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1. Complications and Practice Variation in the Use of Peripherally Inserted Central Venous Catheters in People With Cystic Fibrosis: The Prospective Study of Peripherally Inserted Venous Catheters in People With Cystic Fibrosis Study

Author

Gifford, Alex H., Hinton, Alexandra C., Jia, Shijing, Nasr, Samya Z., Mermis, Joel D., Lahiri, Thomas, Zemanick, Edith T., Teneback, Charlotte C., Flume, Patrick A., DiMango, Emily A., Sadeghi, Hossein, Polineni, Deepika, Dezube, Rebecca H., West, Natalie E., Dasenbrook, Elliott C., Lucas, F. Lee, and Zuckerman, Jonathan B.

Published
2023
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9. Cystic Fibrosis Foundation Evidence-Based Guideline for the Management of CRMS/CFSPID.

Author

Green, Deanna M., Lahiri, Thomas, Raraigh, Karen S., Ruiz, Fadel, Spano, Jacquelyn, Antos, Nicholas, Bonitz, Lynn, Christon, Lillian, Gregoire-Bottex, Myrtha, Hale, Jaime E., Langfelder-Schwind, Elinor, La Parra Perez, Álvaro, Maguiness, Karen, Massie, John, McElroy-Barker, Erin, McGarry, Meghan E., Mercier, Angelique, Munck, Anne, Oliver, Kathryn E., and Self, Staci

Subjects
*CYSTIC fibrosis diagnosis, *CYSTIC fibrosis treatment, *METABOLIC syndrome diagnosis, *MEDICAL protocols, *SOCIAL determinants of health, *RESEARCH funding, *GENETIC counseling, *EVIDENCE-based medicine, *MEDICAL screening, *CYSTIC fibrosis, *GENETIC testing
Abstract

A multidisciplinary committee developed evidence-based guidelines for the management of cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen-positive, inconclusive diagnosis (CRMS/CFSPID). A total of 24 patient, intervention, comparison, and outcome questions were generated based on surveys sent to people with CRMS/CFSPID and clinicians caring for these individuals, previous recommendations, and expert committee input. Four a priori working groups (genetic testing, monitoring, treatment, and psychosocial/communication issues) were used to provide structure to the committee. A systematic review of the evidence was conducted, and found numerous case series and cohort studies, but no randomized clinical trials. A total of 30 recommendations were graded using the US Preventive Services Task Force methodology. Recommendations that received $80% consensus among the entire committee were approved. The resulting recommendations were of moderate to low certainty for the majority of the statements because of the low quality of the evidence. Highlights of the recommendations include thorough evaluation with genetic sequencing, deletion/duplication analysis if <2 disease-causing variants were noted in newborn screening; repeat sweat testing until at least age 8 but limiting further laboratory testing, including microbiology, radiology, and pulmonary function testing; minimal use of medications, which when suggested, should lead to shared decision-making with families; and providing communication with emphasis on social determinants of health and shared decision-making to minimize barriers which may affect processing and understanding of this complex designation. Future research will be needed regarding medication use, antibiotic therapy, and the use of chest imaging for monitoring the development of lung disease. [ABSTRACT FROM AUTHOR]

Published
2024
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11. Efficacy and safety of lumacaftor and ivacaftor in patients aged 6–11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial

Author

Rosenfeld, Margaret, Starner, Timothy, Retsch-Bogart, George, Chmiel, James, Orenstein, David, Milla, Carlos, Rubenstein, Ronald, Walker, Seth, Cornell, Alexandra, Asfour, Fadi, Black, Philip, Colombo, John, Froh, Deborah, McColley, Susanna, Ruiz, Fadel, Quintero, Diana, Casey, Alicia, Mueller, Gary, Flume, Patrick, Livingston, Floyd, Rock, Michael, O'Sullivan, Brian, Schmidt, Howard, Lahiri, Thomas, McNamara, John, Chidekel, Aaron, Sass, Laura, Keens, Thomas, Schaeffer, David, Solomon, Melinda, Chilvers, Mark, Lands, Larry, Junge, Sibylle, Griese, Matthias, Staab, Doris, Pressler, Tacjana, van Koningsburggen-Rietschel, Silke, Naehrlich, Lutz, Reid, Alastair, Balfour-Lynn, Ian, Urquhart, Don, Lee, Timothy, Munck, Anne, Gaudelus, Isabelle Sermet, De Boeck, Christiane, Reix, Philippe, Malfroot, Anne, Bui, Stéphanie, Selvadurai, Hiranjan, Robinson, Philip, Wainwright, Claire, Clements, Barry, Hilton, Jodi, Hjelte, Lena, Ratjen, Felix, Hug, Christopher, Marigowda, Gautham, Tian, Simon, Huang, Xiaohong, Stanojevic, Sanja, Milla, Carlos E, Robinson, Paul D, Waltz, David, and Davies, Jane C

