WordNet

any of a large group of nitrogenous organic compounds that are essential constituents of living cells; consist of polymers of amino acids; essential in the diet of animals for growth and for repair of tissues; can be obtained from meat and eggs and milk and legumes; "a diet high in protein"
something lost (especially money lost at gambling) (同)losses
a disease of the intestinal tract

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蛋白(たんばく)質
負ける,損をする,勝ち目がない

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/04/07 18:32:31」(JST)

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Protein-losing enteropathy refers to any condition of the gastrointestinal tract (e.g. damage to the gut wall) that results in a net loss of protein from the body.^[1]

Signs and symptoms

The signs/symptoms of protein losing enteropathy are consistent with diarrhea, fever, and general abdominal discomfort.^[1] Swelling of the legs due to peripheral edema can also occur, however if the PLE is related to a systemic disease such as congestive heart failure or constrictive pericarditis, then the symptoms could be of the primary disease development.^[2]

Causes

The causes of protein-losing enteropathy can include GI conditions (among other causes) like the following :^[3]

Inflammatory bowel disease.
Idiopathic ulcerative jejunoileitis.
Infection (secondary obstruction)
Neoplasm (secondary obstruction)
Sarcoidosis (secondary obstruction).
Amyloidosis.
Systemic lupus erythematosus (SLE).
Ménétrier's disease.
Zollinger-Ellison syndrome.
Eosinophilic gastroenteritis.
Coeliac disease.
Primary intestinal lymphangectasia

Mechanism

Proteoglycan

The pathophysiology of protein losing enteropathy is a result of plasma proteins loss, which enters GI tract (lumen).^[2] PLE is a complication of a disorder, be it:lymphatic obstruction or mucosal injury.^[4]

In pediatric protein losing enteropathy there are several changes in epithelial cells causing PLE by augmenting the rate of flow of proteins (serum). Congenital molecular abnormalities, or dysfunctional lymphatic drainage might cause epithelial matrix changes.Proteoglycans, which are glycosaminoglycan chains attached to protein, directly causes PLE, as well as, augments inflammatory cytokines. Children who have congenital glycosylation defects usually have protein losing enteropathy.^[5]^[6]

Diagnosis

The diagnosis of protein-losing enteropathy is made by excluding other causes of protein loss. Endoscopy can be used to localize the cause of the protein loss in the bowel. Different methods include faecal excretion of alpha 1-antitrypsin which is a marker of protein-losing enteropathy, as well as, viral serologies which may be useful to detect PLE.^[3]

Treatment

Treatment for protein losing enteropathy depends upon the underlying condition, according to Rychik, et al this could mean treatment of hypoproteinemia or of the intestinal mucosa.^[7]

In terms of treatment for PLE after the Fontan operation treatment must be equal to the level of hypoproteinemia present. Therefore, it is useful to categorize patients based on their serum albumin levels, if less than normal (typically less than 3.5 g/dL) but greater than 2.5 g/dL, this can be seen as a mild form of protein losing enteropathy. Symptomatic management of edema with furosemide (and aldactone) can provide relief for the individual with mild hypoproteinemia.^[8]^[9]

References

^ ^a ^b "Protein-losing enteropathy: MedlinePlus Medical Encyclopedia". www.nlm.nih.gov. Retrieved 2015-12-16.
^ ^a ^b "Protein-Losing Enteropathy: Background, Pathophysiology, Etiology".
^ ^a ^b "Protein-losing Enteropathy. PLE information. What is PLE? | Patient". Patient. Retrieved 2016-02-19.
^ Wyllie, Robert; Hyams, Jeffrey S.; Kay, Marsha (2015-08-03). Pediatric Gastrointestinal and Liver Disease. Elsevier Health Sciences. p. 389. ISBN 9780323240994.
^ Iozzo, Renato V.; Schaefer, Liliana (2015-03-01). "Proteoglycan form and function: A comprehensive nomenclature of proteoglycans". Matrix Biology 42: 11–55. doi:10.1016/j.matbio.2015.02.003. – via ScienceDirect (Subscription may be required or content may be available in libraries.)
^ "Pediatric Protein-Losing Enteropathy: Background, Pathophysiology, Epidemiology".
^ Rychik, Jack; Spray, Thomas L. (2002-01-01). "Strategies to treat protein-losing enteropathy". Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 5 (1): 3–11. doi:10.1053/pcsu.2002.31498. – via ScienceDirect (Subscription may be required or content may be available in libraries.)
^ Rychik, Jack (2007-09-01). "Protein-Losing Enteropathy after Fontan Operation". Congenital Heart Disease 2 (5): 288–300. doi:10.1111/j.1747-0803.2007.00116.x. ISSN 1747-0803.
^ Topol, Eric J.; Califf, Robert M. (2007-01-01). Textbook of Cardiovascular Medicine. Lippincott Williams & Wilkins. p. 519. ISBN 9780781770125.

