IgA肾病研究进展

原发性IgA肾病是世界范围内最常见的肾小球肾炎,是引起终末期肾功能衰竭的重要原因之一。以往认为此病呈良性经过,预后良好,然而近年来的一系列随访研究证实,此病在确诊后5~25年内,有20%~40%的患者发展为终末期肾脏疾病。迄今为止,其发病机制尚不完全明了,临床及病理表现多样,仍无特异性治疗药物,预后各不相同。仅就近年来对该病的研究进展简要综述。     

Profile of admissions to an acute dialysis care unit

The profile of admissions to an acute dialysis care facility of the National University Hospital Renal Centre, Singapore was studied in an attempt to identify the needs of these patients so that admissions may be possibly reduced. All patients above the age of 18 years who were admitted to the dialysis centre in the National University Hospital for dialysis between 1 Jan 2000 and 31 Mar 2000 inclusive were studied. A total of 124 patients were studied. Almost all the patients had end stage renal failure. The commonest cause of end stage renal failure was diabetic nephropathy (58.8%). There were a total of 157 admissions. Most of the admissions were associated with end stage renal failure (96%). Vascular access problems caused 40% of all admissions and 68% of all multiple admissions. The data suggest that a dialysis facility in a multidisciplinary acute hospital needs to meet the requirements of mainly patients who are already in end stage renal failure, those who have vascular access problems and those who have diabetes.     

Chronic renal failure in Jamaican children

In order to document the incidence, aetiology and outcome of chronic renal failure in Jamaican children, paediatric surgeons and hospital based paediatricians island-wide were contacted, and the nephrology records at the island's paediatric nephrology centres searched for data on children < 12-year-old with chronic renal failure diagnosed for the first time between January 1985 and December 2000. Thirty-four children were identified, 21 were male. The cumulative annual incidence of chronic renal failure was 3.2 per million children aged < 12 years. The incidence is likely underestimated, as some children may have been undiagnosed and/or not referred. Glomerulonephritis was the commonest cause of chronic renal failure (50%) followed by obstructive uropathy, reflux nephropathy, renal dysplasia and chronic pyelonephritis (41.2%). Five children (14.7%) had reflux nephropathy (post obstructive in four). Half the children were already in chronic renal failure at time of presentation. Mortality was 65%. In Jamaica, childhood chronic renal failure is due mainly to potentially treatable diseases. Local physicians should be more aware of potentially progressive renal diseases and their prevention. Earlier referral of difficult cases for nephrological consultation is recommended. A paediatric dialysis/transplant programme is needed.     

Autosomal dominant polycystic kidney disease clinical analysis in VGH--Taipei

Autosomal dominant polycystic kidney disease (ADPKD) is the commonest hereditary nephropathy. We collected 92 cases in VGH. Diagnosis was confirmed by intravenous pyelogram, renal sonogram, or renal CAT scan. The incidence of having positive family history was just only 28.3%. Patients were diagnosed at the mean age of 54 +/- 11 years (26-74 years). The common clinical findings were hypertension (73.9%), abdominal mass, proteinuria, anemia, azotemia, abdominal or back pain and pyuria in orders. Hypertension might present in the early stage with normal renal function (near 40%). Polycystic liver was the major extrarenal lesion (57.6%), but the incidence of abnormal liver function was only 10.1%. Enlarged kidneys were not always palpable, even at end stage of renal function (mean age 56 +/- 9 years, 89.4% kidney palpable). Patient's urine amount was usually nonoliguric, even in uremic stage (82.9%). Sepsis was the first cause of death. Cardiovascular disease and uremia were followed in sequence. Their expired mean age was 61 +/- 7 years (53-74 years).     

肾移植术后妊娠1例报告及文献复习

移植术后合并妊娠的病例在国内尚属于较罕见病例,产科医生缺乏相关的诊治经验.北京大学第一医院妇产科曾于2008年收治了1例肾移植术后合并妊娠的患者,经积极治疗和及时终止妊娠,母婴结局良好.本文结合文献复习,讨论肾移植术后妊娠的特点及相关并发症的诊疗.     

