伴排尿障碍的隐性脊柱裂患儿的尿动力学特点

目的 研究伴有排尿功能障碍的隐性脊柱裂患儿的尿动力学特征.方法 对113例有排尿障碍的患儿进行尿动力学检查,其中48例经X线确诊为隐性脊柱裂者为观察组,无脊柱裂65例为对照组.检测项目包括:尿流率测定、充盈期膀胱压力容积测定、压力流率测定、同步括约肌肌电测定、静态尿道压力测定.比较两组间主要尿动力参数异常的发生率.观察组按主要临床症状分为尿失禁、尿频、单纯夜间遗尿和排尿困难4组,应用统计学研究临床症状与尿动力学主要参数的相关性.结果 在检测中发现观察组48例中有46例有不同程度的异常.其中逼尿肌过度活动22例,排尿期逼尿肌活动低下和无收缩21例,最大尿流率降低18例,膀胱容积缩小15例,残余尿量增多12例,低顺应性膀胱7例,逼尿肌外括约肌协同失调4例,最大尿道压降低4例.观察组中逼尿肌过度活动、逼尿肌活动低下、残余尿量增多及低顺应性膀胱发生率更高.按临床症状来看,隐性脊柱裂伴有尿失禁的患儿更多的表现为逼尿肌活动低下及最大尿流率降低,尿频的患儿在尿动力检查中多表现为逼尿肌过度活动及残余尿增多,排尿困难的患儿逼尿肌活动低下的发生率更高,而遗尿的患儿更易检出逼尿肌过度活动.结论 小儿隐性脊柱裂伴有排尿功能障碍的患儿具有多种尿动力学改变,且相同的症状可表现为不同类型的尿动力学异常,临床症状与尿动力学参数有一定的相关性,尿动力检查为其临床诊断和治疗方案制定提供重要客观依据.     

超声测量膀胱壁厚度预测脊柱裂患儿上尿路损害

目的 通过超声测定膀胱壁厚度和尿动力学检查测定膀胱功能,评价隐形脊柱裂患儿膀胱厚度和功能及上尿路损害的相关性,探讨用膀胱壁厚度评估隐性脊柱裂患儿上尿路损害的可能性.方法 选取超声检查确诊上尿路扩张的隐性脊柱裂患儿22例,年龄(8.8±4.9)岁,并选择同期超声检查无上尿路扩张的隐性脊柱裂患儿29例作为对照组,年龄(9.3±5.3)岁.所有患儿均行尿动力学检查,记录最大膀胱容量,充盈期最大逼尿肌压力,逼尿肌漏尿点压和逼尿肌过度活动最高压力.在膀胱充盈至预测正常膀胱容量的60%时行超声检查测量逼尿肌厚度.同时根据超声检查是否扩张将患儿分为有和无上尿路损害组,比较两组膀胱壁厚度的差异,并分析膀胱厚度与尿动力学参数相关性,计算膀胱壁厚度预测上尿路损害统计学指标.结果 上尿路损害组平均膀胱壁厚度(3.4±0.25)mm,显著高于无上尿路损害组的(2.5±0.45)mm,差异有统计学意义(P＜0.05).膀胱壁厚度与逼尿肌过度活动最高压力、逼尿肌漏尿点压和充盈期最大逼尿肌压力均呈正相关(r=0.87、0.91和0.85,P＜0.0001,P＜0.0001和P=0.017).膀胱壁厚度≥3.0 mm预测上尿路损害的灵敏度为90.9%,特异性为79.4%,阳性预测值76.9%,阴性预测值为92.0%.受试者工作特征曲线(ROC)显示超声测量膀胱壁厚度能高度预测隐形脊柱裂患儿上尿路损害的发生,曲线下面积(AUC)为0.929.结论 超声测定隐形脊柱裂患儿膀胱壁厚度可以帮助预测上尿路损害,膀胱壁厚度大于3.0 mm提示隐性脊柱裂患儿上尿路损害可能性大.     

