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Biomedical subjects

M Vidailhet

Publications and source records attributed to M Vidailhet.

At least 181 records · Page 10Linked to original sources
PubMed1990

Cerebral venous thrombosis in systemic lupus erythematosus.

We describe six cases of cerebral venous thrombosis in patients with systemic lupus erythematosus. In one patient, cerebral venous thrombosis was the initial manifestation of lupus; in the five others, it occurred 1-33 years after the diagnosis of lupus. The main clinical features of cerebral venous thrombosis were persistent headache in all six patients, focal symptoms in four, and seizures in three; papilledema was present in only one patient. Cerebral venous thrombosis was diagnosed based on angiography or magnetic resonance imaging. Both the transverse (in five patients) and the superior sagittal (in three) sinuses were involved. Extracranial arterial and/or venous thrombosis were present in three patients, abortion in two, thrombocytopenia in four, and lupus anticoagulant in three. The neurologic symptoms resolved rapidly in five patients treated with steroids and heparin. Cerebral venous thrombosis should be suspected in patients with lupus who complain of persistent headache, especially in the presence of neurologic symptoms.

Adult↗
PubMed1989

Concentration and physicochemical characterisation of unsaturated cobalamin binding proteins in amniotic fluid.

Indirect evidence for the presence of intrinsic factor in amniotic fluid has been provided recently, using either a radioisotope binding assay or a radioimmunoassay. We have determined the unsaturated cobalamin binding capacity and the physicochemical properties of the 3 cobalamin binding proteins in 59 amniotic fluids using radioisotope binding assay, gel filtration and isoelectrofocussing. A good correlation with gestational age was found for the total unsaturated Cbl binding capacity (r = 0.735) and for the concentration of unsaturated haptocorrin (r = 0.746), but not for the concentration of unsaturated intrinsic factor (r = 0.003). When their binding capacities were expressed as a percentage of the total unsaturated Cbl binding capacity, the percentage of intrinsic factor, transcobalamin II and transcobalamin III (the less acidic fraction of haptocorrin) decreased and the percentage of haptocorrin increased in function of gestational age. The physicochemical properties of intrinsic factor in amniotic fluid were close to those in gastric juice: the molecular mass was estimated to 49,200 +/- 4,900 Da (n = 24) in Sephacryl S 300 gel filtration, the cobalamin-protein complex was resolved in 2-6 isoproteins isoelectric at a pH range of 4.6-5.8 and with a mean isoelectric point of 5.18 +/- 0.16 (n = 5) in isoelectrofocusing and it crossreacted with anti-intrinsic factor autoantibodies (from a Biermer anaemia serum). Amniotic fluid collected at 13 wk of gestational age was found to contain intrinsic factor and haptocorrin with less acidic isoproteins than those usually observed in gastric juice and serum. This could indicate that sialic acid is less involved in the composition of the carbohydrate core of cobalamin binding glycoproteins in this period of the gestational age than later on and that cobalamin binding proteins have mainly a foetal origin.

Amniotic Fluid↗
PubMed1989

A case of Alagille's syndrome with translocation (4;14) (q21;q21).

This paper reports a case of Alagille's syndrome, in association with a translocation 46,XY,t(4;14)(q21;21). The possible relationship between this autosomal dominant syndrome and the apparently balanced chromosomal rearrangement is discussed.

Bile Ducts, Intrahepatic↗
PubMed1989

[Prospective evaluation of admission for acute abdominal pain in children].

Acute abdominal pain (AAP) is one of the most frequent causes of admission to an emergency department of a childrens' hospital. The diagnosis viewed with the most apprehension is acute appendicitis. We present the results of a prospective study on the evaluation of the clinical and paraclinical symptoms generally observed in an AAP, and discuss the benefit of a diagnostic score for acute appendicitis. Twenty-five different diagnoses were observed, the 5 most frequent being: "non specific" (34.2%), constipation (16%), otorhinolaryngological infection (11.6%), gastroenteritis (10.7%) and acute appendicitis (10.5%). The study of 12 symptoms showed an elevated sensitivity for each one (92-50%), but a low positive predictive value (72-12%). Rigid adhesion to a diagnostic score would have led to unnecessary medical examination.

Abdominal Pain↗
PubMed1988

[Allergy to mercurothiolate in an infant during heparinization of an intracaval catheter].

A 4-month old infant developed an immediate and proven systemic allergic reaction to mercurothiolate. The acute accident occurred while an intracaval catheter was being treated with a dry-frozen heparin which excipient contains mercurothiolate. This conservative agent is present in numerous pharmaceutical preparations for topical and systemic use.

Catheters, Indwelling↗
PubMed1988

Unsaturated B12-binding proteins in amniotic fluid of cystic fibrosis-affected foetus.

The authors hypothesized the possibility of quantitative or qualitative abnormalities of amniotic fluid B12-binding proteins in cystic fibrosis. Seven cystic fibrosis and 59 normal amniotic fluids sampled between 17.5 and 18.5 gestational weeks were studied by radioimmunogel filtration, radioisotope technique, and isoelectric focusing chromatography. In normal amniotic fluid there was an increase of unsaturated B12-binding capacity from 16 to 41 weeks of gestation. There was no statistically significant difference in unsaturated B12-binding capacity, molecular weights and isoelectric points of haptocorrin, transcobalamin 2, and intrinsic factor between cystic fibrosis and normal amniotic fluid. Cobalamin-binding proteins study cannot help in the antenatal diagnosis of cystic fibrosis.

