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)

The objectives of this study are to determine the trend of maternal mortality at the University of Ilorin Teaching Hospital, to identify the causes of death, and to identify ways of minimizing the frequency of preventable deaths. Analysis of 75 cases of maternal deaths seen over 3 1/2 years (January 1983-June 1986) was conducted. During this period, there were 26,905 births, giving a maternal mortality rate of 279/100,000. 84% of the deaths were due to direct causes while the remaining 16% were classified under the indirect and pregnancy related categories. The main direct causes of death include hemorrhage (35.6%), septicemia (24.7%), and anemia (13.7%). Other direct obstetric causes include eclampsia, anesthetic death, hemoglobinopathy, and ruptured uterus. The most important indirect causes were native drug intoxication (6.8%), fulminant hepatitis (5.5%), and pulmonary embolism (2.7%). The maternal mortality was highest in the age ranges 25-29 years (31.5%). Median age and parity were 27 years and 4.5 respectively. While the maternal mortality rate of 2.8/1000 is an improvement over the previous years' (1972-1982) record of 4.3/1000, it is still unacceptable. The majority of these deaths could have been prevented if delivery had occurred in a well equipped hospital where blood transfusion and surgical facilities are available, if sterile manipulations for pregnant women had been employed, if appropriate antenatal care was available, and if specialist anesthetist services were accessible. Recommendations to reduce the maternal mortality rate include improved education and training of traditional birth attendants, improved immunization of women against tetanus, and increased community involvement through education. Furthermore, policy makers must set new priorities such as encouraging greater investment in improving clinics and hospital facilities, improving access to contraception, increasing awareness of the magnitude of the problem and encouraging community leadership and action.
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PMID:Maternal mortality at Ilorin, Nigeria. 1217 82

In Oklahoma since the early 1990s, all newborns have been screened for four metabolic conditions: phenylketonuria, hypothyroidism, galactosemia and hemoglobinopathies. In 2002, 38 affected babies were diagnosed and one expects they are saved from the complications of late diagnosis such as mental retardation or death from sepsis. With advances in genetics and improved biochemical assays, 86% of states now screen for more disorders than Oklahoma, up to 37 in Mississippi. Six recent patient vignettes illustrate the mortality and morbidity of conditions that are screened for elsewhere but not in Oklahoma. In 2001, the Oklahoma Genetics Advisory Council recommended adding three disorders and the State Health Department forecasts that implementation may be complete in 2007. For now, when a patient asks, "Will my baby be screened for as many metabolic conditions as possible?", two answers represent either the public health or the private health care view. The public health answer is, "The state requires screening for four conditions." The health care system answer is, "We can work with you to get 44 conditions tested for, but it will cost money, may not be reimbursed, and has not been proven effective when done on an individual basis." This dilemma, not unique to newborn screening, might be resolved if professional and public opinion strongly supported early expansion.
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PMID:Expanding metabolic screening of newborns: can the health care industry do better than public health? 1461 2

Bacterial infections remain a major cause of morbidity and mortality among young children with sickle cell disease. Susceptibility to infections is mainly observed in homozygous sickle cell disease. The incidence of bacteremias in children under 3 years of age is approximately 8 events/100 patient-years among homozygous subjects and approximately S events/100 patient-years among those with SC hemoglobinopathy. Pneumococci and Salmonellae are the most frequently isolated bacteria. Severe clinical manifestations include septicemia, meningitis, osteomyelitis and pneumonia. M. Pneumoniae and C. Pneumoniae infections may be severe and may induce acute chest syndrome. The high incidence and severity of bacterial infections in these children justify prevention efforts by antibiotic prophylaxis and vaccination. The efficacy of oral penicillin prophylaxis against pneumococcal infections has been well demonstrated and is now recommended from 3 months of age. The antipneumococcal conjugate vaccine has been shown to be safe and immunogenic in young infants.
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PMID:[Infections and antibiotic prophylaxis in sickle cell disease]. 1513 1

