Literature DB >> 21576049

Protein C deficiency: a case review.

Alicia Kelly1, Gregory D Pearson.   

Abstract

Protein C (PC) deficiency is a rare but life-threatening bleeding disorder that can present in the immediate neonatal period. This article presents the case of a baby girl with acute and progressive neonatal purpura fulminans as the presenting feature of PC deficiency. Other common complications of this disease include ophthalmic problems and central nervous system (CNS) changes. Management consists of correcting the coagulopathy, intensive wound care including negative-pressure dressings and skin grafting, and supportive care for the ophthalmic and CNS issues. Long-term follow-up consists of lifelong anticoagulant therapy to avoid recurrence of these complications.

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Year:  2011        PMID: 21576049     DOI: 10.1891/0730-0832.30.3.153

Source DB:  PubMed          Journal:  Neonatal Netw        ISSN: 0730-0832


  2 in total

1.  A delayed presentation of homozygous protein C deficiency in a series of children: a report on two molecular defects.

Authors:  Abdullah A Baothman; Enaam AlSobhi; Hassan A Khayat; Raed E Alsulami; Abdulaziz S Alkahtani; Abdelraheem A Al-Thobyani; Yousef I Marzouk; Mohammad A Abdelaal
Journal:  Clin Case Rep       Date:  2017-02-06

2.  Homozygous protein C deficiency presenting as neonatal purpura fulminans: management with fresh frozen plasma, low molecular weight heparin and protein C concentrate.

Authors:  Hande Kizilocak; Nihal Ozdemir; Gürcan Dikme; Begum Koc; Tiraje Celkan
Journal:  J Thromb Thrombolysis       Date:  2018-02       Impact factor: 2.300

  2 in total

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