Haemophilia in the context of Haemophilia B


Haemophilia in the context of Haemophilia B

⭐ Core Definition: Haemophilia

Haemophilia (British English), or hemophilia (American English) (from Ancient Greek αἷμα (haîma) 'blood' and φιλία (philía) 'love of'), is a mostly inherited genetic disorder that impairs the body's ability to make blood clots, a process needed to stop bleeding. This results in people bleeding for a longer time after an injury, easy bruising, and an increased risk of bleeding inside joints or the brain. Those with a mild case of the disease may have symptoms only after an accident or during surgery. Bleeding into a joint can result in permanent damage while bleeding in the brain can result in long term headaches, seizures, or an altered level of consciousness.

There are two main types of haemophilia: haemophilia A, which occurs due to low amounts of clotting factor VIII, and haemophilia B, which occurs due to low levels of clotting factor IX. They are typically inherited from one's parents through an X chromosome carrying a nonfunctional gene. Most commonly found in men, haemophilia can affect women too, though very rarely. A woman would need to inherit two affected X chromosomes to be affected, whereas a man would only need one X chromosome affected. It is possible for a new mutation to occur during early development, or haemophilia may develop later in life due to antibodies forming against a clotting factor.

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Haemophilia in the context of Alexei Nikolaevich, Tsarevich of Russia

Alexei Nikolaevich (Russian: Алексей Николаевич Романов, romanized: Aleksey Nikolaevich Romanov; 12 August [O.S. 30 July] 1904 – 17 July 1918) was the last Russian tsesarevich (heir apparent). He was the youngest child and only son of Tsar Nicholas II and Tsarina Alexandra Feodorovna. He was born with haemophilia, which his parents tried treating with the methods of peasant faith healer Grigori Rasputin.

After the February Revolution of 1917, the Romanovs were sent into internal exile in Tobolsk, Siberia. After the October Revolution, the family was initially to be tried in a court of law, before the intensification of the Russian Civil War made execution increasingly favorable in the eyes of the Soviet government. With White Army soldiers rapidly approaching, the Ural Regional Soviet ordered the murder of Alexei, the rest of his family, and four remaining retainers on 17 July 1918. Rumors persisted for decades that Alexei had escaped his execution, with multiple impostors claiming his identity. Alexei's remains, along with those of his sister Maria (or Anastasia), were ultimately discovered in a secondary grave near the rest of the Romanov family in 2007. On 17 July 1998, the 80th anniversary of their execution, Alexei's parents, three of his sisters, and the four retainers, were formally interred in the Cathedral of St. Peter and Paul, while Alexei's and Maria's (or Anastasia's) bones remain in Russian state archives. The Romanov family was canonized as passion bearers by the Russian Orthodox Church in 2000.

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Haemophilia in the context of J. B. S. Haldane

John Burdon Sanderson Haldane FRS (/ˈhɔːldn/; 5 November 1892 – 1 December 1964), nicknamed "Jack" or "JBS", was a British-born scientist who later moved to India and acquired Indian citizenship. He worked in the fields of physiology, genetics, evolutionary biology, and mathematics. With innovative use of statistics in biology, he was one of the founders of neo-Darwinism. Despite his lack of an academic degree in the field, he taught biology at the University of Cambridge, the Royal Institution, and University College London. Renouncing his British citizenship, he became an Indian citizen in 1961 and worked at the Indian Statistical Institute until his death in 1964.

Haldane's article on abiogenesis in 1929 introduced the "primordial soup theory", which became the foundation for the concept of the chemical origin of life. He established human gene maps for haemophilia and colour blindness on the X chromosome, and codified Haldane's rule on sterility in the heterogametic sex of hybrids in species. He correctly proposed that sickle-cell disease confers some immunity to malaria. He was the first to suggest the central idea of in vitro fertilisation, as well as concepts such as hydrogen economy, cis and trans-acting regulation, coupling reaction, molecular repulsion, the darwin (as a unit of evolution), and organismal cloning.

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Haemophilia in the context of AIDS denialism

HIV/AIDS denialism is the belief, despite evidence to the contrary, that the human immunodeficiency virus (HIV) does not cause acquired immune deficiency syndrome (AIDS). Some of its proponents reject the existence of HIV, while others accept that HIV exists but argue that it is a harmless passenger virus and not the cause of AIDS. Insofar as they acknowledge AIDS as a real disease, they attribute it to some combination of sexual behavior, recreational drugs, malnutrition, poor sanitation, haemophilia, or the effects of the medications used to treat HIV infection (antiretrovirals).

The scientific consensus is that the evidence showing HIV to be the cause of AIDS is conclusive and that HIV/AIDS denialist claims are pseudoscience based on conspiracy theories, faulty reasoning, cherry picking, and misrepresentation of mainly outdated scientific data. As evidence mounted against denialism, combined with those with HIV/AIDS living much longer, these claims stopped being believed. With the rejection of these arguments by the scientific community, HIV/AIDS denialist material is now targeted at less scientifically sophisticated audiences and spread mainly through the Internet, increased substantially since the COVID-19 pandemic.

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Haemophilia in the context of Family history (medicine)

In medicine, a family history (FH or FHx) consists of information about disorders of direct blood relatives of the patient. Genealogy typically includes very little of the medical history of the family, but the medical history could be considered a specific subset of the total history of a family. Accurate knowledge of a patient's family history may identify a predisposition to developing certain illnesses, which can inform clinical decisions and allow effective management or even prevention of conditions.

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Haemophilia in the context of Romanov Tercentenary

The Romanov Tercentenary (Russian: Трёхсотле́тие до́ма Рома́новых, romanizedTrokhsotlétiye dóma Románovykh, lit.'Tercentenary of the House of Romanov') was a country-wide celebration, marked in the Russian Empire from February 1913, in celebration of the ruling House of Romanov. After a grand display of wealth and power in St. Petersburg, and a week of receptions at the Winter Palace, the imperial family embarked on a tour following Mikhail I Romanov's route after he was elected tsar by the Zemsky Sobor of 1613, a sort of pilgrimage to the towns of ancient Muscovy associated with the Romanov dynasty, in May.

It has been described as an 'extravaganza of pageantry' and a tremendous propaganda exercise; but among its principal goals were to 'inspire reverence and popular support for the principle of autocracy', and also a reinvention of the past, 'to recount the epic of the "popular tsar", so as to invest the monarchy with a historical legitimacy and an image of enduring permanence at this anxious time when its right to rule was being challenged by Russia's emerging democracy', a retreat 'to the past, hoping it would save them from the future'. Throughout the jubilee, the leitmotiv as it were was the cult of seventeenth century Muscovy, with its patrimonialism (with the tsar owning Russia as a private fiefdom), personal rule with the tsar a representation of God on earth, and the concept of a mystical union between the 'Little Father Tsar' and his Orthodox subjects, who revered and adored him. In the celebrations, the symbols of the tsar were in the centre, with all symbols of the state pushed far into the background.

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Haemophilia in the context of Haemophilia in European royalty

Haemophilia figured prominently in the history of European royalty in the 19th and 20th centuries. Queen Victoria and her husband, Prince Albert of the United Kingdom, through two of their five daughters – Princess Alice and Princess Beatrice – passed the mutation to various royal houses across the continent, including the royal families of Spain, Germany and Russia. Victoria's youngest son, Prince Leopold, Duke of Albany, also had the disease, though none of her three elder sons did. Tests on the remains of the Romanov imperial family show that the specific form of haemophilia passed down by Queen Victoria was probably the relatively rare haemophilia B. The presence of haemophilia B within the European royal families was well known, with the condition once popularly termed the 'royal disease.'

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