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Biomedical subjects

A Gaudric

Publications and source records attributed to A Gaudric.

At least 91 records · Page 5Linked 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↗
PubMed1994

[Bouchut tubercles and AIDS. Apropos of 3 cases].

Tuberculosis choroidal granulomas ("tubercules de Bouchut") are rare, even in patients with AIDS and active tuberculosis. The authors report the clinical and angiographical findings of three cases. Choroidal involvement was bilateral, with multiple lesions, mostly located at the posterior pole. The size of the granuloma ranged from 1/8 to 1/2 of a disc diameter. Fluorescein angiography showed early prolonged hypofluorescence and late moderate hyperfluorescence. The lesions remained stable for months despite treatment of tuberculosis, and then gradually subsided. In two cases, the choroidal granulomas were discovered before the diagnosis of disseminated tuberculosis and guided the investigations, which allowed the identification of Mycobacterium Tuberculosis bacillus in the body. In the third case, pulmonary tuberculosis had already been diagnosed. The number of tuberculosis cases is increasing together with the number of AIDS cases. This should make the observation of tuberculosis choroidal granulomas more frequent. The discovery of the typical aspects of granulomas described above during fundus examination of AIDS patients can help in diagnosing tuberculosis.

AIDS-Related Opportunistic Infections↗
PubMed1993

[Ablation of the internal limiting membrane, macular unfolding and visual outcome in surgery of idiopathic epimacular membranes].

The files of 40 patients operated on for epimacular membranes were reviewed in order to correlate the functional and anatomical results with the presence or absence of internal limiting membrane on histological specimens. Only the eyes with idiopathic membranes were considered for the study. Visual acuity and fluorescein angiography were obtained preoperatively and at the 6 months postoperative visit. The surface of the posterior pole was measured on fluorescein angiogram pre and postoperatively by computerized image analysis. Light microscopy looked for the presence of significant segments of internal limiting membrane. In 28 cases (group 1) long segments of internal limiting membrane were adherent to the epimacular membrane and in 12 cases (group 2) no internal limiting membrane fragments or only small fragments were present. Mean postoperative visual acuity was the same in the two groups; 64% of eyes from group I and 50% from group 2 had visual acuity superior or equal to 0.5 (NS). Image analysis showed that the posterior pole surface area increased by 25% in group 1 but only by 15% in group 2 (p < 0.025). Internal limiting membrane ablation does not preclude good visual recovery and is correlated with improved unfolding of the posterior pole.

Fluorescein Angiography↗
PubMed1992

Quantification of angiogenesis due to basic fibroblast growth factor in a modified rabbit corneal model.

The rabbit corneal angiogenesis assay was modified to allow quantification of the neovascular response induced by growth factors. Basic Fibroblast Growth Factor (bFGF) was used for this experiment. bFGF was diluted in phosphate buffer solution (PBS) in such a way that 2 microliter of solution contained concentrations ranging from 17 to 1,200 ng. Each drop was absorbed by a 1 x 2 mm particle of a dried 70% hydratable hydrogel and implanted into a mid-stroma corneal pocket, 2 mm from the limbus. Iodinated bFGF was used to measure growth factor diffusion in the cornea, which was found to be isotropic. Autoradiography showed that bFGF was stored in the cornea at both epithelial and endothelial level. Corneal neovascularization occurred on the second day after implantation and was maximal on the 7th day. At this time the neovascular surface was measured by planimetry on corneal photographs and compared with controls. This method allows precise definition of the dose of any substance to be implanted into the cornea and induces a rapid neovascular response, thus allowing quantitative evaluation of neovascularization within one week. A neovascular response was detectable for doses as low as 35 ng of bFGF and increased proportionally to the dose of bFGF implanted.

Animals↗
PubMed1992

[Surgery of idiopathic epimacular membranes. Prognostic factors].

Seventy consecutive cases of idiopathic epimacular membrane removal were reviewed in order to evaluate the prognosis factors of this surgery. The characteristics analyzed where the duration of the symptoms, pre and postoperative visual acuity and the macular changes on fluorescein angiography. The mean follow-up after surgery was 9 months. Mean preoperative visual acuity was 0.17 and the mean best postoperative visual acuity 0.43. Visual acuity improved by 2 lines or more in 81% of the cases and the mean improvement was 3.5 lines. The complications of epimacular membrane surgery included nuclear cataract (10 eyes underwent cataract surgery), peripheral retinal breaks (3%), and retinal detachment (3%) successfully operated. Three of the main preoperative data were related to better postoperative visual outcome: preoperative visual acuity: eyes with visual acuity of 0.16 or more tended to have better postoperative vision than eyes with poorer preoperative vision (mean postoperative visual acuity 0.5 and 0.34 respectively; p < or = 0.05); duration of visual impairment before surgery: eyes with a visual impairment of less than 2 years had better postoperative vision than the others (mean postoperative visual acuity, 0.5 and 0.3 respectively; p < or = 0.05); the absence of preoperative angiographic macular edema: eyes without preoperative macular edema had better postoperative vision than the others (mean postoperative visual acuity: 0.5 and 0.4 respectively; p < or = 0.05). The normal postoperative pattern of macular vessels and foveolar shape was also a statistically significant factor of good vision recovery.

Adult↗
PubMed1992

[Changes in the fundus in subacute sclerosing panencephalitis. Apropos of 23 cases].

