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P39 Nutritional status in patients with different forms of epidermolysis bullosa
  1. Makarova S,
  2. Namazova-Baranova L,
  3. Murashkin N,
  4. Yasakov D,
  5. Epishev R,
  6. Petrovskaya M,
  7. Ereshko O,
  8. Chumbadze T
  1. Federal State Autonomous Institution ‘National Scientific and Practical Centre of Children’s Health’ Of the Ministry of Health of the Russian Federation

Abstract

Background and aims Epidermolysis Bullosa (EB) refers to a group of rare inherited disorders characterised by skin and/or mucous blistering even after minor injuries. Nutritional deficiency in EB, especially in dystrophic form, is the result of associated states such as: dental/gum diseases, oral, pharyngealandoesophageal blistering, microstomia, fixed tongue, dysphagia, oesophageal stricture, gastro-oesophageal reflux.

Study Objective To assess the nutritional status of children suffering from different forms of EB.

Materials and methods The study included 60 children aged 1–16 years suffering from Epidermolysis Bullosa Simplex (EBS) (n=17) and Dystrophic Epidermolysis Bullosa (DEB) (n=43). The assessment of physical development indices was carried out using WHO ANTRO+ computer program. Z-score values (weight index (WAZ), height index (HAZ), body mass index (BAZ)) within > −2 /<+1 are a normal. BAZ indices > −3/<-2 represent a moderate degree of nutritional deficiency, < −3 – a severe one. Comprehensive assessment of the factors affecting the nutritional status in EB was performed using the THINC validated scale (Tool to Help Identify Nutritional Compromise).

Results WAZ, HAZ and BAZ in children with EBS were in normal limits in most of patients (82.3%), 2 children had undernutrition (BAZ > −3/< −2), 1 had BAZ >+1.

WAZ in patients with DEB was within normal limits only in 16 children (37.2%), increased (>+1) – in 1 child (2.3%). Other children had a decreased WAZ: within > −3/< −2 in 11 children (25.5%), and below −3 in 15 children (34.8%). HAZ was within normal limits in 26 children (60.5%), increased (>+1) – in 3 children (7.0%), decreased within > −3/< −2 – in 9 children (20.9%), below −3 – in 5 children (11.6%). BAZ was within normal limits in 13 children (30.2%). The moderate nutritional deficiency (BAZ >-3/<-2) was detected in 11 (25.5%), and severe nutritional deficiency (BAZ below −3) – in 18 children (41.8%).

THINC scale values ranged from 10 to 55 (Me 30±10.47) in children with EBS; from 25 to 95 (Me 60±17.63) in patients with DEB.

Conclusions The performed analysis shows evident disorders of the nutritional status in children with a dystrophic form of EB. The THINC tool should be used for a comprehensive assessment of nutritional status and nutritional compromise, effectiveness of the administered treatment, including dietary therapy, over time.

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