464:, usually accompanied by intense vomiting, weakness, and in some cases, watery diarrhea, and an unraised, nonitchy splotchy/swirly rash. These stomach attacks can last one to five days on average and can require hospitalization for aggressive pain management and hydration. Abdominal attacks have also been known to cause a significant increase in the patient's white blood cell count, usually in the vicinity of 13,000 to 30,000. As the symptoms begin to diminish, the white count slowly begins to decrease, returning to normal when the attack subsides. As the symptoms and diagnostic tests are almost indistinguishable from an
675:, a characteristic that distinguishes it from allergic reactions. It is particularly difficult to diagnose HAE in patients whose episodes are confined to the gastrointestinal tract. Besides a family history of the disease, only a laboratory analysis can provide final confirmation. In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The former is used during the reaction cascade in the complement system of immune defense, which is permanently overactive due to the lack of regulation by C1-INH.
806:(HAE), specific stimuli that have previously led to attacks may need to be avoided in the future. It does not respond to antihistamines, corticosteroids, or epinephrine. Acute treatment consists of C1-INH (C1-esterase inhibitor) concentrate from donor blood, which must be administered intravenously. In an emergency, fresh frozen blood plasma, which also contains C1-INH, can also be used. However, in most European countries, C1-INH concentrate is only available to patients who are participating in special programmes. The medications
3206:
3254:
3242:
331:
3230:
745:. The pathogenesis of this disorder is suspected to be related to unopposed activation of the contact pathway by the initial generation of kallikrein and/or clotting factor XII by damaged endothelial cells. The end product of this cascade, bradykinin, is produced in large amounts and is believed to be the predominant mediator leading to increased vascular permeability and vasodilation that induces typical angioedema "attacks".
392:
40:
3218:
484:
edema will occur is impossible. Most patients have an average of one episode per month, but there are also patients who have weekly episodes or only one or two episodes per year. The triggers can vary and include infections, minor injuries, mechanical irritation, operations or stress. In most cases, edema develops over a period of 12–36 hours and then subsides within 2–5 days.
400:
483:
HAE may also cause swelling in a variety of other locations, most commonly the limbs, genitals, neck, throat and face. The pain associated with these swellings varies from mildly uncomfortable to agonizing pain, depending on its location and severity. Predicting where and when the next episode of
602:, can increase the probability of an angioedema episode in susceptible patients. If the episode occurs at all after the consumption of these foods, its onset may be delayed overnight or by some hours, making the correlation with their consumption somewhat difficult. In contrast, consumption of
762:
is a commonly prescribed antihistamine for angioedema. Some patients have reported success with the combination of a nightly low dose of cetirizine to moderate the frequency and severity of attacks, followed by a much higher dose when an attack does appear. Severe angioedema cases may require
698:. It is characterized by repetitive episodes of swelling, frequently of the face, lips, tongue, limbs, and genitals. Edema of the gastrointestinal mucosa typically leads to severe abdominal pain; in the upper respiratory tract, it can be life-threatening.
666:
The hereditary form (HAE) often goes undetected for a long time, as its symptoms resemble those of more common disorders, such as allergy or intestinal colic. An important clue is the failure of hereditary angioedema to respond to
534:(serpin) normally inhibits the association of C1r and C1s with C1q to prevent the formation of the C1-complex, which - in turn - activates other proteins of the complement system. Additionally, it inhibits various proteins of the
460:, including dental work and other stimuli, can cause attacks. There is usually no associated itch or urticaria, as it is not an allergic response. Patients with HAE can also have recurrent episodes (often called "attacks") of
3467:
3448:
1785:
794:(ARB), which has a similar mechanism but does not affect bradykinin. However, this is controversial, as small studies have shown some patients with ACE inhibitor angioedema can develop it with ARBs, as well.
2723:
1699:
Malde B, Regalado J, Greenberger PA (January 2007). "Investigation of angioedema associated with the use of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers".
653:
levels may be elevated if the attack was due to an acute allergic (anaphylactic) reaction. When the patient has been stabilized, particular investigations may clarify the exact cause;
2159:
1525:
Israili ZH, Hall WD (August 1, 1992). "Cough and angioneurotic edema associated with angiotensin-converting enzyme inhibitor therapy. A review of the literature and pathophysiology".
2123:
503:
and increases vascular permeability, leading to rapid accumulation of fluid in the interstitium. This is most obvious in the face, where the skin has relatively little supporting
1777:
2584:
1613:
Brown NJ, Ray WA, Snowden M, Griffin MR (July 1996). "Black
Americans have an increased rate of angiotensin converting enzyme inhibitor-associated angioedema".
2152:
1508:
3292:
1137:
Bork K; Barnstedt Se (August 2003). "Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema".
2145:
2073:
Donaldson VH, Evans RR (July 1963). "A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C' 1-esterase".
1656:
Dykewicz, MS (August 2004). "Cough and angioedema from angiotensin-converting enzyme inhibitors: new insights into mechanisms and management".
2577:
1823:
1917:
1123:
1089:
790:. In people with ACE inhibitor angioedema, the drug needs to be discontinued and an alternative treatment needs to be found, such as an
786:
so it can no longer degrade bradykinin; thus, bradykinin accumulates and can cause angioedema. This complication appears more common in
2377:
2225:
1854:
1321:
2414:
1386:
834:
may also be useful because it blocks the activation of C4 and can be used in patients with liver disease, whereas androgens cannot.
2570:
267:
721:
protein (type I HAE) or dysfunctional forms of the same protein (type II HAE). Type III HAE has been linked with mutations in the
507:, and edema develops easily. Bradykinin is released by various cell types in response to numerous different stimuli; it is also a
3285:
411:, swell over the period of minutes to hours. The swelling can also occur elsewhere, typically in the hands. The swelling can be
2541:
818:
per dose in the United States, prices that tripled in two years. In those given icatibant, specialists monitor is recommended.
920:
first described the clinical picture of angioedema in 1882, though there had been some earlier descriptions of the condition.
