原发性干燥综合征肾损害的临床病理分析

目的：探讨原发性干燥综合征患者肾员害的临床表现、免疫学特点和肾脏病理改变。方法：回顾性分析14例原发性干燥征伴肾损害患者的临床表现、实验室指标、肾活检病理及免疫病理、肾组织肾小球及小管间质病变程度采用半定量积分表示。结果：（1）育龄期妇女发病多见，肾脏受累以低渗尿和肾小管酸中毒（RTA）最为常见（92．9％），其中I型RTA8例，Ⅲ型RTA2例，混合型RTA2例，不完全型RTA1例。尿蛋白阳性的患者均表现为小管源性蛋白尿，镜下血尿少见。（2）免疫系统异常，13例患者有多种自身抗体阳性，冷球蛋白阳性率为50％（6／12），血清球蛋白升高71．4％，IgG异常升高71．4％（8／11），IgM异常升高9．09％（1／11），IgA异常升高18．2％（2／11）。（3）肾活检病理改变11例为慢性间质性肾炎，3例肾小管间质基本正常，肾小球均有系膜增生，部分有C3（50％）、IgM(21.4%),IgG(14.3%)沉积。小管基膜和间质血管壁均无免疫球蛋白沉积。结论：干燥综合征伴肾损害以尿浓缩功能减低和肾小管酸中毒最为多见，肾小管间质病变为慢性间质性肾炎，血管病变发生率低，高球蛋白血症和小管间质的功能和形态改变有一定联系。     

范可尼综合征的临床特点与生化异常

目的 进一步认识范可尼综合征（FS）的病因、临床特点与生化异常。方法 对我院确诊的42例FS患者进行回顾性分析。结果 42例FS中完全型19例，不完全型23例，主要表现为近端肾小管酸中毒（41例）伴物质转运异常，包括低钾血症（21例）、低磷血症（29例）、低尿酸血症（19例）、肾性糖尿（38例）、氨基酸尿（36/37例）和低分子蛋白尿（21例）。病因：特发性或原因不明21例，继发性21例；其中间质性肾炎8例，干燥综合征5例。临床表现以乏力、多饮、多尿、肾性骨病最常见，伴肾功能不全者18例。14例患者接受肾活检，结果均显示有不同程度的小管-间质病变，其中4例伴有肾小球病变。结论 FS病因多样，继发性FS并不少见，一般均伴有Ⅱ型肾小管酸中毒，并常有肾小管-间质的病理损害。     

白塞病合并肾脏损害三例分析并文献复习

目的 分析白塞病合并肾脏病变的临床和肾脏活组织检查（简称：肾活检）组织学特点。方法 分析3例白塞病患者肾脏表现的临床、实验室检查及肾脏活检表现，对相关文献进行复习并与本研究结果对比。结果 3例患者出现肾脏病变时均伴有全身病变活动。2例表现为无症状蛋白尿，肾活检为肾小球病变；另1例表现为肾小管酸中毒和蛋白尿，肾脏活检为肾小管间质病变，3例均有不同程度的肾间质病变。所有3例的肾脏病变均较轻，经糖皮质激素和免疫抑制剂联合治疗后临床表现减轻。结论 白塞病肾脏受累在临床上不多见，肾脏损害以蛋白尿多见。病理上肾小球、肾小管及肾间质均可以受累，而肾血管则均未累及。本研究报道的3例患者均有肾间质病变，肾脏组织学损害较轻微。     

NIDDM肾病患者肾小管间质病变的研究

本研究着重观察了２６例非胰岛素依赖性糖尿病（ＮＩＤＤＭ）肾病患者肾小管间质病变的特点，包括肾小管功能的改变、肾组织学检查及部分患者的尿ＥＧＦ，对小管间质损伤在糖尿病肾病（ＤＮ）发生发展中的作用作了初探。结果发现：①ＮＩＤＤＭ肾病小管间质功能受损具有无明显节段性分布的特点，既有近端小管细胞损伤，也有远端小管的损伤；在形态学上表现为肾小管萎缩、基膜增厚、间质扩张、弥漫炎细胞浸润及不同程度的纤维化。②在肾小球滤过功能明显受损之前，小管间质病变即已出现，并随着肾功能的恶化而逐步加重，提示小管间质损害在ＤＮ的发生发展中起着与肾小球病变同样重要的作用。③肾功能未明显受损、小管间质病变程度较轻的ＤＮ患者，尿ＥＧＦ明显增高。随着肾功能的明显恶化，小管间质萎缩纤维化的加重，尿ＥＧＦ排泄量也逐渐降低，表明ＥＧＦ很可能参与了ＤＮ小管间质病变，在其损伤的早期阶段起了一定作用。     

