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| | __NOTOC__ |
| | '''For patient information, click [[Adrenal hemorrhage (patient information)|here]]''' |
| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = Waterhouse-Friderichsen syndrome | | | Name = Adrenal hemorrhage | |
| Image = | | | Image = Adrenal-hemorrhage-202.jpg| |
| Caption = | | | Caption = Ultrasonography: Adrenal hemorrhage. <br> [http://www.radswiki.net Image courtesy of RadsWiki]| |
| DiseasesDB = 29316 |
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| ICD10 = {{ICD10|A|39|1|a|30}}, {{ICD10|E|35|1|e|20}} |
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| ICD9 = {{ICD9|036.3}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = |
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| eMedicineSubj = med |
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| eMedicineTopic = 3009 |
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| MeshID = D014884 |
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| }} | | }} |
| {{SI}} | | {{Adrenal hemorrhage}} |
| {{GS}} | | {{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}} |
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| {{Editor Join}} | | {{SK}} Waterhouse-Friderichsen syndrome (WFS); hemorrhagic adrenalitis |
| | == [[Adrenal hemorrhage overview|Overview]] == |
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| ==Overview== | | == [[Adrenal hemorrhage historical perspective|Historical Perspective]]== |
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| '''Adrenal hemorrhage''' or '''Waterhouse-Friderichsen syndrome''' (WFS) is massive, usually bilateral, [[hemorrhage]] into the [[adrenal gland]]s caused by [[Wiktionary:fulminant|fulminant]] [[meningococcemia]].<ref name=Robbins_2005>{{cite book | author = Kumar V, Abbas A, Fausto N | title = Robins and Coltran: Pathological Basis of Disease | edition = 7th | publisher = Elsevier | year = 2005 | pages = pp. 1214–5 | id = ISBN 978-0721601878}}</ref> WFS is characterised by overwhelming bacterial infection, rapidly progressive [[hypotension]] leading to [[Shock (medical)|shock]], [[disseminated intravascular coagulation]] (DIC) with widespread [[purpura]], particularly of the skin, and rapidly developing [[adrenal insufficiency|adrenocortical insufficiency]] associated with massive bilateral adrenal hemorrhage.
| | == [[Adrenal hemorrhage classification|Classification]] == |
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| ==Epidemiology== | | == [[Adrenal hemorrhage pathophysiology|Pathophysiology]] == |
| ''Meningococcus'' is another term for the [[bacteria]]l species ''[[Neisseria meningitidis]]'', which causes the type of [[meningitis]] which usually underlies this syndrome. Meningococcal meningitis occurs most commonly in children and young adults, and can occur in epidemics. In the United States it is the cause of about 20% of meningitis cases. At one time it was common among military recruits, but administration of the preventive meningococcal vaccine has greatly reduced this number. Freshman college students living in dormitory housing who have not been vaccinated are another risk group.
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| WFS can also be caused by ''[[Streptococcus pneumoniae]]'' infections, a common bacterial pathogen typically associated with meningitis in the adult and elderly population.<ref name=Robbins_2005 /> ''[[Staphylococcus aureus]]'' has recently also been implicated in [[Pediatrics|pediatric]] WFS.<ref name=Adem_2005>{{cite journal |author=Adem P, Montgomery C, Husain A, Koogler T, Arangelovich V, Humilier M, Boyle-Vavra S, Daum R |title=Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children |journal=N Engl J Med |volume=353 |issue=12 |pages=1245-51 |year=2005 |id=PMID 16177250}}</ref>
| | == [[Adrenal hemorrhage causes|Causes]] == |
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| Routine vaccination against meningococcus is recommended for people who have poor [[spleen|splenic]] function (who, for example, have had their spleen [[splenectomy|removed]] or who have [[Sickle-cell disease|sickle-cell anemia]] which damages the spleen), or who have certain immune disorders, such as [[Complement system|complement]] deficiency.<ref name=Rosa_2004>{{cite journal |author=Rosa D, Pasqualotto A, de Quadros M, Prezzi S |title=Deficiency of the eighth component of complement associated with recurrent meningococcal meningitis--case report and literature review |journal=Braz J Infect Dis |volume=8 |issue=4 |pages=328-30 |year=2004 |id=PMID 15565265}}</ref>
| | == [[Adrenal hemorrhage differential diagnosis|Differentiating Adrenal Hemorrhage from other Diseases]] == |
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| ==Etiology== | | == [[Adrenal hemorrhage epidemiology and demographics|Epidemiology and Demographics]]== |
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| * Adrenal hemorrhage occurs secondary to both traumatic conditions and atraumatic conditions.
