A case of a patient with the waterhousefriderichsen syndrome has been described. Acute adrenal failure is usually due to septicaemia, especially meningococcal waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome radiology reference. Septic shock caused by neisseria meningitidis infection, defined as waterhousefriderichsen syndrome in 1946, has a high mortality rate of between 21% and 44%. Syndromes are the group of symptoms that collectively indicate or characterize a disease. The importance of the postmortem interval for the diagnosis of. Fatal pneumococcal sepsis with waterhousefriderichsen. Medical definition of waterhouse friderichsen syndrome. The disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little1 in 1901. Here we report a fatal case of this syndrome due to neisseria.
Sepsis in these patients is typically secondary to encapsulated bacteria, with streptococcus pneumoniae being the most frequent pathogen. Seventyfour such cases have been described in the literature. Waterhousefriderichsen, meningococo, shock septico, waterhousefriderichsen syndrome, meningococcal, septic shock, lcc. First case reported in adults waterhouse friderichsen syndrome secondary to staphylococcus haemolyticus is concluded. Pdf waterhousefriderichsen syndrome in an adult patient with. Medical definition of 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. The importance of the postmortem interval for the diagnosis of waterhousefriderichsen syndrome by neisseria meningitidis in a series of forensic cases. Septic shock caused by waterhousefriderichsen 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. She had had posttransfusion hepatitis 18 years before, and chronic hepatitis was diagnosed 5 years before admission but not treated. Waterhouse friderichsen syndrome, a rare type of septicemia blood poisoning of rapid and severe onset, marked by fever, collapse and sometimes coma, hemorrhage from skin and mucous membranes, and severe bilateral hemorrhage of the adrenal cortical tissue. 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.
Waterhouse friderichsen syndrome a rare disorder caused by invasion of the blood by meningococcus organisms often in the course of an attack of meningitis. The waterhouse friderichsen syndrome is the association of purpura fulminans with suprarenal necrosis and haemorrhage. Massive adrenal haemorrhage waterhousefriderichsen syndrome 1 is an uncommon, but usually fatal, consequence of overwhelming sepsis. Despite the predominant association with meningococcal. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Neisseria meningitidis cause a fulminating fatal infection, frequently with few or no signs or preconditions. The waterhousefriderichsen syndrome was described by the british physician r. Disseminated intravascular coagulation and the waterhouse. Waterhousefriderichsen syndrome an overview sciencedirect. This is an alphabeticallysorted list of medical syndromes. 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.
Waterhousefriderichsen syndrome must be differentiated from. Jump to navigation jump to search this is an alphabeticallysorted. Waterhousefriderichsen syndrome wfs is a catastrophic sepsis1 with a higher incidence in children,24 and is usually associated with adrenal gland hemorrhage, which is consideredalong with the disseminated intravascular coagulation dicas the most fearsome complication. Waterhousefriderichsen syndrome pathology britannica.
Waterhouse friderichsen syndrome annals of internal. Waterhousefriderichsen syndrome definition of waterhouse. Waterhouse in 1911 as a case of suprarenal apoplexy 1 and by c. Waterhousefriderichsen syndrome as a result of non. Bilateral adrenal glands showed evidence of extensive hemorrhage, consistent with the waterhousefriderichsen syndrome. Download as ppt, pdf, txt or read online from scribd. One is stunned by the rapid and usually fatal progression of this clinical picture. Skin histology was consistent with diffuse disseminated intravascular coagulation dic and purpura fulminans. First described in 1894 by arthur francis voelcker 18611946 and in 1901 by the british dermatologist ernest gordon graham little 18671950. Waterhousefriderichsen syndrome medical definition merriam. Files are available under licenses specified on their description page.
Waterhousefriderichsen syndrome journal of clinical pathology. She had confluent petechiae, ecchymosis, and purpuric lesions on her face. Waterhousefriderichsen syndrome medical definition. Neisseria meningitidis and purpura fulminans in a woman. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. Fulminating meningococcemia with bilateral adrenal hemorrhage. There is bleeding into the adrenal glands causing adrenal failure and shock. Pdf waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. When caused by neisseria meningitidis, wfs is considered the most severe form of meningococcal sepsis. 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. Fulminating meningococcemia with bilateral adrenal. Neisseria meningitidis is a rare but serious pathogen that leads to lifethreatening septic shock. Neisseria meningitidis and purpura fulminans in a woman with.
The adrenal glands produce and release different hormones that the body. The syndrome is most common in children under five and may last only a few hours. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding. Waterhousefriderichsen syndrome complicating primary biliary sepsis due to. Pdf first case reported in adults waterhousefriderichsen syndrome secondary to. Feb 01, 2017 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 journal is mainly focused on clinical practice and scientific communication, targeted to internists and other medical specialists. The disease now known as the waterhouse friderichsen syndrome was recognized as an entity by little1 in 1901. Waterhousefriderichsen syndrome a rare disorder caused by invasion of the blood by meningococcus organisms often in the course of an attack of meningitis.
Autopsy, forensic microbiology, waterhousefriderichsen syndrome. The presenting clinical picture is frequently pharyngitis, fever, and. Fatal purpura fulminans and waterhousefriderichsen syndrome. Rechtsmedizinisches institut, universitat zurich, zurich.
The presence of dic in the waterhousefriderichsen syndrome suggests that heparin should be a logical form of treatment. Waterhouse friderichsen syndrome can be caused by a number of different organisms see below. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage waterhousefriderichsen syndrome. Adrenal gland and paraganglia waterhouse friderichsen syndrome more common in children, particularly before age 2 years usually bilateral. Pathology outlines waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome, a rare type of septicemia blood poisoning of rapid and severe onset, marked by fever, collapse and sometimes coma, hemorrhage from skin and mucous membranes, and severe bilateral hemorrhage of the adrenal cortical tissue. This case on autopsy showed massive bilateral hemorrhage into the adrenal glands. Asplenic patients are at increased risk for sepsis and fulminant infection.
Enlarging purple spots appear on the skin and there is rapid collapse and coma. All structured data from the file and property namespaces is available under the creative commons cc0 license. Thieme ejournals dmw deutsche medizinische wochenschrift. The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. First case reported in adults waterhousefriderichsen syndrome secondary to staphylococcus haemolyticus is concluded.
Medicina interna diagnostico y tratamiento miguel matarama penate raimundo llanio navarro pedro muniz iglesias. Estudio sobre diferencias significativas entre padres y madres. Adrenal gland and paraganglia waterhousefriderichsen syndrome more common in children, particularly before age 2 years usually bilateral. 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. First reported as an entity by waterhouse in 1911, the syndrome received its present title following friderichsens report in 1918 of a similar case with a fulminating course. 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. 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 importance of the postmortem interval for the diagnosis. Waterhousefriderichsen syndrome information mount sinai. Meningococcemia is a lifethreatening infection that occurs when the bacteria neisseria meningitidis invades the. An experimental model that produces adrenal cortical hemorrhage with endotoxin has been described.
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