Subacute sclerosing panencephalitis in a vaccinated, internationally adopted child.
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Biomedical subjects
Publications and source records attributed to J De Koster.
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Tuberculosis is a world-wide persistent problem. Treatment is usually successful when the bacteria are drug-susceptible and patient compliance ensured. We report our results of treatment of high-risk patients without social security coverage in Brussels. Double and triple fixed antituberculous agents in combined tablets were used. Cure was obtained without drug toxicity in all patients who completed therapy. Only 19.5% of patients failed to complete therapy.
To test the efficacy of a combined alpha- and beta-receptor agonist in acute bronchiolitis, we compared inhaled racemic epinephrine with salbutamol in a double-blind, crossover, randomized protocol. Twenty-four infants, 4.6 +/- 0.5 (mean +/- SEM) months of age, with their first episode of bronchiolitis were tested. After sedation with chloral hydrate, a clinical score and pulmonary mechanics measurements using simultaneous signals of airflow volume and transpulmonary pressure were recorded. After baseline measurements, infants received either nebulized salbutamol, 0.03 ml/kg, or racemic epinephrine, 0.1 ml/kg. Thirty minutes later, there was a significant decrease in clinical score after treatment with racemic epinephrine compared with the baseline score (p < 0.001); this difference was not present after salbutamol inhalation (p = 0.42). Only 13 patients had a decrease in clinical score after salbutamol therapy, in comparison with 20 infants treated with racemic epinephrine (p < 0.01). Both drug decreased respiratory rate, but the decrease was greater after the use of racemic epinephrine (p < 0.001). There was a significant decrease in inspiratory, expiratory, and total pulmonary resistance after treatment with racemic epinephrine compared with baseline values (p < 0.01) but no significant change after salbutamol inhalation. There was no significant correlation between the clinical score and pulmonary mechanics either at baseline or after drug treatment. We conclude that racemic epinephrine is superior to salbutamol in the treatment of infants with their first episode of acute bronchiolitis.
The effect of large doses of salbutamol (S) and ipratropium bromide (IB) were tested in a double-blind, randomized, crossover study. Nine patients with cystic fibrosis (CF), aged 12.8 +/- 2 years (mean +/- SE), were studied for 8 h on 2 separate days. Pulmonary function tests (PFTs) included spirometry (FEV1), lung volumes (FRC), and airway resistance (Raw) measured by body plethysmography. Heart rate (HR) and oxygen saturation (SaO2) were measured before each test. On 1 day patients received S 200 micrograms, S 400 micrograms, and IB 80 micrograms, by inhalation at 45-min interval (sequence A). On the other day, the sequence was IB 80 micrograms, S 200 micrograms, and S 400 micrograms (sequence B). The PFTs were obtained at baseline, 45 min after each inhalation, and 4 and 8 h after baseline measurements. Baseline PFTs (mean +/- SE) were not significantly different on the 2 study days (FEV1, 1.48 +/- 0.1 vs 1.42 +/- 0.1 L; FRC, 2.77 +/- 0.6 vs 2.87 +/- 0.6 L; Raw, 4.04 +/- 0.2 vs 4.00 +/- 0.3 cm H2O/L/s). The FEV1 and Raw improved from baseline after each inhalation, and at 4 and 8 h during both days (p < 0.05). Forty-five minutes after S 200 micrograms, plus S 400 micrograms, FEV1, FRC, and Raw were not significantly different compared with the values 45 min after IB 80 micrograms, plus S 200 micrograms (1.67 +/- 0.1 vs 1.63 +/- 0.1 L; 2.81 +/- 0.6 vs 2.65 +/- 0.5 L; and 2.98 +/- 0.2 vs 2.66 +/- 0.1 cm H2O/L/s, respectively). The PFTs were not significantly different after maximal doses of IB (80 micrograms) compared with S (600 micrograms). The HR and SaO2 were not significantly different from baseline throughout the study period. These results indicate that both single and sequential therapy have a similar acute bronchodilator effect provided that large doses are used. We speculate that adrenergic and muscarinic pathways are equally important in airflow obstruction in patients with CF.
Stridor is a frequent symptom in the neonate. Often it consists of a discrete inspiratory stridor in an alert child or of a transient symptom of a viral respiratory infection. In both cases no specific therapy is required. Whenever stridor occurs in the first months of life, or when it is severe or chronic, or when stridor also persists throughout expiration, an underlying anatomic anomaly frequently exists. The following steps are important to the diagnostic management: besides a detailed and complete history and physical examination, an X-ray of the airways and, if necessary, laryngo/bronchoscopy, angio(cardio)graphy or computerized tomography of the chest and the cervical region. Subsequently, appropriate therapeutic management can be initiated by proficient and accomplished physicians.
Transcutaneous needle aspiration (PTT) is a diagnostic technique which consists of biopsying pathological lung tissue using a needle (which in general is specifically designed for the purpose) which is introduced into the thorax transcutaneously. The target is lined up, either radioscopically or using a CT scanner and this enables the needle to be positioned in the centre of the lesion radiologically where one or more biopsies can be taken. According to the type and diameter of the needle these biopsies will enable a histological examination to be performed in addition to a cytological and bacteriological examination. This performance is particularly indicated in the final assessment of a periphery pulmonary nodule which is suspected of being neoplastic where bronchofibroscopy has been non contributory and where an immediate thoracotomy (diagnostic or therapeutic) is not recommended for one or other reason. When performed for this indication the technique often achieves a sensitivity of 90% with an average specificity of 98%. The diagnostic yield for benign tumours is less. It is always necessary to maintain a degree of vigilance in view of the risk of false negative or "benign" results. The complications exist above all of pneumothoraces which occur in 20-30% of cases and which will require a drain in 5-10% of cases. Haemorrhage and haemoptysis are less frequent but potentially fatal even in the absence of any coagulation disturbance.
Four clinical cases of pulmonary artery sling are reported. As an early recognition of this congenital anomaly can be life-saving for many infants, the most important elements in diagnosis and therapy are described.
The C syndrome of multiple congenital anomalies is described in a male infant with pseudohypoaldosteronism. The association of these 2 rare autosomal recessive conditions is discussed.