~(99m)Tc-DTPAγ摄影机肾脏造影对小儿肾功能与形态的评价

与15年前相比,二乙撑三胺五乙酸~(99m)锝(~(99m)Tc-DTPA)用于肾、脑闪烁造影已较多见。由于~(99m)Tc-DTPA 只通过肾小球滤过从血浆中排出,因此,注射~(99m)Tc-DTPA 后,通过它的血浆清除率测定就可获得肾小球滤过率(GFR)。运用~(99m)Tc-DTPAγ照相可获得相当出色的肾造影影片,可得到肾和尿路的影像,并可得到单个肾脏的总 GFR(相对肾功能)。对于成年人,无需采集血和尿标本,从肾造影就可准确地计算出 GFR(绝对肾功能)。然而,外部测量受几何形状的影响,因此,成人的这种方法未经校正而用于儿童是无效的。     

先天性肾积水患儿肾脏水通道蛋白表达与肾实质厚度和肾小球滤过率的相关性

目的：探讨先天性肾积水患儿肾脏水通道蛋白AQP1-4 的表达与肾实质厚度和肾小球滤过率（GFR）变化之间的关系。方法：利用Western blot检测AQP1-4蛋白在10例先天性肾积水患儿(年龄62.3±18.3个月)10个肾组织和6例来自肾母细胞瘤手术切除患儿的正常肾脏组织(年龄62.7±17.1个月)中的相对表达量。同时对患侧肾脏肾实质厚度和GFR进行评估。积水肾脏AQP1-4表达与GFR以及肾实质厚度之间进行Pearson相关分析检验。结果：肾积水组AQP1-4蛋白相对表达均明显低于正常组（P＜0.05）。B超测量术前积水侧肾脏肾实质厚度平均为4.59±2.25 mm。99mTc-DTPA 测定积水侧肾脏GFR较对侧肾脏明显下降（40±12 mL/min vs 105±20 mL/min, P＜0.05）。积水组肾脏中AQP1-4蛋白相对表达量与肾实质厚度之间呈正相关,与患侧肾脏GFR之间亦呈正相关。积水侧肾脏肾实质厚度与GFR之间呈正相关。结论：先天性积水患儿肾脏AQP1-4蛋白表达下降,其表达量与肾实质厚度和肾脏GFR的变化呈正相关。     

核医学肾脏显像对儿童肾积水患者分肾功能的评估价值

核医学肾脏显像包括肾动态显像和肾静态显像。利尿肾动态显像如应用于儿童肾积水患者,在评估分肾功能的同时,可协助判断尿路梗阻的性质。由于婴儿肾脏肾小球发育不成熟,建议行肾动态显像时,<2岁患者使用肾小管分泌型显像剂^99mTc-双半胱氨酸(^99mTc-EC),>2岁者使用肾小球滤过型显像剂^99mTc-喷替酸(^99mTc-DTPA)。分析肾动态显像的分肾功能及肾图曲线时,需注意各种因素的影响。基于双血浆法的肾小球滤过率(glomerular filtration,GFR)评估肾功能较准确,但因有创、操作复杂而难于普遍应用于临床;基于肾动态显像法评估GFR的影响因素多,需综合判读。肾静态显像主要用于评估肾盂肾炎及肾脏瘢痕的病变范围及严重程度,也可用于评估分肾功能。近年来SPECT/CT在核医学领域广泛应用,如进行^99mTc-DMSA SPECT/CT断层显像,可更准确地评估肾积水患者双肾功能。本文对核医学肾脏显像在儿童肾积水患者分肾功能评估中的价值进行评述。     

