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BACKGROUND As diagnostic methods for primary ciliary dyskinesia are not generally available, we tested whether clinical criteria allow to preselect patients with a high probability of this disease, who should be further investigated in a specialized centre. PATIENTS AND METHODS In patients with chronic cough we compared parameters of the case history with the finding of a reduced ciliary beat frequency (CBF). Data sheets of 323 patients (133 females, 190 males) aged 1 week through 40 years (median age 4.5 years) were available for analysis. Of these patients 46 (14%) had a reduced CBF. RESULTS In this group the following features were found significantly more frequently compared to patients with normal CBF: neonatal respiratory disorder (odds ratio (OR) 9.0; 95% confidence interval (95% CI) 3.2;25), situs inversus (OR 8.1; 95% CI 2.5;26), retention of airway secretions (OR 6.7; 95% CI 2.4;19), recurrent pneumonia (OR 4.1; 95% CI 1.8;9.5), bronchiectasis (OR 3.5; 95% CI 1.2;11), asthma with poor response to treatment (OR 2.4; 95% CI 1.1;5.3). At least one of these potential indicators was present in 91% of the patients with reduced CBF. CONCLUSIONS In patients with chronic cough specific parameters of the case history indicate a high probability of a reduced ciliary beat frequency which is an indicator for primary ciliary dyskinesia. If none of these findings is present, a reduced CBF is highly unlikely.

BACKGROUND: Children with interstitial pneumonitis (IP) of unknown origin often have to undergo open lung biopsy to establish a final diagnosis. Open lung biopsy is an invasive procedure with major potential complications. In the meantime, CT-guided transthoracic lung biopsy (TLB) has become a common diagnostic procedure in adults. OBJECTIVE: The aim of this study was to retrospectively evaluate the efficacy and radiation exposure of low-dose CT-guided TLB in children with non-infectious IP of unknown origin. METHODS: Twelve children (7-males, age range: 7 months-15 years) with non-infectious IP of unknown origin and inconclusive clinical tests underwent CT-guided TLB with a 20-gauge biopsy instrument. A low-dose protocol with acquisition of single slices was used on a 16-row CT scanner: 80 kVp, 20 mAs, slice thickness 10 mm. Biopsy specimens were processed by standard histopathological and immunohistochemical techniques and effective doses were individually calculated. RESULTS: All biopsies were performed without major complications. Two children (17 %) developed a small pneumothorax/pulmonary haemorrhage that resolved spontaneously. A final diagnosis could be established in 9/12 patients (75 %) by CT-guided TLB. In 2 patients (17 %) the results of TLB were inconclusive; however, the clinical suspicion could be disproved. Open lung biopsy was performed in 1 patient (8 %), which demonstrated idiopathic pulmonary fibrosis. On average, the effective dose of CT-guided TLB was 0.78 mSv (0.4 - 1.1 mSv). CONCLUSION: Low-dose CT-guided TLB can be a helpful method for investigating children with non-infectious IP of unknown origin thus making open lung biopsy unnecessary. Application of a low-dose protocol leads to a significant reduction of radiation exposure in CT-guided TLB.

Children with interstitial pneumonitis (IP) of unknown origin often have to undergo open lung biopsy to establish a final diagnosis. Open lung biopsy is an invasive procedure with major potential complications. In the meantime, CT-guided transthoracic lung biopsy (TLB) has become a common diagnostic procedure in adults.The aim of this study was to retrospectively evaluate the efficacy and radiation exposure of low-dose CT-guided TLB in children with non-infectious IP of unknown origin.Twelve children (7-males, age range: 7 months-15 years) with non-infectious IP of unknown origin and inconclusive clinical tests underwent CT-guided TLB with a 20-gauge biopsy instrument. A low-dose protocol with acquisition of single slices was used on a 16-row CT scanner: 80 kVp, 20 mAs, slice thickness 10 mm. Biopsy specimens were processed by standard histopathological and immunohistochemical techniques and effective doses were individually calculated.All biopsies were performed without major complications. Two children (17 %) developed a small pneumothorax/pulmonary haemorrhage that resolved spontaneously. A final diagnosis could be established in 9/12 patients (75 %) by CT-guided TLB. In 2 patients (17 %) the results of TLB were inconclusive; however, the clinical suspicion could be disproved. Open lung biopsy was performed in 1 patient (8 %), which demonstrated idiopathic pulmonary fibrosis. On average, the effective dose of CT-guided TLB was 0.78 mSv (0.4 - 1.1 mSv).Low-dose CT-guided TLB can be a helpful method for investigating children with non-infectious IP of unknown origin thus making open lung biopsy unnecessary. Application of a low-dose protocol leads to a significant reduction of radiation exposure in CT-guided TLB.

