scholarly journals Staged Surgical Palliation for HLHS in a Girl with Severe Factor X Deficiency

2018 ◽  
Vol 07 (01) ◽  
pp. e12-e15 ◽  
Author(s):  
Ahmed Elmahrouk ◽  
Mohamed Ismail ◽  
Abdulbadee Bugis ◽  
Nashwa Badawy ◽  
Hesham Aboelghar ◽  
...  
Keyword(s):  
Team Approach ◽  
Factor X ◽  
Hematologic Disorder ◽  
Factor X Deficiency ◽  
Factor Deficiency ◽  
Perioperative Bleeding ◽  
Cavopulmonary Anastomosis ◽  
Surgical Palliation ◽  
Stage 1 ◽  
Left Heart Syndrome

Background Factor X deficiency (also known as Stuart–Prower factor deficiency) is an autosomal recessive extremely rare hereditary hematologic disorder, affecting around 1:1,000,000 of the general population. Case Presentation This case report describes a patient with hypoplastic left heart syndrome and severe factor X deficiency, who underwent staged surgical palliation. From stage 1 Norwood palliation, through superior cavopulmonary anastomosis and ending with total cavopulmonary connection with satisfactory hemostasis and no significant perioperative bleeding complication. Conclusion The need to maintain hemostasis while aiming to prevent intracardiac thrombosis requires multidisciplinary team approach including hematologist, cardiac surgeon, pediatric cardiac intensivist, and anesthesiologist along with meticulous hemostasis during surgery and careful monitoring of coagulation profile in the postoperative period.

Acquired Factor X (Stuart Factor) Deficiency in a Patient with Mycoplasma Pneumonial Infection

1979 ◽  
Author(s):  
F Peuscher ◽  
W van Aken ◽  
A Swaak ◽  
L Sie ◽  
L Statius van Eps
Keyword(s):  
Prothrombin Complex Concentrate ◽  
Influence Factor ◽  
Systemic Amyloidosis ◽  
Bleeding Tendency ◽  
Factor X ◽  
Factor X Deficiency ◽  
Factor Deficiency ◽  
Fresh Plasma ◽  
La Jolla ◽  
Affect Factor

An isolated deficiency of factor X is known to occur in a hereditary form, the Stuart-Prower disease, and in an acquired form in some patients with para-proteinaemia and sporadically in systemic amyloidosis. Transient deficiency of factor X in the presence of normal levels of factors II, VII and V appears to be rare. In the literature, only three cases have been described. We have studied a patient with a severe haemorrhagic diathesis and concomitant mycoplasma pneumonial infection. The bleeding tendency proved to be due to isolated factor X deficiency. No circulating inhibitors of factor X were present. Systemic amyloidosis could not be demonstrated. Factor X-related antigen could not be detected (this test was kindly performed by Dr.Daryl S.Fair, Scripps Clinic and Research Foundation, La Jolla, U.S.A.). Treatment with vitamin K, prothrombin complex concentrate, fresh plasma and whole blood proved not to influence factor X activity in the patient’s plasma. However, 20 days after admission⋅to hospital both factor X activity and antigen spontaneously returned to normal. These results suggest that the synthesis of factor X was transiently defective. Since other conditions known to affect factor X activity could not be demonstrated, it is postulated that the acquired factor X deficiency in this patient was related to the infection with mycoplasma pneumoniae.

Acquired Factor X (Stuart Factor) Deficiency in a Patient with Mycoplasma Pneumonial Infection

1979 ◽  
Author(s):  
F.W. Peuscher ◽  
W.G. van Aken ◽  
A.J.G. Swaak ◽  
L.H. Sie ◽  
L.W. Statius van Eps
Keyword(s):  
Prothrombin Complex Concentrate ◽  
Influence Factor ◽  
Systemic Amyloidosis ◽  
Bleeding Tendency ◽  
Factor X ◽  
Severe Haemorrhage ◽  
Factor X Deficiency ◽  
Factor Deficiency ◽  
La Jolla ◽  
Affect Factor

An isolated deficiency of factor X is known to occur in a hereditary form, the Stuart-Prower disease, and in an acquired form in some patients with para-proteinaemia and sporadically in systemic amyloidosis. Transient deficiency of factor X In the presence of normal levels of factors II, VII and V appears to be rare. In the literature, only three cases have been described. We have studied a patient with a severe haemorrhage diathesis and concomitant mycoplasma pneumonlal infection. The bleeding tendency proved to be due to isolated factor X deficiency. No circulating inhibitors of factor X were present. Systemic amyloidosis could not be demonstrated. Factor X-related antigen could not be detected (this test was kindly performed by Dr. Daryl S. Fair, Scripps Clinic and Research Foundation, La Jolla, U.S.A.). Treatment with vitamin K, prothrombin complex concentrate, fresi plasma and whole blood proved not to influence factor X activity in the patient’s Plasma. However, 20 days after admission·to hospital both factor X activity and antigen spontaneously returned to normal. These results suggest that the synthesis of factor X was transiently defective. Since other conditions known to affect factor X activity could not be demonstrated, it is postulated that the acquired factor X deficiency in this patient was related to the infection with mycoplasma pneumoniae.

