Idiopathic pure sudomotor failure: anhidrosis due to deficits in cholinergic transmission.

BACKGROUND: Acquired idiopathic generalized anhidrosis (AIGA) represents a heterogeneous clinical syndrome including sudomotor neuropathy and failure of the sweat glands. However, most AIGA cases comprise idiopathic pure sudomotor failure (IPSF), a distinct subgroup without sudomotor neuropathy or sweat gland failure. METHODS: Eight patients with IPSF (mean +/- SD age 20 +/- 5 years) were assessed by thermoregulatory and pilocarpine-induced sweating tests, as well as emotional sweating using sudorometer (4 cases), microneurography of skin sympathetic nerve activity (2 cases), and skin biopsies from the forearm or axilla (3 cases). RESULTS: Clinical features of IPSF comprise early onset; acute or sudden onset; concomitant sharp pain or cholinergic urticaria over the entire body; lack of autonomic dysfunction other than generalized anhidrosis; elevated serum IgE levels; and marked response to steroid. Sudomotor function testing revealed complete absence of thermoregulatory sweating, but well-preserved emotional sweating; pilocarpine did not induce sweating, and microneurography revealed that bursts of skin sympathetic nerve activity were not decreased; and skin biopsy displayed no morphologic abnormalities in sweat glands. The first two findings suggest lesions on the postsynaptic side of the nerve-sweat gland junction. CONCLUSION: The lesions in IPSF may be in the muscarinic cholinergic receptors of sweat glands. Allergic mechanisms are probably involved in its pathophysiology.

Ascending fractional pulse pressure closely relating to large artery function.

It is not known whether ascending fractional pulse pressure (PPf) is related to large artery function. This study was to evaluate whether PPf is associated with large artery function by augmentation index. A total of 190 subjects were enrolled (age range: 50 to 78 years) who had normal contractions, no local asynergy, and no history of myocardial infarction. The ascending aortic pressure was measured using a fluid-filled system. To quantify the relative magnitude of the pulsatile to mean artery pressure, we normalised the pulse pressure to the mean pressure and referred to this value as PPf and the association between the PPf and the augmentation index was investigated. Augmentation index showed significant associations with PPf by univariate analysis (R = 0.690, P < 0.001). The associations between PPf and the factors influencing large artery function were examined by multivariate analysis, and PPf revealed significant associations with age, mean aortic pressure, heart rate and type 2 diabetes (R(2) = 0.477, P < 0.001). The results were that ascending aortic PPf is closely associated with large artery function detected by augmentation index.

Low fractional diastolic pressure in the ascending aorta increased the risk of coronary heart disease.

Although the fractional pulse pressure in the ascending aorta is related to the occurrence of coronary heart disease (CHD) and restenosis after percutaneous transluminal coronary angioplasty, the relative values of diastolic pressure in the ascending aorta at the onset of CHD have not been reported. The purpose of this study was to evaluate whether the relative values of diastolic pressure are associated with the risk of CHD. For this study, we enrolled 406 patients with chest pain, normal contractions, no local asynergy, and no history of myocardial infarction. We measured the ascending aortic pressure using a fluid-filled system. To quantify the relative diastolic pressure, we normalised the diastolic pressures to the mean pressure and referred to this value as the fractional diastolic pressure (FDP). We investigated the association between the FDP and the risk of CHD. Low FDP in the ascending aorta was associated with an increased risk of CHD. The multiple-adjusted odds ratio of CHD was 1.68 (95% CI, 0.67-4.22) in FDP for the middle tertile of the level. The multiple-adjusted odds ratio of CHD was 2.20 (1.16-4.75) in FDP for the lowest tertile compared with the highest tertile. FDP was associated with the risk of CHD.

Pulsatility of brachial artery pressure is associated with an increased risk of coronary artery disease in men.

