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Literature DB >> 22942571

Factor XIII Deficiency in Siblings: Importance of Prophylactic Replacement.

Kiran Kini¹, Deepti Chopra, Pushpa G Kini.

Abstract

Factor XIII deficiency, an autosomal recessive trait, can result in serious bleeding manifestation. This case report presents two brothers with Factor XIII deficiency. Though the younger sibling had been screened and diagnosed prophylactic replacement therapy had not been initiated unlike the elder brother. He presented with intracranial haemorrhage needing surgical evacuation while the elder brother remained symptom free on regular prophylactic replacement of FFP.

Entities: Disease

Year: 2011 PMID： 22942571 PMCID： PMC3155714 DOI： 10.1007/s12288-011-0083-1

Source DB: PubMed Journal: Indian J Hematol Blood Transfus ISSN： 0971-4502 Impact factor: 0.900

9 in total

1. A hitherto undescribed congenital haemorrhagic diathesis probably due to fibrin stabilizing factor deficiency.

Authors: F DUCKERT; E JUNG; D H SHMERLING
Journal: Thromb Diath Haemorrh Date: 1960-12-15

2. Delayed umbilical bleeding--a presenting feature for factor XIII deficiency: clinical features, genetics, and management.

Authors: Rashida Anwar; Adrian Minford; Louise Gallivan; Chi H Trinh; Alexander F Markham
Journal: Pediatrics Date: 2002-02 Impact factor: 7.124

3. Human Factor XIII from plasma and platelets. Molecular weights, subunit structures, proteolytic activation, and cross-linking of fibrinogen and fibrin.

Authors: M L Schwartz; S V Pizzo; R L Hill; P A McKee
Journal: J Biol Chem Date: 1973-02-25 Impact factor: 5.157

1 in total

1. Factor XIII deficiency presenting with intracerebral bleed.

Authors: Kamal Kumar Sawlani; Shyam Chand Chaudhary; Amitava Roy; Anil Kumar Tripathi
Journal: BMJ Case Rep Date: 2013-01-10

1 in total

Factor XIII Deficiency in Siblings: Importance of Prophylactic Replacement.

1. A hitherto undescribed congenital haemorrhagic diathesis probably due to fibrin stabilizing factor deficiency.

2. Delayed umbilical bleeding--a presenting feature for factor XIII deficiency: clinical features, genetics, and management.

3. Human Factor XIII from plasma and platelets. Molecular weights, subunit structures, proteolytic activation, and cross-linking of fibrinogen and fibrin.

4. Amino acid sequence studies on factor XIII and the peptide released during its activation by thrombin.

5. Prenatal diagnosis in factor XIII-A deficiency.

6. Bleeding disorder with abnormal wound healing, acid-soluble clots and normal factor XIII.

7. Factor 13 deficiency with severe hemorrhagic diathesis.

Review 8. Prophylaxis in rare coagulation disorders -- factor XIII deficiency.

9. International registry on factor XIII deficiency: a basis formed mostly on European data.

1. Factor XIII deficiency presenting with intracerebral bleed.