左侧多发性肾上腺囊肿并左侧重复肾双输尿管畸形1例

目的探讨多发性肾上腺囊肿合并重复肾双输尿管畸形的诊断、鉴别诊断和治疗。方藩回顾分析1例左侧多发性肾上腺囊肿并左侧重复肾双输尿管畸形的临床资料。并结合文献进行讨论。结果术前联合应用B超、CT检查，诊断为左肾上腺多发囊肿；行IVU检查。进一步发现了重复肾双输尿管畸形，避免了漏诊和误诊。行后腹腔镜肾上腺囊肿探查术。术中见左肾上腺有2个囊肿。因术前CT及术中均可见囊壁钙化，术中行冰冻病理检查诊断为假性囊肿，行囊肿切除术。随访18个月未见复发，结论治疗方法取决于囊肿大小、重复肾的功能、是否有并发症等。对囊壁有钙化者，术中应行冰冻病理检查以确定有无恶性病变。后腹腔镜肾上腺囊肿切除术是外科首选的治疗方法。     

肾周尿囊肿的诊断与治疗

目的探讨肾周尿囊肿的早期诊断与合理治疗。方法回顾分析６例肾周尿囊肿：５例有外伤史，１例有输尿管结石、肾积水史，术前经Ｂ超、逆行肾盂造影（ＲＰＧ）、ＣＴ检查确诊，２例ＲＰＧ检查发现瘘管。结果手术治疗４例：肾切除１例，囊肿去顶术３例，其中２例加瘘管切除；２例经皮穿刺引流后治愈。术后随访４个月～７年，囊肿无复发。结论肾周尿囊肿经Ｂ超筛选，结合ＲＰＧ、ＣＴ检查可明确诊断。早期发现且无输尿管梗阻者首选囊肿穿刺引流治疗，有瘘管或有输尿管梗阻者宜手术治疗原发病，同时处理囊肿并切除瘘管。     

38例原发性干燥综合征并肾脏损害的病理与临床分析

目的 结合肾活检及相关实验室检查，探讨原发性干燥综合征(PSS)肾脏损害的临床及病理特征。方法 38例PSS肾脏损害者进行肾小球功能、肾小管功能及部分肾活检检查。结果 25／38例为肾小管性肾中毒(RTA)，2／38例合并低钾性麻痹，表现为肾病综合征及肾小球肾炎(GN)分别为7例及4例，7月8例有轻度肾功能衰竭。结论 PSS肾损害常见，以RTA和GN为主要表现，泼尼松治疗可以减少间质淋巴细胞和浆细胞浸润，改善肾功能，纠正RTA。     

少年型肾单位肾痨Ⅰ型临床特点和基因诊断

目的:探讨少年型肾单位肾痨Ⅰ型(NPHP1)的临床特点和基因诊断方法。方法:2016年7月我院肾内科收治1例约一年内快速进展为终末期肾脏病、接受规律透析的男性患儿(12岁),详尽分析和收集其临床资料,并利用二代测序技术对该患儿进行基因检测。结果:患儿并无特殊的临床表现,无高血压、无浮肿,尿沉渣检查显示:无明显血尿和尿蛋白定性检查常为阴性。但尿β_2-MG、尿α_1-MG和尿视黄醇结合蛋白等小分子尿蛋白显著增加,尿蛋白电泳有较高比例的小分子肾小管尿蛋白(13.8%),尿比重低(0.015),显示明确的肾小管间质损伤。双肾彩超和CT检查,双肾大小、形态如常,边缘轮廓欠清,双肾均有多个囊肿。显著的肾小管间质损伤和肾脏多发囊肿,符合既往多数NPHP1报道的临床和医学形象学的特征。基因检测结果患儿的样本NPHP1整体纯合缺失,该变异为致病性变异。其父母均检测到NPHP1的杂合变异,但无任何临床表现。基因检测结果和临床表现均符合少年型肾单位肾痨1(NPHP1),为常染色体隐性遗传。结论:NPHP1是常染色体隐性遗传病,除显著肾小管间质损伤和双肾囊肿外,临床表现常无特异性,故提高对NPHP认识和警惕,加强基因检测是防止误漏诊,对提高我国终末期肾脏病病因的精准诊断和合理防治尤为重要。     

