Successful Liver Transplantation for a Child With Life-Threatening Recurrent Bleeding Episodes Due to Congenital Factor X Deficiency: A Case Report

2012 ◽  
Vol 44 (2) ◽  
pp. 583-584 ◽  
Author(s):  
S.H. Bang ◽  
S.H. Oh ◽  
K.M. Kim ◽  
S.M. Song ◽  
J.J. Seo ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Case Report ◽  
Recurrent Bleeding ◽  
Factor X ◽  
Factor X Deficiency ◽  
Life Threatening ◽  
Bleeding Episodes ◽  
Congenital Factor

P11 Congenital factor X deficiency and pregnancy: a case report

2009 ◽  
Vol 123 ◽  
pp. S142
Author(s):  
A. Mamopoulos ◽  
S. Vakalopoulou ◽  
A. Fileli ◽  
C. Vosnakis ◽  
E. Lefkou ◽  
...  
Keyword(s):  
Case Report ◽  
Factor X ◽  
Factor X Deficiency ◽  
Congenital Factor

A Rare Case of Congenital Factor X Mild Deficiency Presenting as Life-Threatening Hemorrhagic Shock in a Neonate

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S151-S151
Author(s):  
Rachelle Mendoza ◽  
Tahmineh Haidary ◽  
Steven Kang
Keyword(s):  
Bleeding Event ◽  
Coagulation Factor ◽  
Hemodynamic Instability ◽  
Fresh Frozen Plasma ◽  
Bleeding Disorder ◽  
Activity Level ◽  
Factor X ◽  
Factor X Deficiency ◽  
Life Threatening ◽  
Congenital Factor

Abstract Introduction Congenital factor X deficiency is one of rarest bleeding disorders, occurring in 1 out of 1 million births. Its rarity limits its consideration in newborns presenting with hemodynamic instability. It is autosomal recessive and seen frequently in the consanguineous population. Patients with factor X deficiency are classified into three groups, based on factor X activity level: severe (<1%), moderate (1%-4%), and mild (6%-10%). Severe deficiency presents with bleeding diathesis early in life. Methods A 3-day-old male newborn, delivered at term via spontaneous vaginal delivery, presented in a well-baby clinic for a routine bilirubin check. Family history was negative for bleeding disorder or consanguinity. Nurse noted persistent blood oozing at heel stick site and oral, nasal, and umbilical stump bleeding. The patient immediately developed respiratory distress and shock. Sepsis, vitamin K deficiency, and congenital metabolic syndrome were considered. Liver function, WBC count, and other chemistry were normal, and cultures were negative. Hemoglobin was low and platelet count was elevated. PT (30.3 seconds) and aPTT (53.3 seconds) were both prolonged. Mixing patient’s sample with normal plasma corrected PT (13.1 seconds) and aPTT (25.7 seconds), indicating a factor deficiency. Results Coagulation factor assays revealed normal levels of factors VII, VIII, IX, and II. Factor X activity (12.9%) was low. The patient’s condition improved after multiple pRBC and plasma transfusions. He was placed on a daily 25-mL/kg dose of fresh-frozen plasma, which maintained his PT at 17.6 to 19.1 seconds and aPTT at 34.1 to 48.0 seconds. Factor X level increased to 20% after plasma transfusion. Conclusion Congenital bleeding disorder should be considered for neonates presenting with bleeding and shock. Factor X deficiency is suspected when both PT and aPTT are prolonged and corrected with mixing studies. Although factor levels of 10% to 40% are considered adequate for hemostasis, our patient with 12% factor X activity presented with a life-threatening bleeding event.

scholarly journals Pediatric split liver transplantation for congenital factor X deficiency: first 10-year follow-up of a case with portal vein stenting

2021 ◽  
Vol 35 (1) ◽  
pp. 66-70
Author(s):  
Jung-Man Namgoong ◽  
Shin Hwang ◽  
Dae-Yeon Kim ◽  
Tae-Yong Ha ◽  
Gi-Won Song ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Vein ◽  
Factor X ◽  
Split Liver Transplantation ◽  
Factor X Deficiency ◽  
Split Liver ◽  
Congenital Factor

scholarly journals A Case Report of Early Gastric Cancer in Association with Congenital Factor X Deficiency.

