Follow-up of Children after Fetal Treatment for Obstructive Uropathy

Background : Improvement in the ability to evaluate fetuses with obstructive uropathy, combined with technologic advances, has allowed successful fetal treatment of these conditions in Japan. We analyzed the prenatal and postnatal clinical courses of patients who underwent shunt placement in utero.
Methods : We treated 6 patients who underwent fetal intervention and were transferred to us for urologic management. Gestational age at detection of abnormalities ranged from 11 weeks to 31 weeks, and fetal intervention was done between 16 weeks and 32 weeks. Excluding 1 infant who was delivered 6 days after the intervention, the mean period between shunt placement and delivery was 17 weeks.
Results : Urologic pathology included prune belly syndrome with urethral hypoplasia in 3 patients, cloacal anomaly in 1, posterior urethral valves in 1, and pelviureteric junction stenosis of a solitary kidney in 1. Four patients required ventilation support in the neonatal period, and 2 of them suffered frequent infections during follow-up. Five patients who underwent vesico-amniotic shunt placement continue to require clean intermittent catheterizations via vesicostomy because of hypoactive detrusors and hypoplastic urethras. After urologic treatment, serum creatinine in 5 patients reached a minimum of less than 0.4 mg/dL at some time during the first year of life. Another patient with a minimum creatinine level of 1.0 mg/dL has moderate renal insufficiency.
Conclusion : From our series, it appears that early (before 20 weeks of gestation) shunt placement in severe lower urinary tract obstruction may benefit renal and pulmonary function. However, its efficacy in regard to bladder function remains questionable: shunt placement does not permit regular storage and evacuation, which may be essential for functional bladder development.     

Urinothorax as a manifestation of nondilated obstructive uropathy following renal transplantation

A 12-year-old patient developed prolonged nondilated urinary obstruction and pleural effusion shortly after undergoing renal transplantation. Renal sonography, angiography, and isotope renography failed to identify an obstructive process. On the 18th postoperative day, pleural effusion was noted in the right hemithorax, and by day 24, increased perinephric fluid was observed on renal scan. Following a nephrostomy, the pleural effusion resolved and renal function improved remarkably. A ureterovesical junction obstruction and renal pelvis tear that were later discovered were repaired. Whenever a ureteral obstruction is suspected the diagnosis should be pursued vigorously, despite normal radiologic findings, especially in the presence of pleural effusion. Consideration of the possibility of urinothorax in such cases may obviate the need for lung biopsy.     

Increased Urologic Complications in Children After Kidney Transplants for Obstructive and Reflux Uropathy

In the cyclosporine era, reports on pediatric kidney transplant (KTx) patients with obstructive and reflux uropathy are limited by small numbers, short follow-up, and/or lack of control groups. Our single-center study evaluated long-term outcomes (patient and graft survival, urinary tract infections [UTIs], urologic complications) in a large cohort of KTx recipients (<20 years old). We matched our 117 study patients with obstructive and reflux uropathy with 117 controls whose KTx was needed for other reasons; all 234 underwent their KTx between April 25, 1984, and October 23, 2002. The mean age was 8.0 +/- 6.2 years; mean follow-up, 133 +/- 67 months. The urologic complication rate was higher in study patients (43%) than in controls (11%) (p < 0.0001), as was the UTI rate (45% vs. 2%; p < 0.0001). The metabolic acidosis and UTI rates were higher in study patients who did (vs. did not) undergo bladder augmentation (p < 0.0001). We found no significant difference between study patients and controls in patient or graft survival, acute or chronic rejection, or mean estimated glomerular filtration rates. Unique to our study is the finding of higher metabolic acidosis and UTI rates in study patients who underwent bladder augmentation.     