肾周尿囊肿3例报告并文献复习

目的探讨肾周尿囊肿的诊断和治疗。方法报告3例肾周假性尿囊肿的诊治经验,结合文献对其病因、临床表现、诊断和治疗进行讨论。结果 3例均有肾或输尿管损伤史,术前均行B超、CT及静脉尿路造影（IVU）检查,诊断为肾周囊性包块,行手术治愈,术后随访6个月~2年无复发。结论肾周尿囊肿大多数有明确外伤史或肾及输尿管结石梗阻史,术前首选B超检查,结合CT、IVU或逆行肾盂造影（RPG）可明确诊断,根据有无输尿管梗阻及有无瘘管形成选用不同的治疗方法,能取得良好的治疗效果。     

肾外肾盏1例

患者 ,男 ,2 8岁。因左肾区胀痛不适 5年于 1 999年 1 1月 2 2日入院。患者自 5年前间歇出现不明原因的左肾区疼痛 ,无放射 ,每次持续数分钟至数天不等 ,服用一般止痛药效果不佳。无血尿及膀胱刺激症状。体检 :一般情况可 ,双肾未扪及 ,左肾区轻度叩击痛 ,输尿管行径无压痛。尿常规偶见红细胞 ,尿培养阴性 ,尿细胞学检查阴性。B超 :左肾大小正常。肾门部可见一 8cm× 6cm× 6cm囊性肿物 ,边缘清 ,囊内回声均质。CT提示左肾内侧囊性肿物。IVU示左肾大小正常 ,肾盂扩张 ,大小约 7cm× 5cm,肾盏呈细管状 ,无明显扩张 ,肾盂输尿管连接处狭窄…     

经皮肾穿刺获取肾组织中无肾小球的原因探讨（附303例报告）

经皮肾穿刺活检（PRB）简称肾穿刺,自1951年首次开展以来,经过50多年的发展,尤其是B超实时引导及自动活检枪的应用,PRB技术已日臻完善。但是,肾穿刺有时仍会发生取材不良的情况,取出的肾组织为肾髓质和（或）皮髓质交界处的组织,无或很少有肾皮质,也无肾小球。这让很多临床和病理医师、患者及其家属困惑不解。为何会发生这种情况呢？检索国内外文献,尚未见分析报道。为此,我们拟对这一状况进行探讨。     

髓质海绵肾的诊断和治疗（附8例报告）

目的 探讨髓质海绵肾的临床特点及其诊断、治疗原则。方法 回顾性分析8例髓质海绵肾患者的临床资料。结果 8例患者经对症治疗后临床症状均得到缓解，无1例死亡。结论 髓质海绵肾的主要临床表现为肾绞痛、血尿及尿频。CT能早期发现肾锥体内细小结石及肾集合管扩张，腹部平片（kidney ureter bladder，KUB）及静脉尿路造影（intravenous urography，IVU）可以确诊。治疗以保肾、解痉利尿、防治泌尿系感染等措施为主。     

狼疮肾炎临床与病理相关性分析

目的：探讨狼疮肾炎（LN）的临床表现与病理类型之间的关系。方法：对近5年来收治的159例系统性红斑狼疮（SLE）的病例临床特征进行回顾性分析，并重点对已经在B超或彩超引导下行经皮肾穿刺活检术，光镜、免疫组化及电镜检查，取得病理检查结果的48例LN进行统计分析。结果：48例中，男5例，女43例，男女比例为1：9；临床类型：亚临床型2例，肾炎综合征22例及肾病综合征24例；病理类型：Ⅱ型9例，Ⅲ型5例，Ⅳ型19例，V型13例，Ⅵ型2例；肾小管间质轻度损害35例，中度损害10例，重度损害3例。LN的病理类型与临床表现有一定的相关性，临床活动性（SLEDAI）与病理类型无相关性。结论：肾活检有助于对系统性红斑狼疮肾损害进行明确诊断，有助于了解其病理活动性、慢性指数及肾小管间质损害程度，有助于选择最佳的治疗方案和判断预后。从临床表现和实验室检查可初步推测病理类型，但临床活动性与病理活动性存在不一致性；病理活动性及慢性评分能更准确了解肾损害情况。     

