Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Fresh Look at an Old Foe.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are some of the less common cutaneous adverse drug reactions with significant mortality. Objectives: This study was undertaken with the objective of studying the demographics and clinical profile of SJS/TEN and identifying parameters associated with mortality. Materials and Methods: All patients with SJS/TEN over 10 years (2010-2020) were included in the study. Data obtained from in-patient and out-patient records were analysed. Results: A total of 82 patients with SJS/TEN were admitted to our centre over a period of 10 years. Patients with SJS were significantly younger than those with TEN, with a male: female ratio >1 in SJS and <1 in TEN. The most commonly implicated drugs were antiepileptics (n = 29, 35.4%), antibiotics (n = 20, 24.4%). and Non-steroidal antiinflammatory drugs (NSAIDs) (n = 7, 8.5%). The mortality rate in the TEN group was 16% (n = 8). Certain factors such as cutaneous lesions preceding mucosal lesions at onset, high mean Body surface area (BSA) of denudation and a transfer to intensive care unit (ICU) more than 7 days after admission were significantly associated with higher mortality. There was no difference between survivors and deaths in terms of delay in hospitalisation, total disease duration, implicated drug, delay in initiation of therapy, the onset of re-epithelialisation, Severity-of-illness score for TEN (SCORTEN) and total duration of hospital stay. Conclusion: Factors significantly associated with increased mortality in TEN were cutaneous onset of lesions, mean BSA of involvement and transfer to the intensive care unit (ICU) beyond day 7 of admission.

Neuropsychiatric manifestations are associated with increased mortality in Indian patients with lupus: A single centre retrospective observational study.

OBJECTIVE: To study the prevalence of different NPSLE manifestations in our cohort and to compare clinical and immunological features and outcomes including mortality of patients with NPSLE and SLE controls without NP involvement. METHODS: This was a retrospective study in a tertiary care referral centre. All patients of SLE seen in the last 10 years and fulfilling the SLICC criteria with neuropsychiatric manifestations as per the ACR definitions were included. Patients of SLE without NP involvement were sequentially assigned as controls in a ratio of 1:2. RESULTS: Of the 769 patients diagnosed with SLE from Jan 2011 to December 2020, 128 (16.6%) had NPSLE manifestations as per the ACR definitions. The commonest NPSLE manifestation was seizures (6.5%) followed by cerebrovascular accident (3.9%). NPSLE manifestation occurred at the first presentation of SLE in 99/128 (77.3%) patients and 58 (45.3%) patients had more than one NPSLE manifestation. Lupus anticoagulant and anticardiolipin antibody were tested in 120 patients and were positive in 16 (13.3%) and 12 (10%), respectively. No difference was found in anti-ribosomal p, lupus anticoagulant and anticardiolipin antibodies between the cases and controls. Twenty-one (16.4%) deaths occurred in patients with NPSLE (median follow-up of 40 months) as compared to 13 (5%) in controls (median follow-up of 32 months) (p = <0.001). The cumulative survival of patients with NPSLE was lower as compared to controls (p < 0.001). Relapse of NPSLE was seen in 11(8.6%) patients and was associated with mortality (p = 0.017). CONCLUSIONS: Seizures and cerebrovascular accidents are the commonest NPSLE syndromes in our patients. The presence of NPSLE was associated with high mortality in Indian patients with lupus.

Quantifying Burnout among Emergency Medicine Professionals.

BACKGROUND: Burnout is a syndrome explained as serious emotional depletion with poor adaptation at work due to prolonged occupational stress. It has three principal components namely emotional exhaustion(EE), depersonalization(DP) and diminished feelings of personal accomplishment(PA). Thus, we aimed at measuring the degree of burnout in doctors and nurses working in emergency medicine department (EMD) of 4 select tertiary care teaching hospitals in South India. METHODS: A cross sectional survey was conducted among EMD professionals using a 30-item standardized pilot tested questionnaire as well as the Maslach burnout inventory. Univariate and Multivariate analyses were conducted using binary logistic regression models to identify predictors of burnout. RESULTS: Total number of professionals interviewed were 105 of which 71.5% were women and 51.4% were doctors. Majority (78.1%) belonged to the age group 20-30 years. Prevalence of moderate to severe burnout in the 3 principal components EE, DP and PA were 64.8%, 71.4% and 73.3% respectively. After multivariate analysis, the risk factors [adjusted odds ratio (95% confidence intervals) for DP included facing more criticism [3.57(1.25,10.19)], disturbed sleep [6.44(1.45,28.49)] and being short tempered [3.14(1.09,9.09)]. While there were no statistically significant risk factors for EE, being affected by mortality [2.35(1.12,3.94)] and fear of medication errors [3.61(1.26, 10.37)] appeared to be significant predictors of PA. CONCLUSION: Degree of burn out among doctors and nurses is moderately high in all of the three principal components and some of the predictors identified were criticism, disturbed sleep, short tempered nature, fear of committing errors and witnessing death in EMD.

