The disease was so named because the patients all had myoclonus epilepsy and mitochondrialdysfunction wasfound uponmuscle biopsy2. Pathology outlines waterhousefriderichsen syndrome. About europe pmc funders joining europe pmc governance roadmap outreach. When caused by neisseria meningitidis, wfs is considered the most severe form of meningococcal sepsis. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. Despite the predominant association with meningococcal infection, there are numerous other. The importance of the postmortem interval for the diagnosis of. The adrenal glands produce and release different hormones that the body. Waterhousefriderichsen syndrome wfs was reported by waterhouse in 1911 1. Neisseria meningitidis induced fatal waterhousefriderichsen. An autopsy case of pneumococcal waterhousefriderichsen.
Waterhousefriderichsen syndrome wfs is a catastrophic sepsis. Facebook is showing information to help you better understand the purpose of a page. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Waterhousefriderichsen syndrome information mount sinai. Waterhouse friderichsen syndrome is seen most frequently in patients with asplenia, and. Adrenal gland and paraganglia waterhousefriderichsen syndrome. An experimental model that produces adrenal cortical hemorrhage with endotoxin has been described. When should merrf myoclonus epilepsy associated with ragged. Muerte subita por sepsis meningococica scielo espana. When should merrf myoclonus epilepsy associated with. The bacterial infection leads to massive bleeding into one or usually both adrenal glands. Fatal purpura fulminans and waterhousefriderichsen. Waterhousefriderichsen syndrome as a result of non.
In the uk, it is most frequently seen as a result of meningococcal infection, 2 and at necropsy often leads to a presumptive diagnosis of meningococcal septicaemia. Waterhouse friderichsen syndrome wfs is a group of symptoms resulting from the failure of the adrenal glands to function normally as a result of bleeding into the gland. The disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little1 in 1901. Waterhousefriderichsen, meningococo, shock septico. The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Definition waterhousefriderichsen syndrome wfs or hemorrhagic adrenalitis or fulminant meningococcemia, is defined as adrenal gland failure due to bleeding into the adrenal glands, caused by severe bacterial infection most commonly the meningococcus neisseria meningitidis. Waterhouse friderichsen syndrome is named after rupert waterhouse 18731958, an english physician, and carl friderichsen 18861979, a danish pediatrician, who wrote papers on the syndrome, which had been previously described.
Waterhouse friderichsen syndrome is typically caused by neisseria meningitidis, but has been reported in sepsis from streptococcus pneumoniae, neisseria gonorrhoeae, pseudomonas aeruginosa, escherichia coli, haemophilus influenzae, and staphylococcus aureus. Heyde syndromethe link between aortic stenosis and gastrointestinal bleeding. Adrenal gland and paraganglia waterhouse friderichsen syndrome. Fatal purpura fulminans and waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome radiology reference. Waterhousefriderichsen syndrome wfs is a group of symptoms resulting from the failure of the adrenal glands to function normally as a result of bleeding into the gland. See actions taken by the people who manage and post content. Sepsis in these patients is typically secondary to encapsulated bacteria, with streptococcus pneumoniae being the most frequent pathogen. An autopsy case of pneumococcal waterhousefriderichsen syndrome with. It is characterized by an altered perception of re a l i t y, emotional disturbances, and crises of panic and anxiety with somatization. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Waterhouse friderichsen syndromemassive adrenal haemorrhage in the setting of overwhelming clinical sepsisis usually taken at necropsy to indicate meningococcal infection, and may be the only. Pdf tribute to the centenary of waterhousefriderichsen.
Waterhousefriderichsen syndrome, meningococcal, septic shock. Waterhouse friderichsen syndrome can be caused by a number of different organisms see below. First described in 1894 by arthur francis voelcker 18611946 and in 1901 by the british dermatologist ernest gordon graham little 18671950. Dostoevsky and stendhals syndrome edson jose amancio1 abstract stendhals syndrome occurs among travelers when they encounter a work of art of great beaut y. Fatal pneumococcal sepsis with waterhousefriderichsen. Here we report a fatal case of this syndrome due to neisseria. Universita degli studi di milano, facolta di medicina e chirurgia, sezione. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage waterhousefriderichsen syndrome. Waterhouse friderichsen syndrome also known as purpura fulminans 9 or hemorrhagic adrenalitis 10 is characterized by adrenal insufficiency that results from atraumatic adrenal hemorrhage in consequence of septicemia.