scholarly journals Assessment of Mortality and Its Associated Risk Factors in Patients with Transfusion Dependent Thalassemia in India

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 973-973
Author(s):  
Rakesh Dhanya ◽  
Rajat Kumar Agarwal ◽  
Amit Sedai ◽  
Ankita Kumari ◽  
Lalith Parmar ◽  
...  

Introduction: An assessment of morbidity and mortality caused by transfusion dependent thalassemia in India has never really been done despite thalassemia being the most prevalent life threatening non-communicable disorder of childhood. There is little structured understanding identifying the key risk factors feeding into research and policy making for effective management of thalassemia. With an estimated 10,000-12,000 children born with thalassemia each year in India, in addition to increasing focus on early pregnancy targeted screening, it seems critical to increase our understanding of risk factors associated with early mortality and morbidity. This is also relevant to family counselling about management options. Methodology: A retrospective analysis of mortality key risk factors in patients suffering from thalassemia major from 5 thalassemia day care centres in India was carried out. This included a total of 1,087 patients (656 males and 431 females with a median age of 8.6 years) enrolled for care between 1 Jan 2010 - 31 Oct 2018 at these centres. These centres were set up by a non-profit organization in collaboration with blood banks and /hospital facilities with the objective to provide comprehensive thalassemia care; A common web-based application was employed (ThalCare™). This system was used to track information associated with treatment including disease history at enrolment, demographic data and follow-up information. All analyses were performed with R Statistical software (3.5.2). Survival analysis was done from the age at presentation to the centre till October 2018. The reasons for mortality were categorized. Overall survival was also separately analyzed for patients in their 1st,2nd, 3rd or subsequent decades of life. The Cnaan and Ryan approach was used as patients entered and left the study cohort (left censored and right truncated data) and observation began only at enrolment and not at disease onset Results: The median age at enrolment was 5.4 years and the median follow up at the centre was 2.5 years. A total of 86 patients were cured by bone marrow transplantation (BMT), 13 of them moved to other centres for care and 41 patients died during the study period (28 males and 13 females). The median age at death was 15.4 years. Actuarial survival at 26.9 years of age was 50% (Figure 1) and under-five mortality was 7 times higher than the general population. Patients with transfusion-transmitted infections (TTI) had 3.4 times higher risk of death (p=0.031). Serum ferritin >4,000 ng/dL was associated with 4.6 times higher risk of mortality compared to ferritin <1,000 ng/dL (p=0.00063). Hemoglobin drop >2 gm/dL/week had 7.7 times higher mortality risk compared to <1 gm/dL/week (p<0.0001). Social determinants (sex, economic status and distance from centre), splenectomy, age at first transfusion and even cardiac complications were not associated with higher mortality risk. Results are summarized in Table 1. A multivariable analysis of risk factors which emerged as univariately significant showed that Hb drop of > 2 gm/week (hazard ratio 5.58 ,p=0.0007) and lack of attention towards care for possible prevention from TTI (hazard ratio 2.86, p=0.0004) are factors independently associated with high mortality. Table 2 shows that in patients born after the year 2,000 overall survival is 85.2% compared to 29.4% for patients born earlier. Main causes of death were infection, iron overload, TTIs, and alloimmunization; In a quarter of patients the cause of death was unknown (Figure 2). Patients who received more than 4 years of adequate care had more than 66% mortality risk reduction (p<0.0001). Conclusion: Comprehensive care right from an early age at dedicated management centres is key to improving life expectancy of thalassemia patients in India. Optimizing blood transfusion, intensifying chelation and preventing TTIs seem particularly important. Sustained efforts in these areas coupled with increased prevention and access to safe BMT will ease the burden for both families and public healthcare. Disclosures No relevant conflicts of interest to declare.

