Macroglossia and carpal tunnel syndrome associated with multiple myeloma: a case report

Background  

Macroglossia is an unusual symptom in primary care. It may be an indicator of an underlying plasma cell dyscrasia or multiple myeloma in the elderly.     

Immunoglobulin light chain amyloidosis associated with multiple myeloma in a young patient: a case report

The authors present an uncommon case of systemic amyloidosis associated with multiple myeloma in a 35-year old woman. Systemic amyloidosis commonly presents in association with clonal plasma cell proliferative disorders, and less frequently as secondary or of a hereditary origin. Amyloidosis is usually associated with multiple myeloma in older patients and frequently has an unfavourable prognosis.     

100例多发性骨髓瘤患者细胞遗传学分析

目的 总结我国多发性骨髓瘤（MM）患者的细胞遗传学特点及其临床意义。方法 回顾性分析我院100例MM患者的细胞遗传学结果。结果 （1）患者总体的克隆性染色体畸变（CA）检出率为17．2％;纳入亚克隆后CA检出率为37．0％;其中哑二倍体最多见;13号染色体异常（C13A）检出率为6．0％,其荧光原位杂交（FISH）检出率为33．3％;14号染色体易位和／或14q32异常检出率6．0％;（2）单因素分析示克隆性CA、非超二倍体和C13A都使患者的总体生存时间（OS）和疾病进展时间（TTP）显著缩短;多因素分析中仅C13A具有独立预后意义。结论 C13A、非超二倍体、超二倍体、免疫球蛋白重链（IgH）易位等CA在我国MM患者中具有重现性。预后分析湿示C13A是独立预后不良因素。     

Clinical and laboratory features of newly diagnosed multiple myeloma: a retrospective, single-centre analysis

Multiple myeloma （MM）, a common malignant plasma-cell proliferative disease, accounts for more than 10% of hematologic malignancies. There have been many studies conducted to analyze the clinical and laboratory features of patients with MM. The purpose of this study was to determine the clinical and laboratory features of patients with newly diagnosed MM in a large cohort of consecutive patients seen at the Blood Disease Hospital of Chinese Academy of Medical Swence during a 13-year period.[第一段]     

Brief communication: Facility characteristics associated with the use of electronic health records in residential care facilities

The integration of electronic health records (EHRs) across care settings including residential care facilities (RCFs) promises to reduce medical errors and improve coordination of services. Using data from the 2010 National Survey of Residential Care Facilities (n=2302), this study examines the association between facility structural characteristics and the use of EHRs in RCFs. Findings indicate that in 2010, only 3% of RCFs nationwide were using an EHR. However, 55% of RCFs reported using a computerized system for one or more (but not all) of the functionalities defined by a basic EHR. Ownership, chain membership, staffing levels, and facility size were significantly associated with the use of one or more core EHR functionalities. These findings suggest that facility characteristics may play an important role in the adoption of EHRs in RCFs.     

自体造血干细胞移植治疗IgD型多发性骨髓瘤并发心源性休克1例

目的:探讨自体造血干细胞移植治疗多发性骨髓瘤(MM)并发心源性休克的病因、临床表现和监测。方法:回顾性分析2015年5月我科收治的1例IgD型MM患者进行自体造血干细胞移植过程中并发心源性休克的临床资料,并复习相关文献。结果:患者在预处理治疗结束后第7天出现烦躁、胸闷、胸痛、腹痛、呼吸困难、血压下降、四肢厥冷表现,心肌酶谱、肌钙蛋白、B型钠尿肽升高明显,心电图提示T波低平,异常Q波;给予利尿、强心、营养心肌、控制输液量等抢救治疗,患者病情控制。结论:在自体造血干细胞移植前应全面评估患者的心脏功能及既往药物治疗史;在移植过程中,密切观察患者心肌损害的临床表现和心肌指标,及时发现、尽快诊断、及早治疗。     

浆细胞白血病--5例报告及与多发性骨髓瘤比较

目的：探讨浆细胞白血病(plasma cell leuremia，PCL)的疾病性质。方法:将我院收治的5例PCL与10例多发性骨髓瘤(multiple myeloma，MM)在临床表现、实验室检查及治疗反应上作比较。结果：PCL具有以下特点：①发病年龄较轻，中位年龄43．5岁；②起病较急，起病至确诊时间明显短干MM；③出血、肝脾淋巴结肿大、胸骨压痛硬发热均较MM多见；④骨髓原 幼浆细胞数明显高于MM；⑤治疗反应差干MM。结论：PCL是一类不同于MM的特殊疾病。     

