scholarly journals Haemophilic Pelvic Pseudotumour: A New Surgical Option

Healthcare ◽  
2021 ◽  
Vol 9 (10) ◽  
pp. 1269
Author(s):  
Gianluigi Pasta ◽  
Roberta Ruggieri ◽  
Salvatore Annunziata ◽  
Alessandro Gallese ◽  
Vincenzo Pio Gagliardi ◽  
...  
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Clotting Factor ◽  
Recurrent Bleeding ◽  
Spontaneous Bleeding ◽  
Polyglycolic Acid Mesh ◽  
Rod System ◽  
Plasma Factor

Background: Haemophilia is an inherited coagulopathy caused by the absence or dysfunction of clotting factor VIII or IX. Clinical manifestations are generally secondary to recurrent bleeding episodes mainly in the musculoskeletal system. Bleeding symptoms appear early in life and, when the disease is severe (when plasma factor VIII or IX activity is <1% of normal), joint and muscle bleeding may occur spontaneously. A pseudotumour is a recurrent, chronic, encapsulated, slowly expanding, muscle hematoma. Haemophilic pseudotumour is a rare complication of haemophilia which occurs, as a condition either from repeated spontaneous bleeding or coming from a traumatic origin, in 1–2% of haemophilic patients. Case report: A 32-year-old man with severe haemophilia A referred to our Clinic with a massive right iliac wing pseudotumour complicated by Staphylococcus aureus superinfection and skin fistulisation. In this report we describe the medical management and surgical treatment by the adoption of a novel surgical technique which involves the use of a pedicle-screw and rod system (PSRS), a polyglycolic acid MESH and bone cement in order to build up an artificial ilium-like bony mass. This case report highlights the importance of interdisciplinary approach and the efficacy of eradicating surgery as treatment, especially in the case of large and long-lasting lesions.

TT Virus Is Present in a High Frequency of Italian Hemophilic Patients Transfused With Plasma-Derived Clotting Factor Concentrates

Blood ◽  
1999 ◽  
Vol 94 (12) ◽  
pp. 4333-4336 ◽  
Author(s):  
Benjamin P. Chen ◽  
Maria Grazia Rumi ◽  
Massimo Colombo ◽  
Yu-Huei Lin ◽  
Latha Ramaswamy ◽  
...  
Keyword(s):  
Factor Viii ◽  
Odds Ratio ◽  
Causal Effect ◽  
Clotting Factor ◽  
Hepatitis Viruses ◽  
Tt Virus ◽  
Plasma Factor ◽  
Immunodeficiency Virus ◽  
Factor Viii Concentrates ◽  
Clotting Factor Concentrates

The prevalence of the blood-borne TT virus (TTV) in Italian hemophiliacs treated with different preparations of factor VIII was determined. Of the 178 hemophilic patients (mean age, 29 years), TTV-DNA was found in 123 (69%), in comparison to 22 of 100 (22%) blood donors (P < .0001). Of the 123 patients who tested positive for TTV, significant numbers were also infected with human hepatitis viruses and/or human immunodeficiency virus (HIV): 31% had TTV and hepatitis C virus (HCV), 22% had TTV, and at least 2 of the 4 known human blood-borne viruses tested, whereas 15% had TTV alone. The risk of acquiring TTV alone was only slightly higher in recipients of unmodified plasma factor concentrates (78%, odds ratio, 1.24; 95% confidence interval [CI], 0.27 to 5.79) than in patients treated with virus inactivated concentrates (67%), whereas the risk was significantly lower in recipients of recombinant factors (11%, odds ratio, 0.09; 95% CI, 0.01 to 0.52). Serum alanine aminotransferase (ALT) levels were elevated in 2 of 27 patients (7%) with TTV alone compared with 43 of 56 patients (77%) coinfected with TTV and HCV and compared with 16 of 21 patients (76%) with HCV alone. Taken together, these results indicate that TTV frequently infects Italian hemophiliacs treated with plasma-derived factor VIII concentrates, both unmodified and virus-inactivated. Our results do not suggest a causal effect of TTV on chronic liver disease in these patients.