2743:
2243:
767:
therapy, which generally leads to a good response. In cases where allergic attack is progressing towards airway obstruction,
526:
due to a deficiency in one of its prime inhibitors, C1-esterase (aka: C1-inhibitor or C1INH), and continuous production of
2128:
1736:"Angioedema associated with angiotensin-converting enzyme inhibitor use: outcome after switching to a different treatment"
1448:
3593:
3486:
3060:
2748:
2546:
514:
Various mechanisms that interfere with bradykinin production or degradation can lead to angioedema. ACE inhibitors block
1960:
3278:
2853:
1117:
791:
663:. HAE type III is a diagnosis of exclusion consisting of observed angioedema along with normal C1 levels and function.
3608:
1083:
378:
926:
remarked in 1888 that some cases may have a hereditary basis; he coined the term "hereditary angio-neurotic edema".
415:
or painful. There may also be slightly decreased sensation in the affected areas due to compression of the nerves.
360:
2846:
2481:
2398:
2309:
1221:
Bork K, Barnstedt SE, Koch P, Traupe H (2000). "Hereditary angioedema with normal C1-inhibitor activity in women".
263:
713:
form. They are distinguished by the underlying genetic abnormality. Types I and II are caused by mutations in the
1870:"Metallopeptidase activities in hereditary angioedema: effect of androgen prophylaxis on plasma aminopeptidase P"
3603:
2551:
2421:
2293:
356:
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2137:
1740:
1576:
783:
515:
3598:
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862:
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The skin of the face, normally around the mouth, and the mucosa of the mouth and/or throat, as well as the
271:
2718:
2401:
2253:
2220:
1527:
873:, to prevent attacks of HAE types I and II in people over age 12. Lanadelumab inhibits the plasma enzyme
352:
1473:
Sabroe RA, Black AK (February 1997). "Angiotensin-converting enzyme (ACE) inhibitors and angio-oedema".
758:
In allergic angioedema, avoidance of the allergen and use of antihistamines may prevent future attacks.
3184:
826:
In acquired angioedema, HAE types I and II, and nonhistaminergic angioedema, antifibrinolytics such as
3222:
2955:
2863:
2768:
2758:
2661:
2387:
2367:
2282:
2277:
2272:
2267:
2262:
2197:
1266:"Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III"
686:
Acquired angioedema (AAE) can be immunologic, nonimmunologic, or idiopathic. It is usually caused by
531:
1018:"Angioedema in the emergency department: a practical guide to differential diagnosis and management"
3503:
3137:
2753:
2738:
2668:
2646:
2436:
2184:
341:
3613:
3065:
2656:
2536:
2521:
2517:
945:
345:
3152:
3132:
2841:
2698:
1830:
568:
121:
57:
3543:
1799:
1107:
1073:
3107:
3075:
3000:
2829:
2819:
2511:
2491:
2212:
1502:
803:
706:
519:
302:
180:
2450:
3234:
3102:
2531:
2235:
1317:
950:
898:
710:
709:(HAE) exists in three forms, all of which are caused by a genetic mutation inherited in an
634:
317:
may be used instead. In the United States the disease affects about 100,000 people a year.
1952:"Emergency Medicine, Allergy Physicians Partner to Create New Standards" (July 3, 2014).
8:
3471:
3246:
3189:
3097:
3050:
2924:
2763:
1868:
Drouet C, DĂ©sormeaux A, Robillard J, Ponard D, Bouillet L, Martin L, et al. (2008).
1378:
870:
584:
431:
314:
220:
192:
763:
desensitization to the putative allergen, as mortality can occur. Chronic cases require
3310:
3159:
3112:
3021:
2383:
2258:
2047:
1894:
1869:
1681:
1669:
1638:
1552:
1486:
1290:
1265:
1246:
1203:
1044:
1017:
858:; kinins (especially bradykinin) are responsible for the manifestations of angioedema.
851:
657:
levels, especially depletion of complement factors 2 and 4, may indicate deficiency of
625:
typically has a smaller, but still present, increase in the probability of an episode.
595:
435:
134:
68:
61:
3480:
3476:
1712:
1626:
1234:
936:
was diagnosed with angioedema in 2020 and later passed from the disease on
October 31
814:
may be used to treat attacks. In 2017 these medications cost between 5,700 and 14,000
511:
mediator. Dampening or inhibiting bradykinin has been shown to relieve HAE symptoms.
426:
of the airway occurs, with gasping or wheezy inspiratory breath sounds and decreasing
3514:
3253:
3210:
3142:
3122:
3016:
2858:
2789:
2352:
2343:
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1190:
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1113:
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902:
730:
654:
523:
504:
456:
In hereditary angioedema (HAE), often no direct cause is identifiable, although mild
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148:
111:
52:
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Kostis JB, Kim HJ, Rusnak J, Casale T, Kaplan A, Corren J, Levy E (July 25, 2005).
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1230:
1185:
1146:
1039:
1029:
917:
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2562:
854:. These agents increase the level of aminopeptidase P, an enzyme that inactivates
3508:
3356:
3258:
3085:
2991:
2940:
2798:
2473:
2118:
1964:
1590:
1571:
906:
827:
734:
642:
457:
224:
1778:"Study: Drug Costs for Rare Hereditary Angioedema Disorder Tripled in Two Years"
1540:
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3519:
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2914:
2733:
2728:
2594:
2526:
2426:
2248:
2020:
J. L. Milton. On giant urticaria. Edinburgh
Medical Journal, 1876, 22: 513-526.
2000:
1885:
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1735:
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779:
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672:
668:
461:
294:
168:
47:
Allergic angioedema: this child is unable to open his eyes due to the swelling.
3560:
3557:
3461:
3457:
2167:
1995:
1034:
563:
dominant fashion and therefore mainly affects women; it can be exacerbated by
3587:
3302:
3169:
2824:
2724:
Eosinophilic, polymorphic, and pruritic eruption associated with radiotherapy
2113:
1935:
1016:
Bernstein, JA; Cremonesi, P; Hoffmann, TK; Hollingsworth, J (December 2017).
923:
889:
precursors and is produced in excess in individuals with HAE types I and II.
695:
622:
465:
290:
164:
3270:
3412:
3333:
2919:
2834:
2693:
2683:
2625:
2620:
2502:
2314:
2094:
2061:
2011:
Marcello Donati. De medica historia mirabili. Mantuae, per Fr. Osanam, 1586
1903:
1763:
1720:
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1599:
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1363:
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1242:
1199:
1158:
1053:
742:
718:
659:
646:
469:
1634:
1548:
1494:
1421:
694:. It can also occur as a side effect to certain medications, particularly
3538:
3402:
3323:
3318:
2976:
2950:
2945:
2651:
2641:
1957:
1445:"A 68-Year-Old Woman With Recurrent Abdominal Pain, Nausea, and Vomiting"
1408:
Zuraw B.L. (September 2008). "Clinical practice. Hereditary angioedema".
