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Biomedical subjects

M Dumoulin

Publications and source records attributed to M Dumoulin.

At least 55 records · Page 3Linked to original sources
PubMed1988

Cardiorespiratory exercise testing after venous switch operation in children with complete transposition of the great arteries.

In 14 children who underwent a venous switch operation for complete transposition of the great arteries, exercise testing was performed 3 to 15 years (mean 8) after the operation. Exercise performance capacity was assessed by the determination of the ventilatory threshold during exercise and by the oxygen uptake (VO2) reached at a heart rate of 170 beats/min (VO2,170). The ventilatory threshold was defined as the highest oxygen uptake (VO2) at which the pulmonary ventilation (VE) stops to increase linearly when related to VO2. During exercise above this threshold a disproportionate increase in VE relative to VO2 is observed. The mean ventilatory threshold was significantly lower (p less than 0.01) than the normal mean value and averaged 72 +/- 15%, 67 +/- 15% and 70 +/- 13% of the predicted normal value for children of comparable age, weight and height, respectively. The mean value for VO2,170 also was significantly lower (p less than 0.05) than the normal mean value for children of comparable age, weight and height, and averaged 81 +/- 20%, 81 +/- 18% and 80 +/- 18%, respectively. Compared with normal control subjects of the same sex and age, the ventilatory threshold was surpassed sooner (p less than 0.001) and reached after 2 +/- 1 min instead of the 4 +/- 1 min required by the controls. In nearly half of the patients, a lower than normal (i.e. below the 95% confidence limit) heart rate response to exercise was observed. Theoretically, this could be interpreted as indicating a normal or high physical performance capacity.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1988

Cardiac chamber growth pattern determined by two-dimensional echocardiography.

Two-dimensional echocardiographic measurements of the right and left ventricles from the parasternal long-axis and apical four-chamber views and an estimated right ventricular-left ventricular dimensional ratio were obtained in 173 normal subjects, ranging from 1 day to 15 years of age. The end-diastolic dimensions of both the right and left ventricles increases in proportion to the logarithmic function of body surface area. The right ventricular-left ventricular dimensional ratio decreases immediately after birth and continues to decrease during the first months of life. Newborns have altered ventricular shapes as seen in the pathological conditions of right ventricular volume or pressure overload. After the first months of life, the ventricular shapes remain constant throughout childhood. The 95% prediction intervals for the data were computed, and we suggest that the confidence limits of the normal data may be useful for quantitative echocardiographic evaluation in childhood.

Adolescent↗
PubMed1988

Transient neonatal tricuspid regurgitation: a Doppler echocardiographic study of three cases.

Three patients with normal hearts and no pulmonary abnormality had neonatal tricuspid regurgitation causing cardiorespiratory distress and cyanosis. The signs of tricuspid regurgitation resolved over a few weeks. In the acute phase echocardiography showed gross dilatation of the right atrium and ventricle. The interatrial septum bulged into the left atrium during the whole cardiac cycle. Doppler echocardiography showed clinically significant tricuspid regurgitation, a right to left shunt through the foramen ovale, reduced flow through the pulmonary valve, and in two patients ductal flow into the pulmonary artery. In one patient tricuspid regurgitation was so great that it impeded the opening of the pulmonary valve and produced functional "atresia" of the pulmonary valve. The presence of regurgitant blood flow through the pulmonary valve showed that the "atresia" was functional rather than organic. Doppler echocardiographic study is useful in distinguishing functional neonatal tricuspid regurgitation from structural abnormality of the tricuspid valve.

Cardiomegaly↗
PubMed1988

[Myocardial infarct complicating thrombopenia induced by pentosan polysulfate].

The authors report a case of thrombopenia induced by pentosane polysulfate (PSP), complicated by a myocardial infarction. Biological tests showed a platelet pro-aggregating effect of PSP and an IgG immunoglobulin fixed to the platelets. A review of the literature shows that PSP-induced thrombopenias must be known as well as those induced by heparins and that they have similar complications. The thrombopenia may persist with an inappropriate substitution with heparin and the clinical picture may be aggravated. It is desirable to carry out systematic screening for a cross-allergy and prevent it by using anti-vitamin K.

Female↗
PubMed1987

Transformation and rescue of a flightless Drosophila tropomyosin mutant.

In the Drosophila flightless mutant Ifm(3)3, a transposable element inserted into the alternatively spliced fourth exon of the tropomyosin I (TmI) gene prevents proper expression of Ifm-TmI, the tropomyosin isoform found in indirect flight muscle. We have rescued the flightless phenotype of Ifm(3)3 flies using P-element-mediated transformation with a segment of the Drosophila genome containing the wild-type TmI gene plus 2.5 kb of 5' flanking and 2 kb of 3' flanking DNA. The inserted TmI gene is expressed with the proper developmental and tissue specificity, although its level of expression varies among the five transformed lines examined. These conclusions are based on analyses of flight, myofibrillar morphology, and TmI RNA and protein levels. A minimum of two copies of the inserted TmI gene per cell is necessary to restore flight to most of the flies in each line. We also show that the Ifm-TmI isoform is expressed in the leg muscle of wild-type flies and is decreased in Ifm(3)3 leg muscle. Homozygous Ifm(3)3 mutants do not jump. The ability to jump can be restored with a single copy of the wild-type TmI gene per cell.

