[Effect of danazol on heterozygous c protein coagulation deficiency exacerbated by Salmonella typhi sepsis]

Area of Science:

• Pediatric Hematology
• Infectious Diseases
• Vascular Medicine

Background:

• Heterozygous protein C deficiency is a rare inherited thrombophilia.
• Sepsis can precipitate severe thrombotic events in patients with underlying coagulopathies.
• Salmonella typhi sepsis is a known trigger for disseminated intravascular coagulation and thrombosis.

Observation:

• An 8-month-old male infant presented with sepsis and arterial thrombosis in the lower right limb.
• The patient had a pre-existing heterozygous protein C deficiency, which was exacerbated by the Salmonella typhi infection.
• Necrosis of the distal limb was evident, posing a risk of amputation.

Findings:

• Combined treatment with heparin and danazol led to increased functional protein C levels.
• The necrotic phenomenon in the limb resolved, and amputation was averted.
• Danazol demonstrated efficacy in managing the thrombotic complications associated with protein C deficiency.

Implications:

• This case highlights the critical role of managing underlying thrombophilias during severe infections.
• Danazol may be a valuable therapeutic option for patients with protein C deficiency experiencing severe thrombotic events.
• Early and aggressive treatment can prevent severe morbidity, such as limb loss, in pediatric thrombophilia cases.