scholarly journals Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -

2021 ◽  
Vol 16 (1) ◽  
pp. 75-80
Author(s):  
Jeong Wook Park ◽  
Jinyoung Seo ◽  
Sang Hun Kim ◽  
Ki Tae Jung

Background: Hereditary angioedema (HAE) is a rare disease caused by the deficiency of C1 esterase inhibitor. HAE has a risk of life-threatening complications such as capillary leak syndrome (CLS) and disseminated intravascular coagulation (DIC).Case: A 42-year-old man with HAE presented for deceased-donor kidney transplantation. Prophylactic fresh frozen plasma (FFP) was given before surgery because of the risk of edema development. With careful management during anesthesia, there were no problems during surgery. However, generalized edema, hypotension, hypoalbuminemia, massive drainage of serosanguineous fluids from the intraabdominal space, and DIC occurred on the day after surgery. CLS was suspected and sustained hypotension with generalized edema became worse despite treatment with albumin, danazol, FFP, and vasoactive drugs. The patient’s condition worsened despite intensive care and he died due to shock.Conclusions: The anesthesiologist should prepare for the critical complications of HAE and prepare the appropriate treatment options.

PEDIATRICS ◽  
1986 ◽  
Vol 77 (5) ◽  
pp. 670-676
Author(s):  
Patrick Yuen ◽  
Alfred Cheung ◽  
Hsiang Ju Lin ◽  
Faith Ho ◽  
Jun Mimuro ◽  
...  

Severe and recurrent purpura fulminans developed in a Chinese boy at one day of age. Results of coagulation studies performed on the patient during attacks were compatible with the diagnosis of disseminated intravascular coagulation. Subsequent investigations have revealed that the patient is homozygous and that his parents are heterozygous for protein C deficiency. Cryoprecipitate and fresh frozen plasma induced a remission, and administration of warfarin has been successful in preventing recurrence of attacks for as long as 8 months without infusion of any plasma components. None of the family members who are heterozygous for protein C deficiency have had thrombotic episodes.


Author(s):  
Anne Craig ◽  
Anthea Hatfield

Part one of this chapter tells you about the physiology of blood and oxygen supply, about anaemia and tissue hypoxia, and the physiology of coagulation. Drugs that interfere with clotting are discussed. Bleeding, coagulation, and platelet disorders are covered as well as disseminated intravascular coagulation. Part two is concerned with bleeding in the recovery room: how to cope with rapid blood loss, managing ongoing blood loss, and how to use clotting profiles to guide treatment. There is also a section covering blood transfusion, blood groups and typing. Massive blood transfusion is clearly described, there are guidelines about when to use fresh frozen plasma, when to use platelets, and when to use cryoprecipitate. The final section of the chapter is about problems with blood transfusions.


2011 ◽  
Vol 25 (6) ◽  
pp. 379-382 ◽  
Author(s):  
Sandra C. Christiansen ◽  
Bruce L. Zuraw

Background Hereditary angioedema (HAE) patients suffering from laryngeal attacks in the United States faced severely limited treatment options until 2008. These potentially life-threatening episodes occur in over one-half of the patients affected by HAE during their lifetimes. Acute therapy had been relegated to supportive care, intubation, and consideration of fresh frozen plasma (FFP)–-the latter with the potential for actually accelerating the speed and severity of the swelling. Methods In this article we will review the recently approved and emerging HAE treatments that have evolved from the recognition that bradykinin generation is the fundamental abnormality leading to attacks of angioedema. Results Acute therapy for laryngeal attacks will be discussed including purified plasma–derived C1 inhibitor (C1INH), recombinant C1INH, an inhibitor of plasma kallikrein (ecallantide), and a B2 receptor antagonist (icatibant). Prophylactic care has also been transformed from a reliance on attenuated androgens with their attendant side effects to C1INH replacement. Conclusion The arrival of these novel therapies promises to transform the future management of HAE.


2017 ◽  
Vol 27 (5) ◽  
pp. 1004-1007
Author(s):  
Young Kwon Koh ◽  
Jae Hee Lee ◽  
Yeong Bong Park

AbstractDisseminated intravascular coagulation is a rare complication of Kawasaki disease and appears in <0.1% of Kawasaki disease patients. We report a case of refractory Kawasaki disease complicated with disseminated intravascular coagulation and giant coronary aneurysm. A 5-month-old boy presented with Kawasaki disease with coagulopathy. Although the coagulopathy improved after fresh-frozen plasma and antithrombin-III administration, the fever persisted despite two rounds of intravenous immunoglobulin, along with intravenous methylprednisolone pulse therapy and infliximab administration. Despite all efforts to treatment, the patient had giant coronary aneurysms and died suddenly.


2020 ◽  
Vol 92 (11) ◽  
pp. 51-56
Author(s):  
P. A. Vorobyev ◽  
A. P. Momot ◽  
L. S. Krasnova ◽  
A. P. Vorobiev ◽  
A. K. Talipov

