title: [Delayed severe decompensation of leucinosis. Significance of emergency blood exchange transfusion. Apropos of 2 cases].
abstract: The observations of two gypsy cousins born to consanguineous parents and exhibiting classical leucinemia are reminders that severe, life-threatening ketotic decompensations may occur at any time. Emergency exchange-transfusions in these two children aged 3 and 5 years reversed the course of extremely severe attacks. Medium term evaluations showed very satisfactory neuropsychologic development in both children. Pathogenesis and prevention of such episodes are discussed.
authors.affiliation: Service de Pédiatrie, Néonatologie, Centre Hospitalier Intercommunal de Tarbes.
authors.forename: J C
authors.initials: JC
authors.lastname: Netter
authors.forename: S
authors.initials: S
authors.lastname: Sabathie
authors.forename: G
authors.initials: G
authors.lastname: Cossarizza
authors.forename: F
authors.initials: F
authors.lastname: Rance
authors.forename: M
authors.initials: M
authors.lastname: Petrus
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications / Q000628:therapy*; D002675:Child, Preschool; D004630:Emergencies; D005078:Exchange Transfusion, Whole Blood; D005192:Family Health; D005260:Female; D006801:Humans; D007662:Ketosis / Q000209:etiology; D007930:Leucine / Q000378:metabolism*; D013997:Time Factors
chemical_list: D007930:Leucine
vernacular_title: Décompensations sévères tardives des leucinoses. Intérêt de l'exsanguino-transfusion en urgence. A propos de deux cas.