儿童Caroli's病8例

目的 探讨儿童Caroli's病的临床特点及诊断方法 ,以提高对该病的认识.方法 回顾性分析本院1998年2月-2007年12月收治的8例Caroli's病患儿临床资料、实验室检查及影像学表现,8例均行腹部B超及cT检查.结果 Caroli's患儿男5例,女3例;平均年龄6.3岁.病史5 d～4个月.临床表现分别为肝脾大5例,呕血伴腹痛3例,发热伴尿色混浊1例.8例中5例属门静脉周围纤维化型,患儿有不同程度的肝硬化,5例肝脾大,其中3例出现门静脉高压,1例有胆囊炎.余3例为单纯型,其中1例并婴儿型多囊肾.实验室检查2例肝功能异常,1例肾功能异常.影像学检查示肝大,肝实质内散在多发不规则无回声区,与胆管相连.CT检查可见"中心点"征,为特异性征象.1例可见食管胃底静脉明显扩张.本组8例均采用保守治疗,无1例手术治疗.结论 Caroli's病临床表现多样,对于腹痛及肝大的患儿尤应注意.B超及CT检查对诊断Caroli's病具有重要价值,有助于早期明确诊断.可采取保守或手术治疗.     

不同基因突变致肾单位肾痨及相关综合征患儿临床表型特点及基因分析

目的 提高对肾单位肾痨（nephronophthisis,NPHP）及相关综合征患儿临床表型和基因特点的认识。方法 回顾性分析2018年1月—2022年11月河北医科大学第二医院儿科诊治的8例NPHP及相关综合征患儿的临床资料，对其临床特征和基因检测结果进行分析。结果 8例NPHP患儿中，男5例，女3例，起病年龄15个月至12岁，就诊时均存在不同程度肾功能异常。8例患儿中，2例以发育落后首发，2例以贫血为首发，2例为体检发现肾功能异常；肾外表现包括心血管异常2例，骨骼发育异常2例，肝功能异常1例，视网膜色素变性1例，内脏镜面转位1例。8例患儿肾脏B超均有结构改变，4例尿常规显示存在轻至中度蛋白尿。5例为NPHP1基因突变，NPHP3、IFT140、TTC21B基因突变各1例，共发现4个新突变位点。结论 NPHP及相关综合征患儿常以发育落后或贫血为首发表现，部分患儿合并肾外表现。对不明原因肾功能异常患儿，应考虑到NPHP及相关综合征，高通量测序技术有助于明确诊断。[中国当代儿科杂志，2023,25(8):831-836]     

小儿单纯性肾囊肿临床分析

目的通过分析小儿单纯性肾囊肿的发病规律、诊断和治疗方法,提高对该病的认识及诊疗水平。方法回顾性分析6例单纯性肾囊肿患儿的临床资料。男5例,女1例,平均年龄7.8岁（13个月至13岁）。均因腹痛、无痛性肉眼血尿、腹部包块而就诊。均经B超、CT检查明确诊断。结果2例囊肿直径〈4cm,予保守治疗。4例囊肿直径〉4cm,有明显临床症状,予经后腹膜腹腔镜肾囊肿去顶减压术,术后均放置负压引流管,其中3例于术后2～4d拔除,1例因肾周漏尿放置46d。6例均康复出院。4例手术患儿随访2个月至5年无异常,B超检查无复发。2例非手术患儿中,1例随访7年,无腹痛、血尿等不适,B超检查提示囊肿较发现时缩小约60%,另1例B超随访6年,囊肿无明显变化。结论小儿单纯性肾囊肿少见,以男性多见,具有囊肿大小与年龄无关、囊肿无增长趋势等特点,B超、CT等影像学检查可明确诊断,经后腹膜腹腔镜下肾囊肿去顶术是安全、有效的治疗方法。     

