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Refractory familial hypokalaemic periodic paralysis leading to cardiovascular compromise
  1. Dominic O Awuah1,
  2. Murtaza S Hussain1,
  3. Anoosha Ponnapalli1,
  4. Smit Sunil Deliwala1,
  5. Elfateh M Seedahmed2 and
  6. Ghassan Bachuwa1
  1. 1Internal Medicine, Hurley Medical Center, Flint, Michigan, USA
  2. 2Pulmonary Critical Care, Hurley Medical Center, Flint, Michigan, USA
  1. Correspondence to Dr Murtaza S Hussain; mhussai2{at}hurleymc.com

Abstract

Familial hypokalaemic periodic paralysis (FHPP) is a rare neuromuscular disorder that is classified under periodic paralysis (PP), which is characterised by episodes of muscle weakness. Common triggers include intense exercise, fasting or consumption of carbohydrate-rich meals. Hypokalaemic PP has an incidence of 1 in 100 000; despite the temporal association, cardiac manifestations are exceedingly rare. We present a case of FHPP, a channelopathy presenting with severe refractory hypokalaemia. The challenges with our patient were maintaining potassium levels within normal ranges and initiating a close follow-up plan. Due to the lack of clinical guidance in our case, many aspects of care, including surveillance, medications and genetic testing, remain unaddressed. Medical management includes aggressive correction with supplements, potassium-sparing diuretics and carbonic anhydrase inhibitors. Severe cases of dysrhythmias, especially ventricular fibrillation, require electrophysiology evaluation and possible implantation of a defibrillator to prevent sudden cardiac death.

  • fluid electrolyte and acid-base disturbances
  • pacing and electrophysiology
  • arrhythmias
  • metabolic disorders
  • adult intensive care

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Footnotes

  • Twitter @smitdeliwalaMD

  • Contributors DA—conception, design, draft, review. MH—draft, review. Anoosha Ponnapalli—acquisition, data collection. Abdullahi E. Mahgoub—acquisition, data collection. Elfateh Seedahmed—draft, review. SSD—draft, review. GB—draft, review (multiple authors form attached).

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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