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[PMID]:29235093
[Au] Autor:Di Minno MND; Napolitano M; Dolce A; Mariani G; STER Study Group
[Ad] Endereço:Department of Advanced Biomedical Sciences, Federico II University, Naples, Italy.
[Ti] Título:Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence from the STER registry.
[So] Source:Br J Haematol;180(4):563-570, 2018 02.
[Is] ISSN:1365-2141
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Perioperative bleeding is a major concern in patients with factor VII (FVII) deficiency. Evaluating data of 95 FVII-deficient patients undergoing 110 surgical procedures (61 major, 49 minor), we assessed the impact of type of surgery, bleeding phenotype and FVII coagulant activity (FVII:C) levels on perioperative replacement therapy (RT). Compared to those with higher FVII:C levels, patients with <3% FVII:C received a higher number of RT doses (8 vs. 2, P = 0·003) for a longer RT duration (3 days vs. 1 day, P = 0·001), with no difference in RT dose. Similarly, patients with a history of major bleeds received a higher number of RT doses (8·5 vs. 2-3, P = 0·013) for a longer RT duration (2 days vs. 1 day, P = 0·005) as compared to those with a history of minor bleeds or to asymptomatic patients. No difference in RT was found among major and minor surgical procedures. Overall, multivariate analysis showed that history of major bleeding was the only independent predictor of number of RT doses (ß = 0·352, P = 0·001) and RT duration (ß = 0·405, P = 0·018). Overall, a ≈20 µg/kg perioperative RT was efficacious in 95·5% of cases. The infusion should be repeated ≈8 times in high-risk subsets (i.e. patients with a history of major bleeding).
[Mh] Termos MeSH primário: Deficiência do Fator VII/diagnóstico
Deficiência do Fator VII/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Tomada de Decisão Clínica
Terapia Combinada
Gerenciamento Clínico
Fator VII/administração & dosagem
Deficiência do Fator VII/epidemiologia
Feminino
Hemorragia/etiologia
Hemorragia/cirurgia
Seres Humanos
Masculino
Meia-Idade
Sistema de Registros
Procedimentos Cirúrgicos Operatórios/métodos
Avaliação de Sintomas
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
9001-25-6 (Factor VII)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180221
[Lr] Data última revisão:
180221
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171214
[St] Status:MEDLINE
[do] DOI:10.1111/bjh.15055


  2 / 925 MEDLINE  
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[PMID]:29019885
[Au] Autor:He JP; Feng JX
[Ad] Endereço:Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
[Ti] Título:Two-incision laparoscopic appendectomy for a severe hemophilia A child patient with coagulation factor VII deficiency: Case report and review of literature.
[So] Source:Medicine (Baltimore);96(41):e8197, 2017 Oct.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: The main complication of patients with severe hemophilia is recurrent bleeding events that usually affected musculoskeletal contractures. And replacement therapy methods were continuously improved to minimize adverse impacts brought by those complications. However, only several cases reported about the appendectomy for hemophilia A. We report a case of acute appendicitis treated by two-incision laparoscopy in a boy with hemophilia A and coagulation factor VII deficiency for the first time. PATIENT CONCERNS: An 8y7m-old Chinese boy presented with half a day of right sided abdominal pain, fever, nausea, and vomiting. DIAGNOSES: He received a computed tomography (CT) scan which revealed an enlarged appendix, thickened wall and appendiceal fecalith, and had received a conservative anti-bacterial treatment for his acute appendicitis but failed. He was diagnosed with hemophilia A and coagulation factor VII deficiency. INTERVENTIONS: Two-incision laparoscopic appendectomy was made in success with a careful management of perioperative period. We monitored the clotting factor FVIII level and gave him a replacement therapy. OUTCOMES: The patient had an uneventful recovery. LESSONS: It is important to exclude intraabdominal or retroperitoneal hemorrhage in patients suffering from hemophilia and acute abdominal pain. Pre-operative evaluation of validity of the FVIII replacement therapy is another effective strategy to assess the safety and feasibility of applying an operation procedure. The two-incision laparoscopic appendectomy is an effective treatment for this kind of patients for its minimal trauma and fast recovery characteristics. Our report shows that laparoscopic appendectomy is feasible in a child suffering from hemophilia after adequate blood clotting factor replacement treatment.
[Mh] Termos MeSH primário: Apendicectomia/métodos
Apendicite
Deficiência do Fator VII/complicações
Hemofilia A
Hemorragia/diagnóstico
[Mh] Termos MeSH secundário: Apendicite/complicações
Apendicite/diagnóstico
Apendicite/cirurgia
Criança
Diagnóstico Diferencial
Hemofilia A/complicações
Hemofilia A/diagnóstico
Hemofilia A/fisiopatologia
Seres Humanos
Laparoscopia/métodos
Masculino
Assistência Perioperatória/métodos
Índice de Gravidade de Doença
Tomografia Computadorizada por Raios X/métodos
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171122
[Lr] Data última revisão:
171122
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171012
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008197


