[Myocardiopathy in POEMS syndrome].
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Biomedical subjects
Publications and source records attributed to H Merle.
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The purpose of this study was to confirm that ophthalmological features seen in patients in Martinique, French West Indies, could be linked to infection by HTLV-I. The authors studied 93 HTLV-I infected patients divided into 70 patients with HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) and 23 asymptomatic HTLV-I carriers. They did a complete ophthalmological examination with an assessment of lacrymal secretion by means of three tests: Shirmer 1, break-up time and rose Bengal. Some patients had a biopsy of secondary salivary glands. When possible, detection of HTLV-I antibodies was carried out in the aqueous humor. In 45 of the 93 patients (48.4%) the presence of dry keratoconjunctivitis was recorded. In 22 of these 45 cases, a biopsy of the secondary salivary glands showed the presence of lymphoplasmocytoid infiltrations comparable to the glandular changes that occur with Gougerot-Sjögren syndrome. Among the 93 patients, 15 cases of uveitis were noted (16.1%) with 13 cases of anterior uveitis and 11 cases of vitritis. The inflammation was bilateral in 9 cases (9/15 = 60%). Two cases of cotton wool spots, 3 cases of abnormalities in the distribution of the retinal pigment and 7 cases of corneal lesions were also noted. Higher levels of anti-HTLV-I antibodies were detected in the aqueous humor of 3 patients with uveitis. The coexistence of dry eye (keratoconjunctivitis), uveitis and retinal microangiopathy in patients who are suffering from HAM/TSP could suggest the involvement of an autoimmune or immunological mechanism in the physiopathology of the illness.
Among 50% of patients with hyperthyroidism develop manifestations of ophthalmopathy and there are between 2% and 5% with severe Graves' ophthalmopathy. The optic neuropathy is a complication of Graves' disease and can be very dangerous for the visual acuity and the treatment must be very fast. We report two cases of severe Graves' ophthalmopathy observed in two black patients from Martinique (French West Indies). Orbital irradiation can be choosed after initial unsuccessful treatment with corticotherapy and before a surgical decompression. These three therapeutics can be combined together according to the results of the treatment and require a real coordination between ophthalmologists, endocrinologists and radiotherapeutists as well as a good cooperation from the patient himself.
We report three cases of isolated medial orbital wall blow out fracture with medial rectus entrapment. Few cases have been reported in the literature, predominantly in black people as in this paper. Predominance of this rare fracture in black population could be explained by an anatomic hypothesis based on racial origin. The diagnosis is often suggested by the medial rectus entrapment. Depending on the position of the incarceration in front of or behind the equator of the eyeball, the eye presents pseudo-Duane syndrome or exotropia with decreased adduction. The diagnosis is established by orbital computed tomography visualizing the lamina papyracea of the ethmoid bone fracture and the medial rectus incarceration. This exceptional fracture must be detected to avoid complications: enophthalmos and motility disturbances.
The objective of this study is to describe the clinical and immunological aspects observed in patients with both "tropical spastic paraparesis/HTLV-I associated myelopathy" (TSP/HAM) and ocular dryness. In 10 such patients clinical and biological examinations completed with a biopsy of secondary salivary glands were performed to assess the etiology of the ocular dryness. According to the Chisholm's scale, 70% of the patients had a biopsy grade 3 or grade 4. Polyclonal hypergammaglobulinemia was found in 90% of patients and lymphocytic alveolitis in 80%. Three patients had past medical history of chronic uveltis. All the findings were compatible with Sjögren's syndrome, however no characteristic immunological disorders were found. Antinuclear antibodies and rheumatoid factor proved negative in all cases.
OBJECTIVE OF THE STUDY: The objective of this study is to enter on an inventory and to appreciate the severity of the ocular burn caused by the latex of manchineel tree and to propose a therapeutic attitude. MATERIAL AND METHOD: We report on the results of a prospective study included 11 patients examined in the Department of Ophthalmology (Centre Hospitalier Universitaire de Fort-de-France) between November 1992 and November 1993. All of them presented with ocular burn due to a contact with the latex of the manchineel tree. RESULTS: The study included 11 patients, 8 adults and 3 children, the mean age was 22.9 years (range 4-40). All of them had hyperhemia and conjunctival erosions of various intensity. Ten patients had corneal lesions: superficial punctate keratitis in 5 cases and corneal ulcers in 5 cases. Three cases of corneal ulcerations were associated with an inflammatory reaction of the anterior segment. Both eyes were affected in 4 cases (37%), and only the right one in the other cases (63%). Seven patients had cutaneous lesions (63%), of whom 4 periocular burns of superficial second degree. Full recovery was obtained within 15 days for all the patients. CONCLUSION: Ocular lavage is the first treatment, as for all chemical burns. Local antibiotherapy is used systematically to prevent superinfection. With a proper treatment, a complete recovery with no sequelae is obtained.
The success rate of catheter recanalization of occluded coronary arteries is 50% to 80%. The reason for failure is frequently an occlusion which is to hard. As examples, two patients are reported with very hard coronary artery occlusions which were 5 months and 2 years old, respectively. There had been prior unsuccessful attempted treatment with other techniques. These occlusions could be recanalized with an 0.018 inch laser wire. Afterwards, a balloon dilatation could be carried out via the laser wire. The procedure was successful in both cases.
