Distribution and clinical features of primary immunodeficiency diseases in Chinese children (2004-2009).
Two hundred and one patients have been diagnosed with primary immunodeficiency diseases (PIDs) in our center from January 2004 to December 2009. The male-to-female ratio was 5.29:1. Spectrums of PIDs were as follows: predominantly antibody deficiency disease was the most common category (94 patients, 48.2%), followed by other well-defined immunodeficiency syndromes (40 patients, 20.5%), combined T and B cell immunodeficiencies (33 patients, 16.9%), congenital defects of phagocyte number and/or function (21 patients, 10.8%), and diseases of immune dysregulation (six patients, 3.1%). Agammaglobulinemia was the most frequent disease type. The median of diagnosis lag was 18.0 months. Pneumonia was the most common manifestation of PID patients. Some manifestations were prone to concentrate in certain diseases. As for therapy, 99 patients (50.8%) received intravenous immunoglobulin replacement therapy; 13 patients received hematopoietic stem cell transplantation and nine of them were still alive. In this study, we sought to describe and analyze the distribution, clinical features, and therapy methods of PIDs among children diagnosed in our country and to compare with reports from other countries and regions.
Department of Pediatrics, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China.
Lin-Lin
LL
Wang
Ying-Ying
YY
Jin
Yi-Qun
YQ
Hao
Juan-Juan
JJ
Wang
Chun-Mei
CM
Yao
Xi
X
Wang
Rui-Ming
RM
Cao
Hui
H
Zhang
Yi
Y
Chen
Tong-Xin
TX
Chen
D000293:Adolescent; D000361:Agammaglobulinemia / Q000453:epidemiology / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D000900:Anti-Bacterial Agents / Q000494:pharmacology; D044466:Asian People; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000453:epidemiology / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D003241:Consanguinity; D005190:Family; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007132:Immunoglobulin Isotypes / Q000032:analysis; D007136:Immunoglobulins / Q000276:immunology / Q000494:pharmacology*; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008297:Male; D010585:Phagocyte Bactericidal Dysfunction / Q000453:epidemiology / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012189:Retrospective Studies; D016511:Severe Combined Immunodeficiency / Q000453:epidemiology / Q000276:immunology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D015996:Survival Rate
D000900:Anti-Bacterial Agents; D007132:Immunoglobulin Isotypes; D007136:Immunoglobulins
J Clin Immunol. 2007 Sep;27(5):517-24
17588141
Semin Arthritis Rheum. 2010 Feb;39(4):257-68
19136143
Clin Exp Immunol. 2007 Feb;147(2):306-12
17223972
J Clin Immunol. 2007 Jan;27(1):101-8
17191150
Clin Immunol. 2002 Sep;104(3):221-30
12217331
J Clin Immunol. 2007 Sep;27(5):497-502
17577648
J Clin Immunol. 2006 Nov;26(6):519-32
17024564
Cell Mol Immunol. 2009 Dec;6(6):397-406
20003815
J Clin Immunol. 2008 Mar;28(2):186-93
18008151
J Allergy Clin Immunol. 2002 Apr;109(4):581-91
11941303
J Clin Immunol. 2010 Jan;30(1):10-6
20101521
J Allergy Clin Immunol. 2007 Oct;120(4):776-94
17952897
Clin Exp Rheumatol. 2004 Nov-Dec;22(6):776-80
15638056
J Clin Immunol. 2010 Jan;30(1):121-31
19904586
Singapore Med J. 2003 Nov;44(11):579-86
15007498
Ann Saudi Med. 1999 May-Jun;19(3):206-10
17283454
J Clin Immunol. 1997 Jul;17(4):333-9
9258772
J Clin Immunol. 2005 Mar;25(2):162-73
15821893
Yonsei Med J. 2008 Feb 29;49(1):28-36
18306466
Clin Immunol. 1999 Jul;92(1):34-48
10413651
J Clin Immunol. 2007 May;27(3):308-16
17510807
Lancet. 2001 Apr 7;357(9262):1093-4
11297962
Bone Marrow Transplant. 2008 Jan;41(2):119-26
17968328
Clin Exp Immunol. 2009 Sep;157 Suppl 1:3-11
19630863
Clin Immunol. 2010 May;135(2):264-72
20399414
Medicine (Baltimore). 2006 Jul;85(4):193-202
16862044
J Clin Immunol. 1981 Jan;1(1):31-9
7334068
J Clin Pathol. 2008 Sep;61(9):994-1000
18755724
Trends Immunol. 2006 Jan;27(1):17-23
16290228
J Gastroenterol Hepatol. 2010 Mar;25(3):453-68
20370724
J Clin Immunol. 2006 May;26(3):274-83
16783466
JAMA. 2006 Feb 1;295(5):508-18
16449616
J Clin Immunol. 1998 Sep;18(5):368-71
9793829
J Allergy Clin Immunol. 2009 Dec;124(6):1161-78
20004777
Ann Hum Biol. 2004 Mar-Apr;31(2):263-9
15204368
Bone Marrow Transplant. 2006 Mar;37(5):469-77
16435016
Clin Exp Immunol. 2003 Nov;134(2):314-20
14616793
J Allergy Clin Immunol. 2005 Aug;116(2):426-30
16083801
Curr Opin Pediatr. 2007 Dec;19(6):685-92
18025937
Clin Immunol. 2004 Dec;113(3):234-40
15507387
J Clin Immunol. 2009 May;29(3):343-51
19002574
Clin Exp Immunol. 2006 Aug;145(2):204-14
16879238
Br J Haematol. 2009 Jun;145(6):709-27
19344423
Nat Rev Immunol. 2003 Jul;3(7):521-33
12876555
J Pediatr. 2003 Oct;143(4):525-31
14571234