bluuebunny/crossref_metadata_2025_split huggingface dataset:
Is serum hyaluronan a helpful tool in the management of patients with liver diseases?
Authors: Lindqvist U. 
Published: November 2003

Lindqvist U (University Hospital, Uppsala, Sweden). Is serum hyaluronan a helpful tool in the management of patients with liver diseases? (Minisymposium: Hyaluronan). J Intern Med 1997; 242: 67–71.The results of serial determinations of serum hyaluronan indicate a prognostic value in progressive liver damage. In primary biliary cirrhosis and in cirrhotic alcoholic liver diseases serum hyaluronan level discriminates between early and advanced disease. In alcoholic liver disease serum hyaluronan can be applied as assessment of haemodynamic changes. Serum hyaluronan is of use as a non‐invasive index of liver fibrosis in chronic viral hepatitis. A reversible defect in the hyaluronan receptor of the hepatic endothelial cells was suggested following studies on paracetamol‐induced acute liver damage. In liver transplantation, graft function can be predicted by determination of the venous effluent of hyaluronan.
Declining autopsy rate in Sweden: a study of causes and consequences in Malmö, Sweden
Authors: LINDSTRÖM P. , JANZON L. , STERNBY N. H. 
Published: November 2003

Lindström P, Janzon L, Sternby NH (Helsingborg Hospital; the Division of Epidemiology, Department of Community Medicine; and the Department of Pathology, Lund University, Malmö University Hospital, Sweden). Declining autopsy rate in Sweden: a study of causes and consequences in Malmö, Sweden. J Intern Med 1997; 242: 157–65.ObjectiveIn Sweden there has been a continuous decrease in the autopsy rate during the last 15–20 years. The autopsy rate in the city of Malmö has declined from 81% in 1984 to 34% in 1993. The intention of this study was to improve our understanding of the causes of the decline, and to assess its consequences with regard to cause of death surveillance and case retrieval in epidemiological and clinical research.DesignA retrospective study.SettingThe University Hospital in Malmö, a city in southern Sweden with 230 000 inhabitants.SubjectsAll deceased in 1984 (2900) and 1993 (3198). Cancer incidence 1984 and 1993.Main outcome measuresChanges in autopsy rate in relation to age, sex, place of death and cause of death 1984 and 1993. Changes in the distribution of underlying causes of death in cases submitted and not submitted for a post mortem 1984 and 1993. Changes in cancer incidence and the number of incidentally‐detected new tumours 1984 and 1993.ResultsPatients who died in nursing homes were less often sent for a post mortem than were patients who died at the hospital. From 1984 to 1993 the percentage of people dying in nursing homes increased from 1 to 29%. Care of the elderly has been reorganized, and the number of terminally ill patients who die in nursing homes has increased during the last 20 years in Sweden. This shift in place of death seems to be the single major explanation of the declining autopsy rate in Malmö. The overall autopsy rate in 1993 remained, however, lower than it was in 1984 when a shift in place of death was taken into account. During the period of study there was a decline in the autopsy rate at all departments within the hospital. The distribution of underlying causes of death according to the death certificates was similar in 1984 and in 1993. However, in cases not submitted for autopsy in 1993 there were greater proportions dying from pulmonary and circulatory diseases, respectively, than there were in 1984. The lower autopsy rate in 1993 was for several cancer sites also associated with a reduced number of new cancer cases. The percentage of tumours incidentally detected at autopsy went down in men from 40 to 19% and in women from 39 to 17%.ConclusionPatients submitted for autopsy represented, with regard to age, sex, cause and place of death, a selected group of all deceased. The declining autopsy rate was associated with a difference in the distribution of underlying cause of death and of the incidence of cancer. It is concluded that the changes in the autopsy rate have to be taken into account in studies dealing with time trends of causes of death and incidence of cancer.
Acute intermittent porphyria: prevalence of mutations in the porphobilinogen deaminase gene in blood donors in France
Authors: Nordmann Y. , Puy H. , Da Silva V. , Simonin S. , Robreau A. M. , Bonaiti C. , Phung L. N. , Deybach J. C. 
Published: November 2003

