scholarly journals Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort

Rheumatology ◽  
2020 ◽  
Vol 59 (10) ◽  
pp. 2920-2929 ◽  
Author(s):  
Håvard Fretheim ◽  
Anne-Kristine Halse ◽  
Marit Seip ◽  
Helle Bitter ◽  
Marianne Wallenius ◽  
...  

Abstract Objective SSc is a severe, heterogeneous multi-organ disease where population-based estimates on phenotypic spectrum, overall disease burden and societal impact are largely missing. Here the objective was to provide the first-ever complete national-level data on phenotype and major organ afflictions in SSc. Methods A stepwise strategy was applied to find and characterize every SSc patient resident in Norway from 2000 to 2012. First we identified every case in the country registered with an International Classification of Diseases, Tenth Revision code for SSc (M34). Next we manually reviewed all cases coded as M34 to determine whether they met the 1980 ACR and/or 2013 ACR/EULAR classification criteria for SSc and could be included in the Norwegian SSc cohort (Nor-SSc). Finally, all disease features from SSc onset to study end were reviewed. Results The Nor-SSc cohort included 815 SSc patients. The mean age at diagnosis was 53 years, with 84% females and 77% limited cutaneous SSc. The estimated incidence increased from 4 per million in 2000 to 13 per million in 2012. We identified high cumulative frequencies of internal organ involvement, coexistence of multiple organ afflictions across disease subsets and autoantibody status and stable frequencies of pulmonary arterial hypertension across haemodynamic definitions, but indications of referral-related differences in pulmonary hypertension detection rates across the study area. Conclusion This nationwide cohort study provides new, unbiased evidence for a high disease burden in SSc patients of Caucasian descent and indicates the existence of hurdles preventing equality of assessment across the SSc population.

Author(s):  
Almudena Ávalos Marfil ◽  
Enriqueta Barranco Castillo ◽  
Raúl Martos García ◽  
Nicolás Mendoza Ladrón de Guevara ◽  
Maryna Mazheika

A retrospective population-based study aimed to assess the incidence of endometriosis in the general population in Spain and in each of its autonomous communities. The authors used the incidence of diagnosed endometriosis in the minimum basic dataset at discharge in the national hospital discharge registry of Spain. This analysis was carried out with hospital data with a diagnosis of endometriosis (International Classification of Diseases (ICD)-9 code 617.xx and ICD-10 code N80.xx) and covered the period from 1 January 2014 to 31 December 2017 and a population of 12,775,911 women of reproductive age (15–54 years). The data were then analyzed at the national level and separately for each autonomous community. This nationwide Spanish study estimated the overall incidence of endometriosis among autonomous communities in Spain to be 16.1 per 10,000 women (range, 6.8 to 24). The mean age of the 20,547 women diagnosed with endometriosis during the study period was 36.8 ± 5.4 years. The types (proportions) of endometriosis were uterine (28.4%), tubo-ovarian (35.2%), peritoneal (8.1%), vesical (6.8%) and intestinal (3.2%) endometriosis. Further studies are needed to assess the reasons for the decrease in the observed incidence and for the significant differences in the regional incidence rates of this disease.


2021 ◽  
pp. svn-2021-000909
Author(s):  
Yixin Sun ◽  
Guoyu Zhou ◽  
Jingnan Feng ◽  
Lu Chen ◽  
Guozhen Liu ◽  
...  

Background and objectiveMoyamoya disease (MMD) is an increasingly recognised cause of stroke, mainly described in East Asia. China is the largest nation in Asia, but few studies reported the epidemiology of MMD, especially at a national level. We aimed to estimate the incidence and prevalence of MMD in China.MethodsWe performed a population-based study using data from the national databases of Urban Basic Medical Insurance between 2013 and 2016, covering approximately 0.50 billion individuals. MMD cases were identified by diagnostic code (International Classification of Diseases, 10th Revision I67.5) or related diagnostic text.ResultsA total of 1987 MMD patients (mean age 44.45±14.30 years, female-to-male ratio 1.12) were identified, representing a national crude incidence of 0.59 (95% CI: 0.49 to 0.68) and a prevalence of 1.01 (95% CI: 0.81 to 1.21) per 100 000 person-years in 2016. Rates were higher in females than in males for the incidence (0.66 vs 0.52) and prevalence (1.05 vs 0.90). And the age-specific rates showed a bimodal distribution, with the highest peak in middle-aged group and the second peak in child group.ConclusionsOur results confirm that MMD is relatively common in East Asians, but the rates in China were lower than those in other East Asian countries such as Japan and Korea. The unique epidemiological features, including a relatively weak female predominance and a shift in the highest peak of incidence from children to adults, revealed new sight into MMD. Further research is expected to explore the potential pathogenesis of MMD.


