| Literature DB >> 28742265 |
Sarah Alsubhi1, Amal Alhashem2, Eissa Faqeih3, Majid Alfadhel4, Abdullah Alfaifi4, Waleed Altuwaijri5, Saud Alsahli4, Hesham Aldhalaan6, Fowzan S Alkuraya7,8, Khalid Hundallah1, Adel Mahmoud3, Ali Alasmari3, Fuad Al Mutairi4, Hanem Abduraouf2, Layan AlRasheed2, Saad Alshahwan1, Brahim Tabarki1.
Abstract
We retrospectively reviewed Saudi patients who had a congenital disorder of glycosylation (CDG). Twenty-seven Saudi patients (14 males, 13 females) from 13 unrelated families were identified. Based on molecular studies, the 27 CDG patients were classified into different subtypes: ALG9-CDG (8 patients, 29.5%), ALG3-CDG (7 patients, 26%), COG6-CDG (7 patients, 26%), MGAT2-CDG (3 patients, 11%), SLC35A2-CDG (1 patient), and PMM2-CDG (1 patient). All the patients had homozygous gene mutations. The combined carrier frequency of CDG for the encountered founder mutations in the Saudi population is 11.5 per 10,000, which translates to a minimum disease burden of 14 patients per 1,000,000. Our study provides comprehensive epidemiologic information and prevalence figures for each of these CDG in a large cohort of congenital disorder of glycosylation patients.Entities:
Keywords: ALG9; CDG; Congenital disorder(s) of glycosylation; severe phenotype; skeletal dysplasia
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Year: 2017 PMID: 28742265 DOI: 10.1002/ajmg.a.38358
Source DB: PubMed Journal: Am J Med Genet A ISSN: 1552-4825 Impact factor: 2.802