Restoration of exocrine pancreatic function in child with lumacaftor/ivacaftor therapy in cystic fibrosis

Author(s):  
J. Crowley ◽  
K. Croinin ◽  
D. Mullane ◽  
M. Ní Chróinín
PEDIATRICS ◽  
1986 ◽  
Vol 77 (3) ◽  
pp. 301-306
Author(s):  
Geoffrey Cleghorn ◽  
Lynne Benjamin ◽  
Mary Corey ◽  
Gordon Forstner ◽  
Francesco Dati ◽  
...  

Indirect and qualitative tests of pancreatic function are commonly used to screen patients with cystic fibrosis for pancreatic insufficiency. In an attempt to develop a more quantitative assessment, we compared the usefulness of measuring serum pancreatic lipase using a newly developed enzyme-linked immunosorbent immunoassay with that of cationic trypsinogen using a radioimmunoassay in the assessment of exocrine pancreatic function in patients with cystic fibrosis. Previously, we have shown neither lipase nor trypsinogen to be of use in assessing pancreatic function prior to 5 years of age because the majority of patients with cystic fibrosis in early infancy have elevated serum levels regardless of pancreatic function. Therefore, we studied 77 patients with cystic fibrosis older than 5 years of age, 41 with steatorrhea and 36 without steatorrhea. In addition, 28 of 77 patients consented to undergo a quantitative pancreatic stimulation test. There was a significant difference between the steatorrheic and nonsteatorrheic patients with the steatorrheic group having lower lipase and trypsinogen values than the nonsteatorrheic group (P <.001). Sensitivities and specificities in detecting steatorrhea were 95% and 86%, respectively, for lipase and 93% and 92%, respectively, for trypsinogen. No correlations were found between the serum levels of lipase and trypsinogen and their respective duodenal concentrations because of abnormally high serum levels of both enzymes found in some nonsteatorrheic patients. We conclude from this study that both serum lipase and trypsinogen levels accurately detect steatorrhea in patients with cystic fibrosis who are older than 5 years but are imprecise indicators of specific pancreatic exocrine function above the level needed for normal fat absorption.


Pancreatology ◽  
2016 ◽  
Vol 16 (4) ◽  
pp. S95
Author(s):  
Satoru Naruse ◽  
Shiho Kondo ◽  
Kotoyo Fujiki ◽  
Akiko Yamamoto ◽  
Miyuki Nakakuki ◽  
...  

2013 ◽  
Vol 12 ◽  
pp. S109
Author(s):  
T. Engjom ◽  
B.N. Lrum ◽  
F. Erchinger ◽  
E. Tjora ◽  
O.H. Gilja ◽  
...  

2004 ◽  
Vol 93 (8) ◽  
pp. 1042-1045 ◽  
Author(s):  
J Walkowiak ◽  
A Lisowska ◽  
J Przyslawski ◽  
M Grzymislawski ◽  
M Krawczynski ◽  
...  

2003 ◽  
Vol 124 (4) ◽  
pp. A517
Author(s):  
Jaroslaw Walkowiak ◽  
Karl-Heinz Herzig ◽  
Krystyna Strzykala ◽  
Juliusz Przyslawski ◽  
Marian Grzymislawski ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document