National Institutes Of Health Score Research Articles

Acute-phase proteins as indicators of disease severity and mortality in COVID-19 patients

The aim of the study was to conduct of relationship of acute-phase proteins (APPs) with the severity of COVID-19 defined by National Institutes of Health and according to the criteria of MEWS scale, with the presence of a cytokine storm, oxygen therapy and patient survival. We enrolled 96 patients with COVID-19 and 30 healthy people. The samples were taken on the day of admission and after 9 days on average. Not only commonly used APPs such as CRP, procalcitonin and ferritin and also rarely assayed proteins such as transferrin, haptoglobin, α1-acid glycoprotein and α1-antitrypsin, were tested in the study. The levels of APPs depends on the severity of COVID-19 disease, on the presence of cytokine storm and used oxygen therapy. The greatest APPs changes occurred in the most advanced form of the disease, with the presence of a cytokine storm and the most intense oxygen therapy. The results obtained from MEWS scale were not consistent with National Institutes of Health scores. Studies in the second samples showed the quenching of the acute phase reactions and the effectiveness of oxygen therapy. Only two of the examined APPs i.e. procalcitonin and transferrin, differed between surviving and non-surviving patients, and these two predispose to the role of prognostic factors in Covid-19. In conclusion, the concentration of not all acute-phase proteins depends on the severity of COVID-19 disease, presence of cytokine storm, the used of oxygen therapy and only some of them (procalcitonin and transferrin) are related to the survival outcomes. Of the newly tested acute-phase proteins, only transferrin shows significance as a marker of disease severity and mortality in COVID-19 disease.

Identifying potentially undiagnosed individuals with hyper-IgE syndrome using a scoring system

BackgroundHyper-IgE syndrome (HIES) constitutes a group of rare primary immunodeficiency disorders. The diagnosis relies on the National Institutes of Health (NIH) scoring system, incorporating clinical and laboratory data. Scores greater than or equal to 15 raise a strong suspicion of the disease. In an isolated Israeli population, Zinc Finger 341 deficiency, a subtype of HIES, has a carrier incidence of 1:20, but the prevalence of the clinical syndrome within this community remains unknown. ObjectiveTo estimate the prevalence of potentially undiagnosed HIES cases within this population by using the NIH scoring criteria. MethodsThis retrospective cohort study obtained requisite clinical and laboratory data for NIH score computation from the electronic medical records of Clalit Health Services for the isolated village under scrutiny in comparison to a neighboring village. Subsequently, clinical scores were assigned to each subject, enabling comparative analysis of suspected diagnosis rates between the 2 populations. ResultsAmong the 29,390 studied subjects, 12 had a documented diagnosis of HIES. All were in the study village, and none were from the control village (0.08% vs 0%, P < .01). Within the study village, 235 individuals (1.62%) had an NIH score greater than or equal to 15 and were suspected with having HIES almost doubled compared with the control group at 130 individuals (0.87%) (P < .001). ConclusionThis is the first time the NIH clinical score system has been used for population screening. The significant disparity in the prevalence of suspected, undiagnosed cases between the study village and the control village strongly suggests the potential utility of this tool for preliminary screening.

Association of Serum Krebs von den Lungen-6 (KL-6) Levels and Disease Severity in Patients Hospitalized with COVID-19.

We aimed to evaluate Krebs von den Lungen-6 (KL-6) as a possible biomarker in determining disease severity in patients with moderate and severe COVID-19. This cross-sectional study included moderate or severe COVID-19 patients; critically ill patients who were followed up in the intensive care unit were not included. KL-6 level and routine laboratory test measurements were performed on the first day of admission. The patients were also categorized according to their hyperinflammatory state. The study included 92 patients, 56 (61%) women. The National Institutes of Health (NIH) score was 2 in 52.2% of the patients and 3 in 47.8%. KL-6 levels did not significantly differ in disease severity (NIH score 2 vs. 3; p=0.15). Median KL-6 values were 52.7 (29.1) in patients with <2 COVID-19 hyperinflammatory syndrome score (cHIS) and 61.7 (32.2) in patients with cHIS ≥ 2 (p= 0.077). KL-6 values tended to be higher among the patients with lower lymphocyte counts, but the difference was not statistically significant (<1000 mm³/L p=0.006 and higher cHIS scores ≥2 p=0.07). KL-6 values were also higher in the patients with diabetes mellitus compared to the remaining patients (p =0.036). There was no significant association between the serum KL-6 measured at admission and the severity of COVID-19.