1997 ◽  
Vol 30 (11) ◽  
pp. 2191-2195 ◽  
Author(s):  
Takao Takahashi ◽  
Yoshitaka Yamamura ◽  
Hiroshi Matsuo ◽  
Yasuhiro Kodera ◽  
Tsuyoshi Kito ◽  
...  
Keyword(s):  
Gastric Cancer ◽  
Case Report ◽  
Early Gastric Cancer ◽  
Factor X ◽  
Factor X Deficiency ◽  
Congenital Factor

Immunological Studies on the Blood Coagulation Factor X and Its Warfarin-induced Precursor

1974 ◽  
Vol 31 (01) ◽  
pp. 040-051 ◽  
Author(s):  
Gustav Gaudernack ◽  
Åse Gladhaug Berre ◽  
Bjarne Østerud ◽  
Hans Prydz
Keyword(s):  
Coagulation Factor ◽  
Factor X ◽  
Intrinsic Pathway ◽  
Coagulation Activity ◽  
Factor X Deficiency ◽  
Warfarin Treatment ◽  
The Cross ◽  
Monospecific Antisera ◽  
Purified Antigen ◽  
Congenital Factor

SummaryMonospecific antisera against the human coagulation factor X have been raised in rabbits by injections of purified antigen. Such antiserum was used to study the cross-reacting material without factor X activity which is present in the blood of warfarin-treated patients and animals as well as to study the changes in factor X during coagulation. One patient with congenital factor X deficiency was also studied.A complete identity was found between factor X in Macaca mulatta and human blood. During warfarin treatment antigenically cross-reacting material appeared in plasma. This was not adsorbed on BaSO4, and inhibited the coagulation activity of normal factor X.Both this material, normal factor X and the cross-reacting material in plasma from a patient congenitally deficient in factor X gave rise to split products during coagulation by the intrinsic pathway, i. e. all of them served as substrates for the intrinsic activator of factor X.

scholarly journals Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency

Blood ◽  
2012 ◽  
Vol 119 (22) ◽  
pp. 5111-5117 ◽  
Author(s):  
Aida Inbal ◽  
Johannes Oldenburg ◽  
Manuel Carcao ◽  
Anders Rosholm ◽  
Ramin Tehranchi ◽  
...  
Keyword(s):  
Factor Xiii ◽  
Autosomal Recessive Disorder ◽  
Allergic Reactions ◽  
Open Label ◽  
Impaired Wound Healing ◽  
Bleeding Rate ◽  
Life Threatening ◽  
A Subunit ◽  
Bleeding Episodes ◽  
Congenital Factor

Congenital factor XIII (FXIII) deficiency is a rare, autosomal-recessive disorder, with most patients having an A-subunit (FXIII-A) deficiency. Patients experience life-threatening bleeds, impaired wound healing, and spontaneous abortions. In many countries, only plasma or cryoprecipitate treatments are available, but these carry a risk for allergic reactions and infection with blood-borne pathogens. The present study was a multinational, open-label, single-arm, phase 3 prophylaxis trial evaluating the efficacy and safety of a novel recombinant FXIII (rFXIII) in congenital FXIII-A subunit deficiency. Forty-one patients ≥ 6 years of age (mean, 26.4; range, 7-60) with congenital FXIII-A subunit deficiency were enrolled. Throughout the rFXIII prophylaxis, only 5 bleeding episodes (all trauma induced) in 4 patients were treated with FXIII-containing products. The crude mean bleeding rate was significantly lower than the historic bleeding rate (0.138 vs 2.91 bleeds/patient/year, respectively) for on-demand treatment. Transient, non-neutralizing, low-titer anti-rFXIII Abs developed in 4 patients, none of whom experienced allergic reactions, any bleeds requiring treatment, or changes in FXIII pharmacokinetics during the trial or follow-up. These non-neutralizing Abs declined below detection limits in all 4 patients despite further exposure to rFXIII or other FXIII-containing products. We conclude that rFXIII is safe and effective in preventing bleeding episodes in patients with congenital FXIII-A subunit deficiency. This study is registered at http://www..clinicaltrials.gov as number NCT00713648.

scholarly journals Congenital factor X deficiency in Japan.

1981 ◽  
Vol 133 (1) ◽  
pp. 1-19 ◽  
Author(s):  
KAZUO MORI ◽  
HIDEAKI SAKAI ◽  
NOBORU NAKANO ◽  
SOZO SUZUKI ◽  
KOJI SUGAI ◽  
...  
Keyword(s):  
Factor X ◽  
Factor X Deficiency ◽  
Congenital Factor
Sign in / Sign up

Export Citation Format

Share Document