73:
49:
320:(IVIG) to treat SIgAD, but the consensus is that there is no evidence that IVIG treats this condition. In cases where a patient presents SIgAD and another condition which is treatable with IVIG, then a physician may treat the other condition with IVIG. The use of IVIG to treat SIgAD without first demonstrating an impairment of specific antibody formation is not recommended.
121:
85â90% of IgA-deficient individuals are asymptomatic, although the reason for lack of symptoms is relatively unknown and continues to be a topic of interest and controversy. Some patients with IgA deficiency have a tendency to develop recurrent sinopulmonary infections, gastrointestinal infections
363:
Prevalence varies by population, but is on the order of 1 in 100 to 1 in 1000 people, making it relatively common. SIgAD occurs in 1 in 39 to 1 in 57 people with celiac disease. This is much higher than the prevalence of selective IgA deficiency in the general population. It is also significantly
354:
Patients with
Selective IgA deficiency rarely have severe reactions to blood transfusions. Although Selective IgA deficiency is common, severe reactions to blood transfusions are very rare. People with selective IgA deficiency do not require special blood products unless they have a history of a
307:
The treatment consists of identification of co-morbid conditions, preventive measures to reduce the risk of infection, and prompt and effective treatment of infections. Infections in an IgA-deficient person are treated as usual (i.e., with antibiotics). There is no treatment for the underlying
298:
When suspected, the diagnosis can be confirmed by laboratory measurement of IgA level in the blood. SIgAD is an IgA level < 7 mg/dL with normal IgG and IgM levels (reference range 70â400 mg/dL for adults; children somewhat less).
1645:
1630:
122:
and disorders, allergies, autoimmune conditions, and malignancies. These infections are generally mild and would not usually lead to an in-depth workup except when unusually frequent. They rarely present with severe reactions, including
227:, a part of the body's defenses against infection at the body's surfaces (mainly the surfaces of the respiratory and digestive systems). As a result, bacteria at these locations are somewhat more able to cause disease.
2971:
1756:
105:
that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract. It is defined as an undetectable serum IgA level in the presence of normal serum levels of
2181:
2673:
1749:
2890:
924:
909:
113:, in persons older than 4 years. It is the most common of the primary antibody deficiencies. Most such persons remain healthy throughout their lives and are never diagnosed.
950:
Francisco A. Bonilla, I. Leonard
Bernstein, David A. Khan, Zuhair K. Ballas, Javier Chinen, Michael M. Frank, Lisa J. Kobrynski, Arnold I. Levinson, Bruce Mazer (May 2005).
2809:
1742:
216:âIn IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgAâ. âIn IgA deficiency, B cells express IgA; however, they are of immature
2529:
2174:
2233:
790:
Vosughimotlagh A, Rasouli SE, Rafiemanesh H, Safarirad M, Sharifinejad N, Madanipour A, Dos Santos Vilela MM, HeropolitaĆska-Pliszka E, Azizi G (2023-08-28).
2976:
2274:
2690:
2167:
2686:
2258:
1974:
1822:
2939:
2715:
2590:
2011:
2556:
2341:
308:
disorder. All SIgAD patients, even if asymptomatic, should receive pneumococcal and influenza vaccines, but should avoid live attenuated vaccines.
2775:
2318:
2269:
1568:
Weber-Mzell D, Kotanko P, Hauer AC, et al. (March 2004). "Gender, age and seasonal effects on IgA deficiency: a study of 7293 Caucasians".
2138:
2594:
2570:
1176:"Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined Danish and Swedish study"
2915:
2830:
2792:
2387:
2364:
406:
2483:
2455:
2439:
2143:
1660:
717:
2565:
1127:"Validation of an In-House Assay for Cytomegalovirus Immunoglobulin G (CMV IgG) Avidity and Relationship of Avidity to CMV IgM Levels"
1109:
951:
2902:
2678:
2238:
1416:
2078:
2763:
2751:
1995:
1906:
344:
173:
169:
150:
1456:"Guideline on the investigation and management of acute transfusion reactions Prepared by the BCSH Blood Transfusion Task Force"
2950:
2880:
2633:
2246:
1228:"Review: IgA anaphylactic transfusion reactions. Part I. Laboratory diagnosis, incidence, and supply of IgA-deficient products"
2517:
2701:
2545:
2306:
2148:
2018:
1890:
2981:
2885:
2848:
2814:
1885:
2927:
1998:
1850:
1817:
913:
139:
2802:
2476:
2464:
524:"T and B lymphocyte subpopulations and activation/differentiation markers in patients with selective IgA deficiency"
2865:
2648:
2199:
1440:
317:
199:. Selective IgA deficiency is often inherited, but fewer than half of all cases but has been associated with some
131:
1964:
2739:
2313:
2294:
2226:
1984:
1879:
1874:
1869:
1864:
1859:
1794:
2608:
2656:
2418:
2359:
2033:
1781:
1671:
1344:"Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management"
157:
1529:"Celiac Disease and IgA Deficiency: Complications of Serological Testing Approaches Encountered in the Clinic"
1303:"Hypersensitivity transfusion reactions due to IgA deficiency are rare according to French hemovigilance data"
184:
Selective IgA deficiency is inherited in less than half of cases, but has been associated with differences in
165:
1734:
1273:
1271:
2661:
2496:
2133:
2118:
2114:
2408:
2402:
2242:
1383:
463:
Koskinen S (1996). "Long-term follow-up of health in blood donors with primary selective IgA deficiency".
