Transient deficiency of protein S after past infection process, complications and outcome: clinical case

• Published in: Pediatricheskai͡a︡ farmakologii͡a︡ : nauchno-prakticheskiĭ zhurnal Soi͡u︡za pediatrov Rossii Vol. 20; no. 1; pp. 69 - 76
• Main Authors: Gordeeva, Olga B., Dobrotok, Albina V., Selimzianova, Liliia R., Deeva, Valeria A.
• Format: Journal Article
• Language: English; Russian
• Published: Union of pediatricians of Russia 01.03.2023
• Subjects: clinical case; deficiency of protein s; dic syndrome; hemostasis; purpura fulminans
• ISSN: 1727-5776; 2500-3089
• DOI: 10.15690/pf.v20i1.2532

Background . Purpura fulminans (PF) – is an acute rapidly progressive thrombosis of small-diameter blood vessels located mainly on the skin of the extremities. PF is characterized by high mortality rates. Patients can have serious consequences, including amputations and loss of fingers, foots or even extremities in general. Clinical case description. A clinical case of developing transient deficiency of protein S complicated by idiopathic PF on the 7th day after acute otitis in a 3-year-old boy due to past infection is presented. The progression of the disease developed within a few hours. The patient became hemorrhagic elements on the skin of extremities, and later — tissue necrosis. The conducted therapy facilitated to stop the pathological process in the form of PF, and also prevented the development of severe disabling complications in the child. Conclusion . Differential diagnosis and treatment should be fast and accurate, as the development of PF occurs in mere hours. Diagnostics should include expanded panel of coagulological tests and instrumental examinations, which determines the justifiability of a multidisciplinary approach in the patient management with disorders in the hemostatic system.