Examining Predictors of Favorable 90-day Outcome in Stroke Patients with Active Cancer Undergoing Endovascular Therapy (P8-5.022)

<h3>Objective:</h3> To determine covariates associated with long-term outcomes for patients with acute ischemic stroke (AIS) who had active cancer and underwent endovascular therapy. <h3>Background:</h3> Intraarterial therapy (IAT) is the standard of care for all AIS patients, even those with active cancer. Many studies show poor outcomes for patients with active malignancy compared to those without who undergo IAT. Few studies, however, have identified other characteristics that may contribute to 90-day modified Rankin scale (mRS) scores for patients with active cancer undergoing IAT. <h3>Design/Methods:</h3> This retrospective study included patients (age ≥ 18) admitted from 07/18–10/20 with AIS, treated by IAT, and had active cancer. The primary outcome was 90-day mRS; secondary outcomes were complications. The receiver operating characteristic area under the curve (AUC) was used to determine optimal admission national institutes of health scores (NIHSS) to obtain a 90-day mRS of 0–2. Alpha=0.05. <h3>Results:</h3> There were 53 patients; 42% had a 90-day mRS of 0–2 and 58% had 3–6. The median (interquartile range) age was 71 years (64–81), 58% were male, and the median admission NIHSS was 12 (6–17). Compared to patients who had a 90-day mRS of 3–6, patients with a 0–2 had a significantly lower median admission NIHSS (6.5 (3–15) vs. 15 (9–22), p=0.006), more had IV-tPA (41% vs. 13%, p=0.02), and significantly less experienced a thromboembolic event during their hospital course (17% vs. 57%, p=0.004). No patients with an mRS of 0–2 had a symptomatic intracranial hemorrhage, respectively (0% vs. 10%, p=0.13). The NIHSS cut-off for predicting a favorable 90-day mRS was 8.2 and indicated a moderate fit. <h3>Conclusions:</h3> This data suggests that both admission NIHSS and thromboembolic events were strong predictors of 90-day outcomes. Using an NIHSS cut-off of 8.2 for patients with active cancer in determining who should receive IAT may increase the probability of a favorable 90-day outcome. <b>Disclosure:</b> Dr. Pirahanchi has nothing to disclose. Constance McGraw has nothing to disclose. Dr. Bartt has nothing to disclose. David Bar-Or has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Ampio Pharmaceuticals. David Bar-Or has stock in Ampio Pharmaceuticals.

Abstract TP9: Examining Predictors Of Functional 90-day Outcome Among Stroke Patients With Active Cancer Receiving Endovascular Therapy

Introduction: Intraarterial therapy (IAT) is the standard of care for all AIS patients, even those with active cancer. However, there is little information on the characteristics that may contribute to a functional 90-day modified Rankin scale (mRS 0-2) score for AIS patients with active cancer undergoing IAT. Methods: This retrospective study included patients (age ≥ 18) admitted from 07/18-10/20 with AIS and were treated by IAT. AIS patients with large artery occlusion who had active cancer were identified. Baseline characteristics and outcomes were evaluated to determine predictors of a functional outcome at 90 days. Outcomes were also compared to those without active cancer during the same period. The receiver operating characteristic area under the curve (AUC) was used to determine optimal admission national institutes of health scores (NIHSS) to obtain a 90-day mRS of 0-2. Results: Of the 453 patients who received IAT, 12% had active cancer. Overall, patients were a majority male (50%), a median of 70 (60-81) years of age and had an admission NIHSS of 14 (7-20). Compared to patients without cancer, patients with cancer experienced significantly more thromboembolic events during their hospital stay (40% vs. 11%, p&lt;0.001), but functional outcomes at 90-days were not different between groups. In active cancer patients, an admission NIHSS cut-off of 8.2 (AUC:0.72 (confidence interval (CI): 0.58-0.86), p=0.001) indicating strong fit, and experiencing less thromboembolic events, were significantly associated with functional independency at 90 days. However, the admission NIHSS cut-off for patients without cancer for predicting a functional outcome was 10.5 (AUC: 0.66 (CI: 0.60-.71), p&lt;0.001), indicating moderate fit. Receiving intravenous tissue type plasminogen activator (IV-tPA) and successful reperfusion were other significant predictors of a functional outcome for non-cancer patients. Conclusions: In our population, using an NIHSS cut-off of 8.2 for patients with active cancer in determining who should receive IAT may increase the probability of a favorable 90-day outcome. An admission NIHSS of 10.5 was moderately associated with functional outcome at 90 days, in addition to successful reperfusion and receiving IV-tPA in non-cancer patients.