2869:
2262:
2190:
2108:
2088:
1809:
1010:
Perez EE, Orange JS, Bonilla F, Chinen J, Chinn IK, Dorsey M, El-Gamal Y, Harville TO, Hossny E (2017).
94:
220:
with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cellsâ.
2290:
2221:
2128:
1832:
672:
1454:
Tinegate H, Birchall J, Gray A, Haggas R, Massey E, Norfolk D, Pinchon D, Sewell C, Wells A (2012).
2604:
2600:
2281:
1649:
72:
1503:
134:
due to the presence of IgA in these blood products. Patients have an increased susceptibility to
2578:
1980:
1855:
1593:
1485:
1434:
488:
329:
200:
143:
127:
792:"Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis"
2619:
2492:
2468:
2448:
2444:
1949:
1940:
1925:
1769:
1711:
1682:
1585:
1550:
1477:
1422:
1412:
1365:
1324:
1257:
1249:
1205:
1156:
1105:
1082:
1033:
974:
888:
870:
831:
813:
772:
754:
700:
641:
592:
553:
480:
433:
380:
61:
2512:
2507:
2472:
2432:
2204:
2047:
2028:
1959:
1773:
1765:
1577:
1540:
1467:
1355:
1314:
1239:
1195:
1187:
1146:
1138:
1072:
1064:
1023:
966:
878:
862:
821:
803:
762:
746:
690:
680:
631:
623:
584:
543:
535:
472:
453:
Yel, L. (2010) 'Selective IgA Deficiency', Journal of
Clinical Immunology, 30(1), pp. 10-16.
423:
415:
98:
90:
982:
2500:
2070:
48:
332:. Of note, selective IgA deficiency can complicate the diagnosis of one such condition,
826:
791:
676:
2826:
2734:
2666:
2488:
2460:
2123:
2023:
1845:
1654:
1244:
1227:
1200:
1175:
1077:
1052:
883:
850:
767:
734:
695:
660:
636:
611:
548:
523:
428:
401:
337:
333:
224:
1151:
1126:
970:
2965:
2834:
2524:
2286:
2159:
1581:
1191:
1068:
539:
419:
192:
188:
1597:
1278:
492:
2843:
2770:
2253:
2099:
1911:
1722:
1489:
196:
1142:
1676:
1545:
1528:
952:"Practice parameter for the diagnosis and management of primary immunodeficiency"
866:
750:
1706:
588:
123:
56:
The dimeric IgA molecule. 1 H-chain, 2 L-chain, 3 J-chain, 4 secretory component
1687:
1319:
1302:
1028:
1011:
808:
2541:
2104:
2065:
1639:
1053:"Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID)"
627:
402:"Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID)"
185:
161:
66:
1426:
1253:
874:
817:
758:
2758:
2746:
2722:
1717:
1012:"Update on the use of immunoglobulin in human disease: A review of evidence"
685:
281:
217:
135:
1589:
1554:
1481:
1369:
1328:
1261:
1209:
1160:
1086:
1037:
978:
892:
835:
776:
704:
645:
572:
557:
437:
1301:
Tacquard C, Boudjedir K, Carlier M, Muller JY, Gomis P, Mertes PM (2017).
596:
484:
2876:
2710:
2582:
2055:
1790:
522:
Litzman J, VlkovĂĄ M, PikulovĂĄ Z, StikarovskĂĄ D, Lokaj J (February 2007).
365:
102:
1279:"IgA Deficiency: Immunodeficiency Disorders: Merck Manual Professional"
476:
347:, selective IgA deficiency is not associated with an increased risk of
1622:
1472:
1455:
1360:
1343:
2922:
2628:
2586:
1930:
1799:
1634:
348:
154:
17:
1104:(3rd ed.). St. Louis, Mo.: Mosby/Elsevier. pp. 1265â1280.
659:
Sekine H, Ferreira RC, Pan-Hammarström Q, et al. (April 2007).
164:
subpopulation abnormalities. IgA-deficient patients may progress to
512:
Harrison's
Principles of Internal Medicine, 17th edition, pag. 2058
29:"SIgAD" redirects here. For the signals intelligence activity, see
2934:
2910:
2536:
2336:
2301:
1411:. Norfolk, Derek (5th ed.). London: Stationery Office. 2013.
661:"Role for Msh5 in the regulation of Ig class switch recombination"
30:
2897:
2821:
2797:
2427:
2382:
1665:
721:
275:
261:
255:
239:
2163:
1738:
1174:
Mellemkjaer L, Hammarstrom L, Andersen V, et al. (2002).
