Variable immunodeficiency score upfront analytical link (VISUAL), a proposal for combined prognostic score at diagnosis of common variable immunodeficiency

AbstractThe broad and heterogeneous clinical spectrum that characterizes common variable immunodeficiency (CVID) is associated with quite different disease course and prognosis, highlighting the need to develop tools that predict complications. We developed a multianalyte VISUAL score (variable immunodeficiency score upfront analytical link) aimed to predict severity using individual CVID patient data at baseline of a cohort of 50 CVID patients from two different centers in Portugal and Spain. We retrospectively applied VISUAL to the CVID clinical severity scores proposed by Ameratunga and Grimbacher after 15 years follow-up of our cohort. VISUAL score at CVID diagnosis showed adequate performance for predicting infectious and non-infectious severe complications (Cluster B). Compared to switched memory B lymphocyte phenotype alone, VISUAL provided a more accurate identification of clinically meaningful outcome, with significantly higher sensitivity (85% vs 55%, p = 0.01), and negative predictive value (77% vs 58%) and AUC of the ROC curves (0.72 vs 0.64), with optimal cut-off level of 10. For every increase of 1 point in the VISUAL scale, the odds of being in the higher risk category (Cluster B) increased in 1.3 (p = 0.005) for Ameratunga’s severity score and 1.26 (p = 0.004) for Grimbacher’s severity score. At diagnosis of CVID, VISUAL score ≥ 10 showed 8.94-fold higher odds of severe prognosis than below this threshold. Kaplan–Meier estimates for the VISUAL ≥ 10 points showed significantly earlier progression to Cluster B than those with VISUAL < 10 (p = 0.0002). This prognostic laboratory score might allow close monitoring and more aggressive treatment in patients with scores ≥ 10 on a personalized basis approach. Further studies are needed to prospectively validate VISUAL score.

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Diagnostic and severity scores for Cockayne syndrome

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01686-8 ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

M. A. Spitz ◽

F. Severac ◽

C. Obringer ◽

S. Baer ◽

N. Le May ◽

...

Keyword(s):

Early Diagnosis ◽

Severity Score ◽

Wide Spectrum ◽

Genetic Disorder ◽

Continuous Distribution ◽

Cockayne Syndrome ◽

Clinical Severity ◽

Diagnostic Score ◽

Clinical Diagnostic ◽

Severity Scores

Abstract Background Cockayne syndrome is a progressive multisystem genetic disorder linked to defective DNA repair and transcription. This rare condition encompasses a very wide spectrum of clinical severity levels ranging from severe prenatal onset to mild adult-onset subtypes. The rarity, complexity and variability of the disease make early diagnosis and severity assessment difficult. Based on similar approaches in other neurodegenerative disorders, we propose to validate diagnostic and severity scores for Cockayne syndrome. Methods Clinical, imaging and genetic data were retrospectively collected from 69 molecularly confirmed CS patients. A clinical diagnostic score and a clinical-radiological diagnostic score for CS were built using a multivariable logistic regression model with a stepwise variable selection procedure. A severity score for CS was designed on five items (head circumference, growth failure, neurosensorial signs, motor autonomy, communication skills) and validated by comparison with classical predefined severity subtypes of CS. Results Short stature, enophtalmos, hearing loss, cataracts, cutaneous photosensitivity, frequent dental caries, enamel hypoplasia, morphological abnormalities of the teeth, areflexia and spasticity were included in the clinical diagnostic score as being the most statistically relevant criteria. Appropriate weights and thresholds were assigned to obtain optimal sensitivity and specificity (95.7% and 86.4% respectively). The severity score was shown to be able to quantitatively differentiate classical predefined subtypes of CS and confirmed the continuous distribution of the clinical presentations in CS. Longitudinal follow-up of the severity score was able to reflect the natural course of the disease. Conclusion The diagnostic and severity scores for CS will facilitate early diagnosis and longitudinal evaluation of future therapeutic interventions. Prospective studies will be needed to confirm these findings.