Published
2017
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12. 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

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

Published
2023
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13. Forced Expiratory Volume in 1 Second Variability Helps Identify Patients with Cystic Fibrosis at Risk of Greater Loss of Lung Function

Author

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

Published
2016
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14. Monitoring Oxygen Levels of Premature Babies at Home and in the Clinic—The RHO Trial

Author

Rhein, Lawrence, primary, White, Heather, additional, Simoneau, Tregony, additional, Traeger, Nadav, additional, Lahiri, Thomas, additional, Kremer, Ted, additional, Sheils, Catherine, additional, Meyer, Kathleen, additional, Rosenkrantz, Ted, additional, Sankaran, Krishnan, additional, Hartman, Tyler, additional, Feldman, Henry, additional, and Abu Jawdeh, Elie, additional

Published
2020
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23. Changing epidemiology of life-threatening upper airway infections: the reemergence of bacterial tracheitis

Author

Hopkins, Amelia, Lahiri, Thomas, Salerno, Richard, and Heath, Barry

Subjects
Respiratory tract infections -- Diagnosis, Respiratory tract infections -- Development and progression, Tracheitis -- Diagnosis, Tracheitis -- Development and progression, Prevalence studies (Epidemiology)
Abstract

OBJECTIVE. As a consequence of evolving medical practice, the epidemiology of potentially life-threatening upper airway infections is changing. We report our experience over 9 years with viral croup, epiglottitis, and bacterial tracheitis. PATIENTS AND METHODS. We studied a retrospective case series of patients admitted to Vermont Children&apos;s Hospital with potentially life-threatening upper airway infections viral croup, epiglottitis, or bacterial tracheitis between 1997 and 2006. MEASUREMENT AND MAIN RESULTS. There were 107 patients with viral croup admitted to Vermont Children&apos;s Hospital with 16 (15%) admitted to the pediatric intensive care unit. Three patients with croup (17% of pediatric intensive care unit admissions, 3% of total admissions) required intubation. There were no serious complications. Eighteen patients were admitted with bacterial tracheitis. Ninety-four percent (n = 17) were admitted to the pediatric intensive care unit. Eighty-three percent (n = 15) were intubated. Twenty-eight percent of patients (n = 5) developed serious complications. Two adolescent patients were admitted with epiglottitis. Both were intubated and recovered without complications. Of 35 patients admitted to the pediatric intensive care unit with these potentially life-threatening upper airway infections, 20 patients (57%) developed respiratory failure. Fifteen patients (75%) had bacterial tracheitis, 3 patients (15%) had viral croup, and 2 patients (10%) had nonclassic epiglottitis. CONCLUSIONS. Immunization against Haemophilus influenza type b and widespread use of corticosteroids for the treatment of viral croup have changed the epidemiology of acute infectious upper airway disease. As potentially life-threatening infections, viral croup and epiglottitis have been eclipsed by bacterial tracheitis. In this series, bacterial tracheitis was 3 times more likely to have caused respiratory failure than viral croup and epiglottitis combined. Bacterial tracheitis should be considered in children who present with acute life-threatening upper airway infection. Key Words airway obstruction, epidemiology, croup, infection Abbreviations WBC--white blood cell MRSA--methicillin resistant Staphylococcus aureus ARDS--acute respiratory distress syndrome LOS--length of stay, AS A CONSEQUENCE Of evolving medical practice, the epidemiology of potentially life-threatening upper airway infections is changing. Until recently, viral croup and epiglottitis have been considered the main etiologies of [...]

Published
2006

24. Effect of IL-1[beta] on CRE-dependent gene expression in human airway smooth muscle cells

Author

Lahiri, Thomas, Moore, Paul E., Baraldo, Simonetta, Whitehead, Timothy R., McKenna, Matthew D., Panettieri, Reynold A., Jr., and Shore, Stephanie A.

