Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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 X-linked hyper-IgM (XHIGM) syndrome is an uncommon primary immunodeficiency disease caused by mutations in the gene for CD40 ligand and characterized by normal or elevated serum IgM, reduced levels of IgG and IgA, and defective T-cell function. Because of its rarity, it has been difficult for any single investigator or institution to develop a comprehensive clinical picture of this disorder. Accordingly, a national registry was developed in the United States to provide demographic, genetic, immunologic, and clinical information on a relatively large number of patients with the XHIGM syndrome.A total of 79 patients from 60 unrelated families were registered between January 1997 and July 2002. The estimated minimal incidence was approximately 1/1,030,000 live births. All of the patients had significant IgG deficiency and most had IgA deficiency, but only one-half had elevated IgM levels. Most patients presented initially with a history of an increased susceptibility to infection including Pneumocystis carinii pneumonia. The average age of diagnosis was significantly earlier in patients born into a family with a previously affected individual. However, only one-third of the patients born into a family with a previously affected individual were diagnosed exclusively because of the presence of the positive family history before any clinical symptoms developed. Over half the patients developed symptoms of immunodeficiency and were diagnosed by 1 year of age, and over 90% by 4 years of age. The most prominent clinical infections were pneumonia (81% of patients), upper respiratory infections (49%) including sinusitis (43%) and recurrent otitis (43%), recurrent/protracted diarrhea (34%), central nervous system infections (14%), sepsis (13%), cellulitis (13%), hepatitis (9%), and osteomyelitis (1%). In addition to infections caused by encapsulated bacteria, opportunistic infections were relatively common and were caused by P. carinii, members of the herpes virus family (including cytomegalovirus), Cryptosporidium, Cryptococcus, Candida, Histoplasma, and Bartonella. Sclerosing cholangitis occurred in 5 patients and in 4 of these was associated with Cryptosporidium infection. Eight patients had died at the time of their entry into the Registry; 2 of pneumonia (1 P. carinii and 1 cytomegalovirus), 2 of encephalitis (1 ECHO virus and 1 cytomegalovirus), 2 of malignancy (both hepatocellular carcinoma), 1 of sclerosing cholangitis caused by Cryptosporidium, and 1 of hemolytic uremic syndrome.
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PMID:The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients. 1466 87

It is still not quite well understood why there is no optimal or even a satisfactory antibiotic therapy for listeriosis. Although almost all Listeria strains that induce sepsis, meningitis and encephalitis, as well as many other manifestations--in particular, in immunocompromised individuals--are susceptible to most of the common antibiotics, the cure rate is only approximately 70%. The most effective regimen still consists of a combination of an aminopenicillin (amoxicillin or ampicillin) plus an aminoglycoside. In vitro, this combination is bactericidal, whereas aminopenicillin alone only exerts a weak bactericidal activity against Listeriae. These antibiotics only poorly penetrate the cerebrospinal fluid and thus, only high doses given over a prolonged period of 2-3 weeks are curative. Furthermore, Listeria monocytogenes belongs to the group of facultative intracellular bacteria, which means that a certain population is inaccessible for antibiotics. Theoretically, a drug which is endowed with bactericidal activity superior to that of ampicillin would be preferable. Furthermore, the candidate drug should easily cross the blood-brain barrier into the CNS, be able to accumulate within host cells, reach the cytoplasm and be active under these unusual conditions. Because of all these arguments, the new quinolones are of particular interest; but broad clinical data are still lacking. It is unclear as to whether antibiotics alone will be sufficient to increase the prognosis. Adjunctive therapy with immunomodulators, which are able to reconstitute the defective defence capacities, would presumably create the conditions necessary to finally resolve listeriosis.
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PMID:An update on the medical management of listeriosis. 1526 87

Recent aspects of pathogen determination and therapeutic management are reviewed based on a clinical analysis of 50 of our adult patients with bacterial meningitis (BM). The sensitivity for detecting pathogens using gram-stained smears and cultures of CSF was high in untreated patients, but low in patients previously treated with antibiotics. Latex agglutination for antibodies of pathogens is rapid and has a potential for determining pathogens in partially treated meningitis. The PCR also has a potential for determining pathogens in pre-treated, culture-negative cases and for detecting whether the bacterial pathogen is resistant or sensitive to antibiotics. The initial empiric regimen of antibiotics has been modified with elevation in the detection rate of drug-resistant bacilli. The proportion of patients undergoing treatment with VCM and/or Carbapenems has recently increased at our department. A combination of dexamethasone under administration of antibiotics has also been established as effective in adult BM. On admission, some patients are difficult to diagnose as BM rather than herpes simplex encephalitis (HSVE). A serum CRP value of >2.0 mg/dl proved useful for such different diagnosis in our patients. The predictors of a poor outcome based on multivariate logistic analysis in our BM patients were level of unconsciousness and sepsis.
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PMID:[Bacterial meningitis: determination of pathogens and therapeutic management]. 1565 10

