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)

Since the beginning of the decade we can remark an increasing of publications about laparoscopic approach in splenic surgery. We report about 17 unselected own cases (8 males, 9 females). Main indications were 12 hematologic diseases: 9 idiopathic thrombopenic purpuras, 1 hemolytic anemia, 1 Hodgkin's disease and 1 recurrent splenic infarction with hypersplenism and extramedulary hemapoiesis, further 1 unclear sepsis with focal lesions and splenomegaly, 1 dysontogenetic splenic cyst, 1 splenic co-affection of Wegener's disease, and 2 traumatic lesions. We performed in 13 cases asplenectomy, under these 1 time combined with a laparoscopic hernioplasty and 1 time with a complete staging, further in 1 case a partial splenic resection, in 1 case a diagnostic excision and in 2 cases a hemostyptic management without splenectomy. We had to convert in no case, the lethality rate was also 0. As a complication we noted one postoperative bleeding, we could managing also laparoscopically. Our mean operating time of 137 min was evident shorter than the published ones by other authors. Because of our good results and the excellent tolerance by patients we can recommend this proceeding. Nevertheless, it should be limited for surgeons with a large experience at laparoscopic centers.
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PMID:[Laparoscopic splenic surgery--report of 17 personal cases]. 922 40

Splenectomy in patients with non-Hodgkin's lymphoma (NHL) is performed for either diagnostic or therapeutic reasons. We report on a series of 29 patients with NHL and splenomegaly who underwent splenectomy during the years 1979-1998 in our hospital. According to the indication for splenectomy our patients were categorized in three groups. Group A: In 20 patients splenectomy was performed for diagnostic reasons. Group B: Three patients were splenectomized for autoimmune haemolytic anaemia (AIHA). Group C: Six patients underwent splenectomy because of hypersplenism. A definitive histopathological diagnosis of NHL was obtained in all patients of group A. Hypersplenism and AIHA were resolved in all patients after splenectomy. One (3.5%) patient died postoperatively because of septicemia complicated by disseminated intravascular coagulation. Six postoperative complications were observed in 4 (14%) patients. Splenectomy, with an acceptable surgical risk, has the potential to establish the diagnosis of NHL in patients with splenomegaly without lymphadenopathy and negative bone marrow findings. Moreover, splenectomy has the capacity to modify the disease course in patients with NHL complicated by AIHA or hypersplenism.
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PMID:Splenectomy in patients with malignant non-Hodgkin's lymphoma. 1099 79

Absence of the spleen or splenic function predisposes individuals to risk of overwhelming infection. These infections are most often due to encapsulated organisms, especially pneumococcus, Haemophilus influenzae type b, and meningococcus, but any bacterial agent may cause the rapid onset of septicemia, meningitis, pneumonia, and shock characteristic of the asplenic-hyposplenic condition. The risk is greatest in infants and young children, but asplenic-hyposplenic adults also have an increased risk of infection. Prophylactic antibiotics and immunization with polyvalent pneumococcal, H. influenzae type b, and meningococcal vaccines have reduced the incidence of infections in asplenic-hyposplenic individuals, but even these measures have not eliminated the risk. Surgeons have adopted techniques to save as much splenic tissue as possible and some splenic functions, such as pitting red cells, have been preserved, but conservative surgery has not provided total protection against overwhelming infection. Therapies designed to interrupt the cascade of overwhelming sepsis have not yet been successful. In those cases in which the spleen is surgically removed, the underlying disease or condition leading to splenectomy influences the risk of sepsis. Splenectomy incidental to other operations, such as gastrectomy, results in the lowest risk for overwhelming infection, but this is still some 35-fold greater than the risk for overwhelming infections in the general population. In increasing order of risk, the other main indications for surgical removal of the spleen are idiopathic thrombocytopenia purpura, trauma, transplantation procedures, hereditary spherocytosis, staging Hodgkin's disease, portal hypertension with hypersplenism, and thalassemia. Pathologists should comment on the risk of overwhelming sepsis when spleens are processed as surgical specimens, and should carefully weigh all splenic tissue, including accessory spleens and splenic implants (splenosis), in autopsy cases with and without overwhelming sepsis.
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PMID:Asplenic-hyposplenic overwhelming sepsis: postsplenectomy sepsis revisited. 1117 26

