capillary leak syndrome

WordNet

1. tell anonymously; "The news were leaked to the paper"
2. a euphemism for urination; "he had to take a leak" (同)wetting, making water, passing water
3. have an opening that allows light or substances to enter or go out; "The container leaked gasoline"; "the roof leaks badly"
4. soft watery rot in fruits and vegetables caused by fungi
5. unauthorized (especially deliberate) disclosure of confidential information (同)news leak
6. an accidental hole that allows something (fluid or light etc.) to enter or escape; "one of the tires developed a leak"
7. be leaked; "The news leaked out despite his secrecy" (同)leak out
8. enter or escape as through a hole or crack or fissure; "Water leaked out of the can into the backpack"; "Gas leaked into the basement"
9. of or relating to hair
10. a tube of small internal diameter; holds liquid by capillary action (同)capillary tube, capillary tubing
11. any of the minute blood vessels connecting arterioles with venules (同)capillary_vessel
12. long and slender with a very small internal diameter; "a capillary tube" (同)hairlike
13. a pattern of symptoms indicative of some disease
14. a complex of concurrent things; "every word has a syndrome of meanings"
15. a unit of length of thread or yarn

PrepTutorEJDIC

1. 〈U〉〈C〉(水・空気・ガス・光などが)漏れること / 〈C〉『漏れ口』,漏れ穴 / 〈C〉(秘密などの)漏えい《+『of』+『名』》 / 〈船・屋根・容器などが〉漏る / (…から,…の中へ)〈水・空気・ガス・光など〉漏れる《+『from』(『into』)+『名』》 / 〈秘密などが〉漏えいする《+『out』》 / 〈水・空気・ガス・光など〉‘を'漏らす / (…に)〈秘密など〉‘を'漏らす《+『名』+『to』+『名』》
2. 毛状の,毛のように細い / 毛細管の / 毛細管
3. (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
4. 草原,牧草地(meadow)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/12/11 12:09:54」(JST)

wiki en

[Wiki en表示]

Capillary leak syndrome
Classification and external resources
DiseasesDB	34090
MeSH	D019559

Capillary leak syndrome (also known as systemic capillary leak syndrome, SCLS, or Clarkson’s disease) is an extremely rare medical condition characterized by self-reversing episodes during which the endothelial cells which line the capillaries are thought to separate for a few days, allowing for a leakage of fluid from the circulatory system to the interstitial space, resulting in a dangerous hypotension (low blood pressure), hemoconcentration, and hypoalbuminemia. It is a life-threatening illness because each episode has the potential to cause damage to, or the failure of, vital organs due to limited perfusion. It is often misdiagnosed as polycythemia, polycythemia vera, or sepsis.

History

The syndrome was first described by B. Clarkson in 1960, after whom it was later informally named. Beyond numerous case reports published since then, two comprehensive reviews of clinical and research experience were published in 2010.

Symptoms

Most patients report having a runny nose and/or other flu-like symptoms, or else gastro-intestinal disorders (diarrhea or vomiting), or a general weakness or pain in their limbs, but others get no particular or consistent warning signs ahead of their episode. They subsequently develop thirst and lightheadedness and the following measurable conditions:

• hemoconcentration (Ht up to 80-85%);
• low blood pressure (hypotension);
• hypoalbuminemia;
• partial or generalized edema;
• possible MGUS.

Treatment

An episode of SCLS usually consists of two distinct phases:

The capillary leak phase

The initial stage is the capillary leak phase, lasting from 1 to 3 days, during which up to 70% of total plasma volume may invade cavities in the trunk and extremities. The most common clinical features are fatigue; lightheadedness up to and including syncope (fainting); limb, abdominal or generalized pain; facial or other edema; dyspnea; and hypotension that results in circulatory shock and potentially in cardiopulmonary collapse and other organ distress or damage. Acute renal failure is a risk due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.

The loss of fluid out of the capillaries has similar effects on the circulation as dehydration, slowing both the flow of oxygen delivered to tissues and organs as well as the output of urine. Urgent medical attention in this phase consists of fluid resuscitation efforts, mainly the intravenous administration of saline solution plus hetastarch or albumin and colloids (to increase the remaining blood flow to vital organs like the kidneys), as well as glucocorticoids (steroids like methylprednisolone, to reduce or stop the capillary leak). However, it is important to avoid overly aggressive intravenous fluid administration during this leak phase, because it may cause massive swelling of the extremities and thus serious collateral damage because of induced compartment syndromes.

The recruitment phase

The second stage features the reabsorption of the initially extravasated fluid and it usually lasts just as long as the leak phase. Intravascular fluid overload leads to polyuria and can cause flash pulmonary edema, with possibly fatal consequences. The severity of the problem depends on to the quantity of fluid supplied in the initial phase, the damage that may have been sustained by the kidneys, and the promptness with which diuretics are administered to help the patient discharge the accumulated fluids quickly.