Phase I Study of SHR0302, a Selective JAK1 Inhibitor, Combined with Prednisone for First-Line Treatment of Chronic Graft-Versus-Host Disease after Allo-HSCT

Background: Chronic graft-versus-host-disease (cGVHD) is a life-threatening complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT) that affects various organs leading to a reduced quality of life, with an incidence of 40% to 70%. The standard first-line therapy for cGVHD is prednisone ± a calcineurin inhibitor, but most patients respond inadequately and prolonged corticosteroid use could cause a variety of side effects. SHR0302 is a Janus kinase (JAK) 1 selective inhibitor which has demonstrated efficacy in preclinical models of GVHD. Herein, we reported the safety and efficacy of SHR0302 plus prednisone as first-line treatment for newly diagnosed cGVHD after allo-SCT. Methods: This was a phase I open-label study. Patients who were aged 18-70, and confirmedly diagnosed with first-episode moderate/severe cGVHD requiring systemic immunosuppressive therapy after allo-HSCT were included in the study. cGVHD was defined according to national institutes of health (NIH) criteria. Patients received SHR0302 plus prednisone daily. For each patient, prednisone was administered at an initial dose of 1 mg/kg/d for two weeks, then tapered according to the response of the patient. A 3 + 3 design was implemented for dose finding of SHR0302 at doses of 1mg/d, 2mg/d, 4mg/d, 6mg/d, and 8mg/d. The primary endpoints were safety and tolerability of the combination therapy of SHR0302 and prednisone assessed by NCI-CTCAE v5.0. And the secondary endpoints included the overall response rate (ORR) at week 4 of treatment and the recommended Phase II dose (RP2D). Dose-limiting toxicities (DLTs) were defined as grade 4 hematologic toxicity or grade 3 non-hematologic toxicity associated with SHR0302 which occurred in the first 28 days of study treatment. Results: As of July 30, 2022, 18 patients were enrolled in the study with 3 patients each in the dose level 1-4, and 6 patients in the dose level 5, with a median follow-up of 27 (4-91) weeks. The median age of the patients was 47 (31-64) years, of which 5 patients (27.8%) had moderate cGVHD, and 13 patients (72.2%) had severe cGVHD. The median cGVHD NIH score was 4 (3-7). The primarily affected organs were skin (61.1%) followed by liver (55.6%), and mouth (50.0%). As of data cutoff, only one patient (5.6%) discontinued therapy due to inadequate response. No DLTs were seen in the first 4 dose levels. Only one DLT, grade ≥3 hypercholesterolemia, was observed among 6 patients who received 8mg/d of SHR0302. The patient who experienced DLT had preexisting hypercholesterolemia. A maximum tolerated dose was not reached. 17 patients (94.4%) experienced adverse events (AEs) related to SHR0302, and 4 patients (22.2%) experienced grade ≥3 AEs related to SHR0302. Hypercholesterolemia (61.1%), hypertriglyceridemia (44.4%), and platelet count decreased (33.3%) were the most common SHR0302 treatment-related AEs. No SHR0302 treatment-related serious adverse events were reported. The ORR at week 4 after the first SHR0302 administration was 94.4%, including 4 patients (22.2%) achieving complete response, and 13 patients (72.2%) achieving partial response. Among 18 evaluable patients, only 1 patient treated at dose level 1 showed no response at week 4. Conclusions: In summary, SHR0302 plus prednisone demonstrated encouraging efficacy with manageable toxicity in patients with steroid-naive cGVHD. Based on the results from the dose-escalation, 6-mg and 8-mg dose were selected for the dose-expansion.

