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1. Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials

Author

Nicole Mayer-Hamblett, Felix Ratjen, Renee Russell, Scott H Donaldson, Kristin A Riekert, Gregory S Sawicki, Katherine Odem-Davis, Julia K Young, Daniel Rosenbluth, Jennifer L Taylor-Cousar, Christopher H Goss, George Retsch-Bogart, John Paul Clancy, Alan Genatossio, Brian P O'Sullivan, Ariel Berlinski, Susan L Millard, Gregory Omlor, Colby A Wyatt, Kathryn Moffett, David P Nichols, Alex H Gifford, Margaret Kloster, Katie Weaver, Claire Chapdu, Jing Xie, Michelle Skalland, Melita Romasco, Sonya Heltshe, Noah Simon, Jill VanDalfsen, Anna Mead, Rachael Buckingham, Kathy Seidel, Nikita Midamba, Laurel Couture, Brooke Zappone Case, Wendy Au, Elsie Rockers, Diane Cooke, Amber Olander, Irene Bondick, Miya Johnson, Lisya VanHousen, Boris Nicholson, Michelle Parrish, Dion Roberts, Jillian Head, Jessica Carey, Lindsay Caverly, Joy Dangerfield, Rachel Linnemann, Jason Fullmer, Chelsea Roman, Peter Mogayzel, Deanne Reyes, Amy Harmala, Jerimiah Lysinger, Jonathan Bergeron, Isabel Virella-Lowell, Perry Brown, Lejla Godusevic, Alicia Casey, Lauren Paquette, Thomas Lahiri, Julie Sweet, Scott Donaldson, Joshua Harris, Shelia Parnell, Sylvia Szentpetery, Deborah Froh, Erica Tharrington, Manu Jain, Rachel Nelson, Sharon Kadon, Gary McPhail, Kimberly McBennett, Tia Rone, Elliott Dasenbrook, Dave Weaver, Terri Johnson, Karen McCoy, Raksha Jain, Maria Mcleod, Mary Klosterman, Preeti Sharma, Amy Jones, Gary Mueller, Rachel Janney, Jennifer Taylor-Cousar, Mary Cross, Jordana Hoppe, James Cahill, Zubin Mukadam, Jill Finto, Karen Schultz, Silvia Delgado Villalta, Alexa Smith, Susan Millard, Thomas Symington, Gavin Graff, Diane Kitch, Don Sanders, Misty Thompson, Tahuanty Pena, Mary Teresi, Jennifer Gafford, David Schaeffer, Joel Mermis, Lawrence Scott, Hugo Escobar, Kristen Williams, Dana Dorman, Brian O'Sullivan, Ryan Bethay, Zoran Danov, Kat Turbeville, Jimmy Johannes, Angelica Rodriguez, Bridget Marra, Robert Zanni, Ronald Morton, Terri Simeon, Andrew Braun, Nicole Dondlinger, Julie Biller, Erin Hubertz, Nicholas Antos, Laura Roth, Joanne Billings, Catherine Larson, Priya Balaji, John McNamara, Tammy Clark, Rebecca Griffith, Nancy Martinez, Sabiha Hussain, Halina Malveaux, Marie Egan, Catalina Guzman, Joan DeCelie-Germana, Susan Galvin, Adrienne Savant, Nicole Falgout, Patricia Walker, Teresa Demarco, Emily DiMango, Maria Ycaza, Julie Ballo, Pornchai Tirakitsoontorn, Daniel Layish, Desiree Serr, Floyd Livingston, Sherry Wooldridge, Carlos Milla, Jacquelyn Spano, Rebecca Davis, Okan Elidemir, Subramanyam Chittivelu, Ashley Scott, Sarah Alam, Daniel Dorgan, Matt Butoryak, Daniel Weiner, Harmony Renna, Colby Wyatt, Brendan Klein, Anne Stone, Meg Lessard, Michael S. Schechter, Barbara Johnson, Steven Scofield, Theodore Liou, Jane Vroom, Kathryn Akong, Marissa Gil, Legna Betancourt, Jonathan Singer, Ngoc Ly, Courtney Moreno, Moira Aitken, Teresa Gambol, Ronald Gibson, Allison Lambert, Joan Milton, Sarah Smith, Deanna Green, Diana Hodge, Christopher Fortner, Mary Forell, Rachel Karlnoski, Kapil Patel, Cori Daines, Elizabeth Ryan, Rodolfo Amaro-Galvez, Elizabeth Dohanich, Alison Lennox, Zachary Messer, Holly Hanes, Kay Powell, and Deepika Polineni

Subjects
Pulmonary and Respiratory Medicine
Abstract

Reducing treatment burden is a priority for people with cystic fibrosis, whose health has benefited from using new modulators that substantially increase CFTR protein function. The SIMPLIFY study aimed to assess the effects of discontinuing nebulised hypertonic saline or dornase alfa in individuals using the CFTR modulator elexacaftor plus tezacaftor plus ivacaftor (ETI).The SIMPLIFY study included two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials at 80 participating clinics across the USA in the Cystic Fibrosis Therapeutics Development Network. We included individuals with cystic fibrosis aged 12-17 years with percent predicted FEVFrom Aug 25, 2020, to May 25, 2022, a total of 672 unique participants were screened for eligibility for one or both trials, resulting in 847 total random assignments across both trials with 594 unique participants. 370 participants were randomly assigned in the hypertonic saline trial and 477 in the dornase alfa trial. Participants across both trials had an average ppFEVIn individuals with cystic fibrosis on ETI with relatively well preserved pulmonary function, discontinuing daily hypertonic saline or dornase alfa for 6 weeks did not result in clinically meaningful differences in pulmonary function when compared with continuing treatment.

