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The present guideline aims to improve the evidence-based management of children and adolescents with pediatric community-acquired pneumonia (pCAP). Despite a prevalence of approx. 300 cases per 100 000 children per year in Central Europe, mortality is very low. Prevention includes infection control measures and comprehensive immunization. The diagnosis can and should be established clinically by history, physical examination and pulse oximetry, with fever and tachypnea as cardinal features. Additional signs or symptoms such as severely compromised general condition, poor feeding, dehydration, altered consciousness or seizures discriminate subjects with severe pCAP from those with non-severe pCAP. Within an age-dependent spectrum of infectious agents, bacterial etiology cannot be reliably differentiated from viral or mixed infections by currently available biomarkers. Most children and adolescents with non-severe pCAP and oxygen saturation 92 % can be managed as outpatients without laboratory/microbiology workup or imaging. Anti-infective agents are not generally indicated and can be safely withheld especially in children of young age, with wheeze or other indices suggesting a viral origin. For calculated antibiotic therapy, aminopenicillins are the preferred drug class with comparable efficacy of oral (amoxicillin) and intravenous administration (ampicillin). Follow-up evaluation after 48 - 72 hours is mandatory for the assessment of clinical course, treatment success and potential complications such as parapneumonic pleural effusion or empyema, which may necessitate alternative or add-on therapy.Die vorliegende Fassung der deutschsprachigen AWMF-Leitlinie soll unter Berücksichtigung der vorliegenden Evidenz die medizinische Versorgung von Kindern und Jugendlichen mit ambulant erworbener Pneumonie (pediatric community acquired pneumonia, pCAP) verbessern. In Mitteleuropa steht einer Prävalenz von ca. 300 Fällen pro 100 000 Kinder/Jahr eine sehr geringe Mortalität gegenüber, die Prävention umfasst Hygiene-Maßnahmen und Impfung z. B. gegen Pneumokokken, Hämophilus, Masern und Influenza. Hauptsymptome der pCAP sind Fieber und Tachypnoe, die Diagnosestellung erfolgt primär klinisch durch Anamnese, körperliche Untersuchung und Pulsoxymetrie. Das zusätzliche Vorliegen von Warnsymptomen wie stark reduzierter Allgemeinzustand, Nahrungsverweigerung, Dehydratation, Bewusstseinsstörung oder Krampfanfälle definiert die schwere pCAP in Abgrenzung zur nicht-schweren pCAP. Das Erregerspektrum ist altersabhängig, zur Differenzierung zwischen viraler, bakterieller oder gemischt viral-bakterieller Infektion stehen jedoch keine zuverlässigen Biomarker zur Verfügung. Die meisten Kinder und Jugendlichen mit nicht-schwerer pCAP und O

Zur Sicherstellung einer hohen Behandlungsqualitat wird die medizinische Versorgung von sehr unreifen Fruhgeborenen in Perinatalzentren durchfuhrt. Folge dieser Zentralisation sind sehr grose Entfernungen vom Wohnort insbesondere zu Level-1-Perinatalzentren mit entsprechender Trennung der Familien uber mehrere Wochen. Die VOLL-FRUH-WEIT-Studie untersuchte die daraus resultierenden Belastungen und Bewaltigungsstrategien. Folgende Fragen wurden untersucht: Welche Bedeutung kommt der Wohnortnahe und der notwendigen Betreuung alterer Geschwister bei der Bewaltigung dieser Lebensphase unmittelbar nach Geburt sowie im weiteren Verlauf zu? Welche Probleme entstehen und wie werden Losungsansatze gefunden. Welche Rolle kommt hierbei dem familiaren bzw. sozialen Umfeld bei der Unterstutzung der Eltern zu und welche Ressourcen stehen bei unterschiedlichen Risikofaktoren zur Verfugung? Zur Erfassung des subjektiven Erlebens der Eltern und der Dynamik des Prozesses wurde die Studie in einem qualitativen Langsschnittdesign durchgefuhrt. 14 Eltern(-paare) willigten ein, zu 3 Zeitpunkten nach der Geburt an einem Interview nach der Methode des problemzentrierten Interviews nach Witzel teilzunehmen. Die Auswertung der Interviews erfolgte mittels der qualitativen Inhaltsanalyse nach Mayring. Mit zunehmender Dauer der stationaren Behandlung des Fruhgeborenen steigt die Belastung der Eltern und Geschwisterkinder. Die familiare Trennung stellte bei entfernt wohnenden Eltern die gravierendste Belastung dar. Bei kliniknah wohnenden Familien war das tagliche Pendeln zentraler Belastungsfaktor. Die Unterbringung im kliniknahen Elternhaus konnte die Belastungen durch Trennung oder Pendeln abmildern. Ein familiares Netz, das verbindlich abrufbar war und sowohl emotionalen Beistand als auch konkrete Hilfen bot, stellte unabhangig vom Wohnort eine zentrale Ressource dar. Die haufig problematisierte weite Entfernung zwischen Wohnort und Perinatalzentrum ist nur ein Belastungsfaktor unter vielen. Somit ergibt sich die Notwendigkeit, fruhzeitig eine individuelle Analyse der psychosozialen Lebensumstande und den daraus resultierenden Belastungen vorzunehmen. Darauf aufbauend lassen sich Hilfs- und Unterstutzungsangebote durch das psychosoziale Team gezielt einsetzen. Weiterhin erscheint eine kliniknahe Wohnmoglichkeit fur weit entfernt wohnende Eltern unerlasslich.

