Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the period from 1974 to June 2000 we used the straight ileo-anal Soave pull-through to treat 42 patients (24 affected by total colonic aganglionosis [TCA], 10 with ulcerative colitis and 8 with familial polyposis). The aim of this paper is to show that this operation, associated with total colectomy, is highly recommended, causing a lower number of complications when compared to the various "reservoir" techniques. The mean age of the 24 patients with TCA at the time of the pull-through was 2.8 years; in the ulcerative colitis group, it was 14.3 years and in the familial polyposis group 27.2 years. We always used an ileo-anal deferred anastomosis and never performed temporary loop-diverting ileostomy at the time of the pull-through. In the TCA patients we had no immediate or long-term serious post-operative complications: ileal adaptation, after a frequency of 10 - 12 liquid stools a day, showed a gradual, constant and in some cases amazing improvement in all children. Two years after surgery, the mean stool frequency was 3.6 per 24 hours with no significant differences between the 3 main groups; only 4 children still presented with occasional soiling. After pull-through, all children showed normal growth curves in the long term. There was no malabsorption, no serious electrolyte imbalance, no perianal excoriation, no strictures or intestinal obstruction; their quality of life was considered more than satisfactory by the children's families. We have no direct experience with the various ileal "reservoir" techniques for ulcerative colitis and ileal polyposis nor with colon-sparing operations for TCA; as reported in the literature, all these surgical procedures seem to have a higher number of complications such as pelvic sepsis, pouchitis, enterocolitis, etc. compared with our series; we therefore confirm that total colectomy with the straight ileo-anal Soave pull-through is our treatment of choice, as it is simpler to perform and has fewer short- and long-term complications.
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PMID:Total colectomy and straight ileo-anal soave endorectal pull-through: personal experience with 42 cases. 1171 70

Familial amyloid polyneuropathy type I (FAP-I) is an inherited amyloidosis secondary to systemic deposition of amyloid fibrils containing mutant transthyretin (TTR) variants. The disease has a progressive clinical course and is usually fatal 10 years after its onset. TTR is mainly produced in hepatocytes, and liver transplantation (LT) has been proposed as an effective treatment for FAP-I. The aim of this study is to evaluate the results of LT for FAP-I in Brazil and analyze prognostic factors associated with survival after surgery. Twenty-four patients (median age, 36 years; range, 25 to 52 years) who underwent LT with the diagnosis of FAP-I were evaluated. Surgery was uneventful in all but six patients who died of complications of primary liver nonfunction (n = 1), cardiogenic shock (n = 1), sepsis (n = 3), and hepatic artery thrombosis (n = 3). Overall 1- and 5-year survival rates were 70% and 58%, respectively. Most patients had stabilization or improvement of symptoms after a median follow-up of 36 months (range, 14 to 82 months). Survivors had a shorter disease duration before LT (median, 6 years; range, 2 to 17 years v 9 years; range, 7 to 12 years; P =.02), greater albumin levels (median, 4 g/dL; range, 3 to 4.7 g/dL v 3.6 g/dL; range, 2.6 to 4.1 g/dL; P =.03), and greater modified body mass index scores (median, 735; range, 502 to 1,432 v 659; range, 411 to 803; P =.04) compared with nonsurvivors. However, only disease duration and albumin levels were independently associated with survival in multivariate analysis. In conclusion, LT is an effective therapy for FAP-I. Mortality after surgery is associated with poor nutritional status and long-standing disease before LT. Thus, LT should be performed as early as possible after the onset of FAP-I symptoms to avoid major disability and improve survival.
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PMID:Results of liver transplantation for familial amyloid polyneuropathy type I in Brazil. 1179 83

Classical familial amyloid polyneuropathy may have a course with progressive renal impairment. We studied 62 patients (24 males, 38 females) with FAP, transthyretin variant V30M, and end-stage renal disease (ESRD) treated with hemodialysis, all referred to a single center over a period of 11 years. Clinical course, morbidity and survival after dialysis were analyzed. Patient's mean age at first dialysis was 51.5 +/- 10.7 years, and mean duration of neuropathy was 10.2 +/- 3.8 years. The most frequent form of presentation of FAP nephropathy was nephrotic proteinuria with renal dysfunction. In the year prior to dialysis, renal function declined rapidly, and fluid overload was the main indication to initiate treatment. The presence of decubitus ulcers, significant disability, venous catheter for definitive vascular access for long-term treatment, and permanent bladder catheter, were related to death during the first year of dialysis. The mean duration of renal replacement therapy was 21 months, with a 54.5% one year, and 38.4% two year treatment survival. However, when the duration of neurological symptoms at first dialysis exceeded 10 years, survival was significantly lower. Infections, (41% were decubitus ulcers with sepsis) were the cause of early, as well as late mortality. Early creation of vascular access for hemodialysis, surveillance of skin wounds, and intervention on neurogenic bladder are essential to improve the prognosis of ESRD in FAP.
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PMID:End-stage renal disease and dialysis in hereditary amyloidosis TTR V30M: presentation, survival and prognostic factors. 1518 96

