Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh, India.
Department of Pediatric Biochemistry, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh, India.
Sci Rep. 2021 Aug 19;11(1):16827. doi: 10.1038/s41598-021-96354-z.
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.
如果 z 分数小于-3 个标准差（SD），则为严重；如果在-2 和-3 SD 之间，则为中度；如果在-2 SD 到-1 SD 之间，则为轻度。本研究旨在评估患有 EB 的印度儿童的营养状况，并评估 EB 的严重程度对营养不良严重程度的影响。在这项研究中，使用人体测量参数和世界卫生组织生长图表对儿科 EB 患者进行前瞻性基线营养状况评估，并使用表皮松解症研究临床结局评分工具（iscorEB）评估疾病严重程度与营养状况的相关性。在第二阶段，给予个体化饮食图表以满足能量、蛋白质和微量营养素的需求，并在 6 个月后观察其效果。参与者的中位年龄为 3 岁（IQR-9）。在 57 名患者中，有 40.35%（22.81%-中度，17.54%-重度）的患者存在营养不良，且与 iscorEB 显著相关（r=0.45，p<0.0001）。在二元回归分析中，iscorEB 与中重度营养不良独立相关（p=0.047；OR 1.038，CI 1.011-1.066）。iscorEB 能够以 0.72 的曲线下面积（95%CI 0.58-0.85；p<0.005）识别出中重度营养不良的患者。在第二阶段，隐性营养不良型 EB（RDEB）和显性营养不良型 EB（DDEB）亚型的儿童营养状况显著改善（p<0.0001）。EB 儿童的营养不良严重程度与疾病严重程度显著相关，是中重度营养不良的独立预测因素。
