Objective To evaluate the risk urodynamic factors of upper urinary tract deterioration in children with neurogenic bladder dysfunction secondary to myelodysplasia. Methods Urodynamic and imaging studies were performed in 32 children with myelodysplasia. Results 18 patients had ureterohydronephrosis and 4 of them had vesicoureteric reflux.Of 19 children with detrusor leak point pressure higher than 40 cmH 2O and bladder compliance less than 40ml/cmH 2O, upper tract deterioration was revealed in 17.In contrast,of 13 patients with detrusor leak point pressure less than 40 cmH 2O and bladder compliance higher than 40 ml/cmH 2O, only one had bilateral ureteral dilatation( P

Adolescent ; Female ; Humans ; Patellar Dislocation ; physiopathology ; surgery ; Patellar Ligament ; injuries ; physiopathology ; surgery ; Range of Motion, Articular ; Reconstructive Surgical Procedures

Objective To provide valuable references for ultrasonic diagnosis of pelvic organ prolapsed (POP) by finding an eas ily detecting referential line based on MRI.Methods Data of 107 patients who underwent pelvic MRI were retrospectively analyzed.All the patients were divided into 6 groups according to age:Group 1 (20 29 years old),group 2 (30-39 years old),group 3 (40-49 years old),group 4 (50-59 years old),group 5 (60-69 years old) and group 6 (≥70 years old group).Four reference lines were set basing on the median sagittal view of T2WI:PS-PS line (the line connecting the two endpoint of the pubic symphysis),PIAS line (the line connecting the inferior margin of pubic symphysis and the bottom of sphincter internal anal sphincter),PPC line (the line connecting of the inferior margin of pubic symphysis and the point of the pubococcygeous attached on the rectum) and PM line (the line connecting of the inferior margin of pubic symphysis and the M point [the midpoint of the line from the crosspoint of PPC line and the front wall of the rectum mucosa to the bottom of sphincter internal anal sphincter]).The angles between the horizontal line and PS-PS line,PIAS line,PM line,PPC line (angle 1,angle 2,angle 3,angle 4) were measured,respectively.The differences of the angle among various age-groups were compared.The consistency between the two observes were evaluated.Results PM line was the closest line to the horizontal line.There was no statistical differences of angle 1,angle 2 and angle 3 among the 6 groups (all P＞0.05).The difference of angle 4 among the 6 groups were significant (F=3.42,P=0.01).Intergroup pairwise comparisons showed that significant differences were found between group 1 and group 4,group 5,group 6,between group 2 and group 4,group 5,group 6,between group 3 and group 4,group 5,group 6,respectively (all P＜0.05).And no significant difference was found in the other comparisons.The consistency of the two observers in meaning angle 1,angle 3 and angle 4 were good,but the consistency of angle 2 was poor.Conclusion Of all the referential lines,PM is the closest to the horizontal line and is less influenced by the patient's age.However,the feasibility of using PM lines as the ultrasound referential line is still unclear.

Objective To compare the treatment efficacy of severe hypospadias between two-stage urethral plate reconstruction procedure and two-stage tubularized transverse preputial island flap procedure. Methods From 2010 December to 2014 December, we retrospectively analyzed 82 cases with severe hypospadias, using two-stage urethral plate reconstruction and two-stage tubularized transverse preputial island flap urethroplasty, respectively.Their mean age in the first operation was 2.4 years, ranged from 2 to 3 years.Hypospadias were penoscrotal type in 32 cases, perineal type in 50 cases.In group A, 42 cases, including penoscrotal type in 15 cases, perineal type in 27 cases, with urethral plate reconstruction procedure underwent two-stage urethroplasty.The transected ventral penile urethral plate and to strip the ventral fibrous tissue were needed in correction of chordee of penis.The patients in group A were underwent the second urethroplasty with urethral plate reconstruction procedure 6-12 months later.In group B, 40 cases,including penoscrotal type in 17 cases, perineal type in 23 cases, who underwent one stage operation in the correction of chordee of penis, only with tubularized preputial island flap forming the part of the urethra.The patients in group B were underwent the second urethroplasty 6 ~12 months later.Results Patients were followed up for 1 to 5 years, mean 3.5 years.No recurrence of penis chordee was noticed.In group A, 6 cases (14.3%) had postoperative fistula.The second fistula repairing was perform 6 to 12 months after the second stage operation.However, still 3 cases were noticed the fistula recurrence, which was cured after the fourth operation.In group B, 3 cases (7.5%) developed fistula, which appeared in the second urethroplasty (P<0.01).They were all treated after the second repairing procedure.In group A, 4 cases were noticed urethral stricture, the urethral stricture rate was 9.5%.The stricture occurred 1 to 3 months after the second operation.After dilation and indwelling catheter, 3 cases resolved the problem. Another case still found the recurrence after 2 months, which the one stage urethral plication and second urethral reconstruction were considered to treat this patient.In group B, 1 cases of urethral stricture, the urethral stricture rate was 2.5%(P<0.01).After dilation and indwelling catheter, this case resolved the problem.In group A, 3 cases had urethral diverticulum, which occurred at a rate of 7.1%, whereas without urethral diverticulum occurred in group B (P<0.01).Those patient with urethral diverticulum accepted the diverticulum removing procedure 3 to 6 months after the second stage procedure.One case was found the urethral fistula, which was treated one year later.The success rate of operation was 69.0%in group A, the success rate of the operation was 90.0%in group B.The difference of the success rate between two groups was statistically significant ( P <0.01 ) . Conclusions With respect to two-stage urethral plate reconstruction procedure in treatment of severe hypospadias, there were the low complication rates of postoperative urinary fistula, urethral stricture and diverticulum in two-stage tubularized transverse preputial island flap procedure.Urethral meatus could be done at the glans of the penis.In addition, the two-stage tubularized transverse preputial island flap procedure provides a good opportunity to practice to master complex hypospadias operation.

