title: Liver storage disease in Iran: a ten year study of liver biopsies in Children Medical Center Hospital in Tehran-Iran.
abstract: BACKGROUND
  Liver storage diseases are rare biochemical and inherited diseases that affect multiorgan systems.
  
  
  OBJECTIVE
  This study was performed to determine the rate of storage diseases and their types in liver pathology specimens of subjects who were referred to a tertiary pediatric center.
  
  
  METHODS
  Two pathologists evaluated 2216 pathology specimens (stained with hematoxylin and eosin and periodic acid-Schiff) from subjects who were referred to the largest pediatric tertiary referral center in Iran between 1996 and 2007. Baseline data and clinical and laboratory manifestations were retrieved from the patients' files.
  
  
  RESULTS
  We identified 117 patients who had storage diseases. A combination of clinical and laboratory findings was used to assess the final diagnosis. Glycogen storage disease (GSD) was observed in 85 of cases, compared with lysosomal storage diseases (LSD) in 31 patients and mucopolysaccharidoses in 1 case. LSD was more prevalent in those aged between 1 month and 1 year, whereas GSD was more frequent in those aged between 1 and 6 years. Most of the patients aged between 1 and 6 years. Most patients with LSD and GSD had unknown types of the disease. The most common known types in the LSD and GSD groups were Niemann-Pick disease and GSD type I respectively. The most common clinical and laboratory manifestation was hepatomegaly and abnormal liver enzymes, respectively.
  
  
  CONCLUSIONS
  Most of our patients with storage diseases had Gaucher disease. Hepatomegaly and elevated transaminase levels were the most striking finding. However, with regard to the limitations of our methodology, further studies that collect more accurate data are warranted.
authors.affiliation: Department of Pediatrics Gastroenterology, Children Medical Center Hospital, Tehran University of Medical Sciences, Tehran, IR Iran.
authors.forename: Farzaneh
authors.initials: F
authors.lastname: Motamed
authors.forename: Maryam
authors.initials: M
authors.lastname: Monajemzadeh
authors.forename: Soroush
authors.initials: S
authors.lastname: Seifirad
authors.forename: Mandana
authors.initials: M
authors.lastname: Ashrafi
authors.forename: Abbas
authors.initials: A
authors.lastname: Rasti
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Mahjoub