866:
847:
831:
807:
768:
638:
535:
500:
477:
399:
306:
298:
255:
184:
172:
126:
101:
3440:
621:
may increase the probability of an episode in some patients. The use of
3566:
3417:
3392:
3368:
3174:
3127:
3055:
2898:
2868:
2814:
2673:
2468:
1572:"Incidence and characteristics of angioedema associated with enalapril"
1015:
874:
842:
Future attacks of HAE can be prevented by the use of androgens such as
759:
726:
572:
543:
539:
527:
492:
473:
450:
282:
247:
155:
144:
93:
1355:
733:, and all forms can cause swelling elsewhere in the body, such as the
571:(exact frequency uncertain). It has been linked with mutations in the
3549:
3179:
3147:
2678:
1734:
Cicardi M, Zingale LC, Bergamaschini L, Agostoni A (April 26, 2004).
886:
811:
691:
614:
603:
564:
416:
310:
243:
188:
89:
1918:"FDA Approves Takhzyro (lanadelumab-flyo) for Hereditary Angioedema"
1172:
Bas M, Adams V, Suvorava T, Niehues T, Hoffmann TK, Kojda G (2007).
633:
The diagnosis is made on the clinical picture. Routine blood tests (
330:
3346:
3028:
2793:
2458:
2193:
1733:
1281:
956:
929:
The link with C1 esterase inhibitor deficiency was proved in 1963.
882:
650:
607:
599:
588:
580:
560:
442:
259:
538:
cascade, although effects of its deficiency on the development of
495:
plays a critical role in all forms of hereditary angioedema. This
3407:
3387:
2986:
2773:
933:
843:
687:
618:
587:. This acquired angioedema is associated with the development of
522:, bradykinin formation is caused by continuous activation of the
496:
423:
391:
130:
39:
3497:
3494:
3491:
1342:
Axelrod, S; Davis-Lorton, M (2011). "Urticaria and angioedema".
3452:
2333:
2202:
2132:
1867:
1804:
Australasian
Society of Clinical Immunology and Allergy (ASCIA)
855:
738:
594:
Consumption of foods that are themselves vasodilators, such as
556:
Type II - normal levels, but decreased function of C1INH (15%);
518:, the enzyme that among other actions, degrades bradykinin. In
446:
427:
408:
278:
228:
216:
1936:"FDA OKs New Prophylactic Drug for Rare Hereditary Angioedema"
3341:
901:(ED) visits for angioedema annually, and it ranks as the top
878:
232:
212:
559:
Type III - no detectable abnormality in C1INH, occurs in an
2615:
678:
Angioedema is classified as either hereditary or acquired.
508:
480:(keyhole surgery) that turns out to have been unnecessary.
412:
236:
453:(unknown) or only weakly correlated to allergen exposure.
472:) it is possible for undiagnosed HAE patients to undergo
403:
Angioedema of the face, most strikingly in the upper lip.
231:, abdomen, or arms and legs. Often it is associated with
1976:
Quincke H (1882). "Über akutes umschriebenes Hautödem".
1698:
258:. The version related to bradykinin may occur due to an
1220:
1171:
782:
can induce angioedema. ACE inhibitors block the enzyme
1612:
1341:
729:. All forms of HAE lead to abnormal activation of the
717:
gene, which result in either diminished levels of the
1569:
1112:(5 ed.). Elsevier Health Sciences. p. 182.
690:
and occurs together with other allergic symptoms and
3430:
2030:
Osler W (1888). "Hereditary angio-neurotic oedema".
1264:
Cichon S, Martin L, Hennies HC, et al. (2006).
953:(unexplained angioedema with high eosinophil counts)
897:
In the U.S., there are as many as 80,000 to 112,000
2592:
1263:
289:. Histamine-related angioedema can be treated with
1658:Current Opinion in Allergy and Clinical Immunology
1136:
1078:. Lippincott Williams & Wilkins. p. 133.
1507:: CS1 maint: DOI inactive as of September 2024 (
33:Angiooedema, Quincke's edema, angioneurotic edema
3585:
250:. The version related to histamine is due to an
2072:
1874:The Journal of Allergy and Clinical Immunology
549:The three types of hereditary angioedema are:
441:Sometimes, the cause is recent exposure to an
227:. The swelling may occur in the face, tongue,
3300:
3286:
2578:
2153:
1344:The Mount Sinai Journal of Medicine, New York
1071:
301:. In those with bradykinin-related disease a
1174:"Nonallergic angioedema: role of bradykinin"
725:gene, which encodes the coagulation protein
242:The underlying mechanism typically involves
239:. Onset is typically over minutes to hours.
1524:
1472:
1072:Caterino, Jeffrey M.; Kahan, Scott (2003).
1022:International Journal of Emergency Medicine
1011:
1009:
1007:
1005:
1003:
1001:
999:
997:
995:
993:
530:, another process inhibited by C1INH. This
434:is required in these situations to prevent
359:. Unsourced material may be challenged and
3293:
3279:
3229:
2585:
2571:
2378:Purine nucleoside phosphorylase deficiency
2226:Transient hypogammaglobulinemia of infancy
2160:
2146:
1701:Annals of Allergy, Asthma & Immunology
1520:
1518:
1407:
1335:
991:
989:
987:
985:
983:
981:
979:
977:
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254:to agents such as insect bites, foods, or
38:
1975:
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1468:
1466:
1289:
1189:
1101:
1099:
1067:
1065:
1063:
1043:
1033:
830:or ε-aminocaproic acid may be effective.
553:Type I - decreased levels of C1INH (85%);
379:Learn how and when to remove this message
2122:) is being considered for deletion. See
1655:
701:
610:may be beneficial in reducing symptoms.
398:
390:
268:angiotensin-converting enzyme inhibitors
2029:
1515:
1376:
1312:University of Maryland Medical Center.
