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1.
Chinese Journal of Blood Transfusion ; (12): 542-545, 2023.
Article in Chinese | WPRIM | ID: wpr-1004824

ABSTRACT

【Objective】 To investigate the quality of cryoprecipitates prepared from buffy coat-derived plasma of fresh whole blood at room temperature 20℃-24℃ isolated at different time periods, explore the optimal time for preparing cryoprecipitates, so as to improve the utilization rate of blood. 【Methods】 A total of 250 bags of whole blood collected by CPDA-1 and stored at 20℃-24℃ from October 2020 to December 2020 were randomly selected as the experimental group, and divided into groups A1 (0-8 h), A2 (8-10 h), A3 (10-12 h), A4 (12-14 h) and A5 (14-16 h) (with 50 bags in each group) according to the preparation time point. The upper-buff-coat plasma was separated and quickly frozen as the source for cryoprecipitates. Meanwhile, another 50 bags of fresh frozen plasma prepared within 0-16h after routine storage at 2℃-6℃ were randomly selected as the control group (group B), which was used as the raw plasma to make cryoprecipitate. Coagulation factor Ⅷ (Ⅷ factor) and fibrinogen (FIB) were detected, and the effect of different preparation time and different storage temperature on the content of factor Ⅷ and FIB and the pass rate were compared. 【Results】 In comparison to the control group, the Ⅷ factor content of groups A4 and A5 was significantly decreased, and the differences between groups A4, A5 and B were statistically significant (P0.05). The Factor Ⅷ content ≥60 IU/ bag prepared from buffy coat-derived plasma accounted for 96.4% (1.5 U) in the experimental group. 【Conclusion】 The buffy coat-derived plasma prepared within 12 h at 20℃-24℃ is suitable for preparing 2 U cryoprecipitate coagulation factor, while that prepared within 12-16 h is suitable for preparing 1.5 U cryoprecipitate coagulation factor.

2.
Chinese Journal of Blood Transfusion ; (12): 1143-1147, 2023.
Article in Chinese | WPRIM | ID: wpr-1003952

ABSTRACT

【Objective】 To determine the optimal process conditions for efficiently extracting human prothrombin complex concentrates from human plasma. 【Methods】 Using human plasma as the materials and the yield of prothrombin complex concentrates as the evaluation standard, the preparation process parameters were studied and optimized through design of exporement(DOE), orthogonal experiments, and single factor experiments. 【Results】 The optimal process conditions were as follows: DEAE Sephadex A50 gel was selected, which balanced to pH 7.6, and then the amount of 1.7-2.5 g/L of plasma weight is added into the cryoprecipitate supernatant for adsorption for 40 minutes; Washing solution (0.15-0.175 mol/L sodium chloride) with 3 times the volume of gel was washed 3 times, and eluent (0.5-2.0 mol/L sodium chloride) was washed 3 to 5 times; Add stabilizer (heparin 35 IU, sodium chloride 0.1 mol/L) for ultrafiltration dialysis. 【Conclusion】 By using the optimized process mentioned above, the yield(measured by human coagulation factor IX)can reach 620 000 to 630 000 IU/ton of plasma, which is suitable for large-scale production.

3.
Chinese Journal of Blood Transfusion ; (12): 942-946, 2022.
Article in Chinese | WPRIM | ID: wpr-1004147

ABSTRACT

【Objective】 To understand the current situation of blood components distribution in domestic prefecture-level blood stations through analyzing the components distribution data of 24 prefecture-level blood stations in China. 【Methods】 The data of components distribution of 24 blood stations from 2017 to 2020 as well as the data of blood deployment of 24 blood stations from 2019 to 2020 were collected and analyzed. 【Results】 From 2017 to 2020, positive annual growth in red blood cells, plasma and cryoprecipitate was observed in 22, 19 and 15 out of the 24 blood stations, and the annual growth median rate of above three components was 5.24%, 3.80% and 3.25%, respectively. Among the 24 prefecture-level blood stations, 23 carried out the preparation of cryoprecipitate. 【Conclusion】 The distribution of red blood cells, cryoprecipitate and plasma in prefecture-level blood stations is increasing year by year. However, there is a overstock of plasma, and most blood stations need blood employment.

