scholarly journals Incidence and clinical feature of Myasthenia gravis: A five-year data analysis in Ulaanbaatar, Mongolia

2021 ◽  
Vol 4 (3Suppl) ◽  
pp. 47-54
Author(s):  
Oyunaa Chimedregzen ◽  
Sarangerel Jambal ◽  
Munkhbayar Rentsenbat ◽  
Byambasuren Dagvajantsan
Keyword(s):  
Myasthenia Gravis ◽  
Age Of Onset ◽  
Disease Onset ◽  
Neuromuscular Disorder ◽  
Annual Incidence ◽  
Clinical Feature ◽  
Cross Sectional ◽  
Ocular Symptoms ◽  
Electrophysiological Tests ◽  
Review Period

Myasthenia gravis (MG) is a rare neuromuscular disorder. Till now, there are no studies on the prevalence and incidence of MG in Mongolia. The current study aimed to elucidate the incidence of MG in Ulaanbaatar, the age of onset, and the gender distribution of Mongolian patients with MG. We conducted a cross-sectional, hospital-based study involving MG patients (n=48) all around Ulaanbaatar from 1 January 2015 to 1 January 2020. The clinical diagnosis was assessed with the Myasthenia Gravis Foundation of America (MGFA) classification system. The disease severity was evaluated by using Osserman’s classification. The diagnosis was confirmed with serological and electrophysiological tests. Statistical analysis was performed using SPSS software. A total of 30 patients with MG were registered for the last five years in Ulaanbaatar. The average annual incidence of MG in Ulaanbaatar was 0.65 per 100,000 populations (95%CI 0.26-1.34), 0.60 in males (95%CI 0.25-1.28), and 0.69 in females (95%CI 0.33-1.46). The cumulative incidence in the study period was 3.2 per 100,000 populations. The ratio of males to females was 1:1,3. The median age for onset of MG was 33 years (ranging from 27 to 46 years); 43.3% of patients had ocular and 56.7% generalized symptoms at the disease onset. Only 23.3% of patients remained with purely ocular symptoms (Osserman I stage). The average incidence of MG between 2015 and 2020 was 6,5 per 1.000.000 population, and the annual incidence was relatively stable. Although ocular and generalized symptoms were observed each in about half of the cases, only one-fourth remained with pure ocular signs at the end of the review period.

Adult systemic lupus erythematosus in Egypt: The nation-wide spectrum of 3661 patients and world-wide standpoint

Lupus ◽  
2021 ◽  
pp. 096120332110142
Author(s):  
Tamer A Gheita ◽  
Rasha Abdel Noor ◽  
Esam Abualfadl ◽  
Osama S Abousehly ◽  
Iman I El-Gazzar ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Age Of Onset ◽  
Wide Spectrum ◽  
Age At Onset ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Population Based ◽  
Systemic Lupus ◽  
Cross Sectional

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.

scholarly journals MIND diet associated with later onset of Parkinson's disease

2020 ◽  
Author(s):  
Avril Metcalfe-Roach ◽  
Adam Yu ◽  
Ella Golz ◽  
Kristen Sundvick ◽  
Mihai Cirstea ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Mediterranean Diet ◽  
Dietary Habits ◽  
Age Of Onset ◽  
Disease Onset ◽  
Cross Sectional ◽  
Diet Adherence ◽  
The Mind ◽  
Mind Diet

