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CFTR modulator theratyping: Current status, gaps and future directions.
Clancy JP, Cotton CU, Donaldson SH, Solomon GM, VanDevanter DR, Boyle MP, Gentzsch M, Nick JA, Illek B, Wallenburg JC, Sorscher EJ, Amaral MD, Beekman JM, Naren AP, Bridges RJ, Thomas PJ, Cutting G, Rowe S, Durmowicz AG, Mense M, Boeck KD, Skach W, Penland C, Joseloff E, Bihler H, Mahoney J, Borowitz D, Tuggle KL. Clancy JP, et al. Among authors: borowitz d. J Cyst Fibros. 2019 Jan;18(1):22-34. doi: 10.1016/j.jcf.2018.05.004. Epub 2018 Jun 20. J Cyst Fibros. 2019. PMID: 29934203 Free PMC article. Review.
The theme of the workshop was centered on CFTR theratyping, a term that encompasses the use of CFTR modulators to define defects in CFTR in vitro, with application to both common and rare CFTR variants. ...
The theme of the workshop was centered on CFTR theratyping, a term that encompasses the use of CFTR modulators to define defects in C …
Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis.
Ren CL, Borowitz DS, Gonska T, Howenstine MS, Levy H, Massie J, Milla C, Munck A, Southern KW. Ren CL, et al. Among authors: borowitz ds. J Pediatr. 2017 Feb;181S:S45-S51.e1. doi: 10.1016/j.jpeds.2016.09.066. J Pediatr. 2017. PMID: 28129812
However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive for Pseudomonas aeruginosa. Most studies have only reported short-term outcomes in the first 1-3 years of life; the long-term …
However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive …
Outcomes of infants with indeterminate diagnosis detected by cystic fibrosis newborn screening.
Ren CL, Fink AK, Petren K, Borowitz DS, McColley SA, Sanders DB, Rosenfeld M, Marshall BC. Ren CL, et al. Among authors: borowitz ds. Pediatrics. 2015 Jun;135(6):e1386-92. doi: 10.1542/peds.2014-3698. Epub 2015 May 11. Pediatrics. 2015. PMID: 25963008
The goal of this study was to determine the prevalence, clinical features, and short-term outcomes of infants with CRMS. METHODS: We analyzed data from the US CF Foundation Patient Registry (CFFPR) from 2010 to 2012. ...
The goal of this study was to determine the prevalence, clinical features, and short-term outcomes of infants with CRMS. METHODS: We …
Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST).
McKone EF, Borowitz D, Drevinek P, Griese M, Konstan MW, Wainwright C, Ratjen F, Sermet-Gaudelus I, Plant B, Munck A, Jiang Y, Gilmartin G, Davies JC; VX08-770-105 (PERSIST) Study Group. McKone EF, et al. Among authors: borowitz d. Lancet Respir Med. 2014 Nov;2(11):902-910. doi: 10.1016/S2213-2600(14)70218-8. Epub 2014 Oct 9. Lancet Respir Med. 2014. PMID: 25311995 Clinical Trial.
Patients were eligible if they had a Gly551Asp-CFTR mutation on at least one allele. The primary objective was to assess the long-term safety profile of ivacaftor as assessed by adverse events, clinical laboratory assessments, electrocardiograms, vital signs, and physical …
Patients were eligible if they had a Gly551Asp-CFTR mutation on at least one allele. The primary objective was to assess the long-term …
A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon.
Carlyle BE, Borowitz DS, Glick PL. Carlyle BE, et al. Among authors: borowitz ds. J Pediatr Surg. 2012 Apr;47(4):772-81. doi: 10.1016/j.jpedsurg.2012.02.019. J Pediatr Surg. 2012. PMID: 22498395 Review.
METHODS: We identified areas of interest including pathophysiology, prenatal diagnosis, nonoperative and operative management, postoperative management, and prognosis. We performed a Medline search using the search term meconium ileus for English language articles publishe …
METHODS: We identified areas of interest including pathophysiology, prenatal diagnosis, nonoperative and operative management, postoperative …
Liprotamase long-term safety and support of nutritional status in pancreatic-insufficient cystic fibrosis.
