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Biomedical subjects

C Beylot

Publications and source records attributed to C Beylot.

At least 163 records · Page 9Linked to original sources
PubMed1983

[Arterial manifestations of the Ehlers' Danlos syndrome].

The Ehlers-Danlos syndrome, or rather syndromes includes a heterogeneous group of diseases secondary to abnormalities of collagen fibres, for certain of which the specific biochemical defect has been identified. The preponderance of collagen fibers in the body explains the diversity of clinical manifestations which can occur as part of this form of hereditary connective tissue dysplasia. The involvement of the arteries is the most serious manifestation and determine the prognosis of the disease. The rupture of arteries, true vascular catastrophy occurring in young patients, is most characteristic of the type IV Ehlers-Danlos syndrome due to a deficiency in type III collagen. Aneurysms, and less frequently stenoses are also manifestations of the structural abnormality of the arterial walls. After a brief review of the other clinical manifestations of the Ehlers-Danlos syndrome, the authors analyse the Literature regarding the signs of arterial involvement in view of such a degree of arterial fragility, the authors underline the difficulties involved with surgery and the dangers of arteriography.

Aneurysm↗
PubMed1981

[Hereditary porphyria cutanea in children. Enzymatic studies (author's transl)].

A girl of 7 years presenting porphyria cutanea tarda was found to have a very low level of erythrocytary uroporphyrinogen decarboxylase. Tests on her brother, parents and maternal grand-parents, who were all free of the disease revealed the same level of enzyme in the mother and grandmother, through whom the hereditary factor was obviously transmitted. This supports evidence provided by juvenile cases and familial forms in the literature that heredity plays an important part in a disease which is often considered to be acquired or symptomatic. Despite clinical and biological resemblances, two types of porphyria cutanea can be distinguished: a sporadic form in which enzyme deficiency occurs only in the liver and is revealed by exogenous toxic factors such as alcohol or oestrogens and an hereditary form where the deficiency transmitted by a dominant autosomal mode is found in all hemasynthetising cells.

Child↗
PubMed1980

Unscheduled DNA synthesis in psoriatic skin after ultraviolet irradiation and the effects of a combined treatment with 8-methoxypsoralen and longwave ultraviolet radiation: a clinical study.

Biopsies were taken from psoriatic patients undergoing photochemotherapy and then studied by autoradiography to investigate possible unscheduled DNA synthesis (UDS) following 8-MOP photodamage in epidermal cels. No sparse labelling was found in seventy samples taken from twenty-four different patients during PUVA therapy, indicating a lack of UDS. In contrast, UDS was observed in three patients irradiated with an all-spectrum UV lamp, as a test for DNA damage and excision repair activity. The incorporation of 3HTdR in these keratinocytes which were not undergoing S-phase was not affected by the dose of UV-A received during different stages of PUVA treatment. The results are discussed in terms of repair of the induced photodamage at the level of DNA, and in relation to a possible risk of this treatment when it is given over a long period of time.

Adolescent↗
PubMed1980

[Degos' malignant atrophic papulosis. Ultrastructural study of a new case].

The authors report a case of Degos' malignant atrophic papulosis (MAP) with typical skin lesions from a clinical and histological standpoint. They stress in particular the ultrastructural study of skin biopsies which revealed numerous "paramyxovirus-like" tubulo-reticular structures in the endothelial cells, lymphocytes of the perivascular infiltrate, fibroblasts and keratinocytes. Although the significance of these inclusions remains open to discussion and is probably non-specific, their frequency in MAP is worthy of emphasis.

Adolescent↗
PubMed1980

[Interest of ultrastructural study of skin and muscle biopsies in inborn storage diseases. A report of 18 cases (author's transl)].

Skin and muscle biopsies were performed in 18 patients affected by various inborn storage disorders: mucopolysaccharidosis (MPS), sphingolipidosis, GM1 gangliosidosis, I-cell disease, ceroid-lipofuscinosis (CLF), adrenoleucodystrophy (ALD) and glycogenosis. In most cases, cutaneous and muscle biopsies demonstrate clearly the presence of storage inclusions in different cell types with great reproducibility and sometimes a specificity for a particular disease. Thus, electron microscopic examinations of skin and muscle, often complementary, can given valuable informations at two levels: - either a morphological confirmation depending on the type of the storage disease: e. g. clear, granular and fuzzy inclusions identified with mucopolysaccharidic material, lamellar and pseudomyelinic figures corresponding to lipidic structures are found in different cell types in MPS, gangliosidosis and sphingolipidosis; - or a diagnostic proof of some lysosomal diseases where there are very specific ultrastructural features such as the curvilinear bodies and fingerprint profiles in CLF or the spicular inclusions in ALD. These informations are the more important as the enzymatic defect is unknown in these inherited disorders. We conclude that the ultrastructural examination of a skin and muscle biopsy are an important tool for the diagnosis of a lysosomal storage disease which avoids any more difficult biopsies as brain, kidney, liver.

Adolescent↗
PubMed1979

Particular clinical features of psoriasis in infants and chidren.

The onset of psoriasis is observed before the age of 10 years in 15% of all patients. The clinical pattern often takes on a peculiar form. Psoriasis guttata--or less frequently nummular psoriasis--is the initial phase during childhood. It is very difficult to establish a diagnosis on the basis of incipient features when childhood psoriasis is located on the head, palms, soles, or on the fingers, toes and nails. Intertriginous or flexural psoriasis, psoriasis spinulosa and oral psoriasis is also described. The onset of napkin psoriasis starts at the age of 3 months. Occasionally, Leiner's disease may develop into a typical, chronic psoriasis. We have concentrated our studies on the less common features of childhood psoriasis: the generalized pustular infantile form, congenital psoriatic erythroderma, acquired psoriatic erythroderma, and infantile arthropathic psoriasis.

Adolescent↗