儿童尿路结石及其致急性肾功能衰竭临床分析

目的 探讨儿童尿路结石的高危因素、临床特点及其导致急性肾功能衰竭的诊断及处理方法 .方法 回顾分析2002至2007年我院收治的35例尿路结石病例,分析其临床表现、治疗及转归,以总结出儿童尿路结石的临床特点及治疗方法 .结果 所有患儿均采用腹部B超及腹平片检查确诊;24例患儿并发急性肾功能衰竭,病程与血尿素氮、肌酐水平正相关(r=0.549,P=0.007:r=0.575,P=0.004);肾功能衰竭组X线阳性结石少于非肾功能衰竭组(X2=8.70,P=0.006);尿酸水平高于非肾功能衰竭组(t=3.04,P=0.009);肾功能衰竭组予内科保守或血液透析治疗,同时行膀胱镜逆行引流术,解除梗阻后患儿尿量增加,尿毒症症状消失,肾功能恢复正常.结论 小儿尿路结石临床表现不典型,腹部B超是最好的诊断方法 ;结石导致急性肾功能衰竭需早期诊断及治疗,及时解除结石梗阻至关重要,同时应注意纠正水电解质紊乱及对症治疗;应长期随访,预防结石复发.     

婴幼儿下尿路梗阻13例临床分析

目的 探讨婴幼儿下尿路梗阻的临床特点及早期诊断治疗。方法 回顾性研究13例患儿的临床资料、B超、VCU等，对其诊断治疗进行分析。结果 婴幼儿下尿路梗阻临床特点为症状不典型，体征隐匿，合并尿潴留及泌尿系感染，常有肾功能受损。结论 早期尿流改道是有效的处理措施，是改善患儿肾功能提高存活率的关键。     

3108例食用三聚氰胺污染奶粉儿童筛查结果分析

目的 探讨佛山地区食用三聚氰胺污染奶粉小儿泌尿系结石的分布情况.方法 对因食用三聚氰胺污染奶粉就诊的小儿进行尿常规及B超检查,经临床及B超确诊为泌尿系结石的患儿进一步检查肝、肾功能及腹部平片,并进行临床埘比分析.结果 3 108名就诊小儿中,B超结果提示肾脏强光团51例(1.64%),符合国家卫生部颁发的.肾结石诊断标准8例.B超检查结果异常以食用三鹿奶粉儿为主(P<0.05),且泌尿系B超有无强光团与患儿年龄相关(X2=12.43,P<0.05).结论 食用三聚氰胺污染奶粉引起泌尿系异常与三聚氰胺的含量相关,与年龄相关.     

甲基丙二酸血症26例临床分析并文献复习

目的 探讨甲基丙二酸血症的临床特征和治疗方法.方法 对26例甲基丙二酸血症患儿的临床表现、辅助检查及诊疗情况进行分析.结果 26例患儿中主要临床表现:24例神经系统受累,9例肾脏受累,8例血液系统受累,2例肝脏受累,3例心脏受累,1例肺部受累.气相色谱-质谱法尿有机酸分析显示26例患儿尿甲基丙二酸浓度均明显高于正常.12例失代偿性代谢性酸中毒,8例血同型半胱氨酸升高.头颅CT检查6例中5例异常,MRI检查10例全部异常.19例确诊患儿接受维生素B12治疗,辅以低蛋白饮食及左旋肉碱治疗.对治疗的15例进行平均8.5个月的随访.2例死于严重代谢性酸中毒,12例神经系统症状明显好转,8例以肾脏受累起病的患儿出院后多次复查尿常规、肾功能均正常.结论 甲基 丙二酸血症临床特征差异很大,及早进行气相色谱-质谱法尿有机酸分析明确诊断至关重要,确诊后坚持长期合理治疗是改善预后的有效方法.     

儿童气管性支气管20例临床分析

目的分析经支气管镜检查诊断的气管性支气管患儿的临床特征。方法回顾分析20例接受支气管镜检查而确诊的气管性支气管患儿的镜下结果、临床表现、治疗与转归。结果先天性气管性支气管患儿20例,右侧气管性支气管19例,移位型18例,其中6例为2种或2种以上气道异常;左侧气管性支气管1例,为移位型。20例患儿的临床症状包括持续或反复的喘息,持续性局限性肺炎、肺不张,局限性肺气肿。大部分患儿经临床对症治疗后症状缓解。结论气管性支气管异常在临床上并不少见,其临床表现为治疗反应不佳的反复喘息、肺炎、肺不张以及肺气肿等,对可疑患儿应及时进行支气管镜检查以明确诊断。     

