摘要目的 分析不同年龄段、不同性别儿童胡桃夹综合征(NCS)的临床特点,为早期识别、诊断NCS提供参考.方法 回顾性分析重庆医科大学附属儿童医院肾内科2008年1月至2018年1月收治的单纯NCS112例患儿的临床资料,并进行追踪随访.结果 NCS患儿中女71例(占63.4％),主要分布于学龄前期(28例,82.4％);男41例(占36.6％),主要分布于学龄期(8例,48.5％)和青春期(7例,42.2％).51.2％ (21/41例)的男性患儿表现为活动后血尿合并蛋白尿,60.6％(43/59例)的女性患儿表现为活动后孤立性血尿.46.7％(21/45例)的青春期患儿最易出现活动后腰腹疼痛,且男童发生率(12例,63.2％)高于女童(9例,34.6％),差异有统计学意义(x2=6.939,P＜0.05).青春期组患儿的24 h尿蛋白定量明显高于2组低年龄患儿,且该组中男性患儿[0.09(0.02～0.21) g/d]明显高于女性患儿[0.06(0.01～0.21) g/d],差异有统计学意义(x2=6.48,P＜0.05).本病CT血管造影(CTA)的检出率(67/70例,95.7％)显著高于彩超(75/91例,82.4％),差异有统计学意义(x2=6.721,P＜0.05).青春期NCS患儿的腹主动脉(AA)与肠系膜上动脉(SMA)交角(AMA)明显小于学龄前期、学龄期患儿,左肾静脉(LRV)扩张段与狭窄段的内径之比(a/b)明显大于学龄前期、学龄期患儿,差异有统计学意义(F =4.797,P＜0.05).不同年龄段、不同性别NCS患儿的乏力症状、12 h尿沉渣红细胞计数、1h尿微量蛋白、血肌酐、血尿素氮水平、LRV水平、SMA与AA的距离(D1)、LRV与SMA从AA起源处的距离(D2)差异均无统计学意义(均P＞0.05).96例患儿随访时间为3个月～7年,88例复查尿常规的患儿中有51例(58.0％)尿检恢复正常,54例复查彩超的患儿中25例(46.3％)提示LRV压迫缓解.结论 NCS女性患儿多于男性,女童多见于学龄前期,多表现为活动后孤立性血尿,男童多见于学龄期和青春期,多表现为活动后血尿合并蛋白尿.青春期患儿最易出现活动后腰腹疼痛,男童腰腹痛发生率高于女童.学龄期及青春期NCS患儿的活动后尿蛋白定量更高,AMA夹角更小,a/b比值更大,LRV受压程度更重,以青春期尤其明显.

abstractsObjective To provide a reference for early detection and diagnosis of nutcracker syndrome(NCS) by analyzing the clinical features of children with NCS in different age groups and different gender groups.Methods Data of 112 children with simple NCS diagnosed at the Department of Nephrology of Chongqing Children's Hospital from January 2008 to January 2018 were analyzed retrospectively.Follow-up was conducted.Results In children with NCS,71 girls accounted for 63.4％,mainly in preschool age(82.4％,28 cases);41 boys accounted for 36.6％,mainly in school age(48.5％,8 cases) and adolescence(42.2％,7 cases).Fifty-one point two percent (21/41 cases) of boys presented with both hematuria and proteinuria after activities,60.6％ (43/59 cases) of girls presented with isolated hematuria after activities,while 46.7％ (21/45 cases) children in adolescence were most likely to appear lumbar and abdominal pain after activities,and the incidence of boys(63.2％,12 cases) was higher than girls (34.6％,9 cases),and the differences above were all statistically significant (x2 =6.939,P ＜ 0.05).The 24-hour urinary protein level in adolescent group was significantly higher than that in 2 groups of younger children,and the male children[0.09 (0.02-0.21) g/d] in this group were significantly higher than that in female children[0.06 (0.01-0.21) g/d] (x2 =6.48,P ＜ 0.05).The detection rate of CT angiography (CTA) (95.7 ％,67/70 cases) was significantly higher than that of color Doppler ultrasound (82.4％,75/91 cases),and the difference was statistically significant (x2 =6.721,P ＜ 0.05).Children with NCS in adolescence had smaller aortomesenteric angles (AMA) and larger ratios of the internal diameter of left renal vein(LRV) 's dilation part (a) to the stenosis part(b) (a/b) than those in preschool age and school age,and the difference was statistically significant (F =4.797,P ＜ 0.05).By follow-up of 96 cases for 3 months-7 years,there were 51 cases(58.0％) whose urine was back to normal in 88 cases who had reexamination of urine,and among the 54 patients who underwent color doppler ultrasound,25 cases(46.3％) showed relief of LRV compression.Conclusions There are more girls with NCS than boys,and girls are mainly in preschool age and always present with isolated hematuria,while boys are mainly in school age and adolescence and always presented with both hematuria and proteinuria.Children in adolescence were most likely to appear lumbar and abdominal pain,and boys have a higher incidence rate than girls.Children in school age and adolescence have the relatively higher quantitation level of proteinuria,and the smaller AMA and the larger a/b ratio,the more serious compression of LRV,and it's especially obvious in adolescence.