Subjects
Smooth muscle -- Research, Biological sciences
Abstract

IL-1[beta] inhibits isoproterenol (ISO)-induced relaxation of cultured human airway smooth muscle (HASM) cells. The purpose of this study was to determine whether IL-1[beta] can also suppress ISO-induced cAMP response element (CRE)-dependent gene expression. ISO (10 [micro]M) caused a marked increase in CRE-binding protein (CREB) phosphorylation, which was attenuated by IL-1[beta] (2 ng/ml). This effect of IL-1[beta] was abolished by the cyclooxygenase (COX) inhibitor indomethacin. To examine CRE-driven gene expression, we transiently transfected HASM cells with a construct containing CRE upstream of a luciferase reporter gene. ISO (6 h) caused a sixfold increase in luciferase activity. IL-1[beta] (24 h) alone also increased luciferase activity, although to a lesser extent (2-fold). However, the ability of ISO to elicit luciferase expression was markedly reduced in cells treated with IL-1[beta]. Indomethacin, the MEK and p38 inhibitors U-0126 and SB-203580, the protein kinase A inhibitor H-89, and dexamethasone each completely abolished the ability of IL-1[beta] to induce CRE-driven gene expression but only slightly increased the ability of ISO to induce CRE-driven gene expression in IL-1[beta]-treated cells. IL-1[beta] also attenuated dibutyryl cAMP-induced CRE-driven gene expression, but not dibutyryl cAMP-induced CREB phosphorylation. Tumor necrosis factor-[alpha] (10 ng/ml) also attenuated ISO-induced CRE-driven gene expression, even though it was without effect on ISO-induced cAMP formation or ISO-induced CREB phosphorylation. The results suggest that IL-1[beta] and tumor necrosis factor-[alpha] may attenuate the ability of [beta]-agonists to induce expression of genes with CRE in their regulatory regions at least in part through events downstream of CREB phosphorylation. mitogen-activated protein kinase; cyclooxygenase-2; dexamethasone; cAMP response element binding; luciferase; tumor necrosis factor-[alpha]

Published
2002

28. Transmitted Home Oximetry and Duration of Home Oxygen in Premature Infants

Author

Rhein, Lawrence, primary, White, Heather, additional, Simoneau, Tregony, additional, Traeger, Nadav, additional, Lahiri, Thomas, additional, Kremer, Ted, additional, Sheils, Catherine, additional, Meyer, Kathleen, additional, Rosenkrantz, Ted, additional, Krishnan, Sankaran, additional, Hartman, Tyler, additional, Feldman, Henry A., additional, and Abu Jawdeh, Elie G., additional

Published
2020
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34. The optimization of home oxygen weaning in premature infants trial: Design, rationale, methods, and lessons learned

Author

Procaskey, Alexander, primary, White, Heather, additional, Simoneau, Tregony, additional, Traeger, Nadav, additional, Lahiri, Thomas, additional, Abu Jawdeh, Elie G., additional, Kremer, Ted, additional, Sheils, Catherine, additional, Meyer, Kathleen, additional, Rosenkrantz, Ted, additional, Krishnan, Sankaran, additional, Hartman, Tyler, additional, Feldman, Henry, additional, and Rhein, Lawrence, additional

Published
2018
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35. Efficacy and safety of lumacaftor and ivacaftor in patients aged 6–11 years with cystic fibrosis homozygous for F508del-CFTR : a randomised, placebo-controlled phase 3 trial

