It is grouped with the morganellaceae and is an opportunistic pathogen of humans. Despite the predominant association with meningococcal infection, there are numerous other. Waterhousefriderichsen syndrome radiology reference. The syndrome is most common in children under five and may last only a few hours. The waterhousefriderichsen syndrome is a clinical entity first described in 1911 by waterhouse1 in an eightmonthold child. The disease now known as the waterhousefriderichsen syndrome was recognized as an entity by little 1 in 1901. Pdf first case reported in adults waterhousefriderichsen syndrome secondary to staphylococcus haemolyticus is concluded. Kidney problems and low platelets then occur as the diarrhea is improving. The earliest report of the condition now known as the waterhousefriderichsen syndrome appeared in 1894, when voelcker 1 described it as a fulminating purpura in conjunction with bilateral adrenal hemorrhage. If you have problems viewing pdf files, download the latest version of adobe reader. The waterhousefriderichsen syndrome was described by the british physician r. Post the definition of waterhousefriderichsen syndrome to facebook share the definition of waterhousefriderichsen syndrome on twitter. 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. The adrenal glands produce and release different hormones that the body.
Cushingcushing ss syndrome syndrome 5x increase in mortality zhypertension zdiabetes ztruncal obesity lindholm j, juul s, jorgensen j o, et al. Symptoms include acute adrenal gland insufficiency, and profound shock. It is known to cause wound infections and other species of its genera. 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. Dictionary entries near wa ter housefrid er ich sen syndrome. Sepsis in these patients is typically secondary to encapsulated bacteria, with streptococcus pneumoniae being the most frequent pathogen. Meningokokkensepsis mit nebennierenbeteiligung waterhouse. Waterhousefriderichsen syndrome penn state hershey. 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. Clinical and economic burden of invasive meningococcal. Adrenal insufficiency in the pediatric emergency department rachel tuuri, md, donald zimmerman, md a 12yearold girl with congenital adrenal hyperplasia case 1 cah who is on maintenance doses of a glucocorticoid and mineralocorticoid presents to the emergency department ed with a 3day history of cough, congestion, and sore throat. Cushing syndrome can be broadly divided into exogenous and endogenous causes.
In many cases the distinction between symptoms of waterhousefriderichsen syndrome and complications of waterhousefriderichsen syndrome is unclear or arbitrary. Waterhousefriderichsen syndrome statpearls ncbi bookshelf. Waterhouse friderichsen syndrome wfs instructional tutorial video. Recommended antimicrobial treatment is with penicillin, ceftriaxone or chloramphenicol.
Diseases information specialists for waterhousefriderichsen syndrome. Alternative names fulminant meningococcemia waterhousefriderichsen syndrome. Less common causes are burns, cardiac failure, hypothermia and birth trauma. Fatal purpura fulminans and waterhousefriderichsen. Files are available under licenses specified on their description page. A case of waterhousefriderichsen syndrome resulting from an invasive pneumococcal infection in a patient with a hypoplastic spleen. Most patients with this condition are children, although adults may rarely be affected. The advent of epidemic camrsa has posed a number of emergent issues. Diseases caused by this organism include meningitis, meningococcemia and rash thrombocytopenia and evolving dic, and waterhousefriderichsen syndrome of bilateral hemorrhagic destruction of adrenal glands. Ciliated cells, exotoxin, secondary infection, lymphocytosis pseudomonas. A collection of disease information resources and questions answered by our.
Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Treatment nonpharmacologic incision and drainage of furuncles, abscesses higher failure rate in children w abscesses 5cm nonsystemic antimicrobials local application of topical antimicrobials mupirocin oral antimicrobials macrolides, clindamycin, tmpsmx. Initial symptoms typically include bloody diarrhea, fever, vomiting, and weakness. All structured data from the file and property namespaces is available under the creative commons cc0 license. The term incidence of waterhousefriderichsen syndrome refers to the annual diagnosis rate, or the number of new cases of waterhousefriderichsen syndrome diagnosed each year. Waterhousefriderichsen syndromeadrenal hemorrhage infarct may occur 2 hypotension nasal carriage in 510% chronic form, very rare n. Waterhousefriderichsen syndrome pathology britannica.
