scholarly journals Phenotypic Characterization of Juvenile Idiopathic Arthritis in African American Children

2016 ◽  
Vol 43 (4) ◽  
pp. 799-803 ◽  
Author(s):  
Lauren Fitzpatrick ◽  
K. Alaine Broadaway ◽  
Lori Ponder ◽  
Sheila T. Angeles-Han ◽  
Kirsten Jenkins ◽  
...  

Objective.Juvenile idiopathic arthritis (JIA) affects children of all races. Prior studies suggest that phenotypic features of JIA in African American (AA) children differ from those of non-Hispanic white (NHW) children. We evaluated the phenotypic differences at presentation between AA and NHW children enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry, and replicated the findings in a JIA cohort from a large center in the southeastern United States.Methods.Children with JIA enrolled in the multicenter CARRA Registry and from Emory University formed the study and replication cohorts. Phenotypic data on non-Hispanic AA children were compared with NHW children with JIA using the chi-square test, Fisher’s exact test, and the Wilcoxon signed-rank test.Results.In all, 4177 NHW and 292 AA JIA cases from the CARRA Registry and 212 NHW and 71 AA cases from Emory were analyzed. AA subjects more often had rheumatoid factor (RF)-positive polyarthritis in both the CARRA (13.4% vs 4.7%, p = 5.3 × 10−7) and the Emory (26.8% vs 6.1%, p = 1.1 × 10−5) cohorts. AA children had positive tests for RF and cyclic citrullinated peptide antibodies (CCP) more frequently, but oligoarticular or early onset antinuclear antibody (ANA)-positive JIA less frequently in both cohorts. AA children were older at onset in both cohorts and this difference persisted after excluding RF-positive polyarthritis in the CARRA Registry (median age 8.5 vs 5.0 yrs, p = 1.4 × 10−8).Conclusion.Compared with NHW children, AA children with JIA are more likely to have RF/CCP-positive polyarthritis, are older at disease onset, and less likely to have oligoarticular or ANA-positive, early-onset JIA, suggesting that the JIA phenotype is different in AA children.

2020 ◽  
pp. jrheum.200230
Author(s):  
Justine Maller ◽  
Emily Fox ◽  
KT Park ◽  
Sarah Sertial Paul ◽  
Kevin Baszis ◽  
...  

Objective The incidence of inflammatory bowel disease (IBD) in juvenile idiopathic arthritis (JIA) is higher than in the general pediatric population. However, reports of IBD in the systemic JIA (sJIA) subtype are limited. We sought to characterize sJIA patients diagnosed with IBD and to identify potential contributing risk factors. Methods Using an internationally distributed survey, we identified 16 sJIA patients who were subsequently diagnosed with IBD (sJIA-IBD cohort). 522 sJIA patients without IBD were identified from the CARRA Legacy Registry and served as the sJIA-only cohort for comparison. Differences in demographic, clinical characteristics and therapy were assessed using chi-square test, Fisher’s exact test, t-test, and univariate and multivariate logistic regression as appropriate. Results 75% of sJIA-IBD patients had a persistent sJIA course; 25% had a history of MAS. sJIAIBD subjects were older at sJIA diagnosis, more often non-White, had a higher rate of IBD family history, and were more frequently treated with etanercept or canakinumab compared to sJIA-only subjects. 69% of sJIA-IBD patients successfully discontinued sJIA medications following IBD diagnosis, and sJIA symptoms resolved in 9/12 patients treated with TNF-α inhibitors. Conclusion IBD in the setting of sJIA is a rare occurrence. The favorable response of sJIA symptoms to therapeutic TNF-α inhibition suggests that the sJIA-IBD cohort may represent a mechanistically distinct sJIA subgroup. Our study highlights the importance of maintaining a high level of suspicion for IBD when gastrointestinal involvement occurs in sJIA patients and the likely broad benefit of TNF-α inhibition in those cases.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 1046-1046
Author(s):  
Darko Skrobo ◽  
Naomi Walsh ◽  
Jose Berenguer ◽  
Janice Maria Walshe ◽  
Michaela Jane Higgins ◽  
...  