4.
Chinese Journal of Blood Transfusion ; (12): 1065-1068, 2022.
Article in Chinese | WPRIM | ID: wpr-1004126

ABSTRACT

【Objective】 To analyze the changes of fibrinogen (Fg) and Ⅷ factor levels of cryoprecipitated coagulation factors prepared by different methods and post-preparation quick-freezing time. 【Methods】 The fresh frozen plasma (FFP), prepared from 400mL whole blood, was randomly divided into 6 groups(group A1, A2, A3, B1, B2, B3) with 20 eliquots each, to prepare cryoprecipitate coagulation factors. Group A1, B1 were prepared by automatic cryoprecipitation preparation instrument. group A2, B2 applied the instrument after centrifugation and group A3, B3 were prepared manually. The quick-freezing time after preparation in group A1-A3 and B1-B3 were different(within 1 hour vs. more than 1 hour after preparation). The automated coagulation analyzer was used to measure Fg and Ⅷ factor levels in six groups, and further statistical analysis was carried out. 【Results】 The Fg content (mg) of six groups were 245.29±27.44 in group A1, 227.13±18.68 in group A2, 221.11±20.95 in group A3, 182.12±9.15 in group B1, 163.68±15.50 in group B2, and 155.61±19.28 in group B3, respectively. The Ⅷ factor levels(IU) were of six groups were 115.86±27.99 in group A1, 93.79±36.29 in group A2, 91.92±34.75 in group A3, 83.04±18.82 in group B1, 66.33±19.57 in group B2, and 69.34±13.26 in group B3, respectively. There were no significant differences in gender or age between group As and groups Bs. The levels of Fg and Ⅷ factors in group A1 were significantly higher than those in group A2 and group A3 (P<0.05). In addition, the levels of Fg and Ⅷ factors in group B1 were also obviously higher than those in group B2 and group B3 (P<0.05). Further, the levels of Fg and Ⅷ factors in group As were significantly higher than those in group Bs (P<0.05). 【Conclusion】 The automatic cryoprecipitation preparation instrument plus quick-freezing within 1 hour after preparation contribute to a higher efficiency and better quality than others.

5.
Chinese Journal of Blood Transfusion ; (12): 1135-1138, 2022.
Article in Chinese | WPRIM | ID: wpr-1004074

ABSTRACT

【Objective】 To retrospectively analyze the guiding significance and effect of thromboelogram (TEG) in the clinical use of cryoprecipitation. 【Methods】 A total of 289 patients with fibrinogen reduction, admitted to our hospital between January 2018 and December 2021, were collected. They was divided into control group (using coagulation examination and clinical feature as the transfusion criteria) and observation group (above parameters plus TEG). The TEG index in the observation group before and after transfusion and Fg, APTT, PT, and TT in 2 groups of patients before and after transfusion were monitored. The efficacy and prognosis of different blood products and cryoprecipitate were compared between 2 groups of patients.) 【Results】 The efficacy of choprecipitate transfusion was better in the observation group than the control[Fg index after transfusion (g / L) 1.92±0.92 vs 1.80±1.00, P<0.05]. And less blood products were used in observation group as compared with the control[ RBC(U) 1.93±2.69 vs 2.81±3.25 (P<0.05); FFP(mL) 667±378 vs 879±455(P<0.05)]. No differences were noticed by hospital stay between the two groups, but the prognosis in the observed group was significantly better than that in the control. 【Conclusion】 It’s scientific and reasonable to apply TEG to guide the clinical transfusion of cryoprecipitate, so as to save blood resources and improve the prognosis.

