scholarly journals Results from the United States CTEPH Registry (US-CTEPH-R): Enrollment Characteristics and 1-Year Follow-up

CHEST Journal ◽  
2021 ◽  
Author(s):  
Kim M. Kerr ◽  
C. Greg Elliott ◽  
Kelly Chin ◽  
Raymond L. Benza ◽  
Richard N. Channick ◽  
...  
2019 ◽  
Vol 71 (1) ◽  
pp. 53-62 ◽  
Author(s):  
Gloria H Hong ◽  
Ana M Ortega-Villa ◽  
Sally Hunsberger ◽  
Ploenchan Chetchotisakd ◽  
Siriluck Anunnatsiri ◽  
...  

Abstract Background The natural history of anti-interferon-γ (IFN-γ) autoantibody-associated immunodeficiency syndrome is not well understood. Methods Data of 74 patients with anti-IFN-γ autoantibodies at Srinagarind Hospital, Thailand, were collected annually (median follow-up duration, 7.5 years). Annual data for 19 patients and initial data for 4 patients with anti-IFN-γ autoantibodies at the US National Institutes of Health were collected (median follow-up duration, 4.5 years). Anti-IFN-γ autoantibody levels were measured in plasma samples. Results Ninety-one percent of US patients were of Southeast Asian descent; there was a stronger female predominance (91%) in US than Thai (64%) patients. Mycobacterium abscessus (34%) and Mycobacterium avium complex (83%) were the most common nontuberculous mycobacteria in Thailand and the United States, respectively. Skin infections were more common in Thailand (P = .001), whereas bone (P < .0001), lung (P = .002), and central nervous system (P = .03) infections were more common in the United States. Twenty-four percent of Thai patients died, most from infections. None of the 19 US patients with follow-up data died. Anti-IFN-γ autoantibody levels decreased over time in Thailand (P < .001) and the United States (P = .017), with either cyclophosphamide (P = .01) or rituximab therapy (P = .001). Conclusions Patients with anti-IFN-γ autoantibodies in Thailand and the United States had distinct demographic and clinical features. While titers generally decreased with time, anti-IFN-γ autoantibody disease had a chronic clinical course with persistent infections and death. Close long-term surveillance for new infections is recommended.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1027.2-1027
Author(s):  
A. R. Broder ◽  
W. Mowrey ◽  
A. Valle ◽  
B. Goilav ◽  
K. Yoshida ◽  
...  

Background:The development of ESRD due to lupus nephritis is one of the most common and serious complications of SLE. Mortality among SLE ESRD patients is 4-fold higher compared to lupus nephritis patients with preserved renal function1Mortality in SLE ESRD is also twice as high compared with non-SLE ESRD, even though SLE patients develop ESRD at a significantly younger age. In the absence of ESRD specific guidelines, medication utilization in SLE ESRD is unknown.Objectives:The objective of this study was to investigate the real-world current US-wide patterns of medication prescribing among lupus nephritis patients with new onset ESRD enrolled in the United States Renal Disease Systems (USRDS) registry. We specifically focused on HCQ and corticosteroids (CS) as the most used medications to treat SLE.Methods:Inclusion: USRDS patients 18 years and above with SLE as a primary cause of ESRD (International Classification of Diseases, 9thRevision (ICD9) diagnostic code 710.0, previously validated2). who developed ESRD between January 1st, 2006 and July 31, 2011 (to ensure at least 6 months of follow-up in the USRDS). Patients had to be enrolled in Medicare Part D (to capture pharmacy claims). The last follow-up date was defined as either the last date of continuous part D coverage or the end of the study period, Dec 31, 2013.Results:Of the 2579 patients included, 1708 (66%) were HCQ- at baseline, and 871 (34%) were HCQ+ at baseline. HCQ+ patients at baseline had a slightly lower duration of follow-up compared to HCQ- patients at baseline, median (IQR) of 2.32 (1.33, 3.97) years and 2.55 (1.44, 4.25) years, respectively, p= 0.02. During follow-up period, only 778 (30%) continued HCQ either intermittently or continuously to the last follow-up date, 1306 (51%) were never prescribed HCQ after baseline, and 495 (19%) discontinued HCQ before the last follow-up date. Of the 1801 patients who were either never prescribed or discontinued HCQ early after ESRD onset, 713 (40%) were prescribed CS to the end of the follow-up period: 55% were receiving a low dose <10mg/daily, and 43 were receiving moderate dose (10-20mg daily)Conclusion:HCQ may be underprescribed and CS may be overprescribed in SLE ESRD. Changing the current prescribing practices may improve outcomes in SLE ESRDReferences:[1]Yap DY et al., NDT 2012.[2]Broder A et al., AC&R 2016.Acknowledgments :The data reported here have been supplied by the United States Renal Data System (USRDS). The interpretation and reporting of these data are the responsibility of the author(s) and in no way should be seen as an official policy or interpretation of the U.S. government.Funding: :NIH/NIAMS K23 AR068441 (A Broder), NIH/NIAMS R01 AR 057327 and K24 AR 066109 (KH Costenbader)Disclosure of Interests: :Anna R. Broder: None declared, Wenzhu Mowrey: None declared, Anna Valle: None declared, Beatrice Goilav: None declared, Kazuki Yoshida: None declared, Karen Costenbader Grant/research support from: Merck, Consultant of: Astra-Zeneca


