中华小儿外科杂志最新文献

筛选
英文 中文
Management strategies of type-I esophageal-atresia: a report of 10 cases i型食管闭锁的治疗策略(附10例报告
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.008
Wei Fan, Jinshi Huang, Kuai Chen, J. Tao, Zhiwen Liu, Meihan Xu, Xiang-hui Zeng, Gan Liang, Yang Zhi
{"title":"Management strategies of type-I esophageal-atresia: a report of 10 cases","authors":"Wei Fan, Jinshi Huang, Kuai Chen, J. Tao, Zhiwen Liu, Meihan Xu, Xiang-hui Zeng, Gan Liang, Yang Zhi","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.008","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.008","url":null,"abstract":"Objective \u0000To explore the management strategies of type Ⅰ esophageal atresia (EA) through natural growth and tension-extension. \u0000 \u0000 \u0000Methods \u0000From January 2012 to January 2017, a total of 10 children of type-Ⅰ EA were recruited.There were 5 boys and 5 girls.Difficult postnatal insertion of gastric tube prompted a clinical diagnosis of type-Ⅰ EA after esophagography.Within a month after birth, laparoscopic gastrostomy was performed.During operation, the distance between proximal and distal esophagus was measured on esophageal radiography.And nutrient liquid was supplied via gastrostomy.Saliva was reabsorbed through proximal esophagus and esophageal growth measured regularly.Esophageal extension was achieved through natural growth and tension-extension.Thus thoracoscopic gastroesophagostomy was delayed. \u0000 \u0000 \u0000Results \u0000Two cases of thoracoscopic esophageal anastomosis were achieved through natural growth.The completion ages were 153 and 151 days respectively.In another eight cases, the distance between proximal and distal esophagus was still greater than the total length of four vertebrates at 12 weeks post-birth.Tension-extension was applied for promoting the growth of esophagus and thoracoscopic gastroesophagostomy performed .And esophagectasia was performed postoperatively for counteracting esophageal stenosis.When starting endo-esophogeal lengthening, the mean age was (174.3±86.6) (92-280) days and the mean vertebral number between esophogeal ends (5.1±0.5)(4.5-6.0). And the extended time was (49.0±16.2) (28-69) days.At Day 7 postoperatively, esophogeal contrasting radiography revealed esophogeal anastomotic fistula (n=2). Both cases were cured after conservative measures.Esophogeal stenosis of varying degrees (n=10) required esophogeal dilatation.During follow-ups, all children had excellent growth and development. \u0000 \u0000 \u0000Conclusions \u0000Promoting esophageal extension through natural growth and tension-extension reduces pre-anastomotic surgical trauma and postoperative adhesion.Thoracoscope allows a distinct exsanguine operative field for minimizing surgical trauma.The recovery is satisfactory after treatment.This promising treatment is worth a wider popularization. \u0000 \u0000 \u0000Key words: \u0000Esophageal atresia; Thoracoscopes; Esophageal extension","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"10 1","pages":"993-997"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74496171","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Two-stage repairing of hypospadias cripples with residual severe chordee by reconstructing urethral plates 尿道板重建两期修复尿道下裂伴严重脊索残障
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.003
Y. Qu, Wei-ping Zhang, N. Sun, J. Tian, Minglei Ling
{"title":"Two-stage repairing of hypospadias cripples with residual severe chordee by reconstructing urethral plates","authors":"Y. Qu, Wei-ping Zhang, N. Sun, J. Tian, Minglei Ling","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.003","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.003","url":null,"abstract":"Objective \u0000Managing properly hypospadias cripples has been a great challenge for pediatric urologists.We retrospective reviewed our experience of repairing hypospadias cripples with residual severe chordee by reconstructing urethral plates in two stages. \u0000 \u0000 \u0000Methods \u0000From January 2017 to June 2019, retrospective analysis was performed for the clinical data of 3094 surgical cases of hypospadias.Among 1967 initial cases, 1127 were re-operated.And 41/71 cases of chordee penis were corrected by degloving and another 30 children required resecting urethra for straightening penis and repairing in two stages. \u0000 \u0000 \u0000Results \u0000The initial 30 children had an average age of 27.5 months.During reconstruction of urethral plate, the average age was 96.6 months.Previous operations: 1, n=10; 