Pediatric clear cell sarcoma of the kidney with atriocaval thrombus.

Clear cell sarcoma of the kidney (CCSK) is an uncommon neoplasm that accounts for almost 3 % of pediatric renal tumors. Cavoatrial tumor thrombosis is very rare and because of poor response to chemotherapy, invasive surgical interventions such as open heart surgery may be indicated. A 6-year-old girl with CCSK of right kidney was treated with neoadjuvant chemotherapy. According to poor chemosensitivity, surgical intervention was planned. Right atriotomy was done, but intra-atrial part of tumor was very firm and unsuctionable; so the procedure was completed by laparotomy and en bloc resection of tumoral kidney and its cavoatrial extension through a limited venotomy on inferior vena cava. Although radical resection of CCSK with intracaval involvement should be considered as a multidisciplinary approach and intensive care and supports should be provided, atriocaval tumor in growth in CCSK is firm, non friable and non-adherent, and tumor en bloc resection may be possible through a limited venotomy via primary abdominal approach.

Urinary and serum carbohydrate antigen 19-9 as a biomarker in ureteropelvic junction obstruction in children.

PURPOSE: We evaluated the predictive role of serum and urinary carbohydrate antigen 19-9 in the diagnosis and followup of pediatric ureteropelvic junction obstruction. MATERIALS AND METHODS: The study included 27 children with ureteropelvic junction obstruction who underwent pyeloplasty (group 1), and 41 controls consisting of 27 healthy children (group 2) and 14 children with hydrocele/renal cyst (group 3). Serum and voided urine were evaluated for carbohydrate antigen 19-9 in each group. Additionally urine from the affected pelvis and fluid in hydrocele/renal cyst were collected at surgery in groups 1 and 3. Serum and voided urine samples were obtained at 3, 6 and 9 months after pyeloplasty for carbohydrate antigen 19-9 assessment, and were correlated with clinical factors. RESULTS: Preoperative carbohydrate antigen 19-9 level was significantly greater in group 1 than in controls. The best cutoff values for serum and urinary carbohydrate antigen 19-9 were 13.21 U/ml and 30.6 U/ml, respectively, with significantly higher sensitivity and specificity for urinary values. Obstruction release was followed by improvement of renal function together with significant reduction in urinary and serum carbohydrate antigen 19-9 at 3 months. Initial pelvis diameter and renographic function significantly correlated with urinary carbohydrate antigen 19-9. No significant correlation was found regarding serum carbohydrate antigen 19-9. CONCLUSIONS: Voided urine carbohydrate antigen 19-9 is a noninvasive, clinically applicable marker in congenital obstructive nephropathy. The practical implications of these data for diagnosis and long-term followup in ureteropelvic junction obstruction are significant. Our findings suggest that proper decrease in urinary carbohydrate antigen 19-9 after pyeloplasty is predictive of excellent surgical outcomes and resolution of renal damage.

Vesicoureteral reflux and primary bladder neck dysfunction in children: urodynamic evaluation and randomized, double-blind, clinical trial on effect of α-blocker therapy.

PURPOSE: Primary bladder neck dysfunction has been under diagnosed as a treatable cause of vesicoureteral reflux. We evaluated the effect of prazosin administration on vesicoureteral reflux resolution and urodynamic parameters in children with idiopathic primary reflux and primary bladder neck dysfunction. MATERIALS AND METHODS: A total of 62 children (mean ± SD age 7.9 ± 2.4 years) with documented vesicoureteral reflux and urodynamics proved primary bladder neck dysfunction were randomized to receive either 0.025 mg/kg α-blocker (prazosin, 40 patients) or placebo (22) nightly for 1 week with a subsequent increase to 2 divided doses. Patients were followed for 12 months with clinical evaluation and uroflowmetry performed every 2 months, and each patient underwent complete urodynamic study at 6-month intervals. RESULTS: In the placebo group no uroflowmetry or urodynamic parameter changed significantly at 1-year followup. A 60% decrease in reflux grade was observed in the treatment group compared to 17% in the placebo group. Mean maximal detrusor pressure, post-void residual and opening time were significantly decreased in both followup sessions in the prazosin group (p <0.05). Average flow rate improved from 4.30 to 12.80 ml per second at 6 months and to 13.10 ml per second at 12 months (both p <0.05). CONCLUSIONS: Special attention should be given to secondary causes of vesicoureteral reflux (such as primary bladder neck dysfunction, an underdiagnosed entity in children), since conventional treatment will most likely fail if these conditions are not addressed promptly. In this study prazosin was effective therapy for children with vesicoureteral reflux and primary bladder neck dysfunction.