2015 ◽  
Vol 116 (4) ◽  
pp. 226-230 ◽  
Author(s):  
Sarah Viall ◽  
Sneha Jain ◽  
Kimberly Chapman ◽  
Nicholas Ah Mew ◽  
Marshall Summar ◽  
...  

2018 ◽  
Vol 3 (3) ◽  
pp. 2473011418S0003
Author(s):  
Elizabeth Cody ◽  
Michel Taylor ◽  
James Nunley ◽  
Selene Parekh ◽  
James DeOrio

Category: Ankle Arthritis Introduction/Purpose: Modern total ankle arthroplasties (TAAs) have demonstrated improved survival rates at early- and mid-term follow-up, with revision rates ranging from 4 to 8% at five years. The INFINITY total ankle system (Wright Medical Technology, Arlington, TN) was first used in the United States in 2014. Its advantages include the ability to use patient-specific instrumentation and the option to choose between talar dome resurfacing and flat-cut talar components. While this implant is currently popular in the United States, clinical outcomes have not yet been reported. Our aim was to identify the rate of early revision among patients receiving the INFINITY prosthesis. Methods: Patients from two prospectively-collected databases at the authors’ institution were screened for inclusion in the present study. All patients who underwent a primary TAA with the INFINITY prosthesis and who were at least one year postoperative were included. All surgeries were performed by one of two orthopaedic foot and ankle surgeons with extensive experience in total ankle arthroplasty. The primary outcome was the need for revision surgery, which was defined as removal of one or both metal components. Peri-implant lucency at most recent follow-up was a secondary outcome. Anteroposterior and lateral radiographs at most recent follow-up were graded for lucency independently by two reviewers, both orthopaedic foot and ankle fellows, for individual peri-implant zones (Figure). Each zone was only considered “lucent” if recorded as such by both reviewers. Results: 160 patients underwent TAA with the INFINITY prosthesis between August 2014 and November 2016 with a mean 20 months of follow-up (range, 12-37). Six patients were lost to follow-up. Sixteen patients (10%) underwent revision a mean 1.2 years postoperatively. Revision was performed most commonly for tibial component loosening (seven patients, 4.4%) and deep infection (five patients, 3.1%). Of cases with tibial loosening, progressive lucency and/or subsidence was obvious radiographically in four patients; one patient had equivocal radiographs but loosening was suggested on single-photon emission computed tomography; and two patients revised for persistent pain had loosening confirmed intraoperatively. Of the 108 patients with retained components and at least one year of radiographic follow-up, eight (7.4%) had global lucency around the tibial component at most recent follow-up. Conclusion: Our initial review of patients undergoing TAA with the INFINITY prosthesis demonstrates an elevated early revision rate due to tibial component loosening. The reasons for this finding remain unclear, but could possibly include inadequate bony purchase of the implant’s three prongs, particularly in patients with large preoperative deformities or with imperfect component alignment. We plan to further investigate the possible reasons for this finding in the future by assessing additional patient factors, including age, sex, arthritis type, tobacco use, pre- and postoperative coronal and sagittal alignment, and presence of ipsilateral hindfoot fusion.


