A very rare syndrome and its rare urological complication: Incomplet bladder duplication in Robinow syndrome.

OBJECTIVES: Robinow syndrome is a very rare syndrome characterized by short stature, extremity deformities, costovertebral abnormalities, renal/external genital malformations, and fetal facial appearance. It might be inherited by either autosomal dominant or severe recessive form. Diagnosis is generally established by the aid of genetic mutation and phenotypic findings. The urogenital component of the disease frequently presents with microgenitalia such as micropenis and/or cryptorchidism. METHODS: Herein, a four-year-old boy with Robinow syndrome accompanied by incomplete bladder duplication is discussed. RESULTS: The duplication in the bladder was screened by cystoscopy and corrective surgery was performed. CONCLUSIONS: This rare manifestation is the first for urological findings of Robinow syndrome in literature.

Bladder augmentation in exstrophy vesicae: Long-term results of a single experienced center.

BACKGROUND: In this study, we present our long-term treatment outcomes of bladder augmentation (BA), bladder neck closure, and Mitrofanoff clean intermittent catheterization (CIC) in managing bladder exstrophy (BE). METHODS: This was a retrospective medical records' review of 33 children diagnosed as born with BE, followed up at a tertiary pediatric urology clinic from 1988 to 2020. Outcomes such as surgical interventions, presence of renal calculi, hydronephrosis, and continence status were extracted. RESULTS: The median follow-up of the group was 18.2 (4-26) years. Urinary system stones developed in 10 (30.3%) cases about 8.9 years after BA. Stone development was two times more common in patients who underwent colocystoplasty (33.3%) than those who underwent ileocytoplasty (16.6%). The state of continence of the group was satisfactory in 26 (78.7%; excellent in 23; good in 3 cases) and unsatisfactory (wet) in 6 (18.1%) cases. At the last visit, ultrasonography revealed no hydronephrosis in 23 (69.6%) patients, and the voiding cystourethrogram demonstrated low-grade vesicoureteral reflux in 10 (30.3%) and high-grade vesicoureteral reflux in 2 (6%) patients. CONCLUSIONS: An elaborated plan of surgical reconstruction for classic BE can lead to satisfactory long-term urinary continence in most patients. Ultimate predictors of outcome in BE repair are difficult to ascertain. Consistently, BA, bladder neck closure, and Mitrofanoff CIC continue to stand out at a critical point in the management of those patients with classic BE. Our study demonstrated that augmentation is required to achieve acceptable dryness with high satisfactory dryness rates in BE.

A very rare syndrome and its rare urological complication: Incomplet bladder duplication in Robinow syndrome

Objectives: Robinow syndrome is a very rare syndrome characterized by short stature, extremity deformities, costovertebralabnormalities, renal/external genital malformations, and fetal facial appearance. It might be inherited by either autosomaldominant or severe recessive form. Diagnosis is generally established by the aid of genetic mutation and phenotypic findings.The urogenital component of the disease frequently presents with microgenitalia such as micropenis and/or cryptorchidism.Methods: Herein, a four-year-old boy with Robinow syndrome accompanied by incomplete bladder duplication is discussed.Results: The duplication in the bladder was screened by cystoscopy and corrective surgery was performed.Conclusions: This rare manifestation is the first for urological findings of Robinow syndrome in literature. (AU)

COVID-19 complicated with acute renal failure due to mycotic bezoars in two children. / COVID-19 complicado con fallo renal agudo debido a bezoar micótico en dos niños.

OBJECTIVES: Patients with viral infection areat risk for secondary infections and/or co-infections. Ourstudy aims to describe coronavirus-disease-19 (COVID-19) associated with acute renal failure (ARF) secondary to fungalbezoar in urinary tract in two patients. METHODS: A chart review of two patients with COVID-19 associated pneumonia admitted to an education and training hospital Istanbul, Turkey were evaluated. RESULTS: On the first day of hospitalization, ARF was developed in both of them. Ultrasonography and computedtomography scan revealed image of urolithiasis and hydronephrosis. However, in their cystoscopies, there were densedebris materials leading to obstruction in calyceal system. Antifungal medication was added to the COVID-19 treatment due to the macroscopic aspect during the cystoscopy. Renal functions of the children were improved following the treatment. CONCLUSIONS: Clinicians caring for patients with COVID-19 should perform a comprehensive analysis to detect co-infections.

