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Complement 2 deficiency

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Complement deficiency has historically been associated with early, severe bacterial infections among children. Infection susceptibility is frequently observed.
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Pickering, M.C.; Botto, M.; Taylor, P.R.; Lachmann, P.J.; Walport, M.J. (2001). "Systemic Lupus Erythematosus, Complement Deficiency, and Apoptosis".
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Cole, F. Sessions; Whitehead, Alexander S.; Auerbach, Harvey S.; Lint, Thomas; Zeitz, Howard J.; Kilbridge, Peter; Colten, Harvey R. (July 4, 1985).
1572: 573: 1274: 765: 422: 377: 343: 1577: 1129: 1512: 1429: 1340: 831:"Deficiency of the second component of complement associated with anaphylactoid purpura and presence of mycoplasma in the serum" 223: 1603: 1582: 1452: 1324: 572:
Jönsson, Göran; Truedsson, Lennart; Sturfelt, Gunnar; Oxelius, Vivi-Anne; Braconier, Jean Henrik; Sjöholm, Anders G. (2005).
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can occur. Renal and neuropsychiatric disease, on the other hand, is thought to be uncommon in these SLE patients.
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levels, which could contribute to raised infection susceptibility. More than half of 40 C2D patients in a study had
278:"Immunoglobulin deficiencies and susceptibility to infection among homozygotes and heterozygotes for C2 deficiency" 1398: 1418: 1313: 1308: 1303: 1298: 1293: 1228: 804: 277: 1467: 1215: 134: 1168: 1567: 1552: 404: 1037: 207:
patients with C2D, skin and joint involvement are common, and severe persistent cutaneous and/or subacute
150: 78: 1542: 1522: 1243: 256: 200: 186: 108: 74: 1562: 1266: 466: 182: 104: 60: 1118: 1414: 1289: 428: 308: 215: 190: 178: 630:"Infections of People with Complement Deficiencies and Patients Who Have Undergone Splenectomy" 1383: 1374: 1359: 1203: 1140: 1065: 1057: 1007: 999: 953: 945: 899: 891: 852: 779: 771: 761: 728: 710: 669: 651: 603: 595: 554: 536: 492: 484: 418: 373: 367: 339: 300: 49: 335: 1481: 1462: 1393: 1207: 1199: 1049: 991: 979: 937: 883: 842: 753: 718: 700: 659: 641: 585: 544: 526: 474: 410: 292: 239: 926:"Subacute cutaneous lupus erythematosus in multiple members of a family with C2 deficiency" 1504: 227: 941: 980:"The Molecular Basis for Genetic Deficiency of the Second Component of Human Complement" 470: 1557: 1457: 1279: 847: 830: 723: 688: 664: 629: 122: 1123: 757: 590: 549: 514: 1597: 887: 479: 454: 432: 328: 1533: 1345: 870:
Leddy, John P.; Griggs, Robert C.; Klemperer, Martin R.; Frank, Michael M. (1975).
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Sturfelt, Gunnar; Truedsson, Lennart (2020). "Complement Component C2 Deficiency".
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In roughly 10% of patients, a C2 deficiency is linked to an illness that resembles
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Sjöholm, A.G.; Jönsson, G.; Braconier, J.H.; Sturfelt, G.; Truedsson, L. (2006).
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caused by any one of several different alterations in the structure of
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associated with C2D is regarded as less severe when compared to other
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Sussman, M.; Jones, J. H.; Almeida, June D.; Lachmann, P. J. (1973).
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JP, Callen; SJ, Hodge; KB, Kulick; G, Stelzer; JJ, Buchino (1987).
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Pettigrew, H. David; Teuber, Suzanne S.; Gershwin, M. Eric (2009).
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Sherwood L. Gorbach; John G. Bartlett; Neil R. Blacklow (2004).
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are two other infections commonly seen in C2D patients.
872:"Hereditary complement (C2) deficiency with dermatomyositis" 165:. Clinical manifestations of this SLE-like illness include 276:
Alper CA, Xu J, Cosmopoulos K, et al. (July 2003).
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will be present in approximately 50% of the population.
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C2 deficiency, Complement component 2 deficiency, C2D.
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Ram, Sanjay; Lewis, Lisa A.; Rice, Peter A. (2010).
