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

We report on 3 sisters with a syndrome of unilobed or absent thymus, renal and ureter agenesis/dysgenesis, and intrauterine growth retardation (IUGR). Two of the 3 infants had a unilobed lung and imperforate anus. Recurrence was detected prenatally by the presence of progressive oligohydramnios and IUGR, a unilateral echogenic cystic mass in the renal fossa, and low amniotic fluid disaccharidases in association with an imperforate anus. Several genetic mechanisms can be invoked to explain this occurrence including autosomal recessive inheritance and an unrecognized chromosome imbalance.
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PMID:Thymic-renal-anal-lung dysplasia in sibs: a new autosomal recessive error of early morphogenesis. 226 May 72

The routine use of maternal ultrasonography has led to frequent discovery of fetal anatomical abnormalities of the urinary tract. Herein we report on 35 cases with congenital urogenital anomalies which were found during the last 5 years by the prenatal ultrasonography and referred to our clinic either for treatment or consultation. 1. Findings of prenatal ultrasonography There were 12 cases of hydronephrosis, 7 of multicystic dysplastic kidney, 4 of megaureter, 3 of posterior urethral valve, 2 of ureterocele, 2 of vesicoureteral reflux and 5 other cases. Fetal anomalies were diagnosed during routine maternal examination, as intrauterine growth retardation, oligohydramnios, or the recurrent risk secondary to genetic risk factor or previous abnormal pregnancies. Prenatal diagnosis was made as early as 20 weeks of gestation in a case of hypoplastic urethra with large bladder diverticula. In about 30% of cases, abnormalities were found before 30 weeks of gestation. Oligohydramnios was evident in 5 cases. Prenatal diagnosis was correct in about 60% of cases. In the remainder the diagnosis was either incomplete or incorrect. Errors in the diagnosis resulted from difficulties in the differentiation of dilated ureter, intestinal dilation or intraperitoneal cystic masses. 2. Management in newborn periods Of the patients, 12 were treated urologically in newborn periods. Although patients with distended bladder and dilatation of upper tracts first underwent therapeutic placement of the urethral catheter, upper tract diversion with nephrostomy or tubeless ureterocutaneostomy was required in 2 because of continued elevation of serum creatinine levels. In 2 neonates nephrectomy was indicated for unilateral multicystic kidney, because the large cystic mass might compress the intestine.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Perinatal management of congenital anomalies of the urinary tract detected in utero]. 240 86

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

Between January 1982 and January 1986, 76 pregnant women between 15 and 40 weeks of gestation were referred because of suspected fetal obstructive urinary tract pathology. A total of 14 high-level (ureter) and 17 low-level (urethral) obstructions were diagnosed. High-level obstructions were at the uretero-pelvic level in 11 and at the uretero-vesical level in 3 cases. Increased amniotic fluid volume was observed in 28 per cent. The survival rate was 86 per cent. In the 17 cases of urethral obstruction, oligohydramnios was present in 70 per cent, associated structural defects in 30 per cent, and abnormal karyotype in 6 per cent. Pregnancy was terminated because of progressive massive hydronephrosis in 41 per cent; intrauterine or neonatal death occurred in 47 per cent, resulting in a survival rate of only 12 per cent.
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PMID:Prenatal evaluation and outcome of fetal obstructive uropathies. 328 21

We report on a patient with EEC/EECUT syndrome and concomitant hypoplasia of the thymus and reduction of T cells in secondary lymphatic organs. The patient was born prematurely at 35 weeks of gestational age and exhibited ectodermal dysplasia, ectrodactyly, cleft palate and urinary tract abnormalities. On the left side, a large ureterocele was present. On the right side, an atretic ureter was found. Both conditions had led to intrauterine hydronephrosis, renal dysplasia, oligohydramnios, pulmonary hypoplasia, and death of the child. Ureteral malformations are thought to be of epithelial origin. Autopsy showed only small rudiments of thymic tissue containing single epithelial cells, but were completely devoid of Hassall corpuscules. Again, this clearly points to an ectodermal defect. Although there was severe reduction of T cells in secondary lymphatic organs, the thymic defect would not have necessarily led to immunological deficiency; perhaps this is the reason that an epithelial defect in the thymus of patients with EEC syndrome has not yet been reported. With regard to an updating of the diagnosis of the EEC/EECUT syndrome, an "EEC/EECUT plus" syndrome is suggested.
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PMID:Hypoplastic thymus and T-cell reduction in EECUT syndrome. 906 85

