Etiological Subgroups Research Articles

Accuracy of Multi-organ Point-of-Care Ultrasound for Acute Kidney Injury Etiologies.

This study investigated the diagnostic performance of point-of-care ultrasound (POCUS) for acute kidney injury (AKI) etiological subgroups in emergency department (ED) patients. Multi-organ POCUS including kidney, bladder, inferior vena cava (IVC), lung and cardiac examinations were used to identify five AKI subgroups: hypovolemia, reduced cardiac output, systemic vasodilatation and renal vasomodulation, renal and post-renal. One hundred sixty-five AKI patients were included in the study. The most diagnostic parameter in the post-renal group was the presence of any hydronephrosis, with a sensitivity of 93.3% (95% confidence interval [CI]: 68.1-99.8) and specificity of 85.9% (95% CI: 79.3-91.1). For the reduced cardiac output group, the most diagnostic parameter was IVC maximum diameter >17 mm with a sensitivity of 100% (95% CI: 83.2-100) and specificity of 70.2% (95% CI: 61.6-77.7). For the hypovolemia group, the most diagnostic parameter was IVC maximum diameter ≤17.9 mm with a sensitivity of 81.2% (95% CI: 71.2-88.8) and specificity of 56.5% (95% CI: 44-68.4). For the systemic vasodilatation and renal vasomodulation group, the most diagnostic parameter was diffuse ascites with a sensitivity of 56.3% (95% CI: 29.9-80.2) and specificity of 89.9% (95% CI: 83.8-94.2). None of the parameters were significant for the renal group. We concluded that multi-organ POCUS is of diagnostic value for AKI subgroups.

Trigger Factors for Stroke in Young Adults: A Case-Crossover Study.

Causes of stroke in young adults differ from those in the elderly individuals, and in a larger percentage, no cause can be determined. To gain more insight into the etiology of (cryptogenic) stroke in the young population, we investigated whether trigger factors, such as short-lasting exposure to toxins or infection, may play a role. Patients aged 18-49 years with a first-ever ischemic stroke or intracerebral hemorrhage (ICH) in 17 participating centers in the Netherlands completed a questionnaire about exposure to 9 potential trigger factors in hazard periods and on a regular yearly basis. A case-crossover design was used to assess relative risks (RRs) with 95% confidence intervals (95% CIs) by the Mantel-Haenszel case-crossover method, for any stroke (ischemic stroke and ICH combined) and for different etiologic subgroups of ischemic stroke. One thousand one hundred forty-six patients completed the questionnaire (1,043 patients with an ischemic stroke and 103 with an ICH, median age 44.0 years, 52.6% men). For any stroke, an increased risk emerged within 1 hour of cola consumption (RR 2.0, 95% CI 1.5-2.8) and vigorous physical exercise (RR 2.6, 95% CI 2.2-3.0), within 2 hours after sexual activity (RR 2.4, 95% CI 1.6-3.5), within 4 hours after illicit drug use (RR 2.8, 95% CI 1.7-4.9), and within 24 hours after fever or flu-like disease (RR 14.1, 95% CI 10.5-31.2; RR 13.9, 95% CI 8.9-21.9). Four trigger factors increased the risk of other determined and cryptogenic ischemic stroke, 3 that of cardioembolic stroke, 2 that of large vessel atherosclerosis and likely atherothrombotic stroke combined and stroke with multiple causes, and none that of stroke due to small vessel disease. We identified cola consumption, vigorous physical exercise, sexual activity, illicit drug use, fever, and flu-like disease as potential trigger factors for stroke in the young population and found differences in the type and number of trigger factors associated with different etiologic subgroups of ischemic stroke. These findings might help in better understanding the pathophysiologic mechanisms of (cryptogenic) stroke in the young population.

Computed tomography characteristics of acute pancreatitis based on different etiologies at different onset times: a retrospective cross-sectional study.