970:
16:Disease characterized by rapid swelling
3586:
2542:Terminal complement pathway deficiency
1958:http://healthnews.uc.edu/news/?/24791/
1853:: CS1 maint: archived copy as title (
1463:
1096:
1060:
681:
235:, which are swelling within the upper
3274:
2744:Radiation-induced erythema multiforme
2566:
2141:
1389:from the original on 5 September 2015
1105:
320:
3217:
1451:from the original on 22 October 2012
1377:Moon, Amanda T.; Heymann, Warren R.
583:formation against C1INH; this is an
419:(hives) may develop simultaneously.
357:adding citations to reliable sources
324:
3061:Pseudoverrucous papules and nodules
2749:Radiation-induced hypertrophic scar
2547:Paroxysmal nocturnal hemoglobinuria
13:
1670:10.1097/01.all.0000136759.43571.7f
1487:10.1111/j.1365-2133.1997.tb14887.x
487:
14:
3625:
2126:to help reach a consensus. ›
2105:
774:
3252:
3240:
3228:
3216:
3205:
3204:
2847:Inner ear decompression sickness
2310:Common variable immunodeficiency
2044:10.1097/00000441-188804000-00004
1788:from the original on 2015-10-25.
1442:
1191:10.1111/j.1398-9995.2007.01427.x
1126:from the original on 2017-09-10.
1092:from the original on 2017-09-10.
741:, it can cause life-threatening
395:Angioedema of half of the tongue
329:
264:C1 esterase inhibitor deficiency
3241:
2482:Idiopathic CD4+ lymphocytopenia
2066:
2023:
2014:
2005:
1988:
1969:
1946:
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1324:from the original on 2007-10-12
1151:10.14219/jada.archive.2003.0323
892:
792:angiotensin II receptor blocker
476:(operations on the abdomen) or
449:), but more often it is either
2552:Complement receptor deficiency
2422:Adenosine deaminase deficiency
1956:. University of Cincinnati.
1615:Clinical Pharmacologic Therapy
1475:British Journal of Dermatology
1306:
1257:
1214:
1165:
1130:
837:
1:
3081:environmental skin conditions
2874:High-altitude pulmonary edema
2111:
1741:Archives of Internal Medicine
1713:10.1016/S1081-1206(10)60860-5
1627:10.1016/S0009-9236(96)90161-7
1577:Archives of Internal Medicine
1318:"Page is No Longer Available"
1235:10.1016/S0140-6736(00)02483-1
1075:In a Page: Emergency medicine
963:
797:
748:
3071:Tropical anhidrotic asthenia
2880:High-altitude cerebral edema
2087:10.1016/0002-9343(63)90162-1
1591:10.1001/archinte.165.14.1637
863:Food and Drug Administration
628:
272:lymphoproliferative disorder
7:
3093:frictional/traumatic/sports
2719:Chronic radiation keratosis
2221:X-linked agammaglobulinemia
1541:10.7326/0003-4819-117-3-234
1528:Annals of Internal Medicine
1109:Clinical Dermatology E-Book
939:
821:
753:
649:) are typically performed.
10:
3630:
3594:Effects of external causes
3384:Cause of fluid collection
3185:Pseudoacanthosis nigricans
1886:10.1016/j.jaci.2007.10.048
1755:10.1001/archinte.164.8.910
912:
221:tissue just under the skin
3529:
3434:
3377:
3332:
3309:
3200:
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3009:
2964:
2956:Space adaptation syndrome
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2891:
2864:Chronic mountain sickness
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2782:
2769:Radiation recall reaction
2759:Radiation-induced morphea
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2662:Warm water immersion foot
2634:
2608:
2601:
2490:
2467:
2449:
2397:
2327:
2302:
2234:
2211:
2192:
2183:
1954:UC Academic Health Center
1106:Habif, Thomas P. (2009).
1035:10.1186/s12245-017-0141-z
579:Angioedema can be due to
532:serine protease inhibitor
277:Treatment to protect the
198:
154:
140:
120:
110:
100:
85:
77:
67:
51:
46:
37:
29:
24:
3609:Urticaria and angioedema
3138:Weathering nodule of ear
2754:Radiation-induced keloid
2739:Radiation-induced cancer
2669:Non-freezing cold injury
2647:Immersion foot syndromes
2437:Bare lymphocyte syndrome
2289:Wiskott–Aldrich syndrome
2124:templates for discussion
211:is an area of swelling (
3066:Sclerosing lymphangitis
2657:Tropical immersion foot
2537:Complement 3 deficiency
2522:Complement 4 deficiency
2518:Complement 2 deficiency
1489:(inactive 2024-09-14).
946:Drug-induced angioedema
932:The underground rapper
266:, medications known as
3153:Painful fat herniation
3133:Vibration white finger
2842:Decompression sickness
2699:Erythrocyanosis crurum
877:, which liberates the
737:. If HAE involves the
569:hormonal contraception
546:appear to be limited.
404:
396:
202:~100,000 per year (US)
122:Differential diagnosis
58:Allergy and immunology
3604:Complement deficiency
3108:Fissure of the nipple
3076:UV-sensitive syndrome
3001:Adverse drug reaction
2830:Middle ear barotrauma
2820:Arterial gas embolism
2512:Hereditary angioedema
2368:Ataxia–telangiectasia
2213:Hypogammaglobulinemia
1422:10.1056/NEJMcp0803977
1379:"Acquired Angioedema"
804:hereditary angioedema
707:Hereditary angioedema
702:Hereditary angioedema
520:hereditary angioedema
402:
394:
303:C1 esterase inhibitor
181:C1 esterase inhibitor
3103:Equestrian perniosis
2532:Properdin deficiency
2353:Di George's syndrome
2236:Dysgammaglobulinemia
899:emergency department
771:may be life-saving.
635:complete blood count
606:in combination with
353:improve this section
3599:Medical emergencies
3190:Narcotic dermopathy
3098:Black heel and palm
3051:Dermatosis neglecta
2925:Psychological abuse
2764:Radiation poisoning
871:monoclonal antibody
682:Acquired angioedema
596:alcoholic beverages
585:autoimmune disorder
438:and risk of death.
432:Tracheal intubation
315:Fresh frozen plasma
217:lower layer of skin
193:fresh frozen plasma
3530:External resources
3311:Volume contraction
3160:Uranium dermatosis
3113:Pulling boat hands
3022:Lightning injuries
2384:Hyper IgM syndrome
2294:Hyper-IgE syndrome
2259:Hyper IgM syndrome
2175:disorders causing
1978:Monatsh Prakt Derm
1963:2014-07-14 at the
861:In 2018, the U.S.