Therapy for obstructive uropathy is largely determined by whether the obstruction involves one or both kidneys, and by the age of the patient. In the infant and child, obstructive uropathy is almost always due to a congenital malformation of the ureter, bladder, or urethra. Ultrasonographic prenatal diagnosis has permitted early detection and even fetal intervention for posterior urethral valves, although this form of treatment must be considered experimental at present. More important to the affected infant than optimal renal development is the prevention of pulmonary hypoplasia, which is a consequence of fetal oliguria and oligohydramnios. Congenital ureteropelvic junction (UPJ) obstruction is generally unilateral, and although there is controversy regarding the timing of surgical correction, current evidence favors early pyeloplasty. In the adult, obstructive nephropathy is often acquired, with ureteral obstruction usually a consequence of nephrolithiasis. Removal of the stone can be accomplished surgically or by lithotripsy. Bladder outlet obstruction is usually secondary to prostatic hyperplasia, which may progress slowly, allowing a delay in surgical intervention. Neurogenic bladder may require intermittent catheterization or cholinergic therapy; those with hypertonic bladder may benefit from anticholinergics. Regardless of the patient's age, prompt and accurate diagnosis is essential to planning an optimal strategy for the management of obstructive uropathy.
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PMID:Therapeutic approaches in obstructive uropathy. 981 56

Cloacal malformation occurs in approximately 1 in 50,000 live female births. Prenatal ultrasound may lead to the diagnosis in selected cases. We report an unusual case of prenatally detected single-system hydronephrosis with a nonvisible bladder and worsening oligohydramnios. Labor was induced at 35 weeks' estimated gestational age. On physical examination, a single perineal opening was noted consistent with cloaca. Endoscopy revealed an obstructed ectopic ureter at the level of the sphincter, an undeveloped bladder and vagina, and a fistula to the rectum. A low loop cutaneous ureterostomy and right upper quadrant loop colostomy were performed. The absence of a typical fluid-filled pelvic structure may confound the prenatal diagnosis of cloaca.
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PMID:Cloacal outlet obstruction with an ectopic ureter. 1079 8

The widespread use of obstetric ultrasonography has increased the detection rate of antenatal hydronephrosis. Although most cases of antenatal hydronephrosis are transient, one third persists and becomes clinically important. Ultrasound has made differential diagnosis possible to some extent. Ureteropelvic junction type hydronephrosis (UPJHN) is one of the most common cause of persistent fetal hydronephrosis and occurs three times more in male fetuses. It is usually sporadic and unilateral. However, when bilateral kidneys are involved and presents with severe hydronephrosis, the prognosis may be poor. Typical ultrasound findings of UPJHN is hydronephrosis without hydroureter. The size and appearance of the fetal bladder is usually normal without thickening of the bladder wall. Several grading systems are developed and increasingly being used to define the severity of prenatal hydronephrosis and provides much more information about prediction of postnatal renal prognosis. If fetal urinary tract dilation is detected; laterality, severity of hydronephrosis, echogenicity of the kidneys, presence of ureter dilation should be assessed. Bladder volume and emptying, sex of the fetus, amniotic fluid volume, and presence of associated malformations should be evaluated. Particularly the ultrasonographic signs of renal dysplasia, such as increased renal parenchymal echogenicity, thinning of the renal cortex, the presence of cortical cysts, and co-existing oligohydramnios should be noticed. Unfortunately, there is no reliable predictor of renal function in UPJHN cases. Unilateral hydronephrosis cases suggesting UPJHN are mostly followed up conservatively. However, the cases with bilateral involvement are still difficult to manage. Timing of delivery is also controversial.
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PMID:Prenatal Diagnosis and Findings in Ureteropelvic Junction Type Hydronephrosis. 3301 23