Department of Pediatrics, Laboratory of Pediatric Infectious Diseases, Radboud University Medical Centre, Nijmegen, The Netherlands.
Pediatr Infect Dis J. 2013 Apr;32(4):399-404. doi: 10.1097/INF.0b013e31827db77a.
Children with primary immunodeficiencies, sickle cell disease and cystic fibrosis are at risk to develop invasive bacterial infections caused by respiratory tract pathogens, in particular Streptococcus pneumoniae, Haemophilus influenzae and Staphylococcus aureus. This review article evaluates the role of nasopharyngeal colonization by these pathogens in the high prevalence of respiratory and invasive infections in children with inherited disorders affecting the immune system or the respiratory tract. We conclude that respiratory and invasive diseases that occur in children with primary immunodeficiencies or sickle cell disease are probably a result of increased nasopharyngeal colonization rates compared with healthy children. However, when the inherited disorder is characterized by local airway abnormalities such as in cystic fibrosis, enhanced nasopharyngeal colonization does not seem to play a major role in invasive disease risk. As the evidence for the role of nasopharyngeal colonization in disease risk in these specific patient groups partly comes from experimental studies and animal models, longitudinal studies in children are needed. Detailed understanding of the effect of colonization on the development of respiratory and invasive infections in children with primary immunodeficiencies, sickle cell disease or cystic fibrosis provides a justification for the selective introduction of vaccination and prophylactic antibiotic treatment. Recommendations for the use of (preventive) therapeutic strategies in these patient groups taking into account disease-specific immunologic mechanisms underlying colonization and disease are described.
患有原发性免疫缺陷、镰状细胞病和囊性纤维化的儿童易发生由呼吸道病原体引起的侵袭性细菌感染，特别是肺炎链球菌、流感嗜血杆菌和金黄色葡萄球菌。本文综述了这些病原体鼻咽定植在免疫系统或呼吸道遗传性疾病儿童中呼吸道和侵袭性感染高发中的作用。我们得出结论，与健康儿童相比，原发性免疫缺陷或镰状细胞病儿童发生的呼吸道和侵袭性疾病可能是由于鼻咽定植率增加所致。然而，当遗传性疾病的特征是气道局部异常，如囊性纤维化时，鼻咽定植似乎在侵袭性疾病风险中不起主要作用。由于这些特定患者群体中鼻咽定植在疾病风险中的作用的证据部分来自于实验研究和动物模型，因此需要对儿童进行纵向研究。详细了解定植对原发性免疫缺陷、镰状细胞病或囊性纤维化儿童发生呼吸道和侵袭性感染的影响，为有选择地接种疫苗和预防性抗生素治疗提供了依据。描述了考虑到定植和疾病背后的特定免疫机制，在这些患者群体中使用（预防性）治疗策略的建议。
