Ischemic colitis as a complication in a patient with steroid-dependent nephrotic syndrome

We report the case of a 16-year-old male patient with steroid-dependent nephrotic syndrome who developed ischemic colitis. He was diagnosed as having nephrotic syndrome at 10 years of age and had been administered steroid, cyclosporine A, and mizoribine for 7 years. He presented with severe abdominal pain 5 days after intravenous methylprednisolone pulse therapy; thereafter, massive bloody diarrhea developed. Abdominal ultrasonography and computed tomography revealed a marked thickening of the wall of the transverse colon. Colonoscopy confirmed the diagnosis of ischemic colitis. This is the first report of the development of ischemic colitis in a pediatric patient with nephrotic syndrome.     

Endovascular repair for a descending thoracic aortic aneurysm with a stent-graft covering the celiac artery: Report of two cases

An adequate landing zone for fixation and sealing is necessary for endovascular aneurysm repair (EVAR). This report presents two cases of a successful EVAR for thoracic aortic aneurysms (TAA) with a stent-graft covering the celiac artery (CA) to secure a distal landing zone. Case 1 was a 61-year-old man with a chronic traumatic descending TAA 12 mm away from the CA. Case 2 was a 79-year-old man with a descending TAA proximal to the CA. Preoperative angiography and computed tomography (CT) scan revealed a normal visceral blood flow including the peripancreatic arteries. Endovascular aneurysm repair with coverage of the CA was performed in both cases. Angiography after the EVAR demonstrated good blood flow to the CA branches via the peripancreatic arteries and a CT scan showed thrombosed aneurysms. Both patients were discharged without any abdominal symptoms. Endovascular aneurysm repair with a stent-graft covering the CA may therefore be an acceptable endovascular approach in treating selected TAA patients with a limited distal landing zone.     

Myocardial ischemia due to compression of an unruptured thoracic aortic aneurysm in a patient with Marfan syndrome

We report a 33-year-old woman who had a 60-mm thoracic aneurysm of the ascending aorta with Marfan syndrome and effort angina due to compression of the right coronary artery (RCA) by the aneurysm. Surgery was performed using the Bentall procedure and a coronary artery bypass graft to the RCA. Postoperatively, coronary angiography showed that the coronary flow of the RCA was restored by removing the aneurysmal compression. The patient was discharged without angina on postoperative day 21.     

长春西汀联合泼尼松治疗原发性肾病综合征疗效观察

目的观察长春西汀联合泼尼松治疗原发性肾病综合征的临床疗效。方法选取原发性肾病综合征患者62例,随机分为观察组和对照组。对照组采用泼尼松及常规治疗,观察组在对照组治疗基础上给予长春西汀注射液静脉滴注,两组疗程均为3周。观察两组患者临床改善情况。结果观察组总有效率（83．9％）明显高于对照组总有效率（61．3％）（P〈0．05）,两组治疗后血浆白蛋白、24h尿蛋白定量与本组治疗前比较有统计学差异（P〈0．05）,观察组较对照组改善更显著（P〈0．05）,观察组尿素氮、血肌酐、活化部分凝血活酶时间、纤维蛋白原、D-二聚体较治疗前明显改善（P〈0．05）,与对照组相比有统计学差异（P〈0．05）。结论长春西汀联合泼尼松治疗原发性肾病综合征安全有效,能有效改善血液高凝状态。     