BackgroundThe clinical characteristics and imaging findings of acute pancreatitis (AP) are different across the various etiologies, the results are conflicting, and their time from symptom onset to imaging varies. The imaging findings of different etiologies at different onset times are unclear. This study aimed to investigate the computed tomography (CT) characteristics of AP based on different etiologies at different onset times.MethodsPatients who underwent plain and contrast-enhanced computed tomography (CECT) for the first attack of AP in 3 hospitals (Affiliated Hospital of North Sichuan Medical College, Chinese People’s Liberation Army Western Theater General Hospital, and Suining Central Hospital) from 2015 to 2019 were recruited. According to the different etiologies of AP, the patients were divided into 5 subgroups: biliary AP (n=591), alcoholic AP (n=267), hypertriglyceridemic AP (n=258), mixed causes subgroups (n=199), and “other/idiopathic” AP (n=545). According to the time from onset to CT examination (e.g., 1–3, 4–7, 8–14, 15–28, and >28 days), the onset time was divided into 5 respective phases (I-V). The CT characteristics and clinical and laboratory features were retrospectively reviewed and compared among the different etiology subgroups and onset time.ResultsThe positive rate of CT findings in AP diagnosis based on CECT was 96.7% (1,860/1,924). Necrotizing pancreatitis (NP) occurred in 33.2% (617/1,860) of AP patients with positive CECT findings. Among patients with NP, local complications and severe AP of the modified CT severity index (MCTSI) increased over time in those with biliary AP from 17.1%, 25.2%, and 20.0% in Phase I to 42.9%, 44.0%, and 39.7% in Phase IV [all P<0.05, 95% confidence interval (CI): 0.15 to 0.52, 0.28 to 0.63, and 0.18 to 0.82, respectively]. In contrast, NP, local complications and severe AP of MCTSI in those with hypertriglyceridemic AP decreased over time from 24.3%, 22.5%, and 22.7% in Phase I to 1.3%, 1.2%, and 1.9% in Phase V (all P<0.05, 95% CI: 3.20 to 181.74, 3.31 to 175.74, and 2.00 to 120.78, respectively).ConclusionsThe proportion of NP was 33.2% of positive CECT findings. There may be differences in the CT and clinical manifestations of the different etiologies, and those differences may be related to the onset time.

Association between cumulus cells—mRNA levels of AMHR2 and FSHR with oocyte maturity

BackgroundThe gene expression of anti-Mullerian hormone receptor type 2 (AMHR2) and follicle stimulating hormone receptor (FSHR) in cumulus cells (CCs) isolated from mature and immature oocytes was studied as a possible non-invasive approach for determining oocyte maturity and quality. The CCs of 100 infertile women with different etiologic factors were subdivided into control (CCs from MII) and case (CCs from GV) group. Q-PCR was used to evaluate FSHR and AMHR2 mRNA expression levels in CCs from mature and immature oocytes.ResultsAMHR2 and FSHR genes are significantly overexpressed (4–6 fold) in CCs from immature relative to mature oocyte. The expression level of AMHR2 gene in terms of etiologic subgroups is significantly different (P value 0.000). FSHR mRNA expression levels in CCs show no significant difference regarding etiologic subgroups (P value 0.575).ConclusionIt seems that determining the expression level of AMHR2 and FSHR genes in CCs could help to understanding molecular mechanism of oocyte maturation process.

Unique Pain Responses in Different Etiological Subgroups of Intellectual and Developmental Disabilities.

We studied whether there exist variations in pain responses between different intellectual and developmental disability (IDD) etiologies. Self-reports and facial expressions (Facial Action Coding System = FACS) were recorded during experimental pressure stimuli and compared among 31 individuals with IDD-13 with cerebral palsy (CP), nine with Down syndrome (DS), nine with unspecified origin (UIDD)-and among 15 typically developing controls (TDCs). The CP and DS groups had higher pain ratings and FACS scores compared to the UIDD and TDC groups, and steeper stimulus-response functions. The DS group exhibited the most diverse facial expressions. There were variations in the foci of facial expressions between groups. It appears that different IDD etiologies display distinct pain responses.

