中华小儿外科杂志最新文献

{"title":"A case report of esophageal ectopic thymus in a child and literature review","authors":"Shi-kui Chen, S. Xie, Xiangshang Lin, Qian Wang, Xindi Chen","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.007","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.007","url":null,"abstract":"Objective \u0000To report one rare pediatric case of esophageal ectopic thymus (EET) and review the relevant literatures. \u0000 \u0000 \u0000Methods \u0000The clinical manifestations, diagnosis and treatment of one child with EET were reviewed. And systematic searches of the relevant literatures were performed for \" ectopic thymus\" , \" children\" and \" infant\" in the databases of China Knowledge Network, Weipu, Wanfang and PubMed. \u0000 \u0000 \u0000Results \u0000One girl was admitted with vomiting and transient suffocation for 24 hours. Ultrasound and computed tomography (CT) indicated a substantial mass at upper esophagus. During operation, tumor was located in esophageal lumen. Final histopathologic diagnosis was consistent with EET. She was discharged at Day 8 postoperatively. During a follow-up period of 3 months, there was no occurrence of vomiting, dysphagia or other discomforts. And upper gastrointestinal radiography indicated that esophageal mucosal folds remained intact and esophagus was not narrowed or dilated. Fifty-three cases of EET were collected from the literature search. Ectopic thymus occurred in neck (n=34) and thyroid (n=18). Most cases could be detected by ultrasound and their clinical symptoms were not obvious. Sometimes a painless mass was present around neck or thyroid. And the complication of EET was rare. \u0000 \u0000 \u0000Conclusions \u0000Ectopic thymus is extremely rare in esophageal lumen and ultrasonic detection is often not feasible. It is necessary to confirm mediastinal thymus preoperatively. Especially for infants and toddlers, radical thymectomy results in immune dysfunction. Esophageal endoscopy and angiography may aid in pinpointing the location of lesion. \u0000 \u0000 \u0000Key words: \u0000Thymus gland; Dystopia; Esophagus","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"40 1","pages":"219-223"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76625473","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}

{"title":"Comparison of super-mini-percutaneous nephrolithotomy versus mini-percutaneous nephrolithotomy for renal calculi in children","authors":"X. Zhan, Daniel Wang, Jianguo Zhu, Jin-pu Peng, Moudong Wu, Feng-ju Lu, Guoqing He, Nini An","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.012","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.012","url":null,"abstract":"Objective \u0000To compare the safety and efficacy of super-mini-percutaneous nephrolithotomy (SMP) versus mini-percutaneous nephrolithotomy (MPCNL) in managing renal calculi in children. \u0000 \u0000 \u0000Methods \u0000Medical records were retrospectively reviewed for 40 children with renal calculi. They underwent SMP (n=18) and MPCNL (n=22). In SMP group, there were 14 boys and 4 girls with an age range of (7.90±4.40) years and a stone size of (15.41±5.63) mm; In MPCNL group, there were 14 boys and 8 girls with an age range of (8.62±3.41) years and a stone size of (16.77±3.59) mm. Operative duration, primary stone clearance rate, postoperative complications and other parameters of two groups were statistically analyzed for comparing the characteristics of two operative approaches. \u0000 \u0000 \u0000Results \u0000The time of establishing percutaneous renal channel was shorter in SMP group than that in MPCNL group [(10.75±2.70) vs. (19.68±6.95) min]. And inter-group difference was statistically significant (P=0.016). The first-phase stone clearance, postoperative hemoglobin loss and postoperative complication rate of SMP and MPCNL groups were (83.33±46.72) vs. (101.36±38.91) min, 83.33% vs. 81.81% and (7.59±7.51) vs. (7.75±8.77) g/L. respectively. No significant inter-group difference existed. No blood transfusion was provided. In SMP group, there was Clavien Ⅰ complication (n=1, 5.55%); In MPCNL group (n=5, 22.72%), postoperative fever subsided after aggressive anti-infection treatment. In two groups, there was on onset of Clavien Ⅱ/Ⅲ complications. Ultrasonic follow-ups were conducted for 1-2 years and hydronephrosis or renal atrophy disappeared in both groups. \u0000 \u0000 \u0000Conclusions \u0000Both SMP and MPCNL are safe, effective and mini-invasive for pediatric renal calculi. As compared with MPCNL, SMP has a smaller channel and a thinner mirror body. And SMP is more suitable for infants or toddlers with narrow calyx neck and lower calyx calculi. \u0000 \u0000 \u0000Key words: \u0000Renal calculus; Percutaneous nephrolithotomy; Child","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"7 1","pages":"248-251"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81856658","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}