Liver injury is associated with parenteral nutrition therapy. Severity of injury varies from minimal and transient increases in liver-related blood tests to biliary cirrhosis and liver failure. Severe parenteral nutrition-related liver disease is usually confined to patients who have undergone massive intestinal resection. In these patients, early sepsis appears to cause initial liver injury, and recurring sepsis and inflammation, local or systemic, may result in its perpetuation and progression. Liver disease associated with parenteral nutrition is not necessarily related either to duration of parenteral nutrition or to delayed intestinal feeding. However, treatment includes enteral nutrition to promote enterohepatic circulation of bile acids and management of inflammation and sepsis, including control of intestinal bacterial overgrowth. Restriction of intravenous lipid emulsions may be important. The clinical picture of advanced liver failure related to short bowel syndrome differs from liver failure with an anatomically normal gastrointestinal tract. In the former, hyperbilirubinemia, hepatosplenomegaly, and functional hypersplenism dominate the clinical picture, and severe ascites and esophageal variceal hemorrhage are unusual. Early referral of these patients for intestinal and/or liver transplantation may provide the best chance for long-term survival.
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PMID:Prevention of parenteral nutrition-associated liver disease in children. 1190 41

Glycogen storage disease (GSD) is a rare autosomal-recessive disorder characterized by hypoglycemia, hepatosplenomegaly, seizures, and failure to thrive in infants. Neutropenia and/or neutrophil dysfunction develops in GSD1b, but not in other types. GSD1b results from a deficiency of the glucose-6-phosphate translocase enzyme and the genetic defect maps to chromosome 11q23. Patients with GSD1b are susceptible to recurrent bacterial infections, commonly involving the perirectal area, ears, skin, and urinary tract, although life-threatening infections, such as septicemia, pneumonia, and meningitis occur less frequently. Although the exact mechanism of neutropenia in patients with GSD1b is not known, treatment with recombinant human granulocyte colony-stimulating factor (G-CSF) has reduced the incidence of infections and has improved the quality of life of these patients. Defects in neutrophil chemotaxis and intracellular bacterial killing have been described and appear to be corrected by the use of G-CSF. To date, no cases of myelodysplasia or acute myeloid leukemia have been observed in patients with GSD1b treated with G-CSF. A significant complication of cytokine therapy is the development of hypersplenism, requiring either a reduction in the dosage of G-CSF or splenectomy.
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PMID:Glycogen storage disease. 1195 92

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

Partial splenic embolization (PSE) has been demonstrated to be an effective alternative to splenectomy for patients with hypersplenism. Splenectomy in these patients can be associated with an increased risk of perioperative complications, overwhelming post-splenectomy sepsis (OPSS) and mortality. Partial splenic embolization has the advantages of non- operative intervention and resolution of the complications of hypersplenism. We report the use of this technique in patients with portal hypertension and hypersplenism awaiting liver transplant and patients that have undergone othotopic liver transplantation (OLTx) with persistent hypersplenism post-transplant. Six patients--three awaiting liver transplantation and three patients with persistent hypersplenism status post-OLTx--were treated during the period of 1993-99 at the LSUHSC/Willis Knighton Regional Transplant Center in Shreveport, Louisiana. Three patients were male and three female. All six patients had concomitant thrombocytopenia and neutropenia with platelet counts below 50,000. Patients underwent selective arterial catheterization and embolization via a percutaneous approach with Cook microcoils or PVA particles. The lower pole of the spleen was selectively embolized in all patients to achieve a 30-50% reduction in flow as determined by angiography. Patients were followed with routine computed tomography (CT) scans, platelet and WBC counts for a mean of 26 months in the pre-transplant and 37 months in the post-transplant group. In both groups, all patients had persistent resolution of thrombocytopenia and neutropenia after embolization. In the post-transplant group, one patient had persistent splenomegaly and required splenectomy for pain control. No procedure-related complications occurred in any patient. In this limited review, PSE appears to be a safe and effective treatment of persistent hypersplenism in patients with portal hypertension and those who have undergone OLTx.
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PMID:Partial splenic embolization for hypersplenism before and after liver transplantation. 1237 46