前尿道瓣膜切除术后膀胱功能异常与上尿路损害的关系

目的探讨前尿道瓣膜切除术后造成上尿路持续损害的尿动力学危险因素。方法回顾性分析2007年1月至2020年1月26例前尿道瓣膜切除术后患儿的临床资料,平均年龄3.4岁(5个月至14岁)。瓣膜切除术后4个月至12.5年,平均5.5年。患儿术后均进行尿动力学检查。手术前后均进行血生化(包括尿素氮、肌酐)检查、泌尿系统B超检查、静脉肾脏造影(intravenous pyelography,IVP)和排尿性膀胱尿道造影(voiding cystourethrogram,VCUG)。比较瓣膜切除前后肾和输尿管积水以及膀胱输尿管反流情况,分析造成前尿道瓣膜术后上尿路持续损害的危险因素。结果前尿道瓣膜切除手术前肾和输尿管积水患儿共15例24侧,占57.7%(15/26);膀胱输尿管反流8例11侧,占30.8%(8/26)。瓣膜切除术后有5例7侧肾和输尿管积水消失,占19.2%(5/26);2例3侧膀胱输尿管反流消失。瓣膜切除术后肾和输尿管积水患儿共10例17侧,占38.5%(10/26);膀胱输尿管反流6例8侧,占23.1%(6/26)。有7例11侧肾和输尿管积水较术前加重,占26.9%(7/26),其中4例6侧膀胱输尿管反流较术前加重。根据术后肾和输尿管积水以及膀胱输尿管反流恢复情况,分为上尿路损害加重组(7例)和上尿路损害减轻或消失组(19例)。行瓣膜切除术后尿动力学检查发现,在上尿路损害加重组7例患儿中,压力流率图显示5例依然存在下尿路梗阻或可疑梗阻。上尿路损害减轻或消失组19例患儿压力流率图均显示无梗阻(P<0.05);两组最大尿流率平均值、膀胱顺应性、排尿期最大逼尿肌压力值差异均存在统计学意义(P<0.05);上尿路损害加重组中5例动态VUCG显示排尿时膀胱颈全程开放不全,行膀胱尿道镜检查发现3例膀胱壁增厚,呈小梁样改变和膀胱假性憩室形成,尤其膀胱基底以及膀胱内口附近组织明显增厚。结论前尿道瓣膜是一种罕见的下尿路梗阻性疾病,瓣膜切除之后上尿路损害仍然会持续存在或加重,可能与患儿异常的膀胱功能有关。排尿期最大逼尿肌压力升高、最大尿流率低、膀胱顺应性低以及压力流率图显示梗阻仍存在是导致膀胱功能异常的尿动力学危险因素,可能与前尿道瓣膜患儿同时存在膀胱颈部功能与结构的异常有关。     

重视小儿排尿功能障碍的诊治

小儿排尿功能障碍多见。常见临床表现有尿频、尿急、尿痛、尿失禁、排尿困难和遗尿等。正确诊断是成功有效治疗的基础。尿动力学检查的普及显著提高了诊断和治疗水平,已经成为诊断排尿障碍的常用工具。小儿首选无创尿动力学检查包括排尿日记、尿流率和残余尿测定及排尿方式观察等。有创尿动力学检查包括膀胱尿道置管测压和影像尿动力学检查。引起排尿异常的常见病有排尿功能发育延迟、泌尿系感染、神经源性膀胱、膀胱过度活动症(OAB)、遗尿症等。尿动力学检查有助于理解发病机制和分类从而科学制定治疗方案。排尿功能障碍除了影响生活质量外,常引起逼尿肌和上尿路继发损害,甚至危及生命。因此,应重视小儿排尿功能障碍的诊治。     