原发性肾病综合征并发急性肾损伤患者甲状腺功能的变化

目的观察原发性。肾病综合征（PNS）并发急性肾损伤（AKI）患者甲状腺功能的变化及其影响因素。方法回顾性分析PNS患者77例,其中PNS并发AKI患者27例为PNS＋AKI组;PNS肾功能正常者50例为PNS组;同时设原发性慢性肾小球肾炎肾功能正常组（CGN1组）和CGN肾功能异常组（CGN2组）作为对照组,每组各40例。比较4组患者的血浆总蛋白（TP）、血清白蛋白（Alb）、血肌酐（SCr）、甘油三脂（TG）、胆固醇（TC）、24h尿蛋白定量及甲状腺功能指标游离三碘甲腺原胺酸（盯3）、游离四碘甲腺原胺酸（FT4）、促甲状腺素（TSH）。结果（1）PNS＋AKl组FT3、FT4值较各组明显降低（P〈0.05）;而TSH无明显差异（P〉0.05）。（2）PNS＋AKI组和PNS组TP、Alb、SCr有显著差异（P〉0.05）。（3）多元回归分析示Alb、SCr对FT3影响显著（P〈0.05）。结论PNS合并AKI时较PNS患者FT3、FT4明显降低,而TSH无明显变化,这种改变可能与Alb的进一步降低和肾功能损伤有关。     

Spontaneous remission of nephrotic syndrome in a patient with IgA nephropathy

A patient with spontaneous remission of nephrotic syndrome (NS) associated with IgA nephropathy is described. The patient presented at the age of 8 years with asymptomatic proteinuria, and at the age of 11 years developed classical features of NS. A percutaneous renal biopsy showed mild mesangial prominence without significant hypercellularity, electron-dense deposits within the mesangium, and 3+mesangial staining with IgA and IgG. NS resolved 6 weeks after onset without any form of therapy; absence of protein-uria persisted 6 months later. This report demonstrates clearly that patients with NS associated with IgA nephropathy may undergo spontaneous resolution of their proteinuria.     

Effect of cyclosporine therapy on idiopathic membranous nephropathy presented with refractory nephrotic syndrome

Background. Recent studies suggested the possible benefits of cyclosporine (CsA) therapy in patients with membranous nephropathy, although most of these studies were short-term. An uncontrolled retrospective study was undertaken to evaluate the long-term effect of CsA therapy on idiopathic membranous nephropathy presented with refractory nephrotic syndrome. Methods. The subjects were eight patients with idiopathic membranous nephropathy presenting with refractory nephrotic syndrome. All patients had received a course of corticosteroid therapy before CsA therapy, and had not responded to the corticosteroid, including one or two administrations of intravenous methylprednisolone pulse therapy. The CsA doses were adjusted to maintain trough blood level at 100 ng/ml during the first 3 months and then reduced to maintain the level at 50 ng/ml in patients who had responded to partial remission. Results. CsA therapy induced a marked decrease in proteinuria from the first month, and a significant decrease from month 3 and thereafter. The mean serum total protein and albumin levels rose, and total cholesterol fell significantly with CsA therapy. The serum creatinine level was unchanged during CsA therapy. Three patients showed complete remission and two were in partial remission, while three were nephrotic at 12 months of CsA therapy. From 18 to 24 months of CsA therapy, three patients were in complete remission, four were in partial remission, and one patient was nephrotic. There were no side effects of CsA, except for gum hyperplasia and hypertrichosis in one patient. Conclusion. These results suggest that long-term CsA therapy at a low or moderate dose is potentially effective and safe in most nephrotic patients with idiopathic membranous nephropathy refractory to corticosteroid therapy. Received: February 22, 1999 / Accepted: July 30, 1999     

High-sensitivity C-reactive protein (hs-CRP) level in children with nephrotic syndrome

The aim of this study was to assess the serum concentration of high-sensitivity C-reactive protein (hs-CRP) in children with nephrotic syndrome (NS) treated with prednisone and cyclosporine A (CyA). Patients were divided into three groups: (I) 20 NS children (aged 4–14 years) in relapse and examined twice, (A) before treatment and (B) after proteinuria regression (a 3–4 week course of prednisone therapy); (II) 20 children with steroid-dependent or steroid-resistant NS, treated with CyA, also examined twice, (D) before treatment with CyA, (E) 6 months after therapy. A control group (C) consisted of 20 healthy children. Serum hs-CRP level was determined by a nephelometric method with a Behring Nephelometer 100 Analyzer, Dade Behring. The results showed that median hs-CRP concentration was the highest in children with relapsing steroid-sensitive NS before treatment (IA). After proteinuria regression (IB), the hs-CRP level had decreased and did not differ from that of healthy controls (C) (P > 0.05). In group II, before CyA administration (IID), the level of hs-CRP was normal, but it had increased after 6 months of treatment (IIE) up to a level six-times higher than that of the control group (P < 0.01). We concluded that, in children with steroid-sensitive nephrotic syndrome in relapse, the serum hs-CRP level is increased but returns to normal after 3–4 weeks of glucocorticoid treatment. In children chronically treated with CyA due to NS, serum hs-CRP level increases significantly during the therapy.     

