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Declining coronary heart disease mortality in Iceland: contribution by incidence, recurrence and case fatality rate.

OBJECTIVE: To analyse to what extent the recent decline in coronary heart disease mortality in Iceland is due to changes in incidence, recurrence and case fatality rates. DESIGN: A countrywide registration of myocardial infarction (MI) in people aged 25-74 was performed in Iceland during 1981-1999 according to the MONICA protocol. Possible cases were found by review of all hospital discharge records, autopsy records and death certificates. RESULTS: MI death rate declined by 63% in males and 51% in females, most in the youngest age groups in men (86%) and least in the oldest (49%). In women there was not a significant difference in age groups. Overall the age-adjusted reduction in MI death rate was 55.4% in both sexes combined; of this 23.1% was due to incidence reduction, 22.8% to recurrence reduction and 11.6% to case fatality reduction. In the youngest age groups the decline in incidence contributed most to the decline in MI death rate (62% in men and 71% in women), but thereafter the decline in case fatality in men. In the older age groups decline in recurrence rate has greater weight. CONCLUSION: The recent decline in MI mortality under the age of 75 years in Iceland is due to reduction in incidence and recurrence rate by about 40% each and to reduction in case fatality by 20%.

Adult↗

Stroke in Icelandic patients with hereditary amyloid angiopathy is related to a mutation in the cystatin C gene, an inhibitor of cysteine proteases.

Cystatin C is an inhibitor of lysosomal cysteine proteases and consists of 120 amino acids. A variant of cystatin C lacking the first NH2-terminal residues and having one amino acid substitution at position 68 forms amyloid deposits mainly in the walls of brain arteries, causing fatal strokes in Icelandic patients with familial cerebral hemorrhage secondary to a form of an autosomal dominant amyloidosis. To understand the molecular basis of the genetic defect, the gene encoding cystatin C was isolated from genomic DNA libraries made from normal tissue and the brain of an Icelandic patient with hereditary cerebral hemorrhage with amyloidosis (HCHWA-I). The data indicate that the cystatin C gene encodes a polypeptide of 146 amino acids, of which the first 26 correspond to a secretory peptide signal sequence. The gene contains two intervening sequences that interrupt the coding region at amino acids 55 and 93. Comparison with genes encoding salivary cystatins and kininogen proteins show sequence homology and conservation of exon-intron structure. Except for a mutation in the second exon (CAG instead of CTG in the normal gene, resulting in the substitution of glutamine for a leucine residue), the gene cloned from the brain of the Icelandic patient is identical to the normal cystatin C gene. Thus, HCHWA-I is the first familial type of amyloidosis related to a point mutation in a gene encoding for an inhibitor. The mutation in the structural gene encoding cystatin C appears to be the primary defect in this inherited disorder causing amyloid fibril formation and accumulation followed by cerebral hemorrhage.

Amino Acid Sequence↗

Diversity of penicillin-nonsusceptible Streptococcus pneumoniae circulating in Iceland after the introduction of penicillin-resistant clone Spain(6B)-2.

After the introduction and extensive dissemination of the multidrug-resistant Streptococcus pneumoniae clone Spain(6B)-2 between 1989 and the early to mid-1990s, the prevalence of pneumococcal isolates expressing intermediate resistance to penicillin, mainly of capsular types 6, 19, and 23, also began to increase in Iceland. The purpose of this study was to investigate whether these isolates originated in Iceland or represented strains imported to the country. Isolates were characterized by pulsed-field gel electrophoresis; multilocus sequence typing; determination of pbp1a, pbp2b, and pbp2x gene restriction patterns; and partial sequencing of these pbp genes. The results indicate that, although singular events suggesting horizontal transfer of pbp genes (and capsular genes) were detected, the majority of clones circulating in the country had genetic backgrounds also detected abroad. The major mechanism of dissemination of penicillin resistance in Iceland appears to be the repeated introduction of multiple lineages, followed by clonal spread.

Bacterial Capsules↗

Craniofacial skeletal and soft tissue morphology in Icelandic adults.

