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Biomedical subjects

P Pradat

Publications and source records attributed to P Pradat.

At least 19 recordsLinked to original sources

Recurrence risk for major congenital heart defects in Sweden: a registry study.

During the period 1981-1986, 1605 infants presenting a major congenital heart defect (CHD) were identified in Sweden. Using the personal identification numbers of the mothers, 1507 of them could be linked to the Medical Birth Registry and two controls were selected for each infant. For this total of 4521 infants, 2686 postoccurrence sibs born during the period 1981-1989 were identified from the Medical Birth Registry. The tendency to have one or more sibs was higher among cases than among controls and was correlated to the life status of the proband. This tendency did not vary according to the type of CHD when controlling for life status. The prevalence at birth of CHD was almost four times higher among sibs of CHD infants than among sibs of normal infants. The tendency to have a sib with an extracardiac malformation did not differ between CHD probands and normal probands. The importance of only including births occurring after the proband when evaluating recurrence risks is stressed.

Family Health

The epidemiology of three serious cardiac defects. A joint study between five centres.

The paper reports a joint study made by five member programs of the International Clearinghouse for Birth Defects Monitoring Systems. Three specific heart malformations were studied, hypoplastic left heart syndrome (HLHS), transposition of the great vessels (TGV), tetralogy of Fallot, and some epidemiological characteristics were analyzed. The prevalence at birth was estimated to be 2.0, 2.9, and 2.2 per 10,000 births, respectively. No time trend in the prevalence at birth was observed for any one of the three malformations in the total study population. When only isolated defects were considered (infants without major non-cardiac malformations), all three cardiac anomalies showed an increased rate in infants with low birth weight, short gestational duration and probably twinning. A preponderance for males was observed for each defect but was strongest among infants with tetralogy of Fallot (sex ratio 2.5 for Fallot, 1.4 to 1.5 for the other conditions). There were also differences between the three cardiac defects with respect to percentage of low birth weight, preterm births, and rate and type of associated extracardiac malformations. This paper stresses the advantage of pooling data from different registries in studies of uncommon specific malformations and infrequent characteristics.

Adult

Maternal occupation and congenital heart defects: a case-control study.

This paper reports a Swedish analysis of the possible effect of maternal occupation on the occurrence of congenital heart defects. The analysis included 3324 infants (1108 cases and 2216 controls) born during the period 1982-1986. Information on maternal occupation was retrieved using two sources: the 1985 census and the Medical Birth Registry. A comparison between these two sources using the information on mothers of 756 infants born in 1986 showed a good concordance although 11% of the women were reported with a different employment status. No association appeared between the type of maternal occupation and the occurrence of infant congenital heart defects although some odds ratios were slightly above 1.

Case-Control Studies

[Meningeal melanocytoma or multiple pigmented meningioma of the spinal canal. Report of a case. Review of the literature].

In a 64-year-old male with pain and weakness of the right lower limb, investigations disclosed a cervical tumor, several dorsal tumors, and tumoral infiltration of the lumbosacral area. Histological and, above all, immunohistochemical and ultrastructural features were suggestive of meningeal melanocytoma. Before the use of immunohistochemical and electron microscopy techniques, meningeal melanocytoma was designated by the term pigmented meningioma because its histological features bear some resemblance to those of meningioma. However, only a few cases of meningeal melanocytoma have been documented by immunohistochemistry and electron microscopy. Previously reported patients with meningeal melanocytoma had solitary tumors. In contrast, a review of the literature showed that multiple intraspinal or intraspinal and intracranial meningiomas are not exceptional, although meningiomatous infiltration is considerably less frequent. Treatment of such cases rests on surgery, radiotherapy, and chemoimmunotherapy.

Combined Modality Therapy

A case-control study of major congenital heart defects in Sweden--1981-1986.

This paper reports the results of a case-control study of major congenital heart defects (CHD) in Sweden. During the period 1981-1986, 1,324 such cases were identified and 2,648 controls were selected. Some common maternal characteristics and exposures were studied using information from prospectively collected data. Possible associations with CHD were found for previous perinatal death, maternal diabetes, epilepsy, hydramnios and disproportion between fetus and pelvis. More specific associations were observed between previous spontaneous abortion, epilepsy, hydramnios and truncus anomalies and between diabetes and septal anomalies. However, no associations were found with involuntary childlessness, contraceptive use, or smoking.

Abortion, Induced

Epidemiology of major congenital heart defects in Sweden, 1981-1986.

STUDY OBJECTIVE: The aim was to make an epidemiological study of major congenital heart defects and to make comparisons between groups of heart defects regarding different epidemiological variables. DESIGN: The cases with congenital heart defects were identified from two Swedish registries: the Registry of Congenital Malformations and the Child Cardiology Registry. A classification with nine groups of heart defects is proposed. SETTING: This was a national survey in Sweden during the period 1981-1986. The total number of cases reported during the six years was 1605. Cases that presented a patent ductus arteriosus only and a birthweight below 2500 g were excluded. MEASUREMENTS AND MAIN RESULTS: The prevalence at birth was 2.8 per 1000. When cases with a chromosomal anomaly were excluded, no maternal age effect was observed except a possible under-risk for women above 40 years. The risk for a heart defect seems to increase with increasing parity independent of age. A higher risk was also observed among twin infants compared with singletons although no specificity in cardiac malformation type appeared. Sex ratio, the tendency for having an associated extracardiac malformation, and the proportion of isolated v non-isolated cardiac defects differed between the different groups of heart malformations. CONCLUSIONS: The differences observed between groups of defects indicate different aetiologies and show that this classification may be suitable for further analyses of congenital heart defects.

