By Amin J. Barakat, H. Gil Rushton
This complete, effortless to learn reference addresses the medical implications of congenital anomalies of the kidney and urinary tract (CAKUT) in childrens. Authored through a panel of across the world famous pediatric nephrologists and urologists, chapters talk about medical presentation, workup, interpretation of imaging reports, genetics, prenatal analysis, prevention and remedy of varied anomalies to aid the practitioner comprehend, diagnose and deal with CAKUT. Tables, figures, algorithms and an intensive appendix directory stipulations and syndromes linked to CAKUT are featured to help physicians within the differential prognosis and workup of alternative conditions.
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Extra info for Congenital Anomalies of the Kidney and Urinary Tract: Clinical Implications in Children
Around half of cases have an associated urogenital anomaly when investigated. The commonest is vesicoureteric reflux (VUR), but others include contralateral renal dysplasia, absence of vas deference (perhaps indicating aberrant mesonephric develoment), absent adrenals, and pelvic renal ectopy. Bilateral or unilateral renal agenesis accompanies many syndromes such as the branchiooto-renal syndrome, renal cysts and diabetes syndrome (RCAD) and the hypoparathyroidism, hypoparathyroidism, sensorineural deafness and renal disease syndrome (HDR), Fanconi’s anemia, and Fraser, Kallmann, Di George, and Smith-Lemli-Opitz syndromes.
H. Makari the contralateral kidney or the ipsilateral atretic ureter. VUR has been reported to occur in 15–28 % of patients with MCDK and is low grade in the majority of cases . Ureteropelvic junction (UPJ) obstruction of the contralateral kidney has been reported in up to 15 % of patients with MCDK . Many other urinary tract abnormalities have been reported in association with MCDK with lesser frequency including ureterocele, ureterovesical junction obstruction, and ureteral ectopia. Risk of malignancy: Primary renal malignancies, typically Wilms’ tumor and renal cell carcinoma, have been described to occur in the MCDK in rare instances.
The risk of a Wilms’ tumor developing in an MCDK has been estimated to be approximately 1 in 2000 based on a large series of 7500 Wilms’ tumor samples collected by the National Wilms Tumor Study Pathology Center. All reported instances of Wilms’ tumor developing in an MCDK have occurred before 4 years of age, and no cases of the tumor have been described to occur in the setting of an involuted MCDK . Six cases of renal cell carcinoma developing in an MCDK have been described in the literature, which corresponds to a lower risk for renal cell carcinoma than in a normal kidney .
Congenital Anomalies of the Kidney and Urinary Tract: Clinical Implications in Children by Amin J. Barakat, H. Gil Rushton