No abstract

Erdheim-Chester disease is a rare form of non-Langerhans’ cell histiocytosis. This disorder is characterized by a bone involvement and several extraskeletal manifestations. We describe the case of a patient with a pleural and pericardial effusion leading to tamponade. Pathological examination of pericardium and mediastinal adenopathy was normal. The abdominal computed tomography scan showed two enlarged kidneys suggestive of Erdheim-Chester disease. Bone scan scintigraphy demonstrated symmetrical increased labeling of the long bones. The biopsy of perirenal soft tissue confirmed the diagnosis of Erdheim-Chester disease.

1 The electrophysiological effects of bepridil, its quaternary derivative, CERM 11888 (methylpyrrolidinium bromide) (both 2.5mgkg-1 i.v.) and those of verapamil and diltiazem (0.2mgkg-' i.v.) were studied in closed chest anaesthetized dogs at doses used in clinical studies. 2 The four drugs caused a bradycardia with the following order of potency: bepridil > CERM 11888 > diltiazem > verapamil. 3 All the compounds slowed conduction in the AV node, increased the refractory period (RP) and decreased Wenckebach rates with the following order: verapamil > diltiazem > bepridil > CERM 11888. 4 Verapamil and diltiazem did not affect conduction or the RP in atria while bepridil weakly slowed the former and markedly increased the latter. CERM 11888 caused a lengthening of RP but this was a delayed effect. 5 In the ventricle, bepridil and CERM 11888 caused a small increase in the QRS and a more pronounced increase in the RP. Both compounds increased QTc but did not modify HV. Verapamil and diltiazem had no significant effects at the ventricular level. 6 Our results confirm that the main sites of action of calcium antagonists are the SA and AV nodes. Bepridil has a broader spectrum of activity and also acts at the atrial and ventricular levels. A comparison of the effects of bepridil with those of its quaternary derivative suggests the involvement of an intracellular action in the electrophysiological effects of bepridil.

Despite successful PTCA in the acute phase of MI, an increase in end-systolic volume was observed at 6 months in 28% of patients. Tc-99m sestamibi ECG-gated SPECT performed 3 weeks after the acute phase could predict this enlargement with a high accuracy.

CMRI volumetric quantification of LV volumes and function is highly reproducible at different levels of experience, but not interchangeable with those obtained by ventriculography.