Haff disease medical therapy: Difference between revisions
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==Medical therapy== | ==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.<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 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> | ||
===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>===== | ||
* '''Potassium levels less than 6 mEq/L without EKG changes:''' managed with potassium binders, and use of bicarbonate in fluids | *'''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. | *'''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<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.<br /> | *'''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> === | |||
* '''Disseminated intravascular coagulation:''' should be managed with fresh frozen plasma, cryoprecipitate, and platelet transfusion. | |||
* '''Compartment syndrome:''' emergent orthopedic consultation is required<br /> | |||
==References== | ==References== | ||
Revision as of 06:24, 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
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