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