Animals↗
PubMed1987

Interrupted right aortic arch in DiGeorge syndrome.

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.

Abnormalities, Multiple↗
PubMed1987

Cross sectional echocardiographic assessment of great artery diameters in infants and children.

The pulmonary trunk and aortic root were measured on cross sectional echocardiograms in 173 normal subjects aged from one day to 15 years. Fifteen neonates were reexamined 3-6 days later. The great vessels were visualised in the parasternal long axis and short axis views. All measurements were made in end diastole and end systole by the leading edge method. The internal diameter (inner surface to inner surface) of the pulmonary trunk was also measured. The diameters of the great vessels correlated best with the square root of body surface area. Individual variability in cardiac growth gave a wide scatter of normal values. This was controlled for by calculating the ratio of the pulmonary trunk to aortic root for each subject. This ratio showed little individual variability and, except for the neonatal period, was remarkably constant throughout infancy and childhood (1.06 (0.06)). In the first 24 hours of life the ratio of the pulmonary trunk to the aortic root was significantly larger (1.29 (0.12)) but within one week it decreased to the "normal" ratio found in the older age groups. These normal data should be useful in assessing patients with congenital heart disease, particularly those in whom pulmonary blood flow is abnormal.

Adolescent↗
PubMed1986

[Localization of transcription regulatory sequences. Application to the genes of the prolactin family].

We are studying nucleotide sequences responsible for the regulation of eukaryotic gene expression. Our test system comprises the human genes coding for prolactin (hPRL), growth hormone (hGH-N) and placental lactogen (hCS-B). We have cloned these genes and are searching within their sequences for in vitro binding sites of the human glucocorticoid receptor on the hGH-N and hCS-B genes; the in vivo activity of such DNA sequences by assaying hybrid gene expression in transfected cells; in vivo "enhancer" activity of different hPRL gene fragments linked to a marker gene and transfected in cultured cells.

Base Sequence↗
PubMed1985

Usefulness of echocardiographic assessment of right ventricular and pulmonary trunk size for estimating magnitude of left-to-right shunt in children with atrial septal defect.

M-mode and 2-dimensional echocardiographic studies were performed in 42 patients, aged 1 to 16 years (mean 6), with a secundum or sinus venosus type atrial septal defect (ASD) and normal pulmonary artery pressure. Twenty normal children served as a control group. In patients with ASD the echocardiographic variables were correlated with the magnitude of the left-to-right shunt (Qp/Qs) calculated by the Fick principle. Although M-mode echocardiograms showed increased right ventricular (RV) dimension in 69% of the patients, the correlation between RV dimension index (RV dimension/body surface area) and Qp/Qs was weak (r = 0.49). When RV dimension was related to left ventricular (LV) dimension and expressed by the RV/LV ratio, 90% of the patients were found to have an abnormally large right ventricle. The correlation between the RV/LV ratio and Qp/Qs was fairly good (r = 0.64). In 33 patients (78%), the pulmonary trunk (PT) was adequately visualized and measured on 2-dimensional echocardiograms. The dimension of the PT was related to the aortic root dimension and expressed by the PT dimension/aortic dimension ratio. This ratio was 0.99 +/- 0.06 in normal children and 1.35 +/- 0.23 in patients with ASD (p less than 0.001). The PT/aortic ratio exceeded the upper limit of normal (the normal mean value + 2 standard deviations) in each of the 27 patients with a Qp/Qs of 1.5 or greater. In 5 of the 6 patients with a Qp/Qs of less than 1.5 the PT/aortic ratio was close to 1 and within the normal range. An excellent correlation (r = 0.89) was found between the PT/aortic ratio and Qp/Qs.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1983

Long-term echocardiographic assessment of dilated cardiomyopathy in children.

Left ventricular (LV) dimensions and function were assessed by echocardiography in 22 children with dilated cardiomyopathy. They had survived an initial episode of congestive heart failure in infancy for greater than or equal to 2 years. At the time of echocardiography, when they were 3 to 16 years old, 8 patients (Group 1) still had signs of dilated cardiomyopathy and 14 (Group 2) had lost all roentgenographic and electrocardiographic evidence of heart disease. All 8 patients in Group 1 (average follow-up 4.5 years) had significantly increased LV dimensions. The end-diastolic dimension averaged 144 +/- 18% of the normal value. Fractional LV shortening with systole was significantly reduced and averaged 23 +/- 3%. The E point-septal separation ranged from 7 to 17 mm (mean 12 +/- 4) and was far above the normal limit in all. Of the 14 patients in Group 2, seven (average follow-up 7 years) had normal ventricular dimensions and 7 (average follow-up 10 years) had LV dimensions larger than the upper range of the 95% prediction limit. In 6 of the latter patients the fractional LV shortening with systole was less than or equal to 31% and the E point-septal separation in excess of the upper limit of normal. These findings indicate that about half of the patients who had apparently recovered still had residual lesions as judged from the echocardiogram. In 6 patients in group 1, two-dimensional echo-cardiography allowed the visualization of a thickened endocardium. One of these 6 patients died. The echocardiographic image correlated well with the process of LV endocardial fibroelastosis found at necropsy.

