[Osler-Weber-Rendu disease. A case report]. / Enfermedad de Osler-Weber-Rendu presentacion de un caso.

Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia is an arteriovenous malformation of different organs and systems, due to fibrovascular displasia. The knowledge of its molecular genetic basis has developed in the last few years. Generally, these patients are evaluated each time they have hemorrhagic manifestations as isolated events, rather than integrating these events into a single disorder. This is the case of a patient who had several skin and visceral manifestations years before the diagnosis was made.

[Sjögren syndrome associated with renal tubular acidosis type I]. / Un caso de Síndrome de Sjögren primario complicado con acidosis tubular renal tipo 1.

Primary Sjögren's Syndrome complicated with a renal tubular acidosis type 1 and hypocalcemic paralysis, as the principal clinical manifestation, is uncommon. Although the initial manifestations of the nephropathy are not well understood, it is believed that the invasion of mononuclear cells and the high level of circulating antibodies, play an important role in the pathogenesis of the disease. We present a patient with hypocalcemic paralysis as an initial manifestation of a latent Sjögren's disease. The glandular biopsy was normal, suggesting a mayor participation of an immunological humoral factor in the renal lesion.

Un caso de Síndrome de Sjõgren primario complicado con acidosis tubular renal tipo 1 / Sjõgren syndrome associated with renal tubular acidosis type I

Primary Sjõgren's Syndrome complicated with a renal tubular acidosis type 1 and hypocalcemic paralysis, as the principal clinical manifestation, is uncommon. Although the initial manifestations of the nephropathy are not well understood, it is believed that the invasion of mononuclear cells and the high level of circulating antibodies, play an important role in the pathogenesis of the disease. We present a patient with hypocalcemic paralysis as an initial manifestation of a latent Sjõgren's disease. The glandular biopsy was normal, suggesting a mayor participation of an immunological humoral factor in the renal lesion.

Follow up of young people with high casual blood pressure.

High blood pressure (BP), often borderline hypertension, can be also found in adolescents. In these subjects the haemodynamic pattern, high cardiac output and normal vascular resistance, differs from that of older hypertensives. Although the risk for hypertension is higher in this group than in the general population, only a minority of them will develop sustained hypertension later in life. They can therefore be viewed as an enriched pool of future hypertensives but not as true prehypertensives. The aim of this longitudinal study was to analyse the relation between casual BP measured in high school students and in the same subjects 3 years later. In 1990, an extensive study on BP was carried out in 1062 high school students aged 18 years. Sitting BP, heart rate, weight, and body mass index (BMI) were measured in each subject. After 3 years, the 50 subjects with the highest BP level recorded in 1990 were recalled. Forty-five subjects (90%, 30 males and 15 females) agreed to undergo a second examination. They were seen as outpatients in the Hypertension Centre of our institute. BP was measured with a mercury sphygmomanometer after a 10 min rest three times in 5 min. Systolic and diastolic BP were significantly reduced after 3 years (137 +/- 13 vs 132 +/- 10 P = 0.002; 92 +/- 4 vs 85 +/- 6 P = 0.0001, mm Hg). By means of a multiple regression test, including parameters recorded in 1990, systolic blood pressure (SBP) (R = 0.53 Slg F = 0.0002) and diastolic blood pressure (DBP) (R = 0.60 Slg F = 0.0001) were shown as the main determinants of SBP, while DBP was related only to previous BMI (R = 0.37 Slg F = 0.01). The reduction of both SBP and DBP after 3 years could be explained either by a true, spontaneous decrease of BP or as a consequence of different environmental conditions during the second examination (more prolonged resting time, repeated measurements). However, data of this study demonstrate that casual SBP and DBP are the main determinants of future SBP, thus confirming the prognostic value of casual BP measurement in young people. Moreover, our data emphasises the role of BMI as the main determinant of future high DBP.

Neither physical exercise nor alpha 1- and beta-adrenergic blockade affect plasma endothelin concentrations.

