Purpura fulminans rarely occurs in adults but is seen in patients with meningococcal infections, which have a high mortality rate. Septic shock caused by neisseria meningitidis infection, defined as waterhouse friderichsen syndrome in 1946, has a high mortality rate of between 21% and 44%. Summary we present the case of a patient affected by a waterhouse friderichsen syndrome presenting multiple organ failure and data in relation to acute adrenal insuf. The adrenal glands produce and release different hormones that the body. 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. This case on autopsy showed massive bilateral hemorrhage into the adrenal glands. Septic shock caused by waterhousefriderichsen syndrome. May 20, 2019 neisseria meningitidis, serogroup w, meningococcal infections, acute bacterial meningitis, meningococcal meningitis, purpurafulminans, waterhousefriderichsen syndrome. Nov 03, 1979 full text is available as a scanned copy of the original print version. Fatal purpura fulminans and waterhousefriderichsen.
This may not be the complete list of references from this article. First reported as an entity by waterhouse in 1911, the syndrome received its present title following friderichsen s report in 1918 of a similar case with a fulminating course. Waterhousefriderichsen syndromemassive adrenal haemorrhage in the setting of overwhelming clinical sepsisis usually taken at necropsy to indicate meningococcal infection, and may be the only evidence of this pathogen. Waterhousefriderichsen syndrome an overview sciencedirect. Jump to navigation jump to search this is an alphabeticallysorted. 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.
For language access assistance, contact the ncats public information officer. Seventyfour such cases have been described in the literature. Waterhouse friderichsen syndrome annals of internal. 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. 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. Waterhouse friderichsen syndrome waterhousefriderichsen syndrome. She had confluent petechiae, ecchymosis, and purpuric lesions on her face. If you have problems viewing pdf files, download the latest version of adobe reader. Get a printable copy pdf file of the complete article 439k. The waterhouse friderichsen syndrome was described by the british physician r. Waterhousefriderichsen syndrome genetic and rare diseases.
The information regarding adrenal insufficiency is in conflict with several sources. Sindrome waterhouse friderichsen pdf download 4sq53. Waterhouse friderichsen syndrome is adrenal gland failure due to bleeding into the adrenal gland. Asplenic patients are at increased risk for sepsis and fulminant infection. Waterhousefriderichsen syndrome journal of clinical pathology.
The merrf diagnostic criteria described in 1980 by fukuhara et al included as typical manifestations of the dis. The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Waterhouse friderichsen syndrome is seen most frequently in patients with asplenia, and. Links to pubmed are also available for selected references. Neisseria meningitidis induced fatal waterhousefriderichsen. On february 9, 1994, a 7monthold infant patient 2 who attended the same ccc died from septic shock, purpura, and waterhouse friderichsen syndrome. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Waterhouse in 1911 as a case of suprarenal apoplexy1 and by c. Users can purchase an ebook on diskette or cd, but the most popular method of getting an ebook is to purchase a downloadable file of the ebook or other reading material from a web site such as barnes and noble to be read from the users computer or reading device. Fatal purpura fulminans and waterhousefriderichsen syndrome.
Disseminated intravascular coagulation and the waterhouse. Waterhousefriderichsen syndrome after infection with group a. A case of a patient with the waterhouse friderichsen syndrome has been 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. Waterhousefriderichsen syndrome can be caused by a number of different. Waterhousefriderichsen syndrome radiology reference. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for waterhousefriderichsen. Waterhousefriderichsen syndrome europe pmc article. Files are available under licenses specified on their description page. Waterhouse in 1911 as a case of suprarenal apoplexy 1 and by c. It is sometimes said that the hemorrhage in waterhouse friderichsen syndrome causes an acute adrenal insufficiency, but this is inaccurate, since blood cortisol levels are not decreased. Waterhousefriderichsen syndrome caused by haemophilus. Massive adrenal haemorrhage waterhousefriderichsen syndrome 1 is an uncommon, but usually fatal, consequence of overwhelming sepsis. Waterhouse friderichsen syndrome can be caused by a number of different organisms see below.
Sindrome waterhouse friderichsen pdf download, 74309d72. The disease was so named because the patients all had myoclonus epilepsy and mitochondrialdysfunction wasfound uponmuscle biopsy2. Waterhousefriderichsen syndrome, cir rhosis, pasteurella multocida. First described in 1894 by arthur francis voelcker 18611946 and in 1901 by the british dermatologist ernest gordon graham little 18671950. When should merrf myoclonus epilepsy associated with ragged. Waterhousefriderichsen syndrome as a result of non. It is usually caused by severe meningococcal infection or other severe, bacterial infection. Get a printable copy pdf file of the complete article 221k, or click on a page image below to browse page by page.
While waterhouse friderichsen syndrome is characterized mainly by acute, disseminated intravascular clotting with subsequent consumptioncoagulopathy, this. The waterhousefriderichsen syndrome wfs, also known as purpura fulminans, is a potentially lethal condition described as acute hemorrhagic necrosis of. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. The presenting clinical picture is frequently pharyngitis, fever, and. A unique fatal case of waterhousefriderichsen syndrome caus. Pdf waterhousefriderichsen syndrome in an adult patient with. Neisseria meningitidis cause a fulminating fatal infection, frequently with few or no signs or preconditions. Sepsis in these patients is typically secondary to encapsulated bacteria, with streptococcus pneumoniae being the most frequent pathogen. However, because her clinical presentation suggested meningococcemia, a prophylactic regimen of rifampin was prescribed for infants, toddlers, and staff at the ccc she attended. Doenca dos legionarios wikipedia, a enciclopedia livre.
Symptoms include acute adrenal gland insufficiency, and profound shock. Waterhouse friderichsen syndrome waterhouse friderichsen syndrome. Fulminating meningococcemia with bilateral adrenal. The waterhouse friderichsen syndrome is a clinical entity first described in 1911 by waterhouse 1 in an eightmonthold child. Pdf waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. I am 31 years old and have had severe yeast infection for over 8 years. She had had posttransfusion hepatitis 18 years before, and chronic hepatitis was diagnosed 5 years before admission but not treated.
The bacterial infection leads to massive bleeding into one or usually both adrenal glands. We report a case of waterhousefriderichsen syndrome associated with group a streptococcus gas toxic shock syndrome in a previously healthy man. Apr 03, 20 waterhousefriderichsen syndrome wfs by stacy arvinna binti jamarun group 3 6th year 2nd semester slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Full text get a printable copy pdf file of the complete article 1. 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. All structured data from the file and property namespaces is available under the creative commons cc0 license.
Waterhousefriderichsen 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. Here we report a fatal case of this syndrome due to neisseria. The waterhousefriderichsen syndrome was described by the british physician r. One is stunned by the rapid and usually fatal progression of this clinical picture. 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. Waterhouse friderichsen syndrome must be differentiated from severe meningococcal septicaemia by clinical, haematological and bloodcoagulation analysis. Neisseria meningitidis and purpura fulminans in a woman. The presence of dic in the waterhousefriderichsen syndrome suggests that heparin should be a logical form of treatment. Most patients with this condition are children, although adults may rarely be affected. Fatal pneumococcal sepsis with waterhousefriderichsen. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage waterhouse friderichsen syndrome.
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