A novel method for the evaluation of right ventricular dysfunction in acute pulmonary embolism: Myocardial work indices.

PURPOSE: The presence of right ventricular dysfunction indicates a higher risk status in patients with pulmonary embolism (PE). The RV strain evaluated by speckle-tracking echocardiography seems to be more reliable method in the evaluation of RV dysfunction as compared to standard echocardiographic measures. In this study, we aimed to determine the value of myocardial-work indices in evaluating serial changes of RV function in acute PE. METHODS: Our study comprised 83 consecutive acute PE patients who admitted to our tertiary cardiovascular hospital. Echocardiography was performed within the first 24-hours of hospitalization, and RV and LV myocardial-work parameters were obtained along with standard echocardiographic parameters. The change in the RV/LVr detected on tomography was selected as the primary outcome measure, and its' predictors were analyzed with classical linear regression and a generalized additive model (GAM). RESULTS: Among the LV-RV strain and myocardial work parameters, the RV global longitudinal strain (GLS) has borderline statistical significance in predicting the RV/LVr change whereas the RV global work efficiency (RV-GWE) strongly predicted RV/LVr change (p: 0.049 and <0.001, respectively). CONCLUSION: In this study, classical linear regression and GAM analyses showed that RV-GWE seems to offer a better prediction of RV/LVr change in patients with acute PE.

A comparison of hypersensitivity reactions between intravenous and intramuscular applications of native E. coli asparaginase in children with acute lymphoblastic leukemia.

INTRODUCTION: Asparaginase is an indispensable drug in treating childhood acute lymphoblastic leukemia (ALL). Hypersensitivity reactions (HSR) are the most common side effects and interfere with the antineoplastic activity of the drug. This study aims to compare the intramuscular (IM) and intravenous (IV) administration routes of Native Escherichia coli Lasparaginase (L-ASNase) in terms of hypersensitive reactions. METHODS: L-ASNase was randomly administered IV or IM to newly diagnosed ALL patients and HSR was monitored in all patients for 1 h following the end of the IV infusion and for 2 h following the end of the IM administration of L-ASNase. Based on a retrospective review of clinical charts, reactions were identified. In order to determine the severity of each allergic reaction, we used the Common Terminology Criteria for Adverse Events (CTCAE) version 4.03 for allergic reactions. RESULTS: A total of 1032 doses of L-ASNase were administered to 85 patients (42 males and 43 females) during the study period. Among 85 patients, 30 reactions were recorded, which means that 35% of the patients reacted. According to the CTCAE, twenty-nine out of 30 reactions (97%) were grade 2, while one (3%) was grade 4. In terms of individual doses, there was a non-significant trend toward increased incidence of reactions with IV administration (3.8% versus 0.9%, p = 0.064). The rate of reactions was higher in patients who received all IV doses (n: 60) as compared to those who received all IM doses (n: 25) (31.7% vs. 3.5%; chi-square= 8.415, p value=0.04). Based on the risk groups and HSR incidence, it was found that high risk group (HRG) patients were significantly more likely to develop HSR compared to the standart risk group (SRG) and intermediate risk group (MRG) patients (chi-square p = 0.003, CI: 95%; odds ratio: 3.12 and 5.91, respectively). CONCLUSIONS: In conclusion, IM administration of L-ASNase causes significantly less HSR to L-ASNase than the IV route. Patients with HRGALL have a higher risk of HSR. Since L-ASNase is still used in many developing countries and there are problems in the supply of Erwinia chrysanthemi ASNase (Erwinia), LASNase can be administered IM to reduce the frequency of HSR.

Prognostic Importance of Pulmonary Artery Pulsatility Index and Right Ventricular Stroke Work Index in End-Stage Heart Failure Patients.

