Producción CyT
Congreso
Autoría
Braslavsky, Debora
;
Ballerini, María G
;
MARTIN, AYELEN
;
Rodriguez María Eugenia
;
Gil, Melina
;
Keselman, Ana
;
Ropelato, María G
;
Pennisi, Patricia
;
Bergadá, Ignacio
Fecha
2023
Editorial y Lugar de Edición
Karger AG
Resumen
Información suministrada por el agente en
SIGEVA
Background: Persistent hypoglycemia in pediatric patients isthe clinical manifestation of different hormonal and metabolic disordersand expose them to a high risk of brain damage. Appropriatemanagement should include staggered strategy to achieve theunderlying etiology.Aim: To describe an infant with recurrent hypoglycemia due toStage 4 Neuroblastoma (4SNB) behaving as an “IGF2-OMA”.Case Report: A 2-month boy admitted with a huge high consistencyabdominal mass and multiple bluish cu...
Background: Persistent hypoglycemia in pediatric patients isthe clinical manifestation of different hormonal and metabolic disordersand expose them to a high risk of brain damage. Appropriatemanagement should include staggered strategy to achieve theunderlying etiology.Aim: To describe an infant with recurrent hypoglycemia due toStage 4 Neuroblastoma (4SNB) behaving as an “IGF2-OMA”.Case Report: A 2-month boy admitted with a huge high consistencyabdominal mass and multiple bluish cutaneous nodules.Male genitalia, Tanner stage I, testicular volume of 2 ml. 4SNB wasconfirmed. While being critically ill, recurrent hypoglycemic episodesoccurred (glycemia levels 13 to 36 mg/dL). Critical sampleshowed non-detectable insulin and C peptide, bOHbutirate 0.05mmol/L, NEFA 0.49 mmol/L, GH 0.27 ng/mL, IGF1 <15 ng/mL,IGFBP3 0.9 μg/mL and cortisol 15.1 μg/dL. Non clear cause of persistenthypoglycemia in the presence of large abdominal tumor,raised the question of tumor´s overproduction of IGF2 andBig-IGF2.Methods: IGF2, Big-IGF2 and IGFBP-2 measurement wereevaluated by western immunoblotting from patient´s serum samplesat three stages of the disease, two healthy infants and twogrowth hormone deficient (GHD) patients age-matchedcontrols.Results: Strong high molecular weight IGF2 bands (Big-IGF2,MW above 17Kda) were present only in samples from index caseduring clinical recurrent hypoglycemia and absent in controls,while bands corresponding to IGF2 (MW below 17KDa) wereobserved in healthy controls and absent in GHD patients. IGFBP2bands were more intense in the index patient than in GHD andhealthy controls. Temporal measurement of Big-IGF2 bandsshowed progressive reduction, in correlation to clinical improvementand normalization of blood glucose. Hypoglycemia wasattributed to the diagnosis of IGF2-OMA and only support treatmentwas instituted to control glycemia.Conclusions: IGF2 overproduction is a rare complication ofmany solid tumors and should be considered on the differentialdiagnosis if hypoglycemia occurs. Excessive IGF2 and Big-IGF2interferes in the normal GH-IGFs-Insulin pathways by bindinginsulin receptors and IGF1 receptors and overstimulates the postreceptor pathways. The biochemical pattern of hyperinsulinemic hypoglycemia in the absence of elevated serum insulin and C peptideassociated to low GH, IGF-1 and IGFBP3 mimicking GHD,suggests IGF2/Big-IGF2 overproduction as the stimulating factorof glucose consumption as well as inhibition of the GH/IGF axis.IGF2-OMAs should be consider as a potential cause of hypoglycemiain infants and children with solid tumors without clear evidenceof other common causes.
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Palabras Clave
IGFBP2IGF12HYPOGLYCEMIA