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Biomedical subjects

J Duprey

Publications and source records attributed to J Duprey.

At least 19 recordsLinked to original sources
PubMed1992

[Partial 3-beta-hydroxysteroid dehydrogenase deficiencies].

An overview of the partial 3 beta-hydroxysteroid dehydrogenase deficiency is presented. The classical, congenital or early postnatal form is characterized by a salt-losing syndrome and/or ambiguous genitalia. The late-onset forms, only recognized for the last ten years and diagnosed with an increasing frequency, are to be systematically suspected in the presence of clinical hyperandrogenism with or without oligomenorrhea. This deficit, involved in both adrenal and gonadal tissues, seems to be transmitted by an autosomal recessive gene. An ovarian 3 beta-hydroxysteroid dehydrogenase deficit can be a primary cause of some cases of polycystic ovary syndrome and the relations with this affection are disputed. The increased ratios of delta 5 steroids/delta 4 steroids ensure the diagnostic conviction while the elevated ratio of 17-hydroxy-pregnenolone/17-hydroxyprogesterone and the normal ratio of 11-desoxycortisol/cortisol allow to eliminate the possibility of a 21-hydroxylase or 11 beta-hydroxylase deficiency, respectively. The treatment is based above all on the glucocorticoid utilization, which can lead to the return of menses and the ovulatory function, but the cutaneous symptoms of hyperandrogenism will be better controlled by cyproterone acetate out of situations of stress.

3-Hydroxysteroid Dehydrogenases↗
PubMed1990

[Stress and immunity. The role of stress in auto-immunity of Basedow's disease].

The possible influence of stress on the immune system, long since suspected by the clinicians in their daily practice, was confirmed by human and animal studies, some of which being recent. Stress generally exert an immunosuppressive effect, but some of its characteristics (nature, duration, intensity, controllability of the stressing situation) can modulate this response, amplifying or reversing it. The concerned mechanisms are complex, involving the autonomic nervous system, the hypothalamo-pituitary complex and its target-glands through hormonal receptors born on immunocompetent cells. The immune system, conversely, is able to inform the brain about the interference of non cognitive stimuli (viruses, bacteria, tumors) through immunologic cell-derived immunohormones active in the central nervous system. Thus, close immune-neuroendocrine interactions exist, in order to cope with stress of all kinds. The stress can act in Graves' disease by depressing the T suppressive function via the hypothalamo-pituitary-adrenal axis, by eliciting the secretion of catecholamines capable of initiating an hyperthyroidism, by facilitating through its immunosuppressive action a viral infection that can have a part in initiating the auto-immune process.

Autoimmunity↗
PubMed1989

Linkage analysis of hereditary thyroid carcinoma with and without pheochromocytoma.

The use of polymorphic DNA segments as markers for the gene for the multiple endocrine neoplasia (MEN) syndrome, type 2a, allows the identification of family members at high risk for developing medullary carcinoma of the thyroid and other tumors, especially pheochromocytoma. Several families have also been identified in which medullary thyroid carcinoma is inherited, but pheochromocytoma is not seen. We have analysed 18 families, 9 with MEN 2A and 9 with medullary carcinoma of the thyroid without pheochromocytoma, with probes specific for the pericentromeric region of chromosome 10 and conclude that the mutations for the two presentations are closely situated. Genetic heterogeneity of the susceptibility locus was not seen among this sample of 18 families. The genetic mutation for medullary carcinoma was in disequilibrium with the marker alleles of the two closely linked probes, IRBPH4 and MCK2. These data suggest that different mutant alleles of the same gene or closely linked mutations account for the variation in penetrance of pheochromocytoma in families with hereditary medullary thyroid carcinoma.

Adolescent↗
PubMed1989

[Thorburn's posture. A sign of lesion of the cervical cord].

In two infants hypertonia of the scapular muscles (C5-C6 level) associated with flacid palsy in other muscles of the upper limb produced an unusual posture. This one, described in adults by Thorburn, suggests a traumatic or ischaemic lesion of the cervical cord.

Arm↗
PubMed1988

[Improvement of the prognosis of Basedow's disease by using high doses of carbimazole].

