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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemochromatosis, or primary iron overload, is a variably expressed genetic metabolic disorder greatly modified by sex, age, diet, and alcohol consumption. Although a diagnosis has been made at the bedside by careful documentation of the slow resolution of subcutaneous iron pigment, clinical diagnosis is frequently overlooked, and even autopsy may fail to reveal hemochromatosis as the cause for cirrhosis. Genetic linkage studies have confirmed the extremely high prevalence of this disorder. Untreated patients may succumb to sepsis caused by organisms such as Vibrio vulnificus, Yersinia species, and others whose virulence is altered by iron availability.
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PMID:Hemochromatosis and infection: alcohol and iron, oysters and sepsis. 248 33

We present a patient with idiopathic liver hemochromatosis and mild secondary cirrhosis complicated by Yersinia sepsis and miliary liver abscesses proven by echography and CT.
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PMID:Ultrasound and CT of multifocal liver abscesses caused by Yersinia enterocolitica. 267 27

A 67-year-old man with Escherichia coli bacteremia and meningitis was found to have hemochromatosis. To my knowledge this is the first documented case of E coli meningitis occurring in the setting of hemochromatosis. The case raises issues regarding the role of chronic liver disease in the pathogenesis of gram-negative sepsis and the impact of iron loading on host immunocompetence and bacterial virulence.
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PMID:Escherichia coli bacteremia, meningitis, and hemochromatosis. 389 40

Acute abdomen, irreversible shock and sudden death are a typical although infrequent complication in patients with hemochromatosis. The author presents a further case of this syndrome and discusses the two leading pathogenetic interpretations described in the literature: sudden release of ferritin, and endotoxin shock. Clinical and post-mortem findings from this patient and a review of 19 cases from the literature suggest that most patients with this syndrome die from a primary bacterial peritonitis with gram negative sepsis and endotoxin shock.
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PMID:[Acute abdomen with irreversible shock, a rare but typical complication of hemochromatosis]. 390 80

We analyzed the clinical data and liver histology for iron overload in 74 renal allograft recipients. Twenty of the 74 patients had histological evidence of hemosiderosis. Four patients had hemochromatosis. Of the 2 noninvasive diagnostic tests the serum ferritin level was more reliable than percent saturation of transferrin in predicting the histological diagnosis of hemosiderosis. Of the 20 patients with hemosiderosis 14 died either from liver failure or concomitant sepsis. Female patients and those who received long-term dialysis had higher susceptibility for developing hemosiderosis. Of the 6 patients treated with phlebotomies, the response was good in 4 and incomplete in 2. Hemosiderosis and hemochromatosis should be considered in the differential diagnosis of posttransplant liver disease. Intermittent phlebotomies if carried out early may prevent the progression of hemosiderosis to micronodular cirrhosis.
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PMID:Hemosiderosis and hemochromatosis in renal transplant recipients. Clinical and pathological features, diagnostic correlations, predisposing factors, and treatment. 390 17

The patient was a 59-year-old man who had been in hospital suffering from aplastic anemia with transfusion hemosiderosis. Sudden onset of weakness, shaking chills and headache was observed after his staying out overnight on July 25, 1981. His temperature was 39.3 degrees C and he complained of abdominal pain and abdominal distension. His blood pressure dropped to a dangerous level and tonic convulsions that had begun in the upper body gradually extended to the whole body and he died 23 hours after his return. V. vulnificus was isolated by the blood culture performed before death. During his stay away from the hospital, he had eaten raw cuttlefish, which was considered to be the source of infection. V. vulnificus is one of the halophilic marine vibrios and is isolated frequently in summertime from the sea foods and sea water near Japan. It has been disclosed that the presence of underlying diseases such as liver cirrhosis, hemochromatosis can predispose a person to fatal sepsis by V. vulnificus. In this case, besides leukocytopenia, the presence of hemosiderosis induced by many transfusions was considered to be a major cause leading to the fulminating course of the disease.
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PMID:[Fatal Vibrio vulnificus infection in a patient with aplastic anemia]. 667 24

