71:
334:
288:(PCP), which is common in infants with hyper IgM syndrome, is a serious illness. PCP is one of the most frequent and severe opportunistic infections in people with weakened immune systems. Many CD40 Ligand Deficiency are first diagnosed after having PCP in their first year of life. The fungus is common and is present in over 70% of healthy people's lungs, however, Hyper IgM patients are not able to fight it off without the administration of
2593:
2623:
2633:
1369:
2613:
270:
45:
2603:
484:
in males. Limited data is available on the frequency of AICDA deficiency, another subtype of X-HIGM, but it is believed to affect less than 1 in 1,000,000 individuals. Globally, all forms of HIGM make up approximately 0.3% to 2.9% of all patients diagnosed with primary immunodeficiency disorders (PIDs).
360:
efficiently switch to other types of antibodies as needed to attack invading bacteria, viruses, and other pathogens. In people with hyper IgM syndromes, the B cells keep making IgM antibodies because they can't switch to a different antibody. This results in an overproduction of IgM antibodies and an
483:
All forms of hyper-IgM syndrome are rare. According to the US X-HIGM registry, the prevalence of X-linked hyper IgM syndrome (X-HIGM) during the period from 1984 to 1993 was approximately 1 in 1,000,000 live births. The estimated frequency of CD40L deficiency, a subtype of X-HIGM, is 2 in 1,000,000
843:
Leven, Emily A.; Maffucci, Patrick; Ochs, Hans D.; Scholl, Paul R.; Buckley, Rebecca H.; Fuleihan, Ramsay L.; Geha, Raif S.; Cunningham, Coleen K.; Bonilla, Francisco A.; Conley, Mary Ellen; Ferdman, Ronald M.; Hernandez-Trujillo, Vivian; Puck, Jennifer M.; Sullivan, Kathleen; Secord, Elizabeth A.
261:
Heterozygous females in X-linked hyper IgM syndrome (HIGM1) are usually asymptomatic. However, immunologic testing has revealed that they exhibit reduced expression of CD40L when CD4+ T lymphocytes are activated. In some cases, females with significant reduction in circulating lymphocytes carrying
248:
The majority of patients with HIGM syndrome present with a broad spectrum of clinical symptoms even with a same genetic defects. They usually develop symptoms in infancy and second year of life, including increased susceptibility to infections by extracellular bacteria, sinus & ear infections,
1153:
Vavassori, Valentina; Mercuri, Elisabetta; Marcovecchio, Genni E; Castiello, Maria C; Schiroli, Giulia; Albano, Luisa; Margulies, Carrie; Buquicchio, Frank; Fontana, Elena; Beretta, Stefano; Merelli, Ivan; Cappelleri, Andrea; Rancoita, Paola MV; Lougaris, Vassilios; Plebani, Alessandro (5 March
462:
The primary goal is to address the underlying defect in CD40L or other gene mutations causing HIGM. The potential for precise correction of the CD40LG gene in T cells and hematopoietic stem/progenitor cells (HSPC) to treat X-linked hyper-IgM Syndrome (HIGM1) is a promising avenue of research.
463:
However, the actual therapeutic efficacy of this approach is not yet fully understood and requires further investigation to determine its true potential. In addition to HSCT, supportive measures are crucial for managing infections and complications associated with HIGM. This may include
1308:
1293:
421:. In females, the diagnosis of HIGM1 is extremely rare. Heterozygous females are usually asymptomatic unless there is skewed X-chromosome inactivation. The diagnosis of hyper IgM syndrome can be done via the following methods and tests:
1415:
901:
Lanzi, Gaetana; Ferrari, Simona; Vihinen, Mauno; Caraffi, Stefano; Kutukculer, Necil; Schiaffonati, Luisa; Plebani, Alessandro; Notarangelo, Luigi
Daniele; Maria Fra, Anna; Giliani, Silvia (23 December 2010).