852:methyltestosterone
711:autosomal dominant
651:Mast cell tryptase
436:respiratory arrest
405:
397:
321:Signs and symptoms
135:contact dermatitis
62:emergency medicine
3581:
3580:
3428:
3427:
3268:
3267:
3017:Electrical injury
2859:Altitude sickness
2707:
2706:
2560:
2559:
2445:
2444:
2344:thymic hypoplasia
2329:T cell deficiency
2323:
2322:
1784:(Press release).
1356:10.1002/msj.20288
1270:Am. J. Hum. Genet
951:Gleich's syndrome
903:allergic disorder
788:African-Americans
731:complement system
524:complement system
505:connective tissue
468:(e.g. perforated
422:In severe cases,
389:
388:
381:
287:cricothyroidotomy
262:problem known as
252:allergic reaction
206:
205:
149:cricothyroidotomy
116:Based on symptoms
112:Diagnostic method
19:Medical condition
3621:
3432:
3431:
3295:
3288:
3281:
3272:
3271:
3256:
3244:
3243:
3232:
3231:
3220:
3219:
3208:
3207:
3044:physical factors
3040:skin conditions
2972:Hypersensitivity
2689:Cold intolerance
2606:
2605:
2593:Consequences of
2587:
2580:
2573:
2564:
2563:
2432:ZAP70 deficiency
2363:Nezelof syndrome
2209:
2208:
2190:
2189:
2177:immunodeficiency
2162:
2155:
2148:
2139:
2138:
2099:
2098:
2070:
2064:
2055:
2027:
2021:
2018:
2012:
2009:
2003:
1992:
1986:
1985:
1973:
1967:
1950:
1944:
1943:
1932:
1926:
1925:
1914:
1908:
1907:
1897:
1865:
1859:
1858:
1852:
1844:
1842:
1841:
1835:
1829:. Archived from
1828:
1820:
1814:
1813:
1811:
1810:
1796:
1790:
1789:
1774:
1768:
1767:
1757:
1731:
1725:
1724:
1696:
1690:
1689:
1653:
1647:
1646:
1610:
1604:
1603:
1593:
1567:
1561:
1560:
1522:
1513:
1512:
1506:
1498:
1470:
1461:
1460:
1458:
1456:
1440:
1434:
1433:
1405:
1399:
1398:
1396:
1394:
1374:
1368:
1367:
1339:
1333:
1332:
1330:
1329:
1310:
1304:
1303:
1293:
1261:
1255:
1254:
1218:
1212:
1211:
1193:
1169:
1163:
1162:
1134:
1128:
1127:
1103:
1094:
1093:
1069:
1058:
1057:
1047:
1037:
1013:
918:Heinrich Quincke
869:, an injectable
817:
384:
377:
373:
370:
364:
333:
325:
225:mucous membranes
81:Minutes to hours
73:Area of swelling
42:
22:
21:
3629:
3628:
3624:
3623:
3622:
3620:
3619:
3618:
3584:
3583:
3582:
3577:
3576:
3525:
3524:
3443:
3429:
3424:
3373:
3357:pulmonary edema
3328:
3305:
3299:
3269:
3264:
3196:
3086:Electrical burn
3043:
3042:resulting from
3041:
3039:
3033:
3005:
2992:Arthus reaction
2960:
2941:Motion sickness
2929:
2903:
2887:
2803:
2799:Oxygen toxicity
2778:
2703:
2630:
2597:
2595:external causes
2591:
2561:
2556:
2493:
2486:
2474:Lymphocytopenia
2472:
2463:
2441:
2417:
2404:
2393:
2349:hypoparathyroid
2331:
2319:
2298:
2230:
2200:
2179:
2166:
2127:
2108:
2103:
2102:
2071:
2067:
2028:
2024:
2019:
2015:
2010:
2006:
1993:
1989:
1974:
1970:
1965:Wayback Machine
1951:
1947:
1934:
1933:
1929:
1916:
1915:
1911:
1866:
1862:
1846:
1845:
1839:
1837:
1833:
1826:
1824:"Archived copy"
1822:
1821:
1817:
1808:
1806:
1798:
1797:
1793:
1776:
1775:
1771:
1732:
1728:
1697:
1693:
1654:
1650:
1611:
1607:
1584:(14): 1637–42.
1568:
1564:
1523:
1516:
1500:
1499:
1471:
1464:
1454:
1452:
1441:
1437:
1416:(10): 1027–36.
1410:N. Engl. J. Med
1406:
1402:
1392:
1390:
1375:
1371:
1340:
1336:
1327:
1325:
1316:
1311:
1307:
1276:(6): 1098–104.
1262:
1258:
1229:(9225): 213–7.
