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)

721 patients with liver cirrhosis were regularly screened by sonography and determination of alpha fetoprotein during a period of eleven years (1.1.1982-1.1.1993). In 137 of them hepatocellular carcinoma (HCC) was diagnosed; 28 (20.4%) had a unilocular HCC with a diameter up to 5 cm. Diagnosis was regularly verified by sonographic guided puncture, in rare cases by laparoscopy and biopsy. Beside a diameter of 5 cm the tumor should be localized at least 5 mm from the main structures in the hilus, and not in the centre of the liver; furthermore multilocular hepatocellular carcinomas and intra- and extrahepatic metastases were contraindications. Child-Pugh-classification should be A+B and urea synthesis rate at least 6 g per day. In 21 patients (75%) a portal hypertension was diagnosed; 19 (68%) had bled from esophageal varices; in case of one bleeding a therapeutic sclerotherapy and in case of recurrent variceal hemorrhage an elective shunt operation were performed. Surgical resection was carried out with controlled hypotension and temporary occlusion of the hepatoduodenal ligament. Tumor was removed by segmentectomy or bisegmentectomy and in rare cases by enucleation. There were 3 clinical deaths (10.7%); causes of death were liver failure and (2) sepsis (1). All patients could be followed up to January 1, 1993; there were 12 further deaths of liver failure, tumor recurrence or second tumor. 13 patients are still living. Thus the live expectancy for one year was 80, for 5 years 50 and for 10 years 30%. There is no doubt, that it is possible to detect hepatocellular carcinoma in patients with liver cirrhosis early by regular sonography and determination of alpha-fetoprotein.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Single hepatocellular carcinoma (phi < or = 5 cm) in liver cirrhosis. Early diagnosis and surgical removal]. 826 41

Six out of 1,612 patients operated on for biliary diseases between January 1974 and January 1985, had Caroli's disease. A summary of the clinical records is included. The treatment performed was: external drainage in a patient with sepsis, who did not improve and died; left hepatic lobectomy and biliary-jejunal diversion in two patients; right extended lobectomy in one patient; distal spleno-renal shunt in a patient with severe biliary cirrhosis and recurrent hemorrhage from esophageal varices. The last patient suffered from a combination of a choledochal cyst and dilatation of the left intrahepatic branches, and underwent complete resection of the choledochal cyst, and biliary jejunal anastomosis on a porta-hepatis using a Roux-en-Y jejunal loop. Currently, five patients are recovering and are free of symptoms. The authors discuss the indications for liver resection and resection of a choledochal cyst.
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PMID:Surgical treatment of congenital dilatation of the biliary system. 850 44

Five fatal cases of Japanese patients with type 1 Gaucher disease were studied. The causes of death included hemorrhage secondary to esophageal varices (two cases), respiratory distress (one case), hepatic failure (one case) and postoperative sepsis (one case). All of the patients had previous splenectomies, four patients had bone involvement and hepatic cirrhosis. The identified Gaucher genotypes were 1448C/1213G, 1603T/1603T, 1448C/1390G, and 1213G/1213G. The prognosis of type 1 Gaucher disease is generally good. We propose that patients with a similar clinical course and genotype to those presented in the present study should receive prompt comprehensive treatment. Patients with the 1213G mutation, pulmonary and liver involvement and a previous splenectomy should be considered as candidates for early vigorous treatment.
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PMID:Clinical and genetic studies of five fatal cases of Japanese Gaucher disease type 1. 874 12

Portal hypertension hemorrhage (PHH) due to esophageal varices (EV) rupture in nearly 80% of cases, or gastric varices (GV) in the remaining 20%, account for one-fifth of the GI tract bleedings in a general hospital. Its frequency, but more importantly, its magnitude, that causes up to one-third of the cirrhotic casualties, deserves constant update in its management. Diverse inherent patient factors influence the course of any PHH, i.e., a) remaining liver function, which is determinant; b) variceal size; c) sepsis, and d) alcohol intake. Mortality due to PHH is 27% during the first week, 41% within 6 weeks and 75% by one year of follow-up after the index hemorrhage. Time of intervention is then of utmost importance. All these key circumstances determine the ultimate course of the bleeding event, in many cases to a greater degree than the opportunity and quality of the specific treatment itself. This diversity of influential factors also jeopardizes adequate patient randomization in trials designed to compare treatment modalities. During the last decade, EV sclerosis, when compared to conventional medical treatment (non-beta blockers), has proved useful to stop active bleeding in 71 vs. 31% of cases, decreasing early and late recurrence from 70 to 40%, and direct bleeding-related mortality from 24 to 9%, even when global mortality remains around 14% per year. Disappointing as it seems, remaining liver function is the determinant issue, but a biased underestimation factor may also play a role, due to greater surgical rescue of patients in the medical branch compared to EV sclerosis, 6 vs. 28%. Minor morbidity in 14% of sclerosis treatment has given way to endoscopic ligation with similar results and less morbidity. Prophylactic EV sclerosis was prohibited by prospective controlled trials, which demonstrated significant increase in bleeding and mortality, even though there might be a subgroup of patients with large varices or endoscopic prognostic signs of bleeding that decrease by 10% their incidence expected 35%/year bleeding. GV bleeding remains a challenge; where cyanoacrylate may be needed to improve immediate control and prevent recurrence of PHH. These patients, as well as those failures to endoscopic treatment are candidates for intrahepatic portosystemic shunt (TIPS), although long-lasting control is achieved, in most cases, by liver transplant.
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PMID:[Therapeutic endoscopy in portal hypertension. When, how and how far in 1998]. 1006 21

