Prognosis and acute complications at the first onset of idiopathic nephrotic syndrome in children: a nationwide survey in Japan (JP-SHINE study)

Author(s):  
Mai Sato ◽  
Kenji Ishikura ◽  
Takashi Ando ◽  
Kaori Kikunaga ◽  
Chikako Terano ◽  
...  

Abstract Background Information on the epidemiology of idiopathic nephrotic syndrome (INS) in children, complications of INS and the side effects of steroid therapy is scarce. Methods The Japanese Pediatric Survey Holding Information of Nephrotic Syndrome, a nationwide cohort study, was conducted by the Japanese Study Group of Renal Disease in Children and enrolled 2099 children with newly diagnosed INS between 1 January 2010 and 31 December 2012. We conducted a follow-up study of the complications during the first onset and the patients’ prognosis in this cohort. Results We obtained follow-up data on 999 children (672 males) with a median age at onset of 4.5 years [interquartile range (IQR) 2.8–9.4] and a median follow-up period of 4.1 years (IQR 2.5–5.1). At the first onset, 24% of patients experienced severe acute kidney injury (AKI), defined as a serum creatinine increase to a level two or more times the baseline. On logistic regression analysis, age, hematuria, severe hypoalbuminemia (serum albumin <1.0 g/dL) and severe bacterial infection were not independent factors, but female sex {hazard ratio [HR] 1.5 [95% confidence interval (CI) 1.1–1.7]} and hypertension [HR 4.0 (95% CI 2.6–6.0)] were significantly related to AKI. During the observation period, ocular hypertension requiring treatment occurred in 17.4% of patients, among which 0.4% received surgical treatment. Progression to frequently relapsing nephrotic syndrome/steroid-dependent nephrotic syndrome in 3 years was seen in 44.2% of the patients and was shown by the Cox regression analysis to be significantly related to younger age and days until remission at the first episode, but not to sex, hematuria, the minimum serum albumin level or AKI. Two patients died during the observation period. One patient showed progression to end-stage kidney disease. Conclusion Based on the results of a multicenter questionnaire survey, the overall survival and renal survival rates were found to be excellent. However, proper management of complications, particularly in AKI and ocular hypertension, is mandatory.

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4253-4253
Author(s):  
Hanne Rozema ◽  
Robby Kibbelaar ◽  
Nic Veeger ◽  
Mels Hoogendoorn ◽  
Eric van Roon

The majority of patients with myelodysplastic syndromes (MDS) require regular red blood cell (RBC) transfusions. Alloimmunization (AI) against blood products is an adverse event, causing time-consuming RBC compatibility testing. The reported incidence of AI in MDS patients varies greatly. Even though different studies on AI in MDS patients have been performed, there are still knowledge gaps. Current literature has not yet fully identified the risk factors and dynamics of AI in individual patients, nor has the influence of disease modifying treatment (DMT) been explored. Therefore, we performed this study to evaluate the effect of DMT on AI. An observational, population-based study, using the HemoBase registry, was performed including all newly diagnosed MDS patients between 2005 and 2017 in Friesland, a province of the Netherlands. All available information about treatment and transfusions, including transfusion dates, types, and treatment regimens, was collected from the electronic health records and laboratory systems. Follow-up occurred through March 2019. For our patient cohort, blood products were matched for AB0 and RhD, and transfused per the 'type and screen' policy (i.e. electronic matching of blood group phenotype between patient and donor). After a positive antibody screening, antibody identification and Rh/K phenotyping was performed and subsequent blood products were (cross)matched accordingly. The observation period was counted from first transfusion until last transfusion or first AI event. Univariate analyses and cumulative frequency distributions were performed to study possible risk factors and dynamics of AI. DMT was defined as hypomethylating agents, lenalidomide, chemotherapy and monoclonal antibodies. The effect of DMT as a temporary risk period on the risk of AI was estimated with incidence rates, relative risks (RR) and hazard ratios (HR) using a cox regression analysis. Follow-up was limited to 24 months for the cox regression analysis to avoid possible bias by survival differences. Statistical analyses were performed using IBM SPSS 24 and SAS 9.4. Out of 292 MDS patients, 236 patients received transfusions and were included in this study, covering 463 years of follow-up. AI occurred in 24 patients (10%). AI occurred mostly in the beginning of the observation period: Eighteen patients (75%) were alloimmunized after receiving 20 units of RBCs, whereas 22 patients (92%) showed AI after 45 units of RBCs (Figure 1). We found no significant risk factors for AI in MDS patients at baseline. DMT was given to 67 patients (28%) during the observation period. Patients on DMT received more RBC transfusions than patients that did not receive DMT (median of 33 (range: 3-154) and 11 (range: 0-322) RBC units respectively, p<0,001). Four AI events (6%) occurred in patients on DMT and 20 AI events (12%) occurred in patients not on DMT. Cox regression analysis of the first 24 months of follow-up showed an HR of 0.30 (95% CI: 0.07-1.31; p=0.11). The incidence rates per 100 person-years were 3.19 and 5.92 respectively. The corresponding RR was 0.54 (95% CI: 0.16-1.48; p=0.26). Based on our results, we conclude that the incidence of AI in an unselected, real world MDS population receiving RBC transfusions is 10% and predominantly occurred in the beginning of follow-up. Risk factors for AI at baseline could not be identified. Our data showed that patients on DMT received significantly more RBC transfusions but were less susceptible to AI. Therefore, extensive matching of blood products may not be necessary for patients on DMT. Larger studies are needed to confirm the protective effect of DMT on AI. Disclosures Rozema: Celgene: Other: Financial support for visiting MDS Foundation conference.


