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[Protein C congenital deficiency. A case report]
María Lourdes de Lemus-Varela1, José de Jesús Arriaga-Dávila, Martha PatriciaSalinas-López
1Departamento de Neonatología, Hospital de Pediatria del Centro Médico Nacional de Occidente, IMSS, Guadalajara, Jal., México. lulalemus@hotmail.com
Gaceta Medica De Mexico
|July 20, 2005
Summary
Congenital Protein C deficiency in a newborn caused severe thrombosis and necrosis, necessitating amputation. Further research into Protein C monoclonal antibodies is crucial for improved treatment and quality of life.
Area of Science:
- Biochemistry
- Hematology
- Pediatric Medicine
Background:
- Protein C is a vitamin K-dependent hepatic protein crucial for regulating thrombin formation and preventing thrombosis.
- Congenital deficiencies in Protein C can lead to severe thrombotic events, particularly in newborns.
Observation:
- A male newborn presented with progressive cyanosis and a necrotic lesion on the right foot.
- Clinical signs included pallor and tachycardia, suggestive of a severe systemic process.
Findings:
- Laboratory results revealed significantly prolonged partial thromboplastin time (58.29 sec) and reduced Protein C functionality (20%).
- Despite initial treatment with antibiotics and heparin, the patient required intratuberous amputation due to extensive necrosis.
Implications:
- This case highlights the critical role of Protein C in neonatal hemostasis and the devastating consequences of its deficiency.
- Further investigation into the therapeutic potential of Protein C monoclonal antibodies is warranted for managing congenital Protein C deficiency and improving patient outcomes.