Clinical of tracheal bronchus in 20 children

目的 分析经支气管镜检查诊断的气管性支气管患儿的临床特征.方法 回顾分析20例接受支气管镜检查而确诊的气管性支气管患儿的镜下结果、临床表现、治疗与转归.结果 先天性气管性支气管患儿20例,右侧气管性支气管19例,移位型18例,其中6例为2种或2种以上气道异常;左侧气管性支气管1例,为移位型.20例患儿的临床症状包括持续或反复的喘息,持续性局限性肺炎、肺不张,局限性肺气肿.大部分患儿经临床对症治疗后症状缓解.结论 气管性支气管异常在临床上并不少见,其临床表现为治疗反应不佳的反复喘息、肺炎、肺不张以及肺气肿等,对可疑患儿应及时进行支气管镜检查以明确诊断.     

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99mTc-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99mTc-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99mTc-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）。99mTc-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　99mTc-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。     

Analysis of renal biopsies performed in children with abnormal findings in urinary mass screening

Background: School urinary mass screening tests are performed to make early diagnosis and provide proper treatment for chronic renal diseases. However, very few systemic analyses or studies have been reported regarding final diagnosis made on children with abnormal urinary screening results. Aim: To study the cases of renal biopsy in children detected in urinary screening. Methods: We retrospectively analysed 461 cases of renal biopsy performed on children referred to us with abnormal school urinary mass screening results who satisfied indications for renal biopsy. Results: Pathologically abnormal findings were observed in 285 (61.8%) patients. Thin glomerular basement membrane disease was detected in 127 (27.5%) cases and IgA nephropathy in 121 (26.2%) cases. Among those 461 children, microscopic haematuria was observed in 289 (62.7%) patients, proteinuria in nine (2.0%), and both in 163 (35.4%). In addition, a statistically higher rate of pathological abnormalities on renal biopsy was noted in the group with microscopic haematuria combined with proteinuria and also in cases with more severe haematuria.

Conclusion: School urinary mass screening has greatly contributed to diagnosing chronic renal diseases. Continuous medical observation is required when abnormal urinalysis is observed, and a more aggressive medical approach such as renal biopsy should also be performed if necessary.     

Survival of childhood polycystic kidney disease following renal transplantation: the impact of advanced hepatobiliary disease

Childhood PKD encompasses the diagnoses of AR and ADPKD, glomerulocystic disease, and syndromes such as tuberous sclerosis or Jeune's syndrome. Given the fact that a majority of PKD children with ESRD carry the diagnosis of ARPKD, natural history studies assessing the long-term prognosis of PKD patients following renal transplantation must focus on morbidity and mortality issues related to complications from congenital hepatic fibrosis. Using the NAPRTCS registry, we analyzed the patient and graft survival rates of 203 PKD patients and 7044 non-PKD patients undergoing renal transplantation between 1987 and 2001. Deceased PKD patients, all with a diagnosis of ARPKD, were further identified and characterized using a special questionnaire submitted to the principal investigators. Overall graft and patient survival rates were not significantly different between PKD and non-PKD patients. No differences in rates of acute rejection or time to first rejection were noted between PKD and non-PKD patients. The relative risk of living longer than 3 yr in the PKD patients was not significantly different from non-PKD patients (RR = 0.70, p = 0.28). Sepsis was identified as a likely factor in the cause of death in nine (64%) ARPKD patients and was comfirmed with a positive blood culture in four patients. Despite similar graft and patient survival rates among PKD and non-PKD children following renal transplantation, our results suggest that ARPKD transplant recipients appear to be at increased risk for sepsis that may be related to hepatic fibrosis and ascending cholangitis. The utility of early liver transplantation in ARPKD patients with significant hepatobiliary disease is discussed.     

