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Extremely elevated creatine kinase

MedGen UID:
868172
•Concept ID:
C4022564
•
Finding
Synonyms: Extremely elevated CPK; Extremely elevated creatine phosphokinase; Extremely elevated phospho-CK serum level; Extremely elevated serum CK level; Extremely high CPK level
 
HPO: HP:0030235

Definition

An increased creatine kinase level more than 50X above the upper normal level. [from HPO]

Conditions with this feature

Proximal myopathy with extrapyramidal signs
MedGen UID:
816615
•Concept ID:
C3810285
•
Disease or Syndrome
Myopathy with extrapyramidal signs is an autosomal recessive disorder characterized by early childhood onset of proximal muscle weakness and learning disabilities. While the muscle weakness is static, most patients develop progressive extrapyramidal signs that may become disabling (summary by Logan et al., 2014). Brain MRI in 1 patient showed congenital malformations, including polymicrogyria and cerebellar dysplasia (Wilton et al., 2020).
See: Condition Record
Proximal myopathy with extrapyramidal signs

Professional guidelines

PubMed

An evidence-based narrative review of the emergency department evaluation and management of rhabdomyolysis.
Long B, Koyfman A, Gottlieb M
Am J Emerg Med 2019 Mar;37(3):518-523. Epub 2019 Jan 2 doi: 10.1016/j.ajem.2018.12.061. PMID: 30630682
The management of acute coronary insufficiency.
Baker J, Wagner GS, Greenfield JC Jr
Dis Mon 1977 May;23(8):1-39. doi: 10.1016/s0011-5029(77)80007-2. PMID: 324738

Curated

American College of Medical Genetics and Genomics, Newborn Screening ACT Sheet, No Pathogenic Variant in Dystrophin (DMD) Gene after elevated creatine kinase muscle isoform (CK-MM), Genetic Neuromuscular Disease, 2020

Recent clinical studies

Etiology

Biomarkers of Cardiac Stress and Injury in Athletes: What Do They Mean?
Donnellan E, Phelan D
Curr Heart Fail Rep 2018 Apr;15(2):116-122. doi: 10.1007/s11897-018-0385-9. PMID: 29520707
Safety of high-dose atorvastatin therapy.
Waters DD
Am J Cardiol 2005 Sep 5;96(5A):69F-75F. doi: 10.1016/j.amjcard.2005.06.028. PMID: 16126026
Bench-to-bedside review: Rhabdomyolysis -- an overview for clinicians.
Huerta-Alardín AL, Varon J, Marik PE
Crit Care 2005 Apr;9(2):158-69. Epub 2004 Oct 20 doi: 10.1186/cc2978. PMID: 15774072Free PMC Article
Statin-associated myopathy.
Thompson PD, Clarkson P, Karas RH
JAMA 2003 Apr 2;289(13):1681-90. doi: 10.1001/jama.289.13.1681. PMID: 12672737
Rhabdomyolysis, myoglobinuria and exercise.
Milne CJ
Sports Med 1988 Aug;6(2):93-106. doi: 10.2165/00007256-198806020-00004. PMID: 3062736

Diagnosis

Acute viral myositis: profound rhabdomyolysis without acute kidney injury.
Kietaibl AT, Fangmeyer-Binder M, Göndör G, Säemann M, Fasching P
Wien Klin Wochenschr 2021 Aug;133(15-16):847-850. Epub 2021 Apr 27 doi: 10.1007/s00508-021-01866-3. PMID: 33905028Free PMC Article
An evidence-based narrative review of the emergency department evaluation and management of rhabdomyolysis.
Long B, Koyfman A, Gottlieb M
Am J Emerg Med 2019 Mar;37(3):518-523. Epub 2019 Jan 2 doi: 10.1016/j.ajem.2018.12.061. PMID: 30630682
Statin-Associated Autoimmune Myopathy: A Systematic Review of 100 Cases.
Nazir S, Lohani S, Tachamo N, Poudel D, Donato A
J Clin Rheumatol 2017 Apr;23(3):149-154. doi: 10.1097/RHU.0000000000000497. PMID: 28277343
Bench-to-bedside review: Rhabdomyolysis -- an overview for clinicians.
Huerta-Alardín AL, Varon J, Marik PE
Crit Care 2005 Apr;9(2):158-69. Epub 2004 Oct 20 doi: 10.1186/cc2978. PMID: 15774072Free PMC Article
Rhabdomyolysis, myoglobinuria and exercise.
Milne CJ
Sports Med 1988 Aug;6(2):93-106. doi: 10.2165/00007256-198806020-00004. PMID: 3062736