Author

Ratjen, Felix, primary, Hug, Christopher, additional, Marigowda, Gautham, additional, Tian, Simon, additional, Huang, Xiaohong, additional, Stanojevic, Sanja, additional, Milla, Carlos E, additional, Robinson, Paul D, additional, Waltz, David, additional, Davies, Jane C, additional, Rosenfeld, Margaret, additional, Starner, Timothy, additional, Retsch-Bogart, George, additional, Chmiel, James, additional, Orenstein, David, additional, Milla, Carlos, additional, Rubenstein, Ronald, additional, Walker, Seth, additional, Cornell, Alexandra, additional, Asfour, Fadi, additional, Black, Philip, additional, Colombo, John, additional, Froh, Deborah, additional, McColley, Susanna, additional, Ruiz, Fadel, additional, Quintero, Diana, additional, Casey, Alicia, additional, Mueller, Gary, additional, Flume, Patrick, additional, Livingston, Floyd, additional, Rock, Michael, additional, O'Sullivan, Brian, additional, Schmidt, Howard, additional, Lahiri, Thomas, additional, McNamara, John, additional, Chidekel, Aaron, additional, Sass, Laura, additional, Keens, Thomas, additional, Schaeffer, David, additional, Solomon, Melinda, additional, Chilvers, Mark, additional, Lands, Larry, additional, Junge, Sibylle, additional, Griese, Matthias, additional, Staab, Doris, additional, Pressler, Tacjana, additional, van Koningsburggen-Rietschel, Silke, additional, Naehrlich, Lutz, additional, Reid, Alastair, additional, Balfour-Lynn, Ian, additional, Urquhart, Don, additional, Lee, Timothy, additional, Munck, Anne, additional, Gaudelus, Isabelle Sermet, additional, De Boeck, Christiane, additional, Reix, Philippe, additional, Malfroot, Anne, additional, Bui, Stéphanie, additional, Selvadurai, Hiranjan, additional, Robinson, Philip, additional, Wainwright, Claire, additional, Clements, Barry, additional, Hilton, Jodi, additional, and Hjelte, Lena, additional

Published
2017
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36. Handbook of Cystic Fibrosis

Author

Filbrun, Amy G., Lahiri, Thomas., Ren, Clement L., Filbrun, Amy G., Lahiri, Thomas., and Ren, Clement L.

Published
2016

37. Interleukin-6 family cytokines: signaling and effects in human airway smooth muscle cells

Author

LAHIRI, THOMAS, LAPORTE, JOHANNE D., MOORE, PAUL E., PANETTIERI, REYNOLD A. JR., and SHORE, STEPHANIE A.

Subjects
Interleukin-6 -- Physiological aspects, Cyclooxygenases -- Physiological aspects, Smooth muscle -- Physiological aspects, Cytokines -- Physiological aspects, Respiratory organs -- Physiological aspects, Biological sciences
Abstract

Interleukin (IL)-1[Beta] induces cyclooxygenase (COX)-2 expression and prostanoid formation in cultured human airway smooth muscle (HASM) cells. In other cell types, IL-6 family cytokines induce COX-2 or augment IL-1[Beta]-induced COX-2 expression. The purpose of this study was to determine whether IL-6 family cytokines were involved in COX-2 expression in HASM cells. RT-PCR was used to demonstrate that the necessary receptor components for IL-6-type cytokine binding are expressed in HASM cells. IL-6 and oncostatin M (OSM) each caused a dose-dependent phosphorylation of signal transducer and activator of transcription-3, whereas IL-11 did not. IL-6, IL-11, and OSM alone had no effect on COX-2 expression. However, OSM caused dose-dependent augmentation of COX-2 expression and prostaglandin (PG) E2 release induced by IL-1[Beta]. In contrast, IL-6 and IL-11 did not alter IL-1[Beta]-induced COX-2 expression. IL-6 did increase IL-1[Beta]-induced [PGE.sub.2] formation in unstimulated cells but not in cells stimulated with arachidonic acid (AA; [10.sup.-5] M), suggesting that IL-6 effects were mediated at the level of AA release. Our results indicate that IL-6 and OSM are capable of inducing signaling in HASM cells. In addition, OSM and IL-1[Beta] synergistically cause COX-2 expression and [PGE.sub.2] release. oncostatin; cyclooxygenase; prostaglandin; signal transducer and activator of transcription

Published
2001

38. p38 MAP kinase regulates IL-1 [Beta] responses in cultured airway smooth muscle cells

Author

LAPORTE, JOHANNE D., MOORE, PAUL E., LAHIRI, THOMAS, SCHWARTZMAN, IGOR N., PANETTIERI, REYNOLD A. JR., and SHORE, STEPHANIE A.

Subjects
Smooth muscle -- Physiological aspects, Airway (Medicine) -- Physiological aspects, Protein kinases -- Physiological aspects, Interleukin-1 -- Physiological aspects, Biological sciences
Abstract