Published
2023

2. A Randomized Clinical Trial of Antimicrobial Duration for Cystic Fibrosis Pulmonary Exacerbation Treatment

Author

Christopher H. Goss, Sonya L. Heltshe, Natalie E. West, Michelle Skalland, Don B. Sanders, Raksha Jain, Tara L. Barto, Barbra Fogarty, Bruce C. Marshall, Donald R. VanDevanter, Patrick A. Flume, Gregory Omlor, Brenda Bourne, Dion Roberts, Vicki Roberts, Samya Nasr, Dawn Kruse, Rachel Linnemann, Tsion Hailemichael, Caralee Forseen, Heidi Stapp, Natalie West, S. Patel, A. Claudio, Jerimiah Lysinger, Amy Harmala, George Solomon, Latona Kersh, Karen Miller, Dixie Durham, Ahmet Uluer, Robert Fowler, Carla Frederick, Nadine Caci, Charlotte Teneback, Julie Sweet, Michael Parkins, Clare Smith, Jennifer Goralski, Kelsey Haywood, Patrick Flume, Caroline Brailsford, Dana Albon, Christie Aderholt, Kimberly McBennett, Cindy Schaefer, Alpa Patel, April Hunt, Lauren Schumacher, Hari Polenakovik, Linda Clark, Jerry Nick, Katie Poch, Dana Kissner, James Cahill, Jorge Lascano, Erin Silverman, John McArdle, Alison Champagne, Robert Vender, Lisa Allwein, Lance Cohen, Norma (Jean) Barton, Tara Barto, Ami Patel, Cynthia Brown, Nia Vorhees, Michael Crosser, Lawrence Scott, Alix Ashare, Barbara Rodgers, Robert Zanni, Lisa Koval, Andrew Braun, Sophia Chiron Stevens, Maria Tupayachi Ortiz, Patricia Graham, Julie Biller, Erin Hubertz, Kathryn Moffett, Tammy Clark, Rebecca Griffith, Nancy Martinez, Sabiha Hussain, Fei Chen, Marie Egan, Catalina Guzman, Janice Wang, Aileen Espinal, Patricia Walker, Anne Kukral, Emily DiMango, Sarah Fracasso Francis, Carlos Milla, Colleen Dunn, Subramanyam Chittivelu, Ashley Scott, Daniel Dorgan, Sharon Ng, Joseph Pilewski, Rose Lanzo, Nauman Chaudary, Ryan Hayden, Steven Scofield, Barb Johnson, Brian Morrissey, Brandt Robinson, Douglas Conrad, Jenna Mielke, Moira Aitken, Chami Sanlors, Ravi Nayak, Freda Branch, Daniel Rosenbluth, Molly Siegel, Anil Ghimire, Mary Forell, Cori Daines, Monica Varela, Leslie Couch, Rebekah Hibbard, Allen Dozor, Armando Ramirez, Victor Ortega, Kathryn Kennedy, David Fish, and Karen Longtine

Subjects
Adult, Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Time Factors, Cystic Fibrosis, Exacerbation, Critical Care and Intensive Care Medicine, Cystic fibrosis, law.invention, Randomized controlled trial, law, Internal medicine, medicine, Humans, Pseudomonas Infections, Respiratory system, Respiratory Tract Infections, business.industry, Cystic Fibrosis Pulmonary Exacerbation, Editorials, Respiratory infection, Original Articles, Middle Aged, medicine.disease, Antimicrobial, Anti-Bacterial Agents, Respiratory Function Tests, Clinical trial, Pseudomonas aeruginosa, Disease Progression, Female, business
Abstract

RATIONALE: People with cystic fibrosis (CF) experience acute worsening of respiratory symptoms and lung function known as pulmonary exacerbations. Treatment with intravenous antimicrobials is common; however, there is scant evidence to support a standard treatment duration. OBJECTIVES: To test differing durations of intravenous antimicrobials for CF exacerbations. METHODS: STOP2 (Standardized Treatment of Pulmonary Exacerbations 2) was a multicenter, randomized, controlled clinical trial in exacerbations among adults with CF. After 7–10 days of treatment, participants exhibiting predefined lung function and symptom improvements were randomized to 10 or 14 days’ total antimicrobial duration; all others were randomized to 14 or 21 days’ duration. MEASUREMENTS AND MAIN RESULTS: The primary outcome was percent predicted FEV(1) (ppFEV(1)) change from treatment initiation to 2 weeks after cessation. Among early responders, noninferiority of 10 days to 14 days was tested; superiority of 21 days compared with 14 days was compared for the others. Symptoms, weight, and adverse events were secondary. Among 982 randomized people, 277 met improvement criteria and were randomized to 10 or 14 days of treatment; the remaining 705 received 21 or 14 days of treatment. Mean ppFEV(1) change was 12.8 and 13.4 for 10 and 14 days, respectively, a ‒0.65 difference (95% CI [‒3.3 to 2.0]), excluding the predefined noninferiority margin. The 21- and 14-day arms experienced 3.3 and 3.4 mean ppFEV(1) changes, a difference of ‒0.10 (‒1.3 to 1.1). Secondary endpoints and sensitivity analyses were supportive. CONCLUSIONS: Among adults with CF with early treatment improvement during exacerbation, ppFEV(1) after 10 days of intravenous antimicrobials is not inferior to 14 days. For those with less improvement after one week, 21 days is not superior to 14 days. Clinical trial registered with www.clinicaltrials.gov (NCT02781610).

