Abstract：
OBJECTIVE:To describe the epidemiology, pathogenesis and clinical features of hepatocellular carcinoma (HCC) in patients with acute intermittent porphyria (AIP). DESIGN:A retrospective population-based mortality study. SUBJECTS:All inhabitants who died between 1978-1990 (2122) including 33 with AIP, in two municipalities in northern Sweden with a high prevalence of AIP. INTERVENTIONS:Death certificates and hospital records were examined. Histological re-examination of paraffin-embedded specimens from patients with HCC was performed and hepatitis B virus content analysed. RESULTS:HCC was found in 27% of patients with AIP versus 0.2% of the deceased non-AIP subjects, P < 0.0001. HCC was more common in women (men:women 1:2) and in manifest AIP (manifest: latent 2:1). Liver cirrhosis was more common in AIP patients (12%), especially in women, compared with controls (0.5%), P < 0.0001. CONCLUSIONS:AIP patients seem to have an increased risk of developing HCC. This tumour is more common in patients with manifest AIP and in women, a reversal of the usually reported gender ratio for HCC. No cause for developing HCC other than AIP was found. The pathogenesis may be explained by abnormalities in porphyrin metabolism and by intrinsic production of mutagenic substances, resulting in a condition of systemic overload of oxidative stress, enhancing mutation rate and liver cell injury. Liver cirrhosis appears to be more common in AIP patients and may be a preliminary stage to HCC. All AIP gene carriers aged > or = 55 should be screened for HCC.
journal_name
J Intern Medjournal_title
Journal of internal medicineauthors
Andersson C,Bjersing L,Lithner Fdoi
10.1046/j.1365-2796.1996.21847000.xsubject
Has Abstractpub_date
1996-10-01 00:00:00pages
195-201issue
4eissn
0954-6820issn
1365-2796journal_volume
240pub_type
杂志文章abstract：OBJECTIVES:To establish a population-based 24-h ambulatory blood pressure (ABP) reference material with day/night mean BP calculated by standardized and true bedtimes. DESIGN:A cross-sectional study of 200 randomly selected subjects (20 men and 20 women in each 10-year age interval 20-70 years) in Linköping, Sweden. ...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1046/j.1365-2796.1996.322856000.x
更新日期：1996-11-01 00:00:00
abstract：OBJECTIVE:Atherosclerosis is characterized by accumulation of lipids, cells and extracellular matrix (ECM) proteins in the arterial wall. Collagen type I (COL1), a component of the arterial ECM, is cleaved by matrix metalloproteinases (MMPs) and known to be remodelled in atherosclerosis. We explored whether the MMP-med...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/joim.12819
更新日期：2019-01-01 00:00:00
abstract：:Cardiovascular disease remains the most prevalent cause of human morbidity and mortality in ageing Western societies. Basic and translational scientific efforts have focused on the development and improvement of diagnostic and therapeutic strategies to limit the burden of associated diseases, such as stroke and myocar...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1111/joim.12298
更新日期：2014-12-01 00:00:00
abstract：:Studies of zinc status in insulin-dependent diabetes mellitus (IDDM) have shown contradictory results. Zinc is essential for many enzymes involved in the human metabolism and may play a role in the biosynthesis and storage of insulin in the B-cell. We therefore prospectively followed 26 patients (14 males and 12 femal...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.1989.tb01353.x
更新日期：1989-07-01 00:00:00
abstract：:During the period 1974-1983, Yersinia enterocolitica infection was diagnosed in 458 hospitalized patients by antibody response or isolation of the micro-organism. Eight (1.75%) patients showed signs of acute pancreatitis with elevated serum or urine levels of amylase; two patients had acute insulin-dependent diabetes....
