title

بررسی تغییرات MRI مغز در بیماران مبتلا به PKU مراجعه کننده به بیمارستان مفید

کریم زاده, پروانه and جعفری, نرگس and احمدآبادی, فرزاد and شریعتمداری, فخرالدین and نعمتی, حمید and احدی, عادل and کریمی دردشتی, ساناز and میرزا رحیمی, مهرداد and زارع نوقابی, جواد and دست برهان, زهرا (1393) بررسی تغییرات MRI مغز در بیماران مبتلا به PKU مراجعه کننده به بیمارستان مفید. Iranian Journal of Child Neurology ــ 8 (2). pp. 53-56. شاپا 1735-4668

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Title

Study on MRI Changes in Phenylketonuria in Patients Referred to Mofid Hospital/Iran

English Abstract

Objective Phenylketonuria is one of the most common metabolic disorders and the first known cause of mental retardation in pediatrics. As Screening for phenylketonuria (PKU) is not a routine neurometabolic screening test for neonates in Iran, many PKU cases may be diagnosed after developing the clinical symptoms. One of the findings of PKU is myelination disorders, which is seen as hypersignal regions in T2-weighted (T2W) and FLAIR sequences of brain MRI. The aim of our study was to assess MRI changes in PKU patients referred to Mofid Children’s Hospital, 2010-2011. Materials & Methods We studied all PKU cases referred to our clinic as a referral neurometabolic center in Iran for brain MRI and assessed the phenylalanine level at the time of Imaging. The mean phenylalanine level (in one year), clinical manifestations, and MRI pattern based on Thompson scoring, were evaluated. Results The mean age of our study group was 155±99 months and the mean diagnosis age was 37±27.85 months. There were 15 patients with positive and 15 with negative family history. The mean phenylalanine level at the time of imaging was 9.75±6.28 and the mean 1 year phenylalanine level was 10.28±4.82. Seventy percent of our patients had MRI involvement, in whom 20% showed atrophic changes, in addition to white matter involvement. Based on modified Thompson scoring, the score for our study group was 4.84. The maximum involvement in MRI was in occipital region, followed by parietal, frontal, and temporal zones. There was not any correlation between MRI score and patients’ age. But we found significant relationship between MRI score and the age of regimen cessation. No correlation was seen between phenylalanine level (at the time of Imaging) and MRI score. But there was a relationship between mean 1 year phenylalanine level and MRI score. Conclusion According to the results of this study, brain MRI and white matter involvement can be used for evaluation of long-term control of phenylalanine level in PKU cases.

Item Type:Article
زبان سند : انگلیسی
نویسنده اول :پروانه کریم زاده
نویسنده مسئول :نرگس جعفری
نویسنده :فرزاد احمدآبادی
نویسنده :عادل احدی
نویسنده :مهرداد میرزا رحیمی
نویسنده :جواد زارع نوقابی
نویسنده :زهرا دست برهان
Additional Information:Indexed in: PubMed/Pubmed Central , EMBASE , Scopus , ISC(Islamic World Science Citation Center) , ABI(Bibliographic database) , IMEMR (Index Medicus for Eastern Mediterranean Region) , Index Copernicus , EBSCO (CINAHL) , Proquest (CSA: In Neuroscience Database) , DOAJ Google scholar , SID(Scientific Information Database) , Iranmedex , Magiran , IranJournal (RiceSt)
کلیدواژه ها (انگلیسی):Phenylketonuria ; MRI ; Thompson score
Subjects:WD Nutrition Disease and metabolic diseases
WL Nervous system
WS Pediatrics
Divisions:Faculty of Medicine > Department of Pediatrics , Community Medicine
ID Code:5686
Deposited By: Dr Adel Ahadi
Deposited On:12 Mar 1393 08:58
Last Modified:16 Aug 1394 09:20

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