Hong Kong Journal of Paediatrics

Volume 8 Number 1, January 2003
Abstract

X-linked Agammaglobulinaemia in Hong Kong Chinese

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

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. (HK J Paediatr (new series) 2003;8:15-20)

Key words : Agammaglobulinaemia; Bronchiectasis; Growth; Immunodeficiency



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