Restorative proctocolectomy is now the elective surgical procedure of choice for most patients with ulcerative colitis or familial adenomatous polyposis. There are four causes of failure including acute and chronic sepsis, poor function for mechanical or functional reasons, mucosal inflammation (including pouchitis and retained rectal mucosa) and neoplastic transformation. Failure rates themselves range from 5% to nearly 20%. Followed over a period of 20 years, the failure rate can be summarised approximately as 5% at five years, 10% at ten years and 15% at 15 years.
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PMID:Salvage surgery after restorative proctocolectomy. 1577 Dec 82

Colorectal carcinoma emergencies during pregnancy are exceptionally rare. Three women 38, 31 and 36 years old, in the third trimester of gestation received treatment, respectively, for acute abdomen due to perforation of rectal carcinoma, ileus due to a sigmoid tumor, and deep venous thrombosis (DVT) from a cecal tumor compromising the right iliac vein. In the first two patients urgent cesarean sections were carried out with Hartmann's procedure and a loop colostomy was performed to resolve the ensuing intraabdominal sepsis and ileus, respectively. In the third patient, a cesarean section was carried out to treat the underlying DVT more aggressively, while right colectomy was postponed for three weeks. Restoration of the alimentary tract was achieved two months later in the first case, while in the second and third cases total colectomy due to familial polyposis and right colectomy were performed three weeks after the cesarean section. An overview of the clinical features, diagnostic pitfalls and therapeutic approaches to manage complications of colorectal cancer during pregnancy are discussed.
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PMID:Colorectal cancer emergencies during pregnancy case reports. 1700 43

The short bowel syndrome (SBS) can result from a variety of conditions, including postoperative complications and malignancy. Continence-preserving operations are generally performed for either ulcerative colitis (UC) or familial polyposis (FAP). These procedures can be associated with high morbidity and the potential for future malignancy. Our aim was to determine the causes and consequences of SBS in patients undergoing these procedures. Twenty-four patients (12 men and 12 women) 18 to 64 years of age were identified with SBS after continence-preserving procedures. Eighteen had pelvic procedures, and six had continent ileostomies. All SBS patients had a proximal ostomy. Remnant length measured <60 cm in five patients, 60-120 cm in ten patients, and >120 cm in nine patients. Overall 13 patients required long-term PN. Four FAP patients with desmoid tumors died. One patient with UC underwent intestinal transplant and expired. Follow-up ranges from 6 to 192 months. Overall 14 patients had UC, nine had FAP, and one had functional disease. Eight patients with an initial diagnosis of UC had subsequent Crohn's disease necessitating further resection and pouch excision. Eight patients (five with UC, two FAP, and one with functional disease) had postoperative complications, including obstruction or mesenteric ischemia requiring resections. One UC patient developed adenocarcinoma in a continent ileostomy. Seven of the nine FAP patients required resection for desmoid tumors. Six of these underwent resection alone. Three died at 10, 11, and 13 months after SBS from liver failure and sepsis while awaiting transplant. One patient has recurrent desmoid at 30 months, another is alive and well at 48 months, and the other patient, who was not a transplant candidate, died from an unrelated cardiac operation at 23 months. A single patient underwent resection with simultaneous multivisceral transplantation. SBS can develop after continence-preserving procedures. This occurs with inflammatory bowel disease when unsuspected Crohn's disease is present or complications occur. SBS related to desmoid tumors has a poor prognosis in patients undergoing resection alone. A more aggressive approach to intestinal transplantation in these patients may be warranted.
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PMID:Short bowel syndrome after continence-preserving procedures. 1796 30