TGF-β1与IgAN肾小管间质病变相关性研究

目的 探讨转化生长因子β1（TGF—β1）与IgA肾病（IgAN）。肾小管间质病变的关系。方法60例IgAN患者行肾活检,按小管间质损害程度积分分为无病变、轻度、中度及重度组。用ELISA法检测血、尿TGF-β1,同时检测其临床指标及肾小管功能指标。结果不同肾小管间质病变组间的肾小球积分、肾血管积分、血肌酐、肌酐清除率、24h尿蛋白定量差异有意义;肾小管间质病变与血、尿TGF-β1及尿仅,微球蛋白（α1-MG）、β2微球蛋白（β2-MG）、N-乙酰-β-氨基葡萄糖苷酶（NAG）呈正相关。结论血、尿TGF-β1及尿α1-MG、β2-MG、NAG可预示肾小管间质病变的进展和预后,其中尿TGF-β2与IgAN小管间质病变的相关性最强。     

木通中毒的肾脏损害

目的;了解中药木通中毒肾脏损害患者的临床病理特点及近期预后。方法：回顾性分析３例木通中毒肾脏损害患者的临床表现,肾活检病理资料及转归情况。结果：３例患者均是在服用１剂木通汤剂后发病,其中２例急性中毒,１例在服药６月才明显诊断,早期主要临床表现为：（１）消化道症状,非少尿型肾功能减退及低钾血症。（２）肾脏损伤除肾功能减退外,３例都有尿ＮＡＧ酶及溶菌酶升高,尿渗量降低及尿酸化功能异常,２／３例有肾性糖     

尿蛋白成分与选择分数预测肾小管间质损伤程度

蛋白尿、肾小管间质损伤与肾功能恶化的进展速度紧密联系。但临床上常有蛋白尿程度与小管间质损伤不平行的现象。蛋白尿的质（即尿蛋白成分）而非量可能在肾小管间质损伤过程中起重要作用，但目前尿中导致小管间质损伤的主要蛋白成分仍不确定。本研究通过检测成人原发性肾病综合征患者的尿蛋白成分，计算尿蛋白IgG选择分数（selective index，SI），分析其与小管间质损伤的关系，试图寻找可能反应肾脏病理损伤程度、预测肾功能恶化进展的非创伤性指标。     

范可尼综合征

范可尼综合征上海第二医科大学仁济医院（２００００１）张庆怡范可尼（Ｆａｎｃｏｎｉ）综合征是小儿及成人近端肾小管多种功能障碍的疾病，表现为：氨基酸尿、磷酸盐尿、葡萄糖尿、重碳酸盐丢失和分子量低于５００００ｄ的蛋白尿、肾小管性酸中毒及对后叶加压素抵抗的多...     

肾小管间质疾病中巨噬细胞浸润的机制及其意义

肾小管间质病变是慢性肾功能不全的重要病理表现，目前普遍认为肾间质炎症在其中发挥主要作用。单核、巨噬细胞（macrophage，MP）浸润是肾脏小管问质病变中的重要病理特征，已经证实巨噬细胞与肾脏固有细胞及细胞外基质（ECM）相互作用导致组织损伤，促使肾问质纤维化形成。有关MP与肾小管间质疾病关系的研究是目前国际肾脏病界研究的一个热点问题，本文就肾小管间质疾病中MP浸润的发生机制及其意义进行综述。     

由木通肾毒性研究带来的思考—提高对中草药肾毒性的认识

本期刊登了二篇有关木通肾毒性的研究及一篇马兜铃酸对肾小管细胞的体外观察。尹广等［１］报告了３例由单剂木通煎剂所造成的严重小管间质性损害病例，其后果都很严重，尽管只服了一剂木通（剂量１０～３０ｇ），但均出现了非少尿性急性肾衰，继之以长期的肾损害，在长达１／２～１年的随访中仍存在肾功能不全，肾组织出现了慢性小管间质性损害，即使经过积极处理，估计最后仍难免进入终末期肾衰。裘奇等［２］的实验研究报告进一步证实木通煎剂口服可导致大鼠急性肾功能衰竭，出现氮质血症及糖尿，肾脏病变以皮、髓交界处最为明显，近端小…     