Transurethral autologous myoblast injection for treatment of urinary incontinence in children with classic bladder exstrophy.

PURPOSE: The application of autologous myoblasts is an area of active research that may represent an improved alternative for the treatment of urinary incontinence. In this study we investigated the effectiveness of autologous myoblast injection for the treatment of urinary incontinence in children with classic bladder exstrophy. MATERIALS AND METHODS: Seven boys and 1 girl with persistent urinary incontinence were entered in the study. All children had undergone staged bladder repair and bladder neck reconstruction, and 5 patients had received 1 to 3 transurethral injections of bulking agent. Autologous myoblasts were isolated from abdominal muscle biopsy and cultured before endourethral injection. After the procedure patients underwent pelvic floor electrical stimulation and continued pelvic floor exercises that had been started at least 1 year before injection. The clinical outcomes (based on a 24-hour voiding diary), and cystometric and urodynamic studies were evaluated. Followup ranged from 12 to 18 months (average 15.3). RESULTS: There was a significant, time dependent improvement in urinary continence. At final followup all 7 boys (88% of patients) were socially dry (daytime dryness more than 3 hours), including 3 (38%) who were completely dry. Urodynamic studies revealed an increase in mean bladder capacity (p <0.001), detrusor leak point pressure (p <0.001) and average maximum urinary flow (p <0.01). All 7 boys (vs only 2 patients preoperatively) achieved normal voiding with demonstrable voiding detrusor contraction in the presence of a compliant stable bladder (p <0.05). CONCLUSIONS: Our results suggest that transurethral autologous myoblast injection is a valid option for the treatment of structural urinary incontinence in children with classic bladder exstrophy. However, favorable preoperative urodynamic profiles and postoperative pelvic floor electrical stimulation may have contributed to the outcome in this series.

Effect of Salmeterol-Fluticasone Combination and Tiotropium on Clinical and Physiological Improvement of Bronchial Anthracofibrosis: a Double Blind Randomized, Cross Over, Placebo Controlled, Clinical Trial.

BACKGROUND: Bronchial anthracosis is the black discoloration of bronchial mucosa that exhibits similar manifestations to Chronic Obstructive Pulmonary Disease ( COPD). The etiology of this obstructive lung disease has not been elucidated and standard therapy for this disease has not been introduced in the literature. The objective of this study is to determine the efficacy of the salmeterol-fluticasone inhaler and tiotropium as two safe treatments of obstructive lung disease for the treatment of symptomatic subjects of anthracofibrosis of the lung. MATERIALS AND METHODS: Twenty anthracofibrosis subjects who suffered from dyspnea were enrolled in this three-phase, cross over, placebo-controlled clinical trial. The primary outcome variable was quality of life (evaluated with the CAT questionnaire). Clinical findings and spirometry were the secondary outcome variables. Both of these drugs were delivered by an inhaler and were made identically by the reference manufacturer. Salmeterol-fluticasone was prescribed with a spacer and tiotropium by its special device, and the method of utilization was taught to the patients. RESULTS: Twenty anthracofibrosis subjects were enrolled in this three-phase, five-month course of treatment with either salmeterol-fluticasone or tiotropium inhalers. The response to therapy was not good; neither for salmeterol-fluticasone nor for tiotropium in the short course of the treatment. However, the overall results of 5 months of therapy with both of the drugs have shown improvement in 57% of the subjects. The most prominent results were found in the CAT score [25.1±5.54 before the trial, which decreased to 19.2±5.14 (Z score=2.7, P=0.007)] and clinical findings especially sputum, chest pain, and wheezing (81, 94 and 92% before the trial and 50, 56, 54% after the trial, respectively). Neither clinical findings nor spirometry was able to predict a good response to salmeterol-fluticasone or tiotropium. CONCLUSION: The combination of salmeterol-fluticasone and tiotropium inhaler was able to improve the clinical findings of symptomatic anthracofibrosis patients.

Evaluation of the Effect of Meatal Stenosis on the Urinary Tract by using Ultrasonography.

