Treatments for Franek-Bocker-Kahlen syndrome
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Hypernatremia:
Treatment
(In a Page: Signs and Symptoms)
-
Patients with severe dehydration and hypotension should be treated emergently with IV fluids (lactated Ringer's or NSS)
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Calculate free water deficit:
0.6 ×weight (kg) ×[(Na+measured/140) – 1]
–Correct free water deficit over 48–72 hours; give patient
maintenance fluids and replacements for ongoing losses
–Reduce serum Na+by no more than 10–15 mEq/L/day
(0.5 mEq/L/hour) in chronic hypernatremia and 1
mEq/L/hr in acute hypernatremia
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Too-rapid correction of serum Na+can precipitate seizures or cerebral edema with ensuing herniation
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Isovolemic hypernatremia: Replace fluid with D5W (replace half of fluid deficit in the first 24 hours)
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Hypovolemic hypernatremia: Replace fluid with NSS
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Hypervolemic hypernatremia: Administer D5W and loop diuretics both to decrease hypertonicity by increasing Na+excretion and to add free H2O while removing volume
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Source: In a Page: Signs and Symptoms, 2004
Hypernatremia:
Treatment
(In A Page: Pediatric Signs and Symptoms)
-
If dehydration is present
–Fluid resuscitation with normal saline (20 cc/kg bolus)
–Water deficit =0.6 ×weight ×(1
– 140/[Na+])
–Administer hypotonic IV solutions to correct sodium and rehydrate over 48 hours
-
Too rapid correction can result in water shift into brain cells (due to the presence of “idiogenic” osmoles produced in response to the hypernatremia) resulting in cerebral edema
-
Central DI
–Treat with exogenous vasopressin (DDAVP)
- Nephrogenic DI
–Treat with infusions of hypotonic saline or D5W (depending on the clinical situation and hydration status)
–Allow free access to water
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Source: In A Page: Pediatric Signs and Symptoms, 2007
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