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However, the major advantage <strong>of</strong> using measurements exhaled NO, particularly over sputum<br />

eosinophil counts, blood tests or airway challenges is obvious -<br />

particulady in children. It is a<br />

non-invasive test, analogous to lung function testing to which patients are very familiar' It is<br />

also likely to be available in more clinics and can be performed more quickly and more<br />

cheaply with immediate results.<br />

g.g Nitric oxide levels in primar.v ciliary dyskinesia<br />

This is an area where NO measurement was found early to be <strong>of</strong> particular value. Initially No<br />

was found to be absent in nasal measurements in four children with Kartagener's syndrome<br />

(Lundberg, Weitzberg et al. 1994). This led to the evaluation <strong>of</strong> 21 children with primary<br />

ciliary dyskinesia (PCD) in whom both nasal and oral NO was found to be significantly lower<br />

when compared to 60 healthy children. While there was some overlap with regard to<br />

individual lower airway results, only one child was an outlier from each group in the nasal<br />

NO ranges (Karadag, James et al. 1999). This has been confirmed in a recent study where the<br />

impairment <strong>of</strong> NO output was less pronounced in lower than the upper nasal respiratory tract<br />

after sampling at several sites in 17 PCD and 28 healthy subjects (Matrut, Escudier et al.<br />

2006). In another large cross-sectional study involving 102 subjects, the concentration <strong>of</strong><br />

exhaled NO was significantly lower in both PCD and CF patients than bronchiectasis and<br />

healthy subjects. <strong>The</strong> nasal NO, however, was markedly reduced only in the PCD subjects<br />

(Horvath, Loukides et al. 2003). This change appears to occur early in life as seen in two<br />

infants with pCD aged four and six months having significantly lower levels <strong>of</strong> NO compared<br />

to five healthy controls (Baraldi, Pasquale et a|.2004). <strong>The</strong>se low NO levels found are despite<br />

levels <strong>of</strong> nitrite, nitrate and S-nitrosothiol when measured in exhaled breath condensate being<br />

no different from normals (Csoma, Bush et al. 2003)'<br />

Use <strong>of</strong> NO as a screening tool for this condition has been investigated. From the study above<br />

reviewing children already diagnosed with PCD, the authors determined a cut <strong>of</strong>f <strong>of</strong> 250ppb<br />

for nasal NO as having both a positive and a negative predictive value <strong>of</strong> 0.95. This correctly<br />

detected 20 <strong>of</strong> 21 subjects as having PCD and conectly detected 19 <strong>of</strong> 20 as not having PCD<br />

(Karadag, James et al. Lggg). Measuring 31 children with PCD, 2l with non-CF<br />

bronchiectasis, 17 with CF, 35 with asthma and 53 healthy controls, a nasal NO <strong>of</strong> 250ppb<br />

gave a sensitivity <strong>of</strong> 977o and a specificity <strong>of</strong> gOVo for a diagnosis <strong>of</strong> PCD (Narang, Ersu et al'<br />

2OOZ). Of 34 children referred for investigation for possible PCD to a tertiary clinic, the 17<br />

that proved positive on biopsy again had significantly lower level <strong>of</strong> nasal NO. This group<br />

determined a nasal NO <strong>of</strong> 105ppb gave a specificity <strong>of</strong> 88Vo and a positive predictor value <strong>of</strong><br />

24r

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