416:
The diagnosis of X-linked hyper IgM syndrome (HIGM1) is established in males with typical clinical and laboratory findings by identifying a hemizygous pathogenic variant in the CD40LG gene through molecular
126:
They are resulting from mutations in the pathway from B-cell activation to isotype class switching. Patients with HIGM are usually diagnosed within the first two years of life and experience severe
400:
and antigen presenting cells (APCs) is known as the underlying cause of HIGM syndromes. CD40L-CD40 interaction is the first step in B cell stimulation for class switch recombination (CSR) and
1408:
1401:
217:
caused by the mutation in the NEMO (nuclear factor ÎșB essential modulator) complex, which, when mutated, is unable to phosphorylate IÎșB downstream of CD40 signaling.
2662:
130:. This syndrome is also known as immunoglobulin class switch recombination (Ig-CSR) deficiencies. The most common causes are mutations in the CD40 Ligand (
2122:
1633:
1481:
467:, immunoglobulin replacement therapy, and close monitoring of respiratory and gastrointestinal infections. Additionally, anti-microbial therapy, use of
2463:
1670:
734:
Yazdani, Reza; Fekrvand, Saba; Shahkarami, Sepideh; Azizi, Gholamreza; Moazzami, Bobak; Abolhassani, Hassan; Aghamohammadi, Asghar (1 January 2019).
2062:
1797:
2657:
1499:
468:
2362:
962:
Dunn, Clinton P.; de la Morena, M. Teresa (1993), Adam, Margaret P.; Mirzaa, Ghayda M.; Pagon, Roberta A.; Wallace, Stephanie E. (eds.),
1802:
2498:
1840:
1254:
1226:
1156:"Modeling, optimization, and comparable efficacy of T cell and hematopoietic stem cell gene editing for treating hyperâIgM syndrome"
2616:
1737:
1654:
1565:
498:
263:
408:
is affected. Certain insults, usually from encapsulated bacteria and toxin, then have a greater opportunity to damage the body.
2488:
1922:
1807:
1677:
1549:
198:
162:
289:
1544:
2468:
2112:
1244:
1657:
1509:
1476:
1216:
493:
2092:
180:
1623:
2521:
1643:
1538:
1533:
1528:
1523:
1518:
1453:
235:
221:
213:
187:
169:
147:
2313:
2235:
2230:
2150:
1910:
1692:
1440:
1323:
231:
gene. UNG is responsible for the cleavage of cytosines that have been deaminated by AID in single-stranded DNA.
120:
1393:
2667:
2293:
2267:
1792:
1777:
1773:
1384:
436:
405:
250:
1885:
1833:
786:"Class-Switch Recombination (CSR)/Hyper-IgM (HIGM) Syndromes and Phosphoinositide 3-Kinase (PI3K) Defects"
464:
282:
227:
123:
characterized by low or absent levels of serum IgG, IgA, IgE and normal or increased levels of serum IgM.
262:
the CD40L mutation due to skewed X-chromosome inactivation can present with symptoms resembling HIGM1 or
239:
is the least-characterized of the HIGM types, as the gene is unknown. Resembles HIGM2, but AID is normal.
2536:
2422:
2395:
2368:
2320:
2274:
1969:
1767:
1747:
1468:
736:"The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management"
447:
Flow
Cytometry (evaluate the presence and function of certain immune cells, such as T cells and B cells)
401:
202:
903:
735:
2556:
2516:
2383:
2197:
2192:
2172:
2107:
1959:
1787:
1491:
205:
with CD40L deficiency but B cells from CD40 deficient patients are unable to undergo class switching
70:
2636:
2578:
2561:
2220:
1937:
1312:
165:. Therefore, the only antibody secreted by the B cell is IgM, the least specific class of antibody.
2622:
2526:
2483:
2473:
2262:
2215:
2182:
2177:
1927:
1905:
941:
763:
472:
254:
79:
1102:
784:
Jhamnani, Rekha D.; Nunes-Santos, Cristiane J.; Bergerson, Jenna; Rosenzweig, Sergio D. (2018).
