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Biomedical subjects

M Binaghi

Publications and source records attributed to M Binaghi.

At least 19 recordsLinked to original sources
PubMed1994

[Does hemangioblastoma exist outside von Hippel-Lindau disease?].

Hemangioblastoma may arise in isolation ("sporadic" cases) or as a major manifestation of von Hippel-Lindau (VHL) disease, an autosomal dominant disorder with a prevalence of at least 1/36,000. In addition of central nervous system hemangioblastomas (cerebellum, spinal cord and retina), affected patients may develop renal cysts or carcinomas, pheochromocytomas and pancreatic cysts. A multidisciplinary group including neurosurgeons, geneticists, pathologists and clinicians from all involved specialities has been organized to develop a national registration of all hemangioblastoma and VHL patients. The findings of a preliminary 10-year study (1983-1993) conducted in France are presented. Two hundred thirteen cases of hemangioblastoma were reviewed for their location and genetic features. The majority (77%) of the tumors were located in the cerebellum whereas 23% were located inside the spinal canal. By thorough clinical examination of the patients and systematic genetic inquiry of their family background, it was found that 34.3% of the total (58.7% before age 30) were afflicted with VHL disease. Spinal hemangioblastomas were more often related to VHL disease than infra-tentorial locations (50% versus 36.6%). In addition, mean age at diagnosis in VHL disease was significantly younger than in sporadic cases (33.5 +/- 10 versus 43.6 +/- 15 years). Recent progress in VHL molecular genetics led to the identification of the mutated gene to the distal part of the short arm of chromosome 3 (3p25-3p26), paving the way to presymptomatic diagnosis and, hopefully, to elucidation of pathogenesis, which may offer a further glimpse into tumorigenesis in general. Because of the usually early adulthood onset, accurate presymptomatic diagnosis of affected members would be of great benefit to VHL families. However, the fact that very few mutations in the VHL gene are identified precludes molecular diagnosis of "sporadic" hemangioblastomas. In summary, this study reveals that VHL-related hemangioblastoma is a more common clinical problem that it was previously reported. Thus, all patients with an apparently isolated central nervous system hemangioblastoma should be investigated for evidence of VHL disease.

Adolescent↗
PubMed1987

Eight-year follow-up of central serous chorioretinopathy with and without laser treatment.

A long-term retrospective study was conducted on two groups of patients affected with typical central serous chorioretinopathy (CSCR). One group was not treated and the other was treated by direct argon laser photocoagulation. CSCR shows a long-term good prognosis of visual acuity. The visual acuity of laser-treated eyes improves significantly (P less than or equal to 0.05), particularly in single focus cases (P less than or equal to 0.01). Unfortunately, these results cannot be statistically compared to the natural course of the non-treated patients because of the initial inhomogeneity of the two groups. Laser treatment did not produce any long-term complications. The recurrence rate in treated and untreated eyes was similar.

Adult↗
PubMed1985

Sjögren-like syndrome after drug-induced toxic epidermal necrolysis.

7 of 9 patients recovering from a drug-induced toxic epidermal necrolysis (TEN) which they had had 2 months to 4 years before had xerostomia, or keratoconjunctivitis sicca, or both. 5 patients had symptoms; 2 had severe visual impairment. In 5 cases there was lymphocytic infiltration of small salivary glands; in 2 patients this was identical to that of Sjögren syndrome. None of the patients had antinuclear antibody. TEN may occur during acute graft-versus-host disease (GVHD) and these observations are reminiscent of the sicca syndromes reported during chronic GVHD. They suggest that autoimmune mechanisms may be involved in drug-induced TEN and that Sjögren syndrome may occur as the result of a drug reaction.

Adult↗
PubMed1985

[Ocular sequelae and sicca syndrome following Lyell's syndrome].

The mucous lesions are customary in toxic epidermal necrolysis. The acute ocular lesions have led to definitive sequelae. 22 out of 32 patients surviving to a Lyell's syndrome lend themselves to an ophthalmological supervision. 13 of these 22 patients (59 p. 100) presented a sicca syndrome with a decrease of the lacrimal secretion (12 cases) and/or salivary secretion (8 cases). The sicca syndrome appeared sometimes from the beginning of the acute phase of the Lyell's syndrome or more often a few weeks later. This decrease of the lacrimal flux went along with objective corneal lesions among 11 patients, and 6 of them had a change of the visual function. A biopsy of the accessory salivary glands was performed on 7 of these patients having a reduction of the salivary flux. 5 of these 7 biopsies showed lymphocytic infiltrates giving in 2 cases a nodular aspect, grade III of Chisholm's classification, held to be pathognomonic of Sjögren syndrome. The 13 patients having presented a sicca syndrome differed from the other 9 patients (without sicca syndrome) by a higher severity of their Lyell's syndrome and by a higher age. None of our patients with dry syndrome had antinuclear antibody detected by immunofluorescence on sections of rat's livers. Sicca syndromes with sometimes lymphocytic infiltrate similar to those of Sjögren's syndrome were occasionally imputed to drug reactions. The occurrence of "Sjögren like's syndrome" after a toxic epidermal necrolysis suggests the intervening of autoimmune phenomena in the pathogenesis of this toxic epidermal necrolysis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1985

[Hypereosinophilic syndrome with ocular involvement].