小儿致密物沉积病的临床与病理分析

Objective To analysis the clinical and pathological characteristics of children with dense deposit disease (DDD). Methods 12 Children diagnosed as DDD by electron microscope were enrolled in this study. The clinical and pathological data were analyzed. Results Of the 12 cases, 7 were males and 5 females, mean age 9.1±3.9(5-13) years at onset, the duration from onset to renal biopsy was 1 month to 5 years and the follow-up period was 1-9 years. All cases had heavy proteinuria >50 mg/(kg·d), and persistent microscopic hematuria with recurrent gross hematuria during the course. Seven cases had hypertension (≥140/100 mm Hg, 1 mm Hg=0.133 kPa), 5 cases had transient or recurrent abnormal renal function, and mild to severe anemia were observed in 8 cases respectively. All the cases had lower serum C3(0.15-0.55 g/L). Clinically, 10 cases were diagnosed as nephritic syndrome (one case had partial lipodystrophy at the sa me time), and 2 cases were diagnosed as acute nephritic syndrome. Immunofluorescence study showed intense deposition of C3 along GBM, TBM and the wall of Bowman's capsule in a ribbon-like pattern and in the mesangial regions as coarse granules in all the cases. Under light microscopy, 9 cases showed the feature of membrane proliferative glomerulonephritis (MPGN), 1 case with focal segmental glomeruloselerosis (FSGS), 1 case with endocapillary proliferative glomerulonephritis (EnPGN) and 1 case with proliferative sclerosis (PSGN). Crescents were seen in 3 cases. Under electron microscopy, ribbon-like or linear electron-dense intramembranous deposits were identified in the lamina dense of GBM, and often along TBM and the wall of Bowman's capsule. All patients showed steroid resistance. After methylprednisone treatment, some patients showed transient remission. During the followup stage of 1-9 years, 3 cases showed normal urinalysis, 5 cases showed partial remission, 2 cases progressed to end stage renal disease (ESRD) and 2 cases were lost. Conclusion DDD is an in dependentiy rare disease with pathological-clinical rarities. Children with DDD presented with persistently lower C3, heavy proteinuria, recurrent gross hematuria and anemia. The characteristic immunopathologic finding is intense deposition of C3 along the GBM. Under electron microscopy, ribbon-like or linear electron-dense deposits in the lamina dense of the GBM, TBM and the wall of Bowman's capsule. Electron microscopic examination to demonstrate the intramembranous dense deposits is definitive diagnosis, regardless of the finding of light microscopy. All of them showed steroid resistant. Patients with steroid and CTX treatment showed some clinical improvement of their urinalysis.     

Outcome of Multi-Cystic Dysplastic Kidneys in Children

Background:

Renal cystic diseases are important causes of chronic kidney disease (CKD).

Objectives:

We report the pattern of renal cystic disease in children and evaluate the outcome of children with multicystic dysplastic kidney (MCDK).

Patients and Methods:

Retrospective study of all children with cystic kidney diseases at King Abdulaziz University hospital from 2006 to 2014.

Results:

Total of 55 children (30 males); 25 MCDK, 22 polycystic kidney diseases (PKD), 4 nephronophthises and 4 renal cysts. Consanguinity was positive in 96.2%. MCDK and simple renal cyst patients had good renal function while PKD and nephronophthisis developed renal impairment. Most MCKD were diagnosed ante-natally, 16 of them were followed up for 3.4 (1.97) year. Their last creatinine was 33.9 (13.5) umol/L. MCDK was spontaneously involuted at mean age of 2.6 (1.3) years in 56%.

Conclusions:

MCDK is the commonest cystic renal disease and diagnosed ante-natally in the majority of cases. It has a good prognosis.     

急性脑部炎症合并视觉障碍患儿22例临床特征及预后

目的 分析急性脑部炎症合并视觉障碍患儿临床特征及预后。方法 对2003年1月至2014年6月中国医科大学附属盛京医院小儿神经内科22例临床诊断为急性脑部炎症合并视觉障碍患儿的临床症状、实验室检查、视觉诱发电位、神经影像学特点等临床资料进行回顾性分析。结果 发病＜1周就诊入院的10例、1～2周8例、＞2～4周2例、＞4周2例。以视觉障碍首发就诊的7例。确诊为病毒性脑炎合并视神经炎9例,病毒性脑炎合并视神经乳头炎1例,病毒性脑炎及化脓性脑膜炎合并视觉障碍性质待定各1例,播散性脑脊髓炎合并视神经炎的2例,脑干脑炎合并暴露性眼炎的1例,病毒性脑炎合并右眼晶体点状混浊、双眼视网膜发育不良的1例,视神经脊髓炎1例,病毒性脑炎合并皮质盲1例,播散性脑脊髓炎合并视乳头炎、疑似小柳原田综合征1例,多发性硬化症可能性大3例。全部患儿均进行了视觉诱发电位检查,其中视觉传导通路异常18例。10例行眼底照相,结果异常7例。均行头磁共振（MR）平扫检查,结果异常10例,其中6例脑内病变范围广泛。18例行丙种球蛋白联合激素冲击治疗,其中出院时14例视觉障碍症状好转。出院后随访6个月至8年,20例预后较好、2例预后较差。结论 急性脑部炎症合并视觉障碍病因多样,除考虑常见病,如视神经炎、视神经乳头炎、视神经脊髓炎、脑血管病外,还应考虑到少见疾病,如皮质盲、小柳原田综合征等。早期行视觉诱发电位、眼底照相检查对于疾病诊断与鉴别有意义,积极治疗对改善预后有重要作用。     

