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

A family with autosomal dominant inheritance of sacral agenesis is described. Ten members were affected; four had associated presacral teratomas and anterior sacral meningoceles, giving rise to serious complications in three, including bacterial meningitis, local recurrence of teratoma and perianal sepsis. Three of those with presacral masses presented initially with anorectal anomalies. Other associated abnormalities included tethering of the cord, hydrocephalus, duplex ureter, hydronephrosis, vesicoureteric reflux, neurogenic bladder, bicornuate uterus, rectovaginal fistula and hereditary spherocytosis. Early diagnosis and surgical excision of a presacral mass is advised to prevent future morbidity and mortality.
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PMID:Hereditary sacral agenesis with presacral mass and anorectal stenosis: the Currarino triad. 205 99

Of 18 pregnancies in 11 renal transplant recipients, three were terminated and in the remaining 15 (in 8 women) there were 10 live births (including one set of twins), five intrauterine deaths, and one spontaneous abortion. Graft function deteriorated in six women, from obstruction of the transplanted ureter in two, recurrent glomerulonephritis in two, rejection in one, and pelvi-ureteric junction obstruction in one. Hypertension worsened or developed in all but one of the pregnancies and proteinuria appeared in eight. Of the 10 live births only one reached 38 weeks gestation (mean 35 weeks) and four neonates were small for gestational age. One infant died early from intraventricular hemorrhage and hyaline membrane disease, one fetus had hydrocephalus, and the others were normal. Factors associated with a poor fetal outcome were deterioration in graft function during pregnancy, pre-existing hypertension, or the development of hypertension before the third trimester.
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PMID:Problems associated with pregnancy in renal allograft recipients. 266 32

Hydrocephalus is a frequently encountered problem in infancy and is most commonly treated by placement of ventriculoperionteal (VP) or ventriculoatrial (VA) shunts. Other sites for insertion of the distal shunt have included the stomach, ureter, and fallopian tube. This report describes an experience with ventricular gallbladder shunts (VGB) in 25 children performed from 1970 to 1985. There were 13 girls and 12 boys ranging in age from 6 months to 16 years. Diagnosis included meningomyelocoele (7), congenital hydrocephalus (7), postmeningitic complications (5), intracranial tumor (4), and intraventricular hemorrhage (2). Indications for operation included VP shunt infection (15), massive ascites following VP shunt (3), VA shunt infection (4), and distal shunt malfunction due to fibrinous adhesions or cysts (secondary to infection; (3). Three patients had early shunt failure due to proximal obstruction (2) and gallbladder atony (1). Shunt revision was required in two and the atony was successfully treated with cholecystokinin in one. Fourteen shunts remain in place, two patients have been lost to follow-up, and three children died from unrelated causes. Seventy percent of the 20 patients available for long-term follow-up have functional shunts in place. The VGB shunt procedure remains an attractive alternative for patients with hydrocephalus in whom intraperitoneal and intravascular shunts are no longer feasible.
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PMID:Ventricular gallbladder shunts: an alternative procedure in hydrocephalus. 361 54

The urinary tract has been used previously for shunting of cerebrospinal fluid. However, nephrectomy has always accompanied the procedure, making it less appealing despite adequate relief of hydrocephalus. By using reimplantation of the ureter and a psoas hitch we have performed the first reported ventriculoureterostomy without nephrectomy. To date the results in 3 patients have been excellent from a neurosurgical and urologic standpoint.
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PMID:Ventriculoureteral shunt for hydrocephalus without nephrectomy. 735 24

A very rare case of cloacal exstrophy associated with other multiple congenital anomalies is presented.Other congenital anomalies included exomphalos; myelomeningocele; microcolon with blind end and rectal agenesis; absence of external genitalia, appendix, pubic arch with separation of rami, left kidney, and ureter; duplication of the uterus and vagina; lacunar defect of cranial bones; Arnold-Chiari malformations; and hydrocephalus.The literature on this subject is reviewed.
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PMID:Multiple congenital anomalies with cloacal exstrophy. 746 98

Surgical relief of hydrocephalus is achieved mainly with ventriculoperitoneal or ventriculoatrial shunting. In some patients, frequent reoperations are required because of infection, obstruction, or other complications, thus subjecting them to excessive operative morbidity and risk of neurological damage. One option that has been rarely addressed in recent years is drainage of cerebral spinal fluid into the urinary tract by way of a ventriculoureteral shunt. Patients who have endured multiple revisions of standard cerebral spinal fluid shunts may benefit from a relatively lower frequency of reoperation following ventriculoureteral shunting. There are several complications peculiar to this procedure, however, including ascending infection from the bladder, proximal shunt migration out of the ureter or distal migration into the bladder, failed urinary diversion, and electrolyte disturbances associated with volume depletion. Long-term follow-up of patients with ventriculoureteral shunts has not been reported. We describe the course of 4 patients successfully treated with ventriculoureteral shunts for an average of more than five years per patient. Although all eventually required reoperation, the frequency of reoperation with the ventriculoureteral shunts was markedly lower than with the standard shunts in these patients. Ventriculoureteral shunting should be considered for cerebral spinal fluid drainage in selected patients with multiple failures of standard shunts, provided the bladder is a low pressure reservoir with no urine infection.
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PMID:Long-term follow-up of ventriculoureteral shunts for treatment of hydrocephalus. 836 27

