We believe that our report may assist emergency clinicians in recognizing the condition promptly leading to early treatment, including plasma. Waterhousefriderichsen syndrome penn state hershey medical. 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 syndrome, friderichsenwaterhouse syndrome. Neisseria meningitidisinduced acute systemic meningococcal disease is an emergency and a fatal condition that has a high mortality rate. Dem waterhousefriderichsensyndrom liegt als pathogenetisches. Pathology outlines waterhousefriderichsen syndrome. On february 9, 1994, a 7monthold infant patient 2 who attended the same ccc died from septic shock, purpura, and waterhouse friderichsen syndrome. A unique fatal case of waterhousefriderichsen syndrome caus.
Neisseria meningitidis cause a fulminating fatal infection, frequently with few or no signs or preconditions. Files are available under licenses specified on their description page. The waterhousefriderichsen syndrome wfs is associated with some infectious diseases leading to substantial morbidity by producing acute adrenal insufficiency associated with hemorrhage and dysfunction of the suprarenal glands, at a time when their response is crucial to address acute stress table 16. Find, read and cite all the research you need on researchgate. The waterhousefriderichsen syndrome wfs, also known as. Full text full text is available as a scanned copy of the original print version. Waterhousefriderichsen syndrome europe pmc article.
The waterhousefriderichsen syndrome was described by the british physician r. A case of waterhousefriderichsen syndrome resulting from. A condition occurring mainly in children under 10 years of age, characterized by vomiting, diarrhea, extensive purpura, cyanosis, tonicclonic convulsions, and circulatory collapse, usually with meningitis and hemorrhage into the adrenal glands. Wfs is fatal unless treatment for the bacterial infection is started right. Waterhousefriderichsen syndrome medigoo health tests.
Waterhousefriderichsen syndrome uf health, university of. Get a printable copy pdf file of the complete article 439k. This results in adrenal crisis, or acute adrenal insufficiency, which is when the adrenal gland suddenly stops producing hormones. If you have problems viewing pdf files, download the latest version of adobe reader. Waterhousefriderichsen syndrome occurs when the blood vessels in the adrenal gland rupture during a severe bacterial infection, turning the adrenal glands into sacks of blood. 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. We, therefore, report a case of waterhouse friderichsen syndrome resulting from an invasive pneumococcal infection in a patient with a hypoplastic spleen. Meningococcemia is a lifethreatening infection that occurs when the bacteria neisseria meningitidis invades the blood stream. This report describes three fatal cases of the syndrome in which the causative organism proved to be a streptococcus.
Waterhousefriderichsen syndrome article about waterhouse. We, therefore, report a case of waterhousefriderichsen syndrome resulting from an invasive pneumococcal infection in a patient with a hypoplastic spleen. Ideal sources for wikipedias health content are defined in the guideline wikipedia. If early diagnosis and antibacterial treatment, along with steroids, is not. Over the years, there have been reports of bilateral adrenal hemorrhage ah n association with multiple etiologies, including various systemic. Surgical indications are strictly limited in such patients. Waterhouse friderichsen syndrome a rare disorder caused by invasion of the blood by meningococcus organisms often in the course of an attack of meningitis. Waterhousefriderichsen syndrome wfs is a condition characterized by adrenal gland failure caused by bleeding into adrenal glands as a result of severe bacterial infection, the most common of which is the meningococcus neisseria meningitidis.
The waterhousefriderichsen syndrome is a clinical entity first described in 1911 by waterhouse 1 in an eightmonthold. Waterhousefriderichsen syndrome wfs is a rare clinical conundrum first described by rupert waterhouse and carl friderichsen as bilateral adrenal hemorrhage in the setting of bacterial sepsis among children in the first decade of the 20th century. Dexamethasone for the treatment of tuberculous meningitis in adolescents and adults. Waterhousefriderichsen syndrome definition of waterhouse. Seventy per cent of all cases reported are in children under the age of two years. Wfs is reported to occur in splenectomized patients, and in patients with congenital asplenia or decreased splenic function 25. Friderichsen definition of friderichsen by medical dictionary. Waterhouse in 1911 as a case of suprarenal apoplexy1 and by c.
