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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Infections with Vibrio vulnificus resulting in septicemia and high mortality have been correlated with pre-existing liver disease and hemochromatosis. As these conditions are associated with impaired iron metabolism and as iron availability in the host has been implicated in the pathogenicity of a number of bacterial infections, the role of iron as a possible factor in the pathogenesis of V. vulnificus was examined. Injection of mice with iron resulted in a lowering of the 50% lethal dose from 10(6) to 1.1 cells and in a reduction in the time of death postinfection. Elevated serum iron levels were also produced by damaging livers with injections of CCl4. The inoculum size required to kill these mice was directly correlated with serum iron levels. Since the portal of infection of this organism may be ingestion of contaminated seafood, the effects of iron upon orally induced infection were also studied. The effects of adding iron, transferrin, or Desferal (an iron chelate) upon the growth of V. vulnificus in human and rabbit sera were also examined. Iron appeared to be the limiting factor in the ability of this organism to survive or grow in mammalian sera. These results, both in vitro and in vivo, provided strong evidence that iron may play a major role in the pathogenesis of V. vulnificus.
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PMID:Role of iron in the pathogenesis of Vibrio vulnificus infections. 730 36

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

Rhinocerebral phycomycosis is an uncommon opportunistic infection with ubiquitous fungi of the class Phycomycetes, starting in the nose and extending to the paranasal sinuses and then intracranially. The condition is often characterized by poor prognosis because of occlusion of the internal carotid artery. This disease is commonly associated with predispositions such as uncontrolled diabetes mellitus, which is the most common, immunosuppressive states and metabolic bankruptcy including leukemia, lymphoma, myeloma, malnutrition, uremic or diarrheal acidosis, severe burns, anemia, carcinoma, radiotherapy, liver cirrhosis, hemochromatosis, tuberculosis, septicemia, long-term medication of steroid, antibiotics and antimetabolite, drug addiction, cytotoxic drug administration and AIDS. Cases with unknown predisposition, however, have been infrequently reported in the literature. The authors report a case of rhinocerebral phycomycosis in which concurrence of Candida species instead of the above-mentioned common predispositions was considered a potential predisposition. To our knowledge, only 1 report in which Candida species are referred to as a potential predisposition for this disease has been previously issued. A 85-year-old man was admitted to our hospital on March 2, 1994 because of generalized convulsion. He had received a total extirpation of an ascending colon cancer in July 1993. On admission, physical inspection showed no abnormalities and neurological examination revealed obtunded consciousness without other abnormalities. He had no diabetes mellitus. Hematological and blood chemistry values were normal except for CA19-9 of 45 U/ml.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of rhinocerebral phycomycosis]. 760 36

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

Yersinia enterocolitica is a gram-negative bacillus that thrives in conditions associated with iron overload. We describe an unusual case of a diabetic patient with a previously unrecognized hemochromatosis presenting with Y. enterocolitica septicemia. He was admitted because of a 10 day history of abdominal pain, fever and jaundice. Blood cultures grew Y. enterocolitica. The abdomen CT scan showed multiple liver and splenic abscesses. Antibiotic treatment with ciprofloxacin (2 months) resulted in a good clinical response. Serum iron studies showed iron overload. Liver biopsy revealed moderate fibrosis and early cirrhosis with large amounts of hemosiderin granules deposited in hepatocytes and bile duct epithelium. This report reviews the literature and highlights that iron overload must be ruled out in Yersinia septicemia patients.
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PMID:[Multiple hepatosplenic abscesses caused by Yersinia enterocolitica in a patient with hemochromatosis]. 956 96

