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

Systemic salmonellosis is a recognized complication of sickle cell anemia (SCA). In our initial study of SCA host defences against salmonella, we evaluated the bactericidal activity of serum against Salmonella typhimurium. When compared to controls, sera from eight out of nineteen SCA patients were deficient in bactericidal function. Levels of factor B, haemolytic complement and agglutinating antibody were similar in SCA and control sera. However, abnormalities that might theoretically account for the decreased antibacterial activity were observed in many SCA sera. These abnormal findings included: (a) defective function of the alternative complement pathway (decreased bacterial killing in the presence of Mg EGTA); (b) low serum C3 concentration; and (c) decreased total iron-binding capacity (TIBC), with a resultant increase in per cent saturation of iron-binding capacity. Of these deficiencies only the abnormal alternative pathway function was significantly associated with decreased serum bactericidal activity. A suggested function of serum bactericidal activity is prevention of bacteraemia by susceptible organisms. Thus diminished serum bactericidal capacity may increase the risk of Salmonella bacteraemia in some individuals with sickle cell disease.
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PMID:Deficiency of serum bactericidal activity against Salmonella typhimurium in sickle cell anaemia. 34 54

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

Five patients are described with disseminated histoplasmosis and systemic salmonellosis. Four of these patients were also immunocompromised because of the acquired immunodeficiency syndrome in two patients and renal transplantation in another two patients. Histologic studies in two patients showed histiocytes that were heavily laden with Histoplasma capsulatum yeast-phase organisms. We postulate that diffuse parasitization of the reticuloendothelial system (RES) by Histoplasma organisms may cause "RES blockade," which then predisposes to systemic salmonellosis, as reported in certain other infections and in sickle cell anemia.
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PMID:Systemic salmonellosis in patients with disseminated histoplasmosis. Case for 'macrophage blockade' caused by Histoplasma capsulatum. 354 24

Case report of a 28-year old black sickle cell anemia patient with salmonella osteomyelitis of the radius. Aside from sickle cell anemia patients this skeletal complication of enteric salmonellosis is an extreme rarity. Description of the typical roentgenological features including intracortical fissures and sequestration.
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PMID:[Salmonella osteomyelitis in sickle cell anemia]. 408 Oct 14

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 salmonella group of bacteria consists of at least 1700 subtypes and causes widespread gastroenteric disease throughout the world, there being approximately two million cases yearly in the United States alone. An uncommon but interesting complication is the hematogenous lodgment of organisms in other sites of the body with resultant localized abscess formation, often months or years later. We present a review of 15 cases seen in our affiliated hospitals over a nine-year period. Remote salmonellosis most often occurs in patients with underlying systemic disease, such as lupus, malignancy, diabetes, and sickle cell disease, or in association with therapy that suppresses host resistance including steroids and cancer chemotherapy. The most frequent sites of involvement are bone marrow, joints, meninges, pleura, and blood vessels, or in an area of locus minoris resistentiae. The latter include scars, hematomas, fracture sites, prosthetic devices, and neoplasms. Our experience would suggest that these infections, which can remote both in location and time from the original gastrointestinal episode, are perhaps more frequent today than is generally realized. Diagnosis is often delayed because of lack of familiarity with this entity. Treatment consist of either drainage or excision of infected tissue often combined with long-term antibiotics.
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PMID:Remote salmonellosis: surgical masquerader. 707 24

A 22-year-old man had Salmonella panama osteomyelitis of the left distal tibia. He had endured a period of untreated diarrhea without fever 6 years before. The osteomyelitis was treated successfully with surgical debridement followed by 9 weeks of oral cotrimoxazole 960 mg twice daily. Salmonella osteomyelitis is rare. Most cases occur in patients with sickle cell anemia. Other conditions of local or generalized immunosuppression are also risk factors, but none were established in this patient, nor was he a chronic carrier. In reviewing the literature, no case of Salmonella panama osteomyelitis in an otherwise healthy patient was found. Although the osteomyelitis in this patient was possibly secondary to Salmonella enteritis 6 years before, the authors believe that enteric Salmonella infections should not be treated with antibiotics unless the infection is accompanied by systemic symptoms. Otherwise, the risk of chronic carriership is substantially increased. In case of Salmonella panama osteomyelitis, surgical debridement is recommended as the main component of treatment, followed by a prolonged period of specific antibiotic therapy.
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PMID:Salmonella panama osteomyelitis in an otherwise healthy patient. A case report. 749 74

Population statistics of Amsterdam between the 17th and 19th centuries indicate that 20-30% of young married people had been born in foreign lands. At the present time, 6% of the country's population, nearly 1 million people, are direct descendants of foreign parents: 240,000 Surinamese, 210,000 Turks, 170,000 Moroccans, and 80,000 from the Antilles. 40% of foreigners live in the four large cities, and there they make up about 15% of the population; 30-50% of children in these cities have foreign born parents. Among health concerns affecting these people are parasitic diseases, tuberculosis, salmonellosis, and the importation of infections such as viral B hepatitis, which so far has been successfully controlled. About 4% of the foreigners (30,000 people) carry a defective gene, and when two such people marry, in 25% of cases a child can be born with a severe defect as well as thalassemia major (mainly children of Moroccans and Turks) and sickle cell anemia (Surinamese and Antillans). 20-40% of children from tropical or subtropical areas also have lactase enzyme deficiency, which gives them stomach complaints because of incomplete metabolism of milk sugar. In recent years it has been reported that asthma and respiratory infections with longer hospitalizations occur more frequently among foreign children. Infant mortality is also 2-3 times higher among them. Intercultural aspects affecting Turkish and Moroccans immigrants include communication problems, primarily those of the first generation, which should be facilitated by language centers and educational materials. Generation conflicts arise from contrasts between homelife and the outside world as well as from the fact that many of the parents are illiterate. Cultural difference are rooted in Islam, which requires loyalty to the group with traditional role patterns. Other problems pertain to the social isolation of the mother and the lower position of women, and the uncertain legal position of foreigners, which can result in sometimes unwarranted feelings of discrimination.
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PMID:[International and intercultural aspects of pediatrics and adolescent health care]. 812 29

Although osteomyelitis due to Salmonella infection is known to be associated with sickle cell anemia, various hemoglobinopathies and immune suppressive states, it may also occur in normal hosts. A 16-year-old Chinese boy without sickle cell disease or any other condition that would compromise the immune system had osteomyelitis of the lumbar spine caused by Salmonella enteritidis. The condition was treated conservatively with ciprofloxacin (quinolone group). This may be the first reported case in which a patient with spinal osteomyelitis due to Salmonella infection, who was otherwise healthy, was successfully treated nonoperatively with quinolone.
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PMID:Osteomyelitis of the spine due to Salmonella infection--conservative treatment with quinolone: a case report. 903 84

Salmonella infection in sickle cell disease patients is generally well-known but presentation as superficial abscess is relatively uncommon. Two sisters aged 4 1/2 and 6 years presented with superficial subcutaneous abscesses that were caused by the same strain of Salmonella enteriditis group C. Despite in vitro susceptibility with a MIC of 0.03 microgram/ml and an adequate dosage of ciprofloxacin there was a relapse with widespread dissemination of the same organism in the younger sister who subsequently developed multiple osteolytic infections. Change of treatment to chloramphenicol produced a cure in both patients.
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PMID:Superficial Salmonella abscesses in two siblings with sickle cell diseases. 932 91


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