6.
Chinese Journal of Blood Transfusion ; (12): 1261-1262, 2021.
Article in Chinese | WPRIM | ID: wpr-1004023

ABSTRACT

【Objective】 To establish a novel preparation method of cryoprecipitate coagulation factor from overcooled liquid-state plasma. 【Methods】 The fresh liquid plasma was kept at -11℃ to -13℃ for a period of time. It can remain in the liquid state with some coagulation factors generated due to supercooling. Then cryoprecipitate can be obtained from the liquid plasma by siphon method. 【Results】 The average fibrinogen content yielded in cryoprecipitate, prepared from 50 samples of 16-hour-stored fresh liquid plasma, was (186.02±22.72) mg, with the average recovery rate of (37.51±7.42) %, and the average content of coagulation FⅧ was (104.66±22.88) IU, with the average recovery rate of (46.62±5.58) %. 【Conclusion】 The cryoprecipitate coagulation factors could be obtained not only from fresh frozen-thawed plasma, but also from overcooled liquid plasma which is simple and stable, also meets the requirements of relative standards.

7.
Chinese Journal of Blood Transfusion ; (12): 1379-1381, 2021.
Article in Chinese | WPRIM | ID: wpr-1003987

ABSTRACT

【Objective】 To study the concentration, content and yield of coagulation factor Ⅷ(FⅧ) and fibrinogen(Fg) in 25 mL and 45 mL cryoprecipitate. 【Methods】 Forty aliquots(200 mL fresh whole blood) were divided into group A and group B, with 20 samples in each group. Fresh frozen plasma(FFP) was prepared within 6 hours(anticoagulant ACD) according to the standard operating procedure for component preparation. After one week, the prepared FFP was prepared into(25±5) mL and(45±5) mL cold precipitates by siphon method. After freezing for one week, the concentrations of FⅧ and Fg were detected after meltingin by water bath at 37℃, and the content and yield were calculated. 【Results】 The FⅧ concentration, content and yield in group A and group B were(2.990±0.988) vs(2.744±0.940) IU/mL, (74.75±24.71) vs(113.75±30.06)IU, and(70.1±16.6) vs(85.0±7.6)%, respectively.The Fg concentration, content and yield was(6.013±1.679) vs(5.844±0.683) g/L, (150.33±41.99) vs(252.23±26.90)mg, and(41.7±8.6) vs (49.1±9.6)%, respectively. Statistical analysis suggested that the content and yield of FⅧ and Fg were statistically different between the two groups(P0.05). 【Conclusion】 FⅧ and Fg of low-volume cryoprecipitate presented lower content and yield, but slightly higher concentration. Both products can meet the quality requirements of whole blood and blood component. Therefore, reducing product capacity appropriately is suggested when preparing cryoprecipitate with 200mL whole blood

8.
Malaysian Journal of Medicine and Health Sciences ; : 308-310, 2021.
Article in English | WPRIM | ID: wpr-978741

ABSTRACT

@#Recurrent pregnancy loss (RPL) can be defined as loss of pregnancy on or before 20 weeks of gestation. About half of the cases, cause of recurrent miscarriage is unknown. Bleeding disorders induced miscarriage has to be thoroughly investigated for the sake of both mother and fetus. Here is an interesting case report of a 24-year-old patient who was diagnosed to have afibrinogenemia after three consecutive miscarriages. Fibrinogen level was 5 mg/dl with prolonged prothrombin time greater than 180 seconds and activated thromboplastin time greater than 180 seconds. We managed with periodic cryoprecipitate transfusion. Pregnancy course was uneventful and delivered a healthy female child at 34 weeks of gestation under supervision of multidisciplinary team. Here we are discussing the management and how we approached the case to have a successful pregnancy outcome.