2018 ◽  
Vol 133 (2_suppl) ◽  
pp. 60S-74S ◽  
Author(s):  
Patricia Sweeney ◽  
Tamika Hoyte ◽  
Mesfin S. Mulatu ◽  
Jacquelyn Bickham ◽  
Antoine D. Brantley ◽  
...  

Objectives: The Care and Prevention in the United States Demonstration Project included implementation of a Data to Care strategy using surveillance and other data to (1) identify people with HIV infection in need of HIV medical care or other services and (2) facilitate linkages to those services to improve health outcomes. We present the experiences of 4 state health departments: Illinois, Louisiana, Tennessee, and Virginia. Methods: The 4 state health departments used multiple databases to generate listings of people with diagnosed HIV infection (PWH) who were presumed not to be in HIV medical care or who had difficulty maintaining viral suppression from October 1, 2013, through September 29, 2016. Each health department prioritized the listings (eg, by length of time not in care, by viral load), reviewed them for accuracy, and then disseminated the listings to staff members to link PWH to HIV care and services. Results: Of 16 391 PWH presumed not to be in HIV medical care, 9852 (60.1%) were selected for follow-up; of those, 4164 (42.3%) were contacted, and of those, 1479 (35.5%) were confirmed to be not in care. Of 794 (53.7%) PWH who accepted services, 694 (87.4%) were linked to HIV medical care. The Louisiana Department of Health also identified 1559 PWH as not virally suppressed, 764 (49.0%) of whom were eligible for follow-up. Of the 764 PWH who were eligible for follow-up, 434 (56.8%) were contacted, of whom 269 (62.0%) had treatment adherence issues. Of 153 PWH who received treatment adherence services, 104 (68.0%) showed substantial improvement in viral suppression. Conclusions: The 4 health departments established procedures for using surveillance and other data to improve linkage to HIV medical care and health outcomes for PWH. To be effective, health departments had to enhance coordination among surveillance, care programs, and providers; develop mechanisms to share data; and address limitations in data systems and data quality.


10.36469/9871 ◽  
2014 ◽  
Vol 1 (3) ◽  
pp. 254-265 ◽  
Author(s):  
Ariel Berger ◽  
John Edelsberg ◽  
Simon Teal ◽  
Marko A. Mychaskiw ◽  
Gerry Oster

Background: Pulmonary arterial hypertension (PAH) is a disease characterized by dyspnea, fatigue, chest pain and syncope. As there is no known cure for PAH, the goal of treatment is to control symptoms and slow disease progression. Sildenafil, a phosphodiesterase-5 inhibitor, has been indicated to improve exercise capacity in PAH in both the United States and the European Union since 2005; since 2009, it also has been indicated in the United States to delay clinical worsening. Patterns of sildenafil use in PAH patients have not been reported. Objectives: To describe patterns of treatment with sildenafil among commercially insured patients in the United States with PAH. Methods: Using a large U.S. healthcare claims database, we identified all patients with evidence of PAH (International Classification of Disease, 9th Revision, Clinical Modification [ICD-9-CM] diagnosis codes 416.0, 416.8) and receipt of sildenafil between January 1, 2005 and September 30, 2008. The date of each patient’s earliest pharmacy claim for sildenafil was designated as his or her “index date”; patients with &lt;6 months of data prior to this date were excluded. Post-index use of sildenafil was then examined in terms of the numbers of pharmacy claims and therapy-days, the medication possession ratio (MPR), and the incidence of therapy switching. Results: We identified a total of 855 PAH patients who began sildenafil therapy and met all other entry criteria. Mean (standard deviation [SD]) follow-up was 423.4 (313.0) days. Over this period, these patients averaged 7.1 (6.8) (median, 5) pharmacy dispensings for sildenafil, representing 273.4 (254.8) therapy-days (median, 180). Mean MPR was 71% (median, 83%). Fourteen percent of sildenafil patients switched to another agent during follow-up. Conclusions: In “real-world” clinical practice, many PAH patients beginning treatment with sildenafil remain on therapy for extended periods and are relatively compliant with treatment.