应用Schwartz公式和99Tcm-DTPA肾动态显像评估肾小球滤过率的一致性研究

目的 评价应用Schwartz公式评估肾小球滤过率（GFR）的准确性,为儿科临床准确和便捷地评估GFR提供参考。方法 选择2002年 4月至2006 年12月在复旦大学附属儿科医院肾病科诊断为慢性肾脏病（CKD）的患儿为研究对象。患儿于入院后第2日早晨空腹采血,以碱性苦味酸法测定血肌酐（SCr）水平,测量身高,采用Schwartz公式计算GFR（eGFR）。入院后第2或3日行99Tcm-DTPA肾动态显像,获得GFR（mGFR）。采用NKF-K/DOQI推荐的CKD分期标准,依据mGFR分为CKD 1~5期。mGFR与eGFR的相关性采用Pearson相关分析;eGFR估算mGFR的准确性采用相对预测误差（MPE）和绝对预测误差(MAE)表示,eGFR和mGFR的一致性采用Bland-Altman检验。结果 170例CKD患儿进入分析,其中男100例,女70例;年龄2.3～17.8（9.3±3.9）岁。肾病综合征75例,肾小球肾炎28例,泌尿道感染49例,急性肾功能衰竭10例和慢性肾功能衰竭8例。CKD 1期 80例,2期 40例,3期 27例,4期 17例,5期6例。①eGFR和mGFR总体上有显著相关性（r＝0.871）;CKD 1期患儿的eGFR和mGFR呈显著弱相关性,CKD 2~4期患儿eGFR和mGFR无显著相关性。②eGFR预测mGFR的MPE和MAE随肾功能损害程度的加重呈增高趋势,CKD 1期79/80例（98.8%）、CKD 2期32/40例（80.0%）患儿eGFR预测mGFR的MPE均落在±30％内;CKD 3~5期患儿eGFR预测mGFR的准确性较差。③Bland-Altman检验结果提示,eGFR和mGFR的一致性CKD 1和2期患儿较好,CKD 3和4期患儿较差。结论 Schwartz公式对于肾功能损害较轻的CKD 1和2期患儿预测mGFR的准确性较高,CKD 3～5期患儿的准确性较差。对于CKD 3~5期患儿仍应行99Tcm-DTPA肾动态显像以获得准确的GFR。     

肾静态显像联合DWI对儿童上尿路感染的诊断价值

目的评价~(99m)Tc-DMSA肾静态显像方法联合磁共振扩散加权成像(DWI)检查对儿童上尿路感染的诊断价值。方法收集2017年6月至2018年6月就诊于内蒙古医科大学附属医院儿科明确诊断为泌尿道感染的49例患儿的临床资料,所有患儿均进行~(99m)Tc-DMSA肾静态显像、B超及DWI检查,对检查结果进行统计分析。结果 49例患儿中,男性患儿29例,女性患儿20例,年龄3月龄至16岁,2岁以内的患儿15例(30.6%)。明确诊断泌尿道感染的49例患儿中经~(99m)Tc-DMSA肾静态显像检查,结果正常42例(85.7%),异常7例(14%),共7个肾有肾脏损害,其中6例(12%)6个肾呈急性肾盂肾炎改变, 1例(2%)1个肾有肾瘢痕形成; 49例患儿经DWI检查,结果46例(93.8%)正常,3例(6%)3个肾发现肾脏损害,均表现为肾炎改变。49例确诊为泌尿道感染患儿中,有1例(2%)经B超检查发现肾脏病变。三种方法检测结果对比统计分析,DMSA及DMSA联合DWI检测方法检出率均明显高于B超的检出率(P均0.05)。~(99m)Tc-DMSA肾静态显像联合DWI检查共9例(18%)患儿为上尿路感染。结论~(99m)Tc-DMSA肾静态显像联合DWI检查可提高儿童上尿路感染的诊断率。DWI检查可作为上尿路感染的辅助检查方法。     