9.
Article | IMSEAR | ID: sea-206762

ABSTRACT

Background: Blood transfusion is a life saving measure. Various pregnancy complications and disorders of labor present as risk factors for extra blood loss during pregnancy and cause severe hemodynamic instability. This along with complications due to abortion (spontaneous or induced) and ruptured ectopic pregnancy show up as conditions needing transfusion in the day-to-day practice of obstetrics. In a country like India, limited and fixed resources of blood, forces us to titrate the use of blood and its components. Normally, blood loss during birth is well-tolerated because of changes during pregnancy.Methods: This is a retrospective observational study done at tertiary care hospital. This study is based on study of indoor patients admitted during one year duration. Detailed history and all necessary investigations were carried out. Details regarding blood transfusion were taken indication of blood transfusion, number and type of unit transfused, number of patients given blood components, indications where single unit was transfused. Analysis of the data was done.Results: Anemia followed by antepartum hemorrhage followed by postpartum hemorrhage was the major cause for blood and blood product transfusion. Approximately 60% patients required two units of PCV (Packed Cell Volume) transfusion. Anemia in pregnancy was the major cause of single unit PCV transfusion.Conclusions: A proper knowledge for blood and blood product transfusion is needed to make it available for people who are actually in need and also to decrease the economic burden. Measures to prevent anemia should be implemented. Active management of third stage of labour (AMTSL) should be done to avoid postpartum hemorrhage. Single unit transfusion should be avoided.

10.
J. venom. anim. toxins incl. trop. dis ; 25: e20190038, 2019. tab, ilus
Article in English | LILACS, VETINDEX | ID: biblio-1040381

ABSTRACT

Fibrin biopolymers, previously referred as "fibrin glue" or "fibrin sealants", are natural biomaterials with diverse applications on health. They have hemostatic, adhesive, sealant, scaffold and drug delivery properties and have become widely used in medical and dental procedures. Historically, these biomaterials are produced from human fibrinogen and human or animal thrombin, and the possibility of transmission of infectious diseases by human blood is not ruled out. In the 1990s, to overcome this problem, a new heterologous biomaterial composed of a thrombin-like enzyme purified from Crotalus durissus terrificus venom and a cryoprecipitate rich in fibrinogen extracted from buffaloes Bubalus bubalis blood has been proposed. Therefore, a systematic review of studies on exclusively heterologous fibrin sealants published between 1989 and 2018 was carried out using the following databases: PubMed, SciELO and Google Scholar. The keyword used was "heterologous fibrin sealant". The search resulted in 35 scientific papers in PubMed, four in SciELO and 674 in Google Scholar. After applying the inclusion/exclusion criteria and complete reading of the articles, 30 studies were selected, which formed the basis of this systematic review. It has been observed that the only completely heterologous sealant is the one produced by CEVAP/UNESP. This heterologous biopolymer is proven effective by several studies published in refereed scientific journals. In addition, clinical trials phase I/II for the treatment of chronic venous ulcers authorized by the Brazilian Health Regulatory Agency (ANVISA) were completed. Preliminary results have indicated a safe and promising effective product. Phase III clinical trials will be proposed and required to validate these preliminary findings.(AU)


Subject(s)
Biopolymers , Fibrin , Hemostatics , Thrombin
11.
Article in English | LILACS-Express | LILACS, VETINDEX | ID: biblio-1484695

ABSTRACT

Abstract Hemostatic and adhesive agents date back to World War II, when homologous fibrin sealant came onto scene. Considering that infectious diseases can be transmitted via human blood, a new heterologous fibrin sealant was standardized in the 1990s. Its components were a serine protease (a thrombin-like enzyme) extracted from the venom of Crotalus durissus terrificus snakes and a fibrinogen-rich cryoprecipitate extracted from the blood of Bubalus bubalis buffaloes. This new bioproduct has been used as a coagulant, sealant, adhesive and recently as a candidate scaffold for mesenchymal stem cells and bone and cartilage repair. This review discusses the composition of a new heterologous fibrin sealant, and cites published articles related to its preclinical applications aiming at repairing nervous system traumas and regenerating bone marrow. Finally, we present an innovative safety trial I/II that found the product to be a safe and clinically promising candidate for treating chronic venous ulcers. A multicenter clinical trial, phase II/III, with a larger number of participants will be performed to prove the efficacy of an innovative biopharmaceutical product derived from animal venom.

