Cystic Fibrosis–Related Pancreatic Cysts Decrease in Size and Number Upon Treatment With Cystic Fibrosis Transmembrane Conductance Regulator Modulators

Vries, Jonathan M de; Green, Deanna; Kučera, J; Fabbrini, Abigail L; Kidder, Molly; Brown, Jerry M.; Wilsey, Michael

doi:10.1097/mpa.0000000000001567

Cited by 2 publications

(1 citation statement)

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“…Newer therapies, such as CFTR modulators, have shown great promise in treating CFTR-related pancreatic disease ( 10 ). However, future studies are needed to determine whether CFTR modulator treatment may provide risk-reduction of pancreatic ductal stone formation in the setting of CFTR-related CP.…”

Section: Discussionmentioning

confidence: 99%

No Stone Left Unturned: Pediatric Pancreatic Stones Presenting With Obstructive Jaundice

deVries¹,

Sidhu²,

Kimsey³

et al. 2022

JPGN Reports

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Pancreatic lithiasis, the formation of calcifications in the pancreatic duct, occurs uncommonly in pediatric patients but can occur more frequently with chronic pancreatitis (CP). Cystic fibrosis (CF) is one of the major causes of pancreatic lithiasis in pediatric patients, with mutations in the CF transmembrane conductance regulator (CFTR) gene reported in up to 23% of pediatric CP patients. Mutations in the CFTR gene can lead to mild cases of CF, which may delay diagnosis and treatment. In such cases, pancreatitis can be the presenting symptom in children with CF. We report a unique case of a 10-year-old female with previously undiagnosed and untreated CF presenting with abdominal pain, vomiting, and obstructive jaundice. Her pancreatic lithiasis and biliary obstruction were successfully treated with endoscopic retrograde cholangiopancreatography (ERCP).

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Section: Discussionmentioning

confidence: 99%