New-onset diabetes after transplantation--role of oral glucose tolerance test for diagnosis and study of risk factors.

To determine the role of the oral glucose tolerance test in the early detection of new-onset diabetes after transplantation (NODAT) and to compare the various risk factors and insulin kinetics in the transplant patients, we studied 41 live-related renal allograft recipients who were not diabetic before transplantation. Immunosuppression included triple drug therapy (cyclosporine, azathioprine and steroids) and rejection episodes were treated with methyl prednisolone (30 mg/kg IV × 3 days). All the study patients were subjected to an oral glucose tolerance test (OGTT) at Day 90 post-transplant and classified as having normal glucose tolerance (NGT), impaired fasting glucose (IFG), impaired glucose tolerance (IGT) and NODAT as per the World Health Organization guidelines. Insulin levels were also determined at 0, ½ hour, 1 hour and 2 hours during OGTT. NODAT was noted in 29.2% of the study patients, IFG in 4.8% of the study patients and NGT in 65.8% of the study patients. All the groups had normal fasting plasma glucose, but higher than normal insulin levels, suggesting insulin resistance. The patients with overt NODAT had, in addition, low fasting insulin (insulin secretory defect). OGTT may be used for the early detection of NODAT. Although insulin resistance is detected in the majority of post-transplant patients, NODAT also reveals also an insulin secretory defect.

The right sided syndrome, congenital absence of kidney and testis.

Unilateral renal agenesis (URA) is a developmental defect associated with ano-malies of the genitourinary system. The associations vary from absence of testis alone to high anorectal anomalies in other patients. We present two interesting patients with URA, encountered recently. Our first case was diagnosed with URA at the age of 11 years, which was detected on sonography, when he presented with pain abdomen. The presence of an epididymal cyst masked the absence of ipsilateral testes leading to delay in the diagnosis. Our second case was diagnosed with URA during the neonatal period when he presented with anorectal agenesis. He underwent abdomino-anal pull-through operation and later clinical course was complicated by recurrent cystitis, secondary vesicoureteral reflux and hydroureteronephrosis of solitary kidney, progressing to chronic kidney disease.

Posterior reversible encephalopathy syndrome in a patient with hepatitis B induced type 1 membranoproliferative glomerulonephritis.

Posterior reversible encephalopathy syndrome (PRES) is a rare complication of nephrotic syndrome and corticosteroid therapy. Here, we discuss an 18 year old man with type 1 membranoproliferative glomerulonephritis (MPGN) secondary to hepatitis B infection who developed posterior leukoencephalopathy while on therapy with lamivudine and prednisone. He developed seizures and vision loss. He also had hypertension, but no sudden elevation was recorded at any time. Magnetic resonance imaging revealed patchy areas of altered signal intensity involving cortical gray and subcortical white matter in the bilateral frontoparietal regions, occipital cortices, temporal cortices and cerebellar hemispheres, and hyperintensity on T2W and FLAIR sequences. Tapering of prednisone and controlling hypertension resulted in clinical improvement within a few days, and in a month MRI was normal. Diagnosing PRES requires a high index of suspicion when treating similarly susceptible patients. PRES as a complication during the treatment of MPGN secondary to hepatitis B has, to our knowledge, never been reported previously in the literature.

Computed tomography-fluoroscopy image integration-guided catheter ablation of atrial fibrillation.