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.1992.tb00970.x
更新日期：1992-05-01 00:00:00
abstract：:Ebola virus disease (EVD) is a zoonotic disease that causes severe haemorrhagic fever, with high fatality rates of up to 90% in humans. Today, there is no effective treatment available. Person-to-person transmission occurs through exposure to blood or body fluids, which can threaten other household members and first-l...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1111/joim.12386
更新日期：2015-09-01 00:00:00
abstract：:The syndrome defined as "idiopathic CD4 lymphocytopenia' (ICL) is a rare disease of unknown aetiology, often associated with severe depression of immune defences and the occurrence of opportunistic infections. A case is reported wherein a severe immunodeficiency syndrome with persistent idiopathic CD4+ lymphopenia dev...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1046/j.1365-2796.1996.447785000.x
更新日期：1996-07-01 00:00:00
abstract：:In healthy individuals, insulin resistance is associated with physiological conditions such as pregnancy or body weight gain and triggers an increase in beta cell number and insulin secretion capacity to preserve normoglycaemia. Failure of this beta cell compensation capacity is a fundamental cause of diabetic hypergl...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1111/joim.12096
更新日期：2013-09-01 00:00:00
abstract：:Interleukin-7 (IL-7) is required for the development and survival of T cells and plays a critical role in modulating T-cell homeostasis. This review will address current understanding of IL-7 biology, review recent clinical experiences and discuss potential future clinical applications of IL-7, or IL-7 blockade, in th...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1111/j.1365-2796.2009.02085.x
更新日期：2009-08-01 00:00:00
abstract：:Originally isolated from a haematophagous hookworm, recombinant nematode anticoagulant protein c2 (rNAPc2) is an 85-amino acid protein with potent anticoagulant properties. Unlike conventional anticoagulants that attenuate blood coagulation via inhibition of thrombin or activated factor X (FXa) at the downstream porti...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1046/j.1365-2796.2003.01224.x
更新日期：2003-10-01 00:00:00
abstract：OBJECTIVES:A description of the clinical presentation, diagnostic procedure and mode of therapy in three patients suffering from chronic mesenteric ischaemia. DESIGN AND INTERVENTIONS:In all cases, the diagnosis was made on the basis of abdominal complaints in combination with angiographic findings. The primary treatm...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.1995.tb01178.x
更新日期：1995-03-01 00:00:00
abstract：:Prions represent a group of proteins with a unique capacity to fold into different conformations. One isoform is rich in beta-pleated sheets and can aggregate into amyloid that may be pathogenic. This abnormal form propagates itself by imposing its confirmation on the homologous normal host cell protein. Pathogenic pr...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1111/j.1365-2796.2011.02387.x
更新日期：2011-07-01 00:00:00
abstract：BACKGROUND:Studies have shown that lipoprotein(a) [Lp(a)], an important carrier of oxidized phospholipids, is causally related to calcific aortic valve stenosis (CAVS). Recently, we found that Lp(a) mediates the development of CAVS through autotaxin (ATX). OBJECTIVE:To determine the predictive value of circulating ATX...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/joim.12519
更新日期：2016-11-01 00:00:00
abstract：:We present three patients with variant angina pectoris and episodes of cardiac arrest. All of them had typical clinical symptoms, ST-segment changes in electrocardiogram, and coronary artery spasm confirmed by arteriography. They were treated with high doses of calcium antagonists and nitrates. An automatic cardiovert...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1046/j.1365-2796.2002.01044.x
更新日期：2002-10-01 00:00:00
abstract：BACKGROUND:Although numerous multicentre studies have estimated the association between ozone exposure and mortality, there are currently no nationally representative multicentre studies of the ozone-mortality relationship in China. OBJECTIVE:To investigate the effect on total (nonaccidental) and cause-specific mortal...
journal_title：Journal of internal medicine
pub_type： 杂志文章,meta分析,多中心研究
doi：10.1111/joim.12724
更新日期：2018-05-01 00:00:00
abstract：OBJECTIVE:The relationship between physical activity and cognitive function is intriguing but controversial. We performed a systematic meta-analysis of all the available prospective studies that investigated the association between physical activity and risk of cognitive decline in nondemented subjects. METHODS:We con...
journal_title：Journal of internal medicine
pub_type： 杂志文章,meta分析,评审
doi：10.1111/j.1365-2796.2010.02281.x
更新日期：2011-01-01 00:00:00
abstract：:Mutations in the cardiac Na+ channel gene SCN5A are responsible for multiple lethal ventricular arrhythmias including Brugada syndrome and congenital long QT syndrome. Here we report a case of Brugada syndrome with ST elevation in the right precordial and inferior leads accompanied by atrial standstill and spontaneous...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1046/j.0954-6820.2003.01247.x
更新日期：2004-01-01 00:00:00
abstract：OBJECTIVE:There is growing evidence that endogenous hypertriglyceridaemia is frequently accompanied by a state of insulin resistance. The present study was performed to determine whether patients with primary endogenous hypertriglyceridaemia commonly have abnormalities in plasma concentrations and turnover rates of fre...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1046/j.1365-2796.1998.00298.x
更新日期：1998-04-01 00:00:00
abstract：:A 59-year-old male patient presented with invalidating osteomalacia of 2.5 years' duration. The osteomalacia was caused by severe malabsorption due to gluten-sensitive enteropathy (GSE). There were no other signs or symptoms of GSE in this patient. Clinical presentation with monosymptomatic osteomalacia is very unusua...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.1992.tb00554.x
更新日期：1992-07-01 00:00:00
abstract：:Poikilothermia syndrome is a rare cause of intrinsic thermoregulatory failure. Patients with this syndrome regulate body temperature poorly, if at all. Recently, a patient was referred to us who had clinical evidence of poikilothermia syndrome, as well as long-standing multiple sclerosis. Computerized tomography and m...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1046/j.1365-2796.1998.00384.x
更新日期：1998-11-01 00:00:00
abstract：OBJECTIVE:To study the prognostic value of neutrophil gelatinase-associated lipocalin (NGAL) in chronic heart failure (HF) of ischaemic aetiology. BACKGROUND:Neutrophil gelatinase-associated lipocalin is a marker of kidney injury as well as matrix degradation and inflammation and has previously been shown to be increa...
