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IntroductionPatients with systemic sclerosis (SSc) have an increased risk of malignancy. In this study, we aimed to analyze the prevalence of cancer, the risk factors and the impact on overall survival.Patients and methodsWe analyzed clinical (history of cancer, toxic exposition, organ involvement), immunological and treatment data in a monocentric cohort of SSc patients followed between January 2004 and December 2017.ResultsTwo hundred and ten patients with SSc were included. During the follow-up, twenty-one patients (10 %) were diagnosed with malignancies. The underlying malignancies were breast adenocarcinoma (n = 6, 28%), lung cancer (n = 6, 28%), colorectal (colic adenocarcinoma, carcinoid tumor of the appendix), ovarian and cervix uteri, melanoma, kidney and papillary thyroid carcinoma (one of each). The median time between the first visit and the diagnosis of cancer was 4 [2–10] years. The overall survival in SSc patients with cancer was not significantly different from patients without cancer, with median survival during the first quartile (75%) at 12 years for patients with cancer and 11.6 years for those without cancer (P = 0.9). The history of renal scleroderma crisis (HR 10.99, IC95% [1.95–62.07]; P = 0.006) and the presence of anti-topoisomerase I antibodies (HR 5.5, IC95% [1.40–21.67]; P = 0.01) were associated with an increased risk of cancer, whereas the presence of gastroesophageal reflux was inversely associated with the cancer occurrence (HR 0.22, IC95% [0.056–0.867]; P = 0.03).ConclusionThe history of renal scleroderma crisis and the positivity of anti-topoisomerase I antibodies were associated with an increased risk of cancer in SSc patients in this monocentric study.  相似文献   

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Aims

To evaluate the prevalence of reflux oesophagitis (RE) in systemic sclerosis (SS), define its severity and analyze the risk factors.

Patients and methods

The study involved 173 consecutive cases of SS recruited prospectively over a period of 18 years (between April 1988 and June 2006, on 1980 ACR criteria). The mean age is 40.6 ± 13.4 years, the sex-ratio 0.14 and the average duration 6.7 ± 7.6 years. According to LeRoy et al. classification, 141 cases involved a located cutaneous form (81.5%) and 32 cases a diffuse form (18.5%). All the patients underwent a questionnaire concerning the presence and gradation of symptoms of reflux and/or dysphagia, an endoscopy and a standard oesophageal manometry. Twenty-four hour pH Monitoring was carried out on 89 patients.

Results

RE was found in 62 patients (35.8%), being mild or moderate (A and B LA) in 41 cases (23.7%) and severe (C and D) or complicated in 21 cases (12.1%). Presence of RE is not influenced by age, sex, duration or cutaneous extension of the disease. On the other hand, presence of oesophageal symptoms is a good indicator of the presence of mucous lesions. A pathological acid GER is almost constant in cases of RE in both upright and supine positions. GER index is higher in presence of RE than in its absence. The presence of the latter is statistically dependent on both lower oesophageal sphincter (LES) incompetence and oesophageal motor disorders. Moreover, the LES pressure and the distal oesophageal contractile amplitude wave are significantly lower in presence of RE than in its absence.

Conclusions

Reflux oesophagitis is observed in over 30% of scleroderma. Its presence is not related to the age, sex, duration or extension of the disease. It is especially linked to the presence of GER and severe oesophageal motor disorders.  相似文献   

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Introduction

Retinopathy is a chronic complication with severe functional consequences in patients with sickle cell disease. Its prevalence is not well known in sub-Saharan Africa because of the absence of screening. We report here the results of a routine screening for sickle retinopathy in a Comprehensive Sickle Cell Center in Sub-Saharan Africa.

Methods

Screening of sickle retinopathy was carried out in all sickle cell patients aged 10 and over, followed between 2010 and 2012. Retinopathy was screened by dilated indirect fundoscopic examination and retinal angiography, if necessary. The gender, age and hematological parameters of patients with sickle retinopathy were compared with those of controls randomly selected from the cohort of sickle cell patients without retinopathy followed during the same period.

Results

The overall prevalence of sickle cell retinopathy was 8.8% (142/1604): 12.4% (91/731) in SC, 5.2% (38/734) in SS, 9.4% (5/53) in Sβ°-thalassemia patients and 9.3% (8/86) in Sβ+-thalassemia patients. Proliferative retinopathy was more common in SC patients (P < 0.01). High levels of hemoglobin or of hematocrit were associated with retinopathy in all patients and with proliferative retinopathy in SC patients. In SS or Sβ0thalassemia patients, high leukocyte count was associated with proliferative retinopathy. Low fetal hemoglobin level was associated with retinopathy in all groups.

Conclusion

The prevalence of sickle cell retinopathy is high and negatively associated to the level of fetal hemoglobin. The efficiency of a routine screening for sickle cell retinopathy must be assessed in Africa as well as the benefit of phlebotomy and hydroxyurea therapy as a preventive treatments.  相似文献   

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Aim of the studyThe purpose of our study was to review the population at risk of upper limb arterial injury, to determinate the rate of upper limb salvage and the predictive factors of limb loss.MethodsThis was a retrospective study, involving 128 patients with upper extremity arterial trauma operated between January first, 2006 and June 30, 2017. Exclusion criteria were arterial ligation, primary limb amputation and arterial iatrogenic injuries. End points were immediate technical success, primary patency and limb salvage rate.ResultsThe average age was 27.7 years with a sex ratio M/F = 41, causes of trauma were self-inflicted wounds (51%), assaults (23%), road traffic accidents (10%), work accidents (9%) and domestic accidents (7%). Injured arteries were brachial (66.5%) usually because of self-inflicted injuries; arteries of the forearm (31%) and axillery arteries (2.5%). The techniques of arterial repair were vein graft interposition in 52% of cases, end-to-end anastomosis in 23%, primary arterial repair in 21% and venous patch in 4%. Eight reconstructions occluded during the first week (6.25%). Four patients required secondary amputation and limb salvage rate was 96.8%. After a median follow-up time of 62 days, only 21% were followed at 3 months. Mechanism of injury, soft tissue loss and arterial reconstruction thromboses were selected as factors influencing the rate of limb salvage. One death occurred at day 14 secondary to multi-component poly-trauma.ConclusionPrompt diagnosis, appropriate multidisciplinary management of the upper extremity arterial trauma and a readiness to revise the vascular repair early in the event of failure will maximize patient survival and upper extremity salvage. Associated soft tissue injury is a poor limb salvage factor.  相似文献   

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The anoperineal Crohn??s disease lesions pose a several problems in diagnostic and therapeutic steps. The frequency of these lesions was around 21 to 23% in the literature [1]. The data in the literature are still insufficient in the absence of studies devoted to this location particularly debilitating. The purpose of this literature review is to highlight the current diagnostic and therapeutic modalities and new recommendations.  相似文献   

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