0.05). A rise in CAP threshold and reduction in CM amplitude after perfusion were found in the other three groups(P

Objective To discuss the management of the congenital vesicoureteral junction obstruction (UVJO). Methods A retrospective cohort study was performed of patients who underwent ureteral reimplantation due to UVJO between 2003 and 2008. Of the 73 patients with 83 ureters (male 49 and female 24, age range 8 months to 13 years, median 41 months). Forty-one cases were on the left, 22 were on the right and 10 were bilateral obstruction. The most common presenting symptoms were intermittent abdominal pain and urinary infection. All patients were evaluated preoperatively by ultrasound, voiding cystourethrogram, intravenous pyelogram and diagnosed as UVJO. Reimplantation was done by the Cohen technique in all patients. Results Follow-up of 46 patients ranged 0.5-3.5 years postoperatively, including ultrasound, voiding cystourethrogram, intravenous pyelogram. Hydronephrosis improve in 41 patients, had no change in 5 ureters. Conclusions Symptomatic children or advanced hydroureteronephrosis are definite indication for surgical treatment of UVJO patients. Surgical management could be effective for most of patients.

Objective To evaluate the long-term functional results, complications and patient's satisfaction level in patients performed augmented enterocystoplasty and continent urinary diversion using the appendix. Methods From 1999 to 2005, there were 22 children (12 males and 10 females) underwent augmented enterocystoplasty and continent urinary diversion using the appendix. Surgical results were reviewed retrospectively. There were 11 eases with bladder and urethra dysfunction attributed to neurogenic bladder, 2 cases with complex genitourinary malformation associated with an imperforated anus, 2 cases with exstrophy-epispadias complex, 2 cases with posterior urethral valves, 3 cases with failed urethrovaginal fistula repair and 2 epispadias cases with post failure of Young-Dees- Leadbetter bladder neck reconstruction. Upper urinary tract dilatation and hydroureteronephrosis were found in 17 eases (28 units), including grade Ⅱ-Ⅴ vesicoureteral reflux in 15 children (24 units). Simultaneous procedures included ureteral reimplantation in 15 cases and bladder neck closure in 14 cases. The appendix was used as the catheterizable conduit placed in the right lower abdomen and clean intermittent catheterization was performed in all patients. Outcomes were assessed by urodynamic study, IVU, ultrasound, voiding cystourethrography, BUN, Cr and electrolyte test. Results Mean follow-up was 3.6 years, ranging from 1.5-6 years. Complications included stomal stenosis requiring dilatation in 2 cases and leakage in 2 cases without bladder neck closure and 1 case required surgical revision. All patients achieved excellent stomal continence. No metabolic acidosis and bladder stone was noted. Upper urinary tract impairment had not worsened in all patients. Conclusion Augmented enterocystoplasty and continent urinary diversion using the appendix are associated with high continence, compliance and satisfaction rate and a low complication rate in the treatment of pediatric patients with disorders related to bladder and urethral dysfunction.

