Innovative Journal of Pediatrics
The Scientific Journal of Growth & Development Research Center
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Infantile Systemic Hyalinosis: Report of 17-year Experience
Author(s):
Azadeh Afshin1,*, Seyed Reza Raeeskarami2, Yahya Aghighi3, Abdolreza Malek4, Ali Zamani5, Vahid Ziaee6Published online:Dec 09, 2014
Article type:Case Report
Received:Feb 03, 2014
Accepted:Oct 29, 2014
How to Cite:Afshin A, Raeeskarami SR, Aghighi Y, Malek A, Zamani A, et al. Infantile Systemic Hyalinosis: Report of 17-year Experience. Inn J Pediatr. 2014;24(6):. doi:
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.Cases Presentation: 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.Conclusion: 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.
References
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Copyright
© 2014, Author(s). This open-access article is available under the Creative Commons Attribution 4.0 (CC BY 4.0) International License (https://creativecommons.org/licenses/by/4.0/), which allows for unrestricted use, distribution, and reproduction in any medium, provided that the original work is properly cited.
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