153:
normal levels. These deficiencies can affect only one subclass or involve an association of two subclasses, such as IgG2 and IgG4. IgG deficiencies are usually not diagnosed until the age of 12. Some of the IgG levels in the blood are undetectable and have a low percentage such as IgG4, which makes it hard to determine if a deficiency is actually present. IgG subclass deficiencies are sometimes correlated with bad responses to pneumococcal polysaccharides, especially IgG2 and or IgG4 deficiency. Some of these deficiencies are also involved with
66:
40:
235:) could be possibly responsible for the phenotype. Affected could be both males and females. Recent studies show that children with a subclass deficiency in early childhood develop normal subclass levels and the ability to make antibodies to polysaccharide vaccines as they get older. However, IgG subclass deficiencies may persist in some children and adults.
251:
is suspected, carrier detection (in which at least half of mothers are usually expected to be carriers) could be accomplished. Measurement of IgG subclass levels alone is not universally recommended, because the normal levels of different IgG subclasses values may vary between individuals and by age.
152:
IgG1 is present in the bloodstream at a percentage of about 60-70%, IgG2-20-30%, IgG3 about 5-8 %, and IgG4 1-3 %. IgG subclass deficiencies affect only IgG subclasses (usually IgG2 or IgG3), with normal total IgG and IgM immunoglobulins and other components of the immune system being at
168:
Most of the patients do not present any symptoms of the disease; however, because IgG deficiency is often combined with any other form of selective antibodies subclass deficiency, their combination could lead to a clinically significant immunodeficiency. Patients with any form of IgG subclass
184:
In healthy children, IgG2 antibodies start out low and gradually expand with age. Since IgG2 is the important component of the immune response against polysaccharides, selective IgG2 deficiency could result in recurrent infection with encapsulated bacteria.
719:
704:
797:
160:
While all the IgG subclasses contain antibodies to components of many disease-causing bacteria and viruses, each subclass serves a slightly different function in protecting the body against infection.
243:
All patients with IgG deficiency require extensive diagnostic evaluation before the patient is diagnosed with a clinically significant IgG subclass deficiency. Depending on a clinical presentation,
169:
deficiency may occasionally suffer from recurrent respiratory infections similar to the ones seen in other antibody deficiency syndromes, chiefly infections with encapsulated bacteria like
790:
371:
Dhooge IJ, van Kempen MJ, Sanders LA, Rijkers GT (June 2002). "Deficient IgA and IgG2 anti-pneumococcal antibody levels and response to vaccination in otitis prone children".
137:. It is possible to have either a global IgG deficiency, or a deficiency of one or more specific subclasses of IgG. The main clinically relevant form of IgG deficiency is IgG
783:
1210:
322:"HLA-A and -B alleles and haplotypes in 240 index patients with common variable immunodeficiency and selective IgG subclass deficiency in central Alabama"
1015:
863:
94:
1052:
227:
No significant correlation between concrete gene mutation and the IgG deficiency was discovered; the dysfunction in the regulatory elements (such as
1179:
881:
465:
636:
1184:
1119:
1036:
947:
294:
1189:
1059:
931:
221:
105:. IgG subclass deficiencies are also an integral component of other well-known primary immunodeficiency diseases, such as
926:
106:
1039:
891:
858:
247:, test for total serum immunoglobulins (IgG, IgA, IgM and subclass levels of IgG) and other tests are performed. If
1005:
110:
1025:
920:
915:
910:
905:
900:
835:
181:. An increased frequency of viral upper respiratory infections may not be an indication of antibody deficiency.
1074:
822:
734:
171:
775:
1174:
1159:
1155:
154:
277:
177:
1149:
1129:
850:
289:
1169:
873:
244:
79:
65:
145:
deficiency is not usually encountered without other concomitant immunoglobulin deficiencies, and IgG
723:
232:
228:
217:
1021:
896:
667:
617:
300:
213:
200:
is a complicated process, containing different interaction between cells and molecules (such as
990:
981:
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810:
659:
609:
574:
556:
515:
442:
388:
353:
54:
1088:
1069:
1000:
814:
806:
651:
601:
564:
546:
505:
432:
424:
380:
343:
333:
269:
248:
83:
47:
216:-induced DNA damage), and complex DNA repair machinery (including uracil-N-glycosylase and
1111:
220:
pathways)), there are many levels where malfunction could possibly result into defects in
260:
Treatment is mostly aimed on treatment and prevention of infections. Frequently used are
1164:
1064:
728:
569:
534:
494:"Chronic pediatric pulmonary disease and primary humoral antibody based immune disease"
437:
412:
273:
209:
87:
655:
384:
348:
321:
39:
1204:
605:
671:
592:
Pan Q, Hammarström L (December 2000). "Molecular basis of IgG subclass deficiency".
1140:
952:
763:
621:
739:
510:
493:
1145:
1106:
713:
428:
261:
59:
560:
188:
IgG4 subclass deficiency is very common, but mostly completely asymptomatic.
