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Qazi Markouizos syndrome

MedGen UID:
443987
Concept ID:
C2931142
Disease or Syndrome
Synonyms: Dysharmonic skeletal maturation muscular fibre disproportion; Hypotonia, psychomotor retardation, seizures, delayed and dysharmonic skeletal maturation, and congenital fibre type disproportion; Puerto Rican infant hypotonia syndrome
SNOMED CT: Dysharmonic skeletal maturation and muscular fiber disproportion syndrome (721887007); Puerto Rican infant hypotonia syndrome (721887007); Qazi Markouizos syndrome (721887007)
 
Monarch Initiative: MONDO:0010816
OMIM®: 600096
Orphanet: ORPHA3010

Definition

This syndrome is characterised principally by non-progressive central hypotonia, chronic constipation, severe psychomotor retardation, abnormal dermatoglyphics, dysharmonic skeletal maturation and disproportionate muscle fibres. Seizures or an abnormal electroencephalograph were also reported. To date, the syndrome has been reported in three unrelated Puerto Rican boys. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVQazi Markouizos syndrome
Follow this link to review classifications for Qazi Markouizos syndrome in Orphanet.

Recent clinical studies

Clinical prediction guides

Qazi QH, Markouizos D, Rao C, Sheikh T, Beller E, Kula R
J Med Genet 1994 May;31(5):405-9. doi: 10.1136/jmg.31.5.405. PMID: 8064821Free PMC Article

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