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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The risk of bacterial sepsis in the surgically or functionally asplenic host is reviewed. The lowest morbidity occurs in patients splenectomized because of trauma to the spleen; the highest morbidity occurs in patients splenectomized for thalassemia. There is approximately a 50% mortality associated with sepsis secondary to asplenia and the pneumococcus is responsible for over 50% of the cases. Normal spleen function and alteration in host defense occurring as a consequence of asplenia is discussed. Finally, alternatives to and indications for splenectomy as well as prophylactic measures are considered. It is concluded that, at the present time, antibiotic coverage for an indefinite period of time may be indicated for surgically or functionally asplenic patients.
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PMID:Bacterial infection and the asplenic host: a review. 0 50

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

Variations in the serial immunoglobulins of 52 children splenectomized for a variety of indications were studied and compared with two groups of children, one postoperative and one without operation. The most significant finding was the constant decrease in Ig M in every instance. Our series of splenectomized children seems to confirm the facts noted by other authors. The incidence of serious infections has been clearly significant and is clearly postsplenectomy sepsis. The age of the patients has a great influence on the seriousness of the septic process, these being more severe on younger children. The influence has also been revealed of the cause which motivated the operation, this corresponding in the first place to thalassemia and in the second place through spherocytosis and pseudohormones. It is evident that the immunologic role of the spleen during early childhood, especially under the age of 5 yr is important. The greatest risk or postsplenectomy sepsis is in the first 2 yr of life. Splenectomy should be delayed until after the age of 5 if clinical circumstances permit. If not, prophylactic chemotherapy should be carried out during the first 18 mo after splenectomy.
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PMID:Immunological studies in the postsplenectomy syndrome. 111 95

We report our experience with high dose intravenous immunoglobulin (IVIg) in 3 thalassemic patients who had evidence of possible immune hemolysis. In 2 patients who had serious sepsis, their responses to IVIg were only partial and transient. The other patient who had marked splenomegaly had no evidence of response to IVIg. Both serious infections and large spleen may hamper the effect of IVIg and should be considered before IVIg is to be used in thalassemia.
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PMID:High-dose intravenous immunoglobulin in the management of immune hemolysis in patients with thalassemic disease: factors which determine refractoriness. 172 38

Splenic function in patients with sickle B+ (SB+) thalassemia has been poorly documented. We evaluated the clinical course and splenic function in 12 children with SB+ thalassemia with simultaneous technetium sulfur colloid spleen scans and determination of pitted erythrocytes by direct interference contrast microscopy (DICM). All patients displayed normal uptake of radiocolloid. Mean percentage of pitted erythrocytes was 2.2% compared to 0.06% in 10 normal eusplenic controls and 13.8% in 10 sickle cell patients. In this group of children, who were carefully monitored for 136 patient years, there was no episode of bacteremia/sepsis, and a low prevalence of vaso-occlusive episodes. The slight increase in percentage of pitted erythrocytes of SB+ thalassemia patients does not seem to herald any clinically relevant loss of splenic function. SB+ thalassemia children younger than 10 years of age who do not seem to present a higher risk of invasive bacterial infections than eusplenic children, should receive conservative treatment for isolated febrile episodes and should not be submitted to prophylactic penicillin.
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PMID:Spleen function in children with sickle B+ thalassemia. 194 16

Splenic function in sickle hemoglobinopathy syndromes was assessed to determine the developmental pattern of splenic dysfunction. Nonvisualization of the spleen using technetium-99 metastable (99mTc) spleen scans correlated strongly with pocked (vesiculated) RBCs greater than or equal to 3.5%. Cross-sectional analysis of pocked RBC data from 2,086 patients showed differences in the developmental pattern of splenic dysfunction between several disorders. In hemoglobin SS disease (sickle cell anemia) and hemoglobin S beta(0) thalassemia, splenic dysfunction (greater than or equal to 3.5% pocked RBCs) often occurred in the first 6 to 12 months of life. In hemoglobin S beta(+) thalassemia, splenic dysfunction occurred less frequently and later. Splenic dysfunction in hemoglobin SC disease (sickle cell-hemoglobin C) was intermediate. The level of pocked RBCs was inversely associated with fetal hemoglobin (P less than .007) and directly associated with age (P less than or equal to .001). These patterns of splenic dysfunction reflect the known severity of hemolysis and intravascular sickling and are consistent with the epidemiology of severe bacterial meningitis and sepsis in these diseases. Serial measurement of pocked RBCs permits determination of the onset of splenic dysfunction and the time of increased susceptibility to severe bacterial infections.
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PMID:Developmental pattern of splenic dysfunction in sickle cell disorders. 241

