Duplex, ectopic, and horseshoe kidneys

2015 ◽  
Author(s):  
Michiel F. Schreuder
Keyword(s):  
Urinary Tract ◽  
Vesicoureteral Reflux ◽  
Horseshoe Kidney ◽  
Abdominal Ultrasound ◽  
High Rate ◽  
Ectopic Ureter ◽  
Duplex System ◽  
Renal Ectopia ◽  
Contralateral Kidney ◽  
Duplex Systems

A duplex urinary tract, irrespective of the degree of duplication, is present in 0.8% at autopsy, of which about 20–35% is bilateral. The majority of duplex systems are incomplete, indicating that the ipsilateral ureters fuse before entering the bladder. A complete duplex system shows anomalies of the upper moiety, with associated ureterocele or ectopic ureter, and of the lower moiety, frequently associated with vesicoureteral reflux. Renal ectopia is a rare (1/1000) congenital defect where the kidney is not located in the renal fossa, and is associated with a high rate of hydronephrosis, vesicoureteral reflux, and abnormal contralateral kidney. In a horseshoe kidney (present in 1/400 to 1/1800), fusion of the two kidneys takes place, but the two renal moieties are still located on both sides of the midline. As the lower poles are fused in the midline, a horseshoe kidney is usually located lower than normal and orientation of the renal axis is shifted, which may guide diagnosis during abdominal ultrasound.

scholarly journals The role of voiding cystourethrography in the investigation of children with urinary tract infections

2016 ◽  
Vol 10 (5-6) ◽  
pp. 210 ◽  
Author(s):  
Linda C. Lee ◽  
Armando J. Lorenzo ◽  
Martin A. Koyle
Keyword(s):  
Urinary Tract ◽  
Vesicoureteral Reflux ◽  
Urinary Tract Infections ◽  
Renal Scarring ◽  
High Rate ◽  
Voiding Cystourethrography ◽  
Low Grade ◽  
Endoscopic Injection ◽  
Febrile Uti ◽  
Tract Infections

Urinary tract infections (UTIs) represent a common bacterial cause of febrile illness in children. Of children presenting with a febrile UTI, 25‒40% are found to have vesicoureteral reflux (VUR). Historically, the concern regarding VUR was that it could lead to recurrent pyelonephritis, renal scarring, hypertension, and chronic kidney disease. As a result, many children underwent invasive surgical procedures to correct VUR. We now know that many cases of VUR are low-grade and have a high rate of spontaneous resolution. The roles of surveillance, antibiotic prophylaxis, endoscopic injection, and ureteral reimplantation surgery also continue to evolve. In turn, these factors have influenced the investigation of febrile UTIs.Voiding cystourethrography (VCUG) is the radiographic test of choice to diagnose VUR. Due to its invasive nature and questionable benefit in many cases, the American Academy of Pediatrics (AAP) no longer recommends VCUG routinely after an initial febrile UTI. Nevertheless, these guidelines pre-date the landmark Randomized Intervention of Children with Vesicoureteral Reflux (RIVUR) trial and there continues to be controversy regarding the diagnosis and management of VUR. This paper discusses the current literature regarding radiographic testing in children with febrile UTIs and presents a practical risk-based approach for deciding when to obtain a VCUG.

scholarly journals Vesicoureteral Reflux and Duplex Systems

2008 ◽  
Vol 2008 ◽  
pp. 1-3 ◽  
Author(s):  
John C. Thomas
Keyword(s):  
Vesicoureteral Reflux ◽  
Surgical Approaches ◽  
Duplex System ◽  
Ectopic Ureterocele ◽  
Definitive Management ◽  
Duplex Systems

Vesicoureteral reflux (VUR) is the most common anomaly associated with duplex systems. In addition to an uncomplicated duplex system, reflux can also be secondary in the presence of an ectopic ureterocele with duplex systems. Controversy exists in regard to the initial and most definitive management of these anomalies when they coexist. This paper will highlight what is currently known about duplex systems and VUR, and will attempt to provide evidence supporting the various surgical approaches to an ectopic ureterocele and duplex system and the implications of concomitant VUR.

scholarly journals Fanconi Bickel syndrome: clinical phenotypes and genetics in a cohort of Sudanese children

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Salwa A. Musa ◽  
Areej A. Ibrahim ◽  
Samar S. Hassan ◽  
Matthew B Johnson ◽  
Asmahan T. Basheer ◽  
...  
Keyword(s):  
Glucose Transporter ◽  
Novel Mutation ◽  
Wide Spectrum ◽  
Rare Condition ◽  
Neonatal Diabetes ◽  
Abdominal Ultrasound ◽  
Sub Saharan Africa ◽  
High Rate ◽  
Genetic Characteristics ◽  
Sub Saharan

Abstract Background Fanconi-Bickel syndrome (FBS) is a rare condition of carbohydrate metabolism, caused by a recessive defect in the facilitative glucose transporter GLUT2 encoded by the SLC2A2 gene and characterized by a wide spectrum of phenotypical features. There is a paucity of reported data on FBS from Sub-Saharan Africa. Here, we describe the clinical, biochemical and genetic characteristics of our patients with FBS from Sudan, a country with a high consanguinity rate. Patients & methods Eleven patients from ten unrelated Sudanese families were included. Clinical & biochemical data were documented and imaging studies done including bone survey and abdominal ultrasound. Liver biopsy was done to confirm the pathological diagnosis in 45% of cases and molecular genetics was performed through contribution with the Exeter genomics laboratory for ten patients. Results Reported consanguinity was 70% among our patients. Growth was significantly impaired at presentation with mean weights of (-5.3 ± 1.8) SD and heights (-5.4 ± 2.5) SD. Severe chest deformity was present in (27%) and all patients showed features of rickets at presentation. Three patients had neonatal diabetes requiring insulin therapy of which one has been reported before. Six families lost undiagnosed siblings with similar clinical presentations. We identified a total of four homozygous pathogenic SLC2A2 variants in our patients, one of whom had a novel mutation. Conclusions FBS is not uncommon in Sudan where there is a high rate of consanguinity. Many cases are likely missed because of variable presentation and lack of public and professionals’ awareness. This is the first series to describe this condition from Sub-Saharan Africa.

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