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Objective:To evaluate the therapeutic efficacy of interposition urethroplasty on glandular hypospadias and severe penile curvature.Methods:A total of 9 cases of congenital glandular hypospadias and 2 cases of congenital severe penile curvature (>30°) treated with the interposition urethroplasty technique in the Shenzhen Children′s Hospital from November 2008 to December 2019 were retrospectively analyzed.The mean age of initial surgery was 40 (25-109) months.Two cases were surgically treated with one-staged interposition urethroplasty and the remaining were treated with two-staged interposition urethroplasty.They were followed up for 8 months to 12 years, including the penile morphology, urination, urethrocutaneous fistula, urethral stricture and uroflowmetry.Continuous variables were presented as ± s deviation, Student′s t-test was used for comparison between groups. Results:The median length of interposition urethra was 3.2 (2.2-4.2) cm.The cosmetic appearance of penis was good without residual chordee, urethral stricture or urethral diverticulum in all patients.Two cases had urethrocutaneous fistula(one-staged repair and two-staged repair were performed in one case respectively), which were successfully repaired by re-operation.The maximum of uroflowmetry in patients at 3 months [(8.3±1.0) mL/s] and 6 months [(6.7±1.9) mL/s] after surgery was significantly lower than that of children in healthy control group [(10.5±3.7) mL/s] ( t=3.221, 3.864, all P<0.05). However, there was no significant difference in 1-year maximum of uroflowmetry postoperatively between surgically treated patients [(10.5±3.7) mL/s] and healthy control group ( P>0.05). Conclusions:Interposition urethroplasty is an effective and safe treatment for glandular hypospadias and/or severe penile curvature in children.
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Objective:To summarize the clinical manifestations of four patients with 46, XY disorders of sex development(46, XY DSD)due to doublesex and mab-3 related transcription factor 1(DMRT1)gene variant/haploinsufficiency, and to improve the understanding of clinicians for this disease.Methods:The medical history, physical examination, endocrine function assessment, gonadal pathology, and genetic data of 4 patients with 46, XY DSD were retrospectively collected.Results:A heterozygous new missense mutation in DMRT1 was found in one child. The chief complain was primary amenorrhoea at the age of 15 years, with the external masculinisation score(EMS)0. The DMRT1 haploinsufficiency was found in 3 cases, 1.2 Mb, 5.1 Mb, and 6.0 Mb fragments were deleted at the 9p, and one of 3 cases had 33.3 Mb repeats in the 5p. All patients visited doctor under 1 year. Two patients were raised as females, and one was raised as male. All chief complains were external genital abnormalities, EMS of them were 1, 0, and 5 respectively. Endocrine evaluation of 2 out of 4 children showed varying degrees of primary hypogonadism, and presented with complete gonadal dysgenesis. One patient showed a well function of Leydig cells and poorly function of Sertoil cells, and presented with mixed gonadal dysgenesis. One of 3 cases was diagnosed with gonadoblastoma at the age of 18 months. Patient No.4 didn′t agree with the gonadal biopsy. The chromosome karyotypes of 4 children were 46, XY.Conclusions:The visiting ages of 46, XY DSD patients caused by DMRT1 variation were older than those of patients caused by DMRT1 haploinsufficiency. The clinical manifestations are complex, and gonadal function can vary from normal to complete gonadal dysgenesis. Such patients are at high risk of gonadoblastoma and young onset. Gonadal biopsy should be performed as early as possible.
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Objective@#Comparison of the efficacy of mini flank incision and laparoscopy in the treatment of infants with ureteropelvic junction obstruction.@*Methods@#We retrospectively analyzed 85 cases of infants with ureteropelvic junction obstruction in our hospital from Jan. 2016 to Jan. 2018, all the patients underwent dismembered pyeloplasty. According to the surgical approach, they were divided into two groups: There were 45 cases in the mini flank incision group, including 39 males and 6 females, aged from 1 month to 3 years, with a median age of 4 months, and 40 patients in the laparoscopy group, including 33 males and 7 females, aged from 2 months to 3 years, with a median age of 9 months. The clinical effects of the two groups were compared.@*Results@#The patients were followed up for 12-24 months. The operation time[(68.0±15.3)min vs.(79.6±18.8)min], fasting time[(5±1)h vs.(14±8)h] and indwelling time of peri-renal drainage tube[(3.1±1.4)d vs.(4.3±2.2)d] in the mini flank incision group were shorter than those in the laparoscopy group (P<0.01), but there was no significant difference in the amount of blood loss[(2.1±0.9)ml vs.(2.2±0.6)ml] during the operation (P>0.05). The recurrence rate[0 vs.5%(2/40)] and the incidence of complications[11.1%(5/45)vs. 17.5%(7/40)] in the mini flank incision group were lower than those in the laparoscopy group, but there was no statistical difference (P>0.05).@*Conclusions@#Pyeloplasty via mini flank incision or laparoscopy are both safe, effective and cosmetic.Mini flank incision surgery has the advantage of short operation time, easy to master and less disturbance of physiological function. Pyeloplasty via mini flank incision it is one of the reasonable options for the treatment of infants with ureteropelvic junction obstruction.