328:
Prognosis is excellent, although there is an association with
110:
106:
1125:
Prince HE, Gary L. Norman, Walter L. Binder (November 2002).
2972:
Noninfectious immunodeficiency-related cutaneous conditions
1342:
Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A (2017).
849:
Swain S, Selmi C, Gershwin ME, Teuber SS (December 2012).
733:
Swain S, Selmi C, Gershwin ME, Teuber SS (December 2019).
449:
447:
851:"The clinical implications of selective IgA deficiency"
735:"The clinical implications of selective IgA deficiency"
2891:
Junctional epidermolysis bullosa with pyloric atresia
921:
Choosing Wisely: An
Initiative of the ABIM Foundation
914:"Five Things Physicians and Patients Should Question"
1612:
400:
Hammarström L, Vorechovsky I, Webster D (May 2000).
2858:
2785:
2700:
2647:
2618:
2555:
2417:
2401:
2374:
2351:
2328:
2213:
2198:
2087:
2064:
2046:
1994:
1924:
1899:
1831:
1808:
1789:
1780:
1697:
1616:
1100:Mark Ballow (2008). "85". In Robert R. Rich (ed.).
925:
American
Academy of Allergy, Asthma, and Immunology
910:
American
Academy of Allergy, Asthma, and Immunology
60:
41:
1102:Clinical immunology : principles and practice
355:severe allergic reaction to a blood transfusion.
2530:Congenital insensitivity to pain with anhidrosis
1051:Hammarström L, Vorechovsky I, Webster D (2000).
2175:
1750:
1527:McGowan KE, Lyon EM, Butzner JD (July 2008).
1407:"5.2 Non-infectious hazards of transfusion".
8:
2275:Gonadotropin-releasing hormone insensitivity
904:
902:
371:It is more common in males than in females.
2691:Congenital amegakaryocytic thrombocytopenia
223:There is an inherited inability to produce
83:Selective immunoglobulin A (IgA) deficiency
2776:Autoimmune lymphoproliferative syndrome 1A
2414:
2259:Follicle-stimulating hormone insensitivity
2210:
2182:
2168:
2160:
1975:Purine nucleoside phosphorylase deficiency
1823:Transient hypogammaglobulinemia of infancy
1805:
1786:
1757:
1743:
1735:
1613:
1384:"SHOT Report, Summary and Supplement 2017"
1307:Journal of Allergy and Clinical Immunology
1016:Journal of Allergy and Clinical Immunology
959:Annals of Allergy, Asthma & Immunology
945:
943:
941:
316:There is a historical popularity in using
160:arrests, but it does not present the same
71:
47:
38:
2940:X-linked severe combined immunodeficiency
2716:TNF receptor associated periodic syndrome
1544:
1471:
1359:
1318:
1243:
1199:
1150:
1076:
1027:
882:
825:
807:
796:Allergy, Asthma & Clinical Immunology
766:
694:
684:
635:
547:
427:
232:
2728:Selective immunoglobulin A deficiency 2
2319:Aspirin-exacerbated respiratory disease
392:
2903:EDAR hypohidrotic ectodermal dysplasia
2831:Familial exudative vitreoretinopathy 4
2388:Familial exudative vitreoretinopathy 1
2139:Terminal complement pathway deficiency
1432:
2595:Hereditary hemorrhagic telangiectasia
2571:Persistent MĂŒllerian duct syndrome II
2342:Jansen's metaphyseal chondrodysplasia
1296:
1294:
1221:
1219:
1005:
1003:
855:Journal of Translational Autoimmunity
739:Journal of Translational Autoimmunity
338:high levels of certain IgA antibodies
42:Selective immunoglobulin A deficiency
7:
2916:Nevoid basal-cell carcinoma syndrome
2365:Familial hypocalciuric hypercalcemia
1057:Clinical and Experimental Immunology
508:
506:
504:
502:
407:Clinical and Experimental Immunology
343:As opposed to the related condition
2977:Predominantly antibody deficiencies
2679:Surfactant metabolism dysfunction 4
2144:Paroxysmal nocturnal hemoglobinuria
718:Online Mendelian Inheritance in Man
573:"Genetic Aspects of IgA Deficiency"
97:. People with this deficiency lack
2928:BMPR1A juvenile polyposis syndrome
2849:LDLR Familial hypercholesterolemia
1348:Scandinavian Journal of Immunology
1245:10.21307/immunohematology-2019-454
201:congenital intrauterine infections
25:
2239:Luteinizing hormone insensitivity
364:more common in those with type 1
2307:Nephrogenic diabetes insipidus 1
1907:Common variable immunodeficiency
1582:10.1111/j.1365-2362.2004.01311.x
1409:Handbook of transfusion medicine
1192:10.1046/j.1365-2249.2002.02004.x
1069:10.1046/j.1365-2249.2000.01131.x
540:10.1111/j.1365-2249.2006.03274.x
420:10.1046/j.1365-2249.2000.01131.x
340:usually seen in celiac disease.