Endothelins (ET) are recently discovered vasoconstrictor agents released from endothelial cells and have been the object of intense investigation by researchers. Many of the factors that seem to influence the release of ET are modified by prolonged exercise. The purpose of this study was to investigate the effect of physical exercise on ET plasma concentrations and the effect of alpha- and beta-blockade on ET concentrations at rest and during exercise. Fifteen young volunteers (age 20-35 years) performed an exercise test on a bicycle ergometer. The starting workload of 50 W was increased by 30 W every 3 min until maximal heart rate was achieved; after a 2 min recovery period at 50 W the test continued for 15 min at 60% maximal work load. Blood samples were taken for ET determination before and after the test. After 1 week, the test was repeated. In the 2 days before either the first or the second test, each volunteer randomly received carvedilol (C) (25 mg), an alpha 1-adrenoceptor and beta-adrenoceptor blocker. There was no significant difference in ET concentrations after exercise with or without C administration (1.24 +/- 0.66, 1.42 +/- 0.83, 1.66 +/- 1.15, 1.61 +/- 0.87 pg/mL), showing that prolonged aerobic exercise does not affect plasma ET levels. Moreover, in our healthy young volunteers, blockade of alpha- and beta-adrenoceptors had no effect on ET levels at rest and after exercise.

Effects of chronic treatment with losartan on blood pressure, endothelin-like immunoreactivity and nitric oxide in normotensive rats.

BACKGROUND: Losartan is a new angiotensin II type 1 (AT1) receptor antagonist and an antihypertensive drug. Nitric oxide is a vasodilating agent and endothelins are powerful vasoconstrictors, both synthesized by and released from endothelial cells. Angiotensin II promotes the release of endothelins in cultured cells and this effect is prevented by losartan. Nitric oxide is also synthesized in the macula densa; therefore this substance may affect the regulation of renin excretion. OBJECTIVE: The aim of the present study was to evaluate the effects of losartan on blood pressure, endothelin-like immunoreactivity and nitric oxide in normotensive rats. MATERIALS AND METHODS: Male Wistar-Kyoto rats were divided into two groups. One group (n = 10) was treated with losartan at 10 mg/kg once a day by gavage for 4 weeks and a placebo group (n = 10) was given the same volume of water once a day by gavage. Blood pressure was measured weekly with a tail cuff and 24-h urine was collected at the beginning and at the end of the study. After 4 weeks all rats were killed and blood samples taken. Endothelin-like immunoreactivity was determined in plasma and urine using a 125I endothelin radioimmunuoassy kit. The stable metabolic products of nitric oxide, NO2- and NO3-, were measured in urine by the brucine method. RESULTS: After 4 weeks blood pressure was significantly lower in the losartan group (131 +/- 4 versus 118 +/- 6 mmHg, P = 0.001). Plasma endothelin-like immunoreactivity was similar in both groups while 24-h urinary endothelin-like immunoreactivity was significantly increased in the losartan group (29 +/- 25, 32 +/- 21, 43 +/- 19, 72 +/- 30 pg/24 h; F = 0.0003). NO2- and NO3- were unchanged in both groups. CONCLUSIONS: Our data show that chronic AT1 receptor blockade does not modify plasma endothelin-like immunoreactivity but increases urinary endothelin-like immunoreactivity. The significance of this finding remains obscure. It may represent a compensatory mechanism against the sustained vasodilation caused by losartan. Nitric oxide does not seem to affect the antihypertensive effect of losartan, since the urinary excretion of nitric oxide metabolites was unchanged.

Nifedipine reduces postexercise proteinuria in young volunteers.

Previous studies have demonstrated that it is possible to prevent postexercise proteinuria with angiotensin-converting enzyme inhibitors. To determine whether calcium antagonists have the same effect, 40 young healthy volunteers underwent maximal aerobic exercise with and without nifedipine 10 mg per os 1 h before the first or second trial. Urinary excretion of albumin (UAE), transferrin (UTE) and alpha 1-microglobulin (UME) were examined before and after each trial. UAE, UTE and UME were significantly increased after exercise. Nifedipine significantly decreased UAE (p = 0.001) and UTE (p = 0.02) after exercise, and slightly decreased the maximal work load and the basal excretion of albumin. UME was unchanged. Therefore, the results of this study demonstrate that nifedipine administration before exercise significantly reduces postexercise proteinuria.