12.
J. venom. anim. toxins incl. trop. dis ; 23: 21, 2017. tab, graf, ilus
Article in English | LILACS, VETINDEX | ID: biblio-954833

ABSTRACT

Hemostatic and adhesive agents date back to World War II, when homologous fibrin sealant came onto scene. Considering that infectious diseases can be transmitted via human blood, a new heterologous fibrin sealant was standardized in the 1990s. Its components were a serine protease (a thrombin-like enzyme) extracted from the venom of Crotalus durissus terrificus snakes and a fibrinogen-rich cryoprecipitate extracted from the blood of Bubalus bubalis buffaloes. This new bioproduct has been used as a coagulant, sealant, adhesive and recently as a candidate scaffold for mesenchymal stem cells and bone and cartilage repair. This review discusses the composition of a new heterologous fibrin sealant, and cites published articles related to its preclinical applications aiming at repairing nervous system traumas and regenerating bone marrow. Finally, we present an innovative safety trial I/II that found the product to be a safe and clinically promising candidate for treating chronic venous ulcers. A multicenter clinical trial, phase II/III, with a larger number of participants will be performed to prove the efficacy of an innovative biopharmaceutical product derived from animal venom.(AU)


Subject(s)
Animals , Snake Venoms , Fibrinogen , Fibrin Tissue Adhesive , Serine Proteases , Crotalid Venoms
13.
Journal of the Korean Medical Association ; : 387-394, 2016.
Article in Korean | WPRIM | ID: wpr-198615

ABSTRACT

The objective of this review is to evaluate the safety and effectiveness of the amniopatch procedure for the treatment ofpreterm premature rupture of the membranes. The searches were conducted via electronic databases including Ovid-MEDLINE, Ovid-Embase, the Cochrane Library, and eight Korean databases. In the study design, in addition to randomized controlled trials, case report studies in which patients underwent the amniopatch procedure were included. Two reviewers independently selected data in standardized form and assessed the methodological quality. Quality evaluation was performed by the SIGN (Scottish Intercollegiate Guideline Network) method. A total of 11 studies (2 cohort studies, 1 case series, and 8 case reports) were included. There were no serious maternal or fetal complications. It was reported that there were lower rates of maternal chorioamnionitis after the amniopatch relative to conservative treatment (control). The mean gestational age at delivery was 27.7 weeks (a total of 70 cases in 10 studies; spontaneous group, 27.6 weeks; iatrogenic group, 27.8 weeks). The amniopatch was successful in 46.6% of cases (33/71 cases in 11 studies). The overall neonatal survival rate was 55.3% (52/94 cases in 11 studies). Neonatal morbidity was 23.4% (11/47 cases in 7 studies). Although this systematic review, did not find clear evidence of the safety and effectiveness, the amniopatch procedure is a viable treatment option to prolong a pregnancy with previable premature rupture of membranes.


Subject(s)
Female , Humans , Pregnancy , Blood Platelets , Chorioamnionitis , Cohort Studies , Gestational Age , Membranes , Rupture , Survival Rate
14.
Chinese Journal of Primary Medicine and Pharmacy ; (12): 1497-1499, 2016.
Article in Chinese | WPRIM | ID: wpr-486695

