Leiomyosarcoma of renal vein, initially resembling pheochromocytoma.

We report the case of a 43-year-old woman who presented with mild left-sided abdominal pain. Computed tomography and MRI findings revealed a 10.7 × 8.5 × 12 cm left-sided soft tissue mass on the renal vessels pushing away the left kidney. The combination of the patient's medical history (episodes of paroxysmal hypertension accompanied with dizziness and sweating) and radiological findings initially pointed toward the diagnosis of a paraganglioma or ectopic pheochromocytoma. Surgical removal of the tumor and left nephrectomy was performed. Histological findings were consistent with a renal vein leiomyosarcoma of a high malignancy grade. There is no evidence of local recurrence or metastatic disease for more than 2 years postoperatively, although the patient did not receive adjuvant chemotherapy or radiation therapy.

Surgical management of primary aldosteronism. not everything that shines is gold.

BACKGROUND: Primary aldosteronism (PA) is a syndrome which includes a group of clinical entities in which aldosterone production is inappropriately high and nonsupressible by sodium loading. The most frequent causes of PA are adrenal adenoma and unilateral or bilateral primary hyperplasia. METHODS: We report a case of a 55-year-old man with a 10-year history of hypertension in whom functional hormonal studies were indicative of PA. Because adrenal venus sampling was not available at our hospital, the investigation was conducted with a computed tomography (CT) scan and a scan with 131-iodocholesterol (NP-59) which both revealed a left adrenal adenoma. RESULTS: The tumor was excised laparoscopically without any complications and the histological findings confirmed the diagnosis of an aldosterone-producing adenoma. Blood pressure remained normal despite the discontinuation of antihypertensive drugs, further supporting that the adrenal tumor was indeed the cause of high blood pressure. Unfortunately, blood pressure began to rise again 2 months later, and laboratory findings indicated the presence of PA once again. Spironolactone was instituted and blood pressure significantly improved and was finally controlled by the addition of amlodipine. CONCLUSION: We report this case to underline the difficulties in the discrimination between adenoma and hyperplasia in everyday clinical practice. Although the CT and scintigraphic findings strongly pointed toward an adenoma, the fact that PA re-appeared shortly after the operation, indicated that the underlying cause of the PA was hyperplasia and not adenoma after all.

Double probe pH-monitoring findings in patients with benign lesions of the true vocal folds: comparison with typical GERD and the effect of smoking.

We conducted a pH-monitoring study to determine the prevalence of pathologic gastroesophageal reflux (GER+) and laryngopharyngeal reflux (LPR+) in patients with resected benign true vocal fold lesions (TVFLs) and positive reflux finding score (RFS). We compared our findings with those of patients with typical GER disease (GERD) symptoms and normal laryngoscopy. In the group of patients with TVFLs, we compared the pH-monitoring findings of smokers with those of non-smokers. Seventy-two [females 32, mean (SD) age 49.3 (13.1) years] patients with resected TVFLs (polyps: 32, nodules: 20, Reinke's edema: 12, granulomas: 4, leukoplakia: 4) and 24 [females 14, mean (SD) age 42.2 (13.4) years] patients with typical GERD symptoms, who served as controls for the hypopharyngeal measurements, underwent 24-h double probe, hypopharyngeal and distal esophageal, ambulatory pH monitoring. Thirty-eight (52.8%) patients with TVFLs had GER+ and 52 (72.2%) had LPR+. More laryngopharyngeal reflux episodes (LPREs) were detected in patients with TVFLs compared to those with GERD (P < 0.001). With respect to the specific TVFLs, 12 (37.5%) patients with polyps had GER+ and 24 (75%) had LPR+, 6 (30%) patients with nodules had GER+ and 12 (60%) had LPR+, 6 (50%) patients with Reinke's edema had GER+ and 8 (66.7%) had LPR+ and all the patients with granuloma or leucoplakia had both GER+ and LPR+. Twenty (55.6%) of the 36 smokers and 32 (88.9%) of the 36 non-smokers with TVFLs had LPR+ (P = 0.003), while GER+ was recorded in 16 (44.4%) smokers and 22 (61.1%) non-smokers (P = 0.238). Smokers had significantly less LPREs (P < 0.001). In conclusion, 24-h double probe pH monitoring may detect GER+ and/or LPR+ in a substantial proportion of patients with resected TVFLs and positive RFS. Our study suggests that LPR+ is more prevalent in patients with TVFLs compared with typical GERD patients and that non-smokers with TVFLs are more likely to have LPR+ than smokers with TVFLs.

