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

A fatal case of Waterhouse-Friderichsen syndrome resulting from infection in a previously healthy 74-year-old woman is reported. The patient died suddenly within 14 hours after presentation. The diagnosis of Waterhouse-Friderichsen syndrome as the cause of death was established post mortem based on autopsy findings, microscopic examination, measurement of serum procalcitonin concentration (113 ng/ml), and outcome of postmortem bacteriologic cultures that grew in heart and spleen blood samples. Since the introduction of as a new group in the family in 1983, more recent case studies have established its clinical significance and pathogenic potential to cause severe, life-threatening bacteremia and sepsis. is a rare pathogen that should be added to the list of unusual bacteria causing Waterhouse-Friderichsen syndrome.
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PMID:Fatal Waterhouse-Friderichsen syndrome due to Ewingella americana infection. 1260 97

An experimental model that produces adrenal cortical hemorrhage with endotoxin has been described. When stimulated by thorotrast, endotoxin, or its tropic hormone (ACTH), the adrenal cortex is susceptible to the development of a hemorrhagic reaction during endotoxemia. The hemorrhagic reaction resembles that described in the Waterhouse-Friderichsen syndrome. A pathophysiologic mechanism for the occurrence of adrenal hemorrhage occurring during acute sepsis is presented. Increased metabolic activity associated with the production of corticosteroids seems to make the adrenal cortex susceptible to endotoxin-induced hemorrhage. Adrenal hemorrhage observed during sepsis, as in the Waterhouse-Friderichsen syndrome, may be attributable to endotoxemia occurring during or shortly after stimulation of the adrenal cortex by infection. Significant differences between adrenal cortical hemorrhage and the Shwartzman phenomenon are described.
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PMID:ENDOTOXEMIA AND ADRENAL HEMORRHAGE. A MECHANISM FOR THE WATERHOUSE-FRIDERICHSEN SYNDROME. 1426 70

Waterhouse-Friderichsen syndrome--massive adrenal haemorrhage in the setting of overwhelming clinical sepsis--is usually taken at necropsy to indicate meningococcal infection, and may be the only evidence of this pathogen. This report describes three fatal cases of the syndrome in which the causative organism proved to be a streptococcus. The organisms were detected during routine coroners' autopsies with histology and microbiological investigations. In two cases, the syndrome followed Streptococcus pneumoniae infection and in a third beta haemolytic streptococcus group A. Thus, adrenal haemorrhage alone cannot be taken to indicate meningococcal disease and other pathogens, particularly streptococcus, must be considered.
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PMID:Waterhouse-Friderichsen syndrome as a result of non-meningococcal infection. 1474 54

Splenectomized patients are likely to suffer from severe infections, such as sepsis and meningitis. This syndrome is called overwhelming postsplenectomy infection (OPSI) in Europe and America. We present an adult case of OPSI syndrome, which occurred as respiratory insufficiency, and thrombocytopenia. The course is rapid, the clinical symptoms are serious, and the prognosis is very poor. Clinical examination showed cyanosis, mandibular hypertonia, psychomotor anxiety and purpura. Laboratory findings were thrombocytopenia, leukocytosis, hypoglycemia and altered coagulation parameters. A chest X-ray showed right pulmonary aspecific thickening. The autopsy findings occurred as Waterhouse-Friderichsen syndrome.
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PMID:OPSI (overwhelming postsplenectomy infection) syndrome: a case report. 1563 88

The aim of the study was to investigate invasive meningococcal disease in the North-West of Poland, associated with a case fatality rate of 42.9%, where among the first 11 cases, eight had fatal outcome. All fatal cases were diagnosed as fulminant meningococcal severe sepsis with Waterhouse-Friderichsen syndrome and disseminated intravascular coagulation. Serotyping and pulsed-field gel electrophoresis analysis revealed that the high case fatality rate was not associated with the dissemination of one epidemic clone. However, six cases, all with good outcomes, were caused by C:2b:(P1.2,P1.5) isolates of the same pulsed-field gel electrophoresis type belonging to ST8 complex/Cluster A4.
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PMID:Invasive meningococcal disease associated with a very high case fatality rate in the North-West of Poland. 1648 4

Overwhelming postsplenectomy infection (OPSI) syndrome is a rare condition, but is associated with high mortality. However, recognition and clinical management of OPSI is not well established. The prevalence of splenectomy increased recently because it was a clinically effective treatment for hepatitis C virus-associated thrombocytopenia before the introduction of the interferon/ribavirin combination therapy. We reviewed the literature characterizing the clinicopathological features of OPSI and assessed the most effective and feasible administration of the condition. A Medline search was performed using the keywords 'overwhelming', 'postsplenectomy infection', 'postsplenectomy sepsis', 'chronic liver disease', and/or 'splenectomy'. Additional articles were obtained from references within the papers identified by the Medline search. Durations between splenectomy and onset of OPSI ranged from less than 1 wk to more than 20 years. Autopsy showed that many patients with OPSI also had Waterhouse-Friderichsen syndrome. Although the mortality rate from OPSI has been reduced by appropriate vaccination and education, the precise pathogenesis and a suitable therapeutic strategy remain to be elucidated. Protein energy malnutrition (PEM) is commonly observed in cirrhotic patients. Since the immune response in patients with PEM is compromised, a more careful management for OPSI should therefore be applied for cirrhotic patients after splenectomy. In addition, strict long-term follow up of OPSI patients including informed consent will lead to a better prognosis.
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PMID:Overwhelming postsplenectomy infection syndrome in adults - a clinically preventable disease. 1818 51

