69:
37:
259:
1077:
328:
988:
973:
954:
1123:
216:
229:
gene on the X chromosome. Most patients develop diabetes and diarrhea and many die due to autoimmune activity against many organs. Boys are affected, while girls are carriers and might experience mild disease.
724:
608:
1243:
1116:
1109:
1722:
1236:
775:
159:, although non-endocrine organs can be affected. There are three types of APS, and there are a number of other diseases which involve endocrine autoimmunity.
1229:
1857:
842:
Improda, Nicola; Capalbo, Donatella; Cirillo, Emilia; Cerbone, Manuela; Esposito, Andrea; Pignata, Claudio; Salerno, Mariacarolina (1 November 2014).
716:
600:
1805:
1800:
1401:
1154:
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754:
202:
168:
85:
80:
1756:
1578:
1181:
630:"Immunodysregulation, polyendocrinopathy and enteropathy X-linked | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"
1176:
1171:
1003:
896:
844:"Cutaneous vasculitis in patients with autoimmune polyendocrine syndrome type 1: report of a case and brief review of the literature"
1573:
906:
629:
571:
1432:
311:
1568:
1614:
1476:
485:
1471:
1210:
384:
1694:
1847:
1751:
1166:
660:"Clinical and molecular features of the immunodysregulation, polyendocrinopathy, enteropathy, X linked (IPEX) syndrome"
1842:
1653:
1456:
1684:
1362:
572:"Autoimmune polyglandular syndrome type 2 | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"
1714:
1537:
316:
1628:
1397:
1383:
1017:
209:
syndrome due to multifactorial gene involvement resulting in adrenal insufficiency plus hypothyroidism and/or
1852:
1501:
1264:
1632:
1092:
746:
184:
238:
Each "type" of this condition has a different genetic cause. IPEX syndrome is inherited in males by an
1790:
1785:
1769:
1679:
1609:
1466:
1378:
45:
1812:
992:
172:
68:
545:
519:
1817:
1699:
1689:
1583:
1481:
1461:
1298:
1256:
1161:
239:
220:
206:
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1509:
1101:
1519:
1514:
1314:
1303:
1028:
967:
922:
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883:
865:
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769:
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679:
501:
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352:
105:
57:
963:
789:
Weiler, Fernanda GuimarĂŁes; Dias-da-Silva, Magnus R.; Lazaretti-Castro, Marise (2012-02-01).
1563:
1497:
1414:
1409:
1350:
1277:
1252:
873:
855:
802:
687:
671:
493:
455:
417:"Type I Polyglandular Autoimmune Syndrome: Practice Essentials, Background, Pathophysiology"
1221:
546:"Type II Polyglandular Autoimmune Syndrome: Practice Essentials, Pathophysiology, Etiology"
1704:
1674:
1558:
1486:
1340:
1293:
1193:
1133:
210:
156:
416:
295:
For this condition, differential diagnosis sees that the following should be considered:
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637:
579:
1588:
1542:
1335:
878:
843:
692:
659:
36:
997:
1836:
1763:
1355:
90:
62:
807:
790:
272:
of this condition for example, sees that the following methods/tests are available:
1658:
1448:
1330:
1319:
1057:
520:"Type III Polyglandular Autoimmune Syndrome: Background, Pathophysiology, Etiology"
340:
331:
188:
148:
1022:
747:"Orphanet: Immune dysregulation polyendocrinopathy enteropathy X linked syndrome"
1345:
1325:
1309:
791:"Autoimmune polyendocrine syndrome type 1: case report and review of literature"
376:
247:
196:
52:
gene, which causes autoimmune polyendocrine syndrome type 1 when non-functional)
1060:
1033:
926:
327:
982:
276:
258:
152:
869:
860:
816:
683:
1203:
1198:
1052:
444:"Polyglandular Autoimmune Syndromes: Immunogenetics and Long-Term Follow-Up"
887:
824:
701:
505:
469:
460:
443:
1188:
675:
497:
483:
Eisenbarth GS, Gottlieb PA (2004). "Autoimmune polyendocrine syndromes".
250:
location is Xp11.23, is involved in the mechanism of the IPEX condition.
192:
1268:
281:
262:
946:
931:
1637:
977:
958:
305:
658:
Wildin, R. S.; Smyk-Pearson, S.; Filipovich, A. H. (1 August 2002).
337:
Immunosuppressive therapy may be used in type I of this condition.
217:
Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome
1604:
1272:
225:
1011:
1008:
299:
1225:
1105:
895:
Shoenfeld, Yehuda; Cervera, Ricard; Gershwin, M. Eric (2008).
795:
448:
The
Journal of Clinical Endocrinology & Metabolism
343:
can also be used for type I under certain conditions.
