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)

A 31-year-old man, who had undergone splenectomy 18 months previously because of hereditary spherocytosis, suddenly became ill, with fever, vomiting, epigastric pain and shock, and died 10 hours after the onset of his symptoms. Autopsy showed influenzal viremia, pneumococcemia and bilateral adrenal hemorrhage. The rapid course of the patient's illness emphasizes the serious risk of sepsis for individuals who have had a splenectomy. Anti-influenza immunization in such patients should be considered.
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PMID:Postsplenectomy sepsis due to influenzal viremia and pneumococcemia. 0 5

One hundred and eighty-two patients undergoing splenectomy in infancy and childhood were followed for periods of 2 to 15 years. Serious infections occurred in 11 patients (6%) with death in 6 (3.3%). In 10 patients the infection was sepsis, and in all but one patient the infection occurred within 2 years of splenectomy. Among children over 2 years of age the risk of infection was still appreciable except when the spleen was removed incidentally or for traumatic rupture. Splenectomy for thalassemia and portal hypertension resulted in an increased risk of serious infections when compared with removal of the spleen for hereditary spherocytosis, idiopathic thrombocytopenic purpura, trauma, or for technical reasons in the course of another operation. Post-splenectomy infections tended to follow a characteristic pattern. The infecting organism was predominantly pneumococcus, the course was fulminating and the mortality high.
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PMID:The morbidity and mortality of splenectomy in childhood. 55 12

A family with autosomal dominant inheritance of sacral agenesis is described. Ten members were affected; four had associated presacral teratomas and anterior sacral meningoceles, giving rise to serious complications in three, including bacterial meningitis, local recurrence of teratoma and perianal sepsis. Three of those with presacral masses presented initially with anorectal anomalies. Other associated abnormalities included tethering of the cord, hydrocephalus, duplex ureter, hydronephrosis, vesicoureteric reflux, neurogenic bladder, bicornuate uterus, rectovaginal fistula and hereditary spherocytosis. Early diagnosis and surgical excision of a presacral mass is advised to prevent future morbidity and mortality.
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PMID:Hereditary sacral agenesis with presacral mass and anorectal stenosis: the Currarino triad. 205 99

Patients with mild hereditary spherocytosis (HS) often undergo splenectomy for the sole purpose of preventing gallstone formation. Splenectomy carries a surgical risk as well as the risk of postsplenectomy sepsis. Gallstones develop in less than half of mild HS patients and do not always cause symptomatic biliary tract disease. Using decision analysis, a quantitative approach to problem solving under conditions of uncertainty, we have compared the likelihood of surviving the complications of gallstones with the likelihood of surviving routine splenectomy. Probability figures for critical events were obtained from the medical literature; final outcome is recovery (utility = 1.0) or death (utility = 0.0). Our analysis shows that expectant management of gallstones is the preferred choice, being associated with a higher utility than is routine splenectomy (0.9980 vs 0.9755, respectively). The utility values for the two choices become equivalent only when the risk of postsplenectomy sepsis is lowered from 0.022 to 0.0001.
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PMID:Splenectomy in mild hereditary spherocytosis: is it worth the risk? 278 57

The most common hematologic and oncologic indications for splenectomy in childhood are hereditary spherocytosis, chronic idiopathic thrombocytopenic purpura, hypersplenism, and Hodgkin's disease. Because of the increased incidence of septic complications after splenectomy, benefits to be gained from the operation should be weighed against the risks. A retrospective study was done on the charts of 42 consecutive children with hematologic and oncologic disorders, who underwent splenectomy between 1967 and 1982. The incidence of septic complications after splenectomy was 12%; sepsis, however, only occurred in patients with severe underlying diseases (three patients with Hodgkin's disease, one patient with systemic lupus erythematosus, and one patient with chronic pseudo-malignant immunoproliferation). In contrast, none of the patients who were splenectomized for other reasons (mainly hereditary spherocytosis and chronic immune thrombocytopenic purpura) had a septic complication. Two patients with end-stage Hodgkin's disease (5%) experienced fatal septic complications. Although splenectomy is well established for diagnostic and therapeutic considerations in patients with Hodgkin's disease, not all of them might benefit from this operation, and studies with a more limited approach to splenectomy might prove to be of the same therapeutical value.
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PMID:Hematological and oncological indications for splenectomy in children. 392 22