TT Virus Is Present in a High Frequency of Italian Hemophilic Patients Transfused With Plasma-Derived Clotting Factor Concentrates

Blood ◽  
1999 ◽  
Vol 94 (12) ◽  
pp. 4333-4336 ◽  
Author(s):  
Benjamin P. Chen ◽  
Maria Grazia Rumi ◽  
Massimo Colombo ◽  
Yu-Huei Lin ◽  
Latha Ramaswamy ◽  
...  
Keyword(s):  
Factor Viii ◽  
Odds Ratio ◽  
Causal Effect ◽  
Clotting Factor ◽  
Hepatitis Viruses ◽  
Tt Virus ◽  
Plasma Factor ◽  
Immunodeficiency Virus ◽  
Factor Viii Concentrates ◽  
Clotting Factor Concentrates

Abstract The prevalence of the blood-borne TT virus (TTV) in Italian hemophiliacs treated with different preparations of factor VIII was determined. Of the 178 hemophilic patients (mean age, 29 years), TTV-DNA was found in 123 (69%), in comparison to 22 of 100 (22%) blood donors (P &lt; .0001). Of the 123 patients who tested positive for TTV, significant numbers were also infected with human hepatitis viruses and/or human immunodeficiency virus (HIV): 31% had TTV and hepatitis C virus (HCV), 22% had TTV, and at least 2 of the 4 known human blood-borne viruses tested, whereas 15% had TTV alone. The risk of acquiring TTV alone was only slightly higher in recipients of unmodified plasma factor concentrates (78%, odds ratio, 1.24; 95% confidence interval [CI], 0.27 to 5.79) than in patients treated with virus inactivated concentrates (67%), whereas the risk was significantly lower in recipients of recombinant factors (11%, odds ratio, 0.09; 95% CI, 0.01 to 0.52). Serum alanine aminotransferase (ALT) levels were elevated in 2 of 27 patients (7%) with TTV alone compared with 43 of 56 patients (77%) coinfected with TTV and HCV and compared with 16 of 21 patients (76%) with HCV alone. Taken together, these results indicate that TTV frequently infects Italian hemophiliacs treated with plasma-derived factor VIII concentrates, both unmodified and virus-inactivated. Our results do not suggest a causal effect of TTV on chronic liver disease in these patients.

scholarly journals Further characterization of factor VIII-deficient mice created by gene targeting: RNA and protein studies

Blood ◽  
1996 ◽  
Vol 88 (9) ◽  
pp. 3446-3450 ◽  
Author(s):  
L Bi ◽  
R Sarkar ◽  
T Naas ◽  
AM Lawler ◽  
J Pain ◽  
...  
Keyword(s):  
Factor Viii ◽  
Light Chain ◽  
Knockout Mice ◽  
Donor Site ◽  
Spontaneous Bleeding ◽  
Knockout Strain ◽  
Plasma Factor ◽  
Pregnancy And Delivery ◽  
Deficient Mice

Previously we created two strains of factor VIII-deficient mice by insertion of a neo gene into (1) the 3′ end of exon 16 and (2) exon 17 of the factor VIII gene. Affected mice of both strains have no plasma factor VIII activity, yet are healthy with no spontaneous bleeding. Factor VIII-deficient females bred with affected males survive pregnancy and delivery. We used reverse transcriptase-polymerase chain reaction of liver RNA to characterize factor VIII mRNA processing. Factor VIII mRNA of the exon 16 knockout strain contains neo sequences plus 17 bp of intron 16 due to use of a cryptic donor site in intron 16. All factor VIII mRNA of the exon 17 knockout strain lacks exon 17 and neo sequences. In skipping exon 17, the intron 16 donor site or a cryptic donor site 46 bp 3′ to the intron 16 donor site are used. Thus, factor VIII deficiency in exon 16 knockout mice is due to truncated protein, while in exon 17 knockout mice it is due to either truncated or partially deleted protein. After immunizing exon 16 knockout mice with human recombinant factor VIII, two monoclonal antibodies were obtained that recognize <7 100 pg of mouse factor VIII light chain. Assay of cryoprecipitate from the plasma of affected mice failed to show factor VIII light chain.

