[The results of treatment of newborns with postrenal anuria due to upper urinary tract obstruction, caused by fungal bezoar].

AIM: To evaluate the results of different methods of urine derivation in patients with postrenal anuria due to upper urinary tract (UUT) obstruction, caused by fungal bezoar. MATERIALS AND METHODS: The results of treatment of postrenal anuria in 8 patients without congenital obstructive anomalies of UUT in 5 clinics from 2004 to 2018 were analyzed. All patients from the birth received continuously two or more antibiotics for diseases not related to kidneys and the urinary tract. Median of gestational age was 32 weeks [31.5; 38.5]. There were 4 boys (50%), and 4 girls (50%). The duration of anuria at the time of hospitalization was 2 days [1.5; 5]. Creatinine level at admission was 218 mol/l [164.5; 392.5] and urea was 17.9 mmol/l [13.2; 24.95]. In all cases, candida albicans was revealed in urine. Postoperative complications were graded according to the Clavien-Dindo classification. RESULTS: All patients received systemic antifungal therapy; UUT drainage was performed in 7 (87.5%) cases. Ureteral catheter was placed bilaterally in 2 (28.6%) patients (4 renal units). There were 5 complications (Clavien-Dindo grade IIIb). Bilateral percutaneous nephrostomy was performed in 3 (42.8%) patients (6 renal units), and there were 2 complications (Clavien-Dindo grade IIIb and V). Open pyelostomy was performed in 2 (28.6%) patients (one bilateral, one on the right side; a total of 3 renal units). There was only one complication of Clavien-Dindo grade V. The average number of surgical procedures required to alleviate UUT obstruction per one renal unit was as following: 2.25 for ureteral catheterization; 1.17 for percutaneous nephrostomy; 1 for open pyelostomy. Regardless of the drainage method, diuresis tended to be normalized and azotemia decreased during the first postoperative day. In one case, the obstruction was eliminated by antifungal therapy without drainage of UUT. Mortality rate was 25% (n = 2). CONCLUSION: Ureteral catheterization is a minimally invasive, safe method for decompression of UUT obstruction, caused by fungal bezoar, which is non-inferior to nephrostomy tube or open pyelostomy. Ureteral catheterization can be used as a primary method of urine derivation in children with UUT obstruction caused by candida bezoar.

[Disorders of sex development 46 XY and bilateral metachronous wilms tumor in a child with mutation in exon 7 of WT1 gene].

Denys-Drash syndrome is characterized by a triad: nephropathy, a 46, XY disorder of sex development, and nephroblastoma with mutations in the gene WT1. A clinical case of a patient with a bilateral metachronous Wilms' tumor, a 46, XY disorder of sex development in the form of a scrotal hypospadias and bilateral abdominal cryptorchidism, without nephropathy with a mutation in 7 exon of gene WT1 is presented in the article. The child underwent left-sided nephrectomy, lower pole right partial nephrectomy, bilateral orchiopexy and two-stage correction of hypospadias. After 7 years from the start of treatment and 3 years after the last procedure, the childs condition has been assessed as satisfactory. The presented case, according to the analysis of literature, has not been previously described, therefore, it currently remains as "de novo" and requires further observation.

[Uretero-ureteral anastomosis (ureteropyeloanasto-mosis) for the duplicated upper urinary tract in children].

INTRODUCTION: The duplex kidney is one of the most common congenital abnormalities of the urinary tract, and various surgical procedures exist for the management of this condition. Depending on the surgeon preferences, patients with duplex kidney may undergo heminephrectomy with ureterectomy using open or laparoscopic approach, uretero-cysto-anastomosis of the ectopic ureter or two ureters in a single block. Recently, there have been reports of inter-ureter anastomoses. Here we report our multicenter experience in open and laparoscopic uretero-ureteral anastomosis (UUA). The study aimed to improve the treatment results in children with urodynamic dysfunction due to the duplicated upper urinary tract. MATERIALS AND METHODS: We retrospectively analyzed medical records of 64 children treated from 2007 to 2017. There were 22 (32.8%) boys and 43 (67.2%) girls with mean age 40.2 months at the time of surgery. All of them had duplex kidneys, including 27 (42.2%) right-sided and 37 (57.8%) left-sided. Of them, 15 (23.4%) children underwent distal UUA (DUAA), and 49 (76.6%) had proximal UUA (PUUA). RESULTS: After surgery, acute pyelonephritis occurred in three patients. In the early postoperative period, a prolonged urinary leakage from surgical site drainage was observed in three (6.1%) patients after laparoscopic PUUA. One (4.2%) child developed a stumpitis. Thus, complications occurred in 7 (10.9%) children; one of them (1.6%) needed additional treatment. CONCLUSION: Distal and proximal UUA is a safe and effective surgical treatment for urinary outflow disorders in patients with the duplicated upper urinary tract, minimizing the risk of the duplex kidney dysfunction.

[Laparoscopic heminephrureterectomy for duplex kidney in children].

INTRODUCTION: Duplication of the upper urinary tract is one of the most common congenital urological anomalies. In patients with critically decreased or lost function of one of the renal segments, heminephrureterectomy is usually the treatment of choice. Until recently, this was an open surgery; in cases of complete removal of the ureter, an additional incision in the iliac region was required. Currently, heminephrureterectomy is increasingly performed laparoscopically. We report the experience in laparoscopic heminephrureterectomy (LHNUE) in 10 clinics in Russia and Belarus. Some of them have already used this technique for 10 years. AIM: The study aimed to to improve the treatment results in children with urodynamic dysfunction due to duplicated upper urinary tract. MATERIALS AND METHODS: We retrospectively analyzed medical records of 111 children treated from 2007 to 2016. There were 26 (23.4%) boys and 85 (76.6%) girls with mean age 44.6 months (from 2 to 170) at the time of surgery. All children included in the study had complete duplex kidneys, including 51 (45.9%) right-sided and 60 (54.1%) left-sided. All the children underwent LHNUE for a critical decrease or absence of function of the upper or lower segment of the duplex kidney caused by the following pathology: obstruction of the ureterovesical junction with the development of the megaureter of the upper ureteral segment in 57 (51.4%) patients; ureterocele in 28 (25.2%); extra-vesical ectopic ureter with urinary incontinence in 10 (9.0%) girls; high-grade UVR in 16 (14.4%) patients. RESULTS: There were no conversions in this series of patients. The mean operative time was 135 minutes (60-240 min.). All children included in the study were followed for 1 to 9 years after surgery. Complications occurred in 17 (15.3%) patients, of whom 12 (10.8%) required repeat surgery. In one patient with the loss of lower pole function, the treatment result was considered unsatisfactory. CONCLUSION: LHNUE for duplex kidney is performed by a few clinics and is still at the stage of development and accumulation of experience. Nevertheless, LHNUE, though an effective treatment modality, carries the risk of reducing or losing the function of the retained segment.