2596:
2478:
2448:
2427:
2390:
2373:
2288:
2252:
2242:
2137:
2057:
2001:
1964:
1949:
1890:
1857:
1826:
1608:
1599:
1584:
1428:
1250:
1222:
1193:
1175:
1083:
1075:
1034:
1016:
971:
933:
925:
883:
865:
825:
807:
755:
703:
127:
97:
59:
197:
gene and it is inherited by autosomal recessive manner. It has similar phenotype of impaired
161:
results in defective signaling to B cells, which do not receive the needed signal to undergo
2298:
2283:
2160:
2035:
1991:
1706:
1687:
1618:
1432:
1424:
1183:
1167:
1065:
1024:
1008:
915:
873:
857:
815:
797:
747:
366:
333:
52:
529:"X-linked Immunodeficiency With Hyper IgM Clinical Presentation: History, Physical, Causes"
2626:
2571:
2432:
2417:
2400:
2340:
2335:
2279:
2257:
1954:
1900:
1880:
1729:
418:
393:
1374:
528:
2458:
2412:
2225:
2155:
1974:
1782:
1682:
1504:
1317:
1188:
1155:
1070:
1053:
1029:
996:
878:
845:
820:
785:
321:
306:
2651:
2541:
2330:
2325:
2187:
2127:
2030:
1012:
397:
341:
Different genetic defects cause HIgM syndrome, the vast majority are inherited as an
945:
767:
2606:
2546:
2531:
2357:
2082:
1932:
1917:
1863:
1758:
1570:
1352:
17:
1328:
920:
2405:
2350:
2247:
2102:
2077:
1895:
453:
Blood test(Immunoglobulin levels, Antibody response, Complete blood count (CBC))
431:
311:
2493:
751:
266:. Among the presentation consistent with hyper IgM syndrome are the following:
2378:
2345:
2117:
2067:
2052:
2047:
2040:
2025:
2015:
1763:
1724:
1302:
861:
695:
441:
258:
64:
1179:
1079:
1020:
963:
929:
869:
811:
802:
759:
2566:
2308:
2303:
2167:
2145:
2020:
1984:
1347:
1171:
316:
294:
278:
1197:
1087:
1038:
975:
937:
887:
829:
707:
694:
Johnson, Judith; Filipovich, Alexandra H.; Zhang, Kejian (1 January 1993).
209:
upon activation with agonists and cytokines as per their intrinsic defect .
269:
2072:
1849:
1714:
1449:
904:"Different molecular behavior of CD40 mutants causing hyper-IgM syndrome"
353:
349:
342:
301:
206:
179:
gene. In this type, B cells cannot recombine genetic material to change
1285:
638:"OMIM Entry â # 608106 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 5; HIGM5"
610:"OMIM Entry â # 606843 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 3; HIGM3"
585:"OMIM Entry â # 605258 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 2; HIGM2"
557:"OMIM Entry â # 308230 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 1; HIGM1"
404:(SHM) resulting in the generation of various Ig isotypes. Consequently,
2551:
2011:
1979:
1872:
357:
134:) gene located at Xq26.3-27 leading to X-linked HIGM (XHIGM) in males.
44:
663:"OMIM Entry â 608184 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 4; HIGM4"
1589:
1458:
1297:
385:
158:
153:
1128:
662:
609:
556:
389:
268:
175:
381:
193:
1822:
1397:
637:
584:
425:
370:
362:
345:
recessive genetic trait and most with the condition are male.
1218:
Immunology: Clinical Case
Studies and Disease Pathophysiology
1818:
249:
skin infections. Furthermore, these patients are prone to
142:
Five types of hyper IgM syndrome have been characterized:
995:
Davies, E Graham; Thrasher, Adrian J (27 November 2016).
173:(autosomal recessive), characterized by mutations of the
846:"Hyper IgM Syndrome: a Report from the USIDNET Registry"
352:
that all B cells produce initially before they undergo
257:, hematologic abnormalities, lymphoproloferation and
157:
gene. In this type, lack of CD40L on the surfaces of
1275:
2509:
2447:
2208:
2136:
2091:
2000:
1871:
1856:
1746:
1723:
1705:
1653:
1583:
1558:
1490:
1467:
1448:
1439:
1338:
1279:
970:, Seattle (WA): University of Washington, Seattle,
106:
96:
88:
78:
58:
37:
356:due to exposure to a recognized antigen. Healthy
997:"Update on the hyper immunoglobulin M syndromes"
1052:Etzioni, Amos; Ochs, Hans D. (1 October 2004).