  3 / 925 MEDLINE  
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[PMID]:28672139
[Au] Autor:Passarelli PC; Pasquantonio G; D'Addona A
[Ad] Endereço:Resident in Oral Surgery, Catholic University of Sacred Heart of Rome, Rome, Italy. Electronic address: piercarminepassarelli@hotmail.it.
[Ti] Título:Management of Surgical Third Lower Molar Extraction and Postoperative Progress in Patients With Factor VII Deficiency: A Clinical Protocol and Focus on This Rare Pathologic Entity.
[So] Source:J Oral Maxillofac Surg;75(10):2070.e1-2070.e4, 2017 Oct.
[Is] ISSN:1531-5053
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: The purpose of the present study was to analyze the management of surgical third molar extraction and postoperative progress in patients with a diagnosis of factor VII deficiency. Close collaboration between the oral-maxillofacial surgeon and hematologist will allow the team to categorize the risk and operate safely, thereby minimizing the incidence and severity of intraoperative and postoperative complications. MATERIALS AND METHODS: The present retrospective study included 7 patients with factor VII deficiency who had undergone third lower molar surgery. Their factor VII deficiency ranged from 10.5 to 21.0%. Recombinant activated factor VII (rFVIIa) (coagulation factor VIIa [recombinant]; NovoSeven RT; Novo Nordisk, Bagsvaerd, Denmark) was transfused intravenously in a single dose of 25 µg/kg body weight, 30 minutes before surgical extractions. After the surgery, betamethasone, an analgesic, and an ice pack were administered. RESULTS: Pretreatment with recombinant activated factor VII resulted in excellent hemostasis. No hemorrhagic complications and no postoperative major bleeding were observed. CONCLUSIONS: The extraction of the third lower molar appears to be a safe procedure for patients with factor VII deficiency when appropriate prophylaxis with rFVIIa is used.
[Mh] Termos MeSH primário: Perda Sanguínea Cirúrgica/prevenção & controle
Deficiência do Fator VII/complicações
Fator VIIa/uso terapêutico
Dente Serotino/cirurgia
Hemorragia Pós-Operatória/etiologia
Hemorragia Pós-Operatória/prevenção & controle
Extração Dentária
[Mh] Termos MeSH secundário: Adulto
Protocolos Clínicos
Feminino
Seres Humanos
Masculino
Proteínas Recombinantes/uso terapêutico
Estudos Retrospectivos
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Recombinant Proteins); AC71R787OV (recombinant FVIIa); EC 3.4.21.21 (Factor VIIa)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171012
[Lr] Data última revisão:
171012
[Sb] Subgrupo de revista:AIM; D; IM
[Da] Data de entrada para processamento:170704
[St] Status:MEDLINE