Background: The MIND diet has been linked with prevention of Alzheimer's disease and cognitive decline but has not been fully assessed in the context of Parkinson's disease (PD). Objective: To determine whether MIND diet adherence is associated with the age of Parkinson's disease onset in a manner superior to that of the Mediterranean diet. Methods: Food Frequency Questionnaires from 167 participants with PD and 119 controls were scored for MIND and two versions of Mediterranean diet adherence. Scores were compared between sex and disease subgroups, and PD diet adherence was correlated with age of onset using univariate and multivariate linear models. Results: The female subgroup adhered more closely to the MIND diet than the males, and diet scores were not modified by disease status. Later age of onset correlated most strongly with MIND diet adherence in the female subgroup, corresponding to differences of up to 17.4 years (p<0.001) between low and high dietary tertiles. Greek Mediterranean adherence was also significantly associated with later PD onset across all models (p=0.05-0.03). Conversely, only Greek Mediterranean adherence remained correlated with later onset across all models in men, with differences of up to 8.4 years (p=0.002). Conclusions: This cross-sectional study finds a strong correlation of age of onset of PD with dietary habits, suggesting that nutritional strategies may be an effective tool to delay PD onset. Further studies may help to elucidate potential nutrition-related sex-specific pathophysiological mechanisms and differential prevalence rates in PD.

FEMALES HAVE EARLIER DISEASE ONSET THAN MALES AMONG HLA-CW6 POSITIVE PSORIASIS PATIENTS

2021 ◽  
pp. 1-2
Author(s):  
Akhilesh Behra
Keyword(s):  
Age Of Onset ◽  
Care Center ◽  
Tertiary Care ◽  
Disease Onset ◽  
Cross Sectional Study ◽  
P Value ◽  
Cross Sectional ◽  
Male And Female ◽  
Female Patients ◽  
Males And Females

Background- Psoriasis is a chronic inammatory relapsing skin disorder. Environmental and genetic factors play an important role in the development of disease. HLA-Cw6 most strongly associated with disease. There is also a difference in HLA-Cw6 positivity in respect to gender, which affect occurrence of disease in males and females. Aims & Objectives- This study was aimed to determine the association of HLA-Cw6 positive and negative psoriasis individuals had any signicant differences in respect to disease onset among male and female Materials & Methods- An Institute based Cross sectional study was done in a tertiary care center in eastern India. All patients attending skin OPD were included in the study. Detailed history and blood samples were collected from patients. HLA-Cw6 typing has done by sequence-specic PCR method. Results- HLA-Cw6 positive female patients had a signicantly early age of onset than male patients (p value-0.009334) (20.88 vs. 27.91yr), while HLA-Cw6 negative patients did not show any signicant difference of age of onset between male and female ( p value- 0.406905) Conclusion- Although men are more commonly affected than female, HLA-Cw6 positive psoriasis female patients show earlier disease onset. This results show that genetic variations in terms of HLA-Cw6 are reected in the age of onset among males and females.

Apolipoprotein E polymorphism and age of onset for Alzheimer's disease in a bi-ethnic sample

2004 ◽  
Vol 16 (3) ◽  
pp. 317-326 ◽  
Author(s):  
Dylan G. Harwood ◽  
Warren W. Barker ◽  
Raymond L. Ownby ◽  
Peter St. George-Hyslop ◽  
Michael Mullan ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Apolipoprotein E ◽  
Age Of Onset ◽  
Disease Onset ◽  
Cross Sectional Study ◽  
Community Dwelling ◽  
Cross Sectional ◽  
Ε4 Allele ◽  
The Impact

Objective: This study examined the association between the Apolipoprotein-E ε4 allele (APOE ε4) and age of disease onset in a bi-ethnic sample of community dwelling Alzheimer's disease (AD) patients.Design: Cross-sectional study of AD patients evaluated at a University-affiliated outpatient memory disorders clinic.Subjects: A clinic-based cohort of white non-Hispanic (WNH; n=601) and white Hispanic (WH; n=359) patients diagnosed with possible or probable AD according to NINCDS-ADRDA diagnostic criteria.Measures: Global cognitive functioning of the subjects was evaluated using the Mini-mental State Exam. The age of onset of AD was calculated from the patient's current age minus the reported duration of disease obtained from a knowledgeable family member.Results: A significant relationship was discovered between APOE ε4 and age of onset for WNH, with lower ages of onset among patients carrying the ε4/ε4 and ε3˜/ε4 genotypes in relation to patients with the ε3/ε3 genotype. The results revealed a more modest effect for APOE genotype in the WH cohort, with a lower age of onset witnessed among ε4 positive patients (ε2/ε4, ε3/ε4 and ε4/ε4 genotypes) in comparison to ε4 negative patients (ε2/ε2, ε2/ε3 and ε3/ε3 genotypes).Conclusion: The association between the ε4 allele and earlier age of onset was more pronounced in WNH compared to WH patients, suggesting the impact of APOE polymorphism on clinical phenotype may be different for distinct ethnic groups in the U.S.

