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Biomedical subjects

M Hermier

Publications and source records attributed to M Hermier.

At least 127 records · Page 7Linked to original sources
PubMed1985

[Regressive bilocular acute pseudotumoral demyelinization. Second lesion development after 10 years of remission].

The authors report what seems to be the 4th case of acute pseudotumoral demyelinating disease with regressive evolution. Onset was marked by hemiplegia with tumoral pattern on CAT-scan and pneumoencephalography and an important cystic cavity at surgery. Controlateral hemiplegia occurred 2 months later. After a period of partial recovery a long period of remission was observed, followed by a relapse 10 years after the first symptoms. This case suggests that remissions reaching 10 years are possible in such pseudotumoral demyelinating disease, but also shows that there are late secondary evolutions, as in multiple sclerosis.

Acute Disease↗
PubMed1984

[Obstruction of the celiac trunk and the superior mesenteric artery].

Pancreatic, hepatic and intestinal troubles occurred in a 8 year-old acrogeria girl who was treated for Wilm's tumor by cobalt therapy at 2 1/2 years. Complete obstruction of the coeliac trunk and proximal superior mesenteric artery was shown, associated with hypoplasia of the abdominal aorta and its collaterals in the irradiated areas. Acrogeria might enhance the toxic effects of radiotherapy on vessels.

Celiac Artery↗
PubMed1984

[Hypereosinophilic syndrome with endomyocardial involvement in children. Critical analysis of the literature apropos of a case].

A 6 years old girl presented with hypereosinophilia of 7 months duration and cardiac failure from restrictive myocardiopathy, leading to death in 3 years 6 months. Transient blast cells were found. Sixteen previously reported pediatric cases of hypereosinophilia of unknown cause with cardiac involvement are studied. The meaning of eosinophilic leukemia is discussed, the frequency of cardiac damage and the role of hypereosinophilia in the mechanism of cardiac injury are emphasized. Therapy may stabilize or reverse cardiac involvement. In the cases with intractable congestive failure, surgical treatment must be recommended.

Adrenal Cortex Hormones↗
PubMed1984

[Medium chain acyl-CoA dehydrogenase deficiency. Apropos of a case with demonstration of this enzyme deficiency].

The medium chain acyl-CoA deshydrogenase defect: a new inherited metabolic disorder. This enzymatic defect blocks the catabolism of non esterified fatty acids during fasting. Thus, this disease is revealed by a coma due to hypoglycemia in a young child; the presence of dicarboxylic aciduria in such a situation is the main evidence for this diagnosis. Finally, the enzymatic studies performed on skin fibroblasts show a defect in medium chain acyl-CoA deshydrogenase. When a child is investigated away from a coma episode, the ketotic diet induces dicarboxylic aciduria but must be performed in an intensive care unit for its dangers.

Acyl-CoA Dehydrogenase↗
PubMed1983

[Case of mucolipidosis type I with a primary alpha-D-neuraminidase deficiency].

The authors describe a case of mucolipidosis I, the 9th reported in the world literature. The diagnosis was suspected in a 5 year old boy, from the appearance of hurler-like facial features. Lumbar kyphosis, dysostosis multiplex, cherry-red macular spot and foam cells in the bone marrow and it was confirmed by the characteristic oligosacchariduria (on thin layer chromatography) and excess sialic acid in cultured fibroblasts which also showed profoundly diminished activity of alpha D Neuraminidase (on two substrates - N-acetyl-Neuramin Lactose and 4 Methyl umbelliferyl N-acetyl-Neuraminide). The parents had intermediate enzyme activities, suggesting heterozygotism. After a review of the literature, the authors attempt to define the place of mucolipidosis I in the sialidoses.

Acetylglucosaminidase↗
PubMed1983

[Coffin-Siris syndrome. Critical study of the literature apropos of a case].

The authors report a case of the Coffin Siris syndrome which associates a ungueo-digital syndrome (special by the bilateral aplasia or severe hypoplasia of nails and third phalanx of fifth toes and fingers) to other anomalies: facies with thinly fine hairs contrasting with bushy and dense eyebrows and body hypertrichosis, hypotonia and mental retardation. The connections of the Coffin Siris syndrome with the trisomy 9 p+ syndrome and the fetal hydantoïn syndrome are discussed.

Abnormalities, Multiple↗
PubMed1983

[Budd-Chiari syndrome caused by obstruction of the hepatic veins].

A Budd-Chiari syndrome was observed in a 11 year-old girl who was admitted to hospital for abdominal pain and distension, soon followed by severe shock. X-ray investigations, surgery and autopsy showed partial stenosis of the common portion of the left hepatic veins associated with a complete obstruction of other hepatic veins but without alteration of the inferior vena cava.

Autopsy↗
PubMed1983

[Primary gastroduodenal ulcers in children. A multicenter study of 58 cases of endoscopic or surgical diagnosis].

A retrospective cooperative survey allowed analysis of 58 endoscopically (56) or surgically (2) proven ulcers in children. Drugs or stress induced lesions and ulcers in neonates were excluded from the study. The distribution was as follows: 39 (67%) males, 19 (33%) females, 42 (72%) duodenal, 16 (28%) gastric; 45 children were older than 7 years; a family history of ulcer was present in parents and/or siblings of 12 patients (38%). Ulcer was diagnosed on the occasion of a complication in 39 children (67%). Endoscopy performed in 53 children first detected the ulcer in 51 (in 5 instances at the second examination only). In the remaining 5 children, ulcer was proven endoscopically after radiologic examination was performed. The endoscopic and radiologic data were compared at the time of diagnosis and during follow-up. 31 children were checked endoscopically 3 months after diagnosis and lesions were still present in 21. 10 children were operated on. In these children, evolution is unpredictable and endoscopic check-ups are necessary.

Adolescent↗