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乳糜瀉

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乳糜瀉（英語：coeliac disease 或 celiac disease）又稱麩質敏感性腸病（gluten-sensitive enteropathy）[15]，是具有遺傳傾向，對含麥膠食物不耐受而導致的腸源性吸收障礙的小腸自體免疫性疾病[10][16]。

乳糜瀉
Coeliac disease
又稱麥膠性腸病（gluten enteropathy）、非熱帶脂肪瀉（non-tropical steatorrhea）
乳糜瀉患者的小腸絨毛切片，顯示小腸絨毛消失，陷窩肥厚且淋巴細胞浸潤。
讀音
徵狀無徵狀或非特異性徵狀、腹脹、腹瀉、便秘、吸收不良、體重下降、疱疹性皮膚炎（英語：dermatitis herpetiformis）[1][2]
併發症缺鐵性貧血、骨質疏鬆症、不孕、癌症、神經系統疾病、其他自體免疫性疾病[3][4][5][6][7]
起病年齡任何年齡[1][8]
病程終身[6]
類型autoimmune disease of gastrointestinal tract[*]、麩質相關疾病[*]、疾病
病因對麩質的反應[9]
診斷方法家族病史（英語：Family history (medicine)）、血液抗體檢測、腸道活檢、基因檢測、對移除麩質的反應[10][11]
鑑別診斷炎症性腸病、腸道寄生蟲、腸激躁症、囊腫性纖維化[12]
治療無麩質飲食[13]
盛行率約為1/135[14]
分類和外部資源
醫學專科胃腸學、內科學
ICD-11DA95
ICD-9-CM579.0
OMIM609754、​612008、​612005、​612006、​607202、​611598、​612007、​612011、​612009
DiseasesDB2922
MedlinePlus000233
eMedicine932104、​373864
Orphanet555
[編輯此條目的維基數據]

典型的徵狀包含胃腸道徵狀，像是慢性腹瀉、腹脹、吸收不良、降低食慾，以及使孩童生長遲緩（英語：failure to thrive）。這個病通常發生在六個月大到兩歲之間[1]。不典型的徵狀比較常見，尤其是病人年紀大於兩歲時[8][17][18]。腸胃道徵狀可能輕微或沒有表現，另外也可能會有許多徵狀影響到身體任何部位（英語：systemic disease），或甚至是沒有顯著徵狀表現[1]。乳糜瀉剛開始是被發現於孩童身上[8][6]，但其實任意年紀都可以發病[1][8]。此病常常和其他自體免疫性疾病共病，如一型糖尿病和甲狀腺炎等[6]。

機制

編輯

乳糜瀉是因人體不適應麩質進而引發過敏反應的結果。麩質是小麥、大麥及裸麥等穀物含有的一組蛋白質[19][20][21]。一般而言，適量的食用燕麥，若其中無摻混其他含有麩質的穀物，通常不會對兒童患者造成影響[20][22]，而食用後會出現乳糜瀉問題的概率則可能與於燕麥的品種有關[20][23]。

此症是遺傳性疾病[24]，患者攝取到麩質後，身體裏的異常免疫系統對此產生反應，並可能導致生成數種自身抗體而影響許多不同的器官[25][26]。在小腸中，自身抗體會引發炎症反應，並可能造成生長於小腸內壁的絨毛長度變短（絨毛萎縮）[24][27]，而這會影響營養吸收，患者因而經常貧血[24][21]。

診斷

編輯

確診通常非常困難，大部分患者在正確診斷前都經歷較長的時間[28]。現在有多個檢測手段可以使用。患者徵狀的嚴重程度可能決定了這些檢測的順序，但如果患者已經在進行無麩質飲食，則所有上述的檢測都無效。小腸的損傷通常在飲食中去掉麩質後幾周開始恢復，而抗體的水平也在數月後下降。對於那些正在進行無麩質飲食的人群，可能需要在其飲食中每日有一餐加入含麩質的食物，持續六周後再進行檢測。[29]

