[Acute asthma attacks in childhood]. / Der akute Asthmaanfall im Kindesalter.

The diagnosis of an acute asthmatic attack in a child is made on a clinical basis. The severity of the exacerbation can be assessed by physical examination and measurement of the transcutaneous oxygenation saturation. A blood gas analysis can be helpful in this assessment. A child with a severe asthma exacerbation should be promptly referred to an emergency department of a hospital. Oxygen should be given to keep the oxygen saturation above 92% and short-acting, selective beta-2 agonists should be administered. Beta-2 agonists can be delivered by intermittent nebulization, continuous nebulization or by metered dose inhaler (MDI) with a spacer They can also be given intravenously in patients who are unresponsive to escalating therapy. The early administration of systemic corticosteroids is essential for the management of acute asthma in children. When tolerated, systemic corticoseroids can be given orally but inhaled corticosteroids are not recommended. Oxygen delivery, beta-2 agonists and steroid therapy are the mainstay of emergency treatment. Hypovolemia should be corrected either intravenously or orally. Administration of multiple doses of ipratropium bromide has been shown to decrease the hospitalization rate in children and adolescents with severe asthma. Clinical response to initial treatment is the main criterion for hospital admission. Patients with failure to respond to treatment should be transferred to an intensive care unit. A critical aspect of management of the acute asthma attack in a child is the prevention of similar attacks in the future.

Which factors account for renal stone formation in cystic fibrosis?

AIMS: Studies dealing with the increased tendency to stone formation noted in cystic fibrosis, focus on enteric hyperoxaluria. It is well recognized, however, that low urine volume, hypocitraturia and perhaps even hypercalciuria are further risk factors for stone formation. METHODS: Nineteen patients with cystic fibrosis (14 boys and 5 girls, aged 10-23, median 15 years) underwent a standard protocol for metabolic evaluation of the lithogenic tendency. In 10 patients, the study was repeated after treatment with recombinant human growth hormone 43 microgram/kg body weight daily for 12 months. RESULTS: The metabolic evaluation disclosed low urine output in 12, hyperoxaluria in 8 and hypocitraturia in 9 of the 19 cystic fibrosis patients. The mentioned parameters were not influenced by treatment with recombinant human growth hormone. CONCLUSION: The report indicates that in cystic fibrosis low urine volume, hypocitraturia and hyperoxaluria act in concert and contribute to the likelihood of stone formation. This tendency is not modified by treatment with recombinant human growth hormone.

Antihypertensive efficacy of amlodipine in children with chronic kidney diseases.

In adults the calcium antagonist amlodipine given once a day has proved to be an attractive addition to the antihypertensive armamentarium. The present report describes our experience in 43 paediatric outpatients (26 boys and 17 girls, aged between 1.1 and 19, median 9.8 years) with chronic kidney diseases. The patients were given amlodipine for 16 weeks as part of their antihypertensive treatment. Before amlodipine arterial pressure was 150 (142-163)/90 (84-95) mm Hg (median and interquartile range). Six patients withdrew from amlodipine because of oedema, flushing or headache. In the remaining patients amlodipine 7.7 (6.9-9.4) mg/m(2) body surface area once a day significantly decreased arterial pressure by 17 (13-22)/10 (7-13) mm Hg. The efficacy of amlodipine was more pronounced in girls than in boys. No changes in heart rate, body weight and circulating haemoglobin, sodium, potassium and creatinine were noted. In none of the patients circulating potassium, sodium or creatinine changed by more than 0.5 mmol/l, 5 mmol/l respectively 20%. In 11 patients concomitantly treated with cyclosporine the dosage and the trough-level of this agent were stable throughout the trial. In conclusion the present experience in paediatric outpatients with chronic kidney diseases supports the view that amlodipine is an effective and rather well tolerated antihypertensive drug when given once a day.

Early detection of lung disease and its association with the nutritional status, genetic background and life events in patients with cystic fibrosis.

Progression of lung disease is the most prominent cause of morbidity and death in patients with cystic fibrosis (CF), but the severity of lung disease and the rate of lung function decline are highly variable. An attempt was made to define accurate estimates of disease progression in these patients early diagnosed and prospectively evaluated until 10 years of age. The primary question to ask was whether functional abnormalities detected already in infancy are associated with functional derangements later on in life, and may be useful as parameters of prognostic value. Early diagnosis of CF can best be achieved by screening of mutation by new techniques (buccal cell brushing) in infants, even when the sweat test or accurate blood sampling is not available. Moreover, in infants lung function can be assessed by infant whole-body plethysmography enabling the study of the interrelationship with delayed weight gain and growth retardation, as well as the associations with the most common disease-causing mutations. Out of a cohort of 80 infants (39 males, 41 females) with CF a follow-up study was started with 50 CF infants diagnosed during infancy (mean age 4.6 +/- 4.0 months; range 0.1-12.7 months) and prospectively evaluated at 6-month intervals during the first 2 years of life. Moreover, in 32 CF children out of this cohort, follow-up was continued until 10 years of age. Differences were encountered with respect to the different events occurring during the first years of life, especially the onset of chronic colonization with Pseudomonas aeruginosa. The association between infant lung function and specific mutations (DeltaF508 homozygotes, frameshift DeltaF508/3905insT compound heterozygotes and nonsense DeltaF508/R553X compound heterozygotes) furthermore revealed that differences in lung function within the genetic groups are mainly related to the degree of pulmonary hyperinflation. Pulmonary hyperinflation was also associated with the degree of impaired nutritional status. An association between impaired gas exchange characteristics at 10 years of age and the degree of pulmonary hyperinflation during infancy finally demonstrates that by early mutation screening, lung function testing and assessment of the nutritional status predictors of disease progression later on in life can be defined. Therefore, preventive therapeutic measures should primarily be based on such prognostic factors.

