Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors present a review of 431 children biopsied and studied with the following histochemical and immunohistochemical techniques: 1) acetylcholinesterase activity; 2) alphanaphthylesterase activity; 3) S-100 protein immunohistochemical technique; 4) glyoxylic acid method. Two hundred forty-eight patients of our series presented different forms of dysganglionosis, 12 of them (4.8%) presenting neuronal intestinal dysplasia type B. In 7 cases, NID type B was diffuse, whereas in 5 recto-colonic NID type B was confined to the splenic flexure. Male:female ratio was 9:3. Familial recurrence was present in 2 of the 12 cases of our series, affected by severe neuronal intestinal dysplasia extended to the small intestine, associated with intestinal malrotation and short bowel syndrome. Four of the 7 cases of diffuse NID type B and 2 of the 5 cases of rectocolonic NID type B were surgically treated. Three patients with diffuse NID died from sepsis within the 2nd year of life. This study confirms that NID type B is a form of dysganglionosis which can be diagnosed in a Mediterranean country if histochemical techniques are applied in the study of a large series of constipated and pseudo-Hirschsprung patients. From a pathogenetic point of view, the authors compared the histochemical findings of biopsies from their series of NID patients with those of recto-colonic biopsies from patients with MEN II B syndrome. The similarity of GI symptoms in MEN II B and NID pediatric patients suggests that the two disorders could be the result of mutations affecting the same domain of the RET proto-oncogene.
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PMID:Neuronal intestinal dysplasia: clinical experience in Italian patients. 785 85

Intestinal fistulae are an uncommon but serious complication of pelvic exenteration. To characterize factors leading to fistula formation and to define optimal management of this complication, we reviewed 533 cases of patients who underwent pelvic exenteration at the University of Texas M. D. Anderson Cancer Center between 1957 and 1990. Forty-two of those patients developed an intestinal fistula following total (n = 29), anterior (n = 12), or posterior (n = 1) exenteration which was not tumor related. Prior to routine pelvic floor reconstruction, the fistula rate was 16%. With the advent of omental pedicle grafts and gracilis flaps, the rate decreased to 4.5%. The fistulae described included those from the small bowel to the pelvic cavity (n = 15) or the neovagina (n = 8), and from the large bowel to the neovagina (n = 8). Complex fistulae were noted in 11 patients. Early fistulae, those that developed during initial hospitalization, occurred in 25 patients and were mainly related to infectious complications. Twenty-three patients underwent attempted surgical repair of fistulae. Eleven died during their hospitalization of sepsis, recurrent wound complications, or fistula. Late fistulae, those that developed after discharge, occurred in 17 patients and were mainly related to delayed healing. Early and late fistulae did not differ in location. Only two patients with late fistula formation died from complications of therapy. Significant long-term morbidity, however, included short bowel syndrome. Based on our review, we conclude the following: (1) Pelvic floor reconstruction, careful attention to surgical technique and aggressive treatment of infections reduces the risk of early fistula formation; (2) in cases associated with significant infection, treatment should be surgical; and (3) in stable patients, conservative management with hyperalimentation and bowel should be considered.
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PMID:Intestinal fistulae formation following pelvic exenteration: a review of the University of Texas M. D. Anderson Cancer Center experience, 1957-1990. 789 87

Small bowel transplantation (SBT) would, in theory, be the treatment of choice for patients suffering from the short bowel syndrome. Although SBT has been done with a considerable degree of success in some centers [36, 145], it is by no means an established or widely applicable therapy for those with short bowel syndrome. The small bowel is unique among vascularized organ grafts because it not only elicits a vigorous rejection reaction but is also capable of inducing graft-versus-host disease (GVHD). Rejection of the graft does not only lead to loss of function but also to bacterial translocation. The risk of fatal sepsis is aggravated by the immunosuppression given to prevent rejection. Here, the history of SBT is described, and recent developments in experimental and clinical SBT, as well as future prospects for this theoretically optimal treatment modality for patients dependent on total parenteral nutrition (TPN) for life, are outlined.
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PMID:Small bowel transplantation: an overview. 811 3

