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By Everlon Cid Rigobelo

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1999) Autoimmune hypoglycemia. J. (2002) Fatty acid oxidation disorders-. H. (2001) Mitochondrial fatty acid oxidation disorders. , Cohen P, (2008) Disordes of growth hormone/insulin-like growth factor secretion and action. : Pediatric endocrinology. M. (2004) Initial hypoglycemia and neonatal brain injury in term infants with severe fetal acidaemia. , Brodhel J. (2002) The mutation spectrum of the facilitative glucose transporter gene SLC2A2 (GLUT2) in patients with Fanconi-Bickel syndrome. , Salvo I.

However, in addition to hyperinsulinism, or persistent hyperinsulinemic hypoglycemia of infancy (PHHI), hypoglycemia occurs due to limited glycogen stores (eg, prematurity, intrauterine growth retardation), depleted glycogen stores (eg, stress in perinatal asphyxia, starvation), in ketotic hypoglycemia, easily depleted glycogen stores, in combination with inadequate production of glucose through gluconeogenesis, contribute to hypoglycemia (Cranmer, 2009, Guideline, 2004). Thus, fatty acid oxygenation is required to provide substrate for gluconeogenesis and ketogenesis.

POMC mutations has been indentified as a cause of hypocorticism in this atypical clinical presentation (Krude et al, 1998). Idiopathic ACTH deficiency can be acquired in childhood, adolescence and also later in life. An association with autoimmune thyroiditis or celiac disease suggests autoimmune hypophysitis. g. head trauma, pituitary infarction, infection, cranial radiation and tumors. Patients with primary adrenal insufficiency are hyperpigmented due to oversecretion of proopiomelanocortin (POMC) and subsequently high ACTH and MSH levels, hypoglycemia is usually associated with typical mineral disturbances.

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