以肾脏受累为主要表现的ANCA相关性小血管炎的诊治探讨

目的 探讨以肾脏受累为主要表现的抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎的早期诊断及适时合理治疗。方法 对21例肾脏受累为主要表现的ANCA相关性小血管炎患者的临床资料进行回顾性分析。结果 21例中pANCA阳性18例、cANCA阳性3例．4例表现为急进性肾炎综合征，其中2例伴有咯血；7例表现为慢性肾衰，其中鼻中隔穿孔2例、失明2例；5例表现为急性肾衰；5例以尿检异常、乏力、持续低熟、贫血、体重下降等表现入院。7例经甲基强的松龙、环磷酰胺冲击治疗，病情稳定，肾功能好转或正常；3例经甲基强龙、环磷酰胺冲击治疗配合血液透析，肾功能恢复正常；3例仍需维持性血透；2例死于透析后心衰、1例死于突发肺出血并消化道出血；5例经用激素治疗(未用MP、CTX冲击治疗)，临床症状消失，肾功能正常。结论 伴有肾脏受累的ANCA相关性小血管炎临床表现缺乏特异性．早期诊断、早期适当治疗是改善预后的关键。     

硬皮病相关自身抗体谱与中国系统性硬化患者临床相关性分析

目的分析硬皮病相关自身抗体谱[包括抗Scl-70抗体、抗RNA多聚酶Ⅲ抗体（ARA-Ⅲ）、抗着丝点抗体（ACA）和抗U1核糖核蛋白（U1RNP）抗体]与中国系统性硬化症（SSc）患者临床特征的相关性。方法前瞻性序贯纳入入选欧洲抗风湿病联盟硬皮病试验研究组（EULAR Scleroderma Trial and Research group,EUSTAR）的92例中国SSc患者,记录其临床表现和实验室检查结果,同时检测抗Scl-70抗体、ARA-Ⅲ、ACA和抗U1RNP抗体。统计分析上述自身抗体与患者各种临床特征之间的相关性。结果 92例患者中,弥漫型SSc65例,局限型SSc19例,重叠综合征6例,伴有雷诺现象的未分化结缔组织病2例。抗Scl-70抗体、ARA-Ⅲ、ACA和抗U1RNP抗体的阳性率分别为55%、13%、13%和25%。存在抗Scl-70抗体者具有较高的皮肤硬化修订Rodnan评分、免疫球蛋白（Ig）M及血小板水平,但肺动脉高压发生率、IgG水平则显著减低（P〈0.05）,与肺间质病变或肾危象的相关性未获证实。ARA-Ⅲ与皮肤硬化评分,毛细血管扩张,心、肺、肾脏受累等临床特点均无显著相关性。存在ACA的患者尿酸水平较高,但关节炎或关节痛症状和肺间质病变少见（P〈0.05）。存在抗U1RNP抗体者肺动脉高压和心脏受累的发生率、IgG和乳酸脱氢酶水平均增高,但白细胞和血小板较低（P〈0.05）。结论在SSc患者血清中检测上述硬皮病相关自身抗体有助于预测某些临床表现和脏器病变的发生。中国患者自身抗体的临床相关性可能与其他地区患者存在种族差异,值得进行更大样本的研究证实。     

ACUTE RENAL FAILURE – CHANGING TRENDS

A prospective study of 120 patients of acute renal failure was done at CH (EC) Calcutta to document the changing trends in the incidence and etiology of acute renal failure and to assess the prognostic factors. Mean age of patients was 33.7 ± 20.2 years. 75% had a medical risk factor. The etiological factors were volume depletion (13.3%), septicaemia (25.6%), falciparum malaria (16.6%), a transplant related renal failure (5.7%) and drugs (16.7%). Renal histology showed acute tubular necrosis to be the most common lesion (53.3%). In 13.3% patients who died with acute renal failure, associated infection and multisystem involvement was more common.KEY WORDS: Acute renal failure     