OBJETIVOS: Los pacientes con infecciones virales están en riesgo de infecciones secundarias y co-infecciones. Nuestro estudio tiene como objetivos describir COVID-19 asociado a fallo renal agudo secundario a bezoarmicótico en el tracto urinario en dos pacientes.MÉTODOS: Se evaluó la historia clínica de dos pacientes con COVID-19 asociado a neumonía e ingresados en un hospital académico en Istambul, Turquía. RESULTADOS: En el primer día de hospitalización, el fallo renal agudo se desarrolló en ambos. La ecografía renal y tomografía computerizada mostraron imagen de urolitiasise hidronefrosis, aunque, en la cistoscopia había un material denso que obstruía el sistema caliceal. La medicación antifúngica se añadió al tratamiento de COVID-19 debido al aspecto macroscópico durante la cistoscopia. La función renal de los niños mejoró con el tratamiento. CONCLUSIONES: Los médicos que tratan pacientes con COVID-19 deberían hacer un análisis completo para detectar co-infecciones.

Covid-19 complicated with acute renal failure due to mycotic bezoars in two children / Covid-19 complicado con insuficiencia renal aguda por bezoares micóticos en dos niños

Objetives: Patients with viral infection areat risk for secondary infections and/or co-infections. Ourstudy aims to describe coronavirus-disease-19 (COVID-19)associated with acute renal failure (ARF) secondary to fungal bezoar in urinary tract in two patients.Methods: A chart review of two patients with COVID-19associated pneumonia admitted to an education and training hospital Istanbul, Turkey were evaluated.Results: On the first day of hospitalization, ARF was developed in both of them. Ultrasonography and computedtomography scan revealed image of urolithiasis and hydronephrosis. However, in their cystoscopies, there were densedebris materials leading to obstruction in calyceal system.Antifungal medication was added to the COVID-19 treatment due to the macroscopic aspect during the cystoscopy.Renal functions of the children were improved following thetreatment.Conclusions: Clinicians caring for patients withCOVID-19 should perform a comprehensive analysis todetect co-infections.(AU)

Objetivos: Los pacientes con infeccionesvirales están en riesgo de infecciones secundarias y co-infecciones. Nuestro estudio tiene como objetivos describirCOVID-19 asociado a fallo renal agudo secundario a bezoar micótico en el tracto urinario en dos pacientes.Métodos: Se evaluó la historia clínica de dos pacientescon COVID-19 asociado a neumonía e ingresados en unhospital académico en Istambul, Turquía.Resultados: En el primer día de hospitalización, el fallorenal agudo se desarrolló en ambos. La ecografía renal ytomografía computerizada mostraron imagen de urolitiasise hidronefrosis, aunque, en la cistoscopia había un material denso que obstruía el sistema caliceal. La medicaciónantifúngica se añadió al tratamiento de COVID-19 debidoal aspecto macroscópico durante la cistoscopia. La funciónrenal de los niños mejoró con el tratamiento.Conclusiones: Los médicos que tratan pacientes conCOVID-19 deberían hacer un análisis completo para detectar co-infecciones.(AU)

Impact of Extracorporeal Stent Placement during Laparoscopic Pyeloplasty on Operative Duration.

