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)

Hemoglobin SD-Los Angeles is an uncommon sickle hemoglobinopathy. We describe a boy with documented Hb SD-Los Angeles who had experienced acute splenic sequestration, pneumococcal sepsis, aplastic crisis and functional asplenia during his first two years of life. We suggest that children with Hb SD-Los Angeles are at similar risks for the life-threatening complications which characterize sickle cell anemia and should receive the same comprehensive medical care currently recommended for children with Hb SS disease.
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PMID:Life-threatening complications in a child with hemoglobin SD-Los Angeles disease. 654 33

The risk of Haemophilus influenzae septicemia/meningitis to children who have sickle cell anemia (SS) has been determined to be greater than that seen among normal infants. Of ten bacteriologically proven cases, eight episodes of infection were observed among 234 children with sickle cell anemia (645 person-years), who were less than 5 years of age. There was one case per 69 infants with sickle cell anemia who were less than 18 months old and one case per 36 children with sickle cell anemia between 19 and 59 months of age. Unexpectedly, two infections occurred among 224 children (824 person-years), aged 5 to 9 years; both died. Contrary to the rapid clinical course of pneumococcal infections in children with sickle cell anemia H influenzae septicemia was regularly heralded by a greater than 24-hour prodrome of upper respiratory tract infection, low-grade fever, and otitis media. Three (30%) preventable deaths occurred. Antibiotic therapy for the febrile child with sickle cell anemia must be predicated on the known 400-fold increased risk of pneumococcal septicemia in those less than 5 years old and the fourfold risk of H influenzae septicemia in those less than 9 years of age.
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PMID:Is there an increased risk of Haemophilus influenzae septicemia in children with sickle cell anemia? 660 25

Morbidity and mortality patterns were examined among 968 pediatric patients on the island of Dominica. These children, whose ages ranged from newborn to 13 years, were seen by the consulting pediatrician at Princess Margaret Hospital during a 9-month period in 1978-79; 852 children were seen as inpatients. A total of 477 cases of infectious disease were diagnosed among inpatients alone. Stool examination in a subsample of these children revealed parasites (mostly Trichuris) in roughly half. Also found was a relatively high prevalence of chronic health problems, especially rheumatic heart disease (34 cases), mental retardation (28 cases), epilepsy (31 cases), and sickle cell anemia (21 cases). Examination of the hospital records of 100 of the inpatients ages 6 months-5 years demonstrated that 34% were low weight-for-age according to the World Health Organization classification. There were 34 deaths (9 pediatric patients and 255 newborns). The high neonatal mortality is attributed to an unusually high incidence of immaturity and prematurity, irregular and insufficient hospital oxygen supply, and a septicemia epidemic. Although these findings reflect patterns of the more serious diseases, they could be useful in planning preventive health measures. The high prevalence of malnutrition points to a need for nutrition education, promotion of breastfeeding, promotion of vegetable growing, and the introduction of a home-based growth chart. The high incidence of diarrhea, typhoid fever, and helminthiases highlights problems with general hygiene, latrines, and water supply. There is also a need for follow-up facilities for children with rheumatic heart disease, epilepsy, and sickle cell anemia. It is suggested that hospital care could be improved by dividing pediatric and neonatology wards into 5 units: isolation ward, malnutrition ward, semi-intensive care unit, general pediatrics, and pediatric surgery.
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PMID:Morbidity and mortality patterns among pediatric patients in Dominica (West Indies). 662 10

Despite the lack of successful drug development during the last 10 years, significant advances have been made in the management of a number of the complications of sickle cell disease. Identification of the vascular changes in the retina has led to the use of argon or zenon laser therapy to prevent progression of these changes to retinal hemorrhage and loss of visual acuity. Aseptic necrosis of the head of the femur or humerus is now successfully managed with the insertion of a bone graft or prosthesis. Exchange transfusions are believed to have prevented recurrent cerebrovascular accidents. The use of prophylactic penicillin and pneumococcal vaccine may have reduced the incidence of sepsis, pneumonia, and meningitis due to susceptible organisms in young children. While this progress can be said to have improved the quality of life of many patients, other complications of sickle cell disease remain as therapeutic dilemmas. The ability to prevent complications is hampered both by a lack of specific therapeutic measures and ignorance as to which patients are likely to develop complications.
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PMID:Management of sickle cell disease: progress during the past 10 years. 667 Jul 16

Polyvalent pneumococcal vaccine and oral penicillin prophylaxis are frequently used in patients with functional or anatomic asplenia to protect them from fulminant Streptococcus pneumoniae sepsis. We studied nasopharyngeal colonization with pneumococci in 34 patients with sickle cell anemia (aged 6 months to 5 years) receiving penicillin prophylaxis and in 63 age- and race-matched comparison patients. Patients with sickle cell anemia had fewer positive initial pneumococcal nasopharyngeal cultures than did the comparison group (14.5% vs 34.4%, P = 0.03) and significantly lower carriage rates during the respiratory illness season of November to March (8.7% vs 40.5%, P = 0.005). Penicillin prophylaxis did not result in emergence of penicillin-resistant pneumococci or in an increased carriage rate of Haemophilus influenzae type b. Our data suggest a mechanism of action for penicillin prophylaxis and provide some evidence for the relative safety of this regimen.
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PMID:Effect of penicillin prophylaxis on nasopharyngeal colonization with Streptococcus pneumoniae in children with sickle cell anemia. 669 Jun 71

