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Biomedical subjects

C Toledano

Publications and source records attributed to C Toledano.

At least 19 recordsLinked to original sources

Impact of pain in health related quality of life of patients with systemic sclerosis.

OBJECTIVES: Systemic sclerosis (SSc) has an heterogenous clinical pattern, with variable organ involvement and degrees of severity. Like in other rheumatic diseases, the self-questionnaires have been used to evaluate SSc globally. The aim of the study is as to evaluate the quality of life (QoL) in patients with either diffuse or limited SSc, and to examine the impact of pain on the QoL scores. METHODS: Patients with SSc, either diffuse or limited SSc, were included in a cross-sectional study. The QoL was evaluated with the short-form 36 (SF-36) and the functional repercussion with the SSc-modified Health Assessment Questionnaire (S-HAQ). RESULTS: A total of 89 patients (67 with diffuse and 22 with limited SSc) were included. The SF-36 score values were lower in SSc patients than those reported in the general population. The physical component scores (PCS) of the SF-36 was significantly worse in diffuse compared with limited SSc (P < 0.05). The PCS was significantly negatively related to the number of clinical manifestations (ANOVA, P < 0.0001). The mental component score (MCS) was not influenced by the type of SSc or the number of clinical manifestations presented by the patient. The QoL of SSc patients was highly correlated with pain (R = 0.69) and disability (R = 0.70). Interestingly, the QoL of SSc patients was only slightly correlated with cutaneous (R = 0.42) and pulmonary involvement (R = 0.57). CONCLUSION: The QoL of patients with SSc is strongly influenced by the type of SSc, the burden of clinical manifestations, the functional disability and by the pain, whatever its cause. The treatment of pain should be considered as priority to improve the QoL of SSc patients.

Adolescent↗

[Cardiopulmonary function before and after cyclophosphamide treatment in severe systemic sclerosis: comparison of monthly intravenous bolus and autologous haematopoietic stem cell transplantation].

PURPOSE: Cyclophosphamide in monthly intravenous bolus is used to treat severe forms of systemic sclerosis with pulmonary involvement. Since 1996, cyclophosphamide therapeutic intensification with autologous haematopoietic stem cells transplantation allowed significant improvement in skin and functional scores in severe systemic sclerosis. Cyclophosphamide potential cardiotoxicity in this setting has been questioned. METHODS: To analyse cyclophosphamide potential cardiopulmonary toxicity (as graded with WHO classification), we retrospectively studied all severe systemic sclerosis patients treated with cyclophosphamide either during autologous haematopoietic stem cells transplantation procedure (group A) or intravenous cyclophosphamide (group B) recruited in 7 French centers volunteers for the study. Parameters to evaluate heart and lung functions at inclusion, then at last follow-up between 6 and 12 months after start of treatment, were compared using the Mann-Whitney test. RESULTS: (Mean+/-standard deviation): Groups A (N=14) and B (N=13) were similar at the beginning of the study in terms of skin, renal, heart and lung involvement. Cyclophosphamide total dose (/m(2)) received in group A was superior (P=0.02) to the one in group B. After respective follow-up of 10+/-2.8 (group A) and 9.9+/-2.7 (group B) months, cyclophosphamide cardio toxicity (group A: N=3; group B: N=2), evolution of the left ventricular ejection fraction and arterial and pulmonary pressures did not differ in the two groups. CONCLUSION: In spite of higher cyclophosphamide doses during autologous haematopoietic stem cells transplantation than bolus treatment, cardiopulmonary toxicity appeared not increased. The ongoing European ASTIS trial will compare the respective benefits of these 2 cyclophosphamide regimens in severe Systemic sclerosis.

Adolescent↗

[Therapeutic intensification and autologous stem cell transplantation in autoimmune diseases].

THE PATHOPHYSIOLOGY of most autoimmune diseases is often poorly understood. EXPERIMENTAL CONSIDERATIONS and clinical experience suggest that high doses immunoablation followed by stem cell transplantation is a therapeutic option to consider for certain severe autoimmune disorders. THE CONCEPT OF RESTORING NORMAL IMMUNE REACTIVITY must in part br true since current results of 466 transplants (445 autologous, 21 allogeneic) patients suffering from various autoimmune diseases show a beneficial outcome in approximately 2/3 of the patients. TO IMPROVE THE EFFICACY AND SAFETY OF SUCH AN AGGRESSIVE PROCEDURE in patients with potentially affected vital organs by the underlying autoimmune disease, it is especially important to follow international consensus guidelines and to centrally collect clinical data for in depth analysis in the EBMT International Stem Cell Project for Autoimmune Disease in Basel, Switzerland. PHASE III STUDIES ARE RUNNING FOR SYSTEMIC SCLEROSIS (Astis, Autologous Stem cell Transplantation International Rheumatoid Arthritis Trial) started in 2003. A STUDY PROJECT IS PLANNED FOR MULTIPLE SCLEROSIS (Astims, Autologous Stem cell Transplantation International Multiple Sclerosis).

Adolescent↗

Validation of French version of the Scleroderma Health Assessment Questionnaire (SSc HAQ).