A reduction in specific airway conductance has been reported in infants with a history of an apparent life-threatening event (ALTE). It is unclear, however, whether this reflects upper or lower airway narrowing. We performed a controlled study to determine small airway patency in infants with ALTE. Lung function tests were performed in 26 infants with a history of ALTE and 27 healthy controls. Partial expiratory flow-volume curves were obtained during quiet sleep using the rapid chest compression technique; thoracic gas volume (TGV) and expiratory airway resistance (RAW) were measured by whole body plethysmography. Compliance of the respiratory system (Crs) was measured using the single breath occlusion technique. The median maximal flow at functional residual capacity (VmaxFRC) was 85 ml/s (range 10-198 ml/s) in patients and 123 (range 47-316 ml/s) in controls (P = 0.003). VmaxFRC corrected for TGV was 0.5 s(-1) (range 0.06-1.3 s[-1]) and 0.9 s(-1) (range 0.4-1.8 s[-1]), respectively (P = 0.001). TGV, RAW and Crs were not significantly different between patients and controls.Reduced small airway patency may play a role in the pathogenesis of ALTE.

Definition und Atiologie. Bei der EAA (Synonym: Hypersensitivitatspneumonie) handelt es sich um eine generalisierte Entzundung des Lungeninterstitiums und der Alveolen aufgrund chronischer Inhalation feinster Partikel meist organischer Herkunft. Haufige Allergene im Kindesalter sind Vogelantigene (Vogelhalterlunge) wie Federn oder Exkremente von Tauben, Wellensittichen, Papageien, Huhnern, Gansen, Enten (und somit auch Bettfedern). Weitere Allergene sind Bakterien und Schimmelpilze aus feuchtem Heu, Kompost, Gewachshausern (Farmerlunge), Klimaanlagen (Befeuchterlunge) oder feuchtem Mauerwerk (sick building). Seltenere Allergene sind Medikamente (z. B. Nitrofurantoin, Methotrexat) oder anorganische Substanzen bei Verwendung von Farben und Lacken.

Ein 11 Wochen alter Junge erkrankte akut mit Bewustseinsstorung und Hepatopathie mit Cholestase und Gerinnungsstorung, allerdings normalen Ammoniakwerten. Trotz mehrfach nachgewiesener Dikarbonsaureausscheidung im Urin lies sich kein Enzymdefekt im Fettsaureabbau nachweisen. Aufgrund einer zunehmenden Atemwegsobstruktion in Verbindung mit Fettstuhlen wurde die Diagnose Mukoviszidose vermutet und mittels Schweistest und Genanalyse bestatigt. Die intensive Therapie der Mukoviszidose fuhrte zur raschen Normalisierung samtlicher pathologische Befunde. Diskussion: Dieser Fall zeigt, das bei Reye-ahnlichen Krankheitsbildern und einer Dikarbonsaureausscheidung im Sauglingsalter auch an eine Mukoviszidose gedacht werden mus.

