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)

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

Infections involving bone continue to be a common problem. In children this is usually an acute haematogenous osteomyelitis. Early diagnosis with culture of an aspiration specimen is of paramount importance. Treatment with antibacterial agents is often successful unless pus is obtained in aspiration. In cases with an established abscess, surgical drainage is often needed in addition to antibiotic treatment. Staphylococcus aureus is the most common causative organism, although other microbes are often found in special circumstances such as in neonates, patients with sickle cell disease and those with nail puncture wounds. In adults, a pyogenic osteomyelitis is often due to direct trauma and generally is chronic in nature. Surgical debridement is the mainstay of treatment in these cases. Antibiotic treatment is often helpful but not curative by itself. Fungal and mycobacterial osteomyelitis is especially common in immunocompromised hosts. Amphotericin B remains the preferred treatment for fungal infections. Long term antituberculous multiple drug therapy is often sufficient to treat mycobacterial osteomyelitis.
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PMID:Osteomyelitis. Common causes and treatment recommendations. 768 Sep 83

Patients with sickle cell disease often develop acute chest syndrome (ACS). Signs of ACS include chest pain, fever, prostration, and pulmonary opacities. Pneumonia and infarction have been implicated in the pathogenesis of this syndrome. Infarction as a result of microvascular occlusion and pneumonia are not easily differentiated with chest radiography or ventilation-perfusion scintigraphy. The authors evaluated the ability of thin section (3-mm) chest computed tomography (CT) to help diagnose microvascular occlusion in ACS and thus help differentiate two of its most likely causes. CT scans of the chest of 10 patients with moderate to severe ACS were retrospectively reviewed by two observers, who listed the number of bronchopulmonary segments showing consolidation; areas of ground-glass attenuation due to early hemorrhagic edema; and paucity or absence of small vessels, arterioles, and venules. In all patients, the degree of hypoxia was out of proportion to the extent of consolidation evident at chest radiography. The CT scans showed microvascular occlusion and areas of ground-glass attenuation in nine patients. Infection was ruled out in eight patients. High-resolution CT may play an important role in the initial evaluation and timely selection of an appropriate treatment regimen aimed at improving tissue perfusion, thus forestalling irreversible organ damage and chronic pulmonary arterial hypertension in patients with sickle cell disease.
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PMID:Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion. 845 35

Sickle cell disease sometimes presents with knee arthropathy secondary to osteonecrosis. We report our experience with 9 total knee arthroplasties in 5 patients. The joint fluid, synovium, capsule, and excised bones were sent for culture. Tissue cultures from 5 knees grew organisms. The outcome was: 6 excellent, 1 good, 1 satisfactory, and 1 poor. Infection in 1 case led to removal of the prosthesis and subsequent arthrodesis. 1 patient had persistent peroneal nerve palsy. There were no hematological complications. We suggest that osteonecrosis in sickle cell disease should be considered septic.
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PMID:9 Knee arthroplasties for sickle cell disease. 849 73

In the United States, sickle cell disease primarily affects people of African descent, and the multisystemic complications caused by the resultant vaso-occlusive state create a multitude of diagnostic considerations. In the musculoskeletal system, likelihood is high for avascular necrosis of the femoral humeral head, as a consequence of skeletal infarcts, and also for leg ulceration and osteomyelitis; in the eyes, the incidence of proliferative retinopathy is high; in the urinary tract, dehydration is common, and causes for renal failure are many; in the pulmonary system, pneumonia is of prime concern, as are sickle cell chest syndrome (from occlusion within the microvasculature of the lung) and the deadly sickle cell chronic lung disease, for which pulmonary function tests are important in early asymptomatic stages. Cholelithiasis occurs in 40% of young adult patients with sickle cell disease and can be confused with sickle cell hepatopathy, and rheumatologic and immunologic diseases can occur concomitantly with sickle cell disease, with similar symptoms. The chance for stroke in patients with sickle cell disease is 25%, and early recurrence is common, although the pathogenesis has been more clearly elucidated through computed tomography and magnetic resonance imaging. Infection with Streptococcus pneumoniae has high mortality because of the asplenia associated with sickle cell disease.
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PMID:Sickle cell disease: the clinical manifestations. 855 48

