Waterhouse-Friderichsen syndrome

ウォーターハウス・フリーデリクセン症候群

WordNet

1. a pattern of symptoms indicative of some disease
2. a complex of concurrent things; "every word has a syndrome of meanings"

PrepTutorEJDIC

1. (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/12/08 10:03:17」(JST)

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Waterhouse–Friderichsen syndrome
Classification and external resources
ICD-10	A39.1, E35.1
ICD-9	036.3
DiseasesDB	29316
MedlinePlus	000609
eMedicine	med/3009
MeSH	D014884

Waterhouse–Friderichsen 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 (or rarely viral) infection (most commonly the meningococcus Neisseria meningitidis).

The bacterial infection leads to massive hemorrhage into one or (usually) both adrenal glands. It is characterized by overwhelming bacterial infection meningococcemia leading to massive blood invasion, organ failure, coma, low blood pressure and shock, disseminated intravascular coagulation (DIC) with widespread purpura, rapidly developing adrenocortical insufficiency and death.

Signs and symptoms

WFS is the most severe form of meningococcal septicemia. 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 petechial and purpuric with a dusky gray color. Low blood pressure (hypotension) is the rule and rapidly leads to septic shock. The cyanosis of extremities can be impressive and the patient is very prostrated or comatose. In this form of meningococcal disease, meningitis generally does not occur. There is hypoglycemia with hyponatremia and hyperkalemia, and the ACTH stimulation test demonstrates the acute adrenal failure. Leukocytosis need not to be extreme and in fact leukopenia may be seen and it is a very poor prognostic sign. C-reactive protein levels can be elevated or almost normal. Thrombocytopenia is sometimes extreme, with alteration in prothrombin time (PT) and partial thromboplastin time (PTT) suggestive of diffuse intravascular coagulation (DIC). Acidosis and acute renal failure can be seen as in any severe sepsis. Meningococci can be readily cultured from blood or CSF, and can sometimes be seen in smears of cutaneous lesions. Dysphagia, atrophy of the tongue, and cracks at the corners of the mouth are also characteristic features.

Causes

Multiple species of bacteria can be associated with the condition:

• Meningococcus is another term for the bacterial species Neisseria meningitidis; blood infection with said species usually underlies WFS. While many infectious agents can infect the adrenals, an acute, selective infection is usually Meningococcus.
• Pseudomonas aeruginosa can also cause WFS.
• WFS can also be caused by Streptococcus pneumoniae infections, a common bacterial pathogen typically associated with meningitis in the adult and elderly population.
• Mycobacterium tuberculosis could also cause WFS. Tubercular invasion of the adrenal glands could cause hemorrhagic destruction of the glands and cause mineralocorticoid deficiency.
• Staphylococcus aureus has recently also been implicated in pediatric WFS.
• It can also be associated with Haemophilus influenzae.
• Cytomegalovirus can cause adrenal insufficiency, especially in the immunocompromised.

Ebola virus infection may also cause similar acute adrenal failure.

Prevention

Routine vaccination against meningococcus is recommended by the Centers for Disease Control and Prevention for all 11–18 year olds and people who have poor splenic function (who, for example, have had their spleen removed or who have sickle-cell disease which damages the spleen), or who have certain immune disorders, such as a complement deficiency.

Treatment

Fulminant meningococcemia is a medical emergency and needs to be treated with adequate antibiotics as fast as possible. Benzylpenicillin was once the drug of choice with chloramphenicol as a good alternative in allergic patients. Ceftriaxone is an antibiotic commonly employed today. Hydrocortisone can sometimes reverse the hypoadrenal shock. Sometimes plastic surgery and grafting is needed to deal with tissue necrosis.

Historical

Waterhouse–Friderichsen syndrome is 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.

References

External links

• Whonamedit.com page on the eponym

UpToDate Contents

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• 1. 髄膜炎菌感染の臨床症状 clinical manifestations of meningococcal infection
• 2. 原発性副腎不全（アジソン病）の原因 causes of primary adrenal insufficiency addisons disease
• 3. 成人における副腎不全の臨床症状 clinical manifestations of adrenal insufficiency in adults
• 4. 髄膜炎菌感染の治療および予防 treatment and prevention of meningococcal infection
• 5. 黄色ブドウ球菌感染症の臨床症状 clinical manifestations of staphylococcus aureus infection

English Journal

• Fatal dog bite in the absence of significant trauma: Capnocytophaga canimorsus infection and unexpected death.

• Stiegler D, Gilbert JD, Warner MS, Byard RW.SourceMedical University of Vienna, Vienna, Austria.
• The American journal of forensic medicine and pathology." abstractLink="yes" alsec="jour" alterm="Am J Forensic Med Pathol.2010 Jun;31(2):198-9.
• Sudden and unexpected nontraumatic death in individuals with asplenia or hyposplenia is usually due to fulminant bacterial sepsis, most often involving Streptococcus pneumoniae, Neisseria meningitidis, and Hemophilus influenzae. We report a case of a previously well 40-year-old man who died 5 hours
• PMID 20407357

• PMID 19858241

Japanese Journal

• 非髄膜炎菌性 Waterhouse-Friderichsen 症候群を発症した2型糖尿病患者の1剖検例

• 白神 敦久,七條 加奈,川内 千徳,関本 悦子,柴田 泰伸,重清 俊雄,斉藤 慎一郎,森本 訓明,樋口 幸夫,佐竹 宣法
• 糖尿病 52(9), 799-804, 2009-09-30
• NAID 10025606846

• 脾臓低形成・糖尿病例に発症した劇症型肺炎球菌感染症による Waterhouse-Friderichsen 症候群の一例

• 深光 岳,古賀 まゆみ,河岡 徹,有好 邦夫,石原 得博,小田 梨恵
• 山口医学 58(3), 105-110, 2009-06-30
• NAID 10025981437

Related Links

• Waterhouse-Friderichsen syndromeとは - コトバンク

世界大百科事典 第2版 - Waterhouse-Friderichsen syndromeの用語解説 - 上気道感染様症状の発症後，まず敗血症の症状として全身に直径1～3mmの紅斑が出現，2～3日で急速に消退し，ついで髄膜刺激症状や脳神経麻痺が出現する。

• Waterhouse-Friderichsen syndrome: MedlinePlus Medical ...

Rarely, Waterhouse-Friderichsen syndrome can be caused by the use of medications that promote blood clotting. Other causes include: Low platelet counts Primary antiphospholipid syndrome Renal vein thrombosis Steroid use ...

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リンク元	「ウォーターハウス・フリーデリクセン症候群」
関連記事	「syndrome」

「ウォーターハウス・フリーデリクセン症候群」

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英

Waterhouse-Friderichsen syndrome

同

ウォーターハウス-フリーデリクセン症候群、Waterhouse-Friderichsen症候群。ウォーターハウス・フリードリクセン症候群。Waterhause Friderichsen症候群？

概念

• 髄膜炎菌敗血症など播種性血管内凝固症候群、全身の皮膚・粘膜出血、副腎出血を来たす。24時間以内に死亡しうる。

• 病原体：Neisseria meningitidis >> Pseudomonas aeruginosa, Streptococcus pneumoniae, Haemophilus influenzae(APT.363)
• 病因：exdotoxin-induced vasculitis and DIC(APT.363)
• 病態：(Neisseria meningitidis)mild pharyngitis -> florid septicemia with hypotension and shock with in hours -> destruction of adrenal corten -> adrenal insufficiency(APT.363)

「syndrome」

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• n.

• 症候群