Fifty of 52 infants with cord blood hemoglobin electrophoresis patterns indicative of a possible sickling hemoglobinopathy (SH) were followed up prospectively. A retrospective group of 39 children whose mothers were contacted two to four years after the birth of a child with a positive newborn screening test formed a comparison group. During an average follow-up period of 30 months, children of mothers who were informed early of a positive cord blood test were hospitalized more readily for complications of an SH. During this same period, five (6%) of 88 infants with fetal and sickle hemoglobin (FS) or fetal, sickle, and C hemoglobins (FSC) in their cord blood died, all before 6 months of age. All five deaths occurred in infants whose parents were unaware of their child's disease; four of the five deaths may have been caused by sepsis. These results alone did not prove that screening significantly reduces mortality but did show that newborn screening increases parental awareness of complications and may, therefore, improve the prognosis in sickle cell disease in infancy.
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PMID:Newborn screening for sickling hemoglobinopathies. Houston, 1976 to 1980. 669 13

Splenic dysfunction accounts for the greatest deficit in immune function in children with homozygous SCD. This dysfunction, coupled with the natural immunologic immaturity of all young children subjects the young child with SCD to an immense risk of severe pyogenic infections. To data, experience would suggest that pneumococcal vaccines may provide only modest protection in the child with SCD less than 5 years of age. Antipneumococcal antibody responses are poor in children less than two years of age. All reported failures of pneumococcal vaccine among children with SCD have occurred in children less than three years of age and offending pneumococci have been of groups 6 and 23. Prophylactic antibiotic regimens have yet to be submitted to rigid scientific investigation and their utility in eliminating the risk of pneumococcal sepsis in asplenic hosts is unknown.
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PMID:Infections in sickle cell anemia: pathogenesis and control. 703 72

One-hundred seventy-four children with sickle cell disease (SCD) were immunized with a single dose of tetradecavalent pneumococcal vaccine. Preimmunization and postimmunization antibody against 13 of the 14 pneumococcal capsular antigens was measured by indirect hemagglutination (IHA). The ability of each antigen to stimulate antibody following immunization was characterized by one of three types of responses: (1) poor antibody response regardless of the age at immunization (capsular types 6A, 14, and 19F); (2) improving antibody response with advancing age at immunization (capsular types 1, 4, 9N, 12F, 18C, and 23F); and (3) good antibody response regardless of age at immunization (capsular types 2, 3, 7F, and 8). An increase in antibody following immunization was significantly correlated (P less than 0.0005) with an increasing level of preimmunization antibody titer for all 13 antigens. Through the first 24 months of study, two episodes of pneumococcal sepsis caused by group 23 pneumococci were documented in two children immunized prior to 24 months of age (incidence rate, 4.40/100 patient-years in children less than 5 years of age), and one additional episode caused by a group 23 pneumococcus occurred in a 5 7/12-year-old child (incidence rate, 0.66/100 patient-years in children greater than 5 years of age). These observations suggest that anamnestic immune response significantly contributed to the enhanced antibody response observed in older children and adults. Only modest vaccine efficacy may be expected among children with SCD who receive a single dose of pneumococcal vaccine.
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PMID:Pneumococcal polysaccharide immunization of children with sickle cell disease. II. Serologic response and pneumococcal disease following immunization. 709 74

One measure used to prevent overwhelming sepsis due to Streptococcus pneumoniae in children with defective splenic function is oral penicillin prophylaxis. However, a frequently cited argument against this approach is the likelihood of poor compliance. Compliance was studied by examining urine specimens for penicillin by the Sarcina lutea disc diffusion technique in 22 surgically asplenic children, two patients following bone marrow transplantation, and 38 infants and young children with sickle cell disease. Multiple specimens (mean 3.5 per patient) were examined in 43 of the children. Overall, 125/188 (66%) of the urine samples contained penicillin, indicating compliance within the previous 12 to 24 hours. Compliance tended to improve on subsequent clinic visits. These relatively good results were attributed to an intensive educational program in which repetitive efforts are made to counsel patients and parents about the risks of life-threatening infection. Poor compliance should no longer be invoked as a reason not to study the efficacy of prophylactic penicillin in functionally or surgically asplenic subjects.
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PMID:Oral penicillin prophylaxis in children with imparied splenic function: a study of compliance. 714 49

Streptococcus pneumoniae infection has been the predominant cause of death among children with sickle cell anemia (SS). We report our observed change in the pattern of progression of septicemia to meningitis and death in nonimmunized SS children who were not receiving prophylactic penicillin in the face of a persistently high incidence of pneumococcal disease. Of 233 SS children less than ages 6 years observed for 781 person-years, the overall incidence rate of pneumococcal septicemia was 5.9 episodes per 100 person-years. Prior to July 1972, of 23 children who had pneumococcal septicemia, eight (35%) died and meningitis developed in 15 (65%), whereas since July 1972, 11 children have had pneumococcal septicemia, but no children died and meningitis developed in only two (18%). This decrease in major morbidity is attributed to the establishment of a clinical program that provides close medical supervision of the SS child with fever and the rapid institution of parenteral antibiotic therapy.
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PMID:Pneumococcal septicemia in children with sickle cell anemia. Changing trend of survival. 723 Mar 69


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