Late diagnosis of sitosterolemia in an adult case with unexplained hemolytic anemia.

Sitosterolemia is a rare autosomal recessive disease that lead to an increase in the intestinal absorption and decreased biliary excretion plant sterols. It is caused by mutations in ABCG5 and ABCG8 genes, encoring sterolin-1 and sterolin-2 protein. The main clinical manifestations are xanthomas, premature atherosclerosis, arthralgia and, of note, hematological alterations. As in many other systemic diseases, hematological manifestations may be the only notable finding, for this reason we want to highlight the importance of multidisciplinary work and raise awareness of this rare disease that can lead to serious consequences if not treated prematurely. Here we present a case of this disease as well as its entire diagnostic process developed from a simple analytical alteration.

Performance of the UASB reactor during wastewater treatment and the effect of the biogas bubbles on its hydrodynamics.

A lab-scale Upflow Anaerobic Sludge Blanket (UASB) reactor was used as a model for evaluating synthetic and complex industrial wastewater treatment, using a solar heater to control temperature. Also, hydrodynamics was assessed using the Computational Fluid Dynamics (CFD) method. Initially, the UASB reactor was operated with synthetic wastewater at Hydraulic Retention Time (HRT) of 24 h in 20 ± 2 °C and 30 ± 2 °C to measure the biogas bubbles production for CFD study. COD removal efficiencies of 85 ± 3% and 95 ± 3%, respectively, with production of 27 and 39 ml CH4/h, correspondingly, were observed. After that, the reactor was fed with complex industrial wastewater. It was evaluated at 24 h in both temperatures. At 30 °C, low COD removal efficiency was observed, being 48 ± 13%, with methane production of 20 ± 3 ml CH4/h. The plug flow pattern was observed in the CFD modelling at HRT of 24 h and 20 °C without considering biogas bubbles interaction. Similar hydrodynamic behaviour was observed at HRT of 24 h and 30 °C. Nonetheless, when biogas bubbles were considered in the CFD modelling, hydrodynamics significantly changed, passing from a plug flow to a complete mix flow pattern.

ICU outcomes in adult cardiac surgery patients in relation to ultrafiltration type.

Ultrafiltration (UF) is used to ameliorate the deleterious effects of cardiopulmonary bypass (CPB) in cardiac surgery patients. There are two different methods; conventional ultrafiltration (CUF), performed during CPB, and modified ultrafiltration (MUF), performed after CPB is finished. It has not been established which is better, and controversy remains regarding the optimal UF strategy. The objective of this study was to evaluate if MUF alone, or combined with CUF, could achieve greater fluid removal and contribute to better postoperative clinical outcomes. Also, the potential technique complications were studied. This was a prospective study which enrolled 125 consecutive adult patients receiving elective cardiac surgery with CPB. We analysed three treatment groups: MUF, CUF and both. Ultrafiltration was performed using a non-pulsatile CPB with a non-occlusive roller pump, Sarns 9000, and a polysulfone ultrafilter, Minntech. We studied pre- and intraoperative data and immediate postoperative clinical outcomes: total amount of drainage, transfusion needs, respiratory outcome, cardiac, renal and neurologic complications. Statistical analysis was performed using SPSS 11.0. All three groups were homogeneous and did not have differences in terms of demographic factors, previous history, risk scores, intervention and operative data. Volume of filtrate removal in the group which applied both techniques was larger than in the CUF or MUF groups alone (2569+/-823 vs 1679+/-651 vs 1398+/-353 ml, respectively, p=0.0001); however, despite this difference, there was no difference in the immediate postoperative fluid balances between the groups (596+/-1244 vs 880+/-1054 vs 986+/-1190 ml, p=0.30). Respiratory parameters and postoperative morbidity data analysed (total amount of drainage, transfusion needs, haemoglobin, acute lung injury, time with inotropes, ventricular failure, cardiogenic shock, neurologic complications and renal failure) were similar in all three groups, without statistical differences. Extubation time (10+/-7 vs 8.9+/-3 vs 9.4+/-7.9 hours, p=0.72) and ICU stay (56.6+/-72 vs 66.5+/-109 vs 44.2+/-25 hours, p=0.43) also were similar between the groups. We did not find any technique complication associated with any patient. In the present study, with adult patients receiving elective cardiac surgery, the combined ultrafiltration group had a larger fluid removal. However, neither type of ultrafiltration nor amount of filtered volume was accompanied by different postoperative ICU clinical outcomes. Ultrafiltration was considered a safe and reliable technique, with no related complications.

Systemic lupus erythematosus presenting as pseudocyesis.

A patient with SLE whose initial manifestation was with a rare delirious disorder manifested as pseudocyesis is described. The psychiatric manifestations were considered to be due to SLE and treated with pulse steroid therapy. Special attention is focused on treatment and MRI.