{"title":"Research advances of Gorham-Stout syndrome","authors":"Shuyi Zhao, Tianjing Liu, E. Wang","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.020","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.020","url":null,"abstract":"As an extremely rare disorder of unknown etiology, Gorham-Stout syndrome (GSS) occurs at any age. However, it is frequently found in children and youth. It is characterized by large bone dissolving as a characteristic tumor-like lesion. Most patients often present with multiple osteolysis, swelling, pain, limited mobility and severe pathologic fractures. Due to a low incidence, most reports have focused upon individual cases. Without a definite diagnostic and therapeutic standard, it has limited reference values for clinicians. With a purpose of enhancing its clinical awareness, this review summarized the etiology, pathogenesis, clinical manifestations, imaging findings, pathological features, diagnosis, differential diagnosis, complications, treatments and prognosis of GSS. \u0000 \u0000Key words: \u0000Osteolysis, Essential; Child; Lymphatic vessels","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"96 1","pages":"280-284"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89103817","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}

{"title":"Diagnosis and treatment of biliary hypoplasia: a multicenter survey","authors":"Yuan-Lung Yang, J. Zhan, Ying-chao Li, Hongxia Ren, Baohong Zhao, Weibing Tang, Heying Yang","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.008","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.008","url":null,"abstract":"Objective \u0000To enhance the awareness of biliary hypoplasia (BH) through exploring its diagnosis, treatment and prognosis. \u0000 \u0000 \u0000Methods \u0000For this retrospective study, the clinical data of BH were collected at five domestic hospitals from January 2009 to January 2017. There were 18 boys and 12 girls with an operative age of (83±36) days, a birth weight of (2.75±1.0) kg and a postnatal time of jaundice at (30±6) days. Their birth was term (n=21) and premature (n=9). And the stool was pale yellow stool (n=28) and white (n=2); dark urine (n=19); hepatomegaly & splenomegaly (n=18); mild ascites (n=12). \u0000 \u0000 \u0000Results \u0000All of them were operated. The procedures included cholecystostomy (n=20), cholangiography & biliary flushing (n=8) and non-treatment after cholangiography (n=2). And postoperative medications included antibiotics (n=15), hormone (n=22), hepatic protector & cholagogue (n=30) and gamma globulin (n=9). The follow-up period was 40 to 288 weeks. Jaundice subsided (n=21) and persisted (n=9) within 6 months. And 4/9 had the complications of itchiness (n=3) (1/3 with growth retardation) and anemia (n=1). They were divided into two groups of good prognosis (n=21) and poor prognosis (n=9) according to whether or not jaundice clearing at 6 months post-operation (icteric regression, total bilirubin ≤20 μmol/L), the growth up to standard and presence/absence of complications. Hepatocyte degeneration (n=6 vs. 8) and spot-like necrosis (n=3 vs. 7) were found in good and poor prognosis group. And the difference was statistically significant (P=0.004 & 0.002). \u0000 \u0000 \u0000Conclusions \u0000As a rare disease, BH may be diagnosed by cholangiography and liver biopsy and treated by surgery and postoperative medications. Surgical options include cholecystostomy and flushing. The overall prognosis is excellent. A poor prognosis may be associated with hepatic injury. \u0000 \u0000 \u0000Key words: \u0000Biliary Tract; Dysplasia; Retrospective studies; Multicenter study","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"1 1","pages":"224-229"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89643206","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}