Morbidity in Alagille syndrome in 6 Malaysian children

We retrospectively studied the records of 6 Malaysian children who were diagnosed with Alagille Syndrome (AGS) according to this criteria from January 1999 to January 2001, at the Institute of Paediatrics, Kuala Lumpur Hospital. Four patients (66%) had a positive family history. Thirteen individuals (6 patients and 7 relatives) were diagnosed with AGS in these 5 families. Only 6/13 (46%) of them presented with liver involvement. All 6 patients presented with typical facies and cholestasis (100%). Three (50%) presented with portal hypertension (PHT) with synthetic liver dysfunction (1 died), 1/6 (17%) have PHT and normal synthetic liver function. Two have cleared their jaundice but have biochemical evidence of hepatitis and hepatomegaly, four have congenital heart disease 5/6 posterior embryotoxon, 2/6 butterfly vertebrae, 4/6 hyperlipidaemia and 4/6 failure to thrive. One patient has a Jagged-1 gene disruption at the translocation breakpoint locus 20p12.3 2n = 46,XX,t(12.20) (q22, p12.3). 5/6 (83%) are still alive. Two-thirds of our patients developed chronic liver disease by 3 years of age. Two-thirds of the index patients have a family history. Only 46% of individuals in these families have clinical evidence of liver involvement. Mortality depends on cardiac/renal disease, end-stage liver failure and intercurrent infection.     

The Effect of Urography on Renal Function in Patients with Multiple Myeloma

Reports in the medical literature of seven patients with multiple myeloma who died of acute renal failure following intravenous urography prompted a study of 39 patients with multiple myeloma who were subjected to intravenous urography at the Cleveland Clinic from 1940 to 1959. Four developed acute renal failure and two died within three weeks. All four revealed evidence of renal damage, or insufficiency, or both prior to urography. Thirty-five patients, 15 of whom had renal damage, had no untoward reaction to intravenous urography. These observations suggest that urography is associated with a small but definite risk in patients who have multiple myeloma and renal involvement.     

A profile of renal/renovascular abnormalities in patients of hypertension

After a thorough dinical examination and laboratory investigations, ultrasonography (USG) followed by minute sequence urography (MSU) and aortography and/or selective renal angiography were done in 108 patients, clinically suspected of having renal hypertension of which 32 patients (29.6%) were found to be positive for renal/renovascular disease. Out of 82 patients, who were only young hypertensives, a definite diagnosis of renal/renovascular disease could be made in only 15 cases (18.3%), whereas the pick up rate increased to 65.4% in the rest of the 26 patients who had some other clinical criteria besides hypertension and it further increased up to 92.3% (out of these 26 patients) who had 2 or more than 2 clinical criteria of selection of patients besides hypertension. USG was useful in diagnosing renal parenchymal disease/ suggesting renal abnormalities in 30 cases and MSU was able to detect renal changes in 31 patients. Aortography and/or selective renal angiography could make a definite diagnosis of renovascular disease in 17 cases and renal agenesis in one case.     

血液透析患者发生脑出血的危险因素及预后分析

目的:探讨慢性肾功能衰竭维持血液透析合并脑出血的危险因素及其预后。方法:回顾性分析2009年1月至2013年1月13例慢性肾功能衰竭维持血液透析合并脑出血患者的临床资料。结果:13例脑出血患者的危险因素包括高血压、高龄、糖尿病肾病、凝血机制异常等综合因素;13例患者中2例好转、11例死亡,病死率84%;存活患者多伴有不同程度的后遗症。结论:慢性肾功能衰竭维持血液透析并发脑出血患者的预后较差,伴有高血压病、糖尿病、凝血机制及血小板功能异常是慢性肾功能衰竭维持血液透析患者促发脑出血的危险因素。     

血液透析抢救小儿急性肾功能衰竭的临床体会

目的评价血液透析在抢救小儿急性肾功能衰竭(ARF)中的临床疗效。方法分析2010年1月至2011年12月间我院应用血液透析治疗52例小儿ARF的临床资料。结果全组52例,治愈42例(80.8%),好转8例(15.4%),死亡2例(3.8%),共发生并发症68例次,其中低血压42例次,失衡综合征16例次,高血压4例次,肌肉痉挛4例次,致热源反应2例次。结论血液透析是抢救小儿ARF的有效措施,能明显缓解病情,提高ARF患儿的生存率,减少并发症的发生。     

小儿紫癜性肾炎94例临床分析

本文对184例过敏性紫癜中合并肾炎的94例(51.1％)进行了分析,其主要临床表现为:皮肤紫癜(100％)、腹痛和(或)便血(66.0%),关节炎(60.0％)、尿改变(100％)、浮肿(23.4％)、高血压(16.0％)和肉眼血尿(7.4％)。近期治愈和好转率共87.2％。随访出院后1～13年的30例,临床痊愈24例、未愈4例、死亡2例。文中强调长期随访。临床表现兼有显著浮肿,肾号功不良及肉眼血尿者预后不良。     

Advanced systemic sclerosis complicated by pulmonary hypertension and complete atrioventricular block: a case report.

BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology characterized by fibrosis of the skin and visceral organs, in which the heart is frequently (40-70% of patients) and severely involved. Pulmonary hypertension affects 10-15% of patients with SSc and is one of the most important complications adversely influencing their survival. CASE REPORT: The case report presents a 59-year-old male patient with advanced systemic sclerosis whose initial examination revealed pulmonary hypertension, rhythm and atrioventricular conduction disturbances, and elevated level of NT-proBNP. After six months the patient deteriorated; an increase in NT-proBNP level and progression of pulmonary hypertension were observed. CONCLUSIONS: The described case is followed by a discussion of cardiovascular involvement in systemic sclerosis and emphasizes that heart involvement in SSc may have very serious clinical implications.     

抗U1核糖核蛋白抗体在系统性硬化症诊治中的临床意义

目的探讨抗U1核糖核蛋白（U1RNP）抗体在系统性硬化症（SSc）诊治中的价值。方法前瞻性序贯纳入在北京协和医院入选欧洲抗风湿病联盟硬皮病试验研究组（EUSTAR）的131例中国汉族SSc患者,记录其临床表现、脏器损伤和实验室检查结果（包括抗U1RNP抗体的检测结果）,分析抗U1RNP抗体阳性组与阴性组临床特征及实验室指标的特点。结果131例SSc患者中含87例弥漫型SSc、36例局限型SSc、8例重叠综合征,抗U1RNP抗体阳性率为28．2％（37／131）。弥漫型SSc和局限型SSc的抗U1RNP抗体阳性率相近,分别为28．7％（25／87）和25．0％（9／36）,P=0．673。抗U1RNP抗体阳性者较阴性者易重叠系统性红斑狼疮,肺动脉高压和心脏受累发生率增加,白细胞和血小板计数减低（均P〈0．05）;而在皮肤硬化评分及关节炎、肌炎、肺间质病变发牛率上差异无统计学意义（均P〉0．05）。抗U1RNP抗体阴性的SSc有更高的抗DNA拓扑异构酶Ⅰ抗体检出率。结论抗U1RNP抗体是中国SSc患者的常见自身抗体,与其他自身抗体联合检测有助于SSc的诊断,对预测肺动脉高压、心脏和血液系统受累有重要价值。     

IgA肾病合并高血压病88例临床病理特点分析

目的：研究合并高血压病的原发性IgA肾病（IgAN）的临床病理特点。方法：对2007年1月-2013年6月在我院肾内科经肾活检证实为IgAN且临床诊断高血压病的88例患者的临床病理资料进行回顾性分析。结果：IgAN合并高血压病患者平均年龄（44.7±11.27）岁。其中有高血压家族史48例,占54.5%。高血压病史（中位数为36个月）长于IgAN病史（中位数为2个月）,P＜0.01。临床表现主要为无症状尿检异常,共66例（占75.0%）;其次是肾病综合征和慢性肾炎（分别占10.2%和11.4%）。肾小球滤过率估算值（eGFR）＜90 mL/min 29例（占33.0%）,其中伴低尿渗透压6例（6/9）,伴尿N-乙酰-β-D氨基葡萄糖苷酶（NAG）升高21例（21/29）;伴左室壁增厚和（或）左房增大52例（52/87）;伴高血压视网膜病变76例（76/80）。肾活检病理显示Haas 1级～3级病变合计占87.5%,重度肾小球硬化占19.3%,中重度肾间质病变占34.1%,肾细小动脉病变占78.4%。结论：合并高血压病的IgAN患者肾脏临床病理表现多数较轻;部分存在肾小球滤过功能异常者多合并肾小管功能异常,部分病例较重的肾小管间质病变与肾小球病变程度不平行,普遍存在肾细小动脉病变。对于合并高血压病的IgAN病例而言,高血压可能是影响肾脏预后的主要因素。     

Raised blood urea in the elderly: a clinical and pathological study.

