| Literature DB >> 26453364 |
Sarah AlSubhi1, Amal AlHashem2, Anas AlAzami3, Kalthoum Tlili4, Saad AlShahwan1, Dirk Lefeber5, Fowzan S Alkuraya3,6, Brahim Tabarki7.
Abstract
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.Entities:
Keywords: ALG9-CDG; Congenital disorders of glycosylation; Epilepsy; Hydrops fetalis; Skeletal dysplasia; Whole exome sequencing
Year: 2015 PMID: 26453364 PMCID: PMC4867846 DOI: 10.1007/8904_2015_504
Source DB: PubMed Journal: JIMD Rep ISSN: 2192-8304