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Objective Comparison of the efficacy of mini flank incision and laparoscopy in the treatment of infants with ureteropelvic junction obstruction.Methods We retrospectively analyzed 85 cases of infants with ureteropelvic junction obstruction in our hospital from Jan.2016 to Jan.2018,all the patients underwent dismembered pyeloplasty.According to the surgical approach,they were divided into two groups:There were 45 cases in the mini flank incision group,including 39 males and 6 females,aged from 1 month to 3 years,with a median age of 4 months,and 40 patients in the laparoscopy group,including 33 males and 7 females,aged from 2 months to 3 years,with a median age of 9 months.The clinical effects of the two groups were compared.Results The patients were followed up for 12-24 months.The operation time[(68.0 ± 15.3) min vs.(79.6 ± 18.8) min],fasting time [(5 ± 1) h vs.(14 ± 8) h] and indwelling time of peri-renal drainage tube [(3.1 ± 1.4) d vs.(4.3 ± 2.2) d] in the mini flank incision group were shorter than those in the laparoscopy group (P ＜ 0.01),but there was no significant difference in the amount of blood loss[(2.1 ± 0.9) ml vs.(2.2 ± 0.6) ml] during the operation (P ＞ 0.05).The recurrence rate [0 vs.5 ％ (2/40)]and the incidence of complications[11.1％ (5/45) vs.17.5％ (7/40)] in the mini flank incision group were lower than those in the laparoscopy group,but there was no statistical difference (P ＞ 0.05).Conclusions Pyeloplasty via mini flank incision or laparoscopy are both safe,effective and cosmetic.Mini flank incision surgery has the advantage of short operation time,easy to master and less disturbance of physiological function.Pyeloplasty via mini flank incision it is one of the reasonable options for the treatment of infants with ureteropelvic junction obstruction.
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Objective To study the clinical features, diagnosis and treatment of torsion of testes in newborn. Method Neonates who were diagnosed with neonatal testicular torsion and admitted to Shenzhen Children's Hospital from March 2008 to July 2018 were studied. The clinical data such as days in age, time of onset, clinical manifestations, time of ultrasound examination, characteristics of ultrasound examination, surgery time, surgical types, postoperative conditions, pathological findings, and follow-up results were retrospectively analyzed. Result A total of 12 infants with torsion of testes were enrolled. The average onset time was 2.9 d, ranged from 1～10 d. The time of onset was within 24 h after birth in six infants. The median duration from onset to seeing a doctor was 3.5 d, ranged from 2 h to 28 d. First manifestations being reported grammer were scrotal swelling or mass, including 7 cases on the left side and 5 cases on the right side. Among them, 9 cases were associated with redness or cyanosis of the scrotum. Ultrasound was characterized by the disappearance or significant reduction of testicular parenchymal blood flow signal, and the sensitivity of ultrasound was 100％. The average time from admission to operation was (2.1±1.1) h. All the 12 infants had orchiectomy,after necrosis of unilateral testicle was confirmed. Eight of them underwent contralateral test icular fixation. The average operation time was 46 min. There was no wound bleeding or infection postoperatively, and the average hospital stay was 6.4 d. The pathological features were blurred residual contour of the seminiferous tubule (9 cases) or the disappearance of the seminiferous tubule structure (3 cases). After 3 to 24 months of follow-up, no contralateral testicular torsion or atrophy was found. Conclusion The rate of testicular necrosis in children with torsion of testes is high. The newborn with scrotal swelling should be diagnosed promptly with color Doppler ultrasound. If necessary, surgical exploration should be performed in time.
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Objective To investigate the clinical efficacy of 0.1％ mometasone furoate cream in the treatment of phimosis in children.Methods A prospective study was carried out over two years period on an outpatient which basis on two groups of patients with severe phimosis.598 children with severe phimosis (Kikiros classification 4-5) aged from 2 years old to 11 years old and 8 months were selected.311 cases in the observation group and 287 cases in the control group.The observation group applied a steroid cream the foreskin twice a day for 4 weeks,and the control group used local handling of the foreskin twice a day for 4 weeks.The effects of the two groups after 2 and 4 weeks of treatment were compared.Results 29 cases in the observation group and 47 cases in the control group were loss of follow up.In the steroids group which including 282 patients,68.8％ of patients (194 cases) showed a complete response (full retraction of the foreskin) to the therapy.The total efficiency rate of the 4 stage phimosis group is higher than the 5 grade phimosis group.Patients who had a history of balanoposthitis or urinary tract infection showed poorer improvement in preputial retraction.A total of 28 out of 240 patients (11.7％) in the control group showed a complete response to the therapy.The total efficiency rate of the observation group was significantly higher than the control group (x2 =173.121,P ＜ 0.01).There were 4 cases of discomfort in the observation group and 6 cases of foreskin injured in the control group.Conclusions Topical application of 0.1％ mometasone furoate cream in the treatment of severe phimosis in children is an effective,safe and simple non-invasive treatment with less adverse reactions.
