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)

Hydroxyurea (HU) is one of several agents that have been shown to enhance hemoglobin (Hb) F levels in patients with sickle cell disease and may be useful as a therapy for beta-globinopathies. However, limited information exists on the effects of HU in patients with thalassemia. Accordingly, we examined the hematologic effects of orally administered HU in 13 patients with beta-thalassemia/Hb E, including four patients who had been splenectomized. These patients were treated with escalating doses (final range, 10 to 20 mg/kg/d) for 5 months and were observed in the outpatient hematology clinic every 2 to 4 weeks. Complete blood counts including reticulocyte counts, amounts of Hb E and Hb F, G gamma:A gamma and alpha:non-alpha globin biosynthetic ratios were evaluated before and during treatment. Almost all patients responded with an average increase of 33% in Hb F levels, from a mean (+/- SD) of 42% +/- 11% to 56% +/- 8% (P < .0001), and a reciprocal decline in the percentage of Hb E from 59% +/- 9% to 49% +/- 8% (P < .001). Reticulocytosis was decreased from a mean (+/- SD) of 18.0% +/- 15.6% to 11.7% +/- 9.1% (P < .05); there was also a slight (10%) but statistically significant increase in hemoglobin levels and an improved balance in alpha:non-alpha globin chains ratios. The side effects were minimal in most patients, although these patients tended to tolerate a lower dose of HU before significant myelosuppression than has been our previous experience in sickle cell disease. One splenectomized patient died of sepsis during the trial. We conclude that increased Hb F production in beta-thalassemia/Hb E patients, with an improvement in the alpha:non-alpha globin ratios and, probably, the effectiveness of erythropoiesis, can be achieved using HU. Longer trials of HU in this population, including at other doses and in combination with other agents, appear warranted.
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PMID:Hydroxyurea increases hemoglobin F levels and improves the effectiveness of erythropoiesis in beta-thalassemia/hemoglobin E disease. 856 58

Fulminant pneumococcal sepsis is a rare but life-threatening illness usually occurring in patients with known splenic absence (postsplenectomy) or dysfunction (sickle cell anemia). Several medical illnesses, not typically recognized as being associated with abnormal spleen function, may be complicated by fulminant pneumococcal sepsis. We report a case of fatal pneumococcal sepsis in a patient diagnosed with mixed connective tissue disease who likely had systemic lupus erythematosus and unsuspected splenic fibrosis. Medical illnesses associated with functional asplenia, hematological findings suggesting splenic dysfunction, and confirmatory tests of hyposplenism are discussed.
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PMID:Fatal pneumococcal septicemia in a patient with a connective tissue disease. 865 35

Streptococcus pneumoniae sepsis is the most common invasive infection among patients with sickle cell disease. The risk of a recurrent episode of sepsis and subsequent death in those patients who have had a previous septic event is much higher. Patients with sickle disease who have had pneumococcal sepsis should continue penicillin prophylaxis indefinitely and should not be candidates for out-patient management of febrile episodes.
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PMID:Recurrent Streptococcus pneumoniae sepsis in children with sickle cell disease. 915 75

The prevalence of drug-resistant Streptococcus pneumoniae (DRSP) has increased worldwide. Although unfavorable outcomes in meningitis due to DRSP have been well-described, the clinical impact of DRSP on other manifestations of pneumococcal infection warrants further study. Empiric combination vancomycin and extended-spectrum cephalosporin (cefotaxime or ceftriaxone) therapy is indicated for the following clinical indications: purulent meningitis, life-threatening pneumonia, and suspected sepsis in patients predisposed to invasive pneumococcal disease, for example, sickle cell disease, HIV infection, and nephrotic syndrome. In addition to clinical management issues, other implications of the emergence of DRSP include identification of resistant strains, local and national surveillance, and prevention. Preventive measures include judicious antibiotic use, appropriate use of the currently available 23-valent pneumococcal vaccine, and development and implementation of a protein-conjugate vaccine.
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PMID:The clinical impact of resistance in the management of pneumococcal disease. 942 4

Patients with sickle cell disease (SCD) are predisposed to infections. There is a paucity of recent information on the incidence of post-splenectomy infectious complications in these patients. The purpose of this study was to determine whether splenectomy increases infectious complications in SCD. Twenty-nine patients with SCD had splenectomy for sequestration crises at our hospital between 1988 and 1992; 16 of them received all of their follow-up care at our institution. These 16 charts were reviewed for infectious-related admissions, hospital days, days of i.v. antibiotics, positive cultures, and episodes of sepsis. For each patient, these parameters in the pre- and postoperative period were compared and expressed as number per year. The mean age at time of splenectomy was 2.5 +/- 0.4 years and the mean follow-up was 4.5 +/- 0.4 years. There was no significant difference in the pre- and postoperative periods for admissions, hospital days, days of i.v. antibiotics, positive cultures, or episodes of sepsis per year. There were also no operative deaths. The incidence of pre-splenectomy sepsis was 0.04 +/- 0.03 episodes per year compared to 0.09 +/- 0.04 (P = ns) episodes/year after splenectomy. Sepsis occurred at an average of 20.8 (range 2-30) months postoperatively; Streptococcus pneumoniae was the most common causative organism. The total mortality after splenectomy in SCD patients was 3.4% (1/29) over a nearly 5-year period. Although infections are common in children with SCD, there was no increase in infections or episodes of sepsis in SCD patients who underwent splenectomy.
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PMID:Septic complications after splenectomy for sickle cell sequestration crisis. 956 17

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

Heparin Cofactor II (HCII) is a glycoprotein in human plasma which inactivates thrombin rapidly in the presence of dermatan sulfate. Inhibition occurs by formation of a stable equimolar complex between HCII and thrombin. HCII association with thrombotic events has not always been observed, thus decreased HCII does not appear to be a strong risk factor for thromboembolic events. Reduced HCII levels have been detected in different clinical conditions, such as hepatic failure, disseminated intravascular coagulation, thalasemina, sickle cell anemia. Increased physiological levels have been found in pregnant women and oral contraception. In our laboratory, we measured HCII plasmatic levels in the normal Buenos Aires city population and in patients under different clinical conditions, such as sepsis, diabetis, burns, oral anticoagulation and in patients treated with heparin, hyperhomcysteinemia in whom septic and diabetic patients showed decreased values. HCII thrombin inhibition possibly takes place in extravascular sites where dermatan sulfate is present. HCII activity would be important in the regulation of wound healing, inflammation, or neuronal development.
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PMID:[Heparin cofactor II, a thrombin inhibitor with a still not clarified physiologic role]. 1034 31

A common chronic feature of sickle cell disease is the presence of painful, punched-out leg ulcers. Other cutaneous findings in patients with homozygous sickle cell disease have not been described in the literature. We present a case of a 50-year-old black woman with sickle cell disease who was admitted for acute onset of arm and hip pain. After admission she deteriorated clinically, with multiorgan failure and mental status changes. Examination of the skin revealed erythematous papules and plaques with scaly centers and purpura on the upper trunk. The clinical differential diagnosis was vasculitis versus sepsis. Skin biopsy of two representative lesions was performed. Hematoxylin- and eosin-stained sections showed a superficial perivascular mixed inflammatory infiltrate with numerous eosinophils and extravasated erythrocytes, some of which exhibited bizarre morphology of sickled red blood cells. These findings indicated that the patient's cutaneous lesions, possibly multifactorial in origin, could be a component of her sickle cell crisis. This case is presented as an unusual one in which evaluation of erythrocyte morphology contributed to patient management and to emphasize the importance of examining erythrocyte morphology as a part of the histologic evaluation of stained tissue.
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PMID:Purpura as a cutaneous association of sickle cell disease. 1044 83

Sickle cell disease in patients undergoing open heart procedures presents a multitude of challenges to the medical staff. With improved techniques of cardiopulmonary bypass, surgery, and anesthesia for treating patients with sickle cell disease, perfusionists will likely encounter patients with this genetic disorder on a more frequent basis. A 40-year-old black woman was admitted to our institution with recurrent Staphylococcus epidermidis and sepsis. She underwent transesophageal echocardiography and cardiac catheterization and was subsequently diagnosed with severe aortic insufficiency. The aortic valve was replaced. Herein, we report our experience in the preoperative, perioperative, and postoperative management of this patient. We present a concise update on the current literature and techniques used by others in similar cases, and we provide a brief section on future considerations to assist fellow practitioners in recognizing this disease and meeting the accompanying challenges.
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PMID:Sickle cell disease and aortic valve replacement: use of cardiopulmonary bypass, partial exchange transfusion, platelet sequestration, and continuous hemofiltration. 1065 57

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


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