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Evaluation of liquid or foam sclerotherapy in small varicose veins (ceap c1) with venous clinical severity score

Revista da Associação Médica Brasileira ◽

10.1590/1806-9282.64.12.1117 ◽

2018 ◽

Vol 64 (12) ◽

pp. 1117-1121 ◽

Cited By ~ 2

Author(s):

Mehmet Ali Kaygin ◽

Umit Halici

Keyword(s):

Severity Score ◽

Skin Necrosis ◽

Varicose Veins ◽

Clinical Severity ◽

Foam Sclerotherapy ◽

Clinical Severity Score ◽

Severity Scores ◽

Study Results ◽

Venous Clinical Severity Score ◽

The Mean

SUMMARY OBJECTIVE: We aimed to evaluate the efficacy of liquid or foam sclerotherapy of varicose veins using venous clinical severity scores and possible complications. METHODS: A total of 318 patients (268 females, 50 males) who were treated with liquid or foam sclerotherapy between January 2012 and December 2012 were included in this study. RESULTS: Skin necrosis was observed in only 6 patients (1. 8%), thrombophlebitis in 10 patients (3. 1%), and hyperpigmentation in 18 patients (5. 6%) in this study group. The mean venous clinical severity score was calculated as: pain score, 1. 23 ± 0.88; varicose vein score,1.85 ± 0. 8; edema score, 0.64 ± 0.77). Pain and edema decreased at the control examination, 1 month after completion of sclerotherapy sessions. Varicose veins completely disappeared after sclerotherapy. While the decrease in edema in the foam sclerotherapy group was significantly less (P<0.001), the decline in pain showed an increasing trend (P=0.069). While skin necrosis did not develop after foam sclerotherapy, rates of pigmentation and local thrombophlebitis were similar (P>0.05). CONCLUSION: In conclusion, we observed that both sclerotherapy methods are effective with a low rate of complications, alleviating the complaints of patients with small varicose veins, and providing considerable improvement in venous clinical severity scores.

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Assessing the severity of cardiovascular disease in 213 088 patients with coronary heart disease: a retrospective cohort study

Open Heart ◽

10.1136/openhrt-2020-001498 ◽

2021 ◽

Vol 8 (1) ◽

pp. e001498

Author(s):

Salwa S Zghebi ◽

Mamas A Mamas ◽

Darren M Ashcroft ◽

Martin K Rutter ◽

Harm VanMarwijk ◽

...

Keyword(s):

Cardiovascular Disease ◽

Coronary Heart Disease ◽

Heart Disease ◽

Severity Score ◽

Hospital Admissions ◽

Clinical Severity ◽

Validation Dataset ◽

Operating Characteristics ◽

Baseline Severity ◽

Severity Scores

ObjectiveMost current cardiovascular disease (CVD) risk stratification tools are for people without CVD, but very few are for prevalent CVD. In this study, we developed and validated a CVD severity score in people with coronary heart disease (CHD) and evaluated the association between severity and adverse outcomes.MethodsPrimary and secondary care data for 213 088 people with CHD in 398 practices in England between 2007 and 2017 were used. The cohort was randomly divided into training and validation datasets (80%/20%) for the severity model. Using 20 clinical severity indicators (each assigned a weight=1), baseline and longitudinal CVD severity scores were calculated as the sum of indicators. Adjusted Cox and competing-risk regression models were used to estimate risks for all-cause and cause-specific hospitalisation and mortality.ResultsMean age was 64.5±12.7 years, 46% women, 16% from deprived areas, baseline severity score 1.5±1.2, with higher scores indicating a higher burden of disease. In the training dataset, 138 510 (81%) patients were hospitalised at least once, and 39 944 (23%) patients died. Each 1-unit increase in baseline severity was associated with 41% (95% CI 37% to 45%, area under the receiver operating characteristics (AUROC) curve=0.79) risk for 1 year for all-cause mortality; 59% (95% CI 52% to 67%, AUROC=0.80) for cardiovascular (CV)/diabetes mortality; 27% (95% CI 26% to 28%) for any-cause hospitalisation and 37% (95% CI 36% to 38%) for CV/diabetes hospitalisation. Findings were consistent in the validation dataset.ConclusionsHigher CVD severity score is associated with higher risks for any-cause and cause-specific hospital admissions and mortality in people with CHD. Our reproducible score based on routinely collected data can help practitioners better prioritise management of people with CHD in primary care.