Cohort study of infantile epileptic spasms syndrome: etiological analysis and treatment of corticosteroids

BackgroundInfantile epileptic spasms syndrome (IESS) is the most common type of severe epilepsy in infants. However, etiological frequency and optimized therapy, particularly corticosteroid regimen and dose, remain unknown. MethodsAn ambispective study of an IESS-diagnosed cohort was conducted. Etiologies were evaluated based on the 2017 International League Against Epilepsy classification system. Patients received intravenous dexamethasone or methylprednisolone for 3–5 consecutive days, followed by usual-dose (2 mg/kg/d) oral prednisone for 60–90 days with tapering doses for 1–2 months or high-dose (4 mg/kg/d) oral prednisone for 9–11 days with tapering doses for 2–4 weeks. Treatment responses were compared between the usual and high-dose prednisone groups after propensity score matching. Correlation analysis between treatment responses and underlying etiology was performed. ResultsOf the 441 included participants, 218 (49.4%) cases had proven etiologies. The most common etiology of IESS was acquired-structural (23.6%), followed by genetic (15.4%) and congenital-structural (7.0%). Hypoxic-ischemic encephalopathy (55, 52.8%) was the most common acquired-structural etiology. Among the 242 patients administered corticosteroids, 95 received usual-dose oral prednisone and 147 received high-dose oral prednisone. After propensity score matching, 54 patients were included in the usual-dose and high-dose groups, respectively, and treatment effectiveness was compared. There were no significant differences in seizure freedom at days 13–14 (55.6% vs. 51.9%, p = 0.700) and continued seizure freedom between days 14–42 (29.6% vs. 38.9%, p = 0.311) post corticosteroid administration between the usual- and high-dose prednisone groups. The proportion of children achieving seizure cessation at days 13–14 (χ2 = 1.470, p = 0.698) and days 14–42 (χ2 = 0.928, p = 0.836) was similar in the different etiological subgroups. Unknown etiological group showed significantly higher resolution of hypsarrhythmia than other etiological groups (χ2 = 10.761, p = 0.009). Both usual-dose and high-dose group showed tolerance to full-dose corticosteroids and similar adverse events over the observation period. ConclusionIESS etiology was primarily related to structural causes. Clinical response in short-term follow-up was independent of prednisone dosage and underlying etiology. Better EEG responses may occur in patients with unknown etiology.

Kissing Viabahn VBX stent graft reconstruction of thoracic central veins for management of superior vena cava syndrome

ObjectiveIn the present study, we evaluated the technical and clinical outcomes of thoracic central vein reconstruction for superior vena cava (SVC) syndrome using kissing Viabahn VBX stent grafts (W.L. Gore & Associates, Flagstaff, AZ). MethodsAll adult patients with SVC syndrome who had undergone attempted bilateral brachiocephalic vein-to-SVC reconstruction using kissing VBX stent grafts at an academic hospital between August 2019 and February 2021 were reviewed. The technical results, adverse events, imaging follow-up findings, and clinical outcomes were recorded. Patency over time was assessed using Kaplan-Meier analysis. ResultsA total of 28 patients (16 women and 12 men; mean age, 52.0 years) constituted the study cohort. Of the 28 patients, 17 (60.7%) had had benign and 11 (39.3%) malignant etiologies. The presenting symptoms included neck swelling (n = 17; 60.7%), bilateral upper extremity swelling (n = 15; 53.6%), dyspnea (n = 7; 25%), unilateral upper extremity swelling (n = 4; 14.3%), and dysphagia (n = 1; 3.6%). SVC reconstruction with VBX stent grafts in a kissing configuration was successfully completed in 27 of the 28 patients (96.4%). Four major adverse events were noted in the benign etiology subgroup (23.5%), including intraprocedural hemopericardium (n = 3) and delayed pneumothorax (n = 1). Of the 28 patients, 27 (96.4%) had experienced resolution of their presenting symptoms. The mean clinical follow-up for the living patients was 358.8 ± 77.2 days (range, 78-645 days). The mean imaging follow-up for the living patients was 272.6 ± 91 days (range, 26-594 days). The primary, primary-assisted, and secondary patency rates at 12 months were 71.8%, 88.8%, and 100%, respectively. ConclusionsFor the management of SVC syndrome, thoracic central vein reconstruction with kissing VBX stent grafts was feasible with a high rate of symptom resolution and acceptable patency. However, this technique should not be recommended for those with benign SVC syndrome owing to the high risk of cardiac tamponade.