2, n=10; 3, n=3; 4, n=3 cases, 5, n=2; 7, n=2 and previous surgical approaches included Duckett/Duckett & Duplay (n=9), TIP(n=8), staging operation (n=7), free grafting (n=3) and unknown mode (n=3). All 30 cases had severe penile curvature with an average of 52.9 (30-100) degrees.And 17 cases required concurrent folding of dorsal white membrane.The average length of urethral defect was 4.5 cm and the longest defect 8 cm.After urethral transection, urethra plate was reconstructed by local skin and neourethra reconstructed in a second stage.Twenty-one children were reoperated.Urethral fistula (n=3, 14%) was successfully repaired again.Up to now, there was no instance of penile curvature during follow-ups. \u0000 \u0000 \u0000Conclusions \u0000Special attention should be paid to correct penile chordee while managing hypospadias during initial operations.For most cases of hypospadias cripples with severe chordee, urethral transection and neourethral reconstruction may be performed in stages.Prepuce or local penile skin remains the first choice reconstructing urethral plates. \u0000 \u0000 \u0000Key words: \u0000Hypospadias; Penis ventral curvature; Urethroplasty, surgery in stages; Postoperative complications","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"128 1","pages":"967-970"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84200971","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Treatment experience and related factors of urethral diverticulum after primary repair of hypospadias in children 小儿尿道下裂一期修复后尿道憩室的治疗体会及相关因素分析
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.004
Xin Liu
{"title":"Treatment experience and related factors of urethral diverticulum after primary repair of hypospadias in children","authors":"Xin Liu","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.004","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.004","url":null,"abstract":"Objective \u0000To summarize the related factors of urethral diverticulum formation after primary repair of hypospadias in children and to explore the appropriate treatments of urethral diverticulum. \u0000 \u0000 \u0000Methods \u0000The clinical data of urethral diverticulum after hypospadias operation were retrospectively analyzed from December 2012 to March 2019 .The clinical characteristics of diverticulum were summarized.Age, hypospadias grade, surgical approaches, diameter of glans, length of neourethra, secondary coverage and urethral stricture history, potential factors with postoperative urethral diverticulum, were analyzed by logistic regression analysis.P<0.05 was used as a statistical criterion. \u0000 \u0000 \u0000Results \u0000Postoperative urethral diverticulum was found in 17 hypospadias children.Urethral diverticulum disappeared in 4 cases after indwelling urethral catheter.Among them, surgery was performed once (n=5) and multiple times (n=3). Five cases had no dysuria or abnormal urinary tract with no surgery.Statistical analysis showed that the grade of hypospadias, the method of surgical repairment (Duckett’s procedure), the length of neourethra and the history of urethral dilatation were all related factors for urethral diverticulum after hypospadias surgery. \u0000 \u0000 \u0000Conclusions \u0000Grade of hypospadias, approach of surgical repair (Duckett’s procedure), length of neourethra and history of urethral stricture are all related factors affecting urethral diverticulum after hypospadias surgery in children.Urethral diverticulum without symptoms may be observed conservatively in children.Early urethral dilation is prescribed for urethral diverticulum.And diverticulum without distal urethral stricture may be treated simply by urethral diverticuloplasty; diverticulum with distal urethral stricture requires removing stricture and then urethroplasty. \u0000 \u0000 \u0000Key words: \u0000Hypospadias; Urethral diverticula; Postoperative complications","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"1 1","pages":"971-975"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90836776","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Risk factor analysis for urethral stricture after hypospadias repair requiring urethral plate transaction 尿道下裂修补术后尿道狭窄需要尿道板置换的危险因素分析
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.005
W. Ru, Zheming Xu, Yiding Shen, Hengyou Wang, Linfeng Zhu