Published
2021

3. List of Contributors

Author

Fredrick M. Abrahamian, Michael J. Aldape, Edelweiss Aldasoro, Upton D. Allen, Hythem Al-Sum, Milan J. Anadkat, Katherine Anders, Emmanouil Angelakis, Brian John Angus, Anastasia Antoniadou, Fabio Arena, Joop E. Arends, Jose R. Arribas, Andrew W. Artenstein, John C. Atherton, John N. Aucott, Tar-Ching Aw, Hilary M. Babcock, Robin Bailey, Thomas C. Bailey, Adam Z. Banks, David J. Barillo, Ernie-Paul Barrette, Martijn P. Bauer, Roger Bayston, C. Ben Beard, Justin Beardsley, Nick J. Beeching, Rodolfo E. Bégué, Guido Beldi, Constance A. Benson, Elie F. Berbari, Jean-Michel Berenger, Christoph Berger, Jose I. Bernardino, Jacques Bille, Alexander C. Billioux, Ari Bitnun, Iain Blair, Stéphane Blanche, Thomas P. Bleck, Chantal P. Bleeker-Rovers, Gijs Bleijenberg, Karen C. Bloch, Johannes Blum, Emily A. Blumberg, Robert A. Bonomo, Marc J.M. Bonten, Rafik Bourayou, Emilio Bouza, K. Ashley Brandt, Florence Bretelle, Sylvain Brisse, Warwick J. Britton, Itzhak Brook, Matthijs C. Brouwer, Sarah K. Browne, Amy E. Bryant, Silja Bühler, Eileen M. Bulger, R. Mark L. Buller, Leah A. Burke, Christian Burri, Marcus W. Butler, Thierry Calandra, David P. Calfee, Antonia Calvo-Cano, D. William Cameron, Joseph A. Carcillo, Gail Carson, Stephen T. Chambers, Remi N. Charrel, Vinh Chau Van Nguyen, Stéphane Chevaliez, Tom M. Chiller, Eirini Christaki, Kevin K. Chung, David B. Clifford, Nathan Clumeck, Jonathan Cohen, John Collinge, Christopher P. Conlon, Curdin Conrad, Fiona J. Cooke, Jennifer Rittenhouse Cope, G. Ralph Corey, John H. Cross, Burke A. Cunha, Cheston B. Cunha, Benoit D'Journo, George L. Daikos, Johannes M.A. Daniels, Robert N. Davidson, Nicholas P.J. Day, Kevin M. De Cock, Thushan I. de Silva, Henry J.C. de Vries, Stéphane de Wit, Julie Delaloye, David W. Denning, David T. Dennis, Shireesha Dhanireddy, Elodi J. Dielubanza, David J. Diemert, Mehmet Doganay, Tom Doherty, Christiane Dolecek, Arjen M. Dondorp, Abby Douglas, Michel Drancourt, Grégory Dubourg, Michael N. Dudley, Guillaume Durand, Benjamin J. Eckhardt, Androulla Efstratiou, Miquel B. Ekkelenkamp, Ambika Eranki, Hakan Erdem, Gerome V. Escota, Heather L. Evans, Alice Chijioke Eziefula, Florence Fenollar, Alan Fenwick, Joshua Fierer, Roger G. Finch, James M. Fleckenstein, Christina Forstner, Federico Foschi, Pierre-Edouard Fournier, Martyn A. French, Kenneth L. Gage, Lynne S. Garcia, Joaquim Gascon, Arturo S. Gastañaduy, Philippe Gautret, William M. Geisler, Khalil G. Ghanem, Tommaso Giani, Maddalena Giannella, Bruce L. Gilliam, Michel Gilliet, Carol A. Glaser, Youri Glupczynski, John W. Gnann, Ellie J.C. Goldstein, Bruno Gottstein, Frederique Gouriet, Patti E. Gravitt, Michael D. Green, Stephen T. Green, Andreas H. Groll, Roy M. Gulick, Arjun Gupta, Gilbert Habib, Stephan Harbarth, Marianne Harris, Frederick G. Hayden, David J. Hetem, Philip C. Hill, Bernard Hirschel, Aimee C. Hodowanec, Louis Hoffart, Christian Hoffmann, Steven M. Holland, Peter W. Horby, David J. Horne, Sami Hraiech, Mark W. Hull, Angela Huttner, Richard J.M. Ingram, Jasmin Islam, Michael G. Ison, Scott H. James, Claire Jenkins, Stephen G. Jenkins, Jørgen Skov Jensen, Christine Johnston, Theodore B. Jones, Stephen J. Jordan, Kathleen G. Julian, Yasuyuki Kato, Carol A. Kauffman, Keith S. Kaye, Michael P. Keane, James Keeney, Paul Kelly, Stephen J. Kent, Winfried V. Kern, Yoav Keynan, Andrea A. Kim, Isabelle Koné-Paut, Chris Kosmidis, Aloys C.M. Kroes, Frank P. Kroon, Thomas G. Ksiazek, F. Matthew Kuhlmann, Ed J. Kuijper, Jennie H. Kwon, George B. Kyei, Karine Lacombe, Philippe Lagacé-Wiens, Jean-Christophe Lagier, Theresa Lamagni, Luce Landraud, Fanny Lanternier, Kerry L. LaPlante, Stephen D. Lawn, Steven J. Lawrence, Hakan Leblebicioglu, Nelson Lee, James E. Leggett, Philippe Lehours, Pierre-Yves Levy, Rainer G. Leyh, Rebecca A. Lillis, Direk Limmathurotsakul, Jennifer Lin, H.D. Alan Lindquist, Benjamin A. Lipsky, Christina Liscynesky, David Looney, Olivier Lortholary, Franklin D. Lowy, Benjamin J. Luft, Philip A. Mackowiak, Paul A. MacPherson, Valérie Maghraoui-Slim, Patrick W. Mallon, Julie E. Mangino, Oriol Manuel, Oscar Marchetti, Kristen M. Marks, Kieren A. Marr, Jeanne Marrazzo, Jonas Marschall, David H. Martin, Frédéric Matonti, Richard S. Matulewicz, Kenneth H. Mayer, Russell J. McCulloh, Rose McGready, Rennatus Mdodo, Simon Mead, Francis Mégraud, Graeme Meintjes, Sarah C. Metcalf, Marian G. Michaels, Giovanni Battista Migliori, Michael A. Miles, Alastair Miller, Matthew J. Mimiaga, Marie-Paule Mingeot-Leclercq, Elizabeth Ann Misch, Makedonka Mitreva, Julio S.G. Montaner, Caroline B. Moore, Patricia Muñoz, Jose Muñoz, Clinton K. Murray, Didier Musso, Mable Mutengo, Misha M. Mutizwa, Kurt G. Naber, Pavithra Natarajan, Santiago Neme, Paul N. Newton, Ronald A. Nichols, Lindsay E. Nicolle, François Nosten, Luigi D. Notarangelo, Thomas B. Nutman, Paul Nyirjesy, P. Ronan O'Connell, Steven M. Opal, L. Peter Ormerod, Douglas R. Osmon, Marie Boulze Pankert, Giuseppe Pantaleo, Laurent Papazian, Diane M. Parente, Philippe Parola, Shadi Parsaei, Manuel A. Pascual, Rupa Patel, Eleni Patrozou, Jean-Michel Pawlotsky, Sharon J. Peacock, Jean-Claude Pechère, Ivan Pelegrin, Barry S. Peters, Edgar J.G. Peters, Jeannine M. Petersen, Lyle R. Petersen, Vidmantas Petraitis, Luu-Ly Pham, Albert Picado, Adrian Pilatz, Benoit Pilmis, María-Jesús Pinazo, Mathias W. Pletz, Jason M. Pogue, Evelyn L. Polgreen, Philip M. Polgreen, Klara M. Posfay-Barbe, William G. Powderly, Rachel Presti, Guy Prod'hom, Mirja Puolakkainen, Thomas C. Quinn, Didier Raoult, Raymund R. Razonable, Robert C. Read, Robert R. Redfield, Rob J. Rentenaar, Steven J. Reynolds, Camillo Ribi, Malcolm D. Richardson, Michele L. Ritter, Antoine Roch, Jürgen Kurt Rockstroh, Amanda Rojek, José R. Romero, Suzan H.M. Rooijakkers, Daniel Rosenbluth, Sergio D. Rosenzweig, Gian Maria Rossolini, Ethan Rubinstein, Greg Ryan, Steven A. Safren, Vikrant V. Sahasrabuddhe, Pekka A.I. Saikku, Mohammad M. Sajadi, Michelle R. Salvaggio, Carlos A.Q. Santos, Michael J. Satlin, Anthony J. Schaeffer, Christoph Schimmer, Robert T. Schooley, Richard F. Schumacher, Beverly E. Sha, Daniel S. Shapiro, Gerard Sheehan, David M. Shlaes, Shmuel Shoham, Cameron P. Simmons, Dennis W. Simon, Matthew S. Simon, Kari A. Simonsen, Mary P.E. Slack, Tyrel T. Smith, Jack D. Sobel, Maria Souli, Shruti Sridhar, James M. Steckelberg, Dennis L. Stevens, Heather Strah, A. Willem Sturm, Somnuek Sungkanuparph, Sarah J. Tabrizi, Evelina Tacconelli, Chen Sabrina Tan, Randy A. Taplitz, Guillemette Thomas, Lora D. Thomas, Franck Thuny, Guy Thwaites, Frederic Tissot, Tone Tønjum, Francesca J. Torriani, Christian Toso, Paul M. Tulkens, Allan R. Tunkel, Claire E. Turner, Andrew P. Ustianowski, Françoise van Bambeke, Reinout van Crevel, Diederik van de Beek, Christian van Delden, Menno M. van der Eerden, Jos W.M. van der Meer, Tom van der Poll, Jakko van Ingen, Jos van Putten, Bernard P. Vaudaux, Sten H. Vermund, Raphael P. Viscidi, Kumar Visvanathan, Govinda S. Visvesvara, Lorenz von Seidlein, Florian M.E. Wagenlehner, Anna Wald, Thomas J. Walsh, David C. Warhurst, David W. Warnock, David A. Warrell, Mary J. Warrell, Adilia Warris, Richard R. Watkins, David J. Weatherall, Rainer Weber, Wolfgang Weidner, Jonathan R. White, Peter J. White, James Whitehorn, Richard J. Whitley, Christopher J.M. Whitty, Willem Joost Wiersinga, Mark H. Wilcox, Thomas N. Williams, Cara C. Wilson, Mary Elizabeth Wilson, Hilmar Wisplinghoff, Robin Wood, Richard G. Wunderink, David Wyles, Zhi-Tao Yang, Jonathan S. Yoder, Najam A. Zaidi, Andrea J. Zimmer, Jane N. Zuckerman, and Alimuddin Zumla