Decreased platelet nitric oxide contributes to increased circulating monocyte-platelet aggregates in hypertension.

AIMS: The aim of this study was to determine the effect of blood pressure (BP) on platelet nitric oxide (NO) signalling and on formation of circulating monocyte-platelet aggregates (MPA), as well as the role of platelet NO in modulating MPA in hypertension. METHODS AND RESULTS: We first examined platelet NO signalling in 23 untreated hypertensive (UH) and 23 normotensive (NT) subjects. Platelets from hypertensives exhibited reduced NO synthase activation by albuterol or collagen, as well as suppressed basal and stimulated NO-attributable cyclic guanosine-3',5'-monophosphate, compared with NT. In a second study, comprising 106 subjects with a wide BP range, circulating MPA showed a strong positive correlation with BP. On multiple regression analysis, using a model incorporating systolic BP (SBP), diastolic BP, age, lipids, gender, and smoking status, the only independent predictor of MPA was SBP. Nitric oxide synthase inhibition with N(G)-monomethyl-L-arginine increased MPA in NT but not in hypertensives, whereas the NO donor spermine NONOate (SNO) decreased MPA in NT but not in hypertensives. Platelet P-selectin expression was higher in hypertensives than in NT, and its expression was suppressed by SNO in NT only. CONCLUSION: Platelet NO production and responsiveness are suppressed with raised BP, and this may contribute to the increase in platelet P-selectin and hence in circulating MPA in hypertension.

The interaction of vasoactive substances during exercise modulates platelet aggregation in hypertension and coronary artery disease.

BACKGROUND: Acute vigorous exercise, associated with increased release of plasma catecholamines, transiently increases the risk of primary cardiac arrest. We tested the effect of acute submaximal exercise on vasoactive substances and their combined result on platelet function. METHODS: Healthy volunteers, hypertensive patients and patients with coronary artery disease (CAD) performed a modified treadmill exercise test. We determined plasma catecholamines, thromboxane A2, prostacyclin, endothelin-1 and platelet aggregation induced by adenosine diphosphate (ADP) and collagen at rest and during exercise. RESULTS: Our results during exercise showed a) platelet activation (increased thromboxane B2, TXB2), b) increased prostacyclin release from endothelium and c) decreased platelet aggregation in all groups, significantly more in healthy volunteers than in patients with CAD (with hypertensives lying in between these two groups). CONCLUSION: Despite the pronounced activation of Sympathetic Nervous System (SNS) and increased TXB2 levels during acute exercise platelet aggregation decreases, possibly to counterbalance the prothrombotic state. Since this effect seems to be mediated by the normal endothelium (through prostacyclin and nitric oxide), in conditions characterized by endothelial dysfunction (hypertension, CAD) reduced platelet aggregation is attenuated, thus posing such patients in increased risk for thrombotic complications.

Pheochromocytoma presenting as diabetic ketoacidosis.

Pheochromocytoma is a rare form of secondary hypertension and may be potentially lethal if left untreated. The classical symptoms are paroxysmal hypertension, headaches, palpitations and sweating. They are caused by sudden catecholamine release. Hyperglycemia is reported in some patients with pheochromocytoma but diabetic ketoacidosis is an extremely rare complication of pheochromocytoma with only four cases reported. We report a case of a young woman with pheochromocytoma manifested as diabetic ketoacidosis.

Male and female sexual dysfunction: is hypertension an innocent bystander or a major contributor?

Sexual dysfunction represents a major quality-of-liferelated health problem, and available data indicate that essential hypertension is a risk factor for sexual dysfunction in both men and women. Male and female sexual dysfunction is more prevalent in hypertensive than normotensive individuals, and several mechanisms have been implicated in the pathogenesis of sexual dysfunction in hypertensive patients. Several factors affect the sexual function of hypertensive function, such as severity and duration of hypertension, age, and antihypertensive therapy. Older antihypertensive drugs (diuretics, ß-blockers, centrallyacting) exert negative results while never drugs have either neutral (Ca-antagonists, ACE-inhibitors) or beneficial effects (angiotensin receptor blockers). Female sexual dysfunction, although more frequent than the male one, remains largely under investigated possibly due to the lackof effective treatment. A better understanding of sexual functioning and appropriate education of doctors at medical schools and specific seminars would result in a more effective approach of sexual dysfunction by practitioners dealing with hypertensive patients.

Vascular endothelial growth factor and nitric oxide serum levels in arterial hypertension.