936:
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1713:
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1627:
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1536:
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1426:
1396:
1371:
1286:
1263:
1043:
940:
114:
104:
96:
76:
56:
26:
21:
1723:Transfusion-associated graft-versus-host disease
1237:
1117:
8:
442:Dittmar, Manuela; Kahaly, George J. (2003).
774:: CS1 maint: numeric names: authors list (
1741:
1643:
1548:
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1283:
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1124:
1110:
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937:
898:Diagnostic Criteria in Autoimmune Diseases
67:
35:
18:
901:. Springer Science & Business Media.
877:
859:
806:
691:
459:
30:Autoimmune polyglandular syndromes (APSs)
326:
257:
203:Autoimmune polyendocrine syndrome type 2
169:Autoimmune polyendocrine syndrome type 1
100:FOXP3 gene is involved in the mechanism
1757:Allergic bronchopulmonary aspergillosis
364:
767:
411:
409:
407:
405:
403:
401:
1579:Post-streptococcal glomerulonephritis
745:RESERVED, INSERM US14 -- ALL RIGHTS.
7:
370:
368:
141:polyendocrine autoimmune syndromes
133:polyglandular autoimmune syndromes
125:Autoimmune polyendocrine syndromes
14:
1796:Autoimmune polyendocrine syndrome
1145:Autoimmune polyendocrine syndrome
1093:Autoimmune polyendocrine syndrome
22:Autoimmune polyendocrine syndrome
1858:Diseases of immune dysregulation
1433:Hemolytic disease of the newborn
1075:
312:Severe combined immunodeficiency
175:syndrome due to mutation of the
147:), are a heterogeneous group of
1615:Subacute bacterial endocarditis
1477:Immune thrombocytopenic purpura
808:10.1590/S0004-27302012000100009
757:from the original on 2017-04-19
727:from the original on 2017-04-17
611:from the original on 2017-04-17
387:from the original on 2017-04-17
155:activity against more than one
1:
1695:Postorgasmic illness syndrome
1132:Disorders involving multiple
1752:Hypersensitivity pneumonitis
1167:Multiple endocrine neoplasia
1818:Systemic autoimmune disease
1654:Allergic contact dermatitis
1574:Hypersensitivity vasculitis
1457:Autoimmune hemolytic anemia
664:Journal of Medical Genetics
1874:
1324:common allergies include:
715:Reference, Genetics Home.
375:Reference, Genetics Home.
1211:Woodhouse–Sakati syndrome
1140:
634:rarediseases.info.nih.gov
576:rarediseases.info.nih.gov
317:X linked thrombocytopenia
43:
34:
1569:Henoch–Schönlein purpura
1384:Eosinophilic esophagitis
861:10.1186/1471-2431-14-272
1685:Hashimoto's thyroiditis
1472:Guillain–Barré syndrome
721:Genetics Home Reference
605:Genetics Home Reference
381:Genetics Home Reference
223:due to mutation of the
1786:Autoimmune adrenalitis
461:10.1210/jc.2002-021845
334:
291:Differential diagnosis
265:
330:
261:
185:adrenal insufficiency
1791:Autoimmune hepatitis
1770:Transplant rejection
1680:Giant cell arteritis
1610:Rheumatoid arthritis
1467:Goodpasture syndrome
1379:Autoimmune urticaria
676:10.1136/jmg.39.8.537
498:10.1056/NEJMra030158
46:autoimmune regulator
1848:Autoimmune diseases
1257:autoimmune diseases
219:(IPEX syndrome) is
173:autosomal recessive
110:Endoscopic, CT scan
48:protein (from the
1843:Endocrine diseases
1700:Reactive arthritis
1690:Multiple sclerosis
1584:Reactive arthritis
1482:Pemphigus vulgaris
1462:Bullous pemphigoid
1363:Penicillin allergy
1299:Allergic urticaria
1162:Carcinoid syndrome
1044:External resources
335:
266:
240:X-linked recessive
221:X-linked recessive
207:autosomal dominant
181:hypoparathyroidism
179:gene resulting in
1830:
1829:
1826:
1825:
1731:
1730:
1623:
1622:
1532:
1531:
1528:
1527:
1520:Pernicious anemia
1515:Myasthenia gravis
1392:
1391:
1315:Atopic dermatitis
1304:Allergic rhinitis
1219:
1218:
1070:
1069:
923:Diseases Database
552:. 8 December 2023
353:Immunosuppression
151:characterized by
122:
121:
106:Diagnostic method
16:Medical condition
1865:
1813:Sjögren syndrome
1742:
1644:
1549:
1445:
1424:
1284:
1253:Hypersensitivity
1246:
1239:
1232:
1223:
1134:endocrine glands
1126:
1119:
1112:
1103:
1079:
1078:
938:
912:
891:
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863:
829:
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810:
786:
780:
779:
773:
765:
763:
762:
742:
736:
735:
733:
732:
712:
706:
705:
695:
655:
649:
648:
646:
645:
636:. Archived from
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620:
619:
617:
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597:
591:
590:
588:
587:
578:. Archived from
568:
562:
561:
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542:
536:
535:
533:
531:
516:
510:
509:
486:N. Engl. J. Med.
