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Biomedical subjects

J De Blic

Publications and source records attributed to J De Blic.

At least 19 recordsLinked to original sources
PubMed2005

[Practical advice for exercise-induced asthma in children: experience of the exercise training centre of Necker-Enfants malades hospital].

Now, to care exercise-induced asthma is not only to prescribe drugs. It is a global and interdisciplinary approach: the pulmonary rehabilitation, matching a therapeutic education and a physical training, with the goal of promoting a regular physical activity in the asthmatic child, achieving physiological benefits and improvement of quality of life. Getting from the experience of Necker-Enfants Malades Hospital's Training Centre, a few advises encourage the physical practice of the asthmatic child, and decrease risks of exercise-induced asthma: optimisation of treatments;progressive beginning and end of exercises; use of the diaphragmatic breathing, keeping up with the exercise; use of the ventilatory threshold (or dysponea threshold) as intensity of the aerobic training; practice of different activities promoting play and conviviality in sports and allowing the integration of sports in the daily life of the asthmatic child.

Adolescent↗
PubMed2003

[Streptococcus pneumoniae purulent pleurisy and hemolytic uremic syndrome. A case report].

A 22-month-old infant developed purulent pleurisy caused by Streptococcus pneumoniae and a hemolytic uremic syndrome. The diagnosis was suggested by the classical triad: hemolytic anemia, renal failure and thrombocytemia confirmed by renal biopsy which demonstrated extensive cortical necrosis. Renal involvement was severe, justifying an indication for renal transplantation.

Hemolytic-Uremic Syndrome↗
PubMed2002

[The AIRE Study: data analysis of 753 European children with asthma].

PROBLEM: To analyse the paediatric data from the AIRE study (Asthma insights and reality in Europe) describing the standard of asthma control in Europe. MATERIAL AND METHODS: Among the 73,880 households contacted by telephone in 7 countries 753 children under the age of 16 were identified. An interview was conducted with a close relative to determine the frequency and severity of symptoms during the recent month, visits to casualty and hospital admissions during the past year, the medications used, the effects on the lives of the children, the opinions of the parents on the level of control of asthma and the monitoring methods used. RESULTS: 38.2% of children reported diurnal symptoms and 28% disturbance of sleep at least once a week. The consequences were important: limitation of sporting activities (29.5%) and absence from school (42.7% in the past year). Treatment was inadequate: 26% of children used inhaled steroids yet 45.9% had persistent asthma. The level of control was overestimated: 61% of parents of children with severe persistent asthma considered the asthma to be well controlled. CONCLUSION: European asthmatic children are poorly controlled, under-treated and rarely followed up. The consequences, particularly absence from school, should stimulate an improvement in their therapeutic and educational management.

Absenteeism↗
PubMed2001

Clinical biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism.

Pulmonary surfactant is a multimolecular complex located at the air-water interface within the alveolus to which a range of physical (surface-active properties) and immune functions has been assigned. This complex consists of a surface-active lipid layer (consisting mainly of phospholipids), and of an aqueous subphase. From discrete surfactant sub-fractions one can isolate strongly hydrophobic surfactant proteins B (SP-B) and C (SP-C) as well as collectins SP-A and SP-D, which were shown to have specific structural, metabolic, or immune properties. Inborn or acquired abnormalities of the surfactant, qualitative or quantitative in nature, account for a number of human diseases. Beside hyaline membrane disease of the preterm neonate, a cluster of hereditary or acquired lung diseases has been characterized by periodic acid-Schiff-positive material filling the alveoli. From this heterogeneous nosologic group, at least two discrete entities presently emerge. The first is the SP-B deficiency, in which an essentially proteinaceous material is stored within the alveoli, and which represents an autosomal recessive Mendelian entity linked to the SFTPB gene (MIM 1786640). The disease usually generally entails neonatal respiratory distress with rapid fatal outcome, although partial or transient deficiencies have also been observed. The second is alveolar proteinosis, characterized by the storage of a mixed protein and lipid material, which constitutes a relatively heterogeneous clinical and biological syndrome, especially with regard to age at onset (from the neonate through to adulthood) as well as the severity of associated signs. Murine models, with a targeted mutation of the gene encoding granulocyte macrophage colony-stimulating factor (GM-CSF) (Csfgm) or the beta subunit of its receptor (II3rb1) support the hypothesis of an abnormality of surfactant turnover in which the alveolar macrophage is a key player. Apart from SP-B deficiency, in which a near-consensus diagnostic chart can be designed, the ascertainment of other abnormalities of surfactant metabolism is not straightforward. The disentanglement of this disease cluster is however essential to propose specific therapeutic procedures: repeated broncho-alveolar lavages, GM-CSF replacement, bone marrow grafting or lung transplantation.