151:common variable immunodeficiency
142:and a higher risk of developing
138:and recurrent episodes of other
2247:Male-limited precocious puberty
2079:Idiopathic CD4+ lymphocytopenia
2634:Leber's congenital amaurosis 1
2546:Gastrointestinal stromal tumor
2149:Complement receptor deficiency
2019:Adenosine deaminase deficiency
1460:British Journal of Haematology
1388:Serious Hazards of Transfusion
571:Cunningham-Rundles C. (1990).
336:, as the deficiency masks the
212:Pathogenesis of IgA Deficiency
176:are found in the same family.
1:
1143:10.1128/CDLI.9.4.824-827.2002
971:10.1016/s1081-1206(10)61142-8
1546:10.1373/clinchem.2008.103606
867:10.1016/j.jtauto.2019.100025
751:10.1016/j.jtauto.2019.100025
665:Proc. Natl. Acad. Sci. U.S.A
2227:Congenital hypothyroidism 1
1818:X-linked agammaglobulinemia
589:10.1007/978-1-4757-9065-8_4
2998:
2886:Glanzmann's thrombasthenia
2866:Immunoglobulin superfamily
2518:RabsonâMendenhall syndrome
2200:G protein-coupled receptor
1504:"IgA deficient components"
1320:10.1016/j.jaci.2017.03.029
1029:10.1016/j.jaci.2016.09.023
809:10.1186/s13223-023-00826-y
612:"Selective IgA Deficiency"
318:intravenous immunoglobulin
132:intravenous immunoglobulin
28:
2740:Hyper-IgM syndrome type 3
628:10.1007/s10875-009-9357-x
55:
46:
2815:CenaniâLenz syndactylism
2657:Type I cytokine receptor
2295:Hirschsprung's disease 2
2034:Bare lymphocyte syndrome
1886:WiskottâAldrich syndrome
312:Use of IVIG as treatment
166:panhypogammaglobulinemia
2497:Thanatophoric dysplasia
2291:Waardenburg syndrome 4a
2134:Complement 3 deficiency
2119:Complement 4 deficiency
2115:Complement 2 deficiency
686:10.1073/pnas.0700815104
153:(CVID) feature similar
2951:cell surface receptors
2803:DonnaiâBarrow syndrome
2477:JacksonâWeiss syndrome
2465:AntleyâBixler syndrome
2449:KAL2 Kallmann syndrome
2403:Enzyme-linked receptor
2243:Leydig cell hypoplasia
1439:: CS1 maint: others (
225:immunoglobulin A (IgA)
140:respiratory infections
2263:XX gonadal dysgenesis
2191:Cell surface receptor
2109:Hereditary angioedema
1965:Ataxiaâtelangiectasia
1810:Hypogammaglobulinemia
95:hypogammaglobulinemia
2982:Transfusion medicine
2609:LoeysâDietz syndrome
2129:Properdin deficiency
1950:Di George's syndrome
1833:Dysgammaglobulinemia
1570:Eur. J. Clin. Invest
1226:Vassallo RR (2020).
1131:Clin Vaccine Immunol
172:. Selective IgA and
988:on 11 November 2011
677:2007PNAS..104.7193S
149:IgA deficiency and
144:autoimmune diseases
2579:TGF beta receptors
1981:Hyper IgM syndrome
1891:Hyper-IgE syndrome
1856:Hyper IgM syndrome
1772:disorders causing
1698:External resources
1533:Clinical Chemistry
1508:transfusion.com.au
1180:Clin. Exp. Immunol
528:Clin. Exp. Immunol
477:10.1007/BF01540915
330:autoimmune disease
168:characteristic of
128:blood transfusions
117:Signs and symptoms
2959:
2958:
2643:
2642:
2493:Hypochondroplasia
2469:Pfeiffer syndrome
2445:Pfeiffer syndrome
2397:
2396:
2157:
2156:
2042:
2041:
1941:thymic hypoplasia
1926:T cell deficiency
1920:
1919:
1732:
1731:
1473:10.1111/bjh.12017
1361:10.1111/sji.12499
381:B cell deficiency
291:
290:
101:(IgA), a type of
80:
79:
36:Medical condition
16:(Redirected from
2989:
2513:Donohue syndrome
2473:Crouzon syndrome
2433:Robinow syndrome
2415:
2211:
2184:
2177:
2170:
2161:
2029:ZAP70 deficiency
1960:Nezelof syndrome
1806:
1787:
1774:immunodeficiency
1759:
1752:
1745:
1736:
1614:
1602:
1601:
1565:
1559:
1558:
1548:
1539:(7): 1203â1209.
1524:
1518:
1517:
1515:
1514:
1500:
1494:
1493:
1475:
1451:
1445:
1444:
1438:
1430:
1404:
1398:
1397:
1395:
1394:
1380:
1374:
1373:
1363:
1339:
1333:
1332:
1322:
1298:
1289:
1288:
1286:
1285:
1275:
1266:
1265:
1247:
1232:Immunohematology
1223:
1214:
1213:
1203:
1171:
1165:
1164:
1154:
1137:(6): 1295â1300.
