Renal tubular dysgenesis without pulmonary hypoplasia

Only a few cases of renal tubular dysgenesis (RTD) have been diagnosed. RTD is associated with a very early death, always due to lethal pulmonary hypoplasia. We report an additional patient, interesting for two reasons: (1) the rarity of RTD, (2) the absence of microscopic and macroscopic anatomical alterations of the lungs. The infant had only a mild, transient pulmonary maladaptation; after a few days of life the pulmonary function returned to normal. Although renal function dramatically improved after institution of peritoneal dialysis at 6 days of life, the patient died after 2 weeks with an overwhelmingCandida sepsis. We suggest that the lack of pulmonary hypoplasia might be due to a short exposure to oligohydramnios.     

Renal innervation and denervation: lessons from renal transplantation reconsidered

Renal transplantation assures complete denervation of the kidney. Human renal transplantation provided an opportunity to examine the consequences of renal denervation on renal functional capacity in human subjects. These initial studies led to an anatomical and physiological assessment of the time course of renal neural degeneration and reinnervation following renal transplantation or surgical division of the renal nerves. Together with a growing body of information concerning the extensive intrinsic innervation of the kidney's structural components, there has developed a comprehensive understanding of the overall neural control of kidney function.     

Renal hypoplasia and situs inversus totalis

A spectrum of renal abnormalities of patients with situs inversus has been reported. Renal dysplasia is the most common. Herein is described for the first time, an association of situs inversus totalis, unilateral congenital renal hypoplasia and external ear cartilage deformity.     

Three-year Oncologic and Renal Functional Outcomes After Robot-assisted Partial Nephrectomy

Background

With the wider adoption of minimally invasive partial nephrectomy (PN), intermediate- and long-term outcomes data are needed to make firm conclusions about oncologic and functional efficacy, especially for robot-assisted PN (RPN).

Objective

To report intermediate-term oncologic and renal functional outcomes of RPN.

Design, setting, and participants

We performed a chart review of patients who had undergone RPN since June 2006; patients with a minimum of 2 yr of follow-up were included in this study. Length of follow-up was calculated from the date of surgery to the date of last clinical follow-up. Patients who were either lost to follow-up or who had follow-up outside of our center were sent surveys.

Intervention

Transperitoneal RPN with or without hilar clamping.

Outcome measurements and statistical analysis

The demographic, preoperative, and postoperative data were statistically analyzed. The Kaplan-Meier method was used to calculate overall survival (OS), cancer-specific survival (CSS), and cancer-free survival (CFS). Upstaging of chronic kidney disease (CKD) was calculated, as well. Univariate and multivariate analyses were performed to show predicting factors for the latest estimated glomerular filtration rate (eGFR).

Results and limitations

Of 427 patients, 134 had a minimum follow-up of 2 yr, and 70 had a minimum of 3–6 yr of follow-up. The mean age was 59.1 ± 12.5 yr, body mass index (BMI) was 29.8 ± 6.2 kg/m², and Charlson comorbidity index (CCI) score was 4.2 ± 1.6. The mean tumor size on computed tomography (CT) scan was 3.0 ± 1.6 cm, RENAL score was 7.2 ± 1.8, estimated blood loss (EBL) was 270.7 ± 291.9 ml, operative time was 189.1 ± 54.8 min, and warm ischemia time (WIT) was 17.9 ± 10.3 min. A total of two intraoperative complications (1.5%) and five high-grade Clavien complications (3.7%) occurred. Patients stayed on average for 3.7 ± 1.7 d in the hospital, and the average follow-up was 3.0 ± 0.9 yr. OS was 97.01% at 3 yr and 90.20% at 5 yr; CFS was 98.92% at 3 yr and 98.92% at 5 yr; and CSS was 99.04%, as projected by the Kaplan-Meier method. The mean preoperative GFR was 88.2 ± 0.8 ml/min per 1.73 m²; the latest postoperative GFR was 80 ± 24 ml/min per 1.73m², with a 8 ± 17.4% change. There was a 20.2% upstaging of CKD postoperatively, but no patients started dialysis.

