多中心视角下慢性肉芽肿病（CGD）的临床和人口统计学特征：马来西亚26年的经验
Clinical and demographic pattern of chronic granulomatous disease (CGD) from a multicenter perspective: Malaysia's experience over 26 years.
作者信息
Noh Lokman Mohd, Latiff Amir Hamzah Abdul, Ismail Intan Hakimah, Noah Rahim Md, Wahab Asrul Abdul, Hamid Intan Juliana Abd, Ripen Adiratna Mat, Abdullah Nasuruddin B, Razali Kamarul Azhar, Zainudin Norzila, Bakon Florence, Kok Long Juan, Ali Adli, Aziz Bilkis Banu SAbd, Latif Hasniah Abdul, Mohamad Siti Mardhiana, Zainudeen Zarina Thasneem, Hashim Ilie Fadzilah, Sendut Iean Hamzah, Nadarajaw Thiyagar, Jamil Faizah Mohamed, Ng David C E, Abidin Mohd Azri Zainal
机构信息
Department of Paediatrics, Hospital Tunku Azizah, Ministry of Health Malaysia, Kuala Lumpur, Malaysia.
Department of Paediatrics, UKM, Kuala Lumpur, Malaysia.
出版信息
Allergy Asthma Clin Immunol. 2021 May 17;17(1):50. doi: 10.1186/s13223-021-00551-4.
BACKGROUND
A retrospective review of clinical manifestations and demographic pattern of patients diagnosed as chronic granulomatous disease (CGD) from 7 hospitals in Malaysia. An analysis of the available database would establish clinical characteristics, diagnoses and outcome including microbiologic pattern. Studying the demography allows us to document the occurrence of CGD amongst multiethnic groups and its geographical distribution for Malaysia.
METHODS
Data from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed.
RESULTS
Twenty patients were diagnosed as CGD. Males (N = 13, 65%) outnumber females (N = 7, 35%). CGD is commonest amongst the Malays (65%) followed by the Chinese (15.0%), Indians (10.0%) and natives of Borneo (10.0%), reflecting the ethnic composition of the country. The mean age of diagnosis was 3.7 years. There was a positive family history in 40% of the cases. Abscess was the main presenting feature in 16 patients (80%) with one involving the brain. Pneumonia occurred in 10 (50%) and one with complicated bronchiectasis. Catalase-positive bacteria were the most commonly isolated pathogen with Chromobacterium violaceum predominating (N = 5, 25%) with consequent high mortality (N = 4, 80%). All CGD patients with C. violaceum infection displayed CD4 + (T helper cells) lymphopenia.
CONCLUSION
This study has shown CGD occurs in the major ethnic groups of Malaysia. To the best of our knowledge, this is the first and the largest series of chronic granulomatous disease in South East Asia which may be reflective of similar clinical pattern in the region. C. violaceum infection is associated with a higher mortality in CGD patients in Malaysia. All the CGD patients with C. violaceum infection in this patient series displayed CD4 + (T helper) lymphopenia. We recorded rare clinical manifestation of CGD viz. brain abscess and bronchiectasis.
背景
对马来西亚7家医院诊断为慢性肉芽肿病（CGD）的患者的临床表现和人口统计学模式进行回顾性研究。对现有数据库进行分析将确定临床特征、诊断及结局，包括微生物学模式。研究人口统计学情况可使我们记录CGD在马来西亚多民族群体中的发病情况及其地理分布。
方法
整理并分析马来西亚原发性免疫缺陷网络（MyPIN）1991年至2016年诊断为CGD的病例数据。
结果
20例患者被诊断为CGD。男性（n = 13，65%）多于女性（n = 7，35%）。CGD在马来人中最为常见（65%），其次是华人（15.0%）、印度人（10.0%）和婆罗洲原住民（10.0%），这反映了该国的种族构成。诊断时的平均年龄为3.7岁。40%的病例有家族史阳性。16例患者（80%）的主要表现为脓肿，其中1例累及脑部。10例（50%）发生肺炎，1例合并支气管扩张。过氧化氢酶阳性细菌是最常分离出的病原体，其中紫色杆菌占主导（n = 5，25%），导致高死亡率（n = 4，80%）。所有感染紫色杆菌的CGD患者均表现为CD4 +（辅助性T细胞）淋巴细胞减少。
结论
本研究表明CGD在马来西亚的主要种族群体中均有发生。据我们所知，这是东南亚首个也是最大的慢性肉芽肿病系列研究，可能反映了该地区类似的临床模式。在马来西亚，紫色杆菌感染与CGD患者的较高死亡率相关。本患者系列中所有感染紫色杆菌的CGD患者均表现为CD4 +（辅助性T）淋巴细胞减少。我们记录到了CGD罕见的临床表现，即脑脓肿和支气管扩张。
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