[Clinical features and genetic testing of a child with hepatic failure syndrome type 2]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2022 Feb 10;39(2):181-184. doi: 10.3760/cma.j.cn511374-20200718-00526.
[Article in Chinese]

Abstract

Objective: To explore the genetic basis for a child with infantile liver failure syndrome type 2 (ILFS type 2).

Methods: Clinical features of the child were analyzed. Next generation sequencing was also carried out for him.

Results: The child was found to harbor compound heterozygous variants of the NBAS gene, which included a novel nonsense c.2746A>T (p.R916X, 1456) variant in exon 24 and a missense c.3596G>A (p.C1199Y) mutation in exon 31, which has been associated with ILFS type 2. The two variants were respectively inherited from his father and mother.

Conclusion: The compound heterozygous variants of c.3596G>A and c.2746A>T of the NBAS gene probably underlay the ILFS type 2 in this child.

MeSH terms

  • Child
  • Exons / genetics
  • Genetic Testing*
  • High-Throughput Nucleotide Sequencing
  • Humans
  • Liver Failure*
  • Male
  • Mutation