Waterhousefriderichsen syndrome caused by haemophilus. Get a printable copy pdf file of the complete article 221k, or click on a page image below to browse page by page. 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 information mount sinai. This case on autopsy showed massive bilateral hemorrhage into the adrenal glands. O medicinanet e o maior portal medico em portugues. 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. Neisseria meningitidis cause a fulminating fatal infection, frequently with few or no signs or preconditions.
First described in 1894 by arthur francis voelcker 18611946 and in 1901 by the british dermatologist ernest gordon graham little 18671950. 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 syndrome can be caused by a number of different. Waterhouse friderichsen syndrome is seen most frequently in patients with asplenia, and. The waterhouse friderichsen syndrome is a clinical entity first described in 1911 by waterhouse 1 in an eightmonthold child. Waterhouse friderichsen secondaria to ful minant meningococemia abstract. Pathology outlines waterhousefriderichsen syndrome. 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. For language access assistance, contact the ncats public information officer. Lilacsmeningococcemia fulminante sindrome waterhouse. Sindrome di waterhousefriderichsen dizionario medico. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for waterhousefriderichsen.
Clinical characterization of cases with meningococcal disease by w5. Meningitis meningococcal meningitis freelance clinical microbiologist. 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. Here we report a fatal case of this syndrome due to neisseria. Waterhousefriderichsen syndrome as a result of non.
Most patients with this condition are children, although adults may rarely be affected. 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. An exceptional case of a 15yearold puerpera with septic shock caused by group a. Waterhousefriderichsen syndrome genetic and rare diseases. Waterhousefriderichsen syndrome is adrenal gland failure due to bleeding into the adrenal gland. 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. Adrenal gland and paraganglia waterhouse friderichsen syndrome more common in children, particularly before age 2 years usually bilateral. This report describes three fatal cases of the syndrome in which the causative organism proved to be a streptococcus. The presenting clinical picture is frequently pharyngitis, fever, and. Tribute to the centenary of waterhousefriderichsen syndrome.
E caratterizzata da insufficienza surrenalica secondaria associata a. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding. One is stunned by the rapid and usually fatal progression of this clinical picture.
Massive adrenal haemorrhage waterhousefriderichsen syndrome 1 is an uncommon, but usually fatal, consequence of overwhelming sepsis. Apr 03, 20 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. When caused by neisseria meningitidis, wfs is considered the most severe form of meningococcal sepsis. Files are available under licenses specified on their description page. Fatal purpura fulminans and waterhousefriderichsen syndrome. The adrenal glands produce and release different hormones that the body. 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. All structured data from the file and property namespaces is available under the creative commons cc0 license. Upon arrival even with persistent hypotension despite double.
Septic shock caused by neisseria meningitidis infection, defined as waterhouse friderichsen syndrome in 1946, has a high mortality rate of between 21% and 44%. The disease now known as the waterhouse friderichsen syndrome was recognized as an entity by little1 in 1901. 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. If you have problems viewing pdf files, download the latest version of adobe reader. 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. 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.
The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Symptoms include acute adrenal gland insufficiency, and profound shock. Waterhousefriderichsen, meningococo, shock septico. Septic shock caused by waterhousefriderichsen syndrome. Meningococcemia is a lifethreatening infection that occurs when the bacteria neisseria meningitidis invades the blood stream. The disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little1 in 1901. Waterhousefriderichsen syndrome radiology reference. Waterhouse friderichsen syndrome, meningococcal, septic shock. It is usually caused by severe meningococcal infection or other severe, bacterial infection. Muerte subita por sepsis meningococica scielo espana.