Stručni rad
Hereditary angioedema type I in a female patient: a case report
INGRID PRKAČIN
orcid.org/0000-0002-5830-7131
; Clinical Hospital Merkur, Ivana Zajca 19, Zagreb, Croatia
ANA BAN
; Clinical Hospital Merkur, Ivana Zajca 19, Zagreb, Croatia
GORDANA CAVRIĆ
; Intensive Care Unit, Merkur Clinical Hospital, Zagreb, Croatia
DUBRAVKA BARTOLEK HAMP
; Intensive Care Unit, Merkur Clinical Hospital, Zagreb, Croatia
Sažetak
Hereditary angioedema (HAE) is rare autosomal dominant disease, characterised by spontaneous and recurrent swellings
in various parts of the body. The main inflammatory factor in HAE is bradykinin (a key mediator of non-allergic angioedema)
and it is responsible for capillary leak. C1 esterase inhibitor (C1-INH) is a protease inhibitor that blocks the activation of the
classic complement pathway, but there are also many others biochemical pathways, including kinin. Type I HAE is defined
by low plasma levels of a normal C1 inhibitor (C1-INH).
Case presentation. A 34-year-old female patient presented to the hospital complained of swollen and painful legs, flatulence,
palpebral and labial edema, dyspnea, dysuria, frequent herpetic infections of mouth and nose, subfebrile body temperature
and was hoarse. Over the last 20 years she suffered from occasional edemas of the extremities and abdomen. Laboratory
testing showed a reduced level of C1-INH, and with other test results normal we diagnosed the patient with HAE type I. For
emergency situations, we prescribed icatibant (B2 bradykinin receptor antagonist) subcutaneously.
Individuals with HAE report episodic attacks during childhood that become more severe during adolescence. There is nonpitting
edema in 3 predisposed places : subcutaneous tissue, the abdomen and the larynx. Edemas amplify during 12-24
hours and disappear during 3-5 days, with migrations to other locations. Treatment can be prophylactic, for acute attacks
(icatibant) and before medical treatments.
HAE is a potentially lethal disease and should be considered if repeating edema of various body parts, painful swelling and
tightening of the skin are present.
Ključne riječi
hereditary angioedema; bradykinin; B2 bradykinin receptor antagonist
Hrčak ID:
134174
URI
Datum izdavanja:
1.6.2014.
Posjeta: 1.110 *