Assessment of Growth Hormone and Insulin-like Growth Factor 1 in Children with Epidermolysis Bullosa Dystrophica

Abstract

Abstract Epidermolysis bullosa dystrophica (EBD) is characterized by muco-cutaneous fragility with blistering, scarring and severe growth retardation attributed to many factors. This cross-sectional study included 51 patients aged 1-12 years with EBD. Weight and height were measured, with the calculation of weight standard deviation score (SDS), height SDS, and body mass index (BMI), followed by plotting them on Egyptian growth curves. Serum levels of basal growth hormone (GH), insulin like growth factor 1 (IGF-1), hemoglobin (HB) level, erythrocyte sedimentation rate (ESR), and thyroid functions (TSH and T4) were measured. Growth hormone stimulation test was performed in 10 patients. Weight SDS and height SDS were significantly lower than normal measurements (P< 0.05*). Growth hormone, growth hormone stimulation, and IGF-1 were significantly lower than the normal range (P< 0.05*). HB levels were significantly lower than normal, while ESR levels were significantly elevated (P <0.001*). A negative correlation was found between ESR and basal GH, and a positive correlation between ESR and IGF1. In conclusion, children with generalized DEB have poor growth and low circulating GH and IGF-1 levels, likely due to malnutrition, anemia, and inflammation that suppresses GH/IGF-1 axis. Future treatments targeting the correction of GH and IGF1 levels and anti-inflammatory treatment should be considered.