以肾损害为首发的多发性骨髓瘤19例临床分析

目的探讨多发性骨髓瘤肾损害患者的临床表现、早期诊断及其治疗效果。方法对我院19例以肾脏损害为首发的多发性骨髓瘤患者的临床资料进行回顾性分析。结果所有患者均有不同程度的贫血（血红蛋白55～94g/L）及肾脏损害：尿蛋白（＋～）伴肾衰竭（血清肌酐142～1328μmol/L）。其中6例（31.5%）高钙血症,11例（57.9%）高尿酸血症;,9例（47.3%）高球蛋白血症,6例（31.5%）异常M蛋白,3例（15.8%）溶骨性破坏或骨质疏松。确诊后有2例患者中断、放弃治疗,其余经综合治疗后5例（26.3%）完全缓解,6例需依赖血液透析。结论多发性骨髓瘤肾损害临床表现复杂,对中老年表现为贫血,蛋白尿及肾衰竭患者应考虑该病的可能,完善骨髓穿刺或肾活检术,确诊后有效的化疗,输液、利尿,积极治疗高钙血症、感染及高尿酸血症,血液透析等,可延缓肾损害的进展,改善预后。     

减低剂量的硼替佐米治疗老年性多发性骨髓瘤临床研究

目的 回顾分析1.0 mg/m2和0.7 mg/m2两种低剂量硼替佐米治疗老年(≥60岁)多发性骨髓瘤患者的疗效和相关毒副反应,探讨硼替佐米在老年患者治疗上的剂量选择.方法 2006年6月至2012年1月在军事医学科学院附属医院血液科接受低于标准剂量的硼替佐米治疗的老年多发性骨髓瘤患者共26例,其中16例接受1.0 mg/m2剂量治疗,10例接受0.7 mg/m2剂量治疗.观察疗效和相关毒副反应.结果 第1个疗程结束后1.0 mg/m2组和0.7 mg/m2组有效的例数分别为13例和8例,第2个疗程结束后仍有效的例数分别为9例和5例,两组间差异无统计学意义(P＞0.05).硼替佐米的主要不良反应为消化道症状、周围神经病变、血小板减少和感染等,两组间的发生率差异均无统计学意义(P＞0.05).结论 老年多发性骨髓瘤患者采用1.0 mg/m2和0.7 mg/m2硼替佐米治疗剂量均相对安全有效;对于不能耐受1.0 mg/m2者,也可选择0.7 mg/m2剂量.     

多发性骨髓瘤使用双磷酸盐引起颌骨坏死四例

目的 提高对在多发性骨髓瘤(MM)中使用双磷酸盐引起颌骨坏死的警觉.方法 报道4例MM患者使用双磷酸盐12～44个月致上、下颌骨坏死的临床表现及治疗经过.并复习了近年国外文献,讨论其发病的危险因素、预防措施及治疗策略.结果 4例确诊MM患者,男3例、女1例,均联合化疗并使用帕米磷酸二钠治疗后12～44个月出现右上颌、左下颌部及双下颌骨软组织肿痛伴溢脓,X线检查提示颌骨坏死.经全身使用抗生素,并停用双磷酸盐,加强醋酸氯已定漱口,脓肿治愈.结论 MM长期化疗联合使用双磷酸盐可引起颌骨坏死,上颌及下颌骨均可受累,尚无满意的治疗方法.     

常染色体隐性遗传Alport综合征基因突变分析方法研究

目的 通过一例常染色体隐性遗传型Alport综合征患者家系的基因突变分析,探讨常染色体隐性遗传Alport综合征的基因诊断方法.方法 提取该患者外周血细胞的基因组DNA,应用基因组DNA PCR测序法对COL4A3和COL4A4基因各外显子进行突变筛查.对于基因组DNA序列异常者,从外周血细胞和EB病毒转染的细胞中提取总RNA,应用cDNA RT-PCR测序方法分析突变.同时在家系和正常人群中进行验证.结果 使用基因组DNA样本经PCR直接测序法检测到COL4A3基因上两个致病性新突变(IVS 39+1 G＞A剪接突变和c.1729-1737 de19bp缺失突变);经RT-PCR测序方法验证其与基因组DNA样本中检测到的突变一致,并证实剪接突变产生异常的转录产物.结论 基因组DNA PCR测序法和cDNA RT-PCR-测序法均检测到Alport综合征患者COL4A3的致病突变,两种方法结果一致;cDNA RT-PCR测序法因为其相对快捷,并且可以了解突变转录情况而优于经典的基因组筛查突变方法.     

