Literature DB >> 1519965

Protein S deficiency: early presentation and pulmonary hypertension.

J O'Sullivan1, R Chatuverdi, M K Bennett, S Hunter.   

Abstract

Protein S deficiency usually presents in adults with recurrent or unexplained thrombosis and is thought to have autosomal dominant inheritance. We describe the case of a 5 year old girl with pulmonary hypertension but no evidence of venous thrombosis until postmortem examination. Her parents were first cousins. This potentially treatable condition should be sought in children and their relatives screened.

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Year:  1992        PMID： 1519965      PMCID： PMC1793844          DOI： 10.1136/adc.67.7.960

Source DB:  PubMed          Journal:  Arch Dis Child        ISSN： 0003-9888            Impact factor:   3.791


  6 in total

1.  Arterial thrombosis and protein S deficiency.

Authors:  P Sié; B Boneu; R Biermé; M L Wiesel; L Grunebaum; J P Cazenave
Journal:  Thromb Haemost       Date:  1989-11-24       Impact factor: 5.249

2.  A protein S deficient family with portal vein thrombosis.

Authors:  G Sas; G Blaskó; I Petrö; J H Griffin
Journal:  Thromb Haemost       Date:  1985-10-30       Impact factor: 5.249

3.  Protein S deficiency associated with "juvenile" arterial and venous thromboses.

Authors:  P M Mannucci; A Tripodi; R M Bertina
Journal:  Thromb Haemost       Date:  1986-06-30       Impact factor: 5.249

4.  Two genes homologous with human protein S cDNA are located on chromosome 3.

Authors:  J K Ploos van Amstel; A L van der Zanden; E Bakker; P H Reitsma; R M Bertina
Journal:  Thromb Haemost       Date:  1987-12-18       Impact factor: 5.249

5.  Recurrent venous thromboembolism in patients with a partial deficiency of protein S.

Authors:  P C Comp; C T Esmon
Journal:  N Engl J Med       Date:  1984-12-13       Impact factor: 91.245

6.  Inherited deficiency of protein S in a Japanese family with recurrent venous thrombosis: a study of three generations.

Authors:  T Kamiya; T Sugihara; K Ogata; H Saito; K Suzuki; J Nishioka; S Hashimoto; K Yamagata
Journal:  Blood       Date:  1986-02       Impact factor: 22.113
  6 in total
  2 in total

1.  Purpura fulminans in severe congenital protein C deficiency: monitoring of treatment with protein C concentrate.

Authors:  F M Müller; W Ehrenthal; G Hafner; D Schranz
Journal:  Eur J Pediatr       Date:  1996-01       Impact factor: 3.183

2.  Inherited resistance to activated protein C in a boy with multiple thromboses in early infancy.

Authors:  W Zenz; W Muntean; S Gallistl; B Leschnik; A Beitzke
Journal:  Eur J Pediatr       Date:  1995-04       Impact factor: 3.183
  2 in total

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