Diagnosis of primary ciliary dyskinesia: An official American thoracic society clinical practice guideline

Adam J. Shapiro, Stephanie Davis, Deepika Polineni, Michele Manion, Margaret Rosenfeld, Sharon D. Dell, Mark A. Chilvers, Thomas W. Ferkol, Maimoona A. Zariwala, Scott D. Sagel, Maureen Josephson, Lucy Morgan, Ozge Yilmaz, Kenneth N. Olivier, Carlos Milla, Jessica E. Pittman, M. Leigh Anne Daniels, Marcus Herbert Jones, Ibrahim A. Janahi, Stephanie WareSam J. Daniel, Matthew L. Cooper, Lawrence M. Nogee, Billy Anton, Tori Eastvold, Lynn Ehrne, Elena Guadagno, Michael R. Knowles, Margaret W. Leigh, Valery Lavergne

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Background: This document presents the American Thoracic Society clinical practice guidelines for the diagnosis of primary ciliary dyskinesia (PCD). Target Audience: Clinicians investigating adult and pediatric patients for possible PCD. Methods: Systematic reviews and, when appropriate, meta-Analyses were conducted to summarize all available evidence pertinent to our clinical questions. Evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach for diagnosis and discussed by amultidisciplinary panelwith expertise in PCD. Predetermined conflict-of-interest management strategies were applied, and recommendations were formulated, written, and graded exclusively by the nonconflicted panelists. Three conflicted individuals were also prohibited from writing, editing, or providing feedback on the relevant sections of the manuscript. Results: After considering diagnostic test accuracy, confidence in the estimates for each diagnostic test, relative importance of test results studied, desirable and undesirable direct consequences of each diagnostic test, downstream consequences of each diagnostic test result, patient values and preferences, costs, feasibility, acceptability, and implications for health equity, the panel made recommendations for or against the use of specific diagnostic tests as compared with using the current reference standard (transmission electron microscopy and/or genetic testing) for the diagnosis of PCD. Conclusions: The panel formulated and provided a rationale for the direction as well as for the strength of each recommendation to establish the diagnosis of PCD.

Original languageEnglish (US)
Pages (from-to)e24-e39
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume197
Issue number12
DOIs
StatePublished - Jun 15 2018

Fingerprint

Kartagener Syndrome
Practice Guidelines
Routine Diagnostic Tests
Thorax
Conflict of Interest
Manuscripts
Patient Preference
Genetic Testing
Transmission Electron Microscopy
Meta-Analysis
Pediatrics
Costs and Cost Analysis

Keywords

  • Diagnosis
  • Kartagener syndrome
  • Nitric oxide
  • Primary ciliary dyskinesia
  • Situs inversus

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

Cite this

Diagnosis of primary ciliary dyskinesia : An official American thoracic society clinical practice guideline. / Shapiro, Adam J.; Davis, Stephanie; Polineni, Deepika; Manion, Michele; Rosenfeld, Margaret; Dell, Sharon D.; Chilvers, Mark A.; Ferkol, Thomas W.; Zariwala, Maimoona A.; Sagel, Scott D.; Josephson, Maureen; Morgan, Lucy; Yilmaz, Ozge; Olivier, Kenneth N.; Milla, Carlos; Pittman, Jessica E.; Leigh Anne Daniels, M.; Jones, Marcus Herbert; Janahi, Ibrahim A.; Ware, Stephanie; Daniel, Sam J.; Cooper, Matthew L.; Nogee, Lawrence M.; Anton, Billy; Eastvold, Tori; Ehrne, Lynn; Guadagno, Elena; Knowles, Michael R.; Leigh, Margaret W.; Lavergne, Valery.

In: American Journal of Respiratory and Critical Care Medicine, Vol. 197, No. 12, 15.06.2018, p. e24-e39.

