Solid tumor screening recommendations in trisomy 18

The purpose of this study was to determine whether trisomy 18 patients are at an increased risk of tumor development and require formal tumor screening recommendations. A literature search of trisomy 18 patients with reports of tumors or malignancies, and compilation of all previously reported as well as new unreported cases was performed. 67 patients with trisomy 18 were found to have documented malignancies. 44 patients had hepatoblastomas, 21 patients had Wilms tumors, one patient had a functional neurogenic neoplasia, and one patient had Hodgkins lymphoma. The increasing numbers of reported malignancies in patients with trisomy 18 supports the indication for an early screening process. Specific screening recommendations are outlined consisting of imaging exams and laboratory values performed at specific intervals.     

Comparison of docetaxel and docetaxel-irinotecan combination as second-line chemotherapy in advanced non-small-cell lung cancer: a randomized phase II trial.

BACKGROUND: The aim of this study was to evaluate whether docetaxel (taxotere) treatment with or without irinotecan improved patient outcomes with similar toxicity in recurrent non-small-cell lung cancer (NSCLC). PATIENTS AND METHODS: Patients with recurrent platinum-refractory NSCLC with Eastern Cooperative Oncology Group performance status of 0-2 were randomized to either docetaxel 30 mg/m(2) and irinotecan 60 mg/m(2) (days 1 and 8) or docetaxel 75 mg/m(2) (day 1), both administered every 3 weeks. RESULTS: A total of 130 patients were randomized. The response rate (RR) (20% versus 14%), overall survival (6.5 months versus 6.4 months) and 1-year survival (37% versus 34%) were similar in the combination and docetaxel arms, respectively. The combination arm demonstrated a longer time to tumor progression (TTP) (5.6 versus 4.8 months; P=0.065). Grade 3-4 neutropenia and anemia were similar in the combination and docetaxel arms. Grades 3-4 non-hematological toxicity (except diarrhea) was mild and was similar in the two groups. Grade 3-4 thrombocytopenia (17% versus 6%; P=0.04) and diarrhea (12% versus 3%; P=0.05) occurred more frequently in the combination arm. CONCLUSIONS: The administration of irinotecan with docetaxel in platinum-refractory NSCLC prolonged TTP, but did not improve significantly RR, median survival or 1-year survival. Second-line docetaxel monotherapy offers significant and reproducible efficacy in platinum-refractory NSCLC.     

Extracardiac applications of MR blood pool contrast agent in children

Magnetic resonance (MR) angiography has significantly reduced the need for diagnostic conventional angiography and is preferred over CT angiography in children because of its lack of ionizing radiation. The availability of gadofosveset trisodium (the only clinically approved blood pool MR contrast agent) has led to an increase in applications of MR for vascular imaging and an improvement in diagnostic quality of MR angiography. Gadofosveset is a gadolinium-based contrast agent that binds reversibly to albumin, resulting in increased paramagnetic effect and longer intravascular residence. This allows for high-resolution arterial and venous MR angiography, assessment of flow characteristics of vascular malformations, dynamic vascular imaging, and multi-station imaging with a single low-dose gadolinium contrast injection. The purpose of this pictorial essay is to facilitate understanding of the kinetics and safety profile of gadofosveset trisodium, discuss technical aspects of imaging, and illustrate advantages and extracardiac applications in pediatric body imaging.     

Atrial High-Rate Episodes in Patients with Devices Without a History of Atrial Fibrillation: a Systematic Review and Meta-analysis

Cardiovascular Drugs and Therapy - Atrial high-rate episodes (AHREs) recorded with cardiac implantable electronic devices (CIEDs) have been associated with the development of clinical atrial...     

Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia

Although the indications for transfusions in sickle cell syndromes are well listed, and chronic transfusion has become practicable since the recent advances in chelation therapy have essentially eliminated the risk of secondary iron overload, multi-transfused, non-compliant to long-term chelation therapy patients confront the complication of iron overload and secondary hemosiderosis. In thalassemia major patients, combined therapy with desferrioxamine and deferiprone has maximized tissue iron removal and may reduce the overall occurrence of hemosiderotic heart failure. Despite this, safety and contradictions of chelating agents are still controversial. The aim of this report is to present the results of this combination in a long-term transfused sickle beta-thalassemic patient suffering from severe heart failure and liver dysfunction.     

The management of atrial fibrillation in heart failure: an expert panel consensus

Heart Failure Reviews - Heart failure (HF) and atrial fibrillation (AF) often coexist, being closely interrelated as the one increases the prevalence and incidence and worsens the prognosis of the...     

Haemodynamic factors and the important role of local low static pressure in coronary wall thickening

BACKGROUND/STUDY OBJECTIVES: The purpose of our study was to investigate the possible correlation between blood flow physical parameters and the wall thickening in typical human coronary arteries. METHODS: Digitized images of seven transparent arterial segments prepared post-mortem were adopted from a previous study in order to extract the geometry for numerical analysis. Using the exterior outline, reconstructed forms of the vessel geometries were used for subsequent computational fluid dynamic analysis. Data was input to a pre-processing code for unstructured mesh generation. The flow was assumed to be two-dimensional, steady, laminar with parabolic inlet velocity profile. The vessel walls were assumed to be smooth, inelastic and impermeable. Non-Newtonian power law was applied to model blood rheology. The arterial wall thickening was measured and correlated to the wall shear stress, static pressure, molecular viscosity, and near wall blood flow velocity. RESULTS: Wall shear stress, static pressure and near wall velocity magnitude exhibit negative correlation to wall thickening, while molecular viscosity exhibits positive correlation to wall thickening. CONCLUSION: There is a strong correlation between the development of vessel wall thickening and the blood flow physical parameters. Amongst these parameters the role of local low wall static pressure is predominant.     

The pathophysiological relationship and clinical significance of left atrial function and left ventricular diastolic dysfunction in β‐thalassemia major

Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in β‐thalassemia major. We investigated the mechanical and endocrine function of the left atrium and ventricle to identify early signs of dysfunction. We studied 90 patients (mean age: 29 ± 11 years) with β‐thalassemia and normal left ventricular function and 90 age and sex‐matched healthy controls. Patients and controls underwent a thorough cardiac echocardiographic study and measurements of the b‐type (NT‐proBNP) and atrial natriuretic peptides (proANP). Patients underwent 24‐hr Holter recordings for arrhythmia monitoring. In the patient group, atria were affected early during the course of the disease, prior to diastolic and systolic left ventricular dysfunction. The E/E′ratio (E Doppler mitral fast inflow to the corresponding tissue Doppler E) continually increased with age (P < 0.05) and reached levels indicating left ventricular diastolic dysfunction (E/E′ > 15) in the third decade whereas indexes of active and passive atrial function decreased gradually throughout life. In controls, the E/E′ ratio continually increased with age but with later (fifth decade) appearance of diastolic dysfunction and a compensatory increase in atrial active function. Both natriuretic peptides were significantly increased in patients compared to controls (558 ± 141 and 2,580 ± 1,830 fmol/mL for NT‐proBNP and proANP versus 332 ± 106 and 1,331 ± 1,134 fmol/mL, respectively). Atrial fibrillation was found in a subgroup of 23 (26%) patients, older in age with mild diastolic function and enlarged, depressed atria. In conclusion, atrial mechanical depression seems to be a very early sign of cardiac damage. It may become echocardiographically evident even before diastolic and systolic dysfunction and is associated to supraventricular arrhythmias. Am. J. Hematol. 89:13–18, 2014. © 2013 Wiley Periodicals, Inc.