1521: 1498: 1480: 1428: 1358: 1333: 1265: 1242: 1223: 1214: 1085: 48: 26: 21: 455:"Clinical Significance of Complement Deficiencies" 327: 640:(4). American Society for Microbiology: 740–780. 525:(3). American Society for Microbiology: 359–395. 1038:"Complement deficiency and disease: An update" 334:. Lippincott Williams & Wilkins. pp.  1184: 689:"Complement and systemic lupus erythematosus" 8: 752:. Vol. 76. Elsevier. pp. 227–324. 990:(1). Massachusetts Medical Society: 11–16. 111:bacterial infections, as well as a risk of 84:It has been associated with an increase in 1409:Purine nucleoside phosphorylase deficiency 1257:Transient hypogammaglobulinemia of infancy 1239: 1220: 1191: 1177: 1169: 1082: 459:Annals of the New York Academy of Sciences 59: 35: 18: 846: 722: 704: 663: 645: 589: 548: 530: 478: 369:Textbook of Microbiology & Immunology 121:C2D is linked to abnormalities in serum 841:(4). Oxford University Press: 531–539. 268: 137:-related invasive infection, primarily 1573:Terminal complement pathway deficiency 214:C2 deficiency has also been linked to 7: 942:10.1001/archderm.1987.01660250072021 835:Clinical and Experimental Immunology 574:"Hereditary C2 Deficiency in Sweden" 1578:Paroxysmal nocturnal hemoglobinuria 187:Anti-double-stranded DNA antibodies 406:Encyclopedia of Medical Immunology 14: 591:10.1097/01.md.0000152371.22747.1e 513:Figueroa, J E; Densen, P (1991). 372:. Elsevier India. pp. 125–. 1341:Common variable immunodeficiency 876:The American Journal of Medicine 480:10.1111/j.1749-6632.2009.04633.x 1513:Idiopathic CD4+ lymphocytopenia 984:New England Journal of Medicine 1583:Complement receptor deficiency 1453:Adenosine deaminase deficiency 1: 758:10.1016/s0065-2776(01)76021-x 634:Clinical Microbiology Reviews 519:Clinical Microbiology Reviews 209:cutaneous lupus erythematosus 1054:10.1016/j.molimm.2005.06.025 888:10.1016/0002-9343(75)90537-9 415:10.1007/978-1-4614-8678-7_10 252:Systemic Lupus Erythematosus 147:Haemophilus influenza type b 113:Systemic Lupus Erythematosus 93:systemic lupus erythematosus 91:It can present similarly to 1252:X-linked agammaglobulinemia 1048:(1–2). Elsevier BV: 78–85. 996:10.1056/nejm198507043130103 936:(1). Arch Dermatol: 66–70. 220:membrane glomerulonephritis 115:(SLE) or SLE-like disease. 44:Structure of the C2 protein 1620: 882:(1). Elsevier BV: 83–91. 43: 34: 1468:Bare lymphocyte syndrome 1320:Wiskott–Aldrich syndrome 687:Walport, Mark J (2002). 224:Henoch-Schonlein purpura 135:Streptococcus pneumoniae 1568:Complement 3 deficiency 1553:Complement 4 deficiency 1549:Complement 2 deficiency 930:Archives of Dermatology 297:10.1023/A:1024540917593 242:have also been linked. 201:complement deficiencies 71:Complement 2 deficiency 22:Complement 2 deficiency 749:Advances in Immunology 125:levels, such as lower 79:complement component 2 1604:Complement deficiency 1543:Hereditary angioedema 1399:Ataxia–telangiectasia 1244:Hypogammaglobulinemia 465:(1). Wiley: 108–123. 366:Parija (2009-01-01). 