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2821-2821
Author(s):  
Alfonso Quintás-Cardama ◽  
Jenny Shan ◽  
Sherry Pierce ◽  
Hagop M. Kantarjian ◽  
Srdan Verstovsek

Abstract Abstract 2821 BACKGROUND: While patients (pts) with primary or post-polycythemia vera (PV)/post-essential thrombocythemia (ET) myelofibrosis (MF) have a median overall survival of 5–6 years, the survival of particular subsets of pts with MF is highly variable. Pts transforming to acute myeloid leukemia (AML) represent a large proportion of those with the poorest prognosis. Prognostic tools capable of identifying pts at the highest risk of death and transformation may provide an opportunity for tailored early therapeutic intervention (e.g. allogeneic stem cell transplantation [allo-SCT]). OBJECTIVES: To identify risk factors that predict for short survival and for high risk to transformation to AML in a large cohort of pts with MF diagnosed at MD Anderson Cancer Center. PATIENTS AND METHODS: 649 pts with MF referred from Feb-1966 to Jun-2010 were assessable for survival analysis, including 177 (27%) with post-PV or post-ET MF. The median follow-up was 19 months (range, 1–180). We first evaluated an extensive list of baseline risk factors for overall survival (OS) by univariate analysis (UVA): age, sex, prior exposure to alkylating agents, hydroxyurea, immunomodulatory drugs (i.e. thalidomide derivatives), and others, MF duration, performance status, splenomegaly, hepatomegaly, hemoglobin, white blood cell count (WBC), platelets, monocytes, peripheral blood (PB) or bone marrow (BM) blast percentage (continuous variable), chromosomal abnormalities, JAK2V617F mutation (yes vs no, and as a continuous variable), total bilirubin, LDH, creatinine level. Multivariate analysis (MVA) identified age ≥60 (p=0.004;HR=1.63), baseline platelets <100×109/L (p<0.001;HR=1.62), BM blast >10% (p=0.002;HR=2.18), worst karyotype (del17p, −5, −7, complex; p<0.001;HR=2.44), transfusion dependency (≥2 red cell transfusions; p<0.001;HR=2.64), PS ≥1 (p=0.003;HR=1.47), LDH >2000 U/L (p<0.001;HR=1.62), prior hydroxyurea (p<0.001;HR=1.69), and male gender (p=0.005;HR=1.41) as independent poor prognostic factors for OS. Using the corresponding hazard ratios, a dynamic risk model for survival in MF was derived based on weighted scores of 0–1, 2–3, 4–5, ≥6. The number of pts and median OS for the 4 risk groups were: Low: 84 (13%), median OS not reached; Intermediate-1: 191 (29%), 74 months; Intermediate-2: 208 (32%), 29 months; High :166 (26%), 11 months. We then examined the same baseline variables used in the OS analysis to identify independent risk factors for AML transformation. MVA identified WBC<3×109/L (p=0.02), PB blasts ≥5% (p=0.01), BM blasts ≥5% (p=0.02), and unfavorable karyotype (p=0.04). The presence at baseline of BM blasts ≥10% and worst karyotype were identified as independent poor prognostic factors for both OS and AML-transformation. Pts with one or both of these two risk factors (n=80, 12% of the population) defined a subgroup of pts with a median OS of only 10 months. This subgroup was considered to have MF at significantly higher risk (compared to pts without any of these 2 adverse factors) for transformation to AML (1-year transformation-free survival 82% vs 98%, p<0.001). Ten of 80 (13%) of pts with any of these two factors progressed to AML within 12 months whereas only 10 of 568 (2%) pts without those two factors progressed to AML within 12 months. The yearly rates of transformation for the whole study group during the first, second, and third year of follow-up were 3%, 2%, and 3%, respectively. CONCLUSION: Analysis of a large population of pts with either primary or post-PV or post-ET MF identified risk factors that at any time during the course of MF predict for short OS and for high risk for AML transformation AML. Pts with such factors may be candidates for more aggressive therapeutic approaches such as allo-SCT. Disclosures: No relevant conflicts of interest to declare.


2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Antonio Bellasi ◽  
Luca Di Lullo ◽  
Domenico Russo ◽  
Carlo Ratti ◽  
Mario Gennaro Cozzolino ◽  
...  

