Full Text Journal Articles by
Author Sonya L Heltshe

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Pulmonary Outcomes Associated with Long-Term Azithromycin Therapy in Cystic Fibrosis.

Dave P Nichols, Katherine Odem-Davis, Jonathan D Cogen, Christopher H Goss, Clement L Ren, Michelle Skalland, Ranjani Somayaji, Sonya L Heltshe,

Rationale: Chronic azithromycin is commonly used in cystic fibrosis based on short controlled clinical trials showing reductions in pulmonary exacerbations and improved FEV1. Long-term effects are unknown.Objectives: Examine pulmonary outcomes among chronic azithromycin users compared with matched controls over years of use and consider combined azithromycin use in cohorts using ... Read more >>

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2020, 201(4):430-437]

Cited: 0 times

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Changes in Airway Microbiome and Inflammation with Ivacaftor Treatment in Patients with Cystic Fibrosis and the G551D Mutation.

J Kirk Harris, Brandie D Wagner, Edith T Zemanick, Charles E Robertson, Mark J Stevens, Sonya L Heltshe, Steven M Rowe, Scott D Sagel,

Rationale: Modulation of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein improves clinical outcomes in patients with CF and specific CFTR genetic mutations. It remains unclear how improving CFTR function modifies existing airway infection and inflammation.Objectives: To compare sputum microbiome and markers of inflammation before and after 6 months ... Read more >>

Ann Am Thorac Soc (Annals of the American Thoracic Society)
[2020, 17(2):212-220]

Cited: 0 times

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Contraceptive use among women with cystic fibrosis: A pilot study linking reproductive health questions to the Cystic Fibrosis Foundation National Patient Registry.

Emily M Godfrey, Sheila Mody, Malaika R Schwartz, Sonya L Heltshe, Jennifer L Taylor-Cousar, Raksha Jain, Sandra Sufian, Tatiana Josephy, Moira L Aitken,

OBJECTIVES:To examine contraceptive use, pregnancy intention and the association of hormonal contraceptive type with adverse health outcomes among women with cystic fibrosis (CF). STUDY DESIGN:We recruited 150 women with CF, ages 18-49 from three adult CF programs to complete an online survey regarding their pregnancy and contraceptive use history. Survey ... Read more >>

Contraception (Contraception)
[2020, 101(6):420-426]

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Fecal dysbiosis in infants with cystic fibrosis is associated with early linear growth failure.

Hillary S Hayden, Alexander Eng, Christopher E Pope, Mitchell J Brittnacher, Anh T Vo, Eli J Weiss, Kyle R Hager, Bryan D Martin, Daniel H Leung, Sonya L Heltshe, Elhanan Borenstein, Samuel I Miller, Lucas R Hoffman,

Most infants with cystic fibrosis (CF) have pancreatic exocrine insufficiency that results in nutrient malabsorption and requires oral pancreatic enzyme replacement. Newborn screening for CF has enabled earlier diagnosis, nutritional intervention and enzyme replacement for these infants, allowing most infants with CF to achieve their weight goals by 12 months ... Read more >>

Nat. Med. (Nature medicine)
[2020, 26(2):215-221]

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Utilizing centralized biorepository samples for biomarkers of cystic fibrosis lung disease severity.

Scott D Sagel, Brandie D Wagner, Assem Ziady, Tom Kelley, John P Clancy, Monica Narvaez-Rivas, Joseph Pilewski, Elizabeth Joseloff, Wei Sha, Leila Zelnick, Kenneth D R Setchell, Sonya L Heltshe, Marianne S Muhlebach,

BACKGROUND:Circulating biomarkers reflective of lung disease activity and severity have the potential to improve patient care and accelerate drug development in CF. The objective of this study was to leverage banked specimens to test the hypothesis that blood-based biomarkers discriminate CF children segregated by lung disease severity. METHODS:Banked serum samples ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2019, :]

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Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males.

Katharine E Secunda, Jennifer S Guimbellot, Borko Jovanovic, Sonya L Heltshe, Scott D Sagel, Steven M Rowe, Manu Jain,

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2020, 201(8):996-998]

Cited: 0 times

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Does Ivacaftor Taken Twice a Day Keep the Pseudomonas Away?

Alex H Gifford, Sonya L Heltshe,

Ann Am Thorac Soc (Annals of the American Thoracic Society)
[2019, 16(11):1366-1367]

Cited: 0 times

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CFTR Modulator Use Is Associated with Higher Hemoglobin Levels in Individuals with Cystic Fibrosis.

Alex H Gifford, Sonya L Heltshe, Christopher H Goss,

RATIONALE:Understanding how cystic fibrosis transmembrane conductance regulator (CFTR) modulators influence comorbid conditions like anemia is of great interest to the cystic fibrosis community. OBJECTIVES:To test the hypothesis that CFTR modulators are associated with higher hemoglobin (Hgb) levels. METHODS:Annualized Hgb and other laboratory, demographic, and anthropometric data were abstracted from the ... Read more >>

Ann Am Thorac Soc (Annals of the American Thoracic Society)
[2019, 16(3):331-340]

Cited: 1 time

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Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV1) in cystic fibrosis patients receiving ivacaftor treatment.

