Case report
Living-donor lobar lung transplantation for primary ciliary dyskinesia

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Abstract

A ventilator-dependent patient with primary ciliary dyskinesia underwent successful living-donor lobar lung transplantation. The case was a 24-year-old woman who had developed recurrent lower respiratory infection and became ventilator-dependent due to severe bronchiectasis. Transmission electron microscopy of the resected bronchus demonstrated inner dynein arm deficiency.

Section snippets

Comment

There has been growing appreciation of the significant role played by the mucociliary transport system in the body. The mucociliary transport system is an important defense mechanism by which the human body usually maintains its homeostasis by protecting the body against invading particles, including bacteria. This system includes two major functional mechanisms, mucous secretional and ciliary transport systems, each of which is usually complementary and cooperative. Cystic fibrosis (CF) is

Acknowledgements

We acknowledge Professor Yuji Ohtsuki, Department of Pathology II, Kochi Medical University, for his electron micrograph courtesy.

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