Abstract Background and Aims It is estimated that Chronic Kidney Disease (CKD) accounts for 5 to 10 million deaths annually, mainly due to cardiovascular (CV) diseases. Although traditional CV risk factors are prevalent, other non-traditional CV risk factors such as vascular calcification (VC) are believed to contribute to this disproportionate CV risk burden in CKD subjects. We sought to investigate the association of Coronary Artery Calcification (CAC) progression with all-cause mortality in a cohort of patients new to hemodialysis (HD). Method This is a post hoc analysis of the Independent study (NCT00710788) originally designed to test the impact of 2 different phosphate binder regimens on various hard as well as surrogate endpoint in HD subjects. A total of 412 (88.4% of the Independent study cohort) underwent repeated CAC quantification according to the Agatston methods at study inception as well as after 12 months of follow-up. The square root method was used to assess CAC progression (CACP) and survival analyses were used to check the association of CACP and all-cause mortality. Results 412 middle age (65 years) men and women (51.2%) were considered. Detectable CAC was present in about 2 out 3 patients (68.2%) at study inception. At 12 months of follow-up completion, about 1 out of 3 subjects (33.1%) experience a significant CACP. CACP was associated with older age and use of calcium-based phosphate binders. At study completion (median follow-up: 36 months) 106 patients expired of all-cause. Age, diabetes mellitus, atherosclerotic CV events, baseline CAC extension were predictors of unfavorable outcome. Multivariable adjusted analysis confirmed an independent association of both baseline CAC (Hazard Ratio 1.29; 95% Confidence Interval: 1.17-1.44) and CACP (HR: 5.16; 95%CI: 2.61-10.21) with all-cause mortality. However, CACP diminished the risk associated with baseline CAC (p for interaction term 0.002) and use of calcium-free phosphate binders significantly weakened the link between CACP (HR. 1.95; 95%CI: 0.92-4.16) and mortality Conclusion Baseline CAC as well as CACP predict mortality in incident to HD individuals. Nevertheless, CACP mitigates the risk associated with baseline CAC and calcium-free phosphate binders attenuates the association of CACP and mortality, suggesting that CACP modulation may impact survival in this population


2018 ◽  
Vol 133 (2) ◽  
pp. 177-181 ◽  
Author(s):  
John A. Schinka ◽  
Katherine C. Leventhal ◽  
William A. Lapcevic ◽  
Roger Casey

Objectives: Increased mortality has been documented in older homeless veterans. This retrospective study examined mortality and cause of death in a cohort of young and middle-aged homeless veterans. Methods: We examined US Department of Veterans Affairs records on homelessness and health care for 2000-2003 and identified 23 898 homeless living veterans and 65 198 non-homeless living veterans aged 30-54. We used National Death Index records to determine survival status. We compared survival rates and causes of death for the 2 groups during a 10-year follow-up period. Results: A greater percentage of homeless veterans (3905/23 898, 16.3%) than non-homeless veterans (4143/65 198, 6.1%) died during the follow-up period, with a hazard ratio for risk of death of 2.9. The mean age at death (52.3 years) for homeless veterans was approximately 1 year younger than that of non-homeless veterans (53.2 years). Most deaths among homeless veterans (3431/3905, 87.9%) and non-homeless veterans (3725/4143, 89.9%) were attributed to 7 cause-of-death categories in the International Classification of Diseases, 10th Revision (cardiovascular system; neoplasm; external cause; digestive system; respiratory system; infectious disease; and endocrine, nutritional, and metabolic diseases). Death by violence was rare but was associated with a significantly higher risk among homeless veterans than among non-homeless veterans (suicide hazard ratio = 2.7; homicide hazard ratio = 7.6). Conclusions: Younger and middle-aged homeless veterans had higher mortality rates than those of their non-homeless veteran peers. Our results indicate that homelessness substantially increases mortality risk in veterans throughout the adult age range. Health assessment would be valuable for assessing the mortality risk among homeless veterans regardless of age.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Runzhen Chen ◽  
Chen Liu ◽  
Peng Zhou ◽  
Yu Tan ◽  
Zhaoxue Sheng ◽  
...  

