A Genetics Study in the Foreskin of Boys with Hypospadias.

Introduction: Hypospadias is a malformation of the genitourinary system in males, characterized by the placement of the urethral opening in the ventral surface of the penis. Although controversies continue about etiology, endocrine disrupting chemicals that disrupt normal endocrine signaling at the receptor or signal transduction level are thought to play an essential role in etiology. This study aimed to investigate the receptor gene expressions of the sex hormones and FGFR2, HOXA13, and TGFB1, which are considered to play an essential role in developing hypospadias. Methods: The samples from the foreskin of 26 patients with hypospadias and 26 healthy children who underwent circumcision operations were collected. ESR1, AR, FGFR2, HOXA13, and TGFB gene expressions were investigated by real-time PCR in samples obtained during surgery. Results: In the hypospadias group, ESR1 expression was increased (p = 0.013), and AR and FGFR2 expressions were decreased, which were found to be statistically significant (p = 0.027 and p = 0.003, respectively). There was no statistically significant difference between hypospadias and control groups in TGFBand HOXA13expression levels (p > 0.05). Discussion: The results suggest that sex hormone receptors and FGFR2 may play an essential role in developing male external genital structures at the gene level. The defects in the expression of these genes can contribute to understanding the development of hypospadias.

Dorsal Versus Ventral Dartos Flap to Prevent Fistula Formation in Tubularized Incised Plate Urethroplasty for Hypospadias.

PURPOSE: The purpose of this study is to evaluate the results of two different flap procedures for prevention of urethrocutaneous fistula in hypospadias patients undergoing tubularized incised plate urethroplasty . PATIENTS AND METHODS: We retrospectively reviewed 89 patients who underwent hypospadias repair. The standard technique of tubularized incised plate urethroplasty was used. There were 45 patients in Group 1 and 44 patients in Group 2, in which ventral and dorsal dartos flaps were used to cover the neourethra respectively. Surgical complications were assessed as the main outcomes. The results were analyzed with Chi-square and Mann-whitney u tests. RESULTS: There was no significiant difference between the groups in terms of age and meatus location. We observed postoperative surgical complications in 15 (33.3 %) patients in Group 1 and in 4 (9.1 %) patients in Group 2. The complications noted in the Group 1 were urethrocutaneous fistula in 10 patients ( 22.2 %) and meatal stenosis in 5 patients ( 11.1 %). In Group 2, fistula was observed in 2 patients (4.6 %) and stenosis in again 2 patients (4.6 %). Urethrocutaneous fistulas occurred statistically more frequently when ventral based dartos flaps were used (P < .05). CONCLUSION: Several flap procedures and their modifications have been suggested to avoid fistula formation. Within these procedures, dartos flaps are reported to be very useful for primary distal or proximal hypospadias repair and reoperations. In this study, we concluded that ; vascularized dorsal preputial dartos flap procedure is safe and more effective than ventral based flap in prevention of fistula formation.

Can serum Neutrophil Gelatinase Associated Lipocalin and Kidney Injury Molecule-1 help in decision making for surgery in antenatally dedected hydronephrosis.