scholarly journals Factor VII Deficiency in Systemic Primary Amyloidosis: A Rare Case

2018 ◽  
Vol 5 (4) ◽  
pp. 28
Author(s):  
Fadime Ersoy Dursun ◽  
Erdal Akyar ◽  
Gokhan Uygun ◽  
Zafer Baslar ◽  
Bengu Cobanoglu
Keyword(s):  
Prothrombin Time ◽  
Laboratory Tests ◽  
Rare Case ◽  
Common Factor ◽  
Factor Vii ◽  
Primary Amyloidosis ◽  
Factor X ◽  
Factor Vii Deficiency ◽  
Factor X Deficiency ◽  
Factor Deficiency

Introduction: Isolated and combined factor deficiencies are known to occur in systemic primary amyloidosis. The most common factor deficiency known in these cases is isolated factor X deficiency. Other factor deficiencies are relatively less frequent. Isolated factor VII deficiency occurs very rarely in cases of systemic primary amyloidosis.Case report: A 58-year-old male patient previously presenting to another health center with complaints of generalized edema, fatigue, and itching had proteinuria and then he was diagnosed with systemic primary amyloidosis after the renal biopsy for proteinuria etiology. The patient’s laboratory tests showed prolongation of prothrombin time and factor VII deficiency. The patient responded well to the treatment for primary amyloidosis and factor VII deficiency.Discussion: In cases of systemic primary amyloidosis, if the etiology of prolonged prothrombin time involves no liver disease, warfarin use, or malabsorption, physicians should always keep in mind rare factor deficiencies such as factor VII deficiency, along with common factor deficiencies.

Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome

2006 ◽  
Vol 16 (6) ◽  
pp. 556-562 ◽  
Author(s):  
Jeffrey Phillip Jacobs ◽  
James Anthony Quintessenza ◽  
Paul J. Chai ◽  
Harald L. Lindberg ◽  
Alfred Asante-Korang ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Low Flow ◽  
Left Heart ◽  
Donor Heart ◽  
Hypoplastic Left Heart ◽  
Panel Reactive Antibody ◽  
Cavopulmonary Anastomosis ◽  
Stage 1 ◽  
Left Heart Syndrome ◽  
Reactive Antibody

Objective:Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients.Methods:We transplanted 68 consecutive children, with diagnoses of hypoplastic left heart syndrome in 31, cardiomyopathy in 20, and post-operative complex congenital heart disease in 17. Of these patients, 9 (13.2%) were neonates, and 46 (67.6%) were infants. Median age was 118.5 days. Operative technique involves bicaval cannulation and anastamoses with continuous low flow bypass, and either short periods of circulatory arrest or continuous low flow antegrade cerebral perfusion for reconstruction of the aortic arch. Initial reperfusion of the donor heart utilizes glutamate and aspartate substrate enriched white blood cell filtered cardioplegia. Immunosuppressive therapy includes induction (pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin) and initial maintenance (calcineurin inhibitor, an anti-proliferative agent, and a weaning steroid protocol). Of the 31 patients with hypoplastic left heart syndrome, 23 underwent primary transplantation, and 8 underwent rescue transplantation from failing staged palliation in seven, or attempted biventricular repair in one. Of the seven patients who had failing staged palliation, three had undergone only the Norwood Stage 1 operation, 2 had undergone a Norwood Stage 1 operation and a Glenn superior cavopulmonary anastomosis and two had undergone a Norwood Stage 1 operation, a Glenn superior cavopulmonary anastomosis, and a completion Fontan operation.Results:The group undergoing primary transplantation was younger (p equals 0.007), weighed less (p equals 0.003), and waited longer for an appropriate donor heart (p equals 0.021) compared to those requiring rescue transplantation. No significant difference exists between the groups with regards to donor heart ischaemic time or post-transplant length of hospital stay. Thirty day survival (p equals 0.156) and overall survival (p equals 0.053) was better in those having primary transplantation, although these differences were not statistically significant when a p value of less than 0.05 is considered to be significant. In those having primary transplantation, no patients had elevated panel reactive antibody greater than 10%. Half of the 8 requiring rescue transplantation had panel reactive antibody greater than 10%, and this subgroup did especially poorly.Conclusion:Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.

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.

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