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Immunoreactive trypsinogen levels in newborn screened infants with an inconclusive diagnosis of cystic fibrosis.
Ooi CY, Sutherland R, Castellani C, Keenan K, Boland M, Reisman J, Bjornson C, Chilvers MA, van Wylick R, Kent S, Price A, Mateos-Corral D, Hughes D, Solomon M, Zuberbuhler P, Brusky J, Durie PR, Ratjen F, Gonska T. Ooi CY, et al. Among authors: durie pr. BMC Pediatr. 2019 Oct 22;19(1):369. doi: 10.1186/s12887-019-1756-4. BMC Pediatr. 2019. PMID: 31640630 Free PMC article.
CF transmembrane conductance regulator (CFTR)-related metabolic syndrome (CRMS) or CF screen positive inconclusive diagnosis (CFSPID). These infants have an uncertain long-term outcome and it is currently unclear around time of diagnosis, which infants are at higher risk o …
CF transmembrane conductance regulator (CFTR)-related metabolic syndrome (CRMS) or CF screen positive inconclusive diagnosis (CFSPID). These …
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.
Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, De Boeck K, Donaldson SH, Dorkin HL, Dunitz JM, Durie PR, Jain M, Leonard A, McCoy KS, Moss RB, Pilewski JM, Rosenbluth DB, Rubenstein RC, Schechter MS, Botfield M, Ordoñez CL, Spencer-Green GT, Vernillet L, Wisseh S, Yen K, Konstan MW. Clancy JP, et al. Among authors: durie pr. Thorax. 2012 Jan;67(1):12-8. doi: 10.1136/thoraxjnl-2011-200393. Epub 2011 Aug 8. Thorax. 2012. PMID: 21825083 Free PMC article. Clinical Trial.
Additional data are needed to determine how improvements detected in CFTR function secondary to VX-809 in the sweat gland relate to those measurable in the respiratory tract and to long-term measures of clinical benefit. CLINICAL TRIAL NUMBER: NCT00865904....
Additional data are needed to determine how improvements detected in CFTR function secondary to VX-809 in the sweat gland relate to those me …
Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis.
Beharry S, Ackerley C, Corey M, Kent G, Heng YM, Christensen H, Luk C, Yantiss RK, Nasser IA, Zaman M, Freedman SD, Durie PR. Beharry S, et al. Among authors: durie pr. Am J Physiol Gastrointest Liver Physiol. 2007 Mar;292(3):G839-48. doi: 10.1152/ajpgi.00582.2005. Epub 2006 Nov 9. Am J Physiol Gastrointest Liver Physiol. 2007. PMID: 17095751 Free article.
We used a congenic C57Bl/6J cystic fibrosis transmembrane conductance regulator (Cftr)(-/-) mouse model, which develops cystic fibrosis (CF)-like pathology in all organs, to evaluate the short- and long-term therapeutic effects of dietary docosahexaenoic acid (DHA). Thirty …
We used a congenic C57Bl/6J cystic fibrosis transmembrane conductance regulator (Cftr)(-/-) mouse model, which develops cystic fibrosis (CF) …
Pathogenesis of malnutrition in cystic fibrosis, and its treatment.
Pencharz PB, Durie PR. Pencharz PB, et al. Among authors: durie pr. Clin Nutr. 2000 Dec;19(6):387-94. doi: 10.1054/clnu.1999.0079. Clin Nutr. 2000. PMID: 11104588 Review.
Our target is to achieve a completely normal nutritional status. Long term follow-up of these patients has shown significantly better survival in patients who achieve normal nutritional status. ...
Our target is to achieve a completely normal nutritional status. Long term follow-up of these patients has shown significantly better …
Vitamin K and the management of patients with cystic fibrosis.
Durie PR. Durie PR. CMAJ. 1994 Oct 1;151(7):933-6. CMAJ. 1994. PMID: 7922929 Free PMC article. Review.
In addition, various factors--including pancreatic failure, liver disease, bowel resection and long-term use of antibiotics--can put some of these patients at risk of vitamin K deficiency. ...
In addition, various factors--including pancreatic failure, liver disease, bowel resection and long-term use of antibiotics--can put …
Cystic fibrosis: nutrition.
Durie PR, Pencharz PB. Durie PR, et al. Br Med Bull. 1992 Oct;48(4):823-46. doi: 10.1093/oxfordjournals.bmb.a072580. Br Med Bull. 1992. PMID: 1458303 Review.
Undernutrition, with loss of energy stores and lean tissue may in turn contribute to progressive deterioration of lung function. When this occurs, long-term invasive methods of nutritional support can restore energy balance....
Undernutrition, with loss of energy stores and lean tissue may in turn contribute to progressive deterioration of lung function. When this o …
Effects of cisapride in patients with cystic fibrosis and distal intestinal obstruction syndrome.
Koletzko S, Corey M, Ellis L, Spino M, Stringer DA, Durie PR. Koletzko S, et al. Among authors: durie pr. J Pediatr. 1990 Nov;117(5):815-22. doi: 10.1016/s0022-3476(05)83350-5. J Pediatr. 1990. PMID: 2231217 Clinical Trial.
In comparison with unselected patients with cystic fibrosis and pancreatic insufficiency who were receiving enzyme supplements and who had no distal intestinal obstruction, fecal fat losses (percentage of intake) were almost twice as high in the study group with DIOS (31.2 +/- 20 …
In comparison with unselected patients with cystic fibrosis and pancreatic insufficiency who were receiving enzyme supplements and who had n …
Lavage treatment of distal intestinal obstruction syndrome in children with cystic fibrosis.
Koletzko S, Stringer DA, Cleghorn GJ, Durie PR. Koletzko S, et al. Among authors: durie pr. Pediatrics. 1989 May;83(5):727-33. Pediatrics. 1989. PMID: 2717290
The efficacy, adverse reactions, and long-term effects of intestinal lavage treatment with a balanced electrolyte solution (Golytely) was evaluated in patients with cystic fibrosis and distal intestinal obstruction syndrome. ...
The efficacy, adverse reactions, and long-term effects of intestinal lavage treatment with a balanced electrolyte solution (Golytely) …