Chromosome Aberrations

Is trisomy 18 increasing in Sweden? An analysis of the syndrome during a ten year period and a comparison with a French registry.

This paper reports an analysis of trisomy 18 in Sweden between 1978 and 1987 and a comparison with data from the Rhône-Alpes/Auvergne registry in France. A strong maternal age dependency is seen, which parallels that observed for Down syndrome. The patterns of registered malformations in the two data sets are quite similar. A progressive increase of the prevalence at birth of trisomy 18 is seen in Sweden during the ten years but not in France. This increase seems to result, at least in part, from an increased identification of the trisomy among malformed infants. A parallel decrease in the reporting of infants with the "cardinal" trisomy 18 malformations is indicated.

Abnormalities, Multiple

[Chondromyxoid fibroma of the cervical spine. Apropos of a case treated by partial vertebrectomy].

A case of chondromyxoid fibroma revelated by cervicalgias and involving the right part of the 5th cervical vertebra is reported. This uncommon cartilaginous tumor is usually described in the metaphysis of long bones and appears very rare in the spine. If radiological aspects have been reported, the majors series do not describe a typical appearance of vertebral lesions; our patient is one of the first to have been evaluated by CT scan. Chondromyxoid fibromas are benign tumors, but recurrence is possible especially when treated by curettage alone. In our case, operated on two stages, the resection seemed sufficiently large and CT control on the 10th month did not show evidence of recurrence. Clinical and radiologic findings, and surgical management of these vertebral tumors are discussed.

Adult

[Ependymoma of the intradural filum terminale in adults. 20 cases].

Twenty cases of ependymomas of the intradural filum terminale in adults have been reviewed. Their pathology was quite uniform, of a myxopapillary type, similar to the low grade ependymoma described by Kernohan, which represent about 23% of the tumours of cauda equina. Mean age of the patients was 35.7 years. Mean time between the first symptom and the diagnosis was 46 months. Clinical symptoms were often non specific, with low back pain and radiculalgias. At the time of operation, clinical signs were essentially motor deficits usually moderate (11 cases), sphincter disturbances (10 cases), and sensory loss (9 cases). In 3 patients with rapid worsening, an intratumoral haemorrhage was found. In 2 other cases, intracranial hypertension was the main symptom: in the first, it was related to hydrocephalus probably caused by spinal subarachnoid haemorrhage; in the second, there was no ventricular dilatation. In this series, neuroradiological examinations had consisted mainly in myelographies. C.T. scan has been performed in 3 patients; in only one case it has allowed to visualize a presacral extension. One patient had preoperative M.R.I.: the association of an expansive lesion with upper cyst in conus medullaris and presence of blood in the sacral area permitted the diagnosis of ependymoma of the filum terminalis. The average size of the tumours was 8 cm. Total removal has been possible in 15 cases (and in 2 of the 5 giant tumours), subtotal removal in 2 cases, and partial removal in 3 cases. In 4 patients where existed an intraspinal cord extension above the conus, it has been resected completely, except for one case with recurrence. Patients with a total removal had a good functional recovery (13/15). No recurrence has been observed in this group. In conclusion, with M.R.I., one may hope an earlier diagnosis, condition of radical surgery. So, radiotherapy which is not without risk, could be avoided.

Adolescent

Experimental autoimmune encephalomyelitis in a low-susceptible rat strain.

The susceptibility to the induction of experimental autoimmune encephalomyelitis has been investigated in the previously little known Wistar/Furth rat stain. These rats have a low sensitivity, similar to that of the Fischer and PVG strains previously studied at our laboratory. However, some differences appear in the histological response of the three strains when a treatment with pertussis or cyclophosphamide is performed prior to the immunization, suggesting that different temporal patterns exist in the development of histological signs, according to the genetic background. The infiltrating cells may have different functions in rats of different genotypes.

Animals

[Medullary compression revealing the presence of an epidural nodular lymphoma (author's transl)].

A case of medullary compression is reported which was found to be due to an epidural lymphoma of the nodular type, which was centrofollicular, with only slight lymphocytic differentiation, and appeared to be isolated. The hour-glass appearance of the tumour suggested, however, that it was not the primary tumour. The short term outcome appeared to be favourable after surgery and radiotherapy but a limited period of chemotherapy is being given. Reports on the frequency of medullary compression during malignant non-Hodgkinson lymphomas vary between 1 and 15% of cases, and many cases have revealed the actual cause, especially in neurosurgical practice. Most authors do not accept the existence of lymphomas primarily located in the epidural space, and feel that they result from metastatic invasion or by contiguity. Prolonged survival can be hoped for after mixed treatment of these tumours, especially in histologically favourable forms.

Aged