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Macro creatine kinase types 1 and 2: clinical significance in neonates and children as compared with adults
Insights
This study reports on macro creatine kinase (CK) in pediatric patients, identifying macro CK type 1 in children for the first time and linking macro CK type 2 to myocardial damage in children and malignancies in adults.
Area of Science:
- Biochemistry
- Clinical Chemistry
- Pediatrics
Background:
- Creatine kinase (CK) is a crucial enzyme in cellular energy metabolism.
- Macro creatine kinase (macro CK) is an uncommon form of CK, typically observed in adults.
- Understanding macro CK prevalence and associations in pediatric populations is limited.
Observation:
- Seven pediatric patients (4 days to 12 years) presented with significant macro CK activity.
- Two pediatric cases of macro CK type 1 (CK-BB isoenzyme bound to IgG) were identified.
- Five pediatric cases of macro CK type 2 (polymeric mitochondrial CK) were observed.
Findings:
- Macro CK type 1, previously seen mainly in older women, is reported in children for the first time.
- Four of five pediatric macro CK type 2 cases showed evident myocardial muscle damage.
- Adult macro CK type 2 cases were predominantly associated with neoplasms, often with liver metastases.
Implications:
- Macro CK type 1 occurrence in children broadens its known demographic profile.
- Macro CK type 2 in pediatric patients may indicate myocardial damage, potentially reversible.
- Macro CK type 2 in adults is a marker for poor prognosis in malignancies.
Abstract:
We examined the case histories of seven pediatric patients (ages four days to 12 years) whose sera showed significant activities of "macro" creatine kinase (CK; EC 2.7.3.2): two cases of macro CK type 1 (CK-BB isoenzyme bound to IgG) and five cases of macro CK type 2 (polymeric complexes of mitochondrial CK). The diagnoses of the two children with macro CK type 1 were similar to those in 55 adult patients with this form of CK found earlier during screening of a population of 5000. Macro CK type 1 has generally been noted only in older women; this is the first report of it in children. In four of the five cases of macro CK type 2 we found in children, myocardial muscle damage was clinically evident. In contrast, the 26 cases of adults with macro CK type 2 found during the same screening generally had neoplasms, most with metastases to the liver. We suggest that the finding of macro CK type 2 is an indicator of cellular necrosis; in pediatric cases with myocardial damage the associated conditions may be reversible but in adults with malignancies the finding is associated with an extremely poor prognosis.