title: [Evaluation of diagnostic and therapeutic methods in hyperinsulinism in newborn infants and infants. Apropos of a retrospective study of 26 cases].
abstract: This report concerns 26 cases of hyperinsulinism in neonates and infants. Clinically, the syndrome beginning in the neonatal period seems to differ by several aspects from the disease with a later onset: diagnostic difficulties, treatment modalities and long term prognosis. Insulinemia determined when the patient presents with hypoglycemia is often (75 to 80% of cases) normal. The diagnosis of hyperinsulinism has to be based on the clinical characteristics of hypoglycemia (timing of the episodes, response to glucagon) and on specific biological data (C-peptide, plasma ketone bodies and branched-chain amino acids at the time of hypoglycemia induced by fasting). The pathogenesis of hyperinsulinism is still poorly understood and has several etiologies.
authors.affiliation: Départment de Pédiatrie, Hôpital des Enfants-malades, Paris.
authors.forename: P
authors.initials: P
authors.lastname: Labrune
authors.forename: J P
authors.initials: JP
authors.lastname: Bonnefont
authors.forename: C
authors.initials: C
authors.lastname: Nihoul-Fékété
authors.forename: C
authors.initials: C
authors.lastname: Nezeloff
authors.forename: W
authors.initials: W
authors.lastname: Gepts
authors.forename: P
authors.initials: P
authors.lastname: Czernichow
authors.forename: R
authors.initials: R
authors.lastname: Rappaport
authors.forename: J M
authors.initials: JM
authors.lastname: Saudubray
mesh_terms: D005069:Evaluation Studies as Topic; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006946:Hyperinsulinism / Q000175:diagnosis / Q000628:therapy; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008722:Methods; D011247:Pregnancy; D012189:Retrospective Studies; D013997:Time Factors
vernacular_title: Evaluation des méthodes diagnostiques et thérapeutiques de l'hyperinsulinisme du nouveau-né et du nourrisson. A propos de l'étude rétrospective de 26 enfants.