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Biomedical subjects

P Dhermy

Publications and source records attributed to P Dhermy.

At least 19 recordsLinked to original sources
PubMed1989

Terrien's disease: clinical and ultrastructural studies, five case reports.

Terrien's disease occurs in middle-aged patients and is characterised by an insidious thinning of the cornea near the limbus. In most cases, this results in a peripheral ectasia associated with a severe degree of astigmatism. Inflammatory signs are rarely observed in this marginal corneal degeneration which is of unknown aetiology. Electron and light microscopic studies have been performed on five specimens from penetrating keratoplasties. Anatomical and clinical correlations showed the marked marginal degenerations of the corneal stroma to be consistently associated with lipid deposits, but without inflammatory cell infiltrate. These changes are in agreement with previous reported pathological descriptions of Terrien's disease.

Adult↗
PubMed1989

[Terrien's disease, apropos of 6 cases. Ultrastructural study].

Terrien's disease is characterized by slowly progressive thinning of the pre-limbal cornea in middle-aged patients. It usually leads to peripheral ectasia with marked astigmatism. It is rarely accompagnied by inflammation and its cause is unknown. Very few histopathologic examinations have been published, in particular those using electron microscopy We present detailed results concerning 6 patients in whom perforating keratoplasty permitted histopathologic and electron microscopic examination. Clinical and histological features reveal marked degeneration of the peripheral corneal stroma, with lipid accumulation unaccompagnied by significant inflammatory cellular infiltration in each case. Of interest is the association in one patient with keratoconus. The generally accepted pathogenic theories are fully discussed. The wealth of electron microscopic data provides a complete review of stromal degenerative changes.

Adult↗
PubMed1988

[Histiocytofibroma of the sclerocorneal limbus].

A case of epibulbar benign fibrous histiocytoma is presented, with an optical microscopic study. A greyish vascularised nodule developed at the corneoscleral limbus in a 65 year-old male. The clinical diagnosis was confirmed by histopathologic evaluation. Complete surgical excision had a curative effect.

Aged↗
PubMed1987

Ocular changes in some progressive hereditary nephropathies.

Ocular involvement is a common feature of three hereditary nephropathies: cystinosis, nephronophthisis and Alport's syndrome. The follow-up of 25 cases of infantile cystinosis over a period of 26 years demonstrated that the corneal and retinal epithelium were affected in the same way as the kidney epithelium. Corneal involvement induced photophobia and discomfort, but actual blindness was mainly due to retinal involvement, and therefore no corneal graft was performed. The use of topical cysteamine appears to be promising, but its production raises many questions so no definitive conclusions may be made. Since 1965, 51 patients with nephronophthisis have had ocular examinations; all patients had characteristic clinical symptoms and histological findings. The first group, which consisted of 18 children, all under 10 years of age, was found to have obvious chorioretinal degeneration. The second group, which consisted of 11 children, had a normal ocular examination and normal electroretinogram (ERG). The third group, consisting of 22 children, had a normal clinical examination but ERG tracings with variable alterations. In 28 instances of Alport's syndrome, two types of ocular abnormalities have been observed. In six cases, an anterior lenticonus was noted, which caused a major decline in visual acuity. Retinopathy, which did not affect vision, was observed in 13 cases.

Adolescent↗
PubMed1987

Acute corneal edema in pellucid marginal degeneration or acute marginal keratoconus.

This article reports a case of bilateral corneal pellucid marginal degeneration. The right cornea had an acute hydrops. Both eyes underwent penetrating keratoplasty. A histopathological study of the corneal specimens was performed by light and electron microscopy. The histological changes observed on the right cornea showed breaks on Bowman's layer, edema and disorganization of the stromal collagen, and break of Descemet's membrane. The ultrastructural changes were similar to those observed in acute keratoconus, leading to the belief that these two corneal diseases are closely related.

Acute Disease↗
PubMed1987

Adenocarcinoma of retinal pigment epithelium.

This report describes a 41-year-old man with an intraocular tumour misinterpreted clinically as choroidal melanoma. The fluorescein angiographic features were not fully characteristic of uveal malignancy, and indeed histopathology revealed the diagnosis of adenocarcinoma of the retinal pigment epithelium. It is suggested that, in cases with the fundus and angiographic findings described here, the rare possibility of adenocarcinoma of retinal pigment epithelium should be kept in mind. Of particular interest were the changing pathological findings in the various parts of the tumour, which paralleled the fluorescein angiographic pattern.

Adenocarcinoma↗
PubMed1987

Ocular changes in long-term evolution of infantile cystinosis.

Infantile cystinosis is an autosomal recessive lysosomal disorder of aminoacid metabolism leading to a storage of cystine crystals in the cells of many tissues, but mainly in the kidney and the eye. The ocular symptoms and long term evolution were studied in a series of 25 patients at the Enfants Malades Hospital. The follow-up over 26 years demonstrated that infantile cystinosis affected mainly corneal and retinal epithelium just as it affected the kidney epithelium. Corneal involvement was a constant finding after one year of age. It induced photophobia, which appeared between three and four years when a superficial punctate keratopathy appeared. A characteristic retinopathy was observed as early as three years of age. It was constantly present at seven years of age and caused loss of vision. When ERG results were compared to the most recent measurement of visual acuity, a correlation was observed between retinopathy and visual defect. For this reason, no corneal grafts were performed. The use of topic cysteamine appeared promising but no definitive conclusions could be made.

Adolescent↗
PubMed1987

[Acanthamoeba keratitis. Clinical, histological and ultrastructural study].

Acanthamoeba keratitis is a rare but very severe necrotizing stromal keratitis due to a non-parasitic free-living soil and freshwater amoeba. It affects more readily soft and hard lens wearers. It is often clinically mistaken for herpetic keratitis because of the similarity of the signs and the difficulty in performing the specific microbiological tests. This is the reason why the diagnosis is most often made at the time of penetrating keratoplasty and histological study of the buttons with special stains. Medical treatment is effective only when starting at the beginning of the disease.

Acanthamoeba↗
PubMed1987

[Orbital localization of clear-cell sarcoma of tendons and aponeuroses. Apropos of a case report].

Clear cell sarcoma of tendons and aponeuroses actually is a well identified soft tissue tumor. A primary orbital involvement is presented, with an optical and electron microscopic study. The orbital topography of such a tumor seems to be an exceptional opportunity. The histological study showed the typical pattern of this clear cell tumor. Ultrastructurally, there are not absolute specific features to establish the diagnosis, except the finding of intra-cellular melanosomes. Histogenesis has been debated for a long time between a melanotic or a synovial origin. According to present knowledge, this tumor may be attributed to a neural crest origin.

Adult↗