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)

We compiled the results of pars plana vitrectomy in rare proliferative and hemorrhagic diseases. In retinal vasculitis of unknown origin (perhaps Eales' disease), six out of seven eyes had visual improvement; in sickle cell disease, two out of three eyes improved. Three eyes with presumed toxocariasis were successfully treated. In retrolental fibroplasia (RLF), only lighter degrees of the cicatricial stage with traction detachments can be treated. Some cases may simulate Coats' disease. There are also cases which simulate the typical picture of RLF, where there is no history of prematurity or oxygen usage. Five patients with RLF were treated, three of these successfully. Cryoglobulinemia (one case) and hereditary hemorrhagic telangiectasia (one case) were successfully treated. Senile macular degeneration can also be an indication for vitrectomy (two out of three eyes were successfully treated).
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PMID:Results of vitrectomy for rare proliferative and hemorrhagic diseases. 57 91

Erythropoietin is a glycoprotein hormone that plays a vital role in erythropoiesis. It is mainly produced in the fetal liver till the third trimester of pregnancy. At that point, the kidney interstitium takes over this function and becomes the main source of erythropoietin. Hypoxia stimulates erythropoietin production by a mechanism that may require a heme protein as a second messenger. Erythropoietin stimulates the maturation of erythroid precursors (colony-forming unit-erythroid and burst-forming unit-erythroid) via at least two types of cell surface receptors. The higher-affinity receptors appear to be more important in modulating the effects of erythropoietin in vivo. Changes in intracellular calcium may ultimately mediate the action of erythropoietin on erythroid precursors. A specific and sensitive radioimmunoassay is now available for accurately measuring erythropoietin levels. All forms of erythrocytosis except polycythemia vera are associated with elevated erythropoietin levels. Levels are also high in cord blood obtained following fetal asphyxia. Reduced levels are seen in patients with anemia due to renal diseases. The response of erythropoietin to the degree of anemia appears to be attenuated in patients with cancer, chronic diseases, and human immunodeficiency virus (HIV) infection. Erythropoietin has been successfully used for treating patients with anemia due to renal failure. Its use has also been approved for the treatment of anemia patients receiving zidovudine for HIV infection. Encouraging results have been observed when erythropoietin was used to treat anemia due to rheumatoid arthritis, hematological malignancies, and prematurity. It has also been used to increase the yield of autologous blood collected prior to an elective surgical procedure. However, it has not proved to be useful in sickle cell anemia and myelodysplastic syndromes.
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PMID:Erythropoietin. Biology and clinical applications. 178 66

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

Recombinant human erythropoietin represents a potential therapeutic alternative to red blood cell transfusions in a number of pediatric anemias. It is effective in correcting anemia associated with chronic renal failure and may significantly reduce the morbidity associated with childhood CRF. Most exposures to allogeneic blood products in pediatrics for treatment of anemia with blood transfusions occur in neonatal intensive care units. If proven effective in treating anemia in premature babies, r-HuEPO will be responsible for a major reduction in the use of blood transfusions in clinical neonatology. Carefully designed, placebo-controlled clinical trials will be required to establish the role of r-HuEPO in anemia of prematurity. Recombinant human erythropoietin also may be useful to increase the amount of blood that can be collected before elective surgical procedures. Another potential indication is to raise the hematocrits of infants with large intracardiac shunts who develop congestive heart failure coincident with the developmental fall in hemoglobin concentration after birth. Finally, r-HuEPO may one day play a role in modifying the expression of globin genes and, thereby, ameliorate the course of sickle cell disease and beta thalassemia. Many questions surrounding the use of r-HuEPO in infancy and childhood are being addressed in ongoing clinical trials.
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PMID:Recombinant erythropoietin in pediatrics: a clinical perspective. 218 91

When pregnancy occurs in women suffering from major sickle cell disease there is a double risk for the mother and for the fetus. Pregnancy favours the development of the classical complications of the disease and fetal distress in utero or prematurity bring with them an increased level of perinatal morbidity and mortality. Routine blood transfusion carried out prophylactically might seem to be a solution that lessens the risk. In fact, a comparative study of two series of patients did not give rise to the conclusion that there was any benefit from carrying out the procedure. Careful supervision of the progress of the pregnancy would seem to be the one essential factor to improve the prognosis.
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PMID:[The value of systematic prophylactic blood transfusions in the outcome of pregnancies in patients with severe sickle cell anemia. Study of 63 deliveries in Guadeloupe]. 345 21

