Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twelve cases of pneumococcal septicemias are studied in a department of internal medicine. These septicemias involved pneumonias often large, multilobular and bilateral, with often pleural effusion purulent or not and in one occurrence meningitis. There was three deaths by acute respiratory failure in one case of liver cirrhosis and in another case of chronic lymphocytic leukemia. Conditions of antibiotherapy and possible intensive care are discussed, prognosis factors as splenic insufficiency (in sickle cell anemia and after splenectomy) and immune defense against pneuococcus are recalled.
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PMID:[Remarks about twelve cases of pneumococcal septicemias (author's transl)]. 21 13

A total of 422 patients with sickle cell disorders have been observed for 3,442 patient years. During this period, 53 episodes of septicemia or meningitis occurred, indicating a risk of 12.5% from these infections for each individual. If only patients with SS hemoglobinopathy (sickle cell anemia) (323 patients) are considered, the risk was 15.2%. The case fatality ratios for sepsis and meningitis were 35% and 10%, respectively. Disease due to Streptococcus pneumoniae occurred, almost exclusively, among children with SS hemoglobinopathy who were less than 5 years of age. After the first decade, illnesses among patients with all types of sickle cell disorders were frequently associated with an identifiable source of infection, a chronic course, and frequent involvement of Gram-negative organisms.
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PMID:Bacterial meningitis and septicemia in sickle cell disease. 87 15

Advances in imaging hardware for positron emission tomography and single-photon emission CT, coupled with a wide variety of radiopharmaceutical agents, have allowed these techniques to be used in the evaluation of neoplasm, stroke, epilepsy, and dementia. Cerebral perfusion agents continue to be the mainstay of single-photon emission CT imaging but, in addition to the evaluation of ischemia, it has seen an increasing role in the study of dementia, neuropsychiatric disorders, and seizures. Positron emission tomography scanning has had similar applications but it is playing a greater part in the evaluation of neoplasms, including primary gliomas and pituitary adenomas. Stable-xenon CT has shown value in the study of ischemia associated with meningitis, sickle cell disease, chronic subdural hematomas, and cerebral arteriovenous malformations. MR diffusion imaging shows promise in the evaluation of white matter pathology and some tumors.
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PMID:Physiologic imaging of the brain. 173 6

Maternal mortality is examined from June 1980 to December 1986 at Mulago, Nsambyo, Old Kampala, Rubaga, and Mengo Hospitals in Kampala, Uganda. Clinical or immediate causes, direct and indirect, were recorded from case summary forms based on ICD9 definitions of obstetric complications. The nonabortion maternal mortality rate (NAMMR) was 2.65/1000 deliveries (580 deaths); the abortion-related maternal mortality rate (ARMMR) was 3.58/1000 abortions. The hospital maternal mortality rate was 2.0/1000 deliveries. 75% of maternal deaths of women of 28 weeks' gestation or more had delivered outside the hospital. NAMMR doubled between 1980-86, a statistically significant increase. ARMMR increases were almost significant. 75% were direct obstetric and 21% were indirect obstetric causes. 38% had clinical anemia, 29% had some sepsis, 18% had substantial bleeding, and 14% had obstructed labor. Other contributing conditions were pneumonia, ruptured uterus, laparotomy, evacuations and curettage, malaria, preeclampsia, sickle cell anemia, pulmonary embolism, malnutrition, tetanus, meningitis, prolonged labor, and hepatitis. At admission, 48% were in poor condition, 30% in good condition, and 22% in fair condition. 27% had sickle cell anemia, high blood pressure, multiple pregnancy, or malaria at admission. 64% were admitted within 24 hours after delivery, 67% 1-7 days after delivery, and 92% 7-42 days after delivery. Those in good condition were all admitted 7 days postdelivery. 41% of deaths were due to lack of drugs, 7% lack of fluids, 20% with theater problems, 14% with doctor-related factors, and 3% with midwife-related factors. Better information is needed on mortality before delivery, mortality in hospitals vs. outside, and mortality from abortion, and ectopic and hydatidiform molar pregnancies. An explanation given for the increase in maternal mortality is the decline in economic conditions. Abortion complications may be due to the concealment practiced. Causes are consistent with trends from the 1950s, 1960s, and 1970s in Uganda and developing countries in general. Availability and accessibility of gynecological and obstetric services needs great improvement. Training traditional birth attendants and obtaining rural ambulance services are also needed. Health workers lack creativity and imagination for developing country conditions; scarce resources are not the only problem.
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PMID:Incidence and causes of maternal mortality in five Kampala hospitals, 1980-1986. 176 15