Adolescent↗
PubMed1983

[Rubella deafness. Audiometric, ophthalmologic, epidemiological aspects].

Case-reports of 75 children, with deafness due to an embropathy from maternal rubella during the first months of pregnancy, are reviewed. Emphasis is placed on the frequency of interauricular functional asymmetry, of pigmented retinitis alone or associated with a cataract, and of dysmaturity at birth and persistence of overall delayed development. Also stressed are the large proportion of women born outside the French Metropolitan area who contracted rubella during pregnancy, and the very high frequency of errors in interpreting clinical and biological signs in suspected rubella, leading to an impressive number of wrong suggestions with marked effects on the future. Following a detailed description of 15 of the 75 cases studied, the results obtained are analyzed. Nearly half of the cases presented an interauricular auditory functional asymmetry, which is therefore one of the elements of etiological diagnosis and which enables better adaptation of a hearing aid for rubella-induced deafness. The very high frequency of pigmented retinitis alone also constitutes and element of etiological diagnosis, and enabled at least 7 p. cent of overall cases of perception deafness to be assessed as resulting from a rubella cause. The pigmented retinitis is sometimes central and, contrary to classical notions, provokes visual dysfunction. Difficulty in applying a correct diagnosis during the initial episode, and the usual lack of information given to the family in case of rubella during pregnancy, are common features in the vast majority of cases.

Audiometry↗
PubMed1982

Congenital atresia of left coronary ostium.

A two-year-old girl, who presented with congestive heart failure and an extensive anterolateral infarction, was thought to have anomalous origin of the left coronary artery from the pulmonary artery. She improved rapidly and remained symptom free until 13 years of age, when she died suddenly. At necropsy the right coronary artery was found to be normal but in the left aortic sinus a dimple was the only remnant of the left coronary ostium. The proximal segment of the left long term follow-up of a patient with this rare anomaly.

Child, Preschool↗
PubMed1981

Study of platelet diameter distribution in patients with valvular heart disease and with prosthetic heart valve replacement.

We have studied the platelet count and the megathrombocyte index (MI), or percentage of platelets that have a diameter greater than 2.9 micrometers, in 100 patients with valvular heart disease. The mean platelet count is significantly lower than in controls (p less than 0.0005) and the MI is significantly increased (p less than 0.01). No difference was observed in these two parameters according to the variety of the valvular heart disease. 45 of the same patients were studied after prosthetic heart valve replacement. Our results show a significant elevation of platelet count and a significant decrease of MI compared with the presurgical values in all groups except in the mitral one in which MI remains increased. This work demonstrates the presence of platelet abnormalities which reveal a compensated thrombocytolytic state in patients with valvular heart disease and its partial correction after aortic, but not mitral, valve replacement.

Adolescent↗
PubMed1979

Aneurysm of the left atrial appendage.

A 2-year old asymptomatic girl is presented in whom the chest x-ray film led to the discovery of a grossly distorted cardiac shadow. Angiocardiography showed a giant aneurysm originating from the left atrium. At operation an intrapericardial aneurysm of the left atrial appendage, measuring 12 X 4 X 4 cm, was found. It communicated with the left atrium through an orifice measuring 3 cm. The aneurysm was excised.

Angiography↗
PubMed1978

Cardiovascular malformations in Turner's and Noonan's syndrome.

The cardiovascular findings in 9 patients with Turner's syndrome and 9 patients with Noonan's syndrome are described. Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal defect associated with mild supravalvar pulmonary stenosis and anomalous drainage of the right upper pulmonary veins. In the majority of patients the electrocardiogram was different from the pattern usually seen in pulmonary valve stenosis: the QRS axis in the frontal plane was superiorly oriented in 7 out of 9 cases and in 2 patients evidence of right ventricular hypertrophy was lacking in the right praecordial leads; in 5 patients an rS complex was seen in the left praecordial leads. Gross thickening of pulmonary valve cusps was found at operation in 4 of the 8 patients who were operated on. Although phenotypically related, Turner's and Noonan's syndromes are associated with different and distinct cardiovascular anomalies.

Adolescent↗
PubMed1976

Hypertrophic cardiomyopathy in Friedreich's ataxia.

The cardiac findings in two sibs with Friedreich's ataxia are described. The clinical signs were suggestive of hypertrophic obstructive cardiomyopathy. During left heart catheterization a systolic pressure gradient across the left ventricular outflow tract could be provoked by an infusion of isoprenaline. Left ventricular angiocardiograms and echocardiograms showed gross thickening of the interventricular septum. In one patient a systolic anterior movement of the anterior leaflet of the mitral valve was seen. The importance of serial echocardiographic examination for patients with Friedreich's ataxia is emphasized.

Adolescent↗