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Biomedical subjects

M F Kahn

Publications and source records attributed to M F Kahn.

At least 271 records · Page 15Linked to original sources

[Subacute and chronic joint involvement occurring during the course of familial Mediterranean fever (author's transl)].

Subacute and chronic joint involvement occur in less than 5 p. cent of patients with familial mediterranean fever, whereas acute joint lesions are observed in approximately 3 out of 4 cases. The various aspects of these atypical forms are discussed in relation to 10 such cases. In most of these patients the lesions were merely due to the prolongation of the acute process, presenting as a subacute mono-arthrites lasting for several months before regression. X Rays show only regional demineralisation, sometimes predominating in the subchondral zone. Symptoms totally regress without sequelae. A time destructive arthropathy may however develop, especially in the hip. The presentation associates limitation of joint motion with, on X Rays, joint space narrowing and osteophytes. In 2 of our cases and one case reported in the literature, there was a progressive development of a chronic caxopathy without any previous acute involvement of this joint. At last, in some cases, familial mediterranean fever may be associated with a known arthritidis, usually ankylosing spondylitis.

Adolescent↗

[Vitamin-resistant rachitism related to the X chromosome in adults. A report on three cases, one of whom had associated articular chondrocalcinosis (author's transl)].

Three cases of X-linked vitamin-resistant rachitism and discovered in adult life are reported. In all 3 cases there were morphological anomalies affecting mainly the limbs which were incurved and reduced stature. In two of the patients the disorder was progressive and presented as pain, walking difficulties, and Looser-Milkman striae. In the third case the hypophosphatemia was asympomatic, but it enabled the early diagnosis of a progressive rachitic disorder in the patient's child; treatment with 1-25 dihydroxycholecalciferol alone caused renewed growth, disappearance of radiological anomalies, and regression of biological disorders. Articular chondrocalcinosis was also present in one of the patients. This could be a coincidence and systematic search for this condition in cases of vitamin-resistant rachitism will be necessary to establish a possible relationship between the two affections.

Adult↗

[Acute leukaemias after treatment using cytotoxic agents for rheumatological purpose. 19 cases among 2006 patients(author's transl)].

The authors undertook a retrospective study to determine the number of acute leukaemias developing amongst 2006 patients suffering from chronic inflammatory rheumatic conditions and connective tissue disorders, treated with cytotoxic agents. The follow-up period ranged from 1 to 13 years. Nineteen leukaemias were found, essentially granulocytic, with a latent period of 5.7 +/- 2, 8 years after the beginning of treatment. This incidence of almost 1% of leukaemias is probably less than the actual percentage since a number of patients were lost on follow up and since the period of observation is as yet too short. The majority of patients has been treated for more than one year. No cases were seen amongst patients treated for less than six months, or with less than 1g of chlorambucil or 50 g of cyclophosphamide. The risk would seem to be the same for both alkylating agents. No patients treated with azathioprine developed leukaemia, but few patients received this drug. Amongst 35 patients treated for severe psoriatic arthropathy with chlorambucil, 4 developed leukaemia. This particularly high percentage is such that all trials of alkylating agent in this condition should be stopped. The prevalence of leukaemia seen in the series as a whole is comparable to that found in mass studies carried out in various malignant diseases treated by cytotoxics. Awareness of this risk should, lead to even stricter limitations before the use of cytotoxic drugs in rheumatological conditions.

Acute Disease↗

[The clinical significance of soluble nuclear antigen specific antibodies (author's transl)].

Anti-ENA antibodies have been found in 176 sera which nearly all contained antinuclear antibodies giving a speckled pattern of nuclear fluorescence. The charts of 134 of these 176 patients were available for a thorough clinical study. Among these 134 patients, 59 had a well defined Connective Tissue Disease including 40 SLE, 31 had a limited clinical syndrome made of Raynaud's phenomenon, inflammatory polyarthritis, swollen fingers and hyperglobulinemia and 34 had a complex clinical picture associating signs of more than one connective tissue disease. Some of the patients in this third group could be considered as-having the Mixed Connective Tissue Disease (MCTD) described by Sharp et al. Anti-RNP antibodies were more common in this series than the other anti-ENA antibodies. However, no narrow specificity could be assigned to any of these antibodies. This is true of the non anti-RNP antibodies, the anti-Sm in particular, which were found in 49 patients of whom 32 had SLE existing alone or in association with features of other connective tissue diseases and 17 had another connective tissue disease or the afore-mentioned limited clinical syndrome. In any case, the anti-ECT antibodies never reach the diagnostic value of the anti-DNA antibodies.

Adolescent↗

[The rheumatism of acne conglobata (author's transl)].

The authors report a case of painful joint, para-articular and muscular involvement accompanying acne conglobata. Fifteen other cases of rheumatism associated with this very special type of acne have been reported. The patients affected are young men suffering from acne conglobata (severe form of acne characterised by its ulcerating course and the possibility of its being accompanied by systemic manifestations), who suddenly develop fever, an altered general state and assymetrical arthralgia involving mainly the large joints, accompanied by signs of muscular involvement. The course is one of spontaneous recovery, relapses being possible. This falls within the context of the group of rheumatic disorders associated with the signs of an inflammatory skin disease.

Acne Vulgaris↗

Clinical significance of antibodies to soluble extractable nuclear antigens (anti-ENA).

Clinical and biological manifestations have been studied in 134 patients whose serum had antibodies to soluble extractable nuclear antigens (ENA). 85 of the patients had anti-RNP antibodies, 18 had anti-Sm antibodies, and 31 had antibodies to one or more soluble nuclear antigen. In all groups, the predominant clinical manifestations were polyarthritis, Raynaud's phenomenon, fever, and skin involvement. Renal disease was less common in those patients with anti-RNP antibodies than in the other patients. Most patients with definite renal disease (13 out of 15) also had circulating anti-DNA antibodies. The final diagnoses in these 134 patients were well defined connective tissue disease in 59; overlap syndromes in 34; a limited clinical syndrome made up of polyarthritis Raynaud's phenomenon--often with swollen fingers--and/or hypergammaglobulin-aemia in 31, and various other clinical conditions in 10.

Adolescent↗

[Juvenile arthritis reevaluated in rheumatology in adult age. 30 cases].

The authors describe and comment on 30 observations of peripheral polyarthritis starting before puberty and reveluated in an adult rheumatologoy service at the average age of 29 and during an observation period of about 16 years. The actual classification of juvenile polyarthritis proves to be valid in this group since the clinical forms are still observed in adulthood. However, during the evolution, there is a shifting from one form to another. The systemic forms can reoccur in adulthood even after several years of quiescence, accompanied by visceral complications. The pauciarticular forms as a rule remain benign. As in childhood, they frequently show antinuclear factors. Some forms, initially peripheral, can show axial signs. The presence of HLA B27 antigens allow them to be linked up with ankylosing spondylarthritis. But this is not the case for all the forms with sacroiliac functions. The evolution since childhood is often characterized by long remissions which can still persist by the time of the visit, which is then motivated by resulting mechanical and local arthrotic problems. Despite often severe anatomical symptoms, the functional handicap is limited, allowing quite a remarkable adaptation. Most of the patients lead an active professional life. Yet despite this and despite an often satisfactory sex life, half of the patients have a psychological handicap which makes it necessary for them to be placed under special carl. No therapeutic conclusion, particularly about the efficiency of the basic therapy, can be drawn from this study.

Adult↗