Laporte, Johanne D., Paul E. Moore, Thomas Lahiri, Igor N. Schwartzman, Reynold A. Panettieri, Jr., and Stephanie A. Shore. p38 MAP kinase regulates IL-1 [Beta] responses in cultured airway smooth muscle cells. Am J Physiol Lung Cell Mol Physiol 279: L932-L941, 2000.--We have previously reported that interleukin (IL)-1 [Beta] causes [Beta]-adrenergic hyporesponsiveness in cultured human airway smooth muscle (HASM) cells by increasing cyclooxygenase (COX)-2 expression. The purpose of this study was to determine whether p38 mitogen-activated protein (MAP) kinase is involved in these events. IL-1[Beta] (2 ng/ml for 15 min) increased p38 phosphorylation fourfold. The p38 inhibitor SB-203580 (3 [micro] M) decreased IL- 1[Beta]-induced COX-2 by 70 [+ or -] 7% (P [is less than] 0.01). SB-203580 had no effect on [PGE.sub.2] release in control cells but caused a significant (70-80%) reduction in [PGE.sub.2] release in IL-1[Beta]-treated cells. IL-1[Beta] increased the binding of nuclear proteins to the oligonucleotides encoding the consensus sequences for activator protein (AP)-1 and nuclear factor (NF)-[Kappa]B, but SB-203580 did not affect this binding, suggesting that the mechanism of action of p38 was not through AP-1 or NF-[Kappa]B activation. The NF-[Kappa]B inhibitor MG-132 did not alter IL-1[Beta]-induced COX-2 expression, indicating that NF-[Kappa]B activation is not required for IL-1[Beta]-induced COX-2 expression in HASM cells. IL-1[Beta] attenuated isoproterenolinduced decreases in HASM stiffness as measured by magnetic twisting cytometry, and SB-203580 abolished this effect. These results are consistent with the hypothesis that p38 is involved in the signal transduction pathway through which IL-1[Beta] induces COX-2 expression, [PGE.sub.2] release, and [Beta]-adrenergic hyporesponsiveness. mitogen-activated protein; interleukin-1[Beta]; human airway smooth muscle; SB-203580; nuclear factor-[Kappa]B; activator protein-1; prostaglandin [E.sub.2]; cyclooxygenase-2; [Beta]-adrenergic responsiveness; cytoskeletal mechanics; magnetic twisting cytometry

Published
2000

39. Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis

Author

Lahiri, Thomas, primary, Hempstead, Sarah E., additional, Brady, Cynthia, additional, Cannon, Carolyn L., additional, Clark, Kelli, additional, Condren, Michelle E., additional, Guill, Margaret F., additional, Guillerman, R. Paul, additional, Leone, Christina G., additional, Maguiness, Karen, additional, Monchil, Lisa, additional, Powers, Scott W., additional, Rosenfeld, Margaret, additional, Schwarzenberg, Sarah Jane, additional, Tompkins, Connie L., additional, Zemanick, Edith T., additional, and Davis, Stephanie D., additional

Published
2016
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40. FrontMatter.

Author

Filbrun, Amy G., Lahiri, Thomas, and Ren, Clement L.

