs 10 [11%]), mood disorders (0 vs 2 [2%]), and attention-deficit hyperactivity disorder (4 [24%] vs 10 [11%]). Significant differences were also observed: more global-developmental (5 [29%] vs 5 [6%], P < .009) and expressive-language (4 [24%] vs 5 [6%], P < .034) delay and more difficulties with school performance (11 [65%] vs 32 [36%], P < .025), especially with language-related tasks (6 [35%] vs 5 [6%], P < .001). CONCLUSION: Our results confirm the presence of additional epilepsy comorbidities in patients with absence epilepsy when rolandic spikes are present. Rolandic spikes in patients with absence epilepsy may be a marker of additional cognitive challenges that physicians should be aware of.

Assessing the readability and patient comprehension of rheumatology medicine information sheets: a cross-sectional Health Literacy Study.

OBJECTIVES: Patients are often provided with medicine information sheets (MIS). However, up to 60% of patients have low health literacy. The recommended readability level for health-related information is ≤grade 8. We sought to assess the readability of MIS given to patients by rheumatologists in Australia, the UK and Canada and to examine Australian patient comprehension of these documents. DESIGN: Cross-sectional study. SETTING: Community-based regional rheumatology practice. PARTICIPANTS: Random sample of patients attending the rheumatology practice. OUTCOME MEASURES: Readability of MIS was assessed using readability formulae (Flesch Reading Ease formula, Simple Measure of Gobbledygook scale, FORCAST (named after the authors FORd, CAylor, STicht) and the Gunning Fog scale). Literal comprehension was assessed by asking patients to read various Australian MIS and immediately answer five simple multiple choice questions about the MIS. RESULTS: The mean (±SD) grade level for the MIS from Australia, the UK and Canada was 11.6±0.1, 11.8±0.1 and 9.7±0.1 respectively. The Flesch Reading Ease score for the Australian (50.8±0.6) and UK (48.5±1.5) MIS classified the documents as 'fairly difficult' to 'difficult'. The Canadian MIS (66.1±1.0) were classified as 'standard'. The five questions assessing comprehension were correctly answered by 9/21 patients for the adalimumab MIS, 7/11 for the methotrexate MIS, 6/28 for the non-steroidal anti-inflammatory MIS, 10/11 for the prednisone MIS and 13/24 for the abatacept MIS. CONCLUSIONS: The readability of MIS used by rheumatologists in Australia, the UK and Canada exceeds grade 8 level. This may explain why patient literal comprehension of these documents may be poor. Simpler, shorter MIS with pictures and infographics may improve patient comprehension. This may lead to improved medication adherence and better health outcomes.