Wiskott-Aldrich syndrome. A study of 6 cases with determination of the immunoglobulins A, D, G, M and ND.
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Biomedical subjects
Publications and source records attributed to G Berglund.
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A psychometric analysis of the Mental Adjustment to Cancer (MAC) scale was performed in a heterogeneous Swedish sample of cancer patients (n = 868). The homogeneity of the original subscales proved to be satisfactory (alpha coefficients 0.61-0.81). The sample was randomly split into two subgroups, and a factor analysis was carried out in one of them using the LISREL 8.20 procedure. This yielded four factors called 'Hopeless', 'Positive', 'Anxious' and 'Avoidant' including 28 of the 40 original items (alpha coefficients 0.58-0.81). The novel factor structure was cross-validated and confirmed in the second subgroup. In contrast to the original scale (one item), 'Avoidance', was indexed by three items. The distinction between mental adjustment and coping is discussed. It is concluded that both versions of the MAC scale are measures of mental adjustment including emotional reactions as well as coping.
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To explore the relationship between blood pressure on the one hand and body composition variables and fat cell size and number on the other, these factors were determined in a population sample (n = 120) of 49-year-old men with blood pressure (BP) ranging from very low to very high. BP increased linearly with body weight throughout the entire BP range, while body fat and fat cell size increased with increasing BP in the nonhypertensive but not in the hypertensive BP range. Body cell mass and fat cell number were unrelated to BP. Fat cell size (but not fat cell number) was positively correlated with most variables of glucose metabolism. It can be concluded that in normal men: the previously well-established relationship between body weight and BP is explained by an association between body fat and BP; BP is positively correlated with increased fat cell size, indicating hypertrophic obesity, while BP is unrelated to fat cell number; and increased fat cell size is associated with impaired glucose tolerance and hyperinsulinemia. The results point to the possibility that hypertrophic obesity and essential hypertension may be related to each other via common pathogenetic mechanisms.
The renal hemodynamic response to subpressor doses of angiotensin II (AII; 0.1 and 0.5 ng/min/kg) was investigated in untreated 49-year-old men (n = 50) representing a wide blood pressure range. Renal blood flow, renal vascular resistance (RVR), glomerular filtration rate (GFR), filtration fraction (FF), plasma renin activity (PRA), plasma AII, plasma aldosterone, and the urinary excretion of sodium and norepinephrine were studied. The higher the initial blood pressure the greater was the increase in RVR in response to AII infusion (p less than 0.002), indicating an increased renal vascular reactivity with increase in initial blood pressure. The AII infusion gave a significant rise in RVR in both the borderline and hypertensive group, but gave no increase in RVR in the normotensive group, implying an enhanced sensitivity of the renal vasculature in the borderline and hypertensive group. The increase in RVR was greater in the hypertensive than in the borderline group, i.e., the hypertensives had a steeper dose-response curve than the borderline group, which points to the presence of structural vascular changes in the renal vessels in the hypertensives. The increase in RVR in response to AII was positively correlated to sodium intake and plasma aldosterone concentration, indicating that these two factors might modulate the renal vascular reactivity. These factors could, however, only partly explain that RVR increased more the higher the initial blood pressure. Thus, the results indicate that there is an increased reactivity of the renal vascular bed to AII in essential hypertension. The increased reactivity seems to be mediated through an increased sensitivity of the renal vasculature to AII in mild essential hypertension and also through the presence of structural vascular changes in established hypertension. These factors may lead to a reduced excretion of sodium and water and may therefore be of importance in the development and progression of essential hypertension.
To study left ventricular (LV) diastolic function of the heart in relation to blood pressure (BP) and other signs of hypertensive cardiac and peripheral vascular changes, isovolumic relaxation time and early diastolic filling were determined in four BP groups of untreated 49-year-old men: normotensive subjects (n = 20), men with borderline hypertension (n = 30), mild hypertension (n = 45), and moderate to severe hypertension (n = 24). Isovolumic relaxation time, measured as the distance between aortic closure (A2, phonocardiography) and mitral valve opening (echocardiography), and early diastolic filling, measured as the distance between mitral valve opening and the O point of the apexcardiogram, tended to increase with BP level, and the total interval from aortic closure to the O point (A2O interval) was significantly prolonged in the two groups with mild and moderate to severe hypertension. A prolonged A2O interval (greater than or equal to 117% of expected value at observed heart rate) was seen in several hypertensives, who had no obvious increase in LV wall thickness on M mode echocardiography. This suggests that a prolonged LV relaxation time may be an early sign of cardiac involvement in primary hypertension. In the group with moderate to severe hypertension, an increase in LV wall thickness was seen together with an increase in resistance at maximal dilation in the calf. This supports the theory that when changes in cardiac structure develop they occur in parallel with structural changes also in the periphery.
Knowledge of the benefit of antihypertensive treatment relies on several large-scale trials, such as the Veterans Administration (VA) Studies, the Hypertension Detection and Follow-up Program (HDFP), the Australian National Blood Pressure Study (ANBP Study), and the Gothenburg Primary Preventive (GPP) study. These studies have shown that antihypertensive drugs decrease the incidence of directly hypertension-related complications like stroke, cardiac decompensation, and accelerating hypertension. It is less clear whether they influence the incidence of coronary events. In the HDFP, the ANBP Study, and the GPP study, the participants were recruited by screening large proportions of the total population. The proportion of the total screened population who met the inclusion criteria and hence were included was 3.3% in the ANBP, 6.9% in the HDFP, and 13.6% in the GPP trial. In most Western countries a substantially larger proportion of the total population is already being treated for hypertension. It is therefore not possible to use the results of these studies to justify treatment of larger segments of the population than we treat today. This is especially important because the milder the hypertension, the less effective the treatment is; hence, more patients will be given treatment without deriving benefit from it.
To test the usefulness of noninvasive ambulatory 24-hour blood pressure recording, the Del Mar Avionics system was used in a double-blind clinical trial in which 31 hypertensive patients were randomly allocated to receive placebo or pafenolol (25 mg or 50 mg), a novel, long-acting, highly selective beta-blocker, once daily. The results of 24-hour blood pressure and heart rate recording after 4 weeks of treatment were compared with a previous 24-hour recording performed after a 4-week placebo run-in period using the 3-hour mean of recordings performed every 7.5 minutes both day and night. Furthermore, 24-hour means were analyzed in each patient before and after 4 weeks. The system was easy to use and, judging from two placebo periods in the same patients, the reproducibility was good. The 24-hour blood pressure and heart rate recordings showed a clear dose-response relationship for pafenolol that could not be detected by ordinary casual readings. A daily dose of 25 mg of pafenolol significantly reduced blood pressure during the 9 hours after tablet intake (p less than 0.01), while 50 mg per day of pafenolol resulted in a significant reduction throughout the 24-hour period (p less than 0.01). The same pattern was seen for heart rate, which indicates a greater degree of beta-blockade during treatment with the higher dose. These results indicate that the tested noninvasive equipment is a useful tool for monitoring ambulatory 24-hour blood pressure. It gives important information impossible to obtain from single casual readings. This noninvasive method should be further evaluated to define its place in clinical work and as a research tool.
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