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

From december 1984 to december of 1991, 12 children underwent on orthotopic liver transplantation (OLT): 6 had extrahepatic biliary atresia (EHBA), 2 had Byler disease, 2 hepatocellular carcinoma (HCC), 1 Alagille Syndrome and 1 had a hyperacute Wilson disease. The children, transplanted for the most part a broad, return for observation 3 months after OLT. A patient with hyperacute Wilson's Disease had 2 emergency OLTs and died of sepsis (due to Aspergillus); another with EHBA, operated for hepatoportoenterostomy, without result, died after OLT because of a ruptured aortic aneurysm. The other 10 are living with a variable follow-up between 8 months and 7 years. The post-operatory complications were present in 4 cases: in the same patient (15 months old) a hepatic artery thrombosis and then a portal vein thrombosis were observed; 3 patients had to have their biliary-digestive anastomosis redone. 7 of 10 patients had acute rejection. During the first month after OLT infection episodes were mostly due to bacteria (G-), Candida and Pneumocystis carinii (blood and intraabdominal sepsis). In the second period (1-3 months) there were viral infections, in particular CMV. An emergency transplanted patient, incompletely vaccinated, developed HBV infection. During long term follow-up (after the 3rd months from OLT) the children usually have mild infections of the respiratory and urinary tracts. After 1 year, they have a mean annual growth velocity that is between the 50th and 90th percentile. They showed a good rehabilitation. Their hospitalizations rate was reduced if compared with the period before OLT. Health, motor function and general behavior improved significantly.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Liver transplant in childhood: our experience]. 129 33

A retrospective study of 17 babies admitted to the neonatal intensive care unit of the Royal Maternity Hospital, Belfast, was undertaken to determine the causes and prognosis of conjugated hyperbilirubinaemia (direct fraction greater than 20% of total) over a five year period. Mean gestational age was 29 weeks and mean birth weight was 1,240g with a 2:1 male preponderance. All babies had a complicated clinical course involving prolonged periods of parenteral nutrition and many episodes of sepsis. Liver damage was not found to be a contributory factor to death in any baby who died before the age of one year. Bilirubin levels in the survivors had returned to normal within one year. No permanent pathological cause of cholestasis, such as biliary atresia, was ascribable to any of the cases, indicating that extensive investigation to exclude anatomical causes in this population is unlikely to prove rewarding.
 ...
PMID:Cholestasis in a neonatal intensive care unit. 191 21

Twenty-five pediatric orthotopic liver transplantations (OLTs) performed in 22 patients at Sainte-Justine Hospital were reviewed for infections complications. One patient died within 12 hours posttransplantation and is excluded. The patients had an average age of 6.1 years (range, 1.25 to 19 years) and an average weight of 20.4 kg (range, 11 to 55 kg). Two patients (9%) were cytomegalovirus (CMV) seropositive and 9 of 19 patients (48%) were Epstein-Barr virus (EBV) seropositive preoperatively. Five of the donors (20%) were CMV seropositive. The most common indications for OLT were biliary atresia (8) and tyrosinemia (7). There were 4 deaths, for an overall mortality rate of 19%. In 3 patients, deaths were related to infection (CMV hepatitis and duodenitis with aortoduodenal fistula, adult respiratory distress syndrome [ARDS] with Streptococcus viridans pneumonia, Escherichia coli cholangitis with progressive hepatic failure). Fifteen patients (72%) had 41 major infections, most of them bacterial, during the first month posttransplantation. These include pneumonia (25%), line sepsis (17%), cholangitis (14%), and tracheitis (14%). There was only one major viral infection, a CMV hepatitis that occurred in the first month posttransplantation. Three patients had fungal infections (8%) associated with hepatic artery thrombosis and recurrent cholangitis. All three patients required retransplantation. There was only one protozoal infection (Pneumocystis carinii pneumonia) causing life-threatening respiratory failure, from which patient recovered without sequelae. Infection still remains a serious complication of OLT. Bacterial infection is common and is usually associated with technical complications. The low rate of CMV infection is related to low incidence of CMV in the donor pool and the minimal use of strong immunosuppressants.
 ...
PMID:Infectious complications of pediatric liver transplantation. 191 82