Nordmann Y, Puy H, Da Silva V, Simonin S, Robreau AM, Bonaiti C, Phung LN, Deybach JC (Centre Français des Porphyries, Hopital Louis Mourier, Colombes Cedex, France, and the Institut Gustave Roussy, Cedex, France). Acute intermittent porphyria: prevalence of mutations in the porphobilinogen deaminase gene in blood donors in France. J Intern Med 1997; 242: 213–217.ObjectivesAcute intermittent porphyria (AIP) is an autosomal dominant disorder resulting from a 50% deficiency in porphobilinogen deaminase (PBG deaminase). The true prevalence in the general population of mutations in the PBG deaminase gene capable of causing AIP is unknown. However, it is important to identify asymptomatic carriers of AIP mutations because all are at risk to have an acute attack.DesignWe measured erythrocyte PBG deaminase from 3350 healthy blood donors. When a clear cut deficiency (< mean minus 2.5 SD) was found, the PBG deaminase gene was analysed by molecular biology technics.SubjectsFour subjects with PBG deaminase deficiency were identified. Two had mutations in the PBG deaminase gene which are known to cause AIP.ConclusionWe conclude that, in France, the mutations of the PBG deaminase gene show a high prevalence in the healthy population. If only these two confirmed latent cases are used for the calculation, in France the minimal prevalence of the AIP gene is 1:1675.
A superfemale with primary Sjögren's syndrome which involved systemic organs
Authors: MIYAKAWA H. , IYONAGA K. , ARIMA S. , YONEKAWA Y. , SUGA M. , ANDO M. 
Published: November 2003

Miyakawa H, Iyonaga K, Arima S, Yonekawa Y, Suga M, Ando M (First and Third Departments of Internal Medicine, Kumamoto University School of Medicine, Kumamoto, Japan). A superfemale with primary Sjögren's syndrome which involved systemic organs (Case report). J Intern Med 1997; 242: 261–5.A 52‐year‐old Japanese woman complicated by a sex chromosomal anomaly as a superfemale, a mosaic of XXXXX/XXXX/XXX/XX/XO, with mild mental retardation, was hospitalized for dry mouth, dry eyes, and proteinuria. The sialography of the right parotid gland showed a globular‐type gland enlargement. A definite diagnosis of primary Sjögren's syndrome (SS) was made, and further examinations revealed not only typical sicca syndrome but also systemic extraglandular lymphocytic infiltration; interstitial pneumonitis, glomerular‐ and interstitial nephritis, superficial gastritis, thyroiditis, and a severe excitation conductive impairment of heart. We report a very rare case of superfemale with primary SS which involved systemic organs.
IgA nephropathy: 30 years on
Authors: Pettersson E. 
Published: November 2003

Pettersson E (Karolinska Institute, Huddinge University Hospital, Stockholm, Sweden). IgA nephropathy: 30 years on (Review). J Intern Med 1997; 242: 349–53.Thirty years since its discovery, IgA nephropathy (IgAN) has turned out to be the commonest primary glomerulonephritis around the world. The major presenting symptom is haematuria, with or without proteinuria. IgAN was initially considered a benign disease, but long‐term follow‐up studies have shown the course to be slowly progressive with up to 50% of the patients developing terminal renal failure after several decades. The aetiology and pathogenesis are still unclear, but IgAN seems to be associated with a dysregulation of the immune response which is not very well understood. There is today no specific treatment. Effective blood pressure control is the cornerstone of therapy.
Moderate beer consumption and positive biochemical changes in patients with coronary atherosclerosis
Authors: GORINSTEIN S. , ZEMSER M. , BERLINER M. , GOLDSTEIN R. , LIBMAN I. , TRAKHTENBERG S. , CASPI A. 
Published: November 2003

Gorinstein S, Zemser M, Berliner M, Goldstein R, Libman I, Trakhtenberg S, Caspi A (School of Pharmacy, Hebrew University of Jerusalem, Jerusalem, Israel). Moderate beer consumption and positive biochemical changes in patients with coronary atherosclerosis. J Intern Med 1997; 242: 219–24.ObjectivesThe aim of this study was to evaluate the influence of moderate beer consumption on lipid metabolism and antioxidant activity in patients (pts) with coronary artery disease (CAD).SubjectsForty‐eight male pts with CAD not alcohol beverages consumers were randomly divided into experimental (EG) and control (CG) groups, 24 pts each.SettingRehovot University Hospital, Israel.InterventionEvery patient of the EG during a period of 30 consecutive days consumed 330 ml of Maccabee beer (>20 g of alcohol). The pts of the CG did not consume alcohol during the trial period.MethodsA wide range of tests including total cholesterol, LDL–C, HDL–C, total tocopherol and α–tocopherol.ResultsOnly in the pts of the EG were found a tendency to an increase of the level of HDL–C and a statistically significant rise in the level of total tocopherol (P < 0.025) and α–tocopherol (P < 0.025).ConclusionsEven a short period of moderate beer consumption leads to some favourable biochemical changes in blood of pts with CAD which are widely regarded as indicators of CAD prevention.
The emergence of mouse models of atherosclerosis and their relevance to clinical research
Authors: Smith J. D. , Breslow J. L. 
Published: November 2003