Conclusions

This study reaffirms that RPN is effective in renal function preservation and oncologic control at an intermediate follow-up interval.     

Spatiotemporal alterations in Sprouty-2 expression and tyrosine phosphorylation in nitrofen-induced pulmonary hypoplasia

Background/Purpose

Pulmonary hypoplasia (PH) is a life-threatening condition of newborns presenting with congenital diaphragmatic hernia (CDH). Sprouty-2 functions as a key regulator of fibroblast growth factor receptor (FGFR) signalling in developing foetal lungs. It has been reported that FGFR-mediated alveolarization is disrupted in nitrofen-induced PH. Sprouty-2 knockouts show severe defects in lung morphogenesis similar to nitrofen-induced PH. Upon FGFR stimulation, Sprouty-2 is tyrosine-phosphorylated, which is essential for its physiological function during foetal lung development. We hypothesized that Sprouty-2 expression and tyrosine phosphorylation are altered in nitrofen-induced PH.

Methods

Time-pregnant rats received either nitrofen or vehicle on gestation day 9 (D9). Foetal lungs were dissected on D18 and D21. Pulmonary Sprouty-2 gene and protein expression levels were analyzed by qRT-PCR, Western blotting and immunohistochemical staining.

Results

Relative mRNA expression of Sprouty-2 was significantly decreased in hypoplastic lungs without CDH (0.1050 ± 0.01 vs. 0.3125 ± 0.01; P < .0001) and with CDH (0.1671 ± 0.01 vs. 0.3125 ± 0.01; P < .0001) compared to controls on D18. Protein levels of Sprouty-2 were markedly decreased in hypoplastic lungs on D18 with decreased tyrosine phosphorylation levels on D18 and D21 detected at the molecular weight of Sprouty-2 consistent with Sprouty-2 tyrosine phosphorylation. Sprouty-2 immunoreactivity was markedly decreased in hypoplastic lungs on D18 and D21.

Conclusion

Spatiotemporal alterations in pulmonary Sprouty-2 expression and tyrosine phosphorylation during the late stages of foetal lung development may interfere with FGFR-mediated alveolarization in nitrofen-induced PH.     

Effects of maternal vitamin A status on kidney development: a pilot study

Nephron endowment ranges widely in normal human populations. Recent autopsy studies have drawn attention to the possibility that subtle congenital nephron deficits may be associated with increased risk of developing hypertension later in life. Since modest maternal vitamin A deficiency reduces nephron number in rats, we designed a pilot study to determine the prevalence of maternal vitamin A deficiency in Montreal (Canada) and Bangalore (India) and the usefulness of newborn renal volume as a surrogate for nephron endowment. Among 48 pregnant Montreal women, two (4%) had one isolated mid-gestation retinol level slightly below the accepted limit of normal (0.9 μmol/L), whereas 25 (55%) of 46 pregnant women in Bangalore had at least one sample below this limit. Average estimated retinoid intake was correlated with mean serum retinol in pregnant women from Bangalore. In Montreal where maternal vitamin A deficiency was negligible, we found that newborn renal volume (estimated by renal ultrasonography at 2–6 weeks of age) was correlated with surface area at birth and was inversely correlated with serum creatinine at 1 month. Interestingly, renal volume adjusted for body surface area in Montreal (184±44 ml/m²) was significantly greater than in Bangalore (114±33 ml/m²) (p<0.01). Definitive studies are needed to establish whether maternal vitamin A deficiency accounts for subtle renal hypoplasia in Indian newborns. If so, there may be important public health implications for regions of the world where maternal vitamin A deficiency is prevalent.     

