Painful neck mass in a five-year-old boy. Acute suppurative thyroiditis.
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Child, Preschool↗
Biomedical subjects
K De Boeck
Publications and source records attributed to K De Boeck.
Ultrastructural expression of primary ciliary dyskinesia after ciliogenesis in culture.
During the period 1990-1999 84 PCD patients were identified and characterized. The expression of inherited abnormalities in primary ciliary dyskinesia after ciliogenesis was investigated in 41 patients with dynein deficiency, 6 patients with absence of the central pair of microtubules and 24 PCD patients with normal ultrastructure. In patients with dynein deficiency, the outer dynein arms counts were 1.9 +/- 1.0 in the biopsies and 1.6 +/- 0.7 after ciliogenesis. Secondary abnormalities were found in 15.8 +/- 20.4% of the transverse sections of cilia and only in 1.0 +/- 1.3% after ciliogenesis. Ciliary orientation was 28 +/- 11 degrees and 24 +/- 10 degrees respectively in biopsies and cultures. In patients with absence of the central pair this was found in 15 +/- 16% in biopsies and 21 +/- 19% after ciliogenesis. The values for the outer dynein arm were 8.4 +/- 0.3 and 8.7 +/- 0.2 and for the secondary abnormalities were 11.7 +/- 7.3% and 0.5 +/- 1.3% in the biopsies, respectively after ciliogenesis. In patients with normal ultrastructure the scores for the dynein arms were similar. Secondary abnormalities were found in 12.2 +/- 11.7% in the biopsies and 0.6 +/- 0.9% after ciliogenesis while ciliary orientation was respectively 21 +/- 7 degrees and 25 +/- 8 degrees. In conclusion, inherited abnormalities in primary ciliary dyskinesia are expressed after ciliogenesis, while secondary abnormalities are virtually absent, thereby facilitating the ultrastructural diagnosis.
Biopsy↗
Failure of local defense mechanisms in cystic fibrosis.
Cystic fibrosis (CF) is an hereditary disease with pancreatic insufficiency and chronic respiratory tract infections leading to irreversible lung damage as its main features. The typical pathogen for the respiratory disease is Pseudomonas aeruginosa. Although the insight into the pathophysiology and genetics of CF have evolved over the past 10 years, there is not yet a full understanding of the increased susceptibility for respiratory infections, specifically with Pseudomonas aeruginosa. In this overview we elucidate the current knowledge and describe the existing hypothesis on the possible links between the pathophysiological defects and the failure of the local defense mechanism in CF airways.
Chronic Disease↗
Is the correct use of a dry powder inhaler (Turbohaler) age dependent?
BACKGROUND: The metered-dose inhalers are the most commonly used devices in the treatment of asthma, but dry powder inhalers (eg, Turbohaler) are being increasingly used. Studies evaluating how well children can use a Turbohaler are lacking. OBJECTIVE: We assessed whether the correct use of a Turbohaler could be easily taught to unselected stable asthmatic children. METHODS: One hundred sixty-one asthmatic children aged 5 to 17 years (mean, 9.8 years) consecutively attending the outpatient clinic were included in study. After a demonstration and 10 minutes of training, the inhalation technique was checked in a standardized way (yes/no response). Keeping the device upright, proper preparation of the drug dose and inspiratory flow on inhalation were measured by the Turbohaler trainer. RESULTS: One hundred thirty-three children (83%) performed every step correctly (ie, 96% of children older than 8 years but only 55% of children between 5 and 8 years; P <.001). Of 28 children incorrectly using the Turbuhaler-trainer, 20 generated insufficient inspiratory flow through the device. There was no significant difference in airway obstruction (expressed as percent of predicted forced vital capacity, FEV1, and Tiffeneau index) between correct and incorrect users, but when measured through the pneumotachograph, mean peak inspiratory flow (expressed as percent predicted) was significantly lower in those children incorrectly using the device. Turbohaler use was reevaluated after 4.7 +/- 2.0 months in a subset of 64 patients. Fifty-three of 64 (83%) children again used the device correctly. Only 3 of 13 who used the device incorrectly at the first evaluation used it correctly at the second evaluation. CONCLUSIONS: We conclude that the correct use of the Turbohaler can be easily taught to asthmatic children older than 8 years. Those who use the device correctly after initial instructions continue to do so afterwards.