A 22 year old female was admitted to the emergency department with high fever up to 41,5 degrees C, tachycardia, and arterial hypotension. Clinically, she presented with bilateral pulmonary coarse crackles. Diagnosis on admission was pneumonia with septic shock. Intriguingly, procalcitonin (PCT) was increased early, reaching up to 435 ng/mL, while C-reactive protein levels were only moderately increased, with several days delay. The sepsis was originated from a multi-resistant pseudomonas aeruginosa pneumonia. Remarkably, the course of PCT levels reflected the severity of septic shock in that it paralleled noradrenaline demand. Ten months previously, the patient had been diagnosed with acute disseminated brainstem encephalitis (ADEM), and had received two cycles of intravenous cyclophosphamide. Our case illustrates that PCT is an early marker for sepsis and it indicates that PCT may also be a valuable marker for the severity of sepsis in immunosuppressed patients.
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PMID:[Procalcitonin as a marker for severe sepsis in an immunosuppressed patient]. 1571

On September 11, 2001, the Connecticut Department of Public Health (CDPH) initiated daily, statewide syndromic surveillance based on unscheduled hospital admissions (HASS). The system's objectives were to monitor for outbreaks caused by Category A biologic agents and evaluate limits in space and time of identified outbreaks. Thirty-two acute-care hospitals were required to report their previous day's unscheduled admissions for 11 syndromes (pneumonia, hemoptysis, respiratory distress, acute neurologic illness, nontraumatic paralysis, sepsis and nontraumatic shock, fever with rash, fever of unknown cause, acute gastrointestinal illness, and possible cutaneous anthrax, and suspected illness clusters). Admissions for pneumonia, gastrointestinal illness, and sepsis were reported most frequently; admissions for fever with rash, possible cutaneous anthrax, and hemoptysis were rare. A method for determining the difference between random and systemic variation was used to identify differences of >/=3 standard deviations for each syndrome from a 6-month moving average. HASS was adapted to meet changing surveillance needs (e.g., surveillance for anthrax, smallpox, and severe acute respiratory syndrome). HASS was sensitive enough to reflect annual increases in hospital-admission rates for pneumonia during the influenza season and to confirm an outbreak of gastrointestinal illness. Follow-up of HASS neurologic-admissions reports has led to diagnosis of West Nile virus encephalitis cases. Report validation, syndrome-criteria standardization among hospitals, and expanded use of outbreak-detection algorithms will enhance the system's usefulness.
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PMID:Hospital admissions syndromic surveillance--Connecticut, September 200-November 2003. 1571 28

A 6-year-old girl with community-acquired disseminated infection caused by methicillin-resistant Staphylococcus aureus (MRSA) is described. She had sepsis, meningo-encephalitis, pyomyositis, osteomyelitis, pericarditis and pulmonary embolisation caused by a multi-resistant strain of MRSA. Vancomycin is not routinely recommended as the first-line antimicrobial agent for suspected Staphylococcus aureus infection; however, it should be considered pending susceptibility results in patients presenting with severe sepsis in areas where the prevalence of MRSA is high.
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PMID:Community-acquired disseminated methicillin-resistant Staphylococcus aureus infection: case report and clinical implications. 1581 50

Serious infection with the bacterium L. monocytogenes mainly manifests as sepsis and/or meningitis. A particular entity is Listeria brain stem encephalitis, which is characterized by progressive brain stem deficits. The condition is fatal unless early treated. The purpose of the present study was to assess the incidence of brain stem encephalitis in a population-based listeriosis material. Medical records from 212 of the 240 patients with serious listeriosis reported in Norway from 1977 to 2000, as well as autopsy material from 8 of these patients, were available. This material was searched for clinical and neuropathological evidence of brain stem infection. Findings indicating brain stem encephalitis were present in 19 of the 172 patients with adult listeriosis (11%) but none of the 40 pregnancy-related listeriosis cases. None of the 19 patients had been diagnosed with Listeria brain stem infection originally. We conclude that brain stem encephalitis is relatively common in this Norwegian listeriosis material.
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PMID:Brain stem encephalitis in listeriosis. 1584 51