UpToDate Contents

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1. 蛋白漏出性胃腸症 protein losing gastroenteropathy
2. Management of patients post-Fontan procedure
3. 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
4. 全身性エリテマトーデスの消化器症状 gastrointestinal manifestations of systemic lupus erythematosus
5. 小児における浮腫の評価およびマネージメント evaluation and management of edema in children

English Journal

Chronic diarrhea, ascites, and protein-losing enteropathy in an infant with hepatic venous outflow obstruction after liver transplantation.

Hourigan SK, Anders RA, Mitchell SE, Schwarz KB, Lau H, Karnsakul W.SourceDivision of Pediatric Gastroenterology Department of Pathology Division of Interventional Radiology Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Pediatric transplantation.Pediatr Transplant.2012 Nov;16(7):E328-31. doi: 10.1111/j.1399-3046.2012.01686.x. Epub 2012 Apr 10.
Hourigan SK, Anders RA, Mitchell SE, Schwarz KB, Lau H, Karnsakul W. Chronic diarrhea, ascites, and protein-losing enteropathy in an infant with hepatic venous outflow obstruction after liver transplantation. Abstract: An 18-month-old female status post-orthotopic liver transplant for biliary atr
PMID 22489846

In-111 transferrin scintigraphy in cirrhosis with hypoalbuminemia: Evidence for protein-losing enteropathy in a small group of selected cases.

Dahlqvist GE, Jamar F, Zech F, Geubel AP.SourceDepartment of Hepato-gastroenterology, St Luc University Hospital, Université Catholique de Louvain , 1200 Brussels , Belgium.
Scandinavian journal of gastroenterology.Scand J Gastroenterol.2012 Oct;47(10):1247-52. Epub 2012 Jul 2.
Abstract Background. Hypoalbuminemia commonly observed in cirrhosis is considered to be mainly related to hepatocellular dysfunction. However, the correlation between the decrease in serum albumin and liver function is far from linear and arguments in favor of an additive role of protein-losing
PMID 22989334

Japanese Journal

Intestinal uptake of ^<99m>Tc-MDP : a case report of protein-losing enteropathy correlated with pathology findings from autopsy

MARTINEZ-SANCHIS Begona,CORTES-VIZCAINO Virginia,FRONTADO-MORALES Lorena,SOPENA-NOVALES Pablo
Annals of nuclear medicine 25(2), 139-141, 2011-02-01
NAID 10028999207

Related Pictures

★リンクテーブル★

リンク元	「蛋白漏出性胃腸症」「蛋白喪失性腸症」「タンパク質喪失性腸症」「タンパク質漏出性腸症」

「蛋白漏出性胃腸症」

Protein-losing enteropathy
Classification and external resources
ICD-10	K90.4
ICD-9-CM	579.8
OMIM	226300
DiseasesDB	10811
MedlinePlus	002277
eMedicine	med/1926 ped/1909
MeSH	D011504
	[edit on Wikidata]

　　[★]

英: protein-losing gastroenteropathy PLGE, proteinlosing gastroenteropathy
同: タンパク質漏出性胃腸症、滲出性腸症 exudative enteropathy、本態性低タンパク血症 essential hypoproteinemia、異化亢進性低タンパク血症 hypercatabolic hypoproteinemia
関: 低タンパク血症; 蛋白漏出性腸症 protein-losing enteropathy、蛋白喪失性腸症、タンパク質喪失性腸症、タンパク質漏出性腸症

[show details]