幼年大鼠阿霉素肾病早期肾组织核转录因子κB与血管紧张素Ⅱ1型受体表达的相关性及干预治疗

目的　探讨阿霉素肾病幼年大鼠肾损伤早期 ,肾组织血管紧张素Ⅱ 1型受体 (AT1)与核转录因子 (NF)κB(P65/Rel A)表达的趋势、相关性及其潜在的病理意义 ,及给予血管紧张素转换酶抑制剂苯那普利和血管紧张素受体阻断剂氯沙坦的干预影响。方法　以阿霉素肾病幼年大鼠为实验性肾病模型 ,于肾病早期第 1、2、3周观察大鼠蛋白尿、血生化各指标的变化。用免疫组化方法检测大鼠肾组织AT1蛋白表达 ,用原位杂交方法检测大鼠肾组织P65/Rel AmRNA表达的情况 ,并从时相和组织定位表达的趋势上评价AT1和P65/Rel A表达的相关性。结果　 ( 1)阿霉素注射后第 1周大鼠即出现明显蛋白尿 ,于第 3周即达大量蛋白尿程度 ( 12 3 2± 7 7)mg/ 2 4h ,同时血生化各指标趋于增高。肾小管中可见大量蛋白管型 ,肾间质中可见大量炎性细胞浸润。 ( 2 )肾小管间质区AT1蛋白和P65/Rel AmRNA表达趋势明显上调 ,第 1、2、3周AT1组化半定量分别为 ( 19 8± 1 1) %、( 2 5 0±2 6 ) %、( 37 1± 1 0 ) % ,假手术组 :( 10 3± 0 8) %、( 10 4± 1 6 ) %、( 10 2± 1 5 ) % ,AT1蛋白主要分布于各级肾小管上皮细胞胞浆和核膜 ,P65/Rel AmRNA表达于小管上皮细胞胞浆 ,随病变的进展 ,其核转位趋势明显增加 ,阳染信号由胞浆转至细胞核 ,P65/R     

每六周皮下注射促性腺激素释放激素类似物缓释剂干预女孩特发性中枢性性早熟

目的　观察延长促性腺激素释放激素类似物 (GnRHa)缓释剂注射间期对特发性中枢性性早熟女孩下丘脑 垂体 性腺轴抑制情况和临床症状改善方面的效果。方法　 4 6例特发性中枢性性早熟女孩随机分为两组 ,A组 :2 6例 ,年龄 (8 3± 1 4 )岁 (6 1～ 11 2 )岁 ,乳房开始发育年龄 (6 6±1 4 )岁 (2～ 8)岁 ,骨龄 (8 9± 1 5 )岁 (6 8～ 11 5 )岁 ;每 6周腹部皮下注射曲普瑞林 3 75mg ,平均剂量为 2 8± 0 6 (1 8～ 4 1) μg/ (kg·d) ;B组 :2 0例 ,年龄 (8 1± 1 3)岁 (5 1～ 10 3)岁 ,乳房开始发育年龄 (7 0± 1 2 )岁 (4～ 8)岁 ,骨龄 (8 9± 1 4 )岁 (6～ 11 5 )岁 ;每 4周肌肉注射曲普瑞林 3 75mg,平均剂量为 4 8± 1 1(3 2～ 7 4 ) μg / (kg·d)。两组均连续用药 1年以上。治疗过程中注意监测以下指标 :性发育情况、身高、体重、生长速率、血性激素的水平、骨龄等。结果　每 6周皮下注射曲普瑞林3 75mg的治疗方案与每 4周肌肉注射普瑞林 3 75mg的治疗方案均能使患儿的第二性征减退、停止发育 ,乳房发育明显受到抑制 ,乳腺回缩或乳腺组织变松软 ;卵巢体积缩小 ,直径大于 0 4cm的卵泡消失 ;促性腺激素分泌减少 ,性激素下降至青春前期水平 ;A、B两组的年生长速率 (cm/年 )分别由治疗前的 6 3±     

乙肝疫苗致血小板减少性紫癜1例

患儿,女,2岁,首次注射乙肝疫苗后,次日颈部出现出血点,第3d遍及全身,查血小板23×10~9/L,收住儿科。查体:T37.6℃,R30次/min,P120次/min,全身皮肤粘膜出血点及淤斑,躯干部为著,心肺无异常,肝右肋下2cm,神经系统无殊,注射处无异常。入院查血小板18×10~9/L,骨髓报告为继发性血小板减少性紫癜,给予强的松20mg/d口服2周,症状消失,第3周用地塞米松7.5mg加葡萄糖静点,14d为1疗程,2疗程后,血小板(140～180)×10~9/L,改口服巩固治疗,血小板     

