柯士文症候群之牙科處置：兩篇病例報告

Dental Management of Patients with Kostmann Syndrome: Two Case Reports

張伯森(Po-Sen Chang) ； 蔡蔭玲(Aileen I Tsai) ； 莊麗娟(Li-Chuan Chuang) ； 黃慈睿(Tzu-Jui Huang) ； 徐嘉璘(Chia-Lin Hsu)

《臺灣兒童牙醫學雜誌》 20卷1期 (2020/03) Pp. 41-49

https://doi.org/10.6319/TJPD.202003_20(1).0006

摘要

嗜中性白血球是顆粒性白血球的一種，也是人體內數量最多的白血球。嗜中性白血球在免疫功能扮演重要的角色，主要是藉由吞噬作用來抵禦外來感染源的侵入。嗜中性白血球減少症指血液中絕對嗜中性白血球數目低於1500/mm^3。柯士文症候群是一種骨髓細胞形成障礙，無法在骨髓中順利分化成嗜中性白血球，絕對嗜中性白血球數目會減少至500/mm^3以下，甚至常常低於200/mm^3。因此柯士文症候群的病患細菌感染的機率會增加。本病例報告指出牙醫師在處置有關嗜中性白球減少症時之重要性，以及口腔衛生指導的意義和定期回診的重要。

關鍵字

柯士文症候群 ； 先天性嗜中性白血球減少症 ； 顆粒性白血球群落刺激因子 ； 造血幹細胞移植

並列摘要

Neutrophils are a type of granulocyte, and the most abundant type of white blood cells in humans. Their main function, vital to the human immune system, is resisting the invasion of foreign infection sources through phagocytosis. Neutropenia occurs when the absolute neutrophil count (ANC) drops below 1500/mm^3. Kostmann syndrome is a disorder of myelopoiesis and characterized by an impaired ability to differentiate neutrophils in bone marrow with an ANC chronically below 500/mm^3, usually below 200/mm^3. Kostmann syndrome is often associated with an increased susceptibility to bacterial infections. This case report emphasizes the importance of dentists in managing diseases linked with neutrophil and highlights the significance of instructing oral hygiene and the importance of timely dental checkups.

並列關鍵字

Kostmann syndrome ； congenital neutropenia ； granulocyte colony stimulating factor ； hematopoietic stem cell transplantation