Database of diseases - Allergy

BURNS GAINED BY C1-ESTERAZY INHIBITOR
 
Is angioedema caused by the body self-limiting?
 
This is possible in rare cases, usually occurring in the family, if the patient has a congenital deficiency of a substance called an esterase C1 inhibitor. The C1 esterase inhibitor inhibits the constituent mechanisms of the so-called complement - an important element of the immune system. Constant, high (80-70%) inhibitor deficiency causes disease symptoms that may already occur in childhood, but most often they are found at the age of puberty.

What symptoms may help to detect the causative agent in the form of C1-esterase inhibitor deficiency?
 
Such symptoms may include: thyroid enlargement, which may indicate an autoimmune process and / or hormonal disorder, enlargement of the lymph nodes of the spleen or liver (suspicious of cancer), abnormalities in the functioning of joints, kidneys, central nervous system, skin or mucous membranes. The diagnostic principle of diagnosis is next to the medical history and visual inspection of the patient - blood counts, ESR, urinalysis, liver biochemistry, etc.


How do we treat sudden attacks of angioneurotic edema, including those caused by a constant deficiency of the C1-esterase inhibitor?

In severe cases, it is necessary to give - oral or intravenous - corticosteroids, especially in rapidly growing laryngeal edema; in addition, adrenaline and antihistamines. Intravenous injection of the highly purified C1 esterase inhibitor is sometimes used to treat edema attacks. The symptoms disappear within a few hours and the risk of laryngeal edema disappears.

First aid, if a violent anaphylactic reaction occurs, must be given immediately

1. Every minute counts - immediately call for medical help, secure a substance suspected of triggering an anaphylactic reaction

2. Do not leave victims without intensive care - because the loss of consciousness may occur suddenly and unexpectedly,

3. The drug of choice is epinephrine (adrenaline), which must be given immediately - people susceptible to the occurrence of anaphylaxis should not part with the first aid sets,
the entire family of the patient should be familiar with the contents and manner of using the kit.



ed. Edward Ozga Michalski, MA
Consultation by prof. dr hab. med. Andrzej Danysz.

What are the symptoms of a constant C1-esterase inhibitor deficiency?
 
They are sudden and rapidly growing edema. They occur most often on the hands and legs. Body tissues become under the influence of a "clumped" edema, i.e. they do not reproduce the trace of finger pressure examining the doctor. Abdominal pain attacks are common without diarrhea. There is a possibility of rapidly growing swelling of the glottis, which may threaten the sufferer from suffocation.


Do we know the reasons for inhibitor deficiency in patients?
 
A distinction is made between hereditary and acquired C1 esterase inhibitor deficiency. In the case of hereditary deficiency, the reason for deficiency is the lack of activity of one of the two genes coding for the production of this substance. The acquired form may be caused by: proliferative diseases of the lymphatic system, prostate cancer, autoimmune defect.


What stimuli trigger a disease attack in people with a constant deficiency of a C1-esterase inhibitor?
 
The reasons for the appearance of violent edema are not fully understood. However, the stimulus causing sudden build-up of symptoms can be injury (bruising with the body) or, for example, the use of an anesthetic during tooth extraction.