Haff disease medical therapy: Difference between revisions
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==Overview== | ==Overview== | ||
The medical therapy of Haff disease consists of managing rhabdomyolysis, electrolyte abnormalities, and supportive care. | |||
==Medical therapy== | ==Medical therapy== | ||
===Management of Rhabdomyolysis=== | ===Management of [[Rhabdomyolysis]]=== | ||
The goal of rhabdomyolysis is adequate fluid hydration with normal isotonic saline to prevent acute kidney injury. Urine output of 200 to 300 mL/h should be maintained with daily serial monitoring of CPK level for to document a downward trend. In case of fluid overload from aggressive fluid resuscitation, loop diuretics may be considered.<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref> In severe cases with CPK level > 30,000 IU/L, alkalization of the urine with bicarbonate can be considered.<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref><ref name="pmid31843046">{{cite journal| author=Pei P, Li XY, Lu SS, Liu Z, Wang R, Lu XC | display-authors=etal| title=The Emergence, Epidemiology, and Etiology of Haff Disease. | journal=Biomed Environ Sci | year= 2019 | volume= 32 | issue= 10 | pages= 769-778 | pmid=31843046 | doi=10.3967/bes2019.096 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31843046 }} </ref> | The goal of rhabdomyolysis is adequate fluid hydration with normal [[isotonic saline]] to prevent acute kidney injury. Urine output of 200 to 300 mL/h should be maintained with daily serial monitoring of [[CPK elevation|CPK]] level for to document a downward trend. In case of fluid overload from aggressive fluid resuscitation, [[Loop diuretic|loop diuretics]] may be considered.<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref> In severe cases with CPK level > 30,000 IU/L, alkalization of the urine with bicarbonate can be considered.<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref><ref name="pmid31843046">{{cite journal| author=Pei P, Li XY, Lu SS, Liu Z, Wang R, Lu XC | display-authors=etal| title=The Emergence, Epidemiology, and Etiology of Haff Disease. | journal=Biomed Environ Sci | year= 2019 | volume= 32 | issue= 10 | pages= 769-778 | pmid=31843046 | doi=10.3967/bes2019.096 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31843046 }} </ref> | ||
===Management of Electrolytes Abnormalities=== | ===Management of Electrolytes Abnormalities=== | ||
Rhabdomyolysis is associated with hyperkalemia and hypocalcemia.<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref> | Rhabdomyolysis is associated with [[hyperkalemia]] and [[hypocalcemia]].<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref> | ||
=====Hyperkalemia<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref>===== | =====Hyperkalemia<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref>===== | ||
| Line 19: | Line 21: | ||
=====Hypocalcemia<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref>===== | =====Hypocalcemia<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref>===== | ||
*'''Symptomatic hypocalcemia:''' should be treated with IV calcium gluconate. | *'''Symptomatic hypocalcemia:''' should be treated with [[IV calcium gluconate]]. | ||
=== Other Supportive Care<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref> === | ===Other Supportive Care<ref name="pmid28846335">{{cite journal| author=| title=StatPearls | journal= | year= 2022 | volume= | issue= | pages= | pmid=28846335 | doi= | pmc= | url= }} </ref>=== | ||
* '''Disseminated intravascular coagulation:''' should be managed with fresh frozen plasma, cryoprecipitate, and platelet transfusion. | *'''[[Disseminated intravascular coagulation]]:''' should be managed with fresh frozen plasma, cryoprecipitate, and platelet transfusion. | ||
* '''Compartment syndrome:''' emergent orthopedic consultation is required<br /> | *'''[[Compartment syndrome]]:''' emergent orthopedic consultation is required<br /> | ||
==References== | ==References== | ||
Revision as of 06:26, 23 April 2022
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S
Overview
The medical therapy of Haff disease consists of managing rhabdomyolysis, electrolyte abnormalities, and supportive care.
Medical therapy
Management of Rhabdomyolysis
The goal of rhabdomyolysis is adequate fluid hydration with normal isotonic saline to prevent acute kidney injury. Urine output of 200 to 300 mL/h should be maintained with daily serial monitoring of CPK level for to document a downward trend. In case of fluid overload from aggressive fluid resuscitation, loop diuretics may be considered.[1] In severe cases with CPK level > 30,000 IU/L, alkalization of the urine with bicarbonate can be considered.[1][2]
Management of Electrolytes Abnormalities
Rhabdomyolysis is associated with hyperkalemia and hypocalcemia.[1]
Hyperkalemia[1]
- Potassium levels less than 6 mEq/L without EKG changes: managed with potassium binders, and use of bicarbonate in fluids
- Potassium levels 6 mEq/L or above with or without EKG changes: ampule of D50 followed by zero units of regular insulin, and IV sodium bicarbonate.
Hypocalcemia[1]
- Symptomatic hypocalcemia: should be treated with IV calcium gluconate.
Other Supportive Care[1]
- Disseminated intravascular coagulation: should be managed with fresh frozen plasma, cryoprecipitate, and platelet transfusion.
- Compartment syndrome: emergent orthopedic consultation is required