Who gets renal bone disease before beginning dialysis?

To identify patients at risk from renal bone disease we compared the demographic characteristics of 243 patients with end stage renal failure grouped according to the presence (97 (40%] or absence of severe renal bone disease as judged by histological criteria. Youth, female sex, tubulointerstitial types of nephropathy, and a long duration of uraemia were all identified as significant independent risk factors for the development of bone disease. The relative risks from being female and having tubulointerstitial renal disease were separately identifiable when the estimated observation of renal failure was short (less than four years). The identification of patients at high risk from bone disease may clarify the pathogenesis and treatment strategies of renal osteodystrophy.     

Pregnancy following renal transplantation in Sri Lanka

OBJECTIVE: To assess the outcome and complications of pregnancy following renal transplantation in Sri Lanka. METHODS: Ten pregnancies following transplantation managed between January 1993 and July 1999 by the University Obstetrics and Gynaecology Unit, De Soysa Hospital for Women, Colombo were reviewed. RESULTS: Five women had planned pregnancy with an average duration from transplantation to conception of 2.3 (+/- 0.2) years; five had an unplanned pregnancy within 12 months of transplantation. All were treated with immunosuppressives, with none developing rejection. In the planned pregnancy group, 3 developed pregnancy induced hypertension and 3 impaired glucose tolerance. All delivered mature healthy babies with an average birth weight of 2.6 (+/- 0.3) kg. In the unplanned group, 1 developed cholestatic jaundice and delivered a growth retarded baby at 36 weeks. Another developed severe pulmonary oedema at 34 weeks (due to a past myocardial infarction) resulting in a fresh stillbirth. Two others has mid-trimester foetal deaths complicating severe diabetes mellitus. The conception at 3 months after transplantation developed diabetes mellitus and pregnancy induced hypertension, and delivered a live growth retarded baby. None had deterioration of renal function. CONCLUSION: Although a successful outcome is possible with stringent pre-pregnancy selection, maternal morbidity and foetal wastage can be high in those without.     

3547例慢性肾脏疾病患者分期及相关因素分析

目的：慢性肾脏病（CKD）是人类面临的主要健康问题之一,本研究调查了中南大学湘雅二医院肾内科CKD患者的分期情况及病因、年龄、性别等相关因素分析。方法：回顾性分析2003年1月到2008年12月6年间中南大学湘雅二医院肾内科收住的3 547例CKD3期及以上的第一次住院患者（≥14岁）的资料,参照NKF-K/DOQI指南的标准来定义CKD,按照简化的MDRD公式求eGFR[eGFR=186.3×血肌肝（SCr）^-1.154×年龄^-0.203×0.742（女性）mL/min·1.73 m^2]。记录患者的性别、年龄、住院号、病因及功能诊断、病程、收缩压和舒张压、血红蛋白、尿量、肾功能、血白蛋白、肾脏B超、治疗方式等项目,并进行统计分析。结果：（1）原发性肾小球疾病、高血压病和糖尿病是导致CKD的主要病因,分别占55.20%,14.55%和11.78%。6年间的病因构成无明显差异（P〉0.05）。但病因构成中慢性肾小球肾炎比例有所下降,而高血压肾病、糖尿病肾病的比例有所上升。（2）CKD3期到5期患者所占比例分别为11.59%,23.03%和65.38%。41～60岁的患者占CKD的39.24%,大于60岁的患者占CKD的36.88%。慢性肾小球肾炎患者的年龄常见于21～40和41～60岁年龄组。狼疮性肾炎患者年龄较轻,21～40岁占48.35%。高血压肾病和糖尿病肾病患者年龄较大,95%以上见于40岁以上患者。（3）梗阻性肾病、痛风性肾病多发于男性,而狼疮性肾炎以女性多见。（4）大多数CKD患者伴贫血（94.28%）和高血压（56.91%）,且随CKD病程进展,贫血、高血压发生率及严重程度增高（P〈0.005）。（5）CKD5期患者2 319例,926例行血透,181例行腹透。结论：CKD病因前3位依次为慢性肾小球肾炎,高血压肾病,糖尿病肾病。糖尿病肾病和高血压肾病的比例呈上升趋势。CKD3期到5期患者中以5期患者居多,老年人是CKD的高危人群。     

