Background:Fear of progression (FoP) is one of the most prevalent psychological responses among patients with chronic kidney disease (CKD), impairing mental health and quality of life. However, research on the heterogeneity of illness perceptions among CKD patients remains limited, and the relationship between distinct perception profiles and FoP is not yet well understood. This study aimed to identify latent profiles of illness perception in CKD patients and examine their associations with FoP. Methods:In this cross-sectional study, a total of 428 CKD patients were recruited from four tertiary hospitals through convenience sampling between May 1, 2023, and April 30, 2024. Participants completed questionnaires assessing demographic and clinical characteristics, illness perception, FoP, psychological flexibility, family resilience, and social support. Latent profile analysis was conducted to identify illness perception profiles, multinomial logistic regression was used to examine predictors of profile membership, and stratified regression analyses were performed to assess the associations between illness perception profiles and FoP. Results:Latent profile analysis identified three distinct illness perception profiles. Multinomial logistic regression analysis indicated that CKD stage, number of concomitant symptoms, psychological flexibility, family resilience, and social support significantly predicted the latent profiles of illness perception (P < 0.05). Stratified regression analysis further demonstrated that illness perception profiles were independently predicted FoP after controlling for key demographic and clinical variables. Conclusion:CKD patients exhibit heterogeneity in illness perception. Illness perception profiles were associated with FoP. Distinct illness perception profiles are associated with FoP, and are influenced by key psychosocial factors. These findings support the development of tailored, perception-based interventions to reduce FoP among CKD patients.

BACKGROUND:The main treatment method for end-stage renal disease (ESRD) is maintenance hemodialysis (MHD). With the continuous improvement of dialysis technology, the survival period of MHD patients has been effectively prolonged, but dialysis technology still cannot completely replace renal function.OBJECTIVE:To study the dietary compliance and its correlation with thirst in MHD patients and to provide guidance for clinical development of corresponding intervention countermeasures.METHODS:A total of 90 patients who received MHD treatment from March 2021 to March 2022 were selected as objects. The Renal Adherence Attitudes Questionnaire (RAAQ) and the Renal Adherence Behaviour Questionnaire (RABQ) were used to analyze the dietary compliance and thirst status of patients. Pearson correlation analysis was used to analyze the correlation between diet compliance and thirst.RESULTS:Positive correlations were found between VAS and DTI, SXI and TDS (P< 0.05). Social restrictive attitude was positively correlated with VAS, DTI, SXI, TDS, acceptance attitude and compliance in facing difficulties (P< 0.05), and negatively correlated with self-care compliance (r=-0.35, P< 0.05). Health attitude was positively correlated with VAS, DTI and SXI (P< 0.05). Acceptance attitude was positively correlated with DTI, SXI and TDS (P< 0.05). High RAAQ was associated with high VAS (b= 0.11, 95% CI: 0.05, 0.18), DTI (b= 0.28, 95% CI: 0.17, 0.38), SXI (b= 0.24, 95% CI: 0.14, 0.34) and TDS (b= 0.26, 95% CI: 0.13, 0.4).CONCLUSION:The overall performance of dietary compliance in patients with MHD is at a moderate level, and dietary compliance is negatively correlated with disease perception.

This study focuses on the application of nurse-led multidisciplinary collaborative therapy (MDT) management model for calciphylaxis prevention of patients with terminal renal disease. Through the establishment of a multidisciplinary management team spanning nephrology department, blood purification center, dermatology department, burn and plastic surgery department, infection department, stem cell platform, nutrition department, pain department, cardiology department, hydrotherapy group, dermatology group, and outpatient treatment room, the distribution of duties among team members were clarified to bring out the best advantages of a multidisciplinary teamwork during treatment and nursing. For patients with calciphylaxis symptoms in terminal renal disease, a case-by-case management model was carried out with the focus on personalised problem. We emphasised on personalised wound care, precise medication care, active pain management, psychological intervention and palliative care, the amelioration of calcium and phosphorus metabolism disorder, nutritional supplementation, and the therapeutic intervention based on human amniotic mesenchymal stem cell regeneration. The MDT model effectively compensates for traditional nursing mode and could serve as a novel clinical management modality for calciphylaxis prevention in patients with terminal renal disease.