To study the effects of apneic pauses, sighs, and breathing patterns on functional residual capacity (FRC), we measured FRC repeatedly in 48 healthy preterm infants (weight at study 2,042 +/- 316 g [mean +/- SD], postconceptional age 36.6 +/- 2.0 wk), during unsedated sleep using a modified heliox/nitrogen washout technique. Breathing movements and pulse oximeter saturation (SpO2) were recorded throughout and recordings analyzed for the presence of regular and nonregular breathing pattern, apneic pauses, sighs, and desaturations (SpO2 < 90%) during the last 2 min prior to each FRC measurement. FRC was lower during nonregular than during regular breathing pattern (23.3 +/- 7.2 ml/kg versus 26.9 +/- 7.8 ml/kg, p < 0.02); however, this apparent effect of breathing pattern disappeared after controlling the data for apneic pauses. Apneic pauses resulted in a significant decrease in FRC: mean FRC was 20.0 +/- 6.8 ml/kg if measured within 2 min of an apneic pause, 26.0 +/- 6.9 ml/kg if measured after a sigh (p < 0.001), and 24.0 +/- 7.7 ml/kg if there had been neither a sigh nor an apneic pause (p < 0.05). The interval between the apneic pause and the FRC measurement had no effect on FRC. There was an inverse correlation between FRC and the speed with which SpO2 fell during desaturation (r = -O.5, p < 0.03). Apneic pauses resulted in a persistent reduction in FRC in these preterm infants. Sighs appeared to restore FRC. The significant relationship between FRC and the speed of desaturation found in this study underscores the importance of endogenous or exogenous strategies that help to increase FRC, such as sighs or the application of continuous positive airway pressure, for the stability of oxygenation in preterm infants who have difficulty maintaining their oxygenation.

Hintergrund: In Deutschland werden 9% aller Kinder zu früh geboren. Bedingt durch die zentralisierte Versorgung in Perinatalzentren, liegt der Wohnort der Eltern häufig in großer Entfernung zur Klinik. Neben den Sorgen und Ängsten um das frühgeborene Kind, den familiären[for full text, please go to the a.m. URL], 62. Jahrestagung der Norddeutschen Gesellschaft für Kinder- und Jugendmedizin (NDGKJ)

The determination of functional residual capacity (FRC) would be extremely helpful for the controlled adjustment of mechanical ventilation in sick neonates and infants. However, these patients have small lung volumes and usually have been intubated by uncuffed endotracheal tubes (ETT). Therefore, the open-circuit nitrogen washout technique (N2wo) may give false FRC values if the inspired oxygen concentration (FIO2) is high and leakage around the ETT is present. We evaluated the N2wo as supplied by the Pediatric Pulmonary System 2600 (Sensor-Medics) in a small-sized lung model by 570 measurements using five different ventilator settings, an FIO2 increasing up to 0.9, different bypass flows between 0 and 12 L/min, and various patterns of leakage, either during inspiration or exhalation, or both. We found the most reliable results (error, 0.6%; CV, 0.7%) with a bypass flow of 6 L/min. Absolute N2 volumes as small as 14 mL could be measured using an FIO2 as high as 0.9 with only slight loss of accuracy (error, 4%; CV, 2.8%). During leakage, FRC had been underestimated with a very strong correlation to the total amount of leakage over the measurement period, which was irrespective of the ventilatory parameters (r = 0.9, P < 0.001). The regression equation could, therefore, be used for FRC correction in the lung model. However, most of the miscalculation was due to N2 loss during expiratory leakage, which quite simply and reliably can be excluded by an end-inspiratory occlusion test.(ABSTRACT TRUNCATED AT 250 WORDS)

Comparative validation studies of pulse oximeters from different manufacturers have predominantly been performed in adult patients. However, due to differences in the patient-sensor interface, the results from those studies may not be transferable to the paediatric population. We therefore performed 123 simultaneous measurements (37 patients) of transcutaneous O2-saturation (StcO2) from 3 pulse oximeters and compared these with the arterial O2-saturation (SaO2) from a co-oximeter (Radiometer OSM 3). The median age of the 37 patients at the time of study was 2.0 months (range 1 day-5 years), their weight 4.7 kg (0.8-15.0 kg). The mean difference between StcO2 and SaO2 was -0.6% (SD 5.2%) for the Kontron 7840 pulse oximeter, -0.9% (SD 2.3%) for the Nellcor N200, and -1.2% (SD 3.5%) for the Radiometer OXI3 pulse oximeter. The corresponding 95% intervals were -7.4(-) + 9.6%, -5.7(-) + 4.3% and -7.0(-) + 6.3% for the Kontron, Nellcor and Radiometer instrument, respectively. These results demonstrate that neither of the 3 instruments systematically under- or overestimated SaO2. However, there were differences in precision, particularly in the low range of SaO2.

