Medical definition of waterhouse friderichsen syndrome. Considered as a medical emergency, it requires immediate medical attention as progression of the disorder might lead. Meningococcemia is a lifethreatening infection that occurs when the bacteria neisseria meningitidis invades the. Waterhousefriderichsen syndrome radiology reference. 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.
Waterhouse friderichsen syndrome can be caused by a number of different organisms see below. Waterhousefriderichsen syndrome wfs by stacy arvinna binti jamarun group 3 6th year 2nd semester paedriatic disease 2. 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. Pathological features of waterhousefriderichsen syndrome in.
Fatal purpura fulminans and waterhousefriderichsen syndrome. Friderichsenwaterhouse syndrome or disease icd10cm. Alternative names fulminant meningococcemia waterhouse friderichsen syndrome. 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. The term incidence of waterhouse friderichsen syndrome refers to the annual diagnosis rate, or the number of new cases of waterhouse friderichsen syndrome diagnosed each year. Meningococcemia is a lifethreatening infection that occurs when the bacteria neisseria meningitidis invades the blood stream. 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. Waterhousefriderichsen syndrome is a medical emergency and needs to be treated with adequate antibiotics as fast as possible. Publications home of jama and the specialty journals of. Alternative names fulminant meningococcemia waterhousefriderichsen syndrome. Feb 04, 2016 waterhouse friderichsen syndrome is most often associated with meningococcal disease accounts for 80% of cases. We, therefore, report a case of waterhousefriderichsen syndrome resulting from an invasive pneumococcal infection in a patient with a hypoplastic spleen. The waterhousefriderichsen syndrome wfs is the morphological substrate of a rapidly, mostly irreversible shock due to endotoxemia similar.
Waterhouse friderichsen syndrome waterhousefriderichsen. Waterhouse in 1911 as a case of suprarenal apoplexy1 and by c. In addition to bacterial infection, severe stress increases the risk of adrenal hemorrhage, perhaps because elevated acth secretion results in increased blood supply to an organ with limited venous drainage. The diagnosis of waterhousefriderichsen syndrome as the cause of death was established post mortem based on autopsy findings, microscopic examination.
A case of waterhousefriderichsen syndrome resulting from an. Between 1997 and 2002, five cases of fatal waterhousefriderichsen syndrome wfs that occurred in infancy or childhood were investigated in our institute. This means that waterhousefriderichsen syndrome, or a subtype of waterhousefriderichsen syndrome, affects less than 200,000 people in the us population. The waterhouse friderichsen syndrome is a clinical entity first described in 1911 by waterhouse 1 in an eightmonthold child. Waterhousefriderichsen syndrome is a descriptor in the national library of medicines controlled vocabulary thesaurus, mesh medical subject headings.
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 bleeding is caused by severe bacterial infection. The waterhouse friderichsen syndrome is a clinical entity first described in 1911 by waterhouse in an eightmonthold child. How to say waterhousefriderichsen syndrome in english. Emerging infectious diseases hemorrhage and shock associated. Definition waterhousefriderichsen syndrome wfs or hemorrhagic adrenalitis or fulminant meningococcemia, is defined as adrenal gland failure due to bleeding into the adrenal. Seventy per cent of all cases reported are in children under the age of two years. 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 waterhouse friderichsen syndrome.
Learn waterhousefriderichsen syndrome other adrenal disorders pathology for medicine faster and easier with picmonics unforgettable images and stories. Find, read and cite all the research you need on researchgate. Waterhousefriderichsen syndrome pathology britannica. Pdf first case reported in adults waterhousefriderichsen syndrome secondary to. Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity. Friderichsenwaterhouse syndrome or disease references in the icd10cm index to diseases and injuries. The disease now known as the waterhouse friderichsen syndrome was recognized as an entity by little1 in 1901. Waterhouse friderichsen syndrome wfs is a collection of symptoms resulting from the failure of the adrenal glands to function normally as a result of bleeding into the gland. Home adrenal gland and paraganglia waterhouse friderichsen 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.