Vesicoureteric reflux and urinary tract infection in children

An association between vesicoureteric reflux (VUR) and renal damage was found in 1960. In 1973, the term reflux nephropathy (RN) was first used to describe the renal damage caused by VUR. Follow up studies show that about 10%-20% of children with RN develop hypertension or end stage renal disease. It is now evident that there is a sex difference in the development of RN. In most males with RN, the kidneys are congenitally abnormal. In females it is an acquired condition, the most severe damage being sustained by recurrent urinary tract infections (UTIs). The purpose of current UTI guidelines is to identify VUR or any other abnormality of the urinary tract. Since the advent of routine antenatal ultrasonography, there is no longer a need to identify an abnormality of the urinary tract after the first reported UTI. Routine investigations are not required. Recurrent UTIs and a family history of VUR need further evaluation. There is also an urgent need to establish the long term value of prophylactic antibiotics in children with VUR.     

Natural course of penicillamine nephropathy: a long term study of 33 patients

To elucidate the natural course of the nephropathy associated with penicillamine and thereby facilitate its clinical management 33 patients with rheumatoid arthritis who developed proteinuria during treatment with oral penicillamine were studied in detail throughout their renal illness. Renal biopsies were performed, and creatinine clearance and proteinuria were measured serially for 74 months (range 16-148 months). Fourteen patients developed proteinuria within six months after the start of treatment and 27 within 12 months. When treatment was stopped the proteinuria reached a median peak of 4.2 g/24 h (range 0.3-15.0 g/24 h) at one month (range 0-7 months) before resolving spontaneously by six months (12 patients), 12 months (21), or 18 months (29). In all patients but one, who developed carcinoma of the renal pelvis, proteinuria resolved by 21 months and its median duration was eight months. The median first and last measurements of creatinine clearance showed no appreciable change (80 ml/min and 78 ml/min), and no patient died from or needed treatment for renal failure. The HLA-B8 or HLA-DR3 alloantigen, or both, were identified in 10 patients. Renal biopsy specimens showed membranous glomerulonephritis in 29 patients, minimal change nephropathy in two, and electron dense deposits in the mesangial regions in two. In all the patients whose nephropathy was due solely to treatment with penicillamine the proteinuria resolved completely when the drug was withdrawn; renal function did not deteriorate, and corticosteroids were unnecessary.     

Medical causes and histological pattern of glomerulonephritis

Glomerulonephritis (GN) is one of the commonest causes of chronic renal failure (CRF) and end stage renal failure (ESRF). The outcome largely depends on the underlying medical cause, clinical findings and histological appearance. 1188 patients were taken in this study. Of these, 980 had primary and 208 secondary GN. Commonest histological types of primary GN were mesangial proliferative glomerulonephritis (31%) and membranous nephropathy (21%). Among patients with secondary GN, predominant cause was lupus nephritis (76%) followed by poststreptococcal GN (18%). Clinically, nephrotic syndrome was present in 54%, hypertension in 39% and renal failure in 28%. But clinical pictures were variable among different histological types of GN.     

同种异体原位心脏移植成功3例报告

目的:总结分析3例同种异体原位心脏移植术的临床经验。方法:采用Shumway&Stanford方法对3例终末期扩张型心肌病病人行同种异体原位心脏移植术。其中1例伴有严重糖尿病和中度肺动脉高压,术前心跳骤停心肺复苏成功;1例极度肥胖,体重108kg(身高172cm);1例62岁高龄伴肾功能不全和中度肺动脉高压。供体均为脑死亡者,供心保护采用4℃改良StThomas液;术后免疫抑制治疗采用环孢素A、骁悉、强的松三联治疗,根据血环孢素浓度及心内膜活检调整环孢素A用量。结果:3例均康复,未发生术后超急性或急性排斥反应。例2术后出现一过性右心功能不全,经大量利尿和无创通气治疗,48h后完全恢复;例3术后并发急性肾功能衰竭和肺部感染,经连续肾脏替代(CRRT)和调整抗生素治疗痊愈。病人心功能均恢复正常。例1需长期胰岛素治疗糖尿病。3例均完全过上了正常人的生活,2例已恢复手术前工作。结论:同种异体原位心脏移植是治疗终末期心脏病的有效方法。     