scholarly journals Effect of Polygenic Score on Alzheimer’s Disease Risk Depends on Age and APOE Status

2020 ◽  
Author(s):  
Brian Fulton-Howard ◽  
Alison M. Goate ◽  
Robert P. Adelson ◽  
Jeremy Koppel ◽  
Marc L. Gordon ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Genetic Risk ◽  
Disease Risk ◽  
Age Of Onset ◽  
Low Cost ◽  
Screening Method ◽  
Disease Onset ◽  
Risk Scores ◽  
Cross Sectional

AbstractPolygenic risk scores (PRS) have the potential to serve as a low-cost, non-invasive screening method for Alzheimer’s disease (AD). However, to what extent age and the Apolipoprotein E-ε4 (APOE4) risk allele influence the effect of PRS is underexplored. In a cohort of 346 superager controls (age ≥ 90 years), 2,930 controls (age 60-89) and 1,760 AD cases, we computed APOE-independent PRS for AD. When using superager controls, subjects with PRS in the top decile had nearly five times greater odds of having AD than subjects in the lowest decile (OR=4.91, P=2.24×10−6). In our cross-sectional cohort, PRS modifies the age of onset for AD among APOE4 carriers, but not among non-carriers. Among APOE4 carriers, PRS in the top decile was associated with a five years earlier AD onset than the lowest decile (70.0 vs 75.0 years; t-test P=2.4×10−5). These findings suggest that APOE-independent genetic risk disproportionally affects younger APOE4 carriers, leading to earlier disease onset, while older controls carry less genetic risk.

P020 The male gender predicts the hip involvement among JIA patients

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
Makhlouf Yasmine ◽  
Hanene Ferjani ◽  
Wafa Triki ◽  
Kaouther Maatallah ◽  
Dorra Ben Nessib ◽  
...  
Keyword(s):  
Rheumatoid Factor ◽  
Disease Duration ◽  
Age Of Onset ◽  
Disease Onset ◽  
Limited Range ◽  
Male Gender ◽  
Cross Sectional ◽  
Radiographic Findings ◽  
Limited Range Of Motion ◽  
Significant Difference

Abstract Background Juvenile idiopathic arthritis (JIA) represents a heterogeneous group of chronic arthritides that affect children aged 16 years and under. Regardless of the clinical presentation, hip involvement ranges between 20% to50% of cases [1]. The aim of the present study was to examine the role of gender and disease onset in coxitis involvement. Methods We conducted a cross-sectional study including children with JIA according to the International League of Associations for Rheumatology (ILAR)). Transcribed data included age, sex and the characteristics of the disease (subtype of JIA, disease duration). Regarding coxitis, we collected radiographs, ultrasound (US) and magnetic resonance imaging (MRI) of the hip when performed. Coxitis was defined by clinical (limited range of motion) and/or radiographic findings (destruction, synovitis, bone marrow oedema). We divided patients into two groups: G1: presence of coxitis and G2: absence of coxitis. Results The study included 62 patients with a male perdominance: sex ratio was 2.3. The mean age of onset of the disease was 11.4 years [3–16]. The frequency of each JIA subset was as follows: polyarticular with rheumatoid factor (n = 2), polyarticular without rheumatoid factor (n = 4), systemic (n = 1), enthesitis-related arthritis (n = 44), oligoarthritis (n = 8), psoriatic arthritis (n = 3). A Hip involvement was reported in 71% of cases and was bilateral in 81% of patients. Coxitis was not correlated with the disease duration (P = 0.7). A positive correlation was found between the age of onset of the disease and the presence of coxitis (12.2 vs 9.6, P = 0.005). However, hip involvement was higher among males without significant difference (76.7% vs 58%, P = 0.132). Conclusion Unlike the literature data, our study showed a high frequency of hip involvement in Tunisian children with JIA. We found an association between the male gender and coxitis involvement without significant difference. However, coxitis was more frequent in later onset of the disease.

