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)

Many things have changed in our management of the urinary tract of children and young adults. Important contributions from many sources have resulted in the refinement of therapy and the progression from diversion to undiversion and reconstruction. Primary urinary diversion is now uncommon. Patients with exstrophy, for example, are treated with primary bladder closure as newborns, and newborns with valves often are treated with primary valve ablation. Children with neurogenic bladder dysfunction resulting from myelodysplasia are rarely diverted, but are started at an early age with ICC. It is hoped that in future the contents of this article on diversion techniques and undiversion will be of historical interest, however. Note: The editors also have found the use of the Mitrofanoff procedure, utilizing a nonrefluxing tunnel and a catheterizable stoma made out of the ureter or appendix, to be a very valuable addition to the reconstructive surgeon's armamentarium. We also feel that use of detubularized bowel for either bladder augmentation or replacement provides more efficient storage capability. Occasional reports of extremely significant diarrhea after removal of the ileocecal segment from the intestinal tract have been reported in children with myelodysplasia.
...
PMID:Pediatric urinary diversion and undiversion. 365 9

A total of 14 women and 6 men 19 to 39 years old (mean age 27 years) with myelodysplasia underwent undiversion 8 to 29 years (mean 16) after ileal conduit diversion. The main reasons for diversion were incontinence in 17 patients and failed ureteral reimplants in 3, and those for undiversion were a desire for an improved quality of life in 16, increasing hydronephrosis in 4 and stomal problems in 3. Preoperative assessment included upper and lower tract imaging, and video urodynamics. Operations on the ureters included reimplantation into an intussuscepted nipple valve in 8 patients, tunneled reimplants into a sigmoid augmentation in 3 and the ureters joined to either the bladder or lower ureter without interposing bowel in 9. All reimplantations were done with nonrefluxing techniques. A total of 18 patients underwent bladder augmentation and 2 women in whom cystectomy was performed for pyocystis underwent substitutions. Simultaneous continence procedures in 18 patients included trigonal tubularization in 2, artificial sphincter implantation in 2, a bladder neck sling in 5 or bladder neck tapering and a sling in 9. The patients were followed for a mean of 69 months (range 21 to 133). Eight patients required reintervention within 1 year for problems, such as anastomotic leak in 1, bladder neck obstruction in 1, incontinence in 1, artificial urinary sphincter revisions in 1 and bladder stones in 1. One patient had a recurrent renal calculus 10 years after undiversion. All patients experienced either persistence of normal upper tract appearance or improvement and/or stabilization of hydronephrosis. Mean bladder capacity was 77 cc preoperatively and 480 cc postoperatively, while mean pressure at capacity decreased from 50 to 14 cm. water with detubularized augmentation. Of the patients 17 are completely dry, 2 wear 1 pad per day and 1 has enuresis. All but 1 patient who voids with straining are on intermittent self-catheterization. All patients, on followup interviews, reported an improved quality of life without a stoma. We conclude that undiversion provides an improved quality of life and an acceptable morbidity rate. The choice of operation depends on the anatomy of the patient. We prefer a nonprosthetic type of incontinence procedure when intermittent self-catheterization is to be done. No long-term morbidity has yet been noted.
...
PMID:Urinary undiversion in adults with myelodysplasia: long-term followup. 801 64

We report a case of extramedullary myeloid cell tumor of the urinary bladder in an elderly male with a three year history of myelodysplastic syndrome (refractory anemia with excess blasts), noninvasive papillary transitional cell carcinoma of the urinary bladder, and in situ transitional cell carcinoma of the left ureter. Light microscopy demonstrated a poorly differentiated neoplasm composed of medium to large cells with eosinophilic cytoplasm. The tumor cells showed immunohistochemical expression of myeloperoxidase, lysozyme, CD15, CD68 and CD43. Bone marrow examination following cystectomy demonstrated refractory anemia with excess blasts (6-10%) and a normal karyotype. Cytogenetics, approximately 1 year after cystectomy, demonstrated a deletion of the short arm of chromosome number 12. Four years after presentation, the patient succumbed to pulmonary aspergillosis.
...
PMID:Extramedullary myeloid cell tumor of the urinary bladder in a patient with myelodysplastic syndrome. 1054 34

An 84-year-old man presented with the chief complaints of fever and disturbance of consciousness at another hospital. A sheath dilator had been inserted into the urethra instead of an indwelling catheter at the hospital. Since he had previously been treated for myelodysplastic syndrome in our Department of Internal Medicine, he was transferred to our hospital. He presented at our Department with the chief complaints of urinary difficulty and lower abdominal fullness. Kidney-ureter-bladder x-ray and computed tomography revealed the sheath dilator in the uretha and bladder. First, we performed a percutaneous cystostomy under local anesthesia. Second, we utilized optical forceps and successfully removed the sheath dilator transurethrally under intravenous anesthesia.
...
PMID:[A case of a foreign body (sheath dilator) in the urethra and bladder]. 2224 Mar 5

Concurrent emergence of nephroblastoma (Wilms Tumor; WT) and neuroblastoma (NB) is rare and mostly observed in patients with severe subtypes of Fanconi anemia (FA) with or without VACTER-L association (VL). We investigated the hypothesis that early consequences of genomic instability result in shared regions with copy number variation in different precursor cells that originate distinct embryonal tumors. We observed a newborn girl with FA and VL (aplasia of the thumbs, cloacal atresia (urogenital sinus), tethered cord at L3/L4, muscular ventricular septum defect, and horseshoe-kidney with a single ureter) who simultaneously acquired an epithelial-type WT in the left portion of the kidney and a poorly differentiated adrenal NB in infancy. A novel homozygous germline frameshift mutation in PALB2 (c.1676_c1677delAAinsG) leading to protein truncation (pGln526ArgfsX1) inherited from consanguineous parents formed the genetic basis of FA-N. Spontaneous and induced chromosomal instability was detected in the majority of cells analyzed from peripheral lymphocytes, bone marrow, and cultured fibroblasts. Bone marrow cells also showed complex chromosome rearrangements consistent with the myelodysplastic syndrome at 11 months of age. Array-comparative genomic hybridization analyses of both WT and NB showed shared gains or amplifications within the chromosomal regions 11p15.5 and 17q21.31-q25.3, including genes that are reportedly implicated in tumor development such as IGF2, H19, WT2, BIRC5, and HRAS.
...
PMID:Shared Copy Number Variation in Simultaneous Nephroblastoma and Neuroblastoma due to Fanconi Anemia. 2311 54

Although a well-known complication after transplantation, multiple non-skin malignancies within a patient are rare. We report on a kidney transplant recipient who over the course of 20 years developed breast cancer twice, a uroepithelial carcinoma, and myelodysplasia transforming into acute leukaemia. Breast cancer was treated as usual. The transitional cell carcinoma was managed with partial cyst ureterectomy with transposition of the native ureter to the graft. Withdrawal of immunosuppression followed under a "watchful waiting" regime. In conclusion, alertness is requested regarding development of malignancies. Creative solutions are necessary in the management of such patients. Under exceptional circumstances, withdrawal of immunosuppression may be an option.
...
PMID:Three solid malignancies and a myelodysplastic syndrome with a protracted course after kidney transplantation. 2459 58