Acta medica Scandinavica最新文献

A simple and practical provocative test is needed for early asymptomatic pheochromocytoma, which is a major risk for patients with multiple endocrine neoplasia type 2 (MEN-2). We measured plasma catecholamines before and after submaximal exercise in 26 MEN-2 gene carriers, eight of whom with asymptomatic pheochromocytoma, nine with medullary thyroid carcinoma and 10 after uni- or bilateral adrenalectomy. Seventeen clinically healthy individuals and 11 patients with neurovegetative lability and symptoms mimicking pheochromocytoma served as controls. Plasma adrenaline, noradrenaline and dopamine increased after exercise except for adrenaline after bilateral adrenalectomy. The post-exercise levels of adrenaline and the adrenaline/dopamine ratio were significantly higher in the pheochromocytoma patients compared to the healthy controls and the patients with neurovegetative lability, while the patients with medullary thyroid carcinoma represented an intermediate group with a high probability of developing adrenal tumors. The present method is a physiological test with a high sensitivity and specificity. It is practical and well suited for repeated examinations and seems to be of value for the detection of early pheochromocytoma in MEN-2 patients. Furthermore, the test could be used in the differential diagnosis between pheochromocytoma and neurovegetative lability.

Hyperlipoproteinemia type V, with serum triglyceride concentrations of about 20 mmol/l, was detected in a pair of monozygotic, 40-year-old twin brothers. One of them had had recurrent attacks of pancreatitis, the other not. The endocrine and exocrine pancreatic functions were apparently normal, supporting that the pancreatitis was secondary to the hypertriglyceridemia. After successful lipid-lowering therapy the attacks of abdominal pain disappeared and remained absent during a 13-year follow-up period. The other twin died of ischemic heart disease nine years after the discovery of his lipid abnormality. The reduction of his lipid levels had been much less successful. His average "atherogenic index" (the ratio of cholesterol in low density to that in high density lipoproteins) was normal but increased to a very high value if cholesterol in very low density lipoproteins was also included together with the low density ones in the numerator. Lipoprotein particles modified in composition may have contributed to an increased uptake through a scavenger pathway and promoted atherosclerosis.

We report two studies, in which fibre/placebo tablets were added to a weight reduction regimen in the treatment of moderately obese women. In Study I, 60 females were treated for a two-month period with general dietary advice, providing a mean daily energy intake of 1,400 kcal. In addition, the fibre group received a 5 g dietary fibre supplement. In Study II, 45 females were treated for a three-month period with a similar programme, in which the recommended daily energy intake was 1,600 kcal and the fibre supplement 7 g/day. In both groups weight changes, hunger ratings, blood pressure, defecation pattern and possible side-effects were recorded every second week. Before treatment mean body weight was 95.4 kg (Study I) and 99.3 kg (Study II). Six patients dropped out of Study I, and four out of Study II. In Study I mean weight loss, 7.0 kg, in the fibre group was significantly higher (p less than 0.05) than 6.0 kg in the placebo group. In Study II mean weight loss in the fibre group of 6.2 kg was significantly higher than the 4.1 kg in the placebo group (p less than 0.05). No significant difference in hunger feeling between the groups was found. Systolic blood pressure was reduced in all four groups at the end of the treatment, whereas diastolic blood pressure was reduced only in the fibre group in Study II. The results suggest that dietary fibre is of additive value in the treatment of moderately obese patients. The fibre supplement, however, needs to be comparatively high.

alpha 1-Antitrypsin (AAT) deficiency in adults predisposes to lung and liver disease, but its natural history is incompletely known. To better characterize the liver disease, all known deceased adult Swedish patients known to us with homozygous (PiZZ) AAT-deficiency, who had undergone autopsy during the 20-year period 1963-82 were reviewed. Of 94 such patients, 35 had cirrhosis (27 males and eight females) with a mean age at death of 65.5 +/- 10.5 (SD) years compared to 53.6 +/- 12.8 years (p less than 0.01) for the 59 non-cirrhotic patients. The longer survival suggests less severe lung disease in the cirrhotic group. Clinically these patients most frequently presented with ascites or other signs of portal hypertension. Evidence of alcohol overconsumption, chronic viral hepatitis, or autoimmune disease was rare. Aside from low plasma AAT levels, laboratory and other clinical features were indistinguishable from those of decompensated cirrhosis of any etiology. The prognosis was generally grave with a mean survival of two years after diagnosis. Fourteen of the 35 cirrhotics (10 males and four females) had primary liver cancer (PLC) at autopsy. We conclude that cirrhosis and PLC are more frequent complications in elderly patients with AAT-deficiency than was previously known. These complications had a marked male predominance.

A follow-up examination was performed one month to 20 years after adrenalectomy in 28 cases with surgically treated primary aldosteronism due to adrenal adenoma. The mean age at diagnosis was 45, and the mean duration of hypertension seven years. Severe hypertension with a diastolic blood pressure of 130 mmHg or more was observed in 35%. Postoperatively cerebrovascular catastrophe developed in two cases, both of which belonged to the group of patients with severe hypertension. Normalization of blood pressure was observed in 70% and in the remaining subjects the blood pressure was lower than at diagnosis. The blood pressure response to adrenalectomy appeared unpredictable in view of such parameters as the initial blood pressure, age at diagnosis, and duration of the hypertensive state. Toxicosis during pregnancy and metrorrhagia was observed with unexpectedly high frequency in this study population. Low ambulatory plasma renin activity was recorded at the follow-up in 15 out of 18 subjects studied in the absence of evidence of hyperaldosteronism.

The mortality in pacemaker-treated patients is due to underlying disorders, and is increased in patients with ischemic heart disease, congestive heart failure, diabetes and renal dysfunction. We have recently shown that the HLA B27-associated inflammatory disease process is the probable underlying cause in 15-20% of permanently paced men. Consequently, we undertook this study to evaluate any impact on mortality of HLA B27 and associated rheumatic disorders. The mortality among pacemaker patients was compared with that of the general population. Comparisons were also made between pacemaker patients with and without HLA B27 and associated disorders. We did not find any influence on mortality associated with the immunogenetic marker HLA B27 or with HLA B27-associated rheumatic disorders.

The phospholipase A2 and acyltransferase activities characteristic of human plasma lecithin: cholesterol acyltransferase have been evaluated in incubation mixtures of lipoprotein depleted plasma of fish eye disease patients and autologous HDL or homologous normal HDL3. Both enzyme activities were strongly reduced as compared to those of normal controls. These findings further support the claim that fish eye disease plasma has a specific lack of high density lipoprotein lecithin:cholesterol acyltransferase (alpha-LCAT deficiency), although the cholesterol esterification of combined VLDL and LDL in such plasma proceeds at a normal rate.

The effects of cessation of smoking were studied in 343 patients with intermittent claudication. One year after the initial examination 39 (11%) had stopped smoking and 304 (89%) continued to smoke. The outcome in these two groups was compared. They were comparable regarding baseline characteristics. Rest pain did not develop in any of the non-smokers. In smokers the cumulative proportion with rest pain was 16% after seven years (p less than 0.05). The cumulative proportions with myocardial infarctions after 10 years were 11 and 53%; the cumulative rates of cardiac deaths 6 and 43%; and the 10-year survival 82 and 46% among non-smokers and smokers, respectively. In multivariate Cox regression analyses the association between smoking and infarction (p less than 0.05) and cardiac death (p less than 0.05) was significant. The survival curves deviated and when they were compared after one year's follow-up the association between smoking and mortality was significant (p less than 0.05). The findings provide further evidence for the fact that it is of utmost importance that patients with intermittent claudication stop smoking.