関: capillary leak syndrome

WordNet

(physical chemistry) a sample of matter in which substances in different phases are in equilibrium; "in a static system oil cannot be replaced by water on a surface"; "a system generating hydrogen peroxide"
instrumentality that combines interrelated interacting artifacts designed to work as a coherent entity; "he bought a new stereo system"; "the system consists of a motor and a small computer"
a group of independent but interrelated elements comprising a unified whole; "a vast system of production and distribution and consumption keep the country going" (同)scheme
a complex of methods or rules governing behavior; "they have to operate under a system they oppose"; "that language has a complex system for indicating gender" (同)system of rules
a group of physiologically or anatomically related organs or parts; "the body has a system of organs for digestion"
a procedure or process for obtaining an objective; "they had to devise a system that did not depend on cooperation"
the living body considered as made up of interdependent components forming a unified whole; "exercise helped him get the alcohol out of his system"
tell anonymously; "The news were leaked to the paper"
a euphemism for urination; "he had to take a leak" (同)wetting, making water, passing water
have an opening that allows light or substances to enter or go out; "The container leaked gasoline"; "the roof leaks badly"
soft watery rot in fruits and vegetables caused by fungi
unauthorized (especially deliberate) disclosure of confidential information (同)news leak
an accidental hole that allows something (fluid or light etc.) to enter or escape; "one of the tires developed a leak"
be leaked; "The news leaked out despite his secrecy" (同)leak out
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"
of or relating to hair
a tube of small internal diameter; holds liquid by capillary action (同)capillary tube, capillary tubing
any of the minute blood vessels connecting arterioles with venules (同)capillary_vessel
long and slender with a very small internal diameter; "a capillary tube" (同)hairlike
a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
affecting an entire system; "a systemic poison"

PrepTutorEJDIC

〈C〉(関連した部分から成る)『体系』,系統,組織[網],装置 / 〈C〉(教育・政治などの)『制度』,機構;《the~》体制 / 〈C〉(思想・学問などの)『体系』,学説 / 〈C〉(…の)『方法』,方式,やり方《+of doing》 / 〈U〉正しい方針(筋道,順序) / 〈U〉《the~》(身体の)組織,系統 / 〈U〉《the~,one's~》身体,全身
〈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』》
毛状の,毛のように細い / 毛細管の / 毛細管
(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
(身体・言語のように)体系の,組織の / 全身の,全身的

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/03/02 17:50:54」(JST)

wiki en

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,^[1] 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.^[2]^[3]

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:^[2]^[3]

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:^[2]^[3]

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.^[2] 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.^[2]^[3] Acute renal failure is a risk due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.^[2]^[3]

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).^[2] 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.^[3]

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.^[2]^[3] 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.^[2]

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.^[4] The second, more recent approach pioneered in France involves monthly intravenous infusions of intravenous immunoglobulin (IVIG), and the growing case-report evidence is encouraging.^[5]^[6]^[7]^[8]

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.^[9]

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%.^[2]^[3] 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.^[9] 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

