FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4247332261> ?p ?o ?g. }

• W4247332261 endingPage "1149" @default.
• W4247332261 startingPage "1147" @default.
• W4247332261 abstract "Most coagulation disorders are detected preoperatively, provided that complete history of bleeding tendency as well as adequate preoperative blood testing are obtained [1-3]. However, standard preoperative blood tests only detect platelet and coagulation factor disorders and fail to detect rare disorders, such as factor XIII deficiency and fibrinolysis disorders, which may be responsible for severe hemorrhage. We report a case of severe bleeding after adenoidectomy in a child with a positive history of previous surgical bleeding in whom extensive preoperative blood testing was normal. This severe bleeding was due to an alpha2 plasmin inhibitor (alpha (2) PI) deficiency, a rare congenital disease, resulting in activation of fibrinolysis and requiring specific treatment with antifibrinolytic agents. Case Report A 6-yr-old boy was scheduled for adenoidectomy. During the preoperative visit, careful questioning revealed a history of severe bleeding after a previous adenoidectomy 4 yr earlier in another hospital. Coagulation tests performed preoperatively at that time were normal and remained normal when the child was readmitted the same day for posterior nasal packing. He was discharged the following day with a possible diagnosis of thrombopathie. There was no family history of bleeding, and the mother had undergone a cesarean section without any complication, but the child's parents were first cousins. The current physical examination revealed only a small hematoma at the site of blood puncture. Because of this history of previous postoperative hemorrhage, complete coagulation testing was performed preoperatively. All tests were normal (Table 1), and no further exploration was undertaken.Table 1: Coagulation Tests Before the Operation and After the Second Bleeding EpisodeThe child was admitted as an outpatient and underwent adenoidectomy under general anesthesia. Bleeding was observed soon after the end of surgery, and the child returned to the operating room in the evening for surgical hemostasis. No surgical cause was found. Bleeding reappeared the following morning, and the child was sent to the operating room for the third time. After the third operation, hemoglobin was 5.8 g/dL, and anemia was poorly clinically tolerated. One unit of packed red blood cells was transfused, and treatment with 1-deamino-8D-arginine vasopressin and epsilon amino caproic acid was initiated because a disorder of fibrinolysis was suspected, despite a normal euglobulin clot lysis time. This was confirmed the following day when an exploration of alpha2 PI using a chromogenic test (Biogenic, Molndal, Sweden) showed an alpha2 PI deficiency (alpha2 PI activity 24%; normal values >60%). Blood samples were obtained from both parents, and a moderate deficiency in alpha2 PI activity was detected in both parents (56% and 58% for the father and the mother, respectively). The child was then treated with epsilon amino caproic acid for 1 mo, and the bleeding did not reappear. The child was discharged home 10 days after surgery. Discussion This report describes severe bleeding after adenoidectomy in a six-year-old child due to an alpha2 plasmin inhibitor deficiency that results in activation of fibrinolysis. This case report is unusual, as extensive blood testing performed preoperatively failed to identify the cause of previous bleeding tendency. Indeed, standard coagulation tests are aimed at detecting platelet and coagulation factor disorders but are inappropriate in detecting rare disorders, such as factor XIII deficiency or fibrinolysis disorders as in our child. The only test commonly performed to explore fibrinolysis, euglobulin clot lysis time, was normal in our child, which suggests that this test is not sensitive enough to detect some fibrinolysis disorders. This test is only a very global exploration of fibrinolysis, and is shortened or normal in patients with alpha2 PI deficiency [4]. The failure to detect this fibrinolysis disorder preoperatively resulted in severe postoperative bleeding, prolonged hospitalization, multiple operations, and red blood cell transfusion. The first description of alpha2 PI deficiency in humans was described by Koie et al. [5] in 1978. Few other cases have been reported since that time [4,6-8]. alpha2 PI is a plasma proteinase inhibitor that acts as a primary and fast-acting inhibitor of plasmin [9]. In addition, alpha2 PI interferes with adsorption of plasminogen into fibrin, inhibiting the plasminogen activator-induced lysis of fibrin clot. As a result, alpha2 PI plays an important role in the regulation of fibrinolysis. In most published reports, the diagnosis of congenital alpha2 PI deficiency is made in adolescents or young adults [4,6,7,10] but not in children, except for one report of three young Japanese alpha2 PI-deficient sisters [8]. In these reports, the patients always described, retrospectively, a bleeding tendency. We describe the first case of postoperative diagnosis of an alpha2 PI deficiency in a young child, revealed by severe postoperative bleeding. The child's parents are consanguineous (first cousins), as are at least three families described in the literature [5,6,11]. The genetic transmission appears to be autosomal recessive [10,12-14]. Patients with a severe bleeding tendency have a very low plasma concentration of alpha2 PI or a very low alpha2 PI activity and are homozygous for the deficit (alpha2 PI activity <2%) [14]. Clinically, homozygous patients have had a severe hemorrhagic diathesis since early childhood, characterized by prolonged bleeding and ecchymoses after minor trauma and spontaneous joint hemorrhage mimicking hemophilia. Our case is different from reported cases because the alpha2 PI activity was 24% despite homozygous alpha2 PI deficiency (both parents are heterozygous), and the bleeding tendency was moderate. Conversely, heterozygous patients (alpha2 PI activity 40%-60%) are either asymptomatic, as are our patient's parents, or have a moderate bleeding tendency [4,7,11]. alpha (2) PI deficiency is due either to an alteration in protein synthesis [15-17] or to the synthesis of an abnormal alpha2 PI protein with a very low biological activity [12,18] caused by a mutation on the gene encoding this protein [19]. Hemorrhage definitively stopped after treatment with epsilon amino caproic acid. This antifibrinolytic agent inhibits plasminogen activators and has a direct antiplasmin activity that destroys excess plasmin. Thus, this treatment counterbalances the alpha (2) PI deficiency responsible for the excess plasmin. In conclusion, we report an alpha2 PI deficiency diagnosed after severe postoperative hemorrhage in a six-year-old child. This exceptional coagulation abnormality is associated with an increased bleeding tendency in homozygous patients. Standard coagulation tests are unable to detect fibrinolysis disorders, and fibrinolysis should be explored with specific methods when a bleeding tendency is detected and complete coagulation tests are normal. Preoperative diagnosis of alpha2 PI deficiency is important, as bleeding can be easily prevented by treatment with antifibrinolytic agents." @default.