微创腔内隔绝术治疗降主动脉夹层动脉瘤

目的：探讨腔内隔绝术（ＥＶＧＥ）治疗降主动脉夹层动脉瘤（ＤＡＡ）的临床应用价值。方法：２３例ＤｅｂａｋｅｙⅢ型ＤＡＡ患者,经股动脉将直形人造血管－支架复合体（移植物）导入夹层动脉瘤裂口处,支架张开使人造血管固定于裂口附近的动脉壁上,将裂口封闭,消除动脉瘤破裂的危险。结果：３例术中出现内漏的病人,经即时附加导入移植物而将漏门封闭。全部获得成功。结论：ＥＶＧＥ治疗ＤＡＡ,创伤小、并发症少、术后恢复快,有广阔的临床应用前景。     

Spectrum of glomerulonephritides in adults with nephrotic syndrome in Pakistan

Background  There is currently little information in literature about the pattern of glomerulonephritides (GN) in adults with nephrotic syndrome in this part of the world, particularly that involving the use of immunofluorescence (IMF) and electron microscopy (EM). A few studies reported are based on light microscopic study alone and hence do not reflect the true pattern of GN underlying nephrotic syndrome. We carried out this study in the Department of Histopathology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, Pakistan to determine, for the first time, the true pattern of GN in adult nephrotic patients from Pakistan. SIUT is a tertiary care center for renal and urologic disease in Pakistan. The Histopathology Laboratory of SIUT is equipped with all the modalities, including EM, required for precise diagnosis of glomerular disease. Methods  This is a retrospective clinicopathologic study involving retrieval of clinical and pathological data from a review of original renal biopsy reports of adult patients with nephrotic syndrome who presented at the adult nephrology clinic of SIUT from July 1996 till July 2006. Two cores of renal tissue were routinely obtained. One core was fixed in 10% buffered formalin and processed for light microscopy; the other core was divided into two halves, for EM and the IMF study. Results  A total of 316 adult patients were included. Of these, 201 (63.6%) were male and 115 (36.4%) were female. Mean age was 28.4 ± 10.51 years with a range of 16–78 years. The spectrum of pathological lesions in the adult nephrotic population was wide and comprised focal segmental glomerulosclerosis (FSGS) (39.87%), followed by membranous GN (MGN) (26.58%), minimal change disease (MCD) (14.82%), mesangiocapillary GN (4.3%), mesangioproliferative GN (4.11%), post-infectious GN (2.84%), IgA nephropathy (2.53%), and other rare lesions. Conclusions  Results from this study indicate that FSGS is the single most common cause of nephrotic syndrome in adult nephrotic patients, followed by MGN, and MCD. Our data are similar to those reported in recent series from the US. The study defines the pattern of glomerular disease in adult nephrotic patients for the first time in this region, because it is based on light microscopy, serology, IMF, and EM findings. Disclosure: No financial interest of any of the authors is involved. The authors declare that the results of this paper have not been published previously in whole or in part, except in abstract form.     