Hepatic transplantation is the only therapeutic choice for end-stage pediatric liver diseases. The survival improvement, registered in the last few years is mainly due to the employment of cyclosporine in therapy, but also to new and sophisticated surgical techniques and immunosuppressive drugs. The indications in children are: biliary atresia after unsuccessful Kasai procedure, paucity of intrahepatic bile ducts (of syndromic and not syndromic type), some metabolic diseases (alfa1 antitrypsine deficiency, hereditary tyrosinemia), post infective cirrhosis, acute fulminant hepatic failure, hepatic malignancies. Absolute contraindications include severe systemic illness, severe cardiac or kidney failure, thrombosis or abnormalities of caval and portal veins, systemic sepsis, HIV infection. Other drawbacks are mental deficiency and the inability of family to care for the child and follow therapy after discharge. Relative contraindications are: HBsAg positivity, HIV positivity without infection, malnutrition. Finally the scarcity of donors of liver of adequate size is an important limitation for transplant especially in childhood.
 ...
PMID:[Liver transplant in children. I]. 269 3

Eighteen partial splenic embolization procedures (PSEs) were performed in 17 children for hypersplenism (13) and/or esophageal variceal hemorrhage (12). The underlying disease was biliary atresia (BA) in nine children, portal vein thrombosis (PVT) in four, and biliary cirrhosis (BC) in four. From 20% to 90% of the spleen was embolized. Immediate morbidity was high, albeit minor, and the initial hospitalization was protracted for an average of 16 days. The children were followed from 4 to 81 months (average, 34.2). Four patients with BA patients subsequently had liver transplantation at an average of 20 months after PSE. In ten of 13 patients with hypersplenism, hematologic indexes returned to and remained normal throughout follow-up. The three exceptional patients (who had only 20%, 60% and 60% splenic embolization) developed recurrent mild hypersplenism, one of whom was reembolized and is free from hypersplenism 22 months later. Variceal hemorrhage was ameliorated in all 12 patients (average, 2.4 episodes of hemorrhage per year before PSE, 0.5 per year afterwards). Overwhelming postsplenectomy sepsis did not occur in an aggregate follow-up of 48.5 years. PSE is a legitimate treatment alternative for hypersplenism and for esophageal varices in children.
 ...
PMID:Splenic embolization in children: long-term efficacy. 278 59

Children with end-stage liver disease now form a major sub-group of patients considered suitable for liver transplantation (ltp), and enjoy better survival statistics after transplantation than do adults. Since June 1984, a paediatric ltp programme has been developed in Brisbane with an initial working relationship and ongoing close links with two USA centres (Pittsburgh, and the UCLA Medical Center). Fourteen children with end-stage liver disease have been referred to the Queensland Liver Transplantation Programme for formal assessment. Following frank, informed discussion with their parents, 10 of these children were offered the option of ltp. During the transition stage, two infants with biliary atresia were referred to UCLA at their parents' request and, subsequently, eight children aged from 9 months to 6 years have been placed on a transplant candidacy list in Brisbane. A donor procurement team with access to a Queensland Government jet has been available to cover all mainland States except Western Australia. Six of the children have now had orthotopic ltp (two children at the UCLA Medical Center; four children at the Royal Children's Hospital, Brisbane). One UCLA patient died with a non-functioning graft, and one Brisbane patient died 5 weeks post-transplant with rejection, hepatic artery thrombosis and sepsis. The other four children are alive and well, three with normal liver function and one with unexplained intrahepatic cholestasis, during the 1-20 month follow-up to date. Three further children have died of their liver disease without a donor of an appropriate blood group and size being found, and one patient still awaits a suitable donor.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Liver transplantation in children: experience with the development of an Australian pilot programme. The Queensland Liver Transplantation Programme. 353 23