海蓝组织细胞增多症(sea blue histiocytosis,SBH)是由于机体脂质代谢紊乱而导致的组织细胞内神经鞘磷脂和脑磷脂过度堆积的一种罕见的代谢性疾病,其发病机制不清,目前临床分为原发性SBH和继发性SBH,其中原发性SBH十分罕见,临床以继发性SBH为主[1],血液性疾病及脂质代谢紊乱相关性疾病是继发SBH的主要原因[2],同时增殖的海蓝组织细胞也可浸润机体各脏器引起其功能异常,促进疾病发展.2021年10月2日南昌大学第一附属医院收治1例以急性肝衰竭为首发症状的慢性粒细胞白血病(CML)伴SBH患者,现对其临床资料进行回顾性分析,报告如下.

舞蹈病-棘红细胞增多症(chorea-acanthocyto-sis,ChAc)是一种罕见的常染色体隐性遗传病,其临床特征较为复杂,可累及运动、神经、精神及内分泌等多个系统,常伴有头颈部不自主运动、进行性舞蹈样运动障碍、认知能力下降等临床表现,极易与麦克劳德综合征、类亨廷顿病2型、泛酸激酶相关的神经退行性等疾病相混淆[1];液泡蛋白分类同源物13A(vacuolar protein sorting homolog 13A,VPS13A)基因编码蛋白chorein对于维持细胞膜正常结构及神经元细胞功能具有重要作用,其突变与CHAc发病相关.现将南昌大学第一附属医院诊治的1例VPS13A基因双位点纯合突变致ChAc患者报告如下.

Abstract Calciphylaxis is a rare cutaneous vascular disease that manifests with intolerable pains, non-healing skin wounds, histologically characterized by calcification, fibrointimal hyperplasia, and microvessel thrombosis. Currently, there are no standardized guidelines for this disease. Recent studies have recognized a high prevalence of thrombophilias and hypercoagulable conditions in calciphylaxis patients. Here, we report a case of uremic calciphylaxis patient whom was refractory to conventional treatments and then received a salvage strategy with intravenous and local hAMSC application. In order to investigate the therapeutic mechanism of hAMSCs from the novel perspective of hypercoagulability, coagulation-related indicators, wound status, quality of life and skin biopsy were followed up. Polymerase chain reaction (PCR) was performed to determine the distribution of hAMSCs in multiple tissues including lung, kidney and muscle after infusion of hAMSCs for 24 h, 1 week and 1 month in mice aiming to investigate whether hAMSCs retain locally active roles after intravenous administration. Improvement of hypercoagulable condition involving correction of platelet, D-dimer and plasminogen levels, skin regeneration and pain alleviation were revealed after hAMSC administration over one-year period. Skin biopsy pathology suggested regenerative tissues after 1 month hAMSC application and full epidermal regeneration after 20 months hAMSC treatment. PCR analysis indicated that hAMSCs were homing in lung, kidney and muscle tissues of mice even until tail vein injection of hAMSCs for 1 month. We propose that hypercoagulability is a promising therapeutic target of calciphylaxis patients, which can be effectively improved by hAMSC treatment.

目的:调查江苏省腹膜透析患者随访管理现状.方法:采用便利抽样法,于2021年8月调查江苏省13个地级市40家医院,采用自制问卷调查透析中心规模、随访方式、随访人员、随访频率、随访内容等.结果:40家医院共有腹膜透析随访患者6 479人,随访方式多样.医院门诊随访频率基本符合《腹膜透析标准操作规程》要求.负责随访的护理人员包括专职腹膜透析护士 48名和兼职腹膜透析护士 108名.各家医院随访内容均有差别,但透析相关内容、并发症预警是关注重点;对生活质量、社会支持相关内容关注度低.结论:江苏省40家医院腹膜透析中心人员配置不足,部分医院随访内容略有缺失,建议逐步改善人员配置情况,总结腹膜透析患者居家透析过程中的问题及相应重点随访的内容并形成固定模块,为随访者及时获取核心、必需的随访指标结果提供依据.

Calciphylaxis is a rare disease with severe pain and high-mortality due to cutaneous ischemic necrosis and infection that currently lacks proved effective therapies. The occurrence of calciphylaxis in end stage kidney disease (ESKD) patients is known as calcific uremic arteriolopathy (CUA), which is characterized histologically by dermal microvessel calcification, intimal fibroplasia and microthrombosis. Here we innovatively treated a severe CUA patient with human amnion-derived mesenchymal stem cells (hAMSCs). A 34-year-old uremic woman was presented with progressive, painful malodorous ulcers in buttocks and mummified lower limbs. Skin pathological features supported the diagnosis of calciphylaxis. The patient was refractory to conventional multidisciplinary symptomatic therapies. With the approval of our hospital ethics committee, she was treated with hAMSCs including intravenous and local intramuscular injection, and external application of hAMSC culture supernatant to the wound area. During 15-month follow-up, the patient had regeneration of skin and soft tissues, with improved blood biochemical, inflammatory, mineral and bone metabolic indices and immunoregulation effects. After 15-month hAMSC treatment, the score of pain visual analog scale (VAS) decreased from 10 to 0, Bates-Jensen wound assessment tool (BWAT) score decreased from 65 to 13, and wound-quality of life (Wound-QoL) questionnaire score decreased from 68 to 0. We propose that hAMSC treatment is promising for CUA patients. The therapy is potentially involved in the multiple beneficial effects of inhibiting vascular calcification, stimulating angiogenesis and myogenesis, modulating adverse inflammatory and immunologic responses, promoting re-epithelialization and restoring skin integrity.

