Biomedical subjects
Publications and source records attributed to C Amiel-Tison.
The main lines in prevention are analyzed in two fields. Prevention of brain damage during birth process is studied according to the data of a prospective study, showing the obstetrical situations where the fetus is at risk. Prevention of fetal infection is shown on a few clinical examples.
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Abnormal hypertonia of the neck extensor muscles in newborns may be appreciated by observing spontaneous posture, evaluating resistance to repeated passive flexion of the head, and eliciting the head straightening reflexes. This sign correlates well with other signs of insult to the central nervous system. Of 1743 newborns over 37 wk gestational age, 17 appeared to have signs of cerebral insult according to the classical criteria of abnormal state of consciousness, tone, and reflexes. Of these 17, 12 (70%) had neck extensor hypertonia. 38 newborns appeared to have mild signs of cerebral insult with abnormalities of tone and excitability. Of these 38, 14 (37%) had neck extensor hypertonia. Only 12 (0.7%) of 1655 newborns with otherwise normal neurological examination had neck extensor hypertonia. Further studies are necessary to evaluate the prognostic value of neck extensor hypertonia in the neonatal period.
Immunoassayable plasma renin activity (PRA) was measured in 16 healthy preterm infants of different gestational ages. A total of 52 measurements have been performed between the second week of life and the date of the expected term. PRA was found markedly increased in preterm infants and a highly significative negative correlation with postmenstrual age was proved.
Two cases of cerebral gigantism occurring in related boys (cousins of 3rd degree) are discussed. It is difficult to argue from these cases in favour of a precise type of hereditary transmission. The hypothesis of a dominant trait with weak penetrance cannot be excluded. A genetic heterogeneity of the Sotos syndrome is very likely.
Thirty-nine cases of salt and water retention have been observed in low birth weight infants, preterm or small for dates, during the past five years. The onset on the oedema was delayed and appeared on average at the age considered to be term, that is at about 40 weeks corrected gestational age. The iatrogenic nature of the oedema is shown by the evidence in the population at risk of preterm and small for dates infants: It is frequent when the milk is non-modified cow's milk which contains 21-25 mEq/1 of sodium, less common with modified cow's milk containing 11 mEq/sodium1 and and never with breast milk (7 mEq/1). In the absence of treatment neurological signs appeared, as showed by intracranial hypertension. One injection only of frusemide was often sufficient to interrupt the vicious circle.
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A partial 10q trisomy due to a de novo t(10;22) translocation is reported. The comparison with other published cases of partial 10q trisomy leads to the description of a particular syndrome essentially due to a trisomy for bands 10q24 to 10q26.
The first results of long-term evolution of 99 children, most of which were premature, and who survived after intermittent positive pressure respiration for hyaline membrane disease, have been analyzed. No matched series could be set up. With a follow-up ranging from 3 1/2 years to 6 years, 51% of the children were normal, 12.5% presented with severe neurological and psychological sequellae, 31% had acceptable, sometimes slight handicap. As compared with series of the literature, prognosis was improved with intensive care and artificial respiration. This is far more obvious for the children at stage III, of which none survived without artificial respiration.
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Modern obstetrics can no longer be content to statistically analyse deaths only but must also concern itself with injuries. Cerebral damage in the new-born at term may result in grave sequelae. A study was made in the maternity department of the Port-Royal hospital of 65 infants, born at 38 weeks or later, with neurological signs, over the period 1968/69. The study was aimed at clarifying neonatal neurological signs, analysing the obstetric circumstances in which the complications occurred and observing future development of the children involved. In most instances a combination of neurological signs was present, falling into three broad clinical categories on the basis of severity. These neurological signs may appear during the hours after birth even when the Apgar score was satisfactory. Analysis of the cases showed that obstetric factors were responsible for the neurological disorder in 45 cases. The obstetric circumstances were almost always identical: abnormalities of presentation, dynamic problems during labour, and trials of labour. Modern methods for in-utero foetal surveillance are excellent but can nerve replace a perfect and hard won understanding of basic obstetric principles. Follow-up showed that sequelae remained in 7 children. It would appear, then, that in addition to "high risk" pregnancies, there exist "high risk" deliveries in part unpredictable before the onset of labour. There exists also a group of children in whom risk is increased and in whom surveillance over many years is indicated.
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