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Further Delineation of the ALG9-CDG Phenotype.
AlSubhi, Sarah; AlHashem, Amal; AlAzami, Anas; Tlili, Kalthoum; AlShahwan, Saad; Lefeber, Dirk; Alkuraya, Fowzan S; Tabarki, Brahim.
Afiliação
  • AlSubhi S; Divisions of Pediatric Neurology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
  • AlHashem A; Division of Genetics, Department of Pediatrics, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
  • AlAzami A; Department of Genetics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
  • Tlili K; Department of Radiology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
  • AlShahwan S; Divisions of Pediatric Neurology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
  • Lefeber D; Department of Neurology, Translational Metabolic Laboratory of Genetic, Endocrine and Metabolic Diseases, Radboud University Medical Center, Nijmegen, The Netherlands.
  • Alkuraya FS; Department of Genetics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
  • Tabarki B; Department of Anatomy and Cell Biology, College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
JIMD Rep ; 27: 107-12, 2016.
Article em En | MEDLINE | ID: mdl-26453364
ALG9-CDG is one of the less frequently reported types of CDG. Here, we summarize the features of six patients with ALG9-CDG reported in the literature and report the features of four additional patients. The patients presented with drug-resistant infantile epilepsy, hypotonia, dysmorphic features, failure to thrive, global developmental disability, and skeletal dysplasia. One patient presented with nonimmune hydrops fetalis. A brain MRI revealed global atrophy with delayed myelination. Exome sequencing identified a novel homozygous mutation c.1075G>A, p.E359K of the ALG9 gene. The results of our analysis of these patients expand the knowledge of ALG9-CDG phenotype.
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Texto completo: 1 Bases de dados: MEDLINE Idioma: En Revista: JIMD Rep Ano de publicação: 2016 Tipo de documento: Article País de afiliação: Arábia Saudita

Texto completo: 1 Bases de dados: MEDLINE Idioma: En Revista: JIMD Rep Ano de publicação: 2016 Tipo de documento: Article País de afiliação: Arábia Saudita