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Objective To study the clinical features of complicated upper urinary tract infection in children,aiming to provide a reference for clinical diagnosis and treatment of the disease.Methods The clinical data of 68 cases with complicated upper urinary tract infection hospitalized at the Department of Urinary Surgery NO.1,Children's Hospital of Shenzhen between January 2013 and August 2015 were retrospectively analyzed.Results A total of 68 cases,in which 44 were male,24 were female,and repeated infections were found in 15 cases.Fever was the most common clinical manifestation(48 cases,70.59％),while frequent urination and odynuria were rare(9 cases,13.24％).A total of 57 strains had been cultured form the submitted specimens which were obtained from the 68 cases,including 41 strains of gram-negative bacteria,15 strains of gram-positive bacteria and 1 strain of fungus.Two different strains were cultured in 3 children.Fourteen strains of Escherichia coli had 11 extended spectrum beta lactamases (ESBLs)-positive strains.In the 11 strains of klebsiella pneumoniae,8 trains were ESBLs-positive.The drug resistant rates of gram-negative bacteria to Ampicillin and Cefuroxime sodium were both more than 90％.The sensitive rates to Piperacillin/Tazobactam was more than 90％.Thirty-one cases of complicated upper urinary tract infection were cured by administering Piperacillin/Tazobactam,while 15 cases were cured by changing Cefuroxime sodium to Piperacillin/Tazobactam according to the drug sensitivity results.Conclusions Clinical manifestations of complicated upper urinary tract infection are untypical,and fever is the most common symptom.Repeated infection is common.The gram-negative bacteria is the dominant pathogen causing the complicated upper urinary tract infection.ESBLs-positive bacteria accounts for high proportion.The drug resistance rate to penicillin and the first,second generation of the cephalosporin is high.The drug sensitive rate of piperacillin tazobactam is high,with good prognosis.
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Objective To summarize the clinical manifestations of 46, XX ovotesticular disorder of sex development (DSD) caused by a NR5A1 heterozygous mutation. Methods The first case of 46,XX ovotesticular DSD was caused by a NR5A1 heterozygous mutation in China and was reported with a review of 11 similar cases in the literatures since July 2016. Results A 5. 6-year-old child raised as female was born with ambiguous genitalia. The left gonad was palpable in the inguinal region while the right one was located in abdomen. Gonadal histology showed both ovotestis. Vaginoscopy revealed a short, blind-ending vagina. No uterine was detected by laparoscopy. Repeated karyotype results were 46, XX with SRY gene negative. A heterozygous de novo mutation ( p. Arg92Trp) in the accessory DNA-binding region of NR5A1 gene was found in that child. Conclusions We reported for the first time in China a new phenotype caused by a NR5A1 heterozygous mutation-46,XX ovotesticular DSD. According to the review of literatures, such mutation seemed with incomplete penetrance. It could cause both 46, XX DSD and 46, XY DSD with varied manifestations. The possible underlying mechanism might relate to the impairment of the binding between the mutant protein and target DNA which might lead to a decreased inhibition of the male developmental pathway through downregulation of female antitestis genes.
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Objective To summarize the clinical manifestations of 46, XX ovotesticular disorder of sex development (DSD) caused by a NR5A1 heterozygous mutation. Methods The first case of 46,XX ovotesticular DSD was caused by a NR5A1 heterozygous mutation in China and was reported with a review of 11 similar cases in the literatures since July 2016. Results A 5. 6-year-old child raised as female was born with ambiguous genitalia. The left gonad was palpable in the inguinal region while the right one was located in abdomen. Gonadal histology showed both ovotestis. Vaginoscopy revealed a short, blind-ending vagina. No uterine was detected by laparoscopy. Repeated karyotype results were 46, XX with SRY gene negative. A heterozygous de novo mutation ( p. Arg92Trp) in the accessory DNA-binding region of NR5A1 gene was found in that child. Conclusions We reported for the first time in China a new phenotype caused by a NR5A1 heterozygous mutation-46,XX ovotesticular DSD. According to the review of literatures, such mutation seemed with incomplete penetrance. It could cause both 46, XX DSD and 46, XY DSD with varied manifestations. The possible underlying mechanism might relate to the impairment of the binding between the mutant protein and target DNA which might lead to a decreased inhibition of the male developmental pathway through downregulation of female antitestis genes.
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Objective To research the operation method for hypospadias by one-stage urethroplasty.Methods According to the length of urethra defect,18 cases were operated by using one-stage urethroplasty with perineal and preputial island flaps.Results 16 cases were successful in all patients(88.9%),except one case of urethrostenosis and urethral fistula respective.The case of urethrostenosis recovered by dilation of urethra after 5 months and the patient of urethral fistula was successfully repaired by operation after 8 months.All patients were followed up for 6～26 months with satisfactory penis and micturition.Conclusion The technique of one-stage urethroplasty by using perineal and preputial island flaps is the one of best choices for hypospadias.