758:
265:
663:
613:
578:
519:
446:
392:
357:
696:
338:
1096:
831:
197:
276:. In most severe cases, the immunoglobulin replacement therapy (such as
971:
840:
708:
205:
201:
551:
635:
Maarschalk-Ellerbroek LJ, Hoepelman IM, Ellerbroek PM (May 2011).
779:
102:
98:
224:
mechanisms, and therefore in antibody production decrease.
97:, which may occur with or without additional decreases in
637:"Immunoglobulin treatment in primary antibody deficiency"
535:"Immunoglobulin class-switch recombination deficiencies"
466:"IgG Subclass Deficiency | Immune Deficiency Foundation"
373:
International
Journal of Pediatric Otorhinolaryngology
686:
149:
deficiency is very common but usually asymptomatic.
1128:
1105:
1087:
1035:
965:
940:
872:
849:
830:
821:
749:
690:
212:-mediated signaling), intrinsic B-cell mechanisms (
53:
29:
24:
413:"Educational paper: primary antibody deficiencies"
791:
644:International Journal of Antimicrobial Agents
320:Barton JC, Bertoli LF, Acton RT (June 2003).
93:IgG deficiency is often found in children as
8:
1016:Purine nucleoside phosphorylase deficiency
864:Transient hypogammaglobulinemia of infancy
846:
827:
798:
784:
776:
687:
95:transient hypogammaglobulinemia of infancy
64:
38:
21:
568:
550:
509:
436:
347:
337:
411:Driessen G, van der Burg M (June 2011).
33:Selective deficiency of immunoglobulin G
312:
1180:Terminal complement pathway deficiency
86:isotype are reduced relative to other
533:Durandy, Anne; Kracker, Sven (2012).
157:and have been linked to IgG4 levels.
82:where the proportional levels of the
7:
487:
485:
460:
458:
456:
406:
404:
402:
1211:Predominantly antibody deficiencies
1185:Paroxysmal nocturnal hemoglobinuria
14:
656:10.1016/j.ijantimicag.2010.11.027
948:Common variable immunodeficiency
606:10.1034/j.1600-065X.2000.17815.x
539:Arthritis Research & Therapy
295:Common variable immunodeficiency
1120:Idiopathic CD4+ lymphocytopenia
268:(as anti-inflammatory agents),
1190:Complement receptor deficiency
1060:Adenosine deaminase deficiency
417:European Journal of Pediatrics
1:
385:10.1016/S0165-5876(02)00068-X
121:IgG has four subclasses: IgG
859:X-linked agammaglobulinemia
1227:
511:10.1016/j.rmed.2010.11.013
208:interaction (resulting in
429:10.1007/s00431-011-1474-x
46:
37:
1075:Bare lymphocyte syndrome
927:Wiskott–Aldrich syndrome
492:Dosanjh A (April 2011).
172:Streptococcus pneumoniae
107:Wiskott–Aldrich syndrome
1175:Complement 3 deficiency
1160:Complement 4 deficiency
1156:Complement 2 deficiency
280:) could be considered.
196:Since the formation of
16:Form of immune disorder
178:Haemophilus influenzae
1150:Hereditary angioedema
1006:Ataxia–telangiectasia
851:Hypogammaglobulinemia
594:Immunological Reviews
339:10.1186/1471-2350-4-3
290:Hypogammaglobulinemia
164:Clinical presentation
111:ataxia–telangiectasia
1170:Properdin deficiency
991:Di George's syndrome
874:Dysgammaglobulinemia
498:Respiratory Medicine
326:BMC Medical Genetics
245:complete blood count
80:dysgammaglobulinemia
301:Hyper-IgM syndromes
1022:Hyper IgM syndrome
932:Hyper-IgE syndrome
897:Hyper IgM syndrome
813:disorders causing
750:External resources
214:cytidine deaminase
1198:
1197:
1083:
1082:
982:thymic hypoplasia
967:T cell deficiency
961:
960:
773:
772:
470:primaryimmune.org
73:
72:
19:Medical condition
1218:
1070:ZAP70 deficiency
1001:Nezelof syndrome
847:
828:
815:immunodeficiency
800:
793:
786:
777:
688:
676:
675:
641:
632:
626:
625:
589:
583:
582:
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489:
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476:
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451:
450:
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397:
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368:
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249:X-linked disease
69:
68:
48:Immunoglobulin G
42:
22:
1226:
1225:
1221:
1220:
1219:
1217:
1216:
1215:
1201:
1200:
1199:
1194:
1131:
1124:
1112:Lymphocytopenia
1110:
1101:
1079:
1055:
1042:
1031:
987:hypoparathyroid
969:
957:
936:
868:
838:
817:
804:
774:
769:
768:
745:
744:
699:
685:
680:
679:
639:
634:
633:
629:
591:
590:
586:
532:
531:
527:
491:
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483:
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472:
464:
463:
454:
410:
409:
400:
370:
369:
365:
319:
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314:
309:
286:
274:corticosteroids
258:
241:
218:mismatch repair
194:
166:
148:
144:
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136:
132:
128:
124:
119:
63:
20:
17:
12:
11:
5:
1224:
1222:
1214:
1213:
1203:
1202:
1196:
1195:
1193:
1192:
1187:
1182:
1177:
1172:
1167:
1165:MBL deficiency
1162:
1153:
1136:
1134:
1126:
1125:
1123:
1122:
1116:
1114:
1103:
1102:
1100:
1099:
1093:
1091:
1085:
1084:
1081:
1080:
1078:
1077:
1072:
1067:
1065:Omenn syndrome
1062:
1046:
1044:
1033:
1032:
1030:
1029:
1010:
1009:
1003:
994:
977:
975:
963:
962:
959:
958:
956:
955:
950:
944:
942:
938:
937:
935:
934:
929:
924:
918:
913:
908:
903:
894:
892:IgM deficiency
889:
887:IgG deficiency
884:
882:IgA deficiency
878:
876:
870:
869:
867:
866:
861:
855:
853:
844:
825:
819:
818:
805:
803:
802:
795:
788:
780:
771:
770:
767:
766:
754:
753:
751:
747:
746:
743:
742:
731:
716:
700:
695:
694:
692:
691:Classification
684:
683:External links
681:
678:
677:
650:(5): 396–404.