診斷一般是透過血液抗體測試及腸道活體組織切片進行，會用特殊的基因檢測作為輔助[10]。不過診斷不容易直接進行[28]，多半血液中的抗體檢驗是陰性的[30][31]，而腸道健康的絨毛也只有少許變化[32]。病患在確診前多半已有嚴重徵狀，且持續了幾年[33][34]。目前越來越多的確診案例來自於無徵狀患者的篩檢結果[35]。不過目前尚無足夠實證佐證篩檢的效果[36]。此病症是由對於麩質蛋白質的永久不耐所造成[10]，和更罕見的小麥過敏（英語：wheat allergy）不同[37]。

治療

編輯

目前認為對此病唯一有效的治療方式是讓病患採取嚴格的終生無麩質飲食。這樣可讓大多數的患者腸粘膜復元、改善徵狀並降低產生併發症的風險[13]。如果不加以治療，此病將可能導致如腸道淋巴瘤等癌症，並會略為增加早期死亡的風險[3]。世界各地罹患此病的人口比率各不相同，從1/300人至1/40不等，平均則是約在1/100至1/170間[14]。據估計，罹有此病者中有高達80％並未被確診出來，通常是因為他們的胃腸道徵狀極為輕微或根本沒有出現徵狀，以及因為大眾缺乏對於徵狀和診斷標準的知識所致[5][33][38]。統計上，此病的女性患者較男性略多一些[39]。

參考文獻

編輯
  1. ^ 1.0 1.1 1.2 1.3 1.4 Fasano A. Clinical presentation of celiac disease in the pediatric population. Gastroenterology (Review). April 2005, 128 (4 Suppl 1): S68–73. PMID 15825129. doi:10.1053/j.gastro.2005.02.015. 
  2. ^ Symptoms & Causes of Celiac Disease | NIDDK. National Institute of Diabetes and Digestive and Kidney Diseases. June 2016 [24 April 2017]. （原始內容存檔於2017-04-24）.  （頁面存檔備份，存於互聯網檔案館）
  3. ^ 3.0 3.1 Lebwohl B, Ludvigsson JF, Green PH. Celiac disease and non-celiac gluten sensitivity. BMJ (Review). October 2015, 351: h4347. PMC 4596973  . PMID 26438584. doi:10.1136/bmj.h4347. Celiac disease occurs in about 1% of the population worldwide, although most people with the condition are undiagnosed. It can cause a wide variety of symptoms, both intestinal and extra-intestinal because it is a systemic autoimmune disease that is triggered by dietary gluten. Patients with coeliac disease are at increased risk of cancer, including a twofold to fourfold increased risk of non-Hodgkin’s lymphoma and a more than 30-fold increased risk of small intestinal adenocarcinoma, and they have a 1.4-fold increased risk of death. 
  4. ^ 引用錯誤：沒有為名為Lund2015的參考文獻提供內容
  5. ^ 5.0 5.1 Celiac disease. World Gastroenterology Organisation（英語：World Gastroenterology Organisation） Global Guidelines. July 2016 [23 April 2017]. （原始內容存檔於2017-03-17）. 
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  9. ^ 引用錯誤：沒有為名為TovoliMasi2015的參考文獻提供內容
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  11. ^ 引用錯誤：沒有為名為VivasVaquero2015的參考文獻提供內容
  12. ^ Ferri, Fred F. Ferri's differential diagnosis : a practical guide to the differential diagnosis of symptoms, signs, and clinical disorders 2nd. Philadelphia, PA: Elsevier/Mosby. 2010: Chapter C. ISBN 978-0323076999. 
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  14. ^ 14.0 14.1 Fasano A, Catassi C. Clinical practice. Celiac disease. The New England Journal of Medicine (Review). December 2012, 367 (25): 2419–26. PMID 23252527. doi:10.1056/NEJMcp1113994. 
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  22. ^ Pinto-Sánchez MI, Causada-Calo N, Bercik P, Ford AC, Murray JA, Armstrong D, Semrad C, Kupfer SS, Alaedini A, Moayyedi P, Leffler DA, Verdú EF, Green P. Safety of Adding Oats to a Gluten-Free Diet for Patients With Celiac Disease: Systematic Review and Meta-analysis of Clinical and Observational Studies (PDF). Gastroenterology. August 2017, 153 (2): 395–409.e3 [2020-02-20]. PMID 28431885. doi:10.1053/j.gastro.2017.04.009. （原始內容存檔 (PDF)於2021-01-29）. 
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外部連結

編輯
 
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