Objective: To investigate the genetic and genomic profiling of juvenile myelomonocytic leukemia (JMML) and factors affecting its survival rate. Methods: Clinical characteristics, cytogenetics, molecular biology results and survival status of children with 27 JMML cases admitted to the Hematology Department of Children's Hospital, Capital Institute of Pediatrics from December 2012 to December 2021 were analyzed retrospectively, and the outcomes of the children were followed up. Kaplan-Meier method was used for survival analysis. Univariate analysis was used for analyzing factors affecting the overall survival (OS) rates of patients who received hematopoietic stem cell transplantation (HSCT). Log-Rank test was used for comparison of survival curves. Results: Among 27 JMML cases, there were 11 males and 16 females. The age of disease onset was 28 (11,52) months. There are 20 cases of normal karyotype, 4 cases of monosomy 7, 1 case of trisomy 8,1 case of 11q23 rearrangement and 1 case of complex karyotype. A total of 39 somatic mutations were detected.Those involved in RAS signal pathway were the highest (64%(25/39)), among which PTPN11 mutation was the most frequent (44% (11/25)). A total of 17 cases (63%) received HSCT, 8 cases (30%) did not receive HSCT, and 2 cases (7%) lost follow-up. For children receiving transplantation, the follow-up time after transplantation was 47 (11,57) months. The 1-year OS rate of high-risk transplantation group (17 cases) and high-risk non transplantation group (6 cases) was (88±8)% and (50±20)% respectively, with a statistically significant difference (χ2=5.01, P=0.025). The 5-year OS rate of the high-risk transplantation group was (75±11)%. The survival time of those who relapsed or progressed to acute myeloid leukemia after transplantation was significantly shorter than that of those who did not relapse (χ2=6.80, P=0.009). The OS rate of patients with or without PTPN11 mutation was (81±12) % and (67±19)% respectively (χ2=0.85, P=0.356). Conclusions: The main pathogenesis involved in JMML is gene mutation related to RAS signaling pathway, and the most common driver gene of mutation is PTPN11. Allogeneic HSCT can significantly improve the survival rate of high-risk JMML patients. The recurrence or progression after transplantation was related to poor prognosis.

Background Assess risk factors for early death in patients who underwent urgent-start peritoneal dialysis (USPD). Methods Patients who initiated USPD in five peritoneal dialysis centers from 2013 to 2019 were screened in this multicenter retrospective cohort study. Risk factors for all-cause mortality within 3 months were explored. Results A total of 1265 USPD patients with 43 early deaths were included. Cox regression analyses showed that age older than 60 years (hazard ratio [HR], 3.054; 95% CI [1.597, 5.842]; p = 0.001), albumin less than 30 g/L (HR, 2.234; 95%CI [1.207, 4.136]; p = 0.011), blood glucose greater than 7 mmol/L (HR, 2.766; 95%CI [1.477, 5.180]; p = 0.001), higher estimated glomerular filtration rate (eGFR; HR, 1.121; 95%CI [1.071, 1.172]; p = 0.000), and poor stages of heart failure (class IV compared with class 0-I; HR, 5.165; 95%CI [2.544, 10.486]; p = 0.000) were independent predicting factors for early death. Conclusions Risk factors for early death were older age, hypoproteinemia, hyperglycemia, higher eGFR, and severe heart failure.

Aim Peritoneal dialysis (PD)-associated peritonitis (PDAP) is a severe complication of PD. It is an important issue about whether it can be cured. At present, there is no available prediction model for peritonitis cure. Therefore, this study aimed to develop and validate a prediction model for peritonitis cure in patients with PDAP. Methods Patients with PD who developed PDAP from four dialysis centers in Northeast China were followed up. According to the region of PD, data were divided into training and validation datasets. Initially, a nomogram for peritonitis cure was established based on the training dataset. Later, the nomogram performance was assessed by discrimination (C-statistic), calibration, and decision curves. Results Totally, 1,011 episodes of peritonitis were included in the final analysis containing 765 in the training dataset and 246 in the validation dataset. During the follow-up period, peritonitis cure was reported in 615 cases from the training dataset and 198 from the validation dataset. Predictors incorporated in the final nomogram included PD duration, serum albumin, antibiotics prior to admission, white cell count in peritoneal dialysate on day 5 (/μl) ≥ 100/μl, and type of causative organisms. The C-statistic values were 0.756 (95% CI: 0.713–0.799) in the training dataset and 0.756 (95% CI: 0.681–0.831) in the validation dataset. The nomogram exhibited favorable performance in terms of calibration in both the training and validation datasets. Conclusion This study develops a practical and convenient nomogram for the prediction of peritonitis cure in patients with PDAP, which assists in clinical decision-making.

