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)

A rhabdoviral disease occurred in farmed populations of market sized Japanese flounder (hirame) Paralichthys olivaceus in the Seto Inland Sea of Japan in 1996. The causative agent was identified as viral hemorrhagic septicemia virus (VHSV) based on morphological, immunological, and genetic analyses. Diseased fish that were artificially injected with a representative virus isolate showed the same pathological signs and high mortality as observed in the natural outbreak. This is the first report of an outbreak of VHSV infection in cultured fish in Japan. Clinical signs of diseased fish included dark body coloration, an expanded abdomen due to ascites, congested liver, splenomegaly, and a swollen kidney. Myocardial necrosis was most prominent and accompanied by inflammatory reactions. Necrotic lesions also occurred in the liver, spleen and hematopoietic tissue, and were accompanied by circulatory disturbances due to cardiac failure. Hemorrhagic lesions did not always appear in the lateral musculature. Transmission electron microscopy revealed many rhabdovirus particles and associated inclusion bodies containing nucleocapsids in the necrotized myocardium. The histopathological findings indicated that the necrotizing myocarditis could be considered a pathognomonic sign of VHSV infection in Japanese flounder.
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PMID:An outbreak of VHSV (viral hemorrhagic septicemia virus) infection in farmed Japanese flounder Paralichthys olivaceus in Japan. 1177 99

Splenic infarcts are common in patients with sickle cell anemia (SCA), but these are usually small and repetitive, leading ultimately to autosplenectomy. Massive splenic infarcts on the other hand are extremely rare. This is a report of our experience with 8 (4 males and 4 females) cases of massive splenic infarction in patients with SCA. Their ages ranged from 16 to 36 years (mean 22 years). Three presented with left upper quadrant abdominal pain and massive splenic infarction on admission, while the other 5 developed massive splenic infarction while in hospital. In 5 the precipitating factors were high altitude, postoperative, postpartum, salmonella septicemia, and strenuous exercise in one each, while the remaining 3 had severe generalized vasoocclusive crises. Although both ultrasound and CT scan of the abdomen were of diagnostic value, we found CT scan more accurate in delineating the size of infarction. All our patients were managed conservatively with I.V. fluids, analgesia, and blood transfusion when necessary. Diagnostic aspiration under ultrasound guidance was necessary in two patients to differentiate between massive splenic infarction and splenic abscess. Two patients required splenectomy during the same admission because of suspicion of secondary infection and abscess formation, while a third patient had splenectomy 2 months after the attack because of persistent left upper quadrant abdominal pain. In all the 3 histology of the spleen showed congestive splenomegaly with massive infarction. All of our patients survived. Two patients subsequently developed autosplenectomy while the remaining 3 continue to have persistent but asymptomatic splenomegaly. Massive splenic infarction is a rare and unique complication of SCA in the Eastern Province of Saudi Arabia, and for early diagnosis and treatment, physicians caring for these patients should be aware of such a complication.
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PMID:Massive splenic infarction in Saudi patients with sickle cell anemia: a unique manifestation. 1189 8

Traumatic rupture of the spleen in the newborn is a rare event and is seldom reported in the literature. It can occur to an abnormally enlarged spleen or to a normal spleen. In the latter, it usually is associated with difficult delivery. Previously, the majority of patients died probably because of delayed or missed diagnoses. The classic presentation is a triad of bleeding, abdominal distension, and hemoperitoneum. High index of suspicion and improvement in diagnostic tools like ultrasonography and computed tomography are important contributions in early diagnosis so that appropriate treatment can be implemented. Splenectomy is no longer the standard treatment because it increases the chance of postsplenectomy sepsis. Instead, treatment should aim for hemostasis and preservation of spleen. The authors present a case of traumatic rupture of spleen in a normal newborn with normal labor and delivery. The first symptom began at 16 hours of age and evolved to a full-blown classic picture. The baby was saved without splenectomy, and the recovery was smooth and uneventful. This is probably the first reported case of a patient treated without splenectomy in this locality. Discussion of the condition and review of the literature also are presented.
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PMID:Splenic rupture in a newborn. 1191 38