PURPOSE: Circumcision is one of the oldest surgical procedures that originated for religious purposes. Circumcision in infancy is a common procedure in our country and secondary meatal stenosis due to circumcision is a common complication. The aim of our study is to determine the effect of meatal stenosis on the lower and upperurinary tract of circumcised boys by using ultrasonography. MATERIALS AND METHODS: In this cross-sectional study, we enrolled 87 circumcised boys between 4 to 8 years old with severe meatal stenosis. Clinical findings of our subjects were gathered by a checklist that included: thin stream of urine, upward urine stream deviation, infrequent voiding, urinary tract infections, voiding dysfunction, and urge incontinency. In lab data analysis, complete blood cell count (CBC), urine analysis, urine culture, blood urea nitrogen (BUN), and plasma creatinine level were evaluated. Ultrasonography detected hydronephrosis, hydroureter, bladder wall thickening in a full and empty bladder, bladder volume, and residual urine volume. RESULT: Narrowing of urine stream is commonly seen (about 54%) among patients with severe meatal stenosis, and similarly in sonographic evaluations the most common symptoms among patients was thickening of the bladder wall that increased in an empty and a full bladder (about 82%). CONCLUSION: The author of this study recommends performing long-term follow up after circumcision and ultrasonographyto detect meatal stenosis before permanent renal damage occurs.

Effect of Borago Officinalis Extract on Moderate Persistent Asthma: A Phase two Randomized, Double Blind, Placebo-Controlled Clinical Trial.

BACKGROUND: Borago officinalis and its derivatives are used in folk medicine to treat asthma because of its special effect on allergic disorders. It suppresses the tumor necrosis factor-alpha (TNF-alpha) and delivers gamma-linolenic acid. The objective of this clinical trial was to determine the effect of Borago officinalis on clinical and physiological findings in moderate persistent asthma. MATERIALS AND METHODS: This prospective, randomized, double blind, placebo-controlled, clinical trial was conducted on patients aged 15-90 years with moderate asthma and forced expiratory volume in one second (FEV1) of 60-79% of predicted who presented to a sub-specialty clinic of pulmonary medicine. We randomly allocated subjects to receive either Borago extract (5 mL three times a day) or a matched placebo for one month. The primary outcome was the asthma control test (ACT) score and fractional exhaled nitric oxide (FENO) test. Secondary outcomes included clinical findings, spirometry, and sputum cytology including inflammatory cells. RESULTS: Thirty-eight subjects with a mean age of 46.8±15.3 years and mean duration of asthma of 71±103 months were enrolled in our study. Cough, dyspnea, wheezing, nocturnal symptoms, and airway hyper-responsiveness reduced significantly in the Borago group after the treatment and ACT scores improved significantly (10.8±5.26 before and 15.4±5.12 after the trial). Flare up of asthma and emergency department visits in the Borago group also decreased significantly (3.6±2.33 to 2±1.86 flare ups per month and 0.62±0.9 to 0.05±0.23 for emergency department visits per month). Physiological parameters including spirometry, FENO, and sputum cytology including eosinophil and neutrophil did not change significantly. CONCLUSION: Borago improved the clinical findings of asthma, but it was not able to suppress the inflammation involved in asthma.

Surgical results of anderson-hynes dismembered pyeloplasty without internal drainage in adults with ureteroplevic junction obstruction.

BACKGROUND: Anderson-Hynes dismembered pyeloplasty is the gold standard therapeutic approach to ureteropelvic junction obstruction (UPJO). Use of a drainage method to protect the suture line from leakage is a matter of controversy. OBJECTIVES: We have compared the surgical outcome of Anderson-Hynes dismembered pyeloplasty for UPJO repair, with or without internal stenting. PATIENTS AND METHODS: Eighty-two patients with UPJO were evaluated from 1996 to 2002. Complicated or emergent cases were excluded. Classic standard dismembered pyeloplasty was performed. Internal drainage, with a double j catheter, was performed in several patients, randomly. Another drain was also placed in the retroperitoneal space. The follow-up of patients was planned weekly, with patient visits and urine analysis and intravenous pyelography (IVP) and diethylene-triamine-pentaacetate (DTPA) scan after one month. RESULTS: The study group consisted of 51 male and 31 female patients, who were mostly in the age range of 20 - 40 years. Comparing the two techniques of pyeloplasty with or without internal drainage, there was no significant difference between groups regarding extravasation and anastomosis complications, such as leakage, stenosis, urinoma formation or evidence of obstruction on postoperative IVP or DTPA scan. However, a higher incidence of catheter related urinary symptoms and flank pain was reported among those with internal stent. CONCLUSIONS: Pyeloplasty, with adequate spatulation, hemostasis and a watertight anastomosis, represents the mainstay of successful pyeloplasty and there may be no significant benefit for urethral stenting, especially in non-complicated cases.

Vesicorectal fistula detected on direct radionuclide cystography--importance of fecal matter imaging.

