The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Most patients with this condition are children, although adults may rarely be affected. Fatal purpura fulminans and waterhousefriderichsen syndrome. Full text get a printable copy pdf file of the complete article 1. The adrenal glands produce and release different hormones that the body. A unique fatal case of waterhousefriderichsen syndrome caus. Waterhousefriderichsen syndrome, cir rhosis, pasteurella multocida. 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. Doenca dos legionarios wikipedia, a enciclopedia livre. Septic shock caused by waterhousefriderichsen syndrome. 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.
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. Waterhouse friderichsen syndrome waterhousefriderichsen. Sepsis in these patients is typically secondary to encapsulated bacteria, with streptococcus pneumoniae being the most frequent pathogen. One is stunned by the rapid and usually fatal progression of this clinical picture. Waterhousefriderichsen syndrome caused by haemophilus. The waterhousefriderichsen syndrome wfs, also known as purpura fulminans, is a potentially lethal condition described as acute hemorrhagic necrosis of. A case of a patient with the waterhouse friderichsen syndrome has been described. Pdf waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Neisseria meningitidis induced fatal waterhousefriderichsen. This report describes three fatal cases of the syndrome in which the causative organism proved to be a streptococcus. This may not be the complete list of references from this article. While waterhouse friderichsen syndrome is characterized mainly by acute, disseminated intravascular clotting with subsequent consumptioncoagulopathy, this. The information regarding adrenal insufficiency is in conflict with several sources.
Waterhousefriderichsen syndrome europe pmc article. The importance of the postmortem interval for the diagnosis of waterhouse friderichsen syndrome by neisseria meningitidis in a series of forensic cases. The disease was so named because the patients all had myoclonus epilepsy and mitochondrialdysfunction wasfound uponmuscle biopsy2. When caused by neisseria meningitidis, wfs is considered the most severe form of meningococcal sepsis. If you have problems viewing pdf files, download the latest version of adobe reader. Nov 03, 1979 full text is available as a scanned copy of the original print version. May 20, 2019 neisseria meningitidis, serogroup w, meningococcal infections, acute bacterial meningitis, meningococcal meningitis, purpurafulminans, waterhousefriderichsen syndrome. Files are available under licenses specified on their description page. Sindrome waterhouse friderichsen pdf download 4sq53. Fatal pneumococcal sepsis with waterhousefriderichsen. She had confluent petechiae, ecchymosis, and purpuric lesions on her face. Waterhouse friderichsen syndrome must be differentiated from severe meningococcal septicaemia by clinical, haematological and bloodcoagulation analysis. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for waterhousefriderichsen.
Waterhousefriderichsen syndrome can be caused by a number of different. The condition is most frequently seen in children younger than 10 years old. The presenting clinical picture is frequently pharyngitis, fever, and. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage waterhouse friderichsen syndrome. Waterhousefriderichsen syndrome an overview sciencedirect. Waterhouse in 1911 as a case of suprarenal apoplexy 1 and by c. 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 waterhousefriderichsen syndrome. Massive adrenal haemorrhage waterhousefriderichsen syndrome 1 is an uncommon, but usually fatal, consequence of overwhelming sepsis. Waterhousefriderichsen syndrome radiology reference. She had had posttransfusion hepatitis 18 years before, and chronic hepatitis was diagnosed 5 years before admission but not treated. Waterhouse friderichsen syndrome is seen most frequently in patients with asplenia, and.
Waterhouse in 1911 as a case of suprarenal apoplexy1 and by c. The waterhouse friderichsen syndrome is a clinical entity first described in 1911 by waterhouse 1 in an eightmonthold child. When should merrf myoclonus epilepsy associated with ragged. The disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little1 in 1901. Waterhouse friderichsen syndrome is adrenal gland failure due to bleeding into the adrenal gland. Waterhousefriderichsen syndrome as a result of non.
Seventyfour such cases have been described in the literature. The shock, purpura and intravascular clotting are probably the result of an endotoxin mediated immune. First described in 1894 by arthur francis voelcker 18611946 and in 1901 by the british dermatologist ernest gordon graham little 18671950. However, because her clinical presentation suggested meningococcemia, a prophylactic regimen of rifampin was prescribed for infants, toddlers, and staff at the ccc she attended. Waterhouse friderichsen syndrome annals of internal. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Disseminated intravascular coagulation and the waterhouse.
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. Waterhousefriderichsen syndrome genetic and rare diseases. Waterhousefriderichsen syndrome after infection with group a. 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. 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. I am 31 years old and have had severe yeast infection for over 8 years. The merrf diagnostic criteria described in 1980 by fukuhara et al included as typical manifestations of the dis. 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. Asplenic patients are at increased risk for sepsis and fulminant infection. Fulminating meningococcemia with bilateral adrenal. Neisseria meningitidis cause a fulminating fatal infection, frequently with few or no signs or preconditions.
Waterhousefriderichsen syndrome journal of clinical pathology. 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. Get a printable copy pdf file of the complete article 221k, or click on a page image below to browse page by page. 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. For language access assistance, contact the ncats public information officer. The waterhouse friderichsen syndrome was described by the british physician r. Purpura fulminans rarely occurs in adults but is seen in patients with meningococcal infections, which have a high mortality rate. The waterhousefriderichsen syndrome was described by the british physician r.
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. Here we report a fatal case of this syndrome due to neisseria. Fatal purpura fulminans and waterhousefriderichsen. The bacterial infection leads to massive bleeding into one or usually both adrenal glands. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. On february 9, 1994, a 7monthold infant patient 2 who attended the same ccc died from septic shock, purpura, and waterhouse friderichsen syndrome. Sindrome waterhouse friderichsen pdf download, 74309d72. Pdf waterhousefriderichsen syndrome in an adult patient with. 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 can be caused by a number of different organisms see below. Waterhousefriderichsen syndrome, also known as hemorrhagic adrenalitis, is a condition that describes acute adrenal failure, caused by hemorrhage within the adrenal glands after a severe bacterial infection, in particular meningococcal disease. It is usually caused by severe meningococcal infection or other severe, bacterial infection. The most common cause of acute adrenal insufficiency is the socalled waterhousefriderichsen syndrome, which can develop in patients with meningococcal.
Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. We report a case of waterhousefriderichsen syndrome associated with group a streptococcus gas toxic shock syndrome in a previously healthy man. Links to pubmed are also available for selected references. Symptoms include acute adrenal gland insufficiency, and profound shock. 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 defined as adrenal gland failure due to bleeding. Jump to navigation jump to search this is an alphabeticallysorted. 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. Neisseria meningitidis and purpura fulminans in a woman.
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