Thalassemia is the commonest hemoglobinopathy in Malaysia. Patients with thalassemia major are transfusion dependent, and a large proportion of them will require splenectomy. As this particular group of patients is immunocompromized, overwhelming sepsis is a recognized complication. We report a series of three patients who all developed intra-abdominal abscesses following splenectomy.
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PMID:Case report post-splenectomy sepsis in thalassemic patients. 1591 58

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

Routine clinical and laboratory assessments facilitate diagnosis of erythropoietin (EPO) resistant anemia by allowing early identification of patients with non-adherence. Any new event that impairs response to EPO (e.g., catheter sepsis) must be promptly controlled. Because of the confounding interaction of its risk factors, initial evaluation should include nutrition, dialysis adequacy, hemorrhage, bone mineral metabolism, and inflammation. Prevention of EPO resistance is more cost effective and should include adequate dialysis and nutritional supplements. Blood loss during hemodialysis (HD) procedures should be minimized. If there is laboratory proof of iron deficit intravenous repletion is most effective. Oxidative stress may be attenuated by vitamins E and C, while optimal control of hyperparathyroidism will enhance EPO stimulation. Contaminated dialysates should be suspected if there is EPO-stimulating agents (ESA) resistance at the same time among most members of a dialysis program. Heavy metal toxicity should be suspected in high-risk patients. The impact of co-morbidities such as hemoglobinopathy, glucose 6 phosphate dehydrogenase (G6PD) deficiency and connective tissue diseases must be excluded in an appropriate setting. In conclusion, given the multiple risk factors of EPO resistance promotion of the overall health status will most likely yield an enduring benefit. Finally, there are experimental trials of gene-based (therapy) to stimulate endogenous EPO synthesis with the goal of avoiding the off-target effect of excessive dosing.
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PMID:Resistance to erythropoietin-stimulating agents: etiology, evaluation, and therapeutic considerations. 2142 25

Yersinia enterocolitica is a gram-negative cocobacillus causing a range of illness from self-limited enteritis to invasive disease, including septicemia. It is a particularly virulent pathogen in patients with underlying hemoglobinopathies who are predisposed to iron overload. A substantial risk factor for disease in children and infants is exposure to the household preparation of chitterlings. Early identification of these patients is critical in the pediatric intensive care unit as this cause of septicemia can be missed with the potential for significant morbidity. We report an interesting case of Yersinia septicemia in a patient with iron overload disease from chitterling ingestion managed in the pediatric intensive care unit.
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PMID:Yersinia enterocolitica Septicemia After Chitterling Ingestion in a Pediatric Patient With Iron Overload Disease: A Case Report. 2733 68

Intravascular hemolysis produces injury in a variety of human diseases including hemoglobinopathies, malaria, and sepsis. The adverse effects of increased plasma hemoglobin are partly mediated by depletion of nitric oxide (NO) and result in vasoconstriction. Circulating plasma proteins haptoglobin and hemopexin scavenge extracellular hemoglobin and cell-free heme, respectively. The ability of human haptoglobin or hemopexin to inhibit the adverse effects of NO scavenging by circulating murine hemoglobin was tested in C57Bl/6 mice. In healthy awake mice, the systemic hemodynamic effects of intravenous coinfusion of cell-free hemoglobin and exogenous haptoglobin or of cell-free hemoglobin and hemopexin were compared with the hemodynamic effects of infusion of cell-free hemoglobin or control protein (albumin) alone. We also studied the hemodynamic effects of infusing hemoglobin and haptoglobin as well as injecting either hemoglobin or albumin alone in mice fed a high-fat diet (HFD) and in diabetic (db/db) mice. Coinfusion of a 1:1 weight ratio of haptoglobin but not hemopexin with cell-free hemoglobin prevented hemoglobin-induced systemic hypertension in healthy awake mice. In mice fed a HFD and in diabetic mice, coinfusion of haptoglobin mixed with an equal mass of cell-free hemoglobin did not reverse hemoglobin-induced hypertension. Haptoglobin retained cell-free hemoglobin in plasma, but neither haptoglobin nor hemopexin affected the ability of hemoglobin to scavenge NO ex vivo. In conclusion, in healthy C57Bl/6 mice with normal endothelium, coadministration of haptoglobin but not hemopexin with cell-free hemoglobin prevents acute hemoglobin-induced systemic hypertension by compartmentalizing cell-free hemoglobin in plasma. In murine diseases associated with endothelial dysfunction, haptoglobin therapy appears to be insufficient to prevent hemoglobin-induced vasoconstriction.NEW & NOTEWORTHY Coadministraton of haptoglobin but not hemopexin with cell-free hemoglobin prevents hemoglobin-induced systemic hypertension in mice with a normal endothelium. In contrast, treatment with the same amount of haptoglobin is unable to prevent hemoglobin-induced vasoconstriction in mice with hyperlipidemia or diabetes mellitus, disorders that are associated with endothelial dysfunction.
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PMID:Endothelial dysfunction inhibits the ability of haptoglobin to prevent hemoglobin-induced hypertension. 2831 63