INTRODUCTION: Congenital obstructive uropathies are among leading reasons for renal failure in children. Answers to questions such as what the critical threshold of obstruction is or which degree of obstruction disrupts the development of the kidney still remain unclear. Several biomarkers such as Kidney Injury Molecule 1 (KIM-1) and Neutrophil Gelatinase Associated Lipocalin (NGAL) may help clinicians in the clinical evaluation and appropriate planning of the disease. OBJECTIVE: This study aimed to investigate whether serum and urinary KIM-1 and NGAL levels contribute to conventional methods in decision-making for surgery in the postnatal period of infants with antenatal hydronephrosis. STUDY DESIGN: 34 patients with the diagnosis of antenatal hydronephrosis were evaluated prospectively. Renal pelvis diameters of all patients were above 10 mm in the ultrasonography (USG). Patients underwent diuretic renal scintigraphy after neonatal period. Patients were divided into two groups as surgery or follow-up based on USG and scintigraphy findings. Blood and urine samples were collected at first visits in both groups and again at the 3. Postoperative month in the surgery group. Serum and urinary NGAL and KIM-1 levels were measured by ELISA method. Study data were compared through the Mann-Whitney U and Wilcoxon Signed-Ranks test. RESULTS: There were 10 patients in the surgery group and 24 patients in the follow-up group. The age and gender did not differ between the groups. The surgery group had significantly higher median serum NGAL values (259.2 ng/mL) than that in the follow-up group (46.8 ng/mL, p = 0.028). The postoperative reduction of the median serum NGAL to 68.1 ng/mL compared to preoperative level was also found to be significant (p = 0.037) in the surgery group. Between the groups and within the surgery group no statistically significant difference was detected in terms of median urinary NGAL, and serum and urine KIM-1 levels. DISCUSSION: USG and renal scintigraphy are frequently used in determining whether patients with antenatal hydronephrosis need surgical intervention in the postnatal period. Several new biomarkers might help clinicians in decision making for surgery. KIM-1 and NGAL levels can be measured both in urine and serum. To our knowledge, this is the only study where serum NGAL and KIM-1 levels were measured in patients with antenatal diagnosis. Small sample size, lack of long term findings and control group are limitations of our study. CONCLUSION: Serum NGAL levels of patients with antenatal hydronephrosis may help in decision making on the surgical intervention.

Effective predictors for surgical decision in antenatal hydronephrosis: a prospective multiparameter analysis.

OBJECTIVE: The majority of antenatal hydronephrosis disappears spontaneously. In our study, we have investigated the effective predictors for surgical decision in antenatal hydronephrosis. MATERIAL AND METHODS: Forty-four patients found to have renal pelvic anteroposterior (AP) diameter more than 10-mm on antenatal ultrasonograpy were followed after birth. The study groups were divided into two groups: follow-up and surgery group. On follow-up, longitudinal diameter of the kidney, parenchymal thickness of the kidney, AP diameter of renal pelvis, AP diameter of middle calyces on ultrasonograpy, and differential renal function, 20th minute clearance, half-life of radionuclide tracer (T1/2), Tmax, Tmax-T1/2, normalised residual activity (NORA) on diuretic renography were evaluated. Ultrasonograpy and diuretic renography measurements were compared in patients whose hydronephrosis resolve or proceeded to surgery. RESULTS: Forty-four patients were diagnosed as antenatal hydronephrosis, spontaneous resolution occurred in 23 (52%), and surgery was performed in 21 patients (48%). Mean age at operation was 8.5±6.5 months (3-24 months). Mean AP diameter of renal pelvis was 29.5±14.2 mm for surgery group, 13.6±4.2 mm for follow-up group (p<0.001). Univariate analyses showed significance for all ultrasonographic measurements and only the differential renal function by diuretic renography. Multivariate logistic regression analysis showed significance for AP diameter of renal pelvis (odds ratio 1.37; 95% Cl 1.13-1.66), and differential renal function (odds ratio 1.14; 95% Cl 1.01-1.29). CONCLUSION: AP diameter of renal pelvis and differential renal function were the most effective parameters for surgical decision. These parameters can be used for appropriate management of antenatal hydronephrosis.

Effectiveness of endoscopic balloon dilatation in grade 2a and 2b esophageal burns in children.

PURPOSE: To evaluate the predictability of the initial endoscopic evaluation of the effectiveness of endoscopic balloon dilatation (EBD) in childhood esophageal strictures caused by corrosive ingestion. MATERIAL AND METHODS: Medical records of 635 endoscopies caused by corrosive ingestion between January 2000 and December 2015 in children between the ages of 0 and 18 years were retrospectively analyzed. Among them, five children with grade 2a and 15 with grade 2 b who developed esophageal strictures were evaluated for the effectiveness of endoscopic balloon dilatation. RESULTS: The stricture rate was 5/136 (3.6%) in grade 2a and 17/25 (68%) in grade 2 b esophageal burns. Strictures with grade 2a burn had seven (1-10) EBD sessions, and grade 2 b had 8.8 (1-30) EBD sessions. For grade 2a burns, the treatment period was 15 months and 18.8 months for grade 2 b burns. Three patients with grade 2 b and two patients with grade 2a are still on the EBD program. CONCLUSION: Initial endoscopy for caustic ingestion and esophageal injury grading may help to provide healthcare givers with information about future stricture formation and management.

A Diagnostic Dilemma for the Pediatrician: Radiolucent Tracheobronchial Foreign Body.

BACKGROUND: The purpose of this study is to determine the role of clinical history, physical examinations, and radiological findings in the evaluation of patients with suspected radiolucent foreign body aspiration. METHODS: The medical records of 236 children (under the age of 18 years), on whom a rigid bronchoscopy was performed between 1999 and 2015 because of suspected radiolucent foreign body aspiration, were analyzed retrospectively. Sensitivity, specificity, positive and negative predictive values of clinical history, physical examinations, and radiological findings were evaluated. RESULTS: In 71.1% of all cases, the children were under the age of 3 years. The bronchoscopy showed the presence of a foreign body in 52.9% of cases, with the locations of the foreign bodies being as follows: (1) right main bronchus, 47.2%; (2) left main bronchus, 36.0%; (3) trachea, 11.2%; (4) both bronchi, 5.6%. Organic foreign bodies were found in 78% of the patients, whereas inorganic foreign bodies were detected in 22% of the patients. The sensitivity and specificity of clinical history, physical examinations, and radiological findings were 98.4% and 54.9%, 47.2% and 74.7%, and 35.2% and 92.7%, respectively. CONCLUSION: Tracheobronchial foreign body aspirations usually occur prior to the age of 3 years, with the most frequently aspirated foreign bodies being food or items of a radiolucent nature. Clinical history, physical examinations, and radiological findings are not able to detect the presence of a radiolucent foreign body aspiration in children. Therefore, a bronchoscopy should be performed on children in whom a choking event has been witnessed, even in cases of normal radiological and clinical findings.

The outcomes of two different bulking agents (dextranomer hyaluronic acid copolymer and polyacrylate-polyalcohol copolymer) in the treatment of primary vesico-ureteral reflux.

PURPOSE: Subureteral injection of bulking agents in the endoscopic treatment of vesicoureteral reflux is widely accepted therapy with high success rates. Although the grade of vesicoureteric reflux and experience of surgeon is the mainstay of this success, the characteristics of augmenting substances may have an effect particularly in the long term. In this retrospective study, we aimed to evaluate the clinical outcomes of the endoscopic treatment of vesicoureteric reflux (VUR) with two different bulking agents: Dextranomer/hyaluronic acid copolymer (Dx/HA) and Polyacrylate polyalcohol copolymer (PPC). MATERIALS AND METHODS: A total 80 patients (49 girls and 31 boys) aged 1-12 years (mean age 5.3 years) underwent endoscopic subureteral injection for correction of VUR last six years. The patients were assigned to two groups: subureteral injections of Dx/HA (45 patients and 57 ureters) and PPC (35 patients and 45 ureters). VUR was grade II in 27 ureters, grade III in 35, grade IV in 22 and grade V in 18 ureters. RESULTS: VUR was resolved in 38 (66.6%) of 57 ureters and this equates to VUR correction in 33 (73.3%) of the 45 patients in Dx/HA group. In PPC group, overall success rate was 88.8% (of 40 in 45 ureters). Thus, Thus, this equates to VUR correction in 31 (88.5%) of the 35 patients. CONCLUSIONS: Our short term data show that two different bulking agent injections provide a high level of reflux resolution and this study revealed that success rate of PPC was significantly higher than Dx/HA with less material.

The outcomes of two different bulking agents (dextranomer hyaluronic acid copolymer and polyacrylate-polyalcohol copolymer) in the treatment of primary vesico-ureteral reflux

ABSTRACT Purpose Subureteral injection of bulking agents in the endoscopic treatment of vesicoureteral reflux is widely accepted therapy with high success rates. Although the grade of vesicoureteric reflux and experience of surgeon is the mainstay of this success, the characteristics of augmenting substances may have an effect particularly in the long term. In this retrospective study, we aimed to evaluate the clinical outcomes of the endoscopic treatment of vesicoureteric reflux (VUR) with two different bulking agents: Dextranomer/hyaluronic acid copolymer (Dx/HA) and Polyacrylate polyalcohol copolymer (PPC). Materials and Methods A total 80 patients (49 girls and 31 boys) aged 1-12 years (mean age 5.3 years) underwent endoscopic subureteral injection for correction of VUR last six years. The patients were assigned to two groups: subureteral injections of Dx/HA (45 patients and 57 ureters) and PPC (35 patients and 45 ureters). VUR was grade II in 27 ureters, grade III in 35, grade IV in 22 and grade V in 18 ureters. Results VUR was resolved in 38 (66.6%) of 57 ureters and this equates to VUR correction in 33 (73.3%) of the 45 patients in Dx/HA group. In PPC group, overall success rate was 88.8% (of 40 in 45 ureters). Thus, Thus, this equates to VUR correction in 31 (88.5%) of the 35 patients. Conclusions Our short term data show that two different bulking agent injections provide a high level of reflux resolution and this study revealed that success rate of PPC was significantly higher than Dx/HA with less material.

Unusual complication of a urinary stone in a child: spontaneous rupture of the renal pelvis with the migration of calculus into the retroperitoneum.

Spontaneous perforation of the renal pelvis and extravasation of urine into the perinephric space is an extremely rare condition in childhood. It is mostly related to underlying congenital urinary tract abnormalities and caused by urolithiasis or infection in the setting of urinary obstruction. Here, we report a case of an 18-month-old female patient with rupture of the renal pelvis by an extruded kidney stone and its management.

Intestinal ischemia-reperfusion induced diaphragm contractility dysfunction: Electrophysiological and ultrastructural study in a neonatal rat model.

AIM: To evaluate the remote effect of intestinal ischemia reperfusion (IR) injury mediated by tumor necrosis factor alpha (TNF-α) on diaphragm contractility functions and whether administration of NAC may counteract the possible detrimental effects in an experimental neonatal rat model. METHODS: 40 Wistar rat pups were randomized into four groups; ten animals in each. Intestinal ischemia was conducted by obstructing mesentery of intestines by a silk loop. In the control group; only laparotomy was performed. After 1h ischemia, reperfusion was conducted for 1h in 1h group, 24h for 24h group and 24h for 24h+NAC group but administration of NAC (150mg/kg/day) intraperitoneally twice a day was performed. Inflammatory response was evaluated by tissue TNF-α level and contractility functions by mechanic activity studies of the diaphragm. Electrophysiology of the diaphragm and the phrenic nerve was conducted to determine neuropathy or myopathy and transmission electron microscopy was performed to evaluate ultrastructural changes in the phrenic nerve. RESULTS: Diaphragm tissue TNF-α level significantly increased in 1h and 24h groups (P=0.004, P=0.0001; respectively). Diaphragm mechanic activation force and duration significantly decreased at 1h and 24h (P=0.004, P=0.02 and P=0.0001, P=0.0001; respectively). NAC administration significantly prevented decrease in the maximal contraction and the duration (P<0.001). Phrenic nerve compound action potential (CMAP) amplitude significantly decreased in 1h group (P<0.0001) and NAC administration significantly prevented this decrease when compared with 24h group (P<0.001). In diaphragmatic needle electromyography, the duration of motor unit potentials (MUP) was prolonged significantly when compared with control group. Contractility and electrophysiological studies were indicating primarily neuropathy in diaphragm dysfunction. Histopathology revealed axonal and myelin degeneration in the 1h and 24h group, but less injury in the NAC administered group. CONCLUSIONS: Intestinal IR induced elevation of TNF-α level in the diaphragm. Impairment in the diaphragm contractility and neuropathic changes in the phrenic nerve occurred even in the first hour of reperfusion. NAC administration prevented these detrimental effects.

Dangerous Toys for Teenagers: Air Weapons.

INTRODUCTION: Air weapons are used for sport, hunting, firearm training or just for fun, especially by teenagers, worldwide. These weapons are generally regarded as toys, and injuries from these weapons are thought to be harmless by users, parents and even officials. Improvements in weapons technology make the penetration power of these toys similar to conventional hand guns. To increase awareness about the serious injuries associated with these guns. CASE PRESENTATION: The medical records of four teenage boys shot by air weapons between January 2012 and January 2013 in Mersin, Turkey, were retrospectively reviewed in this study. Of the four boys, two needed prompt thoracic intervention due to pneumo/hemothorax, one needed urgent abdominal exploration due to pneumoperitoneum and bleeding from the spleen and one was treated conservatively. CONCLUSIONS: Air weapons can cause serious injuries among children. Increased public awareness, limitations to their usage and strict legislation are needed to protect children.