BACKGROUND: Right ventricular (RV) failure is an important cause of morbidity and mortality in patients with left ventricular (LV) end-stage heart failure (ESHF). Pulmonary artery pulsatility index (PAPi) and RV stroke work index (RVSWI) are invasive parameters related to RV function. This study aimed to investigate the prognostic impact of PAPi and RVSWI in these patients. METHODS AND RESULTS: In this study, 416 patients with ESHF were included. The adverse cardiac event (ACE) was defined as LV assist device implantation, urgent heart transplantation, or cardiac mortality. There were 218 ACE cases and 198 non-ACE cases over a median follow-up of 503.50 days. Patients with ACE had lower PAPi and similar RVSWI compared to those without ACE (3.1 ± 1.9 vs. 3.7 ± 2.3, p = 0.003 and 7.3 ± 4.9 vs. 6.9 ± 4.4, p = 0.422, respectively). According to the results of multivariate analysis, while PAPi (from 2 to 5.65) was associated with ACE, RVSWI (from 3.62 to 9.75) was not associated with ACE (hazard ratio [HR]: 0.75, 95% confidence interval [CI] [0.55-0.95], p = 0.031; HR: 0.79, 95% CI: [0.58-1.09], p = 0.081, respectively). Survival analysis revealed that PAPi ≤2.56 was associated with a higher ACE risk compared to PAPi >2.56 (HR: 1.46, 95% CI: 1.11-1.92, p = 0.006). PAPi ≤2.56 could predict ACE with 56.7% sensitivity and 51.3% specificity at 1 year. Furthermore, the association between RVSWI and ACE was nonlinear (J-curve pattern). Low and high values seem to be associated with higher ACE risk compared to intermediate values. CONCLUSION: The low PAPi was an independent risk for ACE and it had a linear association with it. However, RVSWI seems to be have a nonlinear association with ACE (J-curve pattern).

Central Nervous System Fungal Infections in Children With Leukemia and Undergoing Hematopoietic Stem Cell Transplantation: A Retrospective Multicenter Study.

BACKGROUND: Central nervous system fungal infections (CNSFI) are seen in patients with hematologic malignancies and have high morbidity and mortality. Because of their rarity, there is limited data on CNSFI in children with no established treatment protocols or guidelines. MATERIALS AND METHODS: In this multicenter retrospective study, 51 pediatric patients with leukemia, 6 of whom had undergone bone marrow transplantation, with proven or probable CNSFI were evaluated. Fungal infections were defined as proven or probable based on European Organisation for Research and Treatment of Cancer criteria. Proven CNSFI was diagnosed by appropriate central nervous system (CNS) imaging or tissue sample findings in combination with positive microbiological results of cerebrospinal fluid. A positive culture, microscopic evidence of hyphae, a positive result of the galactomannan assays are defined as positive microbiological evidence. Probable CNSFI was defined as appropriate CNS imaging findings together with proven or probable invasive fungal infections at another focus without CNS when there is no other explanatory condition. Data was collected by using the questionnaire form (Supplemental Digital Content 1, http://links.lww.com/JPHO/A541 ). RESULTS: Seventeen patients had proven, 34 patients had probable CNSFI. Headaches and seizures were the most common clinical findings. The median time between the onset of fever and diagnosis was 5 days. The most common fungal agent identified was Aspergillus . Sixteen patients received single-agent, 35 received combination antifungal therapy. Surgery was performed in 23 patients. Twenty-two patients (43%) died, 29 of the CNSFI episodes recovered with a 20% neurological sequelae. CONCLUSION: CNSFIs should be considered in the differential diagnosis in patients with leukemia and refractory/recurrent fever, headache, neurologicalocular symptoms, and a radiologic-serological evaluation should be performed immediately. Early diagnosis and prompt management, both medical and surgical, are essential for improving clinical outcomes.

Effect of Group 2 Pulmonary Hypertension Subgroups on Outcomes: Impact of the Updated Definition of Pulmonary Hypertension.

