原发性膜增生性肾炎的远期预后及影响因素分析

目的 :前瞻性观察原发性膜增生性肾炎 (MPGN)患者的自然病程 ,了解其远期预后及影响预后的各种因素　 方法 :1982年 1月～ 2 0 0 0年 12月间 ,在本院经肾活检确诊并常年定期随访的MPGN 74例 ,Ⅰ型和Ⅲ型MPGN各 37例。以SCr>130 μmol/L作为肾功能损害 ,SCr>80 0 μmol/L确定为终未期肾衰 (ESRF) ,并用Kaplan Meier方法计算患者的肾功能损害发生率及肾脏存活率　 结果 :在平均随访 48 6 (13～ 15 2 )月后 ,共有 43例 (5 8 1%)患者发生程度不等的肾功能损害 ,其中ESRF的发生率为 16 2 %。Ⅰ型MPGN有 2 7例出现肾衰 (73 0 %) ,Ⅲ型MPGN患者中 16例发生肾功能损害 (4 3 2 %) ,两者肾功能损害发生率相比差异显著 (P <0 0 5 )。进一步用Kaplan Meier方法计算 ,得出本组MPGN患者 5年和 8年的肾存活率分别为 90 %和 6 4%。按患者的临床症候群将其分为高血压型(HT)、肾病综合征型 (NS)及非肾病综合征型 (non NS)三组进行比较 ,HT组患者病情进展最快 ,而NS组与non NS组患者无明显差别。进一步将患者分为大量蛋白尿 (尿蛋白 >3g/ 2 4h)与非大量蛋白尿 (尿蛋白 <3g/ 2 4h)两组进行分析 ,显示大量蛋白尿者的疾病进展速度快 ,远期预后差。观察同时发现 ,长期激素治疗患者的肾存活率与非激素治疗者相似 ;ACEI治疗组肾     

Mutations of the human polycystic kidney disease 2 (PKD2) gene

Autosomal dominant polycystic kidney disease (ADPKD) is an inherited nephropathy, usually of late onset (onset between third to seventh decade), primarily characterized by the formation of fluid‐filled cysts in the kidneys. It is one of the most frequent inherited conditions affecting approximately 1:1,000 Caucasians. Two major genes have been identified and characterized in detail: PKD1 and PKD2, mapping on chromosomes 16p13.3 and 4q21‐23, respectively. A third gene, PKD3, has been implicated in selected families. Polycystic kidney disease of types 1 or 2 follows a very similar course of symptoms, both being multisystem pleiotropic disorders of indistinguishable picture on clinical grounds. The only difference is that patients with PKD2 mutations run a milder course compared to PKD1 carriers, with an average 10–20 years later age of onset and lower probability to reach end‐stage‐renal failure. The proteins polycystin‐1 and ‐2 are trans‐membranous glycoproteins hypothesized to participate in a common signaling pathway, interacting with each other and with other proteins, and coordinately expressed in normal and cystic tissue. Renal cysts most probably arise after a second somatic event, which inactivates the inherited healthy allele of the same gene, or perhaps one of the alleles of the other gene counterpart, generating a trans‐heterozygous state. This article reviews the reported mutations in PKD2. Mutations of all kinds have been reported over the entire sequence of the PKD2 gene, with no apparent significant clustering and with some evidence of genotype/phenotype correlation. Most families harbor their own private mutations but a few recurrent events have been reported in unrelated families. Hum Mutat 18:13–24, 2001. © 2001 Wiley‐Liss, Inc.     

The egocentric spatial reference frame used in dorsal-lateral prefrontal working memory in primates

The dorsal-lateral prefrontal cortex (dlPFC) has been proposed to be the site of spatial working memory (WM), and this concept has had a profound influence on functional studies of the prefrontal cortex (PFC). The concept of spatial WM has been understood to mean that the location of an object is memorized for a short period of time. However, this concept of space is a simplification. To process the spatial information, different spatial frames can be used. In this review, the authors present data from their own laboratory to argue that the dlPFC is related to the egocentric spatial information processing (ESIP) in WM. The goal of this review is to introduce and discuss the egocentric spatial reference frame (ESRF) located in the dlPFC. The ESIP in the PFC might be involved in self-recognition.     

Potassium disorders--clinical spectrum and emergency management

Potassium disorders are common and may precipitate cardiac arrhythmias or cardiopulmonary arrest. They are an anticipated complication in patients with renal failure, but may also occur in patients with no previous history of renal disease. They have a broad clinical spectrum of presentation and this paper will highlight the life-threatening arrhythmias associated with both hyperkalaemia and hypokalaemia. Although the medical literature to date has provided a foundation for the therapeutic options available, this has not translated into consistent medical practice. Treatment algorithms have undoubtedly been useful in the management of other medical emergencies such as cardiac arrest and acute asthma. Hence, we have applied this strategy to the treatment of hyperkalaemia and hypokalaemia which may prove valuable in clinical practice.     