Neben den bekannten Gram-positiven Hospitalkeimen MRSA und VRE nimmt in den letzten Jahren das Problem der multiresistenten Gram-negativen Erreger auf den neonatologischen Intensivstationen zu. "Extended Spektrum beta-Laktamasen" (ESBL) sind Enzyme, die von Gram-negativen Bakterien produziert werden und in der Lage sind, Oxyimino-Cephalosporine und Monobactame zu inaktivieren. Im Zeitraum von Juli bis Dezember 2007 wurde bei 26 Neonaten auf der neonatologischen Intensivstation des Klinikums Oldenburg, einem Krankenhaus der Maximalversorgung mit 800 Betten, eine Besiedlung mit ESBL-bildenden Escherichia coli festgestellt. Aufgrund des erhohten Risikos fur eine Neugeborenensepsis und Meningitis beschloss das Ausbruchsmanagement-Team (AMT) beim Auftreten der ersten Falle, eine weitere Ausbreitung des multiresistenten Erregers durch die Etablierung von entsprechenden Hygienemasnahmen zu unterbinden. Hierzu gehorten die Isolierung der Patienten, die Schulung des Personals und der Eltern uber ESBL-Bildner, die Intensivierung der Handehygiene, das regelmasige Screening aller Kinder auf der Station, Umgebungsuntersuchungen und letztendlich Personalscreening. Durch diese Masnahmen konnte der Ausbruch jedoch nicht gestoppt werden. Als "ultima ratio" entschied sich das AMT fur den Einsatz eines Probiotikums mit Lactobacillus acidophilus und Bifidobacterium infantis. Durch diese Masnahme konnte der Ausbruch innerhalb kurzer Zeit beendet werden. Die Aufarbeitung des Ausbruchs mittels Multiplex-PCR fur bla-CTX-M und Pulsfeld-Gel-Elektrophorese (PFGE) zeigte, dass der Ausbruch nicht durch einen einzelnen Klon verursacht wurde. 22 von 26 Neonaten und zwei Mitarbeiterinnen aus der Pflege waren mit einem CTX-M-15 positiven beta-Laktamase produzierenden Escherichia coli Stamm besiedelt, der in der PFGE identische Muster zeigte.

BACKGROUND: Respiratory syncytial virus (RSV) infection is an important cause of viral respiratory tract infection in children. In contrast to other confirmed risk factors that predispose to a higher morbidity and mortality, the particular risk of a preexisting neuromuscular impairment (NMI) in hospitalized children with RSV infection has not been prospectively studied in a multicenter trial. METHODS: The DMS RSV Paed database was designed for the prospective multicenter documentation and analysis of all clinically relevant aspects of the management of inpatients with RSV infection. Patients with clinically relevant NMI were identified according to the specific comments of the attending physicians and compared with those without NMI. RESULTS: This study covers 6 consecutive seasons; the surveillance took place in 14 pediatric hospitals in Germany from 1999 to 2005. In total, 1568 RSV infections were prospectively documented in 1541 pediatric patients. Of these, 73 (4.7%) patients displayed a clinically relevant NMI; 41 (56%) NMI patients had at least 1 additional risk factor for a severe course of the infection (multiple risk factors in some patients; prematurity in 30, congenital heart disease in 19, chronic lung disease 6 and immunodeficiency in 8). Median age at diagnosis was higher in NMI patients (14 vs. 5 months); NMI patients had a greater risk of seizures (15.1% vs. 1.6%), and a higher proportion in the NMI group had to be mechanically ventilated (9.6% vs. 1.9%). Eventually, the attributable mortality was significantly higher in the NMI group (5.5% vs. 0.2%; P < 0.001 for all). Multivariate logistic regression confirmed that NMI was independently associated with pediatric intensive care unit (PICU) admission (OR, 4.94; 95% CI, 2.69-8.94; P < 0.001] and mechanical ventilation (OR, 3.85; 95% CI, 1.28-10.22; P = 0.017). CONCLUSION: This is the first prospective multicenter study confirming the hypothesis that children with clinically relevant NMI face an increased risk for severe RSV-disease. It seems reasonable to include NMI as a cofactor into the decision algorithm of passive immunization.

Es werden Infekte der oberen und Infekte der unteren Atemwege unterschieden. Bezuglich der Infektionserreger stellen aber die Atemwege von der Nase bis zu den Alveolen ein Kontinuum dar. Gewisse Erreger zeigen Vorliebe fur bestimmte Abschnitte der Atemwege (Abb. 8.1).