Published
2016

41. Whole-Exome Sequencing Identifies Rare and Low-Frequency Coding Variants Associated with LDL Cholesterol

Author

Lange, Leslie A., primary, Hu, Youna, additional, Zhang, He, additional, Xue, Chenyi, additional, Schmidt, Ellen M., additional, Tang, Zheng-Zheng, additional, Bizon, Chris, additional, Lange, Ethan M., additional, Smith, Joshua D., additional, Turner, Emily H., additional, Jun, Goo, additional, Kang, Hyun Min, additional, Peloso, Gina, additional, Auer, Paul, additional, Li, Kuo-ping, additional, Flannick, Jason, additional, Zhang, Ji, additional, Fuchsberger, Christian, additional, Gaulton, Kyle, additional, Lindgren, Cecilia, additional, Locke, Adam, additional, Manning, Alisa, additional, Sim, Xueling, additional, Rivas, Manuel A., additional, Holmen, Oddgeir L., additional, Gottesman, Omri, additional, Lu, Yingchang, additional, Ruderfer, Douglas, additional, Stahl, Eli A., additional, Duan, Qing, additional, Li, Yun, additional, Durda, Peter, additional, Jiao, Shuo, additional, Isaacs, Aaron, additional, Hofman, Albert, additional, Bis, Joshua C., additional, Correa, Adolfo, additional, Griswold, Michael E., additional, Jakobsdottir, Johanna, additional, Smith, Albert V., additional, Schreiner, Pamela J., additional, Feitosa, Mary F., additional, Zhang, Qunyuan, additional, Huffman, Jennifer E., additional, Crosby, Jacy, additional, Wassel, Christina L., additional, Do, Ron, additional, Franceschini, Nora, additional, Martin, Lisa W., additional, Robinson, Jennifer G., additional, Assimes, Themistocles L., additional, Crosslin, David R., additional, Rosenthal, Elisabeth A., additional, Tsai, Michael, additional, Rieder, Mark J., additional, Farlow, Deborah N., additional, Folsom, Aaron R., additional, Lumley, Thomas, additional, Fox, Ervin R., additional, Carlson, Christopher S., additional, Peters, Ulrike, additional, Jackson, Rebecca D., additional, van Duijn, Cornelia M., additional, Uitterlinden, André G., additional, Levy, Daniel, additional, Rotter, Jerome I., additional, Taylor, Herman A., additional, Gudnason, Vilmundur, additional, Siscovick, David S., additional, Fornage, Myriam, additional, Borecki, Ingrid B., additional, Hayward, Caroline, additional, Rudan, Igor, additional, Chen, Y. Eugene, additional, Bottinger, Erwin P., additional, Loos, Ruth J.F., additional, Sætrom, Pål, additional, Hveem, Kristian, additional, Boehnke, Michael, additional, Groop, Leif, additional, McCarthy, Mark, additional, Meitinger, Thomas, additional, Ballantyne, Christie M., additional, Gabriel, Stacey B., additional, O’Donnell, Christopher J., additional, Post, Wendy S., additional, North, Kari E., additional, Reiner, Alexander P., additional, Boerwinkle, Eric, additional, Psaty, Bruce M., additional, Altshuler, David, additional, Kathiresan, Sekar, additional, Lin, Dan-Yu, additional, Jarvik, Gail P., additional, Cupples, L. Adrienne, additional, Kooperberg, Charles, additional, Wilson, James G., additional, Nickerson, Deborah A., additional, Abecasis, Goncalo R., additional, Rich, Stephen S., additional, Tracy, Russell P., additional, Willer, Cristen J., additional, Altshuler, David M., additional, Abecasis, Gonçalo R., additional, Allayee, Hooman, additional, Cresci, Sharon, additional, Daly, Mark J., additional, de Bakker, Paul I.W., additional, DePristo, Mark A., additional, Donnelly, Peter, additional, Fennell, Tim, additional, Garimella, Kiran, additional, Hazen, Stanley L., additional, Jordan, Daniel M., additional, Kiezun, Adam, additional, Lettre, Guillaume, additional, Li, Bingshan, additional, Li, Mingyao, additional, Newton-Cheh, Christopher H., additional, Padmanabhan, Sandosh, additional, Pulit, Sara, additional, Rader, Daniel J., additional, Reich, David, additional, Reilly, Muredach P., additional, Schwartz, Steve, additional, Scott, Laura, additional, Spertus, John A., additional, Stitziel, Nathaniel O., additional, Stoletzki, Nina, additional, Sunyaev, Shamil R., additional, Voight, Benjamin F., additional, Akylbekova, Ermeg, additional, Atwood, Larry D., additional, Barbalic, Maja, additional, Barr, R. Graham, additional, Benjamin, Emelia J., additional, Bis, Joshua, additional, Bowden, Donald W., additional, Brody, Jennifer, additional, Budoff, Matthew, additional, Burke, Greg, additional, Buxbaum, Sarah, additional, Carr, Jeff, additional, Chen, Donna