Proteinuria in diabetic patients in a primary health care setting in Sarawak

Diabetic nephropathy is now the number one cause of end stage renal failure in Malaysia. This places a huge burden on patients and the health care system especially in developing countries with limited health care resources, such as in Sarawak in East Malaysia. This study describes the prevalence of proteinuria/microalbuminuria in diabetic patients treated in Klinik Kesihatan Tanah Puteh. Early detection of proteinuria/microalbuminuria allows remedial measures to be taken to retard the progression of nephropathy. Forty-eight percent of the cases had proteinuria and microalbuminuria was found in 16%. Seventy-eight percent of cases with proteinuria were on treatment with angiotensin-converting-enzyme inhibitors or angiotensin-receptor blockers. Seventy-five percent of patients had hypertension but only 6% achieved the targeted BP of < 130/80 mmHg.     

Chronic renal insufficiency in pregnancy

This article attempts to assess the nature, severity and management of the risks associated with pregnancy in chronic renal insufficiency and end-stage renal disease, including dialysis and transplant recipients. Women with serum creatinine levels of >125 mmol/l are at an increased risk for deterioration in renal function, hypertension with superimposed pre-eclampsia and obstetric complications. Rigid control of hypertension is crucial for a successful pregnancy outcome. A range of antihypertensive drugs are available with angiotensin converting enzyme inhibitors being contraindicated. Women on dialysis have low fertility rates that return to normal following renal transplantation. Immunosuppresive drugs are not associated with increased congenital anomalies. Transplant recipients are at an increased risk for infections that may have implications for the fetus. All groups have an increased risk for prematurity and intrauterine growth restriction. The percentage of pregnancies resulting in surviving infants in women with renal insufficiency and transplant recipients ranges from 80-100%. For women who conceive after dialysis, the likelihood of a surviving infant is approximately 50%.     

IgA肾病临床与病理分型

目的探讨原发性IgA肾病的病理与临床疗效及预后之间的关系。方法对55例原发性IgA肾病的患者进行肾穿刺后分析其病理分型、临床表现、疗效及预后。结果病理分型轻者多表现为无症状血尿和／或蛋白尿,疗效较好,而病理分型重者临床表现多为肾病综合征伴高血压、肾功能不全,疗效差,预后差,提示病理分型与临床表现、疗效及预后有明显相关。结论早期肾穿刺活检及早治疗,可以延缓肾功能衰竭进展。     

Malignant hypertension in women of childbearing age and its relation to the contraceptive pill

Eleven of 34 women aged 15-44 with malignant phase hypertension were taking oral contraceptives at presentation. All had had normal blood pressure before starting to take the pill. In four the interval between the start of oral contraception and the diagnosis of malignant hypertension was less than four months, and in eight no other cause for the hypertension was found. Underlying renal disease and renal failure were less common among pill users than among non-users with malignant hypertension who were of similar age. No pill user became normotensive after withdrawal of the pill, but blood pressure was well controlled (diastolic less than 90 mm Hg) in three patients taking only one drug. By contrast, all 23 non-users needed two or more antihypertensive drugs to control blood pressure. Ten year survival was 90% among pill users and 50% among non-users. These results suggest that oral contraceptives may be a common cause of malignant hypertension in women of child-bearing age. If the pill is stopped and underlying renal disease excluded the long term prognosis for such patients is excellent.     