The Role of Coagulation Factor VIII Substutution Therapy in the Management of Type A Hemophilia Complications

2018 ◽  
Vol 69 (4) ◽  
pp. 994-996
Author(s):  
Oana Viola Badulescu ◽  
Dan Vintila ◽  
Bogdan Mihnea Ciuntu ◽  
Manuela Ciocoiu ◽  
Paul Dan Sirbu
Keyword(s):  
Factor Viii ◽  
Clinical Manifestations ◽  
Coagulation Factor ◽  
Type A ◽  
Severe Form ◽  
Microcytic Anemia ◽  
Clotting Factor ◽  
Motor Deficits ◽  
Substitution Therapy ◽  
Prolonged Aptt

Hemophilia is a pathologycharacterized by a disturbance in the normal clotting process of the blood, as a result of a functional deficit of clotting factor VIII or IX.There are two types of hemophilia: type A and type B, the former expressing a deficit of factor VIII, while the latter a deficit of factor IX.C.L., a 43 years old male, was diagnosed at the age of 8 months with a severe form of type A hemophilia. From that time, the patient presentedhemorrhagic episodes, resulting either from trauma or appearing spontaneously and requiring factor VIII substitution therapy and hemostatic treatment.Bloodwork show a prolonged aPTT, as well as microcytic anemia with a low iron serum level, as a result of chronic blood loss.The particularity of this case consists in the high degree of development and recurrence of hemophilia associated complications, leading to superior and inferior limb motor deficits and chronic pain which is currently managed with opioid medication. Also,the patient suffers from chronic hepatitis due to infection with B and C hepatitis viruses as a result of previous lifesaving plasma substitution therapy.Even though the clinical manifestations of hemophilia excert a high cost on the patient�s life as well as the medical system, timely prophylactic factor VIII substitution therapy from the moment of diagnosis until the age of 18 can reduce both the toll on the patient�s health and quality of life and also on the medical costs. Thus, we stress the fact that prophylactic therapy can have a beneficial effect both on patient�s life and also on the health system.

Familial Clustering of Factor VIII and von Willebrand Factor Levels

1998 ◽  
Vol 79 (02) ◽  
pp. 323-327 ◽  
Author(s):  
Jeanine Houwing-Duistermaat ◽  
Hans van Houwelingen ◽  
Jeroen Eikenboom ◽  
Rogier Bertina ◽  
Frits Rosendaal ◽  
...  
Keyword(s):  
Factor Viii ◽  
Blood Group ◽  
Von Willebrand Factor ◽  
Familial Aggregation ◽  
Univariate Analysis ◽  
Clotting Factor ◽  
Thrombotic Risk ◽  
Plasma Factor ◽  
Von Willebrand ◽  
Willebrand Factor

SummaryRecently, we found that high levels of clotting factor VIII (>150 IU/dl) are common and make an important contribution to thrombotic risk. The determinants of high factor VIII:C are unclear and might be partly genetic. Therefore, we tested the influence of age, blood group and von Willebrand factor (VWF) levels on factor VIII:C levels, and investigated whether factor VIII:C levels are genetically determined. We performed an analysis of 564 female relatives of hemophilia A patients, who had visited our center for genetic counseling. In univariate analysis, AB0 blood group, age and VWF antigen (VWF:Ag) levels all influenced factor VIII:C levels. After adjustment for the effect of VWF:Ag levels, both blood group and age still had an effect on factor VIII:C levels. In sister pairs, the Pearson correlation coefficient between factor VIII:C levels was 0.17 (p = 0.024) and this correlation remained positive (0.15, p = 0.046) after correction for the influence of VWF:Ag. In mother-daughter pairs, no correlation of factor VIII:C levels was found. The correlation of VWF:Ag levels in sisterpairs was 0.41 (p <0.001) and in mother-daughter pairs 0.44 (p <0.001), in line with the assumption that VWF:Ag levels are under control of autosomal genes. Familial influence on plasma factor VIII:C and VWF:Ag levels was investigated with a recently developed familial aggregation test. This test verifies whether familial aggregation of a particular parameter exists in a set of pedigrees. In 435 women from 168 families, factor VIII:C as well as VWF:Ag levels correlated significantly within families, which suggests a familial influence. The familial aggregation was more prominent for VWF:Ag levels than for factor VIII:C levels, possibly because the genetic effect on VWF:Ag levels is larger than on factor VIII:C levels. Our results support the presence of a familial influence on factor VIII:C as well as on VWF:Ag levels.Our results support the presence of a familial influence on factor VIII:C as well as on VWF:Ag levels.

