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Biomedical subjects

M F Kahn

Publications and source records attributed to M F Kahn.

At least 235 records · Page 13Linked to original sources
PubMed1986

[Treatment of systemic scleroderma with D-penicillamine. Results in 14 patients treated for more than a year].

The authors review the theoretical bases for the treatment of systemic scleroderma (SS) by D-penicillamine (DP). They then present a review of the papers published over the last 20 years on the subject. Although Rodnan's team has conducted studies against a control series and the majority of published works have concluded on the effectiveness of this treatment, there has not been any randomised prospective study, to date, to clearly demonstrate this effectiveness. The authors report a series of 14 patients with SS treated for a mean of 2 years with 900 mg/day of DP. DP seemed to be effective on the cutaneous lesions in 12 of the patients treated at a maximum of 3 years after the frank onset of the disease. Treatment failed in 2 patients who were only treated late in the disease. Objective results were obtained on the pulmonary lesion, but they are more difficult to interpret. 5 patients had to interrupt DP treatment because of intolerance. Tiopronine was tried in 4 of these patients with 3 interpretables follow-ups. This agent was well tolerated and effective in 2 of these 3 patients.

Adult↗
PubMed1986

[Severe pulmonary fibrosis disclosing primary Gougerot-Sjögren syndrome. Apropos of 5 cases, 2 of which were fatal].

Five cases (four women and one man) with primary Gougerot-Sjögren syndromes presenting as interstitial pulmonary fibrosis are reported. The main point of interest was the severity of the pulmonary changes: effort dyspnoea from the outset, radiological interstitial pneumonitis, restrictive syndrome with a CO diffusing capacity less than 50 p. 100 of theoretical values in 4 cases. Broncho-alveolar lavage was performed in all patients: it showed a high polynuclear neutrophil and particularly high eosinophil counts. Lung biopsy showed severe fibrotic changes. The pulmonary changes dominated the clinical presentation in all patients and the Gougerot-Sjögren syndrome was only diagnosed after systematic examination completed by biopsy of the accessory salivary glands. In the absence of an associated connective tissue disease, these cases of the Gougerot-Sjögren syndrome were considered to be primary. Four patients were given steroid therapy but this did not prevent a fatal outcome in two cases after 6 and 14 months respectively. The clinical states of the other patients were unaffected by treatment. Twenty-one other, but less severe cases, have been previously reported. They confirm that interstitial pulmonary fibrosis observed during the Gougerot-Sjögren syndrome does not differ from idiopathic pulmonary fibrosis and that observed in other connective tissue diseases. Nevertheless, analysis of our cases showed that biopsy of the accessory salivary glands should be part of the routine investigation of apparently idiopathic pulmonary fibrosis. These pulmonary changes may be life-threatening when associated with a primary Gougerot-Sjögren syndrome.

Adult↗
PubMed1986

[Amyloidosis in adult Still's disease. Apropos of 2 cases].

Two cases of amyloidosis were observed in a personal series of 42 patients with the adult from of Still's disease. Two other cases have been previously reported in the literature. In the first case, amyloidosis occurred 18 months after the onset of the disease and involved the kidneys and gastrointestinal tract with a fatal outcome in 6 months. In the second case, renal amyloidosis was observed 4 years after the onset of Still's disease and progressed to renal failure in 7 months, necessitating chronic haemodialysis. These cases show that the adult form of Still's disease may rarely be complicated by amyloidosis, the prognosis of which is particularly poor.

Adult↗
PubMed1985

[Anatomic and nosologic analysis of 100 cases of diffuse angiitis seen in rheumatology].