Combined Pancreas and En Bloc Kidney Transplantation Using a Bladder Patch Technique From Very Small Pediatric Donors

Very small pediatric donors are underutilized for pancreas and kidney transplantation for the fear of inadequate islet mass and higher incidence of technical complications, and the lower age limit for such donors is not well defined. We present here two cases of combined pancreas and en bloc kidney transplantation from very small pediatric donors (14 and 18 months old) to adult type 1 diabetic and uremic patients. The conventional surgical procedure for simultaneous pancreas and kidney transplantation with systemic venous and bladder exocrine drainage was successfully applied to very small organs. For both, we utilized the recently described bladder patch technique for ureteral reconstruction. One patient developed venous thrombosis (partial thrombosis of the splenic and mesenteric veins) and the other urine leak (from a midportion of the medial ureter without compromising the bladder patch) after the transplants; both were successfully managed and the patients demonstrated immediate and sustained pancreas and kidney graft functions for 12 and 2 months posttransplantation. These cases illustrate the feasibility of combined pancreas and en bloc kidney transplantation from very small pediatric donors using a bladder patch technique to avoid small ureteral anastomosis.     

Distal Ureteral Atresia Associated with Crossed Renal Ectopia with Fusion: Recovery of Renal Function after Release of a 10-Year Ureteral Obstruction

We report on a 10-year-old boy with distal ureteral atresia associated with crossed renal ectopia with fusion. He was admitted with a high fever associated with a urinary tract infection. The diagnosis was established by antegrade and retrograde pyelography. The upper hydronephrotic portion of the kidney, obstructed for 10 years, recovered its function after nephrostomy placement. To our knowledge, this is the first patient whose renal function has recovered despite an ureteral obstruction of 10-years' duration. Therefore, we recommend a transient nephrostomy placement even for far advanced pediatric hydronephrosis, to test for the possibility of functional recovery.     

多层螺旋CT尿路造影与逆行肾盂造影在诊断输尿管梗阻性疾病中的价值对比

目的：探讨多层螺旋CT尿路造影（CTU）和逆行肾盂造影（RP）在诊断输尿管梗阻性疾病中的准确性和应用价值。方法：对40例输尿管梗阻性病变患者均先后行CTU和RP,以输尿管镜或开放手术或（和）病理检杏结果为标准,两者进行对照分析。结果：40例输尿管梗阻性疾病患者中。肿瘤6例,先天性狭窄9例,结石8例,良性狭窄17例。CTU和RP诊断准确率均为92．5％（37／40）（P〉0．05）,病因诊断符合率分别为90％（36／40）和57．5％（23／40）（P〈0．05）。结论：CTU对输尿管梗阻性病变诊断的准确率和病因符合率高,较RP有更好的病因诊断价值,可作为IVU显影不良者的首选补充检查方法,但仍存在一定局限性,需选择性应用。     

Partial Bladder Transplantation with En Bloc Kidney Transplant—The First Case Report of a 'Bladder Patch Technique' in a Human

Transplantation of the urinary bladder has not been reported in humans. We transplanted a portion of the donor bladder with an en bloc kidney graft in a 12-month-old girl. The child had a congenital hypoplastic single kidney with an ectopic ureteral opening into the vagina. Her native bladder was extremely small. Bilateral kidneys were transplanted en bloc with their ureters connected to a patch of the donor bladder, which encompassed the bilateral ureterovesical junctions (UVJs) (bladder patch technique). Approximately one-third of the donor bladder wall was used. The bladder patch reperfused well via blood supply from the ureters. Posttransplant cystoscopy with retrograde cystogram revealed a viable transplanted bladder with normal emptying of transplanted ureters. No reflux across the donor UVJs was seen in a voiding cystourethrogram. The child is doing well with normal renal function at 18-month follow-up.     

Steroid Sparing with Tacrolimus and Mycophenolate Mofetil in Renal Transplantation

Evidence suggests that steroid sparing in renal transplantation is associated with good outcomes, although there are limited data regarding steroid sparing in Tacrolimus and Mycophenolate Mofetil (MMF)-based regimes. In this study we describe the use of these agents in 101 consecutive patients undergoing renal transplantation using only a 7-day course of prednisolone. Median follow-up was 33 months (range 18-44). Patient and graft survival at 1 year were 100% and 98%, respectively. The acute rejection rate at both 6 and 12 months was 19%, with two episodes beyond 12 months. Anti-CD25 monoclonal antibody (anti-CD25 mAb) was administered to 25 patients at high immunological risk: a trend toward a lower rejection rate was seen in these patients compared with those at lower risk but not receiving induction therapy (8% vs. 22%; p = 0.11). Two patients experienced recurrent rejection. Of the twenty-three rejection episodes in total, 26% showed vascular involvement. Allograft function was preserved at 12 months with a mean creatinine of 144 micromol/L and mean estimated glomerular filtration rate (GFR) of 55 mL/min. At 12 months, the incidence of post-transplant diabetes mellitus was 3.5%. This steroid-sparing regime is associated with excellent patient and graft outcomes, and a low incidence of side effects.     

