LIVER STORAGE DISEASE IN IRAN: A TEN YEAR STUDY OF LIVER BIOPSIES IN CHILDREN MEDICAL CENTER HOSPITAL IN TEHRAN-IRAN

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MOTAMED FARZANEH; MONAJEMZADEH MARYAM; SEIFIRAD SOROUSH; ASHRAFI MANDANA; RASTI ABBAS; MAHJOUB FATEMEH

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Journal: Hepatitis Monthly Year:2011 | Volume:11 | Issue:8 (37) Page(s): 652-655

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Title

LIVER STORAGE DISEASE IN IRAN: A TEN YEAR STUDY OF LIVER BIOPSIES IN CHILDREN MEDICAL CENTER HOSPITAL IN TEHRAN-IRAN

Author(s)

Keywords

EPIDEMIOLOGY

GAUCHER DISEASE

LYSOSOMAL STORAGE DISEASES

MUCOPOLYSACCHARIDOSES

GLYCOGEN STORAGE DISEASE

Abstract

Background: Liver storage diseases are rare biochemical and inherited diseases that affect multiorgan systems.Objectives: 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.Patients and 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.

Cites

ANALYSIS OF GENETIC VARIATION OF RS1542705 MARKER IN SMPD1 GENE REGION AS AN INFORMATIVE MARKER FOR MOLECULAR DIAGNOSIS OF NIEMANN- PICK DISEASE IN ISFAHAN POPULATION

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APA: Copy

MOTAMED, FARZANEH, MONAJEMZADEH, MARYAM, SEIFIRAD, SOROUSH, ASHRAFI, MANDANA, RASTI, ABBAS, & MAHJOUB, FATEMEH. (2011). LIVER STORAGE DISEASE IN IRAN: A TEN YEAR STUDY OF LIVER BIOPSIES IN CHILDREN MEDICAL CENTER HOSPITAL IN TEHRAN-IRAN. HEPATITIS MONTHLY, 11(8 (37)), 652-655. SID. https://sid.ir/paper/306435/en

Vancouver: Copy

MOTAMED FARZANEH, MONAJEMZADEH MARYAM, SEIFIRAD SOROUSH, ASHRAFI MANDANA, RASTI ABBAS, MAHJOUB FATEMEH. LIVER STORAGE DISEASE IN IRAN: A TEN YEAR STUDY OF LIVER BIOPSIES IN CHILDREN MEDICAL CENTER HOSPITAL IN TEHRAN-IRAN. HEPATITIS MONTHLY[Internet]. 2011;11(8 (37)):652-655. Available from: https://sid.ir/paper/306435/en

IEEE: Copy

FARZANEH MOTAMED, MARYAM MONAJEMZADEH, SOROUSH SEIFIRAD, MANDANA ASHRAFI, ABBAS RASTI, and FATEMEH MAHJOUB, “LIVER STORAGE DISEASE IN IRAN: A TEN YEAR STUDY OF LIVER BIOPSIES IN CHILDREN MEDICAL CENTER HOSPITAL IN TEHRAN-IRAN,” HEPATITIS MONTHLY, vol. 11, no. 8 (37), pp. 652–655, 2011, [Online]. Available: https://sid.ir/paper/306435/en

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