Nutritional and metabolic profile of children with epidermolysis bullosa.
The clinical features of epidermolysis bullosa (EB) include oral, pharyngeal, gastrointestinal, and total-body blistering. This results in the potential for decreased oral intake, malabsorption, anemia, and depressed visceral protein stores, and a multifactorial etiology for the development of malnutrition and growth retardation. Thus, it was the purpose of this study to document the nutritional and metabolic profile of seven children with junctional or recessive dystrophic EB as compared to seven age- and sex-matched controls. Each child underwent a comprehensive nutritional assessment, including evaluation of anthropometric, dietary, and biochemical values and determination of resting energy expenditures. This study demonstrated that subjects with EB are statistically different for all anthropometric values studied and represent a population suffering from the effects of acute and chronic malnutrition. Nutrient deficiencies were reported for zinc, magnesium, calcium, potassium, and iron; vitamines A, D, E, B1, B12, and B6; protein, and calories. Comparison of laboratory values revealed significantly lower values for hemoglobin, hematocrit, and zinc. This research illustrates the magnitude of the growth deficits, and nutrient and biochemical deficiencies present in children with EB. The results provide a strong argument for the value of nutritional assessment and intervention and their potential impact in this population. Optimizing nutritional status may be one viable method of improving the morbidity and mortality associated with the disease and ultimately improving the overall quality of life.
Lechner-Gruskay
D
D
Department of Clinical Nutrition, Children's Hospital of Philadelphia, Pennsylvania 19104.
Honig
P J
PJ
Pereira
G
G
McKinney
S
S
D000886:Anthropometry; D002648:Child; D002149:Energy Intake; D004734:Energy Metabolism; D004820:Epidermolysis Bullosa / Q000378:metabolism*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009751:Nutritional Requirements