Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eighteen of 384 patients entered on the Brent sickle cell disease register died between 1974 and 1989, a mortality of one per 128 years of follow up. Two children died from acute splenic sequestration and a third died from fulminant pneumococcal septicaemia: none was taking prophylactic penicillin. Acute chest syndrome was the cause of death in eight young adults and one child. Three deaths occurred after surgery. Cerebrovascular accidents contributed to the cause of death in three cases and there were two sudden unexplained deaths. Ten of the deaths occurred at home or within 24 hours of admission to hospital. Post mortem examinations were made in 14 cases, but the histological appearances of acute chest syndrome were often not recognised. In most cases for whom information was available, the cause of death (chest syndrome, pneumococcal sepsis, postoperative complications) could have been prevented.
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PMID:Patterns of mortality in sickle cell disease in the United Kingdom. 206 23

60 Jamaican children with homozygous sickle cell (SS) disease underwent splenectomy, 14 for prophylaxis against recurrent acute splenic sequestration and 46 for treatment of sustained hypersplenism. Age at operation varied from 9 months to 16 years. Patients were followed up for 1 month to 27 years (median 6 years), with a total of 369 years of patient-observation. None of the 3 patients who died, at ages 2 1/2, 6 1/2, and 21 years, had received prophylaxis against infection. Overwhelming sepsis was possible but not confirmed in the first two deaths which occurred 11 months and 2 1/2 years after operation; the third died from chronic renal failure 11 years after splenectomy. After operation, there were no confirmed cases of pneumococcal septicaemia or meningitis, and the commonest clinical event was the acute chest syndrome.
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PMID:Role of splenectomy in homozygous sickle cell disease in childhood. 614 Apr 33

The acute chest syndrome is a clinical entity appearing in patients suffering from sickle cell anaemia. It presents with pleuritic pain, fever, leucocytosis and pulmonary infiltrates in the thoracic radiology. The etiological diagnosis is difficult, and it is necessary to distinguish between pneumonia and pulmonary infarction. This syndrome is quite frequent among the patients at risk, and can be lethal according to the severity and the etiology of the event. A case of acute chest syndrome due to a S. pneumoniae sepsis is presented. The interest of the case lies in the rareness of this disease in our population and the peculiar evolutive clinical features of this case, with the development of intracranial hypertension and death.
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PMID:[Acute thoracic syndrome]. 798 60

Fat embolism of necrotic bone marrow could be a frequent cause of acute chest syndrome (ACS) in sickle cell syndromes (SC), as suggested by postmortem findings. To check this hypothesis in living patients, we evaluated the presence of fatty macrophages recovered by bronchoalveolar lavage (BAL) in ACS. We investigated 20 consecutive cases of ACS by BAL, and identification of alveolar cells containing fat droplets was performed using oil red O (ORO), a specific neutral fat stain. The specificity of the method was determined on control groups, including eight SC patients without acute chest syndrome and 15 non-SC patients. A cut-off of > 5% of alveolar macrophages containing fat droplets was determined from the control groups to assess the diagnosis of fat embolism. In 12 ACS episodes, BAL exhibited > 5% of fatty macrophages, ranging from 10% to 100% (median value 46.5%). In 11 cases, fat embolism was associated with proven (n = 8) or probable (n = 3) bone marrow infraction, which mostly predated ACS. Eight ACS episodes were associated with a low percentage (< or = 5%) of fatty alveolar macrophages and could be related to a cause other than fat embolism in six episodes, such as sepsis, in-situ thrombosis, or rib infarcts generating hypoventilation. This study supports the diagnostic yield of BAL for fat embolism, which can be incriminated in 60% of cases of ACS in this adult population.
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PMID:Bronchoalveolar lavage in adult sickle cell patients with acute chest syndrome: value for diagnostic assessment of fat embolism. 863 Jun 22

Hemoglobin S/O(Arab) (Hb S/O(Arab)) is a rare compound heterozygous hemoglobinopathy characterized by the presence of two variant beta-globin chains: beta6Glu --> Val (Hb S) and beta121Glu --> Lys (Hb O(Arab)). The diagnosis of Hb S/O(Arab) requires electrophoresis on both cellulose acetate and citrate agar, since Hb O(Arab) co-migrates with Hb C at alkaline pH and close to Hb S at acidic pH. To date only case reports and small series of patients with Hb S/O(Arab) have been described. To better characterize the clinical and laboratory aspects of this unusual disorder, we reviewed the Duke University Medical Center experience. We identified 13 African-American children and adults with Hb S/O(Arab) ranging in age from 2.7 to 62.5 years. All patients had hemolytic anemia with a median Hb of 8.7 gm/dL (range 6.1-9.9 gm/dL), and a median reticulocyte count of 5.8% (range 1.2-10.3%). The peripheral blood smear typically showed sickled erythrocytes, target cells, polychromasia, and nucleated red blood cells. All 13 patients have had significant clinical sickling events including acute chest syndrome (11), recurrent vasoocclusive painful events (10), dactylitis (7), gallstones (5), nephropathy (4), aplastic crises (2), avascular necrosis (2), leg ulcers (2), cerebrovascular accident (CVA) (1), osteomyelitis (1), and retinopathy (1). Four patients have died, including two from pneumococcal sepsis/meningitis at ages 5 and 10 years, one of acute chest syndrome at age 14 years, and one of multiorgan failure at age 35 years. We conclude that Hb S/O(Arab) disease is a severe sickling hemoglobinopathy with laboratory and clinical manifestations similar to those of homozygous sickle cell anemia.
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PMID:Hemoglobin S/O(Arab): thirteen new cases and review of the literature. 1020 1