The purpose of the study was to describe the craniofacial characteristics of Icelandic adults on lateral skull cephalograms. The material consisted of 155 (47.8%) males and 169 (52.5%) females. The mean ages were 35.5 and 34.2 years, respectively. Twenty-two skeletal reference points and 11 soft tissue points were digitized and processed by standard methods with the Dentofacial Planner computer software program. The 45 variables calculated were both angular and linear. Two-sample t-tests were used to study the differences between sexes. Mandibular prognathism was significantly greater in males (P < or = 0.05), but the inclination of both the upper and lower jaws was greater in females (P < or = 0.01). Linear measurements were usually larger for males. The lips were less protrusive in males (P < or = 0.01), but the thickness was greater compared with females (P < or = 0.001). The nose was significantly more protrusive in males (P < or = 0.001). When the Icelandic sample was compared with closely related ethnic groups, such as the Swedes and the Danes, it was interesting to note that the Icelanders seem to be more like the Swedes than the Danes.

Adult↗

Craniofacial changes in Icelandic children between 6 and 16 years of age - a longitudinal study.

The aim of the present study was to describe the craniofacial changes between 6 and 16 years of age in a sample of Icelandic children. Complete sets of lateral cephalometric radiographs were available from 95 males and 87 females. Twenty-two reference points were digitized and processed by standard methods, using the Dentofacial Planner computer software program. Thirty-three angular and linear variables were calculated, including: basal sagittal and vertical measurements, facial ratio, and dental, cranial base and mandibular measurements. For the angular measurements, gender differences were not statistically different for any of the measurements, in either age group, except for the variable s-n-na, which was larger in the 16-year-old boys (P < or = 0.001). Linear variables were consistently larger in the boys compared with the girls at both age levels. During the observation period mandibular prognathism increased but the basal sagittal jaw relationship, the jaw angle, the mandibular plane angle and cranial base flexure (n-s-ba) decreased in both genders (P < or = 0.001). Maxillary prognathism increased only in the boys from 6 to 16 years. Inclination of the lower incisors and all the cranial base dimensions increased in both genders during the observation period. When the Icelandic sample was compared with a similar Norwegian sample, small differences could be noted in the maxillary prognathism, mandibular plane angle and in the inclination of the maxilla. Larger differences were identified in the inclination of the lower incisors. These findings could be used as normative cephalometric standards for 6- and 16-year-old Icelandic children.

Adolescent↗

Cancer incidence among Icelandic pesticide users.

BACKGROUND: This study was done to examine the cancer risk among pesticide users in Iceland. METHODS: We have followed a cohort of 2449 licensed pesticide users, students from a horticultural college, members of a pension fund for market gardeners, horticulturists and vegetable farmers up until the end of 1993 in the Icelandic Cancer Registry of cancer incidence. The observed number of cancers was compared with expected values calculated on the basis of cancer incidence for males and females in Iceland. RESULTS: The standardized incidence ratio (SIR) for all cancer sites was 0.80. Among females the increased incidence for cancer of lymphatic and haematopoietic tissue was significant (SIR = 5.56, 95% confidence interval (CI) 1.12-16.23). The incidence of rectal cancer was three times that expected (SIR = 2.94, 95% CI: 1.07-6.40), and this cancer was even more predominant among the licensed pesticides users (SIR = 4.63, 95% CI: 1.49-10.80). All cancers of the rectum were adenocarcinoma, however, one was adenocarcinoma in villous adenoma and one adenocarcinoma in tubulo-villous adenoma. CONCLUSION: The results provide some support for the suggestion that pesticide exposure may lead to cancer of the lymphatic and haematopoietic tissue in females. We suggest that some of the pesticides to which the licensed pesticide users were exposed may lead to rectal cancer.

Aged↗

Violent behaviour among adolescents in Iceland: a national survey.

BACKGROUND: Violence among adolescents may have serious developmental, physical, and mental health consequences for the affected individuals. In this study, we assessed the prevalence of violent behaviour and its correlates among 15-16 year old schoolchildren in Iceland. METHODS: In 1997, a cross-sectional survey was conducted among a random half of all Icelandic schoolchildren aged 15-16 years. The overall response rate was 91% (N = 3872). In the present study, socio-demographic background, social support, negative life events, psychological distress, and substance use were considered in relation to violent behaviour using logistic regression techniques. RESULTS: The majority of the respondents reported having committed violence within the last year. Boys were more likely to use violence than girls (odds ratio [OR] = 5.6; 95% CI: 4.7, 6.6). Respondents who had experienced >/=4 negative life events in the past year were more likely to use violence than respondents with no negative life events (OR = 3.0; 95% CI: 2.2, 4.2). Smokers were more likely than non-smokers to use violence (OR = 1.7; 95% CI: 1.2, 2.2), and adolescents who had used alcohol >20x in their lifetime were more than twice as likely to commit violence compared with those who had never used alcohol (OR = 2.5; 95% CI: 1.8, 3.4). CONCLUSION: Rates of violent behaviour among Icelandic schoolchildren were high. Gender, parental support, life stress, anger/aggression, and substance use were all significantly related to the perpetration of violent acts.