Asthma↗
Genotype-phenotype correlations for the paranasal sinuses in cystic fibrosis.
Genotype-phenotype correlations in cystic fibrosis (CF) have been found for lung and pancreatic function, but not for paranasal sinus disease. Because such correlations may have pathophysiological and clinical implications, the correlation of mutations, in particular DeltaF508, with paranasal sinus disease was investigated in 113 CF patients with known genotype. The clinical importance of paranasal sinus disease was evaluated using three parameters: polyps, overall clinical severity of upper airway problems, and surgery. Polyps were evaluated by nasal endoscopy and graded on a five-point scale. Four severity groups were distinguished based on history, clinical records, and examination: no upper airway problems; more problems than in control subjects; severe, recurrent or chronic problems; and paranasal sinus surgery cases. DeltaF508 homozygosity correlated with clinical severity (p < 0.02) and with the presence of polyps on endoscopy (p < 0.05). The relative risk for paranasal sinus surgery in DeltaF508 homozygous CF patients was 2.33. In conclusion, there are genotype-phenotype correlations for paranasal sinus disease in CF. DeltaF508 homozygosity is a risk factor for paranasal sinus disease in CF.
Adolescent↗
Middle ear disease in cystic fibrosis.
A prospective cross-sectional study was done to investigate the involvement of middle ear disease in cystic fibrosis. Eighty-eight patients with cystic fibrosis, aged 1-28 years, were examined during the winter period 1995-1996 at regular follow-up visits to detect history and prevalence of inflammatory and infectious middle ear disease by questionnaire, otoscopy and tympanometry. One third of the patients had a history of otitis media, but only in 9% did this occur more than once. Hearing problems were recorded in 8%, but this was severe in only one case. Otoscopy showed 141 normal tympanic membranes, 17 times retraction, 15 times otitis media with effusion, once an acute otitis media in both ears. Only one patient had a grommet in one ear. More than 70% of the tympanograms were normal. A flat curve, type B according to Jerger's classification, was found in 11 ears. The youngest age group had a higher prevalence of abnormal findings on otoscopy and tympanometry, while patients who had undergone paranasal sinus surgery had less frequently abnormal findings. In conclusion, no evidence that cystic fibrosis is associated with increased prevalence of middle ear disease has been found in this prospective cross-sectional study.
Acoustic Impedance Tests↗
Response to bronchodilators in clinically stable 1-year-old patients with bronchopulmonary dysplasia.
UNLABELLED: Bronchodilators are often used in the treatment of patients with bronchopulmonary dysplasia (BPD). However, few studies evaluate their efficacy in patients with stable disease beyond the newborn period. Therefore, pulmonary function was measured before and after aerosol treatment with salbutamol (0.25 ml Ventolin 0.5%) and subsequently after aerosol with ipratropium bromide (0.25 ml Atrovent 0.025%). Studies were performed at the corrected postnatal age of 52 +/- 2 weeks in 52 patients who had been ventilated after birth because of newborn lung disease. Twenty-two of these 52 patients had developed BPD. Pulmonary function was measured after sedation and using the PEDS system. Expiratory resistance (median 52.1 versus 39.1 cmH2O/l/s; P < .008) and inspiratory resistance (median 42.5 vs 27.8 cmH2O/l/s; P < .04) were significantly worse in BPD patients at the age of 1 year. Half of the BPD patients had a decrease in pulmonary resistance after salbutamol. However, there was no statistically significant decrease in pulmonary resistance after salbutamol or ipratropium in the BPD patients as a group. After salbutamol pulmonary resistance significantly worsened in the patients who did not develop BPD. CONCLUSION: Although individual patients may benefit, routine administration of bronchodilators seems not warranted in stable BPD patients at the age of 1 year.