The authors report a case of idiopathic hypereosinophilia with Löffler endocarditis in a 19 year old male. Retinal arteriolar occlusions were observed in the pre-equatorial region and documented by angiography. Histologic examination of the globes showed obliteration of the arterioles by a fibrinous coagulum without signs of vasculitis. Clinical data appears to suggest an embolic origin of the occlusions rather than thrombosis in situ.

Adult↗
PubMed1985

[Ocular complications of Lyell's syndrome: recent concepts apropos of 26 cases].

Acute ocular lesions are usual during Toxic Epidermal Necrolysis (T.E.N.) and may induce persistent alterations. These were thought to be of cicatricial nature. 26 patients recovering from TEN had a systematic ophthalmological follow-up of at least six months after the acute stage (mean: 3 years). 11 of 26 patients (42%) exhibited a dry eye, associated in 7 with decreased salivary flow. The sicca syndrome appeared during the acute phase of TEN or, more often, a few weeks later. The reduction of the lacrymal flow induced corneal lesions in all 11 patients and 6 patients suffered permanent visual impairment. Biopsies of labial accessory salivary glands showed a lymphocytic infiltration of the glandular tissue in 5 of 7 cases. In 2 cases the lymphocytic infiltrate was nodular, grade III of Chisholm's classification, considered as pathognomonic of Sjogren's syndrome. The occurrence of Sjogren-like syndrome in patients recovering from TEN suggests an auto-immune pathogenesis for TEN, and is one more analogy between TEN and graft-versus-host disease.

Conjunctival Diseases↗
PubMed1984

[Fluorescein angiography in the diagnosis of periarteritis nodosa].

Periarteritis Nodosa (P.A.N.) is a systemic connective tissue disease with a variety of manifestations that includes ocular involvement in 20% of cases. The diagnosis of this condition is difficult due to the absence of any specific clinical signs or laboratory findings. However, histologic studies have demonstrated a segmental vasculitis that is often necrotic. Ocular findings frequently include choroidal involvement that is characteristic. Nevertheless, angiographic studies of this disease are extremely rare. The findings in three patients suspected of having P.A.N. are presented. Fluorescein angiography established the diagnosis of P.A.N. in two cases and ruled-out its presence in the third case. In the first case angiography demonstrated a retinal vasculitis with multiple arteriolar and capillary occlusions. There was also ischemic involvement of the choriocapillaris and a mild anterior optic nerve vasculitis. All findings resolved, leaving numerous Elschnig spots. In the second case the angiogram showed acute multifocal ischemia of the choriocapillaris. The ocular examination and fluorescein angiogram in the third case were entirely normal, thereby ruling-out P.A.N. on the basis of insufficient criteria. Acute multifocal choroidal ischemia is present in a variety of rare conditions: Toxemia of pregnancy, Disseminated Intravascular coagulopathy, Moskowitz Disease (T.T.P.), Leukemia and Malignant Hypertension. However, the presence of multifocal choroidal ischemia in the presence of a systemic connective tissue disorder strongly favors the diagnosis of P.A.N. The relative contributions of co-existent Malignant Hypertension and P.A.N. in producing choroidal ischemia are discussed. The spectrum of clinical manifestations and laboratory findings in P.A.N. as well as hypotheses concerning pathogenesis (immune-complex deposition) are described. Among all systemic vasculitis , only P.A.N., and rarely Scleroderma, feature choroidal involvement. This is possibly due to the fact that the degree of vasculitis in P.A.N. is sufficiently severe to cause clinically significant choroidal involvement.

Adult↗
PubMed1984

[Effects of red krypton and monochromatic green argon lasers in the foveal region. A clinical and experimental study].

The destruction of senile neovascular membranes in the central avascular region of the macula using argon laser is difficult and associated with complications. Yellow pigment in this region absorbs the blue component of the conventional argon laser radiation. In this study, we compared laser of different wavelenghts: red krypton and green argon. Light and electron microscopic studies on non-human primates were performed at one hour and three weeks following laser burns. With the red krypton laser maximum damage was seen at the level of choriocapillaris and the choroïd, although the pigment epithelium was also destroyed. With the green argon laser the maximum absorption occurred at the level of the pigment epithelium. A clinical and angiographic study showed that the destruction of juxtafoveal neovascular membranes by both these lasers was possible after one or several treatment sessions. After an average follow-up period of 45 months, visual acuity was stable or improved in 10 out 17 eyes treated with the green argon laser and in 27 out of 53 eyes treated with the red krypton laser. Our results suggest that the red krypton laser offers a better alternative for the treatment of neovascular membranes located near the foveola since less unnecessary damage is induced. Outside the xanthophyllic area there is no major difference between the different wavelengths.

Animals↗