Ziele: Bei Kindern mit chronisch-interstitiellen Lungenerkrankungen gehort die offene Lungenbiopsie zum Goldstandard der histopathologischen Diagnosesicherung. Das Verfahren ist aufwandig und belastend und mit erheblicher Morbiditat und Mortalitat verbunden. Die CT-navigierte transthorakale Lungenbiopsie (TLB) ist ein wenig invasives, gut etabliertes Verfahren in der Erwachsenenmedizin, wo es vornehmlich in der Diagnostik unklarer Lungenrundherde eingesetzt wird. Ist die CT-navigierte TLB unter Anwendung eines Niedrigdosis-Protokolls eine geeignete Methode zur histopathologischen/mikrobiologischen Diagnostik bei Kindern mit chronisch-interstitiellen Lungenerkrankungen? Methode: Bei sechs Kindern (Alter: 9 Monate –16 Jahre) mit unklarer interstitieller Lungenerkrankung wurde eine CT-navigierte TLB mit einem 20-G Biopsieinstrument durchgefuhrt. Alle Untersuchungen erfolgten unter Analgosedierung an einem Multidetektor-CT unter Anwendung eines Niedrigdosisprotokolls. Die Biopsate wurden nach Standardmethoden histopathologisch, bakteriologisch und virologisch analysiert. Ergebnis: Alle Biopsien wurden ohne wesentliche Komplikationen durchgefuhrt; ein Patient entwickelte einen geringen, spontan resorbierten Pneumothorax. Adaquates Untersuchungsmaterial wurde bei funf von sechs Kindern gewonnen. Die Biopsate zeigten eine chronisch interstitielle Alveolitis bei funf Patienten. Bei drei Kindern war die Chlamydia pneumonia-PCR positiv; jeweils ein Kind zeigte eine positive Mycoplasma pneumoniae-PCR/CMV-PCR. Die durchschnittliche effektive Dosis betrug 0,81 mSv. Schlussfolgerung: Die CT-navigierte TLB kann ein hilfreiches Instrument in der Abklarung von Kindern mit unklaren interstitiellen Lungenerkrankungen sein. Mit der Anwendung von Niedrigdosisprotokollen kann die Strahlenbelastung minimiert werden. In unserer Patientengruppe traten keine wesentlichen Komplikationen auf. Ein Nachteil der Methode besteht in der mangelhaften Beurteilung von Lungengefasen und Bronchiolen.

The further development of newborns with meconium aspiration (MA) has been studied retrospectively by various investigators. However, results are inconsistent (2, 3, 5, 6). In the present study 25 children with MA were investigated in the age of 1 to 11 years. The parents answered a questionnaire, the children were examined physically. All the 11 children older than 5 years were subjected to spirometry, bodyplethysmography, and a histamine provocation test. An age matched control group of 28 children was investigated in the same way. No differences were found between children with MA and the control group concerning history and physical examination. Only the symptom "cough without cold" was found significantly more frequently in preschool children (1 to 5 years) with MA as compared to control children (p < 0.01). However, none of the parameters of lung function testing revealed any difference between the two groups. Also, the medians of the histamine thresholds in both samples were normal and did not differ. In summary, in a group of 25 children with MA, when compared to children without MA, only the symptom "cough without cold" was found significantly more frequently during preschool age. No evidence of long term pulmonary sequalae was seen in children with MA.

The further development of newborns with meconium aspiration (MA) has been studied retrospectively by various investigators. However, results are inconsistent (2, 3, 5, 6). In the present study 25 children with MA were investigated in the age of 1 to 11 years. The parents answered a questionnaire, the children were examined physically. All the 11 children older than 5 years were subjected to spirometry, bodyplethysmography, and a histamine provocation test. An age matched control group of 28 children was investigated in the same way. No differences were found between children with MA and the control group concerning history and physical examination. Only the symptom "cough without cold" was found significantly more frequently in preschool children (1 to 5 years) with MA as compared to control children (p0.01). However, none of the parameters of lung function testing revealed any difference between the two groups. Also, the medians of the histamine thresholds in both samples were normal and did not differ. In summary, in a group of 25 children with MA, when compared to children without MA, only the symptom "cough without cold" was found significantly more frequently during preschool age. No evidence of long term pulmonary sequalae was seen in children with MA.