FREQUENCY AND IMPLICATIONS OF SEVERE NEONATAL PROTEIN C DEFICIENCY
FREQUENCY AND IMPLICATIONS OF SEVERE NEONATAL PROTEIN C DEFICIENCY
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新生儿严重蛋白质 C 缺乏的频率和影响
DOI：
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发表时间：
1987
影响因子：
6.7
通讯作者：
L. Jacobson
中科院分区：
文献类型：
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作者：
M. Manco‐Johnson;T. Abshire;L. Jacobson
The newborn infant has a physiologically low level of protein C which rises very slowly in postnatal life. The frequency and significance of severe neonatal protein C deficiency has not been reported. In this study, protein C levels were measured in 110 newborn infants at the time of birth using functional (amidolytic, Cact) and immunologic (Laurell rocket, Cag) assays. The protein C levels were compared with a marker of thrombin activation (D-dimer fragment of fibrin, +D-D) and infants were subsequently followed for signs and symptoms of thrombosis. Results are summarized below (protein C levels are expressed as U/ml). Thirteen infants had protein C levels compatible with the homozygous deficiency state. Extremely low levels of protein C (<0.20 U/ml) were not found in well term infants and were rarely noted in stable preterm infants. D-D were infrequently present and no thrombosis occurred. Near term infants born with fetal distress frequently showed +D-D but rarely demonstrated extremely low levels of protein C. None of these infants required indwelling arterial catheters and no thromboses occurred. Preterm infants with severe respiratory distress showed lower protein C levels at birth (p <0.01). Although 71% had +D-D, thromboses in these infants were all related to invasive catheterizations. In contrast, the study population of twins demonstrated a high frequency of severe protein C deficiency with negative D-D and frequent thromboses, three of which occurred in the absence of instrumentation. In summary, severe protein C deficiency and thrombin activation are common in sick preterm infants with the risk of thrombosis increased by intravascular catheterization. In contrast, twins with severe protein C deficiency may manifest a thrombotic risk which is independent of thrombin activation or catheterization.
