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

Newborn screening for sickle cell disease has been recommended as a method of decreasing patient mortality. However, its effectiveness in accomplishing this has not been reliably measured. To help determine the effectiveness, 10 years of experience in newborn screening have been summarized. The effects of early patient enrollment in a comprehensive treatment program on long-term morbidity and mortality are reported. From 1975 to 1985, 84,663 newborns were screened regardless of race or ethnic background. Bart's hemoglobin was present in 5%, hemoglobin AS in 2.6%, and hemoglobin AC in 0.75%. Excluding Bart's, approximately 3.6% of all newborns were carriers for hemoglobinopathy. Sickle cell disease occurred in 1:951 births (58 hemoglobin SS, 25 hemoglobin FSC, three hemoglobin S-beta +-thalassemia, and three hemoglobin S-beta O-thalassemia). In addition, one in every 4,233 newborns had a clinically significant thalassemia syndrome (eight hemoglobin FE, ten hemoglobin F only, two hemoglobin H). Compared with other newborn screening programs in California, (congenital hypothyroidism, 1:3,849; phenylketonuria 1:22,474, galactosemia 1:74,103), hemoglobinopathies are the most prevalent congenital disease. Eighty-one newborns with sickle cell disease were followed for 7.2 years. Patients experienced 513 hospitalizations, including 13 episodes of sepsis with or without meningitis and ten acute sequestration crises. The overall mortality rate for patients with sickle cell anemia diagnosed in the newborn period was 1.8%. In comparison, the clinical course of 64 patients with sickle cell anemia diagnosed after 3 months of age and followed for an average of 9.4 years was analyzed. Five of these patients died. In two of these, sickle cell anemia was diagnosed at the time of the death.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Newborn screening for sickle cell disease: effect on mortality. 336 74

Kingella kingae is a rare human pathogen. Most reported infections are in children and involve endocardium, vascular space, and skeletal tissues. We report herein two cases of K. kingae infection recently seen in adults. Kingella kingae caused acute meningitis in a patient with sickle cell anemia and in the second patient with alcoholic liver disease, sepsis with a petechial rash. The clinical presentation due to K. kingae closely resembled that caused by related Neisseria genus.
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PMID:Manifestations of Kingella kingae infections in adults: resemblance to neisserial infections. 370 97

A fatal case of pneumococcal septicaemia and meningitis is described in a vaccinated nine-year-old Saudi child with sickle cell disease. Non-compliance to prophylactic penicillin might have been the cause of its fatal illness.
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PMID:A fatal pneumococcal infection in a vaccinated 9-year-old child with sickle cell disease. 375 Mar 99

Three hundred and twenty five episodes of pneumococcal bacteraemia occurred at St Thomas's Hospital during 1970-84, accounting for 13.3% of all episodes of bacteraemia. Twice as many cases occurred in male as in female patients, and common predisposing factors included chronic chest disease, alcoholism, haematological malignancies, cirrhosis, and sickle cell anaemia. Mortality was 28.6% overall but only 11.8% among patients who received antibiotic treatment for at least 24 hours. Most patients (261) had pneumonia, 26 had meningitis, and eight were children with occult bacteraemia. The commonest serotype of pneumococcus in adults was type 3 (39 episodes), and these strains were associated with a high mortality. Other factors determining a fatal outcome included underlying disease (such as cirrhosis, malignancy, and chronic chest disease) and extrapulmonary infection. Almost half the survivors were treated for 10 days or less and became afebrile within 48 hours.
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PMID:Pneumococcal bacteraemia: 325 episodes diagnosed at St Thomas's Hospital. 391 50

Streptococcus pneumoniae is the leading pathogen in children with sickle cell disease. Forty children younger than 20 years of age who had sickle cell disease and septicemia, meningitis, or osteomyelitis/septic arthritis were identified. The causes included Streptococcus pneumoniae (20%) and gram-negative organisms (mainly Salmonella) (70%). The gram-negative infections occurred in the first decade of life in 45% of our patients. We believe that this pattern of infection is different and related to the mild nature of sickle cell disease in our patients and to their persistent splenic function. The administration of pneumococcal vaccination may also have played a role. Microinfarcts of the intestinal wall allow the access of gram-negative organisms to the circulation. In places where gastrointestinal tract infections, especially Salmonella, are common, antibiotic therapy effective against these organisms is recommended initially with adjustment after identification and sensitivities are known.
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PMID:Pattern of bacterial infections in homozygous sickle cell disease. A report from Saudi Arabia. 402 63