小儿后尿道瓣膜切除后合并排尿异常的尿动力学研究

目的 探讨经尿道镜后尿道瓣膜切除术后伴有排尿异常患儿上尿路、膀胱功能及预后.方法 回顾性分析2002年7月至2012年2月收治的行尿道镜电灼后尿道瓣膜术后获得随访的58例患儿的病例资料,归纳总结其存在的临床症状、影像学异常以及尿动力学检查结果,并将其分为排尿正常组(10例)和排尿异常组(48例),对其年龄分布、上尿路情况、尿动力学参数进行比较分析.结果 术后仍存在的临床症状有:尿失禁18例(31.0％),反复泌尿系感染8例(13.8％),排尿费力、滴尿15例(25.9％),尿频4例(6.9％),尿不尽5例(8.6％),无明显症状10例(17.2％),肾功能衰竭3例(5.2％).影像学检查示:术后仍存在双肾积水者50例91侧(86.2％),存在膀胱输尿管反流23例27侧(39.7％).58例患儿中56例(96.6％)存在不同程度的尿动力学异常.逼尿肌不稳定者占16例(27.6％％);逼尿肌收缩无力者6例(10.3％);残余尿量＞10 ml者25例(43.1％);腹压参与排尿者23例(39.7％).比较排尿正常组与排尿异常组单侧肾输尿管积水比例(11.1％ vs 88.9％)、双侧肾输尿管积水比例(9.8％ vs 90.2％)、单侧膀胱输尿管反流比例(6.7％ vs 93.3％)、双侧膀胱输尿管反流比例(12.5％ vs 87.5％),差异均具有统计学意义(P＜0.05).排尿正常组与排尿异常组逼尿肌漏尿点压力[(29.1±5.5)CmH2O vs (50.4±4.8)CmH2O]、膀胱顺应性[(12.1±3.8)ml/cmH2O vs (4.0±0.1)ml/cmH2O]、残余尿量比较[(21.3±8.1)ml vs (45.7±9.6)ml],差异均有统计学意义(P＜0.05).结论 后尿道瓣膜患儿解除梗阻后多数患儿仍存在不同程度膀胱功能问题,排尿异常组膀胱功能及上尿路情况明显差于排尿正常组.后尿道瓣膜患儿术后应注意排尿情况,定期做尿动力学检查,对症处理,以更好的保护肾功能.     

脊髓栓系的尿动力学表现及临床意义

目的：探讨及评价脊髓栓系的尿动力学表现及临床意义。方法：对66例脊髓栓系患儿行尿动力学检查、MRI和排泄性膀胱尿道造影。结果：58例患儿发现有尿动力学异常改变，逼尿肌反射亢进35例，反射低下25例，逼尿肌括约肌不协调27例。54例膀胱顺应性，逼尿肌压增加，53例 残余尿增加，51例膀胱容量减少。8例尿动力学表现正常。脊髓圆锥位置正常5例，位于L3－L5间32例，L5以下29例。输尿管反流16例。结论：脊髓栓系可导致尿动力学发生不同的异常改变，脊髓圆锥位置与尿动力学表现的类型及上尿路损害无相关关系，上尿路损害与逼尿肌括约肌不协调、逼尿肌压和患儿的年龄密切相关。尿动力学检查是评价下尿路功能和治疗的客观指标。     

腰骶部脊髓脊膜膨出并发神经源性膀胱的临床研究

随访，复查腰骶部囊性脊柱裂术后患儿，观察神经源性膀胱发病情况。方法：对３８例行尿流动力学，排尿性光膀胱尿道造影，Ｂ超及静脉尿路造影检查。结果；１．脊髓脊膜膨出占囊性脊柱裂的６２％，脊髓脊膜同并发神经源性膀胱发病率为９６％；２．骨质缺损≥１．５ｃｍ×１．５ｃｍ多为脊髓脊膜膨出。３．共有８例上尿路功能受损，残余尿量均≥６０ｍｌ，其中４例充盈期膀胱内压力≥１．９６ｋＰａ，而３例膀胱逼尿肌－尿道括约肌     

