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Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency

C1-inhibitor deficiency is a rare disease which incorporates acute self-limiting intermittent swelling of the subcutaneous tissue and mucous membranes. Attacks most frequently affect the face and/or the upper airway. Isolated angioedema of the small bowel is an uncommon manifestation and often accom...

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Autores principales: Hanevelt, Julia, de Vos tot Nederveen Cappel, Wouter Hugo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9577887/
https://www.ncbi.nlm.nih.gov/pubmed/36241357
http://dx.doi.org/10.1136/bcr-2022-250710
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author Hanevelt, Julia
de Vos tot Nederveen Cappel, Wouter Hugo
author_facet Hanevelt, Julia
de Vos tot Nederveen Cappel, Wouter Hugo
author_sort Hanevelt, Julia
collection PubMed
description C1-inhibitor deficiency is a rare disease which incorporates acute self-limiting intermittent swelling of the subcutaneous tissue and mucous membranes. Attacks most frequently affect the face and/or the upper airway. Isolated angioedema of the small bowel is an uncommon manifestation and often accompanied by diagnostic delay. In the present case, abdominal pain turned out to be the first and only utterance of an acquired C1-inhibitor deficiency, secondary to a splenic marginal zone lymphoma. Imaging showed wall thickening of the small intestine, ascites and splenomegaly. The abdominal pain and intestinal wall thickening with surrounding ascites on imaging spontaneously resolved each episode within 2–3 days. Gastrointestinal manifestations of angioedema may mimic an acute abdomen, and subsequently one-third of these patients undergo unnecessary surgery prior to a definite diagnosis. This emphasises the importance of considering the diagnosis in case of an ‘extraordinary colic’.
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spelling pubmed-95778872022-10-19 Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency Hanevelt, Julia de Vos tot Nederveen Cappel, Wouter Hugo BMJ Case Rep Case Reports: Rare disease C1-inhibitor deficiency is a rare disease which incorporates acute self-limiting intermittent swelling of the subcutaneous tissue and mucous membranes. Attacks most frequently affect the face and/or the upper airway. Isolated angioedema of the small bowel is an uncommon manifestation and often accompanied by diagnostic delay. In the present case, abdominal pain turned out to be the first and only utterance of an acquired C1-inhibitor deficiency, secondary to a splenic marginal zone lymphoma. Imaging showed wall thickening of the small intestine, ascites and splenomegaly. The abdominal pain and intestinal wall thickening with surrounding ascites on imaging spontaneously resolved each episode within 2–3 days. Gastrointestinal manifestations of angioedema may mimic an acute abdomen, and subsequently one-third of these patients undergo unnecessary surgery prior to a definite diagnosis. This emphasises the importance of considering the diagnosis in case of an ‘extraordinary colic’. BMJ Publishing Group 2022-10-14 /pmc/articles/PMC9577887/ /pubmed/36241357 http://dx.doi.org/10.1136/bcr-2022-250710 Text en © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
spellingShingle Case Reports: Rare disease
Hanevelt, Julia
de Vos tot Nederveen Cappel, Wouter Hugo
Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency
title Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency
title_full Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency
title_fullStr Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency
title_full_unstemmed Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency
title_short Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency
title_sort presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired c1-inhibitor deficiency
topic Case Reports: Rare disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9577887/
https://www.ncbi.nlm.nih.gov/pubmed/36241357
http://dx.doi.org/10.1136/bcr-2022-250710
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