BACKGROUND: Pulmonary hypertension (PH) is a common complication of end-stage heart failure (ESHF) and associated with increased mortality. The definition of PH has recently been changed from a mean pulmonary arterial pressure (PAPm) ≥25 mmHg to a PAPm >20 mmHg. Since this change, there are no data evaluating group 2 PH subgroups on outcomes. The purpose of this study was to determine the impact of updated group 2 PH subgroups on outcomes, as well as to evaluate the clinical, echocardiographic, and haemodynamic characteristics of subgroups, and determine predictors of PH in patients with ESHF. METHOD: A total of 416 patients with ESHF with left ventricular ejection fraction (LVEF) ≤25% were divided into three groups. Pulmonary hypertension was defined as PAPm >20 mmHg. Primary outcome was defined as left ventricular assist device (LVAD) implantation, urgent heart transplantation (HT), or death. Secondary outcome was defined as LVAD implantation and HT. RESULTS: Over a median follow-up of 503.5 days, combined pre- and postcapillary PH (Cpc-PH) displayed greater risk of primary outcome than those with isolated postcapillary (Ipc-PH) (hazard ratio [HR], 1.57; 95% confidence interval [CI], 1.29-1.91; p<0.001) and those with no PH (HR, 2.47; 95% CI, 1.68-3.63; p<0.001). Patients with Ipc-PH demonstrated greater risk than those with no PH (HR, 1.57; 95% CI, 1.57-1.90; p<0.001). Likelihood ratios of updated PH criteria and old PH criteria (PAPm ≥25 mmHg) in identifying primary outcome were 75.6 (R2=0.179) and 72.09 (R2=0.164). Patients with PAPm 21-24 mmHg had a higher primary outcome than those with PAPm ≤20 mmHg. Severe mitral regurgitation, LVEF, grade 3 diastolic dysfunction, diabetes, and cardiac output were predictors of PH. CONCLUSIONS: Pulmonary hypertension increases the risk of LVAD, urgent HT, or death, and Cpc-PH further increases risk in patients with ESHF. Compared to the previous definition, a new PH definition better discriminates death, going to urgent HT, or LVAD implantation for PH subgroups.

Temporal association of contamination obsession on the prehospital delay of STEMI during COVID-19 pandemic.

BACKGROUND: One of the modifiable risk factors for ST elevation myocardial infarction is prehospital delay. The purpose of our study was to look at the effect of contamination contamination obsession on prehospital delay compared with other measurements during the Covid-19 pandemic. METHOD: A total of 139 patients with acute STEMI admitted to our heart center from 20 March 2020 to 20 June 2020 were included in this study. If the time interval between the estimated onset of symptoms and admission to the emergency room was >120 min, it was considered as a prehospital delay. The Beck Depression Inventory (BDI), the Beck Anxiety Inventory (BAI), and Padua Inventory-Washington State University Revision (PI-WSUR) test were used to assess Contamination-Obbsessive compulsive disorder (C-OCD). RESULT: The same period STEMI count compared to the previous year decreased 25%. The duration of symptoms onset to hospital admission was longer in the first month compared to second and third months (180 (120-360), 120 (60-180), and 105 (60-180), respectively; P = 0.012). Multivariable logistic regression (model-2) was used to examine the association between 7 candidate predictors (age, gender, diabetes mellitus (DM), hypertension, smoking, pain-onset time, and coronary artery disease (CAD) history), PI-WSUR C-OCD, and admission month with prehospital delay. Among variables, PI-WSUR C-OCD and admission month were independently associated with prehospital delay (OR 5.36 (2.11-13.61) (P = 0.01); 0.26 (0.09-0.87) p < 0.001] respectively]. CONCLUSION: Our study confirmed that contamination obsession was associated with prehospital delay of STEMI patients, however anxiety and depression level was not associated during the pandemic.

Prognostic significance of malnutrition scores in elderly patients for the prediction of contrast-induced acute kidney injury.

BACKGROUND: Malnutrition reflects the general condition of a patient including physical condition, protein turnover, and immune competence. Contrast-induced acute kidney injury (CI-AKI) is a disorder that adversely affects the prognosis of older adults. In our study, we aimed to show the relationship between CI-AKI and malnutrition status in elderly patients over 65 years of age with chronic coronary artery disease (CAD). METHODS: Study enrolled 360 consecutive patients with coronary angiography performed because of chronic coronary artery disease. Patients pre-procedural and post-procedural blood samples were taken and prognostic nutritional index (PNI), controlling nutritional status (CONUT) score, and geriatric nutritional risk index (GNRI) malnutrition scores were calculated. RESULTS: The median age of the patients included in the study was 69 (67-72, IQR) and CI-AKI was seen in 91 (25.2%) patients. Univariate regression analysis showed that age, diabetes mellitus, baseline creatinine, body weight-adapted contrast agent, haemoglobin, left ventricular ejection fraction, CONUT score, PNI score, and GNRI score were independent predictors of CI-AKI. In model 1, increase in CONUT score (2 to 5) (OR: 3.21 (2.11-4.88), in model 2, increase in PNI score (37.4 to 45) (OR: 0.34, (0.24-0.49)), and in model 3, increase in GNRI score (89.5 to 103.8) (OR: 0.55, (0.38-0.81)) were independently associated with the presence of CI-AKI. PNI showed better results than other models in discriminating the predictable capability for CI-AKI. CONCLUSION: Malnutrition assessment of elderly patients before performing diagnostic or interventional coronary procedures could help clinicians to identify patients with elevated risk for CI-AKI.