  4 / 925 MEDLINE  
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[PMID]:28176610
[Au] Autor:Girolami A; Cosi E; Ferrari S; Girolami B; Lombardi AM
[Ad] Endereço:a Department of Medicine , University of Padua Medical School , Padua , Italy.
[Ti] Título:Bleeding manifestations in heterozygotes with congenital FVII deficiency: a comparison with unaffected family members during a long observation period.
[So] Source:Hematology;22(6):375-379, 2017 Jul.
[Is] ISSN:1607-8454
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:OBJECTIVES: To determine whether heterozygotes with FVII deficiency have a bleeding tendency or not. PATIENTS AND METHODS: Eighty-four patients (OK) heterozygous for FVII deficiency, at the onset of the study, were paired with unaffected family members and followed for a long period of time (mean 22.6 years) for the occurrence of bleeding. Diagnosis of heterozygosis had to be based on family studies, clotting, immunological assays and genetic analysis. RESULTS: The mean FVII activity level was 0.51 IU/dl (range 35-65) and 94 IU/dl (range 88-118) in the heterozygotes and in the normal counterparts, respectively. Documented bleeding manifestations occurred in eight heterozygotes and in seven normal subjects. Statistical analysis of the difference was not significant. Bleeding manifestations were easy bruising, bleeding after tooth extractions, menorrhagia, epistaxis with no difference among the two groups. There was no strict correlation between bleeding and FVII activity levels. CONCLUSIONS: The study indicates that heterozygotes for FVII deficiency show rare bleeding manifestations which are also present in the unaffected family members with normal FVII levels. This indicates that Factor VII activity levels played no role in the occurrence of the bleeding symptoms. Furthermore, FVII levels of around 0.40 IU/dl are capable of assuring a normal hemostasis.
[Mh] Termos MeSH primário: Deficiência do Fator VII/complicações
Deficiência do Fator VII/genética
Fator VII/genética
Hemorragia/diagnóstico
Hemorragia/etiologia
Heterozigoto
Mutação
[Mh] Termos MeSH secundário: Adolescente
Adulto
Testes de Coagulação Sanguínea
Ativação Enzimática
Deficiência do Fator VII/terapia
Família
Feminino
Seres Humanos
Masculino
Fenótipo
Valores de Referência
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
9001-25-6 (Factor VII)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170707
[Lr] Data última revisão:
170707
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170209
[St] Status:MEDLINE
[do] DOI:10.1080/10245332.2017.1286540


  5 / 925 MEDLINE  
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[PMID]:28111927
[Au] Autor:Agostini-Vulaj D; Francis CW; Refaai MA
[Ad] Endereço:Departments of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY, USA.
[Ti] Título:Management of concomitant factor VII deficiency and Factor V Leiden mutation.
[So] Source:Int J Lab Hematol;39(1):e10-e13, 2017 Feb.
[Is] ISSN:1751-553X
[Cp] País de publicação:England
[La] Idioma:eng
[Mh] Termos MeSH primário: Deficiência do Fator VII/terapia
Fator V/genética
Mutação
[Mh] Termos MeSH secundário: Deficiência do Fator VII/sangue
Deficiência do Fator VII/genética
Seres Humanos
Masculino
Meia-Idade
[Pt] Tipo de publicação:CASE REPORTS; LETTER
[Nm] Nome de substância:
0 (factor V Leiden); 9001-24-5 (Factor V)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170124
[St] Status:MEDLINE
[do] DOI:10.1111/ijlh.12572


  6 / 925 MEDLINE  
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[PMID]:27046037
[Au] Autor:Jenkins TL; Zheng CX; Murchison AP; Bilyk JR
[Ad] Endereço:Wills Eye Hospital, Philadelphia, Pennsylvania, U.S.A.
[Ti] Título:Orbital Compartment Syndrome Following Post-Traumatic Subgaleal Hematoma.
[So] Source:Ophthal Plast Reconstr Surg;33(2):e33-e36, 2017 Mar/Apr.
[Is] ISSN:1537-2677
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Orbital extension of subgaleal hematoma is rare. This report describes the case of an otherwise healthy 10-year-old girl who developed delayed contralateral proptosis and external ophthalmoplegia after relatively minor right-sided forehead trauma. She was found to have bilateral subgaleal hematomas communicating with a left superior subperiostial orbital hematoma. Over the course of 2 days, she developed an orbital compartment syndrome requiring emergent canthotomy and cantholysis, followed by surgical incision and drainage of her scalp hematoma without orbitotomy. Hematologic work-up revealed heterozygous factor VII deficiency.
[Mh] Termos MeSH primário: Síndromes Compartimentais/etiologia
Traumatismos Craniocerebrais/complicações
Hematoma/etiologia
Doenças Orbitárias/diagnóstico
[Mh] Termos MeSH secundário: Criança
Deficiência do Fator VII/complicações
Feminino
Seres Humanos
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170317
[Lr] Data última revisão:
170317
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160406
[St] Status:MEDLINE
[do] DOI:10.1097/IOP.0000000000000684