We have attempted to define a normal range for blood urea and creatinine for elderly inpatients and to determine the relative importance of pre-renal, renal and post-renal pathology in those with renal impairment. A total of 118 admissions to an acute geriatric unit and 67 separate post mortems in patients over 67 years of age were studied prospectively. Up to 123 items of data were coded and analysed including blood urea and creatinine, clinical or pathological changes associated with renal disease, clinical outcome and post mortem findings. We determined our own ''normal'' hospital ranges for urea (1.4-13.2 mmol/l) and creatinine (48-141 mumol/l) from plasma values in 76 patients with no evidence of renal impairment, either on admission or in the past. Using these values 41% of post mortem cases and 25% of clinical admissions had a raised blood urea. Pre-renal conditions such as cardiac failure, dehydration and gastrointestinal haemorrhage, either alone or in combination, were present in 56% of these patients. Urea and creatinine values were substantially higher in patients who died in hospital as opposed to those who were discharged or transferred. Creatinine values were greater in those with intrinsic renal disease or post-renal obstruction as compared to patients with pre-renal causes of renal impairment. Patients with histological evidence of extensive glomerulosclerosis or nephrosclerosis had higher urea and creatinine levels than those with only minor ageing changes.     

Renal replacement therapy in multiple myeloma and systemic amyloidosis

Renal failure frequently complicates both multiple myeloma and systemic amyloidosis. Renal replacement therapy (RRT) may be poorly tolerated and its role in such patients is not clearly defined. Of fifty patients (26 males and 24 females) referred to a single centre because of renal failure associated with multiple myeloma or systemic amyloidosis 37 progressed to end-stage renal failure and 30 of these patients received RRT. Nine patients have been treated by CAPD, 13 by haemodialysis, and 8 patients have required both forms of dialysis. Overall one year and two year survival rates were 66 % and 57 % respectively. The median duration on RRT was 7.5 months (range 1–96 months) with a 51% one year, and a 46% two year survival rate. Of 7 patients with amyloidosis who underwent renal transplantation, 3 died within 6 months of transplantation. Undiagnosed cardiac involvement contributed to this early mortality. We conclude that renal replacement therapy is appropriate for some patients with multiple myeloma and systemic amyloidosis who develop endstage renal failure. Careful asssessment and selection of patients is necessary prior to renal transplantation.     

Treatment and renal outcome of lupus nephritis: single center experience

Systemic Lupus Erythematosus (SLE) is a multisystemic autoimmune disease with renal involvement being one of the most frequent and serious manifestations of the disease. The aim of the study is to analyze the treatment and renal outcome of patients with lupus nephritis (LN) WHO class III and IV on cyclophosphamide (CYC). We retrospectively identified 41 patients with biopsy proven LN who was given either oral or intravenous CYC. The male: female ratio was 4:37; with a mean age of 31.7 +/- 9.8 years at presentation. 36 patients (87.8%) had LN class IV and only five patients (12.2%) with LN class III. The mean serum creatinine at presentation was 87.4 +/- 37.2 micromol/L with mean follow-up of 84 +/- 78 months. A total of 30 patients (73.2%) completed 12 courses of IV CYC and one patient (2.4%) completed three months of oral CYC. 71.0% (n = 22) had complete response (CR), 25.8% (n = 8) had partial response and 3.2% (n = 1) had no response (NR). Of the remaining 11 patients, two patients (4.9%) died during the treatment, three patients (7.3%) defaulted treatment and five patients (12.2%) are still receiving ongoing treatment. Presence of hypertension (p < 0.003) and evidence of chronicity on renal biopsy (p < 0.016) were significantly correlated with the progressive deterioration of renal function in our population. In conclusion, hypertension and evidence of chronicity on renal biopsy, proved to be risk factors for progressive renal impairment in our study population. The achieved global outcome can be considered good.     