Adult↗
PubMed2001

[Endoscopic exploration of the airways in children].

Airway endoscopic exploration in children has benefited from miniaturization of flexible bronchoscopes, allowing examination in young and even premature infants. Whether endoscopy is performed under conscious sedation and topical anesthesia or under deep sedation, the painlessness of the procedure are very important for the child's comfort. Bronchoscopy may provide direct anatomical information or indirect information from secretions sampling, bronchoalveolar lavage, or bronchial and transbronchial biopsies. Indications are wide, mainly detection of airway obstruction via an intrinsic or extrinsic mechanism, identification of an infectious agent in immunocompromised patients, exploration of chronic interstitial pneumonitis, and interventional endoscopy.

Adolescent↗
PubMed2000

Long-term outcome of idiopathic pulmonary hemosiderosis in children.

We retrospectively analyzed the long-term outcome of idiopathic pulmonary hemosiderosis (IPH) in 15 children. IPH started at a mean age of 5 years, and the mean duration of follow-up was 17.2 years (range, 10-36 yr). Four patients developed immune disorders, 3 cases of rheumatoid polyarthritis or rheumatoid polyarthritis-like diseases and 1 case of celiac disease. Respiratory outcome showed that 3 patients had severe symptoms: 2 patients developed severe pulmonary fibrosis resulting in major chronic respiratory insufficiency, and 1 patient had severe asthma. Twelve patients (80%) had mild or no respiratory problems and were able to lead a normal life. According to chest X-ray and pulmonary function test data, 4 patients had normal chest X-ray and no evidence of restrictive syndrome, 6 patients had an interstitial pattern on chest X-ray and evidence of restrictive pattern, 1 patient had an interstitial pattern but normal lung function, and 1 patient had a normal chest X-ray but evidence of mixed obstructive and restrictive pattern. Our results show that long-term survival is possible in patients with IPH. Factors of poor prognosis seem to be the presence of antineutrophil cytoplasm antibodies (ANCA) or other autoantibodies. No other clinical or biological predictive factors for prolonged survival were found.

Adolescent↗
PubMed2000

[Asthma and the child below 5 years of age: diagnosis and treatment].

Asthma is the most frequent chronic disease in pediatrics and the increase in its prevalence is a major public health problem. Diagnosis may be difficult in the young child, symptomatology most often occurring following a viral infection. It is important not to ignore a foreign body in the airways or fibrocystic disease and asthma remains, particularly in infants, a diagnosis of elimination. Misdiagnosis or insufficient treatment of asthma may risk the development of irreversible histological lesions and also could compromise pulmonary growth and the child's lung reserve. Spacer devices and nebulisers enable inhaled therapy to be administered to very young children. The value of early diagnosis is to institute appropriate treatment notably in severe asthma with inhaled corticosteroid therapy, the aim being to reduce remodelling lesions of the airways. The minimal effective dose should be defined to minimalise side-effects. The treatment of asthma is not restricted to pharmacotherapy: attempts should be made to reduce intercurrent viral infections, domestic pollution (including smoking) and allergenic concentrations. However, as for all chronic diseases, the clinician will encounter poor compliance. The work of education and support of health professionals is fundamental to the management of asthma.

Age Factors↗
PubMed1999

CT scan patterns of pulmonary alveolar proteinosis in children.

BACKGROUND: To study computed tomographic (CT) findings in children with pulmonary alveolar proteinosis (PAP) more extensively. OBJECTIVE: To describe the CT features at the time of diagnosis and after therapeutic broncho-alveolar lavage (BAL). MATERIALS AND METHODS: We retrospectively reviewed the CT scans of five children (aged 3 months to 4 years) examined because of incidental bronchitis (n = 1), disease in a sibling (n = 1) and relapsing fever, cough and dyspnoea (n = 3). Each patient had an initial CT scan. Two asymptomatic cases were not treated but were followed up by plain chest films. The other three had BAL and follow-up CT. RESULTS: Initial CT in all cases showed a diffuse reticulomicronodular pattern associated in three cases with posterior bilateral alveolar infiltrates. CT in the two asymptomatic patients remained unchanged or slightly improved without BAL. After BAL, a variable decrease of lung infiltrates was observed. CONCLUSIONS: Correlation between the extent of alveolar consolidation and severity of disease was found. Anatomical and pathological considerations allow us to consider that the classical reticulomicronodular pattern is not due to an interstitial infiltration but to alveoli filled with the abnormal material characteristic of PAP.

