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. Waterhousefriderichsen syndrome is seen most frequently in patients with asplenia, and. The word syndrome comes from the greek word sundrom, which means concurrence of symptoms, or from word sundromos, which means running together. 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. Thieme ejournals dmw deutsche medizinische wochenschrift. Waterhousefriderichsen syndrome infectious disease a clinical crisis characterized by acute adrenal insufficiency, often accompanied by septicemia. Disseminated intravascular coagulation and the waterhousefriderichsen syndrome. The waterhousefriderichsen syndrome is a clinical entity first described in 1911 by waterhouse1 in an eightmonthold child. Waterhousefriderichsen 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. 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. Adrenal gland and paraganglia adrenal insufficiency waterhouse friderichsen syndrome. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. 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. This case on autopsy showed massive bilateral hemorrhage into the adrenal glands.
Causes the adrenal glands are two triangleshaped glands. Neisseria meningitidis, serogroup w, meningococcal infections, acute bacterial meningitis, meningococcal meningitis, purpurafulminans, waterhousefriderichsen syndrome. Pathology outlines waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome wfs linkedin slideshare. Because of consumption of coagulation factors and platelets, dic also has a hemorrhagic phase. Waterhousefriderichsen syndrome as a result of non. Medical definition of waterhouse friderichsen syndrome. Files are available under licenses specified on their description page. Waterhouse friderichsen syndrome waterhousefriderichsen syndrome. If you have problems viewing pdf files, download the latest version of adobe reader. Reyes syndrome is a rare disorder that causes brain and liver damage. The waterhouse friderichsen syndrome is a clinical entity first described in 1911 by waterhouse1 in an eightmonthold child. Although it can happen at any age, it is most often seen in children.
Waterhousefriderichsen syndrome caused by haemophilus. Medical definition of waterhousefriderichsen syndrome. Septic shock caused by 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 disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little1 in 1901. Meningococcemia is a lifethreatening infection that occurs when the bacteria neisseria meningitidis invades the blood stream. Search genetic and rare diseases information center gard. Fatal purpura fulminans and waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome definition of waterhouse. 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. The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Neisseria meningitidis cause a fulminating fatal infection, frequently with few or no signs or preconditions. Waterhousefriderichsen syndromemassive adrenal haemorrhage in the setting of overwhelming clinical sepsisis usually taken at necropsy to indicate. Waterhousefriderichsen syndrome information mount sinai. 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 described in 1894 by arthur francis voelcker 18611946 and in 1901 by the british dermatologist ernest gordon graham little 18671950. Waterhousefriderichsen syndrome pathology britannica. Waterhousefriderichsen syndrome an overview sciencedirect.
Between 1997 and 2002, five cases of fatal waterhousefriderichsen syndrome wfs that occurred in infancy or childhood were investigated in our institute. While waterhouse friderichsen syndrome is characterized mainly by acute, disseminated intravascular clotting with subsequent consumptioncoagulopathy, this sequence plays no demonstrable role. Waterhouse friderichsen syndrome can be caused by a number of different organisms see below. Waterhouse in 1911 as a case of suprarenal apoplexy1 and by c. While waterhousefriderichsen syndrome is characterized mainly by acute, disseminated intravascular clotting with subsequent consumptioncoagulopathy, this sequence plays no demonstrable role. The journal is mainly focused on clinical practice and scientific communication, targeted to internists and other medical specialists. One is stunned by the rapid and usually fatal progression of this clinical picture. Waterhousefriderichsen syndrome radiology reference. Home adrenal gland and paraganglia waterhousefriderichsen syndrome.
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. Summary we present the case of a patient affected by a waterhouse friderichsen syndrome presenting multiple organ failure and data in relation to acute adrenal insuf. The syndrome is most common in children under five and may last only a few hours. Here we report a fatal case of this syndrome due to neisseria. For language access assistance, contact the ncats public information officer. Adrenal gland and paraganglia adrenal insufficiency waterhousefriderichsen syndrome. 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. 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.
The disease now known as the waterhouse friderichsen syndrome was recognized as an entity by little1 in 1901. The presenting clinical picture is frequently pharyngitis, fever, and. Disseminated intravascular coagulation american journal of. Waterhouse friderichsen syndrome infectious disease a clinical crisis characterized by acute adrenal insufficiency, often accompanied by septicemia. Treatment of the bleeding patient with dic is supportive with the use of blood components. 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. It is characterized by overwhelming bacterial infection meningococcemia leading to. Neisseria meningitidis induced fatal waterhousefriderichsen. 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. 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. Pdf waterhousefriderichsen syndrome in an adult patient with. Waterhouse friderichsen syndrome caused by haemophilus influenzae type b in an immunocompetent young adult.
Syndrome definition of syndrome by medical dictionary. Waterhousefriderichsen syndrome also known as purpura fulminans 9 or hemorrhagic adrenalitis 10 is characterized by adrenal insufficiency that results from atraumatic adrenal hemorrhage in. Waterhousefriderichsen syndrome multimedia encyclopedia. All structured data from the file and property namespaces is available under the creative commons cc0 license. Massive adrenal haemorrhage waterhousefriderichsen syndrome 1 is an uncommon, but usually fatal, consequence of overwhelming sepsis. Waterhouse friderichsen syndrome is seen most frequently in patients with asplenia, and.
Causes the adrenal glands are 2 triangleshaped glands. 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. 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%. Waterhouse friderichsen syndrome wfs is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. It follows facial paralysis and seems to be due to straying of the regenerating nerve fibers, some of those destined for the salivary glands going to the lacrimal glands. Pdf waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. 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. 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 must be differentiated from severe meningococcal septicaemia by clinical, haematological and bloodcoagulation analysis. The condition is most frequently seen in children younger than 10 years old. 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. Home adrenal gland and paraganglia waterhouse friderichsen syndrome. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding. Neisseria meningitidis is a rare but serious pathogen that leads to lifethreatening septic shock. The waterhousefriderichsen syndrome was described by the british physician r. Waterhousefriderichsen syndrome must be differentiated from severe meningococcal septicaemia by clinical, haematological and bloodcoagulation analysis. When caused by neisseria meningitidis, wfs is considered the most severe form of meningococcal sepsis. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free.
Pathological features of waterhousefriderichsen syndrome in. Waterhouse friderichsen syndrome waterhousefriderichsen. The term incidence of waterhousefriderichsen syndrome refers to the annual diagnosis rate, or the number of new cases of waterhousefriderichsen syndrome diagnosed each year. Typically, it is caused by neisseria meningitidis the bacterial infection leads to massive bleeding into one or usually both adrenal glands.
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