LUPUS NEPHRITIS COMPLICATED WITH MALIGNANT HYPERTENSION： FROM RENAL VASCULAR PATHOLOGY TO CLINICAL RELEVANCE

Objective To investigate the clinical and pathological characteristics of lupus nephritis patients complicated with malignant hypertension. Methods We retrospectively studied 19 patients with lupus nephritis complicated with malignant hypertension who underwent renal biopsy between January 2002 and December 2006. Results Of 19 patients, 3 were men and 16 were women, with a mean age of 24.4±7.7 years old. All had posi-tive antinuclear antibodies and low serum complement was found in 13 patients. All were anemic and 12 of them were thrombocytopenic. Impaired renal function was found in 17 patients with an average serum creatinine of 184.5±88.9μmol/L. Severe intrarenal arteriolar lesion was found in all patients. Six patients had lupus vasculopathy, 11 patients had renal thrombotic microangiopathy lesion, 2 had severe arteriosclerosis. All patients received steroids and immuno-suppressive drugs, 15 received angiotensin-converting enzyme inhibitor （ACEI）/angiotensin receptor blocker （ ARB ） with resultant well-controlled blood pressure. Thrombocytopenia and hemolytic anemia resolved remarkably. The renal function improved or recovered in 14 of 17 patients, and 3 developed end-stage renal disease on mainte-nance dialysis. Conclusions Severe intrarenal vascular lesion complicated with renal nephritis parallels clinical manifestation of malignant hypertension. Renal pathology is the key of treatment strategy emphasizing on the significance of renal vascular involvement and type. On the basis of immunosuppressive drugs and steroids to control systemic lupus activity, timely initiation of ACEI/ARB could be of benefit to blood pressure control and long term renal survival.     

Systemic Vasculitis with Renal Involvement — A Review

Twenty five patients with renal vasculitis presenting over an eight year period were reviewed. Ten had microscopic polyarteritis, 6 classic polyarteritis, 5 overlap syndrome, 2 Churg-Strauss syndrome and 2 Wegener’s granulomatosis. Clinical features included hypertension, pulmonary involvement, neurological involvement and arthralgia. Serum creatinine was over 500 umol/1 in 13 patients, 10 of whom required dialysis. Visceral angiography was positive in 80% of those studied, Focal and segmental necrotising glomerulonephritis was the commonest renal lesion. Treatment consisted of corticosteroids and cytotoxic agents in most cases. Plasmapheresis was used for rapidly progressive renal failure, severe pulmonary haemorrhage or cerebral vasculitis. Improvement or stabilisation of renal function was seen in 68% of patients treated. There were 4 early deaths and one late death. The diagnosis, histology, treatment and outcome of renal vasculitis is discussed. The importance of early diagnosis and treatment is emphasised in this potentially reversible cause of acute renal failure.     

糖尿病肾病患者到肾科就诊时临床特点分析

目的分析糖尿病肾脏患者初次到肾病专科就诊时的临床特点,探讨糖尿病患者到肾病专科就诊的时机。方法回顾2006年1月—2008年6月首次到肾内科就诊,并取得较完整临床资料糖尿病肾病患者138例,男66例,女72例,平均年龄（61&#177;11）岁。观察到肾科就诊的原因、肾功能情况、并发症、治疗用药情况及随访情况等。结果87.15%的患者是由内分泌医生介绍转诊,就诊原因为肾功能衰竭64例（46.38%）,顽固性水肿54例（39.13%）,大量蛋白尿20例（14.49%）。就诊时52.17%需要进行肾脏替代治疗。就诊时合并症：高血压123例（89.13%）,贫血95例（68.84%）,冠心病56例（40.58%）,糖尿病心肌病53例（38.46%）,心功能不全49例（35.51%）,感染43例（31.16%）,心律失常24例（17.39%）,高血压心脏病18例（13.04%）。随访维持性血透58例患者至2008年6月已有17例死亡。结论大部分患者就诊较晚,部分已为肾功能衰竭,合并症多,影响长期肾脏替代治疗和寿命。糖尿病患者应早期到肾病专科就诊。     

Melioidosis: the Johor Bahru experience

A 5 year retrospective review of cases of melioidosis was carried out in Sultanah Aminah Hospital, Johor Bahru. There were 44 new cases of melioidosis which was proven by either blood or pus culture growing Burkholderia pseudomallei from the period between January 1999 and December 2003. Of these, 38 (86.4%) were males compared to only 6 (13.6%) females. Thirty-one (70.5%) were Malays, 7 (15.9%) were Chinese, 5 (11.4%) were Indians and 1 (2.2%) was a Sarawakian. The peak age group was between 50 and 59 years (31.8%). Out of these 44 new cases, only 32 medical records could be retrieved and analysed. Twenty-four out of 32 patients (75%) analysed had diabetes mellitus, 4 had chronic or end stage renal failure (CRF/ESRF) and only 1 had Human Immunodeficiency Virus (HIV). One case of "near drowning" was also recorded. Twenty-one out of 44 patients or 47.7% died, of which 8 (38.1%) died within 24 hours of admission. Pulmonary involvement was recorded in 62.6% of the patients but many had signs and symptoms of multiorgan involvement.