Waterhousefriderichsen syndrome medical definition merriam. Waterhousefriderichsen syndrome wfs is a rare clinical conundrum first. Waterhousefriderichsen syndrome a rare disorder caused by invasion of the blood by meningococcus organisms often in the course of an attack of meningitis. Fulminant meningococcal sepsis waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding. Fulminant meningococcemia waterhouse friderichsen syndrome. In patients with a fulminant infection, a maculopapular petechial eruption, purpura fulminans, or an ecchymotic lesion are worrisome signs reflecting disseminated intravascular coagulation dic and hint at waterhousefriderichsen syndrome wfs. Friderichsen definition of friderichsen by medical. Pdf waterhousefriderichsen syndrome is one of the fatal. Waterhousefriderichsen syndrome wfs is an emergency condition, characterized by fever, cyanosis, bruises, andor shock. Waterhouse friderichsen syndrome, first reported in 1911 by rubert waterhouse, is characterized by fever, rash, purpura, coagulopathy, and shock. Waterhousefriderichsen syndrome definition waterhousefriderichsen syndrome wfs is a group of symptoms resulting from the failure of the adrenal glands to. Waterhouse friderichsen syndrome annals of internal. Definition waterhousefriderichsen syndrome wfs or hemorrhagic adrenalitis or fulminant meningococcemia, is defined as adrenal gland failure due to bleeding into the adrenal.
Adrenal gland and paraganglia waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal. The presence of purpuric or petechial spots on the skin, associated with a massive hemorrhagic destruction of both adrenals and the presence of a fulminating septicemia, is recognized as the waterhousefriderichsen syndrome. The disease now known as the waterhouse friderichsen syndrome was recognized as an entity by little1 in 1901. Click on the link to view a sample search on this topic. Anesthesia requires aggressive resuscitation using plasma expanders and inotropes, with the aid of invasive hemodynamic monitoring through arterial lines, central venous catheter, and pulmonary arterial catheter, if indicated.
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. Waterhouse friderichsen syndrome waterhouse friderichsen syndrome. In the second decade of the 20th century, rupert waterhouse and carl friderichsen described suprarenal apoplexy or hemorrhage in infants. Waterhousefriderichsen syndrome radiology reference. Little earlier had described 4 cases, and aegerters 1936 report1. Waterhousefriderichsen syndrome as a result of non. The sudden occurrence and rapid development of multiple petechiae, cyanosis and profound collapse in the course of an acute infection herald dramatically the condition known as waterhouse friderichsen syndrome. Waterhouse friderichsen syndrome waterhousefriderichsen. Adrenal hemorrhage has been associated with meningococcemia waterhousefriderichsen syndrome, but pseudomonas aeruginosa was the most common pathogen in one report of 51 children. Links to pubmed are also available for selected references. Waterhousefriderichsen syndrome dictionary definition.
On february 9, 1994, a 7monthold infant patient 2 who attended the same ccc died from septic shock, purpura, and waterhousefriderichsen syndrome. The waterhouse friderichsen syndrome is a clinical entity first described in 1911 by waterhouse 1 in an eightmonthold. It has been suggested by many authors that this syndrome is more common than what literature reports indicate, many cases probably being missed due to lack of familiarity with the condition. Waterhouse friderichsen syndrome can be caused by a number of different organisms see below. The syndrome is most common in children under five and may last only a few hours. Here, we report a rare case of wfs, presenting with purpura fulminans, resulting from an invasive pneumococcal infection. Get a printable copy pdf file of the complete article 439k, or click on a page image below to browse page by page. Waterhousefriderichsen syndrome statpearls ncbi bookshelf. A case of waterhousefriderichsen syndrome resulting from an. Waterhouse friderichsen syndrome wfs is an emergency condition, characterized by fever, cyanosis, bruises, andor shock. 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, 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. The adrenal glands produce and release different hormones that the body.
