scholarly journals Management of Neuraxial Analgesia in a Parturient with Factor XIII Deficiency: A Case Report and Proposed Management Algorithm

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
David B. Carroll ◽  
Conrad Myler ◽  
Natthapol Songdej ◽  
Khaled Sedeek ◽  
Dmitri Bezinover
Keyword(s):  
Case Report ◽  
Factor Xiii ◽  
Hemorrhagic Stroke ◽  
Factor Xiii Deficiency ◽  
Neuraxial Analgesia ◽  
Management Algorithm ◽  
Coagulation Defect ◽  
Active Labor ◽  
History Of ◽  
Fxiii Deficiency

Factor XIII (FXIII) deficiency is a rare coagulation defect that can be associated with significant bleeding. A 28-year-old pregnant woman, with a history of hemorrhagic stroke secondary to severe congenital FXIII deficiency, presented in active labor requesting an epidural. Factor XIII levels had been monitored throughout her pregnancy and treated with intermittent factor XIII infusions to maintain factor levels above 30% of normal. After careful multidisciplinary peripartum evaluation and FXIII replacement, neuraxial analgesia was performed without complication. Neuraxial analgesia can be performed without complication in patients with FXIII deficiency if FXIII levels are carefully managed and no other coagulopathy exists.

scholarly journals Factor XIII deficiency leading to preseptal haematoma post-strabismus surgery

2019 ◽  
Vol 12 (11) ◽  
pp. e231457
Author(s):  
Mayank Jain ◽  
Ramesh Kekunnaya ◽  
Akshay Badakere
Keyword(s):  
Factor Xiii ◽  
Fresh Frozen Plasma ◽  
Strabismus Surgery ◽  
Rare Form ◽  
Factor Xiii Deficiency ◽  
Fresh Frozen ◽  
History Of ◽  
Frozen Plasma ◽  
Fxiii Deficiency ◽  
Routine Coagulation

A young girl with constant exotropia was planned for surgery. Thorough preoperative workup was done and the patient underwent strabismus surgery. The girl developed preseptal haematoma on the third postoperative day with marked chemosis and oozing of blood from the conjunctival cul-de-sac. A history of factor XIII (FXIII) deficiency was later revealed by the caretakers. The patient was admitted and fresh frozen plasma was transfused for 5 days along with intravenous tranexamic acid. Orbital ultrasound and CT scan were done to confirm the location of the haematoma. The child improved significantly after 5 days and the proptosis subsided. FXIII deficiency is a rare form of bleeding disorder that is not revealed on routine coagulation profile tests. Fresh frozen plasma and recombinant FXIII are now available for treatment.

New Families with Hereditary Hemorrhagic Trait due to Deficiency of Fibrin Stabilizing Factor (F. XIII)

1968 ◽  
Vol 20 (03/04) ◽  
pp. 534-541 ◽  
Author(s):  
O Egeberg
Keyword(s):  
Factor Xiii ◽  
Family Members ◽  
Recessive Inheritance ◽  
Factor Xiii Deficiency ◽  
History Of ◽  
Congenital Factor

SummarySevere hemorrhagic disorder due to congenital factor XIII deficiency is described in two unrelated Norwegian girls.Plasma cephalin time was for both patients extraordinarily short during episodes of bleeding and hematomas. No such hyperactivity reaction was demonstrable in unaffected condition some months later.Estimations of blood factor XIII levels revealed a partial defect in the parents of both children, and also in some other family members, consistent with an autosomal incompletely recessive inheritance of the defect. Some of the presumptive heterozygotes had a history of light bleeding phenomenons; whether this was related to their partial lack of factor XIII is so far uncertain.

Factor XIII Deficiency and Intracranial Bleed: Surgical Management and Prophylaxis with Cryoprecipitate

2021 ◽  
Author(s):  
Sunil V. Furtado ◽  
Pranoy Hegde ◽  
Rasmi Palassery ◽  
B. P. Karunakara
Keyword(s):  
Factor Xiii ◽  
Perioperative Management ◽  
Severe Headache ◽  
Resonance Imaging ◽  
Factor Xiii Deficiency ◽  
Limb Weakness ◽  
High Propensity ◽  
Intracranial Bleed ◽  
The Brain ◽  
Fxiii Deficiency