OBJECTIVES: Ureteric stent insertion during laparoscopic pyeloplasty is the common practice for the reconstruction of ureteropelvic junction obstruction (UPJO). The long and hard learning curve of the method leads still controversy among surgeons. The utility of extracorporeal stent insertion in terms of shortening the length of operation time will be discussed in this study. METHODS: A total of 36 children who underwent pyeloplasty for UPJO were evaluated retrospectively. Indications for pyeloplasty were: Obstruction findings in renal scintigraphy, progressive kidney function loss, increasing in anteroposterior pelvis diameter in renal ultrasonography, and current clinical symptoms (febrile urinary tract infection and flank pain). Extracorporeal stent insertion procedure was performed as the following order: Ureteropelvic area and ureter were visualized transperitoneal by three trochars, and UPJO was excised. Thereafter, the ureter is taken out of the skin from pelvic trochar entrance and is spatulated. JJ stent is placed into the ureter. Following this move, the ureter is taken into the intra-abdominal area and first ureteropelvic suture is performed intra-abdominally. RESULTS: Thirteen of patients were girls and 23 were boys. Open surgery was applied for 15 patients (Group 1) and laparoscopic pyeloplasty (Group 2) to 21 patients. In Group 2, JJ stent was placed intracorporeally for six patients (Group 2a) and extracorporeally for the other 15 patients (Group 2b). The average age in Group 1 was 49.2±52 months; it was 86±29 months in Group 2a and 144±52 months in Group 2b, and the significant difference was present (p<0.001). There was statistically significant difference between Group 1 and Group 2 regarding with mean hospital stay (40 h in Group 1 and 42 h in Group 2; p=0.001). Post-operative JJ stent removal time was 58.8 days in Group 1 and 52.89 days in Group 2. In Group 2b patients, placing the stent extracorporeally took a significantly shorter operation time and the difference between the operation times of the groups (2a [192 min±3.76] and 2b [135 min±2.6] [p<0.001]) was statistically significant. No statistical difference was found between Group 1 (9.87±5.5 mm) and Group 2 (12.91±5.3 mm) in terms of renal anteroposterior diameters in the control ultrasonographic evaluation at the post-operative 2nd year (p=0.23). There was no difference between the two groups as a result of the evaluation of renal functions by scintigraphy at the post-operative 1st year (Group 1: 3.95±2%; and Group 2: 0.78±5.3%). CONCLUSION: According to the consequences in this study, extracorporeally ureteric stent insertion during laparoscopic pyeloplasty shortens the length of operation duration so that extracorporeal insertion might be preferred in cases where it is difficult to place the stent during laparoscopic pyeloplasty.

An Unusual Urological Manifestation of Williams-Beuren Syndrome: Posterior Urethral Valve.

Williams-Beuren syndrome (WBS) is a genetic, well-defined, rare, neurodevelopmental disorder characterized by intellectual disability, congenital heart defects, abnormal facial features, and growth, endocrine, and genitourinary abnormalities. The genitourinary abnormalities in WBS frequently include congenital structural renal defects, vesicoureteral reflux, nephrocalcinosis, proteinuria, and chronic renal insufficiency. Treatment of patients with posterior urethral valve (PUV) remains a clinical challenge, requiring long-term management from early infancy into adulthood in order to avoid progressive renal insufficiency. To my knowledge, this is the first worldwide case of WBS with PUV in a 12-year-old boy. Due to the delayed detection of the defect, chronic renal disease occurred as a risk for him. This case demonstrates the importance of early diagnosis of genitourinary anomalies such as PUV to prevent chronic renal disease in boys and especially in patients with WBS.

Serum creatinine levels in cases of posterior urethral valve: 29 years experience of a pediatric urology reference center.

