title: Infantile Systemic Hyalinosis: Report of 17-year Experience.
abstract: BACKGROUND
  Infantile Systemic Hyalinosis (ISH) is a very rare autosomal recessive disorder characterized by connective tissue involvement as hyaline deposition in skin, gastrointestinal tract, muscles, glands and other organs.
  
  
  METHODS
  We report eight Iranian children (4 male and 4 female) with ISH referred to our hospital from 1996 to 2013. The illness had been diagnosed by clinical manifestations and disease progression. Six of them died and two are alive but very sick.
  
  
  CONCLUSIONS
  ISH is a very rare disorder with poor prognosis. Seventy five percent of our 8 patients died before 2 years old due to severe diarrhea, malabsorption and/or infection.
authors.affiliation: Department of Pediatrics, ; Valiye-Asr Hospital, Imam Hospital Complex, ; Pediatric Rheumatology Research Group, Rheumatology Research Center.
authors.forename: Seyed Reza
authors.initials: SR
authors.lastname: Raeeskarami
authors.affiliation: Department of Pediatrics, ; Valiye-Asr Hospital, Imam Hospital Complex.
authors.forename: Yahya
authors.initials: Y
authors.lastname: Aghighi
authors.affiliation: Valiye-Asr Hospital, Imam Hospital Complex.
authors.forename: Azadeh
authors.initials: A
authors.lastname: Afshin
authors.affiliation: Department of Pediatrics, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
authors.forename: Abdolreza
authors.initials: A
authors.lastname: Malek
authors.affiliation: Department of Pediatrics, ; Valiye-Asr Hospital, Imam Hospital Complex.
authors.forename: Ali
authors.initials: A
authors.lastname: Zamani
authors.affiliation: Department of Pediatrics, ; Pediatric Rheumatology Research Group, Rheumatology Research Center, ; Children's Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Tehran.
authors.forename: Vahid
authors.initials: V
authors.lastname: Ziaee
keywords: Blond Hair; Hyaline Deposits; Infantile Systemic Hyalinosis; Joint Contractures; Skin Thickness