An exceptional case of a 15-year-old puerpera with septic shock caused by Group A beta-hemolytic Streptococcus and Waterhouse-Friderichsen syndrome is reported. The findings were observed at the necropsy. The characteristics of the diagnosis, pathogenesis and evolution of this puerperal infection (sepsis), associated with adrenal hemorrhage and insufficiency are reviewed in this paper.
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PMID:[Puerperal septic shock due to beta-hemolytic Streptococcus and Waterhouse-Friderichsen syndrome]. 1928 40

Sudden and unexpected nontraumatic death in individuals with asplenia or hyposplenia is usually due to fulminant bacterial sepsis, most often involving Streptococcus pneumoniae, Neisseria meningitidis, and Hemophilus influenzae. We report a case of a previously well 40-year-old man who died 5 hours after hospital admission. At autopsy Waterhouse-Friderichsen syndrome was identified and Capnocytophaga canimorsus was subsequently isolated on antemortem blood cultures. Infection of humans with this organism is most often due to dog bite or contact. Upon specific inquiry it was ascertained that 2 days before admission the deceased had suffered a superficial bite to his hand by his pet Staffordshire Bullterrier dog. His relevant history included a previous splenectomy following blunt abdominal trauma. Asplenia and hyposplenia at autopsy should prompt microbiological testing with consideration of unusual organisms such as C. canimorsus. Although histories of animal contact or injury are often not available at the time of autopsy, this should also be considered in cases of apparent fulminant sepsis. In individuals with asplenia or hyposplenia, dog bites do not have to involve excessive tissue trauma, vascular compromise, or blood loss to be lethal.
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PMID:Fatal dog bite in the absence of significant trauma: Capnocytophaga canimorsus infection and unexpected death. 2040 57

Asplenia may refer to the spleen's surgical removal, functional impairment, or congenital absence. It is a risk factor for the development of severe bacterial infection. Functional asplenia is likely the most common presentation of this entity and has many etiologies. Those that are previously undiagnosed may present completely well until an episode of overt sepsis develops. The true incidence of mortality secondary to functional asplenia remains elusive. As lifetime mortality remains exceedingly high in the asplenic population regardless of etiology, markers of hyposplenism are important to detect. The present report describes an infant with trisomy 21 and previously undiagnosed functional asplenia who ultimately experienced overwhelming pneumococcal sepsis with features of Waterhouse-Friderichsen syndrome and died within 12 hours of initial presentation. It is a poignant reminder of what features to be cognizant of on peripheral blood smear in a previously well child, who may be at risk for a devastating consequence.
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PMID:A case of functional asplenia and pneumococcal sepsis. 2173 Aug

We report an autopsy case of rapid progressive Waterhouse-Friderichsen syndrome (WFS) associated with Streptococcus pneumonia infection in a previously healthy man. Although he once visited a hospital about 6 hours before death, the both physical and serological examination did not show any sign of overwhelming infection. Autopsy showed massive adrenal hemorrhage without inflammation, and showed proliferation of gram positive cocci and microthrombosis in the vessels of many organs. The pathological change of respiratory tract was extremely minimal. Size and weight of the spleen possible decreased than normal. However, histological examination showed that obscuration of germinal center and decreasing the immunological cells of mantle and marginal zone. Immunohisitochemically, marked decreasing the marginal zone macrophages, which are positive for specific intercellular adhesion molecule grabbing nonintegrin receptor-1 (SIGN-R1) and macrophage receptor with collagenous structure (MARCO), were decreased comparing with age-matched control case. Polymerase chain reaction (PCR) assay using each DNA, extraction from formalin-fixed paraffin-embedded specimen (FFPE) samples of lung, adrenal gland, heart, spleen, and kidney showed positive the ply gene and the lytA gene specific for Streptococcus pneumonia. Present case showed possible acquired atrophy of spleen, especially decreasing marginal zone macrophage may correlate with rapid progression of sepsis of Streptococcus pneumonia with massive adrenal hemorrhage. In addition, present case showed the usefulness of PCR using FFPE for the postmortem diagnosis of WFS.
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PMID:An autopsy case of pneumococcal Waterhouse-Friderichsen syndrome with possible functional asplenia/hyposplenia. 2626 63


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