以蛋白尿为首发症状的多发性骨髓瘤61例临床和病理分析

多发性骨髓瘤(multiple myeloma,MM)是克隆性浆细胞异常增生性疾病,随着人口老龄化,MM的发病率呈上升趋势.据国内外统计,多发性骨髓瘤肾损害发生率为60%～90%.蛋白尿是MM肾病早期的表现,部分患者仅表现为蛋白尿,数年后才出现骨髓瘤的其他症状或肾功能不全,故易误诊为肾小球肾炎、无症状性蛋白尿或隐匿性肾炎.本研究回顾了我科自1996年至今以蛋白尿为首发症状的MM患者61例的临床资料,旨在提高肾内科医生对该病的认识,避免漏诊和误诊.     

Multiple myeloma associated with light-chain amyloidosis manifesting as gastric retention: a case report and review of the literature

We report a case of multiple myeloma associated with light-chain amyloidosis in a 62-year old woman. The patient came to hospital with the main complaint of epigastric pain and gastroscopy showed gastric retention. The patient had been diagnosed with multiple myeloma associated with light-chain amyloidosis after biopsy of the gastric mucosa and bone marrow aspirate. A review of the literature was also performed.     

颅脑损伤脑积水36例临床分析

颅脑损伤后脑积水加重患者的病情,是严重影响患者预后、致残和致死的重要因素之一.为正确认识发病机制及正确进行治疗,现将我院1995年11月～2002年11月收治的36例颅脑损伤后脑积水进行如下分析.     

不同剂量硼替佐米联合地塞米松治疗难治复发多发性骨髓瘤

目的 评价硼替佐米联合地塞米松方案治疗难治复发多发性骨髓瘤(MM)的有效性和安全性.方法 前瞻性设计2种剂量的硼替佐米联合地塞米松的化疗方案,随机分组对照治疗难治复发MM患者23例,1O例患者应用硼替佐米1.0 mg/m2,第1、4,8、11天给药,地塞米松20 mg/d,第1～4天;标准剂量组(13例):硼替佐米1.3 mg/m2,地塞米松40 mg/d,第1、4、8、11天给药,均21 d为1个疗程.采用欧洲造血干细胞移植合作组织(EBMT)标准观察疗效.结果 中位随访9.5(3-15)个月.硼替佐米1.0 mg/m2组与1.3 mg/m2组相比:有效率分别为70.O%和61.5%(P>0.05);缓解率分别为20.O%和38.5%(P>0.05).硼替佐米1.0 mg/m2组仅1例CTCAE 3级以上不良反应,硼替佐米1.3 mg/m2组感染性发热发生率38.5%(5/13),治疗相关死亡率15.4%(2/13).结论 硼替佐米1.3 mg/m2与1.0 mg/m2组相比有效率相似,缓解率较高,而毒副作用明显增加.     

Ruling out cardiac failure: Cost-benefit analysis of a sequential testing strategy with NT-proBNP before echocardiography

Abstract

Objectives. To estimate the possible economic benefit of a sequential testing strategy with NT-proBNP to reduce the number of echocardiographies.

Methods. Retrospective study in a third-party payer perspective. The costs were calculated from three Swedish counties: Blekinge, Östergötland, and Uppland. Two cut-off levels of NT-proBNP were used: 400 and 300 pg/mL. The cost-effectiveness of the testing strategy was estimated through the short-term cost avoidance and reduction in demand for echocardiographies.

Results. The estimated costs for NT-proBNP tests and echocardiographies per county were reduced by 33%–36% with the 400 pg/mL cut-off and by 28%–29% with the 300 pg/mL cut-off. This corresponded to a yearly cost reduction of approximately €2–5 million per million inhabitants in these counties.

Conclusion. The use of NT-proBNP as a screening test could substantially reduce the number of echocardiographies in the diagnostic work-up of patients with suspected cardiac failure, as well as the associated costs.     

循证护理对减少或预防患者 PICC置管并发症的M eta分析

目的：综合评价循证护理对减少或预防患者经外周静脉置入中心静脉导管（PICC ）置管并发症发生的应用效果。方法计算机检索中国知网、万方数据库、中国生物医学文献数据库、VIP资源,Pubmed数据库,收集国内外应用循证护理的方法减少或预防PICC置管并发症的随机对照研究,采用RevM an 5．0软件对符合纳入标准的随机对照研究进行M eta分析。结果共纳入17个随机对照研究,3092例研究对象。研究结果显示,循证护理组患者PICC置管并发症（静脉炎、穿刺点感染、导管脱出或异位及导管堵塞）发生率明显低于对照组（P＜0．05）。结论循证护理可有效减少或预防PICC置管并发症的发生,提高护理质量的同时改善PICC置管患者生活质量,值得在临床上推广应用。