T., additional, Chen, Ida Y., additional, Chen, Wei-Min, additional, Concannon, Pat, additional, D’Agostino, Ralph, additional, DeStefano, Anita L., additional, Dreisbach, Albert, additional, Dupuis, Josée, additional, Durda, J. Peter, additional, Ellis, Jaclyn, additional, Fox, Caroline S., additional, Fox, Ervin, additional, Funari, Vincent, additional, Ganesh, Santhi K., additional, Gardin, Julius, additional, Goff, David, additional, Gordon, Ora, additional, Grody, Wayne, additional, Gross, Myron, additional, Guo, Xiuqing, additional, Hall, Ira M., additional, Heard-Costa, Nancy L., additional, Heckbert, Susan R., additional, Heintz, Nicholas, additional, Herrington, David M., additional, Hickson, DeMarc, additional, Huang, Jie, additional, Hwang, Shih-Jen, additional, Jacobs, David R., additional, Jenny, Nancy S., additional, Johnson, Andrew D., additional, Johnson, Craig W., additional, Kawut, Steven, additional, Kronmal, Richard, additional, Kurz, Raluca, additional, Lange, Leslie A., additional, Larson, Martin G., additional, Lawson, Mark, additional, Lewis, Cora E., additional, Li, Dalin, additional, Lin, Honghuang, additional, Liu, Chunyu, additional, Liu, Jiankang, additional, Liu, Kiang, additional, Liu, Xiaoming, additional, Liu, Yongmei, additional, Longstreth, William T., additional, Loria, Cay, additional, Lunetta, Kathryn, additional, Mackey, Aaron J., additional, Mackey, Rachel, additional, Manichaikul, Ani, additional, Maxwell, Taylor, additional, McKnight, Barbara, additional, Meigs, James B., additional, Morrison, Alanna C., additional, Musani, Solomon K., additional, Mychaleckyj, Josyf C., additional, Nettleton, Jennifer A., additional, North, Kari, additional, O’Leary, Daniel, additional, Ong, Frank, additional, Palmas, Walter, additional, Pankow, James S., additional, Pankratz, Nathan D., additional, Paul, Shom, additional, Perez, Marco, additional, Person, Sharina D., additional, Polak, Joseph, additional, Quinlan, Aaron R., additional, Raffel, Leslie J., additional, Ramachandran, Vasan S., additional, Rice, Kenneth, additional, Sanders, Jill P., additional, Schreiner, Pamela, additional, Seshadri, Sudha, additional, Shea, Steve, additional, Sidney, Stephen, additional, Silverstein, Kevin, additional, Smith, Nicholas L., additional, Sotoodehnia, Nona, additional, Srinivasan, Asoke, additional, Taylor, Kent, additional, Thomas, Fridtjof, additional, Tsai, Michael Y., additional, Volcik, Kelly A., additional, Wassel, Chrstina L., additional, Watson, Karol, additional, Wei, Gina, additional, White, Wendy, additional, Wiggins, Kerri L., additional, Wilk, Jemma B., additional, Williams, O. Dale, additional, Wilson, Gregory, additional, Wolf, Phillip, additional, Zakai, Neil A., additional, Hardy, John, additional, Meschia, James F., additional, Nalls, Michael, additional, Singleton, Andrew, additional, Worrall, Brad, additional, Bamshad, Michael J., additional, Barnes, Kathleen C., additional, Abdulhamid, Ibrahim, additional, Accurso, Frank, additional, Anbar, Ran, additional, Beaty, Terri, additional, Bigham, Abigail, additional, Black, Phillip, additional, Bleecker, Eugene, additional, Buckingham, Kati, additional, Cairns, Anne Marie, additional, Caplan, Daniel, additional, Chatfield, Barbara, additional, Chidekel, Aaron, additional, Cho, Michael, additional, Christiani, David C., additional, Crapo, James D., additional, Crouch, Julia, additional, Daley, Denise, additional, Dang, Anthony, additional, Dang, Hong, additional, De Paula, Alicia, additional, DeCelie-Germana, Joan, additional, Drumm, Allen DozorMitch, additional, Dyson, Maynard, additional, Emerson, Julia, additional, Emond, Mary J., additional, Ferkol, Thomas, additional, Fink, Robert, additional, Foster, Cassandra, additional, Froh, Deborah, additional, Gao, Li, additional, Gershan, William, additional, Gibson, Ronald L., additional, Godwin, Elizabeth, additional, Gondor, Magdalen, additional, Gutierrez, Hector, additional, Hansel, Nadia N., additional, Hassoun, Paul M., additional, Hiatt, Peter, additional, Hokanson, John E., additional, Howenstine, Michelle, additional, Hummer, Laura K., additional, Kanga, Jamshed, additional, Kim, Yoonhee, additional, Knowles, Michael R., additional, Konstan, Michael, additional, Lahiri, Thomas, additional, Laird, Nan, additional, Lange, Christoph, additional, Lin, Lin, additional, Lin, Xihong, additional, Louie, Tin