Icatibant: a novel approach to the treatment of angioedema related to the use of angiotensin-converting enzyme inhibitors.

Angioedema related to the use of angiotensin-converting enzyme inhibitors (AE-ACEi) has, so far, been treated with antiallergic drugs with questionable results. Because angioedema in this setting is likely related to increased levels of bradikinin, we decided to use icatibant, a bradikinin receptor antagonist licensed for use in hereditary angioedema, in a patient with AE-ACEi. In the same patient, the time to resolution of the angioedema during previous attacks was about 2 days when classic antiallergic drug regimens were used; when icatibant was used, this time shortened to 10 hours. Icatibant is a promising drug in the treatment of AE-ACEi.

An Italian registry of chest pain patients in the emergency department: clinical predictors of acute coronary syndrome.

BACKGROUND: The aim of this study was to describe the population of patients arriving in several Italian Emergency Departments (EDs) complaining of chest pain suggestive of acute coronary syndrome (ACS) in order to evaluate the incidence of ACS in this cohort and the association between ACS and different clinical parameters and risk factors. METHODS: This is an observational prospective study, conducted from the 1st January to the 31st December 2014 in 11 EDs in Italy. Patients presenting to ED with chest pain, suggestive of ACS, were consecutively enrolled. RESULTS: Patients with a diagnosis of ACS (N.=1800) resulted to be statistically significant older than those without ACS (NO ACS; N.=4630) (median age: 70 vs. 59, P<0.001), and with a higher prevalence of males (66.1% in ACS vs. 57.5% in NO ACS, P<0.001). ECG evaluation, obtained at ED admission, showed new onset alterations in 6.2% of NO ACS and 67.4% of ACS patients. Multiple logistic regression analysis showed that the following parameters were predictive for ACS: age, gender, to be on therapy for cardio-vascular disease (CVD), current smoke, hypertension, hypercholesterolemia, heart rate, ECG alterations, increased BMI, reduced SaO2. CONCLUSIONS: Results from this observational study strengthen the importance of the role of the EDs in ruling in and out chest pain patients for the diagnosis of ACS. The analysis put in light important clinical and risk factors that, if promptly recognized, can help Emergency Physicians to identify patients who are more likely to be suffering from ACS.

Rendu-Osler-Weber disease: experience with 56 patients.

Rendu-Osler-Weber disease, or hereditary hemorrhagic telangiectasia (HHT), is an autosomal dominant disease characterized by systemic vascular dysplasia. The prevalence varies and ranges, according to region, from 1/3500 to 1/5000. Data concerning Italy are not available. The diagnosis is based on the following criteria: family history, epistaxis, telangiectases and visceral arteriovenous malformations. The diagnosis is to be considered definite if three criteria are present and suspected if two criteria are present. From September 2000 to March 2002, 100 patients (63 males, 37 females, mean age 45.5 +/- 17.3 years) potentially affected by HHT were evaluated in the HHT Center of the "Augusto Murri" Internal Medicine Section at the University of Bari (on a day-hospital or hospitalization basis). The diagnosis of HHT was confirmed in 56 patients and suspected in 10. Magnetic resonance imaging revealed cerebral arteriovenous malformations in 8.5% of patients. In 14.6% of patients contrast echocardiography revealed pulmonary arteriovenous malformations subsequently confirmed at multislice computed tomography in all cases but one. In 48.2% of subjects hepatic vascular malformations were revealed by echo color Doppler ultrasonography, whereas abdominal multislice computed tomography was positive in 63.8% of patients. In 64% of the 25 patients, who underwent endoscopy, gastric telangiectases were found. In 3 out of 6 patients presenting with pulmonary arteriovenous malformations, embolotherapy was performed with success. In our patients, the use of tranexamic acid caused a reduction in the frequency of epistaxis. The future objectives of the HHT Center of Bari are to increase knowledge of the disease, to cooperate with other centers with the aim of increasing the number of patients studied and to avoid the limits of therapeutic and diagnostic protocols of a rare disease such as HHT.

Unusual cause of chest pain on radiograph.

Although pneumomediastinum (PM) is a cause of chest pain, which can be diagnosed on a plain chest radiograph, emergency physicians frequently miss the diagnosis. As follows a description of findings of PM on a chest radiograph.

Acute Medicine and Emergency Medicine are not (Yet?) The Same.

The acquisition of "Level 3" competencies in the setting of the Emergency Room from StR in Acute Medicine raises the question of the boundaries and competences of Acute Medicine and Emergency Medicine. The possibility that phisycians with a training both in Emergency Medicine and Acute Medicine will be involved in the management of Emergency Admission Units and Emergency Medicine Departments in the future is herein considered.