The modified Scleroderma Health Assessment Questionnaire (SSc HAQ) is a functional score to assess systemic sclerosis (SSc) comprising the HAQ disease index (HAQ-DI) plus five specific visual analogue scales (VAS). Since it was validated in English-speaking patients only, its general use in any other language necessitates prior cross-cultural adaptation and validation. We designed this study to assess its value in French-speaking patients and to validate the French version according to international recommendations. We elaborated a French version using the "forward-backward" method. We then validated its psychometric properties with 100 consecutive SSc French-speaking patients who had undergone simultaneous clinical and paraclinical examination. In addition, we calculated the SSc HAQ score, a new outcome measure, which is obtained by pooling the eight domains from the HAQ-DI with the five organ VAS. Our study confirmed the psychometric properties of the SSc HAQ in non-English-speaking patients with (a) structural validity: the major component analysis, performed on the HAQ-DI and the five VAS, yielding a two-factor structure; (b) convergent validity: with high correlation coefficients between the SSc HAQ score and the physical component score of the SF-36 (r=-0.74, p<0.0001); (c) discriminant validity: the SSc HAQ score was better in patients with limited than with diffuse SSc (0.5+/-0.5 vs 1.1+/-0.7, respectively, p<0.0001) in relation to the number of clinical involvements; (d) reproducibility was high using the test-retest procedure (r=0.98). This study showed the value of the SSc HAQ, which is a simple, discriminant, reproducible self-administered questionnaire to evaluate French-speaking SSc patients. In addition, we suggest the use of a new outcome measure, the SSc HAQ score, to assess this systemic disease more accurately.

Disability Evaluation↗

[Quality of life assessment with the MOS-SF36 in patients with systemic sclerosis].

OBJECTIVE: To investigate the metric properties and the validity of the Medical Outcome Study Short Form 36 (SF-36), a questionnaire to assess the quality of life, in patients with either diffuse or limited systemic sclerosis (SS), and to examine the effect of the disease on quality of life. METHODS: Cross sectional study of 86 patients with a SS (64 diffuse SS, 22 limited SS). Disease severity was assessed by clinical examination, pulmonary functional tests and Health Assessment Questionnaire (HAQ) modified for scleroderma. RESULTS: The SF-36 scores values were lower in diffuse than in limited sclérodermie systémique. The Physical Component Score was worse in patients with than without any clinical involvement. This score increased in relation with the number of clinical involvements. The quality of life of patients with SS was correlated to its functional repercussion. CONCLUSION: The quality of life in SS patients is correlated with the clinical severity of the disease. The use of SF-36 to measure the quality of life is useful for the clinical evaluation of patients with SS.

Adolescent↗

Inherited palmoplantar keratoderma and sensorineural deafness associated with A7445G point mutation in the mitochondrial genome.

We report a French pedigree with members having an inherited combination of non-epidermolytic palmoplantar keratoderma (NEPPK) and sensorineural deafness. The penetrance of both features was incomplete. Additional ectodermal defects were absent. The expression of numerous epidermal proteins (keratins, fillagrin, cornified envelope proteins, intercellular junction proteins including connexin 26, and loricrin) defined with immunolabelling was normal in the proband. The combination was shown to be associated with the A7445G point mutation in the mitochondrial genome (mtDNA). This mutation is responsible for a subtype of NEPPK which is so far the only mtDNA mutation-associated keratoderma.

Adolescent↗

[Granuloma faciale: efficacy of cryosurgery in 2 cases].

BACKGROUND: Granuloma faciale is a rare condition of unknown pathogenesis. Treatment often gives less than satisfactory results. We report two cases successfully treated with cryosurgery. CASE REPORT: A 42-year-old woman had a round 65 x 40 mm erythematous violet-colored papulous plaque on the right cheek. Histology reported a dense inflammatory infiltration of the superficial and mid derma around dilated vessels under a normal epidermis. The infiltration was composed of histiocytes, lymphocytes, plasma cells, neutrophils and eosinophils and was compatible with granuloma facial. Surgical treatment was performed but was followed by recurrence within a few months. Class I local steroids, cotton swab cryotherapy and oral dapsone remained ineffective. Cryosurgery however led to cure without recurrence at 1 year follow-up. The second patient was a 52-year old man with an oval reddish-brown papulous plaque on the right preauricular area. The plaque measured 40 mm in diameter and histology favored granuloma facial. Three cotton swab cryotherapy sessions and one surgical excision procedure were followed by recurrence. Cryosurgery provided successful cure without recurrence at 3.5 years follow-up. DISCUSSION: Most destructive treatments for granuloma facial, including pulverization cryotherapy, surgery, and CO(2) or argon laser are unsuccessful in preventing recurrence. Cryosurgery was proposed in 1977 but few cases have been reported since. Medical treatments have been variously successful with most authors reporting unsatisfactory results. In our two cases, surgical excision was rapidly followed by recurrence and in one case dapsone was ineffective. Cryosurgery provided rapid regression of the lesion and recurrence-free cure at 1 and 3.5 years follow-up respectively. Cryosurgery is an effective treatment for readily recurrent eosinophilic granuloma facial and should be proposed as first intention therapy.

Adult↗

Ion-pair extraction of histamine from biological fluids and tissues for its determination by high-performance liquid chromatography with fluorescence detection.

A procedure for the selective ion-pair extraction of histamine from various biological fluids and tissues by using the ion-pairing agent bis(2-ethylhexyl)hydrogen-phosphoric acid has been developed. This procedure enabled routine determination of histamine in plasma, urine and tissue samples of humans and rats by reversed-phase high-performance liquid chromatography with fluorescence detection using o-phthaldialdehyde.

Animals↗