Waterhousefriderichsen syndrome definition of waterhouse. The waterhouse friderichsen wf syndrome is a clinicopathological entity consisting of a the. 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. If you have problems viewing pdf files, download the latest version of adobe reader. Waterhousefriderichsen syndrome as a result of non. A case of a patient with the waterhouse friderichsen syndrome has been described. Department of internal medicine, general hospital 71. Waterhouse friderichsen syndrome annals of internal. Waterhousefriderichsen, meningococo, shock septico, waterhousefriderichsen syndrome, meningococcal, septic shock, lcc.
A free powerpoint ppt presentation displayed as a flash slide show on id. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. Waterhousefriderichsen syndrome an overview sciencedirect. Fatal purpura fulminans and waterhousefriderichsen syndrome. Waterhouse friderichsen syndrome can be caused by a number of different organisms see below. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding. Waterhousefriderichsen syndrome genetic and rare diseases. Disseminated intravascular coagulation and the waterhouse. 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. She had had posttransfusion hepatitis 18 years before, and chronic hepatitis was diagnosed 5 years before admission but not treated. Adrenal gland and paraganglia waterhouse friderichsen syndrome more common in children, particularly before age 2 years usually bilateral. 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. The disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little1 in 1901. Download as ppt, pdf, txt or read online from scribd.
The bacterial infection leads to massive bleeding into one or usually both adrenal glands. 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. Fatal pneumococcal sepsis with waterhousefriderichsen syndrome. Massive adrenal haemorrhage waterhousefriderichsen syndrome 1 is an uncommon, but usually fatal, consequence of overwhelming sepsis. 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. Seventyfour such cases have been described in the literature. Symptoms include acute adrenal gland insufficiency, and profound shock.
Waterhousefriderichsen syndrome can be caused by a number of different. Ppt sindrome carcinoide powerpoint presentation free. Files are available under licenses specified on their description page. Adrenal gland and paraganglia waterhousefriderichsen syndrome more common in children, particularly before age 2 years usually bilateral.
The presenting clinical picture is frequently pharyngitis, fever, and. Infectious causes of the waterhousefriderichsen syndromea. Our case describes a previously healthy young woman, statuspost splenectomy 15 years prior, who presented to our hospital after only a few hours of symptoms, and then rapidly declined, resulting in death within 12 hours of symptoms onset. Pathology outlines waterhousefriderichsen syndrome.
All structured data from the file and property namespaces is available under the creative commons cc0 license. The merrf diagnostic criteria described in 1980 by fukuhara et al included as typical manifestations of the dis. Jump to navigation jump to search this is an alphabeticallysorted. 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. Neisseria meningitidis cause a fulminating fatal infection, frequently with few or no signs or preconditions.
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. Sepsis in the asplenic host can be fulminant and rapidly progressive, with mortality rates approaching 50%. The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Fatal pneumococcal sepsis with waterhousefriderichsen. This case on autopsy showed massive bilateral hemorrhage into the adrenal glands. The waterhousefriderichsen syndrome wfs, also known as purpura fulminans, is a po. The disease known as the waterhousefriderichsen syndrome was described as an entity by graham little1 in 1901. Pdf waterhousefriderichsen syndrome is one of the fatal. 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.
Enfermedades infecciosas enfermedades infecciosas enfermedades infecciosas. The adrenal glands produce and release different hormones that the body. She had confluent petechiae, ecchymosis, and purpuric lesions on her face. First described in 1894 by arthur francis voelcker 18611946 and in 1901 by the british dermatologist ernest gordon graham little 18671950. Waterhouse friderichsen syndrome is adrenal gland failure due to bleeding into the adrenal gland. There is bleeding into the adrenal glands causing adrenal failure and shock. 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. An experimental model that produces adrenal cortical hemorrhage with endotoxin has been described. First case reported in adults waterhousefriderichsen syndrome secondary to staphylococcus haemolyticus is concluded. One is stunned by the rapid and usually fatal progression of this clinical picture. The importance of the postmortem interval for the diagnosis of waterhouse friderichsen syndrome by neisseria meningitidis in a series of forensic cases.
The journal is mainly focused on clinical practice and scientific communication, targeted to internists and other medical specialists. Fulminating meningococcemia with bilateral adrenal. Meningococcemia initial description of the syndrome. 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. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Download as pptx, pdf, txt or read online from scribd. 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. Pdf waterhousefriderichsen syndrome in an adult patient with. Waterhouse friderichsen syndrome infectious disease a clinical crisis characterized by acute adrenal insufficiency, often accompanied by septicemia. Septic shock caused by waterhousefriderichsen syndrome. Pdf fatal pneumococcal sepsis with waterhousefriderichsen. When should merrf myoclonus epilepsy associated with ragged. Waterhousefriderichsen syndrome a rare disorder caused by invasion of the blood by meningococcus organisms often in the course of an attack of meningitis. Facebook is showing information to help you better understand the purpose of a page.
First case reported in adults waterhouse friderichsen syndrome secondary to staphylococcus haemolyticus is concluded. Here we report a fatal case of this syndrome due to neisseria. The disease was so named because the patients all had myoclonus epilepsy and mitochondrialdysfunction wasfound uponmuscle biopsy2. Waterhousefriderichsen syndrome radiology reference. It is usually caused by severe meningococcal infection or other severe, bacterial infection. When caused by neisseria meningitidis, wfs is considered the most severe form of meningococcal sepsis. See actions taken by the people who manage and post content. For language access assistance, contact the ncats public information officer. Most patients with this condition are children, although adults may rarely be affected. This report describes three fatal cases of the syndrome in which the causative organism proved to be a streptococcus. All structured data from the file and property namespaces is available under the creative. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for waterhousefriderichsen.
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