Published
2017

5. Cystic Fibrosis Pulmonary Guidelines

Author

Randall L. Rosenblatt, Daniel Rosenbluth, Marcia Katz, Henry L. Dorkin, Patrick A. Flume, Moira L. Aitken, Michelle S. Howenstine, Manu Jain, David C. McGiffin, Joseph M. Pilewski, Ahmet Uluer, Craig W. Lillehei, Beryl J. Rosenstein, Hector H. Gutierrez, Terry B. White, James D. Acton, Randall K. Young, Thomas M. Egan, Kathryn A. Sabadosa, Steven Strausbaugh, Mary K Lester, Sally E. Mitchell, Douglas S. Holsclaw, Bruce C. Marshall, Mike Mulligan, David M. Orenstein, Donna Beth Willey-Courand, Peter J. Mogayzel, Susanna A. McColley, Souheil Saddekni, Melissa Chin, Lynne M. Quittell, Paul Mohabir, Christopher M. Oermann, James R. Yankaskas, Michael P. Boyle, Robert J. Kuhn, R. Duane Davis, Jill Fleige, Michael J. Rock, Elizabeth Tullis, Anne M Downs, James L. Cunningham, Peter J Murphy, John L. Colombo, Linda L. Wolfenden, Thomas W. Ferkol, Patricia M. Joseph, Jeffrey S. Wagener, George Z. Retsch-Bogart, Christopher H. Goss, Karen A. Robinson, Carlos Milla, Felix Ratjen, Michael S. Schechter, Patricia E. Burrows, Aruna Sannuti, Charles B. Huddleston, Robert L. Vender, Leslie Hazle, Mark J. Sands, Charles T. Burke, Wickii T. Vigneswaran, Ronald C. Rubenstein, Guillermo A. doPico, Jerry A. Nick, Wyn Hoover, and Mark H. Wholey

Subjects
Pulmonary and Respiratory Medicine, Spirometry, Hemoptysis, medicine.medical_specialty, Aircraft, Cystic Fibrosis, Delphi Technique, Weight Lifting, medicine.medical_treatment, Decision Making, Bronchial Arteries, Critical Care and Intensive Care Medicine, Severity of Illness Index, Cystic fibrosis, Positive-Pressure Respiration, Bronchoscopy, Intensive care, Administration, Inhalation, Pulmonary fibrosis, Secondary Prevention, medicine, Humans, Lung transplantation, Intensive care medicine, Lung, Pleurodesis, Saline Solution, Hypertonic, Travel, medicine.diagnostic_test, business.industry, Patient Selection, Anti-Inflammatory Agents, Non-Steroidal, Respiratory disease, Pneumothorax, medicine.disease, Embolization, Therapeutic, Anti-Bacterial Agents, Hospitalization, Chest Tubes, business, Lung Transplantation
Abstract