Endotoxin has been identified as a principal mediator of sepsis, often with resulting multiple organ failure. Although interferon gamma (IFN-gamma) has a central role in controlling bacterial infection through the activation of macrophages and T lymphocytes, it can also enhance the harmful effects of the inflammatory response. To examine the role of IFN-gamma in lipopolysaccharide (LPS)-induced injury, we administered LPS (20 or 800 microg/mouse) alone or as low-dose LPS (20 microg/mouse) 7 days after heat-killed Propionibacterium acnes (P. acnes) injection into wild-type C57BL/6 (B6) mice or IFN-gamma-deficient (GKO) mice (B6 background). Although low-dose (20 microg) LPS alone had no effect on survival, the administration of 800 microg LPS alone resulted in 100% mortality in both B6 and GKO mice without significant hepatic mononuclear cellular infiltration or differences in elevated plasma tumor necrosis factor alpha (TNF-alpha), interleukin 6 (IL-6), and IL-12 levels. In contrast, mortality after low-dose (20 microg) LPS challenge in P. acnes-primed B6 mice was 100%, but 0% in GKO mice. In vivo plasma cytokine (IFN-gamma, TNF-alpha, IL-6, and IL-12) levels and in vitro cytokine production by hepatic mononuclear cells were significantly higher in B6 mice compared with GKO mice. Associated hepatic mononuclear cellular infiltration, multifocal liver necrosis, hepatomegaly, and splenomegaly were found in B6 mice, but not in GKO mice. Finally, the anti-inflammatory NK1.1+CD4+ cell proportion of hepatic infiltrating mononuclear cell numbers 7 days after P. acnes administration was significantly reduced in B6 compared with GKO mice, whereas the proportion of inflammatory NK1.1+CD4- cells was increased. In conclusion, these data suggest that IFN-gamma mediates P. acnes-primed low-dose LPS injury through the hepatic infiltration of mononuclear cells and the subsequent elevation of inflammatory cytokines after LPS challenge, whereas the lethal effects of high-dose LPS alone does not depend on the presence of IFN-gamma.
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PMID:The resistance of P. acnes--primed interferon gamma-deficient mice to low-dose lipopolysaccharide-induced acute liver injury. 1191 26

Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3 years, range 1-22.3 years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5 years (range 3.1-33.6 years). One and 5 years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to bile duct dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and bile duct dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n = 3), sclerotherapy (n = 2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n = 1). Of the nine survivors (mean age 12.8 years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.
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PMID:Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease. 1211 59

Partial splenic embolization (PSE) has been demonstrated to be an effective alternative to splenectomy for patients with hypersplenism. Splenectomy in these patients can be associated with an increased risk of perioperative complications, overwhelming post-splenectomy sepsis (OPSS) and mortality. Partial splenic embolization has the advantages of non- operative intervention and resolution of the complications of hypersplenism. We report the use of this technique in patients with portal hypertension and hypersplenism awaiting liver transplant and patients that have undergone othotopic liver transplantation (OLTx) with persistent hypersplenism post-transplant. Six patients--three awaiting liver transplantation and three patients with persistent hypersplenism status post-OLTx--were treated during the period of 1993-99 at the LSUHSC/Willis Knighton Regional Transplant Center in Shreveport, Louisiana. Three patients were male and three female. All six patients had concomitant thrombocytopenia and neutropenia with platelet counts below 50,000. Patients underwent selective arterial catheterization and embolization via a percutaneous approach with Cook microcoils or PVA particles. The lower pole of the spleen was selectively embolized in all patients to achieve a 30-50% reduction in flow as determined by angiography. Patients were followed with routine computed tomography (CT) scans, platelet and WBC counts for a mean of 26 months in the pre-transplant and 37 months in the post-transplant group. In both groups, all patients had persistent resolution of thrombocytopenia and neutropenia after embolization. In the post-transplant group, one patient had persistent splenomegaly and required splenectomy for pain control. No procedure-related complications occurred in any patient. In this limited review, PSE appears to be a safe and effective treatment of persistent hypersplenism in patients with portal hypertension and those who have undergone OLTx.
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PMID:Partial splenic embolization for hypersplenism before and after liver transplantation. 1237 46

Ninety-five HIV-infected children aged 3-5 and 6-14 years were examined. Clinical picture matched primary symptoms of stage IIV (phase of generalized lymphadenopathy by Pokrovsky V.I. classification, 1989) and included basic symptoms associated with HIV cytopathogenic action. Local opportunistic infections, either without damage of vital organs (phase IIIA of the secondary stage), or with damage of these organs (phase IIIB) were observed in patients with pre-AIDS. Generalized opportunistic infections and malignant tumors developed in patients with AIDS (phase IIIB and terminal stage IV). High frequency of bacterial infections, bacterial sepsis, splenomegaly, respiratory, gastrointestinal, cerebral, cardiac, common-infections syndromes, and rapid progression characterized the disease in young children. Local and generalized viral, fungal and protozoal infections were more frequently revealed in older children. Immunological alterations (T cell immunodeficiency, polyclonal activation of B lymphocytes, abnormalities of neutrophil metabolism) were diognosed in all children with phase IIV and were the most pronounced in young children. Progressive B lymphocyte polyclonal activation in young children, T cell immunodeficiency in older children and activation of neutrophil metabolism in those with pre-AIDS were observed. T cell immunodeficiency, B lymphocyte polyclonal activation, decreased neutrophil metabolism progressed steadily in all the patients with AIDS.
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PMID:Clinical and Immunological Features of HIV-Infection in Children Aged 3-5 and 6-14 Years. 1268 84