OBJECTIVES: The purpose of this study was to evaluate whether the pulsatility of brachial artery pressure is related to an increased risk of coronary artery disease (CAD). On the basis of vascular mechanics, we recently reported that relative pulse pressure can predict the occurrence of restenosis after percutaneous transluminal coronary angioplasty. We also hypothesized that relative pulse pressure of the brachial arterial pressure waveform is associated with an increased risk of CAD. DESIGN: A cross-sectional study. PATIENTS: We enrolled 172 men who had the same cardiac performances. MAIN OUTCOME MEASURES: We measured their brachial artery pressures with a sphygmomanometer. To quantify the relative magnitude of the pulsatility to diastolic pressure, we made use of the ratio of pulse pressure to diastolic pressure (PP/DP). We investigated the effects of the PP/DP in relation to the risk of CAD. RESULTS: PP/DP was associated with an increased risk of CAD. The prevalence rates of significant stenosis were 28.1% for the lowest, 43.1% for the middle and 49.1% for the highest tertile of PP/DP levels. The age-adjusted odds ratio of CAD was 2.23 (95% confidence interval 0.98-5.04) for the middle tertile of the PP/DP level and 2.55 (1.10-5.93) for the highest tertile compared with the lowest tertile. CONCLUSIONS: The pulsatility of the brachial artery pressure was associated with an increased risk of CAD.

Pulsatility of ascending aortic blood pressure waveform is associated with an increased risk of coronary heart disease.

BACKGROUND: Although it was reported that pulse pressure of the peripheral artery could differentiate patients with coronary heart disease (CHD) from those without CHD, it is not known whether pulsatility of the ascending aortic pressure waveform differentiates patients with CHD from those without CHD. The purpose of this study was to evaluate whether the pulsatility of ascending aortic pressure is associated with an increased risk of CHD. METHODS: For this study, we enrolled 293 subjects who had chest pain, normal contractions, no local asynergy, and no history of myocardial infarction. We measured the ascending aortic pressure using a fluid-filled system. To quantify the relative magnitude of the pulsatile to mean artery pressure, we normalized the pulse pressure to the mean pressure and referred to this value as the fractional pulse pressure (PPf). We investigated the association between the PPf and the risk of CHD. RESULTS: The PPf of the ascending aorta was associated with an increased risk of CHD. The multiple-adjusted odds ratio of CHD was 2.93 (95% CI, 1.44 to 5.94) for the middle tertile of the PPf level and was 3.93 (95% CI, 1.74 to 8.85) for the highest tertile compared with the lowest tertile. CONCLUSION: Ascending aortic pulsatility is related to an increased risk of CHD.

Pulsatility of ascending aortic pressure waveform is a powerful predictor of restenosis after percutaneous transluminal coronary angioplasty.

BACKGROUND: Because ascending aortic pressure has a greater effect on coronary perfusion during diastole than systole, we hypothesized that a high coronary diastolic-to-systolic pressure ratio prevents coronary lesions from restenosing after percutaneous transluminal coronary angioplasty (PTCA) and that ascending aortic pulsatility relative to mean pressure is higher in patients with restenosis than in those without restenosis. The purpose of this study was to evaluate prospectively whether the morphology of the ascending aortic pressure wave can be used to predict restenosis after PTCA. METHODS AND RESULTS: We measured the coronary artery diameter and the aortic pressure before PTCA. To quantify the relative magnitude of the pulsatile-to-mean aortic pressure, we normalized the pulse pressure to mean pressure and referred to this value as the fractional pulse pressure (PPf). We prospectively investigated the effect of PPf in relation to subsequent risk of restenosis after PTCA in patients with coronary artery disease. PPf was a powerful predictor of restenosis. Crude cumulative incidence rates of restenosis were 17.6% for the lowest, 33.3% for the middle, and 77. 8% for the highest tertile of PPf levels. After adjustments for age, smoking habits, systolic blood pressure, type 2 diabetes, hypercholesterolemia, old myocardial infarction, vessel location, vessel size, and sex, the odds ratio of restenosis was 33.5 (95% confidence interval, 2.04 to 550.6) for the highest tertile of the PPf level compared with the lowest tertile level. CONCLUSIONS: Pulsatility of the ascending aortic pressure is a predictive factor for restenosis after PTCA.