Diffuse inflammatory angiitis is seen in extremely varied aetiological and nosological situations. In some cases, the vascular disease represents the only sign of systemic disease. In other cases, it mays be associated with or complicated by diffuse systemic inflammatory conditions. This study analysed 100 cases of inflammatory angiitis with systemic manifestations seen over a period of 7 years in a rheumatology department. Patients with scleroderma, erythema nodosa, Behçet's syndrome and angiitis associated with microbial infectious diseases were excluded from this study. 24 cases in which the angiitis was clinically certain, but in which the vascular lesion was not confirmed histologically were also excluded. The type of histological lesion and the nosological distribution of these 100 cases are described in detail. The results of this analysis confirm the very great variety of the clinical and histological presentations. A number of rare syndromes were observed. Classical periarteritis nodosa only represented 14 per cent of the whole series and only 3 of the 14 cases had a demonstrable aetiology (2 HBs, 1: desensitisation to Candidin). The authors stress the histological diversity of inflammatory angiitis associated with rheumatoid arthritis and systemic lupus erythematosus. All sizes of vessels may be involved and all types of lesions can be observed in these two diseases. The authors also emphasise the lesser known clinical and histological features (stenosing endarteritis of the great vessels in systemic lupus erythematosus) and previously unreported cases (valvular lesions in 2 cases of Mac Duffie's syndrome, progression of 2 cases of delayed urticaria to pressure towards a clinical lupus with unusual laboratory findings).(ABSTRACT TRUNCATED AT 250 WORDS)

Arthritis, Rheumatoid↗
PubMed1985

[Information of the rheumatic patient in the 19th century].

From the beginning of the 19th century, universities considered it necessary to publish books on gout and rheumatic diseases which today we would describe as constituting public information. These books were intended for young doctors as well as the general public and patients. Rheumatology is probably the first specialty in which such an approach was considered to be useful. The current efforts in the area of public information therefore constitute a historical continuation of this approach.

Books↗
PubMed1985

[Controlled prospective study of clinical and biological thyroid parameters in rheumatoid polyarthritis].

15 patients with rheumatoid arthritis (14 women and 1 man aged between 30 and 75 years) were compared to a control group of 12 women and 1 man (aged between 22 and 77 years) admitted to the rheumatology unit at the same time for benign diseases. Both groups were examined for: the presence of a goitre, family history and/or clinical hormonal dysfunction, the plasma levels of total cholesterol, free thyroxin (FT4), free triiodothyronine (FT3), before and 2 hours after 50 micrograms of thyrotropin IV, thyrostimulin half an hour before and one hour after thyrotropin and the levels of the antimicrosomal anti-thyroid antibodies and the anti-thyroglobulin antibodies. None of the patients with rheumatoid arthritis had any clinical hormonal dysfunction. However, 6 of the 15 patients presented a homogeneous goitre, 5 of these 6 patients had a family history of goitre and 2 had positive antibodies. In comparison with the control group, the 15 cases of rheumatoid arthritis had a significant decrease (m +/- sd) in the FT4 (13.20 +/- 2.50 pmol/l vs 15.60 +/- 2.47; p less than 0.02), FT3 (3.80 +/- 1.12 pmol/l vs 5.50 +/- 0.93; p less than 0.001) and a rise in the T3 with low thyrotropin (5.70 +/- 1.60 vs 8.50 +/- 1.50; p less than 0.001), while the levels of thyrostimulin and the thyrostimulin peak under thyrotropin were not modified.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1984

[Multiple hyperostosis with unilateral sacroiliitis. A new spondyloarthropathy].

Six patients (4 male and 2 female) presenting with an original, predominantly osseous disease are reported. The disease is characterized by a chronic inflammatory process affecting the thorax, the spine, the femur in 1 case and in all patients only one of the sacro-iliac joints. The condition had begun 11 to 28 years previously and had been active for 6 to 24 years. One case started with regressive polyarthritis. There was no visceral involvement. Radiograms showed bone condensation with hyperostosis, sometimes hypertrophic, unrelated to any articular space. Depending on each patient, the lesions involved the clavicles, the sternum, the posterior arch of the ribs, the vertebrae, the inferior femoral metaphysis. The only joints involved were the sacro-iliac and anubrio-sternal joints. The areas affected showed high radioisotope uptake. There was discrete biochemical inflammatory syndrome. Three of the 6 patients belonged to the HLA group B 27. The multiple biopsies performed showed no specific lesions. The disease proceeds by successive attacks lasting several months and responding poorly to anti-inflammatory drugs. One patient developed Crohn's disease, another, retroperitoneal fibrosis. In some respects, this condition is remindful of sterno-clavicular hyperostosis and of the osteo-arthropathy in palmoplantar pustulosis or acne conglobata. It seems to constitute a new variety of spondylo-arthropathy intermediate between the above-mentioned diseases and genuine ankylosing spondylitis.