Two parameters of tidal breathing, the ratio of time to reach peak tidal expiratory flow to the total expiratory time (Tme/TE) and the ratio of volume exhaled at peak tidal expiratory flow to the total exhaled volume (dV/VT) were used to assess lung function in 21 sedated infants (aged 6–14 mo) with different degrees of airway obstruction. These parameters were compared with airway resistance as percentage predicted (Raw%) and maximum expiratory flow at functional residual capacity corrected for lung volume (VmaxFRC/TGV) VmaxFRC/TGV values correlated significantly with Tme/TE (r = 0.630, p = 0.002) as well as with dV/VT (r = 0.728. p - 0.001). Raw% values showed only a weak correlation with dV/VT (r - - 0.435, p - 0.048). We conclude that Tme/Te and dV/VT are both able to detect airway obstruction in infants and that these parameters corrclate much better with the forced expiratory flow values obtained by the rapid thoracic compression method than with airway resistance, determined by body plethysmography.

1550 nm optical transmission is the preferred technology to span long distances in hybrid-fiber-coax networks. In order to achieve a good transmission performance, however, impairments induced by Stimulated Brillouin Scattering (SBS), Self-Phase Modulation (SPM) and chirp have to be avoided. This paper presents the results of investigations on impairments with respect to standard single mode fiber as well as advanced fiber types such as non-zero dispersion shifted fibers and wide band attenuation optimized fibers.

Fur die sozialmedizinische Begutachtung von Erkrankungen der Atmungsorgane konnen mehrere Dimensionen der medizinischen Diagnostik von besonderer Bedeutung Scin.

The nitrogen washout technique usually involves exposure of the patient to 100% oxygen for several minutes. This may be dangerous in preterm infants who are at risk of retinopathy of prematurity (ROP). We wanted to know whether heliox (79% He, 21% O2) can be used instead of oxygen when determining functional residual capacity (FRC). FRC measurements were made in 14 preterm infants [median (range) gestational age at birth 34 wk (27-37 wk), and at time of study 36 wk (33-40 wk)] who were breathing room air. FRC was measured using a computerized infant pulmonary function system, beginning in random order with either 100% O2 followed by heliox or vice versa. There was no systematic difference between the two methods with regard to lung volume measurements: mean (SD) FRC values, corrected for body weight, were 22.9 (7.1) mL/kg for O2 and 23.4 (7.0) mL/kg for heliox. We did not observe a systematic influence of the type of washing gas used (heliox or oxygen) on FRC in these infants. Our results suggest that the use of heliox instead of pure oxygen may be a suitable and safer alternative for FRC measurements with the nitrogen washout technique in preterm infants who are breathing low concentrations of inspired oxygen and are still at risk of ROP.

Im Vergleich zum Erwachsenenalter gibt es fur die Bronchoskopie im Kindesalter einen anderen Indikationsbereich, Unterschiede in der Methode sowie in den Anforderungen an die Anasthesie aufgrund der besonderen anatomischen und physiologischen Gegebenheiten im Kindesalter.

Hohe Atemfrequenzen und kleine Atemzugvolumina sind die physiologischen Besonderheiten des Neugeborenen. Es kann nur eine geringe Atemarbeit aufbringen und hat ein auserst verletzliches Lungengewebe [4]. Weitere spirometrische Besonderheiten, die auch bei der Entwicklung von Neugeborenenbeatmungsgeraten berucksichtigt werden mussen, sind eine niedrige Residualkapazitat und Compliance des respiratorischen Systems (Lunge und Thoraxwand) sowie eine hohe pulmonale Resistance infolge der kleinen Bronchiolen.

