Management of rare coagulation disorders in 2018

Jain, Shilpa; Acharya, Suchitra S.

doi:10.1016/j.transci.2018.10.009

Cited by 38 publications

(54 citation statements)

References 89 publications

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“…Like the SARS and MERS, the most common complication was acute respiratory distress syndrome, owing to direct injury of SARS-CoV-2 and secondary injury of inflammation ( Rabaan et al, 2020 ). Injury of vascular endothelium and hematopoietic function by viral toxins and inflammatory factors caused coagulation disorders ( Jain & Acharya, 2018 ). So far, there were no effective antiviral drugs for SARS-CoV-2.…”

Section: Discussionmentioning

confidence: 99%

“…Acute cardiac injury was diagnosed if serum levels of cardiac biomarkers (eg, troponin I) were above the 99th percentile upper reference limit, or new abnormalities were shown in electrocardiography and echocardiography ( Shi et al, 2020 ). Coagulation disorders was defined as a 3-second extension of prothrombin time or a 5-second extension of activated partial thromboplastin time ( Jain & Acharya, 2018 ). Nosocomial infection was diagnosed if the patients had clinical symptoms or signs of nosocomial pneumonia or bacteraemia, and was combined with a positive culture of a new pathogen from a lower respiratory tract specimen (including the sputum, transtracheal aspirates, or bronchoalveolar lavage fluid, or from blood samples taken≥48 h after admission) ( Garner, Jarvis, Emori, Horan, & Hughes, 1988 ).…”

Section: Methodsmentioning

confidence: 99%

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Clinical characteristics of 312 hospitalized older patients with COVID-19 in Wuhan, China

Lü

Zhang

et al. 2020

Archives of Gerontology and Geriatrics

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Highlights Most of the patients with COVID-19 who need to be admitted to hospital are older. Older patients with COVID-19 had much more co-morbidity, complications and mortality. Age, SOFA score, APACHEⅡ score, platelet count＜125 × 109/L, d-dimer, creatinine＞133 μmol/L, interleukin-6, and lung consolidation on admission were independent risk factors for severe cases among older patients with COVID-19. More attention should be paid to older patients with COVID-19.

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Clinical characteristics of 312 hospitalized older patients with COVID-19 in Wuhan, China

Lü

Zhang

et al. 2020

Archives of Gerontology and Geriatrics

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“…If it is not available, fresh frozen plasma or cryoprecipitates can also be used. Clinical trials have also shown the efficacy of purified plasma-derived factor XIII concentrate and recombinant factor XIII-A 2 in treating these patients 19…”

Section: Discussionmentioning

confidence: 99%

Factor XIII deficiency with intracranial haemorrhage

Ejaz¹,

Saleem

Ali

et al. 2019

BMJ Case Rep

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A 5-year-old girl presented to paediatric emergency with fever and seizures for a short duration. At first, meningitis was suspected and management was started empirically. There was no improvement in the clinical condition of the patient and investigations revealed spontaneous intracranial haemorrhage (ICH) secondary to factor XIII deficiency. The child was transfused cryoprecipitate and managed conservatively for ICH. She became asymptomatic and was kept on monthly cryoprecipitate transfusions. This case report summarises factor XIII deficiency in ICH which was not suspected initially, but diagnosed later on after CT scan head and factor XIII assay. This report also highlights events occurring during its management.

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“…Inherited quantitative or qualitative deficiencies of coagulation factors such as factor I (fibrinogen-FI), factor II (FII), factor V (FV), combined FV and factor VIII (FVIII), factor VII (FVII), factor X (FX), factor XI (FXI), factor XIII (FXIII), and vitamin K-dependent clotting factors (FII, FVII, FIX, FX) called as rare bleeding disorders (RBDs) (2). The deficiency of any of the coagulation factors may result in a coagulopathy leading to bleeding episodes (3,4). Hemophilia A (FVIII) and B (FIX) and together with von Willebrand disease, they account for 95% to 97% of all coagulopathies (5,6), however RCDs are much less prevalent, ranging from case in 500,000 to 1 in 2 million in the general population and it could be vary by country (7).…”

Section: Introductionmentioning

confidence: 99%

Inherited Rare Factor Deficiencies: Single-centre Experience

Koç¹,

Zülfikar²

2021

meandros

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Amaç: Kanama insan hayatındaki en önemli sorunlardan bir tanesidir. Koagülasyon sisteminde yer alan faktörlerin eksikliği, yokluğu veya fonksiyon bozukluğu uzamış kanama, morbidite ve mortaliteye sebep olmaktadır. Faktör eksikliklerinin büyük çoğunluğu faktör VIII, faktör IX ve von Willebrand faktör eksikliği olup diğer faktör eksiklikleri nadir faktör eksiklikleri olarak adlandırılır. Bu çalışmada, nadir faktör eksikliği olan hastaların klinik özellikleri, laboratuvar bulguları, tedavileri ve uygulanan cerrahi girişimler sunulmuştur. Gereç ve Yöntemler: Çocuk hematolojisi ve onkolojisi bilim dalında Temmuz 1997 ve Haziran 2020 tarihleri arasında takip edilen faktör VII eksikliği dışında nadir faktör eksikliği tanısı alan hastalar çalışmaya dahil edildi. Hastaların demografik özellikleri, klinik bulguları, aile hikayeleri, protrombin zamanı/aktive parsiyel Objective: Bleeding is one of the most important problems in humans. Absence, deficiency or dysfunctions of protein factors in the coagulation system can cause prolonged bleeding, morbidity or mortality. Although factor VIII, factor IX and von Willebrand factor deficiencies are the most common, deficiencies in all other factors exist, called rare factor deficiencies. This study aimed to present the clinical presentations, laboratory findings, treatments, and surgical interventions in patients with rare factor deficiencies other than factor VII followed up in our clinic. Materials and Methods: Patients who were diagnosed with rare factor deficiency other than factor VII in the department of pediatric hematology and oncology between July 1997 and June 2020 were included in this study. Patients' demographic characteristics, clinical presentations, family history, prothrombin time, activated partial thromboplastin time and factor levels, treatments and surgical interventions were recorded retrospectively from patients' files. Results: Nineteen patients were included in the study, of which 7 (37%) had factor X deficiency, 5 (25%) had factor XI, 3 (16%) had factor V+VIII, 2 (10%) had factor V and 1 (5%) had factor I and XIII deficiencies. Parents of 12 patients had consanguinity marriages. All patients with factor X deficiency had bleeding episodes, and three of them were under prophylaxis with prothrombin complex concentrate. Other patients were under on-demand treatment. In total, 19 surgical interventions (11 minor; 8 major) were performed. Conclusion: Rare bleeding disorders are very uncommon and heterogeneous, with variable associations between coagulation factor activity and bleeding phenotype. A multidisciplinary and expertise team (haematologists, nurses, gynaecologists, obstetricians, orthopaedist, etc.) is necessary for the treatment and regular followup of patients with rare bleeding disorders.

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Management of rare coagulation disorders in 2018

Cited by 38 publications

References 89 publications

Clinical characteristics of 312 hospitalized older patients with COVID-19 in Wuhan, China

Clinical characteristics of 312 hospitalized older patients with COVID-19 in Wuhan, China

Factor XIII deficiency with intracranial haemorrhage

Inherited Rare Factor Deficiencies: Single-centre Experience

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