Veinous thrombectomy. Six case reports]

The aim of this study is to raise up the effect of surgical thrombectomy among other alternative therapies. This retrospective study reports 6 patients (mean age 63 years) admitted with phlegmasia cerulea dolens. All patients underwent surgical venous thrombectomy associated with infracava filter insertion in 2 cases. One patient died in the early postoperative course. In all other cases we noticed good early and late outcome both on clinical examination and duplex scanning assessment. In conclusion, surgical venous thrombectomy can be considered as a good and efficient procedure in the presence of phlegmasia cerulea dolens in order to relieve ischemia and to prevent whenever possible severe chronic venous disorders. However, fibrinolytic therapy might achieve as good results as surgery. Thus, the latter is to be reserved to very severe veinous ischemia with limb loss threatening where fibrinolytic therapy fails or is contre-indicated.     

Saffron (Crocus sativus L.): A Source of Nutrients for Health and for the Treatment of Neuropsychiatric and Age-Related Diseases

Saffron (Crocus sativus L.) is a medicinal plant, originally cultivated in the East and Middle East, and later in some Mediterranean countries. Saffron is obtained from the stigmas of the plant. Currently, the use of saffron is undergoing a revival. The medicinal virtues of saffron, its culinary use and its high added value have led to the clarification of its phytochemical profile and its biological and therapeutic characteristics. Saffron is rich in carotenoids and terpenes. The major products of saffron are crocins and crocetin (carotenoids) deriving from zeaxanthin, pirocrocin and safranal, which give it its taste and aroma, respectively. Saffron and its major compounds have powerful antioxidant and anti-inflammatory properties in vitro and in vivo. Anti-tumor properties have also been described. The goal of this review is to present the beneficial effects of saffron and its main constituent molecules on neuropsychiatric diseases (depression, anxiety and schizophrenia) as well as on the most frequent age-related diseases (cardiovascular, ocular and neurodegenerative diseases, as well as sarcopenia). Overall, the phytochemical profile of saffron confers many beneficial virtues on human health and, in particular, on the prevention of age-related diseases, which is a major asset reinforcing the interest for this medicinal plant.     

UHRF1 regulation of the Keap1–Nrf2 pathway in pancreatic cancer contributes to oncogenesis

The cellular defence protein Nrf2 is a mediator of oncogenesis in pancreatic ductal adenocarcinoma (PDAC) and other cancers. However, the control of Nrf2 expression and activity in cancer is not fully understood. We previously reported the absence of Keap1, a pivotal regulator of Nrf2, in ～70% of PDAC cases. Here we describe a novel mechanism whereby the epigenetic regulator UHRF1 suppresses Keap1 protein levels. UHRF1 expression was observed in 20% (5 of 25) of benign pancreatic ducts compared to 86% (114 of 132) of pancreatic tumours, and an inverse relationship between UHRF1 and Keap1 levels in PDAC tumours (n = 124) was apparent (p = 0.002). We also provide evidence that UHRF1‐mediated regulation of the Nrf2 pathway contributes to the aggressive behaviour of PDAC. Depletion of UHRF1 from PDAC cells decreased growth and enhanced apoptosis and cell cycle arrest. UHRF1 depletion also led to reduced levels of Nrf2‐regulated downstream proteins and was accompanied by heightened oxidative stress, in the form of lower glutathione levels and increased reactive oxygen species. Concomitant depletion of Keap1 and UHRF1 restored Nrf2 levels and reversed cell cycle arrest and the increase in reactive oxygen species. Mechanistically, depletion of UHRF1 reduced global and tumour suppressor promoter methylation in pancreatic cancer cell lines, and KEAP1 gene promoter methylation was reduced in one of three cell lines examined. Thus, methylation of the KEAP1 gene promoter may contribute to the suppression of Keap1 protein levels by UHRF1, although our data suggest that additional mechanisms need to be explored. Finally, we demonstrate that K‐Ras drives UHRF1 expression, establishing a novel link between this oncogene and Nrf2‐mediated cellular protection. Since UHRF1 over‐expression occurs in other cancers, its ability to regulate the Keap1–Nrf2 pathway may be critically important to the malignant behaviour of these cancers. © 2015 The Authors. Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.     

Bronchopulmonary aspergillosis: study of 17 cases

Aspergillosis is a fungic infection depending on the local or general physiologic and immunologic state of the host. We report the result of retrospective five year study (1995-1999) about 17 cases in the laboratory of Parasitology-Mycology of Rabta hospital in Tunis. Six aspergillomas were observed, they occurred after a pulmonary tuberculosis, two cases of allergic broncho-pulmonary aspergillosis described in two asthmatic patients, nine cases of invasive pulmonary aspergillosis complicating two cancers, one leukaemia, six chronic granulomatous disease. Aspergillus fumigatus is the most frequent species (67%). The clinical and biological characteristic of those will be studied, and compared with those of the literature.     

