229 Pancreatic genotype-phenotype co-variations in cystic fibrosis

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• 作者：T. Engjom^a ; ^b ; B.N. Læ ; rum^c ; ^d ; K. Nylund^b ; F. Erchinger^a ; ^e ; O.H. Gilja^a ; ^b ; ^f ; G. Dimcevski^a ; ^b
• 刊名：Journal of Cystic Fibrosis
• 出版年：2015
• 出版时间：June 2015
• 年：2015
• 卷：14
• 期：supp_S1
• 页码：S117
• 全文大小：66 K

文摘

Pancreas insufficiency prevalence (PIP) has been suggested by Ooi and co-workers as a tool for predicting pancreatic disease in cystic fibrosis (CF). We aimed to correlate PIP to pancreatic phenotype.

Methods

We included 42 CF patients. Exocrine pancreatic function was examined by faecal elastase or endoscopic secretin test. CFTR genotype was assessed by standard CFTR screening kits or CFTR sequencing. PIP corresponding to the least severe mutation was adapted from Ooi, or calculated from our own database. Pancreatic ultrasonography was performed: First a secretin-stimulated ultrasonography with area tracing of the fluid filled descending duodenum, then a dedicated ultrasonography of the pancreas including contrast enhancement by Sonovue® and pancreas/liver signal-intensity measures. Perfusion data were calculated by DCE-US software. PIP score grouped patients according to genotype severity as severe genotype (PIP <0.4) or mild genotype (PIP ≤0.4).

Results

We found reduced f-elastase, d-bicarbonate and pancreatic volume output (d-Area), increased pancreatic echo-intensity (LP SIR) and reduced pancreatic blood flow in the group with severe compared to the group with mild pancreatic genotype. Results are presented in the table.

Conclusion

Severe PIP predicts affected pancreas represented by exocrine pancreatic failure, fatty infiltration and reduced pancreas perfusion.