Muscle Ultrasound in Patients with Glycogen Storage Disease Types I and III

Autor: Deborah A Sival, Natasha M. Maurits, Johannes H. van der Hoeven, Christiaan P Sentner, R.J. Verbeek, Terry G J Derks, G. Peter A. Smit
Přispěvatelé: Center for Liver, Digestive and Metabolic Diseases (CLDM), Movement Disorder (MD), ​Basic and Translational Research and Imaging Methodology Development in Groningen (BRIDGE), Pediatric surgery
Jazyk: angličtina
Rok vydání: 2016
Předmět:
0301 basic medicine
Male
INVOLVEMENT
Acoustics and Ultrasonics
Myopathy
CHILDREN
Electromyography
030105 genetics & heredity
Glycogen Storage Disease Type I
Glycogen storage disease type III
Muscle ultrasound density
Gastroenterology
SPINA-BIFIDA APERTA
chemistry.chemical_compound
Glycogen Storage Disease Type III
0302 clinical medicine
DEBRANCHER DEFICIENCY
Glycogen storage disease
Muscle force
Child
Ultrasonography
Glycogen storage disease type I
Muscle Weakness
Radiological and Ultrasound Technology
medicine.diagnostic_test
Glycogen
MYELOMENINGOCELE
Anatomy
Child
Preschool

Female
medicine.symptom
DIETARY-MANAGEMENT
Adult
medicine.medical_specialty
congenital
hereditary
and neonatal diseases and abnormalities

Biophysics
03 medical and health sciences
Echogenicity
Internal medicine
NEUROMUSCULAR DISORDERS
SKELETAL
medicine
Humans
Radiology
Nuclear Medicine and imaging

Muscle
Skeletal

business.industry
Muscle weakness
nutritional and metabolic diseases
medicine.disease
REFERENCE VALUES
Cross-Sectional Studies
chemistry
business
030217 neurology & neurosurgery
Zdroj: Ultrasound in Medicine and Biology, 42(1), 133-142. ELSEVIER SCIENCE INC
Ultrasound in Medicine and Biology, 42(1), 133-142. Elsevier USA
Verbeek, R J, Sentner, C P, Smit, G P A, Maurits, N M, Derks, T G J, van der Hoeven, J H & Sival, D A 2016, ' Muscle Ultrasound in Patients with Glycogen Storage Disease Types I and III ', Ultrasound in Medicine and Biology, vol. 42, no. 1, pp. 133-142 . https://doi.org/10.1016/j.ultrasmedbio.2015.08.013
ISSN: 0301-5629
DOI: 10.1016/j.ultrasmedbio.2015.08.013
Popis: In glycogen storage diseases (GSDs), improved longevity has resulted in the need for neuromuscular surveillance. In 12 children and 14 adults with the "hepatic'' (GSD-I) and "myopathic'' (GSD-III) phenotypes, we cross-sectionally assessed muscle ultrasound density (MUD) and muscle force. Children with both "hepatic'' and "myopathic'' GSD phenotypes had elevated MUD values (MUD Z-scores: GSD-I. 2.5 SD vs. GSD-III. 1 SD, p
Databáze: OpenAIRE