title

ارزیابی یکصدمورد بیوپسی عضلانی در کودکان در بیمارستانهای مفید و طوس تهران در یک دوره 2 ساله

نیلی پور, یلدا and شریعتمداری, فخرالدین and عبدلله گرجی, فاطمه and روزرخ, محسن and غفرانی, محمد and کریم زاده, پروانه and تقدیری, محمدمهدی and احمدآبادی, فرزاد and دلاور کسمایی, حسین (1392) ارزیابی یکصدمورد بیوپسی عضلانی در کودکان در بیمارستانهای مفید و طوس تهران در یک دوره 2 ساله. Iranian journal of child neurology ــ 7 (2). pp. 21-17. شاپا 1735-4668

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Title

Evaluation of One Hundred Pediatric Muscle Biopsies During A 2-Year Period in Mofid Children And Toos Hospitals

English Abstract

How to Cite This Article:Nilipor Y, Shariatmadari F, Abdollah gorji F, Rouzrokh M, Ghofrani M, Karimzadeh P, Taghdiri MM, Delavarkasmaei H, Ahmadabadi F, Bakhshandeh bali MK, Nemati H, Saket S, Jafari N, Yaghini O, Tonekaboni SH. Evaluation of One Hundred Pediatric Muscle Biopsies During A 2-Year Period in Mofid Children And Toos Hospitals. Iran J Child Neurol. 2013 Spring;7(2):17-21. Objective Muscle biopsy is a very important diagnostic test in the investigation of a child with suspected neuromuscular disorder. The goal of this study was to review and evaluate pediatric muscle biopsies during a 2-year period with focus on histopathology diagnosis and correlations with other paraclinic studies. Materials & Methods We investigated 100 muscle biopsies belonging to patients with clinical impression of neuromuscular disorder. These patients have been visited consecutively by pediatric neurologists during 2010 to 2012. Samples were investigated by standard enzyme histochemical and immunohistochemical techniques. Result Sixty-nine (69%) males and 39 (39%) females with a mean age of 5.7 years were evaluated. Major pathologic diagnoses were Muscular dystrophy (48 cases), Neurogenic atrophy (18 cases), nonspecific myopathic atrophy (12cases), congenital myopathy (6 cases), storage myopathies (4 cases) and in 6 cases there was no specific histochemical pathologic finding. EMG was abnormal in 79 cases. Degree of correlation between EMG and biopsy result was significant in children ≥ 2 years of age. Conclusion This study confirms the high diagnostic yields of muscle biopsy especially only if standard and new techniques such as enzyme study and immunohistochemistry are implemented. Also, we report 11 cases of Merosin negative congenital muscular dystrophy. This is the largest documented case series of Merosin deficient congenital muscular dystrophy reported from Iran.

Item Type:Article
زبان سند : انگلیسی
نویسنده اول :یلدا نیلی پور
نویسنده مسئول :فخرالدین شریعتمداری
Additional Information:Indexed in: PubMed/Pubmed Central , EMBASE , Scopus , ISC(Islamic World Science Citation Center) , CABI(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):Muscle biopsy; Congenital myopathy; Muscular dystrophy; Merosin; EMG
Subjects:WE Musculoskeletal system
WL Nervous system
WS Pediatrics
Divisions:Faculty of Medicine > Department of Pediatrics , Community Medicine
ID Code:5282
Deposited By: Dr Farzad Ahmadabadi
Deposited On:15 Dec 1392 12:51
Last Modified:13 Feb 1394 09:40

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