1046 Background: The introduction of anti-H2 targeted therapies has resulted in substantially improved outcomes for patients (pts) with H+MBC, yet despite survival prolongation, most patients so-treated will still ultimately die from MBC. Some patients do however, achieved prolonged remissions. In this report we outline the long-term outcomes of patients with H+MBC who were treated in our institution, with at least five-year follow-up from the diagnosis of MBC. Methods: As part of our larger single-institution “Thousand Patient HER-2 Database”, we conducted a retrospective review of all patients in whom a diagnosis of H+MBC was made prior to December 2015 (range 2000-2015 years). The DR5 category included only those who had never experienced relapse or progression following initial anti-H2 therapy for MBC, and who were alive at 5 years. Patients were designated as (1) DR5, defined as never relapse with an overall survival (OS) > 5 years; (2) nonDR, which included those who had no or shorter remission, but also included nine pts who did achieve a 5 year CR, but who subsequently relapsed. OS was calculated from the date of diagnosis of MBC. The frequency distribution was assessed by Fisher’s Exact Test or Chi-Square Test, as appropriate. OS and PFS were calculated according to Kaplan Meier method, and evaluated by Log-rank test. Univariate and multivariate Cox proportional hazards regression analysis was used to evaluate the effect of clinicopathological features on OS and PFS. Results: A total of 245 patients diagnosed with advanced H+MBC were identified. The median survival was 38 months, (range 0.3 – 248 months). Among these, 85 patients (35%) experienced an OS > 5 years, with 34 designated as DR5. The median OS for DR5 was 117 months, whereas nonDR (n = 211) had median OS of 33 months. The median age was similar between groups (DR5 53 yrs vs nonDR 56 yrs). A higher incidence of visceral disease was present in nonDR compared to DR5 (69% vs 44%). Of all patients diagnosed with de novo H+MBC, 23% achieved DR5. Presence of visceral disease, number of metastases and site of metastases were statistically significant negative predictors of achieving DR5 (P < 0.05). Presence of ER positive disease was not associated with OS. Conclusions: A meaningful subset of patients (14%) with advanced H+MBC achieve prolonged remission beyond five years with H2 targeted therapy. Nearly one quarter of those with de novo H+MBC achieve DR5. As de novo H+MBC now constitutes a higher proportion of all H+MBC than it did in the pre-trastuzumab era, an increasing proportion of H+MBC may now be achieving DR5. Prospective identification of variables to predict DR5 could assist in the stratification of patients for whom additional therapy is needed.


2017 ◽  
Vol 19 (1) ◽  
pp. 1-7 ◽  
Author(s):  
Hyun Gi Kim ◽  
Seung-Koo Lee ◽  
Jung-Dong Lee

OBJECTIVE Young patients with moyamoya disease can exhibit infarction after revascularization surgery. This analysis of the characteristics of infarction after encephaloduroarteriosynangiosis (EDAS) in young patients with moyamoya disease was undertaken in an effort to elucidate the infarction mechanism. METHODS The authors retrospectively collected clinical information and reviewed pre- and postoperative MRI studies from cases involving patients younger than 18 years who underwent EDAS for the treatment of moyamoya disease between January 2012 and February 2015. Infarction patterns were categorized into watershed, territorial, or mixed pattern. The Wilcoxon rank sum test, chi-square test, and Fisher exact test were used to compare the clinical and imaging variables between patient groups. The characteristics of patients with and without postoperative infarction were compared using univariate and multivariate analysis. The cumulative proportion of patients without postoperative infarction according to operation stage was calculated using the Kaplan-Meier method and the resulting curves were compared using the log-rank test. RESULTS In 100 patients, 171 EDAS procedures had been performed. There were 38 cases of preoperative infarction in 35 patients and 20 cases of postoperative infarction in 13 patients. Territorial infarction was more frequent in the postoperative infarction group than in the preoperative infarction group (55.0% vs 37.8%, p = 0.037). Infarction was more common on the bilateral or contralateral side of the operation after first-stage EDAS (9 [75.0%] of 12 infarctions) than in the second-stage operation (2 [25.0%] of 8 infarctions), but the difference was not statistically significant (p = 0.068). The frequency of postoperative infarction was not significantly different depending on the stage of the operation (p = 0.694). CONCLUSIONS An acute infarction pattern after EDAS was more frequently territorial, suggesting an underlying occlusive mechanism. Operation stage did not affect the rate of postoperative infarction occurrence.