Effect of non-dipper pattern on echocardiographic myocardial work parameters in normotensive individuals.

BACKGROUND: It is known that non-dipper pattern (NDP) is associated with adverse outcomes in hypertensive patients. However, there is insufficient data on the outcome of NDP in normotensive individuals. Using myocardial work (MW) analysis, as a new echocardiographic examination method, this study aimed to determine the early myocardial effects of NDP in normotensive individuals. METHODS: This study included 70 normotensive individuals who were followed by ambulatory blood pressure monitoring (ABPM). The subjects were divided into two groups according to dipper pattern (DP) and NDP. Conventional, strain, and MW findings were compared between the groups by making echocardiographic evaluations. RESULTS: The demographic characteristics, laboratory parameters, and measurements of cardiac chambers, and left ventricular (LV) walls were similar between the groups. There was no statistical difference between the groups in terms of LV 3-2-4 chambers strains and global longitudinal strain (GLS) values. LVMW parameters, global work index (GWI), and global constrictive work (GCW) were not statistically different between groups (2012 ± 127, 2069 ± 137, p = 0.16; 2327 ± 173, 2418 ± 296, p = 0.18, respectively). However, global waste work (GWW) and global work efficiency (GWE) parameters were different between the groups (144 ± 63.9, 104 ± 24.8, p < 0.001; 93.2 ± 3.17, 95.4 ± 1.28, p < 0.001, respectively). In regression analysis, GWW was independently associated with NDP. GWW model showed better results with higher likelihood chi-square and R2 values than GLS model in discriminating the predictable capability for NDP status. CONCLUSION: The results of MW analysis in this study showed that GWW values were higher and the GWE values were lower in normotensive individuals with NDP.

The TAPSE/PASP ratio and MELD score in patients with advanced heart failure.

INTRODUCTION: The aim of this study was to explore the relationship between the tricuspid annular plane systolic excursion/pulmonary arterial systolic pressure (TAPSE/PASP) ratio and model for end-stage liver disease (MELD) score in patients with advanced heart failure. METHODS: A total of 103 patients with advanced heart failure evaluated for candidacy for heart transplantation were included in this study. TAPSE was measured by M­mode echocardiography and cardiac catheterization was performed. TAPSE/ PASP ratio and MELD score were calculated. RESULTS: The median age of patients was 49 (40.5-54) years and the majority were male (92%). The percentage of patients with ischemic cardiomyopathy was 40%. The mean value of the group's MELD score was 10 ± 3.3 and the median value of TAPSE/PASP 0.24 (0.18-0.34). There was a moderate negative correlation between TAPSE/PASP and MELD score (r: -0.38, p < 0.001). Right atrial pressure (RAP) and left ventricular end-diastolic pressure (LVEDP) were also negatively correlated with TAPSE/PASP (correlation coefficients were r: -0.562 and r: -0.575, respectively). In patients with a lower TAPSE/PASP ratio, MELD score, LVEDP and RAP were higher and tricuspid regurgitation was more severe, but there were no significant differences between cardiac output (CO) and mean aortic pressure (mean BP). The presence of ischemia was found to be an independent predictor for lower values of TAPSE/PASP. CONCLUSION: The lower TAPSE/PASP obtained on echocardiography may be a sign of the multi-organ failure defined as a high MELD score in patients with advanced heart failure.

Relative Fat Mass Index can be solution for obesity paradox in coronary artery disease severity prediction calculated by SYNTAX Score.

