Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between January 1985 and June 1995 a total of 12 patients (9 female, 3 male) underwent total reconstruction of the lower urinary tract using gastric tissue. Their mean age was 10 years (range, 5-25 years). Total gastric bladder substitution was performed in seven patients, whereas five other patients had composite continent reservoirs (stomach plus bowel) created. The diagnoses were cloacal exstrophy, classic bladder exstrophy, bladder rhabdomyosarcoma, bilateral ectopic ureters, and VACTERL association. The mean follow-up period was 4.5 years (range, 16 months to 9 years). The average bladder capacity was 309 ml, and the compliance averaged 12.9 ml/cmH2O. Continence was achieved in all patients, but the continence mechanism often required revision. Renal deterioration was noted only in one patient due to an obstruction at the site of a transureteroureterostomy. The complications included hyponatremic, hypochloremic metabolic alkalosis (two patients); prolapse of the neovagina (one patient); a ureterovesical junction obstruction (three patients); and revision of the neourethra or Mitrofanoff (six patients). Revision of the gastric tube used as the catheterizable channel was performed in three instances. Lengthening of the tube, including nippling in one instance, corrected persistent incontinence. Distal stenosis of a gastric tube required several plastic procedures for correction. A ureteral Mitrofanoff developed a distal stricture necessitating revision. Persistent leaking of an appendiceal Mitrofanoff was corrected with reimplantation. A tapered ileal neourethra required a tunneling procedure followed by Teflon injection to provide urinary continence. Complications seen most often involved the continence mechanism of these gastric neobladders or composite urinary reservoirs. Persistence, patience, and perseverance led to total urinary continence and adequate reservoirs for urine storage in all patients.
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PMID:Continent gastric pouch. 873 Nov 28