{"title":"Correlation between fetal cardiovascular malformations and chromosome abnormalities","authors":"Xiaolei Xie, W. Tan, Fuguang Li, Suhuan Tang","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.006","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.006","url":null,"abstract":"Objective \u0000To explore the correlation between fetal cardiovascular malformations and chromosome abnormalities. \u0000 \u0000 \u0000Methods \u0000Retrospective analysis was conducted for clinical data of 72 pregnant women with fetal cardiovascular system dysplasia from January 2013 to August 2018. And all 72 fetuses with abnormal cardiovascular system dysplasia were singletons with termination of pregnancy (n=45), loss of follow-up (n=6) and normal pregnancy (n=21). \u0000 \u0000 \u0000Results \u0000Twenty-two cases of abnormal karyotypes were detected in 72 patient samples with an abnormal rate of 30.6%(22/72), including 18-trisomy (n=10), 13-trisomy (n=3), 21-trisomy (n=2), sex chromosome mosaicism (n=1) and chromosomal structure abnormalities (n=6). As for fetal chromosome aneuploidy, the number of 18-trisomy accounted for 66.7%(10/15). There were single cardiovascular malformations (n=29), abnormal karyotypes (n=3), cardiovascular with other system malformations (n=43) and abnormal karyotypes (n=19). The abnormal karyotypic rate of cardiovascular malformations with other systems was higher than that of single cardiovascular malformations (44.2% vs. 10.3%, P=0.0035). As for fetal single cardiovascular system malformations, the distributions of gestational weeks were as follows: 28 weeks (n=4) & abnormal karyotype (n=1). As for fetal cardiovascular with other systemic malformations, the gestational weeks were 28 weeks (n=3) & abnormal karyotype (n=1). The abnormal karyotypic detection rates of cardiovascular malformation associated with urogenital, craniocerebral, facial neck, skeletal & extremity abnormalities were 100%(3/3), 71.4%(10/14), 58.8%(10/17) and 52.9%(9/17) respectively. However, no abnormal karyotype was detected in 8 cardiovascular cases with concurrent gastrointestinal malformations. \u0000 \u0000 \u0000Conclusions \u0000The number of chromosomes or structural changes is closely correlated with the abnormal development of fetal cardiovascular system. \u0000 \u0000 \u0000Key words: \u0000Fetus; Cardiovascular abnormalities; Chromosomes; Karyotyping","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"26 1","pages":"215-218"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90777634","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}

{"title":"Transplantation of free great toe fibular flap for repairing traumatic soft tissue defects of hands in children","authors":"Yiyang Liu, Li-feng Shen, B. Lin, Kai Huang, Qiong Zhang, Chun Zhang, Q. Guo","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.015","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.015","url":null,"abstract":"Objective \u0000To explore the efficacy and application value of free great toe fibular flap transplantation for traumatic soft tissue defect of hands in children. \u0000 \u0000 \u0000Methods \u0000From July 2013 to April 2018, 11 children with hand soft tissue defects due to mechanical trauma were treated with free fibular flap. There were 10 boys and 1 girl with an average age of 11.5(5-17) years. The area of transplanted skin flaps was at 3.0 cm×1.5 cm the smallest and at 5.5 cm×2.5 cm the largest. The first dorsal metatarsal arteries (or dorsalis pedis) were anastomosed with radial arteries. Full-thickness inguinal skin was employed for free skin grafting at donor site of flaps. \u0000 \u0000 \u0000Results \u0000The average operative duration was 3.5 hours. One artery and two veins were anastomosed. Postoperative vascular crisis occurred in 1 case. Skin flaps survived after timely surgical exploration. And all flaps survived completely in 11 cases. Necrosis of skin graft occurred in 3 cases and there was subcrustal healing after dressing change. After an average follow-up period of 10 months, skin flaps were satisfactory in appearance, normal in color, excellent elasticity and texture and basically consistent with the surrounding tissues of recipient area. According to Michigan Hand Outcome Questionnaire, the outcomes were \" very satisfied\" with appearance (n=11), \" very satisfied\" with the function evaluation of injured fingers (n=7), \" satisfied\" (n=2), \" unsatisfactory\" (n=1) and \" very unsatisfactory\" (n=1). There was no obvious disturbance of toe motor function at donor site of flap. \u0000 \u0000 \u0000Conclusions \u0000On the basis of mastering microvascular anastomosis technique and paying attention to comprehensive managements of perioperative period of children, the transplantation of free great toe fibular flap for repairing soft tissue defect in hands has achieved satisfactory outcomes. The recipient area is aesthetic with concealed donor area and excellent functions of fingers and toes. It is worthy of wider clinical applications. \u0000 \u0000 \u0000Key words: \u0000Surgical flap; Microsurgery; Transplantation; Child; Soft tissue injuries; Hand injuries","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"14 1","pages":"262-267"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84851172","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}

