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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In Omaha, from 1974 to 79, 30 (12.5%) of 240 patients with Haemophilus influenzae bacteremia or meningitis had a wide variety of conditions known to be associated with increased susceptibility to bacterial infection. Neonates and adults accounted for 47 per cent of the infections. Non-type b and non-typable strains caused 41 per cent of the episodes. Forty-one per cent of patients had bacteremia with no detectable focus of infection. The incidence of meningitis was low. Mortality was 28 per cent, considerably higher than in patients who were previously healthy. A review of the medical literature indicated that low-birth weight infants and patients with leukemia and other malignancies undergoing chemotherapy, splenectomy, congenital asplenia, sickle cell anemia, immunoglobulin deficiency diseases, cerebrospinal fluid shunts, and skull defects are at greater risk for systemic H. influenzae disease than the general population.
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PMID:Systemic Hemophilus influenzae infection. A study of risk factors. 697 94

Streptococcus pneumoniae is the most frequent bacterial cause of pneumonia, otitis media, and bacteremia and is the third most common cause of meningitis in infants and children. Infants have the highest rates of pneumococcal disease. Recent studies showed that the peak incidence of meningitis occurs among infants three to five months of age, that of otitis media occurs among infants six to 12 months of age, and that of hospitalization for pneumonia occurs among infants 13 to 18 months of age. A predominance of males was noted in most studies of pneumococcal disease. Blacks have higher rates of bacteremia and meningitis than do whites; this difference is explained only in part by the greater susceptibility to infection of children with sickle cell disease. However, the incidence of otitis media is lower in blacks than in whites. The predominant pneumococcal serotypes change with age, with time, and with geographic location. Certain serotypes are responsible for most cases of bacteremia or meningitis. Types present in the pneumococcal vaccine currently available represent 85% to 97% of the strains that have caused disease in the United States in recent years.
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PMID:The epidemiology of pneumococcal disease in infants and children. 702 43

Opsonisation of heat-killed baker's yeast, functional activity of the total alternative pathway of complement, and factor B detected functionally and immunochemically were significantly reduced in 72 children with sickle cell disease compared with 40 age-matched black control children. There was significant correlation between functional activity of the total alternative pathway and functionally measured factor B, but not between factor B measured functionally and immunochemically. The opsonisation defect could be corrected in vitro by normal serum, and factor B-depleted serum, and was qualitatively similar to that seen in patients with primary yeast opsonisation deficiency. Serial studies showed that these serum defects were persistent. Reduction in the activity of components of the alternative pathway of complement and opsonisation was found in 4 patients who had recovered from pneumococcal meningitis and in one who developed osteomyelitis. Defects of yeast opsonisation and complement which are common in patients with sickle cell disease, may partly explain the children's increased susceptibility to infection, and might help to identify individuals especially at risk.
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PMID:Defective yeast opsonisation and functional deficiency of complement in sickle cell disease. 709 89

Pneumococcal sepsis and/or meningitis are major causes of morbidity and mortality in young children with sickle cell disease. Abnormal complement activity, poor splenic function and a lack of type-specific pneumococcal antibody are responsible for the severity and frequency of these infections. A program consisting of early institution of antibiotic therapy for febrile episodes, antimicrobial prophylaxis, and administration of pneumococcal vaccine may be effective in reducing the incidence of pneumococcal disease. Specific guidelines for infection prevention are presented. Other infections that are more frequent or more severe in children with sickle cell disease (e.g., Salmonella, Haemophilus and mycoplasma infections) are also discussed.
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PMID:Infections in children with sickle cell anemia. Special reference to pneumococcal and salmonella infections. 716 89

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

Bacterial septicemia and meningitis are major causes of death in young children with sickle cell anemia. We report a prospective study of 182 episodes of fever among 22 children with sickle cell diseases identified at birth. The majority of patients with fever exceeding 39.5 degrees C were treated with antibiotics intravenously until results of blood cultures were known. Infectious diseases were documented in 38% of all febrile events. Six episodes of bacterial septicemia, two associated with meningitis, were successfully treated with no mortality or residual morbidity. These data form the basis of suggestions for management of the young child with sickle cell disease and fever.
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PMID:Fever in young children with sickle cell disease. 735 79

Acute pulmonary complications of sickle cell anemia are sickle cell lung disease and bacterial pneumonias. Chronic abnormalities in lung function include a restrictive ventilatory defect and perhaps increased venous admixture to the pulmonary circulation. Coexisting sarcoidosis may complicate sickle cell anemia and interact to potentiate sickling. Sickle cell lung disease, or acute "chest syndrome," occurs with greatest frequency in adults, is due primarily to pulmonary infarction, and may lead to cor pulmonale. On the other hand, bacterial pneumonia due to Streptococcus pneumoniae occurs with greater frequency in infancy and childhood. Mycoplasma and other organisms may also cause pneumonia with protracted illness and slow resolution. Bacteremia and meningitis may be further complications, particularly in children. Precise diagnosis of the acute febrile pulmonary episode is often difficult. In adults the illness is commonly self-limited. However, a vigorous diagnostic approach is warranted in all severely ill patients.
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PMID:The lung in sickle cell disease: a clinical overview of common vascular, infectious, and other problems. 746 92

