Diversity of genetic alterations of primary central nervous system lymphoma in Hispanic versus non-Hispanic patients.

PURPOSE: Primary central nervous system lymphoma (PCNSL) is a rare type of non-Hodgkin lymphoma. Previous studies have identified MYD88, CD79b and PIM1 as the most common genetic mutations in PCNSL. The extent to which mutations vary by ethnicity is unknown. The purpose of this study was to describe differences in genetic mutations and survival by Hispanic ethnicity in PCNSL. METHODS: 30 patients with PCNSL were examined for mutations in 275 genes by DNA analysis and 1408 genes by RNA analysis utilizing next generation sequencing. RESULTS: 60% of patients were Hispanic. 125 different mutated genes were detected. The most commonly affected genes were: MYD88 (44%), CARD11 (21%), CD79b (17%), PIM1 (17%) and KMT2D (17%) . MYD88 mutation was less frequent in Hispanic patients (27% vs 66%, P=.02). More Hispanic patients had >3 mutated genes (89% vs 55 %. P=.03). Two-year progression-free survival (PFS) and overall survival (OS) in Hispanic vs. non-Hispanic patients (PFS 60% vs 27%, P=.09), (OS 60% vs 36%, P=.23). MYD88, CARD11, PIM1, and KMT2D were not associated with significant differences in OS or PFS. CD79b mutation correlated with superior 2-yr PFS (P=.04). CONCLUSIONS: We identified highly recurrent genetic alterations in PCNSL. Our data suggest that heterogeneity in some mutations may be related to ethnicity. There was no statistically significant difference in 2-yr PFS and OS in our Hispanic patients. Studies on larger population may further help to describe differences in tumor biology, and outcomes in Hispanic patients.

Calculated plasma volume status predicts outcomes after transcatheter aortic valve implantation.

OBJECTIVES: Congestion can worsen outcomes after transcatheter aortic valve implantation (TAVI), but can be difficult to quantify non-invasively. We hypothesised that preprocedural plasma volume status (PVS), estimated using a validated formula that enumerates percentage change from ideal PV, would provide prognostic utility post-TAVI. METHODS: This retrospective cohort study identified patients who underwent TAVI (2007-2017) from a prospectively collected database. Actual ([1-haematocrit] × [a + (b × weight (Kg))] and ideal (c × weight (Kg)) PV were quantified from equations where a, b and c are sex-dependent constants. Calculated PVS was then derived (100% x [(actual - ideal PV)/ideal PV]). RESULTS: In 564 patients (mean age 82±7 years, 49% male), mean PVS was -2.7±10.2%, with PV expansion (PVS >0%) evident in 39%. Only logistic European System for Cardiac Operative Risk Evaluation (EuroSCORE) independently predicted a PVS >0% (OR 1.85, p=0.002). On Cox analyses, a PVS >0% was associated with greater mortality at 3 (HR 2.29, 95% CI 1.11 to 4.74, p=0.03) and 12 months (HR 2.00, 95% CI 1.23 to 3.26, p=0.006) after TAVI, independently of, and incremental to, the EuroSCORE and New York Heart Association class. A PVS >0% was also independently associated with more days in intensive care (coefficient: 0.41, 95% CI 0.04 to 0.78, p=0.03) and in hospital (coefficient: 1.95, 95% CI 0.48 to 3.41, p=0.009). CONCLUSION: Higher PVS values, calculated simply from weight and haematocrit, are associated with greater mortality and longer hospitalisation post-TAVI. PVS could help refine risk stratification and further investigations into the utility of PVS-guided management in TAVI patients is warranted.

Tissue Doppler-Derived Left Ventricular Systolic Velocity Is Associated with Lethal Arrhythmias in Cardiac Device Recipients Irrespective of Left Ventricular Ejection Fraction.

BACKGROUND: Life-threatening arrhythmias (LTAs) can trigger sudden cardiac death or provoke implantable cardioverter-defibrillator (ICD) discharges that escalate morbidity and mortality. Longitudinal myofibrils predominate in the subendocardium, which is uniquely sensitive to arrhythmogenic triggers. In this study, we test the hypothesis that mitral annular systolic velocity (S'), a simple routinely obtained tissue Doppler index of LV long-axis systolic function, might predict lethal arrhythmias irrespective of left ventricular ejection fraction (LVEF). METHODS: This is a retrospective analysis of data from 302 patients (mean age, 68 years; LVEF, 32%; 77% male; 52% ischemic; 35% primary prevention; and 53% cardiac resynchronization therapy defibrillator [CRT-D]) who were followed up (median, 15 months) at two centers after receipt of an ICD or CRT-D for diverse indications. S', averaged from tissue Doppler-derived medial and lateral mitral annular velocities, was correlated with the primary outcome of time to sustained ventricular tachycardia (VT) or fibrillation (VF) needing device therapy. RESULTS: The median S' was 5.1 (interquartile range, 4.0-6.2) cm/sec and lower in CRT-D than ICD subjects (4.5 [3.8-5.6] cm/sec vs 5.5 [4.8-6.8] cm/sec, P < .001). Fifty-six (19%) subjects had LTA. Each 1 cm/sec higher S' correlated to a 30% decreased risk of LTA (hazard ratio = 0.70; 95% CI, 0.57-0.87; P = .001) independently of age, sex, ß-blocker use, center, ICD use, and LVEF. Adding S' to the baseline Cox model improved net reclassification (P = .02). An S' > 5.6 cm/sec was the best cutoff and linked to a 58% lower LTA risk than an S' ≤ 5.6 cm/sec (95% CI, 0.23-0.85; P = .02). CONCLUSIONS: A higher S' is associated with a reduced probability of LTA in cardiac device recipients irrespective of LVEF and may have the potential to be used clinically to titrate medical, device, and ablative therapies to mitigate future arrhythmic risk.

