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Biomedical subjects

J M Vignaud

Publications and source records attributed to J M Vignaud.

60 records · Page 4Linked to original sources

[Amicrobic pustulosis hypoparathyroidism epilepsy: treatment by 25-hydroxycholecalciferol].

We report one case of amicrobic pustulosis cured with Dedrogyl (25-hydroxycholecalciferol), active metabolite of the vitamin D. The importance of our case stems from the physiopathologic study of this cure. The patient is a 16-year-old boy suffering from a hypoparathyroidism since his younger days. Consequently, he shows a hypocalcemia and convulsive seizures due to the hypoparathyroidism. Those seizures had been wrongly attributed to a primary epilepsy of a neurologic origin and a treatment with Phenobarbital had been instituted. In fact, they were side-effects of the hypoparathyroidism and the Phenobarbital had only aggravated the hypoparathyroidal hypocalcemia as a result of its effect on the metabolism of the vitamin D (deviation of this metabolism by enzyme induction at the level of the liver). The Dedrogyl (25-hydroxycholecalciferol) has restored a normal phospho-calcium balance and the hypocalcemia has disappeared as well as the convulsive seizures which, one year later, had not reappeared while they had previously been continual. And above all, standing back one year, we have recorded a complete cure of the amicrobic pustulosis on account of the Dedrogyl.

Adolescent↗

Low-grade osteosarcoma of metacarpal bone.

A radiograph of the second left metacarpal bone in a 34-year-old man showed irregular lytic areas in the diaphysis that was expanded with perpendicular periosteal reactions. Examination of the first biopsy specimen indicated a fibrous dysplasia. Examination of the second biopsy specimen revealed an unequivocal grade I intramedullary osteosarcoma, desmoid to fibrous dysplasia-like in histological pattern. Three years after an adequate en bloc resection of the metacarpal bone, the man was healthy. The long-term course is usually favorable.

Adult↗

Melanotic neuroectodermal tumor of infancy.

A 6-month-old female presented with a swelling of the left superior maxillary bone. A radiograph revealed a radiolucent lesion in the left anterior maxilla, and CT, showed a solid mass. At surgery, the lesion was a cystic dental tumor of ectopic location which was treated by excision. The pathological diagnosis was melanotic neurorectodermal tumor. This is an uncommon benign tumor of the neural crest origin that occurs mainly in the maxilla (70% of cases) but can occur in other areas such as the skull and the mandible. CT reveals a hyperdense mass and MR shows a hypointense mass on T1-weighted images and an iso-intense mass on T2-weighted images. The evolution is usually benign after surgical removal.

Diagnosis, Differential↗

Experimental emphysema following one intravenous infusion of trypsin.

This study was undertaken to investigate the effects of a deficit in protease inhibitor (AT) induced by intravenously administered trypsin on the development of elastase-induced emphysema. Rats receiving a perfusion of trypsin (4.5 mg/kg body wt) intravenously (TIV rats) or one instillation of elastase (92 IU/subject) into the trachea (ELAS rats) were compared with rats receiving both trypsin and elastase (TIVELAS rats). Compared with 8 sham-injected rats, the serum AT activity of 14 TIV rats decreased slightly (5.5%) 150 min after the beginning of the perfusion. In six other TIV rats sacrificed early after the perfusion, a granulocyte sequestration with edema and vascular thrombi demonstrated early lung injury. Anatomical studies of lung and determination of the mean linear intercept (MLI) were carried out 56 days after the administration of the enzymes. Emphysema was confirmed by a significant (P less than .001) MLI increase (about 150 microns) in 22/24 TIV, 20/21 ELAS, and 21/21 TIVELAS rats in comparison with 40 control rats (78 microns). These similar results of the treated rats show that trypsin did not worsen elastase-induced emphysema and also indicate that trypsin given intravenously alone induces emphysema as does elastase when introduced into the airways. The AT activity decrease consequent to proteolysis by trypsin and pulmonary leucostasis may contribute to this trypsin-triggered emphysema.

Animals↗

[Intestinal ganglioneuromatosis: histochemical, histoenzymological and ultrastructural study of a case].

Report of a child with disseminated ganglioneuromatosis of the gut. The complexity of the intestinal nervous system malformation is proved by histochemical, histoenzymological and ultrastructural studies. The malformation is characterized by: hyperplasia and hypertrophy of enteric plexus and nerves bundles in the meso, high acetylcholinesterase activity, aplasia of the sympathetic innervation with the exception of perivascular plexus, qualitative and likely quantitative integrity of the endocrine digestive system. These data are compared with similar observations in the literature.

Acetylcholinesterase↗