1219:
1215:
1170:
1166:
1139:J Am Dent Assoc
1135:
1131:
1120:
1104:
1097:
1086:
1070:
1061:
1014:
971:
966:
942:
915:
907:hospitalization
895:
881:bradykinin and
840:
828:tranexamic acid
824:
815:
800:
777:
756:
751:
735:digestive tract
704:
684:
643:kidney function
631:
490:
488:Pathophysiology
385:
374:
368:
365:
350:
334:
323:
295:corticosteroids
175:
169:corticosteroids
20:
17:
12:
11:
5:
3627:
3617:
3616:
3614:Drug eruptions
3611:
3606:
3601:
3596:
3579:
3578:
3575:
3574:
3563:
3546:
3534:
3533:
3531:
3527:
3526:
3523:
3522:
3511:
3500:
3483:
3464:
3444:
3439:
3438:
3436:
3435:Classification
3426:
3425:
3423:
3422:
3421:
3420:
3415:
3410:
3405:
3397:
3396:
3395:
3390:
3381:
3379:
3375:
3374:
3372:
3371:
3366:
3361:
3360:
3359:
3354:
3352:cerebral edema
3344:
3338:
3336:
3330:
3329:
3327:
3326:
3321:
3315:
3313:
3307:
3306:
3298:
3297:
3290:
3283:
3275:
3266:
3265:
3263:
3262:
3250:
3238:
3226:
3214:
3201:
3198:
3197:
3195:
3194:
3193:
3192:
3187:
3182:
3177:
3172:
3162:
3157:
3156:
3155:
3150:
3145:
3143:Wrestler's ear
3140:
3135:
3130:
3125:
3123:Surfer's knots
3120:
3115:
3110:
3105:
3100:
3090:
3089:
3088:
3078:
3073:
3068:
3063:
3058:
3053:
3047:
3045:
3035:
3034:
3032:
3031:
3026:
3025:
3024:
3013:
3011:
3007:
3006:
3004:
3003:
2997:
2996:
2995:
2994:
2989:
2984:
2979:
2968:
2966:
2965:Adverse effect
2962:
2961:
2959:
2958:
2953:
2948:
2943:
2937:
2935:
2931:
2930:
2928:
2927:
2922:
2917:
2915:Physical abuse
2911:
2909:
2905:
2904:
2902:
2901:
2895:
2893:
2889:
2888:
2886:
2885:
2884:
2883:
2877:
2871:
2866:
2861:
2851:
2850:
2849:
2839:
2838:
2837:
2832:
2827:
2822:
2811:
2809:
2805:
2804:
2802:
2801:
2796:
2786:
2784:
2780:
2779:
2777:
2776:
2771:
2766:
2761:
2756:
2751:
2746:
2741:
2736:
2734:Radiation burn
2731:
2729:Radiation acne
2726:
2721:
2715:
2713:
2709:
2708:
2705:
2704:
2702:
2701:
2696:
2691:
2686:
2681:
2676:
2671:
2666:
2665:
2664:
2659:
2654:
2644:
2638:
2636:
2632:
2631:
2629:
2628:
2623:
2618:
2612:
2610:
2603:
2599:
2598:
2590:
2589:
2582:
2575:
2567:
2558:
2557:
2555:
2554:
2549:
2544:
2539:
2534:
2529:
2527:MBL deficiency
2524:
2515:
2498:
2496:
2488:
2487:
2485:
2484:
2478:
2476:
2465:
2464:
2462:
2461:
2455:
2453:
2447:
2446:
2443:
2442:
2440:
2439:
2434:
2429:
2427:Omenn syndrome
2424:
2408:
2406:
2395:
2394:
2392:
2391:
2372:
2371:
2365:
2356:
2339:
2337:
2325:
2324:
2321:
2320:
2318:
2317:
2312:
2306:
2304:
2300:
2299:
2297:
2296:
2291:
2286:
2280:
2275:
2270:
2265:
2256:
2254:IgM deficiency
2251:
2249:IgG deficiency
2246:
2244:IgA deficiency
2240:
2238:
2232:
2231:
2229:
2228:
2223:
2217:
2215:
2206:
2187:
2181:
2180:
2165:
2164:
2157:
2150:
2142:
2136:
2135:
2107:
2106:External links
2104:
2101:
2100:
2065:
2022:
2013:
2004:
1987:
1968:
1945:
1927:
1909:
1860:
1815:
1791:
1769:
1726:
1691:
1648:
1605:
1562:
1514:
1462:
1435:
1400:
1369:
1350:(5): 784–802.
1334:
1305:
1282:10.1086/509899
1256:
1213:
1164:
1145:(8): 1088–94.
1129:
1119:978-0323080378
1118:
1095:
1084:
1059:
968:
967:
965:
962:
961:
960:
959:(C1-inhibitor)
954:
948:
941:
938:
914:
911:
894:
891:
839:
836:
823:
820:
799:
796:
780:ACE inhibitors
776:
775:Drug induction
773:
755:
752:
750:
747:
703:
700:
696:ACE inhibitors
683:
680:
669:antihistamines
630:
627:
577:
576:
557:
554:
489:
486:
462:abdominal pain
387:
386:
337:
335:
328:
322:
319:
291:antihistamines
204:
203:
200:
196:
195:
165:antihistamines
158:
152:
151:
142:
138:
137:
124:
118:
117:
114:
108:
107:
106:Family history
104:
98:
97:
87:
83:
82:
79:
75:
74:
71:
65:
64:
55:
49:
48:
44:
43:
35:
34:
31:
27:
26:
18:
15:
9:
6:
4:
3:
2:
3626:
3615:
3612:
3610:
3607:
3605:
3602:
3600:
3597:
3595:
3592:
3591:
3589:
3573:
3569:
3568:
3564:
3562:
3559:
3556:
3552:
3551:
3547:
3545:
3541:
3540:
3536:
3535:
3532:
3528:
3521:
3517:
3516:
3512:
3510:
3506:
3505:
3501:
3499:
3496:
3493:
3489:
3488:
3484:
3482:
3478:
3474:
3473:
3469:
3465:
3463:
3459:
3455:
3454:
3450:
3446:
3445:
3442:
3437:
3433:
3419:
3416:
3414:
3411:
3409:
3406:
3404:
3401:
3400:
3398:
3394:
3391:
3389:
3386:
3385:
3383:
3382:
3380:
3376:
3370:
3367:
3365:
3362:
3358:
3355:
3353:
3350:
3349:
3348:
3345:
3343:
3340:
3339:
3337:
3335:
3331:
3325:
3322:
3320:
3317:
3316:
3314:
3312:
3308:
3304:
3301:Disorders of
3296:
3291:
3289:
3284:
3282:
3277:
3276:
3273:
3261:
3260:
3255:
3251:
3249:
3248:
3239:
3237:
3236:
3227:
3225:
3224:
3215:
3213:
3212:
3203:
3202:
3199:
3191:
3188:
3186:
3183:
3181:
3178:
3176:
3173:
3171:
3170:Skin pop scar
3168:
3167:
3166:
3163:
3161:
3158:
3154:
3151:
3149:
3146:
3144:
3141:
3139:
3136:
3134:
3131:
3129:
3126:
3124:
3121:
3119:
3118:Runner's rump
3116:
3114:
3111:
3109:
3106:
3104:
3101:
3099:
3096:
3095:
3094:
3091:
3087:
3084:
3083:
3082:
3079:
3077:
3074:
3072:
3069:
3067:
3064:
3062:
3059:
3057:
3054:
3052:
3049:
3048:
3046:
3036:
3030:
3027:
3023:
3020:
3019:
3018:
3015:
3014:
3012:
3008:
3002:
2999:
2998:
2993:
2990:
2988:
2985:
2983:
2980:
2978:
2975:
2974:
2973:
2970:
2969:
2967:
2963:
2957:
2954:
2952:
2949:
2947:
2944:
2942:
2939:
2938:
2936:
2932:
2926:
2923:
2921:
2918:
2916:
2913:
2912:
2910:
2906:
2900:
2897:
2896:
2894:
2890:
2881:
2878:
2875:
2872:
2870:
