Long-term Follow-up Assessing Renal Angiomyolipoma Treatment Patterns, Morbidity, and Mortality: An Observational Study in Tuberous Sclerosis Complex Patients in the Netherlands.

BACKGROUND: Long-term data from patients with tuberous sclerosis complex (TSC)-associated renal angiomyolipoma (angiomyolipoma) are limited. STUDY DESIGN: Retrospective observational study. SETTING & PARTICIPANTS: Adult patients with TSC treated at the University Medical Center Utrecht (the Netherlands) from January 1990 through April 2012. PREDICTORS: Patient age and angiomyolipoma stage, based on computed tomography lesion count, size, and impact on renal anatomy, with higher stage representing higher angiomyolipoma burden. Patients in stages 3 or higher were considered at high risk for hemorrhage and candidates for selective arterial embolization. OUTCOMES: Kidney-related outcomes included hypertension, anemia, decreased kidney function, dialysis, kidney transplantation, nephrectomy, kidney-related blood transfusions, and mortality. Observed mortality was compared to the Dutch National Bureau of Statistics using standardized mortality ratio. RESULTS: Median follow-up was 15.8 years, of which staging was available for 5.4 years. Of 351 patients with TSC, 244 (69.5%) had confirmed angiomyolipoma; 144 (59.0%) reached stage 3 or higher. Age and angiomyolipoma stage were positively correlated: median age in the none-detected stage was 36.8 years, increasing to 43.6 years for stage 6. Embolization was performed in 117 patients; 57 had 2 or more embolization procedures. Higher stage was associated with hypertension, anemia, decreased kidney function, and transfusion. Hypertension, anemia, and decreased kidney function were more common in patients who underwent selective arterial embolization. 7 patients required dialysis, 7 received a kidney transplant, and 16 underwent nephrectomy. 29 deaths were recorded, most commonly related to renal complications (n=9[31%]). Mortality was significantly higher in the study cohort versus the general population (standardized mortality ratio, 4.8; 95% CI, 3.4-6.9). LIMITATIONS: Duration of follow-up with staging was too short to observe stage progression in most patients. CONCLUSIONS: Despite the use of preventive selective arterial embolization, patients with TSC exhibit clinically significant kidney disease and excess mortality, largely because of kidney-related complications.

Transitional safety incidents as reported by patients and healthcare professionals in the Netherlands: A descriptive study.

BACKGROUND: Care transitions between general practice and hospital are hazardous regarding patient safety. For developing an improvement strategy adjusted to local settings, understanding of type and potential causes of transitional safety incidents (TSIs) is needed. OBJECTIVES: To provide a broad overview of the nature of TSIs reported by patients and healthcare professionals. METHODS: We collected data (2011-2015) from three hospitals and 56 affiliated general practitioners (GPs) in two Dutch regions (one urban, one rural). We collected data from patients through a survey, interviews and incident reporting weeks, and from GPs and hospital specialists through incident reporting systems, surveys, interviews and focus group discussions. We classified reported TSIs according to type, cause and severity. RESULTS: In total, 548 TSIs were reported by 411 patients and 137 healthcare professionals; 368 of 548 TSI reports contained sufficient information for classification into aspects of the care transition process, 191 of 548 for cause, and 149 of 548 for severity. Most TSIs concerned handover correspondence from hospital to GP (26%), referral (14%) and communication/collaboration (14%). Concerning cause, reported TSIs could be attributed to organizational (48%) and human factors (43%). Twenty-four percent concerned unsafe situations, 45% near misses and 31% adverse events. Patients and healthcare professionals reported differently on referral (17% vs 9%), repeated diagnostic testing (20% vs 1%), and uncertainty about assigned responsible physician (10% vs 3%). CONCLUSION: Reported TSIs typically concerned informational discontinuity. One third caused harm to the patient. Patients report different TSIs than healthcare professionals, suggesting a different view.