Hydrocephalus is a prevalent pediatric problem, and ventricular peritoneal shunting is the preferred procedure for surgical treatment. A system may become dysfunctional if the distal end of the catheter fails to drain because of intraabdominal adhesions, cerebral spinal fluid cysts, or peritonitis. Then additional revisions become necessary. Pediatric surgeons can come to the aid of their neurosurgical colleagues by placing the distal end of the catheter into the pleural space, cardiac atrium, ureter, stomach, or fallopian tube. These have all met with limited success. The ventricular cholecystic shunt has been used in some difficult-to-manage cases of hydrocephalus, and our experience with the procedure is described. In the complicated patient, we have had success placing the distal catheters in the gallbladder. Between 1986 and 1995 sixteen patients have had ventriculocholecystic shunts placed. The mean age was 10.8 years, ranging from 0.9 to 23 years. Prior shunting procedures averaged 4.1 (range 0 to 11). The follow-up period ranged from 4 weeks to 7.5 years, with a median follow-up of 3 years. No shunts were removed or revised specifically because of gallbladder complications such as infected bile, stones, or distal catheter occlusion. Seven patients experienced other shunt-related problems that were not caused by distal catheters, ie, staphylococcal infections and intracerebral problems. Eleven of the original shunts are still functioning. Two patients died of causes not related to the ventriculoperitoneal shunt. Because of their simplicity of placement, low incidence of complication, and similarity to ventriculoperitoneal shunts, the ventricular cholecystic shunt should be considered when other sites are unattainable.
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PMID:Ventricular cholecystic shunts in children. 904 18

Recent advances in pediatric surgery have been made in several fields. Hydrocephalus is again being treated by draining the cerebrospinal fluid into either the ureter, the mastoid antrum or the peritoneal cavity. Funnel chest should be corrected surgically. Congenital atresia of the esophagus is best treated by a one-stage operative repair. Patent ductus should be closed. Operations are available for cyanotic children. Intussusception is again being treated by barium enema in selected cases. Megacolon can be benefited by surgical procedures, which now are directed at the distal spastic segment rather than the proximal dilated segment.
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PMID:Advances in pediatric surgery. 1300 76

Carcinomatous meningitis from urothelial carcinoma of the bladder and ureter is rare. A 77-year-old man with invasive bladder cancer and right ureter cancer had been treated with 3 courses M-VAC (methotrexate, vinblastine, epirubicin, cisplatin) chemotherapy. After chemotherapy we performed radical cystectomy and right nephroureterectomy (ileal-neobladder) (TCC, G3, pT3, N0, M0). Sixteen months after operation, patient complained of anorexia, muscular weakness, stiff neck. CT of chest and abdomen, and bone scintigraphy showed no metastasis. Brain CT and MRI showed hydrocephalus but no evidence of parenchymal metastasis. Because we suspected carcinomatous meningitis, we performed lumbar puncture. Cerebrospinal fluid cytology revealed class V (urothelial carcinoma). Patient died 6 days after diagnosis of carcinomatous meningitis.
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PMID:[Carcinomatous meningitis from urothelial carcinoma of bladder and ureter: case report]. 1562 93

Mutations in the X-chromosomal gene (L1CAM) for cell adhesion molecule L1 are associated with a heterogeneous group of conditions that include agenesis of the corpus callosum, hydrocephalus, spastic paraplegia, adducted thumbs and mental retardation (L1-spectrum disease, CRASH or MASA syndrome). Although L1CAM is expressed during renal development and L1cam-deficient mice have congenital malformations of the kidney and the urinary tract, L1CAM mutations have not been associated with renal anomalies in men. We report on a boy with prenatally detected hydrocephalus. After his birth, bilateral duplex kidneys and ureters, with a unilateral mega-ureter serving a hydronephrotic upper pole, as well as agenesis of the corpus callosum, adducted thumbs, spasticity, and mental retardation were recognized, fulfilling the criteria of an L1-spectrum disease. Genetic testing of the patient and his mother identified a 2 bp deletion in the invariant splice consensus sequence of intron 18 of L1CAM, predicting a largely truncated or absent protein. At the age of 9 years, 7 years after heminephrectomy, the boy has normal renal function. This observation suggests that patients with L1CAM mutations may have renal abnormalities as seen in the L1cam-deficient mouse model. L1CAM might, therefore, also be considered a possible candidate gene for renal malformations.
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PMID:L1CAM mutation in a boy with hydrocephalus and duplex kidneys. 1729 22


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