Biopsy↗
PubMed1999

Early and prolonged intravenous immunoglobulin replacement therapy in childhood agammaglobulinemia: a retrospective survey of 31 patients.

OBJECTIVE: To evaluate the outcome of children who received prolonged intravenous immunoglobulin (IVIg) replacement therapy early in life for X-linked agammaglobulinemia (XLA). STUDY DESIGN: We performed a retrospective study of the clinical features and outcome of patients with genetic and/or immunologic results consistent with XLA. Patients receiving IVIg replacement therapy within 3 months of the diagnosis and for at least 4 years between 1982 and 1997 were included. RESULTS: Thirty-one patients began receiving IVIg replacement therapy at a median age of 24 months and were followed up for a median time of 123 months. IVIg was given at doses >0.25 g/kg every 3 weeks, and mean individual residual IgG levels ranged from 500 to 1140 mg/dL (median, 700 mg/dL). During IVIg replacement, the incidence of bacterial infections requiring hospitalization fell from 0.40 to 0.06 per patient per year (P <. 001). However, viral or unidentified infections still developed, including enteroviral meningoencephalitis (n = 3) causing death in one patient, exudative enteropathy (n = 3), and aseptic arthritis (n = 1). At last follow-up, 30 patients were alive at a median age of 144 months (range, 58 to 253 months). Among 23 patients who were evaluated by respiratory function tests and computed tomography, 3 had an obstructive syndrome, 6 had bronchiectasis, and 20 had chronic sinusitis. CONCLUSION: Early IVIg replacement therapy achieving residual IgG levels >500 mg/dL is effective in preventing severe acute bacterial infections and pulmonary insufficiency. More intensive therapy may be required to fully prevent the onset of bronchiectasis, chronic sinusitis, and nonbacterial infections, particularly enteroviral infections, in all cases.

Agammaglobulinemia↗
PubMed1999

[Asthma in children below 5 years of age: diagnosis and treatment].

Asthma is the most frequent chronic disease in pediatrics and the increase in its prevalence is a major public health problem. Diagnosis may be difficult in the young child, symptomatology most often occurring following a viral infection. It is important not to ignore a foreign body in the airways or fibrocystic disease and asthma remains, particularly in infants, a diagnosis of elimination. Misdiagnosis or insufficient treatment of asthma may risk the development of irreversible histological lesions and also could compromise pulmonary growth and the child's lung reserve. Spacer devices and nebulisers enable inhaled therapy to be administered to very young children. The value of early diagnosis is to institute appropriate treatment notably in severe asthma with inhaled corticosteroid therapy, the aim being to reduce remodelling lesions of the airways. The minimal effective dose should be defined to minimalize side-effects. The treatment of asthma is not restricted to pharmacotherapy: attempts should be made to reduce intercurrent viral infections, domestic pollution (including smoking) and allergenic concentrations. However, as for all chronic diseases, the clinician will encounter poor compliance. The work of education and support of health professionals is fundamental to the management of asthma.

Asthma↗
PubMed1997

Successful bronchotracheal reconstruction in esophageal bronchus: two case reports.

Esophageal bronchus is the most common congenital bronchopulmonary foregut malformation. Current surgical treatment is resection of anomalous pulmonary tissue, which is often hypoplastic and destroyed by infection. The authors report two cases of bronchotracheal reconstruction. The diagnosis was early, before 15 days of age. The anomalous pulmonary tissue had a pulmonary arterial supply and venous drainage as assessed by angiography and a good functional capacity on selective ventilation. Bronchotracheal anastomosis was successful in both cases: a right main bronchus at 25 days of age and a left main bronchus at 13 days of age. One child underwent reoperation 1 year later for bronchomalacia of the reimplanted bronchus. Both children are well with normal growth 3 and 7 years after surgery. Chest roentgenograms showed normal and symmetrical lung aeration. Tracheal reimplantation may be preferred to pulmonary resection when the anomalous pulmonary tissue is not destroyed. The pulmonary functional capacity is increased and the complications of pneumonectomy avoided.

