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Acute hepatic porphyria and hepatocellular carcinoma.

https://arctichealth.org/en/permalink/ahliterature234296
Source
Br J Cancer. 1988 Jan;57(1):117-20
Publication Type
Article
Date
Jan-1988
Author
R. Kauppinen
P. Mustajoki
Author Affiliation
Third Department of Medicine, University of Helsinki, Finland.
Source
Br J Cancer. 1988 Jan;57(1):117-20
Date
Jan-1988
Language
English
Publication Type
Article
Keywords
Adult
Aged
Aged, 80 and over
Carcinoma, Hepatocellular - complications
Female
Finland
Humans
Liver Diseases - complications
Liver Neoplasms - complications
Male
Middle Aged
Porphyrias - complications
Risk
Abstract
In this study we examined the case histories of 163 living and 82 deceased adult Finnish patients with acute hepatic porphyria. There were 184 patients with acute intermittent porphyria and 61 patients with variegate porphyria. Among the 124 of the 163 living patients, who were traced 1984-1985, no hepatocellular carcinoma was found. Among the 82 deceased patients the cause of death was porphyria in 29 (36%), cardiovascular disease in 23 (29%) and hepatocellular carcinoma in 7 (9%). Of the 7 patients with hepatocellular carcinoma, 6 had acute intermittent porphyria and one had variegate porphyria. In acute hepatic porphyria, as compared with the total population, the calculated risk of hepatocellular carcinoma is increased 61-fold.
Notes
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Cites: Hepatogastroenterology. 1986 Feb;33(1):2-53007316
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PubMed ID
2831925 View in PubMed
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Frequency of low erythrocyte porphobilinogen deaminase activity in Finland.

https://arctichealth.org/en/permalink/ahliterature224027
Source
J Intern Med. 1992 Apr;231(4):389-95
Publication Type
Article
Date
Apr-1992
Author
P. Mustajoki
R. Kauppinen
L. Lannfelt
L. Lilius
J. Koistinen
Author Affiliation
Third Department of Medicine, University of Helsinki, Finland.
Source
J Intern Med. 1992 Apr;231(4):389-95
Date
Apr-1992
Language
English
Publication Type
Article
Keywords
Adult
Aminolevulinic Acid - diagnostic use
Blood Donors
Erythrocytes - enzymology
Female
Finland - epidemiology
Humans
Hydroxymethylbilane Synthase - blood
Male
Pedigree
Porphyria, Acute Intermittent
Porphyrias - epidemiology - genetics
Prevalence
Abstract
The frequency of low erythrocyte porphobilinogen deaminase (PBGD) activity was investigated in 2234 blood donors and in 30 patients with acute intermittent porphyria. The mean enzyme activities (+/- SD) were 3.38 +/- 0.58 U and 1.82 +/- 0.41 U, respectively. Eighteen blood donors without any history of symptoms of porphyria or haematological disease had low PBGD activity (less than 2.20 U), and they were studied further. All of them also had subnormal concentrations of the erythrocyte enzyme protein, as determined by an immunological method. Lymphocyte PBGD activity was within the normal range, but this parameter does exhibit a wide overlap between normal and porphyric values. Urinary excretion of porphobilinogen was moderately increased in two of the blood donors. In four of the 18 families of the blood donors with low PBGD activity several first-degree relatives had low erythrocyte enzyme activity, consistent with a dominant mode of inheritance. The 5-aminolaevulinic acid loading-test was normal in the blood donors with familial occurrence of low erythrocyte PBGD. It is concluded that inherited defects in erythrocyte PBGD occurred among Finnish blood donors with a frequency of about 1 in 500. The defects may be identical with those in acute intermittent porphyria (AIP), but other mechanisms are also possible, e.g. a mutation in the erythroid-specific part of the PBGD gene.
PubMed ID
1588264 View in PubMed
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Genetic polymorphism of platelet glycoprotein IIIa in patients with acute myocardial infarction and acute ischaemic stroke.