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Outcomes associated with ablation compared to combined ablation and transilluminated powered phlebectomy in the treatment of venous varicosities

Phlebology The Journal of Venous Disease ◽

10.1177/0268355515604257 ◽

2016 ◽

Vol 31 (9) ◽

pp. 618-624 ◽

Cited By ~ 12

Author(s):

Andrea T Obi ◽

Bradley N Reames ◽

Trent J Rook ◽

Sandford O Mouch ◽

Arya Zarinsefat ◽

...

Keyword(s):

Radiofrequency Ablation ◽

Severity Score ◽

Venous Insufficiency ◽

Varicose Veins ◽

Clinical Severity ◽

Severity Scores ◽

Venous Clinical Severity Score ◽

Operative Complications ◽

Thrombotic Complications ◽

Procedure Type

Background Patients with painful varicose veins and venous insufficiency can be treated by eliminating axial reflux only or by eliminating axial reflux plus phlebectomy with transilluminated powered phlebectomy. This study was undertaken with the aim of determining and improving signs and symptoms of venous disease (measured by venous clinical severity score) and complications (by routine surveillance ultrasound and long-term post-operative follow up) for each treatment strategy. Methods We performed a retrospective evaluation of prospectively collected data from 979 limbs undergoing procedures for significant varicose veins and venous insufficiency from March 2008 until June 2014 performed at a single tertiary referral hospital. Patient demographics, Clinical Etiology Anatomy and Pathophysiology classification, venous clinical severity scores pre- and post-procedure, treatment chosen, and peri-operative complications were collected; descriptive statistics were calculated and unadjusted surgical outcomes for patients stratified by the procedure performed. Multivariable logistic regression was used to evaluate the relationship between procedure type and thrombotic complications after adjusting for patient characteristics, severity of disease, pre-operative anticoagulation, and post-operative compression. Result Venous clinical severity scores improved more with radiofrequency ablation + transilluminated powered phlebectomy as compared to radiofrequency ablation alone (3.8 ± 3.4 vs. 3.2 ± 3.1, p = 0.018). Regarding deep venous thrombosis, there was no significant difference between radiofrequency ablation + transilluminated powered phlebectomy vs. radiofrequency ablation alone. There was no statistical difference in asymptomatic endovenous heat-induced thrombosis or infection, although there were slightly more hematomas and cases of asymptomatic superficial thrombophlebitis with combined therapy. On multivariable analysis, only procedure type predicted thrombotic complications. Conclusion Ablation of axial reflux plus transilluminated powered phlebectomy produces improved outcomes as measured by venous clinical severity score, with slight increases in minor post-operative complications and should be strongly considered as initial therapy when patients present with significant symptomatic varicose veins and superficial venous insufficiency. Implementation of a standardized thromboprophylaxis protocol with individual risk assessment results in few significant thrombotic complications amongst high-risk patients, thus potentially obviating the need for routine post-operative duplex.

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Correlation between Chest CT Severity Scores and the Clinical Parameters of Adult Patients with COVID-19 Pneumonia

Radiology Research and Practice ◽

10.1155/2021/6697677 ◽

2021 ◽

Vol 2021 ◽

pp. 1-7

Author(s):

Ghufran Aref Saeed ◽

Waqar Gaba ◽

Asad Shah ◽

Abeer Ahmed Al Helali ◽

Emadullah Raidullah ◽

...

Keyword(s):

Severity Score ◽

Length Of Hospital Stay ◽

Chest Ct ◽

Clinical Severity ◽

Rt Pcr ◽

Original Cohort ◽

Severity Scores ◽

The Mean ◽

Oxygen Requirements ◽

Serum Crp

Purpose. Our aim is to correlate the clinical condition of patients with COVID-19 infection with the 25-point CT severity score by Chang et al. (devised for assessment of ARDS in patients with SARS in 2005). Materials and Methods. Data of consecutive symptomatic patients who were suspected to have COVID-19 infection and presented to our hospital were collected from March to April 2020. All patients underwent two consecutive RT-PCR tests and had a noncontrast HRCT scan done at presentation. From the original cohort of 1062 patients, 160 patients were excluded leaving a total number of 902 patients. Results. The mean age was 44.2 ± 11.9 years (85.3% males, 14.7% females). CT severity score was found to be positively correlated with lymphopenia, increased serum CRP, d-dimer, and ferritin levels ( p < 0.0001 ). The oxygen requirements and length of hospital stay were increasing with the increase in scan severity. Conclusion. The 25-point CT severity score correlates well with the COVID-19 clinical severity. Our data suggest that chest CT scoring system can aid in predicting COVID-19 disease outcome and significantly correlates with lab tests and oxygen requirements.

