Clinical value of measurement of pulmonary radioaerosol mucociliary clearance in the work up of primary ciliary dyskinesia

Munkholm, Mathias; Nielsen, Kim Gjerum; Mortensen, Jann

doi:10.1186/s13550-015-0118-y

EJNMMI Research

Table 5 Elaboration of patients with significant discrepancies between test results

From: Clinical value of measurement of pulmonary radioaerosol mucociliary clearance in the work up of primary ciliary dyskinesia

Variables	Patient
Variables	1	2	3	4	5
Age, years	15	6	15	17	10
PRMC	PCD/SCD	PCD/SCD (A normal PRMC was found 2 years later)	PCD/SCD	Inconclusive	Inconclusive
Ciliary motility study	Abnormal (but some sequences are described as being normal)	Abnormal	Abnormal (described as only slightly abnormal and partly inconclusive test)	Abnormal (described as being only slightly abnormal)	Abnormal (described as being only slightly abnormal)
EM	Normal	Abnormal	Normal	Normal	Abnormal (described as being only slightly abnormal)
Nasal NO	Normal	Normal	Normal	–	Normal
Final clinical diagnosis	Not PCD	Not PCD	Not PCD	Not PCD	Not PCD
Comments on final clinical diagnosis	Recurring infections apparently due to immune deficiency	Today, asymptomatic	Unknown restrictive lung disease and recurring upper airway infections	Abnormal ciliary study might be due to SCD	Today, asymptomatic
	Recurring infections apparently due to immune deficiency	Abnormal PRMC, ciliary study and EM might be due to SCD			Abnormal ciliary study and EM might be due to SCD
	Abnormal PRMC and ciliary study might be due to SCD	Abnormal PRMC, ciliary study and EM might be due to SCD	Abnormal PRMC and ciliary study might be due to SCD		Abnormal ciliary study and EM might be due to SCD
Variables	Patient
Variables	6	7	8	9	10
Age, years	20	34	61	8	13
PRMC	Inconclusive	Inconclusive	Normal	Normal	Normal
Ciliary motility study	Abnormal (described as being only slightly abnormal)	Abnormal (described as being only slightly abnormal)	Abnormal (described as being only slightly abnormal)	Abnormal	Abnormal (described as being only slightly abnormal)
EM	–	Normal	–	Normal	–
Nasal NO	–	Normal	–	Normal	–
Final clinical diagnosis	Not PCD	Not PCD	Not PCD	Not PCD	Not PCD
Comments on final clinical diagnosis	Recurring aspergilloma	Abnormal ciliary study might be due to SCD	Asymptomatic today	Asthma	Asthma
	Recurring aspergilloma		Asymptomatic today	Today asymptomatic	Today, only few symptoms
	Abnormal ciliary study might be due to SCD		Abnormal ciliary study might be due to SCD	Abnormal ciliary study might be due to SCD	Abnormal ciliary study might be due to SCD
Variables	Patient
Variables	11	12	13	14	15
Age, years	10	11	7	8	13
PRMC	Normal	Normal	Normal	Normal	Normal
Ciliary motility study	Abnormal (the specimen was infected)	Abnormal	Abnormal (described as being only slightly abnormal)	Abnormal	Abnormal
EM	Normal	Abnormal	–	Normal	Normal
Nasal NO	Normal	Normal	Normal	Normal	Normal
Final clinical diagnosis	Not PCD	Not PCD	Not PCD	Not PCD	Not PCD
Comments on final clinical diagnosis	Severe asthma and atopic dermatitis	Today, asymptomatic	Asthma and allergies	Today asymptomatic	Abnormal ciliary study might be due to SCD
	Abnormal ciliary study might be due to SCD	No explanation has been found for the patient’s earlier airway symptoms	Today, only few airway symptoms	Abnormal ciliary study might be due to SCD
	Abnormal ciliary study might be due to SCD	Abnormal ciliary study and EM might be due to SCD	Abnormal ciliary study might be due to SCD	Abnormal ciliary study might be due to SCD
Variables	Patient
Variables	16	17	18	19	20	21
Age, years	24	5	7	9	37	12
PRMC	Normal	Normal	PCD/SCD	PCD/SCD	PCD/SCD	Inconclusive
Ciliary motility study	Abnormal	Abnormal (described as being only slightly abnormal)	Abnormal	Abnormal	Abnormal	Abnormal
EM	–	Normal	Normal (described as being partly inconclusive)	Normal	Normal (described as being partly inconclusive)	Normal
Nasal NO	Normal	Normal	–	–	–	Abnormal
Final clinical diagnosis	Not PCD	Not PCD	Verified PCD	Verified PCD	Verified PCD	Verified PCD
Comments on final clinical diagnosis	Abnormal ciliary study might be due to SCD	Asthma	Clinically PCD is plausible with frequent airway infections	Clinically PCD is plausible with frequent airway infections and severe basal bronchiectasis	Clinically PCD is plausible with frequent airway infections	Clinically PCD is plausible with chronic productive coughing, bronchiectasis and situs inversus
Comments on final clinical diagnosis	Abnormal ciliary study might be due to SCD	Abnormal ciliary study might be due to SCD	Clinically PCD is plausible with frequent airway infections		Clinically PCD is plausible with frequent airway infections

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