scholarly journals Bleeding Post Constriction Ring Release Surgery Causing Lower Limb Ischemia in an Infant with Undetected Hemophilia B

2021 ◽  
Author(s):  
S. Raja Sabapathy ◽  
Monusha Mohan ◽  
G. Venkateswaran ◽  
Shashi Ranjani
Keyword(s):  
Postoperative Period ◽  
Previous History ◽  
Limb Ischemia ◽  
Clotting Factor ◽  
Recurrent Bleeding ◽  
Coagulation Disorder ◽  
Coagulation Disorders ◽  
Constriction Ring ◽  
Excess Morbidity ◽  
Constriction Ring Syndrome

AbstractBleeding in the immediate postoperative period causing compromised limb circulation is an alarming complication. It is known to occur in coagulation disorders like hemophilia. When such complications happen in a child with no previous history of bleeding problems, one has to have a low threshold for suspecting a coagulation disorder. Repeated diffuse bleeding in the whole of the surgical wound with no specific bleeders must raise the suspicion and appropriate laboratory tests must immediately be ordered. Bleeding in coagulation disorders can stop only with supplementation of the appropriate missing clotting factor. Early diagnosis is important to avoid excess morbidity. We are reporting a 6-month-old child who underwent surgery for constriction ring syndrome in the limbs with Z-plasty please replace with and developed impending limb ischemia due to bleeding in the immediate postoperative period. The article emphasizes the need to think of the possibility while encountering recurrent bleeding in the postoperative period.

Clinical Factors Associated with Progression to AIDS in the Italian Cohort of HIV-Positive Hemophiliacs

1994 ◽  
Vol 72 (01) ◽  
pp. 033-038 ◽  
Author(s):  
N Schinaia ◽  
A M G Ghirardini ◽  
M G Mazzucconi ◽  
G Tagariello ◽  
M Morfini ◽  
...  
Keyword(s):  
Cumulative Incidence ◽  
National Registry ◽  
Factor Ix ◽  
Clotting Factor ◽  
Coagulation Disorders ◽  
Factors Associated ◽  
Kaplan Meier ◽  
Significant Difference ◽  
Congenital Coagulation Disorders ◽  
Clotting Factor Concentrates

SummaryThis study updates estimates of the cumulative incidence of AIDS among Italian patients with congenital coagulation disorders (mostly hemophiliacs), and elucidates the role of age at seroconversion, type and amount of replacement therapy, and HBV co-infection in progression. Information was collected both retrospectively and prospectively on 767 HIV-1 positive patients enrolled in the on-going national registry of patients with congenital coagulation disorders. The seroconversion date was estimated as the median point of each patient’s seroconversion interval, under a Weibull distribution applied to the overall interval. The independence of factors associated to faster progression was assessed by multivariate analysis. The cumulative incidence of AIDS was estimated using the Kaplan-Meier survival analysis at 17.0% (95% Cl = 14.1-19.9%) over an 8-year period for Italian hemophiliacs. Patients with age greater than or equal to 35 years exhibited the highest cumulative incidence of AIDS over the same time period, 32.5% (95% Cl = 22.2-42.8%). Factor IX recipients (i.e. severe B hemophiliacs) had higher cumulative incidence of AIDS (23.3% vs 14.2%, p = 0.01) than factor VIII recipients (i.e. severe A hemophiliacs), as did severe A hemophiliacs on less-than-20,000 IU/yearly of plasma-derived clotting factor concentrates, as opposed to A hemophiliacs using an average of more than 20,000 IU (18.8% vs 10.9%, p = 0.02). No statistically significant difference in progression was observed between HBsAg-positive vs HBsAg-negative hemophiliacs (10.5% vs 16.4%, p = 0.10). Virological, immunological or both reasons can account for such findings, and should be investigated from the laboratory standpoint.

Congenital Constriction Ring Syndrome in Lower Limb-A Rare Entity

2012 ◽  
Vol 3 (5) ◽  
pp. 425-426
Author(s):  
Dr. Anoop Kumar Singh Dr. Anoop Kumar Singh ◽  
◽  
Dr. Anumita Sinha ◽  
Dr. Archika Dr. Archika
Keyword(s):  
Lower Limb ◽  
Rare Entity ◽  
Constriction Ring ◽  
Constriction Ring Syndrome

scholarly journals Preserved Renal Function in Kidney Transplantation over a Thrombosed Aortobifemoral Bypass Graft: The Role of Retrograde Flow and Early Thrombolysis