尿动力学检查对小儿神经源性膀胱手术方式选择的意义

目的 探讨尿动力学检查对小儿神经源性膀胱手术方式选择的意义.方法 回顾性分析我院手术治疗的神经源性膀胱患儿的资料41例.所有患儿术前完成B型超声、排尿性膀胱尿道造影(voiding cystourethrogram,VCUG)、99mTc-DTPA肾动态显像(DTPA)、尿动力学检查.其中34例行储尿期+排尿期膀胱压力描记.结果 尿动力学检查提示逼尿肌过度活动23例,活动低下11例;括约肌过度活动21例,活动低下13例.根据患儿不同的临床症状,参考尿动力学检查结果,选用不同的手术方式.具体术式包括:回肠代膀胱扩大+阑尾代可控性膀胱流出道(Mitrofanoff)+阑尾代顺行结肠灌洗造瘘(Malone)+膀胱颈悬吊(Sling)2例,回肠代膀胱扩大+Mitrofanoff+ Sling 5例,回肠代膀胱扩大+输尿管再植+ Mitrofanoff+ Sling 7例,回肠代膀胱扩大+Mitrofanoff+右肾切除+Sling 1例,回肠代膀胱扩大+输尿管再植+ Mitrofanoff 18例,回肠代膀胱扩大+Mitrofanoff 2例,输尿管再植+Mitrofanoff 3例,膀胱颈封闭+回肠代膀胱扩大+Malone+回肠卷管代可控性膀胱流出道(Monti)1例,Mitrofanoff 2例.术后39例患儿获得随访,随访时限3个月～5年,7例出现术后并发症,大部分患儿术后恢复满意.结论 小儿神经源性膀胱的手术方式选择主要依据其临床症状以及超声、影像、同位素检查结果,尿动力学检查可明确膀胱尿道功能障碍的类型,对于手术的方案制定也有重要参考意义.     

瓣膜膀胱综合征尿动力学研究

目的　探讨瓣膜膀胱综合征(VBS)患儿的尿动力学表现及其意义。方法　16 例男性后尿道瓣膜电切术后患儿,年龄平均(3.2±1.8)岁。分为2组,全部患儿均在2岁前行后尿道瓣膜电切术。第一组7例,平均年龄(1.6±0.3)岁,术后至尿动力学检查间隔时间小于1 年。第二组9 例,平均年龄(4.5±1.2)岁,间隔时间大于1年,平均(3.5±1.3)年。对比两组尿动力学结果。结果　第二组最大逼尿肌收缩压(Pdet.max)和膀胱顺应性(BC)分别为(56.2±14.1) cmH2 O和(12.5±7.4)ml/cmH2O,低于第一组Pdet.max(95.1±18.3 ) cmH2 O和BC(52.4±26.9) ml/cmH2 O;第二组残余尿量(PVR)和最大膀胱容量(MBC)分别为(96.6±52.4) ml 和(217.4±61.7) ml,大于第一组PVR(42.8±38.9) ml和MBC(138.1±20.1) ml,差异有统计学意义(P<0.05)。两组分别有4 例和2例有逼尿肌不稳定(DI),Fisher’s精确概率检验两者差异无统计学意义(P>0.05)。在第二组中2例逼尿肌无收缩,有4 例出现较特殊的排尿,表现为逼尿肌收缩波不光滑, 该4 例Pdet.max平均(62.3±9.1) cmH2O,PVR平均(87.5±41.9) ml。结论　VBS膀胱功能异常多发.年龄较大患儿逼尿肌功能较年龄较小患儿差。尿动力学检查能及时发现膀胱功能异常和指导下一步治疗。因此,所有PUV患儿均应行该检查以了解膀胱功能,     

肛门直肠畸形术后排尿功能障碍的评价和治疗

目的 评价肛门直肠畸形术后排尿功能障碍的原因及治疗对策.方法 肛门直肠畸形术后患儿10例,男7例,女3例,年龄1～12岁.肛门闭锁直肠尿道球部瘘4例,肛门闭锁直肠尿道前列腺部瘘3例,泄殖腔畸形1例(共同管＜3 cm),肛门闭锁并球形结肠1例,肛门闭锁直肠前庭瘘1例.10例患儿均有排尿困难,其中3例伴有尿失禁.MRI显示2例合并脊髓栓系.排泄行膀胱尿道造影显示3例合并左侧输尿管Ⅳ°反流及肾积水,其中1例存在后尿道憩室,无1例发现尿道狭窄.尿动力学检查显示9例膀胱容量及残余尿增加,充盈期逼尿肌压正常,无逼尿肌过度活动,尿流率下降,其中8例逼尿肌收缩力下降,1例逼尿肌收缩力正常.另外1例直肠前庭瘘合并脊髓栓系患儿膀胱容量减少、残余尿增多、尿流率下降,充盈期逼尿肌压升高,合并逼尿肌过度活动.直肠尿道瘘合并后尿道憩室患儿行后矢状入路尿道憩室切除,泄殖腔畸形和直肠尿道前列腺部瘘术后合并输尿管反流患儿行左侧输尿管再植,8例合并神经性膀胱的患儿坚持清洁间歇导尿.结果 随访6个月～5年,泄殖腔畸形患儿1年后仍存在左侧输尿管反流及肾积水,直肠尿道球部瘘合并尿道憩室患儿输尿管反流及肾积水消失,无排尿困难及残余尿,直肠尿道前列腺部瘘合并左侧输尿管Ⅳ°反流及肾积水患儿输尿管反流消失,仍需间歇导尿,其余7例患儿无1例出现上尿路损害.结论 肛门直肠畸形合并脊髓发育不良及手术损伤可导致神经性膀胱.术中直肠尿道瘘处理不当可能导致尿道憩室或尿道狭窄.清洁间歇导尿是神经性膀胱的首要治疗方法,对于后尿道憩室可行尿道憩室切除术.     