Pneumonia causes about three million deaths a year in young children, nearly all of which are in developing countries. Streptococcus pneumoniae (the pneumococcus) is the most important bacterial cause of pneumonia in young children and so is likely to be responsible for a high proportion of these deaths. The pneumococcus is also responsible for a substantial proportion of the 100,000-500,000 deaths that occur from meningitis in children each year. The incidence of invasive pneumococcal disease in children in the developing world is several times higher than in industrialized countries. This discrepancy may, in part, be due to socio-economic differences but genetic factors may also play a role. Children with sickle cell disease have a substantially increased risk of invasive pneumococcal infection and a search is being made for other possible genetic risk factors. Infection with human immunodeficiency virus (HIV) also predisposes to invasive pneumococcal disease and so the incidence of this disease in young children is expected to rise as increasing numbers of African and Asian children are born with a perinatally acquired HIV infection. Until recently, pneumococcal infections could be treated effectively with penicillin, a cheap and safe antibiotic. However, pneumococci that are resistant to penicillin are becoming prevalent in many countries, necessitating a change to more costly antibiotics which may be beyond the reach of the health services of poor, developing countries. The spread of antibiotic resistance has provided an added stimulus to the development of vaccines that might be able to prevent pneumococcal disease in infants. Recently developed polysaccharide-protein conjugate vaccines show promise and are now undergoing field trials. How deployment of these vaccines will influence the balance between invasive pneumococcal infections and asymptomatic nasopharyngeal carriage of pneumococci is uncertain.
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PMID:The epidemiology of pneumococcal infection in children in the developing world. 1036 3

The pattern of acute illness was determined in 102 adolescents and adults with sickle cell anaemia who presented to the emergency unit of a Lagos hospital. The patients had a mean age of 20.5 years (SD 13.1) and a male-female ratio of 1.5. The symptoms included fever (72%), fatigue and weakness (59%), anorexia (59%) and pain (57.5%) while major clinical signs were pallor (100%), jaundice (71%) and hepatomegaly (68%). Sixty-eight per cent of patients had sickle cell crises, including one with hemiplegic stroke, 10% with combined anaemia and pain crises, 33% with anaemia crises only and 23.5% with pain crises only. Sixty-three per cent had infection which was malaria in 24.5%, bacterial in 17% and viral in 6%. Of 16 patients with pyrexia of unknown origin, seven responded to treatment with chloroquine and eight to antibiotics. Infection was detected in 50% of the patients with sickle cell crises. The association between anaemia crises and malaria was significant (P < 0.05). Of the eight deaths, seven (88%) had anaemia crises. In contrast to studies conducted two decades ago in the same hospital, the prevalence of anaemia crises now exceeds that of pain crises and malaria now exceeds that of bacterial infection. Severe symptomatic anaemia (anaemia crisis) was more frequently associated with infection (mostly malaria) than was bone pain crisis. The Girdle pain crisis more frequently resulted in a fatal outcome than the uncomplicated bone pain crisis.
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PMID:Acute sickle cell syndromes in Nigerian adults. 1093 Nov 63