タンパク漏出性胃腸疾患 : 6 件
タンパク喪失性胃腸疾患 : 11 件
蛋白漏出性胃腸疾患 : 13 件
蛋白喪失性胃腸疾患 : 9 件

蛋白漏出性胃腸症 : 約 14,700 件

概念

病態の総称

消化管粘膜より管腔にタンパク質(主にアルブミン) が漏出する為に、低蛋白血症を伴った病態。主症状は浮腫である。吸収不良症候群とのoverlapが多い

病態生理に基づく病因の分類

YN.A-87 参考1

1. 粘膜における蛋白質の透過性亢進

メネトリエ病(Ménétrier病)、腸管内細菌増殖、全身エリテマトーデス、アミロイドーシス、バッド・キアリ症候群、クロンカイト・カナダ症候群

2. 炎症：びらん・潰瘍により蛋白質が漏出する。さらに全層性の腸管病変(悪性腫瘍腫瘍、クローン病)によりリンパ管が閉塞し、リンパが漏出する機序もある→3.

非特異性小腸潰瘍、クローン病、消化管腫瘍、腸炎(偽膜性腸炎など)

3. 血流・リンパのうっ滞

収縮性心膜炎、うっ血性心不全、肝硬変、腸リンパ管拡張症、悪性リンパ腫

検査

血液生化学

アルブミン：低下
A/G比：正常　　→　　アルブミン低値ながらA/G比が正常なのが特徴。

免疫血清学

免疫グロブリン：低下　　→　　免疫グロブリンも漏出してしまう

糞便検査：α1アンチトリプシンクリアランス試験(α1-アンチトリプシン消化管クリアランス試験)：血清蛋白中の約4%を占めるATの糞便中排泄量を求める試験。腸粘膜からの蛋白漏出の程度をみる。20mL/日以上の排泄があれば陽性と診断する。

YN A-87

低蛋白血症、低アルブミン血症、カルシウム低下、鉄低下、鉄欠乏性貧血、リンパ球減少、好酸球増加

鑑別

蛋白漏出性胃腸症：蛋白↓、コレステロール↓
ネフローゼ症候群：蛋白↓、コレステロール↑

参考

1. [charged] Protein-losing gastroenteropathy - uptodate [1]

「蛋白喪失性腸症」

　　[★]

英: protein-losing enteropathy
関: 蛋白漏出性腸症、タンパク質喪失性腸症、タンパク質漏出性腸症

「タンパク質喪失性腸症」

　　[★]

英: protein-losing enteropathy
関: 蛋白喪失性腸症、蛋白漏出性腸症、タンパク質漏出性腸症

「タンパク質漏出性腸症」

　　[★]

英: protein-losing enteropathy
関: 蛋白喪失性腸症、蛋白漏出性腸症、タンパク質喪失性腸症

[nih-1] "Protein-losing enteropathy: MedlinePlus Medical Encyclopedia". www.nlm.nih.gov. Retrieved 2015-12-16.

[emed-2] "Protein-Losing Enteropathy: Background, Pathophysiology, Etiology".

[pat-3] "Protein-losing Enteropathy. PLE information. What is PLE? | Patient". Patient. Retrieved 2016-02-19.

[4] Wyllie, Robert; Hyams, Jeffrey S.; Kay, Marsha (2015-08-03). Pediatric Gastrointestinal and Liver Disease. Elsevier Health Sciences. p. 389. ISBN 9780323240994.

[5] Iozzo, Renato V.; Schaefer, Liliana (2015-03-01). "Proteoglycan form and function: A comprehensive nomenclature of proteoglycans". Matrix Biology 42: 11–55. doi:10.1016/j.matbio.2015.02.003. – via ScienceDirect (Subscription may be required or content may be available in libraries.)

[6] "Pediatric Protein-Losing Enteropathy: Background, Pathophysiology, Epidemiology".

[7] Rychik, Jack; Spray, Thomas L. (2002-01-01). "Strategies to treat protein-losing enteropathy". Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual 5 (1): 3–11. doi:10.1053/pcsu.2002.31498. – via ScienceDirect (Subscription may be required or content may be available in libraries.)

[8] Rychik, Jack (2007-09-01). "Protein-Losing Enteropathy after Fontan Operation". Congenital Heart Disease 2 (5): 288–300. doi:10.1111/j.1747-0803.2007.00116.x. ISSN 1747-0803.

[9] Topol, Eric J.; Califf, Robert M. (2007-01-01). Textbook of Cardiovascular Medicine. Lippincott Williams & Wilkins. p. 519. ISBN 9780781770125.

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案内

案内

protein-losing enteropathy