OBJECTIVE:To develop and validate a risk prediction model of treatment failure in patients with peritoneal dialysis-associated peritonitis (PDAP). METHODS:We retrospectively analyzed the data of patients undergoing peritoneal dialysis (PD) in 3 dialysis centers in Jilin Province who developed PDAP between January 1, 2013 and December 31, 2019. The data collected from the Second Hospital of Jilin University and Second Division of First Hospital of Jilin University) were used as the training dataset and those from Jilin Central Hospital as the validation dataset. We developed a nomogram for predicting treatment failure using a logistic regression model with backward elimination. The performance of the nomogram was assessed by analyzing the C-statistic and the calibration plots. We also plotted decision curves to evaluate the clinical efficacy of the nomogram. RESULTS:A total of 977 episodes of PDAP were included in the analysis (625 episodes in the training dataset and 352 episodes in the validation dataset). During follow-up, 78 treatment failures occurred in the training dataset and 35 in the validation dataset. A multivariable logistic regression prediction model was established, and the predictors in the final nomogram model included serum albumin, peritoneal dialysate white cell count on day 5, PD duration, and type of causative organisms. The nomogram showed a good performance in predicting treatment failure, with a C-statistic of 0.827 (95% CI: 0.784-0.871) in the training dataset and of 0.825 (95% CI: 0.743-0.908) in the validation dataset. The nomogram also performed well in calibration in both the training and validation datasets. CONCLUSION:The established nomogram has a good accuracy in estimating the risk of treatment failure in PDAP patients.

在我国,肾脏疾病的发病率逐年增高,与此同时,终末期肾病的治疗任务也越来越大,血液透析在肾脏替代治疗中扮演着重要的角色,但透析中低血压(intradialytic hypotension,IDH)的发生己成为影响透析患者预后的主要危险因素之一.IDH主要表现为:恶心、呕吐、腹部不适、心绞痛,严重时会危及患者的生命.因此,对于IDH的预防及治疗至关重要.本文系统分析了IDH的发生机制、高危因素、相关预防及治疗措施.通过对IDH进行充分的认识,积极采取干预措施,减少IDH的发生及其危害,提高患者的透析质量及延长生存时间.

原发性肾病综合征(PNS)是肾小球疾病的常见表现,临床上常将糖皮质激素作为一线治疗药物,多数患者需联合常规免疫抑制治疗,如环磷酰胺、钙调神经磷酸酶抑制剂等,即便如此,仍存在部分患者病情不能得到有效缓解.近年来利妥昔单抗作为一种新型治疗药物,逐渐被应用于难治性PNS的治疗,并取得良好的成效.本文就利妥昔单抗治疗PNS的机制、给药方案、在不同病理类型PNS中的应用及不良反应进行综述,以期为临床提供参考.

Aim Peritoneal dialysis (PD)-associated peritonitis (PDAP) is a severe complation of PD. And it is an important issue about whether it can be cured. At present, there is no available prediction model for peritonitis cure. Therefore, the present work aimed to develop and validate a risk prediction model for peritonitis cure in PDAP patients. Methods PD patients who developed PDAP from four dialysis centers in Northeast China were followed up. According to the region of PD, data were divided into training and validation datasets. First of all, a nomogram for peritonitis cure was established based on the training dataset. Later, performance of the nomogram model was assessed by discrimination (C-statistic), calibration and decision curves. Results In total, 1063 episodes of peritonitis were included in the final analysis, including 806 in the training dataset and 257 in the validation dataset. During the follow-up period, 621 and 199 cases in the training and validation datasets, respectively, reported peritonitis cure. Predictors incorporated in the final nomogram model included PD duration, serum albumin, antibiotics prior to admission, and type of causative organisms. The C-statistic values were 0.76 (95% CI: 0.72–0.80) in the training dataset and 0.77 (95% CI: 0.70–0.84) in the validation dataset. The nomogram exhibited favorable performance in terms of calibration in both the training and validation datasets. Conclusion This study develops a practical and convenient nomogram for the prediction of peritonitis cure in PDAP patients, which assists in the clinical decision-making.