Heart ◽  
2021 ◽  
pp. heartjnl-2021-319129
Author(s):  
Marios Rossides ◽  
Susanna Kullberg ◽  
Johan Grunewald ◽  
Anders Eklund ◽  
Daniela Di Giuseppe ◽  
...  

ObjectivesPrevious studies showed a strong association between sarcoidosis and heart failure (HF) but did not consider risk stratification or risk factors to identify useful aetiological insights. We estimated overall and stratified HRs and identified risk factors for HF in sarcoidosis.MethodsSarcoidosis cases were identified from the Swedish National Patient Register (NPR; ≥2 International Classification of Diseases-coded visits, 2003–2013) and matched to general population comparators. They were followed for HF in the NPR. Treated were cases who were dispensed ≥1 immunosuppressant ±3 months from the first sarcoidosis visit (2006–2013). Using Cox models, we estimated HRs adjusted for demographics and comorbidity and identified independent risk factors of HF together with their attributable fractions (AFs).ResultsDuring follow-up, 204 of 8574 sarcoidosis cases and 721 of 84 192 comparators were diagnosed with HF (rate 2.2 vs 0.7/1000 person-years, respectively). The HR associated with sarcoidosis was 2.43 (95% CI 2.06 to 2.86) and did not vary by age, sex or treatment status. It was higher during the first 2 years after diagnosis (HR 3.7 vs 1.9) and in individuals without a history of ischaemic heart disease (IHD; HR 2.7 vs 1.7). Diabetes, atrial fibrillation and other arrhythmias were the strongest independent clinical predictors of HF (HR 2.5 each, 2-year AF 20%, 16% and 12%, respectively).ConclusionsAlthough low, the HF rate was more than twofold increased in sarcoidosis compared with the general population, particularly right after diagnosis. IHD history cannot solely explain these risks, whereas ventricular arrhythmias indicating cardiac sarcoidosis appear to be a strong predictor of HF in sarcoidosis.


Author(s):  
Hua Wang ◽  
Ke Chai ◽  
Minghui Du ◽  
Shengfeng Wang ◽  
Jian-Ping Cai ◽  
...  

Background: Large-scale and population-based studies of heart failure (HF) incidence and prevalence are scarce in China. The study sought to estimate the prevalence, incidence, and cost of HF in China. Methods: We conducted a population-based study using records of 50.0 million individuals ≥25 years old from the national urban employee basic medical insurance from 6 provinces in China in 2017. Incident cases were individuals with a diagnosis of HF (International Classification of Diseases code, and text of diagnosis) in 2017 with a 4-year disease-free period (2013–2016). We calculated standardized rates by applying age standardization to the 2010 Chinese census population. Results: The age-standardized prevalence and incidence were 1.10% (1.10% among men and women) and 275 per 100 000 person-years (287 among men and 261 among women), respectively, accounting for 12.1 million patients with HF and 3.0 million patients with incident HF ≥25 years old. Both prevalence and incidence increased with increasing age (0.57%, 3.86%, and 7.55% for prevalence and 158, 892, and 1655 per 100 000 person-years for incidence among persons who were 25–64, 65–79, and ≥80 years of age, respectively). The inpatient mean cost per-capita was $4406.8 and the proportion with ≥3 hospitalizations among those hospitalized was 40.5%. The outpatient mean cost per-capita was $892.3. Conclusions: HF has placed a considerable burden on health systems in China, and strategies aimed at the prevention and treatment of HF are needed. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: ChiCTR2000029094.


2018 ◽  
Vol 160 (3) ◽  
pp. 559-566 ◽  
Author(s):  
Ying-Shuo Hsu ◽  
Wei-Chung Hsu ◽  
Jenq-Yuh Ko ◽  
Te-Huei Yeh ◽  
Chia-Hsuan Lee ◽  
...  

