Table 7.

Principles of Treatment for Individuals with HOKPP

GoalMeansPractical Details
To avoid triggering or aggravating factors for paralytic attacksAvoid:
  • Strenuous effort
  • Prolonged immobility
  • Carbohydrate-rich diet
  • Monitor episodes of weakness noting time of day & specific triggers
  • Provide dietary review/counseling
Treatment of paralytic attack:
  • Shorten/prevent aggravation of the weakness episode
  • Normalize kalemia
  • Provide K+ supplementation (oral, or IV if oral impossible or if potassium very low)
  • Avoid glucose intake
  • Do not use slow-release forms of potassium
  • Oral potassium: initially, 1 mEq/kg; add 0.3 mEq/kg after 30 minutes if no improvement
  • IV potassium: 0.3 mEq/kg/h
Preventive treatment for paralytic attacksDaily K+ supplementationSlow-release forms of potassium may be used
Diamox (acetazolamide)
K+-sparing diuretics
Preventive treatment for late-onset myopathyDiamox (acetazolamide)?
Medical precautions
  • Avoid corticosteroids if possible
  • Use alpha- or beta adrenergic drugs w/caution, even in local anesthesia or ophthalmology
Other elements of management
  • Kinesitherapy in case of permanent pelvic deficit
  • Adaptive measures: (1) at school & especially for sports; (2) in the work setting

From: Hypokalemic Periodic Paralysis

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Adam MP, Ardinger HH, Pagon RA, et al., editors.
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