Smith JD, Breslow JL (Laboratory of Biochemical Genetics and Metabolism, The Rockefeller University, New York, NY, USA). The emergence of mouse models of atherosclerosis and their relevance to clinical research (Review). J Intern Med 1997; 242: 99–109.Due to the ability to introduce or mutate genes, the mouse has become the most common experimental animal model for atherosclerosis research. Wildtype mice on a chow diet do not get atherosclerosis. Three ways to induce atherosclerosis in mice are discussed: diet‐induced, apoE deficiency‐induced, and LDL receptor‐deficiency induced. The atherosclerotic lesions in apoE‐deficient mice have been well characterized, and they resemble human lesions in their sites of predilection and progression to the fibroproliferative stage. These mouse models of atherosclerosis are being used to identify genes which modify atherosclerosis susceptibility and in the development of antiatherogenic therapies.
The clinical course and outcome in patients with acute ischaemic stroke and transient ischaemic attack in relation to severe carotid disease
Authors: RÖDÉN‐JÜLLIG Å. 
Published: November 2003

Rödén‐Jüllig Å (Karolinska Institutet, Danderyd Hospital, Stockholm, Sweden). The clinical course and outcome in patients with acute ischaemic stroke and transient ischaemic attack in relation to severe carotid disease. J Intern Med 1997; 242: 355–60.ObjectivesTo determine whether a severe ipsilateral carotid lesion influences the patient's clinical course and outcome in the acute phase of an ischaemic stroke or transient ischaemic attack (TIA).DesignAn evaluation of prospectively‐ collected and recorded clinical information and sonographic findings in patients treated in a stroke unit.SubjectsTwo hundred and sixty‐six consecutive patients with acute ischaemic stroke or TIA in the carotid territory.Main outcome variablesA sonography of the carotid arteries was performed in all cases. Stroke symptoms were continuously monitored and progression and recurrence particularly looked for. Patient outcome at discharge was also recorded.ResultsAn ipsilateral carotid stenosis of ≥ 70% was found in 11%, and 4% had an occlusion. These patients had no statistically significant increase in the rate of progression or recurrence of stroke during the acute phase. Furthermore, they did not become more disabled or in need of extended institutional care.ConclusionsSevere ipsilateral carotid disease does not significantly influence the acute clinical course or outcome in ischaemic stroke patients and there seems to be no need for urgent sonography and treatment.
Myelodysplastic syndrome with vasculitic manifestations
Authors: Pirayesh A. , Verbunt R. J. A. M. , Kluin PH. M. , Meinders A. E. , de Meijer P. H. E. M. 
Published: November 2003

Pirayesh A, Verbunt RJAM, Kluin PHM, Meinders AE, De Meijer PHEM (Leiden University Hospital, Leiden, The Netherlands). Myelodysplastic syndrome with vasculitic manifestations (Case Report). J Intern Med 1997; 242: 425–31.Vasculitis in patients with the myelodysplastic syndrome (MDS) is a rare phenomenon. We describe a patient who presented with necrotic lesions of his toes, which proved to be the result of immune complex mediated vasculitis. This unusual combination of vasculitis and MDS prompted us to review the literature. Forty‐four cases of vasculitis in association with MDS were found. The pathogenesis of the vasculitis in MDS remains speculative, although several reports suggest an immunological mechanism. The temporal relationship could not be determined in 20 (45%) of reported cases. The prognosis of these patients appears to be worse than in patients with MDS without vasculitis. Steroids were used in 41 (93%) of the reported cases. Our patient did not receive any drug therapy, nevertheless his necrotic lesions improved within a few weeks.
Limited Wegener's granulomatosis with central nervous system involvement and fatal outcome
Authors: NORDMARK G. , BOQUIST L. , RÖNNBLOM L. 
Published: November 2003