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Saúl Pampa-Saico ◽  
Sara Jiménez-Alvaro ◽  
Fernando Caravaca-Fontán ◽  
Ana Fernández-Rodríguez ◽  
Maite Rivera-Gorrín ◽  
...  
Keyword(s):  
Renal Function ◽  
Severe Renal Impairment ◽  
Imaging Techniques ◽  
Bypass Graft ◽  
Graft Loss ◽  
Previous History ◽  
Limb Ischemia ◽  
Acute Limb Ischemia ◽  
Retrograde Flow ◽  
Aortobifemoral Bypass

Aortobifemoral bypass (ABFB) thrombosis is not uncommon, and when the artery of a renal graft is implanted on a bypass the risk of graft loss is high. We report the case of a 48-year-old woman with a previous history of ABFB under antiplatelet therapy and a kidney allograft implanted on the vascular prosthesis, who presented with acute limb ischemia and severe renal impairment. Imaging techniques revealed a complete thrombosis of the proximal left arm of the ABFB. However, a faint retrograde flow over the graft was observed thanks to the recanalization of distal left bypass by collateral native arteries. This unusual situation not previously reported in a kidney transplant setting, together with an early diagnosis, allowed graft survival until an early local thrombolysis resolved the problem. Two years later, renal function remains normal.

Acquired Constriction Ring Syndrome

2003 ◽  
Vol 24 (8) ◽  
pp. 640-641 ◽  
Author(s):  
Andrew Michael Smith ◽  
William Peckett ◽  
Mark Davies
Keyword(s):  
Good Prognosis ◽  
Tissue Viability ◽  
Surgical Exploration ◽  
Constriction Ring ◽  
Orthopaedic Community ◽  
Constriction Ring Syndrome

A case of “acquired constriction ring” or “toe-tourniquet” syndrome is presented to increase awareness of the condition in the orthopaedic community. If treated promptly, this condition has a very good prognosis and, if the diagnosis is missed, may lead to amputation. We also discuss the potential causes and presentation of this condition, along with the need for urgent surgical exploration if there are doubts over tissue viability.

scholarly journals Haemophilia and Joint Disease: Pathophysiology, Evaluation, and Management

2011 ◽  
Vol 1 (1) ◽  
pp. 51-59 ◽  
Author(s):  
Karin Knobe ◽  
Erik Berntorp
Keyword(s):  
Joint Disease ◽  
Clotting Factor ◽  
Recurrent Bleeding ◽  
Effective Prevention ◽  
Haemophilic Arthropathy ◽  
Chronic Synovitis ◽  
Bleeding Episodes ◽  
End Stage ◽  
Clotting Factor Concentrates

In patients with haemophilia, regular replacement therapy with clotting factor concentrates (prophylaxis) is effective in preventing recurrent bleeding episodes into joints and muscles. However, despite this success, intra-articular and intramuscular bleeding is still a major clinical manifestation of the disease. Bleeding most commonly occurs in the knees, elbows, and ankles, and is often evident from early childhood. The pathogenesis of haemophilic arthropathy is multifactorial, with changes occurring in the synovium, bone, cartilage, and blood vessels. Recurrent joint bleeding causes synovial proliferation and inflammation (haemophilic synovitis) that contribute to end-stage degeneration (haemophilic arthropathy); with pain and limitation of motion severely affecting patients’ quality of life. If joint bleeding is not treated adequately, it tends to recur, resulting in a vicious cycle that must be broken to prevent the development of chronic synovitis and degenerative arthritis. Effective prevention and management of haemophilic arthropathy includes the use of early, aggressive prophylaxis with factor replacement therapies, as well as elective procedures, including restorative physical therapy, analgesia, aspiration, synovectomy, and orthopaedic surgery. Optimal treatment of patients with haemophilia requires a multidisciplinary team comprising a haematologist, physiotherapist, orthopaedic practitioner, rehabilitation physician, occupational therapist, psychologist, social workers, and nurses.

TRIANGULAR FLAPS: A MODIFIED TECHNIQUE FOR THE CORRECTION OF CONGENITAL CONSTRICTION RING SYNDROME

Hand Surgery ◽  
2011 ◽  
Vol 16 (03) ◽  
pp. 387-393 ◽  
Author(s):  
Puay-Ling Tan ◽  
Yuan-Cheng Chiang
Keyword(s):  
Wide Spectrum ◽  
Lower Limbs ◽  
Modified Technique ◽  
Constriction Ring ◽  
Contour Deformity ◽  
Aesthetic Results ◽  
Advancement Flaps ◽  
A New Technique ◽  
Constriction Ring Syndrome

