ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03199.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.A comprehensive survey of the Swedish hemophilic population (hemophilia A and B) was undertaken in 1980. The total number of known hemophiliacs was 564. The ratio of hemophilia A to B was 4:1. The severe form constituted about 30% of each type. The incidence has remained fairly constant since 1957 and was 1.67 per year per 10000 males born. The prevalence was 6.8 per 100000 inhabitants. There was an increase in the prevalence in all counties but the increase varied considerably, 19–383%. The overall increase of hemophilia was 123%. Among mild cases the increase was 245%, whereas it was only 48% among the severe hemophiliacs. This skewness was attributed to better laboratory diagnoses and increased life expectancy. The proportion of mild cases increased from 35% in 1960 to 54% at the time of the present investigation. The situation was the reverse for the severe cases, a decrease from 45% to 30%. The mean and median ages among moderate and mild hemophiliacs deviated only little from the corresponding figure in the total population. Among severe cases, however, mean and median age were 8–13 years less than in the normal populat

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03200.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.Measurements of radionuclide first‐pass left ventricular ejection fraction (LVEFm) were carried out in 37 patients receiving doxorubicin and/or daunorubicin treatment for their malignant disease. The validity of the systolic time intervals (STI) and echocardiography (ECHO) in the detection of left ventricular dysfunction was evaluated using LVEFmas reference method. LVEFmshowed a significant decrease in left ventricular function with cumulative anthracycline doses in patients with and without previous clinical evidence of cardiovascular disease. A multiple regression analysis showed that the impairment in LVEFmwas significantly correlated with the cumulative anthracycline dose, patient's age and previous cardiovascular disease. In five patients (one without previous cardiac disease, one with coronary artery disease, three with hypertension) the drug had to be withdrawn due to signs or symptoms of cardiac dysfunction. The sensitivity and the specificity of STI and ECHO in the detection of left ventricular dysfunction remained rather low as compared with the results by LVEF

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03201.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.The hepatic injury developing during occupational exposure to chemicals was investigated in 23 male patients, aged 23–49 years, by comparing case histories, liver function tests, hepatic microsomal enzyme activities in vivo and in vitro with histology. The subjects, 15 chemical industry workers and 8 painters, had disturbed liver tests after years of exposure to solvents, paints and lacquers. Characteristic for the patients was a 2–4‐fold increase in serum aminotransferases associated with normal liver or reactive hepatitis with or without fatty liver. AU patients, except subjects with fatty change, had meta‐bolically active liver which was reflected as adaptive and toxic changes in cellular ultrastructure. The biochemical liver tests normalized within 3–6 weeks after cessation of the exposure. The findings demonstrate that occupational exposure to chemical solvente may insidiously damage the liver. The injury is detectable by biochemical, metabolic and histological investigations in the ea

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03202.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.Cardiac conduction disturbances were found in 22 (33%) of 68 patients during the course of ankylosing spondylitis (AS) of more than 25 years' duration. Complete heart block occurred in six patients, four of whom received a permanent pacemaker and one made a complete recovery. The high prevalence of conduction disturbances in AS in this report, exceeding what has previously been reported, is partly explained by the finding of intermittently occurring atrioventricular blocks.

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03203.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.A 43‐year‐old woman and her 47‐year‐old brother were studied because of corneal opacity. They showed a marked decrease in plasma high density lipoproteins (HDL) and a decrease in the ester ratio of plasma total cholesterol. Discoidal particles were found in the HDL2 fraction (d1.063–1.125). A marked heterogeneity of low density lipoproteins was disclosed in both patients by electron microscopy. Apoprotein analysis revealed an increase in apo E and a decrease in apo A‐I and A‐II in both patients. These abnormalites were similar to the data reported in other cases with hereditary lecithin: cholesterol acyltransferase (LCAT) deficiency. However, several interesting dissimilarities have been disclosed as compared with the previously reported cases. Neither patient had proteinuria, and their kidney functions were within the normal limits. The ester ratios of plasma cholesterol of both patients were the highest among the cases reported thus far. Their plasma LCAT activities were 14.4 and 15% of the normal mean values determined by Glomset‐Wright's common‐substrate method. The enzyme activities determined by Stokke‐Norum's self‐substrate method were 40.2 and 29%, respectively. These results may indicate that this inherited disorder is not characterized by absence of plasma LCAT or presence of inhibitory factors in plasma, but by the presence of partially inactive LCAT in

