[Neonatal respiratory distress caused by primary ciliary dyskinesia]. / Neonatale respiratoire problemen door primaire ciliaire dyskinesie.

Two newborns, both boys, presented with unexplained respiratory distress. One developed recurrent pneumonias in the first neonatal week and was diagnosed with primary ciliary dyskinesia at the age of 2.5 years. The other had respiratory problems besides a situs inversus totalis and was diagnosed with primary ciliary dyskinesia in the neonatal period. Although 65-90% of children with primary ciliary dyskinesia present with neonatal respiratory distress, the disease is often diagnosed after a considerable delay. Primary ciliary dyskinesia should be considered in newborns with unexplained respiratory problems and in children with recurrent respiratory problems. The disease is diagnosed by taking a nasal brush biopsy of the cilia and examining it using electron microscopy or using phase contrast microscopy. Early diagnosis and adequate treatment may prevent further lung damage.

Pulmonary interstitial glycogenosis in identical twins.

We present the clinical, radiological, and pathological findings of open lung biopsies from monozygotic prematurely born male twins with respiratory distress at ages 6 and 8 weeks postnatally. Radiological examination showed a reticular nodular interstitial pattern on chest radiography. High-resolution computed tomography (HRCT) revealed ground-glass opacification and thickened interstitial septae in both infants. Lung biopsies showed a similar histology. There was diffuse interstitial thickening of the alveolar septa by mesenchymal cells, without prominent hyperplasia of type 2 pneumocytes, and without airspace exudates. Sections were periodic acid-Schiff (PAS)-positive within the cytoplasm of interstitial cells, indicating the presence of glycogen. Thus the diagnosis of pulmonary interstitial glycogenosis was made. Both infants were treated with glucocorticoids and had a favorable outcome. We speculate that pulmonary interstitial glycogenosis could be a histopathological form of chronic lung disease (CLD) of infancy.

Homologous bone graft for expansion thoracoplasty in Jeune's asphyxiating thoracic dystrophy.

Adult tibial shaft from a bone graft bank was used as a solid homologous bone graft for midsternal expansion thoracoplasty in an infant with Jeune's asphyxiating thoracic dystrophy. The technique appeared successful, but the child grew out of her chest in her second year of life. Expansion thoracoplasty for Jeune's disease probably should only be reserved for children who survive their first year of life without major surgery.

[Acute asthma in children; guidelines by pediatric pulmonologists for diagnosis and treatment]. / Acuut astma bij kinderen; richtlijnen van kinderlongartsen voor diagnostiek en behandeling.

The diagnostic phase in a child with acute asthma should be short and comprise a brief history-taking, inspection and auscultation of the thorax, transcutaneous oxygen measurement and, if possible, peak flow measurement. Blood picture. sputum culture and chest X-ray may be included in the diagnostics if indicated. The primary treatment consists of administration of bronchodilators (beta-2 sympathicomimetics) by inhalation, using a spacer. Repeated inhalation of salbutamol and ipratropium may be necessary. In case of inadequate improvement (spraying necessary every 3 hours for 24-48 hours), hospitalization and systemic administration of corticosteroids are indicated. Other reasons for hospitalization are a transcutaneous oxygen saturation lower than 91%, complications such as subcutaneous emphysema and pneumothorax, exhaustion of child or parents, and rapid aggravation of the clinical picture with rising Pco2 and falling pH in capillary or arterial blood.

Intractable or uncontrolled asthma: psychosocial factors.

The purpose of this study was to address the question of why, when there is a comparable severity of asthma, medical facilities, and treatments, some children develop controlled asthma whereas other children do not and are frequently ill. The major research questions pertained to whether families with a child with uncontrolled asthma differ from families with a child with controlled asthma as regards family characteristics and child-rearing attitudes, whether particular psychosocial variables relate to the severity of the asthma, and whether the interaction between the severity of the asthma and its controllability may clarify the role of psychosocial variables. Two studies were conducted, in which 70 asthmatic children (age range 9-15 years) and their families participated. The children and their caregivers were presented with measures assessing parental child-rearing attitudes, the problem-solving abilities of the caregivers, family functioning, and emotional disorders in the asthmatic children. Contrary to the assumptions derived from the psychosomatic family model, the findings of these studies suggest, among other things, that cohesion of family members and rigid manner of function of caregivers may have a positive rather than a negative influence on the welfare of the asthmatic child. In addition, controlled asthma was found to relate to the correct use of medication, which was predominantly evident in more structured and interdependent family environments. Of major importance is the conclusion that a distinction between controlled and uncontrolled asthma leads to a better insight into the role of psychosomatic variables than a distinction on the basis of the severity of the asthma.