INTRODUCTION: This study examines the feasibility of atrial fibrillation (AF) ablation using registered three-dimensional computed tomography (CT) images of the left atrium with fluoroscopy. METHODS AND RESULTS: A total of 50 consecutive patients with symptomatic AF refractory to medical therapy (32 paroxysmal, 18 persistent, age 55 +/- 10 years) were randomized to undergo a catheter-based AF ablation procedure with or without the CT-fluoroscopy guidance system. All patients underwent preprocedural contrast-enhanced CT imaging and segmentation of the left atrium. For the CT-fluoroscopy group, circumferential lesions encompassing the pulmonary vein (PV) antrum and linear lesions along the roof of the left atrium between the superior PVs and the mitral isthmus were created on the CT image, which was registered with real-time fluoroscopy. The registered images were then used to navigate the ablation catheters to the sites of planned ablation. After the ablation sites were completed, any remaining PV potentials were isolated with electrophysiological guidance. In the control patients, the same technique was performed without using the CT-fluoro guidance system. CT scans were accurately registered to fluoroscopic images with minimal manual correction. Operators could navigate catheters on the registered images to preplanned, extraostial sites for ablation. CT-fluoroscopy guidance decreased procedure duration and fluoro times (P < 0.05). At a mean follow-up of 9 +/- 2 months, 21 patients (84%) in the CT-fluoro guidance group and 16 patients (64%) in the control group have had no recurrence of AF. CONCLUSION: CT-fluoroscopic-guided left atrial ablation is feasible and allows appropriate catheter manipulation in the left atrium.

Feasibility of noncontact intracardiac ultrasound ablation and imaging catheter for treatment of atrial fibrillation.

Atrial fibrillation (AF) affects 1% of the population and results in a cost of 2.8 billion dollars from hospitalizations alone. Treatments that electrically isolate portions of the atria are clinically effective in curing AF. However, such minimally invasive catheter treatments face difficulties in mechanically positioning the catheter tip and visualizing the anatomy of the region. We propose a noncontact, intracardiac transducer that can ablate tissue and provide rudimentary imaging to guide therapy. Our design consists of a high-power, 20 mm by 2 mm, 128-element, transducer array placed on the side of 7-French catheter. The transducer will be used in imaging mode to locate the atrial wall; then, by focusing at that location, a lesion can be formed. Imaging of previously formed lesions could potentially guide placement of subsequent lesions. Successive rotations of the catheter will potentially enable a contiguous circular lesion to be created around the pulmonary vein. The challenge of intracardiac-sized transducers is achieving high intensities (300-5000 W/cm2) needed to raise the temperature of the tissue above 43 degrees C. In this paper, we demonstrate the feasibility of an intracardiac-sized transducer for treatment of atrial fibrillation. In simulations and proof-of-concept experiments, we show a 37 degrees C temperature rise in the lesion location and demonstrate the possibility of lesion imaging.

Registration of 3D computed tomographic images with interventional systems: implications for catheter ablation of atrial fibrillation.

Despite the great promise catheter ablation offers in the treatment of complex arrhythmias such as atrial fibrillation (AF), long procedure times and somewhat suboptimal results hinder the widespread use of this technique. As fluoroscopy does not provide contrast differentiation between the area of interest and the surrounding structures, there is a lack of proper intra procedure image guidance. Segmentation of anatomical structures such as the left atrium (LA) can be performed using images obtained with modalities such as computed tomography (CT). However, unlike the cardiac mapping systems, these imaging systems do not track catheters in real time. This review addresses the evolving concept of image registration to deliver therapy in cardiac arrhythmias.

Peri-infarct ischemia determined by cardiovascular magnetic resonance evaluation of myocardial viability and stress perfusion predicts future cardiovascular events in patients with severe ischemic cardiomyopathy.

BACKGROUND: We assessed whether cardiovascular magnetic resonance imaging (CMR) of peri-infarct ischemia provides prognostic information in severe ischemic cardiomyopathy (ICM) patients referred for revascularization. METHODS: Twenty-one patients with severe ICM were recruited prospectively for combined stress adenosine perfusion, late gadolinium enhancement, and rest perfusion studies. The patients were followed for in-hospital and post-discharge cardiovascular events. RESULTS: During 12+/- 9.8 months follow-up, 67% of the patients with peri-infarct ischemia and 13% of the patients without peri-infarct ischemia had cardiovascular events (p = 0.03). CONCLUSION. In severe ICM patients, the presence of peri-infarct ischemia was associated with a higher incidence of cardiovascular events.

Combined use of 1C and III agents for highly symptomatic, refractory atrial fibrillation.

