A Child with Kabuki Syndrome and Autism Spectrum Disorder.

Noro Psikiyatr Ars

Department of Child and Adolescent Psychiatry, Ankara University School of Medicine, Ankara, Turkey.

Published: September 2016

Kabuki syndrome (KS) is characterized by skeletal abnormalities, short stature, characteristic facial features, postnatal growth delay, and mental retardation. There are only a few case reports that present the coexistence of KS with autism spectrum disorder (ASD) in the literature. Herein we present the case of a boy with KS and ASD and discuss the possible shared etiologies. A 4-year-old boy was brought by his parents with complaints of no speech, hyperactivity, enuresis complex, temper tantrum, self-injury, and harming people or objects. We determined the lack of speech and eye contact, stereotypical behavior, and impaired social interaction and diagnosed him with autism and severe mental retardation via a psychiatric assessment. He had been followed up by pediatricians until he was 2 years old. Pediatricians noted his long eyelids with eversion of the lateral third of the lower eyelid, depressed nasal tip, short stature, long palpebral fissures, brachydactyly, and fetal finger pads in their physical examination. The boy who has an operated ventral septal defect and seizures was diagnosed with KS when he was 5 years old. We recommended his parents to apply to a special education agency and kindergarten for him. Our case is a new example of the coexistence of KS and ASD in addition to the very few cases in the literature. Genetic analyses conducted in the existence of specific genetic syndromes, such as KS, may provide opportunities for understanding the genetic etiology of ASD and new scope in terms of novel treatment approaches.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5378211PMC
http://dx.doi.org/10.5152/npa.2015.10099DOI Listing

Publication Analysis

Top Keywords

kabuki syndrome
8
autism spectrum
8
spectrum disorder
8
short stature
8
mental retardation
8
child kabuki
4
syndrome autism
4
disorder kabuki
4
syndrome characterized
4
characterized skeletal
4

Similar Publications

The Kabuki syndrome (KS) is a rare congenital disease that has two different types, KS1 and KS2, with variant in epigenetic gene KMT2D and KDM6A, respectively. It is associated with multiple abnormalities such as (developmental delay, atypical facial features, cardiac anomalies, minor skeleton anomalies, genitourinary anomalies, and mild to moderate intellectual disability). This syndrome can lead to neonatal hypoglycemia that results from hyperinsulinemia and electrolyte abnormalities.

View Article and Find Full Text PDF

Objective: To explore the clinical and genetic characteristics of two children diagnosed with two rare genetic diseases simultaneously.

Methods: Two children with comorbidity of two genetic diseases due to dual genetic mutations diagnosed at the Third Affiliated Hospital of Zhengzhou University respectively in May 2022 and March 2023 were selected as the study subjects. Clinical and genetic data of the two children were retrospectively analyzed.

View Article and Find Full Text PDF

KDM6A facilitates Xist upregulation at the onset of X inactivation.

Biol Sex Differ

January 2025

Department of Laboratory Medicine and Pathology, School of Medicine, University of Washington, Seattle, WA, 98195, USA.

Background: X chromosome inactivation (XCI) is a female-specific process in which one X chromosome is silenced to balance X-linked gene expression between the sexes. XCI is initiated in early development by upregulation of the lncRNA Xist on the future inactive X (Xi). A subset of X-linked genes escape silencing and thus have higher expression in females, suggesting female-specific functions.

View Article and Find Full Text PDF

Introduction: Congenital Hyperinsulinism (CHI) has not been previously studied in Ukraine. We therefore aimed to elucidate the genetics, clinical phenotype, histological subtype, treatment and long-term outcomes of Ukrainian patients with CHI.

Methods: Forty-one patients with CHI were recruited to the Ukrainian national registry between the years 2014-2023.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!