title: Nutritional status and gastrointestinal structure and function in children with ichthyosis and growth failure.
abstract: Growth failure occurs in several of the ichthyoses, a heterogeneous group of inherited disorders characterized by thickened or scaly skin. This suggests that there may be common pathogenic mechanisms causing failure to thrive. Previous studies have proposed that a hypermetabolic state induced by epidermal inflammation and hyperproliferation or enteropathy leading to malabsorption and nutritional deficiencies might account for the growth failure in icthyosis.
  
  
  OBJECTIVE
  The purpose of this study was to examine the extent of enteropathy and nutritional deficiency in children with severe ichthyosis and growth failure.
  
  
  METHODS
  Ten children with different types of ichthyoses and growth failure were studied.
  
  
  RESULTS
  Evaluation of gastrointestinal structure and function revealed few abnormalities other than mild fat malabsorption in two patients. Total caloric intake exceeded established requirements for age, height, and weight in all patients. Deficiencies of fat-soluble vitamins were identified in a few subjects. Mild elevations in total calcium and magnesium blood level, elevated hematocrit levels, and constipation were observed in many patients.
  
  
  CONCLUSIONS
  Nutritional deficiencies and gastrointestinal abnormalities are uncommon in children with ichthyosis and failure to thrive. Chronic hypovolemia caused by impaired epidermal permeability barrier may be common in this population.
authors.affiliation: Department of Dermatology, University of California San Francisco, San Francisco, California 94143, USA.
authors.forename: Ashley J
authors.initials: AJ
authors.lastname: Fowler
authors.forename: David G
authors.initials: DG
authors.lastname: Moskowitz
authors.forename: Anthony
authors.initials: A
authors.lastname: Wong
authors.forename: Sharon P
authors.initials: SP
authors.lastname: Cohen
authors.forename: Mary L
authors.initials: ML
authors.lastname: Williams
authors.forename: Melvin B
authors.initials: MB
authors.lastname: Heyman
mesh_terms: D002118:Calcium / Q000097:blood; D002648:Child; D015362:Child Nutrition Disorders / Q000209:etiology*; D002664:Child Nutritional Physiological Phenomena; D002675:Child, Preschool; D003248:Constipation / Q000209:etiology; D004041:Dietary Fats / Q000378:metabolism; D002149:Energy Intake; D005260:Female; D041981:Gastrointestinal Tract / Q000473:pathology / Q000503:physiopathology*; D006130:Growth Disorders / Q000209:etiology*; D006400:Hematocrit; D006801:Humans; D007057:Ichthyosis / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D007408:Intestinal Absorption / Q000502:physiology*; D008274:Magnesium / Q000097:blood; D008297:Male; D015596:Nutrition Assessment; D009752:Nutritional Status
chemical_list: D004041:Dietary Fats; D008274:Magnesium; D002118:Calcium