Abstract Background Associations between D-dimer and outcomes of patients with acute coronary syndromes (ACS) remain controversial. This study aimed to investigate the prognostic value of D-dimer in ACS patients treated by percutaneous coronary intervention (PCI). Methods In this observational study, 3972 consecutive patients with ACS treated by PCI were retrospectively recruited. The X-tile program was used to determine the optimal D-dimer thresholds for risk stratifications. Cox regression with multiple adjustments was used for outcome analysis. Restricted cubic spline (RCS) analysis was performed to assess the dose-response association between D-dimer and outcomes. The C-index was calculated to evaluate the additional prognostic value of D-dimer when added to clinical risk factors and commonly used clinical risk scores, with internal validations using bootstrapping methods. The primary outcome was all-cause death. Results During a median follow-up of 720 days, 225 deaths occurred. Based on the thresholds generated by X-tile, ACS-PCI patients with median (420–1150 ng/mL, hazard ratio [HR]: 1.58, 95 % confidence interval [CI]: 1.14–2.20, P = 0.007) and high (≥ 1150 ng/mL, HR: 1.98, 95 % CI: 1.36–2.89, P < 0.001) levels of D-dimer showed substantially higher risk of death compared to those with low D-dimer (< 420 ng/mL). RCS analysis depicted a constant relation between D-dimer and various outcomes. The addition of D-dimer levels significantly improved risk predictions for all-cause death when combined with the fully adjusted models (C-index: 0.853 vs. 0.845, P difference = 0.021), the GRACE score (C-index: 0.826 vs. 0.814, P difference = 0.027), and the TIMI score (C-index: 0.804 vs. 0.776, P difference < 0.001). The predicted mortality at the median follow-up (two years) was 1.7 %, 5.2 %, and 10.9 % for patients with low, median, and high D-dimer, respectively, which was well matched with the observed mortality (low D-dimer group: 1.2 %, median D-dimer group: 5.2 %, and high D-dimer group: 12.6 %). Conclusions For ACS patients treated by PCI, D-dimer level was an independent predictor for adverse outcomes, and provided additional prognostic value when combined with clinical risk factors and risk scores. Risk stratifications based on D-dimer was plausible to differentiate ACS-PCI patients with higher risk of death.


Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1361
Author(s):  
Zoia Razumova ◽  
Husam Oda ◽  
Igor Govorov ◽  
Eva Lundin ◽  
Ellinor Östensson ◽  
...  

Endometrial cancer (EC) is the most common gynecologic malignancy in Sweden and it has various prognostic factors. The LRIG family is a group of three integral surface proteins with a similar domain organization. The study aimed to explore LRIG family as prognostic factor proteins in EC. The initial study cohort included 100 women with EC who were treated at the Department of Women’s and Children’s Health, Karolinska University Hospital Solna, between 2007 and 2012. We assessed the associations between LRIG protein expression and type, grade, and stage of EC, as well as progression-free and overall survival. Immunohistochemistry results revealed that most women in the analytical sample had >50% LRIG1-, LRIG2- and LRIG3-positive cells. A statistically significant association was observed between having a high number of LRIG3-positive cells and superior overall survival (incidence rate ratio = 0.977; 95% confidence interval: 0.958–0.996, p = 0.019). Moreover, positive LRIG3 staining of the cell membrane was associated with reducing in the risk of death (hazard ratio = 0.23; 95% confidence interval: 0.09–0.57). Our results show that LRIG3 expression might be a prognostic factor in EC. The role of LRIG1 and LRIG2 expression remains to be further investigated.


Circulation ◽  
2012 ◽  
Vol 125 (suppl_10) ◽  
Author(s):  
Baback Roshanravan ◽  
Cassiane Robinson-Cohen ◽  
Kushang V Patel ◽  
Greg Levin ◽  
Ian H de Boer ◽  
...  

