A connective tissue disorder may underlie ESSENCE problems in childhood.
BACKGROUND
Ehlers-Danlos syndrome hypermobility type, also known as Joint Hypermobility Syndrome (EDS-HT/JHS), is the most common hereditary disorder of the connective tissue (HDCT). It is characterized by tissue fragility, joint hypermobility and a wide range of articular and non-articular manifestations, which often appear in infancy. The clinical picture of EDS-HT/JHS is poorly known by the medical community, as is the presence of "ESSENCE" (Early Symptomatic Syndromes Eliciting Neurodevelopmental Clinical Examinations) problems in affected children.


OBJECTIVE
The present work reviews the clinical and empirical evidence for ESSENCE difficulties in children with EDS-HT/JHS.


METHODS
A narrative review of the literature was undertaken following a comprehensive search of scientific online databases and reference lists. This included publications of quantitative and qualitative research.


RESULTS
Motor abnormality, hyperactivity/hypoactivity, inattention, speech/language, social interaction, behavioral, sleep, feeding and emotional problems are ESSENCE difficulties for which there is some evidence of an association with EDS-HT/JHS.


CONCLUSIONS
Children with EDS-HT/JHS present ESSENCE problems that often coexist and tend to be recognized before the HDCT. Clinicians encountering children with ESSENCE problems should consider the possibility of an underlying HDCT such as EDS-HT/JHS, probably influencing neurodevelopmental attributes in a subgroup of children. Awareness of these interconnected clinical problems might help improve early referral, diagnosis and treatment of EDS-HT/JHS.
Department of Psychology, Laboratory of Psychopathology and Health Process, University Paris Descartes - Sorbonne Paris Cité, Boulogne-Billancourt, France. Electronic address: carolina.baeza-velasco@parisdescartes.fr.
Carolina
C
Baeza-Velasco
Division of Medicine, University College London, London, UK.
Rodney
R
Grahame
Medical School, University of Chile, Santiago, Chile; Rheumatology Unit, San Juan de Dios Hospital, Santiago, Chile.
Jaime F
JF
Bravo
D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004535:Ehlers-Danlos Syndrome / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D009048:Motor Skills; D019957:Motor Skills Disorders / Q000175:diagnosis; D009123:Muscle Hypotonia; D065886:Neurodevelopmental Disorders / Q000175:diagnosis / Q000503:physiopathology*; D004856:Postural Balance; D011434:Proprioception; D012678:Sensation Disorders / Q000175:diagnosis / Q000503:physiopathology
A typical brain development; Attention deficit; Developmental coordination disorder; ESSENCE; Ehlers-Danlos syndrome; Feeding; Hyperactivity; Hypoactivity; Joint hypermobility syndrome; Neurodevelopmental disorders; Sleep problems