Congenital constriction ring syndrome (CCRS) is a congenital anomaly with a wide spectrum of clinical presentation. The accepted method of correcting a circular constriction is to excise the deep part of the constriction, and break the line of the circular scar with multiple Z- or W-plasties. Specific correction of soft tissue is recommended concurrently, to better correct the "sandglass" deformity. Here we describe a new technique using triangular advancement flaps to correct the deformity in eight patients with constriction ring syndrome, involving either the upper or lower limbs. In this technique, triangular flaps can be introduced either proximal or distal to the ring, depending on the limb circumference, to correct the contour deformity. In all eight patients, a normal extremity contour was obtained, with complete elimination of the deformity caused by the constriction ring. A mean follow-up of 43 months revealed an aesthetically acceptable scar, with no case of recurrence. Triangular flaps designed either proximal or distal to the constriction ring is able to correct the contour deformity of the limbs with great versatility and acceptable aesthetic results.

scholarly journals Coagulation disorders during treatment with cefazolin and rifampicin: rare but dangerous

2021 ◽  
Vol 6 (5) ◽  
pp. 131-134
Author(s):  
Ines Kouki ◽  
Clémence Montagner ◽  
Wladimir Mauhin ◽  
Jonathan London ◽  
Thierry Lazard ◽  
...  
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Clotting Factor ◽  
Severe Bleeding ◽  
Coagulation Disorders ◽  
Severe Vitamin ◽  
Digestive Hemorrhage ◽  
Factor Deficiency

Abstract. We describe a 79-year-old man with spondylodiscitis and unknown pathogen, treated with cefazolin and rifampicin. He developed a massive digestive hemorrhage. Prothrombin time was prolonged with severe vitamin-K-dependent clotting-factor deficiency. Severe bleeding can occur during cefazolin and rifampicin use. This deficiency should be assessed before prescribing cefazolin–rifampicin and prothrombin time monitored.

scholarly journals High Mutational Heterogeneity, and New Mutations in the Human Coagulation Factor V Gene. Future Perspectives for Factor V Deficiency Using Recombinant and Advanced Therapies

2021 ◽  
Vol 22 (18) ◽  
pp. 9705
Author(s):  
Sara Bernal ◽  
Irene Pelaez ◽  
Laura Alias ◽  
Manel Baena ◽  
Juan A. De Pablo-Moreno ◽  
...  
Keyword(s):  
Anticoagulant Activity ◽  
Coagulation Factor ◽  
Coagulation Cascade ◽  
Clotting Factor ◽  
Coagulation Disorder ◽  
Factor V ◽  
Factor V Deficiency ◽  
Advanced Therapies ◽  
Factor V Gene ◽  
V Gene

Factor V is an essential clotting factor that plays a key role in the blood coagulation cascade on account of its procoagulant and anticoagulant activity. Eighty percent of circulating factor V is produced in the liver and the remaining 20% originates in the α-granules of platelets. In humans, the factor V gene is about 80 kb in size; it is located on chromosome 1q24.2, and its cDNA is 6914 bp in length. Furthermore, nearly 190 mutations have been reported in the gene. Factor V deficiency is an autosomal recessive coagulation disorder associated with mutations in the factor V gene. This hereditary coagulation disorder is clinically characterized by a heterogeneous spectrum of hemorrhagic manifestations ranging from mucosal or soft-tissue bleeds to potentially fatal hemorrhages. Current treatment of this condition consists in the administration of fresh frozen plasma and platelet concentrates. This article describes the cases of two patients with severe factor V deficiency, and of their parents. A high level of mutational heterogeneity of factor V gene was identified, nonsense mutations, frameshift mutations, missense changes, synonymous sequence variants and intronic changes. These findings prompted the identification of a new mutation in the human factor V gene, designated as Jaén-1, which is capable of altering the procoagulant function of factor V. In addition, an update is provided on the prospects for the treatment of factor V deficiency on the basis of yet-to-be-developed recombinant products or advanced gene and cell therapies that could potentially correct this hereditary disorder.

scholarly journals Effectiveness of Conservative Treatment without Early Colonoscopy in Patients with Colonic Diverticular Hemorrhage

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Hirosato Doi ◽  
Keita Sasajima ◽  
Masanori Takahashi ◽  
Taira Sato ◽  
Iichirou Ootsu ◽  
...  
Keyword(s):  
Conservative Treatment ◽  
Confidence Interval ◽  
Previous History ◽  
Vital Signs ◽  
Hazard Ratio ◽  
Recurrent Bleeding ◽  
Diverticular Hemorrhage ◽  
History Of ◽  
Enhanced Computed Tomography ◽  
Colonic Diverticular Hemorrhage