BACKGROUND: The relation between body mass index (BMI) and coronary artery disease (CAD) extension remains controversial. A new score was developed to estimate body fat percentage (BFP) known as Relative Fat Mass (RFM) Index. This study aimed to evaluate the value of RFM Index in predicting the severity of the CAD, compared with other anthropometric measurements. METHODS: A total of 325 patients with chronic CAD were investigated. RFM, BFP, BMI and other anthropometric characteristics of patients were measured before angiography. CAD severity was determined by SYNergy between percutaneous coronary intervention with TAXus and cardiac surgery trial (SYNTAX) Score. The association between SYNTAX Score and variables was evaluated using linear regression models. In order to compare the model performance, R-squared (R2), Akaike's information criterion, Bayesian information criterion and root mean square error were used. RESULTS: Univariate linear regression outcome variable, SYNTAX was used to determine whether there was any relationship between variables. Independent variables were included in the multivariable linear logistic regression models. The analysis showed that in model 1, RFM (ß coefficient: 2.31 (0.90 to 3.71), p=0.001)), diabetes mellitus (ß coefficient: 3.72 (1.67 to 3.76), p=0.004)), haemoglobin (ß coefficient: -2.12 (-3.70 to -0.53), p=0.03) and age (ß coefficient: 1.83 (0.29 to 3.37), p=0.02)) were statistically significant. The adjusted R2 values in model 1 were higher than model 2 (BFP) and model 3 (BMI) (0.155, 0.137 and 0.130, respectively), and χ2 values of RFM were higher than BFP and BMI (10.5, 3.4 and 1.0, respectively). CONCLUSION: RFM Index is a more reliable and compatible marker of obesity in showing the severity of CAD compared to BMI.

Comparison of outcomes of children with acute lymphoblastic leukemia treated with BMF protocol across 2 decades.

Acute lymphoblastic leukemia is the most common malignancy of childhood. The aim of this study is to compare the outcome of children with acute lymphoblastic leukemia treated with BFM protocol over two decades at our center. We retrospectively examined the files of 421 patients by dividing them into two groups by decade of treatment, 1995-2005 and 2006-2015. After excluding 117 patients, overall, 304 patients were included in the analysis. From the first to the second decade, the proportion of patients over 12 years of age increased from 7.1% to 16.8% (p < 0.04), the high-risk group increased from 15.5% to 19.5% and patients with central nervous system leukemia increased from 5.2% to 11.4%. The relapse rate remained relatively unchanged during this period (from 12.9% to 12.7%), while the mortality rate decreased from 18.7% to 15.4% (p > 0.05) and the death rate during remission induction treatment decreased from 3.9% to 0.7%. The mortality rate of high-risk and standard-risk patients decreased from 62.5% to 34.5% (p < 0.05) and 11.1% to 3.0% (p > 0.05), respectively. The 5-year overall survival and event-free survival rates for standard-, medium- and high-risk patients were 92.7% ± 6.0%, 87.9% ± 4.7%, and 54.7% ± 13.3% and 92.5% ± 6.3%, 83.2% ± 5.5%, and 48.7% ± 14.7%, respectively. For the cohort, the 5-year overall survival rate was 83.2% ± 4.1% and the event-free survival rate was 79.9% ± 4.7%. These results demonstrate the impact of a standard protocol, experience of staff, achieving better risk stratification on treatment success.

The effect of P wave indices on new onset atrial fibrillation after trans-catheter aortic valve replacement.

OBJECTIVES: Data is scarce regarding the relation between P wave indices and new onset atrial fibrillation (NOAF) after trans-catheter aortic valve replacement (TAVR). AIMS: The present study aimed to find out certain characteristics of P wave that may predict NOAF after TAVR procedure. METHOD: Patients with severe calcific aortic stenosis who had undergone TAVR procedure between 2013 and 2019 in two centers were investigated. P wave abnormalities that have been resumed to reflect impaired atrial conduction; partial and advanced inter atrial block (IAB), P-wave terminal force in lead V1, P wave dispersion, reduced amplitude of P- wave in lead I, P wave peak time in D2 and V1 were evaluated on pre- procedural 12 derivation surface electrocardiography (ECG). The relationship between these parameters and incidence of NOAF during index hospitalization was evaluated. RESULTS: A total of 227 consecutive patients (median age 79 [74-83]; 134 [59%] female) were included in the study. NOAF occurred in 46 (20.3%) patients. P wave duration, P wave dispersion, number of patients with partial and advanced IAB, left atrium diameter, STS score were higher in NOAF patients. Use of general anesthesia and history of prior open heart surgery were also more frequent in NOAF group. In multivariable logistic regression analysis; advanced IAB (OR 6.413 [2.555-16.095] p < 0.01), P wave dispersion (OR 3.544 [1.431-8.780] p = 0.006) and use of general anesthesia (OR 2.736 [1.225-6.109] p = 0.014) were independent predictors of NOAF. CONCLUSION: Among P wave abnormalities evaluated on pre-procedural 12-derivation surface ECG, advanced IAB and P wave dispersion may predict NOAF after TAVR procedure.

