微创经皮肾镜取石术后高热的治疗体会

目的探讨微创经皮肾镜取石术后高热的治疗体会。方法回顾性分析2005年3月至2009年6月微创经皮肾镜取石术后出现高热（体温高于39．0℃）的11例患者的一临床资料。结果在11例高热患者中,2例采用单纯抗生素及对症治疗,9例需采用外科处理,其中包括调整肾造瘘管位置及更换双J管8例,输尿管镜取石1例,11例患者均得到治愈。结论微创经皮肾镜取石术后出现高热的患者,虽然合理使用抗生素是必需的,但是积极的外科处理也是极其重要的。     

三聚氰胺所致婴幼儿肾脏改变：142例分析

目的分析三聚氰胺所致婴幼儿肾结石和肾盂积水的临床表现和处理。方法大同市2253例用过含三聚氰胺奶粉的婴幼儿接受诊治,其中男童1098例,女童1155例,18例〉3岁,其余在3岁以下。均以问题奶粉为主要食物。结果B型超声检查发现肾脏改变142例（6.30％）,其中单纯肾结石101例（男孩61例,女孩40例）,单纯肾盂积水26例（男孩24例,女孩40例）肾结石并发肾盂积水15例（男孩13例,女孩2例）。17例肾结石直径1〉0.4cm,最大的结石直径1.0cm。男孩发病率为8.92％,显著高于女孩（3.81％,P〈0.05）。主要症状包括排尿时哭闹,尿血和腹痛。尿改变39例,其中尿潜血9例、血尿26例、蛋白尿4例。治疗包括停止食用问题奶粉,多饮水,鼓励排尿以增加结石排出的机会和应用低张碱性液体。住院患儿均治愈。结论三氯氰胺进入以污染奶粉为食的婴幼儿体内可造成肾结石和肾积水。男童发病率高于女童。三聚氰胺所致婴幼儿肾结石和肾盂积水经保守治疗有效。     

A retrospective evaluation of Randall's plaque theory of nephrolithiasis with CT attenuation values

Purpose

We examined the computed tomography attenuation values (HU) of renal papillae in stone formers (SF) to determine whether nephrolithiasis is associated with radiographic changes in renal papillae to investigate the Randall’s plaque theory.

Materials and methods

Two observers independently and retrospectively recorded the HU of the renal medullae and cortex in 90 patients with a unilateral single calculus within kidney or ureter, and in 104 cases in control group (CG) matched for age and renal functions.

Results

The patient ages were similar in the stone former and control groups. However, the male-female ratio was significantly greater in the SF group (68:22) than in the CG (42:62, P < 0.0001). Left-right ratio in SF group was 50:40. The inter-rater agreement was κ = 0.53 (95% CI: 0.42, 0.64).Mean HU of all papillae of affected side in stone-formers (ASSF) was significantly greater than that in CG (39.6 versus 29.6, P < 0.0001). When comparing affected and non-affected sides within the SF group, there was no significant difference (39.6 versus 38.4, P = 0.16). The receiver operating characteristic (ROC) analysis showed area under curve = 0.94 with optimal cut-off at 34 HU. At this point the specificity, sensitivity, PPV and NPV were 90%, 90%, 33% and 99%, respectively.

Conclusion

HU of the renal papilla is significantly increased in SF in the affected and the non-affected kidneys when compared to the CG. This finding may form one of the risk indicators to determine the future follow up and clinical management for the potential SF.     

Oral Zinc Sulfate as Adjuvant Treatment in Children With Nephrolithiasis: a Randomized,Double-Blind,Placebo-Controlled Clinical Trial

Background:

Nephrolithiasis in children is associated with a high rate of complications and recurrence.

Objectives:

Since some evidences reported that zinc has an important place amongst inhibitors of crystallization and crystal growth, we decided to assess the effectiveness of oral zinc sulfate as adjuvant treatment in children with nephrolithiasis.

Patients and Methods:

This was a randomized, double-blind, placebo-controlled clinical trial. 102 children in the age range 1 month to 11 years with first nephrolithiasis were recruited. Patients were randomly divided into two equal groups (intervention and control groups). Intervention group received conservative measures for stones and 1 mg/kg/day (maximum 20 mg/day) oral zinc sulfate syrup for 3 months. Control group received placebo in addition to conservative measures, also for 3 months. Patients were followed up by ultrasonography for 9 months, in 5 steps (at the end of 1st, 2nd, 3rd, 6th and 9th month after treatment) assessing size and number of stones in the kidneys.

Results:

Only at the end of the first month, the average number (intervention: 1.15 ± 3.78, control: 1.3 ± 2.84) (P = 0.001) and size (cm) (intervention: 0.51 ± 1.76, control: 0.62 ± 1.39) (P = 0.001) of stones was significantly lower in the intervention group, and in other points there was no significant therapeutic efficacy in oral zinc adjuvant treatment compared to conservative treatment alone. Also, during the 9-month follow-up, the number and size of stones in both groups decreased significantly (both: P < 0.0001) in a way that the decrease in the intervention group showed no difference with the control group.

Conclusions:

Adjuvant treatment with zinc is not more effective than consecutive treatment in children with nephrolithiasis. However, further studies are recommended due to the lack of clinical evidence in this field.     

Cornelia de Lange Syndrome Presenting with Nephrolithiasis and Urolithiasis

We describe a rare case of a viable preterm male infant with Cornelia de Lange syndrome presenting with elevated arterial blood pressures within the first several days of life. Renal ultrasonography revealed hydronephrosis, nephrolithiasis, and urolithiasis. Investigative kidney imaging may be indicated in patients with this syndrome.     

