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

Salmonella infections remain a significant cause of morbidity and mortality in patients with sickle cell disease. In Jamaica, 16 episodes of systemic Salmonella infection were recognized in 308 children with sickle cell disease followed prospectively in a cohort study from birth. There were eight cases with osteomyelitis, seven with septicaemia, and one with meningitis. Salmonella dactylitis was the commonest presentation of osteomyelitis in the young child. Comparison of clinical features between children with Salmonella dactylitis and dactylitis owing to avascular bone necrosis showed that children with osteomyelitis had significantly higher fever, prolonged history and fluctuant swellings. Fever above 38.5 degrees C occurred in four of five children with Salmonella dactylitis, but in only one of 59 with uncomplicated dactylitis (P = 0.01). Chloramphenicol and co-trimoxazole were both effective for systemic infection, and ampicillin alone was inadequate. Three deaths occurred, two from septicaemia and one from meningitis.
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PMID:Systemic Salmonella infections in sickle cell anaemia. 247 63

The pattern of associated Central Nervous System (CNS) disorders in children with homozygous sickle cell anaemia was studied over a 12-month period at the University of Nigeria Teaching Hospital (UNTH), Enugu. The relative incidence of associated neurological disorders was 1.3%, and the major varieties were cerebrovascular accidents (57.5%), meningitis (22.5%) and recurrent convulsions (10%). Intellectual impairment did not feature as a separate entity, but was observed in 27.5% of children with CNS complications. Although recurrent strokes were not frequently encountered, the hypertransfusion therapy was not practised because of relative scarcity of blood donors.
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PMID:Neurological disorders in Nigerian children with homozygous sickle cell anaemia. 261 4

About 120,000 infants are born each year with sickle cell disease (SCD) in Africa. The majority have Hb SS, but Hb SC and Hb S/beta+ thalassaemia are common in west Africa. The development of Plasmodium falciparum and P. malariae is partially inhibited in the Hb SS red cells, but malaria precipitates both haemolytic and infarctive crises, and is the commonest and most important cause of morbidity and mortality. The pneumococcus is likely to be the second major infectious cause of sickness and death. In one rural community, there were less than 2% of the expected number of subjects with SCD surviving beyond 5 years of age. Genetic factors improving prognosis include (1) the Senegal beta chain haplotype, which is linked to a high level of Hb F, and (2) alpha+ thalassaemia. Of environmental factors improving prognosis, the family is of first importance. The commonest age of presentation is 1-3 years. Children present with anaemic crises (malaria, splenic sequestration, folate deficiency, and possibly aplastic), infarctive crises (hand-foot syndrome, bone-pain, pulmonary and abdominal) or acute infections (malaria, pneumonia, septicaemia, meningitis, osteomyelitis). Tragically, many patients in central Africa have been infected by the human immunodeficiency virus (HIV) through blood transfusions; they present with generalised lymphadenopathy and other features of the acquired immunodeficiency syndrome (AIDS). The principles of management are (1) to ensure freedom from malaria, (2) to continue folic acid supplements, (3) to give blood transfusions only when anaemia endangers life, (4) to control pain, (5) to restore hydration, and (6) to prescribe broad spectrum antibiotics in large dosage and without delay, but only when there are definite indications, such as fever (greater than 39 degrees C), acute pulmonary disease, meningitis, and acute osteomyelitis. The advent of HIV and AIDS makes the control of SCD of even greater importance. Principles of control are (1) early diagnosis through appropriate laboratory techniques and selective screening, (2) education of parents, patients, health professionals and public, and (3) the maintenance of health at sickle cell clinics; measures must include antimalarial prophylaxis. SCD programmes should be integrated with primary health care and AIDS control programmes.
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PMID:The presentation, management and prevention of crisis in sickle cell disease in Africa. 265 Jul 73

There is a high incidence of bacterial infections in sickle cell disease, particularly in sickle cell anemia. Pneumonia, urinary tract infections, osteomyelitis, meningitis and pneumococcal septicemia occur mainly in younger patient. The pathological basis for this susceptibility to infections is complex. Defective splenic function is the most important factor. There are also abnormalities of opsonization, alternate complement pathway, antibody production, leucocyte function, and cell-mediated immunity. Pneumococcal immunization and prophylactic penicillin are indicated in the prevention of pneumococcal infections.
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PMID:[Infection and immunity in sickle cell disease]. 269 86

Infectious sinusitis may on occasion be associated with meningitis, subdural empyema, epidural empyema, brain abscess, or osteomyelitis. We report a 29-year-old male patient with frontal sinusitis who developed all of these intracranial complications due to two previously unreported causative organisms, Neisseria sicca and Eubacterium lentum. The fulminant and fatal course resulting from locally invasive disease underscores the importance of early diagnosis and proper treatment of these complications. Possible exacerbating factors in this patient were sickle cell disease and immune compromise due to intravenous drug abuse.
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PMID:Fatal frontal sinusitis due to Neisseria sicca and Eubacterium lentum. 287 55

