Staging and prognostic factors in carcinoma of the base of the tongue.

During the period 1966-1985, 66 patients were submitted for curative treatment of a carcinoma of the base of the tongue in the Netherlands Cancer Institute. Treatment consisted of radiotherapy (59 patients), surgery and post-operative radiotherapy (4 patients) and surgery alone (3 patients). Patients were staged according to the UICC (1982) and UICC (1987)/AJCC (1988) criteria. Regrouping by the latter system caused enlargement of the N2-group and of stage IV. The crude 5-year survival was 22%, the 5-year tumour control was 36% and the locoregional control was 47%. The most important prognostic factors for the tumour-free interval are the T-category (P = 0.01) and stage grouping (UICC 1982) (P = 0.022). The same factors predict the locoregional control (P = 0.005 and 0.02 respectively). Crude survival is lower in smokers, and in patients in poor general condition (P = 0.04 and 0.007 respectively).     

Preoperative embolization in surgical treatment of a primary hemangiopericytoma of the rib: a case report

Primary hemangiopericytoma of the rib is extremely rare and only a few cases have been reported. A 62-yr-old man presented with an aching chest pain and dyspnea. Thoracic computed tomography revealed a homogenous mass expanding the right seventh rib. A diagnosis of hemangiopericytoma was established by percutaneous needle biopsy. Preoperative embolization of the feeding vessels of the tumor was performed in order to prevent perioperative bleeding. There was no significant bleeding during the surgery, where complete resection of the tumor with 7th to 9th ribs with a surgical margin of 5 cm was performed. Postoperative course was uneventful and there has been no recurrence for thirteen months. To our knowledge, there has been no report to apply a preoperative embolization of a primary hemangiopericytoma of the rib.     

Fourteen novel OPA1 mutations in autosomal dominant optic atrophy including two de novo mutations in sporadic optic atrophy

The OPA1 gene, encoding a dynamin-related GTPase that plays a role in mitochondrial biogenesis, is implicated in most cases of autosomal dominant optic atrophy (ADOA). Sixty-nine pathogenic OPA1 mutations have been reported so far. Most of these are truncating mutations located in the GTPase domain coding region (exons 8-16) and at the 3'-end (exons 27-28). We screened 44 patients with typical ADOA using PCR-sequencing. We also tested 20 sporadic cases of bilateral optic atrophy compatible with ADOA. Of the 18 OPA1 mutations found, 14 have never been previously reported. The novel mutations include one nonsense mutation, 3 missense mutations, 6 deletions, one insertion and 3 exon-skipping mutations. Two of these are de novo mutations, which were found in 2 patients with sporadic optic atrophy. The recurrent c.2708_2711delTTAG mutation was found in 2 patients with a severe congenital presentation of the disease. These results suggest that screening for OPA1 gene mutations may be useful for patients with optic atrophy who have no affected relatives, or when the presentation of the disease is atypical as in the case of early onset optic atrophy.     

Byssinosis in carpet weavers exposed to wool contaminated with endotoxin

All the 303 full time day workers in a carpet weaving factory were submitted to a physical examination, chest radiography, and vitalograph test, and answered a respiratory questionnaire. Fifty four healthy non-exposed subjects served as controls. Dust concentrations and concentrations of bacterial endotoxin were measured. Of the 303 workers, 259 (85.5%) had airway symptoms and 62 (20.5%) had maximum mid-expiratory flow (MMF) values of less than 60% compared with 9.2% of the controls. The symptoms in 68 workers (22%) were compatible with byssinosis and 36 of these workers underwent vitalography before starting work and after four hours work on Mondays when significant reductions of their FEV1 and MMF were found. Twenty one of these 36 workers were tested on Tuesday and no differences in these measurements were found between measurements before work started and four hours later. The airborne dust concentrations in the factory were high and bacterial endotoxin was found. These findings suggest that a large number of workers in this carpet weaving factory suffer from a disease indistinguishable from byssinosis even though wool is used almost exclusively, the only cotton being the warp. The finding of endotoxin together with the absence of cotton confirms the theory that "byssinosis" is due to bacterial endotoxin rather than to cotton per se.     

Spondylometaphyseal dysplasia,type VII

Spondylometaphyseal dysplasia was first described by Kozlowski et al. in 1967 as a new dysplastic bone disease, characterized by metaphyseal dysplasia associated with generalized platyspondyly in the vertebral column [1]. Kozlowski et al. have pointed out the autosomal dominant transmission of this disorder at that time. However, later reports showed that the manner of genetic transmission and the degree of the manifestation of the radiological findings could be variable and accordingly seven types of spondylometaphyseal dysplasia were described [2]. In this article, three cases displaying one of the rare forms of spondylometaphyseal dysplasia, type VII are presented and the diagnostic findings as well as the differential diagnostic criteria are discussed.     