2020 ◽  
Vol 17 (3) ◽  
pp. 218-223
Author(s):  
Haichao Wang ◽  
Li Gong ◽  
Xiaomei Xia ◽  
Qiong Dong ◽  
Aiping Jin ◽  
...  

Background: Depression and anxiety after stroke are common conditions that are likely to be neglected. Abnormal red blood cell (RBC) indices may be associated with neuropsychiatric disorders. However, the association of RBC indices with post-stroke depression (PSD) and poststroke anxiety (PSA) has not been sufficiently investigated. Methods: We aimed to investigate the trajectory of post-stroke depression and anxiety in our follow- up stroke clinic at 1, 3, and 6 months, and the association of RBC indices with these. One hundred and sixty-two patients with a new diagnosis of ischemic stroke were followed up at 1, 3, and 6 months, and underwent Patient Health Questionnaire-9 (PHQ-9) and the general anxiety disorder 7-item (GAD-7) questionnaire for evaluation of depression and anxiety, respectively. First, we used Kaplan-Meier analysis to investigate the accumulated incidences of post-stroke depression and post-stroke anxiety. Next, to explore the association of RBC indices with psychiatric disorders after an ischemic stroke attack, we adjusted for demographic and vascular risk factors using multivariate Cox regression analysis. Results: Of the 162 patients with new-onset of ischemic stroke, we found the accumulated incidence rates of PSD (1.2%, 17.9%, and 35.8%) and PSA (1.2%, 13.6%, and 15.4%) at 1, 3, and 6 months, respectively. The incident PSD and PSA increased 3 months after a stroke attack. Multivariate Cox regression analysis indicated independent positive associations between PSD risk and higher mean corpuscular volume (MCV) (OR=1.42, 95% CI=1.16-1.76), older age (OR=2.63, 95% CI=1.16-5.93), and a negative relationship between male sex (OR=0.95, 95% CI=0.91-0.99) and PSA. Conclusion: The risks of PSD and PSA increased substantially 3 months beyond stroke onset. Of the RBC indices, higher MCV, showed an independent positive association with PSD.


Materials ◽  
2021 ◽  
Vol 14 (2) ◽  
pp. 305
Author(s):  
Chung-Min Kang ◽  
Saemi Seong ◽  
Je Seon Song ◽  
Yooseok Shin

The use of hydraulic silicate cements (HSCs) for vital pulp therapy has been found to release calcium and hydroxyl ions promoting pulp tissue healing and mineralized tissue formation. The present study investigated whether HSCs such as mineral trioxide aggregate (MTA) affect their biological and antimicrobial properties when used as long-term pulp protection materials. The effect of variables on treatment outcomes of three HSCs (ProRoot MTA, OrthoMTA, and RetroMTA) was evaluated clinically and radiographically over a 48–78 month follow-up period. Survival analysis was performed using Kaplan–Meier survival curves. Fisher’s exact test and Cox regression analysis were used to determine hazard ratios of clinical variables. The overall success rate of MTA partial pulpotomy was 89.3%; Cumulative success rates of the three HSCs were not statistically different when analyzed by Cox proportional hazard regression analysis. None of the investigated clinical variables affected success rates significantly. These HSCs showed favorable biocompatibility and antimicrobial properties in partial pulpotomy of permanent teeth in long-term follow-up, with no statistical differences between clinical factors.