Gastrointestinal stromal tumours: Preoperative imaging features to predict recurrence after curative resection

PurposeTo identify whether preoperative factors could predict the recurrence after curative resection of gastrointestinal stromal tumours (GISTs) and evaluate the performance of a prediction model using preoperative factors for GIST recurrence compared to a model using preoperative/postoperative factors. MethodThis retrospective study included patients who underwent curative resection and preoperative CT for GIST. CT imaging features as preoperative factors were analysed by two abdominal radiologists. Modified National Institutes of Health scores were accessed as a postoperative factor. Multiple logistic regression analysis was performed to assess the preoperative and postoperative factors in predicting GIST recurrence. Through the logistic regression, two prediction models using preoperative factors only and both preoperative/postoperative factors were constructed, respectively. The internal validation of the prediction models was performed using bootstrapping sampling. ResultsData in 113 patients were evaluated. Among them, 14 patients had recurrence. The multiple logistic regression analysis demonstrated that non-gastric location (odds ratio [OR] = 5.12, p = 0.029), ill-defined margin (OR = 4.93, p = 0.023), and prominent vessels around tumour (OR = 6.78, p = 0.007) were significant factors. The prediction models using these preoperative factors and adding a postoperative factor showed an area under the receiver operating characteristic curve of 0.863 and 0.897, respectively, which were not statistically different. The bootstrapping sampling showed the two models were valid. ConclusionThe prediction model derived from non-gastric location, ill-defined margin, and prominent vessels around tumour can be used preoperatively to estimate the risk of recurrence after resection of GIST.

Tocilizumab for faster and safer remission of Takayasu's arteritis.

Takayasu's arteritis (TAK) is a large-vessel vasculitis. Glucocorticoids (GCs) combined with cyclophosphamide (CTX) is a common treatment option, but their efficacy is limited. The aim of this retrospective study was to study the efficacy and safety of tocilizumab (TCZ) in the treatment of TAK. A total of 63 patients with TAK were divided into two groups according to different treatment strategies: 31 patients in the TCZ group and 32 patients in the CTX group. After 6 months of treatment, disease activity and side effects were evaluated and were compared between the two groups. After 3 months of treatment, the National Institutes of Health (NIH) score (p = 0.005), Indian Takayasu Clinical Activity Score (ITAS) 2010 (p = 0.043), and the Indian Takayasu Activity Score with the Acute-Phase Response (ITAS.A; p = 0.036) were lower in patients treated with TCZ compared with those treated with CTX. In the TCZ group, the proportion of patients with NIH scores ⩽1 reached 50% after 1 month, 90% after 3 months, and 96% after 6 months of treatment, whereas in the CTX group, these proportion were 36%, 30%, and 78%, respectively. Thus, TCZ treatment of TAK is accompanied with a faster remission rate than CTX treatment. Moreover, the total cumulative incidence of adverse events was lower in the TCZ group than in the CTX group (p = 0.035). TCZ was faster and safer in alleviating TAK.

B regulatory cells and monocyte subpopulations in patients with chronic graft-vs-host disease

AimTo assess the correlations of B regulatory cells (Bregs) and monocyte subsets in peripheral blood with the National Institutes of Health (NIH)-consensus-defined clinical manifestations of chronic graft-vs-host disease (cGvHD), in an attempt to establish their role as cellular biomarkers.MethodsThis multidisciplinary prospective study enrolled adult cGVHD patients treated in the University Hospital Center Zagreb and University of Zagreb School of Medicine. Immunophenotypic subpopulations of CD24highCD38high Bregs (CD27-, CD27+, and total) and monocyte (classical, intermediate, and non-classical) counts were correlated with demographic, transplant, and cGVHD-related data. Bivariate correlation analysis was performed to evaluate the correlations between Bregs and monocytes subsets and cGVHD organ involvement, as well as cGVHD severity and immunosuppression intensity.ResultsTwenty-two adult patients (54.5% female) with cGVHD were enrolled. The median (range) age was 44.5 years (24-65). All patients were transplanted for hematologic malignancies and 40.9% had severe NIH cGVHD global score. The median time from cGVHD diagnosis to the analysis was 16.6 months (0-176). The organ most frequently affected with cGVHD were the eyes (68.2%), skin (45.5%), lungs (45.5%), and liver (40.9%). Lower total and CD27-Bregs counts were correlated with worse cGVHD severity, higher immunosuppression intensity, and lung cGVHD, in terms of cell count, but also with skin cGVHD, in terms of percentages. Patients with liver and joint/fascia cGVHD had a lower percentage of non-classical monocytes and patients with more severe global NIH score had a higher classical monocytes count.ConclusionDifferent organs affected by cGVHD are differently associated with different subpopulations of Bregs and monocytes.

