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Microcephalic primordial dwarfism-insulin resistance syndrome

MedGen UID:
1806985
Concept ID:
C5681181
Disease or Syndrome
Synonym: Microcephalic primordial dwarfism, insulin resistance syndrome
SNOMED CT: Microcephalic primordial dwarfism, insulin resistance syndrome (1220596009)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Orphanet: ORPHA436182

Definition

A rare genetic disease with characteristics of severe pre and postnatal growth failure with short stature and microcephaly, facial dysmorphism (including a small jaw and prominent midface), severe insulin resistance, fatty liver, hypertriglyceridemia developing in childhood and primary gonadal failure. Mild global learning difficulties and acanthosis nigricans have also been reported. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMicrocephalic primordial dwarfism-insulin resistance syndrome

Recent clinical studies

Etiology

Alrajhi H, Alallah J, Shawli A, Alghamdi K, Hakami F
BMJ Case Rep 2019 May 30;12(5) doi: 10.1136/bcr-2018-224197. PMID: 31151966Free PMC Article

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