Aim. Clinical characteristics of disseminated intravascular coagulation (DIC) in COVID-19 infection and assessment of the effectiveness of complex therapy for this syndrome at the stages of prevention and treatment of various complications. Materials and methods. The study of publications was carried out through search engines on the Internet using keywords. To diagnose the infection, the COVID-19 program was used on the MeDiCase platform, which is publicly available on www.medicase.pro, which suggests a diagnosis with a sensitivity of 89.47%. The study included 85 patients with acute COVID-19 with mild to moderate disease, aged 11 to 81 years. The presence of the pathogen was confirmed immunologically in 12% of patients; in other cases, the diagnosis was based on the results of an automated survey in the MeDiCase system. All patients, according to the MGNOT recommendations, were prescribed one of the oral direct anticoagulants - Eliquis at a dose of 5 mg 2 times a day, Ksarelto at a dose of 10 mg 2 times a day or Pradax at a dose of 110 mg 2 times a day for at least 2 weeks. All other drugs with antiviral, immunomodulatory effects, antibiotics were canceled. Results. The presence of DIC is substantiated by the morphological picture of changes in organs and tissues, clinical (hematoma-petechial type of bleeding in combination with thromboembolic syndrome and the presence of thrombovasculitis) and laboratory changes: an increase in the level of soluble fibrin-monomer complexes, D-dimer, hyperfibrinogenaemia, less often - thrombocytopenia, violation of fibrinolytic activity. The phenomenon of consumption of clotting factors and profuse bleeding are rare. Direct anticoagulants, fresh frozen plasma transfusions and plasmapheresis are used in the treatment of disseminated intravascular coagulation. The paper presents its own positive results of early prescription at the outpatient stage of direct oral anticoagulants in prophylactic doses (no case of disease progression), individual cases of the use of fresh frozen plasma and plasapheresis. Conclusion. DIC syndrome with the development of thrombovasculitis is the most important pathogenetic mechanism for the development of microthrombotic and hemorrhagic disorders in organs during infection with COVID-19, leading to dysfunction of the lungs, brain and other nerve tissues, kidneys, thromboembolic complications, etc. Many symptoms of the disease may be associated with a violation of the nervous regulation of the functions of organs and systems. Prevention of thrombovasculitis is effective already at the stage of the first manifestation of the disease with the outpatient use of direct anticoagulants (oral, low molecular weight heparins). In case of more severe manifestations (complications) of the disease, additional use of freshly frozen plasma and plasmapheresis is effective.


2011 ◽  
Vol 18 (4) ◽  
pp. 49-53
Author(s):  
Evgeniy Aleksandrovich Tseymakh ◽  
A A Men'shikov ◽  
A V Bondarenko ◽  
S Yu Kuznetsov ◽  
I N Gontarev ◽  
...  

Results of comparative study of cryoplasmic therapy applied at complex treatment of 168 patients with disseminated intravascular coagulation syndrome were presented. In 56 patients complex therapy included cryosupernatant plasma (CSNP) and 112 patients received fresh frozen plasma (FFP). Study of coagulation and fibrinolysis system showed that restoration of fibrinolysis activity, physiologic anticoagulants and normalization of plasma fibrinogen levels occurred sooner when CSNP was used. Application of CSNP promoted the relaxation of disease severity, prevention of thrombotic complications and decrease of lethality (by 14.2%) to a greater extent as compared to FFP use.


2018 ◽  
Vol 46 (1) ◽  
pp. 265-265
Author(s):  
Brandi McCall ◽  
Olakunle Idowu ◽  
Raymond Moreno ◽  
Kristen Price ◽  
Joseph Nates

2020 ◽  
Vol 9 (11) ◽  
pp. 3613
Author(s):  
Boyoung Park ◽  
Junghyun Yoon ◽  
Han Joon Kim ◽  
Yun Kyung Jung ◽  
Kyeong Geun Lee ◽  
...  

Background: This study analyzed the status and trends of transfusion and its associated factors among liver and kidney transplantation recipients. Methods: A total of 10,858 and 16,191 naïve liver or kidney transplantation recipients from 2008 to 2017 were identified through the National Health Insurance Service database. The prescription code for transfusion and the presence, number, and amount of each type of transfusion were noted. The odds ratios and 95% confidence intervals were determined to identify significant differences in transfusion and blood components by liver and kidney transplantation recipient characteristics. Results: In this study, 96.4% of liver recipients and 59.7% of kidney recipients received transfusions related to the transplantation operation, mostly platelet and fresh frozen plasma. Higher perioperative transfusion in women and declining transfusion rates from 2008 to 2017 were observed in both liver and kidney recipients. In liver recipients, the transfusion rate in those who received organs from deceased donors was much higher than that in those who received organs from living donors; however, the mortality rate according to transfusion was higher only in recipients of deceased donor organs. In kidney recipients, a higher mortality rate was observed in those receiving transfusion than that in patients without transfusion. Conclusions: In Korea, the transfusion rates in liver and kidney recipients were relatively higher than those in other countries. Sociodemographic factors, especially sex and year of transplantation, were associated with transfusion in solid organ recipients, possibly as surrogates for other causal clinical factors.


2009 ◽  
Vol 1 (1) ◽  
pp. 33
Author(s):  
Md Abul Kalam Azad ◽  
M Abdul Kader ◽  
M Abdul Jalil Chowdhury ◽  
Tofayel Ahmed

<p>In health there is a balance between the coagulation and anti-coagulation systems, but in disseminated intravascular coagulation (DIC) the coagulation mechanism is activated inappropriately and in a diffuse way. This may lead to thrombosis, but more often haemorrhage occurs when the clotting factors are exhausted. DIC may present as acute, subacute, and rarely chronic form. Here we present a case of chronic DIC following pelvic inflammatory disease (PID) as a consequence of repeated menstruation regulation (MR). We treated her with fresh frozen plasma, fresh blood, doxycycline with significant clinical improvement.</p><p>DOI: 10.3329/bsmmuj.v1i1.3696</p> <p><em>BSMMU J </em>2008; 1(1): 33-34</p>


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