输尿管不全梗阻致兔肾积水动物模型的肾功能MR相关评估

目的 探讨MR灌注成像技术对积水肾脏肾功能的评估价值.方法 应用塑料套管压迫法建立肾盂输尿管连接部梗阻致肾积水动物模型10只.模型建立前后分别对样本行核医学肾动态显像(单光子发射计算机体层成像SPECT)及MR灌注成像,并以病理检查为佐证,观察样本手术前后各项参数的变化,进行MR灌注参数与核素GFR的统计学相关性分析.结论 积水肾脏皮髓质MR灌注参数,包括积水肾脏的肾实质体积,术前(8400.82±1082.25)mm3,术后(7608.00±951.29)mm3 (P=0.021);基于Patlak技术推算出的分肾功能(Patlak SRF,pSRF),术前(0.5709±0.1472)ml/min,术后(0.3455±0.1226)ml/min(P=0.0092)以及核素扫描测得的GFR,术前(277.6±214.5)ml/min,术后(112.8±89.9)ml/min(P=0.009),均较术前有不同程度的缩小或下降.术前、术后pSRF分别与核素扫描测得的GFR呈高度正相关(术前r=0.712,P=0.041,术后r=0.633,P=0.037);而肾实质体积v术前与核素GFR呈正相关(r=0.673,P=0.028),术后基本无相关性(r=0.312,P=0.079).结论 MR灌注成像技术能够比较全面深入的、定量的评估兔梗阻性肾积水时的肾功能变化;检查能够获取足够的信息,其所获取的肾功能指标与核素检查肾功能指标密切相关.

Abstract：

Objective To study the feasibility of MR perfusion imaging in assessing renal function of hydronephrosis in animal model.Methods Ten New Zealand rabbits(10-week-old,Male) underwent ureteropelvic junction obstruction by embolied the right ureter of ureteropelvic junction into a plastic cannula.Before and two months after the surgery,the animals were performed both on SPECT and MR perfusion,MR perfusion parameters were assessed,and statistical analyses were performed on the GFR of SPECT and MR perfusion parameters.Results Majority MR perfusion parameters,such as volume of renal parenchyma(7608.00±951.29 mm3) and pSRF(0.3455±0.1226 ml/min) after operation were decreased than those before operation(8400.82±1082.25 mm3,0.5709±0.1472 ml/min,respectively).Statistic analysis showed that pSRF had good statistically relationship with GFR of SPECT(preoperation r=0.712,p=0.041,postoperation r=0.633,p=0.037).However,there were no obvious relation of volume of renal parenchyma with GFR after operation(r=0.312,p=0.079).Conclusions MR perfusion imaging can accurately assess animal model' renal function in hydronephrosis.MR perfusion nephrogram can provide the sufficient message which may have statistically relation with GFR of SPECT.     

消化系统疾病

071845胆道闭锁的临床及影像学诊断/杨吉刚…∥实用儿科临床杂志.-2006,21(23).-1668~1670071846培菲康治疗小儿厌食症的临床疗效观察/许春娣…∥中国实用儿科杂志.-2007,22(2).-146试验组(125例)口服培菲康散剂,用法为2次/d,6个月至1岁,1支/次;~3岁,2支/次;~5岁,3支/次。对照组(124例)口服胃蛋白酶合剂,用法为3次/d,6个月至1岁,2.5mL/次;~3岁5mL/次;~5岁,10mL/次。疗程4周。结果:临床症状总改善率在试验组为84.0%,对照组为54.0%。食欲、进食量和进食时间的改善率在试验组分别为80.8%、88.8%和64.8%;在对照组分别为67.7%、66.9%与50.8%,2…     

早产儿视网膜病的危险因素分析及随访调查

研究早产儿视网膜病(retinopathy of prematurity,ROP)的发生率、高危因素、治疗与随访情况。方法对2005年7月-2007年12月温州医学院附属第一医院NICU收治的符合ROP筛查标准的早产儿,于生后2周开始由资深眼科医师开始行间接眼底镜检查眼底,并进行随访。结果434例早产儿中ROP的发生率为5.5%(24/434例),24例ROP中Ⅰ期19例,Ⅱ期3例,Ⅲ期2例。Ⅲ期阈值病变者行激光光凝治疗,全部患儿均恢复正常。对434例早产儿行单因素分析得出,胎龄、出生体重、住院时间、吸氧、吸氧浓度、吸氧时间、呼吸暂停、新生儿肺透明膜病(RDS)、肺表面活性剂(PS)的应用、机械通气、输血、光疗时间、感染与ROP的发生有相关性(P<0.05)。Logistic回归分析显示胎龄、出生体重、胎数、吸氧时间、光疗时间、代谢性酸中毒、母亲妊高症、颅内出血是影响ROP发生的主要因素。结论早产是ROP的根本原因,防治各种并发症、合理的氧疗是预防ROP的关键。建立完善有效的ROP筛查制度,早期发现、早期治疗ROP,可改善ROP的预后。     

