Objective:To investigate the efficacy and safety of transurethral incision for the treatment of ureterocele in infants.Methods:A retrospective analysis of 28 cases of ureterocele admitted from March 2012 to May 2023 were reviewed, all of which were less than 1 year old, 16 male and 12 female, with an average age of(5.7±3.5)months. The ureterocele was located on the left side in 8 cases, on the right side in 15 cases, and bilaterally in 5 cases. There were 12 cases of single system ureterocele, of which 7 cases were unilateral and 5 cases were bilateral. Duplex system ureterocele was observed in 16 cases, all of which were unilateral. Clinical manifestations: urinary tract infection in 13 cases, 11 cases of ureterocele or hydronephrosis and ureteral dilation were found during antenatal examination, and 4 cases of ureterocele were found after birth. Urological ultrasound, intravenous pyelography(IVP) and voiding cystourethrography(VCUG) were performed in all children, and 17 cases underwent magnetic resonance urolography (MRU), and confirm the diagnosis of ureterocele preoperatively. All of the cases were performed the transurethral incision.The ureterocele was punctured and incised 1-2 mm at the base of the bulge, and 2-4 points were punctured according to the bulge atrophy. Bilateral ureteroceles were punctured and incised simultaneously. Postoperative urine routine test, urinary tract color ultrasound and VCUG were performed to determine if there is urinary tract infection, hydronephrosis, ureteral dilation and bulging, and whether a second surgery is needed.Results:All operations were conducted successfully. The intraoperative bleeding was less than 3 ml and no intraoperative complications. The operative time was (28.4±10.3) min. The median postoperative follow-up was 34 (32, 36) months. Six cases underwent postoperative VCUG examination. Eleven children were recovered well with single systemic ureterocele. One child developed grade Ⅳ vesicoureteral reflux(VUR)and combined with bladder diverticulum, and ureterocele underwent open diverticulotomy and ureteral reimplantation six months after surgery. Nine children were recovered well with duplex systemic ureterocele. Six cases of children developed infection, of which 2 cases had an infection once within one month after TUI, and the other four cases still had intermittent infections after six months and VCUG was performed, and one case showed grade Ⅲ VUR of the lower ureter, which was observed conservatively, while the other three cases had enlarged cysts but no VUR, and upper heminephrectomy was performed, and the patients recovered well after surgery. Except for these 6 exceptions, in another case, after ten years of follow-up, the ureterocele became larger but no VUR, and the results were good after a second transurethral incision. There was no significant difference in the postoperative infections, new VUR cases, and secondary surgeries between the two groups.Conclusions:Transurethral incision has good surgical effect on children with single system ureterocele and duplex system ureterocele, and has advantages of easy operation, less trauma, safety and effectiveness, and few complications. It deserves to be recommended as the treatment of choice, especially for infants and young children.

Objective:To explore feasible surgical options for management of persistent Müllerian duct syndrome (PMDS) in children.Methods:A retrospective case summary was made.The clinical data of 8 patients who were diagnosed with PMDS and treated at Shanxi Children′s Hospital from September 2011 to November 2020 were retrospectively reviewed.Of the 8 PMDS patients, 6 were sporadic cases, 2 were twins (monochorionic), with an age range from 1 year to 12 years.All cases had normal male external genital organs and a chromosomal karyotype of 46, XY.During the operation, the surgical plan was selected according to clinical classification, vas structure and the development of the initial uterus.After the operation, the Müllerian structure in the abdominal cavity and the testicular development of the children were followed up through color ultrasound at the outpatient department.Results:Four patients had oblique inguinal hernia, 3 had bilateral cryptorchidism, and 1 had transverse testicular ectopia.Müllerian inhibitory hormone (MIH) levels were significantly decreased in 5 cases and normal in 1 case.The other 2 cases were not tested for MIH.All bilateral gonadal biopsies were testicular tissue, but undifferentiated gonadal tissue was detected in 3 cases.Four cases underwent radical surgery after gonadal biopsy.Among these 4 children, 3 received one-stage radical operation, and 1 received repair of oblique hernia before the radical surgery.Three patients had a well-defined vas structure and underwent hysterectomy and bilateral orchidopexy; three patients had a vague vas structure and received orchidopexy with the uterus preserved; one case underwent hysterectomy, orchidopexy on one side and orchiectomy on the other side; one case had bilateral orchidectomy in Fowler-Stephens stage.All the 8 children were followed up for 2 to 12 years.Ultrasound showed that the descending testis was well developed, with no cryptorchidism retraction or hernia recurrence.No tumor was observed in the preserved uterus in the abdominal cavity.Conclusions:The plan for PMDS management in children should be determined based on its clinical classification.Gonadal biopsy is essential, and whether to remove the Müllerian structure depends on the vas structure and the development of the uterus.

