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

Acute Chest Syndrome (ACS) continues to be a major source of morbidity and mortality among patients with sickle cell disease. It is characterized by the presence of pleuritic chest pain, fever, raises on lung auscultation, and pulmonary infiltrates on chest x-ray [Castro et all: Blood 84:643-649]. The pathophysiology of this disorder remains poorly understood leading to the descriptive term "Acute Chest Syndrome" designated by Charache et al. [Arch Intern Med 139:67-69, 1979]. Typical bacterial pathogens are seldom isolated in adults, although they play a significant role in the pathogenesis of this entity in children. Until recently, the technology to accurately study viral infection as a precipitating cause of ACS has been unavailable. Parvovirus B19 is being increasingly recognized as an important human pathogen, and has been established as the cause of transient "aplastic crisis" in patients with sickle cell diseases [Saarien et al: Blood 67:-11411-11417, 1986; Young: Sem Hematol 25:159-172, 1988]. We present three patients with hemoglobin SC variant of sickle cell disease who developed ACS in association with acute parvovirus B19 infection, one of which died of respiratory failure. Parvovirus B19 infection was established by polymerase chain reaction for parvovirus B19 DNA, and the presence of parvovirus B19 specific IgM antibodies. These cases suggest that parvovirus B19 may be associated with more than self-limited illness in patients with sickle cell disease, and that this ubiquitous virus may merit further study as a precipitating cause of ACS.
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PMID:Sickle cell acute chest syndrome associated with parvovirus B19 infection: case series and review. 861 1

Acute chest syndrome is a well described complication of sickle cell anemia. It is characterized by fever, pulmonary infiltrates, pleuritic chest pain and abnormal pulmonary auscultation. Transfusion therapy, either simple transfusion of red blood cells or a total red blood cell exchange, is a cornerstone therapy for these patients. Exchange transfusion is preferred when an acute reduction of the hemoglobin S (HbS) concentration is the therapeutic goal since it allows one to rapidly reduce the percent HbS without increasing blood viscosity or volume (Wayne, Kevy and Nathan, Blood 1993; 81:1109-1123). Hemoglobin electrophoresis may be used to monitor the effectiveness of the exchange in decreasing HbS. The post-exchange HbS electrophoresis results which were obtained in this case initially caused confusion. In this report we discuss the findings and the reasons why such results may be occasionally expected in future similar situations.
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PMID:Unexpected hemoglobin electrophoresis results following red cell exchange in a sickle cell anemia patient with acute chest syndrome. 1017 84

Splenic abscess is not an uncommon complication of patients with sickle-cell disease. Here we describe an 18 year-old boy with sickle cell disease and left upper quadrant abdominal pain. Computerized axial tomography revealed left sided free flowing pleural effusion and splenomegaly with liquefaction and possible gas formation. The splenic fluid grew an unusual organism known as Bacteroides distasonis. The patient received antimicrobial therapy and underwent a splenectomy with full recovery. The spleen was cystically infarcted and measured 22 x 16 x 5 cm. The capsule was thickened and covered by fibrinous exudate. Histopathologic examination of the spleen showed complete necrosis with reparative fibrosis. This case presents an unusual cause of splenic abscess due to Bacteroides distasonis with a subacute to chronic course. The presence of fever and left sided pleuritic chest pain in patients with sickle cell disease should raise the suspicion of splenic abscess.
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PMID:Bacteroides (Parabacteroides) distasonis splenic abscess in a sickle cell patient. 1817 10