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??Abstracts?? Objective To analyze the clinical characteristics and prognosis of acute encephalitis with visual impairment in children. Methods The clinical features?? including clinical manifestations??laboratory tests??Flash visual evoked potential and cranial MRI?? in 22 patients diagnosed with acute encephalitis with visual impairment in children were retrospectively analyzed. Results The disease course was less than one week in 10 cases. The disease course of eight cases was between one week and two weeks?? two cases were between two weeks and four weeks?? two cases were over four weeks. Seven cases had visual impairment as the first symptom. One case was with the diagnosis of viral encephalitis with cortical blindness. Nine cases were with the diagnosis of viral encephalitis with optic neuritis. One case was with the diagnosis of encephalitis with neuropapillitis. One case was with the diagnosis of disseminated encephalomyelitis with optic neuritis. Two cases were with the diagnosis of disseminated encephalomyelitis with optic neuritis. One case was with the diagnosis of brainstem encephalitis with exposure keratitis. One case was with the diagnosis of encephalitis with lens opacity and retinal dysplasia. One case was with the diagnosis of ophthalmoneuromyelitis. One case was likely with the diagnosis of Vogt-Koyanagi-Harada syndrome. In all the cases of Flash visual evoked potential??eighteen were abnormal. In the ten cases of fundus photography??seven were abnormal. In all the cases of cranial MRI??ten were abnormal and six suffered from extensive pathological changes in the brain. In the eighteen cases of hormone therapy??fourteen had good effects. The time of post hospitalization follow-up ranged from 6 months to 8 years. The prognosis of 20 patients was good. Two patients’ prognosis was bad. Conclusions There are different kinds of etiology and diseases in children who are diagnosed with acute encephalitis with visual impairment. Flash visual evoked potential and fundus photography are of important significance in the early stages. High doses of IVIG corticosteroids and hyperbaric oxygen therapy play a key role in improving the prognosis of disease.     

法布里病患儿临床特点及酶替代治疗四例初探

目的分析法布里病患儿的临床特点及使用酶替代药物治疗基本情况。方法对2014年1月至2020年7月间浙江大学医学院附属儿童医院确诊的4例法布里病患儿的临床资料、实验室检查、基因变异及治疗进行回顾性分析。临床观察其酶替代药物阿加糖酶β治疗的效果。结果 4例患儿(男2例、女2例)年龄12.4(6.0~16.8)岁,临床表现各异,其中肢端疼痛1例、少汗2例、尿崩1例,均有左心室肥厚和尿检异常,但均未发现典型皮疹及听力异常。4例患儿均结合临床症状、体征、家族史,通过α-半乳糖苷酶A酶活性、基因检测结果明确诊断。共检出3个GLA基因错义变异 c.424T>C(p.C142R)、c.335G>A(p.R112H)和c.644A>G(p.N215S)。其中前2个变异为经典型法布里病患者变异位点,后者多表现为迟发型但亦有经典型的报道。例1使用阿加糖酶β用量为每次1 mg/kg静脉泵注,每2周用药1次。患儿诉用药后疼痛强度有缓解,少汗症状得到改善。患儿在最初的2个月输注阿加糖酶β过程中未发生严重不良反应,在输注阿加糖酶β 3次后24 h尿蛋白升至1 015.6 mg,未予处理,1周后复查降至正常。结论法布里病在儿童期临床表现多样,需要多学科联合协同诊断并探讨酶替代治疗的时机,阿加糖酶β治疗患儿短期严重不良反应少见。     

Screening System for Asymptomatic Renal Disease in Children in Japan

The introduction in Japan of routine urinalysis for pre-school and school age children has greatly facilitated the discovery of renal disease in asymptomatic children. Over the past 8 years at Kitasato University Hospital, we have studied 113 cases of chronic progressive renal disease based on renal biopsy.
Thirty-one of these 113 patients were found by routine urinalysis at area schools. It is noteworthy that many of the asymptomatic children were found to have renal diseases such as MPGN, FGS, and IgA nephropathy.Although it is difficult to decide whether or not medical management is required for asymptomatic children, the histological findings may give pertinent guidance for planning an appropriate therapeutic program in the early stages of various types of renal disease. This early detection may enable us to observe these potentially progressive renal diseases from the early phase and to provide clues for the investigation of the pathogenesis of these renal diseases.     

