HAE Range menu
printCase reports
Case reports on Hereditary angioedema: manifestations, diagnosis or treatment
Symptoms, Course, and Complications of Abdominal Attacks in Hereditary Angioedema due to C1 Inhibitor Deficiency
Bork K, Staubach P, Eckardt MAJ, and Hardt J.
Am J Gastroenterol. 2006 Mar;101(3):619-27.
Abstract
OBJECTIVES: Recurrent abdominal attacks belong to the cardinal and most distressing symptoms of hereditary angioedema (HAE) due to C1 inhibitor deficiency. They are characterized by crampy pain, but may include vomiting, diarrhea, and other features. Detailed clinical data about the symptoms and course of abdominal attacks have not been reported.METHODS: We retrospectively observed a total of 33,671 abdominal attacks in 153 patients with HAE including a prospectively examined subgroup of 23 patients. Symptoms, course, frequency of attacks, and complications were analyzed. RESULTS: The relation of mild, moderate, and severe attacks was 1:1.4:5.6 in the prospective part of the study. Extra-abdominal symptoms preceded the abdominal symptoms. The mean maximal pain score was 8.4 (range 1-10). Vomiting occurred in 73% (24,696) and diarrhea in 41% (13,682) of the attacks. Circulatory collapse accompanied 4.4% (1,468) of the attacks, with loss of consciousness (LOC) occurring in 2.2% (739). Nine patients could clearly distinguish two types of abdominal attacks: vomiting and diarrhea. Rare complications included tetany, hemorrhagic stools, and intussusception of the colon. In 28% (43) of the patients, recurrent abdominal attacks had started before the characteristic swelling of the skin had ever occurred. A model is proposed to classify the severity of the attacks and to describe the clinical course.
CONCLUSIONS: Abdominal attacks in HAE constitute a more disabling and complex syndrome than previously assumed. Our results add to the understanding of symptoms and course of HAE and may aid in the early recognition of an impending attack and improve clinical management.
Full paper via amjgastro (subscribers only)
Novel pharmacotherapy of acute hereditary angioedema with bradykinin B2-receptor antagonist icatibant
Bas M, Bier H, Greve J, Kojda G, Hoffmann TK.
Allergy. 2006 Dec;61(12):1490–1492
Full paper via Blackwell (subscribers only)
Hereditary angioedema involving the colon: endoscopic appearance and review of GI manifestations.
Koruth JS, Eckardt AJ, Levey JM.
Gastrointest Endosc. 2005 Jun;61(7):907-11
Abstract
Hereditary angioedema (HAE) was first described by Sir William Osler in 1888. This rare genetic form of angioneurotic edema is manifested by edema of the skin, the mucous membranes, and the GI tract.Isolated involvement of the colon is very uncommon in HAE. The endoscopic appearance of the colon during an acute attack has not been previously reported. A case of a patient with an acute attack of HAE of the colon is presented, including the first description of its endoscopic appearance. The GI manifestations of HAE are subsequently reviewed.Full paper via Science direct
| Frequently inquired |
Author: Jerini AG
Source: HAE-Network Status: 3/08 This list is frequently updated. Please send us your comments or suggestions by e-mail. |
