[Current status and perspectives in pediatric pneumology].
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Biomedical subjects
Publications and source records attributed to J Gerbeaux.
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The authors discuss the etiological features of interstitial pneumopathies, with particular emphasis on their characteristics in the newborn and in immunodeficient children, and review the diagnostic findings : clinical and radiological signs, functional disturbances, and results of pulmonary biopsy. The results of 154 needle biopsies in 124 children aged 1 month to 16 years are described. In 103 cases a diffuse pneumopathy was present and 80 % of the biopsies were successful. Precise diagnosis could be made from the histology specimen in 22 cases out of the 82 examined, while in 60 of these cases the specimen could be identified.
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Four children aged between 7 and 19 months with severe bronchopneumonia due to adenovirus type 7, proved by virology and/or serology developed severe hyponatraemia. One of them is reported in detail: it was possible to estimate plasma ADH levels and thereby prove the existence of reversible hypersecretion of the hormone. Whilst the syndrome of hyponatraemia with inappropriate secretion of ADH has not yet been reported in association with severe pneumonia in the child, it is known in adults. The limits of the syndrome and its physiopathology are discussed. It may be due either to vagal stimulation as a result of a fall in left aressure, or to central involvement. Therapeutic implications of the problem are emphasized.
One case of mediastinal tuberculous lymph node with fistulae into the esophagus is reported in a 9 years old girl. The first symptom was the appearance of epigastric abdominal pain, with secondary dysphagia. The diagnosis was confirmed by histology after thoracotomy and surgical excision. Esophageal tuberculosis is rare and seems to be secondary to contamination from mediastinal lymph nodes. Treatment is always medical and often requires surgery.
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During the last six years, the search for a deficiency in alpha-1-antitrypsin (AAT) was carried out in 106 children aged from 5 months to 14 years, having the obstructive pulmonary syndrome. Nine deficiencies were in this manner detected and phenotyped. These are the observations of three of these children which are reported here. Their age ranged between 4 years and a half for two of them and 8 years for the third one, having asthma. The phenotypes are respectively SS, MZ, and ZZ. Following these observations, the problems brought up by the association in AAT deficiency and the obstructive pulmonary syndrome in children are discussed: clinical symptomatology, interpretation of the basic design, pathogenic signification and possible connections with asthma and therapeutic influence. The functional respiratory disorders noted in these children involved a ventilatory asychronism, an overexpansion, and regional abnormalities of ventilation and perfusion. To answer these still outstanding questions, the need for a systematic prospective study carried out on a wide group of children is emphasized.
Circadian rhythms in lung resistance (R1) and dynamic compliance (C1dyn) of 7 healthy children (6 to 10 years) were validated (p less than .05) and then quantified (cosinor method); subjects' synchronization: light on at 0700; light off at 2100. Measurements were performed at fixed per hours (0730, 1130, 1630 and 2230) before and after the inhalation of a beta sympathomimetic bronchodilatator: minus 2mg orciprenaline. The measurements carried out after orciprenaline inhalation show a flattening of the curves, both circadian rhythms in R1 and C1 dyn are not detected (p greater than .05).
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The respiratory function is studied in 3 children, two of which 4 years in age, exhibiting a respiratory obstructive syndrome and an alpha1-antitrypsin deficiency. The study of the respiratory function is global and regional. The disorders noted include a ventilatory asynchronism, a distension, regional anomalies of ventilation and perfusion. The ventilatory asynchronism appears to be an early disorder in the course of alpha1-antitrypsin deficiency. These results have to be related with the development of the elastic fibers during the child's growth.
The energy expenditure (VO2) was measured during the first 36 hours of life in 10 infants of diabetic mothers (IDM) and in 16 normal newborns (NB). The mean VO2 was 5,72 ml/kg/min +/- 0,4 for the NB and 4,94 ml/kg/min. +/- 0,3 for the IDM. The respiratory quotients were similar for both groups. Blood glucose determination from all IDM were greater than 44 mg%. Although the low VO2 for IDM is unexplained, several hypothesis might be anticipated: either lack of utilization of abundant endogenous glycogen stores or increased metabolism of glucose by the cells.
Pulmonary biopsy done with a needle of circular bore, can be performed on very young children. A sample of tissue, big enough to establish a precise diagnosis in 2/3 of cases, can be obtained. The main complication is pneumothorax occuring about once in five. Hemoptysia or hemorrhage has never been observed. A proposed indication of premortem biopsy accelerated the death of a child with congenital pulmonary fibrosis. The search of a diagnosis in diffuse pulmonary diseases is the major indication for pulmonary biopsy in the child.
The authors report a new case of partial immune deficiency of cellular immunity, associated with chronic mucocutaneous candidiasis in a 12 Years-old boy. The disease began very early during the first few weeks of life, with thrush in the mouth. This candidiasis then evolved intermittently and was still present. Numerous cutaneous, pulmonary and ear infections occured throughout this child's life. This morbid association led to a search for an immune deficiency. Humoral immunity was normal. Abnormalities of cellular immunity were as follows: apart from candidine skin anergy, there was a deficiency in the factor which inhibits leukocyte migration, secretion of a factor favouring this migration (MEF). It was also noted the presence of the patient's serum, of a factor inhibiting lymphocyte transformation in the presence of candidine. In spite of treatment with intravenous route, amphotericin B, followed by transfer factor, the oral candidiasis persisted together with the skin anergy to candidine. On the other hand, the serum inhibitory factor disappeared. Pulmonary cryptococcosis probably favoured by corticosteroid treatment, developed on this background of immune deficiency; as usual it spread to the meninges. Treatment associating intraveinous amphotericin B and 5 fluorocytosine oral and later intravenous, total duration 6 months, grave a recovery maintained on a 8 months follow up.