[Xanthelasma: a rare sign of Wegener's disease].
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Biomedical subjects
Publications and source records attributed to P Labalette.
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Metastatic tumors of the iris are rare. They most commonly arise from a primary breast or lung neoplasm and are associated with widespread carcinomatosis. They appear as white pink masses; anterior uveitis and hypertony can be seen. They are most often associated with short life expectancy. We report four cases and we show the interest of radiotherapy.
Pseudoxanthoma elasticum (PXE) is a rare inherited connective tissue disorder which is characterized by elastic tissue degeneration involving the skin, eyes and arteries. We describe the case of a hypercholesterolemic 25 year-old woman with typical genetic dominant form of PXE revealed by distal limb artery involvement after a pregnancy. In young adults with arterial disease, a non atheromatous cause should always be sought for, even if risk factors for atherosclerosis are present.
PURPOSE: Type II measangiocapillary glomerulonephritis is related to dense deposits within the glomerular basal membrane and the basal membrane of the pigment epithelium (Bruch's membrane). Being a vasculitis, an angiographic study by indocyanine green (ICG) could possibly enlarge the semiologic features of this disease. METHODS: The indocyanine green angiographic changes in 3 patients with predialitic renal failure due to type II measangiocapillary glomerulonephritis (MCGN II) (dense deposit disease) are reported. A complete ophthalmologic examination, electroretinography, electro-oculography, fluorescein and indocyanine green angiography (ICG) were performed for each patient. RESULTS: Abnormal hyperfluorescent dots were seen on the same part of the fundus on both fluorescein and ICG angiography, though these locations were different for each of the three patients. These results seem to link the deposits to vascular changes within the choriocapillaris, which opposes them to drusen encountered in age related macular degeneration (ARMD). It appears that the choriocapillary lesions could be similar to the glomerular disease. CONCLUSION: Beyond the diagnostic challenges related to the nephrologic disease, it is known that subretinal neovascularization occurs in some cases of MCGN II, although the pathophysiologic mechanism of the deposits is probably not the same as in ARMD. Therefore, ICG angiography should be performed when MCGN II is first known, serving as an initial examination for further follow-up.
PURPOSE: To better characterize congenital anophthalmos and microphthalmos in order to distinguish which patients need surgical treatment. MATERIALS AND METHODS: A retrospective study of 42 cases with congenital anophthalmos and microphthalmos over a 16 years period was performed. Seven anophthalmos, 20 microphthalmic globes with no associated colobomatous orbital cyst and 15 microphthalmic globes associated with colobomatous orbital cyst were observed. Complete history, pediatrical and ophthalmological examination, electrophysiological feature, oculo-cerebral imagery and karyotype on each of the patients were reviewed. RESULTS: Among all patients, lack of development of the lids was observed in 45% of cases. In our group of anophthalmos, 100% had micro-orbit. In our group of microphthalmic globes with no associated colobomatous orbital cyst, 30% had micro-orbit and in our group of microphthalmic globes associated with colobomatous orbital cyst, 6% had micro-orbit. 75% of patients had ocular anomalies and 39% had systemic anomalies, mostly on the face. Aetiology were found in 36% of cases. Visual evoked potentials and retinal electric feature were useful to better determine visual function. CONCLUSION: Expandable orbital prosthesis would appear to be the most effective therapy for certain cases of anophthalmos and microphthalmos with micro-orbit.