Pemphigus vegetans in a child.

Pemphigus vegetans was diagnosed in a 12-year-old boy based on clinical, histopathological and immunohistological findings. To our knowledge, this is the first case of juvenile pemphigus vegetans to be published in the literature. Suggested treatment with prednisone and azathioprine was refused, and the patient was treated with a decoction of herbs prescribed by a practitioner of traditional Chinese herbal medicine. This treatment gave excellent results. Possible active components of the treatment are discussed.

Newly generated IgE antibodies to Dermatophagoides pteronyssinus in children are directed against components distinct from Der p I and Der p II.

The specificity of newly generated IgE antibodies (Abs) to the house dust mite, Dermatophagoides pteronyssinus, in longitudinal serum samples from 18 young children with an increased risk for IgE-mediated allergy was studied. The first IgE Ab response to house dust mite was detected early in life (mean age, 32 months; range, 11 to 60 months). For 83% of the children, more than half of the newly generated IgE Ab response to house dust mite was directed against components distinct from the major allergens, Der p I (Pl) and Der p II (DpX). These results suggest that the early IgE Ab response to house dust mite is induced by components distinct from the major allergens, Der p I and Der p II.

[Bleomycin and pulmonary function changes in children with malignant lymphomas]. / Bleomycine en longfunctieveranderingen bij kinderen met maligne lymfomen.

In 25 patients under 18 years of age with Hodgkin's disease or non-Hodgkin lymphoma treated with bleomycin as part of the treatment with several cytostatics, the diffusion capacity of the lung for carbon monoxide (DLCO) was determined before, during and after this treatment to investigate the damaging effect of bleomycin on the lungs. The DLCO decreased in 18 of the 25 children; the degree of decrease depended both on the total dosage (max. 120 mg/sq.m body surface) and on the dose per administration (5 or 10 mg/sq.m). Eight of these 18 children were followed up for some time after discontinuation of bleomycin treatment. During the relatively brief follow-up period of one year on average, complete recovery of pulmonary function was seen in none of these children; in two, partial recovery occurred. It is necessary to study the changes of DLCO for a longer period after bleomycin treatment, as well as the factors that influence recovery of pulmonary function in children.

Relationship between IgG1 and IgG4 antibodies to foods and the development of IgE antibodies to inhalant allergens. I. Establishment of a scoring system for the overall food responsiveness and its application to 213 unselected children.

To obtain reference levels for subsequent investigations, we analysed the IgG1 and IgG4 antibody levels to common foods in the sera of 213 unselected children (age 3 months to 14 years). The children were clustered into five age groups and tested on a broad screening panel of common foods. We used the IgG1 and IgG4 RAST with Sepharose-coupled antigens: cows' milk, hens' egg white, banana, legumes (a mixture of soybean and peanut), grains (a mixture of wheat and rice), potato, orange and pork. In all age groups and all antigens, a considerable variability in the antibody response was found. As for some assays more than half of the sera were negative or borderline, statistics based on interval or ordinal scaling were considered inappropriate and we resorted to nominal classification. We decided to use, for each of the assays, the 75-percentile of the age group as a cut-off level. Each antibody titre was thus converted into positive (more than the 75-percentile of that age group) or negative; the number of positive tests was used as the score. This resulted in a sigma G1-score and a sigma G4 score (summed scores for IgG1 and IgG4 antibodies, respectively). The results of the present study indicate that children with a high response to one food tend to have elevated responses to other non-related foods, possibly explained by a defective mucosal barrier and/or a hyperactive immune system. This suggests that a high-food responder phenotype may exist.

Relationship between IgG1 and IgG4 antibodies to foods and the development of IgE antibodies to inhalant allergens. II. Increased levels of IgG antibodies to foods in children who subsequently develop IgE antibodies to inhalant allergens.