ABSTRACT

Objective To investigate the cryoprecipitate fracture hemorrhage clinical application value in the skull fracture hemorrhage,and provide reference for clinical treatment.Methods 80 patients with standard skull frac-ture hemorrhage were selected as the research subjects.The patients were randomly divided into observation group (40 cases)and the control group (40 cases).The control group was given hemostasis,dehydration,protecting stom-ach,antibacterial,nerve nutrition,reducing intracranial pressure,head up in bed and other symptomatic treatment.The observation group received the treatment besides in the control group and added the cryoprecipitate hemostasis,every time given 10U,every 6 -8h 1 time.According to the condition,they were given the cold sediment of 1 -3 times. Then,the cases that fit to give surgery we must cured them by the operation.After treatment 24h,the coagulation inde-xes were examined [prothrombin time (PT),activated partial thromboplastin time (APTT),thrombin time (TT),two D -dimer],plasma fibrinogen (Fbg),International Glasgow (GOS).Finally,we observed clinical prognosis in two groups.Results PT,APTT,TT were significantly shorter than before treatment in the observation group and the con-trol group(t =6.654,5.746,6.193 and 3.342,3.552,3.646,P <0.01 or P <0.05).The PT,APTT and TT of the observation group were significantly shorter than those in the control group (t =3.322,3.406,3.315,all P <0.05). Plasma Fbg was significantly higher than before treatment in two groups(t =5.762,3.592,P <0.01 or P <0.05). Fbg in the observation group was significantly higher than the control group(t =3.407,P <0.05).The clinical prog-nosis in the observation group was significantly better than the control group(χ2 =8.747,P <0.05).Conclusion Cryoprecipitate is a safe and efficient drug.It can effectively improve the blood coagulation dysfunction of patients with fracture of skull base,active endogenous coagulation system,improve hemostatic effect,and reduce the occurrence of progressive cerebral hemorrhage.Therefore,it can improve the prognosis of patients.

15.
Military Medical Sciences ; (12): 199-202, 2015.
Article in Chinese | WPRIM | ID: wpr-460726

ABSTRACT

Objective To compare four pretreatment methods of human plasma coagulation factor Ⅷ( FⅧ) before column purification.Methods Cryoprecipitate was dissolved in Tris , before the supernatant was treated with glycine precipitation, PEG precipitation, acid precipitation and aluminum hydroxide gel adsorption , respectively.Activated partial thromboplastin (APTT) was used to measure the activity of the supernatant clotting FⅧ after treatment.The total activity recovery and specific activity of the final samples were used to weigh the efficacy of those methods .The purity of the intend-ed protein was estimated by non-reducing SDS-PAGE electrophoresis .Results Total activity recovery of glycine precipitati-on was the highest (94.00%±7.60%), followed by that of acidic precipitation (89.47%±2.60%) and PEG precipita-tion (80.92%±9.67%) methods.The lowest was aluminum hydroxide gel adsorption (78.65%±7.52%).Glycine precipitation and PEG precipitation could more effectively remove contaminating protein than acid precipitation and aluminum hydroxide gel adsorption .Treated by four different methods , the specific activity of FⅧ of glycine precipitation sample was the highest (0.6856 ±0.1258 IU/mg), followed by PEG precipitation (0.5773 ±0.0787 IU/mg) and acidic precipitation (0.3885 ±0.0301 IU/mg).The specific activity of aluminum hydroxide gel adsorption was the lowest (0.2879 ±0.0472 IU/mg).Conclusion PEG precipitation is more effective for the actual production process than the other three methods .

16.
Rev. méd. Minas Gerais ; 24(supl.8)dez. 2014.
Article in Portuguese | LILACS-Express | LILACS | ID: lil-749163

ABSTRACT

Dados atuais mostram que apenas 8% da população mundial têm acesso aos 20% de sangue "seguro" para transfusão, devidamente coletados e testados. Apenas 30% das nações do mundo têm serviço transfusional que atende a seu país de forma integrada. Segundo pesquisa conduzida pelo "Committeon Blood and Blood Products of the American Society of Anesthesiologists", grande quantidade do sangue administrado acontece no ambiente cirúrgico. Anestesiologistas devem ser experts em terapia transfusional e conscientes das implicações e complicações que a tomada de decisão pela transfusão pode trazer.