AbstractFactor XIII (FXIII) deficiency is a rare bleeding disorder with affected patients having high propensity for intracranial hemorrhage. A 12-year-old girl presented with severe headache, limb weakness, and rapidly worsening sensorium over 4 days. Magnetic resonance imaging of the brain and computed tomography (CT) of the head showed intraparenchymal bleed. Patient had normal coagulation profile and abnormal FXIII level. The perioperative management included cryoprecipitate transfusion to bring the FXIII value to 74%. She underwent craniotomy and evacuation of the hematoma. Postoperatively, she received prophylaxis against rebleed with cryoprecipitate. In the absence of FXIII concentrate, correction of FXIII deficiency is possible with cryoprecipitate in emergent situations.

scholarly journals Use of Catridecacog in a patient with severe Factor XIII deficiency undergoing surgery

2019 ◽  
Vol 11 (1) ◽  
Author(s):  
Gianluca Sottilotta ◽  
Francesca Luise ◽  
Vincenzo Oriana ◽  
Angela Piromalli ◽  
Rosa Santacroce ◽  
...  
Keyword(s):  
Inguinal Hernia ◽  
Surgical Procedures ◽  
Factor Xiii ◽  
Bleeding Disorder ◽  
Factor Xiii Deficiency ◽  
Fxiii Deficiency ◽  
Congenital Factor

Despite many articles regarding the antihemorrhagic treatment and prophylaxis, there is a lack of experience about how to best conduct major surgical procedures in patients with congenital factor XIII (FXIII) deficiency. Here we report a case of surgery (right inguinal hernia, complicated by heaviness and pain) performed in a patient with FXIII deficiency, receiving recombinant FXIII prophylaxis (Catridecacog 35 UI/kg every 28±2 days). Our experience shows that Catridecacog can be used safely and effectively not only for continued prophylaxis but also in surgery and adds to the very limited body of evidence currently available on surgery in this bleeding disorder.

A Novel Nonsense Mutation of F13A1 Gene Causing Inherited Factor XIII Deficiency in a Chinese Han Family

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4661-4661
Author(s):  
Dawei Wang ◽  
Liang Tang ◽  
Wei Shi ◽  
Heng Mei ◽  
Rui Yang ◽  
...  
Keyword(s):  
Nonsense Mutation ◽  
Factor Xiii ◽  
Family Members ◽  
Coagulation Factor ◽  
Maternal Grandmother ◽  
Chinese Han ◽  
Factor Xiii Deficiency ◽  
Fxiii Activity ◽  
Fxiii Deficiency

Abstract Abstract 4661 Introduction: Coagulation factor XIII (FXIII) is a protransglutaminase that has a major role in the final stage of blood coagulation process by forming cross-links between γ-glutamyl and ε-lysine residues of fibrin chains. The plasma FXIII (pFXIII) circulates in plasma as a heterotetramer (FXIII-A2B2) consisting of two catalytic A subunits (FXIII-A2) and two carrier B subunits (FXIII-B2). Inherited FXIII deficiency is a rare autosomal recessive disease with lifelong bleeding. Most cases of FXIII deficiency are heterogeneous due to mutations in the F13A gene. Currently, more than 100 mutations have been reported. Aim: To identify the genetic defect of inherited coagulation factor XIII (FXIII) deficiency in a Chinese Han family. Methods and Results: A 13 year-old patient complained of poor wound healing after operation and had a history of an excessive bleeding from the umbilical cord stump after her birth. The routine laboratory tests are normal. Her bleeding time is more than 15 minutes and fibrin clot was solubilized very quickly in 5mol/L urea, and became insoluble when normal plasma was mixed with her plasma in vitro. Her plasma FXIII activity was zero with the amine incorporation assay and plasma FXIII antigen was also near zero by one-step sandwich ELISA method, the plasma FXIIIA antigen was zero using an indirect competitive ELISA assay. The plasma FXIIIA antigen, FXIII activity and antigen were assayed in all available family members. The testing results of patient’s grandfather and maternal grandmother were within normal range. But the other pedigree members’ results were lower in different level compare with normal ranges. All members of her family had normal coagulation test. All the exons of F13A gene as well as F13B gene and their flanking regions were amplified by PCR for direct sequencing to identify the mutations in the proband. Direct DNA sequencing of all purified amplification products from the patient’s F13A gene demonstrated a homozygous nonsense mutation in exon8 (C to A transversion at nucleotide 98531 which caused Cys327X). And the patient didn’t have the Val34Leu polymorphism. In the pedigree except of the proband, the Cys327X mutation was found in the heterozygous state in all investigated members except for her grandfather and maternal grandmother. A family study revealed that the mutation was inherited from both parents. The identified mutation was validated by PCR-RFLP technique in the family members and healthy people. Restriction enzyme analysis of amplified exon 8 DNA fragment confirmed that the patient was homozygous for this mutation. Then the quantitative RT-PCR method was used for studying the mRNA expression level of mutant FXIIIA. And the results indicated that F13A mRNA transcripts in heterozygous mutant were reduced by 25% when compared to transcripts in wild-type one, while the homozygous mutant level of F13A mRNA transcript was nearly zero relative to the normal transcript. Conclusion: We have identified a novel Cys327X nonsense mutation in human FXIIIA gene which we have not found in the FXIII database (www.f13-database.de) or in previous publications.And the identified nonsense mutation is causative for severe factor XIII deficiency with a bleeding disorder. Further, in vitro expression studies of the factor XIII mutation is required to confirm their pathological mechanism. Disclosures: No relevant conflicts of interest to declare.