Homoeopathic Management of a Case of Severe Haemophilia Type A with Inhibitor Positive: Case Report

2020 ◽  
Vol 33 (04) ◽  
pp. 302-308
Author(s):  
Hiral Shah ◽  
Tapas Kumar Kundu ◽  
Afroz Farooque Shaikh
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Replacement Therapy ◽  
Psychological Status ◽  
Clotting Factor ◽  
Factor Viii Inhibitor ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Clotting Factors ◽  
Bleeding Episodes

AbstractHaemophilia is an X-linked inherited immunogenetic bleeding disorder resulting from deficiency of clotting Factor VIII (haemophilia A) or Factor IX (haemophilia B). Haemophilia patients suffer from complication of developing autoantibodies/inhibitor against clotting factors used for the treatment; most commonly patients are treated with Factor VIII replacement therapy. In modern medicine, haemophiliacs with inhibitor positive status are treated with bypassing agents such as Factor VIII inhibitor bypassing agent and immune tolerance induction therapy (ITI) because such patients do not respond to traditional factor replacement therapy during an event of active bleeding. Treatment with ITI is very expensive and it requires medical expertise. Moreover, high cost of such treatment is one part of the problem, while its availability is another problem especially in developing countries. The inhibitor status among haemophilia patients is identified by conducting a blood test which measures the Bethesda units (BU) levels in the blood. In this case report, the homoeopathic management of a patient with haemophilia A severe type (Factor VIII <1%), inhibitor positive (4 BU/mL), is presented. The patient underwent treatment for a span of 4 years. After closely assessing the patient's condition and applying the principles of homeopathy medicine selection, his frequent bleeding episodes were treated with homoeopathic medicines such as Hamamelis Virginica Q, Phosphorus, Arnica montana, Rhus toxicodendron, Calendula officinalis, and Pulsatilla nigricans. Intercurrent medicine—Tuberculinum bovinum—was given when the most indicated medicine failed to relieve the symptoms of the case and was given during non-bleeding phase. The medicines not only helped in reducing haemophilia-related bleeding episodes but also improved complaints of pain, relieved skin complaints, and showed improvement in overall psychological status of patient. It can be concluded that homeopathy medicines were able to successfully reduce the frequency of bleeding and intensity of pain in this patient. Owing to reduced bleeding, he required relatively a smaller number of factor replacement treatment compared with earlier when he was not taking homeopathy. Homoeopathy proved to be effective in managing severe haemophilia patient as a supportive therapy and was able to contribute toward reduced inhibitor levels in severe haemophilia patient.

scholarly journals Case Report: Effective Treatment for Acute Chlorpyrifos Poisoning Complicated by a Non-ST-Segment Elevation Myocardial Infarction

2021 ◽  
Vol 8 ◽  
Author(s):  
Changqing Ye ◽  
Qiang Zhang ◽  
Yongsheng Chao ◽  
Chun Yin
Keyword(s):  
Myocardial Infarction ◽  
Case Report ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Organophosphorus Pesticide ◽  
Coronary Intervention ◽  
Organophosphate Insecticide ◽  
St Segment Elevation ◽  
First Case ◽  
St Segment

Background: Acute myocardial infarction (AMI) is a rare complication of acute organophosphorus pesticide poisoning. Although chlorpyrifos has been widely used as an organophosphate insecticide, a few cases of AMI complicated by chlorpyrifos poisoning have been reported thus far. Hence, a suitable treatment strategy remains to be explored.Case Presentation: Based on the clinical manifestations, medical history, results of an auxiliary examination, and serum biomarkers, a 65-year-old male farmer with complaints of nausea, vomiting, chest tightness, and pain was clearly diagnosed as having a severe chlorpyrifos self-poisoning with acute non-ST-segment elevation MI. Because the patient and his family confirmedly refused a coronary intervention, conservative treatment was used instead. It should be noted that there were some conflicts of the management for chlorpyrifos poisoning and AMI. Although rapid atropinization would contribute to the relief of muscarinic symptoms, it would also lead to an increased heart rate and myocardial oxygen consumption in AMI. Furthermore, the reduction of platelet aggregation, which is necessary for coronary recanalization of an AMI patient, is known to aggravate the gastrointestinal injury caused by poisoning. In this case, these conflicts were properly addressed, which led to an excellent effect and prognosis of the patient.Conclusions: To our knowledge, this is the first case report of acute chlorpyrifos poisoning with AMI. It is emphasized that patients with chest pain or coronary heart disease should be treated with atropine more cautiously because of the possible AMI. Moreover, proper resolution of conflicts in the management for chlorpyrifos poisoning and AMI played contributing roles in patient improvement.

