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Query: UMLS:C0403608 (ureter)
9,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with prune belly syndrome present a spectrum of abnormality, both in the abdominal wall and the urinary tract. Ureteral pathology has characteristic features and the ureter may be more severely involved at the bladder end than in its upper portion. Early neonatal investigation is required to determine which patient can be treated in a conservative manner and which require neonatal reconstruction or temporary vesical or upper tract drainage.
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PMID:The early assessment for individualized treatment in the prune belly syndrome. 2 69

A selected group of congenitally dilated ureters was examined under light and electron microscopy, and the findings were compared to the clinicoradiologic features of each ureter. These dilated ureters exhibit qualitative and/or quantitative muscular deficiencies. The obstructed megaureters secondary to posterior urethral valves and the megaureters with reflux show non-specific pathologic changes in response to increased work load or decompensation but infection within the ureter is detrimental to the muscle cells. The ureters in babies with prune belly syndrome suffer from reduced muscle cell population and a distinct myopathy involving the myofilaments within the muscle cells. The dysplastic ureter is morphologically and end stage ureter wherein the muscle cells are severely compromised. Whether this is an underdevelopment or exhaustion from repeated infections is a matter for speculation.
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PMID:Ureteral structure and ultrastructure. Part III. The congenitally dilated ureter (megaureter). 13 28

The prune belly syndrome of marked abdominal muscle deficiency and associated genitourinary anomalies has been found almost exclusively in male subjects. Of the few reported cases of female patients with this entity most are questionable or incomplete forms. The light microscopic histology and electron microscopic ureteral abnormalities in male subjects have been reported previously. A female newborn with marked abdominal muscle deficiency and severe bilateral ureteropyelocaliectasis is described. Chromosomal studies showed 46 XX karyotype. Histologic examination of the ureter by light microscopy was compatible with the diagnosis.
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PMID:Prune belly syndrome in the female subject. 14 43

The massively dilated ureter is a major therapeutic challenge that faces the pediatric urologist. In those instances when more conservative measures, such as control of infection or correction of the primary pathology, have failed or are likely to fail surgical treatment must be directed to the massively dilated ureter itself. The goals of reconstructive procedures are the elimination of residual urine, effective ureteral peristalsis, and efficient and/or urgent urinary drainage. We encountered these clinical settings in 244 children with 366 massively dilated ureters from 1965 through 1974. The underlying pathologic processes included primary megaureter, refluxing megaureter, posterior urethral valves, ureteral duplication with upper role ectopic ureterocele or lower pole refluxing megaureter, simple ureterocele, ureterovesical junction obstruction, neurogenic vesical dysfunction, prune belly syndrome and acquired (iatrogenic) megaureter. The results of several reconstructive techniques are reviewed according to the excretory urogram, cystogram, renal function studies and the presence or absence of urinary infection. Analysis of the results with respect to the underlying pathologic entity responsible for the massively dilated ureter indicates that the etiology is a crucial factor in determining whether surgical treatment should be recommended and the type of surgical treatment that will most likely be successful.
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PMID:The influence of etiology on the surgical management and prognosis of the massively dilated ureter in children. 14 4

We describe a twin with acardia acephalus or "Twin Reversed Arterial Perfusion Sequence" and prune belly sequence in the co-twin. In a former quite similar case a prune belly appearance of the co-twin of an acardiac fetus was found to be secondary to the ascites caused by cardiac failure. In the present case, we are dealing with the prune belly sequence as a separate condition, given the fact there were no signs of ascites or cardiac failure. We also found associated anomalies: agenesis of the left ureter and kidney, dysplastic right kidney and anal atresia. Urinary tract obstruction has never been described in the co-twin of an acardiac amorphous fetus.
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PMID:Acardiac amorphous twin with prune belly sequence in the co-twin. 187 24

