Probucol administration of 4 weeks produced torsades de pointes associated with exacerbated QT interval prolongation in a 36-year-old woman with Romano-Ward syndrome. With discontinuance of probucol, the QT interval corrected for rate shortened from 620 msec to 500 msec and ventricular ectopic beats disappeared completely. Although probucol is known to prolong the QT interval, associated ventricular tachyarrhythmia has not been reported in humans as yet. This case suggests that one should be very careful in the administration of probucol to patients with long baseline QT intervals.

Adult↗

PubMedDate not supplied

A case of Rendu-Osler-Weber disease with cerebral hemangioma, multiple pulmonary arteriovenous fistulas and hepatic arteriovenous fistula.

A 46-year-old woman was admitted to the hospital because of dyspnea and palpitation. Physical examination showed severe anemia and cardiac distress. Telangiectases were found in the conjunctivas, lips and oral mucosa. Chest roentogenogram showed an enlarged cardiac silhoutte and multiple coin lesions in both lower lung fields. We diagnosed her as Rendu-Osler-Weber disease (ROW). The angiographic studies showed hemangioma in the foramen of Monro, bilateral multiple pulmonary arteriovenous fistulas, and hepatic arteriovenous fistula with a large distortion of the proper hepatic artery. Although associated vascular dysplasias have been described in patients with ROW, there is no reports on ROW in which multiple vascular abnormalities concomitantly existed such as the present case. In 163 detailed cases of ROW in Japan, the frequency of vascular lesions was summarized, and this result showed that the vascular lesions of the lung were more frequent than that of the liver or brain, in comparison with countries in Europe and America. It is very important for diagnosis and its prognosis to perform a detailed angiographic examinations and to demonstrate the precise sites of the vascular dysplasias in patients with ROW.

Abnormalities, Multiple↗

PubMedDate not supplied

Familial amyloidotic polyneuropathy in Hokkaido: a case report.

A 54-year-old man began to feel numbness in his hands at the age of 42 (1975). His condition became progressively worse accompanied by muscle weakness of the lower limbs and glove and stocking paresthesia of the extremities. The patient was admitted to our hospital on March 23, 1987. Neurological examination revealed distal dominant muscle weakness, sensory disturbance and areflexia. Electrocardiogram and ultrasound-cardiogram strongly suggested cardiomyopathy. A biopsy of the rectal wall and the cardiac muscle revealed amyloid deposits. The patient, his elder brother and one of his daughters had abnormal serum transthyretin (TTR, a protein referred as prealbumin). Therefore, the diagnosis of familial amyloidotic polyneuropathy (FAP) was confirmed. The patients's brother and daughter mentioned above, however, had no abnormal findings on physical examination and they were thus considered to be asymptomatic carriers. There may be more cases of asymptomatic carriers, if examination of abnormal TTR is more frequently analyzed.

Amyloidosis↗

PubMedDate not supplied

Effect of bunazosin, a selective alpha 1-adrenoceptor blocker, on ischemic myocardium in perfused rat heart.

The effects of bunazosin on the ischemic myocardium were investigated in isolated, perfused working rat hearts. Ischemia decreased the pressure-rate product and tissue adenosine triphosphate and creatine phosphate levels. Reperfusion did not restore the pressure-rate product nor the adenosine triphosphate levels completely. Bunazosin (5 x 10(-7) and 5 x 10(-6) mol/L) preserved the levels of adenosine triphosphate and creatine phosphate after 20 minutes of ischemia and increased the extent of recovery of the pressure-rate product during reperfusion. The results suggest that bunazosin protects the myocardium against ischemic damage.

Adenosine Triphosphate↗