Calciphylaxis is a rare disease characterized histologically by microvessel calcification and microthrombosis, with high mortality and no proven therapy. Here, we reported multidisciplinary regenerative treatment for rescuing a severe uremic calciphylaxis patient with human amnion-derived mesenchymal stem cells (hAMSCs). A 34-year-old uremic woman presented progressive skin ischaemia, large areas of painful malodorous ulcers and mummified legs. The patient was diagnosed as calciphylaxis based on clinical manifestations, laboratory examinations and skin pathological features. Because of her refractory to conventional therapy, treatment with hAMSCs was approved. HAMSCs were administered intravenously to the patient at a dose of 1.0 × 106 cells per kilogram of body weight, local intramuscular injection along the wound edge (2.0 × 104 cells/cm2) and external application of the cell culture supernatant on wound surfaces. Following up 15 months regularly after hAMSCs treatment, the patient's blood biochemical, inflammatory, mineral and bone metabolic indices improved significantly, with immunoregulation effects, regeneration of skin and soft tissue. Besides, wound healing (Figure 1), pain status and quality of life, evaluated by Pain Visual Analog Scale, Bates-Jensen Wound Assessment and the wound-quality of life questionnaire were also improved significantly (Figure 2). This case represented the new method of hAMSC regeneration therapy for uremic calciphylaxis with safety and effectiveness, which deserved further investigation. Before hAMSC treatment, the patient had irregular ulcers on the buttocks and lower extremities, some of which were crusted on the surface or had purulent fishy discharge. Skin and soft tissue necrosis can be seen at the ulcerated area of the buttocks, localized deep to the fascia, with purulent secretions on the surface and brown pigmentation of the skin at the edge of the ulcer (Figure 1A and B). Because of her worsening symptoms and signs refractory to conventional therapy, the patient was given intravenous and local intramuscular injections of hAMSCs, supplemented by application of hAMSC culture supernatant to the wound. After 4 months of treatment, the sacrococcygeal wound has reduced size and become shallow with less exudate, some new granulation tissue was visible. The infection was controlled with pain significantly reduced (Figure 1C). After 12 months, most of the sacrococcygeal wound had healed with only a few erosions (Figure1D). Fifteen months later, her skin scarring of the wound was healed without pain (Figure 1E). The pain Visual Analogue Scale (Figure 2A) showed that the pain was unbearable before treatment (10 points) and disappeared after 15 months of hAMSC treatment (0 point). BWAT (Figure 2B) showed that all the 13 indexes got 5 points (worst) before hAMSC treatment, with a total score of 65 points. After 15 months of hAMSC treatment, each index got 1 point (best), and the total score was 13 points. The Wound-QoL questionnaire (Figure 2C) showed that the 17 items were quantified as 4 points (very serious) before treatment, with a total score of 68 points. After 15 months of hAMSC treatment, the score was 0 (none at all), and the total score was 0.

Calciphylaxis is a rare disease characterized histologically by microvessel calcification and microthrombosis, with high mortality and no proven therapy. Here, we reported a severe uremic calciphylaxis patient with progressive skin ischemia, large areas of painful malodorous ulcers, and mummified legs. Because of the worsening symptoms and signs refractory to conventional therapies, treatment with human amnion-derived mesenchymal stem cells (hAMSCs) was approved. Preclinical release inspections of hAMSCs, efficacy, and safety assessment, including cytokine secretory ability, immunocompetence, tumorigenicity, and genetics analysis in vitro, were introduced. We further performed acute and long-term hAMSC toxicity evaluations in C57BL/6 mice and rats, abnormal immune response tests in C57BL/6 mice, and tumorigenicity tests in neonatal Balbc-nu nude mice. After the preclinical research, the patient was treated with hAMSCs by intravenous and local intramuscular injection and external supernatant application to the ulcers. When followed up to 15 months, the blood-based markers of bone and mineral metabolism improved, with skin soft tissue regeneration and a more favorable profile of peripheral blood mononuclear cells. Skin biopsy after 1-month treatment showed vascular regeneration with mature noncalcified vessels within the dermis, and 20 months later, the re-epithelialization restored the integrity of the damaged site. No infusion or local treatment-related adverse events occurred. Thus, this novel long-term intravenous combined with local treatment with hAMSCs warrants further investigation as a potential regenerative treatment for uremic calciphylaxis due to effects of inhibiting vascular calcification, stimulating angiogenesis and myogenesis, anti-inflammatory and immune modulation, multidifferentiation, re-epithelialization, and restoration of integrity.