L., additional, Lynch, David, additional, Make, Barry, additional, Martin, Thomas R., additional, Mathai, Steve C., additional, Mathias, Rasika A., additional, McNamara, John, additional, McNamara, Sharon, additional, Meyers, Deborah, additional, Millard, Susan, additional, Mogayzel, Peter, additional, Moss, Richard, additional, Murray, Tanda, additional, Nielson, Dennis, additional, Noyes, Blakeslee, additional, O’Neal, Wanda, additional, Orenstein, David, additional, O’Sullivan, Brian, additional, Pace, Rhonda, additional, Pare, Peter, additional, Parker, H. Worth, additional, Passero, Mary Ann, additional, Perkett, Elizabeth, additional, Prestridge, Adrienne, additional, Rafaels, Nicholas M., additional, Ramsey, Bonnie, additional, Regan, Elizabeth, additional, Ren, Clement, additional, Retsch-Bogart, George, additional, Rock, Michael, additional, Rosen, Antony, additional, Rosenfeld, Margaret, additional, Ruczinski, Ingo, additional, Sanford, Andrew, additional, Schaeffer, David, additional, Sell, Cindy, additional, Sheehan, Daniel, additional, Silverman, Edwin K., additional, Sin, Don, additional, Spencer, Terry, additional, Stonebraker, Jackie, additional, Tabor, Holly K., additional, Varlotta, Laurie, additional, Vergara, Candelaria I., additional, Weiss, Robert, additional, Wigley, Fred, additional, Wise, Robert A., additional, Wright, Fred A., additional, Wurfel, Mark M., additional, Zanni, Robert, additional, Zou, Fei, additional, Green, Phil, additional, Shendure, Jay, additional, Akey, Joshua M., additional, Bustamante, Carlos D., additional, Eichler, Evan E., additional, Fox, P. Keolu, additional, Fu, Wenqing, additional, Gordon, Adam, additional, Gravel, Simon, additional, Johnsen, Jill M., additional, Kan, Mengyuan, additional, Kenny, Eimear E., additional, Kidd, Jeffrey M., additional, Lara-Garduno, Fremiet, additional, Leal, Suzanne M., additional, Liu, Dajiang J., additional, McGee, Sean, additional, O’Connor, Timothy D., additional, Paeper, Bryan, additional, Robertson, Peggy D., additional, Staples, Jeffrey C., additional, Tennessen, Jacob A., additional, Wang, Gao, additional, Yi, Qian, additional, Jackson, Rebecca, additional, Anderson, Garnet, additional, Anton-Culver, Hoda, additional, Auer, Paul L., additional, Beresford, Shirley, additional, Black, Henry, additional, Brunner, Robert, additional, Brzyski, Robert, additional, Burwen, Dale, additional, Caan, Bette, additional, Carty, Cara L., additional, Chlebowski, Rowan, additional, Cummings, Steven, additional, Curb, J. David, additional, Eaton, Charles B., additional, Ford, Leslie, additional, Fullerton, Stephanie M., additional, Gass, Margery, additional, Geller, Nancy, additional, Heiss, Gerardo, additional, Howard, Barbara V., additional, Hsu, Li, additional, Hutter, Carolyn M., additional, Ioannidis, John, additional, Johnson, Karen C., additional, Kuller, Lewis, additional, LaCroix, Andrea, additional, Lakshminarayan, Kamakshi, additional, Lane, Dorothy, additional, Lasser, Norman, additional, LeBlanc, Erin, additional, Li, Kuo-Ping, additional, Limacher, Marian, additional, Logsdon, Benjamin A., additional, Ludlam, Shari, additional, Manson, JoAnn E., additional, Margolis, Karen, additional, Martin, Lisa, additional, McGowan, Joan, additional, Monda, Keri L., additional, Kotchen, Jane Morley, additional, Nathan, Lauren, additional, Ockene, Judith, additional, O’Sullivan, Mary Jo, additional, Phillips, Lawrence S., additional, Prentice, Ross L., additional, Robbins, John, additional, Rossouw, Jacques E., additional, Sangi-Haghpeykar, Haleh, additional, Sarto, Gloria E., additional, Shumaker, Sally, additional, Simon, Michael S., additional, Stefanick, Marcia L., additional, Stein, Evan, additional, Tang, Hua, additional, Taylor, Kira C., additional, Thomson, Cynthia A., additional, Thornton, Timothy A., additional, Van Horn, Linda, additional, Vitolins, Mara, additional, Wactawski-Wende, Jean, additional, Wallace, Robert, additional, Wassertheil-Smoller, Sylvia, additional, Zeng, Donglin, additional, Applebaum-Bowden, Deborah, additional, Feolo, Michael, additional, Gan, Weiniu, additional, Paltoo, Dina N., additional, Sholinsky, Phyliss, additional, and Sturcke, Anne, additional

Published
2014
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