  7 / 925 MEDLINE  
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[PMID]:26919454
[Au] Autor:Deshpande R; Ghosh K; Shetty S
[Ad] Endereço:Department of Haemostasis and Thrombosis, National Institute of Immunohaematology (ICMR), Mumbai, India.
[Ti] Título:Synergistic effect of factor VII gene polymorphisms causing mild factor VII deficiency in a case of severe factor X deficiency.
[So] Source:Blood Coagul Fibrinolysis;28(1):105-106, 2017 Jan.
[Is] ISSN:1473-5733
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Congenital combined deficiency of coagulation factors VII and X are mainly attributed to large deletions involving both the genes in chromosome 13 or occasionally due to the coincidental occurrence of independently occurring mutations. We report the molecular basis of congenital combined deficiency of factors VII and X in a 6-year-old female child. Direct DNA sequencing of both factor VII (F7) and factor X (F10) genes showed a novel homozygous missense mutation p.Cys90Tyr (c.307G>A) in exon 4 of F10. No mutations were detected in F7; however, the patient was homozygous for three polymorphic alleles known to be associated with reduced factor VII levels. The present case illustrates the synergistic effect of multiple polymorphisms resulting in phenotypic factor VII deficiency in the absence of a pathogenic mutation.
[Mh] Termos MeSH primário: Deficiência do Fator VII/genética
Fator VII/genética
Deficiência do Fator X/genética
[Mh] Termos MeSH secundário: Criança
Feminino
Seres Humanos
Mutação
Polimorfismo Genético
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
9001-25-6 (Factor VII)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160227
[St] Status:MEDLINE
[do] DOI:10.1097/MBC.0000000000000544


  8 / 925 MEDLINE  
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[PMID]:27586406
[Au] Autor:Woehrle D; Martinez M; Bolliger D
[Ad] Endereço:Abteilung für Anästhesie, Hirslanden Klinik Birshof, Münchenstein, Basel, Schweiz.
[Ti] Título:[Hereditary heterozygous factor VII deficiency in patients undergoing surgery : Clinical relevance].
[Ti] Título:Hereditärer heterozygoter Faktor VII-Mangel beim chirurgischen Patienten : Klinische Relevanz..
[So] Source:Anaesthesist;65(10):746-754, 2016 Oct.
[Is] ISSN:1432-055X
[Cp] País de publicação:Germany
[La] Idioma:ger
[Ab] Resumo:BACKGROUND: A hereditary deficiency in coagulation factor VII (FVII) may affect the international normalized ratio (INR) value. However, FVII deficiency is occasionally associated with a tendency to bleed spontaneously. We hypothesized that perioperative substitution with coagulation factor concentrates might not be indicated in most patients. METHODS: In this retrospective data analysis, we included all patients with hereditary heterozygous FVII deficiency who underwent surgical procedures at the University Hospital Basel between December 2010 and November 2015. In addition, by searching the literature, we identified publications reporting patients with FVII deficiency undergoing surgical procedures without perioperative substitution. RESULTS: We identified 22 patients undergoing 46 surgical procedures, resulting in a prevalence of 1:1500-2000. Coagulation factor concentrates were administered during the perioperative period in 15 procedures (33 %), whereas in the other 31 procedures (66 %), FVII deficiency was not substituted. No postoperative bleeding or thromboembolic events were reported. In addition, we found no differences in pre- and postoperative hemoglobin and coagulation parameters, with the exception of an improved postoperative INR value in the substituted group. In the literature review, we identified five publications, including 125 patients with FVII deficiency, undergoing 213 surgical procedures with no perioperative substitution. DISCUSSION: Preoperative substitution using coagulation factor concentrates does not seem to be mandatory in patients with an FVII level ≥15 %. For decision-making on preoperative substitution, patient history of an increased tendency to bleed may be more important than the FVII level or increased INR value.
[Mh] Termos MeSH primário: Deficiência do Fator VII/complicações
[Mh] Termos MeSH secundário: Adulto
Idoso
Fator VII/uso terapêutico
Deficiência do Fator VII/epidemiologia
Deficiência do Fator VII/genética
Feminino
Heterozigoto
Seres Humanos
Coeficiente Internacional Normatizado
Masculino
Meia-Idade
Assistência Perioperatória
Complicações Pós-Operatórias/sangue
Complicações Pós-Operatórias/prevenção & controle
Hemorragia Pós-Operatória/sangue
Hemorragia Pós-Operatória/prevenção & controle
Prevalência
Estudos Retrospectivos
Tromboembolia/sangue
Tromboembolia/prevenção & controle
Vitamina K/uso terapêutico
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
12001-79-5 (Vitamin K); 9001-25-6 (Factor VII)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170916
[Lr] Data última revisão:
170916
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160903
[St] Status:MEDLINE