Neurosurgery ◽  
2017 ◽  
Vol 83 (5) ◽  
pp. 915-921 ◽  
Author(s):  
Virginia W Osborn ◽  
Anna Lee ◽  
Elizabeth Garay ◽  
Joseph Safdieh ◽  
David Schreiber

Abstract BACKGROUND Although the standard of care for glioblastoma remains maximal safe resection followed by chemoradiation, conflicting reports have emerged regarding the importance of the time interval between these 2 treatments. OBJECTIVE To assess whether differences in the duration between surgery and initiation of chemoradiation for glioblastoma had an impact on overall survival (OS) in a large hospital-based database. METHODS The National Cancer Database was queried to identify patients diagnosed with glioblastoma between 2010 and 2012 treated with surgery followed by chemoradiation. Patients who received biopsy only were excluded. The time from surgery to initiation of radiation therapy was divided into 4 equal quartiles of ≤24, 25 to 30, 31 to 37, and &gt;37 d. Patient characteristics were compared between groups using Pearson Chi Square and Fisher's Exact test. OS was analyzed via the Kaplan–Meier method and compared via the log-rank test. Univariable and multivariable Cox regression were performed to assess for impact of covariables on OS. RESULTS A total of 11 652 patients were included in the analysis. Median duration from surgery to radiation was 30 d. On multivariable regression, black race, larger tumor, gross-total resection, methyguanine-methyl transferase (MGMT+), and treatment at an academic facility were associated with a duration &gt;30 d. On multivariable analysis, there were no significant differences when comparing start within 24 d to 25 to 30 d (hazard ratio [HR] 0.96, 95% confidence interval [CI] 0.90-1.01, P = .13) or &gt; 37 d (HR 0.97, 95% CI 0.91-1.03, P = .26), although a small OS improvement was seen if initiated within 31 to 37 d (HR 0.93, 95% CI 0.88-0.99, P = .02). CONCLUSION There was no clear association between duration from surgery to initiation of chemoradiation on OS.


2013 ◽  
Vol 40 (3) ◽  
pp. 329-334 ◽  
Author(s):  
RICARDO A.G. RUSSO ◽  
MARÍA M. KATSICAS

Objective.Systemic juvenile idiopathic arthritis (SJIA) frequently leads to disability and damage. Predictive factors for a poor outcome include persistent systemic features and younger age at onset. We describe and analyze disease features in patients with early-onset (EO) SJIA (disease onset before age 18 mo) and compare them to patients with later-onset (LO) disease.Methods.Clinical features at onset, activity measures (occurrence of macrophage activation syndrome, remission), and outcome measures for disability [Childhood Health Assessment Questionnaire (CHAQ) ≥ 0.5] and damage [radiographic joint destruction, Juvenile Arthritis Damage Index (JADI) score, growth retardation] observed during followup were analyzed retrospectively in patients with SJIA followed for ≥ 3 years since disease onset.Results.In total 132 patients were included. SJIA started at age ≤ 18 months in 19 (14%) patients and at a later age in 113 (86%) children. At onset, serositis (p < 0.01) and hepatomegaly (p < 0.05) were more frequent in EO patients, who also exhibited lower hemoglobin levels (p < 0.03) and higher platelet counts (p < 0.03) than patients with LO. Macrophage activation syndrome occurred in 20 patients (11 EO and 9 LO; p < 0.0001). Remission was achieved by 49 patients (37%; 4 EO and 45 LO). At last visit, destructive hip disease (p < 0.04), growth retardation (p < 0.01), radiographic damage (p < 0.02), and disability (p < 0.04) were more frequent in patients with EO disease, who had higher JADI scores (p < 0.003).Conclusion.Patients with EO exhibited a more aggressive and destructive disease course than patients with LO SJIA.


