Search PubMed⌕ Search

Biomedical subjects

M Voisin

Publications and source records attributed to M Voisin.

At least 37 records · Page 2Linked to original sources
PubMed1989

[Extracardiac malformations in tetralogy of Fallot].

The purpose of our study was to determine the incidence and type of malformations associated with tetralogy of Fallot (TF). Among 133 patients followed up for 12 years, 30 who had either complete TF (n = 26) or pulmonary atresia and interventricular septal defect (n = 4) presented with another, extracardiac malformation. These malformations were part of a malformative syndrome in 21/30 patients, including 4 trisomies 21, 6 embryofoetopathies unquestionably (antiepileptics n = 2, alcohol n = 1) or possibly (hormonal treatment n = 1) of environmental origin, 6 branchial arch pathologies and 5 miscellaneous syndromes. The extracardiac malformation was isolated in the remaining 9 patients, including 5 cases of skeletal anomaly and one case each of omphalocele and microcephaly. Altogether, in these 30 patients skeletal and neurosensory anomalies were largely predominant. Skeletal anomalies involved the vertebral column in 8 cases (cervical 3, thoracic 4, lumbar 1) and the limbs in 5 cases (2 of which were phocomelias). The 4 patients with pulmonary atresia and interventricular septal defect also presented with an early embryopathy: Shprinzen's velocardiofacial syndrome (n = 2), DiGeorge syndrome (n = 1) and situs inversus (n = 1). We compared TF with other congenital heart diseases in our population and found that the incidence of associated malformations was about average. The various associations are discussed. In genetic syndromes, trisomy 21 predominates and TF is less frequent than atrioventricular canal. In syndromes of environmental origin, the role of antiepileptic drugs (chiefly phenytoin and trimethadione) is well-known, alcohol is less often responsible and the TF-phocomelia association is suggestive of progesterone. In branchial arch syndromes, TF is the usual cardiopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Drug-Induced↗
PubMed1989

[Evaluation of the left-to-right shunt in interatrial septal defects (ostium secundum) using Doppler echocardiography. Apropos of 12 cases].

In 12 patients with inter-atrial communication (ostium secundum) (IAC-OS), and ages ranging between 8 and 63 years (mean = 21 years), the ratio between pulmonary and systemic flow (QP/QS) was evaluated with the use of Doppler ultrasonography and compared with the QP/QS obtained by oxymetric measurement during catheterization. The pulmonary or systemic flow is evaluated from the diameter of the opening (d) and the velocity curve (ITV) recorded by pulsated Doppler in the aorta and the pulmonary artery; Q = d2/4 x ITV x heart rate both examinations (sonogram and catheterization) are performed in less than 24 hours. The results show a good correlation between both methods (R = 0.948) (Y = 0.756 X + 0.692). There is no significant variation between intra- or inter-observer. The findings of this study are comparable to those already published; the main difficulty in evaluating of the QP/QS by Doppler sonography are related to the measurement of the pulmonary diameter and there recording of good velocity curves. The QP/QS evaluated by Doppler sonography from a simplified calculation method advocated by Oloez et al. (QP/QS = d2 Ap x V max Ap/d2 Ao x V max Ao were compared, in retrospect, to the data provided by catheterization. The correlation is also satisfactory (R = 0.893). The Doppler ultrasonography is therefore a reliable and reproducible method in as far as the measurement of QP/QS in young or adults subjects affected with IAC OS.

Adolescent↗
PubMed1988

[Congenital heart diseases and urinary malformations].

The purpose of this study was to evaluate the advisability of a systematic search for uropathy in patients with malformative heart disease. Thirty-three cases of urinary tract malformation associated with congenital cardiopathy are reported. These cases represent 2.8 p. 100 of all cardiac patients seen during the same period. The congenital heart diseases were varied, with a predominance of ventricular septal defect (48 p. 100) followed by dextrocardia, single ventricle and coarctation of the aorta. UT abnormalities included vesico-ureteral reflux (36 p. 100), renal agenesis (5 cases), renal dysplasia or hypoplasia (4 cases), upper UT obstruction (4 cases), hypospadias (3 cases), renal ectopia (3 cases), lower UT obstruction (2 cases), polycystic kidney, megaloureter, horseshoe kidney and supernumerary kidney (1 case each). A study of the literature showed that the two uropathies with a risk of associated cardiopathy are renal agenesis and horseshoe kidney. The cardiopathy-uropathy association was found in isolation (11 cases) or combined with cryptorchidism (2 cases) or with multiple malformations (20 cases). The malformations were diverse, the most frequent being neurosensorial malformations (70 p. 100), followed by skeletal malformations (55 p. 100) equally divided between apine and limbs, genital malformations (35 p. 100) and digestive tract malformations (25 p. 100). A study of the cardiopathy-uropathy concordances failed to elicit any predominant association; in the literature, ventricular septal defect is usually associated with horseshoe kidney.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed1988

[Bronchiolitis in infants. Respiratory functional exploration in the acute phase].