Abstract P212: Admission National Institutes of Health Stroke Scale Associated With Higher Rates of Post Stroke Depression Independent of Imaging Characteristics

Introduction: The etiology of post-stroke depression (PSD) is still not fully understood. It is well known that leukoaraiosis is associated with cognitive decline and depression, but currently unclear if lacunar strokes, thought to be caused by small vessel disease, have higher rates of PSD. A better understanding of PSD etiology can lead to improved treatment of PSD and subsequently better stroke recovery. Methodology: Out of 159 patients that were successfully contacted via phone within 3 months of discharge, there were 22 identified as having an acute stroke or TIA and scored ≥ 2 on the Patient Health Questionnaire (PHQ) assessment. MRI brain was reviewed for stroke size, location, periventricular and deep white matter fazekas score, number of microbleeds, and chronic lacunar strokes. Admission and discharge National Institutes of Health (NIH) stroke scale and stroke etiology were also recorded. Results: There was a significantly positive correlation between NIH score at admission and PHQ2 score (r = 0.184, p =0.044), demonstrating that patients with NIH ≥ 6 are 4.6 times more likely to develop PSD than a patient with NIH &lt; 6 (Odds ratio (OR) = 4.6, CI [1.284-16.591]). There was no significant difference between PSD incidence in stroke size or location. Although not statistically significant, there was a suggestion of more PSD seen in cortical, cardioembolic, and cryptogenic strokes as well as fazekas score ≥ 2 and old lacunar infarcts. The table summarizes characteristics of the 7 TIA and 15 stroke patients suffering from PSD with PHQ ≥ 2: Conclusion: Initial NIH significantly predicted PSD irrespective of leukoaraiosis. This study was limited by the small sample size which could be due to under-diagnosis of PSD. In conclusion, admission NIH is associated with higher rates of PSD, but the underlying etiology is likely multifactorial given the suggestion of higher rates of PSD with higher fazekas score, and cortical, cardioembolic, and cryptogenic strokes.

Prevalence of Persistent Corneal Epithelial Defects in Chronic Ocular Graft-Versus-Host Disease

To establish the prevalence, clinical characteristics, and risk factors for persistent corneal epithelial defects (PED) in patients with chronic ocular graft-versus-host disease (oGVHD) and to determine visual outcomes after healing. Retrospective cohort study. A chart review was conducted of patients in whom chronic oGVHD was diagnosed between January 2011 and December 2018 and their demographic and clinical characteristics were collected. Data were analyzed to determine prevalence of PED, and multivariate logistic regression was performed to determine the risk factors associated with it. A total of 405 patients at a mean age of 60 ± 13 years in whom chronic oGVHD was diagnosed; 58% were men. The prevalence of PED was 8.1%. The median time for PED development after hematopoietic stem cell transplantation was approximately 24months. Median time to PED resolution was 4.5weeks after starting therapy. The mean best-corrected visual acuity declined by 2 lines post-PED resolution. The prevalence rates of corneal ulcer and perforation were 6.2% and 4.0%, respectively, over 8 years. Logistic regression analysis, used to determine factors associated with PED, showed diabetes (P= .006), limbal stem cell deficiency (LSCD) (P= .02), filamentary keratitis (P= .02), subconjunctival fibrosis (P= .02), and a higher National Institutes of Health (NIH) oGVHD score (P= .01) were significant risk factors for PED development. The study found the prevalence rate of PED, corneal ulceration, and corneal perforation in chronic oGVHD to be 8.1%, 6.2%, and 4%, respectively. Analysis showed that oGVHD patients with diabetes, LSCD, filamentary keratitis, subconjunctival fibrosis, and a high NIH score were at higher risk of developing severe corneal disease.