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. Waterhouse friderichsen syndrome occurs when the blood vessels in the adrenal gland rupture during a severe bacterial infection, turning the adrenal glands into sacks of blood. Waterhousefriderichsen syndrome in an adult patient with. Fulminant meningococcemia waterhousefriderichsen syndrome. There is bleeding into the adrenal glands causing adrenal failure and shock. Grampositive cocci were detected on a smear of the patients blood buffy coat, and a latex agglutination test on. To access free multiple choice questions on this topic, click here. Waterhousefriderichsen syndrome medical definition. Here are links to possibly useful sources of information about waterhousefriderichsen syndrome.
The condition is most frequently seen in children younger than 10 years old. All structured data from the file and property namespaces is available under the creative commons cc0 license. First case reported in adults waterhouse friderichsen syndrome secondary to staphylococcus haemolyticus is concluded. Septic shock caused by waterhousefriderichsen syndrome. Here we report a fatal case of this syndrome due to neisseria meningitidis in a 29yearold male patient who was admitted with highgrade fever and chills and vomiting since 7 days, a skin rash over the abdomen and trunk, and altered sensorium since 2 days. The term incidence of waterhousefriderichsen syndrome refers to the annual diagnosis rate, or the number of new cases of waterhousefriderichsen syndrome diagnosed each year. Large petechial hemorrhages in the skin and mucous membranes fever septic shock disseminated intravascular coagulation.
Rechtsmedizinische erkentnisse zum waterhousefriderichsen. Septic shock caused by neisseria meningitidis infection, defined as waterhousefriderichsen syndrome in 1946, has a high mortality rate of between 21% and 44%. Pdf waterhousefriderichsen syndrome in an adult patient with. When caused by neisseria meningitidis, wfs is considered the most severe form of meningococcal sepsis. Waterhousefriderichsen syndrome wfs by stacy arvinna binti jamarun group 3 6th year 2nd semester paedriatic disease 2. Waterhousefriderichsen syndrome wfs is a group of symptoms.
The waterhousefriderichsen syndrome wfs is associated with some. The disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little1 in 1901. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free. Waterhouse supplemented his case with 15 from the literature, while friderichsen added 2 more. Apr 03, 20 occur usually in infants or children younger than 10, occasionally in adults. For language access assistance, contact the ncats public information officer. Brain sciences free fulltext neisseria meningitidis. Waterhouse friderichsen syndrome wfs instructional tutorial video.
Waterhouse friderichsen syndrome definition 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 syndrome genetic and rare diseases. Medical definition of waterhouse friderichsen syndrome. The waterhouse friderichsen syndrome may develop in 10 to 20 percent of children with meningococcal infection.
The bacterial infection leads to massive bleeding into one or usually both adrenal glands. Feb 04, 2016 access to this database is free of charge. May 20, 20 waterhouse friderichsen syndrome wfs instructional tutorial video. The organisms were detected during routine coroners. Dec 31, 2012 waterhouse friderichsen syndrome wfs is a condition characterized by adrenal gland failure caused by bleeding into adrenal glands as a result of severe bacterial infection, the most common of which is the meningococcus neisseria meningitidis.
Waterhousefriderichsen syndrome information mount sinai. Medical definition of waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. This may not be the complete list of references from this article. 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. Dec 07, 2012 overview of waterhousefriderichsen syndrome. Grampositive cocci were detected on a smear of the patients blood buffy coat, and a latex agglutination test on cerebrospinal fluid csf indicated infection with s. Waterhousefriderichsen syndrome an overview sciencedirect. The onset of the illness is nonspecific with fever, rigors, vomiting, and headache.
The presenting clinical picture is frequently pharyngitis, fever, and. 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. In response to acute stress, adrenal changes result in increased size of the cortex. Biography luis del carpio orantes is a medical specialist in internal medicine and also an internist in mexican social security institute. Pdf first case reported in adults waterhousefriderichsen syndrome secondary to staphylococcus haemolyticus is concluded. Waterhousefriderichsen syndrome pathology britannica. Fulminant meningococcal sepsis waterhouse friderichsen syndrome. The waterhousefriderichsen syndrome wfs is the morphological. One is stunned by the rapid and usually fatal progression of this clinical picture. Rechtsmedizinisches institut, universitat zurich, zurich. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection.
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