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Disorders of Hemostasis01:24

Disorders of Hemostasis

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Hepatitis is an inflammatory condition of the liver most commonly caused by hepatotropic viruses (A–E), though non-infectious causes such as alcohol and drugs also exist.Hepatitis AHepatitis A virus (HAV) is a non-enveloped RNA virus of the Picornaviridae family. It is primarily transmitted via the fecal-oral route, typically through ingestion of contaminated food or water. After ingestion, HAV enters the bloodstream through the oropharynx or intestinal epithelium and reaches the liver.
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Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...
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Blood transfusion is a therapeutic measure to restore the blood volume after extensive blood loss due to an accident or a medical procedure. Blood transfusion involves drawing a certain amount of blood from a suitable donor and infusing it into the recipient.
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Colloidal solids are solid particles suspended in solution. They are usually negatively charged, attracting a compact primary layer of positively charged ions, which attract more counterions to form an electrical double layer. Electrostatic repulsion between the charged double layers prevents the particles from colliding, stabilizing the colloids. These solids are often undesirable because they can contain toxins that are difficult to remove. Coagulation is a technique that helps aggregate and...
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Hemophilia in Iran.

Akbar Dorgalaleh1, Ghazaleh Dadashizadeh2, Taregh Bamedi3

  • 1a Department of Hematology and Blood Transfusion, School of Allied Medical Sciences , Iran University of Medical Sciences , Tehran , Iran.

Hematology (Amsterdam, Netherlands)
|February 26, 2016
PubMed
Summary

This review summarizes data on 5369 Iranian patients with Hemophilia A (HA) and B (HB), detailing genetic defects, common bleeding symptoms, and treatment strategies. Findings on inhibitor development and management offer insights for global hemophilia care.

Keywords:
Clinical presentationHemophiliaIranManagementMolecular analysis

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Area of Science:

  • Hematology
  • Genetics
  • Public Health

Background:

  • Hemophilia A (HA) and B (HB) are significant bleeding disorders, with Iran ranking ninth globally in patient population.
  • Numerous studies have investigated various facets of hemophilia in Iran.

Purpose of the Study:

  • To consolidate comprehensive data on Iranian patients with HA and HB.
  • To analyze molecular, clinical, and treatment aspects, including inhibitor development and management.
  • To enhance global understanding of hemophilia and its management.

Main Methods:

  • A systematic review of English and Persian literature.
  • Searches conducted on MEDLINE and Scientific Information Database up to 2015.

Main Results:

  • Data on 5369 patients (4438 HA, 931 HB) in Iran.
  • Genetic defects identified in approximately one-fifth of HA patients.
  • Common clinical presentations include hemarthrosis, epistaxis, ecchymosis, and post-extraction bleeding.
  • Treatment primarily involves on-demand factor replacement therapy (FVIII/FIX concentrates, cryoprecipitate, FFP).
  • Inhibitor development reported in 223 HA and 6 HB patients, managed with rFVIIa and aPCC.
  • Iran's per capita FVIII usage is high globally; FIX usage is highest in the Eastern Mediterranean region.

Conclusions:

  • The extensive data from Iranian patients provide valuable insights applicable to other regions, particularly developing countries.
  • Understanding genetic defects and treatment outcomes is crucial for improving patient care worldwide.