Pediatric-onset multiple sclerosis and other acquired demyelinating syndromes of the central nervous system in Denmark during 1977–2015: A nationwide population-based incidence study

2017 ◽  
Vol 24 (8) ◽  
pp. 1077-1086 ◽  
Author(s):  
Magnus Spangsberg Boesen ◽  
Melinda Magyari ◽  
Nils Koch-Henriksen ◽  
Lau Caspar Thygesen ◽  
Alfred Peter Born ◽  
...  

Background: The incidence of acquired demyelinating syndromes (ADS) including multiple sclerosis (MS) has never been investigated in a Danish pediatric population. Objectives: We estimated the nationwide age- and sex-specific incidence of pediatric ADS including MS. Methods: Data were sourced from the Danish Multiple Sclerosis Registry, providing cases of pediatric MS for 1977–2015, and the National Patient Register, providing cases of ADS during 2008–2015. All medical records were reviewed to validate the register-based diagnoses. Results: We identified 364 cases of pediatric MS occurring during 1977–2015 (incidence rate = 0.79 per 100,000 person-years). MS was exceptionally rare before puberty, but the incidence rose considerably from 9 years in girls and 11 years in boys. The female-to-male ratio was 2.5; the median age at onset was 16 years (range = 7–17 years). The MS incidence rate was relatively stable through the study period. During 2008–2015, we identified 219 ADS cases. The incidence was 2.29 per 100,000 person-years with considerable differences in the age peaks for the separate ADS. Conclusion: The incidence rates of MS and other ADS in Denmark were higher than those reported for some other European countries. Referral bias and classification differences may account for this disparity, in particular the age-intervals and the definition of onset.

Author(s):  
Masoud Etemadifar ◽  
Amir Parsa Abhari ◽  
Ghasem Yadegarfar ◽  
Mehri Salari ◽  
Mohammadreza Ghazavi ◽  
...  

Background: Pediatric-onset multiple sclerosis (POMS) is an autoimmune demyelinating disorder of the central nervous system (CNS), affecting individuals younger than 18 years of age. We sought to characterize the epidemiological and clinical features of patients with POMS in Isfahan, Iran, from April 1997 to March 2020. Methods: The medical records of patients with POMS in the databases of Isfahan Department of Public Health and Isfahan Multiple Sclerosis Society (IMSS) were retrospectively reviewed. The 2006 and 2016 Isfahan Province population censuses were used as reference values for assessing the temporal trend of POMS. Results: From April 1997 to March 2020, 509 individuals under18 years of age were diagnosed with POMS in Isfahan. 404 of these patients (79.4%) were girls, and 105 patients (20.6%) were boys (a female to male ratio of 3.85:1). Most of the patients (83%) were monosymptomatic at onset, with optic neuritis and brainstem-cerebellar disorders being the most frequent initial presentations. Mean ± standard deviation (SD) of age at disease diagnosis was 15.8 ± 2.5 years (ranging from 3 to 18, mode = 18).From April 2019 to March 2020, the crude prevalence and the crude incidence rate of the POMS were  5.42 per 100000 and 1.86 per 100000, respectively. Poisson regression analysis revealed a 3.4% increase in the incidence rate of POMS from April 1997 to March 2020 [relative rate:1.034, 95% confidence interval (CI): 1.021-1.048]. Conclusion: The female to male ratio in our cohort was significantly higher than any other studies conducted previously. The high female to male ratio and increasing incidence of the disease suggest increasing regionalization of care.


2017 ◽  
Vol 48 (1-2) ◽  
pp. 55-60 ◽  
Author(s):  
Angel Chinea ◽  
Carlos F. Ríos-Bedoya ◽  
Cristina Rubi ◽  
Ivonne Vicente ◽  
Eduardo R. Estades ◽  
...  

Background: Multiple sclerosis (MS) affects millions of people worldwide. The estimates for MS incidence are difficult to obtain but are important for understanding MS etiology, developing prevention strategies, and service planning. This study is aimed at estimating the 2014 incidence of MS in Puerto Rico (PR). Methods: The PR MS Foundation's registry was used as the data source. Neurologists, specialty pharmacies, MRI centers, health insurance companies, and the PR Department of Health identified MS cases missed by the registry. MS patients were 18 years and older and met the 2010 revised McDonald criteria. Age-standardized MS incidence rates and their corresponding 95% CIs were computed. Results: A total of 144 new MS cases were diagnosed in 2014. The age-standardized MS incidence rate was 5.1/100,000 (95% CI 4.3-5.9). The incidence rate was 7.1/100,000 (95% CI 5.7-8.5) for females and 2.9/100,000 (95% CI 2.0-3.8) for males. MS cases were mostly females (72.9%) with a mean age of 41.0 years (±1.0). Conclusion: According to the country estimates, PR has a higher MS incidence than other Caribbean and Latin American countries, but a lower incidence compared to countries at higher latitudes. Our findings provide insights into the MS etiology in the Hispanic population that require additional research.