特发性膜性肾病预后影响因素

目的　总结 86例特发性膜性肾病 (IMN)患者早期临床及病理资料特点 ,分析肾脏存活率 ,探讨预后影响因素。方法　回顾性分析 1 983- 2 0 0 1年我院经肾活检证实的 86例IMN患者的临床病理资料特点 ,对其中获得随访的 4 4例 ,以进展至终末期肾衰 (ESRF)或血肌酐浓度 (Scr)增加 1倍 (肾功能恶化 )作为终点判断标准 ,采用Kaplan Meier曲线和COX比例风险模型分别进行预后分析。 结果　 86例IMN患者中 ,蛋白尿 >3.5 g/ 2 4h占 5 4 .7%,肾病综合征占 4 4 .2 %,血尿 2 9.1 %(肉眼血尿仅 1例 ) ,高血压 2 3.3%,肾静脉血栓 3.5 %。病理分期以Ⅰ、Ⅱ期为主 ,占 96 .5 %,小管 间质病变程度较轻。病理改变与肾活检前病程、Scr正相关 ,与内生肌酐清除率呈负相关。以发病日为随访起点 ,5年肾脏存活率 96 %,1 0年 6 1 .8%;以肾穿刺日为起点 ,5年肾脏存活率84 .5 %,1 0年 5 5 .6 %。肾活检时的Scr和血胆固醇浓度与IMN预后相关。结论　肾活检时的Scr和血胆固醇浓度可能是预测IMN预后的有用指标。IMN患者应早就诊 ,早穿刺 ,以了解病情指导治疗 ,从而更好地保护肾功能。     

成人特发性膜性肾病264例的临床与病理分析

目的：了解我国成人膜性肾病的临床与病理特征。方法：回顾性分析1986－1997年的264例住院患,对各年龄段患的临床表现、病理特征进行统计,采用不配对资料t检验分析组间差异。结果：（1）我国膜性肾病好发于21－40岁,61－70岁的患肾功能不全的比较较其他组高,而－60岁的患高血压的发生率较高。（2）临床表现为大量蛋白尿、高血压、肾功能不全及镜下血尿分别为29．9％、13．3％、7．7％及40．2％,28．0％的患表现为肾病综合征。（3）平均随访49个月,16．2％的患发生肾功能恶化,（4）Ⅰ、Ⅱ、Ⅲ、Ⅳ期各占42．3％、48．7％、6．0％和3．0％,肾组织中IgG,C3及C1q的沉积分别为93．2％、98．8％和58．3％。结论：我国膜性肾病的发病年龄相对较年轻,临床上表现为肾病综合征的缃例相对较少,而高血压、镜下血尿肾功能不全的发生率与国外相近。     

HELLP综合征合并急性肾功能衰竭10例分析

目的探讨HELLP综合征合并急性肾功能衰竭（ARF）的临床特点、诊断、治疗和孕产妇及新生儿预后。方法对我院2005年1月-2008年12月以来HELLP综合征合并ARF的10例患者临床资料进行回顾性分析。结果多为妊娠期高血压疾病的并发症,以血压升高、恶心、呕吐、水肿、皮下淤斑或黏膜出血、少尿或无尿为特点,乳酸脱氢酶、转氨酶、血小板、血肌酐等均为诊断及监测疾病变化及预后的指标;在常规治疗HELLP的基础上及时终止妊娠、行血液净化治疗,是防止病情恶化降低孕产妇及围产儿死亡率的关键。结论HELLP综合征合并ARF发病急而凶险,早期诊断、综合治疗、及时终止妊娠及血液净化治疗是改善孕产妇及新生儿预后的关键。     

A survey of chronic renal failure in Jamaica

The prevalence of chronic renal failure (CRF)/end stage renal disease and the accessibility of long term renal replacement therapy in Jamaica were evaluated. The study was conducted at six Jamaican healthcare facilities between July 1998 and December 1999 and included 605 patients with CRF. Men with CRF (57% of patients, mean age of 56.7 years) were significantly older than women (mean age 53.2 years). Hypertension was the most commonly associated medical condition (60.8% of patients) followed by diabetes mellitus (31.4% of patients). The estimated crude point prevalence of CRF in persons 20 years and over at the end of 1999 was 327 per million population. More than one-third of patients with CRF (39%) were receiving renal replacement therapy, the most common modality being haemodialysis, and only 1.8% of patients had received kidney transplantation. The prevalence of chronic renal failure was not increased in areas known to have high soil cadmium levels. Chronic renal failure is a significant public health problem in Jamaica and is placing an increasing financial burden on the healthcare sector.