2000 ◽  
Vol 19 (3) ◽  
pp. 253-263 ◽  
Author(s):  
Thomas Ashby Wills ◽  
Frederick X. Gibbons ◽  
Meg Gerrard ◽  
Gene H. Brody

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Emma Cunningham ◽  
Nicola Gallagher ◽  
Paul Hamilton ◽  
Leeann Bryce ◽  
David Beverland

Abstract Background Hyponatraemia, defined as a serum sodium [Na] concentration below 135 mmol/L, is common following surgery. As inpatient peri-operative stays shorten, there is a need to recognise pre-operative risk factors for post-operative hyponatraemia and complications associated with a peri-operative drop in Na. This audit aimed to investigate the prevalence of, risk factors for, and complications associated with hyponatraemia following elective primary hip and knee arthroplasty. Methods Data were collected within a retrospective audit of inpatient complications and unplanned reattendance or readmission at hospital in consecutive elective primary hip and knee arthroplasty patients in a single high throughput elective primary joint unit. The hospital’s electronic database identified 1000 patients who were admitted electively between February 2012 and June 2013 under the care of a single consultant orthopaedic surgeon for either total hip arthroplasty, total knee arthroplasty, or uni-compartmental knee arthroplasty. Groups were compared using appropriate tests, including chi-square analysis (or Fisher’s exact test), Mann-Whitney U test, Kruskal-Wallis test, and Wilcoxin signed-rank test. Logistic regression analysis was used to determine factors associated with hyponatraemia. Results Of the total 1000 patients, 217 (21.7%) developed post-operative hyponatraemia. Of these, 177 (81.6%) had mild (Na 130–134 mmol/L), 37 (17.1%) had moderate (Na 125–129 mmol/L), and 3 (1.4%) had severe (Na < 125 mmol/L) hyponatraemia. In multivariate analysis, age, pre-operative Na, and fasting glucose on day 1 remained significantly associated with having hyponatraemia post-operatively. There were no significant differences in reattendance at emergency departments and/or readmission within 90 days between those who had post-operative hyponatraemia whilst in hospital (39/217 = 18.0%) and those who did not (103/783 = 13.2%), or between those who were discharged with hyponatraemia (18/108 = 16.7%) and those discharged with normal Na (124/880 = 14.1%). Conclusion Approximately one fifth of elective joint arthroplasty patients had post-operative hyponatraemia. In these patients, older age, lower pre-operative Na and higher fasting glucose predicted post-operative hyponatraemia. We found no evidence that those discharged with hyponatraemia had more reattendance at emergency departments or readmission to hospital. We suggest that otherwise well patients with mild hyponatraemia can safely be discharged and followed up in the community.


2019 ◽  
Vol 25 (4) ◽  
pp. 39
Author(s):  
Ajmal Kaleem ◽  
Rajendran Balamurugan

Introduction: The purpose of this study was to obtain insight into the perioperative condition of the maxillary sinus in the LeFort I osteotomy by evaluating clinically and radiographically. Materials and methods: 25 patients who required conventional LeFort I procedures for orthognathic correction were included in the study. Damage to the maxillary sinus during the procedure and its recovery were prospectively analysed using validated questionnaires for sino-nasal complaints using RSOM-31 (RSOM − rhinosinusitis outcome measure), VAS score (VAS − visual analogue scale) and CT scan to compare and analyse changes in maxillary sinus prior to surgery and postoperatively 2 months after the surgery. The scores obtained from RSOM-31 questionnaire was analysed using Chi-square test, VAS questionnaire was interpreted using Wilcoxon sign rank test and CT scan findings were analysed using Fischer's exact test. Results: Mucosal thickening assessed using CT scan was the only consistent finding that was evident for all the patients who underwent LeFort I osteotomy which showed a statistically significant results of P < 0.05, whereas clinical correlation showed insignificant results of P > 0.05. Conclusion: In our attempt on extensive patient analysis we found that mucosal thickening was the prime alteration that was observed radiographically and no clinical changes were evident.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 5548-5548
Author(s):  
Maria Ornella Nicoletto ◽  
Marco Rocchetto ◽  
Grazia Artioli ◽  
Alessandra Perin ◽  
Cristina Ghiotto ◽  
...  