Cystic fibrosis (CF) is a recessive genetic disease characterized by dehydration of the airway surface liquid and impaired mucociliary clearance. As a result, individuals with the disease have difficulty clearing pathogens from the lung and experience chronic pulmonary infections and inflammation. There may be intermittent pulmonary exacerbations or acute worsening of infection and obstruction, which require more intensive therapies. Hemoptysis and pneumothorax are complications commonly reported in patients with cystic fibrosis.This document presents the CF Foundation's Pulmonary Therapies Committee recommendations for the treatment of hemoptysis and pneumothorax.The committee recognized that insufficient data exist to develop evidence-based recommendations and so used the Delphi technique to formalize an expert panel's consensus process and develop explicit care recommendations.The expert panel completed the survey twice, allowing refinement of recommendations. Numeric responses to the questions were summarized and applied to a priori definitions to determine levels of consensus. Recommendations were then developed to practical treatment questions based upon the median scores and the degree of consensus.These recommendations for the management of the patient with CF with hemoptysis and pneumothorax are designed for general use in most individuals but should be adapted to meet specific needs as determined by the individuals, their families, and their health care providers. It is hoped that the guidelines provided in this manuscript will facilitate the appropriate application of these treatments to improve and extend the lives of all individuals with cystic fibrosis.

Published
2010

6. Forced Expiratory Volume in 1 Second Variability Helps Identify Patients with Cystic Fibrosis at Risk of Greater Loss of Lung Function

Author

Wayne J. Morgan, Donald R. VanDevanter, David J. Pasta, Aimee J. Foreman, Jeffrey S. Wagener, Michael W. Konstan, Wayne Morgan, Michael Konstan, Ted Liou, Susanna McColley, Ann McMullen, Alexandra Quittner, Warren Regelmann, Clement Ren, Margaret Rosenfeld, Greg Sawicki, Michael Schechter, Donald VanDevanter, Jeffrey Wagener, Marlyn Woo, Dana Brasfield, Raymond Lyrene, Lawrence Sindel, Dion Roberts, John Carroll, Robert Warren, Louay Nassri, Paula Anderson, Mark Brown, Amy Silverthorn, Peggy Radford, Gerald Gong, Gregory Legris, Gerald Greene, Reddivalam Sudhakar, Arnold Platzker, Bruce Nickerson, Karen Hardy, Ivan Harwood, Gregory Shay, Bryon Quick, Allan Lieberthal, Richard Moss, Chris Landon, Yvonne Fanous, Jay Lieberman, Eugene Spiritus, Bradley Chipps, Ruth McDonald, Mark Pian, Gerd Cropp, Nancy Lewis, Dennis Nielson, Bertrand Shapiro, Jeff Wagener, Frank Accurso, Milene Saavedra, Karen Daigle, Jacob Hen, Regina Palazzo, Kathryn Dodds, Raj Pad-man, John Goodill, Glenna Winnie, Lea Davies, Tony Kriseman, Jorge Sallent, Joseph Chiaro, Martin Kubiet, Sue Goldfinger, Morton Schwartzman, Carlosenrique Diaz, Kevin Maupin, Eduardo Riff, David Geller, Floyd Livingston, Kunjana Mavunda, Jose Birriel, Luis Faverio, David Rosenberg, David Schaeffer, James Sherman, Mary Wagner, Michael Light, Bruce Schnapf, Gary Montgomery, Kevin Kirchner, Mark Weatherly, Daniel Caplan, Margaret Guill, Valera Hudson, Javeed Akhter, Donald Davison, Steven Boas, Youngran Chung, Rennee Latner, Gabriel Aljadeff, Youngran Chan, Jerome Kraut, Arvey Stone, John Lloyd Still, Girish Sharma, Lanie Eagleton, Patricia Hopkins, Umesh Chatrath, Lucille Lester, Young-Jee Kim, Veena Anthony, Howard Eigan, Michelle Howenstine, Pushpom James, Edward Gergesha, James Harris, Robert Plant, Veljko Zivkovich, Angela Collins, Edward Nassif, Richard Ahrens, Daniel Doornbos, Joseph Kanarek, Richard Leff, Pamela Shaw, Elanor Demoss, Maria Riva, Leonard Sullivan, Michael Anstead, Jamshed Kanga, Nemr Eid, Ron Morton, Bettina Hilman, Kim Jones, Scott Davis, Ralph Harder, Tom Lever, Anne Marie Cairns, Edgar Caldwell, Jonathan Zuckerman, Peter Mogayzel, Beryl Rosenstein, John McQuestion, Donna Perry, Samuel Rosenberg, Robert Gerstle, Andrew Colin, Mary Ellen Wohl, Allan Lapey, William Yee, Brian O'Sullivan, Robert Zwerdling, Ibrahim Abdulhamid, Adrian O'Hagan, John Schuen, Lawrence Kurlandsky, Richard Honicky, Douglas Homnick, John Marks, Bohdan Pichurko, Norma Maxvold, Samya Nasr, Richard Simon, Wan Tsai, Dana Kissner, John McNamara, Nancy Henry, Stephen Marker, Michael Pryor, Lynn Walker, Jim Woodward, Louis Mizell, Suzanne Miller, Daniel Rosenbluth, Philip Black, Michael McCubbin, Alan Cohen, Thomas Ferkol, George Mallory, Anthony Rejent, Bruce Rubin, Gavin Graff, Peter Konig, John Colombo, Peter Murphy, William Boyle, Worth Parker, Chandler Patton, Robert Zanni, Arthur Atlas, Nelson Turcios, Lourdes Laraya-Cuasay, Dorothy Bisberg, Helen Aguila, Sarah Allen, David James, Elizabeth Perkett, Marsha Thompson, Sonia Budhecha, Ruben Diaz, Jonathan Rosen, Robert Kaslovsky, Ronald Percciacante, Drucy Borowitz, Joseph Cronin, Colin McMahon, Lynne Quittell, Robert Giusti, Rubin Cohen, Joan DeCelie-Germana, Jack Gorvoy, Kalpan Patel, Meyer Kattan, Allen Dozor, Emily DiMango, Maria Berdella, Ran Anbar, Debra Ianuzzi, James Sexton, Catherine Tayag-Kier, John McBride, Karen Voter, Mary Dimaio, John Georgitis, Joseph Marc Majure, Maria Martinez, Clarke McIntosh, Margaret Leigh, Hugh Black, James Hughes, Anand Kantak, Robert Wilmott, Gregory Omlor, Robert Stone, Karen McCoy, James Acton, Carl Doershuk, Robert Fink, Michael Steffan, Pierre Vauthy, Patricia Joseph, Santiago Reyes, John Kramer, James Royall, Jay Eisenberg, Michael Wall, Stanley Fiel, Thomas Scanlin, Shroti Phadke, Joel Weinberg, William Sexauer, Stephen Wolf, Douglas Holsclaw, Debra Klein, Stuart Warren, Robert Kinsey, Carlos Perez, Muttiah Ganeshanathan, James Shinnick, Howard Panitch, Laurie Varlotta, Cynthia Robinson, Jose Rodriguez Santana, Mary Ann Passero, Jane Gwinn, Robert Baker, Michael Bowman, Patrick Flume, Daniel Brown, Roxanne Marville, James Wallace, Rodney Parry, Don Ellenburg, John Rogers, Ricky Mohon, Joel Ledbetter, Aram Hanissian, Robert Schoumacher, Preston Campbell, Christopher Harris, Bonnie Slovis, Dennis Stokes, Kathryn Hale, Marcia Katz, Dan Seilheimer, Marianne Sockrider, Allan Frank, James Daniel, James Cunningham, Iley Browning, John Bray, Amanda Dove, Fernando Mandujano, Larry Tremper, Martha Morse, Donna Willey-Courand, Steven Copenhaver, John Pohl, Bennie McWilliams, Marie Martine-Logvinoff, Marsh Wallace, Robert Klein, Rodolfo Amaro, Leslie Couch, Michael Brown, Claude Prestidge, Stephen Inscore, Andrew Lipton, Barbara Chatfield, Theodore Liou, Bruce Marshall, Thomas Lahiri, Donald Swartz, Laurie Whittaker, Karl Karlson, Ignacio Ropoll, Thomas Rubio, Joel Schmidt, David Thomas, John Osborn, Deborah Froh, Benjamin Gaston, Greg Elliott, Ronald Gibson, Bonnie Ramsey, Michael McCarthy, Lawrence Larson, David Ricker, Mark Robbins, Moira Aitken, Julia Emerson, Stephen Aronoff, Kathryn Moffett, Julie Biller, Mark Splaingard, Bradley Sullivan, Paul Pritchard, Stu Adair, Peter Holzwarth, Guillermo Dopico, Keith Meyer, Christopher Green, and Michael Rock