Incremental diagnostic value of 18F-Fluetemetamol PET in differential diagnoses of Alzheimer’s Disease-related neurodegenerative diseases from an unselected memory clinic cohort

To evaluate the incremental diagnostic value of 18F-Flutemetamol PET following MRI measurements on an unselected prospective cohort collected from a memory clinic. A total of 84 participants was included in this study. A stepwise study design was performed including initial analysis (based on clinical assessments), interim analysis (revision of initial analysis post-MRI) and final analysis (revision of interim analysis post-18F-Flutemetamol PET). At each time of evaluation, every participant was categorized into SCD, MCI or dementia syndromal group and further into AD-related, non-AD related or non-specific type etiological subgroup. Post 18F-Flutemetamol PET, the significant changes were seen in the syndromal MCI group (57%, p < 0.001) involving the following etiological subgroups: AD-related MCI (57%, p < 0.01) and non-specific MCI (100%, p < 0.0001); and syndromal dementia group (61%, p < 0.0001) consisting of non-specific dementia subgroup (100%, p < 0.0001). In the binary regression model, amyloid status significantly influenced the diagnostic results of interim analysis (p < 0.01). 18F-Flutemetamol PET can have incremental value following MRI measurements, particularly reflected in the change of diagnosis of individuals with unclear etiology and AD-related-suspected patients due to the role in complementing AD-related pathological information.

The effectiveness and tolerability of clobazam in the pediatric population: Adjunctive therapy and monotherapy in a large-cohort multicenter study

ObjectiveTo evaluate the effectiveness and tolerability of clobazam therapy in the pediatric population in terms of seizure semiology, epileptic syndromes, and etiological subgroups. MethodsA retrospective cohort study was conducted consisting of 1710 epileptic children from eight centers in seven geographic regions of Turkey. The initial efficacy of clobazam therapy was evaluated after three months of treatment. The long-term effectiveness of the drug, overall seizure outcomes, and overall therapeutic outcomes were evaluated during 12 months of therapy. ResultsAnalysis of initial efficacy after the first three months of clobazam therapy showed that 320 (18.7 %) patients were seizure-free, 683 (39.9 %) had > 50 % seizure reductions, and 297 (17.4 %) had < 50 % seizure reductions. A positive response (seizure-free and >50 % seizure reduction) was determined for focal-onset (62.3 %) seizures, epileptic spasms (61.5 %), and generalized onset seisures (57.4). The highest positive response rate among the epileptıc syndromes was for self-limited epilepsy with centrotemporal spikes (SeLECTS). The highest negative response rate was for developmental and/or epileptic encephalopathies (DEEs). Magnetic resonance imaging (MRI) revealed a structural etiological diagnosis in 25.8 % of the cohort. A higher positive response rate was observed at MRI in patients with sequelae lesions than in those with congenital lesions. The seizure recurrence rate was higher in the patient group with epilepsy wıth genetic and metabolic causes, in individuals with more than one seizure type, and in those using three or more antiseizure drugs. ConclusionsThis cohort study provides additional evidence that clobazam is an effective and well-tolerable drug with a high seizure-free rate (18.7 %), a significant seizure reduction rate (57.3 %), and with excellent overall therapeutic outcomes with a low seizure relapse rate and considerable reversible benefits in the pediatric population.

Infantile spasms: Assessing the diagnostic yield of an institutional guideline and the impact of etiology on long-term treatment response.

