Hostname: page-component-78c5997874-94fs2 Total loading time: 0 Render date: 2024-11-14T11:13:42.019Z Has data issue: false hasContentIssue false
  • English
  • Français

Pulmonary arterial thrombosis in a neonate with homozygous deficiency of antithrombin III: successful outcome following pulmonary thrombectomy and infusions of antithrombin III concentrate

Published online by Cambridge University Press:  19 August 2008

K. Roman ,
E. Rosenthal  and
R. Razavi
K. Roman*
Affiliation:
Department of Paediatric Cardiology, Guys Hospital, Guys and St. Thomas NHS Trust, London, UK
E. Rosenthal
Affiliation:
Department of Paediatric Cardiology, Guys Hospital, Guys and St. Thomas NHS Trust, London, UK
R. Razavi
Affiliation:
Department of Paediatric Cardiology, Guys Hospital, Guys and St. Thomas NHS Trust, London, UK
*
Dr Kevin Roman, MRCP, Specialist Registrar, Dept of Paediatric Cardiology, Southampton General Hospital, Tremona Road, Southampton SO16 6YD, UK. Tel: 01703 777222; Fax: 01703 794526; E-mail: roman@roman99.freeserve.co.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

We report a newborn male who presented with severe central cyanosis on the third day of life. Partial thrombotic obstruction of the pulmonary trunk secondary to Antithrombin III (homozygous defect of heparin binding site) deficiency was subsequently diagnosed. Surgical thrombectomy, and infusions of Antithrombin III concentrate, led to a successful outcome. We postulate that intrauterine thrombosis occurred to give this unusual presentation.

Keywords

Antithrombin III deficiencyneonatal thrombosis
Type
Brief Reports
Information
Cardiology in the Young , Volume 10 , Issue 3 , May 2000 , pp. 275 - 278
Copyright
Copyright © Cambridge University Press 2000

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Lane, DA, Olds, RJ, Boisclair, M et al. . Antithrombin III mutation database: first update. Thromb Haemost, 1993; 70: 361–369.Google ScholarPubMed
2.Lane, DA, Olds, RJ, Conard, J et al. . Pleiotropic effects of antithrombin strand IC substitution mutations. J Clin Invest, 1992; 90: 2422–2433.CrossRefGoogle Scholar
3.Lane, DA, Auberger, K, Ireland, H, Roscher, AA, Thein, SL. Prenatal diagnosis in combined antithrombin and factor V gene mutation. Br J Haematol, 1996; 94: 753–755.CrossRefGoogle ScholarPubMed
4.Chowdhury, V, Lane, DA, Mille, B, Auberger, K, Gandenberger-Bachem, S, Pabinger, I, Olds, RJ, Thein, SL. Homozygous antithrombin deficiency: report of two new cases (99 Leu to Phe) associated with arterial and venous thrombosis. Thromb Haemost, 1994; 72: 198–202.Google ScholarPubMed
5.Olds, RJ, Lane, DA, Boisclair, M, Sas, G, Bock, S, Thein, SL. Antithrombin Budapest 3: an antithrombin variant with reduced heparin affinity resulting from the substitution. FEBBS Letts, 1992; 300: 241–246.CrossRefGoogle ScholarPubMed
6.Schmidt, BK, Muraji, T, Zipursky, A. Low antithrombin III in neonatal shock: DIC or non-specific protein depletion? Eur. J Paediatr, 1986; 145:500–503.CrossRefGoogle ScholarPubMed
7.Bjarke, B, Herin, P, Blomback, M. Neonatal aortic thrombosis. Acts Paediatr Scand. 1974; 63: 297–301.CrossRefGoogle ScholarPubMed
8.Sanchez, J, Velasco, F, Alvarez, R, Roman, J, Tortes, A. Aortic thrombosis in a neonate with hereditary antithrombin III deficiency: successful outcome with thrombolytic and replacement treatment. Acts Paediatr 1996: 85:245–247.CrossRefGoogle Scholar
9.Peeters, S, Vandenplas, Y, Jochmans, K, Bougatef, A, DeWaele, M, DeWolf, D. Myocardial infarction in a neonate with hereditary antithrombin III deficiency. Acta Paediatr. 1993: 82: 610–613.CrossRefGoogle Scholar