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Biomedical subjects

J Helias

Publications and source records attributed to J Helias.

15 recordsLinked to original sources

Decrease in heart ventricular ejection fraction during multiple sclerosis.

Recent studies have shown that mitoxantrone is effective in patients with active multiple sclerosis (MS) and that cardiac monitoring is usually required. However, right and left ventricular ejection fractions (VEFs) have never been studied in MS patients as compared with control subjects. Radionuclide angiocardiography (RA) was performed to assess right and left VEFs at rest in 40 consecutive patients with active definite MS [15 men and 25 women; mean age 33.9 +/- 10 years; mean disease duration 8 +/- 6.5 years; 18 had relapsing-remitting and 22 had secondary progressive forms of the disease; mean Expanded Disability Status Scale (EDSS) score 4.8 +/- 1.9]. The control group consisted of 40 subjects free of neurological or cardiovascular disease (17 men and 23 women; 44.6 +/- 13.4 years of age). The VEF values obtained in the control group defined the normal limits (right VEF 32-54%; left VEF 50-74%). A statistically significant decrease of right (P=0.02) and left (P < 0.0001) VEFs was found in MS patients as compared with control subjects. RA showed pathological results for right (7.5%), left (10%) and both (7.5%) VEFs in 25% of MS patients. No correlation was found between VEF and sex, age, disease duration, disease course, EDSS score or previous treatment. Autonomic impairment, which frequently occurs in MS patients, may have accounted for the decrease in VEFs. Further physiological studies are required to determine factor responsible for the decrease of VEFs in MS.

Adolescent↗

[Neurodevelopmental outcome of premature infants born at less than 33 weeks of gestational age and not cerebral palsy at the age of 5 years].

AIM: To appreciate the impact of prematurity, fetal hypotrophy and familial environment on the neurodevelopmental performances of very premature infants without cerebral palsy at the age of five years. POPULATION AND METHODS: We followed a regional cohort of 171 very premature infants (< or = 32 weeks of gestation) until they were five years of age. Cognitive functions were tested with the WPPSI test and the development quotient was assessed by the ability to draw a "bonhomme". Twenty-two premature infants suffered from cerebral palsy diagnosed before the age of two years. Another infant had a moderate diplegia at the five-year examination. We had no information for 16 prematures (9.3% of survivors). Twenty-eight premature infants were considered as having no severe disability on phone or mailed contact, and another child had a severe isolated mental retardation. We examined 104/148 infants, and 96/148 survivors without cerebral palsy passed the tests. The cognitive functions of these premature infants are compared to the performances of a control group made up of 108 children born at term > or = 37 weeks, matched for birthplace and single or twin characteristics of the pregnancy. RESULTS: The values of the different quotients are significantly decreased in the preterm group. The global IQ and the performance IQ are 0.8 SD, verbal IQ is 0.5 SD and the development quotient is 0.4 SD below the values observed in the control group. A performance IQ less than -2 SD for the mean of the control group is observed three times more than in the controls (13.5% vs 3.7%, P < 0.01). Multiple linear regression shows that prematurity explains, independent of hypotrophy and socioeconomic environment, 8% of the variation of the performance IQ (P < 0.01), 2% of the variation of the verbal IQ and 2% of the development quotient (P < 0.05). CONCLUSION: The five-year neurologic outcome of the children born prematurely in this regional study is similar to the results observed in regional studies conducted in Europe: 13.4% of the survivors have cerebral palsy, and the cognitive functions of the children with no cerebral palsy are significantly lower than the term control group. Other risk factors such as hypotrophy, which modulates the developmental quotient, and the socioeconomic status, which modulates the verbal IQ, are underlined.

Cerebral Palsy↗

[Prognostic value after 1 year of post-infarction exercise test with measurement of oxygen consumption].

A symptoms-limited exercise stress test with measurement of myocardial oxygen consumption (VO2) was carried out in 56 patients on the 44th +/- 16 days after infarction and in 48 patients on the 119th +/- 31 days. Analysis of the expired gases was performed by mass spectrography, cycle by cycle. The second test was coupled with an exercise gamma-angiography in 40 cases. The parameters on exercise were analysed in two groups at one year: group 1 asymptomatic and group 2 symptomatic (subgroup 2a with angina but no dyspnoea, subgroup 2b with dyspnoea). At the second test, the peak VO2 was lower in group 2 (19.46 +/- 5.78 ml/mn/kg) than in group 1 (24.2 +/- 6.5 ml/mn/kg) (p less than 0.008) irrespective of the symptom (angina and/or dyspnoea). The oxygen pulse was lower in group 2a with angina at one year (42.4% +/- 14.3%) compared with asymptomatic group 1 patient (58.5 +/- 12.4%) (p = 0.03). The 3 parameters: VO2, blood pressure and ejection fraction on exercise were independent. The lack of physical fitness may partially explain the absence of variation of the peak VO2 at the first test. These preliminary results should be confirmed by a multivariate analysis in a larger patient group.

Aged↗

Assessment of coronary artery disease by thallium scan in type-1 diabetic uremic patients awaiting combined pancreas and renal transplantation.

