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Biomedical subjects

J de Blic

Publications and source records attributed to J de Blic.

At least 127 records · Page 7Linked to original sources

[Anatomoclinical approach in idiopathic pulmonary hemosiderosis. Apropos of 12 cases].

Idiopathic pulmonary hemosiderosis (IPH) is an infrequent condition whose severe and unpredictable prognosis justifies extensive etiologic investigations. We have assembled 12 cases of IPH (nine children), three adults). In seven patients, pulmonary bleeding was demonstrated upon bronchoalveolar bleeding was demonstrated upon bronchoalveolar lavage (BAL) that recovered numerous hemosiderin-laden alveolar macrophages (Golde index: 247 +/- 53). All 12 patients underwent a surgical lung biopsy. Light microscopy studies showed hemosiderosis, often with lymphoid hyperplasia (n = 11), and occasionally with large germinal centers (n = 4), interstitial mastocytes (n = 7), ferric tattoo of the elastic network (n = 4), and a variable degree of interstitial necrosis (n = 4). Ultrastructural studies were performed in six cases and showed swelling of capillary endothelial cells (n = 5), interruptions in the endothelium (n = 3), tattoo of basement membranes (BMs) and elastic tissue (n = 3), intracapillary platelet aggregates (n = 2), and focal thickening of capillary BMs (n = 2). Four biopsies were studied using immunofluorescence (IF): no deposits of immune complexes were found. Indirect IF assays for antiglomerular and alveolar BMs was performed in two patients and was negative in both.

Adolescent↗

[The Pierre-Robin syndrome. Classification and new therapeutic approach].

Rhombencephalic failure of the suction-swallowing, excess of central and obstructive ventilatory arrests with hypoxia and hypercapnia, vagal hypertonia and esophagogastric motor abnormalities are the new clinical signs observed in children presenting with the Pierre Robin's syndrome. A therapeutic management adapted to each of the types I, II, III of the syndrome were defined and a good nursery-nursing allowed a reduction in the mortality-rate from 27 to 5%. Still considered by some as a malformative and glossoptosing disorder whose etiology is only bucco-pharyngeal in origin, this syndrome, common to numerous embryopathies, is a precocious embryonal abnormality of the brain stem neurogenesis, expressed by the dramatic failure of the physiological oro-ventilation system. This syndrome appears to be a peculiar form of dysautonomia of the brain stem development with an uncertain future, often transitory, isolated or associated but in the heart of pediatric internal medicine and its multi-disciplinarity.

Asphyxia↗

Value of bronchoalveolar lavage in the management of severe acute pneumonia and interstitial pneumonitis in the immunocompromised child.

The diagnostic value of 73 bronchoalveolar lavages was assessed in 67 immunocompromised children (aged 3 months to 16 years) with pulmonary infiltrates. Thirty one children had primary and 19 secondary immune deficiency, 14 acquired immunodeficiency syndrome (AIDS), and three AIDS related complex. Bronchoalveolar lavage was performed during fibreoptic bronchoscopy, under local anaesthesia in all but two. One or more infective agents was found in eight of 11 patients with severe acute pneumonia and in 26 of 62 patients with interstitial pneumonitis. In interstitial pneumonitis, the most frequently encountered agents were Pneumocystis carinii (12), cytomegalovirus (8), and Aspergillus fumigatus (3). The yield was related to the severity of interstitial pneumonitis. The mean cellular count and cytological profile in lavage returns from patients with varying infective agents or underlying pathological conditions showed no significant difference, except in those children with AIDS and AIDS related complex who had appreciable lymphocytosis (mean percentage of lymphocytes 28 (SD 17]. In children with AIDS and chronic interstitial pneumonitis lymphocytosis without pneumocystis infection was observed in eight of nine bronchoalveolar lavage returns and was suggestive of pulmonary lymphoid hyperplasia. Finally, bronchoalveolar lavage produced a specific diagnosis from the microbiological or cytological findings in 44 instances (60%). Transient exacerbation of tachypnoea was observed in the most severely ill children but there was no case of respiratory decompensation attributable to the bronchoscopy. Bronchoalveolar lavage is a safe and rapid examination for the investigation of pulmonary infiltrates in immunocompromised children. It should be performed as a first line investigation and should reduce the use of open lung biopsy techniques.