Borowitz D, Stevens C, Brettman LR, Campion M, Wilschanski M, Thompson H; Liprotamase 767 Study Group. Borowitz D, et al. J Pediatr Gastroenterol Nutr. 2012 Feb;54(2):248-57. doi: 10.1097/MPG.0b013e31823315d1. J Pediatr Gastroenterol Nutr. 2012. PMID: 22266487 Clinical Trial.
Liprotamase, a nonporcine, highly purified biotechnology-derived PERT, has demonstrated significant efficacy in fat and protein malabsorption in patients with EPI compared to placebo. This study of liprotamase is the first ever long-term trial of a PERT to evaluate safety …
Liprotamase, a nonporcine, highly purified biotechnology-derived PERT, has demonstrated significant efficacy in fat and protein malabsorptio …
Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study.
Aitken ML, Bellon G, De Boeck K, Flume PA, Fox HG, Geller DE, Haarman EG, Hebestreit HU, Lapey A, Schou IM, Zuckerman JB, Charlton B; CF302 Investigators. Aitken ML, et al. Am J Respir Crit Care Med. 2012 Mar 15;185(6):645-52. doi: 10.1164/rccm.201109-1666OC. Epub 2011 Dec 28. Am J Respir Crit Care Med. 2012. PMID: 22198974 Clinical Trial.
RATIONALE: New treatment strategies are needed to improve airway clearance and reduce the morbidity and the time burden associated with cystic fibrosis (CF). OBJECTIVES: To determine whether long-term treatment with inhaled mannitol, an osmotic agent, improves lung functio …
RATIONALE: New treatment strategies are needed to improve airway clearance and reduce the morbidity and the time burden associated with cyst …
Cystic Fibrosis Foundation practice guidelines for the management of infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome during the first two years of life and beyond.
Cystic Fibrosis Foundation; Borowitz D, Parad RB, Sharp JK, Sabadosa KA, Robinson KA, Rock MJ, Farrell PM, Sontag MK, Rosenfeld M, Davis SD, Marshall BC, Accurso FJ. Cystic Fibrosis Foundation, et al. Among authors: borowitz d. J Pediatr. 2009 Dec;155(6 Suppl):S106-16. doi: 10.1016/j.jpeds.2009.09.003. J Pediatr. 2009. PMID: 19914443 Free PMC article.
Mutations in the CFTR gene can cause CF, but not all CFTR mutations are disease-causing. The term CFTR-related metabolic syndrome (CRMS) is proposed to describe infants identified by hypertrypsinogenemia on NBS who have sweat chloride values <60 mmol/L and up to 2 CFTR …
Mutations in the CFTR gene can cause CF, but not all CFTR mutations are disease-causing. The term CFTR-related metabolic syndrome (CR …
Association of socioeconomic status with the use of chronic therapies and healthcare utilization in children with cystic fibrosis.
Schechter MS, McColley SA, Silva S, Haselkorn T, Konstan MW, Wagener JS; Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis; North American Scientific Advisory Group for ESCF. Schechter MS, et al. J Pediatr. 2009 Nov;155(5):634-9.e1-4. doi: 10.1016/j.jpeds.2009.04.059. Epub 2009 Jul 16. J Pediatr. 2009. PMID: 19608199 Free PMC article.
Safety and preliminary clinical activity of a novel pancreatic enzyme preparation in pancreatic insufficient cystic fibrosis patients.
Borowitz D, Goss CH, Stevens C, Hayes D, Newman L, O'Rourke A, Konstan MW, Wagener J, Moss R, Hendeles L, Orenstein D, Ahrens R, Oermann CM, Aitken ML, Mahl TC, Young KR Jr, Dunitz J, Murray FT. Borowitz D, et al. Pancreas. 2006 Apr;32(3):258-63. doi: 10.1097/01.mpa.0000202952.10612.21. Pancreas. 2006. PMID: 16628080 Clinical Trial.
At the other dosing levels, the mean coefficient of fat and nitrogen absorption increases were 19.1% +/- 24.9% and 17.8% +/- 13.6%, respectively, whereas the mean stool weight decreased by 517 +/- 362 g. CONCLUSIONS: TCT was well tolerated in this short-term exposure study …
At the other dosing levels, the mean coefficient of fat and nitrogen absorption increases were 19.1% +/- 24.9% and 17.8% +/- 13.6%, respecti …
11 results