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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...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BMJ Publishing Group
2022
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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’. |
format | Online Article Text |
id | pubmed-9577887 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BMJ Publishing Group |
record_format | MEDLINE/PubMed |
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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