We report an 11 year old male patient with the history of imperforate anus, which was repaired surgically 4 years ago. He has been complaining of intermittent passing of urine into the rectum recently. The vesicorectal fistula in this patient was proven by imaging of the fecal matter post direct radionuclide cystography study. Our case showed that nuclear medicine imaging can be extended to unanimated objects such as patients' excrements or fluids with important diagnostic yields.

An antenatally diagnosed rhabdomyosarcoma of the bladder treated without extensive surgery.

BACKGROUND: A female fetus was followed up from week 30 of gestation after bilateral hydroureteronephrosis and a large pelvic mass were detected on fetal imaging. At birth, the patient had high respiratory rate, a palpable bladder up to the umbilicus and a large pelvic mass, which compressed the anorectal wall. INVESTIGATIONS: Fetal ultrasonography, fetal magnetic resonance urography, pelvic examination, full hematologic investigation, blood culture, abdominal and pelvic ultrasonography, voiding cystourethrography, abdominal and pelvic spiral CT, measurement of serum tumor marker levels, pathologic examination of the excised specimen, pelvic MRI. DIAGNOSIS: Botryoid subtype of rhabdomyosarcoma occupying most of the bladder and protruding through the urethra, with bilateral hydroureteronephrosis. MANAGEMENT: Catheterization on the second day of life improved the patient's renal function, but her general condition remained unstable and surgical intervention was deferred. On the fourth day, gross hematuria and decreased urinary output were observed, which rapidly progressed to anuria, and she underwent transurethral resection of the protruding part of the tumor and bilateral cutaneous ureterostomy. Subsequently, she received chemotherapy with vincristine, actinomycin D and ifosfamide, and was followed up with serial imaging. At 18 months, MRI showed no evidence of residual tumor, and cystoscopic biopsy confirmed the absence of viable tumor; chemotherapy was stopped. She had no sign of recurrence 24 months after ending chemotherapy.

Application of magnetic resonance urography in diagnosis of congenital urogenital anomalies in children.

Magnetic resonance urography (MRU) has become a useful adjuvant in evaluating urogenital anomalies. In present study, we evaluated the ability of MRU in diagnosis of different congenital urogenital anomalies when the results of conventional imaging modalities were inconclusive. A total of 90 children were included in this series. The children were evaluated with T2-weighted and contrast-enhanced T1-weighted MRU sequences. The results were compared with findings obtained with ultrasonography, intravenous urography, renal nuclide scan, and voiding cystourethrography. MRU was requested in these children because conventional imaging modalities were equivocal or a co-existing urogenital anomaly was suspected. Only those cases that underwent surgery were included in this study and the surgical findings were set as the reference standard in statistical evaluation. The records of 61 boys with mean (range) age of 2.3 years (2 months-12 years) and 29 girls with mean (range) age of 3.3 years (3 months-12 years) were reviewed. The final diagnosis was ureteropelvic junction obstruction (n = 25), vesicoureteral junction obstruction (n = 16), ureterocele (n = 19), ectopic kidney (n = 11), posterior urethral valve (n = 17), and polycystic kidney (n = 2). The overall sensitivity of MRU, intravenous urography, renal nuclide scan, ultrasonography, and voiding cystourethrography in diagnosis of the aforementioned anomalies were 86, 63, 50, 44, and 41%, respectively. MRU was much more sensitive than other imaging modalities in diagnosis of end-ureteral dilation (100%) and ureterocele (89%). MRU provides a reliable noninvasive technique for imaging of the congenital anomalies in the urinary tract of children with T2-weighted MRU sequences providing unenhanced static-water images of the urinary tract as well as depicting adjacent soft-tissue lesions, and T1-weighted MRU technique imitating conventional intravenous urography. Both MRU sequences can be combined for a comprehensive examination of the urinary tract.

Infantile bladder rupture during voiding cystourethrography.

Bladder rupture is rare during infancy and most of reported cases had urethral obstruction or neurogenic bladder. We report two cases of infantile bladder rupture during voiding cystourethrography (VCUG). This report reinforces the criteria for proper VCUG imaging procedure. Consideration of expected bladder volume for body weight, and close monitoring of bladder pressure and injection speed could prevent such complications.

Infantile bladder rupture during voiding cystourethrography

Bladder rupture is rare during infancy and most of reported cases had urethral obstruction or neurogenic bladder. We report two cases of infantile bladder rupture during voiding cystourethrography (VCUG). This report reinforces the criteria for proper VCUG imaging procedure. Consideration of expected bladder volume for body weight, and close monitoring of bladder pressure and injection speed could prevent such complications.