Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0403608 (ureter)
 9,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 26-month-old child diagnosed with prune-belly syndrome and end-stage renal disease who received intraperitoneal implantation of an adult cadaveric renal graft which functioned very well for approximately 6 weeks. The patient then presented with acute renal failure which was proved to be secondary to torsion of the graft, twisting the artery and vein. The ureter was wrapped 360 degrees around the graft. These conditions resulted in loss of the graft and nephrectomy. Ours is the second report of such an occurrence; the first was from a living-related kidney donor. We believe the lack of abdominal wall tone contributes to graft mobility and risk of torsion of the kidney. We recommend that nephropexy be considered in these patients. In addition, the risk of torsion must be at the forefront of the differential diagnosis in a prune-belly renal transplant patient with acute onset of oliguria. Renal sonography with Doppler should be employed as soon as possible so that the graft can be saved.
 ...
PMID:Renal allograft torsion associated with prune-belly syndrome. 774 30

Megalourethra, a rare congenital disorder involving the anterior urethra, is subdivided into two types: Fusiform and scaphoid. Two cases of scaphoid type megalourethra are reported. The first patient, a 5-weeks-old infant diagnosed at birth as having the prune belly syndrome was admitted to the hospital with vomiting and failure to thrive. Intravenous pyelogram revealed marked dilatation of the left ureter and a bladder diverticulum. A penile urethrostomy was performed. The second patient, born to healthy parents after an uneventful pregnancy and delivery, was found to have an enlarged and deformed penis. The baby voided with a poor stream and a concomitant swelling of the penis was noted. Retrograde uretrography showed a sac-like dilatation of the penile urethra. Surgical revision was carried out a 2 stage procedure and was completed (Nesbitt's operation) after 4 months. The patient did well postoperatively and voided with a normal stream without any abnormality of the penile shaft.
 ...
PMID:Congenital megalourethra. 1083 81

We report a rare case that can be classified as prune-belly syndrome in a female. This patient came to our attention during the 19th week of gestation when massive ascites was found by ultrasound and the mother was transferred to our hospital. After birth, it was demonstrated that the infant had complicated anomalies, including abdominal muscular hypoplasia, urogenital sinus, a single, right-sided vaginal ectopic ureter, and hypoplasia of the right kidney. A sibling had a right hypoplastic kidney and right vesicoureteral reflux. Considering the combination of anomalies and the family history of urinary tract disease, her anomalies could be attributed to a genetic mutation.
 ...
PMID:Single vaginal ectopic ureter and renal hypoplasia associated with urogenital sinus and abdominal muscular hypoplasia-a novel subtype of prune-belly syndrome in a female child? 1133 7

Primary megaureter is visualized as a typical dilatation on urography. Adequate oblique projections should be added to depict the juxtavesical tract of normal caliber. In even the most severe forms pyelocaliectasis is absent or mild and the ureter is not tortuous. Sonography as well as fluoroscopy during urography shows hyperperistalsis in the dilated tract and aperistalsis in the juxtavesical tract. Cystography rules out vesicoureteral reflux. In prune-belly syndrome the megaureter is bilateral and massive with no evidence of obstructions. The sonographic finding of hydronephrosis or hydroureteronephrosis and the absence of vesicoureteral reflux on voiding cystography lead to the suspicion of ureteral valves, directly documented on urography as sharp and transverse filling defects. Retrograde pyelography can better define the obstructing area and documents the normality of the underlying tract. With this procedure, the valvular flaps appear with a superior convexity (like a small umbrella). MR-urography still to be validated clinically, will be able to provide novel perspectives.
 ...
PMID:Megaureter and ureteral valves. 1269 59

Congenital anomalies of the lower urinary tract (CALUT) are a family of birth defects of the ureter, the bladder, and the urethra. CALUT includes ureteral anomaliesc such as congenital abnormalities of the ureteropelvic junction (UPJ) and ureterovesical junction (UVJ), and birth defects of the bladder and the urethra such as bladder-exstrophy-epispadias complex (BEEC), prune belly syndrome (PBS), and posterior urethral valves (PUVs). CALUT is one of the most common birth defects and is often associated with antenatal hydronephrosis, vesicoureteral reflux (VUR), urinary tract obstruction, urinary tract infections (UTI), chronic kidney disease, and renal failure in children. Here, we discuss the current genetic and molecular knowledge about lower urinary tract development and genetic basis of CALUT in both human and mouse models. We provide an overview of the developmental processes leading to the formation of the ureter, the bladder, and the urethra, and different genes and signaling pathways controlling these developmental processes. Human genetic disorders that affect the ureter, the bladder and the urethra and associated gene mutations are also presented. As we are entering the postgenomic era of personalized medicine, information in this article may provide useful interpretation for the genetic and genomic test results collected from patients with lower urinary tract birth defects. With evidence-based interpretations, clinicians may provide more effective personalized therapies to patients and genetic counseling for their families.
 ...
PMID:Lower urinary tract development and disease. 2340 57

Patients with prune belly syndrome usually have tortuous ureters, which can cause difficulty in the interpretation of renal scan used to evaluate possible urine leak after renal transplant. We reported a renal scan finding in a pediatric renal transplant patient with prune belly syndrome. The radioactivity in the dilated ureter, which was lateral to the renal transplant, appears to be urine leak.
...
PMID:Suspected Urine Leak in a Pediatric Renal Transplant Patient With Prune Belly Syndrome. 2644 90


<< Previous 1 2