Novel Approach to Imaging Active Takayasu Arteritis Using Somatostatin Receptor Positron Emission Tomography/Magnetic Resonance Imaging

Novel Approach to Imaging Active Takayasu Arteritis Using Somatostatin Receptor Positron Emission Tomography/Magnetic Resonance Imaging

Targeting JAK/STAT pathway in Takayasu’s arteritis

ObjectiveTakayasu’s arteritis (TAK) is a large vessel vasculitis with important infiltration of proinflammatory T cells in the aorta and its main branches, but its aetiology is still unknown. Our work...

In acute ischemic stroke patients with smoking incidence, are more women than men more likely to be included or excluded from thrombolysis therapy?

Background:Clinical factors associated with exclusion from recombinant tissue plasminogen activator in both men and women are not completely understood. The aim of this study is to determine whether there is a gender difference in clinical risk factors that excluded ischemic stroke patients with a history of smoking from recombinant tissue plasminogen activator.Methods:Retrospective data from a stroke registry were analyzed, and multivariable linear regression models were used to determine gender differences. Logistic regression models determined exclusion clinical risk factors for thrombolysis in male and female acute ischemic stroke patients with a history of smoking, while sequentially adjusting for sociodemographic, clinical, and stroke-related variables. The Kaplan–Meier survival analysis was used to determine the exclusion probabilities of men and women with a history of smoking within the stroke population.Results:Of the 1,446 acute ischemic stroke patients eligible for recombinant tissue plasminogen activator, 379 patients with a history of smoking were examined, of which 181 received recombinant tissue plasminogen activator while 198 were excluded from receiving recombinant tissue plasminogen activator. Of the 198 patients, 75 females and 123 males were excluded from receiving recombinant tissue plasminogen activator. After multivariable adjustment for age, National Institutes of Health scores, and stroke-related factors, females who present with weakness/paresis on initial examination (OR = 0.117, 95% CI, 0.025–0.548) and men who present with a history of previous transient ischemic attack (OR = 0.169, 95% CI, 0.044–0.655), antiplatelet medication use (OR = 0.456, 95% CI, 0.230–0.906), and weakness/paresis on initial examination (OR = 0.171, 95% CI, 0.056–0.521) were less likely to be excluded from recombinant tissue plasminogen activator (thrombolysis therapy).Conclusions:In an ischemic stroke population with a history of smoking, female smokers are more likely to be excluded from thrombolysis therapy in comparison to men, even after adjustment for confounding variables.

Takayasu arteritis: clinical importance of extra-vessel uptake on FDG PET/CT

Background[F-18]fluorodeoxyglucose positron emission tomography/computed tomography is routinely used for assessing Takayasu arteritis patients. However, extra-vessel [F-18]fluorodeoxyglucose uptake has not been evaluated in detail in these patients. We aimed to describe the extent and distribution of extra-vascular [F-18]fluorodeoxyglucose uptake on positron emission tomography/computed tomography in Takayasu arteritis patients. Seventy-three [F-18]fluorodeoxyglucose positron emission tomography/computed tomography scans from 64 consecutive Takayasu arteritis patients (59 women, mean age, 35.4 years; range, 13 to 71 years) and 40 scans from age-matched controls (36 women, mean age, 37.8 years; range, 13 to 70 years) were examined. We graded [F-18]fluorodeoxyglucose uptake in large vessels using a 4-point scale and evaluated extra-vessel findings. Factors correlated with disease activity were examined. We evaluated the relationship between disease activity according to the National Institutes of Health score with extra-vessel findings, as well as other inflammatory markers (e.g., white blood cell count and C-reactive protein level).ResultsExtra-vessel involvement was present in 50 of 73 (68.4%) scans, specifically at the following sites: lymph nodes, 1.4%; thyroid glands, 17.8%; thymus, 11.0%; spleen, 1.4%; vertebrae, 45.2%; and pelvic bones, 9.6%. Takayasu arteritis patients had higher [F-18]fluorodeoxyglucose uptake in the spine (P = 0.03) and thyroid glands (P = 0.003) than did controls; uptake in other regions was comparable between groups. Compared with inactive patients, those with active Takayasu arteritis had a higher number of [F-18]fluorodeoxyglucose uptake sites in the vasculature (P = 0.001). Finally, patients with a National Institutes of Health score of ≥ 1 had significantly higher extra-vascular involvement (P = 0.008).ConclusionsExtra-vessel [F-18]fluorodeoxyglucose uptake may be present in the context of Takayasu arteritis-related inflammatory processes. Information on extra-vascular [F-18]fluorodeoxyglucose uptake may be useful for detecting and evaluating inflammatory processes when interpreting positron emission tomography/computed tomography scans obtained from Takayasu arteritis patients.

