title: Recurrent spontaneous subserosal hematoma of ileum causing intestinal obstruction in a patient with menkes disease: A case report.
abstract: BACKGROUND
  Menkes disease (MD) is a disorder of copper metabolism due to ATP7A gene mutation that leads to severe copper deficiency. Deformed blood vessels can be found in many parts of the body, and intracranial hematoma is generally reported.
  
  
  METHODS
  We report a Taiwanese boy with MD who had recurrent spontaneous subserosal hematoma of ileum presenting as intestinal obstruction, with the 2 episodes 23 months apart. The patient returned to the usual physical status after surgical removal of the hematoma.
  
  
  RESULTS
  The defective copper metabolism causes dysfunction of a plenty of copper-dependent enzymes, giving rise to unique kinky hair appearance, progressive neurodegeneration, and connective tissue abnormalities. To our knowledge, this is the first report on recurrent subserosal hemorrhage of intestine in MD.
  
  
  CONCLUSIONS
  Owing to the fragile structure of blood vessels, subserosal hematoma should be considered when patients with MD having intestinal obstruction.
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Nutrition Department of Neonatology Department of Pediatric General Surgery and Urology Department of Genetics and Metabolism, MacKay Children's Hospital, Taipei Department of Medicine, MacKay Medical College, New Taipei City Division of Biochemical Genetics, Department of Medical Research, MacKay Memorial Hospital Department of Early Childhood Care, National Taipei University of Nursing and Health Sciences MacKay Junior College of Medicine, Nursing, and Management, Taipei, Taiwan.
authors.forename: Shu-Chao
authors.initials: SC
authors.lastname: Weng
authors.forename: Chyong-Hsin
authors.initials: CH
authors.lastname: Hsu
authors.forename: Nien-Lu
authors.initials: NL
authors.lastname: Wang
authors.forename: Shuan-Pei
authors.initials: SP
authors.lastname: Lin
authors.forename: Chuen-Bin
authors.initials: CB
authors.lastname: Jiang
mesh_terms: D002675:Child, Preschool; D006406:Hematoma / Q000150:complications*; D006801:Humans; D007077:Ileal Diseases / Q000209:etiology*; D007415:Intestinal Obstruction / Q000209:etiology*; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000150:complications*; D012008:Recurrence