Vascular endothelial growth factor (VEGF) is a specific mitogen for endothelial cells and an inducer of the angiogenic procedure. Endothelial nitric oxide (NO) is a potent vasodilator. Because both are implicated in vascular function, it is possible that they would be significantly affected in arterial hypertension. The aim of the study was the investigation of the levels of the two substances in blood serum and their potential relation in patients with untreated essential arterial hypertension compared to healthy individuals. VEGF levels were found 129.1 +/- 135.9 pg/mL in a group of 28 untreated hypertensive patients vs. 209.0 +/- 133.3 pg/mL in a group of 28 healthy individuals (p = 0.008). Nitrites and nitrates levels (as an indirect index of calculating NO levels) were also lower in hypertensive patients than in healthy individuals (19.8 +/- 9.7 micromol/L vs. 29.6 +/- 15.9 micromol/L, p = 0.014). A positive correlation between NOx (nitrites and nitrates levels) and VEGF was found in healthy individuals (r = 0.55, p = 0.003), but there was no correlation in hypertensive patients. The significant decrease of serum VEGF and NO in arterial hypertension and the existence of a correlation between the two substances in healthy subjects that did not exist in the hypertensive patients are findings that need evaluation.

Colchicine treatment of liver fibrosis.

BACKGROUND/AIMS: Because of its antifibrotic and anti-inflammatory effects, colchicine has been proposed as a treatment for liver disease. The results from clinical trials have however been inconsistent. The aim of the present study was to evaluate the effect of colchicine in the treatment of patients with hepatic fibrosis of various etiologies. METHODOLOGY: Thirty-eight patients were randomized to receive either colchicine 1 mg per day (n=21, group A) or no antifibrotic agent (n=17, group B). Treatment lasted for at least 12 months. Liver biopsy was performed prior to entry and after 12 months. Liver function tests, serum aminoterminal peptide of procollagen III (PIIINP) levels and CD4:CD8 ratio of peripheral blood T lymphocytes (PBTLs) were performed at baseline and bimonthly or every 4 months post-treatment. RESULTS: Mean albumin serum levels increased 12 months post-treatment period only in group A (p<0.05). Mean serum PIIINP levels did not change significantly after 12 months of treatment in group A; in 7 patients a reduction in mean serum PIIINP levels was noticed during 24-month post-treatment follow-up period (p<0.05). At baseline, a correlation between focal or bridging necrosis and CD4:CD8 ratio of PBTLs was noticed in group A (p < 0.05). The mean serum CD4:CD8 ratio was increased after 12 months of colchicine treatment (p<0.05) associated with abrogation of this correlation; comparison between the two groups revealed increased CD4:CD8 ratio in group A at 12 months (p<0.05). The histological findings according to Knodell criteria in both groups remained unchanged after 12 months follow-up. The treatment was well tolerated in all patients. CONCLUSIONS: Long-term colchicine treatment in patients with hepatic fibrosis appears to exert an anti-inflammatory, anti-fibrotic and immunomodulatory effect.

The unilateral measurement of blood pressure may mask the diagnosis or delay the effective treatment of hypertension.

Several studies have indicated the presence of significant interarm blood pressure (BP) differences; this could result in misclassification of BP status. Nevertheless, the findings of these studies were not consistent. This prospective, observational study investigated the presence and magnitude of interarm BP differences and determined the influence of age, gender, arm circumference, smoking, being hypertensive or diabetic, or having a previous history of cardiovascular disease in these differences. The study included 384 subjects, who were patients, ward visitors, and members of the nursing staff of this Department. BP measurements were recorded simultaneously in both arms by using 2 validated, fully automated oscillometric electronic devices. There were significant differences between the right arm and left arm systolic BP (p < 0.0005), between right arm and left arm diastolic BP (p < 0.05), and between right arm and left arm pulse pressure (p = 0.006). The mean interarm differences in systolic and diastolic BP measurements were 1.2 +/-5.0 mm Hg and 0.4 +/-4.2 mm Hg, respectively. There were 13 subjects (3.4%) and 4 subjects (1.04%) with an interarm systolic and diastolic BP difference of > 10 mm Hg, respectively, and a single patient with both interarm systolic and diastolic BP differences of > 10 mm Hg (0.26%). None of the studied demographic or clinical characteristics was a significant predictor of interarm systolic and diastolic BP differences. The authors conclude that significant interarm systolic and diastolic BP differences are frequently present. Therefore, the unilateral measurement of BP may mask the diagnosis or delay the effective treatment of hypertension. It is thus recommended that BP should be simultaneously measured in both arms at the initial consultation and the higher of the 2 readings should be used to guide further management decisions.