Twenty-five type-1 diabetic uremic patients (14 men, 11 women, mean age forty +/- eleven years, range, nineteen to sixty) were prospectively analyzed for coronary artery disease (CAD) by thallium scan (TLS) before synchronus pancreas and kidney transplantation. Duration of diabetes ranged from ten to thirty-two years (mean twenty-two +/- five). Fifteen patients (60%) were in dialysis from two to sixty months (mean twenty +/- eighteen). Advanced diabetic degenerative complications were present in all patients. Twenty exercise and five pharmacologic thallium scans were performed. Forty-four percent of patients (6 men, 5 women, mean age forty-two +/- eleven years, range, twenty-six to sixty) had a positive (reversible or permanent defects) TLS. In 2/11 cases, severe CAD required further cardiac investigations for therapeutic management decision. A statistical correlation with resting ECG nonspecific ST-segment and T-wave abnormalities was observed (p less than 0.05) despite the absence of angor in 8 of the 11 patients. On the contrary, no statistical correlations were found regarding high blood pressure, smoking, hypercholesterolemia, duration of diabetes, and duration of dialysis. In this particular diabetic population (young age, male/female ratio = 1.2) with high incidence of silent ischemia, resting ECG repolarization abnormalities, though predictive, were not specific or prognostic of CAD severity; in these cases, exercise and pharmacologic TLS, a noninvasive and sensitive cardiovascular test, may be of great interest in diagnosis of CAD, allowing adequate cardiac management before, during, and after pancreas transplantation.

Adult↗

Detection of myocarditis during the first year after discovery of a dilated cardiomyopathy by endomyocardial biopsy and gallium-67 myocardial scintigraphy: prospective multicentre French study of 91 patients.

The purpose of this study was to assess the frequency of inflammatory lesions in the myocardium of subjects with dilated cardiomyopathy and to determine if there was any correlation between the results of two methods of evaluation, one (endomyocardial biopsy) invasive and the other (gallium-67 scintigraphy) noninvasive. Of 115 subjects recruited in seven centres, 91 met the inclusion criteria (left ventricular dilatation greater than or equal to 100 ml m-2 and ejection fraction less than 55% with normal coronary arteriography) and had endomyocardial biopsy (mean five specimens) and Ga-67 myocardial scintigraphy after several days. Scanning was considered doubtful 19 times and positive 13 times. The histologic count of mononuclear cells in the myocardial interstitium in 20 fields was greater than 5 cells field-1 in only four cases. No correlation was found between the two methods. Subjectivity in the choice of the criterion of positivity of Ga-67 scintigraphy and difficulties in identifying lymphocytes upon pathological examination were the major problems encountered. Despite limitations, both techniques suggest that cellular infiltrates are minimal and quite infrequent in dilated cardiomyopathy.

Adolescent↗

[Complications of myocardial infarction between the 2d and 4th week].

The complications of myocardial infarction after transfer from the Coronary Care Unit on the 6th day were analysed bu a retrospective study of 3,460 computerised case reports (1973-1980). The mortality rate was 6% (1/3 of hospital deaths) in the period from the 7th day to discharge from hospital (14th to 30th day). Cardiac arrest as not uncommon (20% of all cardiac arrests) but the prognosis was better thn during the initial phase (p less than 0.05) as the mechanism was more commonly ventricular fibrillation or tachycardia (p less than 0.05). This series was compared with a similar population from 1970-1973; an improvement was observed in global hospital mortality (27% previously compared to 17%, in the study series, p less than 0.001). As the population were comparable, this phenomenon seems to be related to better treatment of shock and cardiac failure and the advances in cardiac surgery during the initial phase of infarction. Thd commonest mechanical complication was ventricular aneurysm; its occurrence does not influence the vital prognosis during this period. The incidence of cardiac arrest and death due to cardiac failure is not negligible after the first week of myocardial infarction. Therefore, we do not believe that the hospital period should be reduced after myocardial infarction. Special training of the nursing staff is essential for the successful treatment of these complications. The global prognosis could be improved by the rehabilitation of digitalis therapy and the introduction of new sympathomimetic amines in the acute phase of myocardial infarction.

Adult↗

[Parental guidance].

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Audiology↗

BOR and BO syndromes are allelic defects of EYA1.

Branchio-oto-renal (BOR) syndrome is an autosomal dominant disease characterized by varying combinations of branchial, otic and renal anomalies. By positional cloning, a candidate gene, EYA1, homologous to the drosophila eyes absent gene, has recently been identified at 8q13.3 and shown to underlie this syndrome. The name branchio-oto (BO) syndrome has been used to describe a similar combination of branchial and otic anomalies, without the association of renal anomalies. Whether BOR and BO syndromes involve the same gene was unknown. To address this question, we analyzed two large independent families for which each of the 8 affected members present exclusively with BO syndrome. In both families, linkage analysis mapped the causative gene to the same chromosomal region as EYA1. A search for mutations in 9 of the EYA1 coding exons identified a 2-bp insertion segregating in one family and an 8-bp deletion segregating in the other. These results demonstrate that EYA1 also underlies BO syndrome, and that BOR and BO syndromes are allelic defects of this gene.

Alleles↗