Objective To investigate readmissions among adult inpatients who underwent uvulopalatopharyngoplasty (UPPP) in Taiwan. Design Population-based survey. Setting Retrospective study with the National Health Insurance Database. Methods All cases of inpatient adult UPPP (age >20 years) from 1997 to 2012 were identified through International Classification of Diseases, Ninth Revision, Clinical Modification. Factors associated with readmission within 30 days after surgery were analyzed. Results A total of 38,839 adults with UPPP were identified (mean age, 39.3 years; men, 73.7%). The incidence of UPPP was 14.6 per 100 000 adults, which increased from 1997 to 2012 (6.7 to 16.7 per 100,000, Ptrend < .001). The rates of readmission for any reason, readmission for bleeding, reoperation for bleeding, and 30-day mortality were 4.2%, 1.7%, 1.0%, and 0.14%, respectively. Young age increased the risk of reoperation for bleeding, and old age increased the risk of readmission for any reason and mortality. Men had an increased risk of readmission and reoperation. Hypertension was associated with an increased risk of readmission for any reason (odds ratio [OR], 1.29; 95% CI, 1.10-1.51), bleeding-related readmission (OR, 1.89; 95% CI, 1.52-2.36), and reoperation (OR, 2.47; 95% CI, 1.84-3.30). Concurrent hypopharyngeal surgery was associated with an increased risk of readmission for any reason (OR, 1.34; 95% CI, 1.07-1.66) and bleeding-related readmission (OR, 1.69; 95% CI, 1.25-2.27). Finally, the use of steroids was associated with an increased risk of bleeding-related readmission and reoperation. Conclusions The incidence of adult UPPP increased from 1997 to 2012 in Taiwan. Age, sex, comorbidity, concurrent hypopharyngeal surgery, and drug administration were associated with readmission after inpatient UPPP.


2017 ◽  
Vol 42 (7) ◽  
pp. 673-677 ◽  
Author(s):  
J. Nordenskjöld ◽  
M. Englund ◽  
C. Zhou ◽  
I. Atroshi

The prevalence and incidence of doctor-diagnosed Dupuytren’s disease in the general population is unknown. From the healthcare register for Skåne region (population 1.3 million) in southern Sweden, we identified all residents aged ⩾20 years (on 31 December 2013), who 1998 to 2013 had consulted a doctor and received the diagnosis Dupuytren’s disease (International Classification of Diseases 10th Revision code M720). During the 16 years, 7207 current residents (72% men) had been diagnosed with Dupuytren’s disease; the prevalence among men was 1.35% and among women 0.5%. Of all people diagnosed, 56% had received treatment (87% fasciectomy). In 2013, the incidence of first-time doctor-diagnosed Dupuytren’s disease among men was 14 and among women five per 10,000. The annual incidence among men aged ⩾50 years was 27 per 10,000. Clinically important Dupuytren’s disease is common in the general population. Level of evidence: III


Author(s):  
Farah N. Chowdhury ◽  
G. Sanjaya Chandrarathne ◽  
Kristopher D. Masilamani ◽  
Jennifer T. N. LaBranche ◽  
Shaun Malo ◽  
...  

AbstractBackgroundHereditary hemorrhagic telangiectasia (HHT) is a disease of abnormal vasculature where patients are predisposed to strokes of multiple etiologies. We assessed yearly stroke incidence among Albertans with HHT and compared with the general population. Given the tendency for stroke in HHT patients, we expected HHT patients to have higher stroke incidence, in particular at younger ages.MethodsPopulation-based administrative health data on inpatient and ambulatory admissions were extracted over a 16-year period using International Classification of Diseases (ICD)-9 and ICD-10, Canada codes. We analyzed overall occurrence of strokes in Alberta by age, gender, stroke subtype, and diagnosis of HHT.ResultsThe age-standardized incidence rate of stroke in HHT was 450 per 100,000 compared with 260 per 100,000 in the general population with a rate ratio of 1.73 (95% confidence interval (CI) [1.046-2.842]). This study found a higher HHT prevalence in Alberta (1 in 3800) compared to the world average of 1 in 5000. Women were also more likely to be diagnosed with HHT, with a 3.25:1 female gender preponderance in the yearly incidence.InterpretationThis study not only shows that HHT patients are at higher risk of having a stroke but also quantifies that risk using an age-adjusted metric in Alberta. This province has a higher than expected disease burden of HHT, with the majority of cases affecting women. Our study found that acute ischemic strokes and transient ischemic attacks are far more common than hemorrhage in HHT. As HHT is a rare, multi-system, chronic disease, these patients should be referred to an HHT Centre of Excellence.