Streptococcus pneumoniae is the primary cause of community-acquired pneumonia, meningitis in adults and otitis media in infants and children and the third cause of meningitis in infants and children. Despite the availability of effective therapeutic agents against this pathogen, mortality has remained high, particularly for infections complicated by bacteremia. For many years, there has been a plea for vaccination. The first steps, using whole bacterial vaccines, were taken during the early decades of this century in the gold mining camps of South Africa, where pneumonia was endemic. The efficacy of purified pneumococcal polysaccharide vaccines has since been demonstrated in young adults, such as gold miners and military recruits, as well as for several other groups at risk, such as institutionalized elderly, patients with sickle cell anemia or those who have undergone a splenectomy, and elderly patients with underlying conditions such as chronic obstructive pulmonary disease and chronic cardiovascular disease, but not in infants and severely immunocompromised patients. Serological studies on the immune response to inoculation of pneumococcal polysaccharide antigens have demonstrated a severely impaired antibody response in the last two groups. Therefore, development of more highly immunogenic vaccines, e.g. by linking pneumococcal polysaccharides or parts of them to protein carriers, should be continued in an attempt to offer adequate protection to those who are insufficiently protected by the current 23-valent polysaccharide vaccine. Opportunities to immunize other patients who are at risk for pneumococcal infection and are capable of responding to the current vaccine should not be missed.
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PMID:Pneumococcal polysaccharide vaccines: indications, efficacy and recommendations. 179 58

Ninety-nine Saudi Arabian children aged between 3 months and 15 years attending the sickle cell anaemia (SCA) clinic were studied using a review of case notes and an interview at clinic visits. All the patients come from the southwest and west area of Saudi Arabia. Despite the lack of infant screening for SCA, 73 per cent were diagnosed before the age of 3 years, reflecting severe early symptoms of the disease. The serious complications of SCA were two times higher, the number of admissions were three times higher and the attack rate for pneumococcal meningitis was nine times higher in our study population than previously reported from the Eastern Province of the country. Hand-foot syndrome was the commonest complication affecting 58 per cent of the children. It has been stated previously that there is no need to give penicillin prophylaxis to SCA children in Saudi Arabia, based on the observations in the Eastern Province only. Our study shows that SCA in western and southwestern population of Saudi Arabia is as severe as is described in American blacks. Therefore, we recommend penicillin prophylaxis to be given to the SCA children in the southwest and west regions of the country.
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PMID:A clinico-epidemiological study of sickle cell anaemia in Saudi Arabia. 186 Dec 79

Mortality of infants aged 0-30 months was studied in a subdistrict of the eastern Ivory Coast with a population of 240,000 inhabitants. A cluster sample of the type recommended by the World Health Organization for evaluating progress of the expanded program of immunizations consisted of 2 samples with 30 clusters of 70 children each, 1 taken in urban Abengourou and the other in rural cantons of the subprefecture of Abengourou. A standardized questionnaire was administered to all the mothers about their births within the last 3 years. Supplementary questionnaires concerning all deaths of children of the sample mothers were interpreted by 3 physicians who agreed on a probably diagnosis in each case. The survey covered 2375 infants under 1 year and 1825 aged 12-30 months. The total mortality was 103 deaths in the total sample and 70 for infants aged 0-11 months, for a rate of 29.4%. The difference between the urban rate (31.7%) and the rural rate (26.8%) was not significant. The rate varied significantly by sex for deaths due to malnutrition (11 boys, 1 girl), and pneumopathies (6 girls, 1 boy). Mortality varied significantly according to treatment received and place of death. 55% received traditional treatment and 45% modern treatment. 53% died at home, 36% at a health center, and 9% at the home of a healer. Among infants aged 0-27 days, the cause of death was tetanus for 8, prematurity for 12, neonatal distress for 5, neonatal jaundice for 5, and infection for 2. Among infants aged 1-11 months the cause of death was malaria for 10, meningitis for 7, tetanus for 2, diarrhea for 9, pneumopathy for 3, measles for 4, whooping cough for 2, and unknown for 1. Among infants aged 12-30 months the cause of death was malaria for 11, malnutrition for 12, meningitis for 3, pneumopathy for 4, measles for 1, and sickle cell anemia for 2. Malaria was the single most important cause of death followed by malnutrition for the overall sample. In urban and rural areas respectively, the proportions of infants correctly vaccinated for their age groups were 78.1% and 76.0% for those under 11 months; 92.3% and 80.6% for those 12-17 months; 78.3% and 76.6% for those 18-23 months; and 66.5% and 71.4% for those 24 months and over. Mortality rates varied very significantly by vaccination status. 70 of the children dying had not been vaccinated. Their mortality rate was 19.6%, compared to .5% for children in process of vaccination, 1.1% for children incompletely vaccinated, and .9% for children correctly vaccinated.
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PMID:[Infant mortality and its causes in a sub-district of the Ivory Coast]. 196 15

