Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency.

Introduction: Hereditary angioedema (HAE) is a rare inherited autosomal dominant disease caused by deficiency or dysfunction of C1 inhibitor (C1INH). Clinical symptoms include recurrent subcutaneous and submucosal angioedema of the internal organs. Abdominal attacks affect more than 90% of patients, are often misdiagnosed and result in unnecessary surgical procedures. Aim: To analyse the utility of imaging studies (USG, CT) in patients with C1INH-HAE during an abdominal attack and remission. Material and methods: We enrolled 40 patients with type I and II HAE (30 women, 10 men; mean age 39 years). The diagnosis of C1INH-HAE was based on patient and family history, significantly reduced values of C1INH serum level and activity. Abdominal and pelvic ultrasound were performed in patients within the first 6 h of the abdominal attack and repeated during remission. Moreover, 23 cases underwent abdominal or pelvic computed tomography during acute abdominal symptoms. The most common ultrasound and CT findings showed the transient presence of a significant amount of fluid in the free abdominal cavity and intestinal oedema during the symptom progression and spontaneously disappearing during the seizure in 90% and 50% of patients, respectively. CT revealed also an enlargement of the mesenteric lymph nodes as well as a fat stranding along the bowel wall thickening. Conclusions: Ultrasound or CT imaging facilitates the diagnosis of the patient suspected of having an abdominal attack due to C1INH-HAE. They allow to identify transitional presence of an abundant fluid in the free abdominal cavity and intestinal swelling which spontaneously disappear with a symptoms attack.

[Recurrent abdominal pain and ascites in patients suffering from congenital angioedema due to C1 inhibitor deficiency. Retrospective analysis]. / Nawracajace bóle brzucha i wodobrzusze u pacjentów z wrodzonym obrzekiem naczynioruchowym na tle niedoboru C1 inhibitora. Analiza retrospektywna.

The aim of the study was the estimation of diagnostic value abdominal ultrasonography in recurrent attacks the patients suffering from hereditary angioedema. The retrospective analysis was done in 150 patients with C1 inhibitor deficiency and abdomen attack of angioedema connected with this. In 55 patients severe 2-3 days longlasting abdomen attacks with nausea, diarrhoea and weakening were the first symptoms of the illness and causing particular diagnostic problem. In 95 remaining patients abdomen attack appeared in the course of illness manifesting external angioedema of hands, feets, face, genitalia or larynx. The frequency of abdominal attacks was different from 1-15/year. Diagnostic problem was the reason of laparotomy in 19.3% of patients which did not explain the reason of symptoms. Only in 23 % of patients US of abdomen was done, which revealed the presence of ascites, which disappeared together with abdomen symptoms. US often allowed to show the place of regional angioedema usually in the most cases in intestine wall.

[Isocyanates and formaldehyde--low molecular weight xenobiotics]. / Izocyjaniany i formaldehyd--maloczasteczkowe ksenobiotyki.

Isocyanates and formaldehyde are a low molecular weight environmental xenobiotics. Acute poisonings are relatively rare, chronic exposure not enough recognized. A source, mechanism of toxicity and ability of those low molecular weight compounds to stimulation of different types of immune response are presented in the study.

[Recombinant human C1-inhibitor is effective in the treatment of acute attacks of hereditary angioedema--case report]. / Rekombinowany C1-inhibitor jest skuteczny w leczeniu ostrych napadów wrodzonego obrzeku naczynioruchowego--opis przypadków.

Hereditary angioedema (HAE) is a rare condition, resting on attacks of edema in various localizations, potentially life-threatening if in facial, laryngeal, pharyngeal or gastrointestinal area. The disease is caused by deficiency or impaired activity of C1 inhibitor, therefore C1 inhibitor infusion is the the essential treatment and the only efficient method in an acute attack of HAE. Nowadays C1 inhibitor applied in our patients is obtained from human plasma, what restricts the availability of the drug and carries relevant risks. After many years of research it came to the synthesis of the recombinant protein with features of human C1 inhibitor. Its first usage in Poland took place-in two HAE patients with severe edema in 2004. The course and efficiency of this treatment is reported in the paper. Recombinant human C1 inhibitor occurred efficient and safe in presented case of severe angioedema.

[Exercise-induced asthma and anaphylaxis]. / Astma i anafilaksja indukowana wysilkiem.

The main discussed issues in the study are: epidemiology and the most important conceptions explaining pathomechanism of exercise-induced bronchospasm. Presented recommendations concern physical activities, prophylactic and pharmacological treatment in exercise-induced asthma. The study contains description of diagnostic exercise challenge tests principles and performance procedures. Problem of exercise-induced bronchial asthma related to practice sports is underlined. Reasons and symptoms of exercise-induced anaphylaxis are presented.