The clinical course and the pathologic features of 29 patients with the prune belly syndrome (PBS) are reviewed. There were 26 males and 3 females. In addition to the classical triad of deficient abdominal musculature, urinary tract abnormalities, and cryptorchidism, a broader spectrum of other defects was found including musculoskeletal (58%) and gastrointestinal (31%) abnormalities. Genital anomalies were present in all three female patients. Many of these defects may be inapparent at birth, but are the cause of morbidity and mortality later in life. Severe urinary tract maldevelopment and pulmonary hypoplasia as part of the oligohydramnios syndrome was the most common cause of perinatal deaths. In these patients, major portions of the renal parenchyma were dysplastic, but in survivors, renal dysplasia, when present, was minor by comparison, and affected less than 1/3 of the parenchyma. Although several questions remain unanswered, we believe that the PBS results from the effect of one or more teratogenic agents on the somatic mesoderm, producing inappropriate mesenchymal development and inadequate mesenchymal-epithelial interactions that lead to abnormal development and dilatation of some of its derivatives (abdominal muscles, ureter, bladder, prostate, urethra, and gubernaculum). Although abnormalities in derivatives of the intermediate mesoderm (kidney) may also be produced by the injurious agent(s), they are more likely a result of urinary obstruction. Abnormalities in other organs and systems are the consequence of oligohydramnios.
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PMID:Prune belly syndrome: clinicopathologic study of 29 cases. 260 27

A case of prune belly syndrome which was managed by left ureterostomy and right nephrectomy during the neonatal period is reported. The patient also had anorectal agenesis with rectovesical fistula, urethral atresia and patent urachus. When the patient was 14 years of age, the urinary diversion was converted to a continent diversion by an antireflux ureteroneocystostomy of both the left and the right ureter together with a right cutaneous ureterostomy. The patient manages very well by clean intermittent catheterization of the right ureterostomy and is fully continent.
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PMID:Continent urinary diversion in a patient with prune belly syndrome and urethral atresia. 293 Mar 82

The sonographic features of prune-belly syndrome are illustrated and described, corresponding to a classification based on clinical and radiographic findings. The severity of urinary tract involvement determines to which group a given patient is assigned. In group I, the kidneys are dysplastic, with no appreciable surrounding renal parenchyma. In group II, there is marked dilatation of the ureter and mild dilatation or no dilatation of the renal pelvis and calyces. In group III, the involvement is milder, ranging from the sonographic findings typical of group II to those of normal-appearing urinary tract.
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PMID:The ultrasound spectrum of prune-belly syndrome. 644 56

The prune belly syndrome is a very rare entity, characterized by a marked deficiency of the abdominal muscles, bilateral cryptorchidism and urinary tract abnormalities. The etiology is still unknown; the best accepted theory is the embryologic, due to disturbance in development between the sixth to tenth week. Histologically renal dysplasia is found together with partial or total absence of muscle fibers in the ureter, instead of which there is connective tissue, hypertrophy of the bladder with normal ganglionar cells; the prostatic urethra is dilated. There are three clinical types: neonatal, neonatal urgency and late development. There is not as yet a good management program set out for these children in whom the damage is varied. The best treatment is prophylaxis, control of infection avoiding instrumentation and repeated urological examinations. The prognosis depends on the volumen of functional parenchima and its preservation. Depends also on the degree of dysplasia and of insufficiency and secondary damage due to infection. We are reporting three differents cases and their management; we have obtained good results and we expect a good prognosis according to the special characteristics of their evolution.
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PMID:[Prune bell syndrome]. 644 36

Divided into 9 sections, the difficult subject of megaureters is discussed. The classification comprises the primary and the secondary megaureter as well as the less well defined megacystis-megaureter syndrome. An embryopathy of the Wolffian duct and the ureteric buds is the underlying cause. Their main characteristics are obstruction and dysplasia. The primary megaureter develops supravesically. The secondary megaureter, as far as its cause is concerned, starts infravesically and is to be divided into two subgroups, depending on the time the embryopathy is developing. The megaureter, originating in the early embryonal phase is characterized by dysplasia and obstruction. The megaureter, originating in the late embryonal phase, presents obstruction only. The corresponding nephropathy shows obstructive, refluxive and dysplastic features. The causative distal obstruction ascends, its urodynamic reaction, however, descends in accordance with the rules of the pathophysiology of the smooth muscles of cavitary organs. Dilatation of the vesical bladder and the ureter are both prerequisite and symptom of muscular decompensation. The megacystis-megaureter syndrome is felt to be an extreme type of a simple reflux. The infravesical desobstruction is the most important step of the therapeutic strategy, followed by an antireflux ureterocystoneostomy with modellage. The indications are presented. Special types (the ectopic megaureter of a superior renal anlage, the prune-belly syndrome and the megaureter of the neurogenic bladder) are attributed to the classification as described above.
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PMID:[Congenital megaureter and its implications]. 689 13


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