髓系肉瘤(MS )是一种由原始或幼稚髓系细胞浸润骨髓以外组织器官而形成的肿瘤团块[1 ] ,根据瘤细胞浸润的性质分为白血病性 MS、原发性MS和孤立性 MS (IMS )[2] ,IMS 临床上极为罕见,占 MS 的1％ ～2％[3 ] ,受累的部位以淋巴结、皮肤、骨骼、睾丸及中枢神经系统等为主[4] ,由于IMS患者肿瘤细胞未浸润骨髓及外周血,仅引起局部组织器官功能异常,因而极易造成临床误诊.

目的 探讨肾内科规范化培训带教中引入"非暴力沟通"模式对规培期护士沟通能力及双向满意度的影响.方法 选择本科2017年—2019年共6名规培期护士为研究对象,对护士实施"非暴力沟通"四要素应用的培训和实践.结果 规范化培训前后,对规培期护士均进行临床沟通能力、双向满意度测评,结果显示规范化培训后的得分较培训前有了显著提升,差异有统计学意义(P<0.05).结论 将"非暴力沟通"模式应用于肾内科规培期护士临床带教中,能提高护士沟通能力和双向满意度,值得进一步推广.

噬血细胞综合征(hemophagocytic syndrome,HPS)是由多种因素引起的淋巴细胞、组织细胞非恶性过度活化、增殖,并分泌大量炎性因子,进而引起机体一种严重的炎症反应,临床分为原发性HPS 和获得性HPS [1].原发性HPS 常见于儿童,继发性HPS 以成人居多.感染、风湿性疾病、肿瘤等是引起继发性HPS 常见原因.

弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)是一种常见的侵袭性较强的淋巴类恶性肿瘤,约占非霍奇金淋巴瘤(non-Hodgkin's lymphoma,NHL)的30％～40％[1];浆细胞分化是指成熟活化的B淋巴细胞在各种因素的刺激下向浆细胞分化发育的过程,常为多克隆分化.DLBCL伴单克隆性浆细胞分化较为罕见[2].本研究就南昌大学第一附属医院收治的1例DLBCL伴克隆性浆细胞分化患者进行介绍,以提高实验室人员对此类病例的认识.

目的 探讨不同α缺失型地中海贫血患者红细胞各参数特点.方法 应用PCR-膜反向点杂交技术(reversed dot blot,RDB)、跨越断裂点PCR技术(Gap-PCR)检测α、β地中海贫血基因,全自动血细胞分析仪检测红细胞(RBC)、血红蛋白(Hb)、红细胞平均体积(MCV)、平均红细胞血红蛋白含量(MCH)、平均红细胞血红蛋白浓度(MCHC)、红细胞分布宽度(RDW-CV),运用SPSS19.0系统统计分析试验组与健康对照组以及--SEA/、-α4.2/、-α3.7/、-α3.7/--SEA&-α4.2/--SEA各组之间红细胞各参数是否有统计学意义.结果 ⑴与健康对照组相比,α缺失型地中海贫血组Hb、MCV、MCH、MCHC明显减低,RDW-CV明显增高,差异有统计学意义(P<0.01);⑵以α4.2/、-α3.7/两组相比,--SEA/组MCV、MCH、MCHC三指标明显下降,且-α3.7/--SEA&-α4.2/--SEA组结果下降更为明显,均具有统计学意义(P<0.01);RDW-CV各组间无统计学意义.结论 α缺失型地中海贫血患者大部分红细胞指标可出现明显变化,且这种变化与缺失片段长度密切相关.

患者,男,57岁,因无明显诱因出现左下肢肿痛,右侧大腿瘀斑2周于2017年11月14日入院.2017年6月左下肢外伤后出现大量瘀斑,伴肿胀,当地医院医对症治疗后有所好转,2017年10月因病情复发并进展,伴有轻度胸闷(活动后症状加剧) ,遂来我院就诊.既往有胃溃疡出血病史18年,高血压史2年.入院查体:T 37. 9℃,P 116次/min,R 18次/min,BP 130/82 mm-Hg.贫血外观,巩膜无黄染,浅表淋巴结无肿大,口腔可见白斑,左下肢肿胀、压痛,右腿大片瘀斑.

