Visceral myopathy causing chronic intestinal pseudoobstruction and intestinal failure in a child with Sanjad-Sakati syndrome

J Pediatr Surg. 2010 Feb;45(2):430-4. doi: 10.1016/j.jpedsurg.2009.12.004.

Abstract

Sanjad-Sakati syndrome is a rare autosomal recessive disorder mainly occurring in the Arab Peninsula. This condition is associated with metabolic and septic complications starting in the neonatal period. Chronic intestinal pseudoobstruction owing to visceral myopathy is a rare disabling condition. We report a rare concurrence of Sanjad-Sakati syndrome and chronic intestinal pseudoobstruction in a Saudi child complicated by intestinal failure, sepsis, and early mortality.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / epidemiology*
  • Abnormalities, Multiple / genetics
  • Abnormalities, Multiple / mortality
  • Child
  • Chromosomes, Human, Pair 1 / genetics
  • Chronic Disease
  • Comorbidity
  • Developmental Disabilities / epidemiology
  • Developmental Disabilities / genetics
  • Fatal Outcome
  • Gastrointestinal Motility / genetics
  • Gastrointestinal Motility / physiology
  • Gene Deletion
  • Growth Disorders / epidemiology
  • Growth Disorders / genetics
  • Humans
  • Intellectual Disability / epidemiology
  • Intellectual Disability / genetics
  • Intestinal Pseudo-Obstruction / complications
  • Intestinal Pseudo-Obstruction / epidemiology*
  • Intestinal Pseudo-Obstruction / etiology
  • Intestinal Pseudo-Obstruction / genetics
  • Male
  • Saudi Arabia / epidemiology
  • Syndrome