川崎氏症是什麼?
川崎氏症(Kawasaki disease,簡稱KD,也有人稱為川崎症、川崎病)舊稱黏膜皮膚淋巴結症候群,是一種主要影響中、大型血管的急性發熱性血管炎。它是一種自限性疾病,好發於嬰幼兒時期,80%的病人年齡小於四歲。在美國,其發病率為每十萬人中有5.6例。臺灣每年新增約800例川崎症病例,排名世界第三,僅次於日本和韓國。川崎症並非罕見疾病,但其診斷具有挑戰性,因為沒有特定的實驗室檢測,且其症狀常與常見的兒童感染相似。
川崎氏症的典型症狀有哪些?
川崎症的臨床症狀,常見有全身性發炎反應(例如發燒)以及黏膜皮膚發炎,此外典型的臨床反應還有雙側非滲出性結膜炎、嘴唇和口腔黏膜紅斑、皮疹、四肢末端變化和頸部淋巴結腫大。這些症狀通常在呼吸道或胃腸道症狀前驅期後出現。這些特徵性臨床症狀是川崎氏症診斷標準的基礎,以下為美國心臟協會的診斷標準,若符合就會高度懷疑罹患川崎氏症:
一定會出現:
- 持續五天或以上的發燒
五項裡面有四項:
- 雙側非滲出性結膜充血
- 口腔黏膜變化,例如嘴唇乾裂、草莓舌、咽喉發紅
- 皮膚多形性皮疹(可能出現斑丘疹、麻疹樣、蕁麻疹樣、猩紅熱樣、多形性紅斑樣、膿皰疹樣或環形紅斑樣)
- 手腳腫脹
- 頸部淋巴結腫大(直徑1.5公分或以上)
手指脫皮是川崎氏症的症狀嗎?寶寶手指脫皮,該怎麼辦?
川崎氏症病人在疾病的恢復期可能會出現肢體末端變化,這包括指甲周圍脫皮。因此,手指脫皮可能是川崎症的症狀之一,但要注意的是:並非所有手指脫皮都是川崎症。許多其他疾病,例如濕疹、黴菌感染、接觸性皮膚炎等,也會造成手指脫皮。此外,醫學文獻中也提到在疾病初期,手腳會出現腫脹和掌蹠紅斑。
如果寶寶出現手指脫皮,建議盡快帶孩子就醫,讓醫師進行評估。醫師會根據孩子的整體狀況,判斷是否需要進一步檢查,以確認或排除川崎氏症和其他疾病的可能性。切勿自行診斷或治療,以免延誤病情。
不完全型川崎氏症是什麼?
不完全行川崎氏症是指孩子雖然缺少一個或多個主要特徵,但依然會被診斷為川崎氏症病人,大約占15%–20%。
要注意的是,這些病人仍然有患心臟疾病的風險,因此即使沒有出現所有典型症狀,也應提高警覺。
川崎氏症的併發症有哪些?
川崎氏症最嚴重且常見的併發症是心臟併發症,例如冠狀動脈瘤、心肌炎、心包炎等。如果不即時治療,可能會導致心肌梗塞、心律不整、心臟衰竭等嚴重後果,甚至危及生命。錯過黃金治療期(約10天)可能導致嚴重的併發症,包括心臟病變和死亡風險。要預防併發症的關鍵就是及時診斷、治療。
如何診斷川崎氏症?
由於沒有特定的實驗室檢測,川崎氏症的診斷主要依據臨床症狀。醫師會根據孩子的症狀、病史和體格檢查結果來進行診斷。若懷疑是川崎氏症,醫師可能會安排一些檢查,例如心電圖、超音波心動圖、血管造影等,以評估心臟的狀況。由於亞洲地區川崎氏症的發生率逐年上升。衛生福利部也推動了心臟超音波和心電圖篩檢,旨在盡早發現兒童心臟中的潛在風險,減少川崎氏症的併發症。
如何治療川崎氏症?
川崎氏症的主要治療方法是靜脈注射免疫球蛋白(IVIG)和高劑量阿斯匹靈。IVIG可以有效降低冠狀動脈瘤的發生率。阿斯匹靈則具有抗發炎和抗血小板凝集的作用。早期治療對於預防心臟併發症至關重要。
總結
手指脫皮可能是川崎氏症的症狀之一,尤其是在發燒和其他川崎症相關症狀出現時,更應提高警覺。然而,許多其他疾病也會造成手指脫皮,因此,家長不必過度恐慌。若有任何疑慮,應盡速就醫,讓專業醫師進行診斷和治療。早期診斷和治療對於預防川崎症的心臟併發症至關重要。
Reference
[1] Kawasaki Disease: Global Burden and Genetic Background. /Cardiology research /2020
[2] A framework for understanding Kawasaki disease pathogenesis. /Clinical immunology (Orlando, Fla.) /2020
[3] Kawasaki or Kawasakilike disease? A debate on COVID19 infection in children. /Clinical immunology (Orlando, Fla.) /2021
[4] Bridging a diagnostic Kawasaki disease classifier from a microarray platform to a qRTPCR assay. /Pediatric research /2022
[5] A framework for understanding Kawasaki disease pathogenesis. /Clinical immunology (Orlando, Fla.) /2020
[6] Distinguishing Kawasaki Disease from Febrile Infectious Disease Using Gene Pair Signatures. /BioMed research international /2020
[7] Kawasaki or Kawasakilike disease? A debate on COVID19 infection in children. /Clinical immunology (Orlando, Fla.) /2021
[8] Agerelated differences in clinical characteristics of Kawasaki disease. /Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas /2021
[9] A Thermal Imaging Solution for Early Detection of Preulcerative Diabetic Hotspots. /Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference /2019
[10] Distinguishing Kawasaki Disease from Febrile Infectious Disease Using Gene Pair Signatures. /BioMed research international /2020
[11] Kawasaki or Kawasakilike disease? A debate on COVID19 infection in children. /Clinical immunology (Orlando, Fla.) /2021
[12] Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development. /International journal of molecular sciences /2021
[13] Kawasaki Disease: Global Burden and Genetic Background. /Cardiology research /2020
[14] Kawasaki disease: pathophysiology and insights from mouse models. /Nature reviews. Rheumatology /2020
[15] Mining incomplete clinical data for the early assessment of Kawasaki disease based on feature clustering and convolutional neural networks. /Artificial intelligence in medicine /2020
[16] Potential link between SARSCoV2 and Kawasaki disease: importance of dentists for the diagnosis. /Brazilian oral research /2021
[17] Kawasaki Disease: Global Burden and Genetic Background. /Cardiology research /2020
[18] Mining incomplete clinical data for the early assessment of Kawasaki disease based on feature clustering and convolutional neural networks. /Artificial intelligence in medicine /2020
[19] Potential link between SARSCoV2 and Kawasaki disease: importance of dentists for the diagnosis. /Brazilian oral research /2021
[20] Bridging a diagnostic Kawasaki disease classifier from a microarray platform to a qRTPCR assay. /Pediatric research /2022
[21] Distinguishing Kawasaki Disease from Febrile Infectious Disease Using Gene Pair Signatures. /BioMed research international /2020
[22] Kawasaki or Kawasakilike disease? A debate on COVID19 infection in children. /Clinical immunology (Orlando, Fla.) /2021
[23] Agerelated differences in clinical characteristics of Kawasaki disease. /Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas /2021
[24] Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development. /International journal of molecular sciences /2021