The aim of this retrospective study was to determine whether total parenteral nutrition-related liver disease was improved by intravenous antibiotics given for systemic sepsis. Liver function tests were performed 1 month before, during and 1 month after one episode of sepsis treated for 4 weeks (mean, range: 2-12), with systemic antibiotics, in 12 patients receiving parenteral nutrition for 13 months (mean, range: 1-71) for short bowel syndrome in 10 of them. Cholestatic liver disease appeared in all during nutrition (mean serum alkaline phosphatase activity > 4 N). Liver test abnormalities observed at the beginning of antibiotics treatment were not significantly different from those observed 1 month before sepsis. Antibiotic administration was followed by a significant decrease (P < or = 0.03) in serum activities of alkaline phosphatases, ALT and AST and bilirubinemia of 38, 41, 23 and 47%, respectively. These results support the concept that parenteral nutrition-associated cholestatic liver disease may be related to intestinal bacterial overgrowth and suggest that it may be improved by intravenous antibiotherapy.
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PMID:[Total parenteral nutrition-related cholestatic hepatopathy, is it an infectious disease?]. 818 92

Home parenteral nutrition (HPN) was used for the treatment of 18 patients with chronic intestinal failure (CIF): short bowel syndrome (9), extensive intestinal disease (4), motility disorder (4), entero-enteric fistulas (1). The underlying diseases were: chronic inflammatory (7), mesenteric vascular (4), scleroderma (2), pseudo-obstruction (2), malignancy (2), radiation enteritis (1). HPN was more effective on protein-calorie nutritional status than on fluid and electrolyte balances. About two-thirds of the patients achieved full or partial social rehabilitation. During the 6 months before HPN, there were 20 hospitalizations (mean stay: 55 days). During HPN (mean length of treatment: 22 months/patient) there were 16 hospitalizations (mean stay: 22 days), 8 of which were caused by HPN complications (sepsis and deep vein thrombosis; overall incidence of catheter-related complications: 0.411 per patient-year). Bone demineralization, liver abnormalities and biliary stones developed, respectively, in 57%, 28% and 11% of the cases. The underlying intestinal condition played a role in their pathogenesis. The annual cost of HPN ranged from 40 (Hospital Pharmacy Service) to 80 (commercial firm) million lire per patient. To sum up, HPN improves the nutritional status and the quality of life of patients with CIF, and the risk of complications is acceptable. The medical and social advantages are considered to offset the cost of the technique.
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PMID:Home parenteral nutrition for the management of chronic intestinal failure: a 34 patient-year experience. 828 74

Six patients with extreme short bowel syndrome (4.2 +/- 4.9 cm of residual small bowel) were provided home parenteral nutrition (HPN) for 14,397 days. The average age at onset of HPN was 38 years (18-64 years). Patients maintained body weight at 97% of ideal (86-112%) with mean serum albumin of 3.7 +/- 0.6 g/dL (normal 3.5-5.8 g/dL), serum transferrin of 341 +/- 104 mg/dL (normal 200-400 mg/dL), and mean serum pre-albumin of 27.5 +/- 12.6 mg/dL (normal 16.6-43 mg/dL). Hospital admission for HPN-related complications was required 10.3 times/patient for a total of 864 hospital days and was catheter related in 71% of episodes. Catheter sepsis occurred once per 436 catheter days and required catheter removal in only 33% of instances. Five patients were able to resume an oral diet, five had returned to work or school, and three were married with family. HPN allowed return to a quality productive life with acceptable morbidity following catastrophic massive bowel resection.
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PMID:Home parenteral nutrition after near total enterectomy. 840 83

From 1985 to 1994, home parenteral nutrition (HPN) was used as the method of feeding nine pediatric patients. Indications for HPN included congenital or acquired short bowel syndrome. Crohn's disease, chronic intractable diarrhea, chronic idiopathic intestinal pseudo-obstruction and Hirschsprung's disease. During the period, two patients died; one of sepsis and the other from hepatic failure. Three of the remaining patients have since moved on to oral feeding, but four patients continued on HPN. The majority of these patients have attained a normal weight and height for age while receiving HPN. All patients were fed via an implanted silicone catheter. Catheters were removed and replaced due to complications including nine episodes of infection, four episodes of occlusion, three episodes of breakage and two episodes of dislodgement. Catheter-related sepsis was the most common cause of morbidity and hospital readmission in patients receiving HPN, but was acceptably rare. Staphylococcus aureus was the most frequently cultured organism from either the catheter tip or the blood. HPN is a relatively safe feeding method for patients who would otherwise remain hospitalized for prolonged periods on parenteral nutrition for permanent or prolonged intestinal failure.
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PMID:Home parenteral nutrition in children. 864 94