| | == [[Adrenal hemorrhage risk factors|Risk Factors]] == |
| * Atraumatic causes of adrenal hemorrhage include:
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| ** Stress
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| ** Hemorrhagic diathesis or coagulopathy
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| ** Neonatal stress
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| ** Underlying [[:Category:Adrenal gland neoplasm | adrenal tumors]]
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| ** Idiopathic disease
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| ==Historical== | | == [[Adrenal hemorrhage screening|Screening]]== |
| Waterhouse-Friderichsen syndrome is [[eponym|named after]] Rupert Waterhouse (1873–1958), an English physician, and Carl Friderichsen (1886–1979), a Danish pediatrician, who wrote papers on the syndrome, which had been previously described.<ref name=Waterhouse_1911>{{cite journal | author = Waterhouse R | title = A case of suprarenal apoplexy | journal = [[The Lancet|Lancet]] | year = 1911 | volume = 1 |pages = 577–8 | doi = 10.1016/S0140-6736(01)60988-7 }}</ref><ref name=Friderichsen_1918>{{cite journal | author = Friderichsen C | title = Nebennierenapoplexie bei kleinen Kindern | journal = Jahrb Kinderheilk | year = 1918 | volume = 87 | pages = 109–25 }}</ref>
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| ==Differential Diagnosis== | | == [[Adrenal hemorrhage natural history, complications and prognosis|Natural History, Complications and Prognosis]] == |
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| * Adrenal adenoma
| | == Diagnosis == |
| * Adrenal carcinoma
| | [[Adrenal hemorrhage history and symptoms|History and Symptoms]] | [[Adrenal hemorrhage physical examination|Physical Examination]] | [[Adrenal hemorrhage laboratory findings|Laboratory Findings]] | [[Adrenal hemorrhage electrocardiogram| Electrocardiogram]] | [[Adrenal hemorrhage chest x ray|Chest X Ray]] | [[Adrenal hemorrhage CT|CT]] | [[Adrenal hemorrhage MRI|MRI]] | [[Adrenal hemorrhage ultrasound|Ultrasound]] | [[Adrenal hemorrhage other imaging findings|Other Imaging Studies]] | [[Adrenal hemorrhage other diagnostic studies|Other Diagnostic Studies]] |
| * Adrenal metastases
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| * Adrenal myelolipoma
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| * Genitourinary tuberculosis
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| * Neuroblastoma
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| == Symptoms == | |
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| Waterhouse-Friderichsen syndrome is the most severe form of meningococcal septicemia.
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| The onset of the illness is nonspecific with [[fever]], [[rigors]], [[vomiting]] and [[headache]]. Soon a rash appears; first macular, not much different from the rose spots of [[typhoid]], and rapidly becoming [[petechia]]l and purpuric with a dusky gray color. [[Hypotension]] is the rule and rapidly leads to septic [[shock]]. The [[cyanosis]] of extremities can be impressive and the patient is very prostrated or [[coma]]tose. In this form of meningococcal disease, [[meningitis]] generally does not occur.
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| There is [[hypoglycemia]] with [[hyponatremia]] and [[hyperkalemia]], and the Synachten test demonstrates the acute suprarenal failure. [[Leukocytosis]] need not to be extreme and in fact [[leukopenia]] may be seen and it is a very poor prognostic sign. [[CRP]] levels can be elevated or almost normal. [[Thrombocytopenia]] is sometimes extreme, with alteration in [[PT]] and [[PTT]] suggestive of [[DIC]].