Outcome of the Treatment of Inborn Errors of Metabolism

The use of specific dietary restrictions, cofactor administration, mobilisation of insoluble substances, environmental modifications, product replacement and selective enzyme inhibition are now established for the treatment of some inborn errors of metabolism. There is no generally accepted application for enzyme administration, cytopharmacology (manipulation of the cytoskeleton) or for cell transplantation except for bone marrow transplantation in disorders where the bone marrow is primarily at fault. The other uses of bone marrow transplantation which have been proposed require further evaluation. Results of recent research suggest that the scope of this approach is gradually being widened. There is also scope for development in the field of organ transplantation taking advantage of recent technical¹ and immunological progress. The treatment of inborn errors of metabolism by genetic modification is not yet on a practical clinical level; more laboratory and animal studies are needed before this can be attempted in man. Adenosine deaminase deficiency appears to be the disease in which this will be first attempted using a retroviral vector to insert the gene into the genome of pluripotential bone marrow cells.     

婴幼儿法乐四联症手术治疗近期疗效的影响因素

目的 探讨影响婴幼儿法乐四联症手术治疗近期疗效的各相关因素.方法 将2003年8月至2011年2月经作者一期手术纠治的117例年龄≤3岁的法乐四联症患儿分为疗效良好、疗效较差两组.分析手术时患儿年龄、体重、术前HCT值、McGoon指数、EDVI、主动脉阻断时间、转流时间、室间隔缺损大小、升主动脉与肺动脉干直径比、...     

术中判断肠活力三种方法的比较

术中准确地评价肠活力是外科医师面临的一个普遍问题。应用兔肠缺血模型，比较静脉荧光素、表面血氧测定和激光多普勒三种技术判断肠活力的精确性。结果：诊断效率静脉荧光素法为７８％，表面血氧测定为６８％，激光多普勒为９５％。后者精确度显著优于前二者，且操作简便、迅速，具有临床应用前景。     

Treatment of retinopathy of prematurity

Retinopathy of prematurity is a potentially blinding disorder of premature infants. Retinal ablation of the avascular retina originally described using cryotherapy but now most commonly undertaken with laser photocoagulation, reduces the unfavourable structural outcomes and improves the functional visual acuity outcome. The CRYO-ROP study showed the long-term benefit of treatment of threshold disease compared with no treatment, however even with cryoablation 44.4% of treated eyes had a visual acuity of 6/60 or worse at 10 year follow-up. The ETROP study of earlier treatment for high-risk pre-threshold disease, rather than treatment at threshold, has shown that pre-threshold treatment of type 1 disease produces a significantly improved outcome. Despite treatment some infants develop retinal detachment for which various surgical treatments have been described, although not always with a good functional outcome. Future treatment modalities may include the use of anti-VEGF therapies.     

Spectrum of clinical presentations of vein of Galen aneurysm

Aneurysm of the vein of Galen is a rare intracranial vascular malformation. It is known to have diverse manifestations and varying severity. Four cases with different modes of presentation and outcome are reported. A mortality of 50 per cent was encountered. Among the survivors, one had neurologic sequelae whereas the other had attained age-appropriate developmental milestones. The former was a rare case of spontaneous thrombosis of the aneurysm while the latter was a boy who underwent therapeutic embolization.     

主动脉缩窄的治疗研究进展

主动脉缩窄(coarctation of aorta,CoA)是主动脉的局限性狭窄,狭窄最常见于峡部,是一种常见的先天性心血管畸形,占所有先天性心脏病的5％ ～8％.缩窄导致上肢血压升高,下肢血压降低,并可引起心功能下降,未经治疗的CoA预后不佳.治疗方式包括外科治疗及经皮介入治疗,不同治疗方式的并发症发生率不同,术后长期监测其并发症、心功能是评估预后的重要指标.该文对主动脉缩窄的治疗方式及术后并发症、心功能情况的研究进展作一综述.     

