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Teduglutide in amyloidosis‐associated intestinal failure

Amyloidosis is a heterogeneous disease characterized by tissue deposition of abnormally folded fibrillary proteins that can manifest itself by a wide variety of symptoms depending on the affected organs. GI involvement among amyloidosis patients is common. Its clinical manifestation often presents w...

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Autores principales: Luhn, Clara, Agis, Hermine, Hütterer, Elisabeth, Simonitsch‐Klupp, Ingrid, Dawoud, Christopher, Stift, Anton, Harpain, Felix
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10433832/
https://www.ncbi.nlm.nih.gov/pubmed/37601424
http://dx.doi.org/10.1002/ccr3.7653
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author Luhn, Clara
Agis, Hermine
Hütterer, Elisabeth
Simonitsch‐Klupp, Ingrid
Dawoud, Christopher
Stift, Anton
Harpain, Felix
author_facet Luhn, Clara
Agis, Hermine
Hütterer, Elisabeth
Simonitsch‐Klupp, Ingrid
Dawoud, Christopher
Stift, Anton
Harpain, Felix
author_sort Luhn, Clara
collection PubMed
description Amyloidosis is a heterogeneous disease characterized by tissue deposition of abnormally folded fibrillary proteins that can manifest itself by a wide variety of symptoms depending on the affected organs. GI involvement among amyloidosis patients is common. Its clinical manifestation often presents with nonspecific symptoms such as weight loss, diarrhea, and malabsorption. With no specific treatment existing for GI amyloidosis, therapy focuses on impeding amyloid deposition and managing the patients' symptoms with supportive measures. Here, we present an AL‐amyloidosis patient with GI involvement and intestinal failure (IF) who was successfully treated with the glucagon‐like peptide‐2 (GLP‐2) analogue teduglutide. Over the course of treatment with teduglutide, the patient was able to achieve independence from parenteral nutrition and experienced a significant improvement in quality of life (QoL) as stool frequency and consistency improved, urinary output was stabilized and body weight as well as body composition improved over the course of teduglutide therapy. With no longer being exposed to the burden and associated risks of parenteral nutrition, we were able to reduce the potential morbidity and mortality rate as well as to improve the patient's overall QoL. Intestinal tissue biopsy workup revealed a histopathological correlate for the clinical response; Congo‐Red‐positive intestinal depositions almost completely disappeared within 6 months of teduglutide therapy. Implementing intestinotrophic GLP‐2 analogue teduglutide may enrich the spectrum of treatment options for amyloidosis patients with IF who are dependent on parenteral support.
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spelling pubmed-104338322023-08-18 Teduglutide in amyloidosis‐associated intestinal failure Luhn, Clara Agis, Hermine Hütterer, Elisabeth Simonitsch‐Klupp, Ingrid Dawoud, Christopher Stift, Anton Harpain, Felix Clin Case Rep Case Report Amyloidosis is a heterogeneous disease characterized by tissue deposition of abnormally folded fibrillary proteins that can manifest itself by a wide variety of symptoms depending on the affected organs. GI involvement among amyloidosis patients is common. Its clinical manifestation often presents with nonspecific symptoms such as weight loss, diarrhea, and malabsorption. With no specific treatment existing for GI amyloidosis, therapy focuses on impeding amyloid deposition and managing the patients' symptoms with supportive measures. Here, we present an AL‐amyloidosis patient with GI involvement and intestinal failure (IF) who was successfully treated with the glucagon‐like peptide‐2 (GLP‐2) analogue teduglutide. Over the course of treatment with teduglutide, the patient was able to achieve independence from parenteral nutrition and experienced a significant improvement in quality of life (QoL) as stool frequency and consistency improved, urinary output was stabilized and body weight as well as body composition improved over the course of teduglutide therapy. With no longer being exposed to the burden and associated risks of parenteral nutrition, we were able to reduce the potential morbidity and mortality rate as well as to improve the patient's overall QoL. Intestinal tissue biopsy workup revealed a histopathological correlate for the clinical response; Congo‐Red‐positive intestinal depositions almost completely disappeared within 6 months of teduglutide therapy. Implementing intestinotrophic GLP‐2 analogue teduglutide may enrich the spectrum of treatment options for amyloidosis patients with IF who are dependent on parenteral support. John Wiley and Sons Inc. 2023-08-17 /pmc/articles/PMC10433832/ /pubmed/37601424 http://dx.doi.org/10.1002/ccr3.7653 Text en © 2023 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Luhn, Clara
Agis, Hermine
Hütterer, Elisabeth
Simonitsch‐Klupp, Ingrid
Dawoud, Christopher
Stift, Anton
Harpain, Felix
Teduglutide in amyloidosis‐associated intestinal failure
title Teduglutide in amyloidosis‐associated intestinal failure
title_full Teduglutide in amyloidosis‐associated intestinal failure
title_fullStr Teduglutide in amyloidosis‐associated intestinal failure
title_full_unstemmed Teduglutide in amyloidosis‐associated intestinal failure
title_short Teduglutide in amyloidosis‐associated intestinal failure
title_sort teduglutide in amyloidosis‐associated intestinal failure
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10433832/
https://www.ncbi.nlm.nih.gov/pubmed/37601424
http://dx.doi.org/10.1002/ccr3.7653
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