超声引导下经皮肾穿刺造瘘治疗梗阻性肾功能不全60例报告

目的：评估超声引导下肾盂穿刺造瘘治疗梗阻性肾功能不全的有效性和安全性。方法：采用超声引导下对60例肾后梗阻性肾功能不全患者行一步法经皮肾穿刺造瘘治疗,对其临床疗效和并发症进行观察分析。结果：所有患者都一次穿刺成功,无严重并发症发生,1例血块形成堵塞穿刺造瘘管,改行另外一侧穿刺造瘘引流成功。穿刺成功后肾功能迅速恢复或明显好转。结论：超声引导下经皮肾穿刺造瘘创伤小,恢复快,对于梗阻性肾功能不全患者是一种安全有效的治疗方法。     

黄芪对梗阻性黄疸大鼠肾功能的保护作用

目的:探讨黄芪对梗阻性黄疸大鼠肾功能的保护作用及机理.方法:SD大鼠胆总管结扎后分2组,每组20只,术后分别用3 mL黄芪或生理盐水腹腔注射.假手术组20只.术后10 d、20 d(每小组n=10)心脏取血测定血浆内皮素(ET-1)、血清超氧化物歧化酶(SOD)和丙二醛(MDA)含量,同时测定血清肌酐(Cr)尿素氮(BUN)和直接胆红素(DB),对肾脏行病理形态学观察.结果:血浆ET-1、血清MDA浓度在梗阻10 d即升高且随胆道梗阻时间延长进一步升高,SOD在梗阻10 d即下降,同时伴有血清Cr、BUN、DB的升高和肾脏病理形态学的进行性改变.黄芪治疗组ET-1及MDA显著低于对照组,而SOD显著高于对照组,并能改善肾组织病理形态.结论:大鼠梗阻性黄疸时血浆ET-1升高及氧自由基损害是肾损伤的原因,黄芪通过降低ET-1水平、抗氧化作用对梗阻性黄疸大鼠肾脏起保护作用.     

The Prevalence of Osteopenia in Pediatric Renal Allograft Recipients Varies with the Method of Analysis

BACKGROUND: Pediatric renal allograft recipients often suffer from osteopenia and the potential for increased fractures. Although modern densitometers are widely available, their use in children is complicated by lack of optimal interpretive criteria. METHODS: We reviewed dual energy X-ray absorptiometry (DEXA) studies in 33 patients with functional renal allografts 4.4 +/- 3.6 years after transplantation. We interpreted our data using three previously described methods of assigning bone mineral density (BMD) Z scores. RESULTS: BMD was directly related to age, height, weight, body surface area, and pubertal status (p < 0.001). Using gender-mixed reference data matched by chronological age, the mean BMD Z score was -0.9 +/- 1.3 vs. 0.4 +/- 1.4 when matched by height-age (p < 0.001). Height-age adjustment particularly increased the BMD Z score of pubertal adolescents. In a subset of 22 patients, gender-matched reference data led to different results from the gender-mixed reference population (mean BMD Z score 0.0 +/- 1.7 vs. -0.8 +/- 1.4, p < 0.001). CONCLUSIONS: The perceived prevalence of osteopenia among pediatric kidney transplant recipients differs using analysis based on chronological age, height-age, or gender-matched reference data. Further studies are necessary to determine the clinical significance of measured bone density in this population.     

