Asthma comorbidity of Primary ciliary dyskinesia among Children: JAMA

A recent study published in the
journal JAMA Network Open found that children with primary ciliary dyskinesia
can develop asthma.

Primary ciliary dyskinesia (PCD) is
a condition that affects the structure and function of motile cilia. It occurs
due to a genetic mutation. Literature shows that Asthma can be a comorbidity
for PCD due to the antigen stasis that develops on the airway surface. However,
there is an ambiguity in considering asthma a routine comorbidity for PCD in
children. Hence, researchers conducted a study to assess whether asthma should
be routinely considered a comorbidity among children with PCD.

Also Read: Combination therapy with Montelukast Sodium effective for managing Cough Variant Asthma in children, finds study

A case-control study was carried
out by collecting data from 2 large electronic health record (EHR)–based
databases: the Indiana Network for Patient Care Research (INPCR) cohort of 20
million EHRs and, in a validation cohort, TriNetX, which includes 112 million
EHRs.

Due to the lack of a specific
ICD-10 code for PCD, identifying PCD became a challenge for the authors. Hence,
researchers analyzed cases of co-occurring bronchiectasis (ICD-10 J47) and
situs inversus totalis (ICD-10 Q89.3) that are unlikely to occur without PCD.
The analysis assessed whether individuals with B-SIT also had asthma (ICD-10
J45). Cases and controls were matched by age, sex, ethnicity, and race (1:3
ratio in INPCR, 1:1 in TriNetX). Conditional logistic regression was performed,
adjusting for the matching strategy. A conditional exact test was done to
compare the proportion of asthma between cases and controls for INPCR.

Findings:

  • Out of 266 participants, including 124 B-SIT
    patients and 142 controls, participants were matched for age, sex, ethnicity,
    and race.
  • In the INPCR study, 9 children had B-SIT, and 27
    were in the control group.
  • All 9 B-SIT patients had asthma compared with
    only 1 among the controls (P < .001).
  • In TriNetX, which included 115 with B-SIT and
    same-matched controls, 84 had asthma, and 31 did not.
  • In the controls, 12 had asthma, and 103 did not.
  • The adjusted odds ratio for asthma patients with
    B-SIT was 22.3.
Also Read: Advances in the Diagnosis and Treatment of Primary Ciliary Dyskinesia: A Review

Thus, the study concluded that most
children with likely PCD (B-SIT) had asthma, highlighting the relationship
between PCD and asthma. The researchers underscore the importance of routinely
evaluating asthma as a potential comorbidity in PCD. Further research should be
done to clarify the immunopathogenesis and validation and should consider whether
asthmatic children exhibit PCD.

Further reading: Zein J, Owora
A, Kim HJ, Marozkina N, Gaston B. Asthma Among Children With
Primary Ciliary Dyskinesia. JAMA Netw Open. 2024;7(12):e2449795.
doi:10.1001/jamanetworkopen.2024.49795.

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