Objective To investigate the diagnosis,treatment and prognosis of nephroblastomatosis (Nbm) combined with Wilms'tumor (WT). Methods Clinical data of six patients treated for WT combined with Nbm in Beijing Children's Hospital from 2006 to 2010 were reviewed retrospectively.The patients'ages ranged from five to 14 months.Two of the patients were female and four were male.The WTs were left-sided in four cases and right-sided in two cases.The Nbms were ipsilateral with WT in three cases,contralateral in two cases and bilateral in one case. The Nbms were single In three cases and multiple in three cases.WTs were all single and the maximum diameter was 3,4,8,10,11,and 12 cm respectively.Two paitents underwent nephrectomy.Nephron sparing surgery and upper and lower pole nodule biopsy was conducted in two cases,Nephrectomy and contralateral nephron sparing surgery was conducted in an additional two cases.Adjuvant chemotherapy included vincristine,actinomycin and doxorubicin. Results One patient had tumor recurrence 33 months after a 15 month regimen of postoperative chemotherapy. One patient had tumor recurrence and died after nephron sparing surgery 5 months after a 11 month regimen of chemotherapy.Four patients underwent 6 months of chemotherapy,and it was 9,12,and 21 months respectively after stop of chemotherapy.Another patient was still in chemotherapy. Conclusions Nbm is a pre-neoplastic proliferative process with high risk of developing WT.Chemotherapy may reduce the rate of Nbm malignancy.If Nbm is malignant or chemotherapy is invalid,nephron sparing surgery is recommended.

Objective To establish a stable and repeatable experimental partial bladder outlet obstruction(p-BOO)rat model and to figure out the impaction of p-BOO on detrusor biomechanical properties.Methods P-BOO animal model was established by partialligation of the bladder neck of male Wistar rats,a urethra stricture by laying a trochar outside of bladder neck.The rats were divided into sham-operation group,P-BOO 6 weeks group(P-B006W)and P～BOO 12 weeks group(PBOO12W)by time.Cystomety was performed in P-BOO6W and the rats were divided into detrusor instability group(DI)and destrusor stability group(DS)on the basis of destrusor stability.The active contraction of detrusor muscle stripes to Carbachol was recorded with tensile foree transducer.The compliance and maximum volume of bladder,bladder leak point pressure were examined by filling cystometry.Results The bladders of P-BOO animal model demonstrated typical post obstruction alterations after P-BOO.The maximum volume increased significantly in DI group(10.8±3.0)ml,DS group(10.3±1.9)ml and P-B0012W group(9.5±2.3)ml as compared with that in sham-operation group(2.1±0.3)ml(P＜0.05).The bladder leak point pressures were significantly higher in DI group(39.4±7.1)cm H2O,DS group(35.9±6.2)cmH2O and P-B0012W group(48.6±9.5)cm H2O as compared with that in sham-operation group(16.2±2.1)cm H2O(P＜0.05).The bladder compliances were significantly higher in 13I group(0.27±0.08)ml/cm H2O,DS group(0.29±0.05)ml/cm H2O and P-BOO12W group(0.21±0.05)ml/cm H2O as compared with that in sham-operationgroup(0.13±0.03)ml/cm H20(P＜0.05).The detrusor contractile force of DI group was significantly lower than that in the sham-operation group and DS group(P＜0.05).No definite contraction wave was detected in the detrusor muscle of P-BOO12W group(amplitude＜0.05g).Conclusions There are 2 different types of the detrusor contraction after P-BOO:DI group with impaired contraction and conversely DS group with compensatory contraction.The contractility of detrusor muscle will be damaged and even irreversible contractile function incapacitation will occur if the obstruction is not removed.The effect of bladder stability tO bladder compliance is inconspicuous and there is close correlation between bladder compliance and bladder capacity.

Objective To investigate the clinical and genetic characteristics in a patient with 17β-hydroxy-steroid dehydrogenase (17β-HSD) 3 deficiency, regarding its pathophysiology and pathogenesis. Methods Clinical features and laboratory data were analyzed in a pedigree of 17β-HSD3 deficiency. Blood samples from the patient and his parents were collected. HSD17B3 gene was screened for mutations by PCR and subclone sequencing. Results The patient presented with pubertal virilization and gynecomastia. The physical examination showed female external genitalia and testes in inguinal canals. The chromosome karyotype was 46, XY. Serum FSH, LH, dehydroepiandrosterone sulfate, androstenedione and 17-OH-progesterone levels were raised, whereas plasma testosterone was lowered. Sequencing analysis revealed 4 nucleotide deletion (172-175del) of HSD17B3 gene. Conclusion Virilization and gynecomastia in puberty suggest the probability of 17β-HSD deficiency. It may be verified clinically by hCG-stimulating test and confirmed by gene diagnosis.