Anastomosis, Surgical↗
PubMed1994

Insulin responses to intravenous glucose and the hyperglycemic clamp in cystic fibrosis patients with different degrees of glucose tolerance.

The relationship between altered insulin secretion and impaired glucose tolerance was studied in 32 cystic fibrosis patients, 16 men and 16 women, aged 8-26 y, using oral and i.v. glucose tolerance tests and a hyperglycemic glucose clamp (10 mmol/L). Seven of these subjects were already being treated with insulin; seven had fasting blood glucose levels below 7.2 mmol/L but satisfied diabetic criteria at the oral glucose tolerance test; glucose tolerance was impaired in 13 subjects and normal in five. The insulin responses to the two i.v. glucose stimuli were inversely correlated with the plasma glucose levels (60 and 120 min) and the area under the curve of the oral glucose tolerance test. However, the acute insulin response to i.v. glucose was severely altered in patients with impaired glucose tolerance, whereas plasma insulin levels during the hyperglycemic clamp did not differ from those of healthy subjects. The responses to the two stimuli were dramatically low in the diabetic patients. These results suggest that cystic fibrosis patients with normal or impaired glucose tolerance retain their capacity to secrete insulin. Alterations in the acute phase of glucose-stimulated insulin secretion seem to be principally responsible for the early impairment in glucose tolerance.

Administration, Oral↗
PubMed1993

[Efficacy of a powder inhaler (Turbuhaler) in moderate asthmatic crises in children].

The use of a metered dose inhaler can be difficult in children, and requires the juxtaposition of an inhalation chamber. Powder inhalers offer different means of administration and are simpler to use and can be easily carried in the pocket or in a school satchel. The aim of this study is to assess the use of a beta-2-mimetic powder inhaler (Bricanyl Turbuhaler) in children presenting with an exacerbation of asthma. Thirty children aged from 4 to 14 years were included in an open random study in parallel groups. After a baseline measure of pulmonary function, the children inhaled 500 microgram of powdered terbutaline, or with metered dose aerosol attached to a spacer device (Nebuhaler). A significant bronchodilatation was apparent in the two groups of children which was measured at 15 and 30 minutes after the inhalation of the drug (measures of peak flow and forced expiratory volume in one second and of specific airways resistance were made). The bronchodilator response was similar whatever the method of inhalation. Although the inspiratory flow is decreased in an exacerbation this work shows that it is possible to advise the use of a powder inhaler in children presenting with a moderate exacerbation of asthma.

Acute Disease↗
PubMed1990

[Practical diagnostic approach to anomalies of the aortic arch. Apropos of 31 pediatric cases].

This analysis of 31 cases of anomalies of the aortic arch has confirmed the predominance of subclavian retro-oesophageal arteries and double aortic arches (74% of cases). The age at which the first clinical signs presented was less than one year in 75% of cases. Respiratory signs predominated with recurrent bronchitis (16 cases) and bronchial congestion (9 cases). These signs were not specific for the type of aetiology and were characterised by their chronicity and recurrent nature. Inspiratory and expiratory stridor was found on 8 occasions and was very suggestive of the diagnosis. There was a frequent association of asthma and anomalies of the aortic arch in this series (13 cases) with 6 cases of atopic asthma in early childhood and 7 cases of asthma in infancy. The oesophago-gastroduodenal transit was the key diagnosis which enabled the vascular anomaly to be confirmed. There was, however, a failure on 5 occasions with 2 false negatives and 3 cases where the wrong type was suspected. Fibreoptic bronchoscopy enabled the topography to be established more precisely including the degree of compression (in 14 cases) and showed evidence of associated tracheomalacia in 7 cases. Nuclear magnetic resonance was very helpful, giving a better definition of the anatomical type and of the relationship of the vascular arch with the oesophago-tracheal axis. Broncho-fibroscopy and nuclear magnetic resonance make strong contributions towards the indications for operation.

Adolescent↗
PubMed1989

[Anaphylaxis syndrome induced by exercise].

The authors report the case of a 12 year-old boy with exercise-induced anaphylaxis. Angioedema was the main symptom and was accentuated by ingestion of an orange prior to exercise. Exercise-induced anaphylaxis is due to mast cell degranulation that is triggered by exercise alone or, less commonly, by the combination of a sensitizing food and exercise. The symptoms of exercise-induced anaphylaxis may be moderate or severe, with laryngeal dyspnea and shock. Prevention is based on avoidance of the offending food before exercise and a reduction of the intensity (and even the suppression) of exertion.

Anaphylaxis↗