A distinct cardiac disorder linked to chromosome 1q42-q43 causes exercise-induced polymorphic ventricular tachycardia in structurally normal hearts and is highly malignant. Delayed clinical manifestation necessitates repeated exercise electrocardiography to assure diagnosis in young individuals of the families.

Summary:Transplantation of solid organs including heart, kidney, and liver is associated with rapid bone loss and increased rate of fracture; data on bone marrow transplantation recipients (BMT) are scarce. The purpose of the present study was to examine the magnitude, timing, and mechanism of bone loss following allogeneic BMT, and to study whether bone loss can be prevented by calcium with or without calcitonin. Sixty-nine patients undergoing allogeneic BMT for malignant blood diseases were enrolled into the study. Forty-four (22 women, 22 men) completed 6 months, and 36 patients 1 year follow-up. They were randomized to receive either no additional treatment (n ‫؍‬ 22), or oral calcium 1 g twice daily for 12 months (n ‫؍‬ 12) or the same dose of calcium plus intranasal calcitonin 400 IU/day for the first month and then 200 IU/day for 11 months (n ‫؍‬ 10). Bone mineral density (BMD) at the lumbar spine and three femoral sites (femoral neck, trochanter, Ward's triangle) was measured by dual-energy X-ray absorptiometry (DXA). Bone turnover rate was followed with markers of bone formation and resorption (serum bone-specific alkaline phosphatase (B-ALP), type I procollagen carboxyterminal (PICP) and aminoterminal propeptide (PINP), serum type I collagen carboxyterminal telopeptide (ICTP)). Serum testosterone was assayed in men. Calcium with or without calcitonin had no effect on bone loss or bone markers; consequently the three study groups were combined. During the first 6 post-transplant months BMD decreased by 5.7% in the lumbar spine and by 6.9% to 8.7% in the three femoral sites (P Ͻ 0.0001 for all); no significant further decline occured between 6 and 12 months. Four out of 25 assessable patients experienced vertebral compression fractures. Markers of bone formation reduced: B-ALP by 20% at 3 weeks (P ‫؍‬ 0.027), PICP by 40% (P Ͻ 0.0001) and PINP by 63% at 6 weeks (P Ͻ 0.0001), with a return to baseline by 6 months. The marker of bone resorption, serum ICTP was above normal throughout the whole observation period, with a peak at 6 weeks (77% above baseline, P Ͻ 0.0001). In male patients serum testosterone decreased reaching a nadir (57% below baseline) at 6 weeks (P ‫؍‬ 0.0003). In conclusion, significant bone loss occurs after BMT. It results from imbalance between reduced bone formation and increased bone resorption; hypogonadism may be a contributing factor in men. Bone loss can not be prevented by calcium with or without calcitonin.

All hemodynamically stable patients with chronic pancreatitis and bleeding pseudoaneurysms should undergo prompt initial angiographic evaluation and embolization if possible. Repeated angioembolization is feasible in patients with recurrent bleeding, whether initially embolized or operated. Patients with unsuccessful embolization should undergo emergency hemostatic surgery with ligation of the bleeding vessel in the head of the pancreas and distal resection in patients bleeding from the splenic artery or its branch. The combination of angioembolization and later endoscopic drainage of the pseudocyst via endoscopic retrograde cholangiopancreatography (ERCP) is effective in the majority of the cases of pseudoaneurysms in chronic pancreatitis.

A cross-sectional study of 351 healthy Finnish women aged 20-76 years was done to establish reference values of bone mineral density (BMD) using dual-energy X-ray absorptiometry (DEXA). The effects of age and of several physical and lifestyle factors on BMD of the lumbar spine and proximal femur (femoral neck, trochanter, and Ward's triangle area) were investigated. Altogether 58 women were excluded from the final analysis due to significant spinal osteoarthritis or other diseases or drugs known to influence calcium or bone metabolism. The precision of the method was 0.9, 1.2, 2.7, and 2.4% in the lumbar, femoral neck, Ward's triangle and trochanter area, respectively. Lumbar BMD was increased by 30% (P less than 0.001) in 15 patients with osteoarthritis (21% of women 50 years or older), but it was apparently unaffected in 5 cases with aortic calcification. Except for the trochanter area, BMD diminished along with age, and this was significant after the menopause. The peak of mean BMD was observed at the age of 31-35 years in the spine and at the age of 20-25 years in the femoral neck and Ward's triangle. BMD was in a positive relationship to weight both in premenopausal and postmenopausal women and to the use of oral contraceptives in premenopausal women and to that of estrogen replacement therapy in postmenopausal women. Labors and pregnancies had a weak positive effect on BMD in premenopausal women. As compared with nonusers premenopausal women who had used alcohol showed a slightly decreased BMD of Ward's triangle. In postmenopausal women there was a positive correlation between alcohol intake and BMD.