Subjects
Male, medicine.medical_specialty, Epidemiologic study, Time Factors, Cystic Fibrosis, Cystic fibrosis, Risk Assessment, 03 medical and health sciences, 0302 clinical medicine, Predictive Value of Tests, Internal medicine, Forced Expiratory Volume, medicine, Humans, In patient, 030212 general & internal medicine, Prospective Studies, Prospective cohort study, Child, Lung, Lung function, business.industry, respiratory system, medicine.disease, respiratory tract diseases, 030228 respiratory system, Multicenter study, Predictive value of tests, Pediatrics, Perinatology and Child Health, Cardiology, Physical therapy, Female, business, Risk assessment, circulatory and respiratory physiology
Abstract

Objective To evaluate several alternative measures of forced expiratory volume in 1 second percent predicted (FEV 1 %pred) variability as potential predictors of future FEV 1 %pred decline in patients with cystic fibrosis. Study design We included 13 827 patients age ≥6 years from the Epidemiologic Study of Cystic Fibrosis 1994-2002 with ≥4 FEV 1 %pred measurements spanning ≥366 days in both a 2-year baseline period and a 2-year follow-up period. We predicted change from best baseline FEV 1 %pred to best follow-up FEV 1 %pred and change from baseline to best in the second follow-up year by using multivariable regression stratified by 4 lung-disease stages. We assessed 5 measures of variability (some as deviations from the best and some as deviations from the trend line) both alone and after controlling for demographic and clinical factors and for the slope and level of FEV 1 %pred. Results All 5 measures of FEV 1 %pred variability were predictive, but the strongest predictor was median deviation from the best FEV 1 %pred in the baseline period. The contribution to explanatory power (R 2 ) was substantial and exceeded the total contribution of all other factors excluding the FEV 1 %pred rate of decline. Adding the other variability measures provided minimal additional value. Conclusions Median deviation from the best FEV 1 %pred is a simple metric that markedly improves prediction of FEV 1 %pred decline even after the inclusion of demographic and clinical characteristics and the FEV 1 %pred rate of decline. The routine calculation of this variability measure could allow clinicians to better identify patients at risk and therefore in need of increased intervention.

Published
2014

7. Effects of puberty on cystic fibrosis related pulmonary exacerbations in women versus men

Author

Shelby, Sutton, Daniel, Rosenbluth, Deepa, Raghavan, Jie, Zheng, and Raksha, Jain

Subjects
Male, Sex Factors, Adolescent, Cystic Fibrosis, Puberty, Humans, Female, Child, Gonadal Steroid Hormones, Lung, Article
Abstract

Epidemiologic data from studies of airway diseases, such as asthma, chronic obstructive pulmonary disease, and cystic fibrosis indicate a gender disparity where women have worse outcomes. The explanation for this is largely unknown. We hypothesize that female sex hormones play a role in this gender disparity, predisposing women to more exacerbations and decreased lung function post-puberty.In Cystic Fibrosis, to determine if puberty marks a point of increasing exacerbations and decreasing lung function in women relative to men.Using the United States Cystic Fibrosis Foundation Patient Registry, we used linear regression to compare lung function and rate of pulmonary exacerbations in men versus women before and after puberty.Of 5,137 subjects who met inclusion criteria, 2,689 were male and 2,448 were female. Average age of puberty was found to be 13.2 ± 2.2 years in men and 11.2 ± 2.0 years of age in women. Percent predicted FEV1 pre- and post-puberty were no different between males versus females (P = 0.44 pre-puberty and P = 0.16 post-puberty). In contrast, women had a significantly higher rate of pulmonary exacerbations post-puberty than men (1.17 ± 1.35 exacerbations per year in women versus 0.95 ± 1.27 in men; P0.001) despite controlling for morphometrics, co-morbidities, and microbiologic variables.After puberty, the rate of pulmonary exacerbations increased in adolescent women relative to men with cystic fibrosis, supporting a role for sex hormones in the disease process. Further understanding of the mechanisms that modulate sex hormone receptors in airway disease may serve as future targets for therapy.