Renal adaptation to phosphate deprivation: lessons from the X-linkedHyp mouse

The X-linkedHyp mutation, a murine homologue of X-linked hypophosphatemia in humans, is characterized by renal defects in phosphate reabsorption and vitamin D metabolism. In addition, the renal adaptive response to phosphate deprivation in mutantHyp mice differs from that of normal littermates. WhileHyp mice fed a low phosphate diet retain the capacity to exhibit a significant increase in renal brush-border membrane sodiumphosphate cotransport in vitro, the mutants fail to show an adaptive increase in maximal tubular reabsorption of phosphate per volume of glomerular filtrate (TmP/GFR) in vivo. Moreover, unlike their normal counterparts,Hyp mice respond to phosphate restriction with a fall in the serum concentration of 1,25-dihydroxyvitamin D [1,25(OH)₂D] that can be ascribed to increased renal 1,25(OH)₂D catabolism. The dissociation between the adaptive brush-border membrane phosphate transport response and the TmP/GFR and vitamin D responses observed inHyp mice is also apparent in X-linkedGy mice and hypophysectomized rats. Based on these findings and the notion that transport across the brush-border membrane reflects proximal tubular function, we suggest that the adaptive TmP/GFR response requires the participation of 1,25(OH)₂D or a related metabolite and that a more distal segment of the nephron is the likely target for the 1,25(OH)₂D-dependent increase in overall tubular phosphate conservation.     

Renal funtional reserve in kidney transplant recipients

In the last few years different authors have observed that kidney transplant recipients with good organ function do not have a renal functional reserve (RFR). This condition is accompained by a high glomerular filtration rate (GFR) [2–6]. We studied RFR in patients with very good organ function under different immunosuppressive therapies, who were divided into groups based on the presence or absence of RFR.     

Classification Systems in Renal Pathology: Promises and Problems

Kidney diseases are morphologically heterogeneous. Pathologic classifications of renal disease permit standardization of diagnosis and may identify clinical-pathologic subgroups with different outcomes and/or responses to treatment. To date, classifications have been proposed for lupus nephritis, allograft rejection, IgA nephropathy, focal segmental glomerulosclerosis, antineutrophil cytoplasmic antibody -related glomerulonephritis, and diabetic glomerulosclerosis. These classifications share several limitations related to lack of specificity, reproducibility, validation, and relevance to clinical practice. They offer a standardized approach to diagnosis, however, which should facilitate communication and clinical research.     

Renal functional reserve in children with reduced renal mass: study by two dietary periods

It has been suggested that the renal functional reserve (RFR) defined by the rise in glomerular filtration rate (GFR) after a protein load could disappear in patients with severe nephron loss but with a normal GFR. This study compared, in 17 children, inulin clearance (C _in) measured by the plasma inulin plateau at the end of two 14-day randomized periods differing in protein intake: 100% (low protein, LP), or 200% (high protein, HP) of recommended dictary allowances (RDA). Diets were aimed at maintaining food habits and energy intake. Compliance was assessed by records of the last 3–4 days, an interview with the dietician and by urinary nitrogen measurements. Mean actual protein intake was 109% (56%–139%) RDA for the LP period and 220% (163%–319%) RDA for the HP period.C _in did not change in 14 children with GFR below (n=7) or within (n=7) the normal range.C _in was higher in the HP period than in the LP period (+32, 50, 63%) in 3 children who had a 50% (single kidneys) or a 25% (sclerosed glomeruli) nephron loss. Non-responding children had a GFR below 105 ml/min per 1.73 m². Nephron loss (70% sclerosed glomeruli) was estimated in only 1 child with no RFR. The results suggest that GFR measurement after prolonged dietary stimulation could help in evaluating the severity of nephron loss in children with normal or borderline GFR. The prognostic value of this test has to be confirmed by long-term follow-up.     

