BACKGROUND: Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infants. Its management can be extremely complicated, and may involve medical therapy and surgery. The mainstay of the treatment is to maintain normoglycemia, since hypoglycemia during infancy can have severe neurological consequences. OBJECTIVE: To assess the cognitive and developmental levels and the adaptive skills achieved by children with CHI who were treated medically over the past decade. SUBJECTS AND METHODS: Fourteen children with CHI, under the age of 10 years, who received medical treatment only, underwent a physical and neurological examination and standardized assessments that included the Bayley Scale of Infant and Toddler Development, 3rd Edition, or Kaufman Assessment Battery for Children, the Vineland Adaptive Behavior Scales and the Achenbach Child Behavior Checklist (CBCL) parent questionnaire form. RESULTS: Twelve children (86%) achieved normal range scores in the cognitive and development assessments (Bayley Scale of Infant and Toddler Development or Kaufman Assessment Battery for Children). Only two showed cognitive achievements below the normal range. The Vineland questionnaire, which was based on parental report, showed below normal adaptive skills in eight patients (57%). CONCLUSIONS: In contrast to previous studies showing a high prevalence of neurodevelopmental difficulties in children with congenital hyperinsulinism, our study showed normal cognitive achievements in most children. This may be attributed to the earlier recognition and better management of the disease in the past decade.
BACKGROUND:Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infants. Its management can be extremely complicated, and may involve medical therapy and surgery. The mainstay of the treatment is to maintain normoglycemia, since hypoglycemia during infancy can have severe neurological consequences. OBJECTIVE: To assess the cognitive and developmental levels and the adaptive skills achieved by children with CHI who were treated medically over the past decade. SUBJECTS AND METHODS: Fourteen children with CHI, under the age of 10 years, who received medical treatment only, underwent a physical and neurological examination and standardized assessments that included the Bayley Scale of Infant and Toddler Development, 3rd Edition, or Kaufman Assessment Battery for Children, the Vineland Adaptive Behavior Scales and the Achenbach Child Behavior Checklist (CBCL) parent questionnaire form. RESULTS: Twelve children (86%) achieved normal range scores in the cognitive and development assessments (Bayley Scale of Infant and Toddler Development or Kaufman Assessment Battery for Children). Only two showed cognitive achievements below the normal range. The Vineland questionnaire, which was based on parental report, showed below normal adaptive skills in eight patients (57%). CONCLUSIONS: In contrast to previous studies showing a high prevalence of neurodevelopmental difficulties in children with congenital hyperinsulinism, our study showed normal cognitive achievements in most children. This may be attributed to the earlier recognition and better management of the disease in the past decade.
Authors: Annett Helleskov; Maria Melikyan; Evgenia Globa; Inna Shcherderkina; Fani Poertner; Anna-Maria Larsen; Karen Filipsen; Klaus Brusgaard; Charlotte Dahl Christiansen; Lars Kjaersgaard Hansen; Henrik T Christesen Journal: Front Endocrinol (Lausanne) Date: 2017-07-10 Impact factor: 5.555
Authors: Chris Worth; Laila Al Hashmi; Daphne Yau; Maria Salomon-Estebanez; Diego Perez Ruiz; Caroline Hall; Elaine O'Shea; Helen Stokes; Peter Foster; Sarah E Flanagan; Karen E Cosgrove; Mark J Dunne; Indraneel Banerjee Journal: Orphanet J Rare Dis Date: 2020-06-24 Impact factor: 4.123
Authors: Alena Welters; Thomas Meissner; Jürgen Grulich-Henn; Elke Fröhlich-Reiterer; Katharina Warncke; Klaus Mohnike; Oliver Blankenstein; Ulrike Menzel; Nicolin Datz; Esther Bollow; Reinhard W Holl Journal: Orphanet J Rare Dis Date: 2018-12-22 Impact factor: 4.123
Authors: Sevim Ünal; Deniz Gönülal; Ahmet Uçaktürk; Betül Siyah Bilgin; Sarah E Flanagan; Fatih Gürbüz; Meltem Tayfun; Selin Elmaoğulları; Aslıhan Araslı; Fatma Demirel; Sian Ellard; Khalid Hussain Journal: J Clin Res Pediatr Endocrinol Date: 2016-05-16