The diagnosis of sarcoidosis requires histopathological analysis. Easy accessible site of biopsy is seldom. A systematic labial salivary gland biopsy was performed in 62 suspected sarcoidosis: 22 patients with Löfgren syndrome (group I), and 40 patients with systemic sarcoidosis (group II). Systematic bronchial biopsy was performed in eight patients of group I and ten patients of group II. If systematic biopsies were negative, direct biopsies were performed (lymph node, skin, kidney, liver). In group I, 8/22 labial salivary gland biopsies and 1/8 bronchial biopsies were positive; in group II, 17/40 labial salivary gland biopsies and 5/10 bronchial biopsies were positive. In the other patients, direct biopsies were positive: 27 lymph nodes, eight skin, eight hepatic, four kidney biopsies. In conclusion, labial salivary gland biopsy (even in the absence of sicca syndrome) is more reliable than systematic bronchial biopsies, particularly in Löfgren syndrome and may avoid in 30 to 50% of the cases more aggressive and dangerous biopsies such as liver, kidney or deep lymph nodes biopsies.
Thirty-five patients with toxoplasma retinochoroiditis, receiving medical treatment and then treated with laser photocoagulation around the foci, were retrospectively evaluated for the risk of recurrence of the retinochoroiditis with a Kaplan-Meier representation. The recurrence rates with 95% symmetric confidence intervals were: at 1 year, 12.7 + or - 13%, at 2 years, 19.8 + or - 15%; at 3 years, 24.0 + or - 16%; at 4 years, 33.3 + or - 19%; at 5,6 and 7 years, 53.5 + or - 21%; at 8 and 9 years 66. 8 + or - 22%. With the data provided by our series, it is not possible to show the efficacy of laser photocoagulation as a prevention of recurrence in toxoplasma retinochoroiditis. Moreover, because of their heterogeneity, the recurrence rates from the literature cannot provide precise data for a comparison. Concerning the laser-induced thermal damage, the potential therapeutic mechanism of the laser procedure is discussed.
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OBJECTIVES: Different diagnostic criteria for Behçet's disease are proposed in the literature. We compared the criteria proposed by Mason and Barnes with those proposed by the International Study Group and with those proposed by the Japanese committee. METHODS: This retrospective study included 41 patients with Behçet's disease according to the criteria set by Mason and Barnes. There were 16 patients of French origin and 25 patients of North African origin. RESULTS: Genital ulcerations were more frequent in the North African population (p < 0.007) and typical Behçet skin lesions were more frequent in the French population (p < 0.021). Comparing the clinical data among the 41 patients with Behçet's disease according to the Mason-Barnes criteria revealed that only 83% met the criteria set by the International Study Group and only 54% the criteria of the Japanese committee. For the International Study Group criteria, the requirement for recurrent buccal ulcerations with at least 3 episodes over a 12 month period (a major criteria) was the most limiting element. Likewise, genital ulcerations were not always recurrent. CONCLUSION: These findings confirm the variability of clinical manifestations of Behçet's disease with ethnic origin. Proposed diagnostic criteria vary depending on the study committee and should be used as diagnostic guidelines rather than as criteria required for positive diagnosis.
PURPOSE: This study aimed to determine usefulness of repeating tonometry and averaging of the results in order to increase precision. METHODS: Variance analysis was performed, the intra class correlation coefficient was calculated to determine the confidence intervals (95%) for averaging with two or more results. RESULTS: For one measurement the confidence interval was 7.07 mmHg, for 2 measurement, it was 5.00 mmHg and for 3 measurement 4.08 mmHg. For more measurements, there was little more improvement in precision. CONCLUSION: We therefore recommend to perform the tonometry twice, averaging the 2 results, in order to increase the precision of the final result. If more precision is required a third measurement and averaging of the 3 results can be made. Although it was proven that the influence of carefully repeated aplanation tonometry is not significant on the resultats for 2 or 3 measurements, averaging could be done during a single consultation.
Human infections due to Aspergillosis Fungi are unusual and occur preferentially in defenseless patients. Nonetheless, our patient, a seed-shop owner, was not immuno-depressed and became infected by inhaling or chewing the seeds. Moreover, the aspergillosis was situated in the sphenoidal sinus, which is extremely unusual. In fact, the initial pseudo tumoral and misleading appearance justified a surgical biopsy which revealed a black "truffle" suggestive of aspergillosis and was confirmed by anatomo-pathology.
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