475:, as well as other treatments, may be needed.
396:. Defective interaction of CD40L-CD40 between
151:(X-linked), characterized by mutations of the
1834:
1409:
1215:Strober, Warren; Gottesman, Susan R. (2014).
110:Allogeneic hematopoietic cell transplantation
8:
1246:Walker's Pediatric Gastrointestinal Disease
2123:Reproductive endocrinology and infertility
1868:
1841:
1827:
1819:
1634:Purine nucleoside phosphorylase deficiency
1482:Transient hypogammaglobulinemia of infancy
1464:
1445:
1416:
1402:
1394:
1276:
1054:"The Hyper IgM SyndromeâAn Evolving Story"
69:
43:
34:
2464:Bachelor of Medicine, Bachelor of Surgery
1187:
1069:
1028:
919:
877:
819:
801:
332:
92:Hyper-IgM syndrome type 1, 2, 3, 4 and 5
511:
1798:Terminal complement pathway deficiency
523:
521:
519:
517:
515:
957:
955:
689:
687:
685:
683:
632:
630:
579:
577:
551:
549:
469:granulocyte colony-stimulating factor
7:
2602:
2363:Physical medicine and rehabilitation
779:
777:
729:
727:
725:
2663:Predominantly antibody deficiencies
2612:
1803:Paroxysmal nocturnal hemoglobinuria
253:, gastrointestinal manifestations,
121:primary immune deficiency disorders
27:Primary immune deficiency disorders
2499:Medical Scientist Training Program
1071:10.1203/01.PDR.0000139318.65842.4A
225:characterized by mutations of the
191:characterized by mutations of the
25:
388:that, when bound to CD40 ligand (
2631:
2621:
2611:
2601:
2592:
2591:
1566:Common variable immunodeficiency
1367:
1013:10.1111/j.1365-2141.2010.08077.x
499:Common variable immunodeficiency
384:is a co-stimulatory receptor on
264:common variable immunodeficiency
2632:
1738:Idiopathic CD4+ lymphocytopenia
2489:Doctor of Osteopathic Medicine
1923:Oral and maxillofacial surgery
1808:Complement receptor deficiency
1678:Adenosine deaminase deficiency
1001:British Journal of Haematology
850:Journal of Clinical Immunology
1:
1103:"X-linked hyper IgM syndrome"
964:"X-Linked Hyper IgM Syndrome"
696:"X-Linked Hyper IgM Syndrome"
2658:Syndromes affecting immunity
2469:Bachelor of Medical Sciences
2236:Neurosurgical anesthesiology
921:10.1182/blood-2010-03-274241
1477:X-linked agammaglobulinemia
494:X-linked agammaglobulinemia
2684:
1127:Reference, Genetics Home.
1101:Reference, Genetics Home.
752:10.1016/j.clim.2018.11.007
337:Class switch recombination
199:class switch recombination
2587:
1243:Kleinman, Ronald (2008).
1221:. John Wiley & Sons.
