Table 7.

Recommended Surveillance for Individuals with Epidermolysis Bullosa with Pyloric Atresia

System/ConcernEvaluationFrequency
Mucosal involvement
  • Assessment of oral mucosa, feeding, & esophageal involvement
  • Assessment for hoarse cry (tracheal involvement)
At each visit & per ENT
Skin Assessment of blisters & skin infectionAt each visit & per dermatologist
Gastrointestinal Assessment of gastrointestinal involvementAt each visit
Renal & ureteral anomalies
  • Assess renal function w/serum BUN & creatinine
  • Urinalysis
Per nephrologist
Fluid/electrolyte/
nutritional deficiencies
  • CBC
  • Iron studies
  • Serum zinc
  • Serum vitamin D
  • Selenium, carnitine, vitamin A
Annually
DXA scan for osteopenia/osteoporosisNo guidelines have been established regarding age at which this should begin & frequency.
Ocular Assessment of corneal abnormalitiesAt each visit
Social/Family Assessment of family needs

BUN = blood urea nitrogen; DXA = dual-energy x-ray absorptiometry

From: Epidermolysis Bullosa with Pyloric Atresia

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