Administration, Inhalation↗
Primary ciliary dyskinesia: evolution of pulmonary function.
UNLABELLED: Pulmonary function tests were obtained in 11 patients with primary ciliary dyskinesia. Their mean age was 15 years (range 6-32). Their pulmonary function was obstructive, with a vital capacity (mean+/-SD) of 75%+/-20% predicted, a forced expiratory volume in 1s (FEV1) of 63%+/-20% predicted and a raised residual volume of 169%+/-50% predicted. After inhalation of 200 microg of salbutamol the mean change in FEV1 was + 13.2%+/-9.6% of the baseline value. In the 10 oldest patients, lung function had been measured at regular intervals during 3 20 years. Interestingly, during childhood and adolescence the evolution was not unfavourable: vital capacity increased by 8%+/-20% and FEV1 remained stable (mean change 0.3%+/-12%). Only 2 patients had an unfavourable evolution. CONCLUSION: At time of diagnosis, patients with primary ciliary dyskinesia have partially reversible obstructive airway disease. During regular follow up and therapy, there is no evidence of a further decline in lung function. Patients with associated immunodeficiency or important damage at the start of therapy may have a worse prognosis.
Adolescent↗
Treatment of Pseudomonas aeruginosa lung infection in cystic fibrosis with high or conventional doses of ceftazidime.
In cystic fibrosis patients with Pseudomonas aeruginosa colonization and increasing pulmonary infection, ceftazidime 150 mg/kg/day was compared with 320 mg/kg/day. Changes in clinical findings, laboratory tests, pulmonary function and chest radiographs were evaluated after 14 days of treatment in hospital. Both treatments were associated with a significant improvement, but the higher dose did not offer an additional benefit. An increase in alanine aminotransferase (ALT) occurred after both treatments; with a significantly greater increase after the high-dose therapy (mean increase +/- S.E.M. 8% +/- 2% vs 2% +/- 1 %; P < 0.01). All but one of the ALT values after treatment were within normal limits.
Adolescent↗
Lipid digestion in cystic fibrosis: comparison of conventional and high-lipase enzyme therapy using the mixed-triglyceride breath test.
BACKGROUND: Fat maldigestion occurs in most patients with cystic fibrosis. Conventional pancreatic enzyme replacement therapy partially corrects this defect. In this study, the mixed-triglyceride breath test was used to evaluate whether high-lipase enzymes are equivalent to conventional enzymes in improving fat maldigestion in children with cystic fibrosis. METHODS: Fat digestion was studied in 11 patients with a mean age of 10.5 years. The mean intake of conventional enzyme capsules a day was 19. Four 13C mixed-triglyceride tests were performed on separate days and in random order. One test was taken without enzyme substitution, one with three capsules of 8,000 FIP units Creon (pancreatinum, Kali-chemie Pharma, Hannover, Germany) and one with one capsule of 25,000 FIP units. The fourth test was made with 13C octanoic acid to study gastric emptying time. RESULTS: Without enzyme intake, the mean cumulative percentage of 13C dose exhaled after 6 hours was 7.2+/-3.7%. This increased to 14.4+/-4% with intake of conventional pancreatinum and to 14.3+/-5.1% with intake of high-lipase pancreatinum (p = 0.0008 for both; paired t-test). There was no difference between both treatments. Also, the time course of 13C exhalation measured by percentage of 13CO2 exhaled per hour did not differ between enzyme treatments. CONCLUSIONS: The 13C mixed-triglyceride test is noninvasive and documents improved lipid digestion with pancreatic enzyme replacement therapy. If the lipase dose is kept constant, results obtained with high-lipase preparations are equivalent to those obtained with conventional preparations.
Adolescent↗
Bronchodilator response in 3-6.5 years old healthy and stable asthmatic children.
