The changing face of the exocrine pancreas in cystic fibrosis: the correlation between pancreatic status, pancreatitis and cystic fibrosis genotype

Augarten, Arie; Ben‐Tov, Amir; Madgar, Igael; Barak, Asher; Akons, Hanna; Laufer, J; Efrati, Ori; Aviram, Micha; Bentur, Lea; Blau, Hannah; Paret, Gideon; Wilschanski, Michael; Kerem, Batsheva; Yahav, Yaacov

doi:10.1097/meg.0b013e3282f36d04

Cited by 55 publications

(41 citation statements)

References 26 publications

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“…Most CF patients carrying functionally severe mutations on both alleles have a PI phenotype [13][14][15][16]. A small proportion of patients carrying severe mutations on both alleles are PS at diagnosis, but most progressively transition from PS to PI state.…”

Section: Introductionmentioning

confidence: 99%

Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis

Ooi

Durie

2012

Journal of Cystic Fibrosis

102

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The genotype-phenotype correlation in pancreatitis is unique compared to other organ manifestations but still consistent with the complex monogenic nature of CF. Paradoxically, genotypes associated with otherwise mild phenotypic effects have a greater risk for causing pancreatitis; compared with genotypes associated with moderate to severe disease phenotypes. Greater understanding into the underlying mechanisms of disease is much needed. The emergence of CFTR-assist therapies may potentially play a future role in the treatment of CFTR-mutation associated pancreatitis.

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Section: Introductionmentioning

confidence: 99%

Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis

Ooi

Durie

2012

Journal of Cystic Fibrosis

102

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“…Fecal concentrations of E1 were < 30 μg/g of stool in all PI CF patients (9 μg [6][7][8][9][10][11] At the baseline, no differences between the groups with regard to the diameter of the pancreatic duct in the head, body, and tail of the pancreas were stated. The pancreas in PI CF patients was narrower than in PS CF patients when measured at the level of the head, the body, and the tail.…”

Section: Resultsmentioning

confidence: 99%

“…It was shown that between 10% and 28% of CF patients are PS [6][7][8][9] and this phenotypic property is not readily predicted by the genotype [10]. Only PS CF patients may be affected by pancreatitis, which may lead to insufficiency in the long term [8]. New approaches are needed to identify such patients at an early stage.…”

Section: Introductionmentioning

confidence: 99%

Secretin-enhanced Magnetic Resonance Cholangiopancreatography in Pancreatic Insufficient and Pancreatic Sufficient Cystic Fibrosis Patients

Jończyk‐Potoczna

Nowak

Mądry

et al. 2016

JGLD

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Background & Aims: Although indirect methods of assessment of the exocrine pancreatic function have become the standard of care in the monitoring of pancreatic status, it still remains a current clinical challenge. Our aim was to compare the width of the pancreatic duct in pancreatic insufficient (PI) and pancreatic sufficient (PS) cystic fibrosis (CF) patients using secretin-enhanced magnetic resonance cholangiopancreatography (SE-MRCP). Methods: Thirty-seven CF patients were enrolled for this cross-sectional study, including 21 PI and 16 PS, all of whom underwent SE-MRCP. Measurement of the diameter of the pancreatic duct was performed in the head, body, and the tail of the pancreas at the baseline and after 1, 2, 3, 5, and 10 minutes after secretin administration. Results: The diameter of the pancreatic duct in the head of the pancreas after 5 and 10 minutes of secretin injection was greater in PI than in PS patients (median = 2.0 mm [interquartile range: 1.6-3.0] vs. 2.0 mm [1.0-2.0] and 2.0 mm [1.4-2.0] vs 1.0 mm [1.0-2.0], p=0.047 and p=0.040, respectively). Areas under ROC curves for discriminating between PI and PS patients were 0.693 (95% CI 0.521-0.866) and 0.698 (95% CI 0.528-0.868), respectively. No other differences in the width of the duct were identified at the baseline or during SE-MRCP. Conclusions: The measurement of the diameter of the pancreatic duct during secretin stimulation does not allow for differentiating between PS and PI status in CF patients. Abbreviations: CF: cystic fibrosis; CFTR: cystic fibrosis transmembrane conductance regulator; ELISA: enzyme-linked immunosorbent assay; ERCP: endoscopic retrograde cholangiopancreatography; E1: elastase-1; MRI: magnetic resonance imaging; PI: pancreatic insufficient; PS: pancreatic sufficient; SCT gene: secretin gene; SE-MRCP: secretin-enhanced magnetic resonance cholangiopancreatography; T: tesla; TR: repetition time; TE: echo time.

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“…3 Approximately 80% to 90% of CF patients have pancreatic insufficiency. 5 Cohn et al found an increased association of CFTR mutations in patients with idiopathic chronic pancreatitis. 6 It has been controversial if patients with chronic pancreatitis have an increased risk of developing pancreatic ductal adenocarcinoma (PDA).…”

Section: Introductionmentioning

confidence: 98%

Subject Review: Pancreatic Ductal Adenocarcinoma in the Setting of Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator Gene: Case Report and Review of the Literature

Rittenhouse

Talbott

Anklesaria

et al. 2011

Journal of Gastrointestinal Surgery

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The changing face of the exocrine pancreas in cystic fibrosis: the correlation between pancreatic status, pancreatitis and cystic fibrosis genotype

Cited by 55 publications

References 26 publications

Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis

Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis

Secretin-enhanced Magnetic Resonance Cholangiopancreatography in Pancreatic Insufficient and Pancreatic Sufficient Cystic Fibrosis Patients

Subject Review: Pancreatic Ductal Adenocarcinoma in the Setting of Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator Gene: Case Report and Review of the Literature

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