Luca Bonanni

Clinical and pathological findings of a fatal systemic capillary leak syndrome (Clarkson disease): a case report.

AbstractSystemic capillary leak syndrome (SCLS) is a rare disorder with episodes of hypotension, hypoalbuminemia, and hemoconcentration. During attacks endothelial hyperpermeability results in leakage of plasma proteins into the interstitial space. Attacks vary in severity and may be lethal.A 49-year-old previously healthy man was admitted to hospital for hypovolemic shock, anasarca with pleuropericardial effusion, muscle fatigue, and oliguria occurring after a flu-like syndrome. Laboratory data showed an increase in hematocrit (65%), leucocytes (24.590 &mgr;/L), creatinine (2.5 mg/dL), creatine phosphokinase (10.000 U/L), and a decrease in serum albumin (17 g/L) without proteinuria. Immunoglobulins of class G/&lgr; monoclonal gammopathy were detected (1.3 g/L). The initial suspicions addressed to a protein-loosing syndrome or to an effort-related rhabdomyolysis. Initial therapy was based on steroids, albumin, and high molecular weight plasma expanders (hydroxyethyl starch). Because of high hematocrit, phlebotomy was also performed. The patient had complete clinical remission and a diagnosis of SCLS was finally made. He received prophylactic therapy with verapamil and theophylline that was self-stopped for intolerance (hypotension and tachycardia). He had a new crisis 2 days after a physical effort, and was admitted in intensive care unit. The patient died for severe hypovolemic shock with multiorgan failure and sudden cardiac arrest 15 hours after hospital admission. Postmortem investigation revealed massive interstitial edema of main organs with myocardial hyperacute ischemia.Studies on SCLS are limited for the rarity of the disease and its unpredictable course. Both prophylactic and acute crisis treatments are empirical and optimal management of severe attacks is still lacking.

Fluttering Thrombus in Patent Foramen Ovale With Paradoxical and Cerebral Embolism

A 56-year-old man was admitted with progressive dyspnea that started suddenly the previous day. Physical examination was normal. Pulmonary embolism was diagnosed on a computed tomography scan of the chest, which showed intraluminal filling defects in both pulmonary arteries and in the branches for the right lower lobe; it also showed a clot in …

Myasthenia Gravis following Low-Osmolality Iodinated Contrast Media

We describe the case of 79-year-old man admitted to our general hospital for a 6-week history of progressive dysphagia to solids and liquids associated with weight loss. To reach a diagnosis a total body CT scan with low-osmolality iodinate contrast agent was performed. Two hours later the patient developed an acute respiratory failure requiring orotracheal intubation and mechanical ventilation. The laboratory and neurological tests allow formulating the diagnosis of myasthenia gravis. In literature, other three case reports have associated myasthenic crisis with exposure to low-osmolality contrast media. This suggests being careful in administering low-osmolality contrast media in myasthenic patients.

From the Veins to the Heart: A Rare Cause of Varicose Veins

The presence of pulsating varicous veins is an uncommon finding, generically attributed to right heart failure. The precise causes of this phenomenon have been poorly defined in the literature. The finding of this infrequent condition is important because it may be a sign of major diseases, often not known. Here we described a 75-year-old woman presented to the Angiology Unit for the presence of bilateral pulsatile swelling in her groin and along both lower limbs. A bedside ultrasound examination showed an arterial like pulsating flow both in the superficial and in the deep veins of the lower limbs due to a severe tricuspid regurgitation not previously known.

Are too many inferior vena cava filters used? Controversial evidences in different clinical settings: a narrative review

The use of inferior vena cava filters to prevent pulmonary embolism is increasing mainly because of indications that appear to be unclearly codified and recommended. The evidence supporting this approach is often heterogeneous, and mainly based on observational studies and consensus opinions, while the insertion of an IVC filter exposes patients to the risk of complications and increases health care costs. Thus, several proposed indications for an IVC filter placement remain controversial. We attempt to review the proof on the efficacy and safety of IVC filters in several “special” clinical settings, and assess the robustness of the available evidence for any specific indication to place an IVC filter.

CAN we say that modern radiologic contrast agents are safe in myasthenia gravis?: Letters to the Editor

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A Rare Case of Aortic Valve Thrombosis in Patient with Idiopathic Hypereosinophilic Syndrome.

Idiopathic hypereosinophilic syndrome (HES) is characterized by persistent eosinophilia and eosinophil-mediated organ-system damage. Cardiac thrombosis and thromboembolic complications represent common causes of morbidity and mortality and usually involve cardiac ventricles or mitral and prosthetic valves, while the involvement of the aortic valve is extremely rare in HES. Here we report peculiar multimodality images of an atypical case of extended thrombosis of the aortic valve, complicated by myocardial ischemia and asymptomatic cerebral ischemia, likely due to thrombus embolization, occurring in a 48-year-old man with HES. Prompt anticoagulant and steroid therapy lead to rapid and complete resolution of the thrombotic lesions, allowing preserving the native valve and preventing further embolic events.