scholarly journals Combined Factor V and Factor VIII Deficiency: A Case Report

2016 ◽  
Vol 5 (1) ◽  
pp. 42-44
Author(s):  
Ehsan Shahverdi ◽  
Hassan Abolghasemi ◽  
Fardin Dolatimehr ◽  
Sara Beheshtian ◽  
Shima Melatshahi Chaeichi ◽  
...  
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Autosomal Recessive ◽  
Consanguineous Marriage ◽  
Factor V ◽  
Spontaneous Bleeding ◽  
Factor Viii Deficiency ◽  
Consanguineous Marriages ◽  
Coagulation Defect ◽  
History Of

Background: Combined factor V and factor VIII deficiency (F5F8D, OMIM 227300) is a rare autosomal recessive bleeding disorder. It seems more common among Jews and Iranians, particularly in regions with frequent consanguineous marriages. Case Report: We describe a 5-yearold  girl  born  out  of  consanguineous  marriage  with  a  complaint  of  prolonged  bleeding  after dental extraction. There were no history of spontaneous bleeding and other coagulation defect symptoms. Conclusion: In frequent consanguineous marriage regions, inherited deficiency of factor V and VIII should be considered in patients with coagulation defect symptoms.[GMJ. 2016;5(1):42-44]

scholarly journals Effect of danazol on clotting factor levels, bleeding incidence, factor infusion requirements, and immune parameters in hemophilia

Blood ◽  
1986 ◽  
Vol 68 (3) ◽  
pp. 673-679 ◽  
Author(s):  
P Saidi ◽  
BZ Lega ◽  
HC Kim ◽  
K Jr Raska
Keyword(s):  
Factor Viii ◽  
Plasma Levels ◽  
Fibrinolytic Activity ◽  
Clotting Factor ◽  
Clotting Factors ◽  
Double Blind ◽  
Immune Parameters ◽  
Plasma Factor ◽  
Viii And Ix ◽  
Mucosal Bleeding

Abstract Several recent studies have reported conflicting results on the effectiveness of danazol, an attenuated androgen, in raising plasma levels of clotting factors VIII and IX in patients with hemophilia. We undertook a randomized, double-blind cross-over trial using 8 weeks' administration of danazol (D), 600 mg/d, and 8 weeks' administration of placebo (P) separated by 2 weeks of rest in 12 patients with hemophilia A and four patients with hemophilia B. Plasma factor VIII and IX levels, frequency and type of bleeding episodes, amount of factor concentrate infused, fibrinogen, fibrinolysis assays, antithrombin III, liver function, and immune parameters were followed. During the danazol phase a minimal increase was noted in the average clotting factor levels, an increase that, although statistically significant, was of hemostatically marginal magnitude. Significant increases in protein C and plasminogen levels, however, were observed during the danazol period, suggestive of danazol-mediated enhanced fibrinolysis. Clinically, bleeding frequency was significantly increased, and more clotting factor was consumed during the danazol period. Furthermore, eight episodes of hematuria and oral mucosal bleeding was reported during the danazol phase in contrast to only one episode of hematuria during the placebo phase, consistent with an enhancement of fibrinolytic activity with danazol. We conclude that danazol does not have a hemostatically significant effect on plasma levels of factor VIII and IX but may be associated with enhancement of fibrinolytic activity, resulting in increased bleeding frequency and requiring more clotting factor infusions. Therefore, danazol is not a viable alternative in the treatment of hemophilia.

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