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

Piotr Obtułowicz; Marcin Stobiecki; Wojciech Dyga; Aldona Juchacz; Tadeusz Popiela; Krystyna Obtułowicz

doi:10.5114/ada.2021.108438

eISSN: 2299-0046
ISSN: 1642-395X

Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii

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abstract:

Original paper

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

Piotr Obtułowicz

¹

,

Marcin Stobiecki

^{2, 3}

,

Wojciech Dyga

^{2, 3}

,

Aldona Juchacz

⁴

,

Tadeusz Popiela

^{1, 5}

,

Krystyna Obtułowicz

^{2, 3}

Department of Diagnostic Imaging (NSSU), University Hospital, Krakow, Poland
Department of Clinical and Environmental Allergology, Jagiellonian University Medical College, Krakow, Poland
Hereditary Angioedema Centre, University Hospital, Krakow, Poland
The Greater Poland Centre for Pulmonology and Thoracic Surgery, Poznan, Poland
Department of Radiology, Jagiellonian University Medical College, Krakow, Poland

Adv Dermatol Allergol 2022; XXXIX (4): 749-756

DOI: https://doi.org/10.5114/ada.2021.108438

Online publish date: 2021/08/13

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AMA

Obtułowicz P, Stobiecki M, Dyga W, Juchacz A, Popiela T, Obtułowicz K. Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii. 2022;39(4):749-756. doi:10.5114/ada.2021.108438.

APA

Obtułowicz, P., Stobiecki, M., Dyga, W., Juchacz, A., Popiela, T.,  & Obtułowicz, K. (2022). Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, 39(4), 749-756. https://doi.org/10.5114/ada.2021.108438

Chicago

Obtułowicz, Piotr, Marcin Stobiecki, Wojciech Dyga, Aldona Juchacz, Tadeusz Popiela,  and Krystyna Obtułowicz. 2022. "Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency". Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii 39 (4): 749-756. doi:10.5114/ada.2021.108438.

Harvard

Obtułowicz, P., Stobiecki, M., Dyga, W., Juchacz, A., Popiela, T.,  and Obtułowicz, K. (2022). Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, 39(4), pp.749-756. https://doi.org/10.5114/ada.2021.108438

MLA

Obtułowicz, Piotr et al. "Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency." Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, vol. 39, no. 4, 2022, pp. 749-756. doi:10.5114/ada.2021.108438.

Vancouver

Obtułowicz P, Stobiecki M, Dyga W, Juchacz A, Popiela T, Obtułowicz K. Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii. 2022;39(4):749-756. doi:10.5114/ada.2021.108438.

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 hours 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 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.

keywords:

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

Piotr Obtułowicz 1 , Marcin Stobiecki 2, 3 , Wojciech Dyga 2, 3 , Aldona Juchacz 4 , Tadeusz Popiela 1, 5 , Krystyna Obtułowicz 2, 3

abdominal imaging, bradykinin, C1 inhibitor, hereditary angioedema

Piotr Obtułowicz

¹

,

Marcin Stobiecki

^{2, 3}

,

Wojciech Dyga

^{2, 3}

,

Aldona Juchacz

⁴

,

Tadeusz Popiela

^{1, 5}

,

Krystyna Obtułowicz

^{2, 3}