CFTR modulators increase risk of acute pancreatitis in pancreatic insufficient patients with cystic fibrosis
| Autor: | G. Galante, Helen Machida, Michelle Gould, Tanja Gonska, Jonathan H. Rayment, Haley Smith |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Pulmonary and Respiratory Medicine
medicine.medical_specialty Side effect Cystic Fibrosis Cystic Fibrosis Transmembrane Conductance Regulator Cystic fibrosis Gastroenterology Internal medicine medicine Humans Magnetic resonance cholangiopancreatography biology medicine.diagnostic_test business.industry medicine.disease Cystic fibrosis transmembrane conductance regulator medicine.anatomical_structure Pancreatitis Pediatrics Perinatology and Child Health Acute Disease Mutation biology.protein Acute pancreatitis Exocrine Pancreatic Insufficiency Pancreas business Cftr modulator |
| Zdroj: | Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 21(4) |
| ISSN: | 1873-5010 |
| Popis: | Patients with pancreatic insufficient cystic fibrosis rarely develop acute pancreatitis due to insufficient acinar reserve. We describe a series of five patients under the age of 18 (range 8-16 years) with pancreatic insufficient cystic fibrosis who developed a phenotype in keeping with acute pancreatitis following initiation of CFTR modulator therapy. This occurred at a median of 30 months following CFTR modulator initiation. 3/5 of these patients also developed pancreatic sufficiency or at least an intermediary pancreas status, indicated by fecal elastases above 100 μg/g. This series highlights a mostly unrecognized potential side effect of this therapy as well as the potential of CFTR modulator therapies to improve exocrine pancreatic function, even in adolescent patients. |
| Databáze: | OpenAIRE |
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