862:10.1007/s10875-016-0291-4
465:antimicrobial prophylaxis
392:), sends a signal to the
236:Hyper-IgM syndrome type 6
222:Hyper-IgM syndrome type 5
214:Hyper-IgM syndrome type 4
188:Hyper-IgM syndrome type 3
170:Hyper-IgM syndrome type 2
148:Hyper-IgM syndrome type 1
51:
42:
2423:Transplantation medicine
2314:Clinical neurophysiology
2231:Obstetric anesthesiology
2151:Interventional radiology
1911:Digestive system surgery
1693:Bare lymphocyte syndrome
1545:WiskottâAldrich syndrome
803:10.3389/fimmu.2018.02172
2294:Intensive care medicine
2268:Mass gathering medicine
2113:Maternalâfetal medicine
1793:Complement 3 deficiency
1778:Complement 4 deficiency
1774:Complement 2 deficiency
1172:10.15252/emmm.202013545
1160:EMBO Molecular Medicine
1133:Genetics Home Reference
1107:Genetics Home Reference
790:Frontiers in Immunology
437:Pulmonary function test
406:humoral immune response
251:pulmonary complications
1886:Cardiothoracic surgery
533:emedicine.medscape.com
402:somatic hyper mutation
338:
274:
273:Pneumocystis pneumonia
203:somatic hyper mutation
102:MRI, Chest radiography
2537:Personalized medicine
2396:Reproductive medicine
2321:Occupational medicine
2275:Evolutionary medicine
1768:Hereditary angioedema
1624:Ataxiaâtelangiectasia
1469:Hypogammaglobulinemia
336:
272:
2557:Traditional medicine
2517:Alternative medicine
2384:Addiction psychiatry
2198:Transfusion medicine
2193:Medical microbiology
2108:Gynecologic oncology
1960:Reproductive surgery
1788:Properdin deficiency
1609:Di George's syndrome
1492:Dysgammaglobulinemia
255:autoimmune disorders
2579:History of medicine
2562:Veterinary medicine
2369:Preventive medicine
2221:Adolescent medicine
2063:Infectious diseases
740:Clinical Immunology
361:underproduction of
348:IgM is the form of
18:Hyper-IgM syndromes
2527:Molecular oncology
2484:Doctor of Medicine
2474:Master of Medicine
2391:Radiation oncology
2263:Emergency medicine
2216:Addiction medicine
2183:Clinical chemistry
2178:Clinical pathology
1970:Transplant surgery
1928:Orthopedic surgery
1906:Colorectal surgery
1640:Hyper IgM syndrome
1550:Hyper-IgE syndrome
1515:Hyper IgM syndrome
1431:disorders causing
1385:Hyper IgM syndrome
1339:External resources
1058:Pediatric Research
473:immunosuppressants
339:
275:
244:Signs and symptoms
117:Hyper IgM syndrome
38:Hyper IgM syndrome
2645:
2644:
2479:Master of Surgery
2443:
2442:
2428:Tropical medicine
2374:Prison healthcare
2289:Hospital medicine
2253:Disaster medicine
2243:Aviation medicine
2058:Hospital medicine
1965:Surgical oncology
1950:Pediatric surgery
1944:
1891:Endocrine surgery
1816:
1815:
1701:
1700:
1600:thymic hypoplasia
1585:T cell deficiency
1579:
1578:
1362:
1361:
914:(26): 5867â5874.
163:isotype switching
128:immunosuppression
114:
113:
98:Diagnostic method
32:Medical condition
16:(Redirected from
2675:
2635:
2634:
2625:
2615:
2614:
2605:
2604:
2595:
2594:
2299:Medical genetics
2284:General practice
2161:Nuclear medicine
2036:Gastroenterology
1992:Vascular surgery
1942:
1869:
1843:
1836:
1829:
1820:
1688:ZAP70 deficiency
1619:Nezelof syndrome
1465:
1446:
1433:immunodeficiency
1418:
1411:
1404:
1395:
1371:
1370:
1277:
1267:
1265:
1263:
1239:
1237:
1235:
1202:
1201:
1191:
1150:
1144:
1143:
1141:
1139:
1124:
1118:
1117:
1115:
1113:
1098:
1092:
1091:
1073:
1049:
1043:
1042:
1032:
992:
986:
985:
984:
982:
959:
950:
949:
923:
898:
892:
891:
881:
840:
834:
833:
823:
805:
781:
772:
771:
731:
720:
718:
716:
714:
691:
678:
677:
675:
673:
659:
653:
652:
650:
648:
634:
625:
624:
622:
620:
606:
600:
599:
597:
595:
581:
572:
571:
569:
567:
553:
544:
543:
541:
539:
525:
84:Chronic diarrhea
74:
73:
53:Immunoglobulin M
47:
35:
21:
2683:
2682:
2678:
2677:
2676:
2674:
2673:
2672:
2648:
2647:
2646:
2641:
2583:
2572:Chief physician
2505:
2450:
2439:
2433:Travel medicine
2418:Sports medicine
2401:Sexual medicine
2341:Palliative care
2336:Pain management
2280:Family medicine
2258:Diving medicine
2204:
2132:
2094:
2087:
2003:
1996:
1955:Plastic surgery
1901:General surgery
1881:Cardiac surgery
1862:
1860:
1852:
1847:
1817:
1812:
1749:
1742:
1730:Lymphocytopenia
1728:
1719:
1697:
1673:
1660:
1649:
1605:hypoparathyroid
1587:
1575:
1554:
1486:
1456:
1435:
1422:
1392:
1391:
1390:
1372:
1368:
1363:
1358:
1357:
1334:
1333:
1288:
1274:
1261:
1259:
1257:
1242:
1233:
1231:
1229:
1214:
1211:
1209:Further reading
1206:
1205:
1152:
1151:
1147:
1137:
1135:
1126:
1125:
1121:
1111:
1109:
1100:
1099:
1095:
1051:
1050:
1046:
994:
993:
989:
980:
978:
961:
960:
953:
900:
899:
895:
842:
841:
837:
783:
782:
775:
733:
732:
723:
712:
710:
693:
692:
681:
671:
669:
661:
660:
656:
646:
644:
636:
635:
628:
618:
616:
608:
607:
603:
593:
591:
583:
582:
575:
565:
563:
555:
554:
547:
537:
535:
527:
526:
513:
508:
490:
481:
460:
450:Genetic testing
419:genetic testing
414:
394:B-cell receptor
379:
377:Pathophysiology
354:class switching
331:
246:
140:
68:
33:
28:
23:
22:
15:
12:
11:
5:
2681:
2679:
2671:
2670:
2668:Rare syndromes
2665:
2660:
2650:
2649:
2643:
2642:
2640:
2639:
2629:
2619:
2609:
2599:
2588:
2585:
2584:
2582:
2581:
2576:
2575:
2574:
2564:
2559:
2554:
2549:
2544:
2539:
2534:
2529:
2524:
2519:
2513:
2511:
2510:Related topics
2507:
2506:
2504:
2503:
2502:
2501:
2491:
2486:
2481:
2476:
2471:
2466:
2461:
2459:Medical school
2455:
2453:
2445:
2444:
2441:
2440:
2438:
2437:
2436:
2435:
2425:
2420:
2415:
2413:Sleep medicine
2410:
2409:
2408:
2398:
2393:
2388:
2387:
2386:
2376:
2371:
2366:
2360:
2355:
2354:
2353:
2343:
2338:
2333:
2328:
2323:
2318:
2317:
2316:
2306:
2301:
2296:
2291:
2286:
2277:
2272:
2271:
2270:
2260:
2255:
2250:
2245:
2240:
2239:
2238:
2233:
2226:Anesthesiology
2223:
2218:
2212:
2210:
2206:
2205:
2203:
2202:
2201:
2200:
2195:
2190:
2185:
2180:
2175:
2165:
2164:
2163:
2158:
2156:Neuroradiology
2153:
2142:
2140:
2134:
2133:
2131:
2130:
2125:
2120:
2115:
2110:
2105:
2099:
2097:
2093:Obstetrics and
2089:
2088:
2086:
2085:
2080:
2075:
2070:
2065:
2060:
2055:
2050:
2045:
2044:
2043:
2033:
2028:
2023:
2018:
2008:
2006:
1998:
1997:
1995:
1994:
1989:
1988:
1987:
1977:
1975:Trauma surgery
1972:
1967:
1962:
1957:
1952:
1947:
1946:
1945:
1938:Otolaryngology
1935:
1930:
1925:
1920:
1915:
1914:
1913:
1908:
1898:
1893:
1888:
1883:
1877:
1875:
1866:
1864:subspecialties
1854:
1853:
1848:
1846:
1845:
1838:
1831:
1823:
1814:
1813:
1811:
1810:
1805:
1800:
1795:
1790:
1785:
1783:MBL deficiency
1780:
1771:
1754:
1752:
1744:
1743:
1741:
1740:
1734:
1732:
1721:
1720:
1718:
1717:
1711:
1709:
1703:
1702:
1699:
1698:
1696:
1695:
1690:
1685:
1683:Omenn syndrome
1680:
1664:
1662:
1651:
1650:
1648:
1647:
1628:
1627:
1621:
1612:
1595:
1593:
1581:
1580:
1577:
1576:
1574:
1573:
1568:
1562:
1560:
1556:
1555:
1553:
1552:
1547:
1542:
1536:
1531:
1526:
1521:
1512:
1510:IgM deficiency
1507:
1505:IgG deficiency
1502:
1500:IgA deficiency
1496:
1494:
1488:
1487:
1485:
1484:
1479:
1473:
1471:
1462:
1443:
1437:
1436:
1423:
1421:
1420:
1413:
1406:
1398:
1373:
1366:
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1364:
1360:
1359:
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1343:
1342:
1340:
1336:
1335:
1332:
1331:
1320:
1305:
1289:
1284:
1283:
1281:
1280:Classification
1273:
1272:External links
1270:
1269:
1268:
1255:
1240:
1227:
1210:
1207:
1204:
1203:
1145:
1119:
1093:
1064:(4): 519â525.
