Lifelong treatment with gammaglobulin for primary antibody deficiencies: the patients' experiences of subcutaneous self-infusions and home therapy

Primary antibody deficiencies are chronic conditions and the patients usually need lifelong replacement therapy with gammaglobulin to prevent or reduce infections It has been shown that the gammaglobulm can be given safely as subcutaneous infusions, instead of intramuscular injections or intravenous infusions The major aim of this multi-centre study was to investigate the perceptions of the subcutaneous method among patients using it, both in hospital settings and as self-infusions at home The study included 152 patients 89 women, 63 men, mean age 44 years (range 18-76) Data were collected by using questionnaires The patients were found to have a strongly positive attitude towards receiving the replacement therapy as subcutaneous infusions, perceived the method as effective in preventing infections and wished to retain the treatment However, the younger patients found the subcutaneous infusions more uncomfortable and were less determined to continue with the therapy as compared with the older individuals The responsibility for self-infusions at home was accepted by the patients, leading to an increased independence from the health care personnel and to a feeling of flexibility and freedom As these patients have a chronic disease and are in need of lifelong treatment, it is important to discuss the development of structured education and training programmes in which special emphasis is placed on the support of the younger patients It is suggested that Orem's nursing model of self-care may serve as a conceptual framework for nurses working in this specific area of nursing care     

乳腺癌中RRIG1 mRNA的表达及意义

目的探讨维甲酸受体诱导基因1(retinoic receptor-induced gene 1,RRIG1)在人乳腺癌组织中的表达及其与患者临床病理参数的关系。方法以GAPDH为内对照采用实时荧光定量逆转录聚合酶链反应法(real time quantitative reverse transcrip-tion polymerase chain reaction,RQ RT-PCR)检测76例新鲜乳腺浸润性导管癌组织5、例导管原位癌组织和20例乳腺良性增生组织中RRIG1 mRNA的表达水平,并分析其与患者临床病理参数的关系。结果乳腺导管原位癌、浸润性导管癌组织中RRIG1 mRNA相对表达水平(3.5±2.1、6.7±4.1)明显低于良性增生组织(10.2±6.5),差异具有统计学意义(P=0.004,P=0.036);乳腺浸润性导管癌中淋巴结转移组RRIG1 mRNA相对表达水平(5.2±3.1)明显低于无淋巴结转移组(7.48±4.5),两组之间差异具有统计学意义(P=0.023)。结论 RRIG1可能作为一种新的抑癌基因参与乳腺癌的发生、浸润和转移,对乳腺癌早期诊断和预后评估具有重要的参考价值。     

卡维地洛与美托洛尔治疗高原慢性充血性心力衰竭疗效的对比研究

目的 比较卡维地洛与美托洛尔治疗高原慢性心力衰竭(CHF)的疗效.方法 90例CHF患者随机分成3组:常规治疗组(20例)给予血管紧张素转化酶抑制剂、利尿剂、地高辛等常规心力衰竭治疗.美托洛尔组(34例)、卡维地洛组(36例)在上述治疗基础上分别给予美托洛尔50 mg,2次/d;卡维地洛25 mg,2次/d口服.随访半年,治疗前、后采用超声心动图测定患者心功能并进行疗效观察.结果 治疗后美托洛尔组、卡维地洛组左心室舒张末期内径(LVEDD)[分别为(57.3±6.5)、(57.2±6.9)mm]和左心室收缩末期内径(LVSED)[分别为(46.6±7.0)、(44.0±6.9)mm]显著低于常规治疗组[分别为(64.7±9.1)、(53.4±9.8)mm],左心室射血分数(LVEF)显著高于常规治疗组[分别为(47.5±8.1)%、(52.9±8.5)%、(42.8±9.2)%](P均＜0.05).卡维地洛组LVEF改善优于美托洛尔组(P＜0.05).死亡情况:常规治疗组4例,美托洛尔组1例,卡维地洛组无死亡.美托洛尔组、卡维地洛组病死率均明显低于常规治疗组(P均＜0.05).结论 美托洛尔、卡维地洛均可明显改善高原CHF患者心功能.卡维地洛疗效及耐受性略优于美托洛尔.