1122:
1116:
1115:
1097:
1091:
1090:
1080:
1048:
1042:
1041:
1031:
1007:
998:
997:
995:
993:
987:
981:. Archived from
956:
947:
936:
935:
933:
931:
918:
906:
897:
896:
886:
846:
840:
839:
829:
811:
787:
781:
780:
770:
730:
724:
715:
709:
708:
698:
688:
656:
650:
649:
639:
610:Yel, L. (2010).
607:
601:
600:
568:
562:
561:
551:
519:
513:
510:
497:
496:
460:
454:
451:
442:
441:
431:
397:
233:
99:immunoglobulin A
91:immunodeficiency
76:
75:
51:
39:
21:
2997:
2996:
2992:
2991:
2990:
2988:
2987:
2986:
2962:
2961:
2960:
2955:
2859:Other/ungrouped
2854:
2835:Osteopetrosis 1
2781:
2696:
2639:
2614:
2551:
2501:Muenke syndrome
2407:
2405:
2393:
2370:
2347:
2324:
2202:
2194:
2188:
2158:
2153:
2090:
2083:
2071:Lymphocytopenia
2069:
2060:
2038:
2014:
2001:
1990:
1946:hypoparathyroid
1928:
1916:
1895:
1827:
1797:
1776:
1763:
1733:
1728:
1727:
1693:
1692:
1625:
1611:
1606:
1605:
1567:
1566:
1562:
1526:
1525:
1521:
1512:
1510:
1502:
1501:
1497:
1453:
1452:
1448:
1431:
1419:
1406:
1405:
1401:
1392:
1390:
1382:
1381:
1377:
1341:
1340:
1336:
1300:
1299:
1292:
1283:
1281:
1277:
1276:
1269:
1225:
1224:
1217:
1173:
1172:
1168:
1124:
1123:
1119:
1112:
1099:
1098:
1094:
1050:
1049:
1045:
1009:
1008:
1001:
991:
989:
985:
954:
949:
948:
939:
929:
927:
916:
908:
907:
900:
848:
847:
843:
789:
788:
784:
732:
731:
727:
716:
712:
658:
657:
653:
609:
608:
604:
570:
569:
565:
521:
520:
516:
511:
500:
462:
461:
457:
452:
445:
399:
398:
394:
389:
377:
361:
326:
314:
305:
296:
230:Types include:
209:
207:Pathophysiology
182:
158:differentiation
146:in middle age.
119:
89:) is a kind of
70:
37:
34:
23:
22:
15:
12:
11:
5:
2995:
2993:
2985:
2984:
2979:
2974:
2964:
2963:
2957:
2956:
2954:
2953:
2948:
2944:
2943:
2931:
2919:
2894:
2893:
2888:
2883:
2873:
2872:
2862:
2860:
2856:
2855:
2853:
2852:
2839:
2838:
2827:Worth syndrome
2818:
2806:
2789:
2787:
2786:Lipid receptor
2783:
2782:
2780:
2779:
2767:
2755:
2743:
2731:
2719:
2706:
2704:
2698:
2697:
2695:
2694:
2683:
2682:
2670:
2667:Laron syndrome
2653:
2651:
2645:
2644:
2641:
2640:
2638:
2637:
2624:
2622:
2616:
2615:
2613:
2612:
2598:
2575:
2574:
2561:
2559:
2553:
2552:
2550:
2549:
2542:KIT Piebaldism
2533:
2521:
2515:
2504:
2489:Achondroplasia
2480:
2461:Apert syndrome
2452:
2436:
2423:
2421:
2412:
2399:
2398:
2395:
2394:
2392:
2391:
2378:
2376:
2372:
2371:
2369:
2368:
2355:
2353:
2349:
2348:
2346:
2345:
2332:
2330:
2326:
2325:
2323:
2322:
2310:
2298:
2278:
2266:
2250:
2230:
2217:
2215:
2208:
2196:
2195:
2189:
2187:
2186:
2179:
2172:
2164:
2155:
2154:
2152:
2151:
2146:
2141:
2136:
2131:
2126:
2124:MBL deficiency
2121:
2112:
2095:
2093:
2085:
2084:
2082:
2081:
2075:
2073:
2062:
2061:
2059:
2058:
2052:
2050:
2044:
2043:
2040:
2039:
2037:
2036:
2031:
2026:
2024:Omenn syndrome
2021:
2005:
2003:
1992:
1991:
1989:
1988:
1969:
1968:
1962:
1953:
1936:
1934:
1922:
1921:
1918:
1917:
1915:
1914:
1909:
1903:
1901:
1897:
1896:
1894:
1893:
1888:
1883:
1877:
1872:
1867:
1862:
1853:
1851:IgM deficiency
1848:
1846:IgG deficiency
1843:
1841:IgA deficiency
1837:
1835:
1829:
1828:
1826:
1825:
1820:
1814:
1812:
1803:
1784:
1778:
1777:
1764:
1762:
1761:
1754:
1747:
1739:
1730:
1729:
1726:
1725:
1714:
1702:
1701:
1699:
1695:
1694:
1691:
1690:
1679:
1668:
1657:
1642:
1626:
1621:
1620:
1618:
1617:Classification
1610:
1609:External links
1607:
1604:
1603:
1560:
1519:
1495:
1466:(2): 143â153.