移植肾功能恢复延迟受者供肾及移植肾活检的意义

目的 分析移植肾功能恢复延迟(DGF)受者进行供肾及移植肾活检对其病因诊断的价值及对治疗的指导意义.方法 回顾性分析144例DGF受者的临床表现、实验室检查特点.获取供肾进行修肾时行供肾活检,在B型超声引导下行经皮肾活检术检查移植肾,分别行组织学、免疫病理和超微病理检查.结果 (1)1994-1997年间DGF的发生率为10.16%,1998-2001年间为7.83%,2002-2005年间为7.48%,2006-2009年降至5.35%;(2)DGF受者的临床表现包括无尿(16.67%)、少尿(16.67%)和高血压(47.22%).123例行移植肾B型超声检查者中肾脏体积增大者占79.67%,血管阻力增高者占45.53%;(3)全部DGF受者均存在血肌酐(SCr)不降或下降缓慢,80例SCr为451～707μmol/L,23例SCr持续＞707μmol/L.70.83%的DGF受者尿N-乙酰-BD-氨基葡萄糖苷酶升高,54.86%的DGF受者尿蛋白定性阳性,53.47%的DGF受者尿沉渣镜检红细胞计数＞50万/ml.(4)144例中,发生急性排斥反应者占45.83%,发生钙调磷酸酶抑制剂肾毒性者占15.28%,IgA肾病者占12.50%,缺血再灌注损伤者占7.64%,移植肾组织学形态正常者占7.64%,急性肾小管坏死者占5.56%,急性间质性肾炎3.47%,移植后复发性疾病占1.39%,肾小球毛细血管内增生性病变占0.69%.(5)60.55%的受者除变更免疫抑制方案外,还进行了肾脏替代治疗.结论 尽管[GF的原因复杂,但供肾质量及移植肾早期病理改变与DGF有直接关系;移植肾活检有助临床更改治疗方案.     

Renal retinal dysplasia with diffuse glomerular cysts

A 32-year-old male with renal retinal dysplasia is presented. He also showed hearing loss and growth retardation. Laboratory data showed mild proteinuria, renal dysfunction and type-1 renal tubular acidosis. Computed tomography showed multiple cysts at the corticomedullary junction of both kidneys. Ocular examinations disclosed retinitis pigmentosa. On light microscopy of renal biopsy specimens, diffuse cystic dilatation of Bowman's space as well as dilated tubules with interstitial fibrosis and cellular infiltration were noted. Electron microscopy revealed a peculiar chromatin condensation of epithelial cell nuclei in Bowman's capsules, glomeruli and tubules. The association of renal retinal dysplasia with diffuse glomerular cysts has not, to our knowledge, been previously reported.     

Ehlers-Danlos syndrome coexisting with juvenile nephronophtisis

Ehlers-Danlos syndrome (EDS), a heterogeneous disease of the connective tissues, is diagnosed by a triad of symptoms that include skin hyperextensibility, joint hypermobility and connective tissue fragility. Nephronophtisis (NPH) is an autosomal recessive interstitial nephritis leading to terminal renal insufficiency around puberty. The occurrence of these two rare diseases together is unusual. A review of the literature discloses no case of this association. We report here on a 16-year-old man with undiagnosed EDS, who was referred to our hospital because of renal insufficiency, history of polyuria and polydipsia. Renal ultrasound showed normal kidney size, with a lack of corticomedullary differentiation. Renal biopsy specimen disclosed chronic tubulointerstitial nephritis resembling NPH. Further evaluation identified hypermobile joints and hyperextensible skin, which led to the diagnosis of the EDS. These data suggest that patients with EDS need to be evaluated carefully for the presence of renal anomalies.     

Unique changes in interstitial extracellular matrix composition are associated with rejection and cyclosporine toxicity in human renal allograft biopsies

Renal allograft loss from chronic rejection or cyclosporine toxicity (CsAT) is characterized by progressive interstitial fibrosis, yet the protein composition of these lesions is unknown. The normal tubular basement membrane (TBM) contains laminin (LM), collagen IV (containing collagen IV alpha chain 1 [COL4A1] and COL4A2), thrombospondin (TSP), and fibronectin (FN). Only TSP and FN extend beyond the TBM into the interstitial space. Very scanty amounts of interstitial collagens (I and III) are detected in the interstitium. In a pilot study of human renal allograft biopsy specimens, three patterns of extracellular matrix (ECM) composition were identified. Pattern 1 showed no change in ECM composition; pattern 2 showed generalized accumulation of collagens I and III in the interstitium; and pattern 3 showed new expression of COL4A3 and LM-beta2 in the proximal TBM. Criteria were established for the clinicopathological diagnosis of CsAT and rejection. These diagnoses were correlated with the ECM composition in 22 renal allograft biopsy specimens. Control groups were examined in a similar manner and included native kidney biopsy specimens from patients with other allografts (n = 7), renal biopsy specimens from patients with glomerular disease (n = 9), and renal allograft biopsy specimens from patients without clinicopathological evidence of renal disease. These data show that rejection is associated with pattern 3 and CsAT is associated with pattern 2. Thus, detection of ECM composition may be a useful adjunct to standard microscopy in distinguishing rejection from CsAT in renal allograft biopsy specimens. These data suggest that interstitial fibrosis associated with rejection and CsAT result from different pathogenic mechanisms.     