儿童肾血管性高血压29例诊断方法的回顾性分析

目的 探讨儿童肾血管性高血压（RVH）的早期诊断方法。 方法 回顾性分析北京大学第一医院1996年1月至2011年6月确诊的RVH患儿,分析贻误诊治情况,以及临床症状、影像学检查结果对RVH的诊断价值。 结果 ①29例确诊RVH患儿进入分析,其中男18例,女11例。起病年龄0.5~15岁,平均年龄（7.0±4.4）岁。从首发症状出现至确诊时间为15 d至7年,平均确诊时间11个月。9例（31.0%）患儿起病6个月后确诊,曾分别被误诊为胃肠炎、癫、肾脏和心脏疾病等。3例患儿以头痛伴呕吐起病,补液出现抽搐后发现高血压。首发症状以头痛（10例,34.5%）、呕吐（10例,34.5%）和抽搐（9例,31.0%）多见。起病时平均收缩压和舒张压分别为182和127 mmHg。②单侧肾动脉狭窄25例,双侧肾动脉狭窄4例。23例通过肾动脉造影确诊,其中17例单侧肾动脉狭窄患儿中10例超声检查显示双肾长径相差>1.5 cm;血管超声对肾动脉狭窄检出率为31.3%（5/16例）;CT对肾动脉狭窄检出率为50.0%（3/6例）;肾动态显像对肾功能受损检出率为93.8%（15/16例）。通过肾血管超声检查确诊4例,通过肾动脉超声检查及CT检查确诊1例,通过肾动脉超声检查和肾动脉MRA检查确诊1例。③16/18例（88.9％）卧位血浆肾素和血管紧张素水平增高。12/29例（41.4%）存在低钾血症。10/29例（34.5%）确诊为多发性大动脉炎。 结论 儿童RVH贻误诊断率较高,对于有头痛、呕吐和抽搐者应首先测量血压,尤其在补液治疗前。肾动态显像有助于早期发现患侧肾功能受损,血浆肾素和血管紧张素增高、低钾血症、双肾大小不对称对于诊断具有提示意义,肾血管超声和CT检查敏感度有限,可疑患儿应尽早行肾动脉造影检查。确诊RVH后应尽可能进行病因诊断,尤其应确定是否存在大动脉炎以指导治疗。     

Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review.

BACKGROUND: The duration of follow up to assess the risk of long term renal impairment in Henoch-Sch?nlein purpura (HSP) without nephritic or nephrotic syndrome or renal failure on diagnosis remains undetermined. AIMS: To undertake a systematic review of the literature to assess whether the risk of long term renal impairment without renal involvement on diagnosis could be estimated and to determine the time period when renal involvement is very unlikely after the diagnosis of HSP. METHODS: Search of studies of unselected children with HSP, and available information on urinary findings, renal involvement, and long term renal function follow up. Studies of selected children with HSP nephropathy at diagnosis were excluded. RESULTS: Twelve studies of 1133 children were reviewed. The follow up period ranged from 6 weeks to 36 years. Proteinuria and/or haematuria, which occurred in 34.2%, of which only one fifth were in association with nephritic or nephrotic syndrome, developed in 85% of cases within 4 weeks of the diagnosis of HSP, in 91% within 6 weeks, and in 97% within 6 months. Permanent renal impairment never developed after normal urinalysis; it occurred in 1.6% of those with isolated urinary abnormalities, and in 19.5% of those who developed nephritic or nephrotic syndrome. CONCLUSION: No long term renal impairment occurred after normal urinalysis. Even if urinalysis is normal at presentation, the testing should be continued for six months. There is no need to follow up after the first six months those whose urinalysis remains normal.