Hereditary spherocytosis is a clinically heterogeneous, genetically determined red blood cell membrane disorder resulting in hemolytic anemia. A deficiency of spectrin, the largest and most abundant structural protein of the erythrocyte membrane skeleton, results in the formation of spherocytes which lack the strength, durability, and flexibility to withstand the stresses of the circulation. Clinical manifestations of the disease are primarily dependent on the severity of hemolysis, which additionally results in an increased incidence of pigment gallstones. The likelihood of cholelithiasis is directly related to patient age and is uncommon before 10 years of age. Splenectomy is indicated in virtually every patient. When the disease is diagnosed in early childhood, the risk of overwhelming postsplenectomy sepsis makes it advisable to delay splenectomy until after 6 years of age if possible. At the time of splenectomy, it is important to identify and remove any accessory spleens. If gallstones are present, cholecystectomy should be performed. Although spherocytosis persists following splenectomy, hemolysis is alleviated and clinical cure of the anemia is achieved for most patients. Patients with recessively inherited spherocytosis are exceptions. Although they are significantly benefited by splenectomy, their anemia is not completely corrected. Splenectomy reduces hemolysis in all patients and thereby decreases the risk for development of pigment gallstones. Excision of an enlarged spleen removes the danger of traumatic rupture.
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PMID:Hereditary spherocytosis. Recent experience and current concepts of pathophysiology. 394 20

Three new cases (two fatal) of postsplenectomy sepsis occurring 14, 25, and 26 years after operation for hereditary spherocytosis are described. There are now 25 cases in the published work in which this complication occurred 10 or more years after operation, 14 of which were fatal. The mean age of onset is 37 years. The features of the disease are similar to those seen in other postsplenectomy infections, and pneumococcal infection was responsible in 19 cases (76%). The major predisposing illnesses were trauma, hereditary spherocytosis, and idiopathic thrombocytopenia.
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PMID:Postsplenectomy sepsis 10 years or more after operation. 397 56

Asplenic persons are at increased risk of overwhelming sepsis caused by Streptococcus pneumoniae. Vaccination with polyvalent pneumococcal polysaccharide has been shown to stimulate a nearly normal antibody response in these individuals, indicating that active vaccination might prevent pneumococcal disease in this population. To obtain information on the duration of protective levels of pneumococcal antibody, 33 asplenic children were vaccinated and antibody levels were measured at intervals for up to 4 1/2 years after vaccination. Significant antibody decline was observed in children who had undergone splenectomy because of trauma, but antibody decline was not observed in children whose spleens had been removed because of hereditary spherocytosis. There was a highly significant difference in rates of antibody decline among the 12 antibody serotypes measured: types 1, 4, 6A, 7F, 8, 19F, and 23F showed the greatest decline. Based on measured rates of antibody decline, subprotective antibody levels (antibody nitrogen less than 300 ng/ml) for types 7F, 8, and 19F would be reached 1 to 2 years after vaccination; type 6A never reached the protective level; and antibodies against the remaining eight types either were within the protective range initially or did not show significant decline. Asplenic children may benefit from revaccination with certain antigen types (7F, 8, and 19F) 1 to 2 years after initial vaccination.
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PMID:Decline of serum antibody in splenectomized children after vaccination with pneumococcal capsular polysaccharides. 648 33

Between 1956 and 1981, 306 splenectomies for hematologic diseases were performed at the UCLA Medical Center. Of these operations, more than 75% were performed for therapeutic reasons to control anemia, thrombocytopenia, neutropenia, or painful symptoms of splenomegaly. Of the 65 patients who had idiopathic thrombocytopenic purpura, 77% showed an excellent response, and of the 39 patients who had hereditary spherocytosis, 90% responded. Other diseases with predictably good response rates were autoimmune hemolytic anemias, Felty's syndrome, and hairy cell leukemia. Forty patients with Hodgkin's disease had splenectomies for diagnostic purposes the last 10 years. The overall morbidity and mortality were 24% and 6%, respectively, the most common complications being pneumonia, wound infections, and local postoperative bleeding, and the most common cause of death being sepsis. The review supports the thesis that in carefully selected patients, therapeutic splenectomy can have desirable palliative effects and that diagnostic splenectomy has a sufficiently low risk to warrant its consideration in patients with Hodgkin's disease.
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PMID:Splenectomy for hematologic disease. The UCLA experience with 306 patients. 673 25

Elective splenectomies performed in children aged 0-14 during the period 1968 to 1977 have been reviewed. One hundred and twelve children were splenectomized during the period studied. The indication for splenectomy was usually a haematological disorder e.g. hereditary spherocytosis or idiopathic thrombocytopenia. As splenectomized children run the risk of developing overwhelming sepsis, inquiries have been sent to all children. Two cases of nonfatal pneumococcal septicaemia are reported. Although the incidence of septicaemia is low, spleensaving operations are to be preferred where possible. When splenectomy is performed, pneumococcal antibodies should be determined. If a low titre is found, polyvalent pneumococcal vaccine should be used.
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PMID:Complications after elective splenectomy in children. 679 46


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