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.

Adolescent↗

PubMed1975

[Circadian rhythms op ventilatory mechanical factors in healthy children].

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).

Airway Resistance↗

PubMed1975

[Proceedings: Neoplastic complications of ataxia telangiectasia].

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Adolescent↗

PubMed1975

[Early functional disorders in 3 children exhibiting an obstructive syndrome and an alpha 1 antitrypsin deficiency].

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.

Child↗

PubMed1975

[Study of oxygen consumption in infants of diabetic mothers].

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.

Female↗

PubMed1975

[Pulmonary needle biopsy in children].

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.

Age Factors↗

PubMed1974