{"title":"Outcomes of robot-assisted laparoscopic pyeloplasty for ureteropelvic junction obstruction with small pelvis in children","authors":"C. Tao, D. Tang, Zheming Xu, Long Sun, Z. Gao, Qingjiang Chen, Q. Shu","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.004","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.004","url":null,"abstract":"Objective \u0000To summarize the experience of robotic-assistant laparoscopic pyeloplasty (RALP) for ureteropelvic junction obstruction (UPJO) with small pelvis in children. \u0000 \u0000 \u0000Methods \u0000A retrospective analysis was performed on clinical data of 12 UPJO children (small pelvis) undergoing RALP from March 2018 to October 2019. Their clinical presentations, operative approaches and follow-up data were analyzed. There were 7 boys and 5 girls with a mean age of 7(4-15) years. The involved side was left (n=9) and right (n=3). Ultrasound at 1-7 days preoperatively indicated anterioposterior diameter (APD) 3.5 cm during an on set of abdominal pain. And radionuclide examination hinted at different declining levels of renal function and the function of affected kidney ranged from 6% to 43%. \u0000 \u0000 \u0000Results \u0000RALP was performed all successfully without a conversion into open surgery. The pathogenesis was simple stricture or torsion (n=3), polyps (n=3), ectopic vascular compression (n=4), calculus incarceration with infection (n=1) and postcaval ureter (n=1). The average operative duration was 165(115-225) min and the average ureteropelvic anastomosis time 50(40-60) min. There were no obvious intraoperative complications and the mean volume of blood loss was <10 ml. The average follow-up period was 12(2-22) months. The mean preoperative value of APD was (1.87±0.57)cm and the mean postoperative value of APD (1.16±0.82)cm (P<0.05). \u0000 \u0000 \u0000Conclusions \u0000Robot-assisted laparoscopic pyeloplasty may be employed for pediatric UPJO with small renal pelvis (APD<2.5 cm). Such a technique is easy to learn and clinical application prospects are excellent. \u0000 \u0000 \u0000Key words: \u0000Child; Robot assisted laparoscopic surgery; Hydronephrosis; Ureteropelvic junction obstruction","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"24 1","pages":"205-209"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82756291","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}

{"title":"Surgical outcomes of 14 children with scimitar syndrome","authors":"Shengchun Yang, Li Ma, M. Zou, W. D. Chen, Techang Liu, Mingjie Zhang, Xinxin Chen","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.005","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.005","url":null,"abstract":"Objective \u0000To summarize the diagnostic and surgical experience of children with scimitar syndrome and to explore the immediate/mid-term outcomes of these operations. \u0000 \u0000 \u0000Methods \u0000Retrospective analysis was performed for 14 clinical records of children with scimitar syndrome from January 2010 to December 2017. There were 7 boys and 7 girls with a mean age of (18.23±28.55 months)(23 days to 9 years). The complications included other cardiac abnormalities (n=10), mesocardia (n=8), right pulmonary hypoplasia (n=7) and collaterall circulation (n=8). Follow-ups were conducted for analyzing the immediate/mid-term outcomes of survival and stenosis of corrected scimitar vein (SV). And postoperative survival curves were plotted. \u0000 \u0000 \u0000Results \u0000SVs were dissected from inferior caval vein (IVC) and anastomosed to left artrium (n=9); SVs (2 SVs) were resected together with partial IVC wall and anastomosed to left artrium (n=1); SVs (2 SVs) were resected respectively and anastomosed side-to-side and then anastomosed to left artrium (n=1); SVs were anastomosed to right atrium and interatrium septum be rebuilt (n=2) and SV was connected to artificial conduit and then connected to left atrium (n=1) owing to that SV was too far to left atrium. In the same term of operation, complicated cardiac abnormality was cured. And 2/14 cases died immediately postoperatively with a mortality rate of 14.29%; 1/12 survivors died of pneumonia and respiratory failure at 14 months postoperatively and 1/3/5-year postoperative cumulative survival rates were 86%, 73% and 73% respectively. One corrected SV of 12 survivors was stenosed during discharge and 3 corrected SVs of 11 survivors became stenotic during follow-ups. \u0000 \u0000 \u0000Conclusions \u0000Scimitar syndrome may be complicated with other cardiac abnormality, mesocardia and pulmonary hypoplasia, etc. Most SVs of scimitar syndrome may be cured by anastomosing SV to left artrium. SV should be anastomosed to right atrium instead of left atrium and interatrium septum. Collateral circulation of scimitar syndrome may be plugged during cardiac interventions and even resected intraoperatively. \u0000 \u0000 \u0000Key words: \u0000Scimitar Syndrome; Congenital heart disease; Cardiac surgical procedures","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"50 1","pages":"210-214"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80334748","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}