Celiac Disease in Kosovar Albanian Children: Evaluation of Clinical Features and Diagnosis

2020 ◽  
Vol 16 (3) ◽  
pp. 241-247
Author(s):  
Atifete Ramosaj-Morina ◽  
Alije Keka-Sylaj ◽  
Arbana Baloku Zejnullahu ◽  
Lidvana Spahiu ◽  
Virgjina Hasbahta ◽  
...  
Keyword(s):  
Celiac Disease ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Gastrointestinal Symptoms ◽  
Dominant Mode ◽  
Primary School Children ◽  
Cross Sectional ◽  
Classical Form ◽  
Wide Range ◽  
The Mean

Background: Celiac disease is an immune-mediated disorder characterized by variable clinical manifestations, specific antibodies, HLA-DQ2/DQ8 haplotypes, and enteropathy. Objectives: The aim of this study was to present the clinical spectrum and patterns of celiac disease in Kosovar Albanian children. Methods: A cross-sectional retrospective study was performed with Albanian children aged 0-18 years, treated for celiac disease in the Pediatric Clinic, University Clinical Center of Kosovo from 2005 to 2016. Results: During the study period, 63 children were treated for celiac disease. The mean age at diagnosis was 5.5 years (SD ± 3.31). The mean age at celiac disease onset was 3.3 years (SD ± 2.02), while the mean delay from the first symptoms indicative of celiac disease to diagnosis was 2.2 years (SD ± 2.09). More than 70% of the patients were diagnosed in the first 7 years of life, mainly presented with gastrointestinal symptoms, while primary school children and adolescents mostly showed atypical symptoms (p<0.001). The classical form of celiac disease occurred in 78% of the cases. Sixty (95%) patients carried HLA-DQ2.5, DQ2.2 and/or HLA-DQ8 heterodimers, and only three of them tested negative. Conclusions: Kosovo, as the majority of developing countries, is still facing the classical form of celiac disease as the dominant mode of presentation; as a result, most children with other forms of the celiac disease remain undiagnosed. : Physicians should be aware of the wide range of clinical presentations and utilize low testing thresholds in order to prevent potential long-term problems associated with untreated celiac disease.

scholarly journals Predictors of loss to follow-up in art experienced patients in Nigeria: a 13 year review (2004–2017)

2019 ◽  
Vol 16 (1) ◽  
Author(s):  
Ahmad Aliyu ◽  
Babatunde Adelekan ◽  
Nifarta Andrew ◽  
Eunice Ekong ◽  
Stephen Dapiap ◽  
...  
Keyword(s):  
Patient Adherence ◽  
Cross Sectional Study ◽  
Emergency Plan ◽  
Cross Sectional ◽  
Loss To Follow Up ◽  
Viral Loads ◽  
Review Period ◽  
Lost To Follow Up ◽  
Differentiated Care