Effects of Ureteral Stent on Urologic Complications in Renal Transplant Recipients: A Retrospective Study

The aim of the present study was to analyze the effects of ureteral stents used in renal transplantation on urologic as well as other complications. Cases of renal transplants from living or deceased donors performed in our hospital were retrospectively evaluated. The effects of the routine use of ureteral stents on postoperative complications were investigated. All outcomes and complications encountered during the postoperative follow-up were recorded. The Lich-Gregoire technique, which is a method of extravesical ureteroneocystostomy, was performed on all patients. One hundred and twenty-two patients underwent renal transplantation between 2001 and 2007 in our hospital. Stents were placed routinely in all patients. Leakage was observed in one patient, and one patient developed an obstruction; however, none of the patients developed an infection. A lymphocele developed in one patient. All urologic complications were treated without major morbidity. Graft loss did not occur. Complications following urinary anastomosis have a high rate of morbidity in renal transplantation. Ureteral stenting in renal transplant recipients prevents early urologic complications. The data generated in the current study were compared to the literature.     

An unusual cause of ureteral obstruction in a renal allograft.

We describe a 66-year-old patient, on haemodialysis since 2002because of chronic renal failure due to chronic glomerulonephritisof uncertain origin (supposedly IgA nephropathy). In March 2006,he underwent cadaveric donor kidney transplantation. The allografthad one polar artery, with the anastomosis made on the right     

洋川芎内酯A 对单侧输尿管梗阻大鼠肾间质纤维化的作用及机制研究

目的:探讨洋川芎内酯A(SenA)对单侧输尿管梗阻(UUO)大鼠肾脏病变的保护作用。方法:50只雄性SD大鼠随机分为假手术组、模型组、SenA低剂量(20 mg/kg)组、SenA高剂量(40 mg/kg)组及阳性对照(厄贝沙坦,20 mg/kg)组,行UUO术制备单侧输尿管模型,并于术后21天处死大鼠。根据试剂盒说明书检测血清尿素氮(BUN)、肌酐(Scr)的含量,HE染色法观察肾脏病理改变,免疫组化法检测肾组织α-SMA及CollagenⅠ蛋白的表达,Western blotting检测各组大鼠肾皮质Wnt4、β-catenin、p-GSK-3β、E-cadherin的表达水平。结果:与模型组比较,SenA低、高剂量组及阳性对照组大鼠血清中Scr、BUN含量明显降低(P<0.05),肾脏病变情况得到不同程度的改善。同时,SenA可明显减少大鼠肾组织中α-SMA和CollagenⅠ蛋白的分泌(P<0.05),下调Wnt4、β-catenin及p-GSK-3β的表达水平(P<0.05),并伴有E-cadherin蛋白表达显著上调(P<0.01)。结论:SenA可通过下调Wnt4/β-catenin信号通路,抑制肾组织细胞外基质沉积并改善肾功能,最终改善UUO模型大鼠肾间质纤维化的病变进程。     

恶性梗阻性黄疸根治性切除术后急性肾功能衰竭的预防

目的探讨恶性梗阻性黄疸根治性切除术围手术期肾功能的保护及急性肾功能衰竭（ARF）的预防。方法为降低ARF的发生率，从2004年起本治疗小组在ARF的预防方面采取了一系列措施，包括预防内毒素血症，术前减黄，充分扩容，营养支持，甘露醇、速尿的使用，预防弥散性血管内凝血等。分2个阶段回顾性分析从2000～2007年8年间（第1阶段：2000年1月至2003年12月；第2阶段：2004年1月至2007年12月）本治疗小组行根治性切除术的恶性梗阻性黄疸患者206例围手术期ARF的发生率，并引入了最新的ARF分级标准（RIFLE标准）对ARF进行分级。结果第2阶段恶性梗阻性黄疸的根治性切除率从第1阶段的44．8％提高到57．1％（P〈0．05），围手术期ARF的发生率从15．1％下降至6．7％（P〈0．05），其中ARF危险及损害阶段的发生率在第1阶段与第2阶段比较差异无统计学意义（P〉0．05），而ARF衰竭阶段发生率则由10．5％降至2．5％（P〈0．05），因此ARF发生率的降低主要是通过将急性肾功能损害控制在早期阶段而避免其向衰竭阶段发展，从而使围手术期的死亡率从16．3％降至5．8％（P〈0．05）。结论通过最新的RIFLE标准对ARF进行分级统计，说明我们在围手术期所采取的预防措施有效地控制了恶性梗阻性黄疸患者围手术期ARF的发生和发展。     