Acquired Immunodeficiency Syndrome↗

[Diffuse mediastinal, retroperitoneal and hepatic neurofibromatosis in 4-year-old child. Value of puncture biopsy guided by computerized tomography].

A case of neurofibromatosis in a 4 year-old girl is reported. Extensive localization to mediastinum, retroperitoneal space and liver is particularly infrequent. Histological diagnosis was obtained through a needle biopsy of the retroperitoneal space and the liver under the control of CT scan X-ray. Prognosis seems to be related to the tracheal compression.

Biopsy, Needle↗

[Viruses and asthma].

More than one-third of infants with respiratory syncytial virus (RSV) bronchiolitis subsequently experience recurrent episodes of wheezing. In asthmatic children, viral infections frequently trigger asthmatic attacks. Indeed, viruses induce immunological and respiratory functional disorders. As compared with non-wheezing RSV infected infants, wheezing infants with bronchiolitis or asthma due to RSV have: a) increased T-lymphocyte responsiveness to RSV antigen; b) more persistent IgE bound to nasopharyngeal epithelial cells; c) higher titers of RSV-specific IgE and higher histamine concentrations in nasopharyngeal secretions. In children with an atopic constitution, virus infections may trigger allergic sensitization. Furthermore, some viruses enhance the immunologic and non-immunologic human basophil histamine release. Non-specific bronchial hyperreactivity, which is a fundamental feature of asthma, can also be observed to a variable degree in viral respiratory infections. Pulmonary function testing allows in vivo determination of bronchial sensitivity and bronchial reactivity (respectively threshold dose and dose-response curves). Four factors may be involved in bronchoconstriction: a pre-existing diminution of bronchial diameter (which is lacking in many studies); hyperplasia or hypertrophy of bronchial muscles (which is to be excluded in recent viral infections); a non-specific decrease in the threshold for stimulation of irritant receptors due to airway epithelial injury; a partial beta blockade. Further studies are needed to ascertain whether this bronchial hyperreactivity is congenital or acquired.

Animals↗

[Bronchopulmonary dysplasia].

A retrospective study of 2 125 preterm infants, who were ventilated at the Institut de Puériculture in Paris over 9 years (1974-1982) for respiratory distress at birth, showed that 45 (2%) developed clinical and radiological bronchopulmonary dysplasia (BPD): 8 minimal forms, 23 moderate forms and 14 severe forms, 30 of these patients survived (66%). The pathogenesis of this chronic respiratory disease is multifactorial: oxygen + pressure + duration + endotracheal intubation. Efforts should be made to limit the damaging effect of each of these factors, which should be kept down to the minimum values compatible with adequate oxygenation. The presence of emphysema and of a patent ductus arteriosus has also been incriminated, but they may reflect the severity of the initial lung lesion. Rickets, whose incidence was found to be 27%, majors respiratory distress. In the ensuing months, babies with BPD are susceptible to recurrent viral or bacterial respiratory tract infection, failure to thrive and cor pulmonale. The presence of the mother and the care of a psychomotor development specialist are needed for these infants who will be confined for months in conditions which are unsuited to their sensory, physical, emotional and cognitive development.

Autopsy↗

[Bronchiolitis obliterans: technics of exploration and diagnosis].

Bronchiolitis obliterans is an anatomo-clinical entity which may, in young pediatric patients, follow severe viral infections, especially due to measles virus and adenoviruses. After a transient remission, the disease progresses to chronic respiratory failure with dyspneic paroxysms. Diagnosis is based on different investigations which confirm the absence of proximal bronchiolar obstruction and the presence of bronchiolar obliteration. Deficient pulmonary perfusion due to ventilatory defects are demonstrated by pulmonary scintigraphy and even better by digital subtraction pulmonary angiography. Obliteration of non-cartilaginous bronchioli by granulation tissue produces bronchiolar destruction and explains the permenence of alterations. Dissimilarity of the responses of children to acute respiratory viral infection remains unexplained. The degree of the viral load and of the initial immune deficiency as well as the persistence of the virus within the pulmonary tissue probably account for the chronic pulmonary damage.

Adenovirus Infections, Human↗