Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033377 (prolapse)
 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From May 1985 through July 1987, 22 patients underwent Kock continent ileal reservoir for urinary diversion. There were 19 males and 3 females, between 38 and 82 years old (mean age 63.1 years). A one-stage radical cystectomy and Kock pouch construction were performed in 21 patients. One patients was converted from standard ileal conduit to this new reservoir. The keys to success of the Kock pouch are creation and maintenance of the nipple valve to prevent reflux and to ensure continence. Mesenteric fat is removed with CUSA for 8 cm along the afferent-efferent limbs of the pouch and exclusion of mesentery is limited for only 3-4 cm. This important modification will ensure adequate ileal intussusception and vascular supply to the valves. To prevent eversion and prolapse, the nipple valve is anchored to the wall of reservoir. A strip of sauvage filamentous Dacron serves as a collar to fix the afferent-efferent limbs to the pouch. There were 2 postoperative deaths and two major early complication: 1 acute renal failure and 1 intestinal fistula, both of which were treated conservatively. Late complications occurred in 6 patients. Of these 6 patients, 1 required reoperation and revision of the continence valve mechanism and 1 required hospitalization for entero-pouch fistula. Serum electrolytes and vitamin B12 remained normal in all patients. Patients perform self-catheterization every 4-6 hours during the day and once at night for volumes ranging up to 1,000 ml. The end result in 19 of 20 patients was excellent.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Clinical experience with the Kock continent ileal urinary reservoir]. 337 20

A new inherited neuromuscular disease was identified in 4 patients (1 male, 3 females), offspring of consanguineous marriages, belonging to the same kindred. The proband was a 24-year-old female with history of ptosis and ophthalmoplegia since childhood and progressive intestinal pseudo-obstruction for the last 4 years of her life. A sural nerve biopsy showed axonal and demyelinating neuropathy. Muscle biopsies of pectoral and gastrocnemius revealed myopathic alterations with marked variation in muscle fiber size, atrophy of both fiber types and normal mitochondria. An upper gastrointestinal study showed barium in the stomach after 8 h and jejunal diverticula. Tests for absorption of fat, protein, carbohydrate, folic acid and vitamin B12 were normal. Serum levels of vitamin A and lipoproteins were also normal. The patient underwent partial gastrectomy and gastrojejunostomy. Postoperatively, she developed severe pancreatitis, sepsis, peritonitis and expired. Tissue samples from the proband and from her brother, revealed normal mucosa, but degeneration of smooth muscle of the stomach and small intestine. The myenteric plexus and vagus nerves were normal. The biochemical studies of contractile proteins (myosin, actin, tropomyosin) in the fresh and cultured smooth muscle cells of the proband obtained at the time of gastrectomy showed a 50-75% decrease in the synthesis of different contractile proteins. Turnover of contractile proteins and synthesis and turnover of collagen showed normal values. The reduction in synthesis of contractile proteins may account for the weak peristalsis and be a factor in the pathogenesis of the intestinal pseudo-obstruction.
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PMID:Inherited ophthalmoplegia with intestinal pseudo-obstruction. 668 98