Adolescent↗

Evidence for the introduction of a multiresistant clone of serotype 6B Streptococcus pneumoniae from Spain to Iceland in the late 1980s.

Almost all of the multiresistant pneumococci that appeared suddenly in clinical specimens in Iceland between 1989 and 1992 belonged to serogroup 6. Fifty-seven of these isolates were analyzed for serotype, penicillin-binding protein pattern, multilocus enzyme genotype, and fragmentation pattern obtained by pulsed-field electrophoretic separation of restriction enzyme digests of chromosomal DNA. All isolates were of serotype 6B and had similar or identical patterns in each molecular test. The Icelandic isolates were indistinguishable from a subgroup of multiresistant serotype 6B pneumococci that has been present with high incidence in Spain during the past two decades. The data suggest the import to Iceland of a single multiresistant clone of pneumococcus, most likely from Spain.

Bacterial Proteins↗

Inflammatory bowel disease in Iceland, 1990-1994: a prospective, nationwide, epidemiological study.

BACKGROUND: Two separate nationwide, retrospective epidemiological studies of inflammatory bowel disease (IBD) in Iceland over a combined 40-year period, 1950-1989, have shown a continually rising incidence. The main objective of this study was to investigate the incidence of IBD prospectively for the 5-year period 1990-1994 to determine whether there still was an ongoing increase. METHODS: The retrieval of new cases of ulcerative colitis (UC) and Crohn's disease (CD) was based on a monthly review of all small and large intestinal tissue specimens with any type of inflammation submitted to all three departments of pathology in Iceland. All small intestinal X-ray records suggestive of CD were also reviewed. All possible new cases of IBD were then scrutinized by examination of the clinical records, using accepted criteria for confirmation or exclusion of IBD. RESULTS: A total of 215 cases of UC and 72 cases of CD were diagnosed, yielding a mean annual incidence for UC 16.5/100000 (95% confidence interval (CI) 14.4-18.9), and for CD 5.5/100000 (95% CI 4.3-7.0). The highest combined age related incidence for UC was 28.7/100000 in the group aged 30-39 years, and for CD 8.5/100000 in the groups aged 10-19 and 20-29 years. The most common extent in UC was proctosigmoid and the most frequent localization in CD was colonic only. CONCLUSIONS: This prospective study demonstrates a continuing and statistically significant increase in the incidence of both UC and CD in Iceland. The general changes identified for a few previously suggested environmental factors do not allow any firm conclusions as to their role in the observed increase in incidence. It is possible that there are some other causative factor(s).

Adolescent↗

Genotype-phenotype interactions in Wilson's disease: insight from an Icelandic mutation.

Wilson's disease, an autosomal recessive disorder of copper transport, usually presents with symptoms from the liver or central nervous system. Rarely, the initial manifestation is fulminant hepatic failure. The abnormal gene (ATP7B) is located on chromosome 13q and encodes a copper-transporting ATPase. A large number of mutations have been reported. We describe a previously healthy 16-year-old girl who presented with fulminant hepatic failure. The girl died within 24 h of admission to a hospital from refractory shock. Autopsy revealed cirrhosis and widespread necrosis of the liver. The copper content of the liver was markedly increased (975 micrograms/g dry weight), strongly suggesting a diagnosis of Wilson's disease. Genetic studies revealed that the girl was homozygous for the mutation 2007 del7, which is the mutation found in all Wilson's disease patients previously identified in Iceland. This is the first known case of fulminant hepatic failure due to Wilson's disease in Iceland. Despite the same mutation, the clinical picture is vastly different from other Icelandic patients with Wilson's disease, who all presented with relatively late-onset neurological disease. This suggests that factors other than the specific mutation have significant impact on the phenotype of the disease.

Adolescent↗

The saga of cystatin C gene mutation causing amyloid angiopathy and brain hemorrhage--clinical genetics in Iceland.

Firstly, we review investigations of hereditary cystatin C amyloid angiopathy, which is caused by a mutation in the cystatin C gene. Symptoms of brain haemorrhages, which lead to death in young adults, are the hallmark of this disorder. The mutation can now be detected by the RFLP method using Alu I restriction enzyme and cystatin C cDNA probe. Secondly, we give an overview of other clinical genetic studies in Iceland with emphasis on activities initiated or sponsored by the Genetical Committee of the University of Iceland. The list of references covers most publications on genetic studies of Icelanders.

Amyloidosis↗

Psychometric properties of the Multidimensional Anxiety Scale for Children (MASC) among Icelandic schoolchildren.

A psychometric assessment of the Icelandic version of the Multidimensional Anxiety Scale for Children (MASC) was undertaken among Icelandic schoolchildren, between 10 and 15 years of age. In a first study 625 children between 10 and 15 years of age filled in the MASC. A confirmatory factor analysis indicated that the four-factor structure of the instrument was justified. Further, means and standard deviations of the total scale and the subscales were similar to the original normative data reported by March (1997). In a second study the MASC, the Children Depression Inventory (CDI) and the Revised Children's Manifest Anxiety Scale (RCMAS) were administered to a sample of 249 schoolchildren between 10 and 15 years of age. The results supported the convergent and divergent validity of the MASC. Together the two studies show satisfactory psychometric properties of the Icelandic version of the MASC in a normal population.

Adolescent↗

Orthography as a handicap? A direct comparison of spelling acquisition in Danish and Icelandic.

Spelling of cross-linguistically very similar nonwords was compared in 115 Danish and 77 Icelandic children (primarily 3rd and 4th graders). Danish children made more errors than Icelandic children on word medial consonant doublets and on word initial consonant clusters, even when the groups compared were matched on simpler spelling tasks. These results suggest that the acquisition of phonemic encoding skills is slower in "deep" orthography such as Danish than in more "transparent" orthography such as Icelandic. The effect of orthography was expected for consonant doublets because of the relatively more complex sound-letter correspondences in Danish. For consonant clusters, however, sound-letter correspondences are perfectly regular in both languages. The study thus points to the conclusion that even the mastery of regular sound-letter correspondences may be delayed in deep orthography.

Adult↗

Onychomycosis in Icelandic children.

BACKGROUND: Onychomycosis is a rare disorder in children. Few studies exist on the incidence or prevalence of onychomycosis in children. OBJECTIVE: To examine the epidemiology of childhood onychomycosis in Iceland during the period 1982-2000. METHODS: Results from all mycological samples taken from children in Iceland from 1982 to 2000 were examined. Information about the requesting physician, unique social security number, date of birth, sex, results of culture and microscopy were registered. Growth of a dermatophyte was taken as an indication of a case of onychomycosis. RESULTS: During the period 1982-2000 a total of 493 samples from 408 Icelandic children, aged 0-17 years, were examined. Dermatophytes were cultured from 148 (30.0%) samples. During the period 1982-85, the mean annual incidence of positive cultures was 1.65 per 100,000, increasing to 21.30 per 100,000 for the years 1996-2000. Trichophyton rubrum was the dominating organism and was found in 102/148 cases positive for the growth of a dermatophyte. The incidence of positive dermatophyte cultures increased with age and was found in eight children aged 0-4, and in 57 children aged 10-14 years. CONCLUSION: Onychomycosis is rare in children, but increases with age. It seems that onychomycosis increased during the study period, but it is not clear if this was due to a true increase in the prevalence of onychomycosis or an increased awareness of onychomycosis, or both.

Adolescent↗

Possible changes in the frequency of the human ABO blood groups in Iceland due to smallpox epidemics selection.

The hypothesis is put forward that the low frequency of A and high frequency of O blood group genes in the Icelandic human population is the result of a selective disadvantage of A during severe smallpox epidemics. The hypothesis is supported by data from India in 1965-6, which show a marked selective effect of a smallpox epidemic against the phenotypes A and AB (Vogel & Chakravartti, 1971). The conclusion is drawn that the present-day ABO blood group gene frequencies of the Icelandic population should be used with reservation as markers in the study of the origin of the Icelanders.

ABO Blood-Group System↗

Pregnancies of women with epilepsy: a population-based study in Iceland.

PURPOSE: Women with epilepsy who become pregnant are commonly considered to be at high risk for complications during pregnancy or delivery. The offspring are also considered to have increased risk of perinatal mortality, congenital malformations, and maturational delay. Because few of these studies are population based, potential bias exists because of selection. METHODS: We performed a historical population-based cohort study in Iceland to determine the prevalence of epilepsy among pregnant women, to identify pregnancy and delivery complications in women with epilepsy, and to determine the outcome of their pregnancies as compared with that in the general population of Iceland. We identified all women with active epilepsy who gave birth during a 19-year period in Iceland. RESULTS: In this population, 3.3 in 1,000 pregnancies involve mothers with active epilepsy. The frequency of adverse events (AE) during pregnancy in the women with epilepsy is similar to that observed among all live births in the population, but cesarean section was performed twice as frequently as in the general population. Perinatal mortality rate and mean birth weight are not significantly different in the offspring of women with epilepsy as compared with rest of the population. The risk of major congenital malformations (MGM) is increased 2.7-fold over that expected when a mother is treated with antiepileptic drugs (AEDs) during a pregnancy. CONCLUSIONS: Our study indicates that the rate of complications of pregnancy in mothers with active epilepsy is low and similar to that of the general population with epilepsy. Use of AEDs by the mother during pregnancy significantly increases the risk of MGM in the offspring.

Abnormalities, Drug-Induced↗

Risk of epilepsy in patients with multiple sclerosis: a population-based study in Iceland.

PURPOSE: Several clinical series reported an association between multiple sclerosis (MS) and epilepsy. We conducted a total population study in Iceland to determine the risk for developing epilepsy in patients with MS compared with that expected in the general population. METHODS: Medical records of the 188 incidence cases of clinically definite MS first diagnosed in Iceland during the 25-year study period (1965-1989) were reviewed. The cases were followed up through 1994 or until death to identify those developing seizures or epilepsy. The expected number of cases with epilepsy in the MS-incidence cohort were calculated based on the age-specific incidence for epilepsy in Iceland and the age-specific person years of follow-up in the MS cohort. RESULTS: During the 2,771 person years of observation after diagnosis of clinically definite MS, three MS patients developed epilepsy. One additional case developed epilepsy after onset of MS symptoms but before diagnosis of MS. The cumulative incidence of epilepsy by 10 years after diagnosis of MS was 1.9%. Given the age-specific person years of follow-up after diagnosis of MS, only one case of epilepsy would have been expected; standardized incidence ratio (SIR), 3.0 (95% confidence interval (CI), 0.6-8.8). CONCLUSIONS: MS is a risk factor for developing epilepsy. Patients with MS have a threefold increase in risk for developing epilepsy when compared with that expected in the general population. The reason for this increased risk is unclear and needs further investigation.

Adult↗

Prevalence of epilepsy in rural Iceland: a population-based study.

PURPOSE: To determine the prevalence of epilepsy (recurrent unprovoked seizures) in the population of rural Iceland. METHODS: Review of records of primary-care facilities for the population of rural Iceland supplemented by review of referrals to electroencephalographic facilities and neurologic specialists to identify all individuals with unprovoked seizures or receiving treatment for epilepsy in the calendar year 1993. RESULTS: Crude prevalence was 4.8 per 1,000 and was 4.8/1,000 after age adjustment to the 1970 United States population. Age-adjusted prevalence was higher in male (5.1) than in female subjects (4.4) and increased with advancing age. Sixty-two percent of prevalence cases were of unknown cause. Sixty-three percent of cases had major motor seizures without aura or generalized epileptiform pattern on EEG. Sixty-four percent of patients had had seizures in the year of observation. Seizure control was inversely correlated with number of medications. Ten percent of the prevalence group was taking no medication, although the frequency of seizures was high in this medication-free group. CONCLUSIONS: Prevalence of epilepsy in rural Iceland is consistent with that reported in recent studies in other developed countries. The data provide insights into the approaches of treatment and use of specialized diagnostic testing in the country. The prevalence is similar to that in other European countries but lower than that reported from rural areas in Latin America.

Adolescent↗