Current data have shown that only 8% of the world's population has access to 20% of "safe" blood dedicated to transfusion, properly collected and tested. Only 30% of the nations around the world have a transfusion service able to work nation wide. According to a research by the "Committee on Blood and Blood Products of the American Society of Anesthesiologists", a great share of the blood given to patients occurs in the cirurgical environment. Anesthesiologists must be experts in transfusional therapy and totally a ware of implications and complications concerning the decision of a blood transfusion.

17.
Bol. Acad. Nac. Med. B.Aires ; 92(2): 309-314, jul.-dic. 2014. tab, ilus
Article in Spanish | LILACS | ID: biblio-998762

ABSTRACT

La enfermedad de von Willebrand tipo plaquetario (PT-VWD) y tipo 2B (2B-VWD) son trastornos hemorrágicos raros, caracterizados por agregación plaquetaria a bajas concentraciones de ristocetina (RIPA). El diagnóstico diferencial no es fácil y representa un desafío. Hasta el presente, sólo se habían reportado cinco mutaciones en el gen GP1BA relacionadas con este desorden. Describimos aquí la sexta mutación relacionada con PT-VWD, en un paciente con sintomatología hemorrágica severa, macro-trombocitopenia, leve agregación plaquetaria espontánea, RIPA positivo a 0,3 y 0,4 mg/mL, VWF:RCo/VWF: Ag<0,2 y estudios discriminatorios positivos para PT-VWD. VWFpp/VWF: Ag resultó normal a diferencia del 2B-VWD que en algunas oportunidades resulta afectado. El exón 28 del gen VWF del paciente y su madre no reveló mutaciones. Identificamos una sustitución G>T en el nucleótido 3805 en el gen GP1BA del paciente, resultando en un cambio de Trp a Leu en el residuo 246 (p.W246L), en la región de la GPIBa que une al VWF. Esta mutación no se identificó en su madre ni en 100 controles sanos. Es considerada como dañina por análisis in sílico. Consideramos que esta sustitución es responsable del fenotipo PT-VWD del paciente. Dada la ausencia de la misma en los 100 normales estudiados, no se considera un polimorfismo


Platelet-type von Willebrand disease (PT-VWD) and type 2B von Willebrand disease (2B-VWD) are rare bleeding disorders characterized by increased ristocetin-induced platelet aggregation (RIPA) at low concentrations. Diagnosis of either condition is not easy and the differential diagnosis is especially challenging. Five mutations in the GP1BA gene related to PT-VWD and near 50 patients are currently reported worldwide. We herein describe a patient with severe bleeding symptoms, macro thrombocytopenia, mild spontaneous platelet aggregation, positive RIPA at 0.3 and 0.4 mg/mL, VWF: RCo/VWF: Ag <0.2, normal VWFpp/VWF: Ag ratio, and RIPA mixing tests and cryoprecipitate challenge positive for PT-VWD. GP1BA gene was studied in the patient, his mother, and 100 healthy control subjects. We identified a substitution G>T at nucleotide 3805 in the patient's GP1BA gene, resulting in a Trp to Leu amino acid change at residue 246 (p.W246L), within the VWF binding region. This mutation was absent in his unaffected mother and also in the 100 controls, and was predicted as damaging by in silico analysis. The residue is located in a strongly conserved position in the phylogenetic tree. These findings argue in favor of considering this substitution does not represent a polymorphism, and is therefore responsible for the PT-VWD phenotype of the patient


Subject(s)
Humans , Male , von Willebrand Diseases/genetics , Platelet Glycoprotein GPIb-IX Complex/genetics , Genetic Predisposition to Disease/genetics , Mutation, Missense , von Willebrand Diseases/blood , DNA Mutational Analysis , Family Health , Amino Acid Sequence
18.
Chinese Journal of Primary Medicine and Pharmacy ; (12): 2724-2726, 2014.
Article in Chinese | WPRIM | ID: wpr-454372