Nutrition and lung function in cystic fibrosis patients: review.

Complex interactions between nutrition, skeletal and respiratory muscle function and energy expenditure in cystic fibrosis patients exist. Malnutrition significantly contributes to muscle weakness in patients with chronic obstructive pulmonary disease of the adult or in cystic fibrosis in childhood. Together with a measurable increase in resting energy expenditure the malnutrition, as a consequence of pancreatic insufficiency, leads to pulmonary deterioration. Whether pulmonary disease, pancreatic insufficiency, increased energy expenditure or insufficient intake of nutrition are the starters for the destructive circle or whether the basic defect is responsible for some of the components interacting with each other remains to be determined.

Clinical and physiological improvement after inhalation of low-dose beclomethasone dipropionate and salbutamol in wheezy infants.

UNLABELLED: Twenty-nine of initially 42 infants with recurrent wheeze (20 male and 9 female) with an age range of 2.1-25.2 months were randomly assigned to receive either 100 micrograms beclomethasone dipropionate (BDP) combined with 200 micrograms salbutamol (group BDP-S, n = 9), 200 micrograms salbutamol (group S, n = 8), or placebo (group P, n = 6) 3 times daily for a 6 weeks' treatment period. Six infants had to be treated openly with BDP-S (group O, n = 6) because of deterioration in the disease state. Ten babies were excluded because of incomplete data and poor drug compliance and further 3 because of needed rescue medication. The drugs were inhaled from a metered dose inhaler through a baby-adapted spacer device, the Babyhaler. Control was assessed by symptom diaries, and infant whole-body plethysmography. Values of thoracic gas volume (TGV), airway conductance (Gaw) and specific airway conductance (sGaw) were calculated numerically independent of age and body length in percent predicted and in standard deviation scores using regression equations taken from healthy infants previously evaluated. Functional improvement was considered to have occurred when either TGV, and/or Gaw improved more than 2 SD from baseline. There was a significant improvement in the symptom score (p < 0.05), particularly concerning cough, in addition to a significant decrease in pulmonary hyperinflation (TGV: p < 0.05) and improvement of Gaw (p < 0.01) and sGaw (p < 0.01) in the BDP-S group when compared to the P group. No significant differences were found between the BDP-S and S group and/or between the S and P groups. CONCLUSIONS: In wheezy infants BDP improves clinical status and lung function, when given in combination with salbutamol by a baby-adapted spacer device.

Plethysmographic measurements in the clinical assessment of infants with bronchopulmonary disease.

Whole-body plethysmography makes it possible, to measure, during the same test sequence, the end-expiratory resting level (thoracic gas volume (TGV)), and, hence, an estimate of lung volume, and its close inter-relationship to airway function (airway resistance (Raw), or its reciprocal value airway conductance (Gaw). An overview is given of the physiological background and some equipment required for this technique. Furthermore, the attractive usefulness of whole-body plethysmography in clinical routine is discussed. Based on plethysmographic data obtained in 118 infant survivors of respiratory distress syndrome (RDS), in wheezy infants and infants with cystic fibrosis (CF), the important inter-relationship between changes in end-expiratory resting level (TGV) and the deficit in airway mechanics (Gaw) is shown, and special emphasis is given to the absolute need to obtain these measurements simultaneously. It can be shown that this recommendation is of even greater clinical importance in view of the fact that the younger the child the more frequent and severe the pulmonary hyperinflation present. Finally, this inter-relationship has to be borne in mind when reversibility of functional abnormalities on adrenoceptor agonists is assessed by lung function measurements.

[Assessment of intrapulmonary ventilation disorders in children with bronchial asthma using the nitrogen elimination technique]. / Erfassen intrapulmonaler Verteilungsstörungen bei Kindern mit Asthma bronchiale mittels Stickstoffeliminationstechnik.

Stratification of functional abnormalities evaluated by whole-body plethysmography in asthmatic children can be characterized into three functional groups: pulmonary hyperinflation (H: TGV > mean + 2SD), bronchial obstruction (O: Raw > mean + 2SD) and a mixed type, group M, including both abnormalities. The multibreath nitrogen washout (MBNW) offers the possibility to measure FRC and calculate the amount of trapped gases (TG = TGV-FRCMBNW). Furthermore ventilation inequalities can be estimated by mathematical analysis of the washout curve from which indexes such as the lung clearance index (LCI), the mean dilution number (MDN) and the moment ratio (m2:m0 = M-ratio) can be obtained. In 69 asthmatic children (age 5-17 y; 38 boys, 31 girls) body plethysmography and MBNW were performed in the symptom free interval. The questions were, at what extend TG are present within the different functional groups, and which parameters best describe ventilation inequalities. The group attribution was H: 23, M: 16, O: 30. The highest amount of TG was found in H (36.4 +/- 22.2% TGV), then in M (26.6 +/- 23.1% TGV) and in O (19.4 +/- 16.0% TGV). In 33/50 cases presenting with normal TGV, TG mainly was at cost of a low FRC (13 in H, 8 im M, 12 in O). In 19 cases FRC was higher than TGV (3 in H, 1 in M 15 in O). TG was closely related with LCI, MDN and M-ratio. Most pronounced ventilation inequalities were found in group M showing a correlation only with FRC, but not with TGV.(ABSTRACT TRUNCATED AT 250 WORDS)