Objective: Skeletal muscle dysfunction (sarcopenia) is an under-recognized complication of chronic kidney disease (CKD) that may have important clinical consequences. Gait speed is associated with sarcopenia and comorbid disease burden among older adults; however, little is known about the prognostic significance of gait speed in CKD. We determined the association of gait speed with all-cause mortality in a prospective cohort of non-dialysis CKD patients. Methods: We measured usual gait speed over 4-meters in 309 participants from a prospective study of non-dialysis CKD. Included subjects had an estimated glomerular filtration rate (eGFR ckdepi ) <90mL/min/1.73m 2 , were stroke-free and did not require a wheelchair for ambulation. Study coordinators assessed mortality during follow-up by phone contacts, medical record review, and the social security death index. We evaluated gait speed continuously, and using a cut point of 0.8 m/s, consistent with previous studies. We used Cox's proportional hazards to estimate the association of gait speed with mortality after adjustment for age, sex, race, smoking, diabetes, pre-existing CAD, BMI, eGFR and hemoglobin. Results: Median follow-up time was 2.7 years; range 27 days to 4.8 years. The mean age was 58.9 ± 13 years and mean eGFR by cystatin C (eGFR cysc ) was 48.5 ± 23mL/min/1.73m 2 . There were a total of 31 deaths (10.4%) during follow-up. Unadjusted mortality rates were 23 and 80 deaths per 1,000 person-years among participants who had a gait speed of >0.8m/s versus ≤0.8m/s, respectively. After full adjustment, gait speed ≤0.8m/s was associated with a 2.8-fold greater risk of death compared to a gait speed >0.8 m/s. Gait speed was also strongly associated with mortality when analyzed as a continuous variable ( Table ) and a stronger predictor of death than age, history of CAD, or diabetes. No. Deaths (%) Model 1 + Model 2 # Hazard Ratio 95% CI Hazard Ratio 95% CI Gait speed * 32(10) 0.74 (0.64-0.86) 0.75 (0.64-0.87) >0.8m/s 13 (6) Reference Reference ≤0.8m/s 19(19) 3.49 (1.54-7.95) 2.84 (1.25-6.48) * Gait speed analyzed continuously per 10cm/s increase in speed. +Model 1: Adjusted for age, sex, race, study site #Model 2: adds smoking, BMI, eGFR cysc , diabetes, prevalent coronary disease. Conclusion: Gait speed is strongly associated with death in a cohort of middle-aged CKD patients.


2016 ◽  
Vol 134 (6) ◽  
pp. 543-554 ◽  
Author(s):  
Alessandra Carvalho Goulart ◽  

ABSTRACT CONTEXT AND OBJECTIVE: Stroke has a high burden of disability and mortality. The aim here was to evaluate epidemiology, risk factors and prognosis for stroke in the EMMA Study (Study of Stroke Mortality and Morbidity). DESIGN AND SETTINGS: Prospective community-based cohort carried out in Hospital Universitário, University of São Paulo, 2006-2014. METHODS: Stroke data based on fatal and non-fatal events were assessed, including sociodemographic data, mortality and predictors, which were evaluated by means of logistic regression and survival analyses. RESULTS: Stroke subtype was better defined in the hospital setting than in the local community. In the hospital phase, around 70% were first events and the ischemic subtype. Among cerebrovascular risk factors, the frequency of alcohol intake was higher in hemorrhagic stroke (HS) than in ischemic stroke (IS) cases (35.4% versus 12.3%, P < 0.001). Low education was associated with higher risk of death, particularly after six months among IS cases (odds ratio, OR, 4.31; 95% confidence interval, CI, 1.34-13.91). The risk of death due to hemorrhagic stroke was greater than for ischemic stroke and reached its maximum 10 days after the event (OR: 3.31; 95% CI: 1.55-7.05). Four-year survival analysis on 665 cases of first stroke (82.6% ischemic and 17.4% hemorrhagic) showed an overall survival rate of 48%. At four years, the highest risks of death were in relation to ischemic stroke and illiteracy (hazard ratio, HR: 1.83; 95% CI: 1.26-2.68) and diabetes (HR: 1.45; 95% CI: 1.07-1.97). Major depression presented worse one-year survival (HR: 4.60; 95% CI: 1.36-15.55). CONCLUSION: Over the long term, the EMMA database will provide additional information for planning resources destined for the public healthcare system.


Author(s):  
Jessica G Abell ◽  
Camille Lassale ◽  
G David Batty ◽  
Paola Zaninotto