Neuroimaging and genetic testing have been proposed for diagnostic evaluation of infantile spasms (IS), establishing etiology in ~60% of multicenter IS cohorts. A retrospective analysis of the yield of diagnostic etiology following an institutionally established guideline for investigation/treatment of IS was conducted, and the association between etiological subgroups and sustained response to standard treatment was evaluated. Etiology of IS, neuroimaging, and genetic results were extracted from clinical records. Etiology was categorized as acquired or nonacquired, the latter including syndromic patients, nonsyndromic patients with confirmed etiology, and unknown cases. Regression analyses, using clinical variables including subtypes of etiology, were conducted to determine which factors correlated with favorable (spasm freedom at last follow-up after two or fewerstandard treatments) versus unfavorable treatment outcome (refractory spasms despite two standard treatments or relapse). We included 127 IS patients (60% males) with a follow-up of 2.4years (range = .6-5years). All patients had neuroimaging, and 95% of patients in the nonacquired category (103 of 108 patients) had genetic testing. Etiology was identified in 103 of 127 (81%, 95% confidence interval = .73-.86). At last follow-up, 42 (33%) patients had favorable treatment outcome. No difference in treatment response was observed between acquired and nonacquired etiologies. Among patients with nonacquired etiologies, developmental delay prior to spasms onset increased the odds of unfavorable treatment outcome (p=.014), whereas a clearly recognizable dysmorphic/syndromic etiology was associated with a lower risk for treatment failure (p=.034). In nonacquired etiology without a recognizable dysmorphic/syndrome but with a genetic etiology, unfavorable treatment outcome was more likely (p=.043). Rigorous evaluation with neuroimaging and genetic testing yields an etiological diagnosis in most patients with IS. Among patients with a nonacquired etiology, those with recognizable dysmorphic/syndromic diagnosis had a higher likelihood of a favorable treatment outcome, whereas the absence of such a finding, when associated with an identifiable genetic diagnosis, was associated with unfavorable treatment outcomes.

Predictors of Recurrence in Symptomatic Large Artery Atherosclerosis and Cryptogenic Strokes—A Comparative Study

Background and Purpose: Ischemic stroke has highest recurrence risk in the first-year, ranging from 5.7% to 14%, depending on etiology, with highest reported following cardioembolism and large artery atherosclerosis (LAA), while it is not negligible in cryptogenic strokes. We evaluated the utility of clinical, imaging parameters along with electrographic and echocardiographic biomarkers of atrial dysfunction in 2 etiological groups, namely LAA and cryptogenic strokes and compared the predictors of recurrence risk at 1 year. Methodology: All acute ischemic strokes admitted to Comprehensive Stroke Care Centre, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India for 1 year (July 2019 till June 2020) with 1 year completed follow-up were screened from electronic records. Strokes secondary to LAA and undetermined cause were compared for their clinical, imaging, and cardiac variables to predict risk of recurrence in the first year. Results: Of the 179 patients, 93 had strokes secondary to LAA and 86 had cryptogenic strokes (CS). Seventy-five patients had more than 1 event at presentation/follow-up. Recurrence risk did not differ between the 2 etiological subgroups at 1 year. Hypertension ( P = .016), multiple territory strokes ( P = .02), and dilated left ventricle (LA) chamber ( P = .047) were independently associated with recurrence risk in the entire cohort as well as within the undetermined group. Early hospitalization within 48 h reduced the overall recurrence risk ( P = .01), thus emphasizing the role of early etiological evaluation and initiation of secondary prevention in reducing future events, irrespective of etiology. Conclusion: In optimally managed LAA and cryptogenic strokes, presence of hypertension, multiterritorial infarcts, and dilated LA chambers increases the recurrence risk pointing to a likely cardiac substrate itself contributing to future stroke risk.

Effect of stroke etiology on endovascular thrombectomy with or without intravenous alteplase: a subgroup analysis of DIRECT-MT

BackgroundStroke etiology might influence the clinical outcomes in patients with large vessel occlusion receiving endovascular treatment (EVT) with or without thrombolysis.ObjectiveTo examine whether stroke etiology resulted in different efficacy and...