Severe anemia has remained a major cause of morbidity and mortality in children of Southern Ghana since the early 1960s. Cases of anemia and anemia-associated mortality in the Korle Bu Teaching Hospital (KBTH), Accra, that occurred from January to December 1991 were reviewed. Data on hemoglobin levels, hypochromia, and malaria parasitemia of children referred from January to December 1991 were collected and analyzed to determine the prevalence of moderate/severe malaria parasitemia, anemia, and severe anemia. 10,989 (71.1%) of 15,450 children attending KBTH referred to the laboratory for hematological studies had hemoglobin (Hb) levels below 11.0 g/dl; while 3049 children (27.7%) of anemic patients had Hb levels below 7.0 g/dl. Of these 3049 children with severe anemia, 2185 (71.7%) had Hb levels below 5.0 g/dl, thus requiring urgent blood transfusion. Though the Department of Child Health alone utilized 32.2% of total blood processed by the National Blood Transfusion Service at KBTH, as many as 259 (58.1%) of the 554 deaths (306 male and 248 female) in the emergency room in children beyond the neonatal period were related to severe anemia. The main causes were nutritional anemia (n = 135), anemia associated with severe malaria (n = 56), anemia associated with sickle cell disease (n = 28), anemia associated with protein-energy malnutrition (n = 22), and 18 cases of anemia complicating gastroenteritis, pneumonia, meningitis, and convulsions. 108 (19.5%) deaths occurred because of neonatal sepsis, severe neonatal hyperbilirubinemia, meningitis and bronchopneumonia, severe anemia secondary to hemorrhage of the newborn, and faulty cord ligation. A significant decline occurred in the prevalence of childhood anemia in the developed world following improved counseling in nutrition, fortification of foods with iron, and iron supplementation to infants and schoolchildren with the attendant improvement in growth velocity and intellectual performance. A planned national anemia survey and early consideration of iron supplementation to older infants and preschool children at risk are recommended.
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PMID:Childhood deaths from anaemia in Accra, Ghana. 749 16

Forty-one Saudi Arabian children aged between six months and 13 years had a total of 68 admissions for complications of sickle cell anaemia during 1992. Review of their in-patient records and the relevant out-patient notes revealed a change in the clinical profile as compared to previous years. Hand-foot syndrome is virtually non-existent and vaso-occlusive crisis remains the commonest cause of admission. Introduction of pneumococcal vaccine and prophylactic oral penicillin has had no effect on the frequency of admissions and the admission rate per patient year. We have not seen any septicaemia/meningitis due to pneumococci. No death occurred during 1992.
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PMID:Sickle cell anaemia in Saudi-Arabian children. 764 41

Physicians analyzed the hospital records of 62 sickle cell anemia (SCA) patients who were admitted to the pediatric wing of the University Teaching Hospital in Lusaka, Zambia, between January 1987 and December 1989 and who died. They examined the case fatality rate and the causes of death. During this period, SCA patients comprised 938 of the 31,843 pediatric admissions (2.95%). The case fatality rate of these 938 urban SCA patients was 6.61%, which is much lower than the 1970 rate of 18.57%. The researchers attributed the lower case fatality rate to the comprehensive health care provided by the hospital's sickle cell disease clinic, established in 1971. Sickle cell-related deaths during the study period made up 0.97% of all pediatric deaths. The case fatality rate was 20.17% for all pediatric admissions. SCA-related mortality peaked in the 1-5 year old age group (38.71%) followed by the 6-10 year old age group (20.97%). As for causes of death, the case records of only 44 sickle cell-related deaths were available. The pediatricians were not able to specify the exact clinical diagnosis in 18 case files (29.03%). The major categories of causes of death were infections (29.54%), vaso-occlusive crises (22.72%), and splenic sequestration crises (20.45%). The infections included 6 cases of bronchopneumonia, 4 cases of confirmed malaria, 1 case of pneumococcal meningitis, and 1 case of HIV infection with cardiomyopathy. The researchers were not sure whether the HIV infection or SCA caused cardiomyopathy. An earlier study at the hospital found HIV seroconversion in more and more SCA patients. This study's major obstacles were poor record keeping, poor communication channels, inability to conduct autopsies due to social and cultural reasons, procedural delays, and unavailability of pathologists. These obstacles must be addressed to improve knowledge on death in SCA patients.
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PMID:Clinical analysis of mortality in hospitalized Zambian children with sickle cell anaemia. 783 62


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