The incidence, presentation, significance, and outcome of infants with internal enteric fistula formation secondary to necrotizing enterocolitis (NEC) were examined. Of 130 infants with NEC treated during a 7-year period, an enteric fistula developed in five (4%). The gestational age of these patients (3 boys, 2 girls) ranged from 25 to 40 weeks and their birth weight ranged from 800 to 3,460 g. Two had Down's syndrome. Plain abdominal radiographs showed widespread intramural gas in all, and portal vein gas in two. Four patients required early laparotomy, which confirmed extensive intestinal necrosis; a diverting jejunostomy or ileostomy was constructed in three, and the abdomen was closed with drainage in one. Fistulas were diagnosed by contrast radiology between 16 and 51 days after the onset of NEC, and were jejunocolic (2), ileocolic (2), and colocolic (1). They were associated with enteric stricture(s), an inflammatory mass, and clinical signs of intermittent sepsis. One infant with an ileocolic fistula died of sepsis before definitive surgical treatment. Of the four who underwent surgery, two survived after limited intestinal resection, but one of the two with short bowel syndrome died. Enteric fistula formation is a rare complication of NEC. Typically it occurs with colonic stricture(s) and is associated with signs of incomplete bowel obstruction and intermittent sepsis. Resectional surgery is successful, but there appears to be a significant risk of short bowel syndrome.
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PMID:Enteric fistulas and necrotizing enterocolitis. 888 99

Progressive liver failure in parenteral nutrition (PN)-dependent children with short bowel syndrome carries significant morbidity and mortality. The authors retrospectively reviewed 47 consecutive patients with short bowel syndrome diagnosed from October 1985 through October 1995. All patients were treated according to a protocol designed to promote intestinal motility and discourage bacterial translocation. Elements of the protocol included the use of taurine, vigilant prevention and aggressive treatment of sepsis, meticulous catheter care, early PN cycling, appropriate enteral feeding, and measures designed to inhibit gastrointestinal bacterial translocation, especially gram-negative rods. Complete blood counts and serum liver function studies were compiled from both clinic visits and hospital admissions for each patient every 3 to 6 months while they were on PN. Three patients were lost to follow-up after they had moved out of state. The length of time on PN ranged from 3 months to 9.4 years with an average of 2.2 years. Elevated aspartate aminotransferase (AST), alanine aminotransferase (ALT), and glutamyltransferase (GGT) were present in 82%, 66%, and 84% of patients, respectively. Alkaline phosphatase was elevated in 58% of patients. Eight patients (18%) are still on PN, and 31 (70%) have been weaned off PN. Five patients have died (11%). Three patients (7%) developed cholecystitis requiring cholecystectomy. No patients developed progressive liver failure. These results suggest that PN-related liver failure may be prevented in most patients with short bowel syndrome. Specific measures to prevent PN-related cholestatic jaundice need further investigation.
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PMID:Prevention of liver failure in parenteral nutrition-dependent children with short bowel syndrome. 909 21

Carnitine CAR) plays an important role in the beta-oxidation of fatty acids. Less attention. however, has been paid to CAR compared to other nutrients even in total parenteral nutrition (TPN). To examine CAR metabolism during TPN and the effect of simultaneous oral L-CAR supplementation on CAR levels, the blood CAR level was measured in a 3-year-old boy receiving long-term TPN because of short bowel syndrome. Both the total and acyl CAR in the serum were evaluated under various nutritional conditions including oral supplementation of L-CAR. Low CAR concentrations were observed especially when lipid containing TPN regimens were in place. Oral L-CAR supplementation was not sufficient to restore the low CAR levels in the present index patient even when the dose was increased to 120 mg/kg in accordance with the result of the L-CAR absorption test that revealed poor intestinal absorption of this nutrient. Moreover, a markedly low CAR level was measured during the onset of sepsis in the patient, and the blood CAR was depleted when lipid metabolism was activated by lipid loading or sepsis. To date, the late effects of CAR depletion on child growth have not been well examined. It is recommended that the blood CAR level be maintained at normal levels before any prominent manifestations of the deficiency have developed. The intravenous administration of CAR appears to be necessary to supply a sufficient amount of CAR for patients with severe malabsorption.
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PMID:Carnitine depletion during total parenteral nutrition despite oral L-carnitine supplementation. 914 Dec 53


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