ABSTRACT

Objective To investigate the effects of temperature and time on the quality of cryoprecipitate.Methods Fresh frozen plasmas were divided into the three groups,and the cryoprecipitate were prepared from fresh frozen plasma by siphonage method thawing at 2℃,4℃ or 6℃ water bat.Thawing time,FⅧ and fibfinogen (Fg) were compared among the three groups.Results The thawing time at 2℃,4℃ or 6℃ was (96.3 ± 5.9) min,(89.6 ± 4.3) min,(81.2 ± 2.7) min.The activity of F Ⅷ was (95.36 ± 4.8) IU,(91.21 ± 3.3) IU,(89.8 ±3.1)IU,respectively.The activity of FⅧ was the highest at 2℃ condition and there was significantly different compared with other two groups (t =6.94,P =0.009,t =8.96,P =0.004).The activity of FⅧ in 4℃ group and 6℃ group were comparable (t =0.669,P =0.19).The concentration of Fg at three groups were similar (258.6 ±12.6) mg,(253.3 ±8.2)mg,(255.9 ±9.7) mg (t =0.321,P =0.37;t =0.270,P =0.56;t =0.424,P =0.29).Conclusion The activity of FⅧ at 2℃ condition were the highest but the thawing time was the longest.The activity of FⅧ at 6℃ condition were lower but met quality requirements and the thawing time was very short.There was no significantly different at Fg concentration among three groups.Thus 6℃ is more suitable for blood center to prepare cryoprecipitate from fresh frozen plasmas.

19.
The Journal of Practical Medicine ; (24): 1572-1575, 2014.
Article in Chinese | WPRIM | ID: wpr-451959

ABSTRACT

Objective To explore the effect of Vitamin K1(Vit K1), fresh frozen plasma (plasma) andcryoprecipitate on prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen original(Fbg), thrombin time (TT) of newborns with different gestational ages. Methods The serum of 1,134 newbornsfrom The Third Affiliated Hospital of Guangzhou Medical University was collected from February 2009 to September2012. All newborns had been divided into four groups (according to the gestational age of 28-31+6 weeks, 32-33+6weeks, 34-36+6 weeks and gestational age≥37 weeks).The effect of various interventions (Vit K1, Vit K1+plasmaand Vit K1+cryoprecipitate) on PT, APTT, Fbg, and TT had been recorded. Results (1)The PT and APTT ofeach group with the interventions of Vit k1 were significantly improved (P < 0.05). (2)The PT, APTT, Fbg and TTof each group with the interventions of Vit k1 combined with plasma were significantly improved (P < 0.05). (3)ThePT, APTT and Fbg of each group with the interventions of Vit k1 combined with cryoprecipitate were significantlyimproved (P < 0.05). (4)With Vit k1 combined with plasma, PT and APTT were mostly improved and Fbg wasimproved mostly with Vit k1 combined with cryoprecipitate. Conclusion Vitamin K1, fresh frozen plasma andcryoprecipitate can effectively improvedin the coagulation index of newborns with different gestational ages.

20.
Chinese Journal of Blood Transfusion ; (12)2008.
Article in Chinese | WPRIM | ID: wpr-593504

ABSTRACT

Objective To evaluate the quality of cryoprecipitates prepared from fresh frozen plasma via deepfreeze method and siphonage method thawing at 4℃ water bath,and to select a proper preparation method for cryoprecipitates.Methods Sixty samples of cryoprecipitate prepared by deepfreeze method,and 60 samples of cryoprecipitate prepared by siphonage method were selected randomly.Factor FⅧ,fibrinogen(Fg) in cryoprecipitates were measured by coagulometer,and the qualification rates were calculated.Results The concentration of FⅧ(IU/pack) in the cryoprecipitates prepared via deepfreeze method and siphonage method were(70.30?23.68),and(161.62?39.45),respectively;the qualification rate of FⅧ prepared via the two methods were 43.33%(26/60),and 93.33%(56/60),respectively(P0.05).Conclusion Siphonage method,with a higher qualification rate of FⅧ,is simple and fast,which deserves a popular application.

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