Oscillatory potentials of the electroretinogram in hypertensive patients.

Because alteration of oscillatory potentials of the electroretinogram has been described in diabetic patients without signs of diabetic retinopathy as an early marker of changes in microcirculation, we studied the behavior of these potentials in patients with early-onset hypertension. Electroretinograms were recorded in 24 subjects with essential hypertension (blood pressure > 140/90 mm Hg) and in 9 age-matched normotensive control subjects (blood pressure < 140/90 mm Hg). Diabetes and ocular diseases were considered exclusion criteria. Sitting blood pressure was measured by a single investigator with a mercury sphygmomanometer after each subject had been at rest for 10 minutes. Funduscopic changes in all subjects did not exceed stage I World Health Organization classification. The oscillatory index was calculated by adding waves O1, O2, and O3 within the b wave of the electroretinogram. Statistical analysis was performed with Student's t test for paired and unpaired data and linear regression. The oscillatory index was significantly reduced in hypertensive patients compared with normotensive subjects. An inverse relationship was observed when systolic and diastolic blood pressures were plotted against the oscillatory index. In conclusion, our data demonstrate that the electrical activity of the retina is altered early in the course of hypertension and that the influence of systolic pressure on the oscillatory index is greater than that of diastolic pressure.

Metabolic effects of atenolol and doxazosin in healthy volunteers during prolonged physical exercise.

Nonselective beta-blockers may reduce exercise performance, not only through hemodynamic but also through metabolic effects. During prolonged physical exertion, lipolysis induced by plasma epinephrine occurs through beta-adrenoceptors of adipocytes. Therefore, beta-blockade may reduce release of free fatty acids (FFA) from adipocytes and consequently the energy supply for muscle cells. In this single-blind study, we compared the metabolic effects of atenolol with those of doxazosin, an alpha 1-blocker, during exercise in 26 young volunteers (age 20-35 years). All subjects performed an exercise test on a bicycle ergometer 5 h after consuming a standard breakfast. The starting workload of 50 W was increased by 30 W every 3 min until maximal heart rate (HR) was achieved; after a 2-min recovery period at 50 W the test was continued for 15 min at 60% maximal workload. Before and at the end of the test, blood samples were taken for glucose, lactate, and FFA determination. After 1 week, the test was repeated; the volunteers randomly received atenolol or doxazosin for 2 days before the second test. Exercise performance, plasma glucose, and lactate were not affected by either drug. The concentration of FFA was unchanged in subjects treated with doxazosin but was significantly reduced after the test in subjects treated with atenolol. Our data demonstrate that neither doxazosin nor atenolol impairs exercise performance in young volunteers. Atenolol reduces plasma FFA concentration possibly by inhibiting lipolysis. Doxazosin, in contrast, does not alter this parameter. Therefore, doxazosin may be a antihypertensive drug of potential benefit in treatment of hypertensive patients engaging in sports or undergoing a program of physical training.(ABSTRACT TRUNCATED AT 250 WORDS)

Nasal secretory IgA circadian rhythm: a single-dose suppression test.

The existence of a clear circadian rhythm in the local secretion of nasal IgA is well-known phenomenon which is confirmed in the present research. Since the temporal pattern of secretory IgA (SIgA) strictly parallels the ACTH-cortisol cycle, a dependence was hypothesized in previous research, but the suppression of adrenal rhythm by means of massive doses of exogenous corticosteroids showed no influence on the SIgA cycle. In the present research a more sophisticated method of corticoid-incretion suppression was used; the single-dose suppression test with dexamethasone, which influences only the impulsive phase of the rhythm. In this case too, however, no influence was demonstrated on nasal SIgA secretion.