State-of-the-art proteomics technologies have recently helped to elucidate the unanticipated complexity of red blood cell metabolism. One recent example is citrate metabolism, which is catalyzed by cytosolic isoforms of Krebs cycle enzymes that are present and active in mature erythrocytes and was determined using quantitative metabolic flux analysis. In previous studies, we reported significant increases in glycolytic fluxes in red blood cells exposed to hypoxia in vitro or in vivo, an observation relevant to transfusion medicine owing to the potential benefits associated with hypoxic storage of packed red blood cells. Here, using a combination of steady state and quantitative tracing metabolomics experiments with 13C1,2,3-glucose, 13C6-citrate, 13C515N2-glutamine, and 13C1-aspartate via ultra-high performance liquid chromatography coupled on line with mass spectrometry, we observed that hypoxia in vivo and in vitro promotes consumption of citrate and other carboxylates. These metabolic reactions are theoretically explained by the activity of cytosolic malate dehydrogenase 1 and isocitrate dehydrogenase 1 (abundantly represented in the red blood cell proteome), though moonlighting functions of additional enzymes cannot be ruled out. These observations enhance understanding of red blood cell metabolic responses to hypoxia, which could be relevant to understand systemic physiological and pathological responses to high altitude, ischemia, hemorrhage, sepsis, pulmonary hypertension, or hemoglobinopathies. Results from this study will also inform the design and testing of novel additive solutions that optimize red blood cell storage under oxygen-controlled conditions.
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PMID:Metabolism of Citrate and Other Carboxylic Acids in Erythrocytes As a Function of Oxygen Saturation and Refrigerated Storage. 2909 Feb 12

Pulse oximetry is routinely used in the newborn nursery for clinical monitoring and to detect critical congenital heart disease. The differential diagnoses for reduced peripheral oxygen saturation in an infant include congenital heart disease, respiratory distress syndrome, transient tachypnea of the newborn, persistent pulmonary hypertension of the newborn, meconium aspiration syndrome, pneumonia, pneumothorax, and sepsis. The diagnostic evaluation for neonatal hypoxemia can be invasive and expensive. When this evaluation is unrevealing, other interventions may be tried without clear benefit to the patient, including, but not limited to, supplemental oxygen. Therefore, it is important to consider alternative, albeit rare, diagnoses, including hemoglobinopathies with abnormal oxygen binding properties. Mutations in the structure of alpha- and beta-globin chains can alter the affinity of hemoglobin for oxygen, and changes in oxygen affinity may result in changes in the oxygen saturation detected by pulse oximetry. These changes may or may not be of clinical significance. This case report describes Hemoglobin Sunshine Seth, a rare low-oxygen-affinity hemoglobin variant presenting as reduced peripheral oxygen saturation in an otherwise well-appearing infant male.
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PMID:Hemoglobin Sunshine Seth: A Case Report of Low-Oxygen-Affinity Hemoglobinopathy. 3241 91


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