PMID- 37449771
OWN - NLM
STAT- MEDLINE
DCOM- 20230928
LR  - 20230928
IS  - 1099-0496 (Electronic)
IS  - 1099-0496 (Linking)
VI  - 58
IP  - 10
DP  - 2023 Oct
TI  - Treatment response to pulmonary exacerbation in primary ciliary dyskinesia.
PG  - 2857-2864
LID - 10.1002/ppul.26599 [doi]
AB  - INTRODUCTION: Pulmonary exacerbation (Pex) are common in pediatric primary 
      ciliary dyskinesia (PCD), however changes in forced expiratory volume in 1 s 
      precent predicted (FEV1pp) during Pex are not well described. AIM: To assess the 
      evolution of FEV1pp during Pex and to define factors associated with failure to 
      return to baseline lung function. METHOD: This was a retrospective study of 
      patients with PCD between 2010 and 2022. Pex were defined as the presence of 
      increased respiratory symptoms treated with intravenous (IV) antibiotics. The 
      main outcomes were the changes in FEV1 during therapy and the proportion of 
      patients (responders) achieving >/=90% of baseline FEV1pp values at the end of 
      admission. RESULTS: The study included 52 Pex events in 28 children with PCD. The 
      rate of responders was 32/41 (78%) at the end of admission. Nonresponse was 
      associated with lower median body mass index (BMI) Z-score (-2.4 vs. -0.4, 
      p < .01) and with a history of IV treated Pex in the previous year (p = .06). For 
      the 22 Pex with available FEV1pp measurements at mid admission, the median 
      relative and absolute improvement from admission to Day 7 was 9.1% and 6.2%, 
      respectively (p- .001), and from Days 7 to 14 was 4.4% and 2.8%, respectively 
      (p = .08). CONCLUSION: In children with PCD treated with IV antibiotics, the 
      majority of lung function recovery happens during the first week of IV therapy. 
      Lower BMI was associated with nonresponse to therapy.
CI  - (c) 2023 Wiley Periodicals LLC.
FAU - Gatt, Dvir
AU  - Gatt D
AUID- ORCID: 0000-0001-8666-5115
AD  - Division of Respiratory Medicine, The Hospital for Sick Children, University of 
      Toronto, Toronto, Ontario, Canada.
FAU - Shaw, Michelle
AU  - Shaw M
AUID- ORCID: 0000-0002-0966-6779
AD  - Translational Medicine, Research Institute, The Hospital for Sick Children, 
      Toronto, Ontario, Canada.
FAU - Waters, Valerie
AU  - Waters V
AD  - Department of Pediatrics, Division of Infectious Diseases, The Hospital for Sick 
      Children, University of Toronto, Toronto, Ontario, Canada.
FAU - Kritzinger, Fiona
AU  - Kritzinger F
AUID- ORCID: 0009-0004-0155-7377
AD  - Division of Respiratory Medicine, The Hospital for Sick Children, University of 
      Toronto, Toronto, Ontario, Canada.
FAU - Solomon, Melinda
AU  - Solomon M
AD  - Division of Respiratory Medicine, The Hospital for Sick Children, University of 
      Toronto, Toronto, Ontario, Canada.
FAU - Dell, Sharon
AU  - Dell S
AD  - Department of Pediatrics, Division of Respiratory Medicine, University of British 
      Columbia, Vancouver, British Columbia, Canada.
FAU - Ratjen, Felix
AU  - Ratjen F
AUID- ORCID: 0000-0003-4057-6592
AD  - Division of Respiratory Medicine, The Hospital for Sick Children, University of 
      Toronto, Toronto, Ontario, Canada.
AD  - Translational Medicine, Research Institute, The Hospital for Sick Children, 
      Toronto, Ontario, Canada.
LA  - eng
PT  - Journal Article
DEP - 20230714
PL  - United States
TA  - Pediatr Pulmonol
JT  - Pediatric pulmonology
JID - 8510590
RN  - 0 (Anti-Bacterial Agents)
SB  - IM
MH  - Humans
MH  - Child
MH  - Retrospective Studies
MH  - *Cystic Fibrosis/complications/drug therapy/diagnosis
MH  - Disease Progression
MH  - Lung
MH  - Forced Expiratory Volume
MH  - Anti-Bacterial Agents/therapeutic use
MH  - *Ciliary Motility Disorders
OTO - NOTNLM
OT  - FEV1
OT  - PFT
OT  - antibiotics
OT  - pediatric
OT  - response
EDAT- 2023/07/14 13:08
MHDA- 2023/09/28 06:42
CRDT- 2023/07/14 08:23
PHST- 2023/05/19 00:00 [revised]
PHST- 2023/03/29 00:00 [received]
PHST- 2023/07/03 00:00 [accepted]
PHST- 2023/09/28 06:42 [medline]
PHST- 2023/07/14 13:08 [pubmed]
PHST- 2023/07/14 08:23 [entrez]
AID - 10.1002/ppul.26599 [doi]
PST - ppublish
SO  - Pediatr Pulmonol. 2023 Oct;58(10):2857-2864. doi: 10.1002/ppul.26599. Epub 2023 
      Jul 14.