257:Complement deficiency 75:complement deficiency 1563:Properdin deficiency 1384:Di George's syndrome 1267:Dysgammaglobulinemia 1042:Molecular Immunology 647:10.1128/cmr.00048-09 181:. With a low titer, 151:Neisseria meningitis 105:bacterial infections 532:10.1128/cmr.4.3.359 471:2009NYASA1173..108P 330:Infectious diseases 1415:Hyper IgM syndrome 1325:Hyper-IgE syndrome 1290:Hyper IgM syndrome 1206:disorders causing 693:Arthritis Research 240:Hodgkin's lymphoma 216:rheumatic diseases 191:Anti-Ro antibodies 179:glomerulonephritis 99:Signs and symptoms 1591: 1590: 1476: 1475: 1375:thymic hypoplasia 1360:T cell deficiency 1354: 1353: 1166: 1165: 767:978-0-12-022476-0 424:978-1-4614-8677-0 379:978-81-312-2163-1 345:978-0-7817-3371-7 185:may be positive. 103:C2D is linked to 68: 67: 16:Medical condition 1611: 1463:ZAP70 deficiency 1394:Nezelof syndrome 1240: 1221: 1208:immunodeficiency 1193: 1186: 1179: 1170: 1083: 1073: 1023: 1022: 1020: 1018: 975: 969: 968: 966: 964: 921: 915: 914: 912: 910: 867: 861: 860: 850: 826: 820: 819: 817: 815: 801: 795: 794: 792: 790: 743: 737: 736: 726: 708: 684: 678: 677: 667: 649: 625: 619: 618: 616: 614: 593: 569: 563: 562: 552: 534: 510: 501: 500: 482: 450: 444: 443: 441: 439: 400: 391: 390: 388: 386: 363: 357: 356: 354: 352: 333: 323: 317: 316: 285:J. Clin. Immunol 282: 273: 64: 63: 39: 19: 1619: 1618: 1614: 1613: 1612: 1610: 1609: 1608: 1594: 1593: 1592: 1587: 1524: 1517: 1505:Lymphocytopenia 1503: 1494: 1472: 1448: 1435: 1424: 1380:hypoparathyroid 1362: 1350: 1329: 1261: 1231: 1210: 1197: 1167: 1162: 1161: 1094: 1080: 1035: 1032: 1030:Further reading 1027: 1026: 1016: 1014: 977: 976: 972: 962: 960: 923: 922: 918: 908: 906: 869: 868: 864: 828: 827: 823: 813: 811: 803: 802: 798: 788: 786: 768: 745: 744: 740: 686: 685: 681: 627: 626: 622: 612: 610: 571: 570: 566: 512: 511: 504: 452: 451: 447: 437: 435: 425: 402: 401: 394: 384: 382: 380: 365: 364: 360: 350: 348: 346: 325: 324: 320: 280: 275: 274: 270: 265: 248: 232:cutaneous lupus 228:dermatomyositis 159: 101: 58: 17: 12: 11: 5: 1617: 1615: 1607: 1606: 1596: 1595: 1589: 1588: 1586: 1585: 1580: 1575: 1570: 1565: 1560: 1558:MBL deficiency 1555: 1546: 1529: 1527: 1519: 1518: 1516: 1515: 1509: 1507: 1496: 1495: 1493: 1492: 1486: 1484: 1478: 1477: 1474: 1473: 1471: 1470: 1465: 1460: 1458:Omenn syndrome 1455: 1439: 1437: 1426: 1425: 1423: 1422: 1403: 1402: 1396: 1387: 1370: 1368: 1356: 1355: 1352: 1351: 1349: 1348: 1343: 1337: 1335: 1331: 1330: 1328: 1327: 1322: 1317: 1311: 1306: 1301: 1296: 1287: 1285:IgM deficiency 1282: 1280:IgG deficiency 1277: 1275:IgA deficiency 1271: 1269: 1263: 1262: 1260: 1259: 1254: 1248: 1246: 1237: 1218: 1212: 1211: 1198: 1196: 1195: 1188: 1181: 1173: 1164: 1163: 1160: 1159: 1148: 1137: 1126: 1111: 1095: 1090: 1089: 1087: 1086:Classification 1079: 1078:External links 1076: 1075: 1074: 1031: 1028: 1025: 1024: 970: 916: 862: 821: 796: 766: 738: 679: 620: 564: 502: 445: 423: 392: 378: 358: 344: 318: 291:(4): 297–305. 267: 266: 264: 261: 260: 259: 254: 247: 244: 189:are uncommon. 