The prognosis of GI bleeding depends upon many factors. Patients should be evaluated carefully for risk factors. To avoid complications from GI bleeding, triage should be performed promptly after patient presentation. The history and physical examination should emphasize analysis of risk factors for severe GI bleeding and mortality. Factors that increase the morbidity and mortality include: age greater than 60 years; underlying comorbidity such as pulmonary diseases, liver diseases, renal diseases, encephalopathy, or cancer; physiologic stress from major surgery, trauma, or sepsis; coexisting disease in three organ systems; low hematocrit; melena or hematochezia; and prolonged prothrombin time. Hospitalized patients who require more than five units of packed erythrocytes transfusion or who develop hypotension or hypovolemic shock are more likely to need surgery. Patients with a high APACHE II score, the presence of esophageal varices, active bleeding, or other endoscopic stigmata of recent hemorrhage are more likely to rebleed and undergo surgery. The proliferation of multivariable prognostic scales, as described herein, provides ample evidence that the goal of developing a single comprehensive multivariable scale to accurately assess severity of disease and to determine prognosis of GI bleeding is still not achieved. Yet significant progress has occurred in this field, leading to the hope of developing a universally applicable multivariable scale.
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PMID:Clinical scoring systems for determining the prognosis of gastrointestinal bleeding. 1083 89

Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy. Intestinal transplantation (Tx) can now be recommended for this select group. Life-threatening complications warranting consideration of intestinal Tx include parenteral nutrition-associated liver disease, recurrent sepsis, and threatened loss of central venous access. Because a critical shortage of donor organs exists, waiting times for intestinal Tx are prolonged. Therefore, it is essential that children with life-threatening complications of intestinal failure and parenteral nutrition therapy be identified comparatively early, i.e. in time to receive suitable donor organs before they become critically ill. Children with liver dysfunction should be considered for isolated intestinal Tx before irreversible, advanced bridging fibrosis or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary. Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present. Death resulting from complications of liver failure is especially common during the wait for a combined liver and intestinal transplant, and survival following combined liver and intestinal Tx is probably lower than following an isolated intestinal transplant. The incidence of morbidity and mortality following intestinal Tx is greater than that following liver or kidney Tx, but long-term survival following intestinal Tx is now at least 50-60%. It is probable that outcomes shall improve in the future with continued refinements in operative technique and post-operative management, including immunosuppression.
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PMID:Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation. 1132 44

Pasteurella species are very small gram-negative coccobacilli. They are normal flora found in the oral cavity and gastrointestinal tract of many animals, and can cause various infections including septicemia and pneumonia. Human infection with Pasteurella multocida occurs commonly as a localized cellulitis caused by animal bites. This report described 2 rare cases of P. multocida bacteremia in patients with liver cirrhosis and esophageal varices. Both patients had a history of contact with sick-appearing stray dogs, but neither had been bitten. P. multocida bacteremia should be included in the differential diagnosis of febrile cirrhotic patients with esophageal varices who have a history of non-bite animal exposure. Avoidance of animal contact by immunocompromised patients is the most important factor in preventing pasteurellosis.
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PMID:Pasteurella multocida bacteremia due to non-bite animal exposure in cirrhotic patients: report of two cases. 1182 11

The liver plays a central role in the clotting process, and acute and chronic liver diseases are invariably associated with coagulation disorders due to multiple causes: decreased synthesis of clotting and inhibitor factors, decreased clearance of activated factors, quantitative and qualitative platelet defects, hyperfibrinolysis, and accelerated intravascular coagulation. The bleeding tendency accounts for increased risk of morbidity and mortality in patients with liver disease undergoing diagnostic or therapeutic invasive procedures. Peculiar coagulation disorders are prevalent in patients with acute fatty liver of pregnancy or undergoing liver transplantation. Emerging evidence shows that sepsis further impairs hemostasis in patients with liver cirrhosis bleeding from esophageal varices. Thrombotic events, even if rare in cirrhotic patients, occur mainly in the portal and mesenteric veins. The therapeutic approach to coagulative disorders is also discussed.
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PMID:Coagulation disorders in liver disease. 1192 81

Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3 years, range 1-22.3 years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5 years (range 3.1-33.6 years). One and 5 years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to bile duct dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and bile duct dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n = 3), sclerotherapy (n = 2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n = 1). Of the nine survivors (mean age 12.8 years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.
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PMID:Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease. 1211 59

We report a case of late perforation of the thoracic esophagus with an esophagopleural fistula after endoscopic sclerotherapy for esophageal varices in a Child-Pugh B9 cirrhotic patient. The existence of a thoracic empyema without diffuse mediastinitis allowed management of the fistula by percutaneous drainage-lavage and antibiotic therapy with subsequent closure of the esophageal wall defect and recovery from sepsis. This observation indicates that minimally invasive management of an esophageal perforation complicated by an esophago-pleural fistula is possible in highly selected patients.
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PMID:[Drainage-lavage and closure of a late esophageal perforation with esophagopleural fistula and encysted pleural effusion after endoscopic injection sclerotherapy for varices]. 1473 50


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