Calculated plasma volume status and outcomes in patients undergoing coronary bypass graft surgery.

OBJECTIVES: Congestion is associated with worse outcomes in critically ill surgical patients but can be difficult to quantify noninvasively. We hypothesised that plasma volume status (PVS), estimated preoperatively using a validated formula that enumerates percentage change from ideal plasma volume (PV), would provide incremental prognostic utility after coronary artery bypass graft (CABG) surgery. METHODS: In this retrospective cohort study, patients who underwent CABG surgery (1999-2010) were identified from a prospectively collected database. Actual ([1-haematocrit] x [a+(b x weight [kg])]) and ideal (c x weight [kg]) PV were obtained from equations where a, b and c are sex-dependent constants. Calculated PVS was then derived (100% x [(actual-ideal)/ideal]). RESULTS: In 1887 patients (mean age 67±10 years; 79% male; median European System for Cardiac Operative Risk Evaluation [EuroSCORE] 4), mean PVS was -8.2±9%. While 8% of subjects had clinical evidence of congestion, a relatively increased PV (PVS >0%) was estimated in 17% and correlated with lower serum sodium, higher EuroSCORE and a diagnosis of diabetes mellitus. A PVS≥5.6% was optimally prognostic and associated with greater mortality (HR: 2.31, p=0.009), independently of, and incremental to, EuroSCORE, New York Heart Association class and serum sodium. A PVS≥5.6% also independently predicted longer intensive care (ß: 0.65, p=0.007) and hospital (ß: 2.01, p=0.006) stays, and greater postoperative renal (OR: 1.61, p=0.008) and arrhythmic (OR: 1.29, p=0.03) complications. CONCLUSIONS: Higher PVS values, calculated simply from weight and haematocrit, are associated with worse inpatient outcomes after CABG. PVS could help refine risk stratification and further investigations are warranted to evaluate the potential clinical utility of PVS-guided management in patients undergoing CABG.

An aggressive form of Haemophilus parainfluenzae infective endocarditis presenting with limb weakness.

We present the case of a 49-year-old man with a bicuspid aortic valve who presented to the emergency department with limb weakness and a fever. Blood tests revealed a fulminant septic process with Haemophilus parainfluenzae bacteraemia, anaemia and thrombocytopenia. Imaging revealed a cervical spinal abscess and discitis causing spinal cord compression, in addition to multiple cerebral septic emboli, pleural effusions and ascites. A transoesophageal echocardiogram (TOE) performed post decompression of his spinal collection showed native aortic valve endocarditis with an associated large aortic root abscess. He underwent successful aortic valve surgery and a 6-week course of antibiotic therapy and made an excellent clinical recovery with no long-term complications from his condition.

Validation of open inguinal hernia repair simulation model: a randomized controlled educational trial.

BACKGROUND: A hernia repair open surgical simulation computer software was developed at Imperial College London. A randomized controlled educational trial was conducted to investigate the benefit of the simulation on the development of procedural knowledge. METHODS: Medical students in their clinical years were invited to participate in the trial. Students were block randomized to 4 groups: G1--Interactive Simulation; G2--Non-interactive Simulation; G3--Video Tutorial; G4--Control. On completion, they were objectively assessed on their ability to recall the tasks involved in an open inguinal hernia repair in the form of a multiple choice question (MCQ) and a simulated discussion with a consultant surgeon. RESULTS: Fifty-six students completed the study. Each arm carries similar baseline scores (pre-intervention MCQ) with means 43.33, 38.92, 38.33, and 39.57 in G1 to G4, respectively. MCQ score improvements and final assessment scores proved better in the intervention groups (1, 2, and 3) compared to controls. CONCLUSION: The interactive simulation has shown an objective benefit in teaching medical students the anatomical and procedural knowledge in performing an open inguinal hernia repair.

Management of Brown-Sequard syndrome in cervical disc diseases.