Béla Nagy, Zsolt Bene, Zsolt Fejes, Sonya L Heltshe, David Reid, Nicola J Ronan, Yvonne McCarthy, Daniel Smith, Attila Nagy, Elizabeth Joseloff, György Balla, János Kappelmayer, Milan Macek, Scott C Bell, Barry J Plant, Margarida D Amaral, István Balogh,

BACKGROUND:We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy. METHODS:In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2019, 18(2):271-277]

Cited: 0 times

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Ivacaftor-treated Patients with Cystic Fibrosis Derive Long-Term Benefit Despite No Short-Term Clinical Improvement.

Sonya L Heltshe, Steven M Rowe, Michelle Skalland, Arthur Baines, Manu Jain, ,

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2018, 197(11):1483-1486]

Cited: 2 times

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Pancreatic Enzyme Replacement Therapy Use in Infants With Cystic Fibrosis Diagnosed by Newborn Screening.

Daniel Gelfond, Sonya L Heltshe, Michelle Skalland, James E Heubi, Margaret Kloster, Daniel H Leung, Bonnie W Ramsey, Drucy Borowitz, ,

OBJECTIVES:The aim of the study is to describe pancreatic enzyme practices during the first year of life in infants with cystic fibrosis (CF) and evaluate associations between dosing and outcomes, including growth and gastrointestinal (GI) symptoms. METHODS:We analyzed data from a subset of infants who were in a prospective cohort ... Read more >>

J. Pediatr. Gastroenterol. Nutr. (Journal of pediatric gastroenterology and nutrition)
[2018, 66(4):657-663]

Cited: 2 times

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Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.

Jennifer Guimbellot, George M Solomon, Arthur Baines, Sonya L Heltshe, Jill VanDalfsen, Elizabeth Joseloff, Scott D Sagel, Steven M Rowe, ,

BACKGROUND:The cystic fibrosis transmembrane conductance regulator (CFTR) potentiator ivacaftor is approved for patients with CF with gating and residual function CFTR mutations. We report the results of an observational study investigating its effects in CF patients with non-G551D gating mutations. METHODS:Patients with non-G551D gating mutations were recruited to an open-label ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2019, 18(1):102-109]

Cited: 1 time

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Let's talk about sex: Behaviors, experience and health care utilization in young women with CF.

Sonya L Heltshe, Jennifer L Taylor-Cousar,

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2018, 17(1):5-6]

Cited: 0 times

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Study design considerations for the Standardized Treatment of Pulmonary Exacerbations 2 (STOP2): A trial to compare intravenous antibiotic treatment durations in CF.

Sonya L Heltshe, Natalie E West, Donald R VanDevanter, D B Sanders, Valeria V Beckett, Patrick A Flume, Christopher H Goss, ,

BACKGROUND:Pulmonary exacerbations (PEx) in cystic fibrosis (CF) are common and contribute to morbidity and mortality. Duration of IV antibiotic therapy to treat PEx varies widely in the US, and there are few data to guide treatment decisions. METHODS:We combined a survey of CF stakeholders with retrospective analyses of a recent ... Read more >>

Contemp Clin Trials (Contemporary clinical trials)
[2018, 64:35-40]

Cited: 6 times

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Effects of Diagnosis by Newborn Screening for Cystic Fibrosis on Weight and Length in the First Year of Life.

Daniel H Leung, Sonya L Heltshe, Drucy Borowitz, Daniel Gelfond, Margaret Kloster, James E Heubi, Michael Stalvey, Bonnie W Ramsey, ,

Importance:Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. Objective:To examine incremental weight gain, linear growth, and clinical features in the ... Read more >>

JAMA Pediatr (JAMA pediatrics)
[2017, 171(6):546-554]

Cited: 15 times

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Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections.

Katherine B Hisert, Sonya L Heltshe, Christopher Pope, Peter Jorth, Xia Wu, Rachael M Edwards, Matthew Radey, Frank J Accurso, Daniel J Wolter, Gordon Cooke, Ryan J Adam, Suzanne Carter, Brenda Grogan, Janice L Launspach, Seamas C Donnelly, Charles G Gallagher, James E Bruce, David A Stoltz, Michael J Welsh, Lucas R Hoffman, Edward F McKone, Pradeep K Singh,

Previous work indicates that ivacaftor improves cystic fibrosis transmembrane conductance regulator (CFTR) activity and lung function in people with cystic fibrosis and G551D-CFTR mutations but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently ... Read more >>

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2017, 195(12):1617-1628]

Cited: 38 times

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Standardized Treatment of Pulmonary Exacerbations (STOP) study: Physician treatment practices and outcomes for individuals with cystic fibrosis with pulmonary Exacerbations.

