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Biomedical subjects

J Cabane

Publications and source records attributed to J Cabane.

At least 91 records · Page 5Linked to original sources

[Objective evaluation of sclerodermic skin using the slip-test. Results and validation with 30 patients and 60 controls].

The slip test is a non-invasive way to objectively evaluate the extent of scleroderma by measuring the "slipping" of the skin over an underlying bone. The results obtained in 30 scleroderma patients and 60 controls (178 tests due to multiple measurements) were analyzed to determine which test sites show a significant difference between patients and controls (Student's t-test) and which sites are the most discriminating for the diagnosis of the disease (multivariate analysis). Patient values were clearly distinct from those of controls. The best sites (defined by p < 0.05) were: the mandible, the second and first phalanges of the middle finger, the hands, the forearms, the epicondyles, the sternum, the iliac crests, the patellae, the tibias (the upper and lower fourths) and the feet; in all, 23 sites to which can be added the opening of the mouth (measurement of the maximal interlabial distance), which proved to be a reliable parameter. Multiple regression analysis yielded an equation that identified patients with scleroderma based on 4 variables (right forearm, right iliac crest, sternum and right rib). The slip test is thus the first reproducible quantitative test able to confirm and quantify clinical impressions in scleroderma. It is entirely able to objectively monitor the evolution of the disease under treatment. Nevertheless, complementary studies are needed to verify whether the slip test, which can be performed in 20 minutes during an office visit, can be used advantageously in the routine clinical evaluation of scleroderma.

Adult↗

[Malignant granuloma of the mid-face: value of x-ray computed tomography/MRI imaging].

CT an MR imaging are now an integral part of the work-up of every ulcerative lesion of the mid-face. The CT scan enables an exhaustive evaluation of the bone and cartilage necrosis by using bone densitometry settings. The mass lesions, the inflammatory areas and the thickened mucosa are best demonstrated using the tissue densitometry settings. Injection of iodinated contrast material makes clearer delineations of the abnormalities, but doesn't differentiate well between muco-purulent fluid retentions (in the sinuses for example) and solid neoplastic masses. MRI is the technic of choice for this goal; it also gives helpful images in the sagittal and frontal planes. CT and MR imagings are best combined to guide the surgical biopsies and the radiation therapy when managing the difficult problem of mid-face ulcerative lesions.

Facial Bones↗

Comparison of long term evolution of adult onset and juvenile onset Still's disease, both followed up for more than 10 years.

Still's disease is a clinical entity of unknown origin, which can appear before 15 years of age (juvenile onset Still's disease) or later (adult onset Still's disease). There are few reported data about the long term prognosis of Still's disease and no study compares the long term evolution of adult onset and juvenile onset Still's disease. Eighteen patients fulfilling the American Rheumatism Association criteria for Still's disease were followed up for more than 10 years. Ten (group 1) had juvenile onset Still's disease and eight (group 2) adult onset Still's disease. A comparison of the groups showed no significant differences in the initial systemic manifestations of Still's disease, or in the joint lesions. Both groups had severe sequelae, which appeared between six and 10 years after the initial flare up of Still's disease. Nine patients had articular damage and nine had only arthritis without apparent x ray abnormalities. Nine patients had bilateral hip destruction in less than four years. Of these nine, seven required 13 total hip replacements before the age of 45. In the whole group of 18 patients bilateral involvement of the following joints was also seen: carpus (seven patients), knee (four), tarsus (four), ankle (three); three patients had ankylosis of the cervical spine. The occurrence of amyloidosis (three cases, two deaths) was restricted to group 2. This was the only difference between the groups, as the treatments were identical. It is concluded that the articular prognosis of Still's disease is poor, be it adult onset or juvenile onset, with severe joint destruction in half of the patients.

Adult↗

[Neurologic involvement in malignant mid-face granuloma].

We report 4 cases with neurological disorders due to lethal centrofacial granulomas of unknown origin, and we review the relevant literature. This puzzling disease is characterized by a relentless ulceration of the nose progressing toward the base of the skull, and frequently involving the cranial nerves, the meninges and later the central nervous system. The main clinical point in such situations is to ascertain that no microorganism, no cancer, no specific disease is responsible for the centrofacial lesions, since the microscopic findings may be non-specific. When the entire work-up to exclude all differential diagnoses is completed, the clinician has to deal with lethal centrofacial granuloma. This seems to be a heterogeneous disease, in most of the cases close to malignant T lymphomas. Management is based on radiotherapy, chemotherapy and renutrition with treatment of frequent infectious complications. The prognosis is poor.

Adolescent↗

[Contribution of immunologic technics to the characterization of lethal midline granuloma of unknown origin].