Few data are available on the bronchodilator response in preschool children. This study was set up to study baseline lung function and bronchodilator responses in healthy and asthmatic children younger than 7 yrs old. In 281 preschool children attending kindergarten (age range 2.7-6.6 yrs old) respiratory system resistance (Rrs) and reactance (Xrs) by impulse oscillation system at 5, 10, 15, 20, 25 and 35 Hz as well as resonance frequency (f0) were measured before and 20 min after 200 microg inhaled salbutamol by a metered-dose inhaler connected to a spacer device. Thirty-four of them were diagnosed as asthmatics based on a validated standardized questionnaire. The mean Rrs (+/-SD) at 5 Hz (Rrs,5) was 1.03 (+/-0.24) kPa x L(-1) x s for healthy children and 1.09 (+/-0.26) kPa x L(-1) x s for stable asthmatics. The mean change in Rrs,5 after salbutamol was -0.13 (+/-0.20) kPa x L(-1) x s for the healthy children and -0.09 (+/-0.25) kPa x L(-1) x s for the asthmatic group. The scatter for the measurements was striking. Neither baseline values of impulse oscillation nor its changes after bronchodilator administration was significantly different between healthy and stable asthmatic children. A change in respiratory system resistance at 5 Hz of 40% is to be considered the cut-off for a " positive" bronchodilator response.
Adrenergic beta-Agonists↗
No paradoxical bronchodilator response with forced oscillation technique in children with cystic fibrosis.
STUDY OBJECTIVES: The aim of the present study was to evaluate the forced oscillation technique (FOT) in cystic fibrosis (CF) children and to participate in the discussion about the usefulness of beta2-antagonists in CF. DESIGN: Pulmonary function was measured with spirometry, body plethysmography, and FOT before and after inhalation of 200 microg of albuterol (salbutamol). The following were collected: vital capacity (VC), FEV1, FEV1/VC, airway resistance (Raw), thoracic gas volume, respiratory system resistance (Rrs) and respiratory system reactance (Xrs) at 6 Hz (Rrs6 and Xrs6), and resonance frequency. SETTING: The study was set up at a university hospital with a CF population of 125 children and adolescents. PATIENTS: Data were collected on 20 patients in stable condition able to perform the three lung function tests. MEASUREMENTS AND RESULTS: Mean baseline values (+/-SD) were 0.36+/-0.15 kPa/L/s for Raw, 0.5+/-0.15 kPa/L/s for Rrs6, and 61+/-22% predicted for FEV1. The relationship between FEV1 and Raw or Rrs6 was poor. Xrs6 and FEV1/VC correlated weakly (r=0.56; p < 0.05). After bronchodilator administration, the mean changes +/-SD in percent of baseline were +3 +/- 11% for FEV1, -16 +/- 22% for Raw, and -16 +/- 9% for Rrs6. In six patients, a paradoxical decrease in FEV1 was measured but an increase in Rrs6 was never found; in two patients, an increase of Raw of < 10% was found. In 13 patients, the decrease of Rrs6 was > 12%. CONCLUSIONS: The results suggest that FOT measurements cannot replace baseline spirometric measurements in CF, but that the evaluation of the effect of beta2-agonists on the airway diameter in CF should include an FOT measurement.
Administration, Inhalation↗
Bronchopulmonary dysplasia: correlation of radiographic and clinical findings.