Diagnostic Performance of Spin-Echo Echo-Planar Imaging Magnetic Resonance Elastography in 3T System for Noninvasive Assessment of Hepatic Fibrosis.

ObjectiveTo validate the performance of 3T spin-echo echo-planar imaging (SE-EPI) magnetic resonance elastography (MRE) for staging hepatic fibrosis in a large population, using surgical specimens as the reference standard.Materials and MethodsThis retrospective study initially included 310 adults (155 undergoing hepatic resection and 155 undergoing donor hepatectomy) with histopathologic results from surgical liver specimens. They underwent 3T SE-EPI MRE ≤ 3 months prior to surgery. Demographic findings, underlying liver disease, and hepatic fibrosis pathologic stage according to METAVIR were recorded. Liver stiffness (LS) was measured by two radiologists, and inter-reader reproducibility was evaluated using the intraclass correlation coefficient (ICC). The mean LS of each fibrosis stage (F0–F4) was calculated in total and for each etiologic subgroup. Comparisons among subgroups were performed using the Kruskal–Wallis test and Conover post-hoc test. The cutoff values for fibrosis staging were estimated using receiver operating characteristic (ROC) curve analysis.ResultsInter-reader reproducibility was excellent (ICC, 0.98; 95% confidence interval, 0.97–0.99). The mean LS values were 1.91, 2.41, 3.24, and 5.41 kPa in F0–F1 (n = 171), F2 (n = 26), F3 (n = 38), and F4 (n = 72), respectively. The discriminating cutoff values for diagnosing ≥ F2, ≥ F3, and F4 were 2.18, 2.71, and 3.15 kPa, respectively, with the ROC curve areas of 0.97–0.98 (sensitivity 91.2%–95.9%, specificity 90.7%–99.0%). The mean LS was significantly higher in patients with cirrhosis (F4) of nonviral causes, such as primary biliary cirrhosis (9.56 kPa) and alcoholic liver disease (7.17 kPa) than in those with hepatitis B or C cirrhosis (4.28 and 4.92 kPa, respectively). There were no statistically significant differences in LS among the different etiologic subgroups in the F0–F3 stages.ConclusionThe 3T SE-EPI MRE demonstrated high interobserver reproducibility, and our criteria for staging hepatic fibrosis showed high diagnostic performance. LS was significantly higher in patients with non-viral cirrhosis than in those with viral cirrhosis.

Increased serum citrullinated histone H3 levels in COVID-19 patients with acute ischemic stroke

Prevalence of acute ische­mic stroke (AIS) is increased in patients with coronavirus disease 2019 (COVID-19). A proposed hypothesis is increased virus-induced propensity to hypercoagulation resulting in arterial thrombosis. Our aim was to provide evidence regarding the involvement of neutrophil extracellular trap (NET) formation (NETosis) in COVID-19 related AIS. Twenty-six consecutively enrolled COVID-19+ pneumonia patients with AIS, 32 COVID-19+ pneumonia patients without AIS and 24 AIS patients without COVID-19 infection were included to the study. Clinical characteristics of recruited patients were collected. Serum levels of citrullinated histone H3 (H3Cit; a factor of NETosis), IL-8 and C5a (mediators associated with NETosis) were measured by ELISA (enzyme-linked immunosorbent assay). H3Cit levels were significantly higher in COVID-19+ AIS patients, whereas all study groups showed comparable IL-8 and C5a levels. There were no significant differences among etiological subgroups of AIS patients with or without COVID-19. AIS patients with COVID-19 showed relatively increased white blood cell, lymphocyte, neutrophil, D-dimer, C-reactive protein and procalcitonin levels than control groups. H3Cit levels did not correlate with clinical/prognostic features and inflammation parameters. H3Cit and IL-8 levels were correlated in COVID-19 patients without stroke but not in COVID-19 positive or negative AIS patients. Increased levels of inflammation parameters and H3Cit in COVID-19 related AIS suggest that NETosis may cause susceptibility to arterial thrombosis. However, H3Cit levels do not correlate with clinical severity measures and inflammation parameters diminishing the prognostic biomarker value of NETosis factors. Moreover, the link between IL-8 and NETosis appears to be abolished in AIS.