The lethal midline granuloma is a clinical entity characterized by a relentless ulceration of the upper airway involving the nose, the palate and the face, without any demonstrable etiology. We have applied the cell membrane immunostaining techniques to twelve cases. According to the results, it seems that most of the cases are in fact T-cell lymphomas with membrane staining consistent with either precursor or mature lymphoid T-cells. Some cases, however, exhibit an immunostaining pattern compatible with other origins, the proliferating cells belonging either to the B lymphoid or to the histio-monocytic lineages. We conclude that the lethal midline granuloma is an heterogeneous group of neoplastic diseases, in the most part close to a T cell lymphoma, but with a remarkable clinical unity.

Granuloma, Lethal Midline↗

[Pulmonary toxoplasmosis in AIDS. Report of 3 cases].

Pulmonary toxoplasmosis in AIDS is rare. Its frequency is estimated to be between 0.2 and 3.7% but is probably underestimated; however, it is lower than that of neurotoxoplasmosis and is rarely identified prior to autopsy. We describe herein 3 cases diagnosed in living patients. The clinical presentation is usually severe interstitial pneumonitis, occurring in profoundly immunodeficient patients. Toxoplasma gondii, the infectious agent, must always be sought in the bronchoalveolar lavage of such patients and is sometimes associated with other opportunistic infectious agents, such as Pneumocystis carinii.

Acquired Immunodeficiency Syndrome↗

[Recurrence of atypical pulmonary pneumocystosis treated with pentamidine aerosol].

Two cases are reported of atypical relapses of pneumocystosis in AIDS patients treated with aerosol pentamidine for 14 and 22 months. These pneumopathies are unusual because of their pitted aspect and recurrent spontaneous pneumothoraxes in spite of repeated drainage. They are difficult to diagnose because bronchoalveolar lavage fluid is negative for Pneumocystis carinii, despite their presence in lung biopsies. Histological lesions vary, being granulomatous, necrotizing and invasive, with involvement of the pleura and lymph nodes. Although a highly effective therapy against P. carinii pneumonia, aerosol pentamidine may play a role in these atypical episodes: either by causing bronchial obstructions beyond which the pneumocytotic lesions cannot be reached by lavage and become necrotic, or by favoring the extrapulmonary spread of P. carinii.

Acquired Immunodeficiency Syndrome↗

[Wegener's granulomatosis in elderly subjects. 37 cases].

The clinical, biochemical and prognostic characteristics of 37 patients with Wegener's granulomatosis were studied in relation to age. Eleven patients were older than 60 years when the first sign of the disease appeared. Except for the fact that the renal lesions seemed to be more severe in these patients, there was no significant clinical or biochemical difference between them and patients under 60. Five patients over 60 years of age died during the first 2 years of Wegener's granulomatosis. Death was due to infection in 3 cases, to acute renal failure as complication of intra-alveolar haemorrhage in 1 case and to metastatic cancer in 1 case. Age-related comparisons of survival rates showed that the probability of surviving was lower in elderly subjects: 78 per cent at 1 year and 39 per cent at 3 and 5 years, as opposed to 96 per cent at 1 and 3 years and 84 per cent at 5 years in younger subjects (P less than 0.01). This poor prognosis is probably due to the renal lesions which constitute an important cause of death in the published series. Rapidly progressive glomerulonephritis being amenable to an early and intensive treatment, elderly patients should be treated as energetically as younger patients.

Adrenal Cortex Hormones↗

[Wegener's granulomatosis. Clinical aspects, nosologic problems. Review of the literature apropos of 30 cases].

Thirty cases of Wegener's Granulomatosis are reported, 16 men and 14 women aged 49.3 +/- 14 years at the onset of their illness. The presenting symptoms were oto-rhino-laryngeal in 22 cases and pulmonary in 16 cases. Renal disease was initial in only 1 case. The complete picture comprised oto-rhino-laryngeal (29 cases), pulmonary (26 cases), renal (27 cases), articular (16 cases), muco-cutaneous (19 cases), ocular (15 cases), neurological (13 cases), and muscular (10 cases) involvement. An increased sedimentation rate, leukocytosis, anaemia and thrombocytosis were common findings. The treatment comprised steroid therapy associated with immunosuppressor drugs (28 cases) and plasma exchanges (8 cases). External radiotherapy was used in 5 patients, including 3 with bronchopulmonary lesions, but was not effective. The probability of survival after the initial symptoms was 84 p. 100 at 1 year, 69 p. 100 at 3 years and 59 p. 100 at 5 and 10 years. Eight patients died; infection and renal failure were the principal causes. Late relapse occurred in 3 patients justifying long-term follow-up and the necessity of using the term "cure" with reserve. The clinical and therapeutic aspects are described in the light of the authors' experience and a review of the literature. The concept of localised form of the disease and bordeline forms with atrophic polychondritis, periarteritis nodosa, Churg and Strauss syndrome and lymphomatous granulomatosis are also discussed.

Adult↗