Pelvico-calyceal system rupture due to staghorn calculus with urinoma formation in a boy with neurofibromatosis type 1 and quadriplegia

Nephrolithiasis is a rare condition in children. The urinary tract rupture related to stones formation or migration is atypical in children, but creates serious consequences. We present a case of a 17-year-old quadriplegic patient with neurofibromatosis type 1 and urinoma due to the rupture of calyceal fornices in the course of nephrolithiasis. The boy was admitted with symptoms of severe pneumonia complicated with sepsis and prerenal acute kidney injury. Abdominal ultrasound revealed stone casts in both renal pelvises. Antibiotics, fluid therapy and diuretics were used to improve patient's condition. On the 28th day gross hematuria was observed. The patient's condition was stable, without signs of pain or discomfort. Abdomen ultrasound showed heteroechogenic structure (125 mm × 100 mm × 100 mm) localized between the lower surface of the liver and the right kidney. Contrast CT scan confirmed urinoma under the right kidney capsula. Because of the high risk of its rupture, decision of invasive evacuation of perirenal fluid was made. Using the percutaneous catheter 700 ml of bloody fluid was drained. After 10 days catheter was removed without recurrence of urinoma. Concluding, in children with prolonged immobilization this condition should be taken into consideration in differential diagnosis, also special attention should be paid for accompanying scarce symptoms.     

Characterization of ureteral stents by dual-energy computed tomography: Clinical implications

Dual-energy computed-tomography (DECT) has been suggested as the method of choice for imaging urinary calculi due to the modality’s high sensitivity for detecting stones and its capability of accurately differentiating between uric-acid (UA) and non-UA (predominantly calcium) stones. The clinical significance of the latter feature relates to the differences in management of UA vs non-UA calculi. Like calculi, ureteral stents are assigned color by the dual-energy post-processing algorithm, which may lead to improved or worsened stone visualization based on the resulting stent/stone contrast. Herein we depict the case of a nephrolithiasis patient with bilateral stents, each with different color, clearly displaying the effect of stent color on stone visualization. Further, three-dimensional reconstruction of the DECT images illustrates advantages of this enhancement compared to conventional two-dimensional computed tomography. The resulting stent/stone contrast produces an unanticipated potential advantage of DECT in patients with urolithiasis and stents and may promote improved management decision-making.     

原发性高草酸尿症3型8例病例系列报告并文献复习

背景：原发性高草酸尿症（PH）是一种罕见的由于先天性肝内乙醛酸代谢异常导致的遗传性肾结石/肾钙质沉着症,既往多关注1型和2型PH,PH3的致病基因HOGA1发现较晚,报告不多。 目的：总结PH3临床表型,探讨不同种族人群的PH3热点变异。 设计：病例系列报告。 方法：纳入2015年1月至2021年12月复旦大学附属儿科医院经HOGA1基因变异确诊为PH3的连续病例。从住院病史中采集临床和生物学检测信息,在PubMed、Embase、万方数据库和中国知网数据库中检索PH3病例的中、英文文献,采集病例来源（国家）、例数、性别、起病年龄、诊断年龄、起病临床表现（尿石症、肾钙质沉着症、高钙尿症、高草酸尿症）、随访时间、肾功能（慢性肾脏病2期、3期、4~5期）、随访年龄、尿路结石转归 (活动性结石、无症状结石或结石消失)、HOGA1基因变异位点。 主要结局指标：临床表型和不同种族人群的热点变异。 结果：纳入8例PH3患儿,男7例,女1例;起病年龄中位数10月龄,诊断年龄中位数16月龄。3例以肉眼血尿起病,5例以泌尿道感染起病。影像学均提示肾结石,均无肾钙质沉着表现。3例检测了24 h尿草酸,1例提示高草酸尿症;6例检测了尿钙,5例提示高钙尿症。1例失访,7例随访中位时间25个月,肾小球滤过率均维持稳定,3例肾结石消失。8例均检出HOGA1基因变异（共10个变异位点）,其中复合杂合变异5例,纯合变异3例,经ACMG分级判定6个位点为可能致病变异,4个位点为致病变异。中英文数据库共检索到82篇文献,筛选后23篇文献中321例PH3患者进入本文分析,中国36例（包括本文8例）,欧美293例。中国和欧美PH3患者：起病表现为尿石症的比例分别为83%(30/36)和85%(195/230),肾钙质沉着症分别为3%(1/29)和8%(20/261),高草酸尿症分别为90%(26/29)和96%(66/69),差异均无统计学意义;高钙尿症分别为44%(11/25)和23%（34/150),差异有统计学意义;末次随访时肾功能：中国1例PH3患者25岁时进展至终末期肾病,欧美2例PH3患者分别在8岁和33岁进展至终末期肾病;活动性结石：中国和欧美PH3患者分别为13%(3/23)和37%(22/59),差异有统计学意义。中国PH3患者热点变异为c.834G>A (splice site)、c.834_c.834+1GG>TT (splice site)和c.769T＞G (p.C257G),分别占28%(20/72)、21%(15/72)和11%(8/72);欧美PH3患者热点变异为c.700+5G>T (splice site)和c.944_946delAGG(p.E315del),分别占40%(236/586)和12%(73/586)。 结论：PH3起病年龄和诊断年龄较早,整体预后较PH1和PH2良好,中国与欧美PH3患者HOGA1基因突变可能存在不同的热点变异位点。