2867:
2865:
2862:
2860:
2857:
2856:
2855:
2854:High altitude
2852:
2848:
2845:
2844:
2843:
2840:
2836:
2833:
2831:
2828:
2826:
2825:Aerosinusitis
2823:
2821:
2818:
2817:
2816:
2813:
2812:
2810:
2806:
2800:
2797:
2795:
2791:
2788:
2787:
2785:
2781:
2775:
2772:
2770:
2767:
2765:
2762:
2760:
2757:
2755:
2752:
2750:
2747:
2745:
2742:
2740:
2737:
2735:
2732:
2730:
2727:
2725:
2722:
2720:
2717:
2716:
2714:
2710:
2700:
2697:
2695:
2692:
2690:
2687:
2685:
2682:
2680:
2677:
2675:
2672:
2670:
2667:
2663:
2660:
2658:
2655:
2653:
2650:
2649:
2648:
2645:
2643:
2640:
2639:
2637:
2633:
2627:
2624:
2622:
2619:
2617:
2614:
2613:
2611:
2607:
2604:
2600:
2596:
2588:
2583:
2581:
2576:
2574:
2569:
2568:
2565:
2553:
2550:
2548:
2545:
2543:
2540:
2538:
2535:
2533:
2530:
2528:
2525:
2523:
2519:
2516:
2513:
2509:
2505:
2504:
2500:
2499:
2497:
2495:
2489:
2483:
2480:
2479:
2477:
2475:
2470:
2466:
2460:
2457:
2456:
2454:
2452:
2448:
2438:
2435:
2433:
2430:
2428:
2425:
2423:
2420:
2416:
2413:
2410:
2409:
2407:
2403:
2400:
2396:
2389:
2385:
2382:
2381:
2380:
2379:
2376:
2369:
2366:
2364:
2360:
2359:euparathyroid
2357:
2354:
2350:
2347:
2345:
2341:
2340:
2338:
2335:
2330:
2326:
2316:
2313:
2311:
2308:
2307:
2305:
2301:
2295:
2292:
2290:
2287:
2284:
2281:
2279:
2276:
2274:
2271:
2269:
2266:
2264:
2260:
2257:
2255:
2252:
2250:
2247:
2245:
2242:
2241:
2239:
2237:
2233:
2227:
2224:
2222:
2219:
2218:
2216:
2214:
2210:
2207:
2204:
2199:
2195:
2191:
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2045:
2041:
2038:(2): 362–67.
2037:
2033:
2026:
2017:
2008:
2002:
2001:Who Named It?
1998:
1997:
1991:
1983:
1979:
1972:
1966:
1962:
1959:
1955:
1949:
1941:
1937:
1931:
1923:
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1913:
1905:
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1896:
1891:
1887:
1883:
1880:(2): 429–33.
1879:
1875:
1871:
1864:
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1836:on 2007-09-28
1832:
1825:
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1664:(4): 267–70.
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1535:(3): 234–42.
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1224:
1217:
1209:
1205:
1201:
1197:
1192:
1187:
1184:(8): 842–56.
1183:
1179:
1175:
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1160:
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1148:
1144:
1140:
1133:
1125:
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1085:9781405103572
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930:
927:
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924:William Osler
921:
919:
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908:
905:resulting in
904:
900:
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699:
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664:
662:
661:
656:
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648:
647:liver enzymes
644:
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626:
624:
623:acetaminophen
620:
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466:acute abdomen
463:
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410:
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393:
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369:November 2023
362:
358:
354:
348:
347:
343:
338:This section
336:
332:
327:
326:
318:
316:
313:may be used.
312:
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41:
36:
32:
28:
23:
3565:
3548:
3537:
3513:
3502:
3485:
3466:
3447:
3413:hydrosalpinx
3363:
3334:Hypervolemia
3303:volume state
3257:
3245:
3233:
3221:
3209:
3164:
3092:
3080:
2981:
2920:Sexual abuse
2908:Maltreatment
2835:Pneumothorax
2694:Acrocyanosis
2684:Aerosol burn
2626:Heat syncope
2621:Hyperthermia
2507:
2503:C1-inhibitor
2501:
2418:
2411:
2374:
2373:
2358:
2348:
2342:
2315:ICF syndrome
2117:
2078:
2074:
2068:
2035:
2032:Am J Med Sci
2031:
2025:
2016:
2007:
1994:
1990:
1981:
1977:
1971:
1953:
1948:
1939:
1930:
1921:
1912:
1877:
1873:
1863:
1838:. Retrieved
1831:the original
1818:
1807:. Retrieved
1803:
1800:"Angioedema"
1794:
1781:
1772:
1748:(8): 910–3.
1745:
1739:
1729:
1707:(1): 57–63.
1704:
1700:
1694:
1661:
1657:
1651:
1618:
1614:
1608:
1581:
1575:
1565:
1532:
1526:
1503:cite journal
1481:(2): 153–8.
1478:
1474:
1453:. Retrieved
1447:. MedScape.
1443:Loew, Burr.
1438:
1413:
1409:
1403:
1391:. Retrieved
1382:
1372:
1347:
1343:
1337:
1326:. Retrieved
1313:
1308:
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1181:
1177:
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1142:
1138:
1132:
1108:
1074:
1025:
1021:
931:
928:
922:
916:
896:
893:Epidemiology
860:
841:
825:
801:
778:
757:
743:asphyxiation
722:
719:C1-inhibitor
714:
705:
685:
677:
665:
660:C1-inhibitor
658:
639:electrolytes
632:
612:
593:
578:
548:
513:
499:is a potent
491:
482:
470:appendicitis
455:
440:
421:
406:
375:
366:
351:Please help
339:
281:may include
276:
241:
208:
207:
176:
160:
102:Risk factors
3539:MedlinePlus
3403:hydrothorax
3324:Hypovolemia
3319:Dehydration
2977:Anaphylaxis
2951:Airsickness
2946:Seasickness
2652:Trench foot
2642:Hypothermia
2602:Temperature
2375:peripheral:
2112:‹ The
1782:PR Newswire
1621:(1): 8–13.
