PURPOSE: Ciliary dyskinesia (CD) is a fairly uncommon disorder (1 in 15,000 to 34,000) which, from a pulmonary perspective, leads to difficulty in airway clearance and recurrent infections. Although this disorder is uncommon, it is even more uncommon that a child should have hemosiderin-laden macrophages (HLM) in the bronchoalveolar lavage fluid (0.24 to 1.23 cases per million). The purpose of this study was to determine the incidence of occurrence together.
METHODS: A retrospective study using ICD9 code with chart review was done covering a 17 month period in our institution. Inclusion criteria required a diagnosis of CD, as defined by electron microscopy, and evaluation of bronchoalveolar lavage fluid (BALF) specifically for HLM. All slides from the BALF were prepared with prussian blue by the pathology department. Staff pathologists at St. Joseph’s Hospitals and Clinics reviewed the slides, and only specimens that appeared to be significant in number were reported.
RESULTS: There were a total of 15 identified with the diagonosis of CD. Of those, all had BAL studies looking specifically at HLM. Of the 15, a total of 7 (47%) had HLM identified.
CONCLUSION: There is an increased incidence of hemosiderin-laden macrophages in CD when compared to the general population. Both entities are uncommon, but it appears to be more common in the population with CD to have HLM than the general population.
CLINICAL IMPLICATIONS: Although CD could be difficult to manage and leads ultimately to bronchiectasis and pulmonary complications, it may be more difficult to manage in a child with HLM. The presence of HLM may be secondary to repeat infections, or the increase in HLM might add to infections. Steroid use in the child with CD typically has no role, however if there are HLM, there may be some benefit. With a diagnosis of CD, it may therefore be of benefit to evaluate the BALF for HLM when possible.
DISCLOSURE: Barbara Stewart, None.