https://arctichealth.org/en/permalink/ahliterature202480
Source
J Cardiovasc Risk. 1999 Feb;6(1):13-7
Publication Type
Article
Date
Feb-1999
Author
S. Kekomäki
L. Hämäläinen
R. Kauppinen-Mäkelin
H. Palomäki
M. Kaste
K. Kontula
Author Affiliation
Finnish Red Cross Blood Transfusion Service, Helsinki, Finland.
Source
J Cardiovasc Risk. 1999 Feb;6(1):13-7
Date
Feb-1999
Language
English
Publication Type
Article
Keywords
Adult
Angina Pectoris - genetics
Antigens, Human Platelet - genetics
Brain Ischemia - genetics
Chi-Square Distribution
Disease Susceptibility
Female
Finland - epidemiology
Gene Frequency
Genotype
Humans
Male
Middle Aged
Myocardial Infarction - genetics
Platelet Glycoprotein GPIIb-IIIa Complex - genetics
Polymerase Chain Reaction
Polymorphism, Genetic
Risk factors
Statistics, nonparametric
Abstract
It has recently been suggested that the Leu33Pro polymorphism of the platelet glycoprotein IIIa affects the risk of coronary thrombosis. Finland is genetically isolated and has an incidence of cardiovascular disease among the highest in the world. Interestingly, the prevalence of ischaemic heart disease also varies in different parts of the country, being highest in eastern Finland.
We studied the Leu33Pro polymorphism using polymerase chain reaction in 133 patients with coronary artery disease, 234 patients with cerebrovascular disease and 326 control subjects originating from two areas of Finland.
The frequencies of the Pro33 allele in the patients with acute myocardial infarction and cerebrovascular attack were 13% and 14%, respectively, and did not differ from the controls (13%). Among patients with acute myocardial infarction from the Helsinki area, the family history of premature coronary artery disease was more often positive in carriers of the Pro33 allele than in non-carriers, but after adjustment for multiple comparisons the difference was no longer significant.
We could not confirm the original observation that the Pro33 allele constitutes an independent risk factor for coronary artery disease. Further studies are needed to clarify whether co-occurrence of Pro33 and some unrecognized inherited factor pose an additional risk of vascular disease.
PubMed ID
10197287 View in PubMed
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Hemi-Kock reconstruction following cystectomy: experience and results from a small urological unit.

https://arctichealth.org/en/permalink/ahliterature24213
Source
Ann Chir Gynaecol Suppl. 1993;206:39-42
Publication Type
Article
Date
1993
Author
R. Kauppinen
O. Lindell
Author Affiliation
Department of Surgery, Lapland Central Hospital, Rovaniemi, Finland.
Source
Ann Chir Gynaecol Suppl. 1993;206:39-42
Date
1993
Language
English
Publication Type
Article
Keywords
Bladder Neoplasms - epidemiology - surgery
Cystectomy
Follow-Up Studies
Humans
Ileum - surgery
Male
Middle Aged
Postoperative Complications - epidemiology - physiopathology
Time Factors
Urinary Reservoirs, Continent
Urodynamics - physiology
Abstract
Between December 1988 and January 1992, 11 men underwent lower urinary tract reconstruction by means of the hemi-Kock ileal reservoir following radical cystoprostatectomy (ten patients) and subtotal cystectomy (one patient). Six of the 11 patients developed an early postoperative complication, with a fatal outcome in two of them. One patient succumbed to subarachnoidal bleeding and one to septic shock. During the follow-up, another two men died of advanced bladder carcinoma. The late complication rate was low. Most patients regained their continence within six months. A good continence was achieved in all the nine remaining patients during the day and in six of them at night. This experience suggests that the hemi-Kock reservoir is a useful method for the urinary tract reconstruction following cystectomy.
PubMed ID
8291867 View in PubMed
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Long-term economic evaluation of intensive patient education during the first treatment year in newly diagnosed adult asthma.

