Congenital Disorder of Glycosylation, Type IIL

Alternative Names

  • CDG2L
  • CDG IIL
  • CDGIIL
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WHO-ICD-10 version:2010

Endocrine, nutritional and metabolic diseases

Metabolic disorders

OMIM Number

614576

Mode of Inheritance

Autosomal recessive

Gene Map Locus

13q14.11

Description

CDG2L is an autosomal recessive multisystem disorder apparent from birth or early infancy. It is characterized by poor growth, gastrointestinal and liver abnormalities, delayed psychomotor development, hypotonia, recurrent infections, hematologic abnormalities, increased bleeding tendency, and hyperhidrosis or hyperkeratosis. More variable features include nonspecific dysmorphic facial features and cardiac septal defects. The disorder often results in death in infancy or the first years of life. [From OMIM]

Epidemiology in the Arab World

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Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
614576.1United Arab EmiratesUnknownNM_020751.3:c.1167-24A>GHomozygousAutosomal, RecessiveBertoli-Avella et al. 2021
614576.2Saudi ArabiaMale Failure to thrive; Global developmental ...NM_020751.3:c.1167-24A>GHomozygousAutosomal, RecessiveMonies et al. 2017
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