肾动脉阻断对腹腔镜下保留肾单位术后肾功能的影响

目的：探讨阻断肾动脉主干对腹腔镜下保留肾单位术后患者肾功能的影响.方法回顾分析因肾肿瘤行腹腔镜下保留肾单位手术患者的临床资料,根据术中肾动脉阻断时间、出血量、肿瘤分期、肿瘤病理、术后随访及肾小球滤过率变化,分析肾动脉阻断对保留肾单位手术后肾功能的影响.结果82例患者中,肾透明细胞癌75例,乳头状肾细胞癌3例,嫌色细胞癌4例;肾动脉阻断时间平均(24．5±8．6)min,术中失血量(249．4±66．9)ml.热缺血时间在25 min 以内的患者与26~30 min 患者比较,术后患肾肾功能受损差异无统计学意义(P ＞0．05);热缺血时间为31~40 min 的患者与前两组比较,术后患肾肾功能受损差异有统计学意义(P ＜0．05).结论保留肾单位手术均会使患侧肾功能下降,但阻断肾动脉主干热缺血时间控制在30 min 内对肾功能影响相对较小.     

保留肾单位手术后肾功能预后的影响因素分析

目的:探讨肾肿瘤行保留肾单位手术(NSS)后肾功能预后的影响因素。方法:回顾性分析西京医院2016年12月至2018年12月行NSS治疗的115例肾肿瘤患者的临床资料。男75例,女40例。年龄(49.50±12.94)岁;体质指数(24.59±3.59)kg/m 2;肿瘤最大径(3.66±1.32)cm;R.E.N.A.L.评分(6.43±1.60)分。术前患肾肾小球滤过率(GFR)(48.22±11.48)ml/(min·1.73m 2),健肾GFR(49.73±11.96)ml/(min·1.73m 2),总GFR(97.95±21.32)ml/(min·1.73m 2)。术前患肾有功能肾组织体积(FPV)(132.23±9.11)cm 3。61例行腹腔镜肾部分切除术,54例行机器人辅助腹腔镜肾部分切除术。记录手术时间、热缺血时间(WIT)、术后病理结果。记录术后6个月的血肌酐、患肾GFR、健肾GFR、总GFR、GFR保存率(术后GFR与术前GFR的比值)、患肾FPV、患肾FPV保存率(术后患肾FPV与术前FPV的比值)。患肾FPV通过手术前后的CT影像使用椭球体近似法测量。手术前后GFR和患肾FPV比较采用配对样本t检验。采用Spearman秩相关分析评估各研究因素与患肾GFR保存率间的相关性。多因素分析采用多元线性回归模型分析患肾功能的独立预测因素。以WIT=25 min为截断点将患者分为≤25 min组和>25 min组,比较两组术前患肾GFR,以及术后6个月的患肾GFR和患肾GFR保存率。结果:本组115例手术均顺利完成,中位手术时间135(75~245)min,WIT(24.57±5.51)min。患肾术后GFR(35.50±7.81)ml/(min·1.73 m 2),与术前比较差异有统计学意义(P<0.001),患肾GFR保存率为(74.65±11.10)%。术后6个月患肾FPV保存率为(84.28±4.37)%,与术前比较明显减少(P<0.001)。患肾FPV保存率与患肾GFR保存率呈极强正相关(r=0.802);WIT与患肾GFR保存率呈中等程度负相关(r=-0.503)。多元线性回归分析结果显示,术前患肾GFR(b=-0.150,P=0.008)、WIT(b=-0.443,P<0.001)、患肾FPV保存率(b=1.638,P<0.001)是患肾GFR保存率的独立预测因素。WIT>25 min组和≤25 min组的患肾GFR保存率分别为(68.77±10.88)%和(79.34±8.88)%,差异有统计学意义(P<0.001)。结论:在WIT较短(<30 min)的情况下,正常肾组织数量的保留是影响NSS术后肾功能预后的重要因素,有限的WIT起次要作用。在保证肿瘤完整切除的前提下,应尽可能保留更多的正常肾组织,同时尽量将WIT控制在25 min以内。     