OBJECTIVES: The aim of this retrospective study is to describe the importance of serum creatinine levels for the long-term renal outcome in cases of posterior urethral valve. METHODS: Records of 94 patients who underwent cystoscopic valve ablation were evaluated retrospectively. Demographic variables, radiologic findings, and serum creatinine levels were recorded. Patients were grouped according to the serum creatinine levels in the first month of life. Twenty-four patients' serum creatinine levels were found to be above 1 mg/dL in group 1 and ≤1 mg/dL in 70 patients in group 2. RESULTS: The initial findings of 74 patients were as follows: pyelonephritis in 24 (32.43%) patients, hydronephrosis in 20 (27.02%), and inability to urinate in 18 (24.32%). Prenatal hydronephrosis was present in 36 cases (38.29%). Of the 92 voiding cystograms, 53.26% had findings that included vesicoureteral reflux. The mean serum creatinine levels of the patients in group 1 increased above 1 mg/dL in the 2nd year, the significant difference between the two groups lasted up to 7 years of age, and no significant difference was observed at the age of 7. The mean serum creatinine levels of the patients in group 2 were observed to increase above 1 mg/dL by the 10th year. CONCLUSIONS: In patients with posterior urethral valves, nadir creatinine, especially higher than 1 mg/dL, should be considered as high risk for end-stage renal disease. Therefore, clinicians should improve awareness about early detection and treatment of posterior urethral valves, and these patients should be monitored as early as possible for the risk of chronic kidney disease.

Multidimensional Analysis of Urinary Stone Diseases in Pediatric Patients.

OBJECTIVES: Urinary tract stones are less common in children than in adults. Determining the etiology is the most important step to achieve successful treatment and prevent future recurrence. The aim of this study was to investigate the clinical characteristics and possible risk factors for urinary stone disease in pediatric patients. METHODS: The data of 126 patients with urinary stone disease who were treated in a pediatric nephrology clinic between 2000 and 2014 were analyzed retrospectively. A total 126 patients were enrolled in the study: 70 (55%) male and 56 (45%) female patients were included. The complaints, age of diagnosis, family histories, and stone location were examined. Direct urine microscopic examination, complete urinalysis, and urine culture were performed for all of the patients. Calcium, uric acid, oxalate, citrate, magnesium, and cystine levels were measured in urine collected in a 24-hour period. Serum electrolyte, blood urea nitrogen, creatinine, calcium, phosphorus, uric acid, and albumin levels were measured. Urinary ultrasound was performed. Stone analysis was conducted using the X-ray diffraction method. The mean age of the patients was 55 months (range: 1-162 months) at presentation. RESULTS: In all, 34% of the patients had a family history of urinary stone disease. The rate of previous urinary tract infection was 26%. It was determined that 34% of the patients had been taking vitamin D and 5% had been taking a high dose. Metabolic risk factors determined were: hypercalciuria in 41%, hypocitraturia in 30%, hyperoxaluria in 14%, hyperuricosuria in 10%, and cystinuria in 5%. Among the group, 81% of the patients had kidney stones, 6.5% had ureter stones, and 2.5% had bladder stones. Furthermore, it was determined that 45% of the stones were composed of calcium oxalate, 35% had calcium phosphate stones, 14.2% had uric acid stones, and 13.3% had cystine stones. In 52% of the cases, extracorporeal shock wave lithotripsy was performed, and 71% underwent surgical treatment. CONCLUSION: Metabolic evaluation and stone analysis should be performed to prevent future recurrences in children with urinary stone disease and lifelong follow-up should be emphasized.

The learning curve of sting method for endoscopic injection treatment of vesicoureteral reflux

ABSTRACT Objective: To identify how many endoscopic injection (EI) procedures, STING method, must be performed before reaching an ideal success rate when simulation training has not been received. Materials and Methods: The EI procedures performed by two pediatric urology fellows were investigated. The study excluded patients without primary VUR and those with previous EI or ureteroneocystostomy, lower urinary tract dysfunction, and/or duplicate ureters. The EIs used dextranomer hyaluronate and the STING method, as described by O'Donnell and Puri. Groups number was determined by multiple statistical trials. Statistically significance differences were achieved with one combination that had 35 EI procedures each and with 3 different combination of patients, having 12, 24, and 36 patients, respectively. Therefore, groups were established 12 patients. The first fellow performed 54 EIs, and the second performed 51. Therefore, each of the first fellow's three groups contained 18 EI procedures, and each of the second fellow's 17. Results: The study included 72 patients and 105 ureter units. When the data from both fellows were combined, each of the three groups contained 35 procedures. For the first fellow, the success rates in the first, second, and third groups were 38.3%, 66.6%, and 83.3% (p = 0.02), respectively, and for the second fellow, the success rates were 41.2%, 64.7%, and 82.3% (p = 0.045), respectively. The increased success rates for both fellows were very similar. Conclusions: An acceptable rate of success for EI may be reached after about 20 procedures and a high success rate after about 35-40 procedures.

