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Morning Report: October 25, 2010 Board Review Today ! 12:00 Topic: Genetics PowerPoint Presentation
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Morning Report: October 25, 2010 Board Review Today ! 12:00 Topic: Genetics. Diabetes Insipidus. Polydipsia , polyuria , dilute urine, Hypernatremia , dehydration. Diabetes Insipidus. Central or Neurogenic DI Destruction of posterior pituitary (tumors/trauma)

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Presentation Transcript
slide1

Morning Report:

October 25, 2010

Board Review Today!

12:00

Topic: Genetics

diabetes insipidus
Diabetes Insipidus

Polydipsia, polyuria, dilute urine,

Hypernatremia, dehydration

diabetes insipidus1
Diabetes Insipidus
  • Central or Neurogenic DI
    • Destruction of posterior pituitary (tumors/trauma)
      • Deficiency of vasopressin
  • Nephrogenic DI
    • Renal tubular resistance to vasopressin
    • Intrinsic receptor defect
    • Medications
compulsive water drinker
Compulsive Water Drinker
  • Physiologic inhibition of vasopressin secretion
  • Female Predominance
  • Usually presents in adulthood
    • May be seen in adolescence
  • >10% of patients with schizophrenia
clinical presentation
Clinical Presentation
  • Infants:
    • Poor feeding, FTT
    • Irritability, seizures
      • hypernatremia, dehydration
    • Vomiting after feeds
    • Diapers “dripping wet”
    • Less severe in breast fed babies (solute load)
    • Inquire about family history
clinical presentation1
Clinical Presentation
  • Older Children:
    • Polyuria, polydipsiawith normal glucose
      • Hypernatremia
    • Neurologic deficits or precocious puberty
      • Neurogenic DI
    • Consider obstructive uropathy
    • Medications
    • Systemic disorders
laboratory tests
Laboratory Tests
  • Compulsive water drinker
    • Low serum osmolality
    • coupled with hypo-osmolar urine
  • Vasopressin deficiency/insensitivity
    • High serum osmolality
      • In setting of normal serum glucose and urea
    • coupled with hypo-osmolar urine
water deprivation test
Water Deprivation Test
  • Follow specific protocol
    • close monitoring
  • Diagnostic criteria of DI (short deprivation)
    • Plasma elevation >10mOsm/kg over baseline
    • Urine SpG remains <1.010
ddavp challenge
DDAVP Challenge
  • If urine osm increased > 450mOsm/kg
    • Establishes central DI
  • If urine osm remains < 200 mOsm/kg
    • Likely nephrogenic DI
  • If urine osmincresed > 750 mOsm/kg
    • Likely compulsive water drinker
slide16
MRI
  • Visualizes:
    • Anterior and posterior pituitary
    • Pituitary stalk
  • Possible pathology
    • Suprasellar mass
    • Pituitary cyst
    • Hypoplasia
    • Ectopic pituitary
management
Management
  • Central DI
    • Intranasal DDAVP
    • Oral repletion of water
    • If IV fluids used
      • No more than 3% dextrose
        • Avoid worsening hyperosmolality
        • Avoid glucosuria
management1
Management
  • Nephrogenic DI
    • Low-Osmolar, low Na diet
    • Human milk in infancy
    • Thiazide diuretic
      • Increases Na loss
    • NSAIDS may have benefit
      • Use only if other methods fail
prognosis
Prognosis
  • Consider genetic testing/counseling
  • Behavioral problems
    • Short attention span, hyperactivity, learning delays
      • ? Exacerbated by frequent trips to bathroom, water source ?
    • Nonobstructive functional hydronephrosis
  • May be transient
  • Caution when pt cannot readily access water