Variation in innate immunity genes and risk of multiple myeloma

Multiple myeloma (MM) is a B‐cell lymphoid malignancy suspected to be associated with immunologic factors. Given recent findings associating single‐nucleotide polymorphisms (SNPs) in innate immunity genes with non‐Hodgkin lymphoma, we conducted an investigation of innate immune gene variants using specimens from a population‐based case‐control study of MM conducted in Connecticut women. Tag SNPs (N = 1461) summarizing common variation in 149 gene regions were genotyped in non‐Hispanic Caucasian subjects (103 cases, 475 controls). Odds ratios (OR) and 95% confidence intervals (CI) relating SNP associations with MM were computed using unconditional logistic regression, while the MinP test was used to investigate associations with MM at the gene level. We calculated permutation‐adjusted P‐values and false discovery rates (FDR) to account for the number of comparisons performed in SNP‐level and gene‐level tests, respectively. Three genes were associated with MM when controlling for a FDR of ≤10%: SERPINE1 (P_MinP < 0.0001; FDR = 0.02), CCR7 (P_MinP = 0.0006; FDR = 0.06) and HGF (P_MinP = 0.001; FDR = 0.08). Two SNPs demonstrated robust associations: SERPINE1 rs2227667 (P = 2.1 × 10^?5, P_permutation = 0.03) and HGF rs17501108 (P = 5.0 × 10^?5, P_permutation = 0.07). Our findings suggest that genetic variants in SERPINE1 and HGF, and possibly CCR7, are associated with MM risk, and warrant further investigation in other studies. Copyright © 2010 John Wiley & Sons, Ltd.     

Clinical Perspectives and Pearls from the 2015 ESC NSTE-ACS Guidelines

Four years after the latest edition, the 2015 non-ST-segment elevation acute coronary syndromes guidelines of the European Society of Cardiology have been published. Novel aspects include a new diagnostic algorithm for non-ST-segment elevation myocardial infarction using high-sensitivity cardiac troponins as well as guidance on cardiac rhythm monitoring duration. A large section is dedicated to antiplatelet therapy including initiation and duration of dual-antiplatelet therapy as well as the management of patients requiring, at the same time, long-term oral anticoagulation. New sections include the management of antiplatelet agent in patients requiring coronary artery bypass surgery and of acute bleeding events related to antiplatelet agents, vitamin K antagonist (VKA), and non-VKA oral anticoagulant drugs. Current evidence supports the radial access over the femoral one for coronary angiography and percutaneous revascularization.     

Usefulness of preprocedural soluble CD40 ligand for predicting restenosis after percutaneous coronary intervention in patients with stable coronary artery disease

CD40-CD40 ligand interaction is involved in the inflammatory pathogenesis of atherosclerosis but clinical data about its role in stent restenosis are still limited. We investigated the effect of preprocedural CD40 ligand (sCD40L) on stent restenosis. We enrolled 36 patients (mean age 61.4 +/- 8.5 years) with stable angina who underwent successful stent implantation. Control angiograms were performed in all patients after 6 months. Plasma sCD40L and high-sensitive C-reactive protein levels were measured before stent implantation and at 1 and 6 months after the procedure. Angiographically proven restenosis rate was 27.8%. Plasma sCD40L levels were significantly higher (preprocedural 0.74 +/- 0.79) and more prolonged in patients with stent restenosis compared with patients without stent restenosis (0.02 +/- 0.22 ng/ml, p < 0.001). According to receiver-operator characteristic analysis, sCD40L > 0.41 ng/ml was the best distinguished parameter between patients with and without restenosis. At the multivariate logistic regression analysis, preprocedural sCD40L was an independent predictor (RR 39.4, 95% confidence interval 4.05 to 383.8, p = 0.002) of stent restenosis after adjusting for confounding variables, including diabetes, reference vessel diameter, lesion length, stent diameter, stent length, and baseline high-sensitive C-reactive protein. Sensitivity, specificity, and positive and negative predictive values and likelihood ratio of preprocedural sCD40L levels in stent restenosis were 78%, 92%, 78%, 92%, and 9.37%, respectively. In conclusion, enhanced inflammation of plaque (increased sCD40L) before percutaneous coronary intervention may increase the rate of stent restenosis. Increased preprocedural sCD40L level is an independent predictor of stent restenosis. We can use this marker for the assessment of risk stratification before planning stent implantation.