对国内外慢性病护理质量评价指标体系构建的理论框架、方法以及构建范围进行归纳总结,认为我国慢性病护理质量评价指标体系中应加强对患者结局指标的关注,应注意指标体系构建方法的科学性,还应积极探索多病种慢性病护理质量评价指标体系,构建统一的适合我国国情的慢性病护理质量评价指标体系.

目的 本研究旨在探讨多参数流式细胞术(multiparameter flow cytometry,MFCM)在AML1/ETO融合基因阳性的急性髓系白血病(AML)诊断中的应用价值以及分析AML1/ETO融合基因阳性患者的骨髓细胞免疫表型,为病人早期诊断、分型和预后分层提供依据.方法 将细胞遗传学或分子遗传学检查到AML1/ETO融合基因阳性作为金标准,筛选南昌大学第一附属医院2010年1月-2018年1月期间收治初诊AML1/ETO阳性AML患者44例,阴性患者44例,收集所有患者实验室信息(主要包括骨髓细胞形态学,多参数流式细胞术,细胞遗传学或分子遗传学检测结果).采用四格表分别对骨髓细胞形态学和流式细胞术两种方法诊断AML1/ETO融合基因结果真实性和可靠性进行评价,比较骨髓细胞形态学、流式细胞术检查的诊断效能指标,探讨多参数流式细胞术在AML1/ETO融合基因相关的AML早期诊断中的应用价值并分析AML1/ETO阳性AML的免疫表型特点.结果 AML1/ETO阳性患者均表达CD117、CD34、CD33以及HLA-DR(阳性率100.00％),43例表达CD13(阳性率97.73％),42例表达CD19(阳性率95.45％).CD117、CD34、HLA-DR以及CD19共表达的阳性率高达95.45％,而在44例AML1/ETO阴性患者中,均无此表型特点.多参数流式细胞术检测的灵敏度(81.82％vs25.00％),符合率(88.64％vs62.50％)以及阴性预测值(84.00％vs57.14％)明显高于形态学,漏诊率(18.18％vs75.00％)明显低于形态学,特异度(95.50％vs100％)略低于形态学,综合诊断效能明显优于形态学.结论 多参数流式细胞术在AML1/ETO融合基因阳性AML的诊断中具有重要的应用价值,CD117、CD34、HLA-DR以及CD19的共表达是AML1/ETO融合基因阳性AML独特的免疫表型.

目的 近年来多项研究结果表明许多长链非编码RNA(long noncoding RNA,lncRNA)可作为生物标记物用于结直肠癌的诊断.但是它们的诊断效能并不完全一致.本研究利用Meta分析的方法,对lncRNA在结直肠癌中的诊断价值进行系统性评价.方法 数据库中检索发表的有关循环中IncRNA作为肿瘤标记物用于结直肠癌诊断的研究.根据纳入与排除标准进行文献筛选,利用QUADAS-2(Quality Assessment of Diagnostic Accuracy Studies)对文献进行质量评价.使用双变量随机效应模型合并灵敏度(SEN)、特异度(SPE)、阳性似然比(PLR)、阴性似然比(NLR)、诊断比值比(DOR).总体的受试者工作特征曲线下面积(AUC)和受试者工作特征曲线(SROC)用于总体诊断效能评价.使用Cochran's Q检验和I 2(inconsistency index)检验进行异质性分析.亚组分析和Meta回归用于分析异质性原因.结果 根据纳入与排除标准,最终纳入9篇文献,包括833例结直肠癌患者和688例对照.循环IncRNA诊断结直肠癌的合并灵敏度,特异度,阳性拟然比,阴性拟然比,诊断比值比分别为0.77(95％CI:0.72～0.81),0.83(95％CI:0.76～0.88),4.5(95％CI:3.2～6.4),0.28(95％CI:0.22～0.35),16(95％CI:9～27).曲线下面积为0.86(95％CI:0.83～0.89).另外,根据样本量、种族、IncRNA研究模式、样本类型做亚组分析,结果显示异质性主要来源于样本量以及样本类型,样本量≤60组的特异度优于样本量>60组(0.87vs0.81),样本类型为血浆组的灵敏度优于血清组(0.79vs0.74).结论 循环IncRNA对结直肠癌具有较高的诊断价值,可作为结直肠癌诊断的生物标记物.