Persons with sickle cell anemia who have elevated fetal hemoglobin or lowered erythrocyte mean corpuscular volume are reputed to have less severe clinical manifestations and a greater probability of survival. This study examines the relationship between seven clinical indicators of morbidity in sickle cell anemia and seven hematological parameters that were collected from 214 patients. Risks of sickle cell crisis, acute chest syndrome, hospital admissions, cerebrovascular accident, aseptic necrosis, meningitis/septicemia, and death were used as indicators of morbidity. The hematological parameters included percent fetal hemoglobin, absolute fetal hemoglobin, percent hemoglobin A2, hemoglobin concentration, packed cell volume, mean corpuscular volume, and mean corpuscular hemoglobin concentration. Statistical analyses of the data showed no relationship between the hematological parameters and six of the seven clinical indicators of the severity of sickle cell anemia. The only significant finding was an increased risk of stroke in those patients with lower levels of fetal hemoglobin. Therefore, with this exception, there is no predictable relationship between morbidity and mortality in sickle cell anemia and levels of fetal hemoglobin or erythrocyte indices. Thus, the general belief that there is an association between severity of sickle cell anemia and the levels of fetal hemoglobin has not been established.
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PMID:Lack of influence of fetal hemoglobin levels or erythrocyte indices on the severity of sickle cell anemia. 615 92

Overwhelming infections caused by encapsulated bacteria, salmonella spp. and Plasmodium falciparum (in malarious areas) are an important cause of morbidity and death in patients with sickle cell disease. Bacterial infections afflicting these patients include fulminant meningitis and septicaemia caused by Str. pneumoniae and H. influenzae type b, and non-typhoid salmonellosis. Children less than five years of age are at greatest risk for meningitis and septicaemia, while salmonella osteomyelitis is probably common to all age groups. The most important contributing factors to this increased susceptibility to encapsulated bacteria are: a state of functional asplenia, an opsonophagocytic defect due to an abnormality of the alternative complement pathway, and a deficiency of specific circulating antibodies. Devitalisation of gut and bone due to repetitive vaso-occlusive crises, saturation of the macrophage system with red cell breakdown products of chronic haemolysis, and underlying splenic and hepatic dysfunction all predispose to salmonella infections. Seventy per cent of septicaemias and meningitis among under-fives with sickle cell disease is caused by Str. pneumoniae. Septicaemia frequently presents with sudden fever, few prodromal features, and a deceptive appearance of well-being, followed within hours by rapid relentless progression to shock and death. Adrenal haemorrhage is common, and mortality can be as high as 50 per cent, unless intravenous antibiotic, with or without steroid therapy, is promptly initiated. The clinical presentation of bacterial meningitis, its management and mortality follow the normal patterns, but recurrent meningitis and cerebrovascular morbidity are common in patients with sickle cell disease. An acute pulmonary involvement, indistinguishable from bacterial pneumonia (the 'chest syndrome') is the commonest single complication of sickle cell disease at any age. Str. pneumoniae is responsible for about half of the episodes. The protective values of the pneumococcal vaccine and long-term penicillin prophylaxis remain to be established in sickle cell disease. Over 70 per cent of haematogenous osteomyelitis in sickle cell disease is caused by salmonellae. The distinction from vaso-occlusive bone crisis is often difficult, but the presence of multiple, often symmetrical bone involvement, diaphyseal fissuring and involucrum should suggest osteomyelitis rather than bone infarction. Chloramphenicol remains the drug of choice and often has to be given in high doses for up to six weeks. The role of surgery is limited by the presence of multiple bone involvement and the known anaesthetic risks in this group.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Sickle cell disease and infection. 631 9

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

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

A total of 348 cases of meningitis caused by Streptococcus pneumoniae were reported to and investigated by the New York City Department of Health during the six years 1973-1978. The descriptive epidemiology and completeness of reporting of this disease entity are presented. The total crude incidence was estimated at 1.46 cases per 100,000 population per year. The high-risk age categories were less than 5 years and greater than or equal to 65 years. The incidence of pneumococcal meningitis for blacks with sickle cell disease was much greater than that for other blacks, who, in turn, had a higher rate than whites. The overall case fatality ratio was 0.590. The case fatality increased with increasing age and was higher in nonwhites than in whites. Twenty-four per cent of all cases had predisposing chronic conditions which would have qualified them for the polyvalent pneumococcal vaccine under the present guidelines.
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PMID:The descriptive epidemiology of pneumococcal meningitis in New York City. 683 60


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