2020 ◽  
pp. bjophthalmol-2020-316796
Author(s):  
Su Kyung Jung ◽  
Jiwon Lim ◽  
Suk Woo Yang ◽  
Young-Joo Won

Background/AimsLymphomas are the most frequent neoplasm of the orbit. However, the epidemiology of orbital lymphomas is not well reported. This study aimed to provide a population-based report on the epidemiology of orbital lymphomas and measure the trends in the incidence of orbital lymphoma cancer in South Korea.MethodsNationwide cancer incidence data from 1999 to 2016 were obtained from the Korea Central Cancer Registry. Age-standardised incidence rates and annual percent changes were calculated according to sex and histological types. The analysis according to the Surveillance, Epidemiology, and End Results summary stage classifications was performed from 2006 to 2016. Survival rates were estimated for cases diagnosed from 1999 to 2016.ResultsA total of 630 patients (median age: 54 years) with orbital lymphoma in the orbital soft tissue were included in this study. The age-standardised incidence rates increased from 0.03 to 0.08 per 100 000 individuals between 1999 and 2016, with an annual percent change of 6.61%. The most common histopathological type of orbital lymphoma was extra marginal zone B cell lymphoma, accounting for 82.2% of all orbital lymphomas during 1999–2016, followed by diffuse large B cell lymphoma (9.2%). Five-year, 10-year and 15-year overall survival (OS) of orbital lymphoma was 90.8%, 83.8% and 75.8%, respectively. OS showed a significant decrease as age increased and no significant differences between men and women.ConclusionThe incidence rate of orbital lymphoma is very low in South Korea. However, the incidence rate has increased over the past years. Orbital lymphomas have a worse prognosis as age increases.


2020 ◽  
Vol 105 (10) ◽  
pp. 3134-3140 ◽  
Author(s):  
Trine Koch ◽  
Elvira V Bräuner ◽  
Alexander S Busch ◽  
Martha Hickey ◽  
Anders Juul

Abstract Context Gynecomastia, the proliferation of mammary glandular tissue in the male, is a frequent but little-studied condition. Available prevalence data are based on selected patient populations or autopsy cases with their inherent bias. Objective The objective of this work is to evaluate the age-related incidence and secular trends in gynecomastia in the general population. Design An observational, 20-year national registry study was conducted. Setting This population-based study used nationwide registry data. Participants Participants included all Danish males (age 0-80 years) with a first-time diagnosis of gynecomastia. Main Outcome Measures All Danish males (age 0-80 years) were followed up for incident diagnosis of gynecomastia in the Danish National Patient Registry from 1998 to 2017 using the International Codes of Diseases, 10th revision, and the Danish Health Care Classification System. Age-specific incidence rates were estimated. The hypothesis tested in this study was formulated prior to data collection. Results Overall, a total 17 601 males (age 0-80 years) were registered with an incident diagnosis of gynecomastia within the 20-year study period, corresponding to 880 new cases per year and an average 20-year incidence of 3.4 per 10 000 men (age 0-80 years). The average annual incidence was 6.5/10 000 in postpubertal males age 16 to 20 years and 4.6/10 000 in males age 61 to 80 years, with a respective 5- and 11-fold overall increase in these 2 age groups over the 20-year period. Conclusions The incidence of gynecomastia has dramatically increased over the last 20 years, implying that the endogenous or exogenous sex-steroid environment has changed, which is associated with other adverse health consequences in men such as an increased risk of prostate cancer, metabolic syndrome, type 2 diabetes, or cardiovascular disorders.


2018 ◽  
Vol 2018 ◽  
pp. 1-10
Author(s):  
Saad Alshahrani ◽  
Amr S. Soliman ◽  
Ahmed Hablas ◽  
Mohamed Ramadan ◽  
Jane L. Meza ◽  
...  