{"title":"Clinical characteristics and analysis of misdiagnosing mixed gonadal dysgenesis","authors":"Yongchuan Cai, Yu Mao, Jing Fu, L. Dai, Shaoji Chen, Xunjun Wang, Daorui Qin, Jiewen Zheng, Yu Liu, Yunman Tang","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.014","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.014","url":null,"abstract":"Objective \u0000To explore the clinical characteristics and causes and countermeasures of misdiagnosing mixed gonadal dysgenesis (MGD). \u0000 \u0000 \u0000Methods \u0000From May 2013 to April 2018, clinical data were retrospectively reviewed for 24 MGD children. The average age was 21(10-39) months and the average height 83(71-97) cm. Ten children fell below the average height of the same age group by 2 standard deviations. There were 22 boys and 2 girls. The Prader grade was II (n=3), III (n=15) and IV (n=6). The results of sex hormone determination and sex-related genes were analyzed.Fluorescence in situ hybridization (FISH) for sex chromosome was further tested in 8/10 children with 46, XY chromosome. And gonad specimens were evaluated for histopathology. \u0000 \u0000 \u0000Results \u0000The average level of anti-mullerian hormone (AMH) was 69.42(16.57-189.92) ng/ml. The average testosterone level was 4.93(0.71-8.09) nmol/L after hCG stimulation. WT1 gene mutation was detected in 1 child with a definite diagnosis of Danis-Drash syndrome (DDS). The karyotypes were 45, X/46, XY, 10 cases of 46, XY (n=12, including 8 cases confirmed by FISH as X chimeric XY), 45, X/46, XY/47XYY (n=1) and 45, X/47, XYY/48XYYY (n=1). Forty-eight specimens were classified as dyspastic testis (n=24), undifferentiated gonad tissue (n=1) (previously misdiagnosed as ovary tissue) and fibrous stripe gonads with sex-cord-like structure (n=4). No evidence of malignancy was noticed. \u0000 \u0000 \u0000Conclusions \u0000Ambiguous genitalia is common in MGD children with Muller tube remnant. For suspected MGD children with a 46, XY chromosome, further FISH test for sex chromosome is warranted. And undifferentiated gonad tissue may be found in a streak gonad and ovary tissue is probably mistaken for making a misdiagnosis of ovotesticular DSD. \u0000 \u0000 \u0000Key words: \u0000Gonadal dysgenesis; Sex chromosomes; Histopathology","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"3 1","pages":"257-261"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78733495","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}

{"title":"Pediatric intra-hepatic accessory paraganglioma: one case report","authors":"D. Jiang, Chongli Li","doi":"10.3760/CMA.J.ISSN.0253-3006.2020.03.018","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.0253-3006.2020.03.018","url":null,"abstract":"","PeriodicalId":10157,"journal":{"name":"Chinese Journal of Pediatric Surgery","volume":"37 1","pages":"273-275"},"PeriodicalIF":0.0,"publicationDate":"2020-03-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81087222","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}