Clinical Features in a Danish Population-Based Cohort of Probable Multiple System Atrophy Patients

2016 ◽  
Vol 46 (4) ◽  
pp. 261-267 ◽  
Author(s):  
Charlotte Starhof ◽  
Lise Korbo ◽  
Christina Funch Lassen ◽  
Kristian Winge ◽  
Søren Friis

Background: Multiple system atrophy (MSA) is a rare, sporadic and progressive neurodegenerative disorder. We aimed to describe the clinical features of Danish probable MSA patients, evaluate their initial response to dopaminergic therapy and examine mortality. Methods: From the Danish National Patient Registry, we identified 782 patients diagnosed with conditions potentially compatible with probable MSA (International Classification of Diseases, version 10 (ICD-10) codes G23.2, G23.8 and G23.9) during 1994-2009. Through medical record review, we narrowed our sample to 115 patients who fulfilled the criteria for probable MSA. We recorded clinical features, examined differences by MSA subtype and used Kaplan-Meier survival analysis to examine mortality. Results: The mean age at onset of patients with probable MSA was 60.2 years (range 36-75 years) and mean time to wheelchair dependency was 4.7 years (range 0-15 years). One-third of patients experienced a transient improvement in motor symptoms with use of levodopa. Median survival from disease onset was 6.9 years (range 1-16 years, 95% CI 6.3-7.5) with no apparent variation according to gender or subtype. Conclusions: Our nationwide approach corroborated that MSA is associated with diverse and grave symptoms, only limited response to levodopa, and poor prognosis.

Lupus ◽  
2021 ◽  
pp. 096120332110142
Author(s):  
Tamer A Gheita ◽  
Rasha Abdel Noor ◽  
Esam Abualfadl ◽  
Osama S Abousehly ◽  
Iman I El-Gazzar ◽  
...  

Objective The aim of this study was to present the epidemiology, clinical manifestations and treatment pattern of systemic lupus erythematosus (SLE) in Egyptian patients over the country and compare the findings to large cohorts worldwide. Objectives were extended to focus on the age at onset and gender driven influence on the disease characteristics. Patients and method This population-based, multicenter, cross-sectional study included 3661 adult SLE patients from Egyptian rheumatology departments across the nation. Demographic, clinical, and therapeutic data were assessed for all patients. Results The study included 3661 patients; 3296 females and 365 males (9.03:1) and the median age was 30 years (17–79 years), disease duration 4 years (0–75 years) while the median age at disease onset was 25 years (4–75 years). The overall estimated prevalence of adult SLE in Egypt was 6.1/100,000 population (1.2/100,000 males and 11.3/100,000 females).There were 316 (8.6%) juvenile-onset (Jo-SLE) and 3345 adult-onset (Ao-SLE). Age at onset was highest in South and lowest in Cairo (p < 0.0001). Conclusion SLE in Egypt had a wide variety of clinical and immunological manifestations, with some similarities with that in other nations and differences within the same country. The clinical characteristics, autoantibodies and comorbidities are comparable between Ao-SLE and Jo-SLE. The frequency of various clinical and immunological manifestations varied between gender. Additional studies are needed to determine the underlying factors contributing to gender and age of onset differences.


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.


Author(s):  
David Liebovitz

Electronic medical records provide potential benefits and also drawbacks. Potential benefits include increased patient safety and efficiency. Potential drawbacks include newly introduced errors and diminished workflow efficiency. In the patient safety context, medication errors account for significant patient harm. Electronic prescribing (e-prescribing) offers the promise of automated drug interaction and dosage verification. In addition, the process of enabling e-prescriptions also provides access to an often unrecognized benefit, that of viewing the dispensed medication history. This information is often critical to understanding patient symptoms. Obtaining significant value from electronic medical records requires use of standardized terminology for both targeted decision support and population-based management. Further, generating documentation for a billable encounter requires usage of proper codes. The emergence of International Classification of Diseases (ICD)-10 holds promise in facilitating identification of a more precise patient code while also presenting drawbacks given its complexity. This article will focus on elements of e-prescribing and use of structured chart content, including diagnosis codes as they relate to physician office practices.


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