Nordmark G, Boquist L, Rönnblom L (Boden Hospital and University Hospital, Umeå, Sweden). Limited Wegener's granulomatosis with central nervous system involvement and fatal outcome (Case Report). J Intern Med 1997; 242: 433–36.A 74‐year‐old woman was admitted to the medical department with vertigo and confusion, and suffered seizures three days later. Clinical, radiological and histopathological examination revealed limited Wegener's granulomatosis (WG) with central nervous system (CNS) involvement. Initially she was treated successfully with prednisolone and cyclophosphamide, but she relapsed after 25 months and died four weeks later. Autopsy showed widespread granulomas with vasculitis in the cerebrum. These findings emphasize the importance of considering WG in CNS disease of unclear origin, and demonstrate the aggressive nature of the disease.
Colonic endocrine cells in inflammatory bowel disease
Authors: El‐Salhy M. , Danielsson Å. , Stenling R. , Grimelius L. 
Published: November 2003

El‐Salhy M, Danielsson Å, Stenling R, Grimelius L (University Hospital, Umeå; and University Hospital, Uppsala; Sweden). Colonic endocrine cells in inflammatory bowel disease. J Intern Med 1997; 242: 413–19.ObjectivesTo study colonic endocrine cell types in patients with ulcerative colitis (UC) and Crohn's disease (CD).SettingDepartments of Medicine and Pathology, University Hospitals, Umeå and Uppsala, Sweden.SubjectsSeventeen patients with UC (seven females and 10 males) and 11 patients with CD (five females and six males). Twenty‐two patients (eight females and 14 males) operated on for colon carcinoma and without signs of inflammatory bowel disease were used as controls.MeasurementsThe colonic endocrine cell types were identified by immunohistochemical methods and quantified by computed image analysis.ResultsThe areas of the argyrophil cells as well as those immunoreactive to chromogranin A and serotonin were significantly increased in patients with both UC and CD, compared with those in the controls. In patients with CD, the areas of polypeptide YY(PYY)‐ and pancreatic polypeptide (PP)‐immunoreactive cells were significantly reduced, whilst the area of enteroglucagon‐immunoreactive cells was increased. There was no statistical difference in endocrine cell area between specimens with slight versus severe inflammation, except for PYY and enteroglucagon cell areas in patients with CD. Whilst the former cell area decreased, the latter increased in specimens with severe inflammation. The mean cellular area for each endocrine cell type did not differ between the controls and patients with UC or CD.ConclusionsThe increase in the serotonin‐immunoreactive cell area in patients with both UC and CD might be one of the factors causing reduced colonic contraction and increased intraluminal pressure observed in patients with inflammatory bowel disease. Furthermore, in patients with CD, the decreased PYY‐immunoreactive cell area may explain the decreased absorption and increased secretion found in these patients.
Abnormal ambulatory electrocardiographic findings in patients with the Marfan syndrome
Authors: SAVOLAINEN A. , KUPARI M. , TOIVONEN L. , KAITILA I. , VIITASALO M. 
Published: November 2003

Objectives. The aim of this study was to assess the prevalence of cardiac dysrhythmias and abnormalities of conduction and repolarization in the Marfan syndrome (MFS). Subjects and methods. Forty‐five adult MFS patients (25 men) and healthy age and sex matched controls. A 24‐h ambulatory electrocardiogram was recorded. Results. There was no difference in heart rates between the two groups. Two MFS patients had atrial fibrillation. The median number of premature atrial beats was 12/24 h in the MFS group vs. 6/24 h in the controls (P<0.05), and the respective medians of premature ventricular beats were 17/24 h vs. 1/24 h (P<0.001). Five patients but no healthy person had salvos of 3 premature ventricular complexes (P<0.05). Ventricular premature beats with R on T configuration were recorded in nine patients but in none of the control subjects (P<0.05). Both PQ and QT intervals at heart rates of 60, 80 and 100 beats min−1 were longer in the MFS group compared with healthy persons (P<0.005). Also ST segment depression was seen more often in the MFS group (17/43 vs. 6/45; P<0.05). In patients with MFS, the findings at ambulatory electrocardiography showed no association with echocardiographically determined aortic root diameter, left atrial diameter or left ventricular diameters, wall thickness and systolic function. Nor did the electrocardiographic findings correlate with the presence of mitral or tricuspid valve prolapse. Conclusions. Patients with MFS have a higher prevalence of cardiac dysrhythmias than healthy persons. Likewise they have prolonged atrio‐ventricular conduction time and disturbed depolarization as suggested by longer QT intervals and more common ST segment depression.
The factor VR506Q mutation causing APC resistance is highly prevalent amongst unselected outpatients with clinically suspected deep venous thrombosis
Authors: SVENSSON P. J. , ZÖLLER B. , MATTIASSON I. , DAHLBÄCK B. 
Published: November 2003

