Table of Contents

HK J Paediatr (New Series)
Vol 8. No. 1, 2003

HK J Paediatr (New Series) 2003;8:15-20

Original Article

X-linked Agammaglobulinaemia in Hong Kong Chinese
香港華人的 X-連鎖無丙種球蛋白血症

RCM Lobo, GCF Chan, TL Lee, AKS Chiang, HK Ho, SY Ha, YL Lau


Abstract

We reviewed retrospectively eleven Chinese children diagnosed with X-linked agammaglobulinaemia (XLA) and managed at the Department of Paediatrics & Adolescent Medicine of Queen Mary Hospital from 1987 to 2002. All of the eleven children had presenting signs and symptoms before fourteen months old, but diagnosis was delayed in most patients with the median age of diagnosis at 5.8 years (range 1.3-14.3 years). The respiratory tract was the most commonly affected site of infection (76%) before diagnosis. Haemophilus influenzae was the most commonly isolated microorganism before and after diagnosis. Intravenous immunoglobulin (IVIG) was given in all patients with a median dose of 700 mg/kg four weekly in order to achieve the pre-infusion IgG level within the normal reference range as well as control of clinical infections. The incidence of documented infections before IVIG replacement was 31 per 100 patient-months which decreased to 7.6 per 100 patient-months after IVIG replacement (p<0.0001). The height and weight centiles of the children also increased after IVIG replacement (p<0.01). Bronchiectasis was noted in three out of the eleven children who were diagnosed to have XLA late at 6, 12 and 14 years old. Adequate IVIG replacement could decrease the frequency of infections and normalize growth in children with XLA. It might prevent bronchiectasis if started early in life, which depends on early diagnosis of XLA.

我們回顧性綜述了自 1987 年至 2002 年期間,在瑪麗醫院兒童及青少年科診斷和治療的 11 例患X-連鎖無丙種球蛋白血症(簡稱 XLA)的香港華人兒童病例,這 11 位兒童都在其年齡為 14 月大之前出現症狀和體徵,但多數病人未得到及時診斷,診斷的中位年齡為 5.8 周歲(1.3 周歲-14.3 周歲),呼吸道是診斷前最常見的感染受累部位(佔 76%),流感嗜血杆菌是診斷前後所分離到的最常見微生物,所有病人都被給予靜脈輸入免疫球蛋白(簡稱為 IVIG)進行治療,中位劑量為 700 mg/kg,每 4 周 1 次,以使患兒的 IgG 水平處於正常參考範圍內,並達到臨床控制感染的目的,IVIG 替代治療前所記錄到的病人感染發生頻率為每一百人月(patient-months)有 31 次感染,而 IVIG替代治療後降低到 7.6%(p<0.0001)。IVIG 替代治療後患兒身高和體重百分位數也增加(p<0.01),在 11 例病人中記錄到 3 例病人發生了支氣管擴張症,他們分別在 6 周歲、12 周歲和 14 周歲時被診斷為 XLA,足量 IVIG 替代治療方法能降低感染發生頻率,以及使 XLA 患兒能夠正常生長。如果在生命早期即運用這種治療措施,可能會阻止支氣管擴張症的發生,這要依賴於 XLA 的早期診斷。

Keyword : Agammaglobulinaemia; Bronchiectasis; Growth; Immunodeficiency

關鍵詞:無丙種球蛋白血症、支氣管擴張、生長、免疫缺陷

 
 

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