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December 1990, Vol 144, No. 12 >
Article | December 1990

Picture of the Month FREE

Kip R. Hartman, MD; James S. Rawlings, MD; Murray Feingold, MD
[+] Author Affiliations

Accepted for publication April 16, 1990.

Reprint requests to the National Birth Defects Center, Franciscan Children's Hospital, 30 Warren St, Brighton, MA 02135 (Dr Feingold).


Am J Dis Child. 1990;144(12):1353-1354. doi:10.1001/archpedi.1990.02150360079026.
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Denouement and Discussion 

Protein C Deficiency 

Manifestations  Protein C, produced by the liver, is essential in the regulation of coagulation and is a vitamin K—dependent proenzyme. A proenzyme is an inactive precursor that is converted to an active enzyme by the action of acid, another enzyme, or by other means. Protein C rapidly inactivates the active forms of V and VIII, resulting in an inhibited coagulation process. In the absence of protein C, thromboses (venous and microvascular) and skin necrosis take place. Skin necrosis usually occurs during the first 2 to 12 hours after birth, and consists of ecchymotic areas that progress to indurated purple-black lesions with central bullae and peripheral erythema. They occur most commonly in the scalp (associated with vertex presentation deliveries), buttocks, and areas in which there is soft-tissue trauma due to pressure. The lesions are similar to the skin lesions present in warfarininduced necrosis of the

REFERENCES

1 +
Hartman KR, Manco-Johnson M, Rawlings JS, Bower DJ, Marlar RA.  Homozygous protein C deficiency: early treatment with warfarin . Am J Pediatr Hematol Oncol . 1989;;11:395-401.
2 +
Marlar RA, Montgomery RR, Broekmans AW, et al.  Diagnosis and treatment of homozygous protein C deficiency . J Pediatr . 1989;;114: 528-534.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Hartman KR, Manco-Johnson M, Rawlings JS, Bower DJ, Marlar RA.  Homozygous protein C deficiency: early treatment with warfarin . Am J Pediatr Hematol Oncol . 1989;;11:395-401.
2 +
Marlar RA, Montgomery RR, Broekmans AW, et al.  Diagnosis and treatment of homozygous protein C deficiency . J Pediatr . 1989;;114: 528-534.

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