Evaluation of the Efficacy of Treatment of Kawasaki Disease before Day 5 of Illness

We evaluated the efficacy of treating Kawasaki disease earlier than Day 5 of illness with a standard dose of immunoglobulin and aspirin. We performed a case–control study of patients with Kawasaki disease admitted to Princess Margaret Hospital from 1994 to 1999. Patients with pretreatment coronary aneurysm or those treated after day 10 of illness were excluded. All patients received immunoglobulin (2 g/kg) and aspirin (80–100 mg/kg/day) until fever subsided for 48 hours. Immunoglobulin retreatment was given for persistent fever 48 hours after the first dose of immunoglobulin or recrudescent fever. The case group consisted of 15 patients who received treatment earlier than day 5 of illness, and the control group consisted of 66 patients who were treated on or after day 5. Patients sex, age, duration of posttreatment fever, need for additional immunoglobulin, and coronary artery status were noted. Treatment efficacy was assessed by the duration of posttreatment fever and the prevalence of coronary artery aneurysms. Eighty-one patients were included in this study. There were 15 patients in the case group and 66 in the control group. No significant difference was noted in age and sex between the case and control groups. Thirty-three percent (5/15) and 8% (5/66) of the case and control groups, respectively, had persistent/ recrudescent fever 48 hours after the first dose of immunoglobulin that required retreatment (p = 0.017). Thirteen percent (2/15) and 5% (3/66) of the case and control groups, respectively, had coronary aneurysms (p = 0.158). Treatment of Kawasaki disease before day 5 of illness was associated with persistent/recrudescent fever that required retreatment. However, there was no significant increase in the prevalence of coronary aneurysm if retreatment was given. Poster presented at the third World Congress of Pediatric Cardiology and Cardiac Surgery, Toronto, Ontario, Canada, 2001     

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目的探讨脑性瘫痪的诊断以利防治。方法对南京市儿童医院早期干预门诊确诊的640例脑患儿[早产儿258例(早产儿组),足月儿382例(足月儿组)]分析其病因、早期临床表现及头颅CT征象,进行体格神经系统检查并智测。结果(1)高危因素,两组在颅内出血、肺部疾病早产儿组明显高于足月儿组,缺氧缺血性脑病组足月儿明显高于早产儿组,P均<0·05外余P均>0·05。(2)6个月以内确诊早产儿、足月儿两组各为28、90例的临床表现,反应差、嗜睡、少吃、少哭、少动,肌张力低下,早产儿组明显高于足月儿组,而不停啼哭,护理困难,肌张力增高,足月儿组明显高于早产儿组,P均<0·01及0·05余P均>0·05。(3)早产儿、足月儿两组的身长、体重、头围、MDI和PDI各为73·24±9·82、73·58±11·64(cm),9·12±2·31、9·23±2·80(kg),44·15±3·47、42·99±4·23(cm),44·42±6·09、41·99±7·28(分),43·81±4·95、40·80±4·51(分)。(4)头颅CT表现。早产儿、足月儿两组CT异常率各为95·4%及89·8%,P<0·05。(5)发育落后早产儿组言语落后明显高于足月儿组外,足月儿组身长<2SD明显高于早产儿组P均<0·05,余P均>0·05。结论当患儿具有高危因素、异常临床表现与神经症状、发育明显落后,应结合头颅CT密切随访可早期诊断脑性瘫痪及早干预。     

小儿法洛四联症根治术106例临床分析

目的探讨小儿法洛四联症根治术的特点,以提高手术疗效。方法对1998-02—2005-07在河南省郑州市第七人民医院接受法洛四联症根治术的106例患儿的临床资料进行回顾性分析。结果治愈103例,死亡3例,病死率为2.9%。术后随访率100%,随访3～93个月,无晚期死亡。全部病例心功能皆恢复至Ⅰ或Ⅱ级(NYHA)。结论绝大部分法洛四联症患儿可施行一期根治术,提高手术成功率的关键在于合理的体外循环灌注、严密修补室间隔缺损和恰当疏通右室流出道。