The severity of malnutrition in children with epidermolysis bullosa correlates with disease severity

Abstract WHO defines malnutrition as severe if the z-scores are less than − 3 Standard deviation (SD), moderate if between − 2 and − 3 SD and mild if between − 2 SD to – 1 SD. This study was aimed to assess nutritional aspects of Indian children suffering from EB and to evaluate the effect of severi...

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Autores principales:	Seema Manjunath, Rahul Mahajan, Dipankar De, Sanjeev Handa, Savita Attri, Banchha Nidhi Behera, Sadhna Lal Bhasin, Rishi Bolia
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/c91fd25fbe804d40a5061f8e02fd12d4
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spelling	oai:doaj.org-article:c91fd25fbe804d40a5061f8e02fd12d42021-12-02T18:51:47ZThe severity of malnutrition in children with epidermolysis bullosa correlates with disease severity10.1038/s41598-021-96354-z2045-2322https://doaj.org/article/c91fd25fbe804d40a5061f8e02fd12d42021-08-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-96354-zhttps://doaj.org/toc/2045-2322Abstract WHO defines malnutrition as severe if the z-scores are less than − 3 Standard deviation (SD), moderate if between − 2 and − 3 SD and mild if between − 2 SD to – 1 SD. This study was aimed to assess nutritional aspects of Indian children suffering from EB and to evaluate the effect of severity of EB on the severity of malnutrition. In this study, pediatric EB patients were evaluated prospectively for baseline nutritional status using anthropometric parameters and WHO growth charts, and its correlation with disease severity using instrument for Scoring Clinical Outcomes for Research of Epidermolysis Bullosa-iscorEB. In second phase, an individualized diet chart was given to meet the energy, protein and micronutrients needs and its effects were observed after 6 months. The median age of participants was 3 years (IQR-9). Of 57 patients, malnutrition was seen in 40.35% patients (22.81%-moderate and 17.54%-severe), and significantly correlated with iscorEB (r = 0.45, p < 0.0001). On bivariate regression analysis, iscorEB was independently associated with moderate-to-severe malnutrition (p = 0.047; OR 1.038, CI 1.011–1.066). iscorEB enabled the identification of patients with moderate-to-severe malnutrition with an Area Under Receiver Operating Curve (AUROC) of 0.72 (95%CI 0.58–0.85; p < 0.005). In phase 2, there was significant improvement in nutritional status in children with recessive dystrophic EB (RDEB) and dominant dystrophic EB (DDEB) subtype (p < 0.0001). The severity of malnutrition in EB children significantly correlates with disease severity, and is an independent predictor of moderate-to-severe malnutrition.Seema ManjunathRahul MahajanDipankar DeSanjeev HandaSavita AttriBanchha Nidhi BeheraSadhna Lal BhasinRishi BoliaNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Seema Manjunath Rahul Mahajan Dipankar De Sanjeev Handa Savita Attri Banchha Nidhi Behera Sadhna Lal Bhasin Rishi Bolia The severity of malnutrition in children with epidermolysis bullosa correlates with disease severity
description	Abstract WHO defines malnutrition as severe if the z-scores are less than − 3 Standard deviation (SD), moderate if between − 2 and − 3 SD and mild if between − 2 SD to – 1 SD. This study was aimed to assess nutritional aspects of Indian children suffering from EB and to evaluate the effect of severity of EB on the severity of malnutrition. In this study, pediatric EB patients were evaluated prospectively for baseline nutritional status using anthropometric parameters and WHO growth charts, and its correlation with disease severity using instrument for Scoring Clinical Outcomes for Research of Epidermolysis Bullosa-iscorEB. In second phase, an individualized diet chart was given to meet the energy, protein and micronutrients needs and its effects were observed after 6 months. The median age of participants was 3 years (IQR-9). Of 57 patients, malnutrition was seen in 40.35% patients (22.81%-moderate and 17.54%-severe), and significantly correlated with iscorEB (r = 0.45, p < 0.0001). On bivariate regression analysis, iscorEB was independently associated with moderate-to-severe malnutrition (p = 0.047; OR 1.038, CI 1.011–1.066). iscorEB enabled the identification of patients with moderate-to-severe malnutrition with an Area Under Receiver Operating Curve (AUROC) of 0.72 (95%CI 0.58–0.85; p < 0.005). In phase 2, there was significant improvement in nutritional status in children with recessive dystrophic EB (RDEB) and dominant dystrophic EB (DDEB) subtype (p < 0.0001). The severity of malnutrition in EB children significantly correlates with disease severity, and is an independent predictor of moderate-to-severe malnutrition.
format	article
author	Seema Manjunath Rahul Mahajan Dipankar De Sanjeev Handa Savita Attri Banchha Nidhi Behera Sadhna Lal Bhasin Rishi Bolia
author_facet	Seema Manjunath Rahul Mahajan Dipankar De Sanjeev Handa Savita Attri Banchha Nidhi Behera Sadhna Lal Bhasin Rishi Bolia
author_sort	Seema Manjunath
title	The severity of malnutrition in children with epidermolysis bullosa correlates with disease severity
title_short	The severity of malnutrition in children with epidermolysis bullosa correlates with disease severity
title_full	The severity of malnutrition in children with epidermolysis bullosa correlates with disease severity
title_fullStr	The severity of malnutrition in children with epidermolysis bullosa correlates with disease severity
title_full_unstemmed	The severity of malnutrition in children with epidermolysis bullosa correlates with disease severity
title_sort	severity of malnutrition in children with epidermolysis bullosa correlates with disease severity
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/c91fd25fbe804d40a5061f8e02fd12d4
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The severity of malnutrition in children with epidermolysis bullosa correlates with disease severity

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