The learning curve of sting method for endoscopic injection treatment of vesicoureteral reflux.

OBJECTIVE: To identify how many endoscopic injection (EI) procedures, STING method, must be performed before reaching an ideal success rate when simulation training has not been received. MATERIALS AND METHODS: The EI procedures performed by two pediatric urology fellows were investigated. The study excluded patients without primary VUR and those with previous EI or ureteroneocystostomy, lower urinary tract dysfunction, and/or duplicate ureters. The EIs used dextranomer hyaluronate and the STING method, as described by O'Donnell and Puri. Groups number was determined by multiple statistical trials. Statistically significance differences were achieved with one combination that had 35 EI procedures each and with 3 different combination of patients, having 12, 24, and 36 patients, respectively. Therefore, groups were established 12 patients. The first fellow performed 54 EIs, and the second performed 51. Therefore, each of the first fellow's three groups contained 18 EI procedures, and each of the second fellow's 17. RESULTS: The study included 72 patients and 105 ureter units. When the data from both fellows were combined, each of the three groups contained 35 procedures. For the first fellow, the success rates in the first, second, and third groups were 38.3%, 66.6%, and 83.3% (p = 0.02), respectively, and for the second fellow, the success rates were 41.2%, 64.7%, and 82.3% (p = 0.045), respectively. The increased success rates for both fellows were very similar. CONCLUSIONS: An acceptable rate of success for EI may be reached after about 20 procedures and a high success rate after about 35-40 procedures.

Exhaled breath condensate magnesium levels of infants with bronchiolitis.

Demirkan FG, Yilmaz E, Hangül M, Öztürk D, Demirkan H, Soylak M, Köse M. Exhaled breath condensate magnesium levels of infants with bronchiolitis. Turk J Pediatr 2018; 60: 535-539. The aim of this study is to determine the exhaled breath condensate (EBC) magnesium levels of infants with bronchiolitis and to investigate their relationship with disease severity. Fifty infants with moderate and severe bronchiolitis, grouped according to the bronchiolitis clinical severity scores (CSS), and 25 healthy children were included in the study. EBC was collected using an R tube commercial device; flammable atomic absorption spectrometry was used for the identification of magnesium level in this method. The median concentration of EBC magnesium levels in the group of moderate bronchiolitis patients was 0.82 (0-2.71) µg/ml, and that of severe bronchiolitis patients was 0.57 (0-3.16) µg/ml. The median concentration of EBC magnesium levels in the control group was 0.45 (0.06-2.66) µg/ml. There were no significant differences among the three groups (p > 0.559). The magnesium levels of the two bronchiolitis groups were also found in a wide range. In conclusion, EBC magnesium levels were not different in bronchiolitis patients and do not reflect disease severity.

A comparison of polymer clips and endoloop applications for securing the appendiceal stump during laparoscopic surgery in children.

INTRODUCTION: In this study, for the first time in children a polymer clip and endoloop (EL) for securing an appendiceal stump have been prospectively correlated and evaluated radiologically. METHODS: Forty-nine patients aged 1 to 15 years were operated upon by the same surgeon for acute or perforated appendicitis between May 2008 and May 2009. The appendiceal stump was ligated by an EL or polymer clip. Patients were radiologically evaluated during the postoperative period. RESULTS: In the EL group, the mean operating time for perforated appendicitis was recorded as 57.40 minutes and in nonperforated appendicitis as 39.37 minutes, respectively. In the clips-applied group, these periods were 48.23 and 34.72 minutes, respectively. Clip application is 3 times cheaper than EL. CONCLUSIONS: Polymer clip is an instrument that is cheaper, safe, easily applicable, and takes less time for securing appendiceal stumps compared with EL.