Low-dose Immune Tolerance Induction in Hemophilia: A Single-Center Experience.

INTRODUCTION: The development of inhibitors against factors VIII/IX is the most serious complication in hemophilia. The best treatment strategy for inhibitor eradication is immune tolerance induction (ITI). The aim of this study was to evaluate patients treated with low-dose ITI at a single center with limited resources. MATERIALS AND METHODS: In total, 29 (8.05%) of 360 hemophilia A patients exhibited inhibitors. The data from hemophilia patients with inhibitors undergoing ITI between 1999 and 2017 were collected and analyzed. RESULTS: Seventeen ITIs administered to 15 hemophilia A patients with inhibitors were analyzed, and the data from 13 ITIs conducted in 12 patients were evaluated. The median age at ITI onset was 10 years (range: 1.25 to 52 y). The maximum inhibitor titer before ITI was 30 Bethesda Units (BU) (range: 4.48 to 135), and the median inhibitor titer was 1.25 BU (range: 0 to 5.6) at the onset of ITI. The median time interval between the inhibitor development and ITI onset was 60 months (range: 7 to 264 mo). The median inhibitor titer during ITI was 3.4 BU (range: 0 to 158.7). At the end of the treatment, 4 of the 12 patients (33.3%) exhibited a complete response, 4 (33.3%) had partial responses (with continuing ITI), and 4 (33.3%) exhibited ITI failure. CONCLUSIONS: Treatment of hemophilia patients with inhibitors is challenging, and ITI is the best treatment method; however, a high-dose daily ITI regimen cannot be given to every patient in every country due to its high cost. Our results show that low-dose ITI may be a choice for selected patients with inhibitors.

Pediatric Stroke: A Single-Center Experience.

The aim of this study was to evaluate the clinical characteristics, risk factors, treatment, and outcomes of pediatric stroke cases. A total of 118 patients diagnosed with arterial ischemic stroke (AIS), hemorrhagic stroke, and sinovenous thrombosis (SVT) between January 2000 and December 2011 were included. Neonatal cases were excluded. Demographic and clinical findings were retrospectively examined from medical records. We identified 118 patients with stroke. The age of the patients ranged from 1 to 215 months (17.92 y), with a mean age of 5.19±5.25 years. AIS accounted for the majority of cases (n=69, 58.5%), and the major etiology was cardiac disease (17%). Hemorrhagic stroke accounted for 19.5% (n=23) of the cases, and late hemorrhagic disease of the newborn was the major etiology (43%, n=10). SVT accounted for 22% (n=26) of the cases, and the major etiology was otitis media-mastoiditis (27%, n=7). Hemiplegia and headache were the most frequent symptoms for AIS and SVT, respectively. Stroke is rare in children compared with adults; however, it is detected more frequently with better imaging techniques and increased awareness. We found that children with AIS presented more commonly with hemiplegia and children with SVT with headache and strabismus. We did not find an association between thrombophilia and stroke.

Causes of Hypochromic Microcytic Anemia in Children and Evaluation of Laboratory Parameters in the Differentiation.

Most common causes of microcytic anemia in children are iron deficiency anemia (IDA) and thalassemia. Differentiation of these and detection of coexistence is essential for genetic counseling and to set a treatment plan. Aim is to characterize the frequency of IDA and thalassemia trait (TT) in children presenting with hypochromic, microcytic anemia and to define the significance of blood count parameters in differential diagnosis. Of the 200 enrolled, 107 were male (53.5%). In total 154 had IDA (77%), 27 had ß-TT (13.5%), and in 11 (5.5%) both conditions coexisted. Eight patients had α-thalassemia gene mutations, 3 of these also had IDA. RBC, MCV, Mentzer index, serum iron, TIBC, ferritin were significantly different between IDA and ß-TT patients (P<0.001); however, RDW was not different between the 2 groups (P>0.05). Sensitivity and specificity of Mentzer index for the detection of ß-TT were 100% and 69.4%, respectively. The positive and negative predictive values of Mentzer index in diagnosing ß-TT were 36.6% and 100%, respectively. Differential diagnosis of microcytic anemia is important in children, especially in regions where IDA and thalassemia are both prevalent. We found that 7% of children referred to our clinic for hypochromic, microcytic anemia had both TT and IDA. Our data showed that serum iron, ferritin, TIBC, MCV, and Mentzer index were all valuable markers in diagnosing IDA and were significantly different compared with ß-TT patients; RDW was not different between the 2 groups.