Subacute sclerosing panencephalitis (SSPE) is a fatal slow encephalitis. It is related to a chronic infection of the central nervous system by a defective measles virus. Among 41 patients with SSPE, 23 had ocular involvement. The ophthalmologic involvement preceded the onset of neurological signs in 4 cases, they were detected at first presentation in 9 cases, and they occurred in the course of the disease in 10 cases. The ocular involvement was bilateral in 19 cases. Various ophthalmologic features were reported: retinitis (16 eyes), macular pigment disturbances (4 eyes), optical neuritis (33 eyes), visual agnosia (1 patient). The macular lesions were typical of the disease, but at the acute stage they were liable to be confused with toxoplasmosis. This complication of measles should disappear with the systematic vaccination of 12 month old children.

Adolescent↗
PubMed1991

Pseudophakic retinal detachment.

We reviewed 165 cases of non-referred pseudophakic retinal detachment. Fifty-three eyes (32%) had sustained significant complications due to cataract surgery prior to retinal detachment; 45 eyes (27%) presented with grade B, C or D proliferative vitreoretinopathy. The overall reattachment rate was 84%. This was significantly higher in the posterior chamber lens group than in the iris-fixated lens or anterior chamber lens groups (93 vs. 69 and 81%). Our data show that uneventful extracapsular cataract extraction plus posterior chamber lens implantation offers the best chance of successful detachment surgery.

Adult↗
PubMed1989

Immunological study of acidic fibroblast growth factor (aFGF) distribution in the eye.

During the last ten years, several groups, including the present authors, have detected growth factor activities in various ocular tissues, and the presence of a ubiquitous Eye-Derived Growth Factor (EDGF) has been described. More recently, isolation and characterization of this growth factor activity from the retina led to the identification of two molecules. These molecules were shown to be identical to other growth factors isolated from neuronal and non-neuronal tissues and are now designated as acidic and basic fibroblast growth factor (aFGF, bFGF). The biological function and the reason for the ubiquitous distribution of these factors remain unclear. Understanding may be improved by quantification of this distribution in various tissues during development. In the present study, specific polyclonal antibodies were raised against acidic FGF, aFGF was determined in various ocular tissues by enzyme immunoassay, and the localization of immunoreactive aFGF by immunohistological staining with fluorescent antibodies or with enzyme- or gold-labeled antibodies was studied. In almost all tissues tested aFGF was found; but the retina, cornea, and vitreous body contained the highest levels of aFGF per gram of tissue. In the retina, aFGF was associated primarily with the nerve fiber layer and the inner and outer segments of the photoreceptors, whereas corneal aFGF was detected in the cytoplasma of the basal layer of epithelial cells.

Animals↗
PubMed1989

[Indications for photocoagulation in idiopathic retinal periphlebitis].

Fourteen patients (23 eyes) with idiopathic retinal periphlebitis were followed during a mean time of six years. Argon laser photocoagulation was performed in 18 eyes: in 11 eyes for large retinal ischemic areas, and in 7 eyes for disc or peripheral neovascularization. Beneficial results were obtained in 72.5% of the treated patients. Laser photocoagulation seems to be useful to prevent or reduce ocular neovascularization associated with idiopathic retinal periphlebitis, and to prevent vitreous hemorrhage. Photocoagulation did not increase ocular inflammation and should be limited to ischemic areas.

Adult↗
PubMed1989

[Vitreous hemorrhage and neovascular proliferation in multiple sclerosis].

We report the case of a 40 year-old man with multiple sclerosis, who presented a bilateral proliferative retinopathy responsible for a vitreous hemorrhage. Examination of the periphery of the retina showed diffuse retinal periphlebitis; fluorescein angiograms showed a peripheral retinal ischemic syndrome with secondary neovascularization. Several recent studies have show that retinal periphlebitis is a frequent finding in multiple sclerosis; but to our knowledge a retinal ischemic syndrome with neovascularization has seldom been reported.

Adult↗
PubMed1989

[Collodion baby. Apropos of a case with eye manifestations].

We report a case of a male collodion baby, born at term of a consanguineous couple of Afghan origin. The new-born baby displayed a pseudo-dysmorphic syndrome predominant of the face, with a marked ectropion of the upper and lower eye-lids and chemosis, without corneal involvement. The condition improved with time but complete regression was not obtained. Collodion baby syndrome is a pathologic skin condition observable during the neonatal period. Long term evolution is towards ichthyosis in 90% of cases, and resolution in 10%.

Ectropion↗
PubMed1989

[Retinopathy in pancreatitis].

Three patients with retinopathy associated with acute pancreatitis were observed. Each of them had a long history of alcohol abuse. The first patient was asymptomatic. Second one developed moderate visual loss in one eye (visual acuity 20/40), with a fascicular defect on Goldmann visual field registration. Third patient complained with bilateral and severe visual loss, with a large central scotoma. Cotton-wool patches and hemorrhages were present in the mild types of retinopathy. These cases had a good prognosis. An ischemic edema of posterior pole was associated in severe type of retinopathy. In this case, fluorescein angiography showed occluded macular retinal arterioles. Visual prognosis was poor. Pathogenesis of the lesions is still discussed. The main hypothesis are fat emboli or activated complement-induced granulocyte embolus formation. Efficacity of corticosteroids is not known.

Acute Disease↗
PubMed1989

[Rothmund's syndrome].

Rothmund's syndrome was observed in a 9 year old boy. Parental consanguinity was present, but no siblings were affected. He presented with rapidly progressive bilateral cataracts. Visual recuperation was excellent after "in the bag" phakoemulsification and intercapsular implantation. There were no other ocular anomalies. Extensive erythematous skin changes appeared when the boy was 3 months old and, at the time of presentation, he had poïkiloderma predominantly in those areas exposed to light (face and hands). There were no associated anomalies of the nails or hair, no endocrine disorder nor other malformations. A brief review of this syndrome is presented and treatment of these cataracts discussed.

Cataract↗