Waterhousefriderichsen syndrome is adrenal gland failure due to bleeding into the adrenal gland. This is especially true of patients who reside in longterm care facilities, who have longterm indwelling urethral catheters, or who have a known history of urethral anatomic abnormalities. The presence of the sepsis syndrome associated with a urinary tract infection uti should raise the possibility of urinary tract obstruction. 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. Infectious disease university of missourikansas city. Waterhousefriderichsen syndrome wfs is an emergency condition, characterized by fever, cyanosis, bruises, andor shock. Ceftriaxone is an antibiotic commonly employed today. A blood sample should be drawn, and treatment started immediately before obtaining. Between 1997 and 2002, five cases of fatal waterhousefriderichsen syndrome wfs that occurred in infancy or childhood were investigated in our institute. Medical definition of waterhousefriderichsen syndrome. However, because her clinical presentation suggested meningococcemia, a prophylactic regimen of rifampin was prescribed for infants, toddlers, and staff at the ccc she attended.
Onset of pulmonary edema or acute respiratory distress syndrome requires the use of positive endexpiratory pressure peep. In most other studies that investigated specific outcomes, renal complications and sequelae were not investigated or no events were reported 7,14,22,27,29,31. Avoid nasal intubation with its increased risk of bleeding from the adenoids in patients with dic. Waterhouse in 1911 as a case of suprarenal apoplexy 1 and by c. Pdf waterhousefriderichsen syndrome in an adult patient with. Waterhousefriderichsen syndrome purpura fulminans mrsa. From the first issue of the more recent archive on january 4, 1945, one sees examples of the evolution of modern medicine, such as in a scholarly article on the waterhousefriderichsen syndrome. Meningococcemia is a lifethreatening infection that occurs when the bacteria neisseria meningitidis invades the blood stream. Massive adrenal haemorrhage waterhousefriderichsen syndrome 1 is an uncommon, but usually fatal, consequence of overwhelming sepsis. Adrenal hemorrhage is a relatively uncommon condition with a variable and nonspecific presentation that may lead to acute adrenal crisis, shock, and death unless it is recognized promptly and treated appropriately. Waterhousefriderichsen syndrome rapidly progressive hypotension leading to shock dic with widespread purpura rapidly developing adrenocorticalinsufficieny due to massive bilateral haemorrhage high risk in children basis. Asplenic patients are at increased risk for sepsis and fulminant infection.
Pdf a case of waterhousefriderichsen syndrome resulting. Fulminant meningococcal sepsis waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome infectious disease a clinical crisis characterized by acute adrenal insufficiency, often accompanied by septicemia. 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. First, it has changed the paradigm of medical practice. The occurrence of this condition is also attributed to a disease called waterhouse friderichsen syndrome wfs, which leads to the failure of the adrenal glands, also known as an adrenal crisis, due to excessive bleeding into the glands itself. It is usually caused by severe meningococcal infection or other severe, bacterial infection. Waterhousefriderichsen syndrome medigoo health tests. Meningokokkensepsis mit nebennierenbeteiligung waterhousefriderichsensyndrom. Urticarialike lesions, ulcers, nodules, hemorrhagic blisters, pustules and digital necrosis typically small vessels 7 to 10 days after drug administration and files are available under licenses specified on their description page. Full text get a printable copy pdf file of the complete article 1. Complications of waterhousefriderichsen syndrome are secondary conditions, symptoms, or other disorders that are caused by waterhousefriderichsen syndrome. Waterhousefriderichsen syndrome information mount sinai. Waterhousefriderichsen syndrome wfs is defined as adrenal gland failure due to bleeding.
A case of waterhousefriderichsen syndrome resulting from. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Diseases associated with defects in receptor proteins. Waterhouse friderichsen syndrome adrenal infarction afterduring meningococcal meningits, decreased cortisol level 124. Disseminated intravascular coagulation and the waterhouse. Waterhousefriderichsen syndrome secondary to escherichia. Waterhousefriderichsen syndrome is a medical emergency and needs to be treated with adequate antibiotics as fast as possible. Feb 04, 2016 waterhouse friderichsen syndrome is most often associated with meningococcal disease accounts for 80% of cases. The vast majority are the result of the administration of exogenous glucocorticoids iatrogenic cushing syndrome.
