Pulmonary function in children with homozygous alpha1-protease inhibitor deficiency.

Unlabelled: Alpha1-protease inhibitor (alpha1-PI) deficiency is a well-recognized cause of emphysema in adults; however, the natural history of this disorder in children is unclear. Because of the paucity of data in the paediatric age group, we performed whole body plethysmography, spirometry, and diffusing capacity, in a cohort of 17 homozygous (PiZZ phenotype) children (9 females, 8 males; mean age +/- SEM 13.4 +/- 0.

Two cases of pulmonary tuberculosis presenting with unilateral pulmonary hyperinflation in infancy.

Unlabelled: Two infants with recurrent obstructive symptoms attracted attention because of massive radiologically detected unilateral pulmonary hyperinflation. Further diagnostic procedures including bronchoscopy, revealed a pulmonary tuberculosis with lymph nodes encroaching on the bronchi. Steady improvement of clinical symptoms and hyperinflation was noted under combined antituberculotic therapy including systemic steroids.

Individual time-courses of ECP and EPX during allergen provocation tests in asthmatic children.

To study the time-course of eosinophil cationic protein (ECP) and eosinophil protein X (EPX) during bronchial allergen provocation, we investigated 32 asthmatic children sensitive to house-dust mites as well as 6 non-atopic young adult controls. In all subjects, allergen challenges were performed with house dust mite extracts of Dermatophagoides pteronys-sinus or Dermatophagoides farinae. Blood samples were taken at regular intervals during the 24-h observation period.

Myeloperoxidase and eosinophil cationic protein in serum and sputum during antibiotic treatment in cystic fibrosis patients with Pseudomonas aeruginosa infection.

I order to study the time-course of myeloperoxidase (MPO) and eosinophil cationic protein (ECP) as parameters for monitoring inflammation in cystic fibrosis (CF), we investigated ten patients during both a 14-day intravenous antibiotic treatment and a corresponding self control. Modified Shwachman-Kulczycki score improved significantly (p < 0.008), C-reactive protein (CRP) levels decreased significantly (p < 0.

The role of eosinophils and eosinophil cationic protein in monitoring oral challenge tests in children with food-sensitive atopic dermatitis.

To investigate the role of peripheral blood eosinophils and eosinophil cationic protein as parameters in monitoring oral food challenges, we monitored 25 infants and children with atopic dermatitis for up to 48 hours after 47 placebo-controlled oral food challenges with cow's milk, hen's egg, cow's milk and hen's egg, or placebo for up to 48 hours. Six healthy young nonatopic adult volunteers served as control subjects. Compared with baseline values, peripheral blood eosinophils decreased significantly immediately after clinical reaction in positive challenges (p < 0.

Discrepancies between in vitro and in vivo tests for house dust mite allergy: in domestic exposure a better predictor than sensitization?

We subjected seven asthmatic children to two bronchial allergen challenges, first with an extract from the house dust mite Dermatophagoides pteronyssinus (Der p) and then Dermatophagoides farinae (Der f), or vice versa. All children had elevated specific serum IgE to both species as well as reactions by crossed radioimmuno/electrophoresis (CRIE) to both group I and II allergens from both species. Immunoabsorption and subsequent analysis by CRIE showed a considerable concentration of serum IgE with specificity for epitopes common to the two species of house dust mite.

Twenty-four-hour time course of eosinophil granule proteins ECP and EPX during bronchial allergen challenges in serum of asthmatic children.

To study in vivo monitoring variables for bronchial allergen challenges, we investigated the time course of the eosinophil granule proteins, eosinophil cationic protein (ECP) and eosinophil protein X (EPX) after allergen provocation in serum. Thirty-two asthmatic children sensitive to house-dust mites and six healthy young adult controls were challenged by bronchial allergen provocations with Dermatophagoides pteronyssinus and D. farinae.

Asymptomatic pulmonary involvement in 2 children with Niemann-Pick disease type B.

We present 2 cases of children with Niemann-Pick disease type B, and surprisingly with reticular lung infiltrations, although otherwise healthy in terms of pulmonary manifestation. Finding such reticular infiltrations should lead one to think of the possibility of metabolic storage disease.

Lymphocyte subsets, sIL2-R and sICAM-1 in blood during allergen challenge tests in asthmatic children.

In order to study cell activation in peripheral blood on bronchial allergen provocation up to 24 h, we investigated 32 asthmatic children, sensitive to house-dust mites. Six healthy young adult volunteers served as controls. Lymphocyte subsets (CD3, CD19, CD4, CD8) and activation markers (CD25-T, HLADR-T, CD23) in peripheral blood as well as soluble IL2-R and soluble ICAM-1 in serum were evaluated.

Diurnal variation of urinary histamine and 1-methylhistamine excretion in healthy children.

In order to establish a noninvasive method of monitoring immediate hypersensitivity reactions in children, we studied the diurnal variation of urinary histamine and 1-methylhistamine excretion and the influence of food intake in a group of 14 healthy nonatopic children (aged 2-16 years). Histamine and 1-methylhistamine in spontaneous urine samples were determined by radioimmunoassay. Mean variation of 2-h intervals was much higher for urinary histamine than for 1-methylhistamine (45% of base-line level versus 24%).

Abnormal length of cilia--a cause of primary ciliary dyskinesia--a case report.

A 7-year-old Turkish boy had suffered from chronic coughing from early childhood. Severe bronchiectasis in the right lung was confirmed by bronchography. Ciliary beat frequency determined in a bronchial mucosal biopsy was markedly decreased (5.

Three cases of adolescent near-fatal asthma: what do they have in common?

Three adolescent asthmatics are described, who suffered from near fatal asthmatic attacks. The events occurred suddenly and required intubation and ventilation. However, the bronchial obstruction was fully reversible within less than 24 hours and the recovery complete.