Research output: Contribution to journalArticle

Shapiro, AJ, Davis, S, Polineni, D, Manion, M, Rosenfeld, M, Dell, SD, Chilvers, MA, Ferkol, TW, Zariwala, MA, Sagel, SD, Josephson, M, Morgan, L, Yilmaz, O, Olivier, KN, Milla, C, Pittman, JE, Leigh Anne Daniels, M, Jones, MH, Janahi, IA, Ware, S, Daniel, SJ, Cooper, ML, Nogee, LM, Anton, B, Eastvold, T, Ehrne, L, Guadagno, E, Knowles, MR, Leigh, MW & Lavergne, V 2018, 'Diagnosis of primary ciliary dyskinesia: An official American thoracic society clinical practice guideline', American Journal of Respiratory and Critical Care Medicine, vol. 197, no. 12, pp. e24-e39. https://doi.org/10.1164/rccm.201805-0819ST
Shapiro, Adam J. ; Davis, Stephanie ; Polineni, Deepika ; Manion, Michele ; Rosenfeld, Margaret ; Dell, Sharon D. ; Chilvers, Mark A. ; Ferkol, Thomas W. ; Zariwala, Maimoona A. ; Sagel, Scott D. ; Josephson, Maureen ; Morgan, Lucy ; Yilmaz, Ozge ; Olivier, Kenneth N. ; Milla, Carlos ; Pittman, Jessica E. ; Leigh Anne Daniels, M. ; Jones, Marcus Herbert ; Janahi, Ibrahim A. ; Ware, Stephanie ; Daniel, Sam J. ; Cooper, Matthew L. ; Nogee, Lawrence M. ; Anton, Billy ; Eastvold, Tori ; Ehrne, Lynn ; Guadagno, Elena ; Knowles, Michael R. ; Leigh, Margaret W. ; Lavergne, Valery. / Diagnosis of primary ciliary dyskinesia : An official American thoracic society clinical practice guideline. In: American Journal of Respiratory and Critical Care Medicine. 2018 ; Vol. 197, No. 12. pp. e24-e39.
@article{effdd1b0310049de8fe67bc21b613c56,
title = "Diagnosis of primary ciliary dyskinesia: An official American thoracic society clinical practice guideline",
abstract = "Background: This document presents the American Thoracic Society clinical practice guidelines for the diagnosis of primary ciliary dyskinesia (PCD). Target Audience: Clinicians investigating adult and pediatric patients for possible PCD. Methods: Systematic reviews and, when appropriate, meta-Analyses were conducted to summarize all available evidence pertinent to our clinical questions. Evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach for diagnosis and discussed by amultidisciplinary panelwith expertise in PCD. Predetermined conflict-of-interest management strategies were applied, and recommendations were formulated, written, and graded exclusively by the nonconflicted panelists. Three conflicted individuals were also prohibited from writing, editing, or providing feedback on the relevant sections of the manuscript. Results: After considering diagnostic test accuracy, confidence in the estimates for each diagnostic test, relative importance of test results studied, desirable and undesirable direct consequences of each diagnostic test, downstream consequences of each diagnostic test result, patient values and preferences, costs, feasibility, acceptability, and implications for health equity, the panel made recommendations for or against the use of specific diagnostic tests as compared with using the current reference standard (transmission electron microscopy and/or genetic testing) for the diagnosis of PCD. Conclusions: The panel formulated and provided a rationale for the direction as well as for the strength of each recommendation to establish the diagnosis of PCD.",
keywords = "Diagnosis, Kartagener syndrome, Nitric oxide, Primary ciliary dyskinesia, Situs inversus",
author = "Shapiro, {Adam J.} and Stephanie Davis and Deepika Polineni and Michele Manion and Margaret Rosenfeld and Dell, {Sharon D.} and Chilvers, {Mark A.} and Ferkol, {Thomas W.} and Zariwala, {Maimoona A.} and Sagel, {Scott D.} and Maureen Josephson and Lucy Morgan and Ozge Yilmaz and Olivier, {Kenneth N.} and Carlos Milla and Pittman, {Jessica E.} and {Leigh Anne Daniels}, M. and Jones, {Marcus Herbert} and Janahi, {Ibrahim A.} and Stephanie Ware and Daniel, {Sam J.} and Cooper, {Matthew L.} and Nogee, {Lawrence M.} and Billy Anton and Tori Eastvold and Lynn Ehrne and Elena Guadagno and Knowles, {Michael R.} and Leigh, {Margaret W.} and Valery Lavergne",
year = "2018",
month = "6",
day = "15",
doi = "10.1164/rccm.201805-0819ST",
language = "English (US)",
volume = "197",
pages = "e24--e39",
journal = "American Journal of Respiratory and Critical Care Medicine",
issn = "1073-449X",
publisher = "American Thoracic Society",
number = "12",

}

TY - JOUR

T1 - Diagnosis of primary ciliary dyskinesia

T2 - An official American thoracic society clinical practice guideline

AU - Shapiro, Adam J.

