Primary and secondary liver endocrine tumors: clinical presentation,surgical approach and outcome

BACKGROUND/AIMS: The aim of this study was to evaluate the role of surgical treatment in the management of primary and secondary liver endocrine tumors. METHODOLOGY: We reviewed our experience of surgical treatment for primary and secondary liver endocrine tumors in 34 patients from 1980 to 1999. There were 31 cases of liver metastases from distant primary endocrine tumors treated by curative resection in 11 patients, cytoreductive surgery in 15 and orthotopic liver transplantation in 5. The remaining 3 consisted of primary liver endocrine tumor in 2 patients, liver invasion from primary bile duct endocrine tumor in 1, and all of them were treated by curative liver resection. RESULTS: In patients with distant primary endocrine tumors, the actuarial 5-year survival after curative liver resection, cytoreductive surgery, and orthotopic liver transplantation was of 91%, 50% (being 90% for non-functioning tumors), and 60%, respectively. In patients with primary liver endocrine tumors, long-term survival and disease-free survival (52-240 months) was achieved after curative resection. CONCLUSIONS: When indicated, liver surgery can offer cure and/or improve survival in patients with primary or secondary liver endocrine tumors. The choice of the surgical modality should be evaluated carefully.     

Rationale and design of the IRON-HF study: a randomized trial to assess the effects of iron supplementation in heart failure patients with anemia

BackgroundAnemia is a common finding in heart failure (HF) patients and has been associated with increased morbidity and mortality. It is generally denominated as anemia of chronic disease (ACD), but the association with true ferropenic anemia is common. Many studies have investigated the effects of treating anemia in HF patients with either erythropoietin alone or combination of erythropoietin and intravenous iron. However, the effect of iron supplementation alone in HF patients with ACD, ferropenic anemia, or both is unknown.Methods and ResultsIRON-HF study is a multicenter, investigator initiated, randomized, double-blind, placebo controlled trial that will enroll anemic HF patients with relatively preserved renal function, low transferrin saturation, low iron levels, and low to moderately elevated ferritin levels. Interventions are iron sucrose intravenously 200 mg once per week for 5 weeks, ferrous sulfate 200 mg by mouth 3 times per day for 8 weeks, or placebo. The primary objective is to assess the impact of iron supplementation (intravenously or by mouth) compared with placebo in HF patients with anemia from deficient iron availability. The primary end point is variation of peak oxygen consumption assessed by ergospirometry over 3-month follow-up. Secondary end points include functional class, brain natriuretic peptide levels, quality of life scores, left ventricular ejection fraction, adverse events, HF hospitalization, and death.ConclusionsThe results of IRON-HF should help to clarify the potential clinical impact of mild to moderate anemia correction in HF patients.     

Correlação entre Cardiomegalia pela Radiografia de Tórax e Diâmetro do Ventrículo Esquerdo pela Ecocardiografia em Pacientes com Doença de Chagas

BackgroundCardiomegaly on chest X-ray is an independent predictor of death in individuals with chronic Chagas cardiomyopathy (CCC). However, the correlation between increased cardiothoracic ratio (CTR) on chest X-ray and left ventricular end-diastolic diameter (LVEDD) on echocardiography is not well established in this population.ObjectivesTo assess the relationship between chest X-ray and LVEDD on echocardiography in patients with Chagas disease and its applicability to the Rassi score.MethodsRetrospective study on 63 Chagas disease outpatients who underwent chest X-ray and echocardiography. Cardiomegaly on chest X-ray was defined as a CTR>0.5. LVEDD was analyzed as a continuous variable. ROC curve was used to evaluate the ability of LVEDD in detecting cardiomegaly by chest X-ray, with a cut-off point defined by the highest sum of sensitivity and specificity.ResultsMedian age 61 years [interquartile range 48-68], 56% were women. CCC was detected in 58 patients, five patients had the indeterminate form of Chagas disease. Cardiomegaly was detected in 28 patients. The area under the ROC curve for LVEDD was 0.806 (95%CI: 0.692-0.919). The optimal cut-off for LVEDD was 60 mm (sensitivity = 64%, specificity = 89%). The use of LVEDD on echocardiography as a surrogate for CTR on chest X-ray changed the Rassi score values of 14 patients, with a reduction in the presumed risk in 10 of them.ConclusionLVEDD on echocardiography is an appropriate, highly specific parameter to distinguish between the presence and absence of cardiomegaly on chest X-ray in Chagas disease. (Arq Bras Cardiol. 2021; 116(1):68-74)     

Extreme hyperbilirubinemia: An indicator of morbidity and mortality in sickle cell disease

BACKGROUND Sickle cell disease(SCD) is a disorder that results in increased hospitalizations and higher mortality. Advances in management have resulted in increases in life expectancy and led to increasing awareness of sickle cell hepatopathy(SCH).However, its impact in patients on the natural history and outcomes of SCD is not known. Our study aims to describe the prevalence of extreme hyperbilirubinemia(EH), one form of SCH, its effect on morbidity and mortality,and correlations between sickle cell genotype and SCH type. We hypothesize that EH is associated with higher morbidity and mortality.AIM To investigate the effects of EH on morbidity and mortality among patients with SCD.METHODS This retrospective cohort study was performed using a database of patients with SCD treated at Grady Memorial Hospital between May 2004 and January 2017.Patients with EH(defined as total bilirubin above 13.0 mg/dL) were identified. A control group was identified from the same database with patients with total serum bilirubin ≤ 5.0 mg/dL. Electronic medical records were used to extract demographic information, laboratory values, radiology results, current medications, need for transfusions and mortality data. Two samples T-test, chi-squared test and Fisher's exact test were then used to compare the parameters between the two groups.RESULTS Out of the database, fifty-seven charts were found of patients with bilirubin 13 mg/dL. Prevalence of severe SCH as defined by EH was 4.8%(57/1172). There were no demographic differences between patients with and without EH.Significant genotypic differences existed between the two groups, with hemoglobin SS SCD being much higher in the EH group(P 0.001). Patients with severe EH had a significant elevations in alanine aminotransferase(157.0 ± 266.2 IU/L vs 19.8 ± 21.3 IU/L, P 0.001), aspartate aminotransferase(256.5 ± 485.9 U/L vs 28.2 ± 14.7 U/L, P 0.001) and alkaline phosphatase(218.0 ± 176.2 IU/L vs 85.9 ± 68.4 IU/L, P 0.001). Patients with EH had significantly higher degree of end organ failure measured with quick Sequential Organ Failure Assessment scores(0.42 ± 0.68 vs 0.01 ± 0.12, P 0.001), increased need for blood products(63% vs 5%, P 0.001), and exchange transfusions(10.5% vs 1.3%, P = 0.022).CONCLUSION Among patients with SCD, elevated levels of total bilirubin are rare, but indicative of elevated morbidity, mortality, and need for blood transfusions.Large differences in sickle cell genotype also exist, but the significance of this is unknown.