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

From 1974 to 1990, 336 Bacteroides isolates were obtained from 312 specimens from 274 patients. They comprised 180 (54%) B. fragilis isolates, 55 (16%) B. theta-iotaomicron, 36 (11%) B. vulgatus, 34 (10%) B. distasonis, 21 (6%) B. ovatus and 10 (3%) B. uniformis. Infections in 253 (92%) patients were polymicrobial, but in 21 (8%) children, a Bacteroides sp. was isolated in pure culture. Most Bacteroides isolates were from peritoneal fluid (114), abscesses (110), wound infections (20), blood cultures (10) and from patients with pneumonia (14) or chronic otitis media (8). Predisposing conditions were present in 145 (53%) children; these were previous surgery (46), trauma (28), malignancy (21), prematurity (19), immunodeficiency (18), steroid therapy (12) foreign body (10), diabetes (9) and sickle cell disease (7). The micro-organisms isolated most commonly mixed with Bacteroides spp. were anaerobic cocci (221), Escherichia coli (122), Fusobacterium spp. (38) and Clostridium spp. (30). All patients received antimicrobial therapy in conjunction with surgical drainage or correction of pathology in 197 (72%) cases. All but 12 (5%) patients recovered. These data illustrate the importance of Bacteroides spp. in infections in children.
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PMID:Bacteroides infections in children. 762 59

New drugs, recently available for treatment of different forms of anaemia, have somehow changed the therapeutic scenario in paediatric haematology. The aim of this review is to focus on the newest molecules discussing indications, clinical usefulness and related problems. Erythropoietin, the specific growth factor of red cell precursors, is now an established option for anaemia of chronic renal failure, prematurity, bone marrow transplantation and chemotherapy. Anti-CD20 monoclonal antibody, a novel cytotoxic molecule for mature B lymphocytes, has proven to be effective in the treatment of refractory autoimmune cytopenias. Haemoglobin analogues are currently under investigation, in order to obtain a synthetic oxygen-carrier that can substitute blood transfusions. Finally drugs that are able to increase the production of haemoglobin F have been used in thalassemias and haemoglobinopathies. For patients with sickle cell disease, hydroxyurea is no longer an experimental tool; it has given rise to several trials, where it has proven to be effective in terms of both clinical and haematological improvement.
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PMID:New drugs for childhood anemia. 1460 71

Dramatic medical and technological advances over the past 15 years have resulted in the survival into adulthood of children with chronic health conditions. As this population subset has increased, the demand of caring for these children in the managed care arena has become challenging from a clinical, fiscal, and member satisfaction perspective. A disease management program was designed for children, ages birth through age 18, identified as having special needs at the time of birth or at any point throughout childhood related to disease processes such as diabetes, sickle cell disease, genetic aberrations, or the multiple complications of extreme prematurity. Components of the program included identification of the population, coordinated risk assessment, and ongoing case management interventions. Most important, outcome indicators were tracked to demonstrate program effectiveness. The formulation and function of a dedicated disease management database is also discussed.
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PMID:Caring for children with special healthcare needs in the managed care environment. 1692 94

The objectives of our study were to describe the epidemiology of child-health indicator diseases in western Jamaica, examine differences in indicator diseases between sex and age, and generate hypotheses about causes of disease burden. International Classification of Disease, 10th Revision, coded discharge diagnoses were collected from consecutive admissions for 2003-2005 from a public tertiary care hospital. Mortality data were not coded. Perinatal disease was the most common cause of mortality, with hyaline membrane disease the primary cause. Younger children, particularly males, are disproportionately affected by all indicator diseases (P < 0.001) and more likely to die from acute respiratory tract infections and infectious diseases (P < 0.05). Sickle cell disease was the fourth most common diagnosis. Children in western Jamaica are most affected by diseases of prematurity. These children experience disease burden similar to that of children in other developing countries, but fewer neonatal sepsis and insect-borne infections, and more hematologic illness.
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PMID:Descriptive epidemiology of mortality and morbidity of health-indicator diseases in hospitalized children from western Jamaica. 1934 83


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