Abstract：

Objective To compare the effect of carvedilol and motoprolol on high altitude chronic congestive heart failure (CHF). Methods Ninety patients with high altitude chronic CHF were divided into three groups randomly:Twenty patients in the regular treatment group treated with angiotensin-converting enzyme inhibitor (ACEI) ,diuretics and digoxin; motoprolol (50 mg twice daily) was given in the motoprolol group( 34cases) additional to regular treatment; carvedilol (25 mg twice daily) was given in the carvedilol group(36cases ) additional to regular treatment. All the patients were followed up for six months and measured the changes of cardiac function by echocardiography. Results Left ventricular end-diastolic dimension (LVEDD) was ( 57. 3 ± 6. 5 ) mm and (57.2 ± 6. 9) mm in the carvedilol group and the motoprolol group respectively, and left ventricular end-systolic dimension (LVESD) was (46. 6 ± 7.0) mm and (44. 0 ± 6. 9 ) mm in the carvedilol group and the motoprolol group respectively, which were all significantly smaller than that in the regular treatment group ([64.7 ±9. 1]mm and [53.4 ±9.8]mm for LVEDD and LVESD,respectively) (Ps ＜0.05). Left ventricular ejection fraction (LVEF) in the carvedilol group and the motoprolol group ( [47.5 ± 8. 1] % and [52. 9 ±8.5] % ,respectively) was higher than that in regular treatment group( [42. 8 ±9. 2]% ) (Ps ＜0. 05).The improvement of LVEF in the carvedilol group was better than that in the motoprolol group (P ＜ 0. 05 ). One case died in the motoprolol group and no death in the carvedilol group,4 cases died in the regular treatment group,the mortality in the motoprolol group and the carvedilol group was significantly lower than that in the regular treatment group. Conclusion Carvedilol and motoprolol significantly improved cardiac function in high latitude CHF patients,and the effect of Carvedilol is slightly better than that of motoprolol.     

Cardiac Resynchronization Therapy (and Multisite Pacing) in Pediatrics and Congenital Heart Disease: Five Years Experience in a Single Institution

Introduction: Clinical evidence supports the use of cardiac resynchronization therapy (CRT) in adults with heart failure, but experience in pediatrics and congenital heart disease (CHD) is limited in terms of patient numbers and follow-up. We sought to determine the functional assessment and clinical outcomes in pediatric and CHD CRT patients followed uniformly at one institution.
Methods: Retrospective review of 60 consecutive patients who underwent CRT between 2002 and 2007.
Results: At implantation, median age was 15.0 years (5 months to 47 years). Overall, 46 patients had CHD (77%) and 14 had dilated cardiomyopathy. Prior to CRT, 92% were on heart failure treatment drugs and 55% had pacemakers. Median follow-up time was 0.7 years (1 day–5.3 years). Median QRS width decreased from 149 to 120 ms (P < 0.001). Median ejection fraction (EF) increased from 36% to 42% (P < 0.001) and improvement was particularly evident in the group with CHD. Of note, 8 of 13 patients with single ventricle morphology had a "strong CRT response," defined as either an improvement of 2–3 ordinal points in NYHA classification and/or increased ventricular function by ≥ 10 EF units. Overall, an improvement in functional status was observed in 39 of 45 patients (87%) with sufficient follow-up data.
Conclusions: Children and CHD patients treated with CRT have acute improvement in ventricular function, but implantation may require individualized planning and unconventional approaches. Future important goals include preimplant determination of CRT responders in pediatric and CHD patients, optimizing lead placement and programing, as well as long-term CRT device management issues.     

转录因子FoxO3a在小鼠牙胚发育过程中的表达

目的：通过观察转录因子FoxO3a在小鼠牙胚发育中的表达特点及分布情况,探讨其在小鼠牙胚发育过程中的作用。方法取出生0 d和3 d的昆明小鼠各3只,处死解剖下颌骨,放于新鲜配置的10％中性甲醛溶液中固定过夜,制备小鼠牙胚0d和3d的组织标本,常规石蜡包埋,近远中向5μm连续切片,采用免疫组化方法观察检测FoxO3 a在小鼠不同时期的牙胚表达情况和分布情况,并对结果进行分析。结果 FoxO3 a在小鼠不同时期的牙胚发育中的分布存在差异,FoxO3a在0d小鼠牙胚中,成牙本质细胞成阴性表达,在3d小鼠牙胚中成牙本质细胞成阳性表达。结论观察FoxO3a在小鼠牙胚发育过程中的表达以及表达的变化,提示FoxO3a可能参与牙本质的形成和矿化。     

阻塞性睡眠呼吸暂停综合征与高脂血症关系长期随访研究

目的随访阻塞性睡眠呼吸暂停综合征(OSAS)与高脂血症的关系。方法对1868人进行前瞻性随访,通过家访式调查,随访期间每年进行1次体检,检查血压、血脂、血糖、心电图及X线胸片等,随访时间为20年。结果确诊OSAS者598/1868(32.0%),男496例(82.9%),女102例(17.1%);无OSAS者(对照组)1270/1868(68.0%)。随访终点OSAS组高脂血症529例,对照组396例(P<0.01)。结论 OSAS患者发生高脂血症可能性较一般人群高,考虑OSAS与高脂血症发生存在相关性。提示OSAS可能是高脂血症的独立危险因素。引起高脂血症的机制可能与OSAS导致食欲增加、活动减少、肥胖等有关。