1044:
1007:(2): 167â180.
987:
951:
893:
856:(5): 490â501.
835:
773:
721:
679:
654:
626:
601:
573:
545:
510:
509:
507:
504:
503:
502:
496:
489:
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451:
448:
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434:
428:
413:
410:
378:
375:
330:
327:
326:
325:
324:(degenerative)
322:Encephalopathy
319:
314:
309:
307:Hypothyroidism
304:
298:
292:
245:
242:
241:
240:
232:
218:
210:
184:
166:
139:
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112:
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62:
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2548:
2545:
2543:
2542:Public health
2540:
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2533:
2530:
2528:
2525:
2523:
2522:Allied health
2520:
2518:
2515:
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2500:
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2199:
2196:
2194:
2191:
2189:
2188:Cytopathology
2186:
2184:
2181:
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2176:
2174:
2171:
2170:
2169:
2166:
2162:
2159:
2157:
2154:
2152:
2149:
2148:
2147:
2144:
2143:
2141:
2139:
2135:
2129:
2128:Urogynecology
2126:
2124:
2121:
2119:
2116:
2114:
2111:
2109:
2106:
2104:
2101:
2100:
2098:
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2056:
2054:
2051:
2049:
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2037:
2034:
2032:
2031:Endocrinology
2029:
2027:
2024:
2022:
2019:
2017:
2013:
2010:
2009:
2007:
2005:
1999:
1993:
1990:
1986:
1983:
1982:
1981:
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1971:
1968:
1966:
1963:
1961:
1958:
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1939:
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1641:
1638:
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1635:
1632:
1625:
1622:
1620:
1616:
1615:euparathyroid
1613:
1610:
1606:
1603:
1601:
1597:
1596:
1594:
1591:
1586:
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1287:
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1278:
1271:
1258:
1256:9781550093643
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1228:9781118966006
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897:
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346:
344:
335:
328:
323:
320:
318:
315:
313:
310:
308:
305:
303:
299:
297:(Hepatitis C)
296:
293:
291:
287:
285:
280:
277:
276:
271:
267:
265:
260:
259:malignancies.
256:
252:
243:
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2547:Rural health
2532:Nanomedicine
2083:Rheumatology
2014: /
1933:Hand surgery
1918:Neurosurgery
1759:C1-inhibitor
1757:
1674:
1667:
1639:
1630:
1629:
1614:
1604:
1598:
1571:ICF syndrome
1514:
1383:
1382:
1381:profile for
1378:
1346:
1322:
1307:
1292:
1260:. Retrieved
1249:. PMPH-USA.