Cyclosporine therapy monitored with abbreviated area under curve in nephrotic syndrome

Cyclosporin A (CsA) is an effective therapy for children with long-lasting nephrotic syndrome (NS). Long-term treatment can result in chronic CsA nephropathy (CsAN) and there is controversy concerning its incidence and severity. Trough levels are commonly used to monitor the drug concentration. We report a retrospective clinical and histological analysis of 18 children (12 males, 6 females) with steroid-dependent nephrotic syndrome (15 patients) and partially steroid-sensitive nephrotic syndrome (3 patients) treated with CsA for a long-term period (mean 4.9 years, range 2.2–6.9). Before CsA treatment all patients had normal creatinine clearance. CsA was started at a dose of 5 mg/kg per day administered orally in two divided doses and adjusted to maintain the mean CsA blood concentration between 250 and 350 ng/ml obtained from abbreviated area under the curve (AUC). A renal biopsy was performed after a mean period of 3.9 years (range 2.2–6.2) from the start of CsA treatment. Tubular, interstitial, and arteriolar lesions were evaluated in order to assess CsAN. The mean CsA dose and the mean CsA blood concentration were 4.4 mg/kg per day (range 3.6–5.8) and 276.6 ng/ml (range 162–346), respectively. No child had a worsening creatinine clearance during CsA treatment and follow-up after CsA discontinuation. If compared with the year before the start of CsA treatment, NS relapses and prednisone (PDN) dose significantly decreased during CsA treatment, 4/year versus 0.8/year (P <0.0001) and 0.9 mg/kg per day versus 0.2 mg/kg per day (P <0.0001), respectively. Histological analysis showed 15 patients with minimal change disease and 3 with focal segmental glomerulosclerosis. Clear-cut lesions diagnostic of CsAN were never found and only mild lesions were observed in 5 children (suggestive of CsAN in 2 patients and consistent with CsAN in 3 patients). Long-term CsA treatment is confirmed to be effective in preventing NS relapses and reducing PDN dose. Renal function is not a reliable indicator of CsAN. With the mean CsA blood concentrations used in our patients CsAN presented a low incidence (28%) and was generally mild. Renal biopsy should be performed 2–3 years from the start of long-term CsA treatment, especially if the mean CsA blood concentrations are not regularly monitored.     

厄贝沙坦联合甲基泼尼松龙冲击治疗原发性系膜增生性肾小球肾炎疗效的观察

目的观察厄贝沙坦联合大剂量甲基泼尼松龙冲击治疗原发性系膜增生性肾小球。肾炎（MsPGN）的早期疗效。方法将43例通过。肾脏活体组织检查确诊为MsPGN患者,分为厄贝沙坦联合大剂量甲基泼尼松龙冲击治疗组（A组）15例;大剂量甲基泼尼松龙冲击治疗组（B组）14例;甲基泼尼松龙片常规剂量治疗组（C组）14例,疗程共4周。检测治疗前及治疗后第1、2、3、4周血清白蛋白（Alb）、24h尿蛋白定量、内生肌酐清除率（Ccr）、尿素氮（BUN）、丙氨酸氨基转氨酶（ALT）、天门冬氨酸氨基转氨酶（AST）及空腹血糖（FBS）。结果A、B组在治疗后第3、4周24h尿蛋白定量减少、Alb升高,与C组比较有统计学差异（P〈0．01）。A组在治疗后第3、4周24h尿蛋白定量减少,与B组比较有统计学差异（P〈0．05）;各组治疗前、后Ccr、BUN、ALT、AST及FBS均无统计学差异（P〉0．05）。结论大剂量甲基泼尼松龙冲击治疗原发性MsPGN能迅速缓解临床症状,起效时间短,降低尿蛋白快。     

Decreased cyclosporine exposure during the remission of nephrotic syndrome

In this paper, we report the pharmacokinetics changes observed in seven children with steroid-resistant nephrotic syndrome (SRNS). They received cyclosporine A (CsA) microemulsion 6 mg/kg/day and, one week later, they were admitted to perform a 12-h pharmacokinetic profile with eight time sample points. The pharmacokinetic profile was repeated at 24 weeks of treatment, when all patients achieved remission. Blood concentration against time curves were constructed for each patient at weeks 1 and 24 of CsA treatment. Peak concentrations (C _max) and the time needed to reach peak concentrations (t _max) were directly determined from these plots. The area under the curve (AUC) was estimated by the trapezoidal rule. There was a statistically significant difference of the AUC, trough levels, and t _max between weeks 1 and 24, with a decrease of AUC from 5,211 ng*h/ml in week 1 to 3,289 ng*h/ml in week 24, the trough levels decreased from 157 ng/ml to 96 ng/ml, and the t _max decreased from 1.85 h to 1.00 h. The higher CsA bioavailability during the nephrotic state has to be considered when managing patients, since the target AUC cannot be the same throughout the treatment.     