The first cohort of Iranian patients with hyper immunoglobulin E syndrome: A long-term follow-up and genetic analysis.

Hyper-IgE syndromes (HIES) are distinct diseases characterized by recurrent cutaneous and lung infections, eczema, and elevated serum IgE level. In this study, clinical manifestations, immunologic findings, and genetic studies of all patients with HIES in the Iranian national registry database were evaluated. A total of 129 HIES patients with a median age of 14.0 (9.0-24.0) years were followed up for a total of 307.8 patient-years. Genetic studies showed heterozygous STAT3 mutations in 19 patients and homozygous DOCK8 mutation in 16 patients. The mean of National Institutes of Health score in STAT3-deficient patients was higher than in patients with DOCK8 mutation (P=0.001). It was shown that the presence of pneumatocele and hematologic complication were significantly frequent in STAT3-deficient cases compared to patients with DOCK8 deficiency (P=0.001 and P=0.002, respectively). Moreover, the median IgE serum levels were higher in patients with STAT3 gene mutation than in patients with DOCK8 gene mutation (P=0.02). The eosinophils' count was enhanced in patients with DOCK8 deficiency than in patients with STAT3 gene defects (P=0.02). Specific molecular study of STAT3 and DOCK8 mutations in patients with HIES clinical phenotype could help the physician to definitively characterize the disease. Since HIES showed the highest rate of unsolved combined immunodeficiency, investigation of other genetic and environmental factors could also help in understanding the mechanism of remaining patients as well as providing strategy into therapeutic modalities.

Abstract WP20: Tandem Occlusions; Does Pretreatment With Thrombolytics Matter

Introduction: Tandem occlusions are a significant treatment challenge for which no standard intervention protocol has been determined. IV tPA alone generally has a poor outcome for tandem occlusions while endovascular therapy (EVT) is thought to produce the best possible outcome. The purpose of this study was to evaluate the safety and efficacy of IV tPA used prior to EVT for tandem occlusions. Hypothesis: IV tPA plus EVT may improve clinical outcome over EVT alone without more adverse events. Methods: There were 56 patients with tandem occlusions over a 3 year period out of 309 (18%) patients with an LVO. The primary clinical outcome was National Institutes of Health (NIH) stroke scale and modified Rankin Scale (mRS) at discharge. Secondary outcomes were discharge disposition (Good: home or acute rehabilitation, Poor: all others), recanalization (Good: TICI ≥ 2B), and mortality. T-test was used to compare outcomes between the group that received EVT plus tPA versus EVT alone. Reasons patients did not receive tPA included recent surgery, therapeutic anticoagulation, or outside treatment window. Results: 23/ 56 (41%) tandem occlusion patients received EVT; 15 (65%) also received tPA. NIH scores on presentation were the same between the group receiving EVT plus tPA and EVT alone (17.13, p = 0.9984), and there was no significant difference between the two groups in respect to discharge NIH (no tPA: 5.0, yes tPA: 6.0; p = 0.7487), discharge mRS (no tPA: 4.25, yes tPA: 3.67; p = 0.4349), TICI scores (63% as compared to 80% with tPA), mortality ( no tPA: 37.5%, yes tPA: 13%; p =0.419), or good discharge disposition (no tPA: 50%, yes tPA 60% p = 0.432). Conclusion: Although not statistically significant, the group that received EVT plus tPA had lower mRS scores, lower mortality rates, increased reperfusion and a higher percentage of discharge to home or acute rehab as compared to the group receiving EVT alone. This suggests that tPA plus EVT may be more effective in treating tandem occlusions without accruing additional adverse effects. Limitations of this study include the small sample size and retrospective nature, demonstrating the need for larger and prospective studies in the future.