{"title":"Risk factor analysis for urethral stricture after hypospadias repair requiring urethral plate transaction","authors":"W. Ru, Zheming Xu, Yiding Shen, Hengyou Wang, Linfeng Zhu","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.005","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.005","url":null,"abstract":"Objective \u0000To explore the risk factors of urethral stricture after urethroplasty for hypospadias and to seek a more appropriate surgical strategy. \u0000 \u0000 \u0000Methods \u0000Clinical data were retrospectively analyzed for 429 cases undergoing primary hypospadias repair with urethral plate transection from February 2008 to October 2017.The mean age was 2.9 (0.3-17.5) years.The meatal location was penile-scrotal (n=349, 81.4%) and perineal (n=80, 18.6%). The operations were were performed by experienced (n=340, 79.3%) and novice surgeons (n=89, 20.7%). The surgical approaches included Duckett’s procedure (n=269, 62.7%), staged flap urethroplasty (n=98, 22.8%) and staged buccal mucosa tubularized tunnel urethroplasty (n=62, 14.5%). \u0000 \u0000 \u0000Results \u0000During a median follow-up period of 64 (14-125) months, there were urethral complications (n=173, 40.3%) and urethral stricture (n=58, 13.5%). Age, hypospadias type and surgical strategy varied widely among surgeons with different seniorities.Among less experienced surgeons, urethral stricture incidence rate was 23.1% during Duckett’s procedure and 5.6% during staged flap urethroplasty.As compared with Duckett’s procedure, staged flap urethroplasty lowered the risk of urethral stricture (OR 0.185, 95% CI 0.038-0.902, P<0.05). Among experienced surgeons, the incidence rate of urethral stricture was 10.1% during Duckett’s procedure, 8.1% during staged flap urethroplasty and 24.6% during staged buccal mucosa tubularized tunnel urethroplasty.As compared with Duckett’s procedure, staged buccal mucosa tubularized tunnel urethroplasty increased the risk of urethral stricture (OR 2.726, 95% CI 1.185-6.268, P<0.05) and staged flap urethroplasty offered no obvious advantage.Within each group, age or hypospadias type was not related with the occurrence of urethral stricture. \u0000 \u0000 \u0000Conclusions \u0000For severe hypospadias with urethral plate transaction, surgical strategy should be based upon surgeon seniorities.Suitable for novice surgeons, staged flap urethroplasty offers no advantage for experienced operators. \u0000 \u0000 \u0000Key words: \u0000Hypospadias; Urethral stricture; Postoperative complications; Urethral plate transection","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"8 1","pages":"976-980"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87497311","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical characteristics, diagnosis and treatment of duodenal duplication 十二指肠重复的临床特点、诊断与治疗
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.014
Kai Wang, C. Peng, W. Pang, Zeng-meng Wang, Dong-yang Wu
{"title":"Clinical characteristics, diagnosis and treatment of duodenal duplication","authors":"Kai Wang, C. Peng, W. Pang, Zeng-meng Wang, Dong-yang Wu","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.014","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.014","url":null,"abstract":"Objective \u0000To explore the clinical characteristics, diagnosis and treatment of duodenal duplication. \u0000 \u0000 \u0000Methods \u0000From May 1, 2007 to May 1, 2018, 4 surgical children were diagnosed pathologically as duodenal duplication.With complete follow-up data, their managements and prognosis were analyzed.There were 2 boys and 2 girls with an age range from 1 month to 9 years.The clinical symptoms included vomiting (n=4), abdominal pain (n=2), abdominal mass (n=2) and ultrasonic finding of abdominal mass (n=1). Ultrasound revealed cystic mass in right upper quadrant (n=4). Cystic wall had the characteristics of intestinal wall and even peristalsis was noticed (n=1). Computed tomography (CT) and upper gastroenterography indicated tissue compressing around cyst.All of them had cystic duodenal duplication.One case was misdiagnosed as pancreatic pseudocyst and underwent external drainage.And subsequent severe abdominal adhesion required cystic mucous stripping.The other 3 patients underwent total cyst removal. \u0000 \u0000 \u0000Results \u0000Pathological examination revealed intestinal mucosa (n=4) and ectopic pancreas (n=2). During follow-ups, 4 children recovered well and there was no occurrence of vomiting, abdominal pain or pancreatitis. \u0000 \u0000 \u0000Conclusions \u0000Duodenal duplication is rare and its clinical manifestations are correlated with size, location, ectopic mucosa and communication with intestinal cavity or pancreatic bile tract.Diagnosis is mainly based upon pathology.Total removal of duplication or mucous stripping has an excellent prognosis.Endoscopic marsupialization should be coupled with mucous stripping for malignant transformation. \u0000 \u0000 \u0000Key words: \u0000Duodenum, abnormalities; Diagnosis; Treatment","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"24 1","pages":"1025-1029"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82473106","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Identification of unfolded protein response in normal and dysplastic acetabular cartilage 正常和发育不良髋臼软骨中未折叠蛋白反应的鉴定