The influence of synthetic antithyroid drug dosage on the course of Graves' disease was evaluated by comparing two groups of matched patients treated with carbimazole. Thirty-seven patients received a rapidly degressive treatment (60 mg/day initially rapidly reduced without replacement thyroid hormone therapy), and 36 patients received a prolonged treatment in high doses (60 mg daily for 6 months with progressive reduction of dosage over 5 months and replacement therapy). There was a highly significant difference in the actuarial curves of patients without relapse during 36 months: 82 per cent in patients with high doses, as against 38 per cent in patients with rapidly degressive treatment (log rank: chi 2 = 7.67, P less than 0.01). The anti-TSH receptor antibody titers decreased more rapidly in patients under prolonged treatment with high doses than in those with the rapidly degressive treatment. It is concluded that carbimazole in high doses is more immunosuppressive than in rapidly degressive doses and that it improves the prognosis of Graves' disease.

Adult↗
PubMed1986

[Does immediate-type respiratory allergy occur regarding Stemphylium? Evaluation of 39 challenge tests].

Does immediate respiratory allergy with stemphylium exist? About 39 provocation tests. We present 39 children with positive cutaneous and/or RAST tests for stemphylium mold. Provocation tests individualize 19 patients (14 boys and 4 girls, 10 to 16 years old in 17/19) with a respiratory sensitivity: 6/10 by passive anterior rhinomanometry and 13/15 by bronchial tests. Clinical symptoms are asthma and spasmodic rhinitis (14/19), predominantly from june to august. We noted a mite allergy in 11 cases and a grass pollenosis in 13 children. Positive cutaneous and/or RAST tests are found for alternaria mold in 13/19 cases. We undertook a specific hyposensitization in 14 children with a three years experience in 10 cases. Tolerance is excellent. This provocation respiratory exploration shows the important role played by stemphylium mold in the etiology of recurring respiratory incidents in children.

Adolescent↗
PubMed1986

[Does mugwort pollenosis occur in children in the northern region?].

Is Mugwort pollenosis frequent among children in North of France? In North of France where no ragweed grows, mugwort (artemisia) is after grasses and plantago, the third pollenosis in children. Among 184 children with summer clinical signs, positive cutaneous tests with a mugwort extract are noted in 40 patients (21%). RAST are positive only in 23 on 34 children with positive cutaneous test. Among the 40 children only two are less 5 years old and 26 are more 10 years old. Respiratory provocation tests confirm in 13/19 cases a sensitization of upper or lower respiratory tract. No food allergy exists among those children.

Allergens↗
PubMed1986

[Role of rhinomanometry in childhood mite allergy apropos of 76 cases].

Place for Passive Anterior Rhinomanometry (PAR) in mite allergic children. PAR is a simple method to investigate chronic or recurrent rhinopathies in children. Among 59 investigations with a Dermatophagoides pteronyssinus extract, we found 27 positive responses, 3 doubtful results and 32 negative explorations. Nasal sensitization is usually important: the liminal dose is less than 250 mcg in 17/28 cases. Most children have no bronchial sensitivity. None of 14 children with negative cutaneous and RAST tests have nasal sensitivity. When cutaneous and RAST tests are both positive, 12/16 positive PAR are found. We note a nasal sensitivity in 3/5 cases with only cutaneous test positive and 12/26 cases with only RAST tests positive. For other mites a nasal sensitivity is found in 8 out of 14 explorations (Tyrophagus and Glyciphagus especially). Among all patients when cutaneous tests, RAST tests and history are doubtful for the real part played by mites in the child rhinopathy, nasal exploration allows more explicit diagnostic and therapy.

Adolescent↗
PubMed1986

[Can Acarus siro be considered an allergenic mite in children? Evaluation of 248 cases].

Among 248 allergic children we noted 44 children (21%) with strongly or moderately positive cutaneous tests (In seven out of these tested children respiratory provocation tests are very positive). They are urban children and their houses are not different from other allergic children. They are mostly boys (72%), more than 5 years old (91%), with an asthma (84%), a radiological sinusitis (45%), an hypereosinophilia (88%) and elevated total IgE (90%). 43/44 children also have positive cutaneous tests to Dermatophagoides pteronyssinus mite. Since respiratory sensitivity is exquisite, we undertook a specific hyposensitization for some patients with encouraging results.

Allergens↗
PubMed1986

[Prognostic value of anti-TSH receptor antibodies in Basedow's disease treated with carbimazole].

