title: Sudden death in septo-optic dysplasia. Report of 5 cases.
abstract: OBJECTIVE
  To report our experience with sudden death in children with septo-optic dysplasia and to identify specific risk factors and suggest preventive measures to minimize mortality.
  
  
  METHODS
  Clinical data from 5 children with septo-optic dysplasia who died suddenly and unexpectedly were evaluated retrospectively.
  
  
  RESULTS
  All children had corticotropin deficiency, all had thermoregulatory disturbances, and 4 children had diabetes insipidus. In at least 4 children, clinical deterioration was caused by fever and dehydration from a presumed viral illness, which appeared to precipitate adrenal crisis.
  
  
  CONCLUSIONS
  Children with septo-optic dysplasia and hypocortisolism are at risk for sudden death during febrile illness. Thermoregulatory disturbances and dehydration from diabetes insipidus may potentiate clinical deterioration. Prevention of sudden death in septo-optic dysplasia requires early recognition and treatment of these major risk factors.
authors.affiliation: Department of Ophthalmology, University of Arkansas for Medical Sciences, USA.
authors.forename: M C
authors.initials: MC
authors.lastname: Brodsky
authors.forename: F A
authors.initials: FA
authors.lastname: Conte
authors.forename: D
authors.initials: D
authors.lastname: Taylor
authors.forename: C S
authors.initials: CS
authors.lastname: Hoyt
authors.forename: R E
authors.initials: RE
authors.lastname: Mrak
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000172:deficiency; D001833:Body Temperature Regulation; D002648:Child; D002675:Child, Preschool; D003645:Death, Sudden / Q000209:etiology*; D003919:Diabetes Insipidus / Q000150:complications; D005260:Female; D006801:Humans; D007003:Hypoglycemia / Q000150:complications; D007018:Hypopituitarism / Q000150:complications / Q000473:pathology; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009900:Optic Nerve / Q000002:abnormalities*; D012189:Retrospective Studies; D012307:Risk Factors; D012688:Septum Pellucidum / Q000002:abnormalities* / Q000473:pathology
chemical_list: D000324:Adrenocorticotropic Hormone