AIM: To raise the awareness of the appropriate management of unusual clinical presentation of cervical disc herniation. MATERIAL AND METHODS: Seven patients with Brown-Sequard syndrome due to cervical disc herniation presented with Brown-Sequard syndrome had been treated surgically as one of them treated with anterior cervical discectomy and three patients treated by anterior cervical discectomy and fusion while three of them have been treated by posterior laminectomy. RESULTS: Patients were 7 three of them were female and four were males and the mean age of our patients is 45.28 ± 16.49 years ranged between 32 and 72 years. History of spinodegenerative disease in the form of chronic neck pain with or without brachialgia was found in 6/7 patients (85.7%) while history of trauma was found in 5/7 patients (71.4%). Most common affected level is C5-C6 disc Outcome after surgical treatment revealed complete recovery in 4/7 patients (57.1%) while incomplete recovery in 2 patients (28.6%) and no improvement in one patient (14.3%). CONCLUSION: Brown-Sequard syndrome is a rare presentation of cervical disc herniation but accurate diagnosis, and early anterior spinal cord decompression lead to complete recovery of these cases.

Bleeding Meckel's diverticulum diagnosed and treated by double-balloon enteroscopy.

Meckel's diverticulum (MD) is the most common congenital anomaly of the gastrointestinal (GI) tract. The diagnosis of symptomatic MD has been cumbersome. Several case reports been published regarding direct visualization of MD with double balloon enteroscopy (DBE); diagnosing a bleeding MD leading to surgical resection. We report the use of DBE for the treatment of a bleeding MD.

Peripartum cardiomyopathy.

In 1971, Demakis and colleagues established the term peripartum cardiomyopathy (PPCM) and defined it by criteria based on the clinical profile of their patients. With the recognition that these criteria are arbitrary and that PPCM often presents earlier in pregnancy, the definition of PPCM has been recently updated by a working group on PPCM of the European Society of Cardiology. This article discusses the cause, clinical presentation, prognosis, and treatment of PPCM, as well as other related topics.

Pregnancy in Marfan syndrome: maternal and fetal risk and recommendations for patient assessment and management.

Pregnancy in women with the Marfan syndrome (MFS) is associated with the potential for a catastrophic and even fatal acute aortic dissection and the risk of having a child who will inherit the syndrome. The approach to pregnancy in patients with MFS is therefore challenging and deserves special considerations. This article presents an extensive review of available clinical information and provides recommendations for the management of patients with MFS during pregnancy.

Assessment of renal hemodynamic effects of nesiritide in patients with heart failure using intravascular Doppler and quantitative angiography.

OBJECTIVES: We evaluated the magnitude and site of action of the nesiritide mediated renal vasodilatory effect in patients with heart failure (HF). BACKGROUND: Nesiritide, a recombinant human B-type natriuretic peptide is approved for the treatment of acute decompensated HF and has been shown to exert favorable hemodynamic, neurohormonal, and symptomatic effects. The renal effect of nesiritide in HF patients has not been well defined. METHODS: In 15 patients with acute decompensated HF, intravascular Doppler and quantitative angiography of the renal artery were used to assess the effect of nesiritide on renal artery diameter and velocity time integral as well as renal blood flow and vascular resistance. Nesiritide was administered intravenously at a standard dose of 2 microg/kg bolus followed by a continuous infusion at a rate of 0.01 microg/kg/min. Assessment of nesiritide effect was made at 15 min. RESULTS: Nesiritide infusion was associated with a significant central hemodynamic effect including a fall in mean pulmonary artery pressure (36 +/- 12 mm Hg to 31 +/- 13 mm Hg, p < 0.001), mean pulmonary capillary wedge pressure (21 +/- 2 mm Hg to 15 +/- 10 mm Hg, p < 0.001), and systemic vascular resistance (1,995 +/- 532 dynes s cm(-5) to 1,563 +/- 504 dynes s cm(-5), r < 0.001), and an increase in cardiac output from 3.9 +/- 1.2 l/min to 4.6 +/- 1.6 l/min (p = 0.001). Nesiritide was also associated with a significant vasodilatory effect on the large conductance renal arteries resulting in an increase in renal artery diameter from 6.2 +/- 0.7 mm to 6.7 +/- 0.8 mm (p < 0.001). At the same time, there was a concomitant fall in mean renal artery pressure (99 +/- 17 mm Hg to 89 +/- 13 mm Hg, p = 0.002) and renal blood flow velocity time integral (27 +/- 15 cm/beat to 23 +/- 15 cm/beat, p = 0.008) and, therefore, no significant change in renal blood flow or renal vascular resistance. CONCLUSIONS: The nesiritide effect on the renal circulation in patients with HF is complex, with a marked vasodilatory action on the large, conductance renal arteries but a concomitant fall in velocity time integral and no effect on renal vascular resistance or renal blood flow. Lack of increase in renal blood flow may be due to a fall in renal blood pressure or an intrarenal vasoconstrictive effect.

Mitomicina-C per-operatória em trabeculectomia / Mitomicin-C in trabeculectomy

Foram estudados 28 pacientes com indicaçäo cirúrgica para tratamento de glaucoma. Em todos os casos, a técnica de escolha foi a trabeculectomia usando-se a Mitomicina-C como terapia adjuvante. Observou-se uma reduçäo média da pressäo intra-ocular de 44,8 por cento, em seis meses de acompanhamento. Dos 28 pacientes, 11 apresentaram complicaçöes no pós-operatório (39,28 por cento).