Here we describe 2 patients with acute leukemia in whom human herpesvirus-6 (HHV-6) encephalitis developed after cord blood transplantation. In patients 1 and 2, generalized seizure and coma developed on day 62 and day 15, respectively, after cord blood transplantation, which failed to engraft in patient 1. Magnetic resonance imaging (MRI) of patient 1's brain showed low-intensity signals at the gyri of the bilateral lateral lobes on T1-weighted images and high-intensity signals on T2-weighted images. MRI of patient 2's brain showed high-intensity signals in bilateral white matter on T2-weighted images and on fluid-attenuated inversion recovery (FLAIR) images. Cerebrospinal fluid examination revealed an increased protein level with pleocytosis in patient 1 and a normal protein level without pleocytosis in patient 2. Polymerase chain reaction analysis detected HHV-6 DNA in the cerebrospinal fluid of both patients. Patient 1 recovered after administration of gancyclovir for 3 weeks. However, she again suffered from encephalitis after discontinuation of gancyclovir, and died of sepsis. Patient 2 died from an anoxic brain caused by generalized seizure. When neurological symptoms and signs appear in hematopoietic stem cell transplantation recipients, we should consider HHV-6 encephalitis and promptly and empirically treat them with gancyclovir or foscarnet.
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PMID:Human herpesvirus-6 encephalitis after unrelated cord blood transplantation. 1601 84

Viral encephalitis is associated with significant morbidity and mortality, particularly when appropriate management is omitted as a result of delayed diagnosis. A case of herpes simplex virus type 1 (HSV-1) encephalitis is presented, demonstrating that the presentation of confusion, speech difficulties and fever with non-specific early brain CT appearances can easily be misdiagnosed as pneumonia with stroke. This case highlights the need for increased awareness of HSV-1 encephalitis among emergency physicians and radiologists, given that the early spectrum of clinical and CT findings can mimic the more common diagnoses of sepsis and stroke.
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PMID:Stroke or encephalitis? 1609 Nov 6

This study investigated the clinical manifestations and outcomes of central nervous system (CNS) infection by enteroviruses. Cases with CNS involvement among all enterovirus-culture-positive cases from January 1995 to June 2003 were retrospectively reviewed. Among 1028 enterovirus-culture-positive cases, there were 333 cases involving the CNS. Of these, the ratio of male to female subjects was 1.78, and the mean (+/- standard deviation) age was 6.83 +/- 5.9 years; 21 were premature neonates, and 10 failed to thrive. Disease entities included 282 cases of aseptic meningitis (84.7%), 44 cases of encephalitis (13.2%), and 7 cases of encephalomyelitis/polio-like syndrome (2.1%). Of these cases, 97.9% (326/333) had fever with peak body temperature at 38.9 degrees C, 85% had headache and vomiting, 70% had meningeal signs, 64% had neck stiffness, 16.6% (55/333) had change of consciousness, 5.4% (18/333) had seizures and 5.2% (17/333) had myoclonic jerks. Mannitol was administered in 77.2% of patients (257/333), along with intravenous immunoglobulin in 6.6% (22/333). Twelve cases received ventilator support. One patient died of hand-foot-and-mouth disease, encephalitis plus cardiopulmonary failure, and 2 premature neonates died of hepatic failure, disseminated intravascular coagulation, sepsis-like syndrome and myocarditis. Eighteen had neurologic sequelae, including 7 with limb weakness, 5 with epilepsy, 2 with sixth cranial nerve palsy, 3 with cerebral palsy, 4 with psychomotor retardation, 2 with spasticity, and 1 with hearing loss. Factors associated with unfavorable outcomes (death or sequelae) included younger age (p=0.0003), higher peak white blood cell count (WBC) [p=0.0009] and skin rash (p=0.005). Younger age and higher peak WBC were poor prognostic factors of severe enterovirus CNS infection. Death was related to neonatal enterovirus infection and enterovirus 71 infection in young children.
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PMID:Clinical features and factors of unfavorable outcomes for non-polio enterovirus infection of the central nervous system in northern Taiwan, 1994-2003. 1634 42


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