Response to Letter Regarding Article “Fluttering Thrombus in Patent Foramen Ovale With Paradoxycal and Cerebral Embolism”

We thank Myers and Kalangos for their interest in our case, and we would like to reply to the issue they raised in their letter. As they report in their article, the management and treatment options of impending paradoxycal embolism are a subject of debate.1 For the American Heart Association, in its statement for the management of …

Intramural hematoma of the esophagus in a woman with chronic renal failure and prophylactic heparin therapy

CASE REPORT An 86 year-old woman experienced chest pain, dyspnea, and dysphagia two days following orthopaedic surgery for a bimalleolus fracture of the right ankle. The patient was on prophylactic low weight molecular heparin and was affected by chronic renal failure. The chest computed tomography (CT) ruled out a pulmonary embolism but showed an intramural esophageal mass involving the entire esophagus. The lesion exhibited a blood like CT attenuation content that did not enhance after contrast administration. The esophagogram with gastrografin did not reveal filling defects nor communications between lumen and mucosa. Magnetic resonance confirmed CT results and excluded an aortic dissection. All radiological findings were suggestive of intramural hematoma of the esophagus. DISCUSSION AND CONCLUSIONS Intramural hematoma of the esophagus (IHE) is part of the spectrum of esophageal injuries that includes Mallory-Weiss tear and Boerhaave’s syndrome. IHE is the result of a hemorrhage within the submucosal layer. Predisposing conditions are abnormal hemostasis, traumatic events, aortic diseases. It can also occur spontaneously (idiopathic). Treatment should be conservative and includes risk factors withdrawal. The hematoma usually resolves in 1 to 3 weeks.

Right Atrial Mass in a Patient With T-Cell Chronic Lymphocytic Leukemia An Unusual Mechanism of Thrombus Formation

71-year-old woman with refractory T-cell (CD4) chronic lymphocytic leukemia who had been treated with chemotherapy and leukapheresis with poor control of leucocytosis was admitted because of fever, cough, and chest pain. A chest x-ray showed a right basal pneumonia, and the cytological examination of sputum showed Aspergillus fumigatus (Figure 1). The ECG at admission showed a firstdegree atrioventricular block (Figure 2A). The patient was started on broad-spectrum antibiotics and liposomal amphotericin B. Shortly before admission, because of sinus tachycardia, an ECG with Holter monitoring was performed; it showed a run of supraventricular tachycardia (Figure 2B). Therefore, while in the hospital, the patient underwent cardiac evaluation. Two-dimensional echocardiography showed a round (2016 mm) floating mass in the right atrium, close to the superior caval vein (Figure 3A, Movie). A thrombus near the distal tip of the central venous catheter was suspected, and the patient was started on nadroparin 6000 U injected subcutaneously twice daily. At the time of echocardiography, blood counts showed severe leucocytosis (white blood cell count, 396.100/L; lymphocytes, 384.940/L). After 2 weeks of therapy with low-molecular-weight heparin, the patient underwent transesophageal echocardiography, which did not show any reduction of the atrial mass and excluded any relation to the central venous catheter (Figure 3B). A cardiac computed tomography scan was performed, which showed an 8-mm defect in the right atrium with irregular shape and contrast enhancement (Figure 3C). The patient died of multiorgan failure 52 days after admission. At autopsy, a multiorgan extensive leukemic infiltration was detected. The atrial mass, located at the junction of the inferior caval vein with the atrium and attached to the crista dividens, measured 201815 mm (figure 3D). Histologically, we found an infiltration of the atrial wall by clusters of cells that reached and disrupted the endocardial atrial surface, providing a likely cause for the stratified thrombotic apposition (Figure 4A through 4D). The clustered cells were CD45-positive (Figure 4C) and CD3-positive (Figure 4D) T-lymphocytes and were confined to the peduncle.A 71-year-old woman with refractory T-cell (CD4+) chronic lymphocytic leukemia who had been treated with chemotherapy and leukapheresis with poor control of leucocytosis was admitted because of fever, cough, and chest pain. A chest x-ray showed a right basal pneumonia, and the cytological examination of sputum showed Aspergillus fumigatus (Figure 1). The ECG at admission showed a first-degree atrioventricular block (Figure 2A). The patient was started on broad-spectrum antibiotics and liposomal amphotericin B. Shortly before admission, because of sinus tachycardia, an ECG with Holter monitoring was performed; it showed a run of supraventricular tachycardia (Figure 2B). Therefore, while in the hospital, the patient underwent cardiac evaluation. Two-dimensional echocardiography showed a round (20×16 mm) floating mass …