Ruptured Left Subclavian Artery Aneurysm in a 41-Year-Old Woman with Neurofibromatosis Type 1

Abstract Introduction Intrinsic lesions of the arterial wall are important manifestations of Neurofibromatosis type 1. Report A 41-year-old woman with Neurofibromatosis type 1, suffering sudden onset of upper back as well as left shoulder and upper chest pain is addressed to our hospital. The contrast-enhanced thoracic computed tomogram demonstrated a huge hematoma due to ruptured left subclavian artery aneurysm treated with endovascular therapy. Discussion A ruptured left subclavian artery is an uncommon but life threatening manifestation in Neurofibromatosis type 1

A new preoperative predictor of outcome in ruptured abdominal aortic aneurysms: the time before shock (TBS)

Background: In patients with ruptured abdominal aortic aneurysm (RAAA) and shock, the time lag between the onset of the symptoms due to RAAA and the presence of a full developed shock syndrome was evaluated to assess its prognostic meaning. This time lag was called time before shock (TBS). Methods: Ninety-four patients operated on between 2002 and 2007 have been retrospectively analyzed regarding TBS and the following parameters: presence of shock, severity of bleeding, age, comorbidities, and gender. According to TBS, on a 10-hour cutoff value, three groups of patients were distinguished: patients with TBS of 10 or less (short TBS), patients with TBS greater than 10 (long TBS), and patients without shock. The relationship of these variables with intraoperative and 30-day mortality was analyzed by both univariate and multivariate analyses. Results: In the univariate analysis, patients with short TBS presented with four-fold mortality compared to patients without shock (p = 0.000), whereas the increase in mortality of the patients with long TBS was nonsignificant (p = 0.448). The mortality in patients with shock (presence of shock) was 3.7 times higher than in patients without shock (p = 0.001). The mortality related to massive bleeding was 3.7 times higher than that associated with moderate bleeding (p = 0.001). An increased mortality with borderline significance level was observed in patients older than 75 years (p = 0.052). The relationship of mortality to the presence of comorbidities and gender was not significant. In the multivariate analysis, the mortality among the patients with short TBS was clearly highest, after either massive or moderate bleeding. In the logistic model with TBS, the Wald test showed as significant both short TBS (p = 0.001) and severity of bleeding (p = 0.033) but not age (p = 0.103) and long TBS(p = 0.0401). The model with TBS presented a better performance than that with shock, showing higher sensitivity, higher values of Youden's J, and a greater proportion of the total variation in mortality. Through the model with TBS, two groups of patients (those 75 years or younger with massive bleeding and those older than 75 years with moderate bleeding), both with short TBS, presented with a high risk of death not predicted by the model with shock. Conclusion: TBS seems to complete the information given by the parameter "presence of shock," and its evaluation allows a more effective judgment of the risk of death, at emergency admission of patients with RAAA

Carotid Body Paragangliomas and Matrix Metalloproteinases

Paragangliomas (PGLs) are slow-growing, typically, benign tumors that arise from the extra-adrenal paraganglion of the autonomic nervous system. PGLs arising from the carotid body are relatively rare tumors but constitute the majority of head and neck PGLs (60e70%).1 Carotid body tumors (CBTs) belong to the classification of PGLs, because they originate from paraganglia in chromaffin-negative glomus cells derived from the embryonic neural crest, functioning as part of the sympathetic nervous system. These cells normally act as special chemoreceptors located along blood vessels, particularly in the carotid bodies (at bifurcation of the common carotid artery in the neck).2 CBTs are usually classified using the criteria described by Shamblin et al.3 Matrix metalloproteinases (MMPs), enzymes that regulate cell matrix composition, play a role in several clinical conditions,4,5 including embryogenesis, wound healing,6,7 inflammation, arthritis, cardiovascular diseases,8 pulmonary diseases, and cancer.9 Several studies10e15 have shown that specific MMPs are involved in cancer processes that promote metastasis.The aim of this study is to examine the levels of MMPs in patients with benign and malignant neoplastic CBTs

Resection of carotid body tumors reduces arterial blood pressure. An underestimated neuroendocrine syndrome

Introduction: Carotid Body Tumors (CBTs) are Paragangliomas (PGLs) located in the head and neck region which usually do not cause overt neuroendocrine symptoms and hypertension. Matrix Metalloproteinases (MMPs) have shown a strong correlation between CBTs and their clinical behavior. Aim of this study is to analyze the relationship between changes in arterial blood pressure and metalloproteinases levels after surgical resection of CBTs. Methods: We performed a multicenter clinical study on 17 patients with benign and malignant CBTs (5 males; 12 females). Tumors were completely resected and biopsies, obtained at the time of surgery, were lysed for Western blot analysis to determine MMPs levels in tissues. An enzyme-linked immune sorbent assay (ELISA) kit was used to determine the concentration of MMPs in plasma fluid. Blood pressure values were measured at admission and at 10 days after surgery. Results: At the time of the admission, blood pressure values were higher in patients with CBTs respect to control patients; moreover in patients with malignant CBTs blood pressure values were higher (P < 0.01) respect to patients with benign CBTs. 10 days after the surgery, we documented a significant decrease (P < 0.01) in blood pressure values and in MMPs levels in all patients with CBTs. Conclusion: These results suggest that, despite the CTBs are considered non-functional tumors, an "underestimated" neuroendocrine activity on arterial blood pressure may be detected