Abstract the waterhousefriderichsen syndrome wfs is the maximum form of a peracute meningococcal sep sis. 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. Waterhouse friderichsen syndrome, first reported in 1911 by rubert waterhouse, is characterized by fever, rash, purpura, coagulopathy, and shock. Waterhousefriderichsen syndrome wfs is an emergency condition, characterized by fever, cyanosis, bruises, andor shock. At the present time almost 150 cases have been reported, the vast majority being in infants and young. Waterhouse friderichsen syndrome is seen most frequently in patients with asplenia, and. Waterhousefriderichsen syndrome journal of clinical pathology. The syndrome of bleeding into the skin and other organs including the adrenals in the presence of a severe septicemia is becoming more frequently recognized by clinicians both here and abroad. A case of waterhousefriderichsen syndrome associated with septicaemia caused by a df2 group bacterium dysgonic fermentertype 2atlanta classification is presented. Causes the adrenal glands are 2 triangleshaped glands. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. Waterhousefriderichsen syndrome information mount sinai.
Adrenal hemorrhage has been associated with meningococcemia waterhousefriderichsen syndrome, but pseudomonas aeruginosa was the most common pathogen in one report of 51 children clinical manifestations of adrenal insufficiency in adults view in chinese. A fatal case of waterhousefriderichsen syndrome resulting from ewingella americana infection in a previously healthy 74yearold woman is reported. Causes the adrenal glands are two triangleshaped glands. The diagnosis of wfs was based on the following clinical as well as morphological criteria. Hemorrhagic necrosis of adrenal glands, usually due to bacteremia, classically neisseria meningitides. All structured data from the file and property namespaces is available under the creative commons cc0 license. Links to pubmed are also available for selected references. Pdf first case reported in adults waterhousefriderichsen syndrome secondary to staphylococcus haemolyticus is concluded find, read and cite all the research you need on researchgate.
May 20, 20 waterhouse friderichsen syndrome wfs instructional tutorial video. Waterhouse friderichsen syndrome waterhousefriderichsen syndrome. The condition is most frequently seen in children younger than 10 years old. It is usually caused by severe meningococcal infection or other severe, bacterial infection. Waterhousefriderichsen syndrome a report of a case in a. It is characterized by overwhelming bacterial infection meningococcemia. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Waterhousefriderichsen syndrome multimedia encyclopedia.
For language access assistance, contact the ncats public information officer. Waterhouse in 1911 as a case of suprarenal apoplexy 1 and by c. Plotzlicher tod bei waterhousefriderichsensyndrom im. The waterhousefriderichsen syndrome in the differential diagnosis of acute abdominal crises. Files are available under licenses specified on their description page. Two hours after the onset of fever and malaise, a 15yearold boy began to have. Diseases caused by this organism include meningitis, meningococcemia and rash thrombocytopenia and evolving dic, and waterhouse friderichsen syndrome of bilateral hemorrhagic destruction of adrenal glands. Acquired postinfectious disease that was first described by arthur francis voelcker in 1894, and subsequently explained by carl friderichsen, danish pediatrician in 1918. Waterhouse friderichsen syndrome complicating primary biliary sepsis due to pasteurella multocida in a patient with cirrhosis. The syndrome also has been associated with other bacterial pathogens, including streptococcus pneumoniae, group a betahemolytic streptococci, neisseria gonorrhoeae, escherichia coli, klebsiella pneumoniae, haemophilus influenzae group b, salmonella choleraesuis, pasteurella. 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. Waterhouse supplemented his case with 15 from the literature, while friderichsen added 2 more.
M ip, j g teo, and a f cheng department of microbiology, chinese university of hong kong, prince of wales hospital, shatin. 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 is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. One is stunned by the rapid and usually fatal progression of this clinical picture. 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 adrenal gland failure due to bleeding into the adrenal gland. A case of waterhousefriderichsen syndrome resulting from. References in the icd10cm index to diseases and injuries applicable to the clinical term friderichsenwaterhouse syndrome or disease friderichsenwaterhouse syndrome or disease a39.
Learn vocabulary, terms, and more with flashcards, games, and other study tools. Pdf waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Pdf waterhousefriderichsen syndrome in an adult patient with. Acute adrenal failure due to massive adrenocortical hemorrhage associated with bacterial sepsis is known as waterhousefriderichsen syndrome fig. If you have problems viewing pdf files, download the latest version of adobe reader. Full text full text is available as a scanned copy of the original print version. Fulminant meningococcal sepsis waterhouse friderichsen syndrome. 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. Attention is drawn to the relation of df2 group septicaemias to dog bites or contact and impaired host defence after splenectomy. Waterhousefriderichsen syndrome as a result of non. Friderichsen syndrome as a result of nonmeningococcal infection. We, therefore, report a case of waterhouse friderichsen syndrome resulting from an invasive pneumococcal infection in a patient with a hypoplastic spleen. Waterhousefriderichsen syndrome penn state hershey medical.