Vascular access is the lifeline of hemodialysis patients. There are great differences in the establishment and use of vascular access in different countries and regions around the world. We believe that on the basis of good evaluation and planning, it is recommended that hemodialysis patients choose native arteriovenous fistula first. In view of the new progress of vascular access views domestic and international at home and abroad in recent years, we organized experts to recommend the establishment and maintenance of arteriovenous fistula (AVF) for the Chinese population, including preoperative evaluation and planning of the establishment of AVF, AVF surgery, perioperative drug intervention measures and postoperative maintenance, and put forward suggestions for future research directions. The recommendations in this consensus are general and clinicians need to make treatment decisions based on the actual situation.

患者,女,47岁,因“发现尿检异常3年,双下肢水肿1个月,加重1周”于2018-12-19收入我科.该患者3年前体检时查尿常规示尿蛋白阳性、潜血阳性,曾因乏力、心前区疼痛、视物模糊等症状就诊于我院血液内科、心血管内科及神经内科等多科室,未系统诊治,于2015-09-11就诊于我科门诊查尿常规示尿蛋白1+、潜血+-,ANA谱示抗dsDNA269 IU/ml、抗心磷脂抗体＞120 RU/ml、ANA筛查1∶100,免疫球蛋白及补体正常,诊断为“抗磷脂综合征”,给予阿司匹林等药物治疗.此后定期于我院门诊复查,尿蛋白及潜血均阳性,肝肾功能及血常规大致正常,抗心磷脂抗体(2016-03-02》 ＞ 120 U/ml.

终末期肾病是慢性肾脏病的终末期阶段,需要依靠血液净化或肾移植来维持患者生命.腹膜透析(peritoneal dialysis,PD)作为血液净化的一种方式,具有操作简便、居家透析、保护残余肾功能等优势.尽管如此,腹膜透析患者的死亡率仍然很高,心血管事件的发生是PD患者主要的死亡原因.本文主要从红细胞分布宽度、血清总胆红素、血尿酸、天冬氨酸转氨酶/丙氨酸转氨酶比值、可溶性人基质裂解素2这几个方面与腹膜透析发生心血管事件的相关性作一综述,以期为临床提供更多参考.

Hypertensive renal damage refers to renal structural and functional damage caused by essential hypertension.Hypertension and renal damage are causal to each other and promote the development of pathological changes.Endothelin (ET) system can regulate blood pressure and renal blood flow through various mechanisms,which is closely related to the occurrence and development of hypertensive renal damage.Endothelin receptors antagonists (ERAs) can block the effect of endothelin by acting on endothelin receptors (ETRs),thereby lowering blood pressure,improving renal function,and delaying the progression of hypertensive renal damage.

Objective To observe the effect of Shengmai decoction combined with ginseng on hemodialysis-related hypotension. Methods From January 2017 to October 2017, 23 cases of hypotension occurred during hemodialysis at the center of hemodialysis in our hospital were treated with Shengmai decoction combined with American ginseng and red ginseng.Results The treatment of oral Shengmai decoction combined treatment for chronic renal disease maintenance hemodialysis patients can improve the blood pressure during dialysis, reduce the incidence of dialysis related hypotension.Conclusion Shengmai decoction combined with ginseng can effectively reduce the incidence of dialysis related hypotension.

随着接受腹膜透析的终末期肾病(ESRD) 患者逐年增加,腹膜透析(peritoneal dialysis,PD) 已成为重要肾脏替代治疗手段之一,腹膜透析相关并发症也逐渐引起我们的注意.腹膜透析相关性胸腹瘘是腹膜透析较少见并发症.腹膜透析相关性胸腹瘘治疗方式多样,其中间断性腹膜透析(intermittent peritoneal dialysis, IPD) 的治疗方法简单有效、副作用小,为引起更多医护工作者的重视,本文报道我院IPD治愈腹膜透析相关性胸腹瘘患者一例.