In clinical studies, frequent hepatic dysfunction associated with crises in sickle cell disease has been noted, but whether irreversible morphologic changes arise from these transient episodes is uncertain. We studied 70 patients with sickle cell disease (57 SS, 12 SC and one S-thalassemia (S-thal) hemoglobin) autopsied at The Johns Hopkins Hospital. They ranged in age from five months to 75 years (average 21 years) and 35 (50 percent) were female, In 64 patients (91 percent), livers were enlarged and had distention of Kupffer cells with phagocytized sickled red cells; this was massive in 10. In 19 patients (27 percent) the sinusoids were markedly distended with sickled red cells and appeared obstructed. Focal parenchymal necroses were present in 24 patients (34 percent) and were explained in 12, eight by cardiac dysfunction and four by sepsis. Reparative changes, portal fibrosis and regenerative nodules were each found in 14 patients (20 percent), only one of whom had a known history of viral hepatitis despite the frequency of transfusions. Cirrhosis of unknown cause was present in seven patients and cardiac cirrhosis in one. Cirrhosis with hemochromatosis was present in three patients and 30 others had parenchymal iron accumulation. Thus, unexplained hepatic necroses, portal fibrosis, regenerative nodules and cirrhosis were frequently encountered in these patients. This spectrum of liver disease appears to be best understood as a consequence of recurrent vascular obstruction, necrosis and repair arising as a component of sickle cell disease.
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PMID:The liver in sickle cell disease. A clinicopathologic study of 70 patients. 744 49

In convalescents after and in patients with sepsis, purulent meningoencephalitis, severe pneumonia the study of iron metabolism provided biochemical criteria of iron excess: low serum transferrin against high transferrin iron, elevated ferritin. The risk of hyperferremia rises considerably after blood or erythrocyte transfusions. The liver got affected in the presence of infectious toxicosis. The authors believe it risky to practice uncontrolled administration of iron preparations in subjects recovering from severe bacterial and inflammatory diseases in view of threatening hemochromatosis.
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PMID:[The iron overload syndrome in patients with severe bacterial inflammatory diseases and convalescents]. 802 Jul 29

Vibrio vulnificus is a halophilic Vibrio that has been isolated repeatedly from sea-water and shellfish during the warm months of the year. It's a virulent pathogen for men and is frequently associated with overwhelming infections including sepsis, gangrene of extremities and high mortality rate. We encountered a 13-year-old boy who had a history of beta-thalassemia major with secondary hemochromatosis, suffering from vomiting, diarrhea, fever and hypotension. Physical examination revealed that ecchymosis, bullae and ulceration were noted over the left leg. Vibrio vulnificus was isolated from the blood. Initially, the patient did not respond to the appropriate antibiotics treatment, subsequently surgical debridement was performed. After that, the patient recovered gradually, and discharged home after 17 days of admission. In conclusion, when patients present with sepsis and/or characteristic skin lesion-hemorrhagic bullae, particularly those with thalassemia major, hemochromatosis or underlying liver disease and a history of marine exposure, clinicians should be alerted to this potentially fatal infection and should commence appropriate assessment and treatment immediately.
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PMID:[Beta-thalassemia major complicated with Vibrio vulnificus septicemia: report of one case]. 817 48

Hepatic associated metabolic disorders represent 5% of the indications for orthotopic liver transplantation (OLTX) according to the European Liver Transplant Registry. We studied the outcome of this group at our institution after OLTX and combined liver/kidney transplantation. Between September 1988 and January 1997, 837 OLTXs were performed in 735 patients. Patient survival and graft function at 1 yr were 91.3 and 86%, respectively. Thirty-nine OLTXs were performed in 38 patients (15 female/23 male, median age +/- SD: 35 +/- 14 yr, range 4-60 yr) due to liver associated metabolic disorders (4.7%). Indications included Wilson's disease (n = 14), alpha-1-anti-trypsin-deficiency (n = 7), hemochromatosis (n = 4), erythropoetic protoporphyria (n = 4), cystic fibrosis (n = 2), Crigler-Najjar syndrome type I (n = 1), glycogenosis type I (n = 1), ornithine-transcarbomylase-deficiency (n = 1). In addition 4 patients suffering from primary hyperoxaluria type I received combined liver/kidney grafts. Survival rate the 1 yr after OLTX and combined OLTX/NTX was 91.8%. Twenty patients received cyclosporin A (55%) and 17 patients tacrolimus (45%) as primary immunosuppression. The mean follow-up was 28.6 months (range 4-73 months). Two patients with hemochromatosis died 1 and 3 months after OLTX, respectively, from Aspergillus sepsis followed by multiorgan-failure. One patient died of malignant lymphoma 5 months after transplantation. One patient required retransplantation 2 months after OLTX following arterial thrombosis and ischemic type biliary lesion. One year after OLTX, all patients demonstrated good graft function, liver grafts (ALT 17.9 +/- 13.6 IU/L, bilirubin 0.8 +/- 0.3.mg/dl, thromboplastin time 94 +/- 15%), and combined liver/kidney grafts (creatinine 2.4 +/- 1.4 mg/dl). OLTX, respectively combined OLTX/NTX, represent a successful therapy for hepatic associated metabolic disorders. Survival rates and graft function are similar to those in liver graft recipients for established indications at our institution. OLTX seems to be an excellent treatment for hepatic based therapy resistant neurological disorders.
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PMID:Orthotopic liver transplantation for hepatic associated metabolic disorders. 964 15


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