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.016
Xiaoyan Ni, Yifeng Lin, Xinhong Pei
{"title":"Identification of unfolded protein response in normal and dysplastic acetabular cartilage","authors":"Xiaoyan Ni, Yifeng Lin, Xinhong Pei","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.016","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.016","url":null,"abstract":"Objective \u0000To explore the role of unfolded protein response in normal and dysplastic acetabular cartilages. \u0000 \u0000 \u0000Methods \u0000The rat model of dysplastic acetabulum was established by maintaining hips in a swaddling position.Hematoxylin & eosin staining was utilized for observing the morphological characteristics of acetabular cartilage in normal and DDH models and the width of growth plate measured, including hypertrophic zone (HZ) and proliferative zone (PZ). The mRNA expressions of chondrocyte differentiating markers (Sox9, Col2a1 & Acan) and hypertrophic markers (Runx2, Col10a1 & Mmp13) were measured by quantitative polymerase chain reaction (qPCR) at different timepoints.The expressions of unfolded protein response genes of Grp78, P50ATF6, XBP1s and phosphorylated eIF2α were detected by qPCR and immunohistochemistry respectively. \u0000 \u0000 \u0000Results \u0000a) As compared with control, upper side of acetabulum became flatter with the progression of DDH.Cells in growth plate were disturbed with a loss of polarity.The width of hypertrophic zone (HZ) and proliferative zone (PZ) of growth plates became significantly shortened(P<0.05); b) The mRNA expressions of Sox9, Col2a1 and Acan and Runx2, Col10a1 and Mmp13 decreased significantly in a time-dependent manner(P<0.05); c) The mRNA expressions of Grp78 were markedly inhibited in acetabular cartilages(P<0.05). The protein expressions of XBP1s, P50ATF6 and p-eIF2α involved in unfolded protein response were significantly suppressed in growth plates(P<0.05). \u0000 \u0000 \u0000Conclusions \u0000Chondrocyte differentiation and endochondral ossification require the participation of a physiological unfolded protein response whereas unfolded protein response is inhibited in DDH model, interfering with normal chondrocyte maturation and endochondral ossification.Thus it may be somewhat involved in the pathological process of dysplastic acetabular cartilage. \u0000 \u0000 \u0000Key words: \u0000Acetabulum; Cartilage; Unfolded protein response; Developmental dysplasia of the hip; Chondrocyte differentiation","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"12 3","pages":"1036-1043"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72392765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A histomorphological study of corpus spongiosum around urethral plate in hypospadias 尿道下裂尿道板周围海绵体的组织形态学研究
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.015
Xingqi Bao, Yi-Chen Huang, Hua Xie, Yiqing Lyu, Ling Yu, Zheng-jun Xi, Zhi Li, Q. Fu, L. Song, Fang Chen
{"title":"A histomorphological study of corpus spongiosum around urethral plate in hypospadias","authors":"Xingqi Bao, Yi-Chen Huang, Hua Xie, Yiqing Lyu, Ling Yu, Zheng-jun Xi, Zhi Li, Q. Fu, L. Song, Fang Chen","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.015","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.015","url":null,"abstract":"Objective \u0000To explore the histomorphological changes of orpus spongiosum around urethral plate in hypospadias. \u0000 \u0000 \u0000Methods \u0000Forty-six hypospadias boys aged from 9 to 48 months were recruited and divided into distal (n=20) and proximal (n=26) groups according to the location of meatus.The average age was (2.1±1.1) years.Another 6 cases of urethral rupture with an average age of (14±4.9) years were selected as the controls.The morphologies of corpus spongiosum were examined intraoperatively and its histopathological features evaluated by hematoxylin & eosin, Masson and immunohistochemistry with α-SMA, CD34 and TGF-β1.Western blot and quantitative reverse transcription-polymerase chain reaction (qRT-PCR) were also performed for measuring the expression of TGF-β1. \u0000 \u0000 \u0000Results \u0000The width of corpus spongiosum around urethral plate was significantly greater in distal group than proximal group[(0.36±0.12) vs.