Perirenal capsule involvement in IgG4-related chronic interstitial nephritis: a case report and literature review

Objective To explore the clinicopathological features and the renal biopsy process of a case of IgG4-related chronic interstitial nephritis with perirenal capsule involved and review associated literature to improve the clinician's understanding for this disease and to perform a better renal biopsy. Methods The onset, diagnosis and treatment course of the disease were described and associated literature were reviewed to summary the clinicopathologic features and key points in renal biopsy. Results The data of the patient showed that the urine specific gravity was 1.011, with urine protein ± and urine sugar 3+. The concentration of hemoglobin was 53 g/L, serum creatinine was 1665 μmol/L, and IgG4 was 9.39 g/L. Computed tomography showed that both kidneys enlarged slightly with decreased density and low density shadow around the kidneys. On contrast-enhanced scan, irregular low-density enhancement areas were found in both kidneys, and the edge of the boundary was not clear. For the first renal biopsy, no renal parenchyma was found except mainly hyaline collagen fibrils. At the second time, 3 pieces of tissues were obtained, which showed chronic interstitial glomerulonephritis. The IgG4 positive plasma cells were about 60/HPF and the IgG4+/IgG+cells ratio was more than 40%. The diagnosis of IgG4-related chronic interstitial glomerulonephritis was confirmed. After corticosteroid treatment, the serum creatinine decreased to 502 μmol/L after the patient got rid of dialysis. Conclusions There are various manifestations of renal damage caused by IgG4-related disease. It is necessary to pay attention to the involvement of the perirenal capsule, and to balance the risk of bleeding and poor sampling in renal biopsy.     

Isolated sarcoid renal granulomatous tubulointerstitial disease

A 37-year-old lady presented with hypercalcemia and acute renal impairment. She had no previous medical problems apart from the use of non steroidal anti-inflammatory drugs for nonspecific body pains. Her abdominal ultrasound scan as well as urine studies were nonspecific. Further workup for hypercalcemia (skeletal survey, high resolution computed tomography (CT) of the chest and abdomen, purified protein derivative (PPD) test, serum protein electrophoresis, tumor markers, immunology screening, and Bence Jones proteinuria) was negative. Serum angiotensin converting enzyme was high. Renal biopsy showed extensive lymphocytes and multinucleated giant cells infiltration forming interstitial non necrotizing granulomata. Immune staining as well as staining for acid fast bacilli was negative. The possibility of sarcoid renal granulomata was raised and the patient was started on oral prednisolone with subsequent normalization of renal functions and serum calcium after one month of treatment.     

A case of pseudo-Bartter's syndrome with marked nephrocarcinosis

A 38-year-old woman was admitted to our hospital on October 21, 1996 for evaluation of thirst, bilateral backache and a feeling of abdominal fullness. She had hypokalemia, normotension, hyperreninemia, hyperaldostronism and hyperplasia of the juxtaglomerular apparatus on renal biopsy. Ultrasonography, intravenous pyelography and computed tomography showed marked bilateral renal calcification. Considering her history of persistent soft stool caused by chronic laxative abuse for 15 to 16 years and past diuretic abuse for several years since 1986, we diagnosed her as pseudo-Bartter's syndrome with nephrocarcinosis. The value of urinary Ca excretion was in the normal range, and acidification disturbance in NH4Cl loading test was revealed. In addition, she had taken analgesics for 2 to 3 years and interstitial nephritis on renal biopsy was seen. It is thus suggested that the cause of nephrocarcinosis in this case was the reduction of Ca solubility in the tubular cavity induced by incomplete renal tubular acidosis associated with analgesic nephropathy or interstitial nephritis caused by hypokalemia.     