Therapy

Dropped head in systemic sclerosis: a case based review.
Shimada T, Higashida-Konishi M, Akiyama M, Hama S, Takei H, Izumi K, Oshima H, Okano Y
Rheumatol Int 2022 Aug;42(8):1483-1489. Epub 2021 Jul 5 doi: 10.1007/s00296-021-04942-z. PMID: 34223951
Statin-Associated Autoimmune Myopathy: A Systematic Review of 100 Cases.
Nazir S, Lohani S, Tachamo N, Poudel D, Donato A
J Clin Rheumatol 2017 Apr;23(3):149-154. doi: 10.1097/RHU.0000000000000497. PMID: 28277343
Safety of high-dose atorvastatin therapy.
Waters DD
Am J Cardiol 2005 Sep 5;96(5A):69F-75F. doi: 10.1016/j.amjcard.2005.06.028. PMID: 16126026
Bench-to-bedside review: Rhabdomyolysis -- an overview for clinicians.
Huerta-Alardín AL, Varon J, Marik PE
Crit Care 2005 Apr;9(2):158-69. Epub 2004 Oct 20 doi: 10.1186/cc2978. PMID: 15774072Free PMC Article
Statin-associated myopathy.
Thompson PD, Clarkson P, Karas RH
JAMA 2003 Apr 2;289(13):1681-90. doi: 10.1001/jama.289.13.1681. PMID: 12672737

Prognosis

Assessment of catabolic state in infants with the use of urinary titin N-fragment.
Fukushima S, Nakanishi N, Fujioka K, Suga K, Shirakawa T, Osawa K, Hara K, Tsutsumi R, Urushihara M, Nakagawa R, Awano H, Oto J, Sakaue H, Iijima K, Matsuo M
Pediatr Res 2022 Jun;91(7):1748-1753. Epub 2021 Jul 17 doi: 10.1038/s41390-021-01658-5. PMID: 34274960
Dropped head in systemic sclerosis: a case based review.
Shimada T, Higashida-Konishi M, Akiyama M, Hama S, Takei H, Izumi K, Oshima H, Okano Y
Rheumatol Int 2022 Aug;42(8):1483-1489. Epub 2021 Jul 5 doi: 10.1007/s00296-021-04942-z. PMID: 34223951
Creatine kinase elevation after robotic surgery for rectal cancer due to a prolonged lithotomy position.
Tsuchiya Y, Munakata S, Tsukamoto R, Okazawa Y, Mizukoshi K, Sugimoto K, Takahashi M, Tomiki YKY, Sakamoto K
BMC Surg 2020 Jun 16;20(1):136. doi: 10.1186/s12893-020-00771-2. PMID: 32546155Free PMC Article
Troponins in acute coronary syndromes.
Scirica BM, Morrow DA
Prog Cardiovasc Dis 2004 Nov-Dec;47(3):177-88. doi: 10.1016/j.pcad.2004.07.004. PMID: 15736583
Rhabdomyolysis, myoglobinuria and exercise.
Milne CJ
Sports Med 1988 Aug;6(2):93-106. doi: 10.2165/00007256-198806020-00004. PMID: 3062736

Clinical prediction guides

Dropped head in systemic sclerosis: a case based review.
Shimada T, Higashida-Konishi M, Akiyama M, Hama S, Takei H, Izumi K, Oshima H, Okano Y
Rheumatol Int 2022 Aug;42(8):1483-1489. Epub 2021 Jul 5 doi: 10.1007/s00296-021-04942-z. PMID: 34223951
Creatine kinase elevation after robotic surgery for rectal cancer due to a prolonged lithotomy position.
Tsuchiya Y, Munakata S, Tsukamoto R, Okazawa Y, Mizukoshi K, Sugimoto K, Takahashi M, Tomiki YKY, Sakamoto K
BMC Surg 2020 Jun 16;20(1):136. doi: 10.1186/s12893-020-00771-2. PMID: 32546155Free PMC Article
Troponins in acute coronary syndromes.
Scirica BM, Morrow DA
Prog Cardiovasc Dis 2004 Nov-Dec;47(3):177-88. doi: 10.1016/j.pcad.2004.07.004. PMID: 15736583
Carnitine-acylcarnitine translocase deficiency, clinical, biochemical and genetic aspects.
Rubio-Gozalbo ME, Bakker JA, Waterham HR, Wanders RJ
Mol Aspects Med 2004 Oct-Dec;25(5-6):521-32. doi: 10.1016/j.mam.2004.06.007. PMID: 15363639
Rhabdomyolysis, myoglobinuria and exercise.
Milne CJ
Sports Med 1988 Aug;6(2):93-106. doi: 10.2165/00007256-198806020-00004. PMID: 3062736

Recent systematic reviews

Statin-Associated Autoimmune Myopathy: A Systematic Review of 100 Cases.
Nazir S, Lohani S, Tachamo N, Poudel D, Donato A
J Clin Rheumatol 2017 Apr;23(3):149-154. doi: 10.1097/RHU.0000000000000497. PMID: 28277343

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • ACMG ACT, 2020
      American College of Medical Genetics and Genomics, Newborn Screening ACT Sheet, No Pathogenic Variant in Dystrophin (DMD) Gene after elevated creatine kinase muscle isoform (CK-MM), Genetic Neuromuscular Disease, 2020

    Consumer resources

    Reviews

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      Related information in PubMed Central Links
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      Related literature resources in PubMed

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