Abstract Background Expanded access to antiretroviral therapy (ART) leads to improved HIV/AIDS treatment outcomes in Nigeria, however, increasing rates of loss to follow-up among those on ART is threatening optimal standard achievement. Therefore, this retrospective cross-sectional study is aimed at identifying correlates and predictors of loss to follow-up in patients commencing ART in a large HIV program in Nigeria. Methods Records of all patients from 432 US CDC Presidents Emergency Plan for AIDS Relief (PEPFAR) supported facilities across 10 States and FCT who started ART from 2004 to 2017 were used for this study. Bivariate and multivariate analysis of the demographic and clinical parameters of all patients was conducted using STATA version 14 to determine correlates and predictors of loss to follow-up. Results Within the review period, 245,257 patients were ever enrolled on anti-retroviral therapy. 150,191 (61.2%) remained on treatment, 10,960 (4.5%) were transferred out to other facilities, 6926 (2.8%) died, 2139 (0.9%) self-terminated treatment and 75,041 (30.6%) had a loss to follow-up event captured. Males (OR: 1.16), Non-pregnant female (OR: 4.55), Patients on ≥ 3-monthly ARV refills (OR: 1.32), Patients with un-suppressed viral loads on ART (OR: 4.52), patients on adult 2nd line regimen (OR: 1.23) or pediatric on 1st line regimen (OR: 1.70) were significantly more likely to be lost to follow-up. Conclusion Despite increasing access to anti-retroviral therapy, loss to follow-up is still a challenge in the HIV program in Nigeria. Differentiated care approaches that will focus on males, non-pregnant females and paediatrics is encouraged. Reducing months of Anti-retroviral drug refill to less than 3 months is advocated for increased patient adherence.

scholarly journals Differential Diagnosis of Chorea—HIV Infection Delays Diagnosis of Huntington’s Disease by Years

2021 ◽  
Vol 11 (6) ◽  
pp. 710
Author(s):  
Jannis Achenbach ◽  
Simon Faissner ◽  
Carsten Saft
Keyword(s):  
Hiv Infection ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Onset ◽  
Diagnostic Delay ◽  
Depression Scale ◽  
Cag Repeat ◽  
Psychiatric Disease ◽  
Disease Manifestation ◽  
Cross Sectional

Background: There is a broad range of potential differential diagnoses for chorea. Besides rare, inherited neurodegenerative diseases such as Huntington’s disease (HD) chorea can accompany basal ganglia disorders due to vasculitis or infections, e.g., with the human immunodeficiency virus (HIV). The clinical picture is complicated by the rare occurrence of HIV infection and HD. Methods: First, we present a case suffering simultaneously from HIV and HD (HIV/HD) focusing on clinical manifestation and disease onset. We investigated cross-sectional data regarding molecular genetic, motoric, cognitive, functional, and psychiatric disease manifestation of HIV/HD in comparison to motor-manifest HD patients without HIV infection (nonHIV/HD) in the largest cohort of HD patients worldwide using the registry study ENROLL-HD. Data were analyzed using ANCOVA analyses controlling for covariates of age and CAG repeat length between groups in IBM SPSS Statistics V.25. Results: The HD diagnosis in our case report was delayed by approximately nine years due to the false assumption that the HIV infection might have been the cause of chorea. Out of n = 21,116 participants in ENROLL-HD, we identified n = 10,125 motor-manifest HD patients. n = 23 male participants were classified as suffering from HIV infection as a comorbidity, compared to n = 4898 male non-HIV/HD patients. Except for age, with HIV/HD being significantly younger (p < 0.050), we observed no group differences regarding sociodemographic, genetic, educational, motoric, functional, and cognitive parameters. Male HIV/HD patients reported about a 5.3-year-earlier onset of HD symptoms noticed by themselves compared to non-HIV/HD (p < 0.050). Moreover, patients in the HIV/HD group had a longer diagnostic delay of 1.8 years between onset of symptoms and HD diagnosis and a longer time regarding assessment of first symptoms by the rater and judgement of the patient (all p < 0.050). Unexpectedly, HIV/HD patients showed less irritability in the Hospital Anxiety and Depression Scale (all p < 0.05). Conclusions: The HD diagnosis in HIV-infected male patients is secured with a diagnostic delay between first symptoms noticed by the patient and final diagnosis. Treating physicians therefore should be sensitized to think of potential alternative diagnoses in HIV-infected patients also afflicted by movement disorders, especially if there is evidence of subcortical atrophy and a history of hyperkinesia, even without a clear HD-family history. Those patients should be transferred for early genetic testing to avoid further unnecessary diagnostics and improve sociomedical care.

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