(0.18±0.10) cm, P<0.05]. Histology and immunohistochemistry of blood vessels indicated an abnormal structure of cavernous sinus in hypospadias group.It was characterized by a wide vascular lumen [(control (7.20±1.12)μm, hypospadias (13.75±8.08), P<0.05], thickening of vascular wall [control (5.40±1.28) μm, hypospadias (14.11±7.59) μm, P<0.05], lowered cavernous sinus density (hypospadias 15.66±1.17, control 7.24±4.19, P<0.05), decreased cavernous trabecula density (hypospadias 9.80±1.92, control 3.68±2.87, P<0.05) and degree of structural abnormity was more obvious in proximal group.Immunohistochemistry of TGF-β1 showed a lower expression of TGF-β1 in hypospadias (P<0.05). Western bolt and qRT-PCR also confirmed the lowered expression of TGF-β1 in proximal hypospadias as compared with control group(all P<0.05). \u0000 \u0000 \u0000Conclusions \u0000The histomorphology of corpus spongiosum is abnormal around urethral plate in hypospadias.And degree of variation and severity of hypospadias are positively correlated. \u0000 \u0000 \u0000Key words: \u0000Hypospadias; Corpus spongiosum; Histology","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"36 1","pages":"1030-1035"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76819967","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Exploring the susceptible or pathogenic genes of biliary atresia by monozygotic twins’ pedigrees 利用同卵双胞胎家系探讨胆道闭锁的易感或致病基因
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.011
Yu Pu, Pan Yongkang, L. Long
{"title":"Exploring the susceptible or pathogenic genes of biliary atresia by monozygotic twins’ pedigrees","authors":"Yu Pu, Pan Yongkang, L. Long","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.011","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.011","url":null,"abstract":"Objective \u0000Currently the pathogenesis of biliary atresia (BA) has remained unclear.This study was to explore the susceptible or pathogenic genes of BA by monozygotic twins’ pedigrees at whole exome level. \u0000 \u0000 \u0000Methods \u0000Five monozygotic twins’ BA pedigrees were collected.There were 4 pedigrees in discordance for BA and 1 pedigree in accordance for BA.After a definite diagnosis of type Ⅲ BA by intraoperative cholangiography, they underwent Kasai operation.Their clinical data were collected and 4 ml peripheral venous blood samples obtained from all family members of 5 monozygotic twins’ BA pedigrees.DNA was isolated from whole peripheral venous blood.Whole exome sequencing was employed and data quality was controlled strictly.The results of 5 pedigrees were analyzed for detecting genetic mutations. \u0000 \u0000 \u0000Results \u0000Twins in F5 pedigree were in accordance for BA.Two compound heterozygous mutations (SSPO, TTN) were shared by two patients in a recessive mode.And 2/6 patients had the mutation of gene ABCA13 in the mode of incomplete penetrance.Located in a conserved sequence, the mutation played a detrimental role in the structure and function of protein.IFNA10 was correlated with the incidence of BA by family-based association test. \u0000 \u0000 \u0000Conclusions \u0000The strategy of exploring the susceptible or pathogenic genes in BA through monozygotic twins’ BA pedigrees at exome level using multiple models is a feasible approach for elucidating the etiology of BA.The mutations of SSPO, ABCA13 and IFNA10 may be associated with BA type Ⅲ. \u0000 \u0000 \u0000Key words: \u0000Biliary atresia; Susceptible genes; Pathogenic genes; Whole exome sequence","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"43 1","pages":"1008-1014"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84061735","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Risk factors of acute kidney injury in children with biliary atresia after living-donor liver transplantation 活体肝移植术后胆道闭锁患儿急性肾损伤的危险因素分析
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.010
Wu Man, Yu Wenli, Zhang Xinyue, Sheng Mingwei, Z. Nan, Ding Yijie, Du Hongyin