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

Recent advances in the diagnosis and treatment of inborn errors of metabolism have improved substantially the prognosis for many of these conditions. This makes it essential that the practicing pediatrician be familiar with the clinical presentation of these disorders. A practical clinical approach to the recognition of inborn errors of metabolism in the young infant is presented in this review. Indications for specific laboratory studies are discussed. Guidelines are provided for the stabilization and emergency treatment of critically ill infants. This approach will identify those infants who will benefit from additional evaluation and specific treatment. Many of the inborn errors of metabolism, including urea cycle defects, organic acidemias, and certain disorders of amino acid metabolism, present in the young infant with symptoms of an acute or chronic metabolic encephalopathy. Typical symptoms include lethargy, poor feeding, apnea or tachypnea, and recurrent vomiting. Metabolic acidosis and/or hyperammonemia are observed in many of these conditions, but there are notable exceptions, including nonketotic hyperglycinemia and molybdenum co-factor deficiency. Therefore, appropriate laboratory testing for metabolic disorders should be performed in any infant who exhibits these findings. Although sepsis may be the initial consideration in a neonate with these symptoms, inborn errors of metabolism should always be in the differential diagnosis, particularly in a full-term infant with no specific risk factors. Hypoglycemia may be the predominant finding in a number of inborn errors of metabolism, including glycogen storage disorders, defects in gluconeogenesis, and fatty acid oxidation defects. The latter disorders, among the most common encountered, exhibit marked clinical variability and also may present as a sudden death, a Reye's-like episode, or a cardiomyopathy. Jaundice or other evidence of hepatic dysfunction is the mode of presentation of another important group of inborn errors of metabolism including galactosemia, hereditary tyrosinemia, neonatal hemochromatosis, and a number of other conditions. A subset of lysosomal storage disorders may present very early with coarse facial features, organomegaly, or even hydrops fetalis. Specific patterns of dysmorphic features and congenital anomalies characterize yet another group of inherited metabolic disorders, such as Zellweger syndrome and the Smith-Lemli-Opitz syndrome. Each of these symptom complexes, and the appropriate evaluation of the affected infants, is discussed in more detail in this review.
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PMID:Inborn errors of metabolism in infancy: a guide to diagnosis. 983 97

Two patients with hemophagocytic lymphohistiocytosis who presented with acute liver failure are reported. Both presented with fever, hepatosplenomegaly, markedly elevated liver function tests, abnormal coagulation profiles, and an increase in serum ferritin. Both infants were diagnosed with neonatal hemochromatosis based on a clinical picture of hepatic insufficiency with hyperferritinemia and were referred for liver transplantation. The first patient died of liver failure and septicemia before transplantation. Review of autopsy material revealed a hepatitis-like pattern and extensive infiltration of liver and other organs including bone marrow by histiocytes, some of which were hemophagocytic. The second patient underwent liver transplantation but died 44 days thereafter from progressive hemophagocytic lymphohistiocytosis. Examination of the resected liver demonstrated a hepatitis-like pattern, proliferation of histiocytes, and hemophagocytosis, and the bone marrow revealed hemophagocytic histiocytosis. Hemophagocytosis recurred in the allograft. Hepatic manifestations are common in hemophagocytic lymphohistiocytosis and overt hepatic failure may occur, but initial presentation as fulminant hepatic failure is not well recognized. Elevated serum ferritin can make the distinction from neonatal hemochromatosis and other forms of neonatal liver failure difficult. Hemophagocytic lymphohistiocytosis should be considered in the differential diagnosis of neonatal liver disease, especially when it is accompanied by cytopenias.
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PMID:Hemophagocytic syndrome presenting as acute hepatic failure in two infants: clinical overlap with neonatal hemochromatosis. 1034 80

Neonatal hemochromatosis (NH) is a rare clinical-pathologic entity defined by severe neonatal liver failure (NLF) of intrauterine onset associated with extrahepatic siderosis that spares reticuloendothelial elements. Whether this entity is the result of a distinct disease process or is the pathologic end-result of many other forms of NLF remains to be assessed. Death from multisystem organ failure usually occurs in the first few days or weeks of life. We report two sisters with neonatal hemochromatosis with different outcome. The first died at 21 days of life for multiorgan failure and sepsis. The second was diagnosed and treated very early by aggressive support with fresh-frozen plasma, packed red cells, platelets, coagulation factors, diuretics, and pressors, and she is well at 2-year-follow-up. The few therapeutic options are discussed. In general, few patients survived NH. Among patients treated only by medical approachs and that did not undergo orthotopic liver transplantation (OLTX), favorable outcome is only very rarely reported. For management of NH infants early recognition of the disease is critical. Our experience seems to suggest that as early is the start of treatment as high is the possibility of survival. At this moment very early aggressive supportive treatment should appear to be the most useful approach to stabilize the patient hoping in spontaneous liver recovery, or, in turn, to permit OLTX before septic complications bring to clinical decline.
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PMID:[Neonatal hemochromatosis: significance of early identification]. 1142 40


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