2021 ◽  
pp. 019459982199338
Author(s):  
Flora Yan ◽  
Dylan A. Levy ◽  
Chun-Che Wen ◽  
Cathy L. Melvin ◽  
Marvella E. Ford ◽  
...  

Objective To assess the impact of rural-urban residence on children with obstructive sleep-disordered breathing (SDB) who were candidates for tonsillectomy with or without adenoidectomy (TA). Study Design Retrospective cohort study. Setting Tertiary children’s hospital. Methods A cohort of otherwise healthy children aged 2 to 18 years with a diagnosis of obstructive SDB between April 2016 and December 2018 who were recommended TA were included. Rural-urban designation was defined by ZIP code approximation of rural-urban commuting area codes. The main outcome was association of rurality with time to TA and loss to follow-up using Cox and logistic regression analyses. Results In total, 213 patients were included (mean age 6 ± 2.9 years, 117 [55%] male, 69 [32%] rural dwelling). Rural-dwelling children were more often insured by Medicaid than private insurance ( P < .001) and had a median driving distance of 74.8 vs 16.8 miles ( P < .001) compared to urban-dwelling patients. The majority (94.9%) eventually underwent recommended TA once evaluated by an otolaryngologist. Multivariable logistic regression analysis did not reveal any significant predictors for loss to follow-up in receiving TA. Cox regression analysis that adjusted for age, sex, insurance, and race showed that rural-dwelling patients had a 30% reduction in receipt of TA over time as compared to urban-dwelling patients (hazard ratio, 0.7; 95% CI, 0.50-0.99). Conclusion Rural-dwelling patients experienced longer wait times and driving distance to TA. This study suggests that rurality should be considered a potential barrier to surgical intervention and highlights the need to further investigate geographic access as an important determinant of care in pediatric SDB.


2011 ◽  
Vol 16 (3) ◽  
pp. 411-414 ◽  
Author(s):  
Kazunari Kaneko ◽  
Takahisa Kimata ◽  
Shoji Tsuji ◽  
Tomohiko Shimo ◽  
Masaya Takahashi ◽  
...  

Author(s):  
YOJANA Gokhale ◽  
Rakshita Mehta ◽  
Uday Kulkarni ◽  
Nitin Karnik ◽  
Sushant Gokhale B.Tech ◽  
...  

Abstract Background: Cytokine storm triggered by Severe Corona Virus Disease 2019 (COVID-19) is associated with high mortality. With high ‘Interlukin -6’ (IL-6) levels reported in COVID-19 deaths in China1, IL-6 is considered to be the key player in COVID-19 cytokine storm. Tocilizumab, a monoclonal antibody against IL-6 receptor, is used on compassionate grounds for treatment of COVID-19 cytokine storm. Aim of this study was to assess effect of tocilizumab on mortality due to COVID-19 cytokine storm.Method: This retrospective, observational study included patients of severe COVID-19 pneumonia with persistent hypoxia (defined as saturation 94% or less on supplemental Oxygen of 15 L per minute through non-rebreathing mask or PaO2/FiO2 ratio of less than 200) who were admitted to tertiary care center in Mumbai, India, between 31st March to 5th July 2020. In addition to standard care, single Inj. Tocilizumab 400mg was given intravenously to 151 consecutive COVID-19 patients with persistent hypoxia from 13th May to 5th July 2020. These 151 patients were retrospectively analysed and compared with historic controls i.e consecutive COVID-19 patients with persistent hypoxia, defined as above (N=118, from our first COVID-19 admission on 31st March to 12th May 2020 ie till tocilizumab was available in hospital). Univariate and multivariate Cox regression analysis was performed for identifying predictors of survival. Statistical analysis was performed using IBM SPSS version 26.Results: On multivariate Cox regression analysis, independent predictors of survival were use of tocilizumab (HR 0.621, 95% CI 0.427-0.903, P 0.013) and higher oxygen saturation.Conclusion: Tocilizumab improved survival in severe COVID-19 pneumonia with persistent hypoxia


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Nora Krau ◽  
Sandra Freitag-Wolf ◽  
Doreen Brehm ◽  
Rainer Petzina ◽  
Georg Lutter ◽  
...  