Waterhousefriderichsen syndrome as a result of non. Hydrocortisone can sometimes reverse the hypoadrenal shock. Waterhousefriderichsen syndrome wfs by stacy arvinna binti jamarun group 3 6th year 2nd semester paedriatic disease 2. The bleeding is caused by severe bacterial infection. Most frequent causes of primary adrenal insufficiency are autoimmune adrenalitis and congenital adrenal hyperplasia. The occurrence of this condition is also attributed to a disease called waterhousefriderichsen syndrome wfs, which leads to the failure of the adrenal glands, also known as an adrenal crisis, due to excessive bleeding into the glands itself. Hemolyticuremic syndrome hus is a group of blood disorders characterized by low red blood cells, acute kidney failure, and low platelets. Gross pathology of waterhousefriderichsen syndrome. Rare complications of severe sepsis include purpura fulminans and bilateral adrenal hemorrhage waterhousefriderichsen syndrome. Waterhouse friderichsen syndrome after infection with group a streptococcus. Request pdf waterhousefriderichsen syndrome secondary to escherichia coli septicaemia waterhousefriderichsen syndrome is defined as acute bilateral haemorrhagic necrosis of the adrenal.
Pdf waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. On february 9, 1994, a 7monthold infant patient 2 who attended the same ccc died from septic shock, purpura, and waterhousefriderichsen syndrome. What links here related changes upload file special pages permanent link page information wikidata. The icd9 codes for external causes of death are actually codes e800 through. International classification of diseases, ninth revision icd9 underlying and multiple cause of death codes for deaths occurring from 1979 1998 important if you decide to print this document, note that it is about 156 pages. Adrenal insufficiency in the pediatric emergency department. Hemorrhagic necrosis of adrenal glands, usually due to bacteremia, classically neisseria meningitides. Waterhousefriderichsen syndrome is one of the fatal complications of meningococcal infection. Cushing syndrome caused by, 317 hyperaldosteronism caused by, 318 adrenal carcinomas cushing syndrome caused by, 317 pglycoprotein in, 232 adrenal cortex, 306 embryologic derivatives of, 563 progesterone production in, 577 adrenal hemorrhage, 630 waterhouse friderichsen syndrome and, 318 adrenal hyperplasia cushing syndrome caused by, 317. Gonorrhea rate tops us chart timesdispatch richmond, va. 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. For language access assistance, contact the ncats public information officer. Karakousis pc, page kr, varello ma, howlett pj, stieritz dd. The waterhousefriderichsen syndrome in the differential diagnosis of acute abdominal crises.
Hypertensive heart and chronic kidney disease with heart failure and with stage 5 chronic kidney disease, or end stage renal disease. The diagnosis of wfs was based on the following clinical as well as morphological criteria. Pathological features of waterhousefriderichsen syndrome. The physician is required to open the file to the patient every eight days. A condition developed due to failure of adrenal gland, caused by bleeding into the adrenal glands. Waterhousefriderichsen syndrome genetic and rare diseases. Waterhousefriderichsen syndrome wfs is a rare clinical.
The presence of dic in the waterhousefriderichsen syndrome suggests that heparin should be a logical form of treatment. This would lead to a publichealth catastrophe as we are running out of effective antibiotics for. Table 6i complete mcc list diagnosis code description. 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.
Dem waterhousefriderichsensyndrom liegt als pathogenetisches prinzip ahnlich wie beim sanarellishwartzmanphanomen ein. Benzylpenicillin was once the drug of choice with chloramphenicol as a good alternative in allergic patients. Definition waterhousefriderichsen syndrome wfs or hemorrhagic adrenalitis or fulminant meningococcemia, is defined as adrenal gland failure due to bleeding into the adrenal. We, therefore, report a case of waterhousefriderichsen syndrome resulting from an invasive pneumococcal infection in a patient with a hypoplastic spleen. Pathology outlines waterhousefriderichsen syndrome. The most frequent complication was waterhouse friderichsen syndrome covering adrenal hemorrhage 11.
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