Over the past 25 years, morbidity and mortality have decreased significantly in children with sickle cell disease, and screening tests are now available to diagnose the disease in newborns. The incidence of sepsis caused by pneumococcal and Haemophilus influenzae infections has declined because of the prophylactic administration of penicillin soon after birth and the timely administration of pneumococcal and H. influenzae type b vaccines. Optimal nutrition can maximize growth in children with sickle cell disease, and timely screening can identify complications such as retinal damage and chronic renal involvement, thereby ensuring prompt treatment. Family physicians and parents who have been educated about sickle cell disease can detect acute, life-threatening complications such as splenic sequestration crisis and acute chest syndrome at their onset, thereby allowing treatment to be instituted without delay.
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PMID:Sickle cell disease in childhood: Part I. Laboratory diagnosis, pathophysiology and health maintenance. 1099 28

During recent years, the high phospholipase A(2) (PLA(2)) concentrations at sites of inflammation and in circulation in several life-threatening diseases, such as sepsis, multi-organ dysfunction and acute respiratory distress syndrome, has generally been ascribed to the non-pancreatic group IIA PLA(2). Recently the family of secreted low molecular mass PLA(2) enzymes has rapidly expanded. In some cases, a newly described enzyme appeared to be cross-reactive with antibodies against the group IIA enzyme. For this reason, reports describing the expression of group IIA PLA(2) during inflammatory conditions need to be reevaluated. Here we describe the identification of the PLA(2) activity in sera of acute chest syndrome patients and in sera of trauma victims. In both cases, the PLA(2) activity was identified as group IIA. This classification was based upon cross-reactivity with monoclonal antibodies against group IIA PLA(2) which do not recognize the recombinant human group V enzyme. Moreover, purification of the enzymatic activity from the two sera followed by N-terminal amino acid sequence analyses revealed only the presence of group IIA enzyme.
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PMID:Sera of patients suffering from inflammatory diseases contain group IIA but not group V phospholipase A(2). 1104 Apr 50

Painful episodes are the most frequent complaints of patients with sickle cell disease. The Emergency Department (ED) has provided management for acute events using the usual triage format for emergencies. A prospective study evaluated the role of the ED in the care of adults with sickle cell disease (SCD). The protocol, thus, addressed issues of acute events related to SCD and provided better care for patients with SCD in the ED. Approximately 37% of ED visits were for painful events. An inciting cause was identified in 35% of painful events and 75% of these required admission to the hospital. A 15-year follow-up prospectively showed similar results and that uncomplicated pain crisis can be treated with ED protocols. Outpatient clinics and urgent centers could reduce these visits. Absolute indications for admission include sepsis, fever >102 degreeF, white cell counts >20 000, worsening anemia, hypoxemia, acute chest syndrome and new CNS events. Patient database in the ED must be revised annually to avoid extensive workup in the ED and a complete history/physical examination, and a CBC could be sufficient for triage in an uncomplicated pain crisis. An acceptable protocol for care should be available at all EDs and a registry and information system for SCD will discourage overutilization of investigational tests and visits to multiple EDs.
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PMID:Evaluation and Management of Sickle Cell Disease in the Emergency Department (An 18-year Experience): 1974--1992. 1183 5

Sickle cell disease (SCD) is a term used to describe a group of genetic disorders of hemoglobin production characterized by a predominance of the abnormal hemoglobin known as hemoglobin S. Common acute complications of SCD in children requiring hospitalization include painful episodes, febrile illness, and splenic sequestration. The staff nurse has an important role in providing prompt treatment and instituting preventative measures to avoid the adverse clinical outcomes of SCD such as acute chest syndrome, severe anemia, cardiovascular instability, and bacterial sepsis. A basic understanding of the pathophysiology of vaso-occlusion, the immune system, hemolysis, and the spleen is essential in the care of a child during an acute complication of SCD. Additionally important are a knowledge of the genetics, pathophysiology, medical and nursing management, and a familiarity with patient and family education material relating to sickle cell disease.
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PMID:Care of the child with sickle cell disease: acute complications. 1202 71

Acute chest syndrome (ACS) is the most common cause of death in patients with sickle cell anemia. Its management is primarily palliative. We performed a Phase I evaluation of purified poloxamer 188 (a non-ionic surfactant) in the management of ACS. Forty-three patients with sickle cell disease and ACS were treated with doses as high as 2960 mg/day by continuous intravenous (IV) infusion. The maximum tolerated dose has not been identified. No evidence of renal toxicity or other limiting adverse events were found. One adult patient died due to sepsis and adult respiratory distress syndrome, which were unrelated to treatment. Poloxamer 188 is safe to administer to patients with ACS, and preliminary data suggest that it may shorten its duration and the length of hospitalization in a dose related manner. Children appeared to benefit more than adults. The data and safety profile justify further studies with purified poloxamer 188 in the treatment of ACS.
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PMID:Safety of purified poloxamer 188 in sickle cell disease: phase I study of a non-ionic surfactant in the management of acute chest syndrome. 1518 51


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