To identify factors predicting aortic stiffness, we studied the modulus of elasticity of the thoracic aorta in relation to sex, obesity, blood pressure, physical activity, smoking, ethanol consumption, salt intake, and serum lipid and insulin levels in 55 healthy people born in 1954. A transverse cine magnetic resonance image of the thoracic aorta was made, and the modulus of elasticity was determined as brachial artery cuff pulse pressure/aortic strain, where strain was determined as the ratio of pulsatile aortic luminal area change to the diastolic luminal area. The average of measurements made in the ascending and descending aorta was used as the elastic modulus of the thoracic aorta. Habitual physical activity, smoking, and alcohol use were quantified by 2-month prospective daily recording and salt intake by 7-day food records. The aortic elastic modulus ranged from 100 to 2091 10 3 dyne/cm 2 (median, 390 10 3 dyne/cm 2 ). In multiple regres-T he stiffness of the thoracic aorta influences aortic conduit function, contributes to blood pressure and left ventricular load, and may also modify the aortocoronary blood flow. 13 People with coronary artery disease have abnormally rigid aortas, 3 " 6 and noninvasive measurement of aortic distensibility has been considered for a targeted screening of coronary atheroma.7 For these reasons it has become timely to know what factors modify aortic pulsatility and in what way. It is generally agreed that the stiffness of the aorta increases with age and in hypertension,'-2 -6 -10 but knowledge of other potential predictors is insufficient and partly contradictory.6 ' 813 Concerning the relation of aortic stiffness to blood cholesterol level, some reports show a positive association, 1112 others a negative one, 613 and the rest no association. 810 Although part of these differences may be attributed to the modifying effect of age, 11 -13 confusing contrasts between the data 6 -12 still remain.The purpose of this study was to examine the predictors of aortic stiffness in a sample of the general adult population homogeneous for age and free of significant cardiovascular disease. In principle, aortic stiffness can be assessed noninvasively either by studying the relation between pulsatile changes in blood pressure and aortic luminal size or more indirectly by measuring the pulse wave velocity through the thoracoabdominal aorta. Key Words • aortic stiffness • magnetic resonance imaging • lipids • insulin • physical activity • sodium intake this study, we used cine magnetic resonance imaging (MRI) to measure the systolic and diastolic crosssectional areas of the ascending and descending thoracic aorta and calculated the modulus of elasticity 14 as an index of aortic stiffness. The present report describes the relations of aortic stiffness to those factors known to modify the risk of cardiovascular diseases, such as sex, obesity, blood lipid levels, physical activity, smoking, alcohol consumption, sodium intake, and serum insulin level. Methods Study PopulationThis wo...

Aortic dilatation and heart valve lesions are common in the Marfan syndrome but whether primary alterations occur in left ventricular (LV) function has not been studied hitherto. LV size, mass and systolic as well as diastolic function were studied by M-mode and Doppler echocardiography and cine magnetic resonance imaging in 22 Marfan children aged 3.0-15.4 years and in 22 age-matched healthy children. No child had significant valve disease. Heart rate and systolic blood pressure were comparable in the groups but diastolic blood pressure was higher in the controls (67 +/- 7 mmHg vs 62 +/- 8 mmHg, P = 0.030). No statistically significant differences were found in LV size, mass or systolic function. The Marfan children had slower LV peak diameter lengthening rates (106 +/- 27 mm.s-1 vs 132 +/- 29 mm.s-1, P = 0.004), prolonged relaxation times (155 +/- 22 ms vs 140 +/- 19 ms, P = 0.023), slower deceleration of the early transmitral velocity (580 +/- 144 cm.s-1 vs 720 +/- 160 cm.s-2, P = 0.006), and smaller early-to-late peak velocity ratios (1.99 +/- 0.40 vs 2.29 +/- 0.46, P = 0.031). These data indicate that LV early diastolic function (relaxation) is impaired in the Marfan syndrome. Weakened elastic recoil due to the underlying connective tissue abnormality may best explain this novel observation.

In this clinical series venous valvular function was better preserved after iliofemoral DVT when treated with catheter-directed thrombolysis.

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