158: 155: 123:immunoglobulin 100: 97: 66: 65: 52: 46: 45: 41: 40: 32: 31: 28: 24: 23: 15: 13: 10: 9: 6: 4: 3: 2: 1616: 1605: 1602: 1601: 1599: 1584: 1581: 1579: 1576: 1574: 1571: 1569: 1566: 1564: 1561: 1559: 1556: 1554: 1550: 1547: 1544: 1540: 1536: 1535: 1531: 1530: 1528: 1526: 1520: 1514: 1511: 1510: 1508: 1506: 1501: 1497: 1491: 1488: 1487: 1485: 1483: 1479: 1469: 1466: 1464: 1461: 1459: 1456: 1454: 1451: 1447: 1444: 1441: 1440: 1438: 1434: 1431: 1427: 1420: 1416: 1413: 1412: 1411: 1410: 1407: 1400: 1397: 1395: 1391: 1390:euparathyroid 1388: 1385: 1381: 1378: 1376: 1372: 1371: 1369: 1366: 1361: 1357: 1347: 1344: 1342: 1339: 1338: 1336: 1332: 1326: 1323: 1321: 1318: 1315: 1312: 1310: 1307: 1305: 1302: 1300: 1297: 1295: 1291: 1288: 1286: 1283: 1281: 1278: 1276: 1273: 1272: 1270: 1268: 1264: 1258: 1255: 1253: 1250: 1249: 1247: 1245: 1241: 1238: 1235: 1230: 1226: 1222: 1219: 1217: 1213: 1209: 1205: 1201: 1194: 1189: 1187: 1182: 1180: 1175: 1174: 1171: 1158: 1154: 1153: 1149: 1147: 1143: 1142: 1138: 1136: 1132: 1131: 1127: 1125: 1121: 1120: 1116: 1112: 1110: 1106: 1105: 1101: 1097: 1096: 1093: 1088: 1084: 1077: 1071: 1067: 1063: 1059: 1055: 1051: 1047: 1043: 1039: 1034: 1033: 1029: 1013: 1009: 1005: 1001: 997: 993: 989: 985: 981: 974: 971: 959: 955: 951: 947: 943: 939: 935: 931: 927: 920: 917: 905: 901: 897: 893: 889: 885: 881: 877: 873: 866: 863: 858: 854: 849: 844: 840: 836: 832: 825: 822: 810: 806: 800: 797: 785: 781: 777: 773: 769: 763: 759: 755: 751: 750: 742: 739: 734: 730: 725: 720: 716: 712: 707: 706:10.1186/ar586 702: 698: 694: 690: 683: 680: 675: 671: 666: 661: 657: 653: 648: 643: 639: 635: 631: 624: 621: 609: 605: 601: 597: 592: 587: 583: 579: 575: 568: 565: 560: 556: 551: 546: 542: 538: 533: 528: 524: 520: 516: 509: 507: 503: 498: 494: 490: 486: 481: 476: 472: 468: 464: 460: 456: 449: 446: 434: 430: 426: 420: 416: 412: 408: 407: 399: 397: 393: 381: 375: 371: 370: 362: 359: 347: 341: 337: 332: 331: 322: 319: 314: 310: 306: 302: 298: 294: 290: 286: 279: 272: 269: 262: 258: 255: 253: 250: 249: 245: 243: 241: 237: 233: 229: 225: 221: 217: 212: 210: 206: 202: 198: 194: 192: 188: 184: 180: 176: 172: 168: 164: 157:Complications 156: 154: 152: 148: 144: 140: 136: 132: 128: 124: 119: 116: 114: 110: 107:, especially 106: 98: 96: 94: 89: 87: 82: 80: 76: 73:is a type of 72: 62: 56: 53: 51: 47: 42: 38: 33: 29: 25: 20: 1548: 1534:C1-inhibitor 1532: 1449: 1442: 1405: 1404: 1389: 1379: 1373: 1346:ICF syndrome 1150: 1139: 1128: 1113: 1098: 1045: 1041: 1017:November 18, 1015:. Retrieved 987: 983: 973: 963:November 18, 961:. Retrieved 933: 929: 919: 909:November 18, 907:. Retrieved 879: 875: 865: 838: 834: 824: 814:November 19, 812:. Retrieved 808: 799: 789:November 18, 787:. Retrieved 748: 741: 696: 692: 682: 637: 633: 623: 613:November 18, 611:. Retrieved 581: 577: 567: 522: 518: 462: 458: 448: 438:November 18, 436:. Retrieved 405: 383:. Retrieved 368: 361: 349:. Retrieved 329: 321: 288: 284: 271: 236:polymyositis 213: 195: 160: 120: 117: 109:encapsulated 102: 90: 83: 70: 69: 1406:peripheral: 385:13 November 230:. Subacute 27:Other names 1539:Angioedema 1525:deficiency 1523:Complement 1500:Leukopenia 1450:autosomal: 1204:complement 1141:DiseasesDB 805:"UpToDate" 263:References 