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Biomedical subjects

P Bioulac

Publications and source records attributed to P Bioulac.

At least 55 records · Page 3Linked to original sources
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↗
PubMed1980

Cutaneous hypermelanosis and intramelanotic lipid droplets.

Intramelanocytic lipid droplets have been observed in 12 patients with different types of hypermelanosis of the skin. These include drug-induced hypermelanosis, hypermelanosis secondary to use of physical agents, hypermelanotic morphea, generalized scleroderma with diffuse hypermelanosis, a hyperkeratotic tumor in xeroderma pigmentosum, aberrant mongolian spot, and methoxsalen-ultraviolet-A-induced hypermelanosis. Lipid storage was selectively observed in melanocytes. Most of the melanocytes with lipid vacuoles showed ultrastructural signs of hyperactive melanosome synthesis and certain cytoplasmic abnormalities such as melanosomal autophagic vacuole formation and mitochondrial alterations. The importance and the origin of the lipid droplets remain unclear.

Adult↗
PubMed1979

[Ultrastructural study of the different zones of the rat liver acinus after portacaval anastomosis].

Liver atrophy is a main feature in rats with a porto caval shunt. Histological studies revealed small size hepatocytes. Ultrastructural differences between periportal and centrolobular zones were noticed, in particular, the dilatation of the nuclear envelope and of the rough endoplasmic reticulum which appeared dilated, desorganized and sometimes without ribosomes, was more pronounced in the periportal zone. Hepatocytes of this zone might be more sensitive to the decrease of O2 and/or hepatotrophic factors.

Animals↗
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↗
PubMed1977

[Kaposi's sarcoma and malignant lymphoma. Discussion of this association (author's transl)].

Kaposi's sarcoma was diagnosed in a 62 year old female at the last stage of an indefined malignant lymphoma. Clinically and histopathologically, some cutaneous aspects were consistent with mycosis fungoides. However, the histological examination of lymph-nodes led to the diagnosis of Hodgkin's disease, sometimes associated with features of Kaposi's sarcoma. During the past few years, the occurrence of Kaposi's sarcoma in the course of cancers, malignant lymphomas and especially during Hodgkin's disease, has been reported. It has been suggested that immunosuppressive therapy undertaken for the initial tumor could account for a viral carcinogenesis. In this respect, the vascular proliferation observed in Kaposi's sarcoma could be compared with the lymphocyte induced angiogenesis, which occurs during the experimental graft versus host reaction. Therefore, Kaposi's sarcoma could be the result of a tumoral rejection. In the case of our observation, it does not seem possible to diagnose an angio-immunoblastic lymphadenopathy; but it is of interest to note that, in this later disease, histological features of vascular neogenesis have also been reported.

Diagnosis, Differential↗
PubMed1975

The diagnosis of mucopolysaccharidoses by electron microscopy of skin biopsies.

An ultrastructural examination was carried out on the skin of six children suffering from Mucopolysaccharidosis I (MPSI or Hurler's disease) and MPS II (Hunter's disease). Both intracellular mucopolysaccharides and lipids were observed. The dermal cells, i.e. fibroblasts, macrophages, were loaded with multiple vacuolar inclusions thought to be of lysosomal origin. They appeared either content-free or filled with granular, fuzzy and/or pseudomyelinic structures. Identical abnormalities were observed within the Schwann cells, smooth muscle cells and keratinocytes. Mast cells showed peculiar "worm-like" inclusions apart from their normal granulations. Since ultrastructure of a skin sample may provide as much data as brain, liver or kidney, cutaneous electron microscopy can be recommended to confirm a diagnosis of MPS.

Cell Nucleus↗