Neu Laxova syndrome and megacystis in the first trimester: Broadening the fetal phenotype

Prenat Diagn. 2023 Dec;43(13):1666-1670. doi: 10.1002/pd.6463. Epub 2023 Nov 14.

Abstract

Neu Laxova syndrome (NLS) is a rare and lethal congenital disorder characterized by severe intra-uterine growth retardation (IUGR), ichthyosis, abnormal facial features, limb abnormalities with arthrogryposis and a wide spectrum of severe malformations of the central nervous system (CNS). NLS is due to biallelic variants in three genes previously involved in serine-deficiency disorders (PHGDH, PSAT1 and PSPH), extending the phenotypic spectrum of these disorders.

MeSH terms

  • Abnormalities, Multiple
  • Brain Diseases
  • Duodenum / abnormalities
  • Female
  • Fetal Diseases
  • Fetal Growth Retardation / diagnosis
  • Fetal Growth Retardation / genetics
  • Humans
  • Ichthyosis* / diagnosis
  • Ichthyosis* / genetics
  • Limb Deformities, Congenital
  • Microcephaly* / genetics
  • Phenotype
  • Pregnancy
  • Pregnancy Trimester, First
  • Urinary Bladder / abnormalities

Supplementary concepts

  • Neu Laxova syndrome
  • Megaduodenum