{"title":"Risk factors of acute kidney injury in children with biliary atresia after living-donor liver transplantation","authors":"Wu Man, Yu Wenli, Zhang Xinyue, Sheng Mingwei, Z. Nan, Ding Yijie, Du Hongyin","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.010","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.010","url":null,"abstract":"Objective \u0000To explore the occurrence and risk factors of acute kidney injury (AKI) after liver transplantation in children with biliary atresia (BA). \u0000 \u0000 \u0000Methods \u0000From June 2012 to October 2015, the clinical data were retrospectively reviewed for 198 pediatric living-donor liver transplant recipients with BA at Central Municipal Hospital.AKI was defined and staged according to the guidelines of Kidney Disease Improving Global Outcomes (KDIGO). The clinical data were compared for AKI versus non-AKI children using logistic regression analysis of the relevant factors of postoperative AKI.Chi-square test was performed for comparing the mortality rate during hospitalization between two groups. \u0000 \u0000 \u0000Results \u0000AKI occurred in 83 patients (41.92%) after liver transplantation.The stages of AKI were Ⅰ (n=47, 23.74%), Ⅱ (n=16, 8.08%) and Ⅲ (n=20, 10.10%). Multivariate logistic regression analysis indicated that intraoperative infusion of fresh frozen plasma was a risk factor for postoperative AKI.And preoperative high estimated glomerular filtration rate (eGFR), low serum creatinine (sCr) and high preoperative pediatric end-stage liver disease (PELD) score were independent risk factors for postoperative AKI.The in-hospital mortality rate of AKI group was significantly higher than that of non-AKI group(7.2% vs. 0.8%) and the mortality rate increased gradually with an aggravation of renal injury(P=0.027). \u0000 \u0000 \u0000Conclusions \u0000The incidence of AKI in BA children is 41.93% after liver transplantation.The patients with lower serum creatinine and higher PELD score have a higher risk of AKI.High PELD score may predict the occurrence of postoperative AKI and provide a reference for the risk of postoperative AKI mortality.It plays an important role in reducing the occurrence of AKI, lowering the mortality rate and improving the prognosis. \u0000 \u0000 \u0000Key words: \u0000Liver transplantation; Biliary atresia; Acute kidney injury; Child","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"4 1","pages":"1002-1007"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78325212","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Risk factors of glan dehiscence after tubularized incised plate urethroplasty hypospadias repair 尿道下裂管切开钢板修补术后龟头开裂的危险因素分析
Chinese Journal of Pediatric Surgery Pub Date : 2019-11-15 DOI: 10.3760/CMA.J.ISSN.0253-3006.2019.11.002
Wei Liu, Xiangyu Wu, Xiaoqing Wang
{"title":"Risk factors of glan dehiscence after tubularized incised plate urethroplasty hypospadias repair","authors":"Wei Liu, Xiangyu Wu, Xiaoqing Wang","doi":"10.3760/CMA.J.ISSN.0253-3006.2019.11.002","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2019.11.002","url":null,"abstract":"Objective \u0000To explore the risk factors of glan dehiscence after TIP repairing in hypospadias children. \u0000 \u0000 \u0000Methods \u0000This retrospective study involved 77 consecutive hypospadias children undergoing TIP repair from November 2017 to September 2018.All patients were followed up for 6-15 months.The relevant clinical data collected included age, glan width, urethral plate width pre-incision, neourethra length, distance between meatus and corona, type of catheter and whether dorsal plication or not.Independent t test, chi-square test and logistic regression were performed for evaluating the risk factors of glan dehiscence. \u0000 \u0000 \u0000Results \u0000Glan dehiscence occurred in 10 (13.0%) patients.Our result indicated significant associations existed between group with glan dehiscence and group without glan dehiscence with respects to glan width, distance between meatus and corona and type of catheter (all P<0.05). \u0000 \u0000 \u0000Conclusions \u0000Glan dehiscence is the most common complication after TIP hypospadias repair.Glan width, distance between meatus and corona and type of catheter were obviously correlated with the development of glan dehiscence.A more extensive glans mobilization with >4.5 mm distance between meatus and corona and 6Fr catheter regardless of age helps to minimize the occurrence of glan dehiscence. \u0000 \u0000 \u0000Key words: \u0000Hypospadias; Postoperative complications; Glan dehiscence","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"11 1","pages":"963-966"},"PeriodicalIF":0.0,"publicationDate":"2019-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73530196","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信