^ Clarkson, Bayard; Thompson, David; Horwith, Melvin; Luckey, E.Hugh (1960). "Cyclical edema and shock due to increased capillary permeability". The American Journal of Medicine 29 (2): 193–216. doi:10.1016/0002-9343(60)90018-8. PMID 13693909.
^ ^a ^b ^c ^d ^e ^f ^g ^h ⁱ ^j 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 2015-01-11). PMC 3017349. PMID 20643990.
^ ^a ^b ^c ^d ^e ^f ^g ^h Kapoor, Prashant; Greipp, Patricia T.; Schaefer, Eric W.; Mandrekar, Sumithra J.; Kamal, Arif H.; Gonzalez-Paz, Natalia C.; Kumar, Shaji; Greipp, Philip R. (2010). "Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience". Mayo Clinic Proceedings 85 (10): 905–12. doi:10.4065/mcp.2010.0159. PMC 2947962. PMID 20634497.
^ Droder, RM; Kyle, RA; Greipp, PR (1992). "Control of systemic capillary leak syndrome with aminophylline and terbutaline". The American Journal of Medicine 92 (5): 523–6. doi:10.1016/0002-9343(92)90749-2. PMID 1580299.
^ Lambert, Marc; Launay, David; Hachulla, Eric; Morell-Dubois, Sandrine; Soland, Vincent; Queyrel, Viviane; Fourrier, François; Hatron, Pierre-Yves (2008). "High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome". Critical Care Medicine 36 (7): 2184–7. doi:10.1097/CCM.0b013e31817d7c71. PMID 18552679.
^ Abgueguen, Pierre; Chennebault, Jean Marie; Pichard, Eric (2010). "Immunoglobulins for Treatment of Systemic Capillary Leak Syndrome". The American Journal of Medicine 123 (6): e3–4. doi:10.1016/j.amjmed.2009.09.034. PMID 20569743.
^ Zipponi, Manuel; Eugster, Roland; Birrenbach, Tanja (2011). "High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome". BMJ Case Reports 2011: bcr1220103599. doi:10.1136/bcr.12.2010.3599. PMC 3089932. PMID 22696704.
^ Pecker, Mark; Adams, Michael; Graham, Walter (2011). "The Systemic Capillary Leak Syndrome: Comment". Annals of Internal Medicine 155 (5): 335. doi:10.1059/0003-4819-155-5-201109060-00017 (inactive 2015-01-11). PMID 21893630.
^ ^a ^b Gousseff, Marie; Arnaud, Laurent; Lambert, Marc; Hot, Arnaud; Hamidou, Mohamed; Duhaut, Pierre; Papo, Thomas; Soubrier, Martin; et al. (2011). "The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry". Annals of Internal Medicine 154 (7): 464–71. doi:10.1059/0003-4819-154-7-201104050-00004 (inactive 2015-01-11). PMID 21464348.

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 2015-01-11). PMC 3017349. PMID 20643990.
Clinical trial number NCT00936325 for "Studies in the Pathogenesis of Systemic Capillary Leak Syndrome" at ClinicalTrials.gov
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. 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
3. 特発性浮腫 idiopathic edema
4. POEMS症候群 poems syndrome
5. 小児における浮腫の病態生理および病因 pathophysiology and etiology of edema in children

English Journal

POEMS syndrome: Importance of the clinical laboratory practitioner's role.

Levinson SS.AbstractBACKGROUND: Polyneuropathy organomegaly, endocrinopathy, monoclonal (M) gammopathy, skin syndrome (POEMS) may be difficult to diagnose as a result of methodological and clinical idiosyncrasies. Four of eleven criteria (M-protein, VEGF, endocrinopathy and thrombocytosis/polycythemia) are closely associated with clinical laboratory testing. POEMS has been largely associated with λ-M-gammopathies. Vascular endothelial growth factor (VEGF) is a recent addition to the major diagnostic criteria. VEGF may alter vascular permeability causing some manifestations of POEMS.
Clinica chimica acta; international journal of clinical chemistry.Clin Chim Acta.2012 Nov 12;413(21-22):1800-7. Epub 2012 Jul 3.
BACKGROUND: Polyneuropathy organomegaly, endocrinopathy, monoclonal (M) gammopathy, skin syndrome (POEMS) may be difficult to diagnose as a result of methodological and clinical idiosyncrasies. Four of eleven criteria (M-protein, VEGF, endocrinopathy and thrombocytosis/polycythemia) are closely asso
PMID 22771968

Down-regulation of aquaporin 1 in rats with experimental acute necrotizing pancreatitis.

Feng DX, Peng W, Chen YF, Chen T, Tian JY, Si HR, Cai J, Rao Y, Han F, Zhao R.SourceFrom the *Department of General Surgery and †Laboratory Center, Putuo Hospital, Shanghai University of Traditional Chinese Medicine, Shanghai, China; and ‡Department of Surgery, Royal University Hospital, College of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
Pancreas.Pancreas.2012 Oct;41(7):1092-8.
OBJECTIVES: The objectives of this study were to investigate the expression of aquaporin 1 in capillary endothelial cells of rats with experimental acute necrotizing pancreatitis (ANP) and to explore its pathogenic role in capillary leak.METHODS: Sixty-four male Sprague-Dawley rats were randomly div
PMID 22481286