Fourteen thalassemia patients (aged 1.6-13.5 years; median age of 6 years) underwent allogeneic bone marrow transplantation (BMT) between March 1984 and May 1987. The preconditioning regimens consisted of oral busulfan, intravenous cyclophosphamide, with or without irradiation. Two of the patients, who received maternal transplants, failed to engraft but experienced autologous recovery. Of the 12 patients who received sibling marrow, two experienced autologous recovery and one developed marrow chimerism. Five patients died of complications of bone marrow transplantation: two died of intracranial hemorrhage, two died of sepsis, and one succumbed to acute graft-versus-host disease associated with cytomegalovirus infection. Six patients engrafted and have been followed for 1-4 years (median of 2.6 years) without intoward events. The overall survival rate was 64% (nine out of 14) with follow-up of 1-4 years. These results demonstrate that bone marrow transplantation can cure thalassemia but infection, graft-versus-host disease, and hemorrhage were major causes of morbidity and mortality in this group of patients. Other factors of importance include the unfavorable influence of engraftment of prior multiple transfusions and sex-mismatched transplantation. In patients who fail to engraft, autologous recovery usually occurs within 2 months of transplantation.
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PMID:Allogeneic bone marrow transplantation for thalassemia in Taiwan: factors associated with graft failure. 261 76

A number of Saudi children (31) with sickle cell disease and thalassemia underwent splenectomy: 12 for frequent blood transfusions, 15 for chronic hypersplenism (most of whom were also the recipients of periodic blood transfusion) and 4 for splenic abscess. The mean age of splenectomy was 8.8 years (8 months-18 years). Eight patients had sickle cell disease, 14 beta-thalassemia and 9 had sickle cell thalassemia. All patients received prophylaxis against pneumococcal infection. There was one postoperative death most probably due to sepsis. Sixteen of those who required frequent preoperative blood transfusions needed no more transfusions, while in 7 the need for transfusions decreased significantly (p less than 0.05). For those with hypersplenism, there was a significant postoperative increase in total hemoglobin (P less than 0.001), RBC (P less than 0.001) and platelet counts (p less than 0.02); and a substantial decrease in reticulocyte counts (p less than 0.05). The common post splenectomy complications were chest infection and a brief episode of pyrexia, but without undue morbidity. The study establishes a definite place for splenectomy in a selected population of children with sickle cell disease and thalassemia.
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PMID:Splenectomy in children with sickle cell disease and thalassemia. 263 77

We report two cases of severe Yersinia enterocolitica infection in children with homozygous thalassemia. One patient had septicemia and the other had mesenteric adenitis. Two factors can enhance the infectivity of Yersinia enterocolitica in children with thalassemia: iron overload and deferoxamine therapy. Laparotomy and cefotaxime-netilmicin therapy were successful in the patient with mesenteric adenitis. In the patient with septicemia, cefotaxime-netilmicin, then doxycycline-netilmicin failed, and recovery was finally achieved under rifampicin-netilmicin. Because of the possibility of septicemic dissemination secondary to digestive Yersinia enterocolitica infection in children with thalassemia, we advocate immediate discontinuation of deferoxamine and prescription of oral antimicrobial therapy (trimethoprim-sulfamethoxazole for instance) in every thalassemic patient with febrile diarrhea.
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PMID:[Yersinia enterocolitica infections and thalassemia major in children]. 266 80

Foremost among the beneficial effects of screening umbilical cord blood is the optimized quality of care that can follow the immediate involvement of an infant with sickle cell disease and his or her family in an appropriate health care system. This is exemplified by the reduction in the case fatality rate of pneumococcal septicemia that has been achieved. Appropriate follow-up of screening also includes transmission of information about the diagnosis of a hemoglobinopathy trait or alpha-thalassemia to affected families and their physicians, with ready availability of education and counseling.
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PMID:Value of screening umbilical cord blood for hemoglobinopathy. 271 9


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