Screening for Fabry disease among 619 hemodialysis patients in Saudi Arabia.

OBJECTIVES: To determine the prevalence of Fabry disease (FD) among Saudi patients on hemodialysis. METHODS: This prospective study was conducted in 3 major hospitals in the Kingdom of Saudi Arabia (KSA). All adult patients (greater than 18 years old) attending the dialysis unit who have end-stage renal disease (ESRD) and on hemodialysis were included. Known patients with FD and those who refused to participate in the study were excluded. All eligible patients were screened for FD using dry blood spot (DBS) for alpha-galactosidase A (α-Gal A). A positive DBS (enzyme activity less than 40%) was followed by another con rmatory enzyme assay. When the second DBS sample was also positive (enzyme activity less than 40%), a Sanger sequencing of the GLA gene was performed. RESULTS: A total of 619 patients with ESRD and on hemodialysis were screened for FD using DBS for α-Gal A enzyme level. Enzymatic activity was below 40% in 11 samples. On retesting, 3 females had less than 20% enzymatic activity suggesting FD. Sanger sequencing of these 3 females showed the variant c.1055C greater than G (p.Ala352Gly) confirming the diagnosis of FD. Family screening of one of these 3 patients revealed one asymptomatic female carrying the same variant. CONCLUSION: The prevalence of FD in this cohort was 4.8 per 1000 patients. Screening of Fabry patients with ESRD seems to be a cost-effective strategy. Furthermore, relatives of the patients identified by screening enhances this screening strategy.

Assessment of professionalism among medical students at a regional university in Saudi Arabia.

Professionalism is the relationship that is built on trust between the medical profession and their community. We aim to assess the understanding and the perception of professionalism by the medical students at University of Bisha (UB) in Saudi Arabia. This was an observational, descriptive, and cross-sectional study conducted among the medical students at UB. A validated questionnaire containing nine questions was distributed to all medical students. The questionnaire addresses the knowledge, attitudes, and perception toward professionalism. Out of the all 122 students studying at the university, 97 responded by filling the questionnaire giving a response rate of 79.5%. Most of the responders gave a positive attitude and perception when asked about presumed scenarios addressing professionalism. The majority of the participants (83.5%) mentioned that it is necessary for the medical student to wear lab coat in the hospital, 67.0% disagreed on medical student to wear accessories during work, and 67.0% disagreed to request your friend to sign on behalf of you when you are absent. Only 15.5% accepted to report on an incidence during daily activities or exam, and 72.2% did not accept to discuss patients issues in public. Most of the medical students enrolled in this study seems to have positive attitude and perception regarding professionalism.

Failure Mode and Effect Analysis (FMEA) may enhance implementation of clinical practice guidelines: An experience from the Middle East.

RATIONALE, AIMS, AND OBJECTIVES: Implementation of clinical practice guidelines (CPGs) has been shown to reduce variation in practice and improve health care quality and patients' safety. There is a limited experience of CPG implementation (CPGI) in the Middle East. The CPG program in our institution was launched in 2009. The Quality Management department conducted a Failure Mode and Effect Analysis (FMEA) for further improvement of CPGI. METHODS: This is a prospective study of a qualitative/quantitative design. Our FMEA included (1) process review and recording of the steps and activities of CPGI; (2) hazard analysis by recording activity-related failure modes and their effects, identification of actions required, assigned severity, occurrence, and detection scores for each failure mode and calculated the risk priority number (RPN) by using an online interactive FMEA tool; (3) planning: RPNs were prioritized, recommendations, and further planning for new interventions were identified; and (4) monitoring: after reduction or elimination of the failure mode. The calculated RPN will be compared with subsequent analysis in post-implementation phase. RESULTS: The data were scrutinized from a feedback of quality team members using a FMEA framework to enhance the implementation of 29 adapted CPGs. The identified potential common failure modes with the highest RPN (≥ 80) included awareness/training activities, accessibility of CPGs, fewer advocates from clinical champions, and CPGs auditing. Actions included (1) organizing regular awareness activities, (2) making CPGs printed and electronic copies accessible, (3) encouraging senior practitioners to get involved in CPGI, and (4) enhancing CPGs auditing as part of the quality sustainability plan. CONCLUSION: In our experience, FMEA could be a useful tool to enhance CPGI. It helped us to identify potential barriers and prepare relevant solutions.