Pulmonary arteriovenous malformations, hereditary hemorrhagic telangiectasia, and brain abscess.

Hereditary hemorrhagic telangiectasia (HHT) is a systemic angiodysplasia inherited as an autosomal dominant disease. Patients with HHT and pulmonary arteriovenous malformations (PAVMs) are at increased risk for brain abscess (BA), a potentially preventable condition as effective treatment for PAVMs is available. In a center dedicated to HHT, a history of BA was found in 6 out of 128 patients with a definite diagnosis: herewith, their histories are reported focusing on mistakes in the diagnosis and management of the disease. Patients with PAVMs and BA had a higher mean hemoglobin concentration (15.1 g/dl vs. 12.2 g/dl, p < 0.006 by Student's t test) compared to patients with PAVMs alone. Other clinical features (genetics, bacteriology, types of PAVMs, treatments, outcomes) are also discussed. Prompt diagnosis and screening for visceral involvement is pivotal for HHT patients and their relatives.

Brain abscess: a need to screen for pulmonary arteriovenous malformations.

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are direct connections between an artery and a vein in the pulmonary circulation associated with hereditary hemorrhagic telangiectasia in up to 88% of cases. Patients with PAVMs are at increased risk of brain abscess (BA). This study aimed to provide preliminary data on the prevalence of PAVMs among BA patients. METHODS: Administrative hospital discharge forms were used to identify patients with BA; possible PAVM patients were screened. RESULTS: 126 patients with BA were identified. Two patients had undiagnosed PAVMs at the time of admission for BA. The age-adjusted incidence of BA was 6.3 cases/1 million/year, with a male:female ratio of 2.0. CONCLUSION: Although PAVMs are rare conditions, they play a role in the development of BA. PAVMs are usually not recognized at the time of BA, thus exposing patients to life-threatening risks.

Diagnosis and management of gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia.

OBJECTIVE: Our aim was to report our experience with treating GI bleeding in patients with hereditary hemorrhagic telangiectasia (HHT). METHODS: Consecutive patients with GI bleeding referred to the Yale University Vascular Malformation Center underwent clinical evaluation and endoscopy. Hb and blood transfusion requirements for 1 yr before and after evaluation were documented. Patients with a mean Hb or= 12 units packed red blood cells (PRBC)/yr were defined as patients with significant bleeding. Drug therapies, including ethinyl estradiol/norethindrone, danazol, and aminocaproic acid, were prescribed on an individual patient basis. RESULTS: The study included 43 HHT patients with a mean age of 57 yr. Endoscopy revealed telangiectases in the esophagus (1/41), stomach (33/41), duodenum (33/41), jejunum (5/9), and colon (10/32). Patients with > 20 telangiectases visualized on esophagogastroduodenoscopy had a significantly lower mean Hb of 7.9, compared with 9.4 (p = 0.007), and a trend toward higher blood transfusion requirements. Non-HHT-related causes of GI bleeding were diagnosed in four patients. During a mean follow up of 18.9 months, the group of 40 patients with HHT-related bleeding had improvements in their mean Hb and blood transfusion requirements. CONCLUSIONS: Some HHT patients with GI bleeding improve on drug therapies, but others fail. Transfusion-dependent GI bleeding is difficult to manage, and optimal management may include both medical and endoscopic treatments.

Vascular endothelial growth factor serum levels are elevated in patients with hereditary hemorrhagic telangiectasia.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a genetic angiodysplasia affecting multiple organs. Two genes involved in the transduction of TGF-beta signalling are responsible for HHT. An additional role for vascular endothelial growth factor (VEGF) has been proposed. Serum VEGF, which has been evaluated in several diseases characterized by aberrant angiogenesis, has never been measured in patients with HHT. AIMS: To evaluate VEGF serum levels in HHT patients as compared to normal subjects. MATERIALS AND METHODS: 32 HHT patients (age 47.7 +/- 16.7 years) and a control group of 37 healthy subjects (age 48.2 +/- 15.5 years) were entered in the study. Each patient underwent serum VEGF dosage using a commercial ELISA specific for the human molecule. RESULTS: The serum level of VEGF in HHT patients was 196.3 +/- 103.2 pg/ml, while it was 152.0 +/- 84.1 pg/ml in the control group. Statistical analysis showed that serum VEGF was significantly higher in HHT patients than in the controls (p < 0.031). CONCLUSIONS: According to a study performed in a murine model, persistence of the activation phase of angiogenesis might be responsible for an increased production of several angiogenic factors, in particular VEGF, in HHT. Our work is the first to suggest an increased expression of VEGF in the serum of subjects with HHT in agreement with the stimulation of VEGF synthesis proposed in the murine model.