Objective. Resistance to activated protein C (APC resistance), caused by a single point mutation in the factor V gene (FV:R506Q), is a major risk factor for venous thrombosis. As the significance of this mutation among unselected outpatients with deep‐vein thrombosis (DVT) is not established, we have studied its prevalence among consecutive outpatients attending the emergency room due to a clinically suspected DVT. Design, setting and subjects. The FV:R506Q mutation was determined in 223 consecutive Swedish outpatients with clinically suspected DVT, and in 288 healthy controls. Using phlebography, the patients were classified as DVT‐positive or DVT‐negative. Main outcome measure. The prevalence of FV:R506Q mutation. Results. The prevalence of the FV∶R506Q mutation was 28% (28/99) in the DVT‐positive subgroup (relative risk: 3.1; 95% CI: 1.7–5.5), and 23% (28/124) in the DVT negative subgroup (relative risk: 2.0; 95% CI: 1.1–3.6), as compared to 11% (32/288) in the control group. In the DVT‐positive subgroup, the FV∶R506Q mutation was most common among younger patients with primary thrombosis (47%) and least common among older patients with secondary thrombosis (19%). The high prevalence of FV:R506Q mutation among DVT‐negative patients was associated with a high frequency of previous venous thrombosis. Thus, 46% (13/28) of the DVT‐negative FV∶R506Q carriers had a history of thrombosis, compared with only 22% (21/96) of the DVT‐negative patients lacking the mutation (P=0.01). Conclusion. To sum up, the FV:R506Q mutation is present in more than a quarter of Swedish DVT‐positive outpatients with clinically suspected DVT, indicating that APC‐resistance is a major thrombotic risk factor contributing to the high incidence of venous thrombosis in Sweden.
Haemophilia prophylaxis in young patients–a long‐term follow‐up
Authors: LÖFQVIST THOMAS , NILSSON INGA MARIE , BERNTORP ERIK , PETTERSSON HOLGER 
Published: November 2003

Objectives. To review long‐term prophylactic factor treatment in young patients with severe haemophilia A and B, focusing on the orthopaedic and radiological outcome. Design. We received 34 patients with severe haemophilia A (n=29) and B (n=5), aged 7–22 years. Age at start of treatment was 1–4.5 years. Dosages of factor concentrate (F VIII and F IX, respectively) were 25–40 IU/kg body weight, three times a week for haemophilia A and twice a week for haemophilia B. The patients had been checked annually over a 5‐year period (1990–95). Orthopaedic and radiological joint scores were evaluated according to recommendations by the World Federation of Haemophilia. Setting. All results were obtained at the Department for Coagulation Disorders, University of Lund, Malmö University Hospital, Malmö, Sweden. Results. Orthopaedic and radiological joint scores were found to have remained unchanged during follow‐up in almost all patients and to be still zero (i.e. no unaffected joints) in 79% (n=27) of the patients. Conclusion. There is a growing international consensus haemophilic arthropathy can be prevented by administering early high‐dose prophylaxis. The results of the present investigation strongly support this opinion.
Do drug costs affect physicians' prescription decisions?
Authors: HART J. , SALMAN H. , BERGMAN M. , NEUMAN V. , RUDNIKI C. , GILENBERG D. , MATALON A. , DJALDETTI M. 
Published: November 2003

Objective. To study the impact of the cost of pharmaceuticals on physicians' decisions about drug prescription. Design. A simulation protocol for the treatment of two patients, one with mild and the other with a severe form of urinary tract infection (UTI), was designed. Thirty family physicians in outpatient clinics and 30 physicians in the internal medicine wards of a Community Hospital participated in the project. They had to prescribe treatment for the patients twice: at phase I, when the drug cost was unknown, and at phase II, after 2 months, when the price of the drugs was brought to their attention. The physicians selected the medication from a list of drugs commonly used for the treatment of UTIs. Results. Analysis of the findings indicates that an awareness of drug costs affects prescription decisions among physicians in hospital wards, whereas family physicians showed a preference for less expensive drugs even before they were informed about drug costs. An extrapolation of the results shows that knowledge about the cost of the drugs usually administered for treatment of UTI, could save at least IS 112883 ($34207) a month to Kupat Holim Klalit (KHK) the health insurance institution to which the outpatient clinics and the hospital belong. Conclusions. When economic aspects of healthcare are considered, information on drug costs may be an important factor in physicians' decision‐making processes and for saving pharmaceutical expenses.