Noncalibrated respiratory inductance plethysmography has been used to measure respiratory function by calculation of the phase angle and, more recently, by determination of the ratio of each time to reach peak tidal expiratory flow to total expiratory time (TPEF/TE). Since TPEF/TE is known to be decreased in airway obstruction when derived from flow signals obtained by a pneumotachograph, we wanted to develop an alternative method to measure rib cage and abdominal respiratory movements. For this purpose, we used two pressure sensors attached to the skin above the umbilicus and in the right medioclavicular line at the fourth intercostal space: "pressure sensor plethysmography". We tested the ability of this method to assess thoracoabdominal asynchrony and TPEF/TE by comparison with respiratory inductance plethysmographic and pneumotachographic measurements in 30 children, aged 1-12 yrs, with airway obstruction. The mean difference (95% confidence interval (95% CI)) between phase angles obtained by respiratory inductance plethysmography and pressure sensor plethysmography was only -5.8 degrees (range -18.0 to +6.4 degrees). Similarly, all methods used to measure TPEF/TE agreed well: mean differences (95% CI) between pneumotachographic and respiratory inductance plethysmographic, pneumotachographic and pressure sensor plethysmographic, and respiratory inductance plethysmographic and pressure sensor plethysmographic measurements of TPEF/TE were +0.01 (range -0.05 to +0.06), -0.03 (-0.09 to +0.03) and -0.03 (-0.10 to +0.04), respectively. We conclude that pressure sensor plethysmography is a simple and noninvasive method, and suitable to measure thoracoabdominal asynchrony and TPEF/TE ratios as well as respiratory inductance plethysmography and pneumotachography.

At the age of four months, a boy with a normal history and family history, suddenly fell ill with a life-threatening pneumocystis carinii-pneumonia. Surprisingly, this opportunistic infection was not brought about by a T-cell deficiency. However, the patient's diagnosis turned out to be the rare "Hyper-IgM-syndrome", confirmed by: serum levels of IgM always at least normal whereas IgG, IgA and IgE were markedly decreased or absent; the development of neutropenia and occasional diarrhea. Generally, infections with pneumocystis carinii are rare in isolated deficiencies of immunoglobulines, but relatively frequent in primary "Hyper-IgM-syndrome" (approx. 12% of the cases described). The boy finally recovered after receiving Cotrimoxacol (20 mg/kg bw/d) in an intensive care unit. Now, at the age of nearly two his condition is almost good under regular substitution of IgG. Cotrimoxacol (4 mg/kg bw/d) is recommended to prevent further pneumocystis carinii infections and most of the pathogenes which frequently appear in neutropenias.

Comparative validation studies of pulse oximeters from different manufacturers have predominantly been performed in adult patients. However, due to differences in the patient-sensor interface, the results from those studies may not be transferable to the paediatric population. We therefore performed 123 simultaneous measurements (37 patients) of transcutaneous O2-saturation (StcO2) from 3 pulse oximeters and compared these with the arterial O2-saturation (SaO2) from a co-oximeter (Radiometer OSM 3). The median age of the 37 patients at the time of study was 2.0 months (range 1 day-5 years), their weight 4.7 kg (0.8-15.0 kg). The mean difference between StcO2 and SaO2 was -0.6% (SD 5.2%) for the Kontron 7840 pulse oximeter, -0.9% (SD 2.3%) for the Nellcor N200, and -1.2% (SD 3.5%) for the Radiometer OXI3 pulse oximeter. The corresponding 95% intervals were -7.4(-) + 9.6%, -5.7(-) + 4.3% and -7.0(-) + 6.3% for the Kontron, Nellcor and Radiometer instrument, respectively. These results demonstrate that neither of the 3 instruments systematically under- or overestimated SaO2. However, there were differences in precision, particularly in the low range of SaO2.

Pulmonary artery sling is often associated with tracheal stenosis. In many cases repair of the vascular anomaly alone does not relieve dyspnea. Primary one-stage repair with long segment tracheal resection (2.4 cm) and relocation of the left pulmonary artery using cardiopulmonary bypass and deep hypothermic circulatory arrest is described in a 6.5-month-old girl weighing 6.5 kg. This technique resulted in normal ventilation and pulmonary flow distribution.

A male preterm infant of 32 weeks of gestation with history of severe polyhydramnios during pregnancy presented soon after birth with polyuria with initial sodium chloride loss subsequently followed by increasing potassium loss. After manifestation of hypokalaemia, hypochloraemia, alkalosis and high urinary prostaglandin concentrations, the diagnosis of the neonatal variant of Bartter's syndrome was made. The treatment consisted of administered of large amounts of fluid with sodium chloride and potassium supplementation and indomethacin (1.5 to 2 mg/kg per day).

A new, nearly monodisperse human serum albumin particle produced by air-pressure-nebulization was inhaled by children and lung-transplant patients.After inhalation of the particles obtained with an air-pressure nebulizer, the initial deposition pattern showed a marked tracheobronchial deposition which could be reproducibly obtained without a special breathing technique, the alveolar deposition being not higher than 10%. With the use of 99mTc, the radiation exposure is limited to a level which is low enough for children, but images can be taken up to 24 hrs later. Further parameters for in vivo characterization of the mucociliary function are the 24 h retention pattern and the velocity of particle motion in the trachea.Within the first 45 minutes, the global clearance rate was 51% in healthy children, which is rather high in comparison with the literature, most likely due to size of particles and the selection of patients with a mean age of 10.7 years. In ciliary dysfunction, the initial clearance rate was 16% and 46% within 24 h.With simplification of the preparation, application, and examination technique, this method is to be used in children, so that a wider use can be anticipated. The normally fast initial elimination of particles allows quick differentiation of normal and impaired ciliary function.

Inhaled frusemide prevents bronchoconstriction in asthmatic adults induced by various triggers. To determine if frusemide provides similar protection in children, whether this is age dependent and equally effective for central and peripheral airways, we performed a double blind, placebo controlled, randomised, crossover study on the effect of inhaled frusemide on lung function changes induced by cold air challenge in 21 asthmatic children. In addition, we measured diuresis before and after inhalation. Bronchodilatation after frusemide was not observed. However, deterioration in lung function after frusemide, compared with placebo, was significantly diminished: forced expiratory volume in one second (FEV1) was -5.7% v -11.5%, peak expiratory flow (PEF) -7.7% v -23.3%, maximum expiratory flow at 50% of vital capacity (MEF50VC) -16.0% v -35.2%, and at 60% of total lung capacity (MEF60TLC) -32.4% v -61.6%, and specific airways conduction -42.0% v -57.7%, respectively. This effect was not age dependent. Diuresis was significantly increased from a mean (SEM) of 198 (34) ml/3 hours before inhaled frusemide to 379 (62) ml/3 hours after nebulisation. We conclude that inhaled frusemide prevents cold air induced bronchoconstriction in asthmatic children and that increased diuresis can be expected with a dose as low as 28 mg of frusemide given by nebuliser.

Juvenile papillomatosis of the larynx is a disease of viral origin and is a rare cause of hoarseness and stridor in childhood. Although the tumours are basically benign, they may present problems in therapy due to their localisation and marked tendency to recur. According to the present state of knowledge, a combination of laser coagulation and alpha-interferon therapy is the treatment of choice. It can be applied on a long-term basis effectively and with few side effects due to individualised dosage determination. We report on a girl patient of 2 1/2 years of age with recurrent juvenile papillomatosis of the larynx. Long-term alpha-interferon therapy was so far successful in preventing progress of the pattern of findings. Treatment is well tolerated after the most suitable dose has been found for a particular patient. No side effects were seen that would have compelled us to discontinue the treatment. We are planning to continue interferon therapy for at least 1 to 2 years with regular endoscopic control.

Pulmonary function tests can be performed in general practice since small computerized spirometers have been developed. Pediatricians also see many patients with respiratory disease but measurement of lung function in children requires special considerations. We examined 10 commercially available spirometers to assess their suitability for testing pulmonary function in children.

To determine whether long-term enteral feedings can improve nutritional status and lung function parameters in patients with cystic fibrosis (CF), 11 patients (8 female, 3 male, age 7 to 23 years) received a percutaneous endoscopic gastrostomy (PEG) since February 1988. All patients were moderately to severely affected and extremely malnourished with a relative underweight of 15% or more. After 9 months of supplemental nocturnal feeding, mean body weight improved from 25.8 +/- 4.7 kg (mean, standard deviation) to 31.6 +/- 7.4 kg (p = 0.003); mean relative underweight decreased from 20.7 +/- 3.4% to 10.4 +/- 8.6% (p = 0.02). Lung function, which was measured either prior to or without any intravenous antibiotics, improved considerably: mean vital capacity increased from 39.5% of the predicted value to 46.8% after 9 months (p = 0.01), the mean peak flow rate improved from 39.5% to 50.8% (p = 0.01). This improvement in lung function is probably secondary to the increased muscle mass of the patients. Long-term nocturnal feedings via a percutaneous gastrostomy tube improve nutritional status and lung function severely in malnourished patients with CF.

We report the complex clinical course over 15 years in a child who at the age of 19 months developed patchy pneumonic infiltrates and bronchiolitis after measles. Chronic atelectasis led to resection of the right upper lung lobe. He developed bilateral bronchiectases, predominantly in the right lung. Ventilation/perfusion scans showed a major reduction of both ventilation and perfusion mainly on the right side. Early recognition of obliterative bronchiolitis is important in order to provide adequate supportive treatment of possible complications.

