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Abstract

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Article in Japanese

Case Report

An adult case of primary ciliary dyskinesia with recurrent pneumonia beginning post partum

Sachi Matsubayashia,b  Eriko Morinoa  Konomi Kobayashia  Motoyasu Iikuraa  Shinyu Izumia  Haruhito Sugiyamaa 

aDepartment of Respiratory Medicine, Center Hospital of the National Center for Global Health and Medicine
bDepartment of Respiratory Medicine, The Jikei University Daisan Hospital

ABSTRACT

A 32-year-old woman presented with recurrent pneumonia that had started 6 months after her delivery. She was admitted for evaluation of the cause and treatment of the pneumonia. She had no history of smoking or chronic sinusitis. The sputum culture tested positive for the pathogen Moraxella catarrhalis, which commonly colonizes the upper respiratory tracts of infants. She was administered antibiotics for a total of 4 weeks, mainly ampicillin/sulbactam 3g, four times a day. Consequently, the area of consolidation on the left lung completely disappeared. However, the shadow reappeared, only after 6 days after cessation of antibiotics. We could not find any deficiency in humoral immunity or cell-mediated immunity, nor any abnormality in the bronchopulmonary structures. Electron microscopic examination of multiple bronchial biopsy specimens obtained from within and outside the areas of consolidation revealed a defect in both the inner and outer dynein arms of the mucosal cilia and microtubular disarrangement. This led to the diagnosis of primary ciliary dyskinesia. Consequently, low-dose erythromycin (200mg twice daily) was started, after which pneumonia did not recur. Exposure to the newborn may have triggered the recurrent pneumonia.

KEYWORDS

Primary ciliary dyskinesia (PCD)  Ultrastructure  Dynein arm  Macrolide 

Received 7 Nov 2019 / Accepted 24 Dec 2019

AJRS, 9(2): 147-150, 2020

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