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| [[Acidosis]] and [[acute renal failure]] can be seen as in any severe [[sepsis]].
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| Meningococci can be readily cultured from blood or CSF, and can sometimes be seen in smears of cutaneous lesions.
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| ==Diagnosis==
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| ===Computed Tomography===
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| *Adenral hematomas characteristically appear round or oval
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| *Stranding of the periadrenal fat is evident as well.
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| *Attenuation value of adenal hematoma depends on its age.
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| **Acute to subacute hematomas contain areas of high attenuation that usually range from 50 to 90 HU.
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| *Adrenal hematomas decrease in size and attenuation over time, and most resolve completely.
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| *Adrenal hematoma may calcify after 1 year.
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| *Organized chronic adrenal hematoma appears as a mass with a hypoattenuating center with or without calcifications. Such masses are termed '''adrenal pseudocysts'''.
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| ===Ultrasonography===
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| *Pattern of echogenicity of an adrenal hematoma depends on its age
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| **Early-stage hematoma appears solid with diffuse or inhomogeneous echogenicity.
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| **As liquefaction occurs, the mass demonstrates mixed echogenicity with a central hypoechoic region and eventually becomes completely anechoic and cystlike.
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| **Calcifications may be seen in the walls of the hematoma as early as 1–2 weeks after onset and gradually compact as the blood is absorbed.
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| *Color Doppler and power Doppler imaging allow confirmation of the avascular nature of the mass.
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| ===Magnetic Resonance Imaging===
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| *Acute stage (less than 7 days after onset): the hematoma typically appears isointense or slightly hypointense on T1-weighted images and markedly hypointense on T2-weighted images.
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| *Subacute stage (7 days to 7 weeks after onset): the hematoma appears hyperintense on T1- and T2-weighted images.
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| *Chronic stage (which typically begins 7 weeks after onset): a hypointense rim is present on T1- and T2-weighted images, which is attributed to hemosiderin deposition and the presence of a fibrous capsule.
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| == Treatment == | | == Treatment == |
| Fulminant meningococcemia is a medical emergency and need to be treated with adequate antibiotics as fast as possible. Benzyl[[penicillin]] is the drug of choice with [[chloramphenicol]] as a good alternative in allergic patients. [[Hydrocortisone]] can sometimes reverse the hypoadrenal [[shock]]. Sometimes plastic surgery and grafting is needed to deal with tissue [[necrosis]].
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| [[Ceftriaxone]] is an antibiotic commonly employed today. | | [[Adrenal hemorrhage medical therapy|Medical Therapy]] | [[Adrenal hemorrhage surgery|Surgery]] | [[Adrenal hemorrhage primary prevention|Primary Prevention]] | [[Adrenal hemorrhage cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Adrenal hemorrhage future or investigational therapies|Future or Investigational Therapies]] |
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| ==References== | | ==Case Studies== |
| {{Reflist|2}}
| | [[Adrenal hemorrhage case study one|Case #1]] |
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| ==External links==
| | {{WikiDoc Help Menu}} |
| * [http://www.whonamedit.com/synd.cfm/2187.html Whonamedit.com] page on the eponym
| | {{WikiDoc Sources}} |
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| == Acknowledgements ==
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| The content on this page was first contributed by: C. Michael Gibson, M.S., M.D.
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| {{Bacterial diseases}} | |
| {{SIB}} | |
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| [[de:Waterhouse-Friderichsen-Syndrom]]
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| [[es:Síndrome de Waterhouse-Friderichsen]]
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| [[Category:Bacterial diseases]] | | [[Category:Bacterial diseases]] |
| [[Category:Syndromes]] | | [[Category:Syndromes]] |
| [[Category:Infectious disease]]
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| [[Category:DiseaseState]] | | [[Category:Disease]] |
| [[Category:Endocrinology]] | | [[Category:Endocrinology]] |
| [[Category:Emergency medicine]] | | [[Category:Emergency medicine]] |
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| [[pl:Zespół Waterhouse'a-Friderichsena]]
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| {{WikiDoc Help Menu}}
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| {{WikiDoc Sources}}
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