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The relation between clinical scores and quality-of-life in long-term follow-up

Phlebology The Journal of Venous Disease ◽

10.1177/0268355516631653 ◽

2016 ◽

Vol 31 (1_suppl) ◽

pp. 99-105 ◽

Cited By ~ 4

Author(s):

Fabio S Catarinella ◽

Fred HM Nieman ◽

Cees HA Wittens

Keyword(s):

Quality Of Life ◽

Negative Correlation ◽

Severity Score ◽

Clinical Severity ◽

Venous Disease ◽

Venous Obstruction ◽

Clinical Severity Score ◽

Severity Scores ◽

Venous Clinical Severity Score

Introduction Quality-of-life and severity scores are both popular measures in medicine. For deep venous obstruction, the VEINES-QOL/Sym and venous clinical severity score (VCSS) are widely used. Combining a patient-reported outcome with a clinical severity score should give a more sensitive outcome for treatment results. To establish and compare their suitability for deep venous disease, we compared the outcomes of both scores in a group of patients who were interventionally treated for deep venous disease. Methods The venous clinical severity scores and VEINES-QOL/Sym scores of a group of patients who had received interventional treatment for deep venous obstruction more than 12 months ago were compared at T0 and T12. Results Both the Spearman’s rho and Pearson’s r show a very weak, negative correlation (statistically significant ( p ≤ 0.05)), between the venous clinical severity score and VEINES-QOL at T12 and between the venous clinical severity score and the VEINES-SYM at both T0 and T12. T0 VCSS-VSYM: rho = −0.219 ( p = 0.052), r = −0.236 ( p = 0.037), T12 VCSS-VQOL: rho = −0.459 ( p = 0.007), r = −0.379 ( p = 0.030), T12 VCSS-VSYM: rho = −0.463 ( p = 0.007), r = −0.432 ( p = 0.012). This negative correlation was not statistically ( p ≥ 0.05) confirmed for T0 VCSS-VSYM. Conclusion The physician-scored venous clinical severity score of patients treated for deep venous obstruction does not correlate well with their self-reported quality-of-life and symptom scores.

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Correlation between Chest CT Severity Scores and the Clinical Parameters of Adult Patients with COVID-19 pneumonia

10.1101/2020.10.15.20213058 ◽

2020 ◽

Author(s):

Ghufran Aref Saeed ◽

Waqar Gaba ◽

Asad Shah ◽

Abeer Ahmed Al Helali ◽

Emadullah Raidullah ◽

...

Keyword(s):

Severity Score ◽

Length Of Hospital Stay ◽

Chest Ct ◽

Clinical Severity ◽

Rt Pcr ◽

Original Cohort ◽

Severity Scores ◽

The Mean ◽

Oxygen Requirements ◽

Serum Crp

AbstractPurposeOur aim is to correlate the clinical condition of patients with COVID-19 infection with the 25 Point CT severity score by Chang et al (devised for assessment of ARDS in patients with SARS in 2005).Material and MethodsData of consecutive symptomatic patients who were suspected to have COVID-19 infection and presented to our hospital, was collected from March to April 2020. All patients underwent two consecutive RT-PCR tests and had a non-contrast HRCT scan done at presentation. From the original cohort of 1062 patients, 160 patients were excluded leaving a total number of 902 patients.ResultsThe mean age was 44.2 ±11.9 years [85.3%males, 14.7%females]. CT severity score found to be positively correlated with lymphopenia, increased serum CRP, d-dimer and ferritin levels (p < 0.0001). The oxygen requirements as well as length of hospital stay were increasing with the increase of scan severity.ConclusionThe 25-point CT severity score correlates well with the COVID-19 clinical severity. Our data suggest that chest CT scoring system can aid in predicting COVID-19 disease outcome and significantly correlates with lab tests and oxygen requirements.

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