Since 1974, 25 patients with biliary atresia underwent a modified Sawaguchi hepatoportoenterostomy. Seventeen of 18 patients operated on before the age of 10 weeks became free of jaundice within 2 to 4 months and had biliary intestinal continuity established by 10 months of age. Two of these patients experienced sudden diminution of bile flow with return of jaundice. Both responded to transconduit repeat resection of the portal fibrous tissue with resolution of jaundice, which allowed take-down of the biliostomy. At last follow-up 1 to 13 years postoperatively, all 17 patients were free of jaundice (94 percent of total). One patient in this group never had drainage of bile and eventually died from sepsis. In contrast, five of seven patients operated on after 10 weeks never had drainage of bile. Four of these patients died from hepatic failure and one from bleeding varices. Two patients initially had bile drainage and became anicteric, but thereafter, acute cessation of bile flow and reccurrence of jaundice occurred. Reoperation resulted in a definitive increase in bile flow in both of these patients. The modified Sawaguchi procedure is definitely preferable to liver transplantation as primary treatment in infants with biliary atresia, especially if operation is performed before the age of 10 weeks.
 ...
PMID:Encouraging results with a modified Sawaguchi hepatoportoenterostomy for biliary atresia. 360 12

Endoscopic inspection of the porta hepatis was undertaken through external enterostomy in nine postoperative patients with biliary atresia. The endoscopic appearance of bile flow in the porta hepatis was classified into three types. 1) Ductal type (D-type), which revealed good bile flow from distinct bile duct orifices (4 cases); 2) Oozing type (O-type), which showed adequate bile flow, but no definite bile duct (3 cases); and 3) Covered type (C-type), in which the porta hepatis was covered with bile "clots" and fibrous tissue (2 cases). The clinical course of the D-type was excellent with immediate disappearance of jaundice and relatively good liver function. In the case of the O-type, jaundice disappeared in 2 but persisted in one. Two patients with C-type died of hepatic failure or sepsis in the early postoperative period. In the D-type intrahepatic bile ducts were well visualized by cholangiography; however, those of the O-type were not so clear. Endoscopic inspection of the porta hepatis is very useful for evaluation of the postoperative state of bilioenteric fistulae in patients with biliary atresia.
 ...
PMID:Endoscopic evaluation of the porta hepatis in patients with biliary atresia. 398 33

The frequency of TPN in surgical infants was 242/686 (37.8%) in neonates and 260/2693 (9.7%) in older infants for the past 15 years. The frequent indications of TPN were intestinal atresia, Hirschsprung's disease and esophageal atresia in the neonatal period, and Hirschsprung's disease, pyloric stenosis and biliary atresia in infancy. The effectiveness of TPN was impressively indicated by better survival rate in the infants with massive intestinal resection received TPN. An amino acid solution (N1-2) was newly devised, based on the analysis of plasma aminograms in 36 infants received TPN with a commercial amino acid solution (Proteamin), and theoretical considerations. It was more useful solution for TPN in 12 young infants. Urinary phosphorus was a considerable parameter for the administration of Vitamin D in TPN. The incidence of TPN-induced hepatic dysfunction was significantly referred to the amount of amino acid in TPN. Closed infusion system with a soft bag and triple bacterial filters in the line was effective for the prevention of sepsis caused by central venous catheter. The nutritional care was important in the treatment of infants with biliary atresia, because generally they had some nutritional defects such as essential fatty acid deficiency, insufficient amino acid metabolism and zinc deficiency. The nutritional care in infants with advanced neuroblastoma favorably altered the course of the disease.
 ...
PMID:[Total parenteral nutrition in surgical treatment of infants: recent progress and problems]. 643 80

At least four bilirubin fractions can be separated and identified by liquid chromatography, the least understood being the "delta" fraction (B delta), which apparently is covalently bound to albumin. To learn more about the incidence and significance of B delta, we assayed serum from 539 infants and children, both by the chromatographic method and the routine colorimetric Jendrassik-Grof method. The proportion of B delta appeared to correlate with both age and disease course. For infants younger than 28 days B delta generally was less than 2% of total bilirubin; for hyperbilirubinemic older infants and children the median B delta value was 35%. High B delta (greater than 50% of total bilirubin) in newborns was associated with intra- and extra-hepatic cholestasis, biliary cirrhosis, biliary atresia, and hepatitis. Among older infants and children, a proportion of low B delta (less than 10%) was found in hemolytic anemias, sepsis, shock, and other non-hepatic jaundice. In several cases, when low B delta was accompanied by increased conjugated bilirubin, the prognosis was very poor. Delayed clearance of B delta from the circulation together with its reactivity in direct diazo methods may interfere with interpretation of values for conjugated bilirubin as measured by classical methods.
 ...
PMID:Delta bilirubin in serum of pediatric patients: correlations with age and disease. 646 72


1 2 3 4 Next >>