Literature DB >> 29270858

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

Hande Kizilocak1, Nihal Ozdemir2, Gürcan Dikme3, Begum Koc3, Tiraje Celkan3.   

Abstract

Purpura fulminans in neonates is a rapidly progressive thrombotic disorder manifesting as hemorrhagic skin infarction and disseminated intravascular coagulation. Being inherited in an autosomal dominant manner, it is a medical emergency. Clinical presentations of patients may vary depending on the genetic mutations. Retinal and intracranial hemorrhages are the worst clinical scenarios with persistent morbidity. During acute phase, fresh frozen plasma, protein C concentrates and anticoagulant therapy should be administered rapidly. Here we report a patient with homozygous protein C deficiency.

Entities:  

Keywords:  Fresh frozen plasma; Low molecular weight heparin; Neonatal purpura fulminans; Protein C concentrate; Protein C deficiency

Mesh:

Substances:

Year:  2018        PMID: 29270858     DOI: 10.1007/s11239-017-1606-x

Source DB:  PubMed          Journal:  J Thromb Thrombolysis        ISSN: 0929-5305            Impact factor:   2.300


  21 in total

Review 1.  How I treat pediatric venous thromboembolism.

Authors:  Guy Young
Journal:  Blood       Date:  2017-08-03       Impact factor: 22.113

2.  Perinatal management of patients at high risk of homozygous protein C deficiency.

Authors:  Chris Barnes; Fiona Newall; Shane Higgins; Susan Carden; Paul Monagle
Journal:  Thromb Haemost       Date:  2002-08       Impact factor: 5.249

3.  A new vitamin K-dependent protein. Purification from bovine plasma and preliminary characterization.

Authors:  J Stenflo
Journal:  J Biol Chem       Date:  1976-01-25       Impact factor: 5.157

4.  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

5.  Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrate.

Authors:  M Dreyfus; J F Magny; F Bridey; H P Schwarz; C Planché; M Dehan; G Tchernia
Journal:  N Engl J Med       Date:  1991-11-28       Impact factor: 91.245

6.  Neonatal purpura fulminans due to protein C deficiency.

Authors:  Shreya Sharma; Jagadeesh Anbazhagan; Nishad Plakkal
Journal:  Arch Dis Child Fetal Neonatal Ed       Date:  2015-03-17       Impact factor: 5.747

7.  Protein C deficiency: a case review.

Authors:  Alicia Kelly; Gregory D Pearson
Journal:  Neonatal Netw       Date:  2011 May-Jun

Review 8.  Heparin for the treatment of thrombosis in neonates.

Authors:  Olga Romantsik; Matteo Bruschettini; Simona Zappettini; Luca Antonio Ramenghi; Maria Grazia Calevo
Journal:  Cochrane Database Syst Rev       Date:  2016-11-07

9.  Ophthalmic manifestations of neonatal protein C deficiency.

Authors:  A Cassels-Brown; A M Minford; S L Chatfield; J A Bradbury
Journal:  Br J Ophthalmol       Date:  1994-06       Impact factor: 4.638

10.  Clinical Data of Neonatal Systemic Thrombosis.

Authors:  Paola Saracco; Rossana Bagna; Chiara Gentilomo; Mariella Magarotto; Alice Viano; Federica Magnetti; Paola Giordano; Matteo Luciani; Angelo Claudio Molinari; Agnese Suppiej; Luca Antonio Ramenghi; Paolo Simioni
Journal:  J Pediatr       Date:  2016-01-16       Impact factor: 4.406

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  1 in total

1.  Four-extremity amputation following disseminated intravascular coagulation and purpura fulminans.

Authors:  Umar F Bhatti; Aaron M Williams; Krishnan Raghavendran; Patrick E Georgoff
Journal:  BMJ Case Rep       Date:  2019-03-20
  1 in total

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