Information and counselling for patients approaching end‐stage renal failure in selected centres across Europe

The EDTNA/ERCA survey of the provision of pre‐ESRF information, education and counselling in renal care was the third project organised through the Collaborative Research Programme (CRP). Data was collected from 35 participating centres in 10 countries. The majority of participating centres had a structured pre‐ESRF programme. Advice and education received by patients in these centres was usually provided by a multidisciplinary team and was coordinated by nurses in half of the centres. Programmes in all countries had similar content in that normal kidney function, medication, diet, haemodialysis, peritoneal dialysis and transplantation were routinely discussed and that issues such as employment, importance of leisure activities, holidays and Kidney Patient Associations were also on the agenda for discussion. Educational material produced by companies was widely used. Policy differences did emerge in attitudes to discussing the “no‐treatment” option with patients accepted for dialysis and in the treatment that would be offered to patients with specific co‐morbidities and life‐style.     

终末期肾功能衰竭透析病人的心理问题研究

采用综合性医院焦虑抑郁量表（ＨＡＤ）、Ｈａｍｉｌｔｏｎ抑郁和焦虑量表（ＨＡＭＤ、ＨＡＭＡ），以及日常生活能力量表（ＡＤＬ）对上海市三所综合性教学医院肾病科住院透析病人进行了透析前后对照研究，同时对这些病人采用ＣＣＭＤ－２的有关诊断标准进行临床诊断性会谈。结果发现，透析病人有较高的心理障碍罹患率（５７．４５％），其中透析前病人的心理问题更为多见，抑郁、焦虑症状的罹患率达８２．９８％。透析后病人的各量表评分均有显著下降（Ｐ＜０．０５），提示透析治疗本身对病人的心理状况有一定的影响。另外，对ＨＡＤ（自评量表）与ＨＡＭＡ、ＨＡＭＤ（他评量表）进行了一致性分析，发现ＨＡＤ与后两者之间有较好的相关性（ｒ＝０．７６和０．８２，Ｐ＜０．０５），提示ＨＡＤ是一个较可靠的量表。     

Predictors of complications following retroperitoneoscopic total and partial nephrectomy

Introduction

Recent reports in the literature suggest an increased risk of complications with retroperitoneal as opposed to transperitoneal approach to partial nephrectomy (PN) and total nephrectomy (TN). We are a large unit performing predominantly retroperitoneoscopic PN and TN. We aim to review our outcomes and perform analysis to elucidate the predictors of complications following the retroperitoneal approach for extirpative kidney surgery.

Spectrum of renal bone disease in end-stage renal failure patients not yet on dialysis.

BACKGROUND: During the last few years the spectrum of renal osteodystrophy (ROD) in dialysis patients has been studied thoroughly and the prevalence of the various types of ROD has changed considerably. Whereas until a decade ago most patients presented with secondary hyperparathyroidism (HPTH), adynamic bone (ABD) has become the most common lesion within the dialysis population over the last few years. Much less is known about the spectrum of ROD in end-stage renal failure (ESRF) patients not yet on dialysis. METHODS: Transiliac bone biopsies were taken in an unselected group of 84 ESRF patients (44 male, age 54+/-12 years) before enrolment in a dialysis programme. All patients were recruited within a time period of 10 months from various centres (n=18) in Macedonia. Calcium carbonate was the only prescribed medication in patients followed up by the outpatient clinic. RESULTS: HPTH was found in only 9% of the patients, whilst ABD appeared to be the most frequent renal bone disease as it was observed in 23% of the cases next to normal bone (38%). A relatively high number of patients (n=10; 12%) fulfilled the criteria of osteomalacia (OM). Mixed osteodystrophy (MX) was diagnosed in 18% of the subjects. There was no significant difference between groups in age, creatinine, or serum and bone strontium and aluminium levels. Patient characteristics associated with ABD included male gender and diabetes, whilst OM was associated with older age (>58 years). CONCLUSIONS: In an unselected population of ESRF patients already, 62% of them have an abnormal bone histology. ABD is the most prevalent type of ROD in this population. In the absence of aluminium or strontium accumulation the relatively high prevalence of a low bone turnover as expressed by either normal bone or ABD and OM is striking.     

Efficacy of Hemopoietic-Stimulating Factors in Patients Undergoing Chronic Hemodialysis

Anemia has been linked to increased mortality and morbidity in renal hemodialysis patients. Other risk factors that contribute to an adverse outcome include the variability of hemoglobin (Hb) levels and the decreased response to erythropoiesis-stimulating factors (ESFs). In this study we evaluated the effectiveness of four different ESFs (epoetin-A, epoetin-B, darbepoetin, and CERA), assessed the variability of Hb levels, and compared ESF dosages which contributed to the achievement of Hb levels in each individual patient with renal failure undergoing chronic hemodialysis maintenance. In conclusion, the four ESFs administered are equally effective in the treatment of anemia in renal hemodialysis patients and they do not influence in a different manner the variability of Hb. The administration of darbepoetin-A and CERA might possibly cause more patients to overshoot the target level of Hb.