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Published on: December 7, 2018
Socio-emotional Problems in Children with CDG
K F E van de Loo1, L van Dongen, M Mohamed
1Department of Medical Psychology, Radboud University Medical Centre, Nijmegen, The Netherlands.
Insights
Children with Congenital Disorders of Glycosylation (CDG) experience a high prevalence of socio-emotional problems, including withdrawn/depressed behavior and social difficulties. Early psychological screening is recommended for these patients.
Area of Science:
- Pediatric Neurology
- Metabolic Disorders
- Neurodevelopmental Disorders
Background:
- Congenital Disorders of Glycosylation (CDG) are inherited metabolic diseases often affecting the nervous system.
- Parents report behavioral issues like anxiety and depression in CDG patients.
- This suggests a potential link between CDG and socio-emotional difficulties.
Purpose of the Study:
- To investigate the prevalence of socio-emotional problems in children with CDG.
- To assess if CDG patients have an increased risk for psychological challenges.
Main Methods:
- Evaluated 18 children diagnosed with CDG.
- Utilized the Child Behavior Checklist (CBCL) for socio-emotional screening.
- Employed the Nijmegen Paediatric CDG Rating Scale (NPCRS) for disease severity assessment.
- Compared results to normative data and control groups (mitochondrial and other chronic metabolic disorders).
Main Results:
- Children with CDG exhibited a high rate of socio-emotional problems.
- Significantly elevated scores were observed for overall problems, withdrawn/depressed behavior, social issues, and somatic complaints.
- Over two-thirds of CDG patients showed abnormal CBCL scores.
- Social problem scores were notably higher in CDG patients compared to control groups.
Conclusions:
- CDG patients face an increased risk of developing socio-emotional problems.
- Routine psychological screening is advised for early identification and management of these issues in CDG.
- This highlights the importance of addressing the psychological well-being of children with CDG.
Background:
Congenital disorders of glycosylation (CDG) form a group of inherited metabolic diseases. Although the clinical presentation shows extreme variability, the nervous system is frequently affected. Several parents of our patients diagnosed with CDG reported behavioral problems, including mood swings, depressive behavior, and anxiety. This raised the question whether patients with CDG have an increased risk for socio-emotional problems.
Methods:
We evaluated 18 children with confirmed CDG. The Child Behavior Checklist (CBCL) was used to screen for socio-emotional problems. To determine the disease progression and severity in CDG, the Nijmegen Paediatric CDG Rating Scale (NPCRS) was used. RESULTS were compared to "norm scores" and to children with mitochondrial disorders and children with other chronic metabolic disorders with multisystem involvement.
Results:
RESULTS showed a high prevalence of socio-emotional problems in children with CDG. Mean total scores, scores on withdrawn/depressed behavior, social problems, and somatic complaints were significantly increased. More than two thirds of our CDG patients have abnormal scores on CBCL. The mean score on social problems was significantly higher compared to our two control groups of patients with other chronic metabolic disorders.
Conclusions:
Patients with CDG have an increased risk of developing socio-emotional problems. A standard screening for psychological problems is recommended for the early detection of psychological problems in CDG patients.
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