Respiratory function tests were performed in infants hospitalized for bronchiolitis immediately after the acute stage, in order to appreciate the function of the respiratory muscles. A study of the ventilatory mechanics, of the transdiaphragmatic pressure, of the ventilatory rate and of the blood gases was performed. Results were compared with those obtained in 10 "healthy" infants. They showed: a decrease in the dynamic compliance (DYN.C) (p less than 0.001) and an increase in the total pulmonary resistances (TPR) (p less than 0.05) providing evidence for the intensity of bronchial and bronchiolar obstruction; an increase in the abdominal and intrathoracic pressures difference (Pdi) (p less than 0.01) which may reach 3 times the normal value and which is proportional to the decrease in DYN.C. (r = 0.74; p less than 0.001), providing evidence for the intensification of the inspiratory diaphragmatic effort and of its adjustment to the degree of obstruction; a reverse of the abdominal pressure curve with respect to that in normal subjects, with an increased pressure level, providing evidence for abdominal distension and increased pressure during all the expiration, suggesting the active participation of the abdominal muscle to expiration; a change in the ventilatory rate, characterized by a high respiratory frequency, a decreased tidal volume, a shortened inspiratory time and a normal inspiratory time/total time ratio. this rate is opposed to that observed in older children with bronchial obstruction. It might optimize the respiratory muscles output, as at this age, they have not yet reached all their contractile capacity.

Acute Disease↗
PubMed1987

[Intestinal malformations and congenital heart diseases].

A series of 21 patients with both congenital heart disease and intestinal malformation seen over a 12-year period is reported. The intestinal malformations were: anorectal malformations (11 cases), duodenal atresia (5), omphalocele (4) and common mesentery (1). Congenital heart diseases consisted of: atrial septal defect (ASD) (10 cases), ventricular septal defect (VSD) (2), tetralogy of Fallot (2) and miscellaneous cardiopathies. In patients with anorectal malformations ASD and VSD predominated (6/11 cases) and multiple malformations syndromes were present in 8 cases, including trisomy 13, Vater syndrome, skeletal (4), neurological (3) and renal abnormalities (3); 3 children died. Duodenal atresia was always associated with left-to-right shunt: VSD (3), ductus arteriosus (2), complete atrioventricular canal (1) and trisomy 21 (2); one child died. Omphalocele coexisted with VSD (2), tetralogy of Fallot (1), dual outlet right ventricle (1), trisomy 21 (1) and multiple malformations syndromes (3); 2 children died. The patient with common mesentery had left-to-right shunt. Comparison of this series with data from the literature showed that children with congenital heart disease have a much higher incidence of intestinal malformations than those with normal heart and that they frequently present with multiple malformations (chromosome aberrations or multiple organ lesions). This multiple malformation complex is particularly common in anorectal malformations where the incidence of congenital heart diseases is 9 to 14%, with predominance of VSD and tetralogy of Fallot. In patients with duodenal atresia trisomy 21 is extremely frequent, and the incidence of cardiopathies is 18%; in the absence of trisomy 21 the cardiopathy is complex.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed1987

[Alveolar microlithiasis in children. Contribution of bronchoalveolar lavage].

This case of pulmonary alveolar microlithiasis emphasizes the rarity of the disease and its exceptional diagnosis in infants which relies on the pulmonary pathologic study. Besides allowing for diagnosis, the study of the alveolar brushing fluid permitted to describe for the first time pictures of post-necrotic cellular calcification which is probably responsible for the disease.

Calcinosis↗
PubMed1987

[Oral occlusion pressure in scoliosis and neuromuscular syndromes].

Oral occlusion pressure, a neuro-muscular index of the activity of the respiratory centers, was measured in 2 groups of children: one of 43 "healthy" children, in order to establish a predictive equation according to age (Y = 1.23 + 8.30 X A-1) and another of children presenting with respiratory failure, either due to an impairment of the thoracic cavity (10 dorsal scoliosis) or to an impairment of the respiratory muscles (29 children with muscular dystrophy or spinal muscular atrophy). The occlusion pressure increases with the degree of the ventilation deficiency in children with scoliosis. It remains paradoxically normal in children with neuro-muscular disease, irrespective of the ventilation deficiency. In the latter, the respiratory centers activity being considered as normal, this situation would indicate their muscular incapacity to correctly express this activity. It would show a severe muscular deficiency with a high risk of occurrence of respiratory distress accidents.

Adolescent↗
PubMed1980

[Kearns' syndrome: a case study (author's transl)].

An anatomoclinical study of a case of Kearns' syndrome is reported. Neuro-ophthalmic symptoms appeared when the child was 13 year-old. Two and a half years later occurred an episode of paroxystic atrioventricular block, after which the triad characterising the syndrome was completed: retinitis pigmentosa, ophthalmoplegia, disorder of heart conduction. The course was unfavorable despite pacemaker insertion. Study of the central nervous system showed spongiosis of the subcortical white substance, of the basal ganglia and of the cranial nerve nuclei. The specialized heart conduction tissue was the site of apparently primary degeneration. The extension of the visceral involvement is discussed in the light of published data.

Adolescent↗
PubMed1979

[Cardiological aspect of Kearns' syndrome. Apropos of 3 cases, with histopathological study of the conduction tissue in one of them].

Three cases of Kearns syndrome are reported. The neuro-ophthalmological signs were comparable with ophthalmoplegia, pigmentary retinal degeneration and polymorphic neuro-muscular and sensory deficits. The electrocardiological signs were observed 2-4 years after the onset of the condition; the cause of death in each case was related to complete heart block. The post-mortem findings in one of the cases were spongial degeneration of the central nervous system and a seemingly primitive degeneration of the Bundle of His and its branches.

Adolescent↗