Congenital laryngomucocele: a rare cause of airway obstruction in a newborn.

Congenital laryngoceles are defined as cystic dilatation of laryngeal saccules and are an extremely rare cause of newborn respiratory distress. A laryngomucocele occurs when the neck of the laryngocele gets obstructed and fills with the mucoid secretions of the saccule. It may cause stridor, respiratory distress, and severe airway obstruction in the narrow airway of a newborn and necessitates urgent surgical intervention. There is only 1 case of congenital laryngomucocele reported in an autopsy examination in the English literature, and here we report the first living congenital laryngomucocele case and discuss the clinical approach.

A rare cause of childhood renal cysts: Xp11.2 translocation renal cell carcinoma.

Pediatric renal cysts are rare, usually asymptomatic and incidentally detected in children. Cyst associated renal cell carcinoma (RCC) or cystic RCC is extremely rare in children. Bosniak classification system has been accepted for the management of cystic renal masses. Xp11.2 translocation RCC is a recently classified distinct subtype and usually affects children and adolescents. We report the case of a 10-year-old girl with Xp11.2 translocation RCC from a cyst of the right kidney.

Challenging diagnosis between intussusception and necrotizing enterocolitis in premature infants.

Although necrotizing enterocolitis (NEC) is a frequently encountered entity in premature infants in the neonatal intensive care unit, intussusception is extremely rare. Abdominal distension, bilious/non-bilious gastric residuals and bloody stool are the common clinical findings of both entities. Here we present three cases of intussusception misdiagnosed as NEC, two of which were complicated with intestinal perforation. Similar clinical findings of NEC and intussusception leads to misdiagnosis and delay in treatment, particularly in premature infants with intussusception.

Pyocalycosis: a report of two cases of complicated hydrocalycosis.

Hydrocalycosis is defined as cystic dilatation of a major calyx with a demonstrable connection to the renal pelvis and an epithelial lining of the cyst wall. Although this condition has long been known, there are no sufficient data concerning this pathology in the literature. In this study, we present two complicated hydrocalycosis--'pyocalycosis'--and discussed the therapeutic approaches.

Relationship of bladder dysfunction with upper urinary tract deterioration in cerebral palsy.

Although lower urinary tract dysfunction (LUTD) in patients with cerebral palsy (CP) has been previously documented by clinical observations and urodynamic tests, its correlation with upper urinary tract deterioration (UUTD) has not been demonstrated. This paper documents symptoms and urodynamic findings of LUTD and their relationship with UUTD in 33 children with CP. By sonography, 4 of these children were found to have UUTD. Age was found to correlate with UUTD, but gender difference and mental or motor functions did not. When comparing urinary symptoms with UUTD, incontinence (n = 31) did not correlate, but on the other hand symptoms of detrusor sphincter dyssynergia (interrupted voiding, urinary retention, hesitancy; n = 5) and culture proven febrile urinary tract infections (n = 4) did. Abnormal urodynamics findings were not diagnostic. We conclude that, apart from incontinence, dysfunctional voiding symptoms and febrile urinary tract infections are valuable indicators of UUTD.

Relationships among vesicoureteric reflux, urinary tract infection and renal injury in children with non-neurogenic lower urinary tract dysfunction.