Background: GDF15 belongs to the transforming growth factor superfamily and has a significant role in regulating inflammatory and apoptotic pathways. GDF15 is an emerging biomarker for risk stratification in cardiovascular disease. Here we analyze its prognostic value in patients with severe symptomatic aortic valve stenosis undergoing transcatheter aortic valve implantation (TAVI). Methods and Results: We prospectively enrolled 217 patients undergoing TAVI (using Edwards Sapien XT prostheses) at our institution over a continuous period of 35 month (2/2011-12/2013). All patients were available for complete follow up. Clinical parameters were determined before the procedure, biomarkers (GDF15 & NTproBNP) were measured before, 3 and 7 days after TAVI. The primary endpoint was survival time, all available prognostic factors were studied by Cox regression analysis with backward selection based on the likelihood ratio criteria. At median follow-up of 349 d (Q1-Q3 106-660d), a total of n=66 deaths occurred. 30d mortality was 6.9%. Mean age was 81.8 years (± 6.0 y) and 55.8% were females. Mean log. Euroscore (ES) was 25.4% (± 17.2%). Median preprocedural GDF15 values were 2256 pg/ml (Q1-Q3 1585.5-3082.0). In univariate analyses, increased GDF15 levels (upper quartile compared to lower three quartiles) revealed a HR of 2.4 (CI 1.5-3.9, p<0.001) for adverse outcome. In addition, also log. ES (p= 0.001), log. ES II (p=0.018), STS-Score (p=0.019), NTproBNP (p=0.037) and atrial fibrillation (p=0.02) demonstrated statistical significance for negative outcome. A multivariate Cox regression analysis including these factors and postprocedural aortic regurgitation, demonstrated that elevated GDF15 had a HR of 2.104 (CI 1.3-3.5; p=0.003) for negative outcome in patients undergoing TAVI, while elevated NTproBNP had HR of 1.412 (CI 0.8-2.4; p=0.212). Moreover, this analysis also revealed the log. ES as an independent risk factor (HR of 2.211, CI 1.3-3.7; p= 0.002). Conclusion: Increased GDF15 levels are associated with a poor prognosis in patients undergoing TAVI. Furthermore, GDF15 showed to be superior to the established biomarker NTproBNP in risk stratification of patients undergoing TAVI providing additional prognostic information.


2020 ◽  
Author(s):  
Ning Ma ◽  
Xin Feng ◽  
Zhongxue Wu ◽  
Daming Wang ◽  
Aihua Liu

Abstract Background Aneurysmal subarachnoid hemorrhage (SAH) is a kind of destructive cerebrovascular disease which could affect people's cognition, even the life expectancy. People with SAH are considered in a fatal situation, especially in the young population. This study aimed to investigate cognitive impairment and related factors in young patients with ruptured anterior communicating artery (ACoA) aneurysms.Methods We conducted a multicentre retrospective follow-up study at three hospitals in China. The young patients (18-50 years) who underwent ruptured ACoA aneurysm treatment by microsurgical clipping or endovascular coiling at three academic institutions in China from January 2015 to November 2017 were recruited. Patient cognition and life quality were assessed by using modified Telephone Interview for Cognitive Status (TICS-m), the modified Rankin Scale (mRS), and the instrumental activities of daily living (IADL) scale 2. Multiple cox-regression analysis was used to identify variables independently associated with cognitive impairment.Results Of the total of 59 patients, 54 (91.5%) achieved good clinical outcomes (mRS score 0-2) and 51 (86.4%) had excellent quality of life (IADL score 8). Ten (16.9%) patients showed cognitive impairments (TICS-m<27). The multivariate COX regression analysis showed that mRS scores of 3-5 at discharge, female sex, and aneurysm size <5 mm was independently associated with cognitive impairment. TICS-m scores at the latest follow-up were similar after open surgery and coiling. Conclusion In this relatively young sample that excluded patients with very poor-grade SAH or serious complications, microsurgical clipping led to better clinical outcomes than endovascular coiling, while cognitive outcomes were similar across treatment modalities. These results are not completely consistent with previous studies, and should therefore be considered in the clinical practice as well as further investigated in larger patient samples.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M I Gonzalez Del Hoyo ◽  
G Cediel ◽  
A Carrasquer ◽  
G Bonet ◽  
K Vasquez-Nunez ◽  
...  