480:
474:
473:
463:
454:(7): 2983–2992.
439:
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432:
430:
428:
413:
396:
395:
393:
392:
372:
72:
71:
39:
19:
1873:
1872:
1868:
1867:
1866:
1864:
1863:
1862:
1833:
1832:
1831:
1822:
1774:
1737:
1727:
1709:
1705:Type 1 diabetes
1675:Coeliac disease
1663:
1635:
1619:
1593:
1559:Arthus reaction
1540:
1524:
1510:Graves' disease
1491:
1487:Rheumatic fever
1436:
1404:
1388:
1367:
1294:Allergic asthma
1275:
1259:
1250:
1220:
1215:
1194:Werner syndrome
1136:
1130:
1100:
1099:
1098:
1080:
1076:
1071:
1066:
1065:
1039:
1038:
949:
919:
909:
894:
841:
838:
836:Further reading
833:
832:
788:
787:
783:
766:
760:
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744:
743:
739:
730:
728:
717:"IPEX syndrome"
714:
713:
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657:
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652:
643:
641:
628:
627:
623:
614:
612:
601:"IPEX syndrome"
599:
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583:
570:
569:
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555:
553:
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543:
539:
529:
527:
518:
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492:(20): 2068–79.
482:
481:
477:
441:
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436:
426:
424:
415:
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399:
390:
388:
374:
373:
366:
361:
349:
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293:
286:Histologic test
256:
236:
211:type 1 diabetes
165:
157:endocrine organ
131:), also called
118:Depends on type
89:
84:
66:
17:
12:
11:
5:
1871:
1869:
1861:
1860:
1855:
1853:Rare syndromes
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1621:
1620:
1618:
1617:
1612:
1607:
1601:
1599:
1595:
1594:
1592:
1591:
1589:Serum sickness
1586:
1581:
1576:
1571:
1566:
1561:
1555:
1553:
1546:
1543:Immune complex
1534:
1533:
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1526:
1525:
1523:
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1512:
1506:
1504:
1493:
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1381:
1375:
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1360:
1359:
1358:
1353:
1348:
1343:
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1317:
1312:
1307:
1301:
1296:
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1234:
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1196:
1186:
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1179:
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942:
941:Classification
935:
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917:External links
915:
914:
913:
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892:
848:BMC Pediatrics
837:
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781:
737:
707:
670:(8): 537–545.
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621:
592:
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537:
526:. 29 June 2022
511:
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423:. 29 June 2022
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268:Diagnosis for
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1765:
1764:Latex allergy
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1666:
1660:
1657:
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1645:
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1634:
1633:cell-mediated
1630:
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1596:
1590:
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1577:
1575:
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1570:
1567:
1565:
1564:Farmer's lung
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751:www.orpha.net
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640:on 2019-01-09
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582:on 2017-04-13
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189:hypogonadism
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1310:Anaphylaxis
1306:(Hay fever)
248:cytogenetic
199:and others.
197:candidiasis
27:Other names
1837:Categories
1779:Autoimmune
1668:Autoimmune
1598:Autoimmune
1441:Autoimmune
1372:Autoimmune
1029:DiseasesDB
761:2017-05-11
731:2017-05-11
644:2017-04-20
615:2017-04-20
586:2017-04-20
391:2017-05-11
359:References
323:Management
308:deficiency
302:deficiency
277:Endoscopic
153:autoimmune
86:APS type 2
1449:Cytotoxic
1204:Metageria
1199:Acrogeria
1053:eMedicine
870:1471-2431
817:0004-2730
684:0022-2593
254:Diagnosis
115:Treatment
81:APS type1
58:Specialty
1738:multiple
1736:Unknown/
1538:Type III
1502:receptor
1351:Tree nut
1189:Progeria
1061:med/1868
1058:med/1867
888:25361846
825:22460196
770:cite web
755:Archived
725:Archived
702:12161590
609:Archived
556:28 April
550:Medscape
530:28 April
524:MEDSCAPE
506:15141045
470:12843130
427:28 April
421:MEDSCAPE
385:Archived
347:See also
193:vitiligo
1745:Foreign
1647:Foreign
1638:T cells
1629:Type IV
1552:Foreign
1427:Foreign
1398:Type II
1341:Seafood
1287:Foreign
1269:allergy
1083:Scholia
1023:D016884
925:(DDB):
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282:CT scan
263:CT scan
1766:(I+IV)
1498:Type V
1336:Peanut
1265:Type I
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234:Cause
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