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The inhibitors – a challenge for the management of patients with hereditary haemophilia A

Abstract

Introduction. Our research strategy was aimed at evaluating the possible implication of the type of factor VIII product administered as substitution treatment to haemophilia A patients in the occurrence of inhibitors and their consequences on the management.

Methods. Scientific articles from July 2015 to July 2017 were searched using the PubMed and PubMed Central databases. The used search terms included “haemophilia A”, “inhibitors”, “plasma-derived factor VIII” and “recombinant factor VIII”.

Results. The risk factors for inhibitors occurrence may be patients-related (genetic and nongenetic) and treatment-related. The possibility of a correlation between the increased purity of factor VIII given as substitution treatment and the occurrence of inhibitors is discussed in the light of literature data. Plasma-derived factor VIII is less immunogenic, but not entirely safe from the point of view of the possibility of transmitting biological agents. It is obvious that there is not enough plasma-derived factor VIII for the planet’s needs. Recombinant factor VIII products have revolutionized the treatment of patients with haemophilia A over the past 3 decades by the disappearance of transfusion-related infections and their complications. They are safer in terms of pathogens and the new long-acting factor VIII products are based on recombinant DNA technology.

Conclusion. Plasma-derived or recombinant factor VIII products must co-exist on the market for the benefit of haemophilic patients. Future solutions could be: less immunogenic factor VIII products, nonfactor replacement strategies, or bispecific antibody that mimics the function of coagulation factor VIII.

Open access
EUS – Fine- Needle Aspiration Biopsy (FNAB) in the Diagnosis of Pancreatic Adenocarcinoma: A Review

Solid masses of the pancreas represent a variety of benign and malignant neoplasms of the exocrine and endocrine tissues of the pancreas. A tissue diagnosis is often required to direct therapy in the face of uncertain diagnosis or if the patient is not a surgical candidate either due to advanced disease or comorbidities. Endoscopic ultrasound (EUS) is a relatively new technology that employs endoscopy and high-frequency ultrasound (US). EUS involves imaging of the pancreatic head and the uncinate from the duodenum and imaging of the body and tail from the stomach. It has been shown to be a highly sensitive method for the detection of pancreatic masses. It is superior to extracorporeal US and computed tomographic (CT) scans, especially when the pancreatic tumor is smaller than 2-3 cm. Although EUS is highly sensitive in detecting pancreatic solid masses, its ability to differentiate between inflammatory masses and malignant disease is limited. Endoscopic retrograde cholangiopancreatography (ERCP) brushing, CT-guided biopsies, and transabdominal ultrasound (US) have been the standard nonsurgical methods for obtaining a tissue diagnosis of pancreatic lesions, but a substantial false-negative rate has been reported. Transabdominal US-guided fine-needle aspiration biopsy (US-FNAB) has been used for tissue diagnosis in patients with suspected pancreatic carcinoma. It has been shown to be highly specific, with no false-positive diagnoses. With the advent of curvilinear echoendoscopes, transgastric and transduodenal EUS-FNAB of the pancreas have become a reality EUS with FNAB has revolutionized the ability to diagnose and stage cancers of the gastrointestinal tract and assess the pancreas. Gastrointestinal cancers can be looked at with EUS and their depth of penetration into the intestinal wall can be determined. Any suspicious appearing lymph nodes can be biopsied using EUS/FNAB. The pancreas is another organ that is well visualized with EUS. Abnormalities such as tumors and cysts of the pancreas can be carefully evaluated using EUS and then biopsied with FNAB. There are many new applications of EUS using FNAB. Researchers are looking to deliver chemotherapeutics into small pancreatic cancers and cysts. Nerve blocks using EUS/FNAB to inject numbing medicines into the celiac ganglia, a major nerve cluster, are now routinely performed in patients with pain due to pancreatic cancer. The aim of this study is to perform a review of the literature regarding the usefulness of EUS/FNAB in the diagnosis of pancreatic adenocarcinoma.