journal_title：Journal of internal medicine
pub_type： 杂志文章,随机对照试验
doi：10.1111/j.1365-2796.2011.02503.x
更新日期：2012-05-01 00:00:00
abstract：:The acquired immunodeficiency syndrome (AIDS) has been associated with abnormalities of adrenocortical function, and hypoaldosteronism due to hyporeninaemic hypoaldosteronism (HHA). We here report the case of a woman with AIDS associated with orthostatic hypotension, persistent hyponatraemia and hyperkalaemia, in whom...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.1991.tb00427.x
更新日期：1991-08-01 00:00:00
abstract：OBJECTIVES:We evaluated mortality from cardiovascular disease (CVD), coronary heart disease (CHD) and all causes in relation to use of any hormone therapy (HT) and HT with oestradiol and norethisterone or levonorgestrel. DESIGN:Population-based cohort study. SETTING AND SUBJECTS:Women in three Norwegian counties were...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.2004.01396.x
更新日期：2004-11-01 00:00:00
abstract：:A 40-year-old woman with previous parathyroidectomy for adenoma was found to have a serum calcium level of 5.35 mmol l-1 (21.4 mg dl-1). Inadvertent calcium overdose had occurred because of her mistaken belief that if some prescribed calcium was good, then more was better. Her misconception is in contrast with that of...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.1990.tb00215.x
更新日期：1990-08-01 00:00:00
abstract：:The fight against doping in sports commenced as a result of the death of a Danish cyclist during the Rome Olympic Games in 1960. The International Olympic Committee (IOC) established a Medical Commission (IOC-MC) which had the task of designing a strategy to combat the misuse of drugs in Olympic Sport. Some Internatio...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1111/j.1365-2796.2008.01993.x
更新日期：2008-08-01 00:00:00
abstract：:Multiple sclerosis (MS), a chronic inflammatory demyelinating and neurodegenerative disease of the central nervous system, is today a leading cause of unpredictable lifelong disability in young adults. The treatment of patients in progressive stages remains highly challenging, alluding to our limited understanding of ...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1111/joim.13045
更新日期：2020-07-01 00:00:00
abstract：BACKGROUND:The aim of the study was to investigate the association between the proximal level of the clot and the severity of pulmonary embolism (PE). METHODS:The cohort consisted of 99 consecutive patients with PE diagnosed by multi-detector computed tomography. A new score was constructed by calculating the mean val...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.2006.01733.x
更新日期：2007-01-01 00:00:00
abstract：OBJECTIVES:In this study, we explore the associations of decreased thyroid hormone levels with inflammation, wasting and survival in biochemically euthyroid patients with end-stage renal disease (ESRD). DESIGN:After exclusion of 23 patients with thyroid-stimulating hormone (TSH) values outside the normal range (0.1-4....
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/j.1365-2796.2007.01865.x
更新日期：2007-12-01 00:00:00
abstract：:We created the GIFTs, fusions of granulocyte-colony macrophage-stimulating-factor with IL-2, or IL-15 or IL-21, in order to stimulate distinct, but complimentary elements of the immune response. We found that the physical coupling of two functionally distinct cytokines as a bifunctional hybrid allowed for synergistic ...
journal_title：Journal of internal medicine
pub_type： 杂志文章,评审
doi：10.1111/j.1365-2796.2010.02314.x
更新日期：2011-01-01 00:00:00
abstract：BACKGROUND:The X-linked Fabry disease (FD) is a multiorgan disorder due to alpha-galactosidase A (α-GAL) deficiency with consequent lysosomal accumulation of globotriaosylceramide (Gb3). We established the immunocytochemical detection of Gb3 in blood cells of FD patients as a new method for FD diagnostics, follow-up an...
journal_title：Journal of internal medicine
pub_type： 杂志文章
doi：10.1111/joim.12801
更新日期：2018-10-01 00:00:00
