Pulmonary manifestations of primary immunodeficiency disorders in children

被引:81
作者
Jesenak, Milos [1 ]
Banovcin, Peter [1 ]
Jesenakova, Barbora [1 ]
Babusikova, Eva [2 ]
机构
[1] Comenius Univ, Ctr Diag & Treatment Primary Immunodeficiencies, Jessenius Fac Med, Dept Pediat, Martin, Slovakia
[2] Comenius Univ, Dept Med Biochem, Jessenius Fac Med, Martin, Slovakia
来源
FRONTIERS IN PEDIATRICS | 2014年 / 2卷
关键词
infectious complications; inheritance; immune system dysregulation; interstitial lung diseases; respiratory tract; non-infectious complications; primary immunodeficiencies; COMMON VARIABLE IMMUNODEFICIENCY; PRIMARY IMMUNE DEFICIENCIES; INTERSTITIAL LUNG-DISEASE; NON-HODGKIN-LYMPHOMA; 10 WARNING SIGNS; COMPUTED-TOMOGRAPHY; GRANULOMATOUS-DISEASE; FOLLOW-UP; DIAGNOSIS; CT;
D O I
10.3389/fped.2014.00077
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Primary immunodeficiencies (PIDs) are inherited disorders in which one or several components of immune system are decreased, missing, or of non-appropriate function. These diseases affect the development, function, or morphology of the immune system. The group of PID comprises more than 200 different disorders and syndromes and the number of newly recognized and revealed deficiencies is still increasing. Their clinical presentation and complications depend on the type of defects and there is a great variability in the relationship between genotypes and phenotypes. A variation of clinical presentation across various age categories is also presented and children could widely differ from adult patients with PID. Respiratory symptoms and complications present a significant cause of morbidity and also mortality among patients suffering from different forms of PIDs and they are observed both in children and adults. They can affect primarily either upper airways (e.g., sinusitis and otitis media) or lower respiratory tract [e.g., pneumonia, bronchitis, bronchiectasis, and interstitial lung diseases (ILDs)]. The complications from lower respiratory tract are usually considered to be more important and also more specific for PIDs and they determinate patients' prognosis. The spectrum of the causal pathogens usually demonstrates typical pattern characteristic for each PID category. The respiratory signs of PIDs can be divided into infectious (upper and lower respiratory tract infections and complications) and non-infectious (ILDs, bronchial abnormalities - especially bronchiectasis, malignancies, and benign lymphoproliferation). Early diagnosis and appropriate therapy can prevent or at least slow down the development and course of respiratory complications of PIDs.
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页数:13
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共 95 条
[1]   Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature [J].
Agarwal, Shradha ;
Cunningham-Rundles, Charlotte .
ANNALS OF ALLERGY ASTHMA & IMMUNOLOGY, 2007, 98 (02) :185-190
[2]   Comparison of pulmonary diseases in common variable immunodeficiency and X-linked agammaglobulinaemia [J].
Aghamohammadi, Asghar ;
Allahverdi, Abdolreza ;
Abolhassani, Hassan ;
Moazzami, Kasra ;
Alizadeh, Hooman ;
Gharagozlou, Mohammad ;
Kalantari, Najmoddin ;
Sajedi, Vahid ;
Shafiei, Alireza ;
Parvaneh, Nima ;
Mohammadpour, Masoud ;
Karimi, Nasser ;
Sadaghiani, Mohammad Salehi ;
Rezaei, Nima .
RESPIROLOGY, 2010, 15 (02) :289-295
[3]   Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency [J].
Al-Herz, Waleed ;
Bousfiha, Aziz ;
Casanova, Jean-Laurent ;
Chatila, Talal ;
Conley, Mary Ellen ;
Cunningham-Rundles, Charlotte ;
Etzioni, Amos ;
Franco, Jose Luis ;
Gaspar, H. Bobby ;
Holland, Steven M. ;
Klein, Christoph ;
Nonoyama, Shigeaki ;
Ochs, Hans D. ;
Oksenhendler, Erik ;
Picard, Capucine ;
Puck, Jennifer M. ;
Sullivan, Kate ;
Tang, Mimi L. K. .
FRONTIERS IN IMMUNOLOGY, 2014, 5
[4]   Invasive aspergillosis in primary immunodeficiencies [J].
Almyroudis, NG ;
Holland, SM ;
Segal, BH .
MEDICAL MYCOLOGY, 2005, 43 :S247-S259
[5]  
Amaya-Mejia Adela Sisy, 2013, Rev Alerg Mex, V60, P110
[6]  
[Anonymous], LEK OBZ
[7]  
[Anonymous], BRONCHITIS
[8]   Ten warning signs of primary immunodeficiency: a new paradigm is needed for the 21st century [J].
Arkwright, Peter D. ;
Gennery, Andrew R. .
YEAR IN HUMAN AND MEDICAL GENETICS: INBORN ERRORS OF IMMUNITY I, 2011, 1238 :7-14
[9]   Multifocal extranodal non-Hodgkin lymphoma involving both the lungs and brain in a child with primary immunodeficiency [J].
Bal, Amanjit ;
Gupta, Anju ;
Sodhi, Kushaljit Singh ;
Das, Ashim ;
Singh, Surjit .
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, 2008, 30 (04) :317-319
[10]   Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency [J].
Bates, CA ;
Ellison, MC ;
Lynch, DA ;
Cool, CD ;
Brown, KK ;
Routes, JM .
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, 2004, 114 (02) :415-421