https://arctichealth.org/en/permalink/ahliterature195736
Source
Respir Med. 2001 Jan;95(1):56-63
Publication Type
Article
Date
Jan-2001
Author
R. Kauppinen
V. Vilkka
H. Sintonen
T. Klaukka
H. Tukiainen
Author Affiliation
South Karelia Central Hospital, Department of Respiratory Diseases, Lappeenranta, Finland. ritva.kauppinen@ekshp.fi
Source
Respir Med. 2001 Jan;95(1):56-63
Date
Jan-2001
Language
English
Publication Type
Article
Keywords
Adolescent
Adult
Aged
Asthma - economics - physiopathology - therapy
Cost-Benefit Analysis
Disease Progression
Female
Finland
Follow-Up Studies
Forced expiratory volume
Health Care Costs
Humans
Male
Middle Aged
Patient Dropouts
Patient Education as Topic - economics - methods
Quality of Life
Self Care - economics
Treatment Outcome
Abstract
The cost-effectiveness of intensive patient education of guided asthma self-management given during the first treatment year was evaluated after 5 years of follow-up. Consecutive, newly-diagnosed asthmatics (n = 162, age 18-76 years) were randomized for intensive (80 patients) vs. conventional patient education. Effectiveness was evaluated in terms of lung functions, airway hyperresponsiveness (PD15), and quality of life as measured by the generic 15D and disease-specific St. George's Respiratory Questionnaire (SGRQ). Total treatment costs were also estimated. All patients had anti-inflammatory treatment from the beginning. Sixty-four intervention group (IG) patients and 70 control group (CG) patients were evaluated after 5 years. Forced expiratory volume in 1 sec (FEV1) improved only in the IG, and only during the first treatment year. However, PD15 improved throughout the follow-up. The unscheduled healthcare costs were significantly higher in the CG than in the IG (P = 0.04) and the relative risk for sickness days due to asthma was lower in the IG than in the CG, odds ratio 0.33 (95% CI 0.28; 0.40). However, because there was no significant difference between the groups in any outcome variable or in total costs at 5 years, the incremental cost-effectiveness ratio could not be calculated. The first year intervention had only a short-term beneficial treatment effect, which the CG could catch up during the two last follow-up years, except in FEV1. The peak expiratory flow (PEF)-based self-management had no advantage over the symptom-based self-management. However, the intervention had a consistent tendency of being less costly in the long-run. It is possible to conclude tentatively that regular effective medical treatment and control visits during the first treatment year is at least as important for the long-term treatment result as intensive patient education.
PubMed ID
11207019 View in PubMed
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Prognosis of acute porphyria: occurrence of acute attacks, precipitating factors, and associated diseases.

https://arctichealth.org/en/permalink/ahliterature224713
Source
Medicine (Baltimore). 1992 Jan;71(1):1-13
Publication Type
Article
Date
Jan-1992
Author
R. Kauppinen
P. Mustajoki
Author Affiliation
Third Department of Medicine, University of Helsinki, Finland.
Source
Medicine (Baltimore). 1992 Jan;71(1):1-13
Date
Jan-1992
Language
English
Publication Type
Article
Keywords
Acute Disease
Age Factors
Cause of Death
Finland - epidemiology
Humans
Life tables
Porphobilinogen - urine
Porphyrias - diagnosis - epidemiology - mortality
Prognosis
Prospective Studies
Questionnaires
Registries
Retrospective Studies
Risk factors
Sex Factors
Abstract
We evaluated the prognosis of acute porphyria among 206 adult Finnish patients with acute intermittent porphyria (AIP) or variegate porphyria (VP). The series represents all known patients with these porphyrias in Finland. Of the 47 patients who had a total of 117 acute attacks during the period 1967-1989, 6 died during an attack and 21 attacks were associated with paresis; the frequency of severe attacks was significantly smaller than before 1967 (p = 0.00002). Most pareses and deaths occurred because of a delay in diagnosis and inappropriate treatment of porphyria. For those patients who were symptom-free at the time of diagnosis (1365 follow-up years), the risk of the first subsequent attack was significantly smaller than for those who had had an acute attack before the diagnosis of porphyria (1047 follow-up years, p = 0.005). In addition, milder symptoms of porphyria were more common among those who had had previous attacks than among those who had not (p less than 0.00001). In AIP the risk of attacks correlated with the excretion of porphobilinogen in the urine during remission among adults (p = 0.03); a low rate of excretion predicted freedom from acute attacks. A regular use of many precipitating drugs was never associated with symptoms of porphyria. Two percent of the surgical operations and 4% of the pregnancies were associated with acute attacks. Nearly one-third of the women had symptoms of porphyria associated with the menstrual cycle, but these seldom proceeded to an acute attack. Forty-six percent of the women had used sex-hormone preparations regularly; 2 of them (4.5%) experienced associated acute attacks. Patients with AIP or VP showed increased incidences of hepatocellular carcinoma, and probably also chronic renal failure and hypertension.
PubMed ID
1549056 View in PubMed
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Quantification of metabolites from single-voxel in vivo 1H NMR data of normal human brain by means of time-domain data analysis.

