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Purpose Latin American reports on pheochromocytomas and paragangliomas (PPGL) are scarce. Recent studies demonstrate changes in clinical presentation and management of these patients. Herein, we assessed the main characteristics of PPGL patients in our academic center over the last four decades. Methods Demographic, clinical, biochemical and perioperative data from 105 PPGL patients were retrospectively and prospectively collected over the 1980-2019 period. Data were organized into four time periods by decades. Results Age at diagnosis, gender, tumor size and percentage of bilaterality, percentage of paragangliomas and metastases remained stable across the four decades. The proportion of genetic testing and incidentalomas increased in recent decades (all p<0.001). Therefore, we compared PPGLs diagnosed as incidentalomas (36%) to those clinically suspected (64%). Incidentalomas had fewer adrenergic symptoms (38 vs. 62%; p<0.001), lower rates of hypertension (64 vs. 80%; p=0.01) and hypertensive crisis (28 vs. 44%; p=0.02); also they had lower functionality (79 vs. 100%; p=0.01) and lower catecholamines levels (8.4 vs. 12.5-fold above upper cut-offs; p=0.04). Regarding management of all PPGLs over the decades, we observed significant increases in both perioperative doxazosin dose (p=0.003) and laparoscopic approach rates (p<0.001), along with a decrease in the length of hospital stays (p=0.007). Conclusions We observed a change in the clinical presentation of PPGL in recent decades, with a marked increase in incidental cases and milder symptoms. The implementation of a multidisciplinary program for adrenal disorders in our institution has translated into more timely diagnoses, more genetic testing, and improvements in perioperative management.

Introduction and aim. Wilson’s disease (WD) is an uncommon cause of acute liver failure (ALF). Our aim was to describe clinical features, diagnostic findings, treatments, and outcomes of patients with ALF due to WD. Material and methods. Retrospective medical record reviews of all patients with ALF due to WD in eight years in Uruguay. Results. WD was the cause of six (15%) of thirty-nine ALF cases. All patients were females, with a mean age of 18 years. Four patients presented with hyperacute liver failure and two with acute failure. Jaundice was the main complaint of all patients. Mean total bilirubin (TB), alkaline phosphatase (AP), AST, and ALT were 27.5 mg/dL, 45.5 IU/l, 156 IU/L, and 51 IU/L, respectively. Ceruloplasmin levels were low in four patients, urinary cooper was high in four, and two had Kayser-Fleischer rings. All patients had Coombs-negative hemolytic anemia, acute kidney injury, histochemical identifiable copper, and advanced fibrosis on liver histology. The average MELD score was 36. All patients were treated with d-penicillamine and listed for urgent liver transplantation (LT). Prometheus® was performed in one patient. Three patients died: two without LT and one after LT. Three patients survived: one without LT (New Wilson Index < 11) and two with LT. The referral time to the program and the total time (referral plus waiting list time) were longer for non-survivors than for survivors (14 vs. 3 days and 23 vs. 8 respectively). Conclusion. All cases had typical clinical, analytical and histopathology characteristics. Early referral was determinant of prognosis.