Comparison of 30-Day MACE between Immediate versus Staged Complete Revascularization in Acute Myocardial Infarction with Multivessel Disease, and the Effect of Coronary Lesion Complexity.

Background and objective: In patients with acute myocardial infarction and multivessel disease, the timing of intervention to non-culprit lesions is still a matter of debate, especially in patients without shock. This study aimed to compare the effect of multivessel intervention, performed at index percutaneous coronary intervention (PCI) (MVI-I) or index hospitalization (MVI-S), on the 30-day results of acute myocardial infarction (AMI), and to investigate the effect of coronary lesion complexity assessed by the Syntax (Sx) score on the timing of multivessel intervention. Materials and methods: We enrolled 180 patients with MVI-I, and 425 patients with MVI-S. The major adverse cardiovascular events (MACE) for this study were identified as mortality, nonfatal myocardial infarction, nonfatal stroke, acute heart failure, ischemia driven revascularization, major bleeding, and acute renal failure developed within 30 days. Results: The unadjusted MACE rates at 30 days were 11.2% and 5% among those who underwent MVI-I and MVI-S, respectively (OR 3.02; 95% confidence interval (CI) 1.51⁻6.02; p=0.002). Associations were statistically significant after adjusting for covariates in the penalized multivariable model (adjusted OR 2.06; 95%CI 1.02⁻4.18; p=0.043), propensity score adjusted multivariable model (adjusted OR 2.46; 95%CI 1.19⁻5.07; p=0.015), and IPW (adjusted OR 2.11; 95%CI 1.28⁻3.47; p=0.041). We found that the Syntax score of lesions did not affect the results. Conclusion: MVI-S was associated with a lower incidence of major adverse cardiovascular events within 30 days after discharge.

Treatment of plasminogen deficiency patients with fresh frozen plasma.

Congenital plasminogen (Plg) deficiency leads to the development of ligneous membranes on mucosal surfaces. Here, we report our experience with local and intravenous fresh frozen plasma (FFP). We retrospectively reviewed medical files of 17 patients and their eight first-degree relatives. Conjunctivitis was the main complaint. Thirteen patients were treated both with intravenous and conjunctival FFP. Venous thrombosis did not develop in any. Genetic evaluation revealed heterogeneous mutations as well as polymorphisms. Diagnosis and treatment of Plg deficiency is challenging; topical and intravenous FFP may be an alternative treatment.

Hemophagocytic Lymphohistiocytosis Associated With Visceral Leishmaniasis.

This is the report of a 2-year-old boy who presented with fever, cytopenia, and splenomegaly. The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) and treated with HLH-2004 protocol. Repeated bone marrow aspiration showed amastigotes on follow-up. In endemic countries, visceral leishmaniasis should be considered in the differential diagnosis to avoid chemotherapy toxicity.

Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate.

Purpura fulminans in neonates is a rapidly progressive thrombotic disorder manifesting as hemorrhagic skin infarction and disseminated intravascular coagulation. Being inherited in an autosomal dominant manner, it is a medical emergency. Clinical presentations of patients may vary depending on the genetic mutations. Retinal and intracranial hemorrhages are the worst clinical scenarios with persistent morbidity. During acute phase, fresh frozen plasma, protein C concentrates and anticoagulant therapy should be administered rapidly. Here we report a patient with homozygous protein C deficiency.

Infarction of the septomarginal band and tricuspid papillary muscle rupture related to alcohol septal ablation for hypertrophic cardiomyopathy.

We presented a 77-year-old man with hypertrophic obstructive cardiomyopathy applied with flail tricuspid leaflet and severe tricuspid regurgitation leading to right heart failure 2 months after the failed septal ablation. The ruptured anterior tricuspid papillary muscle resulted from infarction of the base of anterior papillary muscle of the right ventricle (RV) confirmed by magnetic resonance imaging. As the septomarginal band is frequently lit up by intracoronary contrast that particular attention should be paid to the RV papillary muscles. And, if the papillary muscles or the RV free wall is brightened, then the use of that septal artery should be avoided.