In this study we aimed to investigate the findings in patients with adult-onset Still's disease (AOSD) admitted with fever of unknown origin (FUO) during the last 18 years in our unit, in order to discover the ratio of such patients to all patients with FUO during the same period, and to determine the clinical features of AOSD in FUO. The number and the aetiologies of the patients with FUO diagnosed between 1984 and 2001, and the clinical features of those with AOSD, were taken from the patient files. The diagnosis of AOSD was reanalysed according to the diagnostic criteria of Cush et al. [11]. The presumed diagnoses before a diagnosis of AOSD was established were also noted. The chi(2) and Fisher's exact tests were used for statistical analysis. We studied 130 patients with a diagnosis of FUO, 36 (28%) of whom had collagen vascular diseases. Of these 36 patients, 20 (56%, 12 female, 8 male, mean age 34 years, range 16-65) had AOSD. Clinical and laboratory findings were as follows: fever (100%), arthralgia (90%), rash (85%), sore throat (75%), arthritis (65%), myalgia (60%), splenomegaly (40%), hepatomegaly (25%), lymphadenopathy (15%), anaemia (65%), neutrophilic leukocytosis (90%), increased erythrocyte sedimentation rate (100%), elevated transaminase levels (65%), a negative RF (100%), and a negative FANA (80%). Antibiotics had been prescribed in 18 (90%) of cases. The presumed infectious diagnoses were streptococcal tonsillitis/pharyngitis (50%), infective endocarditis (four patients), sepsis (two patients) and acute bacterial meningitis (two patients). The presumed non-infectious diagnoses were acute rheumatic fever (three patients), seronegative rheumatoid arthritis (two patients) and polymyositis (two patients). Sixteen patients were followed for a mean duration of 30 months (range 2-59). A remission was obtained with indomethacin in three cases (19%), and with prednisolone in the remainder. Relapse was detected in three cases (19%). AOSD is one of the most frequent aetiologies of FUO. During the diagnostic course of a patient with FUO, a maculopapular rash and/or arthralgia and/or sore throat should raise the suspicion of AOSD. Because the disease has heterogeneous clinical findings, certain bacterial infections (e.g. streptococcal pharyngitis and sepsis) are generally considered and the prescribing of antibiotics is common.
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PMID:Fever of unknown origin: a review of 20 patients with adult-onset Still's disease. 1274 Jun 70

The role of preoperative stress single-photon emission computed tomographic (SPECT) imaging in patients with end-stage liver disease who underwent liver transplantation is not well established. We reviewed medical records of patients who had liver transplantation at our institution between January 1998 and November 2001. During this time, 339 patients (213 men, aged 51 +/- 11 years) underwent liver transplantation. Of these, 87 patients had preoperative stress SPECT imaging. Diabetes mellitus (30% vs 11%), hypertension (26% vs 12%), and coronary artery disease (15% vs 7%) were more prevalent in those with than without SPECT (p <0.01 each). The stress SPECT perfusion images were normal in 78 patients (91%) and the left ventricular ejection fraction was 72 +/- 10%. SPECT images revealed ascites in 66% and splenomegaly in 83% of patients. There were 35 total deaths (10%) and 5 nonfatal myocardial infarctions over a mean follow-up of 21 +/- 13 months. Most deaths (32 of 35) were noncardiac and sepsis was the most common cause of death. A normal SPECT study had a 99% negative predictive value for perioperative cardiac events. Kaplan-Meier survival curves showed an 87% 2-year cumulative survival rate in the total group. Thus, in patients undergoing liver transplantation, 2-year survival depends on early noncardiac events. A normal stress SPECT study identified patients at a very low risk for early and late cardiac events despite a higher risk profile. SPECT images also revealed unique findings, such as ascites and splenomegaly, which could produce image artifacts and may interfere with accurate image interpretation.
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PMID:Usefulness of preoperative stress perfusion imaging in predicting prognosis after liver transplantation. 1458 57

Autopsy study from 21 patients with hairy cell leukemia was performed. All patients had the expected widespread involvement of the hematopoietic system. Leukemic infiltration of lymph nodes was detected in 12 cases. Liver involvement was present in 19 patients, leukemic infiltration ranged from focal portal and sinusoidal infiltration to massive infiltration that effaced the hepatic architecture. Twelve patients showed leukemic infiltration of the spleen, remaining 9 patients underwent previous splenectomy for massive splenomegaly. We also found leukemic infiltration of kidneys in four cases, two patients showed leukemic involvement of the lungs. The cause of death was related to impaired immunity (sepsis, bronchopneumonia, etc.) in the majority (73%) of cases.
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PMID:Hairy cell leukemia: an autopsy study. 1496 69


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