Effect of Tricuspid Valve Repair or Replacement on Survival in Patients With Isolated Severe Tricuspid Regurgitation

Controversies remain in the management strategy for isolated tricuspid regurgitation (TR) because of adverse prognosis and uncertainties regarding the benefits of tricuspid valve surgery. We compared the characteristics and outcomes of a large cohort of patients with isolated TR, based on downstream tricuspid valve surgery versus medical management. Consecutive patients with isolated TR graded at least moderate-to-severe by echocardiography identified between January 2004 and December 2018 (n=9,031, age 70 ± 15 years, 60% women) were retrospectively studied. The primary end point was time to all-cause mortality during follow-up. Outcomes were compared by management strategy using unadjusted and adjusted survival and multivariable regression analyses. Tricuspid valve surgery was performed in 632 of 9,031 of the cohort (7%), including 514 valve repairs and 118 valve replacements, with in-hospital mortality in 19 patients (2.9%). Overall, there were 3,985 all-cause deaths (44%) over mean follow-up of 2.6 ± 3.3 years. Tricuspid valve surgery was independently associated with lower mortality rate during follow-up, with hazard ratios (HRs) of 0.53 (95% confidence interval [CI] 0.45 to 0.64), and the association persisted in both primary and secondary TR subgroups. Tricuspid valve surgery also had a significantly higher rate of infective endocarditis and heart failure hospitalizations rates during follow-up, at HRs of 5.55 (95% CI 4.00 to 7.71) and 1.29 (95% CI 1.16 to 1.43), respectively. In conclusion, tricuspid valve surgery is rarely performed in isolated TR, but it is independently associated with greater survival for the overall cohort and both primary and secondary etiology subgroups. Increasing the utilization of this surgery at specialized centers is encouraged to try to improve the clinical outcomes for this challenging clinical entity.

The Relationship Between Renal Function and Imaging Markers and Total Burden of Cerebral Small Vessel Disease.

Previous studies demonstrating the association between renal functions and cerebral small vessel diseases have usually focused on white matter hyperintensity in the general population or lacunar stroke patients. This study aimed to investigate the effects of renal function on imaging markers of cerebral small vessel disease and etiologic subtypes of stroke in patients with acute ischemic stroke or transient ischemic attack. A total of 356 consecutive patients with acute ischemic stroke or transient ischemic attack who were admitted to the Stroke Unit and underwent brain magnetic resonance imaging were evaluated. Demographic data, vascular risk factors, stroke etiology, estimated glomerular filtration rate and severity of cerebral small vessel disease markers, and total cerebral small vessel disease burden were evaluated. There was a significant inverse correlation between estimated glomerular filtration rate and total number of lacunes, periventricular and deep subcortical Fazekas scores, grade of enlarged perivascular spaces in the centrum semiovale, lobar and total cerebral microbleeds, and total cerebral small vessel disease burden. Impaired renal function was an independent risk factor for the presence of lacunes, deep cerebral microbleeds, and increased total burden. Renal function impairment and periventricular white matter hyperintensities were significantly associated with the etiologic subgroup of small vessel occlusion. The results were still significant after the exclusion of patients below 50 years of age. Our results indicate that there is a relationship between renal function impairment and increased total burden, as well as acute ischemic stroke/transient ischemic attack due to small vessel occlusion.

Treatment of Early-onset Scoliosis: Similar Outcomes Despite Different Etiologic Subtypes in Traditional Growing Rod Graduates.