BACKGROUND AND PURPOSE: Abnormalities of the chest wall have been described in bronchopulmonary dysplasia (BPD). Clinical, radiographic and pulmonary function variables were evaluated in 1-year-old children ventilated because of neonatal lung disease in order to quantify these thoracic changes and to evaluate the lung disease. METHODS: The pulmonary status of 51 infants with neonatal lung disease requiring artificial ventilation was reevaluated clinically and radiographically at the age of 1 year. Twenty-two of these infants had developed BPD. Thoracic depth and width were measured clinically and on chest X-ray. The Toce score evaluated the presence of cardiomegaly, hyperinflation, emphysema and interstitial lung disease. Lung function was measured after sedation using previously reported methods. In BPD patients, Toce score and lung function were determined and compared at 1 month and at 1 year of age. RESULTS: In BPD patients, chest depth was significantly smaller when measured clinically as well as on chest radiograph (P < 0.05; Mann-Whitney U-test). There was a statistically significant correlation between chest depth measured clinically and on chest X-ray. Toce score was significantly higher in BPD patients (P < 0.05). In BPD patients intersitial abnormalities and decreased lung compliance were more frequent at the age of 1 month than at the age of 1 year. At the age of 1 year, hyperinflation was more frequent and at that time increased airway resistance was still noted. Thus the type of X-ray abnormality reflects the type of lung function disturbance. CONCLUSION: The flatness of the chest is most likely a consequence of the long-standing lung function abnormalities.
Bronchopulmonary Dysplasia↗
Respiratory syncytial virus bronchiolitis: a double-blind dexamethasone efficacy study.
The efficacy of dexamethasone therapy for primary respiratory syncytial virus bronchiolitis was studied in a double-blind placebo design in 29 previously healthy infants (median age, 194 days). No significant differences were found between the groups in evolution of respiratory rate, oxygen saturation, clinical score, or pulmonary function tests on day 3.
Analysis of Variance↗
DiGeorge syndrome and unilateral symbrachydactyly.
We describe a child with DiGeorge syndrome due to a del22(q11) and a symbrachydactyly of the left hand. The underlying cardiovascular malformations in DiGeorge syndrome may lead to an increased susceptibility to vascular disruptions, giving further support to the subclavian-artery-disruption-sequence theory.
Arm↗
Early ribavirin treatment did not prevent severe disease in high-risk bronchopulmonary dysplasia patients with respiratory syncytial virus infection.
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Antiviral Agents↗
Burkholderia (Pseudomonas) cepacia and cystic fibrosis: the epidemiology in Belgium.
Burkholderia cepacia has become an increasingly recognized pathogen among cystic fibrosis (CF) patients and its potential role in declining pulmonary function or unexpected fatal outcome has caused widespread concern. Direct person-to-person transmission has been documented and a segregation policy of CF patients colonized with B.cepacia from non-colonized CF patients is widely adopted. Since this policy has a dramatic impact on social behaviour of CF patients it is imperative that clinical laboratories accurately isolate and identify B.cepacia in the respiratory secretions. In order to comprehend the epidemiology of B.cepacia in the Belgian CF population a multicentre study was conducted during a period of 1 year (March'93-February'94). B.cepacia was isolated in only 12 of 465 CF patients (2.6%). Routine biochemical tests identified these strains as authentic B.cepacia. However, the combined data from protein and DNA-DNA hybridization analyses revealed that the Belgian CF "B.cepacia" isolates showed patterns different from reference B.cepacia isolates and belong to 3 different, newly identified Burkholderia genomovars, but not to B.cepacia. Comparative analysis of the selective media used for recovery of these "B.cepacia" strains from respiratory secretions indicated that the commercial medium (Mast) containing polymyxin B and ticarcillin as the selective agents was the best and most user-friendly. Molecular typing of these Burkholderia isolates by arbitrarily-primed PCR (AP-PCR) and pulsed-field gel electrophoresis (PFGE) showed that spread of a single strain within a same centre occurred but the mode of transmission remains unknown; inter-centre spread of strains was not observed. Interestingly, neither colonization with a distinct or an epidemic strain (belonging to either of the three newly identified Burkholderia genomovars) nor colonization for a prolonged period of time, led to a rapid deterioration of lung function in these CF patients. It appears essential to determine the prevalence of these "new" Burkholderia genomovars in larger populations of CF patients and to evaluate their virulence and other features as this may have important clinical and practical implications.
Adolescent↗
Tracheal bronchus associated with congenital cystic adenomatoid malformation.
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Bronchi↗