title: Congenital hyperinsulinism: exclusive human milk and breastfeeding.
abstract: Congenital hyperinsulinism is a genetic condition causing dysregulation of insulin and results in persistent hypoglycemia. The most common types are sulfonylurea receptor (SUR1), potassium inward rectifying channel (Kir6.2), glutamate dehydrogenase (GDH), and glucokinase (GK), with SUR1 and Kir6.2 being the most prevalent. It is imperative that these infants undergo diagnostic testing, which includes genetic, neonatal fasting study to induce hypoglycemia, glucagon stimulation, and imaging. Once a diagnosis has been made, surgical intervention may be needed to help regulate blood glucose levels. During this diagnostic process and as the infant is undergoing treatment, there may be little concern for the mother's feeding plan. Because human milk is the preferred form of nutrition for all infants, these mothers should receive prenatal counseling regarding the initiation and maintenance of milk supply. Parenteral nutrition may be necessary to maintain blood glucose to support human milk administration and breastfeeding.
authors.affiliation: Newborn/Infant Intensive Care Unit, Children's Hospital of Philadelphia, Pennsylvania (Ms Edwards and Dr Spatz); and University of Pennsylvania School of Nursing, Wilmington, Delaware (Dr Spatz).
authors.forename: Taryn M
authors.initials: TM
authors.lastname: Edwards
authors.forename: Diane L
authors.initials: DL
authors.lastname: Spatz
mesh_terms: D001942:Breast Feeding / Q000379:methods*; D044903:Congenital Hyperinsulinism / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D003981:Diazoxide / Q000627:therapeutic use; D005260:Female; D005440:Fluid Therapy; D005527:Food, Fortified; D005765:Gastrointestinal Agents / Q000627:therapeutic use*; D005934:Glucagon / Q000627:therapeutic use; D005947:Glucose / Q000627:therapeutic use*; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology / Q000517:prevention & control*; D007231:Infant, Newborn; D008895:Milk, Human; D015282:Octreotide / Q000627:therapeutic use; D010180:Pancreatectomy; D013549:Sweetening Agents / Q000627:therapeutic use*
chemical_list: D005765:Gastrointestinal Agents; D013549:Sweetening Agents; D005934:Glucagon; D005947:Glucose; D003981:Diazoxide; D015282:Octreotide