Abstract Background CHA2DS2-VASc score has been used as a surrogate marker for predicting outcomes beyond thromboembolic risk in patients with atrial fibrillation (AF). Likewise, cardiac troponin I (cTnI) is a predictor of mortality in AF. Purpose This study aimed to investigate the association of cTnI and CHA2DS2-VASc score with long-term prognosis in patients admitted to the emergency department with AF. Methods A retrospective cohort study conducted between January 2012 and December 2013, enrolling patients admitted to the emergency department with AF and having documented cTnI measurements. CHA2DS2-VASc score was estimated. Primary endpoint was 5-year all-cause mortality, readmission for heart failure (HF), readmission for myocardial infarction (MI) and the composite end point of major adverse cardiac events defined as death, readmission for HF or readmission for MI (MACE). Results A total of 578 patients with AF were studied, of whom 252 patients had elevated levels of cTnI (43.6%) and 334 patients had CHA2DS2-VASc score >3 (57.8%). Patients with elevated cTnI tended to be oldercompared with those who did not have cTnI elevation and were more frequently comorbid and of higher ischemic risk, including hypertension, prior MI, prior HF, chronic renal failure and peripheral artery disease. The overall median CHA2DS2-VASc score was higher in those with cTnI elevation compared to those patients elevated cTnI levels (4.2 vs 3.3 points, p<0.001). Main diagnoses at hospital discharge were tachyarrhythmia 30.3%, followed by heart failure 17.7%, respiratory infections 9.5% and acute coronary syndrome 7.3%. At 5-year follow-up, all-cause death was significantly higher for patients with cTnI elevation compared with those who did not have cTnI elevation (56.4% vs. 27%; logrank test p<0.001). Specifically, for readmissions for HF and readmissions for MI there were no differences in between patients with or without cTnI elevation. In addition, MACE was reached in 165 patients (65.5%) with cTnI elevation, compare to 126 patients (38.7%) without cTnI elevation (p<0.001). On multivariable Cox regression analysis, cTnI elevation was an independent predictor of all-cause death (hazard ratio, 1.67, 95% confidence interval [CI]: 1.24–2.26, p=0.001) and of MACE (hazard ratio 1.47, 95% confidence interval 1.15–1.88; P=0.002), but it did not reach statistical significance for readmissions for MI and readmissions for HF. CHA2DS2-VASc score was a predictor on univariate Cox regression analysis for each endpoint, but it did not reach significance on multivariable Cox regression analysis for any endpoint. Conclusions cTnI is independently associated with long-term all-cause mortality in patients attending the emergency department with AF. cTnI compared to CHA2DS2-VASc score is thus a biomarker with predictive capacity for mortality in late follow-up, conferring utility in the risk stratification of patients with atrial fibrillation.


2020 ◽  
Vol 5 (4) ◽  
pp. 368-373
Author(s):  
Shen Li ◽  
Kai Liu ◽  
Yuan Gao ◽  
Lu Zhao ◽  
Rui Zhang ◽  
...  

ObjectiveTo evaluate the prognosis values of systemic immune–inflammation index (SII) in non-chronic cerebral venous sinus thrombosis (CVST).Methodspatients with CVST, admitted to the First Affiliated Hospital of Zhengzhou University, were retrospectively identified from January 2013 to December 2018. We selected patients in acute/subacute phase from database. Functional outcomes of patients were evaluated with the modified Rankin Scale (mRS)—mRS 3–6 as poor outcomes and mRS 6 as death. The overall survival time was defined as the date of onset to the date of death or last follow-up date. Survival analysis was described by the Kaplan-Meier curve and Cox regression analysis. Multivariate logistic regression analysis assessed the relationship between SII and poor functional outcome. The area under the Receiver Operating Curve curve (AUC) was estimated to evaluate the ability of SII in prediction.ResultsA total of 270 patients were included and their duration of follow-up was 22 months (6–66 months), of whom 31 patients had poor outcomes and 24 patients dead. Cox regression analysis showed that SII (HR=1.304, 95% CI: 1.101 to 1.703, p=0.001) was a predictor of death in non-chronic CVST. Patients with higher SII presented lower survival rates (p=0.003). The AUC of SII was 0.792 (95% CI: 0.695 to 0.888, p=0.040) with a sensitivity of 69.6% and specificity of 80.1%. Subgroups analysis demonstrated that SII was an important predictor of poor outcomes in male (OR=1.303, 95% CI: 1.102 to 1.501, p=0.011) and pregnancy/puerperium female (OR=1.407, 95% CI: 1.204 to 1.703, p=0.034).ConclusionsSII was a potential predictor in the poor prognosis of patients with acute/subacute CVST, especially in male and pregnancy/puerperium female.


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