Renal involvement and followup of 130 patients with primary Sjögren's syndrome

OBJECTIVE: To identify the clinical characteristics, pathological changes, and outcome of patients with primary Sj?gren's syndrome (pSS). METHODS: All patients with pSS and renal involvement who were admitted to Ruijin Hospital from April 1993 to December 2006 were included. All the data of clinical features and pathological changes were retrospectively analyzed. Forty-one patients underwent renal biopsies. RESULTS Our study included 130 patients with pSS: 122 women and 8 men. Ages ranged from 16 to 68 years (mean 44.1 +/- 11.52). Ninety-five patients (73.1%) developed renal tubular acidosis (RTA); 91 were found to have distal RTA. Nine patients presented with hypokalemic paralysis. Four patients developed Fanconi syndrome and 3 were proved to have nephrogenic diabetes insipidus. Twenty-seven of 130 patients (20.8%) developed tubular proteinuria and 18/130 (13.8%) presented glomerular involvement. Thirty-five patients (27.7%) developed renal failure (serum creatinine > 115 micromol/l). Most patients (70.8%) had increased serum IgG levels. The incidence of chronic interstitial nephritis was 80.5% among all the biopsy materials. Immunofluorescent staining was negative in most renal tissue. Ninety-six patients were treated with corticosteroids and/or immunosuppressant. Eighteen recovered renal function. CONCLUSION: Patients with pSS commonly present with renal impairment, mainly from renal tubular dysfunction. The combination of corticosteroids and immunosuppressors significantly improves the renal function of patients with pSS. There is a correlation between hypergammaglobulinemia and distal RTA. The renal acidification capacity for patients with hypergammaglobulinemia. should be monitored.     

Hypophosphatemic osteomalacia and adult Fanconi syndrome due to light-chain nephropathy. Another form of oncogenous osteomalacia

Two patients, one with myeloma (Patient 1) and the other with probable chronic lymphocytic leukemia (Patient 2), had reduced renal tubular phosphate reabsorption in the absence of hyperparathyroidism together with other features of the Fanconi syndrome, as consequences of the nephropathy associated with light-chain proteinuria. Both patients had hypophosphatemic osteomalacia, demonstrated for the first time in this condition by iliac bone histomorphometry after in vivo double tetracycline labeling, despite absence of bone pain or Looser zones. Neither patient was vitamin D-depleted, but plasma calcitriol level was normal in Patient 1 and low in Patient 2; only the latter patient had severe muscle weakness. Complete histologic correction of osteomalacia was achieved by treatment in accordance with the biochemical defects--oral phosphate therapy alone in Patient 1 and combined with calcitriol in Patient 2. Both patients are now symptom-free, five and three years after the initial diagnosis of bone disease and hematogenous malignancy. Thirteen previous instances of the same form of osteomalacia were reviewed; in most cases, the Fanconi syndrome developed before its probable cause became apparent. The Fanconi syndrome has also been reported in two cases of osteomalacia due to mesenchymal tumor, but not in osteomalacia associated with prostatic carcinoma. Light-chain nephropathy and consequent renal tubular dysfunction appears to be a third form of oncogenous osteomalacia.     

轻链沉积病肾损害的临床和病理分析

目的：了解轻链沉积病(LCDD)肾损害的临床和病理特征。方法：回顾性分析26例经临床和肾活检确诊的LCDD患者的临床和病理改变。结果：26例LCDD患者起病时的平均年龄为49.4岁(27—72岁)，其中男性21例，女性5例。8例确诊为多发性骨髓瘤(MM)，2例伴有浆细胞异常增生，另有16例病因不明。临床表现为急性肾衰3例(11．5％)，慢性肾衰17例(65．4％)，肾病综合征17例(65．4％)，15.4％的患者起病时即需行肾脏替代治疗。实验室检查发现血清和尿液游离κ、λ轻链的阳性率分别为56．5％和91．7％。肾脏病理改变以系膜结节样病变多见，占53．8％，2例表现为膜增生样病变、1例为膜性病变，中—重度小管间质慢性化病变为本组患者较特征性的病变(92．3％)。肾组织中以λ轻链沉积为主者占65．4％(17例)，κ轻链沉积占34．6％(9例)。18例患者行肾组织电子显微镜检查，均显示肾小球和(或)肾小管基膜内(外)侧不规则的纤细颗粒样电子致密物。结论：LCDD患者临床以肾功能不全伴肾病综合征多见，部分患者合并浆细胞增生性疾病。病理以系膜结节样伴严重的小管间质病变为特征。血清、尿液以游离λ轻链增高多见，肾组织中也以λ轻链沉积居多。     

Acute renal failure following snakebite.