Objective:To explore feasible surgical options for management of persistent Müllerian duct syndrome (PMDS) in children.Methods:A retrospective case summary was made.The clinical data of 8 patients who were diagnosed with PMDS and treated at Shanxi Children′s Hospital from September 2011 to November 2020 were retrospectively reviewed.Of the 8 PMDS patients, 6 were sporadic cases, 2 were twins (monochorionic), with an age range from 1 year to 12 years.All cases had normal male external genital organs and a chromosomal karyotype of 46, XY.During the operation, the surgical plan was selected according to clinical classification, vas structure and the development of the initial uterus.After the operation, the Müllerian structure in the abdominal cavity and the testicular development of the children were followed up through color ultrasound at the outpatient department.Results:Four patients had oblique inguinal hernia, 3 had bilateral cryptorchidism, and 1 had transverse testicular ectopia.Müllerian inhibitory hormone (MIH) levels were significantly decreased in 5 cases and normal in 1 case.The other 2 cases were not tested for MIH.All bilateral gonadal biopsies were testicular tissue, but undifferentiated gonadal tissue was detected in 3 cases.Four cases underwent radical surgery after gonadal biopsy.Among these 4 children, 3 received one-stage radical operation, and 1 received repair of oblique hernia before the radical surgery.Three patients had a well-defined vas structure and underwent hysterectomy and bilateral orchidopexy; three patients had a vague vas structure and received orchidopexy with the uterus preserved; one case underwent hysterectomy, orchidopexy on one side and orchiectomy on the other side; one case had bilateral orchidectomy in Fowler-Stephens stage.All the 8 children were followed up for 2 to 12 years.Ultrasound showed that the descending testis was well developed, with no cryptorchidism retraction or hernia recurrence.No tumor was observed in the preserved uterus in the abdominal cavity.Conclusions:The plan for PMDS management in children should be determined based on its clinical classification.Gonadal biopsy is essential, and whether to remove the Müllerian structure depends on the vas structure and the development of the uterus.

Objective:To investigate the efficacy and safety of transurethral incision for the treatment of ureterocele in infants.Methods:A retrospective analysis of 28 cases of ureterocele admitted from March 2012 to May 2023 were reviewed, all of which were less than 1 year old, 16 male and 12 female, with an average age of(5.7±3.5)months. The ureterocele was located on the left side in 8 cases, on the right side in 15 cases, and bilaterally in 5 cases. There were 12 cases of single system ureterocele, of which 7 cases were unilateral and 5 cases were bilateral. Duplex system ureterocele was observed in 16 cases, all of which were unilateral. Clinical manifestations: urinary tract infection in 13 cases, 11 cases of ureterocele or hydronephrosis and ureteral dilation were found during antenatal examination, and 4 cases of ureterocele were found after birth. Urological ultrasound, intravenous pyelography(IVP) and voiding cystourethrography(VCUG) were performed in all children, and 17 cases underwent magnetic resonance urolography (MRU), and confirm the diagnosis of ureterocele preoperatively. All of the cases were performed the transurethral incision.The ureterocele was punctured and incised 1-2 mm at the base of the bulge, and 2-4 points were punctured according to the bulge atrophy. Bilateral ureteroceles were punctured and incised simultaneously. Postoperative urine routine test, urinary tract color ultrasound and VCUG were performed to determine if there is urinary tract infection, hydronephrosis, ureteral dilation and bulging, and whether a second surgery is needed.Results:All operations were conducted successfully. The intraoperative bleeding was less than 3 ml and no intraoperative complications. The operative time was (28.4±10.3) min. The median postoperative follow-up was 34 (32, 36) months. Six cases underwent postoperative VCUG examination. Eleven children were recovered well with single systemic ureterocele. One child developed grade Ⅳ vesicoureteral reflux(VUR)and combined with bladder diverticulum, and ureterocele underwent open diverticulotomy and ureteral reimplantation six months after surgery. Nine children were recovered well with duplex systemic ureterocele. Six cases of children developed infection, of which 2 cases had an infection once within one month after TUI, and the other four cases still had intermittent infections after six months and VCUG was performed, and one case showed grade Ⅲ VUR of the lower ureter, which was observed conservatively, while the other three cases had enlarged cysts but no VUR, and upper heminephrectomy was performed, and the patients recovered well after surgery. Except for these 6 exceptions, in another case, after ten years of follow-up, the ureterocele became larger but no VUR, and the results were good after a second transurethral incision. There was no significant difference in the postoperative infections, new VUR cases, and secondary surgeries between the two groups.Conclusions:Transurethral incision has good surgical effect on children with single system ureterocele and duplex system ureterocele, and has advantages of easy operation, less trauma, safety and effectiveness, and few complications. It deserves to be recommended as the treatment of choice, especially for infants and young children.