• W4247332261 created "2022-05-12" @default.
• W4247332261 creator A5006481887 @default.
• W4247332261 creator A5021281126 @default.
• W4247332261 creator A5032287845 @default.
• W4247332261 creator A5044592922 @default.
• W4247332261 creator A5057915181 @default.
• W4247332261 date "1997-05-01" @default.
• W4247332261 modified "2023-10-17" @default.
• W4247332261 title "Severe Postadenoidectomy Bleeding Revealing Congenital alpha2 Antiplasmin Deficiency in a Child" @default.
• W4247332261 cites W1495536584 @default.
• W4247332261 cites W1497223052 @default.
• W4247332261 cites W1963526833 @default.
• W4247332261 cites W1967001444 @default.
• W4247332261 cites W1969390613 @default.
• W4247332261 cites W1988449687 @default.
• W4247332261 cites W2013826141 @default.
• W4247332261 cites W2019885356 @default.
• W4247332261 cites W2047235984 @default.
• W4247332261 cites W2063466417 @default.
• W4247332261 cites W2071379587 @default.
• W4247332261 cites W2071817156 @default.
• W4247332261 cites W2089200801 @default.
• W4247332261 cites W2090093793 @default.
• W4247332261 cites W3115253558 @default.
• W4247332261 cites W4237351485 @default.
• W4247332261 cites W4237592780 @default.
• W4247332261 doi "https://doi.org/10.1213/00000539-199705000-00038" @default.
• W4247332261 hasPublicationYear "1997" @default.
• W4247332261 type Work @default.
• W4247332261 citedByCount "5" @default.
• W4247332261 countsByYear W42473322612018 @default.
• W4247332261 countsByYear W42473322612019 @default.
• W4247332261 countsByYear W42473322612020 @default.
• W4247332261 crossrefType "journal-article" @default.
• W4247332261 hasAuthorship W4247332261A5006481887 @default.
• W4247332261 hasAuthorship W4247332261A5021281126 @default.
• W4247332261 hasAuthorship W4247332261A5032287845 @default.
• W4247332261 hasAuthorship W4247332261A5044592922 @default.
• W4247332261 hasAuthorship W4247332261A5057915181 @default.
• W4247332261 hasBestOaLocation W42473322611 @default.
• W4247332261 hasConcept C187212893 @default.
• W4247332261 hasConcept C71924100 @default.
• W4247332261 hasConceptScore W4247332261C187212893 @default.
• W4247332261 hasConceptScore W4247332261C71924100 @default.
• W4247332261 hasIssue "5" @default.
• W4247332261 hasLocation W42473322611 @default.
• W4247332261 hasLocation W42473322612 @default.
• W4247332261 hasOpenAccess W4247332261 @default.
• W4247332261 hasPrimaryLocation W42473322611 @default.
• W4247332261 hasRelatedWork W1506200166 @default.
• W4247332261 hasRelatedWork W2039318446 @default.
• W4247332261 hasRelatedWork W2080531066 @default.
• W4247332261 hasRelatedWork W2410491650 @default.
• W4247332261 hasRelatedWork W2604682584 @default.
• W4247332261 hasRelatedWork W2748952813 @default.
• W4247332261 hasRelatedWork W2899084033 @default.
• W4247332261 hasRelatedWork W3032375762 @default.
• W4247332261 hasRelatedWork W3108674512 @default.
• W4247332261 hasRelatedWork W4242858705 @default.
• W4247332261 hasVolume "84" @default.
• W4247332261 isParatext "false" @default.
• W4247332261 isRetracted "false" @default.
• W4247332261 workType "article" @default.