The major cause of death in sickle cell anaemia is from infection, especially infection caused by Streptococcus pneumoniae. Meningitis, pneumonia and septicaemia caused by this organism are the primary types of infection leading to death. Children under three years of age are at highest risk. We have known for over twenty years that approximately 30 per cent of the infants born with sickle cell anaemia will become infected in the first three years of life and one-third can be expected to die from the infection. These data were the reason that we conducted the Prophylactic Penicillin Study (PROPS), a trial to investigate the effectiveness of oral prophylactic penicillin in preventing severe infection due to S. pneumoniae. This investigation was a very efficient, cost effective study because of its timeliness and its conduct within the framework of an ongoing study. Moreover, the question being answered was simple and focused with up-to-date data that permitted accurate estimates of sample size and incidence.
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PMID:Sickle cell anaemia trial. 211 33

Patients with sickle cell disease are predisposed to infection caused by Streptococcus pneumoniae. However, there has been only one published case of bacteremic pneumococcal meningitis in an adult with sickle cell anemia. We report here the cases of six adults with sickle cell disease, pneumococcal sepsis, and meningitis. Five patients were male and one was female. Their ages ranged from 18 to 34 years (mean, 25.7 years). Five patients had the SS and one had the SC hemoglobin phenotype. Only one patient had received pneumococcal vaccine (14 valent). This vaccine did not protect against the pneumococcal serotype causing his infection. All patients had high fever (mean, 39.8 degrees C [103.7 degrees F]) on admission; five had generalized weakness and four had neck stiffness. Leukocyte counts were greater than 30,000/mm3 in all patients. Streptococcus pneumoniae was isolated from the blood and the cerebrospinal fluid in all patients. The cerebrospinal fluid showed pleocytosis in six patients, an elevated protein level in five, and hypoglycorrhachia in two. Complications included renal failure in four patients, disseminated intravascular coagulation in one, and seizures in another. Two patients died. Pneumococcal sepsis and meningitis are uncommon in adults with sickle cell disease, but they carry a high morbidity and mortality. Wider use of the new 23-valent polysaccharide vaccine in these patients is recommended.
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PMID:Pneumococcal sepsis and meningitis in adults with sickle cell disease. 240 40

This review article discusses the stages in the development of research on group B streptococcus (GBS), otherwise called Streptococcus agalactiae. Emphasis was placed on the bacteriology, clinical spectrum of disease, immunity to GBS infections and antibiotic susceptibility of the causative organism. The organism, first recognized by Billroth in 1873, is classified into order Eubacteriales, family Lactobacillceae, class Schizomycetes and genus Streptococcus on the basis of its biochemical and physiological characteristics. It is subdivided into types Ia, Ib, Ic, II, III, X and R on the basis of carbohydrate and protein antigens present on its cell wall. Bovine strains of GBS are found in the bovine teat while human strains are present in the female vagina, the oro-pharynx, anorectum and the external auditory canal of newborns. It could be transmitted vertically from mother to child in-utero and during parturition. Cross infection by the nursery staff could also occur during the immediate post partum period. Two types of diseases are caused in the newborn: the early disease occurring within a week of birth; and the late disease presenting during the late neonatal period. The former usually presents in the form of septicaemia while the latter presents as meningitis. Adult infections include puerperal sepsis, pyelonephritis and a wide range of other infections. Usually they are associated with other underlying clinical conditions such as malignancy, diabetes mellitus and sickle cell disease. The organism is sensitive to penicillin which is the drug choice in treating established infections by GBS. Control measures are based on treatment of cases, eradication of vaginal colonization and chemoprophylaxis of infants at risk. An effective vaccine may become available in the near future.
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PMID:Review of group B streptococci and their infections. 241 64


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