Relationships between the levels of serum 25-hydroxyvitamin D and interleukin-6 in patients with Takayasu's arteritis

Objective: The main purpose of this paper is to evaluate the level of serum 25-hydroxyvitamin D (25-OH-D) and clinical significance in patients with Takayasu's Arteritis (TA). Methods: A total of 62 untreated TA patients (TA group) and 60 healthy people (control group) were enrolled. The level of serum 25-OH-D were measured and compared between groups. The correlations of 25-OH-D level with related indicators were analyzed. Results: Lower concentrations of serum 25-OH-D were detected in TA patients compared with healthy subjects[(11.8±4.7) μg/L versus (23.2±8.3) μg/L](P<0.001). In active stage group, the 25-(OH)-D level of TA were significantly lower than that in remission stage group[(10.5±4.3) μg/L vs (13.3±4.9) μg/L](P=0.021). In all patients with TA, serum 25-OH-D correlated negatively with interleukin (IL)-6 (r=-0.296, P=0.023). There was no correlation between serum 25-OH-D level and C-reactive protein , erythrocyte sedimentation rate , National Institutes of Health Score, Indian Takayasu Clinical Activity Score 2010, and anti-tumor necrosis factor (TNF)-alpha antibody. Conclusion: A high prevalence of vitamin D deficiency was observed in patients with TA. The serum 25-OH-D level were negatively correlated with IL-6.

Joinville stroke biobank: study protocol and first year's results.

Blood samples were collected from patients included in the Joinville Stroke Registry and four Brazilian cities. Demographic socio-economic data, cardiovascular risk factors, Causative Classification System for Ischemic Stroke, Trial of Org 10172 in Acute Stroke Treatment and National Institutes of Health scores, functional stroke status (modified Rankin) and brain images were recorded. Additionally, controls from both geographic regions were recruited. High-molecular-weight genomic DNA was obtained from all participants. A total of 2,688 patients and 3,282 controls were included. Among the patients, 76% had ischemic stroke, 12% transient ischemic attacks, 9% hemorrhagic stroke and 3% subarachnoid hemorrhage. Patients with undetermined ischemic stroke were most common according the Trial of Org 10172 in Acute Stroke Treatment (40%) and Causative Classification System for Ischemic Stroke (47%) criteria. A quarter of the patients were under 55 years of age at the first-ever episode. We established the Joinville Stroke Biobank and discuss its potential for contributing to the understanding of the risk factors leading to stroke.

Predictors for Permanent Discontinuation of Systemic Immunosuppression in Severely Affected Chronic Graft-Versus-Host Disease Patients

Predicting the duration of systemic therapy in patients with chronic graft-versus-host disease (cGVHD) is of critical clinical importance when counseling patients and for treatment planning. cGVHD characteristics associated with this outcome have not been studied in severely affected patients. The National Institutes of Health (NIH) cGVHD scoring provides a standardized set of organ severity measures that could represent clinically useful and reproducible predictive characteristics. We analyzed 227 previously treated patients most with moderate (n = 54) or severe (n = 170) cGVHD defined by NIH criteria who were prospectively enrolled in a natural history protocol (NCT00092235). Patients received a median of 4 prior systemic therapy regimens and were seen at the NIH for a single time-point visit and were then monitored for survival and ability to discontinue cGVHD systemic therapy. With a median follow-up of 71.1 months, the cumulative incidence of systemic therapy discontinuation was 9.5% (95% confidence interval, 6.0% to 13.9%) at 2 years and 27.7% (95% confidence interval, 20.9% to 34.8%) by 5 years after the initial visit. Factors associated with a higher incidence of immunosuppression discontinuation included lower NIH global severity (P = .019) and lung (P = .030) scores and less extensive deep sclerosis (<37% body surface area, P = .024). Lower patient- and clinician-reported 0 to 10 severity NIH scores and noncyclosporine prophylaxis regimens were also associated with higher incidence of immunosuppression discontinuation (P < .05). In conclusion, we found low success rates for immune suppression discontinuation in previously treated patients who were severely affected with cGVHD. NIH scoring and clinical measures provide new standardized disease-specific tools to predict discontinuation of systemic therapy.