Tension hemothorax caused by a ruptured aneurysm of the descending thoracic aorta: Report of a case

(Received for publication on Mar. 10, 1999; accepted on Jan. 7, 2000)     

Amelioration of steroids and cyclosporine-resistant nephrotic syndrome by pravastatin

We report the case of a girl with steroids and cyclosporine (CsA) resistant focal segmental glomerulosclerosis (FSGS) whose proteinuria and hypoproteinaemia were dramatically resolved by pravastatin. She had been in a nephrotic condition for 6 years. Prednisolone, pulse methylprednisolone therapy, low-density lipoprotein (LDL) apheresis, CsA, cyclophosphamide and mizoribine (MZR) had proved to be ineffective. She was started on pravastatin for her hyperlipidaemia 6 and a half years from onset, in addition to the baseline therapy, which included CsA; remission of the nephrotic syndrome was unexpectedly attained after 10 months of treatment. The baseline therapy has not been changed since the inclusion of pravastatin. This case suggests that, in patients with hyperlipidaemia, the response to CsA could be restored by lowering cholesterol levels with statins. The decrease of cholesterol levels might have improved the pharmacokinetics of CsA in this patient. Furthermore, the anti-inflammatory and immuno-modulatory effects, recently attributed to statins, may also have been involved in the improvement experienced by our patient. There were no conflicts of interest in the writing of this article.     

清利解毒活血汤联合西药治疗难治性肾病综合征45例疗效观察

目的探讨中西医结合疗法对难治性肾病综合征（RNS）的疗效。方法对45例有病理资料的RNS患者采用清利解毒活血汤联合泼尼松、环磷酰胺治疗,并对病理类型、系膜细胞及肾小管问质损害程度与疗效的关系进行分析。结果45例RNS完全缓解率48．9％,总有效率84．4％。其中系膜增生性肾炎、IgA肾病、局灶性节段性硬化性肾炎及微小病变肾病疗效较好,膜性肾病及膜增殖性肾炎疗效较差,系膜细胞损害程度及肾小管间质损害程度与疗效密切相关。结论清利解毒活血汤联合泼尼松、环磷酰胺对RNS有较好的疗效,但对膜性肾病、膜增殖肾炎以及系膜细胞损害严重或肾小管间质损害严重患者疗效尚不太理想。     

经食管超声心动图在胸主动脉瘤介入治疗中的应用

血管内修复术创伤性小，可应用于治疗多种主动脉疾病，如动脉瘤和主动脉夹层。经食管超声心动图(TEE)对主动脉疾病很敏感。术前可通过TEE找到撕裂的内膜片、发现内膜破口、区分类型、区分真假腔及了解心脏状况。术中TEE用于引导导管插至正确位置、观察支架放置过程、监测心功能和室壁运动状况、评价手术疗效。术后随诊通过TEE观察支架内血流情况、检出并发症如内漏等。     

环孢素A治疗儿童难治性肾病综合征26例疗效分析

目的探讨环孢素A治疗儿童难治性肾病综合征的临床疗效。方法 26例难治性肾病综合征患儿入院后口服环孢素A,剂量为2～4mg（/kg·d）,同时联合激素治疗,疗程3个月。治疗前及治疗后检测24h尿蛋白定量、血浆白蛋白、血浆胆固醇、尿素氮、血肌酐等指标。结果 26例患儿经治疗后,完全缓解14例,占53.85%;部分缓解10例,占38.46%;未缓解2例,占7.69%。结论环孢素A联合激素能有效治疗难治性肾病综合征。