369710

Clinical Characterization of 53 Egyptian children diagnosed as Duchenne Muscular Dystrophy in tertiary unit in upper Egypt

Article

Last updated: 04 Jan 2025

Subjects

-

Tags

Pediatrics and neonatology.

Abstract

Background: Muscular dystrophies, which are characterized by progressive muscle atrophy, are a heterogeneous group of inherited muscular diseases. Aim of work: Clinical aspects in children with Duchenne muscular dystrophy at Sohag University Hospital. Subjects and methods: This was cross-sectional research performed at the pediatric department and pediatric neurology unit, Sohag University Hospital over two years, from 2022 to 2023. Results: In our research, 53 children had a confirmed Duchenne diagnosis, divided into two groups according to ambulation: Group A: 33 (62.3%) ambulant children. Group B: 20 (37.7%) non-ambulant children. The verbal and total IQ tests were significantly higher in group A than group B. The EF (ejection fraction) and FS (fraction shortening) were significantly higher in group A than group B. All patients had hyporeflexia. Regarding muscle state, there was calf hypertrophy in 33 (100%) of group A and 3 (15%) in group B, and Muscle wasting in 17 (85%) of group B compared to no cases in group A, which was significantly different. As regard skeletal abnormalities, 27 (81.8%) patients in group A had lordosis compared to 4 (20%) patients in group B, while 4 (20%) had scoliosis, 5 (25% had scoliosis and joint stiffness), and 5 (25% had joint stiffness) in group B, with no cases in group A with a statistically significant difference. Functional ability (North Star score) was positively correlated with young age. Conclusion: Younger age and ambulant status were associated with better cardiac, muscular, skeletal, cognitive, functional outcomes and abilities in Duchenne muscular dystrophy patients.

DOI

10.21608/smj.2024.278924.1460

Keywords

Characterization, Duchenne muscular dystrophy, Egyptian children

Authors

First Name

nehal

Last Name

abdo

MiddleName

sami

Affiliation

Pediatric Department, Faculty of Medicine, Sohag University, Sohag, Egypt.

Email

nehal.samy.ped@gmail.com

City

sohag

Orcid

-

First Name

Mostafa

Last Name

Abo Sedira

MiddleName

Mohamed

Affiliation

Department of Pediatrics, Faculty of Medicine, Sohag University, Sohag, Egypt

Email

aabosdera@yahoo.com

City

Sohag

Orcid

-

First Name

Abdelrahim

Last Name

Sadek

MiddleName

Abdrabou

Affiliation

Department of Pediatrics ,Faculty of Medicine, Sohag University, Sohag, Egypt.

Email

abdelreheam_sadek@med.sohag.edu.eg

City

Sohag

Orcid

0000-0001-6059-0936

First Name

Eman

Last Name

Fahmy

MiddleName

Mohamed

Affiliation

Department of Pediatrics , Faculty of Medicine , Sohag University, Sohag, Egypt

Email

emyfa2021@gmail.com

City

Sohag

Orcid

-

Volume

28

Article Issue

3

Related Issue

47914

Issue Date

2024-09-01

Receive Date

2024-03-23

Publish Date

2024-09-01

Page Start

74

Page End

82

Print ISSN

1687-8353

Online ISSN

2682-4159

Link

https://smj.journals.ekb.eg/article_369710.html

Detail API

https://smj.journals.ekb.eg/service?article_code=369710

Order

369,710

Type

Original Article

Type Code

785

Publication Type

Journal

Publication Title

Sohag Medical Journal

Publication Link

https://smj.journals.ekb.eg/

MainTitle

Clinical Characterization of 53 Egyptian children diagnosed as Duchenne Muscular Dystrophy in tertiary unit in upper Egypt

Details

Type

Article

Created At

24 Dec 2024