5548 Background: The clinical outcome of double OC and BC is specifically unknown either BRCA+ and in double tumours NH patients. Methods: The present databases made of 106 patients, 67 cases of NH (negative, no-tested or ongoing test for BRCA1/2 mutations) and 39 of BRCA+, were constituted to identify the clinical and pathological features of BC and OC. The primary endpoint was to evaluate biological characteristics of both cancers and clinical outcome of OC in coexistence with BC. Patients were censored at last follow-up or death (any cause) for determination of overall survival (OS). OS were determined using the Kaplan-Meier method and log-rank test to compared the different levels of a variable. Pearson Chi-Square or Fisher’s exact test were used to compare relationship between variables in to groups and Mann-Whitney U test to compare the medians. Results: 32/39 (82 %) BRCA+ and 44/67 (66 %) NH had BC as their first malignancy. As regards the genetic test on NH patients, 28 were BRCA negative, 22 have not been tested and in 10 patients the test is still in progress. All BRCA2 patients had BC as first malignancy, while 20/22 of BRCA1. Bilateral BC was more frequent in BRCA+ than in NH (33 % vs 9 %), resulted in a fivefold higher risk (p = 0.002). III-IV stage OC at diagnosis was 79% in BRCA+ vs 55 % in NH (p = 0.013); indeed BRCA+ patients have a threefold higher risk (however moderate) to develop an advanced stage OC. Death for progression of ovarian cancer involved both groups, and third neoplasm was involved in death cause in 1/1 of BRCA and 5/6 of NH. Two BRCA1 with OC as first neoplasm are alive. Conclusions: III-IV stage OC is more frequent in BRCA+ than in NH, and the main cause of disease progression and death is due to OC. Eventually the most relevant conclusive assessment is the suggestion of a more conserving management for BC and an intensive follow-up for OC in patients with double tumours, irrespective of their pathological or genetic features. Prospective trials are also indicated.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e18552-e18552
Author(s):  
ZiBin Liang ◽  
Siyang Wang ◽  
Shuai Yang ◽  
Feiyu Long ◽  
Lifeng Jiang ◽  
...  

e18552 Background: Nasopharyngeal carcinoma (NPC) is a typical type of head and neck cancers with its high prevalence areas in Southeast of China. On account of the prolongation of life, the severe complications developed such as radiation encephalopathy (REP). Currently many methods are used to cure REP but the outcomes are limited, such as corticosteroids, hyperbaric oxygen, antiplatelet agents and so on. The aim of this study is 1) to evaluate the protective effect of Calcium leucovorin (LV), a reduced folic acid, on REP in NPC patients. 2) to investigate the progression-free survival (PFS) and overall survival (OS) in NPC treated with or without LV. Methods: We conducted a retrospective, observational cohort study using de-identified, curated data in the Fifth Affiliated Hospital of SYSU included 1075 patients diagnosed as NPC of Stage II, III, or IVa who had been treated from December 2002, through February 2014. The Leucovorin-Group (LV-Group) of 163 NPC patients were matching the preceding criteria and agreed to receive PFL chemotherapy (cisplatin 80 mg/m², 5-fluorouracil 1000 mg/m²/d, and leucovorin 120 mg/m²) every 3 weeks. The Non-leucovorin-Group (NLV-Group) of 913 patients with similar disease status who did not receive leucovorin during the same period. Histologically there were 260 patients of PF chemotherapy (PF-Group) and 653 patients of other regimen chemotherapy (NPF-Group) in NLV-Group. REP were evaluated according to MRI and CT scan imaging studies. Time to progress (TTP), the real-world local control rate (rwLCR) and OS were compared across the two cohorts using the log-rank test. REP were compared using Chi-Square and Fisher’s Exact Test. Results: The TTP of the LV-Group as compared to the NLV-Group at 3, 5, 8, 10,12 and 16 years were 98.96% vs 94.97%, 95.57% vs 90.08%, 92.01% vs 85.22%, 90.67% vs 84.39%, 88.59% vs 84.26% and 88.54% vs 81.06%, respectively. The incident of REP in LV-Group is lower than that of the NLV-Group significantly( P= 0.0413). The OS of LV-Group as compared to PF-Group at 3, 5,10 and 12 years were 97.42% vs. 91.24%, 94.61% vs. 87.44%, 88.74% vs. 81.98%和88.74% vs. 78.57%, respectively. The OS of LV-Group got higher than that of PF-Group ( P= 0.0269). The OS of LV-Group was significantly higher than that of PF-Group with 90.83% vs. 82.12% in stage I-III(P = 0.0348). The rwLCR of LV-Group as compared to PF Group were 97.06% vs. 73.91% in stage T4 ( P= 0.0133). Conclusions: Leucovorin can significantly reduce the incidence of REP of NPC combined with PF regiment. It may improve the tumor radiosensitivity in NPC patients.


Sign in / Sign up

Export Citation Format

Share Document