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

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

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

There is a high incidence of bacterial infections in sickle cell disease, particularly in sickle cell anemia. Pneumonia, urinary tract infections, osteomyelitis, meningitis and pneumococcal septicemia occur mainly in younger patient. The pathological basis for this susceptibility to infections is complex. Defective splenic function is the most important factor. There are also abnormalities of opsonization, alternate complement pathway, antibody production, leucocyte function, and cell-mediated immunity. Pneumococcal immunization and prophylactic penicillin are indicated in the prevention of pneumococcal infections.
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PMID:[Infection and immunity in sickle cell disease]. 269 86

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

A retrospective analysis of 69 case-reports of children with homozygous sickle cell anemia hospitalized from 1964 through 1985 at the Kinshasa University Pediatric Hospital highlights these patient's high susceptibility to bacterial septicemia. Among causative organisms, the most prevalent were salmonellae (20 cases), pneumococci (15 cases), and klebsiella (12 cases). Clinical features of bacterial septicemia are identical in children with and without sickle cell anemia. Bone and/or joint infections are usually found in salmonella septicemia and meningeal or pleuropulmonary localizations in pneumococcal septicemia. Eighteen children (26%) died, including 10 with pneumococcal septicemia (5 cases) and 10 with Salmonella septicemia (5 cases). Poor prognosis factors include resistance to commonly used antimicrobial agents, frequently found with Salmonella organisms, and concomitant meningeal infection.
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PMID:[Bacterial septicemias in children with homozygous sickle cell anemia. Analysis of 69 cases]. 274 15

To determine the effects of blood transfusions on splenic function in older patients with sickle cell anemia, we investigated splenic function in 12 patients who had had cerebrovascular accidents and who were being treated at two collaborating centers using different transfusion protocols. Splenic function was assessed by radionuclide scan and pocked erythrocyte count. Patients were 6 to 18 years of age and had been receiving transfusions for 7 months to 10 years (median 4.2 years). Of the 12 children, five had normal or increased splenic size and function (normal scan and normal or minimally elevated pocked erythrocyte count). All were receiving intensive transfusion therapy, with the aim of maintaining the hemoglobin S level at less than 20%. The other seven patients had abnormal splenic function (absent radionuclide uptake and elevated pocked erythrocyte count); each was receiving less intensive transfusion therapy, with the pretransfusion hemoglobin S level usually at 30% to 40%. No patient developed bacterial septicemia while receiving hypertransfusion therapy. We conclude that splenic function during a long-term transfusion program is variable, depending in part on the "intensity" of transfusion therapy. Apparent splenic involution and fibrosis may be a reversible event in some patients.
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PMID:Splenic phagocytic function in children with sickle cell anemia receiving long-term hypertransfusion therapy. 279 47

Children with sickle cell anemia have an increased susceptibility to bacterial infections, especially to those caused by Streptococcus pneumoniae. We therefore conducted a multicenter, randomized, double-blind, placebo-controlled clinical trial to test whether the regular, daily administration of oral penicillin would reduce the incidence of documented septicemia due to S.pneumoniae in children with sickle cell anemia who were under the age of three years at the time of entry. The children were randomly assigned to receive either 125 mg of penicillin V potassium (105 children) or placebo (110 children) twice daily. The trial was terminated 8 months early, after an average of 15 months of follow-up, when an 84 percent reduction in the incidence of infection was observed in the group treated with penicillin, as compared with the group given placebo (13 of 110 patients vs. 2 of 105; P = 0.0025), with no deaths from pneumococcal septicemia occurring in the penicillin group but three deaths from the infection occurring in the placebo group. On the basis of these results, we conclude that children should be screened in the neonatal period for sickle cell hemoglobinopathy and that those with sickle cell anemia should receive prophylactic therapy with oral penicillin by four months of age to decrease the morbidity and mortality associated with pneumococcal septicemia.
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PMID:Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. 308 21