In order to precise their prognostic value, the anti-TSH receptor antibodies (TRAK) have been measured in 70 cases of Graves' disease treated with carbimazole. Results (expressed in percentage of inhibition of labeled TSH binding) were grouped into 7 mean values: before treatment, from 15 days to 3 months, from 3 to 12 months, 2nd, 3rd, 4th and 5th years. For all cases together, a progressive decrease was observed: 40, 37, 29, 19, 14, 10, 7 p. 100. In 16 cases with good outcome, the mean values are significantly lower than those of 29 cases with bad outcome, respectively: 33 vs 46, 29 vs 43, 17 vs 30, 12 vs 27, 11 vs 22, 3 vs 15, 1 vs 11 p. 100. The TRAK titers at whatever t time are correlated to the clinical state at t + 2 years: about 30 p. 100 relapses if the TRAK titer is less than 10 p. 100; about 80 p. 100 relapses if it is 80 p. 100. So, the anti-TSH receptor antibodies have their place among the prognostic parameters of Graves' disease treated with carbimazole.

Antibodies↗
PubMed1985

[Malignant pheochromocytoma. A case].

A case of malignant phaeochromocytoma is reported. The primary tumour was removed in 1970. Metastases were diagnosed in 1979 and management of the condition included antihypertensive treatment with alphamethylparatyrosine, antitumoral chemotherapy and surgical reduction of secreting tissue. In these rare tumours malignancy can only be confirmed by metastases, i.e. presence of chromaffin cells in loci where they are not usually found. Histology is of little value. High levels of catecholamine precursors or their metabolites in the urine may be a sign of malignancy, but some asymptomatic tumours are only revealed by metastases. Computerized tomography and radioisotope scanning with I131 metaiodobenzylguanidine are the best available methods to locate the lesions. In view of the small number of cases and of the unpredictable course (sometimes spread over many years) of malignant phaeochromocytomas, the effectiveness of treatments with alphamethylparatyrosine, chemotherapy and radiotherapy is difficult to evaluate.

Adrenal Gland Neoplasms↗
PubMed1985

[Glucose intolerance and post-stimulatory hypoglycemia secondary to a probably congenital intrahepatic portacaval anastomosis].

Recurrent malaise in a 63 year old woman were found to be due to hypoglycaemic episodes. During a 5 hour oral glucose tolerance test, the "impaired glucose tolerance" type initial hyperglycaemic wave was followed by a post-stimulative hypoglycaemia. Serum C-peptide levels were normal during the test, but the insulin response which was initially normal became excessive, with a consequent decrease of the C-peptide/insulin ratio, similar to that usually observed in hepatic malfunction. An hepatic ultrasonography, a cavography and a selective superior mesenteric arteriography showed an intra-hepatic porto-caval anastomosis, probably congenital in origin. This vascular abnormality accounts for the blood glucose problems: the porto-caval shunt explains the early hyperglycaemia by defective liver uptake of glucose and secondary hyperinsulinism occurs because of the reduced hepatic degradation of the insulin secreted in normal quantity. The late hyperinsulinism then leads to secondary hypoglycaemia.

Blood Glucose↗
PubMed1984

[Congenital intrahepatic portacaval anastomosis: analysis of manifested glucose abnormalities].

The authors report a case of a high-flow intrahepatic portacaval shunt due to a congenital venous abnormality. Suspected by digestive echography, the abnormality was confirmed by angiography. A precise analysis of glycoregulation showed an "impaired glucose tolerance", followed by post-stimulative hypoglycemia with persistant hyperinsulinemia and lowering of the C peptide/insulin ratio. These abnormalities seem to be related to the shunt only. This observation appears as an experimental model permitting the appreciation of the disturbances in glucose metabolism related to an intrahepatic shunt without any hepatic cell disorder.

Blood Glucose↗
PubMed1981

[Location of an insulinoma by transhepatic phlebography with staged insulin assays (author's transl)].

Insulinoma was easily diagnosed in a 73-year-old woman who had organic hypoglycaemia associated with hyperinsulinism, but the tumour could not be located by echotomography and computerized tomography. The state of the patient's arteries precluded arteriography. Pancreatic phlebography was carried out by the portal transhepatic route and blood was collected at different levels for plasma insulin assays. A very high gradient at the pancreatic isthmus indicated the site of the tumour, which was found on surgery to be precisely there and could be enucleated. This technique cannot be used systematically to locate insulinomas, but it is unquestionably helpful when the tumour cannot be located by other methods or when these are contra-indicated.

Adenoma, Islet Cell↗