This study was carried out on forty cases, classified into 3 groups; group I: 10 healthy controls subjects, group II: 20 patients with hepatosplenic schistosomiasis and group III: 10 bilharzial patients who underwent total splenectomy. All cases were subjected to clinical examination, abdominal ultrasonography, rectal snips and laboratory investigations which included: stool and urine analysis, complete blood picture, IHAT for bilharziasis, liver function tests, viral markers, estimaton of T-lymphocyte subpopulations (CD(+)3, CD(+)4, & CD(+)8) by flow Cytometry, silver stained blood films to detect argyrophilic inclusions and 99mTc sulphur colloid splenic scan which was applied to group II only. The present results revealed varying degrees of hypersplenism (anaemia, leukopenia & thrombocytopenia) in GII. Seventy percent of this group was positive for HbsAg, HCV or both in association with schistosomiasis. Abnormal red blood cells (acanthocytes, target cells, pitted cells & normoblasts) and inclusion bodies (Howell Jolly bodies, argyrophilic inclusions & pappenhiemer bodies) were detected with different values in GIII. CD(+)4 cells were moderately reduced in GII while they were markedly decreased in GIII. CD(+)8 cells were elevated in GII and returned nearly to the normal values in GIII with decrease in number of total T-lymphocytes. Most patients of GII showed marked squestration of 99mTc labelled R.B.Cs. in the spleen with reversed hepatic/splenic ratio (normally hepatic/splenic ratio is over two). IHAT showed positivity in 90% of patients in GII while it was 50% in GIII. Although total splenectomy improved the haematological pattern and the cytopenias, which are prominent features in hepatosplenic schistosomiasis, yet the immunological profile was still altered. So, it is recommended to perform segmental splenectomy with retention of a normal mass of functioning residual spleen to preserve more immunological function and to protect against life-threatening occurrence of post-splenectomy sepsis.
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PMID:Assessment of splenic functions in patients with hepato-splenic schistosomiasis using non-invasive techniques. 1256

DIAGNOSTIC CIRCUMSTANCES: Portal vein thrombosis is the second cause of portal hypertension after cirrhosis in Western countries. Diagnosis can be either made at the acute stage in the context of abdominal pain or after appearance of a porto-portal collateral venous circulation leading to the formation of a portal cavernoma, the diagnosis being made in the circumstance of rupture of oesophageal varicose veins or manifestations of hypersplenism. AETIOLOGICAL SURVEY: In the absence of hepatocellular carcinoma, causes that need to be investigated are cirrhosis, local factors (intra-abdominal sepsis, abdominal surgery, splenectomy or pancreatitis), and one or several prothrombotic affections (acquired or inherited prothrombotic states are present in 70% of cases, with myeloproliferative disease ranking first). REGARDING TREATMENT: Anticoagulant therapy generally allows recanalisation of the thombosed veins in recently constituted thrombosis. Some patients at the portal cavernoma stage can also benefit from anticoagulant therapy: patients with a prothrombotic state without large oesophageal-gastric varicose veins. In the case of large oesophageal-gastric varicose veins that have never bled, treatment to prevent haemorrhages due to portal hypertension according to the same modalities as in cirrhosis must be associated with the prescription of an anticoagulant. In the absence of prothrombotic affection or in patients having already suffered from haemorrhages due to portal hypertension, the benefit of anticoagulant therapy is less clearly established.
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PMID:[Portal vein thrombosis]. 1453 80

This paper presents a comparative prospective study of three modalities of surgical treatment for extrahepatic portal hypertension in children: central splenorenal shunt after splenectomy (CSS), side-to-side lienorenal shunt (SSLR) without splenectomy, and splenectomy and gastroesophageal devascularization (SGD). In an 18-month period, 27 procedures were performed: 10 CSS, 10 SSLR, and seven SGD. The outcomes were evaluated by fall in portal pressures, hematological parameters, shunt patency, splenic regression, and disappearance of esophageal varices. All three procedures were comparable in the fall of portal pressure after surgery. The average blood loss and operating time were statistically significant in favor of SSLR compared with CSS. At 3-month follow-up, shunt patency was confirmed by duplex Doppler study in all the patients in the SSLR group and in nine out of 10 patients in the CSS group. In the CSS and SGD groups, hypersplenism resolved in all the patients. In the SSLR group, blood counts improved in only five out of eight affected children. No patient re-bled during a follow-up of 3-5 years. There were no cases of hepatic encephalopathy or overwhelming postsplenectomy sepsis. In conclusion, CSS is useful when there is a large spleen, severe hypersplenism, and a shuntable splenic vein. SSLR is suitable when there is only mild splenomegaly, mild hypersplenism, and a shuntable splenic vein. Splenectomy and devascularization is the choice when there is no shuntable splenic vein.
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PMID:Extrahepatic portal hypertension in children: observations on three surgical procedures. 1535 94


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