https://arctichealth.org/en/permalink/ahliterature11357
Source
MAGMA. 1995 Sep-Dec;3(3-4):129-36
Publication Type
Article
Author
M. Ala-Korpela
J P Usenius
J. Keisala
A. van den Boogaart
P. Vainio
J. Jokisaari
S. Soimakallio
R. Kauppinen
Author Affiliation
Department of Physical Sciences, University of Oulu, Finland.
Source
MAGMA. 1995 Sep-Dec;3(3-4):129-36
Language
English
Publication Type
Article
Keywords
Aspartic Acid - analogs & derivatives - metabolism
Brain - anatomy & histology - metabolism
Choline - metabolism
Comparative Study
Creatine - metabolism
Humans
Magnetic Resonance Spectroscopy - methods
Protons
Research Support, Non-U.S. Gov't
Signal Processing, Computer-Assisted
Abstract
We present here a combination of time-domain signal analysis procedures for quantification of human brain in vivo 1H NMR spectroscopy (MRS) data. The method is based on a separate removal of a residual water resonance followed by a frequency-selective time-domain line-shape fitting analysis of metabolite signals. Calculation of absolute metabolite concentrations was based on the internal water concentration as a reference. The estimated average metabolite concentrations acquired from six regions of normal human brain with a single-voxel spin-echo technique for the N-acetylaspartate, creatine, and choline-containing compounds were 11.4 +/- 1.0, 6.5 +/- 0.5, and 1.7 +/- 0.2 mumol kg-1 wet weight, respectively. The time-domain analyses of in vivo 1H MRS data from different brain regions with their specific characteristics demonstrate a case in which the use of frequency-domain methods pose serious difficulties.
PubMed ID
8749730 View in PubMed
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RFLP analysis of three different types of acute intermittent porphyria.

https://arctichealth.org/en/permalink/ahliterature228829
Source
Hum Genet. 1990 Jul;85(2):160-4
Publication Type
Article
Date
Jul-1990
Author
R. Kauppinen
L. Peltonen
A. Palotie
P. Mustajoki
Author Affiliation
Third Department of Medicine, University Central Hospital of Helsinki, Finland.
Source
Hum Genet. 1990 Jul;85(2):160-4
Date
Jul-1990
Language
English
Publication Type
Article
Keywords
Acute Disease
Alleles
Ammonia-Lyases - genetics
Family Health
Female
Finland
Gene Frequency
Haplotypes - genetics
Heterozygote
Humans
Hydroxymethylbilane Synthase - genetics
Male
Pedigree
Polymorphism, Restriction Fragment Length
Porphyrias - classification - enzymology - ethnology - genetics
Abstract
Restriction fragment length polymorphism (RFLP) analysis was performed in three Finnish families with different subtypes of acute intermittent porphyria (AIP): 1) cross-reacting immunological material (CRIM)-negative with low erythrocyte porphobilinogen (PBG)-deaminase activity, 2) CRIM-positive with low PBG-deaminase activity and 3) CRIM-negative with normal PBG-deaminase activity. The disease-associated RFLP haplotype (A2B1C2) of the PBG-deaminase gene was the same in each family. In all three families, RFLP linkage analysis resulted in highly positive lod scores. The maximal lod score (4.3) was obtained at the recombinant fraction of zero, thus confirming a tight linkage of AIP to the PBG-deaminase locus. Of the 62 family members tested, 30 had the disease-associated haplotype; in 5 of them, conventional tests for AIP were normal and in one, uncertain. RFLP analysis can thus reveal new gene carriers and help in the diagnosis of individuals with uncertain results in other laboratory tests.
PubMed ID
1973403 View in PubMed
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9 records – page 1 of 1.