Relevant factors in mitral valve reconstructive surgery. Report of 150 cases

150 patients had mitral valve repair for mitral valve incompetence. There were 57 males and 93 females with a mean age of 22 years, 39% were less than 15 years of age. 60% of the patients were in Class II NYHA and 40% in Class III and IV. According to Carpentier's classification, isolated type I was present in 18 patients (12%), type II in 98 patients (64%) and type III in 34 cases (24%). Mitral repair included correction of valve prolapse, valvular enlargement with pericardial patch and annuloplasty with semi-rigid ring in 128 cases and PTFE patch along the posterior leaflet in 12 cases. The perioperative mortality rate was 2.6% (4 patients). All patients had early post-operative echocardiography. According to this, mitral regurgitation was absent or tiny in 135 patients (92%), grade II in 10 cases and grade III in 2 cases. It was moderate or important in twelve patients (8%). In the late post-operative period, three patients were lost to follow-up. All the others patients were reoperated upon for mitral dysfunction in a mean time of 37 months. The reason for reoperation was in the majority of the cases the recurrence of mitral regurgitation related to increase of valvular and sub valvular disease. The late mortality rate is 7% (10 patients). Out of 126 reviewed survivors on the long run, 71 patients (56%) are asymptomatic in class I, 53 patients (42%) in class II and 2 patients in class III NYHA. Mitral valvuloplasty is the preferred procedure in mitral insufficiency surgical management. It is associated to a low early mortality and morbidity rate. Despite the need for reoperation in about 10% of the cases in the long follow-up, mitral repair offers a good quality of life without anticoagulant treatment.     

Genetic profile of Glanzmann's thrombasthenia in south Tunisia. Report of 17 cases (11 families)

INTRODUCTION: Glanzmann's thrombasthenia (GT) is a rare congenital thrombopathy, with a recessive autosomal transmission. We present here the genealogic study of a series of patients suffering from GT. PATIENTS AND METHODS: This is a retrospective study about all the GT patients treated in Sfax hematology department during 18 years. Final diagnosis was established by agregometry. Genealogic study was based on clinical history. RESULTS: 17 cases of GT from 11 families from the south of Tunisia wen collected. The disease was paricularly frequent in the region of Moulares-Gafsa (7 patients). The percentage of consanguinity was also very high (82%), with a third degree consanguinity of 86%. Family investigation revealed 6 previously unknown cases, and 10 deaths subsequent to hemorrhagic manifestations. CONCLUSION: The high rate of consanguinity, the absence of clinical or biological manifestations in the parents, and the ratio of ill to normal subjects in the same family which was about one to four, are suggestive of an autosomal recessive mode of transmission.     

Association of systemic lupus erythematosus and dermatopolymyositis

Systemic lupus erythematosus (SLE) associated with dermatopolymyositis (DM). This association is rare. Diagnosis may be difficult because of their common clinical findings. We report here a case. A 22-year-old man was admitted for arthritis with fever, diffuse myalgia and periorbital skin heliotrope rash. Electromyogram and muscular biopsy were suggestive of DM. The patient was treated with oral prednisone. Two months and a half later, he was admitted for impure nephrotic syndrome in relation with diffuse proliferative glomerulonephritis. Antibodies against native double-stranded- DNA were positive, and normal skin biopsy showed immune complex deposit on dermo-epidermic junction, suggestive of SLE. The patient was treated with high doses of prednisone and 6 monthly intravenous pulses of cyclophosphamide. Skin lesions and nephrotic syndrome improved. Presently, the patient remains asymptomatic. While being of different pathogenesis, SLE and DM may coexist in the same patient.     

Coronary arteriography in severe aortic valve disease

Three hundred and twelve patients with severe aortic valve disease underwent a pre-operative haemodynamic study including coronary arteriography either as routine (age more than 50 years) or because of chest pains, previous myocardial infarction or for patients with risk factors of coronary atherosclerosis. Significant coronary artery disease was present in 9% of all cases. Coronary artery disease was more frequent in patients with angina and in patients with previous myocardial infarction but none of these factors was specific. We conclude that except young patients < 40 years old, with no chest pain and no coronary risk factors, coronary arteriography is recommended in patients with severe aortic valve disease before aortic valve replacement.