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Advanced Endoscopic Imaging Techniques for the Study of Colonic Mucosa in Patients with Inflammatory Bowel Disease

Epidemiology and Immunobiology to a Rational Diagnostic and Therapeutic Approach. 2012, XVIII, 216-217. 10. JEWELL DP, MORTENSEN NJ, STEINHART AH, JOHN A. PEMBERTON, BRYAN F. WARRE. Cancer: New colonoscopic techniques. In: Challenges in Inflammatory Bowel Disease, 2nd Edition, 2008, 294-295. 11. BARKIN JA, SUSSMAN DA, ABREU MT. Chromoendoscopy and advanced imaging technologies for surveillance of patients with IBD. Gastroenterology & Hepatology, 2012; 8 (12): 796-802. 12. KIESSLICH R, FRITSCH J, HOLTMANN M, KOEHLER HH, STOLTE M

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Helicobacter pylori Infection, Gastric Cancer and Gastropanel

level and Helicobacter pylori antibody test for predicting the histology of gastric neoplasm. Journal of Digestive Diseases 2014; 15:293-8. 15. TU H., SUN L., DONG X., GONG Y., XU O., JING J., et al. Temporal changes in serum biomarkers and risk for progression of gastric precancerous lesions: A longitudinal study. Int J Cancer. 2015; 136(2):425-34. 16. YEH J.M., HUR C., WARD Z., SCHRAG D., GOLDIE S.J. Gastric adenocarcinoma screening and prevention in the era of new biomarker and endoscopic technologies: A cost-effectiveness analysis

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HLA Genotyping using Next Generation Sequencing

. 2012; 13 :341. 32. METZKER ML. Sequencing technologies – the next generation . Nat Rev Genet. 2010; 11 (1):31-46. 33. GRADA A., WEINBRECHT K. Next-generation sequencing: methodology and application . J Invest Dermatol. 2013; 133 (8):e11. 34. MELDRUM C, DOYLE MA, TOTHILL RW. Next-generation sequencing for cancer diagnostics: a practical perspective. Clin Biochem Rev. 2011; 32 (4):177-95. 35. ROBINSON J., HALLIWELL JA., MCWILLIAM H., LOPEZ R., PARHAM P., MARSH SG. The IMGT/HLA database . Nucleic Acids Res. 2013; 41 (Database issue

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How much bloating in the irritable bowel syndrome?

, Talley Nj, Thompson Wg, Corazziari E, Whitehead We. The Functional Gastrointestinal Disorders: Diagnosis, Pathophysiology And Treatment. Mclean, Va: Degnon Associates. 1994. 8. Drossman Da, Corazziari E, Talley Nj, Et Al. Rome Ii. The Functional Gastrointestinal Disorders. Mclean, Va: Degnon. 2000. 9. Ottawa On. Trimebutine Maleate And Pinaverium Bromide For Irritable Bowel Syndrome: A Review Of The Clinical Effectiveness, Safety And Guidelines. Canadian Agency For Drugs And Technologies In Health. 2015 Nov. 10. Spiegel

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Liver stiffness in chronic hepatitis C virus infection

hepatitis C: A NICE medical technology guidance . Appl Health Econ Health Policy 2017; 15 :139-54. 20. GHARIB A.M., HAN M.A.T., MEISSNER E.G., KLEINER D.E., ZHAO X., MCLAUGHLIN M., et al. Magnetic resonance elastography shear wave velocity correlates with liver fibrosis and hepatic venous pressure gradient in adults with advanced liver disease . Biomed Res Int 2017; 2017 :2067-479. 21. THE AMERICAN ASSOCIATION FOR THE STUDY OF LIVER DISEASES AND THE INFECTIOUS DISEASES SCOCIETY OF AMERICA. HCV Guidance: recommendations for testing, managing, and treating

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