The rate and pattern of infections in 144 Saudi Arabian children with sickle cell disease (SCD) and matched normal control subjects are reported. All diagnoses of SCD were made at birth by means of screening blood from the umbilical cord. The children were prospectively followed up from birth to 4 years of age. Severe bacterial infections occurred in none of the children with SCD; one of the control children developed pneumococcal meningitis. Acute gastroenteritis was significantly more common among patients with SCD. For the first year of life, patients with SCD had significantly more infections than did the control children; but the reverse was true in the group that was 37 to 48 months of age. Considering all types of infections for all age groups, no difference was noted between patients with SCD and control subjects in terms of infection rate or related hospital admission. There were no deaths caused by infection in this series. We conclude that Saudi Arabian infants and young children of oasis origin with SCD are not at increased risk of infections compared with healthy children of the same age.
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PMID:Infections and sickle cell disease in Eastern Saudi Arabian children. 291 93

All acute admissions of patients with sickle cell disease who lived in the London borough of Brent and attended this hospital were analysed for a period of one year. Sixty three of the 211 patients who were followed up by the haematology department required 161 acute admissions during the year. Most admissions (126) were for the 42 patients with homozygous sickle cell disease; 147 (91%) were for vaso-occlusive episodes, 142 of which were for painful crises, three for cerebrovascular accidents, and two for renal papillary necrosis. Preschool children with sickle cell disease were admitted predominantly with limb pain, whereas in schoolchildren and adults the incidence of trunk pain was higher. Twenty four of the 93 episodes of trunk pain culminated in an episode of severe visceral sequestration usually affecting the lungs, the liver, or the mesenteric circulation. Two patients died: an 18 month old baby with an acute splenic sequestration crisis and a 19 year old man with a severe girdle syndrome (sickling in the mesenteric circulation, liver, and lungs). Infective episodes were rare (11 episodes) but severe: one haemophilus meningitis, two salmonella infections, and three aplastic crises due to parvovirus infections. The average duration of the hospital stay was 7.4 days per admission. It is concluded that because sickle cell disease causes appreciable morbidity in older children, adolescents, and adults a systematic approach to management is needed to deal with acute episodes such as sequestration syndromes.
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PMID:Acute admissions of patients with sickle cell disease who live in Britain. 310 83

Third-generation cephalosporins have been considered for the treatment of systemic salmonelloses because of emerging resistance among Salmonella species to ampicillin, chloramphenicol, and trimethoprim-sulfamethoxazole. Twelve patients with typhoid/paratyphoid fever, nine with nontyphoid salmonella bacteremia, and two with Salmonella meningitis were treated with cefotaxime; one leukemic patient with Salmonella dublin bacteremia received ceftizoxime. All infections were cured except for one in a patient with sickle cell anemia; this patient's illness recurred but was cured with a second course of cefotaxime followed by ceftriaxone. A review of the literature documented cures with cefotaxime in 50 of 61 patients with typhoid/paratyphoid fever, all of four with salmonella osteomyelitis, 12 of 14 with salmonella meningitis, and 44 of 49 with non-typhoid salmonella bacteremia. Ceftriaxone and cefoperazone cured, respectively, 23 of 25 and 32 of 33 patients with typhoid/paratyphoid fever. The relapse rates of typhoid fever treated with cefotaxime, ceftriaxone, and cefoperazone were 6%, 4%, and 0%, respectively. Cefotaxime, ceftriaxone, and cefoperazone are acceptable alternative antibiotics for the treatment of salmonelloses caused by multiresistant organisms.
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PMID:Treatment of typhoid fever and other systemic salmonelloses with cefotaxime, ceftriaxone, cefoperazone, and other newer cephalosporins. 312 77

The criteria of the cerebrovascular Moyamoya disease is defined by the characteristic findings of its cerebral angiograms, as follows; 1) The internal carotid siphon is narrowed or obstructed bilaterally. 2) The "Moyamoya vessels" are observed at the base of the brain or the basal ganglionic regions. 3) Main trunks of the cerebral arteries such as the anterior, the middle, and/or the posterior cerebral arteries are often not or poorly visualized. 4) Its etiology is unknown. It has been known that the occlusion of the internal carotid fork with Moyamoya vessels is not infrequently seen in patients with tuberculous meningitis, sickle cell anemia, head trauma, and so on. In the definition of the disease, patients with known etiology and/or unilateral occlusion in the carotid fork must be excluded. However, the cases who cannot fulfil its criteria of the cerebrovascular Moyamoya disease, but have its characteristic Moyamoya vessels and collateral pathways have been reported. We investigated the findings of cerebral computed tomograms in 13 patients who did not fulfil the criteria of the cerebrovascular Moyamoya disease, but revealed the Moyamoya vessels. The subjects are 5 males and 8 females, ranging 15 to 70 years old. The past histories of 9 patients among them revealed hypertension, radiation therapy for pituitary adenoma, head trauma, aplastic anemia, and the Raynaud phenomenon. By angiographic evaluations, occlusions in the unilateral carotid forks were seen in 7 patients, and stenoses in those were in 5 patients. One patient showed only a severe stenosis in the horizontal portion of the middle cerebral artery.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cases similar to cerebrovascular moyamoya disease--investigation by angiography and computed tomography]. 319 93

We examined a young patient with sickle cell anemia and a history of spinal meningitis who presented with signs of Weber's syndrome. Weber's syndrome is manifested by a homolateral third nerve palsy and contralateral hemiplegia. Evaluation and management of this case is presented, with a discussion on the probable association of Weber's syndrome and the spinal meningitis that occurred.
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PMID:Weber's syndrome. 323 Feb 43


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