BACKGROUND: Despite advances in non-pharmacologic therapy for atrial fibrillation (AF), some patients remain highly refractory. OBJECTIVE: We report our experience with the unique combined use of 1C and III agents in patients with highly refractory paroxysmal atrial fibrillation. MATERIALS AND METHODS: Six patients with symptomatic AF (three persistent) were selected after failing multiple antiarrhythmic medications and radiofrequency ablation. They were started on flecainide or propafenone and sotalol or dofetilide during three days of inpatient monitoring. No patient had coronary artery disease. All patients had loop recorder follow-up and ECG recordings during clinic visits for a mean follow-up of 9 +/- 11 months. RESULTS: After therapy, all patients had complete, sustained control of their symptoms with no evidence of AF or proarrhythmia on monitoring. One patient had recurrence of AF after stopping sotalol and was started back on the drug with complete control. CONCLUSIONS: Combined therapy with a 1C and III agent may be an effective alternative for the treatment of selective, highly refractory AF. Careful patient selection and hospitalization for initiation is necessary to minimize potential proarrhythmic effects. As this is a short-term therapy, further study is needed to assess the extent of efficacy in a larger number of patients.

Single-breathhold, four-dimensional, quantitative assessment of LV and RV function using triggered, real-time, steady-state free precession MRI in heart failure patients.

PURPOSE: To validate a novel, real-time, steady-state free precession (SSFP), single-breathhold technique for the assessment of left ventricular (LV) and right ventricular (RV) function in heart failure patients. MATERIALS AND METHODS: A total of 20 heart failure patients (mean age 59 +/- 17 years) underwent scanning with our new, real-time, spiral SSFP sequence in which each cardiac phase was acquired in 118 msec at a resolution of 1.8 x 1.8 mm. Each cardiac slice (1-cm thick) was automatically advanced based on a cardiac trigger, allowing complete coverage of the heart in a single breathhold. The patients also underwent LV and RV assessment with the gold standard: multiple breathhold, cardiac-gated, segmented k-space strategy. LV and RV end-systolic volume (ESV) and end-diastolic volume (EDV) and LV mass were compared between the two imaging techniques. RESULTS: The new real-time strategy was highly concordant with the gold standard technique in the assessment of LVEDV (r = 0.98), LVESV (r = 0.98), RVESV (r = 0.86), RVEDV (r = 0.91), LVMASS (r = 0.95), RVEF (r = 0.70), and LVEF (r = 0.94). The mean bias (95% confidence interval [CI]) for each parameter is LVEDV: 10.6 cc (cm(3)) (3.8-17.4 cc), LVESV: -0.8 cc (-5.3 to 3.7 cc), RVEDV: 3.7 cc (-5.6 to 13.2 cc), RVESV: -3.1 cc (-11.1 to 4.9 cc), LVMASS: 26 g (12.4-39.8 g), RVEF: -2.9% (1.3 to -7.2 %), LVEF: 1.9% (5 to -1.1%). In addition, data acquisition was only nine +/- two seconds with the real-time strategy vs. 312 +/- 41 seconds for the standard technique. CONCLUSION: In patients with heart failure, real-time, spiral SSFP allows rapid and accurate assessment of RV and LV function in a single-breath hold. Using the same strategy, increased temporal resolution will allow real-time assessment of cardiac wall motion during stress studies.

Acute pancreatitis following kidney transplantation - role of viral infections.

Acute pancreatitis following renal transplantation is an unusual complication that carries a high mortality. Over the last 10 yr, five of 185 patients at our center developed acute pancreatitis. All had live related donors and were on conventional triple drug immunosuppression. Pancreatitis was classified according to the computed tomography scan based on Atlanta Classification. All five patients who developed acute pancreatitis had evidence of symptomatic or serologically active viral infection (chicken pox in two, cytomegalovirus infection in two, hepatitis E virus in one) and no patient without viral infection developed pancreatitis. Overall, 45 patients developed symptomatic or serologically active viral infection. There was a significant association between viral infection and pancreatitis (chi-square test, p < 0.001). Three patients with severe acute pancreatitis died while both patients with mild pancreatitis survived. An active search for viral infections should be made in all patients with acute pancreatitis. Specific antiviral measures may help reduce the mortality of acute pancreatitis in these patients. Consideration must be given to varicella immunization in patients with renal failure.