Sarcoidosis: a prospective observational cohort from Northern Alberta.

INTRODUCTION: Sarcoidosis is a multi-system disease reported to occur with a higher incidence in Alberta than many other health jurisdictions within and outside of Canada. The reasons for this higher incidence are currently not known. Exposure to beryllium can result in a clinically and radiologically identical disease to sarcoidosis. The purpose of our study was to identify patterns with potential occupational or environmental exposures to beryllium amongst individuals with sarcoidosis in Alberta through a tertiary referral center. METHODS: A prospective observational study was carried out at the University of Alberta Hospital. Patients with confirmed sarcoidosis (stages 0-4) were recruited from subspecialty clinics (Respirology, Cardiology, Neurology and Occupational Health). A predetermined list of industries thought to involve potentially relevant exposures for the development of sarcoidosis was used to capture current and previous exposure history. Results were entered into a database and where possible verified by comparing with existing electronic medical records (including histories, physical examination, diagnostic imaging and physiology). RESULTS: A total of 45 patients were recruited, 25 men and 20 women. Of these, 84% of participants reported working in or being exposed to an industry/environment suspected of contributing to development of sarcoidosis over their lifetime. The most frequently reported exposures were within farming and agriculture (27%), oil and gas (20%), metalworking and handling animals (18%). CONCLUSIONS: Amongst this cohort, a high proportion reported working with a potentially relevant exposure. Individuals being assessed for sarcoidosis should have their most responsible physician elicit a detailed work and environmental history. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (4): e2020014).

The use of fructosamine in cystic fibrosis-related diabetes (CFRD) screening.

OBJECTIVE: To determine whether serum fructosamine correlates with glycemic control and clinical outcomes in patients being screened for cystic fibrosis-related diabetes (CFRD). METHODS: Fructosamine and percent predicted forced expiratory volume in 1s (FEV1) were measured in patients undergoing a 2h oral glucose tolerance test (OGTT) for CFRD screening. Fractional serum fructosamine (FSF) was calculated as fructosamine/total protein. RESULTS: FSF exhibited a positive correlation with 2h OGTT results (r2=0.3201, p=0.009), and ROC curve analysis suggested that FSF can identify patients with an abnormal OGTT (AUC=0.840, p=0.0002). FSF also exhibited a negative correlation with FEV1 (r2=0.3732, p=0.035). Patients with FSF≥3.70µmol/g had significantly lower FEV1 (median 47%) compared to those with FSF<3.70µmol/g (median 90%; p=0.015). CONCLUSIONS: FSF correlated with both OGTT results and FEV1, and reliably identified patients with abnormal OGTT results. This simple blood test shows potential as an effective tool in CFRD screening.

Creating a model for improved chronic kidney disease care: designing parameters in quality, efficiency and accountability.

BACKGROUND: Observational and randomized controlled studies suggest that patients with stage 4 and 5 chronic kidney disease (CKD) derive morbidity and mortality benefit from being followed up in multidisciplinary, allied health clinics. It remains unclear how these clinics should be structured in order to optimize an efficient use of resources. The objectives of this study are (i) to describe 'human' resource utilization in an established 'traditional' multidisciplinary CKD clinic and (ii) to optimize efficiency and accountability of this multidisciplinary CKD clinic while maintaining or improving delivered quality of care. METHODS: We conducted a prospective, cohort, intervention study in the multidisciplinary CKD clinics at a university-affiliated hospital in Winnipeg, Canada. There were 480 patients identified as requiring multidisciplinary care (68% male; 32% female; 64% Caucasian, 25% First Nations, 7% Asian; mean age 61), and the majority of these were in stages 4 and 5 CKD (80%). The aetiologies of CKD included diabetes (53%), hypertension (10%) and glomerulonephritis (GN) (19%). At baseline, process engineering analyses were conducted on resource use and workflows within the clinics. The intervention entailed clinic restructuring including changes to scheduling templates and documentation format as well as standardization of practitioner roles. Cross-sectional data to serve as surrogates for quality of care and efficiency were collected 1 year pre- and post-intervention. RESULTS: Optimization of clinic structure did not significantly change the cycle times among nurses, dieticians and pharmacists, but nephrologists' cycle time decreased from 13.8 min [interquartile range (IQR) 8-17] to 10.0 min (IQR 10-15) with P < 0.001. Patient throughput time decreased from 73 min (IQR 51-95) to 68.5 min (IQR 55-80). Compliance with established practice guidelines prior to clinic restructuring was 61% for BP (<130/80); 69% for haemoglobin (110-120 g/dL); 69% for ASA use; 63% for beta-blocker use; 43% for ACEi/ARB use; 64% for statin use, and did not change significantly post-intervention. CONCLUSIONS: Optimization of multidisciplinary CKD clinic structure using a standard process engineering methodology improves resource utilization while maintaining (without compromising) quality of care. The delivery of care is accomplished without the need for additional resources and with decreased reliance on physician input. The methodology proposes a useful algorithm for dynamic monitoring of quality metrics for clinical care linked directly to specific allied health inputs.

An atypical presentation of liver enzyme elevation resulting from bosentan use.

Hepatocellular enzyme elevation is a known side effect of both bosentan and atorvastatin. However, a rise in liver enzyme level not characteristic of either agent individually may represent a reaction to their combination or an atypical reaction to bosentan alone. The present case report describes a patient who had been taking atorvastatin for many years and was started on bosentan for chronic thromboembolic pulmonary hypertension. After 19 weeks of therapy, she developed severe liver enzyme elevation that necessitated the discontinuation of both bosentan and atorvastatin. Although the safety of reintroducing bosentan in such a case is unknown, it was reintroduced in this patient because of the severity of her disease, the demonstrated treatment benefit and the lack of alternative treatment options. On reintroduction of bosentan alone, she again demonstrated significant liver enzyme elevation - this time occurring after only two doses. The present case highlights that bosentan can cause more rapid and severe hepatocellular enzyme elevation than previously believed, thus necessitating more frequent monitoring.