Objective:To investigate the clinical application effect of enhanced recovery after surgery(ERAS) in the perioperative period of primary repair for children with hypospadias.Methods:Non-randomized controlled study.Retrospective analysis was made on clinical data of 206 children with hypospadias admitted to the Department of Urology, Shanxi Children′s Hospital from March 2018 to March 2022.According to whether the concept of ERAS was adopted for perioperative management of children, they were divided into the ERAS group(109 cases) and the non-ERAS group(97 cases). t-test and Chi-square test were used to analyze the differences between the two groups of patients in hospitalization days, hospitalization costs, pain scores on the first day after surgery, average number of bowel movements within 5 days after surgery, first out-of-bed activity time, hospital satisfaction score, and complication rate. Results:Pediatric patients in the ERAS group showed better outcomes compared to the non-ERAS group: shorter hospital stays[(7.71±1.12) d vs.(21.00±1.07) d], lower hospitalization costs[(10 043.21±1 341.00) yuan vs.(12 993.44±1 081.00) yuan], lower postoperative pain scores on the first day[(5.21±1.32) points vs.(6.09±1.07) points], more frequent bowel movements within 5 days[(5.16±0.65) times vs.(2.91±0.48) times], earlier time to first out-of-bed activity[(5.03±0.61) d vs.(7.12±0.57) d], and higher hospital satisfaction scores[(4.71±0.21) points vs.(4.14±0.15) points], and the differences were statistically significant(all P<0.05).Additionally, the ERAS group had lower rates of fever(6.42%, 7/109) and constipation(3.67%, 4/109) before urinary catheter removal compared to the non-ERAS group[21.64%(21/97) and 28.87%(28/97), respectively](all P<0.05).There were no significant differences in the incidences of complications such as urethral prolapse, infection, delayed wound healing, and bleeding, as well as urethral stricture, urethral fistula, and urethral diverticulum after urinary catheter removal between the two groups(all P>0.05). Conclusions:ERAS protocol applied in the perioperative period of primary repair for children with hypospadias can shorten the hospitalization time, lower the hospitalization costs, and accelerate the rehabilitation of children.It is worth further promotion.

Abdominoscrotal hydrocele is rare in clinic. In the past, routine examination and diagnosis were difficult, easy to be misdiagnosed. The daily operations were mostly completed through the groin area or abdominal incision, the wound is large. The application of laparoscopy can clearly diagnose the abdominoscrotal hydrocele through "springing back ball" sign, and can cure the disease by laparoscopic resection of interperitoneal mass and closure of the internal ring. It is worthy of clinical application. In this article, we summarized and analyzed the clinical experience of 15 cases of children with abdominoscrotal hydrocele diagnosed and treated by laparoscopy, to explore the value of the laparoscopic technology in the diagnosis and treatment of the abdominoscrotal hydrocele.

