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MIRAGE Syndrome Enteropathy Responding to Pancrelipase Despite Normal Pancreatic Fecal Elastase: A Case Report

Dalwinder Janjua1, Shiva Shankar1, Munira AlMaazmi1

  • 1Department of Neonatology, Al Jalila Children's Speciality Hospital, Dubai, United Arab Emirates.

The American Journal of Case Reports
|August 22, 2022
PubMed

Insights

MIRAGE syndrome, a rare genetic disorder, can cause severe enteropathy. Pancreatic enzyme supplements effectively treated enteropathy in an infant with MIRAGE syndrome, even with normal elastase levels.

Area of Science:

  • Genetics
  • Pediatrics
  • Gastroenterology

Background:

  • MIRAGE syndrome is a rare genetic disorder caused by SAMD9 gene mutations.
  • It is characterized by myelodysplasia, infection, growth restriction, adrenal hypoplasia, genital phenotypes, and enteropathy.
  • SAMD9 acts as a growth repressor, reducing cellular proliferation.

Observation:

  • A severe enteropathy case in an infant with MIRAGE syndrome was observed.
  • Gastrointestinal symptoms included chronic diarrhea, severe diaper rash, and colonic dilatation.
  • Exocrine pancreatic insufficiency was not previously described as a cause of enteropathy in MIRAGE syndrome.

Findings:

  • The infant's severe enteropathy responded well to porcine-derived pancreatic enzyme supplements.
  • This positive response occurred despite a normal pancreatic fecal elastase level.
  • This suggests pancreatic insufficiency can be a component of MIRAGE syndrome's enteropathy.

Implications:

  • Porcine-derived pancreatic enzymes may be a beneficial treatment for enteropathy in MIRAGE syndrome.
  • This finding expands the understanding of MIRAGE syndrome's gastrointestinal manifestations.
  • Early consideration of pancreatic enzyme supplementation is warranted for infants with MIRAGE syndrome and enteropathy.

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