1446:
1417:
1399:
1375:
1334:
1313:(3): 884â885.
1290:
1267:
1238:(4): 226â233.
1215:
1186:(3): 495â500.
1166:
1117:
1111:978-0323044042
1110:
1092:
1063:(2): 225â231.
1043:
999:
937:
898:
841:
782:
725:
710:
671:(17): 7193â8.
651:
616:J Clin Immunol
602:
577:Adv Hum. Genet
563:
514:
498:
465:J Clin Immunol
455:
443:
414:(2): 225â231.
391:
390:
388:
385:
384:
383:
376:
373:
360:
357:
334:celiac disease
325:
322:
313:
310:
304:
301:
295:
292:
289:
288:
285:
278:
273:
269:
268:
265:
258:
253:
249:
248:
245:
242:
237:
208:
205:
181:
178:
118:
115:
78:
77:
64:
58:
57:
53:
52:
44:
43:
35:
24:
14:
13:
10:
9:
6:
4:
3:
2:
2994:
2983:
2980:
2978:
2975:
2973:
2970:
2969:
2967:
2952:
2949:
2946:
2945:
2941:
2937:
2936:
2932:
2929:
2925:
2924:
2920:
2917:
2913:
2912:
2908:
2907:
2906:
2904:
2900:
2899:
2892:
2889:
2887:
2884:
2882:
2878:
2875:
2874:
2871:
2867:
2864:
2863:
2861:
2857:
2850:
2846:
2845:
2841:
2840:
2836:
2832:
2828:
2824:
2823:
2819:
2816:
2812:
2811:
2807:
2804:
2800:
2799:
2794:
2791:
2790:
2788:
2784:
2777:
2773:
2772:
2768:
2765:
2761:
2760:
2756:
2753:
2749:
2748:
2744:
2741:
2737:
2736:
2732:
2729:
2725:
2724:
2720:
2717:
2713:
2712:
2708:
2707:
2705:
2703:
2699:
2692:
2688:
2685:
2684:
2680:
2676:
2675:
2671:
2668:
2664:
2663:
2658:
2655:
2654:
2652:
2650:
2646:
2635:
2631:
2630:
2626:
2625:
2623:
2621:
2617:
2610:
2606:
2602:
2599:
2596:
2592:
2588:
2584:
2580:
2577:
2576:
2572:
2568:
2567:
2563:
2562:
2560:
2558:
2554:
2547:
2543:
2539:
2538:
2534:
2531:
2527:
2526:
2522:
2519:
2516:
2514:
2510:
2509:
2505:
2502:
2498:
2494:
2490:
2486:
2485:
2481:
2478:
2474:
2470:
2466:
2462:
2458:
2457:
2453:
2450:
2446:
2442:
2441:
2437:
2434:
2430:
2429:
2425:
2424:
2422:
2420:
2416:
2413:
2410:
2409:growth factor
2404:
2400:
2389:
2385:
2384:
2380:
2379:
2377:
2373:
2366:
2362:
2361:
2357:
2356:
2354:
2350:
2343:
2339:
2338:
2334:
2333:
2331:
2327:
2320:
2316:
2315:
2311:
2308:
2304:
2303:
2299:
2296:
2292:
2288:
2287:ABCD syndrome
2284:
2283:
2279:
2276:
2272:
2271:
2267:
2264:
2260:
2256:
2255:
2251:
2248:
2244:
2240:
2236:
2235:
2231:
2228:
2224:
2223:
2219:
2218:
2216:
2212:
2209:
2206:
2201:
2197:
2192:
2185:
2180:
2178:
2173:
2171:
2166:
2165:
2162:
2150:
2147:
2145:
2142:
2140:
2137:
2135:
2132:
2130:
2127:
2125:
2122:
2120:
2116:
2113:
2110:
2106:
2102:
2101:
2097:
2096:
2094:
2092:
2086:
2080:
2077:
2076:
2074:
2072:
2067:
2063:
2057:
2054:
2053:
2051:
2049:
2045:
2035:
2032:
2030:
2027:
2025:
2022:
2020:
2017:
2013:
2010:
2007:
2006:
2004:
2000:
1997:
1993:
1986:
1982:
1979:
1978:
1977:
1976:
1973:
1966:
1963:
1961:
1957:
1956:euparathyroid
1954:
1951:
1947:
1944:
1942:
1938:
1937:
1935:
1932:
1927:
1923:
1913:
1910:
1908:
1905:
1904:
1902:
1898:
1892:
1889:
1887:
1884:
1881:
1878:
1876:
1873:
1871:
1868:
1866:
1863:
1861:
1857:
1854:
1852:
1849:
1847:
1844:
1842:
1839:
1838:
1836:
1834:
1830:
1824:
1821:
1819:
1816:
1815:
1813:
1811:
1807:
1804:
1801:
1796:
1792:
1788:
1785:
1783:
1779:
1775:
1771:
1767:
1760:
1755:
1753:
1748:
1746:
1741:
1740:
1737:
1724:
1720:
1719:
1715:
1713:
1709:
1708:
1704:
1703:
1700:
1696:
1689:
1685:
1684:
1680:
1678:
1674:
1673:
1669:
1667:
1663:
1662:
1658:
1656:
1652:
1651:
1647:
1643:
1641:
1637:
1636:
1632:
1628:
1627:
1624:
1619:
1615:
1608:
1599:
1595:
1591:
1587:
1583:
1579:
1575:
1571:
1564:
1561:
1556:
1552:
1547:
1542:
1538:
1534:
1530:
1523:
1520:
1509:
1505:
1499:
1496:
1491:
1487:
1483:
1479:
1474:
1469:
1465:
1461:
1457:
1450:
1447:
1442:
1436:
1428:
1424:
1420:
1418:9780117068469
1414:
1410:
1403:
1400:
1389:
1385:
1379:
1376:
1371:
1367:
1362:
1357:
1353:
1349:
1345:
1338:
1335:
1330:
1326:
1321:
1316:
1312:
1308:
1304:
1297:
1295:
1291:
1280:
1274:
1272:
1268:
1263:
1259:
1255:
1251:
1246:
1241:
1237:
1233:
1229:
1222:
1220:
1216:
1211:
1207:
1202:
1197:
1193:
1189:
1185:
1181:
1177:
1170:
1167:
1162:
1158:
1153:
1148:
1144:
1140:
1136:
1132:
1128:
1121:
1118:
1113:
1107:
1103:
1096:
1093:
1088:
1084:
1079:
1074:
1070:
1066:
1062:
1058:
1054:
1047:
1044:
1039:
1035:
1030:
1025:
1022:(3): S1âS46.
1021:
1017:
1013:
1006:
1004:
1000:
984:
980:
976:
972:
968:
965:(5): S1âS63.
964:
960:
953:
946:
944:
942:
938:
926:
922:
915:
911:
905:
903:
899:
894:
890:
885:
880:
876:
872:
868:
864:
860:
856:
852:
845:
842:
837:
833:
828:
823:
819:
815:
810:
805:
801:
797:
793:
786:
783:
778:
774:
769:
764:
760:
756:
752:
748:
744:
740:
736:
729:
726:
723:
719:
714:
711:
706:
702:
697:
692:
687:
682:
678:
674:
670:
666:
662:
655:
652:
647:
643:
638:
633:
629:
625:
621:
617:
613:
606:
603:
598:
594:
590:
586:
582:
578:
574:
567:
564:
559:
555:
550:
545:
541:
537:
534:(2): 249â54.
533:
529:
525:
518:
515:
509:
507:
505:
503:
499:
494:
490:
486:
482:
478:
474:
471:(3): 165â70.
470:
466:
459:
456:
450:
448:
444:
439:
435:
430:
425:
421:
417:
413:
409:
408:
403:
396:
393:
386:
382:
379:
378:
374:
372:
369:
367:
358:
356:
352:
350:
346:
341:
339:
335:
331:
323:
321:
319:
311:
309:
302:
300:
293:
286:
284:
283:
279:
277:
274:
271:
270:
266:
263:
259:
257:
254:
251:
250:
246:
243:
241:
238:
235:
234:
231:
228:
226:
221:
219:
214:
213:
206:
204:
202:
198:
194:
190:
187:
179:
177:
175:
171:
167:
163:
159:
156:
152:
147:
145:
141:
137:
133:
129:
125:
116:
114:
112:
108:
104:
100:
96:
92:
88:
84:
74:
68:
65:
63:
59:
54:
50:
45:
40:
32:
27:
19:
2933:
2921:
2909:
2896:
2895:
2842:
2820:
2808:
2796:
2769:
2757:
2745:
2733:
2727:
2721:
2709:
2702:TNF receptor
2672:
2660:
2627:
2564:
2535:
2523:
2506:
2482:
2454:
2438:
2426:
2381:
2358:
2335:
2312:
2300:
2280:
2268:
2252:
2232:
2220:
2193:deficiencies
2100:C1-inhibitor
2098:
2015:
2008:
1971:
1970:
1955:
1945:
1939:
1912:ICF syndrome
1840:
1716:
1705:
1681:
1670:
1659:
1644:
1629:
1576:(3): 224â8.
1573:
1569:
1563:
1536:
1532:
1522:
1511:. Retrieved
1507:
1498:
1463:
1459:
1449:
1408:
1402:
1391:. Retrieved
1387:
1378:
1351:
1347:
1337:
1310:
1306:
1282:. Retrieved
1235:
1231:
1183:
1179:
1169:
1134:
1130:
1120:
1101:
1095:
1060:
1056:
1046:
1019:
1015:
990:. Retrieved
983:the original
962:
958:
928:. Retrieved
920:
858:
854:
844:
799:
795:
785:
742:
738:
728:
713:
668:
664:
654:
622:(1): 10â16.