627:
584:
552:10.1186/ar3904
525:
504:(4): 511–514.
481:
452:
423:(6): 693–702.
398:
379:(2): 133–141.
363:
311:
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303:
298:
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285:
282:
257:
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240:
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193:
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165:
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146:
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118:
117:Classification
115:
88:immunoglobulin
76:IgG deficiency
71:
70:
57:
51:
50:
44:
43:
35:
34:
31:
27:
26:
25:IgG deficiency
18:
15:
13:
10:
9:
6:
4:
3:
2:
1223:
1212:
1209:
1208:
1206:
1191:
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1168:
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1034:
1027:
1023:
1020:
1019:
1018:
1017:
1014:
1007:
1004:
1002:
998:
997:euparathyroid
995:
992:
988:
985:
983:
979:
978:
976:
973:
968:
964:
954:
951:
949:
946:
945:
943:
939:
933:
930:
928:
925:
922:
919:
917:
914:
912:
909:
907:
904:
902:
898:
895:
893:
890:
888:
885:
883:
880:
879:
877:
875:
871:
865:
862:
860:
857:
856:
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848:
845:
842:
837:
833:
829:
826:
824:
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796:
794:
789:
787:
782:
781:
778:
765:
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760:
756:
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748:
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721:
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669:
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661:
657:
653:
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638:
631:
628:
623:
619:
615:
611:
607:
603:
600:(1): 99–110.
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571:
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562:
558:
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78:is a form of
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36:
32:
28:
23:
1141:C1-inhibitor
1139:
1056:
1049:
1012:
1011:
996:
986:
980:
953:ICF syndrome
886:
757:
733:
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473:. Retrieved
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222:class-switch
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176:
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167:
159:
155:pancreatitis
151:
120:
92:
75:
74:
1013:peripheral:
262:antibiotics
30:Other names
1146:Angioedema
1132:deficiency
1130:Complement
1107:Leukopenia
1057:autosomal:
811:complement
545:(4): 218.
475:2023-05-22
307:References
270:mucolytics
266:macrolides
198:antibodies
90:isotypes.
60:Hematology
1050:x-linked:
759:eMedicine
561:1478-6354
256:Treatment
239:Diagnosis
133:, and IgG
55:Specialty
1205:Category
1097:HIV/AIDS
1089:Acquired
1040:combined
832:Antibody
807:Lymphoid
764:med/1161
672:31107473
664:21276714
614:11213812
579:22894609
520:21144721
447:21544519
393:12049826
358:12803653
284:See also
233:enhancer
229:promoter
192:Genetics
836:humoral
823:Primary
740:D017099
622:6553947
570:3580555
438:3098982
1053:X-SCID
1037:Severe
729:279.03
670:
662:
620:
612:
577:
567:
559:
518:
445:
435:
391:
356:
349:166147
346:
297:(CVID)
206:B cell
202:T cell
62:
1043:(B+T)
941:Other
714:D80.3
668:S2CID
640:(PDF)
618:S2CID
332:: 3.
141:. IgG
129:, IgG
125:, IgG
809:and
735:MeSH
724:9-CM
660:PMID
610:PMID
575:PMID
557:ISSN
516:PMID
443:PMID
389:PMID
354:PMID
278:IVIG
272:and
210:CD40
175:and
109:and
720:ICD
705:ICD
652:doi
602:doi
598:178
565:PMC
547:doi
506:doi
502:105
433:PMC
425:doi
421:170
381:doi
344:PMC
334:doi
231:or
103:IgM
101:or
99:IgA
84:IgG
1207::
762::
738::
727::
712::
709:10
666:.
658:.
648:37
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484:^
468:.
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113:.
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989:(
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972:T
970:(
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901:1
899:(
843:)
841:B
839:(
834:/
799:e
792:t
785:v
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707:-
697:D
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383::
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336::
330:4
204:-
147:4
143:3
139:2
135:4
131:3
127:2
123:1
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