Eight patients with acute renal failure following snakebite were studied. Intravascular hemolysis and disseminated intravascular coagulation contributed to the development of acute renal failure in 6 patients. Direct nephrotoxicity causing acute renal failure is postulated in 2 patients, 1 of whom also revealed evidence of mild, disseminated intravascular coagulation. Three patients had histopathological lesions of acute symmetrical cortical necrosis and 3 had acute tubular necrosis. In 1 patient with acute tubular necrosis, in whom direct nephrotoxicity seemed to be responsible for renal failure, the striking histological feature was a uniform debasement and disappearance of tubular epithelium. In 2 patients with a clinical course of acute tubular necrosis, histological lesions could not be documented. All the 5 patients with acute tubular necrosis regained full recovery of renal function, 3 of them with the help of dialysis and 2 with conservative management. None of the 3 patients with acute cortical necrosis survived in spite of intermittent dialysis therapy.     

Tenofovir-associated renal and bone toxicity

Objectives The aims of the study were to describe the clinical presentation and renal and bone abnormalities in a case series of HIV-infected patients receiving treatment with tenofovir (TDF), and to recommend appropriate screening for toxicity related to TDF.
Methods Patients were identified from referrals to a specialist HIV renal clinic. Patients were included if treatment with TDF was assessed as the primary cause of the renal function impairment and clinical data were available prior to and following discontinuation of TDF treatment. Data were collected from case note review and clinic databases.
Results Twenty-two patients (1.6% of all those who received TDF) were identified with TDF-associated renal toxicity. All had normal serum creatinine prior to TDF therapy. All presented with proteinuria. On stopping TDF, renal function improved. Eight patients had confirmed Fanconi syndrome. Twelve patients presented with bone pain and osteomalacia was confirmed on an isotope bone scan in seven of these patients. The findings (in those patients tested) of tubular proteinuria, reduced tubular transport maximum of phosphate (TmP), and glycosuria were all consistent with the proximal tubule being the site of toxicity.
Conclusion Renal toxicity remains a concern in patients treated with TDF. Clinical presentation may be with renal dysfunction, Fanconi syndrome or osteomalacia. Our investigations suggest proximal tubular toxicity as a common pathogenic mechanism.     

Reversible renal glycosuria in acute interstitial nephritis

ABSTRACT:: Renal glycosuria is defined as the excretion of glucose in urine in a normoglycemic state. It results from renal tubular dysfunction or immaturity of tubular function in the newborn. Etiologically, renal glycosuria is of 3 types-benign renal glycosuria, glycosuria with diabetes mellitus (including gestational diabetes) and tubular defects (Fanconi syndrome). Prognosis of benign renal glycosuria is excellent and reversible. Acute interstitial nephritis (AIN) is one of the main causes of acute renal failure and may often result in tubular dysfunction. In this study, the authors report the occurrence of AIN with acute renal failure that contributed to reversible renal glycosuria. The glycosuria observed in the patient of this study was an isolated tubular defect, with no phosphaturia, aminoaciduria or bicarbonaturia. Such a presentation is very rare in adults and has not been previously reported. These findings confirm that AIN with acute renal failure can cause an isolated tubular defect with benign reversible glycosuria in an adult.     

Hepatorenal syndrome associated with obstructive jaundice

In almost all cases of acute renal failure associated with cholestatic jaundice, the occurrence of renal failure is preceded by episodes of shock, hypotension, sepsis, or surgical intervention. The pathologic finding is usually that of acute tubular necrosis. A patient with obstructive jaundice developed renal failure; the clinical and pathologic features were consistent with those found in the hepatorenal syndrome. No episodes of shock or sepsis preceded the onset of that renal failure. At autopsy, the findings were normal.