It is unclear whether traditional growing rod (TGR) treatment outcomes vary by early-onset scoliosis (EOS) subtype. The goal of this study was to compare radiographic outcomes and complications of TGR treatment by EOS subtype. We queried an international database of EOS patients from 20 centers to identify "graduates" who had (1) undergone primary TGR treatment from 1993 to 2014; (2) completed TGR treatment; and (3) had an uneventful clinical examination within 6 months after completion of TGR treatment with no anticipated further intervention. We included 202 patients in 4 etiologic subgroups: neuromuscular (n=65), syndromic (n=57), idiopathic (n=52), and congenital (n=28). Mean age at surgery was 7.1 years (range, 1.6 to 14.9 y); mean duration of follow-up was 8 years (range, 2 to 18.6 y). The groups did not differ by mean age, body mass index, sex, number of lengthenings, or duration of follow-up. The following preoperative differences were significant: (1) greater mean major curve in the neuromuscular versus idiopathic subgroup; (2) shorter spinal height (T1-S1) in the congenital versus idiopathic subgroup; and (3) smaller proportion of ambulatory patients in the neuromuscular subgroup versus all other subgroups. We found no significant differences among subgroups in mean major curve correction or changes in thoracic height (T1-T12), spinal height, or global kyphosis at any point. Rates of deep surgical site infection, implant-related complications, and neurological complications were not different among subgroups. The medical complication rate was significantly lower in the idiopathic group compared with the other groups. Major curve correction and spinal and thoracic height increases did not differ significantly at any point by EOS subtype. Rates of deep surgical site infection, implant-related complications, and neurological complications did not differ by subtype. Except for the lower rate of medical complications in the idiopathic group, our findings suggest that, after TGR treatment, patients can expect similar outcomes regardless of their EOS subtype. Level III, therapeutic.

Impact of Etiological Cytogenetic Abnormalities on the Depth of Immunoparesis and Survival in Newly Diagnosed Multiple Myeloma

Introduction/BackgroundImmunoparesis, or low polyclonal immunoglobulin levels, is commonly seen in multiple myeloma (MM), and is associated with poor clinical outcomes. MM can be divided into subgroups with distinct biology and outcomes based on etiologic cytogenetic abnormalities. These include hyperdiploidy and translocations of t(11;14), t(4;14), t(14;16), and t(14;20), with the latter 3 associated with high-risk disease. We hypothesized that the different etiologic cytogenetic abnormalities drive bone marrow microenvironmental changes, resulting in different degrees of immunoparesis, and subgroup-dependent effects on clinical outcomes. Materials and MethodsWe performed a retrospective review of 985 newly diagnosed patients enrolled in the Myeloma IX and XI trials. Immunoglobulin levels, survival outcomes, and infection rates were evaluated for each cytogenetic subgroup. ResultsA significant proportion of patients with high-risk t(4;14), t(14;16), or t(14;20) had suppressed polyclonal immunoglobulins compared to standard-risk patients with hyperdiploidy or t(11;14). The clinical impact of immunoparesis depended on the cytogenetic subgroup, with the degree of IgM suppression effecting progression-free and overall survival only in the hyperdiploid subgroup. There was no significant difference in infection rates amongst the etiologic subgroups. ConclusionThese findings demonstrate that the etiologic cytogenetic subgroup influences the degree and clinical impact of immunoparesis. This suggests that the underlying cytogenetic abnormality affects remodeling of the bone marrow plasma cell niche, resulting in suppressed normal plasma cell function, and low immunoglobulin levels.

CARE AND CHARACTERISTICS OF PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION (PAH) IN US CLINICAL PRACTICE

CARE AND CHARACTERISTICS OF PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION (PAH) IN US CLINICAL PRACTICE

A pragmatic approach to risk assessment in pulmonary arterial hypertension using the 2015 European Society of Cardiology/European Respiratory Society guidelines

ObjectiveTo optimise treatment of patients with pulmonary arterial hypertension (PAH), the 2015 European Society of Cardiology/European Respiratory Society guidelines recommend using risk stratification, with the aim of patients achieving low-risk...