In the present investigation we have tested the hypothesis that children with a high IgG antibody response to foods have an increased risk of developing IgE antibodies to inhalant allergens. Sera from 106 children with an increased risk of developing IgE-mediated allergy were analysed. During the follow-up, in 54 of these children IgE antibodies to inhalant allergens appeared. A positive/negative IgG1 and IgG4 anti-food score was determined as described previously: sera from age-clustered unselected children were tested for the levels of IgG1 and IgG4 antibodies to common foods. For each IgG RAST and each age group, the 75-percentile was chosen as cut-off value. Each antibody level was thus converted into a positive (higher than the 75-percentile of the age group) or negative value. The number of positive tests was used as the score. High-risk children with a high IgG1 anti-food score more often developed inhalant-specific IgE antibodies than high-risk children with low IgG1 titres: 50% of the children with a high IgG1 anti-food score developed IgE antibodies to grass pollen. Fifty per cent of the children with a high and 14% of the children with a low IgG1 anti-food score developed IgE antibodies to cat dander. For the prediction of the development of IgE anti-mite (house dust mite), the IgG4 anti-food scores appeared less useful than the IgG1 anti-food scores; 46% of the IgG4 high responders versus 22% of the IgG4 low responders acquired IgE anti-mite, whereas for IgG1 these percentages were 73 and 19, respectively.

Comparison of two nonculture techniques for detection of Hemophilus influenzae in sputum. In situ hybridization and immunoperoxidase staining with monoclonal antibodies.

Two nonculture methods, in situ hybridization and immunoperoxidase staining with monoclonal antibodies, were compared for the detection of Hemophilus influenzae in 184 sputa. For in situ hybridization, a biotin-labeled probe of total genomic DNA of H influenzae type b was prepared that hybridizes specifically with H influenzae, H parainfluenzae, H hemolyticus, and H parahemolyticus DNA. Immunoperoxidase staining was done with monoclonal antibody 8BD9 directed against outer membrane protein P6 of H influenzae. Both techniques detected Hemophilus in sputum equally well and were superior to culture: all 30 sputum samples culture-positive for H influenzae were positive on both nonculture tests, and 13 additional positive sputum samples were detected from which Hemophilus was not cultured. The higher sensitivity of the nonculture tests was mainly attributed to culture failure because of overgrowth of H influenzae by other bacteria, especially in patients with cystic fibrosis. The immunoperoxidase staining technique appeared slightly easier and quicker to perform than the in situ hybridization test. For the in situ DNA hybridization probe, DNA can be prepared from any strain of H influenzae. The immunoperoxidase test requires monoclonal antibody 8BD9 but has a higher specificity than the hybridization technique. Both techniques can be reliably applied, especially for the detection of Hemophilus in sputum of patients with cystic fibrosis.

In situ hybridization for the detection of Haemophilus in sputum of patients with cystic fibrosis.

In this study we performed in situ hybridization using biotin-labelled total genomic DNA of Haemophilus influenzae type b as a probe on: (1) smears containing bacteria cultured in vitro: all haemophilus species that can be found in the human respiratory tract appeared to be positive and a large number of other bacterial species appeared to be negative in this in situ hybridization test; (2) sputum smears from 287 patients with bronchitis: the hybridization test was positive on all but 2 of the 44 smears derived from patients whose culture yielded haemophilus and additionally on 12 smears derived from patients, whose culture was negative; and (3) sputum smears from 7 patients suffering from cystic fibrosis (CF): the hybridization test was positive in all these 7 sputum smears, while the culture only yielded haemophilus in 3 cases. The higher sensitivity of the hybridization test compared to culturing could mainly be explained by the failure to detect haemophilus in culture caused by masking due to overgrowth by other bacteria. In conclusion the in situ hybridization test, which can be performed in only 4 h, is a sensitive and specific method for the detection of haemophilus in sputum and is particularly useful in CF patients, where overgrowth by pseudomonas often interferes with diagnosis by culturing.

[Drug treatment of children with CARA]. / Medicamenteuze behandeling van kinderen met CARA.

In the following article a review is given of the pharmacotherapeutic possibilities for the treatment of asthma in children. Both the treatment of chronic wheezing and the severe asthma attack are dealt with, with as much emphasis as possible on the practical approach. The order of choice of the medicaments for the various forms of asthma is shown.