系统性红斑狼疮（SLE）是慢性自身免疫性疾病,表现为免疫耐受功能异常,T、B、NK细胞功能障碍及细胞炎症因子异常激活,造成体内多系统多器官自身免疫反应,出现大量自身抗体和免疫复合物难以被清除 [1] ,若累积中枢神经系统称神经精神狼疮又称狼疮性脑病（NPSLE）。狼疮性脑病的临床表现最常见的是癫痫和轻微精神异常,是患者最常见的死亡原因之一,多发生于SLE的活动期,发生于症状缓解阶

由于肾功能衰竭及生理方面变化的复杂性,CKD合并妊娠的可能性小,并且严重影响孕妇和胎儿的健康,因此,许多学者主张避孕或及早终止妊娠[1].终末期肾病患者成功妊娠分娩的报道不多,我院2016年接诊了一名妊娠合并维持性血液透析并成功分娩的患者,现在报告如下.

Acute kidney injury (AKI) threatens the neonates and small infants' life. Peritoneal dialysis (PD) is the first choice of renal replacement therapy for neonates and small infants with AKI. Because the abdominal wall structure development of neonate or small infant is not mature, the requirements of the operator's technical level are relatively high. Literature in this area is scarce, especially in China. In this study, we report the application of PD in AKI secondary to severe fungal septicemia in a 41-day-old premature-birth infant from China. Furthermore, we briefly review rare existing case reports documenting the application of PD in neonates and small infants with AKI.

Objective To explore the erythropoietin(EPO) dosage for improvement of renal anemia by computerized decision support(CDS), because of anemia management in hemodialysis patients poses significant challenges. Method A total of 564 patients with chronic kidney disease fifth phase who received EPO treatment at the Second Hospital of Jilin University Dialysis Units at least 3-month from October 2013 to April 2014 were enrolled, which included 296 males and 268 females, aged 22-83 years old with mean age of 43.21 years old. All of them were divided into manual group(n = 493) and CDS group(n = 71), The EPO dosage was adjusted by dry weight and hematocrit changes of patients, then hemoglobin changes of patients were monitored and observed. At last counted and analyzed data of 2 groups. Results The age of patients were mainly on the middle-aged group(45-59 years old), diabetes was the main cause of end-stage renal disease(ESRD). The changes of anemia related biochemical indexes were no significant difference between 2 groups. After adjustment for differences in center and baseline, average weekly EPO dosage in CDS group decreased 4 % than that of manual group(rate 0.96; 95 % confidence interval 0.77-1.18), the difference was no statistical significant(P>0.05). The monitoring hemoglobin changes of 2 groups was similar(σ2 = 1.30). The adjusted odds and unadjusted odds for hemoglobin value of 2 groups were analyzed, then the results showed that the mean hemoglobin value in manual group was 118[standard error(SE) 2 g/L], which was lower 1.1(SE, 0.4) g/L than that of CDS group(P < 0.001). But after adjustment for differences in baseline characteristics of 2 groups, CDS group hemoglobin value was lower 1.4(SE, 0.5) g/L than that of manual group. Analyzed linear mixed model concluded that there was the trend increased to 100 - 120 g/L and 110 - 120 g/L in CDS group, the EPO dosage was lower 4 % than that of CDS group, and there was no statistically significant(P>0.05). Conclusion It is demonstrated that comparison of 2 kinds of EPO, the monthly Hb in 2 group patients is 100 - 120 g/L, the treatment effect is no significant difference between 2 groups ,but EPO dosage in CDS group is lower than that of manual group.

Catheter malfunction caused by migration of the catheter tip is an ongoing challenge in patients on continuous ambulatory peritoneal dialysis. Reimplantation of a new catheter by open operation exposes the patient to additional hazards and costs. Non-operative treatment such as manual reduction, appropriate exercises and purgative enema tend to have a poor success rate. In this study, we report our experience with six patients on continuous ambulatory peritoneal dialysis in whom the migrated catheter tip was restored to the pelvic cavity with use of a processed gastroscopic brush.

To investigate the clinical and histopathological features of non-diabetic renal disease (NDRD) superimposed on diabetic nephropathy (DN) in northeastern Chinese patients with type 2 diabetes mellitus (T2D), and compare the changes with those of pure DN and isolated NDRD.