Abstract Background Falls in later life that require admission to hospital have well-established consequences for future disability and health. The likelihood and severity of a fall will result from the presence of one or more risk factors. The aim of this study is to examine risk factors identified for their ability to prevent falls and to assess whether they are associated with hospital admission after a fall. Methods Analyses of data from the English Longitudinal Study of Aging (ELSA), a prospective cohort study. In a sample of 3783 men and women older than 60 years old, a range of potential risk factors measured at Wave 4 (demographic, social environment, physical, and mental functioning) were examined as predictors of fall-related hospitalizations, identified using International Classification of Diseases, 10th Revision (ICD-10) code from linked hospital records in the United Kingdom. Subdistribution hazard models were used to account for competing risk of death. Results Several risk factors identified by previous work were confirmed. Suffering from urinary incontinence (subdistribution hazard ratio = 1.49; 95% CI: 1.14, 1.95) and osteoporosis (subdistribution hazard ratio = 1.48; 95% CI: 1.05, 2.07), which are not commonly considered at an early stage of screening, were found to be associated with hospital admission after a fall. Both low and moderate levels of physical activity were also found to somewhat increase the risk of hospital admission after a fall. Conclusions Several predictors of having a fall, severe enough to require hospital admission, have been confirmed. In particular, urinary incontinence should be considered at an earlier point in the assessment of risk.


Author(s):  
Ramachandran S. Vasan ◽  
Solomon K. Musani ◽  
Kunihiro Matsushita ◽  
Walter Beard ◽  
Olushola B. Obafemi ◽  
...  

Background Black individuals have a higher burden of risk factors for heart failure (HF) and subclinical left ventricular remodeling. Methods and Results We evaluated 1871 Black participants in the Atherosclerosis Risk in Communities Study cohort who attended a routine examination (1993–1996, median age 58 years) when they underwent echocardiography. We estimated the prevalences of 4 HF stages: (1) Stage 0 : no risk factors; (2) Stage A : presence of HF risk factors (hypertension, diabetes mellitus, obesity, smoking, dyslipidemia, coronary artery disease without clinical myocardial infarction), no cardiac structural/functional abnormality; (3) Stage B : presence of prior myocardial infarction, systolic dysfunction, left ventricular hypertrophy, regional wall motion abnormality, or left ventricular enlargement; and (4) Stage C/D : prevalent HF. We assessed the incidence of clinical HF, atherosclerotic cardiovascular disease events, and all‐cause mortality on follow‐up according to HF stage. The prevalence of HF Stages 0, A, B, and C/D were 3.8%, 20.6%, 67.0%, and 8.6%, respectively, at baseline. On follow‐up (median 19.0 years), 309 participants developed overt HF, 390 incurred new‐onset cardiovascular disease events, and 651 individuals died. Incidence rates per 1000 person‐years for overt HF, cardiovascular disease events, and death, respectively, were Stage 0, 2.4, 0.8, and 7.6; Stage A, 7.4, 9.7, and 13.5; Stage B 13.6, 15.9, and 22.0. Stage B HF was associated with a 1.5‐ to 2‐fold increased adjusted risk of HF, cardiovascular disease events and death compared with Stages 0/A. Conclusions In our large community‐based sample of Black individuals, we observed a strikingly high prevalence of Stage B HF in middle age that was a marker of high cardiovascular morbidity and mortality.


2013 ◽  
Vol 142 (7) ◽  
pp. 1362-1374 ◽  
Author(s):  
B. FAUROUX ◽  
J.-B. GOUYON ◽  
J.-C. ROZE ◽  
C. GUILLERMET-FROMENTIN ◽  
I. GLORIEUX ◽  
...  

SUMMARYThe aim of this study was to describe the incidence and risk factors for respiratory morbidity during the 12-month period following the first respiratory syncytial virus (RSV) season in 242 preterm infants [<33 weeks gestational age (GA)] without bronchopulmonary dysplasia and 201 full-term infants (39–41 weeks GA) from the French CASTOR study cohort. Preterm infants had increased respiratory morbidity during the follow-up period compared to full-terms; they were more likely to have wheezing (21% vs. 11%, P = 0·007) and recurrent wheezing episodes (4% vs. 1%, P = 0·049). The 17 infants (14 preterms, three full-terms) who had been hospitalized for RSV-confirmed bronchiolitis during their first RSV season had significantly more wheezing episodes during the follow-up period than subjects who had not been hospitalized for RSV-confirmed bronchiolitis (odds ratio 4·72, 95% confidence interval 1·71–13·08, P = 0·003). Male gender, birth weight <3330 g and hospitalization for RSV bronchiolitis during the infant's first RSV season were independent risk factors for the development of wheezing episodes during the subsequent 12-month follow-up period.


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