scholarly journals Development and validation of a severity scoring system for Zellweger spectrum disorders

2017 ◽  
Vol 93 (3) ◽  
pp. 613-621 ◽  
Author(s):  
F.C.C. Klouwer ◽  
A. Meester-Delver ◽  
F.M. Vaz ◽  
H.R. Waterham ◽  
R.C.M. Hennekam ◽  
...  
Keyword(s):  
Scoring System ◽  
Severity Scoring ◽  
Spectrum Disorders ◽  
Zellweger Spectrum Disorders ◽  
Development And Validation

scholarly journals SAT0541 THE SEVERITY OF FMF MAY BE ASSOCIATED WITH CO-MORBIDITIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1227.3-1228
Author(s):  
M. E. Tezcan ◽  
N. Şen ◽  
M. Yilmaz ◽  
Ö. Volkan ◽  
E. Tükel ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Scoring System ◽  
Disease Duration ◽  
Severe Disease ◽  
Smoking History ◽  
Disease Group ◽  
Severity Scoring ◽  
Mild Disease ◽  
Co Morbidity ◽  
Mediterranean Fever

Background:Familial Mediterranean fever (FMF) is an auto inflammatory disease with recurrent attacks of serositis. Frequent attacks and disease related sequels may be associated with co-morbidities in FMF patients.Objectives:One of the tools for evaluating the FMF severity is the international severity scoring system for FMF (ISSF)1. This score includes disease related sequels, acute phase measurements, attack features and exertional leg pain. Therefore, more severe disease may be link with subclinical inflammation, amyloidosis and frequent, prolonged and widespread attacks. All these components may augment the frequency of non-disease related co-morbidities.Methods:We enrolled 158 FMF patients who fulfilled modifiedTel-HashomerDiagnosisCriteria2. The patients dichotomized based upon disease severity (mild disease or severe disease). Patients with ISSF scores lower or equal to 2 were accepted to have mild disease. Then, we compared frequency of non-disease related co-morbidities between the groups. These co-morbidities arehypertension, hypothyroidism, hyperthyroidism cardiovascular diseases, coronary artery diseases, cerebrovascular diseases, chronic renal disease (non-FMF related), chronic obstructive pulmonary diseases, and diabetes mellitus. This study was approved by the Local Research Ethics Committee and carried out in compliance with the Helsinki Declaration. All the patients gave written informed consent. P-value lower than 0.05 was considered as statistically significant.Results:Demographic features, disease duration, smoking history and body mass index (BMI) were similar between the groups. Frequency of co-morbidity in severe disease group was statistically higher than mild disease group (p=0.02). Most frequent co-morbidity was hypertension in both groups.Table.Features of mild and severe FMF groupsMild (n=135)Severe (n=23)pGender (M/F)47/8811/120.23Age36.4±11.336.5±14.30.68Smoking (%)38 (28.1)5 (21.7)0.52BMI (kg/m2)24.3±9.224.0±8.90.34Disease duration (year)7.7±11.38.6±14.30.09Amyloidosis (%)2 (1.4)3 (13.0)0.02Exon 10 homozygote (%)35 (25.9)9 (39.1)0.19Colchicine dosage (mg/day)1.2±0.41.4±0.50.02ISSF scores0.7 ±0.73.4±0.5<0.001Co-morbidity (%)25 (18.5)9 (39.1)0.02Conclusion:In our FMF patient cohort, we found that severity of the disease may be associated with higher frequency of co-morbidities. Therefore, clinicians should be aware of the high possibility of co-morbidities in patients with more severe FMF and addressed these co-morbidities timely and properly.References:[1]Demirkaya E, et al. Development and initial validation of international severity scoring system for familial Mediterranean fever (ISSF). Ann Rheum Dis 2016;75:1051-6.[2]Berkun Y, et al. Diagnostic criteria of familial Mediterranean fever. Autoimmun Rev 2014;13:388-90.Acknowledgments:NoneDisclosure of Interests:None declared

scholarly journals Correlation between age, sex, and severity of Coronavirus disease-19 based on chest computed tomography severity scoring system

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
Shimaa Farghaly ◽  
Marwa Makboul
Keyword(s):  
Computed Tomography ◽  
Scoring System ◽  
Fatal Outcome ◽  
Age Groups ◽  
Significant Risk ◽  
Chest Ct ◽  
Age Group ◽  
Severity Scoring ◽  
Chest Computed Tomography ◽  
Severity Of The Disease

Abstract Background Coronavirus disease 2019 (COVID-19) is the most recent global health emergency; early diagnosis of COVID-19 is very important for rapid clinical interventions and patient isolation; chest computed tomography (CT) plays an important role in screening, diagnosis, and evaluating the progress of the disease. According to the results of different studies, due to high severity of the disease, clinicians should be aware of the different potential risk factors associated with the fatal outcome, so chest CT severity scoring system was designed for semi-quantitative assessment of the severity of lung disease in COVID-19 patients, ranking the pulmonary involvement on 25 points severity scale according to extent of lung abnormalities; this study aims to evaluate retrospectively the relationship between age and severity of COVID-19 in both sexes based on chest CT severity scoring system. Results Age group C (40–49 year) was the commonest age group that was affected by COVID-19 by 21.3%, while the least affected group was group F (≥ 70 years) by only 6.4%. As regards COVID-RADS classification, COVID-RADS-3 was the most commonly presented at both sexes in all different age groups. Total CT severity lung score had a positive strong significant correlation with the age of the patient (r = 0.64, P < 0.001). Also, a positive strong significant correlation was observed between CT severity lung score and age in both males and females (r = 0.59, P < 0.001) and (r = 0.69, P < 0.001) respectively. Conclusion We concluded that age can be considered as a significant risk factor for the severity of COVID-19 in both sexes. Also, CT can be used as a significant diagnostic tool for the diagnosis of COVID-19 and evaluation of the progression and severity of the disease.

Development of a severity scoring system for acute haemorrhage in anaesthetized domestic cats: the CABSS score

2020 ◽  
Vol 47 (4) ◽  
pp. 499-508
Author(s):  
Gareth E. Zeiler ◽  
Andrea Fuller ◽  
Eva Rioja ◽  
Peter Kamerman ◽  
Roxanne K. Buck ◽  
...  
Keyword(s):  
Scoring System ◽  
Domestic Cats ◽  
Severity Scoring ◽  
Acute Haemorrhage

scholarly journals A novel PEX1 mutation in a Moroccan family with Zellweger spectrum disorders

2017 ◽  
Vol 4 (1) ◽  
Author(s):  
Amale Bousfiha ◽  
Amina Bakhchane ◽  
Hicham Charoute ◽  
Zied Riahi ◽  
Khalid Snoussi ◽  
...  
Keyword(s):  
Moroccan Family ◽  
Spectrum Disorders ◽  
Zellweger Spectrum Disorders
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