AU - Davis, Stephanie

AU - Polineni, Deepika

AU - Manion, Michele

AU - Rosenfeld, Margaret

AU - Dell, Sharon D.

AU - Chilvers, Mark A.

AU - Ferkol, Thomas W.

AU - Zariwala, Maimoona A.

AU - Sagel, Scott D.

AU - Josephson, Maureen

AU - Morgan, Lucy

AU - Yilmaz, Ozge

AU - Olivier, Kenneth N.

AU - Milla, Carlos

AU - Pittman, Jessica E.

AU - Leigh Anne Daniels, M.

AU - Jones, Marcus Herbert

AU - Janahi, Ibrahim A.

AU - Ware, Stephanie

AU - Daniel, Sam J.

AU - Cooper, Matthew L.

AU - Nogee, Lawrence M.

AU - Anton, Billy

AU - Eastvold, Tori

AU - Ehrne, Lynn

AU - Guadagno, Elena

AU - Knowles, Michael R.

AU - Leigh, Margaret W.

AU - Lavergne, Valery

PY - 2018/6/15

Y1 - 2018/6/15

N2 - Background: This document presents the American Thoracic Society clinical practice guidelines for the diagnosis of primary ciliary dyskinesia (PCD). Target Audience: Clinicians investigating adult and pediatric patients for possible PCD. Methods: Systematic reviews and, when appropriate, meta-Analyses were conducted to summarize all available evidence pertinent to our clinical questions. Evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach for diagnosis and discussed by amultidisciplinary panelwith expertise in PCD. Predetermined conflict-of-interest management strategies were applied, and recommendations were formulated, written, and graded exclusively by the nonconflicted panelists. Three conflicted individuals were also prohibited from writing, editing, or providing feedback on the relevant sections of the manuscript. Results: After considering diagnostic test accuracy, confidence in the estimates for each diagnostic test, relative importance of test results studied, desirable and undesirable direct consequences of each diagnostic test, downstream consequences of each diagnostic test result, patient values and preferences, costs, feasibility, acceptability, and implications for health equity, the panel made recommendations for or against the use of specific diagnostic tests as compared with using the current reference standard (transmission electron microscopy and/or genetic testing) for the diagnosis of PCD. Conclusions: The panel formulated and provided a rationale for the direction as well as for the strength of each recommendation to establish the diagnosis of PCD.

AB - Background: This document presents the American Thoracic Society clinical practice guidelines for the diagnosis of primary ciliary dyskinesia (PCD). Target Audience: Clinicians investigating adult and pediatric patients for possible PCD. Methods: Systematic reviews and, when appropriate, meta-Analyses were conducted to summarize all available evidence pertinent to our clinical questions. Evidence was assessed using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach for diagnosis and discussed by amultidisciplinary panelwith expertise in PCD. Predetermined conflict-of-interest management strategies were applied, and recommendations were formulated, written, and graded exclusively by the nonconflicted panelists. Three conflicted individuals were also prohibited from writing, editing, or providing feedback on the relevant sections of the manuscript. Results: After considering diagnostic test accuracy, confidence in the estimates for each diagnostic test, relative importance of test results studied, desirable and undesirable direct consequences of each diagnostic test, downstream consequences of each diagnostic test result, patient values and preferences, costs, feasibility, acceptability, and implications for health equity, the panel made recommendations for or against the use of specific diagnostic tests as compared with using the current reference standard (transmission electron microscopy and/or genetic testing) for the diagnosis of PCD. Conclusions: The panel formulated and provided a rationale for the direction as well as for the strength of each recommendation to establish the diagnosis of PCD.

KW - Diagnosis

KW - Kartagener syndrome

KW - Nitric oxide

KW - Primary ciliary dyskinesia

KW - Situs inversus

UR - http://www.scopus.com/inward/record.url?scp=85049040265&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85049040265&partnerID=8YFLogxK

U2 - 10.1164/rccm.201805-0819ST

DO - 10.1164/rccm.201805-0819ST

M3 - Article

C2 - 29905515

AN - SCOPUS:85049040265

VL - 197

SP - e24-e39

JO - American Journal of Respiratory and Critical Care Medicine

JF - American Journal of Respiratory and Critical Care Medicine

SN - 1073-449X

IS - 12

ER -