1314:Angioedema.
885:from their
867:lanadelumab
848:oxandrolone
838:Prophylaxis
832:Cinnarizine
808:ecallantide
769:epinephrine
613:The use of
567:and use of
536:coagulation
501:vasodilator
478:laparoscopy
307:ecallantide
299:epinephrine
256:medications
185:ecallantide
173:epinephrine
127:Anaphylaxis
78:Usual onset
30:Other names
3588:Categories
3572:Angioedema
3567:Patient UK
3515:DiseasesDB
3418:hyperaemia
3393:transudate
3369:Lymphedema
3364:Angioedema
3175:Skin track
3128:Tennis toe
3056:Pinch mark
3038:Ungrouped
2982:Angioedema
2899:Starvation
2869:Death zone
2815:Barotrauma
2674:Chilblains
2508:Angioedema
2494:deficiency
2492:Complement
2469:Leukopenia
2419:autosomal:
2173:complement
2129:Angioedema
2075:Am. J. Med
1984:: 129–131.
1840:2007-01-26
1809:2020-03-24
1455:19 October
1328:2008-01-08
964:References
875:kallikrein
798:Hereditary
760:Cetirizine
749:Management
727:factor XII
655:complement
573:factor XII
544:thrombosis
540:hemorrhage
528:kallikrein
493:Bradykinin
474:laparotomy
451:idiopathic
283:intubation
248:bradykinin
209:Angioedema
177:Bradykinin
156:Medication
145:Intubation
94:bradykinin
92:mediated,
25:Angioedema
3550:eMedicine
3180:Slap mark
3148:Coral cut
2712:Radiation
2679:Frostbite
2412:x-linked:
2081:: 37–44.
2056:Reprint:
1922:Drugs.com
1393:1 October
1028:(1): 15.
887:kininogen
865:approved
812:icatibant
692:urticaria
629:Diagnosis
615:ibuprofen
604:bromelain
565:pregnancy
417:Urticaria
340:does not
311:icatibant
260:inherited
244:histamine
215:) of the
199:Frequency
189:icatibant
161:Histamine
141:Treatment
90:Histamine
53:Specialty
3555:emerg/32
3399:By site
3347:Anasarca
3235:Glossary
3211:Category
3029:Drowning
2808:Pressure
2794:Asphyxia
2609:Elevated
2459:HIV/AIDS
2451:Acquired
2402:combined
2194:Antibody
2169:Lymphoid
2114:template
2095:14046003
2062:20145434
2052:72808776
1996:synd/482
1961:Archived
1940:Medscape
1904:18158172
1849:cite web
1786:Archived
1764:15111379
1721:17225721
1686:13313000
1678:15238791
1643:12030323
1600:16043683
1557:41952350
1449:Archived
1430:18768946
1387:Archived
1383:MedScape
1364:21913206
1322:Archived
1300:17186468
1251:30105665
1243:10963200
1208:22772933
1200:17620062
1159:12956349
1124:Archived
1090:Archived
1054:28405953
957:Ruconest
940:See also
883:kallidin
822:Acquired
754:Allergic
715:SERPING1
673:steroids
608:turmeric
600:cinnamon
589:lymphoma
581:antibody
561:X-linked
443:allergen
430:levels.
96:mediated
69:Symptoms
3561:ped/101
3558:med/135
3509:D000799
3408:ascites
3388:exudate
3247:Outline
3223:Commons
2987:Allergy
2790:Hypoxia
2774:Sunburn
2635:Reduced
2198:humoral
2185:Primary
2116:below (
1895:4126900
1635:8689816
1549:1616218
1495:9068723
1291:1698720
1178:Allergy
1045:5389952
934:MF DOOM
913:History
844:danazol
765:steroid
688:allergy
619:aspirin
497:peptide
447:peanuts
424:stridor
361:removed
346:sources
270:, or a
131:abscess
3544:000846
3498:610618
3495:106100
3492:606860
3259:Portal
3165:iv use
2934:Travel
2882:(HACE)
2876:(HAPE)
2783:Oxygen
2415:X-SCID
2399:Severe
2133:Curlie
2119:Curlie
2093:
2060:
2050:
1902:
1892:
1762:
1719:
1684:
1676:
1641:
1633:
1598:
1555:
1547:
1493:
1428:
1362:
1298:
1288:
1249:
1241:
1223:Lancet
1206:
1198:
1157:
1116:
1082:
1052:
1042:
879:kinins
856:kinins
739:larynx
458:trauma
445:(e.g.
428:oxygen
409:tongue
297:, and
279:airway
229:larynx
3520:13606
3481:995.1
3477:277.6
3462:T78.3
3458:D84.1
3378:Other
3342:Edema
3010:Other
2405:(B+T)
2303:Other
2048:S2CID
1834:(PDF)
1827:(PDF)
1682:S2CID
1639:S2CID
1553:S2CID
1247:S2CID
1204:S2CID
575:gene.
413:itchy
309:, or
233:hives
213:edema
86:Types
3504:MeSH
3487:OMIM
3472:9-CM
2892:Food
2616:Burn
2171:and
2091:PMID
2058:PMID
1900:PMID
1855:link
1760:PMID
1717:PMID
1674:PMID
1631:PMID
1596:PMID
1545:PMID
1509:link
1491:PMID
1457:2012
1426:PMID
1395:2015
1360:PMID
1296:PMID
1239:PMID
1196:PMID
1155:PMID
1114:ISBN
1080:ISBN
1050:PMID
816:US$
810:and
542:and
509:pain
344:any
342:cite
237:skin
219:and
3468:ICD
3449:ICD
2131:at
2083:doi
2040:doi
1999:at
1890:PMC
1882:doi
1878:121
1750:doi
1746:164
1709:doi
1666:doi
1623:doi
1586:doi
1582:165
1537:doi
1533:117
1483:doi
1479:136
1418:doi
1414:359
1352:doi
1286:PMC
1278:doi
1231:doi
1227:356
1186:doi
1147:doi
1143:134
1040:PMC
1030:doi
850:or
802:In
784:ACE
723:F12
671:or
617:or
598:or
516:ACE
355:by
285:or
246:or
223:or
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Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