Published
2012

8. Contributors

Author

George J. Alangaden, Michael J. Aldape, Jérôme Allardet-Servent, Upton D. Allen, Heidi S.M. Ammerlaan, Emmanouil Angelakis, Andrew Artenstein, David Asboe, Kingsley B. Asiedu, John C. Atherton, Tar-Ching Aw, Seema Baid-Agrawal, Robin Bailey, Christopher Bandel, Philip S. Barie, David J. Barillo, Pierre-Alexandre Bart, Roger Bayston, C. Ben Beard, Nick J. Beeching, Rodolfo E. Bégué, Yves Benhamou, Constance A. Benson, Elie F. Berbari, Anthony R. Berendt, Madhav P. Bhatta, Jacques Bille, Ari Bitnun, Finn T. Black, Iain Blair, Stéphane Blanche, Thomas P. Bleck, Chantal P. Bleeker-Rovers, Gijs Bleijenberg, Karen C. Bloch, Marc J.M. Bonten, Charles A.B. Boucher, Rafik Bourayou, Emilio S. Bouza, William R. Bowie, Barry D. Brause, Sylvain Brisse, Warwick Britton, Itzhak Brook, David W.G. Brown, Christian Brun-Buisson, James C.M. Brust, Amy E. Bryant, André Bryskier, R. Mark L Buller, Karen Bush, Thierry Calandra, D. William Cameron, Michel Caraël, Michael J. Carr, Inmaculada Casas, Stephen T. Chambers, Katarina G. Chiller, Tom M. Chiller, Peter L. Chiodini, Ian Chopra, Anthony C. Chu, Kevin K. Chung, Benjamin M. Clark, Nathan Clumeck, Clay J. Cockerell, Jonathan Cohen, John Collinge, Christopher P. Conlon, G. Ralph Corey, Alan Cross, John H. Cross, Judith Currier, Carmel M. Curtis, Gina Dallabetta, Robert N. Davidson, Jane Davies, Jeremy Day, Nicholas P.J. Day, Cillian F. De Gascun, Stéphane de Wit, Jean Delmont, David T. Dennis, David J. Diemert, Mehmet Doganay, Tom Doherty, Christiane Dolecek, Stéphane Y. Donati, Arjen M. Dondorp, Barbara Doudier, Michel Drancourt, Dimitri M. Drekonja, Richard H. Drew, Jay S. Duker, J. Stephen Dummer, Charles N. Edwards, Miquel B. Ekkelenkamp, Mark C. Enright, Paul R. Epstein, Veronique Erard, Alice Chijioke Eziefula, Mark B. Feinberg, Florence Fenollar, Alan Fenwick, Luis Fernandez, Joshua Fierer, Roger G. Finch, Charles W. Flexner, Ad C. Fluit, Elizabeth Lee Ford-Jones, Pierre-Edouard Fournier, Victoria Fraser, Martyn A. French, Jon S. Friedland, Joseph M. Fritz, E. Yoko Furuya, Kenneth L. Gage, Lynne S. Garcia, Arturo S. Gastañaduy, Khalil G. Ghanem, Maddalena Giannella, Carol A. Glaser, Marshall J. Glesby, Sarah Glover, Youri Glupczynski, John W. Gnann, Andrew F. Goddard, Ellie J.C. Goldstein, Iveth J. González, Sherwood L. Gorbach, Bruno Gottstein, Ravi Gowda, John D. Grabenstein, John M. Grange, Michael D. Green, Stephen T. Green, Danielle T. Greenblatt, Brian Greenwood, Aric L. Gregson, Andreas H. Groll, Aditya K. Gupta, Kok-Ann Gwee, William Hall, Scott M. Hammer, Sajeev Handa, Diane Hanfelt-Goade, Alexandre Harari, Marianne Harris, Barry J. Hartman, Roderick J. Hay, David K. Henderson, Lisa E. Hensley, Luke Herbert, David R. Hill, Timothy J. Hills, John David Hinze, Hans H. Hirsch, Bernard Hirschel, Andy I.M. Hoepelman, Steven M. Holland, Mary M. Horgan, Robin Howe, James M. Hughes, Mark W. Hull, Clark B. Inderlied, Michael G. Ison, Peter J. Jenks, James R. Johnson, Theodore Jones, Mettassebia Kanno, Carol Kauffman, Patrick Kelly, Jason S. Kendler, Yoav Keynan, Ali S. Khan, Grace T. Kho, George R. Kinghorn, Paul E. Klapper, Jan AJW Kluytmans, Menno Kok, Isabelle Koné-Paut, John N. Krieger, Aloys C.M. Kroes, Frank P. Kroon, Christine J. Kubin, Alberto M. La Rosa, Tahaniyat Lalani, David G. Lalloo, Harold Lambert, Luce Landraud, Stephen D. Lawn, Phillipe Lehours Pharm, Marc Leone, Itzchak Levi, Alexandra M. Levitt, H. D. Alan Lindquist, Graham Lloyd, David J. Looney, Franklin D. Lowy, Benjamin J. Luft, William A. Lynn, Mark J. Macielag, Philip A. Mackowiak, Paul A. MacPherson, Valérie Maghraoui-Slim, Janice Main, Vincent Mallet, Julie E. Mangino, Oriol Manuel, Oscar Marchetti, Kristen Marks, Kieren A. Marr, Claude Martin, Pablo Martín-Rabadán, Augusto Julio Martinez, Ellen M. Mascini, Kenneth H. Mayer, Joseph B. McCormick, Rose McGready, Michael W. McKendrick, Simon Mead, Francis Mégraud, André Z. Meheus, Graeme Meintjes, Marian G. Michaels, Michael Miles, Alastair Miller, Matthew J. Mimiaga, Marie-Paule Mingeot-Leclercq, Thomas G. Mitchell, Pamela A. Moise, Julio Montaner, Caroline B. Moore, Philippe Moreillon, Peter Morgan-Capner, Valentina Montessori, Peter Moss, Patricia Muñoz, Kurt G. Naber, Sammy Nakhla, Jai P. Narain, Dilip Nathwani, Paul Newton, Chinh Nguyen, Lindsay E. Nicolle, Michael S. Niederman, Gary J. Noel, S. Ragnar Norrby, François Nosten, Luigi Daniele Notarangelo, Paul Nyirjesy, P. Ronan O'Connell, Jon S. Odorico, Edmund L.C. Ong, Steven M. Opal, L. Peter Ormerod, Douglas R. Osmon, Eric A. Ottesen, Gustavo Palacios, Giuseppe Pantaleo, Laurent Papazian, Philippe Parola, Manuel A. Pascual, Eleni Patrozou, Carlos Paya, Sharon J. Peacock, Jean-Claude Pechère, Mark D. Perkins, Barry Peters, Gaby E. Pfyffer, Paul A. Pham, Peter Piot, Geraldine Placko-Parola, Stainslas Pol, Klara M. Posfay-Barbe, William G. Powderly, Anton Pozniak, Guy Prod'hom, Thomas C. Quinn, Daniel W. Rahn, Aadia I. Rana, Didier Raoult, Raul Raz, Raymund Razonable, Robert C. Read, Stephen J. Reynolds, Malcolm D. Richardson, Christopher C. Robinson, Suzan H.M. Rooijakkers, Daniel Rosenbluth, Sergio D. Rosenzweig, Clarisse Rovery, Robert H. Rubin, Bina Rubinovitch, Kathleen H. Rubins, Ethan Rubinstein, Greg Ryan, Stephen Ryder, Steven Safren, Vikrant V. Sahasrabuddhe, Pekka A.I. Saikku, George Sakoulas, Juan Carlos Salazar, Michelle R. Salvaggio, Kirsten Schaffer, Franz-Josef Schmitz, Robert T. Schooley, Richard-Fabian Schumacher, Euan M. Scrimgeour, James Seddon, Harald Seifert, Graham R. Serjeant, Beverly E. Sha, Keerti V. Shah, Daniel S. Shapiro, Gerard Sheehan, Shmuel Shoham, Cameron P. Simmons, Kari A. Simonsen, Neeraj Singh, Mary P.E. Slack, Jack D. Sobel, Madhuri M. Sopirala, Lisa A. Spacek, Shiranee Sriskandan, Samuel L. Stanley, James M. Steckelberg, Iain Stephenson, Dennis L. Stevens, Walter L. Straus, Willem Sturm, Richard C. Summerbell, Joseph S. Susa, Sarah J. Tabrizi, Marc A. Tack, Randy Taplitz, Pablo Tebas, Marleen Temmerman, Steven F.T. Thijsen, Lora D. Thomas, Gail Thomson, Guy E. Thwaites, Umberto Tirelli, Nina E. Tolkoff-Rubin, Tone Tønjum, Francesca J. Torriani, Gregory C. Townsend, Gloria Trallero Masó, Paul M. Tulkens, Allan R. Tunkel, Emanuela Vaccher, Anaïs Vallet-Pichard, Françoise Van Bambeke, Diederik van de Beek, Jos W.M. van der Meer, Anton M. van Loon, Jos van Putten, Bernard P. Vaudaux, Sten H. Vermund, Hans Verstraelen, Paul Verweij, Raphael P. Viscidi, Kumar Visvanathan, Govinda S. Visvesvara, Lorenz von Seidlein, Florian M.E. Wagenlehner, Victoria Wahl-Jensen, Thomas J. Walsh, David C. Warhurst, David W. Warnock, David A. Warrell, Mary J. Warrell, Adilia Warris, Rainer Weber, Wolfgang Weidner, Vivienne C. Weston, Estella Whimbey, Michael Whitby, Peter J. White, Christopher J.M. Whitty, Rob J.L. Willems, Emrys Williams, Cara Wilson, Mary E. Wilson, Richard E. Winn, Kevin L. Winthrop, Martin J. Wiselka, Hilmar Wisplinghoff, Cameron R. Wolfe, Robin Wood, Natalie Wright, James R. Yankaskas, Najam A. Zaidi, Jonathan M. Zenilman, Yaobi Zhang, Arie J. Zuckerman, Jane Nicola Zuckerman, and Alimuddin Zumla