Natalie E West, Valeria V Beckett, Raksha Jain, Don B Sanders, Jerry A Nick, Sonya L Heltshe, Elliott C Dasenbrook, Donald R VanDevanter, George M Solomon, Christopher H Goss, Patrick A Flume, ,

BACKGROUND:Pulmonary Exacerbations (PEx) are associated with increased morbidity and mortality in individuals with CF. PEx management practices vary widely, and optimization through interventional trials could potentially improve outcomes. The object of this analysis was to evaluate current physician treatment practices and patient outcomes for PEx. METHODS:The Standardized Treatment of Pulmonary ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2017, 16(5):600-606]

Cited: 16 times

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Standardized Treatment of Pulmonary Exacerbations (STOP) study: Observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations.

Don B Sanders, George M Solomon, Valeria V Beckett, Natalie E West, Cori L Daines, Sonya L Heltshe, Donald R VanDevanter, Jonathan E Spahr, Ronald L Gibson, Jerry A Nick, Bruce C Marshall, Patrick A Flume, Christopher H Goss, ,

BACKGROUND:The Standardized Treatment of Pulmonary Exacerbations (STOP) program has the intent of defining best practices in the treatment of pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF). The objective of this analysis was to describe the clinical presentations of patients admitted for intravenous (IV) antibiotics and enrolled in a ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2017, 16(5):592-599]

Cited: 12 times

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Pregnancy among cystic fibrosis women in the era of CFTR modulators.

Sonya L Heltshe, Emily M Godfrey, Tatiana Josephy, Moira L Aitken, Jennifer L Taylor-Cousar,

BACKGROUND:Little is known about how new therapies that partially correct the basic cystic fibrosis (CF) defect (ivacaftor and lumacaftor) might alter hormonal contraceptive effectiveness, impact pregnancy outcomes, or affect pregnancy timing. Examination of pregnancy rates among CF women during periods of CFTR modulator therapy initiation will provide foundation for further ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2017, 16(6):687-694]

Cited: 0 times

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Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor.

Michael S Stalvey, Jesse Pace, Minoo Niknian, Mark N Higgins, Valerie Tarn, Joy Davis, Sonya L Heltshe, Steven M Rowe,

Cystic fibrosis (CF) is known for its impact on the lung and pancreas of individuals; however, impaired growth is also a common complication. We hypothesized that targeting the biological defect in the CF transmembrane conductance regulator (CFTR) protein may affect growth outcomes.In this post hoc analysis, we assessed linear growth ... Read more >>

Pediatrics (Pediatrics)
[2017, 139(2):]

Cited: 5 times

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Use of FEV1 in cystic fibrosis epidemiologic studies and clinical trials: A statistical perspective for the clinical researcher.

Rhonda Szczesniak, Sonya L Heltshe, Sanja Stanojevic, Nicole Mayer-Hamblett,

BACKGROUND:Forced expiratory volume in 1s (FEV1) is an established marker of cystic fibrosis (CF) disease progression that is used to capture clinical course and evaluate therapeutic efficacy. The research community has established FEV1 surveillance data through a variety of observational data sources such as patient registries, and there is a ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2017, 16(3):318-326]

Cited: 13 times

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Heterogeneity in Survival in Adult Patients With Cystic Fibrosis With FEV1 < 30% of Predicted in the United States.

Kathleen J Ramos, Bradley S Quon, Sonya L Heltshe, Nicole Mayer-Hamblett, Erika D Lease, Moira L Aitken, Noel S Weiss, Christopher H Goss,

Lung transplantation (LTx) is frequently considered for patients with cystic fibrosis (CF) when the FEV1 reaches < 30%. This study estimated transplant-free survival for patients with CF and an FEV1 < 30% and identified predictors of death without LTx.We conducted a retrospective cohort study using the CF Foundation Patient Registry from January 1, ... Read more >>

Chest (Chest)
[2017, 151(6):1320-1328]

Cited: 13 times

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Cystic Fibrosis: The Dawn of a New Therapeutic Era.

Sonya L Heltshe, Jonathan Cogen, Kathleen J Ramos, Christopher H Goss,

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2017, 195(8):979-984]

Cited: 3 times

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Potential for Therapeutic Benefit among Cystic Fibrosis Populations Excluded from Clinical Trials or Labeling of Marketed Therapies.

Donald R VanDevanter, Sonya L Heltshe, John J LiPuma,

Remarkable advances in the management of individuals born with cystic fibrosis (CF) would not have been realized without empiric trial and error by CF clinicians with treatments developed and available for other purposes. As the testing and registration of CF-specific treatments have increased, so too have associated health care costs, ... Read more >>

Ann Am Thorac Soc (Annals of the American Thoracic Society)
[2016, 13(11):1890-1893]

Cited: 2 times

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Biomarkers for cystic fibrosis drug development.

Marianne S Muhlebach, J P Clancy, Sonya L Heltshe, Assem Ziady, Tom Kelley, Frank Accurso, Joseph Pilewski, Nicole Mayer-Hamblett, Elizabeth Joseloff, Scott D Sagel,

To provide a review of the status of biomarkers in cystic fibrosis drug development, including regulatory definitions and considerations, a summary of biomarkers in current use with supportive data, current gaps, and future needs.Biomarkers are considered across several areas of CF drug development, including cystic fibrosis transmembrane conductance regulator modulation, ... Read more >>

J. Cyst. Fibros. (Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society)
[2016, 15(6):714-723]

Cited: 14 times

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