The prevention of episodes of SCLS has involved two approaches. The first has long been identified with the Mayo Clinic, and it recommends treatment with beta agonists such as terbutaline, phosphodiesterase inhibitor theophylline and leukotriene receptor antagonists montelukast sodium. The second, more recent approach pioneered in France involves monthly intravenous infusions of intravenous immunoglobulin (IVIG), and the growing case-report evidence is encouraging.

A recent review of clinical experience with 28 European SCLS patients suggests that either prophylactic treatment may reduce the frequency and severity of attacks and may improve survival.

Prognosis

In the Mayo Clinic’s experience, the median survival of 25 patients that were followed over 30 years (counting only SCLS-related deaths) was approximately 15 years, and their 5-year survival rate was 76%. In European experience, the 5-year post-diagnosis survival rate was 85% in 23 patients who had received prophylactic treatment and 20% in 5 patients who had not. However, better identification and management of this condition appears to be resulting in lower mortality and improving survival and quality-of-life results as of late.

References

External links

• Mayo Clinic overview, diagnosis and treatment information.
• Druey, Kirk M.; Greipp, Philip R. (2010). "Narrative Review: Clarkson Disease-Systemic Capillary Leak Syndrome". Annals of Internal Medicine 153 (2): 90–8. doi:10.1059/0003-4819-153-2-201007200-00005 (inactive 2014-03-09). PMC 3017349. PMID 20643990. 
• ClinicalTrials.gov NCT00936325 Studies in the Pathogenesis of Systemic Capillary Leak Syndrome
• National Organization for Rare Disorders.
• Systemic Capillary Leak Syndrome Community
• Overview (2003) at Orphanet

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 特発性全身性毛細血管漏出症候群 idiopathic systemic capillary leak syndrome
• 2. 特発性浮腫 idiopathic edema
• 3. 成人における確定診断を得られていない低血圧やショックの評価および初期アプローチ evaluation of and initial approach to the adult patient with undifferentiated hypotension and shock
• 4. 成人における浮腫の病態生理および病因 pathophysiology and etiology of edema in adults
• 5. 全身性抗腫瘍治療に関連する肺毒性：臨床症状、診断、および治療 pulmonary toxicity associated with systemic antineoplastic therapy clinical presentation diagnosis and treatment

English Journal

• Capillary leak syndrome: initial presentation in a patient with ALK+?anaplastic large cell lymphoma associated with increased levels of serum cytokines.

• Umemoto T, Watanabe T, Ogose T, Kondo R, Nakatsu T, Sakata A, Yamashita M, Gotho T.SourceDivisions of Pediatrics, Tokushima Red Cross Hospital, Tokushima, Japan.
• Leukemia & lymphoma.2011 Jun;52(6):1139-42. Epub 2011 Apr 4.
• PMID 21463119

Japanese Journal

• 今月の症例 Systemic capillary leak syndrome(SCLS)の1例

• 日本内科学会雑誌 105(1), 92-98, 2016-01-10
• NAID 40020712335

• 全身性毛細血管漏出症候群 (特集 血管生物学の進歩と血液内科)

• 血液内科 69(3), 328-335, 2014-09
• NAID 40020207464

• 今月の症例 急激な膿疱化に伴いcapillary leak syndromeを併発した尋常性乾癬の1例

• 臨床皮膚科 = Japanese journal of clinical dermatology 68(9), 674-678, 2014-08
• NAID 40020180291

Related Links

• Idiopathic systemic capillary leak syndrome

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder characterized by episodes of severe hypotension, hypoalbuminemia, and hemoconcentration. During attacks of ISCLS, profound derangements of the vascular ...

• Systemic capillary leak syndrome - Mayo Clinic

Systemic capillary leak syndrome — Learn about diagnosis and treatment options at Mayo Clinic. ... Systemic capillary leak syndrome is a rare disorder characterized by recurrent flares of massive leakage of plasma and other blood ...

Related Pictures

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★リンクテーブル★

リンク元	「毛細管漏出症候群」「毛細血管漏出症候群」
拡張検索	「systemic capillary leak syndrome」
関連記事	「leak」「leaking」「capillary」「syndrome」

「毛細管漏出症候群」

　　[★]

英

capillary leak syndrome

関

毛細血管漏出症候群、全身性毛細管漏出症候群

「毛細血管漏出症候群」

　　[★]

英

capillary leak syndrome

関

毛細管漏出症候群

「systemic capillary leak syndrome」

　　[★]

全身性毛細管漏出症候群

関

capillary leak syndrome

「leak」

　　[★]

• n.

• 漏出、漏れ、漏洩、リーク

• v.

• 漏れる、漏出させる、リークさせる

関

diapedesis、discharge、escape、leakage

　 　 　 　 　 　 　 　 　

「leaking」

　　[★]

• adj.

• 漏出性の

関

leaky

「capillary」

　　[★] 毛細血管 　 　 　 　 　

「syndrome」

　　[★]

• n.

• 症候群