Objective To analyze the correlation of clinical phenotype and genotype and gene mutation frequency characteristics of 21-hydroxylase deficiency,and to provide the basis for clinical diagnosis and methods for early intervention.Methods The clinical phenotypic signs and examination results of 14 cases with 21-hydroxylase deficiency were collected from September 2008 to December 2016 in Children's Hospital of Shanxi Province.Point mutations,deletions and conversion mutations for gene CYP21A2 coding 21-hydroxylase were detected through using next generation sequencing(NGS) and multiplex ligation-dependent probe amplification (MLPA).The captured mutations were further confirmed with Sanger sequencing.Furthermore,the family members underwent the co-segregation validation through the Sanger sequencing or MLPA in those captured mutated sites.Results Among the total 14 cases,9 cases were identified as the salt wasting,5 cases the simple virilizing;10 cases of compound heterozygous mutations,and 4 cases of homozygous mutations.Analysis of the 14 patients revealed 8 different kinds of mutations in CYP21A2 gene.The most frequent mutations of CYP21A2 gene were I2G [50％ (14/28)] and I173N [21.4％ (6/28)],followed by Arg357Trp[10.7％ (3/28)].Del[10.7％ (3/28)] mutations including E247fs,Gly1 1 1fs and R484fs.Q319X [3.6％ (1/28)] and Arg355His[3.6％ (1/28)] were rarely found.Missense mutation was found in 10 cases,splicing mutation in 14 cases,frameshifi mutations in 3 cases,nonsense mutations in 1 case.All of the mutations were inherited from their parents,and no new mutation was found.The most common mutations for salt wasting and simple virilizing were respectively I2G[50％ (9/18)] and I173N [50％ (5/10)].Collectively,genotypes and phenotypes were matched with each other.Conclusions The combination of clinical phenotypes with laboratory examination by gene sequencing and comprehensive analysis,is helpful to early diagnosis,differential diagnosis and optimized treatment,which will improve prognosis and provide guidance for genetic consultancy.

AIM:To investigate the potential role of Sonic hedgehog (Shh) signaling pathways in the radioresistance of esophageal cancer.METHODS:Radioresistant cell line Eca109R was established by repeating X-ray irradiation at dose of 60 Gy in total using Eca109 cells as parental cells.The radiosensitivity of the parental and radioresistant cells was confirmed by colony formation assay.The cell viability was detected by CCK-8 assay.The intracellular protein levels of Shh and Gli1 were determined by Western blot and immunofluorescence.RESULTS:The survival fractions at dose of 2 Gy for Eca109R cells and Eca109 cells were 0.937±0.013 and 0.499±0.042, respectively.The inhibitory rate of cell viability decreased gradually in the Eca109R cells (P<0.05), suggesting that the radioresistant cell line was successfully established.The results of Western blot indicated that the protein expression of Shh and Gli1 was much higher in the Eca109R cells than that in the Eca109 cells (P<0.05).Immunofluorescence staining showed that Gli1 was expressed in the cytoplasm and nucleus, and presented nuclear clustering in the Eca109R cells.The positive rate of Gil1 expression in Eca109 cells was 52.3%± 0.035%, while that in Eca109R cells was 87.6%±0.021% (P<0.05).CONCLUSION:The radioresistance of esophageal cancer may be related to the activation of Shh signaling pathways with over-expression of Gli1 and other related proteins.

Objective To evaluate the effect of rhubarb on intestinal mucosal damage in septic rats by comparing with ulinastatin.Methods A total of 84 healthy Sprague-Dawley rats (half male,half female),aged 3 months,weighing 200-330 g,were randomly divided into 5 groups according to the random number table:control group (group C,n =6),sham operation group (group S,n =6),sepsis group (group Sep),rhubarb group (group R,n=24) and ulinastatin group (group U,n=24).Sepsis was induced by cecum ligation and puncture.In group R,rhubarb 1.2 g/100 g was dissolved in normal saline at room temperature,3 and 4 h later the filtrate about 2-3 ml was obtained and injected through a gastric tube into stomach once every 12 h,and 72 h later sepsis was induced.In group U,ulinastatin 50 000 U/kg (in 2 ml of normal saline) was injected once every 24 h,and 72 h later sepsis was induced.In Sep,R and U groups,at 6,12,24 and 48 h after ligation (T1 4),blood samples were collected from the orbital venous plexus for determination of plasma diamine oxidase (DAO) activity.Results The activity of plasma DAO was significantly higher at T1-4 in Sep,R and U groups than in C and S groups.The activity of plasma DAO was significantly lower at T3,4 in R and U groups than in Sep group.There was no statistical difference in the plasma DAO activity between R group and U group.Conclusion Rhubarb can reduce intestinal mucosal damage in septic rats,which is similar to that of ulinastatin.