143:septicemia 139:meningitis 86:infections 55:Hematology 1443:x-linked: 1157:234599007 1152:SNOMED CT 1062:0161-5890 1004:0028-4793 950:0003-987X 896:0002-9343 776:0065-2776 715:1465-9905 656:0893-8512 600:0025-7974 541:0893-8512 489:0077-8923 433:243396344 175:arthritis 50:Specialty 1598:Category 1490:HIV/AIDS 1482:Acquired 1433:combined 1225:Antibody 1200:Lymphoid 1070:16026838 809:UpToDate 784:11079100 733:12110148 674:20930072 608:15643297 578:Medicine 497:19758139 305:12959222 246:See also 218:such as 1229:humoral 1216:Primary 1012:2582254 958:3467658 904:1090155 857:4583776 848:1553813 724:3240161 665:2952982 559:1889047 467:Bibcode 313:1198276 95:(SLE). 1446:X-SCID 1430:Severe 1135:217000 1068:  1060:  1010:  1002:  956:  948:  902:  894:  855:  845:  782:  774:  764:  731:  721:  713:  672:  662:  654:  606:  598:  557:  550:358203 547:  539:  495:  487:  431:  421:  376:  351:30 May 342:  311:  303:  238:, and 226:, and 177:, and 57:  1436:(B+T) 1334:Other 1124:279.8 1109:D84.1 429:S2CID 309:S2CID 281:(PDF) 203:. In 167:fever 1202:and 1146:1847 1130:OMIM 1119:9-CM 1066:PMID 1058:ISSN 1019:2023 1008:PMID 1000:ISSN 965:2023 954:PMID 946:ISSN 911:2023 900:PMID 892:ISSN 853:PMID 816:2023 791:2023 780:PMID 772:ISSN 762:ISBN 729:PMID 711:ISSN 670:PMID 652:ISSN 615:2023 604:PMID 596:ISSN 555:PMID 537:ISSN 493:PMID 485:ISSN 463:1173 440:2023 419:ISBN 387:2010 374:ISBN 353:2010 340:ISBN 301:PMID 171:rash 149:and 131:IgG4 129:and 127:IgG2 1115:ICD 1100:ICD 1050:doi 992:doi 988:313 938:doi 934:123 884:doi 843:PMC 754:doi 719:PMC 701:doi 660:PMC 642:doi 586:doi 545:PMC 527:doi 475:doi 411:doi 338:–. 293:doi 205:SLE 197:SLE 183:ANA 163:SLE 141:or 1600:: 1155:: 1144:: 1133:: 1122:: 1107:: 1104:10 1064:. 1056:. 1046:43 1044:. 1040:. 1006:. 998:. 986:. 982:. 952:. 944:. 932:. 928:. 898:. 890:. 880:58 878:. 874:. 851:. 839:14 837:. 833:. 807:. 778:. 770:. 760:. 727:. 717:. 709:. 695:. 691:. 668:. 658:. 650:. 638:23 636:. 632:. 602:. 594:. 582:84 580:. 576:. 553:. 543:. 535:. 521:. 517:. 505:^ 491:. 483:. 473:. 461:. 457:. 427:. 417:. 395:^ 336:11 307:. 299:. 289:23 287:. 283:. 234:, 222:, 173:, 169:, 145:. 88:. 81:. 1551:/ 1545:) 1541:/ 1537:( 1502:: 1421:) 1419:1 1417:( 1401:) 1392:( 1386:) 1382:( 1377:: 1367:) 1365:T 1363:( 1316:) 1314:5 1309:4 1304:3 1299:2 1294:1 1292:( 1236:) 1234:B 1232:( 1227:/ 1192:e 1185:t 1178:v 1117:- 1102:- 1092:D 1072:. 1052:: 1021:. 994:: 967:. 940:: 913:. 886:: 859:. 818:. 793:. 756:: 735:. 703:: 697:4 676:. 644:: 617:. 588:: 561:. 529:: 523:4 499:. 477:: 469:: 442:. 413:: 389:. 355:. 315:. 295::

Index


Specialty
Hematology
Edit this on Wikidata
complement deficiency
complement component 2
infections
systemic lupus erythematosus
bacterial infections
encapsulated
Systemic Lupus Erythematosus
immunoglobulin
IgG2
IgG4
Streptococcus pneumoniae
meningitis
septicemia
Haemophilus influenza type b
Neisseria meningitis
SLE
fever
rash
arthritis
glomerulonephritis
ANA
Anti-double-stranded DNA antibodies
Anti-Ro antibodies
SLE
complement deficiencies
SLE

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