Incidence of PTLD in Pediatric Renal Transplant Recipients Receiving Basiliximab, Calcineurin Inhibitor, Sirolimus and Steroids

Pediatric renal transplant recipients were enrolled in a multicenter, randomized, double-blind trial of steroid withdrawal. Subjects received basiliximab, calcineurin inhibitor, sirolimus and steroids. Of 274 subjects enrolled, 19 (6.9%) subjects developed posttransplant lymphoproliferative disorder (PTLD). The relative hazard (RH) for PTLD was 5.3-fold higher in children aged ≤5 versus those >12 years (p = 0.0017). EBV seronegative subjects had a 4.7-fold higher RH compared to EBV positive subjects (p = 0.02). Among EBV donor+/recipient– (D+/R–) subjects, the RH increased by 6.1-fold (p = 0.0001). In a multivariate model, risk factors included recipient age ≤5 years (RH 3.2, 95% CI: 1.1–9.6, p = 0.034) and EBV D+/R– status (RH 7.7, 95% CI: 1.6–35.9, p = 0.010). Of 19 patients with PTLD, 17 are alive with functioning grafts and 2 lost their grafts, 1 of whom subsequently died of recurrent PTLD. This 'robust' immunosuppression protocol was associated with low rejection rates but an unacceptably high incidence of PTLD. The combination of basiliximab, calcineurin inhibitor, sirolimus and steroids resulted in over-immunosuppression in a high-risk pediatric population and we do not recommend its use. Future studies must include routine viral monitoring to permit early identification of viral activity and a protocol driven reduction of immunosuppression aimed at avoiding complications.     

Graft Arterial Stenosis in Kidney En Bloc Grafts From Very Small Pediatric Donors: Incidence,Timing, and Role of Ultrasound in Screening

In previous studies with different donor selection criteria and noncontemporary surgical techniques, graft arterial stenosis (GAS) has been reported to occur more frequently in adult recipients of pediatric en bloc renal allografts (EBKT) as compared to single adult donor allografts. The purpose of our study was to evaluate the incidence of GAS within our EBKT recipient population and to evaluate clinical and imaging features of those cases with GAS. In a retrospective cohort study, we analyzed 182 EBKT performed at a single institution. We identified cases of suspected GAS based on clinical factors, lab results, and noninvasive imaging. Diagnosis of GAS was confirmed by digital subtraction angiography. Two EBKT recipients (1.1% of 182) had angiographically confirmed GAS at 2.5 and 4.5 months after transplant. In both cases, the stenoses were short segment within the proximal (perianastomotic) donor aorta, color Doppler ultrasound demonstrated peak systolic velocities of >400 cm/s, and poststenotic parvus tardus waveforms were present. Both patients underwent angioplasty and demonstrated postintervention improvement in renal function and blood pressure. Restenosis did not occur during follow up. In conclusion, recipients of EBKT have a low incidence of GAS, similar to the lowest reported for adult single allografts.     

丹参对梗阻性黄疸患者肾功能的影响

目的:研究丹参对梗阻性黄疸患者围手术期肾功能的影响.方法:80例患者随机分为两组,试验组40例除常规治疗外给予丹参注射液40 mL静脉给药5 d,对照组40例给予常规治疗.于术前1 d,术后第2、6 d分别抽取两组患者清晨空腹血样及采取24 h尿样,检测血样尿素氮(BUN),肌酐(Cr)及24 h尿肌酐水平,计算肌酐清除率(Ccr).结果:术后2 d,Cr较术前升高,Ccr较术前降低,BUN与术前无差异.术后第6 d,丹参组Cr、BUN、Ccr与术前无差异,而对照组Cr仍较术前增高,Ccr仍较术前降低.结论:丹参对梗阻性黄疸患者围手术期肾功能有促进恢复作用.