Dynamic left ventricular arterial pressure gradient and sick sinus syndrome with heterozygous Fabry's disease improved following implantation of a dual chamber pacemaker.

A 63-year-old woman with heterozygous Fabry's disease, sick sinus syndrome, sinus pauses, congestive heart failure, syncope, and a dynamic pressure gradient between the left ventricle and systemic arteries underwent implantation of a dual chamber (DDD) pacemaker. Following pacemaker implantation, the pressure gradient has been reduced and congestive heart failure controlled for the past 3 years.

[Influence of hypertensive left ventricular hypertrophy on detection of ischemic area with exercise thallium-201 myocardial scintigraphy].

Sixty-four patients with single left anterior descending artery disease having effort angina (group A: 40 patients without hypertension, group B: 10 patients with hypertrophic hypertension, group C: 14 patients with non-hypertrophic hypertension) were assessed the influence of hypertensive left ventricular (LV) hypertrophy on detection of ischemic area. The criterion of hypertrophy by two-dimensional echocardiography was > 12 mm in the wall thickness of interventricular septal or posterior wall. Population in Group B might show low detectability in ischemic area by 201Tl myocardial scintigraphy (positive thallium rate 60%, defect score 2.7 +/- 3.6), and high lung thallium uptake and high frequence of ECG positive among three groups. In semiquantitative analysis, the washout rate of the posterolateral wall and %RD (delayed %uptake-initial %uptake) of the septal wall in patients with Group B were lowest among three groups. However, the washout rate in the septal wall against the posterior wall, and the initial %uptake and the delayed %uptake of the septal wall were not significantly different among three groups. We could conclude that the decreased washout rate in nonischemic area with hypertensive LV hypertrophy might make the ischemic area masked.

Effects of parathyroid hormone-related protein on systemic and regional hemodynamics in conscious rats. A comparison with human parathyroid hormone.

PTHrp was discovered as a humoral hypercalcemic factor of malignancy and has been shown to bind the same receptor as PTH in rat bone cells, canine renal membranes, and rabbit renal microvessels. We investigated the global effect of human PTH(hPTH) and PTHrp on systemic and regional hemodynamics in conscious rats. The hypotensive response to PTHrp was more potent than that to hPTH. Although hPTH (15 micrograms/kg/min, i.v.) caused a significant increase in cardiac output, whereas PTHrp (5 micrograms/kg/min, i.v.) caused no change in cardiac output despite a similar hypotensive effect to hPTH, the effects of PTHrp and hPTHrp on regional hemodynamics were quite similar, and both peptides had a prominent vasodilatory effect on the coronary and hepatic arteries. Therefore, PTHrp appears to have an important role in blood pressure and regional hemodynamics as does hPTH.

[Long-term survival of a patient with pseudoaneurysm of the left ventricle following myocardial infarction].

A 76-year-old man had an extensively calcified left ventricular pseudoaneurysm which was a sequela of acute myocardial infarction suffered 22 years ago. He experienced acute anterolateral myocardial infarction in January 1964. In March 1964, the presence of a left ventricular aneurysm was suspected by chest radiography and fluoroscopy. He was, however, in good health since then. In April 1986, when he was admitted for treatment of acute bronchitis, a large calcified density was found in the left ventricular region on chest radiography. The electrocardiogram was compatible with an old anterolateral myocardial infarction. Two-dimensional echocardiography showed an immobile portion of the left ventricle which contained "moya-moya" (sluggish, smoky) echoes. A saccular aneurysm of the left ventricle was confirmed by radioisotope cardiac pool scans, reconstruction CT and left ventriculography. Due to the poor general condition of the patient, we followed his course without surgery. He died in October 1986. At autopsy, the pseudoaneurysm was markedly calcified, and its wall was adherent to the parietal pericardium. Histologically, the pseudoaneurysmal structure turned out to be a pseudoaneurysm since the saccular wall contained only scar tissue but no myocardial cells. This is a very rare case of a patient with a left ventricular pseudoaneurysm who survived for 22 years after its occurrence.