OBJECTIVE: To determine the relationship between vesicoureteric reflux (VUR), urinary tract infection (UTI), renal damage and the pattern of non-neurogenic lower urinary tract dysfunction (LUTD), and to reveal the possible risk factors for renal damage in children with LUTD. METHODS: For the years 2004-2010, demographic, clinical, laboratory and urodynamic study reports of children with LUTD were retrospectively reviewed. RESULTS: Of 96 patients, there were diagnosed 70 with overactive bladder (OAB), 8 pure dysfunctional voiding (DV) and 18 OAB plus DV. The rate of VUR, UTI and renal damage in patients with OAB plus DV and pure DV was higher than in patients with OAB alone. VUR was significantly higher among the patients who had UTI. Renal scarring was detected in 25 patients, of whom 78% had OAB plus DV and 75% DV. The presence of VUR was associated with a significant increase in the rate of renal damage, and dilating reflux caused significantly greater damage compared to non-dilating reflux. CONCLUSION: OAB plus DV and DV are major risk factors for VUR, UTI and renal damage. The presence of VUR in children with LUTD plays an important role with regard to UTI and renal damage, with dilating VUR a major risk factor associated with renal damage.

Pelvic floor tonicity affects urodynamic measurements in children with myelomeningocele.

OBJECTIVE: In a cystometry procedure in a child with myelomeningocele (MMC), a pressure increase in the abdominal pressure (P (abd)) tracing was detected during filling. This pressure alteration was not related to other known events (straining, talking, rectal contractions). This study was conducted to investigate this phenomenon. MATERIAL AND METHODS: Forty-three children with MMC were enrolled in the study. A slow and gradual pressure increase associated with the bladder filling was sought in the P (abd) tracings. End filling and initial P (abd) gradient more than 3 cm H(2)O were considered as increased P (abd). If the defined pressure event occurs, the bladder was evacuated for verifying the filling-pressure relation. Age, gender, study position, pelvic floor tonicity and cystometric capacity were correlated with the pressure alteration. RESULTS: P (abd) increase was noted in 18 (41.8%) children. The mean P (abd) gradient between end and initial filling was 4.78 ± 1.63 cm H(2)O in these children. No statistically significant difference was noted for age, gender and study position. Statistically significant differences were noted with decreased pelvic floor tonicity and high values of cystometric capacity (p = 0.003 and p < 0.001, respectively). CONCLUSIONS: The pressure increase is thought to be a consequence of a posterior positional change in the bladder during filling die to decreased pelvic floor support in MMC. This pressure alteration was more obvious with increased bladder capacity. Urodynamic studies of children with MMC should be carefully evaluated for the presence of this phenomenon to prevent low measurement of the detrusor pressure, compliance and detrusor leak point pressure values.

Testicular torsion in the left inguinal canal in a patient with inguinal hernia: a difficult case to diagnose.

A 8 month old boy suffering from inconsolable cry and tenderness presented to our hospital. Ten hours had passed from the onset of his symptoms. Physical examination showed a tender mass on the left groin. Patient was evaluated with Doppler ultrasound and Technetium-99m pertechnetate testicular scintigraphy Differential diagnosis of torsion and inflammatory disease could not be made by adjunctive tests. The patient underwent surgery based on clinical findings and necrotic inguinal left gonad was shown. In this study, we discussed the scintigraphic pattern in a patient with torsion in undescended inguinal testicle Conflict of interest:None declared.

Pyeloureterostomy in the management of the lower pole pelvi-ureteric junction obstruction in incomplete duplicated systems.

OBJECTIVES: To report our experience with the pyeloureterostomy (PU) for the treatment of the lower pole PUJO in incomplete duplex systems. The combination of the duplicated collecting system and pelviureteric junction obstruction (PUJO) is a rare association and infrequently reported. Surgical treatment can be challenging in such cases. METHODS: We retrospectively reviewed the medical data of the patients who had surgery from 2001 to 2009, with a diagnosis of PUJO of the lower pole moiety in incomplete duplex system. Demographic, diagnostic, and procedural data were recorded. RESULTS: Seven patients were identified with the lower pole PUJO associated with incomplete duplex systems. Their median age was 49 months (range 2-108 months). Prenatal hydronephrosis was detected in 3 patients, and 4 had a febrile urinary tract infection. PU was performed in 6 patients because of short ureteral length between the ureteropelvic junction and junction of lower and upper pole ureters. One patient was treated with the dismembered pyeloplasty because of sufficient ureteral length of the lower pole. No complications were detected during 14 months of follow-up. CONCLUSIONS: The management of the lower pole PUJO in incomplete duplex systems should be individualized for every patient. PU is a good surgical option in the management of the lower pole PUJO associated with incomplete ureteral duplication.