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03204.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.The renal function is assumed to be normal in most patients with familial amyloidosis with polyneuropathy (FAP) type 1. In the present study of 24 patients with FAP type 1, estimation of the urinary concentrating capacity and ***31Cr‐EDTA clearance demonstrated that the glomerular clearance and the concentrating capacity were impaired in most patients. The concentrating capacity was significantly correlated with the clearance values. Urine electrophoresis indicated a glomerular cause for the proteinuria. It is suggested that the renal dysfunction should be attributed to deposition of amyloid substance in the glomerul

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03205.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.Postprandial hyperglycemia was significantly (p<0.05) reduced during administration of the fibre supplement Metamucil in nine insulin‐dependent diabetics. A similar but less pronounced effect was also found on the plasma gastric inhibitory polypeptide secretion. Six subjects noted an increased frequency of hypoglycemic episodes, and reductions in insulin dosage might have been necessary if the experiment had continued longe

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03206.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.To compare maximal exercise testing with the NYHA functional criteria in grading symptomatic severity in mitral valve disease, a symptom‐limited bicycle exercise test was performed by 40 patients undergoing right and left heart catheterization. In the group as a whole (r=‐0.50) and in patients with combined valvular lesion (r=− 0.72), peak O2uptake (VO2) correlated with the NYHA criteria, whereas exercise duration failed to demonstrate such a relationship. In each hemodynamic subgroup large individual variations in peak VO2were seen, particularly in NYHA class II and if the lesion was less severe. Unusually high O2cost at peak work load was observed in almost half the patients. However, assuming that patients with an aerobic capacity of ≤ 16 ml/kg per min were severely symptomatic (NYHA class III or IV), an agreement between VO2and the NYHA criteria was seen in 30 patients (75%). In the remaining 10 patients the NYHA criteria were more often predictive of the severity of the lesion. In “pure” mitral stenosis, however, a total VO2of<1.11/min was more likely (r=0.62) to reveal significant valvular narrowing (≤1.5 cm2). Neither peak VO2nor the NYHA criteria revealed impaired left ventricular performance or the degree of mitral regurgitation at rest. Rather then being superior, maximal exercise testing seemed to be complementary to the symptom‐rated appraisal. Recognizing the potential limitations of exercise testing, which mainly appeared to be attributable to variations in the threshold of perceived exertion and in the non‐cardiac component of VO2, measurement of peak VO2is likely to provide a basic estimate of the degree of impairment, particularly in the presence of mitral

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03207.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.Cardiopulmonary resuscitation (CPR) was attempted in 222 cases of sudden death at the City Hospital, Reykjavik, during 1976–79. Of the 68 patients (31%) successfully resuscitated, 47 died in the hospital and 21 (9%) were discharged, 17 in good mental and physical condition. The mean combined response and transport time was 12.1 min and the ambulance mean time of response 7.3 min. The first ECG revealed considerable prognostic indications. Of the 90 patients who had ventricular fibrillation on admission, 42 (47%) were successfully resuscitated and 18 (20%) were subsequently discharged. Among 114 patients with asystole, resuscitation was successful in 23 (20%) and two (2%) were discharged. Immediate first aid in situ had a definite prognostic influence. These results compare favourably with those obtained elsewhere where the organization of first aid and emergency transport is similar. They do not, however, match the results achieved by fully specialized resuscitation teams trained to operate outside the hospital. Results of CPR of patients with cardiac arrest out of hospital in Reykjavik show increasing improvement over the years. This may be partly explained by a considerable public debate on this issue in 1978 and subsequent streamlining of activitie

ISSN:0001-6101    

DOI:10.1111/j.0954-6820.1982.tb03208.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY