Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Functional asplenia develops in children with sickle cell anemia. This asplenia is related to the increased incidence of bacterial sepsis that has been documented in these patients. With the use of direct-interference contrast microscopy to quantitate splenic function, we studied children with the sickle hemoglobinopathies. A gradual increase in splenic dysfunction with increasing age was documented in children with homozygous sickle cell disease. Children with the sickle variants also seem to manifest degrees of splenic dysfunction. Direct-interference contrast microscopy is a simple quantitative technique for the evaluation of splenic function in children with the sickle hemoglobinopathies.
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PMID:RBC surface pits in the sickle hemoglobinopathies. 43 77

A patient with inactive systemic lupus erythematosus was successfully treated for pneumococcal sepsis complicated by disseminated intravascular coagulation, shock, renal failure, and functional asplenia. Functional asplenia was diagnosed from the total absence of uptake of intravenously administered 99mtechnetium-labeled sulfur colloid. Ten similar cases of functional asplenia occurring in patients with systemic lupus erythematosus were noted in a review of the literature. Six of these cases, including the current report, were complicated by pneumococcal (5) or salmonella (1) sepsis. The patient presented here had an excellent antibody response to pneumococcal vaccination. Spleen scan abnormalities fully reversed at 1 year. Although functional asplenia is a rare event in systemic lupus erythematosus, it appears to predispose to severe septic complications.
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PMID:Functional asplenia in systemic lupus erythematosus. 228 43

Functional asplenia occurs in 94% of patients with homozygous sickle cell anemia by 5 years of age and may result in fatal septicemia due to encapsulated microorganisms such as Streptococcus pneumoniae. Penicillin prophylaxis in these patients significantly reduces the risk of septicemia; however, continuation of prophylaxis beyond 5 years of age is controversial, since the risk of developing septicemia is reduced after this age and prolonged prophylaxis may lead to emergence of penicillin resistance. Although reports of penicillin-resistant pneumococci in patients receiving penicillin prophylaxis are conflicting, the prevalence of these organisms in the general population in North America increased from 5% in 1989 to more than 35% in 1997. Discontinuation of prophylaxis after age 5 years may be recommended because of lack of benefit, difficulty maintaining compliance, reduced risk of developing pneumococcal bacteremia after that age, and increase in prevalence of penicillin-resistant pneumococci worldwide.
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PMID:Duration of penicillin prophylaxis in sickle cell anemia: issues and controversies. 1064 85

Bacterial infections are considered a major cause of morbidity and mortality in patients, particularly children, with sickle cell disease. Infections including pneumonia, meningitis, osteomyelitis, pyelonephritis and general sepsis are more prevalent in patients with these genetic abnormalities than in normal individuals. Generally, infections are more prevalent in children than in older patients. The most common cause of severe infections in hemoglobinopathies include Diplococcus, Staphylococcus, Pneumococcus, Salmonella and Streptococcus. Several investigations have been conducted to determine the possible defects in the host defense mechanisms. Functional asplenia, defects in alternate pathway and in opsonic activity and phagocytosis of Streptococci, Staphylococci and Salmonella in sickle cell anemia patients are considered important factors predisposing these patients to bacteremia. On the other hand, a beneficial association has been demonstrated between the sickle cell gene and malaria. The hemoglobin S (Hb S) provides a natural resistance against the malarial parasite resulting in an improvement in fitness and survival over the normal (Hb AA) individuals. This communication reviews infections in sickle cell disease with a comparison of results in various populations.
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PMID:Infections in sickle cell disease. 2116 37

Asplenia may refer to the spleen's surgical removal, functional impairment, or congenital absence. It is a risk factor for the development of severe bacterial infection. Functional asplenia is likely the most common presentation of this entity and has many etiologies. Those that are previously undiagnosed may present completely well until an episode of overt sepsis develops. The true incidence of mortality secondary to functional asplenia remains elusive. As lifetime mortality remains exceedingly high in the asplenic population regardless of etiology, markers of hyposplenism are important to detect. The present report describes an infant with trisomy 21 and previously undiagnosed functional asplenia who ultimately experienced overwhelming pneumococcal sepsis with features of Waterhouse-Friderichsen syndrome and died within 12 hours of initial presentation. It is a poignant reminder of what features to be cognizant of on peripheral blood smear in a previously well child, who may be at risk for a devastating consequence.
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PMID:A case of functional asplenia and pneumococcal sepsis. 2173 Aug