Post-Cervical Decompression Parsonage-Turner Syndrome Represents a Subset of C5 Palsy: Six Cases and a Review of the Literature: Case Report

Neurosurgery ◽  
2010 ◽  
Vol 67 (6) ◽  
pp. E1831-E1844 ◽  
Author(s):  
Justin M Brown ◽  
Andrew Yee ◽  
Renee A Ivens ◽  
William Dribben ◽  
Susan E Mackinnon
Keyword(s):  
Case Report ◽  
Turner Syndrome ◽  
Decompression Surgery ◽  
Nerve Reconstruction ◽  
Neuralgic Amyotrophy ◽  
C5 Palsy ◽  
Management Algorithm ◽  
History Of ◽  
Motor Strength ◽  
Structured Approach

Abstract BACKGROUND: Approximately 5% of cervical decompression cases are complicated by postoperative weakness. Parsonage-Turner syndrome (PTS) or neuralgic amyotrophy is known to be precipitated by surgery and unrelated to technical or structural issues. Our practice has seen a number of cases of PTS after cervical decompression surgery. In this case report, we discuss a series of such patients, highlighting the commonalities with the more frequently diagnosed C5 palsy. We conclude with our management algorithm. CLINICAL PRESENTATION: Six patients with post-cervical decompression PTS were referred to our institution during a 32-month period. All patients were examined physically, radiographically, and electromyographically and were followed for up to 2 years or until symptoms resolved. Conservative management was the rule, and surgical intervention, including nerve releases and nerve reconstruction, was undertaken in select circumstances. In the majority of patients (4 of 6 patients), pain management and physical therapy alone were used and achieved eventual resolution of pain and recovery of motor strength. The other 2 patients required adjunctive surgical procedures to maximize their outcomes. CONCLUSION: PTS accounts for a subset of patients experiencing postoperative weakness after cervical decompression operations. Although it is at times difficult to arrive at this diagnosis, an understanding of the history of PTS, among other causes of postoperative weakness, allows a structured approach to these patients. An evidence-based approach to management helps provide the best outcome for a given patient.

Congenital Hemorrhagic Disorders in Jordan

1984 ◽  
Vol 51 (03) ◽  
pp. 331-333 ◽  
Author(s):  
Abdalla S Awidi
Keyword(s):  
Factor Xiii ◽  
Hemophilia A ◽  
Factor Xiii Deficiency ◽  
Factor Xi Deficiency ◽  
Glanzmann’S Thrombasthenia ◽  
Glanzmann's Thrombasthenia ◽  
Fatal Hemorrhage ◽  
Hemorrhagic Disorders ◽  
History Of ◽  
Storage Pool Disease

SummaryThe results of a three year prospective study of inherited bleeding syndromes in Jordan is presented. There were 112 patients from 64 families. Of these there were 42 patients with hemophilia A, 23 with Glanzmann’s thrombasthenia, 22 with von Willebrand’s disease, 11 with Christmas disease, 6 with hypofibrinogenemia, 3 with afibrinogenemia, 2 with factor XIII deficiency, 2 with storage pool disease and 1 with factor XI deficiency. The pattern of inherited bleeding syndromes in Jordan is different from that seen in Europe and U.S.A. in that Glanzmann’s thrombasthenia is very common. High proportion of hemophiliacs were severe. Arthropathy was common. A significant number of bleeders had fatal hemorrhage. In a high proportion of patients, no family history of bleeding was found.

Combined Heterozygous Factor XIII-Deficiency in a Family Case Report

2005 ◽  
pp. 380-382
Author(s):  
E. Rusicke ◽  
V. Ivaskevicius ◽  
D. Klarmann ◽  
C. Escuriola Ettingshausen ◽  
J. Oldenburg ◽  
...  
Keyword(s):  
Case Report ◽  
Factor Xiii ◽  
Factor Xiii Deficiency ◽  
Family Case
Sign in / Sign up

Export Citation Format

Share Document