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Query: UMLS:C0041960 (ureterocele)
 507 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital anomalies of the lower urinary tract are a significant cause of morbidity in infancy. Radiologic investigation is an important source of clinical information in lower urinary tract disorders but should not inconvenience the patient, expose the patient to unnecessary radiation, or delay surgical correction. In pediatric patients with suspected underlying urologic structural anomalies, screening ultrasonography is commonly the initial diagnostic study. If dilatation of the urinary tract is confirmed, voiding cystourethrography is performed to determine the presence of vesicoureteral reflux (VUR) and other causes of upper tract dilatation. If VUR is confirmed, follow-up with nuclear cystography or echo-enhanced cystosonography may be performed. If VUR is excluded, nuclear diuresis renography is the primary test for differentiating between obstructed and nonobstructed megaureter. Intravenous urography can be used to specifically identify an area of obstruction and to determine the presence of duplex collecting systems and a ureterocele. Computed tomography and magnetic resonance (MR) imaging are unsuitable for general screening but provide superb anatomic detail and added diagnostic specificity. MR imaging is mandatory in the evaluation of associated spinal anomalies. MR urography can demonstrate ectopic extravesical ureteric insertions, thereby providing a global view of the malformation. Familiarity with anomalies of the lower urinary tract is essential for correct diagnosis and appropriate management.
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PMID:Anomalies of the distal ureter, bladder, and urethra in children: embryologic, radiologic, and pathologic features. 1223 44

Congenital anomalies affecting the ureter-bladder junction are frequent in newborns and are often associated with other developmental defects. However, the molecular and morphological processes underlying these malformations are still poorly defined. In this study, we identified the leukocyte antigen-related (LAR) family protein tyrosine phosphatase, receptor type, S and F (Ptprs and Ptprf [also known as Lar], respectively), as crucially important for distal ureter maturation and craniofacial morphogenesis in the mouse. Embryos lacking both Ptprs and Ptprf displayed severe urogenital malformations, characterized by hydroureter and ureterocele, and craniofacial defects such as cleft palate, micrognathia, and exencephaly. The detailed analysis of distal ureter maturation, the process by which the ureter is displaced toward its final position in the bladder wall, leads us to propose a revised model of ureter maturation in normal embryos. This process was deficient in embryos lacking Ptprs and Ptprf as a result of a marked reduction in intrinsic programmed cell death, thereby causing urogenital system malformations. In cell culture, Ptprs bound and negatively regulated the phosphorylation and signaling of the Ret receptor tyrosine kinase, whereas Ptprs-induced apoptosis was inhibited by Ret expression. Together, these results suggest that ureter positioning is controlled by the opposing actions of Ret and LAR family phosphatases regulating apoptosis-mediated tissue morphogenesis.
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PMID:Maturation of ureter-bladder connection in mice is controlled by LAR family receptor protein tyrosine phosphatases. 1927 6

Congenital anomalies of the kidney and urinary tract (CAKUT) form a group of heterogeneous disorders that affect the kidneys, ureters and bladder, with frequent asynchronous presentations and multiple CAKUT associations in the same individual. Urinary tract formation is a complex process, dependent of the interaction of multiple genes and their sub-product. The same genic alterations can lead to different molecular expressions and different morphological anomalies. The ureterocele is a cystic dilation of the distal intramural ureter, resulting in obstruction of urine flow, dilation of the ureter and renal pelvis and loss of renal function. Two key steps in the urinary tract ontogenesis may be related to ureterocele development: formation and migration of the ureteric bud and its incorporation in the bladder. This review aims to describe the morphological, cellular and biochemical steps, as well as the genes involved in the occurrence of this anomaly.
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PMID:Genetic Basis of Ureterocele. 2701 24

Congenital anomalies of the kidneys or lower urinary tract (CAKUT) are the most common causes of renal failure in children and account for 25% of end-stage renal disease in adults. The spectrum of anomalies includes renal agenesis; hypoplasia; dysplasia; supernumerary, ectopic or fused kidneys; duplication; ureteropelvic junction obstruction; primary megaureter or ureterovesical junction obstruction; vesicoureteral reflux; ureterocele; and posterior urethral valves. CAKUT originates from developmental defects and can occur in isolation or as part of other syndromes. In recent decades, along with better understanding of the pathological features of the human congenital urinary tract defects, researchers using animal models have provided valuable insights into the pathogenesis of these diseases. However, the genetic causes and etiology of many CAKUT cases remain unknown, presenting challenges in finding effective treatment. Here we provide an overview of the critical steps of normal development of the urinary system, followed by a description of the pathological features of major types of CAKUT with respect to developmental mechanisms of their etiology.
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PMID:Developmental pathology of congenital kidney and urinary tract anomalies. 3119 39