Japanese Journal

A Case of Idiopathic Systemic Capillary Leak Syndrome with High Serum Levels of G-CSF on Exacerbation

Nakagawa Naoki,Ota Hisanobu,Tanabe Yasuko,Kabara Maki,Matsuki Motoki,Chinda Junko,Sakamoto Naka,Fujino Takayuki,Takehara Naofumi,Takeuchi Toshiharu,Kawabe Jun-ichi,Sato Nobuyuki,Kawamura Yuichiro,Fukuhara Takashi,Ikuta Katsuya,Kikuchi Kenjiro,Hasebe Naoyuki
Internal Medicine 50(6), 597-600, 2011
… Systemic capillary leak syndrome (SCLS) is a life-threatening disorder which presents with periodic episodes of hypovolemic shock, due to plasma leakage to the extra-vascular space reflected by accompanying hypoalbuminemia, hemoconcentration and edema often with associated monoclonal gammopathy. …
NAID 130000649892

Synchronized Changes in Serum Vascular Endothelial Growth Factor during the Clinical Course of Chronic Systemic Capillary Leak Syndrome

Kinoshita Yoshiaki,Kasaoka Shunji,Fujita Motoki,Oshima Chiyomi,Kawamura Yoshikatsu,Tsuruta Ryosuke,Maekawa Tsuyoshi
Internal Medicine 49(8), 791-794, 2010
… Systemic capillary leak syndrome (SCLS) is a rare disease characterized by leakage of plasma from blood vessels into the interstitial space due to increased capillary permeability. … We describe a 24-year-old man who was hospitalized with systemic edema, hypoalbuminemia, and disseminated intravascular coagulation. …
NAID 130000251869

Related Pictures

★リンクテーブル★

リンク元	「浮腫」「全身性毛細管漏出症候群」
関連記事	「leak」「systemic」「system」「leaking」「capillary」

「浮腫」

Capillary leak syndrome
Classification and external resources
DiseasesDB	34090
MeSH	D019559
	[edit on Wikidata]

　　[★]

英: edema
関: 水腫、angioedema、全身性浮腫

分類

	圧痕性浮腫		非圧痕性浮腫
	pitting edema		nonpitting edema
病態	水のみが間質に貯留圧痕を残す		水分＋血漿由来物質の蓄積(ムコ多糖、蛋白質）・炎症細胞の浸潤圧痕を残さない
疾患	fast edema	slow edema	甲状腺機能低下症局所性炎症(蜂窩織炎、虫さされ) 強皮症
	低アルブミン血症	心不全腎不全

低アルブミン血症はfast edema　と覚えておく

浮腫の原因

IMD.518

全身性

心性浮腫

うっ血性心不全

腎性浮腫

肝性浮腫

内分泌性浮腫
栄養失調性浮腫・栄養障害性浮腫
薬剤性浮腫
起立性浮腫
特発性浮腫

局所性

内科診断リファレンス p.4

循環血漿量の増加：心不全、腎疾患(急性腎炎、腎不全)、薬剤(ミノキシジル、NSAID、ピオグリタゾン、フルドロコルチゾン、エストロゲン、甘草)、refeeding edema
低アルブミン血症：ネフローゼ症候群、蛋白漏出性胃腸症、肝疾患、低栄養
血管透過性亢進：熱傷、外傷、炎症・敗血症、アレルギー反応(血管性浮腫など)、好酸球増多、糖尿病、IL-2治療、ビタミンB1欠乏。血管内増殖因子(systemic capillary leak syndrome, POEMS症候群, Castleman病などの血管疾患、卵巣過剰刺激症候群)
リンパ管閉塞：リンパ節切除、癌のリンパ管浸潤
間質膠質浸透圧増加：甲状腺機能低下症
静脈還流不全：深部静脈血栓症、IVC閉塞、SVC症候群、静脈弁不全
その他：カルシウム拮抗薬(細動脈抵抗の低下)、特発性浮腫(血管透過性亢進＋循環血漿量増加)