BMJ Open ◽  
2018 ◽  
Vol 8 (1) ◽  
pp. e019113 ◽  
Author(s):  
Jenny Bourke ◽  
Kingsley Wong ◽  
Helen Leonard

ObjectivesTo investigate how well intellectual disability (ID) can be ascertained using hospital morbidity data compared with a population-based data source.Design, setting and participantsAll children born in 1983–2010 with a hospital admission in the Western Australian Hospital Morbidity Data System (HMDS) were linked with the Western Australian Intellectual Disability Exploring Answers (IDEA) database. The International Classification of Diseases hospital codes consistent with ID were also identified.Main outcome measuresThe characteristics of those children identified with ID through either or both sources were investigated.ResultsOf the 488 905 individuals in the study, 10 218 (2.1%) were identified with ID in either IDEA or HMDS with 1435 (14.0%) individuals identified in both databases, 8305 (81.3%) unique to the IDEA database and 478 (4.7%) unique to the HMDS dataset only. Of those unique to the HMDS dataset, about a quarter (n=124) had died before 1 year of age and most of these (75%) before 1 month. Children with ID who were also coded as such in the HMDS data were more likely to be aged under 1 year, female, non-Aboriginal and have a severe level of ID, compared with those not coded in the HMDS data. The sensitivity of using HMDS to identify ID was 14.7%, whereas the specificity was much higher at 99.9%.ConclusionHospital morbidity data are not a reliable source for identifying ID within a population, and epidemiological researchers need to take these findings into account in their study design.


2018 ◽  
Vol 25 (8) ◽  
pp. 1092-1101 ◽  
Author(s):  
José MA Wijnands ◽  
Feng Zhu ◽  
Elaine Kingwell ◽  
Yinshan Zhao ◽  
Okechukwu Ekuma ◽  
...  

Background: The multiple sclerosis (MS) prodrome is poorly characterized. Objective: To phenotype the MS prodrome via health care encounters. Methods: Using data from a population-based cohort study linking administrative and clinical data in four Canadian provinces, we compared physician and hospital encounters and prescriptions filled (via International Classification of Diseases chapters, physician specialty or drug classes) for MS subjects in the 5 years before the first demyelinating claim in an administrative cohort or the clinical symptom onset in an MS clinic-derived cohort, to age-, sex- and geographically matched controls. Rate ratios (RRs), 95% confidence intervals (95% CIs) and proportions were estimated. Results: The administrative and clinical cohorts included 13,951/66,940 and 3202/16,006 people with and without MS (cases/controls). Compared to controls, in the 5 years before the first demyelinating claim or symptom onset, cases had more physician and hospital encounters for the nervous (RR (range) = 2.31; 95% CI: 1.05–5.10 to 4.75; 95% CI: 3.11–7.25), sensory (RR (range) = 1.40; 95% CI: 1.34–1.46 to 2.28; 95% CI: 1.72–3.02), musculoskeletal (RR (range) = 1.19; 95% CI: 1.07–1.33 to 1.70; 95% CI: 1.57–1.85) and genito-urinary systems (RR (range) = 1.17; 95% CI: 1.05–1.30 to 1.59; 95% CI: 1.48–1.70). Cases had more psychiatrist and urologist encounters (RR (range) = 1.48; 95% CI: 1.36–1.62 to 1.80; 95% CI: 1.61–2.01), and higher proportions of musculoskeletal, genito-urinary or hormonal-related prescriptions (1.1–1.5 times higher, all p < 0.02). However, cases had fewer pregnancy-related encounters than controls (RR = 0.78; 95% CI: 0.71–0.86 to 0.88; 95% CI: 0.84–0.92). Conclusion: Phenotyping the prodrome 5 years before clinical recognition of MS is feasible.


Author(s):  
Ana Villaverde-Hueso ◽  
Germán Sánchez-Díaz ◽  
Francisco J. Molina-Cabrero ◽  
Elisa Gallego ◽  
Manuel Posada de la Paz ◽  
...  

The aim of this study is to analyze population-based mortality attributed to cystic fibrosis (CF) over 36 years in Spain. CF deaths were obtained from the National Statistics Institute, using codes 277.0 from the International Classification of Diseases (ICD) ninth revision (ICD9-CM) and E84 from the tenth revision (ICD10) to determine the underlying cause of death. We calculated age-specific and age-adjusted mortality rates, and time trends were assessed using joinpoint regression. The geographic analysis by district was performed by standardized mortality ratios (SMRs) and smoothed-SMRs. A total of 1002 deaths due to CF were identified (50.5% women). Age-adjusted mortality rates fell by −0.95% per year between 1981 and 2016. The average age of death from CF increased due to the annual fall in the mortality of under-25s (−3.77% males, −2.37% females) and an increase in over-75s (3.49%). We identified districts with higher than expected death risks in the south (Andalusia), the Mediterranean coast (Murcia, Valencia, Catalonia), the West (Extremadura), and the Canary Islands. In conclusion, in this study we monitored the population-based mortality attributed to CF over a long period and found geographic differences in the risk of dying from this disease. These findings complement the information provided in other studies and registries and will be useful for health planning.


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