Resilience in Families with a Child with Cancer

The aim of this study was to identify and explore resilience factors associated with family adaption after a child had been diagnosed with cancer. Using a cross-sectional survey research design, parents (n = 26), and children (n = 25) from the same families independently completed six self-report questionnaires, as well as responded to an open-ended question about those qualities that helped their family through the period following the diagnosis. The most significant results came from the children's data. According to these results, connectedness within the family, the experience of control over life events, family routines, positive, and supportive communication, redefinition of crisis situations, and lastly, a passive appraisal of crisis situations, were positively linked to better family adaptation. The identified factors should be strengthened and developed in families finding themselves in a similar situation.     

Colonization with Clostridium difficile in Children with Cancer

Objective

Clostridium difficile is a gram-positive, anaerobic, spore-forming bacillus. Usually it does not cause disease unless a patient who is colonized with toxin-producing strains has been treated with antibiotics, particularly those that change the anaerobic flora of the large intestine.

Methods

We investigated in a prospective study intestinal colonization of C. difficile and its toxins in children with malignancy that used different antibiotics and cytotoxic drugs.

Findings

One hundred fifty-two patients were included in this prospective study. Stool samples were obtained within the first 48 hours after admission and cultured for C. difficile; cytopathic effect of C. difficile was detected on HELA cells, also ELISA test was performed for detection of toxins A and B. 25% of patients had positive culture for C. difficile; 36/38 (92%) revealed positive cytopathic effect on HELA cells. No significant relation was found between age, gender, history of antibiotic consumption and C. difficile positive culture and cytopathic effect on HELA cells. The only relation was seen between cotrimoxazol usage and cytopathic effect on HELA cells (P=0.03).

Conclusion

Although the rate of C. difficile colonization (25.6%) and toxigenic strains (23.7%) in admitted children in hematologic ward is high, the rate of ELISA positive test for toxin A+B was not correspond with culture and cytopatic effect on HELA cell. With respect to sensitivity and specificity of ELISA test, possibility for existence of toxin C with cytopathic effect is high in this type of patients.     

Treatment with Probucol of Children with Familial Hypercholesterolemia

ABSTRACT. Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2±1.45 mmol/l to 7.17±0.84 mmol/l (12.6%). This level was further reduced to 5.92±0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.     

Therapeutic experience with hepatoblastoma associated with trisomy 18

Trisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease‐free and the fourth is alive with remaining of the tumor. These cases suggest that combined chemotherapy and surgical resection may be an option to treat hepatoblastoma associated with trisomy 18 when cardiac pulmonary function is relatively stable.     

Juvenile rheumatoid arthritis with myelofibrosis with myeloid metaplasia

Myelofibrosis with myeloid metaplasia is defined as a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extramedullary hematopoesis and varying degree of myelofibrosis. It may be idiopathic or secondary to a large number of conditions. Here is a rare case of myelofibrosis occurring in a patient with juvenile rheumatoid arthritis.     

Tyrosinemia associated with perinatal infection with cytomegalovirus.

A premature infant presented with elevated concentrations of tyrosine in blood and urine, evidence of hepatocellular damage, demineralization of the bones, and a renal Fanconi syndrome. This is the clinical picture found in hereditary tyrosinemia. The infant also had a perinatal infection with cytomegalovirus.