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Isolated glycerol kinase deficiency

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Disease definition

Isolated glycerol kinase deficiency (GKD) is a very rare X-linked disorder of glycerol metabolism characterized biochemically by elevated plasma and urine glycerol levels, and clinically by variable neurometabolic manifestations, depending on the age of onset, and varying from a life-threatening childhood metabolic crisis to an asymptomatic adult form (infantile GKD, juvenile GKD, and adult GKD).

ORPHA:408

Classification level: Disorder

Synonym(s):
  • Hyperglycerolemia

Source: PubMed ID 33212314

Prevalence: Unknown

Inheritance: X-linked recessive

Age of onset: All ages

ICD-10: E74.8

ICD-11: 5C51.1

OMIM: 307030

UMLS: C0574108

MeSH: C538138

GARD: 2807

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands Polski, Русский
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
English (2012.pdf) - Brit Inher Metab Dis Group
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