27 percutaneous endopyélotomies were performed in 27 patients from February 1989 to January 1996 to treat 20 cases of congenital hydronephrosis and 7 cases of acquired stenosis. Strictures of uretero-pelvic junction were treated by electrocautery with postoperative drainage using a double J stent in 11 cases, or Redon drain in 16 cases. The evaluable results for 25 patients with a mean follow-up of 11 months showed an overall success rate of 80%. This technique is the treatment of choice for acquired stenosis and stage I and II congenital hydronephrosis (Cendron).
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Children (Basel)
November 2024
Department of Pediatrics, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, 00168 Rome, Italy.
Background/objectives: The anteroposterior renal pelvis diameter (APRPD) is used to assess the grade of urinary tract dilatation (UTD). There is no univocal method stratifying the risk of complications related to postnatal UTD. This study aims to identify APRPD cut-offs at birth to determine outcome stratification and second-level exams.
View Article and Find Full Text PDFJ Pediatr Urol
December 2024
Department of Urology, University of Iowa, Iowa City, IA, 52242, USA. Electronic address:
Introduction: Prior analysis of children with grade 3 and 4 congenital hydronephrosis demonstrated that renal medullary pyramidal thickness (PT) is predictive of subsequent pyeloplasty (area under the curve [AUC] = 0.78). The objective of this study was to further analyze the utility of sonographic measurements including PT, anteroposterior pelvic diameter (APD), and renal length with an expansion of the number of infants with hydronephrotic kidneys including grades 2, 3, and 4 hydronephrosis.
View Article and Find Full Text PDFJ Med Genet
December 2024
John T. Macdonald Foundation Department of Human Genetics, University of Miami Miller School of Medicine, Miami, Florida, USA
Introduction: Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant disorder classically associated with multiple basal cell carcinomas, odontogenic keratocysts and skeletal anomalies. However, its significant phenotypic heterogeneity often delays the diagnosis. Here, we undertake the first comprehensive characterisation of NBCCS and congenital urinary tract anomalies.
View Article and Find Full Text PDFCEN Case Rep
December 2024
Department of Woman, Child and of General and Specialized Surgery, Università degli studi della Campania "Luigi Vanvitelli", Via Luigi De Crecchio 2, 80138, Naples, Italy.
Nephrogenic diabetes insipidus (NDI) results from the kidneys' inability to concentrate urine. We describe a 6-month-old male with a history of poor weight gain who presented with an incidental finding of hypernatremia (155 mEq/L) during an episode of acute gastroenteritis. The arginine vasopressin (AVP) test, along with molecular analysis revealing the M272R mutation in the AVP receptor 2 (AVPR2) gene, confirmed the diagnosis of congenital NDI.
View Article and Find Full Text PDFJ Pediatr Urol
November 2024
Department of Urology, Ankara City Hospital, Ankara, Türkiye. Electronic address:
Background: The study aimed at evaluating the role of thiol-disulphide balance parameters [native thiol (SH), total thiol (SH + SS), disulphide (SS), disulphide/native thiol ratio (% SS/SH), disulphide/total thiol ratio (% SS/Total Thiol) and native thiol/total thiol ratio (%SH/Total Thiol)], which are important oxidative stress markers in the congenital ureteropelvic junction (UPJ) stenosis, in the diagnosis of the disease, and its role in determining the need for surgery and follow-up.
Materials And Methods: This prospective study included 30 children diagnosed with congenital intrinsic ureteropelvic junction obstruction (UPJO) and a control group of 30 healthy children admitted to the pediatric clinic between January 2016 and February 2017. The children with UPJO underwent laparoscopic dismembered pyeloplasty.
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