The rural Xuan Wei County, Yunnan Province, China, has an unusually high lung cancer mortality rate that cannot be attributed to tobacco smoke or occupational exposure. The lung cancer rate is associated with 'smoky' coal, in contrast to wood or 'smokeless' coal burned in unventilated homes. This study was conducted to characterize and compare mouse skin tumorigenicity of the coal and the wood combustion emissions and to link the animal data to human lung cancer. Indoor air particles (less than 10 microns) were collected from a central commune where the lung cancer mortality rate is high and smoky coal is the major fuel used and also from a south-western commune where lung cancer mortality rate is low and where wood or smokeless coal are the major fuels used. The organic extracts of these indoor air particles from smoky coal, smokeless coal and wood combustion were analysed for polycyclic aromatic hydrocarbons and assayed for skin tumor initiation activity and complete carcinogenicity in SENCAR mice. The results showed that the organic extract of the emission particles from smoky coal combustion is the most active in tumor initiation among the three combustion emission samples followed by smokeless coal and then wood. The organic extract of the particles from smoky coal combustion was shown to be a potent complete carcinogen, whereas the wood extract was relatively inactive as a complete carcinogen. The extract of particles from the smokeless coal combustion was not tested for complete carcinogenicity because of inadequate supply. Eighty-eight percent of the mice treated with the smoky coal extract showed carcinomas, averaging 1.1 carcinomas per tumor-bearing mouse at the end of the 77 week study. These findings were in agreement with the epidemiological data, which showed that the Xuan Wei residents using smoky coal as a major fuel in homes had a high lung cancer mortality rate. This study demonstrates that the results of the tumorigenicity assays in mice were in agreement with human lung cancer data.

The passive flow-volume and partial forced expiratory flow-volume techniques were used to assess pulmonary function in 14 spontaneously breathing infants with acute respiratory syncytial virus bronchiolitis. Two additional infants were studied while paralysed and ventilated. During the acute stage of the illness there was a significant reduction in forced expiratory flow rates and an increase in respiratory resistance. Although the mean thoracic gas volume for the group was increased, five infants did not compensate for their airways obstruction by hyperinflation. Curvilinear passive flow-volume curves were seen in three of the 14 non-ventilated infants and in both ventilated infants. At follow up three to four months later all passive flow-volume curves were linear. There was a significant reduction in hyperinflation and an increase in forced expiratory flow rates, but values still differed significantly from those in normal infants.

Lung mechanics and partial forced expiratory flows were measured serially in seven normal infants during the first year of life. Lung mechanics were measured by the end inspiratory occlusion technique and partial forced expiratory flows by the rapid chest compression method. Thoracic gas volume, compliance, and partial forced expiratory flows measured at functional residual capacity progressively increased with age and correlated with height cubed. Respiratory system resistance progressively fell whereas volume-corrected flows remained fairly constant over the study period. These findings provide longitudinal lung function data and support the concept of isotropic lung growth. Pediatr Pulmonol 1987; 3; 3–7.

SummaryThe delivery of drugs by inhalation is an integral component of asthma and chronic obstructive pulmonary disease (COPD) management. However, even with effective inhaled pharmacological therapies, asthma, particularly, remains poorly controlled around the world. The reasons for this are manifold, but limitations of treatment guidelines in terms of content, implementation and relevance to everyday clinical life, including insufficient patient education, access to health care and cost of medication as well as poor inhaler technique are likely to contribute. Considering that inhalation therapy is a cornerstone in asthma and COPD management, little advice is provided in the guidelines regarding inhaler selection. The pressurised metered dose inhaler (pMDI) is still the most frequently prescribed device worldwide, but even after repeated tuition many patients fail to use it correctly. In addition, the correct technique can be lost over time. Although several improvements in pMDIs such as a change in the propellant and actuation have resulted in improvements in lung deposition, many dry powder inhalers (DPIs) are easier to use. However, these devices also have limitations such as dependency of drug particle size on flow rate and loss of the metered dose if the patient exhales through the device before inhaling. Improvements in using inhalation devices more efficiently, in inhaler design for supporting patient compliance, and advances in inhaler technology to assure drug delivery to the lungs, have the potential to improve asthma and COPD management and control. New and advanced devices are considered being helpful to minimise the most important problems patients have with current DPIs.