Author(s):  
Philip Moons ◽  
Sandra Skogby ◽  
Ewa‐Lena Bratt ◽  
Liesl Zühlke ◽  
Ariane Marelli ◽  
...  

Background The majority of people born with congenital heart disease require lifelong cardiac follow‐up. However, discontinuity of care is a recognized problem and appears to increase around the transition to adulthood. We performed a systematic review and meta‐analysis to estimate the proportion of adolescents and emerging adults with congenital heart disease discontinuing cardiac follow‐up. In pooled data, we investigated regional differences, disparities by disease complexity, and the impact of transition programs on the discontinuity of care. Methods and Results Searches were performed in PubMed, Embase, Cinahl, and Web of Science. We identified 17 studies, which enrolled 6847 patients. A random effects meta‐analysis of single proportions was performed according to the DerSimonian‐Laird method. Moderator effects were computed to explore sources for heterogeneity. Discontinuity proportions ranged from 3.6% to 62.7%, with a pooled estimated proportion of 26.1% (95% CI, 19.2%–34.6%). A trend toward more discontinuity was observed in simple heart defects (33.7%; 95% CI, 15.6%–58.3%), compared with moderate (25.7%; 95% CI, 15.2%–40.1%) or complex congenital heart disease (22.3%; 95% CI, 16.5%–29.4%) ( P =0.2372). Studies from the United States (34.0%; 95% CI, 24.3%–45.4%), Canada (25.7%; 95% CI, 17.0%–36.7%), and Europe (6.5%; 95% CI, 5.3%–7.9%) differed significantly ( P =0.0004). Transition programs were shown to have the potential to reduce discontinuity of care (12.7%; 95% CI, 2.8%–42.3%) compared with usual care (36.2%; 95% CI, 22.8%–52.2%) ( P =0.1119). Conclusions This meta‐analysis showed that there is a high proportion of discontinuity of care in young people with congenital heart disease. The highest discontinuity proportions were observed in studies from the United States and in patients with simple heart defects. It is suggested that transition programs have a protective effect. Registration URL: www.crd.york.ac.uk/prospero . Unique identifier: CRD42020182413.


2017 ◽  
Vol 223 (1) ◽  
pp. 399-422
Author(s):  
M. Shaheen Siham Abdel Razzaq

The events that took place in Iran during the second world war are considered an important item for Iraqi diplomacy and follow-up by the Foreign Ministry in Iraq. On the other hand, this Iraqi diplomacy was considered to be quite flexible when a reshuffle occurred. It was looking for its causes and  linking them, and then adopting accurate scenarios to protect its interests. Iraq was not far from what was happening in Iran .When Mohammad Reza Pahlavi took power in Iran, The oil conflict has also existed. In addition to Iran's strategic position, making US intervention clear. Which prompted Iran to build an intimate relationship with the United States and strengthen its relations in all respects, especially when Ahmed assigned the strength of the Sultanate to form the ministry on the ninth of August 1924 and consider America a third force used by Ahmed Qawam as a bargaining chip to confront the British and Soviet Union in the region. After the issue of oil emerged on the political scene and at that time, the Iranian government took a deep breath in the embrace of the United States. In fact, Reza Shah Pahlawi inherited a backward country, especially in the economic field. He tried to reform the country's economic recession and make Iran to acquire a new stage, and the result of foreign demand for Iranian oil led to widespread reactions at the internal level and became pro-Western groups to reject the Soviet demand and solidarity with the independent. While the Iranian Communist Party (Toda Party) supported the request and held demonstrations for the immediate admission of the Soviet Union


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