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Creatine kinase variant type I in children with anoxic insult
Insights
Creatine kinase variants (CKV) in children with central hypotonia and hypoxia indicate tissue damage. Neonates with CKV showed higher percentages, suggesting a link between necrosis and Type I CKV pathogenesis.
Area of Science:
- Biochemistry
- Pediatric Neurology
- Clinical Chemistry
Background:
- Central hypotonia and myocardial damage often lead to severe central hypoxia in pediatric patients.
- Creatine kinase variants (CKV) are observed in children with specific neurological and cardiac conditions.
Purpose of the Study:
- To evaluate the prevalence and characteristics of creatine kinase variants (CKV) in pediatric patients with central hypotonia and myocardial damage.
- To investigate the relationship between CKV, birth hypoxia, and neurological outcomes in neonates and older children.
Main Methods:
- Retrospective analysis of 31 pediatric patients with CKV over one year.
- Assessment of clinical history, including central hypotonia, myocardial damage, and birth hypoxia.
- Measurement of total serum creatine kinase (CK) and percentage of CKV.
Main Results:
- Eleven children had CKV; seven were neonates experiencing severe birth hypoxia (Apgar scores 2.8 at 1 min, 4.8 at 5 min).
- Neonates had a mean CKV of 20.7% and total CK of 773 U/L.
- Older children with failure to thrive and cerebral palsy had lower CKV (7.3%) but similar total CK levels.
- Delayed appearance of CKV (3-20 days) was noted in four neonates following increased total CK activity.
Conclusions:
- Tissue necrosis appears to be a significant factor in the pathogenesis of Type I CK variant.
- CKV levels and timing may correlate with the severity of birth hypoxia and subsequent neurological impact in neonates.
Abstract:
We evaluated 31 consecutive patients, including 11 children, with creatine kinase variants (CKV) in our laboratory during a year. All had a clinical history of central hypotonia with myocardial damage resulting in severe central hypoxia. Seven of the 11 were neonates and had Apgar scores of 2.8 (SD 2.3) at 1 min and 4.8 (SD 2.4) at 5 min, which reflected their severe birth hypoxia. For the seven neonates, the mean value for total serum creatine kinase (EC 2.7.3.2; CK) was 773 (SD 553) U/L. The mean percentage CKV was 20.7% (SD 12.5%). The other four children were being evaluated for failure to thrive; all had birth asphyxia with residual cerebral palsy and seizure activity. These older children had a lower percentage of CKV (7.3%, SD 1.01%) than did the neonates, but the total CK (725, SD 1335 U/L) was not significantly different. In four neonates there was a three- to 20-day delay in the appearance of CK variant, which followed a marked increase in total CK activity. Tissue necrosis is apparently an important factor in the pathogenesis of Type I CK variant.