后腹腔镜下保留肾单位9例报告

目的探讨后腹腔镜下保留肾单位手术控制肾蒂及处理肾脏创面的方法。方法我院2003年12月~2007年1月行后腹腔镜下保留肾单位手术9例,术中采取牵拉硅胶管不全阻断肾动脉,在距肿瘤0.5~1.0 cm超声刀切除肿瘤,生物蛋白胶、止血纱布及可吸收线缝合肾脏创面。结果9例手术均获成功,无中转开放手术。手术时间2~3.5 h,平均2.6h;术中出血量50~400 ml,平均150 ml。围手术期无出血、尿漏等并发症。术后病理:8例透明细胞癌,1例血管平滑肌脂肪瘤,标本切缘均为阴性。9例随访4~36个月,平均13个月,肾功能正常,B超或CT显示无局部复发,B超、胸片、骨扫描显示无其他脏器转移。结论掌握一定的控制肾蒂及处理肾脏创面的方法,后腹腔镜下保留肾单位手术安全、有效,可以在临床上推广使用。     

Renal cavernous hemangioma: robot-assisted partial nephrectomy with selective warm ischemia. Case report and review of the literature

Renal hemangioma is a relatively rare benign tumor with a wide range of clinical and radiological presentation, not easy to differentiate preoperatively from a renal cancer. Due to its benign nature complete surgical resection is the recommended therapy and is considered curative.A 73-year old male patient followed-up for a lung carcinoma and a chronic renal failure underwent a CT scan showing a 35-mm mass of the inferior pole of the left kidney.The patient underwent robot-assisted partial nephrectomy with left inferior pole selective warm ischemia. The outcome was favorable and no repercussions on the renal reserve were observed postoperatively.Histopathological characteristics of the surgical specimen were consistent with renal cavernous hemangioma.A robot-assisted operation allows the fine dissection required to carry out a bloodless nephron-sparing surgery without a complete warm ischemia. The use of robot could be noteworthy for nephron-sparing surgery in cases of concomitant chronic renal failure.     

中央型肾癌与外周型小肾癌的保留肾单位手术比较分析

目的 总结中央型肾癌保留肾单位手术特点及临床经验. 方法行保留肾单位手术的.肾癌患者155例.分2组:①中央型38例,其中孤立肾3例,双侧.肾癌5例;38例术前均行磁共振三维血管成像明确患.肾动静脉的解剖结构.②外周型小肾癌117例,其中孤立肾4例,双侧肾癌7例.肿瘤最大径2.0～5.5 cm,临床分期均为Ⅰ期.比较2组患者手术时间、阻断方式及时间、术中出血和术后并发症发生情况.结果 ①38例中央型肾癌患者手术顺利.术后病理诊断为肾透明细胞癌35例、肾嗜色细胞癌2例、肾腺瘤1例.平均手术时间(118±47)min.术中均采用冰屑降温及肾蒂完全阻断,阻断时间(34±16)min.平均出血量150 ml.术后发生尿瘘1例,无继发性出血者.②117例外周型肾癌患者手术顺利.术后病理诊断为肾透明细胞癌106例、肾嗜色细胞癌5例、嫌色细胞癌3例、肾腺瘤3例.平均手术时间(95±39)min.采用单纯肾动脉阻断31例,阻断时间平均(21±9)min.出血量平均250 ml.采用冰屑降温及肾蒂完全阻断86例,阻断时间平均(17±8)min.平均出血量100 ml.术后发生继发性出血4例,无尿瘘发生.中央型组手术时间和肾蒂阻断时间均长于外周型组(P＜0.05);肾蒂完全阻断者手术出血量少于单纯动脉阻断者(P＜0.05),但与肿瘤位置无关P＞0.05).结论 通过细致的术前准备和规范的手术操作,中央型肾癌的保留肾单位手术可以安全完成;除阻断时间稍长外,其他重要的手术指标与外周型小肾癌无明显差异.