Background. Uterine cancer is one of the top-ranking cancers in women with wide international variations in incidence rates. Developed countries have higher incidence rates than the developing countries. Egypt has significantly lower incidence of uterine cancer than other countries in the Middle East. This study aimed at verifying the incidence rate of uterine cancer and characterizing the demographic and clinical profiles of patients residing in the Gharbiah province in the Nile delta region of Egypt. Methods. Data from 660 uterine cancer patients diagnosed during the period of 1999 to 2010 were abstracted from the Gharbiah Cancer Registry, the only population-based registry in Egypt. The data included age, marital status, number of children, residence, smoking, occupation, date and basis of diagnosis, tumor topography, morphology, stage and grade, and treatment. Crude rate, age-standardized rate (ASR), and age-specific rate were calculated and associated with demographic and clinical characteristics of patients. Results. The study confirmed the low ASR of uterine cancer in Egypt, (4.1 per 100,000 (95% CI: 3.8–4.4)). The incidence rate increased significantly over the 12-year period. The crude rate (CR) was 1.95, 95% CI (1.64–2.25) in 1999–2002; 2.9, 95% CI (2.5–3.2) in 2003–2006; and 3.5, 95% CI (3.1–3.9) in 2007–2010. The rate ratio was 1.5, 95% CI (1.2–1.8) in 2003–2006 and 1.8, 95% CI (1.5–2.2) in 2007–2010 compared to 1999–2002. The majority of patients (83%) were postmenopausal with the highest age-specific rate in the 60–69-year age group (22.07 per 100,000 (95% CI: 19.3–25.2). The majority of patients were diagnosed at early stages (60% localized and 5% regional), had adenocarcinoma (68%), and resided in urban areas (54%). Conclusions. The study confirmed the low incidence rate of uterine cancer in the Gharbiah province of Egypt and significant increase in incidence in recent years. Future studies should focus on verifying the possible effect of hysterectomy on lowering the incidence, the factors related to the changes in rates between rural and urban areas, and the possible impact of nutritional and epidemiologic transitions on the increasing rates.


Rheumatology ◽  
2020 ◽  
Author(s):  
Karin Bengtsson ◽  
Helena Forsblad-d'Elia ◽  
Anna Deminger ◽  
Eva Klingberg ◽  
Mats Dehlin ◽  
...  

Abstract Objectives To estimate the incidence and strength of association of extra-articular manifestations [EAMs, here: anterior uveitis (AU), IBD and psoriasis] in patients with AS, undifferentiated SpA (uSpA) and PsA, compared with controls. Methods Three mutually exclusive cohorts of patients aged 18–69 years with AS (n = 8517), uSpA (n = 10 245) and PsA (n = 22 667) were identified in the Swedish National Patient Register 2001–2015. Age-, sex- and geography-matched controls were identified from the Swedish Population Register. Follow-up began 1 January 2006, or six months after the first SpA diagnosis, whichever occurred later, and ended at the first date of the EAM under study, death, emigration, 70 years of age, and 31 December 2016. Incidence rates (IRs) and incidence rate ratios were calculated for each EAM, and stratified by sex and age. Results Incidence rate ratios for incident AU, IBD and psoriasis were significantly increased in AS (20.2, 6.2, 2.5), uSpA (13.6, 5.7, 3.8) and PsA (2.5, 2.3, n.a) vs controls. Men with AS and uSpA had significantly higher IRs per 1000 person-years at risk for incident AU than women with AS (IR 15.8 vs 11.2) and uSpA (IR 10.1 vs 6.0), whereas no such sex difference was demonstrated in PsA or for the other EAMs. Conclusions AU, followed by IBD and psoriasis, is the EAM most strongly associated with AS and uSpA. Among the SpA subtypes, AS and uSpA display a largely similar pattern of EAMs, whereas PsA has a considerably weaker association with AU and IBD.


2020 ◽  
Vol 6 (3) ◽  
pp. 205521732094776
Author(s):  
Anna Castelo-Branco ◽  
Flaminia Chiesa ◽  
Camilla E Bengtsson ◽  
Sally Lee ◽  
Neil N Minton ◽  
...  