There were 37 maternal deaths among the 109,221 livebirths registered during the period 1977-86 in Bahrain, Arabian Gulf. The maternal mortality rate was 33.9/100,000 for the 10-year study period; however, disaggregation reveals a decline in this rate from 42.3/100,000 in 1977-81 to 26.9/100,000 in 1982-86. This decline presumably reflects streamlining of the Ministry of Health's maternity services, including a central maternity hospital with all modern facilities that serves as a referral center for all of Bahrain, 2 peripheral hospitals with provision for blood transfusion and surgical deliveries, and 3 maternity units managed by fully qualified midwives. About 80% of deliveries are covered by these maternity services; only 2.5% of deliveries occur in the home. Despite this highly developed maternity care system, 18 of the maternal deaths were due to direct obstetric cause: hemorrhage, 7; pre-eclampsia and eclampsia, 5; abortion septicemia, 2; bowel perforation during cesarean section, 1; thromboembolism, 2; and amniotic fluid embolism, 1. The causes of the 19 indirect maternal deaths were: pulmonary embolism, 5; infection, 7; cardiac failure, 2; cerebrovascular accident, 2; pulmonary hypertension, 1; and uncertain, 2. Of interest is the finding that sickle cell disease was the underlying cause of maternal death in 12 of the 37 deaths in this series. Sickle cell disease was implicated in 3 of the deaths from hemorrhage, all 5 deaths from pulmonary embolism, 2 deaths from septicemia, and the 2 cases of cardiac failure. In this series, 50% of the patients with sickle cell disease had thromboembolic crises following treatment of anemia with packed cell transfusion. Blood transfusion, especially of packed cells, should be given with caution to these patients since it may precipitate vaso-occlusive crisis by increasing blood viscosity. Since sickle cell disease represents a high risk during pregnancy in this Arab population, such patients should have frequent prenatal check-ups and deliver in a well-equipped hospital.
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PMID:Maternal mortality in Bahrain with special reference to sickle cell disease. 321 81

We studied the interactions of the A- variety of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell anemia (HbSS) to see if G6PD deficiency influenced laboratory and clinical features of HbSS. A total of 801 male patients over age 2 had G6PD electrophoresis on cellulose acetate membranes. Assays of both G6PD activity and hexokinase activity were then done on all samples that had an electrophoretic pattern other than the normal wild type (GdB). The collection of clinical data used a standardized protocol. Using cluster analyses we classified 10.4% males to be G6PD deficient, while 18.4% had the functionally normal GdA+ enzyme. The prevalence of G6PD deficiency did not change significantly when age was stratified by decade, suggesting little survival advantage or disadvantage of the combination of G6PD deficiency and HbSS. Compared to patients who were not G6PD deficient, there were no significant differences in the hemoglobin concentration, mean corpuscular volume, reticulocyte count, bilirubin, or SGOT level in patients with HbSS who had G6PD deficiency. The incidence of painful episodes, sepsis, or acute anemic episodes was similar in both groups. Our results are consistent with recent studies of smaller numbers of patients that have found little influence of G6PD deficiency upon HbSS. Specifically, we found no evidence that G6PD enhanced the severity of hemolysis or increased the incidence of acute anemic episodes or sepsis in HbSS.
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PMID:Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia. 334 44

This matched, case-control study was conducted on 68 neonates with sickle cell disease (SCD) to test the hypothesis that SCD contributes to neonatal jaundice. Previous uncontrolled studies have suggested that SCD leads to a high rate of neonatal jaundice. After matching, two neonates without SCD born in the same year were selected for each patient with SCD by use of random numbers. Matching factors were gestational age, sex, birth weight, and race. Serum bilirubin concentrations and the presence or absence of clinical jaundice were recorded. Information on factors potentially influencing the rate of neonatal jaundice was obtained for the first three days of life: maternal drug, alcohol, and tobacco usage, intrauterine infection, Apgar scores, highest infant hematocrit, culture-proved sepsis, blood group incompatibilities, hemorrhages, and presence of red blood cell sickling. We found no increase in the rate of clinical jaundice and no increase in the bilirubin concentration in either the entire group of patients with SCD, or in the subgroups with either homozygous or S-hemoglobin C disease, compared with their respective controls. We conclude that SCD probably is not a significant factor predisposing to neonatal jaundice.
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PMID:Jaundice in neonates with sickle cell disease. A case-control study. 335 2


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