Cogan's syndrome consists of non syphilitic interstitial keratitis associated with vestibuloauditory dysfunction. This syndrome is rare and very few cases have been reported since Cogan's first characterization in 1945. We present the case of a 24-year-oldman with a history of recurrent access of arthralgia and glomerulonephritis. Bilateral corneal disease consists of peripheral whitish patchy opacities in deep stroma. The patient also shows bilateral severe deafness, fever, meningeal and tetrapyramidal syndrome. Ocular involvement responds well to corticosteroids therapy but hearing loss is severe and often irreversible.
Between August 1991 and December 1993, a total of 7011 percutaneous transluminal coronary angioplasties (PTCA) were performed. In 24 of them, the acute results were unsatisfactory, an autoperfusion balloon catheter was tolerated without complications and prolonged inflation (30 min to one hour) brought no improvement. In these 24 patients (22 men, two women; mean age 60 [33-81] years) the duration of inflation was, if possible, increased to at least 6 hours. In three patients the inflation had to be terminated after 2 or 4 hours, respectively, because of angina or for technical reasons. In 18 of the 24 patients subsequent angiography demonstrated residual restenosis of less than 50%, with a mean degree of stenosis of 18 +/- 14%. But lasting dilatation was not achieved in 6 patients: occlusion persisted in one and marked residual stenosis in two patients, while stent implantation was required in three. These results indicate that, in case of dissection or acute occlusion with failure after prolonged balloon inflation of 30 to 60 min, extending the duration of inflation to many hours can often produce good results so that stent implantation can be avoided.
The possibility of closing an atrial septal defect in adults by means of a percutaneously introduced catheter with an umbrella-type occluder was tested in seven consecutive patients with this defect (two men and five women, aged 35 to 69 years). The diameter of the defect (echocardiographic measurement) ranged from 7 to 25 mm, the left to right shunt from 11% to 54% of pulmonary flow and the pulmonary artery pressure from 24/8 to 110/25 mmHg. In one patient no attempt was made to close the defect because of its size (invasively measured: 40 mm). In another patient the method failed. Closure was successful in four of the patients (left to right shunt < 5%), while in a fifth patient a haemodynamically significant residual shunt (38%) remained, but was closed 2 months later with a second occluder. In one of the patients a portion of the device embolized to the pulmonary artery from which it was removed by catheter.--Thus the intervention was successful in five of six patients. This experience suggests that this type of occluder can also be used successfully in adults.
Retinal artery occlusion related to ocular compression is an uncommon but severe complication of general anaesthesia, most of the time results in blindness. We report a case of central retinal artery occlusion following spinal surgery. We discuss the mechanism of blindness and review the circumstances that may lead to this complication. Proper positioning of the head and avoidance of ocular compression will prevent the occurrence of retinal occlusion during general anaesthesia.
We present the results of ophthalmologic examinations of a consecutive series of 30 patients with HTLV-I associated myelopathy. This is the first ophthalmologic prospective study reported outside the Japanese endemic area. Twenty-one of the patients (70%) had kerato-conjunctivitis sicca in addition to accessory salivary gland lymphocytary infiltration in 7. Two cases of uveo-papillitis, 1 case of cotton-wool spots and 3 cases of retinochoroidal degeneration were also observed. Moreover, an aliquot of the aqueous humour was collected from the anterior chamber in 5 patients and showed high titer of HTLV-I antibodies for the 2 patients with uveo-papillitis. We propose different hypotheses to explain the physiopathologic characteristics of these features.
The human T-lymphotropic retrovirus type I (HTLV-1), isolated in 1980, has been shown to be responsible for two distinct systemic diseases: adult T-cell leukemia and HTLV associated myelopathy (HAM). Recently, an ever increasing number of publications have described other disorders associated with HAM. We report a case of uveitis-optic disc neuritis which, to our knowledge, had not been previously described. The fact that both uvea and optic disc were inflamed would suggest that immune-mediated reactions are involved in the pathogenesis of HAM.
A case of anterior lenticonus is reported in a 26 year old man with hemorrhagic nephritis related to familial Alport's syndrome. Anterior lenticonus is seen only as a part of Alport's syndrome. The authors describe its clinical, histologic and therapeutic aspects. They also discuss the other ocular manifestations, the nephritis and the sensorineural deafness.
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The haemodynamic effects of electrically initiated supraventricular tachycardias were studied in 10 otherwise healthy patients. With the beginning of the dysrhythmia there was a sharp decrease of systemic arterial and left ventricular systolic pressure, dP/dtmax, cardiac index, and stroke volume. Then, these parameters except stroke volume exhibited an increase, but were statistically different from control until the tachycardia was electrically stopped at the tenth minute. Mean pulmonary artery pressure rose steadily and remained on an elevated level which was reached between the third and fifth minute. After cessation there was a short augmentation of systemic arterial and left ventricular systolic pressure as well as of dP/dtmax and cardiac index. Mean pulmonary artery pressure steadily decreased and for all parameters control values were reached at about the third minute. When the tachycardia was terminated at about the same time by an i.v. injection of 10 mg of verapamil the 'post-tachycardial potentiation' was less marked or even absent. Mean arterial and left ventricular pressure was lower and mean pulmonary artery pressure remained elevated as compared with the values observed after electrical termination. Cardiac index and dP/dtmax showed no significant differences. It can be concluded that verapamil in a dosage sufficient to terminate the arrhythmia did not exhibit additional negative inotropic effects.
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