A 38-year-old woman who had familial adenomatous polyposis was admitted to the intensive care unit with an episode of severe sepsis 5 days after undergoing a pancreas-preserving duodenectomy. Laparotomy with removal of an intra-abdominal abscess, followed by closed postoperative continuous lavage for 10 days, was performed. During two courses of planned tracheal extubation, the patient developed an acute lung injury, making a reintubation necessary. In both events, the patient received small doses of continuous morphine before the extubation. Morphine may induce the development of an acute lung injury in patients, whereas the exact pathophysiologic and pharmacologic mechanisms remain unclear.
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PMID:Morphine-induced acute lung injury. 1861 31

Ileal pouch-anal anastomosis (IPAA) surgery preserves fecal continence for improved quality of life in patients who require proctocolectomy for treatment of severe bowel diseases such as inflammatory disease and familial adenomatous polyposis. In IPAA surgery, an ileal reservoir, or pouch, is created and anastomosed to the anal canal. Awareness of the surgical technique and the postoperative anatomy of the IPAA is important to identify complications at computed tomography (CT), magnetic resonance (MR) imaging, and fluoroscopy. Complications include anastomotic leak, abscess, pouchitis, venous thrombus, pouch fistula, and stricture. Leaks from the blind end of the pouch and the pouch-anal anastomosis often result in pelvic abscesses, which may require ultrasonography- or CT-guided drainage; judicious catheter management can help improve clinical outcomes and avoid excessive imaging. Pouchitis may be identified by the presence of a thickened enhancing pouch wall and associated inflammatory changes and lymphadenopathy. The venous system must be scrutinized for thrombi secondary to surgical manipulation and sepsis. Fistulas are likely because of the presence of chronic inflammation or infection and may be seen at MR imaging, CT, or fluoroscopy. Strictures appear as areas of focal luminal narrowing with proximal dilatation, which can lead to obstruction. To avoid repeated exposure to radiation, MR imaging may be performed in patients who must undergo frequent imaging.
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PMID:Ileal pouch-anal anastomosis surgery: imaging and intervention for post-operative complications. 2008 95

Coloproctectomy with ileo-anal anastomosis (CP-IAA) has been in use for 30 years. This intervention is the standard technique when surgery is indicated for familial adenomatous polyposis (FAP) and for ulcerative colitis (UC). Although the surgery is safe with mortality of less than 1%, it is associated with a morbidity of 18-70%. We thought a literature review about long-term complications would be enlightening. Pouchitis is the most common complication; it occurs in 70% of patients over 20 years follow-up; small bowel obstruction affects 25% of patients and pelvic sepsis occurs in 20-30% within 10 years. CP-IAA can impact the patient's sexual life due to erectile and ejaculatory dysfunction, dyspareunia, and incontinence of stool during sexual intercourse. Nevertheless, patients with long-standing UC describe an overall improvement in their sexual function after surgery. The failure rate varies from 3.5 to 15%; major causes of failure are sepsis, unrecognized Crohn's disease, and poor functional results. Cases of dysplasia and cancer have been reported in the reservoir, but more particularly when there is retained colonic glandular mucosa. The transitional zone should be monitored whenever there are risk factors for colon neoplasia. The relatively high morbidity of CP-IAA should not overshadow the good functional results of this technique.
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PMID:Ileal reservoir with ileo-anal anastomosis: long-term complications. 2083 85

Proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the established surgical therapy for familial adenomatous polyposis (FAP) and refractory ulcerative colitis (UC). Despite general patient satisfaction with preserved fecal continence, this procedure is associated with a significant long-term morbidity approaching 70% after 10 years, and with a non-negligible rate of pouch failure leading to removal and permanent ileostomy. Following a concise description of the surgical technique, the normal imaging appearance of the ileal "pouch" reservoir at pelvic CT and MRI is explained. Since awareness of their imaging appearances is needed for a correct diagnosis, we discuss and illustrate common and unusual pouch-related complications, including pouchitis and irritable pouch disease; anastomotic leakages and pelvic abscess collections; fistulas involving the ano-perianal region, urinary bladder, vagina, perineal skin, and subcutaneous planes; anal stenosis and small-bowel obstruction. In our experience, pelvic contrast-enhanced MRI has proven invaluable for the diagnostic assessment of patients with suspected pouch-related complications, allowing differentiation of uncomplicated pouchitis from pelvic sepsis, the latter requiring aggressive therapy and possible even in patients with normal endoscopic findings.
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PMID:Ileal pouch and related complications: spectrum of imaging findings with emphasis on MRI. 2129 55


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