Adolescent↗
PubMed1984

Detection of antilymphocyte antibodies in patients with scleroderma using three different techniques.

The occurrence of antilymphocyte antibodies (AL-Ab) was investigated in the sera of 28 patients with scleroderma. By indirect immunofluorescence, we found these Ab in 53% of the sera. Inhibition of E rosette formation and lymphocytotoxicity revealed these Ab in 42% and 14% of the sera respectively. Most of the Ab (93%) reacted at 0 degrees C. These AL-Ab can be separated into several groups depending on their inhibitory activity on the lymphocyte membrane. In some cases, the receptor seems similar to that of sheep red blood cells. The study of the clinical features of the patients showed few differences between the groups with and those without Al-Ab. It must be noted that all the patients with the CREST syndrome (6 cases) possessed Al-Ab.

Antilymphocyte Serum↗
PubMed1984

[Spinal cord compression in malignant plasmacytic diseases. Apropos of 6 cases].

Myeloma may be complicated or revealed by spinal cord compression. Out of 105 cases of myeloma admitted to this Department, 6 cases of spinal cord compression were observed, with a favourable outcome after treatment by laminectomy combined with radiotherapy. In 5 cases out of 6, spinal cord compression was either the presenting sign or occurred within the first months after diagnosis. Compression occurred in the thoracic cord in 5 cases, and in the lumbar cord in 1 case. The interval between the first symptom and diagnosis varied greatly (from a few hours to 1 year), as did the degree of paraplegia, which ranged from paraparesis to flaccid paraplegia. A favourable outcome occurs in most other reported cases, in contrast with spinal cord compression from metastases. Treatment (laminectomy-radiotherapy or both) remains controversial.

Adult↗
PubMed1983

[Diagnosis of bone metastases. Value of the guided puncture and the posterior iliac crest systematic puncture-biopsy].

The results of bone puncture (P) or aspiration biopsy (AB) in a series of 82 patients with skeletal metastases are reported. In 71 patients, AB of the posterior iliac crest was performed systematically, irrespective of the site of the bone lesion and without aiming at a suspicious area. In 11 patients, P or AB were guided by televised fluoroscopy towards an image of bone metastasis. With either method, a diagnosis of metastasis was made in 42/82 patients, i.e. 50%. With the first method, positive results were obtained in 43.7% of the cases; the figure rose to 54% in cases with known primary cancer and abnormal X-ray image and dropped to 36% in cases with abnormal image and no history of cancer, and to 9 p. 100 in cases with normal X-ray films. In all 11 patients had fluoroscopy-guided P or AB, histological examination of the specimens was positive for metastasis.

Biopsy, Needle↗
PubMed1983

[Antinuclear, anti-DNA and anti-lymphocyte antibodies in systemic scleroderma. 62 cases].

The authors describe a prospective study of serum immunological abnormalities in 62 cases of systemic sclerodermia. Antinuclear antibodies were found in 67 percent of the cases. Contrary to expectation, the homogenous type was the most common. Anticentromeric antibodies were found in 4 of the 10 CREST syndrome patients. Anti-RNP (2/62) and natural anti-DNA antibodies are rarely found in sclerodermia. Non-cytotoxic anti-lymphocytic antibodies were found in more than half of the serums studied. They were frequently found in the CREST cases and in cases associated with Gougerot-Sjögren syndrome. Their significance is unknown. Lastly, almost all of the cases of systemic sclerodermia showed an immunological serum abnormality.

Antibodies, Antinuclear↗