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17 Cards in this Set

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Most important event from transition from fetal to neonatal life is _________.
Adaptation from environment where glucose is readily available (from maternal blood) to environment where glucose is limited and intermittent (eating)
Hormonal changes at birth.
Increased glucagon
Dec'd insulin
Inc'd catechol and GH

Induces: Glycogenlysis, glucneo, lipolysis, and ketogenesis
Which hormones are balanced during euglycemia?
Insulin balanced with GH, Cortisol, and Glucagon
Which hormones predominate during hypoglycemia?
LOTS of insulin

Little GH, Cortisol, Glucagon
Definition of hypoglycemia

Include lab value and symptoms.
Glucose <40

Syx: subtle in babies
May be apnea, resp distress, poor feeding, myoclonic jerks, seizures, somnolence, subnormal temp, sweating
The following are risk factors for ________:
Twins
Prematurity
Diabetic Mother
Small for Gestational Age
Transient neonatal hypoglycemia
Persistent Hypoglycemia:
Differential
CONSIDER EDITING THIS CARD

Hyperinsulinism (genetic)

Hormone Deficiency: panhypopit, isolated GH def, ACTH, gluc, epi deficiencies

Limited Substrates (glycogen storage disease, defect in glucneo)

Disorders of FA oxidation

Drug/tosin

Sepsis

Genetic Dz (Beckwith Wiedemann Syndrome)
Hypoglycemia:
Endocrine Causes vs Metabolic Causes
Endocrine:
Hypopit
Isolated GH deficiency
Adrenal Insuff
Hyperinsulinism

Metabolic causes: MANY
Midline defect and micropenis are signs of ______.
Hypopituitarism
Hepatosplenomegaly is a sign of ____.
Glycogen Storage Disease
Genetic causes of persistent hyperinsulinemic hypoglycemia.
Inactivating mutations ABCC8, KCNJ11 (potassium channel remains closes, leaving Ca2+ open, and insulin constantly released)

Activating mutations GLUD1 (glutamate dehydrogenase--involved in insulin secretion)
Treatment for hyperinsulinemic hypoglycemia.
Diazoxide (binds ABCC8 subunit of K-ATP channel, opens channel, inhibits insulin release)

Ocreotide inhibits beta-cell release of insulin

Surgery (last resort)
Which hormones predominate in hyperglycemia?
HIGH GH, Cortisol, Glucagon

Low Insulin
Neonatal DM:
Definition
Genetic Associations with Transient Disease
Genetic Associations with Permanent Disease
Hyperglycemia within first month of life, lasting over 2 weeks

Abnormalities in chrom 6, complete or partial paternal UPD--maternal chrom 6 is lost

Permanent: homozygous mutation in Pdx-1 or IPF-1; defect in K+-APT channel so that channel is open (ACTIVATING mutation), never release insulin
Treatment for Permanent Neonatal DM
Sulfonylureas (glyburide)

(no need for insulin!!)
Genetic abnormalities in Transient Neonatal DM.
Chrom 6
Genetic abnormalities in Permanent Neonatal DM.
Kir 6.2
SUR1