A plastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70–80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000–2011. Patients were included via the National Patient Registry, and diagnosed according to the Camitta criteria. In total, 257 confirmed cases were identified, with an overall incidence of 2.35 (95% CI: 2.06–2.64) cases per million inhabitants per year. Median age was 60 years (range: 2–92), and median follow up was 76 (0–193) months. Primary treatments included immunosuppressive therapy (63%), allogenic stem cell transplantation (10%), or single-agent cyclosporine/no specific therapy (27%). The 5-year survival was 90.7% in patients aged 0–18 years, 90.5% in patients aged 19–39 years, 70.7% in patients aged 40–59 years, and 38.1% in patients aged ≥60 years. Multivariate analysis showed that age (both 40–59 and ≥60 age groups), very severe aplastic anemia and single-agent cyclosporine/no specific therapy were independent risk factors for inferior survival. In conclusion, younger aplastic anemia patients experience a very good long-term survival, while that of patients ≥60 years in particular remains poor. Apparently, the challenge today is to improve the management of older aplastic anemia patients, and prospective studies to address this medical need are warranted.

Background and Purpose-Data from the Malmö Stroke Registry were analyzed to determine whether any change in survival or nonfatal stroke recurrence rates had occurred during the 4-year period from 1989 through 1992 and whether prognosis was related to area of residence. Methods-The series comprised 2290 patients, 1051 men and 1239 women, followed up for 3 years after their first stroke during the period 1989 through 1992. Results-Of the series as a whole, 959(43.4%) died and 137(6%) suffered a second nonfatal stroke. Multivariate analysis showed age, type of stroke, severity of stroke, and the presence of diabetes mellitus or cardiac disease each to be an independent predictor of mortality, and the presence of diabetes, atrial fibrillation, and history of transient ischemic attacks each to be associated with increased risk of recurrence. Treatment for hypertension was associated with a protective effect. As compared to those with first stroke in 1989, those with first stroke in 1992 were characterized by a lower recurrence rate, which was reduced by 70% in the male subgroup (Pϭ0.003) and by 80% in the female subgroup (Pϭ0.006), the corresponding reduction in all-cause mortality being 30% (Pϭ0.007) and 10% (Pϭ0.5, NS).Recurrence-free survival rates differed markedly between the 17 residential areas studied. Conclusions-The present study showed that survival rates after stroke have improved and recurrence rates have declined in this urban population. Further studies are needed to ascertain to what extent intraurban variation in the proportion of recurrence-free 3-year survivors is to be explained by differences in the severity of initial stroke and other prognostic markers, or in initial treatment and secondary preventive measures. (Stroke. 1998;29:2114-2117.)

Abstract. Engstro Èm G, Berglund G, Go Èransson M, Hansen O, Hedblad B, Merlo J, Tyde Ân P, Janzon L (Malmo È University Hospital, Malmo È, Sweden). Distribution and determinants of ischaemic heart disease in an urban population. A study from the myocardial infarction register in Malmo È, Sweden. J Intern Med 2000: 247; 588±596.Objective. Age adjusted incidence of myocardial infarction has been found to vary substantially between the residential areas of the city of Malmo È. The objective of this study was to assess the extent to which major biological risk factors and socioeconomic circumstances account for the differences in incidence of and mortality from myocardial infarction. Design. Ecological study of risk factor prevalence and incidence and mortality from myocardial infarction. Setting. Seventeen administrative areas in Malmo È, Sweden. Subjects. Assessment of risk factor prevalence was based on 28 466 men and women, ranging from 45 to 73 years old, who were recruited as participants in the Malmo È Diet and Cancer study. Information on serum lipids was available in a random subsample of 5362 subjects. Information about socio-economic level of the residential area was based on statistics from the Malmo È City Council and Statistics Sweden. Main outcome measures. Weighted least square regressions between prevalence of risk factors (i.e. smoking, hypertension, obesity, diabetes, hypercholesterolemia and hypertriglyceridemia), a myocardial infarction risk score, a socio-economic score and incidence and mortality from myocardial infarction. Results. The risk factor prevalence and myocardial infarction incidence was highest in areas with low socio-economic level. Prevalence of smoking, obesity and hypertension was significantly associated with myocardial infarction incidence and mortality rates amongst men (all r . 0.60). Prevalence of smoking was significantly associated with incidence and mortality from myocardial infarction amongst women (r = 0.66 and r = 0.61, respectively). A myocardial infarction risk score based on four biological risk factors explained 40±60% of the intra-urban geographical variation in myocardial infarction incidence and mortality. The socio-economic score added a further 2±16% to the explained variance. Conclusion. In an urban population with similar access to medical care, well-known biological cardiovascular risk factors account for a substantial proportion of the intra-urban geographical variation of incidence of and mortality from myocardial infarction. The socio-economic circumstances further contribute to the intra-urban variation in disease.

The present study was undertaken to investigate the prevalence of ascarid infections in Swedish commercial laying hens in 2004 and 2008 following a recent nationwide change to alternative housing systems but before anthelmintics became available. Also, the influence on prevalence of farm and flock characteristics and management was studied in 2004. The results showed that the overall prevalence was significantly higher in 2008 (38%; n 0 64/169) compared with 2004 (24%; n 0 44/186) (P 0 0.001). Ascarid infections were rare in caged flocks, including furnished (enriched) cages, both years (2.4 to 4.3%), and were significantly more common in non-cage systems in both years (16.7 to 48.6% in 2004, and 28.6 to 77.1% in 2008 depending on the housing system). There was no significant difference in prevalence between hens kept on litter indoors and free-range/organic hens. The absence of a hygiene barrier at the entrance of the house or unit increased the risk of infection (P B 0.001), which suggests that parasite eggs were introduced horizontally to the farms. The risk of infection also increased with the age of equipment used in the barn; for example, the risk increased with an odds ratio of 7.5 (95% confidence interval 0 2.3 to 25) when comparing equipment 1 year old with equipment ]7 years old. The results of this study show that ascarid infections may re-emerge following a change to alternative housing. With the impending ban on conventional battery cages in the member states of the European Union, ascarid infections are likely to increase in importance and efficient control options such as hygiene barriers should be implemented on all farms.

Hereditary erythrocytes disorders include a large group of conditions with heterogeneous molecular bases and phenotypes. We analyzed here a case series of 155 consecutive patients with clinical suspicion of hereditary erythrocyte defects referred to the Medical Genetics Unit from 2018 to 2020. All of the cases followed a diagnostic workflow based on a targeted next-generation sequencing panel of 86 genes causative of hereditary red blood cell defects. We obtained an overall diagnostic yield of 84% of the tested patients. Monogenic inheritance was seen for 69% (107/155), and multi-locus inheritance for 15% (23/155). PIEZO1 and SPTA1 were the most mutated loci. Accordingly, 16/23 patients with multi-locus inheritance showed dual molecular diagnosis of dehydrated hereditary stomatocytosis/xerocytosis and hereditary spherocytosis. These dual inheritance cases were fully characterized and were clinically indistinguishable from patients with hereditary spherocytosis. Additionally, their ektacytometry curves highlighted alterations of dual inheritance patients compared to both dehydrated hereditary stomatocytosis and hereditary spherocytosis. Our findings expand the genotypic spectrum of red blood cell disorders and indicate that multi-locus inheritance should be considered for analysis and counseling of these patients. Of note, the genetic testing was crucial for diagnosis of patients with a complex mode of inheritance.