Sickle cell anaemia (SCA) predisposes a child to infections for various reasons, including increased bone marrow turnover, poor perfusion and functional asplenia leading to decreased opsonisation of polysaccharide encapsulated organisms. Bacteria and viruses that most frequently cause serious infections in children with sickle cell disease are Streptococcus pneumoniae, Haemophilus influenzae type b, Salmonella spp., Escherichia coli, Staphylococcus aureus, Mycoplasma pneumoniae, Chlamydia pneumoniae, parvovirus B19 and hepatitis A, B and C viruses. Penicillin prophylaxis has decreased the incidence of infection-related morbidity and mortality significantly in children with SCA. Children <3 years of age are administered oral penicillin 125mg twice daily, and the dose is increased to 250mg twice daily for the >3 to 5 year age group. Adherence to the penicillin prophylactic regimen is recommended for children with SCA who are >5 years of age. For children with SCA who have recurrent invasive pneumococcal infections, an effort is made to keep the child on penicillin prophylaxis indefinitely. The administration of various childhood vaccines has also made an appreciable impact on the overall morbidity and mortality associated with infection in children with SCA. The administration of the heptavalent conjugate pneumococcal vaccine (PCV7) has provided control of invasive pneumococcal infections, and the prophylactic use of the H. influenzae type b conjugate vaccine has reduced the incidence of septicaemia and meningitis caused by this organism. Other vaccines used prophylactically in children with SCA include hepatitis A and B, and vaccines against influenza and varicella viruses. The immediate administration of intravenous antibacterials, after appropriate blood and urine cultures, is of great importance in the treatment of the febrile child with SCA. Ceftriaxone and cefotaxime have been recommended for the treatment of septic episodes in SCA associated with S. pneumoniae, Haemophilus and Salmonella spp. Infection with Yersinia enterocolitica may be treated with cefotaxime or an aminoglycoside. The prevalence of Helicobacter pylori infection in SCA is unknown. Effective therapies include metronidazole, tetracycline or amoxicillin. Parvovirus infections require supportive care and specific antiviral therapy is not indicated. The judicious use of antimicrobials is encouraged in view of the worldwide emergence of multidrug-resistant strains. The long term sequelae associated with infections in children with SCA can be decreased with the implementation of immunisation programmes and effective and prompt treatment with appropriate antibacterials.
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PMID:Prevention and management of infection in children with sickle cell anaemia. 1173 65

To achieve stable gene transfer into human hematopoietic cells, we constructed a new vector, DeltaAd5/35.AAV. This vector has a chimeric capsid containing adenovirus type 35 fibers, which conferred efficient infection of human hematopoietic cells. The DeltaAd5/35.AAV vector genome is deleted for all viral genes, allowing for infection without virus-associated toxicity. To generate high-capacity DeltaAd5/35.AAV vectors, we employed a new technique based on recombination between two first-generation adenovirus vectors. The resultant vector genome contained an 11.6-kb expression cassette including the human gamma-globin gene and the HS2 and HS3 elements of the beta-globin locus control region. The expression cassette was flanked by adeno-associated virus (AAV) inverted terminal repeats (ITRs). Infection with DeltaAd5/35.AAV allowed for stable transgene expression in a hematopoietic cell line after integration into the host genome through the AAV ITR(s). This new vector exhibits advantages over existing integrating vectors, including an increased insert capacity and tropism for hematopoietic cells. It has the potential for stable ex vivo transduction of hematopoietic stem cells in order to treat sickle cell disease.
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PMID:A high-capacity, capsid-modified hybrid adenovirus/adeno-associated virus vector for stable transduction of human hematopoietic cells. 1177 89

The purpose of this study was to determine the age-related risks, disease-specific risks, and characteristics of serious pneumococcal infections in children with sickle cell disease (SCD) while penicillin prophylaxis was standard. The clinical experiences of three pediatric sickle cell programs spanning January 1, 1992, to May 31, 1998, were combined. Data were collected regarding the patients followed up and the characteristics of bacteremia and meningitis cases. Forty-seven pneumococcal infections (44 bacteremia, 3 meningitis) among 40 patients with SCD were observed. Forty infections occurred in children with homozygous hemoglobin S (SS) during 4108 patient-years at a median age of 22 months; 7 occurred in double heterozygous hemoglobin SC (SC) children during 1777 patient-years at a median age of 23 months. Ten infections occurred among 9 SS children 5 years or older. Most children in whom infections developed were reportedly taking prophylactic penicillin and when older than 24 months old had received Pneumovax (Merck & Co., Inc., West Point, PA, U.S.A. The following pneumococcal serotypes were identified in 15 cases studied: 6A, 6B, 9V, 14, 15B, 18B, 18F, 19F, and 23F. Infections resulted in five deaths and two strokes. The observed severe pneumococcal infection rate in SS children younger than 5 years was less than that reported before penicillin prophylaxis, supporting routine penicillin prophylaxis in this specific population. The optimal duration of penicillin prophylaxis in older children with SCD remains unknown. The administration of 7-valent Prevnar (Wyeth Lederle Vaccines, Philadelphia, PA, U.S.A.) to children younger than 24 months old with SCD should be beneficial, based on the serotype data.
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PMID:Streptococcus pneumoniae sepsis and meningitis during the penicillin prophylaxis era in children with sickle cell disease. 1221 95


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