Aim. This study was aimed to clarify the effectiveness of conservative treatment without performing early colonoscopy and the indications for early colonoscopy in patients with colonic diverticular hemorrhage. Methods. This retrospective study included 142 participants who were urgently hospitalized due to bloody stools and were diagnosed with colonic diverticular hemorrhage between April 2012 and December 2016. At the time of hospital visit, only when both shock based on vital signs and intestinal extravasation on abdominal contrast-enhanced computed tomography were observed, early colonoscopy was performed within 24 hours after hospitalization. However, in other cases, patients were conservatively treated without undergoing early colonoscopy. In cases of initial treatment failure in patients with shock, interventional radiology (IVR) was performed without undergoing early colonoscopy. Results. Conservative treatment was performed in 137 (96.5%) patients, and spontaneous hemostasis was achieved in all patients. By contrast, urgent hemostasis was performed in five (3.5%) patients; three and two attained successful hemostasis via early colonoscopy and IVR, respectively. There were no significant differences between two groups in terms of early rebleeding (7.3% vs. 0%,P=0.690) and recurrent bleeding (22.7% vs. 20.0%, P=0.685). The factors associated with the cumulative recurrent bleeding rates were a previous history of colonic diverticular hemorrhage (hazard ratio 5.63, 95% confidence interval 2.68–12.0, P<0.0001) and oral administration of thienopyridine derivative (hazard ratio 3.05, 95% confidence interval 1.23–7.53, P=0.016). Conclusions. In this series, conservative treatment without early colonoscopy was successful in patients with colonic diverticular hemorrhage.

Developing Infrastructure For International Collaborative Research In Coagulation Disorders: Coagulation Disorders Research Consortium (CDRC) and Coagulation Disorders Data and Specimen Repository (Biorepository)

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4787-4787
Author(s):  
Candace Enockson ◽  
Yiyong Wu ◽  
Narine Hakobyan ◽  
Mindy L. Simpson ◽  
Leonard A. Valentino, MD
Keyword(s):  
Board Of Directors ◽  
Collaborative Research ◽  
Research Funding ◽  
Data Repository ◽  
Blood Coagulation Disorders ◽  
Coagulation Disorder ◽  
Coagulation Disorders ◽  
Advisory Committees ◽  
Tissue Samples ◽  
International Consortium

Research in coagulation disorders is limited by the small population of those affected; the broad, worldwide demographic; and varying access to and availability of care. Hence, there is need to collaborate internationally to achieve research goals for the coagulation disorder community. Here we present the creation of a novel international consortium and biorepository to address this need. The CDRC was established as an international consortium of researchers interested in studying blood coagulation disorders and in advancing knowledge in this area by fostering collaborative research studies focusing on, but not limited to, the diagnosis, evaluation, and treatment of hematological disorders such as hemophilia and thrombosis/thrombophilia. The lack of availability of high quality bio-specimens is often limiting for researchers; the specimen and data repository was therefore created to support such collaborative research. The biorepository will contain tissue samples including: blood [serum from clotted blood and plasma in EDTA, citrate, or citrate + corn trypsin inhibitor]; DNA [from the buffy coat of blood in EDTA]; and procedural/surgical tissue samples [synovial fluid and joint tissue]; as well as relevant medical information collected from consenting healthy (free of known diseases) subjects and from subjects with bleeding or clotting disorders to be archived for use in future studies. A web-based database has been developed to collect and warehouse the deidentified information regarding study subjects participating in the biorepository. Members of the consortium will have the option to submit data and specimens to the biorepository to benefit the research efforts of other members and/or to use the data and specimens from the biorepository to support their own research for studies approved by the member’s IRB or similar regulatory group and the Rush IRB. A primary benefit of participating in this consortium is the opportunity for collaboration with other investigators internationally on key questions that affect patients with bleeding and clotting disorders and access to samples from different racial and ethnic groups for comparative studies. Members are encouraged to develop collaborative studies and grant proposals, particularly in the area of translational research, which will foster the development of improved diagnostic modalities and more effective treatment options for those with coagulation disorders. Data regarding member participation in the consortium and review of sample acquisition will be presented. Disclosures: Valentino: Baxter BioScience: Consultancy, Membership on an entity’s Board of Directors or advisory committees, Research Funding; Bayer HealthCare: Consultancy, Membership on an entity’s Board of Directors or advisory committees, Research Funding; CSL Behring: Consultancy, Membership on an entity’s Board of Directors or advisory committees, Research Funding; GTC Biotherapeutics: Consultancy, Membership on an entity’s Board of Directors or advisory committees, Research Funding; Inspiration Bioscience: Consultancy, Membership on an entity’s Board of Directors or advisory committees, Research Funding; NovoNordisk: Consultancy, Membership on an entity’s Board of Directors or advisory committees, Research Funding; Pfizer: Consultancy, Membership on an entity’s Board of Directors or advisory committees, Research Funding.

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