Amniotic Fluid↗
PubMed1988

[X-ray computed tomography and MRI in Krabbe's disease].

Krabbe's disease whose CT appearance is well known should benefit of the development of MRI as this method is more accurate. MRI permits early diagnosis of leucodystrophy. Yet it must be emphasized that abnormal white matter patterns are not sufficient to permit the diagnosis of Krabbe disease. In the case reported atrophy of the brain, the cerebellum and the brain-stem, demyelination of the white matter and necrosis of corpus callosum were observed. The abnormalities of the brain on MRI pictures help in the diagnosis as they may alert clinicians to the possibility of Krabbe disease in infants with progressive encephalopathy. A definitive diagnosis can be established thanks to the laboratory tests.

Female↗
PubMed1988

[The current practice of prevention of rachitis in France. A survey of 600 physicians].

French legislation prohibiting vitamin D food supplementation, rachitism prophylaxis is based on specific infantile calciferol administration, according to an old ministerial recommendation published in 1963. Out of 1000 questioned physicians, 600 completed the present inquiry. Ninety-nine per cent of them indicated that they systematically prescribe vitamin D for infants, but 18% do not propose it to breast-fed babies; 36% begin prophylactic administration only after 2 weeks and 5% after one month of age; 16% stop prevention between 12 and 18 months, 72% between 2 and 6 years and 12% after 8 years. Expressed on a daily basis, vitamin D dosage is usually rather high, generally slightly higher than the officially recommended intake (1100 to 1400 versus 1000 IU/24 h) and can reach excessive levels, especially in coloured people for which the already large proposed dose (2500 IU/24 h) is exceeded by 13% of inquired physicians. Daily rhythm of supplementation is the most frequent (94% of physicians) before 2 years, semestral or annual afterwards. The mode of administration, diluted in milk (40%), water (27%) or fruit juice (14%) depends on the physicians. In free commentaries, none of them speaks about maternal supplementation during pregnancy. Very few propose special dosage for premature babies. These defects in rachitism prophylaxis are partly due to the obsolescence of official recommendations which postpone the beginning of vitamin D prevention until the age of 6 weeks, do not mention maternal supplementation during pregnancy and do not specify the necessity for vitamin D intake in breast fed infants.(ABSTRACT TRUNCATED AT 250 WORDS)

France↗
PubMed1987

Excretion of cobalamin and haptocorrin in the meconium of cystic fibrosis, premature, and control neonates.

Excretion of haptocorrin (R binder), cobalamin, and other corrinoids was studied in meconium from cystic fibrosis (n = 4), premature (n = 3), and control neonates (n = 13). Corrinoids content was 1.67 +/- 0.92 pmol/mg protein in meconium of cystic fibrosis (CF) neonates but only 0.33 +/- 0.37 and 0.48 +/- 0.47 pmol/mg protein, respectively, in that of prematures and controls. Considering its molecular mass (110,100 +/- 10,100) and its mean isoelectric point (3.67 +/- 0.20), haptocorrin remained undergraded in the meconium of CF neonates whereas it was partially degraded in the meconium of prematures and in most of the meconium from controls. Sequestration of cobalamin by undergraded haptocorrin can explain its increased excretion in CF meconium. Cobalamin-binding capacity of haptocorrin was 22.13 +/- 15.50 pmol/mg protein in CF meconium and about 400-fold lower in meconium of prematures and controls. This may correspond to a fetal intestinal hypersecretion in cases of CF.

Cystic Fibrosis↗
PubMed1986

[Severe vitamin C deficiency and oral pathology. Apropos a case report of Barlow's disease].

Vitamin C deficiency is an exceptional findings but emphasis is placed on the need for detection of latent minimally symptomatic and intricate cases, as illustrated by a 3 1/2 year old child with a latent form of Barlow's disease. Although results of biologic tests were fairly typical, clinical signs were restricted to hypotrophy, gingivostomatitis and proctorrhagia. A literature review failed to determine whether or not specific oral manifestations could be associated with Barlow's disease.

Acute Disease↗
PubMed1986

[Hyalinosis cutis and mucosae. Ultrastructural histochemical aspects indicating intracellular accumulation of glycosaminoglycans].

The exact nature of the genetic defect of hyalinosis cutis et mucosae or d'Urbach-Whiete syndrome is still matter of controversy. The present article reports on three new cases in which several different ultrastructural and biochemical investigations add more arguments to support an anomaly of the glycosaminoglycans degradation in the dermal fibroblasts. Cationic dyes as polyethyleneimine and alcian blue show an intense ultrastructural staining of the abnormal basal laminae and the intracellular lysosomal bodies in cultured fibroblasts. These are related to the accumulation of anionic charged proteoglycans. The primary defect of hyalinosis cutis et mucosae is likely due to a lysosomal defect so far not biochemically defined.

Adolescent↗