PMC4465165_01
Transmission electron microscopy (EM) of representative nasal epithelium cilia from patient #1 with primary ciliary dyskinesia caused by demonstration of cilia lacking of inner dynein arms and ciliary abnormalities of symmetry presumably related to defects in the radial spokes.
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Fig1
In 2007 the nasal brushing was performed, with demonstration of 100% cilia lacking of inner dynein arm associated with axonemal disorganization in 60% of the cilia, presumably related to defects in the radial spokes (Figure 1).
PMC4465165_02
Transmission electron microscopy (EM) of representative nasal epithelium cilia from patient #2 with primary ciliary dyskinesia caused by demonstration of cilia lacking of lacking outer dynein arm.
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Fig2
In 2007 the nasal brushing was performed, with demonstration of 100% cilia lacking outer dynein arm (Figure 2).
PMC4465165_03
Transmission electron microscopy (EM) of representative nasal epithelium cilia from patient #3 with primary ciliary dyskinesia caused by demonstration of cilia lacking of lacking outer dynein arm.
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Fig3
In 2007 the nasal brushing was performed, showing all examined cilia lacking outer dynein arm (Figure 3).
CT-scan performed in patient #3 during hospitalization for pneumonia in lower left lobe.
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Fig4
He was hospitalized for pneumonia to the lower left lobe as reported in the chest CT scan done on September 18, 2013 (Figure 4).
PMC4465165_04
Transmission electron microscopy (EM) of representative nasal epithelium cilia from patient #4 with primary ciliary dyskinesia caused by demonstration of cilia lacking of lacking outer dynein arms.
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Fig5
In 2008 the nasal brushing was performed, showing the absence of outer dynein arms in all the ciliary sections (Figure 5).
CT-scan performed in patient #4 during hospitalization for pneumonia in upper left lobe.
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Fig6
She had more chest CT scans, and the last one was done in 2013 (Figure 6).
PMC4465165_05
Transmission electron microscopy (EM) of representative nasal epithelium cilia from patient #5 with primary ciliary dyskinesia caused by demonstration of cilia lacking of lacking outer dynein arm.
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Fig7
In 2008 the nasal brushing was performed, with demonstration of 100% of cilia lacking outer dynein arms (Figure 7).
PMC4465165_06
Transmission electron microscopy (EM) of representative nasal epithelium cilia from patient #6 with demonstration of cilia with presence of both dynein arms but abnormalities in number and disposition of outer microtubular pair.
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Fig8
About 60% of cilia displayed aberrant number and/or localization of central or peripheral microtubules (Figure 8).
Patient #6, the cilia were asymmetric, some of them lacking of central pair. Basal bodies showed an atypical distribution.
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Fig9
Basal bodies showed an atypical distribution (Figure 9).
PMC4465165_07
Transmission electron microscopy (EM) of representative nasal epithelium cilia from patients #7 with demonstration of 50% normal ciliated cells. In the remaining 50% ciliated cells axonemes lacking both dynein arms were found.
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Fig10
In the remaining 50% ciliated cells axonemes lacking both dynein arms were observed (Figure 10).
PMC4465165_08
Transmission electron microscopy (EM) of representative nasal epithelium cilia from patients #8 with primary ciliary dyskinesia caused by lacking of inner dynein arms.
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Fig11
In 2008 the nasal brushing was performed, with 100% of the cilia lacking inner dynein arms (Figure 11).