[Clinical and genetic analysis of a child with ZTTK syndrome due to heterozygous variant of SON gene]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2022 Mar 10;39(3):316-320. doi: 10.3760/cma.j.cn511374-20210315-00225.
[Article in Chinese]

Abstract

Objective: To explore the genetic etiology of a small-for-date infant with gastrointestinal bleeding, developmental delay and thrombocytopenia (Zhu-Tokita-Takenouchi-Kim syndrome).

Methods: Clinical and laboratory examinations were carried out for the patient. Next-generation sequencing (NGS) was used to detect potential variant associated with the disease. Candidate variant was verified by Sanger sequencing of the child and her parents.

Results: NGS revealed that the child has carried a heterozygous c.5751_5754del variant of the SON gene, which resulted in a frameshift p.V1918Efs*87. The same variant was detected in neither parent.

Conclusion: The heterozygous variant of SON gene probably underlay the ZTTK syndrome in this child. Above finding has enriched the mutational spectrum of the SON gene and provides a basis for genetic counseling and clinical decision-making.

MeSH terms

  • Child
  • Family
  • Female
  • Genetic Testing*
  • Heterozygote
  • Humans
  • Infant
  • Intellectual Disability* / genetics
  • Mutation