A complication risk score to evaluate clinical severity of thalassaemia syndromes
| Autor: | Aurelio Maggio, Vito Di Marco, Mahmoud Hajipour, Shahina Daar, Saqib Hussain Ansari, Amal El-Beshlawy, Gabriella Dardanoni, Salvatore Scondotto, Alessia Pepe, Aldo Filosa, Sylvia T. Singer, Zaki A Naserullah, Fedele Bonifazi, Antonella Meloni, Elliott Vichinsky, Walter Addario Pollina, Angela Vitrano, Mehran Karimi, Adriana Ceci, Paolo Ricchi |
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| Přispěvatelé: | Vitrano A., Meloni A., Addario Pollina W., Karimi M., El-Beshlawy A., Hajipour M., Di Marco V., Hussain Ansari S., Filosa A., Ricchi P., Ceci A., Daar S., Titi Singer S., Naserullah Z.A., Pepe A., Scondotto S., Dardanoni G., Bonifazi F., Vichinsky E., Maggio A. |
| Rok vydání: | 2020 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent complications thalassaemia complication risk score Logistic regression Severity of Illness Index Group A Group B Hemoglobins Young Adult 03 medical and health sciences 0302 clinical medicine Risk Factors Internal medicine medicine Humans prognostic model Blood Transfusion Clinical severity Hemoglobin Framingham Risk Score Ejection fraction Receiver operating characteristic business.industry Hematology Middle Aged Prognosis Chelation Therapy Thalassemia ROC Curve 030220 oncology & carcinogenesis Female Complication business 030215 immunology |
| Zdroj: | British Journal of Haematology. 192:626-633 |
| ISSN: | 1365-2141 0007-1048 |
| Popis: | The thalassaemia syndromes (TS) show different phenotype severity. Developing a reliable, practical and global tool to determine disease severity and tailor treatment would be of great value. Overall, 7910 patients were analysed with the aim of constructing a complication risk score (CoRS) to evaluate the probability of developing one or more complications. Nine independent variables were included in the investigation as predictors. Logistic regression models were used for Group A [transfusion-dependent thalassaemia (TDT)], Group B [transfused non-TDT (NTDT)] and Group C (non-transfused NTDT). Statistically significant predictors included age (years), haemoglobin levels, hepatic transaminases [alanine aminotransferase (ALT) and aspartate aminotransferase] and left-ventricular ejection fraction (LVEF) for Group A; age (years), age at first chelation (months), ALT and LVEF for Group B; and age (years), mean serum ferritin (SF) levels and LVEF for Group C. The area under the receiver operating characteristic curve was 84·5%, 82·1% and 80·0% for Groups A, Group B and Group C respectively, suggesting the models had good discrimination. Finally, the CoRS for each group was categorised into four risk classes (low, intermediate, high, and very high) using the centiles of its distribution. In conclusion, we have developed a CoRS for TS that can assist physicians in prospectively tailoring patients’ treatment. |
| Databáze: | OpenAIRE |
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