Castleman病肾损害的临床病理分析

目的 探讨Castleman病肾损害的临床病理特点。 方法 对10例Castleman病合并肾损害患者的临床病理资料进行分析。全部病例均接受了淋巴结和肾组织的病理检查,肾活检组织分别进行了光镜、免疫荧光和电镜检查。 结果 10例均为男性,平均年龄（49±14）岁。多数患者有水肿;全部患者均有蛋白尿,尿蛋白量（24 h）为（2.79±3.56）g,其中1例为肾病综合征（NS）;8例有血尿;6例并发急性肾功能不全;4例有高血压。多数有发热、乏力、纳差、体质量下降等。其他异常有贫血、血小板减少、浆膜腔积液、红细胞沉降率增块、高γ球蛋白血症、补体降低、C反应蛋白（CRP）升高、肝脾肿大、甲状腺功能低下等。2例符合POEMS综合征,1例有干燥综合征。10例均表现全身多处淋巴结肿大,以颈部、腋窝及腹股沟淋巴结肿大最常见。淋巴结活检病理类型分别为浆细胞型4例,透明血管型3例,混合型3例。肾活检病理诊断分别为血栓性微血管病5例、新月体性肾小球肾炎2例、肾淀粉样变1例、肾小球微小病变1例、慢性肾小管间质肾病1例。所有病例经过免疫抑制剂或COP方案治疗,病情均明显缓解,淋巴结缩小,蛋白尿减少或转阴,大部分病例肾功能恢复正常。 结论 Castleman病肾损害的临床和病理表现具有多样性,急性肾功能不全发生率较高,常并发全身多系统损害,肾脏病理以血栓性微血管病较多见。对于伴有全身多系统异常的肾脏病患者,有必要进行淋巴结影像学检查和淋巴结活检。     

Granulomatous nephritis and chronic renal failure in sarcoidosis. Long-term follow-up studies in two patients

Sarcoid granulomatous nephritis was observed in 2 patients. Renal failure was present in both patients in the absence of hypercalcemia or nephrocalcinosis. Specific tubular dysfunction manifested as an acquired Fanconi syndrome in 1 patient. Steroid therapy was effective in causing regression of the renal granuloma, but there were significant interstitial changes on repeat renal biopsy which were accompanied by persistent tubular dysfunction. Sarcoid granulomatous nephritis may be a not infrequent cause of renal dysfunction in sarcoidosis.     

Significance of CD25 positive cells and macrophages in noncrescentic IgA nephropathy

The aim of this study was to determine whether infiltration by CD25 positive cells, macrophages, and activated macrophages in the kidney is predictive of chronic histological injury and renal prognosis in adults with noncrescentic IgA nephropathy. Renal biopsies of 36 patients with noncrescentic IgA nephropathy were examined by immunohistochemistry for glomerular and interstitial CD4, CD8, and CD25 positive cells, monocytes/macrophage (Mac387), and activated macrophages (27E10). Renal injury (glomerulosclerosis, mesangial cell hypercellularity, tubular atrophy, and interstitial fibrosis) at the time of biopsy and renal prognosis (follow-up creatinine and creatinine clearance) were assessed. The mean follow-up period was 22.5 +/- 16.5 months. The number of interstitial CD8 positive cells was the best predictor of renal injury at the time of biopsy, and was positively correlated with glomerulosclerosis (p = 0.04), tubular atrophy (p = 0.04), and interstitial fibrosis (p = 0.01) but not with mesangial cell hypercellularity. The number of interstitial Mac387 and 27E10 positive cells were the best predictors of renal prognosis (r2 = 0.33 and 0.34 respectively, both p < 0.01). These data suggest the presence of CD8 cells and macrophages in the kidney at the time of biopsy could potentially serve as pathological markers to identify patients with IgA nephropathy, which may warrant more aggressive medical therapy.     

Sonographic pattern of recessive polycystic kidney disease in young adults. Differences from the dominant form.

BACKGROUND: To study the sonographic pattern of autosomal recessive polycystic kidney disease (ARPKD) in early adulthood in order to identify imaging criteria to diagnose this disease and to distinguish between recessive and autosomal dominant polycystic kidney disease (ADPKD) in that age group. METHODS: An abdominal ultrasound was performed on four ARPKD subjects (with a mean age of 20.2) and on 33 ADPKD subjects in early adulthood (29 without renal failure with a mean age of 20.5, and four with renal failure with a mean age of 26.5). Linkage studies with ADPKD and ARPKD markers were compatible with the clinical diagnosis in all cases. RESULTS: The renal sonographic features in ARPKD subjects included multiple small cysts in a normal-sized kidney, increased cortical echogenicity and loss of corticomedullary differentiation. In ADPKD subjects without renal failure, sonographic features included few or multiple cysts of different sizes, in normal-sized kidneys in 22 out of 29 patients (75.8%), normal cortical echogenicity and conserved corticomedullary differentiation, except in patients with nephromegaly. All ADPKD subjects with renal failure had nephromegaly and loss of corticomedullary differentiation. The hepatic sonographic features in ARPKD patients included portal fibrosis and in some cases Caroli's disease, while in ADPKD patients a normal hepatic echostructure was detected in all but one case, in addition to simple hepatic cysts in a few cases. CONCLUSIONS: The evaluation of the sonographic features of the kidneys and those of the liver may help in the differential diagnosis between ARPKD and ADPKD in early adulthood.