  9 / 925 MEDLINE  
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[PMID]:27501477
[Au] Autor:See WS; Chang KO; Cheuk DK; Leung YY; Chan GC; Chan SC; Ha SY
[Ad] Endereço:Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, Special Administrative Region, China.
[Ti] Título:Inhibitor development after liver transplantation in congenital factor VII deficiency.
[So] Source:Haemophilia;22(5):e417-22, 2016 Sep.
[Is] ISSN:1365-2516
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Congenital factor VII (FVII) deficiency is the commonest type of the rare bleeding disorders. Very few cases of congenital FVII deficiency developed inhibitor and liver transplant is considered as definitive treatment. In the literature, twelve patients with congenital FVII deficiency developed inhibitors. Two had spontaneous resolution of inhibitors and one did not respond to high dose recombinant factor VIIa (rFVIIa) and died. Regarding liver transplant in congenital FVII patients, seven patients underwent liver transplant with good prognosis. We report a 5-year-old girl with confirmed severe congenital FVII deficiency since neonatal period. She suffered from recurrent intracranial bleeding despite rFVIIa replacement. After auxiliary liver transplant at the age of 4, she continued to show persistent deranged clotting profile and was found to have inhibitor towards FVII. Interestingly, she was still responsive to rFVIIa replacement.
[Mh] Termos MeSH primário: Deficiência do Fator VII/terapia
Fator VII/uso terapêutico
Hemorragias Intracranianas/prevenção & controle
Transplante de Fígado
Proteínas Recombinantes/uso terapêutico
[Mh] Termos MeSH secundário: Pré-Escolar
Deficiência do Fator VII/complicações
Feminino
Seres Humanos
Lactente
Recém-Nascido
Hemorragias Intracranianas/etiologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Recombinant Proteins); 9001-25-6 (Factor VII)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170315
[Lr] Data última revisão:
170315
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160809
[St] Status:MEDLINE
[do] DOI:10.1111/hae.13047


  10 / 925 MEDLINE  
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[PMID]:27405678
[Au] Autor:de Moerloose P; Schved JF; Nugent D
[Ad] Endereço:Division of Angiology and Haemostasis, University Hospitals and Faculty of Medicine, Geneva, Switzerland.
[Ti] Título:Rare coagulation disorders: fibrinogen, factor VII and factor XIII.
[So] Source:Haemophilia;22 Suppl 5:61-5, 2016 Jul.
[Is] ISSN:1365-2516
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Rare coagulation disorders (RCDs) include the inherited deficiencies of fibrinogen, factor (F) II, FV, combined FV and VIII, FVII, FX, combined FVII and X, FXI, FXIII and combined congenital deficiency of vitamin K-dependent factors (VKCFDs). Despite their rarity, a deep comprehension of all these disorders is essential to really understand haemostasis. Indeed, even if they share some common features each RCD has some particularity which makes it unique. In this review, we focus on three disorders: fibrinogen, FVII and FXIII.
[Mh] Termos MeSH primário: Afibrinogenemia/diagnóstico
Transtornos Herdados da Coagulação Sanguínea/diagnóstico
Deficiência do Fator VII/diagnóstico
Deficiência do Fator XIII/diagnóstico
[Mh] Termos MeSH secundário: Afibrinogenemia/tratamento farmacológico
Transtornos Herdados da Coagulação Sanguínea/tratamento farmacológico
Fator VII/uso terapêutico
Deficiência do Fator VII/tratamento farmacológico
Fator XIII/genética
Fator XIII/uso terapêutico
Deficiência do Fator XIII/tratamento farmacológico
Fibrinogênio/uso terapêutico
Seres Humanos
Mutação de Sentido Incorreto
Sistema de Registros
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
9001-25-6 (Factor VII); 9001-32-5 (Fibrinogen); 9013-56-3 (Factor XIII)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170216
[Lr] Data última revisão:
170216
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160714
[St] Status:MEDLINE
[do] DOI:10.1111/hae.12965



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