If early diagnosis and antibacterial treatment, along with steroids, is not. Waterhousefriderichsen syndrome linkedin slideshare. The information regarding adrenal insufficiency is in conflict with several sources. On february 9, 1994, a 7monthold infant patient 2 who attended the same ccc died from septic shock, purpura, and waterhouse friderichsen syndrome. Woman 24 years old with no history of importance except. Symptoms include acute adrenal gland insufficiency, and profound shock. The patient died suddenly within 14 hours after presentation. Waterhousefriderichsen syndrome is characterized by the abrupt onset of fever, petechiae, arthralgia, weakness, and myalgias, followed by acute hemorrhagic necrosis of the adrenal glands and. Hydrocortisone can sometimes reverse the hypoadrenal shock. Waterhousefriderichsen syndrome wfs linkedin slideshare. Adrenal gland and paraganglia adrenal insufficiency waterhouse friderichsen 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. Waterhouse friderichsen syndrome wfs is an emergency condition, characterized by fever, cyanosis, bruises, andor shock.
In the second decade of the 20th century, rupert waterhouse and carl friderichsen described suprarenal apoplexy or hemorrhage in infants. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal. Picmonic is research proven to increase your memory retention and test scores. In response to acute stress, adrenal changes result in.
Waterhousefriderichsen syndrome europe pmc article. Waterhousefriderichsen syndrome is an abnormal condition of the adrenal glands that occur due to bacterial infection. Typically, it is caused by neisseria meningitidis the bacterial infection leads to massive bleeding into one or usually both adrenal glands. Waterhousefriderichsen syndrome complicating primary biliary. Other bacteria that can cause waterhousefriderichsen syndrome include pseudomonas aeruginosa and haemophilus influenzae.
The waterhousefriderichsen syndrome a report of a case in a soldier, the military surgeon, volume 93, issue 1, 1 july 1943. It is sometimes said that the hemorrhage in waterhouse friderichsen syndrome causes an acute adrenal insufficiency, but this is inaccurate, since blood cortisol levels are not decreased. Pdf first case reported in adults waterhousefriderichsen syndrome secondary to staphylococcus haemolyticus is concluded. Pronunciation of waterhousefriderichsen syndrome with 2 audio pronunciations, 1 synonym, 1 meaning, 6 translations and more for waterhousefriderichsen syndrome. 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. Benzylpenicillin was once the drug of choice with chloramphenicol as a good alternative in allergic patients. Waterhousefriderichsen syndrome definition of waterhouse. A condition developed due to failure of adrenal gland, caused by bleeding into the adrenal glands. The waterhousefriderichsen syndrome was described by the british physician r. Less common causes are burns, cardiac failure, hypothermia and birth trauma. Pdf first case reported in adults waterhousefriderichsen syndrome secondary to staphylococcus haemolyticus is concluded find, read and. The adrenal glands produce and release different hormones that the body. 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. Waterhousefriderichsen syndrome caused by haemophilus influenzae type b in an immunocompetent young adult.
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. When caused by neisseria meningitidis, wfs is considered the most severe form of meningococcal sepsis. Pathology outlines waterhousefriderichsen syndrome. This report describes three fatal cases of the syndrome in which the causative organism proved to be a streptococcus. The disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little1 in 1901. The importance of the postmortem interval for the diagnosis of waterhousefriderichsen syndrome by neisseria meningitidis in a series of forensic cases arranged in couples diplococcus, and is. The root cause of waterhousefriderichsen syndrome is severe bacterial infection, or sepsis.
Medical definition of waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome massive 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 genetic and rare diseases. Waterhouse friderichsen syndrome annals of internal. Waterhousefriderichsen syndrome penn state hershey. Waterhousefriderichsen syndrome medigoo health tests.
Most patients with this condition are children, although adults may rarely be affected. Waterhousefriderichsen syndrome wfs is a collection of symptoms resulting from the failure of the adrenal glands to function normally as a result of bleeding into the gland. Fatal waterhousefriderichsen syndrome due to ewingella. 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 waterhousefriderichsen syndrome.