619:
615:
605:
580:
576:
566:
531:
527:
517:
468:
464:
458:
411:
405:
395:
370:
362:
359:Epidemiology
353:
342:
327:
315:
306:
297:
280:
229:
222:
215:
211:
210:
183:
148:
120:
93:, a type of
86:
82:
81:
26:
2203:(including
1972:peripheral:
1707:MedlinePlus
1354:(1): 3â12.
583:: 235â266.
186:chromosomes
124:anaphylaxis
2966:Categories
2406:(including
2105:Angioedema
2091:deficiency
2089:Complement
2066:Leukopenia
2016:autosomal:
1770:complement
1683:DiseasesDB
1513:2019-04-26
1393:2019-04-26
1284:2008-03-01
930:August 14,
861:: 100025.
745:: 100025.
387:References
264:suggested
162:lymphocyte
67:Immunology
2759:TNFRSF13B
2747:TNFRSF13C
2723:TNFRSF13B
2009:x-linked:
1718:eMedicine
1435:cite book
1427:869523772
1254:0894-203X
992:27 August
875:2589-9090
818:1710-1492
802:(1): 75.
759:2589-9090
324:Prognosis
303:Treatment
294:Diagnosis
282:TNFRSF13B
260:Unknown;
218:phenotype
136:pneumonia
62:Specialty
2947:See also
2877:Integrin
2711:TNFRSF1A
2649:JAK-STAT
2583:Endoglin
2056:HIV/AIDS
2048:Acquired
1999:combined
1791:Antibody
1766:Lymphoid
1723:med/1159
1598:25545688
1590:15025682
1555:18487281
1482:22928769
1370:27763681
1329:28414063
1262:15679454
1210:12452841
1161:12093680
1087:10792368
1038:28041678
979:15945566
893:32743511
836:37641141
827:10463351
777:32743511
720:(OMIM):
705:17409188
646:20101521
558:17223965
493:28529140
438:10792368
375:See also
366:diabetes
103:antibody
2870:AGM3, 6
2771:TNFRSF6
2735:TNFRSF5
2375:Class F
2352:Class C
2329:Class B
2214:Class A
2205:hormone
1795:humoral
1782:Primary
1677:D017098
1490:9150295
1201:1906562
1078:1905641
884:7388344
768:7388344
696:1855370
673:Bibcode
637:2821513
597:2193490
549:1810464
485:8734360
429:1905641
2923:BMPR1A
2674:CSF2RA
2629:GUCY2D
2605:TGFBR2
2601:TGFBR1
2314:PTGER2
2012:X-SCID
1996:Severe
1712:001476
1666:137100
1655:279.01
1596:
1588:
1553:
1488:
1480:
1425:
1415:
1368:
1327:
1260:
1252:
1208:
1198:
1159:
1152:120015
1149:
1108:
1085:
1075:
1036:
977:
891:
881:
873:
834:
824:
816:
775:
765:
757:
722:137100
703:
693:
644:
634:
595:
556:
546:
491:
483:
436:
426:
349:cancer
287:17p11
276:609529
272:IGAD2
256:137100
252:IGAD1
247:Locus
155:B cell
69:
2935:IL2RG
2911:PTCH1
2764:CVID2
2752:CVID4
2591:SMAD4
2587:Alk-1
2566:AMHR2
2525:NTRK1
2484:FGFR3
2456:FGFR2
2440:FGFR1
2337:PTH1R
2302:AVPR2
2282:EDNRB
2270:GnRHR
2234:LHCGR
2002:(B+T)
1900:Other
1688:29569
1640:D80.2
1594:S2CID
1486:S2CID
986:(PDF)
955:(PDF)
917:(PDF)
489:S2CID
267:6p21
244:Gene
236:Type
180:Cause
126:, to
87:SIgAD
31:SIGAD
18:SIgAD
2898:EDAR
2881:LAD1
2844:LDLR
2822:LRP5
2810:LRP4
2798:LRP2
2557:STPK
2508:INSR
2428:ROR2
2383:FZD4
2360:CASR
2254:FSHR
2222:TSHR
1768:and
1672:MeSH
1661:OMIM
1650:9-CM
1586:PMID
1551:PMID
1478:PMID
1441:link
1423:OCLC
1413:ISBN
1366:PMID
1325:PMID
1258:PMID
1250:ISSN
1206:PMID
1157:PMID
1106:ISBN
1083:PMID
1034:PMID
994:2012
975:PMID
932:2012
889:PMID
871:ISSN
832:PMID
814:ISSN
773:PMID
755:ISSN
701:PMID
642:PMID
593:PMID
554:PMID
481:PMID
434:PMID
345:CVID
262:MSH5
240:OMIM
195:and
174:CVID
170:CVID
109:and
2793:LRP
2687:MPL
2537:KIT
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1646:ICD
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