Background Comorbidity is of significant concern in multiple sclerosis (MS). Few population-based studies have reported conditions occurring in MS after diagnosis, especially in contemporary cohorts. Objective To explore incident comorbidity, mortality and hospitalizations in MS, stratified by age and sex. Methods In a Swedish population-based cohort study 6602 incident MS patients (aged ≥18 years) and 61,828 matched MS-free individuals were identified between 1 January 2008 and 31 December 2016, using national registers. Incidence rates (IRs) and incidence rate ratios (IRRs) with 95% CI were calculated for each outcome. Results IRs of cardiovascular disease (CVD) were higher among MS patients than MS-free individuals, (major adverse CVD: IRR 1.42; 95% CI 1.12–1.82; hemorrhagic/ischemic stroke: 1.46; 1.05–2.02; transient ischemic attack: 1.65; 1.09–2.50; heart failure: 1.55; 1.15–2.10); venous thromboembolism: 1.42; 1.14–1.77). MS patients also had higher risks of several non-CVDs such as autoimmune conditions (IRR 3.83; 3.01–4.87), bowel dysfunction (2.16; 1.86–2.50), depression (2.38; 2.11–2.68), and fractures (1.32; 1.19–1.47), as well as being hospitalized and to suffer from CVD-related deaths ((1.91; 1.00–3.65), particularly in females (3.57; 1.58–8.06)). Conclusion MS-patients experience a notable comorbidity burden which emphasizes the need for integrated disease management in order to improve patient care and long-term outcomes of MS.


2017 ◽  
Vol 24 (9) ◽  
pp. 1151-1156 ◽  
Author(s):  
Liesbet M Peeters

Multiple sclerosis (MS) is a progressive demyelinating and degenerative disease of the central nervous system with symptoms depending on the disease type and the site of lesions and is featured by heterogeneity of clinical expressions and responses to treatment strategies. An individualized clinical follow-up and multidisciplinary treatment is required. Transforming the population-based management of today into an individualized, personalized and precision-level management is a major goal in research. Indeed, a complex and unique interplay between genetic background and environmental exposure in each case likely determines clinical heterogeneity. To reach insights at the individual level, extensive amount of data are required. Many databases have been developed over the last few decades, but access to them is limited, and data are acquired in different ways and differences in definitions and indexing and software platforms preclude direct integration. Most existing (inter)national registers and IT platforms are strictly observational or focus on disease epidemiology or access to new disease modifying drugs. Here, a method to revolutionize management of MS to a personalized, individualized and precision level is outlined. The key to achieve this next level is FAIR data.


2008 ◽  
Vol 14 (9) ◽  
pp. 1157-1174 ◽  
Author(s):  
DH Miller ◽  
BG Weinshenker ◽  
M Filippi ◽  
BL Banwell ◽  
JA Cohen ◽  
...  

Background and objectives Diagnosis of multiple sclerosis (MS) requires exclusion of diseases that could better explain the clinical and paraclinical findings. A systematic process for exclusion of alternative diagnoses has not been defined. An International Panel of MS experts developed consensus perspectives on MS differential diagnosis. Methods Using available literature and consensus, we developed guidelines for MS differential diagnosis, focusing on exclusion of potential MS mimics, diagnosis of common initial isolated clinical syndromes, and differentiating between MS and non-MS idiopathic inflammatory demyelinating diseases. Results We present recommendations for 1) clinical and paraclinical red flags suggesting alternative diagnoses to MS; 2) more precise definition of “clinically isolated syndromes” (CIS), often the first presentations of MS or its alternatives; 3) algorithms for diagnosis of three common CISs related to MS in the optic nerves, brainstem, and spinal cord; and 4) a classification scheme and diagnosis criteria for idiopathic inflammatory demyelinating disorders of the central nervous system. Conclusions Differential diagnosis leading to MS or alternatives is complex and a strong evidence base is lacking. Consensus-determined guidelines provide a practical path for diagnosis and will be useful for the non-MS specialist neurologist. Recommendations are made for future research to validate and support these guidelines. Guidance on the differential diagnosis process when MS is under consideration will enhance diagnostic accuracy and precision.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1605-1605
Author(s):  
Malin Hultcrantz ◽  
Therese M-L Andersson ◽  
Ola Landgren ◽  
Paul W Dickman ◽  
Bjorn Andreasson ◽  
...  