1245:
1232:. Retrieved
1217:
1163:
1159:
1148:
1136:. Retrieved
1132:
1122:
1110:. Retrieved
1106:
1096:
1061:
1057:
1047:
1004:
1000:
990:
979:, retrieved
968:GeneReviewsÂź
967:
911:
907:
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739:
711:. Retrieved
699:
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666:
657:
645:. Retrieved
641:
617:. Retrieved
613:
604:
592:. Retrieved
588:
564:. Retrieved
560:
536:. Retrieved
532:
482:
479:Epidemiology
461:
415:
398:CD4+ T cells
380:
347:
340:
284:Pneumocystis
283:
247:
234:
226:
220:
212:
192:
186:
174:
168:
152:
146:
141:
131:
125:
116:
115:
29:
2617:Wikiproject
2406:Venereology
2351:Neonatology
2248:Dermatology
2103:Gynaecology
2095:gynaecology
2078:Pulmonology
1896:Eye surgery
1858:Specialties
1631:peripheral:
1262:27 November
1234:27 November
1138:27 November
1129:"CD40 gene"
1112:27 November
719:update 2013
713:12 November
700:GeneReviews
647:16 November
619:16 November
594:16 November
566:16 November
538:27 November
432:radiography
312:Neutropenia
181:heavy chain
2652:Categories
2379:Psychiatry
2365:(PM&R)
2358:Phlebology
2346:Pediatrics
2173:Anatomical
2138:Diagnostic
2118:Obstetrics
2068:Nephrology
2053:Hematology
2048:Geriatrics
2041:Hepatology
2026:Cardiology
2016:Immunology
1764:Angioedema
1750:deficiency
1748:Complement
1725:Leukopenia
1675:autosomal:
1429:complement
506:References
442:Lymph node
183:production
119:is a rare
65:Immunology
2567:Physician
2451:education
2309:Neurology
2304:Narcology
2168:Pathology
2146:Radiology
2021:Angiology
1985:Andrology
1668:x-linked:
1348:eMedicine
1180:1757-4676
1080:0031-3998
1021:0007-1048
930:0006-4971
870:0271-9142
812:1664-3224
760:1521-6616
746:: 19â30.
672:2 January
458:Treatment
412:Diagnosis
317:Arthritis
295:Hepatitis
286:pneumonia
279:Infection
107:Treatment
60:Specialty
2597:Category
2073:Oncology
2004:medicine
2002:Internal
1850:Medicine
1715:HIV/AIDS
1707:Acquired
1658:combined
1450:Antibody
1425:Lymphoid
1353:ped/2457
1198:33475257
1088:15319456
1039:20180797
976:20301576
946:33972331
938:20702779
888:27189378
830:30319630
768:53566466
708:20301576
667:omim.org
642:omim.org
614:omim.org
589:omim.org
561:omim.org
488:See also
350:antibody
343:X-linked
302:diarrhea
300:Chronic
207:in vitro
80:Symptoms
2637:Outline
2607:Commons
2552:Therapy
2449:Medical
2012:Allergy
1980:Urology
1873:Surgery
1454:humoral
1441:Primary
1375:Scholia
1329:D053306
1189:7933961
1154:2021).
1030:2855828
981:22 June
879:5039943
821:6168630
386:B cells
358:B cells
290:Bactrim
159:T cells
2627:Portal
2494:MDâPhD
1671:X-SCID
1655:Severe
1377:has a
1318:279.05
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1225:
1196:
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810:
766:
758:
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501:(CVID)
430:Chest
369:, and
154:CD40LG
132:CD40LG
67:
2209:Other
1661:(B+T)
1559:Other
1379:topic
1303:D80.5
942:S2CID
908:Blood
764:S2CID
390:CD40L
329:Cause
176:AICDA
138:Types
89:Types
1427:and
1324:MeSH
1313:9-CM
1264:2016
1251:ISBN
1236:2016
1223:ISBN
1194:PMID
1176:ISSN
1140:2016
1114:2016
1084:PMID
1076:ISSN
1035:PMID
1017:ISSN
983:2023
972:PMID
934:PMID
926:ISSN
884:PMID
866:ISSN
826:PMID
808:ISSN
756:ISSN
715:2016
704:PMID
674:2018
649:2016
621:2016
596:2016
568:2016
540:2016
444:test
382:CD40
201:and
194:CD40
1943:ENT
1861:and
1309:ICD
1294:ICD
1184:PMC
1168:doi
1066:doi
1025:PMC
1009:doi
1005:149
916:doi
912:116
874:PMC
858:doi
816:PMC
798:doi
748:doi
744:198
426:MRI
371:IgE
367:IgG
363:IgA
228:UNG
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Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