Published
2010

10. Systemic lupus erythematosus patients with central nervous system involvement show autoantibodies to a 50-kD neuronal membrane protein

Author

Saul Puszkin, Daniel Rosenbluth, Harry Spiera, Veneta Hanson, and Mark D. Horowitz

Subjects
Adult, Male, Pathology, medicine.medical_specialty, Adolescent, Blotting, Western, Immunology, Synaptic Membranes, Fluorescent Antibody Technique, Connective tissue, Nerve Tissue Proteins, Pathogenesis, Antigen, Central Nervous System Diseases, medicine, Animals, Humans, Lupus Erythematosus, Systemic, Immunology and Allergy, skin and connective tissue diseases, Aged, Autoantibodies, Neurons, Lupus erythematosus, biology, Autoantibody, Membrane Proteins, Articles, medicine.disease, Connective tissue disease, medicine.anatomical_structure, Membrane protein, biology.protein, Cattle, Female, Antibody
Abstract

An antibody was detected in the sera from patients with systemic lupus erythematosus (SLE) and central nervous system (CNS) involvement that reacted with a 50-kD antigen in the plasma membrane of brain synaptic terminals. The 50-kD antigen was solubilized with Triton X-100 from preparations enriched with synaptic plasma membranes, and was partially purified by molecular sieve filtration column chromatography. The sera of 19 of 20 CNS-SLE patients showed strong to moderate immunoreactivity with the 50-kD protein in Western blots. Immunoreactivity with the 50-kD protein was also detected in the cerebrospinal fluid of CNS-SLE patients. Control sera from healthy individuals did not react with the 50-kD protein. Low to background reactivity was detected in 35% of a group of SLE patients without CNS manifestations, and in 3% of patients displaying other connective tissue diseases. A total of 100 individuals were tested in this study. Purified autoantibodies to the 50-kD protein from CNS-SLE patients were used for immunofluorescent labeling of neuroblastoma cells. The immunofluorescent staining revealed a distinct macular distribution pattern on the surface of the cell membrane. Taken together, the data suggest that the 50-kD protein may be an important target for autoantibodies, preponderantly found in CNS-SLE patients, and that the antigen may play a role in the pathogenesis of some neurological manifestations in SLE.

Published
1992

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