Abstract Background The Myeloproliferative neoplasms (MPNs) consists of the subtypes polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF), and MPN unclassifiable (MPN-U). The incidence rates of these diseases vary substantially between different reports, ranging from 1.15 to 4.99/100,000 person-years. However, in a recent metaanalysis, there was no significant difference in MPN incidence between Europe and North America and the variations in incidence may therefore reflect the quality of the cancer registers and reporting of MPNs. In addition, there is a limited number of reports on MPN incidence during more recent years. Therefore, we assessed the incidence of MPN based on the Swedish Cancer Register, a high-quality population-based cancer register between 2000 and 2012. Patients and Methods The Swedish Cancer Register was used to identify all patients diagnosed with an MPN between January 1st 2000 and December 31st 2012. These Swedish Cancer Registers have very high levels of quality and completeness. Between 2008 and 2012, the reporting of newly diagnosed MPN to the cancer register was >92%. Information on the Swedish population was obtained from the Human Mortality Database (www.mortality.org). Based on information from these registers, incidence rates of MPNs with 95% confidence intervals (CIs) were calculated. Confidence intervals were estimated on the log scale. In addition, the incidence rate in relation to MPN subtype, age group (18-39, 40-49, 50-59, 60-69, 70-79, and ³80 years), as well as calendar year of diagnosis was assessed. Results A total of 5,442 MPN patients were reported to the cancer register between 2000 and 2012. During these years, there were 1,810 incident cases of PV, 1,862 of ET, 636 of PMF, and 1,134 with MPN-U. Between January 1st 2000 and December 31st 2012, the population in Sweden increased from 8,861,426 to 9,555,893 inhabitants. The overall annual incidence rate of MPN was 5.83 (95% CI 5.68-5.99)/100,000 persons. The incidence rate of PV was 1.94 (1.85-2.03), ET 2.00 (1.91-2.09), PMF 0.68 (0.63-0.74), and MPN-U 1.22 (1.15-1.29) per 100,000 person-years. In addition, there was a strong correlation between age and incidence of MPN with incidence rates being substantially higher among the older age groups (Table). The overall incidence rate of MPNs increased during the study period, from 5.06 (4.55-5.62)/100,000 person-years in the year 2000 to 5.98 (5.45-6.55)/100,000 person-years in 2012. The incidence rate of PV was similar throughout the study period, the incidence was 2.05 (1.74-2.42)/100,000 person-years in 2000 and 2.12 (1.81-2.47)/100,000 person-years in 2012. The annual incidence rate of ET and PMF increased, from 1.62 (1.34-1.95) to 2.49 (2.15-2.87) per 100,000 persons for ET and from 0.36 (0.24-0.53) to 0.86 (0.67-1.10) per 100,000 persons for PMF between 2000 and 2012. Conversely, the incidence of MPN-U decreased, 1.03 (0.81-1.29) to 0.52 (0.38-0.71)/100,000 person-years between 2000 and 2012. Summary and Conclusions In this large population-based study, the incidence of MPN was higher than previously reported in both European and North American studies. As earlier lower incidence rates likely are an effect of limited coverage of cancer registers, there may be an underreporting of MPNs in many European and American countries. The increase in MPN incidence rates during the study period may reflect increasing life expectancy of the Swedish population, improved reporting to the cancer register as well as changes in the classification and diagnostic systems. Similarly, the decrease in incidence of MPN-U is also likely a result of improved diagnostics during more recent years. In conclusion, the MPN incidences rates reported here are presumably more accurate compared to earlier reports due to the high level of coverage and accuracy of the Swedish registers. Table 1. Incidence rates of MPNs overall and in relation to subtype and age at diagnosis Total number MPN diagnosed 2000-2012 Incidence/100 000 person-years (95% confidence interval) All MPN 5,442 5.83 (5.68-5.99) Subtype PV 1,810 1.94 (1.85-2.03) ET 1,862 2.00 (1.91-2.09) PMF 636 0.68 (0.63-0.74) MPN-U 1,134 1.22 (1.15-1.29) Age at diagnosis (years) 18-39 226 0.67 (0.59-0.76) 40-49 361 2.26 (2.04-2.51) 50-59 769 4.92 (4.58-5.28) 60-69 1,228 9.54 (9.02-10.1) 70-79 1,680 18.99 (18.1-19.9) >80 1,178 18.92 (17.87-20.03) Disclosures